Paediatric Guidelines

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GLUT1 Deficiency (Glucose transporter deficiency, GLUT1-DS, Hypoglycorrhachia)

Glutaric Aciduria Type 1 (GA1)

Glycerol Kinase Deficiency (isolated, same as Ketotic Hypoglycaemia)

***IMPORTANT NOTE: some children have glycerol kinase deficiency as part of a contiguous gene deletion causing in addition congenital adrenal hypoplasia and muscular dystrophy. These patients require different emergency management to take account of their endocrine dysfunction.*** Standard , A&E , Child dietary emergency regimen , Making intravenous fluids for metabolic patients ,

Glycogen Storage Disease Type 1a (GSD Type 1A, Glucose-6-phosphatase deficiency)

Glycogen storage disease Type 1b (GSD1b, Glucose-6-phosphate translocase deficiency)

Glycogen Storage Disease Type III (GSD 3, Debrancher deficiency)

Glycogen Synthase Deficiency (GSD0, Glycogen Storage Disease Type Zero)