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Table 1 Characteristics and laboratory values at baseline

From: Characteristics of a newly diagnosed Polish cohort of patients with neurological manifestations of Wilson disease evaluated with the Unified Wilson’s Disease Rating Scale

 

n

Study population Mean (SD) unless otherwise stated

Normal range

Female gender (n [%])

53

23 (43.4)

 

Age (years)

53

36.2 (11.3)

 

Age at onset (years)

52

30.4 (12.5)

 

Onset to diagnosis (years)

52

5.6 (6.6)

 

Presence of Kayser-Fleischer ring (n [%])

53

44 (83.0)

 

Ceruloplasmin (mg/dL)

53

13.7 (6.3)

25–45

Serum copper (μg/dL)

53

62.0 (17.8)

70–140

Urinary copper (μg/24 h)

53

128 (142)

< 50

Non-ceruloplasmin-bound copper (μmol/L)

53

3.4 (2.0)

a

Alanine aminotransferase (IU/L)

53

38 (43)

< 41

Aspartate aminotransferase (IU/L)

53

36 (21)

< 40

Gamma-glutamyltransferase (IU/L)

52

70 (57)

< 42

International normalized ratio

52

1.3 (0.2)

0.8–1.2

Bilirubin (mg/dL)

53

1.1 (0.6)

< 1

Serum albumin (g/dL)

47

4.0 (0.7)

3.5–5.0

  1. SD standard deviation
  2. aNon-ceruloplasmin-bound copper (NCC) is not commonly used in clinical practice in healthy individuals as approximately 80% have negative values. NCC has been reported to be above 4 μmol/L in most patients with Wilson disease [21]