PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT
Yıl: 2017 Cilt: 19 Sayı: 2 Sayfa Aralığı: 125 - 130 Metin Dili: İngilizce DOI: 10.24938/kutfd.317075 İndeks Tarihi: 02-09-2019
PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT
Öz: Pycnodysostosis, known as osteopetrosis acro-osteolytica, is a rare sclerotic bone disease with an autosomal recessive heritage pattern. Characteristic features of the disease are short stature, delayed closure of cranial sutures, partial or total dysplasia of the phalanges, obtuse mandibular angle, skeletal abnormalities and increased bone density. Type I collagen catabolism and bone resorption are defective in these patient due to lysosomal cysteine protease and cathepsin K enzyme defects resulting in dense and more brittle bones. Herein, we report a 45-year-old pycnodysostozis patient with a subtrochanteric transverse fracture of the left femur. We aim to reveal the difficulties encountered during intramedullary nailing of long bone fractures of patients with pycnodysostosis.
Anahtar Kelime: Konular:
Piknodisostozis: Uzun Kemik Kırıklarında Kanal İçi Çivilemenin Cerrahi Zorlukları: Vaka Sunumu
Öz: Osteopetrosis akro-osteolotika olarak da bilinen piknodisostozis, otozomal resesif kalıtım özelliği gösteren, nadir görülen sklerotik bir kemik hastalığıdır. Hastalığın karakteristik özellikleri boy kısalığı, kraniyel sütürlerin geç kapanması, falanksların tam ya da kısmi displazisi, mandibula köşelerinin geniş açılı olması, iskelet anomalileri ve artmış kemik yoğunluğudur. Bu hastalarda, lizozomal sistein proteaz ve katepsin K enzim defektleri nedeniyle kırılgan kemik oluşumuna neden olan tip I kollajen katabolizması ve kemik rezorbsiyon defektleri mevcuttur. Bu makalede, sol subtrokanterik femur kırığı gelişmiş olan piknosiostozisli 45 yaşında bir hasta bildirilmektedir. Amacımız, piknodisostozis hastalarında uzun kemik kırıklarının kanal içi çivilenmesi sırasında karşılaşılan zorlukları gözden geçirmektir.
Anahtar Kelime: Konular:
Belge Türü: Makale Makale Türü: Olgu Sunumu Erişim Türü: Erişime Açık
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| APA | Canbeyli I, Cirpar M, OKTAŞ B, ÖKKEŞİM C (2017). PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT. , 125 - 130. 10.24938/kutfd.317075 |
| Chicago | Canbeyli Ibrahim Deniz,Cirpar Meric,OKTAŞ Birhan,ÖKKEŞİM Cüneyt Emre PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT. (2017): 125 - 130. 10.24938/kutfd.317075 |
| MLA | Canbeyli Ibrahim Deniz,Cirpar Meric,OKTAŞ Birhan,ÖKKEŞİM Cüneyt Emre PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT. , 2017, ss.125 - 130. 10.24938/kutfd.317075 |
| AMA | Canbeyli I,Cirpar M,OKTAŞ B,ÖKKEŞİM C PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT. . 2017; 125 - 130. 10.24938/kutfd.317075 |
| Vancouver | Canbeyli I,Cirpar M,OKTAŞ B,ÖKKEŞİM C PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT. . 2017; 125 - 130. 10.24938/kutfd.317075 |
| IEEE | Canbeyli I,Cirpar M,OKTAŞ B,ÖKKEŞİM C "PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT." , ss.125 - 130, 2017. 10.24938/kutfd.317075 |
| ISNAD | Canbeyli, Ibrahim Deniz vd. "PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT". (2017), 125-130. https://doi.org/10.24938/kutfd.317075 |
| APA | Canbeyli I, Cirpar M, OKTAŞ B, ÖKKEŞİM C (2017). PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT. Kırıkkale Üniversitesi Tıp Fakültesi Dergisi, 19(2), 125 - 130. 10.24938/kutfd.317075 |
| Chicago | Canbeyli Ibrahim Deniz,Cirpar Meric,OKTAŞ Birhan,ÖKKEŞİM Cüneyt Emre PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT. Kırıkkale Üniversitesi Tıp Fakültesi Dergisi 19, no.2 (2017): 125 - 130. 10.24938/kutfd.317075 |
| MLA | Canbeyli Ibrahim Deniz,Cirpar Meric,OKTAŞ Birhan,ÖKKEŞİM Cüneyt Emre PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT. Kırıkkale Üniversitesi Tıp Fakültesi Dergisi, vol.19, no.2, 2017, ss.125 - 130. 10.24938/kutfd.317075 |
| AMA | Canbeyli I,Cirpar M,OKTAŞ B,ÖKKEŞİM C PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT. Kırıkkale Üniversitesi Tıp Fakültesi Dergisi. 2017; 19(2): 125 - 130. 10.24938/kutfd.317075 |
| Vancouver | Canbeyli I,Cirpar M,OKTAŞ B,ÖKKEŞİM C PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT. Kırıkkale Üniversitesi Tıp Fakültesi Dergisi. 2017; 19(2): 125 - 130. 10.24938/kutfd.317075 |
| IEEE | Canbeyli I,Cirpar M,OKTAŞ B,ÖKKEŞİM C "PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT." Kırıkkale Üniversitesi Tıp Fakültesi Dergisi, 19, ss.125 - 130, 2017. 10.24938/kutfd.317075 |
| ISNAD | Canbeyli, Ibrahim Deniz vd. "PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT". Kırıkkale Üniversitesi Tıp Fakültesi Dergisi 19/2 (2017), 125-130. https://doi.org/10.24938/kutfd.317075 |
