title: Treatable renal failure found in non-ambulatory Duchenne muscular dystrophy patients.
abstract: Duchenne muscular dystrophy (DMD) is a progressive muscular disorder in which respiratory and heart failures are the main causes of death. Intensive intervention in respiratory and cardiac function has dramatically improved the prognosis; however, dysfunction in other multiple organs may emerge in the later stages of the disease. We report the case of four non-ambulatory DMD patients who presented with renal failure. Common findings included decreased fluid intake, use of diuretics, and presence of chronic heart failure. The levels of serum cystatin C (CysC), a marker of kidney function unaffected by reduced muscle mass, were elevated in all four patients. In two patients, renal failure improved by increasing fluid intake, and discontinuing or reducing the dose of diuretics. The findings suggest that non-ambulatory DMD patients are at a risk of reduced kidney perfusion, which potentially leads to prerenal failure. Therefore, in DMD patients, dehydration signs and CysC levels should be monitored.
authors: Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
authors: Takahiro
authors: T
authors: Motoki
authors: Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
authors: Yuko
authors: Y
authors: Shimizu-Motohashi
authors: Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan. Electronic address: komakih@ncnp.go.jp.
authors: Hirofumi
authors: H
authors: Komaki
authors: Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
authors: Madoka
authors: M
authors: Mori-Yoshimura
authors: Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
authors: Yasushi
authors: Y
authors: Oya
authors: Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
authors: Eri
authors: E
authors: Takeshita
authors: Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
authors: Akihiko
authors: A
authors: Ishiyama
authors: Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
authors: Takashi
authors: T
authors: Saito
authors: Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
authors: Eiji
authors: E
authors: Nakagawa
authors: Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
authors: Kenji
authors: K
authors: Sugai
authors: Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
authors: Miho
authors: M
authors: Murata
authors: Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
authors: Masayuki
authors: M
authors: Sasaki
mesh_terms: D000293:Adolescent; D000328:Adult; D001806:Blood Urea Nitrogen; D055316:Cystatin C / Q000097:blood; D004326:Drinking; D016189:Dystrophin / Q000235:genetics; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000097:blood / Q000150:complications*; D009154:Mutation; D051437:Renal Insufficiency / Q000097:blood / Q000150:complications* / Q000175:diagnosis* / Q000517:prevention & control; D012307:Risk Factors
chemical_list: D055316:Cystatin C; D016189:Dystrophin
keywords: Chronic renal hypoperfusion; Cystatin C; Duchenne muscular dystrophy; Non-ambulatory; Renal failure