Table 1.
Clinical, Biochemical, and Histological Profile of All the Patients with Non-cirrhotic Portal Fibrosis (NCPF).
| Patient characteristics | Patient 1 | Patient 2 | Patient 3 |
|---|---|---|---|
| Age (years) | 27 | 33 | 19 |
| Sex | Female | Female | Female |
| Presenting complaints | Easy fatigability and recurrent iron deficiency anemia | Easy fatigability and left hypochondrium pain | Chronic diarrhea |
| Clinically palpable splenomegaly | ++ | ++ | +++ |
| Hemoglobin (g/dL; normal12–16 g/dL) | 7.6 | 8.5 | 10.2 |
| Total leukocyte count (4500–11000/mm3) | 3500 | 2450 | 4100 |
| Platelet count (1.5–4.5/mm3) | 80,000 | 1.41 | 76,000 |
| Bilirubin mg/dL (total/conjugated) | 1.4/0.7 | 2.3/1.3 | 1.2/0.4 |
| SGOT/SGOT (U/L) | 40/35 | 18/32 | 34/24 |
| Alkaline phosphatase (U/L) | 254 | 115 | 59 |
| Albumin (g/dL) | 3.7 | 3.4 | 4.0 |
| INR | 1.62 | 1.43 | 1.63 |
| Anti-TTG (normal <3 Unit/mL) | >100 | 78 | 112 |
| Concomitant autoimmune disease | Hypothyroidism | Nil | Subclinical hypothyroidism |
| LSM (kPa) | 11 | 9.1 | 9.8 |
| HVPG (mm of Hg) (normal < 5) | 9 | 8 | 7.5 |
| Varices | Small esophageal varices | Small esophageal varices | Small esophageal varices |
| Spleen size (7.6–13 cm) | 22 | 25 | 20 |
| PV diameter (<13 mm) | 16 | 15 | 18 |
| Liver biopsy | Inconspicuous portal tract with fibrosis | Normal liver biopsy | Peri-portal tract fibrosis |
| Response to GFD | Died of intestinal perforation after eight months of diagnosis after transient response | Yes Gained weight, anemia improved |
Yes Diarrhea improved |
AMA: anti-mitochondrial antibody; ANA: antinuclear antibody; HVPG: hepatic venous pressure gradient; INR: international normalized ratio; kPa: kilopascal; LKM1: liver kidney microsomal antibody 1; SGOT: serum glutamic-oxalacetic transaminase; SGPT: serum glutamic-pyruvic transaminase; TTG: tissue transglutaminase; U/L: unit per liter.
The normal values are given within brackets.