Glycogen storage<unk>  disease<unk>   is caused by the deficiency of phosphorylase B kinase. In 1959, Dr. Hers first discovered glycogen storage<unk> <unk>  disease in the patients with liver phosphorylase deficiency. Glycogen storage<unk>  disease<unk>  is an autosomal recessive disease and some forms are X-linked recessive.<unk>  is classified according to the pattern of inheritance associated with the enzyme deficiency into 2 subtypes, autosomal recessive liver phosphorylase kinase deficiency and x-linked recessive liver phosphorylase kinase deficiency.<unk>  presents at the age of 1-5 years. A positive history of the protuberant abdomen, growth retardation and the slight delay in motor milestones is suggestive of<unk>  and some children have the history of mild fasting hypoglycemia and hypotonia.Patient with the<unk>  presents with the symptoms of hypoglycemia on fasting, such as faintness, weakness,  and nervousness. On physical examination, the increased liver span is present. The mainstay of treatment is the dietary therapy which includes frequent meals, high carbohydrate diet, high protein diet and supplementation of unsaturated fats.
Glycogen storage disease type VI
Glycogen storage disease type I CT
Glycogen storage disease type III
Glycogen storage disease type IV
Glycogen storage disease type I
Glycogen storage disease type II
Glycogen storage disease type XI
Essential fructosuria