CAJ | 학술논문

　　目的报告1例血友病性关节病误诊35年的病例，为临床医师提供借鉴的经验与教训。方法患者，男，42岁；7岁起发病，35年，多家医院均未能确诊，术后方明确血友病性关节病的诊断。结果患者7岁时出现“双髋、双膝酸困不适及疼痛，渐进性加重，伴关节活动幅度逐渐减小”等症状被当地医院误诊为“类风湿性关节炎”对症治疗，因效果不佳，于1个月后在当地人民医院误诊为“双膝骨结核”，给予口服抗结核药物治疗，症状无缓解，之后双髋、双膝关节反复出现疼痛、肿胀、活动受限达35年。于近期加重，我院以“双膝关节类风湿性关节炎”收住院。行膝关节表面置换术，尽管术后给予伤口处加压包扎、冰敷、常压引流等处理，术后第1天引流量高达2400 ml，且伤口敷料持续出现大量渗血。始考虑是否有血液系统疾病漏诊即请血液科会诊。查：特异性凝血因子 VIII 为13.70(正常值：0.60～1.60)、 IX 103.30(正常值：0.80～1.20)方确诊为甲型血友病性关节病。结论本例甲型血友病性关节病长期误诊，教训深刻。提示临床医师须强化病史问诊的能力，重视相关疾病的体征以提高对不同关节炎疾病的鉴别诊断能力，必要时，应及时请相关专科医师会诊。
　　목적보고1례혈우병성관절병오진35년적병례，위림상의사제공차감적경험여교훈。방법환자，남，42세；7세기발병，35년，다가의원균미능학진，술후방명학혈우병성관절병적진단。결과환자7세시출현“쌍관、쌍슬산곤불괄급동통，점진성가중，반관절활동폭도축점감소”등증상피당지의원오진위“류풍습성관절염”대증치료，인효과불가，우1개월후재당지인민의원오진위“쌍슬골결핵”，급여구복항결핵약물치료，증상무완해，지후쌍관、쌍슬관절반복출현동통、종창、활동수한체35년。우근기가중，아원이“쌍슬관절류풍습성관절염”수주원。행슬관절표면치환술，진관술후급여상구처가압포찰、빙부、상압인류등처리，술후제1천인류량고체2400 ml，차상구부료지속출현대량삼혈。시고필시부유혈액계통질병루진즉청혈액과회진。사：특이성응혈인자 VIII 위13.70(정상치：0.60～1.60)、 IX 103.30(정상치：0.80～1.20)방학진위갑형혈우병성관절병。결론본례갑형혈우병성관절병장기오진，교훈심각。제시림상의사수강화병사문진적능력，중시상관질병적체정이제고대불동관절염질병적감별진단능력，필요시，응급시청상관전과의사회진。
Objective To report a case of hemophilic arthritis misdiagnosed for 35 years, and to provide clinicians with experience and lessons for reference. Methods The patient was from the Department of Orthopedics of the 2nd Affiliated Hospital of Shanxi Medical University, who had the disease at the age of 7. Definite diagnosis has not been made even though the patient had been seeking medical advice from county, district and provincial hospitals in the past 35 years. Only after the operation, the diagnosis of hemophilic arthritis was confirmed. Results This 42-year-old male patient felt painful, tired and discomfortable in both hips and knee joints at the age of 7, which was aggravated progressively, with the range of motion reduced gradually. Because of the symptoms stated above, he was misdiagnosed as“rheumatoid arthritis ( RA )”in the local hospital. While the symptomatic treatment did not work well. A month later, he was misdiagnosed as“double knee bone tuberculosis”in the local people’s hospital. Oral anti-tuberculosis drugs could not help relieve the symptoms, and pain, swelling and limited range of motion occurred repeatedly in both hips and knee joints in the following 35 years. The patient’s conditions worsened recently, and he was misdiagnosed as“double knee RA”in our hospital. Total knee arthroplasty was performed. Although such treatments were given to the wound postoperatively as pressure dressing, ice compress, atmospheric drainage and so on, the drainage volume reached up to 2400 ml at the 1st day after the operation and massive bleeding was continuously found at the wound dressing. We began to consider whether the patient had hematological diseases, and then we held a consultation with the doctors from the Hematology Department. The results showed that the specific coagulation factor VIII was 13.70 ( the normal values were 0.60-1.60 ), and the specific coagulation factor IX was 103.30 ( the normal values were 0.80-1.20 ). Finally, the diagnosis of hemophilia arthritis was confirmed. Conclusions The long-term misdiagnosis of this case of hemophilia arthritis is a profound lesson, which reminds the clinicians to strengthen the capability of inquiring the medical history. More attention is needed to detect the signs of related diseases and to improve the ability of differentially diagnosing different kinds of arthritis. When necessary, it is advised to hold a consultation with related specialists without delay.