Hepatopulmonary syndrome as the first and only manifestation of cirrhosis in a patient with hypopituitarism

2021 ◽  
Vol 14 (9) ◽  
pp. e244805
Author(s):  
Eman Alabsawy ◽  
Yassen Serry ◽  
Sreelakshmi Kotha ◽  
Philip Berry ◽  
Giovanni Tritto
Keyword(s):  
Liver Disease ◽  
Fatty Liver Disease ◽  
Hepatopulmonary Syndrome ◽  
Progressive Dyspnoea ◽  
Alcoholic Fatty Liver ◽  
End Stage Liver Disease ◽  
Pituitary Dysfunction ◽  
Vascular Dilatation ◽  
End Stage ◽  
Alcoholic Fatty Liver Disease

Hepatopulmonary syndrome (HPS) is characterised by the development of intrapulmonary arteriovenous blood shunts and vascular dilatation with consequent hypoxaemia, usually in the context of end-stage liver disease (ESLD). The estimated incidence of HPS in ESLD has been reported to be 13%–47%. Chronic liver disease has been described in patients with hypothalamic–pituitary dysfunction, mainly in the form of non-alcoholic fatty liver disease due to metabolic syndrome, with occasional progression to cirrhosis. We report a challenging case of a 27-year-old man with a background of hypopituitarism with no known liver disease who presented with progressive dyspnoea and hypoxaemia and was eventually diagnosed with severe HPS.

Abstract 12917: Prognostic Value of the Combination of Model for End-stage Liver Disease Excluding Inr Score and Non-alcoholic Fatty Liver Disease Fibrosis Score in Patients Admitted for Acute Heart Failure

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Masatsugu Kawahira ◽  
Takahisa Yamada ◽  
Tetsuya Watanabe ◽  
Takashi Morita ◽  
Yoshio Furukawa ◽  
...  
Keyword(s):  
Heart Failure ◽  
Liver Disease ◽  
Liver Fibrosis ◽  
Ejection Fraction ◽  
Fatty Liver Disease ◽  
Fibrosis Score ◽  
Alcoholic Fatty Liver ◽  
End Stage Liver Disease ◽  
End Stage ◽  
Alcoholic Fatty Liver Disease

Background: Cardiohepatic interactions have been a focus of attention in heart failure(HF), and the model for end-stage liver disease excluding INR (MELD-XI) has been shown to be useful for prediction of poor outcome in patients (pts) with acute decompensated heart failure (ADHF). Furthermore, it has been reported that liver stiffness predicts adverse prognosis in pts with HF. Liver fibrosis is assessed by non-invasive fibrosis markers such as Fibrosis-4 (FIB4) index, non-alcoholic fatty liver disease fibrosis score (NFS) and aminotransferase to platelet ratio index (APRI). Recently, a new group of HF pts with mid-range ejection fraction (HFmrEF) has been defined, separated from reduced ejection fraction (HFrEF) and preserved ejection fraction (HFpEF). However, there is little information available on the comparison of prognostic significance of MELD-XI and liver fibrosis scores in ADHF pts, relating to LVEF. Methods: We prospectively studied 466 consecutive ADHF pts (HFrEF (LVEF≦40%): n=164, HFmrEF (40<LVEF<50): n=104 and HFpEF (LVEF≧50%): n=198). At the discharge, we calculated MELD-XI, FIB4 index , NFS, and APRI. The endpoint was all cause death (ACD). Results: During a follow-up period of 2.8±1.5 years, 143 pts had ACD. At multivariate Cox analysis, MELD-XI and FIB4 index was independently associated with ACD irrespective of LVEF. The pts with both greater MELD-XI ([HFrEF]≥25.8, [HFmrEF]≥33.2, [HFpEF]≥30.1) and greater FIB4 index ([HFrEF]≥2.58, [HFmrEF]≥2.58, [HFpEF]≥2.22) had a significantly increased risk of ACD than those with either and none of them in all three groups. Conclusions: The combination of MELD-XI and FIB4 index might be useful for stratifying pts at risk for ACD in ADHF pts with irrespective of LVEF.

Fatty liver disease in children

2011 ◽  
Author(s):  
R. Mark Beattie ◽  
Anil Dhawan ◽  
John W.L. Puntis
Keyword(s):  
Liver Disease ◽  
Alcohol Consumption ◽  
Liver Injury ◽  
Fatty Liver ◽  
Fatty Liver Disease ◽  
Alcoholic Hepatitis ◽  
Alcoholic Fatty Liver ◽  
End Stage Liver Disease ◽  
End Stage ◽  
Alcoholic Fatty Liver Disease

Demographics 406Pathophysiology 406Differential diagnoses 407Presenting features 407Investigation 408Management 409Fatty liver disease is now increasingly recognized in children, particularly in the setting of obesity.The term non-alcoholic steatohepatitis (NASH) was first coined in 1980 by Ludwig to describe a pattern of liver injury in adults in which the liver histology was consistent with alcoholic hepatitis, but in whom significant alcohol consumption was denied. NASH can be considered as part of a broader spectrum of non-alcoholic fatty liver disease that extends from simple steatosis through steatohepatitis that is characterized by the potential to progress to fibrosis, cirrhosis and subsequent end stage liver disease....

scholarly journals Mediterranean Diet and NAFLD: What We Know and Questions That Still Need to Be Answered

Nutrients ◽  
2019 ◽  
Vol 11 (12) ◽  
pp. 2971 ◽  
Author(s):  
Maria Corina Plaz Torres ◽  
Alessio Aghemo ◽  
Ana Lleo ◽  
Giorgia Bodini ◽  
Manuele Furnari ◽  
...  
Keyword(s):  
Liver Disease ◽  
Mediterranean Diet ◽  
Fatty Liver Disease ◽  
Alcoholic Fatty Liver ◽  
End Stage Liver Disease ◽  
Recommended Treatment ◽  
Clinical Scenarios ◽  
Proven Efficacy ◽  
End Stage ◽  
Alcoholic Fatty Liver Disease

Non-alcoholic fatty liver disease (NAFLD) is the hepatic manifestation of metabolic syndrome and is expected to become the leading cause of end-stage liver disease worldwide over the next few decades. In fact, NAFLD encompasses different clinical scenarios, from the simple accumulation of fat (steatosis) to steatohepatitis (NASH), NASH-cirrhosis, and cirrhosis complications. In this context, it is fundamental to pursue strategies aimed at both preventing the disease and reducing the progression of liver fibrosis once liver damage is already initiated. As of today, no pharmacological treatment has been approved for NAFLD/NASH, and the only recommended treatment of proven efficacy are life-style modifications, including diet and physical exercise pointing at weight loss of 5%–7%. Different dietetic approaches have been proposed in this setting, and in this review, we will discuss the evidence regarding the efficacy of the Mediterranean Diet as a treatment for NAFLD. In particular, we will report the effects on liver-related outcomes.

scholarly journals Recent advances in understanding and managing non-alcoholic fatty liver disease

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 720 ◽  
Author(s):  
Somaya Albhaisi ◽  
Arun Sanyal
Keyword(s):  
Liver Disease ◽  
Fatty Liver ◽  
Fatty Liver Disease ◽  
Hepatocellular Cancer ◽  
Radiological Imaging ◽  
Alcoholic Fatty Liver ◽  
End Stage Liver Disease ◽  
Recent Advances ◽  
End Stage ◽  
Alcoholic Fatty Liver Disease

Non-alcoholic fatty liver disease is a leading cause of chronic liver disease that can lead to cirrhosis, hepatocellular cancer, and end-stage liver disease, and it is linked to elevated cardiovascular- and cancer-related morbidity and mortality. Insulin resistance related to metabolic syndrome is the main pathogenic trigger that, in association with adverse genetic, lifestyle, and other factors, precipitates the development of non-alcoholic fatty liver disease. Biochemical markers and radiological imaging, along with liver biopsy in selected cases, help in the disease’s diagnosis and prognostication. Weight loss is the cornerstone treatment of non-alcoholic fatty liver disease; however, it is difficult to achieve and maintain, so pharmacotherapy was developed. The remarkable evolution in understanding disease pathogenesis has led to the development of new medical therapies and even the modification of currently available ones. This review summarizes recent advances in understanding the epidemiology, natural history, pathogenesis, diagnosis, and management of non-alcoholic fatty liver disease.

Paediatric non-alcoholic fatty liver disease: a practical overview for non-specialists

2015 ◽  
Vol 100 (7) ◽  
pp. 673-677 ◽  
Author(s):  
Jake P Mann ◽  
Rajiv Goonetilleke ◽  
Pat McKiernan
Keyword(s):  
Liver Disease ◽  
Fatty Liver ◽  
Fatty Liver Disease ◽  
Adult Care ◽  
Alcoholic Fatty Liver ◽  
Diet And Exercise ◽  
Paediatric Liver Disease ◽  
End Stage ◽  
Alcoholic Fatty Liver Disease

Non-alcoholic fatty liver disease (NAFLD) is the most common paediatric liver disease with a prevalence of almost 10%; therefore, the majority of affected patients are under the care of general practitioners and non-specialists. The condition is caused by central obesity with insulin resistance with additional factors influencing inflammatory activity (steatohepatitis). Ongoing inflammation leads to fibrosis and end-stage liver disease, though this will usually occur after children have transitioned into adult care. However, their main morbidity and mortality is from type 2 diabetes and complications of atherosclerosis. The minority of children undergo biopsy but currently there is no other method to accurately assess the stage of disease. Management is focused at weight loss through a combination of diet and exercise. Here, we present a current review of paediatric NAFLD aimed at non-specialists, with practice points for implementation.

scholarly journals Emerging Roles of Calcium Signaling in the Development of Non-Alcoholic Fatty Liver Disease

2021 ◽  
Vol 23 (1) ◽  
pp. 256
Author(s):  
Chien-Chih Chen ◽  
Li-Wen Hsu ◽  
Kuang-Den Chen ◽  
King-Wah Chiu ◽  
Chao-Long Chen ◽  
...  
Keyword(s):  
Liver Disease ◽  
Fatty Liver ◽  
Fatty Liver Disease ◽  
Critical Role ◽  
Liver Disorder ◽  
Alcoholic Fatty Liver ◽  
Stage Disease ◽  
Increased Risk ◽  
End Stage ◽  
Alcoholic Fatty Liver Disease

The liver plays a central role in energy metabolism. Dysregulated hepatic lipid metabolism is a major cause of non-alcoholic fatty liver disease (NAFLD), a chronic liver disorder closely linked to obesity and insulin resistance. NAFLD is rapidly emerging as a global health problem with currently no approved therapy. While early stages of NAFLD are often considered benign, the disease can progress to an advanced stage that involves chronic inflammation, with increased risk for developing end-stage disease including fibrosis and liver cancer. Hence, there is an urgent need to identify potential pharmacological targets. Ca2+ is an essential signaling molecule involved in a myriad of cellular processes. Intracellular Ca2+ is intricately compartmentalized, and the Ca2+ flow is tightly controlled by a network of Ca2+ transport and buffering proteins. Impaired Ca2+ signaling is strongly associated with endoplasmic reticulum stress, mitochondrial dysfunction and autophagic defects, all of which are etiological factors of NAFLD. In this review, we describe the recent advances that underscore the critical role of dysregulated Ca2+ homeostasis in lipid metabolic abnormalities and discuss the feasibility of targeting Ca2+ signaling as a potential therapeutic approach.

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