en POLSKI
eISSN: 2083-8441
ISSN: 2081-237X
Pediatric Endocrinology Diabetes and Metabolism
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SCImago Journal & Country Rank
3/2022
vol. 28
 
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abstract:
Original paper

The spectrum of manifestations of primary hyperparathyroidism in children and adolescents

Hiya Boro
1
,
Saurav Khatiwada
1
,
Sarah Alam
1
,
Suraj Kubihal
1
,
Vinay Dogra
1
,
Sundeep Malla
2
,
Chitresh Kumar
3

  1. Department of Endocrinology and Metabolism, All India Institute of Medical Sciences (AIIMS), New Delhi, India
  2. Department of Radiodiagnosis, All India Institute of Medical Sciences (AIIMS), New Delhi, India
  3. Department of Surgical Oncology, All India Institute of Medical Sciences (AIIMS), New Delhi, India
Pediatr Endocrinol Diabetes Metab 2022; 28 (3): 178–187
Online publish date: 2022/08/02
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Introduction
Primary hyperparathyroidism (PHPT) is a rare disease in children and adolescents. Early recognition of this disease is important to prevent significant morbidity and mortality.

Material and methods
We included 10 consecutive patients with PHPT aged 14 to 19 years of age and followed-up prospectively upto one year after parathyroidectomy.

Results
Our cohort included 6 females and 4 males. The mean age of the patients was 16.7 ±1.8 years. The symptoms at presentation were musculoskeletal pain (90%), bone deformity (50%), fracture (30%), proximal myopathy (40%), renal stones (50%), reflux symptoms (40%), and pancreatitis (30%). The mean serum calcium was 3.1 ±0.5 mmol/l, mean serum inorganic phosphorus was 0.9 ±0.3 mmol/l and median serum alkaline phosphatase (ALP) was 1911.5 IU/l (IQR: 522.7–5702.3). The median serum intact parathyroid hormone was 133.5 pmol/l (IQR: 69.5 –178.7) while serum 25(OH)D was 47.7 nmol/l (IQR: 23.7–72.7). Hypercalciuria was observed in 7 patients. Hungry bone syndrome was observed in 4 (40%) patients after surgery. Typical parathyroid adenoma was found in 9 (90%) patients while one patient had atypical adenoma with high mitotic index. After one year of surgery, all patients had significant improvement in clinical and biochemical parameters with persistence of residual bone deformities.

Conclusions
Our study showed the spectrum of manifestations of PHPT in children and adolescents and outcomes of parathyroidectomy till one year. Long-term follow-up studies with bigger cohorts are required to understand the true nature of the disease in children and adolescents.

keywords:

primary hyperparathyroidism, parathyroid adenoma, rickets, renal stones, vitamin D deficiency


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