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VIII. EMERGENCY GUIDELINES FOR 1st RESPONDERS, PHYSICIANS & DENTISTS
急救人员，内科医生与牙医的急诊指南
<hr/>1. Avoid all IM injections unless necessary for survival of the patient. They will likely cause flareups and subsequent ossification.
除非在危机患者生命的情况下，否则一律避免所有肌肉（ IM ）注射。因为可能会引起FOP急性发作并随之发生骨化。
2. Peripheral IVs are permissible. Use smallest needle possible with brief tourniquet time. Avoid repeated tourniquet use or over-inflation of blood pressure cuffs.
允许静脉注射。尽可能使用最小的针头与快速的进入静脉。避免反复操作或过度使用血带。
3. Avoid central venous access.
避免中央静脉的介入注射。
4. In case of major trauma, begin corticosteroids immediately (oral) or IV equivalent of oral Prednisone - 1-2 mg/kg once daily for 4 days.
在发生重大创伤时，应立即（口服）或相当于口服量的泼尼松等IV类皮质类固醇 - 每天1-2毫克/千克，为期4天。
5. Pad all bony prominences to prevent pressure ulcers and skin breakdown.
异位骨突出的地方需要用软垫垫起垫，以防止压力下生成褥疮和皮肤溃面。
6. The cervical spine is often partially or completely ankylosed from FOP. Do not manipulate.
颈椎通常会部分或完全融合从。不需要任何处理。
7. The jaw is likely limited in movement or functionally ankylosed. Even if it is mobile, it is extremely susceptible to trauma. Do not passively manipulate. Over-stretching and mandibular blocks are forbidden as they will cause flare-ups.
下颚可能活动有限或者功能性异常。即使它能够移动，也极其容易受到创伤。不要被动受力。严禁过度拉伸和外界处理骨化部位，这样会导致FOP的急性发作。
8. Flare-ups of the anterior neck can compromise breathing and swallowing and should be considered a medical emergency. These submandibular flare-ups require early identification. Provide high dose steroids immediately (methylprednisolone 100 mg iv or Dexamethasone 4 mg iv). Avoid additional trauma with lesional manipulation. Airway monitoring, aspiration precautions, nutritional support, immediate use of corticosteroids.
颈部前部的肌肉发炎和FOP的急性发作会危及呼吸和吞咽，应被视为医疗急诊处理。这些次级FOP爆发应该提早识别。需立即给与高剂量类固醇使用。要避免额外的创伤与病变区手术等外界介入操作。需要进行气道监测，吸入等预防措施，给与营养支持，立即配合使用皮质类固醇进行控制。
9. Head and neck injuries are common from falls as the arms are rigid from ankylosis of the shoulders early in life and cannot be used to protect the head in case of falls.
头部和颈部受伤通常由跌倒引起，因为手臂在FOP发展早期都会因肩部骨化固定而变得僵硬，因此，患者在跌倒时不能极是用来保护头部。
10. With head injury, always brace the neck.
头部受伤时，请始终给颈部给予支撑。
11. With any head injury, even without loss of consciousness, a head CT is mandatory to rule out intracranial bleeding due to the high likelihood of an unprotected impact.
由于头部受伤，即使没有失去知觉，也需要通过CT去排除头部的颅内出血的可能性，因为这种出血极有可能在没有双手保护的情况下造成。
12. Flare-ups of the head in younger patients can appear as very large scalp swellings and initially disfiguring. A conservative approach should be taken with scalp flare-ups, with monitoring, and pain control if necessary. Scalp flare-ups will resolve spontaneously over time and disfigurement will be minimal to none as new ossifications are incorporated into the growing skull.
刚出生的婴儿患者头部的早期肿胀会出现在头皮部位。应采取保守的方法对头皮爆发进行监测，并在必要时控制疼痛。随着时间的推移，鳞片状的爆发会自发地解决，随着新骨化的融入在生长的头骨中后和颅骨的生长，对头部形态的影响将微乎其微。
13. Facial swelling due to scalp flare-ups in FOP is uncommon, and other etiologies for facial swelling should be considered. A brief course of antihistamine should be considered to exclude allergies in FOP patients who present with facial swelling.
由于 FOP 中的头皮爆发而导致的面部肿胀并不常见，需要考虑引起面部肿胀的其他原因。为了排除FOP患者因过敏导致的肿胀，可以使用短期的抗组胺类药物加以控制。
14. For “dirty” or contaminated wounds use tetanus hyperimmune globulin. Avoid tetanus immunization as IM or subcutaneous immunization unless necessary, as this has a high likelihood of inciting a flare-up.
对于较严重的伤口感染，可以使用破伤风免疫球蛋白。当然除非必要，否则应避免将破伤风免疫通过皮下注射接种，因为极有可能引发FOP急性发作。
15. Some hearing impairment is common in FOP. Speak loudly and clearly.
听力的障碍在FOP中很常见。
16. Although stable hearing loss is a common feature of FOP in children, acute hearing loss and ear pain is not and should be evaluated and treated as in any child.
FOP儿童的一个常见特征是听力会受损。但急性的听力下降和耳痛不是FOP的特征，应该去正常的耳科进行治疗。
17. Dental pain is a common issue in FOP patients and must be evaluated and treated promptly, but only after thorough consultation with an FOP dental expert. Overstretching of the jaw and mandibular blocks are forbidden.
牙痛是FOP患者的常见问题，必须及时进行诊断和治疗，但须经过与FOP牙科专家进行彻底咨询。严谨过度伸展下颚和下颚的肿块。
18. Kidney stones are common in adults with FOP. Keep well hydrated.
肾结石在患有FOP的成年人中很常见。保持日常大量饮水。
19. Fractures are common in normotopic as well as heterotopic bone. Closed immobilization with splinting and bracing are recommended. Open reduction is contraindicated unless thoroughly discussed with an FOP specialist.
骨折在正常骨和异位骨中很常见。建议使用夹板和支撑器具进行封闭固定。除非与 FOP 专家进行彻底讨论，否则禁止使用开放性手术治疗。
20. With nausea and vomiting in individuals with an ankylosed jaw, cover empirically with antibiotics for aspiration pneumonia.
通过抗生素去治疗肺炎。
21. Acute and often severe limb swelling can be seen with flare-ups of FOP, especially of the lower extremities. Due to intense inflammation, angiogenesis and capillary leakage, this swelling may grow to extraordinary and alarming size and lead to extravascular compression of nerves and tissue lymphatics. After excluding a possible deep vein thrombosis, the swelling should be treated conservatively with adequate pain control, elevation, and ultimately with safe lymphedema manipulations. Although signs and symptoms of compartment syndrome may prompt consideration of emergent surgical release of pressure, this will exacerbate the flare-up and MUST be avoided.
FOP的急性发作，特别是在下肢会有急性严重的肢体肿胀。伴随着强烈的炎症，肿块内的血管生成和毛细血管渗透，这种肿胀可能增长到异常惊人的大小，并导致神经和组织淋巴的血管外压缩。这时药排除潜在的深静脉血栓，通过保守疗法去控制疼痛和消肿，才能控制淋巴水肿。虽然这种症状需要通过紧急的手术释放压力，但会加速FOP的爆发，必须要避免进行手术。
22. In the case of limb swelling that prompts concern for deep vein thrombosis, Doppler ultrasound evaluation of the venous system may be indicated.
肢体肿胀会引起深静脉血栓的形成注，可以及时通过超声进行诊断。
23. Ask if patient is enrolled in any FOP Clinical Trials and communicate with principal investigator and regional FOP specialist.
询问患者是否参加了任何 FOP 临床试验，今早与FOP 专家沟通。
24. In the case of choking and failure to clear throat manually, perform Heimlich maneuver if there is no evidence for abdominal heterotopic bone that would prevent attempts.
在窒息和手动清理喉咙失败的情况下，请使用海姆里克腹部冲击法，避免因缺氧引起休克。
25. Chest compressions will likely be futile. The chest wall is rigid and immobile.
通过压缩胸部的操作可能不会起到任何作用，因为FOP患者的胸壁可能会骨化并处于僵硬状态。
26. Intubation must be through an awake, fiberoptic nasotracheal approach by an experienced anesthesiologist.
管内麻醉必须由经验丰富的麻醉师进行操作。
27. If an emergency tracheotomy is necessary in an individual with anterior neck ossifications, a dental or other drill may be necessary to create an airway.
前颈部骨化的患者若需要进行紧急的的气管切除术，则可能需要牙科医生的配合介入来创建气道。
28. In emergency situations where patients have difficulty clearing secretions, use bronchodilators, mucolytics, and guaifenesin, with a low threshold for mechanical insufflation-exsufflation devices. Hydration should be optimized with intravenous fluids.
在紧急情况下，对与患者难以清除的分泌物，使用支气管扩张剂、化痰剂和愈创木酚甘油醚。
本文译自：
THE MEDICAL MANAGEMENT OF FIBRODYSPLASIA OSSIFICANS PROGRESSIVA: CURRENT TREATMENT CONSIDERATIONS
进行性骨化性纤维发育不良（FOP）的医疗管理：当前治疗的注意事项
VIII. EMERGENCY GUIDELINES FOR 1st RESPONDERS, PHYSICIANS & DENTISTS
急救人员，内科医生与牙医的急诊指南
From The International Clinical Council on FOP (ICC) & Consultants:
来自国际临床理事会的FOP（ICC）专家和顾问：
发表于：2021.05
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发表于 2023-1-2 10:13:36