Management of repository corticotrophin injection therapy for pulmonary sarcoidosis: a Delphi study.

In patients treated with repository corticotrophin injection (RCI) for pulmonary sarcoidosis, effective management of adverse events may improve adherence. However, management of adverse events may be challenging due to limitations in real-world clinical experience with RCI and available published guidelines.We surveyed 12 physicians with a modified Delphi process using three questionnaires. Questionnaire 1 consisted of open-ended questions. Panellists' answers were developed into a series of statements for Questionnaires 2 and 3. In these, physicians rated their agreement with the statements using a Likert scale.Key consensus recommendations included a starting dose of 40 units twice a week for patients with less severe disease, continued at a maintenance dose for patients who responded, particularly those with chronic refractory sarcoidosis. Panellists reached consensus that concomitant steroids should be quickly tapered in patients receiving RCI, but that concomitant use of immunosuppressive medications should be continued. Panellists developed consensus recommendations for adverse event management, and reached consensus that RCI should be down-titrated or discontinued if other interventions for the adverse effects fail or if the adverse effect is severe.In the absence of clinical evidence, our Delphi consensus opinions may provide practical guidance to physicians on the management of RCI to treat pulmonary sarcoidosis.

Improving Lung Function in Severe Heterogenous Emphysema with the Spiration Valve System (EMPROVE). A Multicenter, Open-Label Randomized Controlled Clinical Trial.

Rationale: Less invasive, nonsurgical approaches are needed to treat severe emphysema.Objectives: To evaluate the effectiveness and safety of the Spiration Valve System (SVS) versus optimal medical management.Methods: In this multicenter, open-label, randomized, controlled trial, subjects aged 40 years or older with severe, heterogeneous emphysema were randomized 2:1 to SVS with medical management (treatment) or medical management alone (control).Measurements and Main Results: The primary efficacy outcome was the difference in mean FEV1 from baseline to 6 months. Secondary effectiveness outcomes included: difference in FEV1 responder rates, target lobe volume reduction, hyperinflation, health status, dyspnea, and exercise capacity. The primary safety outcome was the incidence of composite thoracic serious adverse events. All analyses were conducted by determining the 95% Bayesian credible intervals (BCIs) for the difference between treatment and control arms. Between October 2013 and May 2017, 172 participants (53.5% male; mean age, 67.4 yr) were randomized to treatment (n = 113) or control (n = 59). Mean FEV1 showed statistically significant improvements between the treatment and control groups-between-group difference at 6 and 12 months, respectively, of 0.101 L (95% BCI, 0.060-0.141) and 0.099 L (95% BCI, 0.048-0.151). At 6 months, the treatment group had statistically significant improvements in all secondary endpoints except 6-minute-walk distance. Composite thoracic serious adverse event incidence through 6 months was greater in the treatment group (31.0% vs. 11.9%), primarily due to a 12.4% incidence of serious pneumothorax.Conclusions: In patients with severe heterogeneous emphysema, the SVS shows significant improvement in multiple efficacy outcomes, with an acceptable safety profile.Clinical trial registered with www.clinicaltrials.gov (NCT01812447).

Argon plasma coagulation in the management of uncovered tracheal stent fracture.

Endotracheal and endobronchial stenting, particularly with uncovered stents, can be complicated by stent fracture, granulation tissue formation, direct airway injury, and airway obstruction. While stent removal is possible, it can result in significant complications and long-term benefit is not guaranteed. Argon plasma coagulation can be employed to trim fractured stent fragments and remove granulation tissue simultaneously. In this manuscript, we report a case and describe our experience with using this technique.

Lymphangioleiomyomytosis: Are the Findings of Video-assisted Thoracoscopy Pathognomonic?

A 22-year-old African American female patient presented with a sudden onset of difficulty in breathing for approximately 2 days accompanied by bilateral pleuritic chest pain. Her past medical history was significant for tuberous sclerosis and mental retardation. Preliminary radiographic imaging showed bilateral pneumothoraces for which bilateral chest tubes were subsequently inserted. A computed tomography scan of the chest showed cystic changes compatible with lymphangioleiomyomytosis (LAM). She underwent a video-assisted thoracoscopy with pleurodesis and an open lung biopsy for the confirmation of the diagnosis. Cystic changes involving the visceral pleura were noticed during the procedure. Understanding that "tuberous sclerosis complex" is an illness that could be associated with LAM should prompt clinicians to consider the diagnosis if the patients present with shortness of breath, pneumothorax, diffuse cystic lung changes, or hemoptysis. Hemoptysis results from pulmonary venous hypertension, which is a consequence of LAM. Pleuroscopy or video-assisted thoracoscopy may show unusual findings, as described in this case, which could be pathognomonic for the diagnosis.

Serosanguineous Pleural Effusions in a Patient With HIV and Kaposi Sarcoma: Pleuroscopic Findings.

A 22-year-old white homosexual male presented with severe shortness of breath. He was diagnosed as having human immunodeficiency virus/acquired immunodeficiency syndrome and skin manifestations of Kaposi sarcoma (KS). He had a significant pericardial effusion, which was drained. He had bilateral pleural effusions, the left effusion larger than the right. His effusion was associated with primary effusion lymphoma. He was subjected to a pleuroscopy and the cause of the blood-stained effusion was thought to be due to chronic pleuritis and unrelated to the pleural KS. The patient also had the characteristic lesions of KS on the parietal pleural, which is unusual, as most involve the visceral surface.

The use of narrow band imaging in patients with benign disease: hereditary hemorrhagic telangiectasia.

SUMMARY: : Narrow band imaging (NBI) is a new type of imaging technology that enhances the visibility of blood vessels used in the detection of abnormal angiogenesis in preneoplastic and neoplastic lesions. This technique is used in the diagnosis and management of dysplastic and malignant endobronchial lesions. To our knowledge, this is the first description of the use of NBI to diagnose and manage benign lesions, hereditary hemorrhagic telangiectasia (HHT). The objective of this study was to implement NBI as a tool for use in the bronchoscopic visualization leading to the diagnosis and management of nonmalignant lesions in the tracheobronchial tree. This is including but not limited to HHT. NBI was used to detect the origin of significant hemoptysis in a patient with HHT. The patient had conventional white light (CWL) bronchoscopy, followed by NBI. NBI illuminated the abnormal blood vessels significantly better than CWL bronchoscopy. This led to a more effective diagnosis and management of the abnormal vessels causing the hemoptysis. NBI may provide a higher probability of locating abnormal endobronchial lesions in both benign and malignant diseases than CWL bronchoscopy. It can be used to treat these abnormal lesions as in our patient who presented with hemoptysis. A prospective study is needed to determine whether NMI and CWL are additive as in malignant disease. Furthermore, NBI can be used in lesions of the tracheobronchial tree and the gastrointestinal tract, and pleura, so that the appropriate management can be initiated.

Insertion of intracranial pressure monitors in fulminant hepatic failure patients: early experience using recombinant factor VII.

BACKGROUND: Cerebral edema contributes to the high morbidity and mortality of fulminant hepatic failure (FHF). OBJECTIVE: We report the results of our early experience with insertion of intraparenchymal intracranial pressure (ICP) monitors in these highly coagulopathic patients. METHODS: Eleven consecutive patients with FHF met the criteria for invasive ICP monitoring. Recombinant activated factor VII (rFVIIa) was administered at an average dose of 3 mg intravenous bolus (average, 36.7 microg/kg). We inserted the intraparenchymal ICP monitor within 15 minutes to 2 hours after rFVIIa administration, without waiting for the repeat coagulation results. Postprocedure computed tomographic scans of the brain were obtained in all patients. RESULTS: No hemorrhagic complications were detected on the immediate postprocedure computed tomographic scans. There were no thrombotic complications in this group of patients. CONCLUSION: In this group of patients with FHF, placement of an ICP monitor without hemorrhagic or thrombotic complications was feasible after administration of rFVIIa. This is a report of our early experience, and caution is advised. Further collaborative randomized studies are needed to prove the efficacy, optimal dosing, and cost effectiveness of rFVIIa for the placement of ICP monitors in this group of patients.

Inhaled corticosteroids should be initiated before discharge from the emergency department in patients with persistent asthma.

National and International Guidelines concur that inhaled corticosteroids (ICS) are the preferred long-term maintenance drug therapy for mild persistent asthma for all ages. For moderate and severe persistent asthma, ICS are essential to optimal management, often concurrent with other key therapies. Despite strong evidence and consensus guidelines, ICS are still underused. While some patients who are treated in the emergency department (ED) have intermittent asthma, most have persistent asthma and need ICS for optimum outcomes. Failure to initiate ICS at this critical juncture often results in subsequent lack of ICS therapy. Along with a short course of oral corticosteroids, ICS should be initiated before discharge from the ED in patients with persistent asthma. Although the NIH/NAEPP Expert Panel Report 3 suggests considering the prescription of ICS on discharge from the ED, The Global Initiative for Asthma (GINA) 2008 guidelines recommend initiation or continuation of ICS before patients are discharged from the ED. The initiation of ICS therapy by ED physicians is also encouraged in the emergency medicine literature over the past decade. Misdiagnosis of intermittent asthma is common; therefore, ICS therapy should be considered for ED patients with this diagnosis with reassessment in follow-up office visits. To help ensure adherence to ICS therapy, patient education regarding both airway inflammation (show airway models/colored pictures) and the strong evidence of efficacy is vital. Teaching ICS inhaler technique, environmental control, and giving a written action plan are essential. Lack of initiation of ICS with appropriate patient education before discharge from the ED in patients with persistent asthma is common but unfortunately associated with continued poor patient outcomes.

Mobilizing the patient in the intensive care unit: the role of early tracheotomy.

A large number of studies have evaluated the benefits of early tracheotomy. Heterogeneity in the various studies reviewed in this article is apparent, with early tracheotomy ranging from one to several days, and benefits regarding incidence of pneumonia and mortality are variable. An additional factor likely contributing to the differing results relates to the varied patient populations in the individual studies, which ranged from burn patients to medical ICU patients to trauma patients and head trauma patients. A close look at the studies with the least confounding variables suggests that early tracheotomy has some merit. Most studies suggest that time in the ICU, on mechanical ventilation, and in the hospital is reduced.

Pneumonia in patients who require prolonged mechanical ventilation.

Nosocomial pneumonia is the most important infectious disease in patients who require prolonged mechanical ventilation. Understanding of the etiology helps to prevent ventilator-associated pneumonia (VAP). VAP can develop in four ways: by aspiration, inhalation, hematogenous spread and by contiguous spread. The two most common are aspiration from the oropharyngeal region and inhalation, usually from manipulation of tubing or infected equipment. VAP is prevented by hand-washing, keeping the head of the bed at 45 and, in some cases, by treating the surface bacteria which usually cause VAP. Sputum can be used for the diagnosis of VAP in most of these patients instead of invasive bronchoscopy. However, if the patients are critically ill, then bronchoscopy is used. Treatment in these patients depends on the bacteria. Pseudomonas is treated by two drugs (beta-lactam plus a quinolone or aminoglycoside), Acinetobacteria by ampicillin/sulbactam or carbapenam, extended-spectrum beta-lactam-producing bacteria by carbapenums, and Staphylococcus by vancomycin or linezolid.