An ultrasound scoring system for the diagnosis of liver disease in cystic fibrosis
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The natural history of cystic fibrosis liver disease a prospective cohort study
2023, Journal of Cystic FibrosisComparison of Transient Elastography, ShearWave Elastography, Magnetic Resonance Elastography and FibroTest as routine diagnostic markers for assessing liver fibrosis in children with Cystic Fibrosis
2022, Clinics and Research in Hepatology and GastroenterologyEncephalopathy in cystic fibrosis
2020, Paediatric Respiratory ReviewsCitation Excerpt :More recent publications have proposed the definition as at least 2 of the following findings on at least 2 consecutive interactions over a 6–12 month period: (1) hepatomegaly, with liver edge >2 cm below the costal margin in the midclavicular line and confirmed by ultrasound. ( 2) At least 2 out of 3 of aspartate transaminase (AST), alanine aminotransferase (ALT) and gamma-glutamyl transferase (GGT) above the upper limit of normal and (3) Williams score* of ultrasound abnormalities ≥4 [3,6]. Briefly, the Williams score is a scoring system for the diagnosis of liver disease in patients with cystic fibrosis and is based on three characteristics: (1) gross nodularity of the parenchyma, (2) nodularity of the liver edge and (3) increased periportal echogenicity.
The clinical benefits of long-term supplementation with omega-3 fatty acids in cystic fibrosis patients - A pilot study
2016, Prostaglandins Leukotrienes and Essential Fatty AcidsCitation Excerpt :All patients included into the study were clinically stable, homozygous for the ΔF508 mutation, over 5 years of age, already undergoing azithromycin treatment for at least 3 months, able to perform lung function tests and swallow capsules, and invited to participate between October 2008 and April 2010. The exclusion criteria were: (1) upper or lower respiratory infection within 2 weeks before baseline evaluation or abnormalities on chest X-ray or CT scan; (2) undergoing any chronic (>1 week daily) oral or intravenous anti-inflammatory treatment other than azithromycin within 3 months before study initiation; (3) active bleeding or increased risk of bleeding; (4) coagulation alterations and/or platelets < 50.000/mm3; (5) diabetes; (6) FEV1<40%; (7) significant liver disease, defined as elevated liver function values 2-fold higher than the upper normal range or abnormal ultrasound (Williams score >5 [28]); (8) hypercholesterolemia (>240 mg%); (9) participating in another study; (10) pregnancy. Sixteen patients agreed to participate but one patient was excluded on account of incident diabetes revealed at the baseline visit.
Longitudinal Transient Elastography Measurements Used in Follow-up for Patients with Cystic Fibrosis
2016, Ultrasound in Medicine and BiologyCitation Excerpt :Other tests performed were a physical examination as well as a blood sample for liver enzymes. The abdominal ultrasound was scored according to Williams et al. (1995), which is the sum of a 1–3-point scoring system for coarseness of the parenchyma, nodularity of the liver edge and increased peri-portal echogenicity. A normal ultrasound score is 3, whereas severe cirrhosis receives a score of 9.