Background: Hypoparathyroidism is a rare disease characterized by inadequate parathyroid hormone (PTH), resulting in hypocalcemia and hyperphosphatemia. Hypoparathyroidism can be transient, inherited, or acquired, and is caused by inability to synthesize or secrete PTH, abnormal parathyroid gland development, destruction of parathyroid tissue, or peripheral resistance to PTH.
Methods: Medical records from 20 children and adolescents diagnosed with hypoparathyroidism during 19922015 were reviewed.
Results: Twenty patients (5 males, 15 females) diagnosed at the age of 8.5±6 years (0.1216.9 years) were evaluated. HP developed after surgery in 4 (primary hiperparathyroidism; 2, thyroid cancer; 1, graves disease; 1) of the patients. Six of the patients were diagnosed as isolated HP, 3 as pseudohypoparathroidism, 4 as polyglandular autoimmune syndrome, 1 as Di-George syndrome, 1 as HDR syndrome and 1 as HP secondary to hemosiderosis due diamond blackfan anemia. Sixteen of the patients admitted because of convulsions and tetany. One patient was diagnosed incidentally. The patient with hemosiderosis and the patient with polyglandular autoimmune syndrome were diagnosed during follow-up. Patient with HDR syndrome was diagnosed as HP during an evaluation of psoriasis. 8 patients had calcifications in cranial CT and 2 patients had cataract at the initial diagnosis. All of the patients received calcium and 1,25 OH vitamin D initially. During follow-up two patients developed nephrolithiasis and three patients nephrocalcinosis. Due to hypercalciuria hydrochlorthiazide was initiated in two patients. Recombinant human PTH was initiated to the three patients with nephrocalcinosis but was seized because the dosing problems. Chronic treatment of hypoparathyroidism may be difficult to manage due to the need for a sensitive balance between calcium and phosphate levels in order to prevent nephrolithiasis, nephrocalcinosis, and soft tissue calcification in the kidney.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology