Calcificaciones pulmonares metastásicas, 3 casos clínicos / Metastatic pulmonary calcifications: three clinical cases

Las calcificaciones pulmonares metastásicas, hacen referencia a una enfermedad metabólica, caracterizada por depósitos de calcio en tejido pulmonar sano. La etiología es amplia e incluye enfermedades malignas y benignas, siendo la falla renal la causa más frecuente. Es una condición, que, a pesar de ser frecuente, suele ser subdiagnosticada, por presentar pocos o ningún síntoma. Presentamos tres casos clínicos asociados a enfermedad renal crónica, pre y post trasplante.

Metastatic pulmonary calcifications refer to a metabolic disease, characterized by calcium deposits in healthy lung tissue. The etiology is broad and includes malignant and benign diseases, the kidney failure being the most frequent cause. It is a condition, which, despite being frequent, is usually underdiagnosed, because it presents few or no symptoms. We present three clinical cases associated with pre- and post-transplant kidney disease.

Hemosiderosis pulmonar idiopática, caso clínico en México. Importancia de un diagnóstico y tratamiento oportuno / Idiopathic pulmonary hemosiderosis, clinical case in Mexico. The relevance of performed an early diagnosis and treatment

La hemosiderosis pulmonar idiopática (HPI) es una patología poco frecuente; su distribución geográfica, su incidencia y prevalencia se desconocen de manera exacta a nivel mundial. Tiene una fuerte asociación con condiciones autoinmunes y una adecuada respuesta al tratamiento inmunosupresor. A pesar de ser una patología grave, presenta una tasa de morbilidad y mortalidad mediana, siempre que se realice un diagnóstico y tratamiento precoz. Se presenta el caso clínico de una paciente femenina con diagnóstico de HPI quien cursó con la triada clásica de esta enfermedad: hemoptisis, anemia ferropénica e infiltrados pulmonares difusos. Se descartaron otras causas de hemorragia pulmonar difusa y se realizó el diagnóstico por biopsia pulmonar. Se trató con esteroides sistémicos e inhalados y azatioprina. Tras casi 2 años después del diagnóstico, estando sin tratamiento por 3 meses, presentó una exacerbación con hemorragia pulmonar masiva ocasionando el fallecimiento de la paciente.

Idiopathic pulmonary hemosiderosis (IPH) is a rare pathology; its geographic distribution, incidence and prevalence are not accurately known worldwide. It has a strong association with autoimmune conditions and has an adequate response to immunosuppressive treatment. Despite being a serious pathology, it has a medium morbidity and mortality rate, provided that early diagnosis and treatment is performed. We present the clinical case of a female patient diagnosed with IPH who presented with the classic triad of this disease: hemoptysis, iron deficiency anemia and diffuse pulmonary infiltrates. Other causes of diffuse pulmonary hemorrhage were ruled out and the diagnosis was made by lung biopsy. She was managed with systemic and inhaled steroids and azathioprine. After almost 2 years before the diagnosis, being without treatment for 3 month she had a massive pulmonary hemorrhage, causing the death of the patient.

Características clínicas y radiológicas de las malformaciones pulmonares congénitas: reporte de cuatro casos en un centro de tercer nivel de la ciudad de México / Clinical and radiological characteristics of congenital lung malformations: report of four cases in a third - level center in Mexico city

Las malformaciones pulmonares congénitas (MPC) conforman un grupo de entidades originadas por alteraciones en la embriogénesis del pulmón y de las vías respiratorias que se producen de acuerdo al nivel del árbol traqueobronquial donde se dé el insulto o el momento de la edad gestacional. Las entidades que en la actualidad forman parte de las MPC son: malformación congénita de la vía aérea, secuestro pulmonar, quiste broncogénico, enfisema lobar congénito, atresia bronquial. Su diagnóstico puede realizarse desde la etapa prenatal, al momento del nacimiento, en la edad pediátrica o adulta por la aparición de síntomas o incidentalmente en pruebas radiológicas. El manejo de estas lesiones depende del tipo de malformación y de la severidad de los síntomas, por lo que se debe individualizar la conducta a seguir en cada caso. Aunque la mayoría de los autores recomienda la resección de la lesión, no existe en la actualidad un consenso sobre la indicación de cirugía, sobre todo en pacientes asintomáticos. Nuestro objetivo es describir los hallazgos clínicos, radiológicos y en algunos casos anatomopatológicos así como el tratamiento empleado de cuatro casos clínicos, atendidos en un hospital de tercer nivel que ejemplifican las presentaciones más frecuentes de las MPC.

Congenital pulmonary malformations (CPM) make up a group of entities caused by alterations in the embryogenesis of the lung and the respiratory tract that occur according to the level of the tracheobronchial tree where the insult occurs or the moment of gestational age. The entities that are currently part of the CPM are: congenital malformation of the airway, pulmonary sequestration, bronchogenic cyst, congenital lobar emphysema, bronchial atresia. Its diagnosis can be made from the prenatal stage, at birth or in pediatric or adult age due to the appearance of symptoms or incidentally in radiological tests. The management of these injuries depends on the type of malformation and the severity of the symptoms, so the conduct to be followed in each case must be individualized. Although most authors recommend resection of the lesion, there is currently no consensus on the indication for surgery, especially in asymptomatic patients. Our objective is to describe the clinical, radiological and in some cases histopathological findings, as well as the treatment used in four clinical cases, treated in a tertiary level hospital that exemplify the most frequent presentations of MPC.

Hemosiderosis pulmonar idiopática: seguimiento de pacientes durante 25 años (1995-2019) / Idiopathic pulmonary hemosiderosis: follow-up of patients for 25 years (1995-2019)

La hemosiderosis pulmonar idiopática (HPI) es una causa de hemorragia alveolar difusa. OBJETIVO: describir la evolución de niños con HPI en nuestra institución. Se realizó una revisión retrospectiva con protocolo de seguimiento. Se reclutaron 13 pacientes, 7 hombres. Procedentes de una zona agrícola (6/13). No todos presentaron la tríada diagnóstica completa: infiltrados algodonosos (9/13), anemia (11/13), hemoptisis (9/13). Todos evidenciaron un recuento de hemosiderófagos sobre 30% en el lavado broncoalveolar. Tomografía computada de tórax: normal (5/13), patrón intersticial (5/13), vidrio esmerilado (2/13) y fibrosis (1/13). Espirometría: normal (7/13), restrictiva (4/13), obstructiva (1/13) y no efectuada (1/13). Tratamiento durante la fase aguda: bolos de metilprednisolona (7/13) o prednisona (6/13) o hidrocortisona (1/13). En la fase de mantención se administró: prednisona (13/13) más un inmunosupresor, azathioprina (12/13), hidroxicloroquina (1/13), micofenolato (1/13), más budesonida MDI (13/13). Ocho pacientes detuvieron los sangrados. Dos pacientes fallecieron y hubo cinco embarazos de curso fisiológico en 3 adolescentes. Se observó: a) diferentes modalidades de presentación que retrasaron el diagnóstico; b) gran exposición a pesticidas; c) mejor pronóstico si el diagnóstico y el tratamiento eran precoces, también en niñas adolescentes; d) la mayoría detuvo los episodios de sangrado.

Idiopathic pulmonary hemosiderosis (IPH) is a cause of diffuse alveolar hemorrhage. OBJECTIVE: to describe the evolution of children with IPH in our institution. Retrospective monitoring with a follow-up protocol was carried out. 13 patients, seven males, were recruited. From an agricultural area (6/13). Not all of patients had the complete diagnostic triad: cotton infiltrates (9/13), anemia (11/13), hemoptysis (9/13). Hemosiderin-laden macrophages counting in the bronchoalveolar lavage fluid was over 30% in all the patients. Computed chest tomography was informed as normal (5/13), interstitial pattern (5/13), ground glass (2/13) and fibrosis (1/13). Spirometry: normal (7/13), restrictive (4/13), obstructive (1/13) and not performed (1/13). Treatment during the acute phase: bolus of methylprednisolone (7/13) or prednisone (6/13) or hydrocortisone (1/13). In the maintenance phase: prednisone (13/13) plus an immunosuppressant, azathioprine (12/13), hydroxychloroquine (1/13), mycophenolate (1/13), plus budesonide MDI (13/13). Eight patients stopped the bleeding episodes. Two patients died and there were five physiological pregnancies in 3 adolescents. It was observed:(a) different modes of IPH presentation that delayed its diagnosis; (b) large exposure to pesticides; (c) prognosis improved if diagnosis and treatment were early, also in adolescent girls; (d) most of the patients stopped the bleeding episodes.

Linfangiomatosis pulmonar difusa con compromiso pleural y pericárdico. Reporte de un caso pediátrico / Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report

La linfangiomatosis pulmonar difusa es una enfermedad rara caracterizada por una marcada proliferación y dilatación de los vasos linfáticos en los pulmones, la pleura y el mediastino. Se desconoce la prevalencia, y la etiología no se comprende completamente.Una niña de 22 meses ingresó por poliserositis, con derrame pericárdico y pleural. Requirió pericardiocentesis y avenamiento pleural, y presentó drenaje de quilo (1,5-4 litros/día) sin respuesta al tratamiento médico (ayuno, nutrición parenteral y octreotide). Se realizó biopsia pulmonar. La anatomía patológica mostró hallazgos compatibles con linfangiomatosis difusa pulmonar. Comenzó tratamiento con sirolimus y propanolol, que disminuyeron las pérdidas por el drenaje pleural a la semana. Presentó buena evolución; suspendió aporte de oxígeno y se retiró el drenaje pleural. Se externó al cuarto mes de internación. El diagnóstico temprano de la linfangiomatosis pulmonar difusa es difícil de lograr, pero permite aplicar terapéuticas que evitan la progresión de enfermedad y disminuir la morbimortalida

Diffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs, pleura, and mediastinum. The prevalence is unknown and the etiology is not fully understood.A 22-month-old girl was admitted for polyserositis, with pericardial and pleural effusion. She required pericardiocentesis and pleural drainage, presenting chyle drainage (1.5-4 liters/day) without response to medical treatment (fasting, parenteral nutrition and octreotide). A lung biopsy was performed. The pathological anatomy showed findings compatible with diffuse pulmonary lymphangiomatosis. Treatment with sirolimus and propanolol began, decreasing losses due to pleural drainage one week after treatment. She progressed well, discontinued oxygen supply and pleural drainage was removed, leaving the patient after the fourth month of hospitalization.Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality.

Differential diagnoses of acute ground-glass opacity in chest computed tomography: pictorial essay / Diagnósticos diferenciais de opacidade em vidro fosco aguda na tomografia computadorizada de tórax: ensaio pictórico

ABSTRACT Ground-glass opacity is a very frequent and unspecified finding in chest computed tomography. Therefore, it admits a wide range of differential diagnoses in the acute context, from viral pneumonias such as influenza virus, coronavirus disease 2019 and cytomegalovirus and even non-infectious lesions, such as vaping, pulmonary infarction, alveolar hemorrhage and pulmonary edema. For this diagnostic differentiation, ground glass must be correlated with other findings in imaging tests, with laboratory tests and with the patients' clinical condition. In the context of a pandemic, it is extremely important to remember the other pathologies with similar findings to coronavirus disease 2019 in the imaging exams.

RESUMO A opacidade em vidro fosco é uma alteração muito frequente e pouco específica na tomografia computadorizada de tórax. Ela admite grande leque de diagnósticos diferenciais no contexto agudo, desde pneumonias virais, como as causadas pelo vírus influenza, pela doença do coronavírus 2019 e pelo citomegalovírus, até mesmo lesões de origem não infecciosa, como vaping , infarto pulmonar, hemorragia alveolar e edema pulmonar. Para essa diferenciação diagnóstica, deve-se correlacionar o vidro fosco com os demais achados nos exames de imagem, exames laboratoriais e quadro clínico do paciente. É de suma importância, no contexto de pandemia, recordar as demais patologias com os achados semelhantes aos da doença do coronavírus 2019 nos exames de imagem.

Ecografía pulmonar en neonatos / Lung ultrasound in newborns

Introducción: La ecografía pulmonar presenta diferentes ventajas con respecto a otras técnicas de imagen diagnóstica. Consiste en un procedimiento dirigido, rápido, menos costoso y que no expone a los pacientes a radiación ionizante. Objetivo: Comprobar la capacidad diagnóstica de la ecografía pulmonar en neonatos y su viabilidad como método diagnóstico alternativo a los principales métodos actuales. Métodos: Se realizó una búsqueda bibliográfica en las siguientes bases de datos y buscador de artículos: Medline, Cinahl, Scopus, Web of science, durante los meses de enero y febrero de 2018. Se localizaron 96 artículos, de los cuales se retiraron 80 tras aplicar los criterios de exclusión. La muestra quedó, por lo tanto, conformada por 16 artículos publicados en los últimos cinco años. Resultados: En 6 de los artículos seleccionados, se comparó la ecografía pulmonar con otras pruebas de diagnóstico por imagen, otros 6 evaluaron los hallazgos encontrados en pacientes ya diagnosticados y en los 4 restantes, los autores establecieron una clasificación según los signos ecográficos. Consideraciones finales: La ecografía pulmonar tiene resultados positivos en la totalidad de los estudios analizados, por ello, parece una herramienta diagnóstica de enfermedad pulmonar neonatal viable en un futuro próximo, que, además, tiene múltiples ventajas con respecto a otros métodos(AU)

Introduction: Lung ultrasound has different advantages in respect to other imaging diagnosis techniques. It is a directed, quick, less expensive technique and in which the patient is not exposed to ionizing radiation. Objective: To confirm the diagnosis ability of lung ultrasound in newborns and its feasibility as a diagnosis method to alternate with the current main procedures. Methods: It was carried out a bibliographic search in databases and articles searchers as Medline, Cinahl, Scopus, Web of science during January and February 2018. 96 articles were found but 80 of them were removed after implementing the exclusion criteria. Therefore, the sample was formed by 16 articles published in the last five years. Results: In 6 of the selected articles it was compared lung ultrasound with other imaging diagnosis tests; other 6 articles assessed the findings in already diagnosed patients, and in the other 4 patients it was established a classification according to the echographic signs. Final considerations: Lung ultrasound has positive results in all the studies assessed, so, it looks like a viable tool in the near future for the diagnosis of neonatal lung disease, and in addition it has multiple advantages with respect to other methods(AU)

El Signo De Halo Invertido En Tomografía Computada De Tórax / The Inverted Halo Sign in Thoracic Computed Tomography

El signo del halo invertido se caracteriza por una opacidad central de vidrio esmerilado rodeado por una consolidación del espacio aéreo más densa en forma de una media luna o un anillo. El signo del halo invertido se ha informado en asociación con un am­ plia gama de enfermedades pulmonares, incluidas las infecciones fúngicas pulmonares invasivas, neumonía por pneumocystis, tuberculosis, neumonía adquirida en la comuni­ dad, granulomatosis linfomatoide, granulomatosis de Wegener, neumonía lipoidea y sarcoidosis. También se observa en neoplasmas pulmonares e infarto y después de ra­ dioterapia y ablación por radiofrecuencia de neoplasias malignas pulmonares. También es conocido como signo de halo en reversa o signo del atolón.

The reversed halo sign is characterized by a central ground­glass opacity surrounded by denser air­space consolidation in the shape of a crescent or a ring. The reversed halo sign has been reported in association with a wide range of pulmonary diseases, in­ cluding invasive pulmonary fungal infections, pneumocystis pneumonia, tuberculosis, community­acquired pneumonia, lymphomatoid granulomatosis, Wegener granulomato­ sis, lipoid pneumonia and sarcoidosis. It is also seen in pulmonary neoplasms and in­ farction, and following radiation therapy and radiofrequency ablation of pulmonary malignancies. It is also known as a reverse halo sign or atoll sign

Multiple cavitary lung lesions on CT: imaging findings to differentiate between malignant and benign etiologies / Lesões escavadas pulmonares múltiplas em TC: achados de imagem para diferenciação entre etiologia maligna e benigna

ABSTRACT Objective: To determine the CT findings of multiple cavitary lung lesions that allow the differentiation between benign and malignant etiologies. Methods: We reviewed CT scans, including patients with two or more cavitary lung lesions. We evaluated the number of cavitary lesions, their location, cavity wall thickness, and additional findings, correlating the variables with the diagnosis of a benign or malignant lesion. Results: We reviewed the chest CT scans of 102 patients, 58 (56.9%) of whom were male. The average age was 50.5 ± 18.0 years. Benign and malignant lesions were diagnosed in 74 (72.6%) and 28 (27.4%) of the patients, respectively. On the CT scans, the mean number of cavities was 3, the mean wall thickness of the largest lesions was 6.0 mm, and the mean diameter of the largest lesions was 27.0 mm. The lesions were predominantly in the upper lobes, especially on the right (in 43.1%). In our comparison of the variables studied, a diagnosis of malignancy was not found to correlate significantly with the wall thickness of the largest cavity, lymph node enlargement, emphysema, consolidation, bronchiectasis, or bronchial obstruction. The presence of centrilobular nodules correlated significantly with the absence of malignant disease (p < 0.05). In contrast, a greater number of cavities correlated significantly with malignancy (p < 0.026). Conclusions: A larger number of cavitary lung lesions and the absence of centrilobular nodules may be characteristic of a malignant etiology. However, on the basis of our evaluation of the lesions in our sample, we cannot state that wall thickness is a good indicator of a benign or malignant etiology.

RESUMO Objetivo: Determinar os achados tomográficos de lesões escavadas pulmonares múltiplas que contribuem para a diferenciação entre etiologia benigna e maligna. Métodos: Foram revisados exames de TC, sendo incluídos pacientes com duas ou mais lesões pulmonares escavadas. Avaliaram-se a quantidade de lesões escavadas, sua localização, espessura parietal das lesões e achados adicionais, correlacionando as variáveis com a presença de diagnóstico de benignidade ou de malignidade. Resultados: Foram revisadas TCs de tórax de 102 pacientes, sendo 58 (56,9%) desses do sexo masculino. A média de idade foi de 50,5 ± 18,0 anos. Lesões benignas e malignas foram diagnosticadas em 74 pacientes (72,6%) e em 28 (27,4%), respectivamente. Quanto aos achados tomográficos, a média do número de cavidades foi 3, a da espessura média da parede da maior lesão foi de 6,0 mm, e a do diâmetro da maior lesão foi de 27,0 mm. Houve predomínio das lesões em lobos superiores, especialmente no direito (43,1%). Após a comparação das variáveis estudadas, a espessura parietal da maior escavação, assim como a presença de linfonodomegalia, enfisema, consolidação, bronquiectasias e obstrução brônquica, não apresentaram diferenças estatisticamente significativas para o diagnóstico de malignidade. A presença de nódulos centrolobulares correlacionou-se significativamente com a ausência de doença maligna (p < 0,05). Observou-se também que um número maior de cavidades se correlacionou significativamente com malignidade (p < 0,026). Conclusões: Um maior número de lesões pulmonares escavadas e a ausência de nódulos centrolobulares podem ser características relacionadas à etiologia maligna. Por outro lado, a espessura parietal não permitiu a diferenciação entre etiologia benigna e maligna das lesões em nossa amostra.

Ultrasonido pulmonar en el manejo del paciente crítico. Conceptos básicos y aplicación clínica / Lung ultrasound in critical care. Clinical application

Lung ultrasound has had a great development in the critical patient management in the last decade. It is a safe, non-invasive and radiation-free tool that allows examining the patient at the bedside without the need for transfer. The last characteristic is particularly beneficial in patients with hypoxemia, hemodynamic instability and with high-risk of nosocomial contamination, as currently occurs in the pandemic caused by the outbreak of the new coronavirus 2019 disease (COVID-19). Lung ultrasound can be used to assess lung aeration in the patient under mechanical ventilation, evaluating the response to different strategies, personalizing lung recruitment maneuvers, and guiding the weaning process. This review describes the basic principles of lung ultrasound to obtain the images and interpret them. Lung ultrasound provides anesthesiologists, intensivists and respiratory therapists a safe and reliable tool for the diagnosis and follow-up of the main pulmonary diseases in the critical ill patient.

El ultrasonido pulmonar ha tenido un gran desarrollo en el abordaje del paciente crítico en las últimas décadas. Constituye una herramienta segura, no invasiva y libre de radiación, que permite examinar al paciente sin necesidad de traslado. Esta última característica es particularmente beneficiosa en pacientes hipóxicos, inestables hemodinámicamente o con alto riesgo de contaminación nosocomial, como ocurre actualmente con la pandemia ocasionada por el brote de la enfermedad del nuevo coronavirus 2019 (COVID-19). El ultrasonido pulmonar puede ser usado, además, para evaluar y monitorizar la aireación pulmonar en el paciente en ventilación mecánica, personalizando maniobras de reclutamiento, testeando la respuesta a diferentes estrategias ventilatorias y monitorizando el proceso de weaning. Esta revisión describe los principios básicos del ultrasonido pulmonar para la obtención de imágenes y su interpretación. Proporcionando a médicos anestesiólogos, intensivistas y kinesiólogos respiratorios una herramienta segura y confiable para el diagnóstico y seguimiento de las principales patologías pulmonares en el paciente crítico.

Sífilis secundaria con compromiso pulmonar / Secondary syphilis with pulmonary involvement

La sífilis es una enfermedad de transmisión sexual causada por una espiroqueta, Treponema palidum. Presentamos el caso de una mujer de 62 años de edad, que consultó por fiebre de 38°, dolor torácico en puntada de costado y lesiones pruriginosas en piel. Se realizó examen de laboratorio de sangre periférica, radiografía y tomografía de tórax. Recibió tratamiento antibiótico y fue diagnosticada como neumonía aguda de la comunidad. Debido a la respuesta parcial de los síntomas y persistencia de lesiones pruriginosas se realizó biopsia de piel que informó Treponema palidum, el cual fue confirmado con test serológico VDRL y FtA-abs positivo. La paciente recibió 4 dosis de penicilina G benzatínica con favorable evolución de las lesiones en piel y mejoría de las imágenes radiológicas.

We present the case of a 62-year-old woman who consulted for fever (38°), stabbing thoracic pain (on one side), and pruritic skin lesions. She underwent peripheral blood tests, chest X-rays and CT. Her symptoms were interpreted as severe community-acquired pneumonia. After a treatment with antibiotics, her skin lesions persisted, and other symptoms were only partially relieved. A skin biopsy was performed, which revealed Treponema pallidum. Such finding was confirmed through positive serum VDRL and FTA-ABS tests. The patient received 4 doses of benzathine penicillin G with favorable evolution of skin lesions and improvement of radiological images.

Protocolo ultrasonográfico enfocado en cirugía cardiaca posquirúrgica CCROSS (Cardiac, Cerebral, Renal, Optic nerve, lung ultraSound Study) / Ultrasonographic protocol focused on post cardiac surgery CCROSS (Cardiac, Cerebral, Renal, Optic nerve, lung ultraSound Study)

Resumen El uso de ecocardiografía enfocada es de gran utilidad en la valoración, tratamiento y seguimiento del paciente en estado crítico. Es, junto con la clínica y el estetoscopio, una herramienta que complementa el actuar del médico ante las diversas etiologías que determinan un estado de choque o aumentan la morbimortalidad, especialmente en pacientes posquirúrgicos de cirugía cardíaca, en quienes no se tiene algoritmos de manejo emergente en el posquirúrgico. Ante tal situación, en el Instituto Nacional de Cardiología Ignacio Chávez se ha realizado y propuesto un algoritmo de manejo en los pacientes posquirúrgicos cardíacos: mediante ultrasonografía enfocada, abarcando ecoscopia transtorácica, ultrasonido pulmonar, ultrasonido del nervio óptico y renal mediante la valoración de índices resistivos renales. Diversas sociedades han creado sus protocolos de abordaje en pacientes en estado crítico, por lo que en el Instituto, específicamente en la terapia intensiva cardiovascular, ha creado el protocolo CCROSS (Cardiac, Cerebral, Renal, Optic nerve, lung ultraSound Study) para el abordaje inicial de estos pacientes y se encuentra en marcha actualmente un estudio para su validación, reproducibilidad y eficacia.

Abstract The use of echocardiography is very useful in the evaluation, treatment and follow-up of the patient in critical condition. Along with clinic and the stethoscope, it is a tool that complements the act of the physician faced with the diversity of etiologies that determine the state of shock and increase morbidity and mortality, especially in post cardiac surgery patients, in whom there are no management emergency postsurgical algorithms. In view of this situation, at the National Institute of Cardiology Ignacio Chávez, a management algorithm has been made and improved in cardiac postsurgical patients: through focused ultrasonography, including transthoracic echography, pulmonary ultrasound, optic nerve ultrasound, and renal ultrasound by evaluating renal resistive indices. Several societies have created their protocols for addressing patients in critical condition, so in the Institute, specifically in cardiovascular intensive therapy, has created the CCROSS protocol (Cardiac, Cerebral, Renal, Optic nerve, lung UltraSound Study) for the initial approach of these patients, and it is being carried out a study for its validation, reproducibility and efficacy.

El intersticio pulmonar y sus enfermedades en la infancia. Confusión diagnóstica en el recién nacido. Enfermedades genéticas del surfactante pulmonar. A propósito de un caso clínico: clínica, radiología, estudios y diagnóstico diferencial / The pulmonary interstitium and its diseases in childhood. Diagnostic confusion in the new born. Genetic diseases of pulmonary surfactant. About a clinical case: clinical, radiology, studies and differential diagnosis

Se presenta el caso clínico de un lactante fallecido a los siete meses de edad con cuadro intersticial persistente. Objetivos: describir detalladamente el camino diagnóstico; alertar sobre posibles confusiones en recién nacidos con diagnósticos más frecuentes; detallar los hallazgos clínicos, radiológicos y de anatomía patológica (consultas en el exterior). Metodología: sumatoria de estudios complejos para descartar causas más frecuentes de patología intersticial en el lactante; consultas radiológicas, de anatomía patológica y genética en el exterior del país. Resultado: con diagnóstico de PAP (proteinosis alveolar pulmonar) se encontró una duplicación de material genético a nivel de cromosoma X, correspondiente al gen CSF2RA (colony stimulating factor 2-subunidad a). Este gen codifica al receptor CSF2 cuya citoquina controla la producción, diferenciación y función de granulocitos/macrófagos. (AU)

A clinical case of a deceased seven month old infant presenting persistent interstitial lung compromise is presented. Objectives. Detailed description of the diagnostic pathway used; to alert about possible confusion with other ­ more frequent ­ pathologies in the new borninfant age; to present clinical, radiological, genetic and pathology findings (consultations abroad). Methodology. A complete description of complex studies to rule out other more frequent pathologies is presented together with radiological, pathological and genetic results from consultations abroad. Results. A diagnosis of PAP (pulmonary alveolar proteinosis) was confirmed with duplication of genetic material at CSF2RA gene (colony stimulating factor 2-subunit a). This gene codifies the CSF2 receptor whose cytokine controls production, differentiation and function of granulocytes/macrophages. (AU)

Endobronchial ultrasound: a minimally invasive technology to assist diagnosis of thoracic diseases / Ultrassonografia endobrônquica: tecnologia minimamente invasiva para auxiliar no diagnóstico de doenças torácicas

ABSTRACT The endobronchial ultrasound is a minimally invasive technique that simultaneously associates ultrasound and bronchoscopy, to visualize lung nodule or masses, airway wall, and structures adjacent to the tracheobronchial tree. Endobronchial ultrasound has been incorporated into clinical practice all over the world because of its low risk and high diagnostic yield in neoplastic and non-neoplastic disease.

RESUMO A ultrassonografia endobrônquica é uma técnica minimamente invasiva que associa simultaneamente broncoscopia à ultrassonografia, com a finalidade de visualizar nódulos ou massas pulmonares, paredes das vias aéreas, e estruturas ao redor de toda a árvore traqueobrônquica. A ultrassonografia endobrônquica foi incorporada à prática clínica em todo o mundo devido a seu baixo risco e elevado rendimento diagnóstico em doenças neoplásicas e não neoplásicas.

Niemann-Pick disease type B: HRCT assessment of pulmonary involvement / Doença de Niemann-Pick tipo B: avaliação do comprometimento pulmonar por TCAR

ABSTRACT Objective: To analyze HRCT findings in patients with Niemann-Pick disease (NPD) type B, in order to determine the frequency of HRCT patterns and their distribution in the lung parenchyma, as well as the most common clinical characteristics. Methods: We studied 13 patients (3 males and 10 females) aged 5 to 56 years. HRCT images were independently evaluated by two observers, and disagreements were resolved by consensus. The inclusion criteria were presence of abnormal HRCT findings and diagnosis of NPD type B confirmed by histopathological examination of a bone marrow, lung, or liver biopsy specimen. Results: The most common clinical findings were hepatosplenomegaly and mild to moderate dyspnea. The most common HRCT patterns were smooth interlobular septal thickening and ground-glass opacities, which were both present in all patients. Intralobular lines were present in 12 patients (92.3%). A crazy-paving pattern was observed in 5 patients (38.4%), and areas of air trapping were identified in only 1 case (7.6%). Pulmonary involvement was bilateral in all cases, with the most affected area being the lower lung zone. Conclusions: Smooth interlobular septal thickening, with or without associated ground-glass opacities, in patients with hepatosplenomegaly is the most common finding in NPD type B.

RESUMO Objetivo: Analisar os achados de TCAR em pacientes com doença de Niemann-Pick (DNP) tipo B a fim de avaliar a frequência dos padrões tomográficos e sua distribuição no parênquima pulmonar, além das características clínicas mais frequentes. Métodos: Foram estudados 13 pacientes (3 do sexo masculino e 10 do sexo feminino) com idades variando de 5 a 56 anos. As imagens de TCAR foram avaliadas por dois observadores de forma independente, e os casos discordantes foram resolvidos por consenso. Os critérios de inclusão foram presença de anormalidades na TCAR e diagnóstico confirmado de DNP tipo B por exame anatomopatológico através de biópsias de medula óssea, pulmão ou fígado. Resultados: Os achados clínicos mais comuns foram hepatoesplenomegalia e dispneia leve a moderada. Os padrões tomográficos mais frequentes foram espessamento liso de septos interlobulares e opacidades em vidro fosco, presentes em todos os pacientes. Linhas intralobulares estiveram presentes em 12 pacientes (92,3%). O padrão de pavimentação em mosaico foi observado em 5 pacientes (38,4%). Áreas de aprisionamento aéreo foram identificadas em 1 dos casos (7,6%). O comprometimento pulmonar foi bilateral em todos os casos, sendo o terço inferior dos pulmões a região mais envolvida. Conclusões: O achado de espessamento liso de septos interlobulares, com ou sem opacidades em vidro fosco associadas, em pacientes com hepatoesplenomegalia é o achado mais frequente na DNP tipo B.