Cholelithiasis in patients with variegate porphyria.
Four patients with variegate porphyria (VP) who developed symptoms attributable to cholelithiasis are described. Elective cholecystectomy was performed uneventfully in two of these patients in whom the diagnosis of porphyria had previously been made. The third patient was not known to have porphyria at the time of surgery and developed a fulminating attack which proved fatal. The major haem precursor overproduced in VP is protoporphyrinogen and protoporphyrin was present in the gallstones available for analysis. Our experience suggests that patients with VP have an increased risk of cholelithiasis which may be related to the overproduction of protoporphyrinogen and increased bile concentration of protoporphyrin.
Herrick
A L
AL
University Department of Medicine and Therapeutics, Western Infirmary, Glasgow, United Kingdom.
Moore
M R
MR
Thompson
G G
GG
Ford
G P
GP
McColl
K E
KE
D000328:Adult; D001646:Bile / Q000737:chemistry; D002769:Cholelithiasis / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D011524:Protoporphyrins / Q000032:analysis / Q000378:metabolism
D011524:Protoporphyrins