本文選題：兒童 + 急性再生障礙性貧血　； 參考：《鄭州大學》2012年碩士論文

【摘要】：目的 探討兒童急性再生障礙性貧血病因、臨床特點、實驗室檢查及不同治療方法的臨床療效。 方法 回顧性分析1996年1月-2010年11月在鄭州大學第一附屬醫(yī)院兒內(nèi)科住院、行骨髓穿刺術和(或)骨髓活檢術確診的72例急性再障患兒的資料,收集所有患兒的一般情況(年齡、性別等)、臨床資料(起病原因、臨床表現(xiàn)等)、實驗室檢查(外周血象、骨髓細胞學檢查、骨髓活檢、免疫學指標)等,根據(jù)不同的治療方案,分組為1996年1月-2004年12月：Ⅰ組[大劑量甲基強的松龍(HDMP)方案治療組]10例、2005年1月-2010年11月：Ⅱ組[環(huán)孢菌素A(CSA)+雄激素方案治療組]54例和Ⅲ組(CSA+ATG方案治療組)8例。按中華醫(yī)學會兒科分會血液學組.小兒再生障礙性貧血的診療建議的療效評定標準,比較3組間總有效率的差異。 結果 1.一般情況：72例急性再障患兒,男：女比例為1.18：1,平均年齡(10.75±3.26)歲,其中5～10歲占44.4%,68.1%的患兒居住在農(nóng)村。 2.起病原因：72例急性再障患兒,有化學、物理因素接觸史者占6.9%,其中與農(nóng)藥有接觸史者占12.5%,與苯及其衍生物等有接觸史占者8.3%,有放射線接觸史者占1.39%；其中農(nóng)村患兒占75%(12例),城市患兒占25%(4例)有藥物接觸史者占2.8%,其中氯霉素占2.8%驅(qū)蟲藥物占4.2%噻嗪類利尿劑占1.4%；其中城市患兒占66.7%(10例),農(nóng)村患兒占33.3%(5例)。有病毒感染者占36.1%,其中43.1%的患兒感染CBVIgM;城市患兒占60.6%(40例),農(nóng)村患兒占39.4%(6例)。 3.臨床癥狀：貧血者占70.8%,其中以面色蒼白表現(xiàn)者占76.2%；出血者占68.1%,其中皮膚出血者占75.5%,其余部位出血者(如鼻出血者、牙齦出血等)占小部分；感染者占59.4%,其中呼吸道感染者占87.8%,敗血癥占4.2%,腸道感染占2.78%,牙齦炎占1.39%,皮膚蜂窩組織炎占1.39%。出血伴貧血者占54.2%；感染伴有貧血者占6.94%(5例)；感染伴出血者占8.33%；出血、貧血及感染者占13.9%。 4.體格檢查：不同程度貧血貌的患兒占70.8%,有不同程度面色蒼白及口唇、甲床蒼白,其中重度貧血貌者占90.2%；淋巴結腫大者占45.8%；肝臟腫大者占40.3%；心臟可聞及雜音的患兒占2.8%。 5.輔助檢查： (1)血常規(guī)檢查結果示：外周血一系減低者占12.5%,兩系減低者占30.6%,三系減低者占66.7%,其中L計數(shù)平均(3.72±2.18)×109L-1,L百分比平均(72±18)%；Ret計數(shù)平均(19±8)×109L-1,Ret百分比平均(0.30±0.20)%；MCV在正常范圍者占75%,RDW在正常范圍者占93.1%,MCV正常、RDW增高的患兒占75%,MCH在正常范圍者占77.8%,MCHC在正常范圍者占80.6%。 (2)骨髓細胞學檢查結果示：骨髓增生低下者占72.2%,粒紅系比值減低或倒置者占88.9%,淋巴細胞比值可高達80%～90%,87.5%的患兒可見骨髓小粒,其中非造血組織50%,未見巨核細胞者占58.3%。 (3)骨髓活檢結果示：骨髓增生減低者占83.3%,脂肪組織增生活躍者占91.7%,粒系增生減低者占83.3%,紅系增生減低者占91.7%,未見巨核系增生活躍； (4)免疫學指標示：治療前檢測33例患兒T淋巴細胞亞群示CD3+T、NKT、 CD4+T細胞比例降低,CD8+T細胞比例升高,CD4+/CD8+比值下降或倒置；免疫抑制治療隨訪6個月復查結果示CD4+T細胞比例、CD4+/CD8+比值升高；CD8+T細胞比例降低；CD3+T、NKT細胞比例與治療前無明顯變化；其中15例患兒的CD4+T細胞比例、CD4+/CD8+比值、CD8+T細胞比例未恢復到正常范圍內(nèi),繼續(xù)給予免疫抑制治療6個月后僅有4例未恢復到正常范圍。 6.治療：Ⅰ組總有效率高于Ⅱ組(X2=5.806,P0.05)；Ⅲ組總有效率高于Ⅱ組(X2=6.394,P0.05)；Ⅲ組總有效率高于Ⅰ組(X2=6.102,P0.05) 結論 1.急性再障以5-10歲兒童多見,男孩多于女孩。城市患兒因某些藥物服用及病毒感染多見,農(nóng)村患兒因與農(nóng)藥等化學因素接觸多見。 2.急性再障外周血紅細胞以MCV正常、MCH正常、MCHC正常、RDW升高多見即正細胞正色素非均一性貧血為主。 3.急性再障患兒存在T淋巴細胞亞群功能異常,免疫抑制治療可使其恢復。 4. CSA+ATG聯(lián)合方案治療兒童急性再障的療效最佳。
[Abstract]:objective
Objective to investigate the etiology, clinical characteristics, laboratory examination and clinical efficacy of different treatment methods in children with acute aplastic anemia.
Method
A retrospective analysis was made of 72 cases of acute aplastic anemia in the First Affiliated Hospital of Zhengzhou University, January 1996 -2010, which were hospitalized in the First Affiliated Hospital of Zhengzhou University. The data of children with acute aplastic anemia diagnosed by bone marrow aspiration and / or bone marrow biopsy were collected, and the general conditions (age, sex, etc.), clinical data (cause of disease, clinical manifestation, etc.), and laboratory examination (peripheral blood, bone, bone, bone, and bone) were collected. Myeloid cytology, bone marrow biopsy, immunological index, etc., according to different treatments, were divided into group I (HDMP) group]10 in group I [large dose methylprednisolone (HDMP), November: group]54 in group II (cyclosporin A (CSA) + androgen in group II and group III (CSA+ATG regimen group) 8 cases). According to the hematology section of the pediatrics branch of the Chinese Medical Association, the curative effect evaluation criteria for the diagnosis and treatment of aplastic anemia in children were compared. The difference of the total effective rate between the 3 groups was compared.
Result
1. general situation: 72 cases of acute aplastic anemia, male: the female ratio is 1.18:1, the average age (10.75 + 3.26) years old, 5~10 year old 44.4%, 68.1% children live in the countryside.
2. reasons for the onset of disease: 72 cases of acute aplastic anemia, chemical and physical contact history accounted for 6.9%, of which 12.5% were exposed to pesticides, 8.3% with benzene and its derivatives, 1.39% with radiation exposure history, 75% (12 cases) in rural children, 25% (4 cases) in city children (4 cases), and chlorine in 2.8%. Mycophentin accounted for 1.4% of 4.2% thiazine diuretics, accounting for 66.7% (10 cases), 33.3% (5) in rural children and 36.1% in rural children, of which 43.1% of the children were infected with CBVIgM, 60.6% (40) in urban children and 39.4% in rural areas (6 cases).
3. clinical symptoms: anemia accounted for 70.8%, of which 76.2% were pallid, 68.1% of the bleeding, 75.5% of the skin bleeding, and the rest of the bleeding (such as nosebleed, gingival bleeding) accounted for a small portion; the infected persons accounted for 59.4%, the respiratory tract infection accounted for 87.8%, septicemia accounted for 4.2%, intestinal infection 2.78%, gingivitis in 1.39% Cutaneous cellulitis accounted for 54.2% of 1.39%. bleeding accompanied by anemia, 6.94% (5 cases) with anemia and 8.33% with bleeding, hemorrhage, anemia and infection in 13.9%..
4. physical examination: children with different degrees of anemia accounted for 70.8%, with varying degrees of paleness and lip, a bed paleness, with severe anemia in 90.2%, lymph node enlargement in 45.8%, liver enlargement in 40.3%, and 2.8%. in children with heart smelling and murmur.
5. auxiliary examination:
(1) the results of blood routine examination showed that one of the peripheral blood was 12.5%, the two lines were 30.6%, and the three system was 66.7%, of which the L count was (3.72 + 2.18) * 109L-1, the percentage of L was (72 + 18)%, the average of the Ret count (19 +) 109L-1, the average of Ret (0.30 + 0.20)%, the MCV in the normal range, and the RDW in the normal range. 1%, MCV was normal, RDW increased in 75%, MCH in the normal range accounted for 77.8%, MCHC in normal range accounted for 80.6%.
(2) the results of bone marrow cytology showed that the number of myelodysplastic patients was 72.2%, the ratio of the erythroid system decreased or the inversion was 88.9%, the ratio of lymphocyte was as high as 80% to 90%, and 87.5% of the children had bone marrow particles, among which the non hematopoietic tissue was 50%, and no megakaryocyte was found in 58.3%..
(3) the results of bone marrow biopsy showed that 83.3% of those with hypoplastic myelodysplasia, 91.7% in adipose tissue, 83.3% in granulocytic hypoplasia and 91.7% in erythroid hyperplasia, no megakaryocyplasia was not active.
(4) immunology indicated that before treatment, 33 cases of T lymphocyte subsets showed that the proportion of CD3+T, NKT, CD4+T cells decreased, the proportion of CD8+T cells increased, the ratio of CD4+/CD8+ decreased or inverted; the ratio of CD4+T cells, CD4+/CD8+ ratio increased, CD8+T cell ratio decreased, CD3+T, NKT cell ratio was decreased in the follow-up of immunosuppressive therapy for 6 months. The proportion of CD4+T cells, the ratio of CD4+/CD8+, and the proportion of CD8+T cells in 15 cases were not recovered to the normal range, and only 4 cases were not recovered to the normal range after 6 months of immunosuppressive therapy.
6. treatment: the total effective rate of group I was higher than that of group II (X2=5.806, P0.05), and the total effective rate in group III was higher than group II (X2=6.394, P0.05), and the total effective rate in group III was higher than group I (X2=6.102, P0.05).
conclusion
1. children with acute aplastic anemia are more common in 5-10 years of age, more boys than girls. Children in urban areas are often seen because of some drugs and virus infection, and children in rural areas are exposed to chemical factors such as pesticides.
2. the peripheral blood red blood cells of acute aplastic anemia were normal in MCV, normal in MCH, normal in MCHC and increased in RDW.
3. there are abnormal T lymphocyte subsets in children with acute aplastic anemia, and immunosuppressive therapy can restore them.
4. CSA+ATG combined regimen is the best in the treatment of acute aplastic anemia in children.
【學位授予單位】：鄭州大學
【學位級別】：碩士
【學位授予年份】：2012
【分類號】：R725.5

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