داء تخزين الغليكوجين من النوع الثالث في النساء الحوامل: الإدارة السريرية

Any woman with GSD III who chooses to pursue pregnancy runs some risks and requires careful follow-up by a high risk obstetrician.

The primary goal during pregnancy is to maintain normoglycemia.

Hypoglycemia may be more difficult to control attributable to the accelerated starvation state of pregnancy, thus requiring a more constant and reliable route of glucose/protein administration such as continuous nasogastric feeds, CS every 4 – 6 hours, and central hyperalimentation.

At the time of delivery, important precautions include the use of an intravenous glucose infusion (to prevent hypoglycemia); usually, D10 is preferred.

It is extremely important to maintain euglycemia throughout pregnancy and to avoid upregulation of counter-regulatory hormones (this would result in lipolysis and ketosis, with risk of fetal demise).

Maintenance of normoglycemia and avoidance of hypoglycemia and ketosis is critical throughout the pregnancy and during labor and delivery.

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