Hypertrophic hepatic osteoarthropathy. Clinical, roentgenologic, biochemical, hormonal and cardiorespiratory studies, and review of the literature.
Twenty patients with biopsy proved liver disease, and roentgenologic features of hypertrophic osteoarthropathy have been studied, and the literature has been reviewed. The syndrome is a rare association of many chronic liver diseases, including primary biliary cirrhosis, bile duct carcinoma, benign bile duct stricture, chronic active hepatitis, posthepatitic cirrhosis and alcoholic cirrhosis. Patients may be asymptomatic, although bone pain, arthralgia or arthritis may be presenting symptoms. Ninety per cent of the patients are clinical jaundiced at the time of diagnosis, and 95 per cent have digital clubbing. The distal tibia and fibula are the first bones to become involved, although wrist, foot bones, femurs, hand bones and humeri may be affected in order of frequency. There is no correlation between the presence of esophageal varices or surgical portacaval shunts and the extent of the syndrome, neither is there a correlation with the degree of liver function impairment. Serum calcium and phosphate levels are normal, as is urinary hydroxyproline and estrogen excretion. There was no evidence to implicate elevated levels of growth hormone or overdosage of vitamin A. Although the majority of patients tested had mild arterial hypoxemia, increased cardiac output and evidence of right to left shunting, these were also present in disease-matched control subjects without osteoarthropathy. For screening purposes, patients with chronic liver disease and clubbing should have roentgenologic studies of the lower tibias and fibulas, to select those patients suitable for a more extensive skeletal survey.
D000328:Adult; D002118:Calcium / Q000097:blood; D002302:Cardiac Output; D004967:Estrogens / Q000652:urine; D005260:Female; D013006:Growth Hormone / Q000378:metabolism; D006801:Humans; D008102:Liver Circulation; D008107:Liver Diseases / Q000175:diagnosis* / Q000378:metabolism / Q000503:physiopathology; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D010004:Osteoarthropathy, Primary Hypertrophic / Q000175:diagnosis* / Q000378:metabolism / Q000503:physiopathology; D010100:Oxygen / Q000097:blood; D010281:Parathyroid Hormone / Q000378:metabolism; D010710:Phosphates / Q000097:blood; D011446:Prospective Studies; D012189:Retrospective Studies; D014801:Vitamin A / Q000097:blood
D004967:Estrogens; D010281:Parathyroid Hormone; D010710:Phosphates; D014801:Vitamin A; D013006:Growth Hormone; D010100:Oxygen; D002118:Calcium