RESUMO
Myotonia congenita is a rare heritable disease of skeletal muscles characterized by muscle stiffness (myotonia) and hypertrophy. We report a child with features of autosomal dominant type of myotonia congenita seen at our hospital. A 4-year-old boy admitted for an acute febrile illness was observed to have generalized muscular hypertrophy said to be present since he was 7 months old. It was associated with painless generalized muscle stiffness and slow initiation of movements after period of rest which gradually subsided following a few minutes of activity. He also had history of frequent falls while running, but no difficulty climbing stairs or muscle weakness. Pregnancy and delivery were uneventful. He had normal developmental milestones and was doing well in school. No history of similar problem in any family member and his parents were not consanguineous. On examination he had generalized muscular hypertrophy with a Herculean build. He had demonstrable stiff movements and gait after rest with percussion myotonia. Gower sign was negative. Serum creatinine kinase was normal at 79.2 U/L (Up to 130 U/L) and CK-MB was 24.8 U/L (Up to 24 U/l)). Electromyography (EMG) showed myotonia as sharp waves in a waxing and waning pattern. Muscle biopsy and genetic test were not done due to non-availability. A diagnosis of myotonia congenita most likely autosomal dominant type (Thomsen disease) was made based on strongly suggestive clinical features and EMG in the patient. He was since discharged as acute febrile illness resolved but still has symptoms of myotonia. This is the first reported case of myotonia congenita in our environment supported by clinical and EMG features. Genetic diagnosis should be made available in resource limited settings.
RESUMO
Background: Reports have shown significant increase in the prevalence of obesity in developing countries. There are limited representative data available from African countries on the trends in childhood obesity; because most public health- and nutrition-related efforts have been focused on under-nutrition. Aims: To determine the prevalence of overweight and obesity using body mass index (BMI) among school adolescents. Design: A cross sectional study involving apparently healthy school students in Sokoto. Materials and Methods: Participating schools and students were selected by a multi-stage random sampling. Anthropometric measurements were done and body mass index derived from the ratio of weight per height squared. Subjects with age and sex-adjusted BMIs of 85 th to 95 th and ?95 th percentiles of the International Obesity Task Force were defined as overweight and obese; respectively. Statistical Analysis: Data were analyzed using SPSS version 17.0 software. GraphPad inStat version 3.05 was also used to analyze some variables. Results were expressed as means with standard deviations. P-values 5 were considered significant. Results: The mean BMI of the male subjects was 18.3 ? 2.7kg/m 2 ; and 19.3 ? 3.1kg/m 2 for the females. The prevalence of overweight was 3.3; and that of obesity was 1.4. Conclusions: The prevalence of overweight and obesity appear to be low in the study area; especially when compared to some recent studies in Africa