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长期甲氨蝶呤治疗银屑病和杂合α1-抗胰蛋白酶缺乏诱导肝纤维化?

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世界核心医学期刊文摘（皮肤病学分册） 2006年第11期2卷 56-56页

作者： Mathew J. Igbokwe U.O. Morley N. Burt A.D. 周少娜 department of Histopathology Royal Cornwall Hospital Truro Cornwall TR1 3LJ United KingdomDr.

Background: Dermatologists, or pathologists, occasionally need to decide whether or not to continue methotrexate therapy in a patient with an identifiable risk factor for liver fibrosis, in this instance heterozygous α1-antitrypsin deficiency. Case Presentation: We relate our experience with an elderly male patient, diagnosed as having α1-antitrypsin deficiency on a liver biopsy, genotypically confirmed as PiMZ. He had been receiving methotrexate for psoriasis for 17 years with a cumulative dose of 7,200 mg. He was monitored by biochemical profiling and interval (10) liver biopsies. Non-specific changes were seen on liver histology although grade 1 liver fibrosis was seen in his last 2 biopsies. Conclusion: We suggest that methotrexate therapy is relatively safe in patients with heterozygous α1-antitrypsin deficiency, with no other risk factor. We however advise that the risk of fibrosis should be monitored and that the patient receives appropriate counselling.

关键词：银屑病抗胰蛋白酶缺乏甲氨蝶呤肝纤维化杂合皮肤科医生肝组织学生化检测肝活检累积剂量

以铂类为基础的联合化疗治疗晚期或复发宫颈非鳞状细胞癌患者的结局

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世界核心医学期刊文摘（妇产科学分册） 2006年第2期2卷 49-49页

作者： Kastritis E. Bamias A. Efstathiou E. 朱晓明 department of Clinical Therapeutics Alexandra Hospital Athens University School of Medicine 31 Komninon Street Haidari 124 62 Athens Greece

Background. Data about the outcome and prognostic factors in the group of patients with non-squamous cell advanced or recurrent carcinomas of the uterine cervix are limited. We compared the outcome of patients with non-squamous with that of squamous cell carcinomas after platinum-based combination chemotherapy as first line therapy for stage IV or recurrent cervical carcinoma. Patients and methods. A total of 200 patients with stage IV or recurrent carcinomas of the cervix received platinum-based combination chemotherapy and were included in our analysis. Results. There were 58 patients with non-squamous and 142 patients with squamous cell carcinomas. Response to chemotherapy was 53.5%in nonsquamous vs. 43.5%in squamous carcinomas. histology was not an independent predictor of tumor response (P = 0.797). Response rates were lower in patients with relapse only in a previously irradiated area in both squamous (26.9%vs 53.5%, P = 0.005) and non-squamous carcinomas (47.1%vs 65%, P = 0.270). Weight loss was the only significant predictor of survival in non-squamous histology patients (P 24.47]) (P = 0.064). After adjustment for independent prognostic factors (ECOG performance status and weight loss), differences in survival remained not significant. Conclusion. Our study showed a similar outcome for both squamous and non-squamous stage IV or recurrent cervical carcinomas treated with platinum-based combination chemotherapy.

关键词：联合化疗宫颈鳞状细胞癌非鳞状细胞癌鳞状细胞宫颈癌化疗有效率预后因子照射区域患者体重

局部应用米奎莫特治疗原发性皮肤CD30^+T细胞淋巴瘤有效

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世界核心医学期刊文摘（皮肤病学分册） 2005年第1期1卷 10-10页

作者： Didona B. Benucci R. Amerio P. 张宪旗 department of Immunodermatology Ist. Dermopat. dellImmacolata IRCCSVia deiMonti di Creta 104 00167 Rome Italy

CD30+anaplastic large cell lymphoma is a primary cutaneous lymphoproliferative disorder with a high rate of spontaneous regression (almost 25%). The suggested therapies are radiation, surgery and methotrexate. We describe two patients with nonregressing primary cutaneousCD30+T cell lymphoma that was successfully treated with topical imiquimod 5%cream (Aldara., 3M) three times weekly for 6 weeks. In both cases we obtained complete clinical remission, confirmed by histology. No recurrences were observed during the following 8 months. We consider that topical application of an immune response modifier, such as imiquimod, could be a good alternative to other potentially more dangerous or aggressive treatments.

关键词： T细胞淋巴瘤 CD30 莫特局部应用组织学检查甲氨蝶呤淋巴细胞增殖免疫调节潜在危险如米

丙型肝炎病毒感染所致肝硬化可被一项标准血清生化学指标排除

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世界核心医学期刊文摘（胃肠病学分册） 2005年第12期1卷 56-56页

作者： Islam S. Antonsson L. Westin J. Lagging M. 樊菁 department of Virology and Infectious Diseases Gteborg University Guldhedsgatan 10B SE- 413 46 Gteborg Sweden Dr

Objective. Assessment of liver histology is pivotal in prognostication and decision- making regarding therapeutic intervention in patients with chronic hepatitis C virus (HCV). Being an invasive procedure, the liver biopsy is associated with complications, and a non- invasive alternative would be preferable. Material and methods. Sera samples from 179 patients with chronic HCV infection collected at the time of liver biopsy were analyzed using routinely available biochemical markers of liver disease, and liver histology was evaluated using the Ishak protocol. The relationship between the serum biochemical markers and cirrhosis (Ishak stage ≥ 5) as well as bridging fibrosis (Ishak stage ≥ 3) was examined. Results. A strong association was found in the multivariate logistic regression analysis between fibrosis stage and aspartate aminotransferase (AST), platelet count and prothrombin- INR (inte- rnational normalized ratio). An index (the G teborg University Cirrhosis Index (GUCI)) was calculated using these variables: normalized AST × prothrombin- INR × 100/ platelet count ( × 109/l). Using a cut- off value of 1.0, the sensitivity was 80% and the specificity 78% for diagnosis of cirrhosis, and the negative predictive values (NPV) and positive predictive values (PPV)- were 97% and 31% , respectively. The GUCI score proved slightly superior for sensitivity, specificity, NPV, PPV, and the area under the receiver operating characteristic (ROC) curve for prediction of cirrhosis and bridging fibrosis compared with the AST to platelet ratio index (APRI), which has been reported as a predictor of significant fibrosis and cirrhosis. Conclusions. An index using routinely available biochemical markers can with a high degree of accuracy discriminate patients with from those without hepatitis C- related cirrhosis.

关键词：生化学指标标准血清桥接纤维化肝活检肝脏组织学慢性丙型肝炎纤维化分期门冬氨酸转氨酶凝血酶原肝纤维化

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表皮松解坏死型游走性红斑

世界核心医学期刊文摘（皮肤病学分册） 2006年第6期2卷 8-8页

作者： Kovcs R.K. Korom I. Dobozy A. 李政霄 department of Dermatology and Allergology Faculty of Medicine University of Szeged Hungary Kornyi fasor 6 H-6720 Szeged Hungary Dr.

Background: Necrolytic migratory erythema is considered to be a paraneoplastic dermatosis. The classical symptoms are associated with α-cell pancreatic islet cell tumor or ‘glucagonoma’. Generally, extracutaneous hallmarks of this disease include weight loss, diabetes, anaemia and diarrhoea. Observation: We report a case of a 39-year-old woman with a 3-year history of recalcitrant psoriasiform eruption, who had no other associated symptoms on routine examination. Histologic examinations suggested necrolytic migratory erythema. Abdominal computer tomography was performed, which revealed a tumor in the tail of the pancreas. After distal resection of the pancreas her skin symptoms resolved in a few days time. histology was consistent with glucagonoma. She is clinically well and symptomless and no signs of metastasis after 4 years. Conclusions: It is infrequent to have only necrolytic migratory erythema, hyperglucagonaemia and islet-cell tumor but no other extracutaneous symptoms in glucagonoma syndrome. To our knowledge, ours is the second such case reported in the literature. Skin symptoms are important, often they are the clue to the diagnosis of glucagonoma syndrome.

关键词：表皮松解坏死型游走性红斑胰高血糖素瘤综合征组织病理检查胰岛细胞瘤副肿瘤性女性患者银屑病样皮损典型症状切除术后

伴少突胶质细胞增生的轻微皮质发育畸形:一种新的额叶癫痫病理类型

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中华神经科杂志 2018年第12期51卷 972-976页

作者：毛晨晖窦万臣卢强周良锐金丽日崔瑞雪任海涛赵燕环冯逢彭斌 Ingmar Blumcke 高晶郭玉璞中国医学科学院北京协和医院神经科 100730 中国医学科学院北京协和医院神经外科 100730 中国医学科学院北京协和医院病理科 100730 中国医学科学院北京协和医院核医学科 100730 中国医学科学院北京协和医院放射科 100730 department ofNeuropathology Erlangen University HospitalEdangen DE一91054Germany(Ingmar Bltimcke)

目的通过对1例病理诊断为伴少突胶质细胞增生的轻微皮质发育畸形的额叶癫痫的报道,分析其临床和病理特征,提高对这一新的癫痫病理诊断的认识。方法收集2016年3月就诊于北京协和医院神经科的1例临床表现为额叶癫痫患者的临床资料,总结其... 详细信息

目的通过对1例病理诊断为伴少突胶质细胞增生的轻微皮质发育畸形的额叶癫痫的报道,分析其临床和病理特征,提高对这一新的癫痫病理诊断的认识。方法收集2016年3月就诊于北京协和医院神经科的1例临床表现为额叶癫痫患者的临床资料,总结其影像学和电生理特征及手术切除后病理形态和免疫组织化学染色特征,并与国际文献报道的相关病例和病理改变进行对比。结果患者女性,16岁,癫痫病程12年,临床表现为发作性愣神伴手部摸索和发声,约10次/d,有时表现为全身强直阵挛发作,予多种药物治疗效果均不佳,MRI提示左侧额叶眶额回皮质白质分界欠清,脑电图提示左额叶起源,行左侧前额叶切除术,病理提示额叶皮质神经元构造正常,部分区域灰白质界限不清,皮质下白质内可见片状少突胶质细胞增生,密度明显高于深部白质,增生区白质内异位神经元明显多于深部白质。免疫组织化学:增生细胞Olig-2(+),Ki-67个别(+),阳性率高于深部白质。术后2年随访患者病情稳定,未见颅内新发病灶。结论年轻患者额叶癫痫可存在经典皮质发育不良以外的特殊病理改变,癫痫的病理改变可能不仅仅局限于皮质,白质病理改变对癫痫发病的作用机制尚有待于进一步研究。

关键词：癫痫额叶少突神经胶质皮质发育畸形

重症肌无力伴胸腺增生及纯红细胞再生障碍性贫血

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世界核心医学期刊文摘（神经病学分册） 2005年第2期1卷 50-50页

作者： Suto Y. Araga S. Sakuma K. 李一明 department of Neurology Institute of Neurological Sciences Fac. Me d. Tottori Univ. 36-1 N. Yonago Japan

A case of myasthenia gravis (MG) with thymus hyperplasia and pure red cell apl asia (PRCA) is reported. A 57-year old woman was diagnosed as having MG and wa s treated with thymectomy 26 years ago. The histology of the resected thymus was thymic lymphoid follicular hyperplasia. She developed rapidly progressive anemi a and a bone marrow examination revealed PRCA. Her hematological results improve d with oral administration of cyclosporine A. Cases of MG, thymoma and PRCA have been reported in the literature. We report the first case of MG without thymoma and PRCA.

关键词：重症肌无力胸腺增生再生障碍性贫血胸腺切除术胸腺瘤胸腺组织淋巴滤泡样增生进行性贫血骨髓检查血液学指标

获得性大疱性表皮松解症及线性IgA大疱病混和性大疱病1例

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世界核心医学期刊文摘（皮肤病学分册） 2005年第11期1卷 46-46页

作者： Osawa M. Demitsu T. Toda S. 焦婷 Dr. department of Dermatology Jichi Medical School Omiya Medical Center 1-847 Amanuma-machi Omiyaku Saitama 330-8503 Japan

A 75-year-old Japanese male visited us with bullous eruptions on the extremities. Physical examination revealed large bullae on the hands, lower legs and feet. The oral mucosa was also involved. histology disclosed subepidermal blister with inflammatory cell infiltrates in the dermis. Direct immunofluorescence showed deposits of IgG and IgA at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient’s IgG antibodies reacted with the dermal side of the split, while IgA antibodies reacted with the epidermal side. Immunoblotting showed that the patient’s serum reacted with the NC1 domain of type VII collagen (290-kDa epidermolysis bullosa acquisita antigen) as well as the 120-kDa linear IgA bullous dermatosis antigen, LAD-1. Systemic prednisolone resulted in a favorable response. From the clinicopathological findings, the present case is not consistent with either epidermolysis bullosa acquisita or IgA bullous dermatosis. Therefore,we regarded the case as mixed bullous disease of epidermolysis bullosa acquisita and linear IgA bullous dermatosis. Such a case has not been previously reported.

关键词： IgA大疱病大疱基底膜带直接免疫荧光组织学检查炎细胞浸润免疫印迹法口腔黏膜临床病理学体格检查

甲硝唑和熊脱氧胆酸治疗原发性硬化性胆管炎的随机安慰剂对照试验

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世界核心医学期刊文摘（胃肠病学分册） 2005年第5期1卷 33-33页

作者： FrkkilM. Karvonen A.-L. Nurmi H. 李翔 Division of Gastroenterology Department of Medicine Helsinki University Central Hospital P. O. Box 340 00029 HYKS Finland Dr.

No effective medical therapy is currently available for primary sclerosing cholangitis (PSC). Ursodeoxycholic acid (UDCA)improves liver enzymes, but its effect on liver histology is controversial. Metronidazole (MTZ) prevents PSC-like liver damage in animal models and reduces intestinal permeability. We recruited 80 patients with PSC into a randomized placebo-controlled study to evaluate the effect of UDCA and MTZ (UDCA/MTZ) compared with UDCA/placebo on the progression of PSC. Patients (41 UDCA/placebo and 39 UDCA/ MTZ) were followed every third month. Assessment of liver function test, histological stage and grade, and cholangiography (via ERCP) at baseline showed no differences between the groups. After 36 months, serum aminotransferases γ-glutamyltransferase, and alkaline phosphatase (ALP) decreased markedly in both groups, serum ALP more significantly in the UDCA/MTZ group (-337 ±54 U/L, P histology or ERCP was seen. Long-term studies using a higher dose of UDCA combined with MTZ in larger patient populations are indicated.

关键词：熊脱氧胆酸安慰剂对照肠道渗透性肝脏组织学肝脏损伤联合用药动物模型肝功能检测基线水平血清转氨酶