scholarly journals Cystic fibrosis-related liver disease: a single-center experience

2011 ◽  
Vol 3 (3) ◽  
pp. 21 ◽  
Author(s):  
Paula Catarino Costa ◽  
Celeste Canha Barreto ◽  
Luisa Pereira ◽  
Maria Luisa Lobo ◽  
Maria Adília Costa ◽  
...  

Prospective studies concerning liver disease in pediatric cystic fibrosis patients are scarce. The present study aimed to describe the prevalence and clinical expression of cystic fibrosis - related liver disease, in a cohort of 62 pediatric patients. Descriptive study, resulting from the prospective evaluation, between 1994 and 2009, of 62 pediatric patients (age <18 years) with cystic fibrosis. The follow-up protocol included a clinical assessment every 2 months, liver function tests every 6 months and annual liver ultrasonography. The cumulative prevalence of liver disease was 11.2% (7/62 cases). All patients had ΔF508 mutation and pancreatic insufficiency, none had meconium ileus. The liver involvement became clinically evident at a mean age of 8 years (3-15 years), revealed by hepatomegaly or hepatosplenomegaly (3 cases) and/ or abnormalities of liver function tests (3 cases) changes of liver ultrasound (7 cases) with evidence of portal hypertension (2 cases). Four patients were submitted to liver biopsy; biliary fibrosis was documented in one case, focal biliary cirrhosis in 2 cases and multilobular cirrhosis in another case. Within a median 11.6 years follow-up period (all patients under UDCA therapy after liver disease diagnosis), progression of liver disease was observed in 2 patients; one patient developed refractory variceal bleeding and progressive hepatic failure, requiring liver transplant. The results of the present study agree with those of previous pediatric studies, further documenting clinical expression of liver disease in CF patients, which is usually detected in the first decade of life and emphasize the contribution of ultrasound to early diagnosis of liver involvement. Moreover, although advanced liver disease is a relatively rare event, early isolated liver transplantation may have to be considered at this age group.

2019 ◽  
Vol 12 (9) ◽  
pp. 507-515
Author(s):  
Ceen-Ming Tang

Liver disease is a clinical priority area identified by the RCGP, due to increasing levels of morbidity and mortality. Liver disease causes an estimated 8500 premature deaths in the UK annually, predominantly from a rise in non-alcoholic fatty liver disease, alcohol-related liver disease, and viral hepatitis. There has been a corresponding increase in liver function tests (LFTs) ordered from primary care, often as part of medication reviews or monitoring of chronic disease. GPs will therefore encounter an increasing number of abnormal LFTs in patients asymptomatic of liver disease. This article outlines a framework for investigating abnormal LFTs in primary care.


2021 ◽  
Vol 10 (8) ◽  
pp. 1730
Author(s):  
Hiroshi Miyama ◽  
Yasuyuki Shiraishi ◽  
Shun Kohsaka ◽  
Ayumi Goda ◽  
Yosuke Nishihata ◽  
...  

Abnormal liver function tests (LFTs) are known to be associated with impaired clinical outcomes in heart failure (HF) patients. However, this implication varies with each single LFT panel. We aim to evaluate the long-term outcomes of acute HF (AHF) patients by assessing multiple LFT panels in combination. From a prospective multicenter registry in Japan, 1158 AHF patients who were successfully discharged were analyzed (mean age, 73.9 ± 13.5 years; men, 58%). LFTs (i.e., total bilirubin, aspartate aminotransferase or alanine aminotransferase, and alkaline phosphatase) at discharge were assessed; borderline and abnormal LFTs were defined as 1 and ≥2 parameter values above the normal range, respectively. The primary endpoint was composite of all-cause death or HF readmission. At the time of discharge, 28.7% and 8.6% of patients showed borderline and abnormal LFTs, respectively. There were 196 (16.9%) deaths and 298 (25.7%) HF readmissions during a median 12.4-month follow-up period. The abnormal LFTs group had a significantly higher risk of experiencing the composite outcome (adjusted hazard ratio: 1.51, 95% confidence interval: 1.08–2.12, p = 0.017), whereas the borderline LFTs group was not associated with higher risk of adverse events when referenced to the normal LFTs group. Among AHF patients, the combined elevation of ≥2 LFT panels at discharge was associated with long-term adverse outcomes.


2017 ◽  
Vol 27 (4) ◽  
pp. 1060-1060
Author(s):  
Geraldine J. Ooi ◽  
Paul R. Burton ◽  
William W. Kemp ◽  
Stuart K. Roberts ◽  
Wendy A. Brown

Author(s):  
Giuseppe Chiarioni ◽  
Stefan Lucian Popa ◽  
Andrea Dalbeni ◽  
Carlo Senore ◽  
Daniel Corneliu Leucuta ◽  
...  

Background and Aims: The Western diet is rich in saturated fats, refined sugars and meat consistent with a high-energy load and secondary risk of increased metabolic diseases including nonalcoholic fatty liver disease (NAFLD). However, no data are available on potential benefit of vegan diets in NAFLD and/or nonalcoholic steatohepatitis (NASH). We aimed to study prospectively the effect of a vegan diet, excluding all animal products on liver chemistry in a group of consecutive NAFLD patients. Methods: This was a prospective, pilot study run on 40 consecutive patients affected by NAFLD. Eight subjects refused to join the study for poor diet palatability, leaving 32 patients (19 males, mean age 50 years), with abnormal measures of liver function who agreed to adhere to a vegan diet for six months. The caloric intake was tailored by the dietitian to obtain a weight loss ≥5% of body weight in overweight patients [body-mass index (BMI) ≥25] and ranged from 1500 Kcal to 1800 Kcal. Patients were contacted monthly by phone to reinforce diet and lifestyle advice and were seen at the gastrointestinal clinic when doubtful about diet advice. Results: At six-month follow-up, 6 subjects did not attend the clinic leaving only 26 patients for data analysis. Initial anthropometric values were mean weight 78 kg (range 52-95), mean body mass index (BMI) 26.8 Kg/m2 (range 20.3-31.2). Liver function tests showed mean ALT value 99 U/L (SD±45), mean AST value 54 U/L (SD±44), mean GGT value 160 U/L (SD±122), pre-treatment. After six months mean body weight was 73 Kg (range 52-87), mean BMI was 25.2 Kg/m2 (range 20.3-29.7) (p<0.001 compared to baseline for both parameters). Liver enzymes improved to a mean of ALT value 36 U/L (SD±21), AST value 27 U/L (SD±10) and GGT value 55 U/L (SD±57), respectively (p<0.001 compared to baseline for all enzymes). Normalization of liver function tests as a whole was observed in 20/26 patients (76.9%). A loss of ≥ 5% of body weight was observed in 12 patients (46.1%), but it did not correlate with the normalization of liver function tests (p=0.5). Conclusions: Our data provide preliminary evidence of improved liver enzymes in NAFLD patients with a strict vegan diet and although our study sample is limited, decreased body weight did not seem critical to the outcome.


BMJ Open ◽  
2020 ◽  
Vol 10 (3) ◽  
pp. e032890 ◽  
Author(s):  
Geraldine O'Hara ◽  
Jolynne Mokaya ◽  
Jeffrey P Hau ◽  
Louise O Downs ◽  
Anna L McNaughton ◽  
...  

ObjectivesLiver disease is a major cause of morbidity and mortality in sub-Saharan Africa, but its prevalence, distribution and aetiology have not been well characterised. We therefore set out to examine liver function tests (LFTs) and liver fibrosis scores in a rural African population.DesignWe undertook a cross-sectional survey of LFTs. We classified abnormal LFTs based on reference ranges set in America and in Africa. We derived fibrosis scores (aspartate aminotransferase (AST) to Platelet Ratio Index (APRI), fibrosis-4, gamma-glutamyl transferase (GGT) to platelet ratio (GPR), red cell distribution width to platelet ratio and S-index). We collected information about alcohol intake, and infection with HIV, hepatitis B virus (HBV) and hepatitis C virus (HCV).SettingWe studied a population cohort in South-Western Uganda.ParticipantsData were available for 8099 adults (median age 30 years; 56% female).ResultsThe prevalence of HBV, HCV and HIV infection was 3%, 0.2% and 8%, respectively. The prevalence of abnormal LFTs was higher based on the American reference range compared with the African reference range (eg, for AST 13% vs 3%, respectively). Elevated AST/ALT ratio was significantly associated with self-reported alcohol consumption (p<0.001), and the overall prevalence of AST/ALT ratio >2 was 11% (suggesting alcoholic hepatitis). The highest prevalence of fibrosis was predicted by the GPR score, with 24% of the population falling above the threshold for fibrosis. There was an association between the presence of HIV or HBV and raised GPR (p=0.005) and S-index (p<0.001). By multivariate analysis, elevated LFTs and fibrosis scores were most consistently associated with older age, male sex, being under-weight, HIV or HBV infection and alcohol consumption.ConclusionsFurther work is required to determine normal reference ranges for LFTs in this setting, to evaluate the specificity and sensitivity of fibrosis scores and to determine the aetiology of liver disease.


Author(s):  
Sharon Dempsey ◽  
John Travers ◽  
Gerardine Leer ◽  
Geraldine Connell ◽  
Shona Quinn ◽  
...  

2013 ◽  
Vol 12 (6) ◽  
pp. 826-827 ◽  
Author(s):  
Thomas Karlas ◽  
Marie Neuschulz ◽  
Annett Oltmanns ◽  
Hubert Wirtz ◽  
Volker Keim ◽  
...  

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