發(fā)布時(shí)間：2018-05-03 05:20

  本文選題：朗格漢斯細(xì)胞組織細(xì)胞增生癥 + 兒童　； 參考：《重慶醫(yī)科大學(xué)》2016年碩士論文

【摘要】：目的:探討兒童朗格漢斯細(xì)胞組織細(xì)胞增生癥(Langerhans Cell Histiocytosis,LCH)的臨床以及肺部影像表現(xiàn)特點(diǎn),分析其臨床與肺部影像表現(xiàn)的關(guān)系,提高對(duì)該病診斷能力。方法:回顧性分析重慶醫(yī)科大學(xué)附屬兒童醫(yī)院2008年1月~2015年12月確診為L(zhǎng)CH患兒140例,男79例,女61例,發(fā)病年齡0~15.4歲。根據(jù)臨床表現(xiàn)和疾病浸潤(rùn)范圍,將LCH分為單系統(tǒng)LCH(Single-System Langerhans Cell Histiocytosis,SS-LCH)和多系統(tǒng)LCH(Multi-System Langerhans Cell Histiocytosis,MS-LCH)兩組,根據(jù)肺部有無(wú)受累,將MS-LCH分為肺朗格漢斯細(xì)胞組織細(xì)胞增生癥(Pulmonary Langerhans Cell Histiocytosis,PLCH)和未累及肺部MS-LCH兩組;臨床根據(jù)年齡、受浸潤(rùn)器官數(shù)目以及危險(xiǎn)器官有無(wú)功能受累三大因素將該病分為4級(jí),分別分析各組的臨床及肺部CT表現(xiàn)特點(diǎn),比較臨床分級(jí)與肺部CT表現(xiàn)的相關(guān)性。結(jié)果:1.140例患兒中SS-LCH64例,48例(75%)2歲發(fā)病,58例(90.6%)表現(xiàn)為骨骼系統(tǒng)的單獨(dú)受累;MS-LCH76例,46例(60.5%)≤2歲發(fā)病,各器官均可受累,以肝臟(59/76,77.6%)、皮膚(57/76,75.0%)、脾臟(49/76,64.5%)受累常見。MS-LCH中PLCH33例,皮膚受累29例(87.9%),未累及肺部43例,皮膚受累28例(65.1%),兩組患兒皮膚受累差異具有統(tǒng)計(jì)學(xué)意義(P0.05)。2.SS-LCH臨床分級(jí)Ⅰ~Ⅳ級(jí)的比例分別為25.0%、75.0%、0.0%、0.0%,以Ⅰ、Ⅱ級(jí)為主,MS-LCHⅠ~Ⅳ級(jí)的比例分別為13.2%、19.3%、30.3%、36.8%,以Ⅲ、Ⅳ級(jí)多見,兩組差異有統(tǒng)計(jì)學(xué)意義(P0.001)。PLCH臨床分級(jí)Ⅰ~Ⅳ級(jí)的比例分別為20.9%、18.6%、27.9%、32.6%,未累及肺部的MS-LCH臨床分級(jí)Ⅰ~Ⅳ級(jí)的比例分別為3.0%、21.2%、33.3%、42.4%,兩組均以Ⅲ、Ⅳ級(jí)多見,差異無(wú)統(tǒng)計(jì)學(xué)意義(P0.05)。3.33例PLCH中,肺部CT表現(xiàn)(1)肺間質(zhì)病變30例(90.9%),以肺透光度減低(21/30,70.0%)、“磨玻璃”影(12/30,40.0%)、細(xì)網(wǎng)格狀影(11/30,36.7%)表現(xiàn)為主,21例肺透光度減低、7例“磨玻璃”影、7例細(xì)網(wǎng)格狀影呈雙肺廣泛分布,8例肺透光度減低、2例“磨玻璃”影以雙肺后部明顯。(2)肺部結(jié)節(jié)影和(或)氣囊影16例(48.5%),其中結(jié)節(jié)影10例,氣囊影9例;3例結(jié)節(jié)影、1例氣囊影呈孤立分布,8例結(jié)節(jié)影和(或)氣囊影呈多個(gè)散在分布,4例呈彌漫分布;孤立或多個(gè)散在結(jié)節(jié)影、氣囊影以胸膜下分布(8/12,66.7%)為主,彌漫性結(jié)節(jié)影和(或)氣囊影以雙肺上葉及外、中帶明顯。(3)肺纖維化2例(6.1%)。4.肺部CT表現(xiàn)為肺間質(zhì)病變的患兒中位發(fā)病年齡為1.2歲,病程中位數(shù)為2.5月,結(jié)節(jié)影和(或)氣囊影的患兒中位發(fā)病年齡為0.9歲,病程中位數(shù)為4月,兩組發(fā)病年齡差異無(wú)統(tǒng)計(jì)學(xué)意義(P0.05),兩組病程長(zhǎng)短差異有統(tǒng)計(jì)學(xué)意義(P=0.000)。5.33例PLCH中臨床分級(jí)Ⅰ~Ⅳ級(jí)的結(jié)節(jié)影和(或)氣囊影檢出率分別為0.0%、57.1%、63.6%、35.7%,各臨床分級(jí)檢出率差異無(wú)統(tǒng)計(jì)學(xué)意義(P0.05)。結(jié)節(jié)影和(或)氣囊影數(shù)量≤10個(gè)的患兒臨床分級(jí)以Ⅱ級(jí)(3/16,18.8%)、Ⅲ級(jí)(3/16,18.8%)多見,病變數(shù)量10個(gè)的患兒臨床分級(jí)以Ⅲ級(jí)(4/16,25.0%)、Ⅳ級(jí)(3/16,18.8%)多見,兩組比較差異不顯著(P0.05)。肺間質(zhì)病變?cè)谂R床分級(jí)Ⅰ~Ⅳ級(jí)中均有分布,Ⅲ、Ⅳ級(jí)分布較多,結(jié)節(jié)影和(或)氣囊影分布為Ⅱ~Ⅳ級(jí),肺纖維化分布Ⅲ、Ⅳ級(jí)各1例,肺部CT表現(xiàn)的嚴(yán)重程度與臨床分級(jí)的差異無(wú)統(tǒng)計(jì)學(xué)意義(P0.05)。結(jié)論:1.SS-LCH常2歲發(fā)病,以骨骼單獨(dú)受累多見,臨床分級(jí)以Ⅰ、Ⅱ級(jí)為主;MS-LCH多≤2歲發(fā)病,各器官均可受累,以肝臟、皮膚、脾臟受累多見,臨床分級(jí)以Ⅲ、Ⅳ多見;PLCH均為MS-LCH,常伴有皮膚受累;2.PLCH肺部CT表現(xiàn)以肺間質(zhì)病變多見,常見肺透光度減低、“磨玻璃”影、細(xì)網(wǎng)格狀影,具有雙肺廣泛分布,且肺后部病變表現(xiàn)明顯的特點(diǎn);3.肺部結(jié)節(jié)影、氣囊影是PLCH的特征性表現(xiàn),病變可孤立、散在或彌漫分布;孤立或散在分布結(jié)節(jié)影、氣囊影以胸膜下多見,彌漫性結(jié)節(jié)影和(或)氣囊影以雙肺上葉及外、中帶為著;4.PLCH肺部CT間質(zhì)病變表現(xiàn)早于結(jié)節(jié)影和(或)氣囊影;5.肺部CT結(jié)節(jié)影和(或)氣囊影的檢出率及數(shù)量不隨臨床分級(jí)的增高而增多;PLCH肺部CT表現(xiàn)不隨著臨床分級(jí)的增高而加重。
[Abstract]:Objective: To explore the clinical and pulmonary imaging features of Langerhans Cell Histiocytosis (LCH) in children's Langerhans cell, and to analyze the relationship between the clinical and pulmonary imaging features, and to improve the diagnostic ability of the disease. Methods: a retrospective analysis of the Affiliated Children's Hospital of Medical University Of Chongqing was diagnosed as L in December, January 2008. 140 children with CH, 79 men and 61 women, 0~15.4 years of age. According to the clinical manifestations and the range of disease, the LCH was divided into two groups of single system LCH (Single-System Langerhans Cell Histiocytosis, SS-LCH) and multisystem LCH (Multi-System Langerhans). The Pulmonary Langerhans Cell Histiocytosis (PLCH) and the lung MS-LCH two groups were not involved. The clinical and pulmonary CT manifestations of each group were analyzed according to age, the number of infiltrating organs and the three major factors of the dangerous organ involvement. The clinical and pulmonary CT features were analyzed, and the clinical classification and lung CT performance were compared. Results: 1.140 cases of SS-LCH64, 48 cases (75%) 2 years of age, 58 cases (90.6%) of individual involvement in the skeletal system; MS-LCH76 cases, 46 cases (60.5%) less than 2 years of age, all organs can be involved, the liver (59/76,77.6%), skin (57/76,75.0%), spleen (49/76,64.5%) involvement in common.MS-LCH in PLCH33 cases, skin involvement 29 cases (87.9%), There were no pulmonary involvement in 43 cases and 28 cases of skin involvement (65.1%). The difference in skin involvement in the two groups was statistically significant (P0.05) the proportion of.2.SS-LCH clinical grade I to IV was 25%, 75%, 0%, and 0%, and the proportion of grade I to grade II was 13.2%, 19.3%, 30.3%, 36.8%, respectively, with grade III and IV, and there were statistical differences between the two groups. The proportion of P0.001.PLCH clinical grade I to grade I ~ IV were 20.9%, 18.6%, 27.9%, 32.6%, and the proportion of MS-LCH clinical grade I to IV of the lung was 3%, 21.2%, 33.3%, 42.4%, and two groups were all more common in class III and IV (P0.05).3.33 case PLCH, pulmonary CT manifestation (1) pulmonary interstitial lesions 30 cases (90.9%), lung (1), lung Light transmittance decreased (21/30,70.0%), "glass" shadow (12/30,40.0%), fine mesh shadow (11/30,36.7%), 21 cases of lung light transmittance decreased, 7 cases of "grinding glass" shadow, 7 cases of fine gridding in double lung widely distributed, 8 cases of lung permeability reduction, 2 cases of "glass" shadow in the posterior part of the lungs. (2) 16 cases of pulmonary nodules and (or) airbag shadow (4) 8.5%) among them, there were 10 cases of nodules, 9 cases with air bag shadow, 3 cases of nodule shadow, 1 cases with solitary airbag shadow, 8 cases of nodular shadow and (or) airbag shadow scattered in distribution, 4 cases diffuse distribution, solitary or multiple scattered in the nodule shadow, air bag shadow distribution (8/12,66.7%) as the main, diffuse nodule shadow and (or) air bag shadow in the upper and outer, middle band of double lung (3) 2 cases of pulmonary fibrosis (6.1%).4. pulmonary CT manifestations of pulmonary interstitial lesions in children with a median age of 1.2 years, a median of 2.5 months in the course of disease, 0.9 years of age in children with nodular shadow and (or) air bag shadow, the median of the course of disease in April, and the difference between the two groups of age differences (P0.05), and the difference between the two groups in the duration of the disease. The clinical classification of grade I ~ IV in.5.33 PLCH was 0%, 57.1%, 63.6%, 35.7%, respectively, and there was no significant difference between the clinical classification detection rates (P0.05). The number of patients with nodular shadow and / or air bag shadow was less than 10 (3/16,18.8%), grade III (3/16,18.8%), and the number of lesions was 1. The clinical grades of 0 children were grade III (4/16,25.0%) and grade IV (3/16,18.8%), and the difference between the two groups was not significant (P0.05). The pulmonary interstitial lesions were distributed in grade I to IV, the distribution of grade III and IV was more, the distribution of nodules and (or) airbags was grade II to IV, the distribution of pulmonary fibrosis in 1 cases, and the severity of CT in the lung. There was no statistically significant difference from clinical classification (P0.05). Conclusion: 1.SS-LCH often occurs at 2 years of age and is mostly involved in bone involvement. The clinical classification is mainly in grade I and II; MS-LCH is more than 2 years old. All organs can be involved with liver, skin and spleen, and the clinical classification is III and IV; PLCH is MS-LCH, often accompanied by skin involvement; 2.PLCH lung. CT manifestations were common in the pulmonary interstitial lesions, common pulmonary transmittance reduction, "grind glass" shadow, fine gridding shadow, with a wide distribution of two lungs, and obvious characteristics of the posterior lung lesions; 3. pulmonary nodules, and airbag shadows were characteristic manifestations of PLCH, isolated and diffuse distribution; isolated or scattered in the distribution of nodules, airbag shadow to chest The presence of diffuse nodules and (or) airbag shadows in the upper and outer areas of the lungs was more common. The 4.PLCH pulmonary CT interstitial lesions were earlier than the nodules and (or) airbag shadows; the detection rates and numbers of CT nodules and (or) airbags in the lungs were not increased with the increase of clinical classification; the CT manifestations of the PLCH lung did not increase with the increase of clinical classification.

【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】：2016
【分類號(hào)】：R725

【參考文獻(xiàn)】

相關(guān)期刊論文 前10條

1 袁新宇;李素榮;;兒童肺部郎格罕細(xì)胞組織增生癥病理與影像學(xué)等診斷研究進(jìn)展[J];武警醫(yī)學(xué);2015年06期

2 吳鵬;方擁軍;何璐璐;芮耀耀;周莉;黃婕;王亞萍;;兒童郎格罕斯細(xì)胞組織細(xì)胞增生癥83例臨床分析[J];南京醫(yī)科大學(xué)學(xué)報(bào)(自然科學(xué)版);2015年01期

3 唐曉蕾;王維;劉金榮;楊海明;趙順英;李惠民;;兒童肺受累的朗格罕細(xì)胞組織細(xì)胞增生癥病例分析[J];中華兒科雜志;2014年12期

4 高怡瑾;;朗格罕斯細(xì)胞組織細(xì)胞增生癥現(xiàn)代臨床研究進(jìn)展[J];中國(guó)小兒血液與腫瘤雜志;2014年05期

5 胡培安;施鶯燕;帕米爾;李國(guó)平;呂蕓;喬中偉;;兒童朗格漢斯組織細(xì)胞增生癥侵犯肋骨的X線與CT比較[J];腫瘤影像學(xué);2014年02期

6 黃霞;李丹;趙德育;;兒童肺朗格漢斯細(xì)胞組織細(xì)胞增生癥4例誤診分析[J];江蘇醫(yī)藥;2014年08期

7 張玲;胡桂周;唐浩;胡碧瑩;陳衛(wèi)國(guó);;骨朗格漢斯組織細(xì)胞增生癥的影像學(xué)分析[J];放射學(xué)實(shí)踐;2013年08期

8 王利濤;蘇玉文;張桂英;廖智靈;張瑤;肖嶸;文海泉;陸前進(jìn);;朗格漢斯細(xì)胞組織細(xì)胞增生癥伴發(fā)急性呼吸窘迫綜合征1例[J];中國(guó)皮膚性病學(xué)雜志;2013年08期

9 管波青;吳啟秋;林羽;劉書茂;;脊椎朗格漢斯組織細(xì)胞增生癥13例報(bào)告[J];中國(guó)骨與關(guān)節(jié)外科;2013年02期

10 張穎;儲(chǔ)金秀;李光民;王俊卓;才長(zhǎng)智;齊福新;;小兒朗格漢斯細(xì)胞組織細(xì)胞增生癥胸部影像診斷分析[J];中國(guó)輻射衛(wèi)生;2013年01期

相關(guān)碩士學(xué)位論文 前1條

1 龍泉先;120例朗格漢斯細(xì)胞組織細(xì)胞增生癥患者臨床特征及診療分析[D];廣西醫(yī)科大學(xué);2014年

，

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