argininosuccinic aciduria/arginine

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A randomized controlled trial to evaluate the effects of high-dose versus low-dose of arginine therapy on hepatic function tests in argininosuccinic aciduria.

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Přihlášení Registrace

OBJECTIVE To compare the effects of combinatorial therapy with low-dose arginine and a nitrogen scavenging agent (sodium phenylbutyrate) vs. monotherapy with high-dose arginine on liver function tests in patients with argininosuccinic aciduria (ASA). METHODS Twelve patients with ASA were enrolled in

Arginine therapy of argininosuccinase deficiency.

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Přihlášení Registrace

Argininosuccinic acid (A.S.A.) contains the two waste nitrogen atoms later excreted in urea in healthy people, and it has a renal clearance similar to the glomerular filtration-rate. Therefore, argininosuccinic acid might provide a vehicle for the excretion of waste nigrogen in patients with

Argininosuccinic aciduria: long-term treatment with arginine.

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Přihlášení Registrace

The presentation and 2 year treatment of a patient with argininosuccinic aciduria is reported. Erythrocyte argininosuccinate lyase activity was less than 2% of normal. Long-term management included protein restriction and arginine dietary supplementation. The child experienced three episodes of

Arginine as an essential amino acid in children with argininosuccinase deficiency.

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Přihlášení Registrace

Long-term outcome of patients with argininosuccinate lyase deficiency diagnosed by newborn screening in Austria.

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Přihlášení Registrace

Twenty-three patients with late onset argininosuccinate lyase deficiency (ASLD) were identified during a 27-year period of newborn screening in Austria (1:95,600, 95% CI=1:68,036-1:162,531). One additional patient was identified outside the newborn screening with neonatal hyperammonemia. Long-term

Experience with the treatment of argininosuccinic aciduria during pregnancy.

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Přihlášení Registrace

We present the details of the management and the outcome of a pregnancy of a woman affected with argininosuccinic aciduria. Management with a closely monitored, protein-restricted diet, supplemented with L-arginine, resulted in the birth of a healthy infant boy and an uneventful perinatal course for

Argininosuccinic acid synthetase deficiency in a hamster cell line and its complementation of argininosuccinic aciduria human fibroblasts.

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Přihlášení Registrace

Unlike normal human cells, cultured fibroblasts from patients with argininosuccinic aciduria cannot synthesize arginine from citrulline because they have a deficiency of argininosuccinic acid lyase (ASL). We have found that V79, a Chinese hamster cell line, cannot grow on citrulline. Although these

A mouse model of argininosuccinic aciduria: biochemical characterization.

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Přihlášení Registrace

Argininosuccinate lyase (AL) has several roles in intermediary metabolism. It is an essential component of the urea cycle, providing a pathway for the disposal of excess nitrogen in mammals. AL links the urea cycle to the tricarboxylic acid (TCA) cycle by generating fumarate. Finally, AL is required

One Year Experience of Pheburane(®) (Sodium Phenylbutyrate) Treatment in a Patient with Argininosuccinate Lyase Deficiency.

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Přihlášení Registrace

Argininosuccinate lyase deficiency (ASLD) is a urea cycle disorder (UCD) treated with dietary adjustment and nitrogen scavenging agents. "Pheburane(®)" is a new tasteless and odour-free formulation of sodium phenylbutyrate, indicated in the treatment of UCD.A male patient diagnosed with ASLD was put

Optimizing therapy for argininosuccinic aciduria.

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Přihlášení Registrace

Argininosuccinic aciduria (ASA) is a urea cycle disorder with a complex phenotype. In spite of a lower risk for recurrent hyperammonemic episodes as compared to the proximal disorders of ureagenesis, subjects with ASA are at risk for long-term complications including, poor neurocognitive outcome,

Amino acid and enzyme studies of brain and other tissues in an infant with argininosuccinic aciduria.

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Přihlášení Registrace

Amino acid contents were measured in four regions of autopsied brain from an infant who presented in coma at the age of 7 weeks and died with argininosuccinic aciduria. Argininosuccinic acid lyase activity was greatly reduced in liver, kidney and cultured skin fibroblasts; incorporation of

Argininosuccinic aciduria: clinical and biochemical phenotype findings in Malaysian children.

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Přihlášení Registrace

Argininosuccinic aciduria is an inborn error of the urea cycle caused by deficiency of argininosuccinate lyase (ASL). ASL-deficient patients present with progressive intoxication due to accumulation of ammonia in the body. Early diagnosis and treatment of hyperammonemia are necessary to improve

Systemic hypertension in two patients with ASL deficiency: a result of nitric oxide deficiency?

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Přihlášení Registrace

Argininosuccinic aciduria (ASA) is an inborn error of ureagenesis which if untreated leads to hyperammonemia, accumulation of argininosuccinic acid and arginine depletion. The presence of high blood pressure in patients with ASA has been reported so far as transient in one newborn. We describe the

Argininosuccinate neurotoxicity and prevention by creatine in argininosuccinate lyase deficiency: An in vitro study in rat three-dimensional organotypic brain cell cultures.

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Přihlášení Registrace

The urea cycle disorder (UCD) argininosuccinate lyase (ASL) deficiency, caused by a defective ASL enzyme, exhibits a wide range of phenotypes, from life-threatening neonatal hyperammonemia to asymptomatic patients, with only the biochemical marker argininosuccinic acid (ASA) elevated in body fluids.

Argininosuccinic aciduria. A developmental and biochemical case study.

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Přihlášení Registrace

An infant with argininosuccinic aciduria was detected through the routine newborn screening program for inborn metabolic diseases and has been followed for over 7 years. Treatment consisting of restricted protein intake and arginine base supplementation was initiated at the age of 8 months and was

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