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Pearls and Pitfalls of Metabolic Liver Magnetic Resonance Imaging in the Pediatric Population.
Mojtahed, A, Gee, MS, Yokoo, T
Seminars in ultrasound, CT, and MR. 2020;(5):451-461
Abstract
Recent advances in magnetic resonance imaging (MRI) technology have moved imaging beyond anatomical assessment to characterization of tissue composition. There are now clinically validated MRI-based quantitative techniques for assessing liver fat, iron, and fibrosis, and MRI is now routinely used in metabolic liver disease evaluation in both pediatric and adult patients. These MRI techniques provide noninvasive quantitation of liver metabolic biomarkers that are increasingly relied upon in the clinical management of pediatric patients with nonalcoholic fatty liver disease, metabolic syndrome, and hemochromatosis and/or hemosiderosis. This article provides a review of the clinical indications and technical parameters for performing metabolic liver MRI in the pediatric population, along with common pearls and pitfalls encountered during its performance.
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2.
Rubella Virus Infection, the Congenital Rubella Syndrome, and the Link to Autism.
Mawson, AR, Croft, AM
International journal of environmental research and public health. 2019;(19)
Abstract
Rubella is a systemic virus infection that is usually mild. It can, however, cause severe birth defects known as the congenital rubella syndrome (CRS) when infection occurs early in pregnancy. As many as 8%-13% of children with CRS developed autism during the rubella epidemic of the 1960s compared to the background rate of about 1 new case per 5000 children. Rubella infection and CRS are now rare in the U.S. and in Europe due to widespread vaccination. However, autism rates have risen dramatically in recent decades to about 3% of children today, with many cases appearing after a period of normal development ('regressive autism'). Evidence is reviewed here suggesting that the signs and symptoms of rubella may be due to alterations in the hepatic metabolism of vitamin A (retinoids), precipitated by the acute phase of the infection. The infection causes mild liver dysfunction and the spillage of stored vitamin A compounds into the circulation, resulting in an endogenous form of hypervitaminosis A. Given that vitamin A is a known teratogen, it is suggested that rubella infection occurring in the early weeks of pregnancy causes CRS through maternal liver dysfunction and exposure of the developing fetus to excessive vitamin A. On this view, the multiple manifestations of CRS and associated autism represent endogenous forms of hypervitaminosis A. It is further proposed that regressive autism results primarily from post-natal influences of a liver-damaging nature and exposure to excess vitamin A, inducing CRS-like features as a function of vitamin A toxicity, but without the associated dysmorphogenesis. A number of environmental factors are discussed that may plausibly be candidates for this role, and suggestions are offered for testing the model. The model also suggests a number of measures that may be effective both in reducing the risk of fetal CRS in women who acquire rubella in their first trimester and in reversing or minimizing regressive autism among children in whom the diagnosis is suspected or confirmed.
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3.
Understanding and managing cardiovascular outcomes in liver transplant recipients.
Izzy, M, VanWagner, LB, Lee, SS, Altieri, M, Angirekula, M, Watt, KD
Current opinion in organ transplantation. 2019;(2):148-155
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Abstract
PURPOSE OF REVIEW Cardiovascular disease (CVD) is a common cause of mortality after liver transplantation. The transplant community is focused on improving long-term survival. Understanding the prevalence of CVD in liver transplant recipients, precipitating factors as well as prevention and management strategies is essential to achieving this goal. RECENT FINDINGS CVD is the leading cause of death within the first year after transplant. Arrhythmia and heart failure are the most often cardiovascular morbidities in the first year after transplant which could be related to pretransplant diastolic dysfunction. Pretransplant diastolic dysfunction is reflective of presence of cirrhotic cardiomyopathy which is not as harmless as it was thought. Multiple cardiovascular risk prediction models have become available to aid management in liver transplant recipients. SUMMARY A comprehensive prevention and treatment strategy is critical to minimize cardiovascular morbidity and mortality after liver transplant. Weight management and metabolic syndrome control are cornerstones to any prevention and management strategy. Bariatric surgery is an underutilized tool in liver transplant recipients. Awareness of 'metabolic-friendly' immunosuppressive regimens should be sought. Strict adherence to the cardiology and endocrine society guidelines with regard to managing metabolic derangements post liver transplantation is instrumental for CVD prevention until transplant specific recommendations can be made.
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Prevalence of risk factors for liver disease in a random population sample in southern Germany.
Huetter, ML, Fuchs, M, Hänle, MM, Mason, RA, Akinli, AS, Imhof, A, Kratzer, W, Lorenz, R, ,
Zeitschrift fur Gastroenterologie. 2014;(6):558-63
Abstract
BACKGROUND Chronic liver disease leads to fibrosis and cirrhosis of the liver. This may, in turn, result in chronic liver failure or the development of hepatocellular carcinoma (HCC). Main risk factors for chronic liver disease are viral hepatitis and alcoholism. The present study assessed a randomly selected population in southern Germany for risk factors for chronic liver disease such as fatty liver disease, viral hepatis infection and life-style factors. In addition, the potential association with elevated liver enzymes was investigated. METHODS A total of 2256 subjects (1182 females, 1074 males), aged 18 - 65 years, participated in the study. Each subject underwent a standardized ultrasound examination, and anthropometric and biochemical assessments. Test subjects were randomly selected from the general population of a town in southwestern Germany. Data were acquired from November to December 2002 without further follow-up. RESULTS Several factors were found to be associated with chronic liver disease in the study population. Alcohol consumption >20 g/d was seen in 18.1% (n=409). Metabolic syndrome was diagnosed in 5.9% (n=132). The number of people with a BMI greater than 25 kg/m(2) was 45.1% (n=1017). The prevalence of subjects with chronic hepatitis B was 0.7% (n=15), that of anti-HCV positive patients, 0.6%(n=15). Elevated gGT was seen in 10.4% (n=14) of the patients. Prevalence of hepatic steatosis was 25.0% (n=564). CONCLUSIONS Many cases of chronic liver disease could be prevented by healthy nutrition, optimized medical treatment of associated disorders, and prevention strategies such as routine vaccination, in particular, against hepatitis B virus (HBV).
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Role of the intestinal microbiome in liver disease.
Henao-Mejia, J, Elinav, E, Thaiss, CA, Licona-Limon, P, Flavell, RA
Journal of autoimmunity. 2013;:66-73
Abstract
The liver integrates metabolic outcomes with nutrient intake while preventing harmful signals derived from the gut to spread throughout the body. Direct blood influx from the gastrointestinal tract through the portal vein makes the liver a critical firewall equipped with a broad array of immune cells and innate immune receptors that recognize microbial-derived products, microorganisms, toxins and food antigens that have breached the intestinal barrier. An overwhelming amount of evidence obtained in the last decade indicates that the intestinal microbiota is a key component of a wide variety of physiological processes, and alterations in the delicate balance that represents the intestinal bacterial communities are now considered important determinants of metabolic syndrome and immunopathologies. Moreover, it is now evident that the interaction between the innate immune system and the intestinal microbiota during obesity or autoimmunity promotes chronic liver disease progression and therefore it might lead to novel and individualized therapeutic approaches. In this review, we discuss a growing body of evidence that highlights the central relationship between the immune system, the microbiome, and chronic liver disease initiation and progression.
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[Current trends in liver biopsy indications in chronic liver diseases].
Cadranel, JF, Nousbaum, JB
Presse medicale (Paris, France : 1983). 2012;(11):1064-70
Abstract
Liver biopsy (LB) remains a major tool in chronic liver disease evaluation. Main current indications of LB in chronic liver disease are reviewed in this manuscript. Major development of non-invasive tools for evaluation of liver fibrosis led to decrease of LB indications in patients with chronic hepatitis C. LB is the only tool for exploration of necroinflammatory and fibrosis lesions in chronic hepatitis B as well as in autoimmune hepatitis. LB is the sole exam that can differentiate between bland steatosis and steatohepatitis in the setting of metabolic syndrome and to confirm the diagnosis of alcoholic hepatitis when corticosteroids are indicated.
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The prominent role of the liver in the elimination of asymmetric dimethylarginine (ADMA) and the consequences of impaired hepatic function.
Richir, MC, Bouwman, RH, Teerlink, T, Siroen, MP, de Vries, TP, van Leeuwen, PA
JPEN. Journal of parenteral and enteral nutrition. 2008;(6):613-21
Abstract
Asymmetric dimethylarginine (ADMA) is an endogenous inhibitor of nitric oxide synthase (NOS), the enzyme which converts the amino acid arginine into nitric oxide (NO). ADMA has been identified as an important risk factor for cardiovascular diseases. Besides the role of ADMA in cardiovascular diseases, it also seems to be an important determinant in the development of critical illness, (multiple) organ failure, and the hepatorenal syndrome. ADMA is eliminated from the body by urinary excretion, but it is mainly metabolized by the dimethylarginine dimethylaminohydrolase (DDAH) enzymes that convert ADMA into citrulline and dimethylamine. DDAH is highly expressed in the liver, which makes the liver a key organ in the regulation of the plasma ADMA concentration. The prominent role of the liver in the elimination of ADMA and the consequences of impaired hepatic function on ADMA levels will be discussed in this article.
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Albumin therapy in clinical practice.
Mendez, CM, McClain, CJ, Marsano, LS
Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition. 2005;(3):314-20
Abstract
Albumin is the predominant product of hepatic protein synthesis and one of the more abundant plasma proteins. Among its multiple physiologic roles, it plays an essential part in the generation of colloid-oncotic pressure. In the United States, the indications for which albumin therapy are considered include hypovolemia or shock, burns, hypoalbuminemia, surgery or trauma, cardiopulmonary bypass, acute respiratory distress syndrome, hemodialysis, and sequestration of protein-rich fluids. The use of this relatively expensive therapy accounts for up to 30% of the total pharmacy budget in certain hospitals. The use of albumin therapy in different clinical situations and its influence in morbidity and mortality have been reviewed in multiple randomized controlled trials and meta-analyses. Despite frequent reviews, the use of albumin remains controversial in several clinical situations. At the same time, these valuable reviews seem to have documented the advantages of albumin therapy in the management of ascites and clarified the use of albumin in volume resuscitation. More studies have been recommended to investigate the use of albumin in different doses and its role in hypoalbuminemia. This article will provide an overview of albumin metabolism, use of albumin for volume expansion, the potential therapeutic role of albumin in liver disease, and the role of albumin therapy in nutrition.
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9.
Etiology and pathogenesis of necrolytic migratory erythema: review of the literature.
Tierney, EP, Badger, J
MedGenMed : Medscape general medicine. 2004;(3):4
Abstract
CONTEXT Necrolytic migratory erythema (NME) is a characteristic skin condition seen in the presence of a pancreatic glucagonoma. The presence of NME in the absence of a pancreatic tumor has been termed the pseudoglucagonoma syndrome. In such cases, NME is commonly associated with conditions, such as liver disease, inflammatory bowel disease, pancreatitis, malabsorption disorders (ie, celiac sprue), and other malignancies. There are many theories on the pathogenesis of NME, which include the direct action of glucagon in inducing skin necrolysis, hypoaminoacidemia-inducing epidermal protein deficiency and necrolysis, a nutritional or metabolic deficiency of zinc or essential fatty acids, liver disease, glucagon induction of inflammatory mediators, a substance secreted from pancreatic and other visceral tumors associated with NME, and generalized malabsorption. OBJECTIVE To present a review of the literature on the clinical presentation, etiology, pathogenesis, and treatment of NME. DESIGN Review of the literature on NME occurring in patients both with and without a pancreatic glucagonoma. METHODS We performed a PubMed review of the literature on the etiology and pathogenesis of NME to identify case reports and reviews published in both the internal medicine and dermatology literature. RESULTS Our literature review encompassed 17 primary case reports and literature reviews published in the dermatologic and internal medicine literature on NME in patients both with and without a pancreatic glucagonoma. Although we found no clear consensus among the investigators of a universally accepted pathogenesis for NME, we did identify 4 main categories of etiologic/pathogenetic mechanisms for NME (glucagon excess, nutritional deficiencies, inflammatory mediators, and liver disease) that were discussed by many of the investigators and validated by both clinical and scientific evidence. CONCLUSION The exact pathogenesis and treatment of NME remain ill-defined despite many case reports and studies on NME in the literature. The many systemic diseases and nutritional deficiencies that have been found to be associated with NME suggest a multifactorial model for the pathogenesis of the disease. The most comprehensive, postulated mechanism for NME involves a combination of zinc, amino acid, and fatty acid deficiencies (arising from a wide variety of causes, such as dietary insufficiency, malabsorption syndromes, liver disease, elevated glucagon levels, and disorders of metabolism) that contributes to increased inflammation in the epidermis in response to trauma and to the necrolysis observed in NME. The importance of gaining an understanding of the etiology and pathogenesis of NME lies in the fact that there is no universally accepted mechanism of pathogenesis for NME, and that the only treatment reported to resolve the rash in these patients is to adequately identify and treat the underlying associated systemic condition or nutritional deficiency.
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New developments in the pathophysiology, clinical spectrum, and diagnosis of disorders of fatty acid oxidation.
Treem, WR
Current opinion in pediatrics. 2000;(5):463-8
Abstract
Fatty acid oxidation disorders are among the most common inborn errors of metabolism affecting infants and children. Recognition of this family of defects is critical because careful dietary monitoring, avoidance of fasting, and prompt intervention during common childhood illness can prevent catastrophic cardiac and metabolic decompensation. This review focuses on new molecular and clinical diagnostic aspects of several of these disorders. Recent papers highlight the recognition that the clinical spectrum of disorders of fatty acid oxidation goes far beyond the stereotypical Reyes-like presentation or cardiomyopathy, and now encompasses more cases of sudden infant death syndrome, fulminant hepatic failure, and severe complications during pregnancy.