bluuebunny/crossref_metadata_2025_split huggingface dataset:
Converting an infant with borderline left heart structures to biventricular circulation after an initial hybrid Norwood approach: a case report
Authors: Cassanello Pia , Andreas Eicken , Maria Von Stumm , Stanimir Georgiev 
Published: March 2023

AbstractBackgroundHybrid approach in hypoplastic left heart syndrome (HLHS) implies placement of bilateral pulmonary artery bands and ductal stenting. This approach serves as a less invasive surgical–interventional alternative to the Norwood procedure with reported equivalent survival. Potential benefits include deferrable surgical risks and in some borderline left ventricle patients, when combined with a restrictive inter-atrial communication, it offers a chance for biventricular circulation.Case summaryTerm newborn with total anomalous pulmonary venous connection (TAPVC), hypoplastic left heart, small aortic arch, and atrial septal defect (ASD) who was multi-disciplinary deemed eligible for a hybrid palliation performed on day six of life after TAPVC surgical correction. Subsequently, percutaneous ductal stent implantation was performed (two Sinus Superflex-DS stents) on day 11 of life. Magnetic resonance imaging (MRI) showed a hypoplastic non-apex-forming left ventricle (LV) [left ventricular end-diastolic volume index (LVEDVI) 15 mL/m2]. On day 27th of life, the ASD was closed surgically with a fenestrated patch. At 2 months of age, control cardiac MRI showed striking LV growth (LVEDVI 37 mL/m2). Herein an interventional hybrid take-down was performed with dilatation of both pulmonary arterial bands and closure of the ductal stent with an Amplatzer Vascular plug II, thus achieving biventricular circulation.DiscussionHybrid approach has proven as a safe alternative to the Norwood procedure for HLHS. This case illustrates the importance of multi-disciplinary care in high-risk two ventricular patients by means of LV rehabilitation. Further studies are needed to systematically define and understand the intricacies of potential candidates for staged biventricular repair initially managed with hybrid approach.
Sustainable goal-oriented smart environments: a declarative programming approach
Authors: Bisicchia Giuseppe , Forti Stefano , Brogi Antonio 
Published: March 2023

Abstract
The sustainable management of smart environments enabled by the Internet of Things (IoT) requires new methodologies and tools to suitably handle potentially many users and their objectives on cyber-physical systems, e.g. smart lighting, smart A/C. In this article, we propose a declarative framework to model IoT-enabled smart environments. Our methodology permits (i) expressing user roles and hierarchical environments, (ii) declaring customized policies to mediate user objectives into a target state and (iii) determining valid settings for IoT actuators to achieve such a target also reducing energy consumption. An open-source Prolog prototype of the framework is showcased over two lifelike motivating examples and its scalability is assessed at increasing sizes of the managed smart environment.
Contributions of the Department of Defense Congressionally Directed Medical Research Programs to Advances in Cancer Therapeutics
Authors: Neagley Melanie , Helsel Alexis , Buachie Regina , Behrsing Tracy , Ghosh Sagar , Vaday Gayle , Cunningham Melissa D 
Published: March 2023

ABSTRACT
Nearly a billion dollars is spent annually in the Military Health System (MHS) on cancer diagnosis and treatment, with a large portion of that directed toward breast, prostate, and ovarian cancers. Multiple studies have demonstrated the impact of specific cancers on MHS beneficiaries and Veterans, highlighting the fact that active duty and retired military members have a higher incidence than the general public for many chronic diseases and certain forms of cancer. The Congressionally Directed Medical Research Programs have supported research that has contributed to the development, clinical testing, and commercialization of 11 cancer drugs approved by the Food and Drug Administration to treat breast, prostate, or ovarian cancers. In addition to hallmark funding mechanisms that prioritize innovative, groundbreaking ideas, the Congressionally Directed Medical Research Program’s cancer programs continue to identify new approaches to fill critical gaps across the full research spectrum, including bridging the translational research gap toward developing new treatments for cancer patients in the MHS and in the general American public.
Thermal emission from the hot Jupiter WASP-103 b in J and Ks bands
Authors: Shi Yaqing , Wang Wei , Zhao Gang , Zhai Meng , Chen Guo , Jiang Zewen , Ouyang Qinglin , Henning Thomas , Zhao Jingkun , Crouzet Nicolas , van Boekel Roy , Esteves Lisa 
Published: March 2023

ABSTRACT
Hot Jupiters, particularly those with temperature higher than 2000 K, are the best sample of planets that allow in-depth characterization of their atmospheres. We present here a thermal emission study of the ultrahot Jupiter WASP-103 b observed in two secondary eclipses with CFHT/WIRCam in J and Ks bands. By means of high precision differential photometry, we determine eclipse depths in J and Ks to an accuracy of 220 and 270 ppm, which are combined with the published HST/WFC3 and Spitzer data to retrieve a joint constraints on the properties of WASP-103 b dayside atmosphere. We find that the atmosphere is best fit with a thermal inversion layer included. The equilibrium chemistry retrieval indicates an enhanced C/O (1.35$^{+0.14}_{-0.17}$) and a supermetallicity with [Fe/H]$=2.19^{+0.51}_{-0.63}$ composition. Given the near-solar metallicity of WASP-103 of [Fe/H] = 0.06, this planet seems to be ∼100 more abundant than its host star. The free chemistry retrieval analysis yields a large abundance of FeH, H−, CO2, and CH4. Additional data of better accuracy from future observations of JWST should provide better constraint of the atmospheric properties of WASP-103b.
Chaotic winds from a dying world: a one-dimensional map for evolving atmospheres
Authors: Bromley Joshua , Chiang Eugene 
Published: March 2023

ABSTRACTPlanets that are smaller than Mercury and heated to sublimation temperatures of ∼2000 K lose mass catastrophically in dusty evaporative winds. The winds are observed to gust and recede largely without pattern; transit depths from the Kepler mission vary randomly from orbit to orbit by up to a factor of 10 or more. We explain how chaotic outflows may arise by constructing a map for the wind mass-loss rate as a function of time. The map is built on three statements: (1) The wind mass-loss rate scales in proportion to the surface equilibrium vapour pressure, rising exponentially with ground temperature. (2) Because the wind takes a finite time to escape the planet’s gravity well, the surface mass-loss rate at any time determines the wind optical depth at a later time—the atmosphere has hysteresis. (3) The ground temperature increases with optical depth (greenhouse effect) when the atmosphere is optically thin and decreases with optical depth when the atmosphere is optically thick (nuclear winter). Statement (3) follows from how dust condenses in the face of intense stellar irradiation. As discussed recently, condensates initially naked before the star must be silicate-rich and iron-poor, staying cool enough for condensation by absorbing weakly in the visible and emitting strongly in the infrared. Later, when grains are numerous enough to self-shield from starlight, they may accrete more iron and reverse their visible-to-infrared opacity ratio. Depending on parameters, the map for the wind can regularly boom and bust between a greenhouse and a nuclear winter, or erupt into chaos. Lyapunov times are measured in orbital periods, the time for the wind to turn by Coriolis forces away from the planet’s dayside, out of the Hill sphere.
Flap endonuclease Rad27 cleaves the RNA of R-loop structures to suppress telomere recombination
Authors: Liu Chia-Chun , Chan Hsin-Ru , Su Guan-Chin , Hsieh Yan-Zhu , Lei Kai-Hang , Kato Tomoka , Yu Tai-Yuan , Kao Yu-wen , Cheng Tzu-Hao , Chi Peter , Lin Jing-Jer 
Published: March 2023

Abstract
The long non-coding telomeric RNA transcript TERRA, in the form of an RNA–DNA duplex, regulates telomere recombination. In a screen for nucleases that affects telomere recombination, mutations in DNA2, EXO1, MRE11 and SAE2 cause severe delay in type II survivor formation, indicating that type II telomere recombination is mediated through a mechanism similar to repairing double-strand breaks. On the other hand, mutation in RAD27 results in early formation of type II recombination, suggesting that RAD27 acts as a negative regulator in telomere recombination. RAD27 encodes a flap endonuclease that plays a role in DNA metabolism, including replication, repair and recombination. We demonstrate that Rad27 suppresses the accumulation of the TERRA-associated R-loop and selectively cleaves TERRA of R-loop and double-flapped structures in vitro. Moreover, we show that Rad27 negatively regulates single-stranded C-rich telomeric DNA circles (C-circles) in telomerase-deficient cells, revealing a close correlation between R-loop and C-circles during telomere recombination. These results demonstrate that Rad27 participates in telomere recombination by cleaving TERRA in the context of an R-loop or flapped RNA–DNA duplex, providing mechanistic insight into how Rad27 maintains chromosome stability by restricting the accumulation of the R-loop structure within the genome.
Role of Terlipressin in Patients With Hepatorenal Syndrome-Acute Kidney Injury Admitted to the ICU: A Substudy of the CONFIRM Trial
Authors: Karvellas Constantine J. , Subramanian Ram , Olson Jody C. , Jamil Khurram 
Published: March 2023

IMPORTANCE AND OBJECTIVES:
This study assessed the potential advantages of treating hepatorenal syndrome-acute kidney injury (HRS-AKI) with terlipressin versus placebo in the ICU setting.

DESIGN:
Patients were randomly assigned in a 2:1 ratio to receive terlipressin or placebo for up to 14 days.

SETTING:
A retrospective analysis of data from the phase III CONFIRM study.

PARTICIPANTS:
Adult patients with HRS-AKI admitted to the ICU.

MAIN OUTCOMES AND MEASURES:
In this substudy, we evaluated outcomes of the ICU stay and the need for organ support, including renal replacement therapy (RRT).

RESULTS:
Among 300 patients with HRS-AKI from the CONFIRM study, 45 were treated in the ICU (terlipressin, 31/199 [16%]; placebo, 14/101 [14%]). On ICU admission, baseline demographics were similar across treatment arms, including severity of liver dysfunction. Among patients alive at the end of the ICU stay, those randomized to terlipressin had a significantly shorter median length of ICU stay than placebo (4 vs 11 d; p < 0.001). Terlipressin-treated patients had a significantly larger improvement in renal function from baseline versus placebo (–0.7 vs +0.2 mg/dL; p = 0.001), including when accounting for the interaction between treatment and day-of-patient-admission to the ICU (–0.7 vs +0.9 mg/dL; p < 0.001). Cumulative requirement for RRT through day 90 was improved in the terlipressin arm versus placebo (10/31 [32%] vs 8/14 [57%]; p = 0.12), although not significantly. Of 13 patients who received a liver transplant, five out of five (100%) in the placebo arm needed RRT through day 90 versus five out of eight (63%) in the terlipressin arm.

CONCLUSIONS:
In this subanalysis of CONFIRM, patients admitted to the ICU with HRS-AKI who received terlipressin were more likely to achieve renal function improvement, based on serum creatinine changes by the end of treatment, and had significantly shorter lengths of ICU stay than patients randomized to the placebo arm.
Quality of Life and Family Impact in Down Syndrome, Autism Spectrum Disorder, and Co-occurring Down Syndrome and Autism Spectrum Disorder
Authors: Soccorso Cara , Milliken Anna , Hojlo Margaret , Pawlowski Katherine , Weas Sarah , Sideridis Georgios , Baumer Nicole 
Published: March 2023

Abstract:

Objective:
Families of children with neurodevelopmental disorders have developmental, behavioral, and social-emotional needs that affect quality of life (QoL). This study assesses the validity and utility of a caregiver QoL measure; characterizes QoL in families with children with Down syndrome (DS), autism spectrum disorder (ASD), and a dual diagnosis of DS and ASD (DS + ASD); and compares and explores differences in QoL based on diagnosis.

Methods:
Caregivers of children and adolescents with ASD (n = 610) and DS (n = 177) completed the Pediatric Quality of Life Inventory Family Impact Module 2.0, yielding overall, parent functioning, family functioning, and subscale scores, and a Parent Global Impression (PGI) rating. An ASD cohort (n = 177) was sex matched to the DS cohort (n = 177) to mitigate potential sex bias. Additional analyses compared these groups with children and adolescents with DS + ASD (n = 37).

Results:
Analyses showed that the Pediatric Quality of Life Inventory was valid and reliable in DS, ASD, and DS + ASD populations. No differences were reported in PGI ratings among groups. Caregivers in the DS group demonstrated higher QoL and family functioning compared with the ASD and DS + ASD groups. The DS group reported significantly better Emotional Functioning and Communication and less Worry than the ASD group. Compared with the ASD group, caregivers of the DS + ASD group indicated more concerns with Physical Functioning. Notably, the DS + ASD group had significantly lower levels of QoL than the DS group in nearly all caregiver functioning domains.

Conclusion:
This study highlights differences in QoL within and between neurodevelopmental disorder groups, which may help identify families requiring additional support, advocacy, and community engagement.
Combining Proximal Foam Sclerotherapy and Distal Endovenous Laser Ablation for Recurrent Varicose Vein Treatment
Authors: Bossart Simon , Kos Sebastian , Keo Hak Hong , Staub Daniel , Uthoff Heiko 
Published: March 2023

BACKGROUND
Recurrent varicose veins with neovascularization are a common clinical problem. Although endovenous laser ablation (EVLA) has become the standard treatment modality to treat truncal veins, additional sclerotherapy is required to treat newly formed tortuous veins. A novel laser fiber with an injection channel (nLF) allows for such simultaneous proximal sclerotherapy and offers a potentially more effective treatment option.

OBJECTIVE
The aim of this study was to present our experience using the nLF for combined EVLA and sclerotherapy treatment of symptomatic recurrent varicose veins.

MATERIALS AND METHODS
A retrospective single-center cohort study analyzed the outcomes of all patients with symptomatic recurrent varicose veins, treated with an nLF between September 2020 and August 2022.

RESULTS
In total, 28 combined EVLA/sclerotherapy procedures performed with the nLF in 26 patients were analyzed. In all cases, neovascularizations were treated by sclerotherapy through the nLF catheter, followed by laser ablation of the remaining truncal veins. All follow-up ultrasound examinations showed persistent complete occlusion of the treated truncal veins and neovascularizations. No clinically relevant complications were observed.

CONCLUSION
Combining proximal foam sclerotherapy and distal EVLA using an nLF for the treatment of symptomatic recurrent varicose veins is safe and effective.
How the Science of Radiation Biology Can Help Reduce the Crippling Fear of Low-level Radiation
Authors: Brooks Antone L. , Conca James , Glines Wayne M. , Waltar Alan E. 
Published: March 2023

Abstract
The fear of radiation has been present almost since the discovery of radiation, but has intensified since the “dawn of the atomic age” over 75 y ago. This fear has often served as an impediment to the safe and beneficial uses of radiation and radioactive material. The underlying causes of such fear are varied, can be complex, and are often not associated with any scientific knowledge or understanding. The authors believe that a clear understanding of the current scientific knowledge and understanding of the effects of radiation exposure may be useful in helping to allay some of the fear of radiation. This manuscript attempts to (1) address several scientific questions that we believe have contributed to the fear of radiation, (2) review the data derived from research that can be used to address these questions, and (3) summarize how the results of such scientific research can be used to help address the fear of low-dose and low-dose-rate radiation. Several examples of how fear of radiation has affected public perception of radiological events are discussed, as well as a brief history of the etiology of radiation fear. Actions needed to reduce the public fear of radiation and help fulfill the full societal benefits of radiation and radioactive materials are suggested.
EXACERBATED TOURETTE SYNDROME DIAGNOSED IN ADULTHOOD AFTER RECURRENT BILATERAL RETINAL DETACHMENT
Authors: Mito Tsuyoshi , Okemoto Hajime , Kawasaki Akane , Sasaki Hiroshi 
Published: March 2023

Purpose:
The purpose of this study was to report a case of Tourette syndrome, exacerbated in adulthood, that was diagnosed after bilateral lens luxation and recurrent retinal detachment due to self-injury.

Methods:
This was a case report.

Results:
A 35-year-old man presented with a sudden abnormality in vision and bilateral lens luxation. The patient successfully underwent bilateral lens extraction and intrascleral intraocular lens fixation; however, vitreous hemorrhage and retinal detachment occurred in the left eye. The retinal detachment was caused by a giant retinal tear and retinal dialysis. Vitrectomy was performed. However, retinal detachment recurred with proliferative vitreoretinopathy. Retinal detachment subsequently occurred in the right eye. Self-injury to the eye was observed before surgical treatment. The patient was consequently diagnosed with Tourette syndrome.

Conclusion:
Tourette syndrome, which may be accompanied by self-injurious behavior, is a disorder that typically develops in childhood but rarely exacerbates during adulthood. A diagnosis of Tourette syndrome should be considered in cases of unexplained retinal detachment with traumatic features.
Refractory severe idiopathic thrombocytopenia and treatment challenges
Authors: Shahnazi Anahita , Johnston Brandon , Hargrove Mark , Patolia Harsukh , Junko Gretchen 
Published: March 2023

Introduction:
Refractory idiopathic thrombocytopenia (ITP) is a disease that does not respond to or relapses after splenectomy, requires treatment to reduce the risk of clinically significant bleeding, and is a challenging case to treat.

Presentation of the Case:
A 39-year-old male with a history of chronic ITP presented with a platelet count of 1000/µl and prostatitis. He was started on Ciprofloxacin and started intravenous immunoglobulin along with intravenous methylprednisolone. Then Rituximab was started on day fourth. Since his platelet remained 0/µl, Mycophenolate mofetil (Cellcept) was started on day 14th. Next, a dose of Romiplostim on day 19th was given. Eltrombopag (Promacta) and Tavlesse were started on day 23th and platelets rose to 96×103/µl on day 26th and then 418×103/µl.

Discussion:
Normally, refractory ITP patients who do not respond to first-line treatments require a combination therapy of one to two medicines of the second line, like thrombopoietin receptor agonists. However, this patient’s thrombocytopenia neither responded to first-line treatment nor second-line treatment with Promacta/Romiplostin plus immunosuppressives or Tavlesse.

Conclusion:
Refractory ITP, who has not responded to first-line and second-line treatments, requires treatment with a combination of all first-line and second-line treatments. Furthermore, Promacta, Tavlesse, and Romiplostim have a big role to play in helping the patient.
“Diagnostic Accuracy of Two Manual Muscle Testing Scales for Identifying Upper Extremity Nerve Transfer Candidates in Children with Acute Flaccid Myelitis”
Authors: Werner Julie M. , Wlodarczyk Jordan , Seruya Mitchel 
Published: March 2023

Background:
Manual muscle testing is a mainstay of strength assessment despite not having been compared to intraoperative electrical stimulation of peripheral nerves.

Methods:
Intraoperative electrical stimulation served as the reference standard in evaluating predictive accuracy of the Active Movement Scale (AMS) and the Medical Research Council (MRC) scale. Retrospective consecutive sampling of all patients with AFM who underwent exploration or nerve transfer at a pediatric multidisciplinary brachial plexus and peripheral nerve center March 2016 - July 2020 were included. The nonparametric area under the curve (AUC) was calculated. Optimal cutoff score (Youden’s J) and diagnostic accuracy values were reported. AMS and MRC were directly compared for predictive superiority.

Results:
181 upper extremity nerves (73 donor nerve candidates, 108 recipient nerve candidates) were tested intraoperatively from 40 children (mean age 7.9 years, SD: 4.9). The scales performed similarly (p=.953) in classifying suitable donor nerves with satisfactory accuracy (AUCAMS: 71.5%, AUCMRC: 70.7%; optimal cutoff: AMS >5, MRC >2). The scales performed similarly (p=.688) in classifying suitable recipient nerves with good accuracy (AUCAMS: 92.1%, AUCMRC: 94.9%; optimal cutoff: AMS ≤3, MRC ≤1).

Conclusions:
Manual muscle testing is an accurate, non-invasive means of identifying donor and recipient nerves for transfer in children with acute flaccid myelitis. The utility of these results is in minimizing unexpected findings in the operating room and aiding in the development of contingency plans. Further research may extend these findings to test the validity manual muscle testing as outcome measures of the success of nerve transfer.
Assessing how Age, Sex, Race, and Education Affect the Relationships Between Cognitive Domains and Odor Identification
Authors: Zhong Selena , Wroblewski Kristen E. , Laumann Edward O. , McClintock Martha K. , Pinto Jayant M. 
Published: March 2023

Background:
The associations between cognitive domains and odor identification are well established, but how sociodemographic variables affect these relationships is less clear.

Purpose:
Using the survey-adapted Montreal Cognitive Assessment instrument (MoCA-SA), we assess how age, sex, race, and education shape these relationships.

Methods:
We first used cluster analysis and multidimensional scaling to empirically derive distinct cognitive domains from the MoCA-SA as it is unclear whether the MoCA-SA can be disaggregated into cognitive domains. We then used ordinal logistic regression to test whether these empirically derived cognitive domains were associated with odor identification and how sociodemographic variables modified these relationships.

Study Population:
Nationally representative sample of community-dwelling US older adults.

Results:
We identified 5 out of the 6 theoretical cognitive domains, with the language domain unable to be identified. Odor identification was associated with episodic memory, visuospatial ability, and executive function. Stratified analyses by sociodemographic variables reveal that the associations between some of the cognitive domains and odor identification varied by age, sex, or race, but not by education.

Conclusions:
These results suggest that (1) the MoCA-SA can be used to identify cognitive domains in survey research and (2) the performance of smell tests as a screener for cognitive decline may potentially be weaker in certain subpopulations.