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Items: 1 to 20 of 97

1.

Alternative pathway therapy for urea cycle disorders: twenty years later.

Batshaw ML, MacArthur RB, Tuchman M.

J Pediatr. 2001 Jan;138(1 Suppl):S46-54; discussion S54-5. Review. Erratum in: J Pediatr 2002 Apr;140(4):490.

PMID:
11148549
2.

Survival after treatment with phenylacetate and benzoate for urea-cycle disorders.

Enns GM, Berry SA, Berry GT, Rhead WJ, Brusilow SW, Hamosh A.

N Engl J Med. 2007 May 31;356(22):2282-92.

3.

Alternative-pathway therapy for hyperammonemia.

Shih VE.

N Engl J Med. 2007 May 31;356(22):2321-2. No abstract available.

PMID:
17538092
4.

Effect of alternative pathway therapy on branched chain amino acid metabolism in urea cycle disorder patients.

Scaglia F, Carter S, O'Brien WE, Lee B.

Mol Genet Metab. 2004 Apr;81 Suppl 1:S79-85.

PMID:
15050979
5.

Argininosuccinate lyase deficiency.

Nagamani SC, Erez A, Lee B.

Genet Med. 2012 May;14(5):501-7. doi: 10.1038/gim.2011.1. Epub 2012 Jan 5. Review.

6.

Adult onset urea cycle disorder in a patient with presumed hepatic encephalopathy.

Atiq M, Holt AF, Safdar K, Weber F, Ravinuthala R, Jonas ME, Neff GW.

J Clin Gastroenterol. 2008 Feb;42(2):213-4. doi: 10.1097/01.mcg.0000225628.84168.25.

PMID:
18209596
7.

Long-term management of patients with urea cycle disorders.

Berry GT, Steiner RD.

J Pediatr. 2001 Jan;138(1 Suppl):S56-60; discussion S60-1.

PMID:
11148550
8.

Current strategies for the management of neonatal urea cycle disorders.

Summar M.

J Pediatr. 2001 Jan;138(1 Suppl):S30-9.

PMID:
11148547
9.

Treatment of urea cycle disorders.

Batshaw ML, Monahan PS.

Enzyme. 1987;38(1-4):242-50. Review.

PMID:
3326732
10.

Alternative pathway therapy for urea cycle disorders.

Feillet F, Leonard JV.

J Inherit Metab Dis. 1998;21 Suppl 1:101-11. Review.

PMID:
9686348
11.

Treatment of episodic hyperammonemia in children with inborn errors of urea synthesis.

Brusilow SW, Danney M, Waber LJ, Batshaw M, Burton B, Levitsky L, Roth K, McKeethren C, Ward J.

N Engl J Med. 1984 Jun 21;310(25):1630-4.

PMID:
6427608
12.

Ammonia toxicity and its prevention in inherited defects of the urea cycle.

Walker V.

Diabetes Obes Metab. 2009 Sep;11(9):823-35. doi: 10.1111/j.1463-1326.2009.01054.x. Epub 2009 Jun 16. Review.

PMID:
19531057
13.

Long-term treatment of girls with ornithine transcarbamylase deficiency.

Maestri NE, Brusilow SW, Clissold DB, Bassett SS.

N Engl J Med. 1996 Sep 19;335(12):855-9.

14.

Three cases of intravenous sodium benzoate and sodium phenylacetate toxicity occurring in the treatment of acute hyperammonaemia.

Praphanphoj V, Boyadjiev SA, Waber LJ, Brusilow SW, Geraghty MT.

J Inherit Metab Dis. 2000 Mar;23(2):129-36.

PMID:
10801054
15.

Treatment of urea cycle disorders.

Thoene JG.

J Pediatr. 1999 Mar;134(3):255-6. Review. No abstract available.

PMID:
10064655
16.

[Hyperammonemia type II as an example of urea cycle disorder].

Hawrot-Kawecka AM, Kawecki GP, DuĊ‚awa J.

Wiad Lek. 2006;59(7-8):512-5. Review. Polish.

PMID:
17209350
17.

Simultaneous LC-MS/MS determination of phenylbutyrate, phenylacetate benzoate and their corresponding metabolites phenylacetylglutamine and hippurate in blood and urine.

Laryea MD, Herebian D, Meissner T, Mayatepek E.

J Inherit Metab Dis. 2010 Dec;33 Suppl 3:S321-8. doi: 10.1007/s10545-010-9172-9. Epub 2010 Aug 7.

PMID:
20694517
18.

[Inherited hyperammonemia].

Bik-Multanowski M.

Przegl Lek. 1998;55(6):337-41. Review. Polish.

PMID:
9857711
19.

Urea cycle disorders.

Burton BK.

Clin Liver Dis. 2000 Nov;4(4):815-30, vi. Review.

PMID:
11232359
20.

Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children.

Nagasaka H, Yorifuji T, Kobayashi K, Takikawa H, Komatsu H, Inui A, Fujisawa T, Miida T, Tsukahara H, Takatani T, Hayashi H.

Mol Genet Metab. 2010 Jun;100(2):123-8. doi: 10.1016/j.ymgme.2010.02.008. Epub 2010 Feb 16.

PMID:
20219403
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