Hereditary fructose intolerance (HFI) in the first three months of life usually appears as an acute liver failure that sometimes is associated with sepsis, acute dehydration, and shock. The coagulation abnormalities that cause a severe bleeding syndrome are generally considered to be caused by an impaired synthesis of clotting factors by the liver.
The presence of consumption coagulopathy in conjunction with HFI in infancy has been briefly referred to but not described in detail.1 We found in the literature just one report of HFI associated with disseminated intravascular coagulation (DIC) in an adult patient who had been previously treated with a prothrombin complex concentrate.2
We describe an infant in whom DIC complicating an acute liver failure was the initial-appearing feature of HFI.
Report of a Case.—A female infant, the second child of unrelated parents, was born at term after an uncomplicated pregnancy and delivery. The infant's birth
MAGGIORE G, BORGNA-PIGNATTI C. Disseminated Intravascular Coagulation Associated With Hereditary Fructose Intolerance. Am J Dis Child. 1982;136(2):169-170. doi:10.1001/archpedi.1982.03970380081017