Elevated lactate in Mauriac syndrome: still a mystery.

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State: Public
Version: author
License: CC BY 4.0
Serval ID
serval:BIB_8EAC9E849686
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Elevated lactate in Mauriac syndrome: still a mystery.
Journal
BMC endocrine disorders
Author(s)
Touilloux B., Lu H., Campos-Xavier B., Superti-Furga A., Hauschild M., Bouthors T., Tran C.
ISSN
1472-6823 (Electronic)
ISSN-L
1472-6823
Publication state
Published
Issued date
21/08/2021
Peer-reviewed
Oui
Volume
21
Number
1
Pages
172
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
The Mauriac syndrome was described in 1930 as a peculiar combination of poorly controlled diabetes mellitus type 1, stunted growth and glycogenic hepatopathy. More recently, lactic acidosis was recognized as an additional feature, often induced by insulin treatment.
A 17-year old girl known for diabetes type 1A and Mauriac syndrome was admitted to the emergency room with hyperglycemia of > 41 mmol/l without ketoacidosis. Under a standard insulin regimen, hyperglycemia was rapidly corrected but marked hyperlactatemia occurred.
The mechanism of impaired glucose utilization and lactate elevation independent of ketoacidosis in Mauriac syndrome is intriguing. The rarity of Mauriac syndrome and its resemblance to glycogen storage diseases suggest the presence of a specific metabolic or genetic predisposition that remains to be identified.
Keywords
Elevated lactate, Glycogenic hepatopathy, Mauriac syndrome
Pubmed
Web of science
Open Access
Yes
Create date
03/09/2021 17:03
Last modification date
27/10/2021 5:40
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