Detour for the inexperienced? Migration count data suggest mostly juvenile Greater Spotted Eagles appear in coastal peninsulas in China

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.

Preliminary exploration of animal models of congenital choledochal cysts

BACKGROUND Various animal models have been used to explore the pathogenesis of choledochal cysts(CCs),but with little convincing results.Current surgical techniques can achieve satisfactory outcomes for treatment of CCs.Consequently,recent studies have focused more on clinical issues rather than basic research.Therefore,we need appropriate animal models to further basic research.AIM To establish an appropriate animal model that may contribute to the investigation of the pathogenesis of CCs.METHODS Eighty-four specific pathogen-free female Sprague-Dawley rats were randomly allocated to a surgical group,sham surgical group,or control group.A rat model of CC was established by partial ligation of the bile duct.The reliability of the model was confirmed by measurements of serum biochemical indices,morpho-logy of common bile ducts of the rats as well as molecular biology experiments in rat and human tissues.RESULTS Dilation classified as mild(diameter,≥1 mm to<3 mm),moderate(≥3 mm to<10 mm),and severe(≥10 mm)was observed in 17,17,and 2 rats in the surgical group,respectively,while no dilation was observed in the control and sham surgical groups.Serum levels of alanine aminotransferase,aspartate aminotrans-ferase,total bilirubin,direct bilirubin,and total bile acids were significantly elevated in the surgical group as compared to the control group 7 d after surgery,while direct bilirubin,total bilirubin,and gamma-glutamyltransferase were further increased 14 d after surgery.Most of the biochemical indices gradually decreased to normal ranges 28 d after surgery.The protein expression trend of signal transducer and activator of transcription 3 in rat model was consistent with the human CC tissues.CONCLUSION The model of partial ligation of the bile duct of juvenile rats could morphologically simulate the cystic or fusiform CC,which may contribute to investigating the pathogenesis of CC.

The biosynthesis of alarm pheromone in the wheat aphid Rhopalosiphum padi is regulated by hormones via fatty acid metabolism

Aphids are major insect pests in agriculture and forestry worldwide.Following attacks by natural enemies,many aphids release an alarm pheromone to protect their population.In most aphids,the main component of the aphid alarm pheromone(AAP)is the sesquiterpene hydrocarbon(E)-β-farnesene(EβF).However,the mechanisms behind its biosynthesis and regulation remain poorly understood.In this study,we used the bird cherry–oat aphid Rhopalosiphum padi,which is an important wheat aphid,to investigate the regulatory mechanisms of EβF biosynthesis.Our results showed that EβF biosynthesis occurs during the mature embryo period and the molting period of the 1st-and 2nd-instar nymphs.Triglycerides provide the prerequisite material for EβF production and release.Based on transcriptome sequencing,RNAi analysis,hormone treatments,and quantitative measurements,we found that the biosynthesis of EβF utilizes acetyl coenzyme A produced from fatty acid degradation,which can be suppressed by juvenile hormone but it is promoted by 20-hydroxyecdysone through the modulation of fatty acid metabolism.This is the first systemic study on the modulation of EβF production in aphids.The results of our study provide insights into the molecular regulatory mechanisms of AAP biosynthesis,as well as valuable information for designing potential aphid control strategies.

Novel compound heterozygous mutations in the hemojuvelin gene in a juvenile hemochromatosis patient:A case report

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.

Systematic review and network meta-analysis of different nonsteroidal anti-inflammatory drugs for juvenile idiopathic arthritis

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.

Systemic juvenile idiopathic arthritis–associated lung disease: A retrospective cohort study

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.

Juvenile Xanthogranuloma: A Case Report with Literature Review

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.

Serum Zinc and Copper Level in Juvenile Idiopathic Arthritis (JIA) Patients and Its Correlation with Disease Duration-A Tertiary Hospital Study

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.

Fruit Powder of Tetrapleura tetraptera in Local Feed on Some Zootechnical Performances of Juveniles of Clarias gariepinus in Concrete Tank

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 T0+ (imported feed), T0-, T0.3, T0.4, and T0.5 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 m2 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 T0+ 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 (T0+). 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).

Evaluation of total protein,peroxidase,and nutrients measured by pXRF for the determination of tissue rejuvenation/reinvigoration of Eucalyptus microcorys

Ontogenetic aging of tissues and the gradual decrease of adventitious rooting are known challenges for the clonal propagation of woody species,hampering clonal forestry programs.This study examined possible signatures of tissue rejuvenation/reinvigoration in different propagated materials of Eucalyptus microcorys by analyzing the total protein profile,peroxidase activity,macro-and micronutrient contents,and adventitious rooting of mini cuttings.The analyses were performed on E.microcorys shoots which were successfully obtained by seminal and grafting propagation,micropropagation with epicormic shoots,and indirect organogenesis.Among four mature trees used in the propagation,tissues from the one with the best propagation results were investigated for signs of tissue rejuvenation and/or reinvigoration.Five individuals from each technique were randomly selected and transferred to a semi-hydroponic"channel"system.After four weeks in the seedbed,the total protein,peroxidase activity,nutrient content and rooting of the mini cuttings were evaluated.SDS-PAGE enabled the differentiation of leaf samples obtained by grafting from the other propagation techniques,as revealed by two distinct bands.Materials obtained by micropropagation with epicormic shoots showed the highest peroxidase activity,while those obtained by seminal propagation and from the selected mature tree showed the lowest peroxidase activity.A portable X-ray fluorescence spectroscope(pXRF)identified adequate nutrient content in most of the nutrients tested in materials obtained by seminal and grafting propagation,and by indirect organogenesis.The analysis of adventitious rooting showed that the highest rooting percentage was observed in mini cuttings from seminal propagation(75%)followed by indirect organogenesis(35%).Based on principal component analysis,it was concluded that rooting of mini cuttings from both seminal propagation and indirect organogenesis was associated with phosphorous,sulphur,and potassium contents,which suggests a higher level of tissue rejuvenation/reinvigoration in these propagated plants.Further studies are recommended to search for other methods that present similarities with the responses to adventitious rooting in forest species and thus optimize the rescue and propagation of plants with distinct ontogenetic stages.

Hamartomatous polyps:Diagnosis,surveillance,and management

Hereditary polyposis syndrome can be divided into three categories:Adenomatous,serrated,and hamartomatous polyps.Hamartomatous polyps,malformations of normal tissue presenting in a disorganized manner,are characterized by an autosomal dominant inheritance pattern.These syndromes exhibit hamartomatous gastrointestinal polyps in conjunction to extra-intestinal manifestations,which require conscientious and diligent monitoring.Peutz-Jeghers syndrome,Cowden syndrome,and juvenile polyposis syndrome are the most common displays of hamartomatous polyposis syndrome(HPS).Diagnosis can be pursued with molecular testing and endoscopic sampling.Early identification of these autosomal dominant pathologies allows to optimize malignancy surveillance,which helps reduce morbidity and mortality in both the affected patient population as well as at-risk family members.Endoscopic surveillance is an important pillar of prognosis and monitoring,with many patients eventually requiring surgical intervention.In this review,we discuss the diagnosis,surveillance,and management of HPS.

Hereditary polyposis syndromes remain a challenging disease entity:Old dilemmas and new insights

In this editorial we present an overview and insights of the management of hereditary polyposis syndromes.The primary focus was on familial adenomatous polyposis,juvenile polyposis syndrome and Peutz-Jegher syndrome.Genetic testing has become increasingly available and is easier than ever to integrate into clinical practice.Furthermore,several genes have been added to the expanding list of genes associated with hereditary polyposis syndromes,allowing for precise diagnostics and tailored follow-up.Endoscopic evaluation of patients with hereditary polyposis syndromes is paramount in the surveillance strategies.Current endoscopic procedures include both diagnostic procedures and surveillance as well as therapeutic interventions.Recommendations for endoscopic procedures in the upper and lower gastrointestinal canal were described.Surgery is still a key component in the management of patients with hereditary polyposis syndromes.The increased cancer risk in these patients often render prophylactic procedures or intended curative procedures in the case of cancer development.Surgical interventions in the upper and lower gastrointestinal canal were described with relevant considerations.Development of chemopreventive medications is ongoing.Few drugs have been investigated,including nonsteroidal anti-inflammatory drugs.It has been demonstrated that cyclooxygenase-2 inhibitors may lower the number of polyps.Other medications are currently under investigation,but none have,to date,consistently been able to prevent development of disease.

Giant juvenile fibroadenoma in a 14-year old Chinese female:A case report

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.

Premature Puberty Revealing an Ovarian Tumor in a Five-Year-Old Girl

Background: Ovarian tumors in the girl child are sometimes revealed by the development of secondary sexual characteristics. The authors report the case of a five-year-old girl in whom the disease was revealed by early puberty. Case presentation: A five-year-old girl with an enlarged abdomen for about four months. The onset of pain and the sensation of a mass prompted the consultation. The development of secondary sexual characteristics (SSC) noted by the family had not been mentioned. The patient was classified as pubertal stage 2 according to the Tanner classification. An abdominal ultrasound and a CT scan showed a large left ovarian mass, an enlarged uterus for the patient’s age and a normal right ovary. The hormonal workup was not contributive. The treatment consisted only of a left salpingo-ovarectomy, without complementary chemotherapy. Anatomic pathological examination of the surgical specimen concluded to a juvenile tumor of the granulosa. The evolution was good with a beginning of regression of the HSC one month after the ovarectomy. Discussion: Granulosa tumors are sometimes secretory cancers, generally with a low potential for malignancy and therefore a very good prognosis. Surgery based on total adnexectomy is the first-line treatment. The large size of the tumor, the presence of ascites and capsular rupture are factors of poor prognosis, hence the importance of early diagnosis. Conclusion: Routine comprehensive physical examination should be de rigueur for abdominal masses in girls, especially in the context of various beliefs that may impede early referral to care.

Polyposis found on index colonoscopy in a 56-year-old female-BMPR1A variant in juvenile polyposis syndrome:A case report

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.

Familial Lateral Sclerosis with Juvenile Onset: About 3 Cases and Review of the Literature

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.

Thalidomide Is an Adjunct Therapy for the Refractory Systemic Juvenile Idiopathic Arthritis Patients in a Tertiary Hospital Study

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 12th and 24th 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 12th and 24th 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 12th and 24th weeks of treatment. Improvement of the total score of JADAS-27 was 77.51% at 12th week and 97.52% at 24th 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.

Prolapsed Juvenile Polyp of the Anus in 2 Cases

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.

Vaccination coverage in children with juvenile idiopathic arthritis,inflammatory bowel diseases,and healthy peers:Cross-sectional electronic survey data

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.

Can the Glucose Central Control System Dysfunctions Induce Diabetes Mellitus?

We study afresh how the glucose control system anomalies impact the organicity of the glucose homeostasis and build up events of persistent hyperglycemia and diabetes mellitus. We have used critically the state of art literature related to the subject, in order to cross, to compare, and to organize the relevant contents to create a logical and consistent support to the finds. We show that it is consistent to assume that persistent hyperglycemia and diabetes mellitus can have precursors not only in pancreas, but also in brain, mainly induced by noxious dysfunctions of hypothalamus sensor neurons circuits and external noxious elements, causing pancreas overload, and the consequent exhaustion—overburden.