A 10-Year-Old Boy With a 6-Month History of Gingival Bleeding and Epistaxis

Tracie Wong, MD; Pierre Russo, MD; Petar Mamula, MD Series Editors: David A. Piccoli, MD; Petar Mamula, MD


April 17, 2007

Further Investigation

Further investigation included an abdominal ultrasound, which revealed an enlarged liver with coarsened echotexture and no masses present (not shown). Additional laboratory investigations revealed serologies for hepatitis A, B, C, and cytomegalovirus and Epstein-Barr virus (EBV) were negative. The 24-hour urinary copper excretion, serum ceruloplasmin, alpha-1 antitrypsin level, and alpha-1 antitrypsin phenotyping were all normal. Antiplatelet antibody testing was negative.

A liver biopsy was performed:

Figure 1.

The liver parenchyma is nodular, with fibrous expansion and bridging of portal tracts. (Hematoxylin-eosin stain, magnification 10×)

Figure 2.

"Interface hepatitis," characterized by a mononuclear inflammatory infiltrate at the edge of a portal tract infiltrating into adjacent lobules, is present. (Hematoxylin-eosin stain, magnification 10×)

Antinuclear antibody (ANA) titers were negative; serum anti-smooth muscle antibody (SMA) was positive at titers of 1:2560, and anti F-actin was present at a level of 154 units. Anti-liver-kidney microsome antibody was negative and perinuclear antineutrophil cytoplasmic antibody was detected at titers of 1:80.

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