bluuebunny/crossref_metadata_2025_split huggingface dataset:
Endogenous Glucose Production in Patients With Glycogen Storage Disease Type Ia Estimated by Oral D-[6,6-2H2]-glucose
Authors: Rossi Alessandro , Oosterveer Maaike H , van Dijk Theo H , Bleeker Aycha , Koehorst Martijn , Weinstein David A , Bakker Barbara M , Derks Terry G J 
Published: September 2023

Abstract

Context
Glycogen storage disease type Ia (GSDIa) is an inborn metabolic disorder characterized by impaired endogenous glucose production (EGP). Monitoring of patients with GSDIa is prioritized because of ongoing treatment developments. Stable isotope tracers may enable reliable EGP monitoring.

Objective
The aim of this study was to prospectively assess the rate of appearance of endogenous glucose into the bloodstream (Ra) in patients with GSDIa after a single oral D-[6,6-2H2]-glucose dose.

Methods
Ten adult patients with GSDIa and 10 age-, sex-, and body mass index–matched healthy volunteers (HVs) were enrolled. For each participant, 3 oral glucose tracer tests were performed: (1) preprandial/fasted, (2) postprandial, and (3) randomly fed states. Dried blood spots were collected before D-[6,6-2H2]-glucose administration and 10, 20, 30, 40, 50, 60, 75, 90, and 120 minutes thereafter.

Results
Glucose Ra in fasted HVs was consistent with previously reported data. The time-averaged glucose Ra was significantly higher in (1) preprandial/fasted patients with GSDIa than HV and (2) postprandial HV compared with fasted HV(P &lt; .05). A progressive decrease in glucose Ra was observed in preprandial/fasted patients with GSDIa; the change in glucose Ra time-course was directly correlated with the change in capillary glucose (P &lt; .05).

Conclusion
This is the first study to quantify glucose Ra in patients with GSDIa using oral D-[6,6-2H2] glucose. The test can reliably estimate EGP under conditions in which fasting tolerance is unaffected but does not discriminate between relative contributions of EGP (eg, liver, kidney) and exogenous sources (eg, dietary cornstarch). Future application is warranted for longitudinal monitoring after novel genome based treatments in patients with GSDIa in whom nocturnal dietary management can be discontinued.
Model-Based Analysis of IGF-I Response, Dosing, and Monitoring for Once-Weekly Somapacitan in Children With GH Deficiency
Authors: Kildemoes Rasmus J , Backeljauw Philippe F , Højby Michael , Blair Joanne C , Miller Bradley S , Mori Jun , Lyauk Yassine K 
Published: September 2023

Abstract

Context
Growth hormone (GH) replacement therapy improves longitudinal growth and adult height in children with GH deficiency (GHD). GH stimulates insulin-like growth factor (IGF)-I release, the biomarker used for monitoring GH activity during treatment.

Objective
This study aims to provide model-based insights into the dose–IGF-I responses of once-weekly somapacitan, a novel long-acting GH, compared with daily GH in children with GHD.

Methods
Analyses included dosing information and 1473 pharmacokinetic samples from 210 somapacitan-treated pediatric patients with GHD across 3 trials, including phase 1 (NCT01973244), phase 2 (NCT02616562; REAL 3), and phase 3 (NCT03811535; REAL 4), as well as 1381 IGF-I samples from 186 patients with GHD treated with somapacitan in REAL 3 and REAL 4. Pharmacokinetic/pharmacodynamic modeling to characterize somapacitan dose–IGF-I response and predict the response to dosing day changes.

Results
Relationships were established between somapacitan dose, exposure, change from baseline IGF-I SD score (SDS), and height velocity (HV). A linear model permitted the development of a tool to calculate estimated average weekly IGF-I exposure from a single IGF-I sample obtained at any time within the somapacitan dosing interval at steady state. In practice, the use of this tool requires knowledge of somapacitan injection timing relative to IGF-I sample collection timing. IGF-I SDS simulations support flexible dosing day changes while maintaining at least 4 days between doses.

Conclusion
We characterized the dose–IGF-I response of somapacitan in children with GHD. To support physicians in IGF-I monitoring, we present a practical guide about expected weekly average IGF-I concentrations in these patients and provide insights on dosing day flexibility.
aristaless-like homeobox-3 is wound induced and promotes a low-Wnt environment required for planarian head regeneration
Authors: Akheralie Zaleena , Scidmore Tanner J. , Pearson Bret J. 
Published: September 2023

ABSTRACT
The planarian Schmidtea mediterranea is a well-established model of adult regeneration, which is dependent on a large population of adult stem cells called neoblasts. Upon amputation, planarians undergo transcriptional wounding programs and coordinated stem cell proliferation to give rise to missing tissues. Interestingly, the Wnt signaling pathway is key to guiding what tissues are regenerated, yet less known are the transcriptional regulators that ensure proper activation and timing of signaling pathway components. Here, we have identified an aristaless-like homeobox transcription factor, alx-3, that is enriched in a population of putative neural-fated progenitor cells at homeostasis, and is also upregulated in stem cells and muscle cells at anterior-facing wounds upon amputation. Knockdown of alx-3 results in failure of head regeneration and patterning defects in amputated tail fragments. alx-3 is required for the expression of several early wound-induced genes, including the Wnt inhibitor notum, which is required to establish anterior polarity during regeneration. Together, these findings reveal a role for alx-3 as an early wound-response transcriptional regulator in both muscle cells and stem cells that is required for anterior regeneration by promoting a low-Wnt environment.
Tackling the lack of diversity in cancer research
Authors: Molina-Aguilar Christian , Robles-Espinoza C. Daniela 
Published: September 2023

ABSTRACT
Despite the clear benefit of studying biological samples from diverse genetic backgrounds and geographical locations, our current knowledge of disease is mostly derived from the study of European-descent individuals. In the cancer field, this is reflected in the poor representation of African and Amerindian/Latino samples in most large public data repositories. This lack of diversity is due to several reasons, but here we focus on (1) the lack of support for studies on non-European populations that are performed in low- and middle-income countries (LMICs), and (2) unequal partnerships between scientists in LMICs and those in high-income countries. We argue that expanding access to research funding, increasing the participation of underrepresented scientists in editorial boards and international conferences, facilitating the publication of studies conducted in these countries, and properly acknowledging LMIC researchers' contributions in publications and grant applications will promote equity for scientists working in LMICs. We envisage that this will translate to more impactful research in these countries, which will include more samples from diverse populations. For the cancer field, this will broaden our understanding of pathomechanisms and may help to improve the treatment of patients from all backgrounds.
Thermal acclimation in a non-migratory songbird occurs via changes to thermogenic capacity, but not conductance
Authors: Schweizer Rena M. , Romero Abimael , Tobalske Bret W. , Semenov Georgy A. , Carling Matt D. , Rice Amber M. , Taylor Scott A. , Cheviron Zachary A. 
Published: September 2023

ABSTRACT
Thermoregulatory performance can be modified through changes in various subordinate traits, but the rate and magnitude of change in these traits is poorly understood. We investigated flexibility in traits that affect thermal balance between black-capped chickadees (Poecile atricapillus) acclimated for 6 weeks to cold (−5°C) or control (23°C) environments (n=7 per treatment). We made repeated measurements of basal and summit metabolic rates via flow-through respirometry and of body composition using quantitative magnetic resonance of live birds. At the end of the acclimation period, we measured thermal conductance of the combined feathers and skins. Cold-acclimated birds had a higher summit metabolic rate, reflecting a greater capacity for endogenous heat generation, and an increased lean mass. However, birds did not alter their thermal conductance. These results suggest that chickadees respond to cold stress by increasing their capacity for heat production rather than increasing heat retention, an energetically expensive strategy.
Synthesis of a Two-dimensional Multi-functional Macromonomer and Oxygen Permselectivity of Its Supramolecular and Covalent Polymer Membranes
Authors: Cheng Kehan , Shinohara Ken-ichi , Higashimine Koichi , Teraguchi Masahiro , Kaneko Takashi , Aoki Toshiki 
Published: September 2023

Abstract
Although polymers having small micropores are promising for good gas permselective membranes, such polymers tend to have no membrane-forming ability. In this study, a polymer including micropores was synthesized from two-dimensional fully-conjugated macromonomer (2DM) with multi-acetylene and hydroxyl groups. In spite of the low molecular weight, its solvent-cast membrane (supra-poly(2DM)) showed good oxygen permselectivity. In addition, soluble high molecular weight network poly(2DM) membrane obtained from supra-poly(2DM) membrane showed higher oxygen permselectivity due to the increased number of micropores.
Evaluation of the efficacy and safety of percutaneous transforaminal endoscopic discectomy for multi-segmental lumbar disc herniation
Authors: Zhao Ya-fei , Tian Bin-wu , Ma Qiu-shuang , Zhang Meng 
Published: September 2023

Objective: To investigate the clinical efficacy and safety of percutaneous transforaminal endoscopic discectomy (PTED) in the treatment of multi-segmental lumbar disc herniation (msLDH).
Methods: From January 2021 to December 2021, 75 patients with msLDH admitted to Baoding No.1 Hospital of Traditional Chinese Medicine were selected and divided into PTED group (n=40) and posterior lumbar interbody fusion (PLIF) group (n=35) according to different surgical methods. The operative time, intraoperative blood loss, surgical complications, Oswestry disability index (ODI) and Japanese Orthopedic Association score (JOA) scores were compared between the two groups.
Results: In the PTED group, the average operation time was 57.45±12.01minutes, and the average intraoperative blood loss was 50.57±16.69ml. There were three patients with surgical complications, including one case of hematoma, one case of aggravation of neurological symptoms and one case of new onset of neurological symptoms. In the PLIF group, there were 12 cases undergoing single-segment operation, 15 cases undergoing double-segment operation and 8 cases undergoing three-segment operation, the average operation time was 137.26±34.64minutes, and the average intraoperative blood loss was 456.06±33.06ml, there were four cases of wound fat liquefaction or delayed healing, two cases of hematoma, and three cases of exacerbation of original neurological symptoms or new neurological symptoms. At one month, six months, and one year of postoperative, the ODI and JOA scores of the two groups were significantly improved compared with those preoperative, and the ODI scores of the PTED group were better than those of the PLIF group (t=3.131, 2.263, 3.768, all P<0.05).
Conclusion: The surgical effect of PTED in the treatment of LDH is similar to that of PLIF. However, PTED has the advantages of short operation time, less blood loss, fewer surgical complications, and high surgical safety. It is worthy of clinical promotion.
doi: https://doi.org/10.12669/pjms.39.6.3399
How to cite this: Zhao Y, Tian B, Ma Q, Zhang M. Evaluation of the efficacy and safety of percutaneous transforaminal endoscopic discectomy for multi-segmental lumbar disc herniation. Pak J Med Sci. 2023;39(6):---------. doi: https://doi.org/10.12669/pjms.39.6.3399
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Effects of periodontal initial therapy combined with orthodontic treatment on anterior tooth function and inflammatory factors in gingival crevicular fluid in patients with periodontal disease induced anterior tooth displacement
Authors: Liu Yan , Shi Xie , Lin Gengbing , Guo Nan 
Published: September 2023

Objective: To evaluate the effects of periodontal initial therapy combined with orthodontic treatment on anterior tooth function and inflammatory factors in gingival crevicular fluid in patients with periodontal disease induced anterior tooth displacement.
Methods: This was a clinical comparative study. A total of 140 patients with anterior teeth displacement caused by periodontal disease in Fujian University of Traditional Chinese Medicine from May 2020 to May 2022 were selected and randomly divided into two groups. Patients in the control group received periodontal initial therapy, and those in the study group were provided with orthodontic treatment on the basis of initial therapy. Further comparative analysis was performed focusing on the clinical effects of the two groups, the changes of probing depth, anterior overjet degree, oral function and inflammatory factors in gingival crevicular fluid before and after treatment.
Results: The efficacy of the study group was significantly higher than that of the control group(p=0.00). After treatment, the probing depth, the anterior overjet degree and the rate of bleeding on probing in the study group were significantly lower than those in the control group(P=0.00). Furthermore, the proportion of tooth mobility degrees I, II and III in the study group was significantly lower than that in the control group after treatment(P<0.05). The levels of post-treatment inflammatory factors in the study group were significantly lower than those in the control group(p=0.00).
Conclusion: Periodontal initial therapy combined with orthodontic treatment has a significant effect on anterior teeth displacement caused by periodontal disease, which deserves promotion clinically.
doi: https://doi.org/10.12669/pjms.39.6.7135
How to cite this: Liu Y, Shi X, Lin G, Guo N. Effects of periodontal initial therapy combined with orthodontic treatment on anterior tooth function and inflammatory factors in gingival crevicular fluid in patients with periodontal disease induced anterior tooth displacement. Pak J Med Sci. 2023;39(6):---------. doi: https://doi.org/10.12669/pjms.39.6.7135
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Turner’s Syndrome – genetic, diagnostic methods and clinical treatment
Authors: Łokczewska-Bojar Aleksandra , Ben Rhaiem Rami , Kępka Przemysław , Kuziemkowska Daria , Kuźma Jan , Łącka-Majcher Anna , Tarsa Grzegorz , Skotalczyk Magdalena , Sawińska Zuzanna , Sudelska Katarzyna 
Published: September 2023

Introduction : Turner syndrome (TS) is one of the most common genetic disorders affecting approximatley 1 in 2,000-25000 live-born girls. TS is the result of complete oraz partial monosomy of the C chromosome. About half have monosomy X (45,X) and 5-10% have duplication (isochromosome) of the long arm of one X (46,X,i(Xq)). Most of the rest have mosaicism.(1)
The aim of the study: The purpose of this review paper is to present the observations regarding this disease, including epidemiology, diagnosis and detailed examination of the individual needs of the patient affected by the disease.
Matrials and methods: This review was based on available data collected in the PubMed database and Google Scholar web serach engine, using key words: Turner’s syndrome, diagnosis of Turner Syndrome, sex chromosome aneuploidies
Summary: The article draws attention to the complexity of Turner syndrome, the variety of symptoms and the progress in the diagnosis and therapy of this disease. Early diagnosis, specialist care and modern therapeutic methods have a positive impact on the quality of life of TS patients. Future studies of molecular mechanisms and therapeutic efficacy may improve the care of patients affected by this rare disease.
Inner Moreau Envelope of Nonsmooth Conic Chance-Constrained Optimization Problems
Authors: van Ackooij Wim , Pérez-Aros Pedro , Soto Claudia , Vilches Emilio 
Published: September 2023

Optimization problems with uncertainty in the constraints occur in many applications. Particularly, probability functions present a natural form to deal with this situation. Nevertheless, in some cases, the resulting probability functions are nonsmooth, which motivates us to propose a regularization employing the Moreau envelope of a scalar representation of the vector inequality. More precisely, we consider a probability function that covers most of the general classes of probabilistic constraints: [Formula: see text]where [Formula: see text] is a convex cone of a Banach space. The conic inclusion [Formula: see text] represents an abstract system of inequalities, and ξ is a random vector. We propose a regularization by applying the Moreau envelope to the scalarization of the function [Formula: see text]. In this paper, we demonstrate, under mild assumptions, the smoothness of such a regularization and that it satisfies a type of variational convergence to the original probability function. Consequently, when considering an appropriately structured problem involving probabilistic constraints, we can, thus, entail the convergence of the minimizers of the regularized approximate problems to the minimizers of the original problem. Finally, we illustrate our results with examples and applications in the field of (nonsmooth) joint, semidefinite, and probust chance-constrained optimization problems. Funding: P. Pérez-Aros was supported by Centro de Modelamiento Matemático [Grants ACE210010 and FB210005] and BASAL funds for center of excellence and ANID-Chile grant Fondecyt Regular [Grants 1200283 and 1190110] and Fondecyt Exploración [Grant 13220097]. C. Soto was supported by the National Agency for Research and Development (ANID)/Scholarship Program/Doctorado Nacional Chile [Grant 2017-21170428]. E. Vilches was supported by Centro de Modelamiento Matemático [Grants ACE210010 and FB210005] and BASAL funds for center of excellence and Fondecyt Regular [Grant 1200283] and Fondecyt Exploración [Grant 13220097] from ANID-Chile.
Importance of stoma care nurses in preparing patients for stoma surgery and adjustment to life with a stoma
Authors: Rolls Natasha , Gotfredsen Johanne Louise , Vestergaard Martin , Hansen Anne Steen , Koblauch Henrik 
Published: September 2023

Background: Stoma surgery is a life-changing event and patients must subsequently make significant adjustments to their lives. Aim: The study set out to understand the level of interaction between patients and stoma care nurses (SCNs) in relation to preparing patients for stoma surgery and in adjustment to life with a stoma. Methods: Retrospective, self-reported questionnaires for patients and SCNs. Findings: Most patients (98%) with planned stoma surgery had pre-operative consultations with health professionals in contrast to 36% of patients with unplanned surgery, who did not. One third of patients with unplanned surgery did not feel prepared for life with a stoma based on the information provided during their hospital stay. Two thirds of the nurses reported having sufficient time to prepare patients for stoma surgery and to life with a stoma. Conclusion: SCNs are key in preparing patients for surgery and for life with a stoma. Variations in care were experienced by patients having planned versus unplanned surgeries.
Assessing nurses' attitudes towards pressure ulcer prevention: an updated systematic review
Authors: Avsar Pinar , Renjith Vishnu , Chima Comfort , Patton Declan , O'Connor Tom , Moore Zena 
Published: September 2023

Objective:The aim of this systematic review was to critically appraise and synthesise the existing research literature pertaining to nurses' attitudes toward pressure ulcer (PU) prevention.Method:The systematic review presented in our paper serves as an updated version of the definitive review conducted by Avsar et al. in 2019. Using systematic review methodology, we considered published quantitative studies focusing on nurses' attitudes toward PU prevention as measured using psychometric tests. The search was conducted in April 2022, using PubMed, CINAHL, Scopus, Cochrane and EMBASE databases, and returned 454 records, of which 35 met the inclusion criteria. Data were extracted using a pre-designed extraction tool and all included studies were quality appraised using the evidence-based librarianship (EBL) appraisal checklist.Results:In most studies, distinct measurement instruments were used for measuring nurses' attitudes toward the prevention of PUs: the Moore and Price Attitude Scale and the Attitude towards Pressure Ulcer Prevention Instrument (APuP). In this first update, the mean attitude score was 69% (±14%, range: 33.6–89%). A separate analysis of the new studies alone included in this first update (n=14) indicated a mean attitude score of 62.25% (±17.9%; median: 14%), suggesting a 10.75% lower mean attitude score. In total, 46% (n=16) yielded a score ≥75%. Conversely, Avsar et al. in 2019, 86% (n=18) of studies yielded positive attitude results. Studies from the Middle East show the lowest mean attitude score (mean 55%; ±15%; median 53%; n=9), with studies from Europe displaying the highest mean attitude score (mean 79%; ±6%; median 79%; n=12).Conclusion:The findings suggest that, overall, nurses are relatively positively disposed toward PU prevention. However, there are differences across continents. It is important to highlight also that the nurses have difficulties reflecting this positive attitude into actual preventative strategies.