International Journal of Gynecology & Obstetrics 69 Ž2000. 53᎐54
Brief communication
Polyhydramnios as a sign of fetal pseudohypoaldosteronism H. Narchi b,U , M. Santos a , N. Kulaylat a a
Saudi Aramco-Al-Hasa Health Center, Saudi Aramco Medical Ser¨ ices Organization, Mubarraz, Saudi Arabia b Pediatric Department, Sandwell District General Hospital, West Midlands, UK Received 29 September 1999; received in revised form 15 December 1999; accepted 15 December 1999
Keywords: Polyhydramnios; Pseudohypoaldosteronism; Pregnancy; Neonate
A gravida-11 para-8 mother, married to her first cousin, was diagnosed to have polyhydramnios at 25 weeks of gestation. A term infant, born several years earlier after a pregnancy complicated by polyhydramnios, died suddenly at home at 10 days of age. During the current pregnancy blood pressure was normal. Diabetes and alloimmunization were ruled out. No fetal congenital anomalies were noted on ultrasound. Oral indomethacin therapy resulted in a moderate reduction of the polyhydramnios. However, decompression amniocentesis was still required at 36 weeks for symptomatic relief. A 2900-g female infant was born by cesarean section at term. The physical examination was normal. Over the next few days, weight decreased by 10% despite adequate caloric intake. Serum sodium declined to 124 mEq.rl, and serum potassium increased to U
Corresponding author. Tel.: q44-121-607-3471; fax: q44121-607-3596. E-mail address:
[email protected] ŽH. Narchi.
7.5 mEq.rl, without any vomiting or diarrhea. Blood pressure remained normal. A trial of fludrocortisone did not correct the anomalies, which quickly responded to intravenous NaCl infusion and oral ion-exchange resin. Serum 17-hydroxyprogesterone, cortisol and dehydroepiandrosterone sulfate were normal. Serum aldosterone and plasma renin activity were very high, confirming the diagnosis of pseudohypoaldosteronism ŽPHA.. The infant was started on oral sodium supplementation, ion-exchange resin and oral indomethacin. The electrolyte imbalance was quickly corrected. The child improved and has remained well since. PHA is a rare autosomal recessive condition. There is decreased renal tubular responsiveness to aldosterone. Manifestations include hyponatremia, hyperkalemia, dehydration and increased urinary salt loss. The serum aldosterone and renin levels are high, indicating hyperactivity of the renin᎐angiotensin system. It is likely that the death of the prior sibling was also due to PHA.
0020-7292r00r$20.00 䊚 2000 International Federation of Gynecology and Obstetrics. PII: S 0 0 2 0 - 7 2 9 2 Ž 0 0 . 0 0 1 8 0 - 6
54
H. Narchi et al. r International Journal of Gynecology & Obstetrics 69 (2000) 53᎐54
Other causes of polyhydramnios include fetal Bartter or hyperprostaglandin E syndrome, nephrogenic diabetes insipidus and fetal PHA w1x. In fetal hypoaldosteronism, maternal aldosterone crosses the placenta, ensuring normal fetal homeostatis, with manifestations occurring only after birth. Elevated amniotic fluid aldosterone is reliable for the prenatal diagnosis of Bartter syndrome but this has not been studied in PHA w2x. Maternal indomethacin therapy is successfully used in fetal Bartter syndrome but has not been studied in PHA w3x. Rare causes for polyhydramnios, such as PHA, should be sought when no other obvious cause is found, especially with a history of recurrent polyhydramnios and unexplained neonatal deaths.
Communication between obstetrician and pediatrician would lead to early diagnosis and specific therapy of this life-threatening disease prior to neonatal discharge. Genetic counseling of affected families is mandatory. References w1x Greenberg D, Abramson O, Phillip M. Fetal pseudohypoaldosteronism: another cause of hydramnios. Acta Paediatr 1995;84:582᎐584. w2x Shalev H, Ohaly M, Meizner I, Carmi R. Prenatal diagnosis of Bartter syndrome. Prenat Diagn 1994;14: 996᎐998. w3x Konrad M, Leonhardt A, Hensen P, Seyberth HW, Kockerling A. Prenatal and postnatal management of hyperprostaglandin E syndrome after genetic diagnosis from amniocytes. Pediatrics 1999;103:678᎐683.
