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cystinuria/аргинин
Врската е зачувана во таблата со исечоци
Страница 1 од 105 резултати
Cystinuria phenotyping by oral lysine and arginine loading.
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BACKGROUND
Cystinuria is an inherited disorder of cystine and dibasic amino acids transport that results in urolithiasis because of poor cystine solubility. Three cystinuria phenotypes, differentiated according to urinary amino acid excretion in obligate heterozygotes, were regarded as allelic
[Urinary amino acid pattern before and after intravenous arginine (a comparison with cystinuria)].
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13 patients had during their work up for growth retardation an intravenous arginine load. The urinary amino acid pattern before and after arginine administration was checked in comparison to 10 control patients. There was no significant difference between the individual groups. After the arginine
Jejunal and ileal absorption of dibasic amino acids and an arginine-containing dipeptide in cystinuria.
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Ileal transport of dibasic amino acids has not previously been studied in the intestine of healthy volunteers or cystinuric patients. Experiments have therefore been designed to compare ileal and jejunal absorption of lysine and arginine both in normal subjects and cystinuric patients. In addition,
Intravenous loading with arginine-hydrochloride and ornithine-aspartate in siblings of two families, presenting a familial neurological syndrome associated with cystinuria.
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1. Arginine-hydrochloride and ornithine-aspartate solutions have been infused intravenously to children of two families. Three children of the WOL. family are affected with hyperargininemia and hyperammonemia, due to a lack of arginase. They present a secondary cystine-lysinuria. The three WIL.
Renal transport of lysine and arginine in cystinuria.
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To study the defective transport mechanism of cystinuria, renal tubular reabsorption of lysine or arginine in normal and cystinuria subjects was investigated by increasing the filtered load employing intravenous amino acid infusion. In the normal group the amino acid reabsorption rose with increases
Urinary excretion of total cystine and the dibasic amino acids arginine, lysine and ornithine in relation to genetic findings in patients with cystinuria treated with sulfhydryl compounds.
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Advances in molecular genetics have brought a deeper understanding of cystinuria. This autosomal recessive disease, which is caused by a defective tubular reabsorption of cystine and the three dibasic amino acids arginine, lysine and ornithine, results in a lifelong risk of renal stone formation
A highly selective and sensitive fluorescent sensor based on Tb3+-functionalized MOFs to determine arginine in urine: a potential application for the diagnosis of cystinuria.
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A unique metal-organic framework with the formula [Cd4(H2L)2(L)·H2O]·3H2O (H4L = 5,5'-(1H-1,2,4-triazole-3,5-diyl)diisophthalic acid) was successfully constructed under solvothermal conditions. The frameworks with multiple free Lewis
Intestinal absorption of an arginine-containing peptide in cystinuria.
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Separate tolerance tests involving oral intake of the dipeptide, L-arginyl-L-aspartate, and of a corresponding free amino acid mixture, were carried out in a single type 2 cystinuric patient. Absorption of aspartate was within normal limits, whilst that of arginine was normal after the peptide but
The effect of intravenous lysine on the renal clearances of cystine, arginine and ornithine in normal subjects, in patients with cystinuria and Fanconi syndrome and in their relatives.
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[Reversible functional disorders of the renal tubules after the administration of arginine (functional cystinuria)].
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[Cystinuria update: clinical, biochemical and genetic aspects].
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Cystinuria is an autosomal recessive disorder with an estimated incidence of 1 case in 7000 live births, that results in elevated urinary excretion of cystine and dibasic aminoacids: ornithine, lysine and arginine. Discussed by Sir Archibald Edward Garrod, in 1908, as one of the four first known
Diagnosis and follow-up of cystinuria: use of proton magnetic resonance spectroscopy.
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Proton Nuclear Magnetic Resonance (NMR) Spectroscopy of urine (as well as of other biological fluids) is a very powerful technique enabling multi-component analysis useful in both diagnosis and follow-up of a wide range of inherited metabolic diseases. Among these pathologies, cystinuria is
Renal handling of dibasic amino acids and cystine in cystinuria.
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1. The effect of intravenous infusion of L-lysine and L-arginine on the tubular reabsorption of dibasic amino acids and cystine was studied in normal individuals and in homozygous and heterozygous subjects with cystinuria. 2. The control subjects reabsorbed almost all filtered lysine and arginine
Experimental cystinuria: the cycloleucine model. I. Amino acid interactions in renal and intestinal epithelia.
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The injection of cycloleucine (1-aminocyclopentanecarboxylic acid (ACPC) into rats produces a hyperexcretion of dibasic amino acids and cystine, an aberration resembling cystinuria. This may constitute a model of experimental cystinuria, and the transport of amino acids involved in this disease was
[Cystinuria: diagnosis and therapeutic approach].
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Cystinuria is an aminoaciduria due to the impairment of transport of cystine and dibasic amino acids (arginine, ornithine, and lysine) in the apical membrane of the intestinal epithelium and proximal renal tubule. The result is an absence of cystine reabsorption in the renal tubule producing an
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