A Curious Case of Biopsy-Proven Usual Interstitial Pneumonia.

Usual interstitial pneumonia (UIP) refers to a combination of radiologic and histologic findings, which include patchy interstitial fibrosis with fibroblastic foci and dense acellular collagen that causes architectural distortion due to scarring and honeycomb change with alternating areas of normal lungs. The UIP pattern is not a synonymous term with idiopathic pulmonary fibrosis (IPF). IPF is diagnosed when an etiologic workup has been performed, deemed to be unrevealing, with a radiologic or histologic UIP pattern. While the 2018 American Thoracic Society (ATS)/European Respiratory Society (ERS) guideline categories of UIP help eliminate the need for surgical lung biopsy (SLB) in two categories, i.e., "definite UIP" and "probable UIP," when characterizing a patient in the other categories, clinicians should wary about prolonging SLB in patients to determine the fibrosis pattern. Changes in the treatment and overall prognosis of patients can occur due to SLB confirming a UIP pattern on histology. Here, we report the case of a patient with an indeterminate UIP pattern on high-resolution computed tomography (HRCT) with histopathologic diagnosis of UIP on SLB. With no underlying identifiable cause for the UIP pattern, the patient was diagnosed and managed as IPF, ultimately requiring lung transplantation. This case highlights the importance of pursuing surgical lung biopsy in patients with indeterminate UIP on HRCT scanning to facilitate prompt treatment and guide further management.

Follow-up computed tomography scan in post-COVID-19 pneumonia.

The coronavirus disease 2019 (COVID-19) global pandemic can be a severe illness that leads to morbidity and mortality. With the increasing number of COVID-19 pneumonia survivors, several long-term changes may persist, including abnormal imaging of lung parenchyma. In addition to the clinical course, it is vital to follow up on pulmonary imaging during the post-infectious period, which is not routinely required in other common pulmonary diagnoses. Computed tomography (CT) scan of the chest is an effective and diagnostic tool for pneumonia which gives an insight into structural abnormalities within the lungs, complications, and possible progression of the disease. Several studies have monitored COVID-19 pneumonia and its complications using serial CT chest imaging from the initial phase of infection, hospitalization, and post-discharge. Nonetheless, long-term follow-up imaging data in post-COVID-19 is still limited. We have summarized the findings utilizing a systematic review of the literature regarding COVID-19 pneumonia imaging, including long-term follow-up.

Coronavirus Disease 2019-Induced Tracheomegaly: A Case Report.

Tracheomegaly is a medical condition where the tracheal diameter is greater than the upper limits of normal. Tracheomegaly can be classified as primary or secondary. Primary tracheomegaly is usually congenital. Secondary tracheomegaly can be due to multiple causes, including connective tissue disease, infections, autoimmune diseases like sarcoidosis, and prolonged mechanical ventilation. Here, we describe the first reported case of tracheomegaly secondary to coronavirus disease 2019 (COVID-19) pneumonia and COVID-induced interstitial lung disease (ILD). While many cases of tracheomegaly are asymptomatic, patients can have symptoms like cough, dyspnea, hemoptysis, or even respiratory failure. Tracheomegaly is associated with a higher risk of recurrent lower respiratory tract infections, chronic cough, bronchiectasis, and tracheobronchomalacia. Early recognition of COVID-19-induced tracheomegaly can help initial early management and reduce the incidence of infections.

A Case Report on Mycobacterium abscessus: An Emerging Pathogen.

The incidence of infections by rapidly growing mycobacteria has increased in recent decades. nontuberculous mycobacteria(NTM) represent over 190 species and subspecies and can cause both pulmonary and extrapulmonary symptoms. The Mycobacterium abscessus complex (MABC)is among the most drug-resistant mycobacterial species, and prompt diagnosis and effective eradication are burdensome. We present the clinical course of a 55-year-old female who was diagnosed with M. abscessus and explore her clinical diagnosis and possible treatment options. This case report emphasizes the challenges clinicians face in the prompt diagnosis of M. abscessus and discusses the treatment options in light of the recent guidelines.

Current Utility of Sequential Organ Failure Assessment Score: A Literature Review and Future Directions.

The Sequential Organ Failure Assessment (SOFA) score is commonly used in the Intensive Care Unit (ICU) to evaluate, prognosticate and assess patients. Since its validation, the SOFA score has served in various settings, including medical, trauma, surgical, cardiac, and neurological ICUs. It has been a strong mortality predictor and literature over the years has documented the ability of the SOFA score to accurately distinguish survivors from non-survivors on admission. Over the years, multiple variations have been proposed to the SOFA score, which have led to the evolution of alternate validated scoring models replacing one or more components of the SOFA scoring system. Various SOFA based models have been used to evaluate specific clinical populations, such as patients with cardiac dysfunction, hepatic failure, renal failure, different races and public health illnesses, etc. This study is aimed to conduct a review of modifications in SOFA score in the past several years. We review the literature evaluating various modifications to the SOFA score such as modified SOFA, Modified SOFA, modified Cardiovascular SOFA, Extra-renal SOFA, Chronic Liver Failure SOFA, Mexican SOFA, quick SOFA, Lactic acid quick SOFA (LqSOFA), SOFA in hematological malignancies, SOFA with Richmond Agitation-Sedation scale and Pediatric SOFA. Various organ systems, their relevant scoring and the proposed modifications in each of these systems are presented in detail. There is a need to incorporate the most recent literature into the SOFA scoring system to make it more relevant and accurate in this rapidly evolving critical care environment. For future directions, we plan to put together most if not all updates in SOFA score and probably validate it in a large database a single institution and validate it in multisite data base.

An Unusual and Rare Presentation of Pulmonary Aspergillosis: Endobronchial Aspergilloma.

Aspergillosis is a wide spectrum of the disease process that is caused by the fungus Aspergillus. Endobronchial aspergilloma is a very rare type of aspergillosis which is not yet included in the classification of aspergillosis. Due to its rare nature and a limited number of cases, there are no current treatment guidelines. Here we present the case of a 57-year-old female with an endobronchial aspergilloma. The patient was started on intravenous voriconazole and subsequently discharged on oral voriconazole.

The Role of Extra Corporeal Membrane Oxygenation in Amniotic Fluid Embolism: A Case Report and Literature Review.

Amniotic fluid embolism (AFE) is a rare and life-threatening complication related to pregnancy. Early diagnosis and prompt intervention are important tools for the survival of the patient. Despite early intervention, mortality rate remains high. We present a case of a 19-year-old female who was admitted for labor induction and delivery. Her delivery course was complicated by meconium-stained amniotic fluid. The patient went into acute hypoxic respiratory failure (AHRF) and hemodynamic compromise within half an hour following delivery secondary to AFE. We hereby discuss the role of timely initiation of extra corporeal membrane oxygenation (ECMO) in a case of AFE which could have otherwise turned out to be fatal.

When a Dead Patient Is Not Really Dead: Lazarus Phenomenon.

Lazarus phenomenon refers to autoresuscitation of a patient declared dead after cessation of cardiopulmonary resuscitation (CPR). The Lazarus phenomenon is rarely encountered and pathophysiology is not very well understood, but physicians need to be aware of this phenomenon. It is prudent that a physician leading a CPR effort waits for some time and monitors the patient further using blood pressure and electrocardiogram before confirming that a patient is actually dead.

Primary Pulmonary Involvement in Mucosa-associated Lymphoid Tissue Lymphoma.

Pulmonary nodules have a broad differential diagnosis with primary lung cancer, lung metastases, benign tumors, carcinoid tumors, and infectious granulomas as their common cause. While relatively rare, pulmonary lymphoproliferative disorders such as primary pulmonary lymphomas, primary pulmonary plasmacytomas, secondary lymphomas involving the lung, multiple myeloma involving the lung, leukemias involving the lung should be considered in these patients presenting with lung nodules. Primary pulmonary non-Hodgkin's lymphoma (NHL) is an extremely rare lung tumor accounting for 0.4% of all lymphomas. Mucosa-associated lymphoid tissue (MALT) lymphoma accounts for about 70%-90% of all primary pulmonary lymphomas, constituting less than 0.5% of all the lung neoplasms. Though it usually remains localized, it is a clonal B-cell neoplasm with a potential for systematic spread and transformation to an aggressive B-cell lymphoma. We hereby discuss the case of a 66-year-old woman with primary pulmonary MALT lymphoma.

Pulmonary Infection Caused by Mycobacterium terrae: A Case Report and Literature Review.

Mycobacterium terrae infection can cause progressive debilitating disease. A case of a 63-year-old man with localized pulmonary infection characterized by extensive, thick-walled cavitary lesions is presented. A pneumonectomy was considered as definitive treatment, but the patient would not have tolerated the procedure given his severe deconditioning. Instead, he was placed on lifelong antibiotic treatment, but he continued to deteriorate and passed away. The slow-growing microorganism, Mycobacterium terrae, was isolated from bronchoalveolar lavage cultures seven weeks after specimen collection, five and a half weeks after the patient's death. Clinical, microbiological and therapeutic data from this case and 16 other pulmonary cases from the literature are reviewed. Increased awareness of this microorganism will allow clinicians to consider Mycobacterium terrae in their differential diagnosis when dealing with nontuberculous mycobacteria infections.

Guidelines and controversies in the management of diabetic ketoacidosis - A mini-review.

Diabetic ketoacidosis (DKA) is a complication seen in patients with both type 1 and type 2 diabetes. Due to its large, growing economic impact with associated morbidity, closer look at proper management is important. Factors involved in appropriate management involves fluid resuscitation, insulin regimen, and electrolyte replacement including types of fluid and insulin treatment. The caveat with generalized protocol is application to special populations such as renal or heart failure patients the sequelae of complications due to pathophysiology of the disease processes. This leads to complications and longer length of stay in the hospital, therefore, possibly increased cost and resource utilization during the hospitalization. This review takes a closer look at current guidelines of DKA management and resource utilization, the drawbacks of current management protocols and the cost associated with it. Therefore, a need for amendment to existing protocol or initiation of a newer guideline that properly manages DKA should incorporate special populations and appropriate regimen of fluid resuscitation, insulin therapy and electrolyte management.

Sarcoidosis and Its Splenic Wonder: A Rare Case of Isolated Splenic Sarcoidosis.

Sarcoidosis is a systemic granulomatous disease of unknown etiology characterized by presence of noncaseating granulomas in the involved organs. The pulmonary interstitium is most commonly affected but extrapulmonary involvement can occur in almost any other organ system. Such an involvement can occur with or without the presence of pulmonary involvement, but isolated extrapulmonary involvement has been noted only in around 10% of cases. Isolated splenomegaly is very rare and an uncommon presentation of sarcoidosis. It is clinically challenging because of the extensive differential diagnosis. Among the many considerations are hematologic malignancies, primary splenic or metastatic tumors, infiltrative disorders, inflammatory disorders, and infections. We hereby discuss an interesting case of a 40-year-old female with isolated splenic sarcoidosis.

Capnocytophaga Induced Acute Necrotizing and Exudative Pericarditis with Abscess Formation.

We present the case of a 55-year-old gentleman, with bilateral pulmonary embolism and a large pericardial effusion that lead to a pericardial window with evacuation of creamy pus. Gram stains were negative, with culture growing Capnocytophaga. Pathology revealed acute necrotizing and exudative changes, including frank abscess formation. In developed countries, pericardial abscess and acute pericarditis are uncommon due to availability of broad-spectrum antibiotics. Pericardial abscess due to Capnocytophaga is even more uncommon.

Acute Fibrinous and Organizing Pneumonia: A Case Report and Review of the Literature.

BACKGROUND Acute fibrinous organizing pneumonia (AFOP) is a rare condition of the lung that is associated with acute lung injury, and has a poor prognosis. AFOP is characterized histologically by intra-alveolar fibrin. AFOP has been described to be associated with lung infections, connective tissue disorders, drugs, toxic environmental exposure, and in lung transplantation. However, most cases of AFOP remain idiopathic, and because the condition can present with a wide variety of clinical manifestations, open lung biopsy or video-assisted thoracoscopic (VAT) lung biopsy is necessary for the diagnosis. Currently, treatments for AFOP remain under investigation. CASE REPORT A 35-year-old woman presented with a cough and dyspnea, and was initially diagnosed to have pneumonia.  Due to the progression of her symptoms and increasing respiratory failure she underwent video-assisted thoracoscopic (VAT) biopsy and was diagnosed with AFOP, 19 days following hospital admission. She was treated with mechanical ventilation, intravenous steroids, and cyclophosphamide. She required tracheostomy after 14 days of mechanical ventilation and died two weeks later. CONCLUSIONS AFOP is an uncommon clinical condition, with a poor prognosis, which often has a delay in diagnosis. Some patients benefit from steroids and immunosuppressive therapy. Currently, new treatments for AFOP are under investigation.

Patent foramen ovale in idiopathic pulmonary arterial hypertension: Long-term risk and morbidity.

INTRODUCTION: Little is known about the presence of patent foramen ovale in idiopathic pulmonary arterial hypertension. While there is suspected worsening of hypoxemia confounding assessment and management of pulmonary hypertension, as well as possible increased morbidity from paradoxical emboli, there may be theoretical relief of worsening right-sided pressures by the same mechanism of right-to-left shunting. METHODS: Retrospective review of consecutive patients diagnosed with idiopathic pulmonary arterial hypertension (WHO Group 1) via right heart catheterization, from 1998 to 2010. All patients also underwent a four chamber transthoracic echocardiogram with agitated saline contrast for the evaluation of patent foramen ovale. Primary clinical data was collected and compared between patients with and without patent foramen ovale along with univariable and multivariable predictors of long term survival. RESULTS: One hundred and fifty five patients were included in the study, 42 with patent foramen ovale (27%). Patients with patent foramen ovale were younger at pulmonary arterial hypertension diagnosis and trended towards higher right ventricular systolic pressures on echocardiography and mean pulmonary arterial pressures by right heart catheterization. Predictors of mortality included age, diffusing capacity for carbon monoxide, and severe hypoxemia. Only diffusing capacity and age were predictive of mortality after adjustment for a priori covariables. CONCLUSION: Patent foramen ovale is seen in a quarter of patients with idiopathic pulmonary arterial hypertension and associated with increased prevalence of severe hypoxemia but had no effect on long term survival.

A retrospective comparison of helicopter transport versus ground transport in patients with severe sepsis and septic shock.

BACKGROUND: Helicopter emergency medical services (HEMS) extend the reach of a tertiary care center significantly. However, its role in septic patients is unclear. Our study was performed to clarify the role of HEMS in severe sepsis and septic shock. METHODS: This is a single-center retrospective cohort study. This study was performed at Mayo Clinic, Rochester, MN, in years 2007-2009. This study included a total of 181 consecutive adult patients admitted to the medical intensive care unit meeting criteria for severe sepsis or septic shock within 24 h of admission and transported from an acute care facility by a helicopter or ground ambulance. The primary predictive variable was the mode of transport. Multiple demographic, clinical, and treatment variables were collected and analyzed with univariate analysis followed by multivariate analysis. RESULTS: The patients transported by HEMS had a significantly faster median transport time (1.3 versus 1.7 h, p < 0.01), faster time to meeting criteria for severe sepsis or septic shock (1.2 versus 2.9 h, p < 0.01), a higher SOFA score (9 versus 7, p < 0.01), higher incidence of acute respiratory distress syndrome (38 versus 18 %, p = 0.013), higher need for invasive mechanical ventilation (60 versus 41 % p = 0.014), higher ICU mortality (13.3 versus 4.1 %, p = 0.024), and an increased hospital mortality (17 versus 30 %, p = 0.04) when compared to those transported by ground. Distance traveled was not an independent predictor of hospital mortality on multivariate analysis. CONCLUSIONS: HEMS transport is associated with faster transport time, carries sicker patients, and is associated with higher hospital mortality compared with ground ambulance services for patients with severe sepsis or septic shock.

A case of recurrent massive pulmonary embolism in Klippel-Trenaunay-Weber syndrome treated with thrombolytics.

Klippel - Trenaunay - Weber syndrome (KTWS) is a congenital condition characterized by a triad of capillary malformations of the skin, soft tissue and bone hypertrophy resulting in limb enlargement, and abnormalities of arteriovenous and lymphatic systems of the affected limb. In this case, we present a patient with KTWS receiving chronic anticoagulation that had a massive pulmonary embolism and was successfully treated with thrombolytic therapy. The purpose of this case is to educate readers about this uncommon condition and to increase awareness, recognition and timely treatment of its most common complications, namely thrombosis and pulmonary embolism.

Pretransplant frailty is associated with decreased survival after lung transplantation.

BACKGROUND: Frailty is a condition of increased vulnerability to adverse health outcomes. Although frailty is an important prognostic factor for many conditions, the effect of frailty on mortality in lung transplantation is unknown. Our objective was to assess the association of frailty with survival after lung transplantation. METHODS: We performed a retrospective cohort analysis of all adult lung transplant recipients at our institution between 2002 and 2013. Frailty was assessed using the frailty deficit index, a validated instrument that assesses cumulative deficits for up to 32 impairments and measures the proportion of deficits present (with frailty defined as >0.25). We examined the association between frailty and survival, adjusting for age, sex, and bilateral (vs single) lung transplant using Cox proportional hazard regression models. RESULTS: Among 144 lung transplant patients, 102 (71%) completed self-reported questionnaires necessary to assess the frailty deficit index within 1 year before lung transplantation. Frail patients (n = 46) had an increased risk of death, with an adjusted hazard ratio (HR) of 2.24 (95% confidence interval [CI], 1.22-4.19; p = 0.0089). Frailty was not associated with an increased duration of mechanical ventilation (median, 2 vs 2 days; p = 0.26), intensive care unit length of stay (median, 7.5 vs 6 days; p = 0.36) or hospital length of stay after transplantation (median, 14 vs 10.5 days; p = 0.26). CONCLUSIONS: Pre-transplant frailty was independently associated with decreased survival after lung transplantation. Pre-transplant frailty may represent an important area for intervention to improve candidate selection and lung transplant outcomes.

Diffuse cholangiocarcinoma presenting with hepatic failure and extensive portal and mesenteric vein thrombosis.

A 64-year-old previously healthy man presented with a 4-week history of vague right upper quadrant abdominal pain. Imaging studies revealed extensive portal, splenic, superior and inferior mesenteric vein thrombosis with mosaic perfusion and wedge-shaped areas of liver perfusion abnormalities. An extensive thrombophilia workup including tests for factor V Leiden, prothrombin G20210A, lupus anticoagulant, paroxysmal nocturnal haemoglobinuria, protein C and S, homocysteine and antinuclear antibody titres were all negative. Other laboratory testing revealed an elevated alkaline phosphatase (340 IU/L). Surgical exploration and catheter-directed thrombolysis were not felt to be feasible given the extensive clot burden. He was started on anticoagulation therapy. Over the next 10 days, he required intensive care unit admission due to progressive hepatic encephalopathy and fulminant liver failure. He continued to decline and eventually died of multiorgan failure. Autopsy revealed extensive, diffuse intrahepatic cholangiocarcinoma that had almost entirely replaced his normal liver parenchyma.