Document Detail


Case report: Presentation of lacrimo-auriculodento- digital (LADD) syndrome in a young female patient.
MedLine Citation:
PMID:  19863897     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Lacrimo-auriculo-dento-digital (LADD) syndrome (OMIM #149730) is an autosomal-dominant congenital disorder that can be caused by heterozygous mutations in the tyrosine kinase domains of the genes encoding fibroblast growth factor receptors 2 (FGFR2) and 3 (FGFR3), and has been found in association with a mutation in the FGF10 gene, which encodes an Fgfr ligand. Clinical signs vary, but the condition is characterised by involvement of the lacrimal and salivary systems, cup-shaped ears, hearing loss and dental abnormalities. Additional features may include involvement of the hands and feet with other body systems particularly the kidneys. CASE REPORT: Previous literature on the subject has been reviewed and this case is the first presentation of LADD syndrome in the Republic of Ireland, as a sporadic case in a 12-year-old girl who exhibited a range of dental and digital anomalies. TREATMENT: Her general medical practitioner managed her medical care whilst her oral care necessitated a multidisciplinary approach involving restorative and orthodontic elements. FOLLOW-UP: The initial restorative phase of treatment has successfully improved the appearance of the patient's anterior teeth using direct resin composite build-ups.
Authors:
G J McKenna; F M Burke; K Mellan
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  European archives of paediatric dentistry : official journal of the European Academy of Paediatric Dentistry     Volume:  10 Suppl 1     ISSN:  1818-6300     ISO Abbreviation:  Eur Arch Paediatr Dent     Publication Date:  2009 Nov 
Date Detail:
Created Date:  2009-10-29     Completed Date:  2010-03-04     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101277157     Medline TA:  Eur Arch Paediatr Dent     Country:  England    
Other Details:
Languages:  eng     Pagination:  35-9     Citation Subset:  D; IM    
Affiliation:
Dept. Restorative Dentistry, School of Dentistry, University College Cork, Cork, Ireland. g.mckenna@ucc.ie
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / pathology
Child
Composite Resins
Cuspid / abnormalities
Dental Materials
Dental Restoration, Permanent
Ear, External / abnormalities*
Female
Fingers / abnormalities*
Follow-Up Studies
Humans
Incisor / abnormalities
Lacrimal Apparatus / abnormalities*
Syndrome
Tooth Abnormalities / pathology*
Chemical
Reg. No./Substance:
0/Composite Resins; 0/Dental Materials

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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