Biomaterial Database

Removal of branched-chain amino acids and alpha-ketoisocaproate by haemofiltration and haemodiafiltration. 1996

J B Gouyon, and D Semama, and A Prévot, and J Desgres

Laboratoire de Néphrolgie-Hémaphérèse-Transplantation, Hôpital d'Enfants, Centre Hospitalier Universitaire, Dijon, France.

Venovenous haemofiltration (VVHF) and haemodiafiltration (VVHDF) were performed with a neonatal haemo(dia)filter (Miniflow 10, Hospal) on 8 anaesthetized rabbits infused with branched-chain amino acids (leucine, isoleucine and valine) and alpha-ketoisocaproate. The branched-chain amino acids (BCAA) and alpha-ketoisocaproate blood levels were close to those previously observed in neonates with maple syrup urine disease when extracorporeal blood purification was required. VVHF and VVHDF performances were assessed with two different blood flows (Qb = 8.3 and 16.6 ml/min). VVHDF was performed with four dialysate flow rates (Qd = 0.5, 1.0, 2.0 and 3.0 L/h). Within each period, clearances of the three BCAA were strictly similar. BCAA clearances obtained by VVHF were similar to ultrafiltration rates (respectively, 0.78 +/- 0.14 and 1.79 +/- 0.28 ml/min at high and low Qb; p < 0.05). The alpha-ketoisocaproate clearances obtained by VVHF were 0.39 +/- 0.17 and 0.92 +/- 0.43 ml/min at low and high Qb (not significantly different). Whatever the Qd value, the VVHDF procedures always allowed higher BCAA and alpha-ketoisocaproate clearances as compared with the corresponding VVHF period with similar Qb. BCAA clearances obtained by VVHDF with a 0.5 L/h dialysate flow were 4.1 +/- 0.5 and 5.4 +/- 0.5 mL/min at low and high Qb, respectively. The concurrent alpha-ketoisocaproate clearances were 2.5 +/- 0.8 and 2.9 +/- 1.0 ml/min.

UI	MeSH Term	Description	Entries
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D007651	Keto Acids	Carboxylic acids that contain a KETONE group.	Oxo Acids,Oxoacids,Acids, Keto,Acids, Oxo
D008297	Male		Males
D008375	Maple Syrup Urine Disease	An autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of branched-chain amino acids (AMINO ACIDS, BRANCHED-CHAIN). These metabolites accumulate in body fluids and render a "maple syrup" odor. The disease is divided into classic, intermediate, intermittent, and thiamine responsive subtypes. The classic form presents in the first week of life with ketoacidosis, hypoglycemia, emesis, neonatal seizures, and hypertonia. The intermediate and intermittent forms present in childhood or later with acute episodes of ataxia and vomiting. (From Adams et al., Principles of Neurology, 6th ed, p936)	Branched-Chain Ketoaciduria,Thiamine Responsive Maple Syrup Urine Disease,BCKD Deficiency,Branched-Chain alpha-Keto Acid Dehydrogenase Deficiency,Classic Maple Syrup Urine Disease,Classical Maple Syrup Urine Disease,Intermediate Maple Syrup Urine Disease,Intermittent Maple Syrup Urine Disease,Keto Acid Decarboxylase Deficiency,MSUD (Maple Syrup Urine Disease),Maple Syrup Urine Disease, Classic,Maple Syrup Urine Disease, Classical,Maple Syrup Urine Disease, Intermediate,Maple Syrup Urine Disease, Intermittent,Maple Syrup Urine Disease, Thiamine Responsive,Maple Syrup Urine Disease, Thiamine-Responsive,Branched Chain Ketoaciduria,Branched Chain alpha Keto Acid Dehydrogenase Deficiency,Branched-Chain Ketoacidurias,Ketoaciduria, Branched-Chain,Ketoacidurias, Branched-Chain
D008657	Metabolic Clearance Rate	Volume of biological fluid completely cleared of drug metabolites as measured in unit time. Elimination occurs as a result of metabolic processes in the kidney, liver, saliva, sweat, intestine, heart, brain, or other site.	Total Body Clearance Rate,Clearance Rate, Metabolic,Clearance Rates, Metabolic,Metabolic Clearance Rates,Rate, Metabolic Clearance,Rates, Metabolic Clearance
D011817	Rabbits	A burrowing plant-eating mammal with hind limbs that are longer than its fore limbs. It belongs to the family Leporidae of the order Lagomorpha, and in contrast to hares, possesses 22 instead of 24 pairs of chromosomes.	Belgian Hare,New Zealand Rabbit,New Zealand Rabbits,New Zealand White Rabbit,Rabbit,Rabbit, Domestic,Chinchilla Rabbits,NZW Rabbits,New Zealand White Rabbits,Oryctolagus cuniculus,Chinchilla Rabbit,Domestic Rabbit,Domestic Rabbits,Hare, Belgian,NZW Rabbit,Rabbit, Chinchilla,Rabbit, NZW,Rabbit, New Zealand,Rabbits, Chinchilla,Rabbits, Domestic,Rabbits, NZW,Rabbits, New Zealand,Zealand Rabbit, New,Zealand Rabbits, New,cuniculus, Oryctolagus
D002417	Cattle	Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.	Beef Cow,Bos grunniens,Bos indicus,Bos indicus Cattle,Bos taurus,Cow,Cow, Domestic,Dairy Cow,Holstein Cow,Indicine Cattle,Taurine Cattle,Taurus Cattle,Yak,Zebu,Beef Cows,Bos indicus Cattles,Cattle, Bos indicus,Cattle, Indicine,Cattle, Taurine,Cattle, Taurus,Cattles, Bos indicus,Cattles, Indicine,Cattles, Taurine,Cattles, Taurus,Cow, Beef,Cow, Dairy,Cow, Holstein,Cows,Dairy Cows,Domestic Cow,Domestic Cows,Indicine Cattles,Taurine Cattles,Taurus Cattles,Yaks,Zebus
D005069	Evaluation Studies as Topic	Works about studies that determine the effectiveness or value of processes, personnel, and equipment, or the material on conducting such studies.	Critique,Evaluation Indexes,Evaluation Methodology,Evaluation Report,Evaluation Research,Methodology, Evaluation,Pre-Post Tests,Qualitative Evaluation,Quantitative Evaluation,Theoretical Effectiveness,Use-Effectiveness,Critiques,Effectiveness, Theoretical,Evaluation Methodologies,Evaluation Reports,Evaluation, Qualitative,Evaluation, Quantitative,Evaluations, Qualitative,Evaluations, Quantitative,Indexes, Evaluation,Methodologies, Evaluation,Pre Post Tests,Pre-Post Test,Qualitative Evaluations,Quantitative Evaluations,Report, Evaluation,Reports, Evaluation,Research, Evaluation,Test, Pre-Post,Tests, Pre-Post,Use Effectiveness
D006440	Hemofiltration	Extracorporeal ULTRAFILTRATION technique without HEMODIALYSIS for treatment of fluid overload and electrolyte disturbances affecting renal, cardiac, or pulmonary function.	Arteriovenous Hemofiltration,Venovenous Hemofiltration,Arteriovenous Hemofiltrations,Hemofiltration, Arteriovenous,Hemofiltration, Venovenous,Hemofiltrations,Venovenous Hemofiltrations
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man