Immunohistochemical comparison of a case of inherited distal renal tubular acidosis (with a unique AE1 mutation) with an acquired case secondary to autoimmune disease

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Abstract
Distal renal tubular acidosis (dRTA) is a defect in urinary acidification characterized by an inability to acidify the urine to (conventionally) pH <5.5, even in the presence of systemic acidaemia; it is often associated with nephrocalcinosis, recurrent renal calculi and reduced bone mineral density. The underlying causes of this syndrome are several: it may be inherited or secondary to other conditions, such as autoimmune disease (notably Sjögren's syndrome), drug therapy or nephrocalcinosis itself [1].