BACKGROUND Juvenile arthritis damage index(JADI)is a tool that measures the degree of aggressiveness of the juvenile idiopathic arthritis(JIA)course and assesses articular[JADI-articular damage(JADI-A)]and extraarticu...BACKGROUND Juvenile arthritis damage index(JADI)is a tool that measures the degree of aggressiveness of the juvenile idiopathic arthritis(JIA)course and assesses articular[JADI-articular damage(JADI-A)]and extraarticular[JADI-extraarticular damage(JADI-E)]damage.While aggressive JIA often requires early bio-logic disease-modified antirheumatic drugs(bDMARDs),the utility of JADI as a predictor of treatment response remains underexplored.AIM To evaluate the potential of JADI as a predictor of bDMARD treatment response in JIA patients.METHODS This prospective study included 112 highly active non-systemic JIA biologic-naïve patients with a mean age of 12.2±4.6 years and a median disease duration of 2.5(interquartile range:1-5)years.Their clinical and radiological assessment,juvenile arthritis disease activity score 71,JADI-A,and JADI-E,were evaluated twice:Before the biologic initiation(baseline)and 12 months after(end of study).At baseline,50%had any damage,with 43%with articular damage and 23%with extraarticular damage.RESULTS During the study,JADI-A/JADI-E improved(33.9%/9.8%),worsened(8.9%/5.4%),or remained unchanged(57.1%/84.8%).Patients with baseline damage had higher markers of JIA activity:Polyarticular course,earlier onset age,ANA-positivity,and more active joints.Patients without initial structural damage(JADI“-”)were more likely(odds ratio=3.8,95%confidence interval:1.6-9.0,P<0.004)to achieve a low degree of activity or remission(46.2%),while on biological therapy,their scores were comparable to JADI-positive(18.3%).Pre-biological joint damage according to the JADI-A index(P=0.003),wrist(P=0.035),elbow(P=0.027),cervical spine limitation of motion(P=0.051),and erosions confirmed by magnetic resonance imaging(P=0.002),were associated with poor response to biological treatment and follow-up JIA activity.CONCLUSION Baseline structural damage in JIA is associated with diminished bDMARDs efficacy,increased disability,and shorter remission duration.JADI enhances conventional clinical risk stratification by facilitating timely initiation of bDMARDs,adherence to treat-to-target strategy and tailored patient care.展开更多
The compelling case report by Xie et al,published in a renowned medical journal,is an excellent example of meticulous clinical evaluation,comprehensive labo-ratory testing,advanced imaging,and genetic analysis.The aut...The compelling case report by Xie et al,published in a renowned medical journal,is an excellent example of meticulous clinical evaluation,comprehensive labo-ratory testing,advanced imaging,and genetic analysis.The authors identified novel compound heterozygous mutations in the hemojuvelin gene of a patient diagnosed with juvenile hemochromatosis.They suggested that long-term,strategic phlebotomy might offer a novel therapeutic strategy for severe juvenile hemochromatosis,challenging the traditional treatment paradigms.展开更多
BACKGROUND Chronic idiopathic uveitis(CIU)and juvenile idiopathic arthritis-associated uveitis(U-JIA)are both vision-threatening conditions that share similar autoimmune mechanisms,but treatment approaches differ sign...BACKGROUND Chronic idiopathic uveitis(CIU)and juvenile idiopathic arthritis-associated uveitis(U-JIA)are both vision-threatening conditions that share similar autoimmune mechanisms,but treatment approaches differ significantly.In managing U-JIA,various treatment options are employed,including biological and non-biological disease-modifying anti-rheumatic drugs.These drugs are effective in clinical trials.Given the lack of established diagnostic and treatment guidelines as well as the limited number of therapeutic options available,patients with CIU frequently do not receive optimal and timely immunosuppression.This study highlighted the necessity for additional research to develop novel diag-nostic techniques,targeted therapies,and enhanced treatment outcomes for young individuals with CIU.AIM To compare the characteristics and outcomes of U-JIA and CIU.METHODS A retrospective cohort study analyzed data from 110 pediatric patients(under 18 years old)with U-JIA and 40 pediatric patients with CIU.Data was collected between 2012 and 2023.The study focused on demographic,clinical,treatment,and outcome variables.RESULTS The median onset age of arthritis was 6.4 years(2.7 years;9.3 years).In 28.2%of cases uveitis preceded the onset of arthritis.In 17.3%of cases it occurred simultan-eously.In 53.6%of cases it followed arthritis.Both groups had similar onset ages,antinuclear antibodies/human leukocyte antigen positivity rates,and ESR levels,with a slight predominance of females(60.9%vs 42.5%,P=0.062),and higher C-reactive protein levels in the U-JIA group.Anterior uveitis was more prevalent in patients with U-JIA(P=0.023),although the frequency of symptomatic,unilateral,and complicated forms did not differ significantly.The use of methotrexate(83.8%vs 96.4%)and biologics(64.7%vs 82.1%)was comparable,as was the rate of remission on methotrexate treatment(70.9%vs 56.5%)and biological therapy(77.8%vs 95%),but a immunosuppressive treatment delay in CIU observed.Patients with CIU were less likely to receive methotrexate[hazard ratio(HR)=0.48,P=0.005]or biological treatment(HR=0.42,P=0.004),but they were more likely to achieve remission with methotrexate(HR=3.70,P=0.001).CONCLUSION Treatment of uveitis is often limited to topical measures,which can delay systemic therapy and affect the outcome.Methotrexate and biological agents effectively manage eye inflammation.It is essential to develop standardized protocols for the diagnosis and management of uveitis,and collaboration between rheumatologists and ophthal-mologists is needed to achieve optimal outcomes in the treatment of CIU.展开更多
BACKGROUND Children with juvenile idiopathic arthritis(JIA)and inflammatory bowel disease(IBD)face an elevated risk of severe infection owing to their diseases and the immunosuppressive treatment for disease control.A...BACKGROUND Children with juvenile idiopathic arthritis(JIA)and inflammatory bowel disease(IBD)face an elevated risk of severe infection owing to their diseases and the immunosuppressive treatment for disease control.AIM To compare scheduled vaccination coverage and the levels of post-vaccine antibodies against measles,mumps,rubella(MMR)and hepatitis B in pediatric patients with IBD and JIA.METHODS A comparative cohort study included 97 patients with IBD and 170 patients with JIA.Vaccination history was obtained from medical records,while post-vaccination immunity was assessed prospectively by measuring specific IgG antibody titers using enzyme-linked immunosorbent assays(Vector-Best JSC,Russia;IBL International,Germany)during routine visits between January 2022 and April 2023.RESULTS A complete two-dose MMR course had been administered to 66.3%of IBD patients and 55.9%of JIA patients(P=0.121).By contrast,the three-dose hepatitis B schedule was completed in 74.2%of IBD and 100%of JIA patients(P<0.001).Protective level of anti-vaccine antibodies against measles(47.7%vs 57.7%;P=0.168);mumps(75.3%vs 80.0%;P=0.366);rubella(74.4%vs 98.2%;P<0.0001);and hepatitis B(44.8%vs 50.0%;P=0.514)were detected in IBD and JIA patients,respectively.CONCLUSION Patients with IBD and JIA demonstrated different vaccination coverage patterns and levels of anti-vaccine antibodies.Routine baseline serology and timely booster vaccination should be implemented for all pediatric patients receiving chronic immunosuppression.展开更多
For songbirds,the post-fledging period is critical for development and survival,as young must learn to get food,practice flying,and identify predators to become independent from their parents and disperse or migrate.O...For songbirds,the post-fledging period is critical for development and survival,as young must learn to get food,practice flying,and identify predators to become independent from their parents and disperse or migrate.Obligate avian brood parasites,like cuckoos and cowbirds,lay eggs in nests of other bird species that provide parental care until the parasite’s young become independent.The information on the post-fledging period in songbirds is limited due to the difficulty in following and observing fledglings and it is even scarcer for brood parasites.We studied the behavior,survival,and age of independence of Shiny Cowbird(Molothrus bonariensis)fledglings raised by 2 hosts that differ markedly in body size:the House Wren(Troglodytes aedon)and the Chalk-Browed Mockingbird(Mimus saturninus).We radio-tracked individuals from a few days before they left the nest until they disappeared from the natal territory.We found:(1)chicks left the nest when they were 11-15 days of age and the age of fledgling did not differ between the 2 host species,(2)after leaving the nest,they spent most of the time perched in a hidden place without begging but as they grew,the proportion of time perched without begging decreased and begging in hidden and exposed places increased,(3)we observed approaches between adult parasitic females and young in host territories,(4)young abandoned the host territory and joined conspecific flocks when they were 35-39 days of age,and(5)the estimated post-fledging survival was between 12.5%and 20.8%.展开更多
Background:A germicide like Izal is chemical agent used for reducing and preventing microbial loads in systems for treating water as well as other industries like agriculture and aquaculture.The environmental impact o...Background:A germicide like Izal is chemical agent used for reducing and preventing microbial loads in systems for treating water as well as other industries like agriculture and aquaculture.The environmental impact of germicide is often more than the target intended by those who use them.The purpose of the current study was to ascertain the toxicity levels and behavioral alterations of Juvenile African catfish,Clarias gariepinus,subjected to Izal exposure.Methods:The stock solution was created from the 100 mL by mixing 1 mL of the Izal with 99 mL of water.After that,different nominal concentrations of Izal were prepared using the stock solution by diluting the toxin with tap water in determined amounts(10 L).The fish were exposed to acute concentrations of 1.0,1.5,2.0,2.5 and 3.0 mg/L of Izal had a control that served as a reference point.Results:The lethal concentration of Izal(24 h LC50)was found to be 2.4136 mg/L(95%confidence interval=2.1707–2.6838)for test fish under a semi-static system showing that Izal is moderately poisonous to the species in a dose-dependent manner and time(duration).The highest mortality was recorded in the highest concentration(3.0 mg/L)of Izal while the least mortality was observed in the 1.5 mg/L group.Izal-exposed fish had aberrant behavior,including hyperactivity,gasping of air,respiratory distress,attempts to jump out of the water,erratic swimming,refusal to feed,lethargic movement,and slow response to feed,loss of balance and depigmentation.The severity of the abnormal behavior decreased after 24 h,but remained mildly to moderately high at 2.0 and 2.5 mg/L of Izal.It was found that the death rate increased with Izal concentration and duration.Conclusion:The present study revealed that Izal was moderately toxic to Claris gariepinus juveniles and should be used prudently in the environment to guard against toxicological effect on non-target organisms such as fish.For this reason,the use of the germicides should be with caution especially near water bodies.展开更多
BACKGROUND Juvenile polyps(JPs)are non-neoplastic polyps.In adults,JPs present with hematochezia in only approximately half the patients and are often found incidentally during endoscopic screening.JPs have no mucosal...BACKGROUND Juvenile polyps(JPs)are non-neoplastic polyps.In adults,JPs present with hematochezia in only approximately half the patients and are often found incidentally during endoscopic screening.JPs have no mucosal fascia at the tip,and spontaneous shedding and massive gastrointestinal hemorrhage may occur.Thus,the JP bleeding detected in this case by extravascular contrast leakage on computed tomography scans and treated with endoscopic clipping is rare.CASE SUMMARY A previously healthy 31-year-old male patient presented with a 2-day history of bloody stools.Upon hospital arrival,rectal examination revealed fresh blood,and abdominal computed tomography scans showed extravascular contrast leakage from the lower rectum’s left-side wall.His blood pressure was slightly low at 104/62 mmHg.However,his pulse rate(69 bpm)and oxygen level(99%on room air)were within normal limits.Emergency endoscopy revealed a pedunculated lesion in the rectum covered by a non-neoplastic mucosal epithelium.No neoplastic lesions were observed at the tip of the polyp;however,pulsatile bleeding was detected at the distal end.We performed endoscopic hemostasis by clipping the stem and then performed a polypectomy above the stem to examine the lesion tissue.Histopathological evaluation revealed a cystically dilated gland without neoplastic lesions.A subsequent total colonoscopy revealed two JPs with characteristic edematous,smooth,and reddish surfaces close to the hemorrhagic lesion.Subsequent histopathological evaluation indicated findings characteristic of JP,such as severe inflammatory cell infiltration of the stroma and cystic dilatation of the glandular ducts.CONCLUSION There are no reports of adult JPs presenting with contrast extravasation where endoscopic hemostasis was successful,as in this case.展开更多
The insect fat body is comparable to the liver and adipose tissue in vertebrates,and plays a pivotal role in energy metabolism,nutrient storage,and reproduction.During metamorphosis,the fat body is disassembled via pr...The insect fat body is comparable to the liver and adipose tissue in vertebrates,and plays a pivotal role in energy metabolism,nutrient storage,and reproduction.During metamorphosis,the fat body is disassembled via programmed cell death and cell dissociation.After adult eclosion,the fat body is reconstructed either by repopulation from the remaining juvenile fat body cells or by differentiation from adult progenitor cells.This reconstruction is a prerequisite for initiating the extensive synthesis of vitellogenin(Vg),which is necessary for the maturation of eggs.Despite its significance,the underlying mechanisms of this reconstruction remain inadequately understood.Transcriptome analysis of the fat bodies from migratory locusts at 0-5 days post adult emergence revealed 79 genes associated with chromatin remodeling.Weighted gene co-expression network analysis indicated a positive correlation between chromatin remodeling and fat body reconstitution.Protein-protein interaction analysis revealed that brahma,which encodes the catalytic subunit of the SWI/SNF chromatin remodeling complex,is crucial for post-adult-eclosion fat body development.qRT-PCR analysis demonstrated that the levels of brahma mRNA in the fat body are progressively increased during the previtellogenic stage,then reach the peak and remain elevated in the vitellogenic phase.Furthermore,brahma is expressed in response to gonadotropic juvenile hormone(JH).Knockdown of brahma led to a marked reduction in Vg expression within the fat body,along with arrested ovarian growth.These findings shed light on the involvement of brahmamediated chromatin remodeling in JH-stimulated fat body reconstruction and reproduction of adult female locusts.展开更多
The high Ba-Sr rocks can provide significant clues about the evolution of the continent lithosphere,but their petrogenesis remains controversial.Identifying the Late Cretaceous high Ba-Sr granodiorites in the SE Lhasa...The high Ba-Sr rocks can provide significant clues about the evolution of the continent lithosphere,but their petrogenesis remains controversial.Identifying the Late Cretaceous high Ba-Sr granodiorites in the SE Lhasa Block could potentially provide valuable insights into the continent evolution of the Qinghai-Tibet Plateau.Zircon U-Pb ages suggest that the granodiorites were emplaced at 87.32±0.43 Ma.Geochemically,the high Ba-Sr granodiorites are characterized by elevated K_(2)O+Na_(2)O contents(8.18-8.73 wt%)and K_(2)O/Na_(2)O ratios(0.99-1.25,mostly>1),and belong to high-K calc-alkaline to shoshonitic series.The Yonglaga granodiorites show notably high Sr(653-783 ppm)and Ba(1346-1531 ppm)contents,plus high Sr/Y(30.92-38.18)and(La/Yb)_(N)(27.7-34.7)ratios,but low Y(20.0-22.8 ppm)and Yb(1.92-2.19 ppm)contents with absence of negative Eu anomalies(δEu=0.83-0.88),all similar to typical high Ba-Sr granitoids.The variable zirconεHf(t)values of-4.58 to+12.97,elevated initial^(87)Sr/^(86)Sr isotopic ratios of 0.707254 to 0.707322 and lowεNd(t)values of-2.8 to-3.6 with decoupling from the Hf system suggest that a metasomatized mantle source included significant recycled ancient materials.The occurrence of such high Ba-Sr intrusions indicates previous contributions of metasomatized mantle-derived juvenile material to the continents,which imply the growth of continental crust during the Late Cretaceous in the SE Lhasa.Together with regional data,we infer that the underplated mafic magma provides a significant amount of heat,which leads to partial melting of the juvenile crust.The melting of the metasomatized mantle could produce a juvenile mafic lower crust,from which the high Ba-Sr granitoids were derived from reworking of previous mafic crust during the Late Cretaceous(ca.100-80 Ma)in the SE Lhasa.展开更多
AIM:To characterize a Portuguese population of patients with juvenile idiopathic arthritis(JIA)and to evaluate possible associations between clinical factors and ocular involvement.METHODS:Patients diagnosed with JIA ...AIM:To characterize a Portuguese population of patients with juvenile idiopathic arthritis(JIA)and to evaluate possible associations between clinical factors and ocular involvement.METHODS:Patients diagnosed with JIA in the previous 20y in Hospital Garcia de Orta were included.Data were assessed from Reuma.pt database.Associations between demographic(age and sex),clinical(articular involvement,extra-articular manifestations,biological therapy),laboratory data[anti-nuclear antibodies,anti-cyclic citrullinated peptide(CCP)antibodies,rheumatoid factor,human leukocyte antigen B27(HLA-B27),C-reactive protein,erythrocyte sedimentation rate]and ocular involvement were assessed.Statistical analysis was performed using Chi-square for categorical variables and Mann-Whitney test for continuous variables.RESULTS:Totally 91 patients were included,11(12%)with previous episodes of uveitis.There was a statistically significant preponderance of early age at JIA diagnosis(mean 4.73 vs 9.58y,P=0.008),antinuclear antibodies positivity(P=0.01),and oligoarticular subtype(P=0.04)in the Juvenile idiopathic arthritis-associated uveitis(JIA-U)group.Ocular complications occurred in 36.4%of patients(n=4):cataracts(n=2),band keratopathy(n=1)and posterior synechiae(n=1).The occurrence of complications was corelated with a shorter period between JIA diagnosis and the first JIA-U episode(mean 0.67 vs 4.88y,P=0.012)but not with age at JIA diagnosis or articular involvement.There was erythrocyte sedimentation rate elevation in the 12mo preceding uveitis(mean 40.5 mm/h,range 13-83).CONCLUSION:The occurrence of JIA-U shortly after JIA diagnosis is shown to be a potential risk factor for ocular complications.展开更多
In this manuscript, we present a case report of a child with 16-year-old previously healthy Hispanic male who presented for progressive proximal muscle weakness, rash, and dysphagia. He was admitted to the acute care ...In this manuscript, we present a case report of a child with 16-year-old previously healthy Hispanic male who presented for progressive proximal muscle weakness, rash, and dysphagia. He was admitted to the acute care floor and diagnosed with juvenile dermatomyositis and found to be positive for anti-Mi-2 alpha, anti-Mi-2 beta, and anti-MDA-5 antibodies. He gradually improved with a combination of steroid, immunomodulatory treatment, and physical therapy. This case outlines the clinical course of a patient with this rare disorder as well as the importance of understanding the role of associated antibodies to manage potential long-term sequelae.展开更多
BACKGROUND Prevalence of the main rheumatic diseases in the Republic of Sakha(Yakutia)[RS(Y)],one of the regions of the Russian Federation,differs from the other regions of the Russian Federation due to its ethnic and...BACKGROUND Prevalence of the main rheumatic diseases in the Republic of Sakha(Yakutia)[RS(Y)],one of the regions of the Russian Federation,differs from the other regions of the Russian Federation due to its ethnic and geographic features.Knowledge regarding the prevalence and structure of juvenile idiopathic arthritis(JIA)allows us to shape the work of the pediatric rheumatology service in the region correctly,and optimize the healthcare system and the need for medica-tions.AIM To describe the epidemiological,demographic,clinical,and laboratory characteristics of children with JIA in the RS(Y)and evaluate the main outcomes.METHODS This retrospective cohort study assessed all the data from the medical histories of the patients(n=225)diagnosed with JIA(2016-2023)in the Cardiorheumatology Department of the M.E.Nikolaev National Center of Medicine.Pearson'sχ²test,Fisher's exact test,Mann–Whitney and Kruskal-Wallis tests were used for statistical analyses.RESULTS The ethnic prevalence of JIA is higher in Sakha than in Russian children at 110.1 per 100000 children and 69.4 per 100000 children,respectively.The prevalence of JIA among boys and girls in Sakha was similar,unlike in Russians,where the number of girls predominated.The JIA categories were as follows:(1)Systemic arthritis:3.5%;(2)Oligoarthritis(persistent and extended):33.8%;(3)Rheumatoid factor(RF)(+)polyarthritis:0.9%;(4)RF(-)polyarthritis:14.7%;(5)Enthesitis-related arthritis(ERA):44%;and(6)Psoriatic arthritis:3.1%.Prevalence of the ERA category was 4.4 times higher in Sakha children,but the prevalence of systemic arthritis was 2.9 times lower compared to Russians(P=0.0005).The frequency of uveitis was 10.2%,and the frequency of human leukocyte antigen(HLA)B27 was 39.6%in JIA children.Biologic treatment was received by 40.4%of JIA children and 45.3%achieved remission.CONCLUSION Higher JIA prevalence,male and ERA predominance,related to a higher frequency of HLA B27 are typical in RS(Y).These data might improve the pediatric rheumatology health service.展开更多
本试验旨在研究普通棉籽粕(CSM)和发酵棉籽粕(FCSM)替代鱼粉对中华绒螯蟹(Eriocheir sinensis)幼蟹生长性能、体成分和肝胰腺消化酶活性的影响。选用初始均重为(0.56±0.04)g的中华绒螯蟹幼蟹660只,随机分成11组,每组3个重复,每个重...本试验旨在研究普通棉籽粕(CSM)和发酵棉籽粕(FCSM)替代鱼粉对中华绒螯蟹(Eriocheir sinensis)幼蟹生长性能、体成分和肝胰腺消化酶活性的影响。选用初始均重为(0.56±0.04)g的中华绒螯蟹幼蟹660只,随机分成11组,每组3个重复,每个重复20只。配制CSM添加量分别为0、8.0%、16.0%、24.0%、32.0%、40.0%(分别记为CSM0、CSM8、CSM16、CSM24、CSM32、CSM40组)和FCSM添加量分别为8.0%、16.0%、24.0%、32.0%、47.5%(分别记为FCSM8、FCSM16、FCSM24、FCSM32、FCSM47.5组)的11种等氮(粗蛋白质,38.39%)等能(总能,19.18 M J/kg)试验饲料。试验期为6周。结果表明:CSM8、CSM16、FCSM8、FCSM16、FCSM24组的增重率、特定生长率、成活率与对照组(CSM0组)差异不显著(P>0.05)。CSM8、FCSM8、FCSM16、FCSM24组的全蟹粗蛋白质含量与对照组亦无显著差异(P>0.05)。饲料中CSM添加量超过16%或FCSM添加量超过24%时,增重率、特定生长率、成活率和全蟹粗蛋白质含量显著低于对照组(P<0.05)。不同水平的CSM和FCSM替代鱼粉对全蟹水分、粗脂肪、粗灰分含量以及肝胰腺脂肪酶活性的影响均不显著(P>0.05)。随着CSM或FCSM替代水平的升高,肝胰腺胃蛋白酶和胰蛋白酶活性逐渐下降,而淀粉酶和纤维素酶活性则逐渐升高。在相同替代水平下,FCSM组的胃蛋白酶和胰蛋白酶活性均不同程度地高于CSM组,而FCSM组的纤维素酶活性则显著低于CSM组(P<0.05)。由此得出,作为中华绒螯蟹幼蟹饲料中鱼粉的替代蛋白质源时,CSM的适宜添加量为16%,FCSM的适宜添加量为24%。此外,相同替代水平下FCSM的替代效果好于CSM。展开更多
利用幼畜胚胎体外生产与胚胎移植(Juvenile in vitro embryo transfer,JIVET)技术能够获得大量卵母细胞,缩短世代间隔,加快育种进程。但幼畜卵母细胞形态及代谢与成年动物存在差异,这导致幼畜胚胎发育能力低。目前,对JIVET技术的研究主...利用幼畜胚胎体外生产与胚胎移植(Juvenile in vitro embryo transfer,JIVET)技术能够获得大量卵母细胞,缩短世代间隔,加快育种进程。但幼畜卵母细胞形态及代谢与成年动物存在差异,这导致幼畜胚胎发育能力低。目前,对JIVET技术的研究主要是通过改进激素处理方案及体外培养条件来增加卵母细胞数量并增强卵母细胞发育能力。从供体羔羊的选择、激素处理和卵母细胞的发育等方面对该技术进行了分析。展开更多
Juvenile xanthogranuloma （JXG） is an uncommon benign disease, which usually occurs on the skin of head and neck region, as a single or multiple nodular lesions. Tracheal involvement of JXG is extremely rare, and onl...Juvenile xanthogranuloma （JXG） is an uncommon benign disease, which usually occurs on the skin of head and neck region, as a single or multiple nodular lesions. Tracheal involvement of JXG is extremely rare, and only few cases have been reported in literature. Herein we presented an additional new case and discussed the optimal management of this uncommon disease.展开更多
文摘BACKGROUND Juvenile arthritis damage index(JADI)is a tool that measures the degree of aggressiveness of the juvenile idiopathic arthritis(JIA)course and assesses articular[JADI-articular damage(JADI-A)]and extraarticular[JADI-extraarticular damage(JADI-E)]damage.While aggressive JIA often requires early bio-logic disease-modified antirheumatic drugs(bDMARDs),the utility of JADI as a predictor of treatment response remains underexplored.AIM To evaluate the potential of JADI as a predictor of bDMARD treatment response in JIA patients.METHODS This prospective study included 112 highly active non-systemic JIA biologic-naïve patients with a mean age of 12.2±4.6 years and a median disease duration of 2.5(interquartile range:1-5)years.Their clinical and radiological assessment,juvenile arthritis disease activity score 71,JADI-A,and JADI-E,were evaluated twice:Before the biologic initiation(baseline)and 12 months after(end of study).At baseline,50%had any damage,with 43%with articular damage and 23%with extraarticular damage.RESULTS During the study,JADI-A/JADI-E improved(33.9%/9.8%),worsened(8.9%/5.4%),or remained unchanged(57.1%/84.8%).Patients with baseline damage had higher markers of JIA activity:Polyarticular course,earlier onset age,ANA-positivity,and more active joints.Patients without initial structural damage(JADI“-”)were more likely(odds ratio=3.8,95%confidence interval:1.6-9.0,P<0.004)to achieve a low degree of activity or remission(46.2%),while on biological therapy,their scores were comparable to JADI-positive(18.3%).Pre-biological joint damage according to the JADI-A index(P=0.003),wrist(P=0.035),elbow(P=0.027),cervical spine limitation of motion(P=0.051),and erosions confirmed by magnetic resonance imaging(P=0.002),were associated with poor response to biological treatment and follow-up JIA activity.CONCLUSION Baseline structural damage in JIA is associated with diminished bDMARDs efficacy,increased disability,and shorter remission duration.JADI enhances conventional clinical risk stratification by facilitating timely initiation of bDMARDs,adherence to treat-to-target strategy and tailored patient care.
文摘The compelling case report by Xie et al,published in a renowned medical journal,is an excellent example of meticulous clinical evaluation,comprehensive labo-ratory testing,advanced imaging,and genetic analysis.The authors identified novel compound heterozygous mutations in the hemojuvelin gene of a patient diagnosed with juvenile hemochromatosis.They suggested that long-term,strategic phlebotomy might offer a novel therapeutic strategy for severe juvenile hemochromatosis,challenging the traditional treatment paradigms.
文摘BACKGROUND Chronic idiopathic uveitis(CIU)and juvenile idiopathic arthritis-associated uveitis(U-JIA)are both vision-threatening conditions that share similar autoimmune mechanisms,but treatment approaches differ significantly.In managing U-JIA,various treatment options are employed,including biological and non-biological disease-modifying anti-rheumatic drugs.These drugs are effective in clinical trials.Given the lack of established diagnostic and treatment guidelines as well as the limited number of therapeutic options available,patients with CIU frequently do not receive optimal and timely immunosuppression.This study highlighted the necessity for additional research to develop novel diag-nostic techniques,targeted therapies,and enhanced treatment outcomes for young individuals with CIU.AIM To compare the characteristics and outcomes of U-JIA and CIU.METHODS A retrospective cohort study analyzed data from 110 pediatric patients(under 18 years old)with U-JIA and 40 pediatric patients with CIU.Data was collected between 2012 and 2023.The study focused on demographic,clinical,treatment,and outcome variables.RESULTS The median onset age of arthritis was 6.4 years(2.7 years;9.3 years).In 28.2%of cases uveitis preceded the onset of arthritis.In 17.3%of cases it occurred simultan-eously.In 53.6%of cases it followed arthritis.Both groups had similar onset ages,antinuclear antibodies/human leukocyte antigen positivity rates,and ESR levels,with a slight predominance of females(60.9%vs 42.5%,P=0.062),and higher C-reactive protein levels in the U-JIA group.Anterior uveitis was more prevalent in patients with U-JIA(P=0.023),although the frequency of symptomatic,unilateral,and complicated forms did not differ significantly.The use of methotrexate(83.8%vs 96.4%)and biologics(64.7%vs 82.1%)was comparable,as was the rate of remission on methotrexate treatment(70.9%vs 56.5%)and biological therapy(77.8%vs 95%),but a immunosuppressive treatment delay in CIU observed.Patients with CIU were less likely to receive methotrexate[hazard ratio(HR)=0.48,P=0.005]or biological treatment(HR=0.42,P=0.004),but they were more likely to achieve remission with methotrexate(HR=3.70,P=0.001).CONCLUSION Treatment of uveitis is often limited to topical measures,which can delay systemic therapy and affect the outcome.Methotrexate and biological agents effectively manage eye inflammation.It is essential to develop standardized protocols for the diagnosis and management of uveitis,and collaboration between rheumatologists and ophthal-mologists is needed to achieve optimal outcomes in the treatment of CIU.
文摘BACKGROUND Children with juvenile idiopathic arthritis(JIA)and inflammatory bowel disease(IBD)face an elevated risk of severe infection owing to their diseases and the immunosuppressive treatment for disease control.AIM To compare scheduled vaccination coverage and the levels of post-vaccine antibodies against measles,mumps,rubella(MMR)and hepatitis B in pediatric patients with IBD and JIA.METHODS A comparative cohort study included 97 patients with IBD and 170 patients with JIA.Vaccination history was obtained from medical records,while post-vaccination immunity was assessed prospectively by measuring specific IgG antibody titers using enzyme-linked immunosorbent assays(Vector-Best JSC,Russia;IBL International,Germany)during routine visits between January 2022 and April 2023.RESULTS A complete two-dose MMR course had been administered to 66.3%of IBD patients and 55.9%of JIA patients(P=0.121).By contrast,the three-dose hepatitis B schedule was completed in 74.2%of IBD and 100%of JIA patients(P<0.001).Protective level of anti-vaccine antibodies against measles(47.7%vs 57.7%;P=0.168);mumps(75.3%vs 80.0%;P=0.366);rubella(74.4%vs 98.2%;P<0.0001);and hepatitis B(44.8%vs 50.0%;P=0.514)were detected in IBD and JIA patients,respectively.CONCLUSION Patients with IBD and JIA demonstrated different vaccination coverage patterns and levels of anti-vaccine antibodies.Routine baseline serology and timely booster vaccination should be implemented for all pediatric patients receiving chronic immunosuppression.
基金supported by Grants from the Agencia Nacional de Promoción Científica y Tecnológica,from Consejo Nacional de Investigaciones Científicas y Técnicas(CONICET)and the University of Buenos Aires.
文摘For songbirds,the post-fledging period is critical for development and survival,as young must learn to get food,practice flying,and identify predators to become independent from their parents and disperse or migrate.Obligate avian brood parasites,like cuckoos and cowbirds,lay eggs in nests of other bird species that provide parental care until the parasite’s young become independent.The information on the post-fledging period in songbirds is limited due to the difficulty in following and observing fledglings and it is even scarcer for brood parasites.We studied the behavior,survival,and age of independence of Shiny Cowbird(Molothrus bonariensis)fledglings raised by 2 hosts that differ markedly in body size:the House Wren(Troglodytes aedon)and the Chalk-Browed Mockingbird(Mimus saturninus).We radio-tracked individuals from a few days before they left the nest until they disappeared from the natal territory.We found:(1)chicks left the nest when they were 11-15 days of age and the age of fledgling did not differ between the 2 host species,(2)after leaving the nest,they spent most of the time perched in a hidden place without begging but as they grew,the proportion of time perched without begging decreased and begging in hidden and exposed places increased,(3)we observed approaches between adult parasitic females and young in host territories,(4)young abandoned the host territory and joined conspecific flocks when they were 35-39 days of age,and(5)the estimated post-fledging survival was between 12.5%and 20.8%.
文摘Background:A germicide like Izal is chemical agent used for reducing and preventing microbial loads in systems for treating water as well as other industries like agriculture and aquaculture.The environmental impact of germicide is often more than the target intended by those who use them.The purpose of the current study was to ascertain the toxicity levels and behavioral alterations of Juvenile African catfish,Clarias gariepinus,subjected to Izal exposure.Methods:The stock solution was created from the 100 mL by mixing 1 mL of the Izal with 99 mL of water.After that,different nominal concentrations of Izal were prepared using the stock solution by diluting the toxin with tap water in determined amounts(10 L).The fish were exposed to acute concentrations of 1.0,1.5,2.0,2.5 and 3.0 mg/L of Izal had a control that served as a reference point.Results:The lethal concentration of Izal(24 h LC50)was found to be 2.4136 mg/L(95%confidence interval=2.1707–2.6838)for test fish under a semi-static system showing that Izal is moderately poisonous to the species in a dose-dependent manner and time(duration).The highest mortality was recorded in the highest concentration(3.0 mg/L)of Izal while the least mortality was observed in the 1.5 mg/L group.Izal-exposed fish had aberrant behavior,including hyperactivity,gasping of air,respiratory distress,attempts to jump out of the water,erratic swimming,refusal to feed,lethargic movement,and slow response to feed,loss of balance and depigmentation.The severity of the abnormal behavior decreased after 24 h,but remained mildly to moderately high at 2.0 and 2.5 mg/L of Izal.It was found that the death rate increased with Izal concentration and duration.Conclusion:The present study revealed that Izal was moderately toxic to Claris gariepinus juveniles and should be used prudently in the environment to guard against toxicological effect on non-target organisms such as fish.For this reason,the use of the germicides should be with caution especially near water bodies.
文摘BACKGROUND Juvenile polyps(JPs)are non-neoplastic polyps.In adults,JPs present with hematochezia in only approximately half the patients and are often found incidentally during endoscopic screening.JPs have no mucosal fascia at the tip,and spontaneous shedding and massive gastrointestinal hemorrhage may occur.Thus,the JP bleeding detected in this case by extravascular contrast leakage on computed tomography scans and treated with endoscopic clipping is rare.CASE SUMMARY A previously healthy 31-year-old male patient presented with a 2-day history of bloody stools.Upon hospital arrival,rectal examination revealed fresh blood,and abdominal computed tomography scans showed extravascular contrast leakage from the lower rectum’s left-side wall.His blood pressure was slightly low at 104/62 mmHg.However,his pulse rate(69 bpm)and oxygen level(99%on room air)were within normal limits.Emergency endoscopy revealed a pedunculated lesion in the rectum covered by a non-neoplastic mucosal epithelium.No neoplastic lesions were observed at the tip of the polyp;however,pulsatile bleeding was detected at the distal end.We performed endoscopic hemostasis by clipping the stem and then performed a polypectomy above the stem to examine the lesion tissue.Histopathological evaluation revealed a cystically dilated gland without neoplastic lesions.A subsequent total colonoscopy revealed two JPs with characteristic edematous,smooth,and reddish surfaces close to the hemorrhagic lesion.Subsequent histopathological evaluation indicated findings characteristic of JP,such as severe inflammatory cell infiltration of the stroma and cystic dilatation of the glandular ducts.CONCLUSION There are no reports of adult JPs presenting with contrast extravasation where endoscopic hemostasis was successful,as in this case.
基金supported by the National Natural Science Foundation of China(32172389)the Excellent Youth Foundation of Henan Province,China(232300421029)the Key Research and Development Project of Henan Province,China(221111112200)。
文摘The insect fat body is comparable to the liver and adipose tissue in vertebrates,and plays a pivotal role in energy metabolism,nutrient storage,and reproduction.During metamorphosis,the fat body is disassembled via programmed cell death and cell dissociation.After adult eclosion,the fat body is reconstructed either by repopulation from the remaining juvenile fat body cells or by differentiation from adult progenitor cells.This reconstruction is a prerequisite for initiating the extensive synthesis of vitellogenin(Vg),which is necessary for the maturation of eggs.Despite its significance,the underlying mechanisms of this reconstruction remain inadequately understood.Transcriptome analysis of the fat bodies from migratory locusts at 0-5 days post adult emergence revealed 79 genes associated with chromatin remodeling.Weighted gene co-expression network analysis indicated a positive correlation between chromatin remodeling and fat body reconstitution.Protein-protein interaction analysis revealed that brahma,which encodes the catalytic subunit of the SWI/SNF chromatin remodeling complex,is crucial for post-adult-eclosion fat body development.qRT-PCR analysis demonstrated that the levels of brahma mRNA in the fat body are progressively increased during the previtellogenic stage,then reach the peak and remain elevated in the vitellogenic phase.Furthermore,brahma is expressed in response to gonadotropic juvenile hormone(JH).Knockdown of brahma led to a marked reduction in Vg expression within the fat body,along with arrested ovarian growth.These findings shed light on the involvement of brahmamediated chromatin remodeling in JH-stimulated fat body reconstruction and reproduction of adult female locusts.
基金supported by the National Natural Science Foundation of China[Grants.41802054]supported by a Royal Society SinoBritish Fellowship Trust International Exchanges Award[Grant No.IESR3213093]。
文摘The high Ba-Sr rocks can provide significant clues about the evolution of the continent lithosphere,but their petrogenesis remains controversial.Identifying the Late Cretaceous high Ba-Sr granodiorites in the SE Lhasa Block could potentially provide valuable insights into the continent evolution of the Qinghai-Tibet Plateau.Zircon U-Pb ages suggest that the granodiorites were emplaced at 87.32±0.43 Ma.Geochemically,the high Ba-Sr granodiorites are characterized by elevated K_(2)O+Na_(2)O contents(8.18-8.73 wt%)and K_(2)O/Na_(2)O ratios(0.99-1.25,mostly>1),and belong to high-K calc-alkaline to shoshonitic series.The Yonglaga granodiorites show notably high Sr(653-783 ppm)and Ba(1346-1531 ppm)contents,plus high Sr/Y(30.92-38.18)and(La/Yb)_(N)(27.7-34.7)ratios,but low Y(20.0-22.8 ppm)and Yb(1.92-2.19 ppm)contents with absence of negative Eu anomalies(δEu=0.83-0.88),all similar to typical high Ba-Sr granitoids.The variable zirconεHf(t)values of-4.58 to+12.97,elevated initial^(87)Sr/^(86)Sr isotopic ratios of 0.707254 to 0.707322 and lowεNd(t)values of-2.8 to-3.6 with decoupling from the Hf system suggest that a metasomatized mantle source included significant recycled ancient materials.The occurrence of such high Ba-Sr intrusions indicates previous contributions of metasomatized mantle-derived juvenile material to the continents,which imply the growth of continental crust during the Late Cretaceous in the SE Lhasa.Together with regional data,we infer that the underplated mafic magma provides a significant amount of heat,which leads to partial melting of the juvenile crust.The melting of the metasomatized mantle could produce a juvenile mafic lower crust,from which the high Ba-Sr granitoids were derived from reworking of previous mafic crust during the Late Cretaceous(ca.100-80 Ma)in the SE Lhasa.
文摘AIM:To characterize a Portuguese population of patients with juvenile idiopathic arthritis(JIA)and to evaluate possible associations between clinical factors and ocular involvement.METHODS:Patients diagnosed with JIA in the previous 20y in Hospital Garcia de Orta were included.Data were assessed from Reuma.pt database.Associations between demographic(age and sex),clinical(articular involvement,extra-articular manifestations,biological therapy),laboratory data[anti-nuclear antibodies,anti-cyclic citrullinated peptide(CCP)antibodies,rheumatoid factor,human leukocyte antigen B27(HLA-B27),C-reactive protein,erythrocyte sedimentation rate]and ocular involvement were assessed.Statistical analysis was performed using Chi-square for categorical variables and Mann-Whitney test for continuous variables.RESULTS:Totally 91 patients were included,11(12%)with previous episodes of uveitis.There was a statistically significant preponderance of early age at JIA diagnosis(mean 4.73 vs 9.58y,P=0.008),antinuclear antibodies positivity(P=0.01),and oligoarticular subtype(P=0.04)in the Juvenile idiopathic arthritis-associated uveitis(JIA-U)group.Ocular complications occurred in 36.4%of patients(n=4):cataracts(n=2),band keratopathy(n=1)and posterior synechiae(n=1).The occurrence of complications was corelated with a shorter period between JIA diagnosis and the first JIA-U episode(mean 0.67 vs 4.88y,P=0.012)but not with age at JIA diagnosis or articular involvement.There was erythrocyte sedimentation rate elevation in the 12mo preceding uveitis(mean 40.5 mm/h,range 13-83).CONCLUSION:The occurrence of JIA-U shortly after JIA diagnosis is shown to be a potential risk factor for ocular complications.
文摘In this manuscript, we present a case report of a child with 16-year-old previously healthy Hispanic male who presented for progressive proximal muscle weakness, rash, and dysphagia. He was admitted to the acute care floor and diagnosed with juvenile dermatomyositis and found to be positive for anti-Mi-2 alpha, anti-Mi-2 beta, and anti-MDA-5 antibodies. He gradually improved with a combination of steroid, immunomodulatory treatment, and physical therapy. This case outlines the clinical course of a patient with this rare disorder as well as the importance of understanding the role of associated antibodies to manage potential long-term sequelae.
文摘BACKGROUND Prevalence of the main rheumatic diseases in the Republic of Sakha(Yakutia)[RS(Y)],one of the regions of the Russian Federation,differs from the other regions of the Russian Federation due to its ethnic and geographic features.Knowledge regarding the prevalence and structure of juvenile idiopathic arthritis(JIA)allows us to shape the work of the pediatric rheumatology service in the region correctly,and optimize the healthcare system and the need for medica-tions.AIM To describe the epidemiological,demographic,clinical,and laboratory characteristics of children with JIA in the RS(Y)and evaluate the main outcomes.METHODS This retrospective cohort study assessed all the data from the medical histories of the patients(n=225)diagnosed with JIA(2016-2023)in the Cardiorheumatology Department of the M.E.Nikolaev National Center of Medicine.Pearson'sχ²test,Fisher's exact test,Mann–Whitney and Kruskal-Wallis tests were used for statistical analyses.RESULTS The ethnic prevalence of JIA is higher in Sakha than in Russian children at 110.1 per 100000 children and 69.4 per 100000 children,respectively.The prevalence of JIA among boys and girls in Sakha was similar,unlike in Russians,where the number of girls predominated.The JIA categories were as follows:(1)Systemic arthritis:3.5%;(2)Oligoarthritis(persistent and extended):33.8%;(3)Rheumatoid factor(RF)(+)polyarthritis:0.9%;(4)RF(-)polyarthritis:14.7%;(5)Enthesitis-related arthritis(ERA):44%;and(6)Psoriatic arthritis:3.1%.Prevalence of the ERA category was 4.4 times higher in Sakha children,but the prevalence of systemic arthritis was 2.9 times lower compared to Russians(P=0.0005).The frequency of uveitis was 10.2%,and the frequency of human leukocyte antigen(HLA)B27 was 39.6%in JIA children.Biologic treatment was received by 40.4%of JIA children and 45.3%achieved remission.CONCLUSION Higher JIA prevalence,male and ERA predominance,related to a higher frequency of HLA B27 are typical in RS(Y).These data might improve the pediatric rheumatology health service.
文摘本试验旨在研究普通棉籽粕(CSM)和发酵棉籽粕(FCSM)替代鱼粉对中华绒螯蟹(Eriocheir sinensis)幼蟹生长性能、体成分和肝胰腺消化酶活性的影响。选用初始均重为(0.56±0.04)g的中华绒螯蟹幼蟹660只,随机分成11组,每组3个重复,每个重复20只。配制CSM添加量分别为0、8.0%、16.0%、24.0%、32.0%、40.0%(分别记为CSM0、CSM8、CSM16、CSM24、CSM32、CSM40组)和FCSM添加量分别为8.0%、16.0%、24.0%、32.0%、47.5%(分别记为FCSM8、FCSM16、FCSM24、FCSM32、FCSM47.5组)的11种等氮(粗蛋白质,38.39%)等能(总能,19.18 M J/kg)试验饲料。试验期为6周。结果表明:CSM8、CSM16、FCSM8、FCSM16、FCSM24组的增重率、特定生长率、成活率与对照组(CSM0组)差异不显著(P>0.05)。CSM8、FCSM8、FCSM16、FCSM24组的全蟹粗蛋白质含量与对照组亦无显著差异(P>0.05)。饲料中CSM添加量超过16%或FCSM添加量超过24%时,增重率、特定生长率、成活率和全蟹粗蛋白质含量显著低于对照组(P<0.05)。不同水平的CSM和FCSM替代鱼粉对全蟹水分、粗脂肪、粗灰分含量以及肝胰腺脂肪酶活性的影响均不显著(P>0.05)。随着CSM或FCSM替代水平的升高,肝胰腺胃蛋白酶和胰蛋白酶活性逐渐下降,而淀粉酶和纤维素酶活性则逐渐升高。在相同替代水平下,FCSM组的胃蛋白酶和胰蛋白酶活性均不同程度地高于CSM组,而FCSM组的纤维素酶活性则显著低于CSM组(P<0.05)。由此得出,作为中华绒螯蟹幼蟹饲料中鱼粉的替代蛋白质源时,CSM的适宜添加量为16%,FCSM的适宜添加量为24%。此外,相同替代水平下FCSM的替代效果好于CSM。
文摘利用幼畜胚胎体外生产与胚胎移植(Juvenile in vitro embryo transfer,JIVET)技术能够获得大量卵母细胞,缩短世代间隔,加快育种进程。但幼畜卵母细胞形态及代谢与成年动物存在差异,这导致幼畜胚胎发育能力低。目前,对JIVET技术的研究主要是通过改进激素处理方案及体外培养条件来增加卵母细胞数量并增强卵母细胞发育能力。从供体羔羊的选择、激素处理和卵母细胞的发育等方面对该技术进行了分析。
文摘Juvenile xanthogranuloma （JXG） is an uncommon benign disease, which usually occurs on the skin of head and neck region, as a single or multiple nodular lesions. Tracheal involvement of JXG is extremely rare, and only few cases have been reported in literature. Herein we presented an additional new case and discussed the optimal management of this uncommon disease.
