The purpose of this study is to investigate the abnormality of cough reflex in myotonic dystrophy (MyD) patients. Fourteen MyD patients, with mean age of 54.0 years, and age-matched 14 healthy adults took part in this study. They inspired the nebulized saline solution (control) and citric acid delivered by an ultrasonic nebulizer for 1 minute. Citric acid was dissolved in saline providing incremental concentrations from 5.6mg/ml to 360mg/ml. In this way, participants were promoted to cough. We counted the number of coughs for 2 minutes (1 minute during inspiration, and 1 minute after), and determined the cough threshold as the concentration at which the patient coughed at least 10 times. In the patients group, the thresholds were higher than the control group. We concluded cough reflex was impaired in MyD patients.

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An epidemiological study of developmentally disabled children with motor and mental dysfunctions was conducted in Nagasaki Prefecture. Out of 49, 222 children aged 3 to 6 years old, 79 were found to have severe motor and mental disability. The prevalence rate was 1.60/1000. The etiological factors of their disabilities were as follows: prenatal 39.2%, perinatal 29.1%, postnatal 16.5%, and unknown 10.1%. Both prenatal and perinatal factors were important. In 79 cases, 74 children stayed at home with their families. During our research period of 11 months, 4 of them were dead. The supportive system for such children and their families was suggested to be necessary.

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