Effects of an individualized nutrition intervention on the respiratory quotient of patients with liver failure.

BACKGROUND AND OBJECTIVES: Malnutrition and energy metabolism disorders are characterized by a low respiratory quotient in patients with liver failure and often lead to poor prognosis. Therefore, early nutrition interventions are crucial for patients with liver failure to ameliorate abnormal metabolic status and malnutrition. This study explored the effect of an individualized nutrition intervention on the respiratory quotient of patients with liver failure. METHODS AND STUDY DESIGN: An individualized 2-week nutrition intervention was conducted on patients with nutritional risk caused by liver failure according to patient resting energy expenditure. Patients were separated into two groups for further analysis according to whether their energy intake reached 1.2 times their resting energy expenditure. RESULTS: Fifty-two patients with nutritional risk caused by liver failure were enrolled. Their average respiratory quotient was 0.79 (0.76-0.84) at the baseline. Patients with an energy intake of >=1.2 times their resting energy expenditure had a higher respiratory quotient and lower scores on the model for endstage liver disease and Child-Pugh test than those with an energy intake of <1.2 times their resting energy expenditure at weeks 1 and 2 after the intervention. Moreover, no significant differences were observed between the two groups at the baseline. Respiratory quotient was negatively correlated with the model for end-stage liver disease and Child-Pugh scores. CONCLUSIONS: Individualized nutrition interventions with an energy intake >=1.2 times the patient's resting energy expenditure can effectively improve the respiratory quotient and reduce disease severity in patients with nutritional risk caused by liver failure.

Remaining Challenges in the Treatment of Tyrosinemia from the Clinician's Viewpoint.

This chapter provides a clinical perspective on the challenges that stand between current clinical practice and a cure for hepatorenal tyrosinemia (HT1). HT1 has been transformed in the last 50 years from an aggressive often undiagnosed childhood disease causing liver failure or liver cancer, with infant death in most patients, to a condition that is detectable at birth, and for which treatment with nitisinone (NTBC) and diet can prevent detectable liver or kidney abnormalities. What challenges remain? The properties of the affected metabolic pathway and the broad spectrum of severity seen in untreated patients are incompletely understood but potentially important for patients. Available treatments have potential complications, including liver transplantation (risks of surgery and of immunosuppression to prevent rejection), nitisinone and diet therapy (hypertyrosinemia, corneal opacities, nutritional imbalances and possibly developmental delay). The detection of liver cancer is imperfect and laborious. The effects of tyrosinemia during pregnancy are little-known. Although animal models of HT1 are becoming standard research tools in cell replacement and gene modification therapy, these techniques are not currently applicable to HT1 itself. Treatment adherence is variable, causing concern about long term outcome for some patients. Around the world, there are great disparities in the diagnosis and treatment of HT1. Most affected individuals are born in places where newborn screening for HT1 is not performed and where appropriate treatment is not available. We hope that this list will help to focus on some of these remaining obstacles to a cure for HT1.

Improvement in the nutritional status and clinical conditions of patients with liver failure using a liver diet combined with a branched chain amino acids-enriched elemental diet.

BACKGROUND/AIMS: This study was performed to evaluate any improvement in the nutritional state and clinical symptoms in patients with liver failure and advanced cirrhosis after consumption of a liver diet with restricted energy and protein, in combination with a branched chain amino acids (BCAA)-enriched elemental diet. METHODOLOGY: A BCAA-enriched elemental diet, in combination with a liver diet, characterized by restricted energy and protein, was administered in divided meals to 20 patients with liver failure associated with ascites or hepatic encephalopathy for 4 weeks. RESULTS: The symptom of ascites abated as a result of increased total serum protein and albumin levels after the nutritional intervention in comparison with baseline levels. Ammonia levels were slightly increased without exacerbating hepatic encephalopathy, and the protein nutrition state consequently improved. CONCLUSIONS: Divided meals of a BCAA-enriched elemental diet combined with a liver diet improved the nutritional state and clinical symptoms of patients with liver failure.

When can nutritional therapy impact liver disease?

This article reviews the current literature regarding nutritional therapy in liver disease, with an emphasis on patients progressing to liver failure as well as surgical patients. Mechanisms of malnutrition and sarcopenia in liver failure patients as well as nutritional assessment, nutritional requirements of this patient population, and goals and methods of therapy are discussed. Additionally, recommendations for feeding, micronutrient, branched chain amino acid supplementation, and the use of pre- and probiotics are included. The impact of these methods can have on patients with advanced disease and those undergoing surgical procedures will be emphasized.

Low and medium but not high doses of green tea polyphenols ameliorated dextran sodium sulfate-induced hepatotoxicity and nephrotoxicity.

Our previous study indicated that a diet containing a high dose (1%) of green tea polyphenols (GTPs) disrupted liver and kidney function via a reduction in antioxidant enzyme and heat shock protein (HSP) levels in both colitis and non-treated ICR mice. In the present study, we assessed the effects of 0.01%, 0.1%, and 1% dietary GTPs on liver and kidney physiological functioning in dextran sulfate sodium (DSS)-exposed and normal mice. GTPs at 0.01% and 0.1% significantly suppressed DSS-increased serum aspartate 2-oxoglutarate aminotransferase (AST) and alanine aminotransferase (ALT) levels. In contrast, GTPs at 1% increased kidney weight, serum creatinine levels, and thiobarbituric acid-reactive substances (TBARs) in both the kidney and the liver in normal mice, as compared with DSS-exposed mice. GTPs at 0.01% and 0.1% remarkably upregulated the expression of heme oxygenase-1 (HO-1) and heat shock protein 70 (HSP70) mRNA in the liver and kidney of mice exposed to DSS, whereas GTPs at 1% abolished it. Our results indicate that low and medium doses of GTPs have beneficial effects on DSS-induced hepatotoxicity and nephrotoxicity via upregulation of self-protective enzymes, while these effects disappeared at a high dose.

Control of acute, chronic, and constitutive hyperammonemia by wild-type and genetically engineered Lactobacillus plantarum in rodents.

UNLABELLED: Hyperammonemia is a common complication of acute and chronic liver diseases. Often accompanied with side effects, therapeutic interventions such as antibiotics or lactulose are generally targeted to decrease the intestinal production and absorption of ammonia. In this study, we aimed to modulate hyperammonemia in three rodent models by administration of wild-type Lactobacillus plantarum, a genetically engineered ammonia hyperconsuming strain, and a strain deficient for the ammonia transporter. Wild-type and metabolically engineered L. plantarum strains were administered in ornithine transcarbamoylase-deficient Sparse-fur mice, a model of constitutive hyperammonemia, in a carbon tetrachloride rat model of chronic liver insufficiency and in a thioacetamide-induced acute liver failure mice model. Constitutive hyperammonemia in Sparse-fur mice and hyperammonemia in a rat model of chronic hepatic insufficiency were efficiently decreased by Lactobacillus administration. In a murine thioacetamide-induced model of acute liver failure, administration of probiotics significantly increased survival and decreased blood and fecal ammonia. The ammonia hyperconsuming strain exhibited a beneficial effect at a lower dose than its wild-type counterpart. Improved survival in the acute liver failure mice model was associated with lower blood ammonia levels but also with a decrease of astrocyte swelling in the brain cortex. Modulation of ammonia was abolished after administration of the strain deficient in the ammonium transporter. Intestinal pH was clearly lowered for all strains and no changes in gut flora were observed. CONCLUSION: Hyperammonemia in constitutive model or after acute or chronic induced liver failure can be controlled by the administration of L. plantarum with a significant effect on survival. The mechanism involved in this ammonia decrease implicates direct ammonia consumption in the gut.

[Congenital galactosaemia: an unusual presentation]. / La galactosémie congénitale: une révélation singulière.

Congenital galactosaemia reveals usually in the second and third weeks of life with a severe liver dysfunction. We report on a case of congenital galactosaemia with, on the one hand, an early onset liver failure, without any free interval, and on the other hand, an hemophagocytic syndrome as a severe secondary outbreak with pulmonary haemorrhage. Appropriate diet led to normalisation of liver function. Hemophagocytosis, probably linked to an associated Klebsiella Pneumoniae sepsis, had a favourable outcome after antibiotic and corticosteroid therapy.

Celiac disease in patients with severe liver disease: gluten-free diet may reverse hepatic failure.

BACKGROUND & AIMS: Mild liver abnormalities are common in patients with celiac disease and usually resolve with a gluten-free diet. We investigated the occurrence of celiac disease in patients with severe liver failure. METHODS: Four patients with untreated celiac disease and severe liver disease are described. Further, the occurrence of celiac disease was studied in 185 adults with previous liver transplantation using serum immunoglobulin A endomysial and tissue transglutaminase antibodies in screening. RESULTS: Of the 4 patients with severe liver disease and celiac disease, 1 had congenital liver fibrosis, 1 had massive hepatic steatosis, and 2 had progressive hepatitis without apparent origin. Three were even remitted for consideration of liver transplantation. Hepatic dysfunction reversed in all cases when a gluten-free diet was adopted. In the transplantation group, 8 patients (4.3%) had celiac disease. Six cases were detected before the operation: 3 had primary biliary cirrhosis, 1 had autoimmune hepatitis, 1 had primary sclerosing cholangitis, and 1 had congenital liver fibrosis. Only 1 patient had maintained a long-term strict gluten-free diet. Screening found 2 cases of celiac disease, 1 with autoimmune hepatitis and 1 with secondary sclerosing cholangitis. CONCLUSIONS: The possible presence of celiac disease should be investigated in patients with severe liver disease. Dietary treatment may prevent progression to hepatic failure, even in cases in which liver transplantation is considered.

[Nutritional therapy in liver transplantation]. / Terapia nutricional no transplante hepático.

BACKGROUND: Malnutrition, sometimes severe is common in patients with chronic hepatic diseases who are candidates for liver transplantation. Nutritional therapy can induce partial or total correction of such deficiencies, improving clinical conditions and prognosis of patients who face the great defiance of liver transplantation. AIMS: Brief revision of hepatic role in the metabolism of several nutrients. Description of available methods of dietary therapy and its application both under different abnormal hepatic conditions and pre and post-transplant periods. The role of nutritional intervention in metabolic side effects due to immunosuppressive drugs. CONCLUSION: Nutritional therapy is a valuable adjuvant resource to the clinical treatment of candidates and submitted patients to hepatic transplantation providing better prognosis and improved life quality.

Feeding critically ill patients: current concepts.

Provision of nutritional support to critically ill patients can be challenging. Critical care nurses must be aware of which patients require specific nutritional support, when to initiate nutritional support, and by which route to provide nutritional support. Consultation with a dietitian or nutritional support service can help facilitate this process. The key points in addressing these questions are (1) the nutritional status of the patient or the length of time he or she has been without significant nutrient intake, (2) whether the patient has a hypermetabolic condition that warrants the early use of nutritional support, and (3) the function of the patient's gastrointestinal tract. What to feed depends on the physiological state of the patient. Adjusting the nutrient composition of the feeding solution may prevent metabolic complications and may improve the overall outcome for the patient.

Tentative diet for liver failure containing well-polished rice.

A liver-failure diet (low in protein) that contained rice polished to 50% to reduce the protein content of the diet was given to patients with uncompensated liver cirrhosis and compared with a standard liver-failure diet containing conventionally processed rice. The amount of boiled rice served in each meal could be increased by using well-polished rice and the use of supplementary sources of energy (powdered starch syrup, jelly, cookies, and candy sugar) was unnecessary. In the liver-failure diet containing well-polished rice, the methionine contents could be reduced and the Fischer ratio could be increased. The ingestion rate of the diet with well-polished rice was 80% and the diet was rated favorably in a questionnaire on palatability. Decreases in blood ammonia concentrations were observed in three patients given the liver-failure diet with well-polished rice for 2 wk by the crossover method.