Table 3.

Mainz Severity Score Index of patients with classical type vs cardiac subtype

Demographic data	Classical subjects N = 22	Cardiac subjects N = 22	p
General score	1.0 (0.0–3.0)	0.0 (0.0–1.0)	0.001
Characteristic facial	0 (0)	0 (0)	1.000
Angiokeratoma	2 (9.1)	0 (0)	0.148
Edema	2 (9.1)	0 (0)	0.148
Musculoskeletal	4 (18.2)	0 (0)	0.036
Cornea verticillata	7 (31.8)	0 (0)	0.004
Diaphoresis			0.060
Hypo/hyperhidrosis	1 (4.5)	0 (0)
Anhidrosis	4 (18.2)	0 (0)
Abdominal pain	1 (4.5)	0 (0)	0.312
Diarrhea/constipation	1 (4.5)	0 (0)	0.312
Hemorrhoids	0 (0)	0 (0)	1.000
Pulmonary	2 (9.1)	1 (4.5)	0.550
NYHA			0.769
Class I	4 (18.2)	6 (27.3)
Class II	1 (4.5)	1 (4.5)
Class III	0 (0)	0 (0)
Class IV	0 (0)	0 (0)
Appearance
Neurologic score	5.0 (0.0–6.0)	0.0 (0.0–1.5)	0.002
Tinnitus	0 (0)	1 (4.5)	0.312
Vertigo	2 (9.1)	1 (4.5)	0.550
Acroparesthesia
Occasional	6 (27.3)	3 (13.6)	<0.001
Chronic	9 (40.9)	0 (0)
Fever pain crisis	4 (18.2)	0 (0)	0.036
Cerebrovascular	0 (0)	2 (9.1)	0.148
Psychiatric
Depression	0 (0)	0 (0)	1.000
Fatigue	1 (4.5)	1 (4.5)	1.000
Reduced activity level	0 (0)	2 (9.1)	0.148
Cardiovascular score	10.0 (2.0–12.5)	10.5 (9.0–15.25)	0.277
Left ventricle hypertrophy
Thickening of wall	1 (4.5)	0 (0)	0.197
LVH seen in ECG	3 (13.6)	0 (0)
Cardiomyopathy (<15 mm)	9 (40.9)	11 (50)
Severe cardiomyopathy (>15 mm)	4 (18.2)	8 (36.4)
ECG abnormalities	13 (59.1)	11 (50)	0.545
Hypertension	9 (40.9)	13 (59.1)	0.228
Valve insufficiency	20 (90.9)	19 (86.4)	0.635
Pacemaker	1 (4.5)	1 (4.5)	1.000
Renal score	4.0 (0.0–4.0)	0.0 (0.0–0.0)	0.007
Proteinuria	10 (45.5)	3 (13.6)	0.021
Low GFR	2 (9.1)	1 (4.5)	0.550
Creatinine > 3.5 mg/dL	0 (0)	0 (0)	1.000
Dialysis	1 (4.5)	0 (0)	0.312
Total score	17.5 (13.25–23.0)	14.5 (9.75–18.0)	0.051

We expressed the individual parameter with number (percentage) for categorical variables and median (25th percentile–75th percentile) for numerical variables.

GFR = glomerular filtration rate; LVH = left ventricular hypertrophy.