Verma Ganesh, Chand Ramesh, Anjum Meraz, Rastogi Dushyant, Sharma Nishant, Verma Swalpa, Khan Imran Ahmed
Pediatrics, Uttar Pradesh University of Medical Sciences, Saifai, IND.
Dentistry, Community Health Centre, Khairagarh, IND.
Cureus. 2024 Nov 30;16(11):e74885. doi: 10.7759/cureus.74885. eCollection 2024 Nov.
Background Thalassemia is the most common form of hereditary anemia caused by the impaired synthesis of one of the two globin chains in hemoglobin. A decrease in beta-globin chains occurs in beta-thalassemia, resulting in a relative excess of alpha-globin chains. Thalassemia major is the severe form of thalassemia, which requires frequent blood transfusions for survival. Consequently, the natural course of the disease is affected by blood transfusion-related side effects. Repeated blood transfusions lead to the accumulation of iron in tissues such as the liver, heart, and endocrine glands. Serum ferritin is a biomarker of iron overload. Endocrinopathies are among the most frequently observed complications in thalassemia. Early recognition and treatment are important in order to prevent late irreversible sequelae and improve the quality of life of these patients. This study was conducted to evaluate growth parameters and endocrine function in children with thalassemia major and their relation with serum ferritin. Methods This prospective observational study included all patients between the age groups six months and 14 years with transfusion-dependent thalassemia. We included 62 children admitted during the study period fulfilling eligibility criteria. The data was analyzed using descriptive statistics and making comparisons among various groups. Spearman correlation analysis was done to assess the correlation between serum ferritin and thyroid hormone. The difference of means across the groups was tested with the Mann-Whitney U test for two groups and the Kruskal-Wallis test for more than two groups. Results The mean age of the study participants was 5.66 ± 3.77 years, with the largest group consisting of children aged one to three years, comprising 40.3% of the participants. The majority of participants were boys. This study showed a high prevalence of endocrinopathies in transfusion-dependent thalassemic patients. The most common endocrinopathy was short stature (37.1%), followed by impaired glucose tolerance (28.6%), subclinical hypothyroidism (14.5%), and parathyroid dysfunction (14.5%). Overt diabetes and pubertal delay were not seen. A statistically significant association of ferritin was found with age (p < 0.001), stature (p = 0.001), thryroid-stimulating hormone (TSH) (p = 0.004), and parathyroid function (p = 0.006). Conclusions The prevalence of endocrinopathies in present transfusion-dependent thalassemic cohorts was considerably high, presenting as short stature, impaired glucose tolerance, hypoparathyroidism, and subclinical hypothyroidism. The study showed a weak positive correlation of endocrinopathies with serum ferritin levels. Hence, irrespective of serum ferritin levels, patients with transfusion-dependent thalassemia can have a considerably high prevalence of endocrine complications.
地中海贫血是最常见的遗传性贫血形式,由血红蛋白中两条珠蛋白链之一的合成受损引起。β地中海贫血中β珠蛋白链减少,导致α珠蛋白链相对过剩。重型地中海贫血是地中海贫血的严重形式,需要频繁输血以维持生命。因此,疾病的自然进程受到输血相关副作用的影响。反复输血导致铁在肝脏、心脏和内分泌腺等组织中蓄积。血清铁蛋白是铁过载的生物标志物。内分泌病是地中海贫血中最常见的并发症之一。早期识别和治疗对于预防晚期不可逆后遗症和改善这些患者的生活质量很重要。本研究旨在评估重型地中海贫血患儿的生长参数和内分泌功能及其与血清铁蛋白的关系。
这项前瞻性观察性研究纳入了所有年龄在6个月至14岁之间、依赖输血的地中海贫血患者。我们纳入了研究期间符合入选标准的62名儿童。使用描述性统计分析数据并在不同组之间进行比较。进行Spearman相关性分析以评估血清铁蛋白与甲状腺激素之间的相关性。两组间均值差异用Mann-Whitney U检验,多组间用Kruskal-Wallis检验。
研究参与者的平均年龄为5.66±3.77岁,最大的组是1至3岁的儿童,占参与者的40.3%。大多数参与者为男孩。本研究显示,依赖输血的地中海贫血患者中内分泌病的患病率很高。最常见的内分泌病是身材矮小(37.1%),其次是糖耐量受损(28.6%)、亚临床甲状腺功能减退(14.5%)和甲状旁腺功能障碍(14.5%)。未发现明显糖尿病和青春期延迟。发现铁蛋白与年龄(p<0.001)、身高(p = 0.001)、促甲状腺激素(TSH)(p = 0.004)和甲状旁腺功能(p = 0.006)之间存在统计学显著关联。
目前依赖输血的地中海贫血队列中内分泌病的患病率相当高,表现为身材矮小、糖耐量受损、甲状旁腺功能减退和亚临床甲状腺功能减退。该研究显示内分泌病与血清铁蛋白水平呈弱正相关。因此,无论血清铁蛋白水平如何,依赖输血的地中海贫血患者内分泌并发症的患病率都可能相当高。