INTRODUCTION: Neonatal severe hyperparathyroidism (NSHPT) is a rare disease that presents early with severe hypercalcemia. We reviewed our experience with NSHPT management at a tertiary-care institution. METHODS: A retrospective chart review was conducted for patients managed for NSHPT over the last 10 years. Demographic, clinical, and follow-up data were collected, and descriptive data were generated. RESULTS: Five patients were managed for NSHPT, 3 females and 2 males, with a mean age of 18 days at presentation. The mean preoperative parathyroid hormone and calcium levels were 3607 ng/L and 3.84 mmol/L, respectively. Preoperative parathyroid gland localization tests were all negative. All patients had a total parathyroidectomy and autotransplantation at a mean age of 65 days, with a mean follow-up of 5.5 years. One patient had normal parathyroid hormone and normal calcium levels 9.5 years after surgery without medication. One patient had normal levels without medication for 2 years then needed calcium and vitamin D supplements thereafter (8.5 years postoperatively). Three patients are still on calcium and vitamin D supplementation 5.5 years, 3.5 years, and 8 months, respectively, after surgery. CONCLUSIONS: Neonatal severe hyperparathyroidism is managed effectively with total parathyroidectomy. Preoperative localization studies are not helpful, and autotransplantation has a modest effect in our experience. Copyright 2010 Elsevier Inc. All rights reserved.
INTRODUCTION:Neonatal severe hyperparathyroidism (NSHPT) is a rare disease that presents early with severe hypercalcemia. We reviewed our experience with NSHPT management at a tertiary-care institution. METHODS: A retrospective chart review was conducted for patients managed for NSHPT over the last 10 years. Demographic, clinical, and follow-up data were collected, and descriptive data were generated. RESULTS: Five patients were managed for NSHPT, 3 females and 2 males, with a mean age of 18 days at presentation. The mean preoperative parathyroid hormone and calcium levels were 3607 ng/L and 3.84 mmol/L, respectively. Preoperative parathyroid gland localization tests were all negative. All patients had a total parathyroidectomy and autotransplantation at a mean age of 65 days, with a mean follow-up of 5.5 years. One patient had normal parathyroid hormone and normal calcium levels 9.5 years after surgery without medication. One patient had normal levels without medication for 2 years then needed calcium and vitamin D supplements thereafter (8.5 years postoperatively). Three patients are still on calcium and vitamin D supplementation 5.5 years, 3.5 years, and 8 months, respectively, after surgery. CONCLUSIONS:Neonatal severe hyperparathyroidism is managed effectively with total parathyroidectomy. Preoperative localization studies are not helpful, and autotransplantation has a modest effect in our experience. Copyright 2010 Elsevier Inc. All rights reserved.