Page last updated: 2024-10-19

phenylacetic acid and Hyperammonemia

phenylacetic acid has been researched along with Hyperammonemia in 20 studies

phenylacetic acid : A monocarboxylic acid that is toluene in which one of the hydrogens of the methyl group has been replaced by a carboxy group.

Hyperammonemia: Elevated level of AMMONIA in the blood. It is a sign of defective CATABOLISM of AMINO ACIDS or ammonia to UREA.

Research Excerpts

ExcerptRelevanceReference
" Therefore, we used magnetic resonance spectroscopy (MRS) to monitor cerebral glutamine levels, the major reservoir of ammonia, in a gastric bypass patient with hyperammonemic coma undergoing therapy with N-carbamoyl glutamate and the ammonia-scavenging agents, sodium phenylacetate and sodium benzoate."7.85Brain MRS glutamine as a biomarker to guide therapy of hyperammonemic coma. ( Bellinger, A; Berry, GT; Cohen, DE; Frank, NY; Gershanik, EF; Goldberg, AD; Levy, BD; Lin, AP; Lynch, R; McFaline-Figueroa, JR; Merugumala, SK; O'Donnell-Luria, AH; Rohr, F; Samuels, MA; Simon, T; Tchekmedyian, V; Waisbren, SE, 2017)
"We report the results of a 25-year, open-label, uncontrolled study of sodium phenylacetate and sodium benzoate therapy (Ammonul, Ucyclyd Pharma) in 299 patients with urea-cycle disorders in whom there were 1181 episodes of acute hyperammonemia."5.12Survival after treatment with phenylacetate and benzoate for urea-cycle disorders. ( Berry, GT; Berry, SA; Brusilow, SW; Enns, GM; Hamosh, A; Rhead, WJ, 2007)
"The purpose of the study was to evaluate the influence of establishing a protocol for the use of combined sodium benzoate and sodium phenylacetate (SBSP) (Ammonul®) to treat acute hyperammonemia."3.91Influence of implementing a protocol for an intravenously administered ammonia scavenger on the management of acute hyperammonemia in a pediatric intensive care unit. ( Brossier, D; Goyer, I; Jouvet, P; Marquis, C; Mitchell, G; Ozanne, B; Ziani, L, 2019)
"Patients with neonatal urea cycle defects (UCDs) typically experience severe hyperammonemia during the first days of life, which results in serious neurological injury or death."3.88Prenatal treatment of ornithine transcarbamylase deficiency. ( Akula, VP; Alcorn, D; Benitz, WE; Bernstein, JA; Berquist, WE; Blumenfeld, YJ; Castillo, RO; Concepcion, W; Cowan, TM; Cox, KL; Cusmano, K; Enns, GM; Esquivel, CO; Hintz, SR; Homeyer, M; Hudgins, L; Hurwitz, M; Lyell, DJ; Palma, JP; Schelley, S; Summar, ML; Wilnai, Y, 2018)
" Therefore, we used magnetic resonance spectroscopy (MRS) to monitor cerebral glutamine levels, the major reservoir of ammonia, in a gastric bypass patient with hyperammonemic coma undergoing therapy with N-carbamoyl glutamate and the ammonia-scavenging agents, sodium phenylacetate and sodium benzoate."3.85Brain MRS glutamine as a biomarker to guide therapy of hyperammonemic coma. ( Bellinger, A; Berry, GT; Cohen, DE; Frank, NY; Gershanik, EF; Goldberg, AD; Levy, BD; Lin, AP; Lynch, R; McFaline-Figueroa, JR; Merugumala, SK; O'Donnell-Luria, AH; Rohr, F; Samuels, MA; Simon, T; Tchekmedyian, V; Waisbren, SE, 2017)
"Urea cycle enzyme deficiency (UCED) patients with hyperammonemia are treated with sodium benzoate (SB) and sodium phenylacetate (SPA) to induce alternative pathways of nitrogen excretion."3.85Saline is as effective as nitrogen scavengers for treatment of hyperammonemia. ( de Sain-van der Velden, MGM; Favier, RP; Fieten, H; Holwerda-Loof, NE; Mesu, SJ; Rothuizen, J; Spee, B; van der Ham, M; van Geijlswijk, IM; van Straten, G; Verhoeven-Duif, NM, 2017)
"An infant with a suspected inborn metabolism error was treated with a metabolic cocktail of intravenous sodium phenylacetate (NaPh) and sodium benzoate (NaBz) for hyperammonemia."3.74Phenylacetate and benzoate clearance in a hyperammonemic infant on sequential hemodialysis and hemofiltration. ( Barletta, GM; Bunchman, TE; Crumb, TL; Gardner, JJ; McBryde, KD; Winters, JW, 2007)
"Ammunol (sodium phenylacetate/sodium benzoate) is an intravenously administered, investigational drug used for the treatment of acute hyperammonemia in infants, children, and adults with urea cycle enzyme deficiencies."3.72Pharmacokinetics of sodium phenylacetate and sodium benzoate following intravenous administration as both a bolus and continuous infusion to healthy adult volunteers. ( Altincatal, A; MacArthur, RB; Tuchman, M, 2004)
" This involves the long-term use of oral sodium phenylbutyrate, arginine supplements, or both, depending on the specific enzyme deficiency, and treatment of acute hyperammonemic crises with intravenous sodium benzoate/sodium phenylacetate plus arginine."2.41Alternative pathway therapy for urea cycle disorders: twenty years later. ( Batshaw, ML; MacArthur, RB; Tuchman, M, 2001)
" Glycerol phenylbutyrate (GPB) is safe and effective in reducing ammonia levels in patients with UCD above 2 months of age."1.62Glycerol phenylbutyrate efficacy and safety from an open label study in pediatric patients under 2 months of age with urea cycle disorders. ( Ah Mew, N; Bannick, AA; Berry, SA; Canavan, C; Conway, RL; Diaz, GA; Hainline, B; Inbar-Feigenberg, M; Kok, T; Lichter-Konecki, U; Longo, N; McCandless, SE; Porter, MH; Schulze, A; Vescio, T; Zori, R, 2021)
"Hyperammonemia is a rare, often fatal complication after transplantation."1.43Hyperammonemia Syndrome After Lung Transplantation: A Single Center Experience. ( Bain, KB; Byers, DE; Chen, C; Hachem, RR; Iuppa, JA; Patterson, GA; Trulock, EP; Witt, CA; Yusen, RD, 2016)
"Available medications for treating urea cycle disorders include sodium benzoate (BA), sodium phenylacetate (PAA), and sodium phenylbutyrate (PBA) and are given to provide alternate routes for disposition of waste nitrogen excretion."1.36Simultaneous LC-MS/MS determination of phenylbutyrate, phenylacetate benzoate and their corresponding metabolites phenylacetylglutamine and hippurate in blood and urine. ( Herebian, D; Laryea, MD; Mayatepek, E; Meissner, T, 2010)
" The prepared sodium phenylacetate powder was orally administered to a 16-year-old patient with CTLN2 at a dosage of 12 g/d."1.31Successful treatment of severe hyperammonemia using sodium phenylacetate powder prepared in hospital pharmacy. ( Hashimoto, G; Honda, S; Horiuchi, R; Kaneko, H; Konno, Y; Nagai, K; Oshima, Y; Sekizuka, M; Tomomasa, T; Yamamoto, K, 2002)

Research

Studies (20)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's8 (40.00)29.6817
2010's9 (45.00)24.3611
2020's3 (15.00)2.80

Authors

AuthorsStudies
Endo, F2
Nakamura, K2
Sano, Y2
Dote, N2
Shimizu, K2
Koumura, E2
Raina, R1
Bedoyan, JK1
Lichter-Konecki, U2
Jouvet, P2
Picca, S1
Mew, NA1
Machado, MC1
Chakraborty, R1
Vemuganti, M1
Grewal, MK1
Bunchman, T1
Sethi, SK1
Krishnappa, V1
McCulloch, M1
Alhasan, K1
Bagga, A1
Basu, RK1
Schaefer, F1
Filler, G1
Warady, BA1
Longo, N1
Diaz, GA1
Schulze, A1
Inbar-Feigenberg, M1
Conway, RL1
Bannick, AA1
McCandless, SE1
Zori, R1
Hainline, B1
Ah Mew, N1
Canavan, C1
Vescio, T1
Kok, T1
Porter, MH1
Berry, SA2
O'Donnell-Luria, AH1
Lin, AP1
Merugumala, SK1
Rohr, F1
Waisbren, SE1
Lynch, R1
Tchekmedyian, V1
Goldberg, AD1
Bellinger, A1
McFaline-Figueroa, JR1
Simon, T1
Gershanik, EF1
Levy, BD1
Cohen, DE1
Samuels, MA1
Berry, GT2
Frank, NY1
van Straten, G1
de Sain-van der Velden, MGM1
van Geijlswijk, IM1
Favier, RP1
Mesu, SJ1
Holwerda-Loof, NE1
van der Ham, M1
Fieten, H1
Rothuizen, J1
Spee, B1
Verhoeven-Duif, NM1
Wilnai, Y1
Blumenfeld, YJ1
Cusmano, K1
Hintz, SR1
Alcorn, D1
Benitz, WE1
Berquist, WE1
Bernstein, JA1
Castillo, RO1
Concepcion, W1
Cowan, TM1
Cox, KL1
Lyell, DJ1
Esquivel, CO1
Homeyer, M1
Hudgins, L1
Hurwitz, M1
Palma, JP1
Schelley, S1
Akula, VP1
Summar, ML1
Enns, GM2
Brossier, D1
Goyer, I1
Ziani, L1
Marquis, C1
Mitchell, G1
Ozanne, B1
Chen, C1
Bain, KB1
Iuppa, JA1
Yusen, RD1
Byers, DE1
Patterson, GA1
Trulock, EP1
Hachem, RR1
Witt, CA1
Orton, DJ1
Gifford, JL1
Seiden-Long, I1
Khan, A1
de Koning, L1
Nagasaka, H1
Yorifuji, T1
Kobayashi, K1
Takikawa, H1
Komatsu, H1
Inui, A1
Fujisawa, T1
Miida, T1
Tsukahara, H1
Takatani, T1
Hayashi, H1
Laryea, MD1
Herebian, D1
Meissner, T1
Mayatepek, E1
Houston, B1
Reiss, KA1
Merlo, C1
Honda, S1
Yamamoto, K1
Sekizuka, M1
Oshima, Y1
Nagai, K1
Hashimoto, G1
Kaneko, H1
Tomomasa, T1
Konno, Y1
Horiuchi, R1
MacArthur, RB2
Altincatal, A1
Tuchman, M2
Bunchman, TE1
Barletta, GM1
Winters, JW1
Gardner, JJ1
Crumb, TL1
McBryde, KD1
Jalan, R1
Wright, G1
Davies, NA1
Hodges, SJ1
Rhead, WJ1
Brusilow, SW1
Hamosh, A1
Shih, VE1
Summar, M1
Batshaw, ML1

Clinical Trials (2)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
A Phase 2a Study to Evaluate the Safety and Tolerability of OCR-002 (Ornithine Phenylacetate) in the Treatment of Patients With Acute Liver Failure/Severe Acute Liver Injury[NCT01548690]Phase 247 participants (Actual)Interventional2012-06-30Completed
A Phase 2, Open-Label, Switch-Over, Dose-Escalation Study of the Safety and Tolerability of HPN-100 Compared to Buphenyl® (Sodium Phenylbutyrate) in Patients With Urea Cycle Disorders[NCT00551200]Phase 214 participants (Actual)Interventional2007-10-31Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Change in Ammonia

To evaluate the effect of OCR-002 on ammonia levels in patients with acute liver failure/severe acute liver injury (NCT01548690)
Timeframe: Baseline and 72 Hours

InterventionPercent Change (Mean)
Maximum Dose Level 3.33 g/24h41.2
Maximum Dose Level 6.65 g/24h16.6
Maximum Dose Level 10 g/24h41.8
Maximum Dose Level 20g/24h38.4

Measurement of OCR-002 Plasma Concentration

To evaluate the steady state pharmacokinetic and pharmacodynamic profile of OCR-002 in patients with impaired and intact renal function using urinary phenylacetylglutamine (PAGN) as a surrogate marker (NCT01548690)
Timeframe: 24 Hours after last infusion

Interventionmicrograms per millileter (Mean)
Maximum Dose Level 3.33 g/24h65.6
Maximum Dose Level 6.65 g/24h32.2
Maximum Dose Level 10 g/24h33.4
Maximum Dose Level 20g/24h104.9

Neurological Function Measured by the Orientation Log (O-log)

The orientation log focuses on orientation to place, time, and circumstance. There are 10 items on the orientation log, which are scored 0-3. A spontaneous correct response is awarded 3 points. A spontaneous response that is lacking or incorrect, but a correct response is provided following a logical cue is awarded 2 points. A score of 1 is given if spontaneous and cued responses are lacking or incorrect, but a correct response is provided in a recognition format. A score of 0 is given if the spontaneous, cued, or recognition format does not generate a correct answer. Scores from the 10 items are summed and the final score ranges from 0 to 30. (NCT01548690)
Timeframe: 30 Days

Interventionunits on a scale (Mean)
Maximum Dose Level 3.33 g/24h23.8
Maximum Dose Level 6.65 g/24h24.0
Maximum Dose Level 10 g/24h24.0
Maximum Dose Level 20g/24h24.0

Neurological Function Measured by the West Haven Criteria (WHC) for Hepatic Encephalopathy

The West Haven Criteria (WHC) for Hepatic Encephalopathy measures the severity of encephalopathy and patient's level of consciousness. The scale ranges from 0 to 4; a minimum score of 0 represents a better outcome, and a maximum total score of 4 represents a worse outcome. A score of 0 corresponds to normal consciousness and behavior and normal neurological examination. A score of 1 corresponds to mild lack of awareness, shortened attention span, and impaired addition or subtraction; mild asterixis or tremor. A score of 2 corresponds to lethargy, disorientated or inappropriate behavior, obvious asterixis; slurred speech. A score of 3 corresponds to somnolent but arousable, gross disorientation or bizarre behavior, muscle rigidity and clonus; hyperreflexia. A score of 4 corresponds to coma and decerebrate posturing. (NCT01548690)
Timeframe: 120 hours from start of infusion

Interventionunits on a scale (Mean)
Maximum Dose Level 3.33 g/24h2.4
Maximum Dose Level 6.65 g/24h3.2
Maximum Dose Level 10 g/24h1.6
Maximum Dose Level 20g/24h1.8

Number of Participants That do Not Tolerate the Administered Dose and Had Grade 3 or 4 Treatment Emergent Adverse Events as a Measure of Safety and Tolerability

To evaluate the safety and tolerability of OCR-002 in patients with acute liver failure/severe acute liver injury (NCT01548690)
Timeframe: 30 Days

InterventionParticipants (Count of Participants)
Maximum Dose Level 3.33 g/24h0
Maximum Dose Level 6.65 g/24h0
Maximum Dose Level 10 g/24h0
Maximum Dose Level 20g/24h0

Number of Subjects Experienced Adverse Events

(NCT00551200)
Timeframe: during the period on 100% Buphenyl (up to 4 weeks) or HPN-100 (up to 10 weeks)

Interventionparticipants (Number)
Buphenyl7
HPN-1005

Number of Subjects Experienced Serious Adverse Events

(NCT00551200)
Timeframe: during the period subjects on 100% Buphenyl (up to 4 weeks) or HPN-100 (up to 10 weeks)

Interventionparticipants (Number)
Buphenyl1
HPN-1000

Drug Preference for HPN-100 or Buphenyl® (as Assessed by Global Preference Question)

(NCT00551200)
Timeframe: End of Study

Interventionparticipants (Number)
prefer Buphenylprefer HPN-100
Buphenyl to HPN-10019

Pharmacokinetics (Plasma and Urine PK Parameters of Study Drugs and Their Metabolites)

measured AUC0-24 (Area under the curve from time 0 (pre-dose) to 24 hours) for each metabolite in plasma. Data were collected at 30 minutes and 1, 2, 4, 5, 6, 8, 10, 12, and 24 hours post-first dose. (NCT00551200)
Timeframe: At steady state (1 week) on each medication (Buphenyl® alone, HPN-100 alone)

,
Interventionμg*h/mL (Mean)
AUC0-24 PBA (phenylbutyrate) in plasmaAUC0-24 PAA (phenylacetate) in plasmaAUC0-24 PAGN (phenylacetylglutamine) in plasma
HPN-100 Steady State5405751098
NaPBA Steady State7405961133

Venous Ammonia Levels at the Peak and Mean TNUAC Time-normalized Area Under the Curve)

Data were collected at pre-first dose and at 30 minutes and 1, 2, 4, 5, 6, 8, 10, 12, and 24 hours post first dose. (NCT00551200)
Timeframe: At steady state (1 week) on each medication (Buphenyl® alone, HPN-100 alone), and at steady state (1 week) after each dose escalation

,
Interventionμmol/L (Mean)
in peakin TNAUC (time-normalized area under the curve)
HPN-100 Steady State56.326.5
NaPBA Steady State79.138.4

Reviews

2 reviews available for phenylacetic acid and Hyperammonemia

ArticleYear
Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement therapy.
    Nature reviews. Nephrology, 2020, Volume: 16, Issue:8

    Topics: Arginine; Carnitine; Child; Child, Preschool; Continuous Renal Replacement Therapy; Delphi Technique

2020
Alternative pathway therapy for urea cycle disorders: twenty years later.
    The Journal of pediatrics, 2001, Volume: 138, Issue:1 Suppl

    Topics: Amino Acid Metabolism, Inborn Errors; Animals; Arginine; Humans; Hyperammonemia; Phenylacetates; Phe

2001

Trials

1 trial available for phenylacetic acid and Hyperammonemia

ArticleYear
Survival after treatment with phenylacetate and benzoate for urea-cycle disorders.
    The New England journal of medicine, 2007, May-31, Volume: 356, Issue:22

    Topics: Adolescent; Adult; Age Factors; Age of Onset; Amino Acid Metabolism, Inborn Errors; Ammonia; Carbamo

2007

Other Studies

17 other studies available for phenylacetic acid and Hyperammonemia

ArticleYear
Pharmacokinetics, safety, and tolerability of sodium phenylacetate and sodium benzoate in healthy Japanese volunteers: A phase I, single-center, open-label study.
    Drug metabolism and pharmacokinetics, 2023, Volume: 48

    Topics: Adult; Benzoates; Child; East Asian People; Healthy Volunteers; Humans; Hyperammonemia; Infant; Phen

2023
Pharmacokinetics, safety, and tolerability of sodium phenylacetate and sodium benzoate in healthy Japanese volunteers: A phase I, single-center, open-label study.
    Drug metabolism and pharmacokinetics, 2023, Volume: 48

    Topics: Adult; Benzoates; Child; East Asian People; Healthy Volunteers; Humans; Hyperammonemia; Infant; Phen

2023
Pharmacokinetics, safety, and tolerability of sodium phenylacetate and sodium benzoate in healthy Japanese volunteers: A phase I, single-center, open-label study.
    Drug metabolism and pharmacokinetics, 2023, Volume: 48

    Topics: Adult; Benzoates; Child; East Asian People; Healthy Volunteers; Humans; Hyperammonemia; Infant; Phen

2023
Pharmacokinetics, safety, and tolerability of sodium phenylacetate and sodium benzoate in healthy Japanese volunteers: A phase I, single-center, open-label study.
    Drug metabolism and pharmacokinetics, 2023, Volume: 48

    Topics: Adult; Benzoates; Child; East Asian People; Healthy Volunteers; Humans; Hyperammonemia; Infant; Phen

2023
Glycerol phenylbutyrate efficacy and safety from an open label study in pediatric patients under 2 months of age with urea cycle disorders.
    Molecular genetics and metabolism, 2021, Volume: 132, Issue:1

    Topics: Age of Onset; Ammonia; Child, Preschool; Female; Glycerol; Humans; Hyperammonemia; Infant; Infant, N

2021
Brain MRS glutamine as a biomarker to guide therapy of hyperammonemic coma.
    Molecular genetics and metabolism, 2017, Volume: 121, Issue:1

    Topics: Brain; Coma; Female; Gastric Bypass; Glutamates; Glutamine; Humans; Hyperammonemia; Magnetic Resonan

2017
Saline is as effective as nitrogen scavengers for treatment of hyperammonemia.
    Scientific reports, 2017, 10-13, Volume: 7, Issue:1

    Topics: Animals; Dogs; Female; Hyperammonemia; Male; Nitrogen; Phenylacetates; Random Allocation; Saline Wat

2017
Prenatal treatment of ornithine transcarbamylase deficiency.
    Molecular genetics and metabolism, 2018, Volume: 123, Issue:3

    Topics: Ammonia; Drug Combinations; Female; Glutamine; Humans; Hyperammonemia; Infant, Newborn; Male; Mutati

2018
Influence of implementing a protocol for an intravenously administered ammonia scavenger on the management of acute hyperammonemia in a pediatric intensive care unit.
    Journal of inherited metabolic disease, 2019, Volume: 42, Issue:1

    Topics: Acute Disease; Ammonia; Canada; Child; Child, Preschool; Female; Humans; Hyperammonemia; Infant; Inf

2019
Hyperammonemia Syndrome After Lung Transplantation: A Single Center Experience.
    Transplantation, 2016, Volume: 100, Issue:3

    Topics: Aged; Ammonia; Arginine; Biomarkers; Carnitine; Combined Modality Therapy; Decontamination; Female;

2016
Critically High Plasma Ammonia in an Adolescent Girl.
    Clinical chemistry, 2016, Volume: 62, Issue:12

    Topics: Ammonia; Arginine; Child; Clinical Chemistry Tests; Diagnostic Errors; Fatigue; Female; Fever; Heada

2016
Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children.
    Molecular genetics and metabolism, 2010, Volume: 100, Issue:2

    Topics: Adenosine Triphosphatases; Adolescent; Arginine; ATP Binding Cassette Transporter, Subfamily B, Memb

2010
Simultaneous LC-MS/MS determination of phenylbutyrate, phenylacetate benzoate and their corresponding metabolites phenylacetylglutamine and hippurate in blood and urine.
    Journal of inherited metabolic disease, 2010, Volume: 33 Suppl 3

    Topics: Benzoates; Biomarkers; Biotransformation; Calibration; Chromatography, Reverse-Phase; Glutamine; Hip

2010
Healthy, but comatose.
    The American journal of medicine, 2011, Volume: 124, Issue:4

    Topics: Adult; Brain Edema; Carbohydrates; Enteral Nutrition; Glucose; Humans; Hyperammonemia; Insulin; Male

2011
Successful treatment of severe hyperammonemia using sodium phenylacetate powder prepared in hospital pharmacy.
    Biological & pharmaceutical bulletin, 2002, Volume: 25, Issue:9

    Topics: Adolescent; Chemistry, Pharmaceutical; Humans; Hyperammonemia; Male; Pharmacy Service, Hospital; Phe

2002
Pharmacokinetics of sodium phenylacetate and sodium benzoate following intravenous administration as both a bolus and continuous infusion to healthy adult volunteers.
    Molecular genetics and metabolism, 2004, Volume: 81 Suppl 1

    Topics: Administration, Oral; Adult; Dose-Response Relationship, Drug; Drug Administration Schedule; Humans;

2004
Phenylacetate and benzoate clearance in a hyperammonemic infant on sequential hemodialysis and hemofiltration.
    Pediatric nephrology (Berlin, Germany), 2007, Volume: 22, Issue:7

    Topics: Ammonia; Hemofiltration; Humans; Hyperammonemia; Infant, Newborn; Phenylacetates; Renal Dialysis; So

2007
L-Ornithine phenylacetate (OP): a novel treatment for hyperammonemia and hepatic encephalopathy.
    Medical hypotheses, 2007, Volume: 69, Issue:5

    Topics: Ammonia; Drug Therapy, Combination; Hepatic Encephalopathy; Humans; Hyperammonemia; Liver; Models, B

2007
Alternative-pathway therapy for hyperammonemia.
    The New England journal of medicine, 2007, May-31, Volume: 356, Issue:22

    Topics: Amino Acid Metabolism, Inborn Errors; Humans; Hyperammonemia; Phenylacetates; Sodium Benzoate; Urea

2007
Current strategies for the management of neonatal urea cycle disorders.
    The Journal of pediatrics, 2001, Volume: 138, Issue:1 Suppl

    Topics: Algorithms; Amino Acid Metabolism, Inborn Errors; Antimetabolites, Antineoplastic; Arginine; Dialysi

2001