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- Title
Kidney Function and Left Ventricular Mass in 51 Patients with Fabry Disease.
- Authors
Breunig, F.; Knoll, A.; Beer, M.; Weidemann, F.; Sandstede, J.; Strotmann, J.; Wanner, C.
- Abstract
Objective: Progressive renal disease and left ventricular (LV) hypertrophy demonstrating cardiac involvement are the most common clinical findings in Fabry disease. If there is a connection concerning the severity of cardiac and renal manifestation has yet to be demonstrated. Methods: We investigated 51 patients (25 females) with enzymatic and genetically confirmed Fabry disease before enzyme replacement therapy was initiated. Mean age was 44 ± 14 years (range 5-78). Glomerular filtration rate (GFR) was assessed by measurement of 99Tc DTPA clearance and 24 hour protein excretion was obtained. For cardiac evaluation left ventricular mass was determined by magnetic resonance imaging (MRI) in 39 patients. Following their GFR, patients were assigned to 4 different groups and LV-mass was correlated. Group I included patients with a GFR 0-30, group II 30-60, group III 60-90 and group IV <90 ml/min/1.73m². Results: Among 11 patients in group I, all male, 9 were on renal replacement therapy, 2 had a mean GFR of 23 ± 1 ml/min and a proteinuria of 6,575 and 2,033 mg/die respectively. In group II, with 3 male patients, mean GFR was 41 ± 7.5 ml/min and proteinuria was 2,536 ± 420 mg/die. The 12 patients in group III (6 females) had a mean GFR of 81 ± 6 ml/min and 5 of them showed proteinuria (1,186 ± 570 mg/ die). In group IV with 25 patients (19 females) averaged GFR was 116 ± 16 ml/min and a proteinuria (1,041 ± 650 mg/die) was present in 6 patients. LV hypertrophy was detected in 24 of 39 patients (14 males, 10 females). In group I, LV hypertrophy was present in 6 of 9 patients (66%, 237 ± 75 g), in group II in 3 of 3 (100%, 231 ± 37 g), in group III in 5 of 6 (83%, 167 ± 31 g) and in group IV 10 of 21 patients (47%, 146 ± 17 g) showed LV hypertrophy. Conclusions: In 51 investigated Fabry patients a reduced GFR was present in 26 (52%) and proteinuria in 16 (32%) patients. The amount of urinary protein excretion correlated with the stage of kidney function impairment. Also, coherence between the severity of kidney disease and cardiac involvement, demonstrated by LV mass, was shown.
- Subjects
KIDNEY diseases; HYPERTROPHY; ENZYMES; PROTEINS; PROTEINURIA
- Publication
Kidney & Blood Pressure Research, 2004, Vol 27, Issue 5/6, p300
- ISSN
1420-4096
- Publication type
Article