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find Author "谢玲俐" 5 results
  • 以乳糜胸作为首发表现的原发性系统性淀粉样变性一例并文献复习

    目的探讨原发性系统性淀粉样变性的临床表现、诊断、治疗及预后,提高对累及胸膜的原发性系统性淀粉样变性的临床认识。方法报道四川大学华西医院收治的 1 例以乳糜胸作为首发表现的原发性系统性淀粉样变性患者,回顾性分析国内外文献。以“Amyloidosis,pleura”为检索词,在 PubMed 数据库检索,检索时间为 1968 年 12 月至 2019 年 9 月,共检测到相关英文文献 57 篇,报道 18 例患者。以“淀粉样变,胸膜”为检索词,在中国期刊网全文数据库检索,检索时间为 1981 年 3 月至 2019 年 9 月,共检索到相关中文文献 12 篇,报道 7 例患者。结果58 岁男性患者,临床表现为间断性背痛。查体:胸廓对称,双侧肩胛下角第 7 肋间各有一引流管,引流出乳白色液体。双下肺叩诊呈浊音,双下肺呼吸音低至消失,心脏及腹部查体未见异常。血清免疫固定电泳示可疑 IgA κ 型 M 蛋白条带,血清游离 κ 轻链检测 745 mg/L(参考值 6.7~22.4 mg/L)。胸部 CT 示双侧胸腔积液,双肺门淋巴结增大,腹部增强 CT 示胃壁广泛增厚伴腹腔、腹膜后广泛淋巴结增大,经多次胃镜行胃黏膜组织活检,胃组织刚果红染色阳性,胸腔积液流式细胞学见克隆性浆细胞,骨髓流式细胞学见 4% 克隆性浆细胞,确诊为原发性系统性淀粉样变性。在上述数据库中共检索 69 篇文献,经筛选除外资料不详后,有 18 篇文献 20 例患者明确诊断为胸膜淀粉样变性,符合检索条件。此类患者临床表现及影像学均无特异性,确诊依据胸膜活检刚果红染色阳性或胸腔积液查见淀粉样蛋白。结论原发性系统性淀粉样变性累及胸膜,临床表现及影像学缺乏特征性,与肿瘤胸膜转移、胸膜间皮瘤等疾病易混淆,但多数患者血清中会出现单克隆性免疫球蛋白增多,其诊断主要依靠活检组织病理学检查。治疗方案仍以化疗为主。

    Release date:2020-11-24 05:41 Export PDF Favorites Scan
  • 遗传性抗凝血酶缺乏症一例并文献复习

    目的 加强临床医生对遗传性抗凝血酶缺乏症的认识,促进该病的早期诊断,改善患者预后。方法总结了1例罕见的遗传性抗凝血酶缺乏症患者的临床特点及诊治经过,并以 “遗传性抗凝血酶缺乏症”“临床特征”“达比加群酯”为关键词检索万方中文数据库,以“Hereditary antithrombin deficiency”“clinical characteristics ”“dabigatran etexilate ”为关键词检索PubMed数据库予以文献复习。结果该患者青年时期即发现非寻常部位血栓(颅内静脉窦),本次就诊期间发现多部位血栓形成,低分子肝素抗凝效果不佳,多次查抗凝血酶Ⅲ明显低于正常,进一步行基因全外显子组测序提示存在遗传性抗凝血酶Ⅲ缺乏症相关的SERPINC1基因致病变异,明确诊断为遗传性抗凝血酶缺乏症后予以达比加群酯抗凝治疗好转,随访至今未再复发。检索相关文献发现,遗传性抗凝血酶缺乏症临床相对罕见,该病具有不同的临床和基因分型,诊断的建立需依据相应的流程,其治疗及预后尚缺乏统一的认识。结论遗传性抗凝血酶缺乏症系静脉系统血栓的罕见病因,对于年轻血栓形成患者、复发性血栓或有家族聚集倾向的血栓患者,需警惕此病的可能;通过对该病的早期诊断、早期合理使用抗凝药物有助于改善预后。

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  • A case of hypersensitivity pneumonitis presenting as progressive fibrotic interstitial lung disease and literature review

    ObjectiveTo strengthen the understanding to hypersensitivity pneumonitis and make early diagnosis and standard treatment by analyzing the clinical features, the diagnosis and treatment of a patient diagnosed as hypersensitivity pneumonitis presenting as progressive fibrotic interstitial lung disease in combination with literature review.MethodsThe diagnosis and treatment process and relevant clinical data of the patient were analyzed retrospectively and literatures were reviewed. Based on 282 relevant literatures, the diagnostic methods, treatment and prognostic factors of hypersensitivity pneumonitis were summarized.ResultsThe patient, female, 45 years old, self-employed, was admitted to the hospital due to "cough, sputum for 9 years, dyspnea for more than 6 months" without obvious extrapulmonary clinical manifestations. Creaks at the bottom of the lungs and clubbed toes were found through physical examination. High-resolution computed tomography indicated that the main manifestations were ground glass and grid-like shadows, presenting characteristics of interstitial pneumonia. The pulmonary lesions aggravated gradually, and part of the lung lobe presented honeycomb lung at the time of diagnosis. Eight years ago, she performed fibrobronchoscopy in other hospital revealing an increased lymphocyte proportion (39%) in bronchoalveolar lavage fluid. Lung function suggested very severe restrictive ventilation dysfunction. She was given prednisone for a short time, and the drug was stopped by herself with uncertain efficacy. Later, she performed frozen lung biopsy suggesting peribronchiolar metaplasia interstitial broadening with lymphocytic infiltration, without granuloma or fibroblast lesions. No obvious abnormity was seen in makers of infections and immunology. At this point, the patient was clearly diagnosed as hypersensitivity pneumonitis. Meanwhile, the patient's pulmonary lesions were still mainly made of ground glass and plaques, partly with changes like honeycomb. Poor response was obtained with anti-inflammatory treatment of prednisone for 3 months and anti-fibrosis treatment of pirfenidon for more than 2 months. Literature review resulted in 87 patients, including 39 males (44.82%) and 48 females (55.18%), with an average age of (47.0±18.4) years. Seventy patients (80.46%) had clear allergens. 3.45% patients' lymphocyte percentage in bronchoalveolar lavage fluid ranged from 20% to 39%, and 19.54% patients' lymphocyte percentage was more than 40%. 4.60% of the patients achieved remission through detachment from allergens; 71.27% of the patients achieved remission with glucocorticoid treatment, 14.94% improved with glucocorticoid treatment, and 1.15% died; 6.89% of the patients achieved remission after receiving anti-allergy therapy. Patients with chronic hypersensitivity pneumonia were found with poor prognosis.ConclusionsIn patients with diffuse interstitial pulmonary fibrosis with unknown cause, transbronchoscope freezing lung biopsy as early as possible is a feasible method for early diagnosis and improving prognosis. Patients with hypersensitivity pneumonitis with a long course of pulmonary fibrosis have a poor response to glucocorticoid and other classic treatments, and most of them have a poor prognosis.

    Release date:2021-04-25 10:17 Export PDF Favorites Scan
  • 肺放线菌病一例报告

    目的 通过分析1例肺放线菌病患者的临床特征及诊治过程,结合文献复习,加强临床医生对本病的认识,以期早期诊断、规范治疗,改善患者预后。方法 回顾性分析1例肺放线菌病患者的临床资料,以“肺放线菌病”、“放线菌肺炎”为关键词在万方数据库、中国知网数据库、中国期刊全文数据库,以“pulmonary”和“actinomycosis”为关键词在PubMed数据库检索相关文献进行文献复习,总结肺放线菌病的临床特征、影像学特点、诊断手段、治疗及预后不良相关因素。结果 患者男,53岁,因“反复咳嗽、咳痰4-个月,伴咯血15 d”入院。患者无明显肺外临床表现,内科查体未见明显异常,外院胸部CT提示左下肺团片影,两次痰液送检二代测序,均检出放线菌菌属(检出最多序列数4393),革兰阳性格雷文尼放线菌多(检出最多序列数4258)。入院后予头孢哌酮舒巴坦针静脉滴注抗感染19 d,出院后继续复方磺胺口服,随访至2021年3月患者胸部影像学提示病变已明显吸收好转。结合本例及国内外文献报道的肺放线菌病相关资料,结果显示该病中老年男性多见,口腔卫生条件不良是该病的主要危险因素,临床症状无特异性,主要表现为咳嗽、咳痰、咯血,胸部CT典型表现为团块影并空洞形成,增强后不均匀强化,病灶内见稍低密度或气体密度,经支气管活检、经皮肺穿刺或手术标本病理为主要确诊手段,近年二代测序技术已成为诊断该病的重要工具。放线菌对多种抗菌药物敏感,但抗菌药物治疗疗程长,推荐6~12个月。内科及介入无法控制的大咯血以及抗菌药物治疗长疗程前提下临床症状无缓解、胸部影像学肺部病灶无明显吸收的患者,手术可能是一种治疗选择。结论 肺放线菌病的临床特征及影像学表现均不典型,影像学上常表现为巨大团块,临床上常被误诊为肿瘤。痰液、纤维支气管镜灌洗液标本及经皮肺穿刺组织标本病原微生物二代基因测序有助于确诊。经抗菌药物治疗预后良好,对于止血药物及介入无法控制的大咯血、使用长疗程抗菌药物后临床症状仍缓解不明显或胸部影像学病变未见明显吸收的患者,手术可能是一种治疗选择。

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  • 急性肺栓塞危险分层及预后评估研究进展

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