Abstract
BackgroundHypoparathyroidism is characterised by hypocalcaemia, and standard management is with an active vitamin D analogue and adequate oral calcium intake (dietary and/or supplements). Little is described in the literature about the impact of intercurrent illnesses on calcium homeostasis in children with hypoparathyroidism.MethodsWe describe three children with hypoparathyroidism in whom intercurrent illnesses led to hypocalcaemia and escalation of treatment with alfacalcidol (1-hydroxycholecalciferol) and calcium supplements.ResultsThree infants managed with standard treatment for hypoparathyroidism (two with homozygous mutations in GCMB2 gene and one with Sanjad-Sakati syndrome) developed symptomatic hypocalcaemia (two infants developed seizures) following respiratory or gastrointestinal illnesses. Substantial increases in alfacalcidol doses (up to three times their pre-illness doses) and calcium supplementation were required to achieve acceptable serum calcium concentrations. However, following resolution of illness, these children developed an increase in serum calcium and hypercalciuria, necessitating rapid reduction to pre-illness dosages of alfacalcidol and oral calcium supplementation.ConclusionIntercurrent illness may precipitate symptomatic hypocalcaemia in children with hypoparathyroidism, necessitating increase in dosages of alfacalcidol and calcium supplements. Close monitoring is required on resolution of the intercurrent illness, with timely reduction of dosages of active analogues of vitamin D and calcium supplements to prevent hypercalcaemia, hypercalciuria and nephrocalcinosis.
Highlights
Hypoparathyroidism (HPT) is the partial or complete reduction of parathyroid hormone (PTH) secretion from the parathyroid glands
The standard treatment for HPT is with active vitamin D (calcitriol (1,25(OH2)D)) or its analogue (alfacalcidol (ACD; 1-hydroxycholecalciferol)) and ensuring adequate http://www.endocrineconnections.org
We have reported three cases of HPT where intercurrent illness required escalation in treatment doses of active analogues of vitamin D and oral Ca supplements to maintain adequate corrected calcium (cCa) levels
Summary
Hypoparathyroidism (HPT) is the partial or complete reduction of parathyroid hormone (PTH) secretion from the parathyroid glands. PTH stimulates 1-alphahydroxylase enzyme to produce 1,25(OH)2-vitamin D, which facilitates active absorption of oral calcium (Ca) from the intestine. PTH mobilises Ca from the bone (by increasing the number and activity of osteoclasts to encourage bone resorption) to maintain serum Ca concentration. PTH in addition stimulates renal Ca reabsorption in the distal nephron and in severe HPT increased urinary Ca excretion may occur when serum corrected calcium (cCa) is at the upper end of the reference range. There is scanty information in the medical literature, which describes the impact that intercurrent illness may have on serum cCa concentration in children with HPT. We describe a case series of three children with genetic causes of HPT in whom intercurrent illnesses led to escalation of treatment with ACD along with Ca supplements. We provide specific advice on management of HPT during intercurrent illnesses
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