Morphological appearance and chemical composition of enamel in primary teeth from patients with 22q11 deletion syndrome

Abstract

Patients with 22q11 deletion syndrome have many and complex medical problems, including hypocalcemia and/or hypoparathyroidism. Odontological findings include enamel aberrations in both dentitions. In order to describe enamel morphology, chemical composition in primary teeth, and to investigate the relationship between medical history and morphological appearance, dental enamel was investigated in 38 exfoliated primary teeth from 15 children and adolescents. Morphology was studied by the use of a polarized light microscope, microradiography, scanning electron microscopy, X-ray microanalysis, and secondary ion mass spectrometry. The morphological findings were compared with medical history. The teeth showed, in principle, a normal morphological appearance with regard to prism structure. A high frequency of aberrations, such as hypomineralization, hypoplasia and extra incremental lines, were found. The majority of the aberrations were found around the neonatal line. There was a relationship between high numbers of medical problems in the patients and enamel deviations. The result supports the hypothesis of under-reporting of both hypocalcemia and hypoparathyroidism in patients with 22q11 deletion syndrome.

Dental Findings in Patients With Non-surgical Hypoparathyroidism and Pseudohypoparathyroidism: A Systematic Review

“…Table 2 illustrates study characteristics for Ns-HypoPT studies (Humphreys, 1939; Hansted and Holst, 1952; Hinrichs, 1956; Thew and Goulston, 1962; Pisanty, 1966; Sjöberg, 1966; Greenberg et al, 1969; Hermans et al, 1969; Moshkowitz et al, 1969; Riley, 1969; Nally, 1970; Frensilli et al, 1971; Lovestedt, 1971; Assif, 1977; Pisanty and Garfunkel, 1977; Myllärniemi and Perheentupa, 1978; Lindeberg, 1979; Goepferd and Flaitz, 1981; Illum et al, 1981; Jensen et al, 1981; Nikiforuk and Fraser, 1981; Børglum Jensen et al, 1983; Harrell, 1983; Ingemarsson, 1984; Lyles et al, 1985; de Carvalho et al, 1986; Porter and Scully, 1986; Ahonen et al, 1990; Porter et al, 1992, 1995; Walls and Soames, 1993; Hershkovitz et al, 1995; Lukinmaa et al, 1996; Firth et al, 1997; Jaquez et al, 1997; Perniola et al, 1998; Fukui et al, 2000; Winer and Merke, 2000; Klingberg et al, 2002, 2005, 2007; Al-Malik, 2004; López-Jornet et al, 2005; Oberoi and Vargervik, 2005; Yang et al, 2005; da Silva Dalben et al, 2008; McGovern et al, 2008; Kelly et al, 2009; Pavlic and Waltimo-Siren, 2009; Toka et al, 2010; Wasersprung et al, 2010; Heliövaara et al, 2011; Kollios et al, 2011; Oberoi et al, 2011; Nordgarden et al, 2012; Ponranjini et al, 2012; El Batawi, 2013; Kamarthi et al, 2013; Nortjé, 2013; Ali et al, 2014; Srirangarajan et al, 2014; Laccetta et al, 2015; Matthews-Brzozowska et al, 2015; Bruserud et al, 2016; Ferre et al, 2016; Bjanid et al, 2017; Lewyllie et al, 2017; Mohsenipour et al, 2017) with subgroups A–E corresponding to the classifi...…”

“…Studies have shown that enamel opacities and enamel hypoplasia develop in children who suffer from hypocalcemia during the period of enamel formation (secretion, mineralization, and maturation; Stimmler et al, 1973; Nikiforuk and Fraser, 1981; Fraser and Nikiforuk, 1982; Klingberg et al, 2005; Kelly et al, 2009). However, enamel opacities and hypoplasia are reported more often in the permanent than in the primary dentition (Nordgarden et al, 2012).…”

“…Most studies included both clinical and radiographic dental findings, which could be considered a strength. However, studies used different methods for the description of the patients, e.g., one study concerning IHP and one concerning PHP described dental findings based on only few X-rays (Lovestedt, 1971; Witkop, 1976), and a study on 22q11DS described dental findings based only on 38 exfoliated teeth from 15 different patients (Klingberg et al, 2005). Consequently, these studies did not include findings of teeth other than those that had been exfoliated or examined by radiographic examination.…”

“…The full electronic search strategy for PubMed is shown in Figure 1. In addition to the original search, a hand-search of the reference lists of the publications entered into the review was performed, with special focus on the known Scandinavian authors, e.g., Børglum, Klingberg, and Nordgarden (Børglum Jensen et al, 1983; Klingberg et al, 2005; Nordgarden et al, 2012). Lastly, an additional search on Scandinavian languages (Danish, Swedish, and Norwegian) was performed.…”

Oral health and 22q11 deletion syndrome: thoughts and experiences from the parents’ perspectives

“…Previous clinical and histological studies have reported that high frequency of dental aberrations, foremost enamel hypoplasia and hypomineralisation, as well as high caries prevalence in patients with 22q11DS 11,12 . Apart from dietary and oral hygiene problems, impaired saliva secretion and altered composition regarding electrolytes and proteins are plausible explanations for the cariologic problems seen in many patients with 22q11DS 13 .…”