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Hereditary pseudohypoaldosteronism
Other Resources UpToDate PubMed

Hereditary pseudohypoaldosteronism

Contributors: Catherine Moore MD
Other Resources UpToDate PubMed

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

Differential Diagnosis & Pitfalls

Best Tests

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References

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Last Updated:06/27/2016
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Hereditary pseudohypoaldosteronism
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Hereditary pseudohypoaldosteronism : Diarrhea, Vomiting, Dehydration, Failure to thrive, Hyperkalemia, Metabolic acidosis, Na decreased
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