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Consultant and Honorary Senior Lecturer in Special Care Dentistry; Clinical Lead, Department of Sedation and Special Care Dentistry, Guy’s and St Thomas’ NHS Foundation Trust, London
The features of sickle cell disease (SCD) are described. Two case reports of patients treated in a Dental Institute are presented and the dental management of patients with SCD discussed. Since infection is one of the major risk factors for sickle cell crisis, the prevention of oral disease and infection is vital for this group of patients and there is no contra-indication to the delivery of dental treatment under local anaesthetic with inhalational sedation if required in the primary care setting. Since patients with sickle cell disease are particularly vulnerable to the effects of periods of hypoxia, which may produce significant morbidity, and because of the additional practical challenges in sedating this group of patients, intravenous sedation should be undertaken in a specialist unit.
Clinical Relevance: The increasing prevalence of sickle cell disease highlights the importance of dentists practising in multi-cultural communities having an understanding of this condition and its implications on their clinical practice. This will facilitate the safe management of patients with sickle cell disease.
Article
Sickle cell disease is a hereditary chronic anaemia, which results from the production of defective sickle haemoglobin, HbS, instead of normal haemoglobin, HbA. The gene for HbS production is autosomal recessive, thus an individual inheriting two HbS genes (a homozygote) is said to suffer from sickle cell disease, whilst a heterozygote with only one HbS gene has sickle cell trait. Sickle cell trait has such innocuous features that it does not require treatment and does not affect lifespan. These individuals are usually asymptomatic and rarely display the physical abnormalities or anaemia seen in those with sickle cell disease. In this paper, the term sickle cell disease will therefore be used to describe only the homozygous condition.1
Haemoglobin is a globular protein molecule, composed of two pairs of polypeptide chains, each intricately folded around a haem molecule. Haemoglobin A (HbA) consists of two α and two β chains. The β globin chain contains 146 amino acids and the two genes determining β chain structure are located on the short arm of chromosome 12. Genetic information is coded by the sequence of nucleotides; three bases (a codon) represent the basic unit of information and determine a single amino acid on the globin chains. Sickle haemoglobin (HbS) results from a single point mutation, where the normal codon, GAG, at position 6 on the β chain is replaced by GUG. This change results in the substitution of valine (in HbS) for the glutamic acid that occurs at this position in HbA. This single base substitution results in the tendency of deoxy HbS molecules to form polymers and hence the production of the symptoms of sickle cell disease.2
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