本文選題：馬爾尼菲青霉病 + 兒童�。� 參考：《廣西醫(yī)科大學(xué)》2013年碩士論文

【摘要】：目的：分析兒童馬爾尼菲青霉病(PSM)的臨床特征,探討其治療。 方法：對從2003年1月至2012年12月入住廣西醫(yī)科大學(xué)第一附屬醫(yī)院確診的6例年齡14歲PSM患者的臨床資料進(jìn)行回顧性分析,并結(jié)合國內(nèi)外文獻(xiàn)討論。 結(jié)果：6例均為男性,平均年齡3歲4月(1歲2月,9歲)。病程39天(15天、120天)。2例為人類免疫缺陷病毒(HIV)陽性；4例HIV陰性,其中1例存在G6PD缺乏癥,3例患兒發(fā)病前體弱多病。所有患者都有貧血、消瘦、淋巴結(jié)腫大,5例發(fā)熱,3例出現(xiàn)呼吸系統(tǒng)癥狀、肝腫大,1例脾腫大,1例中樞神經(jīng)系統(tǒng)受累,1例黑便;2例HIV(+)的PSM患兒出現(xiàn)皮疹。2例HIV(+)患兒的PSM患兒白細(xì)胞降低,2例HIV(-)患兒的PSM患者白細(xì)胞升高,1例降低；共有4例(4/6)出現(xiàn)淋巴細(xì)胞下降。所有病例存在淋巴細(xì)胞功能的異常。4例胸部影像學(xué)提示異常,2例提示縱膈、肺門淋巴結(jié)腫大,2例提示兩肺多發(fā)的團(tuán)塊狀密度增高影。病原學(xué)檢查淋巴結(jié)病3/3(100.00%),骨髓4/5(80.00%),血4/6(66.67%),肺泡灌洗液1／1(100.00%)：所以病人均有兩個部位的病原學(xué)陽性。病理特征主要為肉芽腫、壞死性表現(xiàn)。6例中3例未接受抗真菌治療,2例死亡,1例放棄治療出院；余3例分別以氟康唑、兩性霉素B、伏立康唑抗真菌治療后好轉(zhuǎn)出院。 結(jié)論：1、兒童馬爾尼菲青霉病患者,多見HIV感染、存在其他基礎(chǔ)疾病或存在潛在免疫力低下的患兒。2、患兒常常表現(xiàn)為全身播散性感染,可累及肺、淋巴結(jié)、肝、脾、血液、中樞神經(jīng)系統(tǒng)等多個器官及系統(tǒng)。常見發(fā)熱、貧血、淋巴結(jié)、肝脾腫大等臨床表現(xiàn),皮下膿腫、溶骨性等全身化膿性改變少見。3、相對成人,患兒較容易取得病原學(xué)依據(jù),血、淋巴結(jié)、骨髓病原學(xué)陽性率較高。4、病理檢查主要表現(xiàn)為肉芽腫及壞死性改變,化膿性改變少見。5、未抗真菌治療,死亡率高,預(yù)后很差；及時的抗真菌治療,治療效果好。
[Abstract]:Objective: to analyze the clinical features of children with penicilliosis marneffei (PSM) and to explore its treatment. Methods: the clinical data of 6 patients with PSM aged 14 years who were admitted to the first affiliated Hospital of Guangxi Medical University from January 2003 to December 2012 were retrospectively analyzed and discussed in the literature at home and abroad. Results all of the 6 cases were male, with an average age of 3 years, 4 months, 1 year, 2 months and 9 years old. The course of disease was 39 days, 15 days and 120 days. 2 cases were HIV negative in 4 cases. Among them, 1 case had G6PD deficiency and 3 cases had weak disease before onset. All patients had anemia, wasting, lymphadenopathy, 5 cases of fever and 3 cases of respiratory symptoms. Hepatomegaly 1 patient with splenomegaly 1 case with central nervous system involvement 1 case with melanoma 2 cases with PSM and 2 cases with PSM with leukopenia 2 cases with PSM increased leukocyte in 1 case; A total of 4 cases (4 / 6) had lymphocytopenia. All cases had abnormal lymphocyte function in 4 cases. Chest imaging showed abnormal mediastinum in 2 cases, enlarged hilar lymph nodes in 2 cases, and increased mass density in both lungs in 2 cases. Etiology examination of lymphadenopathy 3 / 3 / 100.00000, bone marrow 4 / 5 / 80.000, blood 4 / 6, 66.67, alveolar lavage fluid 1 / 1 / 100.00000: so there are two sites of etiology positive in patients. The main pathological features were granuloma. Among the 6 cases, 3 cases were not treated with antifungal therapy, 2 cases died and 1 case was discharged from hospital, the remaining 3 cases were discharged after antifungal therapy with fluconazole, amphotericin B and voleconazole respectively. Conclusion in children with penicilliosis marneffei, HIV infection is more common, other underlying diseases or potential immunosuppression are found in children. The children often present with disseminated systemic infection, which can involve lung, lymph nodes, liver, spleen, blood. Central nervous system and other organs and systems. Common fever, anemia, lymph node, hepatosplenomegaly and other clinical manifestations, subcutaneous abscess, osteolytic and other systemic suppurative changes rare. The positive rate of bone marrow etiology was high. 4. The pathological examination mainly showed granuloma and necrotic change. The suppurative change was rare. 5. No antifungal treatment, high mortality, poor prognosis, timely antifungal treatment, good therapeutic effect.
【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】：2013
【分類號】：R725.1

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