Congenital nephrotic syndrome: a clinico-pathologic study of thirty children.
BACKGROUND
Congenital nephrotic syndrome is an uncommon disorder that may be caused by several diseases. These may be inherited, sporadic, acquired or part of a general malformation syndrome.


METHODS
We reviewed the clinical characteristics, pathologic findings, and results of medical management in 30 infants who presented to Jordan University Hospital with congenital nephrotic syndrome in the years 1989 to 1999.


RESULTS
Most patients (80%) had parents who were consanguineous. Most patients (80%) were born premature, with an average gestational age of 36 weeks. Most infants (77%) presented the nephrotic syndrome in the first three months of life and 26 (87%) had significant growth retardation. Twenty-five verified episodes of serious bacterial infections occurred in 18 patients. Antibiotic therapy however was successful in all these episodes. Light microscopy of the renal biopsies was consistent with the Finnish type of congenital nephrosis in most patients (83%). Chronic renal insufficiency developed in 17, and five of them needed chronic peritoneal dialysis. Most patients were given albumin transfusion and diuretic therapy especially during episodes of severe edema. Captopril alone or in combination with ibuprofen was given to eight patients, but without a response in any of them. All patients died before the age of 5 years. Most deaths occurred at an average age of 15 months (range 1-60).


CONCLUSIONS
The Finnish type of congenital nephrosis was the most common type in our patients, most of whom died within a few months of the onset of disease. In the developing countries, the management of patients with congenital nephrosis may have to be different from that in the developed countries in view of the high cost of medical management, poor outcome, high risk of serious complications, and high mortality rate.
Hamed
R M
RM
Division of Nephrology, Department of Pediatrics, Jordan University Hospital, Jordan. hamedr@index.com.jo
Shomaf
M
M
D001707:Biopsy, Needle; D002216:Captopril / Q000008:administration & dosage; D002675:Child, Preschool; D018450:Disease Progression; D004232:Diuretics / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007223:Infant; D007597:Jordan / Q000453:epidemiology; D008297:Male; D009404:Nephrotic Syndrome / Q000151:congenital* / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D011239:Prednisolone / Q000008:administration & dosage; D012042:Registries; D051437:Renal Insufficiency / Q000175:diagnosis / Q000401:mortality*; D012307:Risk Factors; D012720:Severity of Illness Index; D015996:Survival Rate
D004232:Diuretics; D002216:Captopril; D011239:Prednisolone