Treatment of HMG-CoA Lyase Deficiency—Longitudinal Data on Clinical and Nutritional Management of 10 Australian Cases

Treatment of HMG-CoA Lyase Deficiency—Longitudinal Data on Clinical and Nutritional Management of 10 Australian Cases

3-Hydroxy-3-Methylglutaryl-CoA Lyase (HMGCL) deficiency can be a very severe disorder that typically presents with acute metabolic decompensation with features of hypoketotic hypoglycemia, hyperammonemia, and metabolic acidosis. A retrospective chart and literature review of Australian patients over...

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Bibliographic Details
Main Authors: Susan Thompson, Ashley Hertzog, Arthavan Selvanathan, Kiera Batten, Katherine Lewis, Janelle Nisbet, Ashleigh Mitchell, Troy Dalkeith, Kate Billmore, Francesca Moore, Adviye Ayper Tolun, Beena Devanapalli, Drago Bratkovic, Cathie Hilditch, Yusof Rahman, Michel Tchan, Kaustuv Bhattacharya
Format: Article
Language:English
Published: MDPI AG 2023-01-01
Series:Nutrients
Subjects:
HMG Co A Lyase
fat oxidation
leucine oxidation
low-protein diet
low-fat diet
ketones
Online Access:https://www.mdpi.com/2072-6643/15/3/531

Internet

https://www.mdpi.com/2072-6643/15/3/531

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