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

Thalassemia Major Clinical Trial

Official title:

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

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT03161899
• Other study ID #: ENST
• Status: Completed
• Start date: December 15, 2018
• Est. completion date: July 20, 2020

Study information

• Verified date: July 2020
• Source: Assiut University
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 246
• Est. completion date: July 20, 2020
• Est. primary completion date: May 24, 2020
• Accepts healthy volunteers: No
• Gender: All
• Age group: 6 Years to 14 Years

Eligibility

Inclusion Criteria:

• patients diagnosed to have thalassemia

Exclusion Criteria:

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

Related Conditions & MeSH terms

• beta-Thalassemia
• Thalassemia
• Thalassemia Major

Locations

Country	Name	City	State
Egypt	Asmaa Refaat Abdelmonem	Assiut

Sponsors (1)

Lead Sponsor	Collaborator
Assiut University

Country where clinical trial is conducted

Egypt, 

References & Publications (3)

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. — View Citation

Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis. 2010 May 21;5:11. doi: 10.1186/1750-1172-5-11. Review. — View Citation

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. — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	percentage of malnutrition	prevalence of malnutrition among thalassemic children attending assiut university children hospital	12 month
Secondary	aetiology poor growth	determination of the most common causes responsible for poor growth seen in thalassemic patients	12 month
Secondary	decrease morbidity	decrease morbidity by providing possible strategies for early prevention of the problem of poor growth occurring in patients with thalassemia major	18 month