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临床试验/NCT03161899
NCT03161899已完成不适用

Evaluation of Nutritional Status in Patients With Thalassemia Major in Assiut University Children Hospital

Assiut University1 个研究点 分布在 1 个国家目标入组 246 人开始时间: 2018年12月15日最近更新:
适应症

试验速览

阶段
不适用
状态
已完成
入组人数
246
试验地点
1
主要终点
percentage of malnutrition

研究概览

简要总结

Thalassemia is a blood disorder passed down through families in which the body makes an abnormal form of hemoglobin. There are 2 main types of thalassemia; Alpha & Beta thalassemia. Alpha thalassemia: occurs when a gene or genes related to the alpha globin protein are missing or mutated.

Beta-thalassemia syndromes are a group of hereditary blood disorders characterized by reduced or absent beta globin chain synthesis. Beta-thalassemias can be classified into:

Silent carrier: completely asymptomatic with normal hematological parameters. Beta-thalassaemia minor (beta-thalassaemia trait): usually asymptomatic; diagnosis is made during a work-up for mild anemia.

Beta-thalassaemia intermedia: usually a similar presentation to beta-thalassaemia major; symptoms are usually less pronounced and the course is usually more insidious.

Beta-thalassaemia major : In which there is complete absence of hemoglobin A

详细描述

In Egypt beta thalassemia-major is the most common type with carrier rate of 5.3 to ≥9%and 1000 new cases born with beta-thalassemia major per 1.5 million live births per year.

Children born with thalassemia major are normal at birth, but develop severe hemolytic anemia during the first year of life. Symptoms are those of anemia (lethargy, poor feeding, pallor...etc.) failure to thrive and organomegaly. Later on they develop signs of extra medullary hematopoiesis .

Optimal nutritional status is important for growth, immune function, bone health and pubertal development . Various reports suggest the incidence of poor growth ranges from 25% to 75% depending on thalassemia syndrome and severity of disease. This marked growth deficits raise a red flag for any pediatrician to evaluate nutritional status of thalassemic patients and detect possible nutritional deficiencies and associated factors.

Etiology of delayed growth and malnutrition reported in thalassemic patient is multifactorial including :

  1. Nutritional deprivation with or without feeding difficulties arising from fatigue and breathlessness
  2. Increased energy expenditure secondary to hyper metabolism with or without heart failure
  3. Gastrointestinal hypoxia which consequently produces anorexia and malabsorption
  4. Reduction of biosynthetic activity of liver.
  5. Disturbance of the endocrine function
  6. Impaired synthetic hepatic function secondary to hemosiderosis and hepatitis.

研究设计

研究类型
Observational
观察模型
Case Only
时间视角
Cross Sectional

入排标准

年龄范围
6 Years 至 14 Years(Child)
性别
All
接受健康志愿者

入选标准

  • patients diagnosed to have thalassemia

排除标准

  • patient with any underlaying systemic diseases other than B thalassemia major.

结局指标

主要结局

percentage of malnutrition

时间窗: 12 month

prevalence of malnutrition among thalassemic children attending assiut university children hospital

次要结局

  • aetiology poor growth(12 month)
  • decrease morbidity(18 month)

研究者

申办方类型
Other
责任方
Principal Investigator
主要研究者

ARAbdelmonem

resident doctor of pediatrics

Assiut University

研究点 (1)

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