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The effects of oxybutynin on urinary symptoms in children with Williams-Beuren syndrome.
Sammour, Zein M; Gomes, Cristiano M; de Bessa, Jose; Pinheiro, Marcello S; Kim, Chong A; Honjo, Rachel S; Trigo-Rocha, Flavio E; Bruschini, Homero; Srougi, Miguel.
Afiliação
  • Sammour ZM; Division of Urology and Genetics Unit of the Children's Institute, University of São Paulo School of Medicine, São Paulo, Brazil.
J Urol ; 188(1): 253-7, 2012 Jul.
Article em En | MEDLINE | ID: mdl-22595063
PURPOSE: Williams-Beuren syndrome is a genomic disorder caused by a hemizygous contiguous gene deletion on chromosome 7q11.23. Lower urinary tract symptoms are common in children with Williams-Beuren syndrome. However, there are few data on the management of voiding symptoms in this population. We report our experience using oxybutynin to treat urinary symptoms in children with Williams-Beuren syndrome. MATERIALS AND METHODS: We prospectively analyzed 42 patients with Williams-Beuren syndrome and significant lower urinary tract symptoms due to detrusor overactivity diagnosed on urodynamics in a 12-week, open-label study. Urological assessment included symptomatic evaluation, the impact of lower urinary tract symptoms on quality of life, frequency-volume chart, urodynamics and urinary tract sonography. After 12 weeks of treatment with 0.6 mg/kg oxybutynin per day given in 3 daily doses, patients were assessed for treatment efficacy and side effects. RESULTS: A total of 17 girls and 19 boys completed medical therapy and were assessed at 12 weeks. Mean ± SD patient age was 9.2 ± 4.3 years (range 3 to 18). The most common urinary complaint was urgency, which occurred in 31 patients (86.1%), followed by urge incontinence, which was seen in 29 (80.5%). Compared to baseline, urinary symptoms were substantially improved. The negative impact of storage symptoms on quality of life was significantly decreased from a mean ± SD of 3.3 ± 1.7 to 0.5 ± 0.9 (p <0.001). Mean ± SD maximum urinary flow improved from 14.2 ± 15.0 to 20.5 ± 6.4 ml per second (p <0.001). CONCLUSIONS: A total of 12 weeks of therapy with 0.6 mg/kg oxybutynin daily resulted in improvement of lower urinary tract symptoms, quality of life and maximum flow rate in most patients with Williams-Beuren syndrome.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Transtornos Urinários / Urodinâmica / Antagonistas Muscarínicos / Síndrome de Williams / Ácidos Mandélicos Tipo de estudo: Clinical_trials / Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Aspecto: Patient_preference Limite: Adolescent / Child / Child, preschool / Female / Humans / Male Idioma: En Revista: J Urol Ano de publicação: 2012 Tipo de documento: Article País de afiliação: Brasil País de publicação: Estados Unidos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Transtornos Urinários / Urodinâmica / Antagonistas Muscarínicos / Síndrome de Williams / Ácidos Mandélicos Tipo de estudo: Clinical_trials / Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Aspecto: Patient_preference Limite: Adolescent / Child / Child, preschool / Female / Humans / Male Idioma: En Revista: J Urol Ano de publicação: 2012 Tipo de documento: Article País de afiliação: Brasil País de publicação: Estados Unidos