bluuebunny/crossref_metadata_2025 huggingface dataset:
Maximizing the benefits of cholesterol-lowering drugs
Authors: Feingold Kenneth R.
Published: July 2019

Purpose of review

Drugs to lower LDL-C levels are very widely used. In this brief review, I will use selected recent studies to delineate several important principles that provide a rationale for how to maximize the benefits of using LDL-C lowering drugs to reduce cardiovascular disease. The focus will be on using statins, ezetimibe, and PCSK9 monoclonal antibodies as recent studies have predominantly utilized these agents.

Recent findings

The key principles to consider when using LDL-C-lowering drugs to reduce cardiovascular disease are: the lower the LDL-C the better; the sooner and the longer one lowers LDL-C the better; the higher the risk of cardiovascular disease the greater the absolute benefit; the higher the baseline LDL-C the greater the absolute benefit; and compared with the benefits of cholesterol-lowering drugs on reducing cardiovascular disease the risk of side effects is very modest.

Understanding and employing these key concepts in caring for patients will allow one to use cholesterol-lowering drugs wisely to maximize the reduction of cardiovascular events.
Middle fossa approach for spontaneous cerebrospinal fluid fistula and encephaloceles
Authors: Tolisano Anthony M. , Kutz Joe Walter
Published: July 2019

Purpose of review

The aim of this article is to describe the middle fossa craniotomy (MFC) approach for the repair of cerebrospinal (CSF) fistula and encephaloceles.

Recent findings

The MFC approach has a greater than 93% success rate for managing CSF fistula and encephaloceles located along the tegmen tympani and tegmen mastoideum. Posterior fossa defects cannot be managed by an MFC approach. Multilayer repair with the combination of soft tissue and durable substances is preferred. Hydroxyapatite bone cement provides a durable repair of thinned or absent areas of bone with a low risk of infection. Concurrent management of symptomatic superior semicircular canal dehiscence may be readily performed. Small keyhole craniotomies with the utilization of the endoscope are possible as a means to minimize temporal lobe retraction.

MFC repair of CSF fistula and encephaloceles is a highly effective approach for the repair of tegmen mastoideum and tegmen tympani defects.
The evolution of presenting signs and symptoms of lateral skull base cerebrospinal fluid leaks
Authors: Naples James G. , Shah Ravi R. , Ruckenstein Michael J.
Published: July 2019

Purpose of review

To review the presenting signs and symptoms of spontaneous cerebrospinal fluid (CSF) leaks of the lateral skull base.

Recent findings

Research continues to demonstrate that CSF leaks from the lateral skull base are insidious, and present with subtle signs and symptoms. Patients commonly present with symptoms of aural fullness, middle ear effusion, and otorrhea following tympanostomy tube insertion that can be confused for chronic otitis media. More recently headache, pulsatile tinnitus, and dizziness/vertigo are being recorded as symptoms at presentation, which is likely a reflection of the association of spontaneous CSF leak with obesity, intracranial hypertension, and superior canal dehiscence. The presence of these less common symptoms in the setting of middle ear effusion should raise suspicion for CSF leak. The rate of meningitis in spontaneous CSF leak is not negligible, and patients should be counseled on this life-threatening risk.

Spontaneous CSF leak from the lateral skull base presents with subtle signs and symptoms and remains a diagnostic challenge. Less common symptoms may represent associations with underlying comorbidities, and awareness of the increasing coincidence of diseases that accompany spontaneous CSF leak is essential to prompt diagnosis and management.
The role of obesity, sleep apnea, and elevated intracranial pressure in spontaneous cerebrospinal fluid leaks
Authors: Rabbani Cyrus C. , Saltagi Mohamad Z. , Nelson Rick F.
Published: July 2019

Purpose of review

Spontaneous cerebrospinal fluid (sCSF) leaks often occurs in middle age, obese females. Here we investigate the role of obesity, idiopathic intracranial hypertension (IIH), and obstructive sleep apnea (OSA) in the pathophysiology of sCSF leaks.

Recent findings

The association of obesity and sCSF leaks has been well established in many studies. It has now been revealed that sCSF leak patients have thinner calvariums along with the skull base. An intracranial process likely leads to calvarium and skull base thinning in sCSF leaks patients since this occurs independent of extracranial bone thinning and independent of obesity. OSA, which is known to cause spikes in intracranial pressure (ICP), has been found to be significantly prevalent in the sCSF population and has been shown to lead to both calvarial and skull base thinning. Chronically elevated ICP (IIH) has also been shown to impact calvarial and skull base thicknesses.

The incidence of sCSF leaks has increased in recent decades along with an increasing rate of obesity. OSA and IIH, which are obesity-related factors and cause transient and chronic elevations in ICP, have now been implicated as critical factors leading to calvarial and skull base thinning and resultant sCSF leaks.
A review of psychological treatments for vomiting associated with paediatric functional gastrointestinal disorders
Authors: Jerson Bradley , Lamparyk Katherine , van Tilburg Miranda A.L.
Published: July 2019

Purpose of review

Vomiting can be a primary symptom or associated with various other functional gastrointestinal disorders (FGIDs). The purpose of this review was to discuss the evidence for psychological treatments for vomiting in pediatric FGID.

Recent findings

Vomiting of functional origin is an increasingly recognized symptom among children and adolescents. It is highly aversive and associated with disability and poor quality of life. Cognitive behavioral therapy, lifestyle modification (especially sleep), diaphragmatic breathing, and hypnosis can be helpful in preventing vomiting episodes and reducing disability. However, no randomized clinical trials have been performed. An evidence base for psychological treatments in children with vomiting of functional origin is highly needed.

Increased evidence is demonstrating value and efficacy of incorporating psychogastroenterology practices into ongoing treatment plans for digestive conditions. Current psychological treatments are focused on prevention of vomiting through stress reduction and lifestyle modification, reduction of disability by limiting avoidance behaviours, as well as counteracting biological factors. However, psychological treatments have not been shown to be helpful during an acute vomiting episode. More research is needed to build an evidence base for psychological treatments in vomiting disorders.
Stem cells: will they cure pediatric heart failure?
Authors: Bernstein Daniel
Published: July 2019

Purpose of review

The purpose of this review is to provide an overview of the state of cardiac regenerative medicine, including the unique opportunities and challenges in its application to pediatric patients.

Recent findings

There has been a rapid proliferation of clinical studies using stem cells in adults with heart failure, yet little convincing evidence of clinically significant improvement. Readers will develop an understanding of the current limitations of stem cell treatments and the challenges to be overcome before they can achieve successful clinical translation.

Clinical trials in cardiac regeneration using stem cells are advancing rapidly despite clear knowledge of mechanism and rigorous evidence in animal models. The potential for cardiac regeneration in children may be greater than in adults, given the smaller degree of scar present in nonischemic heart disease and the greater potential of the younger heart for repair. However, similar to adult trials, there has yet to be convincing evidence of a positive effect in pediatric patients, and rigorous controlled studies are still lacking. There is still much biology to be learned in cardiac regeneration; future clinical trials in children should be based on solid evidence in animal models of both efficacy and safety.
Fibrodysplasia ossificans progressiva: lessons learned from a rare disease
Authors: Akyuz Gulseren , Gencer-Atalay Kardelen , Ata Pinar
Published: July 2019

Purpose of review

Fibrodysplasia ossificans progressiva (FOP) is an extremely rare and severely disabling autosomal dominant disease that is yet to be clearly understood. The purpose of this review is to present recent literature on pathophysiology, clinical features, diagnosis and treatment of FOP.

Recent findings

FOP is characterized by congenital great toe deformity and progressive heterotopic ossifications in connective tissue. Heterotopic ossifications occur after painful flare-ups that can arise spontaneously or can be triggered by minor trauma. Each flare-up ultimately causes restriction of related-joint, and along with the others eventually leads to immobility. Death is usually caused by pulmonary complications because of chest wall involvement. The causative gene of FOP is activin A receptor type 1 (ACVR1), a bone morphogenetic protein-signalling component, which normally acts to inhibit osteoblastogenesis. The treatment of FOP is still preventive and supportive.

Although there are still gaps in the underlying mechanism of FOP, effective treatment options, such as potential pharmacologic targets and cell-based therapies are promising for the future. Some of these were tested without a clinical trial setting, and are currently in the process of evidence-based research.
Pediatric heart transplantation in the current era
Authors: D’Addese Laura , Joong Anna , Burch Michael , Pahl Elfriede
Published: July 2019

Purpose of review

To provide an international perspective and current review of pediatric heart transplantation (PHTx).

Recent findings

Waitlist survival and long-term outcomes in PHTx continue to improve. Strategies to maximize donor pool utilization include ABO incompatible listing for infants and expanded donor-to-recipient weight ranges. However, there is a high degree of practice variation internationally, from listing strategies and donor acceptance practices to chronic immunosuppression regimens, long-term graft surveillance, and consideration for retransplantation.

Common indications for PHTx include end-stage congenital heart disease and cardiomyopathy. Current median graft survival among PHTx recipients ranges from 13 to 22 years. Common morbidities include infection, rejection, renal dysfunction, coronary allograft vasculopathy, and posttransplant lymphoproliferative disease. International registry data, collaborative initiatives to standardize management, and multicenter studies continue to improve knowledge and advancement of the field.
Recent developments in understanding the mechanisms of food allergy
Authors: Schmiechen Zoe C. , Weissler Katherine A. , Frischmeyer-Guerrerio Pamela A.
Published: July 2019

Purpose of review

The prevalence of food allergy is rising globally. This review will discuss recent discoveries regarding the immunologic mechanisms that drive the initial sensitization and allergic response to food antigens, which may inform prevention and treatment strategies.

Recent findings

Tolerance to food antigens is antigen-specific and promoted by oral exposure early in life and maternal transfer of immune complexes via breast milk. IgG can inhibit both the initiation and effector phases of allergic responses to food antigens in mice, and high levels of food-specific IgG4 are associated with acquisition of tolerance in humans. Disruption of the skin barrier provides a route for food sensitization through the actions of mast cells, type 2 innate lymphoid cells, and IL-33 signaling. Regulatory T cells (Tregs) promote acquisition of oral tolerance, although defects in circulating allergen-specific Tregs are not evident in children with established food allergy. Certain microbes can offer protection against the development of IgE and food allergic responses, while dysbiosis increases susceptibility to food allergy.

Tolerance to food antigens is antigen-specific and is promoted by oral exposure early in life, maternal transfer of immune complexes, food-specific IgG, Tregs, an intact skin barrier, and a healthy microbiome.
Management of Anti‐drug Antibodies to Biologic Medications in Children With Inflammatory Bowel Disease
Authors: Cohen Reuven Zev , Schoen Bess T. , Kugathasan Subra , Sauer Cary G.
Published: July 2019

Background:

Treatment of pediatric inflammatory bowel disease (IBD) with monoclonal anti‐ tumor necrosis factor‐alpha (TNFα) can result in immunogenicity and formation of anti‐drug antibodies (ADAs). ADAs are associated with loss of clinical response and worsening disease progression. Data examining treatment interventions to overcome ADA in pediatric patients with IBD are lacking.

Results:

Medical records were reviewed from 234 children and adolescents with IBD treated with infliximab or adalimumab who underwent therapeutic drug monitoring (626 tests). All patients who had detectable antibodies were further analyzed. A total 58 patients (24.8%) developed ADA while being treated with infliximab or adalimumab. The incidence of antibody development was 12.9 per 100 person‐years of anti‐TNF treatment. Twenty‐eight patients underwent dose optimization and 54% had undetectable ADA on follow‐up monitoring. The mean duration of antibody suppression was 16.8 ± 10.9 months in those who were successfully suppressed with optimization. Patients who switched to a second anti‐TNF medication were not more likely to develop antibodies to the second agent.

Conclusions:

With limited therapies for IBD and the chronicity of the disease, we advocate salvage of the current anti‐TNF through dose optimization in pediatric patients with antibody level <10 U/mL.
Incidence of Paediatric Stricturing Duodenal Crohn Disease
Authors: Burgess Christopher J. , Gillett Peter , Mitchell David , Hammond Philip , Henderson Paul , Wilson David C.
Published: July 2019

Objectives:

Stricturing duodenal Crohn disease (CD) is a rare but serious presentation of CD causing significant morbidity. We aim to provide the first robust incidence data and case studies on this severe presentation in children.

Methods:

A regional cohort of prospectively acquired incident cases of paediatric CD diagnosed <16 years of age in South‐East Scotland was captured over a 19‐year period (1999–2018). A retrospective review was conducted on the medical records of all patients together with a review of the available literature and consensus guidelines. Incidence rates for all CD and for duodenal stricturing CD were calculated.

Results:

A total of 247 new cases of paediatric CD were diagnosed within the study period. Median age at diagnosis was 12.5 years with 62% male predominance. Overall paediatric CD incidence rate was 5.70/100,000/year with a specific duodenal B2 phenotype disease incidence rate of 0.05/100,000/year; representing 0.8% of incident cases at diagnosis. Two incident cases of stricturing duodenal CD presented with systemic symptoms of weight loss, abdominal pain, anorexia, and lethargy, together with persistent vomiting suggestive of obstruction. Both cases partially responded to intensive medical therapy but eventually required laparoscopic gastroduodenostomy. A detailed literature search confirmed there are no paediatric incidence data, guidelines, or case reports relating to duodenal stricture as either a presentation or complication of CD.

Conclusions:

Duodenal structuring disease is a rare but serious presentation of CD causing significant morbidity and not currently covered in the paediatric literature or consensus guidelines. Best practice medical and surgical management remain uncertain and require further research.
Management of Biliary Atresia in France 1986 to 2015
Authors: Fanna Martina , Masson Guillaume , Capito Carmen , Girard Muriel , Guerin Florent , Hermeziu Bogdan , Lachaux Alain , Roquelaure Bertrand , Gottrand Frédéric , Broue Pierre , Dabadie Alain , Lamireau Thierry , Jacquemin Emmanuel , Chardot Christophe
Published: July 2019

Objectives:

This study analyses the prognosis of biliary atresia (BA) in France since 1986, when both Kasai operation (KOp) and liver transplantation (LT) became widely available.

Methods:

The charts of all patients diagnosed with BA born between 1986 and 2015 and living in France were reviewed.

Results:

A total of 1428 patients were included; 1340 (94%) underwent KOp. Total clearance of jaundice (total bilirubin ≤20 μmol/L) was documented in 516 patients (39%). Age at KOp (median 59 days, range 6–199) was stable over time. Survival with native liver after KOp was 41%, 35%, 26%, and 22% at 5, 10, 20, and 30 years, stable in the 4 cohorts. 25‐year survival with native liver was 38%, 27%, 22%, and 19% in patients operated in the first, second, third month of life or later, respectively (P = 0.0001). Center caseloads had a significant impact on results in the 1986 to 1996 cohort only. 16%, 7%, 7%, and 8% of patients died without LT in the 4 cohorts (P = 0.0001). A total of 753 patients (55%) underwent LT. Patient survival after LT was 79% at 28 years. Five‐year patient survival after LT was 76%, 91%, 88%, and 92% in cohorts 1 to 4, respectively (P < 0.0001). Actual BA patient survival (from diagnosis) was 81%. Five‐year BA patient survival was 72%, 88%, 87%, and 87% in cohorts 1986 to 1996, 1997 to 2002, 2003 to 2009, and 2010 to 2015, respectively (P < 0.0001).

Conclusions:

In France, 87% of patients with BA survive nowadays and 22% reach the age of 30 years without transplantation. Improvement of BA prognosis is mainly due to reduced mortality before LT and better outcomes after LT.
The Role of Distress and Pain Catastrophizing on the Health‐related Quality of Life of Children With Inflammatory Bowel Disease
Authors: De Carlo Chiara , Bramuzzo Matteo , Canaletti Claudia , Udina Chiara , Cozzi Giorgio , Pavanello Paolo M. , Rampado Stefania , Martelossi Stefano , Giudici Fabiola , Di Leo Grazia , Barbi Egidio
Published: July 2019

Objectives:

Inflammatory bowel disease (IBD) can be particularly challenging during the pediatric age with a relevant impact on patient's health‐related quality of life (HRQoL). Disease activity accounts for only a small part of the variability in HRQoL, and psychological factors can play a significant role. We aimed to evaluate the impact of patient's distress and pain catastrophizing on children and adolescents with IBD.

Methods:

We prospectively recruited children aged 8 to 18 with IBD and recorded demographic and disease characteristics. Patients answered questionnaires on HRQoL (IMPACT III), distress (distress thermometer [DT]), and pain catastrophizing (Pain Catastrophizing Scale‐Children [PCS‐C]). Univariate and multivariate regression models analysis were used to evaluate correlations between patients’ characteristics, disease activity, distress, pain catastrophizing, and HRQoL.

Results:

Seventy‐one patients were enrolled (median age 13.6, 49.3% Crohn disease, 50.7% ulcerative colitis). Median HRQoL, DT, and PCS‐C scores were 78.6 (interquartile range 68.0–87.1), 3.0 (1.0–5.0), and 12.0 (4.0–23.0), respectively. Patient's distress and pain catastrophizing levels significantly correlated with HRQoL. Pain catastrophizing had the strongest impact on HRQoL (Spearman correlation coefficient, ρ = 0.73), followed by distress (ρ = 0.67), and ulcerative colitis severity (ρ = 0.67). The DT and the PCS‐C scores were significantly associated (ρ = 0.46).

Conclusions:

Distress and pain catastrophizing have a significative impact on HRQoL in young patients with IBD. Physicians should recognize the role of these psychological factors and consider cognitive‐behavioral therapy to optimize the patient's health.
Vitamin D Supplementation and T Cell Regulation in Preterm Infants
Authors: Aly Hany , Mohsen Lamiaa , Bhattacharjee Indrani , Malash Amr , Atyia Amr , Elanwary Sherif , El Hawary Rabab
Published: July 2019

The objective of this study was to evaluate the effect of 2 different doses of vitamin D on the expression of T regulatory cells (Treg) in premature infants. A double‐blind randomized controlled trial was conducted on preterm infants born with gestational age between 280/7 and 336/7 weeks. Subjects were randomly assigned to receive 400 or 800 IU/day of vitamin D3 when they achieved 100 mL/kg of enteral feeds. Percentage increase in Treg cell counts were measured by flow cytometry at enrollment, and after 1 and 4 weeks of oral vitamin D supplementation at the allotted doses in both groups. Short‐term morbidity and mortality outcomes were also assessed. A total of 40 infants were enrolled, 20 in each group. The change in Treg count (%) was significantly less in the low‐dose vitamin D3 supplementation group after 1 week (1.9 ± 5.5 vs 60 ± 5.6, P = 0.0005) and after 4 weeks (1.8 ± 5.7 vs 73.7 ± 5.6, P = 0.0028). The 2 groups did not differ in anthropometric measurements, duration of oxygen and respiratory support, and mortality. Length of hospital stay was longer in the low‐dose group (24.9 ± 5.14 vs 22 ± 3.49, P = 0.04). Oral vitamin D supplementation has a dose and time dependent effect on percentage of Treg in infants born prematurely. The 800 IU dose of vitamin D3 did not have apparent short‐term side effects. Larger studies are needed to explore the effect of vitamin D3 dosing on length of hospital stay.
Biliary Atresia as a Disease Starting In Utero
Authors: Mysore Krupa R. , Shneider Benjamin L. , Harpavat Sanjiv
Published: July 2019

Biliary atresia (BA) is the most common reason for pediatric liver transplant. BA's varied presentation, natural history, and treatment with the Kasai portoenterostomy have been well described; however, when BA starts relative to birth has not been clearly defined. In this review, we discuss laboratory, imaging, and clinical data which suggest that most if not all forms of BA may start before birth. This early onset has implications in terms of delivering treatments earlier and identifying possible factors underlying BA's etiology.
Celiac Dietary Adherence Test simplifies Determining Adherence to a Gluten‐free Diet in Swedish Adolescents
Authors: Johansson Katarina , Norström Fredrik , Nordyke Katrina , Myleus Anna
Published: July 2019

Objectives:

The aims of the study were to ascertain whether the Celiac Dietary Adherence Test (CDAT) could contribute in determining adherence to a gluten‐free diet in patients with celiac disease and to evaluate the diet adherence and well being of a study population 5 years after a celiac disease screening known as “Exploring the Iceberg of Celiacs in Sweden.”

Methods:

Through the screening, 90 adolescents (born 1997) were diagnosed with biopsy‐proven celiac disease at 12 years of age. Of them, 70 (78%) came to a 5‐year follow‐up where anti–tissue transglutaminase antibodies 2 was tested and a questionnaire was filled in, including CDAT, which consists of 7 questions related to adherence. Nonparametrical tests were used to determine associations between adherence measures.

Results:

Among the adolescents, 86% were adherent to a gluten‐free diet 5 years after screening, 38% reported their general well being as excellent, 50% very well, and 12% well. Statistically significant associations were seen between anti–tissue transglutaminase antibodies 2 and the CDAT score (P = 0.033), and the self‐reported adherence question and the CDAT score (P < 0.001).

Conclusions:

The screening‐detected adolescents reported a high level of well being and adherence to a gluten‐free diet 5 years after screening. We conclude that the CDAT can be used in clinical practice as an estimation of adherence to a gluten‐free diet. It would be most suitable to use in conjunction with currently used adherence measures, but can also be used as a stand‐alone method when others are not accessible.
Evaluation of Bone Mineral Density in Children With Hemophilia: An Observational Case-Control Study
Authors: Ashritha A. , Delhi Kumar C.G. , Sahoo Jayaprakash , Nalini P.
Published: July 2019

Objectives:

The objectives of this study were to study bone mineral content (BMC), bone mineral density (BMD), vitamin D level, and bone turnover markers in children with hemophilia and compare it with their normal counterparts.

Design:

This was an observational case-control study.

Setting:

This study was conducted in our tertiary care institute during the period spanning from September 2016 to June 2018.

Materials and Methods:

A total of 38 children with hemophilia 2 to 18 years of age and 38 age-matched and sex-matched healthy controls were included. Children with symptomatic hypocalcemia and those receiving drugs affecting BMC and BMD were excluded. Dual-energy x-ray absorptiometry was performed to estimate BMC and BMD. 25-Hydroxyvitamin D [25(OH)D], intact parathormone, osteocalcin, calcium, phosphate and alkaline phosphatase, and spot urine pyrilinks-D/creatinine ratio were estimated in them.

Results:

BMC and BMD in cases was lower than that in controls (P<0.05). Prevalence of low BMC was seen in 22 (58%) and low BMD in 8 (21%) of cases. All controls had normal BMC and BMD for age. The prevalence of low vitamin D level (<20 ng/mL) was seen in 36 (95%) among cases and in 25 (65%) among controls (P<0.001). Serum phosphorus was lower, and serum alkaline phosphatase was higher in cases than in controls (P<0.05).

Conclusion:

BMC, BMD, and vitamin D in hemophiliacs were lower than in the general population.
Unicentric Castleman Disease Mimicking an Autoinflammatory Disorder: A Diagnostic Challenge in a Pediatric Patient With Recurrent Fever
Authors: Kisla Ekinci Rabia Miray , Balci Sibel , Ergin Melek , Kupeli Serhan , Bayram Ibrahim , Yilmaz Mustafa , Kocabas Emine
Published: July 2019

Unicentric Castleman disease (CD) is a rare lymphoproliferative disorder that is characterized by the enlargement of lymph nodes on the neck, mediastinum, and retroperitoneum. Herein, we present a 6-year-old female patient, referred to our medical center because of recurrent fever accompanied by cervical lymphadenopathy and elevated inflammatory markers since 3 years of age. Fever episodes lasting 1 day continued irregularly without any accompanying symptom. MEditerranean FeVer (MEFV) gene analysis showed no mutations; however, as inflammatory markers including serum amyloid A remained markedly high during attack-free periods, colchicines was initiated. The patient did not respond to maximally tolerated doses of colchicine; therefore, we added canakinumab and systemic methylprednisolone, subsequently. Unresponsiveness to 3 doses of bimonthly canakinumab and new-onset hepatosplenomegaly led us to investigate large-vessel vasculitis and malignancy; therefore, we performed Position emission tomography, which further revealed a hypermetabolic retroperitoneal solid mass. After performing the excisional biopsy, the patient has been diagnosed as suffering from hyaline vascular variant CD, confirmed by histopathology. In conclusion, we report a pediatric unicentric CD, which resembled autoinflammatory diseases and responded well to surgical resection, with the normalization of inflammatory markers 1 month after the procedure. CD, even the unicentric and hyaline vascular variant, should be considered in the differential diagnosis of the patients with an autoinflammatory phenotype.
Congenital Hepatoblastoma and Beckwith-Wiedemann Syndrome
Authors: Zivot Andrea , Edelman Morris , Glick Richard , Hong Andrew , Fish Jonathan D.
Published: July 2019

Following the discovery of a fetal hepatic tumor, labor was induced at 38 weeks, and a phenotypically normal female was delivered vaginally. A serum alpha-fetoprotein level at birth was 373,170 ng/mL. Postnatal magnetic resonance imaging confirmed a mass in the right lobe of the liver, and a percutaneous core biopsy revealed an epithelial type hepatoblastoma with predominantly embryonal histology. Methylation testing revealed hypomethylation at imprinting center 2, consistent with a diagnosis of Beckwith-Wiedemann syndrome. This case suggests that Beckwith-Wiedemann syndrome testing should be considered in all patients with hepatoblastoma, even in the absence of other phenotypic stigmata.
A Case of Pulmonary Veno-occlusive Disease Following Hepatic Veno-occlusive Disease After Autologous Hematopoietic Stem Cell Transplantation for Neuroblastoma
Authors: Isshiki Kyohei , Shima Haruko , Yamazaki Fumito , Takenouchi Toshiki , Shimada Hiroyuki
Published: July 2019

Pulmonary veno-occlusive disease (PVOD) is an uncommon form of pulmonary hypertension that is usually difficult to diagnose and is refractory to conservative treatment. PVOD can occur in connection with high-dose chemotherapy or hematopoietic stem cell transplantation, similar to hepatic veno-occlusive disease (HVOD). Here, we present a case of neuroblastoma with PVOD following HVOD after high-dose chemotherapy that was resolved with conservative treatment. Respiratory symptoms or edema after HVOD may suggest PVOD, and prompt diagnosis on high-resolution computed tomography will result in a favorable prognosis.
ADN-to-BSN Articulation, Academic Progression, and Transition
Authors: Jeffreys Marianne R.
Published: July 2019

Background

Although improvements have been made in articulation agreements, credit transfers, and concurrent enrollments, meeting the 80% BSN by 2020 goal falls short.

Problem

Nursing students at key transitional points (such as first- and last-semester students) are faced with numerous academic and career path challenges and opportunities simultaneously, often leaving them overwhelmed and in need of additional support and resources.

Approach

Guided by Jeffreys' Nursing Universal Retention and Success model, a proactive, holistic approach for facilitating articulation, academic progression, and transition is presented.

Outcomes

Implementation with fourth/last-semester associate degree nursing students and newly admitted RN-BSN students yielded positive student feedback and survey data.

Conclusions

Nurse educators can make a positive difference in students' transitional experiences, career path planning, and educational mobility via a coordinated, connected, proactive, holistic approach guided by an evidence-based theoretical framework.
An Integrative Review Examining Student-to-Student Incivility and Effective Strategies to Address Incivility in Nursing Education
Authors: Rose Kristine , Jenkins Sheryl , Mallory Caroline , Astroth Kim , Woith Wendy , Jarvill Melissa
Published: July 2019

Background

Incivility is a low-intensity, discourteous behavior intended to disrupt or harm positive interaction. If allowed, student-to-student incivility can undermine the educational environment.

Purpose

The purpose of the integrative review was to examine factors influencing incivility among nursing students and teaching strategies used to reduce incivility in nursing education.

Methods

Qualitative and quantitative studies were reviewed. The Johns Hopkins Research Evidence Appraisal tool was used to narrow down the selection of articles. Content analysis was used to evaluate the qualitative research.

Results

Five major points of interest were identified: workload and high expectations contributed to incivility, degrees of incivility, effects of incivility, coping mechanisms among individuals, and effective teaching strategies addressing incivility.

Conclusion

Continued research on innovative teaching strategies that raise awareness of civility while reducing incivility is warranted.
A Bayesian Approach to Gateway Course Identification With Implications for the Prenursing Curriculum
Authors: Stankus Jo-Ann , Hamner Mark , Stankey Michael , Mancuso Peggy
Published: July 2019

Background

Analysis of student performance in gateway courses has been an important predictor of successful admission into upper-division nursing.

Purpose

The aim was to explore the utility of a Bayesian statistical framework for determining threshold grades in prenursing courses that serve as gateways for successful admission into upper-division nursing programs.

Methods

Records of 3500 prenursing students who entered the prenursing program of a midsized public university during the past decade were analyzed. The Bayesian framework was used to incorporate conditional probabilistic concepts of sensitivity and specificity to calculate gateway impact of various grade level cutoffs on successful upper-division nursing admission.

Results

Identification, sequencing, and combination of grades attained in these gateway courses revealed different pathways to successful admission into upper-division nursing based on first-semester grade point average and ethnicity.

Conclusions

Identification of primary/secondary gateway courses enhances successful matriculation and provides valuable information for advisors and curriculum planners for prenursing majors.
Assessing the Impact of Telehealth Objective Structured Clinical Examinations in Graduate Nursing Education
Authors: Phillips Tiffany A. , Munn Allison C. , George Tracy P.
Published: July 2019

Background

The National Organization of Nurse Practitioner Faculties supports the incorporation of telehealth in nurse practitioner (NP) education.

Purpose

The purpose of this article is to describe the implementation of a no-cost telehealth simulation and its impact on students in their first NP clinical course.

Methods

Twenty-eight NP students participated in the telehealth objective structured clinical examination (OSCE). Students completed an online survey about the impact of telehealth OSCE simulation both before and after the simulation.

Results

The students' general understanding of the field of telehealth was significantly greater after the telehealth OSCE experience. Directed content analysis revealed 3 themes: usefulness of telehealth, benefit in role preparation, and technology.

Conclusion

The use of telehealth simulation can be an effective strategy to assess clinical competency, provide individualized feedback, and ensure students are using evidence-based practice.
Doctoral Degree Preferences for Nurse Educators
Authors: King Tara Spalla , Melnyk Bernadette Mazurek , O'Brien Tara , Bowles Wendy , Schubert Carolyn , Fletcher Linnea , Anderson Cindy M.
Published: July 2019

Background

Current doctoral degree options may not meet needs of nurses desiring educator roles in academic or health care settings.

Purpose

The purpose of the study was to assess preferences for existing terminal degree options for nurse educators and determine the need for a new doctoral degree in nursing education.

Method

Nurses in practice, academia, and leadership nationwide (n = 826) answered a 19-item needs assessment survey regarding adequacy of and interest in terminal degree options.

Results

More than 60% reported current doctoral degrees available to nurse educators lack content in curriculum development and teaching-learning best practices in nursing. Most data supported a new terminal degree.

Conclusion

A primary factor in nurses choosing not to enroll in a terminal degree program may be limited availability of education aligned with career goals. The doctor of nursing education, a practice degree parallel to the DNP, could prepare nurses for academic and practice educator roles.
Marijuana Use in Pregnancy: A Review
Authors: Thompson Rebecca , DeJong Katherine , Lo Jamie
Published: July 2019

Importance

Marijuana is the most commonly used dependent substance in pregnancy. The main active chemical of marijuana (delta-9-tetrahydrocannabinol [THC]) readily crosses the placenta, and cannabinoid receptors have been identified in fetal brain and placenta. As a result, prenatal marijuana use could potentially have detrimental impact on fetal development.

Objective

This review aims to summarize the existing literature and current recommendations for marijuana use while pregnant or lactating.

Evidence Acquisition

A PubMed literature search using the following terms was performed to gather relevant data: “cannabis,” “cannabinoids,” “marijuana,” “fetal outcomes,” “perinatal outcomes,” “pregnancy,” “lactation.”

Results

Available studies on marijuana exposure in pregnancy were reviewed and support some degree of developmental disruption, including an increased risk of fetal growth restriction and adverse neurodevelopmental consequences. However, much of the existing prenatal marijuana research was performed in the 1980s, when quantities of THC were lower and the frequency of use was less. Additionally, most human studies are also limited and conflicting as most studies have been observational or retrospective, relying primarily on patient self-report and confounded by polysubstance abuse and small sample sizes, precluding determination of a causal effect specific for marijuana. Given the paucity of evidence, it is currently recommended to avoid using marijuana while pregnant or when breastfeeding.

Conclusion and Relevance

There is a critical need for research on effects in pregnancy using present-day THC doses. Once the adverse perinatal effects of marijuana exposure are identified and well characterized, patient education and antenatal surveillance can be developed to predict and mitigate its impact on maternal and fetal health.

Target Audience

Obstetricians and gynecologists, family physicians.

Learning Objectives

After participating in this activity, the provider should be better able to counsel patients regarding prenatal marijuana use; assess patients during pregnancy for marijuana use; and explain recommendations regarding marijuana use while breastfeeding.
Evidence-Based Review of Vaginal Native Tissue Hysteropexy for Uterovaginal Prolapse
Authors: Hoke Tanya P. , Tan-Kim Jasmine , Richter Holly E.
Published: July 2019

Importance

As surgical techniques evolve in the treatment of pelvic organ prolapse and patient preferences are better understood, more studies are investigating uterine-sparing procedures for efficacy, safety, and potentially improved quality of life. Much of the literature reflects the use of mesh material in uterine-sparing procedures, and there is a paucity of data regarding the safety and efficacy of native tissue uterine-sparing procedures for the treatment of pelvic organ prolapse.

Objective

To summarize existing evidence regarding objective and subjective outcomes of uterine-preserving procedures including the Manchester procedure (MP) as well as native tissue uterovaginal hysteropexy with repairs, namely, uterosacral hysteropexy (USH) and sacrospinous hysteropexy (SSH), compared with outcomes of total vaginal hysterectomy (TVH) with repairs for the management of uterovaginal prolapse.

Evidence Acquisition

A review of the literature included MEDLINE, Cochrane, and clinicaltrials.gov databases.

Results

Few level 1 data exist comparing outcomes of native tissue hysteropexy to vaginal hysterectomy for management of uterovaginal prolapse. In general, outcomes of the MP for the management of uterovaginal prolapse revealed that compared with TVH it is associated with shorter operative times, lower estimated blood loss and risk of blood transfusion with no difference in hospital stay, and similar quality of life and sexual function outcomes. Retrospective data suggest no difference with respect to recurrent prolapse of any compartment between USH and TVH with repairs. Level 1 data reveal that SSH has been shown to have similar 1-year outcomes and safety compared with TVH with native tissue suspension. Women with stage 4 prolapse who undergo an SSH may be at higher risk of recurrence and may benefit from an alternative method of apical prolapse repair.

Conclusions and Relevance

More level 1 data are needed in order to robustly understand long-term differences in outcomes between native tissue uterine-conserving versus vaginal hysterectomy surgical approaches in women with uterovaginal prolapse.

Target Audience

Obstetricians and gynecologists, family physicians.

Learning Objectives

After completing this activity, the learner should be better able to explain why uterine-sparing procedures for uterovaginal prolapse are becoming more popular in the United States; compare similarities and differences in subjective and objective outcomes between the MP and TVH; describe subjective and objective outcomes between vaginal native tissue USH and SSH to TVH; and analyze if uterine-sparing surgical procedures impact pregnancy outcomes.
Cystoscopy at the Time of Benign Hysterectomy: A Decision Analysis
Authors: Cadish Lauren A. , Ridgeway Beri M. , Shepherd Jonathan P.
Published: July 2019

(Abstracted from Am J Obstet Gynecol 2019;220:369.e1–369.e7)

Urinary tract injury (bladder or ureter) occurs in up to 4.3% of all hysterectomies. The risk of morbidity is significantly decreased when a urinary tract injury is detected intraoperatively.
Guidelines for Perioperative Care in Gynecologic/Oncology: Enhanced Recovery After Surgery (ERAS) Society Recommendations—2019 Update
Authors: Nelson Gregg , Bakkum-Gamez Jamie , Kalogera Eleftheria , Glaser Gretchen , Altman Alon , Meyer Larissa A. , Taylor Jolyn S. , Iniesta Maria , Lasala Javier , Mena Gabriel , Scott Michael , Gillis Chelsia , Elias Kevin , Wijk Lena , Huang Jeffrey , Nygren Jonas , Ljungqvist Olle , Ramirez Pedro T. , Dowdy Sean C.
Published: July 2019

(Abstracted from Int J Gynecol Cancer 2019; doi: 10.1136/ijgc-2019-000356)

Enhanced Recovery After Surgery (ERAS) is a global initiative dedicated to improving gynecologic/oncology surgical quality with attention to both clinical outcomes and economic impact. The ERAS guidelines, first published in February 2016, require updates on a regular basis in order to keep up with contemporary literature and evidence.
Uterosacral Ligament Suspension Versus Robotic Sacrocolpopexy for Treatment of Apical Pelvic Organ Prolapse
Authors: Smith Benjamin C. , Crisp Catrina C. , Kleeman Steven D. , Yook Eunsun , Pauls Rachel N.
Published: July 2019

(Abstracted from Female Pelvic Med Reconstr Surg 2019;25:93–98)

More than 10% of women with pelvic organ prolapse (POP) will undergo reconstructive pelvic surgery during their lifetime. There are many surgical options available to patients undergoing reconstruction for POP.
Does Maternal Age at Retrieval Influence the Implantation Potential of Euploid Blastocysts?
Authors: Irani Mohamad , Zaninovic Nikica , Rosenwaks Zev , Xu Kangpu
Published: July 2019

(Abstracted from Am J Obstet Gynecol 2019;220:379.e1–379.e7)

The average age of first-time mothers continues to rise steadily on a global scale. Concerningly, maternal age is a critical factor when determining the probability of conception either naturally or through in vitro fertilization.
The Impact of Extending the Second Stage of Labor to Prevent Primary Cesarean Delivery on Maternal and Neonatal Outcomes
Authors: Zipori Yaniv , Grunwald Oren , Ginsberg Yuval , Beloosesky Ron , Weiner Zeev
Published: July 2019

(Abstracted from Am J Obstet Gynecol 2019;220(2):191.e1–191.e7)

Cesarean delivery (CD) is associated with higher morbidity and mortality than vaginal birth. More than one-third of all indications for primary CD are due to labor dystocia (abnormal progression of labor defined as abnormal descent of the fetal head) in the second stage of labor.
Chronic Hypertension: Effect of Blood Pressure Control on Pregnancy Outcome
Authors: Panaitescu Anca M. , Roberge Stephanie , Nicolaides Kypros H.
Published: July 2019

(Abstracted from J Matern Fetal Neonatal Med 2019;32(5):857–863)

Chronic hypertension (cHTN) complicates 1% to 2% of pregnancies. In these pregnancies, there is a 10-fold increase in the risk of preeclampsia (PE) and 2-fold increase in risk of small for gestational age (SGA) neonates.
Sleep disturbances in orthopaedic trauma patients
Authors: Lu Keyin , Barron John O. , Israel Heidi , Cannada Lisa K.
Published: July 2019

Objectives:

To determine the prevalence of sleep disturbances in orthopaedic trauma patients 3 months following surgery and to identify any subset(s) of patients at high risk for prolonged sleep disturbance.

Design:

Prospective cohort.

Setting:

Level 1 Trauma Center.

Patient/Participants:

All patients at an orthopaedic trauma clinic from May 3, 2016 to Feb 23, 2017.

Main outcome measurements:

Baseline patient Pittsburgh Sleep Questionnaire (PSQI) and Insomnia Severity Index (ISI) scores compared to PSQI and ISI scores 3-months postoperatively. Both gender and age stratified data analyses were performed.

Results:

Sixty-six patients met our inclusion criteria and completed both baseline and 3-month surveys. There were 44 males and 22 females. There was a significant increase in PSQI and ISI scores from baseline to 3 months across all patients. Further analysis revealed significant increases from baseline to 3 months in both PSQI and ISI scores for female patients but not male patients. There was a significant difference from baseline to 3 months in patients 50 years old and under but not for patients above 50 years old. No patients required revision surgery in the first 3 months.

Conclusions:

More than half of all patients reported continued sleep disturbance 3 months postoperatively. Females are at particularly increased risk for sleep disturbance. These findings demonstrate that sleep disturbances merit attention in the early stages of the postoperative recovery process.

Level of evidence:

Therapeutic Level II.
PD-L1 Copy Number Variation Does Not Correlate With PD-L1 Expression or Response to Anti-PD-1 Immunotherapy In Patients With Advanced Melanomas
Authors: Pérottet Jérémy , Le Goff Emeline , Legoupil Delphine , Quéré Gilles , Schick Ulrike , Marcorelles Pascale , Uguen Arnaud
Published: July 2019

Predicting the response to PD-1/PD-L1 immune checkpoint blockade in patients with metastatic melanoma remains challenging. In this study, we have investigated for the relationships between PD-L1 expression, PD-L1 copy number variations, and the response to anti-PD-1 therapies. We studied the formalin-fixed paraffin-embedded tumor samples of 36 patients with metastatic melanoma using PD-L1 immunohistochemistry (IHC) and PD-L1/chromosome 9 fluorescent in situ hybridization (FISH). PD-L1 IHC was positive in 3 patients (8.33%, with >5% stained tumor cells) and PD-L1 FISH test revealed 5 (13.8%) PD-L1 amplifications, 8 (22.2%) PD-L1 gains, and 2 (5.5%) PD-L1 losses. Among 14 responders and 13 nonresponders to anti-PD-1 immunotherapy, we concluded that there was no significant relationship between PD-L1 expression, PD-L1 copy number variations, and the response to anti-PD-1 therapies. In our study, the determination of PD-L1 expression using IHC and PD-L1 copy number using FISH was insufficient to predict the response to PD-1/PD-L1 immune checkpoint blockade in patients with advanced melanomas.