| Two Case of Psedohypoaldosteronism Type 1 |
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Ji Eun Lee, Jung Wan Seo, Seung Joo Lee |
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Department of Pediatrics, College of Medicine, Ewah Womans University Hospital, Seoul, Korea |
| 가성 저알도스테론증 1형 2례 |
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이지은, 서정완, 이승주 |
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이화여자대학교 의과대학 소아과학교실 |
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| Abstract |
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Pseudohypoaldosteronism type 1 is a genetic renal tubular disease of salt wasting, presenting in young infants. Tubular unresponsiveness to elevated endogenous an exogenou aldosterone is the suggested pathogenetic mechanism. Oral sodium chloride supplementation relieve the clinical symptoms and electrolyte distrubances.
We experienced 2 cases of PHA type 1 in 38-day and 450day old male infants who were presented with failure to thrive, vomiting and/or dehydration. Laboratory data showed hyponatremia, hyperkalemia, hypochloremia and metabolic acidosis. Renal and adrenal functions were normal. Plasma renin activity and plasma aldosterone concentration were markedly elevated. Under the diagnosis of Psedohypoaldosteronism Type 1, oral supplementation of NaCl and/or kayexalate improved the clinical states of the patients. |
| Key Words:
Psedohypoaldosteronism Type 1, Hyponatremia, Renal salt wasting |
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