Prosthodontic Solution for Pediatric Patient with Ectodermal Dysplasia: A Review Article

Bibin Jacob Emmanuel, Jacob Raja, Prajaktha Ganeshrao Gaikwad, Apurva Goyal, Mukesh Kumar
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Abstract

Background : Ectodermal dysplasia is a category of hereditary disorders characterized by a primary deficiency in the function of the hair, teeth, nails, or sweat glands, as well as another abnormality in any ectodermal tissue. Ears, lips, mouth or nose mucous membranes, and the central nervous system are among them. In ectodermal dysplasia, more than 2 ectoderma structures are deformed and are a related genetic. Past lessons seen from hypohidrotic ectodermal dysplasia act as a model for disclosing the etiology and pathogenesis of other ectodermal dysplasia types by identifying phenotypically similar syndromes can be the result of alteration in various genes that alteration in identical genes could result in different phenotypes and that alteration in the genes downstream the identical signaling pathway which could alter the phenotype significantly. In a letter to Charles Darwin in 1838, Wedderburn described a case of ectodermal dysplasia in 10 Hindu male relatives. Thurnam registered two cases of hypohidrotic type in 1848. In most cases, dentists do not have the thorough knowledge to tackle such cases along with that the inability for the guardians and the child to support the long-term treatment needed for ectodermal dysplasia as the appliances has to keep on changing stands as a blockade in the pathway of a successful treatment. The present analysis focuses on epidemiology, etiology, clinical features, and diagnosis along with different treatment options, on classification, genetic aspects, including clinical manifestation. To restore the esthetic appeal and work of the stomatognathic system, patients also require a multidisciplinary approach for clinical preparation and dental treatment. Follow-ups of the patient with adjustments in their dentures should be made to meet the continuous development occurring in the patient, early dental treatments may enhance the patient’s appearance and psychological problems. Proper communication should be there between the patient and the dentist if the treatment has to be a success. When it comes to children, guardian should actively support the instruction provided by the dentist. Dentist should provide an environment for the child and his/her guardian to convey their problems and an active time allotment should be given to make the treatment successful.
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儿童外胚层发育不良患者的修复方案:综述文章
背景:外胚层发育不良是一类遗传性疾病,其特征是头发、牙齿、指甲或汗腺功能的原发性缺陷,以及外胚层组织的另一种异常。耳朵、嘴唇、嘴巴或鼻子的粘膜以及中枢神经系统都在其中。在外胚层发育不良中,有2个以上的外胚层结构变形,这与遗传有关。以往从少汗性外胚层发育不良中得到的经验教训为揭示其他外胚层发育不良类型的病因和发病机制提供了一个模型,通过发现表型相似的综合征可能是多种基因改变的结果,相同基因的改变可能导致不同的表型,而相同信号通路下游基因的改变可能显著改变表型。在1838年写给查尔斯·达尔文的一封信中,韦德伯恩描述了10名印度男性亲属的外胚层发育不良病例。Thurnam在1848年登记了两例低汗型病例。在大多数情况下,牙医没有足够的知识来处理这些情况,同时,由于器具必须不断更换,监护人和孩子无法支持外表皮发育不良所需的长期治疗,这是成功治疗的障碍。目前的分析侧重于流行病学,病因学,临床特征,诊断以及不同的治疗方案,在分类,遗传方面,包括临床表现。为了恢复口腔系统的美观和工作,患者还需要多学科的临床准备和牙科治疗方法。对患者进行随访,调整假牙,以满足患者持续发展的需要,早期的牙科治疗可能会增强患者的外观和心理问题。如果治疗成功,病人和牙医之间应该有适当的沟通。当涉及到儿童时,监护人应积极支持牙医的指导。牙医应提供一个环境,让儿童及其监护人表达他们的问题,并安排积极的时间,以确保治疗成功。
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