Magnetic resonance cholangiopancreatography in primary sclerosing cholangitis in children

Ferrara, Calogero; Valeri, Gianluca; Salvolini, Luca; Giovagnoni, Andrea

doi:10.1007/s00247-001-0617-z

Cited by 69 publications

(25 citation statements)

References 9 publications

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“…In a group of 33 children, Suzuki et al [23] recently reported that MRCP had 100% diagnostic accuracy for detecting choledochal cysts. Others have reported similar diagnostic accuracy for PSC [21] and biliary atresia [17].…”

Section: Discussionmentioning

confidence: 71%

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The use of magnetic resonance cholangiopancreatography in children

Tipnis¹,

Werlin

2007

Curr Gastroenterol Rep

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Magnetic resonance cholangiopancreatography (MRCP) is a noninvasive method to evaluate a wide variety of pancreatobiliary disorders. These disorders include choledochal cyst, cholelithiasis, choledocholithiasis, biliary atresia, Caroli's disease, primary sclerosing cholangitis, disorders of the pancreatobiliary junction, pancreas divisum, and pancreatic duct abnormalities related to chronic pancreatitis. The use of MRCP in children is increasing as experience with MRCP grows, and its technological accuracy rivals that of endoscopic evaluation. We review the current state of MRCP use in children.

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Section: Discussionmentioning

confidence: 71%

“…Ferrara et al [21] prospectively evaluated 21 children (ages 7-14 years) with clinical suspicion of PSC. They compared MRCP with ERCP and liver biopsy in all cases.…”

Section: Review Of Studies Comparing Mrcp To Gold Standard Evaluationmentioning

confidence: 99%

The use of magnetic resonance cholangiopancreatography in children

Tipnis¹,

Werlin

2007

Curr Gastroenterol Rep

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“…20 Hesitation to perform invasive cholangiography in children presenting with features of AIH might have contributed to some extent to underdiagnosing PSC in the pediatric population; in this regard, a noninvasive procedure for visualization of bile ducts such as magnetic resonance cholangiography may be a useful alternative. 23 Immunosuppressive therapy has a significant beneficial effect in children and adults with AIH alone. Inconsistent results, however, have been reported in children with AIH/PSC overlap.…”

Section: Discussionmentioning

confidence: 99%

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

Feldstein¹

2003

Hepatology

226

214

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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.)

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“…11,13,[18][19][20][21][22] An autoimmune hepatitis-like presentation is common. 11,22 ERCP may demonstrate bile duct abnormalities in these children, 23 but their course is typical of autoimmune hepatitis, although with time, features of PSC become prominent. A rare mode of presentation is with advanced disease as shown by marked splenomegaly or gastrointestinal hemorrhage.…”

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confidence: 99%

Primary sclerosing cholangitis: Summary of a workshop

et al. 2006

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Primary sclerosing cholangitis (PSC) is a rare but important liver disease that leads to cirrhosis and need for liver transplantation in a high proportion of cases. The disease occurs in approximately 1 per 100,000 population per year, usually presents in adulthood, and affects men more often than women. Typical serum biochemical results, autoantibodies and liver biopsy are suggestive but not diagnostic of PSC, the diagnosis requiring cholangiographic demonstration of stricturing and dilatation of the intra-and/or extra-hepatic bile ducts. The natural history of PSC is variable, the average survival being 12 to 17 years. The cause of PSC is still unknown. Although considered an autoimmune disease, PSC has several atypical features and a strong genetic component. The therapy of PSC is unsatisfactory. Standard doses of ursodeoxycholic acid (UDCA) lead to improvements in biochemical abnormalities but not in histology, cholangiographic appearance or survival. Several innovative therapies have been tried in PSC, but with scant evidence of benefit. For patients with high grade strictures, endoscopic dilatation is beneficial. Liver transplantation is successful for end-stage liver disease due to PSC and improves survival. PSC may recur after transplantation but is rarely progressive. The most dreaded complication of PSC is cholangiocarcinoma. Diagnosis of this highly malignant tumor is difficult, and there are no biomarkers for its early detection. Liver transplantation for cholangiocarcinoma has an exceedingly poor outcome, although transplantation with neoadjuvant chemoirradiation holds promise in selected patients. Thus, significant opportunities remain for basic and clinical research into the cause, natural history, and therapy of PSC. 44:746-764.)

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Magnetic resonance cholangiopancreatography in primary sclerosing cholangitis in children

Abstract: MRCP can be proposed as the preliminary non-invasive imaging modality for the diagnosis of PSC in children.

Cited by 69 publications

References 9 publications

The use of magnetic resonance cholangiopancreatography in children

The use of magnetic resonance cholangiopancreatography in children

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

Primary sclerosing cholangitis: Summary of a workshop

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