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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
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 :
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.
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| Measure | Description | Time Frame |
|---|---|---|
| percentage of malnutrition | prevalence of malnutrition among thalassemic children attending assiut university children hospital | 12 month |
| Measure | Description | Time Frame |
|---|---|---|
| aetiology poor growth | determination of the most common causes responsible for poor growth seen in thalassemic patients | 12 month |
| decrease morbidity | decrease morbidity by providing possible strategies for early prevention of the problem of poor growth occurring in patients with thalassemia major |
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Inclusion Criteria:
Exclusion Criteria:
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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.
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| Name | Affiliation | Role |
|---|---|---|
| asmaa refaat, MBBCH | Assuit , faculty of medicine, egypt | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Asmaa Refaat Abdelmonem | Asyut | Egypt |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 15380908 | Background | Soliman AT, El-Matary W, Fattah MM, Nasr IS, El Alaily RK, Thabet MA. The effect of high-calorie diet on nutritional parameters of children with beta-thalassaemia major. Clin Nutr. 2004 Oct;23(5):1153-8. doi: 10.1016/j.clnu.2004.03.001. | |
| 20712792 | Background | Fung EB. Nutritional deficiencies in patients with thalassemia. Ann N Y Acad Sci. 2010 Aug;1202:188-96. doi: 10.1111/j.1749-6632.2010.05578.x. |
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| ID | Term |
|---|---|
| D017086 | beta-Thalassemia |
| ID | Term |
|---|---|
| D013789 | Thalassemia |
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
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| 18 month |
| 20492708 | Background | Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis. 2010 May 21;5:11. doi: 10.1186/1750-1172-5-11. |
| D006402 |
| Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |