bluuebunny/crossref_metadata_2025 huggingface dataset:
Glaucoma With Alkaptonuria as a Result of Pigment Accumulation
Authors: Okutucu Murat , Aslan Mehmet G. , Findik Hüseyin , Yavuz Göksu
Published: February 2019

Purpose:

To report a case of alkaptonuria (AKU) in a patient with bilateral conjunctival and scleral black colorization who was diagnosed with glaucoma thereafter.

Methods:

This is a single case report.

Results:

A 67-year-old male patient with bilateral black colorization of conjunctiva and sclera was referred to our hospital. In the biomicroscopic examination, globular dark pigmentation was observed in the conjunctiva, sclera, and limbal cornea. The patient was diagnosed with a nuclear cataract in both eyes. He also had gray skin pigmentation at his nose and paranasal area. Corneal topography examination revealed irregular astigmatism. Intraocular pressure values were 29 and 31 mm Hg, in the right and left eye, respectively, with Goldmann applanation tonometry. The diagnosis of AKU was made after pathologic assessment of conjunctival biopsy by the internal medicine department.

Conclusions:

AKU is characterized by the accumulation of homogentisic acid in the connective tissues of many organs including the eye. Patients should be carefully examined in ophthalmology clinics in order to not miss systemic diagnoses. It should be kept in mind that AKU may cause iridocorneal angle pigmentation, which leads to glaucoma, and patients should be treated with proper medication when presenting with elevated intraocular pressure values.
Remodeling an Avascular Bleb
Authors: Gupta Viney , Shaikh Nawazish F. , Gupta Shikha
Published: February 2019

Large avascular blebs need to be repaired especially if they are associated with leaks or have had episodes of bleb-related infections. We describe a new technique of using an amniotic membrane graft, that is inserted into the bleb through a small incision, helping to reduce the size of the avascularity, providing support to the bleb, and preventing extraneous infections.
Effect of Scan Size on Glaucoma Diagnostic Performance Using OCT Angiography En Face Images of the Radial Peripapillary Capillaries
Authors: Chang Ryuna , Chu Zhongdi , Burkemper Bruce , Lee Gary C. , Fard Ali , Durbin Mary K. , Reznik Alena , Xu Benjamin Y. , Kashani Amir , Varma Rohit , Wang Ruikang K. , Richter Grace M.
Published: February 2019

Precis:

When comparing 4.5×4.5 mm to 6.0×6.0 mm optical coherence tomography angiography scans of the radial peripapillary capillaries (RPCs) for glaucoma diagnostic capability, there was a trend of 4.5 scans outperforming 6.0 scans, especially for inferior, nasal, and superior quadrants.

Objectives:

The main purpose of this study was to compare diagnostic ability of peripapillary vessel parameters from 4.5×4.5 mm (4.5) and 6.0×6.0 mm (6.0) spectral-domain optical coherence tomography angiography scans of the RPC in detecting primary open-angle glaucoma from nonglaucoma eyes.

Methods:

Consecutive patients from an academic glaucoma clinic underwent 4.5 and 6.0 scans (CIRRUS HD-OCT 5000 with AngioPlex OCT Angiography; ZEISS, Dublin, CA). Automatic segmentation created en face RPC images. Vessel area density, vessel skeleton density, and flux were calculated using custom quantification software, and perfusion density and flux index (FI) using automated quantification software. Area under the curve statistics included age and hypertension in the analysis.

Results:

Of 173 eyes from 123 patients who underwent both 4.5 and 6.0 imaging, 32 primary open-angle glaucoma eyes from 32 patients and 95 nonglaucoma eyes from 95 patients were studied. For the global region of 4.5 versus 6.0 scans, area under the curve was 0.940 and 0.916 for vessel area density (P=0.286); 0.941 and 0.921 for vessel skeleton density (P=0.385); 0.942 and 0.916 for flux (P=0.239); 0.912 and 0.884 for perfusion density (P=0.103); and 0.913 and 0.865 for FI (P=0.159), respectively. For the quadrant regions, 4.5 images significantly outperformed 6.0 images for the superior and inferior quadrants for flux and superior and nasal quadrants for FI (P-values=0.007, 0.047, 0.011, 0.007, respectively); other quadrant differences were not significant.

Conclusions:

Parameters from 4.5 scans generally outperformed those from 6.0 scans in the global and quadrant regions, suggesting greater digital resolution in 4.5 scans of the immediate peripapillary RPC is important in detecting glaucomatous changes.
Effect of Topical Periocular Steroid Use on Intraocular Pressure: A Retrospective Analysis
Authors: Maeng Michelle M. , De Moraes Carlos Gustavo , Winn Bryan J. , Glass Lora R. Dagi
Published: February 2019

Purpose:

To study the effect of periocular steroid use on intraocular pressure (IOP).

Methods:

Charts of adult patients with atopic dermatitis or eczema treated with topical periocular steroid creams and ointments from January 1st, 2007 to October 1st, 2017 were reviewed. Patients with the following were excluded: glaucoma, ocular hypertension, known systemic/topical/injectable steroid history, and lack of documented IOP prior to or during treatment with periocular steroid ointment. Patient data were collected regarding gender, treatment regimen, as well as IOP prior to and during treatment. Steroid responders were identified. Statistical analysis was performed using linear mixed effects models adjusting for follow-up time to test the relationship between pre and posttreatment IOP change adjusting for intereye correlations.

Results:

Thirty-one patients were identified. Twenty-one were treated bilaterally and 10 unilaterally. Five patients were glaucoma suspects. The mean treatment period was 14.2 weeks with a range of 0.1–83.9 weeks. Patients were treated with fluorometholone (42%), loteprednol etabonate (23%), dexamethasone-neomycin-polymyxin B (13%), hydrocortisone 1% or 2.5% (3%), and tobramycin-dexamethasone (19%). In the combined sample, there was no significant IOP change even after adjusting for follow-up time (mean change: +0.44 mm Hg, p = 0.126). However, eyes with baseline IOP ≥ 14 mm Hg had a significant increase (+0.73 mm Hg/year, p = 0.032). Individual steroid responses included the following: 1 intermediate and 30 low responders, of which 19 patients had an IOP change of <1 mm Hg. One patient had a clinically significant intermediate steroid response of 7 mm Hg.

Conclusions:

Periocular steroid treatment causes a statistically significant rise in IOP in eyes with higher baseline IOP measurements, the risk of which increases with follow up. While this change is not always correlated with a clinically significant rise in IOP, clinicians should monitor more closely patients at greatest risk of steroid response.
Descriptors of Vulvodynia: A Multisocietal Definition Consensus (International Society for the Study of Vulvovaginal Disease, the International Society for the Study of Women Sexual Health, and the International Pelvic Pain Society)
Authors: Bornstein Jacob , Preti Mario , Simon James A. , As-Sanie Sawsan , Stockdale Colleen K. , Stein Amy , Parish Sharon J. , Radici Gianluigi , Vieira-Baptista Pedro , Pukall Caroline , Moyal-Barracco Micheline , Goldstein Andrew
Published: February 2019

Objectives

Three scientific societies, the International Society for the Study of Vulvovaginal Disease (ISSVD), the International Society for the Study of Women Sexual Health (ISSWSH), and the International Pelvic Pain Society (IPPS) developed the “2015 ISSVD, ISSWSH, and IPPS Consensus Terminology and Classification of Persistent Vulvar Pain and Vulvodynia” (referred to as the “2015 consensus terminology”).

The terminology included 11 descriptors of vulvodynia. However, the definitions of the descriptors were not included in the 2015 consensus terminology publications. The objective of this article was to provide these definitions.

Materials and Methods

The ISSVD led a discussion on the definitions for the 11 vulvodynia descriptors, with participation from the ISSWSH and IPPS. The definitions were created through a consensus process.

Results

The definitions are described and the rationale for their choice is elucidated.

Conclusions

The definitions of vulvodynia descriptors were determined by a multistaged process of discussion among health care providers with expertise in the pathophysiology, evaluation, and treatment of vulvodynia. The definitions were approved by the ISSVD, ISSWSH, and IPPS. It is recommended that these definitions of vulvodynia descriptors as well as the 2015 consensus terminology be used for the classification of vulvodynia.
Association of Tympanostomy Tubes With Future Risk of Advanced Ear Surgery—A Population Study
Authors: Beyea Jason A. , Paradis Josée , Nguyen Paul , Hall Stephen F.
Published: February 2019

Objective:

To investigate future surgery for chronic ear disease in children who underwent tympanostomy tube (TT) placement, compared with non-surgically treated patients and healthy controls.

Study Design:

Retrospective population-based cohort study.

Setting:

All hospitals in the Canadian province of Ontario.

Patients/Intervention:

Of children aged 18 years and younger, three cohorts were constructed: 1) TT: patients who had undergone at least one TT procedure (n = 193,880), 2) No-TT: patients with recurrent physician visits for middle ear concerns who did not undergo TT (n = 203,283), and 3) Control: an age-sex matched control group who had not had TT or physician visits for recurrent middle ear concerns (n = 961,168).

Main Outcome Measures:

Risk, and odds ratios (ORs) of surgery for chronic ear disease.

Results:

The TT cohort had a higher risk of tympanoplasty (OR 9.50 versus No-TT, p < 0.001; OR 207.90 versus Control, p < 0.001), ossiculoplasty (OR 3.22 versus No-TT, p < 0.001; OR 84.13 versus Control, p < 0.001), atticotomy (OR 4.41 versus No-TT, p < 0.001; OR 44.78 versus Control, p < 0.001), and mastoidectomy (OR 3.22 versus No-TT, p < 0.001; OR 89.12 versus control, p < 0.001).

Conclusion:

This study describes the population risk of subsequent ear surgeries in TT patients. These TT patients have a significantly higher risk of surgery for chronic ear disease versus those patients with recurrent middle ear disease that did not undergo TT, and age-sex matched controls.
Rubinstein–Taybi syndrome 2 with cerebellar abnormality and neural tube defect
Authors: Hadzsiev Kinga , Gyorsok Zsuzsanna , Till Agnes , Czakó Márta , Bartsch Oliver
Published: February 2019

Rubinstein–Taybi syndrome (RSTS) is a rare dominant disorder with intellectual disability, postnatal growth deficiency, and multiple congenital anomalies. Approximately 50–70% of the patients have a mutation in the CREBBP gene (RSTS1) and 5–10% display an EP300 gene mutation (RSTS2). Craniospinal abnormalities such as microcranium, scoliosis, and lordosis are frequent findings in RSTS1, but malformations of the brain or spinal cord are seen only occasionally. Here, we report on a 3-year-old boy with facial abnormalities of RSTS, broad thumbs and halluces, developmental delay, autistic features, cerebellar underdevelopment, and a neural tube defect. Molecular diagnostic of the CREBBP and EP300 genes showed a heterozygous 17-bp deletion (c.5698_5714del AAGGCAGCAGGCCAGGT) in exon 31 of the EP300 gene. Findings underline that small (hypoplastic) cerebellum and neural tube defects belong to the phenotypic spectrum not only of RSTS1 but also of RSTS2. Based on the literature and this observation, we recommend that each individual with RSTS2 should be closely evaluated for neural axis and craniovertebral junction anomalies, and where appropriate, neuroimaging studies should be considered. Our frequency estimate of ~ 6% occult or overt neural tube defects in RSTS2 could represent an underestimate.
Real-time estimation of inpatient beds required in emergency departments
Authors: Noel Guilhem , Bonte Nicolas , Persico Nicolas , Bar Christian , Luigi Stéphane , Roch Antoine , Michelet Pierre , Gentile Stéphanie , Viudesa Gilles
Published: February 2019

Background

Long boarding time in emergency department (ED) leads to increased morbidity and mortality. Prediction of admissions upon triage could improve ED care efficiency and decrease boarding time.

Objective

To develop a real-time automated model (MA) to predict admissions upon triage and compare this model with triage nurse prediction (TNP).

Patients and methods

A cross-sectional study was conducted in four EDs during 1 month. MA used only variables available upon triage and included in the national French Electronic Emergency Department Abstract. For each patient, the triage nurse assessed the hospitalization risk on a 10-point Likert scale. Performances of MA and TNP were compared using the area under the receiver operating characteristic curves, the accuracy, and the daily and hourly mean difference between predicted and observed number of admission.

Results

A total of 11 653 patients visited the EDs, and 19.5–24.7% were admitted according to the emergency. The area under the curves (AUCs) of TNP [0.815 (0.805–0.826)] and MA [0.815 (0.805–0.825)] were similar. Across EDs, the AUCs of TNP were significantly different (P < 0.001) in all EDs, whereas AUCs of MA were all similar (P>0.2). Originally, using daily and hourly aggregated data, the percentage of errors concerning the number of predicted admission were 8.7 and 34.4%, respectively, for MA and 9.9 and 35.4%, respectively, for TNP.

Conclusion

A simple model using variables available in all EDs in France performed well to predict admission upon triage. However, when analyzed at an hourly level, it overestimated the number of inpatient beds needed by a third. More research is needed to define adequate use of these models.
Correlation of Serum and Ascitic Fluid Soluble Form Urokinase Plasminogen Activator Receptor Levels With Patient Complications, Disease Severity, Inflammatory Markers, and Prognosis in Patients With Severe Acute Pancreatitis
Authors: Long Ding , Wang Yujun , Wang Hui , Wu Xiaoling , Yu Li
Published: February 2019

Objectives

The aim of the study was to investigate the correlation of serum and ascitic fluid soluble form urokinase plasminogen activator receptor (suPAR) levels with patients' complications, disease severity, inflammatory markers, and prognosis in patients with severe acute pancreatitis (SAP).

Methods

Fifty patients with SAP, 47 patients with mild acute pancreatitis, and 50 healthy controls were enrolled. Serum samples were obtained from all participants after enrollment; meanwhile, ascitic fluid samples were collected from 20 patients with SAP who developed ascites. Serum and ascitic fluid suPAR levels were determined by enzyme-linked immunosorbent assay.

Results

Serum suPAR level was greatly elevated in patients with SAP than patients with mild acute pancreatitis and healthy controls. Receiver operating characteristic curve showed that serum suPAR presented with good value in predicting risk of pancreatic necrosis, pancreatic infection, and multiple organ dysfunction syndrome, whereas serum suPAR did not predict mortality. Serum suPAR level was also positively correlated with Acute Physiology and Chronic Health Evaluation II score, Balthazar index, and Sequential Organ Failure Assessment score. As to ascitic fluid suPAR, it was positively correlated with serum suPAR level, Acute Physiology and Chronic Health Evaluation II score, Sequential Organ Failure Assessment score, risk of pancreatic infection, and multiple organ dysfunction syndrome.

Conclusions

Serum and ascetic fluid suPAR levels could be served as markers for disease severity and risk of severe complications in patients with SAP.
Percutaneous Drainage Versus Peritoneal Lavage for Pancreatic Ascites in Severe Acute Pancreatitis
Authors: He Wen-Hua , Xion Zhi-Juan , Zhu Yin , Xia Liang , Zhu Yong , Liu Pi , Zeng Hao , Zheng Xi , Lei Yu-Peng , Huang Xin , Zhu Xuan , Lv Nong-Hua
Published: February 2019

Objectives

This study aimed to compare the efficacy of percutaneous drainage (PCD) versus peritoneal lavage (PL) for the treatment for severe acute pancreatitis patients with pancreatic ascites (PAs).

Methods

Severe acute pancreatitis patients with PAs were randomly assigned within 3 days of onset of symptoms to receive either PL or PCD. The primary end point was a composite of mortality or major complications during hospitalization and within 1 month of discharge. Per-protocol analyses were performed.

Results

Between September 2011 and June 2014, 86 patients were randomly assigned to intervention with PL or PCD. Ultimately, 41 patients in the PCD group and 39 patients in the PL group completed the study. The primary end point occurred in 15 (36.6%) of 41 patients in the PCD group and in 17 (43.6%) of 39 patients in the PL group (risk ratio, 0.84; 95% confidence interval, 0.49–1.44; P = 0.27). Mortality or major complications did not differ between the groups. Percutaneous drainage reduced intra-abdominal hypertension; however, PL reduced the incidence of deep venous thrombosis and pancreatic encephalopathy and was associated with a reduced need for intervention.

Conclusions

In our study, the PCD was not superior to the PL in reducing mortality or major complications in severe acute pancreatitis patients with PAs.
Prevalence of Low Bone Density and Comorbid Hypogonadism in Patients With Chronic Pancreatitis
Authors: Gupta Namita , Singh Shailender , Vargas Luciano , Moore Timothy E. , Shostrom Valerie K. , Boerner Brian P.
Published: February 2019

Objectives

Patients with chronic pancreatitis (CP) are at increased risk of low bone mineral density (BMD), although the prevalence of low BMD in patients with CP in the United States is lacking. We aimed to determine the prevalence of low BMD and identify potential risk factors, including hypogonadism and use of opioid medications, in subjects with CP in the United States.

Methods

This was a prospective, observational study. Subjects with CP underwent dual-energy x-ray absorptiometry scan. Blood was assayed for vitamin D, sex hormones, and a metabolic panel. History was obtained for fractures, menopause, hypogonadal symptoms, and opioid medication doses. Low BMD was defined by both World Health Organization and the International Society for Clinical Densitometry criteria.

Results

Depending on criteria used, 37% to 55% of our cohort had low BMD. Subjects with low and normal BMD had similar vitamin D levels. Hypogonadism was present in 27% of nonmenopausal subjects and was associated with reduced lumbar spine BMD in subjects 30 years or older.

Conclusions

Patients with CP are at increased risk of low BMD, which is likely multifactorial. Hypogonadism, possibly related to opioid pain medications, may be an independent risk factor for low BMD in CP.
A Phase II Trial of Ziv-Aflibercept in Patients With Advanced Pancreatic Neuroendocrine Tumors
Authors: Halperin Daniel M. , Lee J. Jack , Ng Chaan S. , Strosberg Jonathan R. , Estrella Jeannelyn S. , Dagohoy Cecile G. , Dasari Arvind , Yao James C.
Published: February 2019

Objectives

Therapies for patients with advanced well-differentiated pancreatic neuroendocrine tumors (pNETs) are insufficient, with patients succumbing to disease despite recent treatment advances. Ziv-aflibercept is a fusion protein of portions of the vascular endothelial growth factor (VEGF) receptors 1 and 2, fused to the Fc portion of immunoglobulin G1, forming a VEGF trap. Perfusion computed tomography (CT) has previously defined hyperperfused neuroendocrine tumors, potentially predicting antiangiogenic benefit.

Methods

We performed a single-arm open-label study, using the Simon optimal 2-stage design, of 6 mg/kg ziv-aflibercept in patients with advanced pNETs. The primary end point was objective radiographic response, with a hierarchically tested co–primary end point of response prediction by baseline hyperperfusion, defined as blood volume greater than 5.25 mL/100 g and permeability surface area product greater than 25 mL/min per 100 g.

Results

Between July 3, 2014, and September 28, 2016, 21 patients were treated. Two patients (9.5%; 95% confidence interval, 1.1%–30.4%) demonstrated objective response, satisfying criteria to open the second stage, but the study was terminated for accrual. Perfusion CT could not be confirmed to predict radiographic response. Toxicities include 1 grade 5 gastrointestinal hemorrhage and 5 incidents of proteinuria requiring treatment discontinuation.

Conclusions

Responses with ziv-aflibercept were consistent with other VEGF inhibitors in pNET, but perfusion CT could not be confirmed to predict outcome.
Plasma Pancreastatin Predicts the Outcome of Surgical Cytoreduction in Neuroendocrine Tumors of the Small Bowel
Authors: Woltering Eugene A. , Voros Brianne A. , Beyer David T. , Thiagarajan Ramcharan , Ramirez Robert A. , Mamikunian Gregg , Boudreaux J. Philip
Published: February 2019

Objectives

Elevated pancreastatin (PST) levels have been shown to be associated with poor prognosis in small bowel neuroendocrine tumors (NETs). We hypothesized that plasma PST levels that remain elevated following surgical cytoreduction portend a poor prognosis in well-differentiated small bowel NETs.

Methods

Patients diagnosed with small bowel NETs who underwent surgical cytoreduction at our institution were identified. Demographics, histopathologic characteristics, and biochemical data were collected. Only patients who had serial preoperative PST (PreopPST) and postoperative PST (PostopPST) levels were included in this study. Patients were sorted into groups by PST level to assess their response to surgical cytoreduction (group 1, PreopPST/PostopPST normal; group 2, PreopPST elevated/PostopPST normal; group 3, PreopPST/PostopPST elevated). Survival rates were calculated from the date of surgery.

Results

PreopPST and PostopPST levels were collected from 300 patients. Patients in groups 1 (n = 74) and 2 (n = 81) had a significant survival advantage compared with patients in group 3 (n = 145) (P < 0.0001). Kaplan-Meier 5- and 10-year survival rates were as follows: group 1: 93% and 82%; group 2: 91% and 65%; and group 3: 58% and 34%, respectively.

Conclusions

Serial monitoring of plasma PST is useful in predicting long-term survival following surgical cytoreduction and can be helpful to identify patients who have a poor prognosis.
Blood Urea Nitrogen Elevation Is a Marker for Pediatric Severe Acute Pancreatitis
Authors: Vitale David S. , Hornung Lindsey , Lin Tom K. , Nathan Jaimie D. , Prasad Swathi , Thompson Tyler , Abu-El-Haija Maisam
Published: February 2019

Objective

The natural course of patients who develop acute pancreatitis (AP) can range from mild to severe acute pancreatitis (SAP). The aim of this study was to evaluate for early predictors of developing SAP during the first episode of AP in a prospective pediatric cohort.

Methods

Clinical data were prospectively collected and subsequently analyzed for pediatric patients presenting with their first episode of AP between March 2013 and January 2017 to Cincinnati Children's Hospital Medical Center.

Results

A total of 118 patients were included in the analysis, and 22 (18.6%) developed SAP. Patients who developed SAP had significantly higher values of blood urea nitrogen (BUN) (P = 0.007), magnesium (P = 0.04), glucose (P = 0.03), sodium (P = 0.03), and C-reactive protein (P = 0.02). A logistic regression model with BUN as a predictor of SAP was superior to any other combination of variables (area under the receiver operating characteristic curve, 0.75; 95% confidence interval, 0.61–0.89; sensitivity, 63%; specificity, 81%; positive predictive value, 43%; negative predictive value, 91%).

Conclusions

This study generated a predictive model using elevated BUN as a significant predictor of SAP. The findings are useful for early identification of pediatric patients at higher risk of developing SAP.
Effect of Ketoprofen and ATB‐352 on the Immature Human Intestine
Authors: Thibault Marie‐Pier , Tremblay Éric , Wallace John L. , Beaulieu Jean‐François
Published: February 2019

Background and Objective:

The use of nonsteroidal anti‐inflammatory drugs (NSAIDs) is associated with a broad spectrum of life‐threatening adverse effects on the immature gastrointestinal tract. NSAID derivatives exploiting the beneficial effects of biologically active gases, such as hydrogen sulfide (H2S), have been developed. Herein, we determined the effects of ketoprofen and ATB‐352, a H2S‐releasing ketoprofen derivative, on selected metabolic pathways previously identified to be significantly altered by indomethacin in the human immature intestine.

Methods:

Ketoprofen and ATB‐352 were tested on human mid‐gestation small intestinal explants maintained in a serum‐free organ culture system for 48 hours. The expression levels of the representative genes involved in selected metabolic pathways were measured by real‐time PCR after a treatment of 48 hours.

Results:

Tested at a concentration that allows more than 80% inhibition of PGE2 production, ketoprofen was found to be less damaging than indomethacin at an equivalent dosage. However, based on the inducibility of cyclooxygenase‐2 transcript expression, we were able to discriminate between responder individuals in which the deleterious effects observed with indomethacin were attenuated, and non‐responder specimens in which the effects were similar to those observed with indomethacin. ATB‐352 did not induce significant changes compared to ketoprofen on these metabolic pathways.

Conclusions:

These results show less damaging effects of ketoprofen compared to indomethacin on the immature intestine and indicate that the intestinal response to this NSAID significantly varies between individuals. However, the results did not allow us to demonstrate a specific beneficial effect of H2S release in organ culture.
Pediatric Endoscopy Practice Patterns in the United States, Canada, and Mexico
Authors: Barakat Monique T. , Triadafilopoulos George , Berquist William E.
Published: February 2019

Background and Aims:

Endoscopic procedures are important for diagnosis and management of many gastrointestinal, liver, and biliary conditions in children. Therapeutic endoscopy procedures, including endoscopic retrograde cholangiopancreatography (ERCP), are performed less frequently in children relative to adults. A formal study to evaluate institutional volumes and practice patterns for advanced therapeutic pediatric endoscopy procedures has, however, not been previously undertaken.

Methods:

A self‐administered 16‐question (5‐minute) online survey assessing practice patterns for performance of pediatric endoscopy procedures was distributed to all registered North American Society for Pediatric Gastroenterology, Hepatology and Nutrition programs. Results were analyzed using descriptive statistics and thematic analysis of free‐text comments.

Results:

Respondents from 82.9% of North American Society for Pediatric Gastroenterology, Hepatology and Nutrition centers completed this survey. Responses revealed that esophagogastroduodenoscopy/colonoscopy are performed at the vast majority of centers (>90%), with most performing >50/year. Therapeutic endoscopy procedures are performed less frequently in the pediatric population, with 18.97% reporting that ERCP is not performed at their institution. Where ERCP is performed, 91.38% reported <25/year. Endoscopic ultrasound is not performed at more than half (53.33%) of institutions. Approximately 71.67% of respondents do not believe their institution's current arrangement for performing pediatric therapeutic endoscopy procedures is adequate.

Conclusions:

Although the range of endoscopic procedures performed in children parallels that performed in adults, there are notable differences in pediatric and adult gastroenterologists’ endoscopy training and procedure volumes. Our results and respondent comments suggest that pediatric patients would benefit from a partnership between pediatric and adult gastroenterologists, with adult gastroenterologists performing more complex therapeutic endoscopic procedures.
Anti‐Saccharomyces cerevisiae Antibodies as a Prognostic Biomarker in Children With Crohn Disease
Authors: Chandrakumar Abin , Georgy Michael , Agarwal Prasoon , ‘t Jong Geert W. , El‐Matary Wael
Published: February 2019

Objective:

Although anti‐Saccharomyces cerevisiae antibodies (ASCAs) could be a useful biomarker in differentiating Crohn disease (CD) from ulcerative colitis (UC), their role as prognostic markers in children with CD has been underinvestigated. This longitudinal prospective observational study aimed to assess the prognostic value of ASCA status among children with CD managed using biologics.

Methods:

The study population comprised children with inflammatory bowel disease diagnosed with CD from 2012 to 2018. Cox regression model with adjustment for a priori covariates was used to examine the response to anti‐tumor necrosis factor (TNF) biological therapy among ASCA‐positive patients in comparison to ASCA‐negative patients.

Results:

There were 273 measurements available from the study cohort comprising children with CD, who were followed up for a median duration of 14 months (interquartile range 5–42). ASCA‐positive patients had a higher risk for moderate to severe clinical disease (odds ratio 2.88; 95% confidence interval [CI] 1.2–7.55) and extensive endoscopic distribution (odds ratio 3.30; CI 1.12–9.74) at baseline in comparison to ASCA‐negative patients, respectively. In comparison to ASCA immunoglobulin G (IgG)‐negative patients, ASCA IgG–positive patients who were treated with biologics had a significantly lower relapse rate (adjusted hazard ratio 0.12; CI 0.02–0.93). Ten (14%) patients had an unstable ASCA value with either ASCA immunoglobulin A or ASCA IgG status changing from positive to negative or vice versa.

Conclusions:

ASCA‐positive children with CD present with more extensive (endoscopic) and clinically severe disease. ASCA IgG is a useful prognostic marker among children with CD who receive biologics.
Neuroimaging Abnormalities in Patients With Nontransfusion-dependent Thalassemia
Authors: Ramamoorthy Jaikumar , Trehan Amita , Ahluwalia Jasmina , Singh Paramjeet , Manoj Rohit , Bansal Deepak
Published: February 2019

Independence from regular transfusions is the hallmark of nontransfusion-dependent thalassemia (NTDT). However, the associated complications need anticipation and screening. One such complication is a hypercoagulable state predisposing to development of thrombosis. We evaluated children with NTDT >10 years of age for prevalence of neuroimaging abnormalities (NIA) and identified associated risk factors. In total, 29 patients were evaluated. Blood counts, serum ferritin, protein C, protein S, antithrombin III, brain magnetic resonance imaging, and angiography was done in all patients. Possible risk factors for thrombosis or cerebrovascular disease were analyzed for association with NIA. The median age was 14 (12 to 15) years. Fifty percent were splenctomized and 31.5% were transfusion naïve. Eleven patients (37.9%) had NIA: 6 with silent cerebral infarction (SCI); 2 with cerebral arteriopathy (CA) and 3 having both CA and SCI. Higher white blood cell (WBC) count was associated with NIA (P=0.034) [silent cerebral infarction (P=0.047) and cerebral arteriopathy (P=0.067)]. Presence of 7 or more risk factors had 4.5 times greater risk of a NIA, especially silent cerebral infarction (SCI) (P=0.03). We conclude that cerebral infarction and arteriopathy seem to start in late childhood. There is a need to develop strategies for preventing neurologic complications in NTDT similar to sickle cell disease.
Parent and Guardian Knowledge of Hematopoietic Cell Transplantation as a Treatment Option for Sickle Cell Disease
Authors: Stallings Alicia M. , Majhail Navneet S. , Nowacki Amy S. , Onimoe Grace I. , Hanna Rabi , Wen Jillian , Umana Jasmine , Quinn Charles T. , Hsu Lewis L. , Piccone Connie M.
Published: February 2019

Beginning early in childhood, patients with sickle cell disease (SCD) are at risk of life-threatening and debilitating health events. Despite the high morbidity and mortality of this disease, hematopoietic cell transplantation (HCT), a curative treatment for SCD, remains underutilized. In the literature there is a paucity of data concerning medical decision maker (MDM) awareness of HCT as a treatment option for SCD. The objective of this study was to estimate the proportion of parents/guardians of children with SCD who are aware of HCT as a treatment option, and to identify the demographic factors associated with knowledge of this therapy’s curative potential. Between November 2015 and December 2016, 327 parents/guardians were surveyed across 4 clinical sites in 3 Midwestern US cities. Although 82% of parents/guardians had heard of HCT in the past and 78% were aware of the therapy’s curative potential, nearly half indicated that they did not know whether HCT could specifically cure their child of the disease. Respondents who had discussed HCT with their child’s physician had 5 times higher odds of being aware of HCT’s curative potential than those who had not. These findings suggest that additional efforts to enhance MDM knowledge of HCT as well as shared decision making in the use of this therapy, is warranted.
Levels of Innovativeness Among Nurse Leaders in Acute Care Hospitals
Authors: Stilgenbauer Deborah J. , Fitzpatrick Joyce J.
Published: February 2019

OBJECTIVE

The purposes of the study were to describe levels of innovativeness in nurse executives, clinical directors, and nurse managers in acute care settings in the United States and to compare innovativeness between the groups.

BACKGROUND

Nurse leaders must navigate the complex ever-changing landscape of healthcare. New strategies are necessary for managing resources and improving patient outcomes.

METHODS

A survey was posted in the American Organization of Nurse Executives newsletter using the Scales for Measurement of Innovativeness to measure innovativeness. The sample included 137 nurse leaders in executive and management positions in acute care settings.

RESULTS

The largest percentage of nurse leaders across all leadership roles fell into the innovativeness category of early majority. Of the total sample, only 2 individuals were categorized as innovators, and only 15 as early adopters. Fifty-one individuals were early majority; 49 were in the late majority; and 20 individuals were at the lowest category of innovativeness, that of laggards. Both nurse executives and clinical directors had significantly higher levels of innovativeness compared with nurse managers. Graduate-level education and certification were not associated with higher levels of innovativeness.

CONCLUSIONS

Understanding the levels and components of innovativeness may assist nurse leaders to affect change in themselves and their organizations.
Pain Assessment for Nursing Home Residents
Authors: Dirk Kennedy , Rachor Geoffrey S. , Knopp-Sihota Jennifer A.
Published: February 2019

Background

The burden of pain in nursing homes is substantial; however, pain assessment for both acute and chronic conditions remains inadequate, resulting in inappropriate or inadequate treatment. Complexities in assessing resident pain have been attributed to factors (barriers and facilitators) arising at the resident, healthcare provider, and healthcare system levels.

Objectives

In this systematic review protocol, we identify our research approach that will be used to critically appraise and synthesize data in order to assess barriers and facilitators to pain assessment in nursing home residents aged ≥65 years.

Methods

This is a Cochrane style systematic review protocol adhering to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses-Protocols reporting standards. This review will include primary (original) qualitative literature concerning either barriers or facilitators to pain assessment in older adult nursing home residents. A thematic analysis approach will be employed in collating and summarizing included data and will be categorized into resident, healthcare provider, and system-level factors. Database searches will include Abstracts in Social Gerontology, CINAHL, Cochrane Central Register of Controlled Trials, Embase, MEDLINE, and Web of Science.

Discussion

The identification of barriers and facilitators to pain assessment in older adult nursing home residents may assist healthcare providers across all platforms and levels of education to improve pain assessment among nursing home residents. Improving the assessment of pain has the potential to improve quality of care and ultimately quality of life for older adult nursing home residents.
Maternal Adverse Childhood Experiences, Family Strengths, and Chronic Stress in Children
Authors: Condon Eileen M. , Holland Margaret L. , Slade Arietta , Redeker Nancy S. , Mayes Linda C. , Sadler Lois S.
Published: February 2019

Background

Researchers have demonstrated that maternal adverse childhood experiences (ACEs), such as abuse and neglect, are associated with prenatal risk factors and poor infant development. However, associations with child physiologic and health outcomes, including biomarkers of chronic or “toxic” stress, have not yet been explored.

Objectives

The purpose of this study was to examine the associations among past maternal experiences, current maternal posttraumatic stress disorder (PTSD) symptoms, and children's indicators of exposure to chronic stress in a multiethnic sample of mothers and children at early school age (4 to 9 years).

Methods

This cross-sectional study included maternal–child dyads (N = 54) recruited from urban community health centers in New Haven, Connecticut. Mothers reported history of ACEs, family strengths, and current PTSD symptoms. Child measures included biomarkers and health and developmental outcomes associated with chronic stress. Correlational and regression analyses were conducted.

Results

Childhood trauma in mothers was associated with higher systolic blood pressure percentile (ρ = .29, p = .03) and behavioral problems (ρ = .47, p = .001) in children, while maternal history of family strengths was associated with lower salivary interleukin (IL)-1β (ρ = −.27, p = .055), salivary IL-6 (ρ = −.27, p = .054), and body mass index z-scores (ρ = −.29, p = .03) in children. Maternal PTSD symptoms were associated with more child behavioral problems (ρ = .57, p < .001) and higher odds of asthma history (ρ = .30, p = .03).

Discussion

Results indicate that past maternal experiences may have important influences on a child's health and affect his or her risk for experiencing toxic stress.
Rebury the “Atavistic Skull” Studied by Lombroso?
Authors: Ciliberti Rosagemma , Armocida Giuseppe , Licata Marta
Published: February 2019

On May 16, 2017, the judgment of the Italian court ended the legal battle concerning the repatriation request of the famous skull, belonging to the “brigand” Giuseppe Villella. During the autopsy examination on the corpse of Villella, Lombroso observed a median occipital dimple on the skull, a feature visible in other mammals, including primates, but absent in humans. This feature could demonstrate an anomalous dimension of the median lobe of Villella's cerebellum. From this anatomical finding, Lombroso consolidated the atavism theory, which established a close connection between morphological features and behavior.

The Lombroso Museum and the University of Turin reiterated the legitimacy of the possession of the skull as cultural property in accordance with the Code of Cultural Heritage and as a find of forensic psychopathology. Finally, the court rejected the request from Villella's hometown, highlighting that the scientific theory is unquestionably invalid, but his existence cannot be denied.

The “special” nature of human remains emerges from the special way in which the Code of Ethics of the International Council of Museums treats them, which also implies respect to the other material of the museum collections.
Diagnostic Accuracy in Fit-for-Purpose PD-L1 Testing
Authors: Cheung Carol C. , Lim Hyun J. , Garratt John , Won Jennifer , Gilks C. Blake , Torlakovic Emina E.
Published: February 2019

PD-L1 testing by immunohistochemistry (IHC) has presented significant challenges not only for clinical laboratories, but also for external quality assurance (EQA) entities that provide proficiency testing (PT) for clinical laboratories. Canadian Immunohistochemistry Quality Control (CIQC) has used educational runs to explore approaches to sample design and analysis of results that would enhance patient safety. As PT for predictive biomarkers requires modeling at every level (design of the run, assessment of the run, and reporting of “pass” or “fail”) based on “fit-for-purpose” principles, CIQC has applied those principles to PD-L1 PT runs. Each laboratory received unstained slides with TMA tissue cores from 104 randomly selected primary NSCLC and tonsil tissues to test with their current PD-L1 assay. Diagnostic sensitivity and specificity were calculated against designated gold standards based on the “3D” approach (drug-disease-diagnostic assay). Depending on the selection of fit-for-purpose gold standards and also on the selection of what was considered fit-for-purpose cut-off points, great variation in the performance (accuracy) of both companion/complementary diagnostic assays and laboratory developed tests was seen. “Fit-for-purpose” in PT for PD-L1 testing entails that the purpose(s) of each PT run is declared a priori, that the PT program has selected/designated purpose-specific gold standard results for the PT challenge, and that the PT materials for the PT run are designed and constructed to enable calculations of diagnostic accuracy.
SOX11-negative Mantle Cell Lymphoma
Authors: Xu Jie , Wang Lifu , Li Jingyi , Saksena Annapurna , Wang Sa A. , Shen Jing , Hu Zhihong , Lin Pei , Tang Guilin , Yin C. Cameron , Wang Michael , Medeiros L. Jeffrey , Li Shaoying
Published: February 2019

Studies have suggested that SOX11 expression has prognostic implications in patients with mantle cell lymphoma (MCL), but the data are controversial. In this study, we describe the clinicopathologic and prognostic features of 75 patients with SOX11-negative MCL. Compared with patients with SOX11-positive MCL, SOX11-negative MCL patients more frequently had leukemic non-nodal disease (21% vs. 4%, P=0.0001). SOX11-negative MCLs more often showed classic morphology (83% vs. 65%, P=0.005), were more often positive for CD23 (39% vs. 22%, P=0.02) and CD200 (60% vs. 9%, P=0.0001), and had a lower proliferation index (Ki67 23% vs. 33%, P=0.04). Overall survival (OS) was not significantly different between patients with SOX11-negative versus SOX11-positive MCL (P=0.63). High Ki67 index and blastoid/pleomorphic morphology were associated with shorter OS in both SOX11-negative (P<0.05) and SOX11-positive MCL groups (P<0.05). A high Mantle Cell Lymphoma International Prognostic Index (MIPI) predicted poorer prognosis in patients with SOX11-negative MCL (P<0.0001), but not SOX11-positive MCL (P=0.09). Nodal involvement and stage III/IV disease were associated with poorer outcome in patients with SOX11-positive MCL (P=0.03 and 0.04, respectively), but not SOX11-negative MCL (P=0.88 and 0.74, respectively). In summary, SOX11-negative MCL is characterized by more frequent leukemic non-nodal disease, classic morphology, more frequent expression of CD23 and CD200, and a lower Ki67 index. Prognostic factors in patients with SOX11-negative MCL include morphology, Ki67 index, and MIPI score.
Lymphangioleiomyoma of the Uterus and Pelvic Lymph Nodes: A Report of 3 Cases, Including the Potentially Earliest Manifestation of Extrapulmonary Lymphangioleiomyomatosis
Authors: Ando Hiroka , Ogawa Minori , Watanabe Yuko , Tsurunaga Kanako , Nakamura Chie , Tamura Hiromi , Ikeda Kayo , Takahashi Ryoko , Nakajima Reina , Yoshida Susumu , Tsujie Tomoko , Doi Reiko , Wakimoto Akinori , Adachi Shiro
Published: February 2019

We present 3 cases of extrapulmonary lymphangioleiomyomatosis (LAM) identified incidentally in the uterine corpus and pelvic nodes resected for other reasons. One patient, a 47-yr-old female with corpus cancer, underwent a total hysterectomy and nodal dissection; the other 2 patients, aged 44 and 49 yr, underwent simple hysterectomy for corpus leiomyomas. None of the patients had evidence of tuberous sclerosis complex or any significant lesions in other organs. An area of spindle cell proliferation, intimately associated with dilated and tortuous lymphatic vessels, was found in the myometrium of all 3 patients, and nodal involvement with spindle cell proliferation was observed in the patient with corpus cancer. The spindle cells had faintly eosinophilic cytoplasm and a bland appearance. They were immunoreactive for α-SMA, gp100 (HMB45), and Melan-A. Tumor cell clusters lined with a single layer of lymphatic endothelium were floating in the lymphatic vessel lumen. These lesions were diagnosed as lymphangioleiomyoma in the uterine corpus and associated lymph nodes. Two of the cases seemed to be the earliest manifestations of extrapulmonary LAM, and the other case represents early-phase metastasis of LAM from the uterus. The present cases support the speculation that the uterus is the primary source of LAM cells.
Structural Gender Dimorphism and the Biomechanics of the Gluteal Subcutaneous Tissue: Implications for the Pathophysiology of Cellulite
Authors: Rudolph Christina , Hladik Casey , Hamade Hassan , Frank Konstantin , Kaminer Michael S. , Hexsel Doris , Gotkin Robert H. , Sadick Neil S. , Green Jeremy B. , Cotofana Sebastian
Published: February 2019

Background:

This study was performed to investigate gender differences in gluteal subcutaneous architecture and biomechanics to better understand the pathophysiology underlying the mattress-like appearance of cellulite.

Methods:

Ten male and 10 female body donors [mean age, 76 ± 16.47 years (range, 36 to 92 years); mean body mass index, 25.27 ± 6.24 kg/m2 (range, 16.69 to 40.76 kg/m2)] were used to generate full-thickness longitudinal and transverse gluteal slices. In the superficial and deep fatty layers, fat lobule number, height, and width were investigated. The force needed to cause septal breakage between the dermis and superficial fascia was measured using biomechanical testing.

Results:

Increased age was significantly related to decreased dermal thickness, independent of sex (OR, 0.997, 95 percent CI, 0.996 to 0.998; p < 0.0001). The mean number of subdermal fat lobules was significantly higher in male body donors (10.05 ± 2.3) than in female body donors (7.51 ± 2.7; p = 0.003), indicating more septal connections between the superficial fascia and dermis in men. Female sex and increased body mass index were associated with increased height of superficial fat lobules. The force needed to cause septal breakage in male body donors (38.46 ± 26.3 N) was significantly greater than in female body donors (23.26 ± 10.2 N; p = 0.021).

Conclusions:

The interplay of dermal support, septal morphology, and underlying fat architecture contributes to the biomechanical properties of the subdermal junction. This is influenced by sex, age, and body mass index. Cellulite can be understood as an imbalance between containment and extrusion forces at the subdermal junction; aged women with high body mass index have the greatest risk of developing (or worsening of) cellulite.