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Titolo:
Oral motor dysfunction and feeding difficulties in nephropathic cystinosis
Autore:
Trauner, DA; Fahmy, RF; Mishler, DA;
Indirizzi:
Univ Calif San Diego, Sch Med, Dept Neurosci, La Jolla, CA 92093 USA Univ Calif San Diego La Jolla CA USA 92093 urosci, La Jolla, CA 92093 USA Univ Calif San Diego, Med Ctr, Dept Occupat Therapy, San Diego, CA 92103 USA Univ Calif San Diego San Diego CA USA 92103 rapy, San Diego, CA 92103 USA
Titolo Testata:
PEDIATRIC NEUROLOGY
fascicolo: 5, volume: 24, anno: 2001,
pagine: 365 - 368
SICI:
0887-8994(200105)24:5<365:OMDAFD>2.0.ZU;2-X
Fonte:
ISI
Lingua:
ENG
Soggetto:
NERVOUS-SYSTEM INVOLVEMENT; CYSTEAMINE; MYOPATHY; CHILDREN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
13
Recensione:
Indirizzi per estratti:
Indirizzo: Trauner, DA Univ Calif San Diego, Sch Med, Dept Neurosci, 9500 Gilman Dr, La Jolla, CA92093 USA Univ Calif San Diego 9500 Gilman Dr La Jolla CA USA 92093 USA
Citazione:
D.A. Trauner et al., "Oral motor dysfunction and feeding difficulties in nephropathic cystinosis", PED NEUROL, 24(5), 2001, pp. 365-368

Abstract

Nephropathic cystinosis is a genetic disorder in which the amino acid cystine accumulates in lysosomes, resulting in multiorgan dysfunction, Progressive neuromuscular dysfunction, with bulbar and upper extremity weakness, has been described in adults with this disorder. The purpose of the present study was to determine whether there was evidence of early bulbar involvement, suggested by feeding difficulties or oral motor dysfunction in these patients, and whether the feeding and oral motor problems were associated withother evidence of neurologic dysfunction, Twenty-two children and adolescents with nephropathic cystinosis were studied. Parents completed questionnaires on feeding history and oral motor problems. Eighteen patients were given an oral motor examination, and 14 received a complete neurologic examination. The majority of children had a history of feeding difficulties. Sevenchildren required a gastrostomy tube. Abnormalities on oral motor examination included hypotonia, abnormal gag reflex, and throaty or congested voice. Abnormalities on neurologic examination included hypotonia, muscle weakness, gross and fine motor dysfunction, and ataxia, The results indicate thatfeeding difficulties and oral motor dysfunction are common in children with cystinosis and appear to correlate with the general degree of neurologic dysfunction. Long-term follow-up is necessary to determine whether the early oral motor problems predict the later development of the progressive myopathy observed in adults with cystinosis, (C) 2001 by Elsevier Science Inc. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 28/03/20 alle ore 13:20:14