Autosomal recessive distal renal tubular acidosis

Orpha code: 402041OMIM code: 602722

Definition

A rare autosomal recessive form of proximal renal tubular acidosis (pRTA) characterized by an isolated defect in the proximal tubule leading to the decreased reabsorption of bicarbonate and consequently causing urinary bicarbonate wastage. Mild growth retardation and reduced bone density are extra-renal complications. Several fractures and delayed puberty are possible features.

Disease data
Classification

Clinical subtype

Synonyms
AR dRTA
AR dRTA
Autosomalna recesywna dystalna RTA
Autosomal recessive distal RTA
ORPHA code
402041
OMIM code
602722
ICD10 code
N25.8
ICD11 code
-

No additional description.

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