Hereditary pseudohypoaldosteronism
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Synopsis
A rare inherited disorder in which there is aldosterone resistance. It is manifested by hyperkalemic metabolic acidosis, urinary salt wasting, normal or increased aldosterone secretion, and normal glomerular filtration rate. Other signs and symptoms include vomiting, diarrhea, failure to thrive, poor feeding, and dehydration. Onset during infancy and childhood. Autosomal dominant form involves salt loss from kidneys; autosomal recessive form involves salt loss from multiple systems (kidney, lungs, salivary, and sweat glands). Severe cases involving multiple organ systems in infants may be life-threatening. Patients with pseudohypoaldosteronism type II, or Gordon syndrome, can also present with hypertension.
Codes
ICD10CM:
E83.89 – Other disorders of mineral metabolism
SNOMEDCT:
77098009 – Pseudohypoaldosteronism
E83.89 – Other disorders of mineral metabolism
SNOMEDCT:
77098009 – Pseudohypoaldosteronism
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Last Updated:06/26/2022
Hereditary pseudohypoaldosteronism