GLYCOGEN STORAGE DISEASE, 11811

11811              GLYCOGEN STORAGE DISEASE

 

The patient was a known sufferer from cholecystitis who was admitted for routine cholecystectomy.  There had been attacks of biliary colic for 8 years and episodes of obstructive jaundice.  She was under treatment with thyroid extract for myxoedema.  At operation a chronically inflamed gallbladder was removed, together with a large stone from the common bile duct.  A week after the operation she became shocked and began to vomit.  The BP was unrecordable.  Intravenous therapy produced some improvement, but renal failure persisted and she died 15 days after operation.  At postmortem the liver was hard and slightly nodular but the spleen was normal in size and was soft and pale.

 

The specimen is a slice of the liver.  It is markedly pale and there is a very fine cirrhosis.  Histology shows a fine cirrhosis with PAS-positive material in the liver cell cytoplasm - (not nuclei).  This finding was apparently unexpected.  Chemical analyses to characterise the glycogen were not performed (the year was 1958).

Last modified: Wednesday, 2 August 2017, 8:40 AM