renal tubular acidosis type IV

Renal tubular acidosis (RTA) is a rare complication of renal involvement of systemic lupus erythematosus (SLE). We describe a 24-year-old male with type IV lupus nephropathy as a presenting manifestation of SLE. He presented with improvement of renal function following induction therapy with three pulses of methylprednisolone and 500 mg biweekly pulses of cyclophosphamide. However, a week after the first pulse of cyclophosphamide, the patient presented with a significant increase in legs edema and severe hyperkalemia...

Renal tubular acidosis (RTAs) are a group of metabolic disorders characterized by metabolic acidosis with normal plasma anion gap. There are three main forms of RTA: a proximal RTA called type II and a distal RTA (type I and IV). The RTA type II is a consequence of the inability of the proximal tubule to reabsorb bicarbonate. The distal RTA is associated with the inability to excrete the daily acid load and may be associated with hyperkalaemia (type IV) or hypokalemia (type I). The most common etiology of RTA type IV is the hypoaldosteronism...

Hump-nosed viper bite can cause acute kidney injury (AKI) and disseminated intravascular coagulation. In some patients, it can cause chronic kidney disease necessitating life-long renal replacement therapy. Lack of effective antivenom makes the management of these patients difficult. A 51-year-old Sri Lankan male was admitted with AKI and disseminated intravascular coagulation following a hump-nosed viper bite. He made a complete recovery with blood product support and hemodialysis. Renal biopsy was performed as his renal recovery was prolonged which revealed patchy tubular atrophy and interstitial inflammation suggestive of subacute interstitial nephritis...

OBJECTIVE: Hyperkalaemia is a common feature in hospitalized patients and often attributed to drugs antagonizing the renin-angiotensin-aldosterone system (RAAS) and/or acute kidney injury (AKI), despite significantly preserved glomerular filtration rate (GFR). The objective of this study was to determine the prevalence and role of renal tubular acidosis type IV (RTA IV) in the development of significant hyperkalaemia. DESIGN: A single-centre retrospective study...

Renal tubular acidosis (RTA) is a group of disorders observed in patients with normal anion gap metabolic acidosis. There are three major forms of RTA: A proximal (type II) RTA and two types of distal RTAs (type I and type IV). Proximal (type II) RTA originates from the inability to reabsorb bicarbonate normally in the proximal tubule. Type I RTA is associated with inability to excrete the daily acid load and may present with hyperkalaemia or hypokalaemia. The most prominent abnormality in type IV RTA is hyperkalaemia caused by hypoaldosteronism...

OBJECTIVE: Carbonic anhydrase (CA) inhibitors topiramate and zonisamide can induce metabolic acidosis in some patients. Our aims were to assess the prevalence and severity of this acidosis and to determine its predictors. METHODS: For 70 patients established on treatment with topiramate (n=55) or zonisamide (n=14) or both (n=1), we measured electrolytes, and genotyped single nucleotide polymorphisms (SNPs) in the main renal CA isoenzymes (II, IV and XII). RESULTS: Twenty-six percent of patients had a metabolic acidosis (serum bicarbonate <20 mmol/l)...

BACKGROUND: The Turkish Renal Tubular Disorders Working Group aimed to form a patient registry database and gathered demographic, clinical, and laboratory data in various hereditary renal tubular disorders (HRTDs). METHODS: A questionnaire comprising HRTDs was sent to the centers. The cohort was composed of 226 patients (109 girls, 117 boys). RESULTS: The distribution of patients according to HRTD was as follows: 45.6% distal renal tubular acidosis (dRTA), 26...

Severe hyperkalemia (>7 mmol/L) is a medical emergency because of possible fatal arrhythmias. We here report the case of a 58-year-old woman surviving extreme hyperkalemia (>10 mmol/L). The patient with a history of congestive heart failure, a DDD pacemaker and mild chronic renal insufficiency was admitted with progressive weakness and sudden onset of hypotension and bradycardia in the absence of any pacemaker action. Laboratory tests revealed an extreme serum potassium level of 10.1 mmol/L, with a slightly elevated serum creatinine of 149 μmol/L...

Metabolic acidosis may occasionally develop in the course of treatment with drugs used in everyday clinical practice, as well as with the exposure to certain chemicals. Drug-induced metabolic acidosis, although usually mild, may well be life-threatening, as in cases of lactic acidosis complicating antiretroviral therapy or treatment with biguanides. Therefore, a detailed medical history, with special attention to the recent use of culprit medications, is essential in patients with acid-base derangements. Effective clinical management can be handled through awareness of the adverse effect of certain pharmaceutical compounds on the acid-base status...

AIM: To contribute to understanding the pathogenesis of hyperkalemia that often occurs in patients with diabetes. MATERIALS AND METHODS: We describe 3 familial cases of mitochondrial diabetes mellitus. The mitochondrial A3243G point mutation was confirmed in a mother and her 2 children. We examined their clinical features and pathological findings, and assessed heteroplasmy of mutant mitochondria DNA (mtDNA) by molecular analysis. RESULTS: The second son had spontaneous hyperkalemia and hyporeninemic hypoaldosteronism...

BACKGROUND: The presence of vacuolar (v)H+-ATPase in distal tubule alpha-intercalated cells is essential for hydrogen excretion and maintenance of acid-base homeostasis. Loss of vH+-ATPase after kidney transplantation could cause posttransplant distal renal tubular acidosis (dRTA). METHOD: Immunostaining of the kidney specific vH+-ATPase of cortical collecting duct cells (CCT) was performed in 37 kidney biopsies taken immediately prior to transplantation and after engraftment (median [range]: 10 [1-181] months)...

OBJECTIVE: To review the current knowledge about primary periodic paralyses (PPs). RESULTS: Periodic paralyses are a heterogeneous group of disorders, clinically characterized by episodes of flaccid muscle weakness, occurring at irregular intervals. PPs are divided into primary (hereditary) and secondary (acquired) forms of which the secondary PPs are much more common than the primary PPs. Primary PPs are due to mutations in genes encoding for subunits of channel proteins of the skeletal muscle membrane, such as the muscular sodium, potassium or calcium channels, or the SCL4A1 protein...

A 21- year-old lady presented with chyluria, severe malnutrition, secondary amenorrhea, profound hypoalbuminemia, heavy proteinuria and renal tubular abnormalities suggestive of Type IV renal tubular acidosis. No particular cause for chyluria could be ascertained. She was successfully treated with an injection of 2% silver nitrate into the left ureter and urinary bladder. She continues to be well after 15 years of follow-up.

BACKGROUND: Neither the prevalence nor the associated risk factors of late post-transplant renal tubular acidosis (RTA) are known. METHODS: We conducted a cross-sectional study with 576 patients for more than 12 months after kidney transplantation, and a glomerular filtration rate (GFR) >40 ml/min. RTA was diagnosed by measurement of the urine anionic gap, urine pH and plasma potassium during acidosis, and fractional bicarbonate excretion after bicarbonate loading...

Kearns-Sayre syndrome (KSS) is a mitochondrial disease caused by large deletions in mitochondrial DNA (mtDNA). In most patients the disease is characterized by mtDNA heteroplasmy, where a mixture of wild-type and mutated mtDNA co-exist within cells in variable proportion, modulating the severity of the phenotype in different tissues. We report on the case of a 14-year-old child with classical symptoms of KSS and a renal phenotype characterized by hypokalaemic alkalosis, hypomagnesaemia, hyperreninaemia, hyperaldosteronism and nephrocalcinosis, resembling Bartter syndrome...

A cohort of 40 male children with Posterior Urethral Valves (PUV) seen in the Paediatric Nephrology/Urology Unit of the University College Hospital, Ibadan are presented. They were reviewed with the aim of determining the clinical course of the disease in a developing country where the facilities for active intervention are not always available. Even though 50% of the patients became symptomatic in the first week of life only 22.5% presented in the whole of the neonatal period. Thirty-seven and a half per-cent (37...

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FK506 is an immunosuppressant that is thought to be less nephrotoxic than cyclosporine A. However, complications due to renal tubular acidosis (RTA) have recently been reported. We report a case of RTA secondary to FK506 administration in liver transplantation. A 6-month-old girl was treated with FK506 after undergoing living donor liver transplantation for fulminant hepatitis. On postoperative day 17, she demonstrated hyperkalaemia and metabolic acidosis; she was diagnosed to have hyperkalaemic distal RTA with aldosterone deficiency (type IV)...

OBJECTIVE: To further study the renal damage of Sjögren's syndrome (SS) and its clinical and pathologic characteristics with biopsy and recent technology. METHODS: 84 patients of SS with renal impairment from 1993 to 1999 were analyzed by routine, immunoassay, tubular function and biopsy examination. RESULTS: 55 of the 84 cases presented with renal tubular acidosis (RTA), 5 with diabetes insipidus and 3 with hypokalemic paralysis. Glomerulopathy occurred in 22 cases (nephrotic syndrome 12, glomerulonephritis 10) and mild renal failure (RF) was found in 14...

The evaluation of dyspnea most often leads to a cardiac or pulmonary diagnosis. In the elderly, however, the cause is commonly multifactorial. The emergency physician should always consider noncardiopulmonary etiologies when treating such patients. We present 2 cases of new-onset type IV renal tubular acidosis (RTA) in older patients taking lisinopril who presented to the emergency department as dyspnea. Both patients had chronic cardiopulmonary illnesses and were initially diagnosed as having either congestive heart failure, asthma exacerbations, or both...