Combined-modality therapy for pulmonary alveolar proteinosis in a remote setting: a case report.

BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of phospholipoproteinaceous material in the alveoli. The presentation is nonspecific but typically includes dyspnea; the spectrum of disease includes rapidly progressive hypoxic respiratory failure. Whole lung lavage (WLL) is the treatment of choice in symptomatic PAP, but transient worsening of oxygenation sometimes requires salvage modalities of support such as extracorporeal membrane oxygenation (ECMO). Granulocyte macrophage colony-stimulating factor (GM-CSF) plays a role in the pathophysiology of PAP. We highlight a case of severe PAP treated with exogenous GM-CSF and sequential lobar lavage due to the unavailability of salvage methods of oxygenation. CASE PRESENTATION: A 36 year old female was admitted with fevers, chills, and progressive dyspnea. On presentation she was tachypneic, tachycardic, and hypoxemic; labs revealed leukocytosis and lactic acidosis. Chest CT identified diffuse ground glass opacities in a 'crazy-paving' pattern. Following intubation due to impending respiratory failure, bronchoscopy with bronchoalveolar lavage was performed. The lavage return stained positive with Periodic Acid Schiff, confirming the diagnosis of PAP. Continued deterioration necessitated treatment; however, at this geographically remote center without ECMO services WLL was judged to carry significant risk. Nebulized GM-CSF was administered without significant improvement. Subcutaneous GM-CSF was administered and isolated subsegmental lavages of the bilateral upper lobes were performed, with rapid improvement in oxygenation. Additional sequential lobar lavage and continued GM-CSF therapy as an outpatient resulted in complete resolution of oxygen requirement and return to normal pulmonary physiology. CONCLUSIONS: The autoimmune form of PAP is the most common, indicating that therapy with GM-CSF may play an important role for many patients. Treatment with WLL may be impractical in some clinical settings due to the expertise and salvage modalities required. Sequential lobar lavage requires less specialized expertise and may incur less risk of refractory hypoxemia. We posit that this combined-modality therapy is ideally suited to geographically-remote centers such as our own.

Adult-Onset Still's Disease: Is This Truly a Diagnosis of Exclusion?

Adult-onset Still's Disease is a rare, idiopathic, inflammatory disorder characterized by arthralgia, evanescent, salmon-colored rash, and daily fevers as well as lymphadenopathy, pharyngitis, splenomegaly, myalgias, and serositis. The inciting etiology of this syndrome is unknown, though it has been hypothesized that infection triggers an autoimmune response. The Yamaguchi Criteria, the most sensitive and widely used diagnostic criteria, requires both a minimum set of criteria to be met as well other potential etiologies to be excluded. By definition, evidence of concomitant infection, malignancy, vasculitis, or connective tissue disease precludes the diagnosis of Adult-onset Still's Disease from being made. We present a very rare case of a patient who met all diagnostic criteria for Adult-onset Still's Disease, had a protracted course refractory to numerous immunosuppressant treatments, and also had evidence of coxsackie B infection with fourfold rise in viral titers on two occasions (both associated with disease flare). Although coxsackie B virus has been linked to Adult-onset Still's Disease at disease presentation, this case is unique in its protracted course and serological evidence of infection temporally related to disease flare. While accepted diagnostic criteria call for this disease to be a diagnosis of exclusion, our case supports the fact that ongoing infection may in fact be an important antigenic driver in persistent and refractory Adult-onset Still's Disease.

Benign Granuloma Masquerading as Squamous Cell Carcinoma Due to a "Floater".

Pathology specimen cross-contamination is a rare phenomenon in diagnostic pathology. Such "floaters" may result in delayed, missed or erroneous diagnoses. We describe the case of a patient with benign granuloma of the lung initially misdiagnosed as squamous cell carcinoma due to a "floater."

Barriers and facilitators associated with colonoscopy completion in individuals with multiple chronic conditions: a qualitative study.

BACKGROUND: A recommendation to undergo a colonoscopy, an invasive procedure that requires commitment and motivation, planning (scheduling and finding a driver) and preparation (diet restriction and laxative consumption), may be uniquely challenging for individuals with multiple chronic conditions (MCCs). This qualitative study aimed to describe the barriers and facilitators to colonoscopy experienced by such patients. MATERIALS AND METHODS: Semistructured focus groups were conducted with male Veterans who were scheduled for outpatient colonoscopy and either failed to complete the procedure or completed the examination. Focus group recordings were transcribed and analyzed by an inductive grounded approach using constant comparative analysis. RESULTS: Forty-four individuals aged 51-83 years participated in this study (23 adherent and 21 nonadherent). Participants had an average of 7.4 chronic conditions (range 2-14). The five most common chronic conditions were hypertension (75%), hyperlipidemia (75%), osteoarthritis/degenerative joint disease (59%), atherosclerotic heart disease (48%), and diabetes mellitus (36%). We identified four unique themes that influenced motivation to undergo a colonoscopy: competing medical priorities, low perceived benefit, a prior negative colonoscopy experience, and pre-existing medical conditions. Additionally, we identified four themes that influenced individuals' ability to complete the examination: difficulty with bowel cleansing, difficulty with travel, worry about exacerbation of pre-existing conditions, and heightened concerns about potential complications. CONCLUSION: MCCs are common in individuals referred for colonoscopy and generate unique barriers to colonoscopy completion related to medication, dietary changes, transportation, preparation processes, symptoms exacerbation, and complication concerns. Future research should examine whether tailored interventions that include education and support in addressing the unique barriers can enhance colonoscopy completion.

Concurrent Systemic Chemoimmunotherapy and Sofosbuvir-Based Antiviral Treatment in a Hepatitis C Virus-Infected Patient With Diffuse Large B-Cell Lymphoma.

Hepatitis C virus (HCV) infection is associated with the development of non-Hodgkin lymphomas. For aggressive lymphomas, such as diffuse large B-cell lymphoma (DLBCL), treatment of HCV infection is typically deferred in treatment-naive patients until after completion of lymphoma therapy [1, 2]. We report a case of HCV-associated stage IV DLBCL successfully treated concurrently using chemoimmunotherapy and a sofosbuvir-based antiviral regimen.

A systematic review of the quality of evidence of ablative therapy for small renal masses.

PURPOSE: We critically assessed the methodological and reporting quality of published studies of ablative techniques for small renal masses. MATERIALS AND METHODS: We performed a systematic PubMed® and EMBASE® literature search from January 1966 to March 2010 to identify all full text, original research publications on ablative therapy for renal masses. Six reviewers working independently in 3 teams performed duplicate data abstraction using Strengthening the Reporting of Observational Studies in Epidemiology criteria, which were pilot tested in a separate sample. RESULTS: A total of 117 original research publications published in a 15-year period (1995 to 2009) met eligibility criteria. No randomized, controlled trials were identified. All studies were observational and 88.9% had 1 arm with no comparison group. Median sample size was 18 patients (IQR 5.5, 40.0) and 53.8% of studies included 20 or fewer patients. Median followup was 14.0 months (IQR 8.0, 23.8) and only 19.7% of studies had an average followup of greater than 24 months. Of the studies 20.5% mentioned the number of operators involved and only 6.0% provided information on their experience level. Of the studies 66.7% addressed the recurrence rate. Disease specific and overall survival was reported in only 15.4% and 16.2% of studies, respectively. CONCLUSIONS: The published literature on the therapeutic efficacy of ablative therapy for renal masses is largely limited to uncontrolled, 1-arm observational studies. In the absence of higher quality evidence ablative therapy outside research studies should be limited to select patients who are not candidates for surgical intervention.