[Lung transplantation for pulmonary alveolar proteinosis: a case report and literature review].

Objective: To observe the efficacy of lung transplantation for pulmonary alveolar proteinosis (PAP) patients and to improve the understanding of the therapy. Methods: The clinical data of a patient with autoimmune PAP treated with sequential homogenous bilateral lung transplantation were described and the literatures were reviewed. Results: This 55-year-old female patient was diagnosed with autoimmune PAP and had been treated with whole lung lavage for 19 times, but only achieved short-term symptomatic relief after each operation. Inhalation of granulocyte macrophage colony stimulating factor occurred allergic reactions. Lung transplantation was performed on February 15, 2022, and a significant improvement in oxygenation and clinical symptoms were observed. The patient remained stable during follow-up. Conclusion: Treatment with lung transplantation is safe and effective for end-stage patients with PAP in the early phase, but the long-term effect remains to be observed.

Lung Transplantation and Simultaneous Modified Ravitch Procedure.

Lung transplantation is an established treatment for a variety of end-stage lung diseases; however, chest wall deformities such as an asymmetric pectus excavatum are often considered a contraindication for lung transplantation. Consequently, the published experience of lung transplants and simultaneous chest wall reconstruction is limited to a few case reports. This article aims to provide a detailed description of surgical steps as well as technical challenges and pitfalls of lung transplantation with a simultaneous modified Ravitch procedure. Exemplary technical aspects will be discussed for a pediatric patient in whom such a combined procedure resulted in an excellent outcome.

Improvement of native pulmonary alveolar proteinosis after contralateral single living-donor lobar lung transplantation: A case report.

PAP is a rare disease characterized by the accumulation of surfactant materials in the alveolar spaces due to the imbalance of surfactant homeostasis (production and clearance). We herein report a case of an 8-year-old girl who developed PAP after BMT from her mother for the treatment of DBA. The anemia was improved by BMT; however, respiratory dysfunction due to graft-versus-host disease gradually progressed. She eventually underwent right single LDLLT from her mother when she was 14 years old. A pathological examination of the excised lung confirmed the finding of diffuse bronchiolitis obliterans and unexpectedly revealed widespread alveolar proteinosis. Interestingly, the GGO of her native left lung on chest X-ray was improved after LDLLT. We present the very unique clinical course of this patient and discuss the mechanisms underlying the development of PAP after BMT and its improvement after LDLLT from the same donor.

Anaesthetic considerations for whole lung lavage for pulmonary alveolar proteinosis.

Pulmonary alveolar proteinosis (PAP) is an uncommon lung disease characterized by excessive accumulation of pulmonary surfactant that usually requires treatment with whole-lung lavage. A 47-year-old female presented with history of dry cough and breathlessness for past 6months. Chest radiograph demonstrated bilateral alveolar shadows and high resolution computerized tomography thorax showed crazy paving pattern. Broncho-alveolar lavage (BAL) and transbronchial lung biopsy confirmed a diagnosis of PAP. Due to worsening hypoxemia and respiratory failure, wholelung lavage was planned and performed. Anaesthetic management involved integrated use of pre-oxygenation, complete lung isolation, one-lung ventilation with optimal positive end-expiratory pressure, vigilant use of positional manoeuvres, and use of recruitment manoeuvres for the lavaged lung. We have discussed valuable strategies for the anaesthetic management of patients undergoing this multifaceted procedure in a case of severe PAP. FUNDING: None declared.

Whole-lung Lavage and Pulmonary Alveolar Proteinosis: Review of Clinical and Patient-centered Outcomes.

OBJECTIVE: The objective of this retrospective review was to evaluate the perioperative and procedural management of patients with pulmonary alveolar proteinosis (PAP) who presented for whole-lung lavage (WLL). DESIGN: The records of all adult patients with PAP who underwent WLL between January 1, 1988 and August 20, 2017 were reviewed and pertinent demographic, preoperative, anesthetic, procedural, and postoperative data were recorded. SETTING: Large academic tertiary referral center. PARTICIPANTS: Forty patients with PAP underwent 79 WLL procedures. INTERVENTIONS: Patients with PAP undergoing WLL. MEASUREMENTS: Successful WLL, defined by visual clearing of lavage fluid, was completed in 91% of cases. Whole-lung lavage was terminated prematurely in 9% of cases (refractory hypoxia most common), while 8% of cases were found to have 30-day complications. There were no cases of intraoperative death, hemodynamic collapse, pneumothorax or hydrothorax, or need for emergent reintubation. Postoperative clinical follow-up at the authors' institution within 6 months of WLL showed 68% of patients reported improvement in symptoms and/or functional status. CONCLUSION: The authors here present a retrospective study describing the perioperative and procedural management of PAP patients undergoing WLL to help familiarize providers with the management of this population (Fig 1). The findings of this study outline a successful and consistent approach to WLL using a multidisciplinary team experienced in this procedure. Even in experienced hands, procedural complications and 30-day postoperative complications emphasize the risk in this complex patient population.

Patrón crazy paving en una mujer joven: hallazgo inesperado y tratamiento no tradicional en un caso de proteinosis alveolar pulmonar / Crazy paving computed tomography pattern in a young woman: unexpected findings and non-traditional treatment in a pulmonary alveolar proteinosis case

El patrón llamado 'crazy paving' en tomografia computada de tórax (TAC) puede deberse a diferentes condiciones siendo una de ellas la Proteinosis Alveolar Pulmonar (PAP), rara condición que puede llevar a insuficiencia respiratoria y a menudo, a la muerte. Presentamos el caso de una mujer joven con una historia de un año de evolución de disnea progresiva y tos seca que consultó por un cuadro de aparición brusca de fiebre, calofríos, malestar general y falla respiratoria hipoxémica severa (PaO2 = 51,9 mmHg con FiO2 = 0,50) en la cual la TAC de tórax mostraba un patrón de empedrado o 'crazy paving' que significó un desafío diagnóstico resuelto finalmente con una biopsia pulmonar quirúrgica que mostró una PAP. Ante el fracaso del tratamiento tradicional de Lavado Pulmonar Total (LPT) se usó una aproximación terapéutica novedosa consistente en una serie de 4 lavados lobares con un perfluorocarbono, Perflubron (PFC) bajo anestesia local seguido por 5 sesiones de Plasmaféresis. Casi inmediatamente después de este tratamiento la paciente evidenció mejoría radiológica y funcional. La PaO2 fue de 89,9 mmHg respirando aire ambiental y la CVF y el VEF1 aumentaron alcanzado respectivamente el 77 y el 75% de sus valores normales de referencia. Dadas las características químicas y físicas del PFC, pensamos que es una alternativa válida al LPT en estos casos.

Crazy paving computed tomography pattern may be due to a number of causes, one of them being Pulmonary Alveolar Proteinosis, a rare condition leading to respiratory failure and often to death. We present the case of a young woman with a one-year history of progressive dyspnea and dry cough, who consulted for an acute onset of fever, chills, malaise and severe hypoxemic respiratory failure (PaO2 = 51.9 mmHg; FiO2 = 0.50) with a 'crazy paving' pattern on chest CT. This diagnostic challenge was resolved by a surgical lung biopsy that showed a pulmonary alveolar proteinosis. Taking into account that the traditional treatment using whole lung lavage had already failed in this patient, a novel therapeutic approach was settled. A series of 4 lobar lavages with a perfluorocarbon (Perflubron) under local anesthesia followed by 5 plasmapheresis sessions were carried out. The patient showed radiographic and functional improvement almost immediately after this treatment. PaO2 was 89.9 mmHg breathing room air and FVC and FEV1 increased to reach 77 and 75% respectively of their normal reference values. Because of its chemical and physical properties we think this novel therapeutic approach should be a valuable alternative to saline solution for whole lung lavage in these cases.

[Pulmonary Alveolar Proteinosis Observed in the Subpleural Area;Report of a Case].

A 39-year-old man consulted our hospital because of an abnormal shadow on a chest X-ray without any symptoms. A chest computed tomography revealed patchy peripheral ground-glass attenuation, in the subpleural area. Bronchoalveolar lavage fluid was clear and transbronchial lung biopsy findings were inconclutive. A video-assisted thoracic surgery-biopsy was performed. The specimens demonstrated accumulation of proteinaceous materials within alveolar spaces. The patient was given a diagnosis of pulmonary alveolar proteinosis.

A novel surfactant protein C L55F mutation associated with interstitial lung disease alters subcellular localization of proSP-C in A549 cells.

BACKGROUND: Heterozygous mutations of SFTPC, the gene-encoding surfactant protein C (SP-C), result in interstitial lung disease (ILD). However, characterization of mutations located in the mature domain of precursor SP-C (proSP-C) is limited. This study examined the molecular pathogenesis of such a mutation of ILD. METHODS: We employed sequencing of SFTPC and established A549 cells stably expressing several proSP-C mutants. Histopathology and transmission electron microscopy (TEM) of lung tissue from a pediatric patient with ILD were assessed. Effects of mutant proSP-C were evaluated by western blotting, immunofluorescence, and TEM. RESULTS: Sequencing of SFTPC revealed a novel heterozygous mutation, c.163C>T (L55F). In lung tissue, abnormal localization of proSP-C was observed by immunohistochemistry, and small and dense lamellar bodies (LBs) in type II alveolar epithelial cells (AECs) were detected by TEM. TEM of A549 cells stably expressing proSP-C(L55F) displayed abnormal cytoplasmic organelles. ProSP-C(L55F) exhibited a band pattern similar to that of proSP-C(WT) for processed intermediates. Immunofluorescence studies demonstrated that proSP-C(L55F) partially colocalized in CD63-positive cytoplasmic vesicles of A549 cells, which was in contrast to proSP-C(WT). CONCLUSION: We detected a novel c.163C>T mutation located in the mature domain of SFTPC associated with ILD that altered the subcellular localization of proSP-C in A549 cells.

A case of autoimmune pulmonary alveolar proteinosis appearing as a localized ground-glass opacity.

Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease caused by abnormal intra-alveolar surfactant accumulation; it commonly appears as a "crazy-paving" pattern on high-resolution computed tomography. Here, we report a rare case of autoimmune PAP appearing as localized ground-glass opacity. An 82-year-old woman underwent chest computed tomography (CT) at another facility for cough, and a 2-cm localized ground-glass opacity was detected at the bottom of the right upper lung lobe. When she presented for follow-up at our hospital 6 months later, she was asymptomatic. The CT examinations performed at that point and 2 months thereafter did not reveal any changes. However, a CT examination performed after 5 months revealed slight increases in size and concentration. Adenocarcinoma in situ or minimally invasive adenocarcinoma was suspected. Incomplete lobulation between the upper and middle lobes of the right lung was detected, and video-assisted thoracoscopic lobectomy of the upper lobe and partial resection of the middle lobe of the right lung were performed. Histological examination revealed alveoli and terminal bronchioles filled with eosinophilic proteinaceous material positive for periodic acid-Schiff stain. The histopathological diagnosis was PAP and positive serum anti-GM-CSF antibody findings confirmed autoimmune PAP.

Implant treatment followed by living donor lung transplant: a follow-up case report.

PATIENTS: Dental implant treatment in patients with complicated systemic disease has been discussed, especially in the context of achieving osseointegration. However, some patients with no pre-existing systemic disease develop it later, during their implant maintenance periods. Organ transplants, and the lifelong administration of immunosuppressants that follows, are also of relevance to post-implant oral health. Thus, strategies to maintain the health of peri-implant tissue in these patients should be considered. Here, we present the case of a patient receiving a living-donor lung transplant during her implant follow-up period. The condition of the lung is affected by that of the oral cavity, so the maintenance is of utmost importance. Throughout the follow-up period, we provided periodical professional maintenance care. DISCUSSION AND CONCLUSION: The patient experienced no complications, alterations in her radiographic findings, or worsening of periodontal indices, despite being extensively medicated with immunosuppressants, steroids and bisphosphonate.

An incident case of primary acquired pulmonary alveolar proteinosis.

This is a case of a primary acquired pulmonary alveolar proteinosis (PAP) in an asymptomatic patient, on the waiting list for kidney transplantation, confirmed on lung biopsy and by identifying anti-GM-CSF antibodies.

Transmission electron microscopy of sputum deposition in the diagnosis of pulmonary alveolar proteinosis.

OBJECTIVE: To clarify the diagnostic value of sputum in pulmonary alveolar proteinosis (PAP) through transmission electron microscopy (TEM) of sputum deposition (SD). METHODS: Eleven SD samples and 9 bronchoalveolar lavage (BAL) sediments from a PAP group including 11 patients were observed by TEM and compared with sputum direct smear, BAL cytology, and lung biopsy histopathology. Eleven healthy adults were chosen as controls. RESULTS: The 11 sputum smears from the PAP group showed no diagnostic component, but TEM of SD revealed 7 of 11 samples had many myelin-like lamellar bodies with degeneration in the cytoplasm of macrophages, alveolar epithelial cells, and extracellular spaces, which suggested PAP. Especially, 2 patients on whom lung biopsy could not be performed and who failed to be diagnosed by BAL fluid were finally diagnosed by TEM of SD. TEM of BAL sediments showed 7 of 9 cases had diagnostic myelin-like lamellar bodies. No statistical significance was found between BAL fluid and SD by TEM. The control group didn't show diagnostic components by cytology or TEM of SD. CONCLUSION: TEM of SD is an important noninvasive diagnostic method especially for patients against lung biopsy and BAL.

Intraoperative use of a chest physiotherapy system during whole lung lavage for pulmonary alveolar proteinosis.

Pulmonary alveolar proteinosis (PAP) is a rare condition caused by the excessive alveolar accumulation of surfactant proteins. The current standard of care for removing these secretions is through therapeutic whole lung lavage (WLL). We describe two successful cases of bilateral WLL involving the novel use of the Vest™ chest physiotherapy system thereby avoiding the need for extensive changes in patient position in the intraoperative period. In brief, it involves the induction of general anesthesia followed by single-lung ventilation while simultaneously performing large volume lavages on the nonventilated lung. The washout was enhanced using the Vest™ system.

Living-donor lobar lung transplantation for pulmonary alveolar proteinosis in an adult: report of a case.

A 43-year-old woman with pulmonary alveolar proteinosis (PAP) was successfully treated with living-donor lobar lung transplantation (LDLLT). The patient's PAP had been diagnosed at age 35. She had been treated with repeated bronchoalveolar lavage and granulocyte-macrophage colony-stimulating factor (GM-CSF) inhalation therapy despite having no serum anti-GM-CSF autoantibodies. At age 42, her respiratory condition became critical and she underwent transplantation from two donors. While careful observation was needed for the recurrence of PAP in the transplanted lungs, she was functioning well without oxygen therapy 1 year after transplantation. This appears to be the first report of LDLLT for PAP in an adult.

Anesthetic management of a patient with a double inferior vena cava and pulmonary alveolar proteinosis who underwent bilateral living-donor lobar lung transplantation.

A 43-year-old woman with pulmonary fibrosis secondary to pulmonary alveolar proteinosis was scheduled to undergo lung transplantation. Before the lung transplantation, she had undergone multiple whole-lung lavage procedures on extracorporeal circulation (ECC), which had caused scarring of the right femoral subcutaneous tissues. Preoperative examination revealed a double inferior vena cava (IVC) with interiliac communication, and the left IVC ended at the left renal vein. Surgical exposure of the right femoral vessels was performed immediately after anesthetic induction for emergent vascular access to establish an ECC. Cardiopulmonary collapse did not occur and the ECC was not required until lung resection. The lung transplantation was completed uneventfully. Congenital IVC anomaly is rare, but may make cannulation through the femoral vein difficult. Scarring of the subcutaneous tissue could result in a difficult "percutaneous" approach to the vessels. Evaluation of the vascular anatomy related to the establishment of an ECC is important before lung transplantation.

Update in nonneoplastic lung diseases.

CONTEXT: Nonneoplastic lung diseases include a wide range of pathologic disorders from asthma to interstitial lung disease to pulmonary hypertension. Recent advances in our understanding of the pathophysiology of many of these disorders may ultimately impact diagnosis, therapy, and prognosis. It is important for the practicing pathologist to be aware of this new information and to understand how it impacts the diagnosis, treatment, and outcome of these diseases. OBJECTIVE: To update current progress toward elucidating the pathophysiology of pulmonary alveolar proteinosis, idiopathic pulmonary hemosiderosis, and pulmonary arterial hypertension, as well as to present classification systems for pulmonary hypertension, asthma, and interstitial lung disease and describe how these advances relate to the current practice of pulmonary pathology. DATA SOURCES: Published literature from PubMed (National Library of Medicine) and primary material from the authors' institution. CONCLUSIONS: Improved understanding of the pathophysiology of pulmonary alveolar proteinosis, pulmonary hypertension, and idiopathic hemosiderosis may impact the role of the surgical pathologist. New markers of disease may need to be assessed by immunohistochemistry or molecular techniques. The classification systems for interstitial lung disease, asthma, and pulmonary hypertension are evolving, and surgical pathologists should consider the clinicopathologic context of their diagnoses of these entities.

Proteinosis alveolar pulmonar, caso clínico / Pulmonary alveolar proteinosis: case-report

La Proteinosis Alveolar Pulmonar es una patología poco frecuente, especialmente en la edad pediátrica, caracterizada por acumulación de material lipoproteináceo proveniente del surfactante en los macrófagos alveolares. Objetivo: Comunicar un caso de proteinosis pulmonar en el cual el uso de la fibrobroncoscopía (FB) permitió el diagnóstico y el tratamiento. Caso Clínico: Escolar de 7 años, que consulta por un cuadro febril, en la cual se plantea inicialmente el diagnóstico de neumonía. Ante la mala evolución, con aumento de las imágenes radiológicas de tipo nodular, el ascenso de los niveles de LDH en plasma, y la mínima sintomatología clínica respiratoria, se planteó el diagnóstico de proteinosis alveolar, realizando una FB con lavado alveolar. Las tinciones de Sudán y PAS confirmaron la sospecha diagnóstica, lo cual fue apoyado por la mejoría radiológica y clínica de la paciente. Discusión: Se discute las formas clínicas de presentación, los hallazgos clínicos, radiológicos y de laboratorio que permiten plantear el diagnóstico. Se destaca el rol de la fibrobroncoscopía como método diagnóstico y terapéutico.