【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(jìn)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(shí)驗(yàn)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(píng)價(jià)資料(北京兒童醫(yī)院版)。2.采用SPSS21.0統(tǒng)計(jì)軟件整理分析數(shù)據(jù),以P0.05為差異有統(tǒng)計(jì)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(shí)-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(shí)行血塊退縮試驗(yàn),有21例(77.8%)血塊退縮試驗(yàn)異常。將27例患兒根據(jù)初診時(shí)出血部位多少分為兩個(gè)或以上及一個(gè)或以下兩組,兩組之間血塊退縮試驗(yàn)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(yàn)分為正常組及異常組,兩組之間貧血嚴(yán)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(duì)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(píng)價(jià)資料(北京兒童醫(yī)院版)。隨訪時(shí)8歲及以上患兒有5例,均未再出血,達(dá)到8分滿分。2例隨訪時(shí)7歲患兒出血傾向較前減輕,達(dá)到7分。1例隨訪時(shí)1歲6月出血傾向較初診時(shí)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(duì)癥處理可以達(dá)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(xì)胞懸液糾正貧血,占80%,有8例貧血糾正困難者同時(shí)輸注機(jī)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(guó)際止血與血栓協(xié)會(huì)2010年推薦的出血評(píng)分標(biāo)準(zhǔn),有34例為異常出血,占53.1%。對(duì)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(píng)分相關(guān)(P0.05),Logistic回歸分析結(jié)果可見貧血與出血評(píng)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(yàn)是否異常對(duì)出血評(píng)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(yàn)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(yàn)是否異常及血小板最大聚集率不能衡量出血嚴(yán)重程度。無貧血和輕度貧血者占多數(shù),嚴(yán)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(píng)分明顯并獨(dú)立相關(guān),而性別、年齡、有無出血誘因、血塊收縮試驗(yàn)是否異常對(duì)出血評(píng)分均無影響。5.同一患兒隨年齡增長(zhǎng)其出血傾向有減輕的趨勢(shì),但仍有重要臟器出血致死的風(fēng)險(xiǎn)。
[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(jí)別】：碩士
【學(xué)位授予年份】：2015
【分類號(hào)】：R725.4

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