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Case Reports
. 2018 Dec 1;10(12):e1238-e1241.
doi: 10.4317/jced.55350. eCollection 2018 Dec.

Aplasia of the lacrimal and major salivary glands (ALSG). First case report in spanish population and review of the literature

David Neagu  1 , Beatriz Patiño-Seijas  1 , Ramón Luaces-Rey  1 , Javier Collado-López  2 , Álvaro García-Rozado-González  3 , José-Luis López-Cedrún-Cembranos  4
Affiliations
Case Reports

Aplasia of the lacrimal and major salivary glands (ALSG). First case report in spanish population and review of the literature

David Neagu et al. J Clin Exp Dent. .

Abstract

Aplasia of the lacrimal and the major salivary glands (ALSG) is a rare disorder with scarce cases described in the recent literature. The pattern of genetic inheritance is autosomal dominant with variable expressivity. A 40 years male patient was referred to the Oral and Maxillofacial Service at the Hospital Universitario de A Coruña diagnosed with complete agenesis of all salivary glands. Our case it is the first of ALSG syndrome in the Spanish literature. Imaging tests are necessary to confirm the lack of formation of salivary glands and alteration of lacrimal system. A mutation of FGF10 has been proposed as the responsible of the syndrome. The management of the lacrimal alteration depends of the clinical findings. Clinical suspicion remains the principal tool to diagnose the syndrome. Key words:ALSG, salivary glands aplasia.

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Conflict of interest statement

Conflict of interest statement: The authors have declared that no conflict of interest exist.

Figures

Figure 1
Figure 1
MRI revealing agenesis of all major salivary glands.
Figure 2
Figure 2
Multiple caries and dry mouth in physical exploration.
Figure 3
Figure 3
OPG revealing affectation of almost all dental pieces.
Figure 4
Figure 4
No tubes or lacrimal puncta are visualized in the ophthalmologic examination.
See this image and copyright information in PMC

References

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