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Extremity Manifestations and Surgical Treatment for Nasu Hakola Disease
Joint Authors
Yıldırım, Ahmet
Ekmekçi, Alp Burak
Arıkan, Murat
Toğral, Guray
Source
Issue
Vol. 2014, Issue 2014 (31 Dec. 2014), pp.1-3, 3 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2014-02-12
Country of Publication
Egypt
No. of Pages
3
Main Subjects
Abstract EN
Nasu-Hakola disease, which is also known as polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL), is a rare and mortal human genetic disorder (Verloes et al., (1997) and Bianchin et al., (2004)).
Nasu-Hakola is a progressive disease characterized by early onset cognitive dementia and bone cysts (both evident by the third decade).
The disease has a worldwide distribution, but most patients have been reported in Finland and in Japan (Montalbetti et al., (2004)).
In the literature less than 200 cases are reported and only a few of them are about the surgical treatment for the extremity (Madry et al., (2007)).
Most patients die by their fourth or fifth decade because of neurologic problems.
Surgeons generally prefer conservative treatment modalities in the treatment of cystic lesions of the bone in this syndrome.
In this case report, we presented a 42-year-old male with Nasu-Hakola disease having bilateral painful talar lipomembranous cystic lesions treated with curettage and iliac bone grafting.
He is in the 3rd year of his followup after surgery and he has not any extremity complaints, but his neurological problems sustain.
Our aim in this study is to show the beneficial aspect of surgical intervention in the cystic lesions of Nasu Hakola disease in the skeleton to obtain the patient a painless joint although surgery is rarely performed in this systemic and progressive disease.
American Psychological Association (APA)
Arıkan, Murat& Yıldırım, Ahmet& Toğral, Guray& Ekmekçi, Alp Burak. 2014. Extremity Manifestations and Surgical Treatment for Nasu Hakola Disease. Case Reports in Orthopedics،Vol. 2014, no. 2014, pp.1-3.
https://search.emarefa.net/detail/BIM-473154
Modern Language Association (MLA)
Arıkan, Murat…[et al.]. Extremity Manifestations and Surgical Treatment for Nasu Hakola Disease. Case Reports in Orthopedics No. 2014 (2014), pp.1-3.
https://search.emarefa.net/detail/BIM-473154
American Medical Association (AMA)
Arıkan, Murat& Yıldırım, Ahmet& Toğral, Guray& Ekmekçi, Alp Burak. Extremity Manifestations and Surgical Treatment for Nasu Hakola Disease. Case Reports in Orthopedics. 2014. Vol. 2014, no. 2014, pp.1-3.
https://search.emarefa.net/detail/BIM-473154
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-473154