Table 5. Conditions associated with abdominal pain in adults with hemochromatosis.

Condition	Comments	References
Likely associated with iron overload
Cirrhosis	in patients with abdominal pain, odds ratio for cirrhosis 9.8 (95% CI: 1.2, 76.9)a	present study
Iron overload	pain mechanism unknown; iron overload and cirrhosis often present; pain sometimes alleviated with phlebotomy alone	present study
Primary liver cancer	typically older men with iron overload and cirrhosisb; pain usually in right upper quadrant; pain relief with indicated surgical or medical treatment + phlebotomy	present study; [20,24,42,49,50]
Portal vein thrombosis	men with iron overload, epigastric or right upper quadrant painc	[51,52]
Spontaneous E. coli peritonitis	iron overload and cirrhosis typical; rapidly progressive, severe abdominal pain, fever, and death ≤5 d after presentationd	[53–58]
Ascites	usually due to cirrhosis; pain more likely with large volumes	[20,24]
Not likely associated with iron overload
Acute or chronic cholecystitis, cholelithiasis	right upper quadrant pain relief with indicated surgical or medical treatment + phlebotomy	present study; [24,42,59]
Hepatic sarcoidosis	cholestasis, extra-hepatic lesions common; right upper quadrant pain relief expected with corticosteroid treatment + phlebotomy	[60–62]
Sigmoid diverticulitis	pain relief with indicated surgical or medical treatment + phlebotomy	present study
Peptic ulcer	pain relief expected with indicated surgical or medical treatment + phlebotomy	[24,42]
Other conditions	abdominal pain has been attributed to nephrolithiasis, perisplenitis, acute pancreatitis, and diabetic neuropathye	[20,24]
No demonstrable abnormality	risk of unexplained abdominal pain is higher in women than men unselected for hemochromatosis	present study; [11]

^aCauses of cirrhosis in the present patients include iron overload, excessive alcohol consumption, chronic viral hepatitis B, and hepatic sarcoidosis.

^bOne patient with hemochromatosis, cirrhosis, and primary liver cancer presented with bleeding esophageal varices and portal vein thrombosis [63]. Another patient with hemochromatosis, cirrhosis, and primary liver cancer presented with spontaneous E. coli peritonitis [55].

^cOne patient had HFE p.C282Y/p.H63D and hepatic steatosis [51].

^dMost patients with hemochromatosis and spontaneous E. coli peritonitis had heavy iron overload and cirrhosis [53–58]. One patient with hemochromatosis died of tuberculous peritonitis [42]. Another patient with hemochromatosis was diagnosed to have peritonitis not otherwise specified [49]. Other adults with hemochromatosis suffered from severe iron overload, and severe, progressive abdominal pain, followed by brief illnesses suggestive of spontaneous bacterial peritonitis leading to death, without positive bacterial cultures [37,64].

^eWe found no reports of persons with hemochromatosis who also had diabetes and gastroparesis.