Mahwi Taha O, Rashid Zagros G, Ahmed Shaho F
Internal Medicine Department, College of Medicine, University of.
Shar Hospital, Ministry of Health, Sulaimaniyah City.
Ann Med Surg (Lond). 2023 Jun 8;85(7):3418-3422. doi: 10.1097/MS9.0000000000000947. eCollection 2023 Jul.
Patients with thalassemia major are liable to have complications from blood transfusion and iron excess despite using iron-chelating agents. It is common to have endocrine complications in these patients. Hypogonadism is one of the most common complications in thalassemic patients. Early detection and treatment are essential to restore puberty and prevent complications from hypogonadism.
The authors conducted this cross-sectional study in the Kurdistan Region of Iraq, from 1 July 2022, to 1 December 2022. Eighty patients with β-thalassemia major who were referred to the endocrinology clinic were enrolled. The patients were evaluated sequentially, starting with a history, followed by a clinical examination and laboratory tests related to endocrine disease. Those who met the inclusion criteria were enrolled in the study, while the others were excluded.
Out of 80 patients with major thalassemia who were referred to the endocrinology clinic, 53 (66.3%) were female, and 27 (33.7%) were male, with a mean (SD) age of 24.86±7.66 (14-59) years. Fifty-five (68.75%) of them had hypogonadism, three patients (3.8%) had hypothyroidism, while two (2.5%) had hypoparathyroidism. Five patients (6.3%) had diabetes. None of the patients had adrenal insufficiency. The mean ferritin level in thalassemic patients with hypogonadism and thalassemic patients without hypogonadism was 2326±2625 ng/ml vs. 1220±2625 ng/ml.
To minimize the risk of endocrinopathy in patients with thalassemia major, they should receive blood transfusions regularly and initiate chelating agents early, because the leading cause of endocrinopathy in thalassemic patients is related to the severity of anemia and iron overload.
重型地中海贫血患者即使使用铁螯合剂，也容易出现输血和铁过载并发症。这些患者出现内分泌并发症很常见。性腺功能减退是地中海贫血患者最常见的并发症之一。早期发现和治疗对于恢复青春期和预防性腺功能减退并发症至关重要。
作者于2022年7月1日至2022年12月1日在伊拉克库尔德地区进行了这项横断面研究。纳入了80例转诊至内分泌科门诊的重型β地中海贫血患者。对患者进行了序贯评估，首先是病史采集，然后是临床检查和与内分泌疾病相关的实验室检查。符合纳入标准的患者纳入研究，其他患者排除。
在转诊至内分泌科门诊的80例重型地中海贫血患者中，53例（66.3%）为女性，27例（33.7%）为男性，平均（标准差）年龄为24.86±7.66（14 - 59）岁。其中55例（68.75%）患有性腺功能减退，3例（3.8%）患有甲状腺功能减退，2例（2.5%）患有甲状旁腺功能减退。5例（6.3%）患有糖尿病。无患者患有肾上腺功能不全。患有性腺功能减退的地中海贫血患者和未患有性腺功能减退的地中海贫血患者的平均铁蛋白水平分别为2326±2625 ng/ml和1220±2625 ng/ml。
为了将重型地中海贫血患者内分泌病的风险降至最低，他们应定期接受输血并尽早开始使用螯合剂，因为地中海贫血患者内分泌病的主要原因与贫血严重程度和铁过载有关。
