Silicoproteinosis en paciente minero en Colombia. Reporte de caso / Silicoproteinosis in a mining patient in Colombia. Case report

La silicosis pulmonar es una enfermedad ocupacional que continúa ocasionando morbilidad en el mundo. Debido a que el sílice es el mineral más abundante en la tierra y en las rocas, son numerosas las fuentes de exposición laboral a la inhalación del polvo de sílice en varios sectores industriales. Por su parte, la silicoproteinosis pulmonar es una forma aguda muy rara de silicosis, que puede desarrollarse con un período de latencia más corto en comparación con la silicosis, luego de la primera exposición al sílice, y se caracteriza por un rápido deterioro de la función pulmonar, sin respuesta efectiva a ningún tratamiento. Por su forma de presentación tan atípica, reportamos el caso de un hombre de 58 años, con antecedente laboral de trabajo en mina de extracción de oro en socavón

Pulmonary silicosis is an occupational disease that continues to cause morbidity in the world. Because silica is the most abundant mineral in soil and rock, sources of occupational exposure to inhalation of silica dust are numerous in various industrial sectors. Alternately, pulmonary silicoproteinosis is a very rare acute form of silicosis, which can develop with a shorter latency period compared to silicosis after the first exposure to silica, and is characterized by a rapid deterioration of lung function, without effective response to any treatment. Due to its atypical form of presentation, we report the case of a 58-year-old man, with a history of working in a gold mine

Diagnóstico de proteinose alveolar pulmonar após exposição à covid-19: relato de caso / Pulmonary alveolar proteinosis diagnosis of after exposure to covid-19: case report / Diagnóstico de proteinosis alveolar pulmonar tras exposición al covid-19: reporte de caso

Os objetivos deste artigo são relatar o caso de um paciente diagnosticado com proteinose alveolar pulmonar (PAP) após piora do quadro clínico decorrente da infecção por covid-19 e realizar uma revisão sobre a PAP na busca de promover o maior conhecimento dos profissionais de saúde sobre essa comorbidade. Participou da pesquisa um paciente de 48 anos de idade, sexo masculino, com relato de quadro de tosse e dispneia há um ano, apresentando exacerbação do quadro respiratório após infecção por SARS-CoV-2, com necessidade de início de suporte de oxigênio via cateter nasal devido à diminuição da saturação e ao comprometimento das atividades da vida diária. Foi realizada tomografia computadorizada de alta resolução do tórax (TCAR) e evidenciada pavimentação em mosaico com comprometimento difuso dos campos pulmonares. Conforme achados clínicos e radiológicos compatíveis com PAP, o paciente foi submetido à lavagem pulmonar total e apresentou melhora da dispneia e da tosse. A proteinose alveolar pulmonar é uma doença que merece relevância devido ao comprometimento clínico que pode ocasionar ao paciente. É necessário um acompanhamento médico periódico para observar a evolução da doença e definir a conduta terapêutica conforme apresentação clínica de cada enfermo. Por vezes, pode ser necessário repetir a lavagem pulmonar total e em casos refratários, buscar alternativas terapêuticas. Tendo em vista o envolvimento pulmonar e os sintomas semelhantes a infecções por SARS-CoV-2, é importante atentar para os diagnósticos diferenciais.

This paper reports the case of a patient diagnosed with pulmonary alveolar proteinosis (PAP) after a worsening clinical picture due to COVID-19 infection, reviewing the literature on PAP to promote greater knowledge on this comorbidity for health professionals. A 48-year-old male patient with reported coughing and dyspnea for one year, presenting exacerbation of respiratory symptoms after SARS-CoV-2 infection, needing to start oxygen support via nasal catheter due to decreased saturation and impairment of daily living activities. High resolution computed tomography (HRCT) of the chest was performed and showed crazy paving with diffuse involvement of the lung fields. As per clinical and radiological findings compatible with PAP, the patient underwent total lung lavage and showed improvement in dyspnea and coughing. Pulmonary alveolar proteinosis deserves relevance due to the clinical impairment it can cause to the patient. Periodic medical follow-up is necessary to observe the disease evolution and define the therapeutic approach according to the clinical presentation of each patient. Sometimes, it may be necessary to repeat the total lung lavage, and to seek therapeutic alternatives in refractory cases. Given the pulmonary involvement and symptoms similar to SARS-CoV-2 infections, one must pay attention to the differential diagnoses.

Los objetivos de este artículo son presentar el caso de un paciente diagnosticado de proteinosis alveolar pulmonar (PAP) tras empeorar el cuadro clínico debido a infección por covid-19 y realizar una revisión sobre la PAP con el fin de generar más conocimiento a los profesionales sanitarios sobre esta comorbilidad. El caso investigado fue de un paciente masculino de 48 años de edad, con reporte de tos y disnea desde hace un año, que presentaba exacerbación del cuadro respiratorio posterior a infección por SARS-CoV-2, lo que requirió inicio de soporte de oxígeno vía catéter nasal por disminución de la saturación y deterioro de las actividades de la vida diaria. Se realizó una tomografía computarizada de alta resolución (TCAR) de tórax y se observó empedrado en mosaico con afectación difusa de los campos pulmonares. Según los hallazgos clínicos y radiológicos compatibles con PAP, se le realizó al paciente lavado pulmonar total y mejoró la disnea y la tos. La proteinosis alveolar pulmonar es una enfermedad que merece relevancia por el compromiso clínico que puede ocasionar al paciente. Es necesario un seguimiento médico periódico para observar la evolución de la enfermedad y definir el abordaje terapéutico según la presentación clínica de cada paciente. También es necesario repetir el lavado pulmonar total y, en casos refractarios, buscar alternativas terapéuticas. Ante la afectación pulmonar y síntomas similares a infecciones por SARS-CoV-2, es importante prestar atención a los diagnósticos diferenciales.

Protocolo de lavado pulmonar total del Hospital Santa Clara de Bogotá, a propósito de una paciente con proteinosis alveolar pulmonar resistente / Whole Lung Lavage Protocol at Hospital Santa Clara of Bogotá: Presentation of a Patient with Resistant Pulmonary Alveolar Proteinosis

RESUMEN La proteinosis alveolar pulmonar es una entidad clínica caracterizada por la acumula ción de material proteináceo, con alta riqueza en surfactante, mediado por una menor aclaración por parte de los macrófagos alveolares. En pacientes adultos, comúnmente se asocia a fenómenos autoinmunes que tienen como resultado una deficiencia del factor estimulante de colonias de granulocitos y macrófagos, lo que implica alteracio nes en la maduración y disfunción celular, lo que provoca disminución de la degrada ción del surfactante y su acumulación en el espacio alveolar. Su diagnóstico corres ponde a un reto para el clínico, sobre la base de los hallazgos en pruebas de función pulmonar, el patrón en "empedrado" (crazy paving) en la tomografía computarizada de tórax de alta resolución y que se confirma al obtener el material proteináceo en el lava do broncoalveolar. Dada su rareza, el tratamiento ideal permanece por ser elucidado y en la actualidad el pilar del tratamiento es el lavado pulmonar total. Reportamos un caso anecdótico de una paciente de 41 años con proteinosis alveolar pulmonar desde 2011, que ha requerido múltiples lavados pulmonares totales, con pobre respuesta a estos, persistencia de disnea y necesidad de oxígeno suplementario a pesar de realizar el procedimiento, pero con tendencia progresiva a la mejoría en los últimos 2 años. La técnica del lavado no está completamente estandarizada y su uso en Amé rica Latina es aún limitado, por lo que publicamos el protocolo utilizado en el Hospital Santa Clara de Bogotá, Colombia.

ABSTRACT Pulmonary alveolar proteinosis is a clinical entity characterized by the accumulation of proteinaceous material, rich in surfactant, mediated by reduced clearance by alveolar macrophages. In adult patients, it is commonly associated with autoimmune phenom ena resulting in a deficiency of the granulocyte-macrophage colony stimulating factor, which implies alterations in cell maturation and dysfunction, causing a decrease in surfactant degradation and its accumulation in the alveolar space. Its diagnosis poses a challenge to the clinician, based on the findings of pulmonary function tests and the crazy paving pattern of the high-resolution computed tomography of the chest, and is confirmed by obtaining the proteinaceous material in the bronchoalveolar la vage. Given its rarity, the ideal treatment remains to be elucidated, with whole lung lavage currently being the cornerstone of treatment. We report an anecdotal case of a 41-year-old female patient suffering from pulmonary alveolar proteinosis since 2011, who has required multiple whole lung lavages, with poor response to these, with persistent dyspnea and supplemental oxygen requirement even though she has performed the procedure, but with a progressive tendency towards improvement in the last 2 years. The lavage technique is not completely standardized and its use in Latin America is still limited, which is why we publish the protocol used in the Hospital Santa Clara of Bogotá, Colombia.

Unilateral Presentation of Pulmonary Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare disorder, in which lipoproteinaceous material accumulates within the alveoli. We report a case of a 27-year-old male patient with acute worsening of breathlessness over the last 7�months and cough with desaturation up to 79% on room air. Contrast-enhanced computerized tomography of the thorax revealed unilateral diffuse crazy-paving pattern likely PAP. Transbronchial lung biopsy confirmed the diagnosis of PAP. The present case highlights the unusual presentation of PAP with unilateral involvement. To the best of our knowledge, this is the first reported case of unilateral PAP from India with a biopsy diagnosis and resolution with whole lung lavage.

Comparative analysis of CT imaging findings on pulmonary alveolar proteinosis and pneumoconiosis / 中国职业医学

@#Objective To analyze the difference in computed tomography (CT) imaging findings between pulmonary alveolar pneumoconiosis Methods proteinosis (PAP) and occupational pneumoconiosis (hereinafter referred to as ). A total of 44 patients with PAP (PAP group) and 44 patients with pneumoconiosis (pneumoconiosis group) were selected as study subjects using Results convenient sampling method. The CT images of these two groups were comparatively analyzed. The detection rates of - - pulmonary CT pattern changes such as map like performance, ground glass opacity, paving stone sign and sphenoid wing like vs vs changes of pulmonary hilum in the PAP group were higher than those in the pneumoconiosis group (77.3% 0.0%, 75.0% vs vs P 2.3%, 56.8% 0.0%, 18.2% 0.0%, all <0.01); the detection rates of lymphadenopathy and calcification of pulmonary hilum, small pulmonary nodules, emphysema and interlobular septal thickening were lower in the PAP group than those in the vs vs vs vs P Conclusion pneumoconiosis group (34.1% 100.0%, 4.5% 100.0%, 2.3% 45.4%, 0.0% 22.7%, all <0.01). Paving - stone sign and map like performance were most commonly found in the CT imaging of patients with PAP, and it is uncommon in pneumoconiosis. These changes could be used as the CT differential diagnosis of the two diseases.

Comparative analysis of CT imaging findings on pulmonary alveolar proteinosis and pneumoconiosis / 中国职业医学

@#Objective To analyze the difference in computed tomography (CT) imaging findings between pulmonary alveolar pneumoconiosis Methods proteinosis (PAP) and occupational pneumoconiosis (hereinafter referred to as ). A total of 44 patients with PAP (PAP group) and 44 patients with pneumoconiosis (pneumoconiosis group) were selected as study subjects using Results convenient sampling method. The CT images of these two groups were comparatively analyzed. The detection rates of - - pulmonary CT pattern changes such as map like performance, ground glass opacity, paving stone sign and sphenoid wing like vs vs changes of pulmonary hilum in the PAP group were higher than those in the pneumoconiosis group (77.3% 0.0%, 75.0% vs vs P 2.3%, 56.8% 0.0%, 18.2% 0.0%, all <0.01); the detection rates of lymphadenopathy and calcification of pulmonary hilum, small pulmonary nodules, emphysema and interlobular septal thickening were lower in the PAP group than those in the vs vs vs vs P Conclusion pneumoconiosis group (34.1% 100.0%, 4.5% 100.0%, 2.3% 45.4%, 0.0% 22.7%, all <0.01). Paving - stone sign and map like performance were most commonly found in the CT imaging of patients with PAP, and it is uncommon in pneumoconiosis. These changes could be used as the CT differential diagnosis of the two diseases.

Comparative analysis of CT imaging findings on pulmonary alveolar proteinosis and pneumoconiosis / 中国职业医学

@#Objective To analyze the difference in computed tomography (CT) imaging findings between pulmonary alveolar pneumoconiosis Methods proteinosis (PAP) and occupational pneumoconiosis (hereinafter referred to as ). A total of 44 patients with PAP (PAP group) and 44 patients with pneumoconiosis (pneumoconiosis group) were selected as study subjects using Results convenient sampling method. The CT images of these two groups were comparatively analyzed. The detection rates of - - pulmonary CT pattern changes such as map like performance, ground glass opacity, paving stone sign and sphenoid wing like vs vs changes of pulmonary hilum in the PAP group were higher than those in the pneumoconiosis group (77.3% 0.0%, 75.0% vs vs P 2.3%, 56.8% 0.0%, 18.2% 0.0%, all <0.01); the detection rates of lymphadenopathy and calcification of pulmonary hilum, small pulmonary nodules, emphysema and interlobular septal thickening were lower in the PAP group than those in the vs vs vs vs P Conclusion pneumoconiosis group (34.1% 100.0%, 4.5% 100.0%, 2.3% 45.4%, 0.0% 22.7%, all <0.01). Paving - stone sign and map like performance were most commonly found in the CT imaging of patients with PAP, and it is uncommon in pneumoconiosis. These changes could be used as the CT differential diagnosis of the two diseases.

Proteinosis alveolar pulmonar: reporte de caso en paciente con linfoma no hodgkin mesentérico / Pulmonary alveolar proteinosis: report of a case in patient with history of mesenteric non-hodgkin lymphoma

Objetivos: Exponer en base a un caso clínico una revisión de literatura reciente sobre Proteinosis alveolar pulmonar (PAP). Presentación del caso: Revisión de ficha clínica electrónica de paciente de sexo masculino de 76 años con antecedente de linfoma no Hodgkin (LNH) mesentérico, estirpe B de tipo folicular, quien acude en forma reiterada a servicios de urgencia por cuadro de dos meses de evolución de fiebre, compromiso del estado general y tos. Al examen físico destaca crépitos en hemitórax derecho. Se realizó Tomografía computada (TC) de tórax que mostró opacidades pulmonares en vidrio esmerilado periféricas, con engrosamiento septal liso y algunas bandas retráctiles subpleurales. Se manejó ambulatoriamente con Azitromicina por una semana. Sin respuesta, evoluciona con baja de peso y diaforesis nocturna. Nueva TC de tórax en enero 2021, muestra nuevos focos de "empedrado" periféricos extensos, descrito como "crazy paving", focos de vidrio esmerilado difusos extensos, sin condensación y con resolución de bandas retráctiles. Estudio infeccioso negativo. Se realiza lavado broncoalveolar (LBA) con estudio histológico de líquido que muestra proceso inflamatorio crónico con abundantes macrófagos y material proteináceo. Discusión: Tras el descarte de patología infecciosa, se orientó el estudio hacia otras causas de enfermedad parenquimatosa pulmonar. Así, resulta fundamental la descripción correcta del patrón imagenológico tomográfico y el LBA que resultaron compatibles con PAP. Conclusión: La PAP es una patología infrecuente, pero una historia clínica adecuada, el planteamiento de diagnósticos diferenciales de neumonía de lenta resolución, asociado el reconocimiento del patrón radiológico característico y el estudio histológico con LBA permiten realizar un diagnóstico certero, con gran implicancia terapéutica.

Objective: To present a review of recent literature on pulmonary alveolar proteinosis (PAP) based on a clinical case. Presentation of the case: Review of electronic clinical record of a 76 years-old masculine patient with history of mesenteric Non-Hodgkin Lymphoma (NHL) follicular-type lineage B, who repeatedly attended the emergency services due to a two-month history of symptoms of fever, compromised general condition and cough. Physical examination revealed crepitus in the right hemithorax. Chest computed tomography (CT) was performed, which showed peripheral ground-glass pulmonary opacities, with smooth septal thickening and some subpleural retractile bands. He was managed on an outpatient basis with Azithromycin for one week. No response; evolves with weight loss and night diaphoresis. New chest CT in January 2021, shows new extensive peripheral "cobblestone" foci, described as "crazy paving", extensive diffuse ground glass foci, without condensation and with resolution of retractile bands. Negative infectious study. Bronchoalveolar lavage (BAL) was performed with a histological study of the fluid showing a chronic inflammatory process with abundant macrophages and proteinaceous material. Discussion: After ruling out infectious pathology, the study was oriented towards other causes of pulmonary parenchymal disease. Thus, the correct description of the tomographic imaging pattern and the BAL that were compatible with PAP are essential. Conclusion: PAP is an infrequent pathology, but an adequate clinical history, the approach to differential diagnoses of slowly resolving pneumonia, associated with the recognition of the characteristic radiological pattern and the histological study with BAL allow an accurate diagnosis to be made, with great therapeutic implications.

Pulmonary Alveolar Proteinosis due to Familial Myelodysplastic Syndrome with resolution after stem cell transplant

ABSTRACT Pulmonary alveolar proteinosis (PAP) is a rare lung disease with an incidence of 0.2 cases per million. PAP has multiple causes, including autoimmune, hereditary, congenital, or secondary. The latter includes hematologic conditions and exposure to different kinds of dust. Most patients present fever, dyspnea, and cough. The chest computed tomography (CT) may reveal the crazy-paving polygonal shapes with superimposed ground glass opacities delimited by thickened interlobular septa; however, this finding is more prevalent in patients with autoimmune PAP. Bronchoalveolar lavage (BAL) shows a milky-opaque appearance with PAS-positive debris on cytology. Treatment is focused on the underlying disease; however, some patients may require whole lung lavage for symptomatic management. We report a case of a 30-year-old female with a history of familial myelodysplastic syndrome (MDS) with GATA 2 mutation who presented to the outpatient clinic with several months of progressive dyspnea and nonproductive cough. The chest CT revealed bilateral ground-glass opacities prominently in the upper lobes. She underwent a bronchoscopy with lavage and biopsy, which revealed fragments of lung parenchyma with intra-alveolar coarse granular eosinophilic material strongly positive for PAS and d-PAS. The overall clinical presentation and histologic findings were diagnostic of PAP. Her GM-CSF was negative, and due to her history of MDS, secondary PAP (S-PAP) was strongly suspected. She underwent a successful allogeneic bone marrow pluripotent stem cell transplant to treat the myelodysplastic syndrome, with a follow-up chest CT showing clear lung parenchyma. The patient had resolution of symptoms about four months after the bone marrow transplant, confirming the diagnosis of S-PAP.

Proteinosis alveolar pulmonar / Pulmonary alveolar proteinosis

Resumen La proteinosis alveolar pulmonar (PAP) es una enfermedad pulmonar difusa, infrecuente, secundaria a una alteración en la homeostasis del surfactante. Se presenta el caso de una mujer de 69 años que ingresó a sala de internación por disnea progresiva hasta clase funcional III, de tres meses de evolución, asociada a tos no productiva. Se constató insuficiencia respiratoria tipo I. Como hallazgos en tomografía de tórax se evidenció engrosamiento del intersticio pulmonar intra e interlobulillar, opacidades en vidrio esmerilado y áreas con tendencia a la consolidación bilateral. Se realizó biopsia pulmonar con diagnóstico histológico de PAP y se efectuó tratamiento con lavado pulmonar total, logrando mejoría clínica. Se destaca la necesidad de tener presente diagnósticos diferenciales de insuficiencia respiratoria e infiltrados pulmonares en el contexto de la pandemia por COVID-19, incluidas las entidades muy poco prevalentes como lo es la PAP.

Abstract Pulmonary alveolar proteinosis (PAP) is a rare, diffuse pulmonary disease due to abnormal surfactant homeostasis. We present the case of a 69-year-old woman who was admitted to the hospital for progressive dyspnea with marked limitation in activity, and non-productive cough, of three months of evolution. Type I respiratory failure was confirmed. Chest tomography findings were interlobular and intralobular septal thickening, ground glass opacities and bilateral consolidation. Histological diagnosis was made and whole-lung lavage was performed with clinical improvement. We highlight the need to keep in mind differential diagnoses of respiratory failure and pulmonary infiltrates during COVID-19 pandemic, even rare entities such as PAP.

Clinical characteristics of pulmonary alveolar proteinosis / 中南大学学报(医学版)

OBJECTIVES@#Pulmonary alveolar proteinosis (PAP) is a rare disease with non-specific and various clinical manifestations, often leading to misdiagnosis. This study aims to raise the awareness of this disease via summarizing the clinical characteristics, diagnosis, and therapy of PAP.@*METHODS@#We retrospectively analyzed clinical data of 25 hospitalized cases of PAP during 2008 and 2019 in the Department of Respiratory and Critical Care Medicine of the Second Xiangya Hospital of Central South University.@*RESULTS@#Cough with unkown reason and dyspnea were common clinical manifastations of PAP. Five patients had a history of occupational inhalational exposure. Sixteen patients had typical image features including ground-glass opacification of alveolar spaces and thickening of the interlobular and intralobular septa, in typical shapes called crazy-paving and geographic pattern. Fourteen patients underwent pulmonary function tests, revealing a reduction in the diffusing capacity for carbon monoxide. The positive rate of transbronchial biopsy was 95%. Five patients received the whole lung lavage and the symptoms and imaging fcauters significantly relieved after five-years follow-up.@*CONCLUSIONS@#PAP is characterized by radiographic pattern and pathology. Transbronchial lung biopsy is effective to make diagnosis of PAP. The whole lung lavage remains a efficient therapy.

Proteinose alveolar pulmonar: Relato de caso / Pulmonary alveolar proteinosis: Case report

A proteinose alveolar pulmonar (PAP) é rara e caracterizada por preenchimento alveolar com material lipoproteináceo. A proteinose alveolar é caracterizada por um alvéolo com material eosinofílico, acelular, finamente granular, com fendas de colesterol. Este relato de caso descreve um paciente do sexo masculino, 2 anos, portador de hipogamaglobulinemia. O paciente foi internado com quadro compatível com Stevens- Johnson após uso de amoxicilina e clavulanato para quadro de otite média aguda. Posteriormente, foi encaminhado à unidade de terapia intensiva devido à piora respiratória clínica e radiológica. Biópsia pulmonar: proteinose alveolar com alvéolos distendidos por material proteináceo, eosinofílico e grumoso com infiltrado linfo- histiocitário local. A proteinose alveolar pulmonar é rara e o diagnóstico correto deve ser realizado para que seja realizado tratamento adequado e acompanhamento da evolução. Deve-se atentar para complicações, especialmente infecções oportunistas.

Pulmonary alveolar proteinosis (PAP) is rare and it is characterized by alveolar filling with lipoproteinaceous material. Alveolar proteinosis is characterized by an alveolus with eosinophilic, acellular, finely granular material, with cholesterol cracks. This case report describes a 2-yearold male patient with hypogammaglobulinemia. The patient was hospitalized with Stevens-Johnson-compatible condition after use of amoxicillin and clavulanate for acute otitis media. Subsequently, he was referred to the intensive care unit due to worsening clinical and radiological breathing. Lung biopsy: alveolar proteinosis with alveoli distended by proteinaceous, eosinophilic and lumpy material with local lymphohistiocytic infiltrate. Pulmonary alveolar proteinosis is rare and the correct diagnosis must be made in order to carry out an appropriate treatment and follow-up of the evolution. Attention should be paid to complications, especially opportunistic infections.

Pulmonary rehabilitation in pulmonary alveolar proteinosis: a case report

Pulmonary alveolar proteinosis is a rare lung disease in which surfactant accumulates in the alveoli causing impairment of gas exchange, pulmonary circulation, restrictive lung functions, respiratory and muscular dysfunction. Pulmonary rehabilitation is a core aspect in the management of patients with chronic respiratory diseases. However so far there are no documented studies on the effects of pulmonary rehabilitation in pulmonary alveolar proteinosis. Here authors report the case of a 20 year old female diagnosed with pulmonary alveolar proteinosis and having considerable activity limitations. Pulmonary rehabilitation program of 24 weeks was designed for her after a thorough assessment. The 6-minute walk distance (6 MWD), forced vital capacity (FVC), diffusion capacity for carbon monoxide (DLCO), exercise induced desaturation (SPO2) and QOL were evaluated using the SF-36 questionnaire. All parameters were recorded pre, post and at a follow up at 6 months after pulmonary rehabilitation. The 6MWD, FVC, DLCO, exercise induced desaturation and QOL improved considerably after 24 weeks of PR. At a follow up at 6 months all measures expect the FVC remained maintained. The authors thus conclude that Pulmonary Rehabilitation is effective in improving the exercise capacity, lung functions and quality of life in pulmonary alveolar proteinosis.

Lavado pulmonar bilateral terapéutico en una adolescente con proteinosis alveolar pulmonar. A propósito de un caso / Therapeutic bilateral lung lavage in an adolescent with pulmonary alveolar proteinosis. Case report

La proteinosis alveolar pulmonar es una enfermedad pulmonar difusa caracterizada por la acumulación anormal de surfactante y lipoproteínas en el espacio alveolar, lo cual empeora el intercambio gaseoso y lleva a un curso variable desde una presentación clínica asintomática hasta una falla respiratoria grave.Se presenta a una adolescente de 16 años con este diagnóstico que fue remitida a nuestro Hospital para la realización de un lavado pulmonar total luego de una historia de disnea progresiva, dificultad respiratoria, caída de la función pulmonar y anormalidades radiológicas. Se le realizaron estudios de función pulmonar y mediciones de laboratorio antes y después del lavado pulmonar terapéutico.Luego, la paciente presentó una inmediata mejoría tanto de los síntomas, radiografía de tórax y mediciones de estudios de función pulmonar como en el intercambio gaseoso. El lavado pulmonar total continúa siendo el estándar de oro para el tratamiento de la proteinosis alveolar pulmonar en casos graves

Pulmonary alveolar proteinosis is an unusual diffuse lung disease characterized by abnormal accumulation of pulmonary surfactant and lipoproteins in the alveolar space, which impairs gas exchange with a variable clinical course, ranging from an asymptomatic clinical presentation to severely affected respiratory failure.A 16-year-old girl with diagnosis of pulmonary alveolar proteinosis presented to our hospital for therapeutic lung lavage after a recent history of progressive dyspnea, respiratory distress, declining lung function measurements, and worsening radiographic abnormalities. We obtained baseline pulmonary function tests and laboratory measurements before and after therapeutic bilateral lung lavage. After the lavage, the patient demonstrated an immediate improvement in symptoms, chest radiograph appearance, pulmonary function test measurements, as well as in gas exchange. Whole lung lavage is still the gold standard for treatment of pulmonary alveolar proteinosis in severe cases.

Whole lung lavage in an infant with pulmonary alveolar proteinosis and literature review / 中华儿科杂志

Objective@#To investigate the safety, feasibility and operation key points of whole lung lavage in infants with pulmonary alveolar proteinosis.@*Methods@#The clinical manifestations, genetic screening, therapeutic interventions and outcome of an infant with pulmonary alveolar proteinosis complicated with respiratory failure who received whole lung lavage in November 2018 in Shanghai Children′s Medical Center Affiliated to Shanghai Jiaotong University School of Medicine were reported. Websites including PubMed, Springer Link, China National Knowledge Infrastructure (CNKI), Weipu Database, and Wanfang Database were searched using the key words of "whole lung lavage" "pediatric" and "pulmonary alveolar proteinosis" for articles published from their establishments to April 2019. Relevant literature was reviewed.@*Results@#A 3-month-old boy had experienced cough, shortness of breath and cyanosis for 1 week prior to admission to pediatric intensive care unit. Physical examination showed hepatosplenomegaly. Complete blood cell count showed mild anemia (hemoglobin 96 g/L) and normal white blood cells. The patient had normal C-reactive protein and normal blood platelet. Biochemical panel showed hypoalbuminemia (31 g/L), mildly elevated glutamic oxaloacetic transaminase (115 U/L) and blood ammonia (165 μmol/L), extremely elevated lactate dehydrogenase (>6 600 U/L) and hyperferritinemia (>4 500 μg/L). Chest computed tomography (CT) revealed decreased transmittance of both lungs, patchy high density shadow and ground glass opacity. Genetic testing revealed a mutation of c.625+1G>A in SLC7A7. Schiff reaction (PAS staining) in bronchoalveolar lavage fluid was positive. The patient was diagnosed with severe pneumonia, respiratory failure, lysinuria urinary protein intolerance, and pulmonary alveolar proteinosis. The patient received sequential unilateral whole lung lavage in 2 days and was successfully weaned from ventilator. He was discharged home breathing room air. Eleven articles (11 in English and non in Chinese) were reviewed. Twenty-one patients were included. After whole lung lavage, 76% (16/21) of the patients had improvement in respiratory function.@*Conclusions@#Whole lung lavage can effectively improve respiratory failure caused by pulmonary alveolar proteinosis in infant patients. The procedure is feasible and safe.

Anestesia para reparación antenatal de mielomeningocele: Presentación de un caso / Anesthesia for myelomenigocele antenatal repair: Case report

INTRODUCTION: The advance in the methods of prenatal diagnosis and surgical techniques have allowed the development of fetal surgery, achieving identification and early treatment of anomalies invalidating extrauterine life. Myelomeningocele (MMC) is the most frequent neural tube defect and its intrauterine correction has demonstrated benefits. OBJECTIVE: To publicize the anesthetic management of a prenatal correction of MMC performed in a public hospital in Chile. CASE REPORT: 31-year-old woman, pregnancy of 25 weeks of gestational age, fetus carrying MMC lumbosacral, who underwent open correction. Procedure performed with incidents under general anesthesia with remifentanil and sevoflorane MAC in 2 and tocolytic prophylaxis. At 48 hours post operative, he presented an acute pulmonary edema compatible (EPA), which was successfully resolved with depletive therapy for 24 hours in the Intensive Care Unit, without the need for mechanical ventilation or use of vasoactive drugs. Discharged one week later in good condition, with interruption of pregnancy by elective caesarean section at 37 weeks, with a newborn without stigmas of neurological sequelae. CONCLUSIONS: The mother-fetus binomial is a challenge for the anesthetist. In intrauterine surgery the need for knowledge about the pharmacology of tocolytics, placental uterine physiology and the complications of the procedure are added.

INTRODUCCIÓN: El avance en los métodos de diagnóstico prenatal y las técnicas quirúrgicas han permitido el desarrollo de la cirugía fetal, logrando identificación y tratamiento precoz de anomalías invalidantes para la vida extrauterina. El mielomeningocele (MMC) es el defecto del tubo neural más frecuente y su corrección intrauterina tiene beneficios demostrados. OBJETIVO: Dar a conocer el manejo anestésico de una corrección prenatal de MMC realizada en un hospital público de Chile. CASO CLÍNICO: Mujer de 31 años, embarazo de 25 semanas de edad gestacional, feto portador de MMC lumbosacro, que se sometió a una corrección por vía abierta. Procedimiento realizado con incidentes bajo anestesia general con remifentanilo y sevoflorano MAC en 2 y profilaxis tocolítica. A las 48 horas postoperatorias presentó cuadro compatible con edema pulmonar agudo (EPA), que se resolvió exitosamente con terapia depletiva por 24 horas en Unidad de Cuidados Intensivos, sin necesidad de ventilación mecánica ni uso de drogas vasoactivas. Dada de alta una semana después en buenas condiciones. El embarazo se interrumpió por cesárea electiva a las 37 semanas, con un recién nacido sin estigmas de secuela neurológica. CONCLUSIONES: El binomio madre-feto es un reto para el anestesista. En cirugía intraútero se suma la necesidad de conocimientos sobre la farmacología de los tocolíticos, fisiología útero placentaria y las complicaciones propias del procedimiento.

Proteinosis alveolar pulmonar / Pulmonary alveolar proteinosis an update

La Proteinosis Alveolar Pulmonar (PAP) es una enfermedad poco frecuente, caracterizada por la acumulación de material lipoproteico derivado del surfactante pulmonar al interior de los alvéolos por una falla de depuración de este material por los macrófagos alveolares, siendo la causa más frecuente de esta disfunción la acción bloqueadora producida por anticuerpos anti factor estimulante de colonias de granulocitos y macrófagos (GM-CSF) lo que lleva a un deterioro del intercambio gaseoso. La evolución es variable abarcando desde la resolución espontánea hasta la insuficiencia respiratoria grave y la muerte. Se describen tres formas de PAP: Genética, secundaria y autoinmune (antes primaria o idiopática) siendo esta última la más frecuente en adultos. Clínicamente, se manifiesta por disnea, tos seca e hipoxemia que pueden ser progresivas. En la radiografía de tórax se encuentran opacidades bilaterales y la tomografía computarizada de tórax de alta resolución (TACAR) muestra vidrio esmerilado con sobre posición de engrosamiento septal intra e interlobulillar, patrón conocido como "crazy paving". El diagnóstico se basa en la clínica y en el lavado broncoalveolar con material PAS positivo. La biopsia quirúrgica es confirmatoria. El tratamiento clásico es el lavado pulmonar total (LPT) para remover el contenido alveolar. Otras alternativas son la administración de GM-CSF subcutáneo o inhalado, plasmaferesis y rituximab, cuyos resultados son variables. Diferentes autores han modificado la forma del LPT y combinado los diferentes métodos de tratamiento con el fin de obtener resultados más rápidos y efectivos.

Pulmonary Alveolar Proteinosis (PAP) is a rare disease characterized by the accumulation of surfactant derived lipoproteinaceous material filling the alveoli, secondary to failure of its clearance by macrophages. Most of the patients are adults that have auto antibodies directed to Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF). The evolution is towards disturbed gaseous exchange with a wide spectrum of disease from spontaneous recovery to death. There are three forms of PAP: genetic, secondary and autoimmune. Symptoms are scarce and patients may present with dyspnea, dry cough and hypoxemia. Chest X ray shows bilateral opacities and thorax CT depicts ground glass opacities surrounded by septal widening, the so called "crazy paving" pattern. Diagnosis is made on clinical and radiological grounds and confirmed by PAS positive staining of bronchoalveolar lavage material or surgical lung biopsy. Accepted treatment is whole lung lavage (WLL) with saline. Alternatives are subcutaneous or inhaled GM-CSF, Plasmapheresis or Rituximab, and even modification of the method of WLL and combination of different manner of treatment.

Characterization and outcomes of pulmonary alveolar proteinosis in Brazil: a case series / Proteinose alveolar pulmonar: caracterização e desfechos em uma série de casos no Brasil

ABSTRACT Objective: Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by the alveolar accumulation of surfactant, which is composed of proteins and lipids. PAP is caused by a deficit of macrophage activity, for which the main treatment is whole-lung lavage (WLL). We report the experience at a referral center for PAP in Brazil. Methods: This was a retrospective study involving patients with PAP followed between 2002 and 2016. We analyzed information regarding clinical history, diagnostic methods, treatments, and outcomes, as well as data on lung function, survival, and complications. Results: We evaluated 12 patients (8 of whom were women). The mean age was 41 ± 15 years. Most of the patients were diagnosed by means of BAL and transbronchial biopsy. The mean number of WLLs performed per patient was 2.8 ± 2.5. One third of the patients never underwent WLL. Four patients (33.3%) had associated infections (cryptococcosis, in 2; nocardiosis, in 1; and tuberculosis, in 1), and 2 (16.6%) died: 1 due to lepidic adenocarcinoma and 1 due to complications during anesthesia prior to WLL. When we compared baseline data with those obtained at the end of the follow-up period, there were no significant differences in the functional data, although there was a trend toward an increase in SpO2. The median follow-up period was 45 months (range, 1-184 months). The 5-year survival rate was 82%. Conclusions: To our knowledge, this is the largest case series of patients with PAP ever conducted in Brazil. The survival rate was similar to that found at other centers. For symptomatic, hypoxemic patients, the treatment of choice is still WLL. Precautions should be taken in order to avoid complications, especially opportunistic infections.

RESUMO Objetivo: A proteinose alveolar pulmonar (PAP) é uma doença rara, caracterizada pelo acúmulo alveolar de substância surfactante, composta por proteínas e lipídios. É causada por um déficit de atividade macrofágica, cujo principal tratamento é a lavagem pulmonar total (LPT). Relatamos a seguir a experiência de um centro de referência brasileiro em PAP. Métodos: Estudo retrospectivo com a avaliação de pacientes com PAP acompanhados entre 2002 e 2016. Informações sobre história clínica, métodos diagnósticos, tratamentos realizados e desfechos, incluindo dados de função pulmonar, sobrevida e presença de complicações, foram analisados. Resultados: Foram incluídos 12 pacientes (8 mulheres), com média de idade de 41 ± 15 anos. A maioria dos pacientes foi diagnosticada por LBA e biópsia transbrônquica. A média do número de LPT realizadas por paciente foi de 2,8 ± 2,5. Um terço dos pacientes não foi submetido à LPT. Quatro pacientes (33,3%) apresentaram infecções associadas (criptococose, em 2; nocardiose, em 1; e tuberculose, em 1) e houve 2 óbitos (16,6%): 1 por adenocarcinoma lepídico e 1 por complicações na indução anestésica pré-LPT. Não houve diferença significativa entre os dados funcionais; porém houve uma tendência de aumento da SpO2 ao se comparar os dados iniciais aos do final do seguimento. A mediana de seguimento foi de 45 meses (1-184 meses), com taxa de sobrevida em 5 anos de 82%. Conclusões: Pelo que sabemos, esta é a maior série de casos de PAP no Brasil. A taxa de sobrevida foi semelhante à encontrada em outros centros. A LPT ainda é o tratamento de escolha para pacientes sintomáticos e hipoxêmicos. Deve-se atentar para complicações, especialmente infecções oportunistas.

Whole-lung lavage in a patient with pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare syndrome in which phospholipoproteinaceous matter accumulates in the alveoli leading to compromised gas exchange. Whole-lung lavage is considered the gold standard for severe autoimmune PAP and offers favorable long-term outcomes. In this case report, we describe the perioperative management and procedural specifics of a patient undergoing WLL for PAP in which an anesthesiologist serves as the proceduralist and a separate anesthesiologist provides anesthesia care for the patient.

CT quantitative analysis and visual scores of pulmonary alveolar proteinosis and correlation with pulmonary function / 中华放射学杂志

Objective To investigate the evaluating the value of CT quantitative analysis and visual scores on pulmonary alveolar proteinosis (PAP). Methods Thirty one patients with PAP who underwent chest HRCT scans and pulmonary function tests(PFTs)between July 2014 and April 2016 in our hospital were collected and analyzed.HRCT mean lung density(MLD)was measured by syngo InSpace Lung Parenchyma Analysis.Using CT visual scores(HRCT score and RE score),two radiologists with three-years experience at least evaluated pulmonary lesions retrospectively. SPSS 21.0 statistical software was used to analyze the data. Spearman correlation analysis was used to evaluate the correlation between MLD, HRCT score and RE score.The correlation between MLD, HRCT score, RE score, the parameters of PFTs, and blood gas parameters were also analyzed by Spearman correlation analysis. Results There were correlations between MLD,HRCT score and RE score.The correlation coefficient among MLD,HRCT score and RE score were r=0.835 vs r=0.768 vs r=0.838 in baseline. The correlation coefficient among MLD, HRCT score and RE score were r=0.827 vs r=0.780 vs r=0.896 in follow-up. And there were also strong correlation between ΔMLD, ΔHRCTscore and ΔREscore. The correlation coefficient among ΔMLD, ΔHRCTscore and ΔREscore were r=0.599 vs r=0.651 vs r=0.867. There were significantly (P＜0.05) negative correlations among MLD, HRCT score, RE score and VA, DLCO in baseline and follow-up retrospectively. There were significantly (P≤0.001) negative correlations among the ΔMLD, ΔHRCTscore, ΔREscore and ΔVA, ΔDLCO.The correlation among MLD, HRCT score, RE score and DLCO was more significant than that of VA. The correlation among ΔMLD, ΔHRCTscore,ΔREscore and ΔDLCO was more significant than that of ΔVA.There were markedly(P＜0.05) negative correlations between MLD and PO2,and the correlation coefficient were r=-0.738 in baseline vs r=- 0.810 in follow-up. Conclusion The HRCT quantitative analysis and HRCT visual scores are feasible to evaluate PAP.