Case Report

Budd-Chiari Syndrome with Multiple Thrombi due to a Familial Arg42Ser Mutation in the Protein C Gene

Table 1

Laboratory test values of the proband upon admission.

Complete blood count
 White blood cell count6,600/μL
 Red blood cell count396 × 104/μL
 Hemoglobin12.4 g/dL
 Hematocrit37.6%
 Platelets16.0 × 104/μL
Blood biochemistry
 Aspartate aminotransferase29 IU/L
 Alanine aminotransferase46 IU/L
 Alkaline phosphatase175 IU/L
 Lactate dehydrogenase190 IU/L
γ-GTP39 IU/L
 Total bilirubin0.8 mg/dL
 Creatine phosphokinase79 IU/L
 Total cholesterol299 mg/dL
 Triglyceride104 mg/dL
 HDL-cholesterol72 mg/dL
 LDL-cholesterol195 mg/dL
 Sodium143 mEq/L
 Chloride108 mEq/L
 Potassium3.9 mEq/L
 Uric acid3.1 mg/dL
 Blood urea nitrogen8 mg/dL
 Creatinine0.57 mg/dL
 Total protein5.1 g/dL
 Albumin3.5 g/dL
 C-reactive protein0.20 mg/dL
 Hemoglobin A1c5.6%
 Thyroid-stimulating hormone1.72 μIU/mL
 Thyroxine, free (fT4)0.8 pg/mL
 Thyroxine, free (fT3)2.0 pg/mL
 BNP21.1 pg/mL
Coagulation
 APTT32.5 s
 PT-INR1.06
 D-dimer1.40 μg/mL
Immunochemistry
 Immunoglobulin G320 mg/dL
 Immunoglobulin A71 mg/dL
 Immunoglobulin M114 mg/dL
 Immunoglobulin E17.6 IU/mL
 Complement titer (CH50)52.8 U/mL
 Complement C3111 mg/dL
 Complement C437 mg/dL
 Antinuclear antibody<40
 Lupus anticoagulant(−)
 HBs antigen(−)
 HCV-antibody(−)
<ELISA>
 MPO-ANCA93 EU
 PR3-ANCA<10 EU
Urinalysis
 pH7.0
 Specific gravity1.010
 Glucose(−)
 Protein(−)
 Cast(−)
 Erythrocytes1–5/HPF
 Leukocytes1–5/HPF

Abbreviations: γ-GTP: gamma-glutamyl transpeptidase; HDL cholesterol: high-density lipoprotein-cholesterol; LDL cholesterol: low-density lipoprotein cholesterol; PR3-ANCA: proteinase-3 antineutrophil cytoplasmic antibody; MPO-ANCA: myeloperoxidase antineutrophil cytoplasmic antibody; APTT: activated partial thromboplastin time; BNP: B-type natriuretic peptide; PT-INR: prothrombin time-international normalized ratio.