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Hematuria in Patients With Beta-Thalassemia Major  

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Introduction. Our information about renal involvement in beta-thalassemia major is limited. Recently, few studies have reported proteinuria, hypercalcuria, phosphaturia, and oversecretion of tubular damage markers; however, hematuria has not yet been meticulously studied in these patients. We investigated hematuria in patients with beta-thalassemia major.Materials and Methods. Urinalysis was performed in 500 patients with beta-thalassemia major under a regular blood transfusion program. In th...

Mohammad Hossein Fallahzadeh; Mohammad Kazem Fallahzadeh; Mehdi Shahriari; Shervin Rastegar; Ali Derakhshan; Mohammad Amin Fallahzadeh

2010-01-01

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Scintigraphic evaluation of bone involvement in beta thalassemia major  

International Nuclear Information System (INIS)

In this article, the authors describe a 19 year old man with beta-thalassemia major and back pain, who was studied with a Tc99m Metilendiphosphonate (Tc99m MDP) bone scan. This case shows many of the complications of beta-thalassemia major in the skeletal system. Also we can see some indirect signs of endocrinopathies associated (Au)

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Portal vein thrombosis after splenectomy for beta-thalassemia major  

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Portal vein thrombosis is a recognized complication after splenectomy for beta-thalassemia major due to the chronic hypercoagulable state which has been recognized to exist in childhood thalassemia and contribute to thromboembolic events. We reporting one patient with beta-thalassemia major developed portal vein thrombosis following splenectomy. (author)

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Hyperhemolysis in a patient with beta-thalassemia major.  

Science.gov (United States)

A case of hyperhemolysis in a 2-year-old boy with beta thalassemia major was noted. After several transfusions, he developed hyperhemolysis with a positive (C3d only) direct antiglobulin test (DAT) and no clinically significant RBC allo- or auto-antibodies. (There was a weak cold antibody, showing a narrow thermal range). Because there was no significant improvement with steroid and immunoglobulin infusions, cyclophosphamide therapy was tried with notable success. PMID:20041094

Morawakage, Lakmali R; Perera, B J C; Dias, P D N; Wijewardana, S K

2009-01-01

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Skeletal Changes in Patients with BetaThalassemia Major in Ahvaz  

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Full Text Available Background and Objective: Thalassemia major has severe clinical symptoms with craniofacial defects that produce esthetic problems in patients. Orthodontic treatment and surgical reconstruction in these patients have had good esthetic results and therefore satisfying pschycosocial effects. Researches have shown an increase in the level of life quality corresponding to health improvement in thalassemic patients. More knowledge and information is necessary for better treatment of skeletal problems in thalassemia. Subjects and Methods: This descriptive and analytic study was performed on 48-beta thalassemia major patient and 48 normal samples. They were divided into two groups (24 persons based on their genders. Including criteria for samples were : being more than 15 years old, not being affected by special disorders, not having orthodontic and orthopedic treatment, having Cl I profile, normal facial height, Cl I molar relationship, normal overbite and over jet and the presence of all permanent teeth (except third molar. Cephalometric analysis was used for evaluation of cephalograms. The results were analyzed using descriptive statistical and paired sample T-test (P<0.005. Results: SNA angle did no significant increase, but SNB and ANB angles significantly decreased (P<0.005. The angle between SN-Occlusal plans, Frankfort - y-axis plans and basal plans showed increase, but the angle between Frankfort - facial plans (P<0.005 and the distance between pog-NB plan showed decrease (P<0.005. Conclusion: Beta thalassemia major induces Cl II malocclusion and long face growth pattern due to decrease in mandibular growth and decrease in posterior facial height. ?Please cite this paper as: Khanehmasjedi M, Bassir L Mombeyni M. Skeletal Changes in Patients with Beta - Talassemia Major in Ahvaz. JundishapurSci Med J. 2012;11(3:295-302

Mashaalah Khanehmasjedi

2012-08-01

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Zinc and Copper Status in Children with Beta-Thalassemia Major  

OpenAIRE

Objective: There are some reports in which a condition of zinc deficiency and its associated outcomes with a change in concentration of serum copper among the thalassemic patients has been highlighted. The aim of this prospective study was to determine the serum zinc and copper levels in children with beta-thalassemia major.Methods: In this cross sectional study all children under 12 years affected by beta thalassemia major (40 patients) were evaluated for serum zinc and copper levels in Qazv...

Hoshyar Mojabi; Ali-Asghar Pahlevan; Parviz Ayazi; Abolfazl Mahyar; Mohammad-Reza Sehhat; Amir Javadi

2010-01-01

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Economic burden of beta-thalassemia/Hb E and beta-thalassemia major in Thai children  

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Full Text Available Abstract Background Hemoglobin E beta-thalassemia (?-thalassemia/Hb E has a variable severity, and the cost of treatment has not been well studied. The aim of this study was to analyze the societal cost of caring for children with ?-thalassemias in Thailand. The study was designed as a prevalence-based cost-of-illness analysis in a societal perspective. Medical records from three public hospitals of children aged 2-18 years with ?-thalassemia/Hb E and homozygous ?-thalassemia were reviewed for direct medical cost determination. For direct non-medical cost and indirect cost, a family member was interviewed. Findings It was found that 201 patients with ?-thalassemia/Hb E (91% and homozygous ?-thalassemia (9% were recruited for this study. Ninety-two (46% were severe thalassemia and 109 (54% were mild to moderate severity. The annual average cost of treatment was US$950; 59% was direct medical cost, 17% direct non-medical cost, and 24% indirect cost. The costs were differentiated by some potential predictors. Significant predictor variables were: hospital, health insurance scheme, blood transfusion pattern, and iron chelation drug use. Conclusions The average annual cost per patient was calculated, and the cost model was estimated. These would be applied for national planning, economic evaluation of treatment and prevention interventions, and budget impact analysis.

Riewpaiboon Arthorn

2010-01-01

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Assessment of Thyroid Function in Children Aged 1-13 Years with Beta-Thalassemia Major  

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Objective:Hypothyroidism usually appears in the second decade of life and is thought to be associated with iron overload in patients with thalassemia major. This study aimed to evaluate thyroid dysfunctions in patients with beta-thalassemia major and to see if they appear in the earlier period of life. Methods:Thyroid function and iron load status were evaluated in 90 children with a mean age of 7.17±3.78 years with beta-thalassemia major by measuring serum free thyroxin (FT4), serum f...

Nurcan Beyaz?t; Deniz Gökalp; Turgay Deniz; Ayfer Gözü Pirinççio?lu; Kenan Haspolat; Murat Söker

2011-01-01

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Evaluation of pulmonary function in beta-thalassemia major patients  

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Objective: To describe and quantify the functional change of the lung in patients with beta-thalassemia major (TM) and determine the correlation between pulmonary function test (PFT) results with hemoglobin, ferritin and age changes. Methodology: Pulmonary function tests were performed on 60 transfusion-dependent patients with TM, ranging in age from 10 to 45 years. Percent-predicted values for forced expiratory volume in one second (FEV1), and forced expiratory flows (FEF) 25-75% were significantly reduced, whereas forced expiratory vital capacity (FVC) and FEV1/FVC were closed to normal limits, indicating a restrictive disease. All factors including; FVC, FEV1, and FEV1/FVC, FEF 25-75% were negatively correlated with age and ferritin levels. In contrast, all factors including; FVC, FEV1, and FEV1/FVC, FEF 25-75% were positively correlated with hemoglobin (Hb). We performed linear regression analysis to study the simultaneous influence of the presence of age, ferritin, and Hb on obstructive PFT indexes. Results: Pulmonary function test results were normal in only 32 (53.3%) of 60 patients and the rest 28 cases (46.7%) showed abnormal pulmonary function. FEV1 and FEF 25% - 75% have significant negative correlation with age (r = - 0.64 p(r) = 0.003 and r = - 0.58 p(r) = 0.02 respectively), also have significant positive correlation with Hb (r = 0.31 p(r) = 0.015 and r = 0.33 p(r) = 0.01 respectively), and only FEF 25% - 75% has significant negative correlation with fer significant negative correlation with ferritin (r -0.26 p(r) = 0.04). Conclusion: The present study has shown that restrictive disease and reduced lung diffusing capacity are the predominant abnormalities of pulmonary function patients with TM. The low hemoglobin concentration and a fall in the diffusing capacity of the alveola - capillary membrane, together with the dependence of the reduced pulmonary diffusing capacity on age and serum ferritin levels, as well as of the entity of restrictive disease on age, suggests that pulmonary dysfunctions in patients with TM are due mainly to lung fibrosis and/or interstitial edema related to iron overload. (author)

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Serum Immunoglobulin Levels in Splenectomized and Non-Splenectomized Patients with Major Beta-Thalassemia  

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Full Text Available Objective:Thalassemia is a common disease in many countries, in which several complications such as infections can occur. Although aberration in the function of the immune system could be a reason for such complication, a little is known about the status of humoral immune system in major beta thalassemia. In this study we measured serum immunoglobulins level in a group of patients with major beta thalassemia.Methods:Ninety nine patients with major beta thalassemia were enrolled in this study divided into two groups of splenctomized and not splenctomized patients. Serum IgG, IgM and IgA levels of these patients were measured and analyzed.Findings:Serum mean levels of IgG and IgM in patients of all ages in both groups were normal. The mean serum IgA level in the group of not splenectomized patients aged less than five years as well as in the splenectomized patients aged more than twenty years was increased. However, it was normal in other age groups.Conclusion:Although this study could not show any defect in the humoral immune system, evaluation of immunoglobulins could be useful to understand the relmarkable high rate of infection in the patients with major beta thalassemia.

Mojgan Kiani-amin

2011-03-01

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Renal function in children with beta-thalassemia major and thalassemia intermedia.  

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In beta-thalassemia, profound anemia and severe hemosiderosis cause functional and physiological abnormalities in various organ systems. In recent years, there have been few published studies demonstrating proteinuria, aminoaciduria, low urine osmolality, and excess secretion of the tubular damage markers, such as urinary N-acetyl-D-glucosaminidase (U(NAG)) and beta2 microglobulin, in patients with thalassemia. The object of this study was to analyze renal tubular and glomerular function in pediatric patients with beta-thalassemia and to correlate the renal findings to iron overload. Thirty-seven patients with beta-thalassemia major and 11 with thalassemia intermedia were studied. Twelve children without iron metabolism disorders or renal diseases served as a control group. No difference in blood urea nitrogen (BUN), serum creatinine, creatinine clearance, electrolytes, fractional excretion of sodium and potassium, and tubular phosphorus reabsorption was found. Serum uric acid was equal in the two groups, but its urine excretion was significantly higher in the thalassemic group. U(NAG) and U(NAG) to creatinine ratio (U(NAG/CR)) were elevated in all patients with thalassemia compared with the control group (p ferritin level. We found that renal tubular function is impaired in children with beta- thalassemia major and intermedia. It is not known whether these functional abnormalities would have any long-term effects on the patients. Further studies are needed, and means of preventing these disturbances should be sought. PMID:18581145

Smolkin, Vladislav; Halevy, Raphael; Levin, Carina; Mines, Miguel; Sakran, Waheeb; Ilia, Katzap; Koren, Ariel

2008-10-01

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Frequency of Sensory Neural Hearing Loss in Major Beta-Thalassemias in Southern Iran  

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Objective:The thalassemias are among the most common genetic disorders worldwide, occurring more frequently in the Mediterranean region. The aim of this study was to determined frequency of sensory-neural hearing loss in major ß- thalassemias transfusion dependent patients in south of Iran. Methods:A cross sectional study on 308 cases of major beta-thalassemia patients referring to Thalassemia Center of Shiraz University of Medical Sciences between 2006-2007 years. The diagnosis of ß- thala...

Abolhassan Faramarzi; Mehran Karimi; Seyed-Taghi Heydari; Mahmoud Shishegar; Masoud Kaviani

2010-01-01

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A cross-sectional study of metabolic and endocrine complications in beta-thalassemia major  

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Iron overload is a major problem in patients with beta-thalassemia major, and it has many structural and metabolic consequences. The aim of this study was evaluation of endocrine disturbances in patients with beta-thalassemia major who were older than 10 years of age. In this cross-sectional study, investigator collected demographic data and medical histories, as well as menstrual history in females, from the medical records of 56 patients with beta-thalassemia major. Patients were examined to determine their pubertal status and the standard deviation score for height for evaluation of short stature. For evaluation of glucose tolerance, a fasting blood glucose and oral glucose tolerance tests were performed. Evidence for diabetes mellitus was based on American Diabetes Association and World Health Organization criteria. Serum levels of calcium, phosphorous, thyroid-stimulating hormone, free thyroxin, luteinizing hormone and follicular-stimulating hormone and estradiol in girls and testosterone in boys were measured. The mean and standard deviation for age in the 56 patients (36 males and 20 females) was 15.62+-4.44 years. Diabetes mellitus was present in 5 patients (8.9%), impaired fasting glucose was found in 16 patients (28.6%) and an impaired glucose tolerance test was found in 4 patients (7.1%). Short stature (standard deviation score <-2) was seen in 25 (70%) boys and 14 (73%) girls. Impaired puberty was found in 40 patients (71%). Hypocalcaemia and primary overtnts (71%). Hypocalcaemia and primary overt hyperthyroidism were present in 23 (41%) and 9 patients (16%), respectively. Only eight patients (14.3%) had no endocrine abnormalities. Despite therapy with deferoxamine to treat iron overload, the risk of secondary endocrine dysfunction remained high. Hypogonadism was one of the most frequent endocrine complications. Impaired glucose tolerance, short stature, hypocalcemia, subclinical and overt hypothyroidism are also frequent. (author)

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A cross-sectional study of metabolic and endocrine complications in beta-thalassemia major  

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Full Text Available Background and Objectives: Iron overload is a major problem in patients with beta-thalassemia major, and it has many structural and metabolic consequences. The aim of this study was evaluation of endocrine disturbances in patients with beta-thalassemia major who were older than 10 years of age. Patients and Methods: In this cross-sectional study, investigators collected demographic data and medical histories, as well as menstrual history in females, from the medical records of 56 patients with beta-thalassemia major. Patients were examined to determine their pubertal status and the standard deviation score for height for evaluation of short stature. For evaluation of glucose tolerance, a fasting blood glucose and oral glucose tolerance test were performed. Evidence for diabetes mellitus was based on American Diabetes Association and World Health Organization criteria. Serum levels of calcium, phosphorous, thyroid-stimulating hormone, free thyroxin, luteinizing hormone and follicular-stimulating hormone, and estradiol in girls and testosterone in boys were measured. Results: The mean and standard deviation for age in the 56 patients (36 males and 20 females was 15.62±4.44 years. Diabetes mellitus was present in 5 patients (8.9%, impaired fasting glucose was found in 16 patients (28.6% and an impaired glucose tolerance test was found in 4 patients (7.1%. Short stature (standard deviation score Conclusion: Despite therapy with deferoxamine to treat iron overload, the risk of secondary endocrine dysfunction remained high. Hypogonadism was one of the most frequent endocrine complications. Impaired glucose tolerance, short stature, hypocalcemia, subclinical and overt hypothyroidism are also frequent.

Najafipour Farzad

2008-01-01

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Assessment of Thyroid Function in Children Aged 1-13 Years with Beta-Thalassemia Major  

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Full Text Available Objective: Hypothyroidism usually appears in the second decade of life and is thought to be associated with iron overload in patients with thalassemia major. This study aimed to evaluate thyroid dysfunctions in patients with beta-thalassemia major and to see if they appear in the earlier period of life.Methods: Thyroid function and iron load status were evaluated in 90 children with a mean age of 7.17±3.78 years with beta-thalassemia major by measuring serum free thyroxin (FT4, serum free triiodothyronine (FT3, total thyroxin (T3, serum total triiodothyronine (T4, thyroid-stimulating hormone (TSH and ferritin levels from serum of patients admitted to the Pediatric Department, Faculty of Medicine University of Dicle between March 2005 and July 2009. A control group formed from an age-sex matched healthy children with a mean age of 6.98±3.66 years was also included. A standard thyrotropin releasing hormone test was applied to 3 patients who had high TSH levels and were classified as subclinical primer hypothyroidism. The study was designed according to the Declaration of Helsinki and informed consent was obtained from the parents of all participants.Findings: All thyroid parameters in patients were in the normal ranges compared with the controls except three of them which had high TSH levels. Serum ferritin level (2703±1649 ng/mL in patients was significantly higher than in controls (81.5±15.5 ng/mL.Conclusion: The work implies that hypothyroidism could be even seen in the first decade of life in patients with beta-thalassemia major in spite of improved hematological cares.

Ayfer Gozu Pirinccioglu

2011-03-01

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Noninvasive analysis of skin iron and zinc levels in beta-thalassemia major and intermedia  

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Diagnostic x-ray spectrometry, a method based on x-ray fluorescence analysis, was used for noninvasive determination of iron and zinc in two distinct skin areas, representing predominantly dermal and epidermal tissues, in 56 patients with beta-thalassemia major and intermedia. The mean iron levels in the skin of patients with beta-thalassemia major and intermedia were elevated by greater than 200% and greater than 50%, respectively, compared with control values. The zinc levels of both skin areas examined were within the normal range. The data indicate that the rate and number of blood transfusions, which correlated well with serum ferritin levels (r . 0.8), are not the only factors that determine the amount of iron deposition in the skin (r less than 0.6). Other sources of iron intake contribute to the total iron load in the tissues, particularly in patients who are not given multiple transfusions. The noninvasive quantitation of skin levels may reflect the extent of iron deposition in major parenchymal organs. Repeated DXS examinations of the skin could monitor the clearance of iron from the tissues of patients with iron overload in the course of therapy with chelating agents.

Gorodetsky, R.; Goldfarb, A.; Dagan, I.; Rachmilewitz, E.A.

1985-01-01

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Noninvasive analysis of skin iron and zinc levels in beta-thalassemia major and intermedia  

International Nuclear Information System (INIS)

Diagnostic x-ray spectrometry, a method based on x-ray fluorescence analysis, was used for noninvasive determination of iron and zinc in two distinct skin areas, representing predominantly dermal and epidermal tissues, in 56 patients with beta-thalassemia major and intermedia. The mean iron levels in the skin of patients with beta-thalassemia major and intermedia were elevated by greater than 200% and greater than 50%, respectively, compared with control values. The zinc levels of both skin areas examined were within the normal range. The data indicate that the rate and number of blood transfusions, which correlated well with serum ferritin levels (r . 0.8), are not the only factors that determine the amount of iron deposition in the skin (r less than 0.6). Other sources of iron intake contribute to the total iron load in the tissues, particularly in patients who are not given multiple transfusions. The noninvasive quantitation of skin levels may reflect the extent of iron deposition in major parenchymal organs. Repeated DXS examinations of the skin could monitor the clearance of iron from the tissues of patients with iron overload in the course of therapy with chelating agents

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Nitroblue tetrazolium test in patients with beta-thalassemia major  

International Nuclear Information System (INIS)

Objective to assess the neutrophil function in thalassemia major (TM)patients and compare it with the control group and to recognize its relevantfactors. This was a retrospective cohort study, which was carried out fromOctober 2007 to February 2008 in the Thalassemia research Center in Boo AliSina Hospital in Sari, Mazandaran, North of Iran. The study populationconsisted of TM patients in Boo Ali Sina Teaching Hospital. The method ofsampling in the case group was systematic and it was target based in thecontrol group. The sample size determined was based on previous studies.Thalassemia major was diagnosed based on hemoglobin electrophoresis (casegroup). The control group was their brothers and sisters, who had +-5 yearsof age difference and were of the same gender as the patients. Datacollection was based on interview, investigating demographic characteristicsand also obtaining medical information from medical records of the patients.The neutrophil function was by performing nitroblue tetrazolium (NBT)reduction test. The test was carried out on both groups and the data wereanalyzed by software using SPSS version 13.0. In this study, 39 patients and39 healthy controls were compared. The average age of the patients was21.6+-5.3 years and it was 22.4+-5.1 years in healthy controls (p=0.7). Therewas a significant correlation between the test's results and the patients age(p=0.008). The rate of impaired NBT results in the patients was 36%, while itwas 10% in controls, whicwas 36%, while itwas 10% in controls, which were significantly different. The neutrophilactivity based on NBT test was 89.9+-11.6% in the case group and 93.7+-2.51%in the control group (p=0.025). This study indicates that neutrophil activityin thalassemia patients was significantly lower, compared to the normalcontrol group, especially in young patients. Based on the results, evaluationof neutrophil function and pyogenic infections in TM patients seemsnecessary. (author)

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High Prevalence of Hypoparathyroidism in Patients with beta-Thalassemia Major  

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Full Text Available "nIntroduction: Hypoparathyroidism (HPT is an irreversible but preventable disorder caused by an iron overload which can be considered a typical complication in patients with beta-thalassemia major. "nPatients and method: Parathyroid function was evaluated in 130 patients in Qom, Iran, who suffered from beta-thalassemia major. Their serum ferritin levels were checked for monitoring of chelation therapy effects. "nResults: The prevalence of HPT was 14.6% (19/130. The median age of patients with HPT was significantly higher than of patients without HPT (18 vs. 15 years; P=0.03. Serum ferritin levels was not significantly different between the two groups (median: 2709 vs. 1512; P=0.95. The prevalence of cardiac diastolic dysfunction in patients with HPT was significantly higher than in normal thalassemic patients (3.1% vs. 15.8%, P=0.04. Patients with hypoparathyroidism demonstrated abnormal glucose metabolism (0% vs. 15.8%; P=0.003. "nConclusion: the high prevalence of hypoparathyroidism demonstrated poor chelation therapy in these patients. Close monitoring of ferritin level was recommended. Also, the measurement of parathyroid hormone on a regular basis for all thalassemic patients was recommended.

Amir Ali Hamidieh

2009-07-01

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Zinc and Copper Status in Children with Beta-Thalassemia Major  

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Full Text Available Objective:There are some reports in which a condition of zinc deficiency and its associated outcomes with a change in concentration of serum copper among the thalassemic patients has been highlighted. The aim of this prospective study was to determine the serum zinc and copper levels in children with beta-thalassemia major.Methods:In this cross sectional study all children under 12 years affected by beta thalassemia major (40 patients were evaluated for serum zinc and copper levels in Qazvin thalassemia center (Qazvin, Iran in 2007. Serum measurements for zinc and copper were performed by atomic absorption spectrophotometer.Findings:The mean concentrations of serum zinc and copper levels were 67.35?20.38 and 152.42?24.17 ?g/dl respectively. Twenty-six (65% of thalassemic patients had zinc concentration under 70 ?g/dl (hypozincemia. None of the thalassemic children had copper deficiency. No significant correlation between serum zinc level with age, weight, height, body mass index, duration of blood transfusion, desferrioxamine dose and ferritin level was observed in thalassemic patients (P=0.3.Conclusion:This study revealed that hypozincemia is common in thalassemic patients, but in contrast, there is no copper deficiency. Further evaluation in this regard is recommended.

Abolfazl Mahyar

2010-09-01

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Frequency of hepatitis B and hepatitis C in multi - transfused beta thalassemia major patients  

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To determine the frequency of hepatitis B and C virus infection among children with beta thalassemia major registered at Military Hospital Rawalpindi. Children attending Thalassemia Centre Military Hospital Rawalpindi for regular blood transfusion were registered. They belonged to different ethnic groups and came from different parts of the country. Their demographic data was recorded, detailed history taken and physical examination was carried out. Their serum samples were tested for hepatitis B surface antigen and anti HCV antibody assay with third generation commercial ELISA method. During the study; 141 patients of beta thalassemia major were screened. Out of them 50 patients (35.5% ,95% confidence interval 27.8-43.5)w ere found hepatitis C virus antibody positive and 1 patient (0.7 %) hepatitis B surface antigen positive. One patient (0.7%) had both hepatitis B and C virus infection. Mean age of hepatitis C infected patients was 10.4+3.85y ears (range 2-16 years). Mean age of uninfected patients was 6.1 + 3.59 years. (p value 0.000) In addition, the results indicate that higher prevalence of anti-HCV was significantly associated with longer duration of transfusion (p value <0.003). In spite of the fact that screened blood is used for transfusions, still a large number of patients have been found infected with hepatitis C. Therefore more accurate techniques are required for screening of blood to prevent transfusion associated transmission. (author)ted transmission. (author)

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Zinc and Copper Status in Children with Beta-Thalassemia Major  

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Full Text Available Objective: There are some reports in which a condition of zinc deficiency and its associated outcomes with a change in concentration of serum copper among the thalassemic patients has been highlighted. The aim of this prospective study was to determine the serum zinc and copper levels in children with beta-thalassemia major.Methods: In this cross sectional study all children under 12 years affected by beta thalassemia major (40 patients were evaluated for serum zinc and copper levels in Qazvin thalassemia center (Qazvin, Iran in 2007. Serum measurements for zinc and copper were performed by atomic absorption spectrophotometer.Findings: The mean concentrations of serum zinc and copper levels were 67.35±20.38 and 152.42±24.17 ?g/dl respectively. Twenty-six (65% of thalassemic patients had zinc concentration under 70 µg/dl (hypozincemia. None of the thalassemic children had copper deficiency. No significant correlation between serum zinc level with age, weight, height, body mass index, duration of blood transfusion, desferrioxamine dose and ferritin level was observed in thalassemic patients (P=0.3.Conclusion: This study revealed that hypozincemia is common in thalassemic patients, but in contrast, there is no copper deficiency. Further evaluation in this regard is recommended.

Hoshyar Mojabi

2010-09-01

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Hypoparathyroidism and intracerebral calcification in patients with beta-thalassemia major  

International Nuclear Information System (INIS)

Background: Hypoparathyroidism is one of the most important endocrine complications of thalassemia major. This study was conducted to evaluate the prevalence of intracerebral calcifications in patients with thalassemia with and without hypoparathyroidism. Methods: 47 beta-thalassemia patients with hypoparathyroidism underwent a brain CT scan to investigate the presence and extent of intracerebral calcification. 30 age- and sex-matched beta-thalassemic patients with normal parathyroid function who had undergone brain CT for headache, or some other minor neurologic problems were also enrolled in the study serving as controls. The amount of intracerebral calcification, hematologic parameters, and some clinical findings were compared between both groups. Results: Intracerebral calcification was present in 54.2% of beta-thalassemia patients with hypoparathyroidism. The most frequent sites of calcification were basal ganglia, and frontoparietal areas of the brain. Thalami, internal capsule, cerebellum and posterior fossa were other less frequently calcified regions of the brain. In contrast, there was no evidence of intracerebral calcifications in the 30 thalassemic patients with normal parathyroid function. There was not a statistically significant difference between serum ferritin concentrations in thalassemia patient with hypoparathyroidism and those with normal parathyroid function (2781 vs. 2178, P > 0.05). Conclusion: Intracranial calcification is a common finding in nial calcification is a common finding in thalassemia patients with hypoparathyroidism, it can be extensive and involves most regions of the brain.

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Hypoparathyroidism and intracerebral calcification in patients with beta-thalassemia major  

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Background: Hypoparathyroidism is one of the most important endocrine complications of thalassemia major. This study was conducted to evaluate the prevalence of intracerebral calcifications in patients with thalassemia with and without hypoparathyroidism. Methods: 47 beta-thalassemia patients with hypoparathyroidism underwent a brain CT scan to investigate the presence and extent of intracerebral calcification. 30 age- and sex-matched beta-thalassemic patients with normal parathyroid function who had undergone brain CT for headache, or some other minor neurologic problems were also enrolled in the study serving as controls. The amount of intracerebral calcification, hematologic parameters, and some clinical findings were compared between both groups. Results: Intracerebral calcification was present in 54.2% of beta-thalassemia patients with hypoparathyroidism. The most frequent sites of calcification were basal ganglia, and frontoparietal areas of the brain. Thalami, internal capsule, cerebellum and posterior fossa were other less frequently calcified regions of the brain. In contrast, there was no evidence of intracerebral calcifications in the 30 thalassemic patients with normal parathyroid function. There was not a statistically significant difference between serum ferritin concentrations in thalassemia patient with hypoparathyroidism and those with normal parathyroid function (2781 vs. 2178, P > 0.05). Conclusion: Intracranial calcification is a common finding in thalassemia patients with hypoparathyroidism, it can be extensive and involves most regions of the brain.

Karimi, M. [Iran-Shiraz-Namazee Hospital, Namazee Square, Hematology Research Center, Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of)], E-mail: karimim@sums.ac.ir; Rasekhi, A.R. [Iran-Shiraz-Namazee Hospital, Namazee Square, Imaging Research Center, Department of Radiology, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of)], E-mail: rasekhia@sums.ac.ir; Rasekh, M. [Iran-Shiraz-Namazee Hospital, Namazee Square, Department of Endocrinology and Metabolism, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of)], E-mail: Rasekhm@sums.ac.ir; Nabavizadeh, S.A. [Iran-Shiraz-Namazee Hospital, Namazee Square, Imaging Research Center, Department of Radiology, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of)], E-mail: nabavia@gmail.com; Assadsangabi, R. [Iran-Shiraz-Namazee Hospital, Namazee Square, Imaging Research Center, Department of Radiology, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of)], E-mail: assadsangabi@yahoo.com; Amirhakimi, G.H. [Iran-Shiraz-Namazee Hospital, Namazee Square, Department of Endocrinology and Metabolism, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of)], E-mail: amirhakimig@sums.ac.ir

2009-06-15

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Bone metabolism and mineral density in patients with beta-thalassemia major  

International Nuclear Information System (INIS)

To evaluate bone metabolism in patients with beta-thalassemia major and to determine the factors associated with the development of osteoporosis. We studied 25 patients with thalassemia major with a mean age of 18.4 years (rang 5-31), age and gender matched 24 healthy controls who were attending the outpatient physical medicine and rehabilitation clinic of Akdeniz University Hospital between January 2004 and March 2004 in Turkey. Bone mineral density (BMD) of lumbar spine (L-1-L4) and proximal femur were determined using dual x-ray absorptiometry (DXA). Venous blood samples were obtained for determination of blood cell count and markers of bone formation and resorption. The BMD values, both at lumbar and femoral neck levels were significantly lower in patients compared to controls. Serum N-telopeptide level was slightly higher, whereas osteocalcin was slightly lower in patients, however, the values were not statistically significant. Plasma levels of insulin like growth factor-1 (IGF-I) and insulin like growth factor for binding protein-3 (IGFBP-3) were significantly lower in patients. Also, serum levels of estradiol and progesterone in females, luteinizing, hormone and follicle-stimulating hormone in both genders were significantly lower in patients. Serum levels of free testosterone and total testosterone were lower in patients, but not statistically significant. Patients also had significantly higher serum phosphorous levels and lower serum calcitonin levels compars and lower serum calcitonin levels compared to controls. The BMD is decreased in thalassemic patients. Growth retardation, growth hormone/IGF-I/IGFP-3 axis dysfunction, gonadal dysfunction and hypothalomo-pituitary-gonadal axis dysfunction may be responsible for the development of osteoporosis in the patients with beta-thalassemia major. (author)

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Pakistani children's experiences of growing up with beta-thalassemia major.  

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In this study, we explored the lived experiences of children with beta-thalassemia major (?-TM). We considered children as experts on their experiences in contrast to the prevalent approach of asking parents or other adults about children's perspectives. The sample consisted of 12 children aged 8 to12 years. There were two stages to data collection. In Stage 1 we employed two focus group discussions and two role plays and analyzed the data thematically. This directly informed Stage 2, consisting of 12 in-depth interviews subjected to interpretative phenomenological analysis. From our findings we show that living with ?-TM involves a continuous struggle between feelings of being different and strategies to minimize these differences to strive for normalcy. We suggest that understanding the experiences of living with ?-TM from children's perspectives can provide unique insights into their experiences, which can fill the gap in the existing, predominantly adult-oriented research on chronic illness. PMID:25249550

Mufti, Gul-E-Rana; Towell, Tony; Cartwright, Tina

2015-03-01

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The effect of repeated transfusions on immunoglobulins levels and complement components in Beta Thalassemia major patients  

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Full Text Available Beta Thalassemia major patients receive repeated transfusions in order to compensate anemia and use desferal to remove iron overload. Comparing immunoglobulins and complement components in the serum of these patients with normal range shows a significant increase (P<0.001 in IgG, IgA and IgM and a decrease in C3c and C4. The regression analysis confirms a relation between the numbers of transfusions and the mentioned immunological factors that means increasing the number of transfusions, increases immunoglobulins and decreases the complement. Also, this evaluation shows that use of washed RBC and regular in take of desferal will prevent excessive increase of immunoglobulins or decrease of complement

Mehrabani K

1996-06-01

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Prospects and future of conservative management of beta thalassemia major in a developing country  

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Objective: To assess the efficacy, prospects and future of conservative management of beta thalassemia major patients in a developing country. Design: Patients registered at IHBTS were studied over a period of three years. They consented to being managed on moderate transfusion regimen, aiming to maintain a pre-transfusion haemoglobin(Hgb) level of 9.0 plus minus 1.0 g per dL. We studied their transfusion requirements, status for transfusion transmitted infections (TTls), serum ferritin levels and complications developing as a result of iron overload. Subjects: Initially all registered patients were included in this study. Sporadic patients as well as dropouts occurring due to any reason, (patients concurrently seeking treatment at other centres as well, or complying poorly to advised chelation therapy) were excluded from the study. The data presented here conforms to a cohort of 60 regular patients who adhered best to our selection criteria. Main Outcome Measures: 1) The study highlights the deficiencies and problems of conservative management for beta thalassemia major. 2) The major impact of our study is the message that conservative management in a poor country, like ours, is a no-win situation. 3) There is an urgent need to immediately start a prevention programme. Results: In the younger patients, blood consumption even on the moderate transfusion regimen is 120ml/kg/year, however with ascending age the consumption increases to 240ml/kg/year. A substantive numbeases to 240ml/kg/year. A substantive number of the patients are either Hep C (35%) or Hep B (1.7%) positive. There are no HIV positive patients. Serum ferritin levels vary widely and could not be controlled due to poor compliance to chelation. 50% of the patients developed one or other complications of iron overload. The cost of treatment depending on the quality of care, is tremendous and beyond the reach of the common man. Conclusions: Conservative management may be the best alternative and at times the only hope for patients in our country. However, in order to decrease the disease load, steps need to be taken to introduce preventive measures. (author)

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Frequency of Sensory Neural Hearing Loss in Major Beta-Thalassemias in Southern Iran  

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Full Text Available Objective:The thalassemias are among the most common genetic disorders worldwide, occurring more frequently in the Mediterranean region. The aim of this study was to determined frequency of sensory-neural hearing loss in major ß- thalassemias transfusion dependent patients in south of Iran.Methods:A cross sectional study on 308 cases of major beta-thalassemia patients referring to Thalassemia Center of Shiraz University of Medical Sciences between 2006-2007 years. The diagnosis of ß- thalassemia major was based on clinical history, complete blood count and hemoglobine electrophoresis. Clinical data such as serum ferritin level, deferoxamine (DFO dose, mean daily doses of DFO (mg/kg and audiometric variables was recorded.Findings:Out of 308 cases, 283 (96.5% had normal hearing and 10 (3.5% sensorineural hearing loss. There was no statically significant difference between two groups regarding mean age, weight, age at the first blood transfusion, age at the first DFO infusion.Conclusion:We found the lowest incidence of sensorineural hearing loss in a large population of patients suffered from major thalassemia who received DFO. We show that DFO is not ototoxic at a low dose. When considering all related literature, as a whole there has been much critical misrepresentation about DFO ototoxicity.

Abolhassan Faramarzi

2010-09-01

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Frequency of Sensory Neural Hearing Loss in Major Beta-Thalassemias in Southern Iran  

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Full Text Available Objective:The thalassemias are among the most common genetic disorders worldwide, occurring more frequently in the Mediterranean region. The aim of this study was to determined frequency of sensory-neural hearing loss in major ß- thalassemias transfusion dependent patients in south of Iran. Methods:A cross sectional study on 308 cases of major beta-thalassemia patients referring to Thalassemia Center of Shiraz University of Medical Sciences between 2006-2007 years. The diagnosis of ß- thalassemia major was based on clinical history, complete blood count and hemoglobine electrophoresis. Clinical data such as serum ferritin level, deferoxamine (DFO dose, mean daily doses of DFO (mg/kg and audiometric variables was recorded. Findings:Out of 308 cases, 283 (96.5% had normal hearing and 10 (3.5% sensorineural hearing loss. There was no statically significant difference between two groups regarding mean age, weight, age at the first blood transfusion, age at the first DFO infusion. Conclusion:We found the lowest incidence of sensorineural hearing loss in a large population of patients suffered from major thalassemia who received DFO. We show that DFO is not ototoxic at a low dose. When considering all related literature, as a whole there has been much critical misrepresentation about DFO ototoxicity.

Seyed-Taghi Heydari

2010-09-01

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Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis  

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Most deaths in beta-thalassemia major result from cardiac complications due to iron overload. Differential effects on myocardial siderosis may exist between different chelators. A randomized controlled trial was performed in 61 patients previously maintained on subcutaneous deferoxamine. The primary end point was the change in myocardial siderosis (myocardial T2(*)) over 1 year in patients maintained on subcutaneous deferoxamine or those switched to oral deferiprone monotherapy. The dose of d...

Piga, Antonio Giulio

2006-01-01

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Correlation of oxidative stress with serum trace element levels and antioxidant enzyme status in Beta thalassemia major patients: a review of the literature.  

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Beta thalassemia major is an inherited disease resulting from reduction or total lack of beta globin chains. Patients with this disease need repeated blood transfusion for survival. This may cause oxidative stress and tissue injury due to iron overload, altered antioxidant enzymes, and other essential trace element levels. The aim of this review is to scrutinize the relationship between oxidative stress and serum trace elements, degree of damage caused by oxidative stress, and the role of antioxidant enzymes in beta thalassemia major patients. The findings indicate that oxidative stress in patients with beta thalassemia major is mainly caused by tissue injury due to over production of free radical by secondary iron overload, alteration in serum trace elements and antioxidant enzymes level. The role of trace elements like selenium, copper, iron, and zinc in beta thalassemia major patients reveals a significant change of these trace elements. Studies published on the status of antioxidant enzymes like catalase, superoxide dismutase, glutathione, and glutathione S-transferase in beta thalassemia patients also showed variable results. The administration of selective antioxidants along with essential trace elements and minerals to reduce the extent of oxidative damage and related complications in beta thalassemia major still need further evaluation. PMID:22645668

Shazia, Q; Mohammad, Z H; Rahman, Taibur; Shekhar, Hossain Uddin

2012-01-01

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Prevalence of Heart Failure in the Cases of Beta-thalassemia Major; Two Years Follow-Up  

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Full Text Available Inroduction: Heart failure (HF is an important cause of morbidity and mortality in the cases of Beta-thalassemia major. The purpose of this study was to estimate HF prevalence in these patients and to assess the survivability of those who were treated with intensive chelating therapy. Design and methods: This cross sectional study included 72 beta-thalassemia major cases, the mean age at the time of referral was 15.7±6.2 years (range 6-35 years and were followed in a prospective 2 year study. A self-reporting symptom questionnaire was administered, a 12-lead ECG was taken and an echocardiography was obtained from all participants. Echocardiography was performed at 6 month intervals or when a new symptom developed. Results: Risk factors (except for iron overload in the study population were hypothyroidism and diabetes mellitus. The male to female ratio was0.75.Twelve patients had left ventricular (LV systolic dysfunction and 57,79% had LV diastolic dysfunction whereas 11,15% had RV failure. Fifty-nine (81% patients had cardiac disease of which diastolic dysfunction was the most common manifestation .Those with systolic dysfunction were older at presentation (22 ± 6 years versus 31 ± 4 years; P <0.001, and had the highest mean serum ferritin level (3,355 ± 1241 ng/mL versus 6,397 ± 1,613 ng/mL; P <0.001. The 2 year survival rate in patients with beta thalassemia in this study was 98%. Conclusions: Diastolic dysfunction is highly prevalent in even asymptomatic beta-thalassemia major patients. The high prevalence of diastolic dysfunction is indicative of a significant amount of the population who are at a high risk for HF

Atooshe Rohani

2013-05-01

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The pancreas in {beta}-thalassemia major: MR imaging features and correlation with iron stores and glucose disturbunces  

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The study aims at describing the MR features of pancreas in beta-thalassemia major, investigating the relations between MR findings and glucose disturbances and between hepatic and pancreatic siderosis. Signal intensity ratios of the pancreas and liver to right paraspinous muscle (P/M, L/M) were retrospectively assessed on abdominal MR imaging studies of 31 transfusion-dependent patients with beta-thalassemia major undergoing quantification of hepatic siderosis and 10 healthy controls, using T1- (120/4/90), intermediate in and out of phase - (120/2.7, 4/20), and T2*-(120/15/20) weighted GRE sequences. Using the signal drop of the liver and pancreas on opposed phase images, we recorded serum ferritin and results of oral glucose tolerance test (OGTT). Decreased L/M and P/M on at least the T2* sequence were noticed in 31/31 and 30/31 patients, respectively, but no correlation between P/M and L/M was found. Patients with pathologic OGTT displayed a higher degree of hepatic siderosis (p < 0.04) and signal drop of pancreas on opposed phase imaging (p < 0.025), implying fatty replacement of pancreas. P/M was neither correlated with glucose disturbances nor serum ferritin. Iron deposition in the pancreas cannot be predicted by the degree of hepatic siderosis in beta-thalassemia major. Fatty replacement of the pancreas is common and may be associated with glucose disturbances. (orig.)

Papakonstantinou, Olympia [University Hospital of Heraklion, Medical School of Crete, Department of Radiology, Heraklion, Crete (Greece); Attikon Hospital, 2nd Department of Radiology, Athens (Greece); Ladis, Vasilios; Kostaridou, Stavroula; Berdousi, Helen; Kattamis, Christos [Thalassemia Unit, University of Athens, ' ' Aghia Sophia' ' Children' s Hospital, Athens (Greece); Maris, Thomas; Gourtsoyiannis, Nicholas [University Hospital of Heraklion, Medical School of Crete, Department of Radiology, Heraklion, Crete (Greece)

2007-06-15

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Life Satisfaction in children and adolescents with beta thalassemia major in southwest Iran  

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Background: Beta thalassemia major has a considerable impact on quality of life. The purpose of this study was to determine the life satisfaction of beta-thalassemic children and adolescents compared to healthy controls. Methods: This research, conducted in 2009, was a controlled, cross-sectional study in which beta-thalassemic patients, who were being followed-up by the Thalassemic Center in Bushehr, a city in southern Iran, were compared with a healthy control group. The Multi-dimensional Student Life Satisfaction Scale (MSLSS) was used to measure the participants’ quality of life in five domains. The chi-squared test, t-test, Pearson’s Product Moment Correlation, and multiple regression analysis were used for the statistical analyses. Results: The unadjusted mean scores of three of the domains, i.e., school, friends, and living environment, and the total score of five domains, i.e., school, friends, living environment, family, and self, were significantly higher in thalassemic patients than in the control group (P parents and society concerning assigning responsibility to patients should be assessed. The assessment should include comparing the satisfaction with life of thalassemic patients with that of their healthy siblings and conducting national studies on the quality of life of thalassemic patients and their satisfaction with life.

Hatami, Gissou; Motamed, Niloofar

2014-01-01

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The survival analysis of beta thalassemia major patients in South East of Iran  

International Nuclear Information System (INIS)

The objective was to determine the survival of beta-thalassemia major patients with transfusion, and its related factors in Southeast of Iran. This cross-sectional study was performed in Zahedan, Iran in 2007. The sample included patients who were referred from all over the Zahedan Thalassemia Center from 1998 to 2006. The data were collected using the patient's records, which were recorded by the staff during transfusion. The data included demographic and medical information blood group, blood RH, the kind of transfused blood [KTB], annual number of transfusions [ANOT], accompanied disease [AD], Hemoglobin [Hb] and ferritin level. For data analysis, the Kaplan-Meyer method, and Long Rank test together with Cox Regression were used. Forty-six of 578 patients died and 99% survived for the first year. The ages survival proportions were 5 (97.9%), 10 (97%), 15 (92.1%), and 20 (81.2%) years. The survival time showed significant relationships with the ANOT p=0.0053, KTB p=0.003, Hb=0.002 and ferritin level p=0.0087, and AD p=0.00. Using regular transfusion, paying attention to screening of transfused blood, increasing the families knowledge on the disease to prevent the bearing of thalassemia fetus, are recommended; finally, the detection and treating of the AD, are of great importance to extend the lifetime of the patients. (author)

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Resolution of alloimmunization and refractory autoimmune hemolytic anemia in a multi-transfused beta-thalassemia major patient.  

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Beta-thalassemia is one of the most prevalent autosomal disorders, which affect more than 400,000 newborn per year worldwide. In India, the carrier rate of beta-thalassemia varies from 3-17%. The overall rate of alloimmunization in thalassemia patients has been reported to be 5-30% in the world, which is mostly contributed by the alloimmunization to minor blood group antigen. Among Asians, the incidence of red cell alloimmunization is 22%. The recommended treatment for beta-thalassemia major is regular blood transfusion every 3 to 4 weeks. The development of anti-red cell antibodies (alloantibodies and/or autoantibodies) can significantly complicate transfusion therapy. Alloantibodies are commonly associated with red cell hemolysis. Red cell autoantibodies appear less frequently, but they can result in clinical hemolysis called autoimmune hemolytic anemia (AIHA), and in difficulty in cross-matching blood. Patients with autoantibodies may have a higher transfusion rate and often require immunosuppressive drugs or alternative treatments including intravenous immunoglobulin (IVIg) and rituximab (anti-CD20 monoclonal antibody). PMID:25161355

Philip, Joseph; Jain, Neelesh

2014-07-01

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Assessment of diastolic function in children and adolescents with beta-thalassemia major by tissue Doppler imaging - Original Article  

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Full Text Available Aim: The purpose of this study was to analyze myocardial diastolic function in patients with beta-thalassemia major before development of overt cardiomyopathy using pulsed wave tissue Doppler imaging, and compare data with conventional Doppler echocardiography.Material and Method: The study included 61 beta-thalassemia major subjects (age 4 to 20 years; mean age, 10.7±4.1 years; 32 females and 29 males with normal left ventricular function and 52 healthy control subjects, matched for age and sex. All participants underwent M-mode echocardiography and left ventricular systolic function was measured; diastolic functions of the right and left ventricul were analysed using tissue Doppler imaging and conventional Doppler echocardiography. SPSS for Windows 13.0 software programme was used for statistical analysis, and the student’s t-test was used to compare data. This study was approved by the ethics committee of the ?stanbul Medical Faculty.Results: Body surface area was significantly smaller in the patients than in the controls (1.0±0.2 vs. 1.2±0.3 m2, p0.05. In patients with beta-thalassemia major, the early diastolic velocities of the myocardium at the base of the left and right ventricle, at the middle segment of the left and right ventricle, and the interventricular septum were found to be higher than controls (p<0.05. We also found significantly higher late diastolic velocities at the base and middle segments of right ventricle, early and late diastolic velocities ratio at the base of the right ventricle and at the middle segment of the left and right ventricle, and the interventricular septum compared with controls (p<0.05.Conclusions: These findings acquired from the young aged beta-thalassemia major patients with normal ventricular systolic function were believed to be results from high preload and hyperdynamic response to chronic anemia rather than true ventricular restriction. We suggest that long-term follow-up studies should be carried out in patients with beta-thalassemia major using tissue Doppler in order to evaluate the diagnostic accuracy of this imaging technique in diagnosis of early stages of cardiac involvement. (Turk Arch Ped 2011; 46: 26-32

Taner Yavuz

2011-03-01

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Beta-thalassemia  

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Abstract Beta-thalassemias are a group of hereditary blood disorders characterized by anomalies in the synthesis of the beta chains of hemoglobin resulting in variable phenotypes ranging from severe anemia to clinically asymptomatic individuals. The total annual incidence of symptomatic individuals is estimated at 1 in 100,000 throughout the world and 1 in 10,000 people in the European Union. Three main forms have been described: thalassemia major, thalassemia intermedia and thalassemia minor...

Origa Raffaella; Galanello Renzo

2010-01-01

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Endocrine dysfunction and growth retardation assessment in children with beta -thalassemia major  

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Children suffering from beta-thalassemia major are reported to have endocrine abnormalities and growth retardation. This study was carried out to study the cause of their growth retardation and determine the extent and rate of endocrine complications. Twenty beta-thalassemic major pubertal children, with mean baemoglobin and ferritin concentration of 8.8±0.6 and 3.597± 1.931, respectively, and twenty pubertal control children were used in the study. The anthropometric measurements that carried out revealed significant low growth rate in patient groups in comparison with control. Patients divided into two groups; I) D-thal with delayed growth and II) S-thal with stunted growth. Basal serum thyoid hormones (T3 and T4) and thyroid stimulating hormone (TSH) were measured in patient groups and control group. T3 showed highly significant decrease (P4 showed non-significant change and TSH showed highly significant increase (P<0.001). Serum growth hormone showed significant lower concentrations in patient groups with values of 2.163±0.9 ng/ml, (P<0.01) and 1.832±1.9ng/ml, (P<0.01) for delayed growth thalassemic group (D-thal) and stunted growth thalassemic group (S-thal), respectively. Serum concentration of insulin growth factor-1 (IGF-1) hormone was studied. D-thal and S-thal had significant lower basal IGF-1 concentrationsof-58.44% (P<0.001) for D-thal and -64.37%, (P<0.001) for for D-thal and -64.37%, (P<0.001) for S-thal

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Side effects of Deferasirox Iron Chelation in Patients with Beta Thalassemia Major or Intermedia  

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Full Text Available Objectives: Chelating agents remain the mainstay in reducing the iron burden and extending patient survival in homozygous beta-thalassemia but adverse and toxic effects may increase with the institution and long term use of this essential therapy. This study aimed to estimate the incidence of deferasirox (DFX side effects in patients with thalassemia major or intermedia.Methods: A retrospective study of 72 patients (mean age: 20.3±0.9 yrs; 36 male, 36 female with thalassemia major or intermedia treated at Sultan Qaboos University Hospital, Oman, was performed to assess the incidence of side effects related to deferasirox over a mean of 16.7 month follow-up period.Results: Six patients experienced rashes and 6 had gastro-intestinal upset. DFX was discontinued in 18 patients for the following reasons: persistent progressive rise(s in serum creatinine (7 patients; 40% mean serum creatinine rise from baseline, feeling unwell (2, severe diarrhea (1, pregnancy (1, death unrelated to chelator (2 and rise in serum transaminases (2. Three patients were reverted to desferoxamine and deferiprone combination therapy as DFX was no longer biochemically effective after 18 months of therapy. There was no correlation between baseline serum ferritin and serum creatinine or a rise in serum creatinine. Cardiac MRI T2* did not change with DFX therapy. However, there was an improvement in liver MRI T2* (p=0.013.Conclusion: Renal side effects related to deferasirox appear to be higher than those reported in published clinical trials. Further larger studies are required to confirm these findings.

Murtadha Al-Khabori

2013-03-01

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Quality of life among Iranian patients with beta-thalassemia major using the SF-36 questionnaire.  

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CONTEXT AND OBJECTIVE Patients with beta-thalassemia major (?-TM) experience physical, psychological and social problems that lead to decreased quality of life (QoL). The aim here was to measure health-related QoL and its determinants among patients with ?-TM, using the Short Form-36 (SF-36) questionnaire. DESIGN AND SETTING Cross-sectional study at the Hematology Research Center of Shiraz University of Medical Sciences, in southern Iran. METHODS One hundred and one patients with ?-TM were randomly selected. After the participants' demographics and disease characteristics had been recorded, they were asked to fill out the SF-36 questionnaire. The correlations of clinical and demographic factors with the QoL score were evaluated. RESULTS There were 44 men and 57 women of mean age 19.52 ± 4.3 years (range 12-38). On two scales, pain (P = 0.041) and emotional role (P = 0.009), the women showed significantly lower scores than the men. Lower income, poor compliance with iron-chelating therapy and presence of comorbidities were significantly correlated with lower SF-36 scores. These factors were also found to be determinants of worse SF-36 scores in multivariate analysis. CONCLUSIONS We showed that the presence of disease complications, poor compliance with iron-chelating therapy and poor economic status were predictors of worse QoL among patients with ?-TM. Prevention and proper management of disease-related complications, increased knowledge among patients regarding the importance of managing comorbidities and greater compliance with iron-chelating therapy, along with psychosocial and financial support, could help these patients to cope better with this chronic disease state. PMID:23903265

Haghpanah, Sezaneh; Nasirabadi, Shiva; Ghaffarpasand, Fariborz; Karami, Rahmatollah; Mahmoodi, Mojtaba; Parand, Shirin; Karimi, Mehran

2013-01-01

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Beta Thalassemia (For Parents)  

Science.gov (United States)

Thalassemias Thalassemias are a group of blood disorders that affect the way the body makes hemoglobin, a ... results in that type of thalassemia. About Beta Thalassemia Beta thalassemia occurs when the gene that controls ...

44

The Prevalence of Adrenal, Parathyroid and Cardiac Dysfunction in Patients with Beta Thalassemia Major  

OpenAIRE

Background: Adrenal and parathyroid insufficiency are uncommon in patients with transfusion dependent Beta Thalassemia (?-TM). Further, myocardial echocardiographic abnormalities are recognized but with a variable outcomes Aim: The aim is to determine the prevalence of adrenal and parathyroid insufficiency in patient with transfusion dependent ?-TM. And to assess left ventricle systolic and diastolic function using Pulsed Doppler (PD) and Tissue Doppler (DT) echocardiogram. Methods:The stud...

Mahdi, Najat E.; Garadah, Taysir S.; Zuheir Hassan; Jaradat, Ahmed A.; Nagalla, Das S.

2013-01-01

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MR imaging technique for the diagnosis of pituitary iron overload in patients with transfusion-dependent beta-thalassemia major.  

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To identify the optimal MR imaging technique for diagnosing pituitary iron overload, we compared spin-echo and gradient-echo MR imaging with measurements of pituitary T2 relaxation times in 30 patients with secondary hemochromatosis due to transfusion-dependent beta-thalassemia major and in 10 healthy volunteers. We found that the optimal MR imaging technique to evaluate pituitary iron overload is the gradient-echo T2*-weighted technique, as it best demonstrated signal reduction in the anterior lobe of the pituitary gland. PMID:9874546

Sparacia, G; Banco, A; Midiri, M; Iaia, A

1998-01-01

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Glycoprotein B genotyping of human cytomegalovirus strains isolated from brazilian patients with sickle cell disease and Beta-thalassemia major.  

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The role of the human cytomegalovirus (HCMV) infection in individuals with hemoglobinopathies is unclear. Our objective was to examine the molecular and genotypic characteristics of HCMV in patients with sickle cell disease, beta-thalassemia major, and volunteer blood donors by viral load quantitation, glycoprotein B (gB) genotyping, and phylogenetic analysis. The patients with sickle cell disease demonstrated the highest HCMV DNA prevalence (13.8%), followed by the patients with beta-thalassemia major (7.6%), and the blood donors (3%). The infection was characterized by a low mean viral load (3.8×10(3) copies/mL), but infections with higher copy numbers were also observed. Genotype gB2 was detected in the majority of cases (90.9%), followed by genotype gB1 (9.1%). No gB3/gB4 genotype was detected. No statistical significance was observed between HCMV DNAemia/gB genotype and hematological alterations or severity of the disease. The high number of sickle cell disease patients with HCMV DNAemia could be due to their partial immune dysfunction (multiple transfusions, spleen dysfunction, hydroxyurea treatment). The extensive HCMV gB2 prevalence in patients with hemoglobinopathies is probably due to HCMV epidemiologic characteristics in the examined region, and can be important during the clinical management of these patients. PMID:25420197

Slavov, Svetoslav N; Kashima, Simone; Wagatsuma, Virginia M D; Silva-Pinto, Ana Cristina; Martinez, Edson Z; Favarin, Maria do Carmo; Covas, Dimas T

2015-03-01

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Pituitary gland height evaluated by MR in patients with {beta}-thalassemia major: a marker of pituitary gland function  

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In transfusion-dependent {beta}-thalassemia major, increased iron deposition in the pituitary gland has a cytotoxic effect leading mainly to hypogonadotropic hypogonadism. Our purpose was to assess in these patients the height of the pituitary gland and to evaluate whether it represents a marker of pituitary gland function. In 29 patients with {beta}-thalassemia major and 35 age- and gender-matched controls the pituitary gland height was evaluated in a midline sagittal scan using a spin echo T1-weighted (500/20 TR/TE) sequence. In all patients, an extensive endocrine evaluation was performed, including measurements of spontaneous and stimulated levels of gonadotropins, thyroid hormones, growth hormone, insulin-like growth factor, and adrenal hormones. The pituitary gland height was lower in thalassemic patients with hypogonadotropic hypogonadism (n=15) (mean 3.48; SD 0.46) than in the age- and gender-matched controls (mean 6.29; SD 0.77), (P<0.001). No statistically significant difference was found between thalassemic patients without hormone dysfunction (n=14) (mean 5.34; SD 1.52) and age- and gender-matched controls (mean 5.91; SD 1.06). We conclude that in thalassemic patients the pituitary gland height is an additional marker of pituitary gland function and might be useful in clinical management. (orig.)

Argyropoulou, M.I.; Metafratzi, Z.; Efremidis, S.C. [Dept. of Radiology, Univ. of Ioannina (Greece); Kiortsis, D.N. [Dept. of Physiology, Univ. of Ioannina (Greece); Bitsis, S.; Tsatoulis, A. [Dept. of Internal Medicine, Univ. of Ioannina (Greece)

2001-12-01

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Genetics Home Reference: Beta thalassemia  

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... Recent literature OMIM Genetic disorder catalog Conditions > Beta thalassemia On this page: Description Genetic changes Inheritance Diagnosis ... Glossary definitions Reviewed July 2009 What is beta thalassemia? Beta thalassemia is a blood disorder that reduces ...

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The Prevalence of Adrenal, Parathyroid and Cardiac Dysfunction in Patients with Beta Thalassemia Major  

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Full Text Available Background: Adrenal and parathyroid insufficiency are uncommon in patients with transfusion dependent Beta Thalassemia (?-TM. Further, myocardial echocardiographic abnormalities are recognized but with a variable outcomes Aim: The aim is to determine the prevalence of adrenal and parathyroid insufficiency in patient with transfusion dependent ?-TM. And to assess left ventricle systolic and diastolic function using Pulsed Doppler (PD and Tissue Doppler (DT echocardiogram. Methods:The study was conducted on patients with ?-TM (n = 99, age 15.92 ± 8.92 years and compared with an age-matched controls (n = 98 age 15.79 ± 8.94 years. In all participants echocardiographic indices of M mode and PD and TD were performed. Blood samples were withdrawn for measuring the serum cortisol, parathyroid and Ferritin. Correlation between the level of cortisol and ferritin level was evaluated. Results: Patients with ?-TM compared with controls, had significantly thicker LV septal wall index of 0.65 ± 0.26 vs 0.44 ± 0.2190, p 0.001 and LV posterior wall of 0.65 ± 0.235 vs 0.43 ± 0.214, p   ± 5.5 vs 5.0  ± 5.6, p = 0.23. Furthermore patients with ?-TM had higher E/A ratio (1.54 ± 0.18 vs 1.23 ± 0.17, p 0.01 and shorter deceleration time (DT (170.53 ± 13.3 vs 210.50 ± 19.20 m sec, p 0.01. The ratio of transmitral E wave velocity to the tissue Doppler E wave at the basal septal mitral annulus (E/Em was significantly higher in ?-TM group (19.68 ± 2.81 vs 13.86 ± 1.41, p 0.05. The tissue Doppler systolic wave (Sm velocity and the early diastolic wave (Em were significantly lower in ?-TM group compared with controls with Sm, of 4.82 ± 1.2 vs 6.22 ± 2.1 cm/sec, p 0.05 and (Em of 3.51 ± 2.7 vs 4.12 ± 2.5 cm/sec p 0.05, respectively. The tricuspid valve velocity was significantly higher in ?-TM patients compared with controls (2.85 ± 0.56 vs 1.743 ± 0.47 m/sec, respectively, p 0.01. The prevalence of adrenal insufficiency in patients with ?-TM was 16%, hypoparathyroidism of 4.5% weak negative correlation between serum level of cortisol and the serum Ferritin. Conclusion: Patients with ?-thalassemia major had a high prevalence of subclinical adrenal insufficiency of 16%, hypoparathyroidism of 4.5% with weak negative correlation between the low level of cortisol ?160 nmol/L and high serum ferritin. Echocardiographic Pulsed Doppler showed a restrictive LV diastolic pattern suggestive of advanced diastolic dysfunction but preserved left ventricle systolic function.

Najat E. Mahdi

2013-07-01

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Prevalence and Intensity of Depression in Mothers of Children with Beta-Thalassemia Major In Talghani Hospital of Gorgan, Iran  

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Full Text Available Background: Thalassemia is a chronic disease that it leads to psychological and social problems for parents. Mothers are at markedly increased risk of suffering from psychological distress and depression because they usually take on a considerable part of extra care that their children need.This study was designed to determine prevalence and intensity of depression in mothers with a thalassemic child. Material and Methods: In this cross – sectional study, 65 mothers of children with thalassemia major (case group and 65 mothers of children without thalassemia major (control group were assessed using the Beck Depression Inventory (BDI. Data were analyzed by using SPSS (v 16.0 for windows. Results: Prevalence of depression was significantly higher in case group than that in control group (84.6%vs. 56.9%, p <0.05. Moderate depression had a highest prevalence in the both groups (33.4% in case group and 30.8% in control group. Prevalence of severe depression in case group was markedly higher than that in control group (29.2% vs. 3.1% p<0.05. There was a significant difference between intensity of depression in mothers of case group that had another child with beta-thalassemia major (p<0.05. Conclusion: Mothers of children with thalassemia major are vulnerable to depression. They need psychosocial support to promote their health.

Nargesbeygom Mirbehbahani

2014-01-01

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Correlation of Oxidative Stress with Serum Trace Element Levels and Antioxidant Enzyme Status in Beta Thalassemia Major Patients: A Review of the Literature  

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Beta thalassemia major is an inherited disease resulting from reduction or total lack of beta globin chains. Patients with this disease need repeated blood transfusion for survival. This may cause oxidative stress and tissue injury due to iron overload, altered antioxidant enzymes, and other essential trace element levels. The aim of this review is to scrutinize the relationship between oxidative stress and serum trace elements, degree of damage caused by oxidative stress, and the role of ant...

Shazia, Q.; Mohammad, Z. H.; Taibur Rahman; Hossain Uddin Shekhar

2012-01-01

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Adrenal glands in beta-thalassemia major: magnetic resonance (MR) imaging features and correlation with iron stores  

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This study aimed at describing the magnetic resonance (MR) imaging features of the adrenal glands in beta-thalassemic patients and at investigating the relation between adrenal and hepatic siderosis. Adrenal signal intensity (SI) was retrospectively assessed on abdominal MR studies of 35 patients with beta-thalassemia major undergoing quantification of hepatic siderosis and 12 healthy controls, using T1- (120/4/90), intermediate - (120/4/20), and T2*- (120/15/20) weighted GRE sequences. Adrenal SI was graded as grade 0 (normal SI on all sequences), grade 1 (hypointensity on T2* alone), or grade 2 (hypointensity on at least T2*). Adrenal size was measured in the thalassemic patients and compared with normative data. Liver-to-muscle (L/M) SI ratios, expressing hepatic siderosis, were estimated on each sequence. Serum ferritin levels were recorded. Adrenal hypointensity (grades 1 and 2) was noted in 24/35 (68.6%) patients. L/M ratios correlated significantly with adrenal SI in all sequences. Patients with grade 1 and grade 2 adrenal SI had significantly decreased L/M ratios compared with grade 0. Serum ferritin correlated significantly with L/M values but not with adrenal SI. Adrenal size was within normal limits. Diffuse hypointensity in normal-sized adrenals is a common MR finding in beta-thalassemic patients and correlates with the degree of hepatic siderosis. (orig.)

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Adrenal glands in beta-thalassemia major: magnetic resonance (MR) imaging features and correlation with iron stores  

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This study aimed at describing the magnetic resonance (MR) imaging features of the adrenal glands in beta-thalassemic patients and at investigating the relation between adrenal and hepatic siderosis. Adrenal signal intensity (SI) was retrospectively assessed on abdominal MR studies of 35 patients with beta-thalassemia major undergoing quantification of hepatic siderosis and 12 healthy controls, using T1- (120/4/90), intermediate - (120/4/20), and T2*- (120/15/20) weighted GRE sequences. Adrenal SI was graded as grade 0 (normal SI on all sequences), grade 1 (hypointensity on T2* alone), or grade 2 (hypointensity on at least T2*). Adrenal size was measured in the thalassemic patients and compared with normative data. Liver-to-muscle (L/M) SI ratios, expressing hepatic siderosis, were estimated on each sequence. Serum ferritin levels were recorded. Adrenal hypointensity (grades 1 and 2) was noted in 24/35 (68.6%) patients. L/M ratios correlated significantly with adrenal SI in all sequences. Patients with grade 1 and grade 2 adrenal SI had significantly decreased L/M ratios compared with grade 0. Serum ferritin correlated significantly with L/M values but not with adrenal SI. Adrenal size was within normal limits. Diffuse hypointensity in normal-sized adrenals is a common MR finding in beta-thalassemic patients and correlates with the degree of hepatic siderosis. (orig.)

Drakonaki, Eleni; Papakonstantinou, Olympia; Maris, Thomas; Gourtsoyiannis, Nicholas [University Hospital of Heraklion, Department of Radiology, Heraklion (Greece); Vasiliadou, Artemis [Aghios Georgios Hospital of Chania, Thalassemia Unit, Chania (Greece); Papadakis, Alex [Venizelion Hospital of Heraklion, Thalassemia Unit, Heraklion (Greece)

2005-12-01

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Beta-thalassemia.  

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Beta-thalassemias are a group of hereditary blood disorders characterized by anomalies in the synthesis of the beta chains of hemoglobin resulting in variable phenotypes ranging from severe anemia to clinically asymptomatic individuals. The total annual incidence of symptomatic individuals is estimated at 1 in 100,000 throughout the world and 1 in 10,000 people in the European Union. Three main forms have been described: thalassemia major, thalassemia intermedia and thalassemia minor. Individuals with thalassemia major usually present within the first two years of life with severe anemia, requiring regular red blood cell (RBC) transfusions. Findings in untreated or poorly transfused individuals with thalassemia major, as seen in some developing countries, are growth retardation, pallor, jaundice, poor musculature, hepatosplenomegaly, leg ulcers, development of masses from extramedullary hematopoiesis, and skeletal changes that result from expansion of the bone marrow. Regular transfusion therapy leads to iron overload-related complications including endocrine complication (growth retardation, failure of sexual maturation, diabetes mellitus, and insufficiency of the parathyroid, thyroid, pituitary, and less commonly, adrenal glands), dilated myocardiopathy, liver fibrosis and cirrhosis). Patients with thalassemia intermedia present later in life with moderate anemia and do not require regular transfusions. Main clinical features in these patients are hypertrophy of erythroid marrow with medullary and extramedullary hematopoiesis and its complications (osteoporosis, masses of erythropoietic tissue that primarily affect the spleen, liver, lymph nodes, chest and spine, and bone deformities and typical facial changes), gallstones, painful leg ulcers and increased predisposition to thrombosis. Thalassemia minor is clinically asymptomatic but some subjects may have moderate anemia. Beta-thalassemias are caused by point mutations or, more rarely, deletions in the beta globin gene on chromosome 11, leading to reduced (beta+) or absent (beta0) synthesis of the beta chains of hemoglobin (Hb). Transmission is autosomal recessive; however, dominant mutations have also been reported. Diagnosis of thalassemia is based on hematologic and molecular genetic testing. Differential diagnosis is usually straightforward but may include genetic sideroblastic anemias, congenital dyserythropoietic anemias, and other conditions with high levels of HbF (such as juvenile myelomonocytic leukemia and aplastic anemia). Genetic counseling is recommended and prenatal diagnosis may be offered. Treatment of thalassemia major includes regular RBC transfusions, iron chelation and management of secondary complications of iron overload. In some circumstances, spleen removal may be required. Bone marrow transplantation remains the only definitive cure currently available. Individuals with thalassemia intermedia may require splenectomy, folic acid supplementation, treatment of extramedullary erythropoietic masses and leg ulcers, prevention and therapy of thromboembolic events. Prognosis for individuals with beta-thalassemia has improved substantially in the last 20 years following recent medical advances in transfusion, iron chelation and bone marrow transplantation therapy. However, cardiac disease remains the main cause of death in patients with iron overload. PMID:20492708

Galanello, Renzo; Origa, Raffaella

2010-01-01

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Bone marrow changes in beta-thalassemia major: quantitative MR imaging findings and correlation with iron stores  

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The purpose of this study is to describe the MR imaging features of bone marrow in beta-thalassemia major and investigate their relation to ferritin, liver and spleen siderosis. Spinal bone marrow was prospectively assessed on abdominal MR studies of 40 transfused beta-thalassemic patients and 15 controls using T1-w, Pd, T2*-w Gradient Echo (GRE) and T1-w turbo Spin Echo (TSE) sequences. Signal intensity (SI) ratios of liver, spleen and bone marrow to paraspinous muscles (L/M, S/M, B/M respectively) and the respective T2 relaxation rates (1/T2) were calculated. Serum ferritin levels were recorded. Bone marrow hypointensity in at least T2*-w GRE sequence was noted in 29/40 (72.5%) patients. Eleven/40 patients exhibited normal B/M on all MR sequences. Five/40 patients had normal B/M and low L/M. B/M correlated with L/M in T1-w TSE sequence only (r = 0.471, p = 0.05). B/M correlated with S/M and mean ferritin values in all sequences (r > 0.489, p < 0.01 and r > - 0.496, p < 0.03 respectively). Marrow 1/T2 did not correlate with ferritin values or liver and spleen 1/T2. B/M in transfused beta-thalassemic patients is related to splenic siderosis and ferritin levels. Although marrow is usually hypointense, it may occasionally display normal SI coexisting with liver hypointensity, a pattern typical of primary hemochromatosis. (orig.)

Drakonaki, Eleni E.; Karantanas, Apostolos H. [University Hospital of Heraklion, Radiology Department, Heraklion, Crete (Greece); Maris, Thomas G. [University of Crete, Department of Medical Physics, Heraklion, Crete (Greece); Papadakis, Alex [Venizelion General Hospital, Heraklion, Crete (Greece)

2007-08-15

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Bone marrow changes in beta-thalassemia major: quantitative MR imaging findings and correlation with iron stores  

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The purpose of this study is to describe the MR imaging features of bone marrow in beta-thalassemia major and investigate their relation to ferritin, liver and spleen siderosis. Spinal bone marrow was prospectively assessed on abdominal MR studies of 40 transfused beta-thalassemic patients and 15 controls using T1-w, Pd, T2*-w Gradient Echo (GRE) and T1-w turbo Spin Echo (TSE) sequences. Signal intensity (SI) ratios of liver, spleen and bone marrow to paraspinous muscles (L/M, S/M, B/M respectively) and the respective T2 relaxation rates (1/T2) were calculated. Serum ferritin levels were recorded. Bone marrow hypointensity in at least T2*-w GRE sequence was noted in 29/40 (72.5%) patients. Eleven/40 patients exhibited normal B/M on all MR sequences. Five/40 patients had normal B/M and low L/M. B/M correlated with L/M in T1-w TSE sequence only (r = 0.471, p = 0.05). B/M correlated with S/M and mean ferritin values in all sequences (r > 0.489, p - 0.496, p < 0.03 respectively). Marrow 1/T2 did not correlate with ferritin values or liver and spleen 1/T2. B/M in transfused beta-thalassemic patients is related to splenic siderosis and ferritin levels. Although marrow is usually hypointense, it may occasionally display normal SI coexisting with liver hypointensity, a pattern typical of primary hemochromatosis. (orig.)

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Amelioration of oxidative stress in red blood cells from patients with beta-thalassemia major and intermedia and E-beta-thalassemia following administration of a fermented papaya preparation.  

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In beta-hemoglobinopathies, such as beta-thalassemia (thal) and sickle cell anemia, the primary defects are mutations in the beta-globin gene. However, many aspects of the pathophysiology are mediated by oxidative stress. Fermented papaya preparation (FPP), a natural health food product obtained by biofermentation of carica papaya, has been shown to limit oxidative stress both in vitro and in vivo. We studied the effect of FPP on two groups of beta-thal patients: beta-thal, major and intermedia, (in Israel) and E-beta-thal (in Singapore). The results indicated that in both groups FPP treatment increased the content of reduced glutathione (GSH) in red blood cells (RBC), and decreased their reactive oxygen species (ROS) generation, membrane lipid peroxidation, and externalization of phosphatidylserine (PS), indicating amelioration of their oxidative status, without a significant change in the hematological parameters. Since the turnover of the erythron is relatively slow, it is possible that longer duration of treatment, probably with the addition of an iron chelator, is required in order to achieve the latter goals. PMID:20127662

Fibach, Eitan; Tan, Ee-Shien; Jamuar, Saumya; Ng, Ivy; Amer, Johnny; Rachmilewitz, Eliezer A

2010-09-01

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Correlation of Echocardiography and MRI T2* in Beta- Thalassemia Major  

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Introduction:Transfusional therapy can lead to iron deposition and damage to the heart, liver and endocrine organs in thalassemia major patients. Cardiomyopathy is one of the major complications of ?-thalassemic patients, resulting from iron overload. It is one of the major causes of morbidity and mortality in these patients. It has been shown that there is no correlation between serum ferritin and liver and heart iron deposition. Endocardial biopsy for iron deposition is the most ac...

Maryam Barzan; Mehrnoosh Kowsarian; Rozita Jalalian; Shahram akhlaghpoor; Mehdi Taremi

2009-01-01

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Application of Single Strand Conformational Polymorphism (PCR-SSCP) in Identification of Some Beta-Globin Gene Mutations in A Group of Egyptian Beta-Thalassemia Patients and Carriers  

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The present study investigated whether the single-strand conformational polymorphism (SSCP) method could be employed to identify (rather than simply detect) four of the most common beta-globin gene mutations in the Egyptian population: IVS-I-110, IVS-I-6, the IVS-I-1, and Codon 39. Using DNA from 90 beta-thalassemia patients and carriers, by PCR the appropriate 238-bp region of the human beta-globin gene was amplified, the reaction products (Single-stranded DNA) were analyzed by none denaturing polyacrylamide gel electrophoresis, and the bands visualized by silver staining. Single-stranded DNA (ssDNA) fragments showed reproducible pattern of bands that were characteristic of the mutations present. With the use of control samples containing six of the 10 possible combinations of the four beta-globin gene mutations under study, we were able to predict the mutations present in 23 out of 90 (26.4%) of the patients studied. These predictions were confirmed independently by the amplification refractory mutation system (ARMS) method. It is concluded that this non-radioactive PCR-SSCP method can be used to reliably identify mutations in beta-thalassemia patients, provided that suitable controls are available. However, usefulness of this method for determining the genotype of beta-thalassaemic individuals is obviously limited by the great number of controls required. Moreover, the ability to detect mutations by SSCP is in general lower compared to other methods, ARMS, DGGE or compared to other methods, ARMS, DGGE or DHPLC, which are reported to detect 49.5% to 73% of the mutations present. The SSCP method is nevertheless much easier to employ than other methods and is especially successful for beta-thalassemia carriers. This method would thus be particularly useful for an initial screening of target groups (prenatal diagnosis)

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Assessment of Cognitive Function in Children With Beta-Thalassemia Major: A Cross-Sectional Study.  

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Multiple risk factors contribute to cognitive impairment in children with ?-thalassemia major. For a more refined understanding of this issue, we attempted to evaluate cognitive function in ?-thalassemia major patients and identify the relationship between possible cognitive dysfunction and the following: demography, transfusion and chelation characteristics, iron overload, and disease complications. We studied 100 ?-thalassemia major children and 100 healthy controls who matched well in terms of age, sex, and socioeconomic status. All participants underwent psychometric assessment using Wechsler Intelligence Scale for Children-Third Edition, Arabic version. The mean Full-Scale IQ and Performance IQ of patients were significantly lower than those of controls, whereas no significant difference was found for Verbal IQ. No significant relationship existed between IQ and any of the assessed parameters. We concluded that Performance IQ, not Verbal IQ, was significantly affected in ?-thalassemia major patients, but there was no clear association between IQ and any of the parameters. PMID:25296920

Raafat, Nelly; El Safy, Usama; Khater, Nahed; Hassan, Tamer; Hassan, Basheir; Siam, Ahmed; Youssef, Amira; El Shabrawy, Amany

2014-10-01

61

Comparative evaluation of renal findings in Beta-thalassemia major and intermedia.  

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Thalassemia is a systematic disease in which the renal involvement has not yet been scrupulously studied. In a cross-sectional study, the renal findings of 50 cases of thalassemia intermedia (group 1) were compared to 58 patients with thalassemia major (group 2). Blood urea nitrogen, serum creatinine, uric acid, calcium, phosphorus, urinalysis, and ultrasonographical findings were evaluated. Mean age was 18 +/- 3.0 in group 1 and 17 +/- 3.5 years in group 2. The mean of serum ferritin levels was 871 +/- 81.8 ng/ml in group 1 vs. 3503 +/- 201 ng/ml in thalassemia major (p > 5) was observed among 19 children (17.6%); 17 of them were in group 1. In contrast, children with thalassemia major had significantly higher serum creatinine (0.89 +/- 0.18 vs. 0.59 +/- 0.37 mg/dl, p < 0.05) and blood urea nitrogen values (12.14 +/- 5.58 vs. 13.85 +/- 3.54 mg/dl, p < 0.05). We conclude that significant renal involvement is not a frequent complication in children and young adults suffering from thalassemia. Hyperuricemia and microscopic hematuria are more common in thalassemia intermedia than thalassemia major. Microscopic hematuria in thalassemia intermedia might be related to either hypercalciuria or hyperuricosuria. PMID:18310868

Ali, Derakhshan; Mehran, Karimi; Moghaddam, Abdolkarim Ghadimi

2008-03-01

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Correlation of Echocardiography and MRI T2* in Beta- Thalassemia Major  

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Full Text Available Introduction:Transfusional therapy can lead to iron deposition and damage to the heart, liver and endocrine organs in thalassemia major patients. Cardiomyopathy is one of the major complications of ?-thalassemic patients, resulting from iron overload. It is one of the major causes of morbidity and mortality in these patients. It has been shown that there is no correlation between serum ferritin and liver and heart iron deposition. Endocardial biopsy for iron deposition is the most accurate test in this regard, which is invasive. Iron shortens MRI parameters T1, T2 and T2* in the involved organs, which creates a potential mechanism for iron quantification. MRI T2* has especially been shown to have an inverse correlation with iron overload in the myocardium. Values less than 20 ms show significant iron deposition. Recently echocardiographic measures have been introduced to estimate the iron status of the myocardium. As diastolic dysfunction may precede systolic dysfunction, echocardiographic indices of diastolic function may be compared with myocardial MRI T2*."nMaterials and Methods: In this study, 35 major thalassemic patients from thalassemia research center at Bou Ali Sina hospital, Sari were enrolled. They have been under transfusion for a mean time of 15 years. Informed consent was filled and echocardiography and MRI T2* has been performed for all patients. The result for each test and the correlation of both procedures are shown in correlation with cardiac performance.

Maryam Barzan

2009-01-01

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Cord Compression due to Extramedullary Hematopoiesis in an Adolescent with Known Beta Thalassemia Major  

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Full Text Available We describe a 16 year-old male with ß thalassemia major and gait disturbances that had not been given blood transfusions due to a severe childhood transfusion reaction. Thoracic spine MRI demonstrated hematopoietic marrow throughout the spine and epidural masses causing cord compression consistent with extramedullary hematopoiesis (EMH. After treatment with steroids, radiotherapy and monitored blood transfusions, the patient demonstrated significant improvement of his paraspinal lesions and near complete resolution of his neurological symptoms. While EMH causing cord compression in adolescents is rare in the current era of bone marrow transplantation or chronic transfusions, it should be considered when thalassemia major patients present with neurological deficits. The well defined imaging features of EMH can play a central role in its diagnosis and management, especially because surgical and / or radiotherapeutic intervention are often considered in cases of failed medical treatment.

Alan COHLER

2009-01-01

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Effects of Vitamin E and Zinc Supplementation on Antioxidants in Beta-Thalassemia Major Patients  

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Objective:In beta thalassemic patients, tissue damage occurs due to oxidative stress and it happens because of the accumulation of iron in the body. This study was conducted to determine the effect of zinc and vitamin E supplementation on antioxidant status in beta-thalassemic major patients. Methods:This double blind randomized clinical trial was carried out on 120 beta thalassemic patients older than 18 years. Patients were randomly categorized in four groups. Zinc (50mg/day) and vitamin E ...

Abbasali Keshtkar; Seyed-Rafie Arefhosseini Arefhosseini; Maryam Rafraf; Maryam Aboomardani; Mohammadreza Rashidi; Hamidreza Joshaghani

2011-01-01

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Comparative Evaluation of Renal Findings in Beta-Thalassemia Major and Intermedia  

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Full Text Available Thalassemia is a systematic disease in which the renal involvement has not yet been scrupulously studied. In a cross-sectional study, the renal findings of 50 cases of thalassemia intermedia (group 1 were compared to 58 patients with thalassemia major (group 2. Blood urea nitrogen, serum creatinine, uric acid, calcium, phosphorus, urinalysis, and ultrasonographical findings were evaluated. Mean age was 18 ± 3.0 in group 1 and 17 ± 3.5 years in group 2. The mean of serum ferritin levels was 871 ± 81.8 ng/ml in group 1 vs. 3503 ± 201 ng/ml in thalassemia major (p < 0.05. Ninety-two percent of the patients in group 1 were on hydroxyurea at the time of evaluation. Serum uric acid was significantly higher in group 1 than group 2 patients (5.74 ± 2.95 vs. 4.12 ± 0.9 mg/dl, p < 0.05. Microscopic hematuria (red blood cell in high power field of urine microscopy > 5 was observed among 19 children (17.6%; 17 of them were in group 1. In contrast, children with thalassemia major had significantly higher serum creatinine (0.89 ± 0.18 vs. 0.59 ± 0.37 mg/dl, p < 0.05 and blood urea nitrogen values (12.14 ± 5.58 vs. 13.85 ± 3.54 mg/dl, p < 0.05. We conclude that significant renal involvement is not a frequent complication in children and young adults suffering from thalassemia. Hyperuricemia and microscopic hematuria are more common in thalassemia intermedia than thalassemia major. Microscopic hematuria in thalassemia intermedia might be related to either hypercalciuria or hyperuricosuria.

Ali Derakhshan

2008-01-01

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Major Beta-thalassemia: Protective or predisposing Factor for Cardiovascular Diseases  

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Full Text Available Introduction: Cardiac dysfunctions have been well known in patients with Major thalassemia. Some studies have focused on differences in blood pressure and heart rate between these patients and normal population, while this view has not been proven in other studies. Given the importance of hemodynamic factors in the health of these individuals, we intend to test the hypothesis as to whether or not hemodynamic factors of these patients differ from normal subjects.Methods: Patients were selected from among the thalassemic patients referred to a blood clinic of one of the third-level hospitals in Tehran. Finally, 50 patients and 50 normal subjects were studied in two groups. All subjects have been assessed using Holter monitoring. Mean average systolic blood pressure and heart rate of subjects were recorded for a period of 24 hours.In addition, demographic data on the subjects, and some laboratory tests (such as serum lipid profile and glucose levels of the patients were also determined and compared between the two groups.Results: 100 people were evaluated in two groups, including 50 patients with thalassemia major and 50 normal subjects, matched by age and gender. The mean age of the subjects was 16.3 ± 6.7 years; and there is no significant difference in mean age of both groups.23 female patients (46% were in the case group, and 21 female patients (42% were in the healthy group, the ratio for which there is no statistically significant difference between the two groups (P> 0. 05. The average level of serum glucose, lipid profiles, as well as none of the hemodynamic factors had no statistically significant difference in the two groups.Only the mean systolic blood pressure was significantly lower in male patients than among men without the disease. Discussion: Despite the lack of difference in blood pressure between the case group and others, it seems that gender could plays a protective role against cardiovascular diseases in men with thalassemia major.

Mitra Kazemi Jahromi

2011-01-01

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Post-transfusion hypertension, convulsion and intracranial haemorrhage in beta-thalassemia major  

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The haematologic disorder b-thalassemia major is common in Pakistan. We describe a patient with undiagnosed thalassemia presenting with hypertension and convulsions and found to have cerebral haemorrhage on neuro-imaging. He had been transfused 2 weeks before this illness. Our experience is similar to a few case reports described in literature that were found to have cerebral haemorrhages post-mortem after a similar clinical presentation. All patients had a blood transfusion within 2 weeks prior to the presentation so association with transfusion has been proposed. We have reviewed the several mechanisms presented and discussed the findings. (author)

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Effects of Vitamin E and Zinc Supplementation on Antioxidants in Beta-Thalassemia Major Patients  

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Full Text Available Objective:In beta thalassemic patients, tissue damage occurs due to oxidative stress and it happens because of the accumulation of iron in the body. This study was conducted to determine the effect of zinc and vitamin E supplementation on antioxidant status in beta-thalassemic major patients. Methods:This double blind randomized clinical trial was carried out on 120 beta thalassemic patients older than 18 years. Patients were randomly categorized in four groups. Zinc (50mg/day and vitamin E (400mg/day supplements were administered for former and latter group, respectively. In the third group both supplements were administered in similar doses. The fourth (control group received no supplement. The effect of supplementations on serum zinc and vitamin E, superoxide dismutase (SOD, glutathione peroxidase (GPX, total antioxidant capacity (TAC and body mass index (BMI were measured at the beginning and the end of the study. Findings:Serum zinc levels in group 1 and 3 were significantly increased (P<0.007 and P<0.005, respectively. Serum vitamin E levels in group 2 and 3 were also increased significantly (P<0.001. Mean GPX activity in group1, 2 and 3 decreased significantly (P<0.015, P<0.032 and P<0.029, respectively. Mean SOD activity and TAC did not show significant change after supplementation. BMI had significant increase in all treated groups (P<0.001. Conclusion:Our results suggest that beta thalassemic patients have enhanced oxidative stress and administration of selective antioxidants may preclude oxidative damage.

Abbasali Keshtkar

2011-03-01

69

Effects of Vitamin E and Zinc Supplementation on Antioxidants in Beta-Thalassemia Major Patients  

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Full Text Available Objective:In beta thalassemic patients, tissue damage occurs due to oxidative stress and it happens because of the accumulation of iron in the body. This study was conducted to determine the effect of zinc and vitamin E supplementation on antioxidant status in beta-thalassemic major patients.Methods:This double blind randomized clinical trial was carried out on 120 beta thalassemic patients older than 18 years. Patients were randomly categorized in four groups. Zinc (50mg/day and vitamin E (400mg/day supplements were administered for former and latter group, respectively. In the third group both supplements were administered in similar doses. The fourth (control group received no supplement. The effect of supplementations on serum zinc and vitamin E, superoxide dismutase (SOD, glutathione peroxidase (GPX, total antioxidant capacity (TAC and body mass index (BMI were measured at the beginning and the end of the study.Findings:Serum zinc levels in group 1 and 3 were significantly increased (P< 0.007 and P< 0.005, respectively. Serum vitamin E levels in group 2 and 3 were also increased significantly (P< 0.001. Mean GPX activity in group1, 2 and 3 decreased significantly (P< 0.015, P< 0.032 and P< 0.029, respectively. Mean SOD activity and TAC did not show significant change after supplementation. BMI had significant increase in all treated groups (P< 0.001.Conclusion:Our results suggest that beta thalassemic patients have enhanced oxidative stress and administration of selective antioxidants may preclude oxidative damage

Mohammadreza Rashidi

2011-03-01

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Gilbert syndrome associated with beta-thalassemia.  

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The authors investigated whether the considerable variability in serum bilirubin levels (STB) found in transfusion-dependent beta-thalassemia, beta-thal intermedia, and heterozygous beta-thalassemia individuals could be related to the coexistence of Gilbert syndrome (GS). The promoter region [A(TA)nTAA] of the bilirubin UDP-glucuronosyltransferase gene (UGT1A1) was analyzed in a total of 128 beta-thalassemia individuals (108 transfusion-dependent beta-thal patients, 20 very mild beta-thal intermedia) and in 33 beta-thal heterozygotes. The control group consisted of 70 healthy children with no history of anemia. The frequency of GS genotype (TA)7/(TA)7 did not differ significantly between the groups studied. A significant difference was observed between serum bilirubin levels (STB) and GS genotypes (TA)7/(TA)7 and (TA)6/(TA)7 and also between (TA)7/(TA)7 and (TA)6/(TA)6 for all groups examined. These results confirm that the (TA)7/(TA)7 GS genotype is one of the factors accounting for the hyperbilirubinemia observed in beta-thalassemia major, intermedia, and heterozygous individuals. PMID:11764096

Tzetis, M; Kanavakis, E; Tsezou, A; Ladis, V; Pateraki, E; Georgakopoulou, T; Kavazarakis, E; Maragoudaki, E; Karpathios, T; Kitsiou-Tzeli, S

2001-12-01

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Efficacy of Carvedilol in Patients with Dilated Cardiomyopathy due to Beta-thalassemia Major a Double-blind Randomized Controlled Trial  

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Full Text Available Objective:Dilated cardiomyopathy is the end result of chronic iron overload in patients with beta thalassemia major. The objective of the present study was to evaluate the safety and efficacy of Carvedilol in patients with beta thalassemia major and dilated cardiomyopathy.Methods:During a six-month period, fourteen patients with beta-thalassemia major and heart failure without diabetes mellitus referred to pediatric cardiology clinic enrolled in this double blind, randomly assigned study. All patients were on anti failure therapy with Digoxin, Captopril and Furosemide. Carvedilol was started at a dosage of 3.12 mg bid and for patients who had a systolic blood pressure >100 mmHg, heart rate >60/min and no signs of low cardiac output the dosage was increased every two weeks to a maximum of 25 mg bid. Clinical signs and symptoms, systolic and diastolic echocardiographic indexes and Tissue Doppler Imaging (TDI data were collected from each patient.Findings:Eight patients received Carvedilol (Group 1 and six received placebo (Group 2. The mean age of patients in Group1 and 2 were 16?0.7 years and 17?3 years respectively. Only one patent in Group 1 tolerated increasing Carvedilol dosage to more than 6.25 mg bid. Changes in New York Heart Association (NYHA classification, Ejection fraction, End diastolic dimension changes, TDI systolic(S, early (Ea and late (Aa diastolic waves were not statistically significant in these two Groups (P>0.05. Pulse Doppler E/A wave ratio of mitral valve in Group1 and Group 2 changed from 1.1?0.37 m/s to 1.8?0.40 m/s and from 1.34?0.30 m/s to 2.6?0.23m/s respectively (P=0.04.Conclusion:Patients with thalassemia and dilated cardiomyopathy have poor tolerance to increasing Carvedilol dosage and develop decreased systolic blood pressure during advancement of the drug dosage. Carvedilol can be effective in prevention of progression of diastolic dysfunction in these patients.

Gholam-Hossein Ajami

2010-09-01

72

No evidence of xenotropic murine leukemia virus-related virus infection in Brazilian multiply transfused patients with sickle cell disease and beta-thalassemia major.  

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Although xenotropic murine leukemia virus-related virus (XMRV) has been regarded as a laboratory contaminant, it remains one of the most controversial viruses. The objective of the study was to determine if XMRV is present in 44 patients with beta-thalassemia major, 48 with sickle cell disease, and 89 volunteer blood donors. After RNA/ DNA extraction from plasma/buffy coat the samples were screened for XMRV sequences by conserved nested GAG primers. None of the RNA samples showed a positive result. Surprisingly, four DNA samples obtained from blood donors were positive for XMRV provirus. The subsequent phylogenetic analysis revealed that these sequences are identical to the positive control (murine leukemia retrovirus) and are probably consistent with laboratory contamination. XMRV infection (provirus and viral RNA) was absent in multiply transfused patients and volunteer blood donors. The positive result obtained from some blood donors probably reflects laboratory contamination. We believe that XMRV does not pose risk to blood transfusion. PMID:25387292

Slavov, Svetoslav Nanev; Otaguiri, Katia Kaori; Macedo, Mayra Dorigan; Rocha-Júnior, Maurício Cristiano; Silva-Pinto, Ana Cristina; Kashima, Simone; Covas, Dimas Tadeu

2014-10-01

73

Directed sibling donor cord blood banking for children with beta-thalassemia major in Greece: usage rate and outcome of transplantation for HLA-matched units.  

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Several cord blood banks store cord blood units from healthy siblings of patients, who are candidates for stem cell transplantation. We analyzed the quality characteristics of 50 cord blood units collected from families with beta-thalassemia major and the outcome of subsequent stem cell transplantations during a 15-year period. All cord blood units were found suitable for banking based on a minimum net volume of 40 ml. The mean volume of the units was 98.9 ml; the mean total nucleated cell count (NC) was 7.8 x 10(8) and the mean CD34+ cell count was 2.8 x 10(6). Eight out of twelve HLA matched collections were released for transplantation. All but one recipient belonged to Pesaro II-III risk classes. Three patients received a cord blood graft with >5 x 10(7) NC/kg . One of them with Pesaro class I disease engrafted, whereas the other two who failed to engraft, were re-transplanted with bone marrow from the same donor later. Cord blood grafts containing NCs bone marrow from the same donor were used in all 5 remaining cases and stable engraftment was achieved. All patients survived, 7/8 thalassemia-free. Cord blood banking from healthy siblings of children with beta-thalassemia major can result in a successful transplantation in cases in which there is HLA compatibility. However, in high-risk patients, the use of combined cord blood and bone marrow grafts seems necessary in order to ensure stable engraftment, especially when cord blood unit cell counts are low. PMID:19931473

Goussetis, Evgenios; Petrakou, Eftichia; Theodosaki, Maria; Kitra, Vasiliki; Peristeri, Ioulia; Vessalas, George; Dimopoulou, Maria N; Spiropoulos, Antonia; Papassavas, Andreas C; Stavropoulos-Giokas, Catherine; Graphakos, Stelios

2010-01-01

74

Erythroblastic inclusions in dominantly inherited beta thalassemias.  

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While the precipitation of unstable variant beta-globin chains has been implicated as a major pathogenic mechanism in dominantly inherited beta thalassemia, their instability and presence in intra-erythroblastic inclusions have not been conclusively shown. We report the investigation of two cases of dominantly inherited beta thalassemia due to heterozygosity for the beta-codon 121 G-T mutation. In one case, we were able to demonstrate the presence of an abnormal beta-globin chain in both peripheral blood reticulocytes and bone marrow erythroblasts, and to assess its stability in relation to the substantial amounts of mutant beta mRNA transcript. The serum transferrin receptor (TfR) level was markedly increased, an indication of increased erythropoietic activity. In both cases, we could show by immunoelectron microscopy that the intra-erythroblastic inclusion bodies, a prominent feature of diseases in this category, contained not only precipitated alpha-globin chains, but also beta chains. The data confirm previous suggestions that the cellular pathology underlying this group of beta thalassemias is related to the synthesis of highly unstable beta-globin chain variants, which fail to form functional tetramers and precipitate intracellularly with the concomitant excess alpha chains, leading to increased ineffective erythropoiesis. PMID:8978308

Ho, P J; Wickramasinghe, S N; Rees, D C; Lee, M J; Eden, A; Thein, S L

1997-01-01

75

[Beta-thalassemias: molecular, epidemiological, diagnostical and clinical aspects].  

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Beta-thalassemia is one of most common autosomal recessive disorders worldwide. In France, 5 to 10 new major or intermedia forms are diagnosed annually and the global prevalence is about 500 cases. Since 20 years and thanks to the generalization of iron chelator treatments, the life expectancy has dramatically increased. Nearly 90% of the ?-thalassemic alleles are point mutations easily identified by Sanger sequencing or dedicated methods. The remaining 10% are deletions detectable by MLPA or CGH Array. The alpha-globin genotype is also essential in the exploration of beta-thalassemia because an alpha-thalassemia improves the clinical state whereas an alpha triplication worsens it. The additional genotyping of a few HbF inducer polymorphisms allows to predict the age of the first transfusion, thanks to a recent dedicated algorithm, making beta-thalassemia one of the first potential application of predictive medicine. Gene therapy, pre-implantatory diagnosis and new drugs (Sotatercept®, hepcidin-like molecules) have also recently contributed to make beta-thalassemia a main scientific topic again. PMID:25486662

Joly, Philippe; Pondarre, Corinne; Badens, Catherine

2014-01-01

76

Co-existence of Phenylketonuria (PKU) and beta-Thalassemia Major in a 16 Years Old Girl: A Case Report  

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While thalassemia major (TM) used to be a prevalent genetic disease in the past, however, (PKU) is quite rare in spite of consanquiness marriage rate of about 40% in the region. Preventive efforts for TM started >20 years ago but neonatal screening for PKU started since 2007. This is the first report of co-existence of thalassemia and PKU in Middle East and in consideration of the prevalence of each genes, this chance association is a very unusual event. We report a case of having PKU and TM

Hossein Karami1; Mehrnoush Kosaryan; Aili Aliasgharian; Ali Abbaskhanian; Rayka Sharifian; Mehrdad Taghipour

2012-01-01

77

Co-existence of Phenylketonuria (PKU and beta-Thalassemia Major in a 16 Years Old Girl: A Case Report  

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Full Text Available While thalassemia major (TM used to be a prevalent genetic disease in the past, however, (PKU is quite rare in spite of consanquiness marriage rate of about 40% in the region. Preventive efforts for TM started >20 years ago but neonatal screening for PKU started since 2007. This is the first report of co-existence of thalassemia and PKU in Middle East and in consideration of the prevalence of each genes, this chance association is a very unusual event. We report a case of having PKU and TM

Hossein Karami

2012-07-01

78

Renal tubular dysfunction with nephrocalcinosis in a patient with beta thalassemia minor  

OpenAIRE

Thalassemia is a hereditary anemia resulting from defect in hemoglobin production. Beta thalassemia is due to impaired production of beta globin chains, leading to a relative excess of alpha globin chains. The term beta thalassemia minor is used to describe heterozygotes, who carry one normal beta globin allele and one beta thalassemic allele. The vast majority of these patients are asymptomatic. However, a variety of renal tubular abnormalities including hypercalciuria, hypo-magnesemia with ...

Prabahar Murugesan; Jain Manish; Chandrasekaran Venkatraman; Indhumathi Elayaperumal; Soundararajan Periasamy

2008-01-01

79

Techniques and clinical outcomes of laparoscopic cholecystectomy in adult patients with beta-thalassemias.  

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Beta-thalassemia, which results from a reduced production of beta-globin chain of hemoglobin, is a common single gene disorder with an extremely heterogeneous clinical picture. Its presentation may vary from mild anemia in beta-thalassemia minor to severe and life-threatening anemia in beta-thalassemia major. Recent advances in supportive treatment of beta-thalassemia major have resulted in substantial increase in survival in these patients, and an increasing number of these patients reach adolescence and adulthood. The incidence of cholelithiasis is reported to be increased in these patients. Although laparoscopic cholecystectomy (LC) has become the gold standard treatment of symptomatic gallstone disease, its experience in adult beta-thalassemic patients has been limited. From May 1992 through April 2000, 10 consecutive adult beta-thalassemic patients with symptomatic gallstone underwent LC at our institution. Data were obtained on the type of beta-thalassemia, presentation, preoperative laboratory findings, history of preoperative transfusion, postoperative complications, postoperative analgesic requirement, length of hospital stay, and follow-up. All operations were completed laparoscopically. The mean operative time was 98.5 minutes. The postoperative analgesic requirement was minimal. There was no mortality. One patient developed fever postoperatively due to lung atelectasis that was managed conservatively. The mean hospital stay was 3 days. Laparoscopic cholecystectomy is feasible, safe, and effective in the treatment of adult beta-thalassemic patients with symptomatic gallstone disease. Technical adjustments are required when operating on patients with beta-thalassemia major. PMID:12819500

Kok, Kenneth Y Y; Yapp, Samuel K S

2003-06-01

80

Sustained improvements in myocardial T2* over 2 years in severely iron-overloaded patients with beta thalassemia major treated with deferasirox or deferoxamine.  

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Long-term controlled studies are needed to inform on the clinical benefit of chelation therapy for myocardial iron removal in transfusion-dependent beta thalassemia patients. In a 1-year nonrandomized extension to the CORDELIA study, data collected from patients with myocardial siderosis provided additional information on deferasirox or deferoxamine (DFO) efficacy and safety. Myocardial (m)T2* increased from baseline 11.6 to 15.9 ms in patients receiving deferasirox for 24 months (n = 74; geometric mean [Gmean ] ratio of month 24/baseline 1.38 [95% confidence interval 1.28, 1.49]) and from 10.8 to 14.2 ms in those receiving DFO (n = 29; Gmean ratio 1.33 [1.13, 1.55]; P = 0.93 between groups). Improved mT2* with deferasirox was evident across all subgroups evaluated irrespective of baseline myocardial (mT2* < 10 vs. ? 10 ms) or liver (LIC <15 vs. ?15 mg Fe/g dw) iron burden. Mean LVEF was stable and remained within normal limits with deferasirox or DFO. Liver iron concentration decreased from high baseline values of 30.6 ± 18.0 to 14.4 ± 16.6 mg Fe/g dw at month 24 in deferasirox patients and from 36.8 ± 15.6 to 11.0 ± 12.1 mg Fe/g dw in DFO patients. The long-term safety profile of deferasirox or DFO was consistent with previous reports; serious drug-related AEs were reported in 6.8% of deferasirox and 6.9% of DFO patients. Continued treatment of severely iron-overloaded beta thalassemia patients with deferasirox or DFO led to sustained improvements in myocardial iron irrespective of high or low baseline myocardial or liver iron burden, in parallel with substantial improvements in liver iron (Clinicaltrials.gov identifier: NCT00600938). PMID:25345697

Pennell, Dudley J; Porter, John B; Piga, Antonio; Lai, Yong-Rong; El-Beshlawy, Amal; Elalfy, Mohsen; Yesilipek, Akif; Kilinç, Yurdanur; Habr, Dany; Musallam, Khaled M; Shen, Junwu; Aydinok, Yesim

2015-02-01

81

Resistance to infection in murine beta-thalassemia.  

OpenAIRE

Clinical evidence suggests that individuals with chronic iron overload may be at increased risk of bacterial infection. We studied this question by using a unique model in which mice homozygous for a deletion in the gene encoding for the beta-major globin develop moderate anemia, splenomegaly, and tissue iron overload, a syndrome similar to beta-thalassemia in humans. Mice heterozygous for the gene deletion were phenotypically normal. Homozygous mice were significantly more susceptible to inf...

Ampel, N. M.; Wyck, D. B.; Aguirre, M. L.; Willis, D. G.; Popp, R. A.

1989-01-01

82

Vitamin E in beta-thalassemia.  

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In homozygous beta-thalassemia low serum level of alpha-tocopherol have been found. The administration of high doses of the vitamin increased the serum level, decreased lipid peroxidation and, in some case, prolonged red blood cell survival; no significant change in transfusion requirement was obtained. Only few data are available about the vitamin E in heterozygous beta-thalassemia. We have studied 131 patients aged 1 to 72 years with thalassemic trait and 218 age-matched controls. Serum lev...

Ghigo, Dario Antonio

1982-01-01

83

Guidelines for diagnosis and management of Beta-thalassemia intermedia.  

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Beta-thalassemia intermedia (?-TI) is a genetic variant of beta-thalassemias with a clinical disorder whose severity falls between thalassemia minor and thalassemia major. Different genetic defects are involved in this disorder and, based on severity of disease, clinical complications like skeletal deformities and growth retardation, splenomegaly, extramedullary hematopoiesis, heart failure, and endocrine disorders may be present in untreated patients. Precise diagnosis and management are essential in these patients for prevention of later clinical complications. Diagnosis of TI is based on clinical and laboratory data. There are some treatment strategies like modulation of gamma-globulin chain production with hydroxyurea or other drugs, transfusion, splenectomy, and stem cell transplantation. Iron chelation therapy is also needed in many of these patients even if they are not transfused. The aim of this manuscript is to review the clinical manifestations, complications, genetic defects, and unmet treatments needs in TI. PMID:25247665

Karimi, Mehran; Cohan, Nader; De Sanctis, Vincenzo; Mallat, Naji S; Taher, Ali

2014-10-01

84

Plastic bronchitis in beta thalassemia minor  

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Plastic bronchitis is a rare pulmonary disorder associated with various conditions like cystic fibrosis, asthma, pulmonary infection and characterized by formation and expectoration of cast which assumes the shape of the bronchial tree. We report a case of a 33-year-old woman with beta thalassemia minor who developed plastic bronchitis.

Yadav, Makaresh; Tirpude, Sneha; Joshi, Jyotsna M.

2013-01-01

85

Molecular analysis of beta-globin gene mutations among Thai beta-thalassemia children: results from a single center study  

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Full Text Available Boonchai Boonyawat,1 Chalinee Monsereenusorn,2 Chanchai Traivaree2 1Division of Genetics, Department of Pediatrics, Phramongkutklao Hospital and College of Medicine, Bangkok, Thailand; 2Division of Hematology/Oncology, Department of Pediatrics, Phramongkutklao Hospital and College of Medicine, Bangkok, Thailand Background: Beta-thalassemia is one of the most common genetic disorders in Thailand. Clinical phenotype ranges from silent carrier to clinically manifested conditions including severe beta-thalassemia major and mild beta-thalassemia intermedia. Objective: This study aimed to characterize the spectrum of beta-globin gene mutations in pediatric patients who were followed-up in Phramongkutklao Hospital. Patients and methods: Eighty unrelated beta-thalassemia patients were enrolled in this study including 57 with beta-thalassemia/hemoglobin E, eight with homozygous beta-thalassemia, and 15 with heterozygous beta-thalassemia. Mutation analysis was performed by multiplex amplification refractory mutation system (M-ARMS, direct DNA sequencing of beta-globin gene, and gap polymerase chain reaction for 3.4 kb deletion detection, respectively. Results: A total of 13 different beta-thalassemia mutations were identified among 88 alleles. The most common mutation was codon 41/42 (-TCTT (37.5%, followed by codon 17 (A>T (26.1%, IVS-I-5 (G>C (8%, IVS-II-654 (C>T (6.8%, IVS-I-1 (G>T (4.5%, and codon 71/72 (+A (2.3%, and all these six common mutations (85.2% were detected by M-ARMS. Six uncommon mutations (10.2% were identified by DNA sequencing including 4.5% for codon 35 (C>A and 1.1% initiation codon mutation (ATG>AGG, codon 15 (G>A, codon 19 (A>G, codon 27/28 (+C, and codon 123/124/125 (-ACCCCACC, respectively. The 3.4 kb deletion was detected at 4.5%. The most common genotype of beta-thalassemia major patients was codon 41/42 (-TCTT/codon 26 (G>A or betaE accounting for 40%. Conclusion: All of the beta-thalassemia alleles have been characterized by a combination of techniques including M-ARMS, DNA sequencing, and gap polymerase chain reaction for 3.4 kb deletion detection. Thirteen mutations account for 100% of the beta-thalassemia genes among the pediatric patients in our study. Keywords: mutation analysis, beta-globin gene, Thai children

Boonyawat B

2014-12-01

86

Intracranial Extramedullary Hematopoiesis in Beta-Thalassemia  

OpenAIRE

Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of...

Karki, Bivek; Xu, Yi-kai; Tamrakar, Karuna; Wu, Yuan-kui

2012-01-01

87

Hematology of a murine. beta. -thalassemia: a longitudinal study  

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Mice homozygous for a spontaneous mutation, in which the ..beta..-major globin gene is deleted, have clinical symptoms of ..beta..-thalassemia. These mice have a hypocellular, hypochromic, microcytic anemia that becomes more severe with increasing age. The defective red cell morphology, decreased osmotic fragility of erythrocytes and shortened red cell life span found in ..beta..-thalassemic mice are similar to those observed in human ..beta..-thalassemia. Synthesis of ..beta..-globin is depressed but not as much as might be expected because the expression of the..beta..-minor globin gene is enhanced to encode two to three times more globin than in normal mice. Splenomegaly, an enlarged pool of stem cells for erythropoiesis, and iron overloading occur in older mice. The fact that these mice remain moderately healthy makes them a very suitable animal model in which to develop and test alternative techniques of gene therapy that could be successfully applied to the treatment of human thalassemia. Homozygous ..beta..-thalassemic mice have large deposits of iron in their tissues, which might make these mice also useful for in vivo tests of the effectiveness and possible long-term side effects for newly developed iron chelators.

Popp, R.A.; Popp, D.M.; Johnson, F.M.; Skow, L.C.; Lewis, S.E.

1986-01-01

88

Renal tubular dysfunction nephrocalcinosis in a patient with BetaThalassemia Minor  

International Nuclear Information System (INIS)

Thalassemia is a hereditary anemia resulting from defect in hemoglobinproduction. Beta thalassemia is due to impaired production. Beta thalassemiais due to impaired production of beta globin chains, leading to a relativeexcess of alpha globin chains. The term beta thalassemia minor is used todescribe heterozygotes, who carry one normal beta globin and one betathalassemic allele. The vast majority of these patients are asymptomatic.However, a variety of renal tubular abnormalities including hypercaliuria,hypomagnesemia with renal magnesium wasting, decreased tubular absorption ofphosphorous, hypouricemia with renal uric acid wasting, renal glycosuria andtubular proteinuria have been described even in patients with betathalassemia minor. We here in report a 24-year old patient who was found tohave thalassemia minor and nephrocalcinosis with evidence of renal tubulardysfunction. Investigations revealed normal renal function, hypercalciuria,reduced tubular reabsorption of phosphorous, hypomagnesemia and renalmagnesium wasting. Screening for aminoaciduria was found to be negative. Anacid loading test revealed normal urinary acidification. Ultrasonogram of theabdomen revealed nephrocalcinosis and splenomegaly. Detailed work up foranemia showed normal white cell and platelet count while peripheral smearshowed microcytic hypochromic anemia with few target cells. Hemoglobinelectrophoresis revealed hemoglobin A of 92%, hemoglobin A2 of 6.2% andhemoglobin F of 1.8% consistent wi6.2% andhemoglobin F of 1.8% consistent with beta thalassemia minor. Her parentalscreening was normal. A diagnosis of beta thalassemia minor with renaltubular dysfunction was made and the patient was started on thiazidediuretics to reduce hypercalciuria and advised regular follow-up. (author)

89

Association of Finger Ridge Pattern and E-Beta-Thalassemia: A Study on Bengalee Population of West Bengal, India  

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Full Text Available There is well documented relationship between dermatoglyphics and specific syndromes of genetic origins. Since beta-thalassemia is a major genetic disorder in West Bengal, India, therefore, rapid diagnosis of major beta thalassemia along with certain preventive measures is of utmost significance. The aim of the present study was to understand the association of the finger prints patterns among the E-beta thalassemia patients of Bengalee Hindu Caste population of West Bengal, India. To achieve the purpose, bilateral fingerprints have been collected from 100 (Male-50, Female-50 diagnosed E-beta thalassemia patients from Bengalee population in West Bengal using standard ink roller technique. Examination on finger pattern type revealed significantly (p < 0.05 higher Whorls among the male patients in comparison to their female counter part. On the other hand, significant (p < 0.05 excess of Arches has been found among the female patients compared to the male patients. Present study envisaged that these finger dermatoglyphics patterns might be helpful for the diagnosis and screening of E-beta thalassemia in Bengalee population.

Piyali Das

2015-02-01

90

Renal tubular dysfunction with nephrocalcinosis in a patient with beta thalassemia minor  

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Full Text Available Thalassemia is a hereditary anemia resulting from defect in hemoglobin production. Beta thalassemia is due to impaired production of beta globin chains, leading to a relative excess of alpha globin chains. The term beta thalassemia minor is used to describe heterozygotes, who carry one normal beta globin allele and one beta thalassemic allele. The vast majority of these patients are asymptomatic. However, a variety of renal tubular abnormalities including hypercalciuria, hypo-magnesemia with renal magnesium wasting, decreased tubular absorption of phosphorus, hypo-uricemia with renal uric acid wasting, renal glycosuria and tubular proteinuria have been described even in patients with beta thalassemia minor. We here in report a 24-year old female patient who was found to have thalassemia minor and nephrocalcinosis with evidence of renal tubular dysfunction. Investigations revealed normal renal function, hypercalciuria, reduced tubular reabsorption of phos-phorus, hypomagnesemia and renal magnesium wasting. Screening for aminoaciduria was found to be negative. An acid loading test revealed normal urinary acidification. Ultrasonogram of the abdomen revealed nephrocalcinosis and splenomegaly. Detailed work up for anemia showed normal white cell and platelet count while peripheral smear showed microcytic hypochromic anemia with few target cells. Hemoglobin electrophoresis revealed hemoglobin A of 92%, hemoglobin A2 of 6.2% and hemo-globin F of 1.8% consistent with beta thalassemia minor. Her parental screening was normal. A diag-nosis of beta thalassemia minor with renal tubular dysfunction was made and the patient was started on thiazide diuretics to reduce hypercalciuria and advised regular follow-up.

Prabahar Murugesan

2008-01-01

91

Intracranial Extramedullary Hematopoiesis in Beta-Thalassemia  

Energy Technology Data Exchange (ETDEWEB)

Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

Karki, Bivek; Xu, Yi Kai; Wu, Yuan Kui [Nan fang Hospital, Southern Medical University, Guangzhou (China); Tamrakar, Karuna [Zhujiang Hospital, Southern Medical University, Guangzhou (China)

2012-03-15

92

Intracranial Extramedullary Hematopoiesis in Beta-Thalassemia  

International Nuclear Information System (INIS)

Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

93

Investigation of RBC Indices and HbA2 Levels in Parents of Beta-Thalassemia Patients: Impacts on Premarital Genetic Counseling  

OpenAIRE

Objective: This study was designed to investigate RBC indices and HbA2 levels in parents of major beta-thalassemia patients to detect possible silent beta- thalassemia carriers and examine its potential impact on the premarital genetic counseling.Materials and Methods: This cross sectional study was performed at Children Medical Center from 2004 to 2006. After genetic counseling and getting informed consent, peripheral blood sampling was carried out on 185 carrier parents of regularly blood t...

Mina Izadyar; Jila Dastan; Tayebeh Sabokbar; Solmaz Shoraka; Azadeh Shojaei; Habib Nasiri; Saeed Reza Ghaffari

2007-01-01

94

Oxidative stress and antioxidant status in beta-thalassemia heterozygotes  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: English Abstract in english Background: Several studies have evaluated the oxidant and antioxidant status of thalassemia patients but most focused mainly on the severe and intermediate states of the disease. Moreover, the oxidative status has not been evaluated for the different beta-thalassemia mutations. Objective: To [...] evaluate lipid peroxidation and Trolox equivalent antioxidant capacity in relation to serum iron and ferritin in beta thalassemia resulting from two different mutations (CD39 and IVS-I-110) compared to individuals without beta-thalassemia. Methods: One hundred and thirty subjects were studied, including 49 who were heterozygous for beta-thalassemia and 81 controls. Blood samples were subjected to screening tests for hemoglobin. Allele-specific polymerase chain reaction was used to confirm mutations for beta-thalassemia, an analysis of thiobarbituric acid reactive species was used to determine lipid peroxidation, and Trolox equivalent antioxidant capacity evaluations were performed. The heterozygous beta-thalassemia group was also evaluated for serum iron and ferritin status. Results: Thiobarbituric acid reactive species (486.24 ± 119.64 ng/mL) and Trolox equivalent antioxidant capacity values (2.23 ± 0.11 mM/L) were higher in beta-thalassemia heterozygotes compared to controls (260.86 ± 92.40 ng/mL and 2.12 ± 0.10 mM/L, respectively; p-value

Luciana de Souza, Ondei; Isabeth da Fonseca, Estevao; Marina Ibelli Pereira, Rocha; Sandro, Percario; Doroteia Rossi Silva, Souza; Marcela Augusta de Souza, Pinhel; Claudia Regina, Bonini-Domingos.

95

Progress toward the genetic treatment of the beta-thalassemias.  

Science.gov (United States)

The beta-thalassemias are congenital anemias that are caused by mutations that reduce or abolish expression of the beta-globin gene. They can be cured by allogeneic hematopoietic stem cell (HSC) transplantation, but this therapeutic option is not available to most patients. The transfer of a regulated beta-globin gene in autologous HSCs is a highly attractive alternative treatment. This strategy, which is simple in principle, raises major challenges in terms of controlling expression of the globin transgene, which ideally should be erythroid specific, differentiation- and stage-restricted, elevated, position independent, and sustained over time. Using lentiviral vectors, May et al. demonstrated in 2000 that an optimized combination of proximal and distal transcriptional control elements permits lineage-specific and elevated beta-globin expression, resulting in therapeutic hemoglobin production and correction of anemia in beta-thalassemic mice. Several groups have by now replicated and extended these findings to various mouse models of severe hemoglobinopathies, thus fueling enthusiasm for a potential treatment of beta-thalassemia based on globin gene transfer. Current investigation focuses on safety issues and the need for improved vector production methodologies. The safe implementation of stem cell-based gene therapy requires the prevention of the formation of replication-competent viral genomes and minimization of the risk of insertional oncogenesis. Importantly, globin vectors, in which transcriptional activity is highly restricted, have a lesser risk of activating oncogenes in hematopoietic progenitors than non-tissue-specific vectors, by virtue of their late-stage erythroid specificity. As such, they provide a general paradigm for improving vector safety in stem cell-based gene therapy. PMID:16339654

Sadelain, Michel; Lisowski, Leszek; Samakoglu, Selda; Rivella, Stefano; May, Chad; Riviere, Isabelle

2005-01-01

96

Molecular basis for dominantly inherited inclusion body. beta. -thalassemia  

Energy Technology Data Exchange (ETDEWEB)

Analysis of the molecular basis of dominantly inherited {beta}-thalassemia in four families has revealed different mutations involving exon 3 of the {beta}-globin gene. It is suggested that the phenotypic difference between this condition and the more common recessive forms of {beta}-thalassemia lies mainly in the length and stability of the abnormal translation products that are synthesized and, in particular, whether they are capable of binding heme and producing aggregations that are relatively resistant to proteolytic degradation.

Thein, S.L.; Hesketh, C.; Wood, W.G.; Clegg, J.B.; Old, J.M.; Weatherall, D.J. (John Radcliffe Hospital, Oxford (England)); Taylor, P. (Royal Victoria Infirmary, Newcastle-upon-Tyne (England)); Temperley, I.J. (Saint James' s Hospital, Dublin (Ireland)); Hutchinson, R.M. (Leicester Royal Infirmary (England))

1990-05-01

97

Molecular characterization of beta-thalassemia in the Sardinian population  

Energy Technology Data Exchange (ETDEWEB)

This study reports the molecular characterization of [beta]-thalassemia in the Sardinian population. Three thousand [beta]-thalassemia chromosomes from prospective parents presenting at the genetic service were initially analyzed by dot blot analysis with oligonucleotide probes complementary to the most common [beta]-thalassemia mutations in the Mediterranean at-risk populations. The mutation which remained uncharacterized by this approach were defined by denaturing gradient gel electrophoresis (DGGE) followed by direct sequence analysis on amplified DNA. The authors reconfirmed that the predominant mutation in the Sardinian population is the codon 39 nonsense mutation, which accounts for 95.7% of the [beta]-thalassemia chromosomes. The other two relatively common mutations are frameshifts at codon 6 (2.1%) and at codon 76 (0.7%), relatively uncommon in other Mediterranean-origin populations. In this study they have detected a novel [beta]-thalassemia mutation, i.e., a frameshift at codon 1, in three [beta]-thalassemia chromosomes. The DGGE procedure followed by direct sequencing on amplified DNA is a powerful approach for the characterization of unknown mutations in this genetic system.

Rosatelli, M.C.; Faa, V.; Sardu, R.; Saba, L. Cao, A. (Universita degli Studi, Cagliari (Italy)); Dozy, A.; Kan, Y.W. (University of California, San Franciso (United States)); Meloni, A. (Instituto di Ricerca sulle Talassemie e Anemie Mediterranee CNR, Cagilari (Italy))

1992-02-01

98

Quality of life among Iranian patients with beta-thalassemia major using the SF-36 questionnaire / Qualidade de vida em pacientes iranianos com beta-talassemia maior usando o questionario SF-36  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: English Abstract in portuguese CONTEXTO E OBJETIVO Pacientes com beta-talassemia maior (?-TM) vivenciam problemas físicos, psicológicos e sociais que levam à diminuição da qualidade de vida (QV). O objetivo foi determinar a QV relacionada à saúde e seus determinantes em pacientes com ?-TM, utilizando questionário SF-36 (Short Fo [...] rm-36). TIPO DE ESTUDO E LOCAL Estudo transversal no Centro de Hematologia e Pesquisa em Universidade de Ciências Médicas de Shiraz, no sul do Irã. MÉTODOS Foram selecionados aleatoriamente 101 pacientes com ?-TM. Após registro demográfico e características da doença, eles foram convidados a preencher o questionário SF-36. A correlação entre fatores clínicos e demográficos com escore de QV foi avaliada. RESULTADOS Havia 44 homens e 57 mulheres, com idade média de 19,52 ± 4,3 (variação 12-38) anos. Em duas escalas, dor (P = 0,041) e aspectos emocionais (P = 0,009), as mulheres apresentaram escores significativamente menores aos dos homens. Menor renda, baixa adesão à terapia quelante de ferro e presença de comorbidades foram correlacionadas com escores SF-36 significativamente menores. Esses fatores foram também considerados determinantes de piores escores de SF-36 em análise multivariada. CONCLUSÕES Mostramos que a presença de complicações da doença, a baixa adesão ao tratamento da terapia quelante de ferro e o baixo status econômico são preditores de pior QV em pacientes com ?-TM. Prevenção e manejo adequado das complicações relacionadas com a doença, aumento do conhecimento dos pacientes sobre a importância do gerenciamento de comorbidades e ter maior adesão ao tratamento quelante de ferro, considerando também o apoio psicossocial e financeiro, poderiam ser úteis para melhor lidar com esse estado de doença crônica. Abstract in english CONTEXT AND OBJECTIVE Patients with beta-thalassemia major (?-TM) experience physical, psychological and social problems that lead to decreased quality of life (QoL). The aim here was to measure health-related QoL and its determinants among patients with ?-TM, using the Short Form-36 (SF-36) questi [...] onnaire. DESIGN AND SETTING Cross-sectional study at the Hematology Research Center of Shiraz University of Medical Sciences, in southern Iran. METHODS One hundred and one patients with ?-TM were randomly selected. After the participants' demographics and disease characteristics had been recorded, they were asked to fill out the SF-36 questionnaire. The correlations of clinical and demographic factors with the QoL score were evaluated. RESULTS There were 44 men and 57 women of mean age 19.52 ± 4.3 years (range 12-38). On two scales, pain (P = 0.041) and emotional role (P = 0.009), the women showed significantly lower scores than the men. Lower income, poor compliance with iron-chelating therapy and presence of comorbidities were significantly correlated with lower SF-36 scores. These factors were also found to be determinants of worse SF-36 scores in multivariate analysis. CONCLUSIONS We showed that the presence of disease complications, poor compliance with iron-chelating therapy and poor economic status were predictors of worse QoL among patients with ?-TM. Prevention and proper management of disease-related complications, increased knowledge among patients regarding the importance of managing comorbidities and greater compliance with iron-chelating therapy, along with psychosocial and financial support, could help these patients to cope better with this chronic disease state.

Sezaneh, Haghpanah; Shiva, Nasirabadi; Fariborz, Ghaffarpasand; Rahmatollah, Karami; Mojtaba, Mahmoodi; Shirin, Parand; Mehran, Karimi.

99

A study of membrane protein defects and alpha hemoglobin chains of red blood cells in human beta thalassemia  

Energy Technology Data Exchange (ETDEWEB)

The soluble pool of alpha hemoglobin chains present in blood or bone marrow cells was measured with a new affinity method using a specific probe, beta A hemoglobin chain labeled with ({sup 3}H)N-ethylmaleimide. This pool of soluble alpha chains was 0.067 {plus minus} 0.017% of hemoglobin in blood of normal adult, 0.11 {plus minus} 0.03% in heterozygous beta thalassemia and ranged from 0.26 to 1.30% in homozygous beta thalassemia intermedia. This elevated pool of soluble alpha chains observed in human beta thalassemia intermedia decreased 33-fold from a value of 10% of total hemoglobin in bone marrow cells to 0.3% in the most dense red blood cells. The amount of insoluble alpha chains was measured by using the polyacrylamide gel electrophoresis in urea and Triton X-100. In beta thalassemia intermedia the amount of insoluble alpha chains was correlated with the decreased spectrin content of red cell membrane and was associated with a decrease in ankyrin and with other abnormalities of the electrophoretic pattern of membrane proteins. The loss and topology of the reactive thiol groups of membrane proteins was determined by using ({sup 3}H)N-ethylmaleimide added to membrane ghosts prior to urea and Triton X-100 electrophoresis. Spectrin and ankyrin were the major proteins with the most important decrease of thiol groups.

Rouyer-Fessard, P.; Garel, M.C.; Domenget, C.; Guetarni, D.; Bachir, D.; Colonna, P.; Beuzard, Y. (Institut National de la Sante et de la Recherche Medicale, Creteil (France))

1989-11-15

100

A study of membrane protein defects and alpha hemoglobin chains of red blood cells in human beta thalassemia  

International Nuclear Information System (INIS)

The soluble pool of alpha hemoglobin chains present in blood or bone marrow cells was measured with a new affinity method using a specific probe, beta A hemoglobin chain labeled with [3H]N-ethylmaleimide. This pool of soluble alpha chains was 0.067 ± 0.017% of hemoglobin in blood of normal adult, 0.11 ± 0.03% in heterozygous beta thalassemia and ranged from 0.26 to 1.30% in homozygous beta thalassemia intermedia. This elevated pool of soluble alpha chains observed in human beta thalassemia intermedia decreased 33-fold from a value of 10% of total hemoglobin in bone marrow cells to 0.3% in the most dense red blood cells. The amount of insoluble alpha chains was measured by using the polyacrylamide gel electrophoresis in urea and Triton X-100. In beta thalassemia intermedia the amount of insoluble alpha chains was correlated with the decreased spectrin content of red cell membrane and was associated with a decrease in ankyrin and with other abnormalities of the electrophoretic pattern of membrane proteins. The loss and topology of the reactive thiol groups of membrane proteins was determined by using [3H]N-ethylmaleimide added to membrane ghosts prior to urea and Triton X-100 electrophoresis. Spectrin and ankyrin were the major proteins with the most important decrease of thiol groups

101

Molecular study and prenatal diagnosis of alpha- and beta-thalassemias in Chinese.  

Science.gov (United States)

Thalassemia is one of the most common single gene diseases worldwide. Populations in southern China and Taiwan have high prevalence rates of alpha- and beta-thalassemias. This review summarizes the current status of molecular studies, carrier screening, and prenatal diagnosis of thalassemia in Chinese. There are three genotypes of alpha-thalassemia 1 and at least six of alpha-thalassemia 2 in Chinese. For alpha-thalassemia 1, the South-East Asian deletion is the most common, followed by the Thai then Philippino deletions. For alpha-thalassemia 2, the rightward deletion is the most common, followed by the leftward deletion, and the nondeletional defects Hb Constant Spring and Hb Quong Sze. Twenty-eight different beta-thalassemia mutations have been reported. Four mutations, IVS-II-654 (C-->T), codons 41/42 frameshift (-TCTT), and nonsense codons 17 (A-->T) and -28 (A-->G), account for more than 90% of mutant alleles. For detection of alpha-thalassemia, polymerase chain reaction-related techniques are mainly used. Southern blot hybridization is still useful, especially for prenatal diagnosis. For detection of beta-thalassemia mutations, analysis of amplification-created restriction sites and reverse dot blot hybridization have been extensively used. In Taiwan, a national screening program incorporating hematological and molecular biological methods for thalassemia detection in pregnant women has been in progress for 5 years. Prenatal diagnosis has been performed in more than 1,800 pregnancies, including 1,500 cases at risk for homozygous alpha-thalassemia 1 and 300 for beta-thalassemia major, resulting in early prenatal diagnosis and termination of pregnancies affected with homozygous alpha-thalassemia 1 and an approximately 70% decrease in the number of newborns affected with beta-thalassemia major. In mainland China, only one large-scale screening program is in place. Characterization of undefined alleles, a higher awareness of the disease among physicians and the general public, and improvement of the service network will be important for early prenatal diagnosis and prevention of the disease in the future. PMID:9481058

Ko, T M; Xu, X

1998-01-01

102

Prevalence of hepatosplenomegaly in beta thalassemia minor subjects in Iran  

International Nuclear Information System (INIS)

Introduction: Thalassemia is the most common hereditary blood disorder in the world. Iran is located on the thalassemic belt and there is a high prevalence of the hepatosplenomegaly in beta thalassemia minor patients which is reported to be very variable. The goal of this research was to study the frequency of these signs in the cases with beta thalassemia minor patients in Iran. Materials and methods: Two hundred and fifty-nine cases that referred to center for pre-marriage tests were divided into two groups according to their MCV, MCH, and HbA2 (beta thalassemia minor cases and control groups). Liver and spleen sizes were determined by ultrasonographic method and the two groups were compared with each other. Results: Average spleen volumes in case and control groups were 163.48 ± 133.97 and 126.29 ± 53.98 mm3, respectively. Average spleen lengths in case and control groups were 10.71 ± 1.52 and 10.60 ± 5.4 cm, respectively. Conclusion: In the regions with high frequency of beta thalassemia, in case of finding large spleen size in the ultrasonography, a probable harmless differential diagnosis will be beta thalassemia minor that is not indicative of any serious disease. Volumetric measurement of spleen is more reliable for detection of splenomegaly in these patients

103

Prevalence of hepatosplenomegaly in beta thalassemia minor subjects in Iran  

Energy Technology Data Exchange (ETDEWEB)

Introduction: Thalassemia is the most common hereditary blood disorder in the world. Iran is located on the thalassemic belt and there is a high prevalence of the hepatosplenomegaly in beta thalassemia minor patients which is reported to be very variable. The goal of this research was to study the frequency of these signs in the cases with beta thalassemia minor patients in Iran. Materials and methods: Two hundred and fifty-nine cases that referred to center for pre-marriage tests were divided into two groups according to their MCV, MCH, and HbA2 (beta thalassemia minor cases and control groups). Liver and spleen sizes were determined by ultrasonographic method and the two groups were compared with each other. Results: Average spleen volumes in case and control groups were 163.48 {+-} 133.97 and 126.29 {+-} 53.98 mm{sup 3}, respectively. Average spleen lengths in case and control groups were 10.71 {+-} 1.52 and 10.60 {+-} 5.4 cm, respectively. Conclusion: In the regions with high frequency of beta thalassemia, in case of finding large spleen size in the ultrasonography, a probable harmless differential diagnosis will be beta thalassemia minor that is not indicative of any serious disease. Volumetric measurement of spleen is more reliable for detection of splenomegaly in these patients.

Karimi, Mehran [Hemostasis and Thrombosis Unit, Hematology Research Center, school of Medicine, Shiraz University of medical sciences, Shiraz (Iran, Islamic Republic of)], E-mail: Karimim@sums.ac.ir; Bagheri, Mohammad Hadi [Department of Radiology, School of Medicine, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of); Tahmtan, Mehdi [Hemostasis and Thrombosis Unit, Hematology Research Center, school of Medicine, Shiraz University of medical sciences, Shiraz (Iran, Islamic Republic of); Shakibafard, Alireza [Department of Radiology, School of Medicine, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of); Rashid, Murtaza [Hemostasis and Thrombosis Unit, Hematology Research Center, school of Medicine, Shiraz University of medical sciences, Shiraz (Iran, Islamic Republic of)

2009-01-15

104

The effects of beta-carotene and vitamin E on erythrocytes lipid peroxidation in beta-thalassemia patients  

Directory of Open Access Journals (Sweden)

Full Text Available BACKGROUND: Thalassemia is the most common hereditary disease in the world. Thalassemic erythrocytes are exposed to higher oxidative stress and lipid peroxidation. The aim of this study was to investigate the effects of beta-carotene and vitamin E on erythrocytes lipid peroxidation in beta-thalassemia patients.
METHODS: A prospective double-blind, placebo-controlled study of the effect of beta-carotene and vitamin E on lipid peroxidation in erythrocytes membranes was performed on 120 beta-thalassemia major patients in four groups. The patients were supplemented for 4 weeks as follows: group 1 with beta-carotene (13 mg/day, group 2 with vitamin E (550 mg/day, group 3 with beta-carotene plus vitamin E and group 4 with placebo. We prepared all capsules for 4 roups in the same shape and color. Measurements of serum beta-carotene and vitamin E were performed by high performance
liquid chromatography. After preparation of ghost cells from blood specimens, malondialdehyde (MDA was determined as index of lipid peroxidation in erythrocytes membranes before and after treatment. RESULTS: The levels of serum beta-carotene and vitamin E were significantly lower and MDA concentrations in erythrocytes membranes were significantly higher in beta-thalassemia patients compared to controls (P<0.001. In groups that treated with vitamin supplements for 4-weeks, lipid peroxidation rates were significantly reduced after treatment (P<0.001, but in placebo group there was not significant difference (P>0.05.
CONCLUSIONS: Our findings provide evidence that an oral treatment with beta-carotene and vitamin E can significantly reduce lipid peroxidation of erythrocytes membranes and could be useful in management of beta-thalassemia major patients. KEYWORDS: Beta-thalassemia major, beta-carotene, vitamin E, malondialdehyde, lipid peroxidation.

Soleiman Mahjoub

2007-12-01

105

Hypercoagulability in sickle cell disease and beta-thalassemia.  

Science.gov (United States)

Sickle cell disease (SCD) and beta-thalassemia (also referred to as beta-thalassemia) are common hereditary hemoglobinopathies with differing pathophysiologies and clinical courses. However, patients with both diseases exhibit increased platelet and coagulation activation, as well as decreased levels of natural anticoagulant proteins. In addition, they are characterized by thrombotic complications that may share a similar pathogenesis. The pathogenesis of hypercoagulability is likely multifactorial, with contributions from the abnormal red blood cell (RBC) phospholipid membrane asymmetry, ischemia-reperfusion injury, and chronic hemolysis with resultant nitric oxide depletion. More studies are needed to better define the contribution of hemostatic activation to the pathophysiology of SCD and beta-thalassemia. Furthermore, adequately controlled studies using anticoagulants and antiplatelet agents are warranted to define the role of hypercoagulability in specific complications of these diseases. PMID:18991650

Singer, Sylvia T; Ataga, Kenneth I

2008-11-01

106

Beta-thalassemias: expression, molecular mechanisms and mutations in Indians.  

Science.gov (United States)

The beta-thalassemias are a heterogenous group of inherited disorders of hemoglobin (Hb) synthesis characterized by a reduction (beta+) or absence (beta zero) of synthesis of the beta globin chains of Hb, resulting in an imbalanced chain synthesis. To understand their expression and molecular basis in Indians, it is essential to review briefly the genetic control of normal Hb production and the structure, organization and regulation of different globin genes. The Indian beta-thalassemia mutations and strategies for prevention are described. PMID:10773945

Colah, R; Mohanty, D

1998-01-01

107

A genetic score for the prediction of beta-thalassemia severity.  

Science.gov (United States)

Clinical and hematologic characteristics of beta(?)-thalassemia are determined by several factors resulting in a wide spectrum of severity. Phenotype modulators are: HBB mutations, HBA defects and fetal hemoglobin production modulators (HBG2:g.-158C>T polymorphism, HBS1L-MYB intergenic region and the BCL11A). We characterized 54 genetic variants at these five loci robustly associated with the amelioration of beta-thalassemia phenotype, to build a predictive score of severity using a representative cohort of 890 ?-thalassemic patients. Using Cox proportional hazard analysis on a training set, we assessed the effect of these loci on the age at which patient started regular transfusions, built a Thalassemia Severity Score, and validated it on a testing set. Discriminatory power of the model was high (C-index=0.705; R(2)=0.343) and the validation conducted on the testing set confirmed its predictive accuracy with transfusion-free survival probability (P<0.001) and with transfusion dependency status (Area Under the Receiver Operating Characteristic Curve=0.774; P<0.001). Finally, an automatized on-line calculation of the score was made available at http://tss.unica.it. Besides the accurate assessment of genetic predictors effect, the present results could be helpful in the management of patients, both as a predictive score for screening and a standardized scale of severity to overcome the major-intermedia dichotomy and support clinical decisions. PMID:25480500

Danjou, Fabrice; Francavilla, Marcella; Anni, Franco; Satta, Stefania; Demartis, Franca-Rosa; Perseu, Lucia; Manca, Matteo; Sollaino, Maria Carla; Manunza, Laura; Mereu, Elisabetta; Marceddu, Giuseppe; Pissard, Serge; Joly, Philippe; Thuret, Isabelle; Origa, Raffaella; Borg, Joseph; Forni, Gian Luca; Piga, Antonio; Lai, Maria Eliana; Badens, Catherine; Moi, Paolo; Galanello, Renzo

2015-04-01

108

Spinal cord compression due to extramedullary hematopoiesis in beta-thalassemia intermedia  

International Nuclear Information System (INIS)

Background: Extramedullary hematopoiesis (EMH) occurs in many disorders, including thalassemias and other hemoglobinopathies, and commonly presents in the spleen and liver. We present a case of spinal cord compression in a patient with beta-thalassemia intermedia, and review the literature and available treatment options. Patient and Methods: A 35-year-old black female with beta-thalassemia intermedia presented with a 3-week history of back pain and lower extremity weakness. Neurologic examination was consistent with spinal cord compression, and gadolinium enhanced magnetic resonance imaging (MRI) confirmed this diagnosis. She was given intravenous steroids and radiotherapy was begun in 200 cGy fractions to a total dose of 2000 cGy. Results: At the completion of radiotherapy the patient was ambulatory with mild residual weakness. MRI scans 16 months later showed smaller, but persistent masses, and she remains asymptomatic 5 years from her diagnosis. Conclusion: Recognition of spinal cord EMH requires prompt physical examination and MRI for accurate diagnosis. EMH can be managed with radiation, surgery, transfusions, or a combination of these therapies. Radiation in conservative doses of (750-3500 cGy) is non-invasive, avoids the surgical risks of potentially severe hemorrhage and incomplete resection, and has a high complete remission rate in the majority of patients. Relapse rates are moderate (37.5%), but retreatment provides excellent chance for second remissions excellent chance for second remission

109

Extraosseous radiotracer uptake on bone scan in beta-thalassemia: report of one case  

International Nuclear Information System (INIS)

Red blood cell transfusion, main therapeutic modality of beta-thalassemia, leads to iron overload which may perturb several metabolic ways. The aim of this paper is to illustrate the uptake abnormalities observed on bone scan of thalassaemic patients and to discuss mechanisms of extraosseous accumulation of the radiopharmaceutical in this pathology. We report a 16-year-old child suffering from beta-thalassemia major undergoing transfusion therapy. A bone scan was indicated to look for osseous infection. This study revealed a little skeletal uptake and abnormal liver, splenic and renal accumulation. A repeat bone scan, performed three weeks later showed a better skeletal uptake which enabled the discovery of focal abnormalities and made the diagnostic easier. The effect of iron overload on radiopharmaceuticals uptake in bone scan is known since 1975. Dissociation of 99mTc from the carrier ligand due to the presence of iron excess seems the most plausible hypothesis. Free 99mTc can be bound to other tissular substrates which can explain extraosseous uptake. The normally available pool for bone is reduced and then the skeletal uptake decreased. This report limits considerably the sensitivity of the bone scan. A well-led iron chelation and eventually the use of diuretic drug may guarantee a better quality of bone scan images. (authors)

110

Pharmacological induction of fetal hemoglobin in sickle cell disease and beta-thalassemia.  

Science.gov (United States)

A number of pharmacological agents are currently available for the induction of fetal hemoglobin (HbF) in patients with sickle cell disease and beta-thalassemia. Here we review the development of this new class of therapeutics and summarize the clinical trials that investigate their efficacy in patients with hemoglobin disorders. Hydroxyurea is the first of these drugs to be approved by the Food and Drug Administration for the treatment of sickle cell disease. Currently, the major focus is the development of safer agents and combinations of drugs that can increase HbF to levels high enough to prevent all complications of the disease. Progress in adapting the same strategy to the treatment of thalassemic disorders has been much slower. Although all the agents that are effective in sickle cell disease have similar HbF-inducing activity in beta-thalassemia, their use has rarely resulted in significant amelioration of the anemia. More research and more effective agents will be needed to make a significant impact on thalassemia. Nonetheless, success in this relatively young field has been very gratifying; before the end of this decade, clinically meaningful induction of HbF may become an achievable goal in most patients with hemoglobin disorders. PMID:11605172

Atweh, G F; Loukopoulos, D

2001-10-01

111

The liver in patients with [beta]-thalassemia major. Determination of iron concentration with Magnetic Resonance Imaging. Il fegato dei pazienti affetti da [beta]-talassemia major. Determinazione della concentrazione di ferro con Risonanza Magnetica  

Energy Technology Data Exchange (ETDEWEB)

The paper investigates the clinical usefulness of liver MRI in a large multicenter study because the control of iron concentration (LIC) is crucial in thalassemia major (TM) prognosis and conflicting results have been reported in small and heterogeneous groups of patients using MRI, the only very common non-invasive procedure. One hundred and eight consecutive TM patients, selected according to a specific protocol, were included in the study. This study shows that LIC can be calculated as a function of SIR. A validation study is necessary before introducing this prediction rule in clinical practice.

Midiri, M. (Bari, Policlinico Univ. (Italy). Ist. di Radiologia); Gallo, C.; Finazzo, M. (Palermo, Policlinico Univ. ' P. Giaccone' (Italy). Ist. di Radiologia ' Pietro Cignolini' )

112

Investigation of RBC Indices and HbA2 Levels in Parents of Beta-Thalassemia Patients: Impacts on Premarital Genetic Counseling  

Directory of Open Access Journals (Sweden)

Full Text Available Objective: This study was designed to investigate RBC indices and HbA2 levels in parents of major beta-thalassemia patients to detect possible silent beta- thalassemia carriers and examine its potential impact on the premarital genetic counseling.Materials and Methods: This cross sectional study was performed at Children Medical Center from 2004 to 2006. After genetic counseling and getting informed consent, peripheral blood sampling was carried out on 185 carrier parents of regularly blood transfused thalassemia children. Then RBC indices and HbA2 concentration were measured. Samples with MCV and MCH higher than and/or HbA2 lower than cut off values were rechecked. Results: In one case, MCV and MCH indices were within the limits defined for non beta- thalassemia carriers. Furthermore, four other cases were found to have decreased values of MCV and MCH but normal HbA2 levels."nConclusion: About 3% of beta-thalassemia carriers in our country may potentially be missed using current screening methods. Further studies are required to assess the need for presenting a new threshold for thalassemia carrier screening. Defining the causative mutations using molecular methods would pave the way for establishing a protocol for a premarital screening program in conditions when one of couples is a confirmed carrier.

Mina Izadyar

2007-06-01

113

ATA box transcription mutation in beta-thalassemia.  

OpenAIRE

DNA sequence analysis of a cloned beta-globin gene from a Chinese patient with beta-thalassemia revealed a single nucleotide substitution (A leads to G) within the ATA box homology and 28 base pairs upstream from the cap site. The patient was homozygous for this particular allele based on restriction mapping at nine different polymorphic sites in the beta-globin gene cluster. Upon transient expression in HeLa cells this gene directed the production of 3-5-fold less beta-globin mRNA than the n...

Orkin, S. H.; Sexton, J. P.; Cheng, T. C.; Goff, S. C.; Giardina, P. J.; Lee, J. I.; Kazazian, H. H.

1983-01-01

114

X-ray scattering signatures of {beta}-thalassemia  

Energy Technology Data Exchange (ETDEWEB)

X-ray scattering from lyophilized proteins or protein-rich samples is characterized by the presence of two characteristic broad peaks at scattering angles equivalent to momentum transfer values of 0.27 and 0.6 nm{sup -1}, respectively. These peaks arise from the interference of coherently scattered photons. Once the conformation of a protein is changed, these two peaks reflect such change with considerable sensitivity. The present work examines the possibility of characterizing the most common cause of hemolytic anaemia in Egypt and many Mediterranean countries; {beta}-thalassemia, from its X-ray scattering profile. This disease emerges from a genetic defect causing reduced rate in the synthesis of one of the globin chains that make up hemoglobin. As a result, structurally abnormal hemoglobin molecules are formed. In order to detect such molecular disorder, hemoglobin samples of {beta}-thalassemia patients are collected, lyophilized and measured using a conventional X-ray diffractometer. Results show significant differences in the X-ray scattering profiles of most of the diseased samples compared to control. The shape of the first scattering peak at 0.27 nm{sup -1}, in addition to the relative intensity of the first to the second scattering peaks, provides the most reliable signs of abnormality in diseased samples. The results are interpreted and confirmed with the aid of Fourier Transform Infrared (FTIR) spectroscopy of normal and thalassemia samples.

Desouky, Omar S. [Radiation Physics Department, National Center for Radiation Research and Technology (NCRRT) (Egypt); Elshemey, Wael M. [Biophysics Department, Faculty of Science, Cairo University (Egypt)], E-mail: waelelshemey@yahoo.com; Selim, Nabila S. [Radiation Physics Department, National Center for Radiation Research and Technology (NCRRT) (Egypt)

2009-08-11

115

Distribution of Hepatitis C Virus Genotypes Amongst the Beta-thalassemia Patients in North of Iran  

OpenAIRE

Beta-thalassemia patients have high prevalence for HCV infection. In developing countries, HCV antibody is reported to be high in this group of patients. This study carried out to determine the distribution of HCV genotypes amongst the beta-thalassemia patients in North of Iran. The present study has been carried out between February and March 2010 amongst a group of 245 beta-thalassemia patients (125 male and 120 female) referred to the hospitals Mazandaran and Guilan provinces for a blood t...

Maryam Farahani; Kivan Saeb; Hamid Reza Nejad; Mina Eghbali; Masood Ghane

2012-01-01

116

GENETIC AND ELECTROPHORETIC STUDY OF TWO FAMILIES WITH BETA-THALASSEMIA  

Directory of Open Access Journals (Sweden)

Full Text Available 19 cases of beta-thalassemia (2 homozygotes and 17 heterozygotes were finding out in a large family in the district of Vaslui by biochemical and genetical metods. For a better estimation of the status of two homozygote patients as well as for the selection of suitable treatment procedures, radiological and hematological investigations were performed. The usefulness of the application of electrophoretic techniques in the finding of beta-thalassemia heterozygotes is pointed out and also is underlined the necessity to undertake studies of selected groups of human population in order to obtain a better estimation of beta-thalassemias frequency in south-west Romania.

I.Capalnasan

1999-01-01

117

Kidney Function Tests in Children with Beta-Thalassemia Minor in Zahedan, Southeast of Iran  

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Full Text Available There is little information regarding kidney function in patients with beta-thalassemia minor. In this study we investigated kidney function tests in 50 children with beta-thalassemia minor (22 boys and 28 girls. Twenty-four-hour urine samples were collected and analyzed for sodium, potassium, calcium, magnesium, creatinine, phosphate, uric acid, protein, and beta2-microglobulin. Blood samples were obtained for hematologic and biochemical analyses including complete blood count, serum ferritin, sodium, potassium, calcium, phosphate, magnesium, creatinine, and uric acid. This group of children with beta-thalassemia showed some evidence of tubulopathy such as proteinuria (32%, beta2-microglobulin excretion (36%, calciuria (4%, phosphaturia (4%, and uricosuria (20%. Our findings support the existence of renal tubular dysfunction in beta-thalassemia minor. However, further studies in large series are needed to shed light on the possible relation of these two distinct diseases.

Simin Sadeghi-Bojd

2011-04-01

118

Kidney function tests in children with beta-thalassemia minor in Zahedan, southeast of Iran.  

Science.gov (United States)

There is little information regarding kidney function in patients with beta-thalassemia minor. In this study we investigated kidney function tests in 50 children with beta-thalassemia minor (22 boys and 28 girls). Twenty-four-hour urine samples were collected and analyzed for sodium, potassium, calcium, magnesium, creatinine, phosphate, uric acid, protein, and beta2-microglobulin. Blood samples were obtained for hematologic and biochemical analyses including complete blood count, serum ferritin, sodium, potassium, calcium, phosphate, magnesium, creatinine, and uric acid. This group of children with beta-thalassemia showed some evidence of tubulopathy such as proteinuria (32%), beta2-microglobulin excretion (36%), calciuria (4%), phosphaturia (4%), and uricosuria (20%). Our findings support the existence of renal tubular dysfunction in beta-thalassemia minor. However, further studies in large series are needed to shed light on the possible relation of these two distinct diseases. PMID:21525581

Sadeghi-Bojd, Simin; Hashemi, Mohammad; Naderi, Majid; Shikhani, Shahriar

2011-07-01

119

Kidney Function Tests in Children with Beta-Thalassemia Minor in Zahedan, Southeast of Iran  

OpenAIRE

There is little information regarding kidney function in patients with beta-thalassemia minor. In this study we investigated kidney function tests in 50 children with beta-thalassemia minor (22 boys and 28 girls). Twenty-four-hour urine samples were collected and analyzed for sodium, potassium, calcium, magnesium, creatinine, phosphate, uric acid, protein, and beta2-microglobulin. Blood samples were obtained for hematologic and biochemical analyses including complete blood count, serum ferrit...

Simin Sadeghi-Bojd; Mohammad Hashemi; Majid Naderi; Shahriar Shikhani

2011-01-01

120

A new TATA box mutation detected at prenatal diagnosis for beta-thalassemia.  

OpenAIRE

During the course of prenatal diagnosis for beta-thalassemia in Chinese patients, we encountered a mutation that was not detectable by oligonucleotides for the known Chinese mutations. Amplification of the beta-globin gene by the polymerase chain reaction and direct DNA sequencing revealed a previously undescribed -30 TATA box mutation which was carried by the father. Prenatal diagnosis was achieved, and the fetus did not inherit this beta-thalassemia allele.

Cai, S. P.; Zhang, J. Z.; Doherty, M.; Kan, Y. W.

1989-01-01

121

Early detection of right ventricular diastolic dysfunction by pulsed tissue Doppler echocardiography in iron loaded Beta thalassemia patients.  

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Early heart iron overload in beta thalassemia major patients can be quantified through T2* cardiovascular magnetic resonance (CMR). To clarify the value of tissue Doppler imaging (TDI) in early detection of myocardial dysfunction in iron loaded thalassemia patients diagnosed by CMR. Two groups were included in the study; Group I: 69 asymptomatic thalassemia patients (28 females, 41 males), mean age 18.1 ± 7.03 years (range 6-39 years); Group II (n = 41) healthy normal controls matched for age and sex. Serum ferritin and CMR were performed to assess the cardiac siderosis (T2*  20 ms. Conventional and Doppler echocardiography of LV, RV dimensions and functions and pulmonary artery pressure were evaluated. Right ventricular diastolic function assessed by tricuspid annular E'/A' was positively correlated with T2* value; lower tricuspid E'/A' ratios were correlated with lower T2* values (r = 0.366, P = 0.002). Tricuspid annular A' was significantly higher in group Ia compared to group Ib (16.7 ± 5.2 vs 12.1 ± 4.0 cm/s, P thalassemia patients. TDI is a promising tool for quantitative assessment of myocardial function and early detection of right ventricular diastolic dysfunction in iron loaded beta thalassemia major patients. PMID:25293426

Agha, Hala Mounir; Beshlawy, Amal; Hamdy, Mona; Sobeih, Alae; Zahrae, Fatma El; Satar, Inas Abd El; AbdelMassih, Antoine; Said, Fadwa; Aziz, Ossama Abd El; Tagui, Mona El; Pennell, Dudley J

2015-03-01

122

Marcadores eletrocardiográficos para detecção precoce de doença cardíaca em pacientes com talassemia beta maior Electrocardiographic markers for the early detection of cardiac disease in patients with beta-thalassemia major  

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Full Text Available OBJETIVO: Analisar comparativamente a dispersão da onda P (DOP em pacientes com talassemia beta maior (?-TM e indivíduos saudáveis (controles para a detecção precoce do risco de arritmias. MÉTODOS: Oitenta e uma crianças com ?-TM, com idades entre 4 e 19 anos, e 74 crianças saudáveis (grupo controle foram submetidas a exame eletrocardiográfico e ecocardiograma transtorácico de rotina para avaliação cardíaca. A DOP foi calculada como a diferença entre as durações máxima e mínima da onda P. RESULTADOS: Houve uma diferença estatisticamente significativa entre o grupo de estudo e o grupo controle no pico de velocidade do fluxo transmitral no início da diástole (E e na razão E/fluxo transmitral tardio (A. A duração máxima da onda P e a DOP foram significativamente maiores nos pacientes com ?-TM do que nos indivíduos controles. CONCLUSÕES: O aumento da DOP em nossos pacientes com ?-TM pode estar relacionado à depressão na condução intra-atrial, devido à dilatação atrial, e ao aumento da atividade simpática. Estes pacientes devem ser acompanhados atentamente devido à possibilidade de ocorrência de arritmias com risco de vida.OBJECTIVE: To comparatively evaluate P-wave dispersion (PWD in patients with ?-thalassemia major (TM and healthy control subjects for the early prediction of arrhythmia risk. METHODS: Eighty-one children with ?-TM, aged 4-19 years, and 74 healthy children (control group underwent routine electrocardiography and transthoracic echocardiography for cardiac evaluation. PWD was calculated as the difference between the maximum and the minimum P-wave duration. RESULTS: There was a statistically significant difference between study and control groups in peak early (E mitral inflow velocity and E/late (A velocity ratio. Maximum P-wave duration and PWD were found to be significantly higher in ?-TM patients than in control subjects. CONCLUSIONS: Increased PWD in our ?-TM patients might be related to depression of intra-atrial conduction due to atrial dilatation and increased sympathetic activity. These patients should be closely followed up for risk of life-threatening arrhythmias.

Kemal Nisli

2010-04-01

123

Marcadores eletrocardiográficos para detecção precoce de doença cardíaca em pacientes com talassemia beta maior / Electrocardiographic markers for the early detection of cardiac disease in patients with beta-thalassemia major  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Languages: English, Portuguese Abstract in portuguese OBJETIVO: Analisar comparativamente a dispersão da onda P (DOP) em pacientes com talassemia beta maior (?-TM) e indivíduos saudáveis (controles) para a detecção precoce do risco de arritmias. MÉTODOS: Oitenta e uma crianças com ?-TM, com idades entre 4 e 19 anos, e 74 crianças saudáveis (grupo contr [...] ole) foram submetidas a exame eletrocardiográfico e ecocardiograma transtorácico de rotina para avaliação cardíaca. A DOP foi calculada como a diferença entre as durações máxima e mínima da onda P. RESULTADOS: Houve uma diferença estatisticamente significativa entre o grupo de estudo e o grupo controle no pico de velocidade do fluxo transmitral no início da diástole (E) e na razão E/fluxo transmitral tardio (A). A duração máxima da onda P e a DOP foram significativamente maiores nos pacientes com ?-TM do que nos indivíduos controles. CONCLUSÕES: O aumento da DOP em nossos pacientes com ?-TM pode estar relacionado à depressão na condução intra-atrial, devido à dilatação atrial, e ao aumento da atividade simpática. Estes pacientes devem ser acompanhados atentamente devido à possibilidade de ocorrência de arritmias com risco de vida. Abstract in english OBJECTIVE: To comparatively evaluate P-wave dispersion (PWD) in patients with ?-thalassemia major (TM) and healthy control subjects for the early prediction of arrhythmia risk. METHODS: Eighty-one children with ?-TM, aged 4-19 years, and 74 healthy children (control group) underwent routine electroc [...] ardiography and transthoracic echocardiography for cardiac evaluation. PWD was calculated as the difference between the maximum and the minimum P-wave duration. RESULTS: There was a statistically significant difference between study and control groups in peak early (E) mitral inflow velocity and E/late (A) velocity ratio. Maximum P-wave duration and PWD were found to be significantly higher in ?-TM patients than in control subjects. CONCLUSIONS: Increased PWD in our ?-TM patients might be related to depression of intra-atrial conduction due to atrial dilatation and increased sympathetic activity. These patients should be closely followed up for risk of life-threatening arrhythmias.

Kemal, Nisli; Yavuz, Taner; Oner, Naci; Salcioglu, Zafer; Karakas, Zeynep; Dindar, Aygun; Umrah, Aydogan; Rukiye, Eker; Turkan, Ertugrul.

2010-04-01

124

Unpredictability of intravenous busulfan pharmacokinetics in children undergoing hematopoietic stem cell transplantation for advanced beta thalassemia: limited toxicity with a dose-adjustment policy.  

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beta-thalassemia is a major health problem worldwide, and stem cell transplantation (SCT) is the only curative option. Oral Busulfan (Bu) based conditioning is widely used in this setting. Due to the variability of Bu systemic exposure, intravenous (i.v.) Bu has been proposed as a standard of care, with no need for drug monitoring and dose adjustment. Patients with beta-thalassemia from countries with limited resources might be at higher risk of erratic Bu metabolism because of liver dysfunction, severe iron overload, and specific ethnic/genetic features. We studied Bu pharmacokinetics in 53 children with advanced beta-thalassemia from Middle Eastern countries who underwent a total of 57 matched related donor SCTs. Forty-two percent of the children required dose adjustment because they did not achieve the therapeutic window after the first dose. With a Bu dose-adjustment policy, regimen-related toxicity was limited. At a median follow-up of 564 days, the probabilities of 2-year survival, current thalassemia-free survival, rejection, and treatment-related mortality were 96%, 88%, 21%, and 4%, respectively. Conditioning with i.v. Bu and dose adjustment is feasible and well tolerated, although recurrence of thalassemia remains an unsolved problem in children with advanced disease. PMID:19963071

Chiesa, Robert; Cappelli, Barbara; Crocchiolo, Roberto; Frugnoli, Ilaria; Biral, Erika; Noè, Anna; Evangelio, Costanza; Fossati, Marco; Roccia, Tito; Biffi, Alessandra; Finizio, Valentina; Aiuti, Alessandro; Broglia, Monica; Bartoli, Antonella; Ciceri, Fabio; Roncarolo, Maria Grazia; Marktel, Sarah

2010-05-01

125

Serum uric acid: an early indicator of oxidative stress in beta thalassemia population.  

Science.gov (United States)

Iron induced alteration in the redox balance is a major complication in the beta thalassemia major patients receiving regular blood transfusion. Variable degrees of association between uric acid level with the free iron and its storage form ferritin in thalassemic patients are suggested to play a significant role in this alteration by an unclear mechanism. In the present study, we made an effort to analyze the association of serum uric acid with oxidative stress and to evaluate the predictive value of serum iron, serum ferritin and serum uric acid level on oxidative stress induced lipid peroxidation in beta thalassemic patients. For this we assayed these parameters in 61 patients of ? thalassemia major and 51 age and sex matched controls of a rural area of West Bengal. Serum TBARS, serum Uric acid, serum Ferritin and serum Iron levels were significantly higher in the patient group (pUric acid, Ferritin and Iron levels in the patient group whereas only serum TBARS levels showed significant correlation with serum iron level in the control group. Regression analysis revealed that uric acid levels have a better predictive value (?=0.3, p=0.001) than serum ferritin value (?=0.013, p=0.871) for indicating TBARS production. PMID:23982550

Dasgupta, S; Dasgupta, A; Mukhopadhayay, T; Bhattacharya, S; Swaika, B; Banarjee, U; Chakrabarty, P

2013-07-01

126

Valores de ferritina sérica em beta talassemia heterozigota / Serum ferritin levels in beta thalassemia carrier  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: Portuguese Abstract in portuguese [...] Abstract in english A low iron level, the commonest nutritional deficiency in the world, is a public health problem in developing countries. On the other hand, an excessive amount of iron is toxic, causing several organic dysfunctions, such as diabetes, cirrhosis, endocrinopathies and heart disease. Researchers have re [...] ported an association of iron overload with beta-thalassemia. The aim of this paper was to compare the serum ferritin levels of women with the beta-thalassemia trait. The results of serologic tests of 137 women of childbearing age were analyzed; 63 had the beta-thalassemia trait and 74 had Hb AA. In the beta-thalassemia carriers, the median ferritin value was 51.90 ng/mL and in the non-carriers 31.60 ng/mL (p = 0.0052). Levels of less than 20 and above 150 ng/mL were observed in 28% and 3% of the non-carriers and in 16% and 11% of the carriers, respectively. With these results it is possible to conclude that women in the reproductive age with the beta-thalassemia trait present higher ferritin levels in the northeastern region of São Paulo State. Further studies are necessary to clarify possible genetic and/or environment factors which interfere in iron absorption.

Isabeth F., Estevão; Maria Cristina S., Souza; Antonio J., Manzato; Claudia R., Bonini-Domingos.

127

Distribution of beta-thalassemia mutations in the Indian population referred to a diagnostic center.  

Science.gov (United States)

Hemoglobinopathies are the most commonly inherited genetic disorders in India. Population screening has identified certain communities in India with high risk of beta-thalassemia, the prevalence of carrier status in some being as high as 17%. Over a period of 6 years we have conducted DNA analysis on 1,233 carriers of 23 beta-thalassemia mutations and Hb E, using the amplification refractory mutation system. The studies included analyses of five common mutations for Asian Indians, namely IVS-I-5 (G-->C), 619 bp deletion, IVS-I-1 (G-->T), and the frameshifts at codons 8/9 (+G) and 41/42 (-TTCT). The occurrence of these was seen in 1,066 (86.45%) of the carriers referred to us, the percentage of mutations varying between 5.03-42.58%. We found codon 15 (TGG-->TAG) in 47 (3.81%) samples which was also considered a common mutation in the Indian population. Rare beta-thalassemia mutations were found in 87 (7.06%) individuals. We have designed five new primers and modified four primers for nine rare mutations. These were seen in nine (0.73%) samples. The beta-thalassemia anomaly in 33 (2.68%) carriers remained uncharacterized. State-wide and community-wide distribution patterns of mutations indicated that IVS-I-5 (G-->C) is the most common beta-thalassemia allele in the Indian population. Sindhis settled in Gujrat, and Maharashtra and Lohanas from Gujrat showed a prevalence of the 619 bp deletion mutation in 49.2 and 45.5% carriers, respectively. High frequency of the IVS-I-1 (G-->T) mutation was also found in Sindhis (25.5%), Punjabi Hindus (34.7%), and Lohanas (31.2%). These studies of mutation patterns in different communities have helped us in the quick identification of beta-thalassemia mutations for prenatal diagnosis. PMID:10975438

Vaz, F E; Thakur, C B; Banerjee, M K; Gangal, S G

2000-08-01

128

Spinal cord compression in {beta}-thalassemia: follow-up after radiotherapy  

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Spinal cord compression due to extramedullary hematopoiesis is a well-described bu rare syndrome encountered in several hematologic disorders, including {beta}-thalassemia. We report a case of a patient with intermediate {beta}-thalassemia and crural paraparesis due to spinal cord compression by a paravertebral extramedullary mass. She was successfully treated with low-dose radiotherapy and transfusions. After splenectomy, she was regularly followed up for over four years without transfusion or recurrence of spinal cord compression. Extramedullary hematopoiesis should be investigated in patients with hematologic disorders and spinal cord symptoms. The rapid recognition and treatment with radiotherapy can dramatically alleviate symptoms. (author)

Fonseca, Silvana Fahel da; Figueiredo, Maria Stella; Cancado, Rodolfo Delfini; Nakadakare, Fernando; Segreto, Roberto; Kerbauy, Jose [Universidade Federal de Sao Paulo (UNIFESP), SP (Brazil). Escola Paulista de Medicina

1998-12-01

129

Beta globin gene cluster haplotypes of the beta thalassemia mutations observed in the Denizli province of Turkey  

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Full Text Available Objective: Our aim was to identify the beta globin gene cluster haplotypes for the beta thalassemia mutations in Turkey on a regional level. Beta thalassemia mutations included in this study were IVS-I-110 (G>A, FSC 8/9 (+G, IVS-II-1 (G>A, IVS-I-5 (G>C, IVS-I-1 (G>A, IVS-I-6 (T>C, and FSC 8 (-AA. Methods: We studied 22 unrelated patients with ?-thalassemia major and 72 unrelated healthy subjects from our Department’s DNA bank. Haplotype analysis was done by polymerase chain reaction (PCR-based restriction enzyme digestion for the beta globin gene cluster of the following polymorphic restriction sites: Hinc II 5’ to ?, Hind III 5’ to G?, Hind III in the IVS-II 5’ to A?, Hinc II in pseudo ?, Hinc II 3’ to pseudo ?, Ava II in ?, and Hinf I 3’ to ?. Associated haplotypes for the normal control samples (72 individuals, 144 chromosomes were determined by Arlequin 3.1 software with unknown gametic phase.Results: According to the results obtained, the most frequent beta globin gene cluster haplotypes in the normal population were (+----++, (+----+-, (-+-++++, and (+-----+, with frequencies of 28.6%, 17.2%, 9.8%, and 8.3%, respectively. IVS-I-110 mutation was linked with the haplotypes (+----++ and (+-----+. Observed haplotypes were (+----++ for FSC 8/9 (+G, (-+-+++- for IVS-II-1 (G>A, (-+-++-+ and -+-++++ for IVS-I-5 (G>C, (+----+- and +------ for IVS-I-1 (G>A, (-++---+ for IVS-I-6 (T>C, and (+-----+ for FSC 8 (-AA.Conclusion: Our region shows the Mediterranean character for the beta thalassemia mutations. According to the obtained results, IVS-I-110 (G>A mutation linked with haplotype VII (+-----+, IVS-I-5 (G>C mutation with haplotype IV (-+-++-+, and codon 8/9 (+G with haplotype I (+----++ were shown for the first time in the Turkish population. The linkage of haplotype (+------ with the IVS-I-1 (G>A mutation is reported for the first time in the published literature. In the Denizli province of Turkey, beta globin gene cluster haplotypes of the normal population are strongly associated with the haplotypes of I (+----++, V (+----+- and IX (-+-++++, respectively.

Anzel Bahad?r

2009-09-01

130

Coexistence of beta-thalassemia and hereditary hemochromatosis in homozygosity: a possible synergic effect?  

Science.gov (United States)

A few considerations, which we found in the literature, inspired us to reevaluate patients previously investigated [characterized for beta-thalassemia (beta-thal) and hereditary hemochromatosis (HH) genes] by our department at Medical Genetics, School of Medicine, University of Foggia, Italy. PMID:19373593

Bukvic, Nenad; Sportelli, Filomena; Sessa, Francesco; Longo, Vittoria; Roberti, Maria Grazia; Santacroce, Rosa; Margaglione, Maurizio

2009-01-01

131

Prevalence of Beta-Thalassemia in premarital screening in Al-Hassa, Saudi Arabia  

International Nuclear Information System (INIS)

The Al-Hassa area is one of the regions in Saudi Arabia where hemoglobinopathies are prevalent. The Saudi Ministry of Health designed a protocol for premarital testing after the royal decree in December 2003. The protocol was implemented in a February 2004 order. The aim of this study was to determine the prevalence of beta-thalassemia trait among subjects coming for premarital screening in the Al-Hassa area. From February 2004 to November 2004, healthy subjects coming to six marriages consultation centers in the Al-Hassa area underwent routine mandatory tests. Subjects were considered to have beta-thalassemia trait if they had a mean corpuscular volume (MCV), 80 fL and/or a mean corpuscular hemoglobin (MCH) 3.2%. Venous blood was taken into ETDA tube and the complete blood count and red blood cell indices were measured by a Coulter automated cell counter on the same day of hemoglobin collection. Electrophoresis was done on cellulose acetate. All Saudi participants (n=8918), including 4218 (47.3%) males and 4700 (52.7%) females were screened. The prevalence of beta-thalassemia trait with high hemoglobin A2 and microcytic hypochromic anemia was 3.4% (307/8918). In countries with a high prelevance of hemoglobinopathies, a premarital screening program is helpful for identification and prevention of high-risk marriages. With a 3.4% prevalence of beta-thalassemia trait in premarital couples, future comprehensive programs are needed to knre comprehensive programs are needed to know the actual prevalence of beta-thalassemia in Al-Hassa. (author)

132

Better differential diagnosis of iron deficiency anemia from beta-thalassemia trait  

OpenAIRE

Objective: Iron deficiency anemia (IDA) and beta-thalassemia trait (ß-TT) are the most common forms of microcytic anemia. This study was conducted to compare the validity of various discrimination indices in differentiating ?-TT from IDA by calculating their sensitivity, specificity and Youden's index.Methods: Totally 323 subjects (173 children and 150 adults) with microcytic anemia were involved in this study. We calculated 10 discrimination indices in all patients with IDA and ?-TT. We d...

Fakher Rahim; Bijan Keikhaei

2009-01-01

133

Beta-thalassemia, 2D animationSite: DNA Interactive (www.dnai.org)  

Science.gov (United States)

The HBB gene encodes an important blood protein called beta globin. A person with beta-thalassemia carries a mutation in both copies of the HBB gene, completely halting production of the beta globin protein. Without beta globin, the important oxygen-carrying protein, hemoglobin, can not be made. Although oxygen can be carried by a less efficient form of hemoglobin, most of the affected red blood cells die.

2008-10-06

134

What is beta-thalassemia?, 2D animationSite: DNA Interactive (www.dnai.org)  

Science.gov (United States)

The HBB gene encodes an important blood protein called beta globin. A person with beta-thalassemia carries a mutation in both copies of the HBB gene, completely halting production of the beta globin protein. Without beta globin, the important oxygen-carrying protein, hemoglobin, can not be made. Although oxygen can be carried by a less efficient form of hemoglobin, most of the affected red blood cells die.

2008-10-06

135

What causes beta-thalassemia?, 2D animationSite: DNA Interactive (www.dnai.org)  

Science.gov (United States)

The HBB gene encodes an important blood protein called beta globin. A person with beta-thalassemia carries a mutation in both copies of the HBB gene, completely halting production of the beta globin protein. Without beta globin, the important oxygen-carrying protein, hemoglobin, can not be made. Although oxygen can be carried by a less efficient form of hemoglobin, most of the affected red blood cells die.

2008-10-06

136

Hematological Indices for Differential Diagnosis of Beta Thalassemia Trait and Iron Deficiency Anemia  

OpenAIRE

Background. The two most frequent types of microcytic anemia are beta thalassemia trait (?-TT) and iron deficiency anemia (IDA). We retrospectively evaluated the reliability of various indices for differential diagnosis of microcytosis and ?-TT in the same patient groups. Methods. A total of 290 carefully selected children aged 1.1–16 years were evaluated. We calculated 12 discrimination indices in all patients with hemoglobin (Hb) values of 8.7–11.4?g/dL. None of the subjects had a ...

Aysel Vehapoglu; Gamze Ozgurhan; Egul Dogan Demir, Ay X. F.; Selcuk Uzuner; Mustafa Atilla Nursoy; Serdar Turkmen; Arzu Kacan

2014-01-01

137

Evolution of a genetic disease in an ethnic isolate:. beta. -Thalassemia in the Jews of Kurdistan  

Energy Technology Data Exchange (ETDEWEB)

{beta}-Thalassemia is a hereditary disease caused by any of 90 different point mutations in the {beta}-globin gene. Specific populations generally carry a small number of mutations, the most common of which are those that are widely distributed regionally. The present study constitutes an extensive molecular characterization of this disease in a small, highly inbred ethnic group with a high incidence of {beta}-thalassemia-the Jews of Kurdistan. An unusual mutational diversity was observed. In 42 sibships 13 different mutations were identified, of which 3 are newly discovered. Four of the mutations are unique to Kurdish Jews and have not been discovered in any other population. A fifth was found outside Kurdish Jews only in an Iranian from Khuzistan, a region bordering Kurdistan. Two-thirds of the mutant chromosomes carry the mutations unique to Kurdish Jews. The authors traced the origin of the mutations to specific geographic regions within Kurdistan. This information, supported by haplotype analysis, suggests that thalassemia in central Kurdistan (northern Iraq) has evolved primarily from multiple mutational events. They conclude that several evolutionary mechanisms contributed to the evolution of {beta}-thalassemia in this small ethnic isolate.

Rund, D.; Cohen, T.; Filon, D.; Rachmilewitz, E.; Oppenheim, A. (Hadassah Univ. Hospital, Jerusalem (Israel)); Dowling, C.E.; Warren T.C.; Kazazian, H.H. Jr. (Johns Hopkins Univ. School of Medicine, Baltimore, MD (United States)); Barak, I. (Kaplan Hospital, Rehovot (Israel))

1991-01-01

138

Measurement of reticulocyte and red blood cell indices in patients with iron deficiency anemia and beta-thalassemia minor.  

Science.gov (United States)

New parameters correlated with the hemoglobin content in reticulocytes (RET-Y) and in red blood cells (RBC-Y) have been suggested as helpful in diagnosing iron deficiency anemia. We have studied RET-Y and RBC-Y indices in two groups of patients with microcytosis to verify if these parameters could be used to differentiate iron deficiency anemia from beta-thalassemia minor. Blood samples from 33 iron-deficient patients, 25 beta-thalassemic minor patients and 50 normal individuals were analyzed on a Sysmex XE-2100 instrument. A significant difference was observed in reticulocyte counting and immature reticulocyte fraction between iron deficiency anemia and beta-thalassemia minor groups, but not in RBC-X and RET-Y parameters. Reticulocyte counting was higher in beta-thalassemia minor and the immature reticulocyte fraction was higher in severe iron deficiency anemia. The ratio RET-Y/mean cell volume was tested and was significantly different when beta-thalassemia minor was compared with mild and severe iron deficiency anemia, and showed better performance than the Mentzer ratio and the Green and King function. A great overlap of RET-Y and RBC-Y individual values was observed in both groups of microcytic anemias; we conclude that these new indices may be used with caution as indicative of iron deficiency, mainly in populations where beta-thalassemia minor is frequent. PMID:15843216

Noronha, José F A; Grotto, Helena Z W

2005-01-01

139

Colloid clearance rate changes in children with homozygous-. beta. -thalassemia in relation to blood transfusion  

Energy Technology Data Exchange (ETDEWEB)

The plasma clearance rate of heat denatured human serum albumin (DHAI-125, 5 mg/kg body weight) was studied in 20 children with homozygous-..beta..-thalassemia before and 7-10 days after blood transfusion. A significant increase of the DHAI-125 clearance rate (P < 0.02) was found 7-10 days after blood transfusion while the spleen presented its minimum size. This finding may be relevant to the improved intrasplenic blood circulation after blood transfusion due to the release of the blood trapped within the spleen.

Dimitriou, P.A.; Karpathios, T.E.; Antipas, S.E.; Fretzayias, A.M.; Kasfiki, A.G.; Melissinos, K.G.; Matsaniotis, N.S.

1980-10-01

140

Infusion of autologous retrodifferentiated stem cells into patients with beta-thalassemia.  

Science.gov (United States)

Beta-thalassemia is a genetic, red blood cell disorder affecting the beta-globin chain of the adult hemoglobin gene. This results in excess accumulation of unpaired alpha-chain gene products leading to reduced red blood cell life span and the development of severe anemia. Current treatment of this disease involves regular blood transfusion and adjunct chelation therapy to lower blood transfusion-induced iron overload. Fetal hemoglobin switching agents have been proposed to treat genetic blood disorders, such as sickle cell anemia and beta-thalassemia, in an effort to compensate for the dysfunctional form of the beta-globin chain in adult hemoglobin. The rationale behind this approach is to pair the excess normal alpha-globin chain with the alternative fetal gamma-chain to promote red blood cell survival and ameliorate the anemia. Reprogramming of differentiation in intact, mature, adult white blood cells in response to inclusion of monoclonal antibody CR3/43 has been described. This form of retrograde development has been termed "retrodifferentiation", with the ability to re-express a variety of stem cell markers in a heterogeneous population of white blood cells. This form of reprogramming, or reontogeny, to a more pluripotent stem cell state ought to recapitulate early hematopoiesis and facilitate expression of a fetal and/or adult program of hemoglobin synthesis or regeneration on infusion and subsequent redifferentiation. Herein, the outcome of infusion of autologous retrodifferentiated stem cells (RSC) into 21 patients with beta-thalassemia is described. Over 6 months, Infusion of 3-h autologous RSC subjected to hematopoietic-conducive conditions into patients with beta-thalassemia reduced mean blood transfusion requirement, increased mean fetal hemoglobin synthesis, and significantly lowered mean serum ferritin. This was always accompanied by an increase in mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), and mean corpuscular hemoglobin concentration (MCHC) in such patients. No adverse side effects in response to the infusion of autologous RSC were noted. This novel clinical procedure may profoundly modify the devastating course of many genetic disorders in an autologous setting, thus paving the way to harnessing pluripotency from differentiated cells to regenerate transiently an otherwise genetically degenerate tissue such as thalassemic blood. PMID:17041717

Abuljadayel, Ilham Saleh; Quereshi, Huma; Ahsan, Tasnim; Rizvi, Shakil; Ahmed, Tamseela; Khan, Sabiha Mirza; Akhtar, Jawaid; Dhoot, Ghazi

2006-01-01

141

Same. beta. -globin gene mutation is present on nine different. beta. -thalassemia chromosomes in a Sardinian population  

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The predominant ..beta..-thalassemia in Sardinia is the ..beta../sup 0/ type in which no ..beta..-globin chains are synthesized in the homozygous state. The authors determined the ..beta..-thalassemia mutations in this population by the oligonucleotide-probe method and defined the chromosome haplotypes on which the mutation resides. The same ..beta../sup 39(CAG..-->..TAG)/ nonsense mutation was found on nine different chromosome haplotypes. Although this mutation may have arisen more than once, the multiple haplotypes could also be generated by crossing over and gene conversion events. These findings underscore the frequency of mutational events in the ..beta..-globin gene region.

Pirastu, M.; Galanello, R.; Doherty, M.A.; Tuveri, T.; Cao, A.; Kan, Y.W.

1987-05-01

142

Medicinal chemistry of fetal hemoglobin inducers for treatment of beta-thalassemia.  

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In this review we summarize the achievements of medicinal chemistry in the field of pharmacological approaches to the therapy of beta-thalassemia using molecules able to stimulate the production of fetal hemoglobin (HbF). We first describe the molecular basis of the pathology and the biochemical rational of using HbF inducers for therapy; we then outlined the in vitro and in vivo experimental systems suitable for screening of such potential drugs, and finally we describe the different classes of compounds with emphasis on their advantages and disadvantages in the treatment. The results of these reviewed studies indicate that: (a) HbF inducers can be grouped in several classes based on their chemical structure and mechanism of action; (b) clinical trials with some of these inducers demonstrate that they are effective in ameliorating the symptoms of beta-thalassemia; (c) a good correlation was found between HbF stimulation in vivo and in vitro indicating that in vitro testing might be predictive of the in vivo response; (d) combined use of different inducers might maximize the effect, both in vitro and in vivo. However, (e) the response to HbF inducers, evaluated in vitro and in vivo, is variable, and some patients might be refractory to HbF induction by certain inducers; in addition, (f) several considerations call for caution, including the fact that most of the inducers exhibit in vitro cytotoxicity, predicting side effects in vivo following prolonged treatments. PMID:17266579

Gambari, Roberto; Fibach, Eitan

2007-01-01

143

Beta-thalassemia- institution based analysis of ethnic and geographic distribution, effect of consanguinity and safety of chorionic villus sampling as a diagnostic, tool for pre-natal diagnosis in selected patients  

International Nuclear Information System (INIS)

To study the ethnic and geographic distribution of Beta-thalassemia amongst the patients included and to study the effect of consanguinity in promoting this disease. Also, to establish the safety of CVS when used as a pre-natal diagnostic tool in aiding the early diagnosis of Beta-thalassemia in selected patients. Study Design: Descriptive Study. Place and Duration of Study: PNS Shifa Karachi, from Jan 2008 to Dec 2008. Patients and Methods: A total of 223 women out of 240 that were referred from all over Sindh to PNS Shifa Hospital Karachi for susceptible gene mutations participated in the study. The standard procedure that was used in this study was trans-abdominal aspiration of chorionic villi through suction needle. The samples were then sent for further analysis to the Pathology Department at PNS Shifa Hospital Karachi. Results: In our study population Beta-thalassemia was most prevalent in Sindhi 107 (48%) followed by Punjabi 46 (21%), 27 (12%) Pathan, and 43 (19%) Balochi. Out of 223 women, 95 were of thalassemia trait, while 85 were of thalassemia major. Fifty five percent of thalassemia trait and 56% of thalassemia major fetus parents were first cousins. The rate of pregnancy loss after performing CVS was 2.0% with no complications reported. Conclusion: It is concluded that highest percentage of thalassemia is in first cousins and sindhi origin families are mostly affected. However CVS is a safe and effective tool for prenatal diagnosis and subsequent counsel prenatal diagnosis and subsequent counselling in selected couples. (author)

144

Beta-thalassemia, hyperlipoproteinemia(a), and metabolic syndrome: its low-cost holistic therapy.  

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Metabolic syndrome (MS) is an emerging global health problem. Although studies highlighting its genetic, lipid, and cardiometabolic associations have been described in detail, the exact cause for these associations is not clear. The authors describe, in this study, the case of a patient who, along with his family members, had clinical evidence of MS. In addition, this patient also exhibited beta-thalassemia minor and hyperlipoproteinemia(a). Lipoprotein [Lp(a)] levels diminished significantly following therapy with bark-stem powder of Terminalia arjuna, an ancient remedy recommended for angina pectoris. The co-existence of these conditions, reflecting both a genetic link and a significant reduction in Lp(a) levels amounting to 24.71% following the administration of T. arjuna, prompted the authors to report on this case. PMID:17388772

Dwivedi, Shridhar; Kumar, Vivek

2007-03-01

145

Regional and ethnic distribution of beta thalassemia mutations and effect of consanguinity in patients referred for prenatal diagnosis  

International Nuclear Information System (INIS)

To determine the regional and ethnic distribution of beta thalassemia mutation and the effect of consanguinity in patients referred for prenatal diagnosis of beta b-thalassemia and to target the high risk population for screening. A total of 499 couples were referred to Gentec Lab., Lahore, from all over Pakistan for prenatal diagnosis of b-thalassemia. After counseling, chorionic villus sampling was done between 10-16 weeks of gestation. DNA analysis was done by Amplification Refractory Mutation System (ARMS) for type of mutation in the Armed Forces Institute of Pathology, Rawalpindi. Ethnicity, race and consanguineous relationship of parents was determined.b-thalassemia was prevalent in Punjabis (60.7%) followed by Saraikees (25.5%). Castewise it was most frequent in Rajputs followed by Jatts, Arain, Sheikhs and Pathans. 56.7% of the couples were first cousins and 19.8% were relatives. The commonest mutations were Frameshift 8-9 (Fr8-9) 33.5%, Intervening Sequence 1-5 (IVS 1-5) 17.2%, Fr4142 - 8%, IVS 1-1 - 5.2%, Deletion 619 (Del 619) 4.2% and Codon 5 (Cd 5) - 4.2%. In samples sent for analysis, 53.1% turned out to be carriers (trait), 25.3% were diseased (thalassemia major) and 21.6% were normal. P-value of all results was less than 0.001. In this series, the highest frequency was found in Punjabi Rajputs. The commonest mutation was Fr 8-9. Most parents were first cousins. Premarital thalassemia carrier testing can effectively reduce the disease. (author)vely reduce the disease. (author)

146

Utilization of denaturing gradient gel electrophoresis for diagnosis of {beta}-thalassemia and ascertainment of new mutations  

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During the past two years we have tested 2,300 Southeast Asians for alpha- and beta-thaleassemia mutations. We found the incidence of hemoglobin E ({beta}{sup 26}) to be 47% among Laotians and 38% among Cambodians. The incidence of beta thalassemia trait is 9% for Laotians and 6% for Cambodians. Thus, the risk for hemoglobin E/{beta}{sup 26} thalassemia, a transfusion-dependent disorder, is increased in these two population groups. Denaturing gradient gel electrophoresis (DGGE) has proven to be useful in testing for beta-thalassemia carriers and identifying new mutations in the beta globin gene. DNA was extracted from venous blood obtained from patients with elevated Hgb A2 (>4%). Five DNA fragments, encompassing the beta globin gene cluster, were amplified by PCR and analyzed, along with known beta gene mutations as controls, by DGGE using different denaturing gradient concentrations. Different mutations at the same nucleotide position can be distinguished by migration pattern on the DGGE (e.g., in IVS-I-1, G{r_arrow}A and T). Compound heterozygotes for {beta}-thalassemia can be detected on the same gel (e.g., HbE/mutation codon 17). New mutations are identified by their migration pattern compared with controls and determined by subsequent sequencing. We have identified three new mutations: codon 82 CAA{r_arrow}AAA in one Cambodian patient; IVS-II-667, T{r_arrow}C and IVS-II-672, A{r_arrow}C in two Laotian patients. When the parent`s genotypes are known, prenatal diagnosis can be obtained within 24 hours. Thus, PCR/DGGE combination is a rapid and reliable diagnostic approach to clinically significant {beta}-thalassemia. The most important steps are carefully designed primers and predetermined gradient concentrations for DGGE.

Ngo, K.Y.; Liu, D.; Lee, J. [Univ. of California, San Diego (United States)] [and others

1994-09-01

147

Comparative analysis of RBC membrane fatty acids, proteins and glycophorin in patients with heterozygous beta thalassemia and iron deficiency anemia  

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Membrane lipid and protein composition was compared in erythrocytes from iron deficiency anemia (IDA) and heterozygous beta thalassemia patients. The study was planned to correlate the influence of iron deficiency with the intrinsic defect of the heterozygous condition on the membrane structural integrity as well as to investigate whether there are differences in membrane changes between the two conditions. Results indicate high levels of saturated fatty acids and low unsaturated fatty acids ...

Sanghani, S. P.; Haldankar, V. A.

2006-01-01

148

A New Index for Discrimination Between Iron Deficiency Anemia and Beta-Thalassemia Minor: Results in 284 Patients  

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The present study reports the results in 284 patients of applying a recently developed index, MCV - (10xRBC), for discrimination between beta-thalassemia trait (?-TT) and Iron Deficiency Anemia (IDA), the two most common causes of microcytic hypochromic anemias. A total of 284 carefully selected patients (130 patients with IDA and 154 with ?-TT) were studied. Sensitivity, specificity and Youden’s index were compared between the proposed index and four other indices, namely England-Fraser,...

Ehsani, M. A.; Shahgholi, E.; Rahiminejad, M. S.; Seighali, F.; Rashidi, A.

2009-01-01

149

Characterization of beta-thalassemia mutations in patients from the state of Rio Grande do Norte, Brazil  

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Full Text Available SciELO Brazil | Language: English Abstract in english 35 unrelated individuals were studied for characterization as either heterozygous or homozygous for beta-thalassemia. Molecular analysis was done by PCR/RFLP to detect the mutations most commonly associated with beta-thalassemia (?0IVS-I-1, ?+IVS-I-6, and ?039). In the patients who showed none of th [...] ese mutations, the beta-globin genes were sequenced. Of the 31 heterozygous patients, 13 (41.9%) had the ?+IVS-I-6 mutation, 15 (48.4%) the ?0IVS-I-1 mutation, 2 (6.5%) the ?+IVS-I-110 mutation and 1 (3.2%) the ?+IVS-I-5 mutation. IVS-I-6 was detected in the four homozygotes. The mutation in codon 39, often found in previous studies in Brazil, was not detected in the present case. This is the first study aiming at identifying mutations that determine beta-thalassemia in the state of Rio Grande do Norte.

Zama Messala Luna da, Silveira; Maria das Vitórias, Barbosa; Thales Allyrio Araújo de Medeiros, Fernandes; Elza Miyuki, Kimura; Fernando Ferreira, Costa; Maria de Fátima, Sonati; Ivanise Marina Moretti, Rebecchi; Tereza Maria Dantas de, Medeiros.

150

Characterization of beta-thalassemia mutations in patients from the state of Rio Grande do Norte, Brazil  

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Full Text Available 35 unrelated individuals were studied for characterization as either heterozygous or homozygous for beta-thalassemia. Molecular analysis was done by PCR/RFLP to detect the mutations most commonly associated with beta-thalassemia (?0IVS-I-1, ?+IVS-I-6, and ?039. In the patients who showed none of these mutations, the beta-globin genes were sequenced. Of the 31 heterozygous patients, 13 (41.9% had the ?+IVS-I-6 mutation, 15 (48.4% the ?0IVS-I-1 mutation, 2 (6.5% the ?+IVS-I-110 mutation and 1 (3.2% the ?+IVS-I-5 mutation. IVS-I-6 was detected in the four homozygotes. The mutation in codon 39, often found in previous studies in Brazil, was not detected in the present case. This is the first study aiming at identifying mutations that determine beta-thalassemia in the state of Rio Grande do Norte.

Zama Messala Luna da Silveira

2011-01-01

151

Study on Efficacy of Hepatitis B Immunization in Vaccinated Beta-Thalassemia Children in Tehran  

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Full Text Available Objective:In thalassemic children, HBV infection is common, thus immunization against HBV will reduce and prevent the rate of infection. The aim of this study was to evaluate the efficacy of HBV immunization and the prevalence of HBV infection in beta-thalassemic children in Tehran. Methods:To assess the efficacy of immunization and determine the immune response of children with beta-thalassemia, sera of 99 children who had received three doses (10/20 ?g of recombinant HBV vaccine in months 0, 1, 6, were selected and tested for HBsAg, HBsAb and anti-HBc by ELISA method. Also, these sera were tested for HBV DNA using nested-PCR method. Findings:In 99 beta-thalassemic children, 89 (89.9 % were anti-HBs positive (responders and 10 (10.1% anti-HBs negative (non-responders.Three cases (3.03% were anti-HBc positive and 1(1.01% was HBsAg positive. HBV DNA was not detected in any of them.Conclusion:Our results have revealed that hepatitis B vaccine is highly immunogenic for thalassemic children and particularly well tolerated.

Mahmood Mahmoodian Shooshtari

2010-06-01

152

Study on Efficacy of Hepatitis B Immunization in Vaccinated beta-thalassemia Children in Tehran  

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Full Text Available Objective:In thalassemic children, HBV infection is common, thus immunization against HBV will reduce and prevent the rate of infection. The aim of this study was to evaluate the efficacy of HBV immunization and the prevalence of HBV infection in beta-thalassemic children in Tehran.Methods:To assess the efficacy of immunization and determine the immune response of children with beta-thalassemia, sera of 99 children who had received three doses (10/20 ?g of recombinant HBV vaccine in months 0, 1, 6, were selected and tested for HBsAg, HBsAb and anti-HBc by ELISA method. Also, these sera were tested for HBV DNA using nested-PCR method.Findings:In 99 beta-thalassemic children, 89 (89.9 % were anti-HBs positive (responders and 10 (10.1% anti-HBs negative (non-responders.Three cases(3.03% were anti-HBc positive and 1(1.01% was HBsAg positive. HBV DNA was not detected in any of them.Conclusion:Our results have revealed that hepatitis B vaccine is highly immunogenic for thalassemic children and particularly well tolerated.

Zohreh Sharifi

2010-06-01

153

Hematological indices for differential diagnosis of Beta thalassemia trait and iron deficiency anemia.  

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Background. The two most frequent types of microcytic anemia are beta thalassemia trait ( ? -TT) and iron deficiency anemia (IDA). We retrospectively evaluated the reliability of various indices for differential diagnosis of microcytosis and ? -TT in the same patient groups. Methods. A total of 290 carefully selected children aged 1.1-16 years were evaluated. We calculated 12 discrimination indices in all patients with hemoglobin (Hb) values of 8.7-11.4?g/dL. None of the subjects had a combined case of IDA and ? -TT. All children with IDA received oral iron for 16 weeks, and HbA2 screening was performed after iron therapy. The patient groups were evaluated according to red blood cell (RBC) count; red blood distribution width index; the Mentzer, Shine and Lal, England and Fraser, Srivastava and Bevington, Green and King, Ricerca, Sirdah, and Ehsani indices; mean density of hemoglobin/liter of blood; and mean cell density of hemoglobin. Results. The Mentzer index was the most reliable index, as it had the highest sensitivity (98.7%), specificity (82.3%), and Youden's index (81%) for detecting ? -TT; this was followed by the Ehsani index (94.8%, 73.5%, and 68.3%, resp.) and RBC count (94.8%, 70.5%, and 65.3%). Conclusion. The Mentzer index provided the highest reliabilities for differentiating ? -TT from IDA. PMID:24818016

Vehapoglu, Aysel; Ozgurhan, Gamze; Demir, Ay?egul Dogan; Uzuner, Selcuk; Nursoy, Mustafa Atilla; Turkmen, Serdar; Kacan, Arzu

2014-01-01

154

History and origin of beta-thalassemia in Turkey: sequence haplotype diversity of beta-globin genes.  

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In the present study we report the sequence haplotypes associated with 22 beta-globin gene mutations present in Turkey. Nine nucleotide polymorphisms and an (AT)xTy motif located at the 5' end of the beta-globin gene form the sequence haplotypes that were investigated in 204 unrelated beta-thalassemia and wild-type chromosomes from Turkey. Twelve sequence haplotypes were observed in the chromosomes analyzed and haplotypic heterogeneity was found in the wild-type beta-globin genes. Samples from the Black Sea region demonstrated a remarkable level of haplotypic heterogeneity in contrast to the homogeneity present in Central Anatolian samples. Of the 22 beta-globin mutations analyzed, 18 were related with single sequence haplotypes. This simple association led to the attempt to determine the origin of these mutations by comparing their frequencies in Turkey with those in other countries and/or the world distribution of the haplotypes carrying them. However, the presence of several exceptions for the "one haplotype/one mutation" rule showed that the beta-globin gene cluster is far from static. Each of the IVS-I-110 (G-->A), Cd 39 (C-->T), IVS-I-6 (T-->C), and -30 (T-->A) beta-globin mutations was associated with a minimum of two sequence haplotypes. This fact is best explained by the likelihood of strong recombination mechanisms taking place, rather than by assuming multiple origins for each of these alleles. According to our results, malarial selection for the oldest beta-thalassemia allele in Anatolia (i.e., IVS-I-110 G-->A) may have occurred between 6500 and 2000 B.C. From that date on, most of the common beta-thalassemia mutations in Turkey were established, and by the 13th century A.D. most of them were brought to frequencies close to those observed at present. PMID:11758688

Tadmouri, G O; Garguier, N; Demont, J; Perrin, P; Ba?ak, A N

2001-10-01

155

Frequency of Beta-globin gene mutations in beta-thalassemia patients from east of Mazandaran  

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Full Text Available (Received 6 Aug, 2008; Accepted 3 Dec, 2008 Abstract Background and purpose: Beta-thalassemia is the most common inherited disorder in the world, especially in Iran. According to Iranian thalassemia society registry, 18616 thalassemia patients now living in Iran, which Mazandaran and Fars provinces have the most patients. Previous reports have shown that the frequency of b-thalassemia carriers is more than 10% in Mazandaran province. Although b-thalassemia is very heterogenous in the molecular level, but in each population, 5 to 10 mutations are more common. In this research common mutation in eastern area of Mazadaran province was investigated. Materials and methods: 5 to 10 ml peripheral blood samples were collected from volunteer patients who were referred to Boali Sina Hospital in Sari. DNA was extracted from blood, then 20 different mutations were screened and detected using two different methods, ARMS-PCR and Reverse-Dot Blot in Thalassemia Research Center in Sari and Amir Kola Thalassemia Center. Results: From 240 chromosomes investigated in 120 b-thalassemia patients in total, 96.25% mutations were identified. 13 different mutations were identified from 231 chromosomes. Among different mutations investigated, IVSII-1G>A was detected as the most common with frequency of 68/3%, which was homozygous in 64 individuals (53/3% and compound heterozygous with other mutations in 34 individuals (28/3% respectively. Mutations C8(-AA, codon22(G>A/ FSC 22/23/24(-7bp, codon 30(G>A, and IVSII-1G>A were identified in 83% of chromosomes which were studied (200 chromosomes from 240. Conclusion: Mutation IVSII-1G>A is the most common mutation in northern provinces (Gilan, mazandaran, Golestan in recent study. Also, comparison of these results with the similar finding from other provinces showed that the distributions of mutations in the northern area are different with northwest, south or southeast of the country.

Mohammad Bagher Hashemi Soteh

2008-01-01

156

A new valid formula in differentiating iron deficiency Anemia from beta-thalassemia trait  

International Nuclear Information System (INIS)

Objective: To compare the validity of a new index (Hb X RDW X 100/ (RBC)/sup 2/ X MCHC) with twelve discriminating functions (DFs) to differentiate iron deficiency anemia (IDA) and beta-thalassemia trait (beta-TT). Methodology: A total of 823 patients (317 IDA and 506 beta-TT) aged 15 to 35 year old were enrolled in this study. The diagnostic sensitivity, specificity and other validity parameters were calculated to assess the diagnostic reliability of the novel index [Keikhaei index (KI)] vis-a-vis the other published DFs [Mentzer Index (MI), Green and King Index (G and KI), red cell distribution width index (RDWI), England and Fraser Index (E and FI), Bessman and Feinstein index (B and FI), Telmissani et al index (TI), Srivastava and Bevington index (S and BI), Shine and Lal index (S and LI), Ricerca et al index (RI), Ehsani et al index (EI), Sirdah et al index (SI), and Red Blood Cell Count(RBC)] were calculated in all patients. Results: All thirteen DFs did not have the sensitivity and specificity of 100%. The KI, RDWI, Gand KI and E and FI showed the most sensitivity and specificity for both IDA and TT; moreover, the lowest reliable indices belonged to B and FI, SandLI and RI. Conclusion: According to Youden's index (YI), DFs in the order of highest to lowest were KI > G and KI > RDWI > E and FI > RBC> M I> EI > TI > SI > S and BI > RI> SandLI >B and FI. (author)

157

Peripheral Blood stem cell transplantation in children with Beta-thalassemia major  

International Nuclear Information System (INIS)

Objective: To share the preliminary data on stem cell transplantation in Pakistan. Results: Engraftment was achieved in all patients except one who required a second dose of bone marrow graft on day +21. Median time to achieve absolute neutrophil count of > 0.5 x 10/sup 9/ /l was 9.0 days (range 8 - 31 days) and platelet count of > 20 x 10/sup 9/ /l was 14 days (12 - 35 days). Acute GVHD was seen in 3 patients, one patient had grade IV gut GVHD; another patient had grade III gut GVHD while third patient had grade II skin GVHD. Median hospital stay was 29 days. Six patients were well and transfusion independent 3 to 36 months post transplant. One episode of primary graft failure required a second dose of bone marrow harvest. Another episode of graft rejection received two doses of donor lymphocytes infusion. There were 4 deaths due to grade IV gut GVHD because of uncontrolled systemic Candida infection and one due to hepatic veno-occlusive (VOD) disease. Conclusion: Allogeneic peripheral blood stem cell transplantation can be safely and economically carried out in Pakistan. Although there had been 4 deaths during 36 months follow-up, with increasing understanding and experience the outcome is expected to improve. (author)

158

Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran  

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Full Text Available Abstract Background The combination of transfusion and chelation therapy has dramatically extended the life expectancy of thalassemic patients. The main objective of this study is to determine the prevalence of prominent thalassemia complications. Methods Two hundred twenty patients entered the study. Physicians collected demographic and anthropometric data and the history of therapies as well as menstrual histories. Patients have been examined to determine their pubertal status. Serum levels of 25(OH D, calcium, phosphate, iPTH were measured. Thyroid function was assessed by T3, T4 and TSH. Zinc and copper in serum were determined by flame atomic absorption spectrophotometry. Bone mineral density (BMD measurements at lumbar and femoral regions have been done using dual x-ray absorptiometry. The dietary calcium, zinc and copper intakes were estimated by food-frequency questionnaires. Results Short stature was seen in 39.3% of our patients. Hypogonadism was seen in 22.9% of boys and 12.2% of girls. Hypoparathyroidism and primary hypothyroidism was present in 7.6% and 7.7% of the patients. About 13 % of patients had more than one endocrine complication with mean serum ferritin of 1678 ± 955 micrograms/lit. Prevalence of lumbar osteoporosis and osteopenia were 50.7% and 39.4%. Femoral osteoporosis and osteopenia were present in 10.8% and 36.9% of the patients. Lumbar BMD abnormalities were associated with duration of chelation therapy. Low serum zinc and copper was observed in 79.6% and 68% of the study population respectively. Serum zinc showed significant association with lumbar but not femoral BMD. In 37.2% of patients serum levels of 25(OH D below 23 nmol/l were detected. Conclusion High prevalence of complications among our thalassemics signifies the importance of more detailed studies along with therapeutic interventions.

Hashemi Reza

2003-08-01

159

Soluble transferrin receptor and immature reticulocytes are not useful for distinguishing iron-deficiency anemia from heterozygous beta-thalassemia  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: English Abstract in portuguese A deficiência de ferro e a beta-talassemia heterozigótica são importantes causas de microcitose e hipocromia. São propostos dois parâmetros laboratoriais para auxiliar na diferenciação entre essas anemias. O número de reticulócitos altamente imaturos e níveis do receptor solúvel da transferrina fora [...] m determinados em pacientes com anemia ferropriva (n = 49) e com beta-talassemia heterozigótica (n = 43). Não houve diferença significativa entre os valores de reticulócitos altamente imaturos e níveis de receptor solúvel da transferrina nos dois grupos, mas foi observada uma correlação entre reticulócitos altamente imaturos e níveis de receptor solúvel da transferrina no grupo com anemia ferropriva, provavelmente devido à um estímulo para a síntese do receptor em resposta à deprivação de ferro nos eritrócitos. Abstract in english Iron deficiency and heterozygous beta-thalassemia are important causes of hypochromic-microcytic anemia. Two laboratory parameters are suggested for the differentiation of such anemia. High-fluorescence reticulocyte counts and soluble transferrin receptor levels were determined in iron-deficiency an [...] emia patients (n = 49) and heterozygous beta-thalassemia patients (n = 43). There was no significant difference in high-fluorescence reticulocyte and soluble transferrin receptor values between the two groups, but a correlation was observed between high-fluorescence reticulocytes and soluble transferrin receptors in iron-deficiency anemia, probably due to increased receptor synthesis as a response to decreased iron content in erythrocytes.

Gisélia Aparecida Freire Maia de, Lima; Helena Zerlotti Wolf, Grotto.

160

Serum ferritin levels, socio-demographic factors and desferrioxamine therapy in multi-transfused thalassemia major patients at a government tertiary care hospital of Karachi, Pakistan  

OpenAIRE

Abstract Background Beta thalassemia is the most frequent genetic disorder of haemoglobin synthesis in Pakistan. Recurrent transfusions lead to iron-overload manifested by increased serum Ferritin levels, for which chelation therapy is required. Findings The study was conducted in the Pediatric Emergency unit of Civil Hospital Karachi after ethical approval by the Institutional Review Board of Dow University of Health Sciences. Seventy nine cases of beta thalassemia major were included after ...

Rehman Anis; Ullah Faizan; Aziz Sina; Khan Muhammad; Riaz Talha; Riaz Haris; Zafar Qandeel; Kazi Abdul

2011-01-01

161

Hemoglobina C em homozigose e interação com talassemia beta Homozygous hemoglobin C and its interaction with beta thalassemia  

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Full Text Available A hemoglobina C (Hb C é originária do oeste da África e é detectada por migração lenta na eletroforese alcalina em acetato de celulose. Consiste na mutação do gene da globina beta no códon 6 (GAG-AAG, resultando na substituição do sexto aminoácido da cadeia beta da hemoglobina humana, o ácido glutâmico, pelo aminoácido lisina. A cromatografia de alto desempenho (HPLC separa completamente as frações C e A2, permitindo caracterizar a presença da interação com talassemia beta. Esta entidade (Hb CC, em homozigoze é considerada benigna em relação à doença falciforme, já que a falcização não faz parte de sua fisiopatologia. A raridade do diagnóstico C homozigoto e C talassemia beta nos pacientes portadores de hemoglobinopatias nos alertou para a necessidade de se conhecer melhor e estudar aspectos clínicos e hematológicos dos casos dessa mutação em homozigose e na interação com a talassemia beta no ambulatório de anemias do Centro Regional de Hematologia e Hemoterapia de Ribeirão Preto, SP, Brasil.Hemoglobin C (Hb C originated in the west of Africa and is detected by alkaline electrophoresis by slow migration in cellulose acetate. It consists of a mutation of the beta globin gene in codon 6 (GAG-AAG, resulting in a substitution of glutamic acid, the sixth amino acid of the beta string of the human hemoglobin, for lysine. High performance chromatography (HPLC separates the C and A2 fractions completely, allowing the characterization of the presence of interactions with thalassemia beta. This entity (Hb CC is considered benign in respect to sickle cell disease, as sickle cells are not part of its physiopathology. The rarity of the diagnosis of homozygous C and beta thalassemia in patients with hemoglobinopathies showed the necessity of studying clinical and hematologic aspects of the cases of this mutation in homozygosis carriers and the interaction with beta thalassemia in the anemias clinic of the Regional Blood Center in Ribeirao Preto, SP, Brazil.

Ivan L. Angulo

2009-01-01

162

Perfil de beta talassemia heterozigota obtido a partir de análise data mining em banco de dados / The profile of beta thalassemia obtained by data mining analysis in a database  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: Portuguese Abstract in portuguese [...] Abstract in english Variations in the phenotypic expression of heterozygous beta thalassemia reflect the formation of different populations. To better understand the profile of heterozygous beta-thalassemia of the Brazilian population, we aimed at establishing parameters to direct the diagnosis of carriers and calculat [...] e the frequency from information stored in an electronic database. Using a Data Mining tool, we evaluated information on 10,960 blood samples deposited in a relational database. Over the years, improved diagnostic technology has facilitated the elucidation of suspected beta thalassemia heterozygote cases with an average frequency of 3.5% of referred cases. We also found that the Brazilian beta thalassemia trait has classic increases of Hb A2 and Hb F (60%), mainly caused by mutations in beta zero thalassemia, especially in the southeast of the country.

Ana L. B., Domingos; Lucas A., Granzotto; Edis, Belini Junior; Thiago Y. K., Oliveira; Ana C. B., Domingos; Claudia R., Bonini-Domingos.

2010-02-01

163

Perfil de beta talassemia heterozigota obtido a partir de análise data mining em banco de dados The profile of beta thalassemia obtained by data mining analysis in a database  

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Full Text Available Variations in the phenotypic expression of heterozygous beta thalassemia reflect the formation of different populations. To better understand the profile of heterozygous beta-thalassemia of the Brazilian population, we aimed at establishing parameters to direct the diagnosis of carriers and calculate the frequency from information stored in an electronic database. Using a Data Mining tool, we evaluated information on 10,960 blood samples deposited in a relational database. Over the years, improved diagnostic technology has facilitated the elucidation of suspected beta thalassemia heterozygote cases with an average frequency of 3.5% of referred cases. We also found that the Brazilian beta thalassemia trait has classic increases of Hb A2 and Hb F (60%, mainly caused by mutations in beta zero thalassemia, especially in the southeast of the country.

Ana L. B. Domingos

2010-02-01

164

Twenty-year outcome analysis of genetic screening programs for Tay-Sachs and beta-thalassemia disease carriers in high schools.  

OpenAIRE

Programs for education, screening, and counseling of senior-high-school students, in populations at high risk for Tay-Sachs and beta-thalassemia diseases, have existed for >20 years in Montreal. Four process and outcome variables are reported here: (i) voluntary participation rates in the high-school cohort; (ii) uptake rates for the screening test; (iii) origin of carrier couples seeking the prenatal diagnosis option in the programs; and (iv) change in incidence of the two diseases. Between ...

Mitchell, J. J.; Capua, A.; Clow, C.; Scriver, C. R.

1996-01-01

165

Safety, tolerability, and pharmacokinetics of ICL670, a new orally active iron-chelating agent in patients with transfusion-dependent iron overload due to beta-thalassemia  

OpenAIRE

ICL670 is an orally active representative of a new class of tridentate iron chelator developed for the treatment of blood transfusion-dependent iron overload in chronic anemias. In this randomized, double-blind study, patients with transfusion-dependent beta-thalassemia received single oral doses of ICL670 ranging from 2.5 to 80 mg/kg to investigate its safety, tolerability, and pharmacokinetics and to obtain preliminary information on pharmacodynamic effects. ICL670 was well tolerated, and n...

Piga, Antonio Giulio

2003-01-01

166

Extraosseous radiotracer uptake on bone scan in beta-thalassemia: report of one case; Fixation extraosseuse du radiotraceur lors de la realisation d'une scintigraphie du squelette chez un patient atteint de beta-thalassemie: a propos d'un cas  

Energy Technology Data Exchange (ETDEWEB)

Red blood cell transfusion, main therapeutic modality of beta-thalassemia, leads to iron overload which may perturb several metabolic ways. The aim of this paper is to illustrate the uptake abnormalities observed on bone scan of thalassaemic patients and to discuss mechanisms of extraosseous accumulation of the radiopharmaceutical in this pathology. We report a 16-year-old child suffering from beta-thalassemia major undergoing transfusion therapy. A bone scan was indicated to look for osseous infection. This study revealed a little skeletal uptake and abnormal liver, splenic and renal accumulation. A repeat bone scan, performed three weeks later showed a better skeletal uptake which enabled the discovery of focal abnormalities and made the diagnostic easier. The effect of iron overload on radiopharmaceuticals uptake in bone scan is known since 1975. Dissociation of {sup 99m}Tc from the carrier ligand due to the presence of iron excess seems the most plausible hypothesis. Free {sup 99m}Tc can be bound to other tissular substrates which can explain extraosseous uptake. The normally available pool for bone is reduced and then the skeletal uptake decreased. This report limits considerably the sensitivity of the bone scan. A well-led iron chelation and eventually the use of diuretic drug may guarantee a better quality of bone scan images. (authors)

Guezguez, M.; Nouira, M.; Sfar, R.; Chatti, K.; Ben Fradj, M.; Ben Ali, K.; Ajmi, S.; Essabbah, H. [CHU Sahloul, Service de Medecine Nucleaire, Sousse (Tunisia); Zrour, S. [EPS F. Bourguiba, Service de Rhumatologie, Monastir (Tunisia)

2009-10-15

167

Reliability of red blood cell indices and formulas to discriminate between beta thalassemia trait and iron deficiency in children.  

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Iron deficiency (ID) and beta thalassemia trait (betaTT) are the most common causes of hypochromia and microcytosis. This study evaluates the reliability of some of the red blood cell (RBC) indices and the formulas used in the differentiation between betaTT and ID in a cohort of 458 children aged between 1.8 and 7.5 years (mean age 5.6+/-1.7 years) with mild hypochromic microcytic anemia. Within this group, 243 were confirmed with ID and 215 with betaTT. Red cell indices derived from automated red cell analyzers were used to evaluate the following discriminant indices and formulas: Mentzer Index (MI), Green and King Index (G&K), England and Fraser Index (E&F), RBC Distribution Width Index (RDWI), RBC distribution width (RDW) and RBC count. Sensitivity (SENS), specificity (SPEC), positive and negative prognostic value, efficiency (EFF) and Youden's Index (YI) were evaluated. For each index or formula Gauss curves were constructed. The highest SENS was obtained with RDWI (78.9%), while the highest SPEC and YI with E&F (99.1 and 64.2% respectively), the highest EFF (80.2%) with G&K. Gauss curves obtained from betaTT and ID children showed a different degree of overlap for each formula or index. In conclusion, none of RBC indices or formulas appears reliable to discriminate between betaTT and ID subjects. PMID:20423571

Ferrara, M; Capozzi, L; Russo, R; Bertocco, F; Ferrara, D

2010-04-01

168

A new index for discrimination between iron deficiency anemia and beta-thalassemia minor: results in 284 patients.  

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The present study reports the results in 284 patients of applying a recently developed index, MCV-(10 x RBC), for discrimination between beta-thalassemia trait (beta-TT) and Iron Deficiency Anemia (IDA), the two most common causes of microcytic hypochromic anemias. A total of 284 carefully selected patients (130 patients with IDA and 154 with beta-TT) were studied. Sensitivity, specificity and Youden's index were compared between the proposed index and four other indices, namely England-Fraser, Mentzer, Srivastava and RBC count. The new index correctly identified 263 (92.96%) patients, standing inferior only to Mentzer which correctly diagnosed 269 (94.71%) patients. The best discrimination index according to Youden's criteria was Mentzer (Youden's index = 90.1) followed by the new index (Youden's index = 85.5). There are remarkable inconsistencies among the results obtained in different studies. Larger studies are needed to establish the optimal discrimination index as well as to confirm the results obtained in the present study. Nevertheless, the epidemiological indices of the proposed discrimination index and the simplicity of its calculation make it acceptable for use in Iran. PMID:19579993

Ehsani, M A; Shahgholi, E; Rahiminejad, M S; Seighali, F; Rashidi, A

2009-03-01

169

A New Index for Discrimination Between Iron Deficiency Anemia and Beta-Thalassemia Minor: Results in 284 Patients  

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Full Text Available The present study reports the results in 284 patients of applying a recently developed index, MCV - (10xRBC, for discrimination between beta-thalassemia trait (?-TT and Iron Deficiency Anemia (IDA, the two most common causes of microcytic hypochromic anemias. A total of 284 carefully selected patients (130 patients with IDA and 154 with ?-TT were studied. Sensitivity, specificity and Youden’s index were compared between the proposed index and four other indices, namely England-Fraser, Mentzer, Srivastava and RBC count. The new index correctly identified 263 (92.96% patients, standing inferior only to Mentzer which correctly diagnosed 269 (94.71% patients. The best discrimination index according to Youden’s criteria was Mentzer (Youden’s index = 90.1 followed by the new index (Youden’s index = 85.5. There are remarkable inconsistencies among the results obtained in different studies. Larger studies are needed to establish the optimal discrimination index as well as to confirm the results obtained in the present study. Nevertheless, the epidemiological indices of the proposed discrimination index and the simplicity of its calculation make it acceptable for use in Iran.

M.A. Ehsani

2009-01-01

170

Twenty-year outcome analysis of genetic screening programs for Tay-Sachs and beta-thalassemia disease carriers in high schools.  

Science.gov (United States)

Programs for education, screening, and counseling of senior-high-school students, in populations at high risk for Tay-Sachs and beta-thalassemia diseases, have existed for >20 years in Montreal. Four process and outcome variables are reported here: (i) voluntary participation rates in the high-school cohort; (ii) uptake rates for the screening test; (iii) origin of carrier couples seeking the prenatal diagnosis option in the programs; and (iv) change in incidence of the two diseases. Between 1972 and 1992, we screened 14,844 Ashkenazi-Jewish students, identified 521 HexA-deficient carriers (frequency 1:28), reached 89% of the demographic cohort in the educational component of the program, and achieved 67% voluntary participation in the subsequent screening phase. The corresponding data for the beta-thalassemia program are 25,274 students (mainly of Mediterranean origin) representing 67% of the cohort with 61% voluntary participation in the screening phase (693 carriers; frequency 1:36). From demographic data, we deduce that virtually all the carriers identified in the high-school screening program remembered their status, had their partner tested if they did not already know they were a carrier couple, and took up the options for reproductive counseling/prenatal diagnosis. In Montreal, the current origin of all couples using prenatal diagnosis for Tay-Sachs and beta-thalassemia diseases is the corresponding genetic screening/testing program, whereas, at the beginning of the programs, it was always because there was a history of an affected person in the family. Incidence of the two diseases has fallen by 90%-95% over 20 years; the rare new cases are born (with two exceptions) outside the target communities or to nonscreened couples. PMID:8808593

Mitchell, J. J.; Capua, A.; Clow, C.; Scriver, C. R.

1996-01-01

171

beta-thalassaemia major hos børn og unge i Danmark  

DEFF Research Database (Denmark)

INTRODUCTION: Beta-thalassemia major occurs with increasing frequency among Danish children as a result of immigration. The aim of the study was to estimate the occurrence of beta-thalassemia major in Denmark, analyse the treatment and organ functions, and identify areas for an improved treatment strategy. MATERIAL AND METHODS: During 1998-99 all Danish pediatric departments were contacted for identification of children aged 0-18 years with beta-thalassemia major. Blood transfusions and chelation therapy were registered, and for Eastern Denmark clinical, endocrine, cardiac, and serologic parameters were performed. RESULTS: Twenty-six children had beta-thalassemia major. Out of these, 20 received blood transfusions, and 17 patients were chelated. Eight patients were not chelated owing to previous bone marrow transplantation, treatment with hydroxyurea or ferritin < 2000 micrograms/l and young age. One patient had died. The body height was between 1.5 and -5.4 SDS (median -1.7) and the sitting height was -0.6 to -5.6 SDS (median -2.3). The bone age was delayed 1-5 years (median -2.5) in six out of ten examined patients, and puberty delayed in four out of five. A dilated left ventricle was documented in one out of eight patients examined. All patients were HIV and hepatitis C negative. For 75% of the children, the parents were related. DISCUSSION: Children and adolescents with beta-thalassemia major in Denmark experience major heterogenicity with regard to treatment and late effects. An earlier and more effective iron chelation therapy together with improved patient support may reduce growth disturbances and endocrine and cardiac late effects. Udgivelsesdato: 2002-Dec-2

Jung, Anne; Main, Katharina Maria

2002-01-01

172

An SP1-binding site polymorphism in the COLIAI gene and osteoporosis in Egyptian patients with thalassemia major.  

Science.gov (United States)

?-Thalassemia major is an inherited blood disorder, which mainly affects the Mediterranean region. Osteoporosis represents an important cause of morbidity in ?-thalassemia major and its pathogenesis has not been completely clarified. Genetic factors play an important role in the pathogenesis of osteoporosis and several candidate gene polymorphisms have been implicated in the regulation of this process. A G?T polymorphism in the regulatory region of the collagen type I alpha 1 (COLIAI) gene at a recognition site for transcription factor Sp1 has been strongly associated with osteoporosis. The aim of the present study was to examine the distribution of COLIAI polymorphism and its relationship with bone mineral density (BMD) at the lumbar spine and femur in patients and controls. In this study, the G?T polymorphism was detected in 31 Egyptian ?-thalassemia major patients and 20 healthy controls and its possible association with BMD was investigated. Alleles S and s were detected by the presence of a G or T nucleotide, respectively, in a regulatory site of the COLIAI gene using polymerase chain reaction (PCR). A total of 80.6% of the ?-thalassemia patients were homozygous for G/G (SS) and 19.4% were heterozygotes for G/T (Ss) polymorphism. There was no ss genotype in our patients. In the control group, 70 and 30% had SS and Ss genotypes, respectively. There was no significant difference between Z-score of patients with SS and Ss at head of femur (P = 1) or at lumbar spine (P = 0.48). Conclusion Our results raise the possibility that genotyping at the Sp1 site could be of clinical value in identifying the thalassemic patients at risk of developing osteoporosis. PMID:21178584

Hamed, Hanan M; Galal, Ashraf; Ghamrawy, Mona E L; Abd El Azeem, Khaled; Hussein, Ibtessam Ramzi; Abd-Elgawad, Mona Fayez

2011-03-01

173

Fatty-acid peroxidation in relation to trace elements in serum of patients with homozygous sickle-cell anemia and beta-thalassemia: a gas-chromatographic study.  

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Due mainly to a deeply disturbed iron metabolism, intense production of oxygen-derived free radicals occurs in genetic hemoglobinopathies such as homozygous sickle-cell anemia and beta-thalassemia. Together with impairments in the natural factors involved in oxy-radical detoxication, this results in intense oxidative stress leading to lipid peroxidation in the blood components. In search of peroxidation effects, we undertook a gas chromatographic study of both the total and phospholipid-bound fatty acids in the serum from sickle-cell disease and beta-thalassemia patients. Specific alterations of pathologic origin have been evidenced in the profiles of total and phospholipidic fatty acids, as well as in the elongation-desaturation ratios of the total fatty acids. Results are consistent with lipid peroxidations and fatty-acid biosynthesis disturbances in both diseases, but more severe in thalassemia than in sickle-cell anemia. Increased serum selenium in the latter disease might exert a protective action against lipid peroxidation. PMID:8748219

Preoteasa, E A; Ionescu, I

1995-01-01

174

Genética molecular de beta talasémicos heterocigotas: Interrelación con parámetros hematológicos / Molecular genetics of the heterozygous beta-thalassemia: Interrelation with hematological parameters  

Scientific Electronic Library Online (English)

Full Text Available SciELO Cuba | Language: Spanish Abstract in spanish En Argentina no hay datos publicados sobre la interrelación entre el fenotipo de la mutación responsable de b-talasemia (b+ ó b0) y los parámetros hematológicos. En el presente trabajo se estudió las 6 mutaciones más frecuentes en la zona del Mediterráneo en 99 portadores de beta talasemia no relaci [...] onados de Rosario y zona de influencia. Los parámetros hematimétricos y la morfología eritrocitaria de sangre periférica de los portadores de beta talasemia no permitieron sospechar una determinada mutación. Se encontró diferencias levemente significativas (p Abstract in english No data have been published in Argentina about the interrelation existing between the phenotype of the mutation responsible for b-thalassemia (b+ or bO) and the hematological parameters. In the present paper, 6 of the most frequent mutations occurred in the Mediterranean zone in 99 non-related carri [...] ers of beta-thalassemia from Rosario and the influence zone, were studied. The hematimetric parameters and the erythrocytic morphology of peripheral blood of the carriers of beta-thalassemia did not allow to suspect of a determined mutation. Slightly significant differences (p

Irma Margarita, Bragós; Nélida Inés, Noguera; Mariana Paula, Raviola; Ángela Cristina, Milani.

2005-04-01

175

Genética molecular de beta talasémicos heterocigotas: Interrelación con parámetros hematológicos Molecular genetics of the heterozygous beta-thalassemia: Interrelation with hematological parameters  

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Full Text Available En Argentina no hay datos publicados sobre la interrelación entre el fenotipo de la mutación responsable de b-talasemia (b+ ó b0 y los parámetros hematológicos. En el presente trabajo se estudió las 6 mutaciones más frecuentes en la zona del Mediterráneo en 99 portadores de beta talasemia no relacionados de Rosario y zona de influencia. Los parámetros hematimétricos y la morfología eritrocitaria de sangre periférica de los portadores de beta talasemia no permitieron sospechar una determinada mutación. Se encontró diferencias levemente significativas (pNo data have been published in Argentina about the interrelation existing between the phenotype of the mutation responsible for b-thalassemia (b+ or bO and the hematological parameters. In the present paper, 6 of the most frequent mutations occurred in the Mediterranean zone in 99 non-related carriers of beta-thalassemia from Rosario and the influence zone, were studied. The hematimetric parameters and the erythrocytic morphology of peripheral blood of the carriers of beta-thalassemia did not allow to suspect of a determined mutation. Slightly significant differences (p < 0.10 were found in the average values of HCM for the different mutations considered. For the genotype b + I-6, it was marginally higher than for the genotype b + II-745. There were no significant differences among the other 4 genotypes

Irma Margarita Bragós

2005-04-01

176

A 65 bp deletion in band 3 gene of beta-thalassemia patients in Indonesia.  

Science.gov (United States)

We investigated whether in addition to the well known genetic alteration in red blood cell membrane band 3 protein, a deletion of 9 amino acids leading to ovalocytosis, other mutations to band 3 also exist. In 12% of our thalassemia major patients investigated, we found two bands in the agarose gel-electrophoresis of PCR products from band 3 gene with a difference of 65 +/- 10 bp, equivalent to a deletion of 20 to 25 amino acids in band 3 protein. Thus, a co-existing band 3-mutant allele in addition to the thalassemic globin gene defects, could also contribute to erythrocyte membrane defects and to the spectrum of clinical symptoms of these thalassemia major patients. PMID:24964668

Dewajanthi, Anna Maria; Lubis, Vita K; Wanandi, Septelia Inawati; Gatot, Djajadiman; Soegianto, Rondang R; Freisleben, Seruni K U; Wahidiyat, Iskandar; Freisleben, Hans-Joachim

2014-01-01

177

Coincidence of Niemann-Pick Disease and Beta-Thalassemia; a Case Report  

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Full Text Available Background: Niemann-Pick disease and ?-thalassemia are distinct conditions with specific clinical and morphological manifestations. ?-thalassemia is the most common inherited blood disorder in Iran whereas Niemann-Pick disease, a lysosomal storage disorder, is rarely found in this country.Case Presentation: This 5-month old girl, a known case of ?-thalassemia major was hospitalized for failure to thrive and hepathosplenomegaly. Because of unusual splenomegaly and liver enzymes disturbance that was not compatible with the first diagnosis, further evaluation revealed cherry red spot and high lipid profile suggestive of lysosomal storage disease. Foamy cells in the bone marrow and low activity of the specific enzyme led to the diagnosis of Niemann-Pick disease.Conclusion: This unique case illustrates the importance of looking for a second pathological condition in a patient whose clinical profile does not support the first diagnosis in its entirety.

Mina Izadyar

2010-12-01

178

Coincidence of Niemann-Pick Disease and beta-Thalassemia; a Case Report  

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Full Text Available Background:Niemann-Pick disease and ?-thalassemia are distinct conditions with specific clinical and morphological manifestations. ?-thalassemia is the most common inherited blood disorder in Iran whereas Niemann-Pick disease, a lysosomal storage disorder, is rarely found in this country. Case Presentation:This 5-month old girl, a known case of ?-thalassemia major was hospitalized for failure to thrive and hepathosplenomegaly. Because of unusual splenomegaly and liver enzymes disturbance that was not compatible with the first diagnosis, further evaluation revealed cherry red spot and high lipid profile suggestive of lysosomal storage disease. Foamy cells in the bone marrow and low activity of the specific enzyme led to the diagnosis of Niemann-Pick disease.Conclusion:This unique case illustrates the importance of looking for a second pathological condition in a patient whose clinical profile does not support the first diagnosis in its entirety.

Fatemeh Farahmand

2010-12-01

179

Study of Serum Haptoglobin Level and its Relation to Erythropoietic Activity in Beta Thalassemia Children  

Science.gov (United States)

Background Serum haptoglobin (Hp) is a reliable marker for hemolysis regardless the inflammatory state. Objective We investigated the possible relation between Hp depletion and hemolysis severity, hepatitis C virus (HCV) infection and iron load in ?-thalassemia children. Methods Twenty two ?-thalassemia major (TM),20 ?-thalassemia intermedia (TI) children with 20 age and sex matched healthy controls were involved. Pre-transfusion hemoglobin level was considered. Serum ferritin, Hp and transferrin receptor levels (sTfR) (by ELISA ), alanine aminotransferase (ALT) and aspartate aminotransferase (AST) (by colorimetric method) were assayed. Markers of hepatitis C virus (HCV) were done by PCR. Results The mean Hp levels among the studied groups were as follows; 8.02 ± 0.93 (mg/dl), 8.6 ±0.72 (mg/dl) and 122 ± 18.5(mg/dl) for TM, TI and the controls respectively. Both patient groups had significantly lower Hp level compared to the controls (Pthalassemia children combined and in each group (TM and TI) as well as among HCV infected children. STfR was the only significant independent predictor for serum Hp level (t= ?5.585, Pthalassemia had significant relation to disease severity and correlated well with their erythropoietic activity, as assessed by the measurement of sTfR without significant relation to HCV infection. Extensive multicenter studies are recommended. PMID:25745546

Ragab, Seham M.; Safan, Manal A.; Badr, Eman A.

2015-01-01

180

Egyptian Constellations  

Science.gov (United States)

The ancient Egyptians had a wide range of constellations, which populated the skies of ancient Egypt (see Chap. 133, "Orientation of Egyptian Temples: An Overview", 10.1007/978-1-4614-6141-8_146) for more than three millennia, whose knowledge has come to us through lists of stars and other representations which have mainly appeared in a dozen of coffins, a few clepsydrae, and a good set of astronomical ceilings. Hence, by making very simple assumptions and using the information provided by the ancient Egyptian sky-watchers, it is possible to uncover a great part of the ancient Egyptian firmament. This celestial tapestry was populated by the images of animals, symbols, and divinities that were most significant in understanding the Egyptian interpretation of the cosmos.

Lull, José; Belmonte, Juan Antonio

181

Egyptian gas for sale  

International Nuclear Information System (INIS)

Israel, Jordan, Palestine, the Lebanon, Turkey and Italy all have at least one factor in common: they're potential customers for Egyptian gas. Once under-used, this major resource is now set to become a valuable export commodity by the turn of the century. (author)

182

Egyptian astronomy.  

Science.gov (United States)

In Egypt, stars and planets were seen as goods and astronomy was practiced by priests in the temples. The most important time cycle of the Egyptian calendar was that of the heliac rising of Sirius during all the history of Ancient Egypt. Thus Egyptian astronomers built a calendar of 365 days, which was never corrected and in which the heliacal raising was evidently shifting at the rate of one day every four years. The year had twelve months of thirty days and a holy period of five days.

Andrillat, H.

183

Evaluating the role of indirect bilirubin, urobilinogen and Shine AND Lal index as an alternative screening tool for beta thalassemia minor  

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Methods: The present study was conducted on 100 (n=100 subjects in blood bank, department of pathology, government medical college Rajkot, Gujarat, India. In first group 50 subjects (Thalassemia minor were selected while in second group 50 (n2=50 normal individuals from hospital staff were selected. Complete-haemogram, serum-direct, indirect and total bilirubin, urine urobilinogen and their sensitivity and specificity were calculated. Results: Of the 50 cases in test group, 41 had higher Indirect Bilirubin level (>0.7 mg/dl, 35 had high urobilinogen level (>1 mg/dl. In control group out of 50 cases, 3 had high indirect bilirubin levels, 4 had high urobilinogen levels. Indirect-bilirubin had sensitivity of 82%, specificity of 94%. Urobilinogen showed sensitivity of 70% and specificity of 92%. Conclusion: Indirect bilirubin and urine-urobilinogen is a valuable, cost-effective screening test for beta-thalassemia-trait with sensitivity and specificity comparable to RBC indices. [Int J Res Med Sci 2015; 3(3.000: 730-737

Ridham A. Khanderia

2015-06-01

184

Improved Hematopoietic Differentiation Efficiency of Gene-Corrected Beta-Thalassemia Induced Pluripotent Stem Cells by CRISPR/Cas9 System.  

Science.gov (United States)

The generation of beta-thalassemia (?-Thal) patient-specific induced pluripotent stem cells (iPSCs), subsequent homologous recombination-based gene correction of disease-causing mutations/deletions in the ?-globin gene (HBB), and their derived hematopoietic stem cell (HSC) transplantation offers an ideal therapeutic solution for treating this disease. However, the hematopoietic differentiation efficiency of gene-corrected ?-Thal iPSCs has not been well evaluated in the previous studies. In this study, we used the latest gene-editing tool, clustered regularly interspaced short palindromic repeats (CRISPR)/CRISPR-associated 9 (Cas9), to correct ?-Thal iPSCs; gene-corrected cells exhibit normal karyotypes and full pluripotency as human embryonic stem cells (hESCs) showed no off-targeting effects. Then, we evaluated the differentiation efficiency of the gene-corrected ?-Thal iPSCs. We found that during hematopoietic differentiation, gene-corrected ?-Thal iPSCs showed an increased embryoid body ratio and various hematopoietic progenitor cell percentages. More importantly, the gene-corrected ?-Thal iPSC lines restored HBB expression and reduced reactive oxygen species production compared with the uncorrected group. Our study suggested that hematopoietic differentiation efficiency of ?-Thal iPSCs was greatly improved once corrected by the CRISPR/Cas9 system, and the information gained from our study would greatly promote the clinical application of ?-Thal iPSC-derived HSCs in transplantation. PMID:25517294

Song, Bing; Fan, Yong; He, Wenyin; Zhu, Detu; Niu, Xiaohua; Wang, Ding; Ou, Zhanhui; Luo, Min; Sun, Xiaofang

2015-05-01

185

Integrative proteome and transcriptome analysis of extramedullary erythropoiesis and its reversal by transferrin treatment in a mouse model of Beta-thalassemia.  

Science.gov (United States)

Beta-thalassemia results from mutations of the ?-hemoglobin (Hbb) gene and reduced functional Hbb synthesis. Excess ?-Hb causes globin chain aggregation, oxidation, cytoskeletal damage, and increased red blood cell clearance. These events result in anemia, altered iron homeostasis, and expansion of extramedullary erythropoiesis. Serum transferrin (Tf) is suggested to be an important regulator of erythropoiesis in murine models of thalassemia. The present study was conducted to establish a quantitative proteomic and transcriptomic analysis of transferrin-modulated extramedullary erythropoiesis in the spleen of wild type and thalassemic Hbb(th3/+) mice. Our LC-MS/MS protein analysis and mRNA sequencing data provide quantitative expression estimates of 1590 proteins and 24?581 transcripts of the murine spleen and characterize key processes of erythropoiesis and RBC homeostasis such as the whole heme synthesis pathway as well as critical components of the red blood cell antioxidant systems and the proliferative cell cycling pathway. The data confirm that Tf treatment of nontransfused Hbb(th3/+) mice induces a systematic correction of these processes at a molecular level. Tf treatment of Hbb(th3/+) mice for 60 days leads to a complete molecular restoration of the normal murine spleen phenotype. These findings support further investigation of plasma-derived Tf as a treatment for thalassemia. PMID:25566950

Vallelian, Florence; Gelderman-Fuhrmann, Monique P; Schaer, Christian A; Puglia, Michele; Opitz, Lennart; Baek, Jin Hyen; Vostal, Jaroslav; Buehler, Paul W; Schaer, Dominik J

2015-02-01

186

Comparing prevalence of Iron Deficiency Anemia and Beta Thalassemia Trait in microcytic and non-microcytic blood donors: suggested algorithm for donor screening  

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Full Text Available Background: The prevalence of microcytosis in donors and Iron Deficiency Anemia (IDA and Beta-Thalassemia trait (BTT in microcytic and non-microcytic donors has not been studied in India. The present study aims at finding the same. Materials and Methods: Initially 925 donor samples were evaluated on cell-counter. Of these, 50 were found to be microcytic. These were subjected to Ferritin and HbA2 determination. Subsequently, an additional 51, age-and-sex matched non-microcytic donor samples were selected to serve as controls. These were subjected to the same tests. Results: The prevalence of microcytosis was 5.4% (50/925. Among the microcytic donors, 52% were IDA, 36% BTT, 8% both, and 4% none. In case of non-microcytic donors 29.4% were IDA, 3.9% BTT, and 66.7% none. Conclusions: The study revealed a high prevalence of IDA and BTT in blood donors and a higher probability of finding these in the microcytic samples. This prompted authors to suggest an algorithm for screening of blood donors for IDA and BTT. The algorithm recommends doing an hemogram on all donor samples, routinely. Ferritin could be done only in microcytic samples. At levels lower than15 ng/ml, it is diagnosed as IDA, and therefore, HPLC is performed only for non-IDA samples with Ferritin levels higher than 15 ng/ml. By employing this algorithm, a substantial number of IDA and BTT could be diagnosed while keeping the number of Ferritin tests small and the number of HPLC tests even smaller and thus making it cost efficient.

Tiwari Aseem

2009-01-01

187

Genotyping of Kell, Duffy, Kidd and RHD in patients with beta Thalassemia / Genotipagem dos sistemas Kell, Duffy, Kidd e RHD em pacientes com beta Talassemia  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: English Abstract in portuguese A determinação dos fenótipos Rh, Kell, Duffy e Kidd, associada ao ABO é utilizada para prevenir a aloimunização a antígenos eritrocitários e participam também no processo de identificação de anticorpos nos pacientes com beta talassemia. Todavia, a fenotipagem desses pacientes é trabalhosa e de difíc [...] il interpretação. Nesta situação, deve ser avaliada uma alternativa ao teste de hemaglutinação para determinar o padrão antigênico dos pacientes. Utilizamos para tal fim o método PCR-RFLP. Foram preparados DNAs de 50 pacientes com beta talassemia que haviam sido anteriormente fenotipados pela hamglutinação e testados para Kell, Kidd, Duffy/GATA mutação por PCR-RFLP. RHD/não-D foi analisado pelo tamanho do produto, do PCR associado à seqüência do gene RHD no intron 4 e exon 10/3' UTR. Os testes de genotipagem foram realizados sem o conhecimento dos resultados dos fenótipos. Para os RHD/não-D, 47 foram RhD+ e RHD+/RHCE+, e 3 foram RhD- e RHD-/RHCE+. Para o Kell, 48 kk foram K2K2 e 2 Kk foram K1K2. Para o Duffy, das 44 amostras que haviam sido normais, GATA box, 8 Fy(a+b-) foram FYA/FYA, 15 Fy(a+b-) foram FYB/FYB e 19 Fy(a+b+) foram FYA/FYB; das outras 4 amostras, 3 foram FYA/FYB e heterozigoto GATA mutação, e 1 Fy(a-b-) era FYB/FYB, homozigoto GATA mutação. Duas amostras fenotipadas como Fy(a+b-), que eram normais GATA, apresentavam as mutações 265T/298A e 2 amostras fenotipadas como Fy(a-b+) haviam sido genotipadas como FYA/FYB. Para o Kidd, 15 Jk(a+b-) foram JKA/JKA, 12 Jk(a-b+) foram JKB/JKB, e 20 Jk(a+b+) foram JKA/JKB. Três amostras fenotipadas como JK(a+b+) haviam sido genotipadas como JKB/JKB. A genotipagem é mais acurada que a fenotipagem para determinação de grupos sangüíneos em pacientes portadores de beta talassemia politransfundidos. A genotipagem nesses pacientes pode ser importante para selecionar hemácias antigenicamente negativas para transfusão de glóbulos vermelhos. Abstract in english Determination of Rh, Kell, Duffy and Kidd phenotypes in addition to ABO is used to prevent the alloimmunization to red blood cells (RBCs) antigens and as part of the antibody identification process in patients with beta Thalassemia. However, phenotyping in these patients can be time consuming and di [...] fficult to interpret. In these situations, it would be valuable to have an alternative to hemagglutination tests to determine the patient's antigen profile. We used PCR-RFLP to genotype such patients. DNA was prepared from 50 patients with beta Thalassemia who had been phenotyped by routine hemagglutination, and tested for Kell, Kidd, Duffy/GATA mutation by PCR-RFLP. RHD/non-D was analysed by PCR product size associated to RHD gene sequence in intron 4 and exon 10/3'UTR. The genotyping assays were performed without knowledge of phenotype results. For RHD/non-D, 47 were RhD+ and RHD+/RHCE+, and 3 were RhD- and RHD-/RHCE+. For Kell, 48 kk were K2K2 and 2 Kk were K1K2. For Duffy, of 44 samples that had normal GATA box, 8 Fy(a+b-) were FYA/FYA, 15 Fy(a+b+) were FYB/FYB, and 19 Fy(a+b+) were FYA/FYB; of the other 4 samples 3 were FYA/FYB and heterozygous GATA mutation, and 1 Fy(a-b-) was FYB/FYB, homozygous GATA mutation. Two samples phenotyped as Fy(a+b-) that had normal GATA , presented the 265T/298A mutations and two samples phenotyped as Fy(a-b+) were genotyped was FYA/FYB.. For Kidd , 15 Jk(a+b) were JKA/JKA, 12 Jk(a-b+) were JKB/JKB, and 20 Jk(a+b+) were JKA/JKB. Three samples phenotyped as JK(a+b+) were genotyped as JKB/JKB. Genotype is more accurate than phenotype for determination of blood groups in polytransfused patients with betaThalassemia. Genotyping in these patients can be helpful to select antigen-negative RBCs for transfusion.

Lilian, Castilho; Maria, Rios; Jordão, Pellegrino Jr; Maria H. M., Carvalho; Fernando L., Alberto; Sara T. O., Saad; Fernando F., Costa.

2000-08-01

188

The pancreas in ?-thalassemia major: MR imaging features and correlation with iron stores and glucose disturbunces  

International Nuclear Information System (INIS)

The study aims at describing the MR features of pancreas in beta-thalassemia major, investigating the relations between MR findings and glucose disturbances and between hepatic and pancreatic siderosis. Signal intensity ratios of the pancreas and liver to right paraspinous muscle (P/M, L/M) were retrospectively assessed on abdominal MR imaging studies of 31 transfusion-dependent patients with beta-thalassemia major undergoing quantification of hepatic siderosis and 10 healthy controls, using T1- (120/4/90), intermediate in and out of phase - (120/2.7, 4/20), and T2*-(120/15/20) weighted GRE sequences. Using the signal drop of the liver and pancreas on opposed phase images, we recorded serum ferritin and results of oral glucose tolerance test (OGTT). Decreased L/M and P/M on at least the T2* sequence were noticed in 31/31 and 30/31 patients, respectively, but no correlation between P/M and L/M was found. Patients with pathologic OGTT displayed a higher degree of hepatic siderosis (p < 0.04) and signal drop of pancreas on opposed phase imaging (p < 0.025), implying fatty replacement of pancreas. P/M was neither correlated with glucose disturbances nor serum ferritin. Iron deposition in the pancreas cannot be predicted by the degree of hepatic siderosis in beta-thalassemia major. Fatty replacement of the pancreas is common and may be associated with glucose disturbances. (orig.)

189

Percutaneous excretion of iron and ferritin (through Al-hijamah) as a novel treatment for iron overload in beta-thalassemia major, hemochromatosis and sideroblastic anemia.  

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Iron overload is a big challenge when treating thalassemia (TM), hemochromatosis and sideroblastic anemia. It persists even after cure of TM with bone marrow transplantation. Iron overload results from increased iron absorption and repeated blood transfusions causing increased iron in plasma and interstitial fluids. Iron deposition in tissues e.g. heart, liver, endocrine glands and others leads to tissue damage and organ dysfunction. Iron chelation therapy and phlebotomy for iron overload have treatment difficulties, side effects and contraindications. As mean iron level in skin of TM patients increases by more than 200%, percutaneous iron excretion may be beneficial. Wet cupping therapy (WCT) is a simple, safe and economic treatment. WCT is a familiar treatment modality in some European countries and in Chinese hospitals in treating different diseases. WCT was reported to clear both blood plasma and interstitial spaces from causative pathological substances (CPS). Standard WCT method is Al-hijamah (cupping, puncturing and cupping, CPC) method of WCT that was reported to clear blood and interstitial fluids better than the traditional WCT (puncturing and cupping method, PC method of WCT). In other word, traditional WCT may be described as scarification and suction method (double S technique), while Al-hijamah may be described as suction, scarification and suction method (triple S technique). Al-hijamah is a more comprehensive treatment modality that includes all steps and therapeutic benefits of traditional dry cupping therapy and WCT altogether according to the evidence-based Taibah mechanism (Taibah theory). During the first cupping step of Al-hijamah, a fluid mixture is collected inside skin uplifting due to the effect of negative pressure inside sucking cups. This fluid mixture contains collected interstitial fluids with CPS (iron, ferritin and hemolyzed RBCs in thalassemia), filtered fluids (from blood capillaries) with iron and hemolyzed blood cells (hemolyzed RBCs, WBCs and platelets). That fluid mixture does not contain intact blood cells (having diameters in microns) that are too big to pass through pores of skin capillaries (6-12nm in diameter) and cannot be filtered. Puncturing skin upliftings and applying second cupping step excrete collected fluids. Skin scarifications (shartat mihjam in Arabic) should be small, superficial (0.1mm in depth), short (1-2mm in length), multiple, evenly distributed and confined to skin upliftings. Sucking pressure inside cups (-150 to -420mmHg) applied to skin is transmitted to around skin capillaries to be added to capillary hydrostatic pressure (-33mmHg at arterial end of capillaries and -13mmHg at venous end of capillaries) against capillary osmotic pressure (+20mmHg). This creates a pressure gradient and a traction force across skin and capillaries and increases filtration at arterial end of capillaries at net pressure of -163 to -433mmHg and at venous end of capillaries at net pressure of -143 to -413mmHg resulting in clearance of blood from CPS (iron, ferritin and hemolyzed blood cells). Net filtration pressure at renal glomeruli is 10mmHg i.e. Al-hijamah exerts a more pressure-dependent filtration than renal glomeruli. Al-hijamah may benefit patients through inducing negative iron balance. Interestingly, Al-hijamah was reported to decrease serum ferritin significantly (by about 22%) in healthy subjects while excessive traditional WCT was reported to cause iron deficiency anemia. Al-hijamah is a highly recommended treatment in prophetic medicine. In conclusion, Al-hijamah may be a promising adjuvant treatment for iron overload in TM, hemochromatosis and sideroblastic anemia. PMID:24857772

El Sayed, Salah Mohamed; Abou-Taleb, Ashraf; Mahmoud, Hany Salah; Baghdadi, Hussam; Maria, Reham A; Ahmed, Nagwa Sayed; Nabo, Manal Mohamed Helmy

2014-08-01

190

MRI of the liver and the pituitary gland in patients with {beta}-thalassemia major: Does hepatic siderosis predict pituitary iron deposition?  

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Our objective was to study, in thalassemic patients, if hepatic siderosis evaluated by MRI could predict the pituitary iron overload. In 36 thalassemic patients (age range 6-44 years, mean age 21.7 years) the liver/fat ratio (L/F), the pituitary/fat ratio (P/F), the liver and pituitary T2 relaxation times were evaluated, by using a multiecho spin-echo sequence. Serum ferritin levels were measured and an extensive endocrine evaluation was performed. The L/F, the P/F and pituitary T2 showed a good correlation with serum ferritin (r=-0.55, r=-0.55 and r=-0.53, respectively; p<0.01). Liver T2 did not show significant correlation with serum ferritin. The variability of L/F explained only the 10.8% of the variability of pituitary T2 and of the P/F. When ferritin was added to the model it predicted only the 26.85% and the 30.8% of the variability of pituitary T2 and of the P/F, respectively. The P/F and pituitary T2 were lower in patients with hypogonadotropic hypogonadism (group 1) compared with those without pituitary dysfunction (group 2). No significant differences of L/F were found between the two groups. Hepatic iron overload evaluated by MR is a poor predictor of pituitary siderosis. The MR studies of the pituitary gland might be necessary to evaluate the pituitary iron overload. (orig.)

Argyropoulou, Maria I.; Efremidis, Stavros C. [Department of Radiology, Medical School, University of Ioannina, 45110 Ioannina (Greece); Kiortsis, Dimitrios N. [Laboratory of Physiology, Medical School, University of Ioannina, 45110 Ioannina (Greece)

2003-01-01

191

MRI of the liver and the pituitary gland in patients with beta-thalassemia major: does hepatic siderosis predict pituitary iron deposition?  

Science.gov (United States)

Our objective was to study, in thalassemic patients, if hepatic siderosis evaluated by MRI could predict the pituitary iron overload. In 36 thalassemic patients (age range 6-44 years, mean age 21.7 years) the liver/fat ratio (L/F), the pituitary/fat ratio (P/F), the liver and pituitary T2 relaxation times were evaluated, by using a multiecho spin-echo sequence. Serum ferritin levels were measured and an extensive endocrine evaluation was performed. The L/F, the P/F and pituitary T2 showed a good correlation with serum ferritin ( r=-0.55, r=-0.55 and r=-0.53, respectively; ppituitary T2 and of the P/F. When ferritin was added to the model it predicted only the 26.85% and the 30.8% of the variability of pituitary T2 and of the P/F, respectively. The P/F and pituitary T2 were lower in patients with hypogonadotropic hypogonadism (group 1) compared with those without pituitary dysfunction (group 2). No significant differences of L/F were found between the two groups. Hepatic iron overload evaluated by MR is a poor predictor of pituitary siderosis. The MR studies of the pituitary gland might be necessary to evaluate the pituitary iron overload. PMID:12541105

Argyropoulou, Maria I; Kiortsis, Dimitrios N; Efremidis, Stavros C

2003-01-01

192

Egyptian Antiquity Information  

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The Egyptian Ministry of Tourism provides an excellent collection of resources useful to both student and scholar. The site provides a number of sections covering topics such as Monuments (pharoanic, Islamic, and early Christian), Rulers of Ancient Egypt, Egyptian Mythology, a modest Virtual Museum, and a lengthy Glossary of Terms. The highlight is an extensive History of Egypt from the Lower Paleolithic Age to 1942. The history is divided into dynasties/historical periods and contains numerous hyperlinks. Additional features include a section on Historical Astrology in Egypt and the complete Egyptian Book of the Dead.

193

Egyptian Arab Republic  

International Nuclear Information System (INIS)

In this work are given the principal news concerning petroleum and natural gas in Egyptian Arab Republic. An important discovery of natural gas has been made in Khalda (Egyptian Arab Republic). The discovery well will be temporarily abandoned until it is connected to the egyptian pipeline system. In 1996 the south Khalda will be explored with at least two well drilling. The transit duties by the Suez canal for liquefied natural gas exports have decreased of 35%. The Arab Petroleum Pipeline Company studies a connection project of the trans saudi pipeline with the Suez mediterranean pipeline. The Egyptian General Petroleum Corporation will furnish 2,5 milliards of m3 per year of natural gas to Israel during 20 years. (O.L.). 2 figs

194

Total Antioxidant Status in Patients with Major ? -Thalassemia  

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Full Text Available Objective:Beta-thalassemia major is an autosomal recessive disease causing severe and hemolytic anemia, which begins about 2-6 months after birth. Iron overload, which arises from recurrent transfusion and ineffective erythropoiesis, can enhance oxidative stress in thalassemic patients. The aim of this study wasto evaluate theserum total antioxidant capacity of patients with ß-Thalassemia major.Methods:Sixty six Iranian patients with ?-thalassemia major and 66 age-gender matched controls were evaluated for serum total antioxidant status (TAS, uric acid (UA, bilirubin and albumin. In addition, serum ferritin and transaminases were recorded in these subjects.Findings: Significant increases of TAS, UA, and bilirubin were observed in the patient group, compared with the control group (P< 0.01. Mean TAS and bilirubin in male patients was higher than in females (P=0.005 and P=0.008, respectively. There was also direct correlation between TAS and albumin (P< 0.001, bilirubin (P< 0.001 and UA (P=0.002.Conclusion: Endogenous antioxidants such as ferritin, UA and bilirubin can result in increased level of TAS in the patients with Beta-thalassemia major. Compensatory excess of TAS to oxidative stress could also be the reason for difference between our findings and previous studies.

Fatemeh Bazvand

2011-06-01

195

Total Antioxidant Status in Patients with Major ?-Thalassemia  

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Full Text Available Objective:Beta-thalassemia major is an autosomal recessive disease causing severe and hemolytic anemia, which begins about 2-6 months after birth. Iron overload, which arises from recurrent transfusion and ineffective erythropoiesis, can enhance oxidative stress in thalassemic patients. The aim of this study was to evaluate the serum total antioxidant capacity of patients with ß-Thalassemia major. Methods:Sixty six Iranian patients with ?-thalassemia major and 66 age-gender matched controls were evaluated for serum total antioxidant status (TAS, uric acid (UA, bilirubin and albumin. In addition, serum ferritin and transaminases were recorded in these subjects. Findings: Significant increases of TAS, UA, and bilirubin were observed in the patient group, compared with the control group (P<0.01. Mean TAS and bilirubin in male patients was higher than in females (P=0.005 and P=0.008, respectively. There was also direct correlation between TAS and albumin (P<0.001, bilirubin (P<0.001 and UA (P=0.002. Conclusion: Endogenous antioxidants such as ferritin, UA and bilirubin can result in increased level of TAS in the patients with Beta-thalassemia major. Compensatory excess of TAS to oxidative stress could also be the reason for difference between our findings and previous studies.

Lili Koochakzadeh

2011-06-01

196

The identification of beta-thalassemia mutants in Brazilians with high Hb F levels / Identificação de mutantes de beta talassemia em grupo de indivíduos com Hb Fetal aumentada da população brasileira  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: English Abstract in portuguese As hemoglobinopatias são um grupo de afecções genéticas que representam problema de saúde pública em muitos países em que sua incidência é alta, com significativa morbidade. Objetivamos identificar defeitos moleculares que pudessem explicar o perfil laboratorial obtido por eletroforese e HPLC com Hb [...] F elevada, em um grupo de indivíduos adultos sem sinais ou sintomas de anemia. Encontramos cinco diferentes mutações que originam beta talassemia por PCR-ASO: três casos com CD 6 (-A), um CD 39, um IVS 1-5, um -87 todas de origem mediterrânea, e um IVS II-654 de origem asiática. As mutações CD 6 (-A), -87 e IVS II-654 foram descritas pela primeira vez na população brasileira. Abstract in english Hemoglobinopathies are a heterogeneous group of genetic disorders which represent a public health problem, with significant morbidity, in countries where the prevalence is high. This study aimed at identifying molecular abnormalities that might explain the laboratorial profile obtained using electro [...] phoresis and high performance liquid chromatography in a group of individuals without signs or clinical symptoms of anemia. Five different mutations for beta-thalassemia were found using PCR-ASO: three cases with CD 6 (-A), one CD 39, one IVI I-6, one -87 (mutations originating in the Mediterranean region) and one IVS II-654 (mutation originating in Asia). This is the first time that the CD 6 (-A), -87 and IVS II-654 mutations have been described in the Brazilian population.

Paula J. A., Zamaro; Claudia R., Bonini-Domingos.

197

Multiple transfused thalassemia major: Ocular manifestations in a hospital-based population  

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Full Text Available Purpose: To study the ocular manifestations in multiple transfused beta-thalassemia major patients and assess the ocular side-effects of iron chelating agents. Materials and Methods: In this prospective observational study, 45 multiple transfused beta-thalassemia major children between six months and 21 years of age were enrolled and assigned groups according to the treatment regimens suggested. Group A received only blood transfusions, Group B blood transfusions with subcutaneous desferrioxamine, Group C blood transfusions with desferrioxamine and oral deferriprone and Group D blood transfusions with deferriprone. Ocular status at the time of enrolment was documented. Subjects were observed quarterly for one year for changes in ocular status arising due to the disease process and due to iron chelation therapy. Children with hemoglobinopathies other than beta-thalassemia major, congenital ocular anomalies and anemia due to other causes were excluded. Results: Ocular involvement was observed in 58% of patients. Lenticular opacities were the most common ocular finding (44%, followed by decreased visual acuity (33%. An increased occurrence of ocular changes was observed with increase of serum ferritin and serum iron levels as well as with higher number of blood transfusions received. Desferrioxamine seemed to have a protective influence on retinal pigment epithelium (RPE mottling. Occurrence of lenticular opacities and RPE degeneration correlated positively with use of desferrioxamine and deferriprone respectively. Follow-up of patients for one year did not reveal any change in ocular status. Conclusion: Regular ocular examinations can aid in preventing, delaying or ameliorating the ocular complications of thalassemia.

Taneja Rashi

2010-01-01

198

Sit Like an Egyptian  

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The topic of Egypt is one that students are naturally intrigued and enthusiastic about. In this article, fifth graders create mosaic and mixed-media collaged chairs in their visual arts class as part of their overall study of the art and culture of ancient Egypt. The idea was to embellish a contemporary chair with Egyptian colors, themes, and…

Moll, Emily

2012-01-01

199

Migration of Egyptians.  

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This study is concerned with recent trends in emigration from Egypt, with particular emphasis on the migration of Egyptians to the Arab oil-exporting countries of the Middle East since 1973. The benefits and costs of this migration for Egypt are considered. PMID:12279539

Oweiss, I M

1980-07-01

200

Interação entre Hb C [beta6(A3Glu>Lys] e IVS II-654 (C>T beta-talassemia no Brasil Hb C [beta6(A3Glu>Lys] and IVS II - 654 (C>T beta thalassemia interaction in Brazil  

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Full Text Available Thalassemias are a heterogeneous group of inherited disorders characterized by a microcytic hypochromic anemia and an imbalance in the synthesis of the globin-chains. Hb C is the second most frequently variant of hemoglobin found in Brazil. The laboratory diagnosis of hemoglobinopathies, including thalassemias, is growing in importance, particularly because of an increasing requirement for neonatal diagnosis of abnormal hemoglobins. Screening tests were carried out using alkaline and acid electrophoresis, globin-chain analysis by cellulose acetate in alkaline pH, isoelectric focusing and HPLC. The molecular characterization was made by PCR-ASO for Hb C and beta thalassemia mutants. Large-scale screening and discriminative methodologies must provide information about the hemoglobin polymorphisms in Brazilian population. HPLC is a powerful tool in these cases. Molecular characterization is important to genetic counseling and clinical management, in particular for the Brazilian population that have an intense racial admixture, with great variability of hemoglobins. In this paper an association between Hb C and beta thalassemia (IVS-II-654 in a black family from Brazil was described.

Claudia R. Bonini-Domingos

2003-06-01

201

Interação entre Hb C [beta6(A3)Glu>Lys] e IVS II-654 (C>T) beta-talassemia no Brasil / Hb C [beta6(A3)Glu>Lys] and IVS II - 654 (C>T) beta thalassemia interaction in Brazil  

Scientific Electronic Library Online (English)

Full Text Available [...] Abstract in english Thalassemias are a heterogeneous group of inherited disorders characterized by a microcytic hypochromic anemia and an imbalance in the synthesis of the globin-chains. Hb C is the second most frequently variant of hemoglobin found in Brazil. The laboratory diagnosis of hemoglobinopathies, including t [...] halassemias, is growing in importance, particularly because of an increasing requirement for neonatal diagnosis of abnormal hemoglobins. Screening tests were carried out using alkaline and acid electrophoresis, globin-chain analysis by cellulose acetate in alkaline pH, isoelectric focusing and HPLC. The molecular characterization was made by PCR-ASO for Hb C and beta thalassemia mutants. Large-scale screening and discriminative methodologies must provide information about the hemoglobin polymorphisms in Brazilian population. HPLC is a powerful tool in these cases. Molecular characterization is important to genetic counseling and clinical management, in particular for the Brazilian population that have an intense racial admixture, with great variability of hemoglobins. In this paper an association between Hb C and beta thalassemia (IVS-II-654) in a black family from Brazil was described.

Claudia R., Bonini-Domingos; Ana C., Bonini-Domingos; Ana R., Chinelato; Paula J. A., Zamaro; Patrícia H. O., Calderan.

2003-06-01

202

Growth Parameters and Vitamin D status in Children with Thalassemia Major in Upper Egypt  

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Aim The aim of this study is to assess the growth parameters, vitamin D, calcium, and phosphorous status in children with thalassemia major receiving packed red cells transfusion with chelation therapy. Patients and Methods In a case control study, 100 patients with beta thalassemia major (aged from 4 to 15 years) were compared with 100 sex- and age-matched children serves as a control group. Anthropometric measurement, Serum level of calcium, phosphorus and vitamin D (25 hydroxycholecalciferol) were estimated for all patients & controls. Results 49% of our patients had short stature. 47% were underweight. BMI of 43 (43%) patients were low. The mean total serum calcium (6.6±1.2 mg/dl) and 25-hydroxycholecalciferol (25-OH Vit D) (10.4±4.6 mcg/dl) levels were significantly lower in our patients than in controls (10.2±1.06 mg/dl and 40.2±12.3 mcg/dl, respectively); each P< 0.001. Conclusion Children with beta thalassemia major have delayed growth and metabolic abnormalities that signify the importance of therapeutic interventions. The presence of these abnormalities may be due to iron overload and poor nutritional support. PMID:24505537

Fahim, Fahim M.; Saad, Khaled; Askar, Eman A.; Eldin, Eman Nasr; Thabet, Ahmed F.

2013-01-01

203

Fascinating Egyptian Mummies  

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Turn up those speakers, and listen to the sounds of eerie, blowing winds in the desert and some woeful Egyptian music. This website starts off by using a multimedia approach to lure visitors into the Mus'e de la civilisation's Fascinating Egyptian Mummies online and museum exhibit. The music follows visitors once they choose the link "Head Into The Tomb". A bouncing yellow arrow all but insists visitors choose the link to "Mummification Process Exhibition". Once the arrow is clicked, visitors hear some menacing Egyptian music and are asked if they want to "Play" a game that tests their skill at determining the steps in the mummification process, or they can choose to skip the game to go directly to the exhibition. The information dispensed here includes the observation that the mummification process took 70 days to complete, and information on some famous mummies is given as well. The next game is about determining which organ goes in which Canopic jar. It's not as easy as it sounds. The final game concerns the weighing of the heart, which visitors can choose to undergo only if they dare.

204

Serum ferritin levels, socio-demographic factors and desferrioxamine therapy in multi-transfused thalassemia major patients at a government tertiary care hospital of Karachi, Pakistan  

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Full Text Available Abstract Background Beta thalassemia is the most frequent genetic disorder of haemoglobin synthesis in Pakistan. Recurrent transfusions lead to iron-overload manifested by increased serum Ferritin levels, for which chelation therapy is required. Findings The study was conducted in the Pediatric Emergency unit of Civil Hospital Karachi after ethical approval by the Institutional Review Board of Dow University of Health Sciences. Seventy nine cases of beta thalassemia major were included after a written consent. The care takers were interviewed for the socio-demographic variables and the use of Desferrioxamine therapy, after which a blood sample was drawn to assess the serum Ferritin level. SPSS 15.0 was employed for data entry and analysis. Of the seventy-nine patients included in the study, 46 (58.2% were males while 33 (41.8% were females. The mean age was 10.8 (± 4.5 years with the dominant age group (46.2% being 10 to 14 years. In 62 (78.8% cases, the care taker education was below the tenth grade. The mean serum Ferritin level in our study were 4236.5 ng/ml and showed a directly proportional relationship with age. Desferrioxamine was used by patients in 46 (58.2% cases with monthly house hold income significant factor to the use of therapy. Conclusions The mean serum Ferritin levels are approximately ten times higher than the normal recommended levels for normal individuals, with two-fifths of the patients not receiving iron chelation therapy at all. Use of iron chelation therapy and titrating the dose according to the need can significantly lower the iron load reducing the risk of iron-overload related complications leading to a better quality of life and improving survival in Pakistani beta thalassemia major patients. Conflicts of Interest: None

Rehman Anis

2011-08-01

205

5.NF Egyptian Fractions  

Science.gov (United States)

This is a task from the Illustrative Mathematics website that is one part of a complete illustration of the standard to which it is aligned. Each task has at least one solution and some commentary that addresses important asects of the task and its potential use. Here are the first few lines of the commentary for this task: Ancient Egyptians used unit fractions, such as $\\frac{1}{2}$ and $\\frac{1}{3}$, to represent all fractions. For example, they might write the number $\\...

206

Daily Egyptian Diversity News Index  

Science.gov (United States)

Developed as part of the online collections at Southern Illinois University-Carbondale's Morris Library, the Daily Egyptian Diversity News Index provides historical insight into the campus climate at this unique school. In 2006, Dr. Seymour Bryson, the associate chancellor for diversity, teamed up with several other colleagues to identify articles in the Daily Egyptian (the University's student newspaper) related to the university's historic minority campus populations. The project entailed surveying microfilm and creating searchable transcripts for online access. Currently, the online archive contains over 1,400 items from the Daily Egyptian, and content includes pieces on African American members of the homecoming court, student activists, musical groups, and student government.

207

Graves' disease mimicking beta-thalassemia trait.  

OpenAIRE

A case of Graves' disease associated with splenomegaly, lymphadenopathy, microcytic hypochromic anaemia, and a raised haemoglobin A2 is presented. The haematological indices returned to normal after conventional treatment with anti-thyroid medication.

Akasheh, M. S.

1994-01-01

208

Serum immunoglobulins in homozygous beta-thalassemia.  

OpenAIRE

IgG, IgA and IgM levels were studied in 187 homozygous beta-thalassemic patients and compared with age-matched normal control subjects. The not yet transfused and the polytransfused nonsplenectomized patients showed a significant increase of all Ig classes. The polytransfused splenectomized patients showed a significant increase only of IgG and IgA. The splenectomized patients, when compared to the nonsplenectomized ones, showed a significant increase of IgG, of IgA in the elder ones and a si...

Tovo, Pier Angelo; Barbera, Cristiana; Sacchetti, Laura

1981-01-01

209

The Egyptian Adventure  

Science.gov (United States)

This project is an integrated study for 8-9 year olds encompassing reading, geography, history, math, and science. The literature groups are reading the following fictional books: Ms. Frizzle\\'s Adventures Ancient Egypt by Cole and Degen, Tut, Tut by Jon Scieska, A to Z Mystery - Mummy Mystery by Ron Roy, and Jigsaw Jones - The Case of the Missing Mummy by James Preller. Various non-fiction resources are also being used by the students: Eyewitness Books - Secret of the Mummies, Mummies, Tombs, and Treasure by Lila Perl, and Mummies by C. Wilcox. These essential questions were created by third grade students: How did the rule of pharoahs begin? Who were important pharoahs? What are some facts about pyramids? Ancient Egypt (British Museum) Ancient Egypt Egypt What were the religious beliefs of the ancient Egyptians? Ancient Egypt (British Museum) Ancient Egypt Egypt What are some facts about the Nile River? Why the ...

Ms. LAi

2007-02-08

210

The Petrie Museum of Egyptian Archaeology, UCL  

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Full Text Available University College London houses one of the world’s most important collections of ancient Egyptian material, the majority excavated by Flinders Petrie, his students and his successors in the late 19th and early 20th centuries. It is a museum of archaeology that helps to explain the development of a discipline that was in its infancy when Petrie worked in Egypt over a century ago. It is a teaching collection, its densely packed cases entrancing, and sometimes intimidating, visitors who rave about its old-fashioned feel, but it is anything but frozen in time.

Jan Picton

2013-10-01

211

Unexpected links between Egyptian and Babylonian mathematics  

CERN Document Server

Mesopotamian mathematics is known from a great number of cuneiform texts, most of them Old Babylonian, some Late Babylonian or pre-Old-Babylonian, and has been intensively studied during the last couple of decades. In contrast to this Egyptian mathematics is known from only a small number of papyrus texts, and the few books and papers that have been written about Egyptian mathematical papyri have mostly reiterated the same old presentations and interpretations of the texts. In this book, it is shown that the methods developed by the author for the close study of mathematical cuneiform texts can also be successfully applied to all kinds of Egyptian mathematical texts, hieratic, demotic, or Greek-Egyptian. At the same time, comparisons of a large number of individual Egyptian mathematical exercises with Babylonian parallels yield many new insights into the nature of Egyptian mathematics and show that Egyptian and Babylonian mathematics display greater similarities than expected.

Friberg, Jöran

2005-01-01

212

Soggy Soil Sours Egyptian Sandstones  

Science.gov (United States)

This article from Geotimes examines the threat posed by rising groundwater to ancient Egyptian monuments. The problem has several sources, including the rise of Egypt's water table over the last 30 years, the prevention of freshwater floods through the cities where the monuments stand, and leaking or non-existing sewage systems. Various solutions are also examined.

Christina Reed

213

The northern Egyptian continental margin  

Science.gov (United States)

Africa displays a variety of continental margin structures, tectonics and sedimentary records. The northern Egyptian continental margin represents the NE portion of the North African passive continental margin. Economically, this region is of great importance as a very rich and productive hydrocarbon zone in Egypt. Moreover, it is characterized by remarkable tectonic setting accompanied by active tectonic processes from the old Tethys to recent Mediterranean. In this article, seismicity of the northern Egyptian continental margin has been re-evaluated for more than 100-years and the source parameters of three recent earthquakes (October 2012, January 2013 and July 2013) have been estimated. Moment tensor inversions of 19th October 2012 and 17th January 2013 earthquakes reveal normal faulting mechanism with strike-slip component having seismic moment of 3.5E16 N m and 4.3E15 N m respectively. The operation of the Egyptian National Seismic Network (ENSN) since the end of 1997 has significantly enhanced the old picture of earthquake activity across northern Egyptian continental margin whereas; the record-ability (annual rate) has changed from 2-events/year to 54-event/year before and after ENSN respectively. The spatial distribution of earthquakes foci indicated that the activity tends to cluster at three zones: Mediterranean Ridge (MR), Nile Cone (NC) and Eratosthenes Seamount (ERS). However, two seismic gaps are reported along Levant Basin (LEV) and Herodotus Basin (HER).

Badawy, Ahmed; Mohamed, Gad; Omar, Khaled; Farid, Walid

2015-01-01

214

Transnational NGOs and Egyptian Authoritarianism  

DEFF Research Database (Denmark)

Among the countries of the “Arab spring” in the Middle East and North Africa only Egypt claims both a popular revolution and a coup. With a civic revolt in 2011 Egyptians mobilized resources to overthrow an authoritarian ruler. Two years later with a military coup the public fragmented into antagonistic factions: (i) those continuously insisting “shariyah”, the legality of the original revolution of the 25th January 2011; and (ii) proponents of the “inqilab”, the army takeover of July 3rd 2013. This paper explores the role of transnational NGOs (TNGOs)1 in this unresolved political stalemate. With nationwide mass protests in January 2011 Egyptians demanded “rahil”, the departure of Hosni Mubarak, the country’s long term autocratic ruler. Protesters called for “Eish” bread (Life) “karama” dignity and “huriya” freedom for all. These euphoric public eruptions marked the culmination of long term entrenched civic engagement. Over the years Egyptian activists established grassroots critical social movements and organizations to directly and indirectly challenge the military regime.

Farah, Abdulkadir Osman

2014-01-01

215

Mineralogy, geochemistry and radioactivity of some Egyptian phosphorite deposits  

International Nuclear Information System (INIS)

Mineralogical, geochemical, and radiogeological studies of forty-nine phosphorite samples from three main mining areas in Egypt, Abu Tartur (Western Desert), Sibaiya (Nile Valley), and Safaga (Red Sea) have been carried out. X-ray diffraction studies of these Egyptian phosphorites show that carbonate-fluorapatite (francolite) is the major phosphorite mineral in these deposits, with partial substitution of Mg and Na for Ca, and (CO3) for (PO4). Other minerals identified using the x-ray diffraction and scanning electron microscope include calcite, dolomite, pyrite, quartz, gypsum, feldspars, micas and clay (smectite). The uranium content and U/P2O5 ratio show low values in Abu Tartur phosphorite deposits relative to the Red Sea and Nile Valley deposits. Autoradiographic studies of the Egyptian phosphorites showed that the radioactivity is unequally distributed among its constituents. The opaque pellets are the most radioactive and the bone fragments are the least radioactive. Geochemically, four distinct groups of minerals were identified in the Egyptian phosphorites according to the degree of correlation between the major apatite components and the trace elements. These mineral groups are apatite, clays, heavy minerals and leached group. The results showed that the phosphorites of Nile Valley are relatively rich in Co and Zn whereas the Red Sea phosphorites are relatively rich in Pb and U. The Western Desert phosphorites are nd U. The Western Desert phosphorites are much richer in Ti as compared to the other two areas. Relative to the world phosphorites, the Egyptian phosphorites are geochemically enriched in Co, Mn, and Ni and impoverished in Sr, Cr, U, Zn, Cu, Ti, and V

216

Mineralogy, geochemistry and radioactivity of some Egyptian phosphorite deposits  

Energy Technology Data Exchange (ETDEWEB)

Mineralogical, geochemical, and radiogeological studies of forty-nine phosphorite samples from three main mining areas in Egypt, Abu Tartur (Western Desert), Sibaiya (Nile Valley), and Safaga (Red Sea) have been carried out. X-ray diffraction studies of these Egyptian phosphorites show that carbonate-fluorapatite (francolite) is the major phosphorite mineral in these deposits, with partial substitution of Mg and Na for Ca, and (CO/sub 3/) for (PO/sub 4/). Other minerals identified using the x-ray diffraction and scanning electron microscope include calcite, dolomite, pyrite, quartz, gypsum, feldspars, micas and clay (smectite). The uranium content and U/P/sub 2/O/sub 5/ ratio show low values in Abu Tartur phosphorite deposits relative to the Red Sea and Nile Valley deposits. Autoradiographic studies of the Egyptian phosphorites showed that the radioactivity is unequally distributed among its constituents. The opaque pellets are the most radioactive and the bone fragments are the least radioactive. Geochemically, four distinct groups of minerals were identified in the Egyptian phosphorites according to the degree of correlation between the major apatite components and the trace elements. These mineral groups are apatite, clays, heavy minerals and leached group. The results showed that the phosphorites of Nile Valley are relatively rich in Co and Zn whereas the Red Sea phosphorites are relatively rich in Pb and U. The Western Desert phosphorites are much richer in Ti as compared to the other two areas. Relative to the world phosphorites, the Egyptian phosphorites are geochemically enriched in Co, Mn, and Ni and impoverished in Sr, Cr, U, Zn, Cu, Ti, and V.

Dabous, A.A.A.

1981-01-01

217

Study on effectiveness of transfusion program in thalassemia major patients receiving multiple blood transfusions at a transfusion centre in Western India  

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Full Text Available Background : Children suffering from beta-thalassemia major require repeated blood transfusions which may be associated with dangers like iron overload and contraction of infections such as HIV, HCV, and HBsAg which ultimately curtail their life span. On the other hand, inadequate transfusions lead to severe anemia and general fatigue and debility. Materials and Methods: Data were obtained from 142 beta-thalassemia major patients aged 3 years or more receiving regular blood transfusions at a transfusion centre in Western India from 1 April 2009 to 30 June 2009. The clinical data and laboratory results were subsequently analyzed. Results: Of the 142 patients, 76 (53.5% were undertransfused (mean Hb <10 gm%. 96 (67% of the patients were taking some form of chelation therapy but out of them only 2 (2% were adequately chelated (S. ferritin <1000 ng/ml. 5 (3.5% of the patients were known diabetics on insulin therapy. 103 (72% of the patients were retarded in terms of growth. The prevalence of transfusion-transmitted infections (TTIs such as HCV, HIV, and HBsAg was respectively 45%, 2%, and 2%, with the prevalence of HCV being significantly more than the general population. The HCV prevalence showed positive correlation with the age of the patients and with the total no of blood transfusions received. As many as 15% (6 out of 40 children who were born on or after 2002 were HCV positive despite the blood they received being subjected to screening for HCV. Conclusions: The study suggests the need to step up the transfusions to achieve hemoglobin goal of 10 gm% (as per the moderate transfusion regimen and also to institute urgent and effective chelation measures with the aim of keeping serum ferritin levels below 1000 ng/ml to avoid the systemic effects of iron overload. In addition, strict monitoring of the children for endocrinopathy and other systemic effects of iron overload should be done. Rigid implementation of quality control measures for the ELISA kits used to detect HCV in donor blood needs to be done urgently. Alternately, more sensitive and specific measures (like NAT testing should be employed for detection of HCV. In the absence of a definitive cure accessible and available to all patients, strict implementation of the above suggested measures will go a long way in improving the quality (and quantity of life in patients of beta-thalassemia major.

Shah Neeraj

2010-01-01

218

Conservation of a Rare Painted Ancient Egyptian Textile Object from the Egyptian Museum in Cairo  

OpenAIRE

This study describes conservation of a painted ancientEgyptian textile object from the collection of Osiris clothsin the Egyptian Museum in Cairo, Egypt. An evaluation ofthe reactivation consolidation technique to reinforcementof ancient Egyptian painted textiles was performed.Various investigation methods were carried out to identifythe fibres, paints, and other materials, which are part ofthe selected object. The condition of the object was alsoinvestigated. Newly prepared painted linen tex...

Yasin Zidan; Omar Abdel-Kareem; Nadia Lokma; Hanaa Ahmed

2008-01-01

219

Detection of a major gene for heterocellular hereditary persistence of fetal hemoglobin after accounting for genetic modifiers  

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[open quotes]Heterocellular hereditary persistence of fetal hemoglobin[close quotes] (HPFH) is the term used to describe the genetically determined persistence of fetal hemoglobin (Hb F) production into adult life, in the absence of any related hematological disorder. Whereas some forms are caused by mutations in the [beta]-globin gene cluster on chromosome 11, others segregate independently. While the latter are of particular interest with respect to the regulation of globin gene switching, it has not been possible to determine their chromosomal location, mainly because their mode of inheritance is not clear, but also because several other factors are known to modify Hb F production. The authors have examined a large Asian Indian pedigree which includes individuals with heterocellular HPFH associated with [beta]-thalassemia and/or [alpha]-thalassemia. Segregation analysis was conducted on the HPFH trait FC, defined to be the percentage of Hb F-containing cells (F-cells), using the class D regressive model. The results provide evidence for the presence of a major gene, dominant or codominant, which controls the FC values with residual familial correlations. The major gene was detected when the effects of genetic modifiers, notably [beta]-thalassemia and the XmnI-[sup G][gamma] polymorphism, are accounted for in this analysis. Linkage with the [beta]-globin gene cluster is excluded. The transmission of the FC values in this pedigree is informative enough to allow detection of linkage with an appropriate marker(s). The analytical approach outlined in this study, using simple regression to allow for genetic modifiers and thus allowing the mode of inheritance of a trait to be dissected out, may be useful as a model for segregation and linkage analyses of other complex phenotypes. 39 refs., 4 figs., 6 tabs.

Thein, S.L.; Weatherall, D.J. (Institute of Molecular Medicine, Oxford (United Kingdom)); Sampietro, M.; Rohde, K.; Rochette, J.; Lathrop, G.M.; Demenais, F.

1994-02-01

220

Investigation of liquid wax components of Egyptian jojoba seeds.  

Science.gov (United States)

Egyptian jojoba seeds newly cultivated in Ismailia desert in Egypt promoted us to determine its lipid components. Fatty alcohols, fatty acids, wax esters and sterols patterns were determined by capillary GLC whereas, tocopherols profile, isopropenoid alcohols and sterylglycosides were determined by HPLC. The Egyptian seeds are rich in wax esters (55 %) with fatty alcohols C20:1 and C22:1 as major components and amounted to 43.0 % and 45.6 % respectively followed by C24:1 and C18:1(9.6 % and 1.3 % respectively). The fatty acids profile showed that C20:1 is the major constituent (60 %) followed by C18:1 and C22:1 (14.5 and 11.8 % respectively) whereas C24:1 was present at low concentration amounted to 1.6 %. In addition, the Egyptian jojoba wax contained C18:2 fatty acid at a level of 8.7 %. Wax esters composition showed that the local wax had C42 and C40 esters as major components amounted to 51.1 and 30.1 % respectively. Also, it had C44 and C38 at reasonable amounts (10.0 and 6.3 % respectively). Whereas C36 and C46 were present at lower concentrations amounted to 1.4 and 1.1 respectively. The sterols analysis showed the presence of campe-, stigma-, beta-sito-, and isofuco- sterol amounting to 18.4 %, 6.9 %, 68.7 %, and 6.0 % respectively. The tocopherols pattern revealed that the local seed wax contained gamma-tocopherol as major constituent (79.2 %) followed by alpha-tocopherol (20.3 %). beta-tocopherol as well as delta-tocopherol were found as minor constituents. The isopropenoid alcohols and the sterylglycosides (free and acylated) were not detected. The wax is proposed to be used in oleo chemistry and cosmetics. PMID:19844068

El-Mallah, Mohammed Hassan; El-Shami, Safinaz Mohammed

2009-01-01

221

Unrelated Umbilical Cord Blood Transplant for Children with ?-Thalassemia Major.  

Science.gov (United States)

Beta thalassemia major, one of the most prevalent hemoglobinopathy throughout the word, can be cured by allogenic stem cell transplantation (SCT) (Bone Marrow Transplant 36:971-975, 2005). Many patients, however, lack a suitably matched related sibling donor. Unrelated umbilical cord blood (UCB) can be used as an alternative stem cell source for these patients. This report describes SCT for nine children with beta-thalassemia major using partially HLA-matched unrelated UCB. Conditioning included oral busulfan 16 mg/kg (day -10 to -7), cyclophosphamide (Cy) 200 mg/kg (day -5 to -2), fludarabine 90 mg/kg (day -13 to -11), and antithymocyte globulin (rabbit) 7.5 mg/kg (day -3 to -1). The infused cell dose was 10.71 × 10(7)/kg total nucleated cells (TNC) (range 6.5-17 × 10(7)/kg TNC). The patients ranged in age from 1.5 to 7 years, in weight from 10.5 to 17 kg. A second transplant with two unrelated cord blood units was attempted in two patients who had primary graft failure. The retransplant recipients were preconditioned with i.v Cy 120 mg/kg (day -3 to -2). Five of the nine patients engrafted promptly with 50-100 % donor chimerism (56 %). They engrafted at a median of 17 days (range 12-19). One patient is transfusion free for 36 months; a second patient is transfusion free for 18 months and a third is transfusion free for 9 months. There was no transplant related mortality. Four of the nine children had autologous recovery without engraftment. Primary graft rejection is the major complication. Post transplant complications were mild hepatic veno-occlusive disease, acute GVHD grade II, and CMV interstitial pneumonia. The chronic GVHD was limited and could be controlled by Methylprednisolone combined with Mycophenolate. The lack of a marrow donor registry in India makes UCBT from related and unrelated donors a good alternative. Transplant should be delayed until the child is at least 18 months of age. The dose of UCB stem cells is the most important factor for engraftment. UCB has the advantages of rapid availability and low risk of severe GVHD despite donor-recipient HLA disparity (Transplant Proc 37:2667-2669, 2005). We demonstrate the feasibility of this procedure in the setting of a developing country. PMID:25548438

Shah, Sandip A; Shah, Kamlesh M; Patel, Kinnari A; Anand, Asha S; Talati, Shailesh S; Panchal, Harsha P; Patel, Apurva A; Parikh, Sonia K; Parekh, Bhavesh B; Shukla, Shilin N; Raut, Shreeniwas S

2015-03-01

222

Effect of hepatic iron concentration and viral factors in chronic hepatitis C-infected patients with thalassemia major, treated with interferon and ribavirin  

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Full Text Available Maryam Jafroodi, Ramin Asadi, Abtin Heydarzadeh, Sepiedeh BesharatiDepartment of Hematology, Gulian University of Medical Sciences, Rasht, Guilan, IranBackground: Beta thalassemia major patients are vulnerable to transfusion-transmitted infection, especially hepatitis C virus (HCV, and iron overload. These comorbidities lead to cirrhosis and hepatocellular carcinoma in these patients. In order to prevent these complications, treatment of HCV infection and regular iron chelating seems to be necessary. The aim of this study was to evaluate the effect of hepatic iron concentration (HIC and viral factors on the sustained virological response (SVR in chronic HCV-infected patients, with beta thalassemia major being treated with interferon and ribavirin.Materials and methods: We enrolled 30 patients with thalassemia major and chronic HCV who were referred to the Hematology Clinic of Guilan University of Medical Sciences, between December 2002 and April 2006. HIC was measured by atomic absorption spectroscopy before treatment. The viral factors (viral load, genotype and HIC were compared between those who achieved a SVR and nonresponders.Results: Mean age of the 30 thalassemic patients, was 22.56 ± 4.28 years (14–30 years. Most patients were male (56.7%. Genotype 1a was seen in 24 (80% cases. SVR was achieved in 15 patients (50%. There were no significant correlations between HIC (P = 1.00, viral load (P = 0.414, HCV genotype (P = 0.068, and SVR. No difference was observed in viral load (P = 0.669 and HIC (P = 0.654 between responders and nonresponders.Conclusion: HIC, HCV viral load, and HCV genotype were not correlated with virological response, and it seems that there is no need to postpone antiviral treatment for more vigorous iron chelating therapy.Keywords: hepatitis C virus, hepatic iron concentration, combination therapy, thalassemia major, interferon alfa, ribavirin

Jafroodi M

2011-07-01

223

Egyptian blue — Cuprorivaite a window to ancient Egyptian technology  

Science.gov (United States)

Egyptian Blue, a multicomponent synthetic blue pigment has been recorded in ancient Egypt since the Fourth Dynasty of the Old Kingdom (2600 2480 B.C.). The pigment consisting of cuprorivaite (CaCuSi4O10) with variable amounts of wollastonite (CaSiO3), Cu-rich glass and cuprite (Cu3O) or tenorite (CuO) was prepared by melting the copper-rich ingredient with lime and desert sand. Low melting temperatures (below 742 °C) were achieved by addition of flux-like plant ashes. The high quality of the pigments collected from monuments of the Fifth Dynasty (2480 2320 B.C.) may indicate that the first manufacture was in early dynastic or perhaps predynastic eras. During the reign of Thutmosis III (18th Dynasty, 1490 1436 B.C.) probably bronze filings were first applied as starting material, thus indicating a technological innovation. This new method was employed till the Roman times.

Jaksch, H.; Seipel, W.; Weiner, K. L.; Goresy, A. El

1983-11-01

224

Evaluation of Endocrine Complications in Patients with Thalassemia Major  

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Full Text Available Aim: Multiple blood transfusions in beta thalassemia patients causes iron overload in various tissues including endocrine glands thereby leading to multiple endocrine dysfunction. The aim of this study was to determine the endocrine complications seen in beta thalassemia patients followed-up in outpatient clinics of Pediatric Hematology Division of Uludag University Faculty of Medicine. Materials and Methods: The files of patients with thalassemia major followed-up in outpatient clinics of Pediatric Hematology Division of Uludag University Faculty of Medicine from January 1976 to August 2008 were retrospectively evaluated for endocrine disorders. All patients had a detailed physical examination including palpation of thyroid gland and pubertal staging. Endocrine evaluation was performed in the Division of Pediatric Endocrinology.Results: A total of 44 [20 female (45.5%; 24 male (54.5%; and mean chronological age 13.54±7.32 (2.75-35.2 years] patients were evaluated. The ratios of patients with endocrine dysfunction were 27.2 % and 90.9%, respectively, when we exclude or include those with osteoporosis/osteopenia or growth failure other than growth hormone deficiency. Of all patients, 27 (61.3% had osteoporosis, 17 (38.6% had growth retardation, 11 (25% had osteopenia, 6 (13.6% had hypogonadism, 3 (6.8% had hypothyroidism, 2 (4.5% had hypoparathyroidism, 1 (2.3 had growth hormone deficiency, and 1 (2.3 had type 1 diabetes mellitus. Mean ferritin levels and monthly transfusion numbers were 1976.15±1494.75 ng/ml and 1.46±0.34, respectively. There were no significant association between ferritin levels, monthly transfusion needs, and endocrine dysfunctions studied. Endocrine dysfunctions did not differ significantly amongst those having different chelating agents. The ratio of patients with growth retardations in 10 to 19-age-group was significantly higher than those in 0 to 9-age-group (30.6% vs 8.3%; p=0.049. Conclusion: Patients with thalassemia major are under increased risk of various endocrine dysfunction. Bone health is significantly compromised. Those younger than 10 years should be closely followed for especially growth retardation and osteoporosis/osteopenia and those who are 10 years of age or older should be followed for all endocrine pathologies, especially for hypogonadism, growth retardation, and osteoporosis. (Journal of Current Pediatrics 2008; 6: 58-65

Birol Baytan

2008-10-01

225

Radiological assessment of the Egyptian Mediterranean coast  

International Nuclear Information System (INIS)

On the basis of extensive sampling, gamma spectrometry, laboratory analyses, data evaluation and comparison with the reference data, the following assessment has been carried out. The radiological assessment has been done for a wide scale study area (about 1100 km) from El- Salloum in the west of the Mediterranean coast of Egypt to El-Arish in the east. The environmental radiological assessment steps are based on the criteria previously mentioned. The sources of radioactivity possibly reach the Egyptian Mediterranean coast can be summarized as: primordial and radiogenic radionuclides (e.g. 40K, 238U and 232Th series), cosmic rays and cosmogenic radionuclides (e.g. 7Be), fertilizers (e.g. superphosphate), black sand (transported by Nile River), fallout (either from nuclear testing or Chernobyl), seawater currents (transported either natural or man-made), Suez Canal (subject to receive a radioactive releasing and effluents from either nuclear power ships or submarines passes through the canal), biological migration and sedimentary longshore movement (e.g. Anguilla anguilla), atmospheric radioactivity (e.g. 222Rn and its daughters), domestic and medical sewage (minor source, short half-life), depleted uranium dust (possibly), satellite and aircraft accident (accidentally) and rarely loss of industrial radioactive source (incidentally). El-Salloum, Rashid and El-Gamil have been considered as concentrated basins for thn considered as concentrated basins for the majority of the radioisotopes. This suggestion was due to the special topographical features of these three stations, which make the accumulation of the radioactive isotopes possible. Calculations of outdoor absorbed dose rate for human population at all stations under investigation from 238U, 232Th and 40K were carried out. The calculated absorbed dose rate has been distinguished the coast into normal areas and Rashid black sand area as high background area. The range of calculated dose was 8.39-38.5 ?Gy/h. Good agreement was observed with NCRP absorbed dose rates 26 (17-40) nGy/h. Calculations of gamma absorbed dose rate in Rashid black sand area collected in July 1998 recorded as 0.72 ?Gy/h. This value was considered as relatively high dose rate and it is remarkable of the high background radiation area at Rashid. Comparing with the reported gamma radiation dose rates in the others high background radiation areas, it was in agreement with Ramsar in Iran (0.7-50 ?Gy/h) and relatively lower than 1-2 ?Gy/h at Guarapari, Meaipe and Cumuruxatiba in Brazil. Annual intake radiation dose was achieved by estimation of the human intake of radioactivity due to fish consumption considering the Egyptian values. Using the mathematical calculation of annual dose per Bq, the total dose to a person, daily fed with 0.0136 kg fish, in one year was 10.3 ?Sv/y, which is mainly from 40K. Annual intake radiation dose was achieved by estimation of the human intake of radioactivity due to fish consumption considering the Egyptian values. Using the mathematical calculation of annual dose per Bq, the total dose to a person, daily fed with 0.0136 kg fish, in one year was 10.3 ?Sv/y, which is mainly from 40K. The only man-made radionuclide detected in the Egyptian Mediterranean coast is 137Cs with average value 0.37 Bq/kg. This average value shows high agreement with the other referenced values in the world. The main source of 137Cs is atmospheric fallout either from nuclear testing or Chernobyl. The marine transport from western Mediterranean could be considered as another source. Surface seawater salinity showed a positive correlation with the concentrations of 40K in surface seawater (r = 0.64). A mathematical model has been deduced to estimate the range of natural 40K in the surface seawater. Assessment of radioactivity bioaccumulators was made among different biota collected from the Egyptian Mediterranean coast. It revealed that, Patella spp., Anguilla spp. and Mytilus spp. were the best marine organisms for having the capability to accumulate 40K. Also, Patella spp., Ang uilla spp.

226

Cutaneous and mucosal manifestations in patients with beta major thalassemia  

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Full Text Available Background and Aim: Thalassemia is one of the most common single gene disorder that results from decrease or absence of globulin chain(s synthesis. Repeated blood transfusion is required for prevention of severe anemia and related side effects, but it is associated with other complications such as cutaneous lesions and mucosal manifestations. This study conducted to assess the frequency of cutaneous and mucosal manifestations in patients with beta thalassemia.Methods: This cross-sectional study was conducted on 300 patients with beta major thalassemia in Zahedan. Data were obtained from medical records and questionnaires as well as through physical examination of patients. The data were statistically described and tested.Results: The mean age of patients was 17.9±6.3 years and 64.3% of patients were men and 35.7% were women. Among cutaneous lesions, freckles were the most common (70.7% and the rarest one was peri orbital pigmentation (0.3%. Gingivitis (41.7% and longitudinal lines on nails (72% were the most common mucosal and nail manifestations.Conclusion: Since cutaneous and mucosal manifestations are very common among patients with beta major thalassemia, regular dermatology follow up of these patients is recommended.

Majid Naderi

2013-03-01

227

Expression of therapeutic misconception amongst Egyptians: a qualitative pilot study  

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Full Text Available Abstract Background Studies have shown that research participants fail to appreciate the difference between research and medical care, labeling such phenomenon as a "therapeutic misconception" (TM. Since research activity involving human participants is increasing in the Middle East, qualitative research investigating aspects of TM is warranted. Our objective was to assess for the existence of therapeutic misconception amongst Egyptians. Methods Study Tool: We developed a semi-structured interview guide to elicit the knowledge, attitudes, and perspectives of Egyptians regarding medical research. Setting: We recruited individuals from the outpatient settings (public and private at Ain Shams University in Cairo, Egypt. Analysis: Interviews were taped, transcribed, and translated. We analyzed the content of the transcribed text to identify the presence of a TM, defined in one of two ways: TM1 = inaccurate beliefs about how individualized care can be compromised by the procedures in the research and TM2 = inaccurate appraisal of benefit obtained from the research study. Results Our findings showed that a majority of participants (11/15 expressed inaccurate beliefs regarding the degree with which individualized care will be maintained in the research setting (TM1 and a smaller number of participants (5/15 manifested an unreasonable belief in the likelihood of benefits to be obtained from a research study (TM2. A total of 12 of the 15 participants were judged to have expressed a TM on either one of these bases. Conclusion The presence of TM is not uncommon amongst Egyptian individuals. We recommend further qualitative studies investigating aspects of TM involving a larger sample size distinguished by different types of illnesses and socio-economic variables, as well as those who have and have not participated in clinical research.

Silverman Henry J

2009-06-01

228

Radiological assessment of the Egyptian Mediterranean Coast  

International Nuclear Information System (INIS)

On the basis of extensive sampling, gamma spectrometry, laboratory analyses, data evaluation and comparison with the reference data, the following assessment has been carried out. The radiological assessment has been done for a wide scale study area (about 1100 km) from El-Salloum in the west of the Mediterranean coast of Egypt to El-Arish in the east. The environmental radiological assessment steps are based on the criteria previously mentioned. The sources of radioactivity possibly reach the Egyptian Mediterranean coast can be summarized as: primordial and radiogenic radionuclides (e.g. 40K, 23'8U and 232Th series), cosmic rays and cosmogenic radionuclides (e.g. 7Be), fertilizers (e.g. superphosphate), black sand (transported by the Nile River), fallout (either from nuclear testing or Chernobyl), seawater currents (transported either natural or man-made), Suez Canal (subject to receive a radioactive releasing and effluents from either nuclear power ships or submarines passing through the Canal), biological migration and sedimentary longshore movement (e.g. Anguilla anguilla), atmospheric radioactivity (e.g. 222Rn and its daughters), domestic and medical sewage (minor source, short half-life), depleted uranium dust (possibly), satellite and aircraft accident (accidental) and rarely loss of industrial radioactive source (incidentally). El-Salloum, Rashid and El-Gamil have been considered as concentrated basins forbeen considered as concentrated basins for the majority of the radioisotopes. This suggestion was due to the special topographical features of these three stations, which make the accumulation of the radioactive isotopes possible. Calculations of outdoor absorbed dose rate for human population at all stations under investigation from 238U, 232Th and 40K were carried out. The calculated absorbed dose rate has been distinguished the coast into normal areas and Rashid black sand area as high background area. The range of calculated dose was 8.39-38.5 nGy/h. Good agreement was observed with NCRP absorbed dose rates 26 (17-40) nGy/h. Calculations of gamma absorbed dose rate in Rashid black sand area collected in July 1998 recorded as 0.72 ?Gy/h. This value was considered as relatively high dose rate and it is remarkable of the high background radiation area at Rashid. Comparing with the reported gamma radiation dose rates in the others high background radiation areas, it was in agreement with Ramsar in Iran (0.7-50 ?Gy/h) and relatively lower than 1-2 ?Gy/h at Guarapari, Meaipe and Cumuruxatiba in Brazil. Annual intake radiation dose was achieved by estimation of the human intake of radioactivity due to fish consumption considering the Egyptian values. Using the mathematical calculation of annual dose per Bq, the total dose to a person, daily fed with 0.0136 kg fish, in one year was 10.3 ?Sv/y, which is mainly from 40K. The only man-made radionuclide detected in the Egyptian Mediterranean coast is 137Cs with an average value 0.37 Bq/kg. This average value shows high agreement with the other referenced values in the world. The main source of 137Cs is atmospheric fallout either from nuclear testing or Chernobyl. The marine transport from western Mediterranean could be considered as another source. Surface seawater salinity showed a positive correlation with the concentrations of 40K in surface seawater (r = 0.64). A mathematical model has been deduced to estimate the range of natural 40K in surface seawater. Assessment of radioactivity bioaccumulators was made among different biota collected from the Egyptian Mediterranean coast. It revealed that, Patella spp., Anguilla spp. and Mytilus spp were the best marine organisms for the capability to accumulate 40K. Also, Patella spp., Anguilla spp. and the intermediate leaves of Posidonia spp. were found as good accumulators of both 214Pb and 214Bi. Anguilla spp. and Mytilus were found to be good accumulators of 228Ac

229

Egyptian and foreign cigarettes Pt. 2  

International Nuclear Information System (INIS)

The concentration of 27 elements was measured using neutron activation analysis in a cigarette brand commercially manufactured in Egypt and in three foreign brands available on the Egyptian market. The cigarette components examined were tobacco, wrapping paper and ash. All results are expressed in absolute values per cigarette. The concentration of Al, As, Ba, Br, Cl, Cs, Eu, Fe, Mn, Na, Ni, Rb, Sb, Sc, Sr, Th, Ti and V in the Egyptian cigarette were above the range determined for the foreign brands examined; however, Ce, Co, Cr, Hf, K, La, Mg, Se and Zn were within that range. Except for K, Mn, Ni and Ti, the above conclusions were the same for elements determined in the ash. The wrapping paper used for the Egyptian cigarette contains the lowest quantity of Cl and Mn. (author)

230

STREPTOMYCETE Producing Antibiotics Isolated From Egyptian Soil  

International Nuclear Information System (INIS)

An investigation was concerned the distribution of Streptomyces species in some types of Egyptian soils. Thirty nine actinomycetes isolates were obtained from 7 types of Egyptian soils; 8 isolates exhibited antimicrobial activities against all tested organisms and three of them were identified as Streptomyces griseoluteus (YM23), Streptomyces aurantiogriseus (S15) and Streptomyces nogalator (H12). RAPD-PCR showed correlation between 8 isolates in 3 clades. The active metabolite was extracted with ethyl acetate and concentrated in vacuum and the crude fraction was purified using thin layer and column chromatography

231

Composition of Egyptian nerolì oil.  

Science.gov (United States)

The bitter orange flower oil (or neroli) is an essential product, largely used in perfumery. Neroli is obtained by hydrodistillation or steam distillation, from the flowers of bitter orange (Citrus aurantium L.). Since a long time neroli production is limited and its cost on the market is considerably high. The annual production in Tunisia and Morocco is ca. 1500 Kg, representing more than 90% of the worldwide production. A small amount ofneroli is also produced in Egypt, Spain and Comorros (not exceeding 150 kg totally). Due to the high cost, the producers and the users have tried to obtain less expensive products, with odor characters close to that of neroli oil to be used as substitute and sometimes as adulterants of the genuine oil. In this study are investigated five samples of Egyptian neroli oils produced in 2008 and 2009, in the same industrial plant, declared genuine by the producer. For all the samples the composition was determined by GC/FID and by GC/MS-LRI; the samples were also analyzed by esGC to determine the enantiomeric distribution of twelve volatiles and by GC-C-IRMS for the determination of the delta13C(VPDB) values of some mono and sesquiterpene hydrocarbons, alcohols and esters. The analytical procedures allowed to quantitatively determining 86 components. In particular the variation of the composition seems to be dependent on the period of production. In fact, the amount of linalool decreases from March to April while linalyl acetate presents an opposite trend, increasing in the same period. The RSD determined for the delta13C(VPDB) are very small (max. 3.89%), ensuring the authenticity of all samples. The results are also discussed in function of the limits provided by the European Pharmacopoeia (EP) (2004), AFNOR (1995) and ISO (2002) regulations for genuine neroli oils. PMID:21834247

Bonaccorsi, Ivana; Sciarrone, Danilo; Schipilliti, Luisa; Trozzi, Alessandra; Fakhry, Hussein A; Dugo, Giovanni

2011-07-01

232

Comparative Studies on Egyptian and Libyan Roselle Seeds as a Source of Lipid and Protein  

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Full Text Available Proceeding from the fact that the seeds of Roselle plant are full of nutritional constituents, however in Egypt and Libya, they are often discarded as a by-product, this study aims to evaluate the nutritional composition of Roselle seeds grown in Egypt and Libya as a source of oil and protein besides making a comparison between whole chemical composition of Roselle seeds grown in both countries. Ground of whole Egyptian and Libyan Roselle seeds powder contained high amount of protein (31.02% ± 0.93% and 28.67% ± 0.45%, crude fat (21.6% ± 0.66% and 16.94% ± 0.86% and total ash (6.89% ± 0.11% and 5.60% ± 0.10%, respectively. However, Egyptian seeds have moisture content, protein, crude fat and total ash higher than Libyan seeds. Crude oil from Egyptian seeds had high refractive index and iodine value in comparison with crude oil from Libyan seeds. There were no remarkable differences between both seeds in acidity percent, unsaponifiable matters percent and saponification value. Linoleic, oleic and palmitic acids were the major fatty acid constituents in Egyptian Roselle seeds. Meanwhile linolenic, linoleic, oleic, stearic, palmitoleic and palmitic acids were the major fatty acid constituents in Libyan Roselle seeds. Crude oil from Egyptian seeds had higher percent of unsaturated fatty acids than crude oil from Libyan seeds. Unsaponifiable matters constituents for extracted oil from Egyptian seeds were free from n-pentacosane (C25 and rich in n-hexacosane (C26. Oil from both seeds had the same content of Beta sito-sterol and stigma-sterol. Both seeds were rich in glutamic acid, aspartic acid, arginine and leucine. Libyan seeds were rich in essential amino acids in comparison with Egyptian seeds. Finally nutritional comparison of Roselle seeds variation depends on the variety, location and environmental conditions during cultivation. Roselle seeds are a good source for extraction of oil and protein. Protein from Roselle seeds could be used as a supplement material for poor food in lysine.

Nady A. Elneairy

2014-12-01

233

Hair-offerings: an enigmatic Egyptian custom  

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Full Text Available The Egyptians did not record the reasons that lay behind the offering of hair. Using an holistic approach, which combines both ethnographic and ethnohistoric evidence, insights may be gained into the ancient remains of these rituals and practices.

G. J. Tassie

1996-11-01

234

Clinical anatomy as practiced by ancient Egyptians.  

Science.gov (United States)

Egypt is famously known for its Nile and pyramids, yet not many people know that Egypt made possible the origin of the anatomical sciences. Several ancient papyri guide us through the Egyptians' exploration of the human body and how they applied anatomical knowledge to clinical medicine to the best of their knowledge. It is through records, such as the Edwin Smith, Ebers, and Kahun papyri and other literature detailing the work of the Egyptian embalmers, physicians, and Greek anatomists, that we are able to take a glimpse into the evolution of the anatomical sciences from 3000 B.C. to 250 B.C. It is through the Egyptian embalmer that we were able to learn of some of the first interactions with human organs and their detailed observation. The Egyptian physician's knowledge, being transcribed into the Ebers and Edwin Smith papyri, enabled future physicians to seek reference to common ailments for diagnosing and treating a variety of conditions ranging from head injuries to procedures, such as trans-sphenoidal surgery. In Alexandria, Herophilus, and Erasistratus made substantial contributions to the anatomical sciences by beginning the practice of human dissection. For instance, Herophilus described the anatomy of the heart valves along with Erasistratus who demonstrated how blood was prevented from flowing retrograde under normal conditions. Hence, from various records, we are able to unravel how Egypt paved the road for study of the anatomical sciences. PMID:21509810

Loukas, Marios; Hanna, Michael; Alsaiegh, Nada; Shoja, Mohammadali M; Tubbs, R Shane

2011-05-01

235

ANTHROPOMETRIC STUDY OF NASAL INDEX OF EGYPTIANS  

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Full Text Available Background: The nasal index determination is one of the most commonly used anthropometric parameters in classifying human races. There are few reports in medical literature concerning nasal index that specifically address particular Egyptian populations. The objective of this study was to determine the normal parameters of external nose (width, height and nasal index in Egyptians. Methods: The study was conducted randomly on healthy Egyptian subjects of both sexes. Nasal height and width were measured using vernier caliper. Then, nasal index was determined for each subject. The obtained data were subjected to statistical analysis. Results: A total of 290 subjects, 144 males and 146 females, aged 1 month– 65 years, were enrolled in the study. The study showed the existence of sexual dimorphism in nasal morphology, appearing after the age 20 years. The mean nasal index in the investigated adults was 68.01; in males and females was 71.46 and 64.56, respectively. Conclusions: The dominant nasal type in Egyptians was in-between mesorrhine "medium" and leptorrhine "narrow" nose. Forensic and anthropological research, as well as cosmetic and reconstructive surgery may benefit from age- and sex- based data of the study.

Abdelmonem Awad Hegazy

2014-12-01

236

Some endocrinal aspects of pancreas in beta thalassemia  

International Nuclear Information System (INIS)

The study was carried out to evaluate endocrinal aspects of the pancreas in thalassemia children, ages 5 - 10 years maintained under either regular or irregular iron chelation therapy. A matched control on healthy children, was included for comparison. As regards laboratory findings, fasting and post prandial blood sugar was significantly elevated in thalassemia children than the control and serum insulin was significantly lower in the irregular iron chelation group than both in the regular one and in the control group. Most cases of diabetic thalassemia children clinically and biochemically were of irregular iron chelation and with older age. They were frequently higher transfused. There is significant rise of serum ferritin in diabetic group than in diabetic thalassemia children. In conclusion, pancreatic dysfunction can be detected in thalassemia children, but regular iron chelation can reduce its occurrence. 10 tabs

237

Beta-thalassemia disease prevention: genetic medicine applied.  

OpenAIRE

We report here an evaluation of a program for thalassemia-disease prevention, comprising education, population screening for heterozygotes, and reproductive counseling; the evaluation includes cost analysis. A preprogram survey in 1978 of 3,247 citizens in the high-risk communities (85% were high-school students) showed that 88% favored a program but that only 31% considered fetal diagnosis as an acceptable option. Screening in high school or before marriage was preferred by 56%. In a 25-mont...

Scriver, C. R.; Bardanis, M.; Cartier, L.; Clow, C. L.; Lancaster, G. A.; Ostrowsky, J. T.

1984-01-01

238

COAGULOPATHY IN BETA-THALASSEMIA: CURRENT UNDERSTANDING AND FUTURE PERSPECTIVES  

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Full Text Available

As the life expectancy of ?-thalassemia patients has markedly improved over the last decade, several new complications are being recognized. The presence of a high incidence of thromboembolic events, mainly in thalassemia intermedia patients, has led to the identification of a hypercoagulable state in thalassemia. In this review, the molecular and cellular mechanisms leading to hypercoagulability in thalassemia are highlighted, and the current clinical experience is summarized. Recommendations for thrombosis prophylaxis are also discussed.

Alessia Marcon

2009-06-01

239

studies on extraction of some nuclear materials from egyptian ores  

International Nuclear Information System (INIS)

The present thesis is devoted to systematic studies on extraction of some nuclear materials from egyptian ores. Extraction of thorium, uranium and rare earths from egyptian monazite and beryllium from egyptian beryl mineral, was investigated. Such elements were taken as a representative group of reactor materials, currently used as fuel and for structural and cladding purposes. In an introducing chapter, various nuclear materials are reviewed. Their importance in nuclear field, physical characteristics, nuclear and non- nuclear uses are surveyed

240

The thermoluminescence (TL) of Egyptian Blue  

International Nuclear Information System (INIS)

Egyptian Blue is a synthesized crystalline pictorial pigment with formula CaCuSi4O10. It has been used in Egypt and Mesopotamia from the 3rd millenium B.C. A preliminary experiment on a recently synthesized sample showed that this pigment is thermoluminescent after ? irradiation (90Sr). As the signal intensity grows linearly with the administered dose within the temperature range commonly used in TL dating, we have been looking for this phenomenon from archaeological pigments. It was encountered with two samples found in excavation. From its intensity and stability we concluded that Egyptian Blue can be dated using TL. This first and positive result encouraged us to extend the method to other types of mineral pigments synthesized by early man, and to suggest that it may be used for direct dating of ancient murals. (author)

241

Attributional style of Egyptians with schizophrenia.  

Science.gov (United States)

The attributional style is one domain of social cognition that involves perceiving, interpreting, and generating responses to others' intentions and behaviours in different situations. This study describes the attributional style of Egyptians with schizophrenia. The study took place in a psychiatric hospital in Egypt. Eight psychiatric wards were randomly selected, and all participants (150) with a diagnosis of schizophrenia participated. Data were collected using the Ambiguous Intention Hostility Questionnaire (AIHQ), the attributional style interview schedule, and the sociodemographic/clinical data sheet. Participants' scores on the AIHQ varied significantly between different situations (intentional, accidental, and ambiguous). The attributional style of the studied participants regarding their perceived psychosocial problems tended to be related to specific causes. These causes were mostly externally attributed to other people and to circumstances, and were perceived not to persist in the future. It would seem that Egyptians with schizophrenia tend to blame other people for negative events if these events are perceived to cause intentional harm. PMID:21054731

Elnakeeb, Mayar; Abdel-Dayem, Samia; Gaafar, Maha; Mavundla, Thandisizwe R

2010-12-01

242

Natural radioactivity for some Egyptian building material  

International Nuclear Information System (INIS)

Study of the radiation hazards for the building materials is interested in most international countries. Measurements of natural radioactivity was verified for some egyptian building materials to assess any possible radiological hazard to man by the use of such materials. The measurements for the level of natural radioactivity in the materials was determined by ?-ray spectrum using HP Ge detector. A track detector Cr-39 was used to measure the radon exhalation rate from these materials. The radon exhalation rates were found to vary from 2.83±0.86 to 41.57 ± 8.38 mBqm-2 h-1 for egyptian alabaster. The absorbed dose rate in air is lower than the international recommended value (55 n Gy h-1) for all test samples

243

Mitochondrial genome sequence of Egyptian swift Rock Pigeon (Columba livia breed Egyptian swift).  

Science.gov (United States)

Abstract The Egyptian swift Rock Pigeon is a breed of fancy pigeon developed over many years of selective breeding. In this work, we report the complete mitochondrial genome sequence of Egyptian swift Rock Pigeon. The total length of the mitogenome was 17,239?bp and its overall base composition was estimated to be 30.2% for A, 24.0% for T, 31.9% for C and 13.9% for G, indicating an A-T (54.2%)-rich feature in the mitogenome. It contained the typical structure of 13 protein-coding genes, 2 ribosomal RNA genes, 22 transfer RNA genes and a non-coding control region (D-loop region). The complete mitochondrial genome sequence of Egyptian swift Rock Pigeon would serve as an important data set of the germplasm resources for further study. PMID:24438285

Li, Chun-Hong; Shi, Wei; Shi, Wan-Yu

2014-01-17

244

Feminist Perspectives on the Egyptian Revolution  

OpenAIRE

The Egyptian Revolution 2011 created a space and opportunity to forward important demands concerning different social and political issues, amongst these matters related to women's status and situations in the society. Feminist and women's rights proponents in Egypt conceptualize the aims of a feminist or women's rights agenda as (women's) human rights and citizen's rights, independence, freedom and choice, though such universal concepts are understood in accordance with particular cultural o...

Hellstrand, Anna

2012-01-01

245

Chronic neutrophilic leukaemia: an Egyptian case  

OpenAIRE

Chronic neutrophilic leukaemia (CNL) is a rare myeloproliferative disorder of elderly patients characterised by sustained neutrophilia. The diagnosis of CNL requires the exclusion of BCR/ABL positive chronic myelogenous leukaemia and leukemoid reaction. We present here a case of a 61-year-old Egyptian man with CNL and 21 months of follow-up. The main symptom of our patient was purpura. Splenomegaly, hepatomegaly and lymph node enlargement were not detected at presentation or throughout the pa...

Elbahrawy, Ashraf; Hamdy, Mahmoud; Morsy, Mohamed Hanafy; Ragab, Ramadan

2009-01-01

246

Chlorpyrifos Exposures in Egyptian Cotton Field Workers  

OpenAIRE

Neurobehavioral deficits have been reported in Egyptian pesticide application teams using organophosphorus (OP) pesticides, but whether these effects are related to OP pesticide exposures has yet to be established. In preparation for a comprehensive study of the relationship between OP pesticide dose and neurobehavioral deficits, we assessed exposure within this population. We conducted occupational surveys and workplace observations, and collected air, dermal patch and biological samples fro...

Farahat, Fayssal M.; Fenske, Richard A.; Olson, James R.; Galvin, Kit; Bonner, Matthew R.; Rohlman, Diane S.; Lein, Pamela J.; Anger, W. Kent

2010-01-01

247

Egyptian plant species as new ozone indicators  

International Nuclear Information System (INIS)

Of more than 30 species of plants from Egypt screened for sensitivity to ozone, four were found to be suitable for use as bioindicators. - The aim of this study was to test and select one or more highly sensitive, specific and environmentally successful Egyptian bioindicator plants for ozone (O3). For that purpose more than 30 Egyptian species and cultivars were subjected to extensive screening studies under controlled environmental and pollutant exposure conditions to mimic the Egyptian environmental conditions and O3 levels in urban and rural sites. Four plant species were found to be more sensitive to O3 than the universally used O3-bioindicator, tobacco Bel W3, under the Egyptian environmental conditions used. These plant species, jute (Corchorus olitorius c.v. local), clover (Trifolium alexandrinum L. c.v. Masry), garden rocket (Eruca sativa c.v. local) and alfalfa (Medicago sativa L. c.v. local), ranked in order of decreasing sensitivity, exhibited typical O3 injury symptoms faster and at lower O3 concentrations than Bel W3. Three variables were tested in search of a reliable tool for the diagnosis and prediction of O3 response prior to the appearance of visible foliar symptoms: pigment degradation, stomatal conductance (gs) and net photosynthetic CO2 assimilation (Pnet). Pigment degradation was found to be unreliable in predicting species sensitivity toiable in predicting species sensitivity to O3. Evidence supporting stomatal conductance involvement in O3 tolerance was found only in tolerant species. A good correlation was found between gs, restriction of O3 and CO2 influx into the mesophyll tissues, and Pnet. Changes in Pnet seemed to depend largely on fluctuations in gs

248

Enhancement of Nuclear Security Measures: Egyptian Case  

International Nuclear Information System (INIS)

Egypt benefited from the peaceful uses of nuclear energy since the second half of twentieth century and established several nuclear and radiation facilities, activities and practices such as research reactors, fuel fabrication facility, gamma irradiators, and utilization of radioactive sources in different applications. Consequently, Egypt’s concern is to enhance and improve the nuclear security systems in the above mentioned facilities, activities and practices as well as enhance the nuclear security regime on the national, regional and international levels. In this paper, several steps taken by Egypt in the field of nuclear security in its various aspects such as the legal and regulatory frameworks, physical protection of nuclear material and facilities, nuclear material accounting and security of radioactive sources will be presented. Egyptian human resources development strategy for the purpose of improving the efficiency, building, upgrading and strengthening the knowledge and skills of the personnel who are working in the nuclear and radiological fields as well as other activities that related to nuclear security will also be discussed. The Egyptian nuclear security strategy is under development taking in its consideration the present nuclear security measures and the future perspective to develop and improve the Egyptian nuclear security infrastructure. (author)

249

Visitor Perceptions of Ancient Egyptian Human Remains in Three United Kindom Museums  

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Full Text Available Although the issues of retention and display of human remains have become topical over the last decade, the thoughts of museum visitors about this topic have not been registered, despite their being the museums’ main stakeholder. The vast majority (82.5% of 300 respondents questioned in the summer of 2002 at three British museums displaying ancient Egyptian human remains supported the idea of having these remains on display. However, a small percentage of visitors (14.2% wanted the remains displayed in a "more appropriate and respectful environment", and this may be the key future challenge for collections with human remains that have no cultural descendants. This paper summarises research into visitor perceptions of ancient Egyptian human remains in museum collections and on display in the United Kingdom and suggests further research into the various aspects of human remains in museums, particularly regarding more recent remains or those with cultural descendants.

Hugh Kilminster

2003-11-01

250

Impaired renal tubular function in pediatric patients with ?-thalassemia major  

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Full Text Available Introduction: ?-thalassemia major due to ineffective erythrocyte life span leads to severe anemiarequiring regular transfusion, which it can lead to major organs damage. Renal damage can beattributed to chronic anemia, iron over load and or defferoxamin toxicity. The object of this study wasto analyze renal tubular and glomerular function in pediatric patients with beta-thalassemia major.Materials and Methods: 39 patients with ? -thalassemia major whose mean ages were 11.8 yr and22 sex and age matched healthy children as control group were studied. Blood rine samplesand uwereobtained for measuring biochemical markers. Urinary N-Acetyl-?.D.glucosaminidase (NAG andurine NAG to creatine ratio (U Cr /NAGratio were measuredResults: No significant differences were found between serum BUN, creatinine, and also creatinineclearance between both groups. Also there were no significant differences in urine osmolality betweenpatients and controls. Urine NAG was significantly higher in thalassemic patients than control group(p< 0.000. There was positive relation between urinary NAG and duration of illness(r = 0314,p<0.01. There was no significant relationship between urinary NAG and serum ferritin. Finally, nosignificant difference was found in GFR of both groups.Conclusion: Our study showed renal tubular function is impaired in in patients with ?-thalassemiamajor as indicated with increased urinary NAG. Further, we have found a positive relationshipbetween urinary NAG and duration of disease. Thus, in spite of normal GFR and other biochemicalmarker such as BUN and creatinine, increased urinary NAG may be as an early marker of renaldisease in patients with ?-thalassemia majo.

Mojgan Mazaheri

2009-02-01

251

Infrared absorption spectra of Egyptian Serpentine Rock  

International Nuclear Information System (INIS)

Infrared absorption spectra of natural egyptian serpentine rock were recorded in the frequency range 200-4000 cm-1, and through the temperature range 200-1000 degree C for 2 hours. The chemical bonds within the lattice structure of serpentine are interpenetrated quantitatively. From the intensities and frequencies shift op the characteristic infrared bands spectra, through heat treatment, we can detect the first endothermic reaction due to the loss of hydroxyl bonds up to 700 degree C, and recrystallization for forsterite refractory at 800 degree C, with the characteristics infrared bands at 506-521 cm-1 of Si-O bending vibrations, at 880-888 cm-1 of Si O-Al vibrations, and at 1000-1075 cm-1 of Si-O stretching vibrations. The infrared bands at 350-398 cm-1 (Si-O bend), and 400-448 cm-1 (Si-O-Mg) are sensitive to follow the phase transformation through heat treatment. Where Egyptian serpentine rock of chemical composition Mg6(Si4 O10) (OH8), or Mg3(Si2 O5)(OH4) or 3 Mg O.2 Si O2.2 H2 O magnesium hydrositicate, trioctahedral 1:1 family layer lattice silicate still with its lattice structure till 700 degree C. endothermic reaction due to the deformation of hydroxyl bonds, and then formation of forsterite of chemical composition 2 Mg O.Si O2 from 800 degree C, and the infrared absorption spectra of egyptian serpentine detect a minor quantities of A12 O3 and this phase of tine is Antigorite Mg3(Si2 O5)(OH)4 trioctahedral 1:1 family and high purest related to its standard. Most of the intensities of the characteristics infrared bands spectra are decreased as temperature increases up to 1000 degree C. 2 figs.,2 tabs

252

A-APR Egyptian Fractions II  

Science.gov (United States)

This is a task from the Illustrative Mathematics website that is one part of a complete illustration of the standard to which it is aligned. Each task has at least one solution and some commentary that addresses important asects of the task and its potential use. Here are the first few lines of the commentary for this task: Ancient Egyptians used unit fractions, such as $\\frac{1}{2}$ and $\\frac{1}{3}$, to represent all other fractions. For example, they might express the n...

253

Electrical properties of Egyptian natural graphite  

International Nuclear Information System (INIS)

The electrical properties of Egyptian natural graphite flakes, obtained from the graphite schists of Wadi Bent, Eastern Desert, were measured. The flakes were ground and compressed into pellets. The standard four probe dc method was used to measure the temperature dependence of the electric resistivity from room temperature down to 12 K. The transverse and longitudinal magnetoresistance were measured in the low magnetic field range at temperatures 300 K, 77 K and 12 K. The transverse magnetoresistance data was used to estimate the average mobility, assuming a simple two-band model. (author). 20 refs, 4 figs, 1 tab

254

Computer tomographic investigation of ancient Egyptian mummies  

Energy Technology Data Exchange (ETDEWEB)

Radiological and computer tomographic examinations of Egyptian mummies have been carried out at the Institute of Anthropology and Human Genetics from 1975 to 1978. These have demonstrated the value of CT in medical archaeology. It enables one to study the soft tissues, the skin (if bandaged), the muscles and any organs retained in situ for magical or religious reason. Measurements of attenuation values indicate the materials which were used for mummifying the skin and organs. Characteristic examples are described and the early results of these examinations are discussed.

Huebner, K.H.; Pahl, W.M.

1981-08-01

255

Computer tomographic investigation of ancient Egyptian mummies  

International Nuclear Information System (INIS)

Radiological and computer tomographic examinations of Egyptian mummies have been carried out at the Institute of Anthropology and Human Genetics from 1975 to 1978. These have demonstrated the value of CT in medical archaeology. It enables one to study the soft tissues, the skin (if bandaged), the muscles and any organs retained in situ for magical or religious reason. Measurements of attenuation values indicate the materials which were used for mummifying the skin and organs. Characteristic examples are described and the early results of these examinations are discussed. (orig.)

256

National Conference on Findings of Egyptian Maternal and Child Health Survey (7-8 September 1992).  

Science.gov (United States)

A national conference was held September 7-8, 1992, by the Central Agency for Mobilization and Statistics to discuss the findings of the Egyptian Maternal and Child Health Survey. The infant mortality rate (IMR) declined from 116 deaths/1000 live births during 1971-75 to 56/1000 during 1986-89. Residence and mother's education had major effects upon IMR. The prevention and control of diarrheal illness could further reduce the rates of infant and child mortality. The survey emphasizes the importance of promoting the awareness of the need for immunization, especially among rural mothers, and stresses the importance of providing medical care during pregnancy and at delivery to the health of both children and mothers. Increasing educational opportunities have contributed to the decline in early marriage and the upward trend in age at first marriage. Compared to urban residents, a higher proportion of rural residents marry their first cousins, while dissolution of marriage is due to either death or divorce. There is an inverse relationship between level of education and completed and complete fertility rates. 63% of women stated their desire to have 2-3 children as the ideal family size, with rural upper Egyptian women tending to report the highest ideal number of children. Egyptian women prefer a combination of sons and daughters with a higher tendency for son preference. There is almost universal knowledge of contraception in Egypt, with the IUD and oral pills the methods used most often by Egyptian women. Approximately 61% of urban women and 38% of rural women were using contraception at the time of the survey. Reasons for future non-use of contraception were the desire to bear more children, fatalism, being infecund, husband's disapproval, and the fear of contraceptives' side effects. PMID:12179787

1993-01-01

257

Applications of external PIXE to ancient Egyptian artefacts  

Energy Technology Data Exchange (ETDEWEB)

The external Proton Induced X-Ray Emission (PIXE) facility at Royal Melbourne Institute of Technology has been used to analyse ancient Egyptian glass samples, an Egyptian wall paint fragment and soil pigments. A 0.35 mm diameter beam of 1.6 MeV protons, extracted from the vacuum through an 8 {mu}m gold coated Kapton foil was used. Analysis of the spectra was carried out with the analysis package PIXAN. The analysis of two Egyptian glass samples enabled the partial determination of the colouring transition metals and the manufacturing technique, indicating them to be consistent with `New Kingdom` glasses. Extended abstract. 14 refs.,3 figs.

Moser, M.; Bubb, I.F.; Johnston, N.; El Bouanani, M.; Stannard, W.B.; Short, R.C. [Royal Melbourne Institute of Technology, Melbourne, (Australia). Department of Applied Physics

1998-06-01

258

Applications of external PIXE to ancient Egyptian artefacts  

International Nuclear Information System (INIS)

The external Proton Induced X-Ray Emission (PIXE) facility at Royal Melbourne Institute of Technology has been used to analyse ancient Egyptian glass samples, an Egyptian wall paint fragment and soil pigments. A 0.35 mm diameter beam of 1.6 MeV protons, extracted from the vacuum through an 8 ?m gold coated Kapton foil was used. Analysis of the spectra was carried out with the analysis package PIXAN. The analysis of two Egyptian glass samples enabled the partial determination of the colouring transition metals and the manufacturing technique, indicating them to be consistent with 'New Kingdom' glasses

259

Modeling and Collective Painting in an Egyptian Primary School.  

Science.gov (United States)

The author's art instruction for young Egyptian children focuses on design, creative expression, and appreciation of Egypt's unique artistic tradition. This article is one of several in this issue on art education in other countries. (SJL)

El Bassiouny, Mahmoud

1980-01-01

260

Genetic drift evolution under vaccination pressure among H5N1 Egyptian isolates  

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Full Text Available Background The highly pathogenic H5N1 is a major avian pathogen that intensively affects the poultry industry in Egypt even in spite of the adoption of vaccination strategy. Antigenic drift is among the strategies the influenza virus uses to escape the immune system that might develop due to the pressure of extensive vaccination. H5N1 mutates in an intensified manner and is considered a potential candidate for the possible next pandemic with all the catastrophic consequences such an eventuality will entail. Methods H5N1 was isolated from the pooled organ samples of four different affected flocks in specific pathogen free embryonated chicken eggs (SPF-ECE. A reverse transcriptase polymerase chain reaction (RT-PCR was performed to the haemagglutingin and neuraminidase. Sequencing of the full length haemagglutingin was performed. Sequence analyses of the isolated strains were performed and compared to all available H5N1 from Egyptian human and avian strains in the flu database. Changes in the different amino acid that may be related to virus virulence, receptor affinity and epitope configuration were assigned and matched with all available Egyptian strains in the flu database. Results One out of the four strains was found to be related to the B2 Egyptian lineage, 2 were related to A1 lineage and the 4th was related to A2 lineage. Comparing data obtained from the current study by other available Egyptian H5N1 sequences remarkably demonstrates that amino acid changes in the immune escape variants are remarkably restricted to a limited number of locations on the HA molecule during antigenic drift. Molecular diversity in the HA gene, in relevance to different epitopes, were not found to follow a regular trend, suggesting abrupt cumulative sequence mutations. However a number of amino acids were found to be subjected to high mutation pressure. Conclusion The current data provides a comprehensive view of HA gene evolution among H5N1 subtype viruses in Egypt. Egyptian H5N1-AIVs are constantly undergoing genetic changes and reveal a complex pattern of drifts. These findings raise the concerns about the value of using influenza vaccines in correlation with the development of antigenic drift in influenza epidemics.

Afifi Manal A

2011-06-01

261

Did the ancient Egyptians migrate to ancient Nigeria?  

OpenAIRE

Literatures concerning the history of West African peoples published from 1900 to 1970 debate the possible migrations of the Egyptians into West Africa. Writers like Samuel Johnson and Lucas Olumide believe that the ancient Egyptians penetrated through ancient Nigeria but Leo Frobenius and Geoffrey Parrinder frowned at this opinion. Using the works of these early 20th century writers of West African history together with a Yoruba legend which teaches about the origin of their earliest ancesto...

Agai, Jock M.

2014-01-01

262

Genetic distance and heterogenecity between quasispecies is a critical predictor to IFN response in Egyptian patients with HCV genotype-4  

OpenAIRE

Abstract Background HCV is one of the major health problems in Egypt, where it is highly prevalent. Genotype 4 is the most common genotype of HCV and its response to treatment is still a controversy. Methods HCV genotype 4 quasispecies diversity within the 5' untranslated region (5'UTR) was studied in a series of 22 native Egyptian patients with chronic hepatitis C virus with no previous treatment who satisfied all NIH criteria for combined treatment of pegylated IFN and ribavirine and was co...

Fouad Inas; Omar Ashraf; Khaled Mohsen M; Bahnassy Abeer A; El-Din Hanaa; Zekri Abdel; El-Hefnewi Mahmoud; Thakeb Fouad; El-Awady Mostafa

2007-01-01

263

Higher Education Externalities in Egyptian Labor Markets  

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Full Text Available Augmenting a Mincerian earnings function with governorate level data, this paper estimated the external return to higher education for individuals in Egypt in 2010. The results suggested that these externalities are negative and exist only for female workers, while for males these externalities were again negative but statistically insignificant. A unit increase in governorate average higher education is associated with a 68% decrease in females’ hourly wage. This could be explained by the fact that education degrees are simply used as a device to signal higher ability without raising productivity. Another reason could be excess supply of higher education graduates in the Egyptian labor market. These results have been tested through a number of robustness checks. Results survived to the introduction of individual and governorate level variables; it is not due to imperfect substitutability across workers; it still holds when treating local human capital as endogenous variable and instrumented it.

Hanan Nazier

2013-03-01

264

Examination of an Egyptian mummy - stereolithography applied  

International Nuclear Information System (INIS)

This paper describes the techniques of three dimensional imaging and stereolithography based on serial CAT-scans applied to the examination of the skull of an Egyptian mummy. Both the three dimensional image and the polymeric cast of the mummy skull presented finer details. It was confirmed that the subject was a male, approximately 30 - 35 years old. Fracturing of the ethmoid bone, e=sequelae to the removal of the brain, was observed in both types of presentations. Apart from this and signs of parodontitis, no pathology was observed. Stereolithography is a most powerful, non-destructive approach to the study of mummies. It might solve some of the problems of reburials, and further be of value in forensic medicine and paleo-ontology. (authors)

265

Radiation levels in ancient Egyptian mummies  

International Nuclear Information System (INIS)

Radiation levels were studied in the mummies room and some galleries in the Egyptian museum as well as in the medical museum of the Faculty of Medicine, Cairo University. ''gamma'' and ? radiation levels show almost background values in direct contrast to some mummies in the closed mummies room and inside the glass cases used for protection. Radon decay products in the room atmosphere and inside the glass cases enclosing some mummies show slightly higher levels than the background which were attributed to inefficient ventilation. After reasonable ventilation during the work these levels approached the normal values. High resolution ?-ray spectroscopy measurements for the aerosol samples on the filters have shown background values. The results indicated that the mummies do not contain any radioactivity content or any radioactive sources as was previously suggested. (Author)

266

Radiation levels in ancient Egyptian mummies  

Energy Technology Data Exchange (ETDEWEB)

Radiation levels were studied in the mummies room and some galleries in the Egyptian museum as well as in the medical museum of the Faculty of Medicine, Cairo University. ``gamma`` and {beta} radiation levels show almost background values in direct contrast to some mummies in the closed mummies room and inside the glass cases used for protection. Radon decay products in the room atmosphere and inside the glass cases enclosing some mummies show slightly higher levels than the background which were attributed to inefficient ventilation. After reasonable ventilation during the work these levels approached the normal values. High resolution {gamma}-ray spectroscopy measurements for the aerosol samples on the filters have shown background values. The results indicated that the mummies do not contain any radioactivity content or any radioactive sources as was previously suggested. (Author).

Hussein, M.I.; Hussein, A.Z.; Barakat, M.F. [Atomic Energy Authority, Cairo (Egypt). National Center for Nuclear Safety and Radiation Control; Nakhla, S.; Iskander, N. [Egyptian Antiquities Organization (Egypt)

1994-07-01

267

Radiation levels in ancient Egyptian mummies  

Science.gov (United States)

Radiation levels were studied in the mummies room and some galleries in the Egyptian museum as well as in the medical museum of the Faculty of Medicine, Cairo University.? and ? radiation levels show almost background values in direct contrast to some mummies in the closed mummies room and inside the glass cases used for protection. Radon decay products in the room atmosphere and inside the glass cases enclosing some mummies show slightly higher levels than the background which were attributed to inefficient ventilation. After reasonable ventilation during the work, these levels apprached the normal values.High resolution ?-ray spectroscopy measurements for the aerosol samples on the filters have shown background values.The results indicated that the mummies do not contain any radioactivity content or any radioactive sources as was previously suggested.

Hussein, Mohamed I.; Hussein, A. Z.; Barakat, M. F.; Nakhla, S.; Iskander, N.

1994-07-01

268

Attitude of Egyptian consumer towards irradiated food  

International Nuclear Information System (INIS)

This study aims at the evaluation of the opinion and attitude of the consumer as to what extent they accept or refuse food preservation by radiation. Also detect the method that can attract the consumers to adopt the technique and ensure the success handling of irradiated in egyptian market. One thousand and twenty two poll sheets were collected. The questionnaire was supported with simplified information about the use of atomic energy and radiation for peaceful purpose. From the results, 62.43% of the total sample size accepted the radiation technology persons that were convinced with the advantage of using irradiated food reached 70.45% . As to keep on being applied of the technology 73.97% of the total sample size agreed persons said yes to irradiated food for consumption if it is made available in the market were 57.53%

269

Zoonotic Chicken Toxoplasmosis in Some Egyptians Governorates  

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Full Text Available Toxoplasmosis is one of the most common diseases prevalent in the world, caused by a coccidian parasite Toxoplasma gondii which infects humans, animals and birds. Poultry consider reliable human source of food in addition it is considered an intermediate host in transmission of the disease to humans. Trails of isolation of local T. gondii chicken strain through bioassay of the suspected infected chicken tissues in mice was carried out and the isolated strain was confirmed as being T. gondii using Polymerase Chain Reaction (PCR. Seroprevalence of antibodies against T. gondii in chicken sera in six Egyptian governorates were conducted by enzyme linked immune-sorbent assay (ELISA using the isolated chicken strain antigen. Moreover, comparison between the prevalence rates in different regions of the Egyptian governorates were been estimated. Isolation of local T. gondii chicken strain was accomplished from chicken tissues and confirmed by PCR technique. The total prevalence rate was 68.8% comprised of 59.5, 82.3, 67.1, 62.2, 75 and 50% in El Sharkia, El Gharbia, Kafr El sheikh, Cairo, Quena and Sohag governorates, respectively. The prevalence rates were higher among Free Range (FR (69.5% than commercial farm Chickens (C (68.5%; while, the prevalence rate was less in Upper Egypt than Lower Egypt governorates and Cairo. This study is the first was used antigen from locally isolated T. gondii chicken strain for the diagnosis of chicken toxoplasmosis. The higher seroprevalence particularly in free range chickens (house-reared refers to the public health importance of chickens as source of zoonotic toxoplasmosis to human.

Ehab Kotb El-Mahllawy

2012-01-01

270

Enhancement of the folate content in Egyptian pita bread  

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Full Text Available Introduction: Egypt has a high incidence of neural tube defects related to folate deficiency. One major food source for folate is pita (baladi bread, which is consumed daily. Bioprocessing (e.g. germination has been reported to increase the folate content in cereals. The aim was to produce pita bread with increased folate content using germinated wheat flour (GWF.Methods: Prior to milling the effects of germination and drying conditions on folate content in wheat grains were studied. Pita bread was baked from wheat flour substituted with different levels of GWF. The folate content in dough and bread and rheological properties of dough were determined.Results: Germination of wheat grains resulted in, depending on temperature, 3- to 4-fold higher folate content with a maximum of 61 µg/100 g DM (dry matter. The folate content in both flour and bread increased 1.5 to 4-fold depending on the level of flour replacement with GWF. Pita bread baked with 50% sieved GWF was acceptable with respect to colour and layer separation, and had a folate content of 50 µg/100 g DM compared with 30 µg/100 g DM in conventional pita bread (0% GWF.Conclusion: Using 50% GWF, pita bread with increased folate content, acceptable for the Egyptian consumer, was produced. Consumption of this bread would increase the average daily folate intake by 75 µg.

Cornelia M. Witthöft

2012-04-01

271

Colorectal cancer in Egyptian patients under 40 years of age.  

Science.gov (United States)

Although colorectal cancer is not a common cancer in Egypt, the age distribution of the disease shows that a high proportion occurs in children and adults under 40 years of age. We reviewed the records of 1,608 colorectal cancer patients treated in 4 cancer hospitals in Egypt during a period of 3 to 10 years. The hospitals in which about 85% of all colorectal cancer cases in Egypt were seen included Egypt's 2 major cancer centers, The National Cancer Institute (NCI) in Cairo and Tanta Cancer Center (TCC) in the mid-Nile Delta region, and 2 major university hospitals, Assiut University in South Egypt and Ain Shams University in Cairo. Our review showed that patients younger than 40 years represented 35.6% of all patients in the 4 cancer hospitals, and that these rates were similar among the hospitals and for the years reviewed. The male-to-female ratio increased from 1.0 to 1.7 for the age groups ranging from 0-9 and 30-39 years, and increased from 1.0 to 1.5 for the age groups ranging from 40-49 to over 60 years. More than half of all the patients had rectal tumors, and about 90% of the cancers were adenocarcinomas; 30.6% of patients younger than 40 years, compared with 13.8% of older patients, had mucin-producing tumors. This study confirmed the occurrence of a high colorectal cancer rate in young Egyptians, and it opens the door to future epidemiologic studies to identify causes and risk factors of this disease pattern in Egypt. PMID:9096661

Soliman, A S; Bondy, M L; Levin, B; Hamza, M R; Ismail, K; Ismail, S; Hammam, H M; el-Hattab, O H; Kamal, S M; Soliman, A G; Dorgham, L A; McPherson, R S; Beasley, R P

1997-03-28

272

Aflatoxin B1 in common Egyptian foods.  

Science.gov (United States)

Samples of common Egyptian foods (17 nuts and seeds, 10 spices, 31 herbs and medicinal plants, 12 dried vegetables, and 28 cereal grains) were collected from markets in Cairo and Giza. A portion of each sample was extracted with chloroform, and the concentrated extract was cleaned by passing through a silica gel column. Aflatoxin B1 was determined by reversed-phase liquid chromatography with UV detection. The highest prevalence of aflatoxin B1 was in nuts and seeds (82%), followed by spices (40%), herbs and medicinal plants (29%), dried vegetables (25%), and cereal grains (21%). The highest mean concentration of aflatoxin B1 was in herb and medicinal plants (49 ppb), followed by cereals (36 ppb), spices (25 ppb), nuts and seeds (24 ppb), and dried vegetables (20 ppb). Among nuts and seeds, the prevalence of aflatoxin B1 was highest (100%) in watermelon seeds, inshell peanuts, and unshelled peanuts. The lowest prevalence and concentrations were in hommos (garbanzo beans). The highest concentrations of aflatoxin B1 were detected in foods that had no potential for field contamination but required drying during processing and storage, such as pomegranate peel, watermelon seeds, and molokhia. PMID:8823921

Selim, M I; Popendorf, W; Ibrahim, M S; el Sharkawy, S; el Kashory, E S

1996-01-01

273

Social media in the 2011 Egyptian uprising.  

Science.gov (United States)

This paper uses Gallup poll data to assess two narratives that have crystallized around the 2011 Egyptian uprising: (1) New electronic communications media constituted an important and independent cause of the protests in so far as they enhanced the capacity of demonstrators to extend protest networks, express outrage, organize events, and warn comrades of real-time threats. (2) Net of other factors, new electronic communications media played a relatively minor role in the uprising because they are low-cost, low-risk means of involvement that attract many sympathetic onlookers who are not prepared to engage in high-risk activism. Examining the independent effects of a host of factors associated with high-risk movement activism, the paper concludes that using some new electronic communications media was associated with being a demonstrator. However, grievances, structural availability, and network connections were more important than was the use of new electronic communications media in distinguishing demonstrators from sympathetic onlookers. Thus, although both narratives have some validity, they must both be qualified. PMID:24798232

Brym, Robert; Godbout, Melissa; Hoffbauer, Andreas; Menard, Gabe; Zhang, Tony Huiquan

2014-06-01

274

The Egyptian legislation for safe transportation of radioactive materials  

International Nuclear Information System (INIS)

According to the Egyptian legislation related the safe transport of radioactive materials, a licence is required for the transport, import and or export these materials. The licence is granted, upon a written application to NCNSRC-AEA. All the procedures and conditions for granting the NCNSRC-AEA licence to handle/ transport radioactive materials/wastes have been developed according to the international and Egyptian legislation. The procedures for transit of ships carrying radioactive materials in Suez Canal are also constructed. The NCNSRC-AEA experts are entitled to accept or to refuse the transit of ships carrying radioactive materials in the Suez Canal, in the Egyptian regional waters, in the sea harbours or in the exclusive economic zones of Egypt according to the national and international regulations. (author)

275

Antidiarrhoeal activity of some Egyptian medicinal plant extracts.  

Science.gov (United States)

The antidiarrhoeal activity of six Egyptian medicinal plant extracts (200 and 400 mg kg(-1)) and their effect on motility of isolated rabbit's duodenum was investigated. Phytochemical screening of the plant extracts for their active constituents was also carried out by TLC. Oral administration of methanol extract from Conyza dioscoridis (CD) or Alhagi maurorum (AM) in a 200 mg kg(-1) dose exhibits a significant antidiarrhoeal effect against castor oil-induced diarrhoea, while Mentha microphylla (MM), Convolvulus arvensis (CA), Conyza linifolia (CL) produced no significant effect. In a dose of 400 mg kg(-1), Mentha microphylla, Conyza dioscoridis, Alhagi maurorum, Zygophyllum album (ZA), and Conyza linifolia produced a significant (Parvensis produced no antidiarrhoeal effect in rats. Methanol extract of Mentha microphylla, Conyza dioscoridis, Zygophyllum album, and Convolvulus arvensis induced a dose-dependent (0.4-2.8 mg ml(-1)) relaxation of rabbit's duodenal smooth muscle. Alhagi maurorum and Conyza linifolia increased the contractile force in concentrations between 0.4 and 1.6 mg ml(-1). Higher concentrations (>3.2 mg ml(-1)) caused a rapid depressant effect. The depressant effect induced by Alhagi maurorum (in a higher dose) and Zygophyllum album appeared to be due to calcium channel blocking effect, since CaCl(2) could not restore the contractile response of the tissue impregnated in calcium free-medium. However, a ganglionic blocking effect appeared to be a possible mechanism of action of Mentha microphylla and Conyza dioscoridis since a stimulant dose of nicotine could not restore the contractile response of the tissue. The effect of Convolvulus arvensis and Conyza linifolia was not through any of the common mediators. Phytochemical screening revealed the presence of tannins, flavonoids, unsaturated sterols/triterpenes, carbohydrates, lactones and proteins/amino acids as major constituents. PMID:15138016

Atta, Attia H; Mouneir, Samar M

2004-06-01

276

MENDELIAN SUSCEPTIBILITY TO MYCOBACTERIAL DISEASE IN EGYPTIAN CHILDREN  

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Full Text Available

Background: Tuberculosis remains a major health problem in developing countries especially with the emergence of multidrug resistant strains. Mendelian Susceptibility to Mycobacterial Disease (MSMD is a rare disorder with impaired immunity against mycobacterial pathogens. Reported MSMD etiologies highlight the crucial role of the Interferon gamma /Interleukin 12 (IFN-g/ IL-12 axis and the phagocyte respiratory burst axis.

Purpose: Screen patients with possible presentations for MSMD.

Methods: Patients with disseminated BCG infection following vaccination, atypical mycobacterial infections or recurrent tuberculosis infections were recruited from the Primary Immune Deficiency Clinic at Cairo University Specialized Pediatric Hospital, Egypt and immune and genetic laboratory investigations were conducted at Human Genetic of Infectious Diseases laboratory in Necker Medical School, France from 2005-2009. IFN-g level in patient’s plasma as well as mutations in the eight previously identified MSMD-causing genes were explored.

Results: Nine cases from eight (unrelated kindreds were evaluated in detail. We detected a high level of IFN-g in plasma in one patient. Through Sanger sequencing, a homozygous mutation in the IFNGR1 gene at position 485 corresponding to an amino acid change from serine to phenylalanine (S485F, was detected in this patient.

Conclusion: We report the first identified cases of MSMD among Egyptian patients, including in particular a new IFNGR1 mutation underlying IFN-gR1 deficiency. The eight remaining patients need to be explored further. These findings have implications regarding the compulsory Bacillus Calmette Guerin vaccination policy in Egypt, especially given the high consanguinity rate.

Keywords: Interferon gamma axis, mycobacterium tuberculosis, BCG, consanguinity

Nermeen Galal

2012-01-01

277

Early Hepatic Complication in First Year after Bone Marrow Transplantation in Major Beta Thalassemic Patients  

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Full Text Available Introduction: Bone marrow transplantation is a good therapeutic modality for beta thalassemia. Liver complications are one of the major causes of morbidity and mortality following BMT. Determination of the factors of liver injury leads to earlier diagnosis after BMT and improves prognosis. Method: We studied 113 major Beta thalassemic patients who have been transplanted from 1990- 2000 in bone marrow transplantation center of Shariati Hospital. 62 were male and 51 were female. 27 pa¬tients were class one, 56 were class two and 30 were class three. The median age of each class were 6.5, 6.3 and 8.7. Conditioning regimen consisted of busulfan (3.5-4mg/Kg and cyclophophamide (40-50mg/Kg.For GVHD prophylaxis we gave cyclosporine ± metothoroxate. Grade of liver fibrosis de¬fined by biopsy in all patients before BMT. All patients and their donors tested for HBSAg, HBSAb, HCVAb, CMVAb with RIA method. We assessed causes of liver dysfunction before and after trans¬plantation and effect of high ferritin level on liver function."nResults: Hepatic dysfunction in first year after transplantation was seen in 86 (76% patients. Causes of liver dysfunction were consisted of 53.1% GVHD, 15.93% cyclosporine hepatotoxicity, 7.07% condi¬tioning regimen hepatotoxicity and VOD. In all three classes hepatic GVHD, cyclosporine toxicity, death and normal liver function post BMT had significant relation with hepatic dysfunction before BMT (P=0.001. In patients with ferritin level more than 1000, there were significant hepatotoxicity with conditioning regimen (P=0.001. 17 (15.04% of patients have been died. Discussion: According to our study hepatic GVHD (%53.1 is the most common cause of hepatic dys¬function in all three classes.

Iravani M

2005-07-01

278

Pictorial Metaphor in Selected Egyptian Newspapers Cartoons during the 25th of January 2011 Egyptian Revolution.  

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Full Text Available Metaphor is persuasive in having cognitive, emotional and aesthetic effect. It has been argued that any visual representation can be considered a metaphor if it represents a metaphoric thought. Chartris-Black’s Critical Metaphor Analysis and Forceville’s model of pictorial metaphor are both used to analyze the data under investigation. Chateris-Black’s Critical Metaphor Analysis is used based on the idea that the use of metaphors helps in conveying the underlying ideologies of the writers. Moreover, Forceville’s model of pictorial metaphor aims at investigating various types of pictorial metaphor in different genres. The political cartoons investigated in this study are drawn from three independent newspapers: Al Dastour, Al Masry Al Youm and Al Youm AL Sabea during the 25th of January 2011 Egyptian Revolution. This study aims at exploring how metaphors are expressed in the visual mode, more specifically in newspapers cartoons, tracing the use of pictorial metaphor in selected independent newspapers cartoons during the eighteen days of the 25th of January 2011 Egyptian revolution, and finally, identifying the cartoonists’ underlying ideological motivations and their attitudes to the revolution, which ultimately direct the audience’s ideology. The results of this study suggest that Chateris-Black’s Critical Metaphor Analysis can be applied not only on the textual level, but also on the semiotic and pictorial ones. In addition, metaphors are frequently used in political cartoons to evoke strong emotive effect and reinforce ideologies to achieve persuasion. Keywords: Pictorial Metaphor, Critical Metaphor Analysis, political cartoon

Ghada Abdel Aziz Ashmawi

2014-03-01

279

Parenting Style, Individuation, and Mental Health of Egyptian Adolescents  

Science.gov (United States)

Three questionnaires that measure parenting style, adolescent-family connectedness, and mental health were administered to 351 Egyptian adolescents. Results show that in rural communities the authoritarian style is more predominant in the parenting of male adolescents, while the authoritative style is more predominant in the parenting of female…

Dwairy, Marwan; Menshar, Kariman E.

2006-01-01

280

The Case for (Social) Entrepreneurship Education in Egyptian Universities  

Science.gov (United States)

Purpose: The purpose of this paper is to explore awareness of social entrepreneurship amongst Egyptian students and to determine what is needed to create more graduate social entrepreneurs. Design/methodology/approach: The theoretical framework is Ajzen's Theory of Planned Behavior. Data collection is a questionnaire survey of 183 of the 2,000…

Kirby, David A.; Ibrahim, Nagwa

2011-01-01

281

Egyptian Film: Gender and Class Violence Three Cycles.  

Science.gov (United States)

Examines the level of physical and verbal violence by gender and social class in Egyptian films in three cycles: romantic musicals and melodramas; war and political genres; and drug and gangster films. Concludes that the outrageous level of violence does not accurately reflect the real society. (Contains 20 references.) (LRW)

Al-Obaidi, Jabbar A.

2000-01-01

282

Evaluation of municipal solid waste management in egyptian rural areas.  

Science.gov (United States)

A two years study was conducted to evaluate the solid waste management system in 143 villages representing the Egyptian rural areas. The study covers the legal responsibilities, service availability, environmental impacts, service providers, financial resources, private sector participation and the quality of collection services. According to UN reports more than 55% of Egyptian population lives in rural areas. A drastic change in the consumption pattern altered the quantity and quality of the generated solid wastes from these areas. Poor solid waste management systems are stigmata in most of the Egyptian rural areas. This causes several environmental and health problems. It has been found that solid waste collection services cover only 27% of the surveyed villages, while, the statistics show that 75% of the surveyed villages are formally covered. The service providers are local villager units, private contractors and civil community associations with a percentage share 71%, 24% and 5% respectively. The operated services among these sectors were 25%, 71% and 100% respectively. The share of private sector in solid waste management in rural areas is still very limited as a result of the poverty of these communities and the lack of recyclable materials in their solid waste. It has been found that direct throwing of solid waste on the banks of drains and canals as well as open dumping and uncontrolled burning of solid waste are the common practice in most of the Egyptian rural areas. The available land for landfill is not enough, pitiable designed, defectively constructed and unreliably operated. Although solid waste generated in rural areas has high organic contents, no composting plant was installed. Shortage in financial resources allocated for valorization of solid waste management in the Egyptian rural areas and lower collection fees are the main points of weakness which resulted in poor solid waste management systems. On the other hand, the farmer's participation in solid waste management through the composting of organic matter and using of food waste as an animal feed are considered strength points. However, throwing of solid waste on the banks of water streams, open dumping and uncontrolled burning of solid waste are environmental damaging behaviors that need to be changed. Integrated solid waste management in the Egyptian rural areas is not yet among the priorities of the Egyptian government. PMID:19712653

El-Messery, Mamdouh A; Ismail, Gaber A; Arafa, Anwaar K

2009-01-01

283

Diagnostic Value of Fructosamine and Glycosylated Hemoglobin in Estimating Blood Glucose Level in Diabetic Patients with Thalassemia Major  

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Full Text Available Background and Objective: Diabetes mellitus is one of complications that thalassemia major patients face with. Hence, blood glucose monitoring is of vital importance to these patients. Because of high level of fetal hemoglobin in these patients, the measurement of hemoglobin A1c is not reliable and should be displaced by fructosamine test. Material and Methods: The current descriptive study was carried out on 33 beta-thalassemia major patients afflicted with diabetes mellitus (21 female and 12 male cases. Blood glucose level, fructosamine, hemoglobin A1c, serum ferritin and fetal hemoglobin were measured. Results: Blood glucose levels are 204±103 mg/dL and 221±101 mg/dL (p=0.63; fetal hemoglobin levels are 9%±7% and 13%±9% (p=0.22; serum ferritin levels are 1744±1534 ng/mL and 3253±1773 ng/mL (p=0.96 in female and male patients, respectively. The level of fructosamine (42±124 mmol/L and glycosylated hemoglobin (8.9%±1.8% are correlated significantly (r=0.69, p<0.01. Both Hemoglobin A1c (r=0.75, p<0.01 and fructosamine (r=0.54, p<0/01 show a significant correlation with blood glucose level. Conclusion: In diabetic thalassemia major patients with frequent blood transfusion, the level of fructosamine and glycosylated hemoglobin are related significantly, therefore; they can be used alternatively. Keywords: Thalassemia major; Fructosamine; Hemoglobin A1c; Diabetes Mellitus

Kosaryan, M. (MD

2013-01-01

284

An investigation into the ancient Egyptian cultural influences on the Yorubas of Nigeria  

OpenAIRE

There are many cultural practices that connect ancient Egyptians to the Yorubas and the new interpretation of the Oduduwa legend suggests that the Yorubas have originated or are influenced mainly by the Egyptians. The attestation of Egypt as the main influencer of the Yoruba culture made Egypt significant in the study of the history of the Yoruba people. Some writers are beginning to think that the ancient Egyptians were responsible for introducing and spreading many cultures amongst the Yoru...

Agai, Jock M.

2013-01-01

285

Egyptian youth and the European Eldorado: Journeys of hope and despair  

OpenAIRE

In this paper, I explore characteristics of Egyptian irregular migrants to Europe and reasons of irregular migration from the point of departure through a field survey in some Egyptian villages known of sending irregular – as well as regular - migrants to Italy and France (mainly). The fieldwork was carried out in eight Egyptian governorates to identify the push factors in the country, with particular attention to the dynamics governing the irregular migratory flows from Egypt to the EU. Th...

Zohry, Ayman

2006-01-01

286

Adopting knowledge discovery in databases for customer relationship management in egyptian public banks  

OpenAIRE

We propose a framework for studying the effect of KDD on CRM in the Egyptian banking sector. We believe that the KDD process and applications may perform a significant role in Egyptian banks to improve CRM, in particular for customer retention. Our believe is supported by the results of the field survey at the largest Egyptian bank. Keywords. Adopting new technology, Knowledge Discovery in Databases (KDD), Customer Relationship Management (CRM), and banking sector.

Kok, Joost; Khedr, Ayman

2006-01-01

287

The implementation of world class manufacturing techniques in Egyptian manufacturing firms: An empirical study  

OpenAIRE

Purpose - The purposes of this paper are to illustrate how the world class manufacturing (WCM) techniques which could be described as outperforming the industry's global best practices have been implemented in the Egyptian manufacturing firms, to identify the critical driving and resisting forces toward WCM techniques implementation in Egyptian manufacturing firms, and to provide guidelines for the successful implementation of WCM by Egyptian manufacturers. Design/methodology/approach - The d...

Salaheldin, S. I.; Eid, R.

2007-01-01

288

Discrimination Capacity of RAPD, ISSR and SSR Markers and of their Effectiveness in Establishing Genetic Relationship and Diversity among Egyptian and Saudi Wheat Cultivars  

OpenAIRE

Problem statement: Yield crop cultivars and landraces are valuable sources of genetic variations that the knowledge and implication of these variations are critical in the plant breeding programs. our major objective of this study is investigating the discriminating capacity of RAPD, ISSR and SSR markers and of their effectiveness in establishing genetic relationship and diversity among Egyptian and Saudi wheat cultivars. Approach: Eleven wheat cultivars and landraces collected from Egypt and...

El-assal, Salah E. D.; Ahmed Gaber

2012-01-01

289

Evaluation of Diet Quality of Egyptian Children and Adolescents using Healthy Eating Index  

Directory of Open Access Journals (Sweden)

Full Text Available Background: Healthful eating is essential for development and well-being. Some dietary patterns are associated with 4 of the 10 leading causes of death (coronary heart disease, certain types of cancer, stroke, and type 2 diabetes. Major improvements in the health of the public can, therefore, be made by improving people's dietary patterns. The U.S. department of Agriculture has developed an index, called the Healthy Eating Index (HEI that was updated in the year 2002. It is based on different aspects of a healthful diet; the Index is designed to provide a measure of overall dietary quality, and the compliance with specific Dietary Guidelines recommendations. The aim of our study is to use the healthy eating index to assess the diet quality of a representative sample of the Egyptian children and adolescents and to examine the association between body mass index and caloric intake of the studied sample. Materials & Methods: This study is a part of National Egyptian survey, Diet, Nutrition and Prevention of Chronic Non-communicable Diseases. The Healthy Eating Index was applied with slight modifications to measure how well the studied Egyptian students' diet conforms to recommended healthy eating pattern. The data were based on representative sample (2145 of children and adolescents (10 -18 years in 7 governorates from. One day of dietary intake data (24 hours recall was collected, during an in-person interview. The Healthy Eating Index measures how well the studied children and adolescents' diets conform to the American Dietary Guidelines recommendations and the Food Guide Pyramid applied in our country. Ten dietary components have been identified and the overall Index has a total possible score ranging from zero to 100. Results: Our results showed that the average Healthy Eating Index score was 59.1 out of a possible 100 and it ranged from 20 to 86, Only 0.5 percent of the students had Healthy Eating scores above 80; while 16.9 percent of them received scores below 50 and the majority (82.5 percent had scores on the Healthy Eating Index between 51 and 80. In an effort to provide a "rating" of the overall student's diet, a grading scale was developed, the majority of students had diets rated as "Needs Improvement", only 0.5 % received diets rated as "Good" and 16.9 % had diets rated as "Poor". Males achieved a slightly higher average Index than females (59.7 Vs 58.2. The average score for food groups is much lower than that for dietary guidelines (23.5 Vs 35.6 out of total score of 50 for each. There was a significant positive correlation of BMI with caloric intake for male adolescents while, for females the correlation was insignificant and negative. More than 80 percent of the sample achieved the recommendations of the American Dietary Guidelines for total fat and cholesterol. Less than two-thirds of the students met the recommendations for saturated fat, Almost 30 percent of the students had the maximum score for sodium. Only 1.0 percent of them received a score of 10 for fibers. Conclusion: the majority of Egyptian children and adolescent' eating patterns, as measured by the HEI, need improvement. The results of the Index are useful in targeting nutrition education and health promotion activities, as it is a single summary measure of diet quality that can be used to monitor changes in food consumption patterns over time. A Food Quality System based on nutrient density can be one tool that can facilitate more healthful food purchases and dietary patterns.

Mervat A. Esmail ­ Wafaa M.A.Saleh ­Nebal A.R.Aboul Ella ­ Asmaa M. Abd

2009-12-01

290

Multielement determination in some egyptian vegetables by instrumental neutron activation analysis  

International Nuclear Information System (INIS)

Nondestructive instrumental neutron activation analysis (INAA) technique, with thermal neutrons, has been applied for multielement determination of major, trace and ultra trace elements in eleven types of the public public egyptian, edible vegetables, namely dill, moulokhyia, okra negro bean, parsley, green pea, grape leaves, spinach, mint, celery and salad chervil, cultivated and collected from El-Maadi, Cairo, E G. Concentrations of Na, K, Ca, Sc, Cr, Fe, Co, Ni, Zn, Rb, Zr, Nb, Mo, Sb, Cs, Ba, La, Ce, Tb, Yb, Hf, Ta, Th and U were determined. The standard reference materials (SRM's) G-2, J G-1 and MAG-1, provided from IAEA, were used, and high accuracy of the work was assured. The results were discussed

291

The Egyptian Revolution: First Impressions from the Field  

Directory of Open Access Journals (Sweden)

Full Text Available Never has a revolution that seemed so lacking in prospects gathered momentum so quickly and so unexpectedly. The Egyptian Revolution, starting on January 25, lacked leadership and possessed little organization; its defining events, on Friday, January 28, occurred on a day when all communication technologies, including all internet and phones, were barred; it took place in a large country known for sedate political life, a very long legacy of authoritarian continuity, and an enviable repressive apparatus consisting of more than 2 million members. But on that day, the regime of Hosni Mubarak, entrenched for 30 years and seemingly eternal, the only regime that the vast majority of the protesters had ever known, evaporated in one day.Though the regime struggled for two more weeks, practically little government existed during that period. All ministries and government offices have been closed, and almost all police headquarters were burned down on January 28. Except for the army, all security personnel disappeared, and a week after the uprising, only a few police officers ventured out again. Popular committees have since taken over security in the neighborhoods. I saw patriotism expressed everywhere as collective pride in the realization that people who did not know each other could act together, intentionally and with a purpose. During the ensuing week and a half, millions converged on the streets almost everywhere in Egypt, and one could empirically see how noble ethics—community and solidarity, care for others, respect for the dignity of all, feeling of personal responsibility for everyone—emerge precisely out of the disappearance of government.Undoubtedly this revolution, which is continuing to unfold, will be the formative event in the lives of the millions of youth who spearheaded it in Egypt, and perhaps also the many more millions of youth who followed it throughout the Arab world. It is clear that it is providing a new generation with a grand spectacle of the type that had shaped the political consciousness of every generation before them in modern Arab history. All those common formative experiences of past generations were also grand national moments: whether catastrophic defeats or triumphs against colonial powers or allies.

Mohammed A. Bamyeh

2011-02-01

292

Egyptian Islamists and the Status of Muslim Women Question  

Directory of Open Access Journals (Sweden)

Full Text Available This paper will explore the gender discourse of contemporary Egyptian Islamists and argue that their gender discourse is not merely a religious and traditional discourse, but that this politico-religious Islamic ideology articulates a quite modern construct of gender equality. The gender discourse of a number of important Egyptian Islamists, al-Banna’, Qutb, al-Ghazali, al-Qaradawi and Ezzat will provide illustrations of these modern developments. Modern elements incorporated in today’s Islamist revivalist approaches create new understandings, neither purely traditional, nor purely modern, that are ‘modern constructs’ that attempt to remain traditional, while integrating specifically modern components. The presence of these two seemingly opposing and contradictory elements may account for the present popularity that Islamist discourses enjoy in many Muslims countries.

Roxanne D. Marcotte

2005-08-01

293

Electrical properties and mineralogical investigation of Egyptian iron ore deposits  

Energy Technology Data Exchange (ETDEWEB)

Electrical properties and X-ray diffraction of 20 hematite sandstone samples of the most economically interesting Egyptian iron ore deposits have been investigated. Samples were collected from two different areas in Egypt (Aswan and Bahariya). Complex impedance measurements in the frequency range from 10 Hz to 100 kHz were performed at room temperature ({approx}20 deg. C). The observed dielectric behavior was characterized by Maxwell-Wagner interfacial polarization at low frequencies and bulk polarization at relatively higher frequencies. The frequency dependence on conductivity shows a classical relaxation behavior followed Jonscher's universal law. The measured electrical properties vary strongly with the frequency and sample composition. The difference in the electrical properties may be attributed to the fluctuations in the concentration of the sample constituents and to the degree of heterogeneity of the grains. The XRD-patterns of Egyptian iron ore deposits prove that the main phases are hematite and quartz.

Gomaa, M.M. [National Research Centre, Geophysical Sciences Dep., El Behooth St., 12622 Dokki, Cairo (Egypt)], E-mail: mmmsgomaa@yahoo.com; Shaltout, A.A.; Boshta, M. [National Research Center, Physics Division, El Behooth St., 12622 Dokki, Cairo (Egypt)

2009-03-15

294

An Ancient Egyptian Diagonal Star Table in Mallawi, Egypt  

Science.gov (United States)

A coffin belonging to an Egyptian Middle Kingdom official Hor-em-hetepu, on public display in the Mallawi Monuments Museum, Egypt, contains a previously-unpublished diagonal star table (or "diagonal star clock"). This table adds to the other twenty-four examples of this type of astronomical record or calendar from around 2100 B.C. The table displays a regular diagonal pattern of decan (star or asterism) names, with some interesting points of content, epigraphy, and typology.

Symons, Sarah; Cockcroft, Robert

2013-11-01

295

Quantitative analysis of ancient Egyptian pigments by external PIXE  

International Nuclear Information System (INIS)

Pigments painted on Egyptian excavations in the 18th Dynasty were analyzed successfully by external PIXE with the aid of the X-ray diffraction. A white pigment was composed on Mg3Ca(CO3)4; red: ?Fe2O3, ?FeO.OH and AsS; pink: Mixtures of white and red pigments; yellow: ?FeO.OH and As2S3; and blue: CaO.CuO.4SiO2. (orig.)

296

Biodiversity of Bacterial Ecosystems in Traditional Egyptian Domiati Cheese?  

OpenAIRE

Bacterial biodiversity occurring in traditional Egyptian soft Domiati cheese was studied by PCR-temporal temperature gel electrophoresis (TTGE) and PCR-denaturing gradient gel electrophoresis (DGGE). Bands were identified using a reference species database (J.-C. Ogier et al., Appl. Environ. Microbiol. 70:5628-5643, 2004); de novo bands having nonidentified migration patterns were identified by DNA sequencing. Results reveal a novel bacterial profile and extensive bacterial biodiversity in Do...

El-baradei, Gaber; Delacroix-buchet, Agne?s; Ogier, Jean-claude

2006-01-01

297

Facial reconstruction of a wrapped Egyptian mummy using MDCT  

OpenAIRE

OBJECTIVE: Facial reconstruction of mummies and corpses in general is important in anthropological, medical, and forensic studies. The purpose of our study was to evaluate the role of MDCT examination for 3D facial reconstruction and report the results of multidisciplinary work performed by radiologists, anthropologists, and forensic police in reconstructing the possible physiognomy of an ancient Egyptian mummy. MATERIALS AND METHODS: Three-dimensional MDCT data were obtained from a well-pres...

Gandini, Giovanni; Boano, Rosa

2004-01-01

298

New Terpenes from the Egyptian Soft Coral Sarcophyton ehrenbergi  

Directory of Open Access Journals (Sweden)

Full Text Available Chemical investigations of the Egyptian soft coral Sarcophyton ehrenbergi have led to the isolation of compounds 1–3 as well as the previously reported marine cembranoid diterpene sarcophine (4. Structures were elucidated by comprehensive NMR and HRMS experimentation. Isolated compounds were in vitro assayed for cytotoxic activity against human hepatocarcinoma (HepG2 and breast adenocarcinoma (MCF-7 cell lines.

Ahmed Elkhateeb

2014-04-01

299

Barriers to E-Commerce Adoption in Egyptian SMEs  

OpenAIRE

E-commerce has been predicted to be a new driver of economic growth for developing countries. The SME sector plays a significant role in its contribution to the national economy in terms of the wealth created and the number of people employed. Small and Medium Enterprises (SMEs) in Egypt represent the greatest share of the productive units of the Egyptian economy and the current national policy directions address ways and means of developing the capacities of SMEs. Many factors could be resp...

Zaied, Abdel Nasser H.

2012-01-01

300

Does Facebook Matter in Egyptian Graduate Environment? A Marketing Perspective  

OpenAIRE

The current study is exploratory, and it aims at uncovering the potential role of Facebook in Egyptian university environment. The research based on 127 questionnaires. It was found that; 42% of the respondents use Facebook for educational purpose; around 85% use Facebook to be in touch with their teachers; more than 75% use Facebook to contact the faculty administration; more than 70% of students are convinced that they have added value when using Facebook in the educational context. These r...

Ahmad Yahia Ebeid

2012-01-01

301

Strategic Leadership and Its Application in Egyptian Universities  

OpenAIRE

Today's universities operate in a climate of great change, along with increased responsibilities and accountability from Internal and external customers. This has resulted in calls for a new kind of leadership working to help the university to improve educational services and face more challenges, called strategic leadership, at the university level. Aim of study defining of Egyptian leadership universities pattern of modern leadership styles, named as Strategic leadership (concept, objective...

Alalfy, Hany R.

2014-01-01

302

Synthesis of Cellulose Acetate Membrane from the Egyptian Rice Straws  

OpenAIRE

In Egypt, approximately 4.5 million tons from 35 million tons of the annual production of recoverable cereal are disposed by burning and it creates a big problem. Isolation of cellulose with high yield and purity is a long-standing goal in cellulose development because of the complexity of cell wall structure of rice straw. Chemical composition of Egyptian rice straw was determined (moisture, ash, LMWC, wax and protein) and total carbohydrates. Cellulose was extracted by different alkaline Na...

Ragab, Tamer I. M.; Wasfy, A. A. F.; Amer, H.; Ayman El-Gendi; Abdel-hady, M. S.; Liebner, F.

2014-01-01

303

The Egyptian Revolution: First Impressions from the Field  

OpenAIRE

Never has a revolution that seemed so lacking in prospects gathered momentum so quickly and so unexpectedly. The Egyptian Revolution, starting on January 25, lacked leadership and possessed little organization; its defining events, on Friday, January 28, occurred on a day when all communication technologies, including all internet and phones, were barred; it took place in a large country known for sedate political life, a very long legacy of authoritarian continuity, and an enviable repressiv...

Bamyeh, Mohammed A.

2011-01-01

304

Unwrapping an Ancient Egyptian Mummy Using X-Rays  

Science.gov (United States)

This article describes a project of unwrapping an ancient Egyptian mummy using x-ray computed tomography (CT). About 600 x-ray CT images were obtained through the mummified body of a female named Tjetmutjengebtiu (or Jeni for short), who was a singer in the great temple of Karnak in Egypt during the 22nd dynasty (c 945-715 BC). The x-ray CT images…

Hughes, Stephen W.

2010-01-01

305

Apelin Serum Level in Egyptian Patients with Chronic Hepatitis C  

OpenAIRE

Objective. Highlighting the apelin system would present a new therapeutic target for liver disease. Apelin; endogenous ligand for the orphan receptor APJ, was recently suggested to be associated with fibrosis progression and cirrhosis in addition to insulin resistance (IR) and inflammation. The present study was conducted to evaluate blood apelin level changes among 73 chronic hepatitis C (CHC) Egyptian patients and if associated with body mass index (BMI), IR, and tumor necrosis factor-alpha...

Abdel-Rahman El-Zayadi; Rizk, Hanan H.; El-mesallamy, Hala O.; Hamdy, Nadia M.

2011-01-01

306

HOW EGYPTIAN CONTRACTING COMPANIES TRANSLATE PROJECTS' QUALITY TO PROJECTS' PERFORMANCE  

OpenAIRE

A model for continuous quality improvement for Egyptian contracting projects’ (CQIM) is developed through the extensive reviewing of the Total Quality Management (TQM), Continuous Quality Improvement (CQI)literature, preceding researches, consensus opinions of managers and experts of the construction industry and the quality management system (ISO 9000). This model comprises of 13 main factors divided into 9 CQI constructs and 4 Indices, the Indices are broken down to 46 practical measures,...

Shawky Hozien, Said; REFAAT HASSAN ABDEL-RAZEK; ADEL AHMED EL-KORDI; SHAFIK SHAWKY KHOURY

2011-01-01

307

Political Unrest Costs Egyptian Tourism Dearly: An Ethnographical Study  

OpenAIRE

This study uses ethnographical research methodology to observe the negative impact of political unrest on the tourism industry and overall economy of Egypt during the nation’s revolution. In the paper, a number of proactive strategies are suggested to insulate Egypt from future acts of violence. Emphasis on the implementation of a destination management system is also recommended to enhance overall knowledge and efficiency in the Egyptian tourism sector and to help alleviate negative turns ...

Mohamed Ahmed Nassar

2012-01-01

308

Did the ancient Egyptians migrate to ancient Nigeria?  

Directory of Open Access Journals (Sweden)

Full Text Available Literatures concerning the history of West African peoples published from 1900 to 1970 debate the possible migrations of the Egyptians into West Africa. Writers like Samuel Johnson and Lucas Olumide believe that the ancient Egyptians penetrated through ancient Nigeria but Leo Frobenius and Geoffrey Parrinder frowned at this opinion. Using the works of these early 20th century writers of West African history together with a Yoruba legend which teaches about the origin of their earliest ancestor(s, this researcher investigates the theories that the ancient Egyptians had contact with the ancient Nigerians and particularly with the Yorubas. Intradisciplinary and/or interdisciplinary implications: There is an existing ideology amongst the Yorubas and other writers of Yoruba history that the original ancestors of the Yorubas originated in ancient Egypt hence there was migration between Egypt and Yorubaland. This researcher contends that even if there was migration between Egypt and Nigeria, such migration did not take place during the predynastic and dynastic period as speculated by some scholars. The subject is open for further research.

Jock M. Agai

2014-07-01

309

Physical inactivity among Egyptian and Saudi medical students  

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Full Text Available Objectives: Medical students, the future doctors, were presumed to be knowledgeable about physical activity and would have future influence on their patients. This study aims to describe the pattern of physical activity, predictors of physical inactivity and perceived barriers to and benefits of physical activity among a sample of Egyptian and Saudi medical students. Methods: A cross-sectional comparative study was carried out on 319 Egyptian and 297 Saudi medical students. The long form of the international physical activity questionnaire (IPAQ was used to measure physical activity. Data was analyzed according to the guidelines for data processing and analysis of the IPAQ. Perceived barriers to and potential benefits of physical activity were reported. Results: Physical inactivity was significantly higher among Saudi than Egyptian medical students (41.1% versus 15.4%, respectively. Logistic regression analysis revealed that the independent predictors of physical inactivity were non-membership in sports clubs (OR =4.6 and use of private cars for transportation (OR=3.9. The most frequent barriers to physical activity are time limitation due to busy study schedule and lack of accessible and suitable sporting places. More than 70% of students perceived that physical activity promotes and maintains health. Conclusions: Because time and access are key barriers to medical student exercise, we believe that provision of free playgrounds in the college to practice sports during free times will promote physical activity in students. [TAF Prev Med Bull 2011; 10(1.000: 35-44

Abdel-Hady El-Gilany

2011-02-01

310

Interferon therapy shifts natural killer subsets among egyptian patients with chronic hepatitis C  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: English Abstract in english Natural killer cells can be divided into five subpopulations based on the relative expression of CD16 and CD56 markers. The majority of natural killer cells are CD56dim, which are considered to be the main cytotoxic effectors. A minority of the natural killer cells are CD56bright, and function as an [...] important source of immune-regulatory cytokines. Shifts of these subsets have been reported in patients with chronic hepatitis C virus infection. We sought to investigate the shift of natural killer subsets among Egyptian patients with chronic HCV and to analyze the influence of interferon therapy on this shift. We applied a flow cytometric analysis of peripheral blood natural killer subsets for 12 interferon-untreated and 12 interferon-treated patients with chronic HCV, in comparison to 10 control subjects. Among interferon-untreated patients, there was a significant reduction of CD56-16+ (immature natural killer) cells. Among interferon-treated patients, the absolute count of natural killer cells was reduced, with expansion of the CD56bright subset and reduction of the CD56dim16+ subset. Natural killer subset counts were not significantly correlated to HCV viral load and were not significantly different among interferon responders and non-responders. In conclusion, HCV infection in Egyptian patients has been observed to be statistically and significantly associated with reduction of the CD56-16+NK subset, while a statistically significant expansion of CD56bright and reduction of CD56dim16+ subsets were observed after interferon therapy. Further studies are required to delineate the molecular basis of interferon-induced shift of natural killer subsets among patients with HCV.

Amal, Fathy; Mohamed Mohy, Eldin; Lobna, Metwally; Mohamed, Eida; Marwa, Abdel-Rehim; Gamal, Esmat.

2010-08-01

311

Egypt: A Simulation of Ancient Egyptian Civilization.  

Science.gov (United States)

This simulation allows students to travel north through the six divisions of the Nile River, with each major phase of the simulation called a "cataract". Students earn travel points as they sail the Nile from its source in Central Africa through the Sixth Cataract, all the way to the First Cataract, and then on to the delta where it flows into the…

Sargent, Marcia; Malcolm, Vivian

312

Premature epiphyseal fusion and extramedullary hematopoiesis in thalassemia  

Energy Technology Data Exchange (ETDEWEB)

The main skeletal abnormalities in ..beta..-thalassemia are widening of medullary spaces, rarefaction of bone trabeculae, thinning of cortical bone, and perpendicular periosteal spiculation. Premature epiphyseal fusion (PEF) and extramedullary hematopoiesis (EH) are found, though more rarely. The incidence of PEF and EH in 64 patients affected by ..beta..-thalassemia is reported. The different incidence of such complications in thalassemia major and intermedia is reported, and a possible correlation with transfusion regimen is also considered.

Colavita, N.; Orazi, C.; Danza, S.M.; Falappa, P.G.; Fabbri, R.

1987-10-01

313

Tolerance, quality and storability of gamma-irradiated Egyptian rice  

International Nuclear Information System (INIS)

The effect of gamma irradiation on some organoleptic and physico-chemical properties and the storability of Egyptian rice was investigated. Radiation up to 50krad was chosen as an adequate dose causing non-significant changes in eating and cooking qualities. The effect of irradiation on degradation of starch and protein molecules is demonstrated on the basis of studies on the viscosity and solubility of rice paste. Irradiation at relatively low dose levels up to 50krad did not affect the chemical and nutritional qualities of rice regarding amino acids and B vitamins. It was also found that irradiation maintains better storability of rice under ambient temperature. (author)

314

Tolerance, Quality and Storability of Gamma-Irradiated Egyptian Rice  

International Nuclear Information System (INIS)

The effect of gamma irradiation on some organoleptic and physico-chemical properties and the storability of Egyptian rice was investigated. Radiation up to 50 krad was chosen as an adequate dose causing non-significant changes in eating and cooking qualities. The effect of irradiation on degradation of starch and protein molecules is demonstrated on the basis of studies on the viscosity and solubility of rice paste. Irradiation at relatively low dose levels up to 50 krad did not affect the chemical and nutritional qualities of rice regarding amino acids and B vitamins. It was also found that irradiation maintains better storability of rice under ambient temperature. (author)

315

Genetic Variability of Antigen B among Echinococcus granulosus Egyptian Isolates  

OpenAIRE

Genetic polymorphisms of encoding antigen B2 gene (AgB2) in Echinococcus granulosus were studied using PCR-RFLP and DNA sequencing among 20 Egyptian isolates. Five isolates from different host origins (humans, camels, pigs, and sheep) were collected and used. All examined isolates of each host group gave very similar patterns of PCR-RFLP after restriction enzyme digestion with AluI, with the gene size of approximately 140 bp and 240 bp for sheep and human isolates, and approximately 150 bp an...

Tawfeek, Gihan M.; Elwakil, Hala S.; Awad, Nabil S.; El-hoseiny, Laila; Thabet, Hala S.; Sarhan, Rania M.; Darweesh, Samar K.; Anwar, Wagida A.

2009-01-01

316

Mitochondrial Mutation In Egyptian Patients With Type 2 Diabetes Mellitus  

Directory of Open Access Journals (Sweden)

Full Text Available Mitochondrial gene mutation plays a role in the development of diabetes mellitus. An A to G substitution at base pair 3243 in the mitochondrial tRNAleu(UUR gene (mt3243 is commonly associated with maternally inherited diabetes and deafness and other diseases. The aim of this study is to detect A to G substitution at base pair 3243 in mitochondrial RNAleu(UUR in the plasma of patients with type 2 diabetes mellitus, and to evaluate insulin sensitivity in all cases. This study, included 41 patients (Group I, 31 cases with type 2 diabetes mellitus and maternal history of diabetes mellitus-and Group II, 10 cases with type 2 diabetes mellitus, bilateral SNHL, maternal history of diabetes mellitus with or without SNHL. Other10 healthy control group was included. Patients and controls were subjected to full medical history and clinical examination. Serum measurements for liver and kidney function tests, fasting and postprandial blood glucose as well as C-peptide levels, in addition to lipid profile were collected. Audiological evaluation for all patients with SNHL was also done. Genetic investigation, for mDNA analysis, done by polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP, to determine the mutation in the mitochondrial gene at position 3243. Results of the study showed that glycemic indices (FPG, 2hPPG and HbA1c, liver enzymes and blood urea were significantly higher among patient group compared to control group (P<0.05. There was no significant difference for values of creatinine and uric acid between cases and controls. Lipid profile was significantly higher among patient group compared to controls (P<0.05, except for HDL-C which was higher in controls however, it did not reach statistical significance. C-peptide values were not significantly different between studied groups. Age at onset of diabetes was relatively earlier in group II than group I. mDNA was present in all plasma samples of patients and controls. mDNA 3243 mutation was detected in the plasma of three patients with diabetes and SNHL with a rate of 7.3% of all diabetic patients and 30% of diabetic patients associated with deafness. The presence of mDNA mutation allowed 294 bp product to be cleaved into 180 and 114 bp fragments and were seen as two bands. In CoclusioN: The A 3243 G mutation is present in Egyptian population and is considered as a cause of maternally inherited diabetes and deafness at a rate of 7.3% of all diabetic subjects and a rate of 30% of diabetics associated with deafness. mDNA mutation is present and detectable in plasma. Maternally inherited diabetes and deafness differs pathophysiologically from the more common forms of type 2 diabetes in that, insulin resistance does not seem to be a major factor.

Fawzi O,A*; Hassan Z,A*; Abdel Kawy S,I**; Al-Diwany O,I**; Adel

2006-06-01

317

Do Corporate Governance Disclosures Matter for Bank Cost of Capital? Empirical Evidence from Accounting Statements of Egyptian Banks  

Directory of Open Access Journals (Sweden)

Full Text Available The purpose of this study is to examine the association between the quality of bank governance mechanisms disclosed in bank annual reports and cost of capital. The Egyptian banking sector has undergone a series of legislative reforms starting with the issuance of the 2003 banking law. The law incorporates the guidelines of the Basel Accords and governance principles, and was declared a major step forward into facing global banking competition and driving financial growth in Egypt. We create two multivariate cross-sectional, time-series regression models to test this relation. Our main results show that there is a highly significant relation between bank governance disclosures and cost of capital. Banks with reported large board size and more executive directors on board are able to obtain finance from cheaper resources. This indicates that cost of equity of Egyptian banks is not just related to accounting performance and risk but also related to how well a bank is governed. Furthermore, the cost of deposits decreases significantly for banks reporting better governance mechanisms.

Mona A. Elbannan

2014-11-01

318

Growth, physiological adaptation, and gene expression analysis of two Egyptian rice cultivars under salt stress.  

Science.gov (United States)

Abiotic stressors, such as high salinity, greatly affect plant growth. In an attempt to explore the mechanisms underlying salinity tolerance, physiological parameters of two local Egyptian rice (Oryza sativa L.) cultivars, Sakha 102 and Egyptian Yasmine, were examined under 50 mM NaCl stress for 14 days. The results indicate that Egyptian Yasmine is relatively salt tolerant compared to Sakha 102, and this was evident in its higher dry mass production, lower leaf Na(+) levels, and enhanced water conservation under salt stress conditions. Moreover, Egyptian Yasmine exhibited lower Na(+)/K(+) ratios in all tissues examined under salinity stress. The ability to maintain such traits seemed to differ in the leaves and roots of Egyptian Yasmine, and the root K(+) content was much higher in Egyptian Yasmine than in Sakha 102. In order to understand the basis for these differences, we studied transcript levels of genes encoding Na(+) and K(+) transport proteins in different tissues. In response to salinity stress, Egyptian Yasmine showed induction of expression of some membrane transporter/channel genes that may contribute to Na(+) exclusion from the shoots (OsHKT1;5), limiting excess Na(+) entry into the roots (OsLti6b), K(+) uptake (OsAKT1), and reduced expression of a Na(+) transporter gene (OsHKT2;1). Therefore, the active regulation of genes related to Na(+) transport at the transcription level may be involved in salt tolerance mechanisms of Egyptian Yasmine, and these mechanisms offer the promise of improved salinity stress tolerance in local Egyptian rice genotypes. PMID:25532120

Mekawy, Ahmad Mohammad M; Assaha, Dekoum V M; Yahagi, Hiroyuki; Tada, Yuma; Ueda, Akihiro; Saneoka, Hirofumi

2015-02-01

319

Chemical composition of water hyacinth (Eichhronia Crassipes) a comparison indication of heavy metal pollution in egyptian water bodies. Vol. 4  

International Nuclear Information System (INIS)

Water hyacinth is tested as an indicator for pollution in egyptian fresh surface waters. Chemical composition of water hyacinth as affected area of collection (water bodies) was studied and the suitability of this plant as a biological indicator for water pollution is discussed. Water hyacinth samples were collected three times per year for two years (1991-1993). Sample sites include one location in the river nile (at Helwan area), one site in Ismaillia canal, (at Mostrod industrial area), and one site in Abo-Zabal drain (at Abo-Zabal city). The concentration of 19 major major and trace elements in plant samples were determined by prompt gamma-ray neutron activation analysis. Results indicated that plant parts as well as location have a significant effect on elements content. Water hyacinth roots showed high affinity for accumulation of trace elements. 5 tabs

320

Legal Elements For Nuclear Security: Egyptian Nuclear Law As A Case Study  

International Nuclear Information System (INIS)

This paper deals with the legal bases for nuclear security. First, It analysis the international legal framework for nuclear security. Second, it analysis the legal bases for the import-export control. The legal aspects related with illicit trafficking (IT) were also reviewed. Third, It deals with the Egyptian nuclear law no. 7 and its executive regulation. The Egyptian legal regime for nuclear security and the role of State System for Accounting and Control of Nuclear Materials (SSAC) in realizing the nuclear security were also discussed. The purpose of the paper is to evaluate the Egyptian legal framework for nuclear security.

321

A study of the prevalence of thalassemia and its correlation with liver function test in different age and sex group in the Chittagong district of Bangladesh.  

Science.gov (United States)

Thalassemia is the name of a group of genetic, inherited disorders of the blood. More specifically, it is a disorder of the hemoglobin molecule inside the red blood cells. According to World health Organization (WHO), there are about 3% beta-thalassemia carrier and about 4% Hb E/beta-thalassemia carrier in Bangladesh. Our objective is to identify the prevalence of beta-thalassemia in our adolescent populations and to review risk factors that would most easily identify a subset of adolescent patients at greatest risk for the development of beta-thalassemia. We also made a study of clinical profile of 53 thalassemic patients, observing the relationship between the patients with their verity ages and sex. The cases are taken on the basis of their age (2-30 years), beta-thalassemia major, clinical jaundice with history of chronic blood transfusion. The cases excluded those who had jaundice due to viral hepatitis or hepatitis due to heavy metal poisoning (Arsenic) and those with spleenectomy. Liver function test has been evaluated in 53 patients. That were recorded with some relevant demographical data such as age, sex, blood group where median age was of 16 years and mean (±SD) age 15.4151 ± 7.90918. Among them were 21 (39.6%) female and 32 (60.4%) male. With an average 15.1% (8 in no.) beta-thalassemia, 7.5% (4 in no.) beta-thalassemia major and 77.4% (41 in no.) E-beta-thalassemia cases have been found in the study. Mean (±SD) TSB in total 53 subjects with age group 2-10 years and 21-30 years is significant. The study revealed that in thalassemic patients when the age is more, the disease progresses with their complication. Hepatic complication is mainly due to being hepatocellular in nature than that of obstructive one. PMID:24826050

Palit, Sarmi; Bhuiyan, Robiul H; Aklima, Jannatul; Emran, Talha B; Dash, Raju

2012-09-01

322

Determination of Wax Content in Egyptian Crude Oils  

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Full Text Available Wax precipitation is one of the most important flow assurance problems. Unfortunately, experimental data are very scarce to confirm existing models for prediction of such production impairment and other hazardous risk; while its curative approaches and production losses add to colossal economic sabotage to the petroleum industry. The present work deals with studying the determination of wax in Egyptian crude oil by gravimetric determination precipitation with lowering temperature of the crude oil .The crude oil studied was from Egyptian western desert (waxy paraffinic crude. Waxes may precipitate due to a temperature decrease because their solubility is so low that a solid phase can appear. The experiments investigated the effect of initial weight of the crude, the time of cooling, and the temperature on the amount of wax produced. The highest amount of wax was produced with 150gm of the crude oil, at temperature of 0oC and this was done within two hours of the time of experiment. Finally the effect of using solvent on the amount wax content was also studied and it was found that the ratio (3:1 of solvent mixture increases achievement of the maximum amount of wax produced.

Prof.Dr.Ramadan Abu El-Ella

2014-02-01

323

Penile length-somatometric parameters relationship in healthy Egyptian men.  

Science.gov (United States)

This study aimed to assess the penile length-somatometric parameters relationship in healthy Egyptian men. Two thousand physically normal men (22-40 years) were subjected to measurement of stretched penile length, glans penis, testis size, index finger, weight, height, span, body mass index (BMI), waist circumference, hip circumference and waist/hip ratio. The mean stretched penile length of the studied subjects was 13.84 ± 1.35 cm (range 12-19 cm), and the mean glans penis length was 2.6 ± 0.4 cm (range 1.7-3.8 cm). Penile length demonstrated positive significant correlation with glans penis length, index finger length, BMI and significant negative correlation with waist/hip ratio. On the other hand, penile length demonstrated nonsignificant correlation with age, weight, height, waist circumference, span or testicular size. It is concluded that the penile length-somatometric parameters relationship in healthy Egyptian men is mostly related to glans penis and index finger lengths. PMID:24698122

Shalaby, M E; Almohsen, A E-R M; El Shahid, A R; Abd Al-Sameaa, M T; Mostafa, T

2014-04-01

324

Antidiabetic effects of a standardized Egyptian rice bran extract.  

Science.gov (United States)

An extract was prepared from Egyptian stabilized rice bran and standardized to contain 2% ?-oryzanol in addition to its content of other bioactives, notably tocotrienol and policosanol. The standardized extract was found to have a concentration-dependent effect on insulin release in vitro, which, however, is not mediated by ?-tocotrienol in rice bran (detected by HPLC) as could have been expected. Policosanol and ?-oryzanol have insulinotropic effects. The in vitro data of rice bran directly translate into in vivo data of rats by using a glucose tolerance test (increase in plasma insulin). Tocotrienols are well known for their apoptotic effect on tumor cells; nevertheless, an attempt was made to study glucose uptake in HEP-G2 cells, which needs to induce an insulin-resistant state by TNF-?. The Egyptian rice bran extract has an antidiabetic effect. ?-Oryzanol, which is a possible precursor of the insulinotropic compound ferulic acid, is a candidate for this effect. Therefore, it is reasonable to assume that the prevalence of diabetes or at least a prediabetic (type 2) situation can be ameliorated by the investigated rice bran extract. The potential usefulness of the extract as a nutraceutical is currently undergoing more thorough investigations. PMID:22566008

Kaup, Rebecca M; Khayyal, Mohamed T; Verspohl, Eugen J

2013-02-01

325

A bleaching earth from egyptian local deposits  

Directory of Open Access Journals (Sweden)

Full Text Available The present investigation deals with the bleaching of vegetable oils using activated clays collected from some deposits in Egypt as compared to Tonsil FF currently used by local oil industry. The comparison was made; not only on the basis of the decolourising power of the earth, but also on the basis of its effects on the oil acidity, formation of the oil peroxides and the decomposition rate of the formed peroxides to aldehydes and ketones during the bleaching process. The activation of the collected earth samples was made using 4N HCl, 6N HCl and 30 % H2SO4. The bleaching tests of the activated samples were performed using the major four oil types processed in Egypt being cottonseed, sunflower, soybean and palm oils. In addition to the laboratory-evaluation tests, the performance of the activated samples, which showed promise on the lab-scale have been also tested on an industrial scale. The industrial application has proved that the activated local earth's can be successfully used as bleaching earth of local oils. Thus it can be used as a substitute of the varieties currently imported and used by the local oil sector.La presente investigación trata de la decoloración de aceites vegetales usando tierras activadas obtenidas de yacimientos egipcios, comparándola con el Tonsil FF usado normalmente en la industria oleícola local. La comparación se realizó, no sólo sobre la base del poder decolorante de la tierra, sino también sobre la base de sus efectos en la acidez del aceite, la formación de peróxidos y la velocidad de descomposición de los peróxidos formados en aldehidos y cetonas durante el proceso de decoloración. La activación de las muestras de tierras recogidas se hizo utilizando ClH 4N, ClH 6N y H2SO4 30 %. Los tests de decoloración de las muestras activadas se llevaron a cabo usando los cuatro tipos mayoritarios de aceites procesados en Egipto: aceite de semilla de algodón, de girasol, de soja y de palma. Además de los tests a escala de laboratorio, la evaluación de las muestras activadas, se realizó a escala industrial. La aplicación industrial ha demostrado que las tierras locales activadas pueden ser utilizadas con éxito como tierras decolorantes de aceites locales. De este modo pueden usarse como sustitutos de las variedades mas frecuentemente importadas y usadas por el sector aceitero.

El Kinawy, Omayma S.

2001-10-01

326

Mithramycin encapsulated in polymeric micelles by microfluidic technology as novel therapeutic protocol for beta-thalassemia  

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Full Text Available Lorenzo Capretto1, Stefania Mazzitelli2, Eleonora Brognara2, Ilaria Lampronti2, Dario Carugo1, Martyn Hill1, Xunli Zhang1, Roberto Gambari2, Claudio Nastruzzi31Engineering Sciences, University of Southampton, Southampton, UK; 2Department of Biochemistry and Molecular Biology, 3Department of Pharmaceutical Sciences, University of Ferrara, Ferrara, ItalyAbstract: This report shows that the DNA-binding drug, mithramycin, can be efficiently encapsulated in polymeric micelles (PM-MTH, based on Pluronic® block copolymers, by a new microfluidic approach. The effect of different production parameters has been investigated for their effect on PM-MTH characteristics. The compared analysis of PM-MTH produced by microfluidic and conventional bulk mixing procedures revealed that microfluidics provides a useful platform for the production of PM-MTH with improved controllability, reproducibility, smaller size, and polydispersity. Finally, an investigation of the effects of PM-MTH, produced by microfluidic and conventional bulk mixing procedures, on the erythroid differentiation of both human erythroleukemia and human erythroid precursor cells is reported. It is demonstrated that PM-MTH exhibited a slightly lower toxicity and more pronounced differentiative activity when compared to the free drug. In addition, PM-MTH were able to upregulate preferentially ?-globin messenger ribonucleic acid production and to increase fetal hemoglobin (HbF accumulation, the percentage of HbF-containing cells, and their HbF content without stimulating ?-globin gene expression, which is responsible for the clinical symptoms of ß-thalassemia. These results represent an important first step toward a potential clinical application, since an increase in HbF could alleviate the symptoms underlying ß-thalassemia and sickle cell anemia. In conclusion, this report suggests that PM-MTH produced by microfluidic approach warrants further evaluation as a potential therapeutic protocol for ß-thalassemia.Keywords: microfluidics, lab-on-a-chip, design of experiments, erythroid differentiation, human erythroid precursor cells

Capretto L

2012-01-01

327

Unusual occurrence of dentoalveolar manifestations in a case with beta thalassemia trait.  

Science.gov (United States)

A case of thalassemia minor with dentoalveolar manifestations in a 22-year-old Iranian male is presented. On the basis of patient complaint of upper incisors mobility, radiographic examination was performed which revealed enlarged medullary space, thin lamina dura, prominent antegonial notch, spiky and short roots of upper incisors and a generalized root resorption. This is the second report of dentoalveolar changes in regard to a minor hemoglubinopathy disorder. In this case treatment was carried out with 1 mg per day oral folic acid for 2 months which relived patient complaint as well as some radiographic changes. PMID:24984659

Alireza, Farhad; Maryam, Allameh

2014-06-01

328

Effective RES blood flow changes in children with homozygous. beta. -thalassemia in relation to blood transfusion  

Energy Technology Data Exchange (ETDEWEB)

Denatured radioiodinated human serum albumin (DHA) clearance studies at a dose of 1 mg/kg body wt., were carried out in 16 thalassemic children, prior to and 7-10 days following blood transfusion, to investigate changes of the effective RES blood flow which might accompany the posttransfusion spleen size diminution. A statistically significant increase (P<0.001) of the DHA plasma clearance rate was observed 7-10 days following blood transfusion denoting an increase of the blood flow to the effective RES while at the same time the spleen diminished in size. It is suggested that changes in the effective RES blood flow in these patients are directly related to changes in the intrasplenic circulatory capacity.

Karpathios, T.; Dimitriou, P.; Giamouris, J.; Nicolaidou, P.; Antipas, S.E.; Matsaniotis, N.

1983-01-01

329

Microwave dielectric properties of human erythrocyte ghosts in normal and pathological state (homozygous beta-thalassemia)  

Energy Technology Data Exchange (ETDEWEB)

The microwave dielectric properties of human normal and homozygous ..beta..-thalassemic erythrocyte ghosts have been measured at the frequency of 10 GHz in the temperature interval from 5 to 50/sup 0/C. The permittivity and the dielectric loss have been analysed in terms of the Maxwell-Wagner equation for heterogeneous systems. The presence of a remarkable amount of interfacial water at the membrane surface has been evidentiated and some differences in the dielectric parameters of normal and pathological ghosts observed.

Ballario, C.; Bonincontro, A.; Cametti, C.; Rosi, A.; Sportelli, L.

1984-04-01

330

Effect of gamma irradiation on membranes of normal and pathological erythrocytes (beta-thalassemia)  

Energy Technology Data Exchange (ETDEWEB)

The influence of ionizing radiation on the membrane of human normal erythrocytes has extensively been studied and a variety of effects including changes in the cation fluxes or in non-electrolytes permeability, in membrane fluidity, in peroxidation of unsaturated lipids as well as chemical composition or structural modifications has been observed. However, only few studies deal with the effects of ionizing radiation on pathological red blood cells. In this work, we have investigated by means of electron spin resonance (ESR) spectroscopy the effects of /sup 60/Co ..gamma..-radiation on the normal and homozygous ..beta..-thalassemic human erythrocyte membranes.

Sportelli, L.; Rosi, A.; Bonincontro, A.; Cametti, C.

1987-03-01

331

Compound heterozygotes and beta-thalassemia: top-down mass spectrometry for detection of hemoglobinopathies.  

Science.gov (United States)

We have shown previously that liquid extraction surface analysis of dried blood spots coupled to high resolution top-down MS may be applied for the diagnosis of hemoglobin (Hb) variants FS, FAS, FC, FAC, FAD in newborn samples. The objective of the current work was to determine whether the structural variant HbE, compound heterozygote variants FSC and FSD, and ?-thalassemia were amenable to diagnosis by this approach. Anonymized residual neonatal dried blood spot samples, taken as part of the routine newborn screening program, were analyzed by liquid extraction surface analysis coupled to high resolution MS/MS. The samples had been previously screened and were known to be FAE, FSC, FSD, or ?-thalassemia. Manual analysis of the mass spectra revealed that, in all cases, the variants may be confirmed. Direct surface sampling MS should be considered as an alternative to current screening techniques for the diagnosis of Hb variants. PMID:24482221

Edwards, Rebecca L; Griffiths, Paul; Bunch, Josephine; Cooper, Helen J

2014-05-01

332

the characterization of exon-1 mutation(s) of beta globin gene in beta thalassemia  

International Nuclear Information System (INIS)

?-thalassemia constitutes one of the most serious health problems worldwide, it is the most common chronic hemolytic anemia in egypt. the aim of this work is to study the mutations of exon-1 of ?-globin gene in ?-thalassaemic children in sharkia governorate. the present study was included 25 healthy children and 50 patients diagnosed as ?-thalassemia. this work showed that the thalassaemic patients had significantly decrease in Hb conc . than the control group (p 2 showed a significant increase as compared with the control group

333

Prevalence of renal tubular dysfunction in beta thalassemia minor in shiraz  

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Full Text Available  Background & objective: ?-Thalassemia minor is an asymptomatic hereditary disease. The first study on the relation of renal tubular dysfunction and ?-thalassemia minor was performed in 2002 but those studies seem inadequate.The main goal of this study is through evaluation of renal tubular function in 100 patients with thalassemia minor. Materials & Methods: 100 patients with ?- thalassemia which confirmed by hemoglobin electrophoresis and CBC as well as RBC indices were studied.14 out of 100 cases exit because of Urinary Tract Infection, diabetes mellitus or hypertension.Complete chemistry profile was performed on serum and urine of all reminder 86 patients (46 female and 40 male. Patients classified into two groups: ?-thalassemia minor with anemia and without anemia. Another control group include 50 healthy individuals also considered.Then data analyzed by proper statistical methods. Results: 20 out of 86 reminder cases e.g. 24% showed at least one index of renal tubular dysfunction.58% of patients was been anemic and 42% non anemic. The most prominent tubular dysfunction was seen in a 29 years old lady with glucosuria and without anemia. conclusion: ?-Thalassemia minor is common in Iran specially in Fars province. This study revealed significant renal tubular dysfunction in patient with ?-thalassemia minor. So it is necessary to check out thalassemic patients for renal function tests periodically. Key words: ?-thalassemia, minor,renal tubular dysfunction

Ali Moradi Nakhodcheri

2012-02-01

334

Effect of gamma irradiation on membranes of normal and pathological erythrocytes (beta-thalassemia)  

International Nuclear Information System (INIS)

The influence of ionizing radiation on the membrane of human normal erythrocytes has extensively been studied and a variety of effects including changes in the cation fluxes or in non-electrolytes permeability, in membrane fluidity, in peroxidation of unsaturated lipids as well as chemical composition or structural modifications has been observed. However, only few studies deal with the effects of ionizing radiation on pathological red blood cells. In this work, we have investigated by means of electron spin resonance (ESR) spectroscopy the effects of 60Co ?-radiation on the normal and homozygous ?-thalassemic human erythrocyte membranes. (orig.)

335

Macrophages support pathological erythropoiesis in Polycythemia Vera and Beta-Thalassemia  

OpenAIRE

Regulation of erythropoiesis is achieved by integration of distinct signals. Among these, macrophages are emerging as erythropoietin-complementary regulators of erythroid development, particularly under stress conditions. We investigated the contribution of macrophages for physiological and pathological conditions of enhanced erythropoiesis. We utilized mouse models of induced anemia, Polycythemia vera and ?-thalassemia in which macrophages were chemically depleted. Our data indicate that ma...

Ramos, Pedro; Casu, Carla; Gardenghi, Sara; Breda, Laura; Crielaard, Bart J.; Guy, Ella; Marongiu, Maria Franca; Gupta, Ritama; Levine, Ross L.; Abdel-wahab, Omar; Ebert, Benjamin L.; Rooijen, Nico; Ghaffari, Saghi; Grady, Robert W.; Giardina, Patricia J.

2013-01-01

336

Hydroxycarbamide-Induced Changes in E/beta Thalassemia Red Blood Cells  

OpenAIRE

In thalassemia, fetal hemoglobin (HbF) augmentation with hydroxycarbamide (also known as hydroxyurea) is not always successful. The expected parallel effects on RBC membrane deformability, cell hydration and membrane phospholipid organization, all important for extending RBC life span and increasing Hb, have been infrequently examined. We analyzed these characteristics in 15 non-transfused E/? 0 thalassemia patients treated with HU (mean 10.2 months). Membrane deformability and cell hydratio...

Sylvia, Singer T.; Elliott, Vichinsky; Sandra, Larkin; Nancy, Olivieri; Nancy, Sweeters; Frans, Kuypers A.

2008-01-01

337

Hydroxycarbamide-induced changes in E/beta thalassemia red blood cells.  

Science.gov (United States)

In thalassemia, fetal hemoglobin (HbF) augmentation with hydroxycarbamide (also known as hydroxyurea) is not always successful. The expected parallel effects on red cell (RBC) membrane deformability, cell hydration, and membrane phospholipid organization, all important for extending RBC life span and increasing Hb, have been infrequently examined. We analyzed these characteristics in 15 nontransfused E/beta(0) thalassemia patients treated with HU (mean 10.2 months). Membrane deformability and cell hydration mildly improved in association with increased HbF levels approaching statistical significance (r = 0.51, P = 0.06). All measures improved considerably in splenctomized patients. These findings underscore the disappointing results of hydroxyurea treatment in clinical trials and the importance of examining the effect on RBC characteristics for the development and understanding of HbF-enhancing agents. PMID:18821710

Singer, Sylvia T; Vichinsky, Elliott P; Larkin, Sandra; Olivieri, Nancy; Sweeters, Nancy; Kuypers, Frans A

2008-11-01

338

An investigation into the ancient Egyptian cultural influences on the Yorubas of Nigeria  

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Full Text Available There are many cultural practices that connect ancient Egyptians to the Yorubas and the new interpretation of the Oduduwa legend suggests that the Yorubas have originated or are influenced mainly by the Egyptians. The attestation of Egypt as the main influencer of the Yoruba culture made Egypt significant in the study of the history of the Yoruba people. Some writers are beginning to think that the ancient Egyptians were responsible for introducing and spreading many cultures amongst the Yorubas. As more Yorubas are tracing their origins and the origins of their culture to ancient Egypt, this research investigates whether the Egyptians were the originators and the main spreaders of the afterlife culture in Yorubaland.

Jock M. Agai

2013-07-01

339

77 FR 8943 - Culturally Significant Objects Imported for Exhibition Determinations: “The Dawn of Egyptian Art”  

Science.gov (United States)

...Significant Objects Imported for Exhibition Determinations: ``The Dawn of...the objects to be included in the exhibition ``The Dawn of Egyptian Art,'' imported from abroad for temporary exhibition within the United States,...

2012-02-15

340

An investigation into the ancient Egyptian cultural influences on the Yorubas of Nigeria  

Scientific Electronic Library Online (English)

Full Text Available There are many cultural practices that connect ancient Egyptians to the Yorubas and the new interpretation of the Oduduwa legend suggests that the Yorubas have originated or are influenced mainly by the Egyptians. The attestation of Egypt as the main influencer of the Yoruba culture made Egypt signi [...] ficant in the study of the history of the Yoruba people. Some writers are beginning to think that the ancient Egyptians were responsible for introducing and spreading many cultures amongst the Yorubas. As more Yorubas are tracing their origins and the origins of their culture to ancient Egypt, this research investigates whether the Egyptians were the originators and the main spreaders of the afterlife culture in Yorubaland.

Jock M., Agai.

341

A Critical Discourse Analysis of Discursive (De-) Legitimation Construction of Egyptian Revolution in Persian Media  

OpenAIRE

This article seeks to explore discursive strategies applied in Fars News to represent the event of Egyptian revolution as a positive/legitimized action and Hosni Mubarak’s regime as the negative/delegitimized other. Van Leeuwen's (2008) model of legitimation is used to show how Fars News applies the legitimation discursive construction, including four main categories of ''authorization'', ''evaluation'', ''rationalization'', and ''mythopoesis'' to legitimize Egyptian revolution.  T...

Bahador Sadeghi; Vahid Jalali

2013-01-01

342

The Relationship between Employee Perceptions of Equity and Job Satisfaction in the Egyptian Private Universities  

OpenAIRE

The purpose of this study was to investigate the relationships between employee perception of equity and job satisfaction in the Egyptian private universities. Data were gathered using a face-to-face survey of 80 teaching staff members at three Egyptian universities. Findings revealed positive relationships between perceptions of equity, where a "motivator" was the outcome in the comparison, and job satisfaction. The study also revealed that there was no relationship between perceptions of eq...

Khalifa, Mohamed Hossam El-din; Truong, Quang

2010-01-01

343

Assessing judicial efficiency of Egyptian First Instance Courts: A DEA analysis  

OpenAIRE

Egypt started a recent judicial reform program in 2007, which can be considered the first ever since the establishment of the National Egyptian Judicial System in 1952. It focuses mainly on solving organizational problems within First Instance Courts (FIC), as they form the active cell of the Egyptian judicial system. However the efficiency of FICs is still doubtable to a large extent. This paper provides for the first time an efficiency analysis of 22 FICs in Egypt using the technique of Dat...

Elbialy, Nora; Garci?a-rubio, Miguel A.

2011-01-01

344

The impact of organizational characteristics on AMT adoption: A study of Egyptian manufacturers  

OpenAIRE

Purpose - This research seeks to empirically explore and examine the impact of organizational characteristics, i.e. firm size, type of production system, organizational design and type of ownership, on advanced manufacturing technology (AMTs) adoption in the Egyptian industrial sector; and to identify the similarities and dissimilarities of the nature of AMT adoption in Egypt compared to different contexts. Design/methodology/approach - The analysis was based on a survey of 200 Egyptian manuf...

Salaheldin, S. I.

2007-01-01

345

Cultivation and subgroup determination of human rotaviruses from Egyptian infants and young children.  

OpenAIRE

Primary African green monkey kidney cells were more sensitive than primary cynomolgus monkey kidney and MA104 cells for supporting the growth of human rotaviruses detected in diarrheal stools of Egyptian infants and young children. In attempts to characterize these Egyptian rotaviruses, only 31% of the strains tested in the form of fecal suspensions were identified as subgroup 1 or 2. After one passage in African green monkey kidney cells, 80% of the strains were identified as subgroup 1 or 2...

Naguib, T.; Wyatt, R. G.; Mohieldin, M. S.; Zaki, A. M.; Imam, I. Z.; Dupont, H. L.

1984-01-01

346

Cats of the Pharaohs: Genetic Comparison of Egyptian Cat Mummies to their Feline Contemporaries  

OpenAIRE

The ancient Egyptians mummified an abundance of cats during the Late Period (664 - 332 BC). The overlapping morphology and sizes of developing wildcats and domestic cats confounds the identity of mummified cat species. Genetic analyses should support mummy identification and was conducted on two long bones and a mandible of three cats that were mummified by the ancient Egyptians. The mummy DNA was extracted in a dedicated ancient DNA laboratory at the University of California – Davis, then ...

Kurushima, Jennifer D.; Ikram, Salima; Knudsen, Joan; Bleiberg, Edward; Grahn, Robert A.; Lyons, Leslie A.

2012-01-01

347

Application of Neutron Activation Analysis Technique for Gold Estimation in South Area Egyptian Mines  

International Nuclear Information System (INIS)

The study presents analytical results of gold concentrations of some Egyptian gold ores. Six samples collected from south eastern of Egyptian mines area have been analyzed using neutron activation analysis technique. The gamma-ray spectra obtained were measured by means of high resolution hyper-pure germanium detection system in conjunction with electronic and computerized multichannel analyzer. The results are compared with previous measurements obtained by another method

348

Epidemiological aspects of Budd-Chiari in Egyptian patients: A single-center study  

OpenAIRE

AIM: To describe the socio-demographic features, etiology, and risk factors for Budd-Chiari syndrome (BCS) in Egyptian patients. METHODS: Ninety-four Egyptian patients with confirmed primary Budd-Chiari syndrome were presented to the Budd-Chiari Study Group (BCSG) and admitted to the Tropical Medicine Department of Ain Shams University Hospital (Cairo, Egypt). Complete clinical evaluation and laboratory investigations, including a thrombophilia workup and full radiological assessment, were pe...

Mohammad Sakr; Eman Barakat; Sara Abdelhakam; Hany Dabbous; Said Yousuf; Mohamed Shaker; Ahmed Eldorry

2011-01-01

349

Egyptian greenhouse cultivation at a higher level with Dutch Technology ; Annual Report 2013  

OpenAIRE

The project ‘Egyptian greenhouse cultivation at a higher level with Dutch technology’ is co-funded under the Top Sector Programme Horticulture and Starting Materials. The project wants to realizes through the use of Dutch technology a higher level of sustainability of Egyptian protected cultivation, especially in the areas of water and crop protection. Collaborating private partners are Koppert and HortiMaX in The Netherlands, and BioEgypt and AllGreen in Egypt. Wageningen UR Greenhouse H...

Elings, A.; Helm, F. P. M.; Blok, C.; Meijer, R. J. M.; Lahiani, Y.; Janmaat, A.; Zaki, M.; Hassan, H.

2014-01-01

350

Moessbauer effect of the origin of the colour in the ancient Egyptian black ware  

International Nuclear Information System (INIS)

Samples of ancient Egyptian pottery, Roman pottery and modern pottery were examined by Moessbauer spectroscopy, infrared spectroscopy and x-ray diffraction with the aim of establishing the origin of the black colour of the Egyptian pottery. The Moessbauer spectra and the values of isomer shift and quadrupole splitting were used to decide on the presence of magnetic or non-magnetic ferrous or ferricions. (A.K.)

351

The future of the independent Egyptian music in the digital era  

OpenAIRE

This thesis is investigating the impact of the digital era with its technological advanced components and revolutionized information platforms on shaping the future of the independent Egyptian music. The author investigated this impact through conducting fifteen semi structured qualitative interviews between the 15th of December 2012 to 25th of January 2013 with the relevant Independent Egyptian Music stakeholders who gave the researcher a better insight of what is the optimal business model(...

Maraghah, Mohammad

2013-01-01

352

Allele frequencies of the human platelet antigen-1 in the Egyptian population  

OpenAIRE

Abstract Background The human platelet alloantigen system HPA-1 in the Egyptian population was examined by polymerase chain reaction using sequence-specific primers (PCR-SSP). The objectives of this study were to evaluate the allele frequency of HPA-1a and -1b in healthy Egyptian individuals and compare these with the international literature. Human platelet antigen (HPA) systems are associated with alloimmunization and organ transplantation rejection as well as the development of cardiovascu...

Han Kyudong; Salem Abdel; Batzer Mark A

2009-01-01

353

LAMQS analysis applied to ancient Egyptian bronze coins  

Energy Technology Data Exchange (ETDEWEB)

Some Egyptian bronze coins, dated VI-VII sec A.D. are analyzed through different physical techniques in order to compare their composition and morphology and to identify their origin and the type of manufacture. The investigations have been performed by using micro-invasive analysis, such as Laser Ablation and Mass Quadrupole Spectrometry (LAMQS), X-ray Fluorescence (XRF), Laser Induced Breakdown Spectroscopy (LIBS), Electronic (SEM) and Optical Microscopy, Surface Profile Analysis (SPA) and density measurements. Results indicate that the coins have a similar bulk composition but significant differences have been evidenced due to different constituents of the patina, bulk alloy composition, isotopic ratios, density and surface morphology. The results are in agreement with the archaeological expectations, indicating that the coins have been produced in two different Egypt sites: Alexandria and Antinoupolis. A group of fake coins produced in Alexandria in the same historical period is also identified.

Torrisi, L., E-mail: lorenzo.torrisi@unime.i [Dipartimento di Fisica dell' Universita di Messina, Salita Sperone, 31, 98166 Messina (Italy); Caridi, F.; Giuffrida, L.; Torrisi, A. [Dipartimento di Fisica dell' Universita di Messina, Salita Sperone, 31, 98166 Messina (Italy); Mondio, G.; Serafino, T. [Dipartimento di Fisica della Materia ed Ingegneria Elettronica dell' Universita di Messina, Salita Sperone, 31, 98166 Messina (Italy); Caltabiano, M.; Castrizio, E.D. [Dipartimento di Lettere e Filosofia dell' Universita di Messina, Polo Universitario dell' Annunziata, 98168 Messina (Italy); Paniz, E.; Salici, A. [Carabinieri, Reparto Investigazioni Scientifiche, S.S. 114, Km. 6, 400 Tremestieri, Messina (Italy)

2010-05-15

354

Does Facebook Matter in Egyptian Graduate Environment? A Marketing Perspective  

Directory of Open Access Journals (Sweden)

Full Text Available The current study is exploratory, and it aims at uncovering the potential role of Facebook in Egyptian university environment. The research based on 127 questionnaires. It was found that; 42% of the respondents use Facebook for educational purpose; around 85% use Facebook to be in touch with their teachers; more than 75% use Facebook to contact the faculty administration; more than 70% of students are convinced that they have added value when using Facebook in the educational context. These results would support the notion of using Facebook as an untraditional, cheap, interactive means to enhance the quality of the educational service that provided by the college (producer and received by the student (customer. Implications were presented and suggestion for further studies was highlighted.

Ahmad Yahia Ebeid

2012-05-01

355

HOW EGYPTIAN CONTRACTING COMPANIES TRANSLATE PROJECTS' QUALITY TO PROJECTS' PERFORMANCE  

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Full Text Available A model for continuous quality improvement for Egyptian contracting projects’ (CQIM is developed through the extensive reviewing of the Total Quality Management (TQM, Continuous Quality Improvement (CQIliterature, preceding researches, consensus opinions of managers and experts of the construction industry and the quality management system (ISO 9000. This model comprises of 13 main factors divided into 9 CQI constructs and 4 Indices, the Indices are broken down to 46 practical measures, while the CQI constructs are further divided into 58 sub-factors consisting of 231 practical requirements, all targeting the evaluation and improvement of the overall project performance (OPP . This model assists its users to assess their points of weaknesses and strengths, by setting-up a road map for improvement, utilizing a guided framework. This model is used as a benchmarking tool to achieve the goal of this research “Projects' Continuous Quality Improvement”.

Said SHAWKY HOZIEN

2011-07-01

356

Environmental Monitoring of Toshki Region As a New Egyptian Community  

International Nuclear Information System (INIS)

Some heavy metal pollutants namely Cd, Co, Cu, Fe, Mn, Ni, PI and Zn were measured in water, soil and some fruit plants; Citrus finensis, Orantte folia, Citrus aurantium and Vitis vinifera in Toshki region. These samples were collected in March 2005. The samples were treated according to the Standard Operation Procedures, digested and analyzed using an atomic absorption spectrophotometer. It was found that the mean concentrations of the mentioned elements in water samples are higher than those corresponding by environmental Law. While, the concentrations of those elements in soil samples are two folds higher than those found in plant samples. The results are related to those give by the Egyptian environmental Law No. 4/1994. In addition, natural radioactivity levels for 226Ra, 232Th, 40K and137Cs in soil were found to be lower than those corresponding for United Nations Scientific committee on the Effect of Atomic Radiation (UNSCEAR) values. The results are discussed and recommendations are suggested

357

Dose Assessment of Natural Radioactivity in Ancient Egyptian Monuments  

International Nuclear Information System (INIS)

Within an environmental radiation investigation of the ancient Egyptian monuments sites, 151 environmental samples were analyzed using ?-ray spectrometers based on HPGe detectors. The results show that the activity concentration values of the naturally occurring radionuclides; 238U (22'6Ra), 232Th, and 40K respectively, are within the world average ranges.Two models for calculation of absorbed dose rate due to gamma emission from radionuclides in the studied soil, limestone and adobe samples were evaluated. The first model was established based on constant value of sample density. The second model takes the density variation into consideration. Two sets of conversion factors were evaluated. A comparison between the results of calculation of the absorbed dose of the studied samples based on the model adopted by UNSCEAR and on our two models was carried out and discussed. (authors)

358

Synchrotron radiation analysis on ancient Egyptian vitreous materials  

International Nuclear Information System (INIS)

Ancient Egyptian vitreous materials, namely faience and glass, share the same elemental composition. But they appear to have originated separately. Faience objects appear as early as the Predynastic period, and glass was introduced from Mesopotamia during the New Kingdom. These faience and glass objects were not of daily use, rather they were regarded as religious symbols or luxury status goods. Most of the products were coloured blue, but we see an increased use of other colours during the New Kingdom (c.1550-1069BC). This tendency corresponds to the period of both territorial and political expansion of Egypt. A non-destructive SR-XRF experiment at SPring-8 was conducted last winter, aiming to determine the regional trait of elemental composition by examining the pattern and ratio of rare earth elements. As a result, we could observe some distinctive rare earth elements that may indicate regional variation. (author)

359

Comparisons of Asset Pricing Models in the Egyptian Stock Market  

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Full Text Available This paper employs GRS test to empirically compare the applicability of five alternatives of asset pricing models for 55 shares listed on the EGX100 for the Egyptian stock market: 1 the CAPM, 2 the Fama-French three factor model, 3 the Cahart four factor model, 4 liquidity-augmented four factor model, 5 and the five factor model (liquidity and momentum-augmented Fama-French three factor model. The sample is split into six portfolios sorted on size and book-to market ratio and 45 shares are excluded due to data unavailability. Our results based on GRS (1989 show evidence that Fama-French model is the best and reject the other models. 

Mohamed Ahmed Shaker

2014-09-01

360

The Radial Structure of Some Middle Egyptian Prepositions  

DEFF Research Database (Denmark)

Prepositions are traditionally treated in dictionaries and grammars by giving a list of usages, often corresponding more or less to the way the preposition is translated in the language of the modern work. This paper suggests an alternative way of approaching prepositions, derived from cognitive linguistics where prepositions are regarded as categories centered on a salient prototype from which various peripheral members of the category are derived. This perspective has the advantage of presenting the meaning of each preposition as a unified category with a specific central meaning and various extensions, instead of merely listing a number of unrelated senses. It is argued that Middle Egyptian prepositions can fruitfully be studied in this framework, and the method is exemplified by examining the conceptual structure of the two frequent prepositions m and r.

Nyord, Rune

2010-01-01

361

Clinical, electrophysiological and immunological study of peripheral nerves in Egyptian patients with monoclonal gammopathies.  

Science.gov (United States)

Monoclonal antibodies are found in approximately 10% of patients with peripheral neuropathy (PN) of unknown etiology. Several autoantibodies, including anti-MAG (myelin-associated glycoprotein) antibodies, have been reported to induce neuropathy. It has been suggested that over 50% of patients with PN and IgM monoclonal gammopathy (MG) have anti-MAG IgM antibodies in their sera. This work aimed at studying the frequency and characteristics of PN in a group of Egyptian patients with MGs and to estimate the serum level of anti-MAG antibodies and its relationship to peripheral nerve dysfunction. Forty patients with MGs were enrolled in the study. Their mean age was 56.65 ± 8.55 years. There were 17 males and 23 females. Patients were subjected to complete general and neurological examination, laboratory investigations including serum LDH, ?2 microglobulin, serum protein electrophoresis, urinary Bence-Jones protein, bone marrow aspiration and/or trephine biopsy, quantitative estimation of serum IgM and IgG by nephelometry, detection of anti-MAG antibodies by indirect immunofluorescence, radiological assessment and nerve conduction study of both upper and lower limbs. Clinical and electrophysiological evidences of PN were found in 32 (80%) out of the 40 patients with MG. Twenty-five patients (62.5%) had distal symmetrical polyneuropathy and seven (17.5%) had mononeuritis or mononeuritis multiplex. The majority of patients (65%) had sensory or predominantly sensorimotor polyneuropathy. The neuropathy was mainly demyelinating in 22 patients (55%) and axonal in the other 10 (25%) patients. Anti-MAG antibodies were positive in nine patients (22.5%) and six of them (66.6%) had PN. The latter was predominantly demyelinating motor neuropathy in 4 and axonal in the remaining 2. However, the relationship between the presence of anti-MAG antibodies and the development and type of PN was not statistically significant. Anti-MAG showed significant association with IgM level (P = 0.003**) and the MG subtypes: Waldenström's macroglobulinemia (WM) and monoclonal gammopathy of undetermined significance (MGUS) (P = 0.004**). The present study showed high frequency (>60%) of distal symmetrical polyneuropathy in Egyptian patients with MG. The neuropathy was predominantly sensory and demyelinating. Anti-MAG antibodies were detected only in 22.5% of the patients, especially those with WM and MGUS and were associated with more motor and demyelinating neuropathy. We recommend that patients with chronic polyneuropathies should be evaluated for underlying plasma cell dyscrasia. PMID:22193843

El-Difrawy, Mohamed M; Zaki, Nadia E; Marouf, Hazem M; Ayad, Mona W; Farag, Ahmed M

2012-01-01

362

Plant Extract Control of the Fungi Associated with Different Egyptian Wheat Cultivars Grains  

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Full Text Available Grain samples of 14 Egyptian wheat cultivars were tested for seed-borne fungi. The deep freezing method was used. Five seed-borne fungi viz., Aspergillus flavus, A. niger, Curvularia lunata, Fusarium moniliforme and Penicillium chrysogenum were isolated from the wheat cultivars viz., Bani Suef 4, Bani Suef 5, Gemmiza 7, Gemmiza 9, Gemmiza 10, Giza 168, Misr 1, Misr 2, Sakha 93, Sakha 94, Shandaweel 1, Sids 1, Sids 2 and Sids 3. A. flavus, A. niger and F. moniliforme were the most prevalent fungal species. Their incidence ranged from 21.0-53.5%, 16.0-37.5%, and 12.0-31.0%, respectively. The antifungal potential of water extracts from aerial parts of five wild medicinal plants (Asclepias sinaica, Farsetia aegyptia, Hypericum sinaicum, Phagnalon sinaicum, and Salvia aegyptiaca were collected from the Sinai Peninsula, Egypt. The antifungal potential of water extracts from the aerial parts of these five plants were tested in the laboratory against the dominant fungi isolated from the wheat cultivars. All the aqueous plant extracts significantly (p ? 0.05 reduced the incidence of the tested seed-borne fungi. But the extract of Asclepias sinaica exhibited the most antifungal activity on tested fungi at all concentrations used when compared with other plant extracts. Maximum infested grain germination was observed in Giza 168 and minimum in Bani Suef 5. Treating grains with plant extract of A. sinaica (10% enhanced the percentage of grain germination of all cultivars in both laboratory and pot experiments. Maximum root and shoot length of seedlings was recorded in Bani Suef 4 during fungal infestation or treatment by plant extract. For one hour before sowing or storage, the aqueous extract of A. sinaica can be used to treat wheat grains, to reduce the fungal incidence. Aqueous extracts of the aerial parts of selected medicinal plants, particularly A. sinaica, are promising for protecting Egyptian wheat grain cultivars against major seed-borne fungi. The aqueous extracts are expected to improve crops.

Mohamed Baka Zakaria Awad

2014-07-01

363

Physical Deterioration of Egyptian Limestone Affected by Saline Water  

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Full Text Available This study is the second in a series of experiments that describe the chemical, physical and thermal properties of archaeological limestone affected by salt and saline water in Egypt. This research aims to study the aggressive physical effects of different types of salts dominated in saline water and their different mechanisms on the acceleration of weathering processes that affect Egyptian limestone. It presents a multidisciplinary approach to characterize, at both micro/macro scales, the behavior of a limestone widely used as a construction material in most of Egyptian monuments when interacting with some types of salt solutions of various concentrations. A systematic optical, morphological, physical and mechanical analysis of the fresh and weathered stone samples were used to evaluate different characteristics through using scientific some techniques such as optical microscope (OP and scanning electron microscope (SEM. In addition to the using of some special computer programs that were used to define different physical and mechanical properties such as weight changes, bulk density, total porosity, water uptake, water content, thermal dilatation and abrasion resistant. The results proved that all investigated samples were gradually affected by the types of salinity paths and salt concentrations. These results will serve as a database for the future comparison of long term behavior of stones before and after the planned conservation of the entire area. So, it is pertinent to device some scientific methods and interventions to reduce all factors of salt effects and removing their harmful aspects from historic fabric of the archaeological buildings through some scientific recommendations

Mohammed EL-GOHARY

2013-12-01

364

Sesame seed sensitization in a group of atopic Egyptian children.  

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Full Text Available Background: There are no published data on the prevalence of sesame allergy/sensitization in Egypt. Objective: In this pilot study, we thought to estimate the frequency of sesame seed sensitization in a group of atopic Egyptian infants and children. Methods: We consecutively enrolled 90 patients with physician diagnosed allergic disease. The study measurements included clinical evaluation for the site and duration of allergy, history suggestive of sesame seed allergy, and family history of allergy, as well as skin prick testing (SPT using a commercial sesame extract, and serum sesame specific IgE (SpIgE estimation. Results: None of the studied patients reported symptoms suggestive of sesame seed allergy. Nevertheless, two children (2.2% showed positive SPT response to sesame (wheal diameter ? 3 mm above the negative control. Only one of them had a wheal diameter which exceeded that of the histamine control. The serum sesame SpIgE exceeded 0.35 IU/ml in all subjects [range = 0.35 - 3.0 IU/ml; median (IQR = 0.9 (0.6 IU/ml]. Serum sesame SpIgE was significantly increased in patients with history of recurrent urticaria (p=0.03. Conclusion: Sesame seed sensitization is not uncommon in atopic Egyptian children. It can be associated with any clinical form of allergy and the causal relationship needs meticulous evaluation. Wider scale population-based studies are needed to assess the prevalence of sesame allergy and its clinical correlates in our country

Samar Ahmad

2013-10-01

365

Compensating Differentials and the Value of Job Security: Evidence from the Egyptian Public Sector  

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Full Text Available This paper considers the determinants of male and female pay in the public and private sectors by estimating a joint model of sector allocation and wage determination using cross-sectional data from the Egyptian 1987 and 1997 labour force surveys. A model of compensating wage differentials is defined and estimated, in order to quantify the value of arguably the three most important non-pecuniary aspects of public sector employment: job security, fringe benefits (especially comprehensive retirement pensions and lower effort and shorter hours which allow workers to supplement income through obtaining a second job. Estimates of the public-private differentials, correcting for differences in characteristics and selectivity, indicate a public sector disadvantage for males and a small advantage for females in 1987. Relative public sector wages improved for both males and females in 1997, and when adjustments for non-wage benefits are included, public sector premia are observed in all segments of the public sector for both males and females. The results highlight the importance of job security as the major factor determining the persistence of queues for public sector jobs in Egypt.

Mona Said

2011-12-01

366

Effect of irradiation and storage on biogenic amine contents in ripened Egyptian smoked cooked sausage  

International Nuclear Information System (INIS)

The effects of ?-irradiation upon the biogenic amine inventory in Egyptian smoked cooked sausages were investigated for the first time during storage for up to 90 days at 4 ° C. Typical contents of biogenic amines in non-irradiated sausages ranged between 125.50 and 596.18 mg/kgDW; irradiation with 4 and 6 kGy decreased said total contents to 105.20-94.82 and 104.98-26.44 mg/kgDW respectively, by the end of storage. Putrescine and cadaverine were the major amines in non-irradiated samples - where it accounted for 33% and 29% respectively, of the total by 90 days; however, tyramine dominated in irradiated samples with 2, 4 and 6 kGy, where it accounted for 44, 52 and 42%. On the other hand, the histamine content in non-irradiated sausage by 90 days of storage (i.e. 109.12 mg/kgDW) clearly exceeded the maximum allowable of 50 mg/kg, unlike happened in their irradiated counterparts. Therefore, the dramatic reduction observed in the histamine levels suggests use of this preservation technique for that traditional meat food. (author)

367

Contraceptive Discontinuation Among Egyptian Women: Results From Egypt Demographic And Health Survey 2005  

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Full Text Available Background: Unintended pregnancy is a major public health problem that affects not only the individuals directly involved but also society. Most couples who want to avoid pregnancy practice contraception. Objective: The objectives of this study were to determine the prevalence of discontinuation of the different methods of contraception and describe the most common reasons of discontinuation of contraception usage. Method: The 2005 Egypt Demographic and Health Survey (EDHS is a nationally representative household survey of 19 474 married women aged 15?49 years. The study sample was selected using a multistage sampling technique. A face-to-face structured interview was conducted with the selected women. The response rate was 99.5% for completing the questionnaires. The 2005 EDHS provides a wealth of information on fertility, family planning, maternal and child health and nutrition, and violence against women. Results: The results of this study showed that 46.4% of women discontinued the use of contraception. Side effects of the method were the most frequent reason of discontinuation. Conclusion: Nearly half of Egyptian women discontinued the use of contraception due to side effects and wanted more children. [TAF Prev Med Bull 2013; 12(1.000: 15-26

Hala Awadalla

2013-02-01

368

Relevance of medieval, Egyptian and American dates to the study of climatic and radiocarbon variability  

International Nuclear Information System (INIS)

Basic radiocarbon dating and dendrochronology have been combined to yield calibrated dates that are more accurate than conventional radiocarbon dates. This has been shown to be true for medieval and Egyptian dynastic dating. Because radiocarbon is a cosmogenically produced radioisotope, heliomagnetic and geomagnetic fields play a major role in its synthesis in the Earth's upper atmosphere. Inasmuch as a calibrated radiocarbon record exists for nearly 10 000 years, we now seem to possess in the short-time variations of the production rate a history of solar activity expressed via heliomagnetic fields carried by the solar wind. In turn, solar activity has a controlling effect on climate on Earth within modifications provided by the complex interactions of the atmosphere-Earth-ocean system. Both radiocarbon measurements and other empirical research methods agree on variations of climate during historically more recent periods on Earth. This leads to the suggestion that the radiocarbon calibration curve may be also a significant indicator or tracer for climatic changes for the Holocene or the Neolithic-Mesolithic. (author)

369

Pre-treatment role of inosine triphosphate pyrophosphatase polymorphism for predicting anemia in Egyptian hepatitis C virus patients  

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Full Text Available AIM: To investigate and clarify, for the first time, the role of inosine triphosphate pyrophosphatase (ITPA polymorphism in Egyptian chronic hepatitis C virus (HCV patients. METHODS:The human genomic DNA of all patients was extracted from peripheral blood cells in order to determine the single nucleotide polymorphism (SNP of ITPA (rs1127354. SNP genotyping was performed by real time polymerase chain reaction (PCR, ABI TaqMan allelic discrimination kit for 102 treatment-naive Egyptian patients with chronic HCV. All patients had no evidence of cardiovascular or renal diseases. They received a combination treatment of pegylated interferon ? (PEG-IFN? as a weekly subcutaneous dose plus an oral weight-adjusted dose of ribavirin (RBV. The majority received PEG-IFN?2a (70.6% while 29.4% received PEG-IFN?2b. The planned duration of treatment was 24-48 wk according to the viral kinetics throughout the course of treatment. Pre-treatment liver biopsy was done for each patient for evaluation of fibrosis stage and liver disease activity. The basal viral load level was detected quantitatively by real time PCR while viral load throughout the treatment course was performed qualitatively by COBAS TaqMan assay. RESULTS: Ninety-three patients (91.2% had ITPA SNP CC genotype and 9 (8.8% had non-CC genotype (CA and AA. The percentage of hemoglobin (Hb decline was higher for CC patients than for non-CC patients, particularly at weeks 4 and 8 (P = 0.047 and 0.034, respectively. During the first 12 wk of treatment, CC patients had significantly more Hb decline > 3 g/dL than non-CC patients: 64.5% vs 22.2% at weeks 8 and 12, respectively, (P = 0.024 and 0.038. Reduction of the amount of the planned RBV dose was significantly higher for CC patients than non-CC patients during the first 12 wk (18% ± 12.1% vs 8.5% ± 10.2%, P = 0.021. The percentage of CC patients with RBV dose reduction was significantly greater than that of non-CC patients (77.4% vs 44.4%, P = 0.044. Multivariate analysis identified only the percentage of RBV dose as a predictor for Hb decline. Platelet decline was significantly higher in non-CC patients than CC patients at weeks 12, 24 and 48 (P = 0.018, 0.009 and 0.026, respectively. CONCLUSION: Rs1127354 ITPA polymorphism plays a decisive role in protecting against treatment-induced anemia and the need for RBV dose reduction in Egyptian HCV patients.

Walaa H Ahmed

2013-01-01

370

Phenolics, Selenium, Vitamin C, Amino Acids and Pungency Levels and Antioxidant Activities of Two Egyptian Onion Varieties  

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Full Text Available Selenium, vitamin C, pungency, amino acids, phenolics content and antioxidant activities of two Egyptian onion varieties, namely white (Giza-6 and red (Beheri onions have been studied. Data analysis showed that the red variety presents higher values for selenium, vitamin C and sulphur-containing amino acids. Concerning pungency, white onion can be classified as intermediate pungency (8.24 ?mol of Pyruvic acid/100 g fresh wt. and red as pungent (11.37 ?mol of pyruvic acid/100 g fresh wt.. The phenolic acids, flavonols, anthocyanins and total phenolics content in red variety (81.59, 70.38, 7.56 and 187.17 mg/100 g fresh wt., respectively were higher than for white variety (72.47, 32.49, 4.90 and 131.65 mg/100 g fresh wt., respectively. Consequently, antioxidant activity was higher for the red variety. Correlation analysis indicates that phenolic compounds beside other factors including Se and sulphur-containing amino acid contents play the major role in the antioxidant activity of onion bulbs. The antioxidant capacity of freeze dried powder from both onion varieties was also tested in sunflower oil-in-water emulsions and hydroperoxide formation was monitored during storage at 40°C. In accordance with differences in Se, sulphur-containing amino acid and phenolics content, Egyptian red onions had better antioxidant activity, while white onions was only effective in the early stages of the oxidation process. These data indicates that red variety has higher potential health benefits related to the presence of antioxidant compounds.

Y.A. Elhassaneen

2009-01-01

371

Major depression  

Science.gov (United States)

Depression - major; Depression - clinical; Clinical depression; Unipolar depression; Major depressive disorder ... Doctors do not know the exact causes of depression. It is believed that chemical changes in the ...

372

Synthesis of Cellulose Acetate Membrane from the Egyptian Rice Straws  

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Full Text Available In Egypt, approximately 4.5 million tons from 35 million tons of the annual production of recoverable cereal are disposed by burning and it creates a big problem. Isolation of cellulose with high yield and purity is a long-standing goal in cellulose development because of the complexity of cell wall structure of rice straw. Chemical composition of Egyptian rice straw was determined (moisture, ash, LMWC, wax and protein and total carbohydrates. Cellulose was extracted by different alkaline NaOH concentration till optimum conditions determined. A series of acetylated celluloses with various degrees of substitution were prepared by homogeneous acetylation of celluloses. The FT-IR, 1H NMR and 13C NMR were used to investigate the changes of chemical structures and physical characteristics. Three cellulosic membranes fabricated from cellulose acetate/polyethersulfone composite. The scanning electron microscope was measured and characterized by pore-free upper surface and a porous bottom surface. A water uptake ratio was measured at room temperature for three membranes of crude (M1, soluble acetone (M2 and soluble chloroform cellulose acetate (M3 as 708, 527 and 710% (w/w, respectively.

Tamer I.M. Ragab

2014-01-01

373

Nuclear safety requirements for operation licensing of Egyptian research reactors  

International Nuclear Information System (INIS)

From the view of responsibility for health and nuclear safety, this work creates a framework for the application of nuclear regulatory rules to ensure safe operation for the sake of obtaining or maintaining operation licensing for nuclear research reactors. It has been performed according to the recommendations of the IAEA for research reactor safety regulations which clearly states that the scope of the application should include all research reactors being designed, constructed, commissioned, operated, modified or decommissioned. From that concept, the present work establishes a model structure and a computer logic program for a regulatory licensing system (RLS code). It applies both the regulatory inspection and enforcement regulatory rules on the different licensing process stages. The present established RLS code is then applied to the Egyptian Research Reactors, namely; the first ET-RR-1, which was constructed and still operating since 1961, and the second MPR research reactor (ET-RR-2) which is now in the preliminary operation stage. The results showed that for the ET-RR-1 reactor, all operational activities, including maintenance, in-service inspection, renewal, modification and experiments should meet the appropriate regulatory compliance action program. Also, the results showed that for the new MPR research reactor (ET-RR-2), all commissioning and operational stages should also meet the regulatory inspection and enforcement action program of the operational rcement action program of the operational licensing safety requirements. (author)

374

Utilization of Egyptian Research Reactor and Modes of Collaboration  

International Nuclear Information System (INIS)

The new Egyptian Research Reactor (ETRR-2) is a Material Testing Reactor (MTR) commissioned in 1997. It is an open pool Research Reactor (RR) using low enriched MTR fuel elements (less than 20% enrichment), cooled and moderated with light water and reflected by beryllium. The reactor power is 22 MW with high neutron flux irradiation positions (flux > 1014 n/cm2.s) and can be operated up to 19 days providing high neutron fluence. Also, the reactor has two fast irradiation positions, two silicon irradiation positions, three radial and one tangential beam tubes, and thermal column. ETRR-2 is a multipurpose reactor, several experimental and production facilities have been installed for Radio Isotope (RI) production (I-131, I-125, Cr-51, Ir-192, and Co-60 ), Neutron Activation Analysis (NAA) applications, Neutron Transmutation Doping (NTD), neutron radiography experiments, and training of personnel. A special hot cell for irradiated material testing has been installed where the impact tests, tensile tests, and other material characterization can be applied for irradiated samples of materials used in Nuclear Power Plant (NPP) and advanced reactors. In this paper, the utilization of ETRR-2 and future plans for development of some of existing and new facilities are presented as well as modes of collaboration with regional countries for sharing RR services. (author)

375

Fast neutron fluxes distribution in Egyptian ilmenite concrete  

International Nuclear Information System (INIS)

This work is concerned with the study of the distribution of fast neutron fluxes in a new type of heavy concrete made from Egyptian ilmenite ores. The neutron source used was a collimated beam of reactor neutrons emitted from one of the horizontal channels of the ET-RR-1 reactor. Measurements were carried-out using phosphorous activation detectors. Iso-flux curves were represented which give directly the shape and thickness required to attenuate the emitted fast neutron flux to a certain value. The relaxation lengths were also evaluated from the measured data for both disc monodirectional source and infinite plane monodirectional source. The obtained values were compared with that calculated using the derived values of relative number densities and microscopic removal cross-sections of the different constituents. The obtained data show that ilmenite concrete attenuates fast neutron flux more strongly than ordinary concrete. A semiemperical formula was derived to calculate the fast neutron flux at different thicknesses along the beam axis. Another semiemperical formula was also derived to calculate the fast neutron flux in ordinary concrete along the beam axis using the corresponding value in ilmenite concrete

376

Barriers to E-Commerce Adoption in Egyptian SMEs  

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Full Text Available E-commerce has been predicted to be a new driver of economic growth for developing countries. The SME sector plays a significant role in its contribution to the national economy in terms of the wealth created and the number of people employed. Small and Medium Enterprises (SMEs in Egypt represent the greatest share of the productive units of the Egyptian economy and the current national policy directions address ways and means of developing the capacities of SMEs. Many factors could be responsible for the low usage of e-commerce among the SMEs in Egypt. In order to determine the factors that promote the adoption of e-commerce, SMEs adopters and non-adopters of e-commerce were asked to indicate the factors inhibiting the adoption of e-commerce. The results show that technical barriers are the most important barriers followed by legal and regulatory barriers, whereas lack of Internet security is the highest barrier that inhibit the implementation of e-commerce in SMEs in Egypt followed by limited use of Internet banking and web portals by SMEs. Also, findings implied that more efforts are needed to help and encourage SMEs in Egypt to speed up e-commerce adoption, particularly the more advanced applications.

Abdel Nasser H. Zaied

2012-07-01

377

First records of polychaetes new to Egyptian Mediterranean waters  

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Full Text Available Nineteen benthic polychaete species were recorded for the first time inthe intertidal zone of the Alexandria coast, south-eastern Mediterranean Sea. They belong toSyllidae (7 species, Hesionidae (3 species, Serpulidae (2 species and7 other families (one species each. Of these species Eunice miurai Carrera-Parra& Salazar-Vallejo 1998 appears to be new to the Mediterranean Sea,while four of the alien species earlier recorded in the Mediterranean were found for thefirst time in Egyptian waters: Opisthosyllis brunnea Langerhans 1879,Loimia medusa Savigny 1822, Syllis schulzi Hartmann-Schröder1960, Phyllodoce longifrons Ben-Eliahu 1972.     The newly recorded species demonstrated markedly different patterns offrequency of occurrence and numerical abundance. Spirobranchus triqueterLinnaeus 1758, S. schulzi, L. medusa and Salvatoria clavataClaparède 1863 were permanent and abundant species in fouling samples along the Alexandria coast.Saccocirrus papillocercus Bobretzky 1872 persisted in the sediments at two sites,with a much higher count at the stressed one, while Protodrilussp. inhabited sediments at two other sites throughout the year, sometimes invery high numbers. In addition, the alien species found earlier, Braniaarminii Langerhans 1881, Odontosyllis fulgurans Audouin &Milne-Edwards 1833 and O. brunnea Langerhans 1879, were frequentlyobserved along the Alexandria coast.

Manal Mohamed Atta

2013-02-01

378

Efficiency of diagnostic biomarkers among colonic schistosomiasis Egyptian patients  

Scientific Electronic Library Online (English)

Full Text Available The schistosomal parasite plays a critical role in the development of malignant lesions in different organs. The pathogenesis of cancer is currently under intense investigation to identify reliable prognostic indices for disease detection. The objective of this paper is to evaluate certain biochemic [...] al parameters as diagnostic tools to efficiently differentiate between colonic carcinoma and colonic carcinoma associated with schistosomal infection among Egyptian patients. The parameters under investigation are interleukin 2 (IL-2), tumour necrosis factor alpha (TNF-?), carcinoembryonic antigen (CEA) levels, tissue telomerase, pyruvate kinase (PK), glucose-6-phosphate dehydrogenase (G-6-PD) and lactate dehydrogenase (LDH) enzyme activities. The results revealed a significant elevation in the level of the tumour markers IL-2, TNF-? and CEA as well as the activities of LDH, telomerase and G-6-PD among non-bilharzial and bilharzial colonic cancer groups, with a more potent effect in bilharzial infection-associated colonic cancer. A significant inhibition in PK activity was recorded in the same manner as compared to normal tissues. The efficacy of this biomarker was also evaluated through detecting sensitivity, specificity, negative and positive predictive values. In conclusion, schistosomal colonic carcinoma patients displayed more drastic changes in all parameters under investigation. The combination of the selected parameters succeeded in serving as biomarkers to differentiate between the two malignant types.

Manal Abdel Aziz, Hamed; Samia Abdel Aziz, Ahmed; Hussein Moustafa, Khaled.

2011-05-01

379

Distribution of Fungi in the Sandy Soil of Egyptian Beaches  

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Full Text Available The mycobiota of the sandy soil of Egyptian beaches was investigated in thirty six sand samples collected from nine different localities in Egypt. The filamentous fungi were identified and assigned to thirty one genera and fifty one species. Greater populations as well as a wider spectrum range of fungal genera and species were obtained in sandy soil of Alexandria beach while Balteem beach was the poorest one. The total count of the genus or species did not always follow the number of cases of isolation. Most of the genera detected belonged to the Deuteromycotina with fewer proportions belonging to the Ascomycotina and Zygomycotina. The genera of highest incidence and their respective numbers of species were: Penicillium (35.72%, 6 spp. and Aspergillus (30.28%, 16 spp.. The species which showed the highest incidence in all cases was P. chrysogenum, followed by P. citrinum, A. flavus, Chaetomium murorum and Trichoderma viride. Several other genera and species were detected at quite low occurrence.

Fatma F. Migahed

2003-01-01

380

Mining of Egyptian Missions Data for Shaping New Paradigms  

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Full Text Available This paper reviews data mining applications of students' databases in educational institutions. Data mining techniques that predict and improve students' retention rates and success is presented. Moreover, the Missions Administration at the Ministry of Higher Education in Egypt and previous analysis done on the missions databases is described. The paper further describes the methodology used for analyzing the database for the ministry of higher education in Egypt. The process starts by extracting a subset of data including the missioners and the mission’s data, countries, specialties, departure and arrival dates and finally the extension requests from the missioners. These data were extracted into a data warehouse for the analysis purpose. The used model discovered the best and the worst countries for student mission. A detailed analysis discovered the best and the worst specialties in the previously discovered countries.Moreover, the analysis revealed the effect of the marital status on the mission of students in foreign countries. A visual display using a chart was used to express the information to business users. This model may help in achieving effective decision making in the reallocation of Egyptian students to other countries.

Laila Mohamed ElFangary

2009-08-01

381

Characterization of Egyptian Smectitic Clay Deposits by Methylene Blue Adsorption  

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Full Text Available Problem statement: The characterization of smectitic clays has been traditionally made using physical and chemical methods which are time-consuming and require costly equipment. Adsorption of methylene blue by these clays has been introduced as a quick and cheap method for the estimation of cation exchange capacity and surface area. Therefore, the aim of the present study was to test the applicability of the methylene blue method for the determination of cation exchange capacity and specific surface area of Egyptian smectitic clay deposits and hence to estimate their content of expansive clays. Approach: The distribution of sand, silt and clay fractions was determined in the studied samples by grain size analysis. The methylene blue test was applied using the halo method for the determination of the optimum adsorption point. Results: A positive correlation was found between the cation exchange capacity of the claystone as determined by the methylene blue value and its clay fraction and between the specific surface area and the cation exchange capacity. Conclusion/Recommendations: It has been concluded that the methylene blue test can be used as a reliable and rapid method for the characterization of smectitic clay deposits and soils containing smectite. These properties are important for engineering and environmental geological assessments of sites for different construction purposes.

Salwa D. Abayazeed

2011-01-01

382

Evolutionstechnik or Selection and Variation in the Egyptian Origami Bird (Avis papyrus)  

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In order to examine the random nature of mutations and natural selection, learners "breed" clutches of Egyptian Origami Birds (Avis papyrus) using random number generators (dice and coins) to mutate several genetic loci: anterior and posterior wing position, wing width, and wing length. The birds are then "released." Only those birds which can fly the furthest survive to produce offspring. After several generations, learners usually note a significant increase in flight distance and duration. In a second experiment, different groups of learners study separate sub-populations of Egyptian Origami Birds, which experience different selection pressures. Details of Origami Bird anatomy, breeding and selection, are in the learner instructions and associated graphic.

Karin Westerling

2009-01-01

383

Whole-body three-dimensional multidetector CT of 13 Egyptian human mummies.  

OpenAIRE

OBJECTIVE: The purpose of this article is to assess the role of multidetector CT and three-dimensional (3D) reconstructions in noninvasive studies of Egyptian mummies. MATERIALS AND METHODS: We studied 13 mummies from the Egyptian Museum in Torino, Italy, dating from Dynasty III to Dynasty IV (2650-2450 B.C.) and from the Ptolemaic period (332-30 B.C.) to the Roman period (30 B.C.-A.D. 395), using a multidetector CT unit with a single volumetric acquisition of the whole body, including lower ...

Gandini, Giovanni; Boano, Rosa

2003-01-01

384

Separation of cerium (III) from egyptian monazite by oxidation to cerium(IV)  

International Nuclear Information System (INIS)

The lanthanides hydrous oxides produced from the egyptian monazite contain about 45% cerium of the total lanthanides. The high concentration of Ce in this matrix complicates separation of different lanthanides from each other. In this paper a method was developed to separate cerium quantitatively from the lanthanide cake produced from egyptian monazite by precipitating tetravalent cerium from other trivalent lanthanides by oxidation of Ce (III) with bleaching powder from dilute solution of nitric hydrochloric acids mixture. The different parameters affecting the separation were studied in details

385

Count like an egyptian a hands-on introduction to ancient mathematics  

CERN Document Server

The mathematics of ancient Egypt was fundamentally different from our math today. Contrary to what people might think, it wasn't a primitive forerunner of modern mathematics. In fact, it can't be understood using our current computational methods. Count Like an Egyptian provides a fun, hands-on introduction to the intuitive and often-surprising art of ancient Egyptian math. David Reimer guides you step-by-step through addition, subtraction, multiplication, and more. He even shows you how fractions and decimals may have been calculated-they technically didn't exist in the land of the pharaohs.

Reimer, David

2014-01-01

386

Biochemical and physiological changes in Egyptian Nile fish subjected to varying levels of gamma irradiation  

International Nuclear Information System (INIS)

Radiation is nowadays to be considered as a new parameter in the ecology of water masses. Aquatic organisms, perhaps more than any other group of organisms, are directly exposed to radiation hazard and may be subjected to continuous low-level exposure from bottom material and from internal sources accumulated within their own bodies, originating partly from radionuclides released from nuclear facilities into the aquatic environment. In recent years, a large number of papers have been published on the uptake, concentration and release of radioactive material by aquatic organisms. However, radiation experiments on fish, a major source of food for human consumption, are still very rare and mostly restricted to studies on the effect of irradiation on eggs and larvae. Since the study of the radiation effect on living aquatic organisms, particularly fish, is important in connection with the problems of preserving water resources for the benefit of mankind, the work presented here has been done to ascertain the effect of varying dose-levels of gamma irradiation on two common Egyptian Nile fish species, the catfish Clarias lazera and the Tilapia nilotica. Investigations carried out on Clarias lazera involved blood and muscle analyses as well as growth rate measurements. The results obtained showed impaired haematological levels, changes in weight of muscle proteins and, chiefly, retardation in growth rate. Investigations carried out on Tilapia nilotica revealed changes in then Tilapia nilotica revealed changes in the activity of certain digestive enzyme systems, glucose level in blood and concentration of the glycogen store in liver and muscles. In discussing the results obtained the authors have taken the relevant literature into consideration. (author)

387

The Restoration and Conservation of Egyptian Alabaster Vessels from the Early ERA in Atfiyah Museum Store - Helwan - Egypt  

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Egypt is considered one of the most countries which contain a lot of cultural heritage; the Ancient Egyptian used a lot of stones for his life like: limestone, sandstone, granite and Egyptian Alabaster. The Egyptian Alabaster is used for his daily and eternal life, he made a lot of funerary furniture from this stone like: vessels, statues, Architectural elements in the temples, tombs and canopic jars to preserve his viscera from decomposition like: stomach, liver … etc in the mummification process. Egyptian Alabaster is a sedimentary rock especially chemical- origin sedimentary rocks, it deposits inside caves and around springs which consists of calcium carbonates (CaCO3), they are very fragile "hardness = 3 in Mohs hardness scale". The Egyptian Alabaster vessels expose to a lot of deterioration factors in the burial and exposure environment after excavation. The study case vessels are made of Egyptian alabaster stone and belong to the early era (First and second Egyptian dynasties) in Atfiyah museum store, these vessels exposed to a lot of deterioration factors in the burial and exposure environment like: soil pressure, air temperature variety, relative humidity and salts. The vessels are conserved at the restoration laboratory in Atfiyah museum store by a lot of restoration and conservation processes like: cleaning - consolidation - assembling process for the separated parts and completion for the lost parts.

Radi Abdel Kader, R.; Sayed Mohamed, S.

2013-07-01

388

Modifiable diarrhoea risk factors in Egyptian children aged <5 years.  

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By conducting a case-control study in two university hospitals, we explored the association between modifiable risk behaviours and diarrhoea. Children aged <5 years attending outpatient clinics for diarrhoea were matched by age and sex with controls. Data were collected on family demographics, socioeconomic indicators, and risk behaviour practices. Two rectal swabs and a stool specimen were collected from cases and controls. Samples were cultured for bacterial pathogens using standard techniques and tested by ELISA to detect rotavirus and Cryptosporidium spp. Four hundred cases and controls were enrolled between 2007 and 2009. The strongest independent risk factors for diarrhoea were: presence of another household member with diarrhoea [matched odds ratio (mOR) 4.9, 95% CI 2.8-8.4] in the week preceding the survey, introduction to a new kind of food (mOR 3, 95% CI 1.7-5.4), and the child being cared for outside home (mOR 2.6, 95% CI 1.3-5.2). While these risk factors are not identifiable, in some age groups more easily modifiable risk factors were identified including: having no soap for handwashing (mOR 6.3, 95% CI 1.2-33.9) for children aged 7-12 months, and pacifier use (mOR 1.9, 95% CI 1.0-3.5) in children aged 0-6 months. In total, the findings of this study suggest that community-based interventions to improve practices related to sanitation and hygiene, handwashing and food could be utilized to reduce the burden of diarrhoea in Egyptian children aged <5 years. PMID:23433452

Mansour, A M; Mohammady, H El; Shabrawi, M El; Shabaan, S Y; Zekri, M Abou; Nassar, M; Salem, M E; Mostafa, M; Riddle, M S; Klena, J D; Messih, I A Abdel; Levin, S; Young, S Y N

2013-12-01

389

Cytokine Gene Polymorphisms in Egyptian Cases with Brain Tumors  

International Nuclear Information System (INIS)

Background: Cytokines are proposed to play important roles in brain tumor biology as well as neuro degeneration or impaired neuronal function. Objectives: This work aimed to check the association of polymorphisms of cytokine genes in Egyptian cases with brain tumors. Methods: This work included 45 cases affected by brain tumors diagnosed as 24 benign and 21 malignant. Their median age was 45 years, and they were 20 males and 25 females. These cases were taken randomly from the Neurosurgery Department of Mansoura University Hospital, Egypt. Case genotypes were compared to 98 healthy unrelated controls from the same locality. DNA was amplified using PCR utilizing sequence specific primers (SSP) for detection of polymorphisms related to TNF-a-308 (G/A), IL-10-1082 (G/A), IL-6-174 (G/C) and IL-1Ra (VNTR) genes. Results: Cases affected with benign brain tumors showed a significant higher frequency of IL-10-1082 A/A [odds ratio (OR=8.0), p<0.001] and IL-6-174 C/C (OR=6.3, p=0.002) homozygous genotypes as compared to controls. Malignant cases, on the other hand, showed significantly higher frequency of IL-6-174 C/C (OR =4.8, p=0.002) homozygous genotype and TNF-a-308 A/A (OR=4.9, p<0.001) homozygous genotype when compared to controls. In the meantime, all cases showed no significant difference regarding the distribution of IL-1Ra VNTR genotype polymorphism compared to controls. Conclusions: Cytokine gene polymorphisms showed a pattern of association with brain tumors which may have potential impact on family counseling and disease management.

390

Biological Assay of Toxoplasma gondii Egyptian Mutton Isolates  

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Full Text Available Mutton signifies one of the most prevalent sources for human toxoplasmosis. However, sheep serological assays don't categorize the virulent strains initiating antibodies, so the biological bioassay of Egyptian mutton isolates with reference to their pathogenicity in both mice and kittens were done in this study for indicating to how extent their zoonotic bio-hazard. A total number of 280 of each sheep blood and tissue samples were collected during slaughtering at Cairo abattoir, Egypt. Sera assayed using Latex Agglutination Test (LAT and immunosorbant assay (ELISA and their corresponding mutton samples were microscopically examined after pepsin digestion for detection of Toxoplasma gondii infection. The sero-positive percent of the naturally infected sheep was 50.4 and 61.4 by LAT and ELISA, respectively, 47.9% of samples were confirmedly positive in both LAT and ELISA results. The microscopical examination revealed that only 28 out of 134 (20.9% of the confirmed sero-positive animals by both tests were found harboring T. gondii tissue cysts in their mutton samples, while high percentage of confirmed sero-positve animals (79.1% (106 out of 134 were biologically tissue cysts free mutton. Biological typing of the 28 T. gondii sheep isolates with reference to mice and kittens' bioassay indicated that 10.7, 50, 21.4 and 17.9% were type I, II, III and avirulent strains, respectively. The high T. gondii infection rate resulted in this study concludes that the feeding of under cooked mutton is a bad health habit as a source for human toxoplasmosis moreover; the T. gondii virulent strains obtained by mutton bioassay indicated that not all sero-positive sheep are connecting zoonotic bio-hazard through their mutton strains.

N.A. Hassanain

2011-01-01

391

Characterisation of aflatoxin and deoxynivalenol exposure among pregnant Egyptian women.  

Science.gov (United States)

Mycotoxins such as the aflatoxins and deoxynivalenol (DON) are frequent contaminants of food. Aflatoxin B1 (AFB1) and DON affect the immune system and restrict growth; additionally AFB1 is carcinogenic. To date there are limited descriptive biomarker data concerning maternal exposures during pregnancy, and none on co-exposures to these mycotoxins. This survey was a cross-sectional assessment providing descriptive data on the concentrations of serum aflatoxin-albumin (AF-alb), urinary aflatoxin M1 (AFM1), and urinary DON for 98 pregnant women from Egypt, in relation to diet and socioeconomic status, during the third trimester. AF-alb was detected in 34 of 98 (35%) samples, geometric mean (GM) of positives = 4.9 pg AF-lys mg(-1) albumin (95% confidence interval (CI) = 4.1-5.8 pg mg(-1)), and AFM1 in 44 of 93 (48%) samples, GM of positives = 19.7 pg mg(-1) creatinine (95%CI = 14.8-26.3 pg mg(-1)). AF-alb and AFM1 levels were positively correlated (R = 0.276, p = 0.007). DON was detected in 63 of 93 (68%), GM of positives = 2.8 ng mg(-1) (95%CI = 2.1-3.6 ng mg(-1)). Aflatoxin and DON biomarkers were observed in 41% of the subjects concurrently. The frequency and level of these biomarkers in Egyptian women were modest compared with known high-risk countries. However, this study represents the first biomarker survey to report on the occurrence of DON biomarkers in an African population, in addition to the co-occurrence of these two potent mycotoxins. This combined exposure may be of particular concern during pregnancy given the potential of toxin transfer to the foetus. PMID:22376138

Piekkola, S; Turner, P C; Abdel-Hamid, M; Ezzat, S; El-Daly, M; El-Kafrawy, S; Savchenko, E; Poussa, T; Woo, J C S; Mykkänen, H; El-Nezami, H

2012-01-01

392

Sociodemographic factors responsible for blindness in diabetic Egyptian patients  

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Full Text Available Khaled Gamal Ibraheem Abueleinen1, Hany El-Mekawey1, Yasser Sayed Saif2, Amr Khafagy1, Hoda Ibrahim Rizk3, Eman M Eltahlawy41Department of Ophthalmology, Cairo University, Cairo, Egypt; 2Department of Ophthalmology, Beni-Suef University, Beni-Suef, Egypt; 3Department of Public Health, Cairo University, Cairo, Egypt; 4Public Health and Community Medicine National Research Center, Cairo, EgyptPurpose: To evaluate factors behind the delay in diagnosis and treatment among Egyptian patients who present with complicated diabetic retinopathy.Methods: Observational cross-sectional study of diabetic patients with advanced diabetic retinopathy. Patients were asked to answer a questionnaire to assess the impact of several sociodemographic factors.Results: A total of 397 patients agreed to take the questionnaire. Diabetic vitreous hemorrhage was the most common ocular complication and was found in 359 patients (90.4%. A total of 158 (39.8% patients knew that diabetes mellitus can be sight threatening, while 240 (60.2% were not aware until they developed sight threatening complication. A total of 179 patients (45.1% had early retirement because of visual loss related to diabetes mellitus. Multivariate logistic regression has shown that education, internist, contact with other patients, and media were respectively significant in predicting the awareness of patients about the sight-threatening effect of diabetic retinopathy.Conclusion: Patient education regarding diabetes and diabetic eye disease is essential for early detection and compliance with treatment. Illiteracy has a significant impact on development of sight-threatening diabetic complications. The internist is the first line of prophylaxis. Media has to participate more in patient education.Keywords: blindness, education, laser photocoagulation, macular edema, vitreous hemorrhage

Abueleinen KGI

2011-11-01

393

Quality of Life in Egyptian Children with Cancer  

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Full Text Available Background and Objectives: Cancer treatments leading to increased survival rates are reported to participate in the creation of debilitating physical and psychosocial deficits for cancer survivors. Measures of health-related quality of life (HRQOL are designed to tap such consequences of cancer treatment together with the impact of the disease itself. Methods: Parents of 67 included patients aged 8 - 12 years, were asked to complete the parent proxy report of PedsQLTM 3.0 Cancer Module (Arabic version, as well as a separate sheet for socio-demographic data. Results: The ratio of Males to females was 1.8:1 among study patients with a median age of 8 years at diagnosis. Hematological malignancies represented 70.1% of the sample, with the highest proportion for ALL (52.2%. Total QOL showed to be relatively low with mean value of 62.29 for the whole group. Subscales with least scores were for; worry (44.11, perceived physical appearance (50.6, and procedural anxiety (55.34. On the other hand, the best score was 75.98 for communication, followed by 72.63 for cognitive problems. The impacts of some medical and socio-demographic variables on QOL and its subscales were elicited in our results. Conclusion: Increased treatment intensity, long duration of hospital admission, higher frequency of hospital visits, female sex, younger age at diagnosis, and large family size were all associated with a poorer total QOL and/or its subscales among Egyptian pediatric cancer patients.

Mohamed Fawzy

2013-08-01

394

A NEW SMALL POTATO PLANTER FOR EGYPTIAN AGRICULTURE  

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Full Text Available Reducing the tuber period for potato plants by planting previously grown sprouts is a new technique. Applying this new agricultural methodology requires modification of the potato planter. The theoretical analysis was carried out to obtain information that can help avoid tuber damage during feeding and until it exits the feeding system. Starting from these considerations, a new potato planter with a spoon-based feeding system was designed and built. The tests conducted verified that the new system performed well when dividing tuber pieces into rows: considering the best growth spacing for potatoes is 20÷30 cm, the new system has a 68% distribution versus the 50,62 % of the old one. The tubers’ spacing in rows are evaluated based on tuber voids and tuber doubles. The results show that there is no difference between the two systems’ configurations, while a general consideration about our machine is that it is better at managing small tuber pieces. The void ratios are largely valid within the range of 0.4 up to 0.8 m/s of planted speeds; moreover, the results revealed that the tuber doubles decrease by increasing planting speed and vice versa with tuber voids. The quality of the potatoes planted is evaluated using the Shatter Index (SI %, which shows how the new configuration is more delicate with the tuber pieces (1.5 % against 17.5 % at 10 rpm or 46 % against 94 % at rpm 20. The new configuration could be an interesting improvement for potato quality and Egyptian potato production.

Amer Eissa Ayman

2011-09-01

395

Analysis of IL28B variants in an Egyptian population defines the 20 kilobases minimal region involved in spontaneous clearance of hepatitis C virus.  

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Spontaneous clearance of hepatitis C virus (HCV) occurs in ~30% of acute infections. Host genetics play a major role in HCV clearance, with a strong effect of single nucleotide polymorphisms (SNPs) of the IL28B gene already found in different populations, mostly infected with viral genotypes 1 and 3. Egypt has the highest prevalence of HCV infection in the world, which is mostly due to viral genotype 4. We investigated the role of several IL28B SNPs in HCV spontaneous clearance in an Egyptian population. We selected nine SNPs within the IL28B genomic region covering the linkage disequilibrium (LD) block known to be associated with HCV clearance in European populations. These SNPs were genotyped in 261 HCV-infected Egyptian subjects (130 with spontaneous clearance and 131 with chronic infection). The most associated SNPs were rs12979860 (P = 1.6 × 10(-7)) and the non-synonymous IL28B SNP, rs8103142 (P = 1.6 × 10(-7)). Interestingly, three SNPs at the two bounds of the region were monomorphic, reducing the size of the LD block in which the causal variants are potentially located to ?20 kilobases. HCV clearance in Egypt was associated with a region of IL28B smaller than that identified in European populations, and involved the non-synonymous IL28B SNP, rs8103142. PMID:22719902

Pedergnana, Vincent; Abdel-Hamid, Mohamed; Guergnon, Julien; Mohsen, Amira; Le Fouler, Lénaïg; Theodorou, Ioannis; Mohamed, Mostafa Kamal; Fontanet, Arnaud; Plancoulaine, Sabine; Abel, Laurent

2012-01-01

396

Synthesis and evaluation of some lube oil additives from Egyptian coal tar phenols  

Energy Technology Data Exchange (ETDEWEB)

n-Dodecyl coal tar phenols obtained from Egyptian coal tar were condensed with different molar ratios of formaldehyde and triethylenetetramine to give three Mannich bases with different structures. The physicochemical characteristics of these compounds were determined and they were evaluated as lube oil additives. They gave promising results as antioxidants, dispersants, pour point depressants and viscosity index improvers.

Ahmed, M.H.; Ghuiba, F.M.; Habib, O.M.O.; Gharieb, H.K. (Mansoura University (Egypt))

1989-06-01

397

Exploring Egyptian EFL Students' Learning Styles and Satisfaction with Web-Based Materials  

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Due to the increasing demand on the incorporation of web-based materials into Egyptian higher educational institutions, this study examined students' learning styles, satisfaction with online learning, and the relationships between these two variables and students' gender. The study was conducted on 51 EFL college students (24 males and 27…

Aliweh, Ahmed Mahmoud

2011-01-01

398

On the research activities in reactor and neutron physics using the first egyptian research reactor  

International Nuclear Information System (INIS)

A review on the most important research activities in reactor and neutron physics using the first Egyptian Research Reactor (ET-RR-1) is given. An out look on: neutron cross-sections, neutron flux, neutron capture gamma-ray spectroscopy, neutron activation analysis, neutron diffraction and radiation shielding experiments, is presented

399

A PCR-RFLP Method for the Analysis of Egyptian Goat MHC Class II DRB Gene  

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Full Text Available This study aimed to analysis the genetic polymorphisms of MHC class II DRB gene in the Egyptian goat using PCR-RFLP method. The amplified fragment with size of 285-bp was digested by two restriction enzymes TaqI and PstI. Restriction digestion of PCR product by TaqI enzyme represented two digested fragments at 122- and 163-bp (T restriction pattern or undigested fragment at 285-bp (t restriction pattern. The frequencies of TT, Tt and tt patterns of MHC class II DRB in the Egyptian goats were 29.5, 61.4 and 9.1%, respectively. After the PstI digestion of the 285-bp PCR products amplified by Egyptian goat DNA, the results showed that the frequency of pp pattern (270- and 15-bp restricted fragments was 29.5% and the frequency of heterozygous