Abstract

Congenital Nephrotic Syndrome (CNS) is an autosomal recessive (AR) disorder characterized by massive proteinuria, hypoalbuminemia, and severe edema, starting in the first 3 months of life. Intussusception on the other hand is a surgical condition requiring a leading point in most of the cases. The occurrence of intussusception in congenital nephrotic syndrome may be due to bowel edema as the leading point. In this report, we present monozygotic twins with congenital nephrotic syndrome, who experienced the first episode of intussusception at ten months of age and then had repeated episodes until they were 18 months old. In our patients, mesenteric lymphadenopathy was reported by a radiologist that could serve as a leading point. Upon laboratory examinations, we could not find any explanation for this report. Intussusception must be one of the differential diagnoses of abdominal pain in nephrotic syndrome patients since prompt diagnosis and appropriate treatment can prevent further complications.