J Clin Med Res

Journal of Clinical Medicine Research, ISSN 1918-3003 print, 1918-3011 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Clin Med Res and Elmer Press Inc

Journal website http://www.jocmr.org

Original Article

Volume 11, Number 5, May 2019, pages 337-344

Serum Oxidative-Antioxidative Status in Patients With Alkaptonuria

Figure 1. Scatter plot of the patient’s age, patients’ plasma TAC, TBARS, AOPP and HGA levels with disease severity score in the AKU patients.

**Table 1.** Patient’s Clinical Features
Clinical features/symptoms	N (%)
Dark urine	21 (100%)
Pigmentation of skin, outer ear and sclera of the eye	16 (76%)
Teeth pigmentation	19 (90%)
Morning stiffness and back pain due to degenerate arthropathy	21 (100%)
Arthropathy of large joints (hips and shoulders)	21 (100%)
Knees pain	13 (62%)
Kidney stones	13 (62%)
Prostate stones	5 (24%)
Aortic stenosis	1 (5%)
Ruptures of muscles, tendons and ligaments	1 (5%)
Knee joint replacement	1 (5%)
Foot pain and swelling of small joints (interphalangeal joints)	1 (5%)

Table 1. Patient’s Clinical Features

Clinical features/symptoms

N (%)

Dark urine

21 (100%)

Pigmentation of skin, outer ear and sclera of the eye

16 (76%)

Teeth pigmentation

19 (90%)

Morning stiffness and back pain due to degenerate arthropathy

21 (100%)

Arthropathy of large joints (hips and shoulders)

21 (100%)

Knees pain

13 (62%)

Kidney stones

13 (62%)

Prostate stones

5 (24%)

Aortic stenosis

1 (5%)

Ruptures of muscles, tendons and ligaments

1 (5%)

Knee joint replacement

1 (5%)

Foot pain and swelling of small joints (interphalangeal joints)

1 (5%)

**Table 2.** Age, Plasma Oxidative Markers’ Levels, Disease Severity Score and Urine HGA Levels in Alkaptonuria Patients and Controls
Variable	Patients^a	Control^a	Mann-Whitney U	P value
Patient score and urine HGA were null in controls. ^aData are means ± standard deviation (SD). *P value < 0.05 versus healthy subjects.
Age (years)	37.86 ± 12.59	36.00 ± 10.62	174.5	0.496
Sex (F/M)	4/17	6/13
TAC (nmol/µL copper reducing equivalent)	13.82 ± 1.63	8.92 ± 1.63	6	0.00*
TAC (nmol/µL uric acid equivalent)	0.63 ± 0.074	0.41 ± 0.074	6	0.00*
TBARS (MDA) (µmol/L)	0.050 ± 0.001	0.049 ± 0.0005	84.5	0.002*
Protein carbonyl (nmol/mL)	1.87 ± 0.34	1.77 ± 0.23	134	0.076
AOPP (µmol/mL)	4.39 ± 0.11	4.39 ± 0.14	161	0.297
HGA level (g/day)	1.23 ± 0.98	-	-	-
Disease severity score	34.24 ± 22.25	-	-	-

Table 2. Age, Plasma Oxidative Markers’ Levels, Disease Severity Score and Urine HGA Levels in Alkaptonuria Patients and Controls

Variable

Patients^a

Control^a

Mann-Whitney U

P value

Patient score and urine HGA were null in controls. ^aData are means ± standard deviation (SD). *P value < 0.05 versus healthy subjects.

Age (years)

37.86 ± 12.59

36.00 ± 10.62

174.5

0.496

Sex (F/M)

4/17

6/13

TAC (nmol/µL copper reducing equivalent)

13.82 ± 1.63

8.92 ± 1.63

0.00*

TAC (nmol/µL uric acid equivalent)

0.63 ± 0.074

0.41 ± 0.074

0.00*

TBARS (MDA) (µmol/L)

0.050 ± 0.001

0.049 ± 0.0005

84.5

0.002*

Protein carbonyl (nmol/mL)

1.87 ± 0.34

1.77 ± 0.23

134

0.076

AOPP (µmol/mL)

4.39 ± 0.11

4.39 ± 0.14

161

0.297

HGA level (g/day)

1.23 ± 0.98

Disease severity score

34.24 ± 22.25

**Table 3.** Correlation of the Patient’s Age, Patients’ Plasma TAC, TBARS, PC, AOPP and HGA Levels With Disease Severity Score in the AKU Patients
Variable	Disease severity score
*P value < 0.05.
Disease severity score	1
Age (years)	0.557	0.000*
TAC (nmol/µL copper reducing equivalent)	-0.046	0.353
TAC (nmol/µL uric acid equivalent)	-0.046	0.353
TBARS (MDA) (µmol/L)	-0.098	0.673
Protein carbonyl (nmol/mL	0.242	0.024*
AOPP (µmol/mL)	0.021	0.530
HGA level (g/day)	-0.043	0.494

Table 3. Correlation of the Patient’s Age, Patients’ Plasma TAC, TBARS, PC, AOPP and HGA Levels With Disease Severity Score in the AKU Patients

Variable

Disease severity score

Spearman’s correlation (r)

P value

*P value < 0.05.

Disease severity score

Age (years)

0.557

0.000*

TAC (nmol/µL copper reducing equivalent)

-0.046

0.353

TAC (nmol/µL uric acid equivalent)

-0.046

0.353

TBARS (MDA) (µmol/L)

-0.098

0.673

Protein carbonyl (nmol/mL

0.242

0.024*

AOPP (µmol/mL)

0.021

0.530

HGA level (g/day)

-0.043

0.494

**Table 4.** Linear Regression Analysis for Disease Severity Score Prediction
Model	Unstandardized coefficients	Standardized coefficients	P value	95% confidence interval for B
SE: standard error. *P value < 0.05.
Constant	22.239	23.343		0.353	-71.1	26.620
Protein Carbonyl(PC)	0	0	0.492	0.024*	0.000	0.001

Table 4. Linear Regression Analysis for Disease Severity Score Prediction

Model

Unstandardized coefficients

Standardized coefficients

P value

95% confidence interval for B

Beta

Lower bound

Upper bound

SE: standard error. *P value < 0.05.

Constant

22.239

23.343

0.353

-71.1

26.620

Protein Carbonyl(PC)

0.492

0.024*

0.000

0.001