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.

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.

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.

Importancia del cepillado bronquial a ciegas en lesiones tumorales periféricas no visibles endoscópicamente / Importance of blind bronchial brushing in peripheral endoscopically non visible lesions

Introducción: el diagnóstico rápido de las enfermedades neoplásicas pulmonares, primarias o metastásica, tiene una influencia importante sobre el resultado del tratamiento y el pronóstico de este grupo de enfermedades con alta letalidad. En el diagnóstico de estas se usan varias técnicas, entre ellas el cepillado bronquial para estudio citológico con el cual se alcanza un gran rendimiento diagnóstico en las lesiones visibles endoscópicamente y que también es de gran utilidad en los tumores periféricos diagnosticados en un porciento significativo y con el mínimo de complicaciones. Objetivo: evaluar los resultados del cepillado bronquial a ciegas en lesiones pulmonares periféricas de posible etiología tumoral. Métodos: estudio descriptivo, de corte transversal retrospectivo, en pacientes a los que se les realizó cepillado bronquial a ciegas en lesiones pulmonares periféricas sugestivas de ser tumorales en el servicio de neumología del Hospital Hermanos Ameijeiras, en un periodo de 10 años. Se tomaron en cuenta variables como: la localización de la lesión, cepillado útil para diagnóstico, positivo y negativo, tamaño de la lesión y los diagnósticos definitivos. Resultados: se realizó cepillado a ciegas a 517 pacientes, fue útil para diagnóstico en 491 (95 por ciento), en 289 (56 por ciento) se obtuvo resultado positivo. Conclusiones: el cepillado a ciegas en lesiones pulmonares periféricas tumorales es una técnica que mejora considerablemente el rendimiento diagnóstico del carcinoma broncógeno con un número bajo de complicaciones(AU)

Introduction: The rapid diagnosis of pulmonary, primary or metastatic neoplastic diseases has significant influence on the outcome of treatment and the prognosis of this group of high lethal diseases. In their diagnosis, several techniques are used, among them the bronchial brushing for cytological study which achieves great diagnostic performance in endoscopically visible lesions and it is also very useful in the peripheral tumors diagnosed in a significant percentage and with minimum complications. Objective: To evaluate the results of blind bronchial brushing in peripheral lung lesions of possible tumor etiology. Methods: A descriptive, retrospective, cross-sectional study in patients who underwent blind bronchial brushing in peripheral pulmonary lesions suggestive of tumor was conducted in the Pneumology Department at Hermanos Ameijeiras Hospital, over a period of 10 years. The variables taken into account were location of the lesion, brushing useful for diagnosis, positive and negative results, size of the lesion, and the definitive diagnoses. Results: Blind brushing was performed on 517 patients, it was useful for diagnosis in 491 (95 percent), a positive result was obtained in 289 (56 percent). Conclusions: Blind brushing technique in peripheral lung lesions considerably improves the diagnostic performance of bronchogenic carcinoma with low number of complications(AU)

Utilidad de los marcadores inflamatorios en el control del asma pediátrica / Use of inflammatory markers for monitoring paediatric asthma

La evaluación del control del asma considera la medición de la sintomatología, la calidad de vida, la función pulmonar y los marcadores inflamatorios. En los últimos años se han multiplicado las publicaciones relacionadas con el estudio de biomarcadores en el manejo del asma pediátrica. A pesar de la gran variedad de marcadores inflamatorios descritos en estudios de investigación, solo un pequeño grupo ha demostrado ser útil en el control de la enfermedad. Los eosinófilos en esputo inducido tienen la evidencia más sólida en la medición del control de asma. El condensado de aire exhalado y los leucotrienos urinarios podrían tener utilidad en el futuro si se logran estandarizar sus procedimientos e interpretación de resultados. El óxido nítrico, la proteína catiónica básica del eosinófilo y la biopsia bronquial con lavado broncoalveolar parecieran tener utilidad solo en un grupo reducido de pacientes.

The assessment of asthma control takes into account the symptoms, quality of life, lung function, and inflammatory markers. In the last few years, there has been a large increase in the number of publications related to the study of biomarkers in the management of paediatric asthma. Despite the large variety of inflammatory markers described in research studies, only a small group has shown to be useful in monitoring the disease. Induced sputum eosinophils offer the most solid evidence in assessing asthma control. Exhaled breath condensate and urinary leucotrienes could be useful in the future if there is standardisation in their procedures and interpretation of the results. Nitric oxide, basic eosinophil cationic protein, and bronchial biopsy with bronchoalveolar lavage, only appeared to be useful in a reduced group of patients.

Bronchoalveolar lavage (BAL) in tapirs (Tapirus terrestris) with Mycobacterium tuberculosis / Lavado broncoalveolar (LBA) em antas (Tapirus terrestris) com Mycobacterium tuberculosis

This paper describes the in vivo Bronchoalveolar lavage (BAL) technique by endoscopy in tapirs (Tapirus terrestris) with clinical signs of tuberculosis. The technique was performed in two tapirs, male and female, from Curitiba Zoo, Paraná, Brazil. A flexible endoscope and a polyethylene catheter were used after the chemical restraint of the animals. For BAL technique, 60mL of saline 0.9% were infused with a polyethylene catheter, introduced by the endoscope's working channel, and 15mL of BAL were recovered, analyzed and submitted to cytocentrifugation. Slides were stained by Papanicolaou, periodic acid-Schiff (PAS) and Ziehl-Neelsen methods contained high quantity of inflammatory cells on light microscopy (macrophages 27.5%, lymphocytes 0.5%, neutrophis 67% and eosinophis 5%). BAL samples were submitted to culture, bacilloscopy and PCR and were negative for both animals. Based on this study, it was concluded that the bronchoalveolar lavage technique in tapirs is feasible, simple, noninvasive, practical and fast, providing an important clinical information in vivo regarding the functional status of the lower respiratory tract...

O presente trabalho descreve a técnica de lavado broncoalveolar (LBA) por endoscopia em antas (Tapirus terrestris) in vivo com sinais clínicos de tuberculose. A técnica foi realizada em duas antas, um macho e uma fêmea, provenientes do Zoológico de Curitiba, Paraná, Brasil, utilizando-se endoscópio flexível e sonda de polietileno, após a contenção química desses animais. Para o LBA, 60mL de solução fisiológica 0,9% foram infundidos com auxílio de cateter de polietileno, introduzido pelo canal de trabalho do endoscópio, e, aproximadamente, 15mL de LBA foram recuperados, acondicionados, analisados e submetidos à citocentrifugação. As lâminas foram coradas pelas técnicas de Papanicolau, ácido periódico de Schiff (PAS) e Ziehl- Neelsen, método que contém altas quantidades de células inflamatórias em microscopia (macrófagos 27,5%, linfócitos 0,5%, neutrófilos 67% e eosinófilos 5%). Amostras de LBA foram submetidas a cultura, baciloscopia e PCR e foram negativas em ambos os animais. Concluiu-se, baseado no presente trabalho, que técnica de lavado broncoalveolar é simples, não invasiva, funcional e rápida. Pode fornecer ao clínico importantes informações acerca do estado de funcionamento do aparelho respiratório in vivo...

Role of fiberoptic bronchoscopy and utility of bronchial washings and brushings in the diagnosis of lung diseases.

Transbronchial lung biopsy via fiberoptic bronchoscope is an extremely useful technique by which bronchial as well as lung biopsies along with brushings and washings can be easily and safely taken.1 Fiberoptic bronchoscopy (FOB) was performed and biopsies were done in 250 patients. In addition, bronchial brushings and washings were also taken in 140 and 115 cases, respectively. Adequate material was obtained in 242 cases. The cases were broadly classified into neoplastic and nonneoplastic categories. Malignancies and specific granulomatous diseases, tuberculosis and sarcoidosis were the main diseases diagnosed. Brushings showed a sensitivity of 88.2% and a specificity of 98.9% for the diagnosis of neoplasms. On the other hand, washings had only a 34.9% sensitivity and a 98.6% specificity in diagnosing neoplastic disorders. We concluded that FOB is a safe and effective tool in the diagnostic work-up of suspected malignancies and neoplastic lung diseases.

Procedimento e complicações anestésicas no manejo de lavagem pulmonar total em paciente obeso com proteinose alveolar pulmonar: relato de caso / Anaesthetic, procedure and complications management of serial whole lung lavage in an obese patient with pulmonary alveolar proteinosis: case report / Procedimiento y complicaciones anestésicas en el manejo del lavado pulmonar total en paciente obeso con proteinosis alveolar pulmonar: relato de caso

JUSTIFICATIVA E OBJETIVOS: O primeiro caso de proteinose alveolar pulmonar (PAP) foi descrito por Rose em 1958, mas ainda é um distúrbio raro. PAP é caracterizada pela deposição de material lipoproteico secundário ao processamento anormal de surfactantes pelos macrófagos. Os pacientes podem ter dispneia progressiva e tosse, às vezes acompanhadas pelo agravamento da hipóxia, e seu curso pode variar de deterioração progressiva a melhora espontânea. Muitas terapias foram usadas, incluindo antibióticos, drenagem postural e ventilação com pressão positiva intermitente com acetilcisteína, heparina e soro fisiológico em aerossol. Atualmente, a base do tratamento é a lavagem pulmonar total (LPT). A LPT, embora seja geralmente bem-tolerada, pode estar associada a algumas complicações. RELATO DE CASO: Relatamos um caso de PAP grave durante o procedimento anestésico e as complicações no manejo da proteinose alveolar pulmonar em um paciente que havia sido submetido a múltiplas e alternadas lavagens de um dos pulmões ao longo de sete anos (os últimos três em nosso hospital), com melhora dos sintomas depois de cada tratamento.

BACKGROUND AND OBJECTIVES: The first case of Pulmonary Alveolar Proteinosis (PAP) was described by Rose in 1958, but it is still a rare disorder. PAP is characterized by deposition of lipoproteinaceous material secondary to abnormal processing of surfactant by macrophages. Patients may suffer from progressive dyspnea and cough that at times is accompanied by worsening hypoxia and its course can vary from progressive deterioration to spontaneous improvement. Many therapies have been used to treat PAP including antibiotics, postural drainage, and intermittent positive pressure breathing with aerosolized Acetylcysteine, heparin and saline. At present, the mainstay of treatment is whole lung lavage (WLL). Although generally well tolerated, WLL can be associated with some complications. CASE REPORT: We report a case of severe PAP through the anaesthetic, procedure and complications management of pulmonary alveolar proteinosis in one patient who has undergone multiple, alternating, single-lung lavages over the past seven years, the last three in our hospital, with improvements in her symptoms following each therapy.

JUSTIFICATIVA Y OBJETIVOS: El primer caso de proteinosis alveolar pulmonar (PAP) fue descrito por Rose en 1958, pero todavía continúa siendo un trastorno raro. La PAP se caracteriza por la deposición de material lipoproteico secundario al procesamiento anormal de surfactantes por los macrófagos. Los pacientes pueden tener disnea progresiva y tos, a veces seguidas de un agravamiento de la hipoxia, y su curso puede variar de deterioración progresiva a una mejoría espontánea. A lo largo de los años, muchas terapias han sido usadas para tratar la PAP, incluyendo antibióticos, drenaje postural y ventilación con presión positiva intermitente con acetilcisteína, heparina y suero fisiológico en aerosol. Hoy por hoy, la base del tratamiento es el lavado pulmonar total (LPT), descrito por primera vez por Ramirez-Rivera y col. Existen tres variantes de la enfermedad: congénita, secundaria y adquirida. Las recientes investigaciones sugieren que en la forma más común (PAP adquirida [idiopática]), la autoinmunidad contra el factor estimulador de las colonias de granulocitos y macrófagos (FEC-GM) pulmonares desempeña un importante papel. El factor recombinante que estimula las colonias de granulocitos y macrófagos parece beneficiarse con un subconjunto de pacientes adultos con PAP y puede representar una alternativa en el tratamiento de la enfermedad, pero la tasa de éxito todavía no es suficiente para substituir la LPT. La LPT, aunque sea generalmente bien tolerada, puede estar asociada con algunas complicaciones. RELATO DE CASO: Relatamos un caso de PAP grave durante el procedimiento anestésico y las complicaciones en el manejo de la proteinosis alveolar pulmonar en un paciente que había sido sometido a múltiples y alternados lavados de uno de los pulmones a lo largo de siete años (los últimos tres en nuestro hospital), con una mejoría de los síntomas después de cada tratamiento.

Anestesia para lavagem pulmonar em paciente pediátrico portador de proteinose alveolar pulmonar / Anesthesia for lung lavage in pediatric patient with pulmonary alveolar proteinosis / Anestesia para el lavado pulmonar en paciente pediátrico portador de proteinosis alveolar pulmonar

JUSTIFICATIVA E OBJETIVOS: A proteinose alveolar pulmonar (PAP) é um transtorno raro, descrito inicialmente em 1958. A lavagem pulmonar total (LPT), proposta na década de 1960 ainda é o tratamento de escolha. Diversas técnicas foram descritas para realizar a lavagem pulmonar em pediatria, no entanto, todas apresentam limitações e riscos. RELATO DO CASO: Paciente de 6 anos e 8 meses, sexo feminino, 25 kg com o diagnóstico de proteinoise alveolar pulmonar submetida a lavagem pulmonar total por fibrobroncoscopia lobar sequencial sob anestesia geral em ventilação espontânea.

BACKGROUND AND OBJECTIVES: Pulmonary Alveolar Proteinosis (PAP) is a rare disorder first described in 1958. The Whole-Lung Lavage (WLL) proposed in the 1960s, remains the treatment of choice. Several techniques have been described to perform lung lavage in pediatric patients; however, all have limitation and risks. CASE REPORT: Female patient, aged 6 years and 8 months, 25 kg, diagnosed with pulmonary alveolar proteinoisis, who underwent whole-lung lavage by sequential lobar fiberoptic bronchoscopy under general anesthesia and spontaneous ventilation.

JUSTIFICATIVA Y OBJETIVOS: La proteinosis alveolar pulmonar (PAP) es un transtorno raro, descrito inicialmente en 1958. El lavado pulmonar total (LPT), propuesto en la década de 1960, es actualmente el tratamiento de elección. Diversas técnicas fueron descritas para realizar el lavado pulmonar en pediatría pero sin embargo, todas tienen limitaciones y riesgos. RELATO DEL CASO: Paciente de seis años y ocho meses, del sexo femenino, 25 kg con el diagnóstico de proteinosis alveolar pulmonar, que fue sometida al lavado pulmonar total por fibrobroncoscopia lobar secuencial bajo anestesia general en ventilación espontánea.

Resultados preliminares y factibilidad del mini lavado bronco-alveolar en pacientes cursando falla respiratoria severa / Mini bronchoalveolar lavage in patients with severe respiratory failure

Background: To recognize the etiological agent responsible for severe acute respiratory failure (ARF) in patients in mechanical ventilation (MV) is important to determine their treatment and prognosis, and to avoid the excessive use of antibiotics. Mini bronchoalveolar lavage (mini BAL) is a blind, non bronschoscopic procedure, used to obtain samples from the lower respiratory tract from patients on mechanical ventilation (MV). Aim: To assess the feasibility, complications and preliminary results of mini BAL among patients with severe ARF on MV. Material and Methods: Prospective study in 17 patients with acute lung injury (ALI ) or acute respiratory distess syndrome (ARDS) on MV and with negative conventional microbiological studies. Mini BAL was performed using standardized protocols. Hemodynamic and respiratory parameters where measured before and after the procedure. Samples obtained were sent to quantitative cultures. Results: At baseline: APACHE II score of 22 ± 6,7, PaO2/FiO2 ratio was 176.6 ± 48.6 and the oxygenation index was 9.74 ± 3.78. All procedures were performed by an ICU resident. Thirty five percent of the procedures had positive cultures and no complications related to the procedures were reported. The procedure lasted an average of 12 minutes and the instilled and rescued volume were 60 ml and 19.6 ml, respectively. There were no significant differences between hemodynamic and respiratory variables before and after the procedure. Conclusions: Mini BAL is a safe, fast and easy technique for obtaining samples from the inferior airway in patients with ALI or ARDS on MV.

Continuous positive airway pressure ventilation during whole lung lavage for treatment of alveolar proteinosis -A case report and review of literature

Pulmonary alveolar proteinosis [PAP] is a rare disease that affects young population usually in the age group of 20-40 years, characterized by the deposition of lipoproteinacious material in the alveoli secondary to abnormal processing of surfactant by macrophages. We report a case of a 15-year-old female who had history of cough with sputum for 3 days along with fever. She was seen in another hospital and was treated as a case of pneumonia where she received antibiotic but with no improvement. Computerized tomography [CT] chest showed diffuse interlobular septal thickening in the background of ground glass opacity giving a picture of crazy paving pattern which was consistent with the diagnosis of PAP. The patient was scheduled to undergo, first right-sided whole lung lavage [WLL] under general anesthesia. Endobronchial intubation using left sided 37 Fr double lumen tube. Continuous positive airway pressure [CPAP] as described in our previously published report was connected to the right lumen of the endobronchial tube. CPAP ventilation was used during the suctioning of lavage fluid phase in order to improve oxygenation. WLL was done using 5 L of warm heparinized saline [500 i.u/litre]. The same procedure was repeated on the left side using 6 L of heparinized normal saline solution. In conclusion, anesthesia in alveolar proteinosis for patients undergoing WLL is challenging to the anesthesiologist. It requires meticulous preoperative preparation with antibiotics, mucolytics and chest physiotherapy. Also it requires careful intraoperative monitoring and proper oxygenation especially during the suctioning phase of the lavaged fluid. With this second case report of successful anesthetic management using the modified CPAP system we recommend with confidence the application of CPAP ventilation to improve oxygenation during WLL

Diagnóstico de neumocistosis en pacientes oncológicos: por la técnica de inmunofluorescencia directa / Neumocistosis in oncological patients: diagnoses for immunofluorescense direct technique

El paciente con enfermedades oncológicas tiene un alto riesgo para desarrollar infecciones respiratorias, y neumonía por Pneumocystis jirovecii. En Venezuela existen pocos estudios sobre la neumocistosis en pacientes oncológicos. El objetivo de este trabajo fue detectar la presencia de Pneumocystis jirovecii en pacientes oncológicos a través de la técnica de inmunofluorescencia directa. Se recibieron, durante 10 meses, 31 muestras respiratorias (lavado broncoalveolar, esputo espontáneo e inducido, aspirados traqueales), de ellas 8 (25,5 por ciento) resultaron positivas. La distribución por tipo de cáncer fue la siguiente: 18 tumores sólidos y 13 leucemias y linfomas. La positividad entre los grupos estudiados no fue estadísticamente significativa (P>0,05). Los exámenes de laboratorio complementarios, relacionados tampoco fueron estadísticamente significativos (P>0,05). Es necesario incluir este diagnóstico en estudio microbiológico diferencial de infecciones del tracto respiratorio inferior en pacientes con cáncer, estos pacientes cursan con una sintomatología general inespecífica y tendrán una alta posibilidad de desarrollar neumocistosis.

The patient with malignancy disease has a high risk to develop respiratory infections for Pneumocystis jirovecii pneumonia. Investigations about pneumocystosis in oncological patients in Venezuela are scarce. The objective of this work was to detect Pneumocystis jirovecii in oncological patients by the method of direct immunofluorescence technique. Thirty one respiratory specimens (bronchoalveolar lavage, spontaneous and induced sputum, and tracheal aspirates) received in 10 months, 8 specimens of them (25.5) were positive the distribution by malignancy disease was the following: 18 solid tumors, and 13 leukemias, and lymphomas. No statistically significant differences were found between the studied groups and positive results (P>0.05). The complementary laboratory tests, related to the presence of Pneumocystis, were not statistically significant either P>0.05). Is necessary to include this diagnosis in the microbiological differential study of low respiratory tract infections in oncological patients, since these patients show unspecific symptoms, and have a high possibility to develop pneumocystosis.

Evolução da pneumonia lipoide exógena em crianças: aspectos clínicos e radiológicos e o papel da lavagem broncoalveolar / Evolution of exogenous lipoid pneumonia in children: clinical aspects, radiological aspects and the role of bronchoalveolar lavage

OBJETIVO: Descrever os aspectos da evolução da pneumonia lipoide em crianças, com base em aspectos clínicos, radiológicos e de achados no lavado broncoalveolar, enfatizando a importância diagnóstica e terapêutica da lavagem broncoalveolar. MÉTODOS: Foram incluídas 28 crianças, com idade média de 20 meses (1-108 meses) e diagnóstico de pneumonia crônica refratária a antimicrobianos e/ou TB. A maioria apresentava um fator de risco para aspiração, e todas apresentavam história de ingestão de óleo mineral para o tratamento de constipação intestinal (23/28) ou de ascaridíase complicada (5/28). A avaliação clínica e tomográfica e análises do lavado broncoalveolar foram realizadas no início do tratamento e em até 24 meses. RESULTADOS: Os sintomas mais frequentes foram taquipneia e tosse. As principais alterações radiológicas foram consolidações (23/28), infiltrado peri-hilar (13/28) e hiperinsuflação (11/28). A TC de tórax mostrou consolidações com broncograma aéreo (24/28), diminuição de atenuação nas áreas de consolidação (16/28), opacidade em vidro fosco (3/28) e padrão em mosaico (1/28). O estudo do lavado broncoalveolar apresentava macrófagos espumosos corados por Sudan, confirmando o diagnóstico da pneumonia lipoide. Após tratamento com múltiplas lavagens broncoalveolares (média = 9,6), 20 crianças tornaram-se assintomáticas, havendo normalização tomográfica em 18. CONCLUSÕES: O diagnóstico de pneumonia lipoide deve ser considerado na pneumonia crônica ou TB refratárias ao tratamento, especialmente se houver história de ingestão de óleo mineral. A broncoscopia com múltiplas lavagens broncoalveolares mostrou-se eficiente para a depuração do óleo aspirado do parênquima pulmonar e a prevenção da fibrose, contribuindo para a redução da morbidade dessa doença, que ainda é pouco diagnosticada.

OBJECTIVE: To present aspects of the evolution of lipoid pneumonia in children, based on clinical, radiological and bronchoalveolar lavage fluid findings, emphasizing the importance of bronchoalveolar lavage for the diagnosis and treatment. METHODS: We included 28 children, with a mean age of 20 months (range, 1-108 months), diagnosed with chronic pneumonia refractory to antimicrobial therapy, with TB or with a combination of the two. Most of the children had at least one risk factor for aspiration, and all of them had a history of mineral oil ingestion for intestinal constipation (23/28) or complicated ascaridiasis (5/28). Clinical evaluations, tomographic evaluations and analyses of bronchoalveolar lavage fluid were carried out at the beginning of treatment and throughout a follow-up period of 24 months. RESULTS: Tachypnea and cough were the most common symptoms. The most common radiological alterations were areas of consolidation (23/28), perihilar infiltrates (13/28) and hyperinflation (11/28). Chest CT scans showed areas of consolidation with air bronchogram (24/28), decreased attenuation in the areas of consolidation (16/28), ground-glass opacities (3/28) and crazy-paving pattern (1/28). In the analysis of the bronchoalveolar lavage fluid, Sudan staining revealed foamy macrophages, confirming the diagnosis of lipoid pneumonia. After treatment with multiple bronchoalveolar lavages (mean = 9.6), 20 children became asymptomatic, 18 of those presenting normal tomographic images. CONCLUSIONS: A diagnosis of lipoid pneumonia should be considered in patients with chronic refractory pneumonia or TB, especially if there is a history of mineral oil ingestion. Bronchoscopy with multiple bronchoalveolar lavages was an efficient treatment for the clearance of mineral oil from the lung parenchyma and the prevention of fibrosis. This strategy contributed to reducing the morbidity of lipoid pneumonia, which remains a rare diagnosis.

Comparison of quantitative bronchoscopic lavage cultures [B-BAL] with blind NG Tube lavage [N-BAL] cultures in the diagnosis of ventilator associated pneumonia [VAP]

To compare quantitative Bronchoscopic Bronchoalveolar Lavage [B-BAL] cultures with blind nasogastric tube bronchoalveolar lavage [N-BAL] cultures in the diagnosis of Ventilator Associated Pneumonia [VAP]. Cross-sectional comparative study. The study was conducted in Medical ICU and Chest ICU at Liaquat National Hospital [LNH], Karachi, from January till August, 2007. Patients admitted in ICU with clinical suspicion of VAP, fulfilling inclusion criteria viz. fever, leukocytosis, new or worsening infiltrate in chest radiograph or purulent secretions were included. Exclusion criteria were deranged coagulopathy, extreme ventilatory and oxygenation demands and tracheal obstruction. All patients had N-BAL using 16 fr Nasogastric tube [NG] without lubrication followed by B-BAL. Samples collected were submitted to the laboratory for quantitative cultures and sensitivities. Results were documented on predesigned proforma. Fifty four patients underwent paired B-BAL and N-BAL sampling. The N-BAL sensitivity and specificity for N-BAL were 87.87% and 85.71% respectively and p-value was <0.001. N-BAL showed sensitivity of 87.87%, specificity of 75.71%, positive predictive value of 89.65%, negative predictive value of 77.77% and p <0.001, which was statistically significant. Significant differences in time required for sample collection and cost of both procedures were also observed. The results favour the use of blind NG tube bronchoalveolar lavage quantitative cultures as it is simple, safe, cost-effective and minimally invasive method of diagnosing VAP

Diagnosis of disseminated toxoplasmosis by polymerase chain reaction in bronchoalveolar lavage fluid of a patient with aids / Diagnóstico de toxoplasmose disseminada através de técnica de reação em cadeia da polimerase realizada no lavado bronco-alveolar de paciente com sida

Pulmonary toxoplasmosis is a challenging diagnosis in immunosuppressed patients with nonspecific clinical picture and radiologic findings. We present a case of pneumonia due to Toxoplasma gondii diagnosed by polymerase chain reaction (PCR) in the bronchoalveolar lavage (BAL) fluid of a patient with acquired immunodeficiency syndrome (AIDS). Coinfection with Pneumocystis jirovecii was found in the same specimen. Direct examination and culture for bacteria, mycobacteria and other fungus were negative. Despite the intensive management, respiratory compromise evolved rapidly, with the need for ventilatory support. Acute respiratory distress syndrome developed, and the patient died of multiple organ failure. This case illustrates that a high index of suspicion is necessary for diagnosis of pulmonary toxoplasmosis, a potentially fatal condition. Due to high diagnostic performance, PCR in BAL fluid should be included in the evaluation of immunosuppressed patients with nonspecific pulmonary diseases.

O diagnóstico de toxoplasmose pulmonar em pacientes imunossuprimidos é difícil, devido ao quadro clínico e aos achados radiológicos inespecíficos. Neste artigo, relatamos o caso de uma paciente com síndrome da imunodeficiência adquirida (SIDA), que apresentou pneumonia por Toxoplasma gondii diagnosticada através de reação em cadeia da polimerase (PCR) no lavado bronco-alveolar (LBA). A paciente apresentava co-infecção com Pneumocystis jirovecii. Os demais exames microbiológicos, como bacterioscópico, cultural para bactérias, micobactérias e fungos, foram negativos. Apesar do manejo intensivo, a paciente evoluiu com síndrome do desconforto respiratório agudo e óbito por falência múltipla dos órgãos. Este caso demonstra que um alto índice de suspeita clínica é necessário para o diagnóstico de pneumonia por Toxoplasma gondii. Devido ao seu desempenho diagnóstico, o PCR para Toxoplasma gondii no LBA deve ser incluído na avaliação de pacientes imunossuprimidos com quadros pulmonares inespecíficos.

TBC ganglionar en escolar: a propósito de un caso / Ganglionar tuberculosis (TBC)in a school: a purpose of a case

Mycobacterium tuberculosis es un bacilo acido alcohol resistente. Las formas clínicas de la enfermedad pueden ser intratoraxicas 80 por ciento o extratoraxicas. El diagnóstico se confirma al aislar el Mycobacterium por cultivo, muestras de aspiración gástrica, esputo, la prueba de Tuberculina para personas asintomáticas, líquido de lavado bronquial, líquido pleural y cefalorraquídeo, o muestra de biopsia. La mejor manera de diagnósticar tuberculosis pulmonar en niños de corta edad con tos no productiva es el material de aspirado estomacal en la mañana. El Tratamiento usado son las drogas de primera línea: Izoniacida, Rifampicina, Pirazinamida. Se trata de Escolar masculino de 7 años de edad, quien es traído por presentar de 4 meses de evolución fiebre no cuantificada que cede momentáneamente con antipirético. Concomitantemente de 8 días de evolución aumento de volumen en región supraclavicular izquierda que fue aumentando progresivamente, motivo por el cual acude a este centro asistencial decidiéndose su ingreso; con antecedente de Hermano falleció de 12 años por TBC Extrapulmonar. Se le realiza RX de torax muestra ensanchamiento del mediastino superior con imagen nodular izquierda y derecha, además de PPD (+) 15 mm.Rx de tórax que muestra ensanchamiento del mediastino superior donde se evidencia imagen nodular izquierda y derecha, así mismo TAC de cuello-tórax-abdominal que reporta Adenomegalia en región supraclavicular izquierda y conglomerados adenomegalicos en mediastino, biopsia de ganglio cervical que posteriormente reporta Bacilo Acido Alcohol Resistente. Se inicia tratamiento con Izoniacida, Rifampicina, Pirazinamida, con evolución clínica satisfactoria.

Lavado broncoalveolar em eqüinos: revisão de literatura Parte 2: achados citológicos / BronchoaIveolar lavage in horses: review of the literature - Part 2: cytological findings / Lavado broncoalveolar en equinos: revisión de literatura - parte 2: hallazgos citológicos

Nesta segunda parte, os autores revisaram as informações relativas à citologia broncoalveolar normal, bem como os padrões citopatológicos da hemorragia pulmonar induzida pelo exercício, da doença pulmonar obstrutiva crônica, doença inflamatória das vias aéreas, dos efeitos da estabulação e das infecções virais.

Normal bronchoalveolar cytology as well the cytopathologic pattern for exercise-incluced pulmonary hemorrhage, chronic obstructive pulmonary disease, inflammatory airway disease, viral infection and also from indoors effects were revised from the authors in this second part.

Evaluation of tracheal wash of horses with exercise-induced pulmonary hemorrhage treated with furosemide

Avaliaram-se os efeitos da furosemida sobre o lavado traqueal de eqüinos portadores de hemorragia pulmonar induzida pelo exercício (HPIE). Nove animais foram distribuídos em três grupos experimentais: grupo-controle, formado por três eqüinos hígidos e não portadores da doença; grupo 1, com três eqüinos portadores e não submetidos a tratamento; e grupo 2, com três eqüinos portadores da doença e tratados com furosemida na dose de 1mg/kg, quatro horas antes da atividade atlética. As amostras do lavado traqueal foram obtidas duas horas após o exercício físico e sua avaliação demonstrou que o uso do medicamento reduziu a severidade da HPIE, refletida pela redução no número relativo de hemossiderófagos (de 7,8 por cento no grupo 1 para 4,2 por cento no grupo 2) e no número de eritrócitos (de 77666 x 10³/ml no grupo 1 para 8000x10³/ml no grupo 2).

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.

Disnea progresiva e infiltrados pulmonares alveolares: acerca de un caso de Proteinosis Alveolar Pulmonar / Progresive Dyspnea, alveolar pulmonar infiltrates about a case of alveolar proteinosis

La evaluación de un paciente con síntomas respiratorios subagudos o crónicos como tos y disnea y con presencia de infiltrados pulmonares difusos requiere un protocolo de investigación para establecer su etiología y tratamiento específico. Se presenta un caso poco frecuente de paciente con enfermedad difusa del parénquima pulmonar, en quien por una serie de exámenes, incluyendo baciloscopías seriadas, radiografía de tórax, tomografía axial computarizada de alta resolución de tórax, espirometría, broncoscopía y biopsia por toracotomía abierta se diagnosticó Proteinosis Alveolar. El paciente ha tenido buena respuesta a los lavados pulmonares....