Kidney function as an underestimated factor for reduced health related quality of life in patients with Fabry disease

Table 1 Characteristics of study participants, stratified by kidney function

	eGFR ≥60 ml/min/1.73 m²n = 77	eGFR <60 ml/min/1.73 m²n = 10	RRT (dialysis or KTx) n = 9	p-value
Age, yrs	38.8 ± 13.1	53.0 ± 11.3	37.0 ± 8.6	0.004
Male gender	39.1 %	60.0 %	100.0 %	0.002
Diagnosis Fabry disease, months	9 (6; 14)	12 (8; 22)	14 (7; 94)	0.31
Enzyme replacement therapy^a	37.7 %	60.0 %	88.9 %	0.01
BMI, kg/m²	22.4 (20.8; 24.9)	23.6 (20.9; 30.8)	21.9 (19.9; 24.4)	0.36
Systolic blood pressure, mmHg	118 ± 15	133 ± 21	131 ± 25	0.01
Diastolic blood pressure, mmHg	75 ± 10	81 ± 14	81 ± 9	0.07
eGFR, ml/min/1.73 m²	96 (83; 110)	37 (25; 45)	dialysis 7 (5; 13) KTx 37 (19; 54)	<0.001
Vascular event				0.05
Minor^b	13.2 %	40.0 %	22.2 %
Major^c	4.0 %	10.0 %	0 %
Pain	44.3 %	77.8 %	71.4 %	0.08
Pain therapy	18.8 %	60.0 %	62.5 %	0.002
VAS^d	2 (1; 3)	2 (0; 3)	2 (2; 3)	0.46

Data are means (± SD), medians (IQR) or proportions; comparison across CKD stages using ANOVA, Kruskal-Wallis test, χ²- test or Fisher’s exact test, as appropriate; abbreviations: eGFR estimated glomerular filtration rate as assessed by the abbreviated modification of diet in renal disease (MDRD) formula, RRT renal replacement therapy (dialysis, or kidney transplantation [KTx]), BMI body mass index; VAS, visual analog scale.
^aInitiation of enzyme replacement therapy within the first three months following the first visit.
^bHistory of transient ischemic attack (TIA), angina pectoris, significant cardiac procedure without coronary stent, arrhythmia.
^cHistory of stroke, myocardial infarction, coronary stent, coronary bypass surgery, ICD-implantation.
^dMedian pain according to the visual analog scale during the three months preceding the first visit in patients reporting pain.

ISSN: 1471-2369