Fatma S.E. Ebeid

Spinal cord compression in childhood pediatric malignancies: multicenter egyptian study.

Objectives: To assess the magnitude of management delay of pediatric malignant spinal cord compression (MSCC). Methods: Twenty-four patients with MSCC were recruited from 3 Egyptian pediatric oncology centers and assessed for MSCC clinical presentations, evaluation, and treatment response. Results: There was a median delay of 42 days from the onset of symptom until confirmed diagnosis. All studied patients presented inability to walk; 79% had pain (more in older patients) and 17% had sphincteric dysfunction. A total of 58.3% had a single level of cord compression, 41.7% had multiple levels. Thoracic spine was commonly involved (41%). Final diagnosis was: neuroblastoma (29.2%), soft-tissue sarcomas (20.8%), neuroectodermal tumor (16.6%), non-Hodgkin lymphoma (12.5%), astrocytoma (4.2%), malignant teratoma (8.4%), Wilms tumor (4.2%), and leukemia (4.2%). Magnetic resonance imaging of the spine was diagnostic in all cases. A total of 83.3% of patients received emergency steroid therapy and 75% showed improvement. Disease-specific therapy was multimodality therapy in 88.5% with 71.42% showing improvement. Lymphomas had the best neurological outcome (100%) followed by soft-tissue sarcomas (80%) and neural tumors (72.7%). The 3-year overall survival was 79.2%. Conclusions: Spinal cord compression is a serious complication and unacceptable management delay can result in preventable loss of function. Emergency magnetic resonance imaging evaluation is the most sensitive diagnostic imaging. Majority of patients improve after definitive therapy.

Psychiatric morbidity in Egyptian children with acute lymphoblastic leukemia and their care providers

AIM OF THE STUDY To evaluate the psychological morbidity of acute lymphoblastic leukemia (ALL) on children and their parents at different stages of illness and to assess the crucial contribution of the psychologist in the pediatric oncology team. METHODS We recruited 103 children with ALL and their 96 parents, and divided them into five groups according to disease phase: diagnosis, initial remission, active treatment, survival and relapsing. We compared these to 22 healthy controls and their parents. Patients and controls were subjected to clinical assessments, the symptoms checklist of the International Classification of Disease ICD (ICD-10), and the Wechsler Intelligence Scale for Children The parents of patients and controls underwent a general health questionnaire, the ICD-10 symptoms checklist, rating scales for anxiety and depression, post-traumatic stress disorder (PTSD) assessment scale, and the physical cognitive affective social economic ego problems (PCASEE) questionnaire for quality of life (QOL) rating. RESULTS Psychiatric morbidity was evident in nearly 60% of leukemic children and their parents and was significantly increased in comparison to controls. Children mostly suffered from adjustment and oppositional defiant disorders. The most common discriminators between patient groups were conduct and attention problems being lowest in newly diagnosed patients, and social aggression being lowest in patients in remission. The three parameters were highest in relapsed patients whose parents mostly had adjustment and depressive disorders. Risk factors for child psychopathology were older age, female gender, and parental psychopathology. Mothers and parents with lower education and professional level were found to be vulnerable. Performance and total intelligence quotient (IQ) were significantly lower in leukemic children, and these were most pronounced in the survivor group. Risk factors for cognitive dysfunction were younger age, longer chemotherapy duration, and lower parental education level. CONCLUSION Most patients and their caregivers suffered from significant psychiatric morbidity, highlighting the need for routine screening to improve psychological outcomes in such cases.

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

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 &bgr;-thalassemia major (&bgr;-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 &bgr;-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 &bgr;-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.

Endothelial monocyte activating polypeptide II in children and adolescents with type 1 diabetes mellitus: Relation to micro-vascular complications.

OBJECTIVES Endothelial monocyte-activating polypeptide II (EMAP II) is a multifunctional polypeptide with proinflammatory and antiangiogenic activity. Hyperglycemia and dyslipidemia appears to be significant factors contributing to increased EMAP-II levels. We determined serum EMAP II in children and adolescents with type 1 diabetes as a potential marker for micro-vascular complications and assessed its relation to inflammation and glycemic control. METHODS Eighty children and adolescents with type 1 diabetes were divided into 2 groups according to the presence of micro-vascular complications and compared with 40 healthy controls. High-sensitivity C-reactive protein (hs-CRP), hemoglobin A1c (HbA1c) and EMAP II levels were assessed. RESULTS Serum EMAP II levels were significantly increased in patients with micro-vascular complications (1539 ± 321.5 pg/mL) and those without complications (843.6 ± 212.6 pg/mL) compared with healthy controls (153.3 ± 28.3 pg/mL; p<0.001). EMAP II was increased in patients with microalbuminuria than normoalbuminuric group (p<0.001). Significant positive correlations were found between EMAP II levels and body mass index, fasting blood glucose, HbA1c, serum creatinine, triglycerides, total cholesterol, urinary albumin creatinine ratio (UACR) and hs-CRP (p<0.05). A cutoff value of EMAP II at 1075 pg/mL could differentiate diabetic patients with and without micro-vascular complications with a sensitivity of 93% and specificity of 82%. CONCLUSIONS We suggest that EMAP II is elevated in type 1 diabetic patients, particularly those with micro-vascular complications. EMAP II levels are related to inflammation, glycemic control, albuminuria level of patients and the risk of micro-vascular complications.

Renal iron deposition by magnetic resonance imaging in pediatric β-thalassemia major patients: Relation to renal biomarkers, total body iron and chelation therapy

BACKGROUND The reciprocal of multiecho gradient-echo (ME-GRE) T2* magnetic resonance imaging (MRI) R2*, rises linearly with tissue iron concentration in both heart and liver. Little is known about renal iron deposition in β-thalassemia major (β-TM). AIM To assess renal iron overload by MRI and its relation to total body iron and renal function among 50 pediatric patients with β-TM. METHODS Serum ferritin, serum cystatin C, urinary albumin creatinine ratio (UACR), and urinary β2-microglobulin (β2 M) were measured with calculation of β2 M/albumin ratio. Quantification of liver, heart and kidney iron overload was done by MRI. RESULTS Serum cystatin C, UACR and urinary β2 microglobulin as well as urinary β2m/albumin were significantly higher in β-TM patients than the control group. No significant difference was found as regards renal R2* between Patients with mean serum ferritin above 2500 μg/L and those with lower serum cutoff. Renal R2* was higher in patients with poor compliance to chelation therapy and positively correlated to indirect bilirubin, LDH, cystatin C and LIC but inversely correlated to cardiac T2*. CONCLUSION kidney iron deposition impairs renal glomerular and tubular functions in pediatric patients with β-TM and is related to hemolysis, total body iron overload and poor compliance to chelation.

One-Year Prospective Study of Community Acquired Influenza and Parainfluenza Viral Infections in Hospitalized Egyptian Children with Malignancy: Single Center Experience.

Background: Respiratory viruses are widespread in the community and easily transmitted to immunocompromised patients. Aims: Assess the prevalence of community-acquired respiratory viral infections among children with cancer presenting with clinical picture suggestive of lower respiratory tract infections (LRTIs), and evaluate its risk factors and prognosis. Methods: Over a year, 90 hospitalized children with malignancy and LRTIs recruited, subjected to clinical assessment, investigated through hematology panel, blood culture, chest x-ray, CT chest and PCR for influenza A and B, parainfluenza (PIV) types 1 and 3 viruses, and respiratory syncytial virus (RSV), and prospectively followed up for the clinical outcome. Results: Viral pathogens were identified in 34 patients (37.7%), with a seasonal peak from April to May. The most frequently detected virus was influenza virus [type A (16 cases; 47%), type B (4 cases; 12%)] followed by parainfluenza virus [PIV1 (9 cases; 26%), PIV3 (3 cases; 15%)], and none had RSV. Bacteria were identified in 26 patients, fungi in four, mixed infections [bacterial/viral and bacterial/fungal] in 13, and 36 cases had unidentified etiology. The majority of patients with influenza and parainfluenza infections had hematological malignancy, presented with fever, and had mild self-limited respiratory illness. Five patients with mixed viral and bacterial infection had severe symptoms necessitating ICU admission. Six patients died from infection-related sequelae; two had mixed PIV and Staphylococcal infections. Conclusions: Community acquired influenza and parainfluenza infections are common in pediatrics patients with malignancy, either as isolated or mixed viral/bacterial infections. Clinical suspicion is essential as hematological and radiological manifestations are nonspecific. Rapid diagnosis and management are mandatory to improve patients’ outcome.

Hazardous Influence of Hydroxyurea on Spermatogenesis in Thalassemia Intermedia Patients: An Egyptian Cohort Study

Background: Hydroxyurea (HU), frequently used in thalassemia intermedia (TI), might have adverse effects on spermatogenesis. Aim: To assess the effects of HU treatment on sperm parameters and potential reversibility on its discontinuation in TI patients. Methods: Twenty fully-pubertal male TI patients regularly followed-up at the Ain Shams University Thalassemia Center were classified according to previous HU treatment (1:1); first group had received HU for ≥ one year, while the second had never received HU. All recruited patients were subjected to full clinical assessments. Sperm parameters (number, abnormal forms, motility and forward progression) were assessed at enrollment and reassessed six- months after stopping HU treatment. Result: Eleven patients on HU therapy had statistically significant lower sperm count in comparison to those who had never received HU. At six months off HU therapy, there was statistically significant improvement of all sperm parameters. Nevertheless, such parameters were still lower than those of patients who had never received HU. Statistically significant relationships were noted between total sperm count and HU dose, compliance and duration of therapy. Conclusion: HU appears to have a hazardous yet reversible effect on sperm health in pubertal TI patients. Counseling should be offered with close follow-up of its effect on fertility.

Immunological role of CD4+CD28null T lymphocytes, natural killer cells, and interferon-gamma in pediatric patients with sickle cell disease: relation to disease severity and response to therapy

Sickle cell disease (SCD) is associated with alterations in immune phenotypes. CD4+CD28null T lymphocytes have pro-inflammatory functions and are linked to vascular diseases. To assess the percentage of CD4+CD28null T lymphocytes, natural killer cells (NK), and IFN-gamma levels, we compared 40 children and adolescents with SCD with 40 healthy controls and evaluated their relation to disease severity and response to therapy. Patients with SCD steady state were studied, focusing on history of frequent vaso-occlusive crisis, hydroxyurea therapy, and IFN-gamma levels. Analysis of CD4+CD28null T lymphocytes and NK cells was done by flow cytometry. Liver and cardiac iron overload were assessed. CD4+CD28null T lymphocytes, NK cells, and IFN-gamma levels were significantly higher in patients than controls. Patients with history of frequent vaso-occlusive crisis and those with vascular complications had higher percentage of CD4+CD28null T lymphocytes and IFN-gamma while levels were significantly lower among hydroxyurea-treated patients. CD4+CD28null T lymphocytes were positively correlated to transfusional iron input while these cells and IFN-gamma were negatively correlated to cardiac T2* and duration of hydroxyurea therapy. NK cells were correlated to HbS and indirect bilirubin. Increased expression of CD4+CD28null T lymphocytes highlights their role in immune dysfunction and pathophysiology of SCD complications.

Risk and prognostic factors for invasive fungal diseases in paediatric patients with malignancy

Background Invasive fungal diseases (IFDs) remain a challenge in the treatment of patients with haematological malignancies. Aims The aim of the study was to evaluate factors that are thought to favour the onset of IFDs and identify prognostic factors that could predict the eventual outcome of these infections. Patients and methods A systematic review of the literature was conducted for an explicit identification of risk and prognostics factors for IFDs in immunocompromised children. We retrospectively reviewed two age-matched and sex-matched patients with refractory acute myeloid leukaemia who had undergone allogeneic bone marrow transplantation. Results Both patients were exposed to the same hospital conditions and had been admitted for the first time at nearly the same period. They had two different paths as regards the development and outcome of IFD. Conclusion Several factors may have an impact on the onset and outcome of IFDs, and identification of these factors is essential to the evaluation and management of IFD and for timely establishment of appropriate antifungal therapy. Egyptian J Haematol 40:-0 ͹ 2015 The Egyptian Society of Haematology.