[Clinical characteristics of Wolfram syndrome].
Zhongguo Dang Dai Er Ke Za Zhi
; 11(2): 113-5, 2009 Feb.
Article
em Zh
| MEDLINE
| ID: mdl-19222947
ABSTRACT
OBJECTIVE:
Wolfram syndrome (WFS) is a rare, autosomal recessive inherited disease characterized by various clinical manifestations. The aim of this study was to investigate clinical characteristics of WFS.METHODS:
One case of WFS was reported. Combined with the clinical data of 8 cases of WFS which had been reported in China between 1994 and 2007, the clinical characteristics of WFS were reviewed.RESULTS:
Insulin-dependent diabetes mellitus as the earliest manifestation was found in all of the 9 patients, with a median onset age of 5.0 years. Optic atrophy occurred in 8 patients (onset age 8.5 years), diabetes insipidus in 7 patients (onset age 8.5 years) and deafness in 7 patients (onset age 9.8 years). Short stature was found in 6 patients and hydroureteronephrosis in 4 patients.CONCLUSIONS:
Insulin-dependent diabetes mellitus was the first presentation in children with WFS. Optic atrophy, diabetes insipidus and deafness were common complications, with a various onset age.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndrome de Wolfram
Tipo de estudo:
Diagnostic_studies
Limite:
Child
/
Humans
/
Male
Idioma:
Zh
Revista:
Zhongguo Dang Dai Er Ke Za Zhi
Ano de publicação:
2009
Tipo de documento:
Article
País de afiliação:
China