The current pandemic of the novel coronavirus SARS-CoV-2 infection has affected over 6 million humans around the planet. The clinical manifestations of Coronavirus disease 2019 (COVID-19) are diverse, ranging from asymptomatic or mild flu-like symptoms to atypical pneumonia, severe respiratory distress syndrome, systemic inflammation, immune dysregulation and dyscoagulation.Inborn errors of immunity (IEI) are a heterogenous group of more than 430 rare congenital disorders with increased susceptibility to infection, autoimmunity, atopy, hyperinflammation and cancer. Autosomal recessive ARPC1B deficiency is an actinopathy, as are DOCK8 deficiency and the Wiskott-Aldrich Syndrome. Defective actin polymerization affects hematopoietic cells, impairing their migration and immunological synapse1, which results in a combined immune deficiency characterized by leukocytosis, eosinophilia, platelet abnormalities and hypergammaglobulinemia; and clinically, by eczema and food allergy, infections caused by bacteria, fungi and viruses, vasculitis, and bleeding diathesis2.Here, we describe a male infant patient with known ARPC1B deficiency who was hospitalized for COVID-19 pneumonia and improved without requiring intensive care or mechanical ventilation.An 8-month-old infant was brought to the emergency department with high-grade fever. His family history is remarkable for one brother who died as a newborn from intracranial bleeding, and an 11-year-old sister with the same genetic defect who underwent hematopoietic stem-cell transplantation twice without success, and is currently on antimycobacterial treatment, antimicrobial prophylaxis and regular subcutaneous immunoglobulin. The patient was first seen at age 1-month old for eczema and rectal bleeding attributed to cow milk protein allergy. At age 4 months, he developed bronchiolitis caused by respiratory syncytial virus (RSV) and oral candidiasis. Laboratory workup revealed leukocytosis (17,500-33,600/mm3), eosinophilia (5,600-20,100/mm3) and a marginally high (467,000) platelet count; as well as high serum IgG (737 mg/dL) and IgA (165 mg/dL) with normal IgM (37.7 mg/dL). CD8+ T lymphocytes were low at 3% (257 cells) and B cells were elevated at 48% (4,116 cells). Whole exome sequencing identified a homozygous 46 base-pair deletion in exon 8 of ARPC1B(chr7:99,392,784 hg38; p.Glu300fs).Upon his arrival to the emergency department he was febrile with tachycardia and signs of septic shock requiring rapid fluid resuscitation. He showed no respiratory or gastrointestinal signs. He also had a post-traumatic ulcerated lesion under the tongue with dark discoloration, which raised a concern for fungal infection. Intravenous antibiotics (ciprofloxacin) with antifungal coverage were started within the first hour, and a dose of intravenous immunoglobulin (IVIG) at 1g/kg. Blood counts revealed leukocytosis, neutrophilia, and mild eosinophilia without lymphopenia, while platelets were initially found within normal limits. A day later, blood culture had grownPseudomonas aeruginosa .During his second day of hospitalization, the patient persisted febrile, tachycardic and tachypneic, with oxygen desaturation into the low 80s. Chest X-ray showed nonspecific bilateral interstitial opacities in the perihilar regions (Figure 1 ). Real-time Polymerase chain reaction (RT-PCR) for SARS-Cov2 came back positive, and he was then transferred to a COVID-19 isolation area. The potassium hydroxide (KOH) test for oral thrush was negative for yeast cells, after which amphotericin was switched to fluconazole. Supplemental oxygen was discontinued on day 6 of hospitalization, when mild thrombocytopenia and a prolonged thromboplastin time (aPTT) (but normal fibrinogen and ferritin serum levels) were reported. After completing 14 days of antimicrobial treatment, the patient was discharged without ever requiring intensive care unit admission or mechanical ventilation.The behavior of COVID-19 in patients with IEI might help dissect the immune response to SARS-Cov2. A few cases of adults with COVID-19 and predominantly antibody deficiencies have been reported3,4; some of them developed acute respiratory distress syndrome (ARDS), while some had a milder course of illness. Based on what we know, innate immune defects in genes involved in type 1 interferon response (such as IRF7, IRF9, TLR3) are the most likely candidates to result in severe disease and death in patients with flu-like virus infection5. In a few cases of fatal influenza A (H1N1), variants in genes associated with familial hemophagocytic lymphohistiocytosis (FHL) and a decreased cytolytic function of NK cells, were also reported6.Our patient was on monthly supplemental IVIG treatment, and he received an additional dose during his hospital stay. This, and his young age, might have ameliorated the clinical course7. He had a favorable evolution, despite the known susceptibility to viral infection and immune dysregulation in ARPC1B deficient patients1. There were no signs of severe infection, ARDS, hyperinflammation or of “cytokine storm” unleashed by SARS-CoV-2. Despite his having a combined immune deficiency, our patient fully recovered without the need of additional supportive measures other than IVIG, supplemental oxygen and antibiotic treatment directed against the documented bacteremia.Although pediatric cases of COVID-19 are fewer compared to adults, some severe presentations and deaths among children have been reported. The presence of a restricted repertoire of IgG (since infants have no previous exposure to coronaviruses) might play a role in the better outcome seen in pediatric patients. Antibody-dependent enhancement has been implicated in the development of severe COVID-19 in the elderly8. Additionally, lung cells from children and women show a lower expression of membrane-bound ACE-2, which may also be protective against severe pneumonia.Conceivably, some immune defects could protect patients with certain IEIs from mounting a full uncontrolled inflammatory response against SARS-Cov2. The cytoskeleton is a regulator of gene transcription, coupling cell mechanics with the activity of NF-κB. Coronaviruses are thought to alter the cytoskeleton architecture to facilitate viral replication and output9. Thus, ARPC1B deficiency and other actinopathies might limit SARS-CoV-2 replication. Furthermore, Th2 cytokines modulate ACE2 (angiotensin-converting enzyme 2) and TMPRSS2 expression in airway epithelial cells10, and children with allergies (asthma and/or allergic rhinitis) have a lower expression of ACE211. Patients with ARPC1B deficiency often have allergic diseases; their Th2-biased response could help explain the milder presentation seen in our patient. Insights from protective mechanisms in children, with and without certain immune defects, could facilitate the identification of therapeutic targets.Lina Maria Castano-Jaramillo1, MDMarco Antonio Yamazaki-Nakashimada1, MDSelma Cecilia Scheffler Mendoza1, MD, MSJuan Carlos Bustamante-Ogando2, MD, MSSara Elva Espinosa-Padilla2, MD, PhDSaul O. Lugo Reyes2, MD, MS.From the (1) Clinical Immunology Service, and the (2) Immunodeficiencies Research Unit, at the National Institute of Pediatrics, Mexico City, Mexico.Conflict of interests: NoneEthical statement: The patient and his family gave written informed consent for the diagnostic procedures and for publication of the case report.KEY WORDS: Primary immune deficiency, inborn errors of immunity, combined immune deficiency, ARPC1B deficiency, actinopathy, children, COVID-19, SARS-Cov-2, allergy, pneumonia, sepsis.
We thank Dr Shah and colleagues for their interest, analysis of the presented data and comments related to our paper1. Circumferential PV isolation using 8mm tip catheter is still currently used in our institution for some patients due to economic reasons, so we can provide AF ablation for a portion of the population for whom there is no private insurance available, with adequate safety and results (recurrence rate in this series was 15.6% in a follow-up of 11±5 months)2. Those catheters have two temperature sensors, thus reducing the risk of clot formation on the tip of the catheter. For the same reason, our institutional standard when using such catheters is to deliver RF applications in temperature-controlled mode with maximum temperature of 55ºC. This mode of RF application is different compared to irrigated tip catheters and the mode of application used in the cited experimental study mentioned by the authors, in which it was used power-controlled RF applications.3 Due to the temperature-controlled mode of RF application, the cooling of esophagus generates a convective cooling of the atrial wall close to the esophagus and the catheter interface, leading to the higher power RF application that was observed in Group III.2 Probably due to this higher power of application, there was a higher rate of esophageal and periesophageal lesions injuries in the esophageal cooling group. This rate was however acceptable, since we used esophagogastroduodenoscopies (EGD) combined with radial endosonographies (EUS), that is a high sensitivity method of screening for esophageal lesion. Additionally, there were no severe or clinically significant lesions in any of the patients. A prior experimental model we performed some years ago also suggests this hypothesis.4 This model was similar to the one used by Montoya and cols3 and we could find deeper lesions with esophageal cooling and temperature-controlled applications, but similar depth, when power-controlled applications were performed.4 In silico models could also be used to evaluate the different effects of esophageal cooling using temperature or power-controlled RF applications. So, we think that the flow used in our studied balloon was not the reason for the findings, but the mode of application, although even in the esophageal cooling group the incidence of lesions was low. This was a prototype balloon used for the first time in clinical studies, and it was not possible to measure inflow and outflow temperature, being not possible to define heat transfer capacity. However, as presented before, as there was a higher RF power in group III we can infer that we achieved some cooling on the esophagus-atrium interface. Tsuchiya and cols showed a reduction in luminal esophageal temperature using an esophageal balloon with irrigation flow similar to our study.5We strongly agree with the authors that a higher flow of irrigation and consequential higher temperature reduction could be more protective, especially using power-controlled RF applications. Additionally, we think that esophageal cooling strategies are a promising strategy to avoid severe esophageal lesions, especially with contact sensor, power-controlled RF applications, allowing more effective atrial lesions close to the esophagus, thus improving AF ablation results. References 1. Shah S, Mercado Montoya M, Zagrodzky J, Kulstad E. Letter to the Editor regarding the paper "Comparative study of strategies to prevent esophageal and periesophageal injury during atrial fibrillation ablation". Journal of Cardiovascular Electrophysiology. 2020.2. de Oliveira BD, Oyama H, Hardy CA, et al. Comparative study of strategies to prevent esophageal and periesophageal injury during atrial fibrillation ablation. J Cardiovasc Electrophysiol. 2020;31(4):924-933.3. Montoya MM, Mickelsen S, Clark B, et al. Protecting the esophagus from thermal injury during radiofrequency ablation with an esophageal cooling device. J Atr Fibrillation. 2019;11(5):2110.4. Scanavacca MI, Neto S, Pisani CF, et al. Cooled intra-esophageal balloon to prevent thermal injury of esophageal wall during radiofrequency ablation. Heart rhythm. 2007;4(5):S117.5. Tsuchiya T, Ashikaga K, Nakagawa S, Hayashida K, Kugimiya H. Atrial fibrillation ablation with esophageal cooling with a cooled water-irrigated intraesophageal balloon: a pilot study. J Cardiovasc Electrophysiol. 2007;18(2):145-150.
Percutaneous atrial septal defect (ASD) closure is the mainstay treatment for ostium secundum ASD and patent foramen ovale1. Patients with ASD may develop atrial fibrillation (AF), mostly due to structural atrial remodeling creating the substrate for macroreentry2,3. Timing of ASD closure is crucial to prevent further development of electrophysiological heterogeneity, thereby reducing morbidity associated with AF, even though patients with ASD closure devices remain at high risk of developing AF4.The rising number of patients undergoing percutaneous ASD closure poses a new challenge in the treatment of coexistent AF. Furthermore, the reduction of surgical ASD treatment with concurrent cryo- or radiofrequency ablation (modified Maze procedure) is contributing to increase the number of patients who would benefit from catheter ablation after transcatheter ASD closure. Although some studies have shown a high acute success rate of catheter ablation in this population5, this treatment is often denied due the higher perceived risk of performing the transseptal puncture (TSP) after percutaneous repair of the defect.Given the lack of definitive data on this topic, in this issue of the Journal, Garg et al. performed the first meta-analysis evaluating the safety and the efficacy of catheter ablation for AF in this subset of high-risk patients with ASD closure devices.
In this issue of BJOG, Mendoza and colleagues report in an observational study the occurrence of a preeclampsia-like syndrome in six out of eight pregnant patients with novel coronavirus disease (COVID-19) who were admitted to the Intensive Care Unit (ICU) with severe pneumonia (Mendoza M, et al. BJOG 2020). There were no symptoms of preeclampsia amongst the 34 pregnant women who had mild forms of COVID-19. Importantly, the authors recorded not only routine laboratory test results, but also measured biophysical and biochemical markers that are typically altered in women with preeclampsia (uterine artery pulsatility index on Doppler ultrasound, serum soluble fms-like tyrosine kinase-1 [sFLT-1] and placental growth factor [PlGF]). Such markers were normal in five of the six cases, in whom the symptoms of preeclampsia resolved after improvement of the maternal clinical situation.The intriguingly high cumulative incidence of preeclampsia symptoms in women with severe coronavirus disease needs to be interpreted with caution due to the observational nature of the study, the small number of pregnant women with severe infection and the possible role of confounding factors. The normal biomarker results in most cases, nevertheless, suggest that severe coronavirus disease can lead to symptoms that mimic those of preeclampsia in the absence of defective placentation, which is further corroborated by the resolution of the symptoms without the delivery of the placenta when overall clinical improvement occurs. It is plausible that such manifestations are the result of widespread inflammation and endothelial damage, in a process that has been denominated “cytokine storm”, responsible for many of the symptoms of the coronavirus-related organ injury (Mehta P, et al. Lancet 2020;395:1033-34) This mechanism includes activation of inflammation pathways that convert arachidonic acid to prostaglandins, thromboxane and eicosanoids, ultimately provoking significant cytokine release. The cascade of events, however, does not appear to influence the levels of specific preeclampsia angiogenic and anti-angiogenic markers such as sFLT-1 and PlGF.A normal sFLT-1: PlGF ratio in women with clinically suspected preeclampsia can be reliably used predict the short-term absence of disease (Zeisler H, et al. N Engl J Med 2016;374:13-22). Although the definition of preeclampsia has changed over the last 20 years to incorporate less specific clinical features of end-organ damage, biomarkers will likely become part of the disease definition in the years to come or, at least, a valuable tool to select subgroups of women at higher risk of preeclampsia-related morbidity and mortality who require closer monitoring or immediate delivery.While larger cohorts derived from national datasets or international registries of coronavirus disease in pregnancy will be essential to confirm or refute this association, the preliminary data published in this study indicate that delivery during severe coronavirus disease should not be based on preeclampsia symptoms alone, particularly at early gestational ages, and that the use of ultrasound and serum biomarkers such as the sFLT-1: PlGF ratio might help to guide clinical management by distinguishing hypertension and endothelial dysfunction caused by COVID-19-related inflammation from true preeclampsia.No disclosures: A completed disclosure of interest form is available to view online as supporting information.
Under the unprecedented pressures of the global coronavirus disease 2019 (COVID-19) pandemic, there is an urgent requisite for successful strategies to safely deliver cardiac surgery. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was first described in early December 2019, and the rapid spread and emergence of this virus has caused significant disruptions in the delivery of healthcare services worldwide.1,2 In particular, provision of cardiac surgery has been disproportionally affected due to reallocation of intensive care resources, such as ventilators.2Additionally, patients with pre-existing cardiovascular disease are likely to have comorbidities which are associated with poorer clinical outcomes in confirmed SARS-CoV-2 cases.3,4 Despite this, Yandrapalli and colleagues have reported the first case of a successful coronary artery bypass graft (CABG) operation in a patient with asymptomatic SARS-CoV-2 infection, which offers insights into how cardiac surgery could be adapted to solve the challenges of this pandemic.5In response to the burden of COVID-19 on healthcare systems in the United Kingdom (UK), elective cardiac surgeries have been delayed owing to the redistribution of intensive care resources and the unquantifiable risk of acquiring COVID-19.2 Likewise, cardiac surgery services have undergone structural remodelling into a centralised system in an attempt to continue provisions of emergency surgery alongside hospital management of COVID-19 patients.2Unsurprisingly, most cardiac surgery units across the globe have seen a sharp decline in surgeries as a result, and one unit reported an 83% reduction in cardiac index cases between 23rd March to 4th May 2020.2 Similar models have been used in Europe to manage healthcare services and increase intensive care capacity. For example in the Lombardy region of Italy, 16 out of 20 cardiac surgical units discontinued services and all urgent cases have been consequently diverted to the remaining four units for centralised services.6 Whilst these measures have been beneficial for supporting the focused management of COVID-19 patients, it is important to reflect upon the future consequences of delayed elective cardiac surgery. Indeed, such patients are likely to have progressive conditions and further work is needed to investigate the long-term impact of COVID-19 on mortality and morbidity in this cohort.The case report by Yandrapalli and colleagues highlight the importance of routine SARS-CoV-2 testing for all patients requiring cardiac surgery, especially for detecting asymptomatic or subclinical infections.5 Active SARS-CoV-2 infection may precipitate an overproduction of early response proinflammatory cytokines in post-operative period, leading to unfavourable surgical outcomes.7,8 Moreover, preliminary studies have shown that patients with established cardiovascular diseases may have a greater risk of increased SARS-CoV-2 infection severity and prognosis.9 Taken together, assessment for active infection is crucial for risk stratification. In addition, clinicians should consider the threshold for surgery when selecting patients for cardiac surgery. An international, multi-centre cohort study by COVIDSurg Collaborative which included 1128 confirmed SARS-CoV-2 patients undergoing a broad range of surgeries revealed that 30-day mortality risk was significantly associated with the patient demographics of male sex, an age of 70 years or older, and poor preoperative physical health status.10 Collectively, the risks and benefits of cardiac surgery should be carefully considered in such patients due to higher mortality risk.10Alternative therapeutic procedures with rapid discharge, such as percutaneous intervention or medical therapy, may be more appropriate to reduce SARS-CoV-2 related mortality and nosocomial infection risk.11Current evidence is limited for postoperative outcomes in cardiac surgery cases. In the aforementioned cohort study by COVIDSurg Collaborative, the 30-day mortality rate was 23.8%.10In addition, the study reported that 51.2% of patients had postoperative pulmonary complications, which was associated with a higher mortality rate of 38.0%.10 In another case report describing an emergency CABG operation, the asymptomatic patient succumbed to pulmonary complications arising from a SARS-CoV-2 infection confirmed postoperatively.12 The authors acknowledge that the undiagnosed infection may have triggered a refractory pathological response after cardiac surgery. Indeed, recent literature has suggested that patients with SARS-CoV-2 are at higher risk of developing thromboembolisms, possibly mediated by the interaction with angiotensin-converting enzyme 2 (ACE2) receptors.13Similarly, there is a consensus that SARS-CoV-2 has direct adverse effects on the myocardium due to high expression of ACE2.14 As such, SARS-CoV-2 can potentially trigger multisystem complications which require vigilant monitoring, especially in patients requiring cardiopulmonary bypass and at high risk of developing thromboembolisms. Cardiac surgery patients represent a vulnerable patient population, and this cohort may experience worse outcomes with SARS-CoV-2 infection based on the current available evidence. In the latest recommendation, UK currently advises all patients who are listed for elective cardiac surgery to self-isolate for 14 days prior to surgery date, in a measure to limit and contain the exposure of such cohort to the smallest possibilities of acquiring COVID-19.Currently, the future of cardiac surgery after the pandemic is unclear as the evidence is still emerging. However, the lessons learnt from these unprecedented times can be taken forward to inform future service planning. Moving forwards, routine screening of patients for SARS-CoV-2 infection will undoubtedly play a key role in identifying asymptomatic or subclinical infections. The preoperative UK National Health Service testing recommendations should be broadened so that all patients undergoing cardiac surgery are screened, given the higher risk of postoperative complications in this population. Similarly, repeat testing is important for monitoring patients for concomitant infections. Alongside changes to hospital protocol, service delivery will inevitably shift. The successful application of telemedicine during the pandemic has already been reported in the delivery of oncology services.15 Moreover, the benefits of telecardiology outside of the COVID-19 era have been previously reported, and cardiology services will likely embrace the utilisation of telemedicine for managing outpatient consultations.16 Units will also have to address the vast backlog of surgeries caused by cancellation of elective cardiac operations in a sustainable manner, with adequate hospital space and personal protective equipment availability.17 In order to resume success services, planning for this eventuality should begin now and patients at significant mortality risk due to delayed surgery need to be prioritised.Ultimately, clear guidelines should be implemented to ensure safe resumption of surgical services, whilst also reassuring patients concerned about safety.3 Whilst the future trajectory of this pandemic is uncertain, the insights from the impact of COVID-19 on cardiac surgery will undoubtedly shape the future delivery of cardiac surgery.
Competence based education (CBE) has become increasingly popular as of late among health professions training programs. Models of CBE have been developed and implemented by major licensing bodies and professional Colleges (and affiliated education programs), such as the Royal College of Physicians and Surgeons of Canada (RCPSC) and the Association of American Medical Colleges (AAMC). In this month’s issue of theJournal of Evaluation in Clinical Practice , several studies describe an experience with designing and implementing a CBE model [1-6]. On the surface, the desire to ensure that health professions trainees are competent is commendable – just as it is likely few would argue that the care they receive should not be evidence based, I suspect that few (if any) would be comfortable with the clinician prescribing or managing said care being less than competent. However, the notion of a need for a CBE model might suggest that there was some issue with competence in the training of past clinicians that needed remedy, i.e. there is a lack of competence among some clinicians entering their profession that is in part a product of their training.When considering public perception of clinician competence, that of physicians in particular, history is not kind. Over the past two millennia, medicine got it wrong more often than right – humoral theory, bloodletting, miasma and contagion were all highly subscribed among the healthcare community but are now relics of a bygone era. In fact, the poor track record of medicine was the basis for a need for evidence based medicine (EBM). Practitioners of healthcare were often portrayed as ineffective, as providing little more than palliative support while nature took its course for better or worse, or even crueler, as charlatans peddling nostrums . Artists were no kinder to clinicians. For example, Shaw’s The Doctors Dilemma portrays a group of physicians as self-absorbed, greedy, overly confident in their unproven (and presumably, ineffective) pet “cures”, with only the poorest among them as having any sense of humility and patient centred focus in his practice . It seems that only within the last century did clinicians develop a good public reputation, much of which might be more appropriately attributed to improvements in hygiene practices/standards of living at a societal level (e.g. the McKeown Thesis) or likewise to medical science and the discovery of “silver bullet” cures (e.g. antibiotics, insulin, etc.) rather than to a change in how clinicians approached the learning of their craft and care of their patients. However, a historical lack of curative success and a poor reputation does not entail a lack of competence. A clinician working in Europe during the first millennium of the Common Era would have been considered competent provided he (or much rarer, she) mastered humoralism according to the teachings of Galen. Is it possible that future generations will look at the clinicians of today – even those who train under CBE – in the same light as we do clinicians of the past? Should a failure of today’s medicine in the eyes of future generations invalidate the competence of contemporary clinicians? I suppose the impact of the answer to such questions on the issue of competence hinges on how we define (and measure) competence.What then makes a clinician competent? The obvious answer is technical knowledge in the clinician’s given area. By that, I assume that for a clinician to be considered competent, she requires a minimum understanding of the content and technique of her given profession contemporaneous with the period of practice.11A minimum understanding of content and technique is required to be accepted into the profession, but it is not the desirable end. Clinicians are expected to participate in a model of lifelong learning with a goal of mastery over that content and technique. However, knowledge might be considered the minimum requirement. Several frameworks of competence outline additional requirements. For example, the “CanMEDS” framework, issued by the RCPSC, identifies “the abilities physicians require to effectively meet the health care needs of the people they serve”22http://www.royalcollege.ca/rcsite/canmeds/canmeds-framework-e, accessed on June 8, 2020., which includes the roles of the physician as medical expert, communicator, collaborator, leader, health advocate, scholar, and professional. The criteria by which these “roles” were selected (and other “roles” excluded), including the theoretical and/or empirical justification for their inclusion as part of “competence” is not clear to me, nor is it entirely clear if available metrics are sufficient in demonstrating that the roles have in fact been achieved by the trainee or how one operationalizes those roles in practice. Are clinicians who lack ability in any of these “roles” incompetent? Does meeting all of them according to some threshold ensure competence? As health professions are typically self-regulating, it is up to the governing bodies of each profession to decide, which might suggest that competence is a product of the times rather than akin to a “natural kind”33If it is the case that “competence” in medical practice, for example, is in fact a standard set by the profession (which I think most people would agree is the case), then judging the competence of past or future physicians by the current standard might be inappropriate. That raises issues about differing standards of competency for currently practicing physicians who trained at different times. In Canada, we are currently going through a transition whereby our training programs have two cohorts – one that is training under the previous “time based” model and the other under the current “competence by design” model. Is it the case that there is a relatively less (or no) guarantee that those training under the previous model are competent? The answer to that question might impact the extent to which we should have confidence in currently practicing clinicians, or at least relative to the next generation. Regardless, all practicing clinicians are accountable to a standard set by their respective professional Colleges, which likely makes the issue moot once trainees enter independent practice..It would be silly to suggest that clinicians who trained under a pre-CBE model are not competent any more than it is to suggest that clinical decisions prior to the adoption of the EBM movement were not based on evidence. Certainly, we have no shortage of competent clinicians practicing today. Those clinicians were accepted into the profession (and maintain standing) on some assessment of competence. However, institutions may have good reason for implementing CBE beyond simple competence concerns. For example, CBE programs can facilitate the development of an infrastructure of accountability that extends beyond activities of remediation or accelerating advancement to independent practice. That infrastructure can be leveraged to ensure transparency in assessment and advancement, identify individualized training needs, etc. that can be important components of ensuring and achieving equitable access to health professions, particularly for traditionally underrepresented populations.44It is important to note that while CBE might drive the development of such an infrastructure, a CBE training model is not necessary to do so. Certainly, institutions can and should be striving to improve on accountability, equitable access, etc., irrespective of a CBE model.On the other hand, we have no shortage of experience with poor decisions, suboptimal patient outcomes, iatrogenic effects, etc., that often raise concern about clinician ability (or competence). Poor outcomes, or at least those not aligned with the expectations of the public (irrespective of if those expectations are realistic) could be construed by some as a result of incompetence. Likewise, inequitable access (within or between communities) to appropriate expertise might raise concern of a lack of professional competence (i.e. the profession is not meeting the needs of the population inclusively). One way such perceptions by the public can be problematic for healthcare professions is that may erode the powerful position of institutionalized healthcare (and its providers) that exists in many societies. One could argue that EBM had a powerful effect on securing the public’s trust in healthcare by leveraging public perception of science as apolitical, objective, etc. Does CBE play a similar role by highlighting high professional standards only achievable by those “worthy” of the profession, who were rigorously assessed using quantifiable (often presented as “objective”) metrics, irrespective of whether that results in better care for patients? If so, then CBE may constitute a political move to retain or grow power rather than a remedial exercise to ensure no one joins the profession without having the skills necessary to provide appropriate care (by some defined public standard) for those seeking service. In other words, one might argue that CBE is not a response to a concern about competence – it is a response to a potential loss of standing relative to alternative modes of care or other social services. I am not suggesting that is necessarily the case, as I know several health professions educators who are honest in their pursuit to train clinicians who will excel in serving and providing care for the community. That seems to be the rule rather than the exception. However, the goals of the educators may not always tightly align with the goals of the institution, which may also be responsible for securing funding, maintaining status, public accountability, etc.Models of health professions training that focus on assessing and achieving defined competencies rather than hoping that important abilities are acquired over a defined time period (that also relies on the reliability and validity of licensing exams) are admirable. It is difficult to argue that achieving competence should not be the explicit focus of training. Perhaps one of the greatest benefits of CBE is that it puts competence to the forefront, just as EBM did for evidence. However, we must be vigilant to ensure that “competence” stays more than a buzzword or a tool of branding. Terms lacking substance can have a negative effect on patient care – too often the terms “patient centred” and “evidence based”, for example, are invoked as placeholders for quality patient care without any evidence to support that whatever intervention or program those terms are describing has any positive impact beyond rhetorical. We have not entered into an era of clinician competence simply because CBE has been implemented. Rather, what I see as the greatest benefit of CBE is the opportunity for improving and ensuring accountability.ReferencesRich J, Young SF, Donnelly C, et al. Competency-based education calls for programmatic assessment: But what does this look like in practice? Journal of Evaluation in Clinical Practice 2020;26(4):Hamza DM, Ross S, Oandasan I. Process and outcome evaluation of a CBME intervention guided by program theory. Journal of Evaluation in Clinical Practice 2020;26(4):Egan R, Chaplin T, Szulewski A, et al. A case for feedback and monitoring assessment in competency-based medical education. Journal of Evaluation in Clinical Practice 2020;26(4):Katoue MG, Schwinghammer TL. Competency-based education in pharmacy: A review of its development, applications, and challenges. Journal of Evaluation in Clinical Practice 2020;26(4):Crawford L, Cofie N, McEwen L, Dagnone D, Taylor SW. Perceptions and barriers to competency-based education in Canadian postgraduate medical education. Journal of Evaluation in Clinical Practice 2020;26(4):Railer J, Stockley D, Flynn L, Hastings-Truelove A, Hussain A. Using outcome harvesting: Assessing the efficacy of CBME Implementation. Journal of Evaluation in Clinical Practice 2020;26(4):Porter R. The Greatest Benefit to Mankind: A Medical History of Humanity. New York: W.W. Norton & Company; 1997.Shaw, B. The Doctor’s Dilemma: A Tragedy. London: Constable and Co.; 1922.McKeown T. The role of medicine: Dream, mirage or nemesis? Nuffield Trust; 1976. Available at: https://www.nuffieldtrust.org.uk/files/2017-01/1485273106_the-role-of-medicine-web-final.pdf. Accessed on June 8, 2020.
Retrieval of central venous catheters fragments often puts us in front of different situations. Having more techniques available for strategic planning of the procedure is imporatnt. The authors propose the simultaneous use of two different approaches for the recovery of a CVC fragment from the pulmonary artery.
Background Currently, the coronavirus disease 2019 (COVID-19) has become pandemic globally. 10-20% of the cases are severe and more than 397,000 deaths have occurred. The risk factors for the mortality of critically ill COVID-19 patients remain to be elucidated. Conclusions Survived severe and non-survived COVID-19 patients had distinct clinical and laboratory characteristics, which were separated by principle component analysis. Logistic regression revealed several risk factors such as elder age, greater affected lobe numbers and higher level of serum CRP for the mortality of severe COVID-19 patients. Longitudinal changes of laboratory findings indicate the advancement of the disease and may be helpful in predicting the progression of severe patients.
Infection with the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) induces the coronavirus infectious disease 19 (COVID-19). Its pandemic form in human population and its probable animal origin, along with recent case reports in pets, make drivers of emergence crucial in carnivore domestic pets, especially cats, dogs and ferrets. Few data are available in these species; we first listed forty-six possible drivers of emergence of COVID-19 in pets, regrouped in eight domains (i.e. pathogen/disease characteristics, spatial-temporal distance of outbreaks, ability to monitor, disease treatment and control, characteristics of pets, changes in climate conditions, wildlife interface, human activity, and economic and trade activities). Secondly, we developed a scoring system per driver, then elicited experts (N = 33) to: (i) allocate a score to each driver, (ii) weight the drivers scores within each domain and (iii) weight the different domains between them. Thirdly, an overall weighted score per driver was calculated; drivers were ranked in decreasing order. Fourthly, a regression tree analysis was used to group drivers with comparable likelihood to play a role in the emergence of COVID-19 in pets. Finally, the robustness of the expert elicitation was verified. Five drivers were ranked with the highest probability to play a key role in the emergence of COVID-19 in pets: availability and quality of diagnostic tools, human density close to pets, ability of preventive/control measures to avoid the disease introduction or spread in a country (except treatment, vaccination and reservoir(s) control), current species specificity of the disease causing agent and current knowledge on the pathogen. As scientific knowledge on the topic is scarce and still uncertain, expert elicitation of knowledge, in addition with clustering and sensitivity analyses, is of prime importance to prioritize future studies, starting from the top five drivers. The present methodology is applicable to other emerging pet diseases.
We present a late presentation of saddle pulmonary embolism and thrombus-in-transit straddle the patent foramen on patient who successfully recovered from severe acute respiratory syndrome coronavirus-2 (COVID-19) pneumonia. Seven days post-discharge (i.e. 28 days after initial COVID-19 symptom onset), she was readmitted to hospital for severe dyspnea. Computer tomography angiogram and echocardiography confirmed the diagnosis. Severe pro-inflammatory and pro-thrombotic states with endothelial involvement have been reported associated with severe COVID-19 infection. However the duration of hypercoagulable state has not yet known. This case highlights the risk of thromboembolic phenomena for prolonged periods of times after recovering from COVID-19 pneumonia.
Extracorporeal membrane oxygenation (ECMO) is a technology that has allowed for further cardiopulmonary support in the setting of respiratory failure refractory to mechanical ventilation. While it has evolved since its first description, one area of improvement continues to be its implementation. With advancements in cannulation techniques, in recent years, there has been a plethora of new cannulas that has been introduced to the market. For urgent venous-venous cannulation, the right internal jugular vein along with either femoral veins remain the most utilized strategy due to minimal need for imaging support. This allows for safe bedside cannulation. However, as the number of days of ECMO support continue to increase bridging patients to an easier to ambulate and more comfortable cannulation strategy is preferred. Therefore, we describe a method for bridging right jugular-femoral cannulation to left subclavian placement of the CrescentTM Dual Lumen Catheter without interrupting ECMO support.
Background and aims: Oral mucositis (OM) is common and distressing toxicity in children on chemotherapy. There is limited number of safe and effective therapeutic options available for OM. Ketamine oral rinse has shown promising results in few studies in adults. This randomized, double-blind placebo-controlled trial aimed to test the efficacy of ketamine mouthwash in reducing chemotherapy-induced severe OM pain in children. Methods: Children aged 8-18 years with severe OM were randomized to a single dose of ketamine mouthwash (4 mg/ml solution; dose 1 mg/kg) or a placebo. A sample size of 44 patients was determined. Pain score (6-point faces scale) was noted at baseline and 15, 30, 45, 60, 120, 180, and 240 min. The outcome variables were a reduction in pain score, need for rescue medications, and adverse events. Results: The baseline characteristics were comparable in the two groups. The mean OM pain at 60 min decreased by 1.64 points (CI 1.13-2.14) in the ketamine group and 1.32 points (CI 0.76-1.87) in the placebo group (p=0.425), with a group difference of 0.32 points. Rescue pain medication (at 60 min) was required in 13.6% in the ketamine group and 18.2% in the placebo group (p=1.000). There were no significant adverse events observed. Conclusions: Among children on cancer chemotherapy with severe OM, ketamine mouthwash at a dose of 1 mg/kg did not significantly reduce OM pain. It did not decrease the need for rescue pain medications. Further research is warranted to test higher doses of ketamine for a clinically significant effect.
The covid-19 pandemic has forced citizens worldwide to rely on social distancing measures as the main tools to prevent the rapid spreading of the virus (1). In pediatric oncology, there were important initial concerns for immunocompromised patients who were considered to be at higher risk of developing severe form of the disease (2,3). Consequently, potential challenges (2) have been identified and advice given by the principal child cancer organizations (3). Although more experience from countries that have been facing the pandemic are being published, results are inconsistent so far ranging from reassuring in Milano (4), Madrid (5) or New York (6) to worrying in France where 4 out of 33 Covid-19 positive patients required intensive care and 1 death at last follow up (7).Over the last weeks, despite the pandemic we were able to maintain “normal“ care for pediatric cancer patients in our institution, including high-dose chemotherapy followed by peripheral stem cells transplantations, or recruitment in early phase clinical trials. Only follow-up visits have been re-scheduled or switched to remote consultations. After almost 2 months of lock-down and still ongoing social distancing measures, an unexpected challenge has emerged. Inddeed, during that period, as usual we had to break bad news: for diagnosis, for relapse or palliative care. Initially, when breaking bad news, I had the feeling something was going wrong, or at least was not going as usual. Was I doing something wrong? Was stress induced by a high level of anxiety due to the lack of specific information on the real risk for adolescents/children with cancer both among the medical team and or parents affecting the “breaking bad news” process?Why didn’t I take that teenagers in my arms after disclosing her a metastatic relapse and she looked in such a distress?Social distancing!Masks to start with. They are of course a barrier to saliva droplets potentially containing covid-19, but most importantly they are also a barrier to adequately transmit and discriminate emotions just relying on eyes expression, looks…beyond tears. Silent communication with long looks without words can sometimes be enough and better that long talks but do parents and children feel the same when half of the face is covered. I asked about it to one of my patients and he answered“I think can read your eyes” . By increasing the physical space between people to avoid virus spreading, but here again, for physicians and some parents/patients, holding hands, holding shoulders, hugging are important non-verbal elements of communications and help showing compassion.We might break social distancing to break bad news, but if not pre-agreed by the patient or its parents, is it acceptable? Couldn’t it be perceived as an additional threat, contribute to alter intuitive communication which is characterized by broad, shared goals and mutual respect?Breaking bad news while trying to maintain social distancing is an unexpected new challenge associated with Covid-19. We will very likely learn to better communicate, read & share our respective emotions even with masks and physical distancing and sometimes allow ourselves exceptions to social distancing. Meanwhile, this impact shall be further evaluated among all stakeholders: patients, their parents, and physicians and adapted strategies to better cope with it developed.
The pandemic of the novel coronavirus disease, COVID-19 is having a serious impact on pediatric patients with cancer. Social distancing, self-quarantining and nationwide lockdown have resulted in restricted movements of patients and families across the country. This has made the optimum management of children with cancer difficult. In this clinical perspective, we discuss the issues related to COVID-19 and pediatric cancer and how we have attempted to optimize the treatment for our patients using telemedicine, reorganizing the day care services, triaging our patients and modifying their treatment plans, partnered with the NGOs and local medical centres to provide care to our patients.
Dear Editor,We read with great interest, the article titled “Evaluation of treatments for Bartholin’s cyst or abscess: A systematic review” by authors BJG Illingworth K Stocking M Showell E Kirk JMN Duffy Published in BJOG volume 127, issue 6, May 2020. The article was particularly relevant owing to the fact that Bartholin’s Abscess is a common gynaecological presentation which we encounter regularly in clinical practice.Whilst this article provided some insight into the various techniques for managing Bartholin’s Abscess around the world , there were a few points that we would like to raise. It was not unexpected that the meta-analysis finally concluded that no single technique was superior to the others. This may very well have been due to the different criteria used by these studies to assess the effectiveness of the procedure. Since the failure or success of any technique is largely based on the effect on the quality of life and perception of the outcome by the patient, it may have been more prudent to compare different surgical interventions in terms of patient acceptance, overall satisfaction and long term outcome. Also this analysis compiled data from countries where health services are provided by the private and /or the government sector where the financial incentives for performing procedures vary widely  . The article does not elaborate on the proportion of patients who had received pharmacological treatment for varying durations prior to these surgical interventions, which undoubtedly may have influenced the outcome. The analysis also does not include any study where marsupialization was performed under local anaesthetic which may be equally if not more effective than the word catheter at equal cost. In the light of current pandemic situation that the whole world is facing, expertise into minimally more invasive gynaecological procedures to be performed in office setting would also be a key point of consideration. We look forward to the authors comments on these factors.Piyushi Sharma,1 Thangamma Katimada-Annaiah,1 Montasser Mahran,1 Dilip Patil,1 Elvyna Lim,1 Joseph Nattey,1 Tarley Davies1Bedford Hospital NHS TrustReferences1. Illingworth BJG, Stocking K, Showell M, Kirk E, Duffy JMN. Evaluation of treatments for Batholin’s Cyst or abscess: A systematic Review. BJOG 2020;127:671-678
A growing body of literature has documented myriad effects of human activities on animal behavior, yet the ultimate ecological consequences of these behavioral shifts remain largely uninvestigated. While it is understood that, in the absence of humans, variation in animal behavior can have cascading effects on species interactions, community structure, and ecosystem function, we know little about whether the type or magnitude of human-induced behavioral shifts translate into meaningful ecological change. Here we synthesize empirical literature and theory to create a novel framework for examining the range of behaviorally mediated pathways through which human activities may affect different ecosystem functions. We highlight the few empirical studies that show the potential realization of some of these pathways, but also identify numerous factors that can dampen or prevent ultimate ecosystem consequences. Without a deeper understanding of these pathways, we risk wasting valuable resources on mitigating behavioral effects with little ecological relevance, or conversely mismanaging situations in which behavioral effects do drive ecosystem change. The framework presented here can be used to anticipate the nature and likelihood of ecological outcomes and prioritize management among widespread human-induced behavioral shifts, while also suggesting key priorities for future research linking humans, animal behavior, and ecology.
Background: We report our experience in aortic arch repair with the E-vita Open hybrid prosthesis and describe the changes in our technique over time. Methods: Between October 2013 and December 2019, 56 patients underwent a total aortic arch replacement with the E-vita Open hybrid prosthesis. Main indications were thoracic aorta aneurysm (n=27) and acute type A aortic dissection (n=18). We analyze the technique and results in the overall series, and compare both between our early (Group I, 25 patients) and late experience (Group II, 31 patients). Results: Overall in-hospital mortality was 7.1% (4), and permanent stroke and spinal cord injury were 3.6% and 1.8% respectively. 15 patients (26.8%) underwent a planned second procedure on the distal aorta: 13 endovascular, 1 open and 1 hybrid. Survival at 1 and 3 years was 90.7% and 80.7%. Group II included more patients with acute dissection (45.2% vs 16%, p=0.02), a higher rate of bilateral cerebral perfusion (100% vs 64%, p<0.001), left subclavian artery perfusion during lower body circulatory arrest (87.1% vs 0%, p<0.001), early reperfusion (96.8% vs 40%, p<0.001), and zone 0-2 distal anastomosis (100% vs 72%, p=0.02). In-hospital mortality (3.2% vs 12%) and permanent stroke (0% vs 8%) tended to be lower in Group II. Conclusions: Total arch replacement with E-vita Open hybrid prosthesis in complex thoracic aorta disease is safe. One-stage treatment is feasible when pathology does not extend beyond the proximal descending thoracic aorta. In any case, it facilitates subsequent procedures on distal aorta if needed.