【摘要】：目的：研究兒童血友病臨床發(fā)病及診斷年齡、臨床表現(xiàn)及治療等情況。 方法：回顧性分析2005年1月至2011年11月廣西醫(yī)科大學(xué)第一附屬醫(yī)院住院收治的65例來自廣西各地的兒童血友病患者的臨床資料。 結(jié)果：65例血友病患兒中血友病A共54例(83.08%)：重型13例(24.07%),中型22例(40.74%),輕型17例(31.48%),亞臨床型2例(3.70%)；血友病B共11例(16.92%)：重型3例(27.27%),中型4例(36.36%),輕型3例(27.27%),亞臨床型1例(9.09%)。血友病A：血友病B=4.9：1。首次出血中位數(shù)年齡為1歲,首次診斷中位數(shù)年齡為2歲2個月。65例患兒中有關(guān)節(jié)出血癥狀33例(50.77%),其中重型血友病13例(81.25%),中型15例(57.69%),輕型5例(25.00%)。65例中發(fā)生關(guān)節(jié)畸形17例(26.15%),其中血友病A重型7例(53.85%),中型6例(27.27%)；血友病B重型2例(66.67%),中型2例(50.00%)。重度出血共10例,均為重型及中型患兒。兩個以上部位出血共27例,其中重、中型患兒占24例。亞臨床型患兒均為醫(yī)源性出血。65例患兒中有明確血友病家族史18例(27.69%)。62例接受過替代治療73例次,急性出血時單純應(yīng)用凝血因子輸注者29例次(39.73%)；使用凝血因子的同時使用冷沉淀和(或)新鮮冰凍血漿35例次(47.95%)；僅使用冷沉淀或新鮮冰凍血漿9例次(12.33%)。無1例進行預(yù)防性替代治療。65例患兒均未進行血制品相關(guān)性感染性疾病的系統(tǒng)監(jiān)測。1例14歲患兒抗-HCV抗體陽性。 結(jié)論：(1)血友病兒童首次診斷年齡與首次出血年齡之間存在差距,存在診斷延遲現(xiàn)象；(2)出血部位、出血嚴重程度與血友病臨床分型密切相關(guān)；(3)關(guān)節(jié)出血及關(guān)節(jié)畸形發(fā)生率較高；(4)存在替代治療不及時、使用因子制劑替代治療率低的狀況；(5)本組患兒均未進行血制品相關(guān)性感染性疾病的系統(tǒng)監(jiān)測。
[Abstract]:Objective: to study the clinical onset, diagnostic age, clinical manifestation and treatment of hemophilia in children. Methods: the clinical data of 65 children hemophilia patients admitted to the first affiliated Hospital of Guangxi Medical University from January 2005 to November 2011 were retrospectively analyzed. Results: of the 65 cases of hemophilia, 54 cases (83.08%) were hemophilia A, 13 cases (24.07%) were severe, 22 cases (40.74%) were medium, 17 cases (31.48%) were mild, and 2 cases (3.70%) were subclinical type. There were 11 cases (16.92%) of hemophilia B, 3 cases of severe type (27.27%), 4 cases of medium type (36.36%), 3 cases of mild type (27.27%) and 1 case of subclinical type (9.09%). Hemophilia A: hemophilia B / 4. 9 / 1. The median age of the first hemorrhage was 1 year old and the median age of the first diagnosis was 2 years and 2 months. Of the 65 children, 33 (50.77%) had symptoms of joint hemorrhage, 13 (81.25%) had severe hemophilia, 15 (57.69%) had moderate hemophilia. Among 65 cases, 17 cases (26.15%) developed joint malformations, including 7 cases (53.85%) of hemophilia A and 6 cases (27.27%) of medium-sized hemophilia. Hemophilia B was severe in 2 cases (66.67%) and medium in 2 cases (50.00%). There were 10 cases of severe hemorrhage, all of them were severe and medium-sized children. There were 27 cases of bleeding in more than two sites, of which 24 cases were severe and medium-sized. 18 cases (27.69%) had a clear family history of hemophilia, 62 cases had received replacement therapy 73 times, 29 cases (39.73%) were treated with coagulation factor infusion alone during acute hemorrhage, and 18 cases (27.69%) had a clear family history of hemophilia, 62 cases had received replacement therapy for 73 times, and 29 cases (39.73%) had only used coagulation factor infusion during acute hemorrhage. Coagulation factors were used in 35 cases (47.95%) with cold precipitation and / or fresh frozen plasma, and 9 cases (12.33%) with only cryoprecipitation or fresh frozen plasma. None of them were treated with prophylactic replacement therapy. 65 children were not systematically monitored for infectious diseases associated with blood products. One 14-year-old child was positive for anti-HCV antibody. Conclusion: (1) there is a difference between the age of first diagnosis and the age of first hemorrhage in hemophilia children, and there is delay in diagnosis, (2) the location of hemorrhage and the severity of hemorrhage are closely related to the clinical classification of hemophilia. (3) the incidence of joint hemorrhage and joint deformity was high; (4) there was a situation that replacement therapy was not timely and the rate of replacement therapy with factor preparation was low; (5) there was no systematic monitoring of infectious diseases associated with blood products in this group.
【學(xué)位授予單位】：廣西醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2012
【分類號】：R725.5

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