Abstract | BACKGROUND: AIM: METHODS: A single-center prospective unselected cohort study of 114 adult EPP patients, who underwent routine laboratory testing, abdominal ultrasonography and transient elastography to assess the presence of steatosis (controlled attenuation parameter,dB/m) and liver stiffness (kPa). RESULTS: 114 adult EPP patients were included. Elevated liver enzymes were found in 6.2% of the patients. Liver steatosis was detected in 29.0%, and significant fibrosis as assessed with liver stiffness measurements was present in 9.6% of patients. BMI positively predicted CAP-values (p = 0.026); and protoporphyrin IX levels (p = 0.043) positively predicted liver stiffness. CONCLUSIONS: This study demonstrates a prevalence of hepatic steatosis and fibrosis in adult EPP-patients comparable to that found in the general population. Protoporphyrin IX levels correlate with increased liver stiffness in EPP.
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Authors | Debby Wensink, Sandra Coenen, J H Paul Wilson, Margreet A E M Wagenmakers, Janneke G Langendonk |
Journal | Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver
(Dig Liver Dis)
Vol. 54
Issue 4
Pg. 515-520
(Apr 2022)
ISSN: 1878-3562 [Electronic] Netherlands |
PMID | 34475006
(Publication Type: Journal Article)
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Copyright | Copyright © 2021. Published by Elsevier Ltd. |
Topics |
- Adult
- Cohort Studies
- Humans
- Liver
(diagnostic imaging)
- Liver Diseases
- Prospective Studies
- Protoporphyria, Erythropoietic
(complications, epidemiology)
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