Primary hyperparathyroidism in children and adolescents

Abstract

Although primary hyperparathyroidism (PHPT) has emerged as a common endocrine disorder in adults, it is still a rare condition in infants and children with an incidence estimated at only 2–5 per 100,000. It is most commonly caused by a single adenoma, but multiple-gland disease can also occur especially in the setting of a familial disease or syndrome. Sporadic PHPT in children is associated with greater morbidity and end-organ damage at the time of diagnosis compared to adults. The most commonly affected organs are the kidney and bone. A greater degree of hypercalcemia and hypercalciuria at presentation compared to adults is present despite similar parathyroid hormone concentrations, suggesting greater target-organ sensitivity and an apparent decrease in the sensitivity of the parathyroid adenoma to negative feedback by calcium in young patients. Surgery is the mainstay of treatment in pediatric patients, preferably performed by a high-volume surgeon with experience in pediatric endocrine surgery.