Genome-wide identification and expression analysis of the Eriobotrya japonica TIFY gene family reveals its functional diversity under abiotic stress conditions.

BACKGROUND: Plant-specific TIFY proteins are widely found in terrestrial plants and play important roles in plant adversity responses. Although the genome of loquat at the chromosome level has been published, studies on the TIFY family in loquat are lacking. Therefore, the EjTIFY gene family was bioinformatically analyzed by constructing a phylogenetic tree, chromosomal localization, gene structure, and adversity expression profiling in this study. RESULTS: Twenty-six EjTIFY genes were identified and categorized into four subfamilies (ZML, JAZ, PPD, and TIFY) based on their structural domains. Twenty-four EjTIFY genes were irregularly distributed on 11 of the 17 chromosomes, and the remaining two genes were distributed in fragments. We identified 15 covariate TIFY gene pairs in the loquat genome, 13 of which were involved in large-scale interchromosomal segmental duplication events, and two of which were involved in tandem duplication events. Many abiotic stress cis-elements were widely present in the promoter region. Analysis of the Ka/Ks ratio showed that the paralogous homologs of the EjTIFY family were mainly subjected to purifying selection. Analysis of the RNA-seq data revealed that a total of five differentially expressed genes (DEGs) were expressed in the shoots under gibberellin treatment, whereas only one gene was significantly differentially expressed in the leaves; under both low-temperature and high-temperature stresses, there were significantly differentially expressed genes, and the EjJAZ15 gene was significantly upregulated under both low- and high-temperature stress. RNA-seq and qRT-PCR expression analysis under salt stress conditions revealed that EjJAZ2, EjJAZ4, and EjJAZ9 responded to salt stress in loquat plants, which promoted resistance to salt stress through the JA pathway. The response model of the TIFY genes in the jasmonic acid pathway under salt stress in loquat was systematically summarized. CONCLUSIONS: These results provide a theoretical basis for exploring the characteristics and functions of additional EjTIFY genes in the future. This study also provides a theoretical basis for further research on breeding for salt stress resistance in loquat. RT-qPCR analysis revealed that the expression of one of the three EjTIFY genes increased and the expression of two decreased under salt stress conditions, suggesting that EjTIFY exhibited different expression patterns under salt stress conditions.

Translation, Cross-Cultural Adaptation, and Validation of the Traditional Chinese Version of the VISA-P Questionnaire.

Background: The Victorian Institute of Sport Assessment-Patella (VISA-P) questionnaire is a widely accepted instrument for measuring the severity of symptoms and pain in patients having sustained patellar tendinopathy. Purpose: To adapt the VISA-P questionnaire cross-culturally to a traditional Chinese version (VISA-P-Ch) and validate its psychometric properties. Study Design: Cohort study (diagnosis); Level of evidence, 3. Methods: The VISA-P questionnaire was adapted to a traditional Chinese version following international recommended guidelines, including translation, synthesis, back translation, revision by expert committee, pretesting, and validation. The psychometric properties were tested in 15 healthy controls and 15 participants with patellar tendinopathy. Face validity was judged by the authors and participants. Known-groups validity was tested by comparing the VISA-P-Ch scores between symptomatic and asymptomatic participants using an independent t test. Concurrent validity was determined by comparing the Blazina classification of the participants against VISA-P-Ch scores using the Spearman correlation coefficient. Test-retest reliability was assessed by calculating the intraclass correlation coefficient (ICC) following a 24- to 48-hour interval. Internal consistency was determined by the Cronbach alpha. Results: The expert committee and participants reported good face validity of the VISA-P-Ch. Significantly higher scores were found in the control group than in the patellar tendinopathy group (98.47 ± 3.04 vs 65 ± 11.9; P < .001). Concurrent validity showed a high correlation between VISA-P-Ch and the Blazina classification system (r = -0.899; P < .01). The test-retest reliability was excellent (ICC = 0.964). Internal consistency was found to be good for both the first and second assessments (Cronbach α = 0.834 and 0.851). Conclusion: The VISA-P-Ch was proven to be a reliable and valid questionnaire with similar psychometric properties as the original VISA-P.

Are children with IgA nephropathy different from adult patients?

BACKGROUND: Previously, several studies have indicated that pediatric IgA nephropathy (IgAN) might be different from adult IgAN, and treatment strategies might be also different between pediatric IgAN and adult IgAN. METHODS: We analyzed two prospective cohorts established by pediatric and adult nephrologists, respectively. A comprehensive analysis was performed investigating the difference in clinical and pathological characteristics, treatment, and prognosis between children and adults with IgAN. RESULTS: A total of 1015 children and 1911 adults with IgAN were eligible for analysis. More frequent gross hematuria (88% vs. 20%, p < 0.0001) and higher proteinuria (1.8 vs. 1.3 g/d, p < 0.0001) were seen in children compared to adults. In comparison, the estimated glomerular filtration rate (eGFR) was lower in adults (80.4 vs. 163 ml/min/1.73 m2, p < 0.0001). Hypertension was more prevalent in adult patients. Pathologically, a higher proportion of M1 was revealed (62% vs. 39%, p < 0.0001) in children than in adults. S1 (62% vs. 28%, p < 0.0001) and T1-2 (34% vs. 8%, p < 0.0001) were more frequent in adults. Adjusted by proteinuria, eGFR, and hypertension, children were more likely to be treated with glucocorticoids than adults (87% vs. 45%, p < 0.0001). After propensity score matching, in IgAN with proteinuria > 1 g/d, children treated with steroids were 1.87 (95% CI 1.16-3.02, p = 0.01) times more likely to reach complete remission of proteinuria compared with adults treated with steroids. CONCLUSIONS: Children present significantly differently from adults with IgAN in clinical and pathological manifestations and disease progression. Steroid response might be better in children.

Problematic Use of Internet Associates with Poor Quality of Life via Psychological Distress in Invididuals with ADHD.

Background: Problematic use of internet (PUI) may have negative impacts on psychological distress and quality of life (QoL). This situation might be more profound in people with attention-deficit/hyperactivity disorder (ADHD) due to poorer behavioral control and regulatory capacity. However, there is little evidence regarding mediated effects in the associations between PUI, psychological distress, and QoL in people with ADHD. Aims: To investigate mediating effects of psychological distress in the associations of problematic smartphone use (PSPU), problematic use of social media (PUSM), and problematic gaming (PG) with QoL in individuals with ADHD. Methods and Procedures: PUI behaviors of participants with ADHD (n = 99) were assessed using the Smartphone Application-Based Addiction Scale, Bergen Social Media Addiction Scale, and Internet Gaming Disorder-Short Form. Psychological distress was assessed using the Depression, Anxiety, Stress Scale and QoL using the Kid-KINDL. Outcomes and Results: Psychological distress mediated the associations between PUI and different domains of QoL, except for self-esteem QoL. There were also positively direct effects between PG and physical QoL, PUSM and friends' QoL, and PSPU and physical QoL. Conclusions and Implications: PUI may associate with poor QoL in people with ADHD via psychological distress. Programs on reducing PUI for people with ADHD are needed.

Cytosine base editing inhibits hepatitis B virus replication and reduces HBsAg expression in vitro and in vivo.

Chronic hepatitis B virus (HBV) infection remains a global health problem due to the lack of treatments that prevent viral rebound from HBV covalently closed circular (ccc)DNA. In addition, HBV DNA integrates in the human genome, serving as a source of hepatitis B surface antigen (HBsAg) expression, which impairs anti-HBV immune responses. Cytosine base editors (CBEs) enable precise conversion of a cytosine into a thymine within DNA. In this study, CBEs were used to introduce stop codons in HBV genes, HBs and Precore. Transfection with mRNA encoding a CBE and a combination of two guide RNAs led to robust cccDNA editing and sustained reduction of the viral markers in HBV-infected HepG2-NTCP cells and primary human hepatocytes. Furthermore, base editing efficiently reduced HBsAg expression from HBV sequences integrated within the genome of the PLC/PRF/5 and HepG2.2.15 cell lines. Finally, in the HBV minicircle mouse model, using lipid nanoparticulate delivery, we demonstrated antiviral efficacy of the base editing approach with a >3log10 reduction in serum HBV DNA and >2log10 reduction in HBsAg, and 4/5 mice showing HBsAg loss. Combined, these data indicate that base editing can introduce mutations in both cccDNA and integrated HBV DNA, abrogating HBV replication and silencing viral protein expression.

Suppression of Strawberry Anthracnose by Paenibacillus polymyxa TP3 In Situ and from a Distance.

Strawberry is a popular fruit with valuable nutrition and an attractive fragrance, but its production and propagation are limited by various diseases, including anthracnose and gray mold. For disease management, biological control measures are environmentally friendly and good alternatives to fungicides to avoid crop losses, reduce carbon emissions, and improve food safety. In this study, Paenibacillus polymyxa TP3, which originated from the strawberry phyllosphere, was shown to antagonize the anthracnose fungal pathogen Colletotrichum siamense and reduce leaf symptoms on strawberry plants. Several mass spectra corresponding to fusaricidin were detected in the confrontation assay of P. polymyxa TP3 and C. siamense by image mass spectrometry. The transcription of fusA and fusG in the fusaricidin biosynthesis gene cluster increased while P. polymyxa TP3 was cultured in the medium containing the culture filtrate of C. siamense, as detected by reverse-transcription polymerase chain reaction, indicating the involvement of fusaricidins in P. polymyxa TP3 antagonism against the anthracnose pathogen. Further disease control assays demonstrated the time frame and spatial mode of P. polymyxa TP3-induced systemic resistance of strawberry against C. siamense. The transcript level of the marker gene FaPDF1.2 of the jasmonic acid pathway increased in strawberry leaves after drenching treatment with P. polymyxa TP3, and the callose deposition was enhanced by further flg22 treatment. In addition, P. polymyxa TP3 treatments of the strawberry mother plants reduced C. siamense infection in the daughter plants, which would be a potent feature for the application of P. polymyxa TP3 in strawberry nurseries and fields to reduce the impact of diseases, especially anthracnose.

Long-Term Indomethacin Treatment in a Chinese Child with Gitelman Syndrome: Case Report and Literature Review on its Efficacy and Tolerance.

BACKGROUND Gitelman syndrome (GS) is a rare inherited autosomal recessive salt-losing renal tubulopathy. Early-onset GS is difficult to differentiate from Bartter syndrome (BS). It has been reported in some cases that cyclooxygenase (COX) inhibitors, which pharmacologically reduce prostaglandin E2(PGE2) synthesis, are helpful for GS patients, especially in children, but the long-term therapeutic effect has not yet been revealed. CASE REPORT A 4-year-old boy was first brought to our hospital for the chief concern of short stature and growth retardation. Biochemical tests demonstrated severe hypokalemia, hyponatremia, and hypochloremic metabolic alkalosis. The patient's serum magnesium was normal. He was diagnosed with BS and treated with potassium supplementation and indomethacin and achieved stable serum potassium levels and slow catch-up growth. At 11.8 years of age, the patient showed hypomagnesemia and a genetic test confirmed that he had GS with compound heterozygous mutations in the SLC12A3 gene. At the age of 14.8 years, when indomethacin had been taken for nearly 10 years, the boy reported having chronic stomachache, while his renal function remained normal. After proton pump inhibitor and acid inhibitor therapy, the patient's symptoms were ameliorated, and he continued to take a low dose of indomethacin (37.5 mg/d divided tid) with good tolerance. CONCLUSIONS Early-onset GS in childhood can be initially misdiagnosed as BS, and gene detection can confirm the final diagnosis. COX inhibitors, such as indomethacin, might be tolerated by pediatric patients, and long-term therapy can improve the hypokalemia and growth retardation without significant adverse effects.

[Therapeutic effect of mycophenolate mofetil or cyclophosphamide in children with Henoch-Schönlein purpura nephritis of different age groups]. / ä¸åŒå¹´é¾„æ®µè¿‡æ•æ€§ç´«ç™œæ€§è‚¾ç‚Žæ‚£å„¿æŽ¥å—éœ‰é šé ¸é ¯æˆ–çŽ¯ç£·é °èƒºæ²»ç–—çš„ç–—æ•ˆå·®å¼‚åˆ†æž.

OBJECTIVES: To investigate the difference in the therapeutic effect of mycophenolate mofetil (MMF) or cyclophosphamide (CTX) in children with Henoch-Schönlein purpura nephritis (HSPN) of different age groups. METHODS: A retrospective analysis was conducted on the clinical data of 135 children with HSPN who were treated with MMF or CTX in the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics, from October 2018 to October 2020. According to the immunosuppressant used, they were divided into two groups: MMF group and CTX group, and according to the age, each group was further divided into two subgroups: ≤12 years and >12 years, producing four groups, i.e, the ≤12 years MMF subgroup (n=30), the >12 years MMF subgroup (n=15), the ≤12 years CTX subgroup (n=71), and the >12 years CTX subgroup (n=19). All children were followed up for at least 12 months, and the above groups were compared in terms of clinical outcomes and the incidence rate of adverse reactions. RESULTS: There was no significant difference in the complete response rate between the MMF group and the CTX group after 3, 6, and 12 months of treatment (P>0.05). There were no significant difference in the complete response rate and the incidence rate of adverse reactions between the >12 years MMF subgroup and the ≤12 years MMF subgroup at 3, 6, and 12 months of treatment (P>0.05). The >12 years CTX subgroup had a significantly lower complete response rate than the ≤12 years CTX subgroup at 6 and 12 months of treatment (P<0.05). The >12 years CTX subgroup had a significantly higher incidence rate of adverse reactions than the >12 years MMF subgroup (P<0.05). CONCLUSIONS: The efficacy and adverse reactions of MMF are not associated with age, but the efficacy of CTX is affected by age, with a higher incidence rate of adverse reactions. CTX should be selected with caution for children with HSPN aged >12 years.

Psychometric properties of the Depression, Anxiety, and Stress Scale (DASS-21) among different Chinese populations: A cross-sectional and longitudinal analysis.

Given that there is limited evidence concerning the psychometric properties of DASS-21 when applied to primary school students, the present study undertook a comprehensive exploration of the psychometric evidence supporting the use of the DASS-21 within this demographic. The research comprised three studies. In Study 1, the basic psychometric properties of internal consistency and construct validity were examined. A total of 3138 primary school students from three provinces in mainland China participated. The internal reliability of the overall scale was 0.93, and for all the subscales, it was higher than 0.80. Construct validity was partially supported. Both exploratory and confirmatory factor analyses upheld the factorial validity of the original three-factor structure. While convergent validity was established, the results showed unsatisfactory discriminant validity. The bifactor model showed that DASS-21 raw scores predominantly indicated the general factor, evidenced by the high explained common variance and omega-hierarchical values. However, the contributions from the three specific factors were minimal, with their omega hierarchical values all below 0.15. In Study 2, a longitudinal design was adopted, tracking 1366 primary school students from Southwest China over a three-month interval. The results further confirmed that the DASS-21 exhibited scalar time-invariance. The latent mean analysis showed that there were no statistically significant differences in the latent means of depression, anxiety, and stress between Time 1 and Time 2. In Study 3, which included 364 college students and 483 enterprise workers, the results demonstrated that the DASS-21 had measurement invariance across different populations. The latent mean analysis further confirmed that, in terms of the latent mean of all three subscales, both college students and enterprise workers had significantly higher scores than primary school students. Overall, the findings indicated that the DASS-21 is a suitable tool for screening schoolchildren for general psychological distress, but it is not suitable for discerning distinct negative mood state disorders.

Lilium Gray Mold Suppression Conferred by the Host Antimicrobial Protein LsGRP1 Involves Main Pathogen-Targeted Manipulation of the Nonantimicrobial Region LsGRP1N.

Antimicrobial protein LsGRP1 protects Lilium from gray mold mainly caused by the destructive pathogen Botrytis elliptica; however, its nonantimicrobial region LsGRP1N conversely promotes spore germination of this fungus. By assaying the effects of LsGRP1N, LsGRP1, and the combination of LsGRP1N and the antimicrobial region LsGRP1C on fungal spore germination, hyphal growth, and Lilium gray mold development, LsGRP1N was found to improve the LsGRP1C sensitivity of B. elliptica and disease suppression by LsGRP1C. B. elliptica cell vitality assays indicated that LsGRP1N pretreatment uniquely enhanced the lethal efficiency of LsGRP1C compared to the control peptides. In addition, LsGRP1N-treated B. elliptica was demonstrated to lower infection-related gene expression and increase host-defense-eliciting activity, as indicated by reverse transcription quantitative polymerase chain reaction and histochemical-staining-based callose detection results, respectively. Therefore, LsGRP1N showed a novel mode of action for antimicrobial proteins by manipulating the main pathogen, which facilitated the development of target-specific and dormant microbe-eradicating antimicrobial agents.

Diversity and Biocontrol Potential of Endophytic Fungi and Bacteria Associated with Healthy Welsh Onion Leaves in Taiwan.

Foliar diseases caused by Stemphylium and Colletotrichum species are among the major biotic factors limiting Welsh onion production in Taiwan. Owing to concerns about the environment and the development of pathogen resistance to existing fungicides, biological control using endophytes is emerging as an eco-friendly alternative to chemical control. The aim of the present study was to isolate endophytes from healthy Welsh onion leaves and investigate their antagonistic potential against the major phytopathogenic fungi associated with Welsh onion plants in Taiwan. A total of 109 bacterial and 31 fungal strains were isolated from healthy Welsh onion leaves and assigned to 16 bacterial and nine fungal genera using morphological and molecular characterization based on DNA sequence data obtained from nuclear internal transcribed spacer (nrITS) (fungi) and 16S rRNA (bacteria). Evaluation of these endophytic isolates for biocontrol activity against leaf blight pathogens Colletotrichum spaethianum strain SX15-2 and Stemphylium vesicarium strain SX20-2 by dual culture assay and greenhouse experiments resulted in the identification of two bacterial isolates (GFB08 and LFB28) and two fungal isolates (GFF06 and GFF08) as promising antagonists to leaf blight pathogens. Among the four selected isolates, Bacillus strain GFB08 exhibited the highest disease control in the greenhouse study. Therefore, Bacillus strain GFB08 was further evaluated to understand the mechanism underlying its biocontrol efficacy. A phylogenetic analysis based on six genes identified Bacillus strain GFB08 as B. velezensis. The presence of antimicrobial peptide genes (baer, bamC, bmyB, dfnA, fenD, ituC, mlna, and srfAA) and the secretion of several cell wall degrading enzymes (CWDEs), including cellulase and protease, confirmed the antifungal nature of B. velezensis strain GFB08. Leaf blight disease suppression by preventive and curative assays indicated that B. velezensis strain GFB08 has preventive efficacy on C. spaethianum strain SX15-2 and both preventive and curative efficacy on S. vesicarium strain SX20-2. Overall, the current study revealed that healthy Welsh onion leaves harbour diverse bacterial and fungal endophytes, among which the endophytic bacterial strain, B. velezensis strain GFB08, could potentially be used as a biocontrol agent to manage the leaf blight diseases of Welsh onion in Taiwan.

Obesity as a clinical predictor for severe manifestation of dengue: a systematic review and meta-analysis.

BACKGROUND: Severe dengue often leads to poor clinical outcomes and high mortality; as a result, it is of vital importance to find prognostic factors associated with the severe form of dengue. Obesity is known to deteriorate many infectious diseases due to impaired immune responses. Several studies have suggested that obese patients with dengue infection tend to have more severe manifestations with poorer prognosis. However, a firm conclusion could not be drawn due to the varied results of these studies. Here, we aimed to conduct a systematic review and meta-analysis to investigate the association between obesity and dengue severity. METHODS: A literature search for relevant studies was conducted in PubMed, Embase, Ovid Medline and Cochrane from inception to September 9, 2022. The two main keywords were "dengue" and "obesity". Mantel-Haenszel method and random effects model was used to analyze the pooled odds ratio with 95% confidence intervals. RESULTS: A total of 15 article involving a total of 6,508 patients were included in the meta-analysis. Included patients in most studies were hospitalized pediatric patients. Only one study included adulthood data. Three cohort studies, four case-control studies, and one cross-sectional studies found a significant association between obesity and dengue severity. In contrast, three cohort studies, three case-control studies, and one cross-sectional study reported no significant relationship between obesity and dengue severity. Our analysis results showed that patient with obesity is 50% (OR = 1.50; 95%CI: 1.15-1.97) more likely to develop severe manifestation of dengue. CONCLUSION: This meta-analysis revealed that overweight could be a clinical predictor for severe disease for pediatric patients with dengue infection.

Two Cases of Acute Focal Bacterial Nephritis With Central Nervous System Manifestations in Children and Literature Review.

Acute focal bacterial nephritis (AFBN) is a kidney disease characterized by a localized bacterial infection that manifests as an inflammatory mass. Most children with AFBN have nonspecific symptoms including fever, vomiting, and abdominal discomfort, and some develop neurological symptoms such as meningeal irritation, unconsciousness, and seizures as their condition worsens. This was 2 cases of AFBN with central nervous system manifestations in children, and we analyzed its possible mechanisms of the clinical and radiographic features. We experience 2 very unusual cases of AFBN which were linked to central nervous system abnormalities. A 6-year-old boy with AFBN and clinically moderate ncephalitis/encephalopathy with a reversible splenial lesion (MERS) presented with neurological symptoms, including unconsciousness and convulsions. The second case involved an 8-year-old child with AFBN-associated acute encephalopathy who exhibited neurological symptoms, including unconsciousness. According to previous research, AFBN is linked to central nervous system impairment. As a result, when a clinician meets a patient with an inexplicable fever caused by a neurological condition, he should pay attention to this diagnosis of AFBN and follow it in the abdominal graph.

Exploring mediational roles for self-stigma in associations between types of problematic use of internet and psychological distress in youth with ADHD.

BACKGROUND: Several studies have linked the problematic use of the Internet (PUI) to psychological distress. Youth with attention deficit hyperactivity disorder (ADHD) are considered a particular disadvantaged population with a high risk of developing PUI, psychological distress, and self-stigma. Nonetheless, the interrelationships of PUI, self-stigma, and psychological distress in adolescents with ADHD are not well understood. AIMS: This study investigated whether self-stigma mediates relationships between different forms of PUI, such as problematic gaming (PG), problematic social media use (PSMU), problematic smartphone use (PSPU), and psychological distress (i.e., depression, anxiety, and stress), in children with ADHD. METHODS AND PROCEDURES: We recruited 100 youth with ADHD (mean age=10.80 [SD=3.07] years; 84 boys) from psychiatric outpatient clinics in Taiwan. All participants were assessed for PUI (via Internet Gaming Disorder-Short Form for PG, Bergan Social Medica Addiction Scale for PSMU, and Smartphone Application-Based Addiction Scale for PSPU), self-stigma (via Self-Stigma Short-Scale), and psychological distress (via Depression, Anxiety, Stress Scale). OUTCOMES AND RESULTS: The results of path and bootstrapping analyses indicated that self-stigma mediated the associations between PSMU and PSPU, but not PG, and depression, anxiety, and stress. CONCLUSIONS AND IMPLICATIONS: This study expands the extant literature by revealing that self-stigma mediates the association between specific forms of PUI and psychological distress in adolescents with ADHD. Interventions aimed at reducing self-stigma and PUI, particularly PSMU and PSPU, may help decrease psychological distress among adolescents with ADHD.

Growth hormone treatment in pre-pubertal short Chinese children with chronic kidney disease prior to transplantation.

BACKGROUND: The effect of recombinant human GH (rhGH) in Chinese children with chronic kidney disease (CKD) is unclear. METHODS: This was a 52-week, multicenter, randomized, open-label, negative-controlled phase 3 study. Prepubertal subjects were randomized 1:1 to either daily subcutaneous injections of rhGH 0.05 mg/kg/day or no treatment for 52 weeks. RESULTS: A total of 68 subjects with a mean age of 7.8 ± 3.27 years were enrolled. At week 52, the height standard deviation score (HT-SDS) in the treated group increased by 0.75 ± 0.58, which was significantly higher compared with 0.17 ± 0.47 in the untreated group (least squares mean 0.58, 95% confidence interval, 0.32-0.84; P < 0.001). At week 52, significant improvements were observed in other growth parameters (height velocity [P < 0.001]), insulin-like growth factor 1 (IGF-1) SDS [P < 0.001], IFG-1/insulin-like growth factor binding protein-3 molar ratio [P < 0.001], and height [P < 0.001]) compared with the untreated control. Seven patients reported treatment-related adverse events (TRAEs) and most TRAEs were mild in severity. Most subjects recovered without further intervention. CONCLUSIONS: Daily rhGH for 52 weeks in children with CKD-induced growth retardation significantly improved HT-SDS and other growth parameters without compromising safety. IMPACT: The efficacy and safety of growth hormone (GH) therapy in Chinese children with chronic kidney disease (CKD) are unclear. This study found that giving short stature Chinese children with CKD daily recombinant human growth hormone (rhGH) for 52 weeks improved growth parameters without compromising safety. This study's information can give physicians the confidence to treat these patients in their clinical practice.

Two cases of fetal hyperechogenic kidneys who had HNF1-ß gene variation.

We report two cases of HNF1-ß gene variation diagnosed in infancy, in whom fetal ultrasonography revealed enhanced echogenicity and multiple cysts in the renal parenchyma of both patients. They were initially diagnosed as autosomal recessive polycystic kidney disease. Gene testing showed a variation of HNF1-ß gene, one showed chromosome 17q12 deletion including HNF1-ß, the other was a de novo nonsense mutation in the HNF1-ß gene. The two children showed different renal function states. Extrarenal phenotypes also vary widely according to HNF1-ß gene variation including early-onset diabetes, autism spectrum, cognitive disorders, liver function abnormalities, and genital malformations, etc. We emphasize the importance of performing gene detection in order to make an accurate diagnosis, especially in those with fetal hyperechogenic kidneys, and so as to carry out reasonable multidisciplinary management. Early intervention for diabetes and neurodevelopmental disorders are especially important.

Difficulties in Managing Children's Learning among Caregivers of Children with Attention-Deficit/Hyperactivity Disorder during the COVID-19 Pandemic in Taiwan: Association with Worsened Behavioral and Emotional Symptoms.

School closures during the COVID-19 pandemic have interfered with children's learning. The aim of this study was to investigate the difficulties in managing children's learning at home and attending afterschool learning programs and their related factors among caregivers of children with attention-deficit/hyperactivity disorder (ADHD) during the COVID-19 pandemic. In total, 252 caregivers of children with ADHD completed a questionnaire collecting difficulties in managing children's learning, parenting styles, children's worsened symptoms of ADHD, oppositional defiant disorder (ODD) and emotion, and increased Internet use. Multivariate logistic regression models were used to examine the factors related to caregiver difficulties in managing children's learning and children's worsened ADHD, ODD, and emotional symptoms. In total, 85.3% of the caregivers had difficulty in asking their children to learn at home; 28.2% had difficulty in taking children to afterschool learning programs. Children's worsened anger was significantly associated with higher caregiver difficulty in asking children to learn at home, whereas parental overprotection was significantly associated with lower caregiver difficulty in asking children to learn at home. Worsened hyperactivity and opposition were significantly associated with higher caregiver difficulty in taking children to attend afterschool learning programs. Interventions for enhancing caregivers' skills to manage children's learning and children's behavioral and emotional symptoms should take the related factors found in this study into consideration.

Age-Related Modifications of Muscle Synergies and Their Temporal Activations for Overground Walking.

Healthy ageing modifies neuromuscular control of human overground walking. Previous studies found that ageing changes gait biomechanics, but whether there is concurrent ageing-related modulation of neuromuscular control remains unclear. We analyzed gait kinematics and electromyographic signals (EMGs; 14 lower-limb and trunk muscles) collected at three speeds during overground walking in 11 healthy young adults (mean age of 23.4 years) and 11 healthy elderlies (67.2 years). Neuromuscular control was characterized by extracting muscle synergies from EMGs and the synergies of both groups were k -means-clustered. The synergies of the two groups were grossly similar, but we observed numerous cluster- and muscle-specific differences between the age groups. At the population level, some hip-motion-related synergy clusters were more frequently identified in elderlies while others, more frequent in young adults. Such differences in synergy prevalence between the age groups are consistent with the finding that elderlies had a larger hip flexion range. For the synergies shared between both groups, the elderlies had higher inter-subject variability of the temporal activations than young adults. To further explore what synergy characteristics may be related to this inter-subject variability, we found that the inter-subject variance of temporal activations correlated negatively with the sparseness of the synergies in elderlies but not young adults during slow walking. Overall, our results suggest that as humans age, not only are the muscle synergies for walking fine-tuned in structure, but their temporal activation patterns are also more heterogeneous across individuals, possibly reflecting individual differences in prior sensorimotor experience or ageing-related changes in limb neuro-musculoskeletal properties.

Clinical effect of different prednisone regimens in the treatment of children with primary nephrotic syndrome and risk factors for recurrence. / ä¸åŒæ–¹æ¡ˆæ³¼å°¼æ¾æ²»ç–—å„¿ç«¥åŽŸå‘æ€§è‚¾ç— ç»¼åˆå¾çš„ç–—æ•ˆåŠå¤å‘å±é™©å› ç´ åˆ†æž.

OBJECTIVES: To study the clinical effect of full-dose prednisone for 4 or 6 weeks in the treatment of children with primary nephrotic syndrome and its effect on recurrence. METHODS: A prospective non-randomized controlled clinical trial was performed on 89 children who were hospitalized and diagnosed with incipient primary nephrotic syndrome from December 2017 to May 2019. The children were given prednisone of 2 mg/(kg·day) (maximum 60 mg) for 4 weeks (4-week group) or 6 weeks (6-week group), followed by 2 mg/(kg·day) (maximum 60 mg) every other day for 4 weeks and then a gradual reduction in dose until drug withdrawal. The children were regularly followed up for 1 year. The two groups were compared in terms of the indices including remission maintenance time and recurrence rate. A Cox regression analysis was used to assess the risk factors for recurrence. RESULTS: Within 3 months after prednisone treatment, the 4-week group had a significantly higher recurrence rate than the 6-week group (P<0.05). After 1-year of follow-up, there was no significant difference between the two groups in the recurrence rate, remission maintenance time, and recurrence frequency (P>0.05). The risk of recurrence increased in children with an onset age of ≥6 years or increased 24-hour urinary protein (P<0.05). CONCLUSIONS: For the treatment of incipient primary nephrotic syndrome, full-dose prednisone regimen extended from 4 weeks to 6 weeks can reduce recurrence within 3 months. The children with an onset age of ≥6 years or a high level of urinary protein should be taken seriously in clinical practice, and full-dose prednisone treatment for 6 weeks is recommended to reduce the risk of recurrence.

[Clinical assessment of moderate-dose glucocorticoid in the treatment of recurrence of primary nephrotic syndrome in children: a prospective randomized controlled trial]. / ä¸­ç­‰å‰‚é‡ç³–çš®è´¨æ¿€ç´ æ²»ç–—æ¿€ç´ æ•æ„Ÿè‚¾ç— ç»¼åˆå¾å¤å‘æ‚£å„¿çš„å‰çž»æ€§éšæœºå¯¹ç §ç ”ç©¶.

OBJECTIVES: To study the clinical effect and adverse drug reactions of different doses of glucocorticoid (GC) in the treatment of children with recurrence of steroid-sensitive nephrotic syndrome (SSNS). METHODS: A total of 67 children who were hospitalized and diagnosed with SSNS recurrence in the Department of Nephrology, Children's Hospital, Capital Institute of Pediatrics, from November 2017 to December 2019 were enrolled. They were randomly divided into a moderate-dose GC group (32 children) and a full-dose GC group (35 children). The two groups were compared in terms of urinary protein clearance, recurrence rate within 6 months, and incidence rate of GC-associated adverse reactions. RESULTS: There was no significant difference in the urinary protein clearance rate between the moderate-dose GC and full-dose GC groups (91% vs 94%, P>0.05). There was also no significant difference in the recurrence rate within 6 months between the two groups (41% vs 36%, P>0.05). At 6 months of follow-up, compared with the full-dose GC group, the moderate-dose GC group had a significantly lower cumulative dose of prednisone [(87±18) mg/kg vs (98±16) mg/kg, P=0.039] and a significantly lower proportion of children with an abnormal increase in body weight (6% vs 33%, P=0.045). The logistic regression analysis showed that prednisone dose ≥10 mg/alternate day at enrollment was a risk factor for recurrence within 6 months in children with SSNS (P=0.018). CONCLUSIONS: For children with SSNS recurrence, moderate-dose GC has similar effects to full-dose GC in the remission induction rate and the recurrence rate within 6 months, with a lower cumulative dose and fewer GC-associated adverse reactions within 6 months than full-dose GC.