No abstract

Although xylose is a major product of hydrolysis of lignocellulosic materials, few yeasts are able to convert it to ethanol. In Pachysolen tannophilus, one of the few xylose-fermenting yeasts found, aldose reductase and xylitol dehydrogenase were found to be key enzymes in the metabolic pathway for xylose fermentation. This paper presents a method for the rapid and simultaneous purification of both aldose reductase and xylitol dehydrogenase from P. tannophilus. Preliminary studies indicate that this method may be easily adapted to purify similar enzymes from other xylose-fermenting yeasts.

Background Sinonasal and skull base malignancies can cause significant adverse effects on functional status and survival. Objective The goal of this study was to systematically review the published literature of patient-reported outcomes pertaining to treatment of sinonasal and skull base malignancy. Methods A systematic literature search of Medline was conducted with PubMed to identify studies that assessed patient-reported outcomes in patients with sinonasal or skull base malignancy. Patient-reported outcomes studies with at least 10 patients published in English from January 2000 to April 2017 were included. Criteria from International Society for Quality of Life guidelines and criteria unique to sinonasal and skull base malignancies were used to calculate a composite score for each article. Studies with the top 33% of scores were categorized as high quality articles. Results Twenty-two articles met inclusion/exclusion criteria. Three studies (14%) reported a priori hypothesis. Eleven (50%) assessed specific quality of life domains and 10 studies (45%) performed statistical analysis on these domains. Specific symptoms were assessed in up to 32% of studies. Eight studies were characterized as high quality; these studies had higher sample sizes and more often assessed patient-reported outcomes prior to treatment compared to low quality studies. Conclusions The goal of the current study was to evaluate the quality of the current patient-reported outcomes literature on sinonasal and skull base malignancies. Areas of improvement for future studies include analysis of individual domains and disease-specific symptoms, reporting a priori hypotheses, and collecting preoperative and longitudinal patient-reported outcomes data.

Williams-Campbell syndrome (WCS) is a rare cystic disorder of the lung due to defective cartilage synthesis in sub-segmental bronchi, typically from fourth-sixth order; the central airway is unaffected. WCS is associated with persistent cough, recurrent chest infection and chronic bronchiectasis. The syndrome has been described more frequently in children, with the first case series reported in 1960. Adult presentation is rare and has been previously described in only a handful of case reports. The late presentation may be due to less severe disease. Recurrent pneumonia is a common presentation in adults, however, prominent broncho-obstructive symptoms such as coughing and wheezing may confuse it with asthma. CASE PRESENTATION:We present the case of a 62-year-old man, lifelong non-smoker with history of childhood asthma, GERD, hypertension, and BPH who presented with a 5 year history of productive cough, sinusitis, occasional hemoptysis, and recurrent chest infections. He received multiple courses of antibiotics for pneumonia and eventually underwent thoracotomy for empyema. He presented to the pulmonology clinic for evaluation of persistent cough and dyspnea. Bronchoscopic evaluation showed dilated airways and polymicrobial infection. PFT was normal with moderately reduced DLCO. CT chest with contrast was suggestive of bilateral cystic bronchiectasis arising after 3rd order bronchial branches with mild emphysematous changes. The patient had previously undergone cystic fibrosis genetic testing, which was negative. Further testing for alpha-1 antitrypsin, quantitative immunoglobulins, ABPA serologies and anti-CCP antibody were all within normal limits. A diagnosis of cystic bronchiectasis secondary to WCS was made on the basis of these characteristic radiological features and the exclusion of other possible causes. DISCUSSION:The diagnosis of WCS requires thorough history-taking, distinctive radiologic findings, and exclusion of other differentials (1). Given the findings of recurrent infections, differentials such as cystic fibrosis, ciliary dyskinesia, and immunoglobulin deficiency should be considered. The chronic broncho-obstructive symptoms with or without barrel chest and clubbing should lead to consideration of alpha-1 antitrypsin deficiency, autoimmune disease, and allergic bronchopulmonary aspergillosis. Thin slice CT films will demonstrate proximal cystic bronchiectasis with characteristic ballooning on inspiration and collapse on expiration (2). 3D rendering of images may also reflect lack of cartilage ring (3). The congenital deficiency of cartilage is responsible for the pathogenesis of this disease, and our patient likely had less severe pathology leading to delayed diagnosis. CONCLUSIONS:Although WCS is most commonly diagnosed in the pediatric population, WCS is important to consider in any patient with chronic bronchiectasis.