肺淋巴管肌瘤病一例临床报告及文献复习

目的提高对肺淋巴管平滑肌瘤病(pulmonary lymphangidei omyomatosis,PLAM)诊断和难治性气胸的认识。方法报道确诊为PLAM的新病例1例,结合国内报道的PLAM病例,对该病的临床特点进行汇总分析。结果肺淋巴管肌瘤病易并发难治性气胸,50%以此为首发症状,80%在疾病过程中发生气胸。气胸常常表现为复发性,双测性。结论胸部高分辨CT对PLAM具诊断价值。对育龄妇女发生的渐进性呼吸困难、咯血、自发性气胸应及时进行胸部高分辨CT检查。并应常规进行腹部和盆腔影像学检查以了解患者是否合并肺外淋巴管平滑肌瘤。     

肺淋巴管肌瘤病48例

肺淋巴管平滑肌瘤病(PLAM)首发症状缺少特异性,临床上常易误诊或漏诊,而在诊断后的处理上缺乏可以参考的权威文献。目前其发病机制亦不清,单一的医学中心又很难募集到足够的病例数,更不用说临床试验,故临床上该病的循证医学证据较少。故本文将国内经病理证实的PLAM的临床资料进行回顾分析,旨在提高其诊治能力。     

肺淋巴管肌瘤病11例报告并文献复习

目的 探讨肺淋巴管肌瘤病( PLAM)的临床和预后.方法 分析经治的11例PLAM病人的资料,并复习文献.结果 11例均为女性;年龄31 ～55岁,平均(44±9)岁;均经肺组织活检明确病理;主要临床表现为进行性呼吸困难10例(发生率91％),咳嗽6例(55％),咯血2例(18％),乳糜胸3例(27％),气胸3例(27％)等;11例患者均进行肺功能检测,均有弥散功能下降,9例行动脉血气分析,其中7例表现为低氧血症,4例合并呼吸衰竭;11例行胸部高分辨率CT (HRCT),均显示两肺弥漫性薄壁囊状阴影.结论 PLAM以呼吸困难,咳嗽,气胸和乳糜胸为主要临床表现;双肺弥漫性薄壁囊状阴影为影像学特征表现;肺组织活检是确诊的主要依据.     

系统性红斑狼疮合并肺淋巴管肌瘤一例

系统性红斑狼疮患者约50％可累及肺及胸膜，主要表现为狼疮肺炎、胸膜炎、肺动脉高压和肺出血等，而临床上合并肺淋巴管平滑肌瘤罕见。现将我院收治的1例系统性红斑狼疮合并肺淋巴管肌瘤报告如下。     

肺淋巴管肌瘤病一例

肺淋巴管肌瘤病一例胡红赵会泽段晋庆刘又宁肺淋巴管肌瘤病（Ｐｕｌｍｏｎａｒｙｌｙｍ－ｐｈａｎｇｉｏｍｙｍａｔｏｓｉｓ，ＬＡＭ）是一种罕见病。１９３７年由ＶｏｎＳｔｏｓｓｅｌ首先报告［１］，１９６６年Ｃｏｒｎｏｇ［２］将本病命名为“淋巴管肌瘤病”。１９８...     

肺淋巴管肌瘤病并肺母细胞瘤1例临床分析并文献复习

目的 加强肺淋巴管肌瘤病（LAM）合并恶性肿瘤的临床、影像、病理特点的认识,提高对该病的早期认识和治疗水平.方法 分析2013年3月收治的1例肺LAM并肺母细胞瘤患者临床资料及诊治经过,并结合相关文献进行复习.截止至2014年2月,以“Lymphangiomyomatosis、cancer”为检索词,在PubMed检索系统进行检索,论著3篇,综述1篇;在万方数据库中以“淋巴管肌瘤病、恶性肿瘤”为检索词进行检索,病例报告1篇.结果 患者,女,37岁,慢性病程,主要症状为咳嗽、咯血、胸痛3个月,既往有“双肾错构瘤”史,曾行右肾错构瘤切除术,术后病理示血管平滑肌脂肪瘤,左肾未予处理.曾有“自发性气胸”病史.胸部高分辨率CT示右中下肺巨大肿块并纵隔淋巴结肿大,考虑恶性病变;肺LAM.血清血管内皮生长因子D为1 092.61 ng/L.免疫组化示CD56灶（＋）,Vim小细胞（＋）,Syn（＋）,CD99（＋/-）,组织改变考虑为肺母细胞瘤.复习国内外文献,尚无LAM合并肺母细胞瘤的病例报道.结论 近十年来,随着LAM分子发病机制的深入研究,LAM目前可定义为低度恶性转移性肿瘤.本例患者为LAM合并高度恶性肿瘤肺母细胞瘤,在国内外尚属首例报道.但究其机制,是LAM细胞癌变为肺母细胞瘤,或是二者同时存在,目前尚无定论.     

肺淋巴管肌瘤病的临床及病理特点

目的 提高对肺淋巴管肌瘤病(lymphangioleiomyomatosis,LAM)的临床及病理诊断和认识水平.方法 回顾分析复旦大学附属中山医院确诊为肺LAM的病例15例,并复习1993-2008年国内文献的73例报道,分析该病的临床及病理特点.其中1例患者死后行尸体解剖,对其全身各脏器进行病理组织学观察.结果 88例患者均为女性,平均发病年龄(37±9)岁,常见临床表现依次为呼吸困难83例(94%)、咯血48例(54%)、气胸41例(47%)、乳糜胸28例(32%).其中35例患者行肺功能检查,32例存在弥散功能障碍,25例表现为阻寨性通气功能障碍,10例表现为混合性通气功能障碍.胸部高分辨率CT检查显示双肺为典型的弥漫性薄壁囊状阴影.病理检查结果显示肺组织旱弥漫性囊状改变,增生的LAM细胞沿细支气管壁、肺泡壁、淋巴管肇和血管壁周围分布,形成结节状.1例尸解病理检查结果显示肺脏、肾脏、淋巴结及软组织等多器官受累.42例行腹部B超及影像学检查,其中23例伴发肺外LAM.结论 LAM常累及全身多个系统,但肺是最主要的受累器官.育龄期妇女出现进行性呼吸困难、气胸、乳糜胸及高分辨CT表现为弥漫性小囊状改变时,应考虑到LAM的可能.确诊需依赖肺组织活检病理检查结果,并应常规进行腹部及盆腔影像学检查,以了解患者是否合并肺外LAM.     

淋巴管肌瘤病3例并文献复习

目的探讨淋巴管肌瘤病(LAM)的临床特征和诊断,提高对LAM的认识和诊疗水平。方法回顾性分析3例LAM患者的临床资料,并复习相关文献。结果本组中女2例(43岁和36岁)、男1例(31岁),均出现无明显诱因的呼吸困难、咳嗽,并不断加重。其中病例1肺功能检查存在混合型通气功能障碍,肺弥散功能障碍;胸部高分辨CT(HRCT)示两肺散在囊状透光影,具有特征性表现。病例2有咯血症状,肺组织活检术后引流出胸腔乳糜液。3例术后病理组织检查确诊,病例1免疫组化染色见淋巴管平滑肌呈SM-action、Desmin阳性。结论LAM主要发生于育龄期妇女,无诱因的呼吸困难是最常见的临床表现,肺功能多表现为阻塞性通气功能障碍和弥散功能障碍;特征性的胸部HRCT检查有较高的诊断价值,病理活检和免疫组化检查可确诊。     

肺毛霉病——附二例报告

目的探讨肺毛霉病的临床表现、诊断和治疗方法。方法本组２例采用纤维支气管镜、开胸探查和痰检查，经病理或真菌培养确诊。结果随着广谱抗生素、抗肿瘤药物、皮质激素和器官移植的广泛应用，发病率有增高趋势。病死率极高，国外报道为８０％，局限于肺部者为６５％，播散型高达９６％。国内报道的１０余例仅１例存活，且多为尸检后明确诊断。本组２例确诊后，其中例１两次行肺叶切除术并于手术前后采用氟康唑治疗，例２单用氟康唑治疗，均痊愈出院。结论对临床可疑病例应及时确诊，有手术适应证者应积极手术切除。氟康唑对肺毛霉病有效     

Pulmonary lymphangioleiomyomatosis: a rare presentation

Pulmonary lymphangioleiomyomatosis (LAM) is a rare idiopathic, cystic disease. We report a case of LAM who presented with nonchylous, bilateral pleural effusion and progressive respiratory failure.     

Efficacy and safety of transbronchial lung biopsy for the diagnosis of lymphangioleiomyomatosis: A report of 24 consecutive patients

Background and objective

Lymphangioleiomyomatosis (LAM) is a diffuse cystic lung disease that occurs in women of childbearing age. LAM can be diagnosed on a clinical basis in patients with typical high‐resolution computed tomography (HRCT) patterns and at least one other corroborating disease feature, such as chylothorax, angiomyolipoma, tuberous sclerosis complex or elevated serum vascular endothelial growth factor (VEGF)‐D. However, patients who do not meet these criteria require tissue confirmation for a definitive diagnosis, and the utility of methods that are less invasive than surgical lung biopsy, such as transbronchial lung biopsy (TBLB), are not well studied. We retrospectively studied the efficacy and safety of TBLB for the diagnosis of LAM.

Methods

From January 1991 to August 2015, 131 consecutive LAM patients were prospectively registered in our study, and a TBLB was conducted for 24 patients. We retrospectively studied the yield and safety of TBLB in this cohort.

Results

All 24 patients were women; the median age was 42 years. HRCT showed multiple round thin‐walled cysts diffusely scattered throughout the lungs. The median level of serum VEGF‐D was 2109 pg/mL. Characteristic pathological findings for LAM were identified in 17 patients (70.8%) by two expert pathologists. The %predicted value for diffusing capacity of carbon monoxide was significantly lower in the 17 TBLB‐positive LAM patients compared to the seven TBLB‐negative LAM patients (P = 0.046). There were no serious adverse events such as pneumothorax or uncontrollable bleeding due to TBLB.

Conclusion

TBLB is a safe and effective method for the pathological diagnosis of LAM.

     

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 。     

Pulmonary fibrosis: a possible diabetic complication

Diabetic complications affect many organs in diabetic patients. Emerging evidence indicates that diabetes can increase the risk of pulmonary dysfunction. Early epidemiological studies from different populations on whether diabetes was an independent risk for pulmonary dysfunction were inconclusive. Recent epidemiological studies and systematic reviews clearly indicate that diabetes is an independent risk factor for pulmonary dysfunction. Given that pulmonary fibrosis is an important predictor of mortality in people with this chronic disease, whether diabetes directly causes pulmonary fibrosis is an important unresolved clinical question. This review combines recent epidemiological data with findings from basic research to indicate that diabetes induces pulmonary fibrosis. We then discuss the possible underlying mechanisms for the histological and biochemical pathology. At the end of this review, we emphasize that diabetic pulmonary fibrosis as a potential diabetic complication warrants more attention.     

肺淋巴管平滑肌瘤病

目的 提高对罕见病淋巴管平滑肌瘤病（ＰＬＡＭ）的认为。方法 对１９９２年对来诊治的３例ＰＬＡＭ患者的临床资料进行分析,并结合文献复习。结果 ＰＬＡＭ是一种罕见的弥漫肺部疾病,临床表现为反复发作自发性气胸、活动后呼吸困难和痰血等。肺功能呈阻塞性或混合性通气功能障,动脉血气示低氧血症。胸部Ｘ线表现为两肺弥漫分布网格状影,高分辨ＣＴ（ＨＲＣＴ）示两肺弥漫分布囊状改变。病理学检查示肺组织淋巴管增生和扩张,     

Pulmonary lymphangioleiomyomatosis. Description and course of 2 cases

Pulmonary lymphangioleiomyomatosis (LAM) is a rare, serious, chronic disease whose etiology is unknown and which affects young women almost exclusively. It produces typical clinical and radiological characteristics. Diagnosis is confirmed on finding histological evidence of irregular proliferation of the smooth muscle cells of the lungs. Treatment is not usually effective and there is rapid worsening of the pulmonary function leading to serious respiratory failure which often results in the patients death. We present two cases of LAM in women, one of whom was 33 and the other 38. In the first case the patient had all the pulmonary findings described in the literature, while the second also had bilateral renal angiomyolipomas. We discuss the histological, radiological and clinical characteristics and evolution of both cases, as well as the treatment given. We also review the literature. It is concluded that LAM should be suspected in young women who have dyspnea, cough and/or hemoptysis and an interstitial radiological pattern, especially when associated with a pleural effusion or pneumothorax.     

Pulmonary lymphangioleiomyomatosis in a menopaused woman

We report an unusual case of pulmonary lymphangioleiomyomatosis in a menopaused woman who had been taking estrogen hormone replacement therapy for several years. The characteristic feature of this uncommon disease is a proliferation of non-tumoral abnormal smooth muscle cells within the alveolar walls, and around the bronchi, lymph nodes and blood vessels. About twenty cases of pulmonary lymphangioleiomyomatosis have been described in menopaused women, who generally were taking estrogen hormone replacement therapy. This subpopulation does not appear to present any particular clinical, functional or radiographic features.     

Pulmonary lymphangioleiomyomatosis: an often fortuitous diagnosis. A case report]

INTRODUCTION: Lymphangioleiomyomatosis is an uncommon disorder of unknown origin, which exclusively occurs in women of reproductive age. The condition is characterized by proliferation of immature smooth muscle cells throughout the lungs, i.e., in the peribronchial, perilymphatic, and perivascular areas. This results in obliteration of the respiratory tract and in the development of cysts. Lymphangioleiomyomatosis has a poor prognosis due to both numerous lung complications and progression of the disease to respiratory failure. EXEGESIS: We report the case of a patient in whom lymphangioleiomyomatosis was fortuitously diagnosed from chest CT scan, itself performed for the diagnosis of pulmonary embolism. This case is therefore of particular interest. CONCLUSION: Our results suggest that the prevalence of lymphangioleiomyomatosis is probably underestimated due to its clinical latency and the absence of specific laboratory tests. Therefore, the development of non-invasive radiological methods should permit early diagnosis of the disease.     

肺曲菌球手术治疗(附10例报告)

本文报告10例肺曲菌球外科手术治疗的经验。由于抗菌素，激素和免疫抑制剂的广泛应用，肺曲菌球的发病率有逐渐增加的趋势。本组病例的主要临床症状是咳血，有典型的肺曲菌球X线表现者4例，其余6例均被误诊并行抗结核治疗数年。内科治疗对肺曲菌球无明显疗效。当频繁大量咳血，不能除外恶性或手术有助于原有基础病变的治疗，则应行外科处理。一般多行肺叶切除，偶而局部肺切除或病灶摘除也可获满意疗效。