Complications and Prognosis of Von Gierke Disease

Complications and Prognosis

Though Von Gierke disease can cause complications in multiple organs, prognosis has been improved with current treatments.


The long term complications of Von Gierke Disease include:

• Gout: Persistent elevation of uric acid due to Von Gierke Disease precipitates gout during puberty. Painful inflammation at joints arises.
• Puberty is often delayed.
• Nearly all females have ultrasound findings consistent with polycystic ovaries. Polycystic ovary syndrome (PCOS) is a condition resulting in an imbalance of female sex hormones in women. In spite of consistent ultrasound findings, other clinical features of PCOS such as acne and hirsutism (excessive hairiness) are not seen in Von Gierke Disease.
• Fertility is not affected (as reported by studies so far)

Other complications include:

• Increased risk of pancreatic disease, heart disease, bone fractures, liver cancer, high blood pressure (hypertension), kidney stones, kidney diseases like nephrocalcinosis (sometimes even kidney failure can occur)
• Short stature
• Recurrent infections with bacteria
• Severe brain damage can occur in neonates due to hypoglycemia (low blood glucose levels)
• Severe bleeding can ensue. Life threatening levels of menstrual bleeding (menorrhagia) have been reported
• Severe anemia

Prognosis (outlook)

Proper treatment allows a reasonable life span for patients. New treatment modalities have allowed survival till adulthood, which was previously rare. Kidney diseases, high blood pressure and liver cancer (hepatic adenomas that turn into cancers) increase mortality in adolescents and young adults.

Reference:

1. Harrison�s Principles of Internal Medicine 18^thEdition
2. The Merck Manual
3. Nelson Textbook of Pediatrics. 18th ed.
4. Cecil Medicine 24^thEdition

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