2% were postpubertal (Tanner stage 5). The distribution of histologic features is also summarized in Table 1. Briefly, the prevalence of severe steatosis (G3) was 39.3%, severe lobular inflammation (G2-3) was 8.9%, hepatocyte ballooning (G1-2) was 23.2%, more than mild portal inflammation Akt inhibitor (G2) was 23.6%, and advanced fibrosis was 37.8%. Simple steatosis was seen in 20 cases (35.7%). Features of SH were noted in the remaining 36 cases (64.3%). Histology was suspicious for SH in 26 cases, including nine cases (35%) with the pediatric pattern and 17 cases (65%) with the adult pattern. Definite SH was diagnosed in 10 cases, two of which (20%) demonstrated the pediatric pattern; the eight remaining cases

of definite SH (80%) exhibited the adult pattern. Thus, simple steatosis was present in a minority of our cohort. The larger subgroup had features of SH, with roughly Torin 1 one-third of those individuals demonstrating pediatric pattern SH and the remaining two-thirds exhibiting the more classical, adult SH pattern. When comparing the severity of the histologic features between boys and girls, we noted

that steatosis grade and fibrosis stage were significantly higher in boys (Wilcoxon rank sum test: P = 0.037 for steatosis and P = 0.02 for fibrosis). Regardless of gender, age was found to correlate with portal inflammation (ANOVA: P = 0.0005); patients with G1-2 portal inflammation were younger than patients with no portal inflammation (G0) (Tukey test: P < 0.05). Although univariate analyses did not show significant associations between pubertal stages and histologic features, there was a trend toward higher grades of portal inflammation in earlier pubertal stages (Wilcoxon rank sum test, P = 0.08). The median SHh grade was 2.5 [2, 4] in the available cohort (n = 20). Univariate analysis showed that

SHh grade was significantly associated with fibrosis stage (P = 0.0008, Fig. 1A-D) and steatosis grade (P = 0.022, Fig. 1D). SHh grades were higher in cases with advanced fibrosis (S3-4) compared to cases with no fibrosis (S0, P = 0.0007) and cases with mild to moderate fibrosis (S1-2, P = 0.009) (Fig. 1A-D). SHh grades were higher in cases with moderate steatosis selleck inhibitor (G2) than in cases with no to mild steatosis (G0-1, P = 0.0091, Fig. 1D). In the ordinal logistic regression model including steatosis grade, fibrosis stage, and gender, the association between SHh grade and fibrosis stage remained significant (P = 0.002), although steatosis grade did not (P = 0.241). There were no significant associations between SHh expression and grades of lobular inflammation, ballooned hepatocytes, or portal inflammation. Liver progenitor cells are known to be Hh-responsive. Therefore, we stained liver sections for the progenitor cell marker, keratin 7 (K7), and Gli2 (a marker of Hh-responsiveness). The median numbers of Gli2+ and K7+ cells per HPF were 227 [121, 380] and 17 [14, 25], respectively (n = 26 for both stains).