Abstract:
:Homozygous beta-thalassemia is usually characterized by severe anemia requiring regular blood transfusion for survival. For homozygous patients with milder clinical manifestations and no dependence on transfusion therapy, the term thalassemia intermedia is usually applied. Genetic mechanisms that may ameliorate the clinical expression of homozygous beta-thalassemia include coinheritance of alpha-thalassemia, inheritance of mild beta-globin gene mutations, and increased gamma-globin chain production, which may partially compensate for the lack of beta-globin chain synthesis. To identify which of these factors may contribute to the modification of childhood homozygous, high-hemoglobin A2 (HbA2) beta-thalassemia in Greece, the interaction of alpha-thalassemia, types of beta-thalassemia mutations, and the presence of a polymorphic site 5' to the G gamma-globin gene, which has been described as associated with increased gamma-globin chain production in some cases, was assessed. The results were analyzed in light of similar studies in 150 randomly selected, homozygous, high-HbA2 beta-thalassemia patients with the aim of assessing whether thalassemia genotypes can provide information useful for prognosis and/or more appropriate management of homozygous beta-thalassemia patients. The results indicate that, in general, the major factor modifying the clinical expression of homozygous, high-HbA2 beta-thalassemia in Greece is the inheritance of mild beta-thalassemia mutations. Although there is not always a complete correlation of genotype with clinical phenotype, the inheritance of two mild beta-thalassemia alleles results in almost all cases (11 of 12 cases in this study) in thalassemia intermedia phenotype.(ABSTRACT TRUNCATED AT 250 WORDS)
journal_name
Pediatr Hematol Oncoljournal_title
Pediatric hematology and oncologyauthors
Kanavakis E,Traeger-Synodinos J,Tzetis M,Metaxotou-Mavromati A,Ladis V,Kattamis Cdoi
10.3109/08880019509029526subject
Has Abstractpub_date
1995-01-01 00:00:00pages
37-45issue
1eissn
0888-0018issn
1521-0669journal_volume
12pub_type
杂志文章abstract::The reported long-term outcome of endemic Burkitt lymphoma (eBL) patients who present with paraplegia is largely unknown. Records of BL patients treated with comparable short-interval cyclophosphamide chemotherapy schedules between 2004 and 2014 at three Baptist mission hospitals in Cameroon were reviewed. Survivors w...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,多中心研究
doi:10.3109/08880018.2015.1085936
更新日期:2015-01-01 00:00:00
abstract::Euthyroid sick syndrome is related to profound changes in thyroid metabolism induced by nonthyroidal diseases. To determine whether children with newly diagnosed Hodgkin disease might present thyroid abnormalities and to establish their predictive value, the authors performed regular thyroid function testing. Seven ch...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010151114886
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abstract::Modern treatment protocols lead to complete remission in a high proportion of patients with childhood acute lymphoblastic leukemia (ALL). However, a large number of them show a relapse of the disease. Treatment failure in these patients is mainly attributable to de novo or acquired resistance to a wide variety of cyto...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010600719303
更新日期:2006-09-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010390232763
更新日期:2003-10-01 00:00:00
abstract::Nesiritide is a recombinant formulation of B-type natriuretic peptide used most commonly in the treatment of adults with decompensated congestive heart failure. The physiologic effects of BNP include natriuresis, diuresis, and smooth muscle relaxation. These physiologic effects result in its beneficial therapeutic eff...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010590935248
更新日期:2005-06-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2020.1779885
更新日期:2020-11-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019409141651
更新日期:1994-03-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010802016342
更新日期:2008-06-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
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更新日期:1996-05-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.3109/08880018.2013.859189
更新日期:2014-05-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
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更新日期:2007-09-01 00:00:00
abstract:BACKGROUND:We aimed to examine the association between vitamin D deficiency and anemia in a nationally representative sample of Korean children and adolescents. METHODS:Cross-sectional data on 2526 children and adolescents aged 10-20 years from the Korea National Health and Nutrition Examination Survey-V (2010-2012) w...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,多中心研究
doi:10.3109/08880018.2014.983623
更新日期:2015-03-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010050120854
更新日期:2000-09-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800100276712
更新日期:2000-01-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.3109/08880018.2014.909913
更新日期:2015-02-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,多中心研究
doi:10.3109/08880018.2015.1102998
更新日期:2016-02-01 00:00:00
abstract::A patient with well-differentiated monoblastic leukemia (ANLL FAB-M5b) is described in whom acute leukemia was diagnosed 25 months after having completed postoperative adjuvant chemotherapy for osteogenic sarcoma of the femur. All analyzed metaphases showed 48xy, dup 1(q12), +3, +9. ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018809031250
更新日期:1988-01-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.1080/088800102753356149
更新日期:2002-01-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800100276532
更新日期:2000-03-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:2004-01-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010600646324
更新日期:2006-07-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019509029555
更新日期:1995-03-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019309016525
更新日期:1993-01-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019109028803
更新日期:1991-10-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800101750238568
更新日期:2001-06-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880019809014013
更新日期:1998-07-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2011.642941
更新日期:2012-02-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880018.2013.798058
更新日期:2013-10-01 00:00:00
abstract::A case of a child with paroxysmal nocturnal hemoglobinuria (PNH) is characterized by an increased sensitivity of the erythrocyte to hemolytic action of complement. The widely used Ham test may not always be reliable. Recently, a panel of monoclonal antibodies has become available to detect various glycosylphosphatidyl...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019309029514
更新日期:1993-10-01 00:00:00
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journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2010.493574
更新日期:2010-10-01 00:00:00