Navegando por Palavras-chave "Muscular dystrophy, Duchenne"

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    Distrofia muscular de Duchenne: análise eletrocardiográfica de 131 pacientes
    (Sociedade Brasileira de Cardiologia - SBC, 2010-05-01) Santos, Maria Auxiliadora Bonfim [UNIFESP]; Costa, Francisco de Assis [UNIFESP]; Travessa, Aline Ferreira [UNIFESP]; Bombig, Maria Teresa Nogueira [UNIFESP]; Fonseca, Francisco Antonio Helfenstein [UNIFESP]; Luna Filho, Bráulio [UNIFESP]; Mussi, Aurélia [UNIFESP]; Souza, Dilma de [UNIFESP]; Oliveira, Acary Souza Bulle [UNIFESP]; Póvoa, Rui Manuel dos Santos [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)
    BACKGROUND: Cardiac involvement is known to occur in patients with Duchenne muscular dystrophy (DMD). The electrocardiogram (ECG) shows some typical changes in DMD, which makes it a useful test for the diagnosis of cardiac lesion in this disease. OBJECTIVE: To evaluate the electrocardiographic changes in patients with DMD and to correlate these changes with the age of the population studied. METHODS: ECG of 131 patients diagnosed with DMD were examined. Several electrocardiographic variables were analyzed, and the patients were divided into two groups - one with and one without changes, for each variable studied. The correlation between the two groups and the age of the patients was analyzed. Garson's criteria were used to establish the electrocardiographic parameters of normality. RESULTS: ECG was abnormal in 78.6% of the patients. All showed normal sinus rhythm. The following percentages were found for the main variables studied: short PR interval = 18.3%; abnormal R waves in V1 = 29.7%; abnormal Q waves in V6 = 21.3%; abnormal ventricular repolarization = 54.9%; abnormal QS waves in inferior and/or upper lateral wall = 37.4%; conduction disturbances in right bundle branch = 55.7%; prolonged QT C interval = 35.8%, and wide QRS = 23.6%. Unpaired t test was used to establish the correlation between age and the electrocardiographic variables studied in the two groups. Statistically significant differences were found only for the abnormal repolarization variable. CONCLUSION: Electrocardiographic abnormalities are common in DMD, revealing early cardiac involvement. Only the abnormal ventricular repolarization variable was more frequent, however at a lower age range (p < 0.05).
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    Empilhamento de ar e compressão torácica aumentam o pico de fluxo da tosse em pacientes com distrofia muscular de Duchenne
    (Sociedade Brasileira de Pneumologia e Tisiologia, 2009-10-01) Brito, Magneide Fernandes [UNIFESP]; Moreira, Gustavo Antonio [UNIFESP]; Pradella-Hallinan, Márcia Lurdes de Cássia [UNIFESP]; Tufik, Sergio [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)
    OBJECTIVE: To evaluate cough efficiency using two manually-assisted cough techniques. METHODS: We selected 28 patients with Duchenne muscular dystrophy. The patients were receiving noninvasive nocturnal ventilatory support and presented FVC values < 60% of predicted. Peak cough flow (PCF) was measured, with the patient seated, at four time points: at baseline, during a spontaneous maximal expiratory effort (MEE); during an MEE while receiving chest compression; during an MEE after air stacking with a manual resuscitation bag; and during an MEE with air stacking and compression (combined technique). The last three measurements were conducted in random order. The results were compared using Pearson's correlation test and ANOVA with repeated measures, followed by Tukey's post-hoc test (p < 0.05). RESULTS: The mean age of the patients was 20 ± 4 years, and the mean FVC was 29 ± 12%. Mean PCF at baseline, with chest compression, after air stacking and with the use of the combined technique was 171 ± 67, 231 ± 81, 225 ± 80, and 292 ± 86 L/min, respectively. The results obtained with the use of the combined technique were significantly better than were those obtained with the use of either technique alone (F[3.69] = 67.07; p < 0.001). CONCLUSIONS: Both chest compression and air stacking techniques were efficient in increasing PCF. However, the combination of these two techniques had a significant additional effect (p < 0.0001).