Glaser D L, Rocke D M, Kaplan F S
Department of Orthopaedic Surgery, University of Pennsylvania School of Medicine, Philadelphia 19104, USA.
Clin Orthop Relat Res. 1998 Jan(346):110-6.
There have been numerous anecdotal reports of catastrophic falls in patients with fibrodysplasia ossificans progressiva. To determine the incidence of serious morbidity and mortality associated with falls in this patient population, the authors surveyed the 135 patient members of the International Fibrodysplasia Ossificans Progressiva Association and an age and gender matched control group. Eighty-one percent of the fibrodysplasia ossificans progressiva population suffered a fall resulting in injury compared with 44% of the controls. Sixty-seven percent of the falls initiated a painful flareup of fibrodysplasia ossificans progressiva leading to permanent loss of movement in almost all patients. Fifty-four percent of all falls suffered by the fibrodysplasia ossificans progressiva group led to permanent disability compared with 4% of all falls in the control group. Although trauma to the head was a common site of injury in both groups, the injury profile in the fibrodysplasia ossificans progressiva group included traumatic brain injuries, intracranial hemorrhage and death whereas the control group suffered mostly minor soft tissue lacerations. Deficiencies in coordinate gait and protective function likely accounted for the severity of injuries especially to the head in the fibrodysplasia ossificans progressiva population. Precautions are recommended that are intended to minimize the risk of injury without compromising a patient's functional level and independence. These recommendations include limitation of high risk activities, protective head gear, safety improvements in living environments, and augmentation of stabilizing and protective functions.
有许多关于进行性骨化性纤维发育不良患者灾难性跌倒的轶事报道。为了确定该患者群体中与跌倒相关的严重发病和死亡率，作者对国际进行性骨化性纤维发育不良协会的135名患者成员以及一个年龄和性别匹配的对照组进行了调查。进行性骨化性纤维发育不良患者群体中有81%的人跌倒后受伤，而对照组这一比例为44%。67%的跌倒引发了进行性骨化性纤维发育不良的疼痛发作，几乎所有患者因此导致永久性运动丧失。进行性骨化性纤维发育不良组所有跌倒中有54%导致永久性残疾，而对照组所有跌倒中这一比例为4%。虽然头部创伤在两组中都是常见的受伤部位，但进行性骨化性纤维发育不良组的损伤类型包括创伤性脑损伤、颅内出血和死亡，而对照组大多是轻微的软组织撕裂伤。协调步态和保护功能的缺陷可能是进行性骨化性纤维发育不良患者群体中尤其是头部受伤严重程度的原因。建议采取预防措施，以尽量降低受伤风险，同时不损害患者的功能水平和独立性。这些建议包括限制高风险活动、佩戴防护头盔、改善生活环境安全以及增强稳定和保护功能。
