Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A severe form of pseudohypoaldosteronism type 1 characterized by salt wasting in multiple organs including the kidney, colon, and sweat and salivary glands. Presentation is in the first few weeks of life with severe dehydration, vomiting and failure to thrive in association with hyponatremia, hyperkalemia and metabolic acidosis as well as elevated aldosterone and renin levels. No remission is reported and patients suffer from recurrent life-threatening episodes of salt loss. Disease data Classification Clinical subtype Synonyms Autosomal recessive PHA1 Autosomalny recesywny pseudohipoaldosteronizm typu 1 Autosomal recessive pseudohypoaldosteronism type 1 Generalized PHA1 ORPHA code 171876 OMIM code 264350 ICD10 code N25.8 ICD11 code GB90.41 *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl