The Riddle of Juvenile Sclerosing Cholangitis

Mieli‐Vergani, Giorgina; Vergani, Diego

doi:10.1002/hep.29208

Cited by 3 publications

(2 citation statements)

References 14 publications

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“…This clinical condition was initially reported only in adult patients. Nowadays, SC is a well-recognised hepatic disorder described in all age groups, including children, in whom some features are unique [3] .…”

Section: Introductionmentioning

confidence: 99%

Cutting edge issues in juvenile sclerosing cholangitis

Giorgio

Vergani

Mieli‐Vergani

2022

Digestive and Liver Disease

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Section: Introductionmentioning

confidence: 99%

Cutting edge issues in juvenile sclerosing cholangitis

Giorgio

Vergani

Mieli‐Vergani

2022

Digestive and Liver Disease

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“…12,13 The hypotheses explaining such differences include the following: (a) adult and pediatric PSC have distinct phenotypes, or (b) adult PSC presents at a more advanced stage compared to pediatric-onset disease. 14,15 There is a paucity of studies directly comparing adult and pediatric PSC subjects. 16 Such a comparison could potentially help elucidate differences in the natural history of adult versus pediatric-onset PSC.…”

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confidence: 99%

Diagnosis of Primary Sclerosing Cholangitis Beyond Childhood is Associated with Worse Outcomes

Tica

Alghamdi

Tait

et al. 2022

Journal of Clinical and Experimental Hepatology

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Background: The elucidation of differences between adult and pediatric-onset primary sclerosing cholangitis (PSC) may inform clinical decision making, and whether results of adult PSC clinical trials can be extrapolated to pediatric subjects. Methods: A single-center retrospective analysis of PSC subjects diagnosed during the epoch 2000-13 was conducted. Demographic, clinical, and laboratory data were compared between PSC subjects diagnosed between 0-18 (pediatric) and 19+ (adult) years of age. An adverse outcome was defined as PSC-related death, liver transplant, or malignancy. Survival without any of these was defined as event-free survival. Results: Analyses of 28 pediatric-diagnosed and 59 adult-diagnosed subjects revealed that incidence of early portal hypertension (PHT; P = 0.2), laboratory parameters of liver disease severity, and fibrosis grade at diagnosis were comparable between adult and pediatric PSC subjects. Adult-diagnosed PSC subjects had higher incidences of adverse outcomes compared to pediatric-diagnosed PSC subjects (P = 0.02). The age group 0-18 years (n = 30) had significantly better event-free survival compared to the age group more than 40 years (n = 25; P = 0.03). The prevalence of PHT in adult PSC subjects was 2.6 that of pediatric PSC subjects. PHT adversely affected outcomes in both adult (P < 0.001) and pediatric (P = 0.01) subjects. Adult PSC subjects were more likely to develop biliary complications (BCs; P = 0.001), ascites (P = 0.004), and variceal bleed (P = 0.03). Adult PSC subjects were more likely to have extra-hepatic co-morbidities (P < 0.001). Adult subjects had a longer follow-up duration compared to pediatric subjects (P = 0.06). Conclusion: Despite having a comparable clinical, laboratory, and histologic biomarkers of liver disease severity at the time of diagnosis, adult PSC subjects had a worse outcome compared to pediatric PSC subjects. Possible reasons for this finding include higher incidence of PHT, BCs, extra-hepatic co-morbidities, and longer duration of follow-up.

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