Publications by authors named "Timothy Craig"

262 Publications

Long-term health-related quality of life in patients treated with subcutaneous C1-inhibitor replacement therapy for the prevention of hereditary angioedema attacks: findings from the COMPACT open-label extension study.

Orphanet J Rare Dis 2021 Feb 15;16(1):86. Epub 2021 Feb 15.

University College Hospital, London, UK.

Background: Long-term prophylaxis with subcutaneous C1-inhibitor (C1-INH[SC]; HAEGARDA, CSL Behring) in patients with hereditary angioedema (HAE) due to C1-INH deficiency (C1-INH-HAE) was evaluated in an open-label extension follow-up study to the international, double-blind, placebo-controlled COMPACT study. The current analysis evaluated patient-reported health-related quality of life (HRQoL) data from 126 patients in the open-label extension study randomized to treatment with C1-INH(SC) 40 IU/kg (n = 63) or 60 IU/kg (n = 63) twice weekly for 52 weeks. HRQoL was evaluated at the beginning of the open-label study and at various time points using the European Quality of Life-5 Dimensions Questionnaire (EQ-5D), the Hospital Anxiety and Depression Scale (HADS), the Work Productivity and Activity Impairment Questionnaire (WPAI), and the Treatment Satisfaction Questionnaire for Medication. The disease-specific Angioedema Quality of Life Questionnaire (AE-QoL) and HAE quality of life questionnaire (HAE-QoL) instruments were administered in a subset of patients. Statistical significance was determined by change-from-baseline 95% confidence intervals (CIs) excluding zero. No adjustment for multiplicity was done.

Results: Mean baseline EQ-5D scores (Health State Value, 0.90; Visual Analog Scale, 81.32) were slightly higher (better) than United States population norms (0.825, 80.0, respectively) and mean HADS anxiety (5.48) and depression (2.88) scores were within "normal" range (0-7). Yet, patients using C1-INH(SC) 60 IU/kg demonstrated significant improvement from baseline to end-of-study on the EQ-5D Health State Value (mean change [95% CI], 0.07 [0.01, 0.12] and Visual Analog Scale (7.45 [3.29, 11.62]). In the C1-INH(SC) 60 IU/kg group, there were significant improvements in the HADS anxiety scale (mean change [95% CI], - 1.23 [- 2.08, - 0.38]), HADS depression scale (- 0.95 [- 1.57, - 0.34]), and WPAI-assessed presenteeism (mean change [95% CI], - 23.33% [- 34.86, - 11.81]), work productivity loss (- 26.68% [- 39.92, - 13.44]), and activity impairment (- 16.14% [- 26.36, - 5.91]). Clinically important improvements were achieved in ≥ 25% of patients for all domains except WPAI-assessed absenteeism (which was very low at baseline). Mean AE-QoL total score by visit ranged from 13.39 to 17.89 (scale 0-100; lower scores = less impairment). Mean HAE-QoL global scores at each visit (115.7-122.3) were close to the maximum (best) possible score of 135.

Conclusions: Long-term C1-INH(SC) replacement therapy in patients with C1-INH-HAE leads to significant and sustained improvements in multiple measures of HRQoL. Trial registration A Study to Evaluate the Long-term Clinical Safety and Efficacy of Subcutaneously Administered C1-esterase Inhibitor in the Prevention of Hereditary Angioedema, NCT02316353. Registered December 12, 2014, https://clinicaltrials.gov/ct2/show/NCT02316353 .
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http://dx.doi.org/10.1186/s13023-020-01658-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7885603PMC
February 2021

Correction to: Inhaled corticosteroids as treatment for adolescent asthma: effects on adult anxiety-related outcomes in a murine model.

Psychopharmacology (Berl) 2021 Feb 10. Epub 2021 Feb 10.

Department of Biobehavioral Health, Pennsylvania State University, 219 Biobehavioral Health Building, University Park, PA, USA.

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http://dx.doi.org/10.1007/s00213-021-05778-yDOI Listing
February 2021

Multiplex Single-Molecule Kinetics of Nanopore-Coupled Polymerases.

ACS Nano 2021 01 28;15(1):489-502. Epub 2020 Dec 28.

Harvard Medical School, Department of Genetics, Boston, Massachusetts 02115, United States.

DNA polymerases have revolutionized the biotechnology field due to their ability to precisely replicate stored genetic information. Screening variants of these enzymes for specific properties gives the opportunity to identify polymerases with different features. We have previously developed a single-molecule DNA sequencing platform by coupling a DNA polymerase to an α-hemolysin pore on a nanopore array. Here, we use this approach to demonstrate a single-molecule method that enables rapid screening of polymerase variants in a multiplex manner. In this approach, barcoded DNA strands are complexed with polymerase variants and serve as templates for nanopore sequencing. Nanopore sequencing of the barcoded DNA reveals both the barcode identity and kinetic properties of the polymerase variant associated with the cognate barcode, allowing for multiplexed investigation of many polymerase variants in parallel on a single nanopore array. Further, we develop a robust classification algorithm that discriminates kinetic characteristics of the different polymerase mutants. As a proof of concept, we demonstrate the utility of our approach by screening a library of ∼100 polymerases to identify variants for potential applications of biotechnological interest. We anticipate our screening method to be broadly useful for applications that require polymerases with altered physical properties.
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http://dx.doi.org/10.1021/acsnano.0c05226DOI Listing
January 2021

How Frequently Is Asthma Objectively Demonstrated before Starting a Biologic? Quality Assessment of a Group Practice of Allergists and Immunologists.

Int J Environ Res Public Health 2020 12 18;17(24). Epub 2020 Dec 18.

Section of Allergy and Immunology, Department of Medicine, Penn State College of Medicine, Hershey, PA 17033, USA.

Worldwide, asthma-related healthcare cost remains a major burden. Individuals with severe asthma account for 50% of that cost. Although they are expensive, biologics such as anti-IL5 and anti-IgE agents promise cost-effectiveness when judiciously used to decrease asthma-related hospitalization and the debilitating side effects of systemic corticosteroids. Before considering biologics to treat patients with asthma, current guidelines recommend confirmation of asthma and control of comorbid diseases. Diagnostic confirmation of asthma can be challenging among individuals with severe asthma. In this quality assessment study, we determined the frequency of objective asthma confirmation and addressing of comorbidities prior to starting biologics at a group practice of allergists and immunologists. We surveyed our specialty providers to understand habit(s) leading to the observed results. We identified 40 adult patients who started on biologic modifiers for asthma over the past 5 years. Only 58% of these patients had a proper diagnosis of asthma. Providers underutilized several diagnostic methods that may prove useful in confirming asthma diagnosis in this patient population. The factors contributing to poor asthma control were rarely addressed. A sense of urgency to initiate biologics was the primary reason for the observed results. Further interventions are needed to improve asthma diagnosis and management prior to the initiation of biologic therapeutics.
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http://dx.doi.org/10.3390/ijerph17249482DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7766929PMC
December 2020

Androgen use in hereditary angioedema: A critical appraisal and approaches to transitioning from androgens to other therapies.

Allergy Asthma Proc 2021 01 21;42(1):22-29. Epub 2020 Dec 21.

Division of Rheumatology, Allergy and Immunology, Department of Medicine, University of California San Diego, La Jolla, California.

Hereditary angioedema (HAE) is a rare genetic disorder clinically characterized by recurrent attacks of subcutaneous and mucosal swelling. Attenuated androgens have been a prophylactic treatment option to reduce the frequency of HAE attacks for > 4 decades. However, the advent of effective on-demand treatments and highly effective, more tolerable, long-term prophylactic therapies has led to a decline in the use of attenuated androgens for the management of HAE in regions where newer therapies are available. A consensus about the best approach for discontinuing or tapering off attenuated androgen therapy does not exist. To develop a consensus on androgen tapering for patients with HAE. We sent an open-ended survey about androgen tapering to 21 physicians who treat HAE, 12 of whom responded. We reviewed the collective experience of the participating physicians in combination with results from a literature review on the topic. The survey and literature review underscored potential concerns related to rapid androgen withdrawal in patients with HAE, including physician and patient concerns that the frequency and severity of attacks would abruptly worsen. In addition, discontinuation of attenuated androgens may have the potential for transient adverse effects, such as an increase in the rate of attacks or effects related to hormone withdrawal. Our survey showed that physicians often taper androgens to prevent increases in HAE attacks and possible withdrawal complications. Based on both experiences of the physicians who responded to our survey and reports in the endocrine literature, we provided recommendations for androgen tapering. However, we noted that the likelihood of adverse effects due to androgen withdrawal in patients with HAE is poorly understood and requires further study.
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http://dx.doi.org/10.2500/aap.2021.42.200106DOI Listing
January 2021

Allergy/Immunology Trainee Experiences During the COVID-19 Pandemic: AAAAI Work Group Report of the Fellows-in-Training Committee.

J Allergy Clin Immunol Pract 2021 01 2;9(1):1-6.e1. Epub 2020 Nov 2.

Department of Medicine, Pediatrics and Biomedical Sciences, Penn State University, Hershey, Pa.

As a result of the coronavirus disease 2019 (COVID-19) global pandemic, medical trainees have faced unique challenges and uncertainties. To capture the experiences of allergy and immunology fellows throughout the United States and Canada during this time, a 17-item electronic questionnaire was distributed to 380 fellow-in-training (FIT) members of the American Academy of Allergy, Asthma, and Immunology enrolled in US and Canadian allergy/immunology fellowship programs. Voluntary and anonymous responses were collected from April 15 to May 15, 2020. In addition to summary statistics, categorical data were compared using χ tests (Fisher's exact). Responses were obtained from FITs across all years of training and primary specialties (Internal Medicine, Pediatrics, and Medicine-Pediatrics) with a response rate of 32.6% (124 of 380). Reassignment to COVID-19 clinical responsibilities was reported by 12% (15 of 124) of FITs, with the largest proportion in the US northeast region. A majority of FITs used telehealth (95%) and virtual learning (82%) during the pandemic. Overall, 21% (25 of 120) of FITs expressed concern about potentially lacking clinical experience for independently practicing allergy and immunology. However, FITs using telehealth reported lower concern compared with those who did not (18.4% [21 of 114] vs 66.7% [4 of 6]; P = .01). The survey shows that allergy and immunology trainee experiences have varied considerably since the COVID-19 outbreak. Notably, the adoption of telehealth and virtual learning was commonly reported, and optimization of these virtual experiences will be helpful. Even outside of pandemics, training on the use of telemedicine may be a sound strategy in preparation for future health care delivery and unexpected events.
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http://dx.doi.org/10.1016/j.jaip.2020.09.036DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7605747PMC
January 2021

Triggers and short-term prophylaxis in patients with hereditary angioedema.

Authors:
Timothy Craig

Allergy Asthma Proc 2020 11;41(Suppl 1):S30-S34

Hereditary angioedema (HAE) is a rare disease that affects 1 in 60,000; however, despite being extremely rare, the severity of the disease can cause significant limitations to quality of life. In addition, attacks can be fatal and require urgent care. We searched PubMed and Google for Hereditary Angioedema and prophylaxis, short term prophylaxis, surgery, medical procedures, dental work, triggers. The main triggers are estrogens, Angiotensin Converting Enzyme Inhibitors (ACI) inhibitors, trauma, dental work, stress, surgery, manipulation of the upper airway, and medical procedures. Prophylaxis is often used long term to prevent attacks; before known triggers, prophylaxis is referred to as short-term prophylaxis (STP). When to initiate STP, what to use, and what dose to use have not been adequately researched, but there is consensus that, whenever the upper airway is manipulated, STP is essential. In addition, consensus has been reached that an IV C1 inhibitor is the preferred STP agent, and it is my opinion that dosing at 20 units/kg allows dosing for all ages and also allows average-size adults to receive >1000 units because failures at 1000 units have been documented in the literature. This article focused on triggers and preprocedural STP and not on pre-event STP, which is often used before important life events; however, medications and dosing are the same for pre-event prophylaxis.
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http://dx.doi.org/10.2500/aap.2020.41.200058DOI Listing
November 2020

Oral once-daily berotralstat for the prevention of hereditary angioedema attacks: A randomized, double-blind, placebo-controlled phase 3 trial.

J Allergy Clin Immunol 2020 Oct 21. Epub 2020 Oct 21.

University of California San Diego, San Diego, Calif.

Background: Berotralstat (BCX7353) is an oral, once-daily inhibitor of plasma kallikrein in development for the prophylaxis of hereditary angioedema (HAE) attacks.

Objective: Our aim was to determine the efficacy, safety, and tolerability of berotralstat in patients with HAE over a 24-week treatment period (the phase 3 APeX-2 trial).

Methods: APeX-2 was a double-blind, parallel-group study that randomized patients at 40 sites in 11 countries 1:1:1 to receive once-daily berotralstat in a dose of 110 mg or 150 mg or placebo (Clinicaltrials.gov identifier NCT03485911). Patients aged 12 years or older with HAE due to C1 inhibitor deficiency and at least 2 investigator-confirmed HAE attacks in the first 56 days of a prospective run-in period were eligible. The primary efficacy end point was the rate of investigator-confirmed HAE attacks during the 24-week treatment period.

Results: A total of 121 patients were randomized; 120 of them received at least 1 dose of the study drug (n = 41, 40, and 39 in the 110-mg dose of berotralstat, 150-mg of dose berotralstat, and placebo groups, respectively). Berotralstat demonstrated a significant reduction in attack rate at both 110 mg (1.65 attacks per month; P = .024) and 150 mg (1.31 attacks per month; P < .001) relative to placebo (2.35 attacks per month). The most frequent treatment-emergent adverse events that occurred more with berotralstat than with placebo were abdominal pain, vomiting, diarrhea, and back pain. No drug-related serious treatment-emergent adverse events occurred.

Conclusion: Both the 110-mg and 150-mg doses of berotralstat reduced HAE attack rates compared with placebo and were safe and generally well tolerated. The most favorable benefit-to-risk profile was observed at a dose of 150 mg per day.
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http://dx.doi.org/10.1016/j.jaci.2020.10.015DOI Listing
October 2020

Effects of Inhaled Corticosteroids and Particle Size on Risk of Obstructive Sleep Apnea: A Large Retrospective Cohort Study.

Int J Environ Res Public Health 2020 10 6;17(19). Epub 2020 Oct 6.

Division of Allergy, Asthma, and Immunology, Penn State Hershey Medical Center, Hershey, PA 17033, USA.

Inhaled corticosteroids (ICS) produce local effects on upper airway dilators that could increase the risk of developing obstructive sleep apnea (OSA). Given that the particle size of ICS changes their distribution, the particle size of ICS may impact the risk of developing OSA. In this large retrospective study, we explore the relationship of ICS use and OSA in patients with asthma. In addition, we seek to determine if this relationship is affected by the particle size of ICS. : Using electronic health records, we established a cohort of 29,816 asthmatics aged 12 and older with a diagnosis of asthma documented by ICD-9 or ICD-10 codes between January 2011 and August 2016. We performed analyses of variance and multivariate logistic regression analysis to determine the effects ICS on the diagnosis of OSA with sub-analysis by particle size of ICS. Uncontrolled asthmatics showed increased odds of receiving a diagnosis of OSA whether when looking at ACT scores (adjusted odds ratio (aOR) 1.60, 95% CI 1.32-1.94) or PFT results (aOR 1.45, 95% CI 1.19-1.77). Users of ICS also had increased odds of OSA independent of asthma control (aOR 1.58, 95% CI 1.47-1.70). Notably, users of extra-fine particle ICS did not have significantly increased odds of having OSA compared to non-users of ICS (aOR 1.11, 95% CI 0.78-1.58). : Use of ICS appears to be an independent risk factor for OSA. Notably, extra-fine particle size ICS do not appear to be associated with an increased risk of OSA.
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http://dx.doi.org/10.3390/ijerph17197287DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7579456PMC
October 2020

Inhaled corticosteroids as treatment for adolescent asthma: effects on adult anxiety-related outcomes in a murine model.

Psychopharmacology (Berl) 2021 Jan 4;238(1):165-179. Epub 2020 Oct 4.

Department of Biobehavioral Health, Pennsylvania State University, 219 Biobehavioral Health Building, University Park, PA, USA.

Rationale: Allergic asthma, typically controlled with inhaled corticosteroids (ICS), is the leading chronic health condition for youth under 18 years of age. During this peri-adolescent period, significant brain maturation occurs. Prior studies indicate that both chronic inflammation and corticosteroid medications increase risk for developing an internalizing disorder like anxiety.

Objectives: To determine if chronic ICS treatments exacerbate or alleviate anxiety symptoms associated with developmental allergic asthma, we used a mouse model to isolate the influence of ICS (fluticasone propionate, FLU) vs. airway inflammation (induced with house dust mite extract, HDM).

Methods: During development, male and female BALB/cJ mice were repeatedly exposed to HDM or saline plus one of four FLU doses (none/vehicle, low, moderate, or high). In adulthood, we assessed lung inflammation, circulating and excreted corticosteroids, anxiety-like behavior, and gene expression in stress and emotion regulation brain regions.

Results: FLU treatment decreased body weight and anxiety-like behavior and increased fecal corticosterone metabolite concentrations and Crhr2 gene expression in ventral hippocampus. FLU effects were only observed in saline/non-HDM-exposed mice, and the FLU doses used did not significantly decrease HDM-induced airway inflammation. Females had greater serum and fecal corticosterone concentrations, less anxiety-like behavior, and lower Crhr1 gene expression in ventral hippocampus and prefrontal cortex than males.

Conclusions: These findings suggest that steroid medications for youth with allergic asthma may not exacerbate anxiety-related symptoms, and that they should be avoided in children/adolescents without a health condition. The results are informative to future work on the use of corticosteroid medications during childhood or adolescent development.
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http://dx.doi.org/10.1007/s00213-020-05666-xDOI Listing
January 2021

US HAEA Medical Advisory Board 2020 Guidelines for the Management of Hereditary Angioedema.

J Allergy Clin Immunol Pract 2021 Jan 6;9(1):132-150.e3. Epub 2020 Sep 6.

Division of Rheumatology, Allergy, and Immunology, Department of Medicine, University of California San Diego, La Jolla, Calif; San Diego Veterans Administration Healthcare, San Diego, Calif. Electronic address:

Scientific and clinical progress together with the development of effective novel therapeutic options has engendered multiple important changes in the diagnosis and management of hereditary angioedema (HAE). We now update and extend the 2013 United States Hereditary Angioedema Association Medical Advisory Board guidelines for the treatment and management of HAE. The guidelines are based on a comprehensive literature review with recommendations indicating both the strength of our recommendation and the quality of the underlying evidence. Guidelines are provided regarding the classification, diagnosis, on-demand treatment, prophylactic treatment, special considerations for women and children, development of a comprehensive management and monitoring plan, and assessment of burden of illness for both HAE due to C1 inhibitor deficiency and HAE with normal C1 inhibitor. Advances in HAE treatment now allow the development of management plans that can help many patients with HAE lead a normal life. Achieving this goal requires that physicians be familiar with the diagnostic and therapeutic transformations that have occurred in recent years.
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http://dx.doi.org/10.1016/j.jaip.2020.08.046DOI Listing
January 2021

Rhinitis 2020: A practice parameter update.

J Allergy Clin Immunol 2020 10 22;146(4):721-767. Epub 2020 Jul 22.

Division of Allergy and Immunology, Department of Pediatrics, The Elliot and Roslyn Jaffe Food Allergy Institute, Icahn School of Medicine at Mount Sinai, New York, NY.

This comprehensive practice parameter for allergic rhinitis (AR) and nonallergic rhinitis (NAR) provides updated guidance on diagnosis, assessment, selection of monotherapy and combination pharmacologic options, and allergen immunotherapy for AR. Newer information about local AR is reviewed. Cough is emphasized as a common symptom in both AR and NAR. Food allergy testing is not recommended in the routine evaluation of rhinitis. Intranasal corticosteroids (INCS) remain the preferred monotherapy for persistent AR, but additional studies support the additive benefit of combination treatment with INCS and intranasal antihistamines in both AR and NAR. Either intranasal antihistamines or INCS may be offered as first-line monotherapy for NAR. Montelukast should only be used for AR if there has been an inadequate response or intolerance to alternative therapies. Depot parenteral corticosteroids are not recommended for treatment of AR due to potential risks. While intranasal decongestants generally should be limited to short-term use to prevent rebound congestion, in limited circumstances, patients receiving regimens that include an INCS may be offered, in addition, an intranasal decongestant for up to 4 weeks. Neither acupuncture nor herbal products have adequate studies to support their use for AR. Oral decongestants should be avoided during the first trimester of pregnancy. Recommendations for use of subcutaneous and sublingual tablet allergen immunotherapy in AR are provided. Algorithms based on a combination of evidence and expert opinion are provided to guide in the selection of pharmacologic options for intermittent and persistent AR and NAR.
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http://dx.doi.org/10.1016/j.jaci.2020.07.007DOI Listing
October 2020

POPS case: A 30-year-old Filipino woman with fevers, lymphadenopathy, painful scalp lesions, and a neck mass.

Allergy Asthma Proc 2020 07;41(4):305-308

Division of Hospital Medicine, Penn State Health Milton S. Hershey Medical Center, Hershey, Pennsylvania.

We described a case of a 30-year-old Filipino woman who presented with fevers, night sweats, left hip pain, painful scalp lesions, and a neck mass. Symptoms began 6 months earlier, with nasal drainage, fever, cough, and occasional hemoptysis, which did not resolve with outpatient antibiotics. A further workup revealed lymphadenopathy and several lytic bone lesions. Her hospital course was later further complicated by the development of a tracheoesophageal fistula secondary to an esophageal mass and, then later, aseptic meningitis. Extensive diagnostic workup and immunologic tests were performed and finally led to the diagnosis. Here, we discussed the diagnostic workup and pathophysiology of the underlying condition. This case illustrated the importance of appropriate immunologic workup to make the diagnosis of a rare condition that proves to be clinically significant and presents challenges in management.
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http://dx.doi.org/10.2500/aap.2020.41.200012DOI Listing
July 2020

Impact of COVID-19 on Pediatric Asthma: Practice Adjustments and Disease Burden.

J Allergy Clin Immunol Pract 2020 09 17;8(8):2592-2599.e3. Epub 2020 Jun 17.

Department of Pediatrics & Child Health, MRC Unit on Child & Adolescent Health, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.

Background: It is unclear whether asthma may affect susceptibility or severity of coronavirus disease 2019 (COVID-19) in children and how pediatric asthma services worldwide have responded to the pandemic.

Objective: To describe the impact of the COVID-19 pandemic on pediatric asthma services and on disease burden in their patients.

Methods: An online survey was sent to members of the Pediatric Asthma in Real Life think tank and the World Allergy Organization Pediatric Asthma Committee. It included questions on service provision, disease burden, and the clinical course of confirmed cases of COVID-19 infection among children with asthma.

Results: Ninety-one respondents, caring for an estimated population of more than 133,000 children with asthma, completed the survey. COVID-19 significantly impacted pediatric asthma services: 39% ceased physical appointments, 47% stopped accepting new patients, and 75% limited patients' visits. Consultations were almost halved to a median of 20 (interquartile range, 10-25) patients per week. Virtual clinics and helplines were launched in most centers. Better than expected disease control was reported in 20% (10%-40%) of patients, whereas control was negatively affected in only 10% (7.5%-12.5%). Adherence also appeared to increase. Only 15 confirmed cases of COVID-19 were reported among the population; the estimated incidence is not apparently different from the reports of general pediatric cohorts.

Conclusions: Children with asthma do not appear to be disproportionately affected by COVID-19. Outcomes may even have improved, possibly through increased adherence and/or reduced exposures. Clinical services have rapidly responded to the pandemic by limiting and replacing physical appointments with virtual encounters.
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http://dx.doi.org/10.1016/j.jaip.2020.06.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7297686PMC
September 2020

The use of exhaled nitric oxide and peak expiratory flow to demonstrate improved breathability and antimicrobial properties of novel face mask made with sustainable filter paper and oil: additional option for mask shortage during COVID-19 pandemic.

Multidiscip Respir Med 2020 Jan 1;15(1):664. Epub 2020 Jun 1.

Division of Immuno-Allergology, Hershey Medical Center, Penn State Medical College, Hershey, PA, USA.

Background: Medical face masks are integral personal protective equipment against infectious airborne disease and become scarce during epidemic outbreaks such as COVID-19. A novel, sustainably manufactured face mask with antimicrobial and anti-inflammatory properties from oil of can be an effective alternative to internationally sold masks.

Methods: This prospective, randomized study assigned subjects (n=67) to either conventional surgical face mask or Lamdong Medical College (LMC) face mask for three hours. Fractional concentration of nitric oxide in exhaled breath (E) and peak expiratory flow (PEF) was measured before and after mask use. Subjective reporting on respiratory symptoms was also analyzed. Masks were then incubated and analyzed for microorganism growth.

Results: Subjects assigned the LMC mask had a lowered E (<0.05) compared to conventional face masks after mask wearing. Subjects with LMC mask use reported higher comfortability (<0.05), breathability (<0.05), and lower allergy symptoms (<0.05). The LMC mask has visually less microorganism growth in the cultured medium, measured by sterile ring radius.

Conclusions: The LMC face mask is a renewably manufactured personal protective tool with antibacterial capacity that can serve as an effective alternative to internationally sold surgical face mask during shortage of mask due to COVID-19.
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http://dx.doi.org/10.4081/mrm.2020.664DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7282423PMC
January 2020

Association Between Self-Reported Dental Hygiene Practices and Dental Procedure-Related Recurrent Angioedema Attacks in HAE Subjects: A Multicenter Survey.

J Allergy Clin Immunol Pract 2020 Oct 10;8(9):3162-3169.e5. Epub 2020 Jun 10.

Division of Immunology/Allergy, Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio. Electronic address:

Background: Hereditary angioedema (HAE) symptoms may be triggered by dental procedures, thereby complicating dental care in individuals affected by the condition.

Objective: This study investigated the self-perceived dental care needs, perceived susceptibility to acute angioedema (AE) attacks after dental procedures, and dental care behavior of patients with HAE.

Methods: A self-administered semistructured web-based questionnaire was distributed to 250 adult patients with HAE (type 1 or 2; 88% type 1) and 256 matched non-HAE controls. Data were analyzed using stratified χ tests, logistic regression, and classification trees.

Results: A total of 46.4% of HAE versus 55.5% of control patients had dental visits within 6 months (P = .04). Dental insurance was a barrier to seeking routine dental visits among both groups. However, significantly fewer patients with HAE had routine dental visits within 6 months despite having dental insurance compared with control patients (48% vs 60%, P = .01). Within the HAE group, a significantly greater number of patients with dental visits at intervals greater than 6 months had a history of recurrent postprocedural AE attacks (odds ratio [OR]: 3.9 [1.7, 8.8], P = .0005) and used antibacterial toothpaste more frequently than those without recurrent AE attacks (OR: 4.7 [1.5, 15.4], P = .005).

Conclusions: These data support the hypothesis that patients with HAE who are predisposed to having AE episodes in response to medical or physical trauma visit the dentist less and engage in specific oral hygiene practices more frequently than matched control patients and patients with HAE who reported that they were less likely to swell after a dental procedure.
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http://dx.doi.org/10.1016/j.jaip.2020.05.041DOI Listing
October 2020

Co-occurrence between C1 esterase inhibitor deficiency and autoimmune disease: a systematic literature review.

Allergy Asthma Clin Immunol 2020 27;16:41. Epub 2020 May 27.

CSL Behring, Marburg, Germany.

Background: Hereditary angioedema (HAE) is caused by a SERPING1 gene defect resulting in decreased (Type I) or dysfunctional (Type II) C1 esterase inhibitor (C1-INH). The prevalence of autoimmune diseases (ADs) in patients with HAE appears to be higher than the general population. A systematic literature review was conducted to examine the co-occurrence between HAE and ADs.

Methods: PubMed/EMBASE were searched for English-language reviews, case reports, observational studies, retrospective studies, and randomized controlled trials up to 04/15/2018 (04/15/2015-04/15/2018 for EMBASE) that mentioned patients with HAE Type I or II and comorbid ADs. Non-human or in vitro studies and publications of C1-INH deficiency secondary to lymphoproliferative disorders or angiotensin-converting-enzyme inhibitors were excluded.

Results: Of the 2880 records screened, 76 met the eligibility criteria and 155 individual occurrences of co-occurring HAE and AD were mentioned. The most common ADs were systemic lupus erythematosus (30 mentions), thyroid disease (21 mentions), and glomerulonephritis (16 mentions). When ADs were grouped by MedDRA v21.0 High Level Terms, the most common were: Lupus Erythematosus and Associated Conditions, n = 52; Endocrine Autoimmune Disorders, n = 21; Gastrointestinal Inflammatory Conditions, n = 16; Glomerulonephritis and Nephrotic Syndrome, n = 16; Rheumatoid Arthritis and Associated Conditions, n = 11; Eye, Salivary Gland and Connective Tissue Disorders, n = 10; and Immune and Associated Conditions Not Elsewhere Classified, n = 5.

Conclusions: Based on literature reports, systemic lupus erythematosus is the most common AD co-occurring with HAE Type I and II. Cause and effect for co-occurring HAE and AD has not been clinically established but could be related to lack of sufficient C1-INH function.
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http://dx.doi.org/10.1186/s13223-020-00437-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7254644PMC
May 2020

Study of the role of exhaled nitric oxide (NO) in predicting controlled or uncontrolled asthma in asthmatic children.

Multidiscip Respir Med 2020 Jan 13;15(1):656. Epub 2020 May 13.

Department of Medicine, Pham Ngoc Thach University, Ho Chi Minh city, Vietnam.

Background: Exhaled nitric oxide (NO), especially fractional concentration of exhaled NO (E) has been used to predict the responsiveness to inhaled corticosteroid (ICS) in children with asthma. However, the use of exhaled NO for predicting asthma control in children is still controversial.

Methods: This was a perspective observational study. Asthmatic children who were naïve to inhaled corticosteroid (ICS) were included in the present study. The measurements of E and A (concentration of NO in the gas phase of the alveolar), spirometry, blood eosinophil counts (BEC), and total IgE levels were done for each asthmatic child. All study subjects started proper asthma treatment after the enrollment.

Results: Ninety three asthmatic children (9±3 years) with moderate (63.4%) to severe (36.6%) asthma were included and finished the 3-month study. The levels of E and A at inclusion were 37±11 ppb and 5.8±1.4 ppb, respectively; the mean of BEC was 617±258 cells/μL; the level of total IgE was 1563±576 UI/mL; 89% of subjects were positive for at least one respiratory allergen. The percentage of severe asthma was reduced significantly after 3 months (P<0.001). Well controlled asthma subjects at 3 months had higher levels of E and lower levels of A at inclusion (P<0.05 and P<0.05). E<20 ppb or A>5ppb had a risk of uncontrolled asthma at 3 months (OR: 1.7, CI 95% [(0.8) - (3.3)], P<0.05; OR: 1.9, CI 95% [(0.9) - (2.7)], P<0.05; respectively). E>35 ppb at inclusion had a positive predictive value for asthma control at 3 months (OR: 3.5, CI 95% [2.2-5.9], P<0.01).

Conclusions: Exhaled NO is a biomarker of asthma which may have a potential role to predict the control of asthma in short-term follow up in asthmatic children.
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http://dx.doi.org/10.4081/mrm.2020.656DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7232017PMC
January 2020

A Review: Does Complement or the Contact System Have a Role in Protection or Pathogenesis of COVID-19?

Pulm Ther 2020 Dec 13;6(2):169-176. Epub 2020 May 13.

Department of Medicine and Pediatrics, Penn State University, 500 University Drive, Hershey, PA, 17033, USA.

Introduction: COVID-19 presentation may include a profound increase in cytokines and associated pneumonia, rapidly progressing to acute respiratory distress syndrome (ARDS). This so-called cytokine storm often leads to refractory edema, respiratory arrest, and death. At present, anti-IL-6, antiviral therapy, convalescent plasma, hydroxychloroquine, and azithromycin among others are being investigated as potential treatments for COVID-19. As the disease etiology and precise therapeutic interventions are still not definitively defined, we wanted to review the roles that complement and the contact system may have in either the treatment or pathogenesis of the disease.

Methods: We searched the recent literature (PubMed) on complement and coronavirus; contact system and coronavirus; bradykinin and coronavirus; and angiotensin receptor and coronavirus. The manuscript complies with ethics guidelines and was deemed exempt from institutional review board approval according to Human Subjects Protection Office guidelines.

Results: Mouse models are available for the study of coronavirus and complement. Although complement is effective in protecting against many viruses, it does not seem to be protective against coronavirus. C3 knockout mice infected with SARS-CoV had less lung disease than wild-type mice, suggesting that complement may play a role in coronavirus pathogenesis. Some evidence suggests that the observed pulmonary edema may be bradykinin-induced and could be the reason that corticosteroids, antihistamines, and other traditional interventions for edema are not effective. Angiotensin-converting enzyme 2 (ACE2) is a co-receptor for SARS-CoV-2, and studies thus far have not concluded a benefit or risk associated with the use of either ACE-inhibitors or angiotensin receptor antagonists. Activation of complement and the contact system, through generation of bradykinin, may play a role in the SARS-CoV-2-induced pulmonary edema, and our search suggests that further work is necessary to confirm our suspicions.
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http://dx.doi.org/10.1007/s41030-020-00118-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7218701PMC
December 2020

Definition, aims, and implementation of GA LEN/HAEi Angioedema Centers of Reference and Excellence.

Authors:
Marcus Maurer Werner Aberer Rosana Agondi Mona Al-Ahmad Maryam Ali Al-Nesf Ignacio Ansotegui Rand Arnaout Luisa Karla Arruda Riccardo Asero Emel Aygören-Pürsün Aleena Banerji Andrea Bauer Moshe Ben-Shoshan Alejandro Berardi Jonathan A Bernstein Stephen Betschel Carsten Bindslev-Jensen Mojca Bizjak Isabelle Boccon-Gibod Konrad Bork Laurence Bouillet Henrik Balle Boysen Nicholas Brodszki Sigurd Broesby-Olsen Paula Busse Thomas Buttgereit Anette Bygum Teresa Caballero Régis A Campos Mauro Cancian Ivan Cherrez-Ojeda Danny M Cohn Célia Costa Timothy Craig Paulo Ricardo Criado Roberta F Criado Dorottya Csuka Joachim Dissemond Aurélie Du-Thanh Luis Felipe Ensina Ragıp Ertaş José E Fabiani Claudio Fantini Henriette Farkas Silvia Mariel Ferrucci Ignasi Figueras-Nart Natalia L Fili Daria Fomina Atsushi Fukunaga Asli Gelincik Ana Giménez-Arnau Kiran Godse Mark Gompels Margarida Gonçalo Maia Gotua Richard Gower Anete S Grumach Guillermo Guidos-Fogelbach Michihiro Hide Natalia Ilina Naoko Inomata Thilo Jakob Dario O Josviack Hye-Ryun Kang Allen Kaplan Alicja Kasperska-Zając Constance Katelaris Aharon Kessel Andreas Kleinheinz Emek Kocatürk Mitja Košnik Dorota Krasowska Kanokvalai Kulthanan M Sendhil Kumaran José Ignacio Larco Sousa Hilary J Longhurst William Lumry Andrew MacGinnitie Markus Magerl Michael P Makris Alejandro Malbrán Alexander Marsland Inmaculada Martinez-Saguer Iris V Medina Raisa Meshkova Martin Metz Iman Nasr Jan Nicolay Chikako Nishigori Isao Ohsawa Kemal Özyurt Nikolaos G Papadopoulos Claudio A S Parisi Jonathan Grant Peter Wolfgang Pfützner Todor Popov Nieves Prior German D Ramon Adam Reich Avner Reshef Marc A Riedl Bruce Ritchie Heike Röckmann-Helmbach Michael Rudenko Andaç Salman Mario Sanchez-Borges Peter Schmid-Grendelmeier Faradiba S Serpa Esther Serra-Baldrich Farrukh R Sheikh William Smith Angèle Soria Petra Staubach Urs C Steiner Marcin Stobiecki Gordon Sussman Anna Tagka Simon Francis Thomsen Regina Treudler Solange Valle Martijn van Doorn Lilian Varga Daniel O Vázquez Nicola Wagner Liangchun Wang Christina Weber-Chrysochoou Young-Min Ye Anna Zalewska-Janowska Andrea Zanichelli Zuotao Zhao Yuxiang Zhi Torsten Zuberbier Ricardo D Zwiener Anthony Castaldo

Allergy 2020 08 27;75(8):2115-2123. Epub 2020 Apr 27.

HAE International (HAEi), Fairfax City, VA, USA.

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http://dx.doi.org/10.1111/all.14293DOI Listing
August 2020

A focus on the use of subcutaneous C1-inhibitor for treatment of hereditary angioedema.

Expert Rev Clin Immunol 2020 05 6;16(5):451-455. Epub 2020 Apr 6.

Department of Allergy and Immunology, Penn State University , State College, PA, USA.

Introduction: HAE is a very debilitating disease that causes significant distress for patients not only during an acute attack but also constant fear for a subsequent attack. It is important to address long-term prophylactic (LTP) therapy to prevent attacks, decrease morbidity and increase the quality of life. When discussing LTP, the drug burden, convenience and efficacy must be taken into account.

Areas Covered: We review the literature and the different phases of clinical trials leading up to approval by the US FDA of subcutaneous highly concentrated C1-Inhibitor (SC-C1-INH), called Haegarda. The dose approved is of 60 IU/kg twice weekly showing significant improvement in the reduction of attacks and need for on-demand therapy for attacks with minimal side effects.

Expert Opinion: SC-C1-INH has added significantly to the armamentarium of physicians that treat HAE. The ability to achieve a steady state of C1-INH above 40% function is key to the success of the drug. The drug burden is an SC injection twice a week that exceeds the newly approved lanadelumab. The benefit may be that the protein that is deficient in HAE is replaced and with this the complement, fibrinolytic, coagulation pathways, and contact system are also regulated; however, evidence that this is of benefit is still lacking.
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http://dx.doi.org/10.1080/1744666X.2020.1750953DOI Listing
May 2020

Environmentally triggered variability in the genetic variance-covariance of herbivory resistance of an exotic plant .

Ecol Evol 2020 Mar 2;10(6):3103-3111. Epub 2020 Mar 2.

Center for Ecological Research Kyoto University Otsu Japan.

The variability in the genetic variance-covariance (G-matrix) in plant resistance and its role in the evolution of invasive plants have been long overlooked. We conducted an additional analysis of the data of a reciprocal transplant experiment with tall goldenrod, , in multiple garden sites within its native range (USA) and introduced range (Japan). We explored the differences in G-matrix of resistance to two types of foliar herbivores: (a) a lace bug that is native to the USA and recently introduced to Japan, (b) and other herbivorous insects in response to plant origins and environments. A negative genetic covariance was found between plant resistances to lace bugs and other herbivorous insects, in all combinations of garden locations and plant origins except for US plants planted in US gardens. The G-matrix of the resistance indices did not differ between US and Japanese plants either in US or Japanese gardens, while it differed between US and Japanese gardens in both US and Japanese plants. Our results suggested that the G-matrix of the plant resistance may have changed in response to novel environmental differences including herbivore communities and/or other biotic and abiotic factors in the introduced range. This may have revealed a hidden trade-off between resistances, masked by the environmental factors in the origin range. These results suggest that the stability of the genetic covariance during invasion, and the environmentally triggered variability in the G-matrices of plant resistance may help to protect the plant against multiple herbivore species without changing its genetic architecture and that this may lead to a rapid adaptation of resistance in exotic plants. Local environments of the plant also have a critical effect on plant resistance and should be considered in order to understand trait evolution in exotic plants.
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http://dx.doi.org/10.1002/ece3.6130DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7083677PMC
March 2020

Research Priorities in Pediatric Asthma: Results of a Global Survey of Multiple Stakeholder Groups by the Pediatric Asthma in Real Life (PeARL) Think Tank.

J Allergy Clin Immunol Pract 2020 06 4;8(6):1953-1960.e9. Epub 2020 Mar 4.

Division of Infection, Immunity and Respiratory Medicine, School of Biological Sciences, The University of Manchester, Manchester, United Kingdom; Allergy Department, 2nd Paediatric Clinic, National and Kapodistrian University of Athens, Athens, Greece. Electronic address:

Background: Pediatric asthma remains a public health challenge with enormous impact worldwide.

Objective: The aim of this study was to identify and prioritize unmet clinical needs in pediatric asthma, which could be used to guide future research and policy activities.

Methods: We first identified unmet needs through an open-question survey administered to international experts in pediatric asthma who were members of the Pediatric Asthma in Real Life Think Tank. Prioritization of topics was then achieved through a second, extensive survey with global reach, of multiple stakeholders (leading experts, researchers, clinicians, patients, policy makers, and the pharmaceutical industry). Differences across responder groups were compared.

Results: A total of 57 unmet clinical need topics identified by international experts were prioritized by 412 participants from 5 continents and 60 countries. Prevention of disease progression and prediction of future risk, including persistence into adulthood, emerged as the most urgent research questions. Stratified care, based on biomarkers, clinical phenotypes, the children's age, and demographics were also highly rated. The identification of minimum diagnostic criteria in different age groups, cultural perceptions of asthma, and best treatment by age group were priorities for responders from low-middle-income countries. There was good agreement across different stakeholder groups in all domains with some notable exceptions that highlight the importance of involving the whole range of stakeholders in formulation of recommendations.

Conclusions: Different stakeholders agree in the majority of research and strategic (eg, prevention, personalized approach) priorities for pediatric asthma. Stakeholder diversity is crucial for highlighting divergent issues that future guidelines should consider.
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http://dx.doi.org/10.1016/j.jaip.2020.01.059DOI Listing
June 2020

The Role of C1-Esterase Inhibitors in the Management of Vasogenic Edema in Glioblastoma.

Case Rep Med 2020 28;2020:7981609. Epub 2020 Jan 28.

Penn State University College of Medicine, Hershey, PA, USA.

Glioblastoma (GB) is one of the most common adult primary brain tumors, classified as a grade IV astrocytoma and highly malignant in nature. As the tumor grows and disrupts the blood-brain barrier (BBB), vasogenic edema can result. The edema has the potential to significantly contribute to a patient's morbidity and mortality. Bradykinin has been theorized to play a role in this process as well as encourage tumor spread. Here we discuss a case in which a patient with vasogenic edema and angioedema refractory to antihistamines and high dose corticosteroids responded to C1-esterase inhibitor (C1INH) therapy. Though data exist concerning the role of bradykinin in GB, no clinical studies using C1INH have been done in humans with GB.
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http://dx.doi.org/10.1155/2020/7981609DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7011394PMC
January 2020

Promotion of early-career discernment by an allergy and immunology medical student interest group.

Ann Allergy Asthma Immunol 2020 03 16;124(3):231-232. Epub 2019 Dec 16.

Department of Medicine and Pediatrics, Penn State University, Hershey, Pennsylvania. Electronic address:

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http://dx.doi.org/10.1016/j.anai.2019.12.008DOI Listing
March 2020

The International/Canadian Hereditary Angioedema Guideline.

Allergy Asthma Clin Immunol 2019 25;15:72. Epub 2019 Nov 25.

39Department of Internal Medicine, Queen's University, Kingston, ON Canada.

This is an update to the 2014 Canadian Hereditary Angioedema Guideline with an expanded scope to include the management of hereditary angioedema (HAE) patients worldwide. It is a collaboration of Canadian and international HAE experts and patient groups led by the Canadian Hereditary Angioedema Network. The objective of this guideline is to provide evidence-based recommendations, using the GRADE system, for the management of patients with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. New to the 2019 version of this guideline are sections covering the diagnosis and recommended therapies for acute treatment in HAE patients with normal C1-INH, as well as sections on pregnant and paediatric patients, patient associations and an HAE registry. Hereditary angioedema results in random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased health-related quality of life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada, as in many countries, continues to be neither optimal nor uniform. It lags behind some other countries where there are more organized models for HAE management, and greater availability of additional licensed therapeutic options. It is anticipated that providing this guideline to caregivers, policy makers, patients, and advocates will not only optimize the management of HAE, but also promote the importance of individualized care. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency and intensive care physicians, primary care physicians, gastroenterologists, dentists, otolaryngologists, paediatricians, and gynaecologists who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.
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http://dx.doi.org/10.1186/s13223-019-0376-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6878678PMC
November 2019

Inexpensive robotic system for standard and fluorescent imaging of protein crystals.

Acta Crystallogr F Struct Biol Commun 2019 Nov 5;75(Pt 11):673-686. Epub 2019 Nov 5.

HarkerBIO LLC, 700 Ellicott Street, Buffalo, NY 14203, USA.

Protein-crystallization imaging and classification is a labor-intensive process typically performed either by humans or by instruments that currently cost well over $100 000. This cost puts the use of crystallization-trial imaging outside the reach of most academic laboratories, and also start-up biotechnology firms, where resources are scarce. An imaging system has been designed and prototyped which automatically captures images from multi-well protein-crystallization experiments using both standard and fluorescent imaging techniques at a cost 28 times lower than current market rates. The machine uses a Panowin F1 3D printer as a base and controls it using G-code commands sent from a Python script running on a desktop computer. A graphical user interface (GUI) was developed to enable users to control the machine and facilitate image capture, classification and editing. A 488 nm laser diode and a 525 nm filter were incorporated to allow in situ fluorescent imaging of proteins trace-labeled with a fluorophore, Alexa Fluor 488. The instrument was primarily designed using a 3D printer and augmented using commercially available parts, and this publication aims to serve as a guide for comparable in-laboratory robotics projects.
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http://dx.doi.org/10.1107/S2053230X19014730DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6839817PMC
November 2019

Hereditary angioedema in children: a review and update.

Curr Opin Pediatr 2019 12;31(6):863-868

Department of Medicine and Pediatrics at Penn State University, College of Medicine, Hershey, Pennsylvania, USA.

Purpose Of Review: Hereditary angioedema (HAE) most often presents in the first two decades of life. Despite these patients often see multiple doctors and go many years before confirmation of the diagnosis. the impact on quality of life, productivity and risk of anxiety, depression, and posttraumatic stress emphasizes the need for early diagnosis and appropriate treatment.

Recent Findings: Over the past decade, therapy in the USA has emerged from fresh-frozen plasma and androgens to more than seven medications that are specific for bradykinin-induced disease. During the same time, treatment has evolved from intravenous to subcutaneous and the future will be a focus on oral therapy.

Summary: Much optimism exists that patients with HAE will live a life with minimal disease and impact on their quality of life making it even more important to diagnose children at an early age.
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http://dx.doi.org/10.1097/MOP.0000000000000832DOI Listing
December 2019