Glycogenosis type III

In glycogenosis type III, the outer chain length of glycogen is reduced in glycogenosis type IV, it is increased. 

In glycogenosis type III (debranching enzyme deficiency) the absorption maximum is around 410 nm in muscle in liver the maximum remains around 460 nm, but there is a pronounced shoulder at 410 nm. In glycogenosis type IV (branching enzyme deficiency) the absorption maximum is around 530 nm. 

Amylo-l,6-glucosidase is the enzyme deficient in glycogenosis type III (GSD III, MIM 232 400). 

M. R. A man with type III glycogenosis associated with cirrhosis and portal hypertension. Gastroenterology 1993 105 1882—1885... 

Erythrohepatic protoporphyria Fructose intolerance Galactosaemia Gaucher s disease Glycogenosis (types I, III, IV) Haemochromatosis Hurler s disease Hypermethioninaemia Mucoviscidosis... 

Epidemiology and aetiology Adenomas have an incidence of 4-10/100,000 inhabitants/year. They are almost exclusively found in women during their reproductive years. Children are rarely affected. (28) In men, the risk is higher when they take anabolic agents. In about 90% of cases, there is a correlation with the intake of oral contraceptives over many years this was pointed out for the first time by J.K. Baum et al. in 1973. (19, 21, 25, 31, 32) There is also an association with the polycystic ovary syndrome. (33) An adenoma can develop in glycogenosis types I and III (with multilocular nodulation) (29) as well as in type IV. (16) The administration of various other drugs (e.g. carbamazepine) has also been associated with adenomas. 

Fig. 3-12. Pools of cytoplasmic granules in liver cell (x 8,000) of patient with Type III glycogenosis (Zamboni)...

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