![]() The diseases which are associated with Fanconi syndrome, for example, Wilson disease, cystinosis, galactosemia, hereditary fructose intolerance and von Gierke disease, are also rare. Type 2 Proximal RTA is rarer than type 1 distal RTA and Fanconi syndrome is very rare as a primary disease. There is over 20% incidence of developing RTA after renal transplant in the setting of rejection or immunosuppressive drugs. The most common Type in the United States and worldwide is type 4 hyperkalemic RTA, the main causes of which are diabetic nephropathy leading to hyporeninemic hypoaldosteronism and urinary tract obstruction. The disease is undiagnosed in most of the cases and also in cases with incomplete RTA. Inherited forms of RTA are much rarer than acquired forms. RTA with its all major subtypes is an uncommon entity. Miscellaneous: Interstitial nephritis, chronic obstruction of the urinary tract, adrenal insensitivity to angiotensin II, renal insufficiency Genetic: Sickle cell disease, pseudohypoaldosteronism Hyporeninemic hypoaldosteronism: most common cause in adults is diabetic nephropathy-destruction of JG apparatus due to vascular hyalinosis ĭrugs: Potassium-sparing diuretics, beta blockers, NSAIDs, calcineurin inhibitors (cyclosporine, tacrolimus), ACEi, ARBs, renin inhibitors, heparin, TMP/SMX Inherited: Mutations in carbonic anhydrase II. ![]() Miscellaneous: Interstitial nephritis, Fanconi syndrome, vitamin D deficiency, secondary hyperparathyroidism, chronic hepatitis, idiopathic Inherited: AD or AR putative mutations in Na-H antiporter in apical membrane and Na-HCO3 cotransporter in the basolateral membrane of proximal tubular cells respectivelyĭrugs: Lead or other heavy metals, carbonic anhydrase inhibitors (e.g., acetazolamide, topiramate), out of date tetracyclines, aminoglycosides, valproate, mercury, tenofovir, and ifosfamide (nephrotoxic)Īutoimmune: Sjogren syndrome, systemic lupus erythematosus (SLE) Hypergammaglobulinemic states: Most common cause in adults-monoclonal gammopathy(light chain), multiple myeloma, amyloidosis Miscellaneous: Idiopathic, familial hypercalciuria, glue sniffing (toluene inhalation in recreational drug abuse) Hypergammaglobulinemic states: Monoclonal gammopathy, multiple myeloma, amyloidosis, cryoglobulinemia, chronic liver diseaseĭrugs: Lithium, amphotericin B, NSAIDs, lead, antivirals Tubulointerstitial diseases: chronic pyelonephritis, chronic interstitial nephritis, obstructive uropathy, renal transplant rejection Nephrocalcinosis: Chronic hypercalcemia, medullary sponge kidney Genetic associations: Marfan syndrome, Ehler Danlos syndrome, sickle cell disease, congenital obstruction of the urinary tract Inherited, AD or AR: Genetic primary causes of distal RTA include mutations of genes that encode the chloride-bicarbonate exchanger (AE1) or subunits of the H-ATPase pump respectively ![]() Autoimmune diseases are the commonest cause in adults: Systemic lupus erythematosus (SLE), Sjogren syndrome, rheumatoid arthritis, systemic sclerosis, thyroiditis, hepatitis, primary biliary cirrhosis. ![]()
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