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Association of sickle cell haemoglobinopathies with dental and jaw bone abnormalities

Association of sickle cell haemoglobinopathies with dental and jaw bone abnormalities INTRODUCTIONSickle cell anaemia (SCA) is a genetic condition characterized by vaso‐occlusive episodes and infectious events (Kato, Hebbel, Steinberg, & Gladwin, ). This haemoglobinopathy is the result of a molecular modification causing the substitution of glutamic acid for valine in the beta chain of haemoglobin, thus leading to the production of modified haemoglobin (Hb S) (Kato et al., ; Steinberg, ). In homozygotes, the presence of Hb S instead of the normal haemoglobin (Hb A) is genetically inherited from both parents and characterizes SCA (Steinberg, ). Another condition considered asymptomatic and associated with the presence of Hb S is sickle cell trait (SCT); this condition is caused by heterozygous inheritance in which an individual receives the recessive gene for Hb S from one parent and a dominant gene for Hb A from the other (Goldsmith et al., ; Steinberg, ; Tsaras et al., ).In hypoxic conditions, biochemical changes in the Hb molecule trigger the formation of polymers that lead to a change in the biconcave discoid configuration of red blood cells, thus causing a shape similar to a sickle (Ferreira et al., ; Kato et al., ). Consequently, sickled cells become less flexible and more adherent to the vascular endothelium, blocking capillaries and restricting blood http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Oral Diseases Wiley

Association of sickle cell haemoglobinopathies with dental and jaw bone abnormalities

Souza, SFC; Carvalho, HLCC; Costa, CPS; Thomaz, EBAF
Oral Diseases , Volume 24 (3) – Jan 1, 2018
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References (50)

Publisher
Wiley
Copyright
Copyright © 2018 John Wiley & Sons A/S. Published by John Wiley &Sons Ltd
ISSN
1354-523X
eISSN
1601-0825
DOI
10.1111/odi.12742
pmid
28833998
Publisher site
See Article on Publisher Site

Abstract

INTRODUCTIONSickle cell anaemia (SCA) is a genetic condition characterized by vaso‐occlusive episodes and infectious events (Kato, Hebbel, Steinberg, & Gladwin, ). This haemoglobinopathy is the result of a molecular modification causing the substitution of glutamic acid for valine in the beta chain of haemoglobin, thus leading to the production of modified haemoglobin (Hb S) (Kato et al., ; Steinberg, ). In homozygotes, the presence of Hb S instead of the normal haemoglobin (Hb A) is genetically inherited from both parents and characterizes SCA (Steinberg, ). Another condition considered asymptomatic and associated with the presence of Hb S is sickle cell trait (SCT); this condition is caused by heterozygous inheritance in which an individual receives the recessive gene for Hb S from one parent and a dominant gene for Hb A from the other (Goldsmith et al., ; Steinberg, ; Tsaras et al., ).In hypoxic conditions, biochemical changes in the Hb molecule trigger the formation of polymers that lead to a change in the biconcave discoid configuration of red blood cells, thus causing a shape similar to a sickle (Ferreira et al., ; Kato et al., ). Consequently, sickled cells become less flexible and more adherent to the vascular endothelium, blocking capillaries and restricting blood

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Oral Diseases – Wiley

Published: Jan 1, 2018

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