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Pediatric and adult liver transplantation in Bahrain: The experiences in a country with no available liver transplant facilities

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World Journal of Transplantation 2024年第1期14卷 96-111页

作者： Hasan M Isa Fatema A Alkharsi Jawad K Khamis Sawsan A Hasan Zainab A Naser Zainab N Mohamed Afaf M Mohamed Shaikha A Altamimi department of pediatrics Salmaniya Medical ComplexManama 26671Bahrain department of pediatrics Arabian Gulf UniversityManama 26671Bahrain department of Medicine Salmaniya Medical ComplexManama 26671Bahrain Public Health Directorate Ministry of HealthManama 26671Bahrain The Overseas Office Supreme Committee for Treatment AbroadMinistry of HealthManama 26671Bahrain

BACKGROUND Liver transplantation(LT)is a life-saving procedure for patients with end-stage liver disease and has become the standard and most effective treatment method for these *** are many indications for LT that vary between countries and *** outcome of LT depends on the available facilities and surgical expertise,as well as the types of liver graft donors *** To assess the clinical characteristics of patients from Bahrain who underwent LT overseas,and analyze factors affecting their *** In this retrospective cohort study,we reviewed the medical records and overseas committee registry information of all pediatric and adult patients who were sent overseas to undergo LT by the Pediatric and Medical departments of Salmaniya Medical Complex and Bahrain Defence Force Hospital via the Overseas Treatment Office,Ministry of Health,Kingdom of Bahrain,between 1997 and *** data,LT indication,donor-recipient relationship,overseas LT center,graft type,post-LT medications,and LT complications,were *** measured included the overall and 5-year LT survival ***’s exact,Pearsonχ2,and Mann-Whitney U tests were used to compare the pediatric and the adults’group in terms of clinical characteristics,donor-recipient relationship,medication,complications,and *** analysis was estimated via the Kaplan-Meier’s *** and multivariate analyses were used to detect predictors of *** Of the 208 eligible patients,170(81.7%)were sent overseas to undergo LT while 38(18.3%)remained on the waiting *** the 170 patients,167(80.3%)underwent LT and were included in the *** majority of the patients were Bahraini(91.0%),and most were males(57.5%).One-hundred-and-twenty(71.8%)were adults and 47(28.3%)were *** median age at transplant was 50.0[interquartile range(IQR):14.9–58.4]*** main indication for pediatric LT was biliary atresia(31.9%),while that of adult LT was

关键词： Overseas liver transplantation End-stage liver disease Liver transplant facilities Liver donor Biliary atresia Hepatitis C

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Efficacy of cognitive-behavioral therapy combined with habit reversal training on anxiety disorders in children with Tourette’s syndrome

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World Journal of Psychiatry 2024年第7期14卷 999-1008页

作者： Yan-Zhen Wang Xi Zhang Xin-Min Han department of Rehabilitation Children’s Hospital of ShanxiTaiyuan 030012Shanxi ProvinceChina department of pediatrics The Affiliated Hospital of Nanjing University of Chinese MedicineNanjing 210029Jiangsu ProvinceChina

BACKGROUND Cognitive-behavioral therapy(CBT)and habit reversal training(HRT)have shown application potential in addressing tic symptoms and comorbid psychiatric *** their theoretical potential,empirical evidence on their combined efficacy remains *** To evaluate the efficacy of CBT combined with HRT on anxiety disorders in children with Tourette’s syndrome(TS).METHODS Clinical data of children with TS admitted to our hospital from January 2022 to June 2023 were collected,and the patients were grouped into the conventional therapy(control)group and the CBT combined with HRT *** charac-teristics,anxiety scores,tic severity scores,treatment adherence,and parental satisfaction were *** analysis was performed using t-tests,chi-square tests,and correlation *** A total of 136 patients,including 65 patients in the control group and 71 patients in the CBT combined with HRT group,were *** CBT combined with HRT group showed remarkable improvements compared with the control ***-intervention assessment revealed a decrease in anxiety scores from 63.52±1.81 to 40.53±1.64(t=2.022,P=0.045),and the Yale Global Tic Severity Scale total score decreased from 22.14±5.67 to 16.28±4.91(t=2.288,P=0.024).Treatment adherence was significantly higher in the CBT combined with HRT group(85.47±7.62%)compared with the control group(82.32±6.54%;t=2.596,P=0.010).Parental satisfaction scores were also higher in the CBT combined with HRT group(8.69±1.77)compared with the control group(7.87±1.92;t=2.592,P=0.011).CONCLUSION This study demonstrates that CBT combined with HRT significantly reduces anxiety symptoms and tic severity in children with TS,with higher treatment adherence and parental *** findings support the potential application of this comprehensive therapeutic approach for TS treatment.

关键词： Tourette's syndrome Cognitive-behavioral therapy Habit reversal training Efficacy evaluation Anxiety disorders

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Perinatal outcomes of re-recurrent gestational diabetes mellitus and their prognostic factors:A retrospective cohort study in China

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Chinese Medical Journal 2024年第4期137卷 499-501页

作者： Xia Xu Yanni Guo Tingting Liao Huale Zhang Rumeng Jiang Shuyao Huang Jianying Yan department of Obstetrics and Gynecology Maternity and Child Health Hospital College of Clinical Medicine for Obstetrics&Gynecology and PediatricsFujian Medical UniversityFuzhouFujian 350001China

To the Editor:Gestational diabetes mellitus(GDM)is a pathologic condition in which glucose intolerance and insulin resistance develop to different degrees during *** with GDM have higher odds of adverse maternal and fetal *** with a history of GDM were at a significantly increased risk for recurrence of GDM in subsequent *** previous and some other studies found an increased incidence of adverse perinatal outcomes in pregnant with recurrent GDM.^([1])So far,women with a prior history of GDM are not recommended to refrain from subsequent pregnancy in light of the limited available *** about those women with a history of recurrent GDM?Should women with recurrent GDM be recommended to avoid further pregnancy?Currently,studies on perinatal outcomes of women with re-recurrent GDM and their prognostic factors are very ***,this retrospective cohort study was performed to explore perinatal outcomes of pregnant woman with re-recurrent GDM and their prognostic factors.

关键词： mellitus pregnancy diabetes

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Clinical Analysis and Mental Health Survey of Hemophilia Carriers:a Cross-sectional Study

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Current Medical Science 2024年第2期44卷 435-440页

作者： Wen WANG Li-juan JIANG Dong-yan CUI Ai ZHANG Xiong WANG Ai-guo LIU Qun HU department of pediatrics Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430030China department of Laboratory Medicine Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430030China

Objective:Hemophilia carriers(HCs),who are heterozygous for mutations in the clotting factor VIII/clotting factor IX gene(F8 or F9),may have a wide range of clotting factor levels,from very low,similar to afflicted males,to the upper limit of normal,and may experience mental health *** purpose of this study was to provide genetic information on mothers of hemophilia patients and to understand the clotting factor activity and phenotype of ***,we aimed to investigate the mental health status of HCs in ***:A total of 127 hemophilia mothers,including 93 hemophilia A(HA)mothers and 34 hemophilia B(HB)mothers,were enrolled in this *** distance PCR,multiplex PCR,and Sanger sequencing were used to analyze mutations in F8 or *** factor activity was detected by a one-stage clotting *** Symptom Checklist 90(SCL-90,China/Mandarin version)was given to HCs at the same time to assess their mental ***:A total of 90.6%of hemophilia mothers were diagnosed genetically as carriers,with inversion in intron 22 and missense mutations being the most common mutation types in HA and HB carriers,*** median clotting factor level in carriers was 0.74 IU/mL(ranging from 0.09 to 1.74 IU/mL)compared with 1.49 IU/mL(ranging from 0.93 to 1.89 IU/mL)in noncarriers,of which 14.3%of HCs had clotting factor levels of 0.40 IU/mL or below.A total of 53.8%(7/13)of HA carriers with low clotting factor levels(less than 0.50 IU/mL)had a history of bleeding,while none of the HB carriers displayed a bleeding *** total mean score and the global severity index of the SCL-90 for surveyed HCs were 171.00(±60.37)and 1.78(±0.59),respectively.A total of 67.7%of the respondents had psychological symptoms,with obsessive-compulsive disorder being the most prevalent and *** pooled estimates of all nine factors were significantly higher than those in the general population(P<0.05).Conclusions:The detection rate of gene mutations

关键词： hemophilia A hemophilia B mutation carrier mental health

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Prevalence of hypertension and its associations with body composition across Chinese and American children and adolescents

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World Journal of pediatrics 2024年第4期20卷 392-403页

作者： Li-Wang Gao Yi-Wen Huang Hong Cheng Xi Wang Hong-Bo Dong Pei Xiao Yin-Kun Yan Xin-Ying Shan Xiao-Yuan Zhao Jie Mi Center for Non-Communicable Disease Management Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthNo.56 Nanlishi RoadXicheng DistrictBeijing100045China department of Epidemiology Capital Institute of PediatricsNo.2 Yabao RoadChaoyang DistrictBeijing100020China Key Laboratory of Major Diseases in Children Ministry of EducationBeijingChina

Background The age of onset of hypertension(HTN)is decreasing,and obesity is a significant risk *** prevalence and racial disparities in pediatric HTN and the association between body composition and blood pressure are insufficiently *** study aimed to evaluate the prevalence of HTN in Chinese and American children and adolescents and to assess the relationship between various body composition indices and *** Seven thousand,five hundred and seventy-three Chinese and 6239 American children and adolescents aged 8–18 years from the 2013–2015 China Child and Adolescent Cardiovascular Health study and the 2011–2018 National Health and Nutrition Examination Surveys were *** pressure and body composition(fat and muscle)were measured by trained *** crude prevalence and age-standardized prevalence rate(ASPR)of primary HTN and its subtypes[isolated systolic hypertension(ISH)and isolated diastolic hypertension(IDH)]were calculated based on 2017 American Academy of pediatrics ***-adjusted linear regression coefficients and odds ratios(ORs)were calculated to assess the associations of body composition indicators with HTN,ISH and *** The ASPR of HTN was 18.5%in China(CN)and 4.6%in the United States(US),whereas the obesity prevalence was 7.4%and 18.6%,and the population attributable risk of HTN caused by overweight and obesity was higher in the US than in *** fat mass,muscle mass and body fat percentage mass were associated with a higher risk of HTN in both *** percent of muscle body mass had a protective effect on HTN and ISH in both countries[HTN(CN:OR=0.83,95%CI=0.78–0.88;US:OR=0.72,95%CI=0.64–0.81);ISH(CN:OR=0.87,95%CI=0.80–0.94;US:OR=0.71,95%CI=0.62–0.81)],and the protective effect was more common among children and adolescents with high levels of physical *** The burden of HTN in Chinese children and adolescents was substantial and much greater than that in the US,and th

关键词： Body composition Children and adolescents China Child and Adolescent Cardiovascular Health Hypertension National Health and Nutrition Examination Surveys

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Genetic variant reanalysis reveals a case of Sandhoff disease with onset of infantile epileptic spasm syndrome

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Acta Epileptologica 2024年第1期7卷 67-73页

作者： Qi Zhang Liping Zou Qian Lu Qiuhong Wang Shuo Dun Jing Wang Medical School of Chinese PLA Beijing100853China department of pediatrics the First Medical Center of PLA General HospitalBeijing100853China

Background Sandhoff disease(SD)i s an autosomal recessive lysosomal disease with clinical manifestations such as epilepsy,psychomotor retardation and developmental ***,infantile SD with onset of infantile epilepsy spasm syndrome(IESS)is extremely *** presentation The case presented here was a 22-month-old boy,who presented with IESS and psychomotor retardation/regression at 6 months of *** patient showed progressive aggravation of seizures and excessive startle *** whole exome sequencing data,which initially revealed negative results,were reanalyzed and indicated a homozygous mutation at the c.1613+4del splice site of the HEXB *** activities ofβ-hexosaminidase A and total hexosaminidase were significantly *** fundus examination showed cherry red spots at the *** IESS can be an epileptic phenotype of infantile *** phenotypes should be adequately collected in genetic *** the case of negative sequencing results,gene variant reanalysis can be performed when the patients show clinically suspicious indications.

关键词： Infantile Sandhoff disease Gene variant reanalysis HEXB gene Infantile epilepsy spasm syndrome Cherry red spot Human phenotype ontology

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Exome sequencing identifies a likely causative variant in 53%of families with ciliopathy-related features on renal ultrasound after excluding NPHP1 deletions

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Genes & Diseases 2024年第5期11卷 40-43页

作者： Konstantin Deutsch Verena Klambt Thomas M.Kitzler Tilman Jobst-Schwan Ronen Schneider Florian Buerger Steve Seltzsam Sherif El Desoky Jameela A.Kari Farkhanda Hafeez Maria Szczepanska Loai A.Eid Hazem S.Awad Muna Al-Saffar Neveen A.Soliman Velibor Tasic Camille Nicolas-Frank Kirollos Yousef Luca M.Schierbaum Sophia Schneider Abdul Halawi Izzeldin Elmubarak Katharina Lemberg Shirlee Shril Shrikant M.Mane Nancy Rodig Friedhelm Hildebrandt Division of Nephrology Department of PediatricsBoston Children’s HospitalHarvard Medical SchoolBostonMA 02215USA department of Pediatric Gastroenterology Nephrology and Metabolic DiseasesCharite Universitatsmedizin BerlinBerlin 13353Germany Berlin Institute of Health at Charitee Universitatsmedizin Berlin BIH Biomedical Innovation AcademyBIH Charite Clinician Scientist ProgramBerlin 10178Germany department of Nephrology and Hypertension FriedrichAlexander-Universitat Erlangen-NurnbergErlangen 91054Germany department of pediatrics Faculty of Medicine King Abdulaziz UniversityPediatric Nephrology Center of ExcellenceKing Abdulaziz University HospitalJeddah 21589Saudi Arabia department of Pediatric Nephrology The Children’s Hospital and Institute of Child HealthLahore 54000Pakistan department of pediatrics Faculty of Medical Sciences in ZabrzeMedical University of Silesia in KatowiceKatowice 40-752Poland Dubai Hospital and Al-Jalila Children’s Specialty Hospital Kidney Center of ExcellenceDubai 4545United Arab Emirates department of Genetics and Genomics UAE UniversityAbu Dhabi 15551United Arab Emirates department of pediatrics Division of Genetics and GenomicsBoston Children’s HospitalHarvard Medical SchoolBostonMA 02215USA department of pediatrics Center of Pediatric Nephrology and TransplantationKasr Al Ainy School of MedicineCairo UniversityCairo 11562Egypt Egypt Center for Research and Regenerative Medicine(ECRRM) Cairo 11511Egypt Medical Faculty Skopje University Children’s HospitalSkopje 1000North Macedonia department of Genetics Yale University School of MedicineNew HavenCT 06520USA

Nephronophthisis-related ciliopathies(NPHP-RC)represent one of the most common causes of chronic kidney disease in the first three decades of life and are characterized by a broad genetic and clinical heterogeneity.1 To date,more than 90 genes have been identified that cause autosomalrecessive NPHP-RC if mutated,accounting for up to 60%of cases.1 Among these,homozygous deletions of NPHP1 are the most common *** genes localize to primary cilia,basal bodies,or the centrosome and lead to a primary ciliary disruption if mutated,thereby causing a broad phenotypical spectrum.1 Patients that suffer from NPHP-RC can either have an isolated renal phenotype,such as polyuria,polydipsia,decreased urinary concentration ability,and secondary enuresis due to loss of tubular function,or present with extrarenal symptoms including retinal degeneration,cerebellar vermis hypoplasia,or hepatic *** are often diagnosed in early adolescence,reaching end-stage renal disease before 25 years of age,but early onset and rapidly progressive forms of NPHP-RC also exist.1 Renal ultrasound indicates kidneys of normal or reduced renal length with increased echogenicity and corticomedullary cysts.

关键词： degeneration urinary likely

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Biological function of miRNA-145-5p in angiotensin II induced renal inflammation

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BIOCELL 2024年第4期48卷 601-611页

作者： BIN LI YUCHENG SHENG XIAOYING XU SHENGCUN WANG HONGYAN SONG JINGYUAN LI HAONAN JI QINGHUA WANG XIAODI ZHOU LONGJU QI department of pediatrics Affiliated Hospital of Nantong UniversityNantong226001China department of Science and Education Affiliated Nantong Hospitalof Nantong UniversityNantong226001China Laboratory Animal Center Nantong UniversityNantong226001China department of Gynecology and Obstetrics and department of Oncology Hai’an Hospital Affiliated to Nantong UniversityNantong226600China

Objective:Chronic kidney disease(CKD)is a progressive disorder characterized by intricate structural and functional alterations in the kidneys,attributable to diverse causative ***,the therapeutic promise of miR-145-5p in addressing renal pathologies has been *** investigation seeks to elucidate the functional role of miR-145-5p in injured kidneys by subjecting human glomerular mesangial cells(HGMCs)to stimulation with Angiotensin II(AngII).Materials and Methods:Cellular viability and the levels of inﬂammatory mediators were evaluated utilizing Cell Counting Kit-8(CCK-8),quantitative real-time polymerase chain reaction(qRT-PCR),and western blot methodologies,both in the presence of AngII incubation and in scenarios of miR-145p overexpression and ***,the cell cycle dynamics were elucidated through Fluorescence-activated Cell Sorting(FACS)***:AngII incubation induced an upregulation of miR-145-5p and inﬂammatory factors including Intercellular Adhesion Molecule 1(ICAM-1),Interleukin 6(IL-6),Interleukin 8(IL-8),and Interleukin 1β(IL-1β).Additionally,it elevated the expression of Cyclin A2,Cyclin D1,and the G2/M cell cycle ***,inhibition of miR-145-5p heightened the levels of inﬂammatory factors and cell cycle regulators induced by AngII *** expression of miR-145-5p correlated with a downregulation of Interleukin 10(IL-10)expression,concurrently promoting HGMC proliferation under AngII ***,ectopic miR-145-5p expression demonstrated a reduction in inﬂammatory factors,cell cyclin regulators,G2/M cell cycle ratio,and overall ***:MiR-145-5p exhibited inhibitory effects on the inﬂammatory response and proliferation induced by Angiotensin II in HGMCs,showcasing its potential as a therapeutic avenue for the treatment of kidney injury.

关键词： miR-145-5p Kidney Angiotensin II Cell cycle Inflammation

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Changes in children’s cardiorespiratory fitness and body mass index over the course of the COVID‑19 pandemic:a 34‑month longitudinal study of 331 primary school children

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World Journal of pediatrics 2024年第2期20卷 185-188页

作者： Gerald Jarnig Reinhold Kerbl Mireille N.M.van Poppel Institute of Human Movement Science Sport and HealthUniversity of GrazMozartgasse 148010 GrazAustria department of pediatrics and Adolescent Medicine LKH Hochsteiermark8700 LeobenAustria

In late 2019,heavy restrictions were enforced on public activities worldwide to diminish the spread of the severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)virus[1].These restrictions,which included school closures and strictly limited sports and leisure opportunities,particularly impacted children and adolescents[2].Lifestyle changes in children and adolescents led to reduced physical activity levels[2,3],increased sedentary behavior[3,4],changes in eating habits,and changes in sleep habits[3].

关键词： respiratory acute children

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Sirolimus can promote the disappearance of renal angiomyolipoma associated with tuberous sclerosis complex:a prospective cohort study

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World Journal of pediatrics 2024年第6期20卷 602-610页

作者： Shuo Dun Yang-Yang Wang Lin Wan Qiu-Hong Wang Qian Lu Xiao-Yan Yang Qi Zhang Hui-Min Chen Lu-Peng Qiu Li-Ping Zou Medical School of Chinese PLA Beijing 100853China department of pediatrics The First Medical CenterChinese PLA General HospitalBeijing 100853China Center of Epilepsy Beijing Institute for Brain DisordersBeijing 100069China

Background Renal angiomyolipoma(RAML)is the most common kidney lesion in patients with tuberous sclerosis complex(TSC),affecting about 80%of *** is a benign tumor that grows over time,usually bilaterally,and can easily lead to kidney complications such as acute ***,we investigated the efficacy and safety of sirolimus in children with TSC-associated RAML and explored the factors affecting tumor disappearance under sirolimus treatment through subgroup *** A prospective cohort study was *** was initiated at 1 mg/(m^(2)×day),and dose adjustments were made by a 2-week titration period to attain a trough blood concentration of 5-10 ng/*** disappearance of RAML in chil-dren after sirolimus treatment was observed,and Cox regression was used to screen the factors affecting tumor *** One hundred and twenty-six patients who met the criteria were *** 3 months,6 months,12 months,and 24 months of follow-up,tumors disappeared in 18(14.3%),30(23.8%),39(31.0%),and 42(33.3%)children,*** disappeared in 50(39.7%)children by the last visit of each individual,and 30(60%)of them occurred within 6 *** multivariate Cox regression analysis showed that patients with a smaller maximum tumor diameter at baseline had a higher tumor disappearance ***-six(29%)patients had stomatitis during the entire treatment period,and no serious *** Sirolimus could promote the disappearance of TSC-related *** disappearance rate was correlated with the maximum diameter at baseline,and the smaller the tumor was,the higher the disappearance *** is well tolerated in the treatment of RAML associated with TSC.

关键词： Angiomyolipoma mTOR inhibitor Sirolimus Tuberous sclerosis

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