Evaluation of Nutritional Status in Thalassemia Major Patients in Assiut Children Hospital
Condition: Thalassemia Major
MeSH terms: Thalassemia; beta-Thalassemia
Study type: Observational

Summary: 
      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
    

Detailed description: 
      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.

      The main aim of this study is to assess the nutritional status of thalassemic patient
      attending Assiut university Children hospital and to determine the factors involved.
    

Observational model: Case-Only
Time perspective: Cross-Sectional
Sampling method: Non-Probability Sample
Study population: 
        all beta thalassemic patients attending the children Hematology Clinic, Children Hospital,
        Assiut University, during the period of 1st of June 2017 till 31 th of may 2018.
      
Keywords: thalassemia - nutrition -growth