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Prevention of complications in glycogen storage disease type Ia with optimization of metabolic control
Author(s) -
Dambska M,
Labrador EB,
Kuo CL,
Weinstein DA
Publication year - 2017
Publication title -
pediatric diabetes
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.678
H-Index - 75
eISSN - 1399-5448
pISSN - 1399-543X
DOI - 10.1111/pedi.12540
Subject(s) - medicine , glycogen storage disease type i , hypoglycemia , metabolic control analysis , glycogen storage disease , glycogen , disease , lactic acidosis , type 2 diabetes , metabolic acidosis , diabetes mellitus , intensive care medicine , pediatrics , endocrinology
Prior to 1971, type Ia glycogen storage disease was marked by life‐threatening hypoglycemia, lactic acidosis, severe failure to thrive, and developmental delay. With the introduction of continuous feeds in the 1970s and cornstarch in the 1980s, the prognosis improved, but complications almost universally developed. Changes in the management of type Ia glycogen storage disease have resulted in improved metabolic control, and this manuscript reviews the increasing evidence that complications can be delayed or prevented with optimal metabolic control as previously was seen in diabetes.