Hypoparathyroidism with extensive intracerebral calcification in patients with beta-thalassemia major.

We report marked intracerebral calcification in eight thalassemic patients with hypoparathyroidism, followed regularly at the Haematology Research Center, Department of Pediatrics, Shiraz, Iran. Their mean age was 16.8 years (range 12-21 years). Six of the eight patients with thalassemia were females. The daily dose of calcitriol was between 0.01 and 0.1 microg/kg b. wt. Calcium-phosphate metabolic control was good or satisfactory in all patients. Three patients had at least one episode of generalized convulsions caused by hypocalcemia, before and during treatment. One patient complained of chronic headache and another patient had a low intelligence quotient. All were on treatment with calcitriol and oral calcium supplementation. The mean serum ferritin concentration was 3225 microg/l (range 2000-6000 microg/l). Calcification was present in the cerebral hemispheres, thalamic nuclei, basal ganglia, the internal capsule, part of the caudate nuclei and the posterior fossa. There was no history of birth asphyxia, head trauma, infections or metabolic diseases in any of the patients. No relationship was observed between the degree of cerebral calcification and the severity of hypoparathyroidism at diagnosis. Our observations stress the importance of a periodic assessment of calcium metabolism, prompt treatment of the endocrinopathy and strict control of calcium metabolism.