Primary sclerosing cholangitis (PSC): clinical, laboratory and survival analysis in children and adults

Floreani, Annarosa; Zancan, Lucia; Melis, Alessandro; Baragiotta, Anna; Chiaramonte, M.

doi:10.1111/j.1478-3231.1999.tb00040.x

Cited by 36 publications

(27 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This proportion is greater than the 28% prevalence reported by others using different diagnostic criteria, 5 and substantially greater than the 7.1% to 10.6% prevalence found in the adult population using the same international scoring system. 9,21,22 These reports, [4][5][6]20 along with the current study, suggest that the expression of PSC in children is skewed toward a hepatic presentation instead of a predominantly cholestatic profile as occurs in the adult population. Whether this difference represents changes occurring in immune homeostasis with maturity or a longer environmental exposure to putative antigens remains elusive at this time.…”

Section: Discussionsupporting

confidence: 60%

Primary sclerosing cholangitis in children: A long-term follow-up study

Feldstein¹

2003

Hepatology

229

214

View full text Add to dashboard Cite

Primary sclerosing cholangitis (PSC) is increasingly diagnosed in children and adolescents, but its long-term prognosis remains uncertain. The aim of this longitudinal, cohort study was to determine the long-term outcome of children with PSC. Fifty-two children with cholangiography-proven PSC (34 boys and 18 girls; mean age 13.8 ؎ 4.2 years; range, 1.5-19.6 years) who were seen at our institution over a 20-year period were followed-up for up to 16.7 years. Two thirds presented with symptoms and/or signs of PSC and 81% had concomitant inflammatory bowel disease (IBD). Twenty-five percent had total alkaline phosphatase activity within the normal range for the age group, but all of them had elevated ␥-glutamyl transpeptidase levels. Autoimmune hepatitis overlapping with PSC was present in 35% of children. A positive but transient clinical and/or biochemical response occurred under therapy with ursodeoxycholic acid, alone or in combination with immunosuppressive medications. During follow-up, 11 children underwent liver transplantation for end-stage PSC and 1 child died. The median (50%) survival free of liver transplantation was 12.7 years. Compared with an age-and gender-matched U.S. population, survival was significantly shorter in children with PSC (P < .001). In a Cox regression model, lower platelet count, splenomegaly, and older age were associated with shorter survival. Presence of autoimmune hepatitis overlapping with PSC (P ‫؍‬ .2) or medical therapy (P ‫؍‬ .2) did not affect survival. In conclusion, PSC significantly decreases survival in this child population. Although pharmacologic therapy may improve symptoms and liver test results initially, it does not seem to impact the long-term outcome. (HEPATOLOGY 2003;38:210-217.)

show abstract

Section: Discussionsupporting

confidence: 60%

Primary sclerosing cholangitis in children: A long-term follow-up study

Feldstein¹

2003

Hepatology

229

214

View full text Add to dashboard Cite

show abstract

“…Bile duct epithelium frequently shows degenerative changes. In our experience, most children present with histologically advanced disease, mirroring the experience of Floreani and colleagues, who found that children with PSC tended to present with a greater severity of clinical and histologic activity than adults (Floreani et al 1999b ). 10.14 b ).…”

Section: Pathologysupporting

confidence: 83%

Pathology of Pediatric Gastrointestinal and Liver Disease

2014

View full text Add to dashboard Cite

“…PSC is another chronic liver disorder shown by inflammatory destruction of small intrahepatic bile ducts as well as extrahepatic bile ducts. 118 PSC is strongly related to inflammatory bowel disease, colorectal cancer, osteoporosis, and cholangiocarcinoma. 119, 120, 121 The role of specific gene deficiencies in the susceptibility to immune-mediated cholangiopathies is highlighted.…”

Section: Etiology and Mechanisms Of Hepatic Apoptosismentioning

confidence: 99%

Molecular mechanisms of hepatic apoptosis

2014

View full text Add to dashboard Cite

Apoptosis is a prominent feature of liver diseases. Causative factors such as alcohol, viruses, toxic bile acids, fatty acids, drugs, and immune response, can induce apoptotic cell death via membrane receptors and intracellular stress. Apoptotic signaling network, including membrane death receptor-mediated cascade, reactive oxygen species (ROS) generation, endoplasmic reticulum (ER) stress, lysosomal permeabilization, and mitochondrial dysfunction, is intermixed each other, but one mechanism may dominate at a particular stage. Mechanisms of hepatic apoptosis are complicated by multiple signaling pathways. The progression of liver disease is affected by the balance between apoptotic and antiapoptotic capabilities. Therapeutic options of liver injury are impacted by the clear understanding toward mechanisms of hepatic apoptosis.

show abstract

Primary sclerosing cholangitis (PSC): clinical, laboratory and survival analysis in children and adults

Cited by 36 publications

References 22 publications

Primary sclerosing cholangitis in children: A long-term follow-up study

Primary sclerosing cholangitis in children: A long-term follow-up study

Pathology of Pediatric Gastrointestinal and Liver Disease

Molecular mechanisms of hepatic apoptosis

Contact Info

Product

Resources

About