title: Congenital myotonic dystrophy with progressive edema and hypoproteinemia.
abstract: We report a patient with congenital myotonic dystrophy who had progressive edema and hypoproteinemia. An atrioseptal defect and patent ductus arteriosus were noted and were considered to be the cause of the right heart failure and edema. Although urinary protein levels were minimal, infusion of albumin did not improve the hypoproteinemia. Administration of dexamethasone increased the serum protein level, but the edema was not ameliorated. Autopsy revealed a slight lymphatic dilation in the small intestine, suggesting protein-losing enteropathy.
authors.affiliation: Department of Pediatrics, Kyoto University, Faculty of Medicine, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Fujii
authors.forename: T
authors.initials: T
authors.lastname: Yorifuji
authors.forename: T
authors.initials: T
authors.lastname: Okuno
authors.forename: S
authors.initials: S
authors.lastname: Toyokuni
authors.forename: S
authors.initials: S
authors.lastname: Okada
authors.forename: H
authors.initials: H
authors.lastname: Mikawa
mesh_terms: D001344:Autopsy; D004487:Edema / Q000150:complications*; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000473:pathology; D006801:Humans; D007019:Hypoproteinemia / Q000150:complications* / Q000473:pathology; D007231:Infant, Newborn; D009223:Myotonic Dystrophy / Q000150:complications* / Q000473:pathology