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Proximal renal tubular acidosis (pRTA) or type 2 renal tubular acidosis (RTA) is a type of RTA caused by a failure of the proximal tubular cells to reabsorb filtered bicarbonate from the urine, leading to urinary bicarbonate wasting and subsequent acidemia. The distal intercalated cells function normally, so the acidemia is less severe than ...
Renal tubular acidosis (RTA) is a medical condition that involves an accumulation of acid in the body due to a failure of the kidneys to appropriately acidify the urine. [1] In renal physiology, when blood is filtered by the kidney, the filtrate passes through the tubules of the nephron, allowing for exchange of salts, acid equivalents, and other solutes before it drains into the bladder as urine.
Distal renal tubular acidosis (dRTA) is the classical form of RTA, being the first described. Distal RTA is characterized by a failure of acid secretion by the alpha intercalated cells of the distal tubule and cortical collecting duct of the distal nephron. [1] This failure of acid secretion may be due to a number of causes.
Fanconi syndrome or Fanconi's syndrome (English: / f ɑː n ˈ k oʊ n i /, / f æ n-/) is a syndrome of inadequate reabsorption in the proximal renal tubules [1] of the kidney.The syndrome can be caused by various underlying congenital or acquired diseases, by toxicity (for example, from toxic heavy metals), or by adverse drug reactions. [2]
It is used to aid in the differential diagnosis of metabolic acidosis. [2] The term "anion gap" without qualification usually implies serum anion gap. The "urine anion gap" is a different measure, principally used to determine whether the kidneys are capable of appropriately acidifying urine.
For their contribution to acid–base homeostasis, the intercalated cells play important roles in the kidney's response to acidosis and alkalosis. Damage to the α-intercalated cell's ability to secrete acid can result in distal renal tubular acidosis (RTA type I, classical RTA)(reference). The intercalated cell population is also extensively ...
[9] [10] A key insight was that in the group of diseases termed 'Renal Tubular Acidosis' (RTA), urinary excretion of ammonium was relatively well preserved. This was unlike the situation in chronic kidney failure. Furthermore, the paper identified a subset of patients with 'incomplete' RTA.
There are four types of renal tubular acidosis. Type 1 is distal renal tubular acidosis and results from a failure of the cortical collecting duct to acidify the urine below pH 5. [48] Some patients with autosomal recessive dRTA also have sensorineural hearing loss. [49] Inheritance of this type of RTA results from either mutations to V-ATPase ...