Tamer Hassan

Correlation between musculoskeletal function and radiological joint scores in haemophilia A adolescents.

Summary.  The functional independence score in haemophilia (FISH) is a performance‐based instrument used to assess musculoskeletal function in patients with haemophilia. We aimed to evaluate the functional independence of haemophilia A adolescents and its correlation to radiological joint scores. A cross‐sectional study was carried out on 50 adolescent haemophilia A patients. Musculoskeletal function was assessed using the FISH and individual joints were assessed radiologically using the Pettersson score and MRI scale. The mean age of our patients was 16 ± 1.1 with a mean FISH of 23.32 ± 4.69 (range 13–28) and a mean Pettersson score of 2.32 ± 3.09 (range 0–13) for the knees, 1.86 ± 2.67 (range 0–11) for ankles and 1.42 ± 2.17 (range 0–10) for elbows. The mean MRI score for the knees was 3.92 ± 2.74 (range 0–10) while that for ankles was 3.16 ± 2.64 (range 0–10) and for elbows was 2.34 ± 2.63 (range 0–10). There was highly significant correlation between both radiological joint scores and FISH and between degree of factor VIII deficiency and each of FISH, Pettersson score and MRI score. MRI was superior to conventional radiography in detection of subchondral cyst formation and erosions at joint margins. Given the highly significant correlation with both radiological joint scores, FISH appears to be a reliable tool for assessment of functional independence in adolescents with haemophilia A. MRI is more sensitive than conventional radiography in detection of early joint abnormalities.

Association between genes encoding components of the IL-10/IL-0 receptor pathway and asthma in children

BACKGROUND Asthma is a chronic inflammation of the airways associated with recurrent symptoms that range from mild to debilitating. Interleukin-10 (IL-10) is a cytokine that displays pleiotropic effects in asthma and allergy. OBJECTIVE To determine whether polymorphisms of IL-10/IL-10R pathway contribute to asthma susceptibility in Egyptian children. METHODS The IL-10 (-1082G/A), IL-10R1 (G330R), and signal transducer and activator of transcription 3 (STAT3) rs2293452 single-nucleotide polymorphism (SNP) were genotyped in 110 atopic children with asthma, 110 non-atopic children with asthma, and 110 healthy children. Serum IL-10 and immunoglobulin E (IgE) levels were determined by enzyme-linked immunosorbent assay. RESULTS A significant association was observed between the IL-10 polymorphism and asthma in both atopic (P = .03) and non-atopic asthma groups (P = .04). The genotype frequencies of IL-10R1 polymorphisms did not differ between all groups. We identified a significant association between STAT3 polymorphism and asthma susceptibility in atopic asthma (P < .001), whereas no such association was observed in the non-atopic asthma group (P = .9). No evidence of gene interactions was found. CONCLUSION Polymorphisms of IL-10 and STAT3 may be useful as a new DNA-based diagnostic biomarker for identifying high-risk children susceptible to asthma.

Assessment of musculoskeletal function and mood in haemophilia A adolescents: a cross‐sectional study

Summary.  Haemophilia A is characterized by the occurrence of frequent spontaneous intra‐articular and intramuscular bleeding. If inadequately treated, it results in progressive damage to joints and muscles leading to crippling deformities and musculoskeletal dysfunction. These complications result in lifelong chronic pain and disability that may greatly affect the patients’ mood. We aimed to evaluate the musculoskeletal function in our haemophilia A patients and its correlation to depressed mood in these patients and determine the impact of degree of factor VIII deficiency, different replacement therapy regimens and frequency of hemarthrosis, on both musculoskeletal function and mood. A cross‐sectional study was carried out on 50 adolescent haemophilia A patients. Musculoskeletal function was assessed using Functional Independence Score for Hemophilia (FISH) and mood status was assessed using Beck Depression Inventory—Short Form (BDI‐SF). The mean FISH score was 23.32 ± 4.69 (range 13–28) and the tasks that obtained lower scores were step climbing, squatting and walking. Of our 50 patients included, 16(32%) were not depressed, 18(36%) were with mild depression, 11(22%) were with moderate depression and 5(10%) were with severe depression. There was a highly significant negative correlation between mean FISH score and mean BDI‐SF score (P < 0.001). The better the replacement therapy regimen, the better the musculoskeletal function that could be obtained in haemophilia A patients and the better the mood.

Hodgkin Lymphoma in Childhood: Clinicopathological features and therapy outcome at 2 Centers From a Developing Country

AbstractHodgkin lymphoma (HL) accounts for 5% to 6% of all childhood cancer. It displays characteristic epidemiological, clinical, and pathological features according to various geographic areas. We aimed to assess the epidemiological aspects, clinicopathological features, and treatment outcome of pediatric HL treated at 2 Egyptian centers: Zagazig University Pediatric Oncology Unit and Benha Special Hospital Pediatric Oncology Unit.We carried a cross-sectional retrospective study by reviewing medical records for all patients admitted with the diagnosis of HL over 8 years in 2 oncology units during the period from January 2004 to January 2012.Age of the patients at presentation ranged from 3 to 14 years (median 6 years) and male: female ratio 1.7:1. Lymphadenopathy was the most common presentation (96.6%). Mixed cellularity subtype was dominant (50.8%), followed by nodular sclerosis (28.9%), lymphocyte-rich (18.6%) with lymphocyte depletion being the least dominant (1.7%). More than half of patients (55.9 %) had advanced disease (Ann Arbor stage III/IV disease). The duration of follow-up ranged from 5 to 87 months (mean 39.8 ± 24.1 months). The 5-year overall survival and event-free survival for patients were 96.6% and 84.7% respectively.In Egypt, HL occurs in young age group, with a higher incidence of mixed cellularity subtype and advanced disease. None of the clinical, epidemiological, or pathological characteristics had a significant association with the overall survival. The outcomes of HL in our 2 centers were satisfactory approaching the international percentage.

Impact of iron deficiency anemia on the function of the immune system in children.

AbstractThe importance of iron deficiency as a public health problem is based ultimately on the seriousness of its consequences on health. The most extensively investigated consequences of iron deficiency involve work performance and immune function. The significance of the effects on work performance is generally accepted. In contrast, data on the influence of iron deficiency on immune function are often perceived as being confusing and contradictory.We aimed to evaluate the effect of iron deficiency anemia on humoral, cellular, nonspecific immunity, and also the effect on the cytokines that are the key factors of many immunologic steps.Forty children with iron deficiency anemia and 20 age and sex-matched healthy children were included. All children were subjected to full medical history, thorough clinical examination, complete blood count, iron indices (serum iron, serum total iron-binding capacity, serum ferritin, and transferrin saturation), immunoglobulin assay (IgA, IgG, and IgM), interleukin (IL)-6 serum level, study of T-lymphocyte subsets, and evaluation of phagocytic function of macrophages and oxidative burst activity of neutrophils.Patients had significantly lower IgG levels, IL-6, phagocytic activity, and oxidative burst of neutrophils than controls, although there was no significant difference between patients and controls with regard to other immunoglobulins and CD4/CD8 ratio. There was significantly positive correlation between serum iron and IL-6 serum level.We concluded that humoral, nonspecific immunity (phagocytic activity and oxidative burst), and the IL-6 are influenced in patients with iron deficiency anemia. Study of these abnormalities after correction of iron deficiency is strongly needed.

Protein C levels in β-thalassemia major patients in the east Nile delta of Egypt

BACKGROUND AND OBJECTIVES Thalassemic patients have an increased risk for thromboembolic complications. To determine if this might be due to a deficiency in protein C, we investigated the status of the protein C anticoagulant pathway in thalassemia major patients and its relationship to the hypercoagulable state. PATIENTS AND METHODS Fifty patients with β-thalassemia major (30 non-splenectomized and 20 splenectomized) and 20 healthy children as a control group were tested for levels of serum ferritin, liver enzymes, serum albumin, fibrinogen, protein C and protein S, thrombin antithrombin complex (TAT) and D-dimer. RESULTS Thalassemic patients had lower levels of protein C and S and higher levels of D-dimer and TAT than the control group. These findings were more obvious in splenectomized patients and in those with infrequent blood transfusion. CONCLUSIONS Protein C plays a major role in the hypercoagulable state in thalassemic patients. These findings raise the issue as to whether it would be cost-beneficial to recommend prophylactic antithrombotic therapy in high-risk thalassemic patients. A wider prospective study is necessary to delineate under which circumstances therapy might be needed, and at what level of protein C deficiency to start prophylactic antithrombotic therapy.

Renal tubular dysfunction in children with sickle cell haemoglobinopathy

Children with sickle cell disease (SCD) are remarkably more prone than others to renal dysfunction. The kidneys, as one of the systemic long‐term hazards in SCD, may be affected by both the haemodynamic changes of chronic anaemia as well as by the consequences of vaso‐occlusion. The aim of this study was to evaluate the proximal tubular function in a group of Saudi children with established SCD.

Oral magnesium supplementation improves glycemic control and lipid profile in children with type 1 diabetes and hypomagnesaemia

Abstract Dietary supplementation with magnesium (Mg) in addition to classical therapies for diabetes may help in prevention or delaying of diabetic complications. We aimed to evaluate the status of serum Mg in children with type 1 diabetes and assessing its relationship to glycemic control and lipid profile. Then evaluating the effect of oral Mg supplementation on glycemic control and lipid parameters. We included 71 children at Pediatric Endocrinology Outpatient Clinic, Zagazig University, Egypt with type 1 diabetes and assessed HBA1c, lipid profile, and serum Mg at the start of study. Patients with serum Mg level < 1.7 mg/dL were given 300 mg Mg oxide for 3 months. After that we reevaluated HBA1c, lipid profile, and serum Mg in all patients. The study included 71 patients with type 1 diabetes (32 males and 39 females); their mean age was 9.68 ± 3.99 years. The mean serum Mg level was 1.83 ± .27 mg/dL. Hypomagnesemia was detected in 28.2% study patients. Serum Mg was found to be positively correlated with high density lipoprotein, mean corpuscular volume and platelet count (P < 0.001), and negatively correlated with age, HbA1c, triglycerides, total cholesterol, low density lipoprotein, and duration of diabetes (P < 0.001). There was significant reduction in HBA1c in group given Mg supplementation. HBA1c was initially 10.11% ± 0.87%. After 3 months of oral Mg supplementation it is reduced to 7.88% ± 0.42% (P < 0.001). There was statistically significant difference in lipid parameters in hypomagnesemic diabetic patients before and after Mg supplementation with significant reduction in serum triglycerides, LDL, and total cholesterol following Mg supplementation with P < 0.001. Although HDL shows a significant increase after Mg supplementation in hypomagnesemic diabetic children with P < 0.001. Correction of hypomagnesemia in type 1 diabetic children with oral Mg supplements is associated with optimization of glycemic control and reduction of atherogenic lipid fraction as well as increase in protective lipid fraction.

Magnetic resonance imaging of the brain in survivors of childhood acute lymphoblastic leukemia

The issue of delayed neurological damage as a result of treatment is becoming increasingly important now that an increased number of children survive treatment for acute lymphoblastic leukemia (ALL). Following modification of the treatment protocols, severe symptomatic late effects are rare, and most adverse effects are detected by sensitive imaging methods such as magnetic resonance imaging (MRI) or by neuropsychological testing. In this study we aimed to determine the prevalence and characteristics of late central nervous system (CNS) damage by MRI and clinical examination in children treated for ALL. A cross-sectional study was carried out at the pediatric oncology unit of Zagazig University, Egypt, and included 25 patients who were consecutively enrolled and treated according to the modified Children’s Cancer Group (CCG) 1991 protocol for standard risk ALL and the modified CCG 1961 protocol for high-risk ALL and who had survived more than 5 years from the diagnosis. All relevant data were collected from patients’ medical records; particularly the data concerning the initial clinical presentation and initial brain imaging. All patients were subjected to thorough history and full physical examination with special emphasis on the neurological system. MRI of the brain was performed for all patients. The mean age of patients was 6.9±3.04 years at diagnosis and was 12.9±3.2 years at the time of study. The patients comprised 14 boys and 11 girls. Abnormal MRI findings were detected in six patients (24%). They were in the form of leukoencephalopathy in two patients (8%), brain atrophy in two patients (8%), old infarct in one patient (4%) and old hemorrhage in one patient (4%). The number of abnormal MRI findings was significantly higher in high-risk patients, patients who had CNS manifestations at diagnosis and patients who had received cranial irradiation. We concluded that cranial irradiation is associated with higher incidence of MRI changes in children treated for ALL. Limitation of cranial irradiation to selected patients contributed to a lower incidence of neurological complications in our study. MRI is a sensitive radiological tool to detect structural changes in children treated for ALL, even in asymptomatic cases.

Impact of health instructions on improving knowledge and practices of haemophilia A adolescents: a single centre experience

Nowadays, health education has been elevated to a higher standing in healthcare systems in managing chronic illness; yet, this approach has not received sufficient support in developing countries as these societies still tend to the traditional stage of ‘treatment after disease’. Adolescence is a critical period and voyage into adulthood can be more challenging for haemophilia teens. For teens with haemophilia, learning to care for their own disorder is a giant step forward in asserting their independence and preparation for adult life.