Short stature in Duchenne muscular dystrophy.
نویسندگان
چکیده
Growth rate was assessed in 187 boys affected with Duchenne muscular dystrophy (DMD) and bone age in 127 among them. Patients' heights were compared with their normal sibs, with expected stature according to midparents' stature and with normal Brazilian children of comparable age. The results showed that DMD boys have a normal length and weight at birth, but a delayed growth starting during the first years of life with a mean approximate decrease of -6.00 cm according to the expected height of the normal Brazilian population. The proportion of DMD patients with short stature (below the 3rd centile) was similar in younger boys (up to 7 years) to that in the older group (more than 7 years) of affected boys. Bone maturation in DMD boys did not differ from their normal sibs. Weight was also decreased in DMD patients as compared with normal sibs of comparable age.
منابع مشابه
P164: Adeno-Associated Viral Vectors in Duchenne Muscular Dystrophy
Duchenne muscular dystrophy (BMD) is an inherited X-link disease. The incidence of this muscle-wasting disease is 1:5000 male live births. Mutation in the gene coding for dystrophin is the main cause of BMD. Most cases of this disease succumb to respiratory and cardiac failure in 3rd to 4th decades. The slow progression of BMD and recent achievement of gene therapies make it as an appropriate c...
متن کاملDetection of the Duplication in Exons 56-63 of Duchenne Muscular Dystrophy Patients with MLPA
Background Duchenne Muscular Dystrophy (DMD) is a deadly X-linked recessive disorder. This genetic disorder affects 1 among 3,500-5,000 males in the world. The majority of the patients are male, due to the type of inheritance. It affects most of the skeletal, the respiratory, and cardiac muscles, causing these vital organs to contract and eventually mortality.<br...
متن کاملCorticosteroid Treatment and Growth Patterns in Ambulatory Males with Duchenne Muscular Dystrophy.
OBJECTIVES To evaluate growth patterns of ambulatory males with Duchenne muscular dystrophy (DMD) treated with corticosteroids compared with ambulatory, steroid-naïve males with DMD and age-matched unaffected general-population males and to test associations between growth and steroid treatment patterns among treated males. STUDY DESIGN Using data from the Muscular Dystrophy Surveillance, Tra...
متن کاملسندرم شوارتز جمپل (میوتونی کندرودیستروفی) و معرفی دو مورد آن از مرکز طبی کودکان
S-J syndrome is a rare autosomal recessive disorder of short stature, blepharophimosis, puckerd mouth, myotonia, muscular weakness, pectus carinatum and stiff joints. Case report: first case: n.1. A boy aged 7 years. His main abnormalities included puckered lip, blephharophimosis, muscular hypertrophy and weakness, pectus carinatum, short stature, shoulder joint limitation and myopia. Myotonic ...
متن کاملScreening the newborn for Duchenne muscular dystrophy: parents' views.
A short report is provided of an interview survey of 69 parents of boys suffering from Duchenne muscular dystrophy to determine their views of neonatal screening and their experiences at the time of diagnosis. Most of the parents favored screening in the neonatal period or in early infancy, and most of them expressed dissatisfaction with present delays, methods of disclosure, and subsequent su...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Growth regulation
دوره 1 1 شماره
صفحات -
تاریخ انتشار 1991