After a median period of 54 months (range 36–88 months) follow-up, five patients were alive at a
14, 17, 18 This syndrome is an autosomal recessive disorder that affects 1/8200 Finnish neonates and is caused by a mutation in the NPHS1 gene, which codes for a podocytic slit-diaphragm protein (nephrin)
Swelling around the eyes is the most common sign of nephrotic syndrome in children
Congenital nephrotic syndrome is an autosomal recessive genetic disorder
Congenital nephrotic syndrome (CNS) is a rare and serious entity of renal diseases diagnosed in infants younger than three months
First described by Gautier and Miville in 1942, congenital nephrotic syndrome (CNS) is defined as the triad of nephrotic range proteinuria (>200mg/mmol creatinine), hypoalbuminaemia and clinically detectable edema, occurring in the first three months of life
Hence, Captopril and Indomethacin were added
The features of congenital nephrotic syndrome are caused by Congenital nephrotic syndrome is present at birth or appears during the first three months of life and infantile nephrotic syndrome during the first year
J Pediatr
What is congenital nephrotic syndrome? Congenital nephrotic syndrome, an inherited disorder characterized by protein in the urine and swelling of the body, occurs primarily in families of Finnish origin and develops shortly after birth
Children with congenital nephrotic syndrome begin to have symptoms of the condition between birth and 3 months
The triad of this syndrome is proteinuria, He was discharged home with indomethacin-captopril in escalating doses according to the clinical response; levothyroxine, alfacalcidol, and iron supplements were Background: There is a paucity of information on epidemiology, diagnosis, and treatment outcomes of congenital nephrotic syndrome (CNS) in developing countries
with captopril and indomethacin
Nephrotic syndrome (NS) is a clinical syndrome defined by massive proteinuria responsible for hypoalbuminemia, with resulting hyperlipidemia, edema, and various complications