Primary Hyperparathyroidism in Children and Adolescents

Adolescents with primary hyperparathyroidism typically have more severe disease than adults. They presented with higher serum calcium levels, more severe symptoms, more renal stones, abdominal pain, skeletal fragility and a higher incidence of hypercalcemic crisis when compared with adults. Sestamibi scan is less helpful in the adolescents than in adults. Similar to the adults, 86 % of adolescent patients have single gland disease, and 95 % are cured at the first operation. Contrary to popular belief, most adolescents have single gland disease and not hyperplasia associated with a genetic disorder. The incidence of primary hyperparathyroidism in this population may be under appreciated and a lower threshold for ordering a screening serum calcium should be considered.

Primary hyperparathyroidism can also be an autosomal dominant genetic disorder that is typically associated with multigland hyperplasia. Primary hyperparathyroidism is most commonly (57%) the presenting manifestation of MEN type I, but can also be the initial feature of the hyperparathyroidism-jaw tumor syndrome, which is associated with parathyroid carcinoma. Primary hyperparathyroidism is less often a manifestation of MEN type II. Children with asymptomatic, mild hypercalcemia are likely to have familial hypocalciuric hypercalcemia (FHH).

Appropriate management of Primary Hyperparathyroidism in children and adolescents requires distinction between FHH, which generally requires no specific treatment, and other forms of Primary Hyperparathyroidism that are best treated by parathyroidectomy. Spontaneous primary hyperparathyroidism may be approached and managed similarly to adults. Parathyroid surgery, the conventional treatment for adults with symptomatic primary hyperparathyroidism, is recommended for all children with primary hyperparathyroidism.

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