EXPORT CITATION

Chapter-51 Congenital Nephrotic Syndrome

BOOK TITLE: Principles and Practice of Pediatric Nephrology

Author
1. Vijayakumar M
2. Nammalwar BR
ISBN
9788180613043
DOI
10.5005/jp/books/11074_51
Edition
1/e
Publishing Year
2004
Pages
4
Author Affiliations
1. Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India, Government Medical College, Manjeri, Kerala, India, Pediatric Kidney Foundation, Chennai, Dr Mehta’s Hospitals Pvt Ltd., Chennai, Government Medical College, Kozhikode, Kerala, India, Mehta Children’s Hospital, Chetpet, Chennai, Tamil Nadu, India, Government Medical College, Kozhikode, Kerela, India, Mehta Children’s Hospital, Chennai, Tamil Nadu, Mehta’s Children Hospital, Chennai, Tamil Nadu, India, Mehta Children’s Hospital, Chennai, Tamil Nadu, India, Dr. Mehta’s Hospital; Chennai Pediatric Kidney Foundation, Chennai, India, Chennai Pediatric Kidney Foundation, Dr Mehta’s Hospitals Pvt. Ltd, Chennai, National Botanical Research Institute, Lucknow-226 001, India, Medical College, Alappey, Kerala, Manipal Institute of Nephrology and Urology, Manipal Hospital, Bangalore, Karnataka, India, Kanchi Kamakoti, Childs Trust Hospital, Chennai
2. Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India, Kanchi Kamakoti CHILDS, Trust Hospital, Chennai, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, India, Institute of Child Health, Hospital for Children; Mehta Children Hospital, Chennai, Tamil Nadu, Mehta Children’s Hospital, Chennai, Tamil Nadu, India, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Mehta's Multispeciality Hospital, Chennai, Tamil Nadu, India, Institute of Child Health, Hospital for Children and Dr Mehta’s Children’s Hospital, Chennai, Tamil Nadu
Chapter keywords

Abstract

Congenital nephrotic syndrome (CNS) is defined as proteinuria leading to clinical symptoms soon after birth up to an arbitary limit of 3 months. Nephrotic syndrome presenting itself after three months but before one year is defined as infantile nephrotic syndrome. This classification is necessary as the underlying histology is different in these two groups. In congenital nephrotic syndrome, Finnish type (CNF) and diffuse mesangial sclerosis (DMS) are more frequent. CNS can be classified into primary and secondary types. Malformation syndromes associated with CNS are also documented. It is an autosomal recessive disease, common in Finnish population. The gene has been localized to chromosome 19q13. 1, which has been named NPHS1. he disease is characterised by severe loss of protein of which 90% is albumin, which begins in utero and is associated with premature delivery, birth weight of 2. 50 kg but birth length within normal range.

Related Books

© 2019 Jaypee Brothers Medical Publishers (P) LTD.   |   All Rights Reserved