bluuebunny/crossref_metadata_2025_split huggingface dataset:
Over expression of CDK4 and MDM2 in a patient with recurrent ALK-negative mediastinal inflammatory myofibroblastic tumor
Authors: Hou Tien-Chi , Wu Pao-Shu , Huang Wen-Yu , Yang Yi-Ting , Tan Kien Thiam , Liu Shih-Hua , Chen Yu-Jen , Chen Shu-Jen , Su Ying-Wen 
Published: March 2020

Abstract

Rationale:
The diagnosis of anaplastic lymphoma kinase (ALK)-negative inflammatory myofibroblastic tumors (IMT) remains challenging because of their morphological resemblance with spindle cell sarcoma with myofibroblastic characteristics.

Patient concerns:
A 69-year-old female patient presented with loco-regional recurrent IMT several times within 8 years after primary treatment and neck lymph node metastasis 3.5 years after last recurrence.

Diagnosis:
The primary, recurrence, and lymph node metastasis lesions were diagnosed as ALK-negative IMTs based on the histopathological features.

Interventions:
Biopsy samples were obtained during repeated surgeries and evaluated for genomic alterations during first and recurrent presentations. The evaluation was done using pathway-driven massive parallel sequencing, and genomic alterations between primary and recurrent tumors were compared.

Outcomes:
Copy number gains and overexpression of mouse double minute 2 homolog (MDM2) and cyclin dependent kinase 4 (CDK4) were observed in the primary lesion, and additional gene amplification of Discoidin Domain Receptor Tyrosine Kinase 2 (DDR2), Succinate Dehydrogenase Complex II subunit C (SDHC), and thyroid stimulating hormone receptor (TSHR) Q720H were found in the recurrent tumors. Metastases to the neck lymph node were observed 3.5 years after recurrence.

Lessons:
Our results indicated genetic evolution in a microscopically benign condition and highlighted the importance of molecular characterization of fibro-inflammatory lesions of uncertain malignant potential.
Mid-lumbar traumatic spondyloptosis without neurological deficit
Authors: Xu Feng , Tian Zhisen , Fu Changfeng , Yao Liyu , Yan Mengjie , Zou Congcong , Liu Yi , Wang Yuanyi 
Published: March 2020

Abstract

Introduction:
Spondyloptosis is a form of vertebral dislocation and the most advanced form of spondylolisthesis. Traumatic spondyloptosis is usually caused by high-energy impact and results in unstable spine deformity and spinal canal deformation, which lead to severe spinal cord injury. Traumatic spondyloptosis is mostly reported in the lumbo-sacral junction, while it is rarely documented in mid-lumbar segments. To the best of the authors’ knowledge, only 16 cases of mid-lumbar spondyloptosis have been described previously. Herein, we present a L3 to L4 spondyloptosis case that did not involve neurological deficit.

Patient concerns:
A 42-year-old man presented to the emergency department after an accident involving a fall. The patient developed severe back pain and spinal deformity, while his neurologic function remained intact. Radiological examinations indicated complete posterior vertebral dislocation at L3 to L4 and a fracture at the bilateral pelvic ischial tuberosity without major vessel injury or severe dura sac compression.

Diagnoses:
L3 to L4 complete vertebral dislocation, pelvic ischial tuberosity fracture.

Interventions:
For treatment, the patient underwent fracture reduction, L3 to L4 intervertebral fusion, and internal fixation 7 days post-injury.

Outcomes:
Postoperative digital radiography showed the correction of the spinal deformity. The patient was pain-free and fully rehabilitated 3 months after the surgery. At the 1-year follow-up, the patient was completely asymptomatic and had achieved normal alignment.

Conclusions:
We reported an L3 to L4 traumatic spondyloptosis case that involved intact neurology, which is the first-ever reported mid-lumbar spondyloptosis case that involved complete posterior column and neural sparing. For the treatment of traumatic spondyloptosis without neurological deficit, restoring stability and preventing secondary cord injury should be taken into consideration.
Impact of HCV cure with drug-acting antivirals in the use of concomitant medication and lipid profile: follow-up data 2 years after the sustained virological response
Authors: González-Colominas Elena , Batlle Marc , Monge-Escartín Inés , Duran Xavier , Viu Anna , de Antonio-Cuscó Marta , Grau Santiago , Bessa Xavier , Carrión Jose Antonio 
Published: March 2020

Background and aim:
Patients with chronic hepatitis C (CHC) frequently associated comorbidities and concomitant medication. Sustained virological response (SVR12) has been related to an increase in cholesterol serum levels and in peripheral vascular resistance. Our aim was to evaluate the impact of SVR12 on the use of concomitant medication and serum lipid profile.

Methods:
Prospective study including patients treated with direct-acting antivirals who had achieved the SVR12. Clinical data and concomitant drugs were analysed at baseline and at least 1 year after SVR12. Differences from baseline to follow-up in the concomitant medication were evaluated by Stuart–Maxwell test and lipid profile by Wilcoxon signed-rank test. Patients were categorized according to the increase/decrease in the number of drugs included in each class (Anatomical Therapeutic Chemical classification system).

Results:
Two hundred twenty-six patients with SVR12 were included, 73.5% were receiving concomitant drugs (49.6% with antihypertensive effect, 30.5% antacids, 16.4% anti-diabetic drugs, and 7.1% lipid-lowering agents). One year after SVR12, total cholesterol serum levels increased from 161 to 179 mg/dl (P < 0.001) and, after a median time of 25.7 months, the use of lipid-lowering drugs increased from 7.8 to 11.5% (P = 0.009). In addition, we observed a trend to use more antihypertensive drugs in older patients (P = 0.06), especially in those with cirrhosis. Anxiolytics decreased after SVR12 from 13.7 to 10.6% (P = 0.035).

Conclusion:
CHC cure is associated with a significant increase in cholesterol serum levels and the use of lipid-lowering agents, as well as the use of drugs with antihypertensive effect in older patients.
Cell-Cycle Arrest Biomarkers: Usefulness for Cardiac Surgery-Related Acute Kidney Injury in Neonates and Infants*
Authors: Bojan Mirela , Pieroni Laurence , Semeraro Michaela , Froissart Marc 
Published: March 2020

Objectives:
Cell cycle arrest urine biomarkers have recently been shown to be early indicators of acute kidney injury in various clinical settings in critically ill adults and children. The product of tissue inhibitor metalloproteinase -1 and insulin-like growth factor binding protein-7 concentrations/1,000 (TIMP-1) × (IGFBP-7) provides stratification of acute kidney injury-risk in adults with critical illness. The present study explores the predictive accuracy of (TIMP-1) × (IGFBP-7) measured early after cardiopulmonary bypass for cardiac surgery-related acute kidney injury in neonates and infants, a population in whom such data are not yet available.

Design:
Prospective, observational.

Setting:
A tertiary referral pediatric cardiac ICU.

Patients:
Fifty-seven neonates and 110 infants undergoing surgery with cardiopulmonary bypass.

Interventions:
None.

Measurements and Main Results:
(TIMP-1) × (IGFBP-7) was measured on the NephroCheck (Astute Medical, San Diego, CA) platform preoperatively, less than 1 hour of cardiopulmonary bypass and 1–3 hours of cardiopulmonary bypass. The incidence of postoperative acute kidney injury, dialysis, and/or death were compared among quintiles of postoperative (TIMP-1) × (IGFBP-7). Multivariable regression was used to assess the added predictive value for renal events of (TIMP-1) × (IGFBP-7) over clinical models. Basal (TIMP-1) × (IGFBP-7) increased with age at surgery (regression coefficient = 0.004 ± 0.001; p = 0.005). (TIMP-1) × (IGFBP-7) increased after cardiopulmonary bypass. Neonates had lower postoperative (TIMP-1) × (IGFBP-7) compared with older infants, despite undergoing longer surgeries and experiencing a higher incidence of postoperative renal events. (TIMP-1) × (IGFBP-7) was not associated with acute kidney injury, dialysis, and/or death and was not a predictor of the aforementioned events when added to a clinical acute kidney injury model including age, duration of cardiopulmonary bypass, and mechanical ventilation prior to surgery.

Conclusions:
These findings question the usefulness of (TIMP-1) × (IGFBP-7) for the prediction of cardiac surgery-related acute kidney injury in neonates and infants when measured within 3 hours of cardiopulmonary bypass.
Dose-Response Study on Surfactant Nebulization Therapy During Nasal Continuous Positive Airway Pressure Ventilation in Spontaneously Breathing Surfactant-Deficient Newborn Piglets*
Authors: Rey-Santano Carmen , Mielgo Victoria , Gomez-Solaetxe Miguel Angel , Ricci Francesca , Bianco Federico , Salomone Fabrizio , Loureiro Begoña 
Published: March 2020

Objectives:
The current clinical treatment of neonates with respiratory distress syndrome includes endotracheal intubation and intratracheal instillation of exogenous surfactant. Nebulization of surfactant offers an attractive alternative. The aims of this study were to test nebulization as a noninvasive method of administering surfactant and determine the optimal dose for the treatment of respiratory distress syndrome–associated pathophysiology of the neonatal lungs.

Design:
Prospective, randomized, animal model study.

Setting:
An experimental laboratory.

Subjects:
Thirty-six newborn piglets.

Interventions:
Different doses (100, 200, 400, and 600 mg/kg) of poractant alfa were administered via a vibrating membrane nebulizer (eFlow-Neos; Pari Pharma GmbH, Starnberg, Germany) or a bolus administration using the intubation–surfactant–extubation (Insure) technique (200 mg/kg) to spontaneously breathing newborn piglets (n = 6/group) with bronchoalveolar lavage–induced respiratory distress syndrome during nasal continuous positive airway pressure (180 min).

Measurements and Main Results:
Pulmonary, hemodynamic, and cerebral effects were assessed. Histologic analysis of lung and brain tissue was also performed. After repeated bronchoalveolar lavage, newborn piglets developed severe respiratory distress syndrome. Rapid improvement in pulmonary status was observed in the Insure group, whereas a dose-response effect was observed in nebulized surfactant groups. Nebulized poractant alfa was more effective at doses higher than 100 mg/kg and was associated with similar pulmonary, hemodynamic, and cerebral behavior to that in the Insure group, but improved lung injury scores.

Conclusions:
In newborn piglets with severe bronchoalveolar lavage–induced respiratory distress syndrome, our results demonstrate that the administration of nebulized poractant alfa using an investigational customized eFlow-Neos nebulizer is an effective and safe noninvasive surfactant administration technique.
Decreased Brain Volumes and Infants With Congenital Heart Disease Undergoing Venoarterial Extracorporeal Membrane Oxygenation
Authors: Chao Brandon K. , Claessens Nathalie H. P. , Lim Jessie Mei , Gorodetsky Carolina , Au-Young Stephanie H. , Guerguerian Anne-Marie , Marini Davide , Blaser Susan , Shroff Manohar , Saini Amandeep K. , Seed Mike , Chau Vann , Miller Steven P. 
Published: March 2020

Objectives:
The aims of this study were to: i) determine the spectrum of brain injury and ii) compare brain volumes between pre- and postoperative brain MRI in the infants receiving extracorporeal membrane oxygenation compared with those who did not require extracorporeal membrane oxygenation.

Design:
Cohort study of infants with d-transposition of the great arteries or single ventricle physiology. Brain volume (cm3) was measured using a segmentation of a volumetric T1-weighted gradient echo sequence. Brain imaging findings (intraventricular hemorrhage, white matter injuries, and stroke) were analyzed with respect to known clinical risk factors for brain injury and adverse neurodevelopmental outcomes. Clinical factors were collected by retrospective chart review. The association between brain volume and extracorporeal membrane oxygenation was evaluated using generalized estimating equations to account for repeated measures.

Setting:
Prospective and single-centered study.

Patients:
One hundred nine infants (median gestational age, 39.1 wk) with d-transposition of the great arteries (n = 77) or single ventricle physiology (n = 32) were studied pre- and postoperatively with MRI as per clinical protocol.

Interventions:
None.

Measurements and Main Results:
Of the 28 infants (26%) receiving extracorporeal membrane oxygenation, 19 (68%) were supported with extracorporeal membrane oxygenation once, and nine (32%) were supported 2–4 times. On postoperative MRI, new white matter injury was found in only five (17%) of the extracorporeal membrane oxygenation infants versus 40 (49%) in the non–extracorporeal membrane oxygenation group (p = 0.073). The rate of stroke (9% vs 10%), intraventricular hemorrhage (24% vs 29%), and hypoxic ischemia (3% vs 14%) did not differ between the non–extracorporeal membrane oxygenation and extracorporeal membrane oxygenation groups (all p > 0.5). Accounting for d-transposition of the great arteries or single ventricle physiology diagnosis, infants requiring extracorporeal membrane oxygenation had slower brain volume with single (β = –1.67) or multiple extracorporeal membrane oxygenation runs ([β = –6.54]; overall interaction p = 0.012).

Conclusions:
Patients with d-transposition of the great arteries or single ventricle physiology undergoing extracorporeal membrane oxygenation at our center have a similar incidence of brain injury but more significant impairment of perioperative brain volumes than those not requiring extracorporeal membrane oxygenation.
Isolation in Parents and Providers of Children With Chronic Critical Illness*
Authors: Wright-Sexton Laura A. , Compretta Caroline E. , Blackshear Chad , Henderson Carrie M. 
Published: March 2020

Objectives:
An increasing number of children with medical complexity spend months or more in PICUs, lending to isolation for their parents and providers. We sought to better describe the experiences of parents and providers of children with chronic critical illness specifically around isolation during PICU admission.

Design:
In-person interviews and surveys of pediatric critical care providers and parents of children with chronic critical illness. Interview transcripts were analyzed for themes.

Setting:
Academic institution; PICU.

Subjects:
Seven PICU physicians, eight nurse practitioners, and 12 parents of children with chronic critical illness.

Interventions:
Surveys and semi-structured interviews.

Measurements and Main Results:
PICU providers acknowledge feeling medically isolated from children with chronic critical illness, fueled by a lack of chronic critical illness training and burnout. Providers also perceive medical isolation in parents of children with chronic critical illness manifesting as a declining level of parental engagement. Parents did not feel medically isolated in our study. Providers also perceive social isolation in families of children with chronic critical illness, identifying the child’s protracted disease and lack of tangible support systems as contributing factors. Parents self-reported adequate social supports but scored high on depression scales suggesting a disconnect between perceived and actual support. Both parents and providers acknowledge that the child’s chronic critical illness could be a source of support.

Conclusions:
PICU providers perceived social and medical isolation in parents of children with chronic critical illness; however, parents did not endorse either directly. A majority of parents showed signs of depression despite reporting good social support. Providers reported feeling medically isolated from children with chronic critical illness and their families related to burnout and insufficient training. Novel methods to address these issues are needed.
A Neurological Appearance of Celiac Disease
Authors: Cavusoglu Dilek , Olgac Dundar Nihal , Oztekin Ozgur , Arican Pinar , Gencpinar Pinar , Baran Masallah 
Published: March 2020

Objective
Celiac disease may present with one or more neurological signs and/or symptoms. We aimed to define the incidence of accompanying neurological manifestations in children diagnosed as having celiac disease.

Methods
The prospective study included 146 children diagnosed as having celiac disease. The medical records (presentation symptoms, clinical findings, serological test, duodenal biopsy results, lack/deficiency of vitamin, tissue type, accompanying autoimmune disorders) and demographic data of all patients were also reviewed.

Results
Thirty-five (23.9%) of the 146 celiac patients exhibited one or more neurological findings. Headache (11.6%) and dizziness (6.1%) were the most common symptoms among neurological manifestations. There was a significant difference between the patients with and without neurological manifestations in terms of sex, biopsy result, and tissue type (P < 0.05). Moreover, there was a statistically significant difference between tissue types of the patients with and without headache (P < 0.05). We found that grade 3a by Marsh classification was the most common type among the patients with and without neurological findings in celiac disease. On neuroimaging evaluation of patients, 1 patient with chronic focal ischemic lesion, 1 patient with Chiari type 1 malformation, and 1 patient with subcortical white matter changes were identified.

Conclusions
Pathophysiology of neurological involvement in celiac disease is liable for various neurological findings. This study contributes to data suggesting that female sex, mild histopathological form, and human leukocyte antigen DQ2 heterozygosity are related to neurological manifestations, and also human leukocyte antigen DQ2 heterozygosity is associated with headache in celiac disease.
Emergent Mental Health Visits to a Pediatric Hospital
Authors: Uspal Neil G. , Jensen Jennifer , Sanchez-Erebia Luis , Strelitz Bonnie , Schloredt Kelly , Gallagher Chelsie , Bradford Miranda C. , Bennett Elizabeth , Paris Carolyn A. 
Published: March 2020

Objective
The aims of this study were to describe firearm storage practices in homes of patients evaluated for mental health (MH) complaints at a tertiary care children's hospital and to describe storage practice changes after treatment.

Methods
We surveyed families of children with MH complaints presenting to the emergency department or psychiatry unit who stored firearms in their homes between February 12, 2016, and January 14, 2017. Patients and families received standard care, including routine counseling on limiting access to methods of suicide. Participants completed surveys at baseline, 7, and 30 days after discharge. The primary outcome was triple safe firearm storage—storage of firearms unloaded, locked, and with ammunition stored and locked separately.

Results
Ninety-one household members of MH patients who stated they had firearms were enrolled at baseline. Seventy-seven (85%) completed at least 1 follow-up survey, and 63 (69%) completed both. At baseline, 21% (19/91) of participants reported engaging in triple safe firearm storage, 26% had an unlocked firearm, 23% had a loaded firearm, and 65% stored ammunition either unlocked or with their firearm. Triple safe storage rates increased to 31% at both 7 days and 30 days. Ten (17%) of 59 (P < 0.01) participants who did not report triple safe storage at baseline and completed a follow-up survey changed to reporting triple safe storage on follow-up.

Conclusions
The majority of firearm-storing family members of children with MH complaints do not follow triple safe storage practices. Storage practices modestly improved after an emergent MH visit, but over two thirds of participants reported unsecured or partially secured firearms 7 and 30 days later.
Decadron, Diamox, and Zantac
Authors: Lang Shih-Shan , Ploof Jillian , Atkin Natalie J. , Tran-Du Kella , Kanuga Bhavya M. , Storm Phillip B. , Heuer Gregory , Yuan Ian , Abend Nicholas S. , Kirschen Matthew P. , Topjian Alexis A. , Li Yimei , Waanders Angela J. , Zorc Joseph J. , Huh Jimmy W. 
Published: March 2020

Objective
Cerebral ventricular shunt failure is common and presents with symptoms that range from headaches to death. The combination of Diamox (acetazolamide), Decadron (dexamethasone), and Zantac (ranitidine) (DDZ) is used at our institution to medically stabilize pediatric patients presenting with symptomatic shunt failure before shunt revision. We describe our experience of this drug combination as a temporizing measure to decrease symptoms associated with shunt failure.

Methods
We performed a single-center retrospective chart review of patients younger than 18 years with ventricular shunt failure who underwent a shunt revision between January 2015 to October 2017 and received DDZ before surgery. The outcome variables evaluated included pre-DDZ and post-DDZ clinical symptoms, pain scores, and vital signs.

Results
There were 112 cases that received DDZ before shunt revision. The 4 most commonly reported symptoms were analyzed. Headache was observed in 42 cases pre-DDZ, and post-DDZ there was a 71% reduction in headache (P < 0.0001); emesis was reported pre-DDZ in 76 cases, and post-DDZ there was an 83% reduction (P < 0.0001); irritability was noted pre-DDZ in 30 cases, and post-DDZ there was a 77% reduction (P = 0.0003); lethargy pre-DDZ was observed in 60 cases, and post-DDZ 73% demonstrated improvement (P < 0.0001). Maximum pain scores significantly decreased post-DDZ (P < 0.0001). Heart rate, systolic, and diastolic blood pressures significantly decreased post-DDZ (P < 0.0001, P < 0.0001, P = 0.0002, respectively).

Conclusions
The combination of Decadron, Diamox, and Zantac is a novel treatment for ventricular shunt failure that may temporarily improve symptoms in patients awaiting shunt revision. Future studies could compare efficacy with other medical treatments.
Pediatric Difficult Airway Response Team Utilization in the Emergency Department
Authors: Kelly Geoffrey S. , Deanehan Julia Kathleen , Dalesio Nicholas M. 
Published: March 2020

Objectives
A multidisciplinary pediatric difficult airway team was created at our institution to respond to hospital-wide airway emergencies. We report the characteristics, indications, and outcomes of these activations that occur in the pediatric emergency department (PED).

Methods
Retrospective, single-center cohort study comprised all difficult airway team activations occurring in the PED from the program's inception in 2008 to 2018. Ages of ≤18 years were included. For each case, detailed information was abstracted, including patient factors, PED context and milieu, airway interventions, and airway outcomes.

Results
There were 15 difficult airway response team activations in the PED during the study period, or 1.4 activations per year. The most common indications for activation were contaminated airways (n = 7; 47%) and history of difficult intubation (n = 4; 27%). Definitive airway management was successful in all cases, except for a single case where intervention was unnecessary. The most commonly performed definitive airway intervention was direct laryngoscopy (n = 6; 40%). There were no instances of emergency front-of-neck access.

Conclusions
Difficult airways in the PED were uncommon. Most cases were resolved with familiar equipment including direct laryngoscopy, video laryngoscopy, and supraglottic airways.
Parotitis on Ultrasound
Authors: Ng Carrie , Tupchong Keegan , Thomas-Mohtat Rosemary 
Published: March 2020

Abstract
The differential diagnosis for facial swelling is broad and can be a diagnostic challenge in the pediatric emergency department. We describe the first pediatric case of acute parotitis with sialolithiasis where the diagnosis was facilitated by point-of-care ultrasound.