Síndrome de Hamman - rich: Reporte de caso / Hamman-Rich Syndrome: case report

Resumen La neumonía intersticial aguda, también conocida como síndrome de Hamman-Rich, se caracteriza por la presencia de insuficiencia respiratoria aguda, hallazgo imagenológico de infiltrados difusos bilaterales del parénquima pulmonar y daño alveolar difuso en histopatología pulmonar. Es una patología con una mortalidad mayor al 50% debido a la falta de una terapia específica más allá del manejo sintomático y soporte ventilatorio. Se expone el caso de un paciente masculino de 56 años quien se presenta con síntomas respiratorios inespecíficos, deterioro progresivo de la oxigenación y hallazgo de opacidades en vidrio esmerilado difusas bilaterales. Además, biopsia pulmonar en la que se evidencia daño alveolar difuso en fase fibroproliferativa en quien se descarta proceso infeccioso, enfermedades autoinmunes y toxicidad medicamentosa, por lo que se hace el diagnóstico. Recibió manejo con pulsos de metilprednisolona con recuperación completa del cuadro. MÉD.UIS.2021;34(3): 103-8.

Abstract Acute interstitial pneumonitis, also known as Hamman Rich syndrome, characterised by acute respiratory failure, bilateral lung infiltrates on radiographs and diffuse alveolar damage (DAD) on lung histopathology, is an exclusion diagnosis which requires a complete study of other possible etiologies of DAD. It is a pathology with a mortality greater than 50% due to not having a specific therapy beyond symptomatic management and ventilatory support. We present a case of a 56 year old male with respiratory symptoms, who has progressive worsening oxygen levels and bilateral ground glass opacities on chest images. Lung biopsy with diffuse alveolar damage, infection, autoimmune diseases, and drug adverse reaction were ruled out. Managed with mechanical ventilation and high dose systemic corticoids with complete recovery. MÉD.UIS.2021;34(3): 103-8.

Radiologic aspects of COVID-19 pneumonia: outcomes and thoracic complications. / Aspectos radiológicos de la neumonía COVID-19: evolución y complicaciones torácicas.

Outcomes vary widely in patients with COVID-19. Whereas some patients have only mild symptoms of short duration, others develop severe disease that leads to acute respiratory distress syndrome requiring prolonged stays in intensive care units. Radiologically, the initial stage is characterized by viral pneumonia with mild expression. In some patients, however, the onset of the immune response results in acute lung damage with organizing pneumonia and diffuse alveolar damage. Moderate-severe disease is associated with a high incidence of pulmonary embolisms, generally peripherally distributed and associated with endothelial damage, prolonged stays in bed, and coagulopathy. Other relatively common complications are spontaneous pneumothorax and pneumomediastinum due to the rupture of alveolar walls and barotrauma in mechanically ventilated patients. Superinfection, generally bacterial and less commonly fungal, is more common in patients with severe disease.

The first COVID-19 autopsy in Spain performed during the early stages of the pandemic.

We describe the implementation of a COVID-19 Autopsy Programme in our Hospital, report the main findings from the first autopsy of the programme and briefly review the reports of lung pathology of these patients.

Autopsy findings from the first known death from Severe Acute Respiratory Syndrome SARS-CoV-2 in Spain.

The new coronavirus SARS-CoV-2, first identified in Wuhan, China in December, 2019, can cause Severe Acute Respiratory Syndrome (SARS) with massive alveolar damage and progressive respiratory failure. We present the relevant autopsy findings of the first patient known to have died from COVID19 pneumonia in Spain, carried out on the 14th of February, 2020, in our hospital (Hospital Arnau de Vilanova-Lliria, Valencia). Histological examination revealed typical changes of diffuse alveolar damage (DAD) in both the exudative and proliferative phase of acute lung injury. Intra-alveolar multinucleated giant cells, smudge cells and vascular thrombosis were present. The diagnosis was confirmed by reverse real-time PCR assay on a throat swab sample taken during the patient's admission. The positive result was reported fifteen days subsequent to autopsy.

Airway Pathological Alterations Selectively Associated With Acute Respiratory Distress Syndrome and Diffuse Alveolar Damage - Narrative Review.

Acute respiratory distress syndrome (ARDS) is a frequent and life-threatening entity. Recently, it has been demonstrated that diffuse alveolar damage (DAD), which is considered the histological hallmark in spite of presenting itself in only half of living patients with ARDS, exerts a relevant effect in the ARDS outcome. Despite the fact that the bronchial tree constitutes approximately 1% of the lung volume, discovering a relation between DAD and bronchial tree findings could be of paramount importance for a few reasons; (a) it could improve the description of ARDS with DAD as a clinical-pathological entity, (b) it could subrogate DAD findings with the advantage of their more accessible and safer analysis and (c) it could allow the discovery of new therapeutic targets. This narrative review is focused on pathological airway changes associated to Diffuse Alveolar Damage in the context of Acute Respiratory Distress Syndrome. It is organized into five sections: main anatomical and functional features of the human airway, why it is necessary to study airway features associated to DAD in patients with ARDS, pathological airway changes associated with DAD in animal models of ARDS, pathological airway changes associated with DAD in patients with ARDS, and the newest techniques for studying the histology of the bronchial tree and lung parenchyma.

O que todo intensivista deve saber a respeito da síndrome do desconforto respiratório agudo e dano alveolar difuso? / What every intensivist should know about acute respiratory distress syndrome and diffuse alveolar damage

RESUMO A síndrome do desconforto respiratório agudo é um desafio para o intensivista. A característica principal desta doença aguda é o dano alveolar difuso, presente em cerca de metade dos pacientes com a síndrome. É claro que o suporte respiratório à síndrome do desconforto respiratório agudo tem melhorado gradualmente nas últimas décadas. É também evidente que todos estes procedimentos são benéficos, já que reduzem a lesão pulmonar e mantêm o paciente vivo. Isto deve ser interpretado como uma estratégia de ganho de tempo, até que o fator desencadeante ou de risco causal melhore, assim como a tempestade inflamatória diminua e o pulmão se cure. Por outro lado, todos - exceto dois tratamentos farmacológicos (bloqueadores neuromusculares e esteroides) - são incapazes de melhorar o desfecho da síndrome do desconforto respiratório agudo. A hipótese de que os resultados farmacológicos negativos podem ser explicados pela heterogeneidade histológica da síndrome do desconforto respiratório agudo tem sido apoiada pelas recentes demonstrações de que a síndrome com dano alveolar difuso tem característica clínico-patológica específica. O dano alveolar difuso é um diagnóstico patológico, e a biópsia pulmonar a céu aberto (a técnica mais comum para obtenção de tecido pulmonar) tem efeitos colaterais graves, sendo necessário que se desenvolvam biomarcadores substitutos para o dano alveolar difuso. O objetivo desta revisão é discutir três tópicos relacionados à síndrome do desconforto respiratório agudo: o relacionamento entre a síndrome do desconforto respiratório agudo e o dano alveolar difuso; como o dano alveolar difuso pode ser representado no quadro clínico; e como o enriquecimento pode melhorar os resultados de estudos clínicos farmacológicos realizados com pacientes com a síndrome e com dano alveolar difuso.

ABSTRACT Acute respiratory distress syndrome is a challenging entity for the intensivist. The pathological hallmark of the acute phase is diffuse alveolar damage, which is present in approximately half of living patients with acute respiratory distress syndrome. It is clear that respiratory support for acute respiratory distress syndrome has gradually been improving over recent decades. However, it is also evident that these procedures are beneficial, as they reduce lung injury and keep the patient alive. This could be interpreted as a time-gaining strategy until the trigger or causal or risk factor improves, the inflammatory storm decreases and the lung heals. However, all except two pharmacological treatments (neuromuscular blockers and steroids) were unable to improve the acute respiratory distress syndrome outcome. The hypothesis that pharmacological negative results may be explained by the histological heterogeneity of acute respiratory distress syndrome has been supported by the recent demonstration that acute respiratory distress syndrome with diffuse alveolar damage constitutes a specific clinical-pathological entity. Given that diffuse alveolar damage is a pathological diagnosis and that open lung biopsy (the most common technique to obtain lung tissue) has several side effects, it is necessary to develop surrogate biomarkers for diffuse alveolar damage. The aim of this narrative review is to address the following three topics related to acute respiratory distress syndrome: (a) the relationship between acute respiratory distress syndrome and diffuse alveolar damage, (b) how diffuse alveolar damage could be surrogated in the clinical setting and (c) how enrichment in diffuse alveolar damage may improve the results of pharmacological clinical trials tried out on patients with acute respiratory distress syndrome.

Acute Respiratory Distress: from syndrome to disease.

The acute respiratory distress syndrome (ARDS) is currently one of the most important critical entities given its high incidence, rate of mortality, long-term sequelae and non-specific pharmacological treatment. The histological hallmark of ARDS is diffuse alveolar damage (DAD). Approximately 50% of ARDS patients present DAD, the rest is made up of a heterogeneous group of histological patterns, many of which correspond to a well-recognized disease. For that reason, if these patterns could be diagnosed, patients could benefit from a treatment. Recently, the effect of DAD in clinical and analytical evolution of ARDS has been demonstrated, so the classical approach to ARDS as an entity defined solely by clinical, radiological and gasometrical variables should be reconsidered. This narrative review aims to examine the need to evolve from the concept of ARDS as a syndrome to ARDS as a specific disease. So we have raised 4 critical questions: a) What is a disease?; b) what is DAD?; c) how is DAD considered according to ARDS definition?, and d) what is the relationship between ARDS and DAD?

Embolismo de líquido amniótico / Amniotic fluid embolism

Se valoró un caso de una femenina quien es internada por embarazo en vías de prolongación para inducción del mismo, posterior a su labor presenta sangrado transvaginal abundante por lo que es ingresada a sala de operaciones, no se le encuentra sitio de sangrado, le realizan histerectomía y fallece; es enviada para su respectiva autopsia, se determina como causa de muerte: embolismo de líquido amniótico. Este artículo pretende revisar la etiología de esta patología, fisiopatología, criterios diagnósticos del mismo, factores de riesgo, diagnósticos diferenciales y su tratamiento.

A case of a female who is hospitalized for pregnancy-way extension for induction thereof, after their work presents TVB abundant so it is entered into operating room were assessed, you will not find the bleeding site, we performed hysterectomy and dies; is sent to the respective autopsy determined the cause of death: amniotic fluid embolism. This article reviews the etiology of this pathology, pathophysiology, diagnostic criteria thereof, risk factors, differential diagnosis and treatment.

Caso anatomopatológico influenza AH1N1 en una paciente embarazada: características clínicas y patológicas / Pathological Case AH1N1 influenza in a pregnant patient: clinical and pathological features

Se describen los hallazgos clínicos y patológicos de la infección por influenza A(H1 N1), basados en un caso de autopsia. La paciente, una mujer de 25 años embarazada de gemelos, con 33 semanas de gestación y s¡ntomas similares a influenza, quien fue admitida en el hospital y murió 13 días después. En la autopsia, el principal hallazgo fue daño alveolar difuso en fase proliferativa...

Juvenile dermatomyositis (JDM) and severe pulmonary involvement: case report / Dermatomiosite juvenil (DMJ) e comprometimento pulmonar grave: relato de caso

A dermatomiosite juvenil (DMJ) é uma doença inflamatória não supurativa dos músculos esqueléticos e da pele.Outros órgãos podem estar envolvidos, como, por exemplo, os pulmões. As complicações pulmonares estão associadas a altas taxas de morbimortalidade e podem ocorrer na fase aguda da doença. Devido à raridade, dificuldade no diagnóstico e gravidade do acometimento pulmonar, relatamos o caso de uma menina de 3 anos com DMJ e envolvimento pulmonar que evoluiu para óbito.

Juvenile dermatomyositis (JDM) is an inflammatory, non-pustular disorder of skeletal muscles and skin. Other organs, such as the lungs, can be involved. Pulmonary complications are associated with high morbimortality rates and can develop in the acute phase of the disease. Due to its rarity, diagnosis difficulty and severity pulmonary involvement, we report the case of a 3-year-old girl with JDM and pulmonary involvement and evolution to death.