肺泡蛋白沉积症16例临床分析

[目的]探讨肺泡蛋白沉积症（PAP）的诊治方法.[方法]回顾性分析16例PAP患者的临床特点、影像学表现及治疗方法.[结果]PAP患者常见症状为咳嗽进行性加重的呼吸困难,体征无特异性.胸部CT特征性表现为＂地图征＂、＂铺路石＂或＂碎石路样＂,肺泡灌洗治疗的14例患者临床症状明显缓解.[结论]PAP患者误诊误治普遍,宜重视影像学特征,早期行支气管镜、肺泡灌洗（BAL）、经支气管肺活检,支气管肺泡灌洗和（或）肺活检是确诊PAP的重要方法,肺泡灌洗是治疗PAP的主要方法.     

肺泡蛋白沉积症2例报告并文献回顾

目的：总结肺泡蛋白沉积症(PAP)的临床特征、诊断及治疗。方法：对2例患者的临床资料进行分析,并回顾复习有关文献。结果：PAP发病呈特发性或继发于其他疾病,病程慢性迁延,临床症状明显,但体征较少;胸部影像学改变突出,胸部CT可见“地图样”改变或“铺路石”征,特征性地表现为症状、体征分离和影像学、体征分离现象;PAP在临床上呈良性经过,支气管肺泡灌洗液检查可用于早期诊断,全肺灌洗治疗效果显著。结论：PAP在临床上特征性地表现为症状、体征分离和影像学、体征分离现象,支气管肺泡灌洗液的病理和电镜检查可用于PAP的早期诊断,全肺灌洗是安全有效的治疗方法。     

肺灌洗治疗肺泡蛋白沉积症的手术配合体会

肺泡蛋白沉积症（PAP）是一种病因不明的慢性肺泡充填性疾病,其特点是肺泡内间歇性蓄积过碘酸雪夫（PAS）染色阳性的富含磷脂的蛋白样物质,从而影响肺泡的气体交换,导致呼吸困难,低氧血症.肺灌洗是目前公认的治疗PAP的最有效方法.本文总结我院2003年2月以来确诊为肺泡蛋白沉积症的患者行单侧肺灌洗术的手术配合体会.     

肺泡蛋白沉积症9例临床分析

目的:通过分析病例,总结经验,提高认识,并结合文献讨论肺泡蛋白沉积症的诊治进展。方法:回顾性分析9例肺泡蛋白沉积症患者的临床资料。结果:呼吸困难为最常见的症状,胸片和CT及肺功能改变可提示诊断,经纤维支气管镜肺泡灌洗和肺活检可确诊,单侧或全肺灌洗治疗可取得良好效果。结论:肺泡蛋白沉积症虽然少见,起病隐匿,临床表现不典型,但只要提高认识,诊断并不困难,经纤维支气管镜肺泡灌洗和肺活检多可获得诊断,治疗以灌洗治疗为首选,效果显著。     

肺泡蛋白沉着症行全肺灌洗术患者20例的护理

肺泡蛋白沉着症（pulmonary alveolar proteinosis,PAP）是一种病因未明的少见肺部疾病,其特征是肺泡腔内间断沉积PAS染色阳性的富含大量磷脂的蛋白样物质,从而影响肺泡的气体交换,导致呼吸困难、低氧血症等一系列临床综合征。该病治疗的关键在于去除肺泡内沉积的蛋白样物质,最直接有效的疗法是全肺支气管-肺泡灌洗。我科从2006年5月至2009年8月来共收治了20例肺泡蛋白沉着症行全肺灌洗术的患者,疗效满意,现将护理体会报告如下。     

肺泡蛋白沉积症的X线、CT诊断

目的：总结肺泡蛋白沉积症（Pulmonary Alveolar Proteinosis PAP)的X线、CT影像学特征。材料与方法：报道经支纤镜活检及肺泡灌洗术证实的肺泡蛋白沉积症5例，复习文献资料，对PAP的X线、CT表现进行综合分析，并探讨其诊断和鉴别诊断。结果：该病X线表现：两肺对称或不对称分布磨玻璃样阴影，自肺门向外放射呈“蝶翼征”；其中可见弥漫结节样阴影。CT表现：1、双肺弥漫性斑片状阴影与磨玻璃样改变，病灶与正常肺组织间分界清楚，呈地图样分布。2、蝶翼征。3、支气管充气征。4、铺路石征（Crazy-Paving sign)。结论：肺泡蛋白沉积症具有上述典型的X线、CT表现，结合临床病史综合分析可作出诊断，支纤镜检及肺泡灌洗术是本病确诊的主要依据。     

特发性肺泡蛋白沉积症行全肺灌洗术的配合

肺泡蛋白沉积症（PAP）是一种病因未明的肺部少见疾病，全肺灌洗是迄今唯一有效的治疗方法，但风险较大，对麻醉及护理配合要求较高。2003年2月至2007年1月，本院对3例肺泡蛋白沉积症患者进行全麻下全肺灌洗，现将麻醉及护理配合进行总结，报告如下。     

一例肺泡蛋白沉积症患者行肺泡灌洗术的护理

肺泡蛋白沉积症（Pulmonary Alveolar Proteinosis，PAP）是一种临床上罕见的病因不明的慢性肺泡填塞性疾病，主要以肺泡内大量沉积磷脂蛋白样物质为特点。临床表现主要为反复咳嗽、咯白色粘痰、呼吸困难。目前，临床上无有效的药物治疗方法。国内外有采用全肺灌洗治疗PAP取得肯定疗效。我科2005年10月收治1例PAP患者，采用肺泡灌洗治疗，取得显著的疗效。现将护理体会报告如下。[第一段]     

17例肺泡蛋白沉积症临床分析

目的：总结肺泡蛋白沉积症（PAP）患者的诊治情况，提高对本病的认识。方法：回顾性分析1996年7月～2006年2月复旦大学附属中山医院肺科经病理确诊的17例PAP患者临床资料。结果：17例患者中14例行肺泡灌洗治疗，治疗后14例患者临床症状、胸部CT、血气、肺功均明显改善。结论：本病主要临床表现为咳嗽、进行性呼吸困难、呼吸音减低。胸部CT示两肺地图样片状阴影，呈碎石铺路样改变。X线胸片示：双肺弥漫性分布，多发斑片状、结节状、毛玻璃状、网织状等阴影。确诊根据病理检查结果。支气管肺泡灌洗仍是迄今唯一有效的治疗方法。     

肺泡蛋白沉积症3例报告并文献复习

目的探讨肺泡蛋白沉积症(PAP)的临床特征、诊断及治疗。方法报道3例肺泡蛋白沉积症,并复习相关文献。结果 PAP临床无特征性,以咳嗽、咳痰、气促多见,但体征较少。胸部CT可见"地图样"改变或"铺路石"征。结论 PAP发病率低,起病隐匿,误诊或漏诊率较高,支气管肺泡灌洗液及肺组织的病理检查可用于PAP的早期诊断,全肺灌洗是安全有效的治疗方法。     

Whole lung lavage in the treatment of pulmonary alveolar proteinosis.

Although a rare condition, pulmonary alveolar proteinosis (PAP) can be a very devastating diagnosis with life-altering consequences. This case study follows the path of a young woman who is currently undergoing whole lung lavage as treatment for pulmonary alveolar proteinosis. The entire concept of flooding a lung with large quantities of saline as a treatment for lung disease is contrary to normal respiratory care. Caring for the patient with PAP provides many challenges for the perianesthesia nurse. Management of the postanesthesia airway, oxygen administration and maintenance of oxygen saturation, and pain relief skills are all of high importance to the patient with PAP. These skills plus the emotional support provided by the experienced perianesthesia nurse can ensure a safe recovery from this unusual procedure.     

国内20年肺泡蛋白沉积症误诊原因汇总分析

目的 汇总分析20年来国内报道的肺泡蛋白沉积症(PAP)的临床特点、影像学特点、确诊方法、误诊情况、治疗及预后,为临床医师快速准确地诊断本病提供重要线索.方法 回顾性分析1988～2008年国内有关肺泡蛋白沉积症的文献资料,总结126例肺泡蛋白沉积症误诊患者的临床资料.结果 126患者误诊为肺炎19例,肺癌24例,支气管肺炎18例,特发性肺间质纤维化(IPF)19例,肺结核15例,嗜酸性肺炎10例,结节病9例,真菌肺炎7例,上呼吸道感染5例.肺泡蛋白沉积症患者的临床表现缺乏特异性,而影像学表现又呈多样性,临床易误诊,确诊有赖于肺泡灌洗液、肺活检以及病理学检查.结论 肺泡蛋白沉积症临床表现缺乏特异性,极易导致误诊;全肺灌洗是肺泡蛋白沉积症最安全和有效的治疗方法.     

Pulmonary alveolar proteinosis complicated with tuberculosis: A case report

BACKGROUNDPulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of phospholipoproteinaceous material in the alveoli. Cases of PAP complicated with tuberculosis are much more complex and have rarely been well recorded. CASE SUMMARYWe describe a 21-year-old Han Chinese patient with suspicious lung infection associated with mild restrictive ventilatory dysfunction and diffusion reduction. High resolution computed tomography revealed a “crazy-paving” appearance and multiple pulmonary miliary nodules around the bronchi. Bronchoalveolar lavage demonstrated a small amount of periodic acid-Schiff positive proteinaceous materials. A serological test for the presence of a Mycobacterium tuberculosis antibody and an interferon-gamma release assay were both positive. The patient received a standard course of first-line anti-tuberculosis treatment after diagnostic bronchoalveolar lavage. To date, clinical remission has been achieved and maintained for five years. CONCLUSIONIn summary, the diagnosis of PAP complicated with tuberculosis was supported by a combination of clinical manifestations, imaging, pulmonary function, laboratory examinations, bronchoalveolar lavage, etc. This case highlighted that diagnostic bronchoalveolar lavage in combination with anti-tuberculosis treatment is a safe and effective option for mild PAP patients with tuberculosis.     

Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor.

Idiopathic pulmonary alveolar proteinosis (I-PAP) is a rare disease of unknown etiology in which the alveoli fill with lipoproteinaceous material. We report here that I-PAP is an autoimmune disease with neutralizing antibody of immunoglobulin G isotype against granulocyte/macrophage colony-stimulating factor (GM-CSF). The antibody was found to be present in all specimens of bronchoalveolar lavage fluid obtained from 11 I-PAP patients but not in samples from 2 secondary PAP patients, 53 normal subjects, and 14 patients with other lung diseases. It specifically bound GM-CSF and neutralized bioactivity of the cytokine in vitro. The antibody was also found in sera from all I-PAP patients examined but not in sera from a secondary PAP patient or normal subjects, indicating that it exists systemically in I-PAP patients. As lack of GM-CSF signaling causes PAP in congenital cases and PAP-like disease in murine models, our findings strongly suggest that neutralization of GM-CSF bioactivity by the antibody causes dysfunction of alveolar macrophages, which results in reduced surfactant clearance.     

Pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis is a rare but potentially treatable disease, characterized by impaired surfactant metabolism that leads to accumulation in the alveoli of proteinaceous material rich in surfactant protein and its component. Novel insights from an animal model aided the discovery of granulocyte macrophage colony stimulating factor (GM-CSF) antibodies as a pathogenetic mechanism in human pulmonary alveolar proteinosis. The vast majority of pulmonary alveolar proteinosis occurs as an autoimmune disease; less commonly, it is congenital or secondary to an underlying disorder such as infection, hematological malignancy, or immunodeficiency. The subacute indolent course of this disease often delays the diagnosis by months to years. Crazy-paving appearance in a geographic distribution is a characteristic feature of this disease visible on high-resolution computed tomography (CT). A definitive diagnosis, however, requires lung biopsy, which typically shows partial or complete filling of alveoli with periodic-acid-Schiff-positive granular and eosinophilic material in preserved alveolar architecture. Patients with minimal symptoms are managed conservatively, whereas patients with hypoxemia require a more aggressive approach. Whole-lung lavage is the most widely accepted therapy for symptomatic pulmonary alveolar proteinosis. Correction of GM-CSF deficiency with exogenous GM-CSF is an alternative therapy. The combination of a systemic treatment (GM-CSF) and a local treatment (whole-lung lavage) augmenting the action of one another is a promising new approach. As the knowledge about this rare disease increases, the role of novel therapies is likely to be better defined and optimized.     

Pulmonary alveolar proteinosis complicated with nocardiosis: A case report and review of the literature

BACKGROUNDPulmonary alveolar proteinosis (PAP) is a pulmonary syndrome wherein large volumes of phospholipid and protein-rich surfactants accumulate within the alveoli. PAP forms include primary (auto-immune PAP), secondary, and congenital. Nocardiosis is a form of suppurative disease induced upon infection with bacteria of the Nocardia genus. Clinically, cases of PAP complicated with Nocardia infections are rare, regardless of form. Unfortunately, as such, they are easily overlooked or misdiagnosed. We describe, here, the case of a patient suffering from simultaneous primary PAP and nocardiosis. CASE SUMMARYA 45-year-old Chinese man, without history of relevant disease, was admitted to our hospital on August 8, 2018 to address complaints of activity-related respiratory exertion and cough lasting over 6 mo. Lung computed tomography (CT) revealed diffuse bilateral lung infiltration with local consolidation in the middle right lung lobe. Subsequent transbronchial lung biopsy and CT-guided lung biopsy led to a diagnosis of primary PAP (granulocyte-macrophage colony-stimulating factor antibody-positive) complicated with nocardiosis (periodic acid-Schiff-positive). After a 6 mo course of anti-infective treatment (sul-famethoxazole), the lesion was completely absorbed, such that only fibrous foci remained, and the patient exhibited significant symptom improvement. Follow-up also showed improvement in pulmonary function and the CT imaging findings of PAP. No whole-lung lavage has been conducted to date. This case highlights that active anti-nocardia treatment may effectively improve the symptoms and alleviate PAP in patients with PAP and nocardia, possibly reducing the need for whole-lung lavage.CONCLUSIONWhen evaluating patients presenting with PAP and pulmonary infections, the potential for nocardiosis should be considered.     

双侧全肺灌洗对血流动力学和呼吸功能的影响

目的探讨肺泡蛋白沉积症(PAP)患者行大容量双侧全肺灌洗过程中血流动力学和呼吸功能的变化。方法对14例行全肺灌洗术的PAP患者临床资料进行回顾性分析。结果每次灌洗液注入肺时引起血流动力学和呼吸功能抑制,表现为中心静脉压(CVP)、胸液成分(TFC)、气道峰压(Peak)、气道平台压(Plat)、气道阻力升高,HR、有创血压(MAP)、每搏输出量(SV)、潮气量(VT)、肺顺应性降低(P<0.05或P<0.01),灌洗液吸出后改善。随着灌洗次数增加,血流动力学和呼吸功能抑制更明显,表现为MAP、CO、SV、心肌加速指数(ACI)、SpO2、呼气末二氧化碳(PetCO2)、体温、肺顺应性降低,CVP、TFC、Peak、气道阻力增加(P<0.05或P<0.01),恢复双肺通气后改善。两侧肺灌洗结束后表现为代谢性酸中毒,pH、BE、HCO3、TCO2降低(P<0.05或P<0.01),恢复双肺通气1h后改善。结论在每次肺灌洗液注入前后,血流动力学和呼吸功能均出现周期性波动。随着灌洗次数增加,全肺大容量肺灌洗使血流动力学和呼吸功能抑制。