Journal of Pediatric Gastroenterology & Nutrition最新文献

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HLA-DQB1*02 Allele In First-Degree Relatives of Patients With Celiac Disease. 乳糜泻患者一级亲属HLA-DQB1*02等位基因的研究
Journal of Pediatric Gastroenterology & Nutrition Pub Date : 2019-11-01 DOI: 10.1097/MPG.0000000000002475
D. Poddighe
{"title":"HLA-DQB1*02 Allele In First-Degree Relatives of Patients With Celiac Disease.","authors":"D. Poddighe","doi":"10.1097/MPG.0000000000002475","DOIUrl":"https://doi.org/10.1097/MPG.0000000000002475","url":null,"abstract":"1. Harvey BM, Eussen S, Harthoorn LF, et al. Mineral intake and status of cow’s milk allergic infants consuming an amino acid-based formula. J Pediatr Gastroenterol Nutr 2017;65:346–9. 2. Champagne ET. Low gastric hydrochloric acid secretion and mineral bioavailability. Adv Exp Med Biol 1989;249:173–84. 3. Burks AW, Harthoorn LF, Van Ampting MT, et al. Synbiotics-supplemented amino acid-based formula supports adequate growth in cow’s milk allergic infants. Pediatr Allergy Immunol 2015;26:316–22.","PeriodicalId":16725,"journal":{"name":"Journal of Pediatric Gastroenterology & Nutrition","volume":"8 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83606420","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Using A Steroid-Sparing Tool In Paediatric Inflammatory Bowel Disease To Evaluate Steroid Use and Dependency. 使用类固醇保留工具在儿童炎症性肠病中评估类固醇使用和依赖。
Journal of Pediatric Gastroenterology & Nutrition Pub Date : 2019-11-01 DOI: 10.1097/MPG.0000000000002442
R. Harris, W. Sim, H. Sutton, V. Garrick, L. Curtis, L. Gervais, V. Merrick, A. Barclay, D. Flynn, R. Tayler, R. Hansen, R. Russell
{"title":"Using A Steroid-Sparing Tool In Paediatric Inflammatory Bowel Disease To Evaluate Steroid Use and Dependency.","authors":"R. Harris, W. Sim, H. Sutton, V. Garrick, L. Curtis, L. Gervais, V. Merrick, A. Barclay, D. Flynn, R. Tayler, R. Hansen, R. Russell","doi":"10.1097/MPG.0000000000002442","DOIUrl":"https://doi.org/10.1097/MPG.0000000000002442","url":null,"abstract":"OBJECTIVES\u0000To evaluate the use of steroids within the paediatric inflammatory bowel disease (PIBD) population at a tertiary paediatric centre over a year; to identify cases of steroid dependency; and assess factors associated with steroid excess.\u0000\u0000\u0000METHODS\u0000The prevalent PIBD population (01/05/17-30/04/18) were reviewed. Data was collected retrospectively from patient records and entered into an online steroid assessment tool (modified for paediatrics).\u0000\u0000\u0000RESULTS\u0000229 patients (181 Crohn's disease (CD), 31 ulcerative colitis (UC), and 17 IBD-unclassified (IBD-U)) were included. 38/229 patients (16.6%) received oral steroids; 12/38 (31.6%) receiving >3-month course. 11/38 (28.9%) received >1 steroid course (maximum 2). 37/229 (16.2%) patients had EEN, with 26/37 (11.4% total cohort) avoiding steroid use during the study period.Quiescent disease activity had a negative correlation with steroid use (11/127 (8.7%) (p < 0.01) versus 27/102 (26.5%) (p < 0.01)), and steroid dependency (3/127 (2.4%) versus 12/102 (11.8%) (p < 0.01)). UC patients were more likely to be steroid dependent (5/31 (16.1%) UC versus 10/198 (5.1%); (p = 0.02)); as were network-managed patients (8/11 (72.7%) versus 7/27 (25.9%); (p = 0.01)). 14/15 (93.3%) of steroid dependent patients had active steroid sparing strategies in place (e.g. commencement, switching or optimisation of therapies).\u0000\u0000\u0000CONCLUSIONS\u0000We have described rates of steroid use and dependency within our PIBD population. EEN served as a steroid sparing tool in 11.4% of the total cohort. Replication of this study in other paediatric centres would allow comparative analysis.","PeriodicalId":16725,"journal":{"name":"Journal of Pediatric Gastroenterology & Nutrition","volume":"309 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76862720","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Celiac Disease in First-Degree Relatives: Homozygosity of DQB1*02 and at least one copy of HLA-DQB1*02 Allele. 一级亲属的乳糜泻:DQB1*02的纯合性和至少一个HLA-DQB1*02等位基因拷贝
Journal of Pediatric Gastroenterology & Nutrition Pub Date : 2019-11-01 DOI: 10.1097/MPG.0000000000002476
Sdepanian Vl, Lopes Lhc, Oliveira Rp, Muniz Jg
{"title":"Celiac Disease in First-Degree Relatives: Homozygosity of DQB1*02 and at least one copy of HLA-DQB1*02 Allele.","authors":"Sdepanian Vl, Lopes Lhc, Oliveira Rp, Muniz Jg","doi":"10.1097/MPG.0000000000002476","DOIUrl":"https://doi.org/10.1097/MPG.0000000000002476","url":null,"abstract":"1. Lopes LHC, Muniz JG, Oliveira RP, et al. Celiac disease in Brazilian firstdegree relatives: the odds are five times greater for HLA DQ2 Homozygous. J Pediatr Gastroenterol Nutr 2019;68:e77–80. 2. De Silvestri A, Capittini C, Poddighe D, et al. HLA-DQ genetics in children with celiac disease: a meta-analysis suggesting a two-step genetic screening procedure starting with HLA-DQ b chains. Pediatr Res 2018;83:564–72. 3. Poddighe D, Capittini C, Gaviglio I, et al. HLA-DQB1 02 allele in children with celiac disease: potential usefulness for screening strategies. Int J Immunogenet 2019. doi:10.1111/iji.12441. [Epub ahead of print].","PeriodicalId":16725,"journal":{"name":"Journal of Pediatric Gastroenterology & Nutrition","volume":"2016 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73656849","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Vitamin D supplementation and T Cell Regulation In Preterm Infants: A Randomized Controlled Trial. 早产儿补充维生素D和T细胞调节:一项随机对照试验。
Journal of Pediatric Gastroenterology & Nutrition Pub Date : 2019-11-01 DOI: 10.1097/MPG.0000000000002448
H. Aly, L. Mohsen, I. Bhattacharjee, Amr Malash, Amr Atyia, Sherif Elanwary, R. Hawary
{"title":"Vitamin D supplementation and T Cell Regulation In Preterm Infants: A Randomized Controlled Trial.","authors":"H. Aly, L. Mohsen, I. Bhattacharjee, Amr Malash, Amr Atyia, Sherif Elanwary, R. Hawary","doi":"10.1097/MPG.0000000000002448","DOIUrl":"https://doi.org/10.1097/MPG.0000000000002448","url":null,"abstract":"The objective of this study was to evaluate the effect of two different doses of vitamin D on the expression of T regulatory cells (Treg) in premature infants. A double blinded randomized controlled trial was conducted on preterm infants born with gestational age (GA) between 28 and 33weeks. Subjects were randomly assigned to receive 400 or 800 IU/day of vitamin D3 when they achieved 100 ml/kg of enteral feeds. Percent increase in Treg cell counts were measured by flow cytometry at enrollment, and after one and four weeks of oral vitamin D supplementation at the allotted doses in both groups. Short-term morbidity and mortality outcomes were also assessed. A total of 40 infants were enrolled, 20 in each group. The change in Treg count (%) was significantly less in the low-dose vitamin D3 supplementation group after one week (1.9 ± 5.5 vs 60 ± 5.6, p = 0.0005) and after four weeks (1.8 ± 5.7 vs 73.7 ± 5.6, p = 0.0028). The two groups did not differ in anthropometric measurements, duration of oxygen and respiratory support, and mortality. Length of hospital stay was longer in the low-dose group (24.9 ± 5.14 vs 22 ± 3.49, p = 0.04). Oral vitamin D supplementation has a dose and time dependent effect on percentage of Treg in infants born prematurely. The 800 IU dose of vitamin D3 did not have apparent short-term side effects. Larger studies are needed to explore the effect of vitamin D3 dosing on length of hospital stay.","PeriodicalId":16725,"journal":{"name":"Journal of Pediatric Gastroenterology & Nutrition","volume":"46 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88611321","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 5
Shortened 8-Week Course of Sofosbuvir/Ledipasvir Therapy in Adolescents with Chronic Hepatitis C Infection. 索非布韦/来地帕韦治疗青少年慢性丙型肝炎感染的缩短8周疗程
Journal of Pediatric Gastroenterology & Nutrition Pub Date : 2019-11-01 DOI: 10.1097/MPG.0000000000002449
D. Serranti, I. Dodi, E. Nicastro, A. Cangelosi, S. Riva, S. Ricci, E. Bartolini, S. Trapani, Greta Mastrangelo, P. Vajro, L. D’Antiga, M. Resti, G. Indolfi
{"title":"Shortened 8-Week Course of Sofosbuvir/Ledipasvir Therapy in Adolescents with Chronic Hepatitis C Infection.","authors":"D. Serranti, I. Dodi, E. Nicastro, A. Cangelosi, S. Riva, S. Ricci, E. Bartolini, S. Trapani, Greta Mastrangelo, P. Vajro, L. D’Antiga, M. Resti, G. Indolfi","doi":"10.1097/MPG.0000000000002449","DOIUrl":"https://doi.org/10.1097/MPG.0000000000002449","url":null,"abstract":"Treatment-naïve, non-cirrhotic adults with chronic hepatitis C virus (HCV) genotype 1 infection and with viremia levels < 6 million IU/mL could be effectively treated with sofosbuvir/ledipasvir for 8 weeks. The aim of this pilot, prospective, open-label, multicentre study was to evaluate the efficacy and safety of this shortened treatment course in adolescents (≥ 12 years). The efficacy endpoint was sustained virological response 12 weeks after the end of treatment (SVR12). Safety was assessed by adverse events and clinical/laboratory data.Fourteen consecutive adolescents (median age 16.5 years, Q1 14.1- Q3 17.4; female 57.1%), vertically-infected, were enrolled and treated (June 2018 - January 2019). Overall, the end of treatment response and SVR12 were 100%. No grade 3-4 adverse event or a serious adverse event was observed.Further studies are needed to confirm the optimal efficacy of the shortened 8-week treatment with sofosbuvir/ledipasvir for treatment-naïve, non-cirrhotic adolescents with chronic HCV genotype 1 infection and pre-treatment viremia level < 6 million IU/mL.","PeriodicalId":16725,"journal":{"name":"Journal of Pediatric Gastroenterology & Nutrition","volume":"40 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78839320","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 13
Celiac Dietary Adherence Test simplifies Determining Adherence to a Gluten-Free Diet in Swedish Adolescents. 乳糜泻饮食依从性测试简化了确定瑞典青少年对无麸质饮食的依从性。
Journal of Pediatric Gastroenterology & Nutrition Pub Date : 2019-11-01 DOI: 10.1097/MPG.0000000000002451
Katarina Johansson, F. Norström, Katrina Nordyke, Anna Myléus
{"title":"Celiac Dietary Adherence Test simplifies Determining Adherence to a Gluten-Free Diet in Swedish Adolescents.","authors":"Katarina Johansson, F. Norström, Katrina Nordyke, Anna Myléus","doi":"10.1097/MPG.0000000000002451","DOIUrl":"https://doi.org/10.1097/MPG.0000000000002451","url":null,"abstract":"OBJECTIVES\u0000The aims of the study were to ascertain whether the Celiac Dietary Adherence Test (CDAT) could contribute in determining adherence to a gluten-free diet in celiac disease patients and to evaluate the diet adherence and well-being of a study population five years after a celiac disease screening known as \"Exploring the Iceberg of Celiacs in Sweden\".\u0000\u0000\u0000METHODS\u0000Through the screening, 90 adolescents (born 1997) were diagnosed with biopsy-proven celiac disease at twelve-years of age. Of them, 70 (78%) came to a five-year follow-up where anti-tissue transglutaminase antibodies 2 (TG2-IgA) was tested and a questionnaire was filled in, including CDAT, which consists of seven questions related to adherence. Non-parametrical tests were utilized to determine associations between adherence measures.\u0000\u0000\u0000RESULTS\u0000Among the adolescents, 86% were adherent to a gluten-free diet five years after screening, 38% reported their general well-being as excellent, 50% very well, and 12% well. Statistically significant associations were seen between TG2-IgA and the CDAT score (p=0.033), and the self-reported adherence question and the CDAT score (p < 0.001).\u0000\u0000\u0000CONCLUSIONS\u0000The screening-detected adolescents reported a high level of well-being and adherence to a gluten-free diet five years after screening. We conclude that the CDAT can be used in clinical practice as an estimation of adherence to a gluten-free diet. It would be most suitable to use in conjunction with currently used adherence measures, but can also be used as a stand-alone method when others are not accessible.","PeriodicalId":16725,"journal":{"name":"Journal of Pediatric Gastroenterology & Nutrition","volume":"43 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81189290","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 18
Management of Anti-drug Antibodies (ADA) to Biologic Medications in Children with Inflammatory Bowel Disease. 炎症性肠病儿童生物药物抗药抗体(ADA)的管理。
Journal of Pediatric Gastroenterology & Nutrition Pub Date : 2019-11-01 DOI: 10.1097/MPG.0000000000002440
R. Cohen, B. Schoen, S. Kugathasan, Cary G. Sauer
{"title":"Management of Anti-drug Antibodies (ADA) to Biologic Medications in Children with Inflammatory Bowel Disease.","authors":"R. Cohen, B. Schoen, S. Kugathasan, Cary G. Sauer","doi":"10.1097/MPG.0000000000002440","DOIUrl":"https://doi.org/10.1097/MPG.0000000000002440","url":null,"abstract":"BACKGROUND\u0000Treatment of pediatric inflammatory bowel disease (IBD) with monoclonal anti-TNFα can result in immunogenicity and formation of anti-drug antibodies (ADA). ADA are associated with loss of clinical response and worsening disease progression. Data examining treatment interventions to overcome ADA in pediatric IBD patients is lacking.\u0000\u0000\u0000RESULTS\u0000Medical records were reviewed from 234 children and adolescents with IBD treated with infliximab or adalimumab who underwent therapeutic drug monitoring (626 tests). All patients who had detectable antibodies were further analyzed. A total 58 patients (24.8%) developed ADA while being treated with infliximab or adalimumab. The incidence of antibody development was 12.9 per 100 person-years of anti-TNF treatment. 28 patients underwent dose optimization and 54% had undetectable ADA on follow-up monitoring. The mean duration of antibody suppression was 16.8 ± 10.9 months in those who were successfully suppressed with optimization. Patients who switched to a second anti-TNF medication were not more likely to develop antibodies to the second agent.\u0000\u0000\u0000CONCLUSIONS\u0000With limited therapies for IBD and the chronicity of the disease, we advocate salvage of the current anti-TNF through dose optimization in pediatric patients with antibody level < 10 U/mL.","PeriodicalId":16725,"journal":{"name":"Journal of Pediatric Gastroenterology & Nutrition","volume":"7 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80108121","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 16
Pancreatic Non-Hodgkin's Lymphoma Presenting as Pancreatitis and Duodenal Polyps in a Pediatric Patient. 一名儿童患者的胰腺非霍奇金淋巴瘤表现为胰腺炎和十二指肠息肉。
Journal of Pediatric Gastroenterology & Nutrition Pub Date : 2019-11-01 DOI: 10.1097/MPG.0000000000002463
Grace E. Kim, Sean M Bingham, C. Gariepy
{"title":"Pancreatic Non-Hodgkin's Lymphoma Presenting as Pancreatitis and Duodenal Polyps in a Pediatric Patient.","authors":"Grace E. Kim, Sean M Bingham, C. Gariepy","doi":"10.1097/MPG.0000000000002463","DOIUrl":"https://doi.org/10.1097/MPG.0000000000002463","url":null,"abstract":"","PeriodicalId":16725,"journal":{"name":"Journal of Pediatric Gastroenterology & Nutrition","volume":"29 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83498436","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Cutaneous Leishmaniasis Presenting As Macrocheilitis In A Post Liver Transplant Pediatric Patient. 儿童肝移植后皮肤利什曼病表现为大颊炎。
Journal of Pediatric Gastroenterology & Nutrition Pub Date : 2019-10-29 DOI: 10.1097/MPG.0000000000002545
J. Menon, N. Shanmugam, M. Vij, M. Reddy, M. Rela
{"title":"Cutaneous Leishmaniasis Presenting As Macrocheilitis In A Post Liver Transplant Pediatric Patient.","authors":"J. Menon, N. Shanmugam, M. Vij, M. Reddy, M. Rela","doi":"10.1097/MPG.0000000000002545","DOIUrl":"https://doi.org/10.1097/MPG.0000000000002545","url":null,"abstract":"A 3-year-old boy, who underwent living donor liver transplantation at 1-year age for biliary atresia presented during his routine follow-up with painful perioral swelling of 2 months’ duration (Fig. 1). He was on a standard tacrolimus-based immunosuppression along with oral prednisolone 1 mg/day. Considering cellulitis, he was treated with intravenous antibiotics for 5 days, but with little improvement. Kaposi sarcoma was considered in immunosuppressed patient and lip biopsy was done, which suggested Leishmaniasis (Fig. 2; arrow showing amastigote). Liposomal Amphotericin 5 mg/kg was given for 7 days. Lesion showed progressive response with complete resolution in a month.","PeriodicalId":16725,"journal":{"name":"Journal of Pediatric Gastroenterology & Nutrition","volume":"12 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73853093","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Management of Biliary Atresia in France 1986-2015: Long Term Results. 1986-2015年法国胆道闭锁的管理:长期结果
Journal of Pediatric Gastroenterology & Nutrition Pub Date : 2019-10-01 DOI: 10.1097/MPG.0000000000002446
Martina Fanna, G. Masson, C. Capito, M. Girard, F. Guérin, B. Hermeziu, A. Lachaux, B. Roquelaure, F. Gottrand, P. Broué, A. Dabadie, T. Lamireau, E. Jacquemin, C. Chardot
{"title":"Management of Biliary Atresia in France 1986-2015: Long Term Results.","authors":"Martina Fanna, G. Masson, C. Capito, M. Girard, F. Guérin, B. Hermeziu, A. Lachaux, B. Roquelaure, F. Gottrand, P. Broué, A. Dabadie, T. Lamireau, E. Jacquemin, C. Chardot","doi":"10.1097/MPG.0000000000002446","DOIUrl":"https://doi.org/10.1097/MPG.0000000000002446","url":null,"abstract":"OBJECTIVES\u0000This study analyses the prognosis of Biliary Atresia (BA) in France since 1986, when both Kasai operation (KOp) and Liver Transplantation (LT) became widely available.\u0000\u0000\u0000METHODS\u0000The charts of all patients diagnosed with BA born between 1986 and 2015 and living in France were reviewed.\u0000\u0000\u0000RESULTS\u00001428 patients were included; 1340 (94%) underwent KOp. Total clearance of jaundice (total bilirubin ≤20 μmol/l) was documented in 516 patients (39%). Age at KOp (median 59 days, range 6-199) was stable over time. Survival with Native Liver (SNL) after KOp was 41%, 35%, 26% and 22% at 5, 10, 20 and 30 years, stable in the 4 cohorts. 25-year SNL was 38%, 27%, 22%, 19% in patients operated in the 1, 2, 3 month of life or later, respectively (p = 0.0001). Center caseloads had a significant impact on results in the 1986-96 cohort only.16%, 7%, 7%, 8% of patients died without LT in the 4 cohorts (p = 0.0001).753 patients (55%) underwent LT. Patient survival after LT was 79% at 28 years. 5-year patient survival after LT was 76%, 91%, 88%, and 92% in cohorts 1 to 4, respectively (p < 0.0001),Actual BA patient survival (from diagnosis) was 81%. 5-year BA patient survival was 72%, 88%, 87%, 87% in cohorts 1986-96, 1997-2002, 2003-09, 2010-15, respectively (p < 0.0001).\u0000\u0000\u0000CONCLUSIONS\u0000In France, 87% of BA patients survive nowadays and 22% reach the age of 30 years without transplantation. Improvement of BA prognosis is mainly due to reduced mortality before LT and better outcomes after LT.","PeriodicalId":16725,"journal":{"name":"Journal of Pediatric Gastroenterology & Nutrition","volume":"43 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77738746","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 52
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