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ADULT syndrome: dental features of a very rare condition.
J Biol Regul Homeost Agents. 2017 Apr-Jun; 31(2 Suppl 1):61-65.JB

Abstract

The Acro-Dermato-Ungual-Lacrimal-Tooth syndrome (ADULT syndrome) is one of the rarest ectodermal dysplasias and it is associated with several malformations involving especially the limbs. The most clinical features are the presence of ectrodactyly, syndactyly, hypermelanosis or multiple lentigines, onhycodysplasia, abnormalities in the lacrimal duct, recurrent conjuntivitis, photophobia, mammarian hypoplasia, hypotrichosis and frontal alopecia, hypohydrosis, cutaneous photosensitivity, nasal bridge prominence, exfoliative dermatitis and xerosis. The ectodermal dysfunction expresses itself with conoid teeth, enamel hypoplasia, dentinal dysplasia and especially hypodontia, with following functional and aesthetic defects. We report the case of an 11-year-old Caucasian girl affected by ADULT syndrome.

Authors+Show Affiliations

Department of Medicine and Surgery, Research Centre into Paediatric Dentistry and Rare Syndromes, University of Insubria, ASST dei Sette Laghi, Dental Clinic, Varese, Italy.Department of Medicine and Surgery, Research Centre into Paediatric Dentistry and Rare Syndromes, University of Insubria, ASST dei Sette Laghi, Dental Clinic, Varese, Italy.Oral Surgery, Università Vita-Salute San Raffaele, Milan Italy.Department of Medicine and Surgery, Research Centre into Paediatric Dentistry and Rare Syndromes, University of Insubria, ASST dei Sette Laghi, Dental Clinic, Varese, Italy.Department of Medicine and Surgery, Research Centre into Paediatric Dentistry and Rare Syndromes, University of Insubria, ASST dei Sette Laghi, Dental Clinic, Varese, Italy.Department of Medicine and Surgery, Research Centre into Paediatric Dentistry and Rare Syndromes, University of Insubria, ASST dei Sette Laghi, Dental Clinic, Varese, Italy.Stomatology and Oral Surgery, Dr Peset University Hospital, Department of Stomatology, University of Valencia, Spain.Department of Medicine and Surgery, Research Centre into Paediatric Dentistry and Rare Syndromes, University of Insubria, ASST dei Sette Laghi, Dental Clinic, Varese, Italy.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

28691455

Citation

Azzi, L, et al. "ADULT Syndrome: Dental Features of a Very Rare Condition." Journal of Biological Regulators and Homeostatic Agents, vol. 31, no. 2 Suppl 1, 2017, pp. 61-65.
Azzi L, Maurino V, Vinci R, et al. ADULT syndrome: dental features of a very rare condition. J Biol Regul Homeost Agents. 2017;31(2 Suppl 1):61-65.
Azzi, L., Maurino, V., Vinci, R., Croveri, F., Boggio, A., Tagliabue, A., Silvestre-Rangil, J., & Tettamanti, L. (2017). ADULT syndrome: dental features of a very rare condition. Journal of Biological Regulators and Homeostatic Agents, 31(2 Suppl 1), 61-65.
Azzi L, et al. ADULT Syndrome: Dental Features of a Very Rare Condition. J Biol Regul Homeost Agents. 2017 Apr-Jun;31(2 Suppl 1):61-65. PubMed PMID: 28691455.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - ADULT syndrome: dental features of a very rare condition. AU - Azzi,L, AU - Maurino,V, AU - Vinci,R, AU - Croveri,F, AU - Boggio,A, AU - Tagliabue,A, AU - Silvestre-Rangil,J, AU - Tettamanti,L, PY - 2017/7/11/entrez PY - 2017/7/12/pubmed PY - 2019/2/7/medline SP - 61 EP - 65 JF - Journal of biological regulators and homeostatic agents JO - J Biol Regul Homeost Agents VL - 31 IS - 2 Suppl 1 N2 - The Acro-Dermato-Ungual-Lacrimal-Tooth syndrome (ADULT syndrome) is one of the rarest ectodermal dysplasias and it is associated with several malformations involving especially the limbs. The most clinical features are the presence of ectrodactyly, syndactyly, hypermelanosis or multiple lentigines, onhycodysplasia, abnormalities in the lacrimal duct, recurrent conjuntivitis, photophobia, mammarian hypoplasia, hypotrichosis and frontal alopecia, hypohydrosis, cutaneous photosensitivity, nasal bridge prominence, exfoliative dermatitis and xerosis. The ectodermal dysfunction expresses itself with conoid teeth, enamel hypoplasia, dentinal dysplasia and especially hypodontia, with following functional and aesthetic defects. We report the case of an 11-year-old Caucasian girl affected by ADULT syndrome. SN - 0393-974X UR - https://wwww.unboundmedicine.com/medline/citation/28691455/ADULT_syndrome:_dental_features_of_a_very_rare_condition_ L2 - https://medlineplus.gov/rarediseases.html DB - PRIME DP - Unbound Medicine ER -