Treatment of inborn errors of urea synthesis: activation of alternative pathways of waste nitrogen synthesis and excretion
about
Brain imaging in urea cycle disordersSevere ornithine transcarbamylase deficiency. Two and a half years' survival with normal developmentNeurological implications of urea cycle disordersSwitch from Sodium Phenylbutyrate to Glycerol Phenylbutyrate Improved Metabolic Stability in an Adolescent with Ornithine Transcarbamylase DeficiencyAmmonia control in children ages 2 months through 5 years with urea cycle disorders: comparison of sodium phenylbutyrate and glycerol phenylbutyrate.Urinary phenylacetylglutamine (U-PAGN) concentration as biomarker for adherence in patients with urea cycle disorders (UCD) treated with glycerol phenylbutyrate.A longitudinal study of urea cycle disorders.A zebrafish model of hyperammonemia.Chronic vomiting in a case of citrullinaemia detected after treatment by total parenteral nutrition.Arginine, an indispensable amino acid for patients with inborn errors of urea synthesisArgininosuccinate lyase deficiency-argininosuccinic aciduria and beyond.Effects of sodium benzoate, a widely used food preservative, on glucose homeostasis and metabolic profiles in humansMinimal ureagenesis is necessary for survival in the murine model of hyperargininemia treated by AAV-based gene therapy.Antepartum ornithine transcarbamylase deficiency.Metabolic response to carnitine in methylmalonic aciduria. An effective strategy for elimination of propionyl groups.Successful treatment of severe carbamyl phosphate synthetase I deficiency.In vivo urea cycle flux distinguishes and correlates with phenotypic severity in disorders of the urea cycle.Identification and application of additional restriction fragment length polymorphisms at the human ornithine transcarbamylase locusGlycerol phenylbutyrate treatment in children with urea cycle disorders: pooled analysis of short and long-term ammonia control and outcomes.Response to treatment in hereditary metabolic disease: 1993 survey and 10-year comparison.Preclinical evaluation of a clinical candidate AAV8 vector for ornithine transcarbamylase (OTC) deficiency reveals functional enzyme from each persisting vector genome.N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia.Hepatocyte gene therapy in a large animal: a neonatal bovine model of citrullinemiaUrinary phenylacetylglutamine as dosing biomarker for patients with urea cycle disorders.Self-reported treatment-associated symptoms among patients with urea cycle disorders participating in glycerol phenylbutyrate clinical trials.Ammonia control in children with urea cycle disorders (UCDs); phase 2 comparison of sodium phenylbutyrate and glycerol phenylbutyrate.Improving long term outcomes in urea cycle disorders-report from the Urea Cycle Disorders ConsortiumDiffusion tensor imaging detects areas of abnormal white matter microstructure in patients with partial ornithine transcarbamylase deficiencyIntellectual, adaptive, and behavioral functioning in children with urea cycle disorders.Population pharmacokinetic modeling and dosing simulations of nitrogen-scavenging compounds: disposition of glycerol phenylbutyrate and sodium phenylbutyrate in adult and pediatric patients with urea cycle disordersStable isotopes in the diagnosis and treatment of inherited hyperammonemiaContinuous hemofiltration in the control of neonatal hyperammonemia: a 10-year experience.Astrocyte glutamine synthetase: importance in hyperammonemic syndromes and potential target for therapyAmmonia-lowering strategies for the treatment of hepatic encephalopathy.A current review of the diagnostic and treatment strategies of hepatic encephalopathy.Diagnosis and management of inborn errors of metabolismNutritional therapy for selected inborn errors of metabolism.Hyperammonemic Encephalopathy Associated With Fibrolamellar Hepatocellular Carcinoma: Case Report, Literature Review, and Proposed Treatment Algorithm.An update on the use of benzoate, phenylacetate and phenylbutyrate ammonia scavengers for interrogating and modifying liver nitrogen metabolism and its implications in urea cycle disorders and liver disease.Pharmaceutical excipients. Adverse effects associated with 'inactive' ingredients in drug products (Part II).
P2860
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P2860
Treatment of inborn errors of urea synthesis: activation of alternative pathways of waste nitrogen synthesis and excretion
description
1982 nî lūn-bûn
@nan
1982 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
1982 թվականի հունիսին հրատարակված գիտական հոդված
@hy
1982年の論文
@ja
1982年論文
@yue
1982年論文
@zh-hant
1982年論文
@zh-hk
1982年論文
@zh-mo
1982年論文
@zh-tw
1982年论文
@wuu
name
Treatment of inborn errors of ...... trogen synthesis and excretion
@ast
Treatment of inborn errors of ...... trogen synthesis and excretion
@en
Treatment of inborn errors of ...... trogen synthesis and excretion
@nl
type
label
Treatment of inborn errors of ...... trogen synthesis and excretion
@ast
Treatment of inborn errors of ...... trogen synthesis and excretion
@en
Treatment of inborn errors of ...... trogen synthesis and excretion
@nl
prefLabel
Treatment of inborn errors of ...... trogen synthesis and excretion
@ast
Treatment of inborn errors of ...... trogen synthesis and excretion
@en
Treatment of inborn errors of ...... trogen synthesis and excretion
@nl
P2093
P1476
Treatment of inborn errors of ...... trogen synthesis and excretion
@en
P2093
A M Brubakk
B K Burton
D Myerberg
I A Schafer
M L Batshaw
P304
P356
10.1056/NEJM198206103062303
P407
P577
1982-06-10T00:00:00Z