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Table 1 Characteristics of the patients with Ataxia-telangiectasia

From: Hepatic fibrosis: a manifestation of the liver disease evolution in patients with Ataxia-telangiectasia

Variables

 

N (%)

Age (n = 25)

Children (< 10 years)

9 (36.0%)

Adolescents (10 to 19 years)

11 (44.0%)

Adults (≥ 20 years)

5 (20.0%)

Nutritional status (n = 25)

Underweight

8 (32.0%)

Adequate

16 (64.0%)

Overweight

1 (4.0%)

Fat mass (n = 24)

Low

4 (16.7%)

Adequate

13 (54.1%)

High

7 (29.1%)

MUAMC (n = 24)

Low

13 (54.1%)

Adequate

11 (45.8%)

WHtR (n = 24)

Adequate

20 (83.3%)

High

4 (16.7%)

Pubertal stage (n = 25)

Prepubertal

9 (36.0%)

Pubertal

8 (32.0%)

Postpubertal

8 (32.0%)

ICARS (n = 25)

Mild ataxia

3 (12.0%)

Moderate ataxia

13 (52.0%)

Severe ataxia

9 (36.0%)

hs-CRP (n = 25)

 > 1 mg/L

7 (28.0%)

 ≤ 1 mg/L

18 (72.0%)

Lipid profile (n = 25)

High total cholesterol

5 (20.0%)

High LDL-c

5 (20.0%)

High triglycerides

6 (24.0%)

Low HDL-c

8 (32.0%)

High NHDL-c

14 (56.0%)

Glucose metabolism (n = 23)

Diabetes

4 (17.4%)

Insulin resistance** (n = 21)

5 (23.8%)

Hepatic steatosis (n = 20)

Absent

7 (35.0%)

Mild

8 (40.0%)

Moderate

5 (25.0%)

Significant liver fibrosis (n = 25)

 

5 (20.0%)

ALT (U/L) (n = 25)

 ≥ 2 xULN

4 (16.0%)

AST (U/L) (n = 25)

 ≥ 2 xULN

2 (8.0%)

  1. N (%) absolute and percentage values
  2. MUAMC mid-upper arm muscle circumference, WHtR waist circumference/height ratio, hs-CRP high sensitivity C reactive protein, ICARS International Cooperative Ataxia Rating Scale
  3. **HOMA-AD Homeostasis Model Assessment- Adiponectin, ALT alanine aminotransferase, AST aspartate aminotransferase, xULN times the upper limit of normal