Abrupt increase in Arctic-Subarctic wildfires caused by future permafrost thaw.

Unabated 21st-century climate change will accelerate Arctic-Subarctic permafrost thaw which can intensify microbial degradation of carbon-rich soils, methane emissions, and global warming. The impact of permafrost thaw on future Arctic-Subarctic wildfires and the associated release of greenhouse gases and aerosols is less well understood. Here we present a comprehensive analysis of the effect of future permafrost thaw on land surface processes in the Arctic-Subarctic region using the CESM2 large ensemble forced by the SSP3-7.0 greenhouse gas emission scenario. Analyzing 50 greenhouse warming simulations, which capture the coupling between permafrost, hydrology, and atmosphere, we find that projected rapid permafrost thaw leads to massive soil drying, surface warming, and reduction of relative humidity over the Arctic-Subarctic region. These combined processes lead to nonlinear late-21st-century regime shifts in the coupled soil-hydrology system and rapid intensification of wildfires in western Siberia and Canada.

Effects of a Preoperative Patient-Controlled Analgesia Education Program Using Smart Learning in Laparoscopic Cholecystectomy Patients.

BACKGROUND: Patients undergoing laparoscopic cholecystectomy develop severe postoperative pain, and this acute pain often becomes chronic. OBJECTIVES: This study determines the effects of preoperative education on patient-controlled analgesia (PCA) through smart learning in patients using PCA after undergoing laparoscopic cholecystectomies. DESIGN: We conducted a quasi-experimental study with a nonequivalent control group pretest-posttest design. PARTICIPANTS: A total of 60 adult patients aged 20-65 years, admitted for laparoscopic cholecystectomy at a hospital in Korea, participated in a smart learning training program. METHODS: The concept of smart learning, which integrates learning content and solutions with the fourth industrial revolution using mobile devices such as smartphones and media tablets was applied in this study. This smart learning training program comprised three phases: (1) prebriefing, where patients accessed PCA knowledge via a program created by researchers and accessible using a mobile web device (e.g., smartphone or an iPad), with training covering pain characteristics, PCA effects, precautions, and usage methods; (2) simulation, where patients learned using PCA with a machine; (3) debriefing, where patients reviewed their knowledge and skills. RESULTS: Comparing the pain between the experimental and control groups, the pain decreased significantly in both the experimental (Z = -4.40, p < .001) and control groups (Z = -4.41, p < .001), with no significant difference between groups (Z = -1.00, p = .319). Preoperative knowledge significantly increased in both the experimental (Z = -4.74, p < .001) and control groups (Z = -3.55, p < .001), with a significant difference between groups (Z = -6.05, p < .001). Total satisfaction with pain control was higher in the experimental group than in the control group. CONCLUSIONS: A structured educational program on PCA use is an effective nursing intervention. PCA educational programs using smart learning could help patients undergoing laparoscopic cholecystectomy understand postoperative pain, promote efficient PCA use, and enhance their satisfaction with pain control.

Durable, Photostable Omniphobic Synthetic Leather Surfaces with Anti-Biofouling Properties for Hygienic Applications.

Globally, the public health domain is increasingly emphasizing the need for surfaces that can resist bacterial contamination, as the consumption of bacteria-infected substance may cause illnesses. Thus, this study aimed to modify polyurethane (PU) synthetic leather surfaces by coating their upper layer with fluorine-functionalized nano-silica particles (FNPs). This simple modification imparted omniphobic characteristics, realizing anti-biofouling and self-cleaning properties. The effectiveness in preventing bacterial adhesion was confirmed by the dip-inoculation method using Escherichia coli O157:H7 and Staphylococcus epidermidis. Bacterial adhesion was evaluated based on bacterial counts using the pour plate method and by directly enumerating from scanning electron microscopy images. The attachment of bacteria to the modified omniphobic FNPs-coated PU leather surface decreased by over 98.2% compared to that on the bare surface. We expect that the method developed in this study will significantly reduce or even eliminate the potential risks associated with various biological cross-contamination scenarios, thereby enhancing hygiene standards.

Aramid-Reinforced UV Curable Adhesive Resins for Use As an Interlayer in Laminated Glass.

Colorless, transparent, and mechanically robust aramid polymers are synthesized from two diamine monomers with strong electron-withdrawing groups, using low-temperature solution condensation with diacid chloride. The aramids dissolved very well in the liquid acrylamide monomers. When N,N-dimethylacrylamide (DMA) is used as a reactive diluent, films with the desired features are produced from the hybrid aramid-DMA resins via ultraviolet (UV) curing. The hybrid films are colorless and transparent in the visible region and showed an increase in the glass transition temperature, tensile strength, and elastic modulus in proportion to the aramid content. Laminated glass is manufactured using the hybrid resin as an interlayer, which exhibits very strong adhesion between the two sheets of glass, is not easily broken by an external impact, and do not scatter fragments. Moreover, the laminated glass do not distort images and functioned very effectively in UV blocking, soundproofing, and suppressing changes in the ambient temperature. Heat treatment further improves the light transmittance and impact resistance of the laminated glass. Laminated glass specimens with various fluorescence colors are also manufactured. Aramid-reinforced films prepared using N,N-diethylacrylamide as a reactive diluent underwent thermally induced phase separation in a wet state, providing smart glass with a privacy protection function.

Empowering the on-site detection of nucleic acids by integrating CRISPR and digital signal processing.

Addressing the global disparity in cancer care necessitates the development of rapid and affordable nucleic acid (NA) testing technologies. This need is particularly critical for cervical cancer, where molecular detection of human papillomavirus (HPV) has emerged as an accurate screening method. However, implementing this transition in low- and middle-income countries has been challenging due to the high costs and centralized facilities required for current NA tests. Here, we present CreDiT (CRISPR Enhanced Digital Testing) for on-site NA detection. The CreDiT platform integrates i) a one-pot CRISPR strategy that simultaneously amplifies both target NAs and analytical signals and ii) a robust fluorescent detection based on digital communication (encoding/decoding) technology. These features enable a rapid assay (<35 minutes) in a single streamlined workflow. We demonstrate the sensitive detection of cell-derived HPV DNA targets down to single copies and accurate identification of HPV types in clinical cervical brushing specimens (n = 121).

Severe Hyponatremia Triggered by Immune Checkpoint Inhibitor Therapy in a Patient With Mulvihill-Smith Syndrome.

Background/Objective: Immune checkpoint inhibitors (ICI), including Programmed Cell Death 1, Programmed Cell Death Ligand 1, and Cytotoxic T-lymphocyte Associated Antigen 4 inhibitors, upregulate T-cell responses against tumor cells and are becoming a cornerstone in the treatment of various advanced solid and hematological cancers. Mulvihill-Smith Syndrome (MSS) is a rare genetic syndrome that has been associated with metabolic abnormalities and early-onset tumors, including malignancies. We report the first known case of ICI-induced hyponatremia attributable to syndrome of inappropriate antidiuretic hormone ADH release (SIADH) in a patient with MSS. Case Report: A 23-year-old female patient with MSS and hepatocellular carcinoma presented with recurrent hyponatremia. Assessment of fluid status and electrolytes revealed a euvolemic, hypotonic process consistent with SIADH shortly after initiating adjuvant therapy with atezolizumab, a Programmed Cell Death Ligand 1 inhibitor. Discussion: Endocrine etiologies for euvolemic hypotonic hyponatremia, including adrenal insufficiency and hypothyroidism, were excluded. The diagnosis of SIADH was confirmed based on electrolyte and osmolality studies. Sodium levels normalized with fluid restriction. Given the onset of hyponatremia 30 days after atezolizumab initiation, we posit that atezolizumab triggered severe hyponatremia due to SIADH. Conclusion: With the expanding utilization of ICIs, including in patients predisposed to malignancies such as MSS, vigilant monitoring for ICI-mediated electrolyte imbalances is crucial. Monitoring for hyponatremia and SIADH in the setting of ICI therapy is recommended.

Neonatal head circumference to maternal mid-transverse pelvic distance ratio as a key anatomical predictor for dystocia: Retrospective case-control study.

OBJECTIVE: This study aimed to examine maternal and neonatal factors in cesarean deliveries due to dystocia, including cephalopelvic disproportion, latent-phase prolongation, and fetal malposition or malpresentation. Additionally, we sought to compare the differences between the dystocia subgroups. METHOD AND MATERIALS: This retrospective case-control study included women who delivered between January 2010 and June 2021 after 37 weeks of pregnancy and underwent abdominal-pelvic CT scans within 5 years before and after delivery. Neonatal factors were extracted from medical charts immediately after delivery. RESULTS: Among the 292 women studied, those with cesarean deliveries for dystocia were older (mean ± SD, 34.2 ± 4.27 vs. 32.2 ± 3.8, p-value = 0.002), had higher pre-pregnancy BMI (22.7 ± 3.67 vs. 21.4 ± 3.48, p-value = 0.012) and term-BMI (27.4 ± 3.72 vs. 25.9 ± 3.66, p-value = 0.010), shorter interspinous distance (ISD, the distance between ischial spine) (10.8 ± 0.76 vs. 11.2 ± 0.85 cm, p-value = 0.003), and longer head circumference (HC) (35 ± 1.47 vs. 34.4 ± 1.36 cm, p-value = 0.003) compared to those who had vaginal deliveries. Univariate logistic regression for dystocia revealed associations between HC/maternal height and HC/ISD ratios (OR, 2.02 [95% confidence interval, CI, 1.4 ~ 2.92], 12.13 [3.2 ~ 46.04], respectively). Multivariate logistic analysis indicated that maternal age, ISD, and HC were significant factors for dystocia (OR, 1.11 [95% CI, 1.01 ~ 1.21], 0.49 [0.26 ~ 0.91], 1.53 [1.07 ~ 2.19], respectively). The subgroup with latent-phase prolongation exhibited the lowest birthweight/term-BMI ratio (124 ± 18.8 vs. 113 ± 10.3 vs. 134 ± 19.1, p-value = 0.013). CONCLUSION: The HC/ISD ratio emerged as a crucial predictor of dystocia, suggesting that reducing term-BMI could potentially mitigate latent-phase prolongation. Further research assessing the maternal mid-pelvis during pregnancy and labor is warranted, along with efforts to reduce BMI during pregnancy.

Discovery of a Novel Bioactive Compound in Orange Peel Polar Fraction on the Inhibition of Trimethylamine and Trimethylamine N-Oxide through Metabolomics Approaches and In Vitro and In Vivo Assays: Feruloylputrescine Inhibits Trimethylamine via Suppressing cntA/B Enzyme.

This study compares the inhibitory effects of orange peel polar fraction (OPP) and orange peel nonpolar fraction (OPNP) on trimethylamine (TMA) and trimethylamine N-oxide (TMAO) production in response to l-carnitine treatment in vivo and in vitro. Metabolomics is used to identify bioactive compounds. The research demonstrates that the OPP effectively regulates atherosclerosis-related markers, TMA and TMAO in plasma and urine, compared to the OPNP. Our investigation reveals that these inhibitory effects are independent of changes in gut microbiota composition. The effects are attributed to the modulation of cntA/B enzyme activity and FMO3 mRNA expression in vitro. Moreover, OPP exhibits stronger inhibitory effects on TMA production than OPNP, potentially due to its higher content of feruloylputrescine, which displays the highest inhibitory activity on the cntA/B enzyme and TMA production. These findings suggest that the OPP containing feruloylputrescine has the potential to alleviate cardiovascular diseases by modulating cntA/B and FMO3 enzymes without directly influencing gut microbiota composition.

Serial Dual Mediating Effects of Parenting Stress on Life Satisfaction among Parents of School-Aged Children with Chronic Conditions.

This study examines the serial dual mediating effects of marital conflict and self-esteem on the relationship between parenting stress and life satisfaction in parents of school-aged children with chronic conditions. This study aims to present foundational data for developing nursing interventions for parents caring for children with chronic illnesses. Of the 2150 parents who participated in the 13th Panel Study on Korean Children (PSKC), 271 raising a child with a chronic illness were enrolled in the study. The serial dual mediating effect was analyzed using PROCESS macro Model 6. The serial dual mediating effects of parenting stress, marital conflict, and self-esteem on parents' life satisfaction were analyzed. Marital conflict and self-esteem had significant serial multiple mediating effects on the relationship between stress and life satisfaction in fathers (B = -0.11, bootstrap 95% CI = -0.16--0.06) and mothers (B = -0.06, bootstrap 95% CI = -0.09--0.03). Our results suggest that marital conflict increases with increasing parenting stress and that increased marital conflict sequentially reduces self-esteem, ultimately diminishing life satisfaction in parents raising a child with a chronic condition. Thus, relevant nursing interventions and social support are essential to boost the life satisfaction of parents raising children with chronic conditions.

Development and evaluation of extracorporeal membrane oxygenation nursing education program for nursing students using virtual reality.

BACKGROUND: This study aims to improve nursing students' ability to care for critically ill patients through education in extracorporeal membrane oxygenation (ECMO) nursing. METHODS: This study developed a virtual reality (VR) simulation program for the five-step ECMO nursing of the Analysis, Design, Development, Implement, and Evaluation (ADDIE) model and used an equivalent control group pre-test and post-test no-synchronized design to verify the effect. The participants of this study were fourth-year nursing students enrolled in nursing departments at three universities in Seoul, Gangwon, and Gyeonggi in South Korea; it included 66 participants, 33 in each of the experimental and control groups. The program consisted of pre-training, orientation, VR simulation, and debriefing. RESULTS: The interaction effect of the intervention and control groups with time points using the ECMO nursing VR simulation program was rejected due to no statistically significant difference in knowledge (F = 1.41, p = .251), confidence (F = 1.97, p = .144), and clinical reasoning capacity (F = 2.85, p = .061). However, learning immersion (t = 3.97, p < .001) and learning satisfaction (t = 4.25, p < .001) were statistically significantly higher in the experimental group than in the control group. CONCLUSION: VR simulation program for ECMO nursing developed in this study is a potential educational method that positively affects the learning immersion and learning satisfaction of nursing students.

An Exploratory Analysis of the Roles of Nurses on a Pediatric Rehabilitation Unit in South Korea Perceived by Pediatric Rehabilitation Professionals.

Rehabilitation addresses not only children's disabilities but also their physical, psychological, social, and cultural impairments. Hence, pediatric rehabilitation adopts a multidisciplinary approach; it encompasses the vital role of not only physicians and rehabilitation therapists, but also of nurses. This study conducts a content analysis of the experiences of healthcare professionals specializing in pediatric rehabilitation to explore the roles nurses working on pediatric rehabilitation units are expected to perform. After analyzing the interviews with 12 experts in pediatric rehabilitation, the roles of pediatric rehabilitation nurses were broadly categorized into five areas (caregivers, team members, counselors, researchers, and educators) with eight sub-groups and 24 specific roles. This study is significant because it provides profound insights into the roles of pediatric rehabilitation nurses in Korea. These insights can serve as foundational data for formulating policies for healthcare personnel in pediatric rehabilitation, and provide evidence for establishing a much-needed system for certified rehabilitation nurses in Korea.

Vanadium Alkylidyne Initiated Cyclic Polymer Synthesis: The Importance of a Deprotiovanadacyclobutadiene Moiety.

Reported is the catalytic cyclic polymer synthesis by a 3d transition metal complex: a V(V) alkylidyne, [(dBDI)V≡CtBu(OEt2)] (1-OEt2), supported by the deprotonated ß-diketiminate dBDI2- (dBDI2- = ArNC(CH3)CHC(CH2)NAr, Ar = 2,6-iPr2C6H3). Complex 1-OEt2 is a precatalyst for the polymerization of phenylacetylene (PhCCH) to give cyclic poly(phenylacetylene) (c-PPA), whereas its precursor, complex [(BDI)V≡CtBu(OTf)] (2-OTf; BDI- = [ArNC(CH3)]2CH, Ar = 2,6-iPr2C6H3, OTf = OSO2CF3), and the zwitterion [((C6F5)3B-dBDI)V≡CtBu(OEt2)] (3-OEt2) exhibit low catalytic activity despite having a neopentylidyne ligand. Cyclic polymer topologies were verified by size-exclusion chromatography (SEC) and intrinsic viscosity studies. A component of the mechanism of the cyclic polymerization reaction was probed by isolation and full characterization of 4- and 6-membered metallacycles as model intermediates. Metallacyclobutadiene (MCBD) and deprotiometallacyclobutadiene (dMCBD) complexes (dBDI)V[C(tBu)C(H)C(tBu)] (4-tBu) and (BDI)V[C(tBu)CC(Mes)] (5-Mes), respectively, were synthesized upon reaction with bulkier alkynes, tBu- (tBuCCH) and Mes-acetylene (MesCCH), with 1-OEt2. Furthermore, the reaction of the conjugate acid of 1-OEt2, [(BDI)V≡CtBu(OTf)] (2-OTf), with the conjugated base of phenylacetylene, lithium phenylacetylide (LiCCPh), yields the doubly deprotio-metallacycle complex, [Li(THF)4]{(BDI)V[C(Ph)CC(tBu)CC(Ph)]} (6). Protonation of the doubly deprotio-metallacycle complex 6 yields 6-H+, a catalytically active species toward the polymerization of PhCCH, for which the polymers were also confirmed to be cyclic by SEC studies. Computational mechanistic studies complement the experimental observations and provide insight into the mechanism of cyclic polymer growth. The noninnocence of the supporting dBDI2- ligand and its role in proton shuttling to generate deprotiometallacyclobutadiene (dMCBD) complexes that proposedly culminate in the formation of catalytically active V(III) species are also discussed. This work demonstrates how a dMCBD moiety can react with terminal alkynes to form cyclic polyalkynes.

A mouse model of Zhu-Tokita-Takenouchi-Kim syndrome reveals indispensable SON functions in organ development and hematopoiesis.

Rare diseases are underrepresented in biomedical research, leading to insufficient awareness. Zhu-Tokita-Takenouchi-Kim (ZTTK) syndrome is a rare disease caused by genetic alterations that result in heterozygous loss of function of SON. While patients with ZTTK syndrome live with numerous symptoms, the lack of model organisms hampers our understanding of SON and this complex syndrome. Here, we developed Son haploinsufficiency (Son+/-) mice as a model of ZTTK syndrome and identified the indispensable roles of Son in organ development and hematopoiesis. Son+/- mice recapitulated clinical symptoms of ZTTK syndrome, including growth retardation, cognitive impairment, skeletal abnormalities, and kidney agenesis. Furthermore, we identified hematopoietic abnormalities in Son+/- mice, including leukopenia and immunoglobulin deficiency, similar to those observed in human patients. Surface marker analyses and single-cell transcriptome profiling of hematopoietic stem and progenitor cells revealed that Son haploinsufficiency shifted cell fate more toward the myeloid lineage but compromised lymphoid lineage development by reducing genes required for lymphoid and B cell lineage specification. Additionally, Son haploinsufficiency caused inappropriate activation of erythroid genes and impaired erythropoiesis. These findings highlight the importance of the full gene expression of Son in multiple organs. Our model serves as an invaluable research tool for this rare disease and related disorders associated with SON dysfunction.

Invasive Pneumococcal Disease After 2 Decades of Pneumococcal Conjugate Vaccine Use.

OBJECTIVES: We sought to describe the evolving epidemiology of invasive pneumococcal disease (IPD) among children in Massachusetts, United States, over the last 2 decades during which sequential 7-valent pneumococcal conjugate vaccines (PCV7) and 13-valent PCVs (PCV13) were implemented. METHODS: Cases of IPD in children aged <18 years were detected between 2002 and 2021 through an enhanced population-based, statewide surveillance system. Streptococcus pneumoniae isolates from normally sterile sites were serotyped and evaluated for antimicrobial susceptibility. IPD incidence rates and rate ratios with 95% confidence intervals (CIs) were calculated. RESULTS: We identified 1347 IPD cases. Incidence of IPD in children aged <18 years declined 72% over 2 decades between 2002 and 2021 (incidence rate ratios 0.28, 95% CI 0.18-0.45). IPD rates continued to decline after replacement of PCV7 with PCV13 (incidence rate ratios 0.25, 95% CI 0.16-0.39, late PCV7 era [2010] versus late PCV13 era [2021]). During the coronavirus disease 2019 pandemic years, 2020 to 2021, the rate of IPD among children aged <18 years reached 1.6 per 100 000, the lowest incidence observed over the 20 years. In PCV13 era, approximately one-third of the IPD cases in children aged >5 years had at least 1 underlying condition (98, 30.3%). Serotypes 19A and 7F contributed 342 (48.9%) of all cases before implementation of PCV13 (2002-2010). Serotype 3 (31, 8.6%), and non-PCV13 serotypes 15B/C (39, 10.8%), 33F (29, 8.0%), 23B (21, 0.8%), and 35B (17, 4.7%) were responsible for 37.8% of cases in PCV13 era (2011-2021). Penicillin nonsusceptibility continued to decline (9.8% vs 5.3% in pre-/late PCV13 era, P = .003), however has become more common among non-PCV13 serotypes compared with vaccine serotypes (14.8% vs 1.4%, P < .001). CONCLUSIONS: Robust ongoing surveillance networks are critical for identifying emerging serotypes and development of next-generation vaccine formulations.

Highly Enantioselective 6π Photoelectrocyclizations Engineered by Hydrogen Bonding.

Photochemical electrocyclization reactions are valued for both their ability to produce structurally complex molecules and their central role in elucidating fundamental mechanistic principles of photochemistry. We present herein a highly enantioselective 6π photoelectrocyclization catalyzed by a chiral Ir(III) photosensitizer. This transformation was successfully realized by engineering a strong hydrogen-bonding interaction between a pyrazole moiety on the catalyst and a basic imidazolyl ketone on the substrate. To shed light on the origin of stereoinduction, we conducted a comprehensive investigation combining experimental and computational mechanistic studies. Results from density functional theory calculations underscore the crucial role played by the prochirality and the torquoselectivity in the electrocyclization process as well as the steric demand in the subsequent [1,4]-H shift step. Our findings not only offer valuable guidance for developing chiral photocatalysts but also serve as a significant reference for achieving high levels of enantioselectivity in the 6π photoelectrocyclization reaction.

Control Volume Analysis of the Infusion Rate in Cephalic and Median Cubital Veins Based on Infusion Bag Height and Peripheral Venous Catheter Inner Diameter: Application of Bernoulli's Equation and Consideration of Frictional Forces.

Purpose: This pilot study aimed to provide basic data on intravenous infusion nursing by analyzing the infusion rate in the cephalic and median cubital veins depending on the height of the infusion bag and inner diameter of the peripheral venous catheter (PVC). Methods: While infusing 0.9% normal saline at 22 °C (room temperature) into elbow cephalic and median cubital veins, the infusion rate may be controlled by adjusting the fluid height and PVC diameter. To assess the validity of the laminar flow assumption, the study estimated the Reynolds number (Re) using the velocity obtained by applying Bernoulli's equation considering the friction coefficient. Results: At a constant fluid height, the infusion rate increased with increasing PVC diameter. At a constant PVC diameter, the infusion rate increased with increasing fluid height. In a comparison between the cephalic and median cubital veins at constant fluid height and PVC diameter, the solution was infused at a higher rate into the cephalic vein, which was under lower venous pressure. Conclusion: The analysis of the infusion rate according to fluid height and PVC diameter provided basic data on intravenous infusion nursing. The results are expected to provide evidence for the standardization of intravenous infusion nursing.

Chiral Diketopyrrolopyrrole-Based Conjugated Polymers with Intramolecular Rotation-Isomeric Conformation Asymmetry for Near-Infrared Circularly Polarized Light-Sensing Organic Phototransistors.

Recent advances in chiral nanomaterials interacting with circularly polarized (CP) light open new expectations for optoelectronics in various research fields such as quantum- and biology-related technology. To fully utilize the great potential of chiral optoelectronic devices, the development of chiral optoelectronic devices that function in the near-infrared (NIR) region is required. Herein, we demonstrate a NIR-absorbing, chiroptical, low-band-gap polymer semiconductor for high-performance NIR CP light phototransistors. A newly synthesized diketopyrrolopyrrole-based donor-acceptor-type chiral π-conjugated polymer with an asymmetric alkyl side chain exhibits strong chiroptical activity in a wavelength range of 700-1000 nm. We found that the attachment of an enantiomerically pure stereogenic alkyl substituent to the π-conjugated chromophore backbone led to strong chiroptical activity through symmetry breaking of the π-conjugation of the backbone in a molecular rotational motion while maintaining the coplanar backbone conformation for efficient charge transport. The NIR CP light-sensing phototransistors based on a chiral π-conjugated polymer photoactive single channel layer exhibit a high photoresponsivity of 26 A W-1 under NIR CP light irradiation at 920 nm, leading to excellent NIR CP light distinguishability. This study will provide a rationale and strategy for designing chiral π-conjugated polymers for high-performance NIR chiral optoelectronics.

A mouse model of ZTTK syndrome reveals indispensable SON functions in organ development and hematopoiesis.

Rare diseases are underrepresented in biomedical research, leading to insufficient awareness. Zhu-Tokita-Takenouchi-Kim (ZTTK) syndrome is a rare disease caused by genetic alterations that result in heterozygous loss-of-function of SON. While ZTTK syndrome patients suffer from numerous symptoms, the lack of model organisms hamper our understanding of both SON and this complex syndrome. Here, we developed Son haploinsufficiency (Son+/-) mice as a model of ZTTK syndrome and identified the indispensable roles of Son in organ development and hematopoiesis. Son+/- mice recapitulated clinical symptoms of ZTTK syndrome, including growth retardation, cognitive impairment, skeletal abnormalities, and kidney agenesis. Furthermore, we identified hematopoietic abnormalities in Son+/- mice, similar to those observed in human patients. Surface marker analyses and single-cell transcriptome profiling of hematopoietic stem and progenitor cells revealed that Son haploinsufficiency inclines cell fate toward the myeloid lineage but compromises lymphoid lineage development by reducing key genes required for lymphoid and B cell lineage specification. Additionally, Son haploinsufficiency causes inappropriate activation of erythroid genes and impaired erythroid maturation. These findings highlight the importance of the full gene dosage of Son in organ development and hematopoiesis. Our model serves as an invaluable research tool for this rare disease and related disorders associated with SON dysfunction.

Cognitive Task Analysis of Escharotomy.

INTRODUCTION: With the Army's emerging doctrine of prolonged field care, and with burns being a common injury among soldiers, non-expert providers must be trained to perform escharotomy when indicated. However, the existing physical simulators and training protocols are not sufficient for training non-experts for performing effective escharotomy. Hence, to provide guidance in developing realistic escharotomy simulators and effective training protocols suitable for prolonged field care, a cognitive task analysis (CTA) is needed. This work aims to obtain educative information from expert burn surgeons regarding escharotomy procedures via the CTA. MATERIALS AND METHODS: The CTA was done by interviewing five subject matter experts with experience in performing escharotomy ranging from 20 to over 100 procedures and analyzing their responses. Interview questions were developed to obtain educative information from expert burn surgeons regarding the escharotomy procedure. A "gold standard protocol" was developed based on the CTA of each of the subject matter experts. RESULTS: The CTA helped identify general themes, including objectives, conditions that mandate escharotomy, signs of successful escharotomy, precautions, challenges, decisions, and performance standards, and specific learning goals such as the use of equipment, vital signs, performing the procedure, and preoperative and postoperative care. A unique aspect of this CTA is that it identifies the background information and preparations that could be useful to the practitioners at various levels of expertise. CONCLUSIONS: The CTA enabled us to compile a "gold standard protocol" for escharotomy that may serve as a guide for practitioners at various levels of expertise. This information will provide a framework for escharotomy training systems and simulators.

Maternal vitamin D deficiency in early pregnancy and perinatal and long-term outcomes.

Background: Vitamin D deficiency is common in pregnant women. Some studies have linked vitamin D deficiency to obstetric complications such as gestational hypertension, gestational diabetes, and preterm birth. Therefore, the objective of this study is to investigate the potential impact of vitamin D deficiency during pregnancy on both perinatal and long-term outcomes. Methods: In this retrospective study, conducted between 2017 and 2021, we analyzed the data of 1079 singleton pregnant women with no medical or surgical complications prior to pregnancy. We evaluated obstetric and perinatal outcomes, as well as neurodevelopmental outcomes using Bayley-III tests, Gross Motor Function Measure, or chart review. Results: The maternal serum vitamin D level in the first trimester was 18.2 ± 9.0 ng/mL. Vitamin D deficiency (<20 ng/mL) was found in 308 (62.0%) women in the first trimester, of which 288 women (26.7%) were in the very deficient group (<10 ng/mL). There were no differences in maternal age, body mass index, and previous preterm birth between the group with vitamin D < 10 ng/mL and ≥10 ng/mL group. There were also no differences in the rates of gestational hypertension, gestational diabetes, and preterm birth between the two groups, except for the rate of preterm birth before 37 weeks of gestation, which was significantly higher in the very deficient group (adjusted odds ratios [aOR] = 7.78, 95%CI [2.23-27.12], p = 0.001). In the very deficient group, the risk of developmental delay was also higher (aOR = 4.28, 95%CI [1.40-13.05], p = 0.011). Conclusions: This is the first study to analyze the effects of maternal vitamin D deficiency during pregnancy on both long-term developmental outcomes and perinatal prognosis. Vitamin D deficiency, defined as a level lower than 10 ng/mL in the first trimester, may increase the risk of preterm birth and developmental delay in children.