[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.

[Dynamics of diabetic nephropathy and other complications of type I diabetes mellitus in the period of 1996-2002 (data from 2 Kaunas outpatient polyclinics)]. / Diabetines nefropatijos ir kitu cukrinio diabeto komplikaciju pokyciai 1996-2002 metais (dvieju Kauno miesto pirminiu sveikatos prieziuros centru duomenimis).

Kidney involvement in diabetes mellitus has negative impact on the outcomes of disease. Strong relationship between progressive diabetic kidney disease and the development of other diabetic complications was found by many investigators. In order to evaluate the dynamics of diabetic nephropathy in type I diabetes mellitus during 6-year period and its relationship with other diabetes mellitus complications and control of glycemia and hypertension, in 2002 we reviewed ambulatory case records of patients, who were followed by endocrinologists and who were investigated by us in 1996. During 6-year period, 5.1% from 156 pts. died and all of them had diabetic nephropathy; 26.9% of pts. moved to general practitioners and never visited endocrinologists again. Only 105 pts. remained under follow-up by endocrinologists. Their mean age 37.6+/-1.3 yrs. Out of all patients, 54% were males and 46% females. Mean diabetes mellitus duration was 19.5+/-0.9 yrs. Control of glycaemia was poor and insufficient in 2/3 of pts. HbA(1C) wasn't checked in 68.9% of pts. Control of arterial hypertension became better, but not sufficiently. During 6-year period persistent proteinuria developed in 12.1% of pts., who had no or transient proteinuria <0.5 g/l in 1996. Persistent proteinuria developed 19.9+/-1.8 yrs. after the diabetes mellitus onset and correlated with hypertension and renal insufficiency. Higher level of proteinuria was associated with worse control of glycemia. Progression of diabetic retinopathy and neuropathy over 6 yrs. were more expressed than in diabetic nephropathy. On average retinopathy developed after 14+/-1.8 yrs. after the diabetes mellitus onset, neuropathy--17.8+/-2.2 yrs., renal failure--21.1+/-2.8 yrs., heart failure--22.9+/-1.9 yrs. and arterial hypertension--12.1+/-1.3 yrs. The prevalence and time of incipient diabetic nephropathy appearance remained unknown because the test for microalbuminuria was not available in the primary health care centres.