| In Sook Lee | 2 Articles |
Neuromuscular disorders
Objective
To compare the progression of muscle fibrosis of various site and its relation between cardiac deterioration in Duchenne muscular dystrophy (DMD). In this study aimed to examine the associations between echocardiogram-based cardiac function indices and fibrosis of the abdominal and lower extremity muscles in patients with DMD to facilitate early detection of cardiac dysfunction and identify its predictors. Methods Twenty-one patients with DMD patients were enrolled in the study. The association between cardiac dysfunction and fibrosis of the abdominal and lower extremity muscles was determined by analyzing the echocardiography and elastography. Non-parametric Spearman rank correlation coefficients were used to examine the pairwise relationships between cardiac function and muscle elasticity. Results All patients were male and non-ambulant. Their mean age was 18.45±4.28 years. The strain ratios of the abdominal muscle and quadriceps muscles were significantly higher than those of the medial gastrocnemius. The strain ratio of the rectus abdominis muscle has a significant negative correlation with left ventricular ejection fraction. Cardiac function and valvular insufficiency were not significantly correlated with muscle strain ratio. According to the result of our study, the only skeletal muscle which showed significant correlation with cardiac dysfunction was degree abdominal muscle fibrosis. Conclusion The degree of fibrosis of respiratory muscles was also significantly associated with cardiac dysfunction; therefore, it can be used as a predictor of cardiac dysfunction in patients with DMD in clinical practice.
To investigate the coexistence rate and related factors of developmental dysplasia of the hip (DDH) and congenital muscular torticollis (CMT), and to determine whether ultrasonography (US) gives good value for screening of DDH in CMT. We prospectively examined 121 infants (73 males and 48 females) diagnosed with CMT to determine the incidence of DDH by US. We also assessed the relationship between neck US findings and DDH occurrence, and investigated the clinical features of CMT related to DDH. 18 patients (14.9%) were diagnosed as having DDH by US. However, most DDH was subclinical and spontaneously resolved. Only 2 patients (1.7%) needed to be treated with a harness. The positive predictive value of clinical examinations for DDH was 52.6% and patients treated by harness were all clinically positive. DDH was more common in the left side (13 left, 4 right, 1 both), but 6 out of 18 DDH (33.3%) cases presented on the contralateral side of CMT. Sex difference was not observed. Breech presentation and oligohydramnios were not related to DDH occurrence. Neck US findings did not correlate with DDH occurrence. The coexistence rate of CMT and DDH was concluded to be 14.9%. If only DDH cases that required treatment were included, the coexistence rate of these two disorders would be lowered to 1.7%. All of these patients showed positive findings in clinical examination. Therefore, hip US should not be recommended routinely for patients with CMT. Citations Citations to this article as recorded by
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