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Kunchur MG, Mauch TJ, Parkanzky M, Rahilly LJ. A review of renal tubular acidosis. J Vet Emerg Crit Care (San Antonio) 2024; 34:325-355. [PMID: 39023331 DOI: 10.1111/vec.13407] [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: 01/08/2021] [Revised: 10/14/2022] [Accepted: 11/11/2022] [Indexed: 07/20/2024]
Abstract
OBJECTIVE To review the current scientific literature on renal tubular acidosis (RTA) in people and small animals, focusing on diseases in veterinary medicine that result in secondary RTA. DATA SOURCES Scientific reviews and original research publications on people and small animals focusing on RTA. SUMMARY RTA is characterized by defective renal acid-base regulation that results in normal anion gap hyperchloremic metabolic acidosis. Renal acid-base regulation includes the reabsorption and regeneration of bicarbonate in the renal proximal tubule and collecting ducts and the process of ammoniagenesis. RTA occurs as a primary genetic disorder or secondary to disease conditions. Based on pathophysiology, RTA is classified as distal or type 1 RTA, proximal or type 2 RTA, type 3 RTA or carbonic anhydrase II mutation, and type 4 or hyperkalemic RTA. Fanconi syndrome comprises proximal RTA with additional defects in proximal tubular function. Extensive research elucidating the genetic basis of RTA in people exists. RTA is a genetic disorder in the Basenji breed of dogs, where the mutation is known. Secondary RTA in human and veterinary medicine is the sequela of diseases that include immune-mediated, toxic, and infectious causes. Diagnosis and characterization of RTA include the measurement of urine pH and the evaluation of renal handling of substances that should affect acid or bicarbonate excretion. CONCLUSIONS Commonality exists between human and veterinary medicine among the types of RTA. Many genetic defects causing primary RTA are identified in people, but those in companion animals other than in the Basenji are unknown. Critically ill veterinary patients are often admitted to the ICU for diseases associated with secondary RTA, or they may develop RTA while hospitalized. Recognition and treatment of RTA may reverse tubular dysfunction and promote recovery by correcting metabolic acidosis.
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Affiliation(s)
| | - Teri Jo Mauch
- University of Nebraska Medical Center and Children's Hospital, Omaha, Nebraska, USA
- University of Utah Health Sciences Center, Salt Lake City, Utah, USA
| | | | - Louisa J Rahilly
- Cape Cod Veterinary Specialists, Buzzards Bay, Massachusetts, USA
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Lee EK, Yang WS. Use of Fludrocortisone for Hyperkalemia in Chronic Kidney Disease Not Yet on Dialysis. Electrolyte Blood Press 2024; 22:8-15. [PMID: 38957547 PMCID: PMC11214912 DOI: 10.5049/ebp.2024.22.1.8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2023] [Revised: 06/23/2024] [Accepted: 06/24/2024] [Indexed: 07/04/2024] Open
Abstract
Background Hyperkalemia is a frequent and potentially lethal complication of chronic kidney disease (CKD). We retrospectively examined the potassium-lowering effect of oral fludrocortisone and its adverse effects in hyperkalemic CKD patients not yet on dialysis. Methods Thirty-three patients (23 men and 10 women, ages 69±14 years) were included. To control hyperkalemia at the outpatient clinic, twenty-one patients (Group 1) received fludrocortisone (0.05-0.1 mg/day) without changes in angiotensin II receptor blockers (ARBs) and calcium polystyrene sulfonate (CPS), while twelve patients (Group 2) were treated with fludrocortisone in addition to stopping ARBs and/or adding low-dose CPS. Results Fludrocortisone was administered for a median of 169 days (interquartile range, 47-445). At the first follow-up after fludrocortisone administration, serum potassium dropped from 6.14±0.32 mEq/L to 4.52±1.06 mEq/L (p<0.001) in Group 1 and from 6.37±0.35 mEq/L to 4.08±0.74 mEq/L (p<0.01) in Group 2. Ten patients in Group 1 and five patients in Group 2 measured serum potassium levels at four outpatient visits before and after fludrocortisone administration, respectively. The frequency of serum potassium ≥6.0 mEq/L decreased from 19/40 (48%) to 2/40 (5%) (p<0.001) in Group 1 and from 11/20 (55%) to 0/20 (0%) (p<0.001) in Group 2. Eleven patients experienced sodium retention-related problems after fludrocortisone administration: 7 with worsening leg edema, 2 with pleural effusions, and 2 with pulmonary edema. Conclusion In pre-dialysis CKD patients, fludrocortisone at low doses effectively reduced serum potassium levels; however, sodium retention was a common adverse effect.
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Affiliation(s)
- Eun Kyoung Lee
- Division of Nephrology, Department of Internal Medicine, Dankook University Hospital, Dankook University College of Medicine, Cheonan, Republic of Korea
| | - Won Seok Yang
- Division of Nephrology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
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Al-Beltagi M, Saeed NK, Bediwy AS, Elbeltagi R, Hasan S, Hamza MB. Renal calcification in children with renal tubular acidosis: What a paediatrician should know. World J Clin Pediatr 2023; 12:295-309. [PMID: 38178934 PMCID: PMC10762599 DOI: 10.5409/wjcp.v12.i5.295] [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: 07/29/2023] [Revised: 09/15/2023] [Accepted: 10/16/2023] [Indexed: 12/08/2023] Open
Abstract
Renal tubular acidosis (RTA) can lead to renal calcification in children, which can cause various complications and impair renal function. This review provides pediatricians with a comprehensive understanding of the relationship between RTA and renal calcification, highlighting essential aspects for clinical management. The article analyzed relevant studies to explore the prevalence, risk factors, underlying mechanisms, and clinical implications of renal calcification in children with RTA. Results show that distal RTA (type 1) is particularly associated with nephrocalcinosis, which presents a higher risk of renal calcification. However, there are limitations to the existing literature, including a small number of studies, heterogeneity in methodologies, and potential publication bias. Longitudinal data and control groups are also lacking, which limits our understanding of long-term outcomes and optimal management strategies for children with RTA and renal calcification. Pediatricians play a crucial role in the early diagnosis and management of RTA to mitigate the risk of renal calcification and associated complications. In addition, alkaline therapy remains a cornerstone in the treatment of RTA, aimed at correcting the acid-base imbalance and reducing the formation of kidney stones. Therefore, early diagnosis and appropriate therapeutic interventions are paramount in preventing and managing renal calcification to preserve renal function and improve long-term outcomes for affected children. Further research with larger sample sizes and rigorous methodologies is needed to optimize the clinical approach to renal calcification in the context of RTA in the pediatric population.
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Affiliation(s)
- Mohammed Al-Beltagi
- Department of Pediatrics, Faculty of Medicine, Tanta University, Tanta 31511, Alghrabia, Egypt
- Department of Pediatrics, University Medical Center, King Abdulla Medical City, Dr. Sulaiman Al Habib Medical Group, Manama, Bahrain, Manama 26671, Manama, Bahrain
| | - Nermin Kamal Saeed
- Medical Microbiology Section, Department of Pathology, Salmaniya Medical Complex, Ministry of Health, Kingdom of Bahrain, Manama 12, Manama, Bahrain
- Medical Microbiology Section, Department of Pathology, Irish Royal College of Surgeon, Bahrain, Busaiteen 15503, Muharraq, Bahrain
| | - Adel Salah Bediwy
- Department of Pulmonology, Faculty of Medicine, Tanta University, Tanta 31527, Alghrabia, Egypt
- Department of Chest Disease, University Medical Center, King Abdulla Medical City, Arabian Gulf University, Dr. Sulaiman Al Habib Medical Group, Manama, Manama 26671, Manama, Bahrain
| | - Reem Elbeltagi
- Department of Medicine, The Royal College of Surgeons in Ireland - Bahrain, Busiateen 15503, Muharraq, Bahrain
| | - Samir Hasan
- Department of Pediatrics, Faculty of Medicine, Tanta University Hospital, Tanta 31511, Algharbia, Egypt
| | - Mohamed Basiony Hamza
- Department of Pediatrics, Faculty of Medicine, Tanta University, Tanta 31511, Algharbia, Egypt
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Yao Y, Audil HY, Aakre CA. 91-Year-Old Woman With Hyperkalemia. Mayo Clin Proc 2023; 98:1552-1556. [PMID: 37793729 DOI: 10.1016/j.mayocp.2023.01.029] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/17/2022] [Revised: 01/24/2023] [Accepted: 01/26/2023] [Indexed: 10/06/2023]
Affiliation(s)
- Yuan Yao
- Resident in Internal Medicine, Mayo Clinic School of Graduate Medical Education, Rochester, MN
| | - Hadiyah Y Audil
- Fellow in Hematology and Medical Oncology, Mayo Clinic School of Graduate Medical Education, Rochester, MN
| | - Christopher A Aakre
- Advisor to residents and Fellow and Consultant in General Internal Medicine, Mayo Clinic, Rochester, MN.
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Adomako EA, Maalouf NM. Type 4 renal tubular acidosis and uric acid nephrolithiasis: two faces of the same coin? Curr Opin Nephrol Hypertens 2023; 32:145-152. [PMID: 36683539 PMCID: PMC9881823 DOI: 10.1097/mnh.0000000000000859] [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] [Indexed: 01/24/2023]
Abstract
PURPOSE OF REVIEW The present review summarizes findings of recent studies examining the epidemiology, pathophysiology, and treatment of type 4 renal tubular acidosis (RTA) and uric acid nephrolithiasis, two conditions characterized by an abnormally acidic urine. RECENT FINDINGS Both type 4 RTA and uric acid nephrolithiasis disproportionately occur in patients with type 2 diabetes and/or chronic kidney disease. Biochemically, both conditions are associated with reduced renal ammonium excretion resulting in impaired urinary buffering and low urine pH. Reduced ammoniagenesis is postulated to result from hyperkalemia in type 4 RTA and from insulin resistance and fat accumulation in the renal proximal tubule in uric acid nephrolithiasis. The typical biochemical findings of hyperkalemia and systemic acidosis of type 4 RTA are rarely reported in uric acid stone formers. Additional clinical differences between the two conditions include findings of higher urinary uric acid excretion and consequent urinary uric acid supersaturation in uric acid stone formers but not in type 4 RTA. SUMMARY Type 4 RTA and uric acid nephrolithiasis share several epidemiological, clinical, and biochemical features. Although both conditions may be manifestations of diabetes mellitus and thus have a large at-risk population, the means to the shared biochemical finding of overly acidic urine are different. This difference in pathophysiology may explain the dissimilarity in the prevalence of kidney stone formation.
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Affiliation(s)
- Emmanuel A. Adomako
- Department of Internal Medicine, Division of Nephrology and Hypertension, University of Kansas Medical Center, Kansas City, Kansas, USA
| | - Naim M. Maalouf
- Charles and Jane Pak Center for Mineral Metabolism and Clinical Research, and Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA
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Sarnowski A, Gama RM, Dawson A, Mason H, Banerjee D. Hyperkalemia in Chronic Kidney Disease: Links, Risks and Management. Int J Nephrol Renovasc Dis 2022; 15:215-228. [PMID: 35942480 PMCID: PMC9356601 DOI: 10.2147/ijnrd.s326464] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2022] [Accepted: 06/22/2022] [Indexed: 12/21/2022] Open
Abstract
Hyperkalemia is a common clinical problem with potentially fatal consequences. The prevalence of hyperkalemia is increasing, partially due to wide-scale utilization of prognostically beneficial medications that inhibit the renin-angiotensin-aldosterone-system (RAASi). Chronic kidney disease (CKD) is one of the multitude of risk factors for and associations with hyperkalemia. Reductions in urinary potassium excretion that occur in CKD can lead to an inability to maintain potassium homeostasis. In CKD patients, there are a variety of strategies to tackle acute and chronic hyperkalemia, including protecting myocardium from arrhythmias, shifting potassium into cells, increasing potassium excretion from the body, addressing dietary intake and treating associated conditions, which may exacerbate problems such as metabolic acidosis. The evidence base is variable but has recently been supplemented with the discovery of novel oral potassium binders, which have shown promise and efficacy in studies. Their use is likely to become widespread and offers another tool to the clinician treating hyperkalemia. Our review article provides an overview of hyperkalemia in CKD patients, including an exploration of relevant guidelines and nuances around management.
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Affiliation(s)
- Alexander Sarnowski
- Department of Renal Medicine and Transplantation, St George’s NHS University Hospitals NHS Foundation Trust, London, UK
| | - Rouvick M Gama
- Department of Renal Medicine and Transplantation, St George’s NHS University Hospitals NHS Foundation Trust, London, UK
| | - Alec Dawson
- Department of Renal Medicine and Transplantation, St George’s NHS University Hospitals NHS Foundation Trust, London, UK
| | - Hannah Mason
- Department of Renal Medicine and Transplantation, St George’s NHS University Hospitals NHS Foundation Trust, London, UK
| | - Debasish Banerjee
- Department of Renal Medicine and Transplantation, St George’s NHS University Hospitals NHS Foundation Trust, London, UK
- Correspondence: Debasish Banerjee, Department of Renal Medicine and Transplantation, St George’s NHS University Hospitals NHS Foundation Trust, Blackshaw Road, SW170QT, London, United Kingdom, Tel +44 2087151673, Email
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Palmer BF, Kelepouris E, Clegg DJ. Renal Tubular Acidosis and Management Strategies: A Narrative Review. Adv Ther 2021; 38:949-968. [PMID: 33367987 PMCID: PMC7889554 DOI: 10.1007/s12325-020-01587-5] [Citation(s) in RCA: 52] [Impact Index Per Article: 13.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/03/2020] [Accepted: 11/26/2020] [Indexed: 12/29/2022]
Abstract
Renal tubular acidosis (RTA) occurs when the kidneys are unable to maintain normal acid−base homeostasis because of tubular defects in acid excretion or bicarbonate ion reabsorption. Using illustrative clinical cases, this review describes the main types of RTA observed in clinical practice and provides an overview of their diagnosis and treatment. The three major forms of RTA are distal RTA (type 1; characterized by impaired acid excretion), proximal RTA (type 2; caused by defects in reabsorption of filtered bicarbonate), and hyperkalemic RTA (type 4; caused by abnormal excretion of acid and potassium in the collecting duct). Type 3 RTA is a rare form of the disease with features of both distal and proximal RTA. Accurate diagnosis of RTA plays an important role in optimal patient management. The diagnosis of distal versus proximal RTA involves assessment of urinary acid and bicarbonate secretion, while in hyperkalemic RTA, selective aldosterone deficiency or resistance to its effects is confirmed after exclusion of other causes of hyperkalemia. Treatment options include alkali therapy in patients with distal or proximal RTA and lowering of serum potassium concentrations through dietary modification and potential new pharmacotherapies in patients with hyperkalemic RTA including newer potassium binders.
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Abstract
Hyperkalemia is a potentially life-threatening electrolyte disorder appreciated with greater frequency in patients with renal disease, heart failure, and with use of certain medications such as renin angiotensin aldosterone inhibitors. The traditional views that hyperkalemia can be reliably diagnosed by electrocardiogram and that particular levels of hyperkalemia confer cardiotoxic risk have been challenged by several reports of patients with atypic presentations. Epidemiologic data demonstrate strong associations of morbidity and mortality in patients with hyperkalemia but these associations appear disconnected in certain patient populations and in differing clinical presentations. Physiologic adaptation, structural cardiac disease, medication use, and degree of concurrent illness might predispose certain patients presenting with hyperkalemia to a lower or higher threshold for toxicity. These factors are often overlooked; yet data suggest that the clinical context in which hyperkalemia develops is at least as important as the degree of hyperkalemia is in determining patient outcome. This review summarizes the clinical data linking hyperkalemia with poor outcomes and discusses how the efficacy of certain treatments might depend on the clinical presentation.
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Affiliation(s)
- John R Montford
- Division of Renal Disease and Hypertension, Department of Medicine, University of Colorado Anschutz Medical Campus, Aurora, Colorado; .,Renal Section, Medicine Service, Veterans Affairs Eastern Colorado Health System, Denver, Colorado; and
| | - Stuart Linas
- Division of Renal Disease and Hypertension, Department of Medicine, University of Colorado Anschutz Medical Campus, Aurora, Colorado.,Division of Nephrology, Department of Medicine, Denver Health and Hospitals, Denver, Colorado
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