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首页> 外文期刊>BMC Musculoskeletal Disorders >Incidentally diagnosed melorheostosis of upper limb: case report
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Incidentally diagnosed melorheostosis of upper limb: case report

机译:偶然诊断为上肢骨髓造血术:病例报告

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Melorheostosis is quite a rare bone disease with still unclear ethiology. Although multifocal affection is highly debilitating with unfavorable prognosis, there is no clear consensus about therapeutical approach. There is still insufficient evidence in the literature for almost a century after the first description. Affected bone has a typical appearance of melting wax. Diagnosis is usually incidental with pain as a leading symptom. Diagnosis itself is relatively easy, routine X-ray examination is sufficient. Even though it could be easily overlooked and mistaken with other diseases. Melorheostosis is incurable, the therapy is mostly focused on maintaining patient quality of life. Presented case is unique in terms of extent of the affection (index finger, metacarp shaft, carpal bones, forearm, humerus and whole scapula) in combination with osteopoikilotic islands in other 3 regions (vertebrae, manubrium sterni and left collar bone). Currently there is only one such a case published in the literature (Campbell), but without osteopoikilotic islands. Melorheostosis was diagnosed in 26-year old female after injury as an incidental finding. This was quite surprising as the patient already suffered by limited movement in the upper limb and pain before the injury. Detailed examination were performed to confirm the diagnosis, no family history was found. Pharmacotherapy with bisphosphonates, non-steroidal antirheumatics and vasodilatans/rheologic drugs seemed to be effective to maintain the relatively good quality of patient life and good performance in daily routine. Questionable is further development of patient performance status and sustainability of conservative treatment in the long term follow up. Conservative treatment with bisphopshonates and COX-2 inhibitors in combination with naftidrofuryl can delay surgery solution.
机译:髓鞘充血是一种罕见的骨病,病因尚不明确。尽管多灶性情感病会严重损害患者的预后,但有关治疗方法尚无明确共识。在首次描述之后的近一个世纪中,文献中仍然没有足够的证据。患骨具有典型的熔化蜡外观。诊断通常是伴随疼痛而导致的主要症状。诊断本身比较容易,常规X线检查就足够了。即使很容易被其他疾病忽视和误解。梅尔霍斯汀病是无法治愈的,治疗主要集中在维持患者的生活质量上。本病例与其他3个区域(椎骨,手掌胸骨和左颈骨)的骨质后遗症结合,在影响范围(食指,掌骨,腕骨,前臂,肱骨和整个肩cap骨)方面是独特的。目前,在文献中(Campbell)只发表了一种这样的病例,但是没有骨质疏松症的岛。意外受伤后,在26岁的女性中诊断出Melorheostosis。这非常令人惊讶,因为该患者在受伤之前已经受到上肢活动受限和疼痛的折磨。进行详细检查以确认诊断,未发现家族病史。用双膦酸盐,非甾体类抗风湿药和血管扩张药/流变药进行药物治疗似乎可以有效地维持患者相对良好的生活质量和日常良好的表现。在长期随访中,患者表现状态的进一步发展和保守治疗的可持续性值得怀疑。与双膦酸盐和COX-2抑制剂联合萘呋太尔的保守治疗可延迟手术解决方案。

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