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J Pediatr Hematol Oncol. 2015 May;37(4):281-4. doi: 10.1097/MPH.0000000000000327.

Cultural preferences and limited public resources influence the spectrum of thalassemia in Egypt.

Author information

1
Thalasemia Centre, Pediatric Hematology/Oncology Department, Ain Shams University, Cairo, Egypt.

Abstract

BACKGROUND:

Cultural beliefs of Egyptians with respect to the origin of thalassemia and its prevention, as well as national resources available for care, often differ from those of Western countries.

OBJECTIVES:

To assess the impact of cultural attitudes and the effect of limited medical and financial resources that could affect the management of Egyptian thalassemic patients.

SUBJECTS:

A cross sectional study included 205 Egyptians β-thalassemia major (β-TM) patients, with a mean age of 149±87.90 months and a male to female ratio of 94:111.

METHODS:

Demographic data stressing on order of birth, consanguineous marriage, and family history of β-TM, transfusion, and chelation therapy, were reported. HCV-Ab, HBV-Ag, and complete blood count were recorded with calculation of mean pretransfusional hemoglobin.

RESULTS:

The age distribution was relatively nonhomogenous, with 39% of patients between 10 and 20 years of age and 16% were younger than 5. There were high family birth rates and 35% of patients were third or more in order of birth and a marked cultural preference for consanguineous marriage, representing 61% of all the parents' marriages, as well as a high rate (59.5%) of a positive family history of β-TM. Patients transfused on low pretransfusion hemoglobin levels around 8 g/dL, and those receiving blood transfusion before the establishment of National Blood Transfusion Services showed a statistically significant higher rate of positive hepatitis B and C viral infections. Chelation therapy tended to start at late age, mean age was around 4 years. Before 2000, subcutaneous deferoxamine was the most widely used chelation, and since then a considerable number of patients (50%) had started to use oral iron chelators.

CONCLUSIONS:

The strong cultural preferences for consanguineous marriage and limited preventive programmes and resources have had a negative impact on the management of Egyptians thalassemic patients.

PMID:
25811748
DOI:
10.1097/MPH.0000000000000327
[Indexed for MEDLINE]

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