应重视弥漫性肺泡出血综合征的诊治策略

弥漫性肺泡出血综合征(diffuse alveolar hemorrhage syndrome,DAHS)是以痰中带血、咯血、呼吸困难、进行性贫血和胸部X线或CT影像呈弥漫性肺泡浸润为特征的临床综合征[1]。DAHS临床较为少见,多数起病急骤,病因复杂,如感染、中毒、药物、化学物质、细胞毒药物、自身免疫系统性疾病、系统性血管炎、血液系统疾病、造血干细胞移植等均可导致DAHS。尽管病因各异,但病理生理机制     

感染性疾病与肺泡出血综合征

1　概念　　肺泡出血综合征是临床特点为呼吸困难、咯血、Ｘ线胸片为双侧弥漫性肺泡浸润以及贫血的一组疾病。通常在发病之初不能被早期诊断 ,典型的咯血和肺泡浸润有时被认为是肺水肿或肺炎。对早期肺泡出血综合征错误的诊断和治疗将导致两种严重后果 :(1)疾病迅速进展导致呼吸衰竭 ,(2 )肺泡出血常常是一种全身系统疾病的早期表现 ,这种疾病会导致肺外器官的损害 ,尤其是肾脏。2　肺泡出血综合征病因　　在肺泡出血的疾病中 ,主要包括严重的免疫性疾病、特发性疾病、感染性疾病、凝血功能障碍疾病和急性肺损伤等原因。二尖瓣狭窄、骨髓移…     

弥漫性肺泡出血

弥漫性肺泡出血是以咯血、缺铁性贫血和胸片以暂时性弥漫肺泡浸润或实变为特征的临床综合征。此综合征可见于一组各种各样的异质性疾病，其中许多疾病还共有肾小球肾炎的临床表现，并可表现为Good-pasture综合征而增加诊断的复杂性和治疗的难度，本文对弥漫性肺泡出血的分类和Good—pasture综合征诊断的主要问题进行了评述。     

肺静脉闭塞病患者的临床特点及预后分析

目的 分析肺静脉闭塞病(PVOD)的临床表现、诊断治疗及预后情况.方法 回顾性分析2008年5月至2011年5月间同济大学附属上海市肺科医院临床诊断PVOD患者的临床特点及预后情况.结果 共5例患者被诊断为PVOD,年龄范围为12 ～42(22±12)岁,其中女性4例,男性1例.患者从首发症状至被确诊为PVOD的时间为2～50(16±20)个月,其中4例曾被误诊为特发性肺动脉高压.患者确诊时心功能均显著下降,其中3例为WHO肺高压功能分级Ⅲ级,另2例为Ⅳ级.所有患者胸部CT检查均提示双肺弥漫性毛玻璃样渗出征象,肺功能检查示肺泡弥散功能显著下降,平均肺泡一氧化碳弥散量/预计值为(38±12)％.右心导管测得患者肺动脉压力和肺血管阻力均显著升高.5例患者均接受传统和肺动脉高压靶向药物治疗.随访至今,其中4例患者因难治性右心衰竭死亡,从出现首发症状至死亡及从确诊PVOD至死亡的时间分别为5～65(27±26)个月和1 ～16(9±9)个月.结论 PVOD是罕见的恶性心肺血管疾病,易被误诊,使用现有肺动脉高压靶向治疗药物疗效不佳、预后差,应及早考虑进行肺移植治疗.     

肺泡出血诊断标准及194例免疫功能低下患者的结果分析

免疫功能低下患者的治疗过程中经常出现肺部并发症,并且是住院和死亡的主要原因。许多因素可能与肺部并发症有关,包括感染、药物和放射线的毒性、肺水肿、肿瘤性疾病、非特异性肺部炎症和肺泡出血。肺泡出血是免疫功能低下患者呼吸系统症状的一个重要原因。作者用支气管肺泡灌洗得到的肺泡巨噬细胞中噬铁细胞(Siderophages,即含铁血黄素吞噬细胞)的百分比确定肺泡出血的诊断。并通过分析194例免疫功能低下患者肺泡出血的临床、生物学和组织学参数的相关性,以及与其他诊断际准的对比,对这种方法进行了评价。 作者对194例免疫功能低下患者进行240次支气     

弥漫性肺泡出血与自身免疫性疾病

弥漫性肺泡出血 (diffusealveolarhemorrhage ,DAH)是以咯血、呼吸困难、缺铁性贫血和X线胸片呈弥漫性肺泡浸润或实变为特征的临床综合征[1] 。DAH临床少见 ,但病情凶险 ,常威胁患者生命。凡引起广泛的肺泡 毛细血管损伤 ,导致肺泡腔内出血的任何病因 ,均可引起DAH(表 1)。然而 ,不同疾病采取的治疗措施不同 ,其预后也截然不同。由于引发DAH的多样性、免疫机制的复杂性 ,目前对于DAH尤其是自身免疫相关DAH在诊断方面存在认识上不足并常延误治疗。本文就自身免疫性疾病相关DAH诊断及治疗进行综述。表 1　弥漫性肺泡出血的常见病因…     

弥漫性肺泡出血

本文通过1例急性早幼粒细胞白血病诱导分化治疗过程中出现的凶险的弥漫性肺泡出血,从影像学可看到病变急起、进展迅速且弥漫分布,经俯卧位通气,有创机械通气及药物治疗后病变较快吸收的动态演变过程,并结合文献讨论急性早幼粒细胞白血病合并弥漫性肺泡出血的发病、影像和治疗,为临床诊断和治疗提供参考。     

自身免疫性疾病相关的弥漫性肺泡出血

弥漫性肺泡出血综合征(diffuse alveolar hemorrhage syn-drome,DAHs)指在病因作用下,肺微血管血液进入肺泡,发生弥漫性肺泡出血(DAH),临床出现呼吸困难、咯血、贫血等症状,胸部X线片示双侧弥漫性肺泡浸润.该综合征是发生在一系列疾病过程中的严重并发症,并非一独立疾病,多来势凶险,危及生命,症状复杂.目前对于DAH尤其是自身免疫性疾病相关的DAH认识不足,常导致诊断及治疗延误,应引起临床医生高度重视.     

浅析弥漫性肺泡出血

弥漫性肺泡出血（Diffuse Alveolar Hemorrhage,DAH）屡见于临床,多数病例经历了漫长的延误诊断。此症大多来势凶险,死亡率高。不典型的多系统受累,迷惑了不少临床医生。及时诊断,正确抢救治疗,才能挽救生命。对其研究较前有长足进步。本病是以咯血、呼吸困难、缺铁性贫血和X线胸片呈弥漫性肺泡浸润为特征的临床综合征。     

系统性红斑狼疮合并弥漫性肺泡出血三例分析

弥漫性肺泡出血是系统性红斑狼疮(SLE)患者一种罕见而严重的并发症。现将我们诊治的582例SLE患者中确诊的资料完整的3例弥漫性肺泡出血报告如下。     

Drug-associated organizing pneumonia: high-resolution CT findings in 9 patients

OBJECTIVE: To assess the high-resolution computed tomography appearances of drug-associated organizing pneumonia (OP). MATERIALS AND METHODS: Nine patients with clinical and biopsy proven drug-associated OP were included in the study. High-resolution computed tomography exams were retrospectively reviewed by 2 observers for the presence, extent, and distribution of parenchymal abnormalities. RESULTS: The commonest findings consisted of air space consolidation (n = 8) and ground-glass opacification (n = 7). The air space consolidation was bilateral and asymmetric in 7 patients, symmetric in 1 patient and in all cases involved mainly the peribronchial or subpleural regions or both. The ground-glass opacities were predominantly bilateral and asymmetric in 6 patients and unilateral in 1 patient. They had a patchy distribution in 4 patients, were diffuse in 2 patients, and subpleural in 1 patient. CONCLUSION: Drug-associated OP most commonly presents with bilateral, asymmetrical air space consolidation in a predominantly peribronchial and subpleural distribution.     

Drug-associated deaths of medical inpatients.

Of 7,423 medical inpatients, 16 (0.22%) died of drug-associated causes. The overall mortality for all medical inpatients was 6.5%. Eleven of the 16 patients who died of drug-associated causes had been terminally ill; the rest had been seriously ill before the fatal drug reaction occurred. Half of the patients had had either hematologic malignant changes or lupus nephritis. Antineoplastic drugs, azathioprine, prednisone, and heparin sodium were the most frequently implicated drugs. In other studies, we have found widely differing incidences of fatal drug reactions, due to a number of different drugs; these disparities are probably related to variations in the types of illnesses amoung different hospital populations and to varying interpretations of the term "drug-associated death." Extrapolation from the available data to a national incidence of drug-associated deaths is not possible. Drug-associated deaths are relatively uncommon and usually occur in the cases of severely or terminally ill patients treated with potentially highly toxic drugs.     

Propafenone hepatotoxicity: report of two new cases

Two patients developed acute cholestatic hepatitis during treatment with propafenone. Viral infections, alcohol abuse, hepatotoxicity by other drugs, and biliary obstruction were excluded as causes. In one patient, liver biopsy showed changes consistent with a drug-associated injury. Another patient had autoimmune antibodies (ANA) in the serum. Following propafenone withdrawal, the clinical and biochemical profiles of both patients improved. Hepatic toxicity from the antiarrhythmic drug propafenone is highly uncommon. Moreover, the drug produces hepatocellular injury by an unknown mechanism. Most of the seven cases reported here had acute cholestatic hepatitis after a latency period of two to four weeks.     

Hepatic epithelioid haemangioendothelioma: challenges in diagnosis and management

Background: Hepatic epithelioid haemangioendothelioma (HEH) is a rare, low grade malignant neoplasm of endothelial origin which is difficult to diagnose and has a variable outcome. We review five HEH cases from our centre with the aim of identifying clinical predictors of outcome and various therapeutic options. Methods: A search was made on the WA Liver Transplant registry for cases with histologically confirmed HEH. Their medical records were reviewed. A literature search was conducted through Medline using terms to compare the results from this series with those of other series. Results: Five patients were identified to have HEH. The mean age was 44.2 years (range 34–53 years). Four of five patients presented with dyspepsia and two patients had clinical evidence of portal hypertension with ascites. Two patients had radiologically diffuse disease and three patients had discrete nodular liver involvement. The mean duration from presentation of symptoms to diagnosis of HEH was 26.8 months. Liver transplantation was performed in one patient with diffuse HEH who is alive with no disease recurrence at 3 years. Three patients with radiologically stable disease followed with 6 monthly surveillance imaging are currently alive and well. The median survival of all five patients was 5 years (range 1.5–16 years) at the time of follow up. Conclusions: These results support the role of surveillance alone for patients with focal and radiologically stable disease. Patients with diffuse HEH with hepatic decompensation should be considered for transplantation. However, numbers are small and an international registry is required to make firm comparisons.     

Further evidence of inherited long QT syndrome gene mutations in antiarrhythmic drug-associated torsades de pointes.

BACKGROUND: Pathophysiologically significant ion-channel mutations have been detected in only a minority of cases of acquired long QT syndrome (LQTS). OBJECTIVE: The aim of this study was to clarify the putative role of subclinical inherited LQTS in drug-associated torsades de pointes (TdP) and to assess the concomitant proarrhythmic factors. METHODS: We evaluated 16 consecutive cases with documented, antiarrhythmic drug-induced TdP who were referred to the Laboratory of Molecular Medicine at Helsinki University for LQTS genetic testing between September 2000 and August 2005. RESULTS: A prolonged QTc interval was observed in 56% of the patients before administration of the drug. TdP was associated with amiodarone in seven, sotalol in six, flecainide in two, and propafenone in one of the cases. Except for the culprit drug, one or more risk factors such as female sex, congestive heart failure, and atrial fibrillation were present in each drug-associated TdP. DNA samples were screened for the four common Finnish founder mutations (KCNQ1 G589D and IVS7-2A-->G, HERG L552S, and R176W), which are known to account for the majority of inherited LQTS in Finland. A total of three (19%) individuals carried one of these four mutations. CONCLUSIONS: Our data show that previously unsuspected LQTS mutations may be present in patients with antiarrhythmic drug-associated TdPs. A normal QTc interval does not exclude the risk of proarrhythmia.     

Primary lymphoma of the thyroid gland. Diagnostic and therapeutic discussion. Apropos of 4 cases

Non Hodgkin lymphomas (NHL) of the thyroid are rather uncommon and account for about 7.7 p.100 of the tumours of the gland. They are remarkable by their usual occurrence in patients with Hashimoto's disease and by controversy about their therapy. The clinical features and the course of four additional cases occurring in four female patients (ages ranged from 61 to 73 years) are reported. The clinical picture was that of a quickly growing diffuse or multinodular goiter, squeezing surrounding neck structures. Antithyroid antibodies were present in 3 cases; microscopical examination showed cytologic features of lymphocytic thyroiditis. Two patients died within a few months, the follow-up is about two years for the others. Two main problems are discussed. First, NHL of the thyroid usually occurs in patients with Hashimoto's disease: how to confidently recognize lymphomas by fine needle biopsy in such cases? Second, which is the best treatment? The authors think that lymphomas of the thyroid have to be considered rather as lymphomas than thyroid tumours. Therefore the cure of the disease may need chemotherapy, in some cases associated with radiotherapy according to careful staging and grading in each case.     

Invasive candidal enterocolitis followed shortly by fatal cerebral hemorrhage in immunocompromised patients

Two immunocompromised adult patients (male, 43 years of age; female, 50 years of age) are described who developed unusual and similarly unique complications. After chemotherapy, both presented with abdominal pain and severe bloody diarrhea. Initial blood cultures were negative, and abdominal CT showed pneumatosis and thickening of colonic wall. Colonoscopy showed severe right-sided colitis. Relentless bloody diarrhea necessitated emergency right hemicolectomy in both cases. Pathologic examination of the specimens showed extensive and severe hematogenous Candida enterocolitis. Both patients died of acute cerebral hemorrhage within 3 days of hemicolectomy. Autopsy, performed on 1 of the 2 cases, showed diffuse severe cerebritis and cerebral hemorrhage secondary to hematogenous invasive candidiasis. This sequence of events is not documented in the English literature on candidal enterocolitis, which is reviewed.     

Acquired aplastic anaemia in adults. I. A retrospective analysis of 40 cases: single factors influencing the prognosis.

In a retrospective analysis of 40 aplastic anaemia patients, an attempt was made to determine prognostic parameters permitting discrimination between short survivors (less than 6 months) and long-term survivors (greater than 6 months). Short survival proved to be significantly associated with a persistently low reticulocyte index and progressive neutropenia. Other factors such as bone marrow cellularity, HBF level, aetiology, or presenting signs were not indicative. Chromosomal aberrations were found in one third of the cases examined, but none had developed into a leukaemic stage after an observation period of 2--5 years. None of the patients with cytogenetic aberrations showed complete restoration of haematopoiesis, in contrast to several of the patients without chromosomal abnormalities. In this series, drug-associated aplastic anaemia was found more often in the short-survival group. These clinical findings indicate that the term 'aplastic anaemia' covers a heterogenous group of disorders.     

Flavored cigar smoking induces acute eosinophilic pneumonia

Two cases of acute eosinophilic pneumonia (AEP) following smoking of flavored cigars were analyzed for characteristic features. None of our patients had a history of smoking flavored cigars/cigarettes in the past. One of them had never smoked, and the second patient was an ex-smoker who quit 17 years ago. Both patients presented with community-acquired pneumonia-like symptoms that did not respond to treatment with antibiotics. Their chest radiographs revealed bilateral diffuse infiltrates. The diagnosis of AEP was established based on the clinical picture, BAL that revealed an average eosinophil count > 45%, and immediate clinical improvement after introducing corticosteroids. All other possible causes were excluded during the initial workup.     

The role of anti-epithelial cell antibodies in the pathogenesis of bilateral radiation pneumonitis caused by unilateral thoracic irradiation

Two cases of bilateral radiation pneumonitis associated with unilateral thoracic irradiation against lung cancer are described. Both patients died of respiratory failure and autopsy was performed. Histologically, bilateral diffuse alveolar damage was demonstrated in both cases, associated with marked organization of hyaline membrane in one case (case 1). In addition, numerous hyperplastic type II pneumocytes which strongly expressed cytokeratins 8, 18 and 19 were observed. In both patients' sera, antibodies against cytokeratin 8, 18 and 19 were demonstrated by a Western immunoblot. The possible association between autoantibodies to cytokeratins and diffuse alveolar damage observed in patients with bilateral radiation pneumonitis are discussed.