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Glycogen Storage Disease Type I and III and Pyruvate Carboxylase Deficiency: Results of Long‐Term Treatment with Uncooked Cornstarch
Author(s) -
ULLRICH K.,
SCHMIDT H.,
TEEFFELENHEITHOFF A.
Publication year - 1988
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1988.tb10695.x
Subject(s) - medicine , glycogen storage disease , pyruvate carboxylase , glycogen , disease , glycogen storage disease type i , endocrinology , starch , gastroenterology , food science , biochemistry , biology , enzyme
. Three patients with glycogen storage disease type I (GSD‐I), three with glycogen storage disease type III (GSD‐III) and one with pyruvate carboxylase deficiency (PCD) could be successfully switched over from continuous nocturnal gastric drip feeding (GDF) to nocturnal feeding with uncooked cornstarch in yoghurt or “quark” (CSF) at the age of 4–20 years. The new kind of therapy is much more convenient for the patients. When followed up to 30 months, patients on CSF showed the same clinical and laboratory findings as during the last two years with GDF. CSF was not introduced to three patients with GSD‐I. Two of them refused the permanent starch‐yoghurt meals. In the third patient the morning blood glucose concentrations were too variable.