Type 2 congenital pulmonary airway malformation and congenital nephrotic syndrome: report of a new association.

Abstract

Type 2 congenital pulmonary airway malformation (CPAM) has been reported in association with many other congenital anomalies. To the best of our knowledge, however, an association of type 2 CPAM with congenital nephrotic syndrome has not been heretofore reported. We present the 1st report of such an association in a boy who had a prenatal diagnosis of cystic lung malformation and was found to have congenital nephrotic syndrome (diffuse mesangial sclerosis) at 1 month of age. A prenatal ultrasonogram had also shown oligohydramnios, and additionally the child had cleft lip and palate. There was no family history of childhood renal or pulmonary disease, and genetic testing for genes mutated in congenital nephrotic syndrome was negative.

• Authors: Karmaine A Millington, Haresh Mani
• Journal: Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society (Pediatr Dev Pathol) Vol. 16 Issue 3 Pg. 210-3 ( 2013) ISSN: 1093-5266 [Print] United States
• PMID: 23350653 (Publication Type: Case Reports, Journal Article)

Topics

• Cleft Lip (complications)
• Cystic Adenomatoid Malformation of Lung, Congenital (complications, pathology)
• Humans
• Infant
• Infant, Newborn
• Kidney (abnormalities)
• Male
• Nephrotic Syndrome (complications, congenital, pathology)