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Article
EVALUATION OF HEARING IN PATIENTS WITH BETA THALASSEMIA MAJOR

Authors: Ahmed Fadhil Hasan --- Husam Haider Salman --- Jaafar Muhsen Khalaf
Journal: Basrah Journal of Surgery مجلة البصرة الجراحية ISSN: 16833589 / ONLINE 2409501X Year: 2018 Volume: 24 Issue: 1 Pages: 47-51
Publisher: Basrah University جامعة البصرة

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Abstract

EVALUATION OF HEARING IN PATIENTS WITH BETA THALASSEMIA MAJOR Ahmed Fadhil Hasan*, Husam Haider Salman# & Jaafar Muhsen Khalaf@ *MB, ChB, FICMS, CABS ENT HNS, ENT Senior Surgeon at Al Shafaa General Hospital. #FRCS, DLO. Retired, Department Of Surgery, College Of Medicine, Basrah University. @MB,ChB, FICMS ENT, ENT Surgeon, Basrah Teaching Hospital, Basrah, IRAQ. Abstract This prospective study was conducted in Basrah Teaching Hospital to evaluate the hearing of β thalassemia major patients, and to find the association between desferoxamine (DFO) therapy and hearing loss (HL). Pure tone eudiometry (PTA) and tympanometry was done to determine hearing thresholds of (23) β thalassemia major patients and (50) non thalassemic subjects. All subjects were more than 4 year old to allow PTA to be done. The criteria of evaluation included a full otological history, drug history, otological examination, audiological evaluation using PTA and tympanometry. This study found that (6) out of (23) thalassemic patients and (6) out of (50) non thalassemic patients have hearing loss. From those thalassemic patients with HL, (5) patients have conductive hearing loss (CHL) and one have sensorineural hearing loss (SNHL), while the non thalassemic patients with HL all have CHL, also we found that (3) out of (5) thalassemic patients not on desferoxamine therapy have HL. In conclusion, β thalassaemia major has no significant statistical association with hearing problems, and there is no significant statistical association between desferoxamine therapy and hearing loss.


Article
Determination of Some Oxidative Stress Parameters and Antioxidants in Sample of Iraqi Beta Thalassemia Major Patients
تحديد بعض معايير جهد الأكسدة ومضادات الأكسدة في عينة من مرضى بيتا الثلاسيميا الكبرى العراقيين

Authors: Amna K. Ahmed أمنه كنعان احمد --- Jabbar H. Yenzeel جبار حميد ينزيل
Journal: Iraqi Journal of Science المجلة العراقية للعلوم ISSN: 00672904/23121637 Year: 2017 Volume: 58 Issue: 1A Pages: 1-3
Publisher: Baghdad University جامعة بغداد

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Abstract

Repeated blood transfusion in beta thalassemia major patients may lead to peroxidative tissue injury by secondary iron overload. In the present study, 100 patients(50 male+50 female) with beta thalassemia major patients with age (5-20) years and 60 healthy control were included during their attendance at Abin Al_Baladi hospital in Baghdad. Malondialdehyde ,Superoxide Dismutase and Vitamin E, were measured by using kits.The results showed A highly significant (p<0.01)increase in the levels of Malondialdehyde and Superoxide Dismutase, whereas, significant p(<0.01)decrease in the levels of vitamin-E, This suggest that oxidative stress and reduced antioxidant defense mechanism play an important role in pathogenesis of beta thalassemia major.

أن تكرار نقل الدم في المرضى بيتا ثلاسيميا الكبرى يؤدي إلى إصابة الأنسجة نتيجه الحديد الزائد الثانوي. في هذه الدراسة،كان 100 عينه من المرضى (50 ذكور + 50 إناث) مع مرضى بيتا ثلاسيميا الكبرى في فئه العمريه (5-20) سنوات و 60 عينه من الاصحاء أثناء حضورهم في مستشفى أبن البلدي في بغداد.مالينديهايد,ودسميتوز وفيتامين(اي),وقياسها باستخدام الكتات وأضهرت النتائج زيادة كبيرة جدا في مستويات ماليندهايد ودستميوز(ع<0.01)في حين انخفاض في فيتامين (اي) ذا يشير إلى أن الاكسدة وانخفاض آلية الدفاع المضادة للأكسدة تلعب دورا هاما في التسبب في بيتا ثلاسيميا الكبرى.


Article
Effect of transfusion interval rate on clinical manifestations of patients with beta- thalassemia major and their correlation with some biochemical parameters

Author: Wafaa Fawzi. AL-Mosawy
Journal: karbala journal of pharmaceutical sciences مجلة كربلاء للعلوم الصيدلانية ISSN: 70272221 Year: 2017 Issue: 13 Pages: 249-257
Publisher: Kerbala University جامعة كربلاء

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Abstract

Background: Regular blood transfusions is the commonest form of life-long treatment for patients with beta-thalassemia major but multiple transfusions of blood causes iron overload and resulting in multiple progressive organ damage that later involved endocrine system, heart, and liver. Objective: This study is aimed to evaluate some clinical manifestations in the adult patients with beta thalassemia major based on interval rate of the blood transfusion and also investigate correlation between clinical status of patients with some biochemical parameters including the level of ferritin, Hb, prolactin, and cortisol.Methodology: Forty one Iraqi β -thalassemia major (TM) patients (19male and 22 female) are recruited from thalassemia center in Karbala at age of 14-24 years. The frequency of blood transfusion rate, blood groups, spleen status, and HCV infection are recorded from the profile of all patients. The weight and height of patients were measured to calculate body mass index (BMI), while the concentration of Hb, ferritin, cortisol, and prolactin are tested at morning just before blood transfusion session. Patients are grouped according to their transfusion interval rate (TIR) into two groups; short TIR group (˂ 3 weeks), and long TIR group (≥ 3 weeks). Results: The results were non-significant in the distribution of gender and blood groups, spleen status, frequency of HCV infection, Hb, cortisol, and prolactin between the two groups, but age average, ferritin level and BMI of long TIR group are significantly lower than those of short TIR patients. On the other hand, long TIR patients revealed non-significant correlation between all parameters and clinical manifestations except for Hb content which is significantly decreased (r = - 0.451, P = 0.039) in splenectomized patients. However, short TIR group showed that Hb content is significantly decreased in both splenectomized (r = - 0.560, P = 0.01) and underweighted (r = - 0.477, P = 0.033) TM patients. Conclusion: We can concluded that the levels of cortisol and prolactin hormones are neither disturbed in TM patients nor correlated with interval rate of transfusion, but Hb level is the key factor that determine TIR and ferritin level leading to retardation in BMI which may be due to another endocrinopathy particularly those that are closely related with metabolic pathways such as growth hormone and thyroxin.


Article
Hemotological and Coagulatory Alteration in Splenectomized Versus Non-Splenectomized Transfusion Dependant Β-Thalassemic Major Patients

Authors: Haider AL-Shamari --- Liqaa M. Majeed Al sharifi --- Farah A. Hussain
Journal: Medical Journal of Babylon مجلة بابل الطبية ISSN: 1812156X 23126760 Year: 2015 Volume: 12 Issue: 2 Pages: 551-560
Publisher: Babylon University جامعة بابل

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Abstract

β-Thalassemia is an inherited hemolytic disorder caused by a partial or complete deficiency of β- globin chain synthesis. Profound hemostatic changes have been observed in patients with β-thalassemia major with history of thromboembolic or haemorrahgic manifestation. The aim of this study was to assess the coagulation parameters; PT, PTT, factor V, factor VIII level in β-thalassemia major and to compare the effect of splenectomy on each and to evaluate the patients for increased risk of thrombosis and/ or bleeding. Sixty patients with transfusion dependent β-thalassemia major ;thirty of them splenectomized (16 females and 14 males), and thirty non-splenectomized (13 females and 17 males) aged between (3-38) years at different stages of the disease were included in this study from Babil hospital for maternity and pediatrics from the first of April 2012 to the 15th of July 2012. Seven milliliters of venous blood were collected from each patients and control persons included in this study by clear venipuncture under aseptic conditions from antecubital fossa with easy withdrawal of blood , subdivided as follows:1*1.8ml in o.2 ml trisodium citrate with gentle mixing for coagulation testing include PT,PTT, factors V and VIII.2*2ml in EDTA tube for routine haematological screening tests include: PCV,WBC, platelet count. Normal pooled plasma for factor assay obtained from 20 normal healthy persons ,plasma was mixed and divided into aliquot each containing about 1ml stored at -40C0 for 3-4 weeks as a control for factor assay study. The mean age was 18.2 years in splenectomized patients and 8.9years in non splenectomized patients. The PCV level was low in both groups 26.8,24.6 in splenectomized and non splenectomized patients respectively with statistical significant difference between them (p value <0.05). WBC and platelets count were both within normal limit ,however there was a statistical difference in platelets count between splenectomized and non splenectomized groups with mean value were 259.6 and 218.7 x109 /L respectively, p value <0.05. WBC count mean were 8.2 and 7.0x109 /L in splenectomized and non splenectomized patients respectively with no statistical significant difference between them p value >0.05. These patients (splenectomized and non splenectomized) have normal mean PT,PTT, which were 14.4 sec, 33 sec for splenectomized and 14.1 sec,33.2 sec for non splenectomized patients respectively with no statistical significant difference between them p value >0.05. Factors V,VIII level were 103%,127.8% for splenectomized patients and 100.3, 128.9% for non splenectomized patients respectively with no statistical significant difference between them p value>0.05 ,but there was statistical significant difference between the control group and the total thalassemic patients (P value <0.05),however splenectomized patients had more episodes of bleeding and thrombotic manifestations than non splenectomized patients which might be due to causes unrelated to coagulation factors V and VIII level. Splenectomy could affect PCV but had no effect on coagulation factors V and VIII.-Bleeding manifestations were more frequent in splenectomized patients with normal factor V,VIII and platelets count.-Thalassemics in Babylon thalassemic center were under transfused.


Article
Immunological Evaluation of Patients with Β-Thalassemia Major in Kerbala City Using Single Radial Immunodiffusion (SRID) Techniqu…………………..………….…

Authors: Zuhair Mohammed Ali Jeddoa --- Sattar jabbar Rahi --- Adel Ateia Abed Ali
Journal: Karbala Journal of Medicine مجلة كربلاء الطبية ISSN: 19905483 Year: 2011 Volume: 4 no 1 Issue: 9 Pages: 939-943
Publisher: Kerbala University جامعة كربلاء

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Abstract

background: Beta-thalassemia major is one of the major health problems in our country. Many studies have confirmed the fact that, these patients have an increased susceptibility to bacterial infections, assumed to be the result of immunological changes.Objective: This study aimed to evaluate some of humoral immunological parameters of thalassemic patients by measuring their serum concentration of Immunoglobulin (IgG), IgM, IgA, and serum complement components C3 and C4.Patients & Methods: A total of forty five beta-thalassemia major patients who wereattending to the thalassemia center in Kerbala teaching pediatrics hospital (24 males and 21females) from January to August of 2009 and fifty of control children with matched age andsex were randomly selected from apparently healthy individuals without any history of recentor recurrent infections, were enrolled in the study. Serum IgG, IgM, IgA, C3 and C4 levelswere measured using Single Radial Immunodiffusion (SRID).Results: The mean of serum IgG, IgM &IgA showed a significant increment (P<0.01) in thalassemic patients as compared with apparently healthy control group, while the serum levels of C3 and C4 were significantly decreased (P<0.01) in thalassemic patients as compared with control group.Conclusion: The elevation of serum levels of studied immunoglobulins can be due to continuous exposure to antigens, repeated infections, and chronic liver disease, while the only probable cause of humoral immune deficiency were found in these patients were due to the decline of serum complement levels (C3 and C4).Recommendations: The determination of serum levels of immunoglobulins and complement components have a beneficial value for evaluation of immunological status of thalassaemia major patients and further work need to be done to evaluate another immunological parameters for example the serum level of cytokines to manipulate the immunological aspects of thalassemic patients.Key word: Beta-Thalassemia major, Immunoglobulin G, IgM, IgA, C3 and C4, (SRID).


Article
Effects of vitamin D supplementation on red cell indices and cytokines in patients with thalassemia: An open-label randomized clinical trial
آثار مكملات فيتامين (D) على مؤشرات الخلايا الحمراء ومستويات السيتوكينات في المرضى الذين يعانون من ثلاسيميا بيتا الرئيسية; تجربة سريرية عشوائية ذات علامة مفتوحة.

Authors: Raz Muhammed HamaSalih راز محمد حمه صالح --- Marwan S.M. Al-Nimer مروان صالح النمر
Journal: Al-Mustansiriyah Journal for Pharmaceutical Sciences مجلة المستنصرية للعلوم الصيدلانية ISSN: 18150993 Year: 2019 Volume: 19 Issue: 4 Pages: 170-179
Publisher: Al-Mustansyriah University الجامعة المستنصرية

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Abstract

Background and aimDeficiency of vitamin D is known as a health problem all over the world and a recognized clinical complication of beta thalassemia patients. Vitamin D acts as a hormone at the nuclear receptor rendering it a beneficial medication for a number of diseases. It is believed that vitamin Dis important in the modulation of the inflammation system by regulating the formation of inflammatory cytokines and immune cells. This study aimed to investigate the effect of vitamin D supplementation on the red cell indices and cytokines levels in patients with beta thalassemia major, in an open label randomized clinical trial.Patients and Methods: this study performed an open-label randomized clinical trial in patients with beta thalassemia major. Forty-six patients completed the eight weeks clinical trial and were allocated to administer oral vitamin D3 supplement of 100,000 IU every two weeks as an add-on treatment. During the study, hematological indices, serum iron, ferritin, vitamin D, calcium and inflammatory markers (interleukin-6, interleukin-2 and interleukin-10) were evaluated before (at baseline) and after vitamin D supplementation for 8 weeks.Results: Vitamin D3 supplements significantly decreases interleukin-6 levels and elevates the serum levels of anti-inflammatory cytokines IL-2 and IL-10, it also significantly reduced serum ferritin level, but it did not alter the hematological indices.Conclusion: Our results suggest that administration of vitamin D has a potential anti-inflammatory role in beta thalassemia patients and reduces serum ferritin levels, which may reduce the burdens of iron overload in thalassemia patients.

خلفية:يعرف نقص فيتامين (D) بأنه مشكلة صحية في جميع أنحاء العالم ومضاعفات سريرية معترف بها لمرضى الثلاسيميا بيتا. يعمل فيتامين (D) كهرمون في المستقبل النووي مما يجعله دواء مفيد لعدد من الأمراض. ويعتقد أن فيتامين (D) مهم في تعديل نظام الالتهابات من خلال تنظيم تشكيل السيتوكينات الالتهابية والخلايا المناعية.الهدف: تهدف هذه الدراسة إلى دراسة آثار مكملات فيتامين (D) على مؤشرات الخلايا الحمراء ومستويات السيتوكينات في المرضى الذين يعانون من ثلاسيميا بيتا الرئيسية، في تجربة سريرية عشوائية ذات علامة مفتوحة.الإعداد: مركز الثلاسيميا ، دليل الصحة ، المستشفى العام فی السليمانية، إقليم كوردستان - العراق.الطريقة:أجرينا تجربة سريرية عشوائية مفتوحة التسمية علی المرضى الذين يعانون من ثلاسيميا بيتا الرئيسية. أكمل ستة وأربعون مريضا التجربة السریریة (الكلينيكية) التي استمرت ثمانية أسابيع وتم تخصيصها لإعطاء مكملات فيتامين D3عن طريق الفم والتي تبلغ 100000 وحدة دولية IU كل أسبوعين كعلاج إضافي. خلال الدراسة، تم تقييم مؤشرات الدم، الحديد في الدم، فيريتين، فيتامين D ، الكالسيوم وعلامات الالتهابات (إنترلوكين -6، إنترلوكين -2 وإنترلوكين -10) قبل (في الأساس) وبعد مكملات فيتامين (D) لمدة ثمانیة أسابيع.النتائج: مكملات فيتامين D3 تقلل بشكل كبير مستويات إنترلوكين -6 وترفع مستويات مصل السيتوكينات المضادة للالتهابات IL-2 و IL-10 ، كما أنه يقلل بشكل كبير من مستوى فيريتين المصل، لكنه لم يغير مؤشرات الدم.خاتمة:تشير نتائجنا إلى أن تناول فيتامين (D) له دور محتمل مضاد للالتهابات في مرضى الثلاسيميا بيتا ويقلل من مستويات فيريتين المصل، مما قد يقلل من أعباء الحديد الزائدة في مرضى الثلاسيميا.


Article
Evaluating Health-Related Quality of Life (HRQoL) in Iraqi Adult and Pediatric Patients with Beta-Thalassemia Major Using Two Different Iron Chelation Therapies
تقييم جودة الحياة النوعية لمرضى الثلاسيميا الكبرى البالغين والاطفال في العراق الذين يستخدمون علاجين مختلفين لطاردات الحديد

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Abstract

Beta Thalassemia major (β-TM) is a common inherited blood disorder in Iraq. Iron overload is caused by repeated blood transfusion; which may result in severe complications. Patients with β-TM require lifelong adherence to a burdensome medical regimen which could potentially impact their ' quality of life (QoL). Hence, it is of utmost importance to pay a great attention to their Health-Related Quality of Life (HRQoL).A cross-sectional study was performed to evaluate health-related quality of life in both adult and pediatric patients using Deferoxamine (DFO) or Deferasirox (DFX) iron chelation therapies (ICTs) and to assess different factors affecting their elated quality of life. Two instruments were used to evaluate quality of life; the WHOQOL-BREF questionnaire was used to evaluate quality of life in adults (DFO group and DFX group), and PedsQL was used to evaluate quality of life in pediatrics (DFO group and DFX group). Several variables were assessed for correlation with quality of life domains.In adults patients; Health-Related Quality of Life (HRQoL) components were significantly lower in patients receiving Deferoxamine (DFO). In pediatric patients, only physical functioning score was significantly lower in Deferoxamine(DFO) receiving patients, the rest of the variables show no statistical differences. Pediatrics have slightly better quality of life (QoL) and lower complications in comparison to adults.Thalassemia impairs quality of life (QoL) in all patients significantly. Improving quality of life (QoL) is achieved by better control of iron overload, iron chelation therapies (ICTs) adverse effects, and complications.

مرض الثلاسيميا الكبرى من الامراض الوراثية الشائعة في العراق. فرط الحديد في الجسم ينتج عن نقل الدم المتكرر والذي يسبب مضافات شديدة. يجب على مرضى الثلاسيميا الكبرى الالتزام مدى الحياة بنظام علاجي مرهق والذي من الممكن ان يؤثر على جودة الحياة النوعية. لذا من الاهمية القصوى ان تولي اهتماما كبيرا بجوانب جودة الحياة النوعية لهم.اجريت هذه الراسة المستعرضة لتقييم مستوى جودة الحياة النوعية لمستخدمي طاردات الحديد Deferoxamine (DFO) او Deferasirox (DFX) للبالغين والاطفال ولتقييم عدة عوامل على جودة الحياة النوعية. تم استخدام نوعين من الاستبيانات لتقييم جودة الحياة النوعية. الاستبيان (WHOQOL-BREF) استخدم لتقييم جودة الحياة النوعية لدى الكبار والاستبيان (PedsQL) استخدم لتقييم جودة الحياة النوعية لدى الاطفال والمراهقين.وتم تقييم تأثير وارتباط عدة عوامل بجودة الحياة النوعية.في المرضى البالغيين كان مستوى جودة الحياة النوعية اقل لدى المرضى الذين يستعملون DFO. في الاطفال فقط درجة الاداء البدني كانت اقل من المرضى الذين يستخدمون ((DFX ولكن باقي المجالات لم يكن هناك اي فرق احصائي ملحوظ بين مرضى (DFO) ومرضى (DFX). ومن الملاحظ ان مستوى جودة الحياة النوعية للاطفال كانت اعلى قليلا ومعدل مضاعفات المرض كانت اقل لدى الاطفال عن ما هو في المرضى البالغين. مرض الثلاسيميا الكبرى يعيق جودة الحياة النوعيه للمرضى بشكل ملحوظ. تحسين جودة الحياة النوعية يتم بالسيطرة على فرط الحديد في الجسم و الحد من التأثيرات الجانبية لطوارد الحديد ومضاعفات المرض.

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