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| 1 | Long-term follow-up of children and adolescents with primary sclerosing cholangitis and autoimmune sclerosing cholangitis显示文摘BACKGROUND: Sclerosing cholangitis(SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate longterm results in children with SC according to the types of SC.METHODS: We retrospectively followed up 25 children with SC over a period of 4-17 years(median 12). The diagnosis of SC was based on biochemical, histological and cholangiographic findings. Patients fulfilling diagnostic criteria for probable or definite autoimmune hepatitis at the time of diagnosis were defined as having autoimmune sclerosing cholangitis(ASC); other patients were included in a group of primary sclerosing cholangitis(PSC). The incidence of the following complications was studied: obstructive cholangitis, portal hypertension, advanced liver disease and death associated with the primary disease.RESULTS: Fourteen(56%) patients had PSC and 11(44%) had ASC. Patients with ASC were significantly younger at the time of diagnosis(12.3 vs 15.4 years, P=0.032) and had higher Ig G levels(22.7 vs 17.2 g/L, P=0.003). The mentioned complications occurred in 4(16%) patients with SC, exclusively in the PSC group: one patient died from colorectal cancer, one patient underwent liver transplantation and two patients, in whom severe bile duct stenosis was present at diagnosis, were endoscopically treated for acute cholangitis. Furthermore, twoother children with ASC and 2 children with PSC had elevated aminotransferase levels. The 10-year overall survival was 95.8% in all patients, 100% in patients without complicated liver disease, and 75.0% in patients with complications.CONCLUSION: In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC. | Vratislav Smolka Eva Karaskova Oksana Tkachyk Kvetoslava Aiglova Jiri Ehrmann Kamila Michalkova Michal Konecny Jana Volejnikova | 2016 | Hepatobiliary & Pancreatic Diseases International2016，15，4： | 2 |
| 2 | Effects of race and family history of type 2 diabetes on metabolie status of women withpolycystie ovary syndrome显示文摘 | Ehrmann DA Kasza K Azziz R | 2005 | J Clin Endocrinol Metab2005，90，1： | 1 |
| 3 | Relationship of calpain-10 genotype to phenotypic features of polycystic ovary syndrome显示文摘 | Ehrmann D A Schwarz P E Hara M | 2002 | J Clin Endocrinol Metab2002，87，4： | 1 |
| 4 | Prevalence of impaired glucose tolerance and diabetes in women with polycystic ovary syndrome显示文摘 | Ehrmann DA Barnes RB Rosenfield RL | 1999 | Diabetes Care1999，22，： | 1 |
| 5 | Polycystic ovary syndrome显示文摘 | Ehrmann D A | 2005 | N Engl J Med2005，352，： | 1 |
| 6 | Communication by Central Bank Committee Members:Different Strategies, Same Effectiveness显示文摘 | Ehrmann Michael Fratzscher Marcel | 2007 | Journal of Money Credit and Banking2007，39，2： | 1 |
| 7 | Prevalence and predictors of the metabolic syndrome in women with polycystic ovary syndrome显示文摘 | Ehrmann DA Liljenquist DR Kasza K | | 0，，： | 1 |
| 8 | Metabolic activity and symbiotic interactions of lactic acid bacteria and yeasts isolated from water kefir显示文摘 | Jasmin Stadie Anna Gulitz Matthias A. Ehrmann Rudi F. Vogel | 2013 | Food Microbiology2013，，2： | 1 |
| 9 | Euro area inflation differentials显示文摘 | Angeloni I Ehrmann M | | 0，，01： | 1 |
| 10 | Modes of periacetabular load transfer to cortical and cancellous bone after cemented versus uncement- ed total hip arthroplasty:a prospective study using computed tomography- assisted osteodensitometry 显示文摘 | Mueller LA Sehmidt R Ehrmann C | 2009 | J Orth0p Res2009，27，2： | 1 |
| 11 | Diagnosis and treatment of polyeystic ovary syndrome: an Endocrine Society clinical prac- tice guideline 显示文摘 | Legro RS Arslanian SA Ehrmann DA | 2013 | J Clin Endocrinol Metab2013，98，12： | 1 |
| 12 | Relation between mean arterial pressure and renal function in the early phase of shock: a prospective, explorative cohort study 显示文摘 | Badin J Boulain T Ehrmann S | 2011 | Crit Care2011，15，3： | 1 |
| 13 | Multi-sensor Microsystems for Contaminants in Air显示文摘 | Althainz P Goschnick J Ehrmann S | 1996 | Sensors and Actuators B1996，33，： | 1 |
| 14 | The relationship of intraca- nal medicaments to postoperative pain in endodontics显示文摘 | Ehrmann EH Messer HH Adams GG | 2003 | Int Endod J2003，36，12： | 1 |
| 15 | Water balance in a banded vegeta- tion pattern : A case study of tiger bush in western Niger显示文摘 | Galle S Ehrmann M Peugeot C | 1999 | Catena1999，37，12： | 1 |
| 16 | Diagnosis and treatment of polycystic ovary syndrome:Society clinical practice guidline显示文摘 | Legro RS Arslanian SA Ehrmann DA | 2012 | J Clin Endocrinol Metab2012，98，12： | 1 |
| 17 | Polycystic ovary syndrome显示文摘 | Ehrmann DA | 2005 | N Engl J Mcd2005，352，： | 1 |
| 18 | A temperature-dependent switch from chaperone to protease in a widely conserved heat shock protein显示文摘 | Spiess C Beil A Ehrmann M | 1999 | Cell1999，97，3： | 1 |
| 19 | A reriew of peripheral refraction techniques显示文摘 | Fedtke C Ehrmann K Holden BA | 2009 | Optom Vis Sci2009，86，3： | 1 |
| 20 | Prevalence and predictors of the metabolic syndrome in women with polycystic ovary syndrome 显示文摘 | Ehrmann D A Liljenquist D R Kasza K | 2006 | J Clin Endocrinol Metab2006，91，1： | 1 |
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