Case Of Pulmonary Alveolar Proteinosis Research Articles

Clinical case of pulmonary alveolar proteinosis in a newborn

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of protein-lipid complexes in the alveoli due to impaired utilization of surfactant by alveolar macrophages; it is most often idiopathic and occurs in healthy men and women aged 30–50 years. The congenital form of PAP is extremely rare, its diagnosis is complicated by the fact that it is impossible to conduct functional lung tests in newborns, and lung biopsy is used extremely rarely. Against this background, data obtained by computed tomography (CT) of the lungs of newborns are of particular importance. The article presents a clinical case of treatment of a newborn with respiratory failure (RF) and signs of pulmonary hypertension, who was admitted to the neonatal intensive care unit of the Children’s City Multidisciplinary Clinical Specialized Center for High Medical Technologies at the age of the first day of life. The article reflects the role of computed tomography in diagnosing the causes of respiratory failure in newborns, as well as the importance of anamnesis and a thorough clinical examination of patients. The presented clinical case demonstrates the need for a multidisciplinary approach to the diagnosis of newborns with pulmonary alveolar proteinosis.

Pulmonary Alveolar Proteinosis: Conducting the first Whole Lung Lavage in Nepal

A case of Pulmonary Alveolar Proteinosis, probably secondary in etiology, was diagnosed at our institute based on clinical, radiological, and pathological evidence. Patient was planned for conducting whole lung lavage as per the guidelines. With this case, we report the first instance of conducting Whole lung lavage in Nepal and the logistics involved.

Rescue of Pap-Mas in Systemic JIA Using Janus Kinase Inhibitors, Case Report and Systematic Review.

Biological disease-modifying anti-rheumatic drugs (bDMARDs) targeting interleukin (IL)-6 and IL-1β represent a steroid-sparing first-line therapy used in systemic-onset juvenile idiopathic arthritis (sJIA). Recently, the occurrence of pulmonary alveolar proteinosis (PAP) in sJIA patients was reported with early-onset and exposure to bDMARDs as potential risk factors. We report on a new case with longitudinal immunomonitoring successfully treated by Janus Kinase inhibitors (JAKi) and review past clinical descriptions of this new entity. We report one case of pulmonary alveolar proteinosis and macrophage activation syndrome (PAP-MAS) with longitudinal immunomonitoring. We then conducted a review of the literature of seven publications reporting 107 cases of PAP-MAS sJIA, and included the main characteristics and evolution under treatment. Of the seven articles analyzed, the incidence of PAP-MAS among sJIA patients varied from 1.28% to 12.9%. We report here a single case among a cohort of 537 sJIA patients followed in the pediatric department of the Hospices Civils de Lyon over the last 15 years. This child presented with all clinical and immunological characteristics of PAP-MAS. After several lines of treatment, he benefited from JAKi and improved with respect to both systemic symptoms and lung disease. In the literature, strategies with monoclonal antibodies targeting either INF-γ or IL-1β/IL-18 have been tested with variable results. Orally taken JAKi presents the advantage of targeting multiple cytokines and avoiding parenteral injections of monoclonal antibodies that may contribute to the pathogenesis. JAKi represent a promising option in the treatment of lung disease associated with sJIA.

Anesthetic management of a case of pulmonary alveolar proteinosis for whole lung lavage

Abstract Pulmonary Alveolar Proteinosis (PAP) is a rare disease of the lungs characterized by deposition of lipo-proteinaceous material in the alveoli. This leads to reduced lung compliance and V/Q mismatch, causing respiratory failure. Patients with PAP may be posted for Whole Lung Lavage (WLL) under anesthesia, which is a challenging case for anesthetists. Preexisting respiratory failure is complicated by requirement of single lung ventilation with the help of double lumen tube during the procedure. Intraoperative ventilation is difficult and is managed with blood gas and airway pressure monitoring. Some degree of hypoxemia and hypercapnia may have to be tolerated. Large volumes of fluid are instilled into the lung which may cause hypothermia, hypervolemia, and electrolyte disturbances. These patients frequently require elective ventilation and intensive care admission in the postoperative period. We report the case of a young woman with PAP, posted for unilateral WLL under anesthesia. We wish to highlight the possible challenges posed by this case and how to conduct a safe anesthesia for this patient.

Case Report: The first reported case of pulmonary alveolar proteinosis with myasthenia gravis in a 27-year-old patient

Background: Pulmonary alveolar proteinosis is a very rare diffuse lung disease characterized by the accumulation of amorphous and periodic acid Schiff-positive lipoproteinaceous material in the alveolar spaces due to impaired surfactant clearance by alveolar macrophages. Three main types were identified: Autoimmune, secondary and congenital. Pulmonary alveolar proteinosis has been previously reported to be associated with several systemic auto-immune diseases. Accordingly, we present the first case report of pulmonary alveolar proteinosis associated with myasthenia gravis. Case: A 27-year-old female patient, ex-smoker, developed a dyspnea on exertion in 2020. The chest X-ray detected diffuse symmetric alveolar opacities. Pulmonary infection was ruled out, particularly COVID-19 infection. The chest scan revealed the “crazy paving” pattern. The bronchoalveolar lavage showed a rosy liquid with granular acellular eosinophilic material Periodic acid-Schiff positive. According to the lung biopsy results, she was diagnosed with pulmonary alveolar proteinosis. The granulocyte macrophage colony-stimulating factor autoantibodies were negative. Nine months later, she was diagnosed with bulbar seronegative myasthenia gravis, confirmed with the electroneuromyography with repetitive nerve stimulation showing significant amplitude decrement of the trapezius and spinal muscles. She was treated with pyridostigmine, oral corticosteroids and azathioprine. Given the worsening respiratory condition of the patient, a bilateral whole lung lavage was performed with a partial resolution of symptoms. Thus, this previously unreported association was treated successfully with rituximab, including improvement of dyspnea, diplopia and muscle fatigability at six months of follow-up. Conclusions: This case emphasizes on the possible association of auto-immune disease to PAP, which could worsen the disease course, as the specific treatment does not exist yet. Hence, further studies are needed to establish clear-cut guidelines for PAP management, particularly when associated to auto-immune diseases.

Pulmonary Alveolar Proteinosis Related to Chronic Cotton Dust Exposure and Hepatitis-C Infection

A case of pulmonary alveolar proteinosis developing in a patient with chronic dust exposure and hepatitis-C infection is reported. He was managed with modified bronchoalveolar lavage and granulocyte-monophase-colony stimulating factor.

A Case of Pulmonary Alveolar Proteinosis in which a New Approach to the Traditional Whole Lung Lavage Technique is Preformed

A Case of Pulmonary Alveolar Proteinosis in which a New Approach to the Traditional Whole Lung Lavage Technique is Preformed

Diagnostic value and safety of medical thoracoscopy under local anesthesia for unexplained diffuse interstitial lung disease: A retrospective study.

ObjectiveWe aimed to explore the safety and diagnostic value of medical thoracoscopic lung biopsy in patients with unexplained diffuse interstitial lung disease (ILD) in a single center pilot study.MethodWe retrospectively analyzed clinical and pathological diagnostic data from 52 patients with diffuse ILD undergoing medical thoracoscopic lung biopsy.ResultsForty-four cases of diffuse ILD were confirmed pathologically, giving a diagnostic rate of 84.6%. Among these 44 patients, 11 patients were diagnosed with cancer, including eight patients with lung adenocarcinoma, three patients with metastases; two from a gastrointestinal malignancy, and one from a granulosa cell tumor of the ovary. There were 17 cases of idiopathic interstitial pneumonia, including nine cases of usual interstitial pneumonia (UIP), four cases of non-specific interstitial pneumonia (NSIP), three cases of cryptogenic organizing pneumonia (COP), and one case of acute interstitial pneumonia (AIP). There were 12 cases of rare interstitial pneumonias, which included six cases of pulmonary alveolar proteinosis, one case each of pulmonary Langerhans cell histiocytosis (LCH) and pulmonary lymphangiomyomatosis, two cases of nodular sarcoidosis, and two cases of chronic eosinophilic pneumonia. We recorded various complications, including bleeding, infection, and pneumothorax. A total of 28 patients (53.8%) experienced at least one of the above complications, but there were no deaths associated with biopsy.ConclusionsMedical thoracoscopic lung biopsy appears a safe and effective method for diagnosing diffuse ILD of unknown cause but further prospective studies, with larger numbers, including comparison with other established techniques are required.

P1‐56: A case of pulmonary alveolar proteinosis with severe respiratory failure improved by segmental lung lavage with fiberoptic bronchoscopy under general anesthesia

P1‐56: A case of pulmonary alveolar proteinosis with severe respiratory failure improved by segmental lung lavage with fiberoptic bronchoscopy under general anesthesia

OP0059 MAVRILIMUMAB (ANTI GM-CSF RECEPTOR Α MONOCLONAL ANTIBODY) REDUCES RISK OF FLARE AND INCREASES SUSTAINED REMISSION IN A PHASE 2 TRIAL OF PATIENTS WITH GIANT CELL ARTERITIS

Background:T helper (Th)1 and Th17 lymphocytes play a role in the pathogenesis of giant cell arteritis (GCA). Current treatments primarily target the Th17 axis, possibly leaving residual Th1 activity. Granulocyte macrophage colony stimulating factor (GM-CSF), a mediator of Th1 and Th17 cells, is a pathogenic factor in GCA.Objectives:To evaluate the efficacy and safety of the GM-CSF inhibitor mavrilimumab in patients with GCA.Methods:Randomized, double-blind, placebo-controlled phase 2 trial enrolling patients with active, biopsy- or imaging-proven new onset (N/O) or relapsing refractory (R/R) GCA. Active disease: GCA symptoms and erythrocyte sedimentation rate (ESR) (>30 mm/hr) and/or C-reactive protein (CRP) (≥1 mg/dL) elevation within 6 weeks from randomization. Corticosteroid-induced remission (resolution of GCA symptoms and CRP <1 mg/dL or ESR <20 mm/hr) was required by baseline. 3:2 randomization to mavrilimumab 150 mg or placebo subcutaneously every 2 weeks and protocol-defined 26-week prednisone taper starting at 20-60 mg/day.Primary efficacy endpoint: time to first adjudicated flare (ESR ≥30 mm/hr and/or CRP ≥1 mg/dL and GCA symptoms or new/worsening vasculitis on imaging) by Week 26 in all treated patients. Key secondary endpoint: sustained remission through Week 26. Safety up to Week 38 was assessed.Results:70 patients (35 N/O, 35 R/R) were enrolled (mavrilimumab [N=42] or placebo [N=28]). Mean (SD) age was 69.7 (7.48) years and 71.4% were female. Flare by Week 26 occurred in 8 (19%) and 13 (46.4%) patients receiving mavrilimumab and placebo, respectively (27.4 percentage points reduction). Median time to flare by Week 26 could not be estimated in the mavrilimumab group due to too few events (Not Estimable) and was 25.1 weeks [95% CI: (16.0, NE)] in the placebo group (HR [95% CI] 0.38 [0.15, 0.92]; p=0.0263) (Figure). Sustained remission at Week 26 occurred in 83.2% of patients receiving mavrilimumab and 49.9% of those receiving placebo (33.4 percentage points increase; p=0.0038). Results were consistent across disease type subgroups (HR for flare: N/O 0.29 [95% CI: 0.06, 1.31; nominal p= 0.0873]; R/R 0.43 [95% CI: 0.14, 1.30]; nominal p=0.1231), although not powered for significance (Table). Adverse events (AEs), mostly mild to moderate, were comparable between groups. There were 5 serious AEs (mavrilimumab 2 [4.8%], placebo 3 [10.7%]), none drug-related. No deaths or vision loss occurred. No adjudicated cases of pulmonary alveolar proteinosis were observed.Table 1.Efficacy at Week 26All Patients [1]SubgroupsN/OR/RMavrilimu-mab (N=42)Placebo (N=28)Mavrilimu-mab (N=24)Placebo (N=11)Mavrilimu-mab (N=18)Placebo (N=17)Patients with Flare, n (%)8 (19.0)13 (46.4)3 (12.5)4 (36.4)5 (27.8)9 (52.9)Time to Flare (weeks) [2]Median, 95% CINE (NE, NE)25.1 (16.0, NE)NE (NE, NE)NE (11.7, NE)NE (16.4, NE)22.6 (16.0, NE)HR (Mavrilimumab vs Placebo), 95% CI [3]0.38 (0.15, 0.92)0.29 (0.06, 1.31)0.43 (0.14, 1.30)P-value [4] [5]0.02630.08730.1231Sustained Remission (%), 95% CI [6]83.2 (67.9, 91.6)49.9 (29.6, 67.3)91.3 (69.3, 97.7)62.3 (27.7, 84.0)72.2 (45.6, 87.4)41.7 (17.4, 64.5)Difference in Proportions (95% CI) [7]33.3 (10.7, 55.8)28.9 (-2.7, 60.5)30.6 (-2.1, 63.2)P-value [5] [7]0.00380.07270.0668NE = Not estimable. [1] Total mITT population. Stratified by randomization strata. [2] Kaplan-Meier. [3] Cox proportional-hazards model; treatment as covariate. [4] Log-rank test. [5] N/O and R/R subgroups not powered for significance; nominal p values reported. [6] Kaplan-Meier Survival Estimates with standard error. [7] Two-sided p-value for the difference in sustained remission between 2 arms using normal approximation. Placebo arm is reference.Conclusion:Mavrilimumab was superior to placebo on the primary and secondary efficacy endpoints of time to flare and sustained remission at week 26 in patients with GCA. Mavrilimumab was well tolerated, and no new safety signals were observed.Disclosure of Interests:Maria C. Cid Speakers bureau: meeting attendance support from Roche and Kiniksa, Paid instructor for: educational from GSK and Vifor, Consultant of: consulting for Janssen, GSK, and Abbvie, Grant/research support from: research grant from Kiniksa, Sebastian Unizony Consultant of: consulting for Janssen and Kiniksa, Grant/research support from: research support from Genentech, Lara Pupim Shareholder of: Kiniksa Pharmaceuticals, Employee of: Kiniksa Pharmaceuticals, Fang Fang Shareholder of: Kiniksa Pharmaceuticals, Employee of: Kiniksa Pharmaceuticals, Joseph Pirrello Shareholder of: Kiniksa Pharmaceuticals, Employee of: Kiniksa Pharmaceuticals, Ai Ren Shareholder of: Kiniksa Pharmaceuticals, Employee of: Kiniksa Pharmaceuticals, Manoj Samant Shareholder of: Kiniksa Pharmaceuticals, Employee of: Kiniksa Pharmaceuticals, Teresa Zhou Shareholder of: Kiniksa Pharmaceuticals, Employee of: Kiniksa Pharmaceuticals, John F. Paolini Shareholder of: Kiniksa Pharmaceuticals, Employee of: Kiniksa Pharmaceuticals

An Unusual Case of Secondary Pulmonary Alveolar Proteinosis in Recently Diagnosed Anti-MDA5 Positive Amyopathic Dermatomyositis

Amyopathic dermatomyositis (AMD) is a subtype of dermatomyositis characterized by more prominent involvement of the skin rather than muscle and often positive for melanoma differentiation-associated gene 5 (MDA-5) antibodies. The most frequent pulmonary involvement in MDA-5 positive AMD is nonspecific interstitial pneumonia. However, rare cases of pulmonary alveolar proteinosis (PAP) have also been reported. Here, we present a case of a 28-year-old male who was recently diagnosed with AMD presenting with shortness of breath and dry cough was eventually diagnosed with secondary pulmonary alveolar proteinosis. This case underscores the multidisciplinary approach and diagnostic challenges associated with the diagnosis of PAP secondary to the rarity of the condition.

PULMONARY ALVEOLAR PROTEINOSIS IN MYELODYSPLASTIC SYNDROME SECONDARY TO GATA-2 MUTATION (MONOMAC SYNDROME)

SESSION TITLE: Medical Student/Resident Chest Infections Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: The majority of the cases of pulmonary alveolar proteinosis (PAP) occur in patients due to autoimmunity with the GM-CSF antibody. We present here a case of a 29-year-old female with GATA-2 mutation (monoMAC) syndrome who presented with dyspnea and was diagnosed with secondary PAP due to myelodysplastic syndrome. CASE PRESENTATION: A 29-year-old female presented to the hospital with the chief complaint of a nonproductive cough for 2 weeks associated with difficulty in breathing with exertion and intermittent fever. She had a history of heterozygous mutation for GATA-2 and was under treatment at a tertiary care center for early Myelodysplastic syndrome secondary to 9q34 deletion. Her medical history was extensive including Mycobacterium avium intracellulare (MAC) Pneumonia and bacteremia diagnosed 8 months before the presentation and was on azithromycin, ethambutol, and rifabutin for it to complete a total of 12 months of treatment. On presentation, she was febrile at 103 degrees Fahrenheit, tachycardiac at 135 beats per minute and saturating 88% on room air. She was placed on 4 L of oxygen by nasal cannula. Labs showed a WBC count of 8.4 10*3/uL (normal 4.0 - 12.0 10*3/uL) with differential showing a low monocyte count at <1 % (normal 2-8 %). Computed Tomography angiogram was obtained which ruled out a pulmonary embolism but showed diffuse and bilateral alveolar and interstitial pulmonary opacification. She had an extensive infectious, autoimmune, metabolic and cardiovascular workup which was all negative. So, bronchoscopy with bronchoalveolar lavage was obtained which showed an exudative effusion with 84 Polymorphonuclear cells, 2 Lymphocytes, 8 Histiocytes, 6 Eosinophils. BAL fluid was negative for respiratory viral PCR, AFB, fungal culture and bacterial culture, galactomannan and Pneumocystis culture. Cytology was also negative for pathogens or malignant cells. Post-Bronchoscopy the patient’s respiratory status improved, and she was weaned off of the oxygen. Pulmonary function tests (PFT) were also obtained there which revealed a non-specific ventilatory defect with decreased Diffusion lung capacity for Carbon monoxide (DLCO). A lung biopsy was obtained there confirming the diagnosis of pulmonary alveolar proteinosis (PAP). The patient had negative anti-GM-CSF antibodies indicating GATA-2 deficiency as the cause of PAP. The patient is scheduled to get a repeat full lung bronchoalveolar lavage and is currently being evaluated for a lung transplant. DISCUSSION: GATA-2 mutation is identified in cases of PAP secondary to hematological malignancies. Whole lung lavage is the standard of care. Treatment of the hematological malignancy may treat the PAP. There is no evidence of corticosteroids in the treatment of secondary PAP. CONCLUSIONS: Clinicians should be mindful of PAP in treating patients with hematological malignancies with respiratory distress. Reference #1: Ishii H, Seymour JF, Tazawa R, Inoue Y, Uchida N, Nishida A, et al. Secondary pulmonary alveolar proteinosis complicating myelodysplastic syndrome results in worsening of prognosis: a retrospective cohort study in Japan. BMC Pulm Med. 2014;14:37. Reference #2: Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med 2002; 166: 215–235. Reference #3: Milleron BJ, Costabel U, Teschler H, et al. Bronchoalveolar lavage cell data in alveolar proteinosis. Am Rev Respir Dis 1991; 144: 1330–1332. DISCLOSURES: No relevant relationships by Fazal Raziq, source=Web Response No relevant relationships by Muhammad Usama, source=Web Response

Mutations in MARS identified in a specific type of pulmonary alveolar proteinosis alter methionyl-tRNA synthetase activity.

Biallelic missense mutations in MARS are responsible for rare but severe cases of pulmonary alveolar proteinosis (PAP) prevalent on the island of La Réunion. MARS encodes cytosolic methionyl-tRNA synthetase (MetRS), an essential translation factor. The multisystemic effects observed in patients with this form of PAP are consistent with a loss-of-function defect in an ubiquitously expressed enzyme. The pathophysiological mechanisms involved in MARS-related PAP are currently unknown. In this work, we analyzed the effect of the PAP-related mutations in MARS on the thermal stability and on the catalytic parameters of the MetRS mutants, relative to wild-type. The effect of these mutations on the structural integrity of the enzyme as a member of the cytosolic multisynthetase complex was also investigated. Our results establish that the PAP-related substitutions in MetRS impact the tRNAMet -aminoacylation reaction especially at the level of methionine recognition, and suggest a direct link between the loss of activity of the enzyme and the pathological disorders in PAP.

A newly identified novel variant in the\xa0CSF2RA gene in a\xa0child with pulmonary alveolar proteinosis: a case report

BackgroundThe congenital form of pulmonary alveolar proteinosis due to colony stimulating factor 2 receptor alpha gene mutations is a rare disease with only a few cases reported worldwide. In this study we report a new case of pulmonary alveolar proteinosis with a novel variant in colony stimulating factor 2 receptor alpha gene.Case presentationA 5-year-old Saudi boy presented with a history of progressive dyspnea over 6 months; he was diagnosed as having pulmonary alveolar proteinosis. A molecular study revealed a novel variation in colony stimulating factor 2 receptor alpha gene. His clinical condition showed significant improvement after whole lung lavage.ConclusionsThis case has the typical presentation of congenital pulmonary alveolar proteinosis due to colony stimulating factor 2 receptor alpha defect with a novel variant in this gene likely to be pathogenic.

Pulmonary alveolar proteinosis and first successful whole lung lavage in Sri Lanka: a case report

BackgroundPulmonary alveolar proteinosis is a rare disease characterized by accumulation of lipoproteinaceous material within alveoli. There are three clinically distinct forms: congenital, acquired and secondary. Whole lung lavage is currently the gold standard therapy for severe cases of pulmonary alveolar proteinosis. In Sri Lanka this is the first reported successful whole lung lavage for a patient with pulmonary alveolar proteinosis.Case presentationWe describe the case of a 15-year-old Sri Lankan girl who presented with symptoms of progressive shortness of breath and dry cough for 6 months’ duration. She had a history of exposure to silica in her household environment. High-resolution computed tomography revealed crazy paving appearance in both lungs suggestive of pulmonary alveolar proteinosis. An open lung biopsy revealed intra-alveolar granular amphophilic material which was strongly periodic acid–Schiff positive and diastase resistant, which is consistent with pulmonary alveolar proteinosis. She was followed up for 2 years with periodical segmental bronchoalveolar lavages which showed minimal improvement in her symptoms as well as in exercise desaturation. Due to severe dyspnea and hypoxemia on exertion, she underwent whole lung lavage. It resulted in a marked improvement in her symptoms, exercise desaturation, and chest X-ray results.ConclusionWhole lung lavage was successfully performed for the first time in Sri Lanka for a patient with pulmonary alveolar proteinosis.

Particle-induced Pulmonary Alveolar Proteinosis and Subsequent Inflammation and Fibrosis: A Toxicologic and Pathologic Review.

This review analyzes the published data on cases of pulmonary alveolar proteinosis (PAP) in workers inhaling crystalline aluminum, indium, silicon, and titanium particles and possible sequelae, that is, inflammation and fibrosis, and compares these findings with those from animal experiments. In inhalation studies in rodents using crystalline indium and gallium compounds, pronounced PAP followed by inflammation and fibrosis down to very low concentration ranges have been reported. Crystalline aluminum, silicon, and titanium compounds also induced comparable pulmonary changes in animals, though at higher exposure levels. Laboratory animal species appear to react to the induction of PAP with varying degrees of sensitivity. The sensitivity of humans to environmental causes of PAP seems to be relatively low. Up to now, no cases of PAP, or other pulmonary diseases in humans, have been described for gallium compounds. However, a hazard potential can be assumed based on the results of animal studies. Specific particle properties, responsible for the induction of PAP and its sequelae, have not been identified. This review provides indications that, both in animal studies and in humans, PAP is not often recognized due to the absence of properly directed investigation or is concealed behind other forms of lung pathology.

自然軽快した肺胞蛋白症の 1 症例

自然軽快した肺胞蛋白症の 1 症例

気管支肺胞洗浄液細胞診にて診断した肺胞蛋白症 4 例の細胞学的特徴

背景 : 肺胞蛋白症 (PAP) は肺胞腔内, 終末気管支内にサーファクタント由来物質が異常貯留をきたす比較的まれな疾患である. 今回われわれは気管支肺胞洗浄液 (BALF) 細胞診で PAP と診断しえた 4 例の BALF の性状ならびに細胞学的所見を比較検討した.

Cytological and electron microscopic findings in a bronchoalveolar lavage sample from a case of pulmonary alveolar proteinosis with radiological correlation

Pulmonary alveolar proteinosis is a rare disease. The definitive diagnosis is usually made from lung biopsy or Broncho alveolar lavage (BAL) samples taken at bronchoscopy. In this case, transmission electron microscopy was performed on an aliquot of the BAL sample. This case illustrates that cytological analysis of BAL samples may be just as diagnostic as histological biopsy and TEM may be performed on BAL samples if adequate and immediate fixation is performed. Authors claim this, to the best of their knowledge, has not previously been reported.

A Case of Pulmonary Alveolar Proteinosis Undergoing Whole Lung Lavage in Combination with Extracorporeal Membrane Oxygenation

Pulmonary alveolar proteinosis (PAP) is a rare disease whose etiology is not completely known and is characterized by deposition of surfactant proteins and phospholipid-rich acid Schiff (PAS) (+) material in alveoli and bronchioles [1]. One third of the patients die because of progressive hypoxemia and secondary infections while a small proportion does not respond to treatment and subsequently fibrosis develops. In this report, a case of pulmonary alveolar proteinosis admitted to intensive care unit due to progressive hypoxemia and treated with bilateral TLL along with veno-arterial (v-a) extracorporeal membrane oxygenation (ECMO) is presented.