External auditory canal cholesteatoma in children: clinical manifestations.

PURPOSE: The purpose of this study was to explore the characteristics of external auditory canal cholesteatoma (EACC) among children and to describe its radiological findings on high-resolution computed tomography (CT) of the temporal bone in order to improve the diagnostic accuracy of primary EACC. METHODS: The clinical records and CT imaging features of 44 patients who were diagnosed with EACC between January 2017 and May 2022 at Shenzhen Children's Hospital were retrospectively reviewed. Clinical features, including external auditory canal wall findings, hearing damage, symptoms and physical examination findings, were analysed against the level of lesion involvement. The correlation between different types of EACC and the incidence of different clinical symptoms was analysed, and the degree of hearing impairment and the rate of bone wall destruction were examined using CT. RESULTS: The mean age at EACC onset was 9.02 ± 3.15 years, and the mean age at onset for EACC involving the right ear was older than that of EACC involving the left ear (P < 0.05). There were 44 patients (46 ears), including 10 ears with type I EACC, 23 ears with type II EACC, and 13 ears with type III EACC. Conductive hearing loss was the main type of hearing impairment observed among EACC patients. There were differences in types I, II and III EACC in terms of hearing impairment; specifically, there was a significant difference in moderate hearing impairment between type II and type III EACC patients (P < 0.05). The four most common symptoms were otorrhea, otalgia, itching and bleeding. The incidence of itching symptoms was greater in type I EACC than the incidence of otorrhea, and the incidence of otorrhea symptoms in type II and type III EACC was significantly greater than that in type I EACC(P < 0.05). There were no significant differences in the fracture rates of the anterior, posterior, superior or inferior walls of the external auditory canal within or between type II and type III EACC patients (P > 0.05). The failure rate of scute damage was significantly higher in type III EACC patients than in type II EACC patients (P < 0.05). CONCLUSION: The presence of otorrhea and hearing loss as well as the identification of granulation tissue during otoscopy suggest the need for a temporal bone CT scan. This imaging modality can aid in the early detection and accurate classification of EACC, thereby guiding the selection of appropriate surgical interventions and greatly assisting in preventing further progression of hearing impairment.

Effectiveness, safety, and treatment pattern of sodium zirconium cyclosilicate in Chinese patients with hyperkalemia: interim analysis from a multicenter, prospective, real-world study (Actualize Study).

Introduction: Sodium zirconium cyclosilicate (SZC) is a nonabsorbed cation-exchanger approved in China for the treatment of hyperkalemia [HK; serum potassium (sK+) levels >5.0 mmol/L]. This is the first real-world study aimed to assess the effectiveness, safety, and treatment patterns of SZC in Chinese patients with HK. Here we present the results of the first interim analysis. Methods: This multicenter, prospective, cohort study included patients aged ≥18 years with documented HK within 1-year before study enrollment day. These patients were followed up for 6 months from the enrollment day after initiating SZC treatment. The treatment was categorized into correction phase (FAS-P1) and maintenance phase (FAS-P2 new and ongoing users). Subgroup analysis was performed in patients on hemodialysis (FAS-H). The primary objective was evaluation of safety profile of SZC; secondary objectives included assessment of treatment patterns of SZC and its effectiveness. Results: Of 421 screened patients, 193, 354, and 162 patients were enrolled in the FAS-P1, FAS-P2, and FAS-H groups, respectively. sK+ levels were reduced significantly from 5.9 mmol/L to 5.0 mmol/L after the correction phase. For the maintenance phase, the mean sK+ levels were maintained at 5.2 mmol/L and 5.0 mmol/L in the FAS-P2 new and ongoing user, respectively, and 5.3 mmol/L in the FAS-H subgroup. A considerable proportion of patients showed normokalemia after 48 h of SZC treatment (FAS-P1:51.3%) which was maintained up to 6 months in the maintenance phase (FAS-P2:44%). SZC was well-tolerated. Conclusion: SZC was effective and safe for the treatment of HK in real-world clinical practice in China.

Clinical efficacy and safety of flexible ureteroscopy and percutaneous nephrolithotomy for large kidney stones: A retrospective comparative study.

BACKGROUND: Renal stones ranging 20-40 mm are very common in China. Although no large-sample clinical studies have confirmed the clinical efficacy and safety of this method, there is also a lack of comparative data with traditional treatment. AIM: To investigate the clinical efficacy of flexible ureteroscopy (FURS) and percutaneous nephrolithotomy (PCNL) by postoperative stone clearance and changes in soluble vascular cell adhesion molecule 1 (sVCAM-1) and kidney injury molecule 1 (KIM-1) levels in patients with large kidney stones (> 2 cm in diameter). METHODS: This single-center observational study was performed at a Chinese hospital between January 1, 2021, and October 30, 2023. All 250 enrolled patients were diagnosed with large kidney stones (> 2 cm) and divided into a FURS group (n = 145) and a PCNL group (n = 105) by the surgical method. The FURS group was treated with flexible ureteroscopy and the PCNL group was treated with percutaneous nephrolithotomy. The operation time, time to palinesthesia, intraoperative blood loss, drop in hemoglobin, length of hospital stay, stone clearance rate, and complications were recorded in the two groups. Preoperative and postoperative serum sVCAM-1 levels, erythrocyte sedimentation rate (ESR), urine KIM-1 levels, preoperative and postoperative pain visual analog scale (VAS) and Wisconsin Stone Quality of Life Questionnaire (WISQOL) scores were also documented. RESULTS: All 250 eligible patients completed the follow-up. There were no significant differences in baseline characteristics between the two groups (P > 0.05). The operation time in the FURS group was significantly greater than that in the PCNL group. The time to ambulation, intraoperative blood loss, decrease in hemoglobin, and length of hospital stay were significantly lower in the FURS group than in the PCNL group. The FURS group also had a significantly higher stone clearance rate and a lower incidence of postoperative complications. There was no significant difference in antibiotic use between the groups. Postoperative serum sVCAM-1 levels, urine KIM-1 levels, and VAS scores were lower in the FURS group than in the PCNL group, but postoperative ESR and WISQOL scores were greater in the FURS group than in the PCNL group. CONCLUSION: FURS demonstrated superior clinical efficacy in treating large kidney stones (> 2 cm in diameter) compared PCNL. It not only improved the postoperative stone clearance rate and reduced complications and recovery time but also positively affected serum SCM-1, ESR, and urine KIM-1 levels, subsequent improvement of patient quality of life.

PROTOCOL: Effectiveness of behavioral interventions for smoking cessation among homeless persons: A systematic review and meta-analysis.

This is the protocol for an updated Campbell systematic review. The objectives are as follows: To evaluate the effect of behavioral interventions on smoking cessation among homeless individuals.

Profiles of Family and School Experiences and Adjustment of Adolescents During the Transition to High School.

Although family and school experiences play an important role in adolescents' adjustment during the transition to high school, most prior studies investigated the effects of these experiences in isolation; their joint implications for both adolescents' concurrent and long-term adjustment outcomes are less clear, and the potential role of individual characteristics within such associations remains understudied. Based on 525 10th graders (Mage = 15.48, SDage = 0.71, 43.6% boys) who participated in a longitudinal study, the present research aimed to identify distinct family and school experience profiles among first-year high school students and examine their associations with adolescents' internalizing problems and externalizing problems, both concurrently and 18 months later. Latent profile analysis revealed four distinctive profiles: thriving, low resources-moderate family risk, developmental stress-high parental conflicts, and developmental stress-high peer victimization profiles. The other three profiles (vs. the thriving profile) reported significantly higher levels of concurrent internalizing problems; while these differences diminished after 18 months. However, the enduring impacts of these profiles on internalizing problems persisted among adolescents with higher levels of environmental sensitivity. Additionally, adolescents characterized by two developmental stress profiles (vs. the thriving profile) exhibited significantly higher levels of externalizing problems both currently and longitudinally. Findings underscore the importance of identifying at-risk populations among adolescents during the transition to high school by including both family and school experiences when examining environmental influence on their adjustment, as well as the necessity to take individual environmental sensitivity into account when examining these associations.

Prospective Investigation of Optical Genome Mapping for Prenatal Genetic Diagnosis.

BACKGROUND: Optical genome mapping (OGM) is a novel assay for detecting structural variants (SVs) and has been retrospectively evaluated for its performance. However, its prospective evaluation in prenatal diagnosis remains unreported. This study aimed to prospectively assess the technical concordance of OGM with standard of care (SOC) testing in prenatal diagnosis. METHODS: A prospective cohort of 204 pregnant women was enrolled in this study. Amniotic fluid samples from these women were subjected to OGM and SOC testing, which included chromosomal microarray analysis (CMA) and karyotyping (KT) in parallel. The diagnostic yield of OGM was evaluated, and the technical concordance between OGM and SOC testing was assessed. RESULTS: OGM successfully analyzed 204 cultured amniocyte samples, even with a cell count as low as 0.24 million. In total, 60 reportable SVs were identified through combined OGM and SOC testing, with 22 SVs detected by all 3 techniques. The diagnostic yield for OGM, CMA, and KT was 25% (51/204), 22.06% (45/204), and 18.14% (37/204), respectively. The highest diagnostic yield (29.41%, 60/204) was achieved when OGM and KT were used together. OGM demonstrated a concordance of 95.56% with CMA and 75.68% with KT in this cohort study. CONCLUSIONS: Our findings suggest that OGM can be effectively applied in prenatal diagnosis using cultured amniocytes and exhibits high concordance with SOC testing. The combined use of OGM and KT appears to yield the most promising diagnostic outcomes.

The role of IL-36 and 37 in hepatocellular carcinoma.

Hepatocellular carcinoma (HCC) has garnered considerable attention due to its morbidity and mortality. Although the precise mechanisms underlying HCC tumorigenesis remain to be elucidated, evidence suggests that host immunity plays a pivotal role in its development. IL-36 and IL-37 are important immunoregulatory cytokines classified as pro-inflammatory and anti-inflammatory respectively. In the context of HCC, the downregulation of intrahepatic IL-36 is inversely correlated with cirrhosis, but positively correlated with 5-year survival rates, suggesting that IL-36 offers protection during HCC development. However, IL-36 may lose its hepatoprotective effects as the disease progresses to HCC in the context of dysregulated immunity in cirrhotic patients. Substantially increased circulating IL-36 in HCC patients is likely a systemic response to HCC stimulation, but is insufficient to suppress progression towards HCC. Intrahepatic IL-37 is suppressed in HCC patients, consistent with the inverse correlation between intrahepatic IL-37 and the level of AFP in HCC patients, suggesting IL-37 exerts hepatoprotection. There is no significant difference in IL-37 among differentiations of HCC or with respect to clinical BCLC stages or cirrhosis status in HCC patients. However, IL-37 protection is demonstrated in an IL-37 transfected HCC animal model, showing significantly reduced tumour size. IL-36/37 may inhibit HCC by enhancing M1 tumour-associated macrophages while not affecting M2 macrophages. The interplay between IL-36 (pro-inflammatory) and IL-37 (anti-inflammatory) is emerging as a crucial factor in host protection against the development of HCC. Further research is needed to investigate the complex mechanisms involved and the therapeutic potential of targeting these cytokines in HCC management.

Nanopore sequencing of infectious fluid is a promising supplement for gold-standard culture in real-world clinical scenario.

Introduction: Infectious diseases are major causes of morbidity and mortality worldwide, necessitating the rapid identification and accurate diagnosis of pathogens. While unbiased metagenomic next-generation sequencing (mNGS) has been extensively utilized in clinical pathogen identification and scientific microbiome detection, there is limited research about the application of nanopore platform-based mNGS in the diagnostic performance of various infectious fluid samples. Methods: In this study, we collected 297 suspected infectious fluids from 10 clinical centers and detected them with conventional microbiology culture and nanopore platform-based mNGS. The objective was to assess detective and diagnostic performance of nanopore-sequencing technology (NST) in real-world scenarios. Results: Combined with gold-standard culture and clinical adjudication, nanopore sequencing demonstrated nearly 100% positive predictive agreements in microbial-colonized sites, such as the respiratory and urinary tracts. For samples collected from initially sterile body sites, the detected microorganisms were highly suspected pathogens, and the negative predictive agreements were relatively higher than those in the microbial-colonized sites, particularly with 100% in abscess and 95.7% in cerebrospinal fluid. Furthermore, consistent performance was also observed in the identification of antimicrobial resistance genes and drug susceptibility testing of pathogenic strains of Escherichia coli, Staphylococcus aureus, and Acinetobacter baumannii. Discussion: Rapid NST is a promising clinical tool to supplement gold-standard culture, and it has the potential improve patient prognosis and facilitate clinical treatment of infectious diseases.

Progress of Research on Conductive Hydrogels in Flexible Wearable Sensors.

Conductive hydrogels, characterized by their excellent conductivity and flexibility, have attracted widespread attention and research in the field of flexible wearable sensors. This paper reviews the application progress, related challenges, and future prospects of conductive hydrogels in flexible wearable sensors. Initially, the basic properties and classifications of conductive hydrogels are introduced. Subsequently, this paper discusses in detail the specific applications of conductive hydrogels in different sensor applications, such as motion detection, medical diagnostics, electronic skin, and human-computer interactions. Finally, the application prospects and challenges are summarized. Overall, the exceptional performance and multifunctionality of conductive hydrogels make them one of the most important materials for future wearable technologies. However, further research and innovation are needed to overcome the challenges faced and to realize the wider application of conductive hydrogels in flexible sensors.

[Analysis of clinical features and genetic variant in a child with Cowden syndrome 1].

OBJECTIVE: To explore the genetic etiology of a child with Cowden syndrome 1 (CS1). METHODS: A child who had visited the Ningbo Women and Children's Hospital on August 26, 2022 was selected as the study subject. Clinical information of the child was collected. Genomic DNA was extracted from peripheral blood samples of the child and his family members and subjected to whole exome sequencing (WES). Candidate variant was verified by Sanger sequencing. RESULTS: The child, a 13-year-old boy, had manifested with severe mental retardation, hyperactivity, autistic behavior, sparse and prominent teeth, macrocephaly, and skin freckles on the penis. His mother had presented with multiple papules, hamartomatous polyps, thyroid adenoma and macrocephaly. WES results revealed that the child has harbored a nonsense c.781C>T (p.Q261*) variant of the PTEN gene, which was inherited from his mother. Based on the guidelines from the American College of Medical Genetics and Genomics, the c.781C>T variant was classified as likely pathogenic (PVS1+PM2_Supporting). CONCLUSION: The c.781C>T variant of the PTEN gene probably underlay the pathogenesis in the child and his mother. Above finding has facilitated genetic counseling for this family.

Effect of traditional therapeutics on prevalence and clinical outcomes of coronavirus disease 2019 in Chinese patients with autoimmune diseases.

The impact of the Coronavirus disease 2019 (COVID-19) pandemic on autoimmune diseases (AID) patients has been an important focus. This study was undertaken to characterize the incidence, clinical manifestations and hospitalization among AID affected by COVID-19 and to analyze the association between immunomodulatory medication and these outcomes. Clinical, demographic, maintenance treatment, symptoms and disease course data and outcomes of AID patients with COVID-19 infection were assessed via an online survey tool and printed copy from 1 January till February 28, 2023. A total of 432 patients with AID were enrolled in the study. The results showed the most common conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) was hydroxychloroquine (HCQ). The usage of csDMARDs didn't increase the risk of COVID-19 infection. Patients who warranted hospitalization were significantly older. ILD was associated with higher hospitalization rate. No csDMARDs other than calcineurin inhibitor (CNI) was associated with increased risk of hospitalization. HCQ intake was associated with cough. Compared with no glucocorticoids (GCs) group, high doses of GCs were accompanied with higher proportion of gastrointestinal symptoms and tachycardia, lower proportion of sore throat and ageusia. GCs didn't provoke the COVID-19 infection in patients with AID, but chronic use of oral GCs was significantly more common in those requiring hospitalization, and higher dose of GCs were correlated with higher risk of hospitalization. 97 patients discontinued csDMARDs after infection, which resulted in an elevated risk of hospitalization. Meanwhile, withdrawal of csDMARDs was associated with higher odds of disease flare and lower proportion of remission than maintenance groups. Collectively, our analysis provides the evidence that maintenance treatment of csDMARDs may be more prudent for AID patients during COVID-19 pandemic.

[Clinical phenotype and genetic analysis of a fetus with Cardiac valvular dysplasia type 1].

OBJECTIVE: To explore the genetic basis for a fetus with Cardiac valvular dysplasia type 1 (CVDP1). METHODS: A CVDP1 fetus identified at the Ningbo Women and Children's Hospital on July 7, 2022 was selected as the study subject. Clinical data of the fetus was collected. The fetus and its parents were subjected to trio-whole exome sequencing (trio-WES), and candidate variants were verified by Sanger sequencing. RESULTS: The fetus had exhibited generalized edema, complex cardiac malformation, abdominal effusion, and enhanced intestinal and renal parenchymal echoes. Trio-WES revealed that it has harbored compound heterozygous variants of the PLD1 gene, namely c.2977C>T (p.R993*) and c.1460G>A (p.W487*), which were respectively inherited from its father and mother. Neither variant was reported previously. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the c.2977C>T (p.R993*) variant was evaluated to be likely pathogenic (PVS1_Moderate+PM2_Supporting+PM3+PP4), whilst the c.1460G>A (p.W487*) variant was evaluated to be pathogenic (PVS1+PM2_Supporting+PP4). CONCLUSION: The c.2977C>T (p.R993*) and c.1460G>A (p.W487*) compound heterozygous variants of the PLD1 gene probably underlay the CVDP1 in the fetus. Above discovery has enriched the mutational spectrum of the PLD1 gene and provided a guidance for genetic counseling and prenatal diagnosis in this family.

Physicochemical properties and solution conformation of polysaccharides from Toona sinensis (A. Juss) Roem leaves.

In this study, two polysaccharide fractions (TSP-1 and TSP-2) were isolated from Toona sinensis leaves. The physicochemical properties and solution conformations of TSP-1 and TSP-2 were investigated. DSC and TG results showed that TSP-1 and TSP-2 had thermal stability. The intrinsic viscosities of TSP-1 and TSP-2 solutions were 11.42 and 6.13 mL/g, respectively. Rheological results showed that the viscosities of TSP-1 and TSP-2 solutions were affected by polysaccharide concentration, Ca2+ and extreme pH. Furthermore, TSP-1 exhibited a weak gel behavior at the concentrations of 0.5 %-2.0 %, while TSP-2 showed a weak gel behavior at the concentration of 2 %. HPSEC-MALLS analysis revealed that the Rg values of TSP-1 and TSP-2 were 96.8 nm and 56.2 nm, respectively. Conformation analysis indicated that TSP-1 behaved as a sphere, while TSP-2 behaved like a rigid rod. These results suggest that TSP-1 and TSP-2 can be used as additives in food, pharmaceutical and cosmetic industries.

Protective role of resilience on the associations between childhood maltreatment and internalising and externalising problems.

Different types of childhood maltreatment have negative effects on individual mental and behavioural outcomes. However, most of previous studies investigated their effects separately. Little is known about the effects of co-occurring maltreatment profiles on adolescents' developmental outcomes and the potential protective factor. The current study sought to identify distinct profiles of childhood maltreatment and examine the effects of profiles of childhood maltreatment on internalising and externalising problems and the protective role of resilience based on two-wave longitudinal data, which was collected from a sample of 670 Chinese adolescents (Mage = 15.50, SDage = 0.75, 48.4% boys). Four profiles of childhood maltreatment, that is, No maltreatment (67.9%), High neglect (23.0%), High abuse and neglect/Low sexual abuse (5.0%), and Multi-maltreatment (4.1%), were identified. Adolescents in High neglect, High abuse and neglect/Low sexual abuse, and Multi-maltreatment profiles were more likely to report internalising and externalising problems. Further, significant moderating effects of resilience only emerged for the association between the High neglect profile and internalising problems, such that high levels of resilience may weaken the association between the High neglect profile and internalising problems. Our findings revealed the importance and utility of identifying maltreatment profiles to tailor treatment based on specific maltreatment experiences. Resilience-oriented intervention could be considered for Chinese adolescents who have experienced high neglect.

Reciprocal Links Between Friendship Quality and Peer Victimization Among Middle Adolescents.

There is a robust association between friendship quality and victimization in adolescence; yet, it remains unclear whether friendship quality may be linked causally with different forms of victimization in middle adolescence. To fill this gap, this study examines the bidirectional associations of friendship quality and relational/verbal victimization with data collected at two time points, 6 months apart, in a sample of 671 middle Chinese adolescents (Mage = 15.63, SDage = 0.73, 49% males). Cross-lagged panel analyses revealed a two-way relationship between friendship quality and victimization, which existed in both verbal and relational victimization. Multi-group panel analyses observed that the cross-lagged associations between friendship quality and relational/verbal victimization were only found for males, but not for females. This result suggests that adolescent males' victimization is both affected by and a predictor of friendship quality, with implications for youth prevention programs.

MFA-UNet: a vessel segmentation method based on multi-scale feature fusion and attention module.

Introduction: The accurate segmentation of retinal vessels is of utmost importance in the diagnosis of retinal diseases. However, the complex vessel structure often leads to poor segmentation performance, particularly in the case of microvessels. Methods: To address this issue, we propose a vessel segmentation method composed of preprocessing and a multi-scale feature attention network (MFA-UNet). The preprocessing stage involves the application of gamma correction and contrast-limited adaptive histogram equalization to enhance image intensity and vessel contrast. The MFA-UNet incorporates the Multi-scale Fusion Self-Attention Module(MSAM) that adjusts multi-scale features and establishes global dependencies, enabling the network to better preserve microvascular structures. Furthermore, the multi-branch decoding module based on deep supervision (MBDM) replaces the original output layer to achieve targeted segmentation of macrovessels and microvessels. Additionally, a parallel attention mechanism is embedded into the decoder to better exploit multi-scale features in skip paths. Results: The proposed MFA-UNet yields competitive performance, with dice scores of 82.79/83.51/84.17/78.60/81.75/84.04 and accuracies of 95.71/96.4/96.71/96.81/96.32/97.10 on the DRIVE, STARE, CHASEDB1, HRF, IOSTAR and FIVES datasets, respectively. Discussion: It is expected to provide reliable segmentation results in clinical diagnosis.

Highly sensitive temperature and refractive index sensor based on no-core fiber cascaded with a balloon-shaped bent single-mode fiber.

A highly sensitive temperature and refractive index (RI) sensor based on no-core fiber (NCF) cascaded with a balloon-shaped bent single-mode fiber (BSBSF) is proposed and demonstrated. The NCF can excite higher-order modes which will be concentrated and transmitted into the BSBSF due to the characteristic of self-imaging effect. The BSBSF has an excellent temperature performance due to the high thermo-optical coefficient and thermal expansion coefficient of the polymer coating. The NCF and BSBSF are both conducive to the excitation of higher-order modes, which induces the sensitivity of the sensor with an efficiency improvement. The experimental results show that the maximum temperature sensitivity is -3.19n m/ ∘ C in the range of 22°C-83°C, which is the highest temperature sensitivity in the cascaded BSBSF structure to our best knowledge. In addition, the maximum RI sensitivity is 232.16 nm/RIU when the RI changes from 1.3234 to 1.3512. Compared with other cascaded BSBSF structures, this sensor has a higher temperature sensitivity and can be applicated in the prospects of food, biology, and environmental monitoring.