ESPE Yearbook of Paediatric Endocrinology

Abstract: N Engl J Med. 2019 May 2;380(18):1738–1747.

In brief: This article carefully reviews available data and provide useful hands-on tips for the management of hypoparathyroidsm. It also points out areas where additional data are needed. This is mandatory reading for any aspiring endocrinologist.

Comment: Hypoparathyroidism is a rare disease resulting in hypocalcemia, which can be asymptomatic or result in symptoms ranging from paresthesia and muscle cramps to seizures and life-threatening laryngospasm. In adults, the most common cause of hypoparathyroidism is injury to or removal of the parathyroid gland during anterior neck surgery. In children, genetic and autoimmune causes are more common and may be isolated or part of a syndrome, e.g. 22q11 deletion or Autoimmune polyendocrine syndrome type 1. The goal of treatment is to maintain the blood calcium level near to the low end of the normal range, while preventing symptoms of hypocalcemia. This is usually achieved with oral calcium and active vitamin D (calcitriol or alfacalcidol) therapy, but may involve treatment with subcutaneous parathyroid hormone therapy. Treatment is commonly associated with side effects of hypercalciuria, nephrocalcinosis, nephrolithiasis, and renal insufficiency, emphasizing the need for careful monitoring and better future therapies.