bluuebunny/crossref_metadata_2025 huggingface dataset:
The Association of Recently Diagnosed Diabetes and Long-term Diabetes With Survival in Pancreatic Cancer Patients
Authors: Jeon Christie Y. , Li Donghui , Cleary Sean , Stolzenberg-Solomon Rachael , Bosetti Cristina , La Vecchia Carlo , Porta Miquel , Toriola Adetunji T. , Hung Rayjean J. , Kurtz Robert C. , Olson Sara H.
Published: February 2018

Objectives

It is unclear whether long-standing diabetes or new-onset pancreatogenic diabetes contributes to poor prognosis in patients with pancreatic ductal adenocarcinoma (PDAC).

Methods

We investigated the influence of diabetes diagnosed shortly before PDAC and long-term diabetes on overall survival in 2792 PDAC patients who had participated in 3 PDAC case-control studies in the Pancreatic Cancer Case-Control Consortium. There were 300 patients with long-term diabetes of more than 3 years' duration (11%) and 418 patients with recently diagnosed diabetes of 3-year duration or less (15%). We performed Cox regression to determine the association of long-term diabetes and recently diagnosed diabetes with overall survival, adjusting for study site, age, sex, race, stage of disease, surgery, chemotherapy, smoking history, and body mass index at diagnosis.

Results

In the overall population, neither long-term diabetes (hazard ratio [HR], 1.10; 95% confidence interval [CI], 0.97–1.26) nor recently diagnosed diabetes (HR, 1.06; 95% CI, 0.94–1.18) was associated with shorter survival. When stratified by stage of disease, long-term diabetes was associated with 42% increase in rate of death in persons with resectable PDAC (HR, 1.42; 95% CI, 1.13–1.78), whereas it was not associated with survival in PDAC patients with more advanced disease.

Conclusion

Long-term diabetes was associated with increased rate of death in patients with resectable PDAC.
Primary and Secondary Organ Failures Cause Mortality Differentially in Acute Pancreatitis and Should be Distinguished
Authors: Padhan Rajesh Kumar , Jain Saransh , Agarwal Samagra , Harikrishnan Suresh , Vadiraja Padmaprakash , Behera Sanatan , Jain Sushil Kumar , Dhingra Rajan , Dash Nihar Ranjan , Sahni Peush , Garg Pramod Kumar
Published: February 2018

Objective

The aim of this study was to study the development of early and late organ failure (OF) and their differential impact on mortality in patients with acute pancreatitis (AP).

Methods

Consecutive patients (N = 805) with acute pancreatitis were included in an observational study. Organ failure was categorized as primary if it occurred early due to pancreatitis per se and secondary if it occurred late due to infected pancreatic necrosis (IPN). Primary outcome was a relative contribution of primary OF, secondary OF, and IPN to mortality.

Results

Of the 614 patients (mean age, 38.8; standard deviation, 14.6 years; 430 males) in a derivation cohort, 274 (44.6%) developed OF, with 177 having primary OF and 97 secondary OF due to sepsis. Primary OF caused early mortality in 15.8% and was a risk factor for IPN in 76% of patients. Mortality in patients with primary OF and IPN was 49.5% versus 36% in those with IPN and secondary OF (P = 0.06) and 4% in those with IPN but without OF (P < 0.001). The results of the 191 patients in the validation cohort confirmed the relative contribution of primary and secondary OF to mortality.

Conclusion

Primary and secondary OF contributed to mortality independently and are distinct in their timing, window of opportunity for intervention, and prognosis.
The 2012 International Consensus Guidelines of Intraductal Papillary Mucinous Neoplasms of the Pancreas (Fukuoka Criteria) Predict the Malignant Potential, Even in Actual Clinical Situations
Authors: Okamura Yukiyasu , Sano Shusei , Sugiura Teiichi , Ito Takaaki , Yamamoto Yusuke , Ashida Ryo , Ishiwatari Hirotoshi , Matsubayashi Hiroyuki , Sasaki Keiko , Uesaka Katsuhiko
Published: February 2018

Objectives

The aim of the present study was to validate the use of the 2012 International Consensus Guidelines for intraductal papillary mucinous neoplasm (IPMN) (Fukuoka criteria) in patients without findings of obstructive jaundice and/or an enhanced solid component.

Methods

This retrospective study included patients who underwent pancreatectomy for IPMN between October 2002 and September 2016. The sensitivity, specificity, and positive and negative predictive values for malignancy were calculated for each feature of high-risk stigmata.

Results

Of the 135 patients who underwent surgery for IPMN, 50 (37.0%) had low/intermediate-grade dysplasia, 25 (18.5%) had high-grade dysplasia, and the remaining 60 (44.5%) had invasive carcinoma. A malignant potential was, thus, present in 63% of all resected cases. Considering an actual clinical situation, the predictors for malignant potential were assessed in 62 patients without findings of obstructive jaundice and/or an enhanced solid component. A multivariate analysis revealed that the Fukuoka criteria were the only independent factor for predicting the malignant potential (odds ratio, 5.69; P = 0.036).

Conclusions

The present study suggested that malignant potential can be accurately detected by faithfully keeping to the Fukuoka criteria. Using these criteria enables us to identify patients with malignant potential, even in the actual clinical situations.
Effect of Reprimo Down-regulation on Malignant Transformation of Intraductal Papillary Mucinous Neoplasm
Authors: Nakazato Tetsuya , Suzuki Yutaka , Tanaka Ryota , Abe Nobutsugu , Masaki Tadahiko , Mori Toshiyuki , Ohkura Yasuo , Sugiyama Masanori
Published: February 2018

Objectives

Reprimo gene is a cytoplasmic protein belonging to a family of molecules controlled by p53 that inhibits cell cycle progression. Ectopic expression of Reprimo results in cell cycle arrest at the G2 phase. The aim of this study was to investigate the impact of Reprimo expression on tumorigenesis of intraductal papillary mucinous neoplasm (IPMN).

Methods

Thirty-seven surgical cases of IPMN were collected retrospectively. Twenty-eight patients had benign IPMNs (low-grade dysplasia, n = 18; intermediate-grade dysplasia, n = 10), and the remaining 9 had malignant IPMNs (high-grade dysplasia, n = 4; invasive carcinoma, n = 5). DNA from tumor samples was extracted. DNA methylation patterns of Reprimo were determined by the methods of methylation-specific polymerase chain reaction and immunohistochemistry. The methylation status of Reprimo was compared between benign IPMNs and malignant IPMNs.

Results

The incidence of aberrant DNA methylation of Reprimo was significantly higher in malignant IPMNs than in benign IPMNs (78% vs 32%, P = 0.016). Furthermore, the incidence of immunohistochemical Reprimo expression was significantly lower in malignant IPMNs than in benign IPMNs (22% vs 82%, P = 0.002).

Conclusions

Reprimo methylation was found more frequently in malignant IPMNs. Reprimo methylation is involved in malignant transformation of IPMNs.
Per-Pass Performance Characteristics of Endoscopic Ultrasound-Guided Fine-Needle Aspiration of Malignant Solid Pancreatic Masses in a Large Multicenter Cohort
Authors: Ge Phillip S. , Wani Sachin , Watson Rabindra R. , Sedarat Alireza , Kim Stephen , Marshall Carrie , Wilson Robert H. , Makker Jitin , Mohamadnejad Mehdi , Komanduri Srinadh , Muthusamy V. Raman
Published: February 2018

Objectives

Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) is widely performed for the evaluation of pancreatic masses. We evaluated the performance characteristics of EUS-FNA in obtaining a diagnosis of pancreatic malignancy.

Methods

We performed a multicenter study of patients who underwent EUS-FNA for a solid pancreatic mass. Endoscopic ultrasound-guided FNA was standardized using a 25-gauge needle, slow-pull stylet technique for specimen acquisition, and on-site cytopathology. For the primary analysis, only malignant cytology was regarded as positive. A secondary analysis was performed in which malignant and/or suspicious cytology was regarded as positive.

Results

A total of 138 patients underwent EUS-FNA. In the primary analysis, the sensitivity of EUS-FNA for malignancy was 56.7% on first pass, 73.3% on second pass, 83.3% on third pass, 89.2% on fourth pass, and 90.8% on fifth pass, with no increase beyond the fifth pass. In the secondary analysis, the sensitivity was 75.0% on first pass, 89.2% on second pass, 93.3% on third pass, and 95.8% on fourth pass, with no increase beyond the fourth pass. No significant relationship was seen between lesion size and diagnostic yield.

Conclusions

Using a 25-gauge needle, the maximal diagnostic yield of EUS-FNA for a solid pancreatic mass is reached after 4 needle passes.
Long-term Outcomes of Gastroenteropancreatic Neuroendocrine Tumors
Authors: Chi Weiwei , Warner Richard R.P. , Chan David L. , Singh Simron , Segelov Eva , Strosberg Jonathan , Wisnivesky Juan , Kim Michelle K.
Published: February 2018

Objectives

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are rare but have been increasing in incidence. Limited data on the long-term outcomes of patients with these tumors are available.

Methods

In this study, we used population-based data from the National Cancer Institute to assess long-term disease-specific survival (DSS) of patients who have undergone surgery for nonmetastatic disease. All patients with NETs of the stomach, small intestine, colon, rectum, appendix, and pancreas diagnosed between 1988 and 2009 were identified from the Surveillance, Epidemiology and End Results registry. Staging was derived from Surveillance, Epidemiology and End Results data using the European Neuroendocrine Tumor Society guidelines. Cases with incomplete staging data were excluded, along with those with stage IV disease, or those who did not undergo surgical resection.

Results

Kaplan-Meier analyses were constructed to determine DSS. Analyses were further stratified according to tumor site, stage at diagnosis, and tumor grade. Overall, 13,348 patients with GEP-NETs meeting the inclusion criteria were identified.

Conclusions

There were excellent outcomes for most GEP-NET patients, with a 20-year DSS of greater than 75% across all sites and stages. Pancreatic tumors had the worst outcomes, but DSS remains greater than 50% at 20 years.
Molecular Comparison of Adult and Pediatric Ulcerative Colitis Indicates Broad Similarity of Molecular Pathways in Disease Tissue
Authors: Li Katherine , Strauss Richard , Ouahed Jodie , Chan Daphne , Telesco Shannon E. , Shouval Dror S. , Canavan James B. , Brodmerkel Carrie , Snapper Scott B. , Friedman Joshua R.
Published: February 2018

Background:

Efficacy data from adult ulcerative colitis (UC) clinical trials are often extrapolated for pediatric prescribing. Consequently, it is important to understand similarities/differences in pediatric and adult UC. Pediatric UC tends to have more extensive disease at presentation, yet genetic studies have not detected pathways that distinguish the populations, and differences in mucosal gene expression between adult and pediatric UC are not well characterized.

Methods:

Using colonic microarray data from a phase 3 trial of golimumab in adult UC (87 UC; 21 healthy), the GSE10616 pediatric dataset (10 UC; 11 healthy), and a phase 1B trial of golimumab in pediatric UC (n = 19), UC expression profiles were compared and unique genes were defined as those with significant changes (|FC|>2×, adjusted P < 0.05) in one population, but not the other (|FC| < 1.2×, adjusted P > 0.05). Pathway and upstream regulator analyses were performed. Profiles by disease extent (extensive [pancolitis] vs limited [left‐sided] involvement) were compared within each population.

Results:

Pediatric and adult disease profiles overlapped substantially, with ~50% to 75% overlap, depending on the fold‐change cutoff used. Conversely, <10% of the disease profiles were unique to each population. Similar canonical pathways were enriched in both datasets. Predicted upstream regulators were also concordant, including lipopolysaccharide, interleukin‐1β, and tumor necrosis factor‐α. Expression profiles of extensive UC were indistinguishable from those of patients with limited involvement in each population.

Conclusions:

The UC gene expression landscape is shared by adults and children, independent of disease extent. This supports extrapolation of efficacy from adults to children in developing new therapies for UC.
Safety and Efficacy of Bedside Percutaneous Endoscopic Gastrostomy Placement in the Neonatal Intensive Care Unit
Authors: Driver Kelly , Schilling Rebecca , Goodwin Annie , Martinez Denise , Amankwah Ernest , Shakeel Fauzia , Wilsey Michael
Published: February 2018

Objective:

The aim of the study is to describe the safety and efficacy of bedside percutaneous endoscopic gastrostomy (PEG) placement in a level 3 neonatal intensive care unit (NICU).

Methods:

A retrospective chart review was performed on 106 infants with a birthweight ⩽6 kg receiving bedside PEG placement at Johns Hopkins All Children's Hospital between 2007 and 2013. Preprocedure, postprocedure, and demographic data were collected. The main safety outcome was postprocedure complication rate and the main efficacy outcome was time to initiate feeds and time on respiratory support.

Results:

The mean birth weight and mean gestational age of our population at the time of procedure were 2.2 kg and 33 weeks, respectively. There were 9 total complications (8.5%) with major complications being only 2 (1.8%). There were no instances of blood stream infections. The mean length of time to initiate feeds was 1.2 days (standard deviation [SD] = 1.2). Ninety‐three percent of patients were extubated within 24 hours.

Conclusions:

Bedside PEG placement is safe with minimal complications. It is associated with little need for ventilator support and allows for early re‐initiation of feeds and early success at reaching goal feedings.
Creation of Experience‐based Celiac Benchmarks
Authors: Fishman Laurie N. , Kearney Jennifer , DeGroote Maya , Liu Enju , Arnold Janis , Weir Dascha C.
Published: February 2018

Objectives:

Transition planning for children with chronic disease includes the development of independence in many self‐management tasks. Conditions that depend on diet have distinct skill sets not well assessed by the traditional transition‐readiness tools. There has been literature that describes age‐appropriate skill acquisition for diabetes and food allergy patients. There are, however, no age‐appropriate benchmarks established for celiac disease (CD).

Methods:

CD experts (including physician, nurse, dietician, social worker, patient, and parent) created a list of celiac‐related tasks, which formed the basis of the survey. Patients with CD, and their parents, were recruited from outpatient celiac clinic and support groups, and invited to report the age each task was mastered.

Results:

Respondents included 204 patients and 155 parents. Mean age was 12 years (standard deviation 4.6) with average of 4 years since diagnosis. The earliest tasks were mastered by a median age of 8 years, such as recognizing GF as gluten‐free, eating safely in a shared space and recognizing basic unsafe foods. Describing the effects of eating gluten or explaining CD to a friend or stranger occurred around age 10. Asking about gluten‐free preparation in a restaurant, and identifying gluten‐free medications or vitamins was mastered around age 12, whereas tasks involved with safe domestic travel or assessing risk in a job environment occurred between 14 and 16. The interquartile range was about 4 years for each question. No significant difference seen between patient and parent reports.

Conclusions:

This novel patient‐centered celiac skill list may improve anticipatory guidance and accelerate self‐management skills.
A Case of Small Cell Lung Carcinoma in a 15-Year-Old Boy and Literature Review
Authors: Barbour Mustafa , Bujold Kenneth E. , Tjarks Brian Joel , Jassim Ali D.
Published: February 2018

Small cell lung carcinoma (SCLC), also known as high-grade neuroendocrine tumor of the lung, is exceedingly rare in the pediatric population. SCLC is usually fast growing and often has metastasized at diagnosis. It frequently responds well to therapy initially, however, has a high relapse and mortality rate. There are limited published data on SCLC in children and no existing pediatric treatment protocols. In this report, we present a case of extensive stage SCLC in a 15-year-old boy who responded to single-agent gemcitabine therapy and review similar cases reported in the medical literature.
Primary Central Nervous System Malignant Melanoma in Children: A Case Series and Review of the Literature
Authors: Choi Daniel K. , Pillay-Smiley Natasha , Marzec Sarah , Wadhwani Nitin R. , DiPatri Arthur J. , Tomita Tadanori , Lulla Rishi R.
Published: February 2018

We describe 2 cases of rapidly progressive primary central nervous system malignant melanoma, and summarize 18 previously reported cases of this extremely rare tumor in children. Both patients presented with focal neurologic symptoms, with no evidence of skin or other organ system involvement. One patient was treated with temozolomide and etoposide, whereas the other was treated with multiple surgical resections, radiation therapy, and a trial of ipilimumab. New molecularly targeted and immune-based therapies used in metastatic melanoma in adults are potential new treatment options, but their efficacy and safety in pediatric patients needs to be established.
Risk Factors and the Prevention of Weight Gain During Induction Chemotherapy in Children With Acute Lymphoblastic Leukemia
Authors: Seki Yuko , Okamoto Yasuhiro , Kodama Yuichi , Nishikawa Takuro , Tanabe Takayuki , Nakagawa Shunsuke , Mizota Michiyo , Kawano Yoshifumi
Published: February 2018

Weight gain is often observed in children with acute lymphoblastic leukemia (ALL) who undergo chemotherapy including steroids. An increase in body mass index (BMI)-standard deviation score (SDS) during induction therapy is reported as a risk factor for obesity after treatment. However, risk factors of an increase in BMI-SDS during induction therapy are not known. Ninety-six patients with ALL who were treated at our hospital between 1996 January and September 2013 were analyzed retrospectively. Daily body weight measurement was initiated in July 2005 in an attempt to control weight. Fifty-four patients were boys and 42 were girls. The median age at onset was 5.1 years (0.5–16.6 y), and 7.3% of patients were overweight/obese at onset. BMI-SDS increased +0.1% (−3.3% to +3.2%) during induction therapy. BMI-SDS increased by 1 and 2 or more SDs in 20% and 3% of patients, respectively. In multivariate analysis, non–high-risk treatment and earlier treatment start date (before daily body weight measurement) were independent risk factors. Ten percent of patients were overweight/obese at 3 years after completion therapy, and high BMI-SDS after induction therapy was a risk factor. Daily body weight measurement might prevent excess weight gain during induction therapy, resulting in patients maintaining a healthy weight after ALL treatment.
Deep Vein Thrombosis in a Young, Healthy Baseball Catcher: A Case Report and Review of the Literature
Authors: Abood Kimberly K. , Paul Megan Rose , Kuo Dennis John
Published: February 2018

Venous thromboembolism is becoming increasingly recognized as a significant cause of morbidity and mortality in the hospitalized pediatric population. However, young healthy athletes can present with unique risk factors for deep vein thrombosis (DVT) that can be overlooked. Here we report a case of an adolescent male with no inherited risk factors or prior history of DVTs who developed a right femoral vein DVT in the context of playing catcher in baseball after recovering from a bout of streptococcal pharyngitis. We review the evidence that suggests that repetitive squatting-induced compression of the femoral vein can cause the venous stasis and endothelial microtrauma that contributed to the formation of this thrombus.
Acquired Hypofibrinogenemia Before Asparaginase Exposure During Induction Therapy for Pediatric Acute Lymphoblastic Leukemia: A Report of 2 Cases and Review of the Literature
Authors: Raikar Sunil S. , Felker James , Patel Kavita N. , Lew Glen , Sidonio Robert F.
Published: February 2018

Coagulopathy in pediatric leukemia patients is typically associated with acute promyelocytic leukemia or after asparaginase use in acute lymphoblastic leukemia. Rarely seen in acute lymphoblastic leukemia, we report 2 patients who presented with normal coagulation markers, but subsequently developed severe hypofibrinogenemia and bleeding in induction before administration of asparaginase. In both cases, cryoprecipitate was administered as initial treatment for bleeding associated with the hypofibrinogenemia. One patient was refractory to cryoprecipitate replacement and required treatment with human fibrinogen concentrate due to the persistence of hypofibrinogenemia with significant bleeding. The hypofibrinogenemia was transient in both cases and resolved within a few weeks.
Decision-Making Data
Authors: Swift Marcie C. , Townsend Robert , Edwards Douglas W. , Loudon Janice K.
Published: February 2018

Purpose:

The purpose of this study was to explore the difference in maximal lifting capability between 2 modes of lifting (traditional crate and XRTS Lever Arm) over multiple days. The differences in absolute strength values were compared with existing criteria for sincere effort during distraction-based lifting. In addition, rate of perceived exertion (RPE) is presented for the 2 modes of lifting on each day.

Primary Practice Setting:

Workers' compensation.

Methodology and Sample:

Forty-four subjects between the ages of 20 and 44 years participated in this study. Investigators established 1 repetition maximum (RM) for each subject performing the crate lift. Subjects were randomly assigned 5 weights ranging from 10% to 100% of their determined 1RM and asked to give their rating of perceived exertion after each lift. The same procedure was repeated 2–5 days later using the XRTS Lever Arm. Paired t tests and Spearman's correlation coefficient were used for data analysis. Alpha was set at less than .05.

Results:

There was a statistically significant difference (p < .04) between maximal lift values for the 2 lifting modes. The percent difference between the modes of lifting was 10.5% ± 6.4%. In addition, there was a positive correlation between the RPE on the 2 modes of lifting (p = .87).

Implications for Case Management Practice:

A functional capacity evaluation (FCE) is typically ordered after the completion of physical rehabilitation and before releasing a patient to full or modified duty. In addition to assessing the ability to function within normal job demands, an assessment of effort by the participant typically takes place during an FCE. Case managers and physicians are presented with information, allowing them to make comparisons between functional lifting abilities displayed during treatment sessions and the FCE. These comparisons may often take place with the subpoena of medical records and may be discussed during the deposition or trial process. If an FCE takes place at a different facility than the physical therapy or work conditioning treatment, 2 different modes of lifting may take place based on the equipment within each facility. The results of this study indicate that the 2 modes of lifting on separate days meet established criteria for lift comparison testing during FCEs.
Blur Detection, Depth of Field, and Accommodation in Emmetropic and Hyperopic Children
Authors: Roberts Tawna L. , Stevenson Scott B. , Benoit Julia S. , Manny Ruth E. , Anderson Heather A.
Published: February 2018

SIGNIFICANCE

Our results demonstrate that blur detection thresholds are elevated in young children compared with adults, and poorer blur detection thresholds are significantly correlated with the magnitude of accommodative microfluctuations. Given that accommodative microfluctuations are greater with greater accommodative responses, these findings may have implications for young uncorrected hyperopes.

PURPOSE

This study investigated the association between subjective blur detection thresholds and accommodative microfluctuations in children 3 years to younger than 10 years old and adults.

METHODS

Blur detection thresholds were determined in 49 children with habitually uncorrected refractive error (+0.06 to +4.91 diopters [D] spherical equivalent) and 10 habitually uncorrected adults (+0.08 to +1.51 D spherical equivalent) using a custom blur chart with 1° sized optotypes at 33 cm. Letters were blurred by convolution using a Gaussian kernel (SDs of 0.71 to 11.31 arc minutes in √2 steps). Subjective depth of field was determined in subjects 6 years or older and adults. Accommodative microfluctuations, pupils, and lag were measured using infrared photorefraction (25 Hz).

RESULTS

Children had greater blur detection thresholds (P < .001), accommodative microfluctuations (P = .001), and depth of field (P < .001) than adults. In children, increased blur detection thresholds were associated with increased accommodative microfluctuations (P < .001), increased uncorrected hyperopia (P = .01), decreased age (P < .001), and decreased pupil size (P = .01). In a multiple linear regression analysis, blur detection thresholds were associated with accommodative microfluctuations (P < .001) and age (P < .001). Increased accommodative microfluctuations were associated with increased uncorrected hyperopia (P = .004) and decreased pupil size (P = .003) and independently associated with uncorrected hyperopia (P = .001) and pupil size (P = .003) when controlling for age and lag.

CONCLUSIONS

Children did not have adult-like blur detection thresholds or depth of field. Increased accommodative microfluctuations and decreased age were independently associated with greater blur detection thresholds in children 3 years to younger than 10 years. Larger amounts of uncorrected hyperopia in children appear to increase blur detection thresholds because the greater accommodative demand and resulting response increase accommodative microfluctuations.
Association between Contrast Sensitivity and Reading with Macular Pathology
Authors: Brussee Tamara , van den Berg Thomas J. T. P. , van Nispen Ruth M. A. , de Boer Ilon , van Rens Ger H. M. B.
Published: February 2018

SIGNIFICANCE

Comparison between the role of spatial and temporal contrast sensitivities in the association with reading may provide insight into how visual tasks (such as reading) are related to primary optical or neural (or both) effects. More insight into primary visual factors influencing reading is important for understanding reading problems.

PURPOSE

The aim of this study was to gain insight into the association between optical and neural components of contrast sensitivity (CS), operationalized as spatial CS (optical and neural) or temporal CS (solely neural), and reading speed in a clinical sample of participants with macular pathologies. The precision and agreement were also investigated.

METHODS

The Mars test and temporal CS implementation of the C-Quant device were used to measure spatial CS and temporal CS, respectively. Tests were performed with 47 participants: mean age, 77 years (range, 52 to 92 years). Associations were investigated with correlations and linear regression models. Precision was defined by coefficients of repeatability. The 95% limits of agreement between spatial CS and temporal CS values were assessed.

RESULTS

Reading speed correlated with both spatial CS (r = 0.35, P = .015) and temporal CS (r = 0.66, P < .001). After correction for visual acuity, central loss, and education level, the association between temporal CS and reading speed was not significant anymore. The coefficients of repeatability and reproducibility were 0.20 and 0.28 log unit (spatial CS) and 0.33 and 0.35 log unit (temporal CS), respectively. The values for temporal CS were 0.08 and 0.13 log unit higher than those for spatial CS.

CONCLUSIONS

For spatial CS and temporal CS, moderate to strong correlations were found, respectively, with reading speed in patients with maculopathies. The stronger association between temporal CS and reading speed is suggested to reflect a high sensitivity for neural integrity of temporal CS. The differences in coefficients of repeatability and reproducibility could be explained by the psychometrical differences between methods.
Retinal Nerve Fiber Layer Protrusion Associated with Tilted Optic Discs
Authors: Chiang Jaclyn , Yapp Michael , Ly Angelica , Hennessy Michael P. , Kalloniatis Michael , Zangerl Barbara
Published: February 2018

SIGNIFICANCE

This study resulted in the identification of an optic nerve head (ONH) feature associated with tilted optic discs, which might potentially contribute to ONH pathologies. Knowledge of such findings will enhance clinical insights and drive future opportunities to understand disease processes related to tilted optic discs.

PURPOSE

The aim of this study was to identify novel retinal nerve fiber layer (RNFL) anomalies by evaluating tilted optic discs using optical coherence tomography. An observed retinal nerve fiber protrusion was further investigated for association with other morphological or functional parameters.

METHODS

A retrospective review of 400 randomly selected adult patients with ONH examinations was conducted in a referral-only, diagnostic imaging center. After excluding other ONH pathologies, 215 patients were enrolled and evaluated for optic disc tilt and/or torsion. Gross anatomical ONH features, including size and rim or parapapillary region elevation, were assessed with stereoscopic fundus photography. Optical coherence tomography provided detailed morphological information of individual retinal layers. Statistical analysis was applied to identify significant changes between individual patient cohorts.

RESULTS

A dome-shaped hyperreflective RNFL bulge, protruding into the neurosensory retina at the optic disc margins, was identified in 17 eyes with tilted optic discs. Available follow-up data were inconclusive regarding natural changes with this ONH feature. This RNFL herniation was significantly correlated with smaller than average optic disc size (P = .005), congenital disc tilt (P < .0001), and areas of rim or parapapillary elevation (P < .0001).

CONCLUSIONS

This study reports an RNFL protrusion associated with tilted optic discs, which has not previously been assessed as an independent ONH structure. The feature is predominantly related to congenital crowded, small optic discs and variable between patients. This study is an important first step to elucidate diagnostic capabilities of tilted disc morphological changes and understanding associated functional deficits.
Hyperchloremia Is Associated With Complicated Course and Mortality in Pediatric Patients With Septic Shock*
Authors: Stenson Erin K. , Cvijanovich Natalie Z. , Anas Nick , Allen Geoffrey L. , Thomas Neal J. , Bigham Michael T. , Weiss Scott L. , Fitzgerald Julie C. , Checchia Paul A. , Meyer Keith , Quasney Michael , Hall Mark , Gedeit Rainer , Freishtat Robert J. , Nowak Jeffrey , Raj Shekhar S. , Gertz Shira , Grunwell Jocelyn R. , Wong Hector R.
Published: February 2018

Objective:

Hyperchloremia is associated with poor outcome among critically ill adults, but it is unknown if a similar association exists among critically ill children. We determined if hyperchloremia is associated with poor outcomes in children with septic shock.

Design:

Retrospective analysis of a pediatric septic shock database.

Setting:

Twenty-nine PICUs in the United States.

Patients:

Eight hundred ninety children 10 years and younger with septic shock.

Interventions:

None.

Measurements and Main Results:

We considered the minimum, maximum, and mean chloride values during the initial 7 days of septic shock for each study subject as separate hyperchloremia variables. Within each category, we considered hyperchloremia as a dichotomous variable defined as a serum concentration greater than or equal to 110 mmol/L. We used multivariable logistic regression to determine the association between the hyperchloremia variables and outcome, adjusted for illness severity. We considered all cause 28-day mortality and complicated course as the primary outcome variables. Complicated course was defined as mortality by 28 days or persistence of greater than or equal to two organ failures at day 7 of septic shock. Secondarily, we conducted a stratified analysis using a biomarker-based mortality risk stratification tool. There were 226 patients (25%) with a complicated course and 93 mortalities (10%). Seventy patients had a minimum chloride greater than or equal to 110 mmol/L, 179 had a mean chloride greater than or equal to 110 mmol/L, and 514 had a maximum chloride greater than or equal to 110 mmol/L. A minimum chloride greater than or equal to 110 mmol/L was associated with increased odds of complicated course (odds ratio, 1.9; 95% CI, 1.1–3.2; p = 0.023) and mortality (odds ratio, 3.7; 95% CI, 2.0–6.8; p < 0.001). A mean chloride greater than or equal to 110 mmol/L was also associated with increased odds of mortality (odds ratio, 2.1; 95% CI, 1.3–3.5; p = 0.002). The secondary analysis yielded similar results.

Conclusion:

Hyperchloremia is independently associated with poor outcomes among children with septic shock.
Beyond Survival: Pediatric Critical Care Interventional Trial Outcome Measure Preferences of Families and Healthcare Professionals*
Authors: Merritt Courtney , Menon Kusum , Agus Michael S. D. , Choong Karen , McNally Dayre , O’Hearn Katie , Watson R. Scott , Wong Hector R. , Duffett Mark , Wypij David , Zimmerman Jerry J.
Published: February 2018

Objectives:

To identify, in addition to survival, preferred outcome measures of PICU family care providers and PICU healthcare professionals for interventional trials enrolling critically ill children, and to describe general attitudes of family care providers and healthcare professionals regarding research in the PICU.

Design:

Cross-sectional survey examining subject experience with clinical research and personal preferences for outcome measures for a hypothetical interventional clinical trial.

Setting:

PICUs within four academic children’s hospitals in the United States and Canada.

Subjects:

Two cohorts including family members of critically ill children in PICUs (family care providers) and multidisciplinary staff working in the PICUs (healthcare professionals).

Interventions:

Administration of a short, deidentified survey.

Measurements:

Demographic data were collated for the two subject groups. Participants were queried regarding their attitudes related to research conducted in the PICU. In addition to survival, each group was asked to identify their three most important outcomes for an investigation examining whether or not an intervention helps seriously ill children recover.

Main Results:

Demographics for family care providers (n = 40) and healthcare professionals (n = 53) were similarly distributed. Female respondents (79.8%) predominated. Participants (98.9%) ascertained the importance of conducting research in the PICU, but significant challenges associated with this goal in the high stress PICU environment. Both quality of life and functioning after leaving the hospital were chosen as the most preferred outcome measure, with 77.5% of family care providers and 84.9% of healthcare professionals indicating this choice. Duration of organ dysfunction was identified by 70.0% of family care providers and 40.7% of healthcare professionals as the second most preferred outcome measure.

Conclusions:

In addition to survival, long-term quality of life/functional status and duration of organ dysfunction represent important interventional trial outcome measures for both families of critically ill children, as well as the multidisciplinary team who provides critical care.
Functional Recovery in Critically Ill Children, the “WeeCover” Multicenter Study
Authors: Choong Karen , Fraser Douglas , Al-Harbi Samah , Borham Asm , Cameron Jill , Cameron Saoirse , Cheng Ji , Clark Heather , Doherty Tim , Fayed Nora , Gorter Jan-Willem , Herridge Margaret , Khetani Mary , Menon Kusum , Seabrook Jamie , Simpson Racquel , Thabane Lehana
Published: February 2018

Objectives:

To evaluate functional outcomes and evaluate predictors of an unfavorable functional outcome in children following a critical illness.

Design:

Prospective observational longitudinal cohort study.

Setting:

Two tertiary care, Canadian PICUs: McMaster Children’s Hospital and London Health Sciences.

Patients:

Children 12 months to 17 years old, admitted to PICU for at least 48 hours with one or more organ dysfunction, were eligible. Patients not expected to survive, direct transfers from neonatal ICU and patients in whom long-term follow-up would not be able to be conducted, were excluded.

Interventions:

None.

Measurements and Main Results:

The primary endpoint was functional outcome up to 6 months post PICU discharge, measured using the Pediatric Evaluation of Disabilities Inventory Computer Adaptive Test. Secondary outcomes included predictors of unfavorable functional outcome, caregiver stress, health-related quality-of-life, and clinical outcomes such as mortality, length of stay, and PICU-acquired complications. One hundred eighty-two patients were enrolled; 78 children (43.6%) had functional limitations at baseline and 143 (81.5%) experienced functional deterioration following critical illness. Ninety-two (67.1%) demonstrated some functional recovery by 6 months. Higher baseline function and a neurologic insult at PICU admission were the most significant predictors of functional deterioration. Higher baseline function and increasing age were associated with slower functional recovery. Different factors affect the domains of functioning differently. Preexisting comorbidities and iatrogenic PICU-acquired morbidities were associated with persistent requirement for caregiver support (responsibility function) at 6 months. The degree of functional deterioration after critical illness was a significant predictor of increased hospital length of stay.

Conclusions:

This study provides new information regarding functional outcomes and the factors that influence meaningful aspects of functioning in critically ill children. Identifying patients at greatest risk and modifiable targets for improvement in PICU care guides us in developing strategies to improve functional outcomes and tailor to the rehabilitation needs of these patients and their families.
Sensory symptom profiles differ between trigeminal and thoracolumbar postherpetic neuralgia
Authors: Rehm Stefanie , Groβkopf Moritz , Kabelitz Maria , Keller Thomas , Freynhagen Rainer , Tölle Thomas R. , Baron Ralf
Published: February 2018

Abstract

Introduction:

Animal experimental evidence suggests that mechanisms of pain generation and response to treatment differ between neuropathic pain in the cephalic and the extracephalic innervation territories.

Objectives:

The objective of the study was to examine whether in humans an identical peripheral painful neuropathy is associated with different pain qualities and sensory abnormalities in the face as compared with the thoracic region.

Methods:

We retrospectively analysed epidemiological and clinical data of 639 patients with postherpetic neuralgia (PHN) in the face and at the trunk who were collected within a cross-sectional cohort survey and compared the respective sensory symptom profiles captured with the painDETECT questionnaire.

Results:

Two hundred twenty-four patients suffered from trigeminal PHN and 415 from thoracolumbar PHN. There were no significant differences in sex-ratio, age, body mass index, and pain duration. Patients with trigeminal PHN were more often severely depressed. Anxiety and sleep scores were not different. The average pain intensity was slightly higher in thoracolumbar PHN than trigeminal PHN (visual analogue scale 5.0 vs 4.6). Postherpetic neuralgia in the thoracolumbar region showed significantly more intense burning sensations, allodynia, painful attacks, and significantly less prickling and numbness than PHN in the face.

Conclusions:

The differences in sensory symptom profiles between facial PHN and truncal PHN might be associated with different pathophysiological mechanisms and different treatment response. Drugs that primarily act on sensitization processes in the peripheral nociceptive system may work better in thoracolumbar PHN than in trigeminal PHN. If new medications are tested in patients with PHN, it would therefore be of interest to include an analysis of the treatment results in regard to subgroups based on the localisation of pain in patients with PHN.
Clear Cell Sarcoma of the Tongue on MRI and PET/CT
Authors: Tordjman Mickael , Dubois Matthieu , de Malherbe Maxime , Denoux Yves , Gillebert Quentin
Published: February 2018

18F-FDG PET/CT and MRI were performed in a 44-year-old woman to characterize a mass of the anterior tongue. MR images showed a voluminous mass, well circumscribed and enhanced heterogeneously after gadolinium chelates injection. There was an intense uptake on PET/CT. Pathological examination and molecular analysis revealed the diagnosis of clear cell sarcoma of the tongue. We present a case of clear cell sarcoma of the tongue, which includes imaging features. It is an extremely rare tumor, with only 3 cases previously reported in the literature.
Superior Pituitary Border Analysis in Immunotherapy-Induced Hypophysitis
Authors: Pucar Darko , Boustani Anne Marie , Bronen Richard A. , Saperstein Lawrence
Published: February 2018

Immunotherapy-induced hypophysitis presents with headache, fatigue, and visual disturbances. The diagnosis is supported by imaging findings of pituitary swelling, enhancement, and hypermetabolism and established by low levels of pituitary hormones. A 64-year-old man with metastatic melanoma on nivolumab presented with a severe headache, initially attributed to sinus disease. Contrast CT was interpreted as minor sinus disease and no pituitary abnormality. Hypophysitis was eventually diagnosed and successfully treated based on PET and laboratory findings. Our retrospective analysis demonstrated abnormal convex superior pituitary border visible on contrast and noncontrast CT and PET. This feature may aid diagnosis in the challenging cases.
Detecting Intestinal Malrotation on Hepatobiliary Scintigraphy
Authors: Miner Lane T. , Tulchinsky Mark
Published: February 2018

Intestinal malrotation displays classic pattern of the small bowel activity on hepatobiliary scintigraphy that could lead to the diagnosis, but only if an interpreting physician looks for it. Presented are 2 cases, one demonstrates diagnostic findings during the first 60-minute phase, whereas the other reveals the diagnosis only on the later sincalide stimulation phase. The currently recommended structured reporting for hepatobiliary scintigraphy does not include examination of bowel activity pattern, which could result in a missed diagnosis. Including interrogation of the small bowel pattern into the structured reporting checklist would ensure consistent detection of this rare but most consequential diagnosis.
SPECT/CT MIBG Imaging Is Crucial in the Follow-up of the Patients With High-Risk Neuroblastoma
Authors: Liu Bin , Servaes Sabah , Zhuang Hongming
Published: February 2018

Background

Planar whole-body imaging with 123I-radiolabeled metaiodobenzylguanidine (123I-MIBG) is routinely used in the follow-up evaluation of neuroblastoma. In recent years, SPECT with integrated low-dose CT (SPECT/CT) has become more accessible. We investigated how much SPECT/CT can have additional diagnostic value over planar imaging in detection of residual and recurrent neuroblastoma.

Methods

A total of 170 planar 123I-MIBG imaging scans with SPECT/CT follow-up scans performed in 147 patients with known high-risk neuroblastoma were retrospectively analyzed. Regions of increased 123I-MIBG uptake on planar images and the findings on SPECT/CT were compared.

Results

In 61% of the studies, the whole-body planar images and SPECT/CT images yielded the same result. In 39% of the time, however, SPECT/CT images provided additional information.

Conclusions

In the follow-up of patients with high-risk neuroblastoma, SPECT/CT can significantly improve planar imaging interpretation and impact patient management.
68Ga-DOTATATE PET/CT Reveals Epstein-Barr Virus–Associated Nasopharyngeal Carcinoma in a Case of Suspected Sphenoid Wing Meningioma
Authors: Unterrainer Marcus , Maihoefer Cornelius , Cyran Clemens C. , Bartenstein Peter , Niyazi Maximilian , Albert Nathalie Lisa
Published: February 2018

In this case of suspected sphenoid wing meningioma, 68Ga-DOTATATE PET/CT showed a somatostatin receptor (SSR)–expressing tumor with extension to the nasopharynx and SSR-expressing cervical lymph nodes. Subsequent biopsy from the nasopharynx revealed an Epstein-Barr virus (EBV)–associated, undifferentiated World Health Organization type 3 nasopharyngeal carcinoma (NPC), a potential clinical pitfall due to the reported high SSR expression of this tumor subtype. In consideration of the high target-to-background contrast, SSR ligands might be superior to 18F-FDG for EBV-associated NPC PET imaging, particularly at the skull base. Somatostatin receptor ligands might furthermore offer interesting theranostic possibilities for patients with advanced/extensive EBV-associated NPC.
177Lu–Prostate-Specific Membrane Antigen Super Scan and Good Response Even After 1 Cycle of Radioligand Therapy
Authors: Aryana Kamran , Zarehparvar moghadam Soroush , Salek Roham , Divband Ghasemali
Published: February 2018

A 76-year-old man with castration-resistant prostate cancer and widespread skeletal metastases underwent 2 cycles of 177Lu–prostate-specific membrane antigen (PSMA) 617 therapy in our department. Whole-body 177Lu-PSMA scan after the first cycle showed diffuse skeletal PSMA-avid lesions, whereas no PSMA uptake was evident in the kidneys with minimal PSMA uptake by salivary glands (super scan). After 6 weeks, he received the second dose of 177Lu-PSMA and whole-body scan after the treatment showed remarkable resolution of skeletal metastases and normal PSMA uptake by the kidneys and salivary glands. To our best knowledge, this is the first case report of 177Lu-PSMA super scan.
Prostate-Specific Membrane Antigen PET/CT Incidental Finding of a Schwannoma
Authors: Dias André H. , Bouchelouche Kirsten
Published: February 2018

We describe a case of a 73-year-old man with newly diagnosed prostate cancer referred to 68Ga-PSMA PET/CT for staging. A focal uptake of PSMA was visualized in the prostate compatible with primary prostate cancer. Outside the prostate, a PSMA-avid paravertebral soft tissue mass was observed. Further investigation with MRI of the same region showed signs compatible with schwannoma. This case shows the importance of including schwannoma in the differential diagnostic evaluation of patients with 68Ga-PSMA–positive foci in paravertebral locations, as schwannomas may show avid PSMA uptake and may potentially lead to an incorrect diagnosis of metastasis.
Pancreatic Malignancy or Not?
Authors: Parihar Ashwin Singh , Singh Harmandeep , Kumar Rajender , Gupta Vikas , Singh Harpreet , Mittal Bhagwant Rai
Published: February 2018

Immunoglobulin G4–related disease is an autoimmune entity with multisystem involvement. 18F-FDG PET/CT can provide important information about disease extent and severity and also aid in treatment response evaluation. We report a case of a 57-year-old woman presenting with abdominal discomfort, belching, and loss of weight in whom 18F-FDG PET/CT provided valuable information in diagnosis and monitoring treatment response.
68Ga–Prostate-Specific Membrane Antigen Expression in Neurocysticercosis Lesions in a Patient With Prostate Carcinoma
Authors: Vadi Shelvin Kumar , Kumar Rajender , Singh Harmandeep , Singh Shrawan Kumar , Mittal Bhagwant Rai
Published: February 2018

68Ga–prostate-specific membrane antigen (PSMA) ligand is a well-known tracer used in prostate cancer. Nevertheless, there are reports of unusual 68Ga-PSMA uptake in variety of benign lesions. We report a case of prostate cancer in which 68Ga-PSMA PET/CT revealed tracer avidity in the enhancing walls of cystic lesions in the brain consistent with the features of neurocysticercosis. The uptake of 68Ga-PSMA in neurocysticercosis is not known and may be attributed to angiogenesis occurring at the periphery of these lesions.