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Colbert GB, Elrggal ME, Gaur L, Lerma EV. Update and review of adult polycystic kidney disease. Dis Mon 2020; 66:100887. [DOI: 10.1016/j.disamonth.2019.100887] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
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Harris T, Sandford R. European ADPKD Forum multidisciplinary position statement on autosomal dominant polycystic kidney disease care: European ADPKD Forum and Multispecialist Roundtable participants. Nephrol Dial Transplant 2019; 33:563-573. [PMID: 29309655 PMCID: PMC6018982 DOI: 10.1093/ndt/gfx327] [Citation(s) in RCA: 26] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/10/2017] [Indexed: 02/02/2023] Open
Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is a chronic, progressive condition characterized by the development and growth of cysts in the kidneys and other organs and by additional systemic manifestations. Individuals with ADPKD should have access to lifelong, multidisciplinary, specialist and patient-centred care involving: (i) a holistic and comprehensive assessment of the manifestations, complications, prognosis and impact of the disease (in physical, psychological and social terms) on the patient and their family; (ii) access to treatment to relieve symptoms, manage complications, preserve kidney function, lower the risk of cardiovascular disease and maintain quality of life; and (iii) information and support to help patients and their families act as fully informed and active partners in care, i.e. to maintain self-management approaches, deal with the impact of the condition and participate in decision-making regarding healthcare policies, services and research. Building on discussions at an international roundtable of specialists and patient advocates involved in ADPKD care, this article sets out (i) the principles for a patient-centred, holistic approach to the organization and delivery of ADPKD care in practice, with a focus on multispecialist collaboration and shared-decision making, and (ii) the rationale and knowledge base for a route map for ADPKD care intended to help patients navigate the services available to them and to help stakeholders and decision-makers take practical steps to ensure that all patients with ADPKD can access the comprehensive multispecialist care to which they are entitled. Further multispecialty collaboration is encouraged to design and implement these services, and to work with patient organizations to promote awareness building, education and research.
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Affiliation(s)
| | | | - Richard Sandford
- Academic Department of Medical Genetics, University of Cambridge School of Clinical Medicine, Cambridge, UK
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Subarachnoid Hemorrhage in Hospitalized Renal Transplant Recipients with Autosomal Dominant Polycystic Kidney Disease: A Nationwide Analysis. J Clin Med 2019; 8:jcm8040524. [PMID: 30999564 PMCID: PMC6517948 DOI: 10.3390/jcm8040524] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2019] [Revised: 04/12/2019] [Accepted: 04/15/2019] [Indexed: 12/21/2022] Open
Abstract
Background: This study aimed to evaluate the hospitalization rates for subarachnoid hemorrhage (SAH) among renal transplant patients with adult polycystic kidney disease (ADPKD) and its outcomes, when compared to non-ADPKD renal transplant patients. Methods: The 2005–2014 National Inpatient Sample databases were used to identify all hospitalized renal transplant patients. The inpatient prevalence of SAH as a discharge diagnosis between ADPKD and non-ADPKD renal transplant patients was compared. Among SAH patients, the in-hospital mortality, use of aneurysm clipping, hospital length of stay, total hospitalization cost and charges between ADPKD and non-ADPKD patients were compared, adjusting for potential confounders. Results: The inpatient prevalence of SAH in ADPKD was 3.8/1000 admissions, compared to 0.9/1000 admissions in non-ADPKD patients (p < 0.01). Of 833 renal transplant patients with a diagnosis of SAH, 30 had ADPKD. Five (17%) ADPKD renal patients with SAH died in hospitals compared to 188 (23.4%) non-ADPKD renal patients (p = 0.70). In adjusted analysis, there was no statistically significant difference in mortality, use of aneurysm clipping, hospital length of stay, or total hospitalization costs and charges between ADPKD and non-ADPKD patients with SAH. Conclusion: Renal transplant patients with ADPKD had a 4-fold higher inpatient prevalence of SAH than those without ADPKD. Further studies are needed to compare the incidence of overall admissions in ADPKD and non-ADPKD patients. When renal transplant patients developed SAH, inpatient mortality rates were high regardless of ADPKD status. The outcomes, as well as resource utilization, were comparable between the two groups.
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Jankowska M, Kuźmiuk-Glembin I, Skonieczny P, Dębska-Ślizień A. Native Nephrectomy in Renal Transplant Recipients With Autosomal Dominant Polycystic Kidney Disease. Transplant Proc 2018; 50:1863-1867. [DOI: 10.1016/j.transproceed.2018.02.100] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/28/2017] [Accepted: 02/06/2018] [Indexed: 12/26/2022]
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Clinical Outcome of Simultaneous Native Nephrectomy and Kidney Transplantation in Patients With Autosomal Dominant Polycystic Kidney Disease. Transplant Proc 2016; 48:840-3. [DOI: 10.1016/j.transproceed.2015.08.047] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/21/2015] [Accepted: 08/18/2015] [Indexed: 12/23/2022]
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Sáez ID, de la Llera JF, Tapia A, Chacón RA, Figueroa PA, Vivaldi BI, Domenech A, Horn CD, Coz F. Pre-transplant treatment of large polycystic kidney. World J Clin Urol 2016; 5:66-71. [DOI: 10.5410/wjcu.v5.i1.66] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/28/2015] [Revised: 12/11/2015] [Accepted: 01/19/2016] [Indexed: 02/06/2023] Open
Abstract
AIM: To evaluate the indications, optimal timing and outcomes of native nephrectomy and other techniques in pretransplant treatment of autosomal dominant polycystic kidney disease (PKD).
METHODS: A literature review was conducted using the PubMed and Epistemonikos databases. Keywords for pre-transplant surgical management of polycystic kidneys were: Transplant, treatment and PKD. Keywords for pre-treatment embolization of PKD were: Embolization, transplant and polycystic kidney disease. The inclusion criterions were all articles found using this search method. The exclusion criterions were articles found to include bias and not attending pre-transplant treatment options. Fifteen articles were included in our final analysis. Ten articles were found regarding embolization of PKD of which three reviews were selected for final analysis. The reviews were divided into pre transplant and intra transplant treatment for the surgical treatment of PKD. All articles meeting inclusion criteria were thoroughly analyzed by two independent reviewers. A third independent reviewer was consulted if the reviewers did not agree upon the inclusion or exclusion of a specific article. No statistical analysis was performed.
RESULTS: Studies vary regarding the technique used (open or laparoscopic), laterality (single or bilateral) and temporality of nephrectomy with respect to renal transplant (pre-transplant or simultaneous to transplant). Several groups argue in favor of simultaneous nephrectomy and kidney transplant since it avoids the deleterious effects of being anefric. Long-term results and patient satisfaction are acceptable. However, it is associated with increased operative time, transfusion rate, morbidity and length of hospital stay. Based on small sample studies, bilateral nephrectomy prior to transplant has been associated with a higher risk of morbidity and mortality. Studies on laparoscopic approach report it as a feasible and safe alternative to the open surgery approach, highlighting its lower complication rate, transfusions and shorter hospital stay. Arterial embolization of the kidney appears as an effective and low morbid alternative for the management of large native kidneys. The reduction in renal size allow transplant in a significant number of patients, which makes it an appealing alternative to surgery.
CONCLUSION: There is limited evidence regarding best pretrasnplant treatment of large PKD but to date embolization seems an appealing alternative to augment space for renal graft allocation.
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Lee VW, Tunnicliffe DJ, Rangan GK. KHA-CARI Autosomal Dominant Polycystic Kidney Disease Guideline: Management of End-Stage Kidney Disease. Semin Nephrol 2016; 35:595-602.e12. [PMID: 26718164 DOI: 10.1016/j.semnephrol.2015.10.011] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Affiliation(s)
- Vincent W Lee
- Department of Renal Medicine, Westmead Hospital, Western Sydney Local Health District, Sydney, Australia; Centre for Transplant and Renal Research, Westmead Institute for Medical Research, University of Sydney, Westmead, Sydney, Australia.
| | - David J Tunnicliffe
- KHA-CARI Guidelines, Centre for Kidney Research, The Children's Hospital at Westmead, Westmead, Sydney, Australia; Sydney School of Public Health, University of Sydney, Sydney, Australia
| | - Gopala K Rangan
- Department of Renal Medicine, Westmead Hospital, Western Sydney Local Health District, Sydney, Australia; Centre for Transplant and Renal Research, Westmead Millennium Institute for Medical Research, University of Sydney, Westmead, Sydney, Australia
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Akoh JA. Current management of autosomal dominant polycystic kidney disease. World J Nephrol 2015; 4:468-479. [PMID: 26380198 PMCID: PMC4561844 DOI: 10.5527/wjn.v4.i4.468] [Citation(s) in RCA: 33] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/01/2015] [Revised: 06/23/2015] [Accepted: 08/31/2015] [Indexed: 02/06/2023] Open
Abstract
Autosomal dominant polycystic kidney disease (ADPKD), the most frequent cause of genetic renal disease affecting approximately 4 to 7 million individuals worldwide and accounting for 7%-15% of patients on renal replacement therapy, is a systemic disorder mainly involving the kidney but cysts can also occur in other organs such as the liver, pancreas, arachnoid membrane and seminal vesicles. Though computed tomography and magnetic resonance imaging (MRI) were similar in evaluating 81% of cystic lesions of the kidney, MRI may depict septa, wall thickening or enhancement leading to upgrade in cyst classification that can affect management. A screening strategy for intracranial aneurysms would provide 1.0 additional year of life without neurological disability to a 20-year-old patient with ADPKD and reduce the financial impact on society of the disease. Current treatment strategies include reducing: cyclic adenosine monophosphate levels, cell proliferation and fluid secretion. Several randomised clinical trials (RCT) including mammalian target of rapamycin inhibitors, somatostatin analogues and a vasopressin V2 receptor antagonist have been performed to study the effect of diverse drugs on growth of renal and hepatic cysts, and on deterioration of renal function. Prophylactic native nephrectomy is indicated in patients with a history of cyst infection or recurrent haemorrhage or to those in whom space must be made to implant the graft. The absence of large RCT on various aspects of the disease and its treatment leaves considerable uncertainty and ambiguity in many aspects of ADPKD patient care as it relates to end stage renal disease (ESRD). The outlook of patients with ADPKD is improving and is in fact much better than that for patients in ESRD due to other causes. This review highlights the need for well-structured RCTs as a first step towards trying newer interventions so as to develop updated clinical management guidelines.
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Chapman AB, Devuyst O, Eckardt KU, Gansevoort RT, Harris T, Horie S, Kasiske BL, Odland D, Pei YP, Perrone RD, Pirson Y, Schrier RW, Torra R, Torres VE, Watnick T, Wheeler DC. Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int 2015; 88:17-27. [PMID: 25786098 PMCID: PMC4913350 DOI: 10.1038/ki.2015.59] [Citation(s) in RCA: 369] [Impact Index Per Article: 36.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2014] [Revised: 01/23/2015] [Accepted: 01/28/2015] [Indexed: 02/06/2023]
Abstract
Autosomal-dominant polycystic kidney disease (ADPKD) affects up to 12 million individuals and is the fourth most common cause for renal replacement therapy worldwide. There have been many recent advances in the understanding of its molecular genetics and biology, and in the diagnosis and management of its manifestations. Yet, diagnosis, evaluation, prevention, and treatment vary widely and there are no broadly accepted practice guidelines. Barriers to translation of basic science breakthroughs to clinical care exist, with considerable heterogeneity across countries. The Kidney Disease: Improving Global Outcomes Controversies Conference on ADPKD brought together a panel of multidisciplinary clinical expertise and engaged patients to identify areas of consensus, gaps in knowledge, and research and health-care priorities related to diagnosis; monitoring of kidney disease progression; management of hypertension, renal function decline and complications; end-stage renal disease; extrarenal complications; and practical integrated patient support. These are summarized in this review.
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Affiliation(s)
| | | | | | | | | | - Shigeo Horie
- Juntendo University Graduate School of Medicine, Bunkyou, Tokyo Japan
| | | | | | - York P. Pei
- University Health Network and University of Toronto, Toronto, Ontario, Canada
| | - Ronald D. Perrone
- Tufts Medical Center and Tufts University School of Medicine, Boston, Massachusetts, USA
| | - Yves Pirson
- Université Catholique de Louvain, Brussels, Belgium
| | | | - Roser Torra
- Fundació Puigvert, REDinREN, Universitat Autónoma de Barcelona, Barcelona, Spain
| | | | - Terry Watnick
- University of Maryland School of Medicine, Baltimore, Maryland, USA
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Kanaan N, Devuyst O, Pirson Y. Renal transplantation in autosomal dominant polycystic kidney disease. Nat Rev Nephrol 2014; 10:455-65. [PMID: 24935705 DOI: 10.1038/nrneph.2014.104] [Citation(s) in RCA: 54] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
In patients with autosomal dominant polycystic kidney disease (ADPKD) evaluated for kidney transplantation, issues related to native nephrectomy, cystic liver involvement, screening for intracranial aneurysms and living-related kidney donation deserve special consideration. Prophylactic native nephrectomy is restricted to patients with a history of cyst infection or recurrent haemorrhage or to those in whom space must be made to implant the graft. Patients with liver involvement require pretransplant imaging. Selection of patients for pretransplant screening of intracranial aneurysms should follow the general recommendations for patients with ADPKD. In living related-donor candidates aged <30 years and at-risk of ADPKD, molecular genetic testing should be carried out when ultrasonography and MRI findings are normal or equivocal. After kidney transplantation, patient and graft survival rates are excellent and the volume of native kidneys decreases. However, liver cysts continue to grow and treatment with a somatostatin analogue should be considered in patients with massive cyst involvement. Cerebrovascular events have a marginal effect on post-transplant morbidity and mortality. An increased risk of new-onset diabetes mellitus and nonmelanoma skin cancers has been reported, but several studies have challenged these findings. Finally, no data currently support the preferential use of mammalian target of rapamycin inhibitors as immunosuppressive agents in transplant recipients with ADPKD.
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Affiliation(s)
- Nada Kanaan
- Division of Nephrology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 10 Avenue Hippocrate, B-1200 Brussels, Belgium
| | - Olivier Devuyst
- Division of Nephrology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 10 Avenue Hippocrate, B-1200 Brussels, Belgium
| | - Yves Pirson
- Division of Nephrology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 10 Avenue Hippocrate, B-1200 Brussels, Belgium
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