TABLE 4.
Histological Characteristics of Liver Biopsies in patients With APECED and APAH
| Patient # | Age at Liver Biopsy (Years) | Sex | Histological Activity Index Score |
IF Stage |
||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Periportal Necrosis | Lobular Inflamm | Portal Inflamm | Inflamm (Total) | Fibrosis (Total) | Piecemeal Necrosis | Lobular Inflamm | Portal Inflamm | Confluent Necrosis | Inflamm (Total) | Fibrosis (Total) | Plasma Cells | Eosinophils | Histological Pattern | |||
| 1 | 7.7 | F | 4 | 3 | 3 | 11 | 1 | 4 | 2 | 3 | 2 | 11 | 2 | Increased | Increased | Chronic hepatitis |
| 2 | 2.0 | M | 3 | 4 | 1 | 8 | 0 | 3 | 3 | 2 | 0 | 8 | 0 | Increased | Not increased | Chronic hepatitis |
| 3 | 31.9 | F | 6 | 4 | 4 | 14 | 3 | 4 | 3 | 3 | 4 | 14 | 3 | Increased | Increased | Chronic hepatitis |
| 4 | 15.1 | F | 3 | 1 | 1 | 5 | 1 | 3 | 1 | 2 | 0 | 6 | 1 | Increased | Not increased | Chronic hepatitis |
| 5 | 8.8 | F | 0 | 1 | 0 | 1 | 0 | 0 | 1 | 0 | 0 | 1 | 0 | Not increased | Not increased | Nonspecific changes |
| 6 | 3.2 | F | 1 | 1 | 1 | 3 | 1 | 1 | 1 | 1 | 0 | 3 | 1 | Not increased | Not increased | Nonspecific changes |
| 7 | 5.9 | F | 6 | 4 | 3 | 13 | 0 | 4 | 4 | 3 | 5 | 16 | 0 | Increased | Not increased | Acute hepatitis |
| 8 | 20.9 | F | 5 | 3 | 3 | 11 | 1 | 3 | 2 | 2 | 4 | 11 | 2 | Increased | Not increased | Chronic hepatitis |
| 9 | 2.7 | F | 3 | 3 | 1 | 7 | 1 | 3 | 2 | 2 | 0 | 7 | 2 | Not increased | Not increased | Chronic hepatitis |
| 10 | 4.3 | F | 5 | 4 | 1 | 10 | 0 | 2 | 3 | 2 | 5 | 12 | 0 | Not increased | Not increased | Acute hepatitis |
| 11 | 9.8 | M | 4 | 3 | 4 | 11 | 1 | 3 | 2 | 3 | 0 | 8 | 2 | Increased | increased | Chronic hepatitis |
| 12* | 2.1 | M | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A |
| 13 | 15.0 | F | 3 | 4 | 1 | 8 | 1 | 3 | 4 | 2 | 3 | 12 | 1 | Increased | Not increased | Chronic hepatitis |
| 14 | 7.0 | M | 6 | 4 | 3 | 13 | 1 | 3 | 3 | 3 | 4 | 13 | 1 | Increased | Not increased | Chronic hepatitis |
| 15 | 2.1 | M | 6 | 4 | 3 | 3 | 0 | 4 | 4 | 3 | 5 | 16 | 0 | Not increased | Increased | Chronic hepatitis |
| 16 | 16.6 | F | 4 | 4 | 3 | 11 | 1 | 4 | 2 | 3 | 2 | 11 | 1 | Increased | Not increased | Chronic hepatitis |
| 17 | 11.7 | F | 4 | 4 | 3 | 11 | 1 | 4 | 2 | 2 | 3 | 11 | 1 | Increased | Increased | Chronic hepatitis |
| 18* | 8.0 | F | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A | N/A |
*
In 2 of the 18 patients with APAH, we could not obtain the prior liver biopsies for review at NIH.
Abbreviations: F, female; Inflamm, inflammation; M, male; N/A, not available.