|
1
|
Chirico V, Silipigni L, Tripodi F, Conti G, Rulli I, Granata F, Cinquegrani A, Santoro D, Gitto E, Chimenz R. Management dilemma of anti-GBM disease and p-ANCA-associated vasculitis with necrotizing skin lesions in a pediatric patient. Pediatr Nephrol 2025:10.1007/s00467-025-06721-5. [PMID: 40029412 DOI: 10.1007/s00467-025-06721-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/24/2024] [Revised: 02/01/2025] [Accepted: 02/03/2025] [Indexed: 03/05/2025]
Abstract
Diffuse alveolar hemorrhage and acute glomerulonephritis characterize pulmonary-renal syndrome, in which anti-glomerular basement membrane antibodies (anti-GBM) and anti-neutrophil cytoplasmic antibodies (ANCA) are often assessed. The treatment is complex, requiring a multidisciplinary approach, based on immunosuppressant therapies, mechanical ventilation, plasma exchange (PLEX), and kidney replacement therapy. This clinical case describes a 7-year-old female hospitalized for edema, hypertension, and acute kidney failure. Laboratory tests showed the coexistence of ANCA and anti-GBM autoantibodies. A kidney biopsy revealed necrotizing crescentic glomerulonephritis with linear deposits of IgG along the GBM. Corticosteroids and cyclophosphamide, followed by rituximab and PLEX represented the therapeutical strategies. Hemodialysis was needed for the fluid overload and acute kidney injury (AKI) management. Posterior reversible leukoencephalopathy syndrome (PRES), hemorrhagic alveolitis, and cutaneous necrotizing skin lesions requiring the amputation of the limb complicated the clinical course, until the death of the patient. Early diagnosis and multidisciplinary and personalized therapies represent the management dilemmas for this disease. The evaluation of new treatments, such as avacopan and imlifidase, is much needed in pediatric-onset disease.
Collapse
Affiliation(s)
- Valeria Chirico
- Pediatric Nephrology and Dialysis Unit, University Hospital "G. Martino", 98124, Messina, Italy
| | - Lorena Silipigni
- Pediatric Nephrology and Dialysis Unit, University Hospital "G. Martino", 98124, Messina, Italy
| | - Filippo Tripodi
- Pediatric Nephrology and Dialysis Unit, University Hospital "G. Martino", 98124, Messina, Italy
| | - Giovanni Conti
- Pediatric Nephrology and Dialysis Unit, University Hospital "G. Martino", 98124, Messina, Italy
| | - Immacolata Rulli
- Neonatal and Pediatric Intensive Care Unit, Department of Human Pathology in Adult and Developmental Age "Gaetano Barresi", University of Messina, 98124, Messina, Italy
| | - Francesca Granata
- Department of Biomedical and Dental Sciences and of Morphological and Functional Images, Section of Radiological Sciences, University of Messina, Messina, Italy
| | - Antonella Cinquegrani
- Department of Biomedical and Dental Sciences and of Morphological and Functional Images, Section of Radiological Sciences, University of Messina, Messina, Italy
| | - Domenico Santoro
- Unit of Nephrology and Dialysis, Department of Clinical and Experimental Medicine, University of Messina, 98125, Messina, Italy
| | - Eloisa Gitto
- Neonatal and Pediatric Intensive Care Unit, Department of Human Pathology in Adult and Developmental Age "Gaetano Barresi", University of Messina, 98124, Messina, Italy
| | - Roberto Chimenz
- Pediatric Nephrology and Dialysis Unit, University Hospital "G. Martino", 98124, Messina, Italy.
| |
Collapse
|
|
2
|
Çimen Güneş E, Tekgöz E, Çolak S, Sayın S, Şirin H, Aylı M, Çınar M, Yılmaz S. Therapeutic apheresis treatment in rheumatic diseases: Insights from a single-center experience. Ther Apher Dial 2025; 29:123-130. [PMID: 39188015 DOI: 10.1111/1744-9987.14199] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2024] [Revised: 07/29/2024] [Accepted: 08/14/2024] [Indexed: 08/28/2024]
Abstract
INTRODUCTION We aimed to evaluate the characteristics of the patients with a rheumatologic disease who underwent TPE. METHOD A single-center, retrospective study was conducted between January 2016 and June 2023. RESULTS Twenty patients with a median age of 51 years received a median of 6 TPE sessions. Concurrently, immunosuppressive therapy was administered to 18 (90%) of them. During the follow-up period, 9 patients (45%) died. Creatinine (p = 0.001), C-reactive protein (p = 0.001), sedimentation rate (p = 0.002), leukocyte (p = 0.003), thrombocyte (p = 0.003), and neutrophil (p = 0.003) counts was decreased after TPE. Similarly, in the ROC analysis of post TPE laboratory parameters, urea, creatinine, CRP, hemoglobin, platelets, and lymphocytes predicted mortality with areas under the curve values ranging from 0.747 to 0.869. In the Cox regression analysis for mortality, creatinine was predictive for mortality (p = 0.030), HR 1.59 (95% CI: 1.05-2.41). CONCLUSION In rheumatologic conditions, TPE is beneficial to fill the gap until the effects of immunosuppressants become apparent.
Collapse
Affiliation(s)
- Ezgi Çimen Güneş
- Department of Internal Medicine, Division of Rheumatology, Gulhane Training and Research Hospital, University of Health Sciences, Ankara, Turkey
| | - Emre Tekgöz
- Department of Internal Medicine, Division of Rheumatology, Gulhane Training and Research Hospital, University of Health Sciences, Ankara, Turkey
| | - Seda Çolak
- Department of Internal Medicine, Division of Rheumatology, Gulhane Training and Research Hospital, University of Health Sciences, Ankara, Turkey
| | - Selim Sayın
- Department of Internal Medicine, Division of Haematology, Gulhane Training and Research Hospital, University of Health Sciences, Ankara, Turkey
| | - Hülya Şirin
- Department of Public Health, Gulhane School of Medicine, University of Health Science, Ankara, Turkey
| | - Meltem Aylı
- Department of Internal Medicine, Division of Haematology, Gulhane Training and Research Hospital, University of Health Sciences, Ankara, Turkey
| | - Muhammet Çınar
- Department of Internal Medicine, Division of Rheumatology, Gulhane Training and Research Hospital, University of Health Sciences, Ankara, Turkey
| | - Sedat Yılmaz
- Department of Internal Medicine, Division of Rheumatology, Gulhane Training and Research Hospital, University of Health Sciences, Ankara, Turkey
| |
Collapse
|
|
3
|
Fernández-Pérez FJ, Fernández-Moreno N, Soria-López E, Rodriguez-González FJ, Fernández-Galeote FJ, Lifante-Oliva A, Ruíz-Hernández C, Escalante-Quijaite E, Rivas-Ruiz F. Granulocyte and monocyte adsorptive apheresis (GMA) in patients with inflammatory bowel disease: A useful therapeutic tool not just in ulcerative colitis but also in Crohn's disease. GASTROENTEROLOGIA Y HEPATOLOGIA 2024; 47:502196. [PMID: 38710467 DOI: 10.1016/j.gastrohep.2024.502196] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/15/2023] [Revised: 01/27/2024] [Accepted: 02/14/2024] [Indexed: 05/08/2024]
Abstract
INTRODUCTION Granulocyte and monocyte adsorptive apheresis (GMA) removes neutrophils and monocytes from peripheral blood, preventing their incorporation into the inflamed tissue also influencing cytokine balance. Published therapeutic efficacy in ulcerative colitis (UC) is more consistent than in Crohn's disease (CD). We assessed clinical efficacy of GMA in UC and CD 4 weeks after last induction session, at 3 and 12 months, sustained remission and corticosteroid-free remission. PATIENTS AND METHOD Retrospective observational study of UC and CD patients treated with GMA. Partial Disease Activity Index-DAIp in UC and Harvey-Bradshaw Index-HBI in CD assessed efficacy of Adacolumn® with induction and optional maintenance sessions. RESULTS We treated 87 patients (CD-25, UC-62), 87.3% corticosteroid-dependent (CSD), 42.5% refractory/intolerant to immunomodulators. In UC, remission and response were 32.2% and 19.3% after induction, 35.5% and 6.5% at 12 weeks and 29% and 6.5% at 52 weeks. In CD, remission rates were 60%, 52% and 40% respectively. In corticosteroid-dependent and refractory or intolerant to INM patients (UC-41, CD-14), 68.3% of UC achieved remission or response after induction, 51.2% at 12 weeks and 46.3% at 52 weeks, and 62.3%, 64.3% and 42.9% in CD. Maintained remission was achieved by 66.6% in CD and 53.1% in UC. Up to 74.5% of patients required corticosteroids at some timepoint. Corticosteroid-free response/remission was 17.7% in UC and 24% in CD. CONCLUSIONS GMA is a good therapeutic tool for both in UC and CD patients. In corticosteroid-dependent and refractory or intolerant to INM patients it avoids biological therapy or surgery in up to 40% of them in one year.
Collapse
|
|
4
|
Guo J, Huang X, Li J, Zhang X, Zhong P, Lu G, Wang X, Luo W, Ning Y. Efficiency of therapeutic plasma exchange in critically ill systemic rheumatologic diseases: A single-center 9-year experience. Ther Apher Dial 2024; 28:784-792. [PMID: 38751182 DOI: 10.1111/1744-9987.14144] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2023] [Revised: 04/07/2024] [Accepted: 05/02/2024] [Indexed: 09/03/2024]
Abstract
INTRODUCTION Therapeutic plasma exchange (TPE), an effective method to eliminate harmful soluble mediators associated with tissue injury, serves as a crucial intervention for systemic rheumatologic diseases (SRDs). However, its value in critically ill SRDs remains uncertain. This retrospective study aims to evaluate the efficacy of TPE in SRDs. METHODS Critically ill SRD patients admitted to the department of intensive care unit of a large tertiary hospital receiving TPE from January 2011 to December 2019 were included. RESULTS A total of 91 critically ill SRD patients received TPE were enrolled. Their mean age was 47.67 ± 16.35 years with a female predominance (n = 68). Significant decrease in SOFA score post-TPE treatment was observed (p < 0.05). There were no TPE-related fatalities. Improvement was observed in 64 (70.32%) patients. CONCLUSION This study shows favorable clinical outcomes. TPE may be an acceptable treatment option for critically ill SRD patients.
Collapse
Affiliation(s)
- Jing Guo
- Department of Intensive Care Unit, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China
- School of Medicine, Xiamen University, Xiamen, Fujian, China
- The School of Clinical Medicine, Fujian Medical University, Fuzhou, Fujian, China
| | - Xiaolong Huang
- Department of Intensive Care Unit, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China
- School of Medicine, Xiamen University, Xiamen, Fujian, China
- The School of Clinical Medicine, Fujian Medical University, Fuzhou, Fujian, China
| | - Jianhua Li
- Department of Intensive Care Unit, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China
- School of Medicine, Xiamen University, Xiamen, Fujian, China
- The School of Clinical Medicine, Fujian Medical University, Fuzhou, Fujian, China
| | - Xiaorong Zhang
- Department of Intensive Care Unit, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China
- School of Medicine, Xiamen University, Xiamen, Fujian, China
- The School of Clinical Medicine, Fujian Medical University, Fuzhou, Fujian, China
| | - Ping Zhong
- BE and Phase I Clinical Trial Center, The First Affiliated Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, Fujian, China
| | - Guiyang Lu
- Department of Intensive Care Unit, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China
- School of Medicine, Xiamen University, Xiamen, Fujian, China
- The School of Clinical Medicine, Fujian Medical University, Fuzhou, Fujian, China
| | - Xinxin Wang
- Department of Intensive Care Unit, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China
- School of Medicine, Xiamen University, Xiamen, Fujian, China
- The School of Clinical Medicine, Fujian Medical University, Fuzhou, Fujian, China
| | - Weiyuan Luo
- Department of Intensive Care Unit, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China
- School of Medicine, Xiamen University, Xiamen, Fujian, China
- The School of Clinical Medicine, Fujian Medical University, Fuzhou, Fujian, China
| | - Yaogui Ning
- Department of Intensive Care Unit, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China
- School of Medicine, Xiamen University, Xiamen, Fujian, China
- The School of Clinical Medicine, Fujian Medical University, Fuzhou, Fujian, China
| |
Collapse
|
|
5
|
Dalkiran T, Mercan M, Ipek S, Güllü UU, Kandur Y, Acipayam C, Dilber C. Therapeutic Plasma Exchange in Pediatric Patients: Results from a Single Center. J Pediatr Intensive Care 2024; 13:282-285. [PMID: 39629152 PMCID: PMC11379518 DOI: 10.1055/s-0041-1742252] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/17/2021] [Accepted: 12/08/2021] [Indexed: 10/19/2022] Open
Abstract
Therapeutic plasma exchange (TPE) can be applied as an effective therapeutic option in children with hematological, neurological, nephrological, and autoimmune/rheumatic disorders. We aimed to report our TPE experience in pediatric patients. In this article, we retrospectively reviewed the records of pediatric patients who underwent TPE between 2019 and 2021. A total of 128 TPE sessions were performed in 25 patients (13 males,12 females; mean age 59.6 ± 11.7 [3-198] months). The TPE indications were sepsis with/without multiorgan dysfunction syndrome in five patients, acute liver failure, hemolytic uremic syndrome caused by Shiga toxin, and autoimmune hemolytic anemia in three patients, respectively, multiple sclerosis, autoimmune encephalitis, and multisystem inflammatory syndrome in children (MIS-C) in two patients each, and myasthenia gravis crisis, meningococcemia, hemolytic uremic syndrome caused by coronavirus disease 2019, hemophagocytic lymphohistiocytosis, autoimmune encephalitis, and metabolic disease (fatty acid oxidation defect, liver failure) in one patient each. Based on our findings, we proposed that the American Society for Apheresis criteria should be updated according to newly described clinical conditions such as MIS-C.
Collapse
Affiliation(s)
- Tahir Dalkiran
- Department of Pediatric Intensive Care, Necip Fazil City Hospital, Kahramanmaras, Türkiye
| | - Mehmet Mercan
- Department of Pediatrics, Necip Fazil City Hospital, Kahramanmaras, Türkiye
| | - Sevcan Ipek
- Department of Pediatrics, Faculty of Medicine, Kahramanmaras Sütçü İmam University, Kahramanmaras, Türkiye
| | - Ufuk Utku Güllü
- Department of Pediatrics, Faculty of Medicine, Kahramanmaras Sütçü İmam University, Kahramanmaras, Türkiye
| | - Yasar Kandur
- Department of Pediatric Nephrology, Faculty of Medicine, Kirikkale University, Kirikkale, Türkiye
| | - Can Acipayam
- Department of Pediatric Hematology and Oncology, Faculty of Medicine, Kahramanmaras Sütçü İmam University, Kahramanmaras, Türkiye
| | - Cengiz Dilber
- Department of Pediatric Neurology, Faculty of Medicine, Kahramanmaras Sütçü İmam University, Kahramanmaras, Türkiye
| |
Collapse
|
|
6
|
Mesaros S, Pekmezovic T, Martinovic V, Ivanovic J, Tamas O, Dinic M, Drulovic J. Beneficial therapeutic plasma exchange response in the treatment of severe relapses in patients with multiple sclerosis. Acta Neurol Belg 2024:10.1007/s13760-024-02606-w. [PMID: 39044118 DOI: 10.1007/s13760-024-02606-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2023] [Accepted: 07/13/2024] [Indexed: 07/25/2024]
Abstract
PURPOSE Therapeutic plasma exchange (PLEX) is effective as a second-line treatment of severe relapses of multiple sclerosis (MS) that failed to respond to standard steroid therapy. Our objective was to evaluate the effectiveness of PLEX in the severe MS relapses in a cohort of patients treated at Neurology Clinic, University Clinical Centre of Serbia, Belgrade, from 2007 until 2020. METHODS This retrospective study comprised 107 MS patients with 127 severe relapses treated with PLEX. Majority of our patients suffered from relapsing remitting MS (83.2%), 12.1% had secondary progressive MS and 4.7% had primary progressive MS. Mean age was 39.2 years (range, 19-79 years), female/male ratio 2.3:1. Pulse corticosteroid treatment was used before PLEX in 99.3% of patients. Median EDSS score at nadire during relapse was 6.0 (range 2.0-10.0). After PLEX, 73.8% relapses showed a marked clinical improvement, 7.1% showed mild improvement and in 19.0% there was no improvement. Median EDSS at discharge was 4.0 (6.0 at nadir of relapse vs. 4.0 at discharge; p<0.0001) and it was sustained at the same level, 6 month after PLEX. Multivariate regression analysis showed that higher EDSS at nadir during relapse (OR=0.63, 95% CI 0.41-0.96, p=0.039) and older age (OR=1.07, 95% CI 1.02- 1.12, p=0.010) were significantly associated with poor treatment response after 6- month follow-up. Adverse events occurred in 17.3 of procedures and they were completely resolved. CONCLUSION Our study in a large cohort of MS patients confirmed that PLEX is effective.
Collapse
Affiliation(s)
- Sarlota Mesaros
- Neurology Clinic, University Clinical Centre of Serbia, Dr Subotica 6,, Belgrade, 11000, Serbia
- Faculty of Medicine, University of Belgrade, Belgrade, Serbia
| | | | - Vanja Martinovic
- Neurology Clinic, University Clinical Centre of Serbia, Dr Subotica 6,, Belgrade, 11000, Serbia
| | - Jovana Ivanovic
- Faculty of Medicine, University of Belgrade, Belgrade, Serbia
| | - Olivera Tamas
- Neurology Clinic, University Clinical Centre of Serbia, Dr Subotica 6,, Belgrade, 11000, Serbia
- Faculty of Medicine, University of Belgrade, Belgrade, Serbia
| | - Marija Dinic
- Faculty of Medicine, University of Belgrade, Belgrade, Serbia
| | - Jelena Drulovic
- Neurology Clinic, University Clinical Centre of Serbia, Dr Subotica 6,, Belgrade, 11000, Serbia.
- Faculty of Medicine, University of Belgrade, Belgrade, Serbia.
| |
Collapse
|
|
7
|
Augusto JF, Benden C, Diekmann F, Zuckermann A. The value of extracorporeal photopheresis as an immunosuppression-modifying approach in solid organ transplantation: a potential solution to an unmet medical need. Front Immunol 2024; 15:1371554. [PMID: 38846942 PMCID: PMC11154098 DOI: 10.3389/fimmu.2024.1371554] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/16/2024] [Accepted: 05/07/2024] [Indexed: 06/09/2024] Open
Abstract
Allograft rejection is a critical issue following solid organ transplantation (SOT). Immunosuppressive therapies are crucial in reducing risk of rejection yet are accompanied by several significant side effects, including infection, malignancy, cardiovascular diseases, and nephrotoxicity. There is a current unmet medical need with a lack of effective minimization strategies for these side effects. Extracorporeal photopheresis (ECP) has shown potential as an immunosuppression (IS)-modifying technique in several SOT types, with improvements seen in acute and recurrent rejection, allograft survival, and associated side effects, and could fulfil this unmet need. Through a review of the available literature detailing key areas in which ECP may benefit patients, this review highlights the IS-modifying potential of ECP in the four most common SOT procedures (heart, lung, kidney, and liver transplantation) and highlights existing gaps in data. Current evidence supports the use of ECP for IS modification following SOT, however there is a need for further high-quality research, in particular randomized control trials, in this area.
Collapse
Affiliation(s)
- Jean-François Augusto
- Department of Nephrology-Dialysis-Transplantation, University Hospital of Angers, Angers, France
| | | | - Fritz Diekmann
- Renal Transplantation Unit, Department of Nephrology and Kidney Transplantation, Hospital Clinic, Barcelona, Spain
| | - Andreas Zuckermann
- Department of Cardiac Surgery, Medical University of Vienna, Vienna, Austria
| |
Collapse
|
|
8
|
Kim HJ, Chung Y, Kim H, Hwang SH, Oh HB, Ko DH. Trends in category and grade for therapeutic plasma exchange in the latest guideline on therapeutic apheresis by the American Society for Apheresis: Hurdles in pursuing evidence-based medicine. Vox Sang 2024; 119:476-482. [PMID: 38357715 DOI: 10.1111/vox.13603] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2023] [Revised: 01/08/2024] [Accepted: 02/01/2024] [Indexed: 02/16/2024]
Abstract
BACKGROUND AND OBJECTIVES The Writing Committee of American Society for Apheresis released the ninth edition of guidelines for therapeutic apheresis in 2023. Categories have been a part of the guidelines since the first edition, and the grading system was introduced in the fifth edition, with updates in every new edition. In this study, we investigated the category and grade change trends through the latest five editions, focusing on therapeutic plasma exchange, to suggest future directions as part of evidence-based medicine. MATERIALS AND METHODS Categories and grades for therapeutic plasma exchange (TPE) were collected and analysed from the fifth through ninth editions. We aligned classification changes to the ninth edition's clinical context and compared its categories and grades with those introduced in the guideline. RESULTS Among 166 total indications in the ninth edition, 118 included TPE procedure, either as a sole treatment or as one of the therapeutic apheresis techniques. The total number of indications changed, but Category III remained predominant throughout the editions. Similarly, Grade 2C consistently emerged as the most prevalent grade. Notably, 24 cases had grade changes. Of the 16 cases with evidence quality changes, the quality weakened in six and improved in 10. Evidence levels were not improved throughout the study period for 102 clinical conditions. CONCLUSION To address gaps in evidence quality, international collaboration is imperative to establish comprehensive large-scale studies or randomized controlled trials. This will refine the use of therapeutic apheresis, including TPE, to foster evidence-based advancements in clinical practice.
Collapse
Affiliation(s)
- Han Joo Kim
- Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
| | - Yousun Chung
- Department of Laboratory Medicine, Kangdong Sacred Heart Hospital, Seoul, South Korea
| | - Hyungsuk Kim
- Department of Laboratory Medicine, Seoul National University Hospital, Seoul, South Korea
| | - Sang-Hyun Hwang
- Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
| | - Heung-Bum Oh
- Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
| | - Dae-Hyun Ko
- Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea
| |
Collapse
|
|
9
|
Bharati J, Jhaveri KD, Salama AD, Oni L. Anti-Glomerular Basement Membrane Disease: Recent Updates. ADVANCES IN KIDNEY DISEASE AND HEALTH 2024; 31:206-215. [PMID: 39004460 DOI: 10.1053/j.akdh.2024.04.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/30/2023] [Revised: 04/01/2024] [Accepted: 04/24/2024] [Indexed: 07/16/2024]
Abstract
Anti-glomerular basement membrane disease is a small-vessel vasculitis involving the kidneys (∼90%) and the lungs (∼60%). Antibodies against the glomerular basement membrane are directly pathogenic in anti-glomerular basement membrane disease; however, recent research has highlighted the critical role of T cells. Novel autoantigens within the glomerular basement membrane are also now recognized. Atypical forms of the disease are reported along with preceding triggers, such as immune checkpoint inhibitors, immunomodulatory drugs, and vaccines. Kidney outcomes in anti-glomerular basement membrane disease remain poor despite significant improvement in patient survival in the last 2 to 3 decades. Treatment typically relies on combined plasmapheresis with intensive immunosuppression. Dialysis dependency at presentation is a dominant predictor of kidney outcome. Histologically, a low (<10%) percentage of normal glomeruli, 100% crescents, together with dialysis dependency at presentation, is associated with poor kidney outcomes. In such cases, an individualized approach weighing the risks and benefits of treatment is recommended. There is a need for better ways to stop the toxic inflammatory activity associated with this disease. In this narrative review, we discuss recent updates on the pathogenesis and management of anti-glomerular basement membrane disease relevant to patients of all ages.
Collapse
Affiliation(s)
- Joyita Bharati
- Glomerular Center, Division of Kidney Diseases and Hypertension, Northwell Health, Great Neck
| | - Kenar D Jhaveri
- Glomerular Center, Division of Kidney Diseases and Hypertension, Northwell Health, Great Neck
| | - Alan D Salama
- University College London (UCL) Department of Renal Medicine, Royal Free Hospital, London, UK
| | - Louise Oni
- Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, UK; Department of Paediatric Nephrology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
| |
Collapse
|
|
10
|
Delong Z, Fugui W, Xin H, Houqing L. A rare case of bilateral frontal lobe lesions due to thyroid storm. ARCHIVES OF ENDOCRINOLOGY AND METABOLISM 2024; 68:e230254. [PMID: 38652700 PMCID: PMC11081050 DOI: 10.20945/2359-4292-2023-0254] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 07/04/2023] [Accepted: 09/21/2023] [Indexed: 04/25/2024]
Abstract
Thyroid storm is a rare but well-known life-threatening complication that occurs due to acute exacerbation of thyrotoxicosis with the increased levels of circulating thyroid hormones. Reports of metabolic encephalopathy associated with thyroid storm are scarce. We describe the case of a 23-year-old male patient with no previous history of abnormal thyroid function who had consumed excessive amounts of alcohol before disease onset. The patient was found unconscious and febrile on a roadside by a passerby and was admitted to our hospital's emergency department. His primary clinical presentation included hyperthermia (40.8 °C), nodal tachycardia (180 beats/min), seizures, coma, and hypoglycemia (2.18 mmol/L). The hypoglycemia was quickly corrected after admission, but his level of consciousness showed no improvement. With aggressive screening, the patient was found to have severe thyroid dysfunction (T3 = 6.67 nmol/L, T4 = 252.00 nmol/L, free T3 = 29.20 pmol/L, free T4 = 65.30 pmol/L, and TSH = 0.001 μIU/mL). After medical treatment, plasmapheresis, hemofiltration, and hemoperfusion, the patient showed substantial improvement in thyroid hormone levels and stabilization of vital signs, but the impaired consciousness and seizures persisted. Multiple computed tomography scans revealed brain abnormalities. Magnetic resonance imaging performed after tracheal extubation revealed bilateral frontal lobe lesions. We reported a case of metabolic encephalopathy in a patient with life-threatening thyroid storm and bilateral frontal lobe lesions. Hypoglycemia may have been involved in the development of encephalopathy in our patient. Health care providers should consider thyroid storm in the differential diagnosis of hyperthermia, seizures, and coma. Early plasmapheresis, hemofiltration, and hemoperfusion can lower T4 levels and improve prognosis in patients with thyroid storm and encephalopathy.
Collapse
Affiliation(s)
- Zhang Delong
- Intensive Care Unit, Tongling Clinical College (Tongling People's Hospital), Anhui Medical University, Tongling, 244000, China
| | - Wang Fugui
- Intensive Care Unit, Tongling Clinical College (Tongling People's Hospital), Anhui Medical University, Tongling, 244000, China
| | - Hu Xin
- Intensive Care Unit, Tongling Clinical College (Tongling People's Hospital), Anhui Medical University, Tongling, 244000, China
| | - Lu Houqing
- Intensive Care Unit, Tongling Clinical College (Tongling People's Hospital), Anhui Medical University, Tongling, 244000, China,
| |
Collapse
|
|
11
|
Priyatha V, Shah AA, Ijaz S, Rahman SU, Ullah H, Tariq W, Salih N. Severe Spinal Cord Inflammation in a Young Woman Diagnosed With Systemic Lupus Erythematosus: A Case Study. Cureus 2024; 16:e54325. [PMID: 38500920 PMCID: PMC10945468 DOI: 10.7759/cureus.54325] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/16/2024] [Indexed: 03/20/2024] Open
Abstract
We describe a case of longitudinally extensive transverse myelitis (LETM), an uncommon and dangerous complication of systemic lupus erythematosus (SLE) that struck a 22-year-old woman with SLE. Chronic autoimmune illness (e.g., SLE) affects the skin, kidneys, joints, blood, and neurological system, among other organs. LETM is a condition where the spinal cord becomes inflamed and damaged, causing neurological problems, such as weakness, sensory loss, and bladder dysfunction. The patient presented with abdominal pain, vomiting, body aches, and fatigue, followed by shock, hypoxia, urinary retention, and constipation. Moreover, she had severe and asymmetric weakness, sensory loss, and areflexia in her limbs. She was diagnosed with LETM based on a nerve conduction study and MRI of the spine, which showed a motor neuron disease pattern and T2 hyperintense signals throughout the spinal cord gray and white matter. She responded well to immunoglobulins, plasma exchange, and high-dose steroids as treatment. Although her prognosis is favorable, there might be some lingering neurological issues or limitations. This instance highlights the significance of treating individuals with SLE as soon as possible after developing LETM.
Collapse
Affiliation(s)
- Vemparala Priyatha
- Internal Medicine, All India Institute of Medical Sciences, Bhubaneswar, Bhubaneswar, IND
| | - Aizaz A Shah
- Internal Medicine, Hayatabad Medical Complex Peshawar, Peshawar, PAK
| | - Saad Ijaz
- Internal Medicine, Hayatabad Medical Complex Peshawar, Peshawar, PAK
| | - Sohaib Ur Rahman
- Internal Medicine, Hayatabad Medical Complex Peshawar, Peshawar, PAK
| | - Hidayat Ullah
- Medical-C Unit, Hayatabad Medical Complex Peshawar, Peshawar, PAK
| | - Wardah Tariq
- Ophthalmology, Rehman Medical Institute, Haripur, PAK
| | - Noman Salih
- Internal Medicine, Hayatabad Medical Complex Peshawar, Peshawar, PAK
| |
Collapse
|
|
12
|
Deville K, Charlton N, Askenazi D. Use of extracorporeal therapies to treat life-threatening intoxications. Pediatr Nephrol 2024; 39:105-113. [PMID: 36988694 DOI: 10.1007/s00467-023-05937-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/25/2022] [Revised: 03/01/2023] [Accepted: 03/02/2023] [Indexed: 03/30/2023]
Abstract
Toxic ingestions are a significant cause of pediatric morbidity and mortality, with some requiring extracorporeal removal for therapy. Given the emergent and life-threatening nature of such scenarios, it is paramount that clinicians caring for intoxicated children be familiar with the subject. This review summarizes the following: (a) the properties of a substance which lend it amenable to removal; (b) the current extracorporeal treatment modalities available for such removal (of which hemodialysis is typically the ideal choice); (c) an introduction and framework to use a quick reference guide from the Extrip organization, which has a website available to guide clinicians' rapid decisions; and (d) new membranes/approaches that may optimize clearance of certain intoxications.
Collapse
Affiliation(s)
- Kyle Deville
- Department of Pediatrics, Division of Pediatric Nephrology, University of Alabama at Birmingham, 1600 5Th Ave S, Park Place Suite 202, Birmingham, AL, 35233, USA
| | - Nathan Charlton
- Department of Emergency Medicine, Division of Toxicology, University of Virginia, Charlottesville, USA
| | - David Askenazi
- Department of Pediatrics, Division of Pediatric Nephrology, University of Alabama at Birmingham, 1600 5Th Ave S, Park Place Suite 202, Birmingham, AL, 35233, USA.
| |
Collapse
|
|
13
|
Fuhrman DY, Thadani S, Hanson C, Carcillo JA, Kellum JA, Park HJ, Lu L, Kim-Campbell N, Horvat CM, Arikan AA. Therapeutic Plasma Exchange Is Associated With Improved Major Adverse Kidney Events in Children and Young Adults With Thrombocytopenia at the Time of Continuous Kidney Replacement Therapy Initiation. Crit Care Explor 2023; 5:e0891. [PMID: 37066071 PMCID: PMC10097539 DOI: 10.1097/cce.0000000000000891] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/18/2023] Open
Abstract
Therapeutic plasma exchange (TPE) has been shown to improve organ dysfunction and survival in patients with thrombotic microangiopathy and thrombocytopenia associated with multiple organ failure. There are no known therapies for the prevention of major adverse kidney events after continuous kidney replacement therapy (CKRT). The primary objective of this study was to evaluate the effect of TPE on the rate of adverse kidney events in children and young adults with thrombocytopenia at the time of CKRT initiation. DESIGN Retrospective cohort. SETTING Two large quaternary care pediatric hospitals. PATIENTS All patients less than or equal to 26 years old who received CKRT between 2014 and 2020. INTERVENTIONS None. MEASUREMENTS AND MAIN RESULTS We defined thrombocytopenia as a platelet count less than or equal to 100,000 (cell/mm3) at the time of CKRT initiation. We ascertained major adverse kidney events at 90 days (MAKE90) after CKRT initiation as the composite of death, need for kidney replacement therapy, or a greater than or equal to 25% decline in estimated glomerular filtration rate from baseline. We performed multivariable logistic regression and propensity score weighting to analyze the relationship between the use of TPE and MAKE90. After excluding patients with a diagnosis of thrombotic thrombocytopenia purpura and atypical hemolytic uremic syndrome (n = 6) and with thrombocytopenia due to a chronic illness (n = 2), 284 of 413 total patients (68.8%) had thrombocytopenia at CKRT initiation (51% female). Of the patients with thrombocytopenia, the median (interquartile range) age was 69 months (13-128 mo). MAKE90 occurred in 69.0% and 41.5% received TPE. The use of TPE was independently associated with reduced MAKE90 by multivariable analysis (odds ratio [OR], 0.35; 95% CI, 0.20-0.60) and by propensity score weighting (adjusted OR, 0.31; 95% CI, 0.16-0.59). CONCLUSIONS Thrombocytopenia is common in children and young adults at CKRT initiation and is associated with increased MAKE90. In this subset of patients, our data show benefit of TPE in reducing the rate of MAKE90.
Collapse
Affiliation(s)
- Dana Y Fuhrman
- Department of Critical Care Medicine, Division of Pediatric Critical Care Medicine, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA
- Department of Pediatrics, Division of Nephrology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA
- The Center for Critical Care Nephrology, Department of Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA
| | - Sameer Thadani
- Department of Pediatrics, Division of Nephrology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX
| | - Claire Hanson
- Department of Critical Care Medicine, Division of Pediatric Critical Care Medicine, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA
| | - Joseph A Carcillo
- Department of Critical Care Medicine, Division of Pediatric Critical Care Medicine, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA
- The Center for Critical Care Nephrology, Department of Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA
| | - John A Kellum
- The Center for Critical Care Nephrology, Department of Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA
| | - Hyun Jung Park
- Department of Human Genetics, Graduate School of Public Health, University of Pittsburgh, Pittsburgh, PA
| | - Liling Lu
- Department of Biostatistics, University of Pittsburgh, Pittsburgh, PA
| | - Nahmah Kim-Campbell
- Department of Critical Care Medicine, Division of Pediatric Critical Care Medicine, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA
| | - Christopher M Horvat
- Department of Critical Care Medicine, Division of Pediatric Critical Care Medicine, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA
- Department of Pediatrics, Division of Health Informatics, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA
| | - Ayse Akcan Arikan
- Department of Pediatrics, Division of Nephrology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX
- Department of Pediatrics, Division of Critical Care Medicine, Baylor College of Medicine, Texas Children's Hospital, Houston, TX
| |
Collapse
|
|
14
|
Cold AIHA and the best treatment strategies. HEMATOLOGY. AMERICAN SOCIETY OF HEMATOLOGY. EDUCATION PROGRAM 2022; 2022:90-95. [PMID: 36485161 PMCID: PMC9821124 DOI: 10.1182/hematology.2022000369] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Cold-reactive autoimmune hemolytic anemia (AIHA) is rare among the hemolytic anemias. It results when 1 of a variety of processes causes the generation of immunoglobulin M (IgM) autoantibodies against endogenous erythrocytes, resulting in complement activation and predominantly intravascular hemolysis. Cold AIHA is typically a primary lymphoproliferative disorder with marrow B-cell clones producing pathogenic IgM. More rarely, secondary cold AIHA (cAIHA) can develop from malignancy, infection, or other autoimmune disorders. However, in children cAIHA is typically post infection, mild, and self-limited. Symptoms include a sequelae of anemia, fatigue, and acrocyanosis. The severity of disease is variable and highly dependent on the thermal binding range of the autoantibody. In adults, treatment has most commonly focused on reducing antibody production with rituximab-based regimens. The addition of cytotoxic agents to rituximab improves response rates, but at the expense of tolerability. Recent insights into the cause of cold agglutinin disease as a clonal disorder driven by complement form the basis of newer therapeutic options. While rituximab-based regimens are still the mainstay of therapy, options have now expanded to include complement-directed treatments and other B-cell-directed or plasma-cell-directed therapies.
Collapse
|
|
15
|
Rathnayaka RMMKN, Nishanthi Ranathunga PEA, Kularatne SAM, Sugathadasa K. Therapeutic Plasma Exchange for Venom-Induced Thrombotic Microangiopathy Following Hump-Nosed Pit Viper (Genus: Hypnale) Bites: A Prospective Observational Study. Wilderness Environ Med 2022; 33:386-398. [PMID: 36244888 DOI: 10.1016/j.wem.2022.07.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2022] [Revised: 07/23/2022] [Accepted: 07/26/2022] [Indexed: 11/07/2022]
Abstract
INTRODUCTION -Thrombotic microangiopathy (TMA), which is the triad of acute kidney injury (AKI), microangiopathic hemolytic anemia (MAHA), and thrombocytopenia, is a rare complication of snakebites, and in Sri Lanka, it is commonly seen with hump-nosed pit viper (HNPV) bites. METHODS -We conducted a prospective observational study of patients with AKI caused by HNPV bites in Teaching Hospital, Ratnapura, Sri Lanka for 6 y, commencing in June 2015. Some patients with TMA underwent therapeutic plasma exchange (TPE) and some did not. These 2 groups were compared. Statistical analysis was carried out using Minitab 18.1. Data were presented as median (IQR). RESULTS -There were 52 (8%) patients with TMA, of whom 21 (45%) were in the TPE group and 26 (55%) were in the non-TPE group. TPE improved time to platelet correction (4 d [IQR, 4-5 d] vs 7 d [IQR, 5-9 d]; P=0.009), time to MAHA correction (5 d [IQR, 3-4 d] vs 7 d [IQR, 6-9 d]; P=0.004), time to prothrombin time (PT)/international normalized ratio (INR) correction (1 d [IQR, 1-2 d] vs 3 d [IQR, 3-4 d]; P=0.003), and time to 20 min whole blood clotting test (WBCT20) correction (2 d [IQR, 1-2 d] vs 3 d [1QR 2-3 d]; P=0.020). Renal recovery was predicted by TPE (P=0.048) and highest creatinine level (P=0.001). There was no association between TPE and dialysis dependency at discharge (P=0.597), length of hospital stay (P=0.220), and the number of dialysis cycles prior to discharge (P=0.540). TPE did not improve the number of blood transfusions (5 packs [IQR, 3-8.5 packs] vs 4 packs [IQR, 0-9 packs]; P=0.290). CONCLUSIONS -TPE is effective for TMA in the early correction of platelet counts, MAHA, PT/INR, and WBCT20 in HNPV bites.
Collapse
Affiliation(s)
- R M M K Namal Rathnayaka
- Department of Pharmacology, Faculty of Medicine, Sabaragamuwa University of Sri Lanka, Hidellana, Ratnapura, Sri Lanka; Department of Veterinary Pathobiology, Faculty of Veterinary Medicine and Animal Science, University of Peradeniya, Peredeniya, Sri Lanka; Intensive Care Unit, Teaching Hospital Ratnapura, Sri Lanka.
| | | | - S A M Kularatne
- Faculty of Medicine, University of Peradeniya, Peredeniya, Sri Lanka
| | | |
Collapse
|
|
16
|
Martin K, Deleveaux S, Cunningham M, Ramaswamy K, Thomas B, Lerma E, Madariaga H. The presentation, etiologies, pathophysiology, and treatment of pulmonary renal syndrome: A review of the literature. Dis Mon 2022; 68:101465. [PMID: 36008166 DOI: 10.1016/j.disamonth.2022.101465] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/15/2022]
Abstract
Pulmonary renal syndrome (PRS) is a constellation of different disorders that cause both rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage. While antineutrophil cytoplasmic antibody associated vasculitis and anti-glomerular basement membrane disease are the predominant causes of PRS, numerous other mechanisms have been shown to cause this syndrome, including thrombotic microangiopathies, drug exposures, and infections, among others. This syndrome has high morbidity and mortality, and early diagnosis and treatment is imperative to improve outcomes. Treatment generally involves glucocorticoids and immunosuppressive agents, but treatment targeted to the underlying disorder can improve outcomes and mitigate side effects. Familiarity with the wide range of possible causes of PRS can aid the clinician in workup, diagnosis and early initiation of treatment. This review provides a summary of the clinical presentation, etiologies, pathophysiology, and treatment of PRS.
Collapse
Affiliation(s)
| | | | | | | | - Beje Thomas
- Medstar Georgetown University Hospital, United States
| | - Edgar Lerma
- Advocate Christ Medical Center, United States
| | | |
Collapse
|
|
17
|
Dowsett T, Oni L. Anti-glomerular basement membrane disease in children: a brief overview. Pediatr Nephrol 2022; 37:1713-1719. [PMID: 34767075 PMCID: PMC8586640 DOI: 10.1007/s00467-021-05333-z] [Citation(s) in RCA: 12] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/03/2021] [Revised: 09/28/2021] [Accepted: 09/29/2021] [Indexed: 01/04/2023]
Abstract
Anti-glomerular basement membrane disease (Anti-GBM), previously known as Goodpasture syndrome, is an extremely rare cause of rapidly progressive glomerulonephritis and chronic kidney disease stage 5 (CKD5) in children. It is associated with acute pulmonary haemorrhage and it has a poor prognosis. It is classified as an autoimmune, small-vessel vasculitis caused by autoantibody formation against the alpha-3 chain in type IV collagen found in the glomerular basement membrane. Evidence of anti-GBM antibodies in serum or histologically are required for diagnosis. Treatment in children is based on very limited adult data and often involves the use of acute apheresis to rapidly remove circulating factors coupled with intensive immunosuppression such as cyclophosphamide and intravenous corticosteroids. There is also an emerging role for the use of biologic agents such as B cell depletion. The evidence base in children with anti-GBM disease is extremely limited. Multi-centre international collaboration is required to provide insight into this disease, better describe its prognosis and work towards improving outcomes. This review article summarises the key features of this disease in children, highlights treatment options and considers areas of unmet need.
Collapse
Affiliation(s)
- Thomas Dowsett
- Department of Paediatric Nephrology, Royal Manchester Children's Hospital, Oxford Road, Manchester, M13 9WL, UK
| | - Louise Oni
- Department of Paediatric Nephrology, Alder Hey Children's NHS Foundation Trust Hospital, Eaton Road, Liverpool, L12 2AP, UK.
- Department of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Eaton Road, Liverpool, L12 2AP, UK.
| |
Collapse
|
|
18
|
Dandu H, Kumar V, Goel A, Khetan D, Chandra T, Bharti VR. A preliminary experience of plasma exchange in liver failure. Asian J Transfus Sci 2022; 16:209-213. [PMID: 36687541 PMCID: PMC9855211 DOI: 10.4103/ajts.ajts_115_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/13/2021] [Revised: 10/01/2021] [Accepted: 12/25/2021] [Indexed: 11/13/2022] Open
Abstract
INTRODUCTION Plasma exchange (PLEX) is one of the experimental modalities of treatment for liver failure. We report our experience of PLEX in patients with acute-(ALF) or acute-on-chronic (ACLF) liver failure. METHODS Hemodynamically stable adult patients with ALF or ACLF, encephalopathy, model for end-stage liver disease (MELD) score ≥ 15, and clinical worsening/no improvement after 72-h of inpatient care were included. PLEX cycles repeated every 48 h, each of 2.5-4.0 h duration with 1-1.5 times of estimated plasma volume, were given. PLEX cycle was repeated till either of the end-points were achieved (i) MELD < 20 for 48 h or reaches below the baseline, whichever is lower, (ii) completed three PLEX cycles, (iii) hemodynamic instability, (iv) or outcome achieved. Outcome of interest was categorized as favorable (discharged in stable condition) or unfavorable (death or discharge in moribund condition). Data are expressed as median (interquartile range). RESULTS Sixteen patients (age 35 [27-48] years; male 8; ALF 5, ACLF 11; MELD 33 [27-37]; CLIF-SOFA 10 [8.5-12]) were included. Participants received 2 (1-3) cycles of PLEX during 13 (11-25) days of hospitalization. Overall, serum bilirubin, INR, creatinine, MELD, and CLIF-SOFA scores were significantly improved after PLEX. Five patients (5/16, 31%) had complete resolution of HE. Eight patients (50%) had a favorable outcome. Those with favorable outcome had significant improvement in serum bilirubin, INR, and CLIF-SOFA scores as compared to those with unfavorable outcome. CONCLUSION PLEX may be effective in patients with ALF or ACLF. More data are needed to establish its role in the management of liver failure.
Collapse
Affiliation(s)
- Himanshu Dandu
- Department of Medicine, King George's Medical University, Lucknow, Uttar Pradesh, India
| | - Vivek Kumar
- Department of Internal Medicine, King George's Medical University, Lucknow, Uttar Pradesh, India
| | - Amit Goel
- Department of Gastro-Medicine, SGPGIMS, Lucknow, Uttar Pradesh, India
| | - Dheeraj Khetan
- Department of Transfusion Medicine, SGPGIMS, Lucknow, Uttar Pradesh, India
| | - Tulika Chandra
- Department of Transfusion Medicine, King George's Medical University, Lucknow, Uttar Pradesh, India
| | - Vipin Raj Bharti
- Department of Medicine, King George's Medical University, Lucknow, Uttar Pradesh, India
| |
Collapse
|
|
19
|
Dumortier J, Chouik Y, Hequet O, Hervieu V, Boillot O. Rituximab and plasmapheresis for severe post-transplant auto-immune hepatitis. Clin Res Hepatol Gastroenterol 2022; 46:101844. [PMID: 34920142 DOI: 10.1016/j.clinre.2021.101844] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2021] [Revised: 11/29/2021] [Accepted: 12/03/2021] [Indexed: 02/04/2023]
Affiliation(s)
- Jérôme Dumortier
- Hospices civils de Lyon, Hôpital Edouard Herriot, Fédération des Spécialités Digestives, Lyon, France; Université Claude Bernard Lyon 1, Lyon, France.
| | - Yasmina Chouik
- Hospices civils de Lyon, Hôpital Edouard Herriot, Fédération des Spécialités Digestives, Lyon, France; Université Claude Bernard Lyon 1, Lyon, France
| | - Olivier Hequet
- Hospices civils de Lyon, Centre hospitalier Lyon-Sud, Etablissement français de sang, Lyon, France
| | - Valérie Hervieu
- Université Claude Bernard Lyon 1, Lyon, France; Hospices civils de Lyon, Hôpital Edouard Herriot, Service d'Anatomie Pathologique, Lyon, France
| | - Olivier Boillot
- Hospices civils de Lyon, Hôpital Edouard Herriot, Fédération des Spécialités Digestives, Lyon, France; Université Claude Bernard Lyon 1, Lyon, France
| |
Collapse
|
|
20
|
Gumulec J, Demel I, Lančová K, Drbohlavová E, Piegzová A, Kořístek Z, Navrátil M, Černý V. Selected severe „haematological“ syndromes in adult intensive care patients. VNITRNI LEKARSTVI 2022; 68:498-507. [PMID: 36575067 DOI: 10.36290/vnl.2022.107] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
Abstract
Haemophagocytic syndrome, diffuse alveolar haemorrhage, catastrophic antiphospholipid syndrome and various types of thrombotic microangiopathies are rare conditions with significant morbidity and mortality. A common feature is late diagnosis, which can affect the success of treatment. The aim of this review article is to summarize the basic diagnostic and therapeutic steps of the present subpopulation of critically ill patients.
Collapse
|
|
21
|
Valenzuela-Vallejo L, Meléndrez-Vásquez D, Durán-Ventura P, Rivera-Nieto C, Lema A, Fernandez M. Severe hypertriglyceridemia as a cause of necrotizing pancreatitis in a pediatric patient with familial hyperchylomicronemia syndrome: A case report. SAGE Open Med Case Rep 2022; 10:2050313X221109972. [PMID: 35837325 PMCID: PMC9274426 DOI: 10.1177/2050313x221109972] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2022] [Accepted: 06/09/2022] [Indexed: 01/04/2023] Open
Abstract
Familial hyperchylomicronemia syndrome is a monogenic autosomal recessive disorder that causes severe and refractory hypertriglyceridemia. This uncommon condition is challenging to diagnose and treat and can lead to comorbidities such as acute pancreatitis. Although treatment options are limited in the pediatric population, strict diets and treatments approved for other dyslipidemias may be implemented in familial hyperchylomicronemia syndrome, given the lack of pharmacological interventions available. We report a 14-year-old female presented to the emergency room with abdominal pain suggestive of acute pancreatitis. Biochemical analysis revealed a triglyceride value of 4260 mg/dL. Treatment for triglyceride reduction with a strict CHILD-2 triglyceride-lowering diet, insulin infusion, fibrates, and multiple plasmapheresis were initially insufficient. Primary hypertriglyceridemia was suspected, and genetic testing identified a homozygous pathogenic variant in the lipoprotein lipase gene, diagnosing familial hyperchylomicronemia syndrome. She was discharged with a maximum dose of fibrate, statin, omega-3 fatty acids, and a restrictive diet. At her 1-month and 9-month follow-ups, her triglyceride values were 756 and 495 mg/dL, respectively, without incident complications. Familial hyperchylomicronemia syndrome is an uncommon condition with limited available literature and treatment options, especially in the pediatric population. Acute pancreatitis secondary to severe hypertriglyceridemia is a condition with a high risk of mortality which requires prompt clinical suspicion and treatment.
Collapse
Affiliation(s)
- Laura Valenzuela-Vallejo
- School of Medicine and Health Sciences—Universidad del Rosario, Bogotá, Colombia
- Endocrinology Department, Fundación Cardioinfantil, Instituto de Cardiología, Bogotá, Colombia
| | - Daniela Meléndrez-Vásquez
- School of Medicine and Health Sciences—Universidad del Rosario, Bogotá, Colombia
- Endocrinology Department, Fundación Cardioinfantil, Instituto de Cardiología, Bogotá, Colombia
| | - Paola Durán-Ventura
- Pediatric Endocrinologist, Pediatrics Endocrinology Department, Fundación Cardioinfantil-Instituto de Cardiología, Bogotá, Colombia
- School of Medicine and Health Sciences—Universidad del Rosario, Bogotá, Colombia
| | - Carolina Rivera-Nieto
- School of Medicine and Health Sciences—Universidad del Rosario, Bogotá, Colombia
- Genetics Department, Fundación Cardioinfantil-Instituto de Cardiología, Bogotá, Colombia
| | - Adriana Lema
- Pediatric Endocrinologist, Pediatrics Endocrinology Department, Fundación Cardioinfantil-Instituto de Cardiología, Bogotá, Colombia
- School of Medicine and Health Sciences—Universidad del Rosario, Bogotá, Colombia
| | - Monica Fernandez
- Pediatric Endocrinologist, Pediatrics Endocrinology Department, Fundación Cardioinfantil-Instituto de Cardiología, Bogotá, Colombia
- School of Medicine and Health Sciences—Universidad del Rosario, Bogotá, Colombia
| |
Collapse
|
|
22
|
Autoimmune Hemolytic Anemia in Chronic Lymphocytic Leukemia: A Comprehensive Review. Cancers (Basel) 2021; 13:cancers13225804. [PMID: 34830959 PMCID: PMC8616265 DOI: 10.3390/cancers13225804] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/25/2021] [Revised: 11/06/2021] [Accepted: 11/11/2021] [Indexed: 12/19/2022] Open
Abstract
Simple Summary This review analyzes the occurrence, clinical characteristics, and prognostic impact and treatment of autoimmune hemolytic anemia (AIHA) in chronic lymphocytic leukemia (CLL). Autoimmune hemolytic anemia is observed in about 10% of CLL. Pathogenesis is multifactorial involving humoral, cellular, and innate immunity, so the different mechanisms are well described in this review which also focuses on drugs associated to CLL-AIHA and on difficulties to diagnose it. There is a comprehensive revision of the main published casistics and then of the treatments; in particular the paper analyzes the main chemo-immunotherapeutic agents used in this setting. Since the therapy depends on the presence and severity of clinical symptoms, disease status, and comorbidities, treatment is nowadays more individualized in CLL and also in CLL-AIHA. Patients not responding to corticosteroids and rituximab are treated with CLL-specific drugs as per current guidelines according to age and comorbidities and new targeted agents against BCR and BCL-2 which can be given orally and have few side effects, are very effective both in progressive CLL and in situations such as AIHA. Abstract Chronic lymphocytic leukemia (CLL) patients have a greater predisposition to develop autoimmune complications. The most common of them is autoimmune hemolytic anemia (AIHA) with a frequency of 7–10% of cases. Pathogenesis is multifactorial involving humoral, cellular, and innate immunity. CLL B-cells have damaged apoptosis, produce less immunoglobulins, and could be responsible for antigen presentation and releasing inflammatory cytokines. CLL B-cells can act similar to antigen-presenting cells activating self-reactive T helper cells and may induce T-cell subsets imbalance, favoring autoreactive B-cells which produce anti-red blood cells autoantibodies. Treatment is individualized and it depends on the presence and severity of clinical symptoms, disease status, and comorbidities. Corticosteroids are the standardized first-line treatment; second-line treatment comprises rituximab. Patients not responding to corticosteroids and rituximab should be treated with CLL-specific drugs as per current guidelines according to age and comorbidities. New targeted drugs (BTK inhibitors and anti BCL2) are recently used after or together with steroids to manage AIHA. In the case of cold agglutinin disease, rituximab is preferred, because steroids are ineffective. Management must combine supportive therapies, including vitamins; antibiotics and heparin prophylaxis are indicated in order to minimize infectious and thrombotic risk.
Collapse
|
|
23
|
Hassaniazad M, Vahedi MS, Samimagham HR, Gharibzadeh A, Beyranvand S, Abbasi H, Nikpoor AR. Improvement of clinical outcome, laboratory findings and inflammatory cytokines levels using plasmapheresis therapy in severe COVID-19 cases. Respir Med 2021; 189:106669. [PMID: 34757278 PMCID: PMC8547850 DOI: 10.1016/j.rmed.2021.106669] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/20/2021] [Revised: 10/09/2021] [Accepted: 10/21/2021] [Indexed: 12/17/2022]
Abstract
INTRODUCTION Cytokine storm is one of the consequences of the severe forms of COVID-19 due to excessive immune response. In this study, we investigated the therapeutic effect of plasmapheresis and its role on the inflammatory cytokines levels in patients suffering from severe COVID-19. METHODS In plasmapheresis group, 22 severe cases of COVID-19 receiving three cycles of plasmapheresis with time interval of 24-36 h and 22 COVID-19 patients as the control group were enrolled. Clinical history and laboratory parameters as well as IL-1, IL-6, IFN-γ and IL-17 cytokines serum levels in the time points of before and after plasmapheresis were studied. RESULTS In severe COVID-19 patients, plasmapheresis significantly improved clinical and laboratory parameters such as cough, weakness, fever, blood oxygen saturation and CRP levels. Serum levels of IL-1, IL-6, IFN-γ and IL-17 in the group of patients receiving plasmapheresis, had a significant decrease following plasmapheresis courses. Although only IL-6 level in the control group had a significant decrease between the days 1-14 of disease. Also, at both time points of before and after plasmapheresis, serum levels of IL-1, IL-6, IFN-γ and IL-17 were inversely correlated to blood oxygen saturation. CONCLUSION Based on the obtained results, plasmapheresis therapy in severe forms of COVID-19 can effectively improve the clinical symptoms of the disease and reduce inflammatory markers. Therefore, it is suggested that plasmapheresis can be evaluated in standard treatment protocols for severe forms of COVID-19.
Collapse
Affiliation(s)
- Mehdi Hassaniazad
- Infectious and Tropical Diseases Research Center, Hormozgan Health Institute, Hormozgan University of Medical Sciences, Bandar Abbas, Iran
| | - Mohammad Sadegh Vahedi
- Infectious and Tropical Diseases Research Center, Hormozgan Health Institute, Hormozgan University of Medical Sciences, Bandar Abbas, Iran
| | - Hamid Reza Samimagham
- Clinical Research Development Center, Shahid Mohammadi Hospital, Hormozgan University of Medical Sciences, Bandar Abbas, Iran
| | - Abdollah Gharibzadeh
- Cardiovascular Research Center, Hormozgan University of Medical Sciences, Bandar Abbas, Iran
| | - Shirin Beyranvand
- Student Research Committee, Hormozgan University of Medical Sciences, Bandar Abbas, Iran
| | - Hossein Abbasi
- Student Research Committee, Hormozgan University of Medical Sciences, Bandar Abbas, Iran
| | - Amin Reza Nikpoor
- Molecular Medicine Research Center, Hormozgan Health Institute, Hormozgan University of Medical Sciences, Bandar Abbas, Iran.
| |
Collapse
|
|
24
|
Srinivas D, Sriganesh K, Chakrabarti D, Venkateswaran P. Effect of Therapeutic Plasma Exchange on Plasma Constituents in Neurointensive Care Unit Patients: A Retrospective Study. JOURNAL OF NEUROANAESTHESIOLOGY AND CRITICAL CARE 2021. [DOI: 10.1055/s-0041-1734412] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022] Open
Abstract
Abstract
Purpose Plasma exchange is one of the recommended therapeutic procedures for autoimmune neurological conditions and involves removal of plasma over multiple sessions for exclusion of autoantibodies responsible for the disease process. This study aimed to evaluate the changes in the concentration of plasma constituents with five cycles of alternate day therapeutic plasma exchange (TPE), identify contributing factors for hypoproteinemia, and examine its impact on clinical outcomes.
Methods This was a single-center, retrospective cohort study involving patients with autoimmune neurological diseases who underwent at least five cycles of TPE in the neurointensive care unit (NICU). Data regarding plasma protein concentrations, serum electrolytes, fluid input/output before and after every TPE cycle and clinical outcomes in terms of duration of ventilation, and NICU and hospital stay were collected from the medical records over a 1-year period.
Results The levels of plasma proteins (total protein, albumin and globulin) (p < 0.001), sodium (p < 0.001), calcium (p < 0.001), and hemoglobin (p = 0.002) declined significantly after TPE. Difference in plasma protein levels before and after TPE did not correlate with durations of mechanical ventilation and hospital and NICU stay. Difference in total protein and globulin correlated negatively with fluid balance and positively with daily protein intake (p < 0.05 for both).
Conclusion A significant decrease in plasma proteins and other plasma constituents is seen with TPE. Changes in plasma proteins are related to hemodilution and protein intake. Decrease in plasma proteins did not affect duration of hospital or NICU stay and duration of mechanical ventilation.
Collapse
Affiliation(s)
- Deepti Srinivas
- Department of Neuroanaesthesia and Neurocritical Care, Apollo Hospitals, Bangalore, Karnataka, India
| | - Kamath Sriganesh
- Department of Neuroanaesthesia and Neurocritical Care, National Institute of Mental Health and Neuro Sciences, Bangalore, Karnataka, India
| | - Dhritiman Chakrabarti
- Department of Neuroanaesthesia and Neurocritical Care, National Institute of Mental Health and Neuro Sciences, Bangalore, Karnataka, India
| | | |
Collapse
|
|
25
|
MESH Headings
- Anemia, Hemolytic, Autoimmune/diagnosis
- Anemia, Hemolytic, Autoimmune/pathology
- Anemia, Hemolytic, Autoimmune/physiopathology
- Anemia, Hemolytic, Autoimmune/therapy
- Blood Transfusion
- Complement Inactivating Agents/therapeutic use
- Glucocorticoids/therapeutic use
- Hemoglobinuria, Paroxysmal/diagnosis
- Hemoglobinuria, Paroxysmal/pathology
- Hemoglobinuria, Paroxysmal/physiopathology
- Hemoglobinuria, Paroxysmal/therapy
- Humans
- Immunologic Factors/therapeutic use
- Rituximab/therapeutic use
Collapse
Affiliation(s)
- Sigbjørn Berentsen
- From the Department of Research and Innovation, Haugesund Hospital, Helse Fonna Hospital Trust, Haugesund, Norway (S.B.); and the Hematology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan (W.B.)
| | - Wilma Barcellini
- From the Department of Research and Innovation, Haugesund Hospital, Helse Fonna Hospital Trust, Haugesund, Norway (S.B.); and the Hematology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan (W.B.)
| |
Collapse
|
|
26
|
Diana JS, Manceau S, Rabeony T, Elie C, Jolaine V, Zamora S, Aubart M, Salvi N, Bodemer C, Bader-Meunier B, Barnerias C, Iserin F, Chardot C, Lacaille F, Renolleau S, Salomon R, Joseph L, Cavazzana M, Lefrere F, Dupic L, Delville M. Therapeutic plasma exchange for life-threatening pediatric disorders. J Clin Apher 2021; 36:823-830. [PMID: 34469617 DOI: 10.1002/jca.21934] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/27/2020] [Revised: 07/12/2021] [Accepted: 08/17/2021] [Indexed: 01/04/2023]
Abstract
INTRODUCTION Therapeutic plasma exchange (TPE) is acknowledged to be an effective treatment in life-threatening pediatric disorders. Apheresis for pediatric diseases has been poorly investigated, and most studies to date featured small numbers of patients and lacked control groups. The objective of the present study was to evaluate the tolerance of TPE in pediatric patients. MATERIALS AND METHODS A retrospective cohort study via a web-based electronic case report form including pediatric patients referred for TPE between January 2005 and December 2014. RESULTS A total of 78 patients (median [range] age: 9.8 [0.53-17.93]) and 731 TPE procedures were analyzed. The indications were antibody-mediated rejection (n = 33; 42%) and desensitization therapy (n = 5; 6%) after solid organ or hematopoietic stem cell transplantation, thrombotic microangiopathy (n = 17; 22%), pediatric inflammatory diseases (n = 16; 21%), kidney diseases (n = 6; 8%), and hyperviscosity syndrome (n = 1; 1%). On average, each patient underwent six procedures during the first session [range: 1-19]. In the 2 weeks following the start of a session, 72 patients (92%) presented a total of 311 adverse events (AEs) potentially related to TPE. The risk of AEs was not related to the indication for TPE, the intensity of care, venous access, plasma substitute use, or body weight. None of the deaths was related to the TPE. CONCLUSION We studied one of the largest retrospective pediatric cohorts described to date. Our experience of TPE children's TPE feasibility concerned specific, life-threatening conditions and otherwise treatment-refractory diseases.
Collapse
Affiliation(s)
- Jean-Sebastien Diana
- Hôpital Necker Enfants Malades, Paris, France.,Université de Paris, Paris, France
| | - Sandra Manceau
- Hôpital Necker Enfants Malades, Paris, France.,Université de Paris, Paris, France
| | | | - Caroline Elie
- Université de Paris, Paris, France.,Institut Imagine, Paris, France
| | - Valerie Jolaine
- Université de Paris, Paris, France.,Institut Imagine, Paris, France
| | | | | | | | - Christine Bodemer
- Hôpital Necker Enfants Malades, Paris, France.,Université de Paris, Paris, France
| | | | | | | | - Christophe Chardot
- Hôpital Necker Enfants Malades, Paris, France.,Université de Paris, Paris, France
| | | | - Sylvain Renolleau
- Hôpital Necker Enfants Malades, Paris, France.,Université de Paris, Paris, France
| | - Remi Salomon
- Hôpital Necker Enfants Malades, Paris, France.,Université de Paris, Paris, France
| | | | - Marina Cavazzana
- Hôpital Necker Enfants Malades, Paris, France.,Université de Paris, Paris, France.,Institut Imagine, Paris, France
| | | | | | - Marianne Delville
- Hôpital Necker Enfants Malades, Paris, France.,Université de Paris, Paris, France.,Institut Imagine, Paris, France
| |
Collapse
|
|
27
|
Ipe TS, Davis AR, Raval JS. Therapeutic Plasma Exchange in Myasthenia Gravis: A Systematic Literature Review and Meta-Analysis of Comparative Evidence. Front Neurol 2021; 12:662856. [PMID: 34531809 PMCID: PMC8439193 DOI: 10.3389/fneur.2021.662856] [Citation(s) in RCA: 23] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/01/2021] [Accepted: 07/12/2021] [Indexed: 01/04/2023] Open
Abstract
Background: Patients with Myasthenia Gravis (MG) can be treated acutely with therapeutic plasma exchange (TPE) or intravenous immune globulin (IVIG). To date, there is no definitive understanding of which of the two treatments is more effective and safer. The purpose of this study was to systematically review the literature on the comparative efficacy and safety of TPE to other available treatments for MG. Methods: A systematic literature search for studies published between 1997 and 2017 was performed per Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines using two database sources, MEDLINE (through the PubMed database) and Cochrane Library. Results: The search strategy resulted in 535 articles whose abstracts were reviewed. Among these, 165 full texts articles were reviewed for eligibility and 101 articles were excluded. Of the 165 articles, 64 articles were included for a systematic literature and 11 articles for a meta-analysis. Conclusions: This systematic literature review and meta-analysis of treatment options showed that there was a higher response rate with TPE than IVIG in acute MG patients and patients undergoing thymectomy. There was no difference in mortality between the two treatment options. Our findings highlight the need for additional randomized clinical trials in these patients with MG.
Collapse
Affiliation(s)
- Tina S. Ipe
- Department of Pathology and Laboratory Medicine, University of Arkansas for Medical Sciences, Little Rock, AR, United States
| | - Adeola R. Davis
- Terumo Blood and Cell Technologies, Lakewood, CO, United States
| | - Jay S. Raval
- Department of Pathology, University of New Mexico, Albuquerque, NM, United States
| |
Collapse
|
|
28
|
Yabanoglu H, Sari R, Eksi Haydardedeoglu F, Kus M, Hargura AS, Arer IM. Preoperative Therapeutic Plasma Exchange and Surgical Treatment in Thyrotoxicosis Patients: A Single-Centre Retrospective Cohort Study. ACTA ENDOCRINOLOGICA (BUCHAREST, ROMANIA : 2005) 2021; 17:346-350. [PMID: 35342473 DOI: 10.4183/aeb.2021.346] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Context Therapeutic plasma exchange (TPE) provides time for thyroidectomy in thyrotoxic patients. Objective TPE is indicated in cases where antithyroid medications cannot be used due to the side effects or attain no adequate hormonal suppression response at the highest dosage and in cases of rapid onset of clinical symptoms. This study presents the treatment results of patients who underwent TPE and were subsequently operated for thyrotoxicosis. Design The patients who underwent thyroidectomy and TPE between January 1999 and February 2019 were retrospectively analyzed. Subjects and Methods The files of 27 patients with thyrotoxicosis who performed TPE prior to surgery were analyzed in relation to the demographic and clinical features. Results We included 15 (55.6%) females, 12 (44.4%) males with a mean age of 44 (23-82) years. The pre-TPE mean free thyroxine (fT4) level was 12 (5-46) pmol/L while free tri-iodothyronine (fT3) level was 34 (17-141) pmol/L. The post-TPE fT4 level was 6 (3-10) pmol/L while the fT3 level was 21 (12-41). There was one case of an allergic reaction during the procedure. In the postoperative follow-up, there was transient hypocalcemia in 8 (29%) patients, permanent hypocalcemia in 1 (3.7%) patient, and surgical site infection in 1 (3.7%) patient. Conclusion Preoperative TPE is an alternative treatment option for thyrotoxic patients. This is an especially effective treatment for patients with inadequate response or adverse reaction to antithyroid drugs or patients who need urgent surgery for thyroid storm.
Collapse
Affiliation(s)
- H Yabanoglu
- Baskent University, "Dr. Turgut Noyan" Teaching and Research Center, Department of General Surgery, Adana, Turkey
| | - R Sari
- Baskent University, "Dr. Turgut Noyan" Teaching and Research Center, Department of General Surgery, Adana, Turkey
| | | | - M Kus
- Baskent University, "Dr. Turgut Noyan" Teaching and Research Center, Department of General Surgery, Adana, Turkey
| | - A S Hargura
- Baskent University, "Dr. Turgut Noyan" Teaching and Research Center, Department of General Surgery, Adana, Turkey
| | - I M Arer
- Baskent University, "Dr. Turgut Noyan" Teaching and Research Center, Department of General Surgery, Adana, Turkey
| |
Collapse
|
|
29
|
Li Y, Qiu Z, Huang L, Cao C. Extracorporeal membrane oxygenation combined with sequential blood purification in the treatment of myocardial damage and cardiac arrest caused by mushroom poisoning. Toxicon 2021; 197:65-69. [PMID: 33872678 DOI: 10.1016/j.toxicon.2021.04.011] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/11/2021] [Revised: 03/29/2021] [Accepted: 04/14/2021] [Indexed: 01/04/2023]
Abstract
Mushroom poisoning is a common clinical problem. Severe mushroom poisoning often causes liver and kidney failure. Although severe myocardial damage is rare, the fatality rate is extremely high. This case report describes a 56-year-old male suffered severe myocardial damage, multiple organ dysfunction, circulatory failure, recurrent malignant arrhythmia, and cardiac arrest after the ingestion of wild mushrooms. He was administered venoarterial extracorporeal membrane oxygenation (VA-ECMO) combined with hemoperfusion, plasma exchange and continuous renal replacement therapy. The heart rhythm gradually stabilized 3 hours after ECMO surgery. On the 6th day after ECMO, heart function recovered. The patient was then weaned from ECMO, and he ultimately recovered and was discharged. In patients with fatal mushroom poisoning leading to refractory arrhythmia and cardiac arrest, early implementation of VA-ECMO combined with sequential blood purification treatment can improve the prognosis and increase the survival rate.
Collapse
Affiliation(s)
- Yang Li
- Department of Emergency Medicine, The First Affiliated Hospital of Nanchang University, No. 17 Yongwaizheng Street, Donghu District, Nanchang, 330006, Jiangxi Province, China
| | - Zhiqiang Qiu
- Department of Emergency Medicine, The First Affiliated Hospital of Nanchang University, No. 17 Yongwaizheng Street, Donghu District, Nanchang, 330006, Jiangxi Province, China
| | - Liang Huang
- Department of Emergency Medicine, The First Affiliated Hospital of Nanchang University, No. 17 Yongwaizheng Street, Donghu District, Nanchang, 330006, Jiangxi Province, China
| | - Chunshui Cao
- Department of Emergency Medicine, The First Affiliated Hospital of Nanchang University, No. 17 Yongwaizheng Street, Donghu District, Nanchang, 330006, Jiangxi Province, China.
| |
Collapse
|
|
30
|
Beştepe Dursun Z, Korkmaz S, Türe Z, Kaynar L, Dursun A, Çelik İ. Efficacy of therapeutic plasma exchange in patients with Crimean-Congo hemorrhagic fever. J Clin Apher 2021; 36:390-397. [PMID: 33485278 DOI: 10.1002/jca.21875] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/07/2020] [Revised: 12/22/2020] [Accepted: 01/04/2021] [Indexed: 12/13/2022]
Abstract
OBJECTIVE To examine the efficacy of therapeutic plasma exchange (TPE) in patients critically ill with Crimean-Congo hemorrhagic fever (CCHF). METHODS Patients with CCHF received supportive treatment (ST) or TPE. After laboratory and clinical evaluations, the patients were divided into mild, moderate, and severe CCHF groups according to the severity score index (SSI). To assess the efficacy of TPE, the incubation period, time of admission to hospital, hospitalization duration, mortality rate and times to recovery of the platelet count and aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were compared between patients receiving ST and TPE. RESULT A total of 119 confirmed CCHF cases was analyzed. The median SSIs were 7 in the TPE group and 5 in the ST group. The SSI stages, median incubation times and admission times were similar in the two groups. However, the duration of hospitalization was longer in the TPE group. The overall mortality rates were 9% (3 of 33 patients) in the TPE group and 16% (5 of 31 patients) in the ST group; the difference was significant. The platelet count recovered after a median of 6 (4-7) days in the ST group. CONCLUSION The mortality rate was lower in the TPE group than in the ST group, but the duration of hospitalization and the time to platelet recovery were longer in the TPE group than in the ST group. TPE did not contribute significantly to the prognosis of patients with intermediate-severity CCHF. However, TPE might be efficacious in patients with severe CCHF.
Collapse
Affiliation(s)
- Zehra Beştepe Dursun
- Department of Infectious Disease, Kayseri City Hospital, University of Health Science University, Kayseri, Turkey
| | - Serdal Korkmaz
- Department of Hematology, Kayseri City Hospital, University of Health Science University, Kayseri, Turkey
| | - Zeynep Türe
- Department of Infectious Disease, Faculty of Medicine, University of Erciyes, Kayseri, Turkey
| | - Leylagül Kaynar
- Department of Hematology, Faculty of Medicine, University of Erciyes, Kayseri, Turkey
| | - Adem Dursun
- Department of Paediatrics, Division of Pediatric Intensive Care, Erzurum City Hospital, University of Health Science University, Erzurum, Turkey
| | - İlhami Çelik
- Department of Infectious Disease, Kayseri City Hospital, University of Health Science University, Kayseri, Turkey
| |
Collapse
|
|
31
|
Park JA. Treatment of Diffuse Alveolar Hemorrhage: Controlling Inflammation and Obtaining Rapid and Effective Hemostasis. Int J Mol Sci 2021; 22:E793. [PMID: 33466873 PMCID: PMC7830514 DOI: 10.3390/ijms22020793] [Citation(s) in RCA: 21] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/10/2020] [Revised: 01/07/2021] [Accepted: 01/11/2021] [Indexed: 02/07/2023] Open
Abstract
Diffuse alveolar hemorrhage (DAH) is a life-threatening pulmonary complication in patients with hematologic malignancies or systemic autoimmune disorders. Pathologic findings show pulmonary capillaritis, bland hemorrhage, diffuse alveolar damage, and hemosiderin-laden macrophages, but in the majority of cases, pathogenesis remains unclear. Despite the severity and high mortality, the current treatment options for DAH remain empirical. Systemic treatment to control inflammatory activity including high-dose corticosteroids, cyclophosphamide, and rituximab and supportive care have been applied, but largely unsuccessful in critical cases. Activated recombinant factor VII (FVIIa) can achieve rapid local hemostasis and has been administered either systemically or intrapulmonary for the treatment of DAH. However, there is no randomized controlled study to evaluate the efficacy and safety, and the use of FVIIa for DAH remains open to debate. This review discusses the pathogenesis, diverse etiologies causing DAH, diagnosis, and treatments focusing on hemostasis using FVIIa. In addition, the risks and benefits of the off-label use of FVIIa in pediatric patients will be discussed in detail.
Collapse
Affiliation(s)
- Jeong A Park
- Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, NY 10065, USA
| |
Collapse
|
|
32
|
Abstract
Thrombocytopenia-associated multiple organ failure is a clinical phenotype encompassing a spectrum of syndromes associated with disseminated microvascular thromboses. Autopsies performed in patients that died with thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, or disseminated intravascular coagulation reveal specific findings that can differentiate these 3 entities. Significant advancements have been made in our understanding of the pathologic mechanisms of these syndromes. Von Willebrand factor and ADAMTS-13 play a central role in thrombotic thrombocytopenic purpura. Shiga toxins and the complement pathway drive the hemolytic uremic syndrome pathology. Tissue factor activity is vital in the development of disseminated intravascular coagulation.
Collapse
Affiliation(s)
- Trung C Nguyen
- Department of Pediatrics, Critical Care Medicine Section, Texas Children's Hospital/Baylor College of Medicine, 6651 Main Street, MC: E 1420, Houston, TX 77030, USA; The Center for Translational Research on Inflammatory Diseases (CTRID), The Michael E. DeBakey Veteran Administration Medical Center, Houston, TX 77030, USA.
| |
Collapse
|
|
33
|
Vinan-Vega M, Mantilla B, Jahan N, Peminda C, Nugent K, Lado-Abeal J, Rivas A. Usefulness of plasmapheresis in patients with severe complicated thyrotoxicosis. Proc (Bayl Univ Med Cent) 2020; 34:279-282. [PMID: 33678963 DOI: 10.1080/08998280.2020.1852007] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022] Open
Abstract
The standard treatment of complicated thyrotoxicosis and thyroid storm with the concomitant use of antithyroid medication, iodine, beta-blockers, and corticosteroids is successful in most cases. However, treatment options are limited when antithyroidal drugs cannot be used or in cases that are refractory to standard treatment. Plasmapheresis provides a safe and effective strategy when initial treatment fails, facilitating the transition to definitive treatments such as thyroidectomy. We present two adults with complicated thyrotoxicosis successfully treated with plasmapheresis as a bridge therapy to thyroidectomy or as an alternative to drug-induced toxicity.
Collapse
Affiliation(s)
- Myrian Vinan-Vega
- Division of Internal Medicine/Endocrinology, Texas Tech University Health Science Center, Lubbock, Texas
| | - Barbara Mantilla
- Division of Internal Medicine/Endocrinology, Texas Tech University Health Science Center, Lubbock, Texas
| | - Nusrat Jahan
- Division of Hematology, Texas Tech University Health Science Center, Lubbock, Texas
| | - Cabandugama Peminda
- Division of Endocrinology, Truman Medical Center-UMKC Health Sciences, Kansas City, Missouri
| | - Kenneth Nugent
- Division of Pulmonary and Critical Care, Texas Tech University Health Science Center, Lubbock, Texas
| | - Joaquin Lado-Abeal
- Division of Endocrinology, Truman Medical Center-UMKC Health Sciences, Kansas City, Missouri
| | - Ana Rivas
- Division of Internal Medicine/Endocrinology, Texas Tech University Health Science Center, Lubbock, Texas
| |
Collapse
|
|
34
|
Coffe C, Pouthier F, Barisien C, Slimane M, Sheytanova A. Therapeutic leukapheresis and thrombapheresis in medical emergencies. Transfus Apher Sci 2020; 59:102997. [PMID: 33189569 DOI: 10.1016/j.transci.2020.102997] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2023]
Abstract
The management of hyperleukocytosis or thrombocytosis by therapeutic cytapheresis in the early 21 st century is far from codified (universal). Therapeutic cytapheresis have been proposed to achieve more rapid cytoreduction in peripheral blood than old universal support in order to quickly prevent potential complications. But, there are no randomized studies demonstrating the superiority of cytapheresis over other treatments alone. In this short review, based on our own experience (since 1980), we will give the indications and the role of cytapheresis procedures and we will try to answer the questions: when is therapeutic cytapheresis appropriate and do they still have a place in 2020, especially as a medical emergency?
Collapse
Affiliation(s)
| | - Fabienne Pouthier
- Department of Cellular and Tissue Engineering, Etablissement Français du sang Bourgogne, Franche Comté, Besançon, France
| | - Christophe Barisien
- Department of Blood Donations, Etablissement Français du sang Bourgogne, Franche Comté, Besançon, France
| | - Mohamed Slimane
- Etablissement Français du sang Bourgogne, Franche Comté, Dijon, France
| | - Antoaneta Sheytanova
- Department of Therapeutic Apheresis, Etablissement Français du sang Bourgogne, Franche Comté, Besançon, France
| |
Collapse
|
|
35
|
Li C, Li H, Su W, Wen YB, Ye W, Ye WL, Cai JF, Qin XZ, Li XM, Li XW. Clinicopathological study of mixed cryoglobulinemic glomerulonephritis secondary to hepatitis B virus infection. BMC Nephrol 2020; 21:395. [PMID: 32928133 PMCID: PMC7490876 DOI: 10.1186/s12882-020-02057-4] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/08/2020] [Accepted: 09/06/2020] [Indexed: 01/04/2023] Open
Abstract
BACKGROUND Cryoglobulinemic glomerulonephritis (CryoGn) caused by hepatitis B virus (HBV) infection was rarely reported. Our study aimed to investigate the clinical features, renal pathology findings, and prognosis in patients with HBV related CryoGn. METHODS This was a retrospective study including seven Chinese patients with HBV related CryoGn in a tertiary referral hospital from April 2016 to March 2019. The clinical and pathological data were collected and analyzed. RESULTS Age at renal biopsy was 47 ± 12 years, with female/male ratio 3/4. Urine protein was 5.6 (3.0, 6.6) g/d and five cases presented with nephrotic syndrome. The baseline eGFR was 23.5 (20.2, 46.3) ml/min per 1.73m2. The extrarenal manifestations included purpura (n = 6), arthralgia (n = 1), peripheral neuropathy (n = 1), and cardiomyopathy (n = 1). Six cases had type II cryoglobulinemia with IgMκ, the other one had type III. The median cryocrit was 4.0 (1.0, 15.0) %. Renal pathologic findings on light microscopy: endocapillary proliferative glomerulonephritis (Gn) (n = 3), membranoproliferative Gn (n = 3), and mesangial proliferative Gn (n = 1). On immunofluorescence microscopy, the predominant type of immunoglobulin deposits was IgM (n = 5). HBsAg and HBcAg deposits were found in one case. Ultrastructural studies showed granular subendothelial and mesangial electron-dense deposits in all patients and microtubules in one case. All patients received antiviral medications. They were given corticosteroid alone (n = 2) or combined with cyclophosphamide (n = 4) or mycophenolate mofetil (n = 1). Two patients received plasmapheresis. The median follow-up time was 18 (6, 37) months. Four patients got remission, two patients died of pneumonia, and one progressed to end-stage renal disease (ESRD). At endpoint of follow-up, 24hUP was 2.1 (0.8-5.2) g/d, and eGFR was 55.3 (20.7, 111.8) ml/min per 1.73m2. The median cryocrit decreased to 1.0 (0, 5.75) %. CONCLUSIONS The etiology of mixed CryoGn should be screened for HBV infection. Endocapillary proliferative Gn and membranoproliferative Gn were the common pathologic patterns. Diagnosis and treatment in early stage benefit patients' renal outcomes. Immunosuppressive therapy should be considered for severe renal disease, based on efficient antiviral therapy.
Collapse
Affiliation(s)
- Chao Li
- Nephrology Department, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences & Peking Union Medical College, Beijing, 100730, China
| | - Hang Li
- Nephrology Department, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences & Peking Union Medical College, Beijing, 100730, China.
| | - Wei Su
- Laboratory Department, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences & Peking Union Medical College, Beijing, 100730, China.
| | - Yu-Bing Wen
- Nephrology Department, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences & Peking Union Medical College, Beijing, 100730, China
| | - Wei Ye
- Nephrology Department, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences & Peking Union Medical College, Beijing, 100730, China
| | - Wen-Ling Ye
- Nephrology Department, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences & Peking Union Medical College, Beijing, 100730, China
| | - Jian-Fang Cai
- Nephrology Department, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences & Peking Union Medical College, Beijing, 100730, China
| | - Xu-Zhen Qin
- Laboratory Department, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences & Peking Union Medical College, Beijing, 100730, China
| | - Xue-Mei Li
- Nephrology Department, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences & Peking Union Medical College, Beijing, 100730, China
| | - Xue-Wang Li
- Nephrology Department, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences & Peking Union Medical College, Beijing, 100730, China
| |
Collapse
|
|
36
|
Abstract
PURPOSE OF REVIEW For over 30 years, the American Society for Apheresis (ASFA) has published practice guidelines on the use of therapeutic apheresis in the Journal of Clinical Apheresis (JCA) Special Issue. These guidelines are periodically reviewed with the addition of new indications, retirement of some former indications and the provision of updated recommendations for current indications based on new published literature. During the last 12 years, updated guidelines have been published every 3 years to provide a reflection of current evidence-based apheresis practice. Recently, the eighth special issue was published. Significant updates and changes to the last edition are discussed in this review. RECENT FINDINGS This review provides a description of the development of the eighth special issue with the evolution of the ASFA guidelines since introduced in 1986. There are no new indications for therapeutic apheresis listed in the 2019 edition, however, several recommended category changes to existing indications have been made. There are also several organizational changes with the renaming of some fact sheets. SUMMARY ASFA has recently published its latest guidelines for therapeutic apheresis in the Journal of Clinical Apheresis 'Eighth Special Edition'. This review describes the evolution of the ASFA guidelines and highlights significant changes to the prior edition.
Collapse
|
|
37
|
Szczepiorkowski ZM. Indications for therapeutic apheresis in hematological disorders. Semin Hematol 2020; 57:57-64. [PMID: 32892844 DOI: 10.1053/j.seminhematol.2020.07.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
The early apheresis devices were developed in 1930s, but therapeutic apheresis only became widely used decades later, when automated cell separators were introduced. Progress in technical development of these devices continues to this day. Initial use of therapeutic apheresis has not been evidence based. Documents such as the Guidelines by the American Society for Apheresis provided hematologist with better tools to assess the role of therapeutic apheresis in daily practice. This review focuses on the use of therapeutic apheresis in patients with hematological disorders. Four separate apheresis modalities most encountered by hematologists are discussed: therapeutic plasma exchange, therapeutic leukocytapheresis, red blood cell exchange, and extracorporeal photopheresis. Examples of indications are provided and discussed. The future of therapeutic apheresis and its role in different diseases is undergoing continuous re-evaluation as disease pathogenesis is better understood and new treatment options become available.
Collapse
Affiliation(s)
- Zbigniew M Szczepiorkowski
- Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH, USA; Geisel School of Medicine at Dartmouth, Hanover, NH, USA; Institute of Hematology and Transfusion Medicine, Warsaw, Poland.
| |
Collapse
|
|
38
|
Xing Y, Wang S, Liu C, Wang H, Zhao M, Jin B, Kong X. Efficacy and safety of dual filtration plasmapheresis combined with biological agents in active refractory rheumatod arthritis: A retrospective cohort study. Medicine (Baltimore) 2020; 99:e20966. [PMID: 32664100 PMCID: PMC7360202 DOI: 10.1097/md.0000000000020966] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/04/2023] Open
Abstract
To investigate the effectiveness of dual filtration plasmapheresis (DFPP), a novel blood purification treatment, as a rapid and sustained disease-modifying therapy for active refractory rheumatoid arthritis (RA).A retrospective cohort study had been conducted. One hundred fifty three patients aged 18 years or older with active refractory RA were treated with DFPP combined with infliximab (IFX), IFX, or glucocorticoid (GC), all the above treatments were combined with methotrexate (MTX).Baseline characteristic of the 153 patients (DFPP: n = 53; IFX: n = 51; GC: n = 49) were similar across groups. The remission rate of CDAI (SDAI) in the DFPP treatment group was significantly higher than that of the IFX and GC group after 3 months of treatment. The remission rate of DFPP treatment group was above 50%, while in IFX and GC group, the rate of CDAI (SDAI) remission was 41.2% (37.3%) and 22.4% (14.2%) after 3 months of treatment.A combination of DFPP and biological agents can quickly induce remission or low disease activity of active refractory RA.
Collapse
Affiliation(s)
- Yida Xing
- Department of Rheumatology, the Second Affiliated Hospital of Dalian Medical University
| | - Shouquan Wang
- Department of Neurosurgery, Dalian Municipal Central Hospital of Dalian Medical University, Dalian, Liaoning
| | - Changyan Liu
- Department of Rheumatology, the Second Affiliated Hospital of Dalian Medical University
| | - Hongjiang Wang
- Department of Rheumatology, the Second Affiliated Hospital of Dalian Medical University
| | - Mingli Zhao
- Department of Rheumatology, the Second Affiliated Hospital of Dalian Medical University
| | - Bo Jin
- College of innovation and Entrepreneurship, Dalian University of Technology, China
| | - Xiaodan Kong
- Department of Rheumatology, the Second Affiliated Hospital of Dalian Medical University
| |
Collapse
|
|
39
|
Nagarajan VD, Morales A, Pleasant L, Shenoi A. Sepsis and thyroid storm in a patient with methimazole-induced agranulocytosis. BMJ Case Rep 2020; 13:13/7/e235536. [PMID: 32636230 DOI: 10.1136/bcr-2020-235536] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2023] Open
Abstract
Paediatric hyperthyroidism cases are mostly caused by Grave's disease. Thyroid storm is a life-threatening condition seen rarely, in severe thyrotoxicosis, occurring in about 1%-2% of patients with hyperthyroidism. Antithyroid medications and beta-blockers are typically the first-line management of thyroid storm. We report a challenging case of a 15-year-old girl who presented with thyroid storm in the setting of septic shock and methimazole-induced agranulocytosis. Since the first-line agents were contraindicated, plasmapheresis was used to control the thyroid storm and as a bridging therapy to the definitive therapy of early thyroidectomy. This is the first paediatric case report that outlines the use of plasmapheresis in the management of complicated thyrotoxicosis in a setting of septic shock.
Collapse
Affiliation(s)
| | - Alba Morales
- Department of Pediatrics, University of Kentucky, Lexington, Kentucky, USA
| | - Lawtanya Pleasant
- Department of Pediatrics, University of Kentucky, Lexington, Kentucky, USA
| | - Asha Shenoi
- Department of Pediatrics, University of Kentucky, Lexington, Kentucky, USA
| |
Collapse
|
|
40
|
Li A, Pavenski K, Kuo KHM, Patriquin CJ. Apheresis education in Canadian residency programs: A needs assessment. Transfus Apher Sci 2020; 59:102780. [PMID: 32505439 DOI: 10.1016/j.transci.2020.102780] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2019] [Revised: 03/31/2020] [Accepted: 03/31/2020] [Indexed: 10/24/2022]
Abstract
Indications for therapeutic and donor apheresis continue to increase and expand into new domains of therapy. The level and amount of apheresis education in residency programs remains heterogeneous, which may translate into varying degrees of clinical confidence in providing care. The purpose of this study was to assess Canadian clinicians' perceptions of their apheresis training in order to help demonstrate a need for a concrete apheresis education in residency curricula. A 22-question survey was distributed to Canadian graduates who recently completed training (2013-2017) in the following specialties: hematology, nephrology, transfusion medicine, and hematologic pathology. Questions regarding clinician perception of their training were asked using a Likert scale. Fifty-seven survey responses (32% response rate) were obtained from recent graduates from hematology (29/57, 51%), nephrology (21/57, 37%), hematologic pathology (4/57, 7%) and transfusion medicine (3/57, 5%). Although most respondents (68%) received some form of apheresis exposure during residency, only 23% reported a formal apheresis rotation. Only 40% felt that the amount of time devoted to apheresis education was sufficient, and only four respondents (7%) felt confident providing independent apheresis care at the end of training. Overall, these findings suggest that a common, dedicated apheresis curriculum in these training programs could possibly increase knowledge and competence of trainees, and provide a more solid foundation in apheresis for future practice.
Collapse
Affiliation(s)
- Amanda Li
- Department of Medicine, University of Toronto, Toronto, Ontario, Canada
| | - Katerina Pavenski
- Department of Laboratory Medicine, St. Michael's Hospital, Toronto, Ontario, Canada
| | - Kevin H M Kuo
- Division of Medical Oncology and Hematology, University Health Network, Toronto, Ontario, Canada
| | - Christopher J Patriquin
- Division of Medical Oncology and Hematology, University Health Network, Toronto, Ontario, Canada.
| |
Collapse
|
|
41
|
Deng J, Zhou F, Wong CY, Huang E, Zheng E. Efficacy of therapeutic plasma exchange for treatment of autoimmune hemolytic anemia: A systematic review and meta‐analysis of randomized controlled trials. J Clin Apher 2020; 35:294-306. [DOI: 10.1002/jca.21790] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/29/2020] [Revised: 04/24/2020] [Accepted: 04/25/2020] [Indexed: 12/27/2022]
Affiliation(s)
- Jiawen Deng
- Faculty of Health Sciences McMaster University Hamilton Ontario Canada
| | - Fangwen Zhou
- Faculty of Health Sciences McMaster University Hamilton Ontario Canada
| | - Chi Yi Wong
- Faculty of Health Sciences McMaster University Hamilton Ontario Canada
| | - Emma Huang
- Faculty of Health Sciences McMaster University Hamilton Ontario Canada
| | - Elena Zheng
- Faculty of Applied Health Sciences University of Waterloo Waterloo Ontario Canada
| |
Collapse
|
|
42
|
Berentsen S. New Insights in the Pathogenesis and Therapy of Cold Agglutinin-Mediated Autoimmune Hemolytic Anemia. Front Immunol 2020; 11:590. [PMID: 32318071 PMCID: PMC7154122 DOI: 10.3389/fimmu.2020.00590] [Citation(s) in RCA: 85] [Impact Index Per Article: 17.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2020] [Accepted: 03/13/2020] [Indexed: 12/12/2022] Open
Abstract
Autoimmune hemolytic anemias mediated by cold agglutinins can be divided into cold agglutinin disease (CAD), which is a well-defined clinicopathologic entity and a clonal lymphoproliferative disorder, and secondary cold agglutinin syndrome (CAS), in which a similar picture of cold-hemolytic anemia occurs secondary to another distinct clinical disease. Thus, the pathogenesis in CAD is quite different from that of polyclonal autoimmune diseases such as warm-antibody AIHA. In both CAD and CAS, hemolysis is mediated by the classical complement pathway and therefore can result in generation of anaphylotoxins, such as complement split product 3a (C3a) and, to some extent, C5a. On the other hand, infection and inflammation can act as triggers and drivers of hemolysis, exemplified by exacerbation of CAD in situations with acute phase reaction and the role of specific infections (particularly Mycoplasma pneumoniae and Epstein-Barr virus) as causes of CAS. In this review, the putative mechanisms behind these phenomena will be explained along with other recent achievements in the understanding of pathogenesis in these disorders. Therapeutic approaches have been directed against the clonal lymphoproliferation in CAD or the underlying disease in CAS. Currently, novel targeted treatments, in particular complement-directed therapies, are also being rapidly developed and will be reviewed.
Collapse
Affiliation(s)
- Sigbjørn Berentsen
- Department of Research and Innovation, Haugesund Hospital, Haugesund, Norway
| |
Collapse
|
|
43
|
Li F, Chen AB, Duan YC, Liao R, Xu YW, Tao LL. Multiple organ dysfunction and rhabdomyolysis associated with moonwort poisoning: Report of four cases. World J Clin Cases 2020; 8:479-486. [PMID: 32047801 PMCID: PMC7000952 DOI: 10.12998/wjcc.v8.i2.479] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/30/2019] [Revised: 12/13/2019] [Accepted: 12/22/2019] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Moonwort is a widely used Chinese herbal medicine. It has various pharmacological effects, such as relieving cough and preventing asthma. To date, multiple organ dysfunction and rhabdomyolysis caused by moonwort poisoning have not been reported.
CASE SUMMARY Here we report four cases of moonwort poisoning that presented with multiple organ dysfunction and rhabdomyolysis accompanied by vomiting, fatigue, and muscle aches. One patient was an adult male, two were adult females, and one was a boy, with an age range of 7–64 years. The adults were treated with hemoperfusion and symptomatic therapies, while the child was treated with plasma exchange and symptomatic therapies. All four patients recovered.
CONCLUSION Blood purification combined with symptomatic treatment may be an effective method for managing multiple organ dysfunction and rhabdomyolysis caused by acute moonwort poisoning.
Collapse
Affiliation(s)
- Fang Li
- Department of Emergency Medicine, The Second Affiliated Hospital of Kunming Medical University, Kunming 650101, Yunnan Province, China
| | - An-Bao Chen
- Department of Emergency Medicine, The Second Affiliated Hospital of Kunming Medical University, Kunming 650101, Yunnan Province, China
| | - Yong-Chun Duan
- Department of Emergency Medicine, The Second Affiliated Hospital of Kunming Medical University, Kunming 650101, Yunnan Province, China
| | - Rui Liao
- Department of Emergency Medicine, The Second Affiliated Hospital of Kunming Medical University, Kunming 650101, Yunnan Province, China
| | - Yu-Wei Xu
- Department of Emergency Medicine, The Second Affiliated Hospital of Kunming Medical University, Kunming 650101, Yunnan Province, China
| | - Li-Li Tao
- Department of Emergency Medicine, The Second Affiliated Hospital of Kunming Medical University, Kunming 650101, Yunnan Province, China
| |
Collapse
|
|
44
|
Sampley S, Sakhuja V, Bhasin D, Singh K, Singh H. Plasmapheresis for Pulmonary Hemorrhage Following Viperine Snakebite: A Case Report with Review of Literature. Indian J Crit Care Med 2020; 24:986-990. [PMID: 33281328 PMCID: PMC7689129 DOI: 10.5005/jp-journals-10071-23635] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2023] Open
Abstract
Introduction Snakebites are one of the commonest occupational hazards in tropical countries and viperine bites are potential to cause systemic toxicity. Coagulopathies and acute kidney injury (AKI) have been documented and easily dealt with in past, but pulmonary hemorrhage has been rarely seen and plasmapheresis has shown promising result for the same. This case reports highlight the effective use of plasmapheresis for pulmonary hemorrhage post-snakebite. Background Viperine snakebite has been associated with high morbidity and mortality due to its toxic systemic envenomization. The important systemic manifestations are coagulopathy, neuromuscular paralysis, AKI, myotoxicity, and cardiovascular collapse. Antivenomization, renal replacement therapy, steroids, and other supportive care are considered to be the mainstay of treatment till date. Pulmonary hemorrhage has been an unusual manifestation of viper bite and rarely reported and steroids have been used in such scenario but with mixed results. Role of plasmapheresis has been documented in the management of snakebite but especially for hematological problems and in limb preservation/salvage strategies. The use of same, for pulmonary hemorrhage has not been studied yet. Here, we present a rare case of pulmonary hemorrhage along with renal failure following viper bite which was successfully treated with plasmapheresis. To the best of our knowledge, it is a rare presentation and has not been reported in the literature reviewed till date. Case description A previously healthy, 36-year-old man presented to our hospital 48 hours after a viper bite. He developed local as well systemic manifestations evident as hemolysis and renal failure. Gradually, he started having hemoptysis followed by respiratory failure requiring ventilatory support. CT chest done was s/o bilateral pulmonary hemorrhages correlating clinically with ongoing tracheal bleed. He had no other bleeding manifestations and had normal coagulation profile. He was initially treated with methylprednisolone therapy, but then did not show any improvement and finally plasmapheresis was done as rescue therapy. Following this, he had improvement in respiratory parameters and settling tracheal bleed with resolution of radiological changes. He was successfully weaned off from the ventilation and also his renal failure also improved with near normalization of pulmonary and renal functions. Conclusion This case highlights the unusual presentation of pulmonary hemorrhage in a patient with viperine bite with normal coagulation and was aggressively managed with plasmapheresis. Hence, plasmapheresis can be used as life-saving modality in patients with systemic envenomization post-viperine bit. How to cite this article Sampley S, Sakhuja V, Bhasin D, Singh K, Singh H. Plasmapheresis for Pulmonary Hemorrhage Following Viperine Snakebite: A Case Report with Review of Literature. Indian J Crit Care Med 2020;24(10):986-990.
Collapse
Affiliation(s)
- Supriya Sampley
- Department of Pulmonology and Critical Care, Medical Intensive Care Unit, Max Super Speciality Hospital, Mohali, Punjab, India
| | - Vinay Sakhuja
- Department of Nephrology, Max Super Speciality Hospital, Mohali, Punjab, India
| | - Deepak Bhasin
- Department of Pulmonology and Critical Care, Max Super Speciality Hospital, Mohali, Punjab, India
| | - Kuldeep Singh
- Department of Transfusion Medicine, Max Super Speciality Hospital, Mohali, Punjab, India
| | - Harpal Singh
- Department of Pulmonology and Critical Care, Max Super Speciality Hospital, Mohali, Punjab, India
| |
Collapse
|
|
45
|
Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting. Blood Rev 2019; 41:100648. [PMID: 31839434 DOI: 10.1016/j.blre.2019.100648] [Citation(s) in RCA: 294] [Impact Index Per Article: 49.0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2019] [Revised: 11/21/2019] [Accepted: 11/25/2019] [Indexed: 12/15/2022]
Abstract
Autoimmune hemolytic anemias (AIHAs) are rare and heterogeneous disorders characterized by the destruction of red blood cells through warm or cold antibodies. There is currently no licensed treatment for AIHA. Due to the paucity of clinical trials, recommendations on diagnosis and therapy have often been based on expert opinions and some national guidelines. Here we report the recommendations of the First International Consensus Group, who met with the aim to review currently available data and to provide standardized diagnostic criteria and therapeutic approaches as well as an overview of novel therapies. Exact diagnostic workup is important because symptoms, course of disease, and therapeutic management relate to the type of antibody involved. Monospecific direct antiglobulin test is considered mandatory in the diagnostic workup, and any causes of secondary AIHA have to be diagnosed. Corticosteroids remain first-line therapy for warm-AIHA, while the addition of rituximab should be considered early in severe cases and if no prompt response to steroids is achieved. Rituximab with or without bendamustine should be used in the first line for patients with cold agglutinin disease requiring therapy. We identified a need to establish an international AIHA network. Future recommendations should be based on prospective clinical trials whenever possible.
Collapse
|
|
46
|
Mansouri Taleghani B, Buser A. Therapeutic Apheresis. Transfus Med Hemother 2019; 46:391-393. [PMID: 31933568 PMCID: PMC6944895 DOI: 10.1159/000504143] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/15/2019] [Accepted: 10/15/2019] [Indexed: 01/04/2023] Open
Affiliation(s)
- Behrouz Mansouri Taleghani
- University Clinic of Hematology and Central Hematology Laboratory, Division of Transfusion Medicine, Bern University Hospital, Inselspital, Bern, Switzerland
| | - Andreas Buser
- Regional Blood Transfusion Service Swiss Red Cross Basel, and Department of Hematology, University Hospital Basel, Basel, Switzerland
| |
Collapse
|
|
47
|
Joury A, Alshehri M, Mahendra A, Anteet M, Yousef MA, Khan AM. Therapeutic approaches in hypertriglyceridemia-induced acute pancreatitis: A literature review of available therapies and case series. J Clin Apher 2019; 35:131-137. [PMID: 31724761 DOI: 10.1002/jca.21763] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/03/2019] [Revised: 10/10/2019] [Accepted: 11/01/2019] [Indexed: 12/11/2022]
Abstract
Hypertriglyceridemia-induced acute pancreatitis (HGAP) is the third most common etiology of acute pancreatitis. HGAP can be attributed to genetic disturbances in triglyceride metabolism or multiple secondary causes. Here, we presented three cases for HGAP and explored different therapeutic approaches for treating HGAP. A case series of three patients who presented with HGAP and underwent different therapeutic approaches was conducted. The first patient was a 37-year-old male who presented with nonsevere HGAP; he was treated with conservative therapy with insulin and heparin infusion, which resulted in clinical and laboratory improvement. The second patient was a 64-year-old male with human immunodeficiency virus on multiple highly active antiretroviral therapy. He presented with severe HGAP and multiorgan failure. After initiation of therapeutic plasma exchange, his HGAP resolved. The third patient was a 28-year-old male who presented with recurrent episodes of HGAP; his conservative therapy failed and was eventually escalated to therapeutic plasma exchange (TPE). HGAP can be attributed to genetic disturbances of lipid or secondary etiologies. A nonsevere form of HGAP can be managed with conventional therapy including insulin and heparin; however, severe HGAP may require TPE.
Collapse
Affiliation(s)
- Abdulaziz Joury
- Department of Internal Medicine, Ochsner Clinic Foundation, New Orleans, Louisiana.,King Salman Heart Center, King Fahad Medical City, Riyadh, Saudi Arabia
| | - Mona Alshehri
- Department of Internal Medicine, Ochsner Clinic Foundation, New Orleans, Louisiana
| | - Arjun Mahendra
- Department of Internal Medicine, Ochsner Clinic Foundation, New Orleans, Louisiana
| | - Mahmoud Anteet
- Department of Radiology, Ochsner Clinic Foundation, New Orleans, Louisiana
| | - Mohammad A Yousef
- Department of Internal Medicine, Ochsner Clinic Foundation, New Orleans, Louisiana
| | - Abdul M Khan
- Department of Pulmonary and Critical Care, Ochsner Clinic Foundation, New Orleans, Louisiana
| |
Collapse
|
|
48
|
Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, Pham HP, Schneiderman J, Witt V, Wu Y, Zantek ND, Dunbar NM, Schwartz GEJ. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice - Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher 2019; 34:171-354. [PMID: 31180581 DOI: 10.1002/jca.21705] [Citation(s) in RCA: 864] [Impact Index Per Article: 144.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating and categorizing indications for the evidence-based use of therapeutic apheresis (TA) in human disease. Since the 2007 JCA Special Issue (Fourth Edition), the committee has incorporated systematic review and evidence-based approaches in the grading and categorization of apheresis indications. This Eighth Edition of the JCA Special Issue continues to maintain this methodology and rigor in order to make recommendations on the use of apheresis in a wide variety of diseases/conditions. The JCA Eighth Edition, like its predecessor, continues to apply the category and grading system definitions in fact sheets. The general layout and concept of a fact sheet that was introduced in the Fourth Edition, has largely been maintained in this edition. Each fact sheet succinctly summarizes the evidence for the use of TA in a specific disease entity or medical condition. The Eighth Edition comprises 84 fact sheets for relevant diseases and medical conditions, with 157 graded and categorized indications and/or TA modalities. The Eighth Edition of the JCA Special Issue seeks to continue to serve as a key resource that guides the utilization of TA in the treatment of human disease.
Collapse
Affiliation(s)
- Anand Padmanabhan
- Medical Sciences Institute & Blood Research Institute, Versiti & Department of Pathology, Medical College of Wisconsin, Milwaukee, Wisconsin
| | - Laura Connelly-Smith
- Department of Medicine, Seattle Cancer Care Alliance & University of Washington, Seattle, Washington
| | - Nicole Aqui
- Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Rasheed A Balogun
- Department of Medicine, University of Virginia, Charlottesville, Virginia
| | - Reinhard Klingel
- Apheresis Research Institute, Cologne, Germany & First Department of Internal Medicine, University of Mainz, Mainz, Germany
| | - Erin Meyer
- Department of Hematology/Oncology/BMT/Pathology, Nationwide Children's Hospital, Columbus, Ohio
| | - Huy P Pham
- Department of Pathology, Keck School of Medicine of the University of Southern California, Los Angeles, California
| | - Jennifer Schneiderman
- Department of Pediatric Hematology/Oncology/Neuro-oncology/Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University, Chicago, Illinois
| | - Volker Witt
- Department for Pediatrics, St. Anna Kinderspital, Medical University of Vienna, Vienna, Austria
| | - Yanyun Wu
- Bloodworks NW & Department of Laboratory Medicine, University of Washington, Seattle, Washington, Yale University School of Medicine, New Haven, Connecticut
| | - Nicole D Zantek
- Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota
| | - Nancy M Dunbar
- Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire
| | | |
Collapse
|
|
49
|
Rolfes L, Pfeuffer S, Ruck T, Melzer N, Pawlitzki M, Heming M, Brand M, Wiendl H, Meuth SG. Therapeutic Apheresis in Acute Relapsing Multiple Sclerosis: Current Evidence and Unmet Needs-A Systematic Review. J Clin Med 2019; 8:jcm8101623. [PMID: 31590282 PMCID: PMC6832170 DOI: 10.3390/jcm8101623] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/16/2019] [Revised: 10/01/2019] [Accepted: 10/02/2019] [Indexed: 12/13/2022] Open
Abstract
Multiple sclerosis (MS) is the most abundant inflammatory demyelinating disorder of the central nervous system. Despite recent advances in its long-term immunomodulatory treatment, MS patients still suffer from relapses, significantly contributing to disability accrual. In recent years, apheresis procedures such as therapeutic plasma exchange (TPE) and immunoadsorption (IA) have been recognized as two options for treating MS relapses, that do not respond to standard treatment with corticosteroids. TPE is already incorporated in most international guidelines, although evidence for its use resulted mostly from either case series or small unblinded and/or non-randomized trials. Data on IA are still sparse, but several studies indicate comparable efficacy between both apheresis procedures. This article gives an overview of the published evidence on TPE and IA in the treatment of acute relapses in MS. Further, we outline current evidence regarding individual outcome predictors, describe technical details of apheresis procedures, and discuss apheresis treatment in children and during pregnancy.
Collapse
Affiliation(s)
- Leoni Rolfes
- Department of Neurology with Institute of Translational Neurology, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany.
| | - Steffen Pfeuffer
- Department of Neurology with Institute of Translational Neurology, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany.
| | - Tobias Ruck
- Department of Neurology with Institute of Translational Neurology, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany.
| | - Nico Melzer
- Department of Neurology with Institute of Translational Neurology, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany.
| | - Marc Pawlitzki
- Department of Neurology with Institute of Translational Neurology, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany.
| | - Michael Heming
- Department of Neurology with Institute of Translational Neurology, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany.
| | - Marcus Brand
- Department of Internal Medicine D, University Hospital Münster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany.
| | - Heinz Wiendl
- Department of Neurology with Institute of Translational Neurology, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany.
| | - Sven G Meuth
- Department of Neurology with Institute of Translational Neurology, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany.
| |
Collapse
|
|
50
|
Zhang Y, He W, He C, Wan J, Lin X, Zheng X, Li L, Li X, Yang X, Yu B, Xian X, Zhu Y, Wang Y, Liu G, Lu N. Large triglyceride-rich lipoproteins in hypertriglyceridemia are associated with the severity of acute pancreatitis in experimental mice. Cell Death Dis 2019; 10:728. [PMID: 31570698 PMCID: PMC6768872 DOI: 10.1038/s41419-019-1969-3] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/14/2019] [Revised: 08/20/2019] [Accepted: 08/26/2019] [Indexed: 02/07/2023]
Abstract
Hypertriglyceridemia severity is linked to acute pancreatitis prognosis, but it remains unknown why a portion of severe hypertriglyceridemia patients do not develop severe acute pancreatitis. To investigate whether hypertriglyceridemia subtypes affect acute pancreatitis progression, we analyzed two genetically modified hypertriglyceridemia mouse models—namely, glycosylphosphatidylinositol high-density lipoprotein binding protein 1 knockout (Gpihbp1−/−) and apolipoprotein C3 transgenic (ApoC3-tg) mice. Acute pancreatitis was induced by 10 intraperitoneal caerulein injections. Biochemical assays and pathological analysis were performed for the severity evaluation of acute pancreatitis. Plasma triglyceride-rich lipoproteins (TRLs), including chylomicrons and very low-density lipoprotein (VLDL), were collected via ultracentrifugation to evaluate their cytotoxic effects on primary pancreatic acinar cells (PACs). We found that the particle sizes of Gpihbp1−/− TRLs were larger than ApoC3-tg TRLs. Severe pancreatic injury with large areas of pancreatic necrosis in the entire lobule was induced in Gpihbp1−/− mice when plasma triglyceride levels were greater than 2000 mg/dL. However, ApoC3-tg mice with the same triglyceride levels did not develop large areas of pancreatic necrosis, even upon the administration of poloxamer 407 to further increase triglyceride levels. Meanwhile, in the acute pancreatitis model, free fatty acids (FFAs) in the pancreas of Gpihbp1−/− mice were greater than in ApoC3-tg mice. TRLs from Gpihbp1−/− mice released more FFAs and were more toxic to PACs than those from ApoC3-tg mice. Chylomicrons from patients showed the same effects on PACs as TRLs from Gpihbp1−/− mice. Gpihbp1−/− mice with triglyceride levels below 2000 mg/dL had milder pancreatic injury and less incidence of pancreatic necrosis than those with triglyceride levels above 2000 mg/dL, similar to Gpihbp1−/−mice with triglyceride levels above 2000 mg/dL but with fenofibrate administration. These findings demonstrated that hypertriglyceridemia subtypes with large TRL particles could affect acute pancreatitis progression and that chylomicrons showed more cytotoxicity than VLDL by releasing more FFAs.
Collapse
Affiliation(s)
- Yue Zhang
- Department of Gastroenterology, The First Affiliated Hospital of Nanchang University, 330006, Nanchang, China
| | - Wenhua He
- Department of Gastroenterology, The First Affiliated Hospital of Nanchang University, 330006, Nanchang, China
| | - Cong He
- Department of Gastroenterology, The First Affiliated Hospital of Nanchang University, 330006, Nanchang, China
| | - Jianhua Wan
- Department of Gastroenterology, The First Affiliated Hospital of Nanchang University, 330006, Nanchang, China
| | - Xiao Lin
- Institute of Cardiovascular Sciences, Peking University Health Science Center, 100191, Beijing, China.,Key Laboratory of Molecular Cardiovascular Science, Ministry of Education, 100191, Beijing, China
| | - Xi Zheng
- Department of Gastroenterology, The First Affiliated Hospital of Nanchang University, 330006, Nanchang, China
| | - Lei Li
- Department of Gastroenterology, The First Affiliated Hospital of Nanchang University, 330006, Nanchang, China
| | - Xueyang Li
- Department of Gastroenterology, The First Affiliated Hospital of Nanchang University, 330006, Nanchang, China
| | - Xiaoyu Yang
- Department of Gastroenterology, The First Affiliated Hospital of Nanchang University, 330006, Nanchang, China
| | - Bingjun Yu
- Department of Gastroenterology, The First Affiliated Hospital of Nanchang University, 330006, Nanchang, China
| | - Xunde Xian
- Institute of Cardiovascular Sciences, Peking University Health Science Center, 100191, Beijing, China.,Key Laboratory of Molecular Cardiovascular Science, Ministry of Education, 100191, Beijing, China
| | - Yin Zhu
- Department of Gastroenterology, The First Affiliated Hospital of Nanchang University, 330006, Nanchang, China
| | - Yuhui Wang
- Institute of Cardiovascular Sciences, Peking University Health Science Center, 100191, Beijing, China. .,Key Laboratory of Molecular Cardiovascular Science, Ministry of Education, 100191, Beijing, China.
| | - George Liu
- Institute of Cardiovascular Sciences, Peking University Health Science Center, 100191, Beijing, China.,Key Laboratory of Molecular Cardiovascular Science, Ministry of Education, 100191, Beijing, China
| | - Nonghua Lu
- Department of Gastroenterology, The First Affiliated Hospital of Nanchang University, 330006, Nanchang, China.
| |
Collapse
|