Abstract

Pentasomy of the x chromosome is a rare syndrome of unknown incidence and there are few reports in the scientific literature, especially in terms of dental care for patients with this syndrome. The aim of this study was to report the dental care given to an adolescent with pentasomy of the X chromosome. The patient presented a cleft palate, impacted teeth, malocclusion, high caries activity, gingivitis and multiple tooth loss due to caries. Despite the difficulties of carrying out the dental work due to involuntary movements and the initial lack of collaboration from the patient the overall dental care was completed on an outpatient basis. The therapeutic success of the treatment was strongly supported by the significant cooperation and relationship that grew up along the period of the treatment, as well as the desire to have the treatment done by both the patient and her parents.

Highlights

  • Pentasomy X is a rare disorder of the sex chromosomes, which only affects females and is characterized by the presence of five chromosomes “x” (49,xxxxx) instead of two (46,xx)

  • This paper aims to report a case of dental care given to an adolescent with pentasomy X

  • The initial phase of the treatment was to stabilize the oral environment and control tooth decay using: Pentasomy X is a rare syndrome of unknown incidence

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Summary

Introduction

Pentasomy X is a rare disorder of the sex chromosomes, which only affects females and is characterized by the presence of five chromosomes “x” (49,xxxxx) instead of two (46,xx). The most common features of this syndrome are delayed psychomotor development with kyphosis, muscular hypotonia, ocular hypertelorism, slanted eyes (epicanthal folds), malformed toes, short neck, thick and flat nose, tooth abnormalities, overlapping toes and clinodactyly. Other signs such as hydrocephalus, microcephaly and/or brachycephaly, high palate, single transverse palmar crease, malformed or low-set, low hairline at the nape and/or pelvis may be present. 507-511, out./dez., 2015 patient and family in terms of the patient’s oral health can cause an accumulation of dental needs[4,5] A lack of understanding between a professional and a RGO, Rev Gaúch Odontol, Porto Alegre, v.63, n.4, p. 507-511, out./dez., 2015 patient and family in terms of the patient’s oral health can cause an accumulation of dental needs[4,5]

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