Prosthetic rehabilitation of an adolescent with hypohidrotic ectodermal dysplasia with partial anodontia: case report. 2008

S Kaul, and R Reddy
MDS (Prosthodontics), ITS-CDSR, Ghaziabad, Uttar Pradesh, India. sriluy@gmail.com

Ectodermal dysplasia is a hereditary syndrome characterized by dysplasia of tissues of ectodermal origin (hair, skin, nails, and teeth) and occasionally, dysplasia of mesodermally derived tissues. The triad of nail dystrophy (onychodysplasia), alopecia, or hypotrichosis (scanty, fine, light hair on the scalp and eyebrows) and palmoplantar hypohidrosis is usually accompanied by lack of sweat glands and partial or complete absence of primary and permanent dentition. Hypohidrotic ectodermal dysplasia usually has an X-linked inheritance and affects only males severely, while female heterozygotes show only minor defects. The clinical management of children with ectodermal dysplasia provides a unique opportunity for cooperative effort between the pedodontist and the prosthodontist. The following case report discusses the management of a young boy with hypohidrotic ectodermal dysplasia. Removable prostheses were employed in the treatment. The aim was to rehabilitate the adolescent prosthodontically and boost him psychologically.

UI MeSH Term Description Entries
D008297 Male Males
D010347 Patient Care Planning Usually a written medical and nursing care program designed for a particular patient. Nursing Care Plans,Goals of Care,Plans, Nursing Care,Care Goal,Care Goals,Care Plan, Nursing,Care Planning, Patient,Care Plans, Nursing,Nursing Care Plan,Plan, Nursing Care,Planning, Patient Care
D003779 Denture Design The plan, delineation, and location of actual structural elements of dentures. The design can relate to retainers, stress-breakers, occlusal rests, flanges, framework, lingual or palatal bars, reciprocal arms, etc. Denture Designs,Design, Denture,Designs, Denture
D003826 Denture, Complete, Lower A complete denture replacing all the natural mandibular teeth and associated structures. It is completely supported by the oral tissue and underlying mandibular bone.
D003832 Denture, Partial, Removable A partial denture designed and constructed to be removed readily from the mouth. Removable Partial Denture,Denture, Removable Partial,Dentures, Removable Partial,Partial Denture, Removable,Partial Dentures, Removable,Removable Partial Dentures
D004476 Ectodermal Dysplasia A group of hereditary disorders involving tissues and structures derived from the embryonic ectoderm. They are characterized by the presence of abnormalities at birth and involvement of both the epidermis and skin appendages. They are generally nonprogressive and diffuse. Various forms exist, including anhidrotic and hidrotic dysplasias, FOCAL DERMAL HYPOPLASIA, and aplasia cutis congenita. Anhidrotic Ectodermal Dysplasia,Aplasia Cutis Congenita,Clouston's Syndrome,Ectodermal Defect, Congenital,Hidrotic Ectodermal Dysplasia,Anhydrotic Ectodermal Dysplasia,Aplasia Cutis Congenita, Nonsyndromic,Autosomal Dominant Hidrotic Ectodermal Dysplasia,Clouston Hidrotic Ectodermal Dysplasia,Clouston Syndrome,Clouston's Hidrotic Ectodermal Dysplasia,Congenital Ectodermal Defect,Defect, Congenital Ectodermal,Ectodermal Dysplasia 2, Hidrotic,Ectodermal Dysplasia Anhidrotic,Ectodermal Dysplasia, Anhydrotic,Ectodermal Dysplasia, Hidrotic,Ectodermal Dysplasia, Hidrotic, Autosomal Dominant,Hidrotic Ectodermal Dysplasia, Autosomal Dominant,Hydrotic Ectodermal Dysplasia,Anhidrotic Ectodermal Dysplasias,Anhidrotic, Ectodermal Dysplasia,Anhidrotics, Ectodermal Dysplasia,Anhydrotic Ectodermal Dysplasias,Cloustons Syndrome,Congenital Ectodermal Defects,Defects, Congenital Ectodermal,Dysplasia Anhidrotic, Ectodermal,Dysplasia Anhidrotics, Ectodermal,Dysplasia, Anhidrotic Ectodermal,Dysplasia, Anhydrotic Ectodermal,Dysplasia, Ectodermal,Dysplasia, Hidrotic Ectodermal,Dysplasia, Hydrotic Ectodermal,Dysplasias, Anhidrotic Ectodermal,Dysplasias, Anhydrotic Ectodermal,Dysplasias, Ectodermal,Dysplasias, Hidrotic Ectodermal,Dysplasias, Hydrotic Ectodermal,Ectodermal Defects, Congenital,Ectodermal Dysplasia, Anhidrotic,Ectodermal Dysplasia, Hydrotic,Ectodermal Dysplasias,Ectodermal Dysplasias, Anhidrotic,Ectodermal Dysplasias, Anhydrotic,Ectodermal Dysplasias, Hydrotic,Hidrotic Ectodermal Dysplasias,Hydrotic Ectodermal Dysplasias,Syndrome, Clouston,Syndrome, Clouston's
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000848 Anodontia Congenital absence of the teeth. It may involve all (total anodontia) or only some of the teeth (partial anodontia, hypodontia), or six or more of the teeth (oligodontia) and both the deciduous and the permanent dentition, or only teeth of the permanent dentition. Dental Agenesis, Familial,Familial Dental Agenesis,Familial Tooth Agenesis,Hypodontia,Oligodontia,Partial Anodontia,Total Anodontia,Hypodontia Oligodontia 1,Tooth Agenesis, Familial,Tooth Agenesis, Selective, 1,Agenesis, Familial Dental,Agenesis, Familial Tooth,Anodontia, Partial,Anodontia, Total,Familial Dental Ageneses,Familial Tooth Ageneses,Oligodontia 1, Hypodontia,Oligodontias,Partial Anodontias,Total Anodontias
D012649 Self Concept Persons' view of themselves. Self Esteem,Self Perception,Self Confidence,Self-Perception,Concept, Self,Confidence, Self,Esteem, Self,Perception, Self,Perceptions, Self,Self Esteems,Self Perceptions,Self-Perceptions

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