bluuebunny/crossref_metadata_2025_split huggingface dataset:
Evaluating Surgical Decision-making in Nonsyndromic Sagittal Craniosynostosis Using a Digital 3D Model
Authors: Hughes Christopher D. , Langa Olivia , Nuzzi Laura , Staffa Steven J. , Proctor Mark , Meara John G. , Ganske Ingrid M. 
Published: May 2021

Background:
Surgical correction of craniosynostosis addresses potentially elevated intracranial pressure and the cranial deformity. In nonsyndromic sagittal synostosis, approximately 15% of patients have elevated intracranial pressure. The decision to operate therefore likely reflects a combination of aesthetic goals, prevention of brain growth restriction over time, surgeon training and experience, and parental expectations. This study examines clinical factors that influence surgical decision-making in nonsyndromic sagittal synostosis.

Methods:
An online survey sent to craniofacial and neurosurgeons presented 5 theoretical patients with varying severities of sagittal synostosis. For each cephalic index, 4 separate clinical scenarios were presented to assess influences of parental concern and developmental delay on the decision to operate.

Results:
Fifty-six surveys were completed (response rate = 28%). Participants were predominantly from North America (57%), had over 10 years of experience (75%), and performed over 20 craniosynostosis procedures annually (50%). Thirty percent of respondents indicated they would operate regardless of head shape and without clinical and/or parental concern. Head shape was the greatest predictor of decision to operate (P < 0.001). Parental concern and developmental delay were independently associated with decision to operate (P < 0.001). Surgeons with more experience were also more likely to operate across all phenotypes (OR: 2.69, P < 0.004).

Conclusions:
Surgeons responding to this survey were more strongly compelled to operate on children with nonsyndromic sagittal craniosynostosis when head shape was more severe. Additional factors, including parental concern and developmental delay, also influence the decision to operate, especially for moderate phenotypes. Geographic and subspecialty variations were not significant.
Arteriovenous Fistula Rescues Radial Forearm Phalloplasty: A Case Report on Patients with Microvascular Obstruction
Authors: Santucci Richard A. , Newsom Keeley D. , Wachtman Galen S. , Crane Curtis N. 
Published: May 2021

Background:
Radial forearm free flap phalloplasty (RFFFP) is the most common surgery performed for genital reconstruction of female-to-male transgender patients. However, up to 19% require anastomotic re-exploration. The postoperative creation of an arteriovenous fistula (AVF) to bypass obstruction and salvage RFFFP was first reported in 1996 and has subsequently been reported by 1 high-volume center in Belgium.

Methods:
Here, we present 2 cases in which intraoperative microvascular obstruction threatened the viability of the RFFF of transgender phalloplasty patients. In each patient, an AVF was created between the radial artery and cephalic vein in the distal flap either after being transferred out of the operating room, as has previously been described, or during initial operation.

Results:
In both cases, the creation of a distal AVF salvaged the neophallus. Importantly, the patient that had been transferred out of the operating room before reintervention suffered partial flap necrosis compared with no flap loss in the patient who had an AVF created during initial surgery. One AVF was ligated 18 days postoperative, whereas the other was never formally closed.

Conclusions:
These cases demonstrate that AVF can be reliably used for RFFFP salvage both intraoperatively and for reintervention. They also suggest that earlier detection of persistent vascular compromise and utilization of AVF can further minimize flap loss. Finally, in contrast with the prior explanation of this technique, timing of AVF ligation may be less critical than previously described. Microsurgeons are reminded that this technique may save complicated flaps in the uncommon case of microcirculatory flap obstruction.
Proposed Framework for Considering SARS-CoV-2 Antigen Testing of Unexposed Asymptomatic Workers in Selected Workplaces
Authors: Schulte Paul A. , Piacentino John D. , Weissman David N. , de Perio Marie A. , Chiu Sophia K. , Radonovich Lewis J. , Trout Douglas , Beezhold Don , Hearl Frank J. , Howard John 
Published: May 2021

Objectives:
To propose a framework for considering SARS-CoV-2 antigen testing of unexposed asymptomatic workers in selected workplaces.

Methods:
This is a commentary based on established occupational safety and health principles, published articles, and other pertinent literature, including non–peer-reviewed preprints in medrixiv.org prior to April 16, 2021.

Results:
Not applicable to this commentary/viewpoint article.

Conclusion:
Antigen testing is a rapidly evolving and useful public health tool that can be used to guide measures to reduce spread of SARS-CoV-2 in the community and in selected workplaces. This commentary provides a proposed framework for occupational safety and health practitioners and employers for considering antigen testing as a method to screen asymptomatic workers in selected non-healthcare settings. When applied selectively, antigen testing can be a useful, effective part of a comprehensive workplace program for COVID-19 prevention and control.
Localized hair loss in infancy: a review
Authors: McKenzie Paige L. , Castelo-Soccio Leslie 
Published: May 2021

Purpose of review
The current review will address the different causes of localized hair loss in infancy. The data presented here will provide clinicians with the latest understanding of different disorders leading to localized hair loss and will provide recommendations for further management of infants who present with alopecia.

Recent findings
Localized hair loss in infancy is common, but its underlying causes vary greatly. Alopecia in infants can be categorized into congenital, genetic, inflammatory, mechanical, and physiologic causes. Decisions regarding further management are complex, as they often involve not only cosmetic concerns, but also work-up of possible systemic medical issues related to hair loss.

Summary
Clinicians must be able to distinguish between the different causes of infantile hair loss so that appropriate work-up and further management can be pursued. Factors such as physical appearance, timing of presentation, dermoscopic exam, histopathology, and associated systemic features can help lead clinicians to the correct diagnosis in the case of an infant with localized alopecia.
Caenorhabditis elegans as a Diagnostic Aid for Pancreatic Cancer
Authors: Kobayashi Masanori , Fujita Akashi , Ogawa Tomoya , Tanisaka Yuki , Mizuide Masafumi , Kondo Nobuaki , Imaizumi Yuki , Hirotsu Takaaki , Ryozawa Shomei 
Published: May 2021

Objectives
Early detection of pancreatic cancer is notoriously difficult. A novel cancer diagnostic method using the ability of nematodes to detect odor of urine samples has been developed (N-NOSE). This method has a high sensitivity and specificity for various cancers; however, it has not yet been verified in pancreatic cancer. We examined the usefulness of this method to aid early diagnosis of pancreatic cancer in a cancer center.

Methods
We collected urine samples and clinical data from patients hospitalized in our division, between July 2017 and February 2019. We excluded patients with a known current or past history of other cancers. We investigated the relationship between the results of N-NOSE and the presence of pancreatic cancer.

Results
There were 95 noncancer cases and 104 pancreatic cancer cases. The sensitivity and specificity of N-NOSE for pancreatic cancer were 84.6% (88/104) and 60% (57/95), respectively. N-NOSE was able to detect stages 0 to I pancreatic cancer and had a higher correlation with early-stage pancreatic cancer than advanced stage.

Conclusions
N-NOSE has sufficient sensitivity and specificity for use in clinical practice, and it holds great potential as a diagnostic aid for pancreatic cancer, especially for early-stage pancreatic cancer.
Endoscopic Pancreatic Drainage Improves Exocrine Pancreatic Function in Patients With Unresectable Pancreatic Cancer
Authors: Domínguez-Muñoz J. Enrique , de la Iglesia-García Daniel , Nieto-García Laura , Álvarez-Castro Ana , San Bruno-Ruz Alba , Monteserín-Ron Luz , López-Díaz Javier , Iglesias-García Julio 
Published: May 2021

Objectives
Exocrine pancreatic insufficiency is a frequent and clinically relevant complication of pancreatic cancer probably secondary to pancreatic duct obstruction. We aimed at evaluating the impact of endoscopic pancreatic drainage on pancreatic function in patients with unresectable pancreatic cancer.

Methods
A double-blind, prospective, randomized, single-center, interventional study was designed. Patients undergoing endoscopic retrograde cholangiopancreatography for jaundice secondary to unresectable pancreatic cancer were randomized to biliary drainage (group A) or biliopancreatic drainage (group B). Pancreatic function was evaluated by 13C-mixed triglyceride breath test before and 2 weeks after endoscopic retrograde cholangiopancreatography. Breath test result is expressed as 13C-cumulative recovery rate. Abdominal symptoms and nutritional markers were evaluated as secondary outcomes.

Results
Twenty patients were included. Sixteen patients had exocrine pancreatic insufficiency, and 13 completed the study (7 in group A and 6 in group B). The median absolute improvement of 13C-cumulative recovery rate was of 23.75% (interquartile range, 9.62–31.74) after biliopancreatic drainage compared with −1.92% (interquartile range, −4.17 to 13.92) after biliary drainage (P = 0.015). Nutritional markers improved after biliopancreatic drainage, but not after biliary drainage.

Conclusions
Biliopancreatic and not biliary endoscopic drainage is associated with a significant improvement of exocrine pancreatic function in patients with unresectable pancreatic cancer.
Liver Metastasis–Directed Ablative Radiotherapy in Pancreatic Cancer Offers Prolonged Time Off Systemic Therapy in Selected Patients
Authors: Lee Grace , Kim Daniel W. , Oladeru Oluwadamilola T. , Niemierko Andrzej , Gergelis Kimberly R. , Haddock Michael G. , Toesca Diego A. S. , Koong Amanda J. , Owen Dawn , Weekes Colin , Hong Theodore S. , Chang Daniel T. , Hallemeier Christopher L. , Wo Jennifer Y. 
Published: May 2021

Objectives
We evaluated the outcomes of metastatic pancreatic cancer (MPC) patients who underwent liver metastases (LMs)–directed ablative radiotherapy (RT) and sought to characterize patients with more favorable prognosis.

Methods
A retrospective analysis of 76 MPC patients who underwent ablative RT (median dose, 50 Gy) to LM at 3 academic centers between 2008 and 2018 was performed. Endpoints were local control (LC), progression-free survival, and overall survival (OS) since RT.

Results
Median follow-up was 10.9 months. Liver metastases were metachronous in 68%. Before RT, LM was responsive/stable on chemotherapy (CTX) in 36% whereas progressive in 43%. Median carbohydrate antigen 19-9 (CA 19-9) at RT was 334 U/mL. After RT, 32% had ≥6 months of CTX break. Twelve-month outcomes were: LC, 66%; progression-free survival, 7%; and OS, 38%. On multivariable analysis, Eastern Cooperative Oncology Group 2–3 (hazard ratio [HR], 13.49; P < 0.01), progressive LM on CTX (HR, 3.26; P < 0.01), and higher CA 19-9 (log10 scale; HR, 1.39; P < 0.01) at RT predicted worse OS.

Conclusions
Ablative RT to LM in setting of MPC may offer LC of systemic disease and thus quality time off CTX. Selected patients with good performance status, stable/responsive LM on CTX, and lower CA 19-9 have more favorable prognosis.
Predictors of Poor Pathological Response to Neoadjuvant Gemcitabine Plus S-1 Chemotherapy in Patients With Pancreatic Ductal Adenocarcinoma
Authors: Mori Shozo , Aoki Taku , Sakuraoka Yuhki , Shimizu Takayuki , Yamaguchi Takamune , Park Kyung-Hwa , Matsumoto Takatsugu , Shiraki Takayuki , Iso Yukihiro , Kubota Keiichi 
Published: May 2021

Objectives
Although neoadjuvant chemotherapy (NAC)–gemcitabine plus S-1 (GS) has been reported to have a survival benefit in patients with resectable pancreatic ductal adenocarcinoma (PDAC), optimal candidates for NAC-GS have not been clearly identified.

Methods
A total of 81 patients with PDAC who underwent pancreatectomy after NAC-GS between 2013 and 2019 were divided into 2 groups based on Evans classification: grade I (<10% tumor cell destruction, n = 19) and grades II and III (>10% tumor cell destruction, n = 62). Univariate and multivariate analyses using clinical characteristics available before initiation of NAC were performed to predict Evans classification grade I (Evans I).

Results
The overall survival in patients with Evans I was significantly lower than that in patients with Evans II and III (P < 0.001). Multivariate analysis revealed a carcinoembryonic antigen level of >3.6 ng/mL (P = 0.001) and C-reactive protein to albumin ratio of >0.062 (P = 0.017) as independent predictors for Evans I disease. Seven of 11 patients who met both criteria had Evans I disease.

Conclusions
Serum carcinoembryonic antigen and C-reactive protein to albumin ratio are associated with Evans I disease in patients with PDAC who receive NAC-GS. Patients who meet both predictors may not be optimal candidates for NAC-GS.
Thromboembolic Complications in the First Year After Acute Pancreatitis Diagnosis
Authors: Robbins Alexandria J. , Lusczek Elizabeth , Bellin Melena D. , Benner Ashley , Alwan Fatima S. , Beilman Gregory J. 
Published: May 2021

Objectives
This study aimed to quantify the prevalence of venous thromboembolic (VTE) events in patients with pancreatitis requiring hospitalization and its impact on outcomes.

Methods
Adult patients admitted from 2011 to 2018 for pancreatitis were identified. Every admission for pancreatitis in the first year after diagnosis was evaluated for a VTE (pulmonary embolism, deep vein thrombosis, or mesenteric vessel thrombosis) within 30 days of discharge. Characteristics of patients who developed a thromboembolic event were compared with those who did not.

Results
There were 4613 patients with pancreatitis identified, 301 of whom developed a VTE (6.5%). Patients who developed a VTE were more likely to be male (P < 0.01), older (P = 0.03), and have an underlying coagulopathy (P < 0.01). Those with VTEs were more likely to die (27% vs 13%, P < 0.01), have more readmissions for pancreatitis (1.7 vs 1.3, P < 0.01), longer length of stay (16 vs 5.5 days, P < 0.01), and be discharged to acute or long-term rehabilitation rather than home (P < 0.01).

Conclusions
Acute pancreatitis requiring hospitalization is associated with high risk of VTE in the first year after diagnosis. Thromboembolic disease is associated with worse morbidity and mortality.
Hypoxia Promotes Pancreatic Cancer Cell Dedifferentiation to Stem-Like Cell Phenotypes With High Tumorigenic Potential by the HIF-1α/Notch Signaling Pathway
Authors: Mu Rui , Zou Yong-Kang , Tu Kui , Wang Dian-Bei , Tang Dan , Yu Zhou , Zhao Lijin 
Published: May 2021

Objectives
This study aimed to investigate the effect and mechanism of hypoxia on pancreatic cancer (PC) cell dedifferentiation and tumorigenic potential.

Methods
Inhibition of hypoxia-inducible factor 1α (HIF-1α) and overexpression of Notch1 in PC HS766T cell lines were by lentiviral transfection. The expression of stem cell-specific markers C-X-C motif chemokine receptor 4, CD44, and Nestin was detected by immunofluorescence and Western blot assays. Cell invasion capacity was examined by Transwell assay. Tumorigenic potential was measured in an in situ tumor transplantation experiment. The expression of HIF-1α, Notch signals, and apoptosis signals was examined by Western blot assay.

Results
Hypoxia promoted PC cells to dedifferentiate into stem-like cells by upregulating HIF-1α and activating Notch signals. Silencing of HIF-1α significantly repressed cell dedifferentiation and invasion, whereas overexpression of Notch1 reversed the effect of HIF-1α repression. In situ tumor transplantation experiment further confirmed that hypoxia promoted tumorigenic ability through upregulating HIF-1α. Moreover, the expression of HIF-1α and Notch1 was significantly increased in human PC tissues, and high expression of HIF-1α was correlated with poor survival rate.

Conclusions
Hypoxia promoted PC cell dedifferentiation to stem-like cell phenotypes with high tumorigenic potential by activating HIF-1α/Notch signaling pathway, indicating a novel role in regulating PC progression.
Mutation Analysis and Disease Features at Presentation in a Multi‐Center Cohort of Children With Monogenic Cholestasis
Authors: Hertel Paula M. , Bull Laura N. , Thompson Richard J. , Goodrich Nathan P. , Ye Wen , Magee John C. , Squires Robert H. , Bass Lee M. , Heubi James E. , Kim Grace E. , Ranganathan Sarangarajan , Schwarz Kathleen B. , Bozic Molly A. , Horslen Simon P. , Clifton Matthew S. , Turmelle Yumirle P. , Suchy Frederick J. , Superina Riccardo A. , Wang Kasper S. , Loomes Kathleen M. , Kamath Binita M. , Sokol Ronald J. , Shneider Benjamin L. ,   Childhood Liver Disease Research Network (ChiLDReN)
Published: May 2021

ABSTRACTObjectives:To advance our understanding of monogenic forms of intrahepatic cholestasis.Methods:Analyses included participants with pathogenic biallelic mutations in adenosine triphosphate (ATP)‐binding cassette subfamily B member 11 (ABCB11) (bile salt export pump; BSEP) or adenosine triphosphatase (ATPase) phospholipid transporting 8B1 (ATP8B1) (familial intrahepatic cholestasis; FIC1), or those with monoallelic or biallelic mutations in adenosine triphosphate (ATP)‐binding cassette subfamily B member 4 (ABCB4) (multidrug resistance; MDR3), prospectively enrolled in the Longitudinal Study of Genetic Causes of Intrahepatic Cholestasis (LOGIC; NCT00571272) between November 2007 and December 2013. Summary statistics were calculated to describe baseline demographics, history, anthropometrics, laboratory values, and mutation data.Results:Ninety‐eight participants with FIC1 (n = 26), BSEP (n = 53, including 8 with biallelic truncating mutations [severe] and 10 with p.E297G or p.D482G [mild]), or MDR3 (n = 19, including four monoallelic) deficiency were analyzed. Thirty‐five had a surgical interruption of the enterohepatic circulation (sEHC), including 10 who underwent liver transplant (LT) after sEHC. Onset of symptoms occurred by age 2 years in most with FIC1 and BSEP deficiency, but was later and more variable for MDR3. Pruritus was nearly universal in FIC1 and BSEP deficiency. In participants with native liver, failure to thrive was common in FIC1 deficiency, high ALT was common in BSEP deficiency, and thrombocytopenia was common in MDR3 deficiency. sEHC was successful after more than 1 year in 7 of 19 participants with FIC1 and BSEP deficiency. History of LT was most common in BSEP deficiency. Of 102 mutations identified, 43 were not previously reported.Conclusions:In this cohort, BSEP deficiency appears to be correlated with a more severe disease course. Genotype–phenotype correlations in these diseases are not straightforward and will require the study of larger cohorts.
Guidance for Beverages in the Diets of Children Younger Than 2 Years
Authors: Baker Susan S. , Bodnar Lisa M. , Benjamin‐Neelon Sara E. 
Published: May 2021

ABSTRACTRecently the National Academies of Sciences, Engineering, and Medicine reported a summary of recommendations from high‐income countries on what and how to feed infants and children from birth to 2 years of age. The report notes generally consistent recommendations against providing sugar‐sweetened beverages (SSB), juice, and beverages that contain nonnutritive sweeteners to infants and children younger than 2 years. When counselling parents and other caregivers, pediatricians should feel empowered to advise against offering these beverages.
Disparities in Telehealth Utilization Within Pediatric Gastroenterology
Authors: Puthenpura Vidya , Du Nan , Hauptman Leora , Porto Anthony F. 
Published: May 2021

ABSTRACTObjectives:While the use of telemedicine has accelerated significantly with the recent pandemic, it has also magnified disparities in access to telemedicine. This study aims to look at telemedicine utilization patterns within a large pediatric gastroenterology practice.Methods:A retrospective study of ambulatory care visits within Yale‐New Haven Hospital's pediatric gastroenterology practice during the peak expansion of the telemedicine program was conducted. Zip code−level socioeconomic data were obtained using the Distressed Communities Index. A multivariate logistic regression to evaluate disparities between the use of video versus telephone visits was computed, and unadjusted and adjusted odds ratios with 95% confidence intervals (CIs) were obtained.Results:A total of 1273 clinic visits were included in analysis. The majority of the patients listed English as their preferred language, had private insurance, and identified as non‐Hispanic White. When adjusting for co‐variates, having public insurance/Medicaid was associated with decreased odds of having video over telephone visits (adjusted odds ratio [aOR] 0.60; 95% CI 0.44–0.80). Those whose primary language was not English continued to have a statistically significant decreased odds of using video visits (Spanish aOR 0.24; 95% CI 0.13–0.44; other aOR 0.29; 95% CI 0.12–0.72). Within the adjusted multivariate logistic regression, race/ethnicity and SES were, however, no longer found to have a statistically significant decreased odds of video visits.Conclusions:The accelerated implementation of telemedicine within pediatric gastroenterology has given rise to disparities in its use. Further studies are needed to understand these disparities and develop interventions to lessen this gap in usage.
Transient Elastography in Pediatric Liver Disease
Authors: Banc‐Husu Anna M. , Bass Lee M. 
Published: May 2021

ABSTRACTTransient elastography is an imaging technique utilizing shear wave technology to measure liver stiffness. Recent studies have shown success in utilizing this technique in children. Transient elastography is useful in estimating degree of fibrosis in various pediatric liver diseases, including biliary atresia, alpha‐1‐antitrypsin deficiency, Alagille syndrome, cystic fibrosis‐related liver disease, and non‐alcoholic steatohepatitis among others. Confounding factors, however, may affect elastography measurements, such as obesity, severe inflammation, nonfasting state, and hepatic congestion, and should be considered whenever interpreting these measurements. Future studies will correlate liver stiffness on transient elastography and severity of disease.