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Articles by G. E Linthorst
Total Records ( 2 ) for G. E Linthorst
  C Wanner , J. P Oliveira , A Ortiz , M Mauer , D. P Germain , G. E Linthorst , A. L Serra , L Marodi , R Mignani , B Cianciaruso , B Vujkovac , R Lemay , D Beitner Johnson , S Waldek and D. G. Warnock
 

Background and objectives: These analyses were designed to characterize renal disease progression in untreated adults with Fabry disease.

Design, setting, participants, & measurements: Data from the Fabry Registry for 462 untreated adults (121 men and 341 women) who had at least two estimated GFR (eGFR) values over a span of ≥12 months before starting enzyme replacement therapy were included.

Results: Most men (86 of 121, 71%) had more rapid loss of kidney function than the normal adult population (loss of eGFR > –1 ml/min per 1.73 m2 per year), whereas fewer women (133 of 341, 39%) had rapid loss of kidney function. Patients with rapid progression had significantly higher mean averaged urinary protein to urinary creatinine ratios (UP/Cr) than patients with slower progression (1.5 versus 0.2 for men; 1.4 versus 0.5 for women; P < 0.0001). Patients were grouped into quartiles based on averaged UP/Cr; renal function in men declined more rapidly with higher UP/Cr, with the steepest declines observed in men with UP/Cr > 1.5 (mean eGFR slope, –5.6 ml/min per 1.73 m2 per year; n = 30). eGFR slope declined more slowly in women, with the steepest declines observed in women with UP/Cr > 1.2 (mean eGFR slope, –1.3 ml/min per 1.73 m2 per year; n = 85). Regression models of eGFR slope indicated that UP/Cr is the most important indicator of renal disease progression in adult Fabry patients. In women, lower baseline eGFR and age were also associated with renal disease progression. Women who had clinical events had more rapid loss of kidney function.

Conclusions: Urinary protein excretion is strongly associated with renal disease progression in men and women with Fabry disease.

  R Schiffmann , D. G Warnock , M Banikazemi , J Bultas , G. E Linthorst , S Packman , S. A Sorensen , W. R Wilcox and R. J. Desnick
 

Background. In Fabry disease, progressive glycolipid accumulation leads to organ damage and early demise, but the incidence of renal, cardiac and cerebrovascular events has not been well characterized.

Methods. We conducted a retrospective chart review of 279 affected males and 168 females from 27 sites (USA, Canada, Europe). The pre-defined study endpoints included progression of renal, cardiac and cerebrovascular involvement and/or death before the initiation of enzyme replacement therapy.

Results. The mean rate of estimated glomerular filtration rate (eGFR) decline for patients was –2.93 for males, and –1.02 ml/min/1.73 m2/year for females. Prevalence and severity of proteinuria, baseline eGFR <60 ml/min/1.73 m2 and hypertension were associated with more rapid loss of eGFR. Advanced Fabry nephropathy was more prevalent and occurred earlier among males than females. Cardiac events (mainly arrhythmias), strokes and transient ischaemic attacks occurred in 49, 11, 6% of males, and in 35, 8, 4% of females, respectively. The mean age at death for 20 male patients was 49.9 years.

Conclusions. Baseline proteinuria, reduced baseline eGFR, hypertension and male gender were associated with more rapid progression of Fabry nephropathy. The eGFR progression rate may increase with advancing nephropathy, and may differ between subgroups of patients with Fabry disease.

 
 
 
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