[The prevalence of Fabry's disease among male patients on hemodialysis in Lithuania (a screening study)]. / Fabry ligos paplitimas tarp hemodializuojamu ligoniu (vyru) Lietuvoje (atrankinis tyrimas).

UNLABELLED: Fabry's disease is an X-linked inborn error of glycosphingolipid metabolism caused by a deficiency of the lysosomal hydrolase alpha-galactosidase A. Due to deficiency of this enzyme activity, a progressive lysosomal accumulation of glycosphingolipids, in particular globotriaosylceramide, takes place within endothelial cells and cells of the vascular and nervous systems, myocardial cells, endothelial, and mesangial and epithelial cells of the kidney, eventually leading to organ dysfunction. The degree of renal involvement generally correlates with the progression of glycosphingolipid accumulation and may lead to renal insufficiency and failure. Renal dysfunction can progress to end-stage renal failure, which usually occurs in the third to fifth decade of life. The prevalence of this disease among males on chronic hemodialysis is different in various countries. Screening for alpha-galactosidase A deficiency by blood spot tests was performed among 536 male dialysis patients in all 42 hemodialysis centers in Lithuania in the period of April-June, 2005. All tests, showed normal galactosidase A enzymatic activity. CONCLUSION: No patient with suspicion of Fabry's disease was found by this screening method.

[Parallels in development of hemodialysis service and kidney transplantations in Lithuania during 1996-2005]. / Hemodializes tarnybos ir inkstu transplantacijos raidos paraleles Lietuvoje (1996-2005).

The aim of our study was to evaluate the changes in hemodialysis service, main demographic characteristics of hemodialysis patients in Lithuania during 1996-2005, and their correlation with the number of recipients on the kidney waiting list. During the study period, we annually visited all hemodialysis centers in Lithuania and collected data about all hemodialysis patients. There was a sharp increase in the number of hemodialysis centers (from 17 to 43), hemodialysis stations (from 25 to 100 per million population, P<0.001), hemodialysis patients (from 60 to 312 per million population, P<0.001), and new hemodialysis patients (from 54.3 to 95 per million population, P<0.01). The mean age of hemodialysis patients increased from 47.2+/-16.1 years in 1996 to 58.8+/-15.6 years in 2005 (P<0.001). Hemodialysis population became older. The percentage of patients aged more than 60 years increased from 22.8% to 53.2% (P<0.001) and aged more than 70 years from 5.4% to 24.4% (P<0.001). The frequency of chronic glomerulonephritis as underlying disease of end-stage renal disease decreased from 54.5% in 1996 to 21.1% in 2005 (P<0.001). There was an increase in the percentage of patients in whom end-stage renal disease was caused by diabetic (from 7.1% to 19.2%, P<0.01) and hypertensive nephropathies (from 3.1% to 13.9%, P<0.05) and chronic pyelonephritis (from 11.2% to 17.9%, P<0.01). The percentage of recipients on the kidney waiting list decreased from 71.4% in 1996 to 21.1% in 2005. In summary, during the last 9 years, hemodialysis service in Lithuania significantly expanded. The number of hemodialysis patients was continuously rising with predominance of diabetic, hypertensive, and elderly patients. Diabetic nephropathy, chronic glomerulonephritis, and pyelonephritis were the main underlying diseases of end-stage renal disease in hemodialysis patients in 2005. The percentage of recipients on the kidney waiting list decreased probably because of the rise in the number of elderly patients and patients with diabetes mellitus in Lithuanian hemodialysis population.

[Development of hemodialysis service in Lithuania during (1996-2003)]. / Hemodializes paslaugu raida Lietuvoje (1996-2003).

The aim of our study was to evaluate the changes of hemodialysis (HD) service and main demographic characteristics of HD patients in Lithuania during seven years period. From 1996 to 2003 we visited annually all HD centers in Lithuania and collected data about all HD patients. There was a sharp increase in the number of HD centers (from 17 to 37), HD stations (from 25 p. m. p. (per million population) to 87 p.m.p.; p<0.001), HD patients (from 60 p.m.p. to 264 p.m.p.; p<0.001) and incidence of new HD patients (from 54.3 p.m.p. to 92 p.m.p.; p<0.01). In 1996 all 17 HD centers in Lithuania were public. Private HD centers appeared in 1998 and reached 43.2% of all HD centers (n=16) in 2003. 44.8% of HD patients were dialyzed in private HD centers. The mean age of HD patients increased from 47.2+/-16.1 years in 1996 to 57.5+/-14.9 years in 2003 (p<0.001). HD population became older. The percentage of patients over 60 years old increased from 22.8% to 49.7% (p<0.001) and over 70 years old--from 54% to 21.9% (p<0.001). The main underlying disease of end-stage renal disease was chronic glomerulonephritis but its rate had decreased from 54.5% in 1996 to 26.5% in 2003 (p<0.001). During this period there was a statistically significant increase in the incidence of end-stage renal disease due to diabetics (from 7.1% to 18.0%; p<0.01), hypertensive nephropathy (from 3.1% to 9.4%; p<0.05), and chronic pyelonephritis (from 11.2% to 18.6%; p<0.01). In summary during the last seven years HD service in Lithuania expanded significantly, and rapid development of private HD was observed. The number of HD patients was rising continuously with predominance of diabetic, hypertensive and elderly patients.

[Rapidly progressive glomerulonephritis syndrome: course, pathomorphology and outcome (data of Kaunas University of Medicine Hospital 1996-2002)]. / Greitai progresuojancio glomerulonefrito sindromas: klinikine eiga, patomorfologija, gydymo veiksmingumas (Kauno medicinos universiteto kliniku 1996-2002 m. duomenimis).

Rapidly progressive glomerulonephritis is rare but severe clinical syndrome, which results in end-stage renal disease if not treated. The prognosis can be improved with early diagnosis and aggressive treatment. The aim of our study was to analyze retrospectively all cases of rapidly progressive glomerulonephritis, diagnosed in Kaunas University of Medicine Hospital since 1996 and to evaluate the clinical, laboratory, immunological, pathomorphological data, treatment and outcome in these patients. In period of 1996-2002, 23 patients were treated in Departments of Nephrology, Rheumatology, and Pediatric Nephrology. Rapidly progressive glomerulonephritis was observed in elderly persons, the mean age 46.48+/-5.28 years. In 21.74% of cases, patients were diagnosed Goodpasture syndrome, in 21.74% of cases immune complex disease and in 56.52%--pauci immune glomerulonephritis which was mainly limited in kidney as necrotizing crescentic glomerulonephritis. Rapidly progressive glomerulonephritis in most cases manifested by weakness, hypertension, fever, involvement of lower airways, infection. Renal biopsies were performed in 18 (78.26%) cases, lung biopsy was made for one patient. Biopsies were made on 30.56+/-7.41 day of hospitalization. Twenty-two (95.65%) patients received intravenous pulses with methylprednisolone and/or cyclophosphamide; 56.52% patients required dialysis. Renal function improved in 47.83% patients; 8 (34.78%) patients died. The causes of death were infections complications, progression of main disease and cardiac arrest.