AIM:To investigate the association between juvenile open angle glaucoma(JOAG)and mental health among Koreans.METHODS:This study used nationally representative data from the 8th Korea National Health and Nutrition Exam...AIM:To investigate the association between juvenile open angle glaucoma(JOAG)and mental health among Koreans.METHODS:This study used nationally representative data from the 8th Korea National Health and Nutrition Examination Survey(KNHANES)2021.Glaucoma diagnosis followed the International Society of Geographical and Epidemiological Ophthalmology criteria based on glaucomatous structural defects,visual field defects,corrected vision,and intraocular pressure.As outcomes,suicidal behaviors,psychiatric counseling,and depression were evaluated through mental health questionnaires.Odds ratios(ORs)with 95%confidence intervals(CIs)were estimated using logistic regression models,adjusting for covariates.RESULTS:Among 7090 participants,3446 met the inclusion criteria for analysis,and 88(2.6%)were diagnosed with open angle glaucoma(OAG).After adjusting for age,sex,and best-corrected visual acuity(VA),participants with OAG were revealed to have significantly higher odds of suicidal behaviors(i.e.,ideation,planning,or attempts)compared with those without OAG(OR:2.70;95%CI:1.12-6.54;P=0.028).This association remained significant after further adjustments for socioeconomic status,lifestyle factors,and presence of chronic conditions(P=0.031 and 0.035,respectively).However,there was no significant difference for the other two outcomes,psychiatric counseling and depression.An age-stratified analysis revealed a stronger association between OAG and suicidal behaviors in younger JOAG participants(<40y)than in older OAG participants(≥40y;OR:3.80 vs 2.22;95%CI:0.79-18.22 vs 0.56-8.80,respectively).CONCLUSION:OAG patients show a higher risk of suicidal behaviors than those without glaucoma particularly in JOAG patients.展开更多
Soaring bird migration often relies on suitable terrain and airflow;therefore,route selection is vital for successful migration.While age and experience have been identified as key factor influencing migration route s...Soaring bird migration often relies on suitable terrain and airflow;therefore,route selection is vital for successful migration.While age and experience have been identified as key factor influencing migration route selection among soaring raptors in the African-Eurasian Flyway,how they shape the migration route of soaring raptors in East Asia is still largely unknown.In this study,we investigated potential variations in the routes and timing in autumn migration of juvenile and older soaring birds,using count data of Greater Spotted Eagles(Clanga clanga)from two coastal sites and two inland sites in China.From 2020 to 2023,we recorded a total of 340 individuals,with the highest site averaging over 90 individuals per autumn,making it one of the world’s top single-season counts and thus a globally important site for this species.We found that 82% and 61% records from coastal sites were juveniles,significantly higher than inland sites(15% and 24%).Juveniles at all four sites exhibited markedly earlier median passage time than non-juveniles,with brief overlapping in their main migration periods.Both coastal sites are located on the tip of peninsulas stretching southwest,requiring long overwater flights if crossing the Bohai Bay or Beibu Gulf,which would be energetically demanding and increase mortality risk.Experienced individuals may have learned to avoid such terrain and subsequent detour,while juveniles are more prone to enter these peninsulas due to lack of experience and opportunities for social learning,or following other raptor species that are more capable of powered flight.Our findings highlight the importance of age and experience in migration route selection of large soaring birds.展开更多
Background: Juvenile Idiopathic Arthritis (JIA) is the most prevalent rheumatic disease in children. It is associated with abnormal levels of serum zinc (Zn) and copper (Cu) as during inflammation serum copper concent...Background: Juvenile Idiopathic Arthritis (JIA) is the most prevalent rheumatic disease in children. It is associated with abnormal levels of serum zinc (Zn) and copper (Cu) as during inflammation serum copper concentration increases and zinc decreases. Objective: To assess the serum Zn and Cu levels in different sub-types of JIA patients and their correlation with the disease duration. Methods: This cross-sectional study was conducted over twelve months at the Pediatric Rheumatology Division, Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University. Sixty-nine JIA cases that fulfilled the International League of Association for Rheumatology (ILAR) criteria were taken as cases and age and sex-matched healthy children were considered as controls. The serum Zn and Cu tests were done using the spectrophotometric method with INDIKO PLUS Drug Analyzer. Data were recorded in a pre-designed questionnaire. Data were checked, verified and analyzed manually where continuous variables were analyzed using unpaired t-test and categorical variables using the ANOVA test. Pearson’s correlation coefficient test was used to see the correlation of serum zinc and copper levels with disease duration. Results: Boys were predominant in both case and control groups, with the majority within the 10 to 16-year-age group. Enthesitis-related arthritis (ERA) was the most common subtype followed by sJIA, Oligo JIA, Poly JIA (RF-) and unclassified subtypes. Disease duration was found less than 12 months in 30.4% of JIA patients. Serum analysis revealed a statistically significant reduction in mean zinc levels and increased copper levels in JIA patients compared to controls. This study observed a negative correlation between serum zinc levels and disease duration, whereas serum copper levels exhibited a positive correlation with disease duration. Conclusion: In conclusion, this study revealed that JIA patients exhibit alterations in serum zinc and copper levels. Serum copper levels showed a positive correlation and serum zinc levels showed a negative correlation with the duration of the disease.展开更多
Juvenile xanthogranuloma (XGJ) is the most common form of non-Langerhansian histiocytosis. We report a pediatric case of multiple XGJ without visceral involvement in a 6-month-old female infant who was hospitalized wi...Juvenile xanthogranuloma (XGJ) is the most common form of non-Langerhansian histiocytosis. We report a pediatric case of multiple XGJ without visceral involvement in a 6-month-old female infant who was hospitalized with disseminated congenital skin nodules, of firm consistency, of variable diameter ranging from a few millimetres to 1 cm, involving the face, scalp, trunk and limbs. There was no mucosal localization. The remainder of the somatic examination was unremarkable. The diagnosis of disseminated XGJ was confirmed by skin biopsy without visceral or systemic involvement. Therapeutic abstention was recommended. This observation underlines the fact that XGJ is a rare clinical entity that should not be overlooked when faced with congenital skin nodules.展开更多
BACKGROUND Juvenile hemochromatosis(JH)is an early-onset,rare autosomal recessive disorder of iron overload observed worldwide that leads to damage in multiple organs.Pathogenic mutations in the hemojuvelin(HJV)gene a...BACKGROUND Juvenile hemochromatosis(JH)is an early-onset,rare autosomal recessive disorder of iron overload observed worldwide that leads to damage in multiple organs.Pathogenic mutations in the hemojuvelin(HJV)gene are the major cause of JH.CASE SUMMARY A 34-year-old male Chinese patient presented with liver fibrosis,diabetes,hypogonadotropic hypogonadism,hypophysis hypothyroidism,and skin hyperpigmentation.Biochemical test revealed a markedly elevated serum ferritin level of 4329μg/L and a transferrin saturation rate of 95.4%.Targeted exome sequencing and Sanger sequencing revealed that the proband had a novel mutation c.863G>A(p.R288Q)in the HJV gene which was transmitted from his father,and two known mutations,c.18G>C(p.Q6H)and c.962_963delGCinsAA(p.C321*)in cis,which were inherited from his mother.The p.R288W mutation was previously reported to be pathogenic for hemochromatosis,which strongly supported the pathogenicity of p.R288Q reported for the first time in this case.After 72 wk of intensive phlebotomy therapy,the patient achieved a reduction in serum ferritin to 160.5μg/L.The patient's clinical symptoms demonstrated a notable improvement.CONCLUSION This study highlights the importance of screening for hemochromatosis in patients with diabetes and hypogonadotropic hypogonadism.It also suggests that long-term active phlebotomy could efficiently improve the prognosis in severe JH.展开更多
This study focused on the supplementation of four-sided spice fruit powder (Tetrapleura tetraptera) on some zootechnical performances of juveniles of Clarias gariepinus and was carried out from February 14 to July 20 ...This study focused on the supplementation of four-sided spice fruit powder (Tetrapleura tetraptera) on some zootechnical performances of juveniles of Clarias gariepinus and was carried out from February 14 to July 20 at the Massoma Fish Farm of Bojongo Mbeidi located in the Littoral Region, Department of Wouri, Douala IV district. The farm benefits from a climate favorable to aquaculture activity. For this purpose, five (5) treatments were tested including T<sub>0+</sub> (imported feed), T<sub>0-</sub>, T<sub>0.3</sub>, T<sub>0.4</sub>, and T<sub>0.5</sub> respectively for the treatments with 0% supplemented food;0.3%;0.4% and 0.5% of Tetrapleura tetraptera powder. A total of 450 Clarias gariepinus fry with an initial average weight of 7 ± 1.5 g, were distributed in happas of 0.49 m<sup>2</sup> each placed in Triplicate in a completely randomized device. Four isoprotein foods with 38% crude protein were formulated to feed these fry at 7% of their biomass per day for 15 days then to their fullness until the end of the experiment. Intermediate fishing was carried out every fortnight. 75 days later, the results recorded show that the highest average survival rate (96.44% ± 3.5%) was recorded with the subjects fed feed supplemented with 0.4% T. tetraptera powder. The average weight (120.93 ± 67.20), weight gain (113.93 ± 67.20), specific growth rate (3.64 ± 0.79% g/d) and total lengths and standard (26.43 ± 1.9 and 23.66 ± 1.76) the highest were recorded with the T<sub>0+</sub> treatment (imported feed). The highest conditioning factor K (2.14 ± 0.15) was recorded with the treatment supplemented with 0.4% T. tetraptera. The lowest conversion ratio (1.28) was recorded with the treatment fed with imported blue crown food (T<sub>0+</sub>). Furthermore, the lowest production cost was recorded with the treatment supplemented with 0.4% T. tetraptera. It appears from this study that the juveniles of C. gariepinus better value the feed substituted with 0.4% of the powder of four sides (Tetrapleura tetraptera).展开更多
BACKGROUND Various non-steroidal anti-inflammatory drugs(NSAIDs)have been used for juvenile idiopathic arthritis(JIA).However,the optimal method for JIA has not yet been developed.AIM To perform a systematic review an...BACKGROUND Various non-steroidal anti-inflammatory drugs(NSAIDs)have been used for juvenile idiopathic arthritis(JIA).However,the optimal method for JIA has not yet been developed.AIM To perform a systematic review and network meta-analysis to determine the optimal instructions.METHODS We searched for randomized controlled trials(RCTs)from PubMed,EMBASE,Google Scholar,CNKI,and Wanfang without restriction for publication date or language at August,2023.Any RCTs that comparing the effectiveness of NSAIDs with each other or placebo for JIA were included in this network meta-analysis.The surface under the cumulative ranking curve(SUCRA)analysis was used to rank the treatments.P value less than 0.05 was identified as statistically significant.RESULTS We included 8 RCTs(1127 patients)comparing 8 different instructions including meloxicam(0.125 qd and 0.250 qd),Celecoxib(3 mg/kg bid and 6 mg/kg bid),piroxicam,Naproxen(5.0 mg/kg/d,7.5 mg/kg/d and 12.5 mg/kg/d),inuprofen(30-40 mg/kg/d),Aspirin(60-80 mg/kg/d,75 mg/kg/d,and 55 mg/kg/d),Tolmetin(15 mg/kg/d),Rofecoxib,and placebo.There were no significant differences between any two NSAIDs regarding ACR Pedi 30 response.The SUCRA shows that celecoxib(6 mg/kg bid)ranked first(SUCRA,88.9%),rofecoxib ranked second(SUCRA,68.1%),Celecoxib(3 mg/kg bid)ranked third(SUCRA,51.0%).There were no significant differences between any two NSAIDs regarding adverse events.The SUCRA shows that placebo ranked first(SUCRA,88.2%),piroxicam ranked second(SUCRA,60.5%),rofecoxib(0.6 mg/kg qd)ranked third(SUCRA,56.1%),meloxicam(0.125 mg/kg qd)ranked fourth(SUCRA,56.1%),and rofecoxib(0.3 mg/kg qd)ranked fifth(SUCRA,56.1%).CONCLUSION In summary,celecoxib(6 mg/kg bid)was found to be the most effective NSAID for treating JIA.Rofecoxib,piroxicam,and meloxicam may be safer options,but further research is needed to confirm these findings in larger trials with higher quality studies.展开更多
A recent study published in World J Clin Cases addressed the optimal non-steroidal anti-inflammatory drugs(NSAIDs)for juvenile idiopathic arthritis(JIA).Herein,we outline the progress in drug therapy of JIA.NSAIDs hav...A recent study published in World J Clin Cases addressed the optimal non-steroidal anti-inflammatory drugs(NSAIDs)for juvenile idiopathic arthritis(JIA).Herein,we outline the progress in drug therapy of JIA.NSAIDs have traditionally been the primary treatment for all forms of JIA.NSAIDs are symptom-relief medications,and well tolerated by patients.Additionally,the availability of selective NSAIDs further lower the gastrointestinal adverse reactions compared with traditional NSAIDs.Glucocorticoid is another kind of symptom-relief medications with potent anti-inflammatory effect.However,the frequent adverse events limit the clinical use.Both NSAIDs and glucocorticoid fail to ease or pre-vent joint damage,and the breakthrough comes along with the disease-mo-difying antirheumatic drugs(DMARDs).DMARDs can prevent disease pro-gression and reduce joint destruction.Particularly,the emergence of biologic DMARDs(bDMARDs)has truly revolutionized the therapeutics of JIA,compared with conventional synthetic DMARDs.As a newly developed class of drugs,the places of most bDMARDs in the management of JIA remain to be well estab-lished.Nevertheless,the continuous evolution of bDMARDs raises hopes of improving long-term disease outcomes for JIA.展开更多
BACKGROUND Previous studies in the pre-biological era showed an association of wrist inflammation in juvenile idiopathic arthritis(JIA)with progressive disease course,polyarticular involvement and failure of methotrex...BACKGROUND Previous studies in the pre-biological era showed an association of wrist inflammation in juvenile idiopathic arthritis(JIA)with progressive disease course,polyarticular involvement and failure of methotrexate treatment.AIM To describe features of JIA,associated with wrist arthritis.METHODS Data from about 753 JIA patients were included in this retrospective cohort study.The clinical and laboratory features of patients with and without wrist involvement were analyzed.RESULTS Wrist involvement was found in oligoarthritis(5.8%),RF(−)/RF(+)polyarthritis(44.9%/15.0%),enthesitis-related arthritis(17.7%),and systemic(58.6%)JIA categories.Unilateral wrist involvement was typical for oligoarthritis patients,bilateral involvement was either equal to that of unilateral involvement or was more frequent in other categories.Wrist arthritis was found to be associated with female sex,a low incidence of uveitis,and more indications of systemic inflammation,including elevated levels of C-reactive protein,erythrocyte sedimentation rate,and platelets,as well as involvement of the cervical spine,temporomandibular,shoulder,elbow,metacarpophalangeal,proximal interphalangeal,distal interphalangeal,hip,ankle,and tarsus arthritis.The number of patients with hip osteoarthritis and hip replacement was also higher.Wrist arthritis was associated with a lower probability of achieving remission[hazard ratio(HR)=1.3(95%CI:1.0-1.7),P=0.055],and a higher probability of being treated with biologics[HR=1.7(95%CI:1.3-2.10,P=0.00009)].CONCLUSION Wrist arthritis in JIA patients is a marker of a severe disease course,characterized by more intensive inflammation,unfavorable outcomes,and.requiring more intensive treatment with early administration of biologics.Close monitoring of wrist inflammation with ultrasound and MR assessment with early biological treatment might improve the outcomes.展开更多
BACKGROUND Lung damage in systemic juvenile arthritis(sJIA)is one of the contemporary topics in pediatric rheumatology.Several previous studies showed the severe course and fatal outcomes in some patients.The informat...BACKGROUND Lung damage in systemic juvenile arthritis(sJIA)is one of the contemporary topics in pediatric rheumatology.Several previous studies showed the severe course and fatal outcomes in some patients.The information about interstitial lung disease(ILD)in the sJIA is scarce and limited to a total of 100 cases.AIM To describe the features of sJIA patients with ILD in detail.METHODS In the present retrospective cohort study,information about 5 patients less than 18-years-old with sJIA and ILD were included.The diagnosis of sJIA was made according to the current 2004 and new provisional International League of Associations for Rheumatology criteria 2019.ILD was diagnosed with chest computed tomography with the exclusion of other possible reasons for concurrent lung involvement.Macrophage activation syndrome(MAS)was diagnosed with HLH-2004 and 2016 EULAR/ACR/PRINTO Classification Criteria and hScores were calculated during the lung involvement.RESULTS The onset age of sJIA ranged from 1 year to 10 years.The time interval before ILD ranged from 1 mo to 3 years.The disease course was characterized by the prevalence of the systemic features above articular involvement,intensive rash(100%),persistent and very active MAS(hScore range:194-220)with transaminitis(100%),and respiratory symptoms(100%).Only 3 patients(60%)developed a clubbing phenomenon.All patients(100%)had pleural effusion and 4 patients(80%)had pericardial effusion at the disease onset.Two patients(40%)developed pulmonary arterial hypertension.Infusion-related reactions to tocilizumab were observed in 3(60%)of the patients.One patient with trisomy 21 had a fatal disease course.Half of the remaining patients had sJIA remission and 2 patients had improvement.Lung disease improved in 3 patients(75%),but 1 of them had initial deterioration of lung involvement.One patient who has not achieved the sJIA remission had the progressed course of ILD.No cases of hyper-eosinophilia were noted.Four patients(80%)received canakinumab and one(20%)tocilizumab at the last follow-up visit.CONCLUSION ILD is a severe life-threatening complication of sJIA that may affect children of different ages with different time intervals since the disease onset.Extensive rash,serositis(especially pleuritis),full-blown MAS with transaminitis,lymphopenia,trisomy 21,eosinophilia,and biologic infusion reaction are the main predictors of ILD.The following studies are needed to find the predictors,pathogenesis,and treatment options,for preventing and treating the ILD in sJIA patients.展开更多
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease deadly. In Africa, little data exists on this condition and the form familial is the most common with a younger age of onset. Our observations relate ...Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease deadly. In Africa, little data exists on this condition and the form familial is the most common with a younger age of onset. Our observations relate to three brothers, born from a consanguineous marriage. The diagnosis of amyotrophic lateral sclerosis was certain placed in all patients according to the criteria of Awaji. The average age of patients was 16.66 years, and the average age of onset of symptoms was 10.33 years old. The female gender was the most represented with a sex ratio of 0.5. Our patients benefited from rilusole, vitamin D and physiotherapy. A only patient presented with dysphagia. However, no cases have been observed at this day.展开更多
Background: Systemic JIA (sJIA) is one of the subtypes of JIA, which is most difficult to treat among all JIA cases. About 50% of sJIA cases did not respond to traditional disease modifying anti-rheumatic drugs (DMAR...Background: Systemic JIA (sJIA) is one of the subtypes of JIA, which is most difficult to treat among all JIA cases. About 50% of sJIA cases did not respond to traditional disease modifying anti-rheumatic drugs (DMARDs)—metho-trexate (MTX). Thalidomide is an immunomodulating and anti-inflammatory drug that induces sustained improvement of refractory sJIA cases. Objectives: To evaluate the efficacy of thalidomide in refractory sJIA patients. Methods: This was a prospective interventional study carried out in the Paediatric Rheumatology and Immunology follow-up clinic run by the Department of Pediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka from January 2019 to July 2020. Twenty-five sJIA patients who were refractory to conventional DMARDs were included in this study. These patients were prescribed thalidomide at a dose of 3 - 5 mg/kg/day for six months and efficacy was assessed by using juvenile arthritis disease activity score (JADAS 27) at 12<sup>th</sup> and 24<sup>th</sup> weeks of treatment. Result: Active joint counts and ESR improvement were observed in 90.69%, 97.67% and 69.84%, 100% of sJIA patients respectively at 12<sup>th</sup> and 24<sup>th</sup> weeks of treatment. Improvement of physicians and parent global assessment of VAS were 77.56%, 97.43% and 70.62% and 96.04% respectively at 12<sup>th</sup> and 24<sup>th</sup> weeks of treatment. Improvement of the total score of JADAS-27 was 77.51% at 12th week and 97.52% at 24<sup>th</sup> of week follow-up which was statistically significant. Somnolence, constipation and paresthesia were found as common adverse effect in this study. Conclusion: Efficacy of thalidomide was assessed by JADAS 27 criteria showed significant improvement in refractory sJIA patients in this study. It may be concluded that Thalidomide is safe and effective as an adjunct therapy of refractory sJIA patients.展开更多
Introduction :Rectal polyps are well-circumscribed, sessile or pedunculated formations that develop on the digestive mucosa. Juvenile polyps are seen in 4% - 12% of cases during pediatric colonoscopies. In children, r...Introduction :Rectal polyps are well-circumscribed, sessile or pedunculated formations that develop on the digestive mucosa. Juvenile polyps are seen in 4% - 12% of cases during pediatric colonoscopies. In children, rectal bleeding is a frequent warning sign, often a recurrent bleed with no impact on general condition. Diagnosis is based on clinical, imaging and digestive investigations, but anatomopathological examination remains the only means of confirmation. There are a number of treatment options, ranging from abstention to surgical excision. We report two (2) cases of isolated hemorrhagic juvenile polyp prolapsed to the anus in order to analyze the diagnostic and therapeutic features of this pathology. Patients and observations: A 7-year-old female patient presented to the pediatric emergency department of the Donka National Hospital with a hemorrhagic anal mass. On clinical examination, the patient was found to be in satisfactory general condition, with a hemorrhagic pedicle mass prolapsed to the anus. The mass was removed under general anesthesia. Postoperative management was straightforward. Conclusion: Juvenile polyps are the most common proctological condition in this age group. Clinical examination must be meticulous, as certain signs may point to a particular pathology. Colonoscopy is the diagnostic test of choice, and can also be used as a therapeutic tool.展开更多
BACKGROUND A giant juvenile fibroadenoma(GJF)is a rare,benign breast tumor that affects females<18 years of age.GJFs are generally suspected based on a palpable mass.GJFs influence breast shape and mammary gland de...BACKGROUND A giant juvenile fibroadenoma(GJF)is a rare,benign breast tumor that affects females<18 years of age.GJFs are generally suspected based on a palpable mass.GJFs influence breast shape and mammary gland development via the pressure effect from their enormous size.CASE SUMMARY Herein we report a case involving a 14-year-old Chinese female with a GJF in the left breast.GJF is a rare,benign breast tumor that usually occurs between 9 and 18 years of age and accounts for 0.5%-4.0%of all fibroadenomas.In severe cases,breast deformation may occur.This disease is rarely reported in Chinese people and has a high clinical misdiagnosis rate due to the absence of specific imaging features.On July 25,2022,a patient with a GJF was admitted to the First Affiliated Hospital of Dali University.The preoperative clinical examination and conventional ultrasound diagnosis needed further clarification.The mass was shown to be an atypical lobulated mass during the operation and confirmed to be a GJF based on pathologic examination.CONCLUSION GJF is also a rare,benign breast tumor in Chinese women.Evaluation of such masses consists of a physical examination,radiography,ultrasonography,computer tomography,and magnetic resonance imaging.GJFs are confirmed by histopathologic examination.Mastectomy is not selected when the patient benefits from a complete resection of the mass with breast reconstruction and an uneventful recovery.展开更多
BACKGROUND Juvenile polyposis syndrome(JPS)is a rare hereditary polyposis disease frequently associated with an autosomal-dominant variant of the SMAD4 or BMPR1A gene.It often manifests with symptoms in children and a...BACKGROUND Juvenile polyposis syndrome(JPS)is a rare hereditary polyposis disease frequently associated with an autosomal-dominant variant of the SMAD4 or BMPR1A gene.It often manifests with symptoms in children and adolescents and is infrequently diagnosed in asymptomatic adults.Establishing the diagnosis is important as patients with JPS have a high risk of developing gastrointestinal cancer and require genetic counselling and close routine follow-up.CASE SUMMARY We report on the case of a 56-year-old female diagnosed with JPS after genetic testing revealed a rare variant of the BMPR1A gene BMPR1A c.1409T>C(p.Met470Thr).She was initially referred for colonoscopy by her general practitioner after testing positive on a screening faecal immunochemical test and subsequently found to have polyposis throughout the entire colorectum on her index screening colonoscopy.The patient was asymptomatic with a normal physical examination and no related medical or family history.Blood tests revealed only mild iron deficiency without anemia.To date,there has only been one other reported case of JPS with the same genetic variant.Subsequent colonoscopies were organised for complete polyp clearance and the patient was returned for surveillance follow-up.CONCLUSION JPS patients can present with no prior symptoms or family history.Genetic testing plays an important diagnostic role guiding management.展开更多
BACKGROUND Patients with immune-mediated diseases,such as juvenile idiopathic arthritis(JIA)and inflammatory bowel disease(IBD)are at increased risk of developing infections,due to disease-related immune dysfunction a...BACKGROUND Patients with immune-mediated diseases,such as juvenile idiopathic arthritis(JIA)and inflammatory bowel disease(IBD)are at increased risk of developing infections,due to disease-related immune dysfunction and applying of immunosuppressive drugs.AIM To evaluate vaccine coverage in patients with IBD and JIA,and compare it with healthy children.METHODS In the cross-sectional study we included the data from a questionnaire survey of 190 Legal representatives of children with JIA(n=81),IBD(n=51),and healthy children(HC,n=58).An electronic online questionnaire was created for the survey.RESULTS There were female predominance in JIA patients and younger onset age.Parents of JIA had higher education levels.Employment level and family status were similar in the three studied groups.Patients with JIA and IBD had lower vaccine coverage,without parental rejection of vaccinations in IBD,compare to JIA and healthy controls.The main reason for incomplete vaccination was medical conditions in IBD and JIA.IBD patients had a lower rate of normal vaccine-associated reactions compared to JIA and HC.The encouraging role of physicians for vaccinations was the lowest in JIA patients.IBD patients had more possibilities to check antibodies before immune-suppressive therapy and had more supplementary vaccinations compared to JIA and HC.CONCLUSION JIA and IBD patients had lower vaccine coverage compared to HC.Physicians'encouragement of vaccination and the impossibility of discus about future vaccinations and their outcomes seemed the main factors for patients with immune-mediated diseases,influencing vaccine coverage.Further investigations are required to understand the reasons for incomplete vaccinations and improve vaccine coverage in both groups,especially in rheumatic disease patients.The approaches that stimulate vaccination in healthy children are not always optimal in children with immunemediated diseases.It is necessary to provide personalized vaccine-encouraging strategies for parents of chronically ill children with the following validation of these technics.展开更多
Background:Juvenile Localized Scleroderma(JLS)is a rare pediatric rheumatic disease characterized by inflammation and skin sclerosis.The side effect of consensus-recommended medications and the risk of disability pose...Background:Juvenile Localized Scleroderma(JLS)is a rare pediatric rheumatic disease characterized by inflammation and skin sclerosis.The side effect of consensus-recommended medications and the risk of disability posed challenges to the JLS treatment.We intend to demonstrate the potential of traditional Chinese medicine in treating JLS with skin ulcers and reducing the dose of glucocorticoid.Method:Here we report a case of a 13-year-old male with JLS who took oral methotrexate tablets of 10 mg/week and methylprednisolone of 6 mg/day for over six months without significant effect and suffered from skin ulcers on the dorsal feet one month after drug cessation.Subsequently,the patient was treated with integrated traditional Chinese and Western medicine of low-dose glucocorticosteroid,adjusted Shenqi Huoxue formula and Jinshe Xiaoyan formula,etc.Results:After integrated treatment,the patient’s dorsal feet ulcers healed and the skin sclerosis and hyperpigmentation improved significantly.Conclusions:This case report suggests that integrated traditional Chinese and Western medicine can be used as an effective treatment for JLS.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
The effects of apple dwarf stocks P22 and SH3 on the juvenile phase of Fuji seedlings were studied. The results showed that the juvenile phase could be shortened by more than two years when 2-year seedlings were graft...The effects of apple dwarf stocks P22 and SH3 on the juvenile phase of Fuji seedlings were studied. The results showed that the juvenile phase could be shortened by more than two years when 2-year seedlings were grafted on the dwarf stocks. The effects of P22 and SH3 were significantly different. Grafted on P22, the flowering node was less, the first flowing site was lower, and the juvenile phase was shorter than SH3. With the two stocks, the average first flowering site was more than 200 cm, the average flowering node was more than 100, and the lateral branch top bud was more than 50% in all first flowing seedlings.展开更多
[Objective] The paper aims to study the attractive effects of 12 Chinese medicinal herbs on Scophthalmus maximus juvenile.[Method] We took 200 Scophthalmus maximus with the average body length of 3 cm,average weight o...[Objective] The paper aims to study the attractive effects of 12 Chinese medicinal herbs on Scophthalmus maximus juvenile.[Method] We took 200 Scophthalmus maximus with the average body length of 3 cm,average weight of 1 g and 60 days of age as the research object,and 12 Chinese medicinal herbs as feed attractants were selected,including Cloves,Cinnamon,Star anise,Kaempferol,Mint,Coptis,Phellodendron,skullcap,Gardenia,Sophora flavescens,Mume,Myrobalan meat were selected.The amount added to the basic diet was 2‰..The times of turbot juveniles going into each inducing room and the times of turbot juveniles biting the feed ball were recorded by maze experiments.Inducing effects of the Chinese herbal medicines were analyzed.[Result] The results showed that the attractant effect of Mume,Gardenia,Scutellaria and kaempferol had the significantly inducing effect on Scophthalmus maximus juvenile.And 2‰ Gardenia,5‰ Matrine,5‰ Scutellaria and 2‰ Kaempferol have the best attractant effects.In compound experients,the attractive effect of mixture of 2‰ matrine and gardenia on Scophthalmus maximus was the best.[Conclusion] The attractive effect on Scophthalmus maximus has something to do with the kind and concentration of Chinese medicinal herbs.展开更多
文摘AIM:To investigate the association between juvenile open angle glaucoma(JOAG)and mental health among Koreans.METHODS:This study used nationally representative data from the 8th Korea National Health and Nutrition Examination Survey(KNHANES)2021.Glaucoma diagnosis followed the International Society of Geographical and Epidemiological Ophthalmology criteria based on glaucomatous structural defects,visual field defects,corrected vision,and intraocular pressure.As outcomes,suicidal behaviors,psychiatric counseling,and depression were evaluated through mental health questionnaires.Odds ratios(ORs)with 95%confidence intervals(CIs)were estimated using logistic regression models,adjusting for covariates.RESULTS:Among 7090 participants,3446 met the inclusion criteria for analysis,and 88(2.6%)were diagnosed with open angle glaucoma(OAG).After adjusting for age,sex,and best-corrected visual acuity(VA),participants with OAG were revealed to have significantly higher odds of suicidal behaviors(i.e.,ideation,planning,or attempts)compared with those without OAG(OR:2.70;95%CI:1.12-6.54;P=0.028).This association remained significant after further adjustments for socioeconomic status,lifestyle factors,and presence of chronic conditions(P=0.031 and 0.035,respectively).However,there was no significant difference for the other two outcomes,psychiatric counseling and depression.An age-stratified analysis revealed a stronger association between OAG and suicidal behaviors in younger JOAG participants(<40y)than in older OAG participants(≥40y;OR:3.80 vs 2.22;95%CI:0.79-18.22 vs 0.56-8.80,respectively).CONCLUSION:OAG patients show a higher risk of suicidal behaviors than those without glaucoma particularly in JOAG patients.
基金Counting at GTL funded by the Shenzhen Zhilan FoundationAlashan SEE Ecological Association+1 种基金Beijing Xianfeng FoundationCounting at PXL was funded by the Alashan SEE Chongqing Center
文摘Soaring bird migration often relies on suitable terrain and airflow;therefore,route selection is vital for successful migration.While age and experience have been identified as key factor influencing migration route selection among soaring raptors in the African-Eurasian Flyway,how they shape the migration route of soaring raptors in East Asia is still largely unknown.In this study,we investigated potential variations in the routes and timing in autumn migration of juvenile and older soaring birds,using count data of Greater Spotted Eagles(Clanga clanga)from two coastal sites and two inland sites in China.From 2020 to 2023,we recorded a total of 340 individuals,with the highest site averaging over 90 individuals per autumn,making it one of the world’s top single-season counts and thus a globally important site for this species.We found that 82% and 61% records from coastal sites were juveniles,significantly higher than inland sites(15% and 24%).Juveniles at all four sites exhibited markedly earlier median passage time than non-juveniles,with brief overlapping in their main migration periods.Both coastal sites are located on the tip of peninsulas stretching southwest,requiring long overwater flights if crossing the Bohai Bay or Beibu Gulf,which would be energetically demanding and increase mortality risk.Experienced individuals may have learned to avoid such terrain and subsequent detour,while juveniles are more prone to enter these peninsulas due to lack of experience and opportunities for social learning,or following other raptor species that are more capable of powered flight.Our findings highlight the importance of age and experience in migration route selection of large soaring birds.
文摘Background: Juvenile Idiopathic Arthritis (JIA) is the most prevalent rheumatic disease in children. It is associated with abnormal levels of serum zinc (Zn) and copper (Cu) as during inflammation serum copper concentration increases and zinc decreases. Objective: To assess the serum Zn and Cu levels in different sub-types of JIA patients and their correlation with the disease duration. Methods: This cross-sectional study was conducted over twelve months at the Pediatric Rheumatology Division, Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University. Sixty-nine JIA cases that fulfilled the International League of Association for Rheumatology (ILAR) criteria were taken as cases and age and sex-matched healthy children were considered as controls. The serum Zn and Cu tests were done using the spectrophotometric method with INDIKO PLUS Drug Analyzer. Data were recorded in a pre-designed questionnaire. Data were checked, verified and analyzed manually where continuous variables were analyzed using unpaired t-test and categorical variables using the ANOVA test. Pearson’s correlation coefficient test was used to see the correlation of serum zinc and copper levels with disease duration. Results: Boys were predominant in both case and control groups, with the majority within the 10 to 16-year-age group. Enthesitis-related arthritis (ERA) was the most common subtype followed by sJIA, Oligo JIA, Poly JIA (RF-) and unclassified subtypes. Disease duration was found less than 12 months in 30.4% of JIA patients. Serum analysis revealed a statistically significant reduction in mean zinc levels and increased copper levels in JIA patients compared to controls. This study observed a negative correlation between serum zinc levels and disease duration, whereas serum copper levels exhibited a positive correlation with disease duration. Conclusion: In conclusion, this study revealed that JIA patients exhibit alterations in serum zinc and copper levels. Serum copper levels showed a positive correlation and serum zinc levels showed a negative correlation with the duration of the disease.
文摘Juvenile xanthogranuloma (XGJ) is the most common form of non-Langerhansian histiocytosis. We report a pediatric case of multiple XGJ without visceral involvement in a 6-month-old female infant who was hospitalized with disseminated congenital skin nodules, of firm consistency, of variable diameter ranging from a few millimetres to 1 cm, involving the face, scalp, trunk and limbs. There was no mucosal localization. The remainder of the somatic examination was unremarkable. The diagnosis of disseminated XGJ was confirmed by skin biopsy without visceral or systemic involvement. Therapeutic abstention was recommended. This observation underlines the fact that XGJ is a rare clinical entity that should not be overlooked when faced with congenital skin nodules.
基金National High Level Hospital Clinical Research Funding,No.2022-NHLHCRF-LX-02-0101.
文摘BACKGROUND Juvenile hemochromatosis(JH)is an early-onset,rare autosomal recessive disorder of iron overload observed worldwide that leads to damage in multiple organs.Pathogenic mutations in the hemojuvelin(HJV)gene are the major cause of JH.CASE SUMMARY A 34-year-old male Chinese patient presented with liver fibrosis,diabetes,hypogonadotropic hypogonadism,hypophysis hypothyroidism,and skin hyperpigmentation.Biochemical test revealed a markedly elevated serum ferritin level of 4329μg/L and a transferrin saturation rate of 95.4%.Targeted exome sequencing and Sanger sequencing revealed that the proband had a novel mutation c.863G>A(p.R288Q)in the HJV gene which was transmitted from his father,and two known mutations,c.18G>C(p.Q6H)and c.962_963delGCinsAA(p.C321*)in cis,which were inherited from his mother.The p.R288W mutation was previously reported to be pathogenic for hemochromatosis,which strongly supported the pathogenicity of p.R288Q reported for the first time in this case.After 72 wk of intensive phlebotomy therapy,the patient achieved a reduction in serum ferritin to 160.5μg/L.The patient's clinical symptoms demonstrated a notable improvement.CONCLUSION This study highlights the importance of screening for hemochromatosis in patients with diabetes and hypogonadotropic hypogonadism.It also suggests that long-term active phlebotomy could efficiently improve the prognosis in severe JH.
文摘This study focused on the supplementation of four-sided spice fruit powder (Tetrapleura tetraptera) on some zootechnical performances of juveniles of Clarias gariepinus and was carried out from February 14 to July 20 at the Massoma Fish Farm of Bojongo Mbeidi located in the Littoral Region, Department of Wouri, Douala IV district. The farm benefits from a climate favorable to aquaculture activity. For this purpose, five (5) treatments were tested including T<sub>0+</sub> (imported feed), T<sub>0-</sub>, T<sub>0.3</sub>, T<sub>0.4</sub>, and T<sub>0.5</sub> respectively for the treatments with 0% supplemented food;0.3%;0.4% and 0.5% of Tetrapleura tetraptera powder. A total of 450 Clarias gariepinus fry with an initial average weight of 7 ± 1.5 g, were distributed in happas of 0.49 m<sup>2</sup> each placed in Triplicate in a completely randomized device. Four isoprotein foods with 38% crude protein were formulated to feed these fry at 7% of their biomass per day for 15 days then to their fullness until the end of the experiment. Intermediate fishing was carried out every fortnight. 75 days later, the results recorded show that the highest average survival rate (96.44% ± 3.5%) was recorded with the subjects fed feed supplemented with 0.4% T. tetraptera powder. The average weight (120.93 ± 67.20), weight gain (113.93 ± 67.20), specific growth rate (3.64 ± 0.79% g/d) and total lengths and standard (26.43 ± 1.9 and 23.66 ± 1.76) the highest were recorded with the T<sub>0+</sub> treatment (imported feed). The highest conditioning factor K (2.14 ± 0.15) was recorded with the treatment supplemented with 0.4% T. tetraptera. The lowest conversion ratio (1.28) was recorded with the treatment fed with imported blue crown food (T<sub>0+</sub>). Furthermore, the lowest production cost was recorded with the treatment supplemented with 0.4% T. tetraptera. It appears from this study that the juveniles of C. gariepinus better value the feed substituted with 0.4% of the powder of four sides (Tetrapleura tetraptera).
基金Supported by the Science and Technology Plan Project of Jingmen Science and Technology Bureau,No.2018YFZD025。
文摘BACKGROUND Various non-steroidal anti-inflammatory drugs(NSAIDs)have been used for juvenile idiopathic arthritis(JIA).However,the optimal method for JIA has not yet been developed.AIM To perform a systematic review and network meta-analysis to determine the optimal instructions.METHODS We searched for randomized controlled trials(RCTs)from PubMed,EMBASE,Google Scholar,CNKI,and Wanfang without restriction for publication date or language at August,2023.Any RCTs that comparing the effectiveness of NSAIDs with each other or placebo for JIA were included in this network meta-analysis.The surface under the cumulative ranking curve(SUCRA)analysis was used to rank the treatments.P value less than 0.05 was identified as statistically significant.RESULTS We included 8 RCTs(1127 patients)comparing 8 different instructions including meloxicam(0.125 qd and 0.250 qd),Celecoxib(3 mg/kg bid and 6 mg/kg bid),piroxicam,Naproxen(5.0 mg/kg/d,7.5 mg/kg/d and 12.5 mg/kg/d),inuprofen(30-40 mg/kg/d),Aspirin(60-80 mg/kg/d,75 mg/kg/d,and 55 mg/kg/d),Tolmetin(15 mg/kg/d),Rofecoxib,and placebo.There were no significant differences between any two NSAIDs regarding ACR Pedi 30 response.The SUCRA shows that celecoxib(6 mg/kg bid)ranked first(SUCRA,88.9%),rofecoxib ranked second(SUCRA,68.1%),Celecoxib(3 mg/kg bid)ranked third(SUCRA,51.0%).There were no significant differences between any two NSAIDs regarding adverse events.The SUCRA shows that placebo ranked first(SUCRA,88.2%),piroxicam ranked second(SUCRA,60.5%),rofecoxib(0.6 mg/kg qd)ranked third(SUCRA,56.1%),meloxicam(0.125 mg/kg qd)ranked fourth(SUCRA,56.1%),and rofecoxib(0.3 mg/kg qd)ranked fifth(SUCRA,56.1%).CONCLUSION In summary,celecoxib(6 mg/kg bid)was found to be the most effective NSAID for treating JIA.Rofecoxib,piroxicam,and meloxicam may be safer options,but further research is needed to confirm these findings in larger trials with higher quality studies.
文摘A recent study published in World J Clin Cases addressed the optimal non-steroidal anti-inflammatory drugs(NSAIDs)for juvenile idiopathic arthritis(JIA).Herein,we outline the progress in drug therapy of JIA.NSAIDs have traditionally been the primary treatment for all forms of JIA.NSAIDs are symptom-relief medications,and well tolerated by patients.Additionally,the availability of selective NSAIDs further lower the gastrointestinal adverse reactions compared with traditional NSAIDs.Glucocorticoid is another kind of symptom-relief medications with potent anti-inflammatory effect.However,the frequent adverse events limit the clinical use.Both NSAIDs and glucocorticoid fail to ease or pre-vent joint damage,and the breakthrough comes along with the disease-mo-difying antirheumatic drugs(DMARDs).DMARDs can prevent disease pro-gression and reduce joint destruction.Particularly,the emergence of biologic DMARDs(bDMARDs)has truly revolutionized the therapeutics of JIA,compared with conventional synthetic DMARDs.As a newly developed class of drugs,the places of most bDMARDs in the management of JIA remain to be well estab-lished.Nevertheless,the continuous evolution of bDMARDs raises hopes of improving long-term disease outcomes for JIA.
基金Supported by Ministry of Science and Higher Education of the Russian Federation,No.075-15-2022-301.
文摘BACKGROUND Previous studies in the pre-biological era showed an association of wrist inflammation in juvenile idiopathic arthritis(JIA)with progressive disease course,polyarticular involvement and failure of methotrexate treatment.AIM To describe features of JIA,associated with wrist arthritis.METHODS Data from about 753 JIA patients were included in this retrospective cohort study.The clinical and laboratory features of patients with and without wrist involvement were analyzed.RESULTS Wrist involvement was found in oligoarthritis(5.8%),RF(−)/RF(+)polyarthritis(44.9%/15.0%),enthesitis-related arthritis(17.7%),and systemic(58.6%)JIA categories.Unilateral wrist involvement was typical for oligoarthritis patients,bilateral involvement was either equal to that of unilateral involvement or was more frequent in other categories.Wrist arthritis was found to be associated with female sex,a low incidence of uveitis,and more indications of systemic inflammation,including elevated levels of C-reactive protein,erythrocyte sedimentation rate,and platelets,as well as involvement of the cervical spine,temporomandibular,shoulder,elbow,metacarpophalangeal,proximal interphalangeal,distal interphalangeal,hip,ankle,and tarsus arthritis.The number of patients with hip osteoarthritis and hip replacement was also higher.Wrist arthritis was associated with a lower probability of achieving remission[hazard ratio(HR)=1.3(95%CI:1.0-1.7),P=0.055],and a higher probability of being treated with biologics[HR=1.7(95%CI:1.3-2.10,P=0.00009)].CONCLUSION Wrist arthritis in JIA patients is a marker of a severe disease course,characterized by more intensive inflammation,unfavorable outcomes,and.requiring more intensive treatment with early administration of biologics.Close monitoring of wrist inflammation with ultrasound and MR assessment with early biological treatment might improve the outcomes.
基金Supported by the Ministry of Science and Higher Education of the Russian Federation,No.075-15-2022-301.
文摘BACKGROUND Lung damage in systemic juvenile arthritis(sJIA)is one of the contemporary topics in pediatric rheumatology.Several previous studies showed the severe course and fatal outcomes in some patients.The information about interstitial lung disease(ILD)in the sJIA is scarce and limited to a total of 100 cases.AIM To describe the features of sJIA patients with ILD in detail.METHODS In the present retrospective cohort study,information about 5 patients less than 18-years-old with sJIA and ILD were included.The diagnosis of sJIA was made according to the current 2004 and new provisional International League of Associations for Rheumatology criteria 2019.ILD was diagnosed with chest computed tomography with the exclusion of other possible reasons for concurrent lung involvement.Macrophage activation syndrome(MAS)was diagnosed with HLH-2004 and 2016 EULAR/ACR/PRINTO Classification Criteria and hScores were calculated during the lung involvement.RESULTS The onset age of sJIA ranged from 1 year to 10 years.The time interval before ILD ranged from 1 mo to 3 years.The disease course was characterized by the prevalence of the systemic features above articular involvement,intensive rash(100%),persistent and very active MAS(hScore range:194-220)with transaminitis(100%),and respiratory symptoms(100%).Only 3 patients(60%)developed a clubbing phenomenon.All patients(100%)had pleural effusion and 4 patients(80%)had pericardial effusion at the disease onset.Two patients(40%)developed pulmonary arterial hypertension.Infusion-related reactions to tocilizumab were observed in 3(60%)of the patients.One patient with trisomy 21 had a fatal disease course.Half of the remaining patients had sJIA remission and 2 patients had improvement.Lung disease improved in 3 patients(75%),but 1 of them had initial deterioration of lung involvement.One patient who has not achieved the sJIA remission had the progressed course of ILD.No cases of hyper-eosinophilia were noted.Four patients(80%)received canakinumab and one(20%)tocilizumab at the last follow-up visit.CONCLUSION ILD is a severe life-threatening complication of sJIA that may affect children of different ages with different time intervals since the disease onset.Extensive rash,serositis(especially pleuritis),full-blown MAS with transaminitis,lymphopenia,trisomy 21,eosinophilia,and biologic infusion reaction are the main predictors of ILD.The following studies are needed to find the predictors,pathogenesis,and treatment options,for preventing and treating the ILD in sJIA patients.
文摘Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease deadly. In Africa, little data exists on this condition and the form familial is the most common with a younger age of onset. Our observations relate to three brothers, born from a consanguineous marriage. The diagnosis of amyotrophic lateral sclerosis was certain placed in all patients according to the criteria of Awaji. The average age of patients was 16.66 years, and the average age of onset of symptoms was 10.33 years old. The female gender was the most represented with a sex ratio of 0.5. Our patients benefited from rilusole, vitamin D and physiotherapy. A only patient presented with dysphagia. However, no cases have been observed at this day.
文摘Background: Systemic JIA (sJIA) is one of the subtypes of JIA, which is most difficult to treat among all JIA cases. About 50% of sJIA cases did not respond to traditional disease modifying anti-rheumatic drugs (DMARDs)—metho-trexate (MTX). Thalidomide is an immunomodulating and anti-inflammatory drug that induces sustained improvement of refractory sJIA cases. Objectives: To evaluate the efficacy of thalidomide in refractory sJIA patients. Methods: This was a prospective interventional study carried out in the Paediatric Rheumatology and Immunology follow-up clinic run by the Department of Pediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka from January 2019 to July 2020. Twenty-five sJIA patients who were refractory to conventional DMARDs were included in this study. These patients were prescribed thalidomide at a dose of 3 - 5 mg/kg/day for six months and efficacy was assessed by using juvenile arthritis disease activity score (JADAS 27) at 12<sup>th</sup> and 24<sup>th</sup> weeks of treatment. Result: Active joint counts and ESR improvement were observed in 90.69%, 97.67% and 69.84%, 100% of sJIA patients respectively at 12<sup>th</sup> and 24<sup>th</sup> weeks of treatment. Improvement of physicians and parent global assessment of VAS were 77.56%, 97.43% and 70.62% and 96.04% respectively at 12<sup>th</sup> and 24<sup>th</sup> weeks of treatment. Improvement of the total score of JADAS-27 was 77.51% at 12th week and 97.52% at 24<sup>th</sup> of week follow-up which was statistically significant. Somnolence, constipation and paresthesia were found as common adverse effect in this study. Conclusion: Efficacy of thalidomide was assessed by JADAS 27 criteria showed significant improvement in refractory sJIA patients in this study. It may be concluded that Thalidomide is safe and effective as an adjunct therapy of refractory sJIA patients.
文摘Introduction :Rectal polyps are well-circumscribed, sessile or pedunculated formations that develop on the digestive mucosa. Juvenile polyps are seen in 4% - 12% of cases during pediatric colonoscopies. In children, rectal bleeding is a frequent warning sign, often a recurrent bleed with no impact on general condition. Diagnosis is based on clinical, imaging and digestive investigations, but anatomopathological examination remains the only means of confirmation. There are a number of treatment options, ranging from abstention to surgical excision. We report two (2) cases of isolated hemorrhagic juvenile polyp prolapsed to the anus in order to analyze the diagnostic and therapeutic features of this pathology. Patients and observations: A 7-year-old female patient presented to the pediatric emergency department of the Donka National Hospital with a hemorrhagic anal mass. On clinical examination, the patient was found to be in satisfactory general condition, with a hemorrhagic pedicle mass prolapsed to the anus. The mass was removed under general anesthesia. Postoperative management was straightforward. Conclusion: Juvenile polyps are the most common proctological condition in this age group. Clinical examination must be meticulous, as certain signs may point to a particular pathology. Colonoscopy is the diagnostic test of choice, and can also be used as a therapeutic tool.
文摘BACKGROUND A giant juvenile fibroadenoma(GJF)is a rare,benign breast tumor that affects females<18 years of age.GJFs are generally suspected based on a palpable mass.GJFs influence breast shape and mammary gland development via the pressure effect from their enormous size.CASE SUMMARY Herein we report a case involving a 14-year-old Chinese female with a GJF in the left breast.GJF is a rare,benign breast tumor that usually occurs between 9 and 18 years of age and accounts for 0.5%-4.0%of all fibroadenomas.In severe cases,breast deformation may occur.This disease is rarely reported in Chinese people and has a high clinical misdiagnosis rate due to the absence of specific imaging features.On July 25,2022,a patient with a GJF was admitted to the First Affiliated Hospital of Dali University.The preoperative clinical examination and conventional ultrasound diagnosis needed further clarification.The mass was shown to be an atypical lobulated mass during the operation and confirmed to be a GJF based on pathologic examination.CONCLUSION GJF is also a rare,benign breast tumor in Chinese women.Evaluation of such masses consists of a physical examination,radiography,ultrasonography,computer tomography,and magnetic resonance imaging.GJFs are confirmed by histopathologic examination.Mastectomy is not selected when the patient benefits from a complete resection of the mass with breast reconstruction and an uneventful recovery.
文摘BACKGROUND Juvenile polyposis syndrome(JPS)is a rare hereditary polyposis disease frequently associated with an autosomal-dominant variant of the SMAD4 or BMPR1A gene.It often manifests with symptoms in children and adolescents and is infrequently diagnosed in asymptomatic adults.Establishing the diagnosis is important as patients with JPS have a high risk of developing gastrointestinal cancer and require genetic counselling and close routine follow-up.CASE SUMMARY We report on the case of a 56-year-old female diagnosed with JPS after genetic testing revealed a rare variant of the BMPR1A gene BMPR1A c.1409T>C(p.Met470Thr).She was initially referred for colonoscopy by her general practitioner after testing positive on a screening faecal immunochemical test and subsequently found to have polyposis throughout the entire colorectum on her index screening colonoscopy.The patient was asymptomatic with a normal physical examination and no related medical or family history.Blood tests revealed only mild iron deficiency without anemia.To date,there has only been one other reported case of JPS with the same genetic variant.Subsequent colonoscopies were organised for complete polyp clearance and the patient was returned for surveillance follow-up.CONCLUSION JPS patients can present with no prior symptoms or family history.Genetic testing plays an important diagnostic role guiding management.
文摘BACKGROUND Patients with immune-mediated diseases,such as juvenile idiopathic arthritis(JIA)and inflammatory bowel disease(IBD)are at increased risk of developing infections,due to disease-related immune dysfunction and applying of immunosuppressive drugs.AIM To evaluate vaccine coverage in patients with IBD and JIA,and compare it with healthy children.METHODS In the cross-sectional study we included the data from a questionnaire survey of 190 Legal representatives of children with JIA(n=81),IBD(n=51),and healthy children(HC,n=58).An electronic online questionnaire was created for the survey.RESULTS There were female predominance in JIA patients and younger onset age.Parents of JIA had higher education levels.Employment level and family status were similar in the three studied groups.Patients with JIA and IBD had lower vaccine coverage,without parental rejection of vaccinations in IBD,compare to JIA and healthy controls.The main reason for incomplete vaccination was medical conditions in IBD and JIA.IBD patients had a lower rate of normal vaccine-associated reactions compared to JIA and HC.The encouraging role of physicians for vaccinations was the lowest in JIA patients.IBD patients had more possibilities to check antibodies before immune-suppressive therapy and had more supplementary vaccinations compared to JIA and HC.CONCLUSION JIA and IBD patients had lower vaccine coverage compared to HC.Physicians'encouragement of vaccination and the impossibility of discus about future vaccinations and their outcomes seemed the main factors for patients with immune-mediated diseases,influencing vaccine coverage.Further investigations are required to understand the reasons for incomplete vaccinations and improve vaccine coverage in both groups,especially in rheumatic disease patients.The approaches that stimulate vaccination in healthy children are not always optimal in children with immunemediated diseases.It is necessary to provide personalized vaccine-encouraging strategies for parents of chronically ill children with the following validation of these technics.
基金The essay is supported by Research Project of Shanghai Municipal Health Care Commission,No.20204Y0410We appreciate the patient and his parents for their cooperation and consent to disclose the case.We express our gratitude for the support from the members of the scientific innovation volunteer team of rare diseases in Shanghai TCM-Integrated School of clinical medicine,Shanghai University of Traditional Chinese Medicine.
文摘Background:Juvenile Localized Scleroderma(JLS)is a rare pediatric rheumatic disease characterized by inflammation and skin sclerosis.The side effect of consensus-recommended medications and the risk of disability posed challenges to the JLS treatment.We intend to demonstrate the potential of traditional Chinese medicine in treating JLS with skin ulcers and reducing the dose of glucocorticoid.Method:Here we report a case of a 13-year-old male with JLS who took oral methotrexate tablets of 10 mg/week and methylprednisolone of 6 mg/day for over six months without significant effect and suffered from skin ulcers on the dorsal feet one month after drug cessation.Subsequently,the patient was treated with integrated traditional Chinese and Western medicine of low-dose glucocorticosteroid,adjusted Shenqi Huoxue formula and Jinshe Xiaoyan formula,etc.Results:After integrated treatment,the patient’s dorsal feet ulcers healed and the skin sclerosis and hyperpigmentation improved significantly.Conclusions:This case report suggests that integrated traditional Chinese and Western medicine can be used as an effective treatment for JLS.
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.
文摘The effects of apple dwarf stocks P22 and SH3 on the juvenile phase of Fuji seedlings were studied. The results showed that the juvenile phase could be shortened by more than two years when 2-year seedlings were grafted on the dwarf stocks. The effects of P22 and SH3 were significantly different. Grafted on P22, the flowering node was less, the first flowing site was lower, and the juvenile phase was shorter than SH3. With the two stocks, the average first flowering site was more than 200 cm, the average flowering node was more than 100, and the lateral branch top bud was more than 50% in all first flowing seedlings.
文摘[Objective] The paper aims to study the attractive effects of 12 Chinese medicinal herbs on Scophthalmus maximus juvenile.[Method] We took 200 Scophthalmus maximus with the average body length of 3 cm,average weight of 1 g and 60 days of age as the research object,and 12 Chinese medicinal herbs as feed attractants were selected,including Cloves,Cinnamon,Star anise,Kaempferol,Mint,Coptis,Phellodendron,skullcap,Gardenia,Sophora flavescens,Mume,Myrobalan meat were selected.The amount added to the basic diet was 2‰..The times of turbot juveniles going into each inducing room and the times of turbot juveniles biting the feed ball were recorded by maze experiments.Inducing effects of the Chinese herbal medicines were analyzed.[Result] The results showed that the attractant effect of Mume,Gardenia,Scutellaria and kaempferol had the significantly inducing effect on Scophthalmus maximus juvenile.And 2‰ Gardenia,5‰ Matrine,5‰ Scutellaria and 2‰ Kaempferol have the best attractant effects.In compound experients,the attractive effect of mixture of 2‰ matrine and gardenia on Scophthalmus maximus was the best.[Conclusion] The attractive effect on Scophthalmus maximus has something to do with the kind and concentration of Chinese medicinal herbs.