فهرست مطالب

Iranian Journal of Blood and Cancer
Volume:1 Issue: 3, Spring 2009

  • تاریخ انتشار: 1388/12/01
  • تعداد عناوین: 8
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  • Correlation of Serum Ferritin Level with Cardiac Function in β-Thalassemia Major
    Hassan Mahmoodi Nesheli, Mohammad Taghi Arzanian, Tahereh Galini Moghaddam, Bibi Shahin Shamsian, Samin Alavi, Latif Gachkar, Said Mojtahedzadeh, Karimollah Hajian Tilaki Page 4
    Background
    The most important problem in regular transfusion dependent β-thalassemia major is cardiac dysfunction due to iron deposition in it. The aim of this study was to evaluate correlation between serum ferritin levels and cardiac function in β-thalassemia major in Mofid Hospital.
    Materials And Methods
    There were 112 β-thalassemia patients with a mean age of 13. 55± 6. 12 years، of whom 49 were males and 63 were females. Patients enrolled in this study were divided into two groups; group 1 (n=33) who were good users of chelation therapy with ferritin levels of <1500 ng/dl and group 2 (n=79) who were not good users of chelation therapy with ferritin levels more than 1500 ng/dl. Clinical history، physical examination، and laboratory investigations along with serum ferritin levels and echocardiography were assessed for all patients.
    Results
    Overall ferritin levels were 200-8600 ng/dl. Mean ejection fraction was 64. 72% and 63. 30% in groups 1 and 2، respectively. There was not a significant difference between two groups. Mean fractional shortening were 29. 30% and 28. 82% in groups 1 and 2، respectively. Again there was not a significant difference.
    Conclusion
    This study has shown that there was no significant correlation between echocardiographic indices and serum ferritin levels in major β-thalassaemia. Thus، other methods should be used to evaluate cardiac iron burden.
  • Majid Yavarian, Mozhgan Shahian, Mehran Karimi, Narges Rezaie Page 89
    Background
    The frequency of pyruvate kinase (PK) deficiency, an autosomal recessive defect, is approximately 3 per 10,000 individuals in Shiraz and surrounding areas, and is increased due to high consanguinity marriage frequency. The purpose of this study is to obtain data on the frequency and spectrum of gene mutation of PK in newborns, from Shiraz and surrounding areas.
    Materials And Methods
    Two hundred eleven neonates with neonatal jaundice were studied for erythrocyte pyruvate kinase activity using the method recommended by International Committee for Standardization in Hematology (ICSH).
    Results
    In 22 the PK enzyme activity was below 60%, where the erythrocyte PK activity from 35 healthy cord bloods ranged from 3.9 – 9.8 IU/g Hb. Genomic DNA analysis for PK-R gene mutation was examined in 12 out of 22 cases (heterozygote 3.8%, 95% CI=0.012-0.064; homozygous 1.8%, 95% CI=0.001-0.036). All mutations in four homozygote and 8 heterozygote neonates in this cohort have been reported from the region previously, except for mutation G1675C that has not been found before. Therefore, the rate of recurrence of PK-R gene defect is 4 times more frequent in those with neonatal jaundice in comparison with the regional frequency (0.038).
    Conclusion
    Erythrocyte enzymopathies, especially PK deficiency, should be considered in the differential diagnosis of non-immune hemolytic anemia as well as for genetic counseling in our area.
  • Khodamorad Zandian, Bijan Keikhaie, Mohammad Pedram, Fatemeh Kianpoor Ghahfarokhi Page 95
    Background
    The aim of this perspective study was to assess the frequency of hemoglobinopathy mutational genes among voluntary hemoglobinopathy carrier couples-to-be referred to thalassemia center, Shafa hospital, affiliated to Ahvaz Jondidishapur University of Medical Sciences (AJUOMS), during their first trimester of pregnancy for genetic screening and counseling for prenatal diagnosis (PND).
    Materials And Methods
    In a four-year period (2000-2004), 93 voluntary couples from Khuzestan province (mostly Ahvaz area) who were proved to be carrier for alpha thalassemia, beta thalassemia, or other hemoglobinapathies underwent PND and detection of causative mutational genes by chorionic villus sampling {CVS) plus direct or indirect DNA analysis at first trimester of pregnancy. First trimester authorized termination of pregnancy was performed, if a fetus was confirmed to have serious disorders.
    Results
    From 93 voluntary couples who underwent PND, 11 (10.3%) couples were confirmed to have fetuses with serious disorders, and interruption of pregnancy was performed for them. Prenatal screening and PND in this study showed that β-thalassemia was the most frequent accounting for 55% of all disorders. Results of other hemoglobinopathy mutational genes analysis showed the frequency of S, D, C, and α–globin mutational gene to be 16.2%, 3.2 %, 1%, and 9.7 %, respectively. Eighteen percent of cases were undetermined. Our data showed that the frequency of sickle cell mutational gene is second to β-thalassemia, α-thalassemia is the third one.
    Conclusion
    It is too clear that prenatal screening and PND is a useful keys methods for identifying the affected fetuses, expensive methods and the Ahvaz is on long distance far from the capital town (Tehran), only the rich voluntary family with high income accepted. this pathway solution. Recently Since 2007 these facility were set up in to Ahvaz Thalassemia center by co- ordination of Ministry of public health services through Iran national thalassemia screening program. Part-affiliated to(AJUOMS). Furthermore, the frequency of 18% others of unknown genetic traits still dictates further detailed studies that can elucidate the other types of genetic abnormities that exist in region.
  • Murtaza Rashid, Ozair Bin Majid, Mehdi Dehghani, Mehran Karimi Page 99
    Background
    Anemia is common throughout the world، remarkably in developing countries. Our aim is to calculate normal hemoglobin and other hematological indices in southwest of Iran.
    Materials And Methods
    A random selection of 500 healthy males and 500 healthy females was done in the age group of 13 to 65 years. A complete blood count (CBC) was performed in all participants.
    Results
    Hemoglobin (Hb) level ranged from 13. 80 to 18. 40 g/dL with a mean value of 15. 96 g/dL in males، and 12 to 15. 90 g/dL with a mean value of 13. 79 g/dL in females. Mean cell volume (MCV) ranged from 80 to 100. 10 fL with a mean value of 86. 63 fL in males، and 80 to 95. 40 fL with a mean value of 86. 80 fL in females. Red blood cell (RBC) count ranged from 4. 5 to 7. 07×106/mm3 with a mean value of 5. 57×106/mm3 in males، and 4. 17 to 6. 84×106/mm3 with a mean value of 4. 88×106/mm3 in females.
    Conclusion
    Although the normal range of various hematological indices of our study nearly were equal to that presented in the literature، some parameters differed such as maximum value of Hb in males، maximum value of hematocrit (HCT) in both sexes، range of MCV in females، and range of mean cell hemoglobin concentration (MCHC) in both sexes. The most prominent feature was the higher maximum value of RBC count in both sexes.
  • Hossein Safizadeh, Nasim Pourdamghan, Batool Mohamadi Page 107
    Background
    Blood transfusion is one of the most crucial needs of the society in multiple diseases and injuries. The only source of blood is donation. Students consist a large and healthy group who are able of providing a large number of blood donation، however، blood donation is rare amongst them. This study was conducted to evaluate students awareness and attitude towards blood donation in Kerman city.
    Materials And Methods
    In this cross sectional study، 500 students in Kerman city were evaluated using a questionnaire. T-test، Chi-square and Pearson tests were used for data analysis.
    Results
    The mean (±SD) score of the students awareness and attitude towards blood donation were 1. 96 ±3. 4 out of 9 and 5. 32±45. 42 out of 60، respectively. Data analysis revealed that there was no significant difference in students attitude between males and females، while males were more aware than females (P<0. 001). There was a positive and significant relationship between the students attitude and awareness (P<0. 001 and r=0. 22). With an increase in students awareness about blood donation، their attitude would improve as well. Only 24. 6% of all students reported a history of blood donation whose awareness and attitude were better than other students (P<0. 001).
    Conclusion
    Generally، evaluated students were not aware enough about blood donation. It is necessary to find obstacles and eliminate them to improve the situation.
  • Anmol Mathur, Manish Jain, Mohit Shiva, Mohit Navlakha, Prabu, Suhas Kulkarni Page 111
    Background
    It is suggested that various forms of tobacco use have some effects on diverse sites of head and neck to develop cancer.
    Material And Methods
    The study examines data for oral cancer in male subjects, collected from the population registered in cancer registry during the year 2007- 2008. All the data collected were entered into the spreadsheets. The statistical package for social sciences (SPSS) software version 11.0 was used for data processing and data analysis.
    Results
    Smoking and chewing develops a high risk of oral cancer. Most of the tobacco consumers are in their 40s. People younger than 30 years used betel-quid in abundance (47.1%). Bidi was the choice of tobacco for 41-50 age group. In people with less than ten years duration of using tobacco products, gutkha was consumed in abundance (20.7%). For frequency of 6-10 times per day, smoking Bidi was consumed in 61.4% of instances.
    Conclusion
    The crux of the problem is that majority of the cases are reported with a significant delay to the health care facility. Nationwide counseling on tobacco cessation is required in a developing country like India.