أزمة الأيض الحادة في داء تخزين الغليكوجين النوع السادس المحفوزة بالصيام المطوّل أو المرض الحموي أو الاضطرابات الهضمية

References

DOI: 10.1038/s41436-018-0364-2

• A metabolic crisis may be precipitated by prolonged fasting or illness in GSD VI and IX.
• Febrile illness can increase glucose requirements, and gastrointestinal illness can make it difficult or impossible to tolerate frequent oral feedings.
• When intravenous dextrose support is required, a concentration of 10% dextrose should be used at a rate that is 1–1.25 times the maintenance rate with appropriate electrolytes.
• The rate can be increased based on blood glucose levels.
• Dextrose support should be weaned over a 2–3 hour period once full enteral intake is tolerated.
• Blood glucose and β-OHB concentrations should be measured upon arrival, and blood glucose should be measured hourly on intravenous fluids until it is determined they are stable and greater than 70 mg/dL.