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[The aglossia-adactylia syndrome].
Helv Paediatr Acta. 1976 Jun; 31(1):77-84.HP

Abstract

We present two own cases of the aglossy-adactyly syndrome and a review of 25 cases of the literature. Our experience concernes two girls. One was followed from birth until 4 1/2 years, the other from 5 to 9 years. This helped us to obtain a good knowledge of the development of these patients. Malformations of the mouth and of the limbs with a normal intelligence characterize the aglossy-adactyly syndrome. The most important features are: shortness of the tongue of variable degree, anomaly of the teeth, hypoplasia of the mandible and ectromelia of different extension with a peripheric predilection and often with an asymmetry. The etiology is unknown. All the known cases are sporadic. The differential diagnosis of the aglossy-adactyly syndrome includes the ankyloglossia superior, the Hanhart syndrome and the oro-farcio-digital syndrome I. Even when the mouth malformations are of a great degree, the functional limitations of the patients with aglossy-adactyly syndrome are very small. This is important in determining the prognosis, in consulting the patients and their parents and for decisions about corrective operations.

Authors

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Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

ger

PubMed ID

939703

Citation

Lecannellier, J, and D Vischer. "[The Aglossia-adactylia Syndrome]." Helvetica Paediatrica Acta, vol. 31, no. 1, 1976, pp. 77-84.
Lecannellier J, Vischer D. [The aglossia-adactylia syndrome]. Helv Paediatr Acta. 1976;31(1):77-84.
Lecannellier, J., & Vischer, D. (1976). [The aglossia-adactylia syndrome]. Helvetica Paediatrica Acta, 31(1), 77-84.
Lecannellier J, Vischer D. [The Aglossia-adactylia Syndrome]. Helv Paediatr Acta. 1976;31(1):77-84. PubMed PMID: 939703.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [The aglossia-adactylia syndrome]. AU - Lecannellier,J, AU - Vischer,D, PY - 1976/6/1/pubmed PY - 1976/6/1/medline PY - 1976/6/1/entrez SP - 77 EP - 84 JF - Helvetica paediatrica acta JO - Helv Paediatr Acta VL - 31 IS - 1 N2 - We present two own cases of the aglossy-adactyly syndrome and a review of 25 cases of the literature. Our experience concernes two girls. One was followed from birth until 4 1/2 years, the other from 5 to 9 years. This helped us to obtain a good knowledge of the development of these patients. Malformations of the mouth and of the limbs with a normal intelligence characterize the aglossy-adactyly syndrome. The most important features are: shortness of the tongue of variable degree, anomaly of the teeth, hypoplasia of the mandible and ectromelia of different extension with a peripheric predilection and often with an asymmetry. The etiology is unknown. All the known cases are sporadic. The differential diagnosis of the aglossy-adactyly syndrome includes the ankyloglossia superior, the Hanhart syndrome and the oro-farcio-digital syndrome I. Even when the mouth malformations are of a great degree, the functional limitations of the patients with aglossy-adactyly syndrome are very small. This is important in determining the prognosis, in consulting the patients and their parents and for decisions about corrective operations. SN - 0018-022X UR - https://www.unboundmedicine.com/medline/citation/939703/[The_aglossia_adactylia_syndrome]_ DB - PRIME DP - Unbound Medicine ER -