title: Hypohidrotic ectodermal dysplasia: a multidisciplinary approach.
abstract: Hypohidrotic ectodermal dysplasia (HED; Christ-Siemens-Touraine syndrome) is a genetic disorder characterized by sparse hair, oligodontia with peg-shaped teeth, reduced sweating, and defects in a number of other ectodermal organs. A partial or complete absence of eccrine glands can lead to recurrent severe overheating that may cause seizures and neurological deficits. This clinical report presents a 14-year-old male patient with hypohidrotic ectodermal dysplasia, including the clinical and radiographic findings, and multidisciplinary treatment. The Schedule for Affective Disorders and Schizophrenia for School Aged Children-Present and Lifetime Version (K-SADS-PL) was administered to patient along with mother to assess for any psychiatric disorders. The screening and rating scales completed by mother and two teachers to evaluate the severity of attention deficit hyperactivity disorder (ADHD) symptoms, and other behavioral problems. Patient's academic performance, adaptive functioning, and problem behavior was evaluated using. The Teacher Report Form. Mental capacity was assessed with the Wechsler Intelligence Scale for Children (WISC-R). Illness Perception Questionnaire Revised was used to systematically assess illness representation attributes and emotional representations of illness. On the psychiatric diagnosis assessment using K-SADS-PL sub-threshold attention deficits and anxiety symptoms were determined. In this case we established a multidisciplinary approach in his treatment with pediatric, dermatological, and dental examinations, beside his psychiatric evaluation. The prosthetic rehabilitation included restoring upper teeth with copings and fabrication of upper and lower complete dentures. Metal framework was not incorporated in the partial denture design allowing modifications as the oral and maxillofacial development continued. Removable complete or partial dentures without metal framework is a treatment of choice until the completion of facial growth at which definitive treatment is considered.
authors.affiliation: Ege University, Turkey. tezanbildik@yahoo.com
authors.forename: Tezan
authors.initials: T
authors.lastname: Bildik
authors.forename: Burcu
authors.initials: B
authors.lastname: Ozbaran
authors.forename: Sezen
authors.initials: S
authors.lastname: Kose
authors.forename: Guldane
authors.initials: G
authors.lastname: Koturoglu
authors.forename: Bulent
authors.initials: B
authors.lastname: Gokce
authors.forename: Asli
authors.initials: A
authors.lastname: Gunaydin
authors.forename: Inci
authors.initials: I
authors.lastname: Altintas
mesh_terms: D000293:Adolescent; D001289:Attention Deficit Disorder with Hyperactivity / Q000175:diagnosis; D003824:Denture, Complete / Q000706:statistics & numerical data; D053358:Ectodermal Dysplasia 1, Anhidrotic / Q000503:physiopathology / Q000523:psychology / Q000628:therapy; D006801:Humans; D008297:Male; D009483:Neuropsychological Tests; D011569:Psychiatric Status Rating Scales; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014888:Wechsler Scales