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Youssry, I., A. Abdel-Salam, R. Ismail, R. Bou-Fakhredin, R. M. Samy, F. Ezz El-Deen, and A. T. Taher, "Enhancing Effect of Hydroxyurea on Hb F in Sickle Cell Disease: Ten-Year Egyptian Experience.", Hemoglobin, vol. 41, issue 4-6, pp. 267-273, 2017 Jul - Nov. Abstract

Patients with sickle cell disease experience hemolytic anemia and vaso-occlusions that result in pain, organ injury, and premature mortality. Several prospective studies have verified the efficacy and tolerability of hydroxyurea (HU), and demonstrated its efficacy in reducing painful vaso-occlusive crises (VOCs) in addition to its ability to increase Hb F levels. We aimed to evaluate the long-term effects of HU therapy on Hb F and assess its long term efficacy and safety in sickle cell disease patients. A retrospective study on 60 sickle cell disease patients was conducted. We studied the laboratory changes, frequency of VOCs per year, frequency of hospital admisions per year and number of transfusions per year, both before and after HU therapy. The follow-up period was 4 to 120 months. Hb F levels after HU therapy positively correlated with the duration of HU therapy, baseline Hb F levels and baseline total hemoglobin (Hb) (r = 0.4, p = 0.04; r = 0.45, p = 0.001; r = 0.5, p = 0.019, respectively) and inversely correlated with baseline total leucocyte count (r = -0.33, p = 0.034). Hydroxyurea therapy was associated with an increase in the total Hb and mean corpuscular volume (MCV) (p = 0.009, p = 0.000; respectively) and with a decrease in total leucocyte count, platelet count and reticulocyte count (p = 0.00, p = 0.03, p = 0.02, respectively). Moreover, a significant reduction in the frequency of VOCs, transfusion frequency and hospital admissions per year after HU therapy was shown in the studied subjects. Hydroxyurea induced an increase in Hb F level, which was maintained over time and was associated with clinical efficacy and acceptable safety.

A, I., A. A, M. RI, and F. MA, "1) Iron therapy and anthropometry: A case-control study among iron deficient preschool children.", Egyptian Ped. Association Gazette , vol. 65, issue 3, pp. 95-100, 2017. iron_def._anemia_article_2017.pdf
El Kholy, A. A., N. A. Mostafa, A. A. Ali, M. M. S. Soliman, S. A. El-Sherbini, R. I. Ismail, N. El Basha, R. I. Magdy, N. El Rifai, and D. H. Hamed, "The use of multiplex PCR for the diagnosis of viral severe acute respiratory infection in children: a high rate of co-detection during the winter season.", European journal of clinical microbiology & infectious diseases : official publication of the European Society of Clinical Microbiology, vol. 35, issue 10, pp. 1607-13, 2016 Oct. Abstract

Respiratory tract infection is a major cause of hospitalization in children. Although most such infections are viral in origin, it is difficult to differentiate bacterial and viral infections, as the clinical symptoms are similar. Multiplex polymerase chain reaction (PCR) methods allow testing for multiple pathogens simultaneously and are, therefore, gaining interest. This prospective case-control study was conducted from October 2013 to February 2014. Nasopharyngeal (NP) and oropharyngeal (throat) swabs were obtained from children admitted with severe acute respiratory infection (SARI) at a tertiary hospital. A control group of 40 asymptomatic children was included. Testing for 16 viruses was done by real-time multiplex PCR. Multiplex PCR detected a viral pathogen in 159/177 (89.9 %) patients admitted with SARI. There was a high rate of co-infection (46.9 %). Dual detections were observed in 64 (36.2 %), triple detections in 17 (9.6 %), and quadruple detections in 2 (1.1 %) of 177 samples. Seventy-eight patients required intensive care unit (ICU) admission, of whom 28 (35.8 %) had co-infection with multiple viruses. AdV, HBoV, HRV, HEV, and HCoV-OC43 were also detected among asymptomatic children. This study confirms the high rate of detection of viral nucleic acids by multiplex PCR among hospitalized children admitted with SARI, as well as the high rate of co-detection of multiple viruses. AdV, HBoV, HRV, HEV, and HCoV-OC43 were also detected in asymptomatic children, resulting in challenges in clinical interpretation. Studies are required to provide quantitative conclusions that will facilitate clinical interpretation and application of the results in the clinical setting.

Makhlouf, M. M., and R. I. Magdy, "The clinical relevance and prognostic significance of microsomal epoxide hydrolase gene polymorphisms and their susceptibility to acquired aplastic anemia: an Egyptian study.", Biomarkers : biochemical indicators of exposure, response, and susceptibility to chemicals, vol. 21, issue 5, pp. 416-23, 2016 Jul. Abstract

BACKGROUND: Microsomal epoxide hydrolase enzyme (mEPHX) is involved in xenobiotics detoxification. Two variants of mEPHX, Tyr113His and His139Arg, have been described. Both may lead to acquired aplastic anemia (AA).

OBJECTIVES: Assessing mEPHX genetic polymorphisms and detecting their impact on susceptibility and prognosis in Egyptian AA patients.

PARTICIPANTS AND METHODS: mEPHX 113 and 139 genotypes were determined by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) in 100 patients with AA and 100 control subjects.

RESULTS: Both mEPHX Tyr113His and His139Arg gene polymorphisms were associated with increased risk of developing AA, and have a significant impact of bad prognosis (p value < 0.01).

CONCLUSIONS: These mEPHX gene polymorphisms can be considered as risk factors and predictive molecular markers for prognosis in AA patients.

El-Beshlawy, A., M. El-Ghamrawy, M. A. El-Ela, F. Said, S. Adolf, A. - R. A. Abdel-Razek, R. I. Magdy, and A. Abdel-Salam, "Erratum to: Response to hydroxycarbamide in pediatric β-thalassemia intermedia: 8 years' follow-up in Egypt.", Annals of hematology, vol. 95, issue 5, pp. 851, 2016 Apr.
Radwan, E. R., and R. L. M. Goda, "Lack of impact of cytotoxic T-lymphocyte antigen 4 gene exon 1 polymorphism on susceptibility to or clinical course of egyptian childhood immune thrombocytopenic purpura.", Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis, vol. 21, issue 4, pp. 378-82, 2015 May. Abstract

Dysfunctional T-lymphocyte immunity plays an important role in the pathophysiology of immune thrombocytopenic purpura (ITP). Cytotoxic T-lymphocyte antigen 4 (CTLA-4)-a surface marker expressed on T regulatory cells and activated T lymphocytes-is a negative modulator of T-cell responses. Polymorphisms of the CTLA-4 may alter the level of antigen expression and hence may influence immune regulation. The study aimed to evaluate the possible contribution of CTLA-4 exon 1 49 A>G polymorphism to the pathogenesis of ITP and its relation to age of disease onset, clinical course, and response to therapy. Genotyping of CTLA-4 exon 1 49 A>G was performed in 100 pediatric patients with ITP and 259 healthy individuals by polymerase chain reaction-restricted fragment length polymorphism. No significant differences existed in genotype or allele distributions between patients and controls for the studied polymorphism. Comparable genotypes and allele frequencies were obtained between the 2 groups after their stratification by age of disease onset, clinical course, or response to therapy. In conclusion, CTLA-4 exon 1 49 A>G polymorphism is not associated with susceptibility to ITP in the Egyptian population; neither it affects the clinical picture of the disease.

El Kholy, A. A., N. A. Mostafa, A. A. Ali, S. A. El-Sherbini, R. I. Ismail, R. I. Magdy, M. S. Soliman, and M. M. Said, "Risk factors of prolonged hospital stay in children with viral severe acute respiratory infections.", Journal of infection in developing countries, vol. 8, issue 10, pp. 1285-93, 2014 Oct 15. Abstract

INTRODUCTION: Severe acute lower respiratory infections (SARIs) are one of the major causes of morbidity and mortality in young children, especially in developing countries. The present study focused on detection of risk factors for prolonged hospital stays among children with viral SARIs.

METHODOLOGY: A sentinel surveillance study was conducted at Cairo University Hospital (CUH) between February 2010 and May 2011. Nasopharyngeal (NP) and oropharyngeal (OP) swabs were collected from all children admitted with SARIs. Viruses were identified using reverse transcription polymerase chain reaction (RT-PCR).

RESULTS: Out of 1,046 children, 380 (36%) were positive for one or more viruses; these included respiratory syncytial virus (RSV) (22.9%), adenovirus (6.2%), parainfluenza viruses (PIVs1-3) (5.1%), human metapneumovirus (HMPV) (4.5%), influenza A (1.4%), and influenza B (0.6%). Viral etiology was mainly detected in children under one year of age (88.9%). Prolonged length of stay was independently associated with the presence of cyanosis and underlying chronic illness (OR 7.4, CI: 1.8-30.32 [p = 0.005], OR 2.5, CI: 1.36-4.64 [p = 0.004], respectively). Virus type did not affect the length of hospital stay (p > 0.05). Oxygen therapy was required in 91% of the patients. A total of 43 patients (11.6%) required intensive care admission. Twenty-one patients (5.5%) died, and 15 of them (71.4%) had an underlying chronic illness.

CONCLUSIONS: The study demonstrated the important burden of respiratory viruses as a cause of SARI in hospitalized children in a tertiary Egyptian hospital. Cyanosis and underlying chronic illness were significantly associated with prolonged length of stay.

El-Beshlawy, A., M. El-Ghamrawy, M. A. El-Ela, F. Said, S. Adolf, A. - R. A. Abdel-Razek, R. I. Magdy, and A. Abdel-Salam, "Response to hydroxycarbamide in pediatric β-thalassemia intermedia: 8 years' follow-up in Egypt.", Annals of hematology, vol. 93, issue 12, pp. 2045-50, 2014 Dec. Abstract

Hydroxycarbamide (hydroxyurea or HU) has been shown to increase fetal hemoglobin (HbF) in patients with β-thalassemia intermedia (TI). The reported effects of HU in increasing the total hemoglobin (Hb) have been inconsistent. Studies of long-term therapy with HU in pediatric TI are rather uncommon. A retrospective observational study was carried out to evaluate the clinical responses to HU in Egyptian patients with β-TI. One hundred patients; children (n = 82, mean age 9.9 ± 4.1 years) and adults (n = 18) were studied for the mean Hb, HbF%, median serum ferritin, transfusion history, and splenic size before and after HU therapy (mean dose 20.0 ± 4.2 mg/kg/day, range 10-29 mg/kg/day) over a follow-up period 4 to 96 months (mean 35.4 ± 19.2 months). Molecular studies were also done for group of patients (n = 42). The overall response rate to HU was 79 %; 46 % were minor responders (with a reduction in transfusion rate by 50 % or more and/or an increase in their total hemoglobin level by 1-2 g/dl) and 33 % major responders (becoming transfusion-free and/or having an increase in total hemoglobin level by >2 g/dl). Mean hemoglobin increased among responders from 6.9 ± 0.9 g/dl to 8.3 ± 1.4 g/dl (p < 0.001). A significant rise in mean HbF (27.0 vs. 42.5 %; p < 0.011) and a decrease in median serum ferritin (800 vs. 644 ng/ml; p < 0.001) were also observed among responders (n = 45). Transfusions stopped in 44 % of pretreatment frequently transfused responders (n = 11/25). Splenic size decreased in 37 % of patients (n = 30/81). The predominant β-thalassemia mutation was 1-6 (T > C) in 32/42 (76 %) of studied patients; 28/32 were responders. Bivariate analysis showed no predictors of response as regards sex, pediatric and adult age, splenic status, or genotype. Hydroxycarbamide is a good therapeutic modality in the management of pediatric as in adult TI patients. It can minimize the need for blood transfusion, concomitant iron overload, and blood-born viral transmission especially in developing countries like Egypt.

MS, A. W., F. H, I. R, and M. N, "Recurrent epistaxis in children: when should we suspect coagulopathy?", Egypt J Otolaryngol, vol. 30, issue 2, pp. 106-111, 2014.
Radwan, E. R., E. A. Youssef, N. I. Sewelam, R. I. M. Goda, and H. H. Arnaout, "Study of T-cell immunoglobulin- and mucin-domain-containing molecule 3 polymorphisms in Egyptian childhood immune thrombocytopenic purpura", Comparative Clinical Pathology, vol. 23, issue 4, pp. 933–939, 2014.
Seif El Dien, H. M., R. I. Esmail, R. E. Magdy, and H. M. Lotfy, "Deferoxamine-induced dysplasia-like skeletal abnormalities at radiography and MRI.", Pediatric radiology, vol. 43, issue 9, pp. 1159-65, 2013 Sep. Abstract

BACKGROUND: Current thalassemia major treatment includes blood transfusion and iron chelation, which is associated with growth disturbances and radiographic changes in the long bone metaphyses.

OBJECTIVE: To explore and discuss the spectrum of deferoxamine-induced bone-dysplasia-like changes in children with thalassemia major in Egypt.

MATERIALS AND METHODS: We studied 59 Egyptian children with thalassemia major and generalized arthralgia. All started deferoxamine treatment at 3 years of age. We conducted skeletal survey and MRI of both knees in radiographically positive children. Each child's age, serum ferritin, age of onset and duration of therapy were compared with the radiologic findings.

RESULTS: Twenty-two (37.3%) children had variable degrees of skeletal dysplasia-like changes similar to those described with deferoxamine intake, mostly around the knees. Mild dysplasia-like changes were seen in 4 (18%) children; moderate changes were seen in 11 (50%) children and severe changes were seen in 7 (31.8%) children. No statistically significant relationships were detected between bone changes and the children's age, age of starting deferoxamine, duration of therapy, or serum ferritin level.

CONCLUSION: A wider spectrum of deferoxamine-induced bone-dysplasia-like changes was recognized despite delayed onset and small doses of therapy. These changes should be considered as a possible cause of arthropathy in children with thalassemia major, especially symptomatic children.

El Kholy, A. A., N. A. Mostafa, S. A. El-Sherbini, A. A. Ali, R. I. Ismail, R. I. Magdy, M. S. Hamdy, and M. S. Soliman, "Morbidity and outcome of severe respiratory syncytial virus infection.", Pediatrics international : official journal of the Japan Pediatric Society, vol. 55, issue 3, pp. 283-8, 2013 Jun. Abstract

BACKGROUND: Respiratory syncytial virus (RSV) is the main cause of severe acute respiratory infection (SARI) in infants and young children. This study aimed to identify risk factors for intensive care unit (ICU) admission, prolonged length of stay (PLOS), and mortality in patients hospitalized with SARI caused by RSV.

METHODS: This prospective cohort study included children hospitalized with SARI (according to the World Health Organization definition) and whose laboratory results proved RSV infection during the period from February 2010 to May 2011.

RESULTS: Out of 240 enrolled patients, 24 patients (10%) were admitted to the ICU, 57 patients (24.3%) had a PLOS of >9 days and 12 patients (5%) died. The presence of cyanosis (P = 0.000; OR, 351.7) and lung consolidation (P = 0.006, OR, 9.3) were independent risk factors associated with ICU admission. The need for ICU admission (P = 0.000; OR, 6.1) and lung consolidation (P = 0.008, OR, 2.46) were independent risk factors associated with PLOS. The presence of an underlying congenital heart disease (P = 0.03, OR, 18.3), thrombocytopenia (P = 0.04, OR, 32.86) and mechanical ventilation (P = 0.000; OR, 449.4) were the only independent risk factors associated with mortality in our study.

CONCLUSIONS: Early recognition of risk factors for complicated RSV disease on admission prompts early interventions and early ICU admissions for these children.

M, Z., E. RI, and M. RI, "Cardiac functions in Deferoxamine or Deferiprone treated patients with Thalassemia Major", Journal of Arab Child (J A C), vol. 23, issue 1, 2012.
HH, A. - A., A. S, and M. RI, "Analysis of G71D Mutation of HAMP Gene and H63D Mutation of HFE Gene in B-Thalassemia Major Patients.", The J Egy Society of Haemat. & Res, vol. 7, issue 1, 2011.
HM, S. E. D., E. RI, and M. RE, "The Role of CT Angiography in Evaluation of Congenital Extra Cardiac vascular Anomalies", The Egyptian Journal of Radiology and Nuclear Medicine, vol. 41, issue 1, pp. 149-158, 2010.
HM, S. E. D., E. RI, and M. RE, "Spectrum of Congenital Anomalies of Pulmonary Arteries (Evaluation by CT Angiography)", The Egyptian Journal of Radiology and Nuclear Medicine, vol. 41, issue 2, pp. 171-180, 2010.