FAQs about Von Gierke Disease

Frequently Asked Questions

1. What doctor treats Von Gierke Disease?

Von Gierke Disease is often diagnosed by a neonatologist since symptoms appear soon after birth. Untreated and undiagnosed children are admitted by hematologists or gastroenterologists for the diagnosis of massively enlarged liver. Consultations with genetics specialist, nephrologist are also warranted at times.


2. Can women with Von Gierke Disease have a successful pregnancy?

Yes. Reports suggest that GSD1 does not affect fertility; women with the disease can have successful pregnancy.

3. What food is best in Von Gierke Disease?

Cornstarch that provides gradually digested glucose is inexpensive and works great. Frequent short feeds are advocated. Diet is the mainstay of treatment and requires close monitoring and adjustment by a highly specialized nutritionist. Excessive carbohydrates and calories are to be avoided while adequate calories and protein for growth are ensured.

Overnight feeding with special tubes called nasogastric tubes are given. In children older than 2-3 years, this is usually replaced by raw cornstarch at bedtime and early morning hours.

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