The impact of illness perception and socio-clinico-demographic factors on perceived quality of life in children and adolescents with thalassemia intermedia.

BACKGROUND/OBJECTIVES: ß-Thalassemia intermedia (ß-TI) accounts for up to one-fourth of ß-thalassemia patients. Evaluating and improving quality of life (QOL) should be a goal in ß-TI follow-up and management strategies. Patients' perceptions of their illness and its treatment may impact their QOL. This study aimed to evaluate QOL and the factors that affect it in children with ß-TI and to determine the impact of the patients' and their mothers' perceptions of the illness on patients' QOL. DESIGN/METHODS: This was a case-control study. A total of 143 children and adolescents (71 ß-TI patients and 72 healthy controls) were enrolled. QOL, as perceived by the children and their mothers, was assessed using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scale. Perceptions of the illness by the mothers and children were assessed using the Brief Illness Perceptions Questionnaire (Brief IPQ). The patients' clinical and sociodemographic data were extracted from their medical records. RESULTS: The controls had higher QOL scores in nearly all domains (P < 0.01). The patients and mothers who had higher illness perception scores had better QOL scores (P < 0.01). Patient age and serum ferritin levels correlated negatively with QOL, while mean hemoglobin levels correlated positively. The use of hydroxyurea in treatment and high illness perception were independent predictors of better QOL. CONCLUSION: QOL is significantly affected in ß-TI patients; maintaining a suitable hemoglobin level and standard levels of body iron are associated with better QOL. Patients' and their mothers' perceptions of the illness play an important role in QOL.

Transfusion transmitted infections in frequently transfused thalassemic children living in Fayoum Governorate, Egypt: Current prevalence and risk factors.

Regular blood transfusion therapy remains the primary treatment in thalassemia major (TM). Transfusion-transmitted infections (TTIs) and iron overload are considered to be the major drawbacks of this therapy. This cross-sectional study aimed to update the prevalence of the hepatitis C virus (HCV) antibody, PCR-confirmed HCV, hepatitis B surface antigen (HBsAg), and human immunodeficiency virus (HIV) antibody among TM children. Clinical and epidemiological factors that can affect HCV infection prevalence rate were studied. This study evaluated 121 children with ßTM, including 61 males and 60 females with a mean age of 7.99±3.57years. Patients were evaluated for the HCV, HBsAg, and HIV-1 & 2 antibodies. All tests were performed using ELISA. HCV positive cases were confirmed by RT-PCR. Twenty-five patients were positive for the HCV antibody (20.7%); 22 were confirmed positive by PCR. Six patients (5%) were HBsAg-positive. No patients were HIV-positive. Older age were associated with an increased frequency of HCV positive infection (P<0.003). More frequent transfusion, ≥10 times/year, and older age, ≥10years, were reported as predictors of HCV infection (P=0.018 and 0.011, respectively). A significant association of HCV between HBV infections was reported (P value=0.01). There was no significant effect of the pre-transfusion or post-transfusion hemoglobin level on the frequency of HCV positive cases. HCV still represents a major health challenge for frequently transfused Egyptian patients. The prevalence rate of HBV infection remains relatively high. Therefore, it is necessary to implement measures to improve blood transfusion screening.

Vitamin D deficiency and osteoporosis in hemophilic children: an intermingled comorbidity.

The aim of this study is to investigate bone state and factors affecting it in children with hemophilia. This is a case-control study that included 37 children with hemophilia and 37 healthy controls. The patients were selected from the outpatient pediatric hematology clinic of Fayoum University Hospital, Egypt. Bone mineral density, serum vitamin D, parathormone, calcium, phosphorus, and calcium creatinine ratio levels were evaluated. Vitamin D level and bone mineral density were significantly lower in hemophiliacs than in control group (P < 0.0001). About 43.2% of cases had moderate vitamin D deficiency, whereas 35.1% had mild deficiency. Vitamin D positively correlated with bone mineral density Z-score, whereas it negatively correlated with total joint score. Positive correlation between bone mineral density and age was also found. Serum levels of urea, urinary calcium creatinine ratio, and parathormone were found to be higher in cases than in control. Also, serum calcium level was found to be lower in patients than in controls. We concluded that vitamin D deficiency is an essential cause of decreased bone mineral density in hemophilic children. Hemophilic arthropathy with consecutive immobilization plays an important role in vitamin D deficiency and decreased bone mineral density.