RESUMEN
The role of lipid abnormalities has been also implicated in the progression of renal diseases. The search for lipid abnormalities in Balkan endemic nephropathy (BEN) has roused sporadic interest and has not been fully elucidated. This study was performed in 54 healthy subjects from the families affected with BEN (group A), 18 members from non-affected families living in the same location (group B), and 25 control subjects (group C). Lipid profiles and lecithin:cholesterol acyltransferase (LCAT) were determined in each subject. The most striking distinction between the groups was that of the LCAT activity, which was abnormally low in group A (39 +/- 2), significantly different (P less than 0.0001) from that of the other groups. Thirty individuals from group A were those accounting for the low LCAT activity (A1). This group had a significantly lower total cholesterol and free cholesterol than all of the other subjects. The entire group A subjects had a significantly lower percentage of free cholesterol than the other two groups. There was no significant difference in HDL cholesterol between any of the groups, but group A1 had significantly higher HDL than group C (P less than 0.04). What emerges from our study is that a certain proportion of subjects from BEN families have a peculiar form of lipid abnormalities associated with an abnormal LCAT activity. At present we have no explanation for these findings. We believe that these changes may have an important role in the pathogenesis of BEN.
Asunto(s)
Nefropatía de los Balcanes/enzimología , Deficiencia de la Lecitina Colesterol Aciltransferasa/complicaciones , Adulto , Nefropatía de los Balcanes/sangre , Nefropatía de los Balcanes/etiología , Colesterol/sangre , HDL-Colesterol/sangre , Humanos , Deficiencia de la Lecitina Colesterol Aciltransferasa/sangre , Persona de Mediana Edad , Triglicéridos/sangreRESUMEN
The aim of this study was to investigate serum levels, urinary excretion and in vitro peripheral blood mononuclear cell (PBMC) production of beta 2-microglobulin in patients with Balkan nephropathy and their families. Increased urinary beta 2-microglobulin excretion was found in Balkan nephropathy, chronic pyelonephritis and glomerulonephritis patients, being highest in the first group. The serum level of beta 2-microglobulin in Balkan nephropathy patients correlated with residual kidney function. Synthesis of beta 2-microglobulin by PBMC, untreated or stimulated by PHA, was not increased in Balkan nephropathy patients or their healthy family members compared to the control group of healthy persons living outside of an endemic region. This study has shown that the increased serum beta 2-microglobulin level in Balkan nephropathy patients is the consequence of the glomerular filtration rate (GFR) reduction. Urinary beta 2-microglobulin excretion was found increased not only in patients but in some healthy members of nephropathic families. beta 2-microglobulin therefore can serve as a marker of the early tubular damage in Balkan nephropathy. However, urinary beta 2-microglobulin is not specific for Balkan nephropathy, lacking specificity required for screening purposes. The different patterns of serum and urinary beta 2-microglobulin, and other urinary proteins, in patients with Balkan nephropathy from patients with chronic pyelonephritis and glomerulonephritis favor the opinion that Balkan nephropathy is a separate clinical entity.