Galactosemia with endogenous production of galactose-1-phosphate and with cystic fibrosis-like appearance at autopsy.

In an infant with galactosemia high levels of galactose-1-phosphate in red blood 
cells and of blood galactose were observed under a "galactose-free'' diet. 
The child did not thrive and developed a liver cirrhosis. At 
the age of 5 months he died unexpectedly. Post mortem examination 
revealed in the pancreas and the small intestine changes suggestive of 
a cystic fibrosis. Since the exogenous administration of galactose by diet 
could be excluded the endogenous production of significant amounts of galactose-1-phosphate 
has to be considered. 