Literature DB >> 29207734

Prenatal Screening for Rare Co-Inheritance of HbE and β-Thalassaemia Traits in Western India.

Parth S Shah1, Hari Shankar P Ray2, Ketan K Vaghasia3, Sandip C Shah4, Mandava V Rao5.   

Abstract

The mutations in Haemoglobin Beta (HBB) gene, bring about less or no production of Hb β-chain synthesis in affected cases, leading from minor to major types depending on haematological indices. In compound heterozygotic conditions, two traits are involved, in which one parent has HbE trait and the other has β-thalassaemia carrier (trait). Here, we report a family of Rajasthan, West India which had a proband (son) having HbE/ β-thalassaemia a co-inherited compound heterozygosity as revealed by DNA sequencing. It also contained upper levels of HbE with altered Hb and red cell indices showing asymptomatic to symptomatic state requiring blood transfusion periodically. The parents and Chorionic Villus Sampling (CVS) were HbE and β-thalassaemia traits only. Such case is rare in Western India and we recommend this family for genetic counseling and genetic testing before they want reproductive choices in future for better management in a society.

Entities:  

Keywords:  Compound heterozygosity; Electrophoresis; Genetic testing; Phenotypic indices; Sequence analysis

Year:  2017        PMID: 29207734      PMCID: PMC5713756          DOI: 10.7860/JCDR/2017/26068.10674

Source DB:  PubMed          Journal:  J Clin Diagn Res        ISSN: 0973-709X


  7 in total

1.  Changes in the epidemiology of thalassemia in North America: a new minority disease.

Authors:  Elliott P Vichinsky; Eric A MacKlin; John S Waye; Fred Lorey; Nancy F Olivieri
Journal:  Pediatrics       Date:  2005-11-15       Impact factor: 7.124

2.  Hemoglobin E-beta thalassemia in Uttar Pradesh.

Authors:  S Agarwal; R Gulati; K Singh
Journal:  Indian Pediatr       Date:  1997-04       Impact factor: 1.411

3.  Global epidemiology of haemoglobin disorders and derived service indicators.

Authors:  Bernadette Modell; Matthew Darlison
Journal:  Bull World Health Organ       Date:  2008-06       Impact factor: 9.408

Review 4.  The hemoglobin E thalassemias.

Authors:  Suthat Fucharoen; David J Weatherall
Journal:  Cold Spring Harb Perspect Med       Date:  2012-08-01       Impact factor: 6.915

5.  Clinical, hematologic and molecular variability of sickle cell-β thalassemia in western India.

Authors:  Malay B Mukherjee; Anita H Nadkarni; Ajit C Gorakshakar; Kanjaksha Ghosh; Dipika Mohanty; Roshan B Colah
Journal:  Indian J Hum Genet       Date:  2010-09

Review 6.  Hb E/beta-thalassaemia: a common & clinically diverse disorder.

Authors:  Nancy F Olivieri; Zahra Pakbaz; Elliott Vichinsky
Journal:  Indian J Med Res       Date:  2011-10       Impact factor: 2.375

7.  Prevalence of thalassemia and hemoglobinopathy in eastern India: A 10-year high-performance liquid chromatography study of 119,336 cases.

Authors:  Santosh Kumar Mondal; Saikat Mandal
Journal:  Asian J Transfus Sci       Date:  2016 Jan-Jun
  7 in total

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