Korean J Nephrol.  1997 Mar;16(1):136-141.

Congenital Nephrotic Syndrome

• Lee YK
• Cha ES
• Kwon MJ
• Lee JS
• Kim PK
• Jeong HJ

Abstract

Congenital nephrotic syndrome(CNS) is a rare disease defined by nephrotic syndrome at birth or within the 1st year of life. This study is performed to investigate a classification based on clinicopathology and to evaluate the clinical course and prognosis according to types of CNS. We performed retrospective clinical study with chart review in 8 patients who were diagnosed as CNS from 1980 to 1995. The results were as follows: Their ages at the onset of illness ranged from birth to 7 months (median 2.2 months) and there were 7 males and 1 female. There were proteinuria, hypoalbuminemia and edema in all cases, accompanied with ascites(7cases), hematuria(5cases), hepatosplenomegaly(2cases), umbilical hernia(1case) and inguinal hernia(1case). A classification of these based on clinicopathology showed CNS of Finnish type in 2 patients, congenital syphilitic nephrotic syndrome in 2, mesangial glomerulosclerosis, minimal change disease, Drash syndrome and undefined CNS in 1 each. Of the 8 patients with CNS, 3 died of sepsis and renal failure, 1 responded to steroid and cyclosporin therapy and is alive at 20 months, 1 responded to penicillin, 1 discharged voluntarily, and 2 were lost to follow-up. In conclusion, it had been considered that all forms of CNS except the secondary ones have a very poor prognosis. But if the appropriate management including early renal transplantation is established under the definite diagnosis which is based on clinicopathology, we can expect long term survival, normal growth and development for the child of CNS.

Keyword

Congenital nephrotic syndrome(CNS)

MeSH Terms

Child
Classification
Cyclosporine
Denys-Drash Syndrome
Diagnosis
Edema
Female
Growth and Development
Humans
Hypoalbuminemia
Kidney Transplantation
Lost to Follow-Up
Male
Nephrosis, Lipoid
Nephrotic Syndrome*
Parturition
Penicillins
Prognosis
Proteinuria
Rare Diseases
Renal Insufficiency
Retrospective Studies
Sepsis
Cyclosporine
Penicillins