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Postoperatively, the patient's factor X levels were controlled daily and corrected using SD-FFP as needed, with no clinically significant blood loss. CONCLUSIONS SD-FFP can be used to manage congenital factor X deficiency in the peripartum period and maintain perioperative blood loss within normal limits.BACKGROUND Birt-Hogg-Dubé Syndrome (BHDS) characterised by skin fibrofolliculomas, kidney tumour and pulmonary cysts/pneumothorax is caused by folliculin (FLCN) germline mutations. The pathology of both neoplasia and focused tissue loss of BHDS strongly features tissue-specific behaviour of the gene. Isolated cysts/pneumothorax is the most frequent atypical presentation of BHDS and often misdiagnosed as primary spontaneous pneumothorax (PSP). Deferential diagnosis of BHDS with isolated pulmonary presentation (PSP-BHD) from PSP is essential in lifelong surveillance for developing renal cell carcinoma. METHODS The expression profiles of microRNAs (miRNAs) in cystic lesions of PSP-BHD and PSP were determined via microarray. The selected upregulated miRNAs were further confirmed in the plasma of an expanded cohort of PSP-BHD patients by reverse transcription quantitative PCR (RT-qPCR). Their diagnostic accuracy was evaluated. Moreover, the cellular functions and targeted signalling pathways of FLCN-regulated miRNAs were assessed in various cell lines and in the lesion tissue contexts. RESULTS Cystic lesions of PSP-BHD and PSP showed different miRNAs profiles with a significant upregulation of miR-424-5p and let-7d-5p in PSP-BHD. The combination of the two effectively predicted BHDS patients. In vitro studies revealed a suppressive effect of FLCN on miR-424-5p and let-7d-5p expressions specifically in lung epithelial cells. The ectopic miRNAs triggered epithelial apoptosis and epithelial transition of mesenchymal cells and suppressed the reparative responses in cells and tissues with FLCN deficiency. CONCLUSION The upregulation of miR-424-5p and let-7d-5p by FLCN deficiency occurred in epithelial cells and marked the PSP-BHD condition, which contributed to a focused degenerative pathology in the lung of PSP-BHD patients. © Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.BACKGROUND Double-triggering is a well-recognized form of patient-ventilator asynchrony in noninvasive ventilation (NIV). This benchtop simulated lung study aimed to determine under which patient and device-specific conditions double-triggering is more prevalent, and how this influences the delivery of NIV. METHODS Two commonly used proprietary NIV devices were tested using a benchtop lung model. Lung compliance, airway resistance, respiratory effort, and breathing frequency were manipulated, and the frequency of double-triggering was assessed. A lung model of very low lung compliance (15 mL/cm H2O) was then used to assess the frequency of double-triggering when breathing frequency and respiratory effort were varied, along with basic NIV settings, including inspiratory pressure and expiratory pressure. [https://www.selleckchem.com/products/clozapine-n-oxide.html Clozapine N-oxide] Minute ventilation and total inspiratory work (as calculated by the simulated lung model) were also correlated with frequency of double-triggering. RESULTS In both devices, double-triggering was observed with reduced lung compliance (P = .02 and P less then .001 for the two devices, respectively). Reduced airway resistance was associated with double-triggering with the one device only (P = .02). Respiratory effort and breathing frequency were not independent predictors of double-triggering across all lung models. In the lung model of very low lung compliance, both devices showed increased double-triggering at a lower breathing frequency (P less then .001 and P less then .001), higher respiratory effort (P = .03 and P less then .001), and greater pressure support (P = .044, P less then .001). Importantly, double-triggering was associated with reduced minute ventilation (P = .007) with one device and increased inspiratory work (P less then .001) with the other device. CONCLUSIONS Both simulated-patient and device characteristics influenced the frequency of double-triggering in NIV, resulting in meaningful consequences in a simulated lung model. Copyright © 2020 by Daedalus Enterprises.BACKGROUND To increase the understanding of the self-extubation phenomena, we assessed its rate in our medical ICU and aimed to identify the risk factors of self-extubation and the risk factors for re-intubation. METHODS We prospectively identified subjects who self-extubated. Their baseline characteristics, including the Richmond Agitation Severity Scale score, reason for intubation, shift, distance of the endotracheal tube tip to the carina, and outcomes were collected retrospectively. For every subject who self-extubated, a control subject was selected from the mechanical ventilation database. RESULTS During the study period, there were 2,578 admissions with 4,072 mechanical ventilation days. Fifty-three cases of self-extubation were recorded, which resulted in a self-extubation event rate of 1.3 per 100 days of mechanical ventilation. Forty-five controls were identified. The most common reason for intubation was hypoxic respiratory failure, followed by the need for airway protection and hypercapnic respird of mechanical ventilation in patients who are agitated and with a longer endotracheal tube to carina distance on chest radiograph. Copyright © 2020 by Daedalus Enterprises.BACKGROUND The objective of the current study was to determine whether overnight pulse oximetry in patients with amyotrophic lateral sclerosis is prognostic of the onset of awake respiratory failure and hospital admissions. METHODS This was an observational study in a cohort of subjects with amyotrophic lateral sclerosis. The study included subjects with a baseline S pO2 ≥ 94% on home overnight pulse oximetry testing. Patients age ≥ 80 y and those with comorbidities and with poor short-term prognosis or sleep apnea were excluded. The subjects were classified as nocturnal desaturators according to percentage of sleep time with S pO2 10% was observed in the overnight pulse oximetry 21 months versus 32 months survival if T90 was ≤ 10%. CONCLUSIONS In subjects with amyotrophic lateral sclerosis, nocturnal desaturation conferred a higher risk of respiratory failure and poorer prognosis. Even in the absence of other clinical criteria, early pulse oximetry should be performed and the need for nocturnal ventilatory support assessed.