患有Β严重地中海贫血的也门儿童的生长模式与血清铁蛋白的关系,也门地中海贫血和遗传性血液疾病协会-也门萨那

Abdullah A. K. Al-Tayar, Ali Ahmed Al-Zaazaai
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引用次数: 4

摘要

背景:地中海贫血是一种异质性遗传疾病,其分类为组成血红蛋白分子的一个或多个珠蛋白链的产生减少或缺失。目的:本研究的主要目的是评估也门地中海贫血和遗传性血液病学会中接受螯合治疗的高输注β地中海贫血儿童的生长模式和生长衰竭率,并与血清铁蛋白水平进行比较。方法:采用比较描述性研究方法,对也门地中海贫血和遗传性血液病协会的109名2-18岁β地中海贫血主要患者(52名男性47名女性)的生长参数(身高、体重)和血清铁蛋白进行测定,根据世界卫生组织,将生长与相同年龄和性别的正常生长图进行比较,然后生长模式为具有血清铁蛋白和含铁血黄素血症程度的camper。结果:在接受调查的所有患者中,无论男女,身高和体重均低于5厘的生长迟缓(67.889%),具体而言,与同年龄和性别的正常儿童的身高相比,有(71.60%)身高在5厘米以下(38.46%为女性患者,61.53%为男性患者),与同性别和年龄的正常儿童体重相比有(67.9%)体重不足且在5厘米以下(37.83%为女性患者和62.16%为男性患者)。结论:与同龄和性别的正常儿童相比,地中海贫血患者明显发生生长迟缓(体重不足和身材矮小),与也门以外其他国家的相同研究相比,也门患者的生长迟缓更多。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Growth Pattern Among Yemeni Children Suffering from Β Thalassemia Major in Relation to Serum Ferritin the Yemeni Society for Thalassemia and Genetic Blood Disorder - Sana'a Yemen
Background: Thalassemia are a heterogeneous collection of genetic disorders categorized by decreased or absent production of one or more globin chains that make up a hemoglobin molecule. Objective: The main aim of the present study was to evaluate the growth pattern and growth failure rate in children with hyper transfused β thalassemia major those on chelating therapy in comparison with serum ferritin level in the Yemeni society for thalassemia and genetic blood disorder. Methods: In this comparative descriptive study, the growth parameters (height, weight) and serum ferritin of 109 patients aged 2-18 years (52 males 47 females) with β-thalassemia major in The Yemeni society for thalassemia and genetic blood disorder Sana`a, were taken, In which the growth was compared with normal growth charts for the same age and gender according to WHO then the growth pattern is camper with serum ferritin and degree of hemosiderosis. Results: Growth retardation below 5 centiles were found in (67.889%) of total surveyed Patients for both height and weight in both gender, in details there are (71.60%) are short and under 5th centile in compare with height of normal children at same age and gender (38.46% is female patients and 61.53% is male patients) and there are (67.9%) are underweight and under 5th centile in compare with weight of normal children at same age and gender (37.83% is female patient and 62.16% is male patients). Conclusion: Growth failure (underweight and short stature) significantly occurs in thalassemia patients compared to normal children of the same age and sex, and such growth retardation was more in Yemeni patients compared with same studies on other countries than Yemen.
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