Open AccessJournal Article
Hematological and biochemical evaluation of β-thalassemia major (βTM) patients in Gaza Strip: A cross-sectional study.
Hani Ayyash,Mahmoud M. Sirdah +1 more
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TLDR
The necessity for strengthening the efforts for regular evaluation and follow-up of the βTM patients which could be used to improve or modify the management protocols and thus ameliorating their deteriorated hematological and biochemical status is justified.Abstract:
Objectives In Gaza Strip, Palestine, β-thalassemia is a major public health problem where more than 300 β-thalassemia major (βTM) patients are currently being managed at governmental hospitals. We set up to evaluate the hematological and biochemical aspects of our βTM patients at the Gaza European hospital and their correlation with iron overload. Methods Our study included 65 transfusion-dependent βTM, as well as 37 apparently healthy subjects as control group. The hematological and biochemical evaluations included complete blood count, coagulation profile liver and kidney function tests, fasting blood sugar, lipid profile, and serum ferritin. Results Deteriorated hematological and biochemical statuses were reported in both males and females of βTM patients as compared to the control group. Statistical comparisons showed no significant differences between males and females βTM patients in all parameters except for total cholesterol. The results concerning the splenectomized versus non-splenectomized patients revealed significantly higher values in splenectomized patients for white blood cell (WBC), platelet, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, albumin, total protein, cholesterol, and potassium concentration compared to the non-splenectomized patients. Patients infected with hepatitis C virus and/or hepatitis B virus showed significant decrease in WBC count as compared to infection free patients, while for serum urea and creatinine, the virally infected βTM patients revealed significantly higher values compared to infection free patients. Conclusion This study justified the necessity for strengthening the efforts for regular evaluation and follow-up of the βTM patients which could be used to improve or modify the management protocols and thus ameliorating their deteriorated hematological and biochemical status.read more
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Clinical and hematological features among β-thalassemia major patients in Jazan region: A hospital-based study
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References
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Journal ArticleDOI
HbVar database of human hemoglobin variants and thalassemia mutations: 2007 update.
Belinda Giardine,Sjozef van Baal,Polynikis Kaimakis,Cathy Riemer,Webb Miller,Maria Samara,Panagoula Kollia,Nicholas P. Anagnou,David H.K. Chui,Henri Wajcman,Ross C. Hardison,George P. Patrinos +11 more
TL;DR: HbVar (http://globin.bx.psu.edu/hbvar) is a locus-specific database (LSDB) developed in 2001 by a multi-center academic effort to provide timely information on the genomic sequence changes leading to hemoglobin variants and all types of thalassemia and hemoglobinopathies as mentioned in this paper.
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Molecular basis of β thalassemia and potential therapeutic targets
TL;DR: Identification of the genetic variants modifying fetal hemoglobin (HbF) production in combination with α globin genotype provide some prediction of disease severity for β thalassemia but generation of a personalized genetic risk score to inform prognosis and guide management requires a larger panel of genetic modifiers yet to be discovered.
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Overwhelming post splenectomy infection syndrome – Review study
TL;DR: Although relatively rare, overwhelming postsplenectomy infection has a high mortality rate with delayed or inadequate treatment, and therefore it is important for Emergency Physicians to be familiar with it.
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Splenectomy and thrombosis: the case of thalassemia intermedia.
Ali T. Taher,Khaled M. Musallam,Mehran Karimi,Amal El-Beshlawy,Khawla Belhoul,Shahina Daar,Mohamed-SalahEldin Saned,Claudia Cesaretti,Maria Domenica Cappellini +8 more
TL;DR: Mannucci et al. as discussed by the authors studied the clinical and laboratory characteristics of patients who eventually develop overt thromboembolic events (TEE) are poorly studied, and found that splenectomized patients who will develop TEE may be identified early on by high NRBC and platelet counts, evidence of pulmonary hypertension (PHT), and transfusion naivety.
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Clinical Classification, Screening and Diagnosis for Thalassemia.
TL;DR: This classification has embraced all other forms of thalassemia syndromes such as α-thalassemia, hemoglobin E/β-thalassesmia and combined α- and β-thalasemias.