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Hypoparathyroidism with extensive intracerebral calcification in patients with beta-thalassemia major
Mehran Karimi1, Farrokh Habibzadeh, Vincenzo De Sanctis
1Haematology Research Center, Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, Iran.
Insights
Thalassemic patients with hypoparathyroidism frequently develop intracerebral calcification despite good calcium-phosphate control. Regular monitoring of calcium metabolism is crucial for managing this condition.
Area of Science:
- Neurology
- Endocrinology
- Hematology
Background:
- Thalassemia is a genetic blood disorder.
- Hypoparathyroidism is a condition characterized by insufficient parathyroid hormone production.
- Intracerebral calcification can occur in various neurological and metabolic disorders.
Purpose of the Study:
- To investigate the occurrence and characteristics of intracerebral calcification in pediatric patients with thalassemia and hypoparathyroidism.
- To assess the relationship between calcification, hypoparathyroidism severity, and treatment with calcitriol and calcium.
Main Methods:
- Retrospective analysis of eight pediatric patients with thalassemia and hypoparathyroidism.
- Evaluation of clinical data, including neurological symptoms, laboratory results (serum ferritin, calcium-phosphate metabolism), and treatment regimens.
- Radiological assessment for intracerebral calcification.
Main Results:
- Marked intracerebral calcification was observed in all eight patients, affecting various brain regions.
- Despite satisfactory calcium-phosphate control with calcitriol and calcium supplementation, three patients experienced hypocalcemic seizures.
- Elevated serum ferritin levels were noted, consistent with thalassemia.
- No correlation was found between calcification severity and hypoparathyroidism at diagnosis.
Conclusions:
- Intracerebral calcification is a significant complication in thalassemic patients with hypoparathyroidism.
- Periodic assessment and strict management of calcium metabolism are essential.
- Prompt treatment of hypoparathyroidism and ongoing monitoring are critical to prevent neurological complications.
Abstract:
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.
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