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依赖输血的β地中海贫血患者的甲状旁腺功能减退症

Hypoparathyroidism in transfusion-dependent patients with beta-thalassemia.

作者信息

Chern Jimmy P S, Lin Kai-Hsin

机构信息

Department of Family Medicine, Tao-Yuan Hospital, Department of Health, Tao-Yuan, Republic of China.

出版信息

J Pediatr Hematol Oncol. 2002 May;24(4):291-3. doi: 10.1097/00043426-200205000-00014.

DOI:10.1097/00043426-200205000-00014
PMID:11972098
Abstract

PURPOSE

To determine the prevalence of hypoparathyroidism in transfusion-dependent patients with beta-thalassemia.

PATIENTS AND METHODS

A total of 28 transfusion-dependent patients with beta-thalassemia were interviewed, and their serum calcium, phosphate, magnesium, and intact parathyroid hormone levels were checked. Serum ferritin levels were measured to monitor the effect of chelation therapy. Blood urea nitrogen, creatinine, total protein, and albumin were measured in patients with undetectable or low intact parathyroid hormone levels.

RESULTS

The prevalence of hypoparathyroidism was 10.7% (3/28). Mean age at diagnosis was 18 years. The serum ferritin levels of patients with hypoparathyroidism were 1,032, 2,102, and 7,680 microg/L. Only one patient had clinical symptoms of hypocalcemia. All three of the patients with hypoparathyroidism had hypogonadism, and 66.7% (2/3) of the patients had insulin-dependent diabetes mellitus.

CONCLUSIONS

Hypoparathyroidism in transfusion-dependent patients with beta-thalassemia seems to be accompanied by other endocrinopathies. Serum ferritin may not have been a reliable indicator of iron overload in the three patients with hypoparathyroidism. Severe iron overload would easily explain these multiple endocrinopathies. This pattern is commonly seen in iron-overloaded patients with thalassemia elsewhere.

摘要

目的

确定依赖输血的β地中海贫血患者中甲状旁腺功能减退症的患病率。

患者与方法

对28例依赖输血的β地中海贫血患者进行访谈,并检测其血清钙、磷、镁和完整甲状旁腺激素水平。测量血清铁蛋白水平以监测螯合疗法的效果。对甲状旁腺激素水平检测不到或较低的患者测量血尿素氮、肌酐、总蛋白和白蛋白。

结果

甲状旁腺功能减退症的患病率为10.7%(3/28)。诊断时的平均年龄为18岁。甲状旁腺功能减退症患者的血清铁蛋白水平分别为1032、2102和7680μg/L。只有1例患者有低钙血症的临床症状。所有3例甲状旁腺功能减退症患者均有性腺功能减退,66.7%(2/3)的患者患有胰岛素依赖型糖尿病。

结论

依赖输血的β地中海贫血患者的甲状旁腺功能减退症似乎伴有其他内分泌疾病。血清铁蛋白可能不是3例甲状旁腺功能减退症患者铁过载的可靠指标。严重的铁过载很容易解释这些多种内分泌疾病。这种模式在其他地方的铁过载地中海贫血患者中很常见。

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