Clinical observations of idiopathic hypoparathyroidism / 대한내과학회지

ABSTRACT

BACKGROUND: Idiopathic hypoparathyroidism is a relatively rare disease characterized by hypocalcemia and hyperphosphatemia due to parathyroid hormone deficiency of unknown cause. It usually develop at young age and various clinical symptoms and signs accompanied with hypocalcemia develop at late period. And rarely associated with polyglandular autoimmune syndrome during follow-up. So we reviewed retrospectively our clinical experiences in diagnosis and management. METHODS: We observed the 6 cases with idiopathic hypoparathyroidism from 1986 to 2002. Four female and two male patients were examined demographic characteristics, clinical manifestations, laboratory findings, radiologic finding, and occurrence of polyglandular autoimmune syndrome. RESULTS: All patients aged from 18 to 51 years and had no familial history. The most frequent two symptoms of idiopathic hypoparathyroidism were paresthesia (67%) and tetany (67%). The incidence of Chvostek's and Trousseau's sign were 83% and 67%, respectively. From the history taking, patient who had psychotic problem was two, ocular lesion was two, and Graves's disease was one before admission. One patient had transient hypogonadism because of acute illness but recovered during follow-up. One patient developed primary adrenal insufficiency after 2 years. Neuroimaging study revealed bilateral calcification and ECG showed Q-T interval prolong. CONCLUSION: Idiopathic hypoparathyroidism may be suspected in patients with various neurologic symptoms and signs associated with hypocalcemia. It was difficult to predict other endocrine dysfunction at diagnosis. Therefore, we need to follow-up laboratory examinations for early detection of accompanied autoimmune disease in idiopathic hypoparathyroidism patient.