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| 1 | Treatment of primary sclerosing cholangitis in children显示文摘Primary sclerosing cholangitis(PSC) is a rare disease of stricturing and destruction of the biliary tree with a complex genetic and environmental etiology.Most patients have co-occurring inflammatory bowel disease. Children generally present with uncomplicated disease, but undergo a variable progression to endstage liver disease. Within ten years of diagnosis, 50% of children will develop clinical complications including 30% requiring liver transplantation.Cholangiocarcinoma is a rare but serious complication affecting 1% of children.Ursodeoxycholic acid and oral vancomycin therapy used widely in children as medical therapy, and may be effective in a subset of patients. Gamma glutamyltransferase is a potential surrogate endpoint for disease activity, with improved survival in patients who achieve a normal value. Endoscopic retrograde cholangiopancreatography is a necessary adjunct to medical therapy to evaluate mass lesions or dominant strictures for malignancy, and also to relieve biliary obstruction. Liver transplantation remains the only option for patients who progress to end-stage liver disease. We review special considerations for patients before and after transplant, and in patients with inflammatory bowel disease. There is presently no published treatment algorithm or guideline for the management of children with PSC. We review the evidence for drug efficacy, dosing, duration of therapy, and treatment targets in PSC, and provide a framework for endoscopic and medical management of this complex problem. | Trevor J Laborda M Kyle Jensen Marianne Kavan Mark Deneau | 2019 | World Journal of Hepatology2019，11，1： | 2 |
| 2 | Hereditary pancreatitis:An updated review in pediatrics显示文摘Hereditary Pancreatitis(HP)has emerged as a significant cause of acute,acute recurrent and chronic pancreatitis in the pediatric population.Given that it presents similarly to other causes of pancreatitis,a positive family history and/or isolation of a gene mutation are vital in its designation.Inheritance patterns remain complex,but mutations involving the PRSS1,SPINK1,CFTR and CTRC genes are commonly implicated.Since being first described in 1952,dozens of genetic alterations that modify the action of pancreatic enzymes have been identified.Among children,these variants have been isolated in more than 50%of patients with chronic pancreatitis.Recent research has noted that such mutations in PRSS1,SPINK1 and CFTR genes are also associated with a faster progression from acute pancreatitis to chronic pancreatitis.Patients with HP are at increased risk of developing diabetes mellitus,exocrine pancreatic insufficiency,and pancreatic adenocarcinoma.Management follows a multi-disciplinary approach with avoidance of triggers,surveillance of associated conditions,treatment of pancreatic insufficiency and use of endoscopic and surgical interventions for complications.With significant sequela,morbidity and a progressive nature,a thorough understanding of the etiology,pathophysiologic mechanisms,diagnostic evaluation,current management strategies and future research considerations for this evolving disease entity in pediatrics is warranted. | Arvind Vasant Panchoo Grant H VanNess Edgardo Rivera-Rivera Trevor J Laborda | 2022 | World Journal of Clinical Pediatrics2022，11，1： | 2 |
| 3 | Dlk, a putative mammalian homeotic gene differentially expressed in small cell lung carcinoma and neuroendocrine tumor cell line显示文摘 | Laborda J Sausville EA Hoffman T | 1993 | J Biol Chem1993，268，6： | 1 |
| 4 | T cell leukemia-associated human Notch/translocation-associated Notch homologue has IkB- like activity and physically interacts with nuclear factor-kB proteins in T cells显示文摘 | GUAN E WANG J LABORDA J | 1996 | J Exp Med1996，183，5： | 1 |
| 5 | DLk,a putative mammalian homeotic gene differentially expressed in small cell lung carcinoma and neuroendocrine tumor cell line显示文摘 | Laborda J Sausville EA Hoffman T | 1993 | J Biol Chem1993，268，6： | 1 |
| 6 | Hemoptysis workup before embolization, Single-Center Experience with a 15-year Period Follow-Up显示文摘 | De Gregorio MA Medrano J Laborda A | 2007 | Tech Vasc Interventional Radiol2007，10，： | 1 |
| 7 | T cell leukemia-associated human Notch/translocation- associated Notch homologue has I kappa B-like activity and physically interacts with nuclear factor-kappa B proteins in T cells显示文摘 | Guan E Wang J Laborda J Norcross M Baeuerle P A Hoffman T | 1996 | J Exp Med1996，183，： | 1 |
| 8 | Mouse resistin modu- lates adipogenesis and glucose uptake in 3T3-L1 preadipoeytes through the ROR1 receptor 显示文摘 | Sánchez-Solana B Laborda J Baladrón V | 2012 | Mol Endocrinol2012，26，1： | 1 |
| 9 | The role of the epidermal growth factor-like protein dlk in cell differentiation显示文摘 | Laborda J | | 0，，： | 1 |
| 10 | 查看详情显示文摘 | F Laborda E Bolea J R Castillo | | 0，，： | 1 |
| 11 | Determi- nation of antimony by electrochemical hydride generation atomic absorption spectrometry in samples with high iron content using chelating resins as on-line removal system 显示文摘 | Bolea E Arroyo D Laborda F Castillo J R | 2006 | Analytica Chimica Acta2006，569，12： | 1 |
| 12 | Quality of quantitative and semiquantitative results in inductively coupled plasma mass spectrometry显示文摘 | F Laborda J Medrano J R Castillo | 2001 | J Anal At Speetrom2001，16，： | 1 |
| 13 | Quality of quantitative and semiquantitative results in inductively coupled plasma mass spectrometry 显示文摘 | F Laborda J Medrano J R Castillo | 2001 | J Anal At Spectrom2001，16，： | 1 |
| 14 | Electrochemical hydride generation as a sample-introduction technique in atomic spectrometry: fundamentals, interferences, and applications 显示文摘 | Laborda F Bolea E Castillo J R | 2007 | Anal Bioanal Chem2007，388，： | 1 |
| 15 | DLk, a putativemam- malian homeotic gene differentially expressed in small cell lung carcinoma and neuroendocrine tumor cell line 显示文摘 | Laborda J Sausville EA Hoffman T | 1993 | J Biol Chem1993，268，6： | 1 |
| 16 | DLk, a putative mammalian homeotic gene differentially expressed in small cell lung carcinoma a- nd neuroendocrine tumor cell line显示文摘 | Laborda J Sausville EA HoffmanT | 1993 | J BioChem1993，268，6： | 1 |
| 17 | The role of the epidermal growth factor like protein Dlk in cell differentiation显示文摘 | Laborda J | 2000 | Histol Histopathol2000，15，1： | 1 |
| 18 | A putative mammalian homcotic gene differentially expressed in small cell lung carcinoma and neuroendocrine tumor cell line显示文摘 | Sausville E A Hoffman T | 1993 | J Biol Chem1993，268，6： | 1 |
| 19 | The role of the epidermal growth factor like protein Dlk in cell differentiation显示文摘 | | 2000 | Histol Histopathol2000，15，1： | 1 |
| 20 | Dlkl specifically interacts with insulin-like growth factor binding protein ! to modulate adipogenesis of 3T3-L1 cells显示文摘 | Nueda M L Garcla-Ramfrez J J Laborda J | 2008 | J Mol Biol2008，379，3： | 1 |
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