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Literature DB >> 18034368

Nutritional management of patients with urea cycle disorders.

Abstract

The nutritional management of patients with urea cycle disorders (UCDs) involves restriction of dietary protein along with provision of adequate protein-free energy, essential amino acid supplements, and vitamins and minerals in combination with nitrogen-scavenging drugs. The present paper discusses nutrition therapy for a range of circumstances: during an acute hyperammonaemic episode and at hospital discharge; before, during, and after surgery; and for lifelong chronic management of UCDs.

Entities: Chemical Disease Species

Mesh：

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Year: 2007 PMID： 18034368 DOI： 10.1007/s10545-007-0718-4

Source DB: PubMed Journal: J Inherit Metab Dis ISSN： 0141-8955 Impact factor: 4.982

22 in total

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Journal: J Pediatr Date: 2001-01 Impact factor: 4.406

Review 2. Protein homeostasis in chronic hemodialysis patients.

Authors: Lara B Pupim; Paul J Flakoll; T Alp Ikizler
Journal: Curr Opin Clin Nutr Metab Care Date: 2004-01 Impact factor: 4.294

Review 3. Ketone bodies: a review of physiology, pathophysiology and application of monitoring to diabetes.

Authors: L Laffel
Journal: Diabetes Metab Res Rev Date: 1999 Nov-Dec Impact factor: 4.876

Review 4. Regulation of skeletal muscle protein metabolism in catabolic states.

Authors: Robert R Wolfe
Journal: Curr Opin Clin Nutr Metab Care Date: 2005-01 Impact factor: 4.294

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Authors: P Nicolaides; D Liebsch; N Dale; J Leonard; R Surtees
Journal: Arch Dis Child Date: 2002-01 Impact factor: 3.791

6. Hemodialysis stimulates muscle and whole body protein loss and alters substrate oxidation.

Authors: T Alp Ikizler; Lara B Pupim; John R Brouillette; Deanna K Levenhagen; Kali Farmer; Raymond M Hakim; Paul J Flakoll
Journal: Am J Physiol Endocrinol Metab Date: 2002-01 Impact factor: 4.310

7. Metabolic response to injury and illness: estimation of energy and protein needs from indirect calorimetry and nitrogen balance.

Authors: C L Long; N Schaffel; J W Geiger; W R Schiller; W S Blakemore
Journal: JPEN J Parenter Enteral Nutr Date: 1979 Nov-Dec Impact factor: 4.016

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

Authors: Fernando Scaglia; Susan Carter; William E O'Brien; Brendan Lee
Journal: Mol Genet Metab Date: 2004-04 Impact factor: 4.797

Review 9. Mechanisms of ammonia-induced astrocyte swelling.

Authors: M D Norenberg; K V Rama Rao; A R Jayakumar
Journal: Metab Brain Dis Date: 2005-12 Impact factor: 3.655

10. Newborn screening: toward a uniform screening panel and system.

Authors:
Journal: Genet Med Date: 2006-05 Impact factor: 8.822

19 in total

Review 1. The liver is a metabolic and immunologic organ: A reconsideration of metabolic decompensation due to infection in inborn errors of metabolism (IEM).

Authors: Tatyana N Tarasenko; Peter J McGuire
Journal: Mol Genet Metab Date: 2017-06-24 Impact factor: 4.797

2. Nonhepatic hyperammonemic encephalopathy due to undiagnosed urea cycle disorder.

Authors: Tashfeen Mahmood; Kenneth Nugent
Journal: Proc (Bayl Univ Med Cent) Date: 2015-07

3. Engineering the gut microbiota to treat hyperammonemia.

Authors: Ting-Chin David Shen; Lindsey Albenberg; Kyle Bittinger; Christel Chehoud; Ying-Yu Chen; Colleen A Judge; Lillian Chau; Josephine Ni; Michael Sheng; Andrew Lin; Benjamin J Wilkins; Elizabeth L Buza; James D Lewis; Yevgeny Daikhin; Ilana Nissim; Marc Yudkoff; Frederic D Bushman; Gary D Wu
Journal: J Clin Invest Date: 2015-06-22 Impact factor: 14.808

Review 4. Hyperammonemia in review: pathophysiology, diagnosis, and treatment.

Authors: Ari Auron; Patrick D Brophy
Journal: Pediatr Nephrol Date: 2011-03-23 Impact factor: 3.714

Review 5. [Consensus on diagnosis and treatment of ornithine trans-carbamylase deficiency].

Authors:
Journal: Zhejiang Da Xue Xue Bao Yi Xue Ban Date: 2020-10-25

6. Nervonic Acid Attenuates Accumulation of Very Long-Chain Fatty Acids and is a Potential Therapy for Adrenoleukodystrophy.

Authors: Marcia R Terluk; Julianne Tieu; Siddhee A Sahasrabudhe; Ann Moser; Paul A Watkins; Gerald V Raymond; Reena V Kartha
Journal: Neurotherapeutics Date: 2022-04-04 Impact factor: 6.088