Enamel defects and tooth eruption disturbances in children with sickle cell anemia.

Caroline Maria Igrejas Lopes,Ana Cláudia Alves E Luna,Maria José Rodrigues,Valdenice Aparecida De Menezes,Marília Cabral Cavalcanti,Kátia Maria Gonçalves Marques

doi:10.1590/1807-3107bor-2018.vol32.0087

Abstract

Sickle cell anemia, a genetic disease caused by a mutation in the beta-globin gene, can present oral manifestations such as delayed tooth eruption and hypomineralized enamel and dentin. The aim of the present study was to evaluate the prevalence and severity of developmental defects of enamel (DDE) and delayed tooth eruption in children with sickle cell anemia. The sample comprised 56 male and female children with sickle cell anemia aged 6 to 12 years and treated at the Hematology and Hemotherapy Center of Pernambuco, Brazil. The data were collected according to the WHO criteria for DDE and tooth eruption. The prevalence of DDE was 58.2% and increased with age, affecting 43.8% of children aged 6 to 8 years and 66.7% of those aged 10 to 12 years (p>0.05; Pearson's chi-square test). There was no significant association between DDE and sex; the most prevalent type of DDE was diffuse opacity (6.2%). Tooth eruption was delayed in 18 children (32.1%). The delay increased with age and was detected in 11.8% of children aged 6 to 8 years, in 20.0% of those aged 8 to 10 years and in 54.2% of those aged 10 to 12 years (p<0.05; Pearson's chi-square test). Delayed tooth eruption was higher in males (36.7%, p>0.05). The prevalence of DDE was high, increased with age and was similar between sexes, while delayed eruption was higher in males and showed a significant association with age.

Highlights

Hemoglobinopathies used to be highly region-specific with a predilection for African, Mediterranean, Middle Eastern, Southeast Asian and Indian subcontinental groups
Sickle cell anemia is a genetic disease caused by a mutation in the beta-globin gene, originating hemoglobin S (HbS), which can polymerize and promote red blood cell sickling when in homozygous form (HbSS).[4]
The present research aims to identify the prevalence and severity of developmental defects of enamel (DDE) and to evaluate delayed tooth eruption in children with sickle cell anemia. This was a cross-sectional study with a census sample of children with sickle cell anemia who sought treatment between September and December 2012 at the Hematology and Hemotherapy Center of Pernambuco (Hemope), a reference center for the treatment of patients with hemoglobin diseases in Recife, in the state of Pernambuco, Brazil, and who met the inclusion criteria

Summary

Introduction

Hemoglobinopathies used to be highly region-specific with a predilection for African, Mediterranean, Middle Eastern, Southeast Asian and Indian subcontinental groups. Because of widespread global migration, they affect at least 5.2% of the global population. There are over 332,000 affected conceptions or births, of which 275,000 are affected by sickle cell disorder requiring early diagnosis and prophylaxis. Most affected children born in high-income countries survive with a chronic disorder, while most of those born in low-income countries die before the age of 5 years, and hemoglobin disorders contribute the equivalent of 3.4% of mortality in children aged under 5 years worldwide or 6.4% in Africa.[1] The major forms of hemoglobinopathies are often severe and their management is difficult and associated with a great psychosocial

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