Sporadic primary hyperparathyroidism in young patients: a separate disease entity?

Arch Surg. 1999 Jun;134(6):651-5; discussion 655-6. doi: 10.1001/archsurg.134.6.651.

Abstract

Hypothesis: Sporadic primary hyperparathyroidism (1 HPT) in young persons is thought to be extremely rare. The exact incidence is unknown and little is known of the characteristics of the disease.

Methods: From 1976 to 1998, 33 patients aged 19 years or younger underwent operation for sporadic 1 HPT at a single institution. Data were recorded regarding the clinical, surgical, pathologic, and biochemical aspects, as well as long-term patient follow-up.

Results: There were 17 male subjects and 16 female subjects ranging in age from 9 to 19 years (median age, 17 years). Thirty-one (94%) were symptomatic: 14 (42%) had renal stones, 9 (27%) had bone disease, 1 (3%) had pancreatitis, and 7 (21%) had vague nonspecific symptoms alone. The high incidence of symptoms was matched by correspondingly high biochemical values (mean serum calcium level, 3.02 mmol/L [12.1 mg/dL]) and large adenomas (mean weight, 967 mg). Five patients (15%) underwent exploration for persistent/recurrent 1 HPT. Thirty-one patients (94%) were normocalcemic postoperatively. One patient was temporarily hypocalcemic. No patient had vocal cord paralysis or paresis. Two patients developed recurrent disease in the mean follow-up period of 10.3 years. None have shown evidence of an inherited disorder.

Conclusions: It appears that 1 HPT in young patients presents as a more severe disease, in terms of symptoms, biochemistry, and extent of pathologic findings. Physicians should be aware that 1 HPT does occur in young persons in a nonfamilial setting and that it may be responsible for a wide spectrum of symptoms. As in the adult population, 1 HPT is safely and effectively treated with surgical intervention.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Child
  • Female
  • Follow-Up Studies
  • Humans
  • Hyperparathyroidism / classification*
  • Hyperparathyroidism / complications
  • Hyperparathyroidism / diagnosis
  • Hyperparathyroidism / surgery
  • Male
  • Remission Induction