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[PMID]:29318368
[Au] Autor:Mirlohi MS; Yaghooti H; Shirali S; Aminasnafi A; Olapour S
[Ad] Endereço:Hyperlipidemia Research Center, School of Allied Medical Sciences, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.
[Ti] Título:Increased levels of advanced glycation end products positively correlate with iron overload and oxidative stress markers in patients with ß-thalassemia major.
[So] Source:Ann Hematol;97(4):679-684, 2018 Apr.
[Is] ISSN:1432-0584
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:The impaired biosynthesis of the ß-globin chain in ß-thalassemia leads to the accumulation of unpaired alpha globin chains, failure in hemoglobin formation, and iron overload due to frequent blood transfusion. Iron excess causes oxidative stress and massive tissue injuries. Advanced glycation end products (AGEs) are harmful agents, and their production accelerates in oxidative conditions. This study was conducted on 45 patients with major ß-thalassemia who received frequent blood transfusions and chelation therapy and were compared to 40 healthy subjects. Metabolic parameters including glycemic and iron indices, hepatic and renal functions tests, oxidative stress markers, and AGEs (carboxymethyl-lysine and pentosidine) levels were measured. All parameters were significantly increased in ß-thalassemia compared to the control except for glutathione levels. Blood glucose, iron, serum ferritin, non-transferrin-bound iron (NTBI), MDA, soluble form of low-density lipoprotein receptor, glutathione peroxidase, total reactive oxygen species (ROS), and AGE levels were significantly higher in the ß-thalassemia patients. Iron and ferritin showed a significant positive correlation with pentosidine (P < 0.01) but not with carboxymethyl-lysine. The NTBI was markedly increased in the ß-thalassemia patients, and its levels correlated significantly with both carboxymethyl-lysine and pentosidine (P < 0.05). Our findings confirm the oxidative status generated by the iron overload in ß-thalassemia major patients and highlight the enhanced formation of AGEs, which may play an important role in the pathogenesis of ß-thalassemia major.
[Mh] Termos MeSH primário: Transfusão de Sangue
Produtos Finais de Glicação Avançada/sangue
Sobrecarga de Ferro/etiologia
Estresse Oxidativo
Reação Transfusional/fisiopatologia
Talassemia beta/sangue
[Mh] Termos MeSH secundário: Adolescente
Adulto
Biomarcadores/sangue
Terapia por Quelação/efeitos adversos
Terapia Combinada/efeitos adversos
Estudos Transversais
Desferroxamina/uso terapêutico
Feminino
Seres Humanos
Irã (Geográfico)
Sobrecarga de Ferro/prevenção & controle
Masculino
Piridonas/uso terapêutico
Receptores Depuradores Classe E/sangue
Adulto Jovem
Talassemia beta/terapia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers); 0 (Glycation End Products, Advanced); 0 (OLR1 protein, human); 0 (Pyridones); 0 (Scavenger Receptors, Class E); 2BTY8KH53L (deferiprone); J06Y7MXW4D (Deferoxamine)
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180309
[Lr] Data última revisão:
180309
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180111
[St] Status:MEDLINE
[do] DOI:10.1007/s00277-017-3223-3


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[PMID]:28471307
[Au] Autor:Bonifazi F; Conte R; Baiardi P; Bonifazi D; Felisi M; Giordano P; Giannuzzi V; Iacono A; Padula R; Pepe A; Caterina Putti M; Ruggieri L; Carlo Del Vecchio G; Filosa A; Maggio A; Ceci A; HTA-THAL Multiregional Registry
[Ad] Endereço:a Fondazione per la Ricerca Farmacologica Gianni Benzi Onlus , Valenzano ( BA ), Italy.
[Ti] Título:Pattern of complications and burden of disease in patients affected by beta thalassemia major.
[So] Source:Curr Med Res Opin;33(8):1525-1533, 2017 Aug.
[Is] ISSN:1473-4877
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:OBJECTIVES: Despite the correct application of blood transfusions and chelation treatments, beta thalassemia patients have many complications. Systematic population analyses on types and frequency of these complications are very few. The aim of this study is to characterize the complications, their risk factors and their clinical and economic impact. METHODS: Complications at baseline and events occurring during one observational year were analyzed in 272 patients aged >12 years. Risk factors were analyzed through chi-squared and unpaired t tests. Logistic regression was applied to perform the risk factors multivariate analysis. RESULTS: A total of 554 complications (1-6 per patient) affected 82.3% of patients. Cardiac complications were less represented than expected. Musculoskeletal diseases were the most represented complications followed by hepatic, sexual and endocrine diseases. Splenectomized patients, born before 1970 and aged >40 years, starting iron chelation therapy when aged >4 years or after receiving more than 20 blood transfusions, presented a significantly higher number of complications. A total of 885 adverse events requiring 34125 additional medical services occurred in 1 year. Of these, 34.9% were related to treatments and 65.1% to other causes. Event numbers, additional medical interventions and cost increased progressively in patients affected by one or more complication compared to patients with no complications. CONCLUSIONS: The pattern of complications changes according to birth cohort and differentiates older from younger patients. The burden of the disease and its costs increase after the onset of the first complication, therefore prevention of complications is fundamental in these patients.
[Mh] Termos MeSH primário: Transfusão de Sangue/métodos
Terapia por Quelação/métodos
Talassemia beta/complicações
[Mh] Termos MeSH secundário: Adolescente
Adulto
Criança
Feminino
Seres Humanos
Modelos Logísticos
Masculino
Adulto Jovem
Talassemia beta/terapia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180213
[Lr] Data última revisão:
180213
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170505
[St] Status:MEDLINE
[do] DOI:10.1080/03007995.2017.1326890


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[PMID]:29384898
[Au] Autor:Zhang X; Hao W; Xu T; Liu S; Jiang H
[Ti] Título:Diagnosis and treatment of neoplastic post-transplant lymphoproliferative disorder following hematopoietic stem cell transplant in ß-thalassemia: A pediatric case report.
[So] Source:Medicine (Baltimore);96(52):e9055, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: Post-transplant lymphoproliferative disorder (PTLD) is the most common form of lymphoproliferation in childhood and is associated with significant morbidity and mortality. In this report we reviewed the case of a pediatric patient who experienced PTLD after allogeneic hematopoietic stem cell transplantation (HSCT) from a human leukocyte antigen (HLA)-identical sibling. METHODS: The clinical characteristics, diagnosis, and treatment of PTLD after sibling HSCT in a 4-year-old boy with severe ß-thalassemia was retrospectively reviewed. RESULTS: Medical records revealed the patient developed a fever and superficial lymphadenopathy and soft palate enlargement 8 months post-HSCT. Pathologic diagnosis indicated non-Hodgkin lymphoma (B-cell type), which resulted in a reduced dose of immunosuppressant and the initiation of chemotherapy (administered according to the BFM95 protocol for 2 courses; 4 courses of rituximab therapy was also administered). Currently, the patient has been disease-free for over 3 years. There are no specific guidelines for the treatment of PTLD. The status of stem cell implantation after transplantation, and graft versus host disease should be evaluated jointly, and rituximab therapy and chemotherapy with BFM-95 may be used for treatment of pediatric PTLD after HSCT. CONCLUSION: The current case represents a unique opportunity to review a pediatric patient with ß-thalassemia. The successful treatment of post-transplant non-Hodgkin B lymphoma may help other physicians in the management of similar pediatric cases.
[Mh] Termos MeSH primário: Transplante de Células-Tronco Hematopoéticas/efeitos adversos
Linfoma não Hodgkin/diagnóstico
Linfoma não Hodgkin/terapia
Talassemia beta/terapia
[Mh] Termos MeSH secundário: Pré-Escolar
Antígenos HLA
Seres Humanos
Linfoma não Hodgkin/etiologia
Masculino
Talassemia beta/complicações
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (HLA Antigens)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180209
[Lr] Data última revisão:
180209
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180201
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009055


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[PMID]:29365300
[Au] Autor:Gupta A; Jain P
[Ad] Endereço:Max Super Specialty Hospital, Dehradun, India mail.guptaankur@gmail.com.
[Ti] Título:Calcified Spleen and Gallstones.
[So] Source:N Engl J Med;378(4):380, 2018 Jan 25.
[Is] ISSN:1533-4406
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Calcinose/diagnóstico por imagem
Cálculos Biliares/diagnóstico por imagem
Esplenopatias/diagnóstico por imagem
[Mh] Termos MeSH secundário: Adulto
Calcinose/complicações
Cálculos Biliares/complicações
Hepatite C Crônica/complicações
Seres Humanos
Masculino
Radiografia
Baço/diagnóstico por imagem
Esplenopatias/complicações
Tomografia Computadorizada por Raios X
Talassemia beta/complicações
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180208
[Lr] Data última revisão:
180208
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180125
[St] Status:MEDLINE
[do] DOI:10.1056/NEJMicm1703915


  5 / 6821 MEDLINE  
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[PMID]:28450766
[Au] Autor:Vincent O; Oluwaseyi B; James B; Saidat L
[Ad] Endereço:Haematology and Blood Transfusion Science Department, University of Lagos, Nigeria.
[Ti] Título:Coinheritance of B-Thalassemia and Sickle Cell Anaemia in Southwestern Nigeria.
[So] Source:Ethiop J Health Sci;26(6):517-522, 2016 Nov.
[Is] ISSN:2413-7170
[Cp] País de publicação:Ethiopia
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Genes for haemoglobin S are found in high frequencies in Nigeria. However, there is little information on beta thalassemia in sickle cell anaemia in this population. The clinical presentation of HbS- ß thalassemia is enormously variable, ranging from an asymptomatic state to a severe disorder similar to homozygous sickle cell disease. MATERIALS AND METHODS: Haemoglobin A and HbF were determined in sickle cell anaemia patients attending LAUTECH Teaching Hospital, Osogbo, by elution after electrophoresis and alkaline denaturation methods respectively. Haematological parameters were estimated using Sysmex KX-21N and percentage target cells using Leishman's staining technique. RESULTS: Exactly 6% f the SCA patients were found to have elevated HbA (>3.3%) and HbF (>1.3%). These patients also had normal erythrocyte indices, increased platelet count, a significantly higher HCT and an increased % target cell. CONCLUSION: These findings confirm that the frequency of beta thalassaemia in sickle cell patients in Nigeria is higher than previously thought. It is therefore important to consider the possibility of this variant in patients with sickle cell anaemia since their course may differ from that of patients with homozygous sickle cell anaemia.
[Mh] Termos MeSH primário: Anemia Falciforme/epidemiologia
Anemia Falciforme/genética
Talassemia beta/epidemiologia
Talassemia beta/genética
[Mh] Termos MeSH secundário: Estudos Transversais
Índices de Eritrócitos
Feminino
Hemoglobina Fetal/análise
Hematócrito
Hemoglobina A2/análise
Seres Humanos
Masculino
Nigéria/epidemiologia
Contagem de Plaquetas
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
9034-53-1 (Hemoglobin A2); 9034-63-3 (Fetal Hemoglobin)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180205
[Lr] Data última revisão:
180205
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170429
[St] Status:MEDLINE


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[PMID]:28467346
[Au] Autor:Soliman AT; De Sanctis V; Yassin M; Wagdy M; Soliman N
[Ad] Endereço:. vdesanctis@libero.it.
[Ti] Título:Chronic anemia and thyroid function.
[So] Source:Acta Biomed;88(1):119-127, 2017 Apr 28.
[Is] ISSN:0392-4203
[Cp] País de publicação:Italy
[La] Idioma:eng
[Ab] Resumo:Anaemia is a global public health problem affecting both developing and developed countries with major consequences for human health as well as social and economic development. It occurs at all stages of the life cycle, but is more prevalent in pregnant women and young children. Iron deficiency anaemia (IDA) impairs thyroid metabolism in animals and human and may negatively affect growth and develpment of children. On the other hand both overt and subclinical hypothyroidism are associated with anemia and adding iron to thyroxine therapy improves both conditions compared to thyroxine therapy alone. In addition patients with chronic hemolytic anemia requiring repeated blood transfusion have high prevalence of hypothalamic-pituitary thyroid axis. Both primary hypothyroidism and central hypothyroidism occur in these patients with increasing prevalence with age, severity of the anemia and higher ferritin concentration denoting poor chelation.  Proper blood transfusion and intensive chelation appears to prevent deterioration of thyroid function and in many cases can reverse thyroid pathology. Physicians treating these forms of anemia should be aware of thyroid disorders in these patients for early screening, prevention and proper management of any thyroid dysfunction.
[Mh] Termos MeSH primário: Anemia Ferropriva/complicações
Hipotireoidismo/etiologia
[Mh] Termos MeSH secundário: Anemia Ferropriva/terapia
Anemia Falciforme/complicações
Anemia Falciforme/terapia
Animais
Transfusão de Sangue
Terapia por Quelação
Suplementos Nutricionais
Seres Humanos
Hipotireoidismo/terapia
Ferro/uso terapêutico
Talassemia beta/complicações
Talassemia beta/terapia
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
E1UOL152H7 (Iron)
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180125
[Lr] Data última revisão:
180125
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170504
[St] Status:MEDLINE
[do] DOI:10.23750/abm.v88i1.6048


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[PMID]:29200160
[Au] Autor:Gomber S; Dabas A; Bagmar S; Madhu SV
[Ad] Endereço:Departments of Pediatrics.
[Ti] Título:Glucose Homeostasis and Effect of Chelation on ß Cell Function in Children With ß-Thalassemia Major.
[So] Source:J Pediatr Hematol Oncol;40(1):56-59, 2018 Jan.
[Is] ISSN:1536-3678
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: To assess the prevalence of impaired glucose tolerance in ß-thalassemia major and correlate it with chelation therapy. MATERIALS AND METHODS: Sixty-seven subjects with ß-thalassemia major, aged 1 to 20 years, were enrolled in our prospective cohort. Clinical details were recorded. Baseline oral glucose tolerance test, serum insulin, C peptide, and insulin resistance were measured. The biochemical profile was repeated after 6 months. RESULTS: The mean age of subjects was 7.43±4.48 years. Eight (11.9%) subjects had impaired fasting glucose, 7 (10.4%) had impaired glucose tolerance, and 1 (1.4%) subject had diabetes at baseline. Subjects with abnormal glucose profile had longer disease duration (95% confidence interval [CI] of difference=-6.64 to -0.68; P=0.019) and higher fasting blood glucose (95% CI of difference=-32.1 to -10.5; P=0.001) and serum ferritin (95% CI of difference=-219.8 to -3.4; P=0.001) than normoglycemic subjects. Insulin resistance and serum ferritin showed significant increase at 6 months (P<0.001 and P=0.001, respectively). Patients on deferiprone alone significantly improved glucose homeostasis on follow-up than those on desferrioxamine or combination therapy of desferrioxamine and deferiprone (P<0.05). CONCLUSIONS: Prolonged disease duration and higher serum ferritin adversely affects glucose homeostasis in thalassemic children. Deferiprone was the most effective chelator to improve glucose homeostasis in chronically transfused thalassemics.
[Mh] Termos MeSH primário: Glicemia/fisiologia
Terapia por Quelação
Desferroxamina/uso terapêutico
Intolerância à Glucose/tratamento farmacológico
Homeostase/efeitos dos fármacos
Piridonas/uso terapêutico
Talassemia beta/complicações
[Mh] Termos MeSH secundário: Adolescente
Glicemia/efeitos dos fármacos
Criança
Pré-Escolar
Desferroxamina/farmacologia
Feminino
Ferritinas/sangue
Intolerância à Glucose/etiologia
Intolerância à Glucose/fisiopatologia
Seres Humanos
Lactente
Resistência à Insulina
Quelantes de Ferro/farmacologia
Quelantes de Ferro/uso terapêutico
Sobrecarga de Ferro/tratamento farmacológico
Sobrecarga de Ferro/etiologia
Masculino
Estudos Prospectivos
Piridonas/farmacologia
Adulto Jovem
Talassemia beta/sangue
Talassemia beta/metabolismo
Talassemia beta/terapia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Blood Glucose); 0 (Iron Chelating Agents); 0 (Pyridones); 2BTY8KH53L (deferiprone); 9007-73-2 (Ferritins); J06Y7MXW4D (Deferoxamine)
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180112
[Lr] Data última revisão:
180112
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171205
[St] Status:MEDLINE
[do] DOI:10.1097/MPH.0000000000001043


  8 / 6821 MEDLINE  
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[PMID]:28801997
[Au] Autor:Nakavachara P; Petchkul J; Jeerawongpanich K; Kiattisakthavee P; Manpayak T; Netsakulnee P; Chaichanwattanakul K; Pooliam J; Srichairatanakool S; Viprakasit V
[Ad] Endereço:Division of Pediatric Endocrinology, Faculty of Medicine Siriraj Hospital, Department of Pediatrics, Mahidol University, Bangkok, Thailand.
[Ti] Título:Prevalence of low bone mass among adolescents with nontransfusion-dependent hemoglobin E/ß-thalassemia and its relationship with anemia severity.
[So] Source:Pediatr Blood Cancer;65(1), 2018 Jan.
[Is] ISSN:1545-5017
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Low bone mass is common among adolescents with transfusion-dependent ß-thalassemia despite adequate transfusion and iron chelation. However, there are few reports regarding bone mineral density (BMD) among adolescents with nontransfusion-dependent thalassemia (NTDT). Indeed, only BMD data in patients with nontransfusion-dependent (NTD) ß-thalassemia intermedia have been reported. No previous study has investigated BMD among adolescents with NTD hemoglobin (Hb) E/ß-thalassemia. OBJECTIVE: To determine the prevalence of low bone mass among adolescents with NTD Hb E/ß-thalassemia and factors relating to low bone mass. METHODS: We investigated BMD of lumbar spine (L2-L4; BMDLS) and total body (BMDTB), as measured by dual-energy X-ray absorptiometry, in 22 adolescents (aged 13.2-20 years) with NTD Hb E/ß-thalassemia. RESULTS: Low bone mass was found to be 18.2% and 22.7% at the lumbar spine (BMDLS Z-score adjusted for bone age and height age) and 13.6% and 9.1% at the total body (BMDTB Z-score adjusted for bone age and height age). Patients with mean Hb level <8 g/dl were more likely to have low bone mass (BMDLS and BMDTB Z-scores adjusted for bone age) compared to those with Hb level ≥ 8 g/dl. Mean Hb level correlated with BMDLS and BMDTB Z-scores adjusted for bone age. CONCLUSION: We demonstrated that a low Hb level was associated with low bone mass among adolescents with NTD Hb E/ß-thalassemia. A significant proportion of low bone mass among these patients highlights the importance of appropriate management, including red cell transfusion, vitamin D and calcium supplementation for improved long-term bone health.
[Mh] Termos MeSH primário: Absorciometria de Fóton
Densidade Óssea
Vértebras Lombares
Talassemia beta
[Mh] Termos MeSH secundário: Adolescente
Adulto
Feminino
Hemoglobina E
Seres Humanos
Vértebras Lombares/diagnóstico por imagem
Vértebras Lombares/metabolismo
Vértebras Lombares/fisiopatologia
Masculino
Índice de Gravidade de Doença
Talassemia beta/diagnóstico por imagem
Talassemia beta/metabolismo
Talassemia beta/fisiopatologia
[Pt] Tipo de publicação:CLINICAL TRIAL; JOURNAL ARTICLE; MULTICENTER STUDY
[Nm] Nome de substância:
9034-61-1 (Hemoglobin E)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171129
[Lr] Data última revisão:
171129
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170813
[St] Status:MEDLINE
[do] DOI:10.1002/pbc.26744


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[PMID]:28748286
[Au] Autor:Klaihmon P; Vimonpatranon S; Noulsri E; Lertthammakiat S; Anurathapan U; Sirachainan N; Hongeng S; Pattanapanyasat K
[Ad] Endereço:Graduate Program in Immunology, Department of Immunology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
[Ti] Título:Normalized levels of red blood cells expressing phosphatidylserine, their microparticles, and activated platelets in young patients with ß-thalassemia following bone marrow transplantation.
[So] Source:Ann Hematol;96(10):1741-1747, 2017 Oct.
[Is] ISSN:1432-0584
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:Bone marrow transplantation (BMT) serves as the only curative treatment for patients with ß-thalassemia major; however, hemostatic changes have been observed in these BMT patients. Aggregability of thalassemic red blood cells (RBCs) and increased red blood cell-derived microparticles (RMPs) expressing phosphatidylserine (PS) are thought to participate in thromboembolic events by initially triggering platelet activation. To our knowledge, there has been no report providing quantitation of these circulating PS-expressing RBCs and RMPs in young ß-thalassemia patients after BMT. Whole blood from each subject was fluorescently labeled to detect RBC markers (CD235a) and annexin-V together with the known number TruCount™ beads. PS-expressing RBCs, RMPs, and activated platelets were identified by flow cytometry. In our randomized study, we found the decreased levels of three aforementioned factors compared to levels in patients receiving regular blood transfusion (RT). This study showed that BMT in ß-thalassemia patients decreases the levels of circulating PS-expressing RBCs, their MPs, and procoagulant platelets when compared to patients who received RT. Normalized levels of these coagulation markers may provide the supportive evidence of the effectiveness of BMT for curing thalassemia.
[Mh] Termos MeSH primário: Plaquetas/metabolismo
Transplante de Medula Óssea
Micropartículas Derivadas de Células/metabolismo
Eritrócitos/metabolismo
Fosfatidilserinas/sangue
Ativação Plaquetária
Talassemia beta
[Mh] Termos MeSH secundário: Adolescente
Aloenxertos
Anexina A5/sangue
Criança
Feminino
Seres Humanos
Masculino
Talassemia beta/sangue
Talassemia beta/terapia
[Pt] Tipo de publicação:CLINICAL TRIAL; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Annexin A5); 0 (Phosphatidylserines)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171129
[Lr] Data última revisão:
171129
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170728
[St] Status:MEDLINE
[do] DOI:10.1007/s00277-017-3070-2


  10 / 6821 MEDLINE  
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[PMID]:29049312
[Au] Autor:Borgio JF
[Ad] Endereço:Department of Genetic Research, Institute for Research and Medical Consultation (IRMC), Imam Abdulrahman Bin Faisal University (Formerly: University of Dammam), Dammam, Saudi Arabia.
[Ti] Título:Impact of annotation error in α-globin genes on molecular diagnosis.
[So] Source:PLoS One;12(10):e0185270, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Recent studies on the variants in duplicated human alpha globin genes (HBA2 and HBA1) actively target the α-globin gene as molecular modulators for the treatment of ß-thalassemia major. Identification of the exact position of variant in HBA1, HBA2 or its patchworks is mandatory to support the therapeutic aims in ß-thalassemia major, by identifying specific modulators for the reactivation of fetal hemoglobin production. Hence, accurate identification of the variants in α-globin genes is crucial for the proper diagnosis, treatment and genetic counseling. METHOD: The objective was to reveal the annotation errors produced in α-globin gene sequence analysis while using different analytic tools. An HBA2 gene sequence with the HBA2:c.95+2_95+6delTGAGG variant and a recently reported HBA12 gene convert have been taken as examples to prove annotation error in α-globin gene from different analytic tools. RESULTS AND DISCUSSION: Although various bioinformatics tools used to predict variants are usually of high reliability, the current study using the an alpha globin 2 sequence with the HBA2:c.95+2_95+6delTGAGG variant and a recently reported HBA12 gene convert, has showcased ambiguous outputs among the three bioinformatics tools used and against the manual analytical method adopted. CONCLUSIONS: This report emphasizes the necessity for caution in the usage of DNA sequence analysis tools during molecular diagnosis and the importance of the selection of more appropriate tools for analysis. Furthermore, ethnic specific sequences should be considered as reference sequence for the analysis to bypass sequence dissimilarities among diverse populations.
[Mh] Termos MeSH primário: alfa-Globinas/genética
Talassemia beta/diagnóstico
[Mh] Termos MeSH secundário: Seres Humanos
Técnicas de Diagnóstico Molecular
Talassemia beta/genética
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (alpha-Globins)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171107
[Lr] Data última revisão:
171107
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171020
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0185270



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