本文選題：皮肌炎 + 寡肌病性　； 參考：《臨床兒科雜志》2017年06期

【摘要】：目的探討托珠單抗對(duì)兒童寡肌病性皮肌炎合并肺間質(zhì)病變的療效。方法回顧分析2例寡肌病性皮肌炎合并肺間質(zhì)病變患兒的臨床特點(diǎn)及治療與預(yù)后,并復(fù)習(xí)相關(guān)文獻(xiàn)。結(jié)果男女各1例,女性患兒10歲11個(gè)月、男性患兒8歲5個(gè)月,起病時(shí)均有氣促,但無(wú)肌肉損害的臨床表現(xiàn);均有典型皮疹,但肌力及肌張力正常。實(shí)驗(yàn)室檢查以血清鐵蛋白、乳酸脫氫酶、谷氨酸氨基轉(zhuǎn)移酶及天冬氨酸氨基轉(zhuǎn)移酶升高為主,肌酸肌酶除首次入院時(shí)稍高,復(fù)查始終在正常范圍。高分辨CT示肺間質(zhì)病變。臨床診斷為寡肌病性皮肌炎合并肺間質(zhì)病變。女性患兒經(jīng)大劑量激素、環(huán)磷酰胺、環(huán)孢素、吡菲尼酮及丙種球蛋白等治療無(wú)效死亡。男性患兒在常規(guī)激素治療的基礎(chǔ)上,加用托珠單抗(240 mg/次,2次),病情穩(wěn)定,隨訪復(fù)查各指標(biāo)均在正常范圍內(nèi)。結(jié)論兒童寡肌病性皮肌炎臨床表現(xiàn)以及實(shí)驗(yàn)室檢查結(jié)果不典型,死亡發(fā)生率高。聯(lián)合托珠單抗治療有效。
[Abstract]:Objective to investigate the effect of tozhu McAb on children with olivary dermatomyositis complicated with pulmonary interstitial disease. Methods the clinical features, treatment and prognosis of 2 cases of olivary dermatomyositis complicated with pulmonary interstitial disease were retrospectively analyzed. Results there were 1 case of male and female, 10 years and 11 months of female, 8 years and 5 months of male. All of them had shortness of breath but no muscle damage, and they all had typical rash, but the muscle strength and muscle tension were normal. Serum ferritin, lactate dehydrogenase, glutamate aminotransferase and aspartate aminotransferase were mainly increased in laboratory examination. High-resolution CT showed pulmonary interstitial lesions. Oligomyosis dermatomyositis with pulmonary interstitial disease was clinically diagnosed. Female children died after high dose of hormone, cyclophosphamide, cyclosporine, pifenidone and gamma globulin. On the basis of routine hormone therapy, male children were treated with tricuspid antibody 240 mg/ twice, the condition was stable, and every index of follow-up was within normal range. Conclusion the clinical manifestations and laboratory findings of children with oligomyopathy dermatomyositis are atypical and the incidence of death is high. Combined with tozumab is effective.
【作者單位】： 蘇州大學(xué)附屬兒童醫(yī)院;
【基金】：國(guó)家自然科學(xué)基金資助項(xiàng)目(No.81370787) 江蘇省臨床醫(yī)學(xué)科技專項(xiàng)——新型臨床診療技術(shù)攻關(guān)(No.SBL 2014030237)
【分類號(hào)】：R725.9

【相似文獻(xiàn)】

相關(guān)期刊論文 前10條

1 韓秀萍;翟寧;王巖;;成人皮肌炎與兒童皮肌炎對(duì)比分析[J];中國(guó)實(shí)用內(nèi)科雜志;2006年21期

2 侯素春;史航;劉曉明;;皮肌炎合并甲狀旁腺機(jī)能減退癥1例[J];中國(guó)皮膚性病學(xué)雜志;2006年12期

3 鄭文軍;嚴(yán)煜林;韋高;梁伶;溫斯健;劉慧;;兒童皮肌炎與成人皮肌炎臨床對(duì)比分析[J];廣西醫(yī)科大學(xué)學(xué)報(bào);2008年06期

4 董俊華,孫諾鵬,張巖;小兒皮肌炎24例臨床分析[J];臨床兒科雜志;1993年06期

5 俞寶田,魏珉;小兒皮肌炎臨床分析[J];臨床皮膚科雜志;1997年05期

6 盧燕,馬松春,何曉琥;小兒皮肌炎的預(yù)后及隨訪[J];中華風(fēng)濕病學(xué)雜志;1999年02期

7 霍耀寧,范雪莉,王金海,劉玉峰;12例小兒皮肌炎臨床分析[J];嶺南皮膚性病科雜志;1999年01期

8 董莉,曹國(guó)強(qiáng),杜凱音;小兒皮肌炎誤診1例[J];海軍醫(yī)學(xué)雜志;2003年03期

9 鄒敏書,余健,何威遜;皮肌炎腎臟損害2例報(bào)告[J];西南國(guó)防醫(yī)藥;2005年05期

10 冉霓,陳志紅,王淑美,張秋業(yè);小兒皮肌炎1例[J];實(shí)用兒科臨床雜志;1994年06期

相關(guān)會(huì)議論文 前2條

1 丁從珠;;兒童發(fā)病型皮肌炎臨床分析[A];全國(guó)自身免疫性疾病專題研討會(huì)暨第十一次全國(guó)風(fēng)濕病學(xué)學(xué)術(shù)年會(huì)論文匯編[C];2006年

2 程月梅;;鄭啟仲治愈皮肌炎1例報(bào)告[A];第24屆全國(guó)中醫(yī)兒科學(xué)術(shù)研討會(huì)、中醫(yī)藥高等教育兒科教學(xué)研討會(huì)、兒科名中醫(yī)講習(xí)班論文匯編[C];2007年

相關(guān)碩士學(xué)位論文 前1條

1 許諾;三種類型幼年皮肌炎臨床特點(diǎn)及預(yù)后分析[D];重慶醫(yī)科大學(xué);2015年

，

本文編號(hào)：1795548