Endoscopic management of lateral sphenoid cerebrospinal fluid leaks: Identifying a radiographic parameter for surgical planning.

Objective: Repair of cerebrospinal fluid (CSF) leaks of the lateral recess of the sphenoid (LRS) sinus can be challenging to accomplish via an endoscopic transphenoidal approach. The endoscopic transpterygoid approach can improve surgical access to the lateral recess but requires more extensive surgical dissection. We review our experience with LRS CSF leak repair via both techniques to determine whether preoperative radiologic data can help predict the most appropriate surgical approach.

Atrioventricular Valve Regurgitation in Single Ventricle Heart Disease: A Common Problem Associated With Progressive Deterioration and Mortality.

The Fontan procedure has provided patients with single ventricle physiology extended survival into adulthood and in many cases has improved their quality of life. Atrioventricular valve regurgitation (AVVR) is common in single ventricle patients and is associated with increased risk of mortality. AVVR is more common in patients with a systemic tricuspid or common atrioventricular valve but is generally progressive irrespective of underlying valve morphology.

Angiotensin-Converting Enzyme Inhibitor Prescription for Patients With Single Ventricle Physiology Enrolled in the NPC-QIC Registry.

Background The routine use of angiotensin-converting enzyme inhibitors (ACEI) during palliation of hypoplastic left heart syndrome is controversial. We sought to describe ACEI prescription in the interstage between stage 1 palliation (stage I Norwood procedure) discharge and stage 2 palliation (stage II superior cavopulmonary anastomosis procedure) admission using the NPC-QIC (National Pediatric Cardiology Quality Improvement Collaborative) registry. Methods and Results Analysis of all patients (n=2180) enrolled in NPC-QIC from 2008 to 2016 included preoperative anatomy, risk factors, and echocardiographic data.

Hospital readmissions after pulmonary resection: post-discharge nursing telephone assessment identifies high risk patients.

Background: We previously reported that post-discharge nursing telephone assessments identified a frequent number of patient complaints. Our aim was to determine if telephone assessments can identify patients at risk for emergency room (ER) visits or hospital readmissions.

Methods: A single-institution, retrospective review was performed on all patients undergoing pulmonary resection over a 12-month period.

Childhood/Adolescent Smoking and Adult Smoking and Cessation: The International Childhood Cardiovascular Cohort (i3C) Consortium.

Background Despite declining US adolescent smoking prevalence from 40% among 12th graders in 1995 to around 10% in 2018, adolescent smoking is still a significant problem. Using the International Childhood Cardiovascular Cohort (i3C) Consortium, which includes 7 international cohorts recruited in childhood and followed into adulthood, the present study was designed to confirm the important relation between adolescent smoking and daily adult smoking and present new data on adult smoking into the forties and comparison of smoking in the United States, Finland, and Australia. Methods and Results Childhood smoking experience during ages 6 to 19 in the 1970s and 1980s was classifiable in 6687 i3C participants who also provided smoking status in their twenties and forties through 2011-2018.

Health-Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta-Analysis.

Background People with a Fontan circulation experience a range of physical, psychosocial and neurodevelopmental challenges alongside, or caused by, their cardiac condition, with significant consequences for health-related quality of life (HRQOL). We meta-analyzed HRQOL outcomes reported by people with a Fontan circulation or their proxies and evaluated predictors of poorer HRQOL. Methods and Results Six electronic databases were searched for peer-reviewed, English-language articles published before March 2019.

Organoid Medicine in Hepatology.

Answer questions and earn https://www.wileyhealthlearning.com/Activity/7025323/disclaimerspopup.

Thromboembolic Events Are Independently Associated with Liver Stiffness in Patients with Fontan Circulation.

Thromboembolism (TE) and Fontan-associated liver disease (FALD) are common and lead to significant morbidity in Fontan circulations. Risk factors for TE and the potential link between TE and FALD are not well understood. The objective of this study was to evaluate the association between TE and the severity of FALD based on radiologic liver stiffness.

Filling in the gaps: Implantable bronchial valves in pediatric patients with persistent leaks.

This series presents an adult procedure that benefits young patients with persistent air leaks. This less invasive procedure can be done outpatient with adult/pediatric bronchoscopists providing patients less invasive procedures with better outcomes.

Scar Formation with Decreased Cardiac Function Following Ischemia/Reperfusion Injury in 1 Month Old Swine.

Studies in mice show a brief neonatal period of cardiac regeneration with minimal scar formation, but less is known about reparative mechanisms in large mammals. A transient cardiac injury approach (ischemia/reperfusion, IR) was used in weaned postnatal day (P)30 pigs to assess regenerative repair in young large mammals at a stage when cardiomyocyte (CM) mitotic activity is still detected. Female and male P30 pigs were subjected to cardiac ischemia (1 h) by occlusion of the left anterior descending artery followed by reperfusion, or to a sham operation.

Impact of Palliation Strategy on Interstage Feeding and Somatic Growth for Infants With Ductal-Dependent Pulmonary Blood Flow: Results from the Congenital Catheterization Research Collaborative.

Background In infants with ductal-dependent pulmonary blood flow, the impact of palliation strategy on interstage growth and feeding regimen is unknown. Methods and Results This was a retrospective multicenter study of infants with ductal-dependent pulmonary blood flow palliated with patent ductus arteriosus (PDA) stent or Blalock-Taussig shunt (BTS) from 2008 to 2015. Subjects with a defined interstage, the time between initial palliation and subsequent palliation or repair, were included.

Engineered oncolytic virus for the treatment of cholesteatoma: A pilot in vivo study.

Objective: Determine if oncolytic herpes simplex virus (oHSV) can eradicate cholesteatoma (CHST) in a gerbil model.

Methods: An in vivo model of CHST was developed in Mongolian gerbils by combining inoculation with double ligation of the external auditory canal (EAC). CHST size and bone thickness were measured using morphometric and volumetric quantification techniques via micro-computed tomography (micro-CT).

Relation of Magnetic Resonance Elastography to Fontan Failure and Portal Hypertension.

Fontan associated liver disease is associated with morbidity and mortality in palliated single-ventricle congenital heart disease patients. Magnetic resonance elastography (MRE) provides a quantitative assessment of liver stiffness in Fontan patients. We hypothesized that MRE liver stiffness correlates with liver enzymes, hemodynamics, portal hypertension, and Fontan failure (FF).

Colonic and perianal ulceration exhibiting vacuolar interface dermatitis in the setting of HIV.

We report a case of noninfectious vacuolar interface dermatitis associated with colonic and perianal ulceration in a patient with acquired immunodeficiency syndrome (AIDS), which responded to immunosuppressive treatment. Our findings suggest that interface dermatitis in the setting of AIDS may warrant further gastrointestinal evaluation and may respond to immunosuppression.

Ischemic Stroke in Adults With Congenital Heart Disease: A Population-Based Cohort Study.

Background Congenital heart disease (CHD) is associated with risk factors for ischemic stroke including cardiac arrhythmias and heart failure. However, few long-term follow-up data exist on ischemic stroke risk and associated mortality in adults with CHD. Methods and Results Using Danish nationwide registries, we identified individuals aged ≥18 years diagnosed with CHD, at any age, from 1963 to 2017 and a sex and birth year-matched (1:10) general population comparison cohort.

Cardiac Surgery in Patients With Trisomy 13 and 18: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

Background Congenital heart disease is common in patients with Trisomy 13 (T13) and Trisomy 18 (T18), but offering cardiac surgery to these patients has been controversial. We describe the landscape of surgical management across the United States, perioperative risk factors, and surgical outcomes in patients with T13 and T18. Methods and Results Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database with T13 and T18 who underwent cardiac surgery (2010-2017) were included.

Two cases of atypical twinning: Phenotypically discordant monozygotic and conjoined twins.

Atypical twinning highlights that complex mechanisms responsible for twinning are not fully understood and may give further insight into the mechanisms involved. To assume that phenotypic difference is the result of dizygotic twinning would be erroneous and could have significant implications in the care and counseling provided to these patients.

FDG PET imaging in multiple myeloma: implications for response assessments in clinical trials.

F-fluorodeoxyglucose positron emission tomography integrated with computed tomography (F-FDG PET/CT) is an effective modality to assess disease burden, detect extra-medullary disease and monitor minimal residual disease (MRD) for multiple myeloma (MM) patients. This modality of imaging is incorporated in the International Myeloma Working Group (IMWG) response criteria that are widely used in MM clinical trials. Interpretative pitfalls are commonly encountered in F-FDG PET/CT studies and proper interpretation requires knowledge of the normal physiologic distribution of the tracer affecting available F-FDG for tumor tissue uptake.

Emergency Consent: Patients' and Surrogates' Perspectives on Consent for Clinical Trials in Acute Stroke and Myocardial Infarction.

Background Emergent informed consent for clinical trials in acute myocardial infarction (AMI) and stroke is challenging. The role and value of consent are controversial, and insufficient data exist regarding patients' and surrogates' experiences. Methods and Results We conducted structured interviews with patients (or surrogates) enrolled in AMI or acute stroke trials at 6 sites between 2011 and 2016.

Recommendations for the design of therapeutic trials for neonatal seizures.

Although seizures have a higher incidence in neonates than any other age group and are associated with significant mortality and neurodevelopmental disability, treatment is largely guided by physician preference and tradition, due to a lack of data from well-designed clinical trials. There is increasing interest in conducting trials of novel drugs to treat neonatal seizures, but the unique characteristics of this disorder and patient population require special consideration with regard to trial design. The Critical Path Institute formed a global working group of experts and key stakeholders from academia, the pharmaceutical industry, regulatory agencies, neonatal nurse associations, and patient advocacy groups to develop consensus recommendations for design of clinical trials to treat neonatal seizures.

Fibrillin-2 is a key mediator of smooth muscle extracellular matrix homeostasis during mouse tracheal tubulogenesis.

Epithelial tubes, comprised of polarised epithelial cells around a lumen, are crucial for organ function. However, the molecular mechanisms underlying tube formation remain largely unknown. Here, we report on the function of fibrillin (FBN)2, an extracellular matrix (ECM) glycoprotein, as a critical regulator of tracheal tube formation.

The dangers of sublethal carvacrol exposure: increases in virulence of during endophthalmitis.

can cause endophthalmitis through secretion of virulence factors, including hemolysin BL (Hbl) and nonhemolytic entertoxin (Nhe). Carvacrol is an extract from oregano oil, with potential for curtailing endophthalmitis, due to antimicrobial and anti-inflammatory qualities. However, sublethal levels of carvacrol increases virulence.