bluuebunny/crossref_metadata_2025_split huggingface dataset:
MACULAR NEUROVASCULAR ABNORMALITIES IN A CHILD WITH INCONTINENTIA PIGMENTI ON HANDHELD OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY
Authors: Patel Pujan R. , Tai Vincent , Imperio Ryan , Viehland Christian , Izatt Joseph A. , Toth Cynthia A. , Chen Xi 
Published: February 2022

Purpose:
To report macular neurovascular abnormalities in a child with incontinentia pigmenti using handheld optical coherence tomography (OCT) and OCT angiography (OCT-A).

Methods:
An eye of a child with incontinentia pigmenti enrolled in BabySTEPS was imaged using an investigational noncontact, handheld swept-source OCT device during examination under anesthesia. Custom MATLAB scripts were used to generate depth-resolved vascular slabs, B-scans with flow overlay, and retinal thickness maps.

Results:
Depth-resolved OCT and OCT-A imaging demonstrated focal areas of decreased capillary flow that corresponded to areas of both inner retinal and outer retinal thinning on retinal thickness maps. Atypical diving of superficial retinal vessels occurred as they traversed from thin retina to normal-thickness retina.

Conclusion:
Depth-resolved OCT and OCT-A identified retinal vascular abnormalities that were not evident on fundus photography or fluorescein angiography. This case depicted concurrent, localized abnormalities in retinal thickness and microvasculature in an eye with incontinentia pigmenti.
NOVEL RETINAL IMAGING ABNORMALITIES IN ALPORT SYNDROME
Authors: Jadon Trishla , Sadda Srinivas , Singh Geetika , Narayan Parvathy , Chhablani Jay , Venkatesh Pradeep 
Published: February 2022

Purpose:
The purpose of this study was to report a novel observation during retinal screening of a child with Alport syndrome.

Methods:
This was a review of case record and imaging files.

Results:
Clinical examination of the retina and standard color fundus photography revealed no abnormality. However, distinct and identical wrinkling of the temporal macula (fingerprint sign) in both eyes was noted on Optos pseudocolor images of the retina. On optical coherence tomography, there were corresponding “saw-tooth” corrugations in the middle layers of the retina. En face images further highlighted the characteristic nature of this unusual observation.

Conclusion:
Fingerprint sign in the retina, a heretofore undescribed feature, is reported in a child with biopsy confirmed Alport syndrome.
The Effect of COVID-19 on Corneal Donor Volumes and Eye Bank Processes: An Analysis From the Eye Bank of Canada (Ontario Division)
Authors: AlShaker Sara M. , Humphreys Christine , Smigielski Natalie , Chan Clara C. 
Published: February 2022

Purpose:
With the rise in COVID-19 cases, the Eye Bank of Canada (Ontario Division), the largest eye bank in Canada, was faced with challenges related to ocular donor suitability which resulted in tissue shortages after the first wave of COVID-19 cases in Ontario, Canada. This article aims to analyze the impact of COVID-19 on ocular tissue donation and transplant surgeries.

Methods:
Trends in ocular donations in 2020 and the transplant rates were compared with the data from the previous year, as a benchmark of normal eye bank activity.

Results:
Ocular donor volumes decreased during the first wave of the COVID-19 pandemic (March–June 2020) by 65% as compared to the same period in 2019. By the end of the year 2020, this had resulted in a total reduction of 29% of ocular donor volumes as compared to 2019. The ocular transplant surgery volumes in the year 2020 decreased by 32% compared to the previous year, mostly secondary to elective surgery shutdown during the first wave. Because of tissue shortages, the Eye Bank of Canada (Ontario Division) had to import 24 corneas from the United States and cancel 7 surgeries in the year 2020.

Conclusions:
The decline in ocular tissue donor volumes and transplant surgery was a result of an interplay of causes related to the COVID-19 pandemic. Most importantly, ruling out of COVID-19 carriers, lockdown measures affecting tissue retrieval processes, and shutdown of elective surgery were the 3 major factors accounting for tissue shortages and surgical volume reductions.
Establishing a Biomarker for the Prediction of Short-Term Graft Detachment After Descemet Membrane Endothelial Keratoplasty
Authors: Muijzer Marc B. , Noordmans Herke-Jan , Delbeke Heleen , Dickman Mor M. , Nuijts Rudy M. M. A. , Dunker Suryan , Imhof Saskia M. , Wisse Robert P. L. 
Published: February 2022

Purpose:
The purpose of this study was to investigate the predictive value of pachymetry mapping 1 day after Descemet membrane endothelial keratoplasty (DMEK) as a biomarker for early graft detachment.

Methods:
This was a post hoc analysis of 65 pseudophakic subjects with Fuchs endothelial dystrophy who underwent DMEK surgery between December 2018 and April 2021 as part of the Advanced Visualization In Corneal Surgery Evaluation international multicenter randomized controlled trial. One eye per patient was included. Preoperatively and 1 day postoperatively, patients underwent anterior segment optical coherence tomography imaging. Using a grid consisting of 25 zones (ie, pachymetry map), corneal thickness and presence of a graft detachment were mapped for each patient. Detachments of any size were considered, regardless of subsequent clinical interventions. Missing data were imputed and subsequently divided into a training and test set. Two prediction methods were evaluated: one model based on absolute corneal thickness and a regression model.

Results:
A total of 65 eyes were included for analysis of which 33 developed any form of graft detachment. Preoperatively, no significant differences were observed between the groups (P = 0.221). Corneal thickness in the corneal zones with a detached graft was significantly increased compared with corneal zones with an attached graft (P < 0.001). The regression prediction model had an area under the curve of 0.87 (sensitivity: 0.79 and specificity: 0.75), whereas the absolute thickness cutoff model only reached 0.65.

Conclusions:
Pachymetry mapping 1 day after DMEK was predictive for early graft detachment, and the prediction model had a good to excellent performance. This aids in identifying patients at risk for graft detachment and subsequent tailored postoperative care.
Hypothermic machine perfusion for liver graft preservation
Authors: Amin Arpit , Panayotova Guergana , Guarrera James V. 
Published: February 2022

Purpose of review
Ex-vivo machine perfusion has emerged as a promising alternative to static cold storage (SCS) for preservation of liver grafts over the last decade. This review describes the mechanistic benefits associated with hypothermic machine perfusion (HMP) for preservation of liver grafts and highlights clinical outcomes of liver transplantation using HMP technology.

Recent findings
Over the last decade, several single-centre studies have shown decreased biliary complications, decreased early allograft dysfunction (EAD) rates and improved patient survival in liver transplant recipients after application of HMP for liver graft preservation. This has led to initiation of prospective, multicentre, randomized controlled trials (RCTs) in both Europe and North America focused on clinical outcomes in liver transplant recipients using HMP-preserved liver grafts. In addition, recent single-centre studies have shown the utility of perfusate biomarker analysis during HMP in predicting EAD after liver transplantation.

Summary
HMP technology has potential to increase the available donor liver organ pool for liver transplant recipients and improve clinical outcomes after liver transplantation. Broader clinical application of HMP in resuscitation and preservation of liver grafts is anticipated over the next decade once regulatory, logistical and financial challenges are overcome.
Cystic fibrosis: candidate selection and impact of the cystic fibrosis transmembrane conductance regulator therapy
Authors: Lehr Carli J. , Pilewski Joseph M. 
Published: February 2022

Purpose of review
Over the past decade, the development of highly effective cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulators has dramatically ameliorated the manifestations of CF for most patients. Perhaps most importantly, CFTR modulators impact the development and progression of advanced lung disease (ALD) and are changing the CF population accessing lung transplant.

Recent findings
A recent phase 3 trial of elexacaftor/tezacaftor/ivacaftor (ETI) demonstrated efficacy for individuals with at least one copy of the most common CF mutation, F508del. Studies of CFTR modulator therapy in patients with ALD have demonstrated similar improvements in lung function, nutrition, and pulmonary exacerbation frequency as seen in individuals with higher lung function. Due to improvements with ETI, rates of lung transplant for CF have declined and individuals are achieving stability in lung function. Nevertheless, the Cystic Fibrosis Foundation guidelines for lung transplant referral should be used to guide referral decisions for all individuals with CF, including those on CFTR modulator therapy, to allow remediation of modifiable barriers to transplant. ETI may be used in the posttransplant setting but for selected individuals and with close monitoring.

Summary
Increasing access to highly effective CFTR modulators has changed the trajectory of lung disease in CF for many, but not all, individuals and there remain individuals who cannot access therapy or whose mutations do not respond to modulators. Lung transplant remains an important treatment option for individuals with advanced CF lung disease. Increasing attention will be required to optimize decisions of when to list for transplant.
Comparison of the Lipidomic Signature of Fatty Liver in Children and Adults
Authors: Mann Jake P. , Jenkins Benjamin , Furse Samuel , Snowden Stuart G. , Alisi Anna , Draijer Laura G. , Karnebeek Kylie , Kelly Deirdre A. , Koot Bart G. , Mosca Antonella , Salvestrini Camilla , van Mourik Indra , Vreugdenhil Anita , Zilbauer Matthias , Koulman Albert ,   EU‐PNAFLD investigators^
Published: February 2022

ABSTRACTObjective:Non‐alcoholic fatty liver disease (NAFLD) is an increasingly common condition in children characterised by insulin resistance and altered lipid metabolism. Affected patients are at increased risk of cardiovascular disease (CVD) and children with NAFLD are likely to be at risk of premature cardiac events. Evaluation of the plasma lipid profile of children with NAFLD offers the opportunity to investigate these perturbations and understand how closely they mimic the changes seen in adults with cardiometabolic disease.Methods:We performed untargeted liquid chromatography—mass spectrometry (LC–MS) plasma lipidomics on 287 children: 19 lean controls, 146 from an obese cohort, and 122 NAFLD cases who had undergone liver biopsy. Associations between lipid species and liver histology were assessed using regression adjusted for age and sex. Results were then replicated using data from 9500 adults with metabolic phenotyping.Results:More severe paediatric NAFLD was associated with lower levels of long chain, polyunsaturated phosphatidylcholines (pC) and triglycerides (TG). Similar trends in pC and TG chain length and saturation were seen in adults with hepatic steatosis; however, many of the specific lipids associated with NAFLD differed between children and adults. Five lipids replicated in adults (including PC(36:4)) have been directly linked to death and cardiometabolic disease, as well as indirectly via genetic variants.Conclusion:These findings suggest that, whilst similar pathways of lipid metabolism are perturbed in paediatric NAFLD as in cardiometabolic disease in adults, the specific lipid signature in children is different.
C-Reactive Protein and the Outcome of a Pediatric Emergency Department 7 Days Revisit
Authors: Glatstein Miguel , Salamon Lotan , Soffer Gili Palnizky , Capua Tali , Scolnik Dennis , Rimon Ayelet 
Published: February 2022

Background
Pediatric emergency department (PED) return visits represent an important quality of care metric and constitute a patient-centered outcome. C-reactive protein (CRP) is an inflammatory biomarker that is commonly used as screening tool in the PED. In this study, we assessed the clinical outcomes of children whose levels of CRP are 150 mg/L or higher at the initial PED visit and if such levels could be useful in predicting outcomes at a second PED visit.

Methods
A historical cohort study of all patients who visited the PED between July 2007 and June 2017 and had a CRP value of 150 mg/L or greater in the setting of a febrile illness. Data of patients with a return visit to the PED within 7 days were assessed for an association between laboratory values, diagnosis and clinical outcome.

Results
One hundred thirty-six index visits were included in this study. One hundred fifteen (84.6%) of the revisits were discharged after their second visit, and 21 (15.4%) were admitted to the inpatient unit. Admitted patients did not differ from patients who were discharged home in diagnosis and CRP levels, but a difference in white blood cell and absolute neutrophil counts was observed.

Conclusions
The intensity of the inflammatory response, as expressed by the high concentrations of CRP in children, does not seem to predict the outcome at a repeat PED visit within 7 days.
Custom Plates in Orthognathic Surgery: A Single Surgeon’s Experience and Learning Curve
Authors: Fleury Christopher M. , Sayyed Adaah A. , Baker Stephen B. 
Published: February 2022

Background:
Virtual surgical planning (VSP) now allows for the fabrication of custom plates in orthognathic surgery. The senior author was an early adopter, using VSP and stereolithographic splints for over a decade, before transitioning to custom plates in 2019. The authors present our experience and learning curve with this new technology and compare results to a prior cohort of orthognathic patients.

Methods:
A retrospective chart review identified patients undergoing orthognathic surgery with the senior author between 2016 and 2021. All underwent VSP and stereolithographic splint formation, and then either traditional or custom-plate fixation. Demographics, perioperative variables, and postoperative outcomes were analyzed. Traditional fixation consisted of craniomaxillofacial plates, bent intraoperatively by the surgeon to adapt to the facial skeleton. Custom plates were prefabricated and prebent to fit drill holes outlined by customized cutting guides.

Results:
Forty-three patients underwent surgery in the study period, 25 (58.1%) with traditional fixation hardware and 18 (41.9%) with custom plates. The surgical technique evolved throughout the custom-plate cohort, with the most recent technique involving custom maxillary plate fixation and traditional mandibular plate fixation. When comparing this group of patients to the prior cohort of traditional fixation patients, operative time significantly decreased (mean 233 minutes versus 283 minutes, P = 0.044), without significant difference in complications.

Conclusions:
Patient-specific cutting guides and custom plates allow for precise spatial positioning of the osteotomized jaw in the orthognathic surgery. Unsurprisingly, obstacles must be overcome in adopting this new technology; here, we outline our experience and technical modifications that have resulted in increased surgical efficiency with comparable outcomes.
Sliding Flap for the Wide Upper Eyelid Margin Defect After Cancer Removal
Authors: Mizobuchi Ryo , Nojiri Gaku , Uchiyama Mizuki , Shimizu Azusa , Kamimori Tomoki , Hayashi Ayato 
Published: February 2022

Background:
Reconstruction of eyelid margin defects following resection of upper-eyelid skin malignancies is typically performed using a lower eyelid switch flap, including eyelash reconstruction. However, a subsequent procedure for flap separation, and prolonged swelling of the flap may occur as a complication.

Objective:
The authors performed anterior lamellar reconstruction using a sliding flap with excess upper eyelid skin, a procedure that is a less invasive and simpler.

Materials and Methods:
The authors performed anterior lamellar reconstruction using a sliding flap in 7 patients with full-thickness upper eyelid margin defect after skin cancer resection. The mean age of the patients was 76.0 years. The horizontal width of the defect in our cohort ranged from 11 to 25 mm and the vertical width ranged from 5 to 10 mm.

Results:
All the flaps and mucosal grafts were well taken, and none of the patients complained of lack of eyelash reconstruction. However, eyelid margin irregularity, possibly due to flap or mucosal graft contraction, was observed in 2 patients, and 1 patient developed keratoconjunctivitis.

Conclusions:
The sliding flap technique is a minimally invasive and simple procedure for wide eyelid margin reconstruction. However, 2 patients developed eyelid margin irregularities and 1 patient developed keratoconjunctivitis. Those complications might have occurred owing to the condition of posterior lamellar reconstruction. Therefore, for the successful use of a sliding flap for anterior lamellar reconstruction, the form of the eyelid edge and the choice of posterior lamellar reconstruction are key considerations. In future, we plan to establish a better reconstructive technique by accumulating more evidence.
Positron Emission Tomography/Magnetic Resonance Imaging (PET/MRI) Versus the Standard of Care Imaging in the Diagnosis of Peritoneal Carcinomatosis
Authors: Furtado Felipe S. , Wu Mark Z. , Esfahani Shadi A. , Ferrone Cristina R. , Blaszkowsky Lawrence S. , Clark Jeffrey W. , Ryan David P. , Goyal Lipika , Franses Joseph W. , Wo Jennifer Y. , Hong Theodore S. , Qadan Motaz , Tanabe Kenneth K. , Weekes Colin D. , Cusack James C. , Crafa Francesco , Mahmood Umar , Anderson Mark A. , Mojtahed Amirkasra , Hahn Peter F. , Caravan Peter , Kilcoyne Aoife , Vangel Mark , Striar Robin M. , Rosen Bruce R. , Catalano Onofrio A. 
Published: February 2022

Objective:
To compare positron emission tomography (PET)/magnetic resonance imaging (MRI) to the standard of care imaging (SCI) for the diagnosis of peritoneal carcinomatosis (PC) in primary abdominopelvic malignancies.

Summary Background Data:
Identifying PC impacts prognosis and management of multiple cancer types.

Methods:
Adult subjects were prospectively and consecutively enrolled from April 2019 to January 2021. Inclusion criteria were: 1) acquisition of whole-body contrast-enhanced (CE) 18F-fluorodeoxyglucose PET/MRI, 2) pathologically confirmed primary abdominopelvic malignancies. Exclusion criteria were: 1) greater than 4 weeks interval between SCI and PET/MRI, 2) unavailable follow-up. SCI consisted of whole-body CE PET/computed tomography (CT) with diagnostic quality CT, and/or CE-CT of the abdomen and pelvis, and/or CE-MRI of the abdomen±pelvis. If available, pathology or surgical findings served as the reference standard, otherwise, imaging followup was used. When SCI and PET/MRI results disagreed, medical records were checked for management changes. Follow-up data were collected until August 2021.

Results:
One hundred sixty-four subjects were included, 85 (52%) were female, and the median age was 60 years (interquartile range 50–69). At a subject level, PET/MRI had higher sensitivity (0.97, 95% CI 0.86–1.00) than SCI (0.54, 95% CI 0.37–0.71), P < 0.001, without a difference in specificity, of 0.95 (95% CI 0.90–0.98) for PET/MRI and 0.98 (95% CI 0.93–1.00) for SCI, P ¼ 0.250. PET/MRI and SCI results disagreed in 19 cases. In 5/19 (26%) of the discordant cases, PET/MRI findings consistent with PC missed on SCI led to management changes.

Conclusion:
PET/MRI improves detection of PC compared with SCI which frequently changes management.
The Longitudinal Course of Low-anterior Resection Syndrome
Authors: Varghese Chris , Wells Cameron I. , O’Grady Greg , Christensen Peter , Bissett Ian P. , Keane Celia ,   on behalf of the Longitudinal LARS Group∗
Published: February 2022

Objective:
We aimed to better understand the longitudinal course of low anterior resection syndrome (LARS) to guide patient expectations and identify those at risk of persisting dysfunction.

Summary Background Data:
LARS describes disordered bowel function after rectal resection that significantly impacts quality of life.

Methods:
MEDLINE, EMBASE, CENTRAL, and CINAHL databases were systematically searched for studies that enrolled adults undergoing anterior resection for rectal cancer and used the LARS score to assess bowel function at ≥2 postoperative time points. Regression analyses were performed on deidentified patient-level data to identify predictors of change in LARS score from baseline (3–6months) to 12-months and 18–24 months.

Results:
Eight studies with a total of 701 eligible patients were included. The mean LARS score improved over time, from 29.4 (95% confidence interval 28.6–30.1) at baseline to 16.6 at 36 months (95% confidence interval 14.2%–18.9%). On multivariable analysis, a greater improvement in mean LARS score between baseline and 12 months was associated with no ileostomy formation [mean difference (MD) –1.7 vs 1.7, P < 0.001], and presence of LARS (major vs minor vs no LARS) at baseline (MD –3.8 vs –1.7 vs 5.4, P < 0.001). Greater improvement in mean LARS score between baseline and 18–24 months was associated with partial mesorectal excision vs total mesorectal excision (MD–8.6 vs 1.5, P < 0.001) and presence of LARS (major vs minor vs no LARS) at baseline (MD –8.8 vs –5.3 vs 3.4, P < 0.001).

Conclusions:
LARS improves by 18 months postoperatively then remains stable for up to 3 years. Total mesorectal excision, neoadjuvant radiotherapy, and ileostomy formation negatively impact upon bowel function recovery.
Introduction to the theme issue ‘Species' ranges in the face of changing environments’
Authors: Rafajlović Marina , Alexander Jake M. , Butlin Roger K. , Johannesson Kerstin 
Published: February 2022

Understanding where, when and how species’ ranges will be modified is both a fundamental problem and essential to predicting how spatio-temporal environmental changes in abiotic and biotic factors impact biodiversity. Notably, different species may respond disparately to similar environmental changes: some species may overcome an environmental change only with difficulty or not at all, while other species may readily overcome the same change. Ranges may contract, expand or move. The drivers and consequences of this variability in species' responses remain puzzling. Importantly, changes in a species’ range creates feedbacks to the environmental conditions, populations and communities in its previous and current range, rendering population genetic, population dynamic and community processes inextricably linked. Understanding these links is critical in guiding biodiversity management and conservation efforts. This theme issue presents current thinking about the factors and mechanisms that limit and/or modify species' ranges. It also outlines different approaches to detect changes in species’ distributions, and illustrates cases of range modifications in several taxa. Overall, this theme issue highlights the urgency of understanding species' ranges but shows that we are only just beginning to disentangle the processes involved. One way forward is to unite ecology with evolutionary biology and empirical with modelling approaches.
This article is part of the theme issue ‘Species’ ranges in the face of changing environments (Part II)’.