Effect of clonidine on the height of a child with glycogen storage disease type VI: A 13 year follow-up study

Abstract

A 9-month-old male was found to have hepatomegaly when he was treated by his doctor for bronchitis. At the age of 2 years and 3 months, glycogen storage disease (GSD) of type VI (GSD VI) was diagnosed in this patient. Despite the recommended diet therapy, his growth was not good, changing under or along the line of −2.0 SD. At the age of 6 years, oral clonidine therapy (0.15 mg/day, 0.2 mg/m2 body surface per day) was started. Six to 10 months after the initiation of clonidine therapy, his height began to increase more than the values for −2.0 SD and once reached the value for −1.0 SD at the age of 10 years. His growth rate and bone age increased. Clonidine therapy was continued regularly for 7 years until the age of 13 years, 11 months. At that time his development was normal and his height reached 150.8 cm (–1.34 SD). However, cessation of the treatment at the patient’s free will resulted in a reduction of the growth rate at age 15 years 6 months. These observations suggest the effect of clonidine therapy on height. Side effects were not noted during the clonidine therapy. Other clinical and laboratory findings of GSD VI also completely improved during treatment. In conclusion, administration of clonidine could be another treatment modality in children with GSD, not only of type VI but also I and III. © 1996 Japan Pediatric Society.