Table 1 Clinical and laboratory features of patients included in the study (n = 25)
From: Spectrum of genomic variations in Indian patients with progressive familial intrahepatic cholestasis
Clinical feature | Value in all patients studied (n = 25) | Value in those with genomic variation (n = 9) | Value in those without genomic variation (n = 16) | p value (with genomic variation versus without genomic variation) |
|---|---|---|---|---|
Age at presentation (months) | 15 (1–144) | 15 (3–144) | 23 (1–48) | 0.089 |
Age at onset of symptoms (months) | 5 (0.25–138) | 3.5 (0.25–138) | 5 (0–18) | 1.123 |
Male (number) | 19 (76%) | 7 | 12 | 1.000 |
History of consanguinity (number) | 7 (28%) | 5 | 2 | 0.058 |
History of cholestasis of pregnancy (number) | 10 (40%) | 3 | 7 | 0.691 |
Intense pruritus (number) | 24 (96%) | 8 | 16 | 0.360 |
History of skin bleeding (number) | 17 (68%) | 5 | 12 | 0.394 |
Jaundice (number) | 25 (1) | 9 | 16 | 1.000 |
Weight z scorea | −2.08 (−3.55 to +0.32) | − 2.35 (−3.55 to +0.32) | −1.98 (−3.52 to +0.21) | 0.205 |
Height z scoreb | − 1.63 (−5.62 to +2.26) | −1.55 (−4.75 to +0.2) | −1.73 (−5.62 to +2.26) | 0.478 |
Palpable liver below right costal margin (cm)c | 4 (1–8) | 3 (2–8) | 4 (1–7) | 0.790 |
Palpable spleen below left costal margin (cm)d | 2 (1–5) | 1 (1–5) | 3 (2–4) | 0.300 |
Total serum bilirubin (mg/dL) | 7.0 (1.3–32.0) | 12.7 (1.3–21.0) | 6.6 (1.7–32.0) | 0.296 |
Serum albumin (g/dL)e | 3.7 (2.7–4.5) | 3.8 (3.2–4.2) | 3.8 (2.7–4.5) | 0.777 |
Serum aspartate aminotransferase (U/L) | 175 (45–1210) | 92 (45–1120) | 214 (69–1210) | 0.844 |
Serum alanine aminotransferase (U/L) | 142 (29–682) | 96 (29–598) | 104 (30–682) | 0.947 |
Serum gamma-glutamyl transpeptidase (U/L) | 28 (8–69) | 25 (8–57) | 33.5 (11–69) | 0.154 |