A Rare Case of Hutchinson-Gilford Progeria Syndrome with Early Dental Loss without Decay

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dc.contributor.authorPekdemir, Tugce N.
dc.contributor.authorOzturk, Duygu
dc.contributor.authorCetindag, Merve T.
dc.contributor.authorAkleyn, Ebru
dc.contributor.authorSariyildiz, Cansu O.
dc.contributor.authorCallea, Michele
dc.contributor.authorYavuz, Izzet
dc.date.accessioned2024-04-24T17:28:21Z
dc.date.available2024-04-24T17:28:21Z
dc.date.issued2018
dc.departmentDicle Üniversitesien_US
dc.description.abstractHutchinson-Gilford progeria syndrome (HGPS) is a rare autosomal recessive genetic disorder that occurs as a point mutation in the LMNA gene. It is a rare hereditary disorder, with approximately 100 cases reported in the medical literature. These patients and our case show features of aged appearance (pseudosenilism), loss of subcutaneous fat texture, growth retardation, sclerodermatous skin, 'horse riding posture', bird-face appearance, beaked nose, high pitched voice, protruding knees and elbows, underweight, short stature, malformation of the teeth, micrognathia, hypodontia, malocclusion, craniofacial disproportion, atherosclerosis and cardiovascular disorders. Unlike typical findings of HGPS, diffuse alopecia and prominent scalp veins were not observed in our case. Patients with HGPS have an average life span of 13 years, owing to myocardial infarction and congestive heart failure and our case also has atherosclerosis and heart failure. The study reported extra-and intraoral findings in a 24-year-old male patient with HGPS who came to our faculty with complaints about absence of teeth and psychological problems caused by absence of teeth and HGPS findings such as pseudosenilism, growth retardation and short stature. The data described necessary dental examinations and treatments for our patient and have reviewed the literature.en_US
dc.identifier.doi10.7454/msk.v22i2.9486
dc.identifier.endpage68en_US
dc.identifier.issn2356-3664
dc.identifier.issn2356-3656
dc.identifier.issue2en_US
dc.identifier.startpage63en_US
dc.identifier.urihttps://doi.org/10.7454/msk.v22i2.9486
dc.identifier.urihttps://hdl.handle.net/11468/20412
dc.identifier.volume22en_US
dc.identifier.wosWOS:000444771200002
dc.identifier.wosqualityN/A
dc.indekslendigikaynakWeb of Science
dc.language.isoenen_US
dc.publisherUniv Indonesiaen_US
dc.relation.ispartofMakara Journal of Health Research
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectAtherosclerosisen_US
dc.subjectDental Careen_US
dc.subjectGrowth Retardationen_US
dc.subjectProgeriaen_US
dc.subjectPseudosenilismen_US
dc.subjectTooth Lossen_US
dc.titleA Rare Case of Hutchinson-Gilford Progeria Syndrome with Early Dental Loss without Decayen_US
dc.titleA Rare Case of Hutchinson-Gilford Progeria Syndrome with Early Dental Loss without Decay
dc.typeArticleen_US

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