Repository logo
 
Publication

Steinert syndrome and repercussions in dental medicine

dc.contributor.authorBaptista, Helena
dc.contributor.authorCardoso, Inês Lopes
dc.date.accessioned2020-03-03T15:47:45Z
dc.date.available2020-03-03T15:47:45Z
dc.date.issued2016
dc.date.updated2020-02-28T16:23:59Z
dc.description.abstractSteinert syndrome, also called myotonic dystrophy type 1, is a genetic disorder with autosomal dominant transmission characterized by myotonia and a multisystemic clinical picture that affects severaltissues of the human body. The most common systemic phenotypes are: muscular, cardiac, respiratory, CNS, ocular, gynecological, digestive, orthopedical, as well as cognitive and psychological symptoms (cognitive decline). Muscles involved in voluntary movement are highly affected by myotonia especially distal muscles of upper limbs. These patients also show changes in face, chewing and pharynx muscles that can lead to swallowing and speech problems, dysphagia and in most cases to food aspiration and suffocation. Poor oral hygiene resulting from reduced motor mobility and reduced saliva flux can lead to gingival inflammation and periodontal disease. Other oral manifestations include disturbances at the temporomandibular articulation, dental occlusion changes and reduction in teeth number as a result of caries. Main causes of death are pneumonia and cardiac arrhythmias. The etiopathogeny of this syndrome is still not clear, conditioning the existence of a specific treatment for this disease. Nowadays, treatments consist on the release of the existing symptoms, in an attempt to give a better life quality to patients. It is very important to implement actions that can prevent complications and consequently decrease death. Treatments should be applied in an early stage of the disease. Bronchoscopy and artificial respiration should be used to prevent pneumonia, and regular electrocardiographic monitoring should be done to evaluate defects in the conductive system. Several approaches have been applied to rehabilitate swallowing dysfunction and avoid aspiration like videofluoroscopy, postural techniques and adjustment of diet type. It is the aim of this paper to clarify the ethiology, diagnosis, systemic and oral characteristics of the syndrome, as well as to discuss treatments to be applied according to patients affected organs.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.doi10.1016/j.archoralbio.2016.12.008pt_PT
dc.identifier.issn0003-9969
dc.identifier.pmid28040606
dc.identifier.slugcv-prod-368714
dc.identifier.urihttp://hdl.handle.net/10284/8601
dc.language.isoengpt_PT
dc.publisherElsevierpt_PT
dc.subjectNeuromuscular disorderpt_PT
dc.subjectMyotonic dystrophypt_PT
dc.subjectSteinert diseasept_PT
dc.subjectManagement of myotonic dystrophypt_PT
dc.subjectHealth considerationspt_PT
dc.titleSteinert syndrome and repercussions in dental medicinept_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage47pt_PT
oaire.citation.startPage37pt_PT
oaire.citation.titleArchives of Oral Biologypt_PT
oaire.citation.volume75pt_PT
person.familyNameLopes Cardoso
person.givenNameInês
person.identifier.ciencia-idF21F-16B4-3715
person.identifier.orcid0000-0002-0693-9831
person.identifier.ridM-7156-2013
person.identifier.scopus-author-id54973754300
rcaap.cv.cienciaidF21F-16B4-3715 | Maria Inês de Avelar Lopes Cardoso
rcaap.rightsclosedAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublicationf39fa18f-2d23-4690-bfa2-f8ba53e40775
relation.isAuthorOfPublication.latestForDiscoveryf39fa18f-2d23-4690-bfa2-f8ba53e40775

Files

Original bundle
Now showing 1 - 1 of 1
No Thumbnail Available
Name:
Steinert_syndrome.pdf
Size:
566.03 KB
Format:
Adobe Portable Document Format
License bundle
Now showing 1 - 1 of 1
No Thumbnail Available
Name:
license.txt
Size:
1.64 KB
Format:
Item-specific license agreed upon to submission
Description: