Histopathologic and Clinical Findings of Congenital Nephrotic Syndrome in Iranian Children: a Study of Two Centers

Abstract

Introduction. Congenital nephrotic syndrome (CNS), an uncommon form of kidney disease, presents during the first year of life and is a diagnostic and therapeutic challenge for the pediatricians as well as pediatric nephrologists. Our study is the first study of Iranian children with CNS in two pediatric nephrology centers in Tehran, Iran.

Materials and Methods. We reviewed medical charts of 30 infants diagnosed with CNS from 1990 to 2005.

Results. There were 15 boys and 15 girls with CNS (mean age, 1.7 months). The presentation of the disease was nephrotic syndrome in 96.6% of the patients. Eighty percent of the patients presented within 3 months of life and 16 in the neonatal period. The Finnish type of CNS was seen in 43.3% and diffuse mesangial sclerosis in 50%. Preterm labor and low birth weight was seen in 20%. A family history of nephrotic syndrome in infancy was noted for 8 children (26.7%). Numerous complications of nephrotic syndrome occurred in 73.3%. Seventy percent of the patients had 27 episodes of infections. Sepsis was seen in 43.3% of the children, of which 61.5% were caused by gram-negative bacteria and 38.6% were caused by Staphylococcus aureus. Thrombotic complications and hypertension developed in 6.6% and 23.3% of the patients, respectively. The mortality rate of patients was 86.6%.

Conclusions. Diffuse mesangial sclerosis is an important cause of CNS. The outcome of our patients was poor and most of our patients died before reaching the age of 5 years old.