Abstract

The aim of this study was to investigate possible associations between sickle cell anemia (SCA) and the severity of dental malocclusion (MO). This was a retrospective cohort study of 93 individuals with SCA (G1) and 186 individuals without the disease (G2). SCA patients were randomly selected by a simple draw from patients treated in the Centro de Hematologia e Hemoterapia do Maranhão (HEMOMAR) in northeastern Brazil. Patients aged between 16 and 60 were included after being tested for the hemoglobin S gene. G2 consisted of individuals living in the same residence as the patients. The Dental Aesthetic Index (DAI), as well as some morphological deviations not included in DAI, were used for the orthodontic evaluation of MO. Poisson regression with robust variance adjustment was employed to estimate relative risk (RR). In the multivariate analysis, SCA was associated with moderate (RR = 1.36) and very severe MO (RR = 8.0). SCA is correlated with anterior tooth loss (RR = 1.94), anterior spacing (RR = 1.66), overjet (RR = 1.87), anterior crossbite (RR = 1.94), and open bite (RR = 1.94). Thus, SCA is a risk factor for moderate and very severe MO.

Highlights

  • Sickle cell anemia (SCA) is an autosomal recessive nonsex-linked hemoglobinopathy predominant among Afro-descendants

  • Because red blood cells need to be constantly produced towing to their short lifespan, SCA patients commonly suffer from hyperplasia and compensatory expansion of the bone marrow, which may result in bone changes even of the craniofacial bone structures

  • The study was conducted in the Centro de Hematologia e Hemoterapia do Maranhão (HEMOMAR), a state healthcare institution responsible for government policies related to hematology and transfusion medicine in the State of Maranhão, Brazil

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Summary

Introduction

Sickle cell anemia (SCA) is an autosomal recessive nonsex-linked hemoglobinopathy predominant among Afro-descendants. Its cause is a point mutation in the β-chain of hemoglobin which results in an abnormal hemoglobin variant called hemoglobin S (HbS).[1] SCA has been categorized as a public health issue as it affects a significant percentage of the world’s population. It is considered the most prevalent inherited disease in Brazil; the state of Bahia has the highest incidence of SCA at 15.4 cases per 10,000 live births and Maranhão is in fourth position with 7.1 cases per 10,000 live births.[2]. A significantly more pronounced vertical pattern (clockwise) is observed as inferior and total face height angles and posterior rotation of the mandible are increased in SCA.[4]

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