Hers' disease

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An inborn glycogen metabolism disorder caused by deficiency of hepatic phosphorylase, associated with an enlarged liver and mild hypoglycaemia.

Description

An inborn glycogen metabolism disorder caused by deficiency of hepatic phosphorylase, associated with an enlarged liver and mild hypoglycaemia. Both sexes affected, onset in infancy and early childhood. Symptoms sometimes so mild that syndrome may pass undetected. Moderate growth retardation; mild to moderate Harris’ syndrome. Inheritance is probably autosomal recessive.

See also Harris' syndrome, or hyperinsulinism, under Seale Harris, American physician, 1870-1957.

Bibliography

  • H. G. Hers:
    Etudes enzymatiques sur fragments hépatiques: Applications à la classification des glycogénoses.
    Revue internationale d'hépatologie, 1959, 9: 35-55.
  • B. Lederer, F. Van Hoof, G. Van den Berghe, H. Hers:
    Glycogen phosphorylase and its converter enzymes in haemolysates of normal human subjects and of patients with type VI glycogen-storage disease. A study of phosphorylase kinase deficiency.
    The Biochemical Journal, London, April 1975, 147 (1): 23-35.

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