A 6-year-old boy with Alagille syndrome, characterised by marked hyperbilirubinaemia, presented with malunion of a pathological fracture of the femur with local bone atrophy and insufficient callus formation. During corrective osteotomy, it was noted that the femur was stained dark green, suggestive of bilirubin deposition. Histology of the resected bone revealed the presence of many histiocytes and osteoclast-like multinucleate giant cells containing bilirubin particles in the cytoplasm causing bone resorption. These findings suggest that bilirubin may activate macrophages to form osteoclast-like multinucleate giant cells, resulting in histiocytic osteolysis.
ChoudhryDKRehmanMASchwartzREPiccoliDA. The Alagille's syndrome and its anaesthetic considerations. Paediatr Anaesth1998;8:79–82.
2.
MaldiniGTorriELucianettiAGuizzettiMPinelliDBertaniA. Orthotopic liver transplantation for Alagille syndrome. Transplant Proc2005;37:1174–6.
3.
BagurAMautalenCFindorJSordaJSomozaJ. Risk factors for the development of vertebral and total skeleton osteoporosis in patients with primary biliary cirrhosis. Calcif Tissue Int1998;63:385–90.
4.
GuanabensNParesARosICaballeriaLPonsFVidalS. Severity of cholestasis and advanced histological stage but not menopausal status are the major risk factors for osteoporosis in primary biliary cirrhosis. J Hepatol2005;42:573–7.
5.
OrmarsdottirSLjunggrenOMallminHMichaelssonKLoofL. Increased rate of bone loss at the femoral neck in patients with chronic liver disease. Eur J Gastroenterol Hepatol2002;14:43–8.
6.
JanesCDicksonEROkazakiRBondSMcDonaghAFRiggsBL. Role of hyperbilirubinemia in the impairment of osteoblast proliferation associated with cholestatic jaundice. J Clin Invest1995;95:2581–6.
7.
GalloJKaminekPTichaVRihakovaPDitmarR. Particle disease. A comprehensive theory of periprosthetic osteolysis: A review. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub2002;146:21–8.
8.
ParwaniAVYangBClarkDPAliSZ. Particle disease: Cytopathologic findings of an unusual case. Diagn Cytopathol2004;31:259–62.
