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Etiology and long-term outcome of extrahepatic portal vein obstruction in children

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World Journal of Gastroenterology 2010年第39期16卷 4968-4972页

作者： Batia Weiss Eyal Shteyer Asaf Vivante Drora Berkowitz Shimon Reif Zvi Weizman Yoram Bujanover Rivka Shapiro Division of pediatric Gastroenterology Hepatology and NutritionEdmond and Lily Safra Children's Hospital Sackler Faculty of Medicine Tel-Aviv University Division of pediatric Gastroenterology Hepatology and NutritionHadassah Medical CenterThe Hebrew University School of Medicine Division of pediatric Gastroenterology Hepatology and NutritionMeyer Children's HospitalRambam Medical Center Rappaport School of Medicine TechnionIsrael Institute of Technology Division of pediatric Gastroenterology Hepatology and NutritionDana Children's HospitalSouraski Medical Center Division of pediatric Gastroenterology Hepatology and NutritionSoroka Medical Center Faculty of Health Sciences Ben-Gurion University of the Negev Department of pediatric Gastroenterology and Nutrition Division of Pediatric TransplantationSchneider Children's Hospital

AIM:To study the management and outcome of children with extrahepatic portal vein obstruction(EHPVO) in a whole country ***:A nationwide multicenter retrospective case series of children with EHPVO was *** on demographics,radiographic studies,laboratory workup,endoscopic and surgical procedures,growth and development,were extracted from the patients' *** of clinical presentation,etiology of EHPVO,management and outcome were ***:Thirty patients,13 males and 17 females,19(63.3%) Israeli and 11(36.7%) Palestinians,were included in the *** at presentation was 4.8± 4.6 years,and mean follow-up was 4.9±4.3 *** anomalies were found in 4 *** incidence of EHPVO in Israeli children aged 0-14 years was 0.72/*** factors for EHPVO were detected in 13(43.3%)patients,including 9 patients(30%) with perinatal risk factors,and 4 patients(13.3%) with prothrombotic states:two had low levels of protein S and C,one had lupus anticoagulant,and one was homozygous for methyltetrahydrofolate reductase *** 56.6% of patients,no predisposing factors were *** most common presenting symptoms were an incidental finding of splenomegaly(43.3%),and upper gastrointestinal bleeding(40%).No differences were found between Israeli and Palestinian children with regard to age at presentation,etiology and clinical *** occurred in 18 patients(60%),at a median age of 3 *** or esophageal banding was performed in 20 *** sclerotherapy complications were *** shunts were performed in 11 patients(36.6%),at a median age of 11(range 3-17)years:splenorenal in 9,mesocaval in 1,and a meso-Rex shunt in 1 *** patient underwent splenectomy due to severe *** were followed up for a median of 3(range 0.5-15)*** patient died aged 3 years due to mucopolysaccharidase deficiency type Ⅲ.None of the patients died due to gastrointestin

关键词： Children Extrahepatic Obstruction Outcome Portal Vein

Serum urate is associated with an increased risk of inflammatory bowel disease: A bidirectional Mendelian randomization study

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World Journal of Clinical Cases 2024年第5期12卷 891-902页

作者： Song Zhang Xue Fang Le Kang Xiang-Yu Sui Miao Liu Yu-Jia Luo Shuo Fu Zhao-Shen Li Sheng-BingZhao Yu Bai Department of Gastroenterology Changhai HospitalShanghai 200433China Department of Gastroenterology Changhai HospitalSecond Military Medical University/Naval Medical UniversityShanghai 200433China Digestive Endoscopy center Changhai HospitalNaval/Second Military Medical UniversityShanghai 200433China National Clinical Research center for Digestive Diseases Shanghai 200433China

BACKGROUND Previous studies have indicated bidirectional associations between urate levels and inflammatory bowel disease(ibd),including ulcerative colitis(UC)and Crohn’s disease(CD).However,it remains unclear whether the observations are causal because of confounding *** To investigate the causal associations between urate levels and ibd using bidirec-tional Mendelian randomization(MR).METHODS Independent genetic variants for urate levels and ibd were selected as instru-mental variables from published genome-wide association studies(GWASs).Summary statistics for instrument-outcome associations were retrieved from three separate databases for ibd(the UK Biobank,the FinnGen database and a large GWAS meta-analysis)and one for urate levels(a large GWAS meta-analysis).MR analyses included the inverse-variance-weighted method,weighted-median estimator,MR-Egger and sensitivity analyses(MR-PRESSO).A meta-analysis was also conducted to merge the data from separate outcome databases using a fixed-effects *** Genetically higher serum urate levels were strongly associated with an increased risk of UC[odds ratio(OR):1.95,95%confidence interval(CI):1.86-2.05]after outlier correction,and the ORs(95%CIs)for ibd and CD were 0.94(95%CI:0.86-1.03)and 0.91(95%CI:0.80-1.04),*** studies have confirmed the positive association between urate levels and ***,genetically predicted ibd was inversely related to urate levels(OR:0.97,95%CI:0.94-0.99).However,no association was observed between genetically influenced UC or CD and urate *** Urate levels might be risk factors for UC,whereas genetically predicted ibd was inversely associated with urate *** findings provide essential new insight for treating and preventing ibd.

关键词： Inflammatory bowel disease Urate levels Antioxidant Mendelian randomization Single nucleotide polymorphism

Bronchiolitis in young infants: is it a risk factor for recurrent wheezing in childhood?

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World Journal of pediatrics 2017年第1期13卷 41-48页

作者： Firas Rinawi Imad Kassis Rina Tamir Amir Kugelman Isaac Srugo Dan Miron pediatric Department A’.HaEmeq Medical center AfulaIsrael pediatric Infectious Disease unit Meyer Children HospitalHaifaIsrael The Ruth and Bnice Rappaport School ofMedicine Technionlsrael Institute ofTechnologyHaifaIsrael pediatric Department.Bnai Zion Medical center Haifalsrael pediatric Infectious Disease Consultation Service.HaEmeq Medical center Afula.Israel.

Background:Acute bronchiolitis in infancy is considered a risk factor for recurrent wheezing episodes in *** present study assessed prevalence,clinical manifestaffons and risk factors for recurrent wheezing events during the first 3 years of life and persistent wheezing events beyond this age in children hospitalized as young infants with acute bronchioliffs.

关键词： acute bronchiolitis recurrent wheezing young infants

Role of axon resealing in retrograde neuronal death and regeneration after spinal cord injury

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Neural Regeneration Research 2019年第3期14卷 399-404页

作者： William Rodemer Michael E.Selzer Shriners Hospital pediatric Research (center center for Neural Repair and Rehabilitation) Department of Neurology the Lewis Katz School of Medicine at Temple University

Spinal cord injury leads to persistent behavioral deficits because mammalian central nervous system axons fail to regenerate. A neuron's response to axon injury results from a complex interplay of neuron-intrinsic and environmental factors. The contribution of axotomy to the death of neurons in spinal cord injury is controversial because very remote axotomy is unlikely to result in neuronal death, whereas death of neurons near an injury may reflect environmental factors such as ischemia and inflammation. In lampreys, axotomy due to spinal cord injury results in delayed apoptosis of spinal-projecting neurons in the brain, beyond the extent of these environmental factors. This retrograde apoptosis correlates with delayed resealing of the axon, and can be reversed by inducing rapid membrane resealing with polyethylene glycol. Studies in mammals also suggest that polyethylene glycol may be neuroprotective, although the mechanism(s) remain unclear. This review examines the early, mechanical, responses to axon injury in both mammals and lampreys, and the potential of polyethylene glycol to reduce injury-induced pathology. Identifying the mechanisms underlying a neuron's response to axotomy will potentially reveal new therapeutic targets to enhance regeneration and functional recovery in humans with spinal cord injury.

关键词： axon resealing regeneration retrograde neuronal death spinal cord injury sea lamprey PEG mitochondrial dysfunction calcium signaling

Effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome

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World Journal of Gastroenterology 2006年第15期12卷 2427-2431页

作者： Mehri Najafi Sani Hamid Reza Kianifar Abdolrazagh Kianee Gholamreza Khatami Division of pediatric Gastroenterology Department of pediatrics Children's Medical Center Hospital of Tehran UniversityTehranIran Division of pediatric Gastroenterology Department of pediatrics Ghaem Medical Center of Mashhad University of Medical SciencesMashhadIran Division of pediatric Cardiology Department of pediatrics Children's Medical Center Hospital of Tehran UniversityTehranIran

AIM： To study the effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome, METHODS： Garlic powder in a capsule form was given to 15 children with hepatopulmonary syndrome （confirmed by contrast echocardiography） at the dosage of 1 g/1.73 m^2 per day. Patients were evaluated clinically and by arterial blood gas every four weeks. RESULTS： The garlic capsule was administered to 15 patients with hepatopulmonary syndrome. There were 10 boys and 5 girls with a mean age of 9.4 ± 3.9 years. The underlying problems were biliary tract atresia （4 patients）, autoimmune hepatitis （4 patients）, cryptogenic cirrhosis （4 patients） and presinusoidal portal hypertension （3 patients）. Eight patients （53.3%） showed an increase of 10 mmHg in their mean arterial oxygen pressure. The baseline PaO2 was 65.6 ± 12.1 mmHg in the responder group and 47.1 ± 11.2 mmHg in nonresponder group. At the end of treatment the mean PaO2 in responders and non-responders was 92.2 ± 7.75 mmHg and 47.5 ± 11.87 mmHg, respectively （P 〈 0.01）. CONCLUSION： Garlic may increase oxygenation and improve dyspnea in children with hepatopulmonary syndrome.

关键词： Hepatopulmonary syndrome Garlic Arterial oxygen pressure pediatric

A genomic association study revealing subphenotypes of childhood steroid-sensitive nephrotic syndrome in a larger genomic sequencing cohort

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Genes & Diseases 2024年第4期11卷 400-410页

作者： Han Chan Fenfen Ni Bo Zhao Huimin Jiang Juanjuan Ding Li Wang Xiaowen Wang Jingjing Cui Shipin Feng Xiaojie Gao Xueying Yang Huan Chi Hao Lee Xuelan Chen Xiaoqin Li Jia Jiao Daoqi Wu Gaofu Zhang Mo Wang Yupeng Cun Xiongzhong Ruan Haiping Yang Qiu Li Department of Nephrology Children's Hospital of Chongqing Medical UniversityNational Clinical Research Center for Child Health and DisordersMinistry of Education Key Laboratory of Child Development and DisordersChongqing Key Laboratory of PediatricsChongqing 400014China Department of Nephrology Shenzhen Children's HospitalShenzhenGuangdong 518034China Department of Nephrology Kunming Children's HospitalKunming Medical UniversityKunmingYunnan 650228China Department of Nephrology Wuhan Children's HospitalTongji Medical College of Huazhong University of Science and TechnologyWuhanHubei 430015China Department of Nephrology Chengdu Women and Children Central HospitalChengduSichuan 610073China pediatric Research Institute Ministry of Education Key Laboratory of Child Development and DisordersNational Clinical Research Center for Child Health and DisordersChina International Science and Technology Cooperation Base of Child Development and Critical DisordersChongqing Key Laboratory of Translational Medical Research in Cognitive Development and Learning and Memory DisordersChildren's Hospital of Chongqing Medical UniversityChongqing 400014China Department of Nephrology John Moorhead Research LaboratoryUniversity College London Medical SchoolRoyal Free CampusUniversity College LondonLondon NW32PFUnited Kingdom

Dissecting the genetic components that contribute to the two main subphenotypes of steroid-sensitive nephrotic syndrome(SSNS)using genome-wide association studies(GWAS)strategy is important for understanding the *** conducted a multicenter cohort study(360 patients and 1835 controls)combined with a GWAS strategy to identify susceptibility var-iants associated with the following two subphenotypes of ssNS:steroid-sensitive nephrotic syn-drome without relapse(SSNswR,181 patients)and steroid-dependent/frequent relapse nephrotic syndrome(SDNS/FRNS,179 patients).The distribution of two single-nucleotide poly-morphisms(SNPs)in ANKRD36 and ALPG was significant between SSNSWR and healthy controls,and that of two SNPs in GAD1 and HLA-DQA1 was significant between SDNS/FRNS and healthy ***,rs1047989 in HLA-DQA1 was a candidate locus for SDNS/FRNS but not for *** significant SNPs were observed between SSNSWR and SDNS/***,chromosome 2:171713702 in GAD1 was associated with a greater steroid dose(>0.75 mg/kg/d)upon relapse to first remission in patients with SDNS/FRNS(odds ratio=3.14;95%confidence interval,0.97-9.87;P=0.034).rs117014418 in APOL4 was significantly associated with a decrease in eGFR of greater than 20%compared with the baseline in SDNS/FRNS patients(P=0.0001).Protein-protein intersection network construction suggested that HLA-DQA1 and HLA-DQB1 function together through ***,SSNSWR belongs to non-HLA region-dependent nephropathy,and the HLA-DQA/DQB region is likely strongly associated with dis-ease relapse,especially in SDNS/*** study provides a novel approach for the GWAS strategy of SsNS and contributes to our understanding of the pathological mechanisms of SSNSWRandSDNS/FRNS.

关键词： Frequentrelapse Genome-wide association study Human leukocyte antigen region Steroid-sensitive nephrotic syndrome

Role of surgery in severe ulcerative colitis in the era of medical rescue therapy

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World Journal of Gastroenterology 2012年第29期18卷 3833-3838页

作者： Bosmat Dayan Dan Turner The pediatric Gastroenterology Unit Shaare Zedek Medical CenterThe Hebrew University of JerusalemJerusalem 91031Israel

Despite the growing use of medical salvage therapy,colectomy has remained a cornerstone in managing acute severe ulcerative colitis(ASC) both in children and in *** should be regarded as a life saving procedure in ASC,and must be seriously considered in any steroid-refractory ***,colectomy is not a cure for the disease but rather the substitution of a large problem with smaller problems,including fecal incontinence,pouchitis,irritable pouch syndrome,cuffitis,anastomotic ulcer and stenosis,missed or de-novo Crohn's disease and,in young females,reduced *** notion has led to the widespread practice of offering medical salvage therapy before colectomy in most patients without surgical abdomen or toxic *** salvage therapies which have proved effective in the clinical trial setting include cyclosporine,tacrolimus and infliximab,which seem equally effective in the short *** predictive rules can identify a subset of patients who will eventually fail corticosteroid therapy after only 3-5 d of steroid therapy with an accuracy of 85%-95%.This accuracy is sufficiently high for initiat-ing medical therapy,but usually not colectomy,early in the admission without delaying colectomy if *** approach has reduced the colectomy rate in ASC from 30%-70% in the past to 10%-20% nowadays,and the mortality rate from over 70% in the 1930s to about 1%.In general,restorative proctocolectomy(ileoanal pouch or ileal pouch-anal anastomosis),especially the J-pouch,is preferred over straight pullthrough(ileo-anal) or ileo-rectal anastomosis,which may still be considered in young females concerned about *** in the acute severe colitis setting,is usually performed in three steps due to the severity of the inflammation,concurrent steroid treatment and the generally reduced clinical *** first surgical step involves colectomy and constructing an ileal stoma,the second-constructing the pouch and the third-c

关键词： Acute severe ulcerative colitis Colectomy Corticosteroids Cyclosporine Infliximab Tacrolimus

PTEN inhibition and axon regeneration and neural repair

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Neural Regeneration Research 2015年第9期10卷 1363-1368页

作者： Yosuke Ohtake Umar Hayat Shuxin Li Shriners Hospitals pediatric Research center Department of Anatomy and Cell Biology Temple University School of Medicine

The intrinsic growth ability of all the neurons declines during development although some may grow better than others. Numerous intracellular signaling proteins and transcription factors have been shown to regulate the intrinsic growth capacity in mature neurons. Among them, PI3 kinase/Akt pathway is important for controlling axon elongation. As a negative regulator of this pathway, the tumor suppressor phosphatase and tensin homolog （PTEN） appears critical to con- trol the regenerative ability of young and adult neurons. This review will focus on recent research progress in axon regeneration and neural repair by PTEN inhibition and therapeutic potential of blocking this phosphatase for neurological disorders. Inhibition of PTEN by deletion in con- ditional knockout mice, knockdown by short-hairpin RNA, or blockade by pharmacological approaches, including administration of selective PTEN antagonist peptides, stimulates various degrees of axon regrowth in juvenile or adult rodents with central nervous system injuries. Im- portantly, post-injury PTEN suppression could enhance axonal growth and functional recovery in adult central nervous system after injury.

关键词： PTEN inhibition antagonist peptide spinal cord injury intrinsic growth capacity axonregeneration functional recovery

Therapeutic Profile and Prognostic Factors of Patients Suffering from Upper Digestive Bleeding at Sikasso Regional Hospital

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Open Journal of Gastroenterology 2024年第9期14卷 300-312页

作者： Oumar Traoré Abdoul Salam Diarra Dramane Touré Tawfiq Abu Amadou Boubeye Maïga Kadiatou Cissé Saïdon Touré Mohomedine Touré Aboudou Dolo Youana Koné Madou Traoré Mamadou Togo Kalba Péliaba Mamadou Samaké Hamadoun Sangho Service of Internal Medicine Regional Hospital Sikasso Mali National center for Scientific and Technological Research Bamako Mali Department of pediatric Health Referral Center of Kalaban Coro Koulikoro Mali Private Practice for Family and Emergency Medicine Casablanca Morocco Service of Nephrology Regional Hospital Sikasso Mali Department of Infectious Disease Regional Hospital Sikasso Mali Service de Mdecine Interne CHU Gabriel Tour Bamako Mali Service dHpato-Gastro-Entrologie CHU Gabriel Tour Bamako Mali Department of Public Health Faculty of Medicine and Odontostomatology University of Sciences Techniques and Technologies of Bamako Bamako Mali

Introduction: Upper digestive hemorrhage is one of the main digestive emergencies and remains a major cause of morbidity and mortality in Mali. The aim of this study was to describe the therapeutic profile and outcome of patients suffering from upper digestive haemorrhage. Methodology: This was a prospective study carried out in the internal medicine department of the Sikasso Regional Hospital from August 2022 to July 2023. All adult patients presenting with upper digestive haemorrhage and having given their consent, were included. Data were analyzed using SPSS version 21 software. Results: Sixty-three patients were enrolled. The mean age was 49.7 ± 18.99 years, with a male-female sex ratio of 2.2. Ruptured esophageal varices (37.5%) and peptic ulcer (25%) were the main etiologies. Pharmacological treatment was dominated by proton pump inhibitors (85.7%). Hemostasis endoscopy accounted for 3.17%. The evolution was marked by hemorrhagic arrest (69.84%), recurrence of hemorrhage (11.11%) and death (19.04%), the main cause of which was hemorrhagic shock (58.3%). We found no statistically significant relationship between prognosis and etiologies (P = 0.11), and length of hospital stay (P = 0.18). Conclusion: Hemostasis endoscopy remains a challenge for Sikasso Hospital. A holistic strategy of communication and community awareness-raising, combined with adequate technical facilities, will help to improve patient care and outcomes.

关键词： Digestive Hemorrhage Digestive Endoscopy Treatment Outcome