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Sample records for egyptian beta-thalassemia major

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

    Laila M. Sherief

    2014-01-01

    Full Text Available 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 children. Serum levels of vitamin A, E, C, tHcy, and MMA were estimated by high pressure liquid chromatography while serum levels of folic acid and B12 were estimated by thin layer chromatography. Serum zinc, copper, and selenium were determined by atomic absorption spectrometry. Results. There was a significant decrease of vitamins A, C, E, and B12 and trace elements zinc, copper, and selenium in thalassemic patients as compared to controls. tHcy and MMA were significantly elevated in patients. No significant correlations were found between the serum levels of the studied vitamins and trace elements as regards age, frequency of transfusion, duration of transfusion, and serum ferritin. Conclusion. The level of various nutritional biomarkers (vitamins A, C, E, and B12 and trace elements zinc, copper, selenium was reduced in chronically transfused Egyptian thalassemic patient. These patients should have periodic nutritional evaluation and supplementation. Multicenter studies are highly recommended.

  2. Evaluation of pulmonary function in beta-thalassemia major patients

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

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

    Hatami, Gissou; Motamed, Niloofar

    2014-01-01

    Background: Beta thalassemia major has a considerable impact on quality of life. The purpose of this study was to determine the life satisfaction of beta-thalassemic children and adolescents compared to healthy controls. Methods: This research, conducted in 2009, was a controlled, cross-sectional study in which beta-thalassemic patients, who were being followed-up by the Thalassemic Center in Bushehr, a city in southern Iran, were compared with a healthy control group. The Multi-dimensional S...

  4. A Study of Leptin Serum Concentrations in Patients with Major Beta-Thalassemia

    I Shahramian; Akhlaghi, E; A. Ramezani; A. Rezaee; Noori, N; Sharafi, E

    2013-01-01

    Background The aim of this study was to evaluate leptin serum levels in patients with major beta thalassemia which was also associated with their ferritin serum levels. Materials and Methods This case-control study was performed on 90 children -6 months to 16 years old, in Zabol, Amir- al- Momenin Hospital. Patients were divided in two groups and were matched in age and sex. All Children were examined and those eligible children who had not known heart disease, iron deficiency anemia, kidney ...

  5. Efficacy of Carvedilol in Patients with Dilated Cardiomyopathy due to Beta-Thalassemia Major; a Double-Blind Randomized Controlled Trial

    Gholam-Hossein Ajami; Hamid Amoozgar; Mohammad Borzouee; Mehran Karimi; Farah Piravian; Afsaneh Ashrafi; Zahra Kheirandish

    2010-01-01

    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. A...

  6. Beta Thalassemia

    ... South Asian (Indian, Pakistani, etc.), Southeast Asian and Chinese descent. 1 Beta Thalassemia ßß Normal beta globin ... then there is a 25% chance with each pregnancy that their child will inherit two abnormal beta ...

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

    Mehrabani K; Mir Ahmadian M; Mohammad K

    1996-01-01

    Beta Thalassemia major patients receive repeated transfusions in order to compensate anemia and use desferal to remove iron overload. Comparing immunoglobulins and complement components in the serum of these patients with normal range shows a significant increase (P<0.001) in IgG, IgA and IgM and a decrease in C3c and C4. The regression analysis confirms a relation between the numbers of transfusions and the mentioned immunological factors that means increasing the number of transfusio...

  8. [Illness knowledge, social support and self care behavior in adolescents with beta-thalassemia major].

    Yang, H C; Chen, Y C; Mao, H C; Lin, K H

    2001-04-01

    The purpose of this study was to explore the relationships among illness knowledge, social support and self-care behavior in adolescents with beta-thalassemia major. The subjects were 58 beta-thalassemia major adolescents recruited from the pediatric hematology outpatient departments of three hospitals in North Taiwan. All data were analyzed by descriptive statistics, one-way ANOVA, t-test, Pearson correlation, and stepwise multiple regression. The results showed that: (1) Scoring in illness knowledge, the best was treatment knowledge, and the worst was the knowledge of complications and symptoms. (2) The result for social support indicated that the family was the major source of support, and that classmates/friends provided the least support for thalassemic adolescents. (3) Scoring in self care behavior, the best was the medical and chelate therapy, and the worst was the management of problems and coping. (4) Individual characteristic differences, including sex, level of education, length of illness, complications of thalassemia, hospitalization due to thalassemic complications and receiving other treatments were correlated with their illness knowledge, social support or self-care behavior. (5) Illness knowledge, social support which were positively correlated with self-care behavior. (6) 50.0% of the variance in self-care behavior can be explained by emotional support from family, general knowledge of thalassemia, treatment knowledge and appraisal support from family. These findings could provide referential material for nursing research and nursing practice. PMID:11548457

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

    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)

  10. Hypoparathyroidism and intracerebral calcification in patients with beta-thalassemia major

    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

    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.

  11. Hypoparathyroidism and intracerebral calcification in patients with beta-thalassemia major

    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.

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

    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 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)

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

    Mehrabani K

    1996-06-01

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

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

    S. H. Hosseini, M.D.

    2007-09-01

    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

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

    Seyed-Taghi Heydari

    2010-09-01

    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.

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

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

    2010-01-01

    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. PMID:23056722

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

    Morteza Ashrafi

    2012-04-01

    Full Text Available Objective: Beta-thalassemia major (β-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 (χ2 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.

  18. BETA THALASSEMIA MAJOR IN A DEVELOPING COUNTRY: EPIDEMIOLOGICAL, CLINICAL AND EVOLUTIONARY ASPECT

    Mohamed Bejaoui

    2013-01-01

    Full Text Available Beta-thalassemia major (TM remains to be one of the major health problems particularly in developing countries. Tunisia is a part of the Mediterranean countries mostly affected by this disease which is highly concentrated in small towns in families with low-income earners. The main objectives of this study are to provide a description of the demographic, clinical features and transfusion-related complications in patients with TM living in Tunisia. A standardized questionnaire was sent to clinicians throughout 33 different medical institutions caring for thalassemic patients. 391 transfusion dependant thalassemic patients with a median age of 10.7 years (range 3 months- 31 years were included in the study.The majority were originated from the north west of the country .A moderate overload between 1501 and 2500ng/ml was found in 61patients, while 81 patients (26.9% had ferritin level more than 2500 ng/ml and greater than 5000ng/ml in 21 patients (6.9%. 51 patients died from complications related to their disease. Heart failure was the main cause of death. The incidence of cardiac, endocrine, and infectious complications will be reviewed. Preventive measures such as health education, carrier screening and premarital screening remain the best ways for lowering the incidence of these diseases, which might be reflected in financial saving, social benefits and health benefits.

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

    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

    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.)

  20. Beta thalassemia major: The effect of age on glomerular filtration rate

    Majid Malaki

    2011-01-01

    Full Text Available Thalassemia is a common hereditary hemoglobinopathy disorder that affects many organs in the body. Estimation of kidney function is important, as it is the vital organ that plays the major role in the elimination of accumulated iron as well as the chelating drugs that have to be used as therapy. Sixty- three patients aged 1-29 years, with a mean ± SD of 14 ± 6.7 years, affected with beta- thalassemia major in Tabriz Children′s Hospital were evaluated for their renal function on the basis of their age, serum iron, serum ferritin and serum creatinine levels along with two methods of estimating glomerular filtration rate (GFR; by Schwartz method for those under 18 years old and using Modification of Diet in Renal Disease (MDRD formula for those who were 18 years and above. Elevation of serum creatinine denoting renal dysfunction was not seen in our patients, but hyperfiltration was a common finding. An increasing GFR was observed, which corresponded to age, but no relationships were seen between serum iron, serum ferritin, regular blood transfusion, chelating therapy to GFR.

  1. Prevalence of occult hepatitis C virus infection in Iranian patients with beta thalassemia major.

    Bastani, Mohammad-Navid; Bokharaei-Salim, Farah; Keyvani, Hossein; Esghaei, Maryam; Monavari, Seyed Hamidreza; Ebrahimi, Mojtaba; Garshasebi, Saba; Fakhim, Shahin

    2016-07-01

    Occult hepatitis C virus infection (OCI) is a new pathological form of chronic hepatitis virus (HCV) infection characterized by the presence of HCV RNA in liver biopsy and/or peripheral blood mononuclear cell (PBMC) specimens and the absence of HCV RNA and anti-HCV antibodies (Abs) in plasma samples. β-thalassemia major is a hereditary recessive blood disease with deficiency in the hemoglobin beta chain. Thalassemic patients need blood transfusion therapy; repeated blood transfusion increases the risk of viral blood-borne infection. The aim of this study was to determine the prevalence of OCI in Iranian patients with β-thalassemia major. From February 2015 to November 2015, a total of 147 Iranian patients with β-thalassemia major were enrolled in this cross-sectional study. After extraction of viral RNA from the plasma and PBMC samples, HCV genomic RNA in the specimens was amplified by RT-nested PCR using primers from the 5'-UTR. The HCV genotypes of the positive specimens were tested using the RFLP assay. To confirm the HCV genotypes, the 5'-UTR fragment was amplified and cloned into the pJET1.2/blunt cloning vector and then sequenced. Out of 147 patients, 106 (72.1 %) were negative for anti-HCV Abs and HCV RNA. HCV RNA was found in PBMC specimens of six (5.7 %) patients, from a total of 106 patients with undetectable plasma HCV RNA and anti-HCV Abs. Therefore, six out of 106 patients had OCI. HCV genotyping revealed that three patients were infected with HCV subtype 1b, two patients were infected with HCV subtype 3a, and one patient was infected with HCV subtype 1a. These results revealed that Iranian patients with beta-thalassemia major might have OCI. Therefore, it seems that the design of a study to identify this infection in patients with β-thalassemia major would provide valuable information. PMID:27132015

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

    Murtadha Al-Khabori

    2013-03-01

    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.

  3. Prenatal diagnosis for beta-thalassemia major in the Iranian Province of Hormozgan.

    Nikuei, Pooneh; Hadavi, Valeh; Rajaei, Minoo; Saberi, Mozhgan; Hajizade, Fozieh; Najmabadi, Hossein

    2008-01-01

    beta-Thalassemias are a group of heterogenous recessive disorders common in many parts of the world. Despite the great advances in the treatment of thalassemia, there is so far no cure, but perhaps bone marrow transplantation (BMT) is a possibility. Prevention, using prenatal diagnosis and selective abortion in the cases where the fetus is found to be affected, should be considered as a sensible alternative. During the past 5 years, 112 couples have been referred to our Center for detection of their beta-thalassemia (beta-thal) carrier status. In this group, common and rare mutations were detected. Of these, 106 couples (94.6%) came for counseling during pregnancy and six (5.4%) came before becoming pregnant. Prenatal diagnosis was performed for the 106 couples at risk. Fetal DNA was obtained from both chorionic villus sampling (CVS) (99) and amniotic fluid (7). Using reverse hybridization, 64 (60.4%) were found to be heterozygous for a beta-thal mutation and 24 (22.6%) were normal. Eighteen (17.0%) were found to carry an affected fetus and these pregnancies were terminated. PMID:19065331

  4. Quality of life, clinical effectiveness, and satisfaction in patients with beta thalassemia major and sickle cell anemia receiving deferasirox chelation therapy

    Senol, Sefika Pinar; Tiftik, Eyup Naci; Unal, Selma; Akdeniz, Aydan; Tasdelen, Bahar; Tunctan, Bahar

    2016-01-01

    Objectives: There is a need to remove excess iron with iron chelation therapy (ICT) to avoid the serious clinical sequelae associated with iron overload in patients with beta thalassemia major (BTM) and sickle cell anemia (SCA). Due to the effects of the diseases and their treatments, ICT is still a major reason for unsatisfactory compliance. The aim of this single-center observational study was to evaluate the quality of life, clinical effectiveness, and satisfaction in pediatric and adult p...

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

    Nargesbeygom Mirbehbahani

    2014-01-01

    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.

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

    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

    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.)

  7. Beta-thalassemia

    Origa Raffaella

    2010-05-01

    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

  8. Genetics Home Reference: beta thalassemia

    ... for Disease Control and Prevention Centre for Genetics Education (Australia) Cold Spring Harbor Laboratory: Your Genes Your Health Disease InfoSearch: Beta Thalassemia Genomics Education Programme (UK) MalaCards: dominant beta-thalassemia Merck Manual ...

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

    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

    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.)

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

    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.)

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

    Alan COHLER

    2009-01-01

    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.

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

    Ali Ahmadzadeh

    2011-01-01

    Full Text Available To evaluate the prevalence of renal tubular dysfunction in children with β-thalassemia (β-T major, we studied the glomerular and tubular function in 140 children with β-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-β-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 β-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.

  13. Deficient activity of the alternative pathway of complement in beta thalassemia major.

    Corry, J M; Marshall, W C; Guthrie, L A; Peerless, A G; Johnston, R B

    1981-06-01

    Patients with thalassemia major suffer frequent and serious infections, especially after splenectomy. To explore the basis for this susceptibility, we examined activity of the complement system in sera from 24 patients. All sera had normal or increased activity of the classic complement pathway. However, six of the 24 (three with and three without splenectomy) had abnormal alternative pathway function, and mean alternative pathway activity was significantly decreased in both splenectomized and nonsplenectomized patients. Mean concentrations of C3, factor B, properdin, and immunoglobulins were normal. Defective alternative pathway function, especially in conjunction with asplenia, could contribute to the propensity to infection that exists in thalassemia. PMID:6908998

  14. Alternative pathway activation in sickle cell disease and beta-thalassemia major.

    deCiutiis, A C; Peterson, C M; Polley, M J; Metakis, L J

    1978-07-01

    Total hemolytic complement activity (CH50), immuno-electrophoretic conversion of Factor B (C3PA), and of C3 were studied in 16 patients with sickle cell disease in a steady state, eight patients in crisis, and ten patients with β-thalassemia major anemia maintained on a constant transfusion regimen. Patients with sickle cell disease in a steady state have moderatley 56 (percent) depressed conversion of Factor B in addition to markedly decreased conversion of C3 in four of ten patients. One of the three sickle cell patients and two of the four thalassemia patients with low C3 conversion levels have died subsequent to the studies. The combination of chronically decreased Factor B conversion in the face of markedly decreased C3 conversion may make these patients occasionally vulnerable to overwhelming infection analagous to the situation seen in postsplenectomy cases. PMID:702579

  15. Antioxidant status in beta thalassemia major: A single-center study

    Faiza Waseem

    2011-01-01

    Full Text Available Background: Homozygous β thalassemia may lead to a marked reduction or absence of normal β chain production and accumulation of unpaired alpha-globin chains. A crucial component in the oxidant susceptibility of the thalassemic RBC is the release of heme and iron from the excessive, unpaired α-globin chains. This release can initiate self-amplifying redox reactions, which deplete the cellular reduction potential (e.g., GSH, oxidize additional hemoglobin and accelerate RBC destruction. Furthermore, β-thalassemia patients are under continuous blood transfusion, which, although life-saving, leads to an iron overload with a resultant increase in non-transferrin-bound iron that may cause greater tissue toxicity than iron in other forms. Iron-induced oxidative stress is known to be one of the most important factors determining cell injury in thalassemic patients. Therefore, we designed this study to obtain a comprehensive picture of the iron overload, antioxidant status and cell damage in β thalassemia major patients undergoing regular blood transfusion. Materials and Methods: A total of 48 diagnosed patients of β thalassemia major and 30 age- and sex-matched healthy subjects were included in the study. Estimation of hemoglobin, hematocrit, glutathione peroxidase (GPX, superoxide dismutase (SOD,vitamin E, serum ferritin, total and direct bilirubin, AST and ALT was carried out. Results: The levels of vitamin E, antioxidant enzymes GPX and SOD were significantly lowered in β thalassemic patients as compared with the control group (P<0.001. Serum total and direct bilirubin, AST and ALT were significantly elevated in thalassemic subjects as compared with the control group, indicating liver cell damage. Conclusion: Thus, our findings indicate that thalassemics are in a state of enhanced oxidative stress and that the administration of selective antioxidants would represent a promising approach toward counteracting oxidative damage and its deleterious

  16. The Correlation between Troponin and Ferritin Serum Levels in the Patients with Major Beta-Thalassemia

    Iraj Shahramian

    2013-06-01

    Full Text Available Background: Thalassemia is a hereditary hemoglobinopathy whose most common complication is cardiac involvement which ends up in these patients’ death. Since troponin is a sensitive and specific marker for the detection of microinfarct, we studied the relationship between troponin and ferritin serum levels for early diagnosis of cardiac involvement in these patients. Materials and Methods: This case-control study was performed on 80 patients, including 40 patients with major thalassemia and normal echocardiography and 40 healthy volunteers ranging from 6 months to 16 years old. All the children were examined and the eligible children who were not infected with known heart disease, iron deficiency anemia, kidney disease, diabetes, fever, and systemic diseases were enrolled into the study after obtaining written informed consents from their parents. At 8:00 A.M. before breakfast, 5cc blood was drawn from these children. After collecting the samples, ferritin and troponin serum levels were evaluated using ELISA and electro- kymonolonsense methods, respectively. The gathered data were analyzed through the SPSS statistical software (v. 20 and T-test. Besides, P value<0.05 was considered as statistically significant. Results: The study results revealed a significant difference between the two groups regarding the mean of the serum levels of troponin (P=0.045 and ferritin (P=0.001. In this study, no significant correlation was observed between serum troponin and ferritin levels and age and BMI in the two groups. Also, no significant relationship was found between serum troponin level and sex (P=0.264. Conclusions: In microinfarct, troponin increases independent of ferritin; therefore, it can be used for early detection of cardiac involvement in thalassemia patients to determine the sub-clinical effects.

  17. HLA-matched family hematopoetic stem cell transplantation in children with beta thalassemia major: the experience of the Turkish Pediatric Bone Marrow Transplantation Group.

    Yesilipek, M Akif; Ertem, Mehmet; Cetin, Mualla; Öniz, Haldun; Kansoy, Savas; Tanyeli, Atila; Anak, Sema; Kurekci, Emin; Hazar, Volkan

    2012-12-01

    From January 1991 to June 2009, 245 children with beta thalassemia major who underwent their first allogeneic HSCT in Turkey and who were followed for a minimum of one yr post-transplantation were enrolled this study. The median age of the patients was 6.6 yr old (range, 1-22 yr). The distribution of Pesaro risk class I, II, and III categories was 41, 130, and 63 children, respectively. The median serum ferritin level was 2203 ng/mL. Eighty-eight patients received bone marrow (BM) stem cells; 137, peripheral blood (PB) stem cells; and 20, cord blood (CB) stem cells. The donors were HLA-matched siblings or parents. Median engraftment times were shorter in PBSCT patients compared with the BMT group (p < 0.001). Grade II-IV acute GvHD was observed in 33 children (13.5%), while cGvHD was observed in 28 patients (12.5%), eight of whom had the extensive form. Thalassemic reconstitution was observed in 43 (17%) of the transplant patients. Post-transplant aplasia occurred in three patients, and the TRM rate was 7.75%. Seventeen patients were lost after 100 days. The thalassemia-free survival and OS rates were 68% (95% CI, 61.8-74.2) and 85.0% (95% CI, 80.2-89.8), respectively. We believe that this study is important because it is the first multicenter national data for children with beta thalassemia major receiving HSCT. PMID:22931438

  18. Decrease of Hepatitis C Burden in Patients With Transfusion Dependent Beta Thalassemia Major, Thalassemia Research Center, 1995 – 2014

    Mehrnoush Kosaryan

    2015-07-01

    Full Text Available Background: Chronic hepatitis C infection used to be one of the most important burdens on patients with transfusion-dependent beta thalassemia major (TDTM. Chronic active hepatitis reduces quality of life, and liver cirrhosis and cancer shorten life expectancy in many cases. Objectives: We compared the characteristics of our patients at the Thalassemia Research Center (TRC regarding hepatitis C infection at two time points. Patients and Methods: A review was conducted in a cohort of 390 TDTM patients with a history of at least one blood transfusion in 2014. Type of treatment protocol for hepatitis C virus (HCV and the number of courses were defined. Descriptive statistics were performed using SPSS software (V16. Results: Screening for HCV started in 1995 at the TRC. Seventy-seven (15% patients were antibody-positive in 1995. Tests for virus detection were not available at the time. Patients have been examined using serum AST, ALT, bilirubin, PT, PTT, and liver biopsy, and 45 were treated using alpha interferon alone. A second liver biopsy was performed at the end of treatment for 21 patients, and a blinded pathologist compared the histology according to the Knodell score. According to normalization of liver enzymes, the treatment was successful (McNemar test, P < 0.02. Based on the Knodell score, 54%, 31%, and 11% had complete, partial, and no response, respectively. A quantitative test for viremia became available thereafter. Thirteen patients who were resistant to alpha interferon have been treated using “Pegasys”™ ± ribavirin. Ten patients responded; however, three have been resistant and are still viremic. Twenty-seven patients received no treatment. Twenty-two (81.4% had negative PCR tests. Five viremic patients refused treatment. A second screening test for HCV antibody was introduced in 2001, and, since then, annual screening for HCV antibody has been performed for all patients. No new case has been found since 2001. During the

  19. Efficacy of Carvedilol in Patients with Dilated Cardiomyopathy due to Beta-Thalassemia Major; a Double-Blind Randomized Controlled Trial

    Afsaneh Ashrafi

    2010-09-01

    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.

  20. Comparative evaluation of NESTROFT and RDW as screening tests for beta thalassemia trait in pregnancy

    Pritibala Patel; Nivedita Sarda; Renu Arora; Harsha Shailesh Gaikwad

    2015-01-01

    Background: Thalassemia is the commonest inherited hemoglobinopathy. It is estimated that there are about 45 million carriers of the beta thalassemia gene and about 15000 affected infants are born every year in India, thereby contributing to about 10% of the total thalassemia babies born all over the world. Beta Thalassemia Trait (BTT) is asymptomatic while Beta Thalassemia Major (BTM) presents with severe anemia and requires lifelong blood transfusion, so emphasis must shift from treatment t...

  1. Viral infections in transfusion-dependent patients with beta-thalassemia major: the predominant role of cytomegalovirus.

    Nigro, G; Lionetti, P; Digilio, G; Multari, G; Vania, A; Midulla, M

    1990-01-01

    For 9 months, 38 transfusion-dependent patients with beta-thalassemia, ranging in age from 3.4 to 19.1 years, were observed for serologic evidence of viral infections, by the collection of serial serum samples. Seventy-six age-matched healthy subjects, two for each patient, were followed as controls. Samples taken at the beginning, middle, and end of the study were tested against 18 viral antigens by complement fixation (CF). In addition, tests for antibodies to HIV, Epstein-Barr virus, hepatitis A virus, and markers for hepatitis B virus were performed. When changes in the antibody titer on CF tests (greater than or equal to 2-fold increase or decrease) or persistently high titers (greater than or equal to 64) were revealed, specific enzyme immunoassays (EIAs) for IgM and IgA antibodies were performed concomitant with CF tests in all sera. When symptomatic infections occurred, viral cultures and/or direct detection of antigens were carried out by immunofluorescence methods, EIA, or latex agglutination tests. Thalassemic patients and controls had similar (p greater than 0.05) overall rates of serologically confirmed viral infections (53 versus 132), but the former group had a higher (p less than 0.01) incidence of cytomegalovirus (CMV) infections (9 versus 4). CMV infections were associated in the thalassemic patients with hepatitis (2 cases), lymphadenitis (2 cases), and upper respiratory tract infection (1 case), while the remaining cases of CMV had a subclinical course. Moreover, the thalassemic patients had a lower (p less than 0.01) incidence of symptomatic infections (27 versus 110) than controls. Therefore, this study showed that both symptomatic and subclinical CMV infections may occur often in thalassemic patients, who otherwise have subclinical viral infections at an overall rate similar to that in healthy subjects. PMID:2173179

  2. Combine-ARMS: a rapid and cost-effective protocol for molecular characterization of beta-thalassemia in Malaysia.

    Tan, K L; Tan, J A; Wong, Y C; Wee, Y C; Thong, M K; Yap, S F

    2001-01-01

    Beta-thalassemia major patients have chronic anemia and are dependent on blood transfusions to sustain life. Molecular characterization and prenatal diagnosis of beta3-thalassemia is essential in Malaysia because about 4.5% of the population are heterozygous carriers for beta-thalassemia. The high percentage of compound heterozygosity (47.62%) found in beta-thalassemia major patients in the Thalassaemia Registry, University of Malaya Medical Centre (UMMC), Malaysia, also supports a need for rapid, economical, and sensitive protocols for the detection of beta-thalassemia mutations. Molecular characterization of beta-thalassemia mutations in Malaysia is currently carried out using ARMS, which detects a single beta-thalassemia mutation per PCR reaction. We developed and evaluated Combine amplification refractory mutation system (C-ARMS) techniques for efficient molecular detection of two to three beta-thalassemia mutations in a single PCR reaction. Three C-ARMS protocols were evaluated and established for molecular characterization of common beta-thalassemia mutations in the Malay and Chinese ethnic groups in Malaysia. Two C-ARMS protocols (cd 41-42/IVSII #654 and -29/cd 71-72) detected the beta-thalassemia mutations in 74.98% of the Chinese patients studied. The CARMS for cd 41-42/IVSII #654 detected beta-thalassemia mutations in 72% of the Chinese families. C-ARMS for cd 41-42/IVSI #5/cd 17 allowed detection of beta-thalassemia mutations in 36.53% of beta-thalassemia in the Malay patients. C-ARMS for cd 41-42/IVSI #5/cd 17 detected beta-thalassemia in 45.54% of the Chinese patients. We conclude that C-ARMS with the ability to detect two to three mutations in a single reaction provides more rapid and cost-effective protocols for beta-thalassemia prenatal diagnosis and molecular analysis programs in Malaysia. PMID:11336396

  3. The Association between Serum Ferritin Level, Tissue Doppler Echocardiography, Cardiac T2* MRI, and Heart Rate Recovery in Patients with Beta Thalassemia Major

    Yuksel, Isa Oner; Koklu, Erkan; Kurtoglu, Erdal; Arslan, Sakir; Cagirci, Goksel; Karakus, Volkan; Kus, Gorkem; Cay, Serkan; Kucukseymen, Selcuk

    2016-01-01

    Background It is generally well-understood that iron-mediated cardiomyopathy is the major complication that can arise from beta thalassemia major (TM). Therefore, early diagnosis, risk stratification, and the effective treatment of beta TM patients are clinically important to optimize long-term positive outcomes. Methods This study included 57 beta TM patients with a mean age of 25 ± 7 years. We determined the serum ferritin level, echocardiography, heart rate recovery (HRR), and cardiac magnetic resonance (CMR) T2* in all patients. CMR T2* findings were categorized as normal myocardium (T2* > 20 ms), and myocardial involvement (T2* ≤ 20 ms). HRR values at 1-5 min (HRR1-5) were recorded; Subsequently. HRR was calculated by subtracting the heart rate at each time point from the heart rate at peak exercise. Results There was a significant negative correlation between the serum ferritin level and the cardiac T2* MRI findings (r = -0.34, p = 0.009). A similar result was found in the negative correlation between serum ferritin and all heart rate recovery values. There was a significant positive correlation between HRR1, HRR2, and HRR3 values, and CMR T2* (T2* heart rate recovery (HRR)1: r = 0.51, p < 0.001; T2* HRR2: r = 0.48, p < 0.001; T2* HRR3: r = 0.47, p < 0.001, respectively). Conclusions The serum ferritin level and echocardiography can be used to predict the presence of myocardial iron load in beta TM patients. Therefore, HRR can be used to screen beta TM patients, and the clinical use of HRR can be a predictive marker for autonomic dysfunction in beta TM patients. PMID:27122954

  4. Anti-Thyroid Peroxidase Antibodies and Male Gender Are Associated with Diabetes Occurrence in Patients with Beta-Thalassemia Major

    Pes, Giovanni M.; Tolu, Francesco; Dore, Maria P.

    2016-01-01

    Background. Intensive transfusion schedule and iron-chelating therapy prolonged and improved quality of life in patients with β-thalassemia (β-T) major. However, this led to an increased risk of developing impaired glucose tolerance or diabetes. In this study we analyzed variables associated with the occurrence of impaired glucose tolerance or diabetes in patients with β-T major. Methods. 388 Sardinian patients were included. Age, gender, duration of chelation therapy, body mass index, and markers of pancreatic and extrapancreatic autoimmunity were analyzed. Results. Multiple logistic regression analysis showed that anti-thyroid peroxidase (TPO) antibodies (Ab) (OR = 3.36; p = 0.008) and male gender (OR = 1.98; p = 0.025) were significantly associated with glucose impairment, while the other variables were not. Ferritin levels were significantly higher in TPOAb positive compared to TPOAb negative patients (4870 ± 1665 μg/L versus 2922 ± 2773 μg/L; p < 0.0001). Conclusions. In patients with β-T major a progressive damage of insulin-producing cells due to secondary hemosiderosis appears to be the most reasonable mechanism associated with glucose metabolism disorders. The findings need to be confirmed with additional well designed studies to address the question of whether TPOAb may have a role in the management of these patients. PMID:27123460

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

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

    2012-01-01

    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

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

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

    2008-01-01

    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 ...

  7. C3 polymorphism in beta-thalassemia.

    Germenis, A; Kalantzi, M; Karamerou, A; Politi, C; Fertakis, A

    1984-01-01

    The distribution of phenotypes and gene frequencies of the third component of complement (C3) were studied in 106 beta-thalassemic patients and in 112 carriers of the beta-thalassemia trait. A statistically significant association was found between the C3F gene and homozygous beta-thalassemia. It can be suggested that this association may be related with the high incidence of infections encountered in these patients. PMID:6419525

  8. Plastic bronchitis in beta thalassemia minor

    Makaresh Yadav; Sneha Tirpude; Joshi, Jyotsna M.

    2013-01-01

    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.

  9. Alpha-globin loci in homozygous beta-thalassemia intermedia.

    Triadou, P; Lapoumeroulie, C; Girot, R; Labie, D

    1983-01-01

    Homozygous beta-thalassemia intermediate (TI) differs from thalassemia major (TM) in being less severe clinically. Associated alpha-thalassemia could account for the TI phenotype by reducing the alpha/non-alpha chain imbalance. We have analyzed the alpha loci of 9 TI and 11 TM patients by restriction endonuclease mapping. All the TM and 7 of the TI patients have the normal complement of four alpha-globin genes (alpha alpha/alpha alpha). One TI patient has three alpha-globin genes (alpha alpha/-alpha), and another TI patient has five alpha genes (alpha alpha/alpha alpha alpha). PMID:6305827

  10. Comparative evaluation of NESTROFT and RDW as screening tests for beta thalassemia trait in pregnancy

    Pritibala Patel

    2015-04-01

    Full Text Available Background: Thalassemia is the commonest inherited hemoglobinopathy. It is estimated that there are about 45 million carriers of the beta thalassemia gene and about 15000 affected infants are born every year in India, thereby contributing to about 10% of the total thalassemia babies born all over the world. Beta Thalassemia Trait (BTT is asymptomatic while Beta Thalassemia Major (BTM presents with severe anemia and requires lifelong blood transfusion, so emphasis must shift from treatment to screening and offering prenatal counseling to affected parents. Methods: It was a hospital based cross sectional study on 500 antenatal women with microcytic hypochromic anemia with hemoglobin <9 gm% and MCV <80 fl and all these women underwent Naked Eye Single Tube Red Cell Osmotic Fragility Test (NESTROFT, High Performance Liquid Chromatography (HPLC and serum ferritin estimation. Results: In our study the sensitivity and specificity of NESTROFT was 93.3% and 95.7% respectively compared to Red cell Distribution Width (RDW with a sensitivity and specificity of 66.6% and 78.9% respectively. Conclusions: For low resource settings like India, screening for beta thalassemia by NESTROFT is a cheaper and more reliable method with a high sensitivity and specificity and can be performed easily by paramedical staff. [Int J Reprod Contracept Obstet Gynecol 2015; 4(2.000: 424-428

  11. Renal tubular dysfunction nephrocalcinosis in a patient with BetaThalassemia Minor

    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 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)

  12. Intracranial Extramedullary Hematopoiesis in Beta-Thalassemia

    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.

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

    Prabahar Murugesan

    2008-01-01

    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.

  14. EXERCISE STRESS ECHOCARDIOGRAPHY WITH TISSUE DOPPLER IMAGING (TDI DETECTS EARLY SYSTOLIC DYSFUNCTION IN BETA-THALASSEMIA MAJOR PATIENTS WITHOUT CARDIAC IRON OVERLOAD

    Umberto Barbero

    2012-06-01

    Full Text Available Iron Overload Cardiomyopathy (IOC is still the main cause of death in thalassemia major (TM patients. Unfortunately, Conventional Echocardiography fails to predict early cardiac dysfunction. As Tissue Doppler Imaging (TDI may demonstrate regional myocardial dysfunction, we wondered if exercise may reveal abnormalities at TDI which are not evident at rest. To try to evaluate left and right myocardial performances at rest and after maximal exercise by both conventional and TDI parameters, 46 beta-TM adult patients and 39 control subjects were enrolled. All patients had a liver iron quantification by Superconducting Quantum Interference Device (SQUID and also a cardiac iron assessment by MRI (T2*: 38 TM patients had no evidence of cardiac iron overload. Whereas TM patients did not shown diastolic dysfunction and all of them presented a good global response to exercise, TDI detected a reduced increase of the S’ waves of left ventricle basal segment during exercise. This finding seems to have some weak but interesting relations with iron overload markers. In conclusion, in our study, exercise stress TDI-echocardiography was able to demonstrate subtle systolic abnormalities that were missed by Conventional Echocardiography. Further studies are required to determine the meaning and the clinical impact of these results.

  15. Prevalence of hepatosplenomegaly in beta thalassemia minor subjects in Iran

    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

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

    Soleiman Mahjoub

    2007-12-01

    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.

  17. Pathogenetic aspects of immune deficiency associated with beta-thalassemia.

    Farmakis, Dimitrios; Giakoumis, Anastasios; Polymeropoulos, Evaggelos; Aessopos, Athanasios

    2003-01-01

    Infectious complications constitute the second most common cause of mortality and a main cause of morbidity in beta-thalassemia. Besides the high risk of blood-borne infections associated with multiple transfusions, the increased susceptibility of these patients to infectious diseases has been attributed to a coexistent immune deficiency. Immune abnormalities have also been held responsible for the frequent occurrence of malignancies in beta-thalassemia, especially leukemia and lymphomas. Recent studies on immune competence in beta-thalassemia have revealed numerous quantitative and functional defects, involving T and B lymphocytes, immunoglobulin production, neutrophils and macrophages, chemotaxis, and phagocytosis, as well as the complement system. Regarding pathogenesis, iron overload, a primary complication of both thalassemia itself and transfusion therapy, is thought to be the main precipitating mechanism, due to the important immunoregulatory properties of iron and its binding proteins; iron excess may derange the immune balance in favor of the growth of infectious organisms. Other factors include multiple transfusions, associated with constant allo-antigenic stimulation, as well as with transmission of immunosuppressive viruses; splenectomy, resulting in increased susceptibility to infections by encapsulated bacteria and to immune system modifications; low levels of zinc, another immune regulator; iron chelation therapy, which predisposes to serious infections by yersinia species; and the circulation of abnormal native thalassemic erythrocytes, forming another permanent immune stimulus. Thus surveillance for infections in patients with beta-thalassemia is crucial, while further studies are warranted on immune function abnormalities and the implicated mechanisms. PMID:12552254

  18. [Hemoglobin C -- beta-thalassemia disease and homozygous beta-thalassemia in a black African family (author's transl)].

    Basset, P; Fall, M; Oudart, J L

    1975-01-01

    The study of a Malian family has allowed to prove existence of two types of beta-thalassemia genes: the beta0 gene which suppresses the synthesis of the beta chain into cis position and the beta+ gene which slows down only partially this synthesis. The difference between this two genes has been possible owing to the hemoglobin C found in this family and induced by the betaC mutated gene. The segregation of the four genes betaA, betaC, beta0 thal, and beta+ thal. has allowed to compare all the possible phenotypes deriving from the combinations by two of these allelic genes. PMID:128735

  19. THE DIAGNOSTIC VALUE OF PULSED WAVE TISSUE DOPPLER IMAGING IN ASYMPTOMATIC BETA- THALASSEMIA MAJOR CHILDREN AND YOUNG ADULTS ; RELATION TO CHEMICAL BIOMARKERS OF LEFT VENTRICULAR FUNCTION AND IRON OVERLOAD .

    Seham Ragab

    2015-08-01

    Full Text Available Background: Cardiac iron toxicity is the leading cause of death among  β-halassaemia major (TM  patients.  Once  heart failure becomes overt , it will be  difficult to reverse . Objectives: To investigate non overt cardiac dysfunctions  in TM patients using  pulsed wave Tissue Doppler  Imaging (TD I and its relation to the iron overload and brain natruritic peptide (BNP. Methods: Thorough  clinical , conventional echo and  pulsed  wave TDI  parameters were compared between  asymtomatic 25 β-TM  patients  and 20 age and gender matched individuals. Serum ferritin and plasma BNP  levels were assayed by  ELISA .  Results: TM patients had significant higher mitral inflow early diastolic (E wave and  non significant other conventional echo  parameters. Pulsed wave TDI revealed systolic and diastolic dysfunctions in the form of significant higher  isovolumetric contraction time (ICT , ejection time ( E T and  isovolumetric relaxation time (IRT with significantly lower  mitral annulus  early diastolic velocity E` (12.07 ±2.06 vs 15.04±2.65 ,P= 0.003  in patients compared to  controls. Plasma BNP was higher in patients compared to the controls.  Plasma BNP and serum ferritin had significant correlation with each other and with pulsed wave conventional and TDI indices of systolic and diastolic functions.  Patients with E/E` ≥ 8 had  significant higher  serum ferritin  and plasma BNP levels compared to those with E/E` ratio < 8 without difference in Hb levels .Conclusion:  Pulsed wave TDI  is an  important diagnostic tool for latent cardiac dysfunction in iron loaded TM patients and is related to iron overload and BNP .

  20. Detection of glycemic abnormalities in adolescents with beta thalassemia using continuous glucose monitoring and oral glucose tolerance in adolescents and young adults with β-thalassemia major: Pilot study

    Ashraf T Soliman

    2013-01-01

    Full Text Available Background: Both insulin deficiency and resistance are reported in patients with β-thalassemia major (BTM. The use of continuous blood glucose monitoring (CGM, among the different methods for early detection of glycemic abnormalities, has not been studied thoroughly in these adolescents. Materials and Methods: To assess the oralglucose tolerance (OGT and 72-h continuous glucose concentration by the continuous glucose monitoring system (CGMS and calculate homeostatic model assessment (HOMA, and the quantitative insulin sensitivity check index (QUICKI was conducted in 16 adolescents with BTM who were receiving regular blood transfusions every 2-4 weeks and iron-chelation therapy since early childhood. Results: Sixteen adolescents with BTM (age: 19.75 ± 3 years were investigated. Using OGTT, (25% had impaired fasting blood (plasma glucose concentration (BG (>5.6 mmol/L. 2-h after the glucose load, one of them had BG = 16.2 mmol/L (diabetic and two had impaired glucose tolerance (IGT (BG > 7.8 and 11.1 mmol/L and 9 with IGT (56%. HOMA and QUICKI revealed levels 0.33 (0.36 ± 0.03, respectively, ruling out significant insulin resistance in these adolescents. There was a significant negative correlation between the β-cell function (B% on one hand and the fasting and the 2-h BG (r=−0.6, and − 0.48, P < 0.01, respectively on the other hand. Neither fasting serum insulin nor c-peptide concentrations were correlated with fasting BG or ferritin levels. The average and maximum blood glucose levels during CGM were significantly correlated with the fasting BG (r = 0.68 and 0.39, respectively, with P < 0.01 and with the BG at 2-hour after oral glucose intake (r = 0.87 and 0.86 respectively, with P < 0.001. Ferritin concentrations were correlated with the fasting BG and the 2-h blood glucose levels in the OGTT (r = 0.52, and r = 0.43, respectively, P < 0.01 as well as with the average BG recorded by CGM (r = 0.75, P < 0.01. Conclusion: CGM has proven to

  1. THERAPEUTIC VALUE OF COMBINED THERAPY WITH DEFERASIROX AND SILYMARIN ON IRON OVERLOAD IN CHILDREN WITH BETA THALASSEMIA

    adel abd elhaleim hagag

    2013-11-01

    Patients and Methods: This study was conducted on 40 children with beta thalassemia major under follow-up at Hematology Unit, Pediatric Department, Tanta University Hospital having serum ferritin level more than 1000 ng/ml and was divided in two groups. Group IA: Received oral Deferasirox (Exjade and silymarin for 6 months. Group IB: Received oral Deferasirox (Exjade and placebo for 6 months and 20 healthy children serving as a control group in the period between April 2011 and August 2012 and was performed after approval from research ethical committee center in Tanta University Hospital and obtaining an informed written parental consent from all participants in this research. Results: Serum ferritin levels were markedly decreased in group IA cases compared with group IB (P= 0.001. Conclusion: From this study we concluded that, silymarin in combination with Exjade can be safely used in treatment of iron-loaded thalassemic patients as it showed good iron chelation with no sign of toxicity. Recommendations: Extensive multicenter studies in large number of patients with longer duration of follow up and more advanced methods of assessment of iron status is recommended to clarify the exact role of silymarin in reduction of iron over load in children with beta thalassemia.

  2. [Assessment of endogenous intoxication and thrombocyte functions in beta-thalassemia].

    Orudzhev, A G; Guseĭnova, E E; Khalilova, I S; Dzhavadov, S A

    2003-03-01

    The parameters of humeral immunity and of the aggregation function of blood platelets were comparatively analyzed in 11 healthy and 26 children with beta-thalassemia; 18 of them had beta-thalassemia of the homozygous type (spleen was extracted in 8 children, and it remained intact in 10 children). It was demonstrated that there was an increased quantity of antibodies to blood platelets and an increased quantity of large, medium-size and small circulating immune complexes in patients with beta-thalassemia and especially in those, who did not undergo splenectomy; there was also an increased quantity of immunoglobulins of classes A, M, and G, a reduced total activity of the complement and a high parameter of the degree of endogenous intoxication, i.e. the content of medium-size molecular peptides. The mentioned disorders were accompanied by worsened aggregation abilities of blood platelets and by their increased disaggregation. Finally, insignificant changes were detected in patients with beta-thalassemia of the heterozygous type. PMID:12715396

  3. Fe-52 imaging of intrathoracic extramedullary hematopoiesis in a patient with beta-thalassemia.

    Adams, B K; Jacobs, P; Byrne, M J; Bird, A R; Boniaczszuk, J

    1995-07-01

    Fe-52 scintigraphy was used to confirm extramedullary hematopoiesis in a patient with beta-thalassemia and intrathoracic masses. Imaging was performed on a standard gamma camera with a high-energy collimator. Tc-99m labeled tin colloid and In-111 chloride scintigraphy failed to reveal uptake by the masses. The exclusion of malignancy obviated the need for invasive diagnostic measures. PMID:7554666

  4. Evaluation of the children with beta-thalassemia in terms of their self-concept, behavioral, and parental attitudes.

    Yalçn, Siddika Songül; Durmuşoğlu-Sendoğdu, Mine; Gümrük, Fatma; Unal, Selma; Karg, Eda; Tuğrul, Belma

    2007-08-01

    This study was planned to explore the self-concept, behavioral, and parental attitudes of the children with beta-thalassemia major, and the factors that affect them. The study was undertaken between January and June 2004 at the Hacettepe University Ihsan Doğramaci Children's Hospital, Pediatric Hematology Unit, Ankara and 43 voluntary children with beta-thalassemia major on regular blood transfusion and iron chelation treatment between the ages of 5.0 and 18.0 years were included into the study. Age, sex, birth order, school performance, hemoglobin value, serum ferritin levels, associated illness, splenectomy status, presence of thalassemic sibling or relatives, death of thalassemic relatives, place of residence, maternal and paternal education were recorded. Parental Attitude Research Instrument, Piers-Harris Self-Concept Scale, and Child Behavior Checklist were applied. Higher educated mothers have lower overprotection (P=0.009), parental discordance (P=0.044), and discipline scores (P=0.002) than lower educated mothers. In cases with death of thalassemic relatives, democratic/equality attitude scores were decreased (P=0.034). With stepwise multiple linear regression analysis, splenectomy, good school achievement, absence of death of thalassemic relatives, and serum ferritin levels were found to increase Piers-Harris Self-Concept Scale; however, total behavior problem score was found to decrease with increasing age, splenectomy and decreasing overprotection subscale of Parental Attitude Research Instrument scores. The self-esteem and behavior problems of children with thalassemia depended not only on the variables related exclusively to the child (age, school achievement) and the illness-associated conditions (splenectomy, serum ferritin levels) but also on the parental attitude (overprotection). PMID:17762492

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

    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 know the actual prevalence of beta-thalassemia in Al-Hassa. (author)

  6. On the origin and spread of beta-thalassemia: recurrent observation of four mutations in different ethnic groups.

    Wong, C.; Antonarakis, S E; Goff, S C; Orkin, S H; Boehm, C. D.; Kazazian, H H

    1986-01-01

    Seven beta-thalassemia genes were characterized after they were identified as candidates for previously undescribed mutations based upon the close association of DNA polymorphism haplotypes in the beta-globin gene cluster with specific ethnic mutations. The molecular defect in four of these genes was identical, a frameshift deletion of four nucleotides (-CTTT) within codons 41 and 42. This gene represents a common Southeast Asian mutation shared by a Laotian beta-thalassemia gene, [framework ...

  7. Effect of pregnancy on differentiation of minor Beta-Thalassemia from iron deficiency

    Ghanei M

    1997-01-01

    Differential diagnosis of Iron-deficiency anemia and Beta-Thalassemia, two common causes of anemia, affects the treatment in pregnant women. To help the diagnosis, we have tried to asses the pure effect of gestation on diagnostic criteria, eliminating iron and folate deficiency. In a prospective study, 46 thalassemic women were given Ferrous Sulphate tablets and Folate. Some indices, CBC and HbA2 were measured before and after treatment during pregnancy. The haemoglobin and HbA2 decreased and...

  8. Identification of the multiple beta-thalassemia mutations by denaturing gradient gel electrophoresis.

    Cai, S P; Kan, Y W

    1990-01-01

    We used denaturing gradient gel electrophoresis to detect the beta-thalassemia mutations in the Chinese population. By amplifying the beta-globin gene in four separate fragments and electrophoresing the amplified DNA in two gels, we were able to distinguish all the 12 known mutations on the basis of the mobility of the homoduplexes and heteroduplexes. We conclude that denaturing gradient gel electrophoresis offers a nonradioactive means of detecting multiple mutations in genetic disorders.

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

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

    1991-01-01

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

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

    Dorina STOICANESCU

    2009-11-01

    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.

  11. Infusion of Autologous Retrodifferentiated Stem Cells into Patients with Beta-Thalassemia

    Ilham Saleh Abuljadayel

    2006-01-01

    Full Text Available Beta-thalassemia is a genetic, red blood cell disorder affecting the beta-globin chain of the adult hemoglobin gene. This results in excess accumulation of unpaired alpha-chain gene products leading to reduced red blood cell life span and the development of severe anemia. Current treatment of this disease involves regular blood transfusion and adjunct chelation therapy to lower blood transfusion–induced iron overload. Fetal hemoglobin switching agents have been proposed to treat genetic blood disorders, such as sickle cell anemia and beta-thalassemia, in an effort to compensate for the dysfunctional form of the beta-globin chain in adult hemoglobin. The rationale behind this approach is to pair the excess normal alpha-globin chain with the alternative fetal gamma-chain to promote red blood cell survival and ameliorate the anemia. Reprogramming of differentiation in intact, mature, adult white blood cells in response to inclusion of monoclonal antibody CR3/43 has been described. This form of retrograde development has been termed “retrodifferentiation”, with the ability to re-express a variety of stem cell markers in a heterogeneous population of white blood cells. This form of reprogramming, or reontogeny, to a more pluripotent stem cell state ought to recapitulate early hematopoiesis and facilitate expression of a fetal and/or adult program of hemoglobin synthesis or regeneration on infusion and subsequent redifferentiation. Herein, the outcome of infusion of autologous retrodifferentiated stem cells (RSC into 21 patients with beta-thalassemia is described. Over 6 months, Infusion of 3-h autologous RSC subjected to hematopoietic-conducive conditions into patients with beta-thalassemia reduced mean blood transfusion requirement, increased mean fetal hemoglobin synthesis, and significantly lowered mean serum ferritin. This was always accompanied by an increase in mean corpuscular volume (MCV, mean corpuscular hemoglobin (MCH, and mean

  12. Effect of pregnancy on differentiation of minor Beta-Thalassemia from iron deficiency

    Ghanei M

    1997-07-01

    Full Text Available Differential diagnosis of Iron-deficiency anemia and Beta-Thalassemia, two common causes of anemia, affects the treatment in pregnant women. To help the diagnosis, we have tried to asses the pure effect of gestation on diagnostic criteria, eliminating iron and folate deficiency. In a prospective study, 46 thalassemic women were given Ferrous Sulphate tablets and Folate. Some indices, CBC and HbA2 were measured before and after treatment during pregnancy. The haemoglobin and HbA2 decreased and MCV increased, all with significant P value. We concluded that HbA2, independent of iron, will decrease during pregnancy and MCV will increase

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

    Mahmood Mahmoodian Shooshtari; Saeideh Milani; Zohreh Sharifi

    2010-01-01

    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 μg) of recombinant HBV vaccine in mont...

  14. Fortuitous diagnosis of the association of hemoglobin J-Broussais with beta + thalassemia.

    Durou, M R; Ribault, A; Dufour, M J; Gallou, G; Ruelland, A; Le Gall, E; Cloarec, L; Legras, B

    1996-01-01

    The authors report a case, not described so far in literature, of an association of HbJ-Broussais [alpha (90 (PG2) lys-->asn beta 2] with beta + thalassemia in a young girl born of Italian father and Breton mother. This association is clinically silent. Biochemistry revealed, besides HbA, the presence of HbJ-Broussais in the proportion of 19.4% and HbA2 value of 3.9%. These percentages, slightly lower than expected, are explained. A familial study is presented. PMID:9092311

  15. Approaches to management of beta-thalassemia intermedia

    Joseph E. Maakaron

    2013-03-01

    Full Text Available Thalassemia intermedia is a genetically diverse group of diseases that is the result of an imbalance in the production of the alpha and beta chains with ensuing chronic hemolysis, ineffective erythropoiesis, and iron overload.Resulting complications include bone changes, hypercoagulability, and end-organ damage due to iron overload. This decade has witnessed major breakthroughs in the management of thalassemia. In this article, we examine these novelties in therapy including iron chelation therapy, stem cell transplant, and gene therapy.Iron chelation therapy has been revolutionized with the advent of deferasirox, a once-daily oral iron chelator, that has been shown to be safe and efficacious.Gene therapy was also at the core of this revolution with the discovery of novel gene elements and viral vectors allowing for better control and improved outcomes.

  16. Prevalence of beta-thalassemia and sickle cell anemia trait in premarital screening in Konya urban area, Turkey.

    Guler, Ekrem; Caliskan, Umran; UcarAlbayrak, Canan; Karacan, Mehmet

    2007-11-01

    Thalassemias and sickle cell anemia (SCA) are common disease in Turkey. To determine the prevalence of beta-thalassemia and SCA traits in Konya urban area of Turkey, all couples applied for marriage procedures were screened. Screening tests included complete blood count and quantitation of hemoglobin for both partners. The subjects were considered to have the beta-thalassemia trait if they had a mean corpuscular volume of less than 80 fL and/or a mean corpuscular hemoglobin level of less than 27 pg and a hemoglobin A2 level of more than 3.2% or a hemoglobin F level of more than 2%. Subjects were considered to have an SCA trait if they were positive for sickle hemoglobin. During the study, premarital screening of hemoglobinopathies was evaluated retrospectively in 72,918 subjects; the thalassemia trait was detected in 1465 subjects (2%), and the SCA trait was detected in 37 subjects (0.05%). Of the carriers of the beta-thalassemia trait, 820 (56%) people had high hemoglobin A2, 513 (35%) people had high hemoglobin F, and 132 (9%) people had both high hemoglobin F and hemoglobin A2. Our results are very similar to Turkey's beta-thalassemia and SCA trait averages. PMID:17984699

  17. Identification of three rare beta-thalassemia mutations in the Pakistani population.

    Khan, S N; Riazuddin, S; Galanello, R

    2000-02-01

    Three rare beta-thalassemia mutations, not reported previously in Asian Indians or the Pakistani population, were identified by single strand conformation polymorphism analysis followed by direct sequencing. Two mutations, IVS-II-848 (C-->A) and initiation codon (ATG-->ACG), were found in the homozygous condition in patients belonging to Balochi and Sindhi ethnic groups of Pakistan, together with heterozygous and homozygous alpha(-3.7) deletions, respectively. A frameshift mutation at codon 44 (-C) was identified in a patient belonging to the Gujrati ethnic group together with IVS-I-1 (G-->T) and a normal complement of four a-globin genes. Haplotype analysis was performed to identify the chromosomal background associated with these mutations, and for tracing the origin and spread of these mutations. PMID:10722111

  18. Urinary iron excretion induced by intravenous infusion of deferoxamine in beta-thalassemia homozygous patients.

    Boturao-Neto, E; Marcopito, L F; Zago, M A

    2002-11-01

    The purpose of the present study was to identify noninvasive methods to evaluate the severity of iron overload in transfusion-dependent beta-thalassemia and the efficiency of intensive intravenous therapy as an additional tool for the treatment of iron-overloaded patients. Iron overload was evaluated for 26 beta-thalassemia homozygous patients, and 14 of them were submitted to intensive chelation therapy with high doses of intravenous deferoxamine (DF). Patients were classified into six groups of increasing clinical severity and were divided into compliant and non-compliant patients depending on their adherence to chronic chelation treatment. Several methods were used as indicators of iron overload. Total gain of transfusion iron, plasma ferritin, and urinary iron excretion in response to 20 to 60 mg/day subcutaneous DF for 8 to 12 h daily are useful to identify iron overload; however, urinary iron excretion in response to 9 g intravenous DF over 24 h and the increase of urinary iron excretion induced by high doses of the chelator are more reliable to identify different degrees of iron overload because of their correlation with the clinical grades of secondary hemochromatosis and the significant differences observed between the groups of compliant and non-compliant patients. Finally, the use of 3-9 g intravenous DF for 6-12 days led to a urinary iron excretion corresponding to 4.1 to 22.4% of the annual transfusion iron gain. Therefore, continuous intravenous DF at high doses may be an additional treatment for these patients, as a complement to the regular subcutaneous infusion at home, but requires individual planning and close monitoring of adverse reactions. PMID:12426631

  19. Bioinformatics e Biostatistics applied to research in pediatric genetic disease. Clinical evidence in IFNλ4 polymorphisms associated with HCV infection in patients with beta thalassemia and WGCNA analysis weighted for IFNλ4 genotype rs12979860 to detect RPL9P18 as hub in HCV infected cell.

    Marceddu, Giuseppe

    2015-01-01

    Genome-wide association studies have identified host genetic variation to be critical for spontaneous clearance and treatment response in patients infected with hepatitis C virus (HCV). We demonstrated the same in patients with thalassemia major infected by genotype 1b of HCV. In the present first part study we retrospectively analyzed 368 anti-HCV positive patients with beta-thalassemia in two Italian major thalassemic centers (Cagliari and Turin). The strongest IFNλ4 SN...

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

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

  1. Screening and genetic counseling for beta-thalassemia trait in a population unselected for interest: comparison of three counseling methods.

    Rowley, P T; Lipkin, M; Fisher, L

    1984-01-01

    We have assessed the effects of screening and genetic counseling for beta-thalassemia trait on knowledge, attitudes, and behavior in a prospective, controlled study of randomly selected adult members of a health maintenance organization. We report here that knowledge of manifestations and of inheritance of thalassemia, previously reported to be high immediately after counseling, were well maintained at 2 and 10 months following counseling. There was no detectable impairment of self-concept. M...

  2. Molecular comparison of delta beta-thalassemia and hereditary persistence of fetal hemoglobin DNAs: evidence of a regulatory area?

    Ottolenghi, S; Giglioni, B; Taramelli, R; Comi, P; Mazza, U; Saglio, G.; Camaschella, C.; Izzo, P; Cao, A; Galanello, R; Gimferrer, E.; Baiget, M.; Gianni, A M

    1982-01-01

    The hematological phenotypes of several Mediterranean patients with delta beta-thalassemia and hereditary persistence of fetal hemoglobin have been characterized. Although clinical and hematological characteristics are essentially superimposable in all heterozygous delta beta-thalassemics, these patients show typical G gamma/A gamma ratios in their Hb F, ranging from approximately 0.07 in Sardinian to approximately 0.15 in Sicilian and approximately 0.35 in Spanish patients. A gamma Sardinian...

  3. Frequency distribution of sickle cell anemia, sickle cell trait and sickle/beta-thalassemia among anemic patients in Saudi Arabia

    Elsayid, Mohieldin; Al-Shehri, Mohammed Jahman; Alkulaibi, Yasser Abdullah; Alanazi, Abdullah; Qureshi, Shoeb

    2015-01-01

    Background: Notwithstanding, the growing incidence of sickle cell hemoglobinopathies (SCH) such as sickle cell anemia (SCA) or sickle cell disease, sickle/beta-thalassemia; the exact prevalence remains obscure in Saudi Arabia. Hence, this study is an attempt to determine the frequency of SCA and sickle cell trait (SCT) among all anemic patients with SCH treated at the King Abdul-Aziz Medical City (KAMC), Riyadh, Saudi Arabia. Furthermore, the hemoglobin (Hb) S and other Hb patterns (Hb AS and...

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

    Baş, Sema Şanal; Özlü, Onur

    2012-01-01

    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...

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

    Zama Messala Luna da Silveira

    2011-01-01

    Full Text Available 35 unrelated individuals were studied for characterization as either heterozygous or homozygous for beta-thalassemia. Molecular analysis was done by PCR/RFLP to detect the mutations most commonly associated with beta-thalassemia (β0IVS-I-1, β+IVS-I-6, and β039. In the patients who showed none of these mutations, the beta-globin genes were sequenced. Of the 31 heterozygous patients, 13 (41.9% had the β+IVS-I-6 mutation, 15 (48.4% the β0IVS-I-1 mutation, 2 (6.5% the β+IVS-I-110 mutation and 1 (3.2% the β+IVS-I-5 mutation. IVS-I-6 was detected in the four homozygotes. The mutation in codon 39, often found in previous studies in Brazil, was not detected in the present case. This is the first study aiming at identifying mutations that determine beta-thalassemia in the state of Rio Grande do Norte.

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

    Zohreh Sharifi

    2010-06-01

    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.

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

    Mahmood Mahmoodian Shooshtari

    2010-06-01

    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 μg 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.

  8. Prevalence of Diabetes and Impaired Glucose Tolerance Test in Patients with Thalassemia Major

    Mehdi Jahantigh; Majid Naderi; Akbar Dorgalaleh; Shadi Tabibian

    2014-01-01

    Background: Diabetes is one of the most common endocrine disorder worldwide that due to high prevalence and chronic nature of diabetes imposes a heavy cost on health care system. Therefore this study aimed to assess prevalence of diabetes among patients with beta thalassemia major. Materials and Methods: This descriptive study was conducted on 364 patients with beta thalassemia major that received at least 100 blood bags. For evaluation of diabetes among these patients, fasting blood sugar...

  9. 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

    Kemal Nisli

    2010-04-01

    Full Text Available OBJETIVO: Analisar comparativamente a dispersão da onda P (DOP em pacientes com talassemia beta maior (β-TM e indivíduos saudáveis (controles para a detecção precoce do risco de arritmias. MÉTODOS: Oitenta e uma crianças com β-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 β-TM do que nos indivíduos controles. CONCLUSÕES: O aumento da DOP em nossos pacientes com β-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 β-thalassemia major (TM and healthy control subjects for the early prediction of arrhythmia risk. METHODS: Eighty-one children with β-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 β-TM patients than in control subjects. CONCLUSIONS: Increased PWD in our β-TM patients might be related

  10. Cell and Gene Therapy for the Beta-Thalassemias: Advances and Prospects.

    Mansilla-Soto, Jorge; Riviere, Isabelle; Boulad, Farid; Sadelain, Michel

    2016-04-01

    The beta-thalassemias are inherited anemias caused by mutations that severely reduce or abolish expression of the beta-globin gene. Like sickle cell disease, a related beta-globin gene disorder, they are ideal candidates for performing a genetic correction in patient hematopoietic stem cells (HSCs). The most advanced approach utilizes complex lentiviral vectors encoding the human β-globin gene, as first reported by May et al. in 2000. Considerable progress toward the clinical implementation of this approach has been made in the past five years, based on effective CD34+ cell mobilization and improved lentiviral vector manufacturing. Four trials have been initiated in the United States and Europe. Of 16 evaluable subjects, 6 have achieved transfusion independence. One of them developed a durable clonal expansion, which regressed after several years without transformation. Although globin lentiviral vectors have so far proven to be safe, this occurrence suggests that powerful insulators with robust enhancer-blocking activity will further enhance this approach. The combined discovery of Bcl11a-mediated γ-globin gene silencing and advances in gene editing are the foundations for another gene therapy approach, which aims to reactivate fetal hemoglobin (HbF) production. Its clinical translation will hinge on the safety and efficiency of gene targeting in true HSCs and the induction of sufficient levels of HbF to achieve transfusion independence. Altogether, the progress achieved over the past 15 years bodes well for finding a genetic cure for severe globin disorders in the next decade. PMID:27021486

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

    Fakher Rahim

    2009-09-01

    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 β-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 β-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 β-TT and IDA. Mean and median mean cell Hb density (MCHD were very close to normal values in both IDA and β-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 β-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 β-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.

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

    Hashemi Reza

    2003-08-01

    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.

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

    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)

  14. Sehgal index: A new index and its comparison with other complete blood count-based indices for screening of beta thalassemia trait in a tertiary care hospital

    Kunal Sehgal

    2015-01-01

    Full Text Available Introduction: Beta thalassemia trait (BTT must be differentiated from iron deficiency anemia to avoid unnecessary iron therapy and for the prevention of thalassemia major by genetic counseling. In a tertiary care hospital, it is vital that the screening tool is not only sensitive but also specific so as to be cost effective and save time. Aim: The aim of this study was to evaluate the new Sehgal index and compare it to existing complete blood count-based indices for the best combination of sensitivity and specificity to predict BTT. Materials and Methods: Study was done in 2 phases - Phase 1: A retrospective analysis of 1022 consecutive high-performance liquid chromatography (HPLC cases from July 2008 to June 2011. Phase 2: A prospective analysis of 973 consecutive HPLC cases from July 1, 2011 to June 10, 2013 was done to confirm the results of Phase 1 and the applicability of the new Sehgal index. Results: Prevalence of BTT was 28.8% (294/1022 and 25.39% (247/973 in Phase 1 and Phase 2, respectively. Receiver operating characteristic-area under the curve and Youden index was highest for new Sehgal index, followed by Mentzers index <14. The prospective study shows results similar to those in Phase 1 confirming the superiority of the above two indices. Conclusion: Sehgal index and Mentzers index <14 showed the best combination of sensitivity and specificity in predicting BTT. The best indices or combination can be used as a "validated flag rule" in the analyzer middleware program in a hospital for identifying suspected cases of BTT.

  15. 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

    Ana L. B. Domingos

    2010-02-01

    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.

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

    Kotb Abbass Metwalley

    2014-01-01

    Full Text Available Background: Oxidative stress in children with β-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 β-thalassemia major and its relation to oxidative stress. Materials and Methods: Sixty children and adolescents with β-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 β--thalassemia is associated with

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

    Ridham A. Khanderia; Amit H Agravat

    2015-01-01

    Background: Beta thalassemia continues to be a significant burden to Western India particularly Saurashtra region of Gujarat. Since cost of treatment is high emphasis must be shifted from treatment to prevention that includes mass screening as most effective tool including RBC indices and peripheral blood smear. These tests have limited availability, re quire sophisticated equipments and are expensive. Thus, there is need for simple, low cost and reliable test which can be used in absence o...

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

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

    1996-01-01

    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 ...

  19. Electrocardiographic Presentation, Cardiac Arrhythmias, and Their Management in β-Thalassemia Major Patients.

    Russo, Vincenzo; Rago, Anna; Papa, Andrea Antonio; Nigro, Gerardo

    2016-07-01

    Beta-thalassemia major (β-TM) is a genetic hemoglobin disorder characterized by an absent synthesis of globin chains that are essential for hemoglobin formation, causing chronic hemolytic anemia. Clinical management of thalassemia major consists in regular long-life red blood cell transfusions and iron chelation therapy to remove iron introduced in excess with transfusions. Iron deposition in combination with inflammatory and immunogenic factors is involved in the pathophysiology of cardiac dysfunction in these patients. Heart failure and arrhythmias, caused by myocardial siderosis, are the most important life-limiting complications of iron overload in beta-thalassemia patients. Cardiac complications are responsible for 71% of global death in the beta-thalassemia major patients. The aim of this review was to describe the most frequent electrocardiographic abnormalities and arrhythmias observed in β-TM patients, analyzing their prognostic impact and current treatment strategies. PMID:27324981

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

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

    1996-10-01

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

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

    Tawhida Y. Abdel Ghaffar

    2011-12-01

    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.

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

    Mehran Karimi

    2008-09-01

    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.

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

    Fatemeh Farahmand; Vajiheh Modaresi; Mina Izadyar; Fatemeh Mahjob

    2010-01-01

    Background: Niemann-Pick disease and β-thalassemia are distinct conditions with specific clinical and morphological manifestations. β-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 β-thalassemia major was hospitalized for failure to thrive and hepathosplenomegaly. Because of unusual splenomegaly and liver enzymes disturbance tha...

  4. Xmni polymorphism and disease severity in patients with beta thalassemia from northern Pakistan

    Thalassemia is a heterogeneous disorder and several genetic factors influence the severity of thalassemia. An accurate and early diagnosis of a mild thalassemia genotype helps to avoid unnecessary transfusion and its complications. The aim of this study is to identify the association between XmnI polymorphism and disease severity in patients with ?-thalassemia from northern Pakistan. Methods: The cross sectional study was conducted at the Department of Haematology, Armed Forces Institute of Pathology (AFIP) Rawalpindi, from September 2006 to June 2009. A total of 90 subjects including 30 with thalassemia major, 30 with thalassemia intermedia and 30 normal individuals were studied. DNA from each subject was tested for 15 ?-thalassemia mutations and the presence of XmnI polymorphism using Amplification Refractory Mutation System and Restriction Fragment Length Polymorphism respectively. Results: One normal and one thalassemia major subject were found to be positive for homozygous and heterozygous XmnI polymorphism respectively. Among the thalassemia intermedia group, XmnI polymorphism was found in 12/30 patients, of whom 10 were homozygous and 2 were heterozygous for it. Conclusion: XmnI polymorphism is an important genotypic factor in Pakistani population for making a prospective diagnosis of thalassemia intermedia and predicting the severity of the disease. (author)

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

    Mina Izadyar

    2010-12-01

    Full Text Available Background: Niemann-Pick disease and β-thalassemia are distinct conditions with specific clinical and morphological manifestations. β-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 β-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.

  6. Assessment of the energy requirements and selected options facing major consumers within the Egyptian industrial and agricultural sectors. Final report

    1978-05-31

    The objectives of the energy assessment study of Egypt are to develop an understanding of the current status of the principal energy users in Egypt's industrial and agricultural sectors; to estimate the energy demand and efficiency for each selected subsector within these major sectors; to identify opportunities for fuel type changes, technology switches, or production pattern changes which might increase the efficiency with which Egypt's energy is used both now and in the future: and based on options identified, to forecast energy efficiencies for selected Egyptian subsectors for the years 1985 and 2000. Study results are presented for the iron and steel, aluminium, fertilizer, chemical, petrochemical, cement, and textile industries and automotive manufacturers. Study results for drainage, irrigation, and mechanization procedures in the agricultural sector and food processing sector are also presented. (MCW)

  7. STUDY OF SERUM HAPTOGLOBIN LEVEL AND ITS RELATION TO ERYTHROPOIETIC ACTIVITY IN BETA THALASSEMIA CHILDREN .

    Seham Ragab

    2015-02-01

    Full Text Available Background  :Serum haptoglobin (Hp is a reliable marker for hemolysis regardless the inflammatory state.  Objective: We investigated the possible relation between Hp depletion and hemolysis severity, hepatitis C virus (HCV infection and iron load in β-thalassemia children. Methods: Twenty  two β-thalassemia major (TM ,20 β-thalassemia  intermedia (TI children with 20 age and sex matched healthy controls were involved. Pre-transfusion hemoglobin level was considered . Serum ferritin , Hp  and transferrin receptor  levels (sTfR  (by ELISA , alanine aminotransferase (ALT and  aspartate aminotransferase (AST  (by colorimetric method were assayed. Markers of hepatitis C virus  (HCV  were done by PCR. Results:  The mean Hp levels among the studied groups were as follows; 8.02 ± 0.93 (mg/dl , 8.6 ±0.72 (mg/dl  and 122  ± 18.5(mg/dl   for TM ,TI and the controls respectively . Both patient groups had significantly lower Hp level compared to the controls (P<0.0001  with significant lower level in TM compared to TI  children ( P= 0.034  .Significant inverse correlations were  found between serum Hp and sTfR levels in thalassemia children combined and in each group (TM and TI as well as among HCV infected children. STfR   was the only significant independent predictor for  serum Hp level (t= -5.585 , P<0.0001 . Among  HCV infected patients , no significant correlation was found between serum Hp and serum transaminases  .Conclusion:  Serum Hp depletion in thalassemia had significant relation to disease severity and correlated   well with their erythropoietic activity, as assessed by the measurement of  sTfR without significant relation  HCV infection . Large sample  multicenter studies are  recommended.

  8. Beta Thalassemia (For Parents)

    ... Looking for Health Lessons? Visit KidsHealth in the Classroom What Other Parents Are Reading Zika & Pregnancy: What ... molecule that stores the genes that determine hair color, eye color, whether or not a person is ...

  9. Predictors of bone disease in Egyptian prepubertal children with β-thalassaemia major

    Tantawy, Azza A.G.; El-Bostany, Eman A.; Matter, Randa M; El-Ghoroury, Eman A.; Ragab, Shadia

    2010-01-01

    Introduction Thalassaemic osteopathy is a multifactorial disorder and limited information exists about bone accrual and bone mineral density (BMD) in prepubertal thalassaemic children. The study aimed to investigate some potential genetic and biochemical bone markers as possible early predictors of BMD variations in children with β-thalassaemia major (TM) before puberty. Material and methods Thirt-one prepubertal children with β-TM, and 43 matched controls were subjected to BMD assessment by ...

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

    Ridham A. Khanderia

    2015-06-01

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

  11. Hemoglobin Neapolis, beta 126(H4)Val----Gly: a novel beta-chain variant associated with a mild beta-thalassemia phenotype and displaying anomalous stability features.

    Pagano, L; Lacerra, G; Camardella, L; De Angioletti, M; Fioretti, G; Maglione, G; de Bonis, C; Guarino, E; Viola, A; Cutolo, R

    1991-12-01

    A novel beta-chain, beta 126(H4)Val----Gly, electrophoretically silent, was detected by reverse-phase high performance liquid chromatography in three unrelated families from Naples (Southern Italy) and accounted for about 30% of the total beta-chains. The amino acid substitution was detected by HPLC fingerprint. The eight heterozygous patients showed hematologic and biosynthetic alterations of mild beta-thalassemia type. The hemoglobin variant showed abnormal stability features. It was unstable in the heat stability and isopropanol precipitation tests, but did not cause a hemolytic syndrome in vivo and was stable in a time-course experiment of biosynthesis in vitro. DNA polymerase chain reaction direct sequencing of the mutated gene from 135 nt upstream of the cap site to 106 nt downstream of the polyadenylation site showed only the beta 126 GTG----GGG mutation, which was confirmed in the other patients by allele-specific oligonucleotide hybridization. The mutation was found to be associated with a type II beta-globin framework and restriction fragment length polymorphism haplotype V. The novel variant was named hemoglobin Neapolis. PMID:1954392

  12. Egyptian Constellations

    Lull, José; Belmonte, Juan Antonio

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

  13. Identification and quantification of the major constituents in Egyptian carob extract by liquid chromatography–electrospray ionization-tandem mass spectrometry

    Asmaa Ibrahim Owis

    2016-01-01

    Full Text Available Background: Carob - Ceratonia siliqua L., commonly known as St John's-bread or locust bean, family Fabaceae - is one of the most useful native Mediterranean trees. There is no data about the chromatography methods performed by high performance liquid chromatography (HPLC for determining polyphenols in Egyptian carob pods. Objective: To establish a sensitive and specific liquid chromatography–electrospray ionization (ESI-tandem mass spectrometry (MSn methodology for the identification of the major constituents in Egyptian carob extract. Materials and Methods: HPLC with diode array detector and ESI-mass spectrometry (MS was developed for the identification and quantification of phenolic acids, flavonoid glycosides, and aglycones in the methanolic extract of Egyptian C. siliqua. The MS and MSn data together with HPLC retention time of phenolic components allowed structural characterization of these compounds. Peak integration of ions in the MS scans had been used in the quantification technique. Results: A total of 36 compounds were tentatively identified. Twenty-six compounds were identified in the negative mode corresponding to 85.4% of plant dry weight, while ten compounds were identified in the positive mode representing 16.1% of plant dry weight, with the prevalence of flavonoids (75.4% of plant dry weight predominantly represented by two methylapigenin-O-pentoside isomers (20.9 and 13.7% of plant dry weight. Conclusion: The identification of various compounds present in carob pods opens a new door to an increased understanding of the different health benefits brought about by the consumption of carob and its products.

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

    Tiwari Aseem

    2009-01-01

    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.

  15. beta-thalassaemia major hos børn og unge i Danmark

    Jung, Anne; Main, Katharina Maria; Scheibel, Elma;

    2002-01-01

    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...... earlier and more effective iron chelation therapy together with improved patient support may reduce growth disturbances and endocrine and cardiac late effects....

  16. beta-thalassaemia major hos børn og unge i Danmark

    Jung, Anne; Main, Katharina Maria; Scheibel, Elma;

    2002-01-01

    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...... 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...

  17. Community genetics and health approaches for bringing awareness in tribals for the prevention of beta-thalassemia in India

    Ranbir S. Balgir

    2011-08-01

    Full Text Available Beta (β thalassemia syndromes are a group of hereditary disorders characterized by a genetic deficiency in the synthesis of β-globin chains. In the homozygous state, β-thalassemia (i.e., thalassemia major causes severe transfusion-dependent anemia. Inherited β-thalassemia syndromes cause high degree of hemolytic anemia, recurrent fever, clinical jaundice, frequent infections, bossing of cheek bones, growth retardation, splenomegaly, etc. and are responsible for high infant morbidity, mortality and fetal wastage in India. The victims include the infants, growing children, adolescent girls, pregnant women and a large chunk of ignorant people. In view of heavy genetic load, frequent requirement of blood transfusions, high cost of treatment and management, physical trauma, and mental and psychological harassment to the patients and their families, it has been realized that preventive community health and genetics approach is the most suitable for India. After carrier detection, prenatal diagnosis, and genetic couselling are the important options for couples at high risk for β-thalassemia. A prerequisite for successful prevention and intervention approach in India is the health education, bringing public awareness, sensitization, and community screening for the identification of heterozygotes or carriers in the concerned community. Some suggestions for the prevention of β-thalassemia in the vulnerable communities of India have been over emphasized for amelioration.β地中海贫血综合症是一簇遗传性异常,其特点是β球蛋白链接合处基因缺失。β地贫(或重型地贫)在纯和状态下导致严重的输液依赖型贫血症。遗传性的β地贫综合症引起严重的溶血性贫血、回归热、显性黄疸、常见感染、疼痛危象、颊骨浮肿、生长迟缓、脾肿大等症状,这导致在印度出现婴儿高发病率、死亡率和胎儿夭折。其受害人群包括婴儿、发

  18. Nonsense-mediated decay mechanism is a possible modifying factor of clinical outcome in nonsense cd39 beta thalassemia genotype

    Maria Concetta Renda

    2012-11-01

    Full Text Available Nonsense-mediated mRNA decay (NMD is a surveillance system to prevent the synthesis of non-functional proteins. In β-thalassemia, NMD may have a role in clinical outcome. An example of premature translation stop codons appearing for the first time is the β-globin cd39 mutation; when homozygous, this results in a severe phenotype. The aim of this study was to determine whether the homozygous nonsense cd39 may have a milder phenotype in comparison with IVS1,nt110/cd39 genotype. Genotypes have been identified from a cohort of 568 patients affected by β-thalassemia. These genotypes were compared with those found in 577 affected fetuses detected among 2292 prenatal diagnoses. The nine most common genotypes, each with an incidence rate of 1.5% or over, and together accounting for 80% of genotype frequencies, underwent statistical analysis. Genotype prevalence was calculated within the overall group. Results are expressed as proportions with 95% confidence intervals; P≤0.05 was considered statistically significant. A binomial distribution was assumed for each group; z-tests were used to compare genotype frequencies observed in the patient group with frequencies in the affected fetus group. In the absence of selecting factors, prevalence of these two genotypes was compared between a cohort of 568 β-thalassemia patients (PTS and 577 affected fetuses (FOET detected during the same period. IVS1,nt110/cd39 was significantly more prevalent in FOET than PTS (P<0.0001, while there was no significant difference in prevalence of cd39/cd39 in FOET compared with PTS (P=0.524. These results suggest a cd39 genotype NMD mechanism may be associated with improved clinical outcomes in thalassemia major. 无义介导的mRNA 降解(NMD) 是一种预防非功能性蛋白质合成的监控系统。在β地中海贫血中,NMD可能对临床结果有影响。第一次出现的过早终止密码子(PTC)为β珠蛋白cd39突变;若为纯合

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

    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

    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.)

  20. Moebius syndrome with macular hyperpigmentation, skeletal anomalies, and hypoplasia of pectoralis major muscle in an Egyptian child

    Rabah M. Shawky

    2015-07-01

    Full Text Available We report a 4 month old female infant, 3rd in order of birth of the first cousin consanguineous parents. The patient has congenital right facial nerve palsy, with asymmetry of facial expression during crying and difficulty in swallowing. Associated anomalies include abnormal facial features, bilateral finger anomalies, bilateral talipes equinovarus, kyphoscoliosis, hypotonia, high frequency hearing loss. Bilateral macular hyperpigmentation was detected in our patient on fundus examination which was not reported previously in Moebius syndrome cases. In addition there is hypoplasia of the right pectoralis major muscle.

  1. Impact of Genetic Polymorphism of methylenetetrahydrofolate reductase C677T on Development of Hyperhomocysteinemia and Related Oxidative Changes in Egyptian β-Thalassemia Major Patients.

    Mai A Abd-Elmawla

    Full Text Available β-thalasemia major (β-TM patients often suffer from various vascular complications together with increased oxidative stress. Hyperhomocysteinemia (Hhcy has been defined as a risk factor for these complications. Genetic polymorphism of methylenetetrahydrofolate reductase (MTHFR C677T has been shown to cause Hhcy particularly in individuals with low B-vitamins. However, the status of homocysteine (hcy in β-TM has not yet been adequately defined.To evaluate the genetic polymorphism of MTHFR C677T among β-TM patients and its prospective contribution to Hhcy and related oxidative changes.Genotyping for MTHFR C677T was done by PCR-RFLP technique. Plasma hcy, vitamin B12, folate, malondialdehyde (MDA, total antioxidant capacity (TAC, oxidized low density lipoprotein (oxLDL, total nitric oxide (NOx and lipid profile were determined in 66 β-TM patients and 66 control subjects of matched age and sex.The prevalence of MTHFR 677TT genotype was significant among β-TM patients (12% compared to (3% controls (OR = 4.9, 95%CI:1.2-24.2,P = 0.03. A strong association between Hhcy and MTHFR TT genotype was observed (OR = 7.7, 95%CI:2.8-20.9 where all β-TM patients with TT genotype were hyperhomocystienemic (≥ 15 μmol/l and having sub-optimal folate level than those with CT or CC genotypes. Hyperhomocystienemic patients have suffered from increased oxidative stress characterized by significant increase in plasma MDA and oxLDL, and a significant reduction of plasma TAC and total NOx. Lipid profile of those patients was severely affected indicated by reduction in HDL and HDL/LDL and elevation in atherogenic index as compared with CC genotype. Other measured parameters were not significantly different among β-TM patients with different MTHFR genotypes.This study suggests that Egyptian β-TM patients with MTHFR 677TT genotype could be at increasing risk of developing Hhcy particularly with folate deficiency. This state of Hhcy may account potentially for most

  2. Identification and Quantification of the Major Constituents in Egyptian Carob Extract by Liquid Chromatography–Electrospray Ionization-Tandem Mass Spectrometry

    Asmaa Ibrahim Owis; El-Motaz Bellah El-Naggar

    2016-01-01

    Background: Carob - Ceratonia siliqua L., commonly known as St John's-bread or locust bean, family Fabaceae - is one of the most useful native Mediterranean trees. There is no data about the chromatography methods performed by high performance liquid chromatography (HPLC) for determining polyphenols in Egyptian carob pods. Objective: To establish a sensitive and specific liquid chromatography–electrospray ionization (ESI)-tandem mass spectrometry (MSn) methodology for the identification of th...

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

    Taneja Rashi

    2010-01-01

    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.

  4. Egyptian Mythological Manuals

    Jørgensen, Jens Kristoffer Blach

    by the native priesthood, much of which has only been published in recent years. As part of this corpus of texts, the ancient Egyptian mythological manuals offer a unique perspective on how the Egyptian priesthood structured and interpreted Egyptian myths. The thesis looks at the different interpretative...... techniques used in the Tebtunis Mythological Manual (Second century CE) and the Mythological Manual of the Delta (Sixth century BCE) and the place of these manuals within the larger corpus of priestly scholarly literature from ancient Egypt. To organize the wealth of local myths the manuals use model...

  5. Egyptian Arab Republic

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

  6. High Prevalence of Alpha- and Beta-Thalassemia in the Kadazandusuns in East Malaysia: Challenges in Providing Effective Health Care for an Indigenous Group

    Tan, Jin-Ai Mary Anne; Lee, Ping-Chin; Wee, Yong-Chui; Tan, Kim-Lian; Mahali, Noor Fadzlin; George, Elizabeth; Chua, Kek-Heng

    2010-01-01

    Thalassemia can lead to severe transfusion-dependent anemia, and it is the most common genetic disorder in Malaysia. This paper aims to determine the prevalence of thalassemia in the Kadazandusuns, the largest indigenous group in Sabah, East Malaysia. α- and β-thalassemia were confirmed in 33.6% and 12.8%, of the individuals studied respectively. The high prevalence of α- and β-thalassemia in the Kadazandusuns indicates that thalassemia screening, genetic counseling, and prenatal diagnosis should be included as part of their healthcare system. This preliminary paper serves as a baseline for further investigations into the health and genetic defects of the major indigenous population in Sabah, East Malaysia. PMID:20871816

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

    Rehman Anis

    2011-08-01

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

  8. Sit Like an Egyptian

    Moll, Emily

    2012-01-01

    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…

  9. D-dimer and portal vein status in splenectomized Egyptian β-thalassemia major patients: a prospective single-thalassemia center experience.

    Elalfy, Mohsen Saleh; Andrawes, Nevine Gamal; Sadek, Azza Mohamad; Hussein, Omar; Abdou, Abeer

    2012-04-01

    Splenectomy is a recognized cause of portal vein thrombosis. Thirty-six β-thalassemia major (β-TM) patients were followed up for 36 months to evaluate changes in D-dimer levels (as a possible marker for thrombosis development) and portal vein status (by portal duplex ultrasound) at both early and late postlaparoscopic splenectomy periods. They were classified into group I if they were splenectomized in the study period (n = 12), or group II if they were splenectomized during the 5 years preceding the period (n = 24). In group I, D-dimer was measured 5 times: 1 day presplenectomy, the 1st week, 6th week, and 6th month postsplenectomy, and at the study end, whereas in group II, D-dimer was measured twice: at the study entry and end. Portal duplex was done 1 week postsplenectomy (group I) and at study end in both groups. Presplenectomy D-dimer levels in group I were significantly higher compared with the 6th month (P = .042) and study end (P = .03), whereas 1st week (postsplenectomy) D-dimer levels had a high mean of 3497.3 ng/mL, lowered at the 6th week (P = .017), at the 6th month (P = .008), and at study end (P = .005). D-dimer levels in group II showed no difference between study entry and end (P = .104). Portal vein "diameter and flow" were within normal findings in both groups. In this 3-year prospective study, a subclinical hypercoagulable state was detected 1 day prior to splenectomy and in the early postsplenectomy period, as evidenced by high D-dimer levels. Laparoscopic splenectomy was not associated with portal venous thrombosis either clinically or by duplex sonography. PMID:22475301

  10. Vitamin D Receptor (VDR) Gene Polymorphisms (FokI, BsmI) and their Relation to Vitamin D Status in Pediatrics βeta Thalassemia Major.

    Elhoseiny, Shereen Mohamed; Morgan, Dalia Saber; Rabie, Asmaa Mohamed; Bishay, Samer Tharwat

    2016-06-01

    Vitamin D is critical for calcium, phosphate homeostasis and for mineralization of the skeleton, especially during periods of rapid growth. Vitamin D Deficiency leads to rickets (in children) and osteomalacia (in adults). Expression and activation of the vitamin D receptor (VDR) are necessary for the effects of vitamin D, in which several single nucleotide polymorphisms have been identified especially (FokI, BsmI). In this study serum 25 (OH) vitamin D3 levels were estimated by Enzyme Linked Immunosorbent Assay [ELISA], VDR (FokI, BsmI) gene polymorphisms were analyzed by polymerase chain reaction-restriction fragment length polymorphism assay [PCR-RFLP].Serum levels of calcium, phosphorus, alkaline phosphatase and ferritin were determined in 50 Pediatrics beta thalassemia major patients and 60 controls. Patients had significantly lower serum calcium (p serum vitamin D3 (p 30 ng/ml). Patients harboring mutant (Ff,ff) and wild (BB) genotypes were associated with lower serum calcium (p = 0.08, 0.02) respectively, lower vitamin D3 levels (p  0.05). In conclusion, these results suggest that the VDR (FokI, BsmI) gene polymorphisms influence vitamin D status, (Ff,ff), BB genotypes had lower vitamin D levels, so they might influence risk of development of bone diseases in beta thalassemia major. PMID:27065588

  11. The Ancient Egyptian Demonology Project

    Weber, Felicitas

    2016-01-01

    “The Ancient Egyptian Demonology Project: Second Millennium BCE” was intended and funded as a three-year project (2013-2016) to explore the world of Ancient Egyptian demons in the 2nd millennium BC. It intends to create a classification and ontology of benevolent and malevolent demons. Whereas ancient Egyptians did not use a specific term denoting “demons”, liminal beings known from various other cultures such as δαίμονες, ghosts, angels, Mischwesen, genies, etc., were nevertheless described ...

  12. Credit risk in Egyptian banks

    Abdou, Hussein

    2006-01-01

    This research aims to identify the currently used techniques in evaluating credit risk in Egypt’s banking sector, then to evaluate these techniques and to develop model(s) to evaluate credit risk in Egyptian banks. In a pilot study, the researcher conducted informal interviews with key personnel in three of the Egyptian banks, in order to evaluate credit risk policies. The pilot study showed that these banks do not use any of the statistical techniques in the evaluation pr...

  13. The Petrie Museum of Egyptian Archaeology, UCL

    Jan Picton

    2013-10-01

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

  14. Effects of blood transfusion on exercise capacity in thalassemia major patients.

    Daniela Benedetto

    Full Text Available Anemia has an important role in exercise performance. However, the direct link between rapid changes of hemoglobin and exercise performance is still unknown.To find out more on this topic, we studied 18 beta-thalassemia major patients free of relevant cardiac dysfunction (age 33.5±7.2 years,males = 10. Patients performed a maximal cardiopulmolmonary exercise test (cycloergometer, personalized ramp protocol, breath-by-breath measurements of expired gases before and the day after blood transfusion (500 cc of red cell concentrates. After blood transfusion, hemoglobin increased from 10.5±0.8 g/dL to 12.1±1.2 (p<0.001, peak VO2 from 1408 to 1546mL/min (p<0.05, and VO2 at anaerobic threshold from 965 to 1024mL/min (p<0.05. No major changes were observed as regards heart and respiratory rates either at peak exercise or at anaerobic threshold. Similarly, no relevant changes were observed in ventilation efficiency, as evaluated by the ventilation vs. carbon dioxide production relationship, or in O2 delivery to the periphery as analyzed by the VO2 vs. workload relationship. The relationship between hemoglobin and VO2 changes showed, for each g/dL of hemoglobin increase, a VO2 increase = 82.5 mL/min and 35 mL/min, at peak exercise and at anaerobic threshold, respectively. In beta-thalassemia major patients, an acute albeit partial anemia correction by blood transfusion determinates a relevant increase of exercise performance, observed both at peak exercise and at anaerobic threshold.

  15. The Iranian Legacy in the 2011 Egyptian Revolution: Military Endurance and US Foreign Policy Priorities

    Alimagham, Pouya

    2013-01-01

    In the latter half of the twentieth century, militaries have been a major source for change in the Middle East.  In 1952, radical nationalist military officers staged the overthrow of the Egyptian monarchy and proclaimed a republic. A year later, the Iranian military, in collusion with the American CIA and the British MI-6, toppled Iran’s democratically-elected government. In the same decade, Iraqi military officers, following on the heels of their Egyptian counterparts, ousted the monarchy i...

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

    Shah Neeraj

    2010-01-01

    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

  17. [Ancient Egyptian Odontology].

    Berghult, B

    1999-01-01

    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

  18. The Islamist Trend in Egyptian Law

    Tamir Moustafa

    2010-01-01

    The past four decades have witnessed profound transformations in the Egyptian legal system and in the Egyptian legal profession. Article 2 of the Egyptian Constitution now enshrines Islamic jurisprudence as the principle source of law, thus establishing an important symbolic marker at the heart of the state and opening avenues for Islamist activists to press litigation campaigns in the courts. Additionally, the Islamist trend gained prominence within the legal profession, a development that i...

  19. Financing Resources for Egyptian Small and Medium Enterprises

    Rola Nabil El Kabbani; Christian Kalhoefer

    2011-01-01

    Even though small and medium enterprises (SMEs) represent the majority of the Egyptian firms, their value added is relatively low. According to previous research, SMEs face a financing gap that causes undercapitalization and represents an obstacle that hinders SMEs from growth. This paper analyzes the current situation of SMEs in Egypt and addresses the question whether the financing problem still exists. In addition, we discuss the role of venture capital as a possible source of financing. B...

  20. Thermoluminescence (TL) of Egyptian Blue

    Schvoerer, M.; Delavergne, M.-C.; Chapoulie, R.

    1988-01-01

    Egyptian Blue is a synthesized crystalline pictorial pigment with formula CaCuSi/sub 4/O/sub 10/. 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 ..beta.. irradiation (/sup 90/Sr). 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.

  1. An Egyptianizing relief from Malta

    Bonanno, Anthony

    1998-01-01

    From the very first announcement of the theme of this congress it was evident that the "Egyptianizing" phenomenon would be one of the most recurring topics in the diverse contributions, especially those concerned with cultural aspects, such as art and religion, outside Egypt itself. It would be presumptuous of me, therefore, and futile, even to try to define the phenomenon. At this stage I would only wish to emphasize the distinction between a) the more ancient version of the "...

  2. Some endocrinal aspects of pancreas in beta thalassemia

    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

  3. Phase I/II Pilot Study of Mixed Chimerism to Treat Hemoglobinopathies

    2016-05-02

    Anemia, Sickle Cell; Complex and Transfusion-dependent Hemoglobinopathies; Thalassemia; Alpha or Beta Thalassemia Major; Diamond-Blackfan Anemia; Bone Marrow Failure Syndromes Characterized by Severe Chronic Anemia

  4. Pharmacoeconomic Education in Egyptian Schools of Pharmacy

    Soliman, Ahmed M.; Hussein, Mustafa; Abdulhalim, Abdulla M.

    2013-01-01

    Objective. To investigate the status of pharmacoeconomics education in Egyptian schools of pharmacy and compile and construct recommendations on how Egypt and similar countries could improve their educational infrastructure in pharmacoeconomics.

  5. The Future of SMEs in Egyptian Economy

    Alasrag, Hussien

    2007-01-01

    Small and Medium Enterprises (SMEs) in Egypt represent the greatest share of the productive units of the Egyptian economy, the current national policy directions address ways and means of developing the capacities of SMEs. This paper reviews the role of The Developmental Role of SMEs in Egyptian Economy. IT attempts at identifying the broad parameters within which an integrated government policy towards SME development can be formulated. The paper identifies the financial and non-financial co...

  6. The Archaeology of Egyptian Monasticism

    Blanke, Louise

    The study of Egyptian monasticism has traditionally relied heavily on the rich corpus of textual sources, while the archaeological remains have been secondary to our understanding of monastic life. This imbalance has resulted in a situation where questions pertinent to the physical remains of...... monasteries ha ve largely remained unanswered. Based on first - hand archaeological material from the White Monastery federation and comparative material obtained through archaeological reports, the thesis addresses Egypt ian Monasticism in the transition from Late Antiquity to the Early Islamic period, by...... examining three main themes through seven chapters. These themes are: 1. the relationship between the archaeological and textual sources pertinent to the White Monastery; 2. the diachronic development of the White Monastery and the process es that caused its abandonment; 3. the economy of the White...

  7. Radiological assessment of the Egyptian Mediterranean coast

    On the basis of extensive sampling, gamma spectrometry, laboratory analyses, data evaluation and comparison with the reference data, the following assessment has been carried out. The radiological assessment has been done for a wide scale study area (about 1100 km) from El- Salloum in the west of the Mediterranean coast of Egypt to El-Arish in the east. The environmental radiological assessment steps are based on the criteria previously mentioned. The sources of radioactivity possibly reach the Egyptian Mediterranean coast can be summarized as: primordial and radiogenic radionuclides (e.g. 40K, 238U and 232Th series), cosmic rays and cosmogenic radionuclides (e.g. 7Be), fertilizers (e.g. superphosphate), black sand (transported by Nile River), fallout (either from nuclear testing or Chernobyl), seawater currents (transported either natural or man-made), Suez Canal (subject to receive a radioactive releasing and effluents from either nuclear power ships or submarines passes through the canal), biological migration and sedimentary longshore movement (e.g. Anguilla anguilla), atmospheric radioactivity (e.g. 222Rn and its daughters), domestic and medical sewage (minor source, short half-life), depleted uranium dust (possibly), satellite and aircraft accident (accidentally) and rarely loss of industrial radioactive source (incidentally). El-Salloum, Rashid and El-Gamil have been considered as concentrated basins for the majority of the radioisotopes. This suggestion was due to the special topographical features of these three stations, which make the accumulation of the radioactive isotopes possible. Calculations of outdoor absorbed dose rate for human population at all stations under investigation from 238U, 232Th and 40K were carried out. The calculated absorbed dose rate has been distinguished the coast into normal areas and Rashid black sand area as high background area. The range of calculated dose was 8.39-38.5 μGy/h. Good agreement was observed with NCRP

  8. Radiological assessment of the Egyptian Mediterranean Coast

    On the basis of extensive sampling, gamma spectrometry, laboratory analyses, data evaluation and comparison with the reference data, the following assessment has been carried out. The radiological assessment has been done for a wide scale study area (about 1100 km) from El-Salloum in the west of the Mediterranean coast of Egypt to El-Arish in the east. The environmental radiological assessment steps are based on the criteria previously mentioned. The sources of radioactivity possibly reach the Egyptian Mediterranean coast can be summarized as: primordial and radiogenic radionuclides (e.g. 40K, 23'8U and 232Th series), cosmic rays and cosmogenic radionuclides (e.g. 7Be), fertilizers (e.g. superphosphate), black sand (transported by the Nile River), fallout (either from nuclear testing or Chernobyl), seawater currents (transported either natural or man-made), Suez Canal (subject to receive a radioactive releasing and effluents from either nuclear power ships or submarines passing through the Canal), biological migration and sedimentary longshore movement (e.g. Anguilla anguilla), atmospheric radioactivity (e.g. 222Rn and its daughters), domestic and medical sewage (minor source, short half-life), depleted uranium dust (possibly), satellite and aircraft accident (accidental) and rarely loss of industrial radioactive source (incidentally). El-Salloum, Rashid and El-Gamil have been considered as concentrated basins for the majority of the radioisotopes. This suggestion was due to the special topographical features of these three stations, which make the accumulation of the radioactive isotopes possible. Calculations of outdoor absorbed dose rate for human population at all stations under investigation from 238U, 232Th and 40K were carried out. The calculated absorbed dose rate has been distinguished the coast into normal areas and Rashid black sand area as high background area. The range of calculated dose was 8.39-38.5 nGy/h. Good agreement was observed with NCRP

  9. Orientation of Egyptian Temples: An Overview

    Belmonte, Juan Antonio

    Archaeoastronomy has never been a favored discipline within Egyptology. As a consequence, important questions such as the orientation of Egyptian temples and the relevance of astronomy in this respect had not been treated with the requisite seriousness and depth. This situation is changing, however, and over the past decade, there have been several serious attempts to perform an extensive analysis of the orientation of Egyptian monuments. The orientations of approximately 400 temples have been measured in the Nile Valley, the Delta, the Oases, and the Sinai, with the aim of providing a clear answer to the question of whether the ancient Egyptian sacred constructions were astronomically aligned or not. This impressive set of data seems to answer this question in the affirmative.

  10. STREPTOMYCETE Producing Antibiotics Isolated From Egyptian Soil

    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

  11. Rare Earth Elements in Egyptian Phosphorites

    I.S. ISMAEL

    2002-01-01

    Egyptian phosphorites from Abu Tartur (Western Desert), El Mahamide mine (Nile Valley) and Rabah mine (Eastern Desert) show variable degrees of relative REE enrichment.Black plateau phosphorites of Abu Tartur are substantially enriched in REE as compared to the Red Sea and Nile Valley phosphorites. P-rich organic matter from the Abu Tartur and Rabah mines recorded negative Ce and positive Eu anomalies. Positive Eu anomaly reveals an anoxic event prior to the phase of Late Cretaceous phosphate formation. Ce is a redox indicator. Mixing of sea water and upwelling during the Late Cretaceous was responsible for the recording of positive Eu and negative Ce anomalies in the Egyptian phosphorites.

  12. Classification of sharks in the Egyptian Mediterranean waters using morphological and DNA barcoding approaches.

    Marie Moftah

    Full Text Available The identification of species constitutes the first basic step in phylogenetic studies, biodiversity monitoring and conservation. DNA barcoding, i.e. the sequencing of a short standardized region of DNA, has been proposed as a new tool for animal species identification. The present study provides an update on the composition of shark in the Egyptian Mediterranean waters off Alexandria, since the latest study to date was performed 30 years ago, DNA barcoding was used in addition to classical taxonomical methodologies. Thus, 51 specimen were DNA barcoded for a 667 bp region of the mitochondrial COI gene. Although DNA barcoding aims at developing species identification systems, some phylogenetic signals were apparent in the data. In the neighbor-joining tree, 8 major clusters were apparent, each of them containing individuals belonging to the same species, and most with 100% bootstrap value. This study is the first to our knowledge to use DNA barcoding of the mitochondrial COI gene in order to confirm the presence of species Squalus acanthias, Oxynotus centrina, Squatina squatina, Scyliorhinus canicula, Scyliorhinus stellaris, Mustelus mustelus, Mustelus punctulatus and Carcharhinus altimus in the Egyptian Mediterranean waters. Finally, our study is the starting point of a new barcoding database concerning shark composition in the Egyptian Mediterranean waters (Barcoding of Egyptian Mediterranean Sharks [BEMS], http://www.boldsystems.org/views/projectlist.php?&#Barcoding%20Fish%20%28FishBOL%29.

  13. ICT BASED TELEMEDICINE FOR THE EGYPTIAN SOCIETY

    Hafez A. Fouad

    2013-12-01

    Full Text Available The One of the most challenging problems that encounter the Egyptian society is the lack of significant health care in the rural areas. This problem leads to more severe problems that face the society; the patients from the different rural areas needs to travel to the Egyptian capital where the most experienced physicians are available. This will make overhead not only on the patient budget but on the country budget since the focus on the capital makes a severe traffic problem which threaten most of the economic sectors. The telemedicine is considered one of the most important solutions that could mitigate the accumulated problems of lack of experienced physicians in the Egyptian rural areas. The application of the telemedicine encounters several challenges in Egypt; the lack in the experience in dealing with the telemedicine in these areas and the problem of insufficient medical experts that could fulfil the gab. In this paper, a new ICT-based telemedicine system is proposed to serve the Egyptian society. The portal is already released and snapshots are included

  14. Islam in Egyptian Education: Grades K-12

    Neill, Charlotte M.

    2006-01-01

    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…

  15. Hair-offerings: an enigmatic Egyptian custom

    G. J. Tassie

    1996-11-01

    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.

  16. Moessbauer effect study of ancient Egyptian pottery

    Moessbauer spectroscopy was used in examining ancient Egyptian pottery. From the values of Moessbauer parameters and the differences for the individual samples, conclusions could be drawn as to the temperature of baking and the kind of clay used in various archaeological periods. (A.K.)

  17. Moessbauer spectroscopic analysis of ancient Egyptian pottery

    Ten pieces of Egyptian pottery ware and eleven silt samples collected at Hierakonopolis (Nile River, Egypt) were studied by Moessbauer spectroscopy. Three Nile silt samples and three pottery sherds were test fired and refired in an oxidized atmosphere up to 11000C. Changes of the Moessbauer parameters depend upon the firing temperatures as well as the firing atmosphere. (Auth.)

  18. A probabilistic model of Ancient Egyptian writing

    Nederhof, Mark Jan; Rahman, Fahrurrozi

    2015-01-01

    This article investigates a probabilistic model to describe how signs form words in Ancient Egyptian writing. This applies to both hieroglyphic and hieratic texts. The model uses an intermediate layer of sign functions. Experiments are concerned with finding the most likely sequence of sign functions that relates a given sequence of signs and a given sequence of phonemes. Postprint

  19. ANTHROPOMETRIC STUDY OF NASAL INDEX OF EGYPTIANS

    Abdelmonem Awad Hegazy

    2014-12-01

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

  20. The Islamist Trend in Egyptian Law (SWP 2)

    Moustafa, Tamir

    2010-01-01

    The past four decades have witnessed profound transformations in the Egyptian legal system and in the Egyptian legal profession. Article two of the Egyptian Constitution now enshrines Islamic jurisprudence as the principle source of law, thus establishing an important symbolic marker at the heart of the state and opening avenues for Islamist activists to press litigation campaigns in the courts. Additionally, the Islamist trend gained prominence within the legal profession, a development that...

  1. Ownership Structure and Firm Performance in the Egyptian Manufacturing Sector

    Abdelgouad, Ahmed Fayez; Pfeifer, Christian; John P. Weche Gelübcke

    2014-01-01

    We use the World Bank enterprise survey for the Egyptian manufacturing sector to study the correlation between the ownership structure (private vs. public, Egyptian vs. Arab foreign vs. non-Arab foreign) and firm performance, which we measure as sales per worker, capacity utilization, and net profit rate. Our main findings indicate that (1) productivity differences between Egyptian private and public firms are not significant, but firms with public ownership have a lower capacity utilization ...

  2. Investigating the use of Egyptian blue in Roman Egyptian portraits and panels from Tebtunis, Egypt

    Ganio, Monica; Salvant, Johanna; Williams, Jane; Lee, Lynn; Cossairt, Oliver; Walton, Marc

    2015-11-01

    The use of the pigment Egyptian blue is investigated on a corpus of fifteen mummy portraits and Roman-period paintings from Tebtunis, Egypt, housed in the Phoebe A. Hearst Museum of Anthropology at the University of California, Berkeley. Egyptian blue has a strong luminescence response in the near infrared that can be exploited to created wide-field images noninvasively showing the distribution of the pigment on a work of art. A growing body of publications in the last decade highlights the increasing use of this tool and its sensitive detection limits. However, the technique is not wavelength specific. Both excitation and emission occur in a broad range. Although Egyptian blue has a strong emission in the NIR, a myriad of other compounds may emit light in this spectral region when excited in the visible. The limited number of studies including complementary analysis to verify the presence of Egyptian blue does not allow its identification on the basis of NIR luminescence alone. Through the use of in situ X-ray fluorescence and X-ray diffraction, and scanning electron microscopy/energy-dispersive spectroscopy of cross sections, this paper confirms the identification of Egyptian blue by NIR luminescence in unexpected areas, i.e., those not blue in appearance.

  3. Contributions of Egyptian Women in Physics (abstract)

    El Nadi, Lotfia

    2009-04-01

    Physics is a dynamic, global field. Progress in research motivates scientists to explore new areas and find useful applications for their work. Femtosecond ultrashort pulsed lasers and progress in nanostructures to study the properties of extremely dense matter, as well as one-dimensional materials, are two examples of innovations that encourage students and scientists—male and female—to pursue physics. Young Egyptian women's contributions to physics grew from 46% in 2003 to 69% in 2008. This paper discusses the role of women in physics in Egypt; presents statistics regarding their contributions and presence at Egyptian universities and institutes; and gives information about their decision making leadership roles. Ideas, applicable in Egypt as well as in developing countries, to address problems facing women are raised.

  4. Rare Earth Elements in Egyptian Phosphorites

    I.S.ISMAEL

    2002-01-01

    Egyptian phosphorites from Abu Tartur(Western Desert),El Mahamide mine(Nile Valley) and Rabah mne(Eastern Desert)show variable degrees of relative REE enrichment.Black plateau phosphorites of Abu Tartur are substantially enriched in REE as compared to the Red Sea and Nile Valley phosphorites.P-rich organic matter from the Abu Tartur and Rabah mines recorded negative Ce and positive Eu anomalies.Positive Eu anomaly reveals an anoxic event prior to the phase of Late Cretaceous phosphate formation.Ce is a redox indicator.Mixing of sea water and upwelling during the Late Cretaceous was responsible for the recording of positive Eu and negative Ce anomalies in the Egyptian phosphorites.

  5. The thermoluminescence (TL) of Egyptian Blue

    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 β 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)

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

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

    2015-06-01

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

  7. The Egyptian Red Sea coastal microbiome: A study revealing differential microbial responses to diverse anthropogenic pollutants.

    Mustafa, Ghada A; Abd-Elgawad, Amr; Ouf, Amged; Siam, Rania

    2016-07-01

    The Red Sea is considered one of the youngest oceanic systems, with unique physical, geochemical and biological characteristics. Tourism, industrialization, extensive fishing, oil processing and shipping are extensive sources of pollution in the Red Sea. We analyzed the geochemical characteristics and microbial community of sediments along the Egyptian coast of the Red Sea. Our sites mainly included 1) four ports used for shipping aluminum, ilmenite and phosphate; 2) a site previously reported to have suffered extensive oil spills; and 3) a site impacted by tourism. Two major datasets for the sediment of ten Red Sea coastal sites were generated; i) a chemical dataset included measurements of carbon, hydrogen, nitrogen and sulfur, metals and selected semi-volatile oil; and ii) a 16S rRNA Pyrotags bacterial metagenomic dataset. Based on the taxonomic assignments of the 16S rRNA Pyrotags to major bacterial groups, we report 30 taxa constituting an Egyptian Red Sea Coastal Microbiome. Bacteria that degrade hydrocarbons were predominant in the majority of the sites, particularly in two ports where they reached up to 76% of the total identified genera. In contrast, sulfate-reducing and sulfate-oxidizing bacteria dominated two lakes at the expense of other hydrocarbon metabolizers. Despite the reported "Egyptian Red Sea Coastal Microbiome," sites with similar anthropogenic pollutants showed unique microbial community abundances. This suggests that the abundance of a specific bacterial community is an evolutionary mechanism induced in response to selected anthropogenic pollutants. PMID:27179234

  8. Fifteen years experience: Egyptian metabolic lab

    Ekram M. Fateen

    2014-10-01

    Conclusion: This study illustrates the experience of the reference metabolic lab in Egypt over 15 years. The lab began metabolic disorder screening by using simple diagnostic techniques like thin layer chromatography and colored tests in urine which by time updated and upgraded the methods to diagnose a wide range of disorders. This study shows the most common diagnosed inherited inborn errors of metabolism among the Egyptian population.

  9. Curse of schistosomiasis on Egyptian liver

    Abdel-Rahman El-Zayadi

    2004-01-01

    @@ INTRODUCTION Schistosomiasis is a chronic parasitic disease caused by a trematode blood fluke of the genus schistosoma that belongs to the schistosomatidae family.The ancient Egyptians contracted the disease more than 4 000 years ago.It was recognized through haematuria,the main sign of urinary bilharziasis was recorded in the Kahun papyrus 1900 B.C.as"a-a-a" disease[1].

  10. Egyptian plant species as new ozone indicators

    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 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

  11. Enhancement of Nuclear Security Measures: Egyptian Case

    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)

  12. Body composition in Egyptian Turner syndrome girls

    Zaki, Moushira Erfan; Afifi, Hanan H.

    2013-01-01

    OBJECTIVE: This cross-sectional study was undertaken to construct the new body fat % curve and provide body composition reference data for adolescent girls with Turner syndrome (TS). They diagnosed cytogenetically by blood karyotyping and not treated with growth hormone (GH). MATERIALS AND METHODS: The study included 70 TS girls from age 13 years to age 17 years. Body composition was measured by bioelectrical impedance. Smoothed centile charts were derived by using the least mean square (LMS) method. RESULTS: The new body fat curves reflect the increase of body fat mass (FM) from age 13 years to age 17 years. Body FM % of Egyptian TS girls was lower when compared with age-matched American untreated TS girls. CONCLUSION: This study presents the new body fat curves and reference values of body composition for untreated Egyptian TS adolescent girls. The present charts can be used for direct assessment of body FM % for Egyptian TS girls and evaluation for cases on GH treatment or other growth promoting therapy. PMID:24019614

  13. Body composition in Egyptian Turner syndrome girls

    Moushira Erfan Zaki

    2013-01-01

    Full Text Available Objective: This cross-sectional study was undertaken to construct the new body fat % curve and provide body composition reference data for adolescent girls with Turner syndrome (TS. They diagnosed cytogenetically by blood karyotyping and not treated with growth hormone (GH. Materials and Methods: The study included 70 TS girls from age 13 years to age 17 years. Body composition was measured by bioelectrical impedance. Smoothed centile charts were derived by using the least mean square (LMS method. Results: The new body fat curves reflect the increase of body fat mass (FM from age 13 years to age 17 years. Body FM % of Egyptian TS girls was lower when compared with age-matched American untreated TS girls. Conclusion: This study presents the new body fat curves and reference values of body composition for untreated Egyptian TS adolescent girls. The present charts can be used for direct assessment of body FM % for Egyptian TS girls and evaluation for cases on GH treatment or other growth promoting therapy.

  14. Cartoons and the Egyptian Transition: A Qualitative Analysis of Egyptian Newspapers

    Sara S. Elmaghraby

    2014-07-01

    Full Text Available Since the Egyptian revolution of 25 January, cartoonists have depicted the transition taking place in this country in several ways. This study aims to analyze the cartoons drawn about this transition period in Egypt in seven different Egyptian newspapers. This is done through a qualitative analysis of 80 cartoons published between 28 June and 4 July 2013, as this period saw the ousting of elected president Mohamed Morsi by the military general Abdel Fattah Al-Sisi. The study observes the different depiction of various topics, actors and visual frames used by the seven newspapers during the analyzed period, in accordance with their particular viewpoint of the transition in Egypt.

  15. Egyptian Romanized Arabic : a study of selected features from communication among Egyptian youth on Facebook

    2010-01-01

    Egyptian Romanized Arabic (ERA) is the Egyptian variety of the writing that arose when Arabic speakers joined the domain of computer-mediated communication. At a time when almost all text on the Web was in English, and only basic Roman letters were supported, they had to use the Roman script to communicate with each other in Arabic. Based on data from more than one hundred users, I hoped to draw a picture of how ERA is written in general. As there is no official orthography or spelling rules ...

  16. Egyptian Art Institutions and Art Education from 1908 to 1951

    Kane, Patrick

    2010-01-01

    This study of Egyptian aesthetics interprets the historical and political context of artistic discourse in the early twentieth century. In a period marked by intense struggle between landlords and rural laborers during the Depression and World War II, the author compares the rise of the Egyptian Surrealists, from the late 1930s, and the…

  17. A new look at old bread: ancient Egyptian baking

    Delwen Samuel

    1999-11-01

    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.

  18. The Sine-Egyptian Joint Business Council Business Forum Held

    2009-01-01

    @@ On December 25, Chairman Wan Jifei led the Chinese business delegation attending the Sino-Egyptian Joint Business Council Business Forum co-hosted by the CCPIT and the Sino-Egyptian Business Council in Cairo, Egypt. Chinese vice Premier Li Keqiang who was on a visit to Egypt attended the forum and addressed the opening ceremony.

  19. A new look at old bread: ancient Egyptian baking

    Delwen Samuel

    1999-01-01

    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.

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

    Karim MAMDOUH ABBAS

    2014-04-01

    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.

  1. German-Egyptian seminar on environmental research

    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)

  2. Electrical properties of Egyptian natural graphite

    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

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

    1993-01-01

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

  4. Physicochemical and radiological studies on some egyptian aquatic areas

    For the purpose of estimating the radiological health and safety to the Egyptian population arises from the natural background involved with coastal areas, it is necessary to make a comprehensive study to investigate the natural radionuclides activities in this environmental ecology. The locations of such areas were chosen from those in which people spend some time (e.g. the Mediterranean and Red Sea beaches, Suez Canal in addition to the River Nile Ismailia Canal and Quaroun Lake). Water and soil samples were brought from these locations and subjected to intensive physical, chemical analysis and radiological investigations. The densities of the collected soil samples were found to fall in the region of clay densities (2.30 - 2.70 g/cm 3 ). The particle size distribution analysis, of these soil samples showed values ranging from 128 Μ m to 1303 Μ according to sample site. The collected samples are mainly silicates with variable amounts of the major cations; Ca++, Mg++, Na+, K+ with some other trace elements. For the radiological investigations 238U, 226Ra, 232Th and 40K activity concentrations were resolved by spectroscopy technique. The majority of samples examined in this work showed variable activities of the naturally occurring radionuclides with fairly low levels compared with literature values reported in other countries. The analysis and discussions of the obtained data are thoroughly performed with the use of equations to estimate the radiation doses

  5. Studies of natural radioactivity of some Egyptian rock phosphates

    226Ra, 235U, 232Th and 40K activity concentrations (Bq/kg) in some Egyptian phosphate samples have been measured using HPGe gamma spectrometer. Also their radium equivalent activities were calculated and discussed. (author)

  6. General fire protection guidelines for Egyptian nuclear installations

    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)

  7. Egyptian international labor migration and social processes: toward regional integration.

    Sell, R R

    1988-01-01

    This article reviews evidence that contemporary Egyptian international labor migration to oil-rich Arab countries has followed a classic social process which starts with a homo economicus phase, advances into a goal reorientation phase, and ends with the establishment of diaspora communities in destination societies. The history of Egyptian migration, current estimates of migration, the role of Egyptians in selected Arab countries, and emergent processes all were found to support the predictions of the social process model. Particularly important support comes from the finding that all social classes participated in this migration. For 1982, the Ministry for Foreign Affairs, based on individual consulate figures, reported 2.9 million migrants in oil-rich countries. Conclusions suggest the likelihood that Egyptian migration processes will promote economic and perhaps social integration in the region. PMID:12281731

  8. Did the ancient Egyptians migrate to ancient Nigeria?

    Jock M. Agai

    2014-01-01

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

  9. Application of Moessbauer spectroscopy in investigating Egyptian archaeology

    This article summarizes the results of applications of the Moessbauer spectroscopy to investigate Ancient Egyptian pottery from the periods: Ancient Egyptian (3200-525 B.C.), Greek-Roman (320 B.C.-640 A.C.) and Early Islamic (800-1000 A.C.). Many objective informations deduced about: provinance, manufacturing techniques for different domestic purposes, civilization transfer between the Arab countries, methods of colouration and applying decorating glazes, and finally dating of ancient pottery. (orig.)

  10. Connecting Philosophy of Ancient Egyptians to Modern Thinking

    Aminuddin Hassan; Nurul A.A.K. Anuar; Norhasni Z. Abiddin

    2012-01-01

    Problem statement: Associating any knowledge from ancient Egyptians to modern civilization and thinking was important and had its own value. The process of understanding knowledge related to ancient Egyptians is actually based on the nature of philosophical thought. Approach: In the discussion of ancient Egypt philosophy, it is important to look at it from the perspectives of the four branches of philosophy; metaphysics, epistemology, axiology and logic. Metaphysics has two elements, which ar...

  11. Ægypticisme, ægyptomani. Egyptian revival

    Langkjær, Michael Alexander

    2001-01-01

    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....

  12. Molecular characterization of eimeria species naturally infecting egyptian baldi chickens.

    Sahar M Gadelhaq

    2015-03-01

    Full Text Available Coccidiosis is a serious protozoal disease of poultry. The identification of Eimeria species has important implications for diagnosis and control as well as for epidemiology. The molecular characterization of Eimeria species infecting Egyptian baladi chickens was investigated.Eimeria species oocysts were harvested from intestines of naturally infected Egyptian baldi chickens. The morphometry characterization of oocysts along with COCCIMORPH software was done. The DNA was extracted initially by freezing and thawing then the prepared samples was subjected to commercial DNA kits. The DNA products were analyzed through conventional polymerase chain reaction by using amplified region (SCAR marker.The PCR results confirmed the presence of 7 Eimeria species in the examined fecal samples of Egyptian baldi breed with their specific ampilicon sizes being E. acervulina (811bp, E. brunette (626bp, E. tenella (539bp, E. maxima (272bp, E. necatrix (200bp, E. mitis (327bp and E. praecopx (354bp. A sequencing of the two most predominant species of Eimeria was done, on E. tenella and E. máxima. Analysis of the obtained sequences revealed high identities 99% between Egyptian isolates and the reference one. Similarly, E. maxima isolated from Egyptian baldi chickens showed 98% nucleotide identities with the reference strain. Only single nucleotide substitution was observed among the Egyptian E. tenella isolates (A181G when compared to the reference one. The Egyptian isolates acquired 4 unique mutations (A68T, C164T, G190A and C227G in compared with the reference sequence.This is the first time to identify the 7 species of Eimeria from Egyptian baladi chickens.

  13. Characterization of Egyptian coal from Sinai using Moessbauer spectroscopy

    The presence of iron bearing minerals in coal makes the Moessbauer Spectroscopy (MS) extremely useful for characterization of coals from different localities. In this paper the MS has been applied to characterize Egyptian coal from Sinai (Maghara). The chemical analysis of this coal is given. The MS results showed that pyritic sulphur (pyrite and marcasite) is the only bearing mineral in Egyptian coal. A review is given for the iron bearing minerals in coals from different countries measured by MS. (author)

  14. Application of Mössbauer spectroscopy in investigating Egyptian archaeology

    Eissa, N. A.; Sallam, H. A.

    1988-12-01

    This article summarizes the results of applications of the Mössbauer spectroscopy to investigate Ancient Egyptian pottery from the periods: Ancient Egyptian (3200 525 B.C.) Greek-Roman (320 B.C. 640 A.C.) and Early Islamic (800 1000 A.C.). Many objective informations deduced about: provinance, manufacturing techniques for different domestic purposes, civilization transfer between the Arab countries, methods of colouration and applying decorating glazes, and finally dating of ancient pottery.

  15. Radiation levels in ancient Egyptian mummies

    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 β 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 γ-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)

  16. Examination of an Egyptian mummy - stereolithography applied

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

  17. Spectral signature of Egyptian crude oils

    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 (λex, λem) 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 λmax between 350 and 500 nm according to the excitation wavelength. The refined heavy oil shows very broad unstructured emission spectra with λmax > 400 nm. As a group, they could certainly be distinguished from the light oil samples and most of the crude oil

  18. Attitude of Egyptian consumer towards irradiated food

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

  19. Did the ancient egyptians discover Algol?

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

    2013-02-01

    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).

  20. Estimates of the effect on hepatic iron of oral deferiprone compared with subcutaneous desferrioxamine for treatment of iron overload in thalassemia major: a systematic review

    Caro J

    2002-11-01

    Full Text Available Abstract Background Beta thalassemia major requires regular blood transfusions and iron chelation to alleviate the harmful accumulation of iron. Evidence on the efficacy and safety of the available agents, desferrioxamine and deferiprone, is derived from small, non-comparative, heterogeneous observational studies. This evidence was reviewed to quantitatively compare the ability of these chelators to reduce hepatic iron. Methods The literature was searched using Medline and all reports addressing the effect of either chelator on hepatic iron were considered. Data were abstracted independently by two investigators. Analyses were performed using reported individual patient data. Hepatic iron concentrations at study end and changes over time were compared using ANCOVA, controlling for initial iron load. Differences in the proportions of patients improving were tested using χ2. Results Eight of 11 reports identified provided patient-level data relating to 30 desferrioxamine- and 68 deferiprone-treated patients. Desferrioxamine was more likely than optimal dose deferiprone to decrease hepatic iron over the average follow-up of 45 months (odds ratio, 19.0, 95% CI, 2.4 to 151.4. The degree of improvement was also larger with desferrioxamine. Conclusions This analysis suggests that desferrioxamine is more effective than deferiprone in lowering hepatic iron. This comparative analysis – despite its limitations – should prove beneficial to physicians faced with the challenge of selecting the optimal treatment for their patients.

  1. Biodiversity of Bacterial Ecosystems in Traditional Egyptian Domiati Cheese▿

    El-Baradei, Gaber; Delacroix-Buchet, Agnès; Ogier, Jean-Claude

    2007-01-01

    Bacterial biodiversity occurring in traditional Egyptian soft Domiati cheese was studied by PCR-temporal temperature gel electrophoresis (TTGE) and PCR-denaturing gradient gel electrophoresis (DGGE). Bands were identified using a reference species database (J.-C. Ogier et al., Appl. Environ. Microbiol. 70:5628-5643, 2004); de novo bands having nonidentified migration patterns were identified by DNA sequencing. Results reveal a novel bacterial profile and extensive bacterial biodiversity in Domiati cheeses, as reflected by the numerous bands present in TTGE and DGGE patterns. The dominant lactic acid bacteria (LAB) identified were as follows: Leuconostoc mesenteroides, Lactococcus garvieae, Aerococcus viridans, Lactobacillus versmoldensis, Pediococcus inopinatus, and Lactococcus lactis. Frequent non-LAB species included numerous coagulase-negative staphylococci, Vibrio spp., Kocuria rhizophila, Kocuria kristinae, Kocuria halotolerans, Arthrobacter spp./Brachybacterium tyrofermentans. This is the first time that the majority of these species has been identified in Domiati cheese. Nearly all the dominant and frequent bacterial species are salt tolerant, and several correspond to known marine bacteria. As Domiati cheese contains 5.4 to 9.5% NaCl, we suggest that these bacteria are likely to have an important role in the ripening process. This first systematic study of the microbial composition of Domiati cheeses reveals great biodiversity and evokes a role for marine bacteria in determining cheese type. PMID:17189434

  2. Biodiversity of bacterial ecosystems in traditional Egyptian Domiati cheese.

    El-Baradei, Gaber; Delacroix-Buchet, Agnès; Ogier, Jean-Claude

    2007-02-01

    Bacterial biodiversity occurring in traditional Egyptian soft Domiati cheese was studied by PCR-temporal temperature gel electrophoresis (TTGE) and PCR-denaturing gradient gel electrophoresis (DGGE). Bands were identified using a reference species database (J.-C. Ogier et al., Appl. Environ. Microbiol. 70:5628-5643, 2004); de novo bands having nonidentified migration patterns were identified by DNA sequencing. Results reveal a novel bacterial profile and extensive bacterial biodiversity in Domiati cheeses, as reflected by the numerous bands present in TTGE and DGGE patterns. The dominant lactic acid bacteria (LAB) identified were as follows: Leuconostoc mesenteroides, Lactococcus garvieae, Aerococcus viridans, Lactobacillus versmoldensis, Pediococcus inopinatus, and Lactococcus lactis. Frequent non-LAB species included numerous coagulase-negative staphylococci, Vibrio spp., Kocuria rhizophila, Kocuria kristinae, Kocuria halotolerans, Arthrobacter spp./Brachybacterium tyrofermentans. This is the first time that the majority of these species has been identified in Domiati cheese. Nearly all the dominant and frequent bacterial species are salt tolerant, and several correspond to known marine bacteria. As Domiati cheese contains 5.4 to 9.5% NaCl, we suggest that these bacteria are likely to have an important role in the ripening process. This first systematic study of the microbial composition of Domiati cheeses reveals great biodiversity and evokes a role for marine bacteria in determining cheese type. PMID:17189434

  3. The Administration of Syria and Palestine under Egyptian Rule (1831-1840)

    HOFMAN, Yıtzhak

    2014-01-01

    This article examines the administration of Syria and Palestine under Egyptian rule from 1831 to 1840 and also gives detailed information about the administrative structure of Syria formed by the Egyptians.

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

    Karim MAMDOUH ABBAS

    2014-01-01

    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 Eg...

  5. The Egyptian legislation for safe transportation of radioactive materials

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

  6. Natural radioactivity and radiological hazard assessment of Egyptian oil ashes.

    Mohammed, Hesham; Sadeek, Sadeek; Mahmoud, Abu Rehab; Diab, Hanan; Zaky, Doaa

    2016-08-01

    Oil fly and boiler ash samples were collected from the four major Egyptian power plants in order to determine their natural radioactivity. Secular equilibrium between (238)U and (232)Th and their decay products is significantly disturbed in oil ash samples. The (226)Ra/(238)U ratios were between 440 and 1993 with an average value of 801, indicating that the concentrations of daughters (226)Ra were very high compared to the parent (238)U in the oil ash samples. While, the average ratios for (210)Pb/(226)Ra in most samples were 1.19 ± 0.05, indicating a secular equilibrium in the (226)Ra-(210)Pb sub series. The natural radioactivity due to (238)U and (232)Th was found to be negligible. While the activity concentrations of (226)Ra ranged from 3205 to 12,320 Bq kg(-1) with an average value of 9284 Bq kg(-1), (210)Pb ranged from 5960 to 13,930 Bq kg(-1) with an average value of 11,513 Bq kg(-1). The results are compared with the reported data from other countries. The average value of radium equivalent activity was 9308 ± 2729 Bq kg(-1), while the external and internal hazard indexes were found to be 25 ± 7 and 50 ± 15, respectively. All the studied radiological parameters were higher than the recommended limit by the IAEA in all ash samples. PMID:27126872

  7. Perceptions, practices, and traditional beliefs related to neonatal jaundice among Egyptian mothers: A cross-sectional descriptive study.

    Moawad, Eman Mohamed Ibraheim; Abdallah, Enas Abdallah Ali; Ali, Yahia Zakaria Abdelalim

    2016-09-01

    Neonatal jaundice (NNJ) is one of the most common neonatal disorders worldwide. It is still a main cause of avoidable brain damage, physical and mental impairment, and probable death in newborns.We aimed to assess perceptions, practices, and traditional beliefs among Egyptian mothers toward NNJ that may contribute to delayed presentation and inappropriate management of hyperbilirubinemia.This descriptive, cross-sectional study was conducted from January to May 2015. We interviewed 400 Egyptian mothers who gave birth in knowledge and attitude scores of Egyptian mothers in most domains with a mean of 6.6 and 20.6, respectively, although the majority of them were illiterate or had low educational attainment. In terms of knowledge, 52.3% of participants had adequate knowledge about NNJ in the aspects of awareness, risk factors, management, and complications. Almost all participants exhibited moderate (89.8%) and high levels (10%) of positive attitudes toward NNJ. Maternal sociodemographic factors influenced knowledge level, attitudes, and behaviors related to NNJ in Egypt. Working mothers and those residing in urban areas were significantly more knowledgeable (P = 0.023 and 0.021, respectively), and attained higher attitude scores (P knowledge and attitudes related to NNJ. However, cultural beliefs and traditional infant care practices still have an impact on mothers regardless of their educational level. PMID:27603393

  8. "Evaluation Of compliance To Iron Chelation Therapy With Defe-Roxamine In Patients With Major Thalassemia In Iran In 2004 "

    M. Izadyar

    2006-06-01

    Full Text Available Background and Aim: With the introduction of long term subcutaneous administration of Deferoxamine there has been a decline in the morbidity and mortality of transfusion-dependent beta thalassemia patients. But parenteral iron chelation therapy is still a burden and a major reason for unsatisfactory compliance and places an additional psychological burden on the patients. There are some factors contributing to low compliance in these patients. To evaluate compliance to Deferoxamine and barriers of non adherence and assessment the prevalence of depression and it’s association with compliance. Materials and Methods: 205 patients with major thalassemia in children medical center older than 6 years old were included. They were classified in 3 groups by compliance index (CI: No. of days of treatment per one month/No. of treatment days prescribed by physician. CI>75 % was considered good ,CI< 50-75%: moderate,CI<50%:weak and 3d group the patients without compliance. For assessment of depression: Beck Depression questionnaires were given to the patients older than 18 years old and Children Depression Inventories (CDI“kovacs” were given to the rest Results: Of 205 patients (110 females (54% and 95 males (46%, 13.3% were non compliant, 14% had poor compliance, 62.7% had good compliance. Females were more compliant than males (P=0.034. Compliance improved in older age groups meaningfully (P=0.037. There was negative association between compliance and serum ferritin level (P=0.02. 22% of children and 12% of Adults had severe depression according to the questionnaires. There was no association between compliance and depression. The most prevalent problem rgarding Desferal injection was local reactions in injection site (83%. Conclusion: As oral chelation therapies are not used routinely, more investigations regarding the noncompliance must be considered; and this method of chelation therapy must be encouraged. Compliance is a multifactorial problem

  9. Composition of Egyptian nerolì oil.

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

    2011-07-01

    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

  10. The association of polymorphic sites in some genes with type 1 diabetes mellitus in a sample of Egyptian children

    El-Kafoury, Ahmed A; Medhat Haroun; Amira Mohamed Embaby; Ali Salem Dawoods

    2014-01-01

    Background: The major histocompatibility complex (MHC) genes have been implicated as the major genetic component in the predisposition to type 1 diabetes mellitus (T1DM). Other loci outside the MHC had also been reported to contribute in the susceptibility of T1DM. The aim of this study was to examine the role of some variants of polymorphic sites in some genes associated with T1DM in a sample of Egyptian children. Patients and methods: 60 patients with T1DM from the diabetes clinic at Ale...

  11. Assessment of female sexual function in a group of uncircumcised obese Egyptian women.

    Elnashar, A R M; Ibrahim, N H; Ahmed, H-Eh; Hassanin, A M; Elgawady, M A

    2015-01-01

    The aim of the present study was to assess female sexual function in an obese group (250 women) and to compare it with a control group (100 women), among 25-35-year-old uncircumcised Egyptian women, using female sexual function index (FSFI) score. FSFI total score of ⩽ 26.55 was considered diagnostic of Female Sexual Dysfunction (FSD). The percentage of FSD in the obese group was 73.6% while it was 71% in the control group, which was statistically insignificant (P > 0.05). The difference between both groups regarding the total (FSFI) score was insignificant (P > 0.05), but arousal and satisfaction domains scores were significantly lower in the obese group. In the obese group, a strong negative correlation between body mass index and arousal, orgasm and the total FSFI score was found. Women with excessive obesity had the lowest total FSFI score. In the obese group, college graduates had the highest total scores and all domain scores of FSFI followed by high school graduates while the least educated women had the lowest scores and when these subgroups were compared, significant differences were found among them. We conclude that in uncircumcised 25-35-year-old Egyptian women, obesity is not a major detrimental factor for FSD, but it may affect some sexual domains such as arousal and satisfaction, although excessive obesity is associated with FSD. Also, educational and cultural factors may have an impact on perception of sex and pleasure. PMID:26155831

  12. Promoting Phonological Awareness Skills of Egyptian Kindergarteners through Dialogic Reading

    Elmonayer, Randa Abdelaleem

    2013-01-01

    The present study examines the effect of dialogic reading (DR) on the promotion of Arabic phonological awareness skills (including syllable awareness, rhyme awareness, and phoneme awareness) of Egyptian kindergarteners. The participants were 67 children enrolled in the second level of kindergarten (ages 5-6), assigned to an experimental group…

  13. Strategic Leadership and Its Application in Egyptian Universities

    Hany R. Alalfy

    2014-11-01

    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.   

  14. Egyptian National Postal Organization : Review of Asset Management Operations

    World Bank

    2009-01-01

    This report presents the missions observations and recommendations. The mission has not been able to review the investment manual and current investment procedures as the relevant documents have not been yet forwarded by Egyptian National Postal Organization (ENPO) as requested. ENPO was established in 1865 and since its creation it has always had a clear mandate of public service that rem...

  15. Application of Moessbauer spectroscopy to study archaeological Egyptian pottery

    Moessbauer spectra have been used as ''fingerprints'' in obtaining information an ancient Egyptian pottery and in fine art. An empirical relation has been found that connects the natural radiation dose with the intensity ratio of the two non-magnetic central peaks. It was suggested that this relation be used for dating ancient pottery. 8 refs, 13 figs, 2 tabs

  16. Shifting Pedagogical Space: Egyptian Educators Use of Moodle

    Richardson, Jayson; Finholt-Daniel, Matt; Sales, Greg; Flora, Kevin

    2012-01-01

    This article focuses on exploring the outcomes of an e-learning initiative in Egypt. Researchers conducted training with 17 Egyptian educators on how to build, maintain, and teach using Moodle, an online content management system. The researchers evaluated the outputs of the training using the Technology Adoption Model (TAM) in an effort to assess…

  17. Muslim Egyptian and Lebanese Students' Conceptions of Biological Evolution

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

    2011-01-01

    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…

  18. Parenting Style, Individuation, and Mental Health of Egyptian Adolescents

    Dwairy, Marwan; Menshar, Kariman E.

    2006-01-01

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

  19. Student Involvement in the Egyptian Quality Assurance System

    Elassy, Noha

    2015-01-01

    Purpose: The purpose of this paper is to study the extent and the quality of student involvement in the quality assurance process (QAP) in Egyptian higher education institutions (HEIs). Design/methodology/approach: In this study, two qualitative methods were used to explore the extent and the quality of student involvement; these were focus groups…

  20. The Case for (Social) Entrepreneurship Education in Egyptian Universities

    Kirby, David A.; Ibrahim, Nagwa

    2011-01-01

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

  1. Evaluation of municipal solid waste management in egyptian rural areas.

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

    2009-01-01

    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

  2. Placentation in the Egyptian slit-faced bat Nycteris thebaica (Chiroptera: Nycteridae)

    Enders, A C; Jones, C J P; Taylor, P J;

    2009-01-01

    Bats are a highly successful, widely distributed group, with considerable variation in placental structure. The Egyptian slit-faced bat Nycteris thebaica is a member of one of the few families with previously undescribed placentation. It was found that, although the interhemal type of the Nycteris...... placenta is endotheliochorial with a single layer of cytotrophoblast, the arborizing pattern of the maternal vessels and especially the extraordinary major placental artery differs from the placenta of the emballonurid bats to which this family is considered to be most closely related. The major placental...... other bat species. The paraplacenta is extensive with abundant fetal vessels underlying cytotrophoblast and syncytial trophoblast layers, fronting on an endometrium that largely lacks uterine epithelial cells but has large decidual cells and is poorly vascularized. The placenta of Nycteris lacks a...

  3. "Confused by Multiple Deities, Ancient Egyptians Embraced Monotheism": Analysing Historical Thinking and Inclusion in Egyptian History Textbooks

    Abdou, Ehaab D.

    2016-01-01

    Egyptian history textbooks are examined through the prism of historical thinking dimensions and skills, utilizing a critical discourse analysis. The analysis focuses on how the textbooks portray two historically significant events: the advent of Christianity (ca. 33 CE) and Islam (ca. 641 CE) to Egypt. It reveals that the historical narrative…

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

    Carpenter JP

    2011-09-01

    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.

  5. Screening for hepatocellular carcinoma by Egyptian physicians

    Sahar; M; Hassany; Ehab; F; Abdou; Moustafa; Mohamed; El; Taher; Afaf; Adel; Abdeltwab; Hubert; E; Blum

    2015-01-01

    AIM: To assess the practice of Egyptian physicians in screening patients for hepatocellular carcinoma(HCC). METHODS: The study included 154 physicians from all over Egypt caring for patients at risk for HCC. The study was based on a questionnaire with 20 items. Each questionnaire consisted of two parts:(1) personal information regarding the physician(name, age, specialty and type of health care setting); and(2) professional experience in the care of patients at risk for HCC development(screening, knowledge about the cause and natural course of liver diseases and HCC risk). RESULTS: Sixty-eight percent of doctors with an MD degree, 48% of doctors with a master degree or a diploma and 40% of doctors with a Bachelor of Medicine, Bachelor of Surgery certificate considered the hepatitis C virus(HCV) genotype as risk factor for HCC development(P < 0.05). Ninety percent of physicians specialized in tropical medicine, internal medicine or gastroenterology and 67% of physicians in other specialties advise patients to undergo screening for HCV and hepatitis B virus infection as well as liver cirrhosis(P < 0.05). Eighty-six percent of doctors in University Hospitals and 69% of Ministry of Health(MOH) doctors consider HCV infection as the leading cause of HCC in Egypt(P < 0.05). Seventy-two percent of doctors with an MD degree, 55% of doctors with a master degree or a diploma, 56% of doctors with an MBBCH certificate, 74% of doctors in University Hospitals and 46% of MOH hospital doctors consider abdominal ultrasonography as the most important investigation in HCC screening(P < 0.05). Sixty-five percent of physicians in tropical medicine, internal medicine or gastroenterology and 37% of physicians in other specialties recommend as HCC screening interval of 3 mo(P < 0.05). Seventy-one percent of doctors with an MD degree, 50% of doctors with a master degree or diploma and 60% of doctors with an MBBCH certificate follow the same recommendation.CONCLUSION: In Egypt, physicians

  6. Striking presence of Egyptian blue identified in a painting by Giovanni Battista Benvenuto from 1524.

    Bredal-Jørgensen, Jørn; Sanyova, Jana; Rask, Vibeke; Sargent, Maria Louise; Therkildsen, Rikke Hoberg

    2011-09-01

    Egyptian blue has been identified in a painting from 1524 by the Italian artist Ortolano Ferrarese (Giovanni Battista Benvenuto). Egyptian blue is the oldest known synthetic pigment, invented by the Egyptians in the fourth dynasty (2613-2494 BC) of the Old Kingdom and extensively used throughout Antiquity. From about 1000 A.D., it disappeared from the historical record and was only reinvented in the late nineteenth and early twentieth century. The discovery of Egyptian blue in Ortolano Ferrarese's painting from 1524 shows that Egyptian blue was in fact available in the period from which it is normally considered not to exist. The identification of Egyptian blue is based on optical microscopy supported by energy-dispersive spectroscopy and visual light photon-induced spectroscopy, and finally confirmed by Raman microspectroscopy. PMID:21678119

  7. Evaluation of the Mechanical Durability of the Egyptian Machine Readable Booklet Passport

    Ahmed Mahmoud Yosri

    2013-12-01

    Full Text Available In 2008 the first Egyptian booklet Machine Readable Passport/ MRP has been issued and its security and informative standard quality levels were proved in a research published in 2011. Here the durability profiles of the Egyptian MRP have been evaluated. Seven mechanical durability tests were applied on the Egyptian MRP. Such tests are specified in the International Civil Aviation Organization / ICAO standard requirements documents. These seven very severe durability tests resulted in that the Egyptian MRP has achieved better & higher results than the values detected in ICAO-Doc N0232: Durability of Machine Readable Passports - Version: 3.2. Hence, this research had proved the complete conformance between the Egyptian MRP mechanical durability profiles to the international requirements. The Egyptian booklet MRP doesn’t need any obligatory modification concerning its mechanical durability profiles.

  8. Premature epiphyseal fusion and extramedullary hematopoiesis in thalassemia

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

    1987-10-01

    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.

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

    Jock M. Agai

    2013-01-01

    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...

  10. THE VALUE RELEVANCE OF THE FINANCIAL STATEMENTS’ BOTTOM LINES IN THE EMERGING EGYPTIAN CAPITAL MARKET

    Abousamak, A.

    2015-01-01

    This study aims to examine the value relevance of the bottom lines of the financial statements in the Egyptian context after the inception of the new version of Egyptian Accounting Standards of 2006. Considering the tradition accounting value of conservatism and the firm size, the price and return models are operationalized using a sample of the most actively traded companies on the Egyptian Stock Market in the period from 2007-2009. Three important conclusions captured from th...

  11. Population continuity or population change: formation of the ancient Egyptian state

    Zakrzewski, Sonia R.

    2007-01-01

    The origins of the ancient Egyptian state and its formation have received much attention, through analysis of mortuary contexts, skeletal material, and trade. Genetic diversity was analyzed by studying craniometric variation within a series of six time-successive Egyptian populations in order to investigate the evidence for migration over the period of the development of social hierarchy and the Egyptian state. Craniometric variation, based upon 16 measurements, was assessed through principal...

  12. The Association between Myocardial Iron Load and Ventricular Repolarization Parameters in Asymptomatic Beta-Thalassemia Patients

    Mehmet Kayrak

    2012-01-01

    Full Text Available Previous studies have demonstrated impaired ventricular repolarization in patients with β-TM. However, the effect of iron overload with cardiac T2* magnetic resonance imaging (MRI on cardiac repolarization remains unclear yet. We aimed to examine relationship between repolarization parameters and iron loading using cardiac T2* MRI in asymptomatic β-TM patients. Twenty-two β-TM patients and 22 age- and gender-matched healthy controls were enrolled to the study. From the 12-lead surface electrocardiography, regional and transmyocardial repolarization parameters were evaluated manually by two experienced cardiologists. All patients were also undergone MRI for cardiac T2* evaluation. Cardiac T2* score <20 msec was considered as iron overload status. Of the QT parameters, QT duration, corrected QT interval, and QT peak duration were significantly longer in the β-TM group compared to the healthy controls. Tp−Te and Tp−Te dispersions were also significantly prolonged in β-TM group compared to healthy controls. (Tp-Te/QT was similar between groups. There was no correlation between repolarization parameters and cardiac T2* MRI values. In conclusion, although repolarization parameters were prolonged in asymptomatic β-TM patients compared with control, we could not find any relation between ECG findings and cardiac iron load.

  13. Prevalence and specificities of red cell alloantibodies in transfusion-dependent beta thalassemia patients in Yazd

    Vaziri, M.; JavadzadehShahshahani, H; Moghaddam, M.; Taghvaee, N

    2015-01-01

    Background Multiple transfusions in thalassemia patients may lead to antibody production against blood group antigens and hemolytic transfusion reaction might occur. In this study, antibody screening test was performed by tube and gel methods to determine the prevalence and specificity of alloantibodies in thalassemia patients. Materials and Methods In this cross-sectional study, overall of 100 thalassemia patients from Yazd thalassemia clinic were recruited from July to September 2013. Two b...

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

    Capretto L

    2012-01-01

    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 γ-globin messenger ribonucleic acid production and to increase fetal hemoglobin (HbF accumulation, the percentage of HbF-containing cells, and their HbF content without stimulating α-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

  15. Prevalence of anti-HAV antibodies in multitransfused patients with beta-thalassemia

    Dimitrios Siagris; Chryssoula Labropoulou-Karatza; Alexandra Kouraklis-Symeonidis; Irini Konstantinidou; Myrto Christofidou; Ioannis Starakis; Alexandra Lekkou; Christos Papadimitriou; Alexandros Blikas; Nicholas Zoumbos

    2008-01-01

    AIM:To detect the prevalence of anti-HAV IgG antibodies in adult multitransfused beta-thalassemic patients.METHODS:We studied 182 adult beta-thalassemic patients and 209 controls matched for age and sex from the same geographic area,at the same time.Anti-HAV IgG antibodies,viral markers of hepatitis B virus (HBV)and hepatitis C virus (HCV) infection were evaluated.RESULTS:Anti-HAV IgG antibodies were detected more frequently in thalassemic patients(133/182;73.1%)than in healthy controls(38/209;18.2%,P<0.0005).When we retrospectively evaluated the prevalence of anti-HAV IgG antibodies in 176/182(96.7%)thalassemic patients,whose medical history was available for the previous ten years,it was found that 83(47.2%)of them were continuously anti-HAV IgG positive,16(9.1%)acquired anti-HAV IgG antibody during the previous ten years,49 (27.8%) presented anti-HAV positivity intermittently and 28 (15.9%) were anti-HAV negative continuously.CONCLUSION:Multitransfused adult beta-thalassemic patients present higher frequency of anti-HAY IgG antibodies than normal population of the same geographic area.This difference is difficult to explain,but it can be attributed to the higher vulnerability .of thalassemics to HAV infection and to passive transfer of anti-HAV antibodies by blood transfusions.

  16. Is there an association of giardiasis with beta-thalassemia minor?

    Yakoob, Javed; Jafri, Wasim; Shaikh, Hizbullah

    2013-01-01

    Abstract. Beta–thalassemia minor  is a symptomless carrier state of a hemoglobinopathy which predisposes to bacterial infections. We report three cases presenting with giardiasis, a parasitic infection of gastrointestinal tract caused by Giardia lamblia. Patients presented with recurrent diarrhea and abdominal pain for over a year. An esophagogastroduodenoscopy finding varied from normal to acute duodenitis. Duodenal biopsy demonstrated changes consistent with mild chronic non-specific inflam...

  17. Macrophages support pathological erythropoiesis in Polycythemia Vera and Beta-Thalassemia

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

    2013-01-01

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

  18. Prevalence of renal tubular dysfunction in beta thalassemia minor in shiraz

    Ali Moradi Nakhodcheri

    2012-02-01

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

  19. Prevalence of anti HCV infection in patients with beta-thalassemia in Isfahan-Iran

    Behrooz Ataei

    2012-01-01

    Conclusions: Our findings revealed that blood transfusion was the main risk factors for HCV infection among beta-thalassemic patients. Therefore, more blood donor screening programs and effective screening techniques are needed to prevent transmission of HCV infection among beta-thalassemic patients.

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

    β-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 β-globin gene in β-thalassaemic children in sharkia governorate. the present study was included 25 healthy children and 50 patients diagnosed as β-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

  1. Rezension zu: Emily Teeter (Hg.), Before the pyramids. The origins of Egyptian civilization

    Köpp-Junk, Heidi

    2013-01-01

    Rezension zu: Emily Teeter (Hg.), Before the Pyramids. The Origins of Egyptian Civilization. The Oriental Institute of the University of Chicago, Oriental Institute Museum Publications 33 (Chicago 2011)

  2. Multielement determination in some egyptian vegetables by instrumental neutron activation analysis

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

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

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

    2009-12-01

    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

  4. The Egyptian Revolution: First Impressions from the Field

    Mohammed A. Bamyeh

    2011-02-01

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

  5. Egyptian Activism against Female Genital Cutting as Catachrestic Claiming

    An Van Raemdonck

    2013-08-01

    Full Text Available This paper deals with questions of the politics of location in knowledge and norm production within the context of Egyptian feminist activism for abandoning female genital cutting practices. It seeks to determine underlying schemes of international campaigning discourse and analyzes how these predicate and complicate Egyptian postcolonial activism. It draws on a broad literature study in addition to fieldwork in Cairo consisting of in-depth interviews with activists and policy makers. My focus is on the national Task Force against FGM from 1994 until 1999 and its subsequent cooptation by the National Council of Childhood and Motherhood. I argue through the concept of catachresis that location matters in setting the terms of anti-FGC discourse and its relation to religion.

  6. Chemical analysis of Yemeni archaeological cheramics and the Egyptian enigma

    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)

  7. Dietary Behaviour Pattern and Physical Activity in Overweight and Obese Egyptian Mothers: Relationships with Their Children's Body Mass Index

    Nayera E. Hassan

    2016-09-01

    CONCLUSION: Improper dietary patterns, nonworking mothers and big family size are associated with obesity among Egyptian women. Emphasis should be given to increasing physical activity and encourage healthier diets among Egyptian mothers and their children.

  8. Unwrapping an Ancient Egyptian Mummy Using X-Rays

    Hughes, Stephen W.

    2010-01-01

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

  9. Isolation of genetically diverse Marburg viruses from Egyptian fruit bats.

    Towner, Jonathan S.; Amman, Brian R.; Sealy, Tara K.; Serena A Reeder Carroll; Comer, James A.; Alan Kemp; Robert Swanepoel; Paddock, Christopher D.; Stephen Balinandi; Marina L Khristova; Formenty, Pierre B.H.; Albarino, Cesar G.; Miller, David M.; Reed, Zachary D.; John T. Kayiwa

    2009-01-01

    Author Summary Marburg virus, similar to its close cousin Ebola virus, can cause large outbreaks of hemorrhagic fever (HF) in rural Africa with case fatalities approaching 90%. For decades, a long-standing enigma has been the identity of the natural reservoir of this deadly virus. In this report, we identify the cave-dwelling Egyptian fruit bat (Rousettus aegyptiacus) as a natural host of Marburg virus based on multiple lines of evidence which include, for the first time ever, the isolation o...

  10. Nile Crossings: Hospitality and Revenge in Egyptian Rural Narratives

    Granara, William E.

    2010-01-01

    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 fo...