A 3-Step Therapeutic Strategy for Severe Alveolar Proteinosis

Noirez, Leslie; Koutsokera, Angela; Pantet, Olivier; Duss, François-Régis; Eggimann, Philippe; Tozzi, Piergiorgio; Gonzalez, Michel; Coronado, Marcos; Nicod, Laurent P.; Lovis, Alban

doi:10.1016/j.athoracsur.2014.12.080

Cited by 4 publications

(8 citation statements)

References 7 publications

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“…Abdelmalak et al published [ 2 ] their experience with high volume wash (up to 50 L in very severe cases) without the need for ECMO and discussed others´approach utilizing hyperbaric oxygen. Noirez et al presented [ 3 ] a 3-step strategy that was used in a patient with PAP-associated refractory hypoxemia and that combined venovenous extracorporeal membrane oxygenation, double-lumen orotracheal intubation, and bilateral multisegmental sequential lavage. In compliance with current literature [ 2 , 3 ] the oxygenation and ventilation was assured by v-v ECMO and double-lumen orotracheal intubation during the procedure.…”

Section: Discussionmentioning

confidence: 99%

“…Noirez et al presented [ 3 ] a 3-step strategy that was used in a patient with PAP-associated refractory hypoxemia and that combined venovenous extracorporeal membrane oxygenation, double-lumen orotracheal intubation, and bilateral multisegmental sequential lavage. In compliance with current literature [ 2 , 3 ] the oxygenation and ventilation was assured by v-v ECMO and double-lumen orotracheal intubation during the procedure. The oxygenation improved dramatically on v-v ECMO in our patient.…”

Section: Discussionmentioning

confidence: 99%

“…The first WLL was performed by Ramirez in 1963 [ 7 ]. WLL can be performed with or without ECMO [ 2 , 3 , 8 ]. The WLL procedure could be accompanied by derangements of the acid–base and electrolyte balance and hypervolemia [ 2 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…There is a variable interindividual progression of this disease which can range from spontaneous recovery to terminal cardiorespiratory failure. Diagnosis is based on the results from bronchoalveolar lavage (BAL), histology and immunohistochemical tests, on the presence of granulocyte-macrophage colony stimulating factor (GM-CSF) antibodies and computer tomography (CT) or high resolution computer tomography (HRCT) results which typically shows areas of patchy ground-glass opacification and interlobular thickening, which together produce the “crazy paving pattern” [ 2 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

“…This can be performed on individual lung segments, lobes or on the whole lung with possibility for longer pauses between each lavage treatment. The whole-lung lavage (WLL) has been described as the most effective method of treatment [ 2 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

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Extracorporeal membrane oxygenation to support repeated whole-lung lavage in a patient with pulmonary alveolar proteinosis in life threatening dyspnoe – a case report

et al. 2015

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BackgroudPulmonary alveolar proteinosis is a rare disorder that is characterized by a large accumulation of lipoproteinaceous material within the alveoli. This causes respiratory failure due to a restriction of gas exchange and changes in the ventilation/perfusion ratio. Clinical symptoms are variable and depend on the severity of damage of the lung parenchyma. Treatment method is whole-lung lavage, where the accumulated lipoproteinaceous material is removed using large quantities of saline.Case presentationThis case report describes a 45 year old patient with advanced pulmonary alveolar proteinosis. Due to the presence of severe global respiratory insufficiency, this patient could not undergo the classic whole-lung lavage using a double-lumen tube and selective lung ventilation. The whole-lung lavage was performed with the support of veno-venous extracorporeal membrane oxygenation. A total of 27 l of warm saline was used.ConclusionAccording to the current published literature, whole-lung lavage with extra-corporeal membrane oxygenation support is a very rare treatment method. Even when taking into account all of the risks associated with whole-lung lavage and v-v extracorporeal membrane oxygenation support, we found that this technique is very effective and, without a doubt, it saved the life of our patient.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Extracorporeal membrane oxygenation to support repeated whole-lung lavage in a patient with pulmonary alveolar proteinosis in life threatening dyspnoe – a case report

et al. 2015

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Characteristics of hospital admissions for pulmonary alveolar proteinosis: analysis of the nationwide inpatient sample (2012–2014)

et al. 2022

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Background Pulmonary alveolar proteinosis (PAP) is a rare clinical syndrome involving the accumulation of lipid-rich proteinaceous material in the alveoli. There is a paucity of published studies on this condition. To better characterize the demographics, complication rates, mortality, and healthcare costs of patients hospitalized for PAP in the United States, a secondary analysis on the Hospital Cost and Utilization Project’s Nationwide Inpatient Sample (NIS) was performed on patients admitted from 2012 to 2014 with a diagnosis of pulmonary alveolar proteinosis. Methods Using the NIS database, a secondary analysis was performed on 500 admissions with the diagnosis “pulmonary alveolar proteinosis.” The clinical variables and outcome measures extracted were: patient demographics, hospital costs, length of stay, frequency of admissions, and inpatient mortality rate. Results Among a weighted estimate of 500 hospital admissions from 2012 to 2014, the number of PAP admissions averaged 4.7 per million. The population was predominantly male (55%) with a mean age of 41.45 (CI 38.3–44.5) from all socioeconomic levels. Inpatient mortality was calculated to be 5%, which may result from the fact that the majority of admitted patients had few or no comorbid conditions (CCI 0.72). The most common procedure performed during admission was a bronchoalveolar lavage. Mean length of stay was 6.2 days (CI 3.9–8.5) and average cost of admission was $29,932.20 (CI 13,739–46,124). Of note, 50% of these admissions were considered “elective.” Conclusions Demographics of patients with PAP who have been hospitalized in the United States are similar to previously reported demographics from prior patient cohorts, specifically a male predominance and a mean age in the 40 s. The inpatient mortality rate of 5% we found is consistent with prior studies demonstrating good disease-specific survival rates. Notably, the cost per admission and overall annual cost associated with PAP hospitalization was calculated to be $29932.20 and $5 million respectively. This reflects the high economic cost associated with hospitalization of PAP patients, and provokes thought about ways to make treatment more cost-effective.

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Novel method of noninvasive ventilation supported therapeutic lavage in pulmonary alveolar proteinosis proves to relieve dyspnea, normalize pulmonary function test results and recover exercise capacity: a short communication

et al. 2018

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Whole lung lavage (WLL) under general anesthesia with a double-lumen endobronchial intubation has remained standard treatment option for pulmonary alveolar proteinosis (PAP) for over fifty years now. To the best of our knowledge, this is the first description of noninvasive ventilation (NIV) as an innovative alternative, which enables safe and effective treatment. NIV support enabled cost-effective lavage of the most affected segments and resulted in restoration and long-term maintenance of exercise capacity and diffusion, without WLL related hypoxaemia, volume overload, intubation, or mechanical ventilation related complications. The study presents all details of performed procedure, including anesthesia, NIV technique and bronchoscopy, therefore this may be easily implemented into clinical practice at other centers conducting PAP treatment. We assume that presented technique of therapeutic lung lavage (TLL) with NIV support may be considered a novel PAP treatment method, however, target population who will benefit the most from such therapy modification must be assessed in large scale prospective trials. (2). This method provides long-lasting beneficial effects. It is usually performed unilaterally, with the patient in a supine position. The lavaged lung is filled up to functional residual capacity (FRC), and cycles of saline instillation and subsequent drainage are repeated, until clear effluent is obtained. Taking into account WLL methodology, it facilitates severe hypoxemia by elicitation of extensive V/Q mismatch due to shunt through the treated lung and respiration conducted by the contralateral lung, which interstitial tissue is often heavily affected by present disease. This mechanism sometimes creates a need for extracorporeal membrane oxygenation ECMO support (3). Moreover, uneven alveolar distribution in the lavaged lung may promote post procedure pulmonary edema which is probably responsible for the demand for prolonged ventilation and oxygen supplementation (4,5). Obviously, due to low incidence of the disease, no gold standard WLL protocols have been developed as yet.The major indications for WLL in centers caring for PAP patients worldwide include radiographic progression, lung function deterioration and hypoxemia (4).WLL, although considered as reasonably safe and efficient if performed in well-experienced specialized centers, has serious limitations, which include post procedure complications, the most common being fever, hypoxemia, wheezing, pneumonia and saline leakage due to malposition of the endotracheal tube (4,6). Also pneumothorax, pleural effusion, cardiac arrest (4) and one case of WLL-associated death (7) were reported. There is a possibility that mortality rate is under reported.Hypoxemia usually occurs during the drainage phase, because blood is again redirected from the ventilated lung to the contralateral lavaged one and a decrease in airway pressure occurs, leading to perfusion of the lung undergoing BAL and thus a fall in partial oxygen pressure PaO 2 (4).Last but no...

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A 3-Step Therapeutic Strategy for Severe Alveolar Proteinosis

Cited by 4 publications

References 7 publications

Extracorporeal membrane oxygenation to support repeated whole-lung lavage in a patient with pulmonary alveolar proteinosis in life threatening dyspnoe – a case report

Extracorporeal membrane oxygenation to support repeated whole-lung lavage in a patient with pulmonary alveolar proteinosis in life threatening dyspnoe – a case report

Characteristics of hospital admissions for pulmonary alveolar proteinosis: analysis of the nationwide inpatient sample (2012–2014)

Novel method of noninvasive ventilation supported therapeutic lavage in pulmonary alveolar proteinosis proves to relieve dyspnea, normalize pulmonary function test results and recover exercise capacity: a short communication

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