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Development and predictive validity of the cirrhosis-associated ascites symptom scale: A cohort study of 103 patients

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World Journal of Gastroenterology 2018年第15期24卷 1650-1657页

作者： Agnete Nordheim Riedel Nina Kimer Anne-Sofie Houlberg Jensen emilie Kristine dahl Mads Israelsen Luise Aamann Lise Lotte Gluud Gastro Unit Medical Davison Copenhagen University Hospital Hvidovre Abdominal Center K Medical Section Copenhagen University Hospital Bispebjerg Department of Gastroenterology and Hepatology University Hospital Zealand Slagelse Department of Gastroenterology and Hepatology Odense University Hospital Department of Hepatology and Gastroenterology Aarhus University Hospital

AIM To develop a scale of domains associated with the health-related quality-of-life(HRQOL) in patients with cirrhosis-related *** We initially undertook literature searches and a qualitative study in order to design a cirrhosis-associated ascites symptom(CAS) scale describing symptoms with a potential detrimental impact on health related quality of life(HRQL)(the higher the score, the worse the symptoms). Discriminatory validity was assessed in a validation cohort including cirrhotic patients with(1) tense/severe;(2) moderate/mild; or(3) no ascites(controls). Patients also completed chronic liver disease questionnaire(CLDQ) and the Euro QoL 5-Dimensions 5-Level(EQ-5D-5L) questionnaire evaluating HRQL. The relation between scale scores was analysed using Spearman correlations. RESULTS The final CAS scale included 14 items. The equivalent reliability was high(Chronbach's alpha 0.88). The validation cohort included 103 patients(72% men, mean age 62.4 years). The mean scores for each question in the CAS scale were higher for patients with severe/tense ascites than for mild/moderate ascites and controls. Compared with controls(mean = 9.9 points), the total CAS scale score was higher for severe/tense ascites(mean = 23.8 points) as well as moderate/mild ascites(mean = 18.6 points)(P < 0.001 both groups). We found a strong correlation between the total CAS and CLDQ score(rho = 0.82, P < 0.001) and a moderate correlation between the CAS and the EQ-5D-5L score(0.67, P < 0.001). CONCLUSION The CAS is a valid tool, which reflects HRQOL in patients with ascites.

关键词： Health-related quality-of-life Cirrhosis Symptom burden Symptom assessment

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Cholesteryl ester storage disease of clinical and genetic characterisation: A case report and review of literature

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World Journal of Clinical Cases 2020年第9期8卷 1642-1650页

作者： Elias Badal Rashu Anders Ellekær Junker Karen Vagner Danielsen emilie dahl Ole Hamberg Line Borgwardt Vibeke Brix Christensen Nicolai J Wewer Albrechtsen Lise L Gluud Gastrounit Copenhagen University Hospital HvidovreHvidovre 2650Denmark Department of Hepatology RigshospitaletCopenhagen UniversityCopenhagen 2100Denmark Centre of Genomic Medicine RigshospitaletCopenhagen UniversityCopenhagen 2100Denmark Department of Paediatrics and Adolescent Medicine RigshospitaletCopenhagen UniversityCopenhagen 2100Denmark Novo Nordisk Foundation Center for Protein Research Faculty of Health and Medical SciencesUniversity of CopenhagenCopenhagen 2200Denmark Department for Clinical Biochemistry RigshospitaletUniversity of CopenhagenCopenhagen 2200Denmark

BACKGROUND Cholesteryl ester storage disease(CESD)is a rare genetic *** symptoms and severity are highly *** is a systemic disease that can lead to the accumulation of fat and inflammation in the liver,as well as gastrointestinal and cardiovascular *** majority of patients require liver transplantation due to decompensated *** replacement therapy has been approved based on a randomized *** study aims to clinically and genetically evaluate two siblings with CESD who underwent liver transplantation,as well as their first-degree family *** SUMMARY The siblings were compound heterozygous for the missense variant in LIPA exon 8,c.894G>A,(***298Gln)and a single base pair deletion,c.482del(***161Ilefs*19).Analyses of single nucleotide polymorphisms showed variants with an increased risk of fatty liver disease and fibrosis for both ***,both patients show signs of recurrence of CESD in the liver after transplantation and additional gastrointestinal and cardiovascular signs of *** family members who were LIPA heterozygous had a lysosomal acid lipase activity below the reference *** of these carriers,a seven-year-old boy,was found to have severe dyslipidemia and was subsequently treated with *** Our study underlines that CESD is a multi-organ disease,the progression of which may occur post-liver *** findings underline the need for monitoring of complications and assessment of possible further treatment.

关键词： Lysosomal acid lipase deficiency Lysosomal storage disease Non-alcoholicfatty liver disease Liver transplantation Sebelipase alfa Case report

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