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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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[Diagnosis of iron overload syndrome].
Polunina, TE, Maev, IV
Eksperimental'naia i klinicheskaia gastroenterologiia = Experimental & clinical gastroenterology. 2010;(5):61-8
Abstract
The article presents a classification of conditions, accompanied with increasing of iron accumulation of the liver cells, normal iron metabolism in the human body, etiology, epidemiology, multifactorial structure of the pathogenesis of HFE homozygous hemochromatosis, clinic of iron overload syndrome. The modern algorithms of diagnostic and tactics of patients with the use of genotyping and correction of iron content.
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8.
Hepatic osteodystrophy.
Goel, V, Kar, P
Tropical gastroenterology : official journal of the Digestive Diseases Foundation. 2010;(2):82-6
Abstract
UNLABELLED Hepatic Osteodystrophy (HO) is a generic definition for the metabolic bone disease that may occur in individuals with chronic liver disease. Hepatic Osteodystrophy is an important but frequently overlooked complication, seen in chronic liver disease patients. This review article illustrates its significance, various causes and methods to diagnose this complication and recent advances and recommendations to treat Hepatic Osteodystrophy. Two distinct bone metabolic processes, osteoporosis (OP) and osteomalacia (OM) are combined together in various proportions in HO syndromes. It has been described in association with most types of chronic liver disease both cholestatic and non-cholestatic. Primary biliary cirrhosis (PBC) is the condition causing osteopenia more frequently, but other cholestatic liver diseases like primary sclerosing cholangitis (PSC), haemochromatosis and alcoholic liver disease are also frequently associated with this disorder. The pathogenesis of bone disease in both adults and children with chronic cholestasis is not completely understood. There has been considerable disagreement regarding the relative importance of osteomalacia versus osteoporosis as the factors leading to osteopenia of liver disease. It can significantly affect morbidity, and quality of life of these patients. Fractures are also associated with an excess mortality. Bone mineral density measurement is the best way to assess the presence and severity of osteopenia in CLD patients, while laboratory tests give important information about the metabolic status of the bone. Since advanced HO is difficult to treat and adversely affects both the quality of life and the long-term prognosis of patients with chronic liver disease, special care is required in order to prevent the development of clinical bone disease in individuals with advanced hepatic disease. CONCLUSION Hepatic Osteodystrophy is under-recognized and less attended complication of CLD. Multiple factors contribute to the development of hepatic Osteodystrophy. Newer diagnostic modalities have improved the detection of HO and Vitamin D repletion, calcium supplementation and Bisphosphonates seem promising. The best course of management for these patients is to review the individual risk factors for osteoporosis, obtain a bone mass measurement, and prescribe age and disease-specific therapies.
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Factors affecting catch-up growth after liver transplantation in children with cholestatic liver diseases.
Pawlowska, J, Socha, P, Jankowska, I
Annals of transplantation. 2010;(1):72-6
Abstract
Growth retardation is one of the most prominent consequences of childhood cholestatic liver diseases. The pathogenesis of malnutrition is multifactorial and includes reduced calorie intake, fat malabsorption, abnormal protein metabolism, and increased energy expenditure. Poor growth is a typical feature of biliary atresia, cystic fibrosis, progressive familial intrahepatic cholestasis and Alagille syndrome. In some of these features it is not only directly related to impaired nutrient absorption. Liver transplantation is required if liver disease progresses to liver failure - improved nutritional status is achieved within weeks after surgery. Still, patients with Alagille syndrome present with only slight catch-up growth after transplantation, while patients with PFIC and biliary atresia grow significantly.
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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.