Abstract Ref Number = APCP1096
CASES REPORT OF CONGENITAL NEPHROTIC SYNDROME
Bob Kevin Pardede,Omega Mellyana,Heru Muryawan,Retno palupi,Susilorini
Department of pediatrics faculty of medicine Diponegoro University Department of Pediatrics Faculty of Medicine Gadjah Mada Universitydr Sardjito Hospital Yogyakarta Department of PediatricsMardi Rahayu Hospital KudusCentral Java
Background : Congenital nephrotic syndrome (CNS) is a nephrotic syndrome presenting in the first year of life consist of below three month of life and infant nephrotic syndrome (3 -12 months). It affects 1 to 3 per 100,000 children worldwide. Both are associated with profound growth failure and death from infection during infancy. The prognosis of CNS is very poor and mortality occurs within the first 6 months.
Case Presentation Summary : Six patient came with history chief complain of lower exstremities edema in the first year of life. Edema became generalized with ascites. The average of birth weight is 2200 – 2600 gram. Four patients were aterm (37-39 weeks) but 2 patient is preterm (35 weeks). APGAR score of all patient were normal. Three patient have family history of kidney disease. Prenatal history was remarkable. Laboratory examination showed mild anemia (9,4-11,7 g/dl), hipoalbumin (1.0–1.9 g/dl), hipercholesterol (>200 mg/dl). Urinalisis showed proteinuria (+3), microscopic hematuria (50 – 250 /LPB). One of patient had hyponatremia. All patient received treatment with albumin infusions, diuretic drugs and kortikosteroid ace-inhibitor at less than 3 months of age and two patient underwent a renal biopsy at 16 month of age. The proteinuria can not disappear and serum protein level 1.2 – 3.5, cholesterol, albumin returned to normal levels during the 16-month follow-up period. Microscopic hematuria disappeared when he was 2 years old. His growth and development was normal. Two off six patient CNS performed renal biopsy with the result showed lesi mesangial focal segmental, nefritis interstitial DD/ Resolving post infection glomerulonephritis DD/ IgA Nephropati, DD/Lupus nephritis.
Learning Points/Discussion : The prognosis of CNS is usually poor and treatment of CNS is difficult. Prolonged survival can be achieved by early aggressive medical management with frequent infusions of albumin, nutritional supplementation, kortikosteroid has been considered the standard treatment for children with CNS.
Keywords: Congenital nephrotic syndrome Spontaneous remission Prolonged survival