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Hyporeninemic Hypoaldosteronism in Infancy: A Familial Disease

Endocrine Pediatric Unit, Hôpital Saint-Vincent-de-Paul Paris, France
INSERM U 36 Paris, France
Foundation de Recherche en Hormonologie Paris, France

Address requests for reprints to: Dr. Fabienne Landier, Hôpital Saint-Vincent-de-Paul, 74, Avenue Denfert-Rochereau, 75014 Paris, France.

Hyporeninemic hypoaldosteronism was found in two male siblings with urinary salt wasting and low plasma sodium levels. The eldest, aged 1 yr, had growth retardation, with hyponatremia and normal plasma potassium levels. The second, aged 2 months, had low plasma sodium and high plasma potassium levels. Both were severely and repeatedly hypoaldosteronemic. Primary adrenal deficiency was excluded by ACTH testing, which showed a good aldosterone rise and normal responses of other steroids. Both children had low PRA compared to that in age-matched normal subjects. The eldest sibling also had decreased total renin, low inactive to total renin ratio, and a subnormal level of angiotensinogen. The father had low plasma angiotensinogen levels. Congenital deficiency of renin activity and/or angiotensinogen production is suggested as the primary abnormality.

Received May 3, 1983.