Górnik-Właszczuk Ewa, Majewski Jacek, Szczygieł Rafał, Dusiel Andrzej
Department of Anaesthesiology and Intensive Therapy, District Hospital of Orthopaedics and Trauma Surgery in Piekary Śląskie, Poland.
Anaesthesiol Intensive Ther. 2013 Apr-Jun;45(2):85-8. doi: 10.5603/AIT.2013.0019.
Osteogenesis imperfecta is a rare, genetically inherited syndrome involving connective tissue. It results in extremely fragile bones and disorders of other organs and body systems. Children with osteogenesis imperfecta are susceptible to bone fractures and often require surgery and anaesthesia. We describe a series of 14 general anaesthetics in three patients suffering from this disease. In one of these cases, perioperative hyperthermia was observed. Anaesthetic management of osteogenesis imperfecta and a possible relationship between this syndrome and malignant and non-malignant hyperthermia are discussed.
成骨不全症是一种罕见的涉及结缔组织的遗传性综合征。它会导致骨骼极其脆弱以及其他器官和身体系统的紊乱。患有成骨不全症的儿童易发生骨折，常常需要手术和麻醉。我们描述了对三名患有这种疾病的患者实施的14次全身麻醉。在其中一例中，观察到围手术期体温过高。本文讨论了成骨不全症的麻醉管理以及该综合征与恶性和非恶性高热之间可能存在的关系。
