Pub Date : 2019-11-01DOI: 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
OBJECTIVES To 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. METHODS The 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). RESULTS 229 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). CONCLUSIONS We 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.
{"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}
Pub Date : 2019-11-01DOI: 10.1097/MPG.0000000000002476
Sdepanian Vl, Lopes Lhc, Oliveira Rp, Muniz Jg
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].
{"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}
Pub Date : 2019-11-01DOI: 10.1097/MPG.0000000000002448
H. Aly, L. Mohsen, I. Bhattacharjee, Amr Malash, Amr Atyia, Sherif Elanwary, R. Hawary
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.
本研究的目的是评估两种不同剂量的维生素D对早产儿T调节细胞(Treg)表达的影响。对胎龄28 ~ 33周的早产儿进行了双盲随机对照试验。当受试者达到100 ml/kg肠内饲料时，随机分配接受400或800 IU/天的维生素D3。在入组时，以及两组按规定剂量口服维生素D 1周和4周后，流式细胞术测量Treg细胞计数的百分比增加。还评估了短期发病率和死亡率结果。共有40名婴儿被纳入研究，每组20名。低剂量维生素D3补充组1周后Treg计数(%)的变化(1.9±5.5 vs 60±5.6,p = 0.0005)和4周后(1.8±5.7 vs 73.7±5.6,p = 0.0028)显著减少。两组在人体测量、供氧和呼吸支持持续时间以及死亡率方面没有差异。低剂量组住院时间更长(24.9±5.14 vs 22±3.49,p = 0.04)。口服维生素D补充剂对早产儿Treg百分比具有剂量和时间依赖性。800国际单位剂量的维生素D3没有明显的短期副作用。需要更大规模的研究来探索维生素D3剂量对住院时间长短的影响。
{"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}
Pub Date : 2019-11-01DOI: 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
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.
{"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}
Pub Date : 2019-11-01DOI: 10.1097/MPG.0000000000002451
Katarina Johansson, F. Norström, Katrina Nordyke, Anna Myléus
OBJECTIVES The 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". METHODS Through 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. RESULTS Among 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). CONCLUSIONS The 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.
{"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}
Pub Date : 2019-11-01DOI: 10.1097/MPG.0000000000002440
R. Cohen, B. Schoen, S. Kugathasan, Cary G. Sauer
BACKGROUND Treatment 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. RESULTS Medical 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. CONCLUSIONS With 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.
{"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}
Pub Date : 2019-11-01DOI: 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}
Pub Date : 2019-10-29DOI: 10.1097/MPG.0000000000002545
J. Menon, N. Shanmugam, M. Vij, M. Reddy, M. Rela
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.
{"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}
Pub Date : 2019-10-01DOI: 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
OBJECTIVES This study analyses the prognosis of Biliary Atresia (BA) in France since 1986, when both Kasai operation (KOp) and Liver Transplantation (LT) became widely available. METHODS The charts of all patients diagnosed with BA born between 1986 and 2015 and living in France were reviewed. RESULTS 1428 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). CONCLUSIONS In 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.
{"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}
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