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Nutritional Biomarkers in Children and Adolescents with Beta-Thalassemia-Major: An Egyptian Center Experience  

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Background and Aim. Trace elements and vitamins play a vital role in human body to perform its function properly. Thalassemic patients are at risk of micronutrient deficiency. This study estimated levels of vitamins A, C, E, B12, folic acid, total homocysteine (tHcy), and methylmalonic acid (MMA) along with trace elements, zinc, copper, and selenium in Beta-thalassemia-major patients. Methods. This study included 108 patients with Beta-thalassemia-major and 60 age and sex matched healthy chil...

Sherief, Laila M.; Abd El-salam, Sanaa M.; Kamal, Naglaa M.; Osama El safy; Almalky, Mohamed A. A.; Azab, Seham F.; Morsy, Hemat M.; Gharieb, Amal F.

2014-01-01

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Portal vein thrombosis after splenectomy for beta-thalassemia major  

International Nuclear Information System (INIS)

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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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 in beta-thalassemia major due to the chronic hypercoagulable state which has been recognized to exist in childhood thalassemia and contribute to thromboembolic events. We are reporting one patient with beta-thalassemia major developed portal vein thrombosis following splenectomy.

Zakaria M. Al-Hawsawi

2004-02-01

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Malocclusion in subjects with beta-thalassemia major  

OpenAIRE

Introduction: Introduction: Beta-thalassemia major is a hemolytic anemia. If these patients are not treated, bone marrow hyperplasia will happen. Hematopoiesis in the jaw results in mandibular and/or maxillary enlargement. To our knowledge, there are few studies about frequency of malocclusion in these patients. Therefore, this study was designed to investigate the frequency of malocclusion in beta-thalassemia patients and compare it to healthy control group. Materials and Methods: This resea...

Shahsevari, F.; Eslami, M.; Ferhvesh, M. H.

2007-01-01

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Malocclusion in subjects with beta-thalassemia major  

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Full Text Available Introduction: Introduction: Beta-thalassemia major is a hemolytic anemia. If these patients are not treated, bone marrow hyperplasia will happen. Hematopoiesis in the jaw results in mandibular and/or maxillary enlargement. To our knowledge, there are few studies about frequency of malocclusion in these patients. Therefore, this study was designed to investigate the frequency of malocclusion in beta-thalassemia patients and compare it to healthy control group. Materials and Methods: This research was a cross sectional study. Seventy beta-thalassemia patients and 70 healthy controls (age and sex matched were examined for malocclusion using Angle classification, WHO malocclusion classification, determination of overjet and overbite. If crossbite, spacing, openbite, crowding and mouth breathing were observed in clinical examination they were recorded. Statistical analysis was done using Chi-square and Variants analysis. Results: Beta-thalassemia patients showed class II malocclusion (angle classification and grade 2 (WHO classification more than healthy control group. There were significant differences in angle & WHO malocclusion classification between two groups (P<0.05. Increased overjet and mouth breathing were seen in patients more than healthy control group. There were significant differences in overjet and mouth breathing between two groups (P<0.05. There was a positive correlation between WHO malocclusion classification and serum ferritin levels, but not between angle malocclusion classification, overjet and serum ferritin levels. Conclusion: Beta-thalassemia major is associated with increased malocclusion and these anomalies will occur if the patients are not treated early. Patients with malocclusion should be visited and followed up by a dentist for dental problems. Fortunately, as a result of new treatments these patients live longer than before. Therefore, it is important to pay attention to their functional and esthetical problems.

F. Shahsevari

2007-08-01

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Endocrine disorders in beta thalassemia major in Indian children  

OpenAIRE

The association of endocrine disorders with Thalassemia was evaluated in a large cohort of Indian children with Beta Thalassemia Major and compared to available data on children from developed countries, employing measurements of standing and sitting height, onset of puberty and of development of Diabetes Mellitus and, or hypoparathyroidism. Age at onset of blood transfusions, average pretransfusion Hemoglobin levels, serum Ferritin, ALT, Random blood sugar, serum calcium, phospho...

Sachdeva, Anupam; Khanna, Virendra Kumar; Subhash, Chander Arya; Dayal, Archana Araya; Yaddanapudi, Ravindranath

2002-01-01

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Hemoglobin D/beta-thalassemia and beta-thalassemia major in a Saudi family.  

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The present report describes the clinical, hematological and molecular characteristics in a family with unique interaction between 3 different mutations discovered during routine workup for bone marrow transplantation. In this report, complete hematological and molecular studies were performed for a large Saudi family. The family consisted of parents and 9 children, which revealed that the father is compound heterozygous for hemoglobin Hb D Punjab/beta-thalassemia, the mother is a carrier for beta-thalassemia and 3 of their children are transfusion dependent beta-thalassemia. Two of the children are compound heterozygous for Hb D Punjab/beta-thalassemia like the father but with different genotype. The other 2 children have Hb D Punjab traits while 2 other children have beta-thalassemia traits. Although, compound heterozygous for Hb D/beta-thalassemia has been well described in the literature, our report emphasizes the importance of careful analysis of the electrophoresis results and the usefulness of molecular studies in premarital screening and other screening hemoglobinopathy programs.

Tarek M. Owaidah

2005-04-01

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

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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 ?-thalas...

Riewpaiboon Arthorn; Nuchprayoon Issarang; Torcharus Kitti; Indaratna Kaemthong; Thavorncharoensap Montarat; Ubol Bang-on

2010-01-01

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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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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.

Mashaalah Khanehmasjedi

2012-07-01

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Assessment Hepatomegaly and liver Enzymes in 100 Patients with beta Thalassemia Major in Mashhad, Iran  

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AbstractBackgroundFrequent blood transfusion in patients with beta thalassemia major can lead to iron overloadespecially in liver. Chronic iron overload could cause cirrhosis of the liver. Transfusiontransmittedhepatitis B and C also could develop cirrhosis in individuals.Materials and MethodsThe present cross- sectional descriptive study is to assess hepatomegaly and liver enzymes in 100patients with beta thalassemia major, ages between 2-18 years old. The study was carried outretrospectivel...

Sh, Kolagari; Noori R; Hashemizadeh H

2012-01-01

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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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Evaluation of Mental Health and Related Factors among Patients with Beta-thalassemia Major in South East of Iran  

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Objective: Beta-thalassemia major (?-TM) is a chronic, genetic and hematological disorder. Children and teenagers with chronic physical illnesses exemplified by thalassemia are vulnerable to emotional and behavioral problems. The aim of this study was to evaluate mental health and its related factors among young patients with beta-thalassemia major. Methods: In this cross-sectional observational descriptive-analytic study, we studied 164 patients suffering from Beta-thalassemia major wit...

Morteza Ashrafi; Mohammad Reza Hormozi; Majid Naderi; Abolfazl Emamdadi

2012-01-01

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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.

Shima Mohammadian

2011-03-01

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

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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 inter...

Murtadha Al-Khabori; Sunil Bhandari; Mohammed Al-Huneini; Khalil Al-Farsi; Vinodh Panjwani; Shahina Daar

2013-01-01

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Lefort 1 osteotomy in a beta-thalassemia major patient--a case report.  

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Maxillary setback of patients with protruded maxilla is a complex procedure. This complexity is aggravated when combined with thalassemia. The Aim of this case report was to treat a beta-thalassemia major girl with severe maxillary protrusion. A 16 year-old beta-thalassemia major girl presented with excessive maxilla, incompetent lips and an everted upper lip was referred for treatment. She had her spleen removed at the age of 9 and her blood transfusion was stopped 3 month before referral. After leveling and aligning with 018 standard edgewise system the patient was sent for Lefort 1 osteotomy. Segmental osteotomy was denied due to excessive bleeding in the procedure. The results showed that the thalassemic patient was effectively treated with presurgical orthodontics, Lefort 1 osteotomy followed by post surgical orthodontics. Very satisfactory esthetic results were obtained in the patient after 4.5 years of active treatment. PMID:23941024

Showkatbakhsh, Rahman; Behnia, Hossein; Jamilian, Abdolreza; Heydarpour, Majid; Fetrati, Assal

2013-01-01

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Peripheral blood and hemoglobin electrophoresis pattern in beta thalassemia major patients receiving repeated blood transfusion  

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Full Text Available One hundred and fifteen beta thalassemia major outpatients attending the Thalassemia Center Department of Child Health, Medical School University of Indonesia Dr. Cipto Mangunkusumo General Hospital for routine blood transfusion and hematology examination, participated in this study. There was a negative correlation between the size of the spleen and the peripheral blood parameters. All peripheral blood parameters tend to decrease with the enlargement of the spleen, and the condition is reversed after splenectomy. We observed that hypersplenism starts when the spleen is as big as S (V – VI. The hemoglobin electrophoresis pattern from beta thalassemia major patients receiving repeated blood transfusion did not show a dense HbF fraction, 90 patients showed a normal hemoglobin electrophoresis pattern. A hemoglobin analysis of both parents could be useful to confirm the diagnosis of beta thalassemia major for patients receiving repeated blood transfusion. In order to get a definite diagnosis, a genetic analysis by bio molecular technique is needed. (Med J Indones 2004; 13: 8-16 Keywords: ? thalassemia major, hematology parameter, hemoglobin electrophoresis

Riadi Wirawan

2004-02-01

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Psychological Aspects in Young Adults with Beta-Thalassemia Major, control group  

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Background and purpose: Beta-thalassemia major (TM), a chronic, genetically determined hematological disorder, has received little investigation on the psychological aspects of the disease and the psychosocial adjustment of patients with this anemia. In the present study, the aim was to explore the nature of psychopathology according to age, sex, school performance, severity and complications of the disease in TM patients compared with demographically matched healthy persons.Materials and Met...

S H Hosseini, M. D.; ? H Khani, M. D.; A R Khalilian, Ph D.; K Vahidshahi, M. D.

2007-01-01

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Hypoparathyroidism and Intracerebral Calcifications in Patients with Beta-Thalassemia Major  

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Background/Objective: 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."nPatients and Methods: Forty-seven beta-thalassemia patients with hypoparathyroidism underwent a brain CT scan to investigate the presence and extent of intracerebral calcifications. Thirty age and sex-matched beta-thalassemic...

Nabavizadeh, A.; Karimi, M.; Rasekhi, A.; Rasekh, M.; Assadsangabi, R.; Gh. Amirhakimi

2008-01-01

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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 < 0.001) and were directly correlated to the amount of transfused iron but not to actual 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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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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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.

Nurcan Beyaz?t

2011-03-01

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EVALUATION OF T LYMPHOCYTE SUBSETS IN CHILDREN WITH BETA THALASSEMIA MAJOR  

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Peripheral blood T lymphocytes and their subsets were studied in 31 patients with beta thalassemia major (age 2-12years) and compared with 14 age-arid sex-matched healthy controls. Three monoclonal antibodies (anti-CD3, anti CD-f, unti-CDS) were simultaneously applied for detection of Th (CD3-, CD4^), Tsk (CD3+, CD8+) and Th/Ts ratio by flow-cytometry respectively. The results of this study showed a slight increase in the number of Tlymphocytes, T 004^, TCDS+, and CD4'*/CDS* ratio; but t...

Danesh, A.; Mir-ahmadian, M.

1999-01-01

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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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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

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Detection of Left Ventricular Regional Function in Asymptomatic Children with beta-Thalassemia Major by Longitudinal Strain and Strain Rate Imaging  

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Objective: Cardiac failure due to iron overload remains the most common cause of death in patients with beta-thalassemia major. This study aimed to evaluate myocardial function in children with beta-thalassemia major using standard echocardiography technique and strain rate imaging.

Bay, Ali; Bas?p?nar, Osman; Leblebisatan, Go?ksel; Yalc??n, Ali Sec?kin; I?rdem, Ahmet

2013-01-01

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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 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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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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Assessment Hepatomegaly and liver Enzymes in 100 Patients with beta Thalassemia Major in Mashhad, Iran  

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Full Text Available AbstractBackgroundFrequent blood transfusion in patients with beta thalassemia major can lead to iron overloadespecially in liver. Chronic iron overload could cause cirrhosis of the liver. Transfusiontransmittedhepatitis B and C also could develop cirrhosis in individuals.Materials and MethodsThe present cross- sectional descriptive study is to assess hepatomegaly and liver enzymes in 100patients with beta thalassemia major, ages between 2-18 years old. The study was carried outretrospectively. One hundred medical records have chosen from 400 samples of thalassemiamajor patients, who are under a regular care of the department of sarvar clinic.ResultsOut of these patients, 55% were male and 45% female. The mean age of thalassemia patients was10.8 4.4 years. The mean and S. D of hemoglobin, ferritin, deferoxamine dosage was 8.5 ±1.5g/dl , 2183 ± 1528 ng , 30 ± 11.16 mg/kg, respectively. Forty six percent of them hadhepatomegaly. The mean and S. D of AST and ALT were 95± 70 IU/L and 70 ?35U/Lrespectively. Splenectomy was performed on 44% of patient.ConclusionHepatomegaly is one of the most common findings in the thalassemic patient that induced withhemosiderosis and hepatitis.

kolagari SH

2012-12-01

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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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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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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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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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Serum levels of lipids and lipoproteins in Syrian patients with beta-thalassemia major.  

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The results might suggest that beta- thalassemia may represent an interesting metabolic model: anemia, an activated macrophage system and defective liver function seem interrelated to the final serum lipoprotein pattern. This suggests that antioxidants counteract lipid peroxidation processes and have a protective effect against oxidative damage of red cells of beta-thalassemia patients.

Faizeh A. Al-Quobaili

2004-07-01

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Assessment of Hypothyroidism in Children with Beta-Thalassemia Major in North Eastern Iran  

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Full Text Available AbstractBackground 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.Materials and MethodsThis research is a descriptive – cross sectional study, carried out in 2009 to assess thyroid function in 100 patients with beta thalassemia major at the ages between 2-18 years. The study was carried out retrospectively and 100 medical records from 400 samples of thalassemia major patients, under regular care of Sarvar Clinic, were assessed. Thyroid function and iron load status were evaluated by measuring the serum total triiodothyronine (T4, thyroid-stimulating hormone (TSH and ferritin levels from the serum of patients, admitted to the Sarvar Clinic. TSH and T4 concentrations were estimated by enzyme-linked immunosorbent assay (ELISA. Primary hypothyroidism was defined by a TSH level >4µIU/ml. Results were analyzed by descriptive statistical methods, with the help of SPSS software.ResultsSubclinical hypothyroidism was seen in 7% patients. All of them had normal T4 levels with elevated TSH levels, consistent with a diagnosis of subclinical hypothyroidism. Mean age of hypothyroid patients was 10.2 ± 2.5 years. Frequency of hypothyroidism was associated with increased serum ferritin levels (p=0.037.ConclusionSubclinical hypothyroidism occurs in a significant proportion of thalassaemia major patients in the absence of obvious clinical signs of hypothyroidism. Regular follow-up for early detection and timely treatment of such complications could improve the quality of life of these patients.

Noori R

2012-09-01

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

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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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Peripheral Blood Stem Cell Transplantation in Patients with Beta-Thalassemia Major  

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Full Text Available From 1996 to 2002, fifty three patients with major beta-thalassemia received allogeneic peripheral blood stem cell trans¬plantation (PBSCT. Median age was 6 years .Twenty two were class I, 17 class II and 14 class III. All of the donors were HLA-identical. Conditioning regimen for class I and II patients consisted of Cyclophosphamide (CY 50 mg/kg/day for 4 days + Busulfan (Bu 3.5 mg/kg for 4 days, while class III patients received 4 mg/kg/day Busulfan for 4 days and 40mg/kg/day Cyclophosphomide for 4 days. G-CSF (Neopogen 5µ/kg IV was given to donors. Graft Versus Host Disease (GVHD prophylaxis regimen consisted of Cyclosporin-A (CsA 3 mg/kg/day plus Methotrexate (MTX 10 mg/m2 on day+1 and 6 mg/m2 on days +3 and +6. The median time for neutrophil and platelet engraftment was day +16 and day +23 post transplantation, respectively. Chronic GVHD (cGVHD was observed in 30 patients (56%. Ten patients (18.8% died. Forty patients are well and transfusion independent. Median time of follow-up was 23 months. Recurrences have been seen at 3 pts, one patient 21 months, the other one 6 months and the last one 8 months after transplantation, who received Donor Lymphocyte infusion (DLI. Event free survival was 72% and overall survival was 80%. In conclusion, we suggest that PBSCT can be considered a safe and effective treatment for children with Beta-thalassemia major and cGVHD is tolerable and manageable in these patients.

Ghavamzadeh A

2004-08-01

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Early markers of renal dysfunction in patients with beta-thalassemia major.  

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Studies of renal involvement in thalassemia syndromes have been varied and few. The most important cause of mortality and morbidity in these patients is organ failure due to iron deposition. We report here a cross-sectional study carried out between February 2005 and February 2006 on all beta-thalassemia major patients being treated in Mofid Children's hospital, Tehran. The aim of the study was to detect renal dysfunction in these patients. The patient cohort consisted of 103 patients with various disease severities. Fresh first morning urine samples were collected and analyzed for sodium (Na), potassium (K), calcium (Ca), creatinine (Cr), phosphate, uric acid (UA), N-acetyl beta-D-glucosaminidase (NAG) and amino acids. We also carried out a complete blood count evaluation and assayed fasting blood sugar and serum ferritin, sodium, potassium, creatinine, uric acid and amino acids in all patients. The mean age of our patient cohort was 12.5+/-5.53 years and 53.4% were female. Abnormal levels of urinary NAG were detected in 35.9% of patients (confidence interval 26-45%). Abnormal levels of fractional excretion (FE)-Na, FE-K and FE-UA and abnormal urine protein Pr/Cr and urine Ca/Cr ratios were present in 29.1, 7.8, 52.4, 0.3 and 22.3% of the patients, respectively. There was a significant relationship between urinary NAG and the age of the patient (R=0.35), duration of deferoxamine therapy (R= 0.31), duration of receiving blood transfusions (R=0.34) and level of fasting blood sugar (R=0.2). We concluded that renal disorders are not rare in patients with beta-thalassemia major and that they may increase in terms of frequency with age, increased duration of transfusion and deferoxamine usage and high levels of blood sugar. PMID:18288499

Mohkam, Masoumeh; Shamsian, Bibi Shahin; Gharib, Atoosa; Nariman, Shahin; Arzanian, Mohammad T

2008-06-01

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Beta-Thalassemia Major and Female Fertility: The Role of Iron and Iron-Induced Oxidative Stress  

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Endocrine complications due to haemosiderosis are present in a significant number of patients with beta-thalassemia major (BTM) worldwide and often become barriers in their desire for parenthood. Thus, although spontaneous fertility can occur, the majority of females with BTM is infertile due to hypogonadotropic hypogonadism (HH) and need assisted reproductive techniques. Infertility in these women seems to be attributed to iron deposition and iron-induced oxidative stress (OS) in various end...

Paraskevi Roussou; Tsagarakis, Nikolaos J.; Dimitrios Kountouras; Sarantis Livadas; Evanthia Diamanti-Kandarakis

2013-01-01

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Comparative Sero-Prevalence Investigation of Helicobacter Pylori Infection in Beta Thalassemia Major Patients, Referred to Taleghani Center, Gorgan, Iran  

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Background and objectives: Recurrent Abdominal Pain (RAP) syndrome is acommon disorder, especially in children with beta thalassemia major. Thesepatients are predisposed to heart diseases which are caused byhemochromatosis (Iron overload), resulting in sudden death. Because of therole of Helico bacter pylori in causing abdominal pain and peptic ulcer, and inincreasing the risk or exacerbating of heart diseases, it can be important in betathalassemia major. This study aimed at determining the ...

Sm, Hedayat Mofidi; Kouhsar F; Hedayat MofidiH

2010-01-01

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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.

Seyed-Taghi Heydari

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.

Abolhassan Faramarzi

2010-09-01

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Psychological Aspects in Young Adults with Beta-Thalassemia Major, control group  

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Full Text Available Background and purpose: Beta-thalassemia major (TM, a chronic, genetically determined hematological disorder, has received little investigation on the psychological aspects of the disease and the psychosocial adjustment of patients with this anemia. In the present study, the aim was to explore the nature of psychopathology according to age, sex, school performance, severity and complications of the disease in TM patients compared with demographically matched healthy persons.Materials and Methods: A controlled anterograde cohort study was conducted at the Thalassemia Unit of Boo-Ali Hospital from June 2003 to November 2005 in Sari, Iran. Psychological aspects were evaluated by the Persian version of symptoms checklist-90-revised questionnaire. Information on relevant demographic characteristics, school performance, severity and complications of the disease was collected by one of the investigators who had created the questionnaire.Results: 125 persons with TM completed the questionnaires and were compared with 125 controls and 250 totally. The mean age of the participants was 18.51± 2.0 years and with a range of 15-25 years. 132 (52.8% were female with equal family status, social and economic status. Patients group reported a significantly lower level of marital status (P<0.01, education level (P<0.0001, school performance (P<0.0001. TM patients were found to have significantly more psychiatric disorders than the control subjects with GSI: 1.16 ± 0.47 vs. 1.01 ± 0.6; (P<0.03, PSD: 54.99 ± 12.59 vs. 46.42 ± 18.76 (P<0.0001, and PSDI 2.02±1.02 vs 2.45 ± 2.22 (P<0.05. We recorded significant changes in the mean scores of somatization (P<0.0001, interpersonal sensitivity (P<0.0001, depression (P<0.003, anxiety (P<0.05 and psychoticism (P<0.03 in the TM patients as compared to the control subjects.Conclusion: These findings show that beta-thalassemia major patients are at risk for psychiatric symptomatology and need appropriate psychiatric consultation.

S. H. Hosseini, M.D.

2007-09-01

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Evaluation of Mental Health and Related Factors Among Patients with Beta-Thalassemia Major in South East of Iran  

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Full Text Available Objective: Beta-thalassemia major (?-TM is a chronic, genetic and hematological disorder. Children and teenagers with chronic physical illnesses exemplified by thalassemia are vulnerable to emotional and behavioral problems. The aim of this study was to evaluate mental health and its related factors among young patients with beta-thalassemia major. Methods: In this cross-sectional observational descriptive-analytic study, we studied 164 patients suffering from Beta-thalassemia major with age range of 15-24 years who referred for treatment to Ali Ebn-e Abitaleb (AS University Hospital in Zahedan, a city in South East of Iran, during 2009- 2010. The demographic data and pattern of mental health were collected by standard general health questionnaire (GHQ-28.Data was analyzed using statistical software SPSS (version 17.0; Student t test and Chi-square (?2 were used. Results: In this study, 96 (58.5% patients were male; the mean age of all patients was 18.78 ±2.28. Based on data analysis, 83 patients (50.8% suspected to have psychiatric disorders (58.8% of girls, 44.8% of boys. In addition, frequency of somatic symptoms, depression disorder, anxiety disorder and social dysfunction in all patients were 7.3%, 11.6%, 8.5% and 4.3% respectively. In illiterate patients, 70.4% suspected to have psychiatric disorder. Except for somatic disorder, other mental disorders were more frequent in girls. No significant association was found between mental state and gender, marital and literacy status and occupation. Conclusion : In this study, due to high prevalence of psychological disorders in young patients with Beta-thalassemia major, especially in girls, we suggest implementing further educational psychological programs to decrease the frequency of disorders. Moreover, conducting more quantitative and comprehensive researches is suggested to evaluate specific effective factors in psycho-social health.

Morteza Ashrafi

2012-04-01

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Renal functions in pediatric patients with beta-thalassemia major: relation to chelation therapy: original prospective study  

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Abstract Background In ?-thalassemia, profound anemia and severe hemosiderosis cause functional and physiological abnormalities in various organ systems. In recent years, there have been few published studies mainly in adult demonstrating renal involvement in ?-thalassemia. This prospective study was aimed to investigate renal involvement in pediatric patients with transfusion dependant beta-thalassemia major (TD-?TM), using both conventional and early markers of glomerula...

ElMelegy Nagla T; Hamed Enas A

2010-01-01

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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

52

Efficacy of Chicory in Decreasing Serum Ferritin and Liver Enzymes in Major Beta Thalassemia Patients  

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Full Text Available AbstractObjectiveThalassemia major is a severe transfusion-dependent anemia that needs iron chalation therapy to remove iron overload. The objectives of the present study were to assess the iron overload liver response to inulin of chicory supplementation by evaluating the serum ferritin and liver enzymes.MethodsAmong 70 beta thalassemia patients, 50 were selected for chelating therapy using inulin of chicory. The initial dose was 1gr given twice a day. Twenty patients were excluded because of Hepatitis B and C and cardiac heart failure.ResultsFrom 50 patients, 47 patients tolerated chicory, which the majority showed dramatic responses. Mean serum ferritin level decreased from 3563.09 ng/ml to 1728.54 ng/ml. Mean serum AST level decreased from 25.44 u/lit to 22.25 u/lit. Mean serum ALT level decreased from 30 .861u/lit to 25.085u/iit. Serum ferritin level decreased significantly after treatment (PV? 0.00, but there was no significant difference in AST (PV=0.379 and Alt (0.367 after chicory treatment.ConclusionThe present results suggest that chicory can reduce iron over load and liver enzymes. Significant differences in serum ferritin were found during intervention, but not in LFT enzymes.

Shahvazian N

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Hypoparathyroidism and Intracerebral Calcifications in Patients with Beta-Thalassemia Major  

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Full Text Available Background/Objective: 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."nPatients and Methods: Forty-seven beta-thalassemia patients with hypoparathyroidism underwent a brain CT scan to investigate the presence and extent of intracerebral calcifications. Thirty 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 as control group. The amount of intracerebral calcifications, hematologic parameters, and some clinical findings were com-pared between both groups."nResults: Intracerebral calcification was present in 54.2% of beta -thalassemai 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. Conversely intracerebral calcification was not seen in the 30 thalassemia patients with normal parathyroid function. There was not a statistically significant difference between serum ferritin concentrations in thalassemia patients with hy-poparathyroidism and those with normal parathyroid function (2781 vs. 2178, P>0.05."nConclusion: Intracranial calcification is a common finding in thalassemia patients with "nhypoparathyroidism. It can be extensive and involves most regions of the brain.

A. Nabavizadeh

2008-01-01

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

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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 ...

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

2013-01-01

56

Beta-thalassemia  

OpenAIRE

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 thalass...

Origa Raffaella; Galanello Renzo

2010-01-01

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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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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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Endocrinopathies in Turkish children with Beta thalassemia major: results from a single center study.  

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The endocrinological complications in ?-thalassemia major patients do affect the life quality to a large extend. In this study, the endocrinological complications of 47 ?-thalassemia patients, who have been followed-up at our hospital's pediatric hematology department, were evaluated. Out of ?-thalassemia major cases included to this study, the 55.3% was male and 44.7% was female. The patients' mean levels of ferritin, whose mean age was 10.0 ± 4.5 years (2-20 years), were 2497 ± 1469 ng/mL (472-8558 ng/mL). At least one endocrinological pathology in 27 out of 47 (57.4%) and more than one endocrinological pathology in 14 out of 47 (29.7%) thalassemia patients were observed. The most frequently observed complication in followed-up cases was vitamin D insufficiency and deficiency (78.2%). The other complications in decreasing order were pubertal failure (41.6%), growth retardation (25.5%), decreased bone-mineral density (22.2%), secondary hyperparathyroidism (11.5%), overt hypothyroidism (4.25%), subclinical hypothyroidism (2.12%), and impaired glucose tolerance (2.12%). There was no statistically significant difference between serum mean ferritin level and endocrin complications (P > .05). Four patients (8.5%) had decreased signal intensity in pituitary magnetic resonance imaging (MRI) but this finding was not associated with ferritin levels (P = .87). MRI parameters were similar between patients with and without gonadal dysfunction. Mean height of the pituitary gland was 4.98 ± 1.1 mm (3-9 mm) and this was similar to those normal values in the literature. Ferritin levels were not correlated with pituitary height (P > .05). Beta thalassemia major, having the potential of leading to multisystemic complications, is a chronic disease that should be treated and followed-up by a multidisciplinary approach. Due to frequently encountered endocrinological complications, beta thalassemic patients should be followed-up regularly by hematology and endocrinology departments in coordination. PMID:24854890

Isik, Pamir; Yarali, Nese; Tavil, Betül; Demirel, Fatma; Karacam, Gül?ah Bayram; Sac, Rukiye Unsal; Fettah, Ali; Ozkasap, Serdar; Kara, Abdurrahman; Tunc, Bahattin

2014-10-01

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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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Thyroid Function Status and Echocardiographic Abnormalities in Patients with Beta Thalassemia Major in Bahrain  

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Background: Thyroid gland dysfunction and echocardiographic cardiac abnormalities are well-documented in patients with transfusion dependent beta-thalassemia major (?-TM). Aim: This cross-sectional analytic study was conducted to investigate left ventricle (LV) diastolic and systolic function using pulsed Doppler (PD) and tissue Doppler (TD) echocardiography and correlate that with serum level thyroid stimulating hormone in patients with ?-TM. Methods: The study was conducted on patients with ?-TM (n = 110, age 15.9 ± 8.9 years) and compared with a control group (n = 109, age 15.8 ± 8.9 years). In all participants, echocardiographic indices of PD and TD were performed and blood samples were withdrawn for measuring the serum level of TSH, free T4, and ferritin. A linear regression analysis was performed on TSH level as the dependent variable and serum ferritin as independent. Stepwise multiple regression analysis was used to determine the odds ratio of different biochemical and echo variables on the risk of developing hypothyroidism. Results: Patients with ?-TM compared with controls had thicker LV septal wall index (0.65 ± 0.26 vs. 0.44 ± 0.21 cm/M2, P < 0.001), posterior wall index (0.65 ± 0.23 vs. 0.43 ± 0.21 cm/m2, P < 0.01) and larger LVEDD index (4.35 ± 0.69 vs.3.88 ± 0.153 mm/m2, P < 0.001). In addition, ?-TM patients had higher transmitral E wave velocity (E) (70.81 ± 10.13 vs. 57.53 ± 10.13 cm/s, P = 0.02) and 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). Furthermore, 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 the ?-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 the ?-TM group compared with controls with Sm, 4.82 ± 1.2 vs. 6.22 ± 2.1 mm/sec, P < 0.05 and (Em), 3.51 ± 2.7 vs. 4.12 ± 2.5 mm/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 subclinical hypothyroidism in patients with ?-TM was 15.4%, with significantly higher mean serum TSH compared with controls (6.78 ± 1.5 vs. 3.10 ± 1.02 ?IU/mL, P < 0.01) and positively correlated with the serum ferritin level (r = 0.34, P = 0.014). On multiple regression analysis, the LV mass, LVEF%, and E/A ratio were not positive predictors of hypothyroidism in patients with ?-TM. Conclusion: We conclude that patients with ?-TM had a high prevalence of subclinical hypothyroidism of 15.4%. Thyroid stimulating hormone was significantly high and positively correlated with the serum ferritin level. Echo cardiographic pulsed Doppler showed a restrictive LV diastolic pattern suggestive of severe diastolic dysfunction with preserved left ventricle systolic function. PMID:23400522

Garadah, Taysir S.; Mahdi, Najat A.; Jaradat, Ahmed M.; Hasan, Zuheir A.; Nagalla, Das S.

2013-01-01

62

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

63

Comparative Sero-Prevalence Investigation of Helicobacter Pylori Infection in Beta Thalassemia Major Patients, Referred to Taleghani Center, Gorgan, Iran  

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Full Text Available Background and objectives: Recurrent Abdominal Pain (RAP syndrome is acommon disorder, especially in children with beta thalassemia major. Thesepatients are predisposed to heart diseases which are caused byhemochromatosis (Iron overload, resulting in sudden death. Because of therole of Helico bacter pylori in causing abdominal pain and peptic ulcer, and inincreasing the risk or exacerbating of heart diseases, it can be important in betathalassemia major. This study aimed at determining the frequency of anti-H.pylori class IgA in patients suffered from beta thalassemia major.Material and Methods: In this descriptive-analytic Study, 132 betathalassemia major Patients and 135 healthy cases were matched by age, sexand ethnic as a control group. The sera were separated from clot in firstSeroepidemiology and along with the filled questionnaires sent toImmunology-hematology Laboratory of paramedical school, in which thesamples were tested by ELISA method with Diagnostic kit for anti-H.pyloriIgA and IgG classes detection. We analyzed the Data by chi square andIndependent T and Fisher tests.Results: the results show that the difference between the anti-H.pylori classIgA in case (22.7% and control group (17.8% is not significant (P=0.6, Butit is significant between anti-H.pylori IgG class, 81.8% for cases and 54.8%for controls (P<0.001.Conclusion: Based on the results, the rate of anti-H.P IgA class in case is1.131 times more than control group, which is not significant. On the otherhand , the rate of anti-H.P IgG is 1.478 times more than control group which issignificant.Key words: Thalassemia, Helicobacter pylori, Gorgan, Sero epidemiology

Hedayat Mofidi SM

2010-01-01

64

Associates of poor physical and mental health-related quality of life in beta thalassemia-major/intermedia  

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  • BACKGROUND: Using two logistic regression models, we determined the associates of poor physical and mental health related quality of life (HRQoL among beta thalassemia patients.
  • METHODS: In this cross-sectional study which was conducted during 2006 and 2007 in outpatient adult thalassemia clinic, Blood Transfusion Organization, Tehran, Iran, Short Form 36 (SF-36 was used for measuring HRQoL in 179 patients with beta thalassemia (major/intermedia. We determined scores higher than third quartiles of obtained PCS and MCS scores as the cutoff points of good HRQoL. Poor HRQoL was defined scores lower than first quartiles of obtained PCS and MCS scores. Two distinct logistic regression models were used to derive associated variables including demographic, clinical, and psychological factors.
  • RESULTS: The regression models suggested that poor physical HRQoL was positively associated with somatic comorbidities (OR = 1.472, CI = 1.021-2.197, p = 0.048 and depression score (OR = 8.568, CI = 2.325-31.573, p = 0.001. The variables that were associated with poor mental HRQoL were anxiety score (OR = 9.409, CI = 1.022-89.194, p = 0.049 and depression score (OR = 20.813, CI = 4.320-100.266, p < 0.001.
  • CONCLUSIONS: Depression is associated with both poor physical and mental HRQoL among patients with major/intermedia beta thalassemia, however somatic comorbidities and anxiety are associated with poor physical and mental HRQoL, respectively.
  • KEYWORDS: Thalassemia, Health Related Quality of Life, Anxiety, Depression, Somatic Comorbidities.

Azita AzarKeyvan

2009-11-01

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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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Phase II clinical evaluation of deferasirox, a once-daily oral chelating agent, in pediatric patients with beta-thalassemia major  

OpenAIRE

BACKGROUND AND OBJECTIVES: Deferasirox (ICL670) is a novel once-daily oral iron chelator developed for the treatment of chronic iron overload from blood transfusions. This study evaluated the safety and tolerability of deferasirox in pediatric patients with transfusion-dependent beta-thalassemia major. Efficacy and pharmacokinetic assessments were secondary objectives. DESIGN AND METHODS: Forty patients equally stratified into two age groups--children (2 to <12 years) and adolescents (12-17 y...

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  

OpenAIRE

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.)

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

69

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

International Nuclear Information System (INIS)

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.)

70

Beta-thalassemia  

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Full Text Available 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. 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.

Origa Raffaella

2010-05-01

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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 - 0.496, 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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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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Associates of poor physical and mental health-related quality of life in beta thalassemia-major/intermedia  

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  • BACKGROUND: Using two logistic regression models, we determined the associates of poor physical and mental health related quality of life (HRQoL) among beta thalassemia patients.
  • METHODS

    Azita AzarKeyvan; Bashir HajiBeigi; Maryam Moghani Lankarani; Mahshid Namdari; Shervin Assaric

    2009-01-01

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Continued improvement in myocardial T2(star) over two years of deferasirox therapy in beta-thalassemia major patients with cardiac iron overload  

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BackgroundThe efficacy of cardiac iron chelation in transfusion-dependent patients has been demonstrated in one-year prospective trials. Since normalization of cardiac T2* takes several years, the efficacy and safety of deferasirox was assessed for two years in patients with beta-thalassemia major in the cardiac sub-study of the EPIC trial.Design and MethodsEligible patients with myocardial T2* greater than 5 to less than 20 ms received deferasirox, with the primary endpoint being the change ...

Pennell, D. J.; Porter, J. B.; Cappellini, M. D.; Chan, L. L.; El-beshlawy, A.; Aydinok, Y.; Ibrahim, H.; Li, C. K.; Viprakasit, V.; Elalfy, M. S.; Kattamis, A.; Smith, G.; Habr, D.; Domokos, G.; Roubert, B.

2011-01-01

75

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

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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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Agranulocyosis in Beta Thalassemia Major Patients treated with Oral Iron Chelating Agent (Deferiprone)  

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Deferiprone is an oral chelating agent that has been recently shown to reduce cardiac siderosis, but is also known to be associated with serious side effects like agranulocytosis which can be fatal. This report is a single centre experience of 5 cases with severe agranulocytosis in amongst 144 patients (3.47%) of thalassemia major on combined chelation therapy with subcutaneous desferrioxamine and oral deferiprone which is much higher than the previous reports.

Wali, Yasser; Shidhani, Azza Al; Daar, Shahina

2008-01-01

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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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Renal tubular dysfunction in pediatric patients with beta-thalassemia major.  

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To evaluate the prevalence of renal tubular dysfunction in children with ?-thalassemia (?-T) major, we studied the glomerular and tubular function in 140 children with ?-T major and compared them to a healthy control group at our center from May 2007 to April 2008. Fresh first morning samples were collected from each patient and analyzed for sodium, potassium, calcium (Ca), protein, uric acid (UA), creatinine (Cr), urine osmolality and urinary N-acetyl-?-D-glucosaminidase (UNAG) activity. Blood samples were also collected for complete blood count, blood urea nitrogen (BUN), fasting blood sugar, serum creatinine (SCr), electrolytes, and ferritin before transfusion. Among the study patients, 72 were males, and the mean age was 11.5 (ranging 7-16) years. SCr levels were all within normal limits and all of them had normal glomerular filtration rate (GFR). The mean UNAG was 17.8 IU/L in the study patients (normal 0.15-11.5 IU/L) and 3.2 IU/L in the control group (P 0.21) (P = 0.006). Nine (6.4%) thalassemic patients with a mean age of 12 years had proteinuria (Upr/UCr > 0.2). Sixty-nine (49.3%) out of the 140 patients and 45 (65.2%) of the patients having UNAG had uricosuria also (UUA/UCr > 0.26). Ten (7%) patients had microscopic hematuria and 10 (7%) patients with a mean age of 13.5 years had glucosuria or diabetes mellitus. We conclude that tubular dysfunction is a relative common complication of the ?-T major; UNAG and its index are the best to detect renal tubular dysfunction in these patients. Currently, periodic measurement of UCa/UCr and UUA/UCr ratios as well as urinalysis are recommended. PMID:21566307

Ahmadzadeh, Ali; Jalali, Amir; Assar, Shiedeh; Khalilian, Hamid; Zandian, Khamorad; Pedram, Mohammad

2011-05-01

81

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

82

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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Renal functions in pediatric patients with beta-thalassemia major: relation to chelation therapy: original prospective study  

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Full Text Available Abstract Background In ?-thalassemia, profound anemia and severe hemosiderosis cause functional and physiological abnormalities in various organ systems. In recent years, there have been few published studies mainly in adult demonstrating renal involvement in ?-thalassemia. This prospective study was aimed to investigate renal involvement in pediatric patients with transfusion dependant beta-thalassemia major (TD-?TM, using both conventional and early markers of glomerular and tubular dysfunctions, and to correlate findings to oxidative stress and iron chelation therapy. Methods Sixty-nine TD-?TM patients (aged 1-16 years and 15 healthy controls (aged 3-14 years were enrolled in this study. Based on receiving chelation therapy (deferoxamine, DFO, patients were divided into two groups: group [I] with chelation (n = 34 and group [II] without chelation (n = 35. Levels of creatinine (Cr, calcium (Ca, inorganic phosphorus (PO4, uric acid (UA and albumin were measured by spectrophotometer. Serum (S levels of cystatin-C (SCysC and total antioxidant capacity (STAC and urinary (U levels of ?2-microglobulin (U?2MG were measured by immunosorbent assay (ELISA. Urinary N-acetyl-beta-D-glucosaminidase (UNAG activity and malondialdehyde (UMDA were measured by chemical methods. Estimated glomerular filtration rate (eGFR was determined from serum creatinine. Results In patient with and without chelation, glomerular [elevated SCysC, SCr, Ualbumin/Cr and diminished eGFR]; and tubular dysfunctions [elevated SUA, SPO4, UNAG/Cr, U?2MG/Cr] and oxidative stress marker disturbances [diminished STAC and elevated UMDA/Cr] were reported than controls. In patients with chelation, SCysC was significantly higher while, STAC was significantly lower than those without chelation. In all patients, SCysC showed significant positive correlation with SCr and negative correlation with eGFR; STAC showed significant positive correlation with eGFR and negative correlation with SCysC, SCr, UNAG/Cr; UMDA/Cr showed significant positive correlation with Ualbumin/Cr, U?2MG/Cr, UNAG/Cr. Conclusions Our data confirm high frequency of glomerular and tubular dysfunctions in TD-?TM pediatric patients which could be attributed to oxidative stress and DFO therapy.

ElMelegy Nagla T

2010-05-01

84

Lipid peroxidation and antioxidant status in beta-thalassemia.  

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Autoxidation of globin chains and iron overload are the suggested mechanisms for the increased oxidative stress in beta-thalassemia. The aim of this study was to evaluate the extend of lipid peroxidation and antioxidant status of patients with beta-thalassemia and iron deficiency anemia (IDA) and compare the results with healthy subjects. Oxidant and antioxidant status of the children with beta-thalassemia major (n = 22) and iron deficiency anemia (n = 19) were studied. Healthy controls (n = 14) were age and sex matched. Fresh anticoagulated venous blood samples were obtained from all children. Conjugated diene (CD) and thiobarbituric acid-reactive (TBARS) substances were analyzed to indicate the oxidative parameters, whereas the erythrocyte superoxide dismutase (SOD) and glutathione peroxidase (GPx) were measured to show the antioxidant status of the children. Plasma TBARS and CD concentrations were elevated in beta-thalassemia compared to IDA. When compared to the controls, elevation in TBARS was significant. In the iron-deficiency group both TBARS and CD levels were decreased compared to the controls. SOD and GPx activities were increased in the beta-thalassemia group. SOD in beta-thalassemia was higher than both IDA and the controls and GPx activity was higher than the IDA group. In vivo lipid peroxidation was increased in children with beta-thalassemia major. This leads to a compensatory increase in antioxidant enzymes, whereas IDA does not lead to lipid peroxidation with a normal antioxidant enzyme activity. PMID:11127401

Meral, A; Tuncel, P; Sürmen-Gür, E; Ozbek, R; Oztürk, E; Günay, U

2000-12-01

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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.

Afsaneh Ashrafi

2010-09-01

86

Genetics Home Reference: Beta thalassemia  

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... Patient support For patients and families Gene Reviews Clinical summary Genetic Testing Registry Genetic testing ClinicalTrials.gov Research studies PubMed Recent literature OMIM Genetic disorder catalog Conditions > Beta thalassemia On this page: Description Genetic changes Inheritance Diagnosis ...

87

Evaluation of Myocardial Performance Index(Mpi in Beta Thalassemia Major Patients at the Ali Asghar Hospital , Zahedan , Iran  

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Full Text Available Introduction : Patients with major thalassemia need recurrent transfusions and if not treated are at risk of heart dysfunction. Heart tissue could be abnormal in patients who use desferral continously due to iron deposits , fibrosis , hypertrophy and side effects of chronic anemia. Cardiac involvement is the main cause of morbidity and mortality in major thalassemics, so we decided to improve early diagnosis of cardiac involvement by measuring myocardial performance index( MPI . Method & Materials : This case-control study was performed from April 2003 to December 2003 at the Ali-Asghar pediatrics hospital, Zahedan, Iran. MPI of both ventricles was measured by Doppler echocardiography in 48 patients with major thalassemia aged between 10-18 years and compared with 48 age , sex-matched controls. Patients had no abnormality in physical examination , chest x-ray and ECG and echocardiography did not show heart failure. Hemoglobin and serum ferritin levels was measured in all patients before echocardiography. Results: Mean age of patients was 12.3±2.4 years and that in the control group was 12.7±2years . Right ventricle isovolumetric relaxation time (RVIRT (107 ± 14 vs 94 ±14 , p0.05 and LVICT (31 ± 13 vs 21 ± 15 , p0.05 in the two groups. Finally, RVMPI (0.59 ± 0.12 Vs 0.46 ± 0.12 , p<0.001 was increased in 87% of patients and LVMPI (0.49 ± 0.12 Vs 0.41± 0.09 , p0.05. Conclusion: This study showed that RVMPI and LVMPI increase in major thalassemia patients that indicates systolic and diastolic dysfunction and RVMPI increases more than LVMPI. On the other hand, MPI has a direct correlation with serum ferritin . On the basis of this study ,we suggest MPI measurement in serial echocardiography in asymptomatic major thalassemia patients.

R Sohrevardi

2004-04-01

88

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

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

OpenAIRE

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

2012-01-01

90

The pro-BNP Serum Level and Echocardiographic Tissue Doppler Abnormalities in Patients with Beta Thalassemia Major  

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Background Doppler echocardiographic studies of the left ventricle (LV) function in patients with ?-Thalassemia Major (?-TM) had shown different patterns of systolic and diastolic dysfunctions associated with abnormal serum brain natriuretic peptide (BNP). Aim This cross-sectional study was designed to study the LV systolic and diastolic functions and correlate that with serum level of N-terminal pro brain natriuretic hormone (NT- pro BNP) in patients with ?-TM using Pulsed Doppler (PD) and Tissue Doppler (TD) echocardiography. Methods The study was conducted on patients with ?-TM (n = 38, age 15.7 ± 8.9 years) and compared with an age-matched controls (n = 38, age 15.9 ± 8.9 years). In all participants, PD and TD echocardiography were performed and blood samples were withdrawn for measuring the serum level of NT-pro BNP, ferritin, and alanine transaminase. Results Patients with ?-TM compared with controls, have thicker LV septal wall index (0.65 ± 0.26 vs. 0.44 ± 0.21 cm, P < 0.001), posterior wall index (0.65 ± 0.23 vs. 0.43 ± 0.21 cm, P < 0.01), and larger LVEDD index (4.35 ± 0.69 vs.3.88 ± 0.153 mm, P < 0.001). In addition, ?-TM patients have higher transmitral E wave velocity (E) (70.818 ± 10.139 vs. 57.532 ± 10.139, p = 0.027) and E/A ratio (1.54 ± 0.17 vs. 1.23 ± 0.19, P < 0.01) and shorter deceleration time (DT) (160.13 ± 13.3 vs. 170.50 ± 19.20 m sec, P < 0.01). Furthermore, 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 (19.6 ± 2.81 vs. 13.868 ± 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 to controls (Sm: 4.82 ± 1.2 vs. 6.22 ± 2.1 mm/sec, P < 0.05; Em: 3.51 ± 2.7 vs. 4.12 ± 2.5 mm/sec P < 0.05, respectively). The tricuspid valve velocity was significantly higher in ?-TM patients compared with controls (2.993 ± 0.569 vs. 1.93 ± 0.471 m/sec, respectively, P < 0.01). The mean serum NT pro-BNP in ?-TM was significantly higher compared with controls (37.6 ± 14.73 vs. 5.5 ± 5.4pg/ml, P < 0.05). The left ventricle ejection fraction (EF%) and fractional shortening (FS%) were not significantly different between both groups. Conclusion We conclude that patients with ?-TM had a significantly higher serum level of NT-pro BNP that is positively correlated with the E/Em ratio on tissue Doppler. Furthermore, we confirm our previous findings that patients with ?-TM exhibit LV diastolic pattern on echocardiogram suggestive of restrictive type with well preserved left ventricle systolic function. PMID:21234293

Garadah, Taysir S.; Mahdi, Najat; Kassab, Salah; Shoroqi, Isa Al; Abu-Taleb, Ahmed; Jamsheer, Anwer

2010-01-01

91

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

Science.gov (United States)

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

2014-12-01

92

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

93

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

Science.gov (United States)

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* 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). Am. J. Hematol. 90:91-96, 2015. © 2014 Wiley Periodicals, Inc. 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

94

Plastic bronchitis in beta thalassemia minor.  

Science.gov (United States)

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. PMID:24049256

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

2013-07-01

95

A Synthetic Model of Human Beta-Thalassemia Erythropoiesis Using CD34+ Cells from Healthy Adult Donors  

OpenAIRE

Based upon the lack of clinical samples available for research in many laboratories worldwide, a significant gap exists between basic and clinical studies of beta-thalassemia major. To bridge this gap, we developed an artificially engineered model for human beta thalassemia by knocking down beta-globin gene and protein expression in cultured CD34+ cells obtained from healthy adults. Lentiviral-mediated transduction of beta-globin shRNA (beta-KD) caused imbalanced globin chain production. Beta...

Lee, Y. Terry; Kim, Ki Soon; Byrnes, Colleen; Vasconcellos, Jaira F.; Noh, Seung-jae; Rabel, Antoinette; Meier, Emily R.; Miller, Jeffery L.

2013-01-01

96

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

Science.gov (United States)

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. PMID:25525381

Boonyawat, Boonchai; Monsereenusorn, Chalinee; Traivaree, Chanchai

2014-01-01

97

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

Directory of Open Access Journals (Sweden)

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

98

Camperdown hemoglobin associated with beta° thalassemia in a Brazilian child  

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Full Text Available We report the coexistence of Hb Camperdown [beta104 (G6 Arg -> Ser] and beta°-thalassemia [beta39 (Gln -> stop codon] in a nine-month-old Brazilian boy. He had a relatively more severe hypochromic and microcytic anemia in comparison to his mother's beta-thalassemia trait. His Hb Camperdown heterozygous father was clinically and hematologically normal. To our knowledge, this is the first description of an association of beta°-thalassemia with Hb Camperdown.

Tania Regina Tozetto-Mendoza

2005-09-01

99

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)

100

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.

101

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

102

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

Directory of Open Access Journals (Sweden)

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

103

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

OpenAIRE

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...

Anzel Bahad?r; Onur Öztürk; Ayfer Atalay; Erol Ömer Atalay

2009-01-01

104

Diffuse Hepatic Calcifications in a Transfusion-Dependent Patient with Beta-Thalassemia: A Case Report  

OpenAIRE

Hepatic calcification is usually associated with infectious, vascular, or neoplastic processes in the liver. We report the first case of beta-thalassemia major with isolated diffuse hepatic calcification in a 23 year old woman, who had been transfusion-dependent since the age of 6 months. She was referred to our center with a chief complaint of abdominal pain. Computed tomography scan of the abdomen revealed diffuse hepatic calcification in the right, left, and caudate lobes of the liver. Her...

Forough Saki; Mohammad Reza Bordbar; Mohammad Hadi Imanieh; Mehran Karimi

2013-01-01

105

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

106

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.

107

RBC alloimmunization in blood transfusion-dependent beta-thalassemia patients in southern Iran.  

Science.gov (United States)

beta-thalassemia is considered a severe, progressive anemia, which needs regular transfusions for life expectancy. One of the most important complications of regular blood transfusions may be alloimmunization, which increases the need for transfusion. This study was performed to investigate the production of red cell alloantibodies in beta-thalassemia patients in Shiraz, southern Iran. Blood sampling was performed among 711 beta-thalassemia patients in Dastgheib hospital in 2002-2004. Direct and indirect coombs tests were performed to check the auto and alloantibodies and a panel test was conducted to detect the type of alloantibodies. Auto and alloantibodies were observed among 1.7% and 5.3% of patients, respectively. The most common alloantibodies were Anti-kell (50%) > Anti-Rh (D) (15.8%) > Anti-Rh (E) (10.5%). All the patients who had developed alloantibody were in the age group of 6 years or more. So for decreasing the rate of alloantibody synthesis, we should crossmatched the packed cells for minor blood groups especially for kell and Rh(E) in addition to major blood groups from the start of transfusion. PMID:17824911

Karimi, M; Nikrooz, P; Kashef, S; Jamalian, N; Davatolhagh, Z

2007-10-01

108

Survey of Hfe Gene C282Y Mutation in Turkish Beta-Thalassemia Patients and Healthy Population: A Preliminary Study.  

Science.gov (United States)

Objective: This study was planned in order to determine the effect of C282Y mutation in development of secondary hemochromatosis in beta-thalassemia patients and to determine the prevalence and allele frequency of this mutation in a healthy control group. Materials and Methods: Eighty-seven children and young adults (46 males and 41 females; mean age: 15.6±6.1 years, range: 3-30 years) with beta-thalassemia major (BTM) and 13 beta-thalassemia intermedia (BTI) patients (6 males and 7 females; mean age: 19.6±3.5 years, range: 13-26 years) were included in the study. The control group comprised 100 healthy blood donors. Results: Neither heterozygous nor homozygous HFE gene C282Y mutation was detected in patients with BTM or BTI, or in control group. Conclusion: The C282Y mutation, which is supposed to be responsible for the majority of hereditary hemochromatosis, was not found to have a role in the development of hemochromatosis in beta-thalassemia patients and was not detected in a healthy Turkish population. However, research on larger cohorts of individuals is required in order to determine the exact prevalence of the HFE gene mutation in Turkish populations from diverse ethnic origins and whether it would have an impact on iron loading in thalassemic populations. PMID:25330520

Unal, Selma; Balta, Günay; Gümrük, Fatma; Xu, Hong-Gui

2014-09-01

109

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

110

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

111

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

112

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

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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

113

Prospective assessment of health-related quality of life in pediatric patients with beta-thalassemia following hematopoietic stem cell transplantation.  

OpenAIRE

Although hematopoietic stem cell transplantation (HSCT) has been widely used to treat pediatric patients with beta-thalassemia major, evidence showing whether this treatment improves health-related quality of life (HRQoL) is lacking. We used child-self and parent-proxy reports to prospectively evaluate HRQoL in 28 children with beta-thalassemia from Middle Eastern countries who underwent allogeneic HSCT in Italy. The PedsQL 4.0 Generic Core Scales were administered to patients and their paren...

Caocci, G.; Efficace, F.; Ciotti, F.; Roncarolo, Mg; Vacca, A.; Piras, E.; Littera, R.; Dawood Markous, Rs; Collins, Gs; Ciceri, F.; Mandelli, F.; Marktel, S.; La Nasa, G.

2011-01-01

114

Hemoglobin D/beta-thalassemia and beta-thalassemia major in a Saudi family.  

OpenAIRE

ABSTRACT The present report describes the clinical, hematological and molecular characteristics in a family with unique interaction between 3 different mutations discovered during routine workup for bone marrow transplantation. In this report, complete hematological and molecular studies were performed for a large Saudi family. The family consisted of parents and 9 children, which revealed that the father is compound heterozygous for hemoglobin Hb D Punjab/beta-thal...

Owaidah, Tarek M.; Al-saleh, Mahasen M.; Al-hellani, Ali M.

2005-01-01

115

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

Science.gov (United States)

Clinical and hematological 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 beta-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; R2=0.343) and the validation conducted on the testing set confirmed its predictive accuracy with transfusion free survival probability (pgenetic 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

2014-12-01

116

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' )

117

COMBINED USE OF ERYTHROCYTE ZINC PROTOPORPHYRINE AND MCV: DIFFERENTIATION OF BETA THALASSEMIA FROM IRON DEFICIENCY ANEMIA  

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Full Text Available Introduction: Due to the prevalence of beta thalassemia and iron deficiency anemia in Irana need for an accurate, quick, inexpensive and simple method for differential diagnosis between these two disorders is felt. In this study the value of zpp measurement as a tool to distinguish iron deficiency from heterozygous beta thalassemia in microcrystal patients was evaluated. Methods: In this study on 140 persons with microcytosis, the hemoglobin electrophoresis, serum iron, TIBC and serum ferritin and protoporphyrine relating to zinc (ZPP were measured. Results: ZPP had been increased in all iron deficient patients and in 53 percent of Heterozygote beta thalassemia patients. Using combination of MCV and ZPP in iron deficient anemic patients and heterozygote beta thalassemia show the 99 percent accuaracy in differentiation of these two disorders. Discussion: According to the results, this method has better accuracy than red blood cells formulation in the screening programs for beta thalassemia and iron defficiency anemia.

H KHOSHAMOOZ

2001-12-01

118

Expression of a beta thalassemia gene with abnormal splicing.  

OpenAIRE

Expression of a cloned human beta thalassemia gene with a single base change at position 5 of IVS 1 has been analyzed 48 hours after transfer of the gene into HeLa cells (transient expression). Little or no normal beta globin mRNA accumulates in the presence of the abnormal beta gene in contrast to significantly more normal beta mRNA produced with other mutations at this same position. By contrast, large amounts of an abnormal beta globin mRNA are present; this is due to the use of a cryptic ...

Lapoumeroulie, C.; Acuto, S.; Rouabhi, F.; Labie, D.; Krishnamoorthy, R.; Bank, A.

1987-01-01

119

Molecular characterization of beta-thalassemia in Pakistan.  

Science.gov (United States)

Beta-thalassemia is one of the most common inherited hemoglobin disorders in Pakistan. The carrier frequency is estimated to be 5.4%. To determine the spectrum of beta-globin gene defects causing beta-thalassemia, we have analyzed a representative sample of 602 alleles from six ethnic groups in Pakistan; 99.2% alleles were characterized, while 0.8% remained unidentified. The spectrum of mutations is heterogeneous and we have found 19 different mutations in all ethnic groups. The four most common mutations, IVS-I-5 (G-->C) (37.7%), codons 8/9 (+G) (21.1%), the 619 bp deletion (12.4%), and IVS-I-1 (G-->T) (9.5%), account for 80.7% of the alleles. There are differences between the ethnic groups and also between provinces. In the four provinces of Pakistan, the IVS-I-5 (G-->C) mutation is more prevalent in Sindh and Balochistan, bordering India in the south and Iran in the southwest, while the codons 8/9 (+G) mutation is more common in the Punjab and the North West Frontier Province, bordering India in the northeast and Afghanistan, respectively. The 619 bp deletion is high (46%) in Gujratis and Memons residing in the Province of Sindh, neighboring the Indian Gujrat. PMID:9730364

Khan, S N; Riazuddin, S

1998-07-01

120

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

121

Diffuse hepatic calcifications in a transfusion-dependent patient with Beta-thalassemia: a case report.  

Science.gov (United States)

Hepatic calcification is usually associated with infectious, vascular, or neoplastic processes in the liver. We report the first case of beta-thalassemia major with isolated diffuse hepatic calcification in a 23 year old woman, who had been transfusion-dependent since the age of 6 months. She was referred to our center with a chief complaint of abdominal pain. Computed tomography scan of the abdomen revealed diffuse hepatic calcification in the right, left, and caudate lobes of the liver. Her medical history disclosed hypoparathyroidism as well as chronic hepatitis C virus infection, which was successfully treated but led to early micronodular cirrhosis on liver biopsy. Other studies done to search for the cause of hepatic calcification failed to reveal any abnormalities. We suspect that hypoparathyroidism caused liver calcification, and should be, therefore, considered in the differential diagnosis of hepatic calcification if other causative factors have been ruled out. PMID:24174700

Saki, Forough; Bordbar, Mohammad Reza; Imanieh, Mohammad Hadi; Karimi, Mehran

2013-09-01

122

Deniz: the electronic database for beta-thalassemia mutations in the Arab world.  

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In addition to its importance as a hub of updated information on the distribution of beta-thalassemia mutations in Arabs, information in Deniz may be used to predict diagnostic strategies that may be offered to natives of unstudied countries. Incidence data may also give important clues on the possible origins of beta-thalassemia in the Arab world. The integration of Deniz with other databases is currently in process and researchers are invited to contribute to the growth of the database.

Ghazi O. Tadmouri

2003-11-01

123

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

OpenAIRE

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 reported 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 r...

Esteva?o, Isabeth F.; Souza, Maria Cristina S.; Manzato, Antonio J.; Bonini-domingos, Claudia R.

2010-01-01

124

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

125

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 | Language: 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

126

Renal complications of beta-thalassemia major in children  

Science.gov (United States)

The success that has been made in the care of patients with thalassemia has led to the emergence of unrecognized complications including several renal abnormalities. Chronic anemia and iron overload as well as the use of iron chelator are believed to lie behind these abnormalities. Many investigators document the presence of tubular dysfunction and abnormalities in glomerular filtration rate in these patients. In this review we will discuss the updates in the diagnosis, pathogenesis and prevention of renal complications of thalassemia. PMID:25232499

Bakr, Ashraf; Al-Tonbary, Youssef; Osman, Ghada; El-Ashry, Rasha

2014-01-01

127

Diagnostic value of fingerprint patterns: an explorative study on beta-thalassemia diagnosis.  

Science.gov (United States)

Rapid diagnosis of major beta thalassemia along with certain preventive measures is of utmost significance. The present study aims to compare the fingerprints in Major beta thalassemic patients (67) and in their parents (76 with minor thalassemia) with the normal fingerprints of control group (144). A forensic medical examiner determined fingerprint types of arch, loop, whorl and other types. Like normal individuals, loop fingerprint pattern was found to be the most common fingerprint type among thalassemic patients. However, the number of whorl fingerprints in all fingers in thalassemic patients was greater than that of normal individuals and the number of loop fingerprints was smaller (p < 0.05). Arch type fingerprint pattern was less frequent in major thalassemic patients compared to minor thalassemic ones (p < 0.05). The findings show that the number of whorl fingerprint patterns in thalassemic patients was greater than that of normal individuals, while the number of loop fingerprint patterns being smaller and the frequency of arch fingerprint pattern in patients with major thalassemia was lower than that of minor thalassemic patients. Therefore, one may choose fingerprint pattern as a simple, affordable and appropriate screening method to help detect the afflicted patients and prevent severe cases of thalassemia. PMID:21280556

Solhi, Hassan; Hashemieh, Mojgan; Nejad, Mohammad Lorgard Dezfuli; Vishteh, Hamid-Reza Khoddami; Nejad, Maryam Rahmati

2010-04-01

128

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.

129

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

Directory of Open Access Journals (Sweden)

Full Text Available 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 reported 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

2010-01-01

130

Prevention of homozygous beta-thalassemia by carrier screening and prenatal diagnosis in Sardinia.  

Science.gov (United States)

We report here results of a 3-year pilot voluntary screening program coupled with prenatal diagnosis directed to the prospective prevention of homozygous beta-thalassemia (beta-thal) in Sardinia. The screening program took two approaches: outreach community testing and hospital testing on request after a period of sensibilization. The outreach testing was very effective as, taking into account the already known number of couples at risk with an affected proband (20), 74% of the couple at risk expected (61) on the basis of the carrier rate were identified. Less effective was the hospital testing in which half of the couples at risk expected were detected (502 with the 199 without an affected proband). After nondirective genetic counseling, approximately 85% of the couples at risk, which had a pregnancy, with no statistically significant difference between those with and those without a proband, requested prenatal testing. This figure showed a steadily increase from the beginning in 1977 to 1980. All the pregnancies (42), but two carrying homozygous fetuses, were terminated on parental request. A continuous hospital survey of thal-major admissions in the different hospitals of the counties showed a steady decline in the incidence figure at birth from 1976 (1:213) to 1978 (1:290). These results showed that even in a medium-developed, rural, Catholic population screening coupled with prenatal diagnosis can be successful in the control of a fatal, recessively inherited disorder. PMID:7258188

Cao, A; Furbetta, M; Galanello, R; Melis, M A; Angius, A; Ximenes, A; Rosatelli, C; Ruggeri, R; Addis, M; Tuveri, T; Falchi, A M; Paglietti, E; Scalas, M T

1981-07-01

131

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

Energy Technology Data Exchange (ETDEWEB)

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

132

Fusobacterium nucleatum prosthetic hip infection in an adult with sickle cell-beta thalassemia.  

Science.gov (United States)

Fusobacterium nucleatum is an anaerobic Gram-negative bacillus commensal to the human oropharynx and gastrointestinal tract which causes an array of human infection, yet it has never been associated with infection of prosthetic joints. We report the first case of prosthetic hip infection caused by F. nucleatum in a man with sickle cell-beta thalassemia. PMID:22002730

Verma, K; McNabb, P C; Kurtz, W; Green, J; Trabue, C H

2012-06-01

133

Cerebrovascular accident in beta-thalassemia major (beta-TM) and beta-thalassemia intermedia (beta-TI).  

Science.gov (United States)

Chronic hypercoagulable state expressed clinically by thromboembolic events has been described in thalassemia. One of the affected organs is the brain where symptomatic and asymptomatic damage has been reported. The present report describes seven cases who presented with the signs of cerebrovascular accident (CVA), five ischemic and two with hemorrhage. Two of them died. All patients were splenectomized, five received regular blood transfusions, and their ferritin levels were between 1,200 and 3,000 mg %. In addition, four patients had congestive heart failure and atrial fibrillation, and three had "Bronze diabetes," The recommendation on the basis of the results is that well-designed clinical trials are indicated to monitor asymptomatic brain damage by magnetic resonance imaging in splenectomized patients over the age of 20 years, who are not regularly transfused and have a high risk to develop thromboembolic events. In this subset of patients, anticoagulant and/or antiplatelet therapy should be considered. Moreover, treatment of additional complications resulting from iron overload, which may contribute to the etiology of CVA such as cardiac failure and arrhythmia with or without "bronze diabetes," is mandatory. PMID:17696211

Karimi, Mehran; Khanlari, Mahsa; Khanlari, Maryam; Rachmilewitz, Eliezer A

2008-01-01

134

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

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Full Text Available 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 transfusion. Qualitative analysis of these samples using ELISA and PCR. The PCR positive samples were subjected to genotyping by RFLP method. Of total 245 beta-thalassemia patients who were the subjects of this study, 28 of these patients were diagnosed through PCR test to have RNA virus. For this reason, the prevalence of this illness in this study group was estimated as 11.42%. By using the RFLP technique, the above genotyping were identified and the prevalence of three genotypes, including 3a, 1a and 1b were proved. The genotype 3a was most prevalent. Out of 28 positive samples, 18 (64.3% samples had this genotype. After that, genotype 1a with 9 positive occurrences (32.1% and genotype 1b with only 1 positive occurrence (3.6% were most prevalent. This study demonstrated that the main reason the beta-thalassemia patients became infected with the genotype of the virus was due to receiving infected blood that entered into Iran during the past two decades.

Maryam Farahani

2012-01-01

135

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)

136

Iron metabolism in heterozygotes for hemoglobin E (HbE), alpha-thalassemia1, or beta-thalassemia and in compound heterozygotes for HbE/ beta-thalassemia  

OpenAIRE

BACKGROUND: Despite large populations carrying traits for thalassemia in countries implementing universal iron fortification, there are few data on the absorption and utilization of iron in these persons. OBJECTIVE: We aimed to determine whether iron absorption or utilization (or both) in women heterozygous for beta-thalassemia, alpha-thalassemia 1, or hemoglobin E (HbE) differed from that in control subjects and compound HbE/beta-thalassemia heterozygotes. DESIGN: In Thai women (n = 103), re...

Zimmermann, M. B.; Fucharoen, S.; Winichagoon, P.; Sirankapracha, P.; Zeder, C.; Gowachirapant, S.; Judprasong, K.; Tanno, T.; Miller, J. L.; Hurrell, R. F.

2008-01-01

137

Prospective assessment of health-related quality of life in pediatric patients with beta-thalassemia following hematopoietic stem cell transplantation.  

Science.gov (United States)

Although hematopoietic stem cell transplantation (HSCT) has been widely used to treat pediatric patients with beta-thalassemia major, evidence showing whether this treatment improves health-related quality of life (HRQoL) is lacking. We used child-self and parent-proxy reports to prospectively evaluate HRQoL in 28 children with beta-thalassemia from Middle Eastern countries who underwent allogeneic HSCT in Italy. The PedsQL 4.0 Generic Core Scales were administered to patients and their parents 1 month before and 3, 6, and 18 months after transplantation. Two-year overall survival, thalassemia-free survival, mortality, and rejection were 89.3%, 78.6%, 10.9% and 14.3%, respectively. The cumulative incidence of acute and chronic graft-versus-host disease (GVHD) was 36% and 18%, respectively. Physical functioning declined significantly from baseline to 3 months after HSCT (median PedsQL score, 81.3 vs 62.5; P = .02), but then increased significantly up to 18 months after HSCT (median score, 93.7; P = .04). Agreement between child-self and parent-proxy ratings was high. Chronic GVHD was the most significant factor associated with lower HRQoL scores over time (P = .02). The child-self and parent-proxy reports showed improved HRQoL in the children with beta-thalassemia after HSCT. Overall, our study provides preliminary evidence-based data to further support clinical decision making in this area. PMID:20870029

Caocci, Giovanni; Efficace, Fabio; Ciotti, Francesca; Roncarolo, Maria Grazia; Vacca, Adriana; Piras, Eugenia; Littera, Roberto; Dawood Markous, Raji Suleiman; Collins, Gary Stephen; Ciceri, Fabio; Mandelli, Franco; Marktel, Sarah; La Nasa, Giorgio

2011-06-01

138

A family case of beta-thalassemia minor and hemoglobin Queens: alpha 34 (B15) Leu-Arg.  

OpenAIRE

We report a Korean family case of beta-thalassemia minor and Hb Queens. This is the first case report of Hb Queens in Korea. A 43-year-old male and his four family members had beta-thalassemia minor which is very rare in Korea. Incidentally, an alpha chain variant with a high isoelectric point was also found in two other family members without clinical problems and was finally identified as alpha 34 (B15) Leu-Arg or Hemoglobin Queens.

Lee, N. Y.; Cho, H. I.; Kim, S. I.; Kim, B. K.; Ohba, Y.; Hattori, Y.

1992-01-01

139

Two missense mutations in the beta-globin gene can cause severe beta thalassemia. Hemoglobin Medicine Lake (beta 32[B14]leucine-->glutamine; 98 [FG5] valine-->methionine).  

OpenAIRE

We studied the molecular basis of transfusion-dependent hemolytic anemia in an infant who rapidly developed the phenotype of beta thalassemia major. DNA sequence of one beta-globin gene of the proband revealed two mutations, one for the moderately unstable hemoglobin (Hb) Köln and another for a novel codon 32 cytosine-thymidine-guanine-->cytosine-adenine-guanine transversion encoding a leucine-->glutamine mutation. A hydrophilic glutamine residue at beta 32 has an uncharged polar side chain ...

Coleman, M. B.; Lu, Z. H.; Smith, C. M.; Adams, J. G.; Harrell, A.; Plonczynski, M.; Steinberg, M. H.

1995-01-01

140

Homozygous delta-beta Thalassemia in a Child: a Rare Cause of Elevated Fetal Hemoglobin  

OpenAIRE

AbstractBackgroundDelta beta (??) thalassemia is an unusual variant of thalassemia with elevated level of fetal hemoglobin (HbF). Homozygous patients of this disorder, unlike ?-thalassemia, show mild anemia. Only few cases of ??-thalassemia have been reported from India in the available indexed English literature.Case presentationA four-year old male child was evaluated for recent-onset jaundice. Hematological investigations showed mild anemia with microcytic hypochromic red cells. A com...

Sk Md, Mittal; Md, Gupta R.; Md, Bhargava M.; Md, Verma S.

2013-01-01

141

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

OpenAIRE

(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 molecul...

Mohammad Bagher Hashemi Soteh; Haleh Akhavan Niaki; Mehrnoosh Kowsarian; Aily Aliasgharian; Ali Banihashemi

2008-01-01

142

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

143

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

144

Prevention of homozygous beta-thalassemia by carrier screening and prenatal diagnosis in Sardinia.  

OpenAIRE

We report here results of a 3-year pilot voluntary screening program coupled with prenatal diagnosis directed to the prospective prevention of homozygous beta-thalassemia (beta-thal) in Sardinia. The screening program took two approaches: outreach community testing and hospital testing on request after a period of sensibilization. The outreach testing was very effective as, taking into account the already known number of couples at risk with an affected proband (20), 74% of the couple at risk...

Cao, A.; Furbetta, M.; Galanello, R.; Melis, M. A.; Angius, A.; Ximenes, A.; Rosatelli, C.; Ruggeri, R.; Addis, M.; Tuveri, T.; Falchi, A. M.; Paglietti, E.; Scalas, M. T.

1981-01-01

145

Prevalence and hematological characteristics of beta-thalassemia trait in Gaziantep urban area, Turkey.  

Science.gov (United States)

Thalassemia is one of the most common hereditary disorders in the Mediterranean region and studies have shown that the prevalence of beta-thalassemia trait is high in the southern part of Turkey. Gaziantep is a city located near this region and, therefore, the authors investigated the prevalence and hematological characteristics of the beta-thalassemia traits in primary school students in Gaziantep. Sixty primary schools were selected from a list of all primary schools using a systematic sampling method. Data were collected by a face-to-face questionnaire. Osmotic fragility testing (OFT) using single-tube 0.36% NaCl solution was used for the screening of beta-thalassemia. Students who were positive in regard to OFT went through a series of testing, including a complete blood count, serum ferritin levels, serum iron, and hemoglobin electroforesis. Chi-square test was used in statistical analysis. Of the 2439 students enrolled to the study from the selected 60 classrooms, 1353 (55.5%) were male and 1086 (44.5%) were female. The OFT was positive in 115 (4.7%) of the participants. CEA and confirmatory HPLC results of the students who were positive OFT indicated that 70 (60.8%) had normal results, 33(28.7%) showed high HbA2 levels, 7 (6.1%) showed high HbA2 and HbF levels, 5(5.2%) showed high HbA2 and Fe-deficiency anemia, and none showed increased HbF levels. The overall prevalence of beta-thalassemia trait was 1.84%. No gender differentials and highest rates among the Kahramanmaras (3.5%) and Sanliurfa (1.7%) born students were the other significant findings of this study. Implementation of a routine carrier-screening program offering genetic counseling, prenatal diagnosis, and selective termination of affected fetuses would be a wise approach to eliminate this disease from the region. PMID:16728362

Gurbak, Mehmet; Sivasli, Ercan; Coskun, Yavuz; Bozkurt, Ali Ihsan; Ergin, Ahmet

2006-01-01

146

Rare association between two genetic conditions: turner syndrome and beta thalassemia minor  

Directory of Open Access Journals (Sweden)

Full Text Available Rare disorders are defined as diseases, including those of genetic origin, which are life-threatening or chronically debilitating, which are of such low prevalence that special combined efforts are needed to address them. We present a case with a rare association between two genetic conditions: Turner phenotype and beta thalassemia minor. Turner syndrome is a chromosomal disorder that is characterized by the absence of all or part of a second sex chromosome in some or all cells. This condition occurs in 1 in 2,500 to 3,000 girls. The physical features include webbing of the neck, short stature, delayed growth of the skeleton, broad chest, cardivascular abnormalities and gonadal dysgenesis. Women with this disorder are usually infertile due to ovarian failure. The clinical diagnosis was confirmed by the cytogenetic and by FISH analysis, which revealed the presence of only one X chromosome. Treatment may include human growth hormone and estrogen replacement therapy. On the other hand, thalassemias are genetic conditions that result from imbalance in the normal coordinated synthesis of the globin subunits that make up the hemoglobin tetramer, leading to decreased and defective production of hemoglobin. Beta thalassemia syndromes are hereditary disorders characterized by a genetic deficiency in the synthesis of beta-globin chains. Beta thalassemia is inherited in an autosomal recessive manner. Thalassemia minor usually presents as an asymptomatic mild microcytic anemia, but our case also had splenomegaly and required splenectomy.

Dorina STOICANESCU

2009-11-01

147

Beta-thalassemia in the Po Delta: selection, geography, and population structure.  

OpenAIRE

The allele frequencies for beta-thalassemia for 51 localities in the province of Rovigo, and in 25 localities in the province of Ferrara, were studied. It was observed that in the province of Ferrara there is a significant cline of frequencies; these decrease from the coast of the Adriatic Sea toward the west. No such gradient was visible in Rovigo. It was advanced, also on the basis of geography documented by ancient maps, that in the province of Rovigo there were multiple foci of selection ...

Barrai, I.; Rosito, A.; Cappellozza, G.; Cristofori, G.; Vullo, C.; Scapoli, C.; Barbujani, G.

1984-01-01

148

Haematological characterisation and molecular basis of asian Indian inversion deletions delta Beta thalassemia: a case report.  

Science.gov (United States)

The hereditary persistence of fetal hemoglobin (HPFH) and delta beta thalassemia are heterogeneous disorders characterised by increased levels of fetal hemoglobin and high level of this Hb continues in adulthood. The distinction between these two conditions is not always possible with routine hematologic analysis and molecular characterisation of the defect is required. We encountered such a rare case of ? ? thalassemia in a 10-year-old male child who presented with features of thalassemia intermedia. Hemoglobin analysis showed 100% HbF while molecular analysis revealed Asian Indian inversion-deletion G?A?(? ?) zero thalassemia. PMID:25386442

Khunger, Jitender Mohan; Gupta, Monika; Singh, Rekha; Kapoor, Rohit; Pandey, Hare Ram

2014-09-01

149

Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with beta thalassemia hemoglobin E disease.  

Science.gov (United States)

Case report of a 28-year-old man with beta thalassemia/hemoglobin E disease who developed a massive hemothorax due to bleeding from multiple large intrathoracic, paraspinal hematopoietic masses. Initially, a thoracotomy was required for the control of bleeding. Postoperatively, the patient received 2,000 ml of packed red cells but he continued bleeding after the blood chemistries and coagulopathy specimen were corrected. The decision was made to return to the operating room for a thoracotomy to control the bleeding. However, before re-operating, the patient underwent a collapse and failed resuscitation. This complication has never been previously reported in Thailand. PMID:24319860

Tantraworasin, Apichat; Saeteng, Somcharoen

2013-07-01

150

Hemoglobin Mississippi (beta 44ser----cys). Studies of the thalassemic phenotype in a mixed heterozygote with beta +-thalassemia.  

OpenAIRE

Hemoglobin Mississippi (HbMS: beta 44ser----cys) has anomalous properties that include disulfide linkages with normal beta-, delta-, gamma-, and alpha-chains, and the formation of high molecular weight multimers. While heterozygotes for HbMS are clinically and hematologically normal and carriers of the beta +-thalassemia gene in our family had mild microcytic anemia, the proband with HbMS-beta +-thalassemia had a hemoglobin level of 7 g/dl, mean corpuscular volume (MCV) of 68 fl, reticulocyte...

Steinberg, M. H.; Adams, J. G.; Morrison, W. T.; Pullen, D. J.; Abney, R.; Ibrahim, A.; Rieder, R. F.

1987-01-01

151

Epidural anesthesia for laparoscopic cholecystectomy in a patient with sickle cell anemia, beta thalassemia, and Crohn's disease -A case report-.  

Science.gov (United States)

A 37-year-old woman diagnosed with sickle cell anemia (SCA), beta (+) thalassemia, Crohn's disease, and liver dysfunction was scheduled for laparoscopic cholecystectomy (LC) due to acute cholecystitis with gall bladder. Regional anesthesia was performed. An epidural catheter was inserted into the 9-10 thoracal epidural space and then 15 ml of 0.5% bupivacaine was injected through the catheter. The level of sensorial analgesia tested with pinprick test reached up to T4. Here we describe the first case of the combination of sickle cell anemia (SCA), beta (+) thalassemia, and Crohn's disease successful anesthetic management with attention to hemodynamics, particularly with regards to liver dysfunction. PMID:23115690

Ba?, Sema ?anal; Ozlü, Onur

2012-10-01

152

New optical coherence tomography fundus findings in a case of beta-thalassemia.  

Science.gov (United States)

Patients with beta-thalassemia may present with an acquired diffuse elastic tissue defect due to degeneration of elastic tissue along with vaso-occlusive findings in the retinal microvasculature. Here we report the case of a patient with granular-like accumulation presenting as black sunburst lesions detected by optical coherence tomography (OCT). A 38-year-old man with beta-thalassemia intermedia associated with angioid streaks complained of deterioration of vision in both eyes. Funduscopic examination revealed small, round, hyperpigmented lesions bilaterally. During the early and late phases of fluorescein angiography, granular hyperfluoresence was present, associated with pigment decompensation and mottled-like hypofluorescence. The main OCT finding was the presence of granuloid-like accumulations at the retinal pigment epithelium level. Granule penetration was also noticed at the photoreceptor layer, while isolated granuloid-like accumulations were found in the inner layers of the macula and choroid. In this case, the new OCT finding was the granular-like hyperpigmented accumulations in the macula located at the level of the retinal pigment epithelium. To the best of our knowledge, our OCT findings show for the first time granuloid-like accumulations representing black sunburst lesions. PMID:23277736

Eleftheriadou, Mi; Theodossiadis, Pg; Rouvas, A; Alonistiotis, D; Theodossiadis, Gp

2012-01-01

153

A comprehensive molecular characterization of beta thalassemia in a highly heterogeneous population.  

Science.gov (United States)

In Iran, the prevalence of beta-thalassemia trait is approximately 4-8% in most areas, and in Mazandaran province 10% of the population are carriers. Twenty four beta-globin gene mutations were identified in 1635 persons with beta-thalassemia trait using reverse dot blot and restriction fragment length polymorphism analysis. The predominant mutations included IVSII-1 (G-A) (61%), codon 30 (G-C) (7.5%), codon 22 (-7bp) (6.2%), codon 8 (-AA) (5.4%) and IVSI-5 (G-C) (3.6%). These mutations were in different haplotypes, with IVSII-1 being the most heterogeneous. Other less frequent mutations included IVS-II-745 (C-G), codon 44 (-C), codon 39 (C-T), codon 5 (-CT), IVS I-110 (G-A), IVSI-130 (G-C), Fr8/9 (+G), IVSI-1 (G-A), and IVSI (-25bp). All rare mutations except IVSI-130 were encountered in a unique haplotype. The diversity of these mutations reflects the historical admixture of genes in the region. The high prevalence of IVSII-1 (G-A) compared to other parts of the country and the world suggests a founder effect. Our data provide a basis for genetic counseling and prenatal diagnosis. PMID:21493114

Akhavan-Niaki, Haleh; Derakhshandeh-Peykar, Poupak; Banihashemi, Ali; Mostafazadeh, Amrollah; Asghari, Beheshteh; Ahmadifard, Mohammad-Reza; Azizi, Mandana; Youssefi, Ali; Elmi, Maryam Mitra

2011-06-15

154

Homozygous delta-beta Thalassemia in a Child: a Rare Cause of Elevated Fetal Hemoglobin  

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Full Text Available AbstractBackgroundDelta beta (?? thalassemia is an unusual variant of thalassemia with elevated level of fetal hemoglobin (HbF. Homozygous patients of this disorder, unlike ?-thalassemia, show mild anemia. Only few cases of ??-thalassemia have been reported from India in the available indexed English literature.Case presentationA four-year old male child was evaluated for recent-onset jaundice. Hematological investigations showed mild anemia with microcytic hypochromic red cells. A comprehensive analysis of hemoglobin by high-performance liquid chromatography (HPLC showed complete absence of HbA and HbA2 with HbF constituting 100% of the hemoglobin. Hemoglobin analysis of both parents showed elevated level of HbF with normal HbA2. A final diagnosis of ??-thalassemia in the child with both parents being carriers was rendered. ConclusionDelta beta-thalassemia is an uncommon cause of markedly elevated fetal hemoglobin beyond fetal period. Clinical and haematological parameters should be evaluated to render an accurate diagnosis.

Mittal SK MD

2013-03-01

155

An intracranial extramedullary hematopoiesis in a 34-year-old man with beta thalassemia: a case report  

OpenAIRE

Abstract Introduction Extramedullary hematopoiesis occurs in approximately 15% of cases of thalassemia. Intracranial deposits of extramedullary hematopoiesis are an extremely rare compensatory process in intermediate and severe thalassemia. Case presentation We present an unusual case of an intracranial extramedullary hematopoiesis with a choroid plexus origin in a 34-year-old Caucasian man with beta thalassemia intermedia, who presented with the complaints of c...

Tabesh Homayoun; Shekarchizadeh Ahmad; Mahzouni Parvin; Mokhtari Mojgan; Abrishamkar Saeid; Abbasi Fard Salman

2011-01-01

156

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)

157

Thyroid function in major thalassemia patients: Is it related to height and chelation therapy?  

OpenAIRE

Background: One of the most common endocrine problems in major beta-thalassemia is hypothyroidism (HT). The aim of this study was to evaluate thyroid function status in major ?-thalassemia patients older than 10 years old.

Eshragi, Peiman; Tamaddoni, Ahmad; Zarifi, Khadijeh; Mohammadhasani, Amir; Aminzadeh, Majid

2011-01-01

158

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

Science.gov (United States)

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

159

Takayasu's arteritis presenting with temporary loss of vision in a 23-year-old woman with beta thalassemia trait: a case report  

OpenAIRE

Abstract Introduction The simultaneous presence of Takayasu's arteritis and beta thalassemia trait is a rare combination. To the best of our knowledge, this is the first case report on Takayasu's arteritis and beta thalassemia presenting together. Case presentation This is a case report of a 23-year-old Asian woman of Pakistani descent who presented with a headache, blurred vision and dizziness. Conclusion The correct diagnosis of our patient was b...

Shabbir Fahad A; Ishaq Mohammad G

2011-01-01

160

Epidural anesthesia for laparoscopic cholecystectomy in a patient with sickle cell anemia, beta thalassemia, and Crohn's disease -A case report-  

OpenAIRE

A 37-year-old woman diagnosed with sickle cell anemia (SCA), beta (+) thalassemia, Crohn's disease, and liver dysfunction was scheduled for laparoscopic cholecystectomy (LC) due to acute cholecystitis with gall bladder. Regional anesthesia was performed. An epidural catheter was inserted into the 9-10 thoracal epidural space and then 15 ml of 0.5% bupivacaine was injected through the catheter. The level of sensorial analgesia tested with pinprick test reached up to T4. Here we describe the fi...

Bas?, Sema S?anal; O?zlu?, Onur

2012-01-01

161

Coexisting Iron Deficiency Anemia and Beta Thalassemia Trait: Effect of Iron Therapy on Red Cell Parameters and Hemoglobin Subtypes  

OpenAIRE

Background. Coexistence of iron deficiency anemia (IDA) and beta thalassemia trait (BTT) has been the topic of few studies. However, no study from our country was found evaluating the effect of iron therapy in patients with concomitant IDA and BTT. Methods. Over a period of two years, 30 patients with concomitant IDA and BTT were included. All the patients had a complete blood count, serum iron studies, and thalassemia screening using BIORADTM hemoglobin testing system. The patients received ...

Verma, Sarika; Gupta, Ruchika; Kudesia, Madhur; Mathur, Alka; Krishan, Gopal; Singh, Sompal

2014-01-01

162

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

163

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

OpenAIRE

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 subcuta...

Taneja Rashi; Malik Pankaj; Sharma Mamta; Agarwal Mahesh

2010-01-01

164

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

165

Glucose homeostasis in Egyptian children and adolescents with ?-Thalassemia major: Relationship to oxidative stress  

Science.gov (United States)

Background: Oxidative stress in children with ?-thalassemia may contribute to shortened life span of erythrocytes and endocrinal abnormalities. Aim: This study was aimed to evaluate glucose homeostasis in Egyptian children and adolescents with ?-thalassemia major and its relation to oxidative stress. Materials and Methods: Sixty children and adolescents with ?-thalassemia major were studied in comparison to 30 healthy age and sex-matched subjects. Detailed medical history, thorough clinical examination, and laboratory assessment of oral glucose tolerance test (OGTT), serum ferritin, alanine transferase (ALT), fasting insulin levels, plasma malondialdehyde (MDA) as oxidant marker and serum total antioxidants capacity (TAC) were performed. Patients were divided into two groups according to the presence of abnormal OGTT. Results: The prevalence of diabetes was 5% (3 of 60) and impaired glucose tolerance test (IGT) was 8% (5 of 60). Fasting blood glucose, 2-hour post-load plasma glucose, serum ferritin, ALT, fasting insulin level, homeostatic model assessment for insulin resistance index (HOMA-IR) and MDA levels were significantly elevated while TAC level was significantly decreased in thalassemic patients compared with healthy controls (P < 0.001 for each). The difference was more evident in patients with abnormal OGTT than those with normal oral glucose tolerance (P < 0.001 for each). We also observed that thalassemic patients not receiving or on irregular chelation therapy had significantly higher fasting, 2-h post-load plasma glucose, serum ferritin, ALT, fasting insulin, HOMA-IR, oxidative stress markers OSI and MDA levels and significantly lower TAC compared with either those on regular chelation or controls. HOMA-IR was positively correlated with age, serum ferritin, ALT, MDA, and negatively correlated with TAC. Conclusions: The development of abnormal glucose tolerance in Egyptian children and adolescents with ?--thalassemia is associated with alteration in oxidant-antioxidant status and increase in insulin resistance. Recommendation: 1- Glucose tolerance tests, HOMA-IR, and MDA should be an integral part of the long-term follow-up of children and adolescents with ?-thalassemia major. 2- Regular iron chelation and antioxidant therapy should be advised for thalassemic patients to improve glucose hemostasis. PMID:24944927

Metwalley, Kotb Abbass; El-Saied, Abdel-Rahman Abdel-Hamed

2014-01-01

166

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)

167

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

168

Serological Evaluation of Major Beta Thalassemia Patients below15 for Cytomegalovirus Infection in Iran  

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Full Text Available Thalassemia is one of the most prevalent human genetic diseases. Thalassemia genes are prevalent all over the world especially in countries on Thalassemia belt which Iran is among them. With due attention to the prevalence of this genetic disease in Iran and patients` vital needs for regularly blood transfusion, it is necessary to study Transfusion-transmitted cytomegalovirus infection in this patients. Current study has been carried out on 309 Thalassemia patients below 15 who were receiving blood from therapeutic centers repeatedly. ELISA method was used to determine CMV-IgM seroposivity for cytomegalovirus, Also 225 healthy people below 15 were selected as control group and finally all data were analyzed by SPSS statistical software. The results of this study showed that 12.9 % of under study Thalassemia patients were seropositive for CMV-IgM antibody and 95.1% were negative. Regional separation of patients showed that the patients resided in Tehran are more infected to this virus than patients of other towns. According to this study prevalence of CMV-IgM antibody significantly higher than normal population, therefore immunity care of these patients and use CMV seronegative blood units and also hematic parents (parents with the same blood and CMV seronegative are important.

2007-01-01

169

Serum YKL-40 Levels and Chitotriosidase Activity in Patients with Beta-Thalassemia Major  

OpenAIRE

Background. YKL-40 association with human disease has been the object of many years of investigation. ?-thalassemia patients are affected by hepatic siderosis, which determines a fibrotic process and tissue remodelling. Chitotriosidase has been found to be increased in thalassemic patients returning to normal in patients submitted to bone marrow transplantation. YKL-40 is associated with macrophage activation in liver and in other tissues. The aim of the study was to analyse the level of ser...

Maria Musumeci; Vincenzo Caruso; Emilia Medulla; Venerando Torrisi; Roberta Migale; Silvia Angeletti; Salvatore Musumeci

2014-01-01

170

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

OpenAIRE

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 thei...

Hashemi Reza; Habibzadeh Mohammadreza; Bouzari Navid; Pourzahedgilani Nima; Kamgar Mohammad; Bekheirnia Mir Reza; Shamshirsaz Alireza; Shamshirsaz Amirhooshang; Aghakhani Shahriar; Homayoun Hooman; Larijani Bagher

2003-01-01

171

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

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Full Text Available 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 divided the patients into two different groups as younger or older than 10 years. Results: None of the indices showed sensitivity and specificity of 100% in the patients older than 10 years, and in the patients younger than 10 years, only Shine & Lal index showed sensitivity close to 90% and specificity of 100%. The most accurate discriminative index for patients younger than 10 years was Shine & Lal and for those older than 10 years it was RDW index. According to Youden's index, Shine & Lal and RBC count showed the greatest diagnostic value in patients younger than 10 years and RDW and RBC count indices in those older than 10 years. Conclusion: None of the indices was completely sensitive and specific in differentiation between ?-TT and IDA. Mean and median mean cell Hb density (MCHD were very close to normal values in both IDA and ?-TT patients, but in the case of mean density of Hb/liter (MDHL, we found that the mean and median were significantly higher than normal values in ?-TT and lower than normal values in IDA patients. In our study, Youden's index of RBC and Shine & Lal were the highest and most reliable indices in differentiating ?-TT from IDA in the patients younger than 10 years. For patients older than 10 years, the most reliable discrimination indices were RBC and RDW.

Fakher Rahim

2009-09-01

172

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)

173

Hydroxyurea as a first-line treatment of extramedullary hematopoiesis in patients with beta thalassemia: Four case reports.  

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Objective and importance Extramedullary hematopoiesis (EMH) is evidenced by erythropoietic masses, which occurs as a compensatory mechanism to overcome hypoxia during chronic anemia. EMH masses in spinal cord could lead to cord compression and neurological symptoms. Besides transfusion, radiotherapy, and surgery, hydroxyurea (HU) is also a treatment strategy in EMH. Clinical presentation We described four cases of beta thalassemia with EMH who were treated with HU as a monotherapy. Intervention (and technique) HU therapy was done in all patients without any transfusion during therapy. Conclusion HU is a good treatment option for patients with EMH and it could be a substitute for radiotherapy and invasive surgery or regular blood transfusion. PMID:24717020

Karimi, Mehran; Cohan, Nader; Pishdad, Parisa

2015-01-01

174

An intracranial extramedullary hematopoiesis in a 34-year-old man with beta thalassemia: a case report  

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Full Text Available Abstract Introduction Extramedullary hematopoiesis occurs in approximately 15% of cases of thalassemia. Intracranial deposits of extramedullary hematopoiesis are an extremely rare compensatory process in intermediate and severe thalassemia. Case presentation We present an unusual case of an intracranial extramedullary hematopoiesis with a choroid plexus origin in a 34-year-old Caucasian man with beta thalassemia intermedia, who presented with the complaints of chronic headache and rapid progressive visual loss. Conclusion An intracranial extramedullary hematopoiesis, although extremely rare, should be considered as a potential ancillary diagnosis in any thalassemic patient and therefore appropriate studies should be performed to investigate the probable intracranial ectopic marrow before any surgical intervention.

Tabesh Homayoun

2011-12-01

175

Takayasu's arteritis presenting with temporary loss of vision in a 23-year-old woman with beta thalassemia trait: a case report  

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Full Text Available Abstract Introduction The simultaneous presence of Takayasu's arteritis and beta thalassemia trait is a rare combination. To the best of our knowledge, this is the first case report on Takayasu's arteritis and beta thalassemia presenting together. Case presentation This is a case report of a 23-year-old Asian woman of Pakistani descent who presented with a headache, blurred vision and dizziness. Conclusion The correct diagnosis of our patient was based on clinical suspicion, appropriate imaging studies, and deliberation of the differential diagnosis. The management of our patient depended on the correct diagnosis of both the diseases.

Shabbir Fahad A

2011-09-01

176

Spectrum of Beta Globin Gene Mutations in Egyptian Children with ?-Thalassemia  

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Background The molecular defects resulting in a ?-thalassemia phenotype, in the Egyptian population, show a clear heterogenic mutations pattern. PCR-based techniques, including direct DNA sequencing are effective on the molecular detection and characterization of these mutations. The molecular characterization of ?-thalassemia is necessary for carrier screening, genetic counseling, and to offer prenatal diagnosis. The aim of the work was to evaluate the different ?-globin gene mutations in two hundred ?-thalassemic Egyptian children. Subjects and Methods This study was carried out on two hundred ?-thalassemic Egyptian children covering most Egyptian Governorates including 158 (79%) children with thalassemia major (TM) and 42 (21%) children with thalassemia intermedia(TI). All patients were subjected to meticulous history taking, clinical examination, complete blood count, hemoglobin electrophoresis, serum ferritin and direct fluorescent DNA sequencing of the ?-globin gene to detect the frequency of different mutations. Results The most common mutations among patients were IVS I-110(G>A) 48%, IVS I-6(T>C) 40%, IVS I-1(G>A) 24%, IVS I-5(G>C)10%, IVS II-848 (C>A) 9%, IVS II-745(C>G) 8%, IVS II-1(G>A) 7%, codon “Cd”39(C> T) 4%, ?87(C>G) 3% and the rare mutations were: Cd37 (G>A), Cd8 (?AA), Cd29(?G), Cd5 (?CT), Cd6(?A), Cd8/9(+G), Cd 106/107(+G), Cd27(C>T), IVS II-16(G> C), Cd 28 (?C), Cap+1(A>C), ?88(C>A), all of these rare mutations were present in 1%. There was a considerable variation in phenotypic severity among patients resulting from the interaction of different ?? and ?+mutations. Furthermore, no genotype-phenotype association was found both among the cases with thalassemia major and the cases with thalassemia intermedia. Conclusion Direct DNA sequencing provides insights for the frequency of different mutations in patients with ?-thalassemia including rare and/or unknown ones. The most common mutations in Egyptian children with beta thalassemia were IVS I-110(G>A) 48%, IVS I-6(T>C) 40%, IVS I-1(G>A)24%, IVS I-5(G>C)10%, IVS II-848 (C>A) 9%, IVS II-745(C>G) 8%, IVS II-1(G>A) 7%. PMID:25408857

El-Shanshory, MR; Hagag, AA; Shebl, SS; Badria, IM; Abd Elhameed, AH; Abd El-Bar, ES; Al-Tonbary, Y; Mansour, A; Hassab, H; Hamdy, M; Alfy, M; Sherief, L; Sharaf, E

2014-01-01

177

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

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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.

178

Early Predictors of Renal Dysfunction in Egyptian Patients with ?-Thalassemia Major and Intermedia  

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Background Better survival of thalassemia patients allowed previously unrecognized renal complications to emerge. Objectives Assess prevalence and early predictors of renal dysfunction in young ?-thalassemia major (?-TM) and intermedia (?-TI) patients. Subjects 66 ?-TM (group I), 26 ?-TI (group II) Egyptian patients and 40 healthy controls. Methods Clinical assessment and laboratory data including kidney and liver function tests, such as serum ferritin, serum bicarbonate, plasma osmolality and urinary total proteins, microalbuminuria (MAU), N-acetyl-?-D-glucosaminidase (NAG), retinol binding protein (RBP), ?-1 microglobulin, bicarbonate, osmolality, creatinine clearance (CrCl), % fractional excretion of bicarbonate (% FE-HCO3). Results The prevalent renal abnormality was proteinuria (71%), followed by increased urinary level of RBP (69.4%), NAG (58.1%), ?-1 microglobulin (54.8%) and microalbuminuria (29%) and also decreased urinary osmolality (58.1%). CrCl was a better assessment of renal function and significantly lowered in thalassemia patients. Tubular dysfunctions were more significant in splenectomized ?-TM patients who showed more elevation of NAG and ?-1 microglobulin and lower urinary osmolality. NAG, RBP and ?-1 microglobulin were negatively correlated with CrCl and positively correlated with serum ferritin and urinary total protein. Z-score analysis for identifying patients with renal dysfunction proved superiority of urine total protein and RBP. Comparative statistics of different frequencies revealed significant difference between the urinary total protein and both MAU and % FE-HCO3. Conclusion Asymptomatic renal dysfunctions are prevalent in young ?-TM and ?-TI patients that necessitate regular screening. Urinary total protein and RBP may be cost-effective for early detection. PMID:25237470

Tantawy, Azza A.G.; El Bablawy, Nagham; Adly, Amira A.M.; Ebeid, Fatma S.E.

2014-01-01

179

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

180

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

181

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

182

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  

OpenAIRE

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 data...

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

2010-01-01

183

Comparison of red cell distribution width and a red cell discriminant function incorporating volume dispersion for distinguishing iron deficiency from beta thalassemia trait in patients with microcytosis  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: English Abstract in portuguese A função discriminante baseada na distribuição do tamanho dos eritrócitos (RDW) e outra função discriminante incorporando o RDW (MCV² x RDW/Hgb x 100) foram determinadas em um grupo de 30 pacientes com anemia ferropriva, 30 pacientes com beta talassemia menor e 30 indivíduos normais. Os valores médi [...] os do RDW e do (MCV² x RDW/ Hgb x 100) foram significativamente maiores em pacientes com anemia ferropriva quando comparados aos obtidos em pacientes com beta talassemia menor (p Abstract in english The red cell distribution width (RDW), and another red cell discriminant function incorporating RDW (MCV² x RDW/Hgb x 100) were determined in a group of 30 patients with iron deficiency anemia, 30 patients with beta thalassemia trait, and 30 normal subjects. Both RDW and (MCV² x RDW/Hgb x 100) mean [...] values were significantly higher in iron deficiency anemia than in beta thalassemia trait (p

Carmen Silvia Passos, Lima; Aparecida Ribeiro de Carvalho, Reis; Helena Zerlotti Wolf, Grotto; Sara Teresinha Ollala, Saad; Fernando Ferreira, Costa.

1265-12-01

184

Factor V G1691A (Leiden is a major etiological factor in Egyptian Budd-Chiari syndrome patients  

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Full Text Available Objective: Budd-Chiari syndrome is a multifactorial disease in which several prothrombotic disorders may predispose patients to the development of thrombosis at this uncommon location (hepatic veins. The aim of this study was to determine the prevalence and characteristics of inherited thrombophilia in Egyptian Budd-Chiari syndrome patients.Materials and Methods: The study included 47 Budd-Chiari syndrome patients (20 children and 27 adults. Genotyping of Factor V G1691A (Leiden, prothrombin G20210A (PT, and methylenetetrahydrofolate reductase C677T were performed using real-time PCR and fluorescence melting curve detection analysis.Results: Factor V Leiden was observed in 29 patients (61.7%. It is the only factor that caused Budd-Chiari syndrome in 18 of the patients and in 5 of the patients with inferior vena cava involvement. Myeloproliferative disease was noted in 12 (25.5% patients, antiphospholipid syndrome in 5 (10.6%, and Behcet’s disease in 3 (6.4%. Interestingly, 3 of the children with Budd-Chiari syndrome had lipid storage disease.Conclusion: Factor V Leiden was a major etiological factor in Egyptian Budd-Chiari syndrome patients, which may have been related to the high frequency of this mutation in the study region. Factor V Leiden was also a strong thrombophilic factor and the leading cause of inferior vena cava thrombosis in these patients. Lipid storage disease should be included as a risk factor for Budd-Chiari syndrome.

Tawhida Y. Abdel Ghaffar

2011-12-01

185

A phase 3 study of deferasirox (ICL670), a once-daily oral iron chelator, in patients with beta-thalassemia  

OpenAIRE

Deferasirox (ICL670) is a once-daily oral iron chelator developed for the treatment of chronic iron overload from blood transfusions. A comparative phase 3 trial was conducted to demonstrate the efficacy of deferasirox in regularly transfused patients with beta-thalassemia aged 2 years or older. Patients were randomized and received treatment with deferasirox (n = 296) or deferoxamine (n = 290), with dosing of each according to baseline liver iron concentration (LIC). The primary endpoint was...

Piga, Antonio Giulio

2006-01-01

186

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  

OpenAIRE

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 difficult 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 patien...

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

2000-01-01

187

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

188

Associations of red blood corpuscle mean volume and hematocrit with severity of beta-globin gene mutations in beta-thalassemia carriers  

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Full Text Available Background and Aim: Thalassemia, a heterogeneous disease, is one of the most common single-gene diseases worldwide. The aim of this study was to find associations between hematological indices and severity of beta-globin gene mutations in beta-thalassemia carriers. Materials and Methods: In this cross-sectional study, 30 beta-goblin gene mutations (b+ and b? in 1206 unrelated beta-thalassemia carriers were investigated. In addition, their hematological indices, including CBC and electrophoresis results, were determined.  The association between genetic findings and hematological parameters (mean corpuscular volume (MCV and mean hematocrit corpuscular hematocrit (MCH were determined using the SPSS software, the statistical test being the t-test.  Results: The results indicated that b+-thalassemia carriers had higher MCV and MCH means than b?-thalassemia carriers. These findings would certainly have practical implications in public health. Conclusion: The results show a significant correlation between two hematological indices and certain types of mutations in beta-thalassemia carriers.

M A Ehsani

2011-04-01

189

beta-thalassemia intermedia in a Brazilian patient with - 101(C > T) and codon 39 (C > T) mutations  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: English Abstract in portuguese CONTEXTO: Neste trabalho foi estudada a alteração molecular em um paciente brasileiro de 72 anos com talassemia beta homozigótica com evolução clínica intermediária, esplenectomizado e necessitando de transfusões ocasionais. Os dados hematológicos mostravam anemia microcítica e hipocrômica (Hemoglob [...] ina = 7,9 g/dl, Volume Corpuscular Médio = 76fl, Hemoblobina Corpuscular Média = 26 pg) e a eletroforese de hemoglobina revelou Hemoglobina Fetal = 14,2%, Hemoglobina A2 = 6,2% e Hemoglobina A = 79,4%. OBJETIVO: Identificar as duas mutações envolvendo um paciente com sintomas de talassemia beta intermediária. TIPO DE ESTUDO: Investigação molecular das possíveis mutações responsáveis pelo quadro clínico descrito. LOCAL: Centro de Biologia Molecular e Engenharia Genética, Universidade Estadual de Campinas, Campinas, São Paulo, Brasil. PROCEDIMENTOS: Após a extração de DNA do sangue deste paciente, foi feita reação de polimerase em cadeia (polymerase chain reaction, PCR) utilizando cinco pares de primers que amplificaram éxons e a região promotora do gene da globina beta. O produto da amplificação foi seqüenciado e os cromatogramas, analisados por programas de computador (Phred, Phrap e Consed). RESULTADOS: Foram encontradas duas mutações responsáveis pela doença;-101 (C > T) e códon 39 (C > T). CONCLUSÕES: Este caso representa a primeira descrição da mutação 101 (C > T) na população brasileira e está associado a evolução clínica benigna. Abstract in english CONTEXT: We verified molecular alterations in a 72-year-old Brazilian male patient with a clinical course of homozygous beta-thalassemia intermedia, who had undergone splenectomy and was surviving without regular blood transfusions. The blood cell count revealed microcytic and hypochromic anemia (he [...] moglobin = 6.5 g/dl, mean cell volume = 74 fl, mean cell hemoglobin = 24 pg) and hemoglobin electrophoresis showed fetal hemoglobin = 1.3%, hemoglobin A2 = 6.78% and hemoglobin A = 79.4%. OBJECTIVE: To identify mutations in a patient with the symptoms of beta-thalassemia intermedia. DESIGN: Molecular inquiry into the mutations possibly responsible for the clinical picture described. SETTING: The structural molecular biology and genetic engineering center of the Universidade Estadual de Campinas, Campinas, Brazil. PROCEDURES: DNA extraction was performed on the patient's blood samples. The polymerase chain reaction (PCR) was done using five specific primers that amplified exons and the promoter region of the beta globin gene. The samples were sequenced and then analyzed via computer programs. RESULTS: Two mutations that cause the disease were found: -101 (C > T) and codon 39 (C > T). CONCLUSIONS: This case represents the first description of 101 (C > T) mutation in a Brazilian population and it is associated with a benign clinical course.

Sylvia Morais de, Sousa; Letícia, Khater; Luís Antônio, Peroni; Karine, Miranda; Marcelo Jun, Murai; Dulcinéia Martins, Albuquerque; Paulo, Arruda; Sara Terezinha Ollala, Saad; Fernando Ferreira, Costa.

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

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: Portuguese Abstract in portuguese 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 glut [...] âmico, 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. Abstract in english 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 th [...] e 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; Sandra B. R., Picado.

191

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  

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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

192

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

193

Two new beta-thalassemia deletions compromising prenatal diagnosis in an Italian and a Turkish couple seeking prevention.  

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When the molecular background of couples requesting prevention is unclear, family analysis and tools to define rare mutations are essential. We report two novel deletion defects observed in an Italian and in a Turkish couple. The first proband presented with microcytic hypochromic parameters without iron deficiency, a normal HbA(2) and an elevated HbF (10.6%). His father presented with a similar phenotype and his wife was heterozygous for the common Mediterranean codon 39 (HBB:c.118C>T) mutation. Having excluded point mutations and common deletions, Multiplex Ligation-dependent Probe Amplification was performed revealing an unknown Ggamma(Agammadeltabeta)(0)-thalassemia defect spanning from the Agamma gene to downstream of the beta-globin gene provisionally named Leiden 69.5 kb deletion. In the second case, the wife presented with a mild thalassemic picture, normal HbA(2), elevated HbF (18.5%) and a beta/alpha globin chain synthesis ratio of 0.62, without iron deficiency or any known beta-thalassemia defect, while the husband was a simple carrier of the common Mediterranean IVS-I-110 (HBB:c.93-21 G>A) mutation. A new large deletion involving the beta-gene and part of the delta-gene was identified by Multiplex Ligation-dependent Probe Amplification provisionally named "Leiden 7.4 kb". PMID:19734421

Phylipsen, Marion; Amato, Antonio; Cappabianca, Maria Pia; Traeger-Synodinos, Jan; Kanavakis, Emmanuel; Basak, Nazli; Galanello, Renzo; Tuveri, Teresa; Ivaldi, Giovanni; Harteveld, Cornelis L; Giordano, Piero C

2009-09-01

194

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

195

Insulin-like growth factor-1 levels in children with Beta-thalassemia minor  

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Full Text Available Objective: Growth retardation in children with b-thalassemia major is multifactorial. Some etiologies described for this condition are hemochromatosis, disturbed growth hormone (GH / insulin growth factor-1 (IGF-1 axis, undernutrition and hypermetabolism. It has also been proven that growth retardation is present in b-thalassemia major children despite regular transfusion and chelation. Our aim was to evaluate the level of IGF-1 in b-thalassemia minor subjects and compare it with that in healthy children. Material and Methods: Fifty children aged 6 months to 15 years with b-thalassemia minor (32 males, 18 females and 50 age- and sex-matched normal healthy children were selected. Medical history was taken and complete physical examination was done in each case; IGF-1 level was checked in all cases. This study was done in Shiraz, southern Iran, during 2005.Results: IGF-1 levels were significantly lower in b-thalassemia minor children than normal children (P = 0.015. This result demonstrates that some etiologies of growth failure in b-thalassemia major other than those described to date can exist, which may be shared with b-thalassemia minor in feature or may be transformed by genes that are either expressed or not.Conclusion: We conclude that in addition to that observed in b-thalassemia major, IGF-1 level is also decreased in b-thalassemia minor, and these two may have similar etiologies.

Mehran Karimi

2008-09-01

196

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

OpenAIRE

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 ...

Fatemeh Farahmand; Vajiheh Modaresi; Mina Izadyar; Fatemeh Mahjob

2010-01-01

197

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

198

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

199

Inflammation and oxidant-stress in beta-thalassemia patients treated with iron chelators deferasirox (ICL670) or deferoxamine: an ancillary study of the Novartis CICL670A0107 trial  

OpenAIRE

BackgroundWe assessed whether oxidant-stress and inflammation in beta-thalassemia could be controlled by the novel oral iron chelator deferasirox as effectively as by deferoxamine.Design and MethodsForty-nine subjects were enrolled from seven sites and studied at baseline, and after 1, 6, and 12 months of therapy. Malondialdehyde, protein carbonyls, vitamins E and C, total non-transferrin bound iron, transferrin saturation, C-reactive protein, cytokines, serum ferritin concentration and liver...

Walter, P. B.; Macklin, E. A.; Porter, J.; Evans, P.; Kwiatkowski, J. L.; Neufeld, E. J.; Coates, T.; Giardina, P. J.; Vichinsky, E.; Olivieri, N.; Alberti, D.; Holland, J.; Harmatz, P.; Thalassemia Clinical Res Network

2008-01-01

200

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  

OpenAIRE

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 electrophoresis 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: t...

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

2010-01-01

201

Laboratory assessment of iron status and reticulocyte parameters in differential diagnosis of iron deficiency anemia and heterozygous beta-thalassemia Avaliação laboratorial do estado do ferro e parâmetros reticulocitários no diagnóstico diferencial da anemia ferropriva e beta-talassemia heterozigótica  

OpenAIRE

Introduction:The soluble form of transferrin receptor (sTfR) has been pointed as a useful parameter to assess the iron status and erythropoiesis activity. Immature reticulocytes present high concentration of membrane transferrin receptor. We tested the correlation between sTfR and reticulocyte parameters in iron deficiency anemia (IDA) and heterozygous beta-thalassemia (hetero beta-thal) patients. Laboratory parameters related to iron status and reticulocytes were studied in order to establis...

Lima, Gise?lia A. F. M.; Grotto, Helena Z. W.

2002-01-01

202

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

203

Fatal fat embolism syndrome in a child with undiagnosed hemoglobin S/beta+ thalassemia: a complication of acute parvovirus B19 infection.  

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Anemia, mental status changes, and fatal respiratory failure complicated a febrile illness in a previously healthy 14-year-old black female. At autopsy, widespread fat emboli and bone marrow necrosis were found. Hemoglobin electrophoresis on an antemortem, pretransfusion specimen revealed hemoglobin S/beta+ thalassemia. Acute parvovirus B19 (PV B19) infection was suspected. Postmortem serum and a variety of paraffin-embedded tissues were assayed for PV B19 DNA using the polymerase chain reaction (PCR). The expected PCR product was identified in the serum specimen and in paraffin-embedded sections of bone marrow, kidney, spleen, parathyroid, thyroid, adrenal, and gastrointestinal tract: lung, liver, ovary, fallopian tube, uterus, brain, heart, and pancreas were negative. PV B19 infection is highly contagious and may be rapidly fatal in children with hemoglobinopathies by several mechanisms, including fat embolism. Therefore, there exists the risk of multiple deaths within a family. The acute infection may be easily and expeditiously diagnosed using serum or a variety of paraffin-embedded tissues. PMID:8963632

Kolquist, K A; Vnencak-Jones, C L; Swift, L; Page, D L; Johnson, J E; Denison, M R

1996-01-01

204

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

205

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

206

Lasting Hb F reactivation and Hb A2 reduction induced by the treatment of Hodgkin's disease in a woman heterozygous for beta-thalassemia and the Swiss type of the heterocellular hereditary persistence of Hb F.  

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A remarkable augmentation of Hb F and a reduction of Hb A2 were observed in a Sicilian woman during and after a course of treatment for Hodgkin's disease. An inverse correlation between the proportion of Hb F and Hb A2 was found over an 8-year period, as well as in populations of red blood cells fractionated by density gradient. She exhibited two genetic defects, the Swiss type of heterocellular hereditary persistence of fetal hemoglobin and a beta-thalassemia trait, which were confirmed by t...

Guerrasio, Angelo

1982-01-01

207

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

OpenAIRE

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 1...

Kosaryan, M.; MR. Mahdavi; Aliasgharian, A.; Mousavi, M.; Roshan, P.

2013-01-01

208

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.)

209

Egyptian "Star Clocks"  

Science.gov (United States)

Diagonal, transit, and Ramesside star clocks are tables of astronomical information occasionally found in ancient Egyptian temples, tombs, and papyri. The tables represent the motions of selected stars (decans and hour stars) throughout the Egyptian civil year. Analysis of star clocks leads to greater understanding of ancient Egyptian constellations, ritual astronomical activities, observational practices, and pharaonic chronology.

Symons, Sarah

210

Egyptian Caste System  

Science.gov (United States)

Understand the culture, character, and societal characteristics of different classes in the ancient Egyptian caste system Understand the culture, character, and societal characteristics of different classes in the ancient Egyptian caste system Go to these sites to learn about the Egyptian Caste System ...

Issen, Ms.

2009-09-17

211

Cerebrovascular events in sickle cell-beta thalassemia treated with hydroxyurea: a single center prospective survey in adult Italians.  

Science.gov (United States)

Stroke is a common cause of morbidity and mortality in sickle cell disease (SCD) and silent cerebral infarction is the most common form of neurologic injury. The frequency and risk factors for new silent cerebral infarction are incompletely understood. Moreover, no recommended treatment has been established. Although hydroxyurea (HU) is recommended for SCD, concerns remain regarding its role in the prevention of cerebrovascular events, including silent cerebral infarction. A single center population of 104 Italian patients with HbS-ß thalassemia treated with HU has been followed for a mean of 11 years. Clinical evaluation and brain imaging by Magnetic Resonance Imaging were done before and during HU treatment. During follow-up, the number of sickle cell crises (86%, 7.8?±?6.9 vs. 1.2?±?0.5 per year, P?major benefit of HU is the increase in HbF; the association of high HbF and reduced cerebrovascular disease has been weak. New treatment strategies should be developed for the prevention of sickle cerebrovascular disease. PMID:23828131

Rigano, Paolo; Pecoraro, Alice; Calvaruso, Giuseppina; Steinberg, Martin H; Iannello, Sonia; Maggio, Aurelio

2013-11-01

212

On improvement in ejection fraction with iron chelation in thalassemia major and the risk of future heart failure  

OpenAIRE

Abstract Background Trials of iron chelator regimens have increased the treatment options for cardiac siderosis in beta-thalassemia major (TM) patients. Treatment effects with improved left ventricular (LV) ejection fraction (EF) have been observed in patients without overt heart failure, but it is unclear whether these changes are clinically meaningful. Methods This retrospective study of a UK database of TM patients modelled the change in EF between serial sca...

Jp, Carpenter; Dj, Pennell; Roughton M; Zi, Cabantchik

2011-01-01

213

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

Energy Technology Data Exchange (ETDEWEB)

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

214

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

Science.gov (United States)

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

215

Genotypes and haplotypes in the 3' untranslated region of the HLA-G gene and their association with clinical outcome of hematopoietic stem cell transplantation for beta-thalassemia.  

Science.gov (United States)

Polymorphisms in the 3' untranslated region (3'UTR) of HLA-G, an important player in immunological tolerance, could be involved in post-transcriptional expression control, and their association with different clinical immune-related conditions including autoimmunity and transplantation is of mounting interest. Most studies have focused on a 14 base pair (bp) insertion/deletion (ins/del), while additional single-nucleotide polymorphisms (SNPs) in the HLA-G 3'UTR have been described but not extensively investigated for their clinical relevance. Here we have comparatively studied the association between 3'UTR haplotypes of HLA-G, or the 14 bp ins/del, with clinical outcome of HLA-identical sibling hematopoietic stem cell transplantation (HSCT) in 147 Middle Eastern beta-thalassemia patients. Sequence based typing of 3'UTR HLA-G polymorphisms in the patients and in 102 healthy Italian blood donors showed strong linkage disequilibrium between the 14 bp ins/del and five 3'UTR SNPs, which together could be arranged into eight distinct haplotypes based on expectation-maximization studies, with four predominant haplotypes (UTRs1-4). After HSCT, we found a moderate though not significant association between the presence of UTR-2 in double dose and protection from acute graft versus host disease (hazard ratio (HR) 0.45, 95% confidence intervals (CI): 0.14-1.45; P = 0.18), an effect that was also seen when the corresponding 14 bp ins/ins genotype was considered alone (HR 0.42, 95% CI: 0.16-1.06; P = 0.07). No association was found with rejection or survival. Taken together, our data show that there is no apparent added value of considering entire 3'UTR HLA-G haplotypes for risk prediction after allogeneic HSCT for beta-thalassemia. PMID:22489942

Sizzano, F; Testi, M; Zito, L; Crocchiolo, R; Troiano, M; Mazzi, B; Turchiano, G; Torchio, M; Pultrone, C; Gregori, S; Chiesa, R; Gaziev, J; Sodani, P; Marktel, S; Amoroso, A; Roncarolo, M G; Lucarelli, G; Ciceri, F; Andreani, M; Fleischhauer, K

2012-05-01

216

Egyptian Clover (Trifolium alexandrinum L. Breeding in Egypt: A Review  

Directory of Open Access Journals (Sweden)

Full Text Available Berseem or Egyptian clover is a winter annual legume of major importance to Egyptian agriculture as a principle source of forage and for conserving soil fertility under intensive cropping. In this review, the published work by authors on breeding Egyptian clover (Trifolium alexandrinum L. for productivity in Egypt has been summarized since 1980. The variability of the genotypes, genotypic and phenotypic correlation, Path coefficient, the breeding strategies, pollination and seed setting for Egyptian clover are presented. The genetics and productivity of Egyptian clover are recorded. The objectives of this review are enrichment the background for the investigators working in Egyptian clover breeding. Finally the achievements and the needed cooperation on the national level are also outlined.

Bahy R. Bakheit

2013-01-01

217

Assessment of Hepatic and Pancreatic Iron Overload in Pediatric Beta-Thalassemic Major Patients by T2* Weighted Gradient Echo Magnetic Resonance Imaging  

OpenAIRE

Background. MRI has emerged for the noninvasive assessment of iron overload in various tissues. The aim of this paper is to evaluate hepatic and pancreatic iron overload by T2? weighted gradient echo MRI in young beta-thalassemia major patients and to correlate it with glucose disturbance and postsplenectomy status. Subjects and Methods. 50 thalassemic patients, in addition to 15 healthy controls. All patients underwent clinical assessment and laboratory investigations. Out of 50 thalassemi...

Youssef, Doaa Mohammed; Fawzy Mohammad, Faten; Ahmed Fathy, Ayman; Aly Abdelbasset, Maha

2013-01-01

218

Ancient Egyptian surgical heritage.  

Science.gov (United States)

Egyptian medicine influenced the medicine of neighboring cultures, including the culture of ancient Greece. From Greece, its influence spread onward, thereby affecting Western civilization significantly. The oldest extant Egyptian medical texts are six papyri: The Edwin Smith Surgical Papyrus and the Ebers Medical Papyrus are famous. PMID:21208098

Saber, Aly

2010-12-01

219

Egyptian Antiquity Information  

Science.gov (United States)

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.

220

Denser Egyptian Fractions  

OpenAIRE

An Egyptian fraction is a sum of distinct unit fractions (reciprocals of positive integers). We show that every rational number has Egyptian fraction representations where the number of terms is of the same order of magnitude as the largest denominator, improving a result from an earlier paper to best-possible form. We also settle, in best-possible form, the related problem of how small M_t(r) can be such that there is an Egyptian fraction representation of r with exactly t ...

Martin, Greg

1998-01-01

221

Dense Egyptian Fractions  

OpenAIRE

Every positive rational number has representations as Egyptian fractions (sums of reciprocals of distinct positive integers) with arbitrarily many terms and with arbitrarily large denominators. However, such representations normally use a very sparse subset of the positive integers up to the largest demoninator. We show that for every positive rational there exist Egyptian fractions whose largest denominator is at most N and whose denominators form a positive proportion of t...

Martin, Greg

1998-01-01

222

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.

223

Congestive heart failure and treatment in thalassemia major.  

Science.gov (United States)

The homozygous beta-thalassemias are a group of genetically inherited hemoglobin (Hb) disorders characterized by dyserythropoietic anemia. According to the degree of anemia, two main forms, sharing a common basic molecular mechanism, are distinguished: thalassemia major (TM) and thalassemia intermedia (TI). The severity of the clinical phenotype differentiates the two forms. Thalassemia major usually presents as a severe anemia requiring life-long transfusion therapy for survival. The dramatic improvement in life expectancy of beta-thalassemia (thal) patients achieved during the past few decades by virtue of therapeutic advances, has motivated investigators' interest in a better understanding of the clinical consequences of this genetic defect. Heart complications still represent the leading cause of mortality from the disease. The mechanisms of cardiac injury along with its treatment and prevention have attracted the main research efforts in this field. In this review, we present existing knowledge and our personal experience of 30 years of follow-up of over 1,000 thalassemic patients, regarding the basis of the cardiac injury, the clinical findings and the global strategy of the therapeutic intervention in TM patients who develop congestive heart failure (CHF). PMID:18274984

Aessopos, Athanassios; Kati, Maria; Tsironi, Maria

2008-01-01

224

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

225

JIT implementation in Egyptian manufacturing firms: some empirical evidence  

OpenAIRE

Abstract: Purpose – This study aims to delineate the major human modifications to be undertaken prior to just-in-time (JIT) implementation in Egyptian manufacturing firms; to discern the benefits obtained from JIT implementation; to identify the problems that Egyptian manufacturing companies typically encounter in implementing JIT philosophy; and to explore the relationship between human modification efforts to be undertaken prior to JIT implementation and JIT success. Design/methodolo...

Salaheldin, S. I.

2005-01-01

226

Ancient Egyptian herbal wines.  

Science.gov (United States)

Chemical analyses of ancient organics absorbed into pottery jars from the beginning of advanced ancient Egyptian culture, ca. 3150 B.C., and continuing for millennia have revealed that a range of natural products--specifically, herbs and tree resins--were dispensed by grape wine. These findings provide chemical evidence for ancient Egyptian organic medicinal remedies, previously only ambiguously documented in medical papyri dating back to ca. 1850 B.C. They illustrate how humans around the world, probably for millions of years, have exploited their natural environments for effective plant remedies, whose active compounds have recently begun to be isolated by modern analytical techniques. PMID:19365069

McGovern, Patrick E; Mirzoian, Armen; Hall, Gretchen R

2009-05-01

227

Print like an Egyptian.  

Science.gov (United States)

Describes a relief printmaking unit for sixth graders with the objective of decorating the inside of a pyramid. Ancient Egyptian imagery was used to help students become familiar with the style. Students designed and printed linoleum prints in different colors. They then critiqued their work and made their selection for the pyramid. (KM)

Weisensee, Marilyn

1990-01-01

228

Sit Like an Egyptian  

Science.gov (United States)

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

229

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

230

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 SciELO Brazil | Language: Portuguese Abstract in portuguese [...] 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

231

Fascinating Egyptian Mummies  

Science.gov (United States)

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.

232

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

233

Senenmut: An Ancient Egyptian Astronomer  

CERN Document Server

The celestial phenomenon have always been a source of wonder and interest to people, even as long ago as the ancient Egyptians. While the ancient Egyptians did not know all the things about astronomy that we do now, they had a good understanding of the some celestial phenomenon. The achievements in astronomy of ancient Egyptians are relatively well known, but we know very little about the people who made these achievements. The goal of this paper is to bring some light on the life of Senenmut, the chief architect and astronomer during the reign of Queen Hatshepsut.

Novakovic, Bojan

2008-01-01

234

Senenmut: An Ancient Egyptian Astronomer  

Science.gov (United States)

The celestial phenomena have always been a source of wonder and interest to people, even as long ago as the ancient Egyptians. While the ancient Egyptians did not know all the things about astronomy that we do now, they had a good understanding of some celestial phenomena. The achievements in astronomy of ancient Egyptians are relatively well known, but we know very little about the people who made these achievements. The goal of this paper is to bring some light on the life of Senenmut, the chief architect and astronomer during the reign of Queen Hatshepsut.

Novakovic, B.

2008-10-01

235

Laboratory assessment of iron status and reticulocyte parameters in differential diagnosis of iron deficiency anemia and heterozygous beta-thalassemia Avaliação laboratorial do estado do ferro e parâmetros reticulocitários no diagnóstico diferencial da anemia ferropriva e beta-talassemia heterozigótica  

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Full Text Available Introduction:The soluble form of transferrin receptor (sTfR has been pointed as a useful parameter to assess the iron status and erythropoiesis activity. Immature reticulocytes present high concentration of membrane transferrin receptor. We tested the correlation between sTfR and reticulocyte parameters in iron deficiency anemia (IDA and heterozygous beta-thalassemia (hetero beta-thal patients. Laboratory parameters related to iron status and reticulocytes were studied in order to establish their clinical value to distinguish both anemias. Material and Methods: Reticulocyte measurements were obtained using a semi-automated analyzer and serum concentration of sTfR was determined by an immunoenzymatic technique. Forty-nine IDA and 43 hetero beta-thal patients were studied. Results: Reticulocyte count and sTfR values were significantly higher in IDA than in hetero beta-thal group, but the best parameter to distinguish both anemias was sTfR index, obtained by the ratio sTfR/ferritin level. Transport compartment was better evaluated by transferrin dosage than by transferrin iron binding capacity (TIBC determination. The association of serum iron with transferrin measurements (transferrin index improved the accuracy of the transferrin test. Discussion: The correlation between highly immature reticulocytes and sTfR level was observed only in IDA group, suggesting that cellular iron deprivation is the main responsible factor for up regulation of the sTfR synthesis in immature red blood cells. High sTfR values in hetero beta-thal patients reflect a degree of ineffective erythropoiesis in this hemoglobinopathy. Conclusion: We concluded that sTfR, ferritin and transferrin measurements are useful and precise parameters to discriminate IDA from hetero beta-thal patients.Introdução: A forma solúvel do receptor da transferrina (sTfR tem sido indicada como um parâmetro útil na avaliação do estado do ferro e da atividade eritropoiética. Reticulócitos imaturos apresentam alta concentração dos receptores de transferrina na sua membrana. Estudamos a correlação entre sTfR e parâmetros reticulocitários em pacientes com anemia ferropriva (AF e com beta-talassemia heterozigótica (beta-tal hetero. Os parâmetros laboratoriais relacionados ao estado do ferro e reticulócitos foram estudados a fim de se estabelecer a utilidade clínica dos mesmos na distinção entre os dois tipos de anemia. Material e métodos: As medidas reticulocitárias foram obtidas usando-se um analisador hematológico semi-automático, e as concentrações de sTfR foram determinadas por técnica imunoenzimática. Foram estudados 49 pacientes com AF e 43 com beta-tal hetero. Resultados: As contagens de reticulócitos e os valores de sTfR foram significativamente mais elevados na AF do que na beta-tal hetero, mas o melhor parâmetro para diferenciar as duas anemias foi o índice de sTfR, obtido pela razão sTfR/ferritina. O compartimento de transporte foi mais bem avaliado pela dosagem de transferrina do que pela capacidade de ligação do ferro à transferrina (TIBC. A associação do ferro sérico à medida de transferrina (índice de transferrina melhorou a acurácia do teste de transferrina. Discussão: A correlação entre reticulócitos imaturos e nível de sTfR foi observada apenas no grupo com AF, sugerindo que a falta de ferro intracelular seja o principal fator responsável pelo estímulo à síntese de sTfR nas células sangüíneas imaturas. Os valores elevados de sTfR nos pacientes com beta-tal hetero refletem um certo grau de eritropoiese ineficaz nessa hemoglobinopatia. Conclusão: Concluímos que as medidas de sTfR, ferritina e transferrina são parâmetros úteis e precisos para diferenciar AF de beta-tal hetero.

Gisélia A.F.M. de Lima

2002-01-01

236

Laboratory assessment of iron status and reticulocyte parameters in differential diagnosis of iron deficiency anemia and heterozygous beta-thalassemia / Avaliação laboratorial do estado do ferro e parâmetros reticulocitários no diagnóstico diferencial da anemia ferropriva e beta-talassemia heterozigótica  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: English Abstract in portuguese Introdução: A forma solúvel do receptor da transferrina (sTfR) tem sido indicada como um parâmetro útil na avaliação do estado do ferro e da atividade eritropoiética. Reticulócitos imaturos apresentam alta concentração dos receptores de transferrina na sua membrana. Estudamos a correlação entre sTfR [...] e parâmetros reticulocitários em pacientes com anemia ferropriva (AF) e com beta-talassemia heterozigótica (beta-tal hetero). Os parâmetros laboratoriais relacionados ao estado do ferro e reticulócitos foram estudados a fim de se estabelecer a utilidade clínica dos mesmos na distinção entre os dois tipos de anemia. Material e métodos: As medidas reticulocitárias foram obtidas usando-se um analisador hematológico semi-automático, e as concentrações de sTfR foram determinadas por técnica imunoenzimática. Foram estudados 49 pacientes com AF e 43 com beta-tal hetero. Resultados: As contagens de reticulócitos e os valores de sTfR foram significativamente mais elevados na AF do que na beta-tal hetero, mas o melhor parâmetro para diferenciar as duas anemias foi o índice de sTfR, obtido pela razão sTfR/ferritina. O compartimento de transporte foi mais bem avaliado pela dosagem de transferrina do que pela capacidade de ligação do ferro à transferrina (TIBC). A associação do ferro sérico à medida de transferrina (índice de transferrina) melhorou a acurácia do teste de transferrina. Discussão: A correlação entre reticulócitos imaturos e nível de sTfR foi observada apenas no grupo com AF, sugerindo que a falta de ferro intracelular seja o principal fator responsável pelo estímulo à síntese de sTfR nas células sangüíneas imaturas. Os valores elevados de sTfR nos pacientes com beta-tal hetero refletem um certo grau de eritropoiese ineficaz nessa hemoglobinopatia. Conclusão: Concluímos que as medidas de sTfR, ferritina e transferrina são parâmetros úteis e precisos para diferenciar AF de beta-tal hetero. Abstract in english Introduction:The soluble form of transferrin receptor (sTfR) has been pointed as a useful parameter to assess the iron status and erythropoiesis activity. Immature reticulocytes present high concentration of membrane transferrin receptor. We tested the correlation between sTfR and reticulocyte param [...] eters in iron deficiency anemia (IDA) and heterozygous beta-thalassemia (hetero beta-thal) patients. Laboratory parameters related to iron status and reticulocytes were studied in order to establish their clinical value to distinguish both anemias. Material and Methods: Reticulocyte measurements were obtained using a semi-automated analyzer and serum concentration of sTfR was determined by an immunoenzymatic technique. Forty-nine IDA and 43 hetero beta-thal patients were studied. Results: Reticulocyte count and sTfR values were significantly higher in IDA than in hetero beta-thal group, but the best parameter to distinguish both anemias was sTfR index, obtained by the ratio sTfR/ferritin level. Transport compartment was better evaluated by transferrin dosage than by transferrin iron binding capacity (TIBC) determination. The association of serum iron with transferrin measurements (transferrin index) improved the accuracy of the transferrin test. Discussion: The correlation between highly immature reticulocytes and sTfR level was observed only in IDA group, suggesting that cellular iron deprivation is the main responsible factor for up regulation of the sTfR synthesis in immature red blood cells. High sTfR values in hetero beta-thal patients reflect a degree of ineffective erythropoiesis in this hemoglobinopathy. Conclusion: We concluded that sTfR, ferritin and transferrin measurements are useful and precise parameters to discriminate IDA from hetero beta-thal patients.

Gisélia A.F.M. de, Lima; Helena Z.W., Grotto.

237

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 ...

Lai, Ms.

2007-02-08

238

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  

OpenAIRE

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...

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

2010-01-01

239

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

240

[Ancient Egyptian Odontology].  

Science.gov (United States)

In ancient Egypt during the reign of Pharaoh Djoser, circa 2650 BC, the Step Pyramid was constructed by Imhotep. He was later worshiped as the God of Medicine. One of his contemporaries was the powerful writer Hesy who is reproduced on a panel showing a rebus of a swallow, a tusk and an arrow. He is therefore looked upon as being the first depicted odontologist. The art of writing begun in Egypt in about 3100 BC and the medical texts we know from different papyri were copied with hieratic signs around 1900-1100 BC. One of the most famous is the Papyrus Ebers. It was purchased by professor Ebers on a research travel to Luxor in 1873. Two years later a beautiful facsimile in color was published and the best translation came in 1958 in German. The text includes 870 remedies and some of them are related to teeth and oral troubles like pain in the mouth, gingivitis, periodontitis and cavities in the teeth. The most common oral pain was probably pulpitis caused by extreme attrition due to the high consumption of bread contaminated with soil and/or quern minerals. Another text is the Papyrus Edwin Smith with four surgical cases of dental interest. The "toothworms" that were presumed to bring about decayed teeth have not been identified in the medical texts. It was not until 1889 W.D. Miller presented a scientific explanation that cavities were caused by bacteria. In spite of extensive research only a few evidence of prosthetic and invasive treatments have been found and these dental artifacts have probably been made post mortem. Some of the 150 identified doctors were associated with treatments of disorders of the mouth. The stele of Seneb from Sa'is during the 26th dynasty of Psamtik, 664-525 BC, shows a young man who probably was a dental healer well known to Pharaoh and his court. Clement of Alexandria mentions circa 200 AD that the written knowledge of the old Egyptians was gathered in 42 collections of papyri. Number 37-42 contained the medical writings. The household remedies in ancient Egypt were unique and future research will most likely give us new answers about pathology and health care of that time and a better understanding of old medical concepts. PMID:11625678

Berghult, B

1999-01-01

241

Effect of Hypertransfusion on Extramedullary Hematopoietic Compression Mass in Thalassemia Major: A Case Report  

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Full Text Available Hereby we report a patient with thalassemia major having extradural cord compression at T3-T9 levels due to a mass of extramedullary hematopoiesis (EMH tissue, whose treatment was successful with hypertransfusion therapy alone. The patient was a 23-year-old man who had not received regular blood transfusion since two years before admission. He suffered from paraparesis with a history of progressive lower limb weakness for 2 months. MRI of the spinal cord demonstrated thoracic extramedullary hematopoietic mass causing spinal cord compression. The patient demonstrated a significant response to hypertransfusion and improvement in the neurologic status started a few days after treatment. Almost complete resolution of the mass was seen in spinal MRI one week after hypertransfusion. Hypertransfusion seems to be a useful method for treatment of spinal cord compression due to a hematopoietic mass. It may be used as the first line therapy.Keywords: Beta-Thalassemia,Hypertransfusion,Spinal Cord Compression

Mohammadreza Emamhadi

2012-01-01

242

Evaluation of Glucose Metabolism, Thyroid Function, Growth and Development Pattern and Calcium Status in Patients with Thalassemia Major  

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Full Text Available Thalassemia major is a genetic disorder. Blood transfusion is critical for survival in these patients. Over the course of the past two and three decade`s hypertransfusion therapy in these patients has increased significant improvement in life expectancy and quality of life. Unfortunately, this type of therapy increased the frequency of complication due to iron overloud. In the past endocrine abnormalities were very common in beta-thalassemia patients but it is more common now. The aim of this study was evaluation of prevalence of endocrine disturbances in patients with thalassemia major greater than 10 years old. Fifty six patients with thalassemia major greater than 10 years enrolled. Physicians collected demographic data and history of therapies as well as menstrual history in female. Patients have been examined to determine their pubertal status and SDS of height for evaluation of short stature. For evaluation of glucose tolerance, fasting blood glucose and oral glucose tolerance test were performed. Serum level of calcium, phosphorous, thyroid stimulating hormone, free thyroxin, luteinizing hormone and follicular stimulating hormone, estradiol in girls and testosterone in boys were measured. Fifty six patients with thalassemia major 10-27 years old were evaluated. In this study prevalence of diabetes mellitus, impaired fasting glucose and impaired glucose tolerance test were 8.9, 28.6 and 7.1%, respectively. Short stature (SDS= -2 was seen in 70 of boys and 73% of girls. Hypocalcaemia and primary overt hypothyroidism were present in 41 and 16%, respectively. 14.3% of our patients have not any endocrine abnormalities. Despite recent therapy with Desferal in the management of beta-thalassemia major, the risk of secondary endocrine dysfunction remains high. Hypogonadism is one of the most frequent endocrine complications. Endocrine evaluation in patients with thalassemia major must be carried out regularly especially in those patients over the age of 10 years in tabriz.

2008-01-01

243

The Marskhod Egyptian Drill Project  

Science.gov (United States)

We describe a possible participation of Egypt in a future Mars rover Mission. It was suggested that Egypt participate through involvement in the design, building and testing of a drill to obtain sub-surface samples. The Space Research Institute of the Russian Academy of Sciences (IKI), formally invited the Egyptian Ministry of Scientific Research to study the concept for potential use on the Russian Mars 2001 Mission. As one of the objectives of the Marskhod mission was the analysis of sub-surface samples, a drilling mechanism in the payload would be essential. The Egyptian expertise in drill development is associated with the archaeological exploration of the Pyramids. A sophisticated drilling system perforated limestone to a depth of 2 m without the use of lubricants or cooling fluids that might have contaminated the Pit's environment. This experience could have been applied to a drill development Mars 2001 mission, which was unfortunately canceled due to economic problems.

Shaltout, M. A. M.

244

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

245

An Egyptian Geographer in Ouessant.  

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Full Text Available Within less than twenty-four hours, I have moved from Cairo, the capital city of Egypt, with its fifteen-million population, to the Ouessant island, with its less-than-a-thousand population. At the last moment before catching the boat sailing from Brest to Ouessant, I picked up a very impressive touristic map. On board, with a coffee and a cappuccino, I made contact with some French passengers who were coming from Marseille to have a holiday in that remote island. For a naïve Egyptian ...

Atef Abdel-Hamid

2010-07-01

246

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

247

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

Energy Technology Data Exchange (ETDEWEB)

[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

248

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

249

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

250

Vascular Endothelial Growth Factor in Children with Thalassemia Major  

Science.gov (United States)

Background The ?-Thalassemia syndromes are the most common hereditary chronic hemolytic anemia due to impaired globin chain synthesis. Vascular endothelial growth factor (VEGF) plays several roles in angiogenesis which is a crucial process in the pathogenesis of several inflammatory, autoimmune and malignant diseases. Endothelial damage and inflammation make a significant contribution to the pathophysiology of ?-thalassemia. Purpose : The aim of the study was to assess serum VEGF level in children with beta-thalassemia major as a marker of angiogenesis. Methods A total of 50 children entered the study, 40 patients with thalassemia major and 10 healthy controls. We used enzyme-linked immunosorbent assay for quantitative evaluation of VEGF. Results VEGF level was significantly higher in patients with ?-thalassemia major than healthy controls (p=0.001). VEGF level was also higher in splenectomised thalassemic patients than non splenectomised ones (p=0.001). There were a positive correlation between VEGF and chelation starting age (p=0.008), and a negative correlation between VEGF and frequency of blood transfusion (p=0.002). Conclusion Thalassemia patients, especially splenectomized, have elevated serum levels of VEGF. Early chelation and regular blood transfusion help to decrease serum VEGF and the risk of angiogenesis. PMID:23795282

Fahmey, Sameh S.; Naguib, Hassan F.; Abdelshafy, Sanna S.; Alashry, Rasha E.

2013-01-01

251

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)

252

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

253

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

254

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

255

Islam in Egyptian Education: Grades K-12  

Science.gov (United States)

This article explores the important role that the religion of Islam plays in the education of Egyptian children. The scrutiny under which the Islamic world finds itself in the after-math of September 11, 2001 has resulted in calls for educational reform, not only from the outside world, but also from the Muslim world itself. The author has a…

Neill, Charlotte M.

2006-01-01

256

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

257

Balearic archipelago: three islands, three beta-thalassemia population patterns.  

Science.gov (United States)

The mutation spectrum of 175 ?-thalassemia (?-thal) carriers, identified in pilot carrier screening on 22,713 individuals from Balearic Islands (Spain), is reported. The ?(0) CD39 (C>T) mutation is the most frequent (61.1%), followed by ?(+) IVS-I-110 (G>A) (12.0%), ?(+) IVS-I-6 (T>C) and ?(0) IVS-1-1 (G>A) (3.4% both) and eight other rare mutations (2.9-0.6%); with a distinct prevalence and distribution between islands. Minorca shows the highest prevalence in Iberian populations, with a single mutation, CD39 (C>T), present in most ?-thal carriers. Ibiza is the only Western Mediterranean population where the most frequent ?-thal mutation is IVS-I-110 (G>A). These results can be explained by a combination of historical-demographic characteristics together with evolutionary forces such as founder effect, genetic drift and probably selection by malaria. Knowledge of the mutational spectrum in the Balearic Islands will enable to optimize mutation detection strategy for genetic diagnosis of ?-thal in these islands. PMID:22376243

López-Escribano, H; Parera, M M; Guix, P; Serra, J M; Gutierrez, A; Balsells, D; Oliva-Berini, E; Castro, J A; Ramon, M M; Picornell, A

2013-02-01

258

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

259

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

260

Coagulopathy in Beta-Thalassemia: Current Understanding and Future Perspectives  

OpenAIRE

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...

Alessia Marcon; Musallam, Khaled M.; Domenica Cappellini, M.; Ali Taher

2009-01-01

261

The Readiness of Faculty Members to Develop and Implement E-Learning: The Case of an Egyptian University  

Science.gov (United States)

Adopting e-learning represents one of the major problems in faculty development plans at Egyptian universities. In this study, a survey was developed, validated, and carried out to examine the readiness of academic staff at South Valley University in Egypt to develop and implement e-learning in their teaching. The survey was also used to determine…

Sadik, Alaa

2007-01-01

262

Early Results of Sarcomeric Gene Screening from the Egyptian National BA-HCM Program  

OpenAIRE

The present study comprised sarcomeric genotyping of the three most commonly involved sarcomeric genes: MYBPC3, MYH7, and TNNT2 in 192 unrelated Egyptian hypertrophic cardiomyopathy (HCM) index patients. Mutations were detected in 40 % of cases. Presence of positive family history was significantly (p?=?0.002) associated with a higher genetic positive yield (49/78, 62.8 %). The majority of the detected mutations in the three sarcomeric genes were novel (40/62, 65 %) and mostly private ...

Kassem, Heba Sh; Azer, Remon S.; Ayad, Maha S.; Moharem-elgamal, Sarah; Magdy, Gehan; Elguindy, Ahmed; Cecchi, Franco; Olivotto, Iacopo; Yacoub, Magdi H.

2013-01-01

263

Resting ecology and behaviour of Egyptian mongooses (Herpestes ichneumon) in southwestern Spain  

OpenAIRE

Nocturnal and siesta resting ecology and behaviour of 24 Egyptian mongooses were studied. Mongooses mainly used underground dens (rabbit warrens and badger setts) and thickets in both periods. Tree hollows were occasionally used. Twenty-one individuals used 338 different nocturnal resting sites on 672 occasions. Mean number of resting sites per individual was 16.1, though resting sites used by each individual increased when sample size did. Mean re-use rate was 1.99 nights/site. The majority ...

Palomares, Francisco; Delibes, M.

1993-01-01

264

Association of factor V Leiden mutation with deep vein thrombosis among Egyptian cases  

OpenAIRE

Background: Deep vein thrombosis (DVT) is a blood clot in a major vein, usually in the legs and/or pelvis. If part of the thrombus breaks off, it becomes an embolism, which can travel through the heart and block the arteries to the lungs. Factor V Leiden (FVL) is a common genetic risk factor for hereditary hypercoagulability disorder in several populations. The present study investigates the association of FVL mutation with DVT among Egyptian cases. Patients & methods: The study included 44 c...

Sherif H Abdeen, Rizk El-baz

2010-01-01

265

Karyotype Analysis and Systematic Relationships in the Egyptian Astragalus L. (Fabaceae)  

OpenAIRE

In this study the karyotype criteria of 35 taxa representing 24 species of Egyptian Astragalus have been analyzed and their impact on the systematic delimitation of the studied species is discussed. Chromosome numbers, based on x = 8 have been found in the majority of Astragalus species in Egypt. A diploid number (2n = 16) was recorded in 22 taxa representing 17 species and polyploid numbers are recorded in six taxa representing three species. Numbers based on x = 7 were recorde...

Abdelfattah Badr; Sharawy, Sherif M.

2007-01-01

266

Evaluation of genetic bases and diversity of Egyptian wheat cultivars released during the last 50 years using coefficient of parentage  

Directory of Open Access Journals (Sweden)

Full Text Available Discerning the genetic diversity of any crop species provides insight into the strength of an applied breeding program and directs future breeding strategies aimed at long-term genetic gain and minimized genetic vulnerability. The number and abundance of ancestral parents present in the pedigree of crop cultivars can provide an average estimation of the depth of the genetic base of the overall crop improvement program. The objectives of this study were to estimate (1 the genetic similarity among 33 Egyptian wheat (Triticum aestivum L. cultivars and different eras of release (1947-2004 and productivity groups based on COP values, and (2 the relative genetic contribution and abundance of ancestral parents from different geographical origins to the total gene pool of Egyptian wheat cultivars. Broad genetic diversity was observed among 33 Egyptian cultivars with average COP value of 0.11 and large numbers of ancestral parents (155 landraces traced to 31 countries. The genetic base ranged from very low in pre 1960’s cultivars such as ‘Giza 139’ (with only 3 landraces in the background to very high in modern cultivars such as ‘Gemmeiza-7’ (with 73 landraces in the background. ‘Hindi-62’, ‘Red Fife’, ‘Hard Red Calcutta’ and ‘Akagomughi’ were the major ancestors with 6, 5, 4, and 4% of total genetic contribution to the Egyptian wheat gene pool, respectively. Egypt, United States of America, Kenya and Ukraine were the major source countries with 16, 11, 9 and 7% of total genetic contribution to this gene pool, respectively. Though Marquis-Thatcher germplasm from North America has the greatest influence on overall Egyptian cultivars, Mexican-based sources of dwarfing and high yield, derived from ancestors such as ‘Akagomughi’ and ‘Daruma’ and exploited by the International Maize and Wheat Improvement Center (CIMMYT, were very prominent in Egyptian cultivars post 1970’s.

Bhoja R. Basnet

2011-04-01

267

Ancient Egyptian roots in the modern medical and pharmaceutical civilisation.  

Science.gov (United States)

The Egyptian civilisation was long before any of the neighbouring countries. Medicine and pharmacy in ancient Egypt were deeply rooted since mellenia of years. The ancient Egyptian medical papyri, discovered more than a century ago, proved that the Egyptians were the first to attain civilisation, which was copied and adopted by all the neighbouring countries and not the reverse as believed by the Europeans that the ancient Greece was the cradle of civilisation. PMID:11609032

El-Gammal, S Y

1994-07-01

268

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

269

Rise of the Israelo-Egyptian cooperation for energy  

International Nuclear Information System (INIS)

Cooperation between Israel and Egyptian Arab Republic relative to the petroleum trade spreads out. The most important project is about a joint venture to build in three years a petroleum refinery able to treat 100 000 barrels by day of egyptian heavy crude oil and light petroleum coming from Golf countries. This refinery would be in the area of Ameriya. An other Israelo-Egyptian project of refinery at Port-Said is under negotiations. Israel signed an Egyptian natural gas purchasing contract and then an agreement to build a gas pipeline to convey this natural gas

270

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)

271

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

272

Immunostimulant Effect of Egyptian Propolis in Rabbits  

OpenAIRE

The present experiment was conducted to study the effect of ethanolic extract of Egyptian propolis given alone or in combination with inactivated Pasteurella multocida vaccine on rabbits challenged with a virulent strain of Pasteurella multocida. Fifty-six New-Zealand rabbits, 6–8 weeks old and non-vaccinated against pasteurellosis, were randomly divided into eight equal groups. The first group was kept as a control for the experiment. The other groups received different treatments with pro...

Nassar, Somya A.; Mohamed, Amira H.; Soufy, Hamdy; Nasr, Soad M.; Mahran, K. M.

2012-01-01

273

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

274

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

275

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)

276

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

Directory of Open Access Journals (Sweden)

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

277

A new look at old bread: ancient Egyptian baking  

Directory of Open Access Journals (Sweden)

Full Text Available Despite abundant archaeological, pictorial and textual evidence of ancient Egyptian life and death, we have little detailed information about the staple diet of most of the population. Now experimental work by a postdoctoral Wellcome Research Fellow in Bioarchaeology at the Institute is revealing how the ancient Egyptians made their daily bread.

Delwen Samuel

1999-11-01

278

Turn-of-the-Century Egyptian School Days.  

Science.gov (United States)

Traces the evolution of Egyptian government secondary schools from the 1880s to the 1920s, showing how their austerity and elitism declined after 1906 and analyzing the interaction of Egyptian, English, and French educational traditions. Explains how the secondary school environment shaped Egypt's political, cultural, and professional leaders…

Reid, Donald M.

1983-01-01

279

SOME IMPORTANT FACTORS AFFECTING EVOLUTION OF ACTIVITY BASED COSTING (ABC SYSTEM IN EGYPTIAN MANUFACTURING FIRMS  

Directory of Open Access Journals (Sweden)

Full Text Available The present investigation aims to determine the factors affecting evolution of Activity Based Costing (ABC system in Egyptian case. The study used the survey method to describe and analyze these factors in some Egyptian firms. The population of the study is Egyptian manufacturing firms. Accordingly, the number of received questionnaires was 392 (23 Egyptian manufacturing firms in the first half of 2013. Finally, the study stated some influencing factors for evolution this system (ABC in Egyptian manufacturing firms.

Karim MAMDOUH ABBAS

2014-04-01

280

Interleukin 28B polymorphisms and therapy response in Egyptian hepatitis C genotype-4 patients.  

Science.gov (United States)

Hepatitis C infection represents a major health problem in Egypt; only 20% of patients undergo spontaneous clearance of the virus and around 25% of all patients progress to develop cirrhosis. More than 90% of Egyptian patients have hepatitis C virus (HCV) genotype-4. Combined pegylated interferon and oral ribavirin are the current standard therapies for HCV-4. The aim of the work is to evaluate the predictive power of the rs12979860 IL28B SNP and rs12980275 IL28B SNP for treatment response in Egyptian patients infected with HCV genotype 4. One hundred eleven HCV patients receiving combined treatment were studied for rs12979860 and rs12980275 polymorphisms by the restriction fragment length polymorphism technique. The rs12979860 CC and rs12979860 AA genotypes were significantly associated with sustained virological response (p=0.001). Our results suggest that studying IL28B polymorphisms contribute to proper prediction of response to standard therapies in Egyptian patients, optimizing cost effectiveness, and minimizing unneeded adverse effect of therapy. PMID:24999753

Gouda, Heba M; El-Saadany, Zainab A; Foad, Neveen B; Salama, Rabab M

2014-09-01

281

German-Egyptian seminar on environmental research  

International Nuclear Information System (INIS)

Industrial development and scientific advancement have opened new frontiers of interest and challenges. Anthropogenic activities are increasingly upsetting the natural environmental balance and are at the same time shifting from local impact to global importance. Science is confronted with the challenge to answer the question of what are the consequences of anthropogenic changes to the environment and to help politics formulate countermeasures for the sake of a sustainable future. Protect results achieved within the Egyptian-German cooperation were presented to the scientific community and to the interested public and discussions on future lines of actions took place. (orig./KW)

282

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.)

283

Rethinking Time Allocation of Egyptian Females  

OpenAIRE

The present research explores for the first time to our best knowledge the extremely biased division of labor within Egyptian households. Time activities in respect of paid and unpaid work are an important aspect of this study. The classical dichotomy of "work in the market" versus "leisure" may serve as a good approximation of the role the male plays in the production activity of the household but does gross injustice to the female since it overlooks the whole time she spends, outside the ma...

Hendy, Rana

2010-01-01

284

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

285

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

286

studies on iron availability in Egyptian soils using tracer technique  

International Nuclear Information System (INIS)

four experiments were conducted to study the available fe in some egyptian soils, representing alluvial,, calcareous, and sandy soils, including the following:1) estimation of soil available iron using different chemical methods as well as E-value and evaluated against biological method. 2) differentiation of iron as Fe2+ and Fe2+ in some selected egyptian soils by using the modified method (charlot,1966). 3) determination of total Fe in soil using neutron activation analysis. 4) the relative importance of Fe-diffusion in egyptian soils under different treatments

287

EGYPTIAN MUTUAL FUNDS ANALYSIS: HISTORY, PERFORMANCE, OBJECTIVES, RISK AND RETURN  

Directory of Open Access Journals (Sweden)

Full Text Available The present research aims to overview the mutual fund in Egypt. The establishment of the first mutual funds was achieved in 1994. Nowadays, the total mutual funds reached 90 funds , approximately. The income funds represent the largest share of the Egyptian mutual funds (40%, growth funds (25% and the private equity funds is at least (1%. The total population of the Egyptian mutual funds reached 22. Finally, the study proved that the Egyptian mutual funds have an impact on fund return , total risk and systemic; when analysis relationship between risk and return. The study found influencing for mutual fund's objectives on Sharpe and Terynor ratios.

Petru STEFEA

2013-10-01

288

Nutritive Value of Irradiated Egyptian Truffles  

International Nuclear Information System (INIS)

In this study, the nutritive value of truffles was evaluated as protein efficiency ratio (C-PER) compared with casein C-PER. of white truffle was higher than brown truffle and therefore had a better nutritional quality over the brown truffle. Egyptian truffle could be considered as a good source of protein with good essential amino acids content and high nutritive value. It was found that white and brown truffles (Al-Kamah) grow in the north westarn coast of the Egyptian desert, white truffle was identified as Tirmania nivea while brown truffle was identified as Terfezia boudieri. Rats were fed on normal diet as (basal diet) for two weeks, then they were fed on the same diet with 10% of casein (control diet), replaced by 20% of irradiated and non irradiated white and brown truffle dried samples as a protein source. The nutritive value parameters were measured at the end of the experiment, gains in body weight, daily food intake, feed conversion ratio, protein efficiency ratio were measured, biological value, the biological effect on liver kideny function serum glucose. LDL and HDL-cholesterol were investigated too

289

General fire protection guidelines for Egyptian nuclear installations  

International Nuclear Information System (INIS)

The purpose of this paper is to establish the regulatory requirements that will provide and ensure fire protection of Egyptian nuclear installations. Two or more classes of occupancy are considered to occur in the same building or structure. Fire protection measures and systems were reviewed for four of the Egyptian nuclear installations. These are Egypt's first research reactor (ET-RR-1) building and systems, hot laboratories buildings and facilities, the building including the AECL type JS-6500 industrial cobalt-60 gamma irradiator ''Egypt's Mega Gamma I'' and Egypt's second research multi-purpose reactor (MPR). A brief review is given about fire incidents in Egypt, and descriptions of the only fire reported at one of the Egyptian nuclear installations over more than 35 years of operating these installations. The study outlines the various aspects of fire protection with a view to define the relevant highlights and scope of an Egyptian guidelines. (author)

290

Ægypticisme, ægyptomani. Egyptian revival : Om begreberne og deres anvendelse  

DEFF Research Database (Denmark)

To see Egypticism as merely a variety of neo-classicism sems too restrictive when one realizes the extent to which Western civilization has been inspired by Egypt. The interchangeability of terms covering that inspiration is confusing, so an attempt has been made to standardize them. The results are often contrary to what the ancients themselves would have understood or condoned. Practical considerations of form or a perenial sense of aesthetics, rather than Egyptian prototypes, may be behind much that looks Egyptian.

Langkjær, Michael Alexander

2001-01-01

291

Nitrogenase Activity of Pseudomonas corrugata Isolated from Egyptian Lettuce  

Directory of Open Access Journals (Sweden)

Full Text Available In the present study an isolate capable of root colonization of Egyptian Lettuce was isolated from the roots after selective enrichment. The isolated strain was identified as Pseudomonas corrugata by using Biochemical and Biolog identification system. The isolate has been found to be positive for nitrogenase activity. This root-colonized bacterium has not been previously isolated from Egyptian Lettuce and could be used as a bio-fertilizer.

Emad A. Abada

2006-01-01

292

Spectral signature of Egyptian crude oils  

Science.gov (United States)

Crude petroleum oils are complex mixtures of diverse hydrocarbons, in widely varying compositions, that originate from a variety of geological sources. Fluorescence emission spectra have been measured for two types of Egyptian crude petroleum oil, its light and heavy products over a broad range of excitation and emission wavelengths. Both types of crude oil products are characterized by spectral signatures with a differing topography: the number of fluorescent peaks, their coordinates (?ex, ?em) on the plane of the three dimensions spectrum, and the shape of the bands formed by the contour line density, changeable in either direction. The refined light oil shows emission spectra at ?max between 350 and 500 nm according to the excitation wavelength. The refined heavy oil shows very broad unstructured emission spectra with ?max > 400 nm. As a group, they could certainly be distinguished from the light oil samples and most of the crude oil.

Ghatass, Z. F.; Nashed, A. W.; Saleh, I. H.; Mohmed, M. M.

2014-11-01

293

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

294

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)

295

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

296

Upgrading the Egyptian Scanning Land mine Detectors  

International Nuclear Information System (INIS)

This article presents and discusses the upgrading processes which were performed to increase the detection capability of the Egyptian Scanning Land mine Detectors, ESCALAD system. The upgrading processes include different designs which were made to mount the detectors tray. These arrangements aim to overcome the effect of the soil surface roughness and stand off distance on the scanning capability. Also a more stable and reliable PCI data acquisition board with fast Digital I/O up to 125 M/s was used. Moreover, data acquisition software which uses different algorithms for background subtraction and 2D-image filtration was build and developed. The software was build using Qt-cross-platform application and other Linux based packages. Examples of images constructed from experimental measurements using the upgraded and modified software are given and discussed. The obtained displayed images show more developed improvements and reliability.

297

Did the ancient egyptians discover Algol?  

Science.gov (United States)

Fabritius discovered the first variable star, Mira, in 1596. Holwarda determined the 11 months period of Mira in 1638. Montanari discovered the next variable star, Algol, in 1669. Its period, 2.867 days, was determined by Goodricke (178). Algol was associated with demon-like creatures, "Gorgon" in ancient Greek and "ghoul" in ancient Arab mythology. This indicates that its variability was discovered much before 1669 (Wilk 1996), but this mythological evidence is ambiguous (Davis 1975). For thousands of years, the Ancient Egyptian Scribes (AES) observed stars for timekeeping in a region, where there are nearly 300 clear nights a year. We discovered a significant periodicity of 2.850 days in their calendar for lucky and unlucky days dated to 1224 BC, "the Cairo Calendar". Several astrophysical and astronomical tests supported our conclusion that this was the period of Algol three millennia ago. The "ghoulish habits" of Algol could explain this 0.017 days period increase (Battersby 2012).

Jetsu, L.; Porceddu, S.; Porceddu, S.; Lyytinen, J.; Kajatkari, P.; Markkanen, T.; Toivari-Viitala, J.

2013-02-01

298

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

299

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

300

Antidiarrhoeal activity of some Egyptian medicinal plant extracts.  

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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 (P3.2 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

301

Language Maintenance and Transmission: The Case of Egyptian Arabic in Durham, UK  

OpenAIRE

This sociolinguistic micro-level study examines experiences of five Egyptian families, living in Durham, UK, who are trying to transmit Egyptian Arabic to their children. Answers to the following questions are sought: first, how these language-minority families describe barriers to and supports for passing on Egyptian Arabic to their children? Second, what language decisions they make in order to fulfill their roles? Third, how such decisions are linked to their identity as Egyptians livin...

Yasser Ahmed Gomaa

2011-01-01

302

Aflatoxin B1 in common Egyptian foods.  

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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

303

Iron forms in some egyptian soils  

International Nuclear Information System (INIS)

The present study is an attempt to find out the available forms of iron (Fe2+ and Fe3+) in five egyptian soils samples, representing alluvial, calcareous and sandy soils. Concerning the iron content of soil either Fe2+ or Fe3+, the tested soil types were relatively arranged in the order alluvial> calcareous> sandy soil. In spite of the considerable variations in the soil content of iron cations, the Fe2+/Fe3+ ratio was almost kept constant around 0.83. The uniformity of the ferrous : ferric ratio in the different tested soil types indicates their similarity in their redox-potential, pH and their environmental conditions, particularly, the aeration and partial O2- pressure degree. Fe2+/Fe3+ being less than unity suggests that the Fe2+ Fe3+ reaction tends towards the forward direction, i.e., to the Fe3+ formation. As a result of the pot experiment, significant correlations have been found between the laboratory determined soil Fe2+ and both of the plant Fe-uptake and the plant dry matter weight

304

?-Globin Mutations in Egyptian Patients With ?-Thalassemia.  

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?-thalassemia is a common hereditary disorder, particularly in Middle Eastern countries. More than 200 mutations in the ? globin gene have been reported; most are point mutations in functionally important regions (HBB; OMIM #141900)). The spectrum of mutations varies significantly between different geographical regions; only a few common mutations of ?-globin cause ?-thalassemia in each population. The aim of this study was to determine the spectrum of mutations that cause ?-thalassemia in the North Coast of Egypt and to investigate their correlation with the phenotypic severity of ?-thalassemia. We carried out our study with a total of 47 Egyptian patients (25 male and 22 female) confirmed to have ?-thalassemia. Evaluation of ?-thalassemia mutations revealed the presence of 10 ?-globin mutations. The most frequently encountered mutations were intronic: IVS 1.6 [T>C] (27.66%) and IVS 1.110 [G>A] (22.35%), followed by IVS 2.848 [C>A], IVS 1.1 (G>A), and IVS 2.745 [C>G]. We observed the exonic and promoter mutations less frequently. A homozygous mutation was found in 24 patients (51%) and compound heterozygous mutations were found in 13 patients (28%). However, in 9 patients (19%), we identified only 1 mutation. In 1 patient (2%), we detected no mutation. The detection rate of the method that we used in our population was 88% (83 of the tested 94 alleles). The results we obtained did not reveal any correlation between genotype and phenotype among patients with ?-thalassemia. PMID:25617386

Elmezayen, Ammar D; Kotb, Samia M; Sadek, Nadia A; Abdalla, Ebtesam M

2015-01-01

305

Transformations of geometrical objects in middle Egyptian mathematical texts.  

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This paper analyzes the algorithmic structure of geometrical problems in Egyptian papyri of the first half of the second millennium B.C. Processes of transformation of quantities from "false" values into actual values, and conversions from quantities expressed in the abstract system of numbers into metrological quantities, are known in Egyptian mathematics. Three further processes are identified in the present contribution: transformations of "false" dimensions of geometrical objects into true dimensions; transformations of geometrical objects into other geometrical objects; transformations of linear measures of monuments. These processes have relevant implications on the algorithmic structure of the problem texts, resulting in particular in the embedding of sub-algorithms and the creation of parallel structures. More in general, their wide employment in Egyptian mathematics has significant philosophic and cultural implications. PMID:25029817

Miatello, Luca

306

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

307

Study of the factors affecting the sorption and fixation of radiocesium on some egyptian soil sediments  

International Nuclear Information System (INIS)

Studies of the sorption process of radiocesium by various Egyptian soil samples have been carried out. These samples were collected from different regions of egypt, North of the eastern desert and cairo-Alexandria desert road. A german sample, which is a pure smectite mineral, was taken as a reference for comparing the deferent investigations. The physical, chemical and mineralogical analyses of these samples were studied. The physical analysis showed that, the soil samples have the clay and silt size fractions mixed with fine sand. The samples having high percentage of the clay and silt fractions showed high specific surface area and cation exchange capacity values. The mineralogical properties showed that, the ,majority of egyptian soils consist manly of the smectite and vermiculite type of clay minerals with different proportions of kaolinite, mica and quartz. The samples (V-1, 46-48 km, K-s and 74-B) containing high smectite or vermiculite minerals have higher cation exchange capacities and specific surface area than the samples (q 2, 78-A and q 7) with high kaolinite, mica and quartz content. Chemical analysis showed that the sample having higher potassium and iron oxides were having lower cation exchange capacity values than the samples containing higher magnesium oxide. 19 figs., 22 tabs., refs

308

Epidemiological Aspects of Intrafamilial Spread of HCV Infection in Egyptian Population: A Pilot Study  

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Full Text Available Aim: Liver disease is a leading cause of morbidity and mortality among Egyptians. The major cause is infection with HCV, with 70 000 up to 140 000 newly reported cases annually. The objectives of this study were to determine the prevalence of anti-HCV antibodies among household contacts of HCV index cases and to identify the possible risk factors of transmission of HCV among Egyptian families. Material and Methods: The present external pilot study (double centre study was performed on a convenient sample of 125 index cases and their 321 household family contacts recruited from Mansoura & Cairo University where 2 questionnaires were used to collect data from the index & their related contacts. The all were exposed to clinical examinations, routine laboratory testing & screening for the prevalence of Anti-HCV antibodies. Results: The prevalence of anti-HCV seropositivity among household contacts of index cases was found to be 13.7% which husbands of female index cases ranked first followed by wives of male index cases (36.36% versus 17.86% respectively, P

Mahmoud El-Bendary

2014-05-01

309

English Majors' Errors in Translating Arabic Endophora: Analysis and Remedy  

Science.gov (United States)

Egyptian English majors in the faculty of Education, South Valley University tend to mistranslate the plural inanimate Arabic pronoun with the singular inanimate English pronoun. A diagnostic test was designed to analyze this error. Results showed that a large number of students (first year and fourth year students) make this error, that the error…

Abdellah, Antar Solhy

2007-01-01

310

Genetic Mutation in Thrombophilic Egyptian Patients  

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Full Text Available Factor V Leiden and the prevalence of PAI-1 promoter 4G/5G polymorphism were examined in 48 Egyptian patients who presented with deep venous thrombosis. They were investigated for the presence of APCR, where positive cases were further subjected to DNA analysis by PCR for detection of factor V Leiden mutation. PAI-1 polymorphism was also studied in patients and 40 healthy individuals matching in age and sex as controls by PCR-RFLP to demonstrate the influence of 4G/5G dimorphism of the PAI-1 promoter gene on thrombotic risk. Present results revealed the presence of APCR in 14/48 (29.2% cases out of the whole thrombotic cases by DNA analysis, only 10/14 (71.4% were found carrying the mutant factor V gene (p<0.05. No synergistic effect was observed between the 2 polymorphisms when analyzed together. There was an association between the 4G allele of the PAI-1 gene and the risk of thromboembolism in patients suffering from DVT compared with healthy control (41.7% vs. 20%; p = 0.04, OR 2.85; 95% CI 1.089-7.493. The prevalence of 5G homozygous carriers was significantly lower in patients than in controls (12.5% vs. 40%; p = 0.006, OR 0.214, 95% CI 0.074-0.621. In conclusion, APCR as a result of factor V Leiden is the most commonly recognized inherited risk factor for thromboembolism. In patients with deep vein thrombosis the 4G polymorphism of PAI-1 gene promoter should be taken into consideration as a facilitating condition for pathological fibrinolysis together with other environmental and genetic factors.

Nehal Salah Hasan

2006-01-01

311

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

312

Assessment of hepatic and pancreatic iron overload in pediatric Beta-thalassemic major patients by t2* weighted gradient echo magnetic resonance imaging.  

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Background. MRI has emerged for the noninvasive assessment of iron overload in various tissues. The aim of this paper is to evaluate hepatic and pancreatic iron overload by T2(?) weighted gradient echo MRI in young beta-thalassemia major patients and to correlate it with glucose disturbance and postsplenectomy status. Subjects and Methods. 50 thalassemic patients, in addition to 15 healthy controls. All patients underwent clinical assessment and laboratory investigations. Out of 50 thalassemic patients, 37 patients were splenectomized. MRI was performed for all subjects. Results. All patients showed significant reduction in the signal intensity of the liver and the pancreas on T2(?)GRD compared to controls, thalassemic patients who had abnormal glucose tolerance; diabetic and impaired glucose tolerance patients displayed a higher degree of pancreatic and hepatic siderosis and more T2(?) drop in their signal intensity than those with normal blood sugar level. Splenectomized thalassemic patients had significantly lower signal intensity of the liver and pancreas compared to nonsplenectomized patients. Conclusion. T2(?) gradient echo MRI is noninvasive highly sensitive method in assessing hepatic and pancreatic iron overload in thalassemic patients, more evident in patients with abnormal glucose tolerance, and is accelerated in thalassemic splenectomized patients. PMID:23606980

Youssef, Doaa Mohammed; Fawzy Mohammad, Faten; Ahmed Fathy, Ayman; Aly Abdelbasset, Maha

2013-01-01

313

Overview of the Egyptian Radiation Monitoring Network and Radioactivity Levels of the Egyptian Territories  

International Nuclear Information System (INIS)

The Chernobyl accident in 1986 arose much concern throughout the world regarding the detection of contamination, control of contaminated food, related legislation and environmental monitoring. In Egypt, the environmental radioactivity monitoring program involves the establishment of a radiation monitoring network stations for continuous monitoring of ambient gamma radiation levels in the air, water, aerosols, dust particles and conventional gas pollutants. The network consists of 42 field stations for gamma monitoring of air, 14stations for beta aerosols and 15 stations for conventional pollutants. soil samples adjacent to the stations distributed all over the Egyptian territories have been collected and analyzed by gamma spectrometry. The natural radionuclides K40, U-238 series Th-232 series and the global fallout Cs-137 are found in all the collected samples. The absorbed dose rates are calculated and presented

314

Terahertz tomographic imaging of XVIIIth Dynasty Egyptian sealed pottery  

OpenAIRE

A monochromatic millimeter-wave imaging system coupled with an infrared temperature sensor has been used to investigate historic objects preserved at the Museum of Aquitaine (France). In particular, two-dimensional and three-dimensional analyses have been performed in order to reveal the internal structure of nearly 3500-year-old sealed Egyptian jars.

Caumes, Jean-pascal; Younus, Ayesha; Salort, Simon; Chassagne, Bruno; Recur, Benoi?t; Zie?gle?, Anne; Dautant, Alain; Abraham, Emmanuel

2011-01-01

315

Berardinelli-Seip syndrome type 1 in an Egyptian child  

OpenAIRE

Berardinelli-Seip syndrome type 1 or Berardinelli-Seip congenital lipodystrophy 1 (BSCL1) is a very rare genetic disorder characterized by lipoatrophy, hypertriglyceridemia, hepatomegaly and acromegaloid features. Its prevalence in Egypt is not known. Here, we report case of a 12-year-old Egyptian boy with the clinical, metabolic and molecular genetics manifestations of BSCL1 including overt diabetes mellitus.

Metwalley, Kotb Abbass; Farghaly, Hekma Saad

2014-01-01

316

Strategic Leadership and Its Application in Egyptian Universities  

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Full Text Available 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, objectives, roles, requirements, and application obstacles. Relate to the suffering of the Egyptian universities of many problems that limit their efficiency and effectiveness. This led to the need to search for new approaches as strategic leadership for eliminate of these problems. The study used a descriptive approach for its suitability for the nature of the study. The study found multiple reasons for the application of strategic leadership style in Egyptian universities as a result of  the problems the leaderships of the Egyptian universities suffer from  which limits its efficiency and effectiveness. Study recommended starting applying this pattern quickly after all the positive results it achieved in many universities.   

Hany R. Alalfy

2014-11-01

317

Muslim Egyptian and Lebanese Students' Conceptions of Biological Evolution  

Science.gov (United States)

In this study, we investigated distinctions among the diversity of religious traditions represented by Lebanese and Egyptian Muslim high school students regarding their understanding and acceptance of biological evolution and how they relate the science to their religious beliefs. We explored secondary students' conceptions of evolution among…

BouJaoude, Saouma; Wiles, Jason R.; Asghar, Anila; Alters, Brian

2011-01-01

318

Expression of therapeutic misconception amongst Egyptians: a qualitative pilot study  

OpenAIRE

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:...

Silverman Henry J; Khalil Susan S; Wazaify Mayyada

2009-01-01

319

Terahertz tomographic imaging of XVIIIth Dynasty Egyptian sealed pottery.  

Science.gov (United States)

A monochromatic millimeter-wave imaging system coupled with an infrared temperature sensor has been used to investigate historic objects preserved at the Museum of Aquitaine (France). In particular, two-dimensional and three-dimensional analyses have been performed in order to reveal the internal structure of nearly 3500-year-old sealed Egyptian jars. PMID:21743572

Caumes, Jean-Pascal; Younus, Ayesha; Salort, Simon; Chassagne, Bruno; Recur, Benoît; Ziéglé, Anne; Dautant, Alain; Abraham, Emmanuel

2011-07-10

320

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

321

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

322

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 ...

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

2012-01-01

323

The "Challengers": The driving forces behind the youth's demand for change in the Egyptian Uprising  

OpenAIRE

This study reflects on what were the most important motivational factors for the Egyptian youth to participate in the 25th of January 2011 revolution. By applying a conceptual framework from social movement theory, it attempts to explain what paved the way for the Egyptian youth's participation in the 18 days of protest in Tahrir Square. The analysis is based on qualitative interviews conducted with Egyptian youth who participated in the revolution, as well as on interviews with area speciali...

Mohn, Giti Carli

2012-01-01

324

The "Challengers":The driving forces behind the youth's demand for change in the Egyptian Uprising  

OpenAIRE

This study reflects on what were the most important motivational factors for the Egyptian youth to participate in the 25th of January 2011 revolution. By applying a conceptual framework from social movement theory, it attempts to explain what paved the way for the Egyptian youth's participation in the 18 days of protest in Tahrir Square. The analysis is based on qualitative interviews conducted with Egyptian youth who participated in the revolution, as well as on interviews with area speciali...

Mohn, Giti Carli

2012-01-01

325

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

326

Combined Therapy with Deferiprone and Desferrioxamine as Compared to Desferasirox on Ventricular Function in Thalassemia Major Patients  

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Full Text Available AbstractBackgroundMyocardial iron overload is the leading cause of death in patients with beta-thalassemia major.Combined therapy with deferiprone(DFP and desferrioxamine (DFOwere suggested to be more effective than deferasirox(DFX for removing heart iron. Deferasirox has recently been made available but its long-term efficacy on cardiac function has not yet been established. Our study aimed to compare the effectiveness of deferiproneanddesferrioxaminewith deferasiroxon ventricular function in thalassemia major patients.Materials and MethodsIn this clinical trialstudy,72thalassemia major (TM patients were randomised to receive either deferipronecombined with desferrioxamineanddeferasirox ,and thencardiac function were evaluated. Data were analysed for left ventricular ejection fractions(LVEFat baselinebyechocardiograpy, following 12 months of treatment.Results72 TM patientswere enrolled in this study lasting 12 months,36 TMwere placed on DFP/DFO (DFP,50–86 mg/kg body weight; DFO, 24–52 mg/kg body weigh,36received DFX(range 18–40 mg/kg body weight.In 36 patients receiving combined therapy, left ventricular ejection fractionincreased from 59.3+/-5.7% to 63.7+/-5.1% (p=0.001 over 12 months [baseline LVEF values 56-61%]. deferasirox showed no change in LVEF (p = 0.93.We found improvement of left ventricular ejection fractionsin the deferiprone combined with desferrioxamine versus the deferasirox group (P = 0.008.ConclusionThe patients treated with combined therapy with deferiprone and desferrioxamineshowed better systolic ventricular function compared to the patients treated with deferasirox.The patients treated with combined therapy with deferiprone and desferrioxamine showed better systolic ventricular function compared to the patients treated with deferasirox.

Sartipzade NH

2012-09-01

327

Surveillance of natural acquired antibodies to Haemophilus influenzae type b among children in Cairo-Egyptian.  

Science.gov (United States)

In Egypt, the incidence of invasive Haemophilus influenzae type b (Hib) disease is not common and the vaccine is still not included into the national immunization program. The aim of this study was to evaluate the natural immunity to Hib in healthy children at Egyptian capitol (Cairo). One hundred and two children were enrolled in our study of age one month to 15 year. Anti-capsular polysaccharide (PRP) of Hib specific IgM and IgG antibody concentrations were estimated using enzyme immunoassay. The majority (64.7%, 75.5%) of children had a protective levels>or=15 microg/ml of specific antibodies (IgM and IgG). Fortunately, a total of 95.5% of the children under 5 years old had a protective level. Our result will provide evidence that in Egypt capitol, the natural immunity against Hib can be acquired at an early age. PMID:16424597

Redwan, El-Rashdy M; Elsawy, Abdelrahman

2005-01-01

328

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

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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

329

Chemical analysis of Yemeni archaeological cheramics and the Egyptian enigma  

International Nuclear Information System (INIS)

Archaeological reconnaissance in Yemen produced samples of mediaeval Islamic ceramics in a 100 km2 region. The ceramics dated from 700 A.d. to 1750 A.D. and initial research indicated that they were all locally made products. 12 types of ceramics were selected for sampling on the basis of stylistic decoration. Six laboratory samples of each type were subjected to neutron activation analysis for the short-lived isotope producing elements using the SLOWPOKE reactor at the University of Toronto. A comparison with Egyptian pottery was carried out. The statistical analysis conducted on the Yemeni and Egyptian pottery produced discrete differences in their elemental composition which the archaeologist and the chemical scientist might not otherwise recognise. It is concluded that artifacts must be analyzed with due respect given to the archeological context, the elemental chemistry, and sound statistical procedures. (author)

330

Ancient Egyptian chronology and the astronomical orientation of pyramids  

Science.gov (United States)

The ancient Egyptian pyramids at Giza have never been accurately dated, although we know that they were built approximately around the middle of the third millennium BC. The chronologies of this period have been reconstructed from surviving lists of kings and the lengths of their reigns, but the lists are rare, seldom complete and contain known inconsistencies and errors. As a result, the existing chronologies for that period (the Old Kingdom) can be considered accurate only to about +/-100 years, a figure that radiocarbon dating cannot at present improve. Here I use trends in the orientation of Old Kingdom pyramids to demonstrate that the Egyptians aligned them to north by using the simultaneous transit of two circumpolar stars. Modelling the precession of these stars yields a date for the start of construction of the Great Pyramid that is accurate to +/-5 yr, thereby providing an anchor for the Old Kingdom chronologies.

Spence, Kate

2000-11-01

331

The Suitability of Some Egyptian Smectitic Clays for Mud Therapy  

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Full Text Available The present study was carried out to assess the suitability of smectitic clays from Fayum, G. Hamza and G. Um Qamar, Egypt, for application in mud therapy. The textural, mineralogical and chemical composition of the Egyptian claystones showed that they are comparable to those of muds used in pelotherapy in several other countries (for example, Italy, Spain, Turkey and Portugal. The Egyptian claystones have good heat capacities and their pastes can be applied in different treatments. The trace elements present in the claystones, namely, As, Pb, Cu, Zn, Co, Ni and Cr are within the normal ranges of average natural mud and shale and they are of no significant concern for human health in mud applications.

S.D. Abayazeed

2012-01-01

332

Nile Crossings: Hospitality and Revenge in Egyptian Rural Narratives  

OpenAIRE

This essay looks at acts of hospitality and revenge as constituent elements of a broad social code in rural Egyptian narratives. By looking at five stories in particular, I argue that hospitality and revenge work in complementarity, and that they often trespass and blur each other’s social and literary borders, creating ambiguity and complexity in the stories. The traditional rules that govern hospitality are at times challenged or inverted by hostile intentions, and revenge may be exacted ...

Granara, William E.

2010-01-01

333

Sociodemographic factors responsible for blindness in diabetic Egyptian patients  

OpenAIRE

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 pr...

Kgi, Abueleinen; El-Mekawey H; Ys, Saif; Khafagy A; Hi, Rizk; Em, Eltahlawy

2011-01-01

334

New Terpenes from the Egyptian Soft Coral Sarcophyton ehrenbergi  

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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

335

Angiographic characteristics of central serous chorioretinopathy in an Egyptian population  

OpenAIRE

AIM: To describe and analyze the demographic characteristics and to determine the angiographic features of acute central serous chorioretinopathy(CSCR) in an Egyptian population. METHODS: A series of consecutive patients presenting with acute idiopathic CSCR to Mansoura Ophthalmology Center Mansoura University who underwent fluorescein angiography (FA) within a 3-year-period (between January 1, 2007 and December 31, 2009) was retrospectively studied. Patient demographics and angiographic feat...

Shahin, Maha M.

2013-01-01

336

Berardinelli-Seip syndrome type 1 in an Egyptian child.  

Science.gov (United States)

Berardinelli-Seip syndrome type 1 or Berardinelli-Seip congenital lipodystrophy 1 (BSCL1) is a very rare genetic disorder characterized by lipoatrophy, hypertriglyceridemia, hepatomegaly and acromegaloid features. Its prevalence in Egypt is not known. Here, we report case of a 12-year-old Egyptian boy with the clinical, metabolic and molecular genetics manifestations of BSCL1 including overt diabetes mellitus. PMID:24959019

Metwalley, Kotb Abbass; Farghaly, Hekma Saad

2014-01-01

337

Kenny-Caffey syndrome type 1 in an Egyptian girl  

OpenAIRE

Kenny-Caffey syndrome type 1 (KCS1) (OMIM 244460) is a rare syndrome characterized by growth retardation, uniformly small slender long bones with medullary stenosis, thickened cortex of the long bones, hypocalcemia possibly with tetany at an early age and normal intelligence. The primary outcome of KCS1 is short stature. We present here an Egyptian girl aged 32 months with typical feature of KCS1.

Metwalley, Kotb Abbass; Farghaly, Hekma Saad

2012-01-01

338

Kenny-Caffey syndrome type 1 in an Egyptian girl.  

Science.gov (United States)

Kenny-Caffey syndrome type 1 (KCS1) (OMIM 244460) is a rare syndrome characterized by growth retardation, uniformly small slender long bones with medullary stenosis, thickened cortex of the long bones, hypocalcemia possibly with tetany at an early age and normal intelligence. The primary outcome of KCS1 is short stature. We present here an Egyptian girl aged 32 months with typical feature of KCS1. PMID:23087875

Metwalley, Kotb Abbass; Farghaly, Hekma Saad

2012-09-01

339

Kenny-Caffey syndrome type 1 in an Egyptian girl  

Science.gov (United States)

Kenny-Caffey syndrome type 1 (KCS1) (OMIM 244460) is a rare syndrome characterized by growth retardation, uniformly small slender long bones with medullary stenosis, thickened cortex of the long bones, hypocalcemia possibly with tetany at an early age and normal intelligence. The primary outcome of KCS1 is short stature. We present here an Egyptian girl aged 32 months with typical feature of KCS1. PMID:23087875

Metwalley, Kotb Abbass; Farghaly, Hekma Saad

2012-01-01

340

Berardinelli-Seip syndrome type 1 in an Egyptian child  

Science.gov (United States)

Berardinelli-Seip syndrome type 1 or Berardinelli-Seip congenital lipodystrophy 1 (BSCL1) is a very rare genetic disorder characterized by lipoatrophy, hypertriglyceridemia, hepatomegaly and acromegaloid features. Its prevalence in Egypt is not known. Here, we report case of a 12-year-old Egyptian boy with the clinical, metabolic and molecular genetics manifestations of BSCL1 including overt diabetes mellitus. PMID:24959019

Metwalley, Kotb Abbass; Farghaly, Hekma Saad

2014-01-01

341

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.)

342

Determinants of Work Relation Perception: Organizational Culture in Egyptian Workplaces  

OpenAIRE

This study aimed at investigating different types of organizational cultures common in Egyptian workplaces andhow they might be influenced by the type of ownership (governmental, public, or private), the region in whichthe organization exists (Cairo, North Egypt, or South Egypt), and the organization size (large vs. small).Organizational cultures were divided, according to the Competing Values Model proposed Cameron and Quinn(1999/2006), into four types: Market, Hierar...

Mohamed Taha Mohamed

2013-01-01

343

Development of benchmark models for the Egyptian residential buildings sector  

OpenAIRE

The aim of this study is to develop representative simulation building energy data sets and benchmark models for the Egyptian residential sector. This study reports the results of a recent field survey for residential apartment buildings in Egypt. Two building performance simulation models are created reflecting the average energy consumption characteristics of air-conditioned residential apartments in Alexandria, Cairo and Asyut. Aiming for future evaluation of the cost and energy affects of...

Attia, Shady

2012-01-01

344

General fire protection guidelines for egyptian nuclear facilities. Vol. 4  

International Nuclear Information System (INIS)

The purpose of this paper is to establish the regulatory requirements of that will provide and ensure fire protection of egyptian nuclear facilities. Those facilities that use, handle and store low and/or medium radioactive substances are included. Two or more classes of occupancy are considered to occur in the same building or structure. Fir protection measures and systems were reviewed for three of the egyptian Nuclear facilities. These are egypt first nuclear reactor (ETRR-1) building and systems, hot laboratories buildings and facilities, and the building including the AECL type Is-6500 industrial cobalt-60 gamma irradiator Egypt's mega gamma I. The study includes the outlines of the various aspects of fire protection with a view to define the relevant highlights and scope of egyptian guideline for nuclear installations. The study considers fire protection aspects including the following items: 1- Site selection. 2- General facility design. 3- Fire alarm, detection and suppression systems. 4- Protection for specific areas/control room, cable spreading room, computer room) 5- Fire emergency response planning. 6- Fire water supply. 7- Emergency lighting and communication. 8- Rescue and escape routes. 9- Explosion protection. 10-Manual fire fighting. 11- Security consideration in the interest of fire protection. 12- quality assurance programme. Therefore, first of all the design stage, then during the construction stage, and later during the operation stage, measures must be taken to forestall the risks associated with the outbreak of fire and to ensure that consequences of fire accidents remain limited

345

Did the ancient Egyptians migrate to ancient Nigeria?  

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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

346

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

347

The Problem of the Pyramid or Egyptian Mathematics from a Postmodern Perspective  

Science.gov (United States)

We consider Egyptian mathematics from a postmodern perspective, by which we mean suspending judgement as to strict correctness in order to appreciate the genuine mathematical insights which they did have in the context in which they were working. In particular we show that the skill which the Egyptians possessed of obtaining the general case from…

Shutler, Paul M. E.

2009-01-01

348

Ancient Egyptian Medical Texts: A Rhetorical Analysis of Two of the Oldest Papyri.  

Science.gov (United States)

Analyzes two ancient Egyptian technical texts, the Edwin Smith Surgery Manual and the Ebers Manual, to identify complex rhetorical dynamics that present or encourage substantive reformulation of medical practice and thinking within a strongly conservative, authoritarian culture. Shows how the ancient Egyptian rhetorical forms allow for challenges…

Lipson, Carol S.

1990-01-01

349

The impact on the Netherlands of the Egyptian greenhouse vegetable chain  

OpenAIRE

This report forms part of a broader analysis of the competitiveness of Dutch tomatoes, cucumbers and peppers on the European market. It describes elements of Porter's competitiveness analysis for the Egyptian horticultural sector. Within this framework, it presents an analysis of the domestic demand, the supply, the structure and strategy of firms, the network and the Egyptian government. It concludes with a SWOT analysis.

Wijnands, J. H. M.

2004-01-01

350

ASPECTS OF OBSTACLES FOR APPLYING ACTIVITY BASED COSTING (ABC SYSTEM IN EGYPTIAN FIRMS  

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Full Text Available The following investigation aims to determine the aspects of obstacles for applying Activity Based Costing (ABC system in the Egyptian case and the significant differences among the effects of such obstacles . The Study used the survey method to describe and analyze the obstacles in some Egyptian firms. The population of the study is Egyptian manufacturing firms. This survey used the number of 392 questionnaires that were used throughout the total of 23 Egyptian manufacturing firms, during the first half of 2013. Finally, the study found some influencing obstacles for applying this system (ABC and there were significant differences among the aspects of obstacles for applying ABC system in the Egyptian manufacturing firms.

Petru STEFEA

2013-10-01

351

BENCHMARKING THE EGYPTIAN MEDICAL TOURISM SECTOR AGAINST INTERNATIONAL BEST PRACTICES: AN EXPLORATORY STUDY  

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Full Text Available This paper argues that any compatible strategy for the development of medical tourism at a developing nation should be based primarily on a comprehensive benchmarking study. It has employed the benchmarking phase of a national project for the development of an Egyptian medical tourism strategy to showcase significance of such benchmarking implications. The benchmarking phase of the Egyptian project has used two main data sets to reach reliable findings: a series of best practice destinations claimed to be key players leading the future of the medical tourism sector worldwide and an extensive survey of the Egyptian medical tourism sector. Arguably, the benchmarking process was crucial for the development of the strategy to measure performance of Egypt’s medical tourism sector against international best practices, to identify gaps in the Egyptian medical tourism sector and to address main areas required to develop ‘service value chain’ for the Egyptian medical tourism sector.

Eman M. Helmy

2011-09-01

352

Study of ocular manifestations in children of thalassemia  

OpenAIRE

Background: Purpose of current study was to study the ocular manifestations in beta-thalassemia major patients and assess the ocular side-effects of iron chelating agents. Methods: Cross sectional study included 45 beta Thalassemia major patients from age group of 6months to 12 years were taken. Full medical history, thorough physical examinations were done to all patients groups, and ophthalmological examination to determine the prevalence of ocular manifestations for all patient groups a...

Gosai, Dhara K.; Mehariya, K. M.; Gosai, Jigarkumar B.

2014-01-01

353

On improvement in ejection fraction with iron chelation in thalassemia major and the risk of future heart failure  

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Full Text Available Abstract Background Trials of iron chelator regimens have increased the treatment options for cardiac siderosis in beta-thalassemia major (TM patients. Treatment effects with improved left ventricular (LV ejection fraction (EF have been observed in patients without overt heart failure, but it is unclear whether these changes are clinically meaningful. Methods This retrospective study of a UK database of TM patients modelled the change in EF between serial scans measured by cardiovascular magnetic resonance (CMR to the relative risk (RR of future development of heart failure over 1 year. Patients were divided into 2 strata by baseline LVEF of 56-62% (below normal for TM and 63-70% (lower half of the normal range for TM. Results A total of 315 patients with 754 CMR scans were analyzed. A 1% absolute increase in EF from baseline was associated with a statistically significant reduction in the risk of future development of heart failure for both the lower EF stratum (EF 56-62%, RR 0.818, p Conclusion These data show that during treatment with iron chelators for cardiac siderosis, small increases in LVEF in TM patients are associated with a significantly reduced risk of the development of heart failure. Thus the iron chelator induced improvements in LVEF of 2.6% to 3.1% that have been observed in randomized controlled trials, are associated with risk reductions of 25.5% to 46.4% for the development of heart failure over 12 months, which is clinically meaningful. In cardiac iron overload, heart mitochondrial dysfunction and its relief by iron chelation may underlie the changes in LV function.

Carpenter JP

2011-09-01

354

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)

355

Globalization and the ethical implications for the Egyptian healthcare system.  

Science.gov (United States)

Globalization is creating an extraordinary transformation to the delivery, financing and access of healthcare throughout the world. Improving standards of treatment, based on higher international standards of care and the offering of far more affordable services, is positioning third-world countries as viable participants in a more global healthcare system. The Egyptian healthcare system is evolving to meet these higher expectations in an effort to attract wealthier international tourists. It is important to understand Egypt's evolving transformation into a medical service destination so policymakers may understand the emerging ethical cocnerns this evolution may impose on this third-world and traditionally underserved population. PMID:21053650

Haley, D Rob; Adel Khalifaa, Mohamed; Bég, Sama A; Sobh, Nesma

2010-01-01

356

Instrumental neutron activation analysis of some Egyptian phosphate ore samples  

International Nuclear Information System (INIS)

Nondestructive instrumental neutron activation analysis (inaa) has been applied for the determination of up to 23 elements in 10 Egyptian phosphate ore samples from two regions in Egypt; Sebaiya (wadi-El-Nile) and the red sea coast (El-Hamrawein). The sterials (S-1, S-2 and G-2) were used in analysis. The relevant nuclear data of the determined elements are listed. The accuracy and precision of the procedure has been checked by simultaneous analysis of S-1 and S-2 standard deviation of each determination as well as the detection limits of the elements are provided. The results of the analysis are presented and discussed in details

357

Language Maintenance and Transmission: The Case of Egyptian Arabic in Durham, UK  

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Full Text Available This sociolinguistic micro-level study examines experiences of five Egyptian families, living in Durham, UK, who are trying to transmit Egyptian Arabic to their children. Answers to the following questions are sought: first, how these language-minority families describe barriers to and supports for passing on Egyptian Arabic to their children? Second, what language decisions they make in order to fulfill their roles? Third, how such decisions are linked to their identity as Egyptians living in an English-dominant country? Finally, what are the factors that helps their children to preserve Egyptian Arabic? The findings, based on data collected through in-depth, semi-structured interviews and participant observation, show that the participants regard Egyptian Arabic as a cultural core value  that is linked with other core values such as religion and identity. Consequently, a number of language-related decisions have been made and implemented to ensure the improvement of their children's Egyptian Arabic spoken proficiency level.

Yasser Ahmed Gomaa

2011-03-01

358

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

359

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

360

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

361

Environmental education in an Egyptian university: The role of teacher educators  

Science.gov (United States)

Drawing on a holistic critical paradigm of ecological sustainability, this study examined the role of teacher educators in environmental education in the Faculty of Education of one Egyptian university. The study sought to critically and collaboratively explore with a sample of six teacher educators their answers, perceptions and perspectives in relation to their knowledge and understanding of environmental problems in local/global contexts and their meanings of curriculum and pedagogical practices for fostering environmental education in their teacher education programs. The participants generally demonstrated a considerable amount of knowledge of the environmental realities and problems facing Egypt encompassing air, water and solid waste sectors. Their views concurred with national and official studies identifying these issues as the most pressing environmental problems in the country. The exploration of the institutional, social and cultural causes and developmental and/or global causes of environmental problems in Egypt led us to articulate different themes relating environmental crisis in Egypt to different issues. These issues included poverty, education, religion and development. One of the major findings of the study was the participants' view that development was the major contributor to the environmental crisis in Egypt. They all stressed that, in its pursuit of economic growth, the government did not pay due attention to the environmental costs. Sharing perspectives from a critical paradigm of ecological sustainability, the participants felt that the government needed to clearly address the economic and ecological dimensions of development. In addition, a few participants affirmed that development is the thread that ties all the different factors together bringing into the conversations other dimensions of development like the social, values, and political dimensions. Addressing the future dimension of development, all of them expressed the need for a development model that takes into consideration ecological as well as human well-being concerns. The study also presented an overview of the participants' own definitions for environmental education based on their beliefs and emerging from their practices. It also explored the content and pedagogy of environmental education within the Faculty as well as the participants' own practices and reports on the action research phase of the study. The participants agreed that the environment needs to be viewed holistically that includes human beings in relation to each other as well as to other beings and their surroundings. While they all praised the role of educators, they also acknowledged the force of substantive challenges for promoting a critical paradigm of environmental education within the faculty as well as in the whole education system and Egyptian society in general. A major finding of the study was that the situation of environmental education in Egyptian universities need closer and immediate attention, and much work remains to transform education and adopt a framework that would integrate environmental education into the core of the educational programs of the faculty. (Abstract shortened by UMI.)

Goueli, Solafa

362

A Primary Master Plan of Gardens’ City- A New City in Egyptian Western Desert (EGCWD  

Directory of Open Access Journals (Sweden)

Full Text Available This paper discusses the primary master plan of the gardens’ city, which is planned to depend on renewable energy. Gardens’ city lies in the Egyptian’s western desert in newly discovered to be developed areas, namely in the new Farafra oases. The general master plan of the city is designed for 117,000 inhabitants, with a final target of settling of 1 Million inhabitants in the oasis. The city has agricultural and industrial based economy, depend on renewable energy (solar and wind energy, and has the first Egyptian college of renewable energy.

Somaya T. Abouelfadl

2013-04-01

363

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.

364

Chemical Evaluation of Irradiated Egyptian Truffles  

International Nuclear Information System (INIS)

The main objective of this study is to investigate and evaluate the chemical compositions of irradiated and non-irradiated truffles (Al-Kamah)as a natural product for human nutrition with concentrated high value protein supplementation. White and brown truffles grow in Egypt and White truffle is identified as Tirmania nivea and brown truffle is identified as Terfezia budiari. Field survey for four truffle seasons including the best habit for production in Sidi Barani and Salum. Chemical composition of fresh and stored truffles indicated some differences between the two studied species. It was found that the white truffle had higher contents of carbohydrate and fat (45.5% and 7.2%), thenbrown truffle (47.5% and 7.5%)respectively, while the browntruffle had the higher contents of proteins, fiber and ash (23.8%, 8%, and 14%) than the white (22.8, 7.5 and 8% resp.). Cysteine was the highest amino acid in white truffle, while isoleucine was the highest one in brown truffle. The major fatty acids appeared in white and brown truffles were linoleic (46 and 28.3%) and Oleic (37.5 and 6209%) of total fatty acids. B-sitosterol was the major sterol in white truffle while stigmasterol in brown truffle (3.4% and 42.6%). The phytochemical screening revealed that numerous compounds are present as saponins, alkaloids, nitrogen bases,sterols, triterpenes and glycosides. The effect of irradiation (2 K Gy) was limited on bio-component. On the other hand, the biochemical components were ier hand, the biochemical components were investigated in irradiated truffle as a method for preservation. Some changes were observed during storage for 2 months. These chages affect the nutrition value of truffle

365

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

366

The kidney in ancient Egyptian medicine: where does it stand?  

Science.gov (United States)

Driven by their deep-seated desire for eternal life in a healthy body, ancient Egyptians were one of the first civilizations to begin collecting and recording medical lore and medicinals that were effective for a healthy body. With its religious origins, medical care was initially provided by priests, but evolved over time into an independent discipline practiced by the swnw (sounou) or physician. What has been preserved of their knowledge in extant medical papyri reflects the great capacity of Egyptians for practical achievement in treating symptoms, but lacks the abstract thought that was to come with the advent of the more rational Greek medicine. The number of prescriptions and incantations for the management of urinary disorders (hematuria, retention, frequency, infection) and dropsy that are mentioned in extant medical papyri likely reflect the frequency with which these problems were encountered. Urine was thought to be formed in the region of the bladder, by a process considered akin to purification. Available studies on preserved mummies indicate that kidney disease was not uncommon. Whether a functional role of the kidney was appreciated at all is highly doubtful. On the other hand, the available evidence suggests an awareness of the kidney (ggt) to which was ascribed a mythological role that may well account for why the kidneys and the heart were the only organs not removed during the process of mummification. PMID:10213808

Salem, M E; Eknoyan, G

1999-01-01

367

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

368

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

369

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

OpenAIRE

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 Pl...

Capretto L; Mazzitelli S; Brognara E; Lampronti I; Carugo D; Hill M; Zhang X; Gambari R; Nastruzzi C

2012-01-01

370

Prevalence of renal tubular dysfunction in beta thalassemia minor in shiraz  

OpenAIRE

 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 o...

Ali Moradi Nakhodcheri; Mohammad Ali Yaghut

2012-01-01

371

Health related quality of life in Middle Eastern children with beta-thalassemia  

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Full Text Available Abstract Background Thalassemia is a common disorder worldwide with a predominant incidence in Mediterranean countries, North Africa, the Middle East, India, Central Asia, and Southeast Asia. Whilst substantial progress has been made towards the improvement of Health related quality of life (HRQoL in western countries, scarce evidence-based data exists on HRQol of thalassemia children and adolescents living in developing countries. Methods We studied 60 thalassemia children from Middle Eastern countries with a median age of 10?years (range 5 to 17?years. HRQoL was assessed with the Pediatric Quality of Life Inventory (PedsQL 4.0. The Questionnaire was completed at baseline by all patients and their parents. The agreement between child-self and parent-proxy HRQoL reports and the relationship between HRQoL profiles and socio-demographic and clinical factors were investigated. Results The scores of parents were generally lower than those of their children for Emotional Functioning (mean 75 vs 85; p?=?0.002, Psychosocial Health Summary (mean 70.3 vs 79.1; p?=?0.015 and the Total Summary Score (mean 74.3 vs 77.7 p?=?0.047. HRQoL was not associated with ferritin levels, hepatomegaly or frequency of transfusions or iron chelation therapy. Multivariate analysis showed that a delayed start of iron chelation had a negative impact on total PedsQL scores of both children (p?=?0.046 and their parents (p?=?0.007. Conclusions The PedsQL 4.0 is a useful tool for the measurement of HRQoL in pediatric thalassemia patients. This study shows that delayed start of iron chelation has a negative impact on children’s HRQoL.

Caocci Giovanni

2012-06-01

372

Health related quality of life in Middle Eastern children with beta-thalassemia  

OpenAIRE

Abstract Background Thalassemia is a common disorder worldwide with a predominant incidence in Mediterranean countries, North Africa, the Middle East, India, Central Asia, and Southeast Asia. Whilst substantial progress has been made towards the improvement of Health related quality of life (HRQoL) in western countries, scarce evidence-based data exists on HRQol of thalassemia children and adolescents living in developing countries. Methods We studied 60 thalass...

Caocci Giovanni; Efficace Fabio; Ciotti Francesca; Roncarolo Maria; Vacca Adriana; Piras Eugenia; Littera Roberto; Markous Raji Suleiman; Collins Gary; Ciceri Fabio; Mandelli Franco; Marktel Sarah; La Nasa Giorgio

2012-01-01

373

Beta-Thalassemia in Iran: New Insight into the Role of Genetic Admixture and Migration  

OpenAIRE

Iran with an area of 1.648 million km2 is located between the Caspian Sea and the Persian Gulf. The Iranian population consists of multiethnic groups that have been influenced by various invasions and migration throughout history. Studies have revealed the presence of more than 47 different ?-globin gene mutations responsible for ?-Thalassemia in Iran. This paper is an attempt to study the origin of ?-Thalassemia mutations in different parts of Iran. Distribution of ?-Thalassemia mutation...

Rezaee, Ali Reza; Banoei, Mohammad Mehdi; Khalili, Elham; Houshmand, Massoud

2012-01-01

374

Homozygous Deletion of Six Olfactory Receptor Genes in a Subset of Individuals with Beta-Thalassemia  

OpenAIRE

Progress in the functional studies of human olfactory receptors has been largely hampered by the lack of a reliable experimental model system. Although transgenic approaches in mice could characterize the function of individual olfactory receptors, the presence of over 300 functional genes in the human genome becomes a daunting task. Thus, the characterization of individuals with a genetic susceptibility to altered olfaction coupled with the absence of particular olfactory receptor genes will...

Ziffle, Jessica; Yang, Wendy; Chehab, Farid F.

2011-01-01

375

Results of minimally toxic nonmyeloablative transplantation in patients with sickle cell anemia and beta-thalassemia.  

Science.gov (United States)

We describe previously transfused patients with sickle cell disease (n = 6) and thalassemia (n = 1) who received nonmyeloablative hematopoietic stem cell transplantation (HCT) to induce stable (full or partial) donor engraftment. Patients were 3 to 20 years (median, 9 years) old. All 7 received pretransplantation fludarabine and 200 cGy of total body irradiation; 2 patients also received horse antithymocyte globulin. Patients received bone marrow (n = 6) or peripheral blood stem cells (n = 1) from HLA-identical siblings, followed by a combination of mycophenolate mofetil and cyclosporine or tacrolimus for postgrafting immunosuppression. After nonmyeloablative HCT, absolute neutrophil counts were transfusions were administered. No grade IV nonhematologic toxicities were observed. One patient experienced grade II acute graft-versus-host disease. Two months after transplantation, 6 of 7 patients had evidence of donor chimerism (range, 25%-85%). Independent of red blood cell transfusions, these 6 patients initially had increased total hemoglobin and hemoglobin A concentrations and a reduction of reticulocytosis and transfusion requirements. There were no complications attributable to sickle cell disease during the interval of transient mixed chimerism. However, after posttransplantation immunosuppression was tapered, there was loss of the donor graft, and all patients experienced autologous hematopoietic recovery and disease recurrence. One patient did not engraft. The duration of transient mixed chimerism ranged from 97 to 441 days after transplantation in patients 4 and 6, respectively, and persisted until immunosuppressive drugs were discontinued after transplantation. In summary, the nonmyeloablative HCT regimens described here produced minimal toxicity and resulted in transient donor engraftment in 6 of 7 patients with hemoglobinopathies. Although complications from the underlying hemoglobinopathies did not occur during the period of mixed chimerism, these results suggest that stable (full or partial) donor engraftment after nonmyeloablative HCT is more difficult to achieve among immunocompetent pediatric patients with hemoglobinopathies than among adults with hematologic malignancies, perhaps in part because recipients may have been sensitized to minor histocompatibility antigens of their donor by preceding blood transfusions. PMID:12931121

Iannone, Robert; Casella, James F; Fuchs, Ephraim J; Chen, Allen R; Jones, Richard J; Woolfrey, Ann; Amylon, Michael; Sullivan, Keith M; Storb, Rainer F; Walters, Mark C

2003-08-01

376

Nature of iron deposits on the cardiac walls in beta-thalassemia by Mössbauer spectroscopy.  

Science.gov (United States)

An identification of the nature and an estimation of the particle size distribution of the iron deposits on thalassemic heart tissue is carried out by variable temperature Mössbauer spectroscopy. Comparison of Mössbauer spectra obtained for the thalassemic heart tissue (I) with those of normal heart tissue (II) and of horse spleen ferritin (III) identifies the iron deposits to be small, superparamagnetic particles of ferritin and/or hemosiderin, two closely related iron storage proteins containing an iron core of (FeOOH)8(FeO x OPO3H2). The dependence of the superparamagnetic relaxation time, tau, of magnetically ordered fine particles on their volume V via the magnetic anisotropy constant K of the material and the condition tau > tauL, the Larmor precession time of the nuclear magnetic moment of 57Fe about an effective magnetic field, for observation of hyperfine structure are used in analyzing the Mössbauer data to yield the particle size distribution. Particle diameters are estimated to be 74 +/- 12 A. PMID:7417508

Kaufman, K S; Papaefthymiou, G C; Frankel, R B; Rosenthal, A

1980-05-22

377

Interference of hemoglobin Hope on beta-thalassemia diagnosis by the capillary electrophoresis Method.  

Science.gov (United States)

The ?-chain hemoglobin (Hb) variants interfere with the diagnosis of ?-thalassemia trait using high-performance liquid chromatography (HPLC) and capillary electrophoresis (CE). We analyzed the effect of Hb Hope, a ?-chain Hb variant frequently found in the Thai population, on ?-thalassemia trait diagnosis. HPLC and CE were used to quantify the level of HbA(2) in 11 whole blood samples containing Hb Hope. The levels of Hb Hope detected by both methods were similar. An elevated HbA(2) level was found in all samples analyzed by the CE method, while 1 was increased when analyzed by HPLC, which was a compound heterozygous of Hb Hope and ?-thalassemia-1 SEA-type deletion. Of 11 samples, 6 had mean corpuscular volumes within the reference range. All samples showed negative results for molecular analysis of ?(0)-thalassemia codon 17, 41/42, and 71/72 mutations and ?-thalassemia 3.5-kb deletion. Therefore, Hb Hope interfered with the diagnosis of ?-thalassemia trait analyzed by CE but not by HPLC. PMID:21685027

Panyasai, Sitthichai; Sukunthamala, Kanyakan; Jaiping, Kanokwan; Wongwiwatthananukit, Sanchai; Singboottra, Panthong; Pornprasert, Sakorn

2011-07-01

378

Oxidative status of valinomycin-resistant normal, beta-thalassemia and sickle red blood cells.  

Science.gov (United States)

Exposure of red blood cells (RBC) to the K+ -ionophore valinomycin (val), causes loss of KCl and water, resulting in cell dehydration, manifested by increased cell density. While almost all normal val-treated RBC dehydrate, in sickle cell anemia (SCA) a portion of the RBC fail to dehydrate and maintain a light density, indicating the existence of val-resistant (val-res) RBC. In thalassemia and sickle cell disease (SCD), although the primary lesion is in the globin genes, damage to the RBC is partly mediated by oxidative stress. We previously showed that such RBC are under oxidative stress, having more reactive oxygen species (ROS) and less reduced glutathione than normal RBC. We now report a relationship between the phenomenon of val-res and the RBC oxidative status: Treatment with oxidants that increase ROS, also increased the frequency of val-res cells. Val-res cells had higher oxidative status than other RBC in the sample. Similar to SCA, thalassemic blood has more val-res cells than does normal blood. Val-res cells in thalassemic and sickle blood showed a higher oxidative status than normal val-res cells. Thus, oxidative stress might be involved in generation of val-res cells. Further studies are required to elucidate the origin and significance of these cells. PMID:16580776

Amer, Johnny; Etzion, Zipora; Bookchin, Robert M; Fibach, Eitan

2006-05-01

379

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

380

E BETA THALASSEMIA COMPLICATED BY STEROID RESPONSIVE AUTO IMMUNE HEMOLYTIC ANEMIA DUE TO SYSTEMIC LUPUS ERYTHEMATOSUS  

OpenAIRE

Thalassemia patients may have superadded acquired hemolytic diseases. This may cause a sudden increase in transfusion requirement. However, such acquired hemolysis is very difficult to diagnose in presence of Thalassemia. We here report a case of thalassemia complicated by hemolytic anemia secondary to SLE. This is probably the first report of this association from India. This combination of two hemolytic diseases is potentially fatal. The autoimmune hemolytic component responded well to immu...

Paul Rudrajit; Seal Koelina; Das Suvabrata; Chakravarti Hridish Narayan; Bhattacharya Raja

2013-01-01

381

E BETA THALASSEMIA COMPLICATED BY STEROID RESPONSIVE AUTO IMMUNE HEMOLYTIC ANEMIA DUE TO SYSTEMIC LUPUS ERYTHEMATOSUS  

Directory of Open Access Journals (Sweden)

Full Text Available Thalassemia patients may have superadded acquired hemolytic diseases. This may cause a sudden increase in transfusion requirement. However, such acquired hemolysis is very difficult to diagnose in presence of Thalassemia. We here report a case of thalassemia complicated by hemolytic anemia secondary to SLE. This is probably the first report of this association from India. This combination of two hemolytic diseases is potentially fatal. The autoimmune hemolytic component responded well to immunosuppressive therapy and there was significant reduction in transfusion requirement.

Paul Rudrajit

2013-04-01

382

The molecular basis of beta-thalassemia intermedia in southern China: genotypic heterogeneity and phenotypic diversity  

OpenAIRE

Abstract Background The clinical syndrome of thalassemia intermedia (TI) results from the ?-globin genotypes in combination with factors to produce fetal haemoglobin (HbF) and/or co-inheritance of ?-thalassemia. However, very little is currently known of the molecular basis of Chinese TI patients. Methods We systematically analyzed and characterized ?-globin genotypes, ?-thalassemia determinants, and known primary genetic modifiers linked to the production o...

Sun Manna; Zhou Tianhong; Li Liyan; Cai Ren; Shang Xuan; Zhang Xinhua; Chen Wanqun; Xiong Fu; Xu Xiangmin

2010-01-01

383

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

384

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.)

385

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

386

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

Directory of Open Access Journals (Sweden)

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

387

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

Scientific Electronic Library Online (English)

Full Text Available SciELO South Africa | Language: English Abstract in english 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.

388

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

389

Genetic Analysis of Rheumatic Fever among Egyptian Families: Consanguinity Pattern, Segregation Analysis and Blood Group Association  

OpenAIRE

To assess genetic background of Rheumatic Fever (RF) among Egyptian families and to test for association to blood group allelic phenotypes. This study was done on 30 Egyptian rheumatic families of which 10 were mutiplex; enrolled from Pediatric Cardiology Clinic, Mansoura University Hospital. Subjects included 30 probands and 1142 relatives of different degrees; they were classified clinically into 46 cases with RF, 136 subjects with recurrent Upper Respiratory Infection (URTI) and/or arthral...

Settin, Ahmad A.; Al-haggar, Mohammad S.; Hala El Marsafawy; Amal Abd Alkader A.; El Baz, Rizk A.; Mansour, Ahmad K.; Al-kasem, Rabab A.; Eman Alam

2006-01-01

390

Prune belly syndrome in an Egyptian infant with Down syndrome: A case report  

OpenAIRE

Abstract Introduction Prune belly syndrome is a rare congenital anomaly of uncertain aetiology almost exclusive to males. The association between prune belly syndrome and Down syndrome is very rare. Case presentation A 4-month-old Egyptian boy was admitted to our institute for management of acute bronchiolitis. He was born at full term by normal vaginal delivery. His mother, a 42-year-Egyptian villager with six other children, had no antenatal or prenatal care. ...

Metwalley Kotb A; Farghalley Hekma S; Abd-Elsayed Alaa A

2008-01-01

391

Consumptive hypothyroidism in an Egyptian baby with benign neonatal hemangiomatosis: a case report  

OpenAIRE

Abstract Introduction Benign neonatal hemangiomatosis is a condition in which multiple cutaneous hemangiomas appear at birth or shortly thereafter; visceral complications are absent. Here, we report a case of a consumption hypothyroidism in an Egyptian baby with benign neonatal hemangiomatosis. Case presentation An 8-month-old Egyptian boy with benign neonatal hemangiomatosis was referred to our institution for evaluation of developmental delay. Initial examinat...

Metwalley Kotb Abbass; Farghaly Hekma Saad

2013-01-01

392

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

393

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. ...

Bahador Sadeghi; Vahid Jalali

2013-01-01

394

BENCHMARKING THE EGYPTIAN MEDICAL TOURISM SECTOR AGAINST INTERNATIONAL BEST PRACTICES: AN EXPLORATORY STUDY  

OpenAIRE

This paper argues that any compatible strategy for the development of medical tourism at a developing nation should be based primarily on a comprehensive benchmarking study. It has employed the benchmarking phase of a national project for the development of an Egyptian medical tourism strategy to showcase significance of such benchmarking implications. The benchmarking phase of the Egyptian project has used two main data sets to reach reliable findings: a series of best practice destinations ...

Helmy, Eman M.

2011-01-01

395

Isolation and Characterization of Highly Potent Mosquitocidal Bacilli from Egyptian Environment  

OpenAIRE

A number of 359 Bacilli isolates were obtained from soil samples collected from different Egyptian governorates. Five Egyptian isolates identified as Lysinibacillus sphaericus yielded highly potent mosquitocidal toxicity. These isolates (Ls 9B24, Ls 3B3, Ls 2LB10, Ls 4N9 and Ls 4LB19) were obtained from soils of Alexandria, Kafr El- Sheikh, New valley and El- Gharbeya governorates respectively. The values of LC50 and LC90 of these isolates showed that the most act...

El-bendary, Magda A.; Gawdat, Noha A.; El-tayeb, Osama M.; Amin, Magdi A.; Foda, Mohamed S.

2013-01-01

396

Cardiac MRI and Iron Overload Cardiomyopathy in Thalassemia Major: A Case Report  

Science.gov (United States)

Introduction: Heart failure is the leading cause of death in patients with Thalassemia major and primarily results from transfusional iron overload. It is essential to assess myocardial iron load. Previous studies have shown that neither serum ferritin nor liver iron concentration gives a reliable measure of cardiac iron accumulation. Cardiac T2* MRI technique offers noninvasive measurement of myocardial iron load. Case Report: A 39-year-old man with a past medical history significant for beta thalassemia major requiring blood transfusions every three-weeks and on iron chelation, presented with a cough, high fevers, and chills. He was subsequently found to have community acquired pneumonia and was treated with ceftriaxone and doxycycline. His hospital course was significant for episodes of atrial fibrillation and non-sustained ventricular tachycardia. An echocardiogram showed normal left ventricular function with an ejection fraction of 70%, which hadn't changed from 2011. Although, the patient didn't have symptoms of heart failure, he likely had ventricular dysfunction that could be masked by the basal high cardiac output which can be seen in patients with chronic anemia and we decided to start the patient on sotalol 80 mg twice a day. The MRI software for multiecho T2* measurement was installed at Queens Medical Center in September 2011 and we were able to obtain images for our patient at that time. His myocardial T2* value was estimated to be 9 milliseconds which suggests an increased risk for the development of future cardiac arrhythmias and heart failure. The repeat cardiac MRI images after discharge showed 8 milliseconds which suggested an interval worsening of the iron deposition within the myocardium. Discussion: We were able to keep track of the progression of his iron cardiomyopathy, and start additional treatment. The patient continues to be followed by a hematologist for management of his hemochromatosis and a cardiologist for his infiltrative heart disease both resulting from his need for chronic blood transfusions. In patients with iron overload cardiomyopathy, their systolic function is preserved until a very late stage as iron deposition begins within the epicardium and extends to the myocardium. This case illustrates the importance of assessing cardiac iron content utilizing cardiac MRI as it is less invasive than cardiac biopsy and may show earlier involvement than echocardiogram.

Azuma, Steven

2014-01-01

397

Fate and metabolism of radiolabelled dicrotophos in Egyptian lactating cows  

International Nuclear Information System (INIS)

The present study was initiated to determine the amount of dicrotophos and its metabolites which might appear in milk and meat of Egyptian lactating cows following dicrotophos treatment.14 C-alkyl dicrotophos was synthesised and its metabolic fate in two cows was investigated. For each cow, two equal dermal applications with 2-week interval were made. One cow was sacrificed 24 hr. after the second application, and the second animal after two weeks later. Paper chromatographic analysis of milk showed the presence of dicrotophos and two of its metabolites. Insecticide residues in the different organs were found in low levels. Treatment produced no negative influence on the state of health or milk production of the cows.1 fig.,2 tab

398

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

399

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

400

Fallout Radioactivity in Some Egyptian Lakes Bottom Sediments  

International Nuclear Information System (INIS)

As a part of the Egyptian environmental radioactivity monitoring program, the fallout radioactivity levels in Qarun, Bardawill and Ed ku lakes bottom sediments have been measured. The specific activities of 137Cs were measured using gamma ray spectrometer based on Hyper pure germanium detector. The specific activities of plutonium isotopes(238Pu, 239+240Pu and 241Pu) were measured using alpha spectrometry based on surface battier detectors and liquid scintillation spectrometry after radiochemical separation. The activity ratios 239+240Pu/137Cs, 239+240Pu/241Pu, and 238Pu/ 239+240Pu were calculated. The results seemed to confirm that fallout radioactivity is mainly due to nuclear weapons testing fallout

401

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

402

The Reasons social media contributed to 2011 Egyptian Revolution  

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Full Text Available In recent years, social media has become very significant for social networking. In the past, itsmain use was personal, but nowadays, its becoming part of all facets of our lives, social andpolitical. In the first quarter of 2011, the Middle East has witnessed many popular uprisings thathave yet to reach an end. While these uprisings have often been termed “FacebookRevolutions” or “Twitter Revolutions”, there are many ambiguities as to the extent to whichsocial media affected these movements. In this paper we discuss the role of social media andits impact on the 2011 Egyptian revolution. Though the reasons for the uprising were manifold,we will focus on how social media facilitated and accelerated the movement.

Rabia Minatullah Sohail, Nadine Chebib

2011-10-01

403

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)

404

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

405

Studies on natural radioactivity of some egyptian building materials  

International Nuclear Information System (INIS)

Using high-resolution y-rays spectrometry, the natural radioactivity of 14 samples of natural and o manufactured Egyptian building materials have been investigated. The samples were collected from local market and construction sites. From the measured gamma-ray spectra, specific activities were determined. The radium equivalent activity in each sample was estimated. Radiological evaluations of these materials indicate that all materials meet the external gamma-ray dose limitation. Calculation of concentration indices by assuming a Markkanen room model is constructed from these materials, to find the excess gamma-ray dose taken over that received from the outdoors. The Austrian Standard ONORM S 5200 is used in testing the building materials

406

Transfusion Transmitted Virus (TTV Infection in Polytransfused Egyptian Thalassemic Children  

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Full Text Available Transfusion Transmitted Virus (TTV is a naked, single stranded DNA virus, which has been discovered in the serum of a patient with post-transfusion hepatitis of unknown etiology. TTV infection is widespread in the general population, however, the mode of its transmission is still unclear. This study was conducted to search for TTV-DNA positivity rates and to evaluate its clinical significance in both Egyptian polytransfused thalassemic children and healthy children. Serum samples from 33 polytransfused thalassemic children and 30 healthy children were studied for the presence of TTV-DNA by nested PCR using primer sets generated from the untranslated region (UTR of the viral genome. TTV-DNA was found positive in 39.4% of polytransfused children versus 40% of healthy children, the high rate of viremia observed in healthy children indicates that transfusion route is not the only mode of TTV spread. 60.6 % of the polytransfused group were HCV positive. Fifty five percent of HCV positive cases showed TTV-DNA positivity, suggesting common route of infection. No significant difference between ALT and AST values in TTV-DNA positive polytransfused children compared to negative cases. ALT and AST values were statistically significantly higher in HCV positive patients compared to negative cases. On the other hand no significant difference was observed of ALT and AST values in isolated HCV infection versus TTV and HCV co-infection. Thus although TTV infection is quite common among Egyptian, no convincing evidence was found to support its involvement in the pathogenesis of hepatitis in children, more data are still needed for a better understanding of the natural history of TTV infection.

N.M. Abdalla

2006-01-01

407

Angiographic characteristics of central serous chorioretinopathy in an Egyptian population  

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Full Text Available AIM: To describe and analyze the demographic characteristics and to determine the angiographic features of acute central serous chorioretinopathy(CSCR in an Egyptian population. METHODS: A series of consecutive patients presenting with acute idiopathic CSCR to Mansoura Ophthalmology Center Mansoura University who underwent fluorescein angiography (FA within a 3-year-period (between January 1, 2007 and December 31, 2009 was retrospectively studied. Patient demographics and angiographic features were studied. Results were compared to those of other Western and Asian populations. RESULTS: Fluorescein angiograms of 86 patients were analyzed. 91% were males. The age range of patients was 24 - 49 years, with a mean age of (38±6 years. The right eye was the presenting eye in 47% of patients. Eighty-seven percent of eyes showed delayed choroidal filling. Thirty-five percent of patients had more than one point of leakage. The macula was the most common site of fluorescein leakage seen in 79% of patients. Peripheral leakage was seen in 14% of patients while peripapillary leakage was seen in 12% of patients. The inkblot leakage pattern was found in 53% of patients. The presenting eye had RPE atrophic changes in 84% of cases. The other eye was assessed in 44 patients (51%. Fifty-five percent of them had signs of RPE atrophic changes. CONCLUSION: In the Egyptian population, CSCR is seen at younger age with higher male-to-female ratio and more frequent smokestack leaks than other populations. Despite younger age group, this series of patient showed higher frequency of bilateral and multifocal disease compared to other studies. Roles of psychological stress and choroidal ischemia in pathogenesis of CSCR need further evaluation.

Maha M Shahin

2013-06-01

408

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

409

Early results of sarcomeric gene screening from the Egyptian National BA-HCM Program.  

Science.gov (United States)

The present study comprised sarcomeric genotyping of the three most commonly involved sarcomeric genes: MYBPC3, MYH7, and TNNT2 in 192 unrelated Egyptian hypertrophic cardiomyopathy (HCM) index patients. Mutations were detected in 40 % of cases. Presence of positive family history was significantly (p=0.002) associated with a higher genetic positive yield (49/78, 62.8 %). The majority of the detected mutations in the three sarcomeric genes were novel (40/62, 65 %) and mostly private (47/62, 77 %). Single nucleotide substitution was the most frequently detected mutation type (51/62, 82 %). Over three quarters of these substitutions (21/27, 78 %) involved CpG dinucleotide sites and resulted from C>T or G>A transition in the three analyzed genes, highlighting the significance of CpG high mutability within the sarcomeric genes examined. This study could aid in global comparative studies in different ethnic populations and constitutes an important step in the evolution of the integrated clinical, translational, and basic science HCM program. PMID:23233322

Kassem, Heba Sh; Azer, Remon S; Saber-Ayad, Maha; Ayad, Maha S; Moharem-Elgamal, Sarah; Magdy, Gehan; Elguindy, Ahmed; Cecchi, Franco; Olivotto, Iacopo; Yacoub, Magdi H

2013-02-01

410

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

Science.gov (United States)

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. PMID:23538996

Ahmed, Walaa H; Furusyo, Norihiro; Zaky, Saad; Sharaf Eldin, Abeer; Aboalam, Hany; Ogawa, Eiichi; Murata, Masayuki; Hayashi, Jun

2013-01-01

411

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

Directory of Open Access Journals (Sweden)

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

412

Return Anomalies “Disposition Effect and Momentum”: Evidence from the Egyptian Stock Market  

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Full Text Available Purpose: The persistence of momentum in stock returns across both developed and emerging markets and the challenges that it poses against the Efficient Market Hypothesis created a need to explain its existence. Grinblatt and Han (2005 formulated a model to explain momentum using a well documented behavioral bias which is the Disposition effect. The focus of this paper is to analyze whether disposition effect drives momentum in the Egyptian stock market as one of the growing emerging markets that faces a considerable lack in behavioral studies. Design/methodology/approach: The study is quantitative in nature studying whether disposition effect drives momentum using a sample of 48 companies through the time period 2004–2010. The relation between disposition effect and momentum will be analyzed empirically using Fama Macbeth cross-sectional regression. Findings: Results show that there is no momentum in stock returns in the Egyptian stock market. In addition they show that disposition effect does not drive momentum in the Egyptian stock market as there is no significant relation between expected return and capital gain overhang. The results reveal useful insights about the Egyptian stock market that can be of beneficial use for both practitioners and academics. Research limitations/implications: Limited number of active companies in the Egyptian stock market as well as the limited available historical data poses some restrictions in the implementation of Fama Macbeth regression and the calculation of reference price. In addition analyzing the profitability of momentum strategies across different market states may be required to provide complete picture about momentum in the market. Practical implications: Relative strength strategies do not earn abnormal return in the Egyptian stock market, so practitioners are not advised to follow such strategies. In addition more advanced market mechanisms should be applied in the Egyptian stock market to improve its efficiency as well as increase the speed of information dissemination in the prices. Originality/value: Detailed analysis of literature review reveals a significant gap in academic studies about the Egyptian stock market. This paper aims to fill this gap by analyzing whether there is momentum in stock returns and whether disposition effect drives momentum in the Egyptian stock market that differs from other markets where Grinblatt and Han (2005 has been previously applied and hence this provides an out of sample test of the model.

Ahmed M. Sakr

2014-01-01

413

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)

414

Mental health in the Middle East: an Egyptian perspective.  

Science.gov (United States)

This article introduces the reader to mental health in the Middle East with an Egyptian perspective, from the Pharaonic era through the Islamic Renaissance, up until the current state. During Pharaonic times, mental illness was not known as such, as there was no separator between Soma and Psyche. Actually, mental disorders were described as symptoms of the heart and uterine diseases, as stated in Eber's and Kahoun's papyri. In spite of the mystical culture, mental disorders were attributed and treated on a somatic basis. In the Islamic era, mental patients were never subjected to any torture or maltreatment because of the inherited belief that they may be possessed by a good Moslem genie. The first mental hospital in Europe was located in Spain, following the Arab invasion, and from then on it propagated to other European countries. The 14th century Kalawoon Hospital in Cairo had four departments, including medicine, surgery, ophthalmology, and mental disorders. Six centuries earlier, psychiatry in general hospitals was recognized in Europe. The influence of Avicenna and Elrazi and their contributions to European medicine is well-known. This article discusses further the current state of the mental health services in Egypt and the transcultural studies of the prevalence and phenomenology of anxiety, schizophrenia, depression, suicide, conversion, and obsessive compulsive disorders. An outline of psychiatric disorders in children is discussed. The problem of drug abuse is also addressed, especially that in Egypt after 1983, where drugs like heroine replaced the common habit of hashish. PMID:10547710

Okasha, A

1999-12-01

415

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

416

Efficiency of diagnostic biomarkers among colonic schistosomiasis Egyptian patients  

Scientific Electronic Library Online (English)

Full Text Available SciELO Brazil | Language: English Abstract in english 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

417

Peanut sensitization in a group of allergic Egyptian children  

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Full Text Available Abstract Background There are no published data on peanut sensitization in Egypt and the problem of peanut allergy seems underestimated. We sought to screen for peanut sensitization in a group of atopic Egyptian children in relation to their phenotypic manifestations. Methods We consecutively enrolled 100 allergic children; 2-10 years old (mean 6.5 yr. The study measurements included clinical evaluation for site of allergy, possible precipitating factors, consumption of peanuts (starting age and last consumption, duration of breast feeding, current treatment, and family history of allergy as well as skin prick testing with a commercial peanut extract, and serum peanut specific and total IgE estimation. Children who were found sensitized to peanuts were subjected to an open oral peanut challenge test taking all necessary precautions. Results Seven subjects (7% were sensitized and three out of six of them had positive oral challenge denoting allergy to peanuts. The sensitization rates did not vary significantly with gender, age, family history of allergy, breast feeding duration, clinical form of allergy, serum total IgE, or absolute eosinophil count. All peanut sensitive subjects had skin with or without respiratory allergy. Conclusions Peanut allergy does not seem to be rare in atopic children in Egypt. Skin prick and specific IgE testing are effective screening tools to determine candidates for peanut oral challenging. Wider scale multicenter population-based studies are needed to assess the prevalence of peanut allergy and its clinical correlates in our country.

Hossny Elham

2011-05-01

418

Efficiency of diagnostic biomarkers among colonic schistosomiasis Egyptian patients  

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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 biochemical 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

2011-05-01

419

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

420

Detailed gravimetric geoid for the Egyptian south-western desert  

Science.gov (United States)

Different gravimetric geoid solutions were carried out for the south-western Egyptian desert. A set of 2682 measured gravity stations were available for this investigation. The discrete gravity data are reduced to the geoid through the free-air reduction. Also terrain reduction is performed using a set of fine 30? × 30? and coarse 3? × 3? Digital Height Models for the test area. Different earth's geopotential models were used for the removing of the reference field. The geoid computations were performed in the space domain using the discrete Stokes integration technique and in the spectral domain using the spherical 2-D FFT technique. For both techniques, the reduced gravity anomalies are gridded on a 3? × 3? geographical grid using the least-squares interpolation technique with local covariance functions. A broad comparison among the different geoid solutions is made. The effect of the inner zone densification is in the order of about 2 cm. The effect of the spherical 2-D FFT approximation is mostly less than 1 cm.

Abd-Elmotaal, H. A.

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