Type Ib pseudohypoaldosteronism: clinical and nosological aspects. Three new cases.

Background. - Type Ib pseudohypoaldosteronism is a congenital disorder char acterized in the newborn by salt loss caused by multiple end-organ resistan ce to aldosterone. An autosomal recessive mode of inheritance has been repo rted. Its particularity is the spontaneous improvement by 18 months to 2 ye ars, due to an improved tubular response of the kidneys to mineralocorticoi ds, or earlier when given salt supplements once the diagnosis is made. Observations. - We observed three children,with this disease, which was rev ealed by day 8 to day 15 of life; one of these presented respiratory sympto ms identical to those of cystic fibrosis, and another one an apparently cha nce association with a rod myopathy. Conclusion. - Recent findings in the literature demonstrate the molecular a spects of pseudohypoaldosteronism and lend to nn interesting comparison wit h cystic fibrosis by explaining their similar physiopathology through the a ctivity of epithelial sodium channels. (C) 1999 Editions scientifiques et m edicales Elsevier SAS.