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
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).
CONCLUSION: 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.
Division of Nephrology, Department of Pediatrics, Jordan
University Hospital, Jordan.