Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2 Presenting with Severe Coagulopathy without Jaundice

Table 1

Initial presenting clinical and laboratory data.

Case	Age at diagnosis	ALT (U/L)	Total bilirubin (mg/dL)	Direct bilirubin (mg/dL)	Serum bile acids (umol/L)	GGT (U/L)	Prothrombin time (seconds)	INR	Vitamin D (25-OH); (ng/mL)
A	5 months	334	3.4	2.8	205.3	33	>120.0	>13.7	<5
B	14 years	57	0.6	0.1	n/a	35	40	4.2	n/a

Values marked n/a mean that no data was available for that patient. Normal reference values: ALT, 0–31 U/L; total bilirubin, 0.1–1.2 mg/dL; direct bilirubin, 0.0–0.4 mg/dL; serum bile acids, 4.5–19.2 umol/L; GGT, 8–51 U/L; prothrombin time, 9.4–11.6 seconds; INR, 0.9–1.1; vitamin D (25-OH), 32–100 ng/mL.