title: Spectrum of pediatric developmental and genetic renal lesions and associated congenital malformations--an autopsy study from north India.
abstract: Pediatric developmental and genetic renal lesions are a known cause of mortality in the perinatal/neonatal period. These lesions are associated with a wide range of extrarenal congenital malformations that influence the outcome of the patients. In this autopsy study, we have analyzed the spectrum of pediatric developmental and genetic renal lesions and their associated congenital malformations. A total of 4,099 autopsies (20 weeks of gestation to 1 year of life) were reviewed, of which 158 cases (3.85%) of pediatric developmental (143 cases) and genetic renal lesions (15 cases) were found. Autosomal recessive polycystic kidney disease was the commonest genetic lesion. Primitive ducts with cuffing of immature mesenchyme--the sine qua non of renal dysplasia--was found in all cases of dysplasia. Associated congenital malformations were seen in all cases and thus a thorough search for them is mandatory. Ductal plate malformation was found in all cases of autosomal recessive polycystic kidney disease and in 1 case of bilateral multicystic dysplasia.
authors.affiliation: Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India. nan@glide.net.in
authors.forename: Nandita
authors.initials: N
authors.lastname: Kakkar
authors.forename: Santosh
authors.initials: S
authors.lastname: Menon
authors.forename: B D
authors.initials: BD
authors.lastname: Radotra
mesh_terms: D001344:Autopsy; D018376:Cardiovascular Abnormalities / Q000235:genetics / Q000473:pathology; D000013:Congenital Abnormalities / Q000453:epidemiology / Q000235:genetics* / Q000473:pathology*; D004065:Digestive System Abnormalities / Q000235:genetics / Q000473:pathology; D005260:Female; D006801:Humans; D015994:Incidence; D007194:India / Q000453:epidemiology; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000002:abnormalities / Q000196:embryology / Q000473:pathology; D007674:Kidney Diseases / Q000453:epidemiology / Q000235:genetics* / Q000473:pathology*; D008297:Male; D021782:Multicystic Dysplastic Kidney / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D009139:Musculoskeletal Abnormalities / Q000235:genetics / Q000473:pathology; D009421:Nervous System Malformations / Q000235:genetics / Q000473:pathology; D017044:Polycystic Kidney, Autosomal Recessive / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D015619:Respiratory System Abnormalities / Q000235:genetics / Q000473:pathology; D012189:Retrospective Studies; D014564:Urogenital Abnormalities / Q000235:genetics / Q000473:pathology