Pseudohypoaldosteronism (PHA) is a rare hereditary salt-wasting syndro
me which is caused by decreased renal tubular responsiveness to aldost
erone. The syndrome consists of hyponatremia, hyperkalemia, dehydratio
n, failure to thrive and increased urinary salt loss. A case of PHA wa
s previously described where fetal polyuria was the probable cause of
hydramnios. We present four new cases of PHA, from two families, who w
ere born after pregnancies complicated by severe hydramnios and premat
ure labor. We suggest that PHA should be included in the differential
diagnosis of hydramnios, since appropriate investigations might lead t
o the early diagnosis and treatment of a life-threatening disease.