bluuebunny/crossref_metadata_2025_split huggingface dataset:
Editorial: COVID-19 immunology and organ transplantation
Authors: Bery Amit I. , Kulkarni Hrishikesh S. , Kreisel Daniel 
Published: February 2021

Purpose of review
The aim of this study was to provide a critical appraisal of the literature on the effects of the COVID-19 pandemic on organ transplantation, with a specific focus on lung transplantation given the predominant pulmonary involvement of the virus.

Recent findings
There was a significant decrease in lung transplant volumes during the first wave of the COVID-19 pandemic due to a combination of reduced availability of donors and an imbalance between waitlist additions and inactivations. SARS-CoV-2 infection was subsequently associated with an exuberant immune response that can lead to the development of postinfectious fibrotic lung disease. Few lung transplants have been performed in previously infected recipients and long-term outcomes remain unknown. Although the lung transplant volume rebounded during the second wave, it is unclear what the long-term effects of healthcare resource limitation and public health measures will have on transplant volumes in the future. Outcomes after SARS-CoV-2 infection in previous lung transplant recipients appear to be worse than the general public, and, although an immunosuppressed state likely contributes to these outcomes, whether immunosuppression should be altered in those exposed to or infected with SARS-CoV-2 remains unanswered in the absence of unequivocal data.

Summary
The COVID-19 pandemic has presented a number of challenges for lung transplant programs across the globe. Multiple research questions remain to be answered in order to optimally manage lung transplant recipients in the context of this pandemic.
Oncologic indications of liver transplantation and deceased donor liver allocation in the United States
Authors: Kubal Chandrashekhar , Mihaylov Plamen , Holden John 
Published: February 2021

Purpose of review
Liver transplantation is a standard therapy for certain liver cancers. The majority of liver transplantation in the United States is through deceased donor liver transplantation (DDLT). A significant disparity between the demand of livers and patients awaiting liver transplantation still remains, relying on United Network for Organ Sharing (UNOS) to make policies to determine priority amongst recipients, including for patients with liver cancer. We review the scope of liver transplantation in patients with liver cancer with a focus on hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (iCCA), and unresectable colorectal liver metastases (CRLM) with respect to current liver allocation policy.

Recent findings
Recently, liver allocation changed in the United States. Under the current allocation policy, select patients with HCC and hilar CCA (hCCA) receive priority with an exception score of median MELD score at transplant (MMAT)-3. There is scope for other liver cancers, such as iCCA and CRLM to be considered, as reasonable outcomes have been achieved in these patients outside of the United States through DDLT and living donor liver transplantation (LDLT).

Summary
With the growing experience of liver transplantation for nonconventional oncologic indications, the current policy for prioritization of liver cancer within deceased donor liver allocation may need to be re-evaluated.
Severe Acute Respiratory Syndrome Coronavirus‐2 Infection in Children With Liver Transplant and Native Liver Disease
Authors: Kehar Mohit , Ebel Noelle H. , Ng Vicky L. , Baquero Jairo Eduardo Rivera , Leung Daniel H. , Slowik Voytek , Ovchinsky Nadia , Shah Amit A. , Arnon Ronen , Miloh Tamir , Gupta Nitika , Mohammad Saeed , Kogan‐Liberman Debora , Squires James E. , Sanchez Maria Camila , Hildreth Amber , Book Linda , Chu Christopher , Alrabadi Leina , Azzam Ruba , Chepuri Bhavika , Elisofon Scott , Falik Rachel , Gallagher Lisa , Kader Howard , Mogul Douglas , Mujawar Quais , Namjoshi Shweta S. , Valentino Pamela L. , Vitola Bernadette , Waheed Nadia , Zheng Ming‐Hua , Lobritto Steven , Martinez Mercedes 
Published: February 2021

ABSTRACTObjective:Increased mortality risk because of severe acute respiratory syndrome coronavirus‐2 (SARS‐CoV2) infection in adults with native liver disease (LD) and liver transplant (LT) is associated with advanced age and comorbid conditions. We aim to report outcomes for children with LD and LT enrolled in the NASPGHAN/SPLIT SARS‐CoV2 registry.Methods:In this multicenter observational cohort study, we collected data from 91 patients <21 years (LD 44, LT 47) with laboratory‐confirmed SARS‐CoV2 infection between April 21 and September 17, 2020.Results:Patients with LD were more likely to require admission (70% vs 43% LT, P = 0.007) and pediatric intensive care unit (PICU) management (32% vs 4% LT, P = 0.001). Seven LD patients required mechanical ventilation (MV) and 2 patients died; no patients in the LT cohort died or required MV. Four LD patients presented in pediatric acute liver failure (PALF), 2 with concurrent multisystem inflammatory syndrome in children (MIS‐C); all recovered without LT. Two LD patients had MIS‐C alone and 1 patient died. Bivariable logistic‐regression analysis found that patients with nonalcoholic fatty LD (NAFLD) (odds ratio [OR] 5.6, P = 0.02) and LD (OR 6.1, P = 0.01, vs LT) had higher odds of severe disease (PICU, vasopressor support, MV, renal replacement therapy or death).Conclusions:Although not directly comparable, LT recipients had lower odds of severe SARS‐CoV2 infection (vs LD), despite immunosuppression burden. NAFLD patients reported to the registry had higher odds of severe SARS‐CoV2 disease. Future controlled studies are needed to evaluate effective treatments and further stratify LD and LT patients with SARS‐CoV2 infection.
Reliable Detection of Intrapulmonary Shunts Using Contrast‐Enhanced Echocardiography in Children With Portal Hypertension or Portosystemic Shunt
Authors: Rock Nathalie M. , Beghetti Maurice , Tissot Cécile , Willi Jean‐Pierre , Bouhabib Maya , McLin Valérie A. , Maggio Albane B.R. 
Published: February 2021

ABSTRACTObjectives:The aim of this study was to analyze if contrast‐enhanced echocardiography (CEE) is as reliable as lung perfusion scintigraphy (LPS) to detect intrapulmonary shunting (IPS) in children with portal hypertension (PHTN) or congenital/surgical portosystemic shunts (PSS) and to define the number of cardiac cycles required to exclude intrapulmonary shunting.Methods:Inclusion criteria for this cross‐sectional study were: (1) presence of PHTN or PSS diagnosed on abdominal ultrasound, (2) technically valid saline contrast echocardiography, (3) lung perfusion scintigraphy within 6 months of CEE. The number of cardiac cycles between right atrial opacification and the arrival of contrast in the left atrium were counted. We analyzed our CEE data at three and five cardiac cycles and compared them with LPS results.Results:The study population was composed of 78 children (38 girls, 49%) ages 2.1–18.8 years (mean 9.8). Sixty‐nine patients had PHTN (88%), and nine had a PSS (11%). Eleven subjects (14%) presented evidence of IPS on LPS. Peripheral oxygen saturation was lower in the subjects with IPS detected on LPS (95.3 ± 1.7% vs 99.0 ± 1.4%; P < 0.01). Comparison of LPS with CEE before three and five cardiac cycles showed that CEE is highly specific (95.7%) as early as three cardiac cycles with markedly better sensitivity (72.7%) when using five cardiac cycles. Furthermore, a negative study using five cardiac cycles ruled out IPS with a 95% negative predictive value. The cardiac cycle at which the bubbles appeared in the left atrium was inversely correlated to the shunt index measured using LPS (r = −0.563; P = 0.001).Conclusion:CEE is sufficient for the screening of IPS in children with PHTN or congenital/surgical PSS, obviating the need for LPS.
Seroprevalence of Hepatitis C in Children Without Identifiable Risk‐Factors
Authors: Melikoki Vasiliki , Kourlaba Georgia , Kanavaki Ino , Fessatou Smaragdi , Papaevangelou Vassiliki 
Published: February 2021

ABSTRACTObjectives:Hepatitis C virus (HCV) remains a major public health burden for >30 years since its discovery. It is estimated that >80 million people have been already infected. Direct‐acting antiviral (DAA) treatment is now approved for young children over the age of 3 years. Treating children before the development of high‐risk behaviors is optimal. Thus, assessing the current epidemiology of HCV in children becomes important and may promote awareness.Methods:Articles describing the prevalence of hepatitis C in children, were systematically reviewed. To assess HCV infection prevalence in the general population, studies discussing high‐risk groups alone were excluded.Results:Data from 58 studies were analyzed. National data was scarce. An overall prevalence of HCV in children of 0.87% was found, ranging from 0.34% in Europe to 3.02% in Africa. Prevalence of viremic infection is important and data synthesis from available data indicated that HCV viremia was detected in 56.8% of children. The prevalence of HCV according to sex was described in 25 studies but no difference between sexes was detected. HCV prevalence was significantly higher in children older than 10 years (0.97%) when compared to those ages under 10 years old (0.75%, P < 0.001).Conclusions:Considering probable underdiagnosis of HCV infection in children, this information reveals that prevalence is substantial. One may argue that future strategies aiming towards HCV elimination, may need to include antiviral treatment of pre‐adolescent children as well.
Validity and Responsiveness of the Patient‐reported Outcomes Measurement Information System in Children With Ulcerative Colitis
Authors: Brenner Erica J. , Long Millie D. , Mann Courtney M. , Lin Li , Chen Wenli , Reyes Camila , Bahnson Kirsten M. , Reeve Bryce B. , Kappelman Michael D. 
Published: February 2021

ABSTRACTObjectives:Patient‐reported outcome measures allow children to directly report on their health and well‐being. We assessed the construct validity and responsiveness of the Patient‐Reported Outcomes Measurement Information System (PROMIS) Pediatric measures in children and adolescents with ulcerative colitis (UC).Methods:Through the Inflammatory Bowel Disease Partners Kids & Teens’ Internet‐based cohort, children with UC reported symptoms related to disease activity (Pediatric Ulcerative Colitis Activity Index), IMPACT‐III health‐related quality of life measure, and 5 PROMIS Pediatric measures (anxiety, depressive symptoms, pain interference, fatigue, and peer relationships). We included participants aged 9 to 17 years and conducted cross‐sectional and longitudinal, mixed‐linear regression analyses to examine the extent to which PROMIS Pediatric scores are associated with and respond to changes in Pediatric Ulcerative Colitis Activity Index and IMPACT‐III.Results:We evaluated 91 participants with UC (mean age 13 years, 57% girls). Better PROMIS Pediatric scores were associated with lower disease activity, in both cross‐sectional and longitudinal analyses. For a change from moderate/severe to remission, observed effect estimates were −5.1 points for anxiety, −5.0 for depressive symptoms, −14.7 for pain interference, −13.7 for fatigue, and 5.3 for peer relationships (P < 0.05 for all domains). Better PROMIS Pediatric scores were associated with improved IMPACT‐III scores (P values <0.01), and changes in scores were moderately correlated with changes in IMPACT‐III over time (adjusted P values <0.01).Conclusions:This study provides evidence for the construct validity and longitudinal responsiveness of the PROMIS Pediatric measures in pediatric patients with UC, thus supporting their use in clinical research and patient care.
Clinical Predictors of Morbidity and Mortality in Hospitalized Pediatric Patients With Ascites
Authors: Ingviya Thammasin , Wasuwanich Paul , Scheimann Ann O. , Felix Grace , Laengvejkal Pavis , Vasilescu Alexandra , Imteyaz Hejab , Seaberg Eric C. , Karnsakul Wikrom 
Published: February 2021

ABSTRACTObjectives:Ascites is a pathologic buildup of fluid in the peritoneal cavity. Knowledge is lacking in clinical outcome in pediatric patients with ascites. We aim to identify and assess clinical variables, associated with morbidity and mortality in pediatric patients who are hospitalized with ascites.Methods:A retrospective cohort study was performed on patients ages 0 to 21 hospitalized at Johns Hopkins Hospital between 1983 and 2010 with an ICD‐9 discharge diagnosis of ascites (789.5, 789.51, 789.59). A total of 518 pediatric patients were studied, all with a diagnosis of ascites during hospitalization. Study outcomes included hospital length of stay (LOS) as a proxy for morbidity and death at hospital discharge for mortality. Variables analyzed included demographic data, ascites etiology and grade, comorbidities, and laboratory markers. Variables were analyzed by log‐linear regression and competing risk model.Results:Among the 3 age groups (0–5, 6–12, and 13–21), the 0 to 5 age group experienced significantly increased LOS (P < 0.001) and mortality (P = 0.027). Ascites etiology of veno‐occlusive disease (VOD) and the presence of hydrothorax or thrombocytopenia was also significantly associated with increased LOS. Ascites with the etiology of congestive hepatopathy and the presence of grade 3 ascites, hepatic encephalopathy, hepatorenal syndrome, hydrothorax, hyponatremia, and thrombocytopenia were associated with increased mortality. Additionally, black pediatric patients have an increased risk of mortality (P = 0.027). Other factors including sex, leukopenia, portal vein thrombosis, and splenomegaly were not associated with LOS or mortality.Conclusions:Morbidity and mortality in pediatric patients hospitalized with ascites are associated with specific demographic and clinical factors. Further studies are required to apply this knowledge to predict the clinical outcomes.
Safety of High‐Volume Plasmapheresis in Children With Acute Liver Failure
Authors: Jørgensen Marianne Hørby , Rasmussen Allan , Christensen Vibeke Brix , Jensen Anne‐mette Bæk , Fonsmark Lise , Andreassen Bente Utoft , Damholt Mette Brimnes , Larsen Fin Stolze 
Published: February 2021

ABSTRACTObjectives:Paediatric acute liver failure (P‐ALF) is a rare condition and is associated with a high mortality rate. Management of P‐ALF aims to stabilise vital organ functions and to remove circulating toxins and provide vital plasma factors that are lacking. High‐volume plasmapheresis (HVP) removes protein‐bound substances and improves survival in adult ALF. It is unknown if this effect can be extrapolated to P‐ALF. The aim of this study is to report the safety and feasibility of HVP in P‐ALF.Methods:Children with P‐ALF were offered HVP if bilirubin was higher than 200 μmol/L or if the aetiology was toxic hepatitis. HVP was performed with fresh frozen plasma corresponding to 10% of the body weight on a minimum of 3 consecutive days. Diagnostics, biochemical and clinical data during HVP as well as outcome data after 3 months were collected from 2012 to 2019 and retrospectively analysed.Results:Sixteen children were treated by HVP and completed at least one series of three treatment sessions with HVP. The only complication seen was an increase in pH > 7.55 in three children within the first 12 hours and was corrected with hydrochloric acid. No bleeding or septic episodes were noted during HVP. Eight children survived without liver transplantation, two survived after successful grafting and a total of six children died. The liver injury unit score between survivors with their own liver and the rest, the two groups was significantly different (P = 0.005).Conclusion:HVP with fresh frozen plasma is feasible and well tolerated in children with P‐ALF. No serious adverse events and no procedure‐related mortality were observed.
High Rates of Central Venous Line Replacement or Revision in Children With Cancer at US Children’s Hospitals
Authors: Buonpane Christie L. , Lautz Timothy B. , Langer Monica 
Published: February 2021

Background:
Most children with cancer utilize a central venous line (CVL) for treatment. Complications often necessitate early replacement, revision, or addition (RRA), but the rate of these procedures is not known. This study sought to determine rates of RRA in pediatric oncology patients, and associated risk factors.

Materials and Methods:
Data queried from the Pediatric Health Information System including patients ≤18 years old with malignancy and CVL placement. Analysis included: first CVL placement of the calendar year and subsequent procedures for 6 months thereafter.

Results:
A total of 6553 children met inclusion criteria (55.9% male, median age 6 years, interquartile range: 2 to 12). RRA within 6 months was required in 25.6% of patients, with 1.7% requiring 5 or more lines. Patients with Central Line-Associated Bloodstream Infection (CLABSI) were 2.78 times more likely to require RRA within 6 months of initial CVL placement, but accounted for only 16% of RRA patients. Factors associated with RRA were age below 1 year, CLABSI, hematologic malignancy, malnutrition, clotting disorder, deep vessel thromboembolism, and obesity. Patients with implantable ports as initial CVL (42%) were less likely to need RRA.

Conclusion:
Twenty-five percent require at least 1 RRA within 6 months, with associated morbidity and costs. Though strongly associated, most revisions were not related to CLABSI episodes.
Underlying CTLA4 Deficiency in a Patient With Juvenile Idiopathic Arthritis and Autoimmune Lymphoproliferative Syndrome Features Successfully Treated With Abatacept—A Case Report
Authors: Mazzoni Marta , Dell’Orso Gianluca , Grossi Alice , Ceccherini Isabella , Viola Stefania , Terranova Paola , Micalizzi Concetta , Guardo Daniela , Massaccesi Erika , Palmisani Elena , Calvillo Michaela , Fioredda Francesca , Malattia Clara , Dufour Carlo , Ravelli Angelo , Miano Maurizio 
Published: February 2021

Background:
Functional variants of the cytotoxic T-lymphocyte antigen-4 (CTLA4) could contribute to the pathogenesis of disorders characterized by abnormal T-cell responses.

Case Presentation:
We report a case of a 13-year-old girl who first presented with polyarticular juvenile idiopathic arthritis poorly responsive to treatment. During the following years the patient developed cytopenias, chronic lymphoproliferation, high values of T-cell receptor αβ+ CD4- CD8- double-negative T cells and defective Fas-mediated T cells apoptosis. Autoimmune lymphoproliferative syndrome was diagnosed and therapy with mycophenolate mofetil was started, with good hematological control. Due to the persistence of active polyarthritis, mycophenolate mofetil was replaced with sirolimus. In the following months the patient developed hypogammaglobulinemia and started having severe diarrhea. Histologically, duodenitis and chronic gastritis were present. Using the next generation sequencing-based gene panel screening, a CTLA4 mutation was detected (p.Cys58Serfs*13). At the age of 21 the patient developed acute autoimmune hemolytic anemia; steroid treatment in combination with abatacept were started with clinical remission of all symptoms, even arthritis.

Conclusions:
Targeted immunologic screening and appropriate genetic tests could help in the diagnosis of a specific genetically mediated immune dysregulation syndrome, allowing to select those patients who can take advantage of target therapy, as in the case of abatacept in CTLA4 deficiency.
Expectations of Motherhood and Quality of Life
Authors: Adams Mary K. , Byrn Mary , Penckofer Sue , Bryant Fred , Almonte Angelica 
Published: February 2021

Abstract

Purpose:
The purpose of this study was to examine 1) discrepancies between expectations of motherhood and the experience of motherhood in the first 6 to 12 weeks postpartum, 2) relationships between maternal quality of life, mood, parental attitudes, and expectations, and 3) predictors of quality of life.

Study Design and Methods:
We used a descriptive, correlational design. The sample consisted of first-time mothers who were at or beyond 34 weeks pregnant with no reported history of anxiety or depression. The following questionnaires were administered during pregnancy and 6 to 12 weeks postpartum: Parenting Expectations Measure, General Anxiety Disorder 7, Edinburgh Postnatal Depression Screen, Intensive Parenting Attitudes Questionnaire, and Ferrans and Powers Quality of Life Index.

Results:
Sixty-one mothers participated. Based on scores from the Parenting Expectations Measure, 44% of participants had expectations of motherhood that were not met. Expectations were a significant predictor of quality of life during pregnancy and postpartum.

Clinical Implications:
Unmet expectations are important to understand when identifying modifiable risk factors of postpartum anxiety and depression in women without other risk factors. A discussion of expectations during antepartum care may minimize poor quality of life which is associated with anxiety and depression in women without anxiety and depressive symptoms.
Prevention of Pruritis following Spinal Morphine for Scheduled Cesarean Birth
Authors: Payne Christopher , Curtis Brian , Dan Devon , Dunston Shaun , Moore Chad , Hefley Justin 
Published: February 2021

Abstract

Background:
Intrathecal morphine provides effective analgesia after cesarean birth, yet up to 90% of women who receive it experience excessive itching, an undesirable dose-dependent effect. Pruritis may increase nursing workload, delay breastfeeding, and decrease patient satisfaction. When 0.1 mg spinal morphine is given, pruritis is markedly reduced while analgesia is preserved.

Purpose:
The purpose of this project was to determine possible causes and solutions for pruritus after cesarean birth.

Methods:
Anesthesia providers were educated and encouraged to limit spinal morphine to 0.1 mg as a strategy to prevent pruritus. In a repeated measures design, the rate of treatment-required pruritus and opioid consumption were measured 24 hours after surgery. The project included an evaluation of 30 medical records before and 30 medical records after the project intervention.

Results:
Preintervention rate of treatment-required pruritis was 37%, all received spinal morphine ≥ 1.5 mg. Postintervention rate of treatment-required pruritis was 13% and 57% after spinal morphine 0.1 mg and 0.2 mg, respectively. Opioid consumption was similar between groups.

Clinical Implications:
Mother–baby nurses can have an impact on the practice of anesthesia providers by advocating for evidence-based dosing of intrathecal morphine to reduce the incidence of pruritis while maintaining effective analgesia for women after cesarean birth.
Metacognition Mediates the Relationship Between Maladaptive Personality Traits and Levels of Personality Functioning
Authors: Pedone Roberto , Barbarulo Anna Maria , Colle Livia , Semerari Antonio , Grimaldi Pietro 
Published: February 2021

Abstract
The impaired ability to reflect on one's own state of mind and that of others (called metacognition or mentalization) is a central feature of personality disorders (PDs). Metacognition involves different specific abilities, which can be selectively impaired with different PDs and personality maladaptive traits. Moreover, research in the field of PDs has indicated that personality features and metacognitive abilities are associated with the severity of personality pathologies. In this study, we tested a mediation model of the interactions between these variables on predicting levels of personality functioning in a sample of adults taken from the general population (N = 775). Results showed that the relationship between personality traits and personality functioning is partially mediated by metacognitive abilities. These findings support the hypothesis that metacognition plays a significant role in predicting the levels of impairment in personality functioning. These findings have several clinical implications.