Liver transplantation for inborn errors of liver metabolism.
Liver transplantation brings complete recovery from end-stage liver disease, and full correction of liver based inborn errors of metabolism.
Health Knowledge, Attitudes, Practice↗
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[Liver transplantation for metabolic liver disease in adulthood].
The clinical presentation of metabolic liver disease is highly variable, covering acute liver failure, liver cirrhosis, hepatic cancer and various extrahepatic manifestations. Both natural course and prognosis after liver transplantation are substantially influenced by extrahepatic manifestations. In many types of metabolic liver disease, timely diagnosis allows for successful medical treatment. However, progressive liver failure and severe extrahepatic damage can be the indication for liver transplantation. In general, standard transplantation criteria also apply for metabolic liver disease. They have to be modified by disease-specific criteria, and extrahepatic damage may necessitate multiorgan transplantation. The overall prognosis after liver transplantation for metabolic liver disease is favorable. Furthermore, several metabolic defects are phenotypically cured by liver transplantation. Alternative treatments like hepatocyte transplantation or gene therapy are still in the experimental stage.
Adult↗
Effects of gastrectomy or colectomy on liver metabolism and liver morphology in an experimental rat model.
BACKGROUND: It is sometimes necessary to translocate part of the gastrointestinal tract into the site of the organ earlier resected. This occurs after total esophageal, stomach or colon resection, which can lead to disturbances in the anatomical and functional balance of the gastrointestinal tract and the body as a whole. The aim of this study was to evaluate the effects of different resective surgical procedures performed on the proximal and distal GI tract in terms of total body weight, selected liver function tests, and ultrastructural changes in the liver in laboratory rats. MATERIAL/METHODS: The total of 57 resective procedures consisted of 31 total gastric and 26 total colon resections. The control group consisted of 12 animals on which only laparotomy was performed. Body weight, stool character, and the general condition of the animals were evaluated from the time of the operation. Twenty-six blood samples were collected for analysis of hemoglobin, total plasma protein, and liver aminotransferases levels. Thirty liver tissue samples from the 3 groups were taken and examined. RESULTS: We observed statistically significant decreases in total body weight, hemoglobin, and total plasma protein levels in the group where reconstructive procedures following gastrectomy were performed. In the group in which reconstructive procedures followed colectomy, a statistically significant decrease in total body weight was observed. Aminotransferase level changes were neither characteristic nor significantly different in any of the three groups involved in the investigation. CONCLUSIONS: Abnormalities of liver structure were observed at the ultrastructural level. Any correlation between these changes, general condition, and selected biochemical parameters requires further controlled clinical and experimental studies.
Alanine Transaminase↗
Liver transplantation for metabolic liver diseases.
Liver transplantation has revolutionized the outcome of metabolic liver diseases that are caused by defects in hepatocytes (e.g., Wilson's disease) or by excessive deposition of substrates secondary to their increased absorption (e.g., hemochromatosis). Early diagnosis and referral are the keys to successful outcome. The timing of liver transplantation for patients on medical therapy depends on a lack of biochemical and clinical evidence of improvement. Overall outcome following liver transplantation depends on the severity of multisystem involvement and preoperative decompensation.
Hemochromatosis↗
Adult liver transplantation for metabolic liver disease.
Liver replacement provides an effective method of replacing a failing liver, and corrects the underlying defect in many metabolic conditions. Results of liver transplantation for metabolic diseases have been encouraging, with the exception of hereditary hemochromatosis, in which infectious and for which cardiac complications appear to increase posttransplant mortality. An improved understanding of the underlying genetic and molecular defect will lead to advances in medical therapy and perhaps will decrease the need for liver replacement. The prospects of gene therapy are being pursued for many metabolic disorders, however until this research leads to direct clinical application, liver transplantation remains the only effective option for many patients with metabolic liver disease.
Amyloidosis↗
Biotin deficiency and liver metabolism in relation to fatty liver and kidney syndrome.
Varying degrees of biotin deficiency were induced by adding freeze-dried, raw egg white to the diet of broiler chicks. Aspects of liver metabolism were studied with reference to fatty liver and kidney syndrome. Mortality was low with 11.8 g egg white/kg diet, or less, but with 17.7 g/kg or more, mortality was very high. High mortality was observed with less than 0.33 microgram biotin/g liver. Associated with low concentrations of liver biotin were substantial increases in liver weight and lipid content in starved birds. The increased liver lipid content was not observed in birds fed ad libitum. The increased liver lipid content in biotin-deficient, starved birds was not reflected in the specific activities of hepatic lipogenic enzymes or hepatic lipogenesis in vivo measured by the incorporation of tritium from 3H-labelled water into liver lipid. Biotin deficiency affected the specific activities of the biotin-requiring enzymes, pyruvate carboxylase and acetyl CoA carboxylase, differently; the latter was unaffected whereas the former decreased concomitantly with liver biotin concentration.
Animals↗
Metabolic liver function and lipoprotein metabolism after orthotopic liver transplantation in patients on immunosuppressive therapy with FK 506 or cyclosporine.
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Adolescent↗
Functional portal flow competition after auxiliary partial orthotopic living donor liver transplantation in noncirrhotic metabolic liver disease.
Auxilliary partial orthotopic liver transplantation (APOLT) was introduced initially as a tentative or permanent support for patients with potentially reversible fulminant hepatic failure and has extended its indication to congenital metabolic disorder of the liver that has otherwise normal functional integrity. Postoperative management of APOLT is complicated because of functional portal flow competition between the native and graft liver. The native portal vein diversion to the graft is sometimes indicated to prevent functional competition; however, it is still an open question whether this technique can be theoretically indicated for APOLT patients. The authors report a on patient with ornithine transcarbamylase deficiency who received APOLT from a living donor without native portal vein diversion. Because of functional portal vein competition between the native and graft liver, the patient had to have portal vein diversion, portal vein embolization, and finally native hepatectomy to induce the graft regeneration after APOLT. After the experience of the current case, primary portal vein diversion for APOLT with noncirrhotic metabolic liver disease patients to prevent functional portal flow competition is recommended.
Anastomosis, Surgical↗
Metabolic liver disease in the pediatric patient.
Most metabolic liver diseases that affect pediatric patients present in the neonatal period with either cholestasis or acute liver failure. Metabolic liver disease in the older child has considerable overlap with adult patients. New diagnostic methods and therapy, including liver transplantation, has radically changed the outcome of many metabolic liver diseases.
Alagille Syndrome↗
Living donor liver transplantation for noncirrhotic inheritable metabolic liver diseases: impact of the use of heterozygous donors.
BACKGROUND: In living donor liver transplantation (LDLT), the liver donor is almost always a blood relative; therefore, the donor is sometimes a heterozygous carrier of inheritable diseases. The use of such carriers as donors has not been validated. The aim of the present study was to evaluate the outcome of LDLT for noncirrhotic inheritable metabolic liver disease (NCIMLD) to clarify the effects of using a heterozygous carrier as a donor. METHODS: Between June 1990 and December 2003, 21 patients with NCIMLD underwent LDLT at our institution. The indications for LDLT included type II citrullinemia (n = 7), ornithine transcarbamylase deficiency (n = 6), propionic acidemia (n = 3), Crigler-Najjar syndrome type I (n = 2), methylmalonic acidemia (n = 2), and familial amyloid polyneuropathy (n = 1). Of these 21 recipients, six underwent auxiliary partial orthotopic liver transplantation. RESULTS: The cumulative survival rate of the recipients was 85.7% at both 1 and 5 years after operation. All surviving recipients are currently doing well without sequelae of the original diseases, including neurological impairments or physical growth retardation. Twelve of the 21 donors were considered to be heterozygous carriers based on the modes of inheritance of the recipients' diseases and preoperative donor medical examinations. All donors were uneventfully discharged from the hospital and have been doing well since discharge. No mortality or morbidity related to the use of heterozygous donors was observed in donors or recipients. CONCLUSIONS: Our results suggest that the use of heterozygous donors in LDLT for NCIMLD has no negative impact on either donors or recipients, although some issues remain unsolved and should be evaluated in further studies.
Acidosis↗
Hexokinase and 'glucokinase' in liver metabolism.
Rat liver contains four hexokinase isoenzymes, one of which, despite often being called 'glucokinase', is no more specific for glucose than the others. However, it does differ from them in displaying a sigmoid kinetic response to glucose, requiring much higher glucose concentrations for activity, and being insensitive to physiological concentrations of glucose 6-phosphate.
Animals↗
Metabolic liver diseases in childhood: Indian scenario.
The present survey was undertaken to ascertain the spectrum of childhood metabolic liver diseases (MLD) and diagnostic methods available in teaching medical institutions in India. Out of 17 medical colleges approached in different parts of the country, pediatricians from 11 institutions agreed to participate. Six colleges had organised pediatric gastroenterology & hepatology services (category I) and five were not having such services (category II). The participants provided information regarding the number of chronic liver disease patients seen at their centres during the past 5-10 years with their etiological diagnosis, the proportion of metabolic liver diseases in these children and the methods used to label etiology. Patients with acute hepatitis B infection and neonatal cholestasis were excluded. In the past 1-9 years, (38-236) and (4-57) children with chronic liver disease (CLD) were seen in category I and category II centres respectively. Chronic hepatitis B and C and metabolic liver diseases were being diagnosed in most of the centres, though in category I other causes of chronic liver diseases were also reported. Metabolic liver diseases constituted 8 to 43% of the reported CLD in category I colleges and 0-46% in the other pediatric centres. Most major categories of metabolic liver diseases were seen at various centres. Indian Childhood Cirrhosis (ICC) was very infrequently reported even in the large hospitals and Wilson's disease was the most frequently diagnosed metabolic liver disease. Reference diagnostic tests for most of the metabolic liver disorders were not accessible to majority of participating institutions. Metabolic liver diseases constitute a significant proportion of childhood chronic liver diseases in our country. There is an important need to set up reference laboratories in the country to facilitate diagnostic work up of metabolic liver diseases at affordable costs.
Acute Disease↗
The predominant role of S-oxidation in rat liver metabolism of thiaarenes.
Thiaarenes are metabolized by liver microsomes of untreated rats predominantly to sulfones and sulfoxides. After pretreatment of rats with monooxygenase inducers, ring oxidation of thiaarenes is also observed. In case of benzo[b]naphtho[2,3-d]thiophene the formation of a p-quinone takes place. Rat liver microsomal metabolism of the thiaarenes tested as substrates did not resemble that of the polycyclic aromatic hydrocarbon (PAH) isosters at all.
Animals↗
Liver metabolic effects of intestinal shock and naloxone treatment in the rat.
The liver metabolic response of rats following a standardized intestinal shock, induced by applying a pressure of 120 cm water on the mesenteric vessels for 60 min, was studied. Immediately prior to the release of the pressure on the vessels saline or naloxone was given either as a single injection or as a continuous infusion. After the reperfusion of the intestine no early disturbances in liver metabolism were found as evidenced from the ATP, glucose and lactate levels in liver biopsies taken 15 min following reflow. Within 60 min of reflow reduction of ATP and increases of glucose and lactate levels occurred. There were no major hemodynamic or liver metabolic differences between saline- and naloxone-treated shocked rats. When saline or naloxone was given as a continuous infusion, the changes in liver metabolism were, however, less severe than those observed in the single injection situation pointing toward a non-specific effect of volume replacement rather than a blockade of opioid receptors. Hepatic hypoxia and/or cellular effects of "shock factors" could be mechanisms of pathophysiologic importance for the disturbed liver metabolism in this shock model.
Acid-Base Equilibrium↗
Living-related liver transplantation in inherited metabolic liver disease: feasibility and cautions.
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Adolescent↗
[Corticosteroids and the liver. Metabolism and indications in liver diseases].
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Adrenal Cortex Hormones↗
INFLUENCE OF ETHANOL ON THE LIVER METABOLISM OF FED AND STARVED RATS.
1. The influence of ethanol on the metabolism of livers from fed and starved rats has been studied in liver-perfusion experiments. Results have been obtained on oxygen consumption and carbon dioxide production, on glucose release and uptake by the liver and on changes in the concentrations of lactate and pyruvate and of beta-hydroxybutyrate and acetoacetate in the perfusion medium. 2. Oxygen consumption and carbon dioxide production were lower in livers from starved rats than in livers from fed rats. Ethanol had no effect on the oxygen consumption of either type of liver. After the addition of ethanol to the perfusion medium carbon dioxide production ceased almost completely, the change being faster in livers from starved rats. 3. With livers from fed rats glucose was released from the liver into the perfusion medium. This release was slightly greater when ethanol was present. With livers from starved rats no release of glucose was observed, and when ethanol was added a marked uptake of glucose from the medium was found. A simultaneous release of glycolytic end products, lactate and pyruvate, into the medium occurred. 4. Acetate was the main metabolite accumulating in the perfusion medium when ethanol was oxidized. With livers from starved rats a slightly increased formation of ketone bodies was found when ethanol was present. 5. The lactate/pyruvate concentration ratio in the perfusion medium increased from 10 to 87 with livers from fed rats and from 20 to 171 with livers from starved rats when the livers were perfused with ethanol in the medium. The beta-hydroxybutyrate/acetoacetate concentration ratio increased from 0.8 to 7.6 with livers from fed rats and from 1.0 to 9.5 with livers from starved rats when ethanol was added to the medium. 6. The effects of ethanol are discussed and related to changes in the redox state of the liver that produce new conditions for some metabolic pathways.
Acetoacetates↗
Metabolic liver disease of childhood.
The broad topic of metabolic liver disease may be divided into three pathophysiologic groups -- storage diseases, cholestatic syndromes, and metabolic defects contributing to hepatocellular necrosis. The important disease entities in each group have been discussed in terms of incidence, clinical course, inheritance, diagnostic criteria, pathogenesis, hepatic pathology, and therapy. An approach to the evaluation of metabolic liver disease has been proposed. It is hoped that this review will be helpful to the clinician who is engaged in the primary care of children.
Child↗