A circumpolar study unveils a positive non-linear effect of temperature on arctic arthropod availability that may reduce the risk of warming-induced trophic mismatch for breeding shorebirds.

Seasonally abundant arthropods are a crucial food source for many migratory birds that breed in the Arctic. In cold environments, the growth and emergence of arthropods are particularly tied to temperature. Thus, the phenology of arthropods is anticipated to undergo a rapid change in response to a warming climate, potentially leading to a trophic mismatch between migratory insectivorous birds and their prey. Using data from 19 sites spanning a wide temperature gradient from the Subarctic to the High Arctic, we investigated the effects of temperature on the phenology and biomass of arthropods available to shorebirds during their short breeding season at high latitudes. We hypothesized that prolonged exposure to warmer summer temperatures would generate earlier peaks in arthropod biomass, as well as higher peak and seasonal biomass. Across the temperature gradient encompassed by our study sites (>10°C in average summer temperatures), we found a 3-day shift in average peak date for every increment of 80 cumulative thawing degree-days. Interestingly, we found a linear relationship between temperature and arthropod biomass only below temperature thresholds. Higher temperatures were associated with higher peak and seasonal biomass below 106 and 177 cumulative thawing degree-days, respectively, between June 5 and July 15. Beyond these thresholds, no relationship was observed between temperature and arthropod biomass. Our results suggest that prolonged exposure to elevated temperatures can positively influence prey availability for some arctic birds. This positive effect could, in part, stem from changes in arthropod assemblages and may reduce the risk of trophic mismatch.

Why do avian responses to change in Arctic green-up vary?

Global climate change has altered the timing of seasonal events (i.e., phenology) for a diverse range of biota. Within and among species, however, the degree to which alterations in phenology match climate variability differ substantially. To better understand factors driving these differences, we evaluated variation in timing of nesting of eight Arctic-breeding shorebird species at 18 sites over a 23-year period. We used the Normalized Difference Vegetation Index as a proxy to determine the start of spring (SOS) growing season and quantified relationships between SOS and nest initiation dates as a measure of phenological responsiveness. Among species, we tested four life history traits (migration distance, seasonal timing of breeding, female body mass, expected female reproductive effort) as species-level predictors of responsiveness. For one species (Semipalmated Sandpiper), we also evaluated whether responsiveness varied across sites. Although no species in our study completely tracked annual variation in SOS, phenological responses were strongest for Western Sandpipers, Pectoral Sandpipers, and Red Phalaropes. Migration distance was the strongest additional predictor of responsiveness, with longer-distance migrant species generally tracking variation in SOS more closely than species that migrate shorter distances. Semipalmated Sandpipers are a widely distributed species, but adjustments in timing of nesting relative to variability in SOS did not vary across sites, suggesting that different breeding populations of this species were equally responsive to climate cues despite differing migration strategies. Our results unexpectedly show that long-distance migrants are more sensitive to local environmental conditions, which may help them to adapt to ongoing changes in climate.

Oral Immunotherapy With Human Secretory Immunoglobulin A Improves Survival in the Hamster Model of Clostridioides difficile Infection.

Coadministration of human secretory IgA (sIgA) together with subtherapeutic vancomycin enhanced survival in the Clostridioides difficile infection (CDI) hamster model. Vancomycin (5 or 10 mg/kg × 5 days) plus healthy donor plasma sIgA/monomeric IgA (TID × 21 days) or hyperimmune sIgA/monomeric IgA (BID × 13 days) enhanced survival. Survival was improved compared to vancomycin alone, P = .018 and .039 by log-rank Mantel-Cox, for healthy and hyperimmune sIgA, respectively. Passive immunization with sIgA (recombinant human secretory component plus IgA dimer/polymer from pooled human plasma) can be administered orally and prevents death in a partially treated CDI hamster model.

The clinical and genetic features of hereditary haemorrhagic telangiectasia (HHT) in central South Africa-three novel pathogenic variants.

Hereditary haemorrhagic telangiectasia (HHT) is supposedly rare in Africa, with only three pathogenic variants documented to date. We describe the clinical and genetic features of HHT patients in central South Africa, who fulfilled the Curaçao criteria. Sixteen patients (median age 38.5 years, range 12-65 years), from six families were included. Fifteen patients were of African descent and one was of Afrikaner descent. The mean epistaxis severity score was 3.18, and the median haemoglobin was 9.5 g/dL (range 3.5-13.5 g/dL). On transthoracic contrast echocardiography 69% had a shunt grade ≥ 1, but only 20% had pulmonary arteriovenous malformations (AVMs) on computed tomography of the chest. Hepatic AVMs were found in 13% of patients, while 13% had brain vascular malformations. Four patients were HIV positive, of whom two had worsening epistaxis while they had opportunistic infections and poor HIV control. We identified six pathogenic variants (four in ENG and two in ACVRL1) in the six probands, three of which had been described previously. Three variants have apparently not been reported previously: ENG c.[1336_1337dup];[ =] p.[(Asp446fs)];[( =)], ENG c.[ 690?_816+?del] p.[(?)], and ACVRL1 c.[268_274delins57];[ =] p.[(Cys90fs)];[( =)]. We confirmed the diagnosis of HHT in sixteen patients and identified pathogenic variants in ENG or ACVRL1 in all six probands in central South Africa, where HHT has been underreported. We describe three pathogenic variants: two of ENG and one of ACVRL1. We will be able to implement pre-symptomatic screening of patients in our area, and improve their management.

Providing a framework of principles for conceptualising the Fontan circulation.

The Fontan operation remains the final palliation for thousands of patients with complex congenital heart disease. By creating a Fontan circuit, control of cardiac output and congestion is wrested away from the ventricle and new haemodynamic forces take control. Understanding how to control the flow in the Fontan circuit will enable clinicians to improve patient management and possibly prevent future complications. CONCLUSION: This review proposes a framework of principles to conceptualise the functionality and limitations of a Fontan circulation.

Percutaneous obliteration of the right ventricle to avoid coronary damage by sinusoids in patients with pulmonary atresia intact ventricular septum during staged single ventricle palliation.

BACKGROUND AND AIMS: Suprasystemic pressure waves can damage the coronary arteries resulting in myocardial ischemia and excess early mortality. We aimed to reduce the coronary pressure wave through the sinusoids by abolishing RV volume with percutaneous devices. METHODS AND RESULTS: Four patients with PA-IVS and coronary sinusoids from the hypertensive rudimentary RV were evaluated at a median age 26.6 months (range: 2.7-51.7). Right ventricle coronary dependent flow to the left ventricular myocardium was excluded. All four patients had dual perfusion with competitive flow from the RV through the sinusoids to the coronary arteries. Devices used were: Amplatzer vascular plug II of 10-16 mm; 27 coils (diameter 5-15 mm) in the oldest patient. Right ventricular angiography after cavity obliteration showed no more significant coronary perfusion through the sinusoids. There were no complications or deaths. Only minor and transient changes in the levels of troponin were observed. Coronary angiography at pre-Fontan evaluation showed no progress of coronary abnormalities in two patients. CONCLUSION: In selected patients with functionally single left ventricle, obliteration of the hypertensive RV cavity by percutaneous devices is safe and abolishes the systolic pressure wave in coronary sinusoids. When performed early, this may halt coronary damage and avoid excess mortality.

Percutaneous embolization of lymphatic fistulae as treatment for protein-losing enteropathy and plastic bronchitis in patients with failing Fontan circulation.

BACKGROUND: To determine the feasibility and clinical result of selective embolization of hepatoduodenal or paratracheal lymphatics in Fontan patients with protein-losing enteropathy (PLE) or plastic bronchitis (PB). METHODS: Dilated lymph vessels in periportal (PLE) or paratracheal (PB) position were percutaneously punctured with a 22G Chiba needle. Intralymphatic position was confirmed by water soluble contrast injection with drainage to hepatoduodenal or tracheal fistulae. After flushing with 10% glucose solution, occlusion of hepatoduodenal or paratreacheal lymphatics was effected by injection of 1-4 cc mixture 4/1 of Lipiodol/n-butyl cyanoacrylate (n-BCA; Histoacryl). RESULTS: Seven patients with proven PLE were treated with periportal lymphatic embolization 10.7 (range: 6.6-13.5) years after the Fontan operation. The Fontan operation was performed at a median age of 3.7 (range: 2.9-5.7) years and PLE started a median of 3.1 (range: 0.9-4.7) years later. Five patients required a second procedure 2-8 months later. Complications were limited (spillage of glue in portal branch, transient cholangitis, and caustic duodenal bleeding). Six of seven patients reported significant improvement in quality of life and normalization of albumin levels after limited follow-up (p < .01). One patient (Fontan at 2.9 years; age 16.4 years) had PB for 2 years. Selective transthoracic cone-beam-directed puncture of left and right paratracheal lymphatics with n-BCA embolization of distal lymphatic fistulae resulted in lasting absence of tracheal casts (11 months). CONCLUSIONS: Embolization of periportal/peritracheal lymphatics is a promising technique in Fontan patients with PLE/PB. Larger series are required to determine incidence and reasons of success/failure, with long-term results and effects on liver function.

Salvage high-intensity focused ultrasound (HIFU) for locally recurrent prostate cancer after failed radiation therapy: Multi-institutional analysis of 418 patients.

OBJECTIVE: To report the oncological outcome of salvage high-intensity focused ultrasound (S-HIFU) for locally recurrent prostate cancer after external beam radiotherapy (EBRT) from a multicentre database. PATIENTS AND METHODS: This retrospective study comprises patients from nine centres with local recurrent disease after EBRT treated with S-HIFU from 1995 to 2009. The biochemical failure-free survival (bFFS) rate was based on the 'Phoenix' definition (PSA nadir + 2 ng/mL). Secondary endpoints included progression to metastasis and cancer-specific death. Kaplan-Meier analysis was performed examining overall (OS), cancer-specific (CSS) and metastasis-free survival (MFS). Adverse events and quality of life status are reported. RESULTS: In all, 418 patients with a mean (SD) follow-up of 3.5 (2.5) years were included. The mean (SD) age was 68.6 (5.8) years and the PSA level before S-HIFU was 6.8 (7.8) ng/mL. The median PSA nadir after S-HIFU was 0.19 ng/mL. The OS, CSS and MFS rates at 7 years were 72%, 82% and 81%, respectively. At 5 years the bFFS rate was 58%, 51% and 36% for pre-EBRT low-, intermediate- and high-risk patients, respectively. The 5-year bFFS rate was 67%, 42% and 22% for pre-S-HIFU PSA level ≤4, 4-10 and ≥10 ng/mL, respectively. Complication rates decreased after the introduction of specific post-RT parameters: incontinence (grade II or III) from 32% to 19% (P = 0.002); bladder outlet obstruction or stenosis from 30% to 15% (P = 0.003); recto-urethral fistula decreased from 9% to 0.6% (P < 0.001). Study limitations include being a retrospective analysis from a registry with no control group. CONCLUSION: S-HIFU for locally recurrent prostate cancer after failed EBRT is associated with 7-year CSS and MFS rates of >80% at a price of significant morbidity. S-HIFU should be initiated early following EBRT failure.

Radiofrequency perforation of the pulmonary valve: an efficient low cost solution.

Objective The aim of the study was to assess the feasibility of using commonly available catheterization laboratory equipment for radiofrequency perforation of the pulmonary valve in patients with pulmonary atresia and intact ventricular septum. Methods The system (off-label use for all items) is made up of a co-axial telescopic arrangement consisting of a 0.014" PT 2 ™ coronary guidewire, for insulation inside a 2.7-F microcatheter which has an inner lumen of 0.021". The microcatheter was passed via a standard 4-F right coronary catheter to just below the atretic pulmonary valve. Radiofrequency (RF) energy was delivered using a standard electrosurgical system. In vitro testing had been performed and indicated that 5-10 W for 2-5 s would be sufficient for valve perforation. Results Radiofrequency perforation was successfully performed in all (n = 5, 100%) patients at a median age of 3 days (range: 1-36) and weight 2.7 kg (range 2.3-3.0). In one patient the pericardium was entered during the initial attempt; the generator was put on coagulation mode during retrieval of the guidewire and no haemopericardium occurred. The pulmonary valve was dilated in all; in three patients (n = 3) the ductus arteriosus was stented during the same session. Conclusion Results of the study show that it is feasible to perforate the pulmonary valve safely using this system. Availability, simplicity and cost are noteworthy benefits.

Dysfunction of the foetal arterial duct results in a wide spectrum of cardiovascular pathology.

OBJECTIVE: Foetal ductal problems may have various cardiopulmonary consequences. This study aimed to identify the spectrum of ductus arteriosus (DA) dysfunction (closure, constriction, kinking, aneurysm and thrombosis) and the resultant clinical and echocardiographic presentation in foetuses and neonates. METHODS AND RESULTS: This is a retrospective analysis of serial pre- and post-natal data of 27 cases of foetal ductal dysfunction diagnosed at a median gestational age of 33 weeks (range 20-39). The most common abnormalities observed were premature closure of the DA in 56% (15/27) and constriction in 29% (8/27). Right ventricular hypertrophy was present in 75% (n = 11/15) of foetuses with premature DA closure, while ventricular dilation (4/7, 57%) was a more common feature in foetuses with ductal constriction. After birth, 63% (17/27) of new borns presented with cyanosis and pulmonary hypertension that required active treatment. Three infants died after birth. Abnormalities resolved spontaneously after birth in about 50% of patients. In some children, pulmonary valve stenosis and regurgitation was progressive and required further treatment. CONCLUSIONS: An abnormal right heart on foetal four-chamber ultrasound view should alert the sonographer to the possible presence of foetal ductal dysfunction. Ductal occlusion, transient or fixed constriction, kinking and aneurysm formation are associated with foetal cardiopulmonary sequelae. Symptoms and pathology is probably related to the type, foetal age, rapidity of progression and duration of intrauterine ductal dysfunction. Correspondingly, clinical outcomes vary ranging from little or no symptoms to severe respiratory distress and even foetal or neonatal death.

Percutaneous intervention for central shunts: new routes, new strategies.

Introduction In traditional locations, the standard Blalock-Taussig shunt presents numerous technical difficulties for percutaneous intervention. We changed our strategy to a central type shunt (Laks-type) with end-to-side pulmonary and side-to-side aortic anastomosis. The aim of this study was to determine whether this modified strategy would allow easier percutaneous manipulation in patients with small pulmonary arteries. Methods All children with a stretchable central vascular graft who required any form of percutaneous intervention were prospectively enrolled in the study. Results Eleven infants were evaluated a median time of 3 months (range 0.9-4.4) following initial shunt placement; the median weight at intervention was 5.7 kg (range: 4.0 - 10.0). All shunts (100%) were easily and swiftly entered without the need for special catheters or co-axial systems. In four patients other interventions in distal pulmonary arteries were first performed: cutting balloon treatment in three and balloon angioplasty of peripheral pulmonary artery stenosis in one. The shunts were then augmented with a stent with a diameter increasing from 3.5 ± 0.4 mm to 4.7 ± 0.8 mm and saturation increasing from 76% (range: 69-88) to 84% (range: 77-88) (P < 0.05). Several months later, two children required further interventions that could easily be performed via the stented shunts. No complications were observed. Conclusions The Laks-type shunt provides easy access for percutaneous procedures of the distal pulmonary arteries including cutting balloons; this shunt can predictably be expanded to augment pulmonary flow. This study highlights how co-operation between the interventionalist and the surgeon can improve strategies to manage these difficult patients.

Reversing left bronchus obstruction by compression of a pulmonary artery stent.

We report a case where endovascular stenting as part of the treatment of complex cardiovascular disease led to airway compression. Using a novel technique, this was successfully reversed. Simultaneous inflations of two balloons-one in the obstructed bronchus and one in the aorta combined with external compression of the chest-resulted in compression the stent. This report illustrates that by thinking "out of the box" and bearing in mind spatial relationships inside the chest, it is possible to diminish the anterior-posterior diameters of a stent. This intervention proved to be successful with relief of the left bronchial compression and improvement of chronic airway infection. © 2016 Wiley Periodicals, Inc.

Cracking a tricuspid perimount bioprosthesis to optimize a second transcatheter sapien valve-in-valve placement.

Bioprosthetic valves degenerate over time. Transcatheter valve-in-valve procedures have become an attractive alternative to surgery. However, every valve increasingly diminishes the diameter of the valvar orifice. We report a 12-year-old female who had a previous transcatheter tricuspid valve-in-valve procedure; cracking the ring of a Carpentier Edwards Perimount valve by means of an ultrahigh pressure balloon allowed implantation of a further larger percutaneous valve. The advantage of this novel approach permits enlarging the inner valve diameter and may facilitate future interventions and prolong time to surgery. © 2016 Wiley Periodicals, Inc.

Can a volume challenge pinpoint the limiting factor in a Fontan circulation?

OBJECTIVE: It is difficult to indicate whether the limitation in a failing Fontan circulation lies within the pulmonary vasculature or the heart. Such differentiation is crucial to direct adequate therapy. This study was set out to determine if a volume challenge could identify the limiting factor. METHODS AND STUDY POPULATION: Thirty-two catheterizations in 28 patients with a Fontan circulation were included. Pressures and oxygen saturations were measured before and after volume challenge (NaCl 0.9%; 15 cc/Kg). The changes in data were grouped based on the location of the major pressure increase. Ventricular function was measured in the resting state. RESULTS: The majority of the patients showed an increase in aortic oxygen saturation, mixed venous oxygen saturation, systolic, pulmonary and systemic venous pressures. The arterio-venous oxygen gradient decreased, suggesting an increase in cardiac output. Different patterns in pressure changes were observed. Most (n=17) showed a similar increase of ventricular end-diastolic pressure and mean venous pressure (MVP); some (n=7) showed a lower increase of MVP, suggesting pulmonary reserve and recruitment; others (n=8) showed a significant higher increase in MVP, suggesting increased pulmonary vascular resistance. All volume challenge was well tolerated. CONCLUSION: Most patients were preload-responsive. The pressure changes following volume load showed patterns with a potential of differentiating between patients with a major pulmonary or cardiac limiting factor.

Art Therapy for Paediatric Pain: A Scoping Review.

Pain is common in paediatric populations and is best treated with a multi-disciplinary approach. Art therapy interventions are gaining popularity in paediatrics; however, there is limited evidence on its impact on pain outcomes in children and adolescents. The objective of this scoping review is to map current research on art therapy's impact as an intervention in paediatric populations experiencing any type of pain (i.e., acute, recurrent, and chronic). Electronic searches were conducted by a medical librarian to identify studies that used art therapy interventions in paediatric populations with pain as an outcome measure. Four reviewers independently screened and selected articles for extraction using Covidence and data were extracted from articles using study objectives. There were five studies that met the inclusion criteria. Four of the five studies reported on pain intensity and all studies reported on emotional functioning. Findings suggest art therapy interventions can be helpful for reducing pain, anxiety, stress, and fear associated with treatment. Further, there is emerging evidence that art therapy can support the management of acute and procedural pain in children. Future research should examine the impacts of integrating art therapy interventions into the multidisciplinary management of paediatric pain.

Delivering stents in congenital heart disease using the double-wire technique: technical considerations.

OBJECTIVES: To evaluate the use of a double-wire technique to deliver stents. BACKGROUND: Placement of a stent in lesions close to bifurcations or without an anatomic obstruction can be problematic. PATIENTS AND METHODS: Stents were implanted in 12 patients between August 2010 and August 2012. Indications were complex anatomic obstruction in nine, external compression of pulmonary veins in two, and exclusion of an aortic aneurysm in one patient. RESULTS: Median age and body weight of the group were 11.6 years (range: 1.6-34.8) and 36 kg (range: 10-78), respectively. All stents were delivered safely and the patency of all side vessels was maintained; the distal end of the stent was flared to a median ratio of 1.4 (range: 1.2-1.8). The clinical objective was met in all patients: in lesions with stenosis, diameter increased from 5.9 mm (range: 1-13) to 9.9 mm (range: 8-17) [P < 0.01; 95% confidence interval (CI): 2.0-7.2]; oxygen saturations improved in the 2 patients after relief of the external pulmonary venous compression and in another an aortic aneurysm was excluded. One patient developed a hemothorax and one required blood transfusion for bleeding from the valve of the sheath. CONCLUSIONS: The double-wire stenting technique is effective to accurately deliver and anchor stents into lesions close to side branches and bifurcations. Side branch patency is maintained; the clinical objective (gradient relief, aneurysm exclusion, relief of external compression) can safely be reached. Technique and balloon selection should be based on the underlying anatomical substrate.

Elicitation of domain knowledge for a machine learning model for paediatric critical illness in South Africa.

Objectives: Delays in identification, resuscitation and referral have been identified as a preventable cause of avoidable severity of illness and mortality in South African children. To address this problem, a machine learning model to predict a compound outcome of death prior to discharge from hospital and/or admission to the PICU was developed. A key aspect of developing machine learning models is the integration of human knowledge in their development. The objective of this study is to describe how this domain knowledge was elicited, including the use of a documented literature search and Delphi procedure. Design: A prospective mixed methodology development study was conducted that included qualitative aspects in the elicitation of domain knowledge, together with descriptive and analytical quantitative and machine learning methodologies. Setting: A single centre tertiary hospital providing acute paediatric services. Participants: Three paediatric intensivists, six specialist paediatricians and three specialist anaesthesiologists. Interventions: None. Measurements and main results: The literature search identified 154 full-text articles reporting risk factors for mortality in hospitalised children. These factors were most commonly features of specific organ dysfunction. 89 of these publications studied children in lower- and middle-income countries. The Delphi procedure included 12 expert participants and was conducted over 3 rounds. Respondents identified a need to achieve a compromise between model performance, comprehensiveness and veracity and practicality of use. Participants achieved consensus on a range of clinical features associated with severe illness in children. No special investigations were considered for inclusion in the model except point-of-care capillary blood glucose testing. The results were integrated by the researcher and a final list of features was compiled. Conclusion: The elicitation of domain knowledge is important in effective machine learning applications. The documentation of this process enhances rigour in such models and should be reported in publications. A documented literature search, Delphi procedure and the integration of the domain knowledge of the researchers contributed to problem specification and selection of features prior to feature engineering, pre-processing and model development.

Don't judge a book by its cover: Exploring low self-reported distress and repressive coping in a pediatric chronic pain population.

Repression has been linked to greater illness, somatic symptoms, and poorer physical health, both in adult and pediatric populations. The current study examined psychological and pain profiles of children with chronic pain who may under-report levels of psychological distress at a first interdisciplinary chronic pain assessment. Children and their caregiver completed measures of psychopathology and pain intensity, while clinicians rated their levels of disability. Based on self-report measures, children were classified as "repressors" (low anxiety/high social desirability) or as "true low anxious" (low anxiety/low social desirability). Groups were then compared on psychological and pain characteristics. Compared to children with true low anxiety, repressors reported lower levels of depressive and somatic symptoms but provided higher ratings on pain intensity, pain-unpleasantness, and self-oriented perfectionism. Caregivers of repressors rated their children as having higher levels of adaptability compared to caregivers of children in the true low anxious group. Groups did not differ on clinician-rated level of disability. Children classified as repressors exhibited different profiles than children classified as having true low anxiety on both psychological outcomes and pain characteristics. Repression may be an important factor to consider for those assessing and treating children with chronic pain.

Treatment strategies for pulmonary sequestration in childhood: resection, embolization, observation?

BACKGROUND: The ideal treatment strategy for pulmonary sequestration whether resection or embolization in childhood is not clearly defined. Our institution has no clear policy, therefore both therapies are performed. OBJECTIVE: The aim of this study was to assess local management strategies of children presenting with pulmonary sequestrations. METHODS: This is a retrospective, single-institutional review. The main inclusion criterion was the established diagnosis of a pulmonary sequestration. Forty-eight patients were divided into three groups based on treatment received: conservative management (n = 5), surgery (n = 22) and embolization (n = 21). RESULTS: The median age at treatment was 8.0 months (range 1.2- 166.0) in the surgical, 4.0 months (range 0.2 - 166.0) in the embolization and 8 months (range 0.3 - 197.0) in the conservatively managed groups, respectively. Age at treatment was similar in the surgical and embolization groups (P = 0.9). Recurrent chest infections were the most common clinical presentation in the surgical group, whilst cardiac failure was the most frequent symptom in the embolization group (P < 0.01; 95% CI: 0.3 to 0.9). There were six complications in the surgical group and one in the embolization group (P = 0.1). In one patient embolization was not possible. Outcomes in both groups were comparable with good results on follow-up. CONCLUSION: Both surgery and endovascular embolization are effective and safe treatments for pulmonary sequestration. The presenting symptoms dictate therapy: surgery if there is infection and embolization if a shunt needs to be abolished. Our institutional policy remains unchanged.