Citations

AbstractBackgroundAlthough the prognosis of core‐binding factor (CBF) acute myeloid leukemia (AML) is better than other subtypes of AML, 30% of patients still relapse and may require allogeneic hematopoietic cell transplantation (alloHCT). However, there is no validated widely accepted scoring system to predict patient subsets with higher risk of relapse.MethodsEleven centers in the US and Europe evaluated 247 patients with t(8;21)(q22;q22).ResultsComplete remission (CR) rate was high (92.7%), yet relapse occurred in 27.1% of patients. A total of 24.7% of patients received alloHCT. The median disease‐free (DFS) and overall (OS) survival were 20.8 and 31.2 months, respectively. Age, KIT D816V mutated (11.3%) or nontested (36.4%) compared with KIT D816V wild type (52.5%), high white blood cell counts (WBC), and pseudodiploidy compared with hyper‐ or hypodiploidy were included in a scoring system (named I‐CBFit). DFS rate at 2 years was 76% for patients with a low‐risk I‐CBFit score compared with 36% for those with a high‐risk I‐CBFit score (P < 0.0001). Low‐ vs high‐risk OS at 2 years was 89% vs 51% (P < 0.0001).ConclusionsI‐CBFit composed of readily available risk factors can be useful to tailor the therapy of patients, especially for whom alloHCT is not need in CR1 (ie, patients with a low‐risk I‐CBFit score).

OBJECTIVES: We assessed the adherence to neuroimaging guidelines and the diagnostically relevant yield of neuroimaging in newly presenting early life epilepsy (ELE). METHODS: There were 775 children with a new diagnosis of epilepsy (&lt;3 years old at onset) who were recruited through the ELE study at 17 US pediatric epilepsy centers (2012–2015) and managed prospectively for 1 year. The data were analyzed to assess the proportion of children who underwent neuroimaging, the type of neuroimaging, and abnormalities. RESULTS: Of 725 children (93.5%) with neuroimaging, 714 had an MRI (87% with seizure protocols) and 11 had computed tomography or ultrasound only. Etiologically relevant abnormalities were present in 290 individuals (40%) and included: an acquired injury in 97 (13.4%), malformations of cortical development in 56 (7.7%), and other diffuse disorders of brain development in 51 (7.0%). Neuroimaging was abnormal in 160 of 262 (61%) children with abnormal development at diagnosis versus 113 of 463 (24%) children with typical development. Neuroimaging abnormalities were most common in association with focal seizure semiology (40%), spasms (47%), or unclear semiology (42%). In children without spasms or focal semiology with typical development, 29 of 185 (16%) had imaging abnormalities. Pathogenic genetic variants were identified in 53 of 121 (44%) children with abnormal neuroimaging in whom genetic testing was performed. CONCLUSIONS: Structural abnormalities occur commonly in ELE, and adherence to neuroimaging guidelines is high at US pediatric epilepsy centers. These data support the universal adoption of imaging guidelines because the yield is substantially high, even in the lowest risk group.

Objectives: Ultrasound guided fascia-Iliaca blocks have been used for pain control following hip arthroscopy. There is little evidence regarding their effectiveness in comparison to other pain control modalities in hip arthroscopy patients. To compare efficacy of ultrasound guided fascia iliac block with intra-articular ropivacaine in controlling pain after hip arthroscopy. Methods: Between 2015 and 2017, 95 patients undergoing hip arthroscopy were randomly assigned to 2 groups. The first group received an ultrasound-guided fascia-iliaca block with 50-60mLs of 0.35% ropivacaine. The second group received intra-articular injection of 20 mL of 0.5% ropivacaine at the completion of the surgical case. Primary outcomes were post-operative pain scores in the recovery room, at PACU discharge, at 2 weeks, 6 weeks, and 3 months. Secondary outcomes included intraoperative and PACU narcotic usage (converted to morphine equivalent use) as well as readmission rates, PACU recovery time and postoperative nausea and vomiting (PONV). Results: Postoperative pain across all points did not significantly differ between the 2 groups. Intraoperative and PACU narcotics did not differ significantly between the 2 groups. Readmission rates, PACU recovery time and PONV did not significantly differ between the 2 groups. There were no associated complications in either group. Conclusion: Ultrasound guided fascia iliaca block for hip arthroscopy had no clinical advantage when compared to one time intra-articular ropivacaine injection. Furthermore, an intraarticular injection decreases costs and is a safe and more efficient modality for postoperative pain control following hip arthroscopy.

Abstract Background In the past decade, opioid use in the general population of the US has increased dramatically. Concomitantly, opioid-related morbidity and mortality have also risen steeply. The causes of opioid over-prescribing are multifactorial, with pressure from medical agencies, patients, aggressive patient-directed marketing, and use of patient surveys as an evaluation tool to unduly influence medical providers' decision-making. Providers also have significant concerns regarding scrutiny from their medical board and exposure to medicolegal liability. Content To provide the basis for best practice recommendations, several aspects of modern opioid prescribing practice require review. The practicing clinician should be aware of: (a) the pervasiveness of current opioid prescribing within the US; (b) the availability and applicability of clinical tools used to assess patient risk; (c) the new guidelines designed to facilitate appropriate and safe medical use of opioids; (d) the incorporation of clinical tools and best documentation practices to reduce medicolegal exposure. Summary On completion of this review, the reader should be able to recognize the magnitude of the problem of opioid over-prescribing in the US and identify several patient risk factors for opioid misuse, abuse, and/or diversion. Specifically, better familiarity with the recently published CDC guidelines for opioid prescribing will help ensure the adoption of best-use practices that maximize therapeutic efficacy while attenuating the possibility of harm to patients. A thorough review of the legal principles and risk-mitigation strategies relevant to opioid prescribing will also lessen the clinician's exposure to medicolegal liability.

ObjectivePrevious studies suggest that the brain‐derived neurotrophic factor (BDNF) Val66Met (rs6265) polymorphism may influence symptom onset in Alzheimer's disease (AD). Our recent cross‐sectional findings suggest that Met66 may influence clinical expression in dominantly inherited AD (DIAD) through its effects on tau. However, it remains unclear whether carriage of Met66 in DIAD results in faster increases in cerebrospinal fluid (CSF) tau and ptau181, and whether these increases are associated with accelerated brain volume loss and memory decline.MethodsA total of 211 subjects (101 mutation noncarriers, 110 mutation carriers), who were cognitively normal, as defined by a Clinical Dementia Rating global score of 0, completed assessments of cognitive function, neuroimaging, and CSF sampling over 3.5 years as part of the Dominantly Inherited Alzheimer's Network.ResultsIn mutation carriers, Met66 carriers showed faster memory decline (4×), hippocampal volume loss (16×), and CSF tau and ptau181increases (6×) than Val66 homozygotes.BDNFdid not influence rates of cortical β‐amyloid accumulation or change in CSF Aβ42levels in mutation carriers. In mutation noncarriers,BDNFgenotype had no effect on change in cognition, brain volume, cortical β‐amyloid accumulation, or change in any CSF measures of tau, ptau181, and CSF Aβ42.InterpretationAs in sporadic AD, the deleterious effects of β‐amyloid on cognitive function, brain volume loss, and CSF tau in DIAD mutation carriers are less in Val66 homozygotes. TheBDNFVal66Met polymorphism should be considered as a potential moderator of clinical trial outcomes in current treatment and prevention trials in DIAD and sporadic AD. Ann Neurol 2018;84:424–435

Systemic capillary leak syndrome (SCLS) is an idiopathic vascular hyperpermeability that leads to profound hypotension, hypoalbuminemia, and hemoconcentration. Sixty years since its inauguration into literature, SCLS is still elusive, underdiagnosed, and profoundly dangerous. Few authors have documented anesthetic implications of classical acute SCLS, a form with complete recovery between exacerbations. However, minimal information is available about chronic SCLS, continuous generalized edema with or without exacerbations. Both forms are postulated to have similar pathogenesis, yet clinical differences are noteworthy, warranting variations in perioperative management. We present a patient with chronic SCLS who underwent general and regional anesthesia for elective surgery.

Purpose: Novel approaches are needed to enhance employee well-being and perhaps supervisors can be an effective agent for worksite health promotion. The aim of this study was to examine the supervisor’s perceived needs, barriers, and role for influencing employee well-being for incorporation into program development. Design: Semistructured, qualitative interviews of supervisors. Setting: Large, integrated academic health-care organization with over 30 000 employees and 2600 supervisors having access to comprehensive well-being programs and a successful well-being champion network comprised of 600 champions. Participants: Twenty supervisors representing clinical, research, and administrative units. Methods: Semistructured, one-on-one interviews were conducted and audio recorded. Analysis included content log development and open coding by a trained analyst to reveal key themes. More formalized content coding using specialized software for qualitative analyses was also conducted. Results: Supervisor responses were wide ranging regarding their perceived and desired role in promoting workplace well-being. Barriers from the supervisor perspective included high current workload, ambivalence about promoting wellness, lack of support from leadership, lack of flexibility and control at work, and difficulty accessing on-site resources. They perceived their potential role in well-being as remaining a positive role model and encouraging their staff in wellness activities. Conclusion: Although findings are generated from a small sample size, these qualitative data provide compelling and early insights into building a workplace well-being strategy leveraging an underutilized key stakeholder, the workplace supervisor.

Purpose: The purpose of the study is to describe short-term efficacy and safety of goniotomy with trabecular meshwork excision using the Kahook Dual Blade (KDB, New World Medical Inc., Rancho Cucamonga, CA) in patients with severe or refractory glaucoma. Method: Retrospective multicentric case series of 53 eyes with severe or refractory glaucoma as defined by ICD-10 conducted in the United States, Mexico, and Switzerland. Primary efficacy outcome was a ≥20% decrease in intraocular pressure (IOP) from baseline at 6 months. Secondary efficacy outcome measures were probability of achieving an IOP≤14 or 18 mm Hg at 6 months and the mean IOP change from baseline at 6 months. Medication use required to obtain target IOP at last follow up and adverse effects were analyzed. Results: The proportion of eyes achieving an IOP reduction of >20% from preoperative baseline at 6 months was 57.7% (n=30). The mean IOP decreased from 18.4±6.1 mm Hg at baseline to 13.9±3.5 mm Hg at month 6 (23.9% reduction; P<0.001). At 6 months, 63.5% and 92.3% achieved an IOP≤14 and ≤18 mm Hg, respectively, and the mean number of glaucoma medications was reduced by 1.2±1.3 (36.6%) compared with baseline (P<0.001). The most common adverse event was hyphema (n=29, 34.9%) with spontaneous resolution in all cases. No severe complications were reported. One case presented with uncontrolled IOP and required glaucoma drainage device surgery at 1 month. Conclusions: Goniotomy with trabecular meshwork excision using the KDB could be an alternative surgery for severe or refractory glaucoma, significantly reducing IOP and medication use at 6 months, with a low rate of complications.