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Corticosteroids for Caustic Esophageal Burns. 糖皮质激素治疗食管烧灼性烧伤。
Pub Date : 2019-12-01 DOI: 10.1097/MPG.0000000000002508
R. Hoffman, M. Burns, S. Gosselin
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引用次数: 0
A Pilot Study of the pedsPCF Item Bank as a Screening Tool for Pediatric Minimal Hepatic Encephalopathy. pespcf题库作为儿童轻度肝性脑病筛查工具的初步研究。
Pub Date : 2019-12-01 DOI: 10.1097/MPG.0000000000002488
Daniella Ohnemus, K. Neighbors, Lisa G. Sorensen, J. Lai, E. Alonso
OBJECTIVESDespite the need for monitoring cognition for minimal hepatic encephalopathy (MHE) in children with portal hypertension, few screening methods exist. The Patient-Reported Outcomes Measurement Information System (PROMIS) Pediatric Cognitive Function (PedsPCF) item bank, a 43-item parent- and self-report questionnaire, could be a useful screening tool. This study aimed to evaluate the PedsPCF item bank as a screening tool and explore its correlation with other neurocognitive measures and clinical indicators of portal hypertension.METHODSPediatric patients with portal hypertension were recruited at Lurie Children's Liver Clinic. A short battery of neuropsychological tests tapping attention, executive functioning, and fine motor speed was administered along with surveys of cognitive functioning (PedsPCF, Behavior Rating Inventory of Executive Function; BRIEF) and quality of life (PROMIS Pediatric-25 Profile).RESULTS18 patients participated (ages 8 to 17). The PedsPCF correlated well with the BRIEF but did not correlate with neurocognitive testing. Qualitative heatmap analysis of the relationship between z-scores and clinical signs of portal hypertension suggests the PedsPCF is less sensitive than the BRIEF. The fine motor task (Grooved Pegboard) appears to offer the highest sensitivity of the tests administered and is also relatively quick and easy to administer.CONCLUSIONSElements of the battery show promise in this small pilot sample. The BRIEF and the Grooved Pegboard may hold the most potential for screening in the clinical setting. Further study is necessary to examine this question in a larger multi-center sample.
目的:尽管有必要监测门静脉高压症患儿轻度肝性脑病(MHE)的认知,但现有的筛查方法很少。患者报告结果测量信息系统(PROMIS)儿科认知功能(PedsPCF)题库是一份43项家长和自我报告的问卷,可能是一个有用的筛查工具。本研究旨在评估PedsPCF题库作为筛查工具的价值,并探讨其与门静脉高压症其他神经认知指标和临床指标的相关性。方法在卢里儿童肝脏诊所招募门静脉高压症患儿。一组简短的神经心理学测试,包括注意力、执行功能和精细运动速度,以及认知功能调查(PedsPCF,执行功能行为评定量表;BRIEF)和生活质量(PROMIS pediatrics -25 Profile)。结果18例患者参与,年龄8 ~ 17岁。PedsPCF与BRIEF相关良好,但与神经认知测试无关。定性热图分析z分数与门静脉高压症临床体征之间的关系表明,PedsPCF的敏感性低于BRIEF。精细运动任务(槽钉板)似乎提供了最高的灵敏度的测试,也相对快速和容易管理。结论该电池的成分在这个小的试点样品中显示出希望。BRIEF和槽状钉板可能在临床筛查中最有潜力。进一步的研究需要在更大的多中心样本中检验这个问题。
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引用次数: 2
Response to Letter: Corticosteroids for Oesophageal Caustic Injuries. 对信函的回应:糖皮质激素治疗食道腐蚀性损伤。
Pub Date : 2019-12-01 DOI: 10.1097/MPG.0000000000002509
A. Akobeng, I. Abdelgadir
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引用次数: 0
Enhancing the Patient Experience for Individuals Undergoing Endoscopic Procedures Using Virtual Reality. 使用虚拟现实增强个体内窥镜手术的患者体验。
Pub Date : 2019-11-26 DOI: 10.1097/MPG.0000000000002577
A. Palanica, M. Leier, Andrew Lee, Yan Fossat, Michael Docktor
The results of medical procedures can often be difficult to translate into comprehensible and engaging information for patients. This randomized controlled trial evaluated the satisfaction and perceived value of a technology, called HealthVoyager, which creates a personalized virtual reality (VR) experience of a patient's endoscopy or colonoscopy findings in comparison to the standard practice (i.e., reviewing printed reports). The platform allows gastroenterologists to create a customized VR patient report to help translate medical knowledge and procedural information to the patient. Forty-one patients (17 HealthVoyager [test]; 24 standard practice [control]) completed a self-report survey assessing their experience for receiving medical information. Results demonstrated that patients were significantly more satisfied in learning about their gastrointestinal condition and procedural results using HealthVoyager rather than with the standard of care. These results have implications for improving the knowledge translation of medical findings between healthcare providers and patients in various disease states and patient populations.
医疗程序的结果往往难以转化为可理解和吸引患者的信息。这项随机对照试验评估了一项名为HealthVoyager的技术的满意度和感知价值,该技术为患者的内窥镜检查或结肠镜检查结果创造了个性化的虚拟现实(VR)体验,与标准实践(即审查打印报告)进行了比较。该平台允许胃肠病学家创建定制的VR患者报告,以帮助将医学知识和程序信息翻译给患者。41例(HealthVoyager[试验]17例;24标准做法[对照])完成了一项自我报告调查,评估他们接受医疗信息的经验。结果表明,患者在使用HealthVoyager而不是使用标准护理来了解他们的胃肠道状况和手术结果时明显更满意。这些结果对改善不同疾病状态和患者群体中医疗保健提供者和患者之间医学发现的知识转化具有重要意义。
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引用次数: 1
The Impact of Celiac Disease on Caregivers' Well-Being: An Integrative Review. 乳糜泻对照顾者幸福感的影响:一项综合综述
Pub Date : 2019-11-26 DOI: 10.1097/MPG.0000000000002572
R. Satherley, S. Coburn, Monique M. Germone
OBJECTIVESA diagnosis of celiac disease (CD) requires individuals to adopt a strict gluten-free diet (GFD). As children with CD must rely on their caregivers for guidance and support with managing the GFD, CD may challenge the caregiver's emotional and social well-being. The primary objective of this mixed-methods systematic review was to synthesize of research investigating the impact of CD on caregiver's well-being.METHODSFive databases were systematically searched from 1990-2018 to identify all empirical studies that assessed well-being in caregivers of children (0-18 years) with CD. Qualitative and quantitative data were extracted separately before being integrated to explore key themes across the studies.RESULTS12 studies were identified that explored the well-being of caregivers of children with CD (3 qualitative, 9 quantitative), reporting on 665 caregivers. The quality of evidence was limited across studies. Synthesis of results revealed three themes (Caregiver Responsibility, Caregiver Well-Being and Concern for Child's Health, Implications for the Family) describing the impact of a child with CD on caregiver well-being.CONCLUSIONSCaregivers of children with CD may experience difficulties that impact their well-being; specific difficulties identified included the impact of caregivers' social activities, finances and anxiety. The findings detailed in this review point towards factors that may guide health care personnel to provide support for the caregivers of children with CD.
目的乳糜泻(CD)的诊断要求个体采用严格的无谷蛋白饮食(GFD)。由于患有乳糜泻的儿童必须依靠照顾者的指导和支持来管理GFD,乳糜泻可能会挑战照顾者的情感和社会福祉。这项混合方法系统综述的主要目的是综合调查乳糜泻对护理者幸福感影响的研究。方法系统检索1990-2018年期间的5个数据库,以确定评估CD儿童(0-18岁)照顾者幸福感的所有实证研究。分别提取定性和定量数据,然后整合以探索研究中的关键主题。结果共有12项研究(3项定性研究,9项定量研究)对665名护理人员进行了调查。所有研究的证据质量有限。综合结果揭示了三个主题(照顾者责任、照顾者福祉和对儿童健康的关注、对家庭的影响),描述了患有乳糜泻的儿童对照顾者福祉的影响。结论:乳糜泻儿童的照顾者可能会遇到影响他们健康的困难;确定的具体困难包括照顾者的社会活动、经济状况和焦虑的影响。本综述中详细的研究结果指出了可能指导卫生保健人员为乳糜泻儿童的照顾者提供支持的因素。
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引用次数: 6
Trough Measurements of Infliximab: The Earlier the Better? 英夫利昔单抗:越早越好?
Pub Date : 2019-11-26 DOI: 10.1097/MPG.0000000000002576
R. Harris, R. Jackson, R. Russell
10. Weisbrod VM, Silvester JA, Raber C, et al. Preparation of gluten-free foods alongside gluten-containing food may not always be as risky for celiac patients as diet guides suggest. Gastroenterology 2019;158:273–5. 11. Hollon JR, Cureton PA, Martin ML, et al. Trace gluten contamination may play a role in mucosal and clinical recovery in a subgroup of diet-adherent non-responsive celiac disease patients. BMC Gastroenterol 2013;13:40. 12. Shah S, Akbari M, Vanga R, et al. Patient perception of treatment burden is high in celiac disease compared with other common conditions. Am J Gastroenterol 2014;109:1304–11.
10. Weisbrod VM, Silvester JA, Raber C,等。对乳糜泻患者来说,将无麸质食物和含麸质食物一起准备并不总是像饮食指南所建议的那样危险。胃肠病学2019;158:273-5。11. Hollon JR, Cureton PA, Martin ML,等。微量麸质污染可能在饮食依从性无反应性乳糜泻患者亚组的粘膜和临床恢复中发挥作用。中华医学会胃肠病学杂志2013;13:40。12. Shah S, Akbari M, Vanga R,等。与其他常见疾病相比,乳糜泻患者对治疗负担的感知较高。[J] .中华肠胃病杂志,2014;39(1):1 - 4。
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引用次数: 0
Intralesional Steroid Injection Therapy for Esophageal Anastomotic Stricture Following Esophageal Atresia Repair. 食道闭锁修复后食管吻合口狭窄局内类固醇注射治疗。
Pub Date : 2019-11-19 DOI: 10.1097/MPG.0000000000002562
P. Ngo, Ali Kamran, Susannah J. Clark, R. Jennings, Thomas E. Hamilton, C. Smithers, B. Zendejas, Jessica L Yasuda, D. Zurakowski, M. Manfredi
OBJECTIVESThe role of intralesional steroid injection (ISI) in the treatment of anastomotic stricture in esophageal atresia (EA) patients remains unclear. The aim of this study was to evaluate the efficacy and safety of ISI.METHODS158 EA patients with at least one ISI for the treatment of esophageal anastomotic stricture between 2010 and 2017 were identified. The change in stricture diameter (ΔD) was compared between procedures with dilation alone (ISI-) and dilation with steroid injection (ISI+).RESULTSA total of 1055 balloon dilations were performed (452 ISI+). The median ΔD was significantly greater in the ISI+ group: 1 mm (IQR 0, 3) vs. 0 mm (IQR -1, 1.5) (P < 0.0001). The ISI+ group had greater percentage of improved diameter (P < 0.0001) and lesser percentages of unchanged and decreased diameters at subsequent endoscopy (P = 0.0009, P = 0.003). Multivariable logistic regression confirmed the significance of ISI on increasing the likelihood of improved stricture diameter with an adjusted odds ratio of 3.24 (95% CI: 2.15-4.88) (P < 0.001). The ΔD for the first 3 ISI+ procedures was greater than the ΔD for subsequent ISI+ procedures: 1 mm (IQR 0, 3) vs. 0.5 mm (IQR -1.25, 2) (P = 0.001). There was no difference in perforation incidence between ISI+ and ISI- groups (P = 0.82).CONCLUSIONSISI with dilation was well tolerated and improved anastomotic stricture diameter more than dilation alone. The benefit of ISI over dilation alone was limited to the first 3 ISI procedures.
目的病灶内类固醇注射(ISI)在食管闭锁(EA)患者吻合口狭窄治疗中的作用尚不清楚。本研究的目的是评价ISI的有效性和安全性。方法选取2010 ~ 2017年间,应用至少一种ISI治疗食管吻合口狭窄的EA患者158例。比较单纯扩张术(ISI-)和类固醇注射扩张术(ISI+)的狭窄直径变化(ΔD)。结果共行球囊扩张1055例,其中ISI+ 452例。ISI+组的中位ΔD显著增大:1 mm (IQR 0,3) vs. 0 mm (IQR - 1,1.5) (P < 0.0001)。ISI+组在随后的内镜检查中,直径改善的百分比更高(P < 0.0001),直径不变和减小的百分比更低(P = 0.0009, P = 0.003)。多变量logistic回归证实了ISI对提高狭窄直径改善可能性的显著性,校正比值比为3.24 (95% CI: 2.15-4.88) (P < 0.001)。前3次ISI+手术的ΔD大于后续ISI+手术的ΔD: 1 mm (IQR 0,3) vs. 0.5 mm (IQR -1.25, 2) (P = 0.001)。ISI+组与ISI-组穿孔发生率差异无统计学意义(P = 0.82)。结论sisi联合扩张术耐受性良好,比单纯扩张术更能改善吻合口狭窄直径。ISI优于单纯扩张的益处仅限于前3次ISI手术。
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引用次数: 16
Clinical Value of Serum Interleukin-33 Biomarker in Infants with Neonatal Cholestasis. 血清白细胞介素-33生物标志物在新生儿胆汁淤积症中的临床价值
Pub Date : 2019-11-19 DOI: 10.1097/MPG.0000000000002565
O. Behairy, A. Elsadek, E. Behiry, Ibrahim A. Elhenawy, N. Shalan, Kamal R Sayied
OBJECTIVESThis study aimed to estimate the value of serum IL-33 levels in infants with cholestasis, correlate serum IL-33 levels with the clinicopathological profile of infants with cholestasis and compare its level with that of healthy infants who served as control METHODS:: sixty infants with cholestasis were enrolled in this study and divided into biliary atresia group and non-biliary atresia group, in addition to thirty healthy infants as a control group. All infants were analyzed for their clinical, biochemical features, histopathological profile and serum level of IL- 33 by ELISA.RESULTSserum level of IL -33 in BA group (median 48.0, IQR: 28.9-106.2) was significantly higher than the non-BA group (median 17.3, IQR: 13.7-18.8 pg/ml) and both were higher than the control group. There was a positive correlation between serum IL-33 and AST, ALT, bilirubin (total and direct) levels and fibrosis stage among the BA group. Serum IL-33 at a cut-off value of 20.8 pg/ml can detect biliary atresia with a specificity of 95% and a sensitivity of 96.7%.CONCLUSIONThe significantly higher production of IL -33 in patients with BA compared to non-BA suggests a potential role of IL -33 for initiation and progression of the disease process. also, IL -33 may have a diagnostic role in infants with BA.
目的探讨胆汁淤积症患儿血清IL-33水平的变化,探讨血清IL-33水平与胆汁淤积症患儿临床病理特征的相关性,并与健康婴儿进行比较。方法:将60例胆汁淤积症患儿分为胆道闭锁组和非胆道闭锁组,并将30例健康婴儿作为对照组。采用ELISA法分析所有患儿的临床、生化、组织病理学特征及血清IL- 33水平。结果BA组血清IL -33水平(中位数48.0,IQR: 28.9 ~ 106.2)显著高于非BA组(中位数17.3,IQR: 13.7 ~ 18.8 pg/ml),且均高于对照组。BA组血清IL-33与AST、ALT、总胆红素和直接胆红素水平及纤维化分期呈正相关。血清IL-33截断值为20.8 pg/ml时,检测胆道闭锁的特异性为95%,敏感性为96.7%。结论:与非BA患者相比,BA患者IL -33的产生明显增加,表明IL -33在疾病过程的开始和进展中可能起作用。此外,IL -33可能在BA婴儿中具有诊断作用。
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引用次数: 11
Anti-TNF Exposure Impacts Vedolizumab Mucosal Healing Rates in Pediatric Inflammatory Bowel Disease. 抗肿瘤坏死因子暴露影响儿童炎症性肠病Vedolizumab粘膜愈合率
Pub Date : 2019-11-14 DOI: 10.1097/MPG.0000000000002556
J. Jossen, B. Kiernan, N. Pittman, M. Dubinsky
BACKGROUNDVedolizumab (VDZ) is effective for treating both adult and pediatric onset IBD. Clinical outcomes, however, have been reported to be superior in patients naïve to anti-TNF. With the growing interest in endoscopic endpoints, we aimed to describe rates of mucosal healing in pediatric patients being treated with VDZ and examine the influence of anti-TNF on outcomes.METHODSWe conducted a retrospective review of all IBD patients ≤21 years of age who initiated VDZ and underwent endoscopy. Primary outcome was mucosal healing (composite of endoscopic (SES-CD and Mayo score UC) and histological remission (Nancy index-UC and CD histologic activity). Descriptive statistics summarized the data. Comparisons were made for endpoints based on anti-TNF exposure using univariate testing.RESULTSSixty-eight patients were included in the final analysis; 35 with UC and 33 with CD. Thirty-two patients (22 UC and 10 CD) were anti-TNF naïve and 36 patients (13 UC and 23 CD) were anti-TNF exposed. The median duration on VDZ prior to endoscopic assessment was 49 (IQR 32-73) weeks. A total of 38% (25/66) of patients met the primary outcome of mucosal healing and did not differ between anti-TNF naïve or exposed. Endoscopic remission was achieved by 51% with significantly more anti-TNF naïve patients reaching this endpoint (66% v. 40%, p = 0.03). Histologic remission was achieved by 42% of patients with a non-significant trend towards improved histologic remission rates in anti-TNF naïve patients (52% v. 33%, p = 0.13).CONCLUSIONVDZ is associated with mucosal healing in pediatric IBD. Anti-TNF exposure significantly impacted endoscopic remission, but not histologic remission in children on VDZ.
背景:vedolizumab (VDZ)对成人和儿童IBD均有效。然而,据报道,naïve患者的临床结果优于抗tnf。随着对内镜终点的兴趣日益增长,我们旨在描述接受VDZ治疗的儿科患者的粘膜愈合率,并检查抗tnf对结果的影响。方法我们对所有≤21岁开始VDZ并接受内镜检查的IBD患者进行回顾性分析。主要结局是粘膜愈合(内镜下(SES-CD和Mayo评分)UC)和组织学缓解(Nancy指数-UC和CD的组织学活性)。描述性统计汇总了数据。使用单变量测试对基于抗tnf暴露的终点进行比较。结果68例患者纳入最终分析;35例合并UC, 33例合并CD。32例(22例合并UC, 10例合并CD)抗tnf naïve, 36例(13例合并UC, 23例合并CD)抗tnf暴露。内镜评估前VDZ的中位持续时间为49周(IQR 32-73)。共有38%(25/66)的患者达到了粘膜愈合的主要结局,抗tnf naïve和暴露tnf之间没有差异。内镜下缓解率为51%,达到这一终点的抗tnf naïve患者显著增加(66% vs 40%, p = 0.03)。42%的患者达到组织学缓解,抗tnf naïve患者的组织学缓解率改善趋势不显著(52% vs 33%, p = 0.13)。结论vdz与儿童IBD的黏膜愈合有关。抗tnf暴露显著影响VDZ患儿的内窥镜缓解,但不影响组织学缓解。
{"title":"Anti-TNF Exposure Impacts Vedolizumab Mucosal Healing Rates in Pediatric Inflammatory Bowel Disease.","authors":"J. Jossen, B. Kiernan, N. Pittman, M. Dubinsky","doi":"10.1097/MPG.0000000000002556","DOIUrl":"https://doi.org/10.1097/MPG.0000000000002556","url":null,"abstract":"BACKGROUND\u0000Vedolizumab (VDZ) is effective for treating both adult and pediatric onset IBD. Clinical outcomes, however, have been reported to be superior in patients naïve to anti-TNF. With the growing interest in endoscopic endpoints, we aimed to describe rates of mucosal healing in pediatric patients being treated with VDZ and examine the influence of anti-TNF on outcomes.\u0000\u0000\u0000METHODS\u0000We conducted a retrospective review of all IBD patients ≤21 years of age who initiated VDZ and underwent endoscopy. Primary outcome was mucosal healing (composite of endoscopic (SES-CD and Mayo score UC) and histological remission (Nancy index-UC and CD histologic activity). Descriptive statistics summarized the data. Comparisons were made for endpoints based on anti-TNF exposure using univariate testing.\u0000\u0000\u0000RESULTS\u0000Sixty-eight patients were included in the final analysis; 35 with UC and 33 with CD. Thirty-two patients (22 UC and 10 CD) were anti-TNF naïve and 36 patients (13 UC and 23 CD) were anti-TNF exposed. The median duration on VDZ prior to endoscopic assessment was 49 (IQR 32-73) weeks. A total of 38% (25/66) of patients met the primary outcome of mucosal healing and did not differ between anti-TNF naïve or exposed. Endoscopic remission was achieved by 51% with significantly more anti-TNF naïve patients reaching this endpoint (66% v. 40%, p = 0.03). Histologic remission was achieved by 42% of patients with a non-significant trend towards improved histologic remission rates in anti-TNF naïve patients (52% v. 33%, p = 0.13).\u0000\u0000\u0000CONCLUSION\u0000VDZ is associated with mucosal healing in pediatric IBD. Anti-TNF exposure significantly impacted endoscopic remission, but not histologic remission in children on VDZ.","PeriodicalId":16725,"journal":{"name":"Journal of Pediatric Gastroenterology & Nutrition","volume":"47 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82711840","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: 10
Normal Gamma Glutamyl Transferase Levels at Presentation Predict Poor Outcome in Biliary Atresia. 出现时正常的谷氨酰转移酶水平可预测胆道闭锁的不良预后。
Pub Date : 2019-11-14 DOI: 10.1097/MPG.0000000000002563
S. Shankar, R. Bolia, H. Foo, C. D'Arcy, N. Hardikar, Martin Wensing, W. Hardikar
OBJECTIVESGamma-glutamyl transferase levels (GGT) are typically elevated in biliary atresia (BA), but normal GGT levels have been observed. This cohort of 'normal GGT' BA has not been described nor has the prognostic value of GGT level on outcomes in BA. We aimed to 1) describe outcomes of a single-centre Australian cohort of infants with biliary atresia and 2) assess the impact of GGT level at presentation on outcomes in BA.METHODSInfants diagnosed with BA between 1991 - 2017 were retrospectively analysed. Outcomes were defined as survival with native liver, liver transplantation (LT) and death. Patients were categorised into normal (<200IU/L) or high GGT groups based on a mean of three consecutive GGT values done prior to Kasai portoenterostomy (KPE). Baseline parameters, age at surgery, clearance of jaundice and outcomes were compared between the two groups.RESULTS113 infants underwent KPE at median 61 (30-149) days. At a median follow-up of 14.2 (0.9-26.3) years, 35% (39/113) patients were surviving with native liver, 55% (62/113) underwent LT and 11% (12/113) died pre-transplant. 12.3% (14/113) patients had normal GGT. Age at KPE and time to clearance of jaundice were similar between normal and high GGT groups. Normal GGT group had shorter time from KPE to LT (11 vs 18months, p = 0.02), underwent LT at a younger age (14 vs 20months, p = 0.04) and had poorer transplant-free survival (p = 0.04) than high GGT group.CONCLUSION12.3% of infants with BA had normal GGT levels at diagnosis. Low GGT levels at presentation in biliary atresia was associated with a poorer outcome.
目的:γ -谷氨酰转移酶(GGT)水平在胆道闭锁(BA)中通常升高,但也观察到正常的GGT水平。这组“正常GGT”的BA尚未被描述,也没有GGT水平对BA预后的预测价值。我们的目的是1)描述澳大利亚单中心胆道闭锁婴儿队列的结果,2)评估出现时GGT水平对BA结果的影响。方法对1991 - 2017年诊断为BA的婴儿进行回顾性分析。结果定义为天然肝生存、肝移植(LT)和死亡。根据Kasai门肠造口术(KPE)前平均连续三次GGT值,将患者分为正常(<200IU/L)或高GGT组。比较两组患者的基线参数、手术年龄、黄疸清除情况和结果。结果113名婴儿在中位时间61(30-149)天接受了KPE。在14.2(0.9-26.3)年的中位随访中,35%(39/113)的患者存活于原生肝脏,55%(62/113)的患者接受了肝移植,11%(12/113)的患者在移植前死亡。12.3%(14/113)患者GGT正常。正常组和高GGT组的KPE年龄和黄疸清除时间相似。与高GGT组相比,正常GGT组从KPE到LT的时间更短(11个月vs 18个月,p = 0.02),接受LT的年龄更早(14个月vs 20个月,p = 0.04),无移植生存期更差(p = 0.04)。结论12.3%的BA患儿在诊断时GGT水平正常。胆道闭锁患者出现低GGT水平与较差的预后相关。
{"title":"Normal Gamma Glutamyl Transferase Levels at Presentation Predict Poor Outcome in Biliary Atresia.","authors":"S. Shankar, R. Bolia, H. Foo, C. D'Arcy, N. Hardikar, Martin Wensing, W. Hardikar","doi":"10.1097/MPG.0000000000002563","DOIUrl":"https://doi.org/10.1097/MPG.0000000000002563","url":null,"abstract":"OBJECTIVES\u0000Gamma-glutamyl transferase levels (GGT) are typically elevated in biliary atresia (BA), but normal GGT levels have been observed. This cohort of 'normal GGT' BA has not been described nor has the prognostic value of GGT level on outcomes in BA. We aimed to 1) describe outcomes of a single-centre Australian cohort of infants with biliary atresia and 2) assess the impact of GGT level at presentation on outcomes in BA.\u0000\u0000\u0000METHODS\u0000Infants diagnosed with BA between 1991 - 2017 were retrospectively analysed. Outcomes were defined as survival with native liver, liver transplantation (LT) and death. Patients were categorised into normal (<200IU/L) or high GGT groups based on a mean of three consecutive GGT values done prior to Kasai portoenterostomy (KPE). Baseline parameters, age at surgery, clearance of jaundice and outcomes were compared between the two groups.\u0000\u0000\u0000RESULTS\u0000113 infants underwent KPE at median 61 (30-149) days. At a median follow-up of 14.2 (0.9-26.3) years, 35% (39/113) patients were surviving with native liver, 55% (62/113) underwent LT and 11% (12/113) died pre-transplant. 12.3% (14/113) patients had normal GGT. Age at KPE and time to clearance of jaundice were similar between normal and high GGT groups. Normal GGT group had shorter time from KPE to LT (11 vs 18months, p = 0.02), underwent LT at a younger age (14 vs 20months, p = 0.04) and had poorer transplant-free survival (p = 0.04) than high GGT group.\u0000\u0000\u0000CONCLUSION\u000012.3% of infants with BA had normal GGT levels at diagnosis. Low GGT levels at presentation in biliary atresia was associated with a poorer outcome.","PeriodicalId":16725,"journal":{"name":"Journal of Pediatric Gastroenterology & Nutrition","volume":"31 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75052734","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: 11
期刊
Journal of Pediatric Gastroenterology & Nutrition
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