【摘要】：目的通過回顧性分析我院64例遺傳性血小板功能障礙疾病住院患兒的病例資料及其進行問卷調(diào)查,分析總結(jié)遺傳性血小板功能障礙疾病的臨床特征和實驗室資料,了解遺傳性血小板功能障礙疾病患兒的日常生活質(zhì)量,旨在為遺傳性血小板功能障礙疾病尤其血小板無力癥的規(guī)范化診斷、治療提供參考依據(jù)。方法1.收集1996年4月-2014年9月重慶醫(yī)科大學(xué)附屬兒童醫(yī)院收治的64例臨床診斷遺傳性血小板功能障礙疾病患兒的臨床資料,部分病例以電話回訪形式獲得出血性疾病日常生活評價資料(北京兒童醫(yī)院版)。2.采用SPSS21.0統(tǒng)計軟件整理分析數(shù)據(jù),以P0.05為差異有統(tǒng)計學(xué)意義。結(jié)果1.64例遺傳性血小板功能障礙疾病患兒,男女比例約為1.21：1。2.64例患兒發(fā)病年齡1小時-14歲3月,中位年齡：17月,發(fā)病年齡1歲及1歲以下有29例(45.3%),3歲及3歲以下有44例(64%)。其中有2例生后即診斷。3.64例遺傳性血小板功能障礙疾病患兒中,有12例父親和(或)母親行血小板聚集功能檢查,有8例父親和(或)母親血小板最大聚集率降低,占66.7%。4.64例遺傳性血小板功能障礙疾病患兒中無明顯誘因出血最常見,有44例,占68.8%,其次分別是上呼吸道感染(10.9%)、外傷(9.4%)后出血較多見。5.出血表現(xiàn)中皮膚紫癜最常見,占64.1%,其次鼻衄、消化道出血、牙齦出血、創(chuàng)傷后出血難止,分別占54.7%、17.2%、14.1%、14.1%。有2例青春期女性患兒,均有月經(jīng)過多表現(xiàn)。6.64例患兒中27例同時行血塊退縮試驗,有21例(77.8%)血塊退縮試驗異常。將27例患兒根據(jù)初診時出血部位多少分為兩個或以上及一個或以下兩組,兩組之間血塊退縮試驗異常率無顯著性差異(P0.05)。7.64例遺傳性血小板功能障礙疾病根據(jù)貧血程度分為正常、輕度、中度、重度、極重度,無貧血和輕度貧血者占53.1%,中度、重度貧血者占46.9%,無極重度貧血者。8.將血塊退縮試驗分為正常組及異常組,兩組之間貧血嚴重程度無顯著性差異(P0.05)。9.出血部位的多少與血小板最大聚集率無明顯差異(P0.05)。10.比較嬰兒期、幼兒期、學(xué)齡前期、學(xué)齡期不同年齡組患兒血小板最大聚集率無明顯差異(P0.05)。11.對其中8例患兒以電話回訪形式完成了出血性疾病日常生活評價資料(北京兒童醫(yī)院版)。隨訪時8歲及以上患兒有5例,均未再出血,達到8分滿分。2例隨訪時7歲患兒出血傾向較前減輕,達到7分。1例隨訪時1歲6月出血傾向較初診時減輕。有1例死亡病例,因肺出血致失血性休克死亡。12.64例遺傳性血小板功能障礙疾病患兒中,所有病例通過局部止血、輸血支持及對癥處理可以達到防止出血的目的。30例中度、重度貧血患兒中,有24例輸注紅細胞懸液糾正貧血,占80%,有8例貧血糾正困難者同時輸注機采血小板止血治療,占26.7%。本組資料中,有2例表現(xiàn)為月經(jīng)過多的青春期女性患兒均予以口服雌激素治療,療效較好。13.64例遺傳性血小板功能障礙疾病患兒按照國際止血與血栓協(xié)會2010年推薦的出血評分標準,有34例為異常出血,占53.1%。對全部64例遺傳性血小板功能障礙疾病患兒發(fā)生異常出血的單因素分析,發(fā)病年齡、是否貧血與出血評分相關(guān)(P0.05),Logistic回歸分析結(jié)果可見貧血與出血評分顯著相關(guān)(B值3.47,OR值32.024,P=0.000),而性別、年齡、有無出血誘因、血塊收縮試驗是否異常對出血評分均無影響。結(jié)論1.IPFD患兒以男性較多。發(fā)病年齡嬰幼兒占多數(shù)。父母行血小板聚集試驗中超過一半有陽性家族史。2.遺傳性血小板功能障礙常無明顯誘因發(fā)生出血,其次是上呼吸道感染及外傷后誘發(fā)。出血癥狀中皮膚紫癜最常見。青春期女性患兒,常有月經(jīng)過多表現(xiàn)。3.血塊退縮試驗是否異常及血小板最大聚集率不能衡量出血嚴重程度。無貧血和輕度貧血者占多數(shù),嚴重出血導(dǎo)致重度貧血少見。起病年齡的早晚與血小板最大聚集率無關(guān)。4.貧血程度與出血評分明顯并獨立相關(guān),而性別、年齡、有無出血誘因、血塊收縮試驗是否異常對出血評分均無影響。5.同一患兒隨年齡增長其出血傾向有減輕的趨勢,但仍有重要臟器出血致死的風(fēng)險。
[Abstract]:Objective To retrospectively analyze the clinical characteristics and laboratory data of 64 cases of hereditary platelet dysfunction disease in our hospital, and to analyze the clinical characteristics and laboratory data of hereditary platelet dysfunction disease. Objective To study the quality of daily life in children with inherited platelet dysfunction and to provide a reference basis for the standardized diagnosis and treatment of hereditary thrombocytopenia. Method 1. Collect the clinical data of 64 cases of hereditary platelet dysfunction disease from April 1996 to September 2014 in the Affiliated Hospital of Chongqing Medical University, some cases receive the daily life evaluation data of hemorrhagic disease (Beijing Children's Hospital Edition) in the form of telephone return visit (Beijing Children's Hospital Edition). SPSS 10.0 was used to analyze the data, and the difference was statistically significant with P0.05. Results 1. 64 cases of hereditary platelet dysfunction disease had an age of 1. 21: 1. 64 children were aged 1 hour to 14 years old, and the median age was 17 months, 29 cases (45.3%) were found under the age of 1 year and 1 year old, 44 cases (64%) were under the age of 3 years and under the age of 3 years. Among them, 2 of them were diagnosed. Among them, 12 fathers and/ or mother had platelet aggregation function examination in 12 cases of hereditary platelet dysfunction disease, and 8 cases of father and/ or mother's platelet maximum aggregation rate decreased. Of the 64 patients with hereditary thrombocytopenia, there were 44 cases (68.8%), followed by upper respiratory tract infection (10. 9%) and trauma (94.4%). The most common cause of skin purpura in bleeding was 64.1%, followed by nosebleed, gastrointestinal bleeding, gingival bleeding, and post-traumatic hemorrhage, accounting for 54. 7%, 17. 2%, 14.1%, 14.1%, respectively. There were 2 children with adolescent girls, and 27 of 64 children had a blood clot withdrawal test at the same time. There were 21 cases (77.8%) of the abnormal blood clot withdrawal test. There was no significant difference between the two groups (P0.05). 64 cases of hereditary thrombocytopenia were divided into normal, mild, moderate, severe and extremely severe according to the degree of anemia. No anemia and mild anemia accounted for 53. 1%, moderate and severe anemia accounted for 46. 9% and non-polar severe anemia. 8. There was no significant difference between the two groups (P0.05). There was no significant difference between the number of bleeding sites and the maximum platelet aggregation rate (P0.05). There was no significant difference in platelet maximum aggregation rate among infants in infancy, early childhood, pre-school period and age group (P0.05). The daily life evaluation data of hemorrhagic disease (Beijing Children's Hospital) was completed in 8 children with telephone return visit. In the follow-up, there were 5 cases with no rebleeding after 8 years of follow-up and 8 full marks. The bleeding tendency of 7-year-old children at follow-up was lower than before, reaching 7 points. There were 1 case of death, hemorrhagic shock death due to pulmonary hemorrhage. 12. 64 cases of hereditary platelet dysfunction disease, all cases were treated by local hemostasis, transfusion support and symptomatic treatment to prevent bleeding. In 30 patients with moderate and severe anemia, There were 24 cases of red blood cell suspension in order to correct anemia, accounting for 80%, and 8 patients with anemia were treated with platelet hemostasis at the same time, accounting for 26. 7%. In this group, 2 children with hereditary thrombocytopenia were treated by oral estrogen, and the curative effect was better. In 64 cases of hereditary platelet dysfunction, 34 cases were abnormal bleeding according to the standard of bleeding recommended by the International Hemostatic and Thrombosis Association in 2010. 53. 1%. A single factor analysis of abnormal bleeding occurred in all 64 patients with inherited platelet dysfunction, the age of onset, whether anemia was associated with bleeding score (P0.05), and the results of logistic regression analysis showed that anemia was significantly correlated with bleeding score (B value 3.47, OR value 32.24, P = 0. 000), while sex, Age, presence or absence of bleeding cause, blood clot retraction test had no effect on bleeding score. Conclusion 1. IPFD children are more male. There are a majority of infants in the age of onset. More than half of the parent's platelet aggregation test had a positive family history. hereditary platelet dysfunction often has no obvious cause of hemorrhage, followed by upper respiratory tract infection and post-traumatic induction. Henoch-Schonlein purpura is most common in hemorrhagic symptoms. in adolescent girls, there are often too many manifestations. The abnormality of the blood clot retraction test and the maximum platelet aggregation rate do not measure the severity of the bleeding. No anemia and mild anemia are in the majority, and severe bleeding is a rare cause of severe anemia. The time of onset of onset was not related to the maximum platelet aggregation rate. There was no significant correlation between anemia degree and bleeding score, but sex, age, presence or absence of bleeding cause, and abnormal blood clot shrinkage test had no effect on bleeding score. 5. There is a tendency to alleviate the bleeding tendency of the same child with age, but there is still a risk of death of important organ hemorrhage.
【學(xué)位授予單位】：重慶醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2015
【分類號】：R725.4

【相似文獻】

相關(guān)期刊論文 前10條

1 ;血小板疾病[J];國外科技資料目錄(醫(yī)藥衛(wèi)生);2002年12期

2 張立平;阿斯匹林抑制血小板功能的臨床研究[J];甘肅科技;2002年03期

3 包承鑫;獲得性血小板功能缺陷癥[J];臨床內(nèi)科雜志;2004年12期

4 包承鑫;;血小板功能監(jiān)測在抗血小板治療中的應(yīng)用現(xiàn)狀[J];血栓與止血學(xué);2012年05期

5 iJ蜀蓮;;其它能影響血小板功能的藥物[J];國外醫(yī)學(xué)參考資料.神經(jīng)病學(xué)神經(jīng)外科學(xué)分冊;1974年01期

6 諸美瞻;;葡萄糖-6-磷酸酶缺陷時血小板功能的損害[J];國外醫(yī)學(xué)參考資料(兒科學(xué)分冊);1976年01期

7 王鴻利;;遺傳性血小板功能缺陷[J];國外醫(yī)學(xué)參考資料(內(nèi)科學(xué)分冊);1977年01期

8 李次芬;;血小板功能與血小板功能試驗[J];國外醫(yī)學(xué).臨床生物化學(xué)與檢驗學(xué)分冊;1983年02期

9 高海泉;汪鐘;;中藥對血小板功能的影響[J];中西醫(yī)結(jié)合雜志;1984年04期

10 李仕勇,楊衛(wèi)東,陳綺年;血小板功能抑制劑防治動脈粥樣硬化的研究現(xiàn)狀[J];安徽醫(yī)學(xué);1985年01期

相關(guān)會議論文 前10條

1 戴克勝;劉光磊;王月丹;李素萍;阮長耿;;不同重力環(huán)境下血小板功能改變及其致病機制研究[A];中國病理生理學(xué)會第九屆全國代表大會及學(xué)術(shù)會議論文摘要[C];2010年

2 戴克勝;王月丹;閆榮;史權(quán)威;元艷宏;王志成;程虹;李素萍;樊瑜波;莊逢源;;重力對血小板功能影響的研究[A];中國空間科學(xué)學(xué)會第七次學(xué)術(shù)年會會議手冊及文集[C];2009年

3 朱妙章;藏益民;陳士良;馬新亮;趙志青;;局部缺血區(qū)血液流變學(xué)和血小板功能的變化特點[A];中國保健醫(yī)學(xué)研究會心臟學(xué)學(xué)會全國第一屆心臟學(xué)學(xué)術(shù)會議論文匯編[C];1995年

4 馬建林;李斌;陳關(guān)良;蘇哲坦;李新明;王圣;吳忠;;冠心病心絞痛患者血小板功能與血脂水平相關(guān)[A];全國第十屆心臟學(xué)學(xué)會、第十三屆心功能學(xué)會、《心臟雜志》編輯部聯(lián)合學(xué)術(shù)會議論文摘要集[C];2007年

5 姚紅霞;黃莉;林麗娥;吳從明;姚志明;王谷云;王華;饒若;符祥俊;唐瑞梅;;原發(fā)性血小板增多癥患者血小板功能指標的變化及其與血小板數(shù)量關(guān)系的研究[A];第12屆全國實驗血液學(xué)會議論文摘要[C];2009年

6 馬中富;李天木;關(guān)開泮;黎湛興;詹紅;徐鐘源;蔣祖勛;;慢性肺心病急性期患者血漿內(nèi)皮素-1與血小板功能關(guān)系的探討[A];中華醫(yī)學(xué)會急診醫(yī)學(xué)學(xué)會第六次全國急診醫(yī)學(xué)學(xué)術(shù)會議論文匯編[C];1996年

7 胡朝暉;鄒云增;;粒細胞集落刺激因子對兔動脈粥樣硬化過程中血小板功能的影響[A];中華醫(yī)學(xué)會心血管病學(xué)分會第十次全國心血管病學(xué)術(shù)會議匯編[C];2008年

8 李虎;賈國良;郭文怡;王海昌;秦濤;王小燕;趙惠強;;紫杉醇慢速釋放支架置入術(shù)后血小板功能的變化[A];中華醫(yī)學(xué)會心血管病分會第八次全國心血管病學(xué)術(shù)會議匯編[C];2004年

9 張堅;王春榮;戴建華;陳孝曙;葛可佑;楊淑華;蔡琪春;張玉玲;趙文華;閻文富;;棕櫚油對成人血脂和血小板功能的影響[A];中國營養(yǎng)學(xué)會第七屆全國營養(yǎng)學(xué)術(shù)會議論文摘要匯編[C];1996年

10 秦濤;李虎;賈國良;郭文怡;王海昌;李偉杰;劉兵;;直接支架置入術(shù)對冠狀動脈循環(huán)白細胞及血小板功能的影響[A];中華醫(yī)學(xué)會心血管病分會第八次全國心血管病學(xué)術(shù)會議匯編[C];2004年

相關(guān)重要報紙文章 前5條

1 呂乃群;戒煙兩周即可改善血小板功能[N];衛(wèi)生與生活報;2006年

2 記者 李天舒 孟曉捷;服阿司匹林預(yù)防血栓應(yīng)檢測血小板功能[N];健康報;2012年

3 北京朝陽醫(yī)院心臟中心主任醫(yī)師 楊新春 丁梟偉;冠心病患者少吃糖多飲水[N];保健時報;2009年

4 王開珍;少女缺乏脂肪會滋生疾病[N];廣東科技報;2004年

5 健康時報記者　 楊波;糖尿病人更易中風(fēng)[N];健康時報;2006年

相關(guān)碩士學(xué)位論文 前10條

1 高敏;遺傳性血小板功能障礙疾病64例臨床分析[D];重慶醫(yī)科大學(xué);2015年

2 張武飛;急性創(chuàng)傷兒童術(shù)中血小板功能變化[D];河北醫(yī)科大學(xué);2008年

3 段衛(wèi);不同強度的規(guī)則運動對血小板功能的影響[D];揚州大學(xué);2001年

4 杜惠琴;葡多酚對血小板功能與血栓形成的影響作用[D];青島大學(xué);2005年

5 李祖蘭;血小板功能實驗分析前質(zhì)量控制的研究[D];中國人民解放軍軍醫(yī)進修學(xué)院;2010年

6 盧潔;Sonoclot監(jiān)測冠心病病人服用不同劑量雙聯(lián)抗血小板藥物后凝血及血小板功能變化[D];天津醫(yī)科大學(xué);2012年

7 曹豐;小冠脈支架對冠心病患者血漿血小板功能及血管活性物質(zhì)作用的臨床研究[D];第四軍醫(yī)大學(xué);2001年

8 程彥;抗炎抗凝雙效融合蛋白TAP-SSL5對血小板功能的影響[D];第三軍醫(yī)大學(xué);2012年

9 肖淳純;不同抗糖尿病治療方案對2型糖尿病患者血小板功能的影響[D];安徽醫(yī)科大學(xué);2008年

10 關(guān)杰;臨床實驗室監(jiān)測抗血小板治療的方法學(xué)評價[D];中國人民解放軍醫(yī)學(xué)院;2014年

，

本文編號：2253536