Manifestações urológicas na distrofia muscular de Duchenne
USA – a pesquisa foi feita no prontuário de 135 pacientes com Duchenne; 50% apresentaram uma manifestação de doença urológica e 28% múltiplas manifestações. As doenças urológicas foram mais frequentes em adolescentes e adultos jovens e a causa de cirurgia mais frequente foi a presença de cálculos nas vias urinárias.
O resumo em inglês pode ser lido abaixo:
(The Journal of Urology, 2013) Urologic Manifestations in Duchenne Muscular Dystrophy
Eric J. Askeland, Angela M. Arlen, Bradley A. Erickson, Katherine D. Mathews, Christopher S. Cooper – USA
Purpose: Duchenne muscular dystrophy (DMD) is a dystrophinopathy affecting males, with multiple organ system complications. To date, urologic complications of DMD have been described only anecdotally. Materials and Methods: Medical charts of 135 DMD and Duchenne-Becker muscular dystrophy (D/BMD) patients were reviewed for demographics and disease progression, urologic diagnoses, intervention and follow-up. Results: Of 135 patients, 67 (50%) had at least one documented urologic diagnosis and 38 (28%) had multiple manifestations. Presence of lower urinary tract symptoms (LUTS) was the most common urologic diagnosis (32%). Survival analysis revealed a median onset of LUTS of 23 years (95% CI 17.7 – 23.9). Twelve patients (9%) required intervention, most commonly due to nephrolithiasis. Urologic morbidity increased with DMD progression when stratified by markers of clinical progression. LUTS were more common in non-ambulatory patients (40.7% vs. 19%, p = 0.007), those with a diagnosis of scoliosis (44% vs. 19.7%, p = 0.003) and/or scoliosis spine surgery (60% vs. 22%, p <0.001) and on invasive respiratory support (53% vs. 29%, p = 0.046). Likewise, nephrolithiasis was more common in non-ambulatory patients (10% vs. 0%, p = 0.017), those with scoliosis (12% vs. 0%, p = 0.004) and/or scoliosis spine surgery (20% vs. 1%, p <0.001) and those on invasive respiratory support (29% vs. 3%, p < 0.001). Only 28% of patients with a urologic manifestation were referred to urology.
Conclusions: As these patients transition into adolescence and adulthood, the increased prevalence of urologic manifestations warrants increased awareness and referral to urologists.
