儿童小细胞贫血症中血红蛋白病的频率和类型。
Frequency and types of haemoglobinopathies in children with microcytic anaemia.
机构信息
Department of Pediatrics, Shifa College of Medicine, Shifa international Hospital, Islamabad, Pakistan.
Department of Pediatrics, Shifa College of Medicne, Islamabad, Pakistan.
出版信息
J Pak Med Assoc. 2021 Jan;71(1(A)):78-80. doi: 10.47391/JPMA.589.
OBJECTIVE
To study the frequency and types of haemoglobinopathies in children with microcytic anaemia.
METHODS
The prospective study was conducted at the Paediatric Out-patient Department of Shifa Falahi Community Health Centre, Islamabad, Pakistan, from July to December, 2018, and comprised patients aged from 3 months to 14 years who had haemoglobin <10mg/dl and mean corpuscular volume <70fL. Serum ferritin and haemoglobin electrophoresis were done to check for iron deficiency anaemia and haemoglobinopathies. Data was analysed using SPSS 23.
RESULTS
Of 175 subjects, 33(18.9%) had haemoglobinopathies and 142(81.1%) had iron deficiency anaemia. Thalassemia trait 18(10.3%) was the leading cause amongst haemoglobinopathies, followed by thalassemia major 8(4.6 %) and intermedia 5(2.9%). There were 2(1.1%) patients with haemoglobin D.
CONCLUSIONS
The prevalence of haemoglobinopathies was high. Identification of haemoglobinopathies is important for proper treatment, antenatal screening and future genetic counselling.
目的
研究小细胞性贫血患儿中血红蛋白病的频率和类型。
方法
本前瞻性研究于 2018 年 7 月至 12 月在巴基斯坦伊斯兰堡 Shifa Falahi 社区卫生中心儿科门诊进行，纳入血红蛋白<10mg/dl 且平均红细胞体积<70fL 的 3 个月至 14 岁患儿。检测血清铁蛋白和血红蛋白电泳以排除缺铁性贫血和血红蛋白病。采用 SPSS 23 对数据进行分析。
结果
175 例患儿中，33 例（18.9%）存在血红蛋白病，142 例（81.1%）存在缺铁性贫血。血红蛋白病中最常见的是 18 例（10.3%）地中海贫血 1 型，其次是 8 例（4.6%）重型地中海贫血和 5 例（2.9%）中间型地中海贫血。有 2 例（1.1%）患儿存在血红蛋白 D。
结论
血红蛋白病的患病率较高。识别血红蛋白病对于正确治疗、产前筛查和未来遗传咨询很重要。
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