Literature DB >> 26339094

Sickle Cell Disease: Reappraisal of the Role of Foetal Haemoglobin Levels in the Frequency of Vaso-Occlusive Crisis.

C Antwi-Boasiako1, E Frimpong1, G K Ababio2, B Dzudzor2, I Ekem3, B Gyan4, N A Sodzi-Tettey1, D A Antwi1.   

Abstract

BACKGROUND: Foetal haemoglobin has been implicated in the modulation of sickle cell crisis. Its level is generally inversely proportional to the severity of sickle cell disease (SCD) for a given sickle cell phenotypes. The main aim of therapy for vaso-occlusive crisis (VOC), which is the hallmark of SCD, is to reduce the chances of sickling through the prevention of polymerization of HbS. One way of preventing this polymerization is by increasing foetal haemoglobin levels.
OBJECTIVES: To determine the relationship between HbF levels and the frequency of crisis in SCD patients in Ghana.
METHOD: A longitudinal retrospective survey covering a period of 30 months was carried out on adult SCD patients at the Sickle Cell Clinic of the Korle-Bu Teaching Hospital.
RESULTS: Eighty-three adults aged 15 to 65 years made up of 40 males and 43 femalea were studied. Analysis of variance (ANOVA) gave significant results in Hb and HbF levels. Higher HbF levels were positively related to less frequent crisis and were significantly high in SCD patients than in controls. HbF effects on the clinical manifestations on SCD were variable.
CONCLUSION: Threshold values of HbF play a role in reducing the frequency of vaso-occlusive crisis in SCD patients and this finding contributes to the body of available literature on SCD severity. However our work does not give the apparent threshold level of helpful HBF Level in SCD.

Entities:  

Keywords:  Frequency of crisis; Haemoglobin F; sickle cell disease

Mesh:

Substances:

Year:  2015        PMID: 26339094      PMCID: PMC4549825          DOI: 10.4314/gmj.v49i2.7

Source DB:  PubMed          Journal:  Ghana Med J        ISSN: 0016-9560


  37 in total

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  5 in total

1.  Pediatric Patients With Sickle Cell Disease at a Public Hospital: Nutrition, Compliance and Early Experience With L-Glutamine Therapy.

Authors:  Moran Gotesman; Guy Elgar; Laura Hernandez Santiago; Abigail Alvarez; Youngju Pak; Henry J Lin; Joseph L Lasky; Eduard H Panosyan
Journal:  In Vivo       Date:  2022 Jul-Aug       Impact factor: 2.406

2.  Risk of Asymptomatic Bacteriuria among People with Sickle Cell Disease in Accra, Ghana.

Authors:  Eric S Donkor; Jonathan A Osei; Isaac Anim-Baidoo; Samuel Darkwah
Journal:  Diseases       Date:  2017-02-15

3.  Levels of Soluble Endothelium Adhesion Molecules and Complications among Sickle Cell Disease Patients in Ghana.

Authors:  Charles Antwi-Boasiako; Eric S Donkor; Fredericka Sey; Bartholomew Dzudzor; Gifty B Dankwah; Kate H Otu; Alfred Doku; Campbell A Dale; Ivy Ekem
Journal:  Diseases       Date:  2018-04-21

4.  Significantly elevated foetal haemoglobin levels in individuals with glucose 6-phosphate dehydrogenase disease and/or sickle cell trait: a cross-sectional study in Cape Coast, Ghana.

Authors:  Patrick Adu; Essel K M Bashirudeen; Florence Haruna; Edward Morkporkpor Adela; Richard K D Ephraim
Journal:  BMC Hematol       Date:  2017-09-25

5.  Renal abnormalities among children with sickle cell conditions in highly resource-limited setting in Ghana.

Authors:  Enoch Odame Anto; Christian Obirikorang; Emmanuel Acheampong; Eric Adua; Sampson Donkor; Bright Oppong Afranie; Matthew Ofori; Emmanuel Akomanin Asiamah; Evans Asamoah Adu
Journal:  PLoS One       Date:  2019-11-19       Impact factor: 3.240

  5 in total

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