Von Gierke's disease

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glycogen storage disease type I

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• Clinical data suggest that the osteoporosis of Von Gierke's disease is due to hypoglycemia or a metabolic sequela thereof, such as insulinopenia, but pathologic study of patients treated by newer techniques of maintaining euglycemia in GSD is needed.
• A detailed description of the course of pregnancy in a patient with von Gierke's disease is presented.
• Type Ia Glycogenosis: von Gierke's Disease Von Gierke's disease is caused by a deficiency in glucose 6-phosphatase, an enzyme present in liver and kidney cells, but not in muscle or brain cells.
• von Gierke's disease is a glycogen storage disease caused by a deficiency of glucose-6-phosphatase.
• Summary: Von Gierke's Disease is rare and its occurrence in associatiorwith hypogonadism though known is extremely rare.

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