BACKGROUND： We hypothesized that the total face mask (TFM) would be perceived as more comfortable than a standard oronasal mask (ONM) by patients receiving noninvasive mechanical ventilation (NIV) therapy for acute respiratory failure (ARF) and would be quicker to apply by respiratory therapists. METHODS： Sixty patients presenting with ARF were randomized to receive NIV via either an ONM or a TFM. Mask comfort and dyspnea were assessed using visual analog scores. Other outcomes included time required to apply， vital signs and gas exchange at set time points， and early NIV discontinuation rates (ie， stoppage while still requiring ventilatory assistance). RESULTS： Mask comfort and dyspnea scores were similar for both groups through 3 h of use. The time required to apply the mask (5 min [interquartile range (IQR)， 2-8] vs 3.5 min [IQR， 1.9-5])， and duration of use (15.7 h [IQR， 4.0-49.8]) vs 6.05 h [IQR， 0.9-56.7]) were not significantly different between the ONM and the TFM group， respectively. Except for heart rate， which was higher at baseline in the TFM group， no differences in vital signs or gas exchange were detected between the groups during the first 3 h (P > .05). Early NIV discontinuation rates were similar for both the ONM group and TFM group (40% vs 57.1%); however， eight patients in the TFM group were switched to an ONM within 3 h， and none from the ONM group was switched to a TFM (P < .05). CONCLUSIONS： Among patients with ARF requiring NIV， the ONM and TFM were perceived to be equally comfortable and had similar application times. Early NIV discontinuation rates， improvements in vital signs and gas exchange， and intubation and mortality rates were also similar. TRIAL REGISTRY： ClinicalTrials.gov; No.： NCT00686257; URL： www.clinicaltrials.gov.
O365 A STUDY OF RISK AND PROGNOSTIC FACTORS IN MATERNAL MORTALITY AND OBSTETRIC NEAR-MISS (ONM) CASES: A DEVELOPING WORLD EXPERIENCE
o365 a study of risk and prognostic factors in maternal mortality and obstetric near-miss (onm) cases： a developing world experience
After conducting a search of the medical literature using the terms “ONM” and “child” we identify only 5 previously published reports of ONM in children. To the best of our knowledge this patient is the youngest patient reported with a diagnosis of ONM.
Strabismus Surgery in Patients With Ocular Neuromyotonia: Potential Unmasking of the Condition and Effective Management Tool.
Abstract BACKGROUND： Ocular neuromyotonia (ONM) is a rare motility disorder in which paroxysms of tonic extraocular muscle contraction from abnormal ocular motor nerve firing result in episodic diplopia and strabismus. Medical therapy with membrane-stabilizing agents has varied success. A surgical approach to treatment has not yet been described. We report the outcomes of strabismus surgery in patients with ONM. METHODS： We describe 3 patients with sixth nerve paresis and ONM of the affected lateral rectus muscle who underwent strabismus surgery. All patients had a history of radiation therapy for intracranial tumors. Ophthalmologic and orthoptic examinations were performed with appropriate medical and neuroradiologic evaluation. Preoperative and postoperative data are presented and analyzed. RESULTS： Two patients were noted to have ONM after their first strabismus surgery for a sixth nerve palsy. Patients 1 and 2 had 3 surgeries， whereas Patient 3 had 1 operation. Extraocular muscles operated on included the medial rectus and lateral rectus. Preoperative primary gaze baseline esotropia ranged from 35 to 75 prism diopters (Δ). All patients achieved improvement in ocular alignment and motility. Postoperative primary gaze deviations ranged from orthotropia to 20Δ of esotropia. Abduction deficits were unchanged or improved. The follow-up period ranged from 15 months to 2 years. CONCLUSIONS： Patients with ONM of a paretic rectus muscle can achieve binocular fusion with strabismus surgery. ONM may manifest postoperatively in patients with a sixth nerve palsy and a contractured medial rectus who， preoperatively， were not noted to have ONM.
Early disintegration of myonuclear fibrous lamina in inclusion body myositis: Ultrastructural and immunohistochemical studies
Abstract Objective Morphological analyses to assess the integrity of myonuclear surface structures in muscle biopsies from patients with inclusion body myositis (IBM) and related disorders were carried out to find clues to the causes of IBM. Methods Muscle biopsies from 26 patients (seven with IBM， five polymyositis， three dermatomyositis， one hereditary inclusion body myopathy associated with Paget disease and front-temporal dementia， three with other non-inflammatory myopathies， four with no pathological abnormalities and three type 2 fiber atrophy only) were assessed by measuring lengths of clearly identifiable muscle outer (ONM) and inner nuclear membranes (INM)， and fibrous laminae (FL) on electron micrographs， and grading severity of degenerative changes in the sarcoplasm into three stages. The nuclei of capillary endothelial cells were also examined and lamin A/C， a major constituent of FL， localized immunohistochemically. Results Compared with patients with no pathology or type 2 fiber atrophy only， polymyositis had significantly less ONM， INM and FL at an advanced stage of degeneration. In dermatomyositis， ONM was moderately decreased. In IBM， ONM， INM and FL were decreased severely from early stages of muscle fiber degeneration. In the one case with inclusion body myopathy associated with Paget disease and front-temporal dementia， all three layers， particularly ONM， were decreased. No significant changes were observed in the nuclei of capillary endothelial cells in IBM or other conditions. In IBM， there was less lamin A/C in myonuclei than in capillary endothelial cell nuclei. Conclusions In IBM， shorter lengths of myonuclear surface structures， particularly of FL， were observed from an early stage of degeneration. Early disintegration of FL might reflect an essential pathomechanism of IBM.
Abducens ocular neuromyotonia as a delayed complication of oropharyngeal carcinoma treated with radiation
Abstract Background Ocular neuromyotonia (ONM) is a disorder characterized by periodic involuntary extraocular muscle contraction that occurs almost exclusively in the setting of prior radiation to the sella or skull base. We present the first case of abducens neuromyotonia associated with oropharyngeal carcinoma. Methods and Results We report a case of a 63-year-old patient with abducens ONM occurring 16 years after radiation treatment for oropharyngeal squamous cell carcinoma. A literature review was performed using Medline and PubMed databases to search for all documented cases of abducens neuromyotonia. Our review found 20 cases of abducens neuromyotonia but none after radiotherapy (RT) to the oropharynx. Conclusion Abducens ONM can occur because of disease at anatomic locations remote from the course of the sixth cranial nerve， most likely because of the irradiated area exceeding the intended field. Our case also supports the fact that RT can significantly precede symptom onset. © 2016 Wiley Periodicals， Inc. Head Neck ， 2016
The linker of and (LINC) complex is a structure that spans the entire (NE) and integrates the nuclear interior with the . /C together with nesprins that mainly reside along the (INM) and outer (ONM) are core components of the LINC complex. Integrity of this specific nuclear structure is critical for muscle and function. In the present study， we analyzed the ultrastructure of the LINC complex observed in two neonates with severe hypotonia and respiratory distress. Disruption of the LINC complex manifests in a wide separation of the ONM from the INM; the loss of (PNS) and delayed muscle were predominating findings. This nuclear phenomenon has never been reported and provides further support for the appearance of a neonatal form of laminopathy.
Nasal obstruction and male gender contribute to the persistence of mouth opening during sleep in CPAP-treated obstructive sleep apnoea
Abstract Top of page Abstract Introduction Methods Results Discussion Acknowledgements References Background and objective During continuous positive airway pressure (CPAP) treatment， some patients with obstructive sleep apnoea syndrome (OSAS) require an oronasal mask (ONM) to prevent excessive mouth leakage. Factors contributing to sleep-related mouth opening under CPAP treatment remain known. We compared mouth opening during sleep in patients treated with CPAP by nasal mask (NM) versus ONM. Methods Cross-sectional prospective study： patients treated with CPAP for at least 4 months underwent a sleep recording using a type 4 monitoring device (Brizzy-Nomics) that records mouth opening via a magnetometric distance meter. Clinical assessment included anthropometry， smoking status and the Mallampati score. Nasal obstruction was assessed by the Nasal Obstruction Symptom Evaluation questionnaire. Results Thirty-eight patients were included， 34 analysed (22 men; age = 57.4 (53; 62) years; body mass index = 32.6 (29.1; 35.2) kg/m 2 ; median (25th; 75th)). Twenty-seven patients were treated with NM and seven with ONM. Patients with ONM were more often active smokers and trended to have greater nasal obstruction and lower forced expiratory volume in 1 s. They also exhibited a greater mouth opening during sleep (median (25th;75th) = 13.0 (11.0; 15.0) vs 6.0 (5.0; 10.0) mm， P < 0.001) and a higher oxygen desaturation index (9.5 (6.2; 15.5) vs 2.9 (1.0; 6.1) events/h， P = 0.009). In multivariate analysis， male gender and nasal obstruction were independently associated with mouth opening under ONM CPAP treatment. Conclusions After several months of CPAP treatment， some patients using ONM persist in keeping their mouths open at night. Nasal obstruction and male gender contribute to this phenomenon.
Medication-related osteonecrosis of the jaw associated with bisphosphonates and denosumab in osteoporosis
Abstract Objective To describe the clinical characteristics and evolution of our series of medication-related osteonecrosis of the jaws (MRONJ) associated with denosumab in osteoporotic patients. Material and Methods We present ten new cases of MRONJ in patients receiving denosumab for osteoporosis. We describe the mean doses of denosumab， previous bisphosphonate intake， and the clinical characteristics associated with the osteonecrosis， such as local contributing factors， symptoms， and evolution after treatment. Results The mean number of denosumab doses was 3.4 ± 2.2. In 90% of patients， there was a prior history of oral bisphosphonate intake， with a mean duration of 46.78 ± 25.11 months. The most common local factor was dental extraction (6 cases; 60%)， and most cases had necrotic bone exposure (9/10， 90%). Sclerosis of the bone was the most common radiographic finding. Stage 1 was the most common ONM stage， found in 80%. ‘Cure’ after conservative treatments was obtained in 71.4%. Conclusions Most of our cases were in the early stages of MRONJ， and the success rate after conservative treatment was high. This article is protected by copyright. All rights reserved.
Trajectories of recovery and dysfunction after acute illness, with implications for clinical trial design.
Oronasal mask (ONM) can be used when mouth leaks impair nasal-CPAP effectiveness. However， ONM's constraint on the chin and straps' traction may alter upper airway (UA) mechanical properties. In contrast， mandibular advancement device associated with nasal-CPAP (NM + MAD) may reduce UA resistance. The aim of this exploratory study was to compare the effects of ONM， NM， and NM + MAD on UA mechanical properties. The three interface modalities were assessed in 11 OSAS patients at 6， 8， 10 cmH(2)O CPAP using a phrenic nerve magnetic stimulation (PNMS) protocol. PNMS-twitches' related flow， pharyngeal pressures (nasopharynx， velopharynx， oropharynx) and UA resistances were determined. Regardless of CPAP level， twitch-induced maximum flow was higher with NM + MAD than with ONM. Velopharyngeal resistance was higher with ONM than with NM + MAD. Oropharyngeal resistance was higher with ONM than with NM. In conclusion， NM + MAD reduced velopharyngeal resistance compared to those measured with ONM and NM alone. We hypothesize that this strategy may help reducing the effective pressure level and thus further limit the risk for mouth leaks. (c) 2012 Elsevier B.V. All rights reserved.