先天性副腎皮質過形成と軟口蓋裂の重複奇形の1例

概要

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A soft cleft palate was complicated with congenital adrenal hyperplasia of saltlosing type in a 2-year-old boy. Specific surgical care was needed in performing the palatoplasty in fear of electrolyte disturbance and shock which were prone to occur in this particular disease as a result of surgical intervention, postoperative dehydration and fever. The clinical manifestation of the adrenal hyperplasia was reviewed. Deficiency of a kind of enzymes assisting the biosynthesis of corticosteroids in the adrenal cortex is a determining factor of the variety of clinical findings of the congenital adrenal hyperplasia. Prevention of electrolytic disorder associated with surgery was described. Within the scope of our review of the literature, there has been no case of congenital adrenal hyperplasia complicated with cleft palate.