Guidelines for management of hereditary angioedema: What is new? What is missing?

Background: Hereditary angioedema is an autosomal dominant disease that presents with recurrent episodic swelling of the submucosal and/or subcutaneous tissues of the cutaneous, gastrointestinal, and respiratory systems. Evaluation and treatment guidelines have been published nationally and internationally to aid the treating provider. Methods: A review of the most cited and most recent updated guidelines was undertaken to review key points and to explore real-world feasibility of incorporating them into clinical practice. The International World Allergy Organization/European Academy of Allergy and Clinical Immunology (WAO/EAACI) Guideline for the Management of Angioedema - The 2017 Revision and Update, and the consensus reports from the Hereditary Angioedema International Working Group, the Joint Task Force on Practice Parameters focused practice parameter update, and the most recently updated US HAEA Medical Advisory Board 2020 Guidelines for the Management of Hereditary Angioedema were reviewed and summarized. Results: Key points that have been consistent throughout the guidelines include recommendations for evaluation and classification of hereditary angioedema as well as evidence-based guidelines for treatment. Further attention is required on the evaluation and continuous assessment of the burden of illness and quality of life (QoL). Conclusion: The guidelines for management of hereditary angioedema provide a framework for the clinician. However, the physician-patient dialog with regard to the patient disease experience, which includes attack frequency, severity, and Qol, must be continually assessed.

Clinical Utility of Intravascular Ultrasound (IVUS) in Carotid Artery Interventions: A Systematic Review and Meta-analysis

Background: Carotid plaque morphology plays an important role in determining outcome of carotid artery stenting (CAS). Intravascular ultrasound (IVUS) and its extension VH (Virtual Histology)-IVUS evaluate plaque characteristics in real time and guide decision making during stenting. To date, there is no consensus about indications of IVUS and its validated methods. This systematic review and meta-analysis aims to evaluate the clinical utility of IVUS in carotid artery interventions (CAS) and develop a future consensus for research and practice parameters. Methods: A systematic review and meta-analysis was performed of the English literature articles published till February 2021. Studies reporting on IVUS parameters and findings and also its performance compared with other imaging modalities were included in review. Pooled prevalence with 95% confidence intervals (CI) was calculated. The statistical analysis was conducted in R version 3.6.2. Results: A total of 2015 patients from 29 studies were included. Proportional meta-analysis was performed on 1566 patients from 11 studies. In 9 studies, stroke/transient ischemic attack (TIA) had a pooled prevalence of 4% (95% CI 3%–5%) while asymptomatic stroke had a pooled prevalence of 46% (95% CI 31%–62%) in 4 studies following IVUS. Two studies reported that IVUS detected more plaque protrusion compared with angiography (n=33/396 vs 11/396). IVUS led to stent type or size change in 8 of 48 cases which were missed on angiography in 3 other studies. Concordance between VH-IVUS and true histology was good at 80% to 85% reported in 2 studies. Conclusions: This systematic review and meta-analysis showed, though IVUS fared better to computed tomography (CT)/magnetic resonance (MR) angiography for better stent selection during CAS, with low to moderate risk of bias in the studies included. However, large scale, preferably randomized controlled studies are needed to predict its role in determining clinical outcome.

Society of Vascular and Interventional Neurology Standards and Parameters for Guideline Development and Publication

BACKGROUND As much of the scope of neurointerventional practice falls outside data covered by existing randomized clinical trials, and, as a result, may have failed to enter into existing guidelines, an evidence‐based framework for guideline and standards development is needed. We establish an evidence‐based framework to guide all subsequent guidelines and brief practice updates produced by the Society of Vascular and Interventional Neurology (SVIN). METHODS The SVIN formed the Guidelines and Practice Parameters committee to develop the structure and procedures for guidelines and brief practice updates. RESULTS In this article, the Guidelines and Practice Parameters committee has outlined the process by which the guidelines will be created and approved by the SVIN. Additionally, the Guidelines and Practice Parameters committee has adopted the American College of Cardiology/American Heart Association framework of Class of Recommendation and Level of Evidence. A unique, additional separation of the Expert Opinion endorsement category has been developed when high‐quality evidence does not exist at the time of the publication. CONCLUSIONS The SVIN has developed an evidence‐based framework for all guideline statements and brief practice updates. The SVIN guidelines and brief practice updates will guide clinicians in the field of interventional neurology to improve and standardize patient care.

Clinical Guideline Highlights for the Hospitalist: Management of Upper Gastrointestinal and Ulcer Bleeding

GUIDELINE TITLE: American College of Gastroenterology: Upper Gastrointestinal and Ulcer Bleeding RELEASE DATE: May 1, 2021 PRIOR VERSION(S): March 1, 2012 DEVELOPER: American College of Gastroenterology Practice Parameters Committee FUNDING SOURCE: American College of Gastroenterology TARGET POPULATION: Adult patients with overt upper gastrointestinal bleeding

Antibiotics for Spinal Cord Stimulation Trials and Implants: A Survey Analysis of Practice Patterns

Background: Spinal cord stimulation (SCS) is an established treatment modality for neuropathic pain. Published guidelines exist to aid physicians in proper antibiotic use during and after spinal cord stimulation trials and implants. In this brief review, we present and analyze the current antibiotic practice patterns of clinicians. Methods: The study protocol was reviewed and granted an exemption by an Institutional Review Board. The survey queried practice parameters in regards to spinal cord stimulation therapy. The American Society of Regional Anesthesia and Pain Medicine (ASRA) and Society of Interventional Spine (SIS) distributed the survey to their active members by emails with a web link to the survey. Results: Our results indicate that 82% and 69% of physicians do not utilize nasal swabs for methicillin-sensitive Staphylococcus aureus (MSSA) or methicillin-resistant Staphylococcus aureus (MRSA), respectively, prior to SCS trial and implantation. During trials, 47% providers administer a single dose of antibiotics, 35% administer antibiotics for the duration of the trial, and 17% do not administer antibiotics. During implantation, 44% of physicians administer a single dose during the procedure, 11% administer antibiotics up to 24 hours, 24% administer antibiotics between 3-5 days, 14% administer antibiotics for more than 5 days, and 4% do not administer antibiotics. Conclusions: Our study suggests a portion of pain physicians do not adhere to the Neuromodulation Appropriateness Consensus Committee (NACC) guidelines in regards to antibiotic administration for SCS trial and implantation. Further analysis and surveys would allow insight into common practices. More information and education would be beneficial to optimize peri-procedure antibiotic use to reduce infection risk and decrease antimicrobial resistance.

Application of the Challenge Point Framework During Treatment of Speech Sound Disorders

Purpose The purpose of this article is to provide trial-by-trial practice performance data in relation to learning (outcome probe data) as collected from 18 treatment sessions provided to children with severe speech sound disorders. The data illustrate the practice–learning paradox: Specific, perfect practice performance is not required for speech production learning. Method We detailed how nine student speech-language pathologists (SSLPs) implemented and modified the motor learning practice conditions to reach a proposed challenge point during speech practice. Eleven participants diagnosed with a severe speech sound disorder received high-intensity speech therapy 3 times per week for 6 weeks. SSLPs implemented treatment procedures with the goal of achieving at least 100 practice trials while manipulating practice parameters to maintain practice at the challenge point. Specifically, child performance was monitored for accuracy in five-trial increments, and practice parameters were changed to increase functional task difficulty when the child's performance was high (four or five correct responses) or to decrease functional task difficulty when the child's performance was low (fewer than four correct responses). The practice stimulus, type and amount of feedback, structure of practice, or level of support might be changed to ensure practice at the challenge point. Results On average, the children achieved 102 practice trials per session at a level of 58% correct responses. Fast achievement of connected speech with the lowest amount of support was associated with high scores on generalization probes. Even with high levels of error during practice, the children improved percent consonants correct with maintenance of learning 3 months posttreatment. Conclusion The results of this study show that it may not be necessary to overpractice or maintain a high degree of performance accuracy during treatment sessions to achieve transfer and retention of speech production learning.

The Role of Infection and Autoimmunity in Urticaria and Angioedema as a Common Entity

Chronic spontaneous urticaria with angioedema is prevalent, affecting approximately 1% of the general population, and has a significant impact on quality of life, according to epidemiological data. This article aims to broaden the view on the mechanisms of urticaria and the role of infection in the current environment. It is not easy to identify the cause of urticaria but appropriate steps to treat an underlying infection can, in some cases, improve the symptoms of urticaria and angioedema, reduce severity and duration, or lead to remission. Although chronic spontaneous urticaria with angioedema is a multifactorial condition involving inflammation, autoimmunity, and coagulation, IgE-mediated autoimmunity, or autoallergy, is thought to play a major role. Every year, more is learnt about the role of cells releasing mediators, underlying autoimmune processes that lead to the development of mast cell activation and urticaria. It has become increasingly clear that mast cell roles in immune system responses are not limited to an allergic role; they are key players in protective immune responses, both innate and adaptive, to various pathogens and in defence of some infections. Several guidelines, consensus papers, and practice parameters have been developed for the management of chronic urticaria. The Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the Global Allergy and Asthma European Network (GA2LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) produce a guideline, which is revised every 4 years by a global panel of experts in the field. Infections may be a cause, aggravating factor, or unassociated bystander in chronic urticaria. The author looked at evidence, using a keyword search, for the role of viral and bacterial infections in acute, acute recurrent, and chronic urticaria and angioedema, including COVID-19, herpes, viral hepatitis, and Helicobacter pylori.

Using Data-Mining CRISP-DM Methodology to Predict Drilling Troubles in Real-Time

Drilling is considered one of the most challenging and costly operations in the oil and gas industry. Several initiatives were applied to reduce the cost and increase the effectiveness of drilling operations. One of the frequent difficulties that faces these operations is unexpected drilling troubles that take place and stops the operation, resulting in losing a lot of time and money, and could lead to safety issues culminating in a fatality situation. For that, the industry is in continues efforts to prevent drilling troubles. Part of these efforts is utilizing the artificial intelligence (AI) technologies to identify troubles in advance and prevent them before maturing to a serious situation. Multiple approaches were tried; however, errors and significant deviation were observed when comparing the prediction results to the actual drilling data. This could be due to the improper design of the artificial intelligent technology or inappropriate data processing. Therefore, searching for dynamic and adequate artificial intelligent technology and encapsulated data processing model is very essential. This paper presents an effective data-mining methodology to determine the most efficient artificial intelligent technology and the applicable data processing techniques, to identify the early symptoms of drilling troubles in real-time. This methodology is CRISP-DM that stands for Cross Industry Standard Process for Data Mining. This methodology consists of the following phases: Business Understanding, Data Understanding, Data Preparation, Modeling, Evaluation and Deployment. During these phases, multiple data-quality techniques were applied to improve the reliability of the real-time data. The developed model presented a significant improvement in identifying the drilling troubles in advance, compared to the current practice. Parameters such as hook-load and bit-depth, were studied. Actual data from several oil fields were used to develop and validate this smart model. This model provided the drilling engineers and operation crew with bigger window to mitigate the situation and resolve it, prevent the occurrence of several drilling troubles, result in big time and cost savings. In addition to the time and cost savings, CRISP-DM provided the artificial intelligent experts and the drilling domain experts with a framework to exchange knowledge and sharply increase the synergy between the two domains, which lead to a common and clear understanding, and long-term successful drilling and AI teams collaboration. The novelty of this paper is the introduction of data-mining CRIPS methodology for the first time in the prediction of drilling troubles. It enabled the development of a successful artificial intelligence model that outperformed other drilling troubles prediction practices.

606 Incidence & Influence of Drug Screening in Children with Hypersomnolence During MSLT: Analysis of the Children’s Wisconsin Cohort

Introduction The Multiple Sleep Latency Test (MSLT) is a validated test for evaluation of hypersomnolence in children and adults. AASM practice parameters recommend performing urine drug screens (UDS) in patients undergoing MSLT to identify substances that alter MSLT results. The study aims are to determine the incidence of positive UDS and their influence on MSLT variables in a cohort of children evaluated for hypersomnolence. Methods All children undergoing an MSLT in Children’s Wisconsin sleep laboratory over an 8-year period (11/1/2012 to 9/30/2020) were included in this retrospective chart review study. Clinical and demographic data, including UDS results were manually abstracted. Record validation conducted by random allocation. Data were summarized as median and IQR or n (%). Chi-square test or Fisher’s exact test were used to examine associations between categorical variables. Results 236 children completed an MSLT. The sample had a median age of 14.1 (IQR 10.5–16.2) years (50.9% female; 63.1% Caucasian). Narcolepsy I (“N1”; n=14; 5.9%), Narcolepsy II (“N2”; n=56; 23.7%), Idiopathic Hypersomnia (“IH”; n=39; 16.5%) were frequently diagnosed. Most children (97.9%) completed a UDS; 60.2% tested positive. Common substances found on UDS were caffeine (62.6%), OTC medications (40.3%), and prescription medications (33.8%), however nicotine (14.3%) and cannabis (5.8%) were also seen. Caffeine was commonly found in those diagnosed with N1 (70%), N2 (69.4%), and IH (54.2%). Fewer children diagnosed with narcolepsy were positive for prescription medications compared to those diagnosed with IH (21.7% versus 41.7%) although results did not reach significance (p=0.08). No child with N1 tested positive for prescription medications. ≥2 substances were found in 43.2% of positive drug screens. OTC medications and caffeine were most commonly co-occurring (23%); OTC analgesics being the most common OTC medication. No association between positivity for >2 substances and sleep diagnosis was found (p=0.5). Conclusion More than half of children undergoing an MSLT had a positive UDS for >1 substance. The impact of these substances on PSG/MSLT parameters (total sleep time, mean sleep onset latency, sleep onset REM periods) is under investigation through additional analysis. Based on the above data our findings support the AASM guidelines of children obtaining a UDS on the day of MSLT. Support (if any):

556 Lost to Follow-Up: Post-Operative Polysomnography in High-Risk, Pediatric OSAs

Introduction Post-operative polysomnography (PSG) is recommended in certain pediatric populations at risk for residual sleep disordered breathing: moderate to severe obstructive sleep apnea syndrome (OSAS), obesity, craniofacial abnormalities, and neurologic disorders. In light of multiple stakeholders involved in follow-up management, variability in completion of a post-operative PSG may exist. We hypothesize that patients with isolated severe OSAS or severe plus a co-morbidity will have greater incidence of a post-operative PSG. Methods A chart review of 373 pediatric patients revealed 67 patients who met inclusion criteria for our high-risk cohort. Chart review included the presence of an ENT, Primary Care, or Sleep Medicine encounter, time to follow-up, the presence of a post-operative PSG, time to post-operative PSG, and the presence of an annual follow-up with any provider. Results Although 83% of our cohort followed-up with any provider, only 31% completed a post-operative PSG. Patients consistently followed-up with ENT 6–8 weeks postoperatively (76%) and haphazardly followed-up with primary care (38%). All patients with a Sleep Medicine follow-up (19%, n=13) completed a post-operative PSG, with 11 of the 13 occurring within 1 year from surgery. There was no significant difference across isolated moderate, isolated severe, or moderate/severe with a comorbidity for incidence of follow-up by specialty, annual follow-up, or post-operative PSG completion. However, patients with isolated severe (AHI >10) completed a PSG on average 13.5 weeks post-operatively which was significantly sooner than 36.2 weeks for isolated moderate OSA (p=0.04). Conclusion Although Sleep Medicine providers may consistently follow AASM practice parameters, variability exists for which patients return to complete a post-operative PSG. Severity of OSAS or presence of a concerning co-morbidity does not seem to correlate with acquiring a postoperative PSG. An inconsistent standard across disciplines may contribute to this discrepancy. These findings will inform future quality improvement discussions with key stakeholders. In light of this baseline assessment, we plan to recommend a standardized, multi-disciplinary care pathway for the management of high-risk, pediatric OSAS. Support (if any) None