autoimmune sclerosing cholangitis progressive familial intrahepatic cholestasis K83.0 K76.8
Conditions
Interventions
Group 1: We conducted a prospective study for which we collected serum samples of 52 children aged 1-18 years between August 2018 and March 2020. We included 29 children with CCLD (with pruritus n=13,
Sponsors
Medizinische Universität Graz
Eligibility
Sex/Gender
All
Age
0 Years to 19 Years
Inclusion criteria
Inclusion criteria: -Male or female subjects between the ages of 0 and 19 years -Diagnosis of CCLD (ASC, PFIC, chronic cholestasis of unknown origin with/without pruritus) -Available information concerning date of birth, sex, date of blood collection
Exclusion criteria
Exclusion criteria: -Interruption of enterohepatic circulation -Chronic diarrhea requiring specific intravenous fluid/ nutritional intervention -Liver transplant -Decompensated cirrhosis (INR = 1.5 (not due to vitamin K deficiency), albumin < 3.0 mg/dl, ascites, hepatic encephalopathy) -Known diagnosis of immunodeficiency -Any pregnant or lactating female -Diabetes mellitus -Chemotherapy
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Determination of total bile acid-/muricholic acid levels and profiles and autotaxin antigen levels in serum of pediatric patients with chronic cholestatic liver diseases. | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. Determination of pruritus via PVAS (Pruritus Visual Analogue Scale). 2. Determination of total bile acid-/muricholic acid levels and profiles by highperformance-liquidchromatography/tandem-massspectrometry. 3. Determination of autotaxin-antigen levels via ELISA Immunoassay. | — |
Countries
Austria, Hungary
Contacts
Public ContactKatharina Meinel
Medizinische Universität GrazKlinik für Kinder- und Jugendheilkunde
Outcome results
None listed