肺泡蛋白沉积症的X线与CT诊断(附4例报告并文献复习)

目的分析肺泡蛋白沉积症的X线与CT表现,提高对本病的认识。方法回顾性分析4例经X线与CT检查并经病理证实的肺泡蛋白沉积症,分析其X线与CT征象。结果4例病变中皆见到弥漫性斑片影与磨玻璃影,CT扫描病灶呈地图样分布。结论CT对肺泡蛋白沉积症的范围、程度及病变轮廓的显示较X线平片准确,根据CT和HRCT表现的典型特征,可以作出可靠的诊断。     

Coexisting Endogenous and Exogenous Llpold Pneumonia and Pulmonary Alveolar Proteinosis in a Patient with Neurodevelopmental Disease

We report a unique case of coexistingexogenous lipoid pneumonia, endogenous lipoid pneumonia (EU), and pulmonary alveolar proteinosis (PAP) in a 5-year-old patient with severe neurodevelop mental disease. The patient presented with gastroesophageal reflux and presumed chronic lung disease resulting from recurrent aspiration pneumonias and succumbed to respiratory failure. The autopsy showed lipid-laden macrophages and periodic acid-SchiJf9ositive granular matm'al i n alveolar spaces and multilamellated structures within both alveolar macrophages and extracellular debris.

These findings were similar to those in previous reports of coexisting E I P and PAP in the setting of gastroesophageal refux [I] . However, the present case diJfered by the presence of scattered large osmiophilic extracellular lipid vacuob. Besides strengthening the association between EIP and PAP and their relationship to gastroesophageal refux, this case suggests that t h q may arise together with exogenous lipoid pneumonia, through related mechanisms, in the setting of neurodevelopmental disease.     

肺泡蛋白沉着症影像学分析（附2例报告）

目的探讨肺泡蛋白沉着症(PAP)的影像特点及诊断。方法对2例经胸腔镜活检病理及肺泡灌洗证实的肺泡蛋白沉着症的胸片和CT表现进行分析。结果PAP胸部影像表现呈多样化，如地图样、碎石路样、肺实变、肺水肿样及肺间质纤维化样表现等。结论肺部影像表现可充分显示肺泡蛋白沉着症的特点，结合临床有助于确诊本病。     

A case of pulmonary alveolar proteinosis treated with whole lung lavage

Pulmonary alveolar proteinosis represents a rare syndrome characterized by the accumulation of proteinaceous phospholipid-laden material in the alveoli. This leads to impaired gas exchange and arterial hypoxemia of varying degrees. The diagnosis is confirmed by lung biopsy. Sequential whole-lung lavage (WLL) first described in 1963 is the standard of care. We report a case of a male diagnosed of having pulmonary alveolar proteinosis (PAP) on transbroncial lung biopsy (TBLB). He was treated with sequential WLL (Left followed by right, Left being more involved on chest X-ray) followed by recombinant GM-CSF, with good result.     

Ocular manifestations in lipoid proteinosis: A rare clinical entity

Lipoid proteinosis is a rare autosomal recessive genodermatosis with abnormal lipid protein complexes deposition in different parts of the body, especially in the skin and mucus membranes of the upper aerodigestive tract. Though ocular involvement in lipoid proteinosis is rare, ophthalmologists may encounter diverse ocular complications accompanying this syndrome in clinical practice. We describe a case of lipoid proteinosis involving bilateral eyelids with pathognomonic moniliform blepharosis in a 33-year-old gentleman who presented with the complaints of itching of eye lids on and off since 10 years.     

Progressive dyspnea associated with a crazy-paving appearance on a chest computed tomography scan

A ‘crazy-paving’ appearance of the lungs on computed tomography scanning of the chest was first described nearly 20 years ago in patients with pulmonary alveolar proteinosis, and was thought to be characteristic of this condition. However, this pattern has subsequently been reported in a variety of pulmonary diseases and is now considered to be nonspecific. The present report describes a case of a 74-year-old man in whom congestive heart failure presented with a crazy-paving appearance of the lungs on a chest computed tomography scan. This uncommon association illustrates the importance of the correlation of clinical and radiographic information.     

支气管镜肺活检在肺泡蛋白沉积症中的诊断价值

目的：评价支气管镜肺活检(transbronchial lung biopsy,TBLB)在肺泡蛋白沉积症(pulmonary alveolar proteinosis,PAP)中的诊断价值和限度。方法：对2006年6月至2012年12月在中南大学湘雅二医院住院、资料完整的 TBLB病理确诊和非TBLB诊断的PAP 25例及TBLB病理误诊为PAP 4例进行回顾性分析。结果：25例PAP患者中,14例 一次TBLB病理确诊[一次TBLB阳性率为56%(14/25)],一次TBLB阴性病例中6例再次TBLB确诊[二次TBLB阳性率为 24%(6/25)],总阳性率为80%(20/25),总阴性率为20%(5/25)。其中,5例经典型CT表现及支气管肺泡灌洗液检查确 诊,并经全肺灌洗证实;而同期TBLB病理检查将其他疾病误诊为PAP者4例。结论：TBLB是很好的诊断方法,但一 次TBLB阴性不能排除PAP,条件允许可重复进行;临床和CT表现典型结合肺泡灌洗液检查阳性可作为临床诊断依 据;不典型的病例需进行TBLB。     

Long-term follow-up and successful treatment of pulmonary alveolar proteinosis without hypercholesterolemia with statin therapy: a case report

Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by the accumulation of excessive surfactant lipids and proteins in alveolar macrophages and alveoli. Oral statin therapy is a novel treatment for PAP with hypercholesterolemia. However, this treatment has never been described in a patient without hypercholesterolemia. Here, we present a case of successful treatment with atorvastatin for a patient with possibly unclassified PAP without hypercholesterolemia who responded poorly to whole lung lavage therapy and inhaled granulocyte-macrophage colony-stimulating factor. After 18 months of atorvastatin treatment, the patient experienced improvements in dyspnea, radiographic abnormalities and pulmonary function. The present case study supports the feasibility of statin therapy for PAP regardless of the level of cholesterol.     

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.