Journal of Clinical and Translational Hepatology

Journal of Clinical and Translational Hepatology

Saturday, 10 / 16 / 2021

Acute Hepatic Phenotype of Wilson Disease: Clinical Features of Acute Episodes and Chronic Lesions Remaining in Survivors

Hisao Hayashi1, Yasuaki Tatsumi1, Shinsuke Yahata2, Hiroki Hayashi3, Kenji Momose4, Ryohei Isaji5, Youji Sasaki5, Kazuhiko Hayashi6, Shinya Wakusawa*7 and Hidemi Goto6

1Department of Medicine, Aichi-Gakuin University School of Pharmacy, Nagoya, Japan; 2Department of Gastroenterology, Hyogo Prefectural Kakogawa Medical Center, Kakogawa, Japan; 3Department of Gastroenterology, Kita-Harima Medical Center, Ono, Japan; 4Department of Gastroenterology, Kobe University School of Medicine, Kobe, Japan; 5Department of Gastroenterology, Konan Kosei Hospital, Konan, Japan; 6Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya, Japan; 7Department of Medical Laboratory Sciences, Nagoya University Graduate School of Medicine, Nagoya, Japan

Abstract

Background and Aims: Wilson disease (WD) is an inherited disorder of copper metabolism, and an international group for the study of WD (IGSW) has proposed three phenotypes for its initial presentation: acute hepatic, chronic hepatic, and neurologic phenotypes. Characterization of the acute hepatic phenotype may improve our understanding of the disease. Methods: Clinical features of 10 WD patients with the acute hepatic phenotype and characteristics of chronic lesions remaining in survivors were assessed by the European Association for the Study of the Liver (EASL) guidelines. Results: All six patients younger than 30 years had survived an acute episode of hemolytic anemia with residual liver disease of cirrhosis or chronic hepatitis. The acute episode was self-limiting in two of the four patients over the age of 30 years and progressed to acute liver failure in the other two patients. One of the two survivors had residual liver disease of chronic hepatitis, while the other had chronic hepatitis and neurologic disease. Neurologic disease remained in a patient who successfully received a liver transplantation. During acute episodes, serum levels of aspartate aminotransferase (AST) and alanine aminotransferase (ALT) changed rapidly along with anemia. Liver-specific ALT levels were age-dependently correlated with hemoglobin (Hb) concentrations. Enzyme reduction was milder for AST than ALT, which resulted in a high AST/ALT ratio in the anemic stage. The anemic stage in two patients transformed to acute liver failure. Conclusions: All survivors of an acute episode of the acute hepatic phenotype had residual liver disease or both liver and neurologic diseases. The rapid changes in liver enzymes during the acute episode and the liver and neurologic diseases remaining in survivors may provide a better understanding of WD.

Doi: 10.14218/JCTH.2015.00032
Journal of Clinical and Translational Hepatology 2015 vol. 3, 239-245  [
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Identification of Nonalcoholic Fatty Liver Disease following Pancreatic Surgery in a Western Cohort Using a Novel Radiographic Technique

Sidney Olefson1, Melissa Jackson1, David J. Grand2, Kevin P. Charpentier3, Nirav Makwana2 and Kittichai Promrat*4

1Department of Internal Medicine, Warren Alpert Medical School of Brown University, Providence, RI, USA; 2Department of Diagnostic Imaging, Warren Alpert Medical School of Brown University, Providence, RI, USA; 3Department of Surgery, Warren Alpert Medical School of Brown University, Providence, RI, USA; 4Division of Gastroenterology and Hepatology, Warren Alpert Medical School of Brown University, Providence, RI, USA

Abstract

Background and Aims: While traditional risk factors for the development of nonalcoholic fatty liver disease (NAFLD) relate to metabolic syndrome, several Asian studies have suggested a high rate of de novo NAFLD following pancreaticoduodenectomy (PD). The aim of this study is to identify de novo NAFLD after pancreatic surgery and its associated risk factors. Methods: A retrospective cohort of patients at a single center that underwent PD or distal pancreatectomy (DP) over 7 years was identified. Pre- and postoperative contrast-enhanced computed tomography scans of the abdomen were reviewed, including attenuation measurements of the liver, spleen, and muscle. Primary outcomes included hepatic attenuation, liver to muscle ratio (LMR), and liver to spleen ratio (LSR). Results: Of the 96 patients (mean age 64.3) included, 70% underwent PD, and 30% underwent DP. The mean LMR decreased significantly from 1.81 to 1.66 (p=0.02), noted only in men. No interaction effect with LMR was observed with surgical type, chemotherapy, blood loss, pancreatic enzyme replacement, or transaminases. LMR decreased in 55% of subjects. Conclusions: Increased fatty infiltration, as evidence by decreased LMR, was found among men that underwent PD and DP within a year of surgery. This may be related to weight loss and malabsorption and deserves further investigation.

Doi: 10.14218/JCTH.2015.00029
Journal of Clinical and Translational Hepatology 2015 vol. 3, 246-253  [
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Patients with Gastric Antral Vascular Ectasia (GAVE) Are at a Higher Risk of Gastrointestinal Bleeding in the Absenceof Cirrhosis

Jennifer Wang1, Jonathan G.Stine*2, ScottL.Cornella1, Curtis K. Argoand Steven M.Cohn2

1Department of Medicine, University of Virginia, Charlottesville, Virginia, USA; 2Division of Gastroenterology and Hepatology, University of Virginia, Charlottesville, Virginia, USA

Abstract

Background and Aims: Gastric antral vascular ectasia (GAVE) is commonly found in patients with cirrhosis, but it is also associated with other diseases in the absence of cirrhosis. Whether GAVE confers a different severity of gastrointestinal (GI) bleeding between patients with and without cirrhosis remains unknown. We aim to examine whether there is a difference in clinically significant GI bleeding due to GAVE in patients with or without cirrhosis. Methods: This is a retrospective case-control study of patients who were diagnosed with GAVE between January 2000 and June 2014. Patients were categorized into cirrhosis and noncirrhosis groups, and those with an additional GI bleeding source were excluded. Univariate comparisons and multivariable models were constructed using logistic regression. Results: In total, 110 patients diagnosed with GAVE on esophagogastroduodenoscopy (EGD) were included in our analysis; 84 patients had cirrhosis (76.4%) and 26 (23.6%) did not. Active GI bleeding was more prevalent in patients without cirrhosis (63.4% vs. 32.1%, p=0.003) despite similar indications  for  EGD, and endoscopic treatment with argon plasma coagulation (APC) was required more often in this group, approaching statistical significance (27% vs. 10.7%, p=0.056). There was no difference in bleeding severity, as evidenced by similar re-bleeding rates, surgery, or death attributed to uncontrolled bleeding. The strongest independent risk factor for GI bleeding was the absence of cirrhosis (odds ratio (OR): 5.151 (95% confidence interval (CI): 1.08-24.48, p=0.039). Conclusions: Patients with GAVE in the absence of cirrhosis are at higher risk for active GI bleeding and require more frequent  endoscopic  treatment  than  similar  patients  with cirrhosis. It may be worthwhile to treat GAVE in this population even in the absence of active bleeding.

Doi: 10.14218/JCTH.2015.00031
Journal of Clinical and Translational Hepatology 2015 vol. 3, 254-259  [
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