A Retrospective Single-Center Review of Primary Sclerosing Cholangitis in Children

Miloh, Tamir; Arnon, Ronen; Shneider, Benjamin L.; Suchy, Frederick J.; Kerkar, Nanda

doi:10.1016/j.cgh.2008.10.019

Cited by 140 publications

(198 citation statements)

References 29 publications

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“…6,7,12 The asymptomatic elevation of liver enzymes (transaminases and/or cholestasis) was the most common presentation, but AIH was easily overlooked. The IgG level was higher and circulating ANA/ASMA more common among PSC/AIH children, confirming the florid autoimmune pattern seen in this disease subgroup of patients.…”

Section: Presentation At the Time Of Diagnosismentioning

confidence: 99%

“…1 Pediatric-onset PSC, however, is rare, with a reported incidence of 0.2/100,000 per year for PSC and of 0.1/100,000 per year for PSC/AIH, 3 hampering the studies on clinical course and prognosis. So far, only five long-term follow-up studies focusing on the pediatric population have been published, 3,[5][6][7][8] and only one was conducted in Europe. 5 The aim of this study was to assess the long-term clinical course and prognosis of a cohort of patients with pediatric onset of PSC in Finland who underwent endoscopic retrograde cholangiography (ERC) at diagnosis and during follow-up, in order to contribute to a better definition of the natural history and surveillance of this condition.…”

Section: Introductionmentioning

confidence: 99%

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Clinical course and prognosis of pediatric-onset primary sclerosing cholangitis

Tenca

Färkkilä

Arola

et al. 2016

United European Gastroenterology Journal

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Background: The natural history of pediatric-onset primary sclerosing cholangitis (PSC) and overlap with autoimmune hepatitis (PSC/AIH) is poorly known. Objective: The aim of this study was to evaluate the clinical outcome of patients with pediatric-onset disease in a tertiary referral center. Methods: We traced 33 patients (median age at diagnosis 16 years), with PSC or PSC/AIH in cholangiography and liver histology diagnosed between December 1993 and 2011, at Helsinki University Hospital. Diagnostic procedures and long-term follow-up were reassessed until the end of December 2013. Results: PSC was confirmed in all 33 patients; 19 of them had an overlap with AIH. At diagnosis, three of 33 had cirrhosis. Inflammatory bowel disease (IBD) was associated in 76% of the patients, mostly ulcerative colitis (70%); treatment of IBD being a minor determinant of the clinical outcome of liver disease. In the last follow-up (median nine years), all patients were alive, and no malignancy occurred. Most patients (91%) were on ursodeoxycholic acid and 12 PSC/AIH patients on immunosuppression. Endoscopic retrograde cholangiography during follow-up showed a progression of intra-hepatic disease in 12 patients (36%). Four patients (12%) had undergone liver transplantation, and one was listed; no recurrence of the disease in the graft was seen. Conclusion:The clinical course and outcome of pediatric-onset PSC and PSC/AIH seem to be favourable in the majority of patients until early adulthood. In about one-third of patients, however, PSC is progressive, challenging the current treatment guidelines and warranting further studies on disease pathogenesis.

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Section: Presentation At the Time Of Diagnosismentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Clinical course and prognosis of pediatric-onset primary sclerosing cholangitis

Tenca

Färkkilä

Arola

et al. 2016

United European Gastroenterology Journal

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“…9 In the present patient, the histopathology demonstrated an obstructive cholangiopathy, but there was no significant liver cirrhosis. Also, there was no laboratory evidence of autoimmune hepatitis.…”

Section: Discussionmentioning

confidence: 83%

Choledochal Cyst Originating from Primary Sclerosing Cholangitis in a Child

Tseng

Lin

et al. 2011

International Surgery

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Choledochal cysts are common in Asian children. Primary sclerosing cholangitis (PSC), which is characterized by inflammation and fibrosis and may lead to bile duct stricture over the intrahepatic or extrahepatic bile duct, is rare in children. Here we report a case of a 10-year-old boy who presented with a choledochal cyst originating from PSC. He had suffered from repeated abdominal pain and cholangitis for 3 years. A type IV choledochal cyst was suspected from the ultrasound and computed tomography image showing a distended gallbladder and dilatation of the bilateral intrahepatic duct at the hepatic hilar area and common bile duct (CBD). During laparotomy, a markedly distended gallbladder was noted and was shown to have no communication with the CBD by intraoperative cholangiogram. Choledochal cysts with extrahepatic and intrahepatic duct dilatation at the hilar area and marked stenosis with nearly total obstruction of the distal CBD were noted. Hepaticojejunostomy was performed. The histopathologic findings demonstrated a typical PSC picture. The patient's postoperative course was uneventful for 8 months after surgery, and he received no medication during a regular follow-up.

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“…PSC is strictly associated with an inflammatory bowel disease (IBD) reaching more than 80% of cases, while, only 2 -7.5% of children or adolescents affected by IBD develop sclerosing cholangitis (Miloh 2009). This form of IBD predominantly has the features of an ulcerative colitis and in a minority of a Crohn's colitis.…”

Section: Clinical Featuresmentioning

confidence: 99%