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.

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 βTM, 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.

Background: Pneumonia is still a leading cause of death among children in developing countries.The aim of the study is to identify some risk factors for poor pneumonia outcome. The studied factors were: male sex, thrombocytosis, thrombocytopaenia, leucocytosis, leucopaenia and anaemia.Patients and methods: A prospective cross sectional study included 242 children with community acquired pneumonia (CAP) who were attending a tertiary care hospital. Work for the study started at October 2012 and ended at August 2014. Medical history, physical examination, complete blood count (CBC) and C-reactive protein were done for all patients.Results: 27.3% of the patients developed pulmonary complications, 17.4% acquired sepsis while death occurred in 3.7% of cases. Multinomial logistic regression was used to detect the independent effect of each studied factor. Thrombocytosis, anaemia and age 62 years were associated with the development of pulmonary complications. Thrombocytopaenia and leucopaenia were associated with the fatal course of the disease. Male sex was a protective factor against pulmonary complications; however it was a risk factor for acquiring sepsis. Leucocytosis did not affect the prognosis of CAP.Conclusion: Sex of the patient and data from complete blood count are very useful for the prediction of pneumonia outcome. Anaemia, thrombocytosis, thrombocytopaenia, and leucopaenia are considered as predictors of bad prognosis. Females are more vulnerable to pulmonary complications while maleness is associated with sepsis and determination of these indicators highlights suspicion of pneumonia prognosis and allows application of early appropriate interventions.

Background/objectives β‐Thalassemia intermedia (β‐TI) accounts for up to one‐fourth of β‐thalassemia patients. Evaluating and improving quality of life (QOL) should be a goal in β‐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 β‐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 β‐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 β‐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.

No abstract

problem owing to the wide prevalence of the disease in these regions. [1] β thalassemia is inherited as an autosomal recessive disorder, which results in reduction or absence in β globin chain. [2] Point mutation in the β-globin gene is the cause of β thalassemia inheritance in majority of cases, while short deletion in the same gene may occasionally be the cause. More than 200 mutations that result in β-thalassemia are present all over the world. [3] Mutation type varies among different populations; in Egypt, there are about 19 mutations, the most common are IVS-I-110 (G->A), IVS-I-1 (G->A), and IVS-I-6 (T->C). [4] If the mutation results in complete absence of β chain, then the phenotype will be βo or β thalassemia major. If the mutation permits some degree of β chain production, then the phenotype will be thalassemia intermedia. Background: β thalassemia has been considered one of the most common genetic diseases. It represents public health concern especially in Middle East and Mediterranean regions. Objective: To determine the influential effect of paternal and maternal age son β thalassemia inheritance. Materials and Methods: This case-control study was conducted at EL Fayoum University Hospital in the period from June 2013 to June 2014. The study included 94 children with β thalassemia. Full medical history was taken. The state of parental consanguinity and paternal and maternal ages at time of the child's birth were recorded (as given by history and confirmed by data from birth record of the child and identity record of the parents). Results were interpreted using Statistical Package for Social Science program. Result: Fathers aged ≥40 years revealed increased risk to possess children with β thalassemia (P = 0.000, 95% CI: −0.038, 0.015). Children of relative parents had significantly increased chance to possess β thalassemia (P = 0.000, 95%CI: 0.219, 0.491). Maternal age did not have any influential effect on β thalassemia inheritance. There were no significant differences between thalassemia major and thalassemia intermedia with respect to father's age and consanguinity. Conclusion: Parents with positive consanguinity and fathers aged ≥40 years showed increased risk to possess children with β thalassemia disease. These results must be considered on applying the rules of preconception screening and genetic counseling to thalassemia. Complementary genetic studies about this issue are recommended.

No abstract

No abstract