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Herediter Multipl Egzositozis Tanısı ile Takip Edilen İki Kardeş.

Authors :
Erol, Meltem
Yiğit, Özgül
Adanır, Oktay
Toksöz, Mehmet
Narin, Halis
Gök, Veysel
Borakay, Dilek
Konya, Murat
Source :
Medical Bulletin of Haseki / Haseki Tip Bulteni. Jun2014, Vol. 52 Issue 2, p116-119. 4p.
Publication Year :
2014

Abstract

Hereditary multiple exostoses is an autosomal dominant disease with abnormal bone formation especially at the long bones. Osteochondromas, which occur in the course of the disease, can cause growth disturbances in affected children. Due to pressure effects of osteochondromas, compression of vessels, nerves and tendons, restriction of joint motion, and neurologic compromise as well as painful local symptoms can be seen. Here, we aimed to present two siblings who had generalized pain and swelling in different parts of the body. We detected multiple osteochondromas in different parts of their bodies, especially at the long bones. Our patients had painful local symptoms. There was no growth retardation, but the presence of many osteochondromas led us to contemplate that it was serious form of the disease. Their father had lesser number of osteochondromas. In this paper, we aimed to emphasize the necessity of close follow-up for the risk of malignant transformation of osteochondromas. [ABSTRACT FROM AUTHOR]

Details

Language :
Turkish
ISSN :
13020072
Volume :
52
Issue :
2
Database :
Academic Search Index
Journal :
Medical Bulletin of Haseki / Haseki Tip Bulteni
Publication Type :
Academic Journal
Accession number :
96579038
Full Text :
https://doi.org/10.4274/haseki.1364