Evolution of Fabry disease in male patients: the Greek experience.

Fabry disease is a progressive metabolic disorder with a clinical course characterized by different phases and a variety of disease manifestations. The first symptoms generally appear in childhood or early adolescence and are followed by late life-threatening complications involving vascular, renal, cardiac, and cerebral systems. We report the clinical and biochemical characteristics of 16 male patients from 10 unrelated families who represent almost the entire cohort of known Fabry patients in Greece. Despite the presence of early symptoms in almost every patient (mean age at onset of symptoms 15.6 years), the diagnosis was delayed for a mean of about 18 years (mean age of diagnosis 36 years). Patients are currently monitored and the majority (15 out 16 patients) treated with Enzyme Replacement Therapy.

High level of recovery of fungi from water and dialysate in haemodialysis units.

The counts of yeasts and filamentous fungi were investigated in the municipal water supplies of haemodialysis centres, in the treated water and the dialysate from all 85 haemodialysis units in Greece, in order to estimate their occurrence, their correlation with contamination indicator bacteria and other influencing factors. Filamentous fungi and yeasts were isolated from 69 (81.2%) and from three (3.5%) feed water samples, from 74 (87.1%) and seven (8.2%) treated water samples and from 66 (77.7%) and 11 (12.9%) dialysate samples respectively. Aspergillus spp and Penicillium spp were the most frequent moulds, while Candida spp were the prevailing yeasts. The occurrence of yeasts was significantly higher in dialysate than in tap water samples. Counts of filamentous fungi in all 255 samples were significantly correlated with the counts of total heterotrophic bacteria and enterococci, whereas the counts of yeasts were correlated with faecal coliforms, total heterotrophic bacteria, as well as enterococci, Pseudomonas spp and total coliforms, while no correlation was detected with the age of either haemodialysis units, the age of water treatment system, the number of artificial kidney machines or the components of the water purification system. High recovery of fungi from haemodialysis aqueous environments implies a potential risk for haemodialysis patients and indicates the need for continuous maintenance and monitoring.

Microbiological quality of water and dialysate in all haemodialysis centres of Greece.

BACKGROUND: Bacterial contamination of treated water and dialysate comprises an important problem for patients undergoing haemodialysis. Both the progressive reduction of the thickness of cellulose membranes and the expanding use of high-flux membranes probably enhance the risk of pyrogenic reactions, therefore increasing the need for atoxic water and non-pyrogenic dialysis fluid. METHODS: Samples of tap water, treated water, and effluent dialysate in all 85 haemodialysis centres in Greece were examined for total heterotrophic bacteria counts employing the pour plate method, total and faecal coliforms, faecal streptococci and pseudomonas spp. using the membrane filter technique, and sulphite-reducing clostridia applying the most probable number method. Overall 255 paired samples were tested from January to March 1997. RESULTS: For total heterotrophic bacteria, the overall compliance of treated water and dialysate to the American Association of Medical Instrumentation standards (<200 c.f.u./ml for water and <2000 c.f.u./ml for dialysate) was 92.6 and 63.7% respectively, whereas the compliance of tap water samples to our national standards (total heterotrophic bacteria < 10 c.f.u./ml and absence of the other indicator bacteria) was 80.7%. The most commonly isolated bacteria were pseudomonas spp., found in 22.2% of treated water and 59.5% of dialysate samples, whereas the respective frequencies were 12.3 and 36.2% for total coliforms, 8.6 and 30.0% for faecal coliforms, 14.8 and 28.7% for faecal streptococci, and sulphite-reducing clostridia were isolated in 5.8% of dialysate samples only. Haemodialysis centres equipped with storage tanks for treated water experienced lower levels of total heterotrophic bacteria, but higher counts of total and faecal coliforms, faecal streptococci, and pseudomonas spp., although the difference was statistically significant only for faecal streptococci counts, (P<0.05). Sixty-seven haemodialysis centres were equipped with bacterial filters, but mean values of all the examined microorganisms were not statistically different from those of the other centres. Faecal streptococci counts in treated water samples were positively correlated with ageing of both haemodialysis centres (P<0.005) and purification system (P<0.05), whereas pseudomonas counts were significantly correlated with ageing of the purification system (P<0.05).