gerstmann-straussler-scheinker disease/triglyceride

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Medium chain triglycerides and hepatic encephalopathy.

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The oral administration of short (C(6)) and medium (C(8) and (C(10)) chain triglycerides produced no clinical or electroencephalographic changes in patients with cirrhosis of the liver. Arterial ammonia levels were also monitored in these patients and showed no significant change after medium chain

Elevated triglycerides levels in two infants with hypoxic-ischemic encephalopathy undergoing therapeutic hypothermia and receiving parenteral nutrition.

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Therapeutic hypothermia has been widely applied to improve the survival/neurodevelopmental outcomes among infants with moderate-to-severe hypoxic ischemic encephalopathy (HIE). Due to their critical condition and concerns over feeding tolerance, it is not uncommon to withhold enteral feeds and

Protective role of antioxidants on thioacetamide-induced acute hepatic encephalopathy: biochemical and ultrastructural study.

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Thioacetamide (TAA) has been used in development of animal models of acute hepatic encephalopathy (AHE). This experimental study was designed to evaluate effects of oral administration of vitamin C, vitamin E and their combination on liver and brain enzymes and their histologic and ultrastructure

[The structural-functional properties of the erythrocyte membranes in patients with ischemic stroke and circulatory encephalopathy].

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68 patients with cerebrovascular disorders (37 cases of ischemic stroke and 31 cases of dyscirculatory encephalopathy) were examined for microhemorheology (erythrocytic form and deformability); fractional composition of neutral lipids; blood phospholipids; those in red cells and their membrane

Prophylaxis of encephalopathies and risk factors of atherogenesis development in the postresuscitation period in rats by means of succinic acid.

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The effect of oral administration of succinic acid was studied in 66 rats exposed to 10 min cardiac arrest with further resuscitation. A total of 30 mg/kg of the drug were administered daily for 5 days starting with day 3 up to day 7 after resuscitation. The experiments have revealed that treatment

Studies on the tolerance of medium chain triglycerides in dogs.

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Two groups of five conscious dogs received total parenteral nutrition (about 100 kcal/kg body weight per 24 hr) continuously for 96 hr (0.28 g triglycerides/kg body weight per hr, constituting more than 55% of the energy supply). The only difference between the two groups was the nature of the 20%

[Metabolic triglyceride storage disorders. A report of 2 cases of systemic carnitine deficiency].

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Two cases of triglyceride storage in liver, kidney, heart, and skeletal muscle are described in infants who died at the age of 1 1/2 years and 4 d, respectively. In the first patient, a previously normal girl, the clinical symptoms began two months before death with encephalopathy (vomiting,

Medium-chain triglyceride supplementation under a low-carbohydrate formula is a promising therapy for adult-onset type II citrullinemia.

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BACKGROUND Citrin, encoded by SLC25A13, is a component of the malate-aspartate shuttle, which is the main NADH-transporting system in the liver. Citrin deficiency causes neonatal intrahepatic cholestasis (NICCD), which usually resolves within the first year of life. However, small numbers of adults

Tissue lipids in hyperammonemic encephalopathies of childhood.

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Cholesterol, triglyceride, free fatty acid, and phospholipid concentrations were measured in 33 brain, liver, and adipose tissue samples obtained from 17 children who died of an acute encephalopathy associated with liver dysfunction and hyperammonemia (hyperammonemic encephalopathy). Eleven patients

Factors in serum and cerebrospinal fluid from children with viral encephalopathies impair glucose transport.

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Since glucose transport appears to be inhibited in viral infections, we looked for inhibitors in cerebrospinal fluid (CSF) and serum of children with febrile convulsions (FC) and Singapore syndrome (SS). When incubated with rat and human adipocytes both fluids from FCs inhibited the utilization of

Medium-chain triglycerides supplement therapy with a low-carbohydrate formula can supply energy and enhance ammonia detoxification in the hepatocytes of patients with adult-onset type II citrullinemia.

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Citrin, encoded by SLC25A13, constitutes the malate-aspartate shuttle, the main NADH-shuttle in the liver. Citrin deficiency causes neonatal intrahepatic cholestasis (NICCD) and adult-onset type II citrullinemia (CTLN2). Citrin deficiency is predicted to impair hepatic glycolysis and de novo

Intensive enteral feeding in advanced cirrhosis: reversal of malnutrition without precipitation of hepatic encephalopathy.

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Ten children with advanced cirrhosis and malnutrition (less than 90% weight for height) were fed for eight weeks with a nasogastric feed comprising whey protein (enriched with branched chain amino acids), fat as 34% medium chain and 66% long chain triglycerides, and glucose polymer. Six of the

Polymerase gamma deficiency (POLG): clinical course in a child with a two stage evolution from infantile myocerebrohepatopathy spectrum to an Alpers syndrome and neuropathological findings of Leigh's encephalopathy.

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OBJECTIVE Description of the clinical course in a child compound heterozygous for POLG1 mutations, neuropathology findings and results of dietary treatment based on fasting avoidance and long chain triglycerides (LCT) restriction. RESULTS At 3(1/2) months of age the patient presented with severe

Ameliorative effect of saffron aqueous extract on hyperglycemia, hyperlipidemia, and oxidative stress on diabetic encephalopathy in streptozotocin induced experimental diabetes mellitus.

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Diabetic encephalopathy is one of the severe complications in patients with diabetes mellitus. Findings indicate that saffron extract has antioxidant properties but its underlying beneficial effects on diabetic encephalopathy were unclear. In the present study, the protective activities of saffron

New electrophysiological assessments for the early diagnosis of encephalopathy and peripheral neuropathy in chronic uraemia.

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The clinical manifestations of neuropathy in chronic uraemia are late in occurring. However, at an early stage it is possible to detect damage to either the central or peripheral nervous system by means of modern electrophysiological investigation methods. We examined 29 patients with CRF (plasma

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