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A novel starch for the treatment of glycogen storage diseases
Author(s) -
Bhattacharya K.,
Orton R. C.,
Qi X.,
Mundy H.,
Morley D. W.,
Champion M. P.,
Eaton S.,
Tester R. F.,
Lee P. J.
Publication year - 2007
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-007-0479-0
Subject(s) - glycogen , glycogen storage disease , human genetics , starch , medicine , biochemistry , chemistry , biology , endocrinology , gene
Summary Objective: To determine whether a new starch offers better short‐term metabolic control than uncooked cornstarch in patients with glycogen storage diseases (GSDs). Study design: A short‐term double‐blind cross‐over pilot study comparing uncooked physically modified cornstarch (WMHM20) with uncooked cornstarch in patients with GSD types Ia, Ib and III. Twenty‐one patients (ages 3–47, 9 female) were given 2 g/kg cornstarch or WMHM20 mixed in water. Blood glucose, lactate and insulin, and breath hydrogen and 13 CO 2 enrichment were measured, at baseline and after each load. The hourly biochemical evaluations terminated when blood glucose was ≤3.0 mmol/L, when the study period had lasted 10 h or when the patient wished to end the test. The alternative starch was administered under similar trial conditions a median of 10 days later. Results: The median starch load duration was 9 h for WMHM20 versus 7 h for cornstarch. Glucose decreased more slowly ( p =0.05) and lactate was suppressed faster ( p =0.17) for WMHM20 compared with cornstarch. Peak hydrogen excretion was increased ( p =0.05) when cornstarch was taken. Conclusion: These data indicate longer duration of euglycaemia and better short‐term metabolic control in the majority of GSD patients with WMHM20 compared to cornstarch.