Habeb Abdelhadi M, Al-Hawsawi Zakaria M, Morsy Mohamed M, Al-Harbi Abdallah M, Osilan Adnan S, Al-Magamsi Mohamed S, Zolaly Mohamed A
Pediatric Department, Maternity and Children`s Hospital, Al-Madinah, Kingdom of Saudi Arabia.
Saudi Med J. 2013 Jan;34(1):67-73.
To define the prevalence, risk factors, and age at diagnosis of endocrinopathies in beta-thalassemia major (BTM) in Northwest Saudi Arabia.
This retrospective cross-sectional study included patients with BTM attending a combined endocrine-hematology clinic in Al-Madinah, Kingdom of Saudi Arabia from March 2009 to December 2010. Clinical and biochemical data from the initial clinic visits were used to define the prevalence and age of diagnosis of endocrinopathies. Demographic and laboratory variables were analyzed to identify significant risk factors.
Eighty-one patients (42 males), aged 2-28 years were screened. Thirty-eight of them (46.9%) had at least one endocrinopathy. Of these, 28.9% (11/38) were aged less than 10 years. Hypogonadism was the most common complication detected in 52.7% (19/36) of patients of pubertal age group and 23.4% (19/81), of all cohort followed by short stature in 20.9% (17/81), subclinical hypothyroidism in 14.8% (12/81) and hypoparathyroidism in 11.1% (9/81). Patients with endocrinopathies were older (p=0.001), had longer duration of transfusion (p=0.001), and were started at a late age on chelation than those without endocrinopathies (p=0.07). Recent serum ferritin was poorly correlated to endocrinopathies (p=0.15).
Endocrinopathies are common in our BTM cohort, and patients with this condition benefit from regular endocrine screening within the first 10 years of life. Although endocrinopathies were more prevalent in older patients; further, longitudinal studies are needed to define the exact age of onset and independent risk factors for these complications.
确定沙特阿拉伯西北部重型β地中海贫血（BTM）患者内分泌疾病的患病率、危险因素及诊断年龄。
这项回顾性横断面研究纳入了2009年3月至2010年12月在沙特阿拉伯王国麦地那一家内分泌 - 血液科联合诊所就诊的BTM患者。初次就诊时的临床和生化数据用于确定内分泌疾病的患病率和诊断年龄。分析人口统计学和实验室变量以确定显著的危险因素。
筛查了81例患者（42例男性），年龄在2 - 28岁之间。其中38例（46.9%）至少患有一种内分泌疾病。其中，28.9%（11/38）年龄小于10岁。性腺功能减退是最常见的并发症，在青春期年龄组患者中占52.7%（19/36），在所有队列中占23.4%（19/81），其次是身材矮小占20.9%（17/81），亚临床甲状腺功能减退占14.8%（12/81），甲状旁腺功能减退占11.1%（9/81）。患有内分泌疾病的患者年龄更大（p = 0.001），输血时间更长（p = 0.001），开始螯合治疗的年龄比未患内分泌疾病的患者晚（p = 0.07）。近期血清铁蛋白与内分泌疾病的相关性较差（p = 0.15）。
内分泌疾病在我们的BTM队列中很常见，患有这种疾病的患者在生命的前10年内受益于定期内分泌筛查。尽管内分泌疾病在老年患者中更普遍；此外，需要进行纵向研究以确定这些并发症的确切发病年龄和独立危险因素。
