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Pediatric primary hyperparathyroidism: Surgical pathology and long-term outcomes in sporadic and familial cases

Published:October 16, 2022DOI:https://doi.org/10.1016/j.amjsurg.2022.10.018

      Highlights

      • Pediatric hyperparathyroidism is frequenyl sporadic.
      • 5% of cases without family history ar attributed to genetic syndromes.
      • Sporadic HPT is due to single adenoma at a similar rate to sporadic adult population (85%).
      • Familila cases have higher recurrence rates with earlier onset, requiring close follow-up ad interventions are often needed.

      Abstract

      Background

      Primary Hyperparathyroidism (PHPT) is rare in pediatric patients. Data regarding surgical outcomes are scarce.

      Methods

      Single-center retrospective review (1994–2020) of patients ≤21 years undergoing surgery for PHPT.

      Results

      66 patients were identified (61% female, 17 ± 3 years). 71% of patients were symptomatic at diagnosis. 32% of patients had known familial syndromes, most commonly MEN-1. 23% of patients without a known mutation had genetic testing, 22% positive. 56% of the total and 19% of the familial cohort underwent focused exploration. Single gland disease was found in 19% of familial vs 85% of sporadic cases, p < 0.00001. Persistence was 9%, all in the sporadic group, p = 0.11. Recurrence was 15%: 38% in the familial vs 2% in the sporadic groups, p=0.0004. Time to recurrence was 59 months (Q1-38, Q3-95), familial 61 vs 124 months sporadic, p=0.001.

      Conclusion

      Pediatric PHPT is frequently sporadic, although 5% of apparent sporadic cases are secondary to syndromes. Familial cases have higher rates of recurrence, requiring closer follow-up.

      Keywords

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