Health-related quality of life in pediatric and adolescent patients with transfusion-dependent ß-thalassemia in upper Egypt (single center study)
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Thalassemia is a major health problem that disturbs the lifestyle of the affected patient. The aim of this work is to detect the impact of thalassemia on the quality of life regarding physical, social, emotional, psychological scored assessment.A case-control survey was conducted in Minia University children's hospital on 64 patients recruiting pediatric hematology outpatient clinic from July 2014 to February 2017. PedsQL™ 4.0 Generic Core Scale (Arabic version) was used to assess HRQOL in 64 thalassemia patients between 8 and 18 years of ages. Other related clinical data of the involved patients were collected from the pediatric hematology records.Mean physical, emotional, social, school performance, psychological and total scores (- 36.9 ± 20.9, 49.4 ± 17, 47.2 ± 21.3, 38.5 ± 15.5, 45.3 ± 13.8, 47.9 ± 38.8 respectively) were significantly decreased compared with control (p = 0.001 for all). The younger age group had better scores regarding social, emotional, psychological and total scores compared to older ones (p = 0.01, 0.03, 0.01 and 0.009 respectively). Older age of starting transfusion was statistically significant protecting factor from poor physical QOL in thalassemia patients (OR = 0.96, p = 0.03). The presence of hepatomegaly was a statistically significant predictor for poor physical QOL (OR = 8.5, p = 0.02). Household income was the statistically significant predictor for poor emotional QOL (OR = 5.03, p = 0.04). High serum ferritin was the statistically significant predictor for poor social QOL (OR = 1.1, CI 95%=, p = 0.04). Regarding poor psychological QOL (OR = 0.94, p = 0.01) and total QOL (OR = 0.94, p = 0.01) scores, older age of starting transfusion was the statistically significant protecting factor.Scheduled programs giving psychosocial help and a network connecting between the patients, school officials, thalassemia caregivers and the physician is required especially in developing countries where the health services are not integrated with social organizations. Special school services for thalassemia patients are required to deal with the repeated absence and anemia induced low mental performance of thalassemia children.Keywords:
Outpatient clinic
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Objective:To determine serum erythropoietin(EPO) levels in children with thalassemia after blood transfusion.Methods:Serum EPO levels were determined by enzymelinked immuno sorbent assay(ELISA) in 63 children,including 8 children with major β-thalassemia after blood transfusion,17 children with thalassemia carrier,and 25 normal children were compared. Results:EPO levels increase obviously(P0.001) of major β-thalassemia,and the difference had no statistical significance compaered after blood transfusion(P0.05 ),thalassemia carrier compared to normal children had no statistical significance (P0.05 ).Conclusion:EPO levels increase obviously about major β-thalassemia after blood transfusion of long term. EPO levels had no difference after blood transfusion and had no difference compared thalassemia carrier to normal children.
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Objective:To investigate the prevalence, genotype and clinic characterization of childrenαβ-thalassemia in Guangdong province. Methods:The DNA samples of 286β-thalassemias diagnosed by reverse dot blot (RDB ) were analyzed by Gap-PCR for α-thalassemia 1 gene and α-thalassemia 2 gene.Results:Of 286 β-thalassemias, 25 were detected to be combined with α-thalassemia, among which 14 were detected to be combined withα-thalassemia 1 gene and 10 were detected to be combined with α-thalassemia 2 gene. 18 cases of β-thalassemia minor were detected to be combined with α-thalassemia, 7 cases of β-thalassemia major were detected to be combined with α-thalassemia. The prevalences were 8.74%, 4.89%, 3.85%, 8.74% and 8.75%, respetively. β-thalassemia major accounted for 28.0 % in 25 cases of childrenαβ-thalassemia. The children of β-thalassemia minor combined withα-thalassemia had no remarkable clinic characterization. The chidren of β-thalassemia major combined with α-thalassemia had obvious clinic characterization such as anaemia and splenohepatomegalia and were detected to be β-thalassemia intermedius type. Conclusion: The prevalence of αβ-thalassemia in Guangdong province is relatively frequent. The genotypes of αβ-thalassemia are associated with the phenotypes. It is very important to investigate the genotype of αβ-thalassemia for guiding the nursing, health care, therapy, marriage and procreation of thalassemia children.
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Hematology
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Abstract Background Regular blood transfusion therapy still remains the cornerstone in the management of β‐thalassemia. Although recommendations are clear for patients with β‐thalassemia major, uniform transfusion guidelines are lacking for patients with hemoglobin E β‐thalassemia. In this study, we aim to describe the adequacy, trends, and determinants of blood transfusion therapy in a large cohort of pediatric patients with β‐thalassemia major and hemoglobin E β‐thalassemia. Methods/procedure This cross‐sectional study was performed among all regularly transfused patents with β‐thalassemia aged 2 to 18 years attending three large thalassemia centers in Sri Lanka. Data were collected using an interviewer‐administered questionnaire, perusal of clinical records, and physical examination of patients by trained doctors. Results A total of 328 patients (male 47%) were recruited; 83% had β‐thalassemia major, whereas 16% had hemoglobin E β‐thalassemia. Sixty‐one percent of patients had low pretransfusion hemoglobin levels (< 9.0 g/dL) despite receiving high transfusion volumes (> 200 mL/kg/year) by a majority (56%). Median pretransfusion hemoglobin was significantly lower in patients with hemoglobin E β‐thalassemia compared with β‐thalassemia major ( P < 0.001); however, there was no difference in requirement for high transfusion volumes over 200 mL/kg/year in both groups ( P = 0.14). Hepatomegaly and splenomegaly were more common in hemoglobin E β‐thalassemia and were associated with lower pretransfusion hemoglobin. Transfusion requirements were higher among patients with hepatitis C and in those who are underweight. Conclusions Over 60% of regularly transfused patients with β‐thalassemia have low pretransfusion hemoglobin levels despite receiving large transfusion volumes. Patients with hemoglobin E β‐thalassemia are undertransfused and specific recommendations should be developed to guide transfusions in these patients.
Transfusion Therapy
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Objective:To investigate the prevalence and genotype distribution of α β-thalassemia by using molecular detection and haematological methods.Methods:396 cases with screening positive in thalassemia were given gene diagnosis of α-thalassemia and β-thalassemia by gap-PCR and reverse dot blot hybridization.Results:There were 109 cases of α-thalassemia,115 cases of β-thalassemia and 26 cases of α β-thalassemia,which is 18.44% of α β-thalassemia in β-thalassemia.There were 7 geneypes in α β-thalassemia,involving 5 β-thalassemia geneypes and 2 α-thalassemia geneypes.No significant differences were found between β-thalassemia and α β-thalassemia in some RBC parameters.Conclusion:The incidence of α β-thalassem is frequent.The hematological analysis can not give specificity for diagnosing α β-thalassemia.Patients with screening positive in β thalassemia should be given gene diagnosis of α and β thalassemia.It is more useful for genetic counselling and prenatal diagnosis of this disease.
Beta thalassemia
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There are high prevalence of β-thalassemia and α-thalassemia in the upper Northern Thailand. Thus, the interaction between β-thalassemia and α-thalassemia can be occurred. The aim of this study was to analyze the incidence of gene interaction between α-thalassemia 1 SEA and THAI type deletions in 317 β-thalassemia carriers who were living in 3 provinces in upper Northern Thailand. The α-thalassemia 1 SEA and THAI type deletions were diagnosed using gap-PCR. The results showed that the double heterozygous of β-thalassemia and α-thalassemia 1 SEA type deletion was observed in 32 samples (10.1%). The hematological parameters, red blood cell indices and Hb A2 levels were compared among the two groups of β-thalassemia carriers with and without α-thalassemia 1 SEA type deletion. The RBC, Hb, Hct, MCHC, RDW-CV and HbA2 levels of the two groups were not significant difference ( p > 0.05) while the MCV and MCH of β-thalassemia carriers with α-thalassemia 1 SEA type deletion were significantly higher ( p < 0.001) than those of β-thalassemia carriers without α-thalassemia 1 SEA type deletion. These results indicated that there is a high prevalence of β-thalassemia and α-thalassemia interaction within the area with high frequency of β-thalassemia and α-thalassemia. Therefore, the default of α-thalassemia 1 diagnosis in β-thalassemia carriers can be a cause of Hb Bart’s hydrops fetalis. The hematological parameters of β-thalassemia carriers with and without α-thalassemia 1 SEA type deletion were similar. Therefore, the gene analysis of α-thalassemia 1 should be performed in all β-thalassemia carriers.
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