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The risk of that outcome was greater in patients who had filled COX-2-inhibitor prescriptions (adjusted odds ratio = 1.84 [95% confidence interval = 1.38 to 2.46]) or opioid prescriptions (1.69 [1.53 to 1.86]), but not in patients who had filled nonselective-NSAID prescriptions (1.07 [0.93 to 1.23]) after the fracture. Results were similar when the outcome definition was changed to just a nonunion diagnosis. Conclusions COX-2 inhibitors, but not nonselective NSAIDs, were associated with a greater risk of nonunion after fracture. Opioids were also associated with nonunion risk, although patients filling prescriptions for opioids may have had more severe fractures. Level of evidence Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.Objectives "Failure to rescue" (FTR) is the failure to prevent a death resulting from a complication of medical care or from a complication of underlying illness or surgery. There is a growing body of evidence that identifies causes and interventions that may improve institutional FTR rates. Why do patients "fail to rescue" after complications in hospital? What clinically relevant interventions have been shown to improve organizational fail to rescue rates? Can successful rescue methods be classified into a simple strategy? Methods A systematic review was performed and the following electronic databases searched between January 1, 2006, to February 12, 2018 MEDLINE, PsycINFO, Cochrane Library, CINAHL, and BNI databases. All studies that explored an intervention to improve failure to rescue in the adult population were considered. Results The search returned 1486 articles. Eight hundred forty-two abstracts were reviewed leaving 52 articles for full assessment. Articles were classified into 3 strategic arms (recognize, relay, and react) incorporating 6 areas of intervention with specific recommendations. Conclusions Complications occur consistently within healthcare organizations. They represent a huge burden on patients, clinicians, and healthcare systems. Organizations vary in their ability to manage such events. Failure to rescue is a measure of institutional competence in this context. We propose "The 3 Rs of Failure to Rescue" of recognize, relay, and react and hope that this serves as a valuable framework for understanding the phases where failure of patient salvage may occur. Future efforts at mitigating the differences in outcome from complication management between units may benefit from incorporating this proposed framework into institutional quality improvement.Bcl-2-associated athanogene 3 (BAG3) myopathy is a rare myofibrillar myopathy characterized by toe walking and clumsiness in the first decade with rapid progression to cardiomyopathy and restrictive lung disease in the second decade. Most patients (18 patients) have the c.626C >T (p.Pro209Leu) mutation. We describe BAG3 myopathy due to p.Pro209Leu in a 13-year-old girl with initial prominent neuropathic phenotype and no cardiac or respiratory involvement. Parents reported toe walking and clumsiness since 3 years old. Examination at the age of 13 years showed findings suggestive of Charcot-Marie-Tooth disease. Nerve conduction studies revealed demyelinating polyneuropathy. Next-generation sequencing panel for inherited neuropathies was unrevealing. Whole exome sequencing identified a de novo mutation in BAG3. Muscle biopsy confirmed myofibrillar myopathy. No cardiac involvement or symptoms of respiratory involvement at the age of 14 years. This case emphasizes the phenotypic variability of BAG3 myopathy and the importance of thorough electrophysiological examination and muscle pathology for establishing a precise diagnosis.Bickerstaff brainstem encephalitis, widely considered to be associated with Miller Fisher and Guillain-Barré syndromes, is a rare disease state defined by the triad of ophthalmoplegia, ataxia, and decreased consciousness. The presence of central nervous system involvement, commonly in the form of impaired arousal, solidifies it as a unique entity. We present a case of this rare syndrome after autologous stem cell transplant.Congenital myasthenic syndromes are clinically and genetically heterogeneous disorders characterized by a neuromuscular transmission defect. Mutations in novel genes have been described in recent years. Among these, MUSK gene mutations are extremely rare, with only 8 families identified worldwide to date. We report a Spanish case, a carrier of one known hetero-allelic missense mutation and one newly identified MUSK gene variant. Our patient presented with congenital onset ophthalmoplegia and palpebral ptosis associated with limb-girdle weakness and exercise intolerance without prominent fatigability, developed during his twenties. He was misdiagnosed as mitochondrial myopathy because of paraclinic and histologic findings, but detailed clinical examination prompted us to reassess him with repetitive stimulation technique, demonstrating decremental response and suggesting myasthenic syndrome. A genetic study confirmed the clinical diagnosis allowing us to started treatment with excellent clinical response.Objectives Half of patients with amyotrophic lateral sclerosis experience sialorrhea due to facial weakness. Although anticholinergic medications are first-line therapy, they often lead to unacceptable side effects. Radiation therapy and botulinum toxin may be considered when medical management fails. In this systematic review, we investigated the effectiveness of these interventions. Methods Eligible studies were retrieved from PubMed and Scopus databases up to March 2017 along with hand-searching of references from primary articles. Results Fourteen studies (N = 138) examined the benefits of botulinum toxin. Studies varied in salivary glands treated, dosages used, and the use of botulinum toxin subtype A or B. A majority of studies showed benefit after treatment. Although most studies reported only mild adverse effects, 2 case studies revealed severe complications including recurrent TMJ dislocations and rapid deterioration in bulbar function. Ten studies (N = 171) examined the benefits of radiation. Most studies reported improvement with only mild adverse events reported. Conclusions Both radiation and botulinum toxin are effective treatments for sialorrhea in patients with amyotrophic lateral sclerosis and should be considered when medical management fails. Radiation may offer longer duration of symptom improvement with fewer complications.Four retrospective studies from the United States, Europe, and Asia address outcomes in juvenile myasthenia gravis. Common features include earlier onset with ocular myasthenia gravis (MG) and generally good outcomes overall. Patients who were seronegative, had equivocal acetylcholine receptor (AChR) antibodies, or had only clustered AChR antibodies had better outcomes. An article highlighting the utility of median nerve slow-repetitive stimulation is reviewed and another showing the high sensitivity of repetitive nerve stimulation in myasthenic crisis is covered. Two articles address the thymus and thymectomy in non-AChR antibody-positive autoimmune MG. Longer term data on eculizumab as well as studies of cyclophosphamide, rituximab, and tacrolimus are summarized. Other topics include the possible role of statins in MG and central nervous system autoimmune comorbidities.Objectives To define whether electrical nerve stimulation (ENS) therapy would promote intraepidermal nerve growth and nerve regeneration in patients with small fiber neuropathy (SFN). Methods This was a prospective study conducted on 8 subjects with previously diagnosed SFN. Nerve conduction testing, punch biopsies, and clinical examinations with a calculation of revised total neuropathy score were conducted on subjects before beginning ENS therapy and at 30 and 60 days after the start of ENS therapy. Results Clinical examination findings and intraepidermal nerve fiber density measurements on day 30 and day 60 did not show statistically significant changes in the treated group compared with the untreated group. Conclusions Despite the success of previous animal studies, no meaningful nerve growth and regeneration in SFN was demonstrated with ENS therapy in this study. Studies of larger subject larger populations with longer duration of ENS treatment are warranted to confirm our findings.Purpose The purpose of this work was to report a new method for the repair of ischemic bleb leaks. Bleb leakage is a serious complication of glaucoma filtering surgery with mitomycin C. Many surgical methods have been proposed to seal the leakage from a bleb; however, this novel plication method is effective, relatively easy to perform, and safe. Methods We describe the case reports of 2 patients with leakage from a bleb who were treated with the new method. The conjunctiva was lifted away from the sclera as extensively as possible around the ischemic conjunctiva toward the fornix using a bleb knife. Thereafter, 10-0 nylon sutures were applied between the nonischemic conjunctiva located just outside the ischemic conjunctiva and the corneal limbus. The ischemic conjunctiva was not removed, but covered with the nonischemic conjunctiva that was advanced toward the corneal limbus by these sutures. Results After treatment, no recurrence of bleb leakage was observed. learn more Moreover, no ischemic changes were observed in the advanced nonischemic conjunctiva or plicated conjunctiva. Conclusion This new method of bleb plication was effective for sealing bleb leakage.Background Transcranial motor evoked potential (TcMEP) monitoring is conventionally performed during surgical procedures without or with minimal neuromuscular blockade (NMB) because of its potential interference with signal interpretation. However, full blockade offers increased anesthetic management options and facilitates surgery. Here, the feasibility of TcMEP interpretation was assessed during full NMB in adult neurosurgical patients. Methods Patients undergoing cervical or lumbar decompression received a rocuronium bolus producing 95% or greater blockade by qualitative train-of-four at the ulnar nerve. TcMEPs were recorded in bilateral thenar-hypothenar and abductor hallucis muscles. Adequacy of response for reliable signal interpretation was determined on the basis of repeatability and clarity, assessed by coefficient of variation and signal-to-noise ratio, respectively. Results All patients had at least 3 of 4 measurable TcMEP limb responses present during full NMB, and 70.8% of patients had measurable responses in all 4 limbs. In total, 82.2% of thenar-hypothenar responses and 62.8% of abductor hallucis responses were robust enough for reliable signal interpretation on the basis of clarity. In addition, 97.8% of thenar-hypothenar responses and 79.1% of abductor hallucis responses met the criteria for reliable signal interpretation on the basis of consistency. Patient demographics, medical comorbidities, and preoperative weakness were not predictive of absent responses during full NMB. Conclusions TcMEP interpretation may be feasible under greater levels of NMB than previously considered, allowing for monitoring with greater degrees of muscle relaxation. Consideration for monitoring TcMEP during full NMB should be made on a case-by-case basis, and baseline responses without blockade may predict which patients will have adequate responses for interpretation.