Clinical,biochemical and histological analysis of seven patients with cholesteryl ester storage disease

Lysosomal acid lipase (LAL) deficiency leads to two phenotypically different diseases: cholesteryl ester storage disease (CESD) and Wolman's disease. Lysosomal acid lipase hydrolyzes cholesteryl esters and triglycerides. Deficiency of LAL results in intralysosomal storage of cholesteryl esters and triglycerides. CESD has a chronic and benign course and is characterized by hepatomegaly and mild hypercholesterolemia. It leads to fibrosis (cirrhosis) and early atherosclerosis. This report presents the clinical, biochemical and microscopic data of seven patients with CESD followed up over 10 years. The physical development of all the study children remained within the normal range; 7 patients had hepatomegaly and 6 also had splenomegaly. Three patients had normal cholesterol, triglycerides and transaminases values; the other four had slightly elevated levels for these parameters. The activity of LAL in all patients was reduced to below 30% of the lower normal value. Histologically, cholesteryl crystals and lipid storage vacuoles in Kupffer cells were present in all examined patients except one. Accumulation of cholesteryl esters was visible on thin-layer chromatography of lipid extracts obtained from liver biopsies.     

Analysis of Circadian Distribution of Premature Ventricular Contractions in Patients After Heart Transplantation

The aim of our study was to analyze circadian distribution of premature ventricular contractions (PVC) and its coupling interval (CI) in patients after orthotopic heart transplantation (HTx). Forty-two patients (5 females, 37 males) were monitored from 2 weeks to 5 years after HTx; 180 24-hour Holter ECG studies were performed. All recordings were divided into two groups: group I, within 1 month after HTX; and group II, after 1 month. Patients with more than 250 PVC/24 hours were selected for distribution of PVC and CI evaluation. Conclusions : Ventricular arrhythmias occur frequently in patients after heart transplantation. In patients with high Lown scale arrhythmias low occurrence (< 250/24 hours) of PVC was frequently observed (IVa: 81.8%; IVb: 84.7%). Similar patterns of circadian distribution (CD) of PVC and CD of HR in denervated heart after HTx suggest the influence of circulating catecholamines on their occurrence.     

Influence of the peptide chain length of new elongated tuftsin analogs on phagocytosis process

In this paper the synthesis of the following elongated tuftsin analogs: Arg-Thr-Lys-Pro-Arg (1), Pro-Arg-Thr-Lys-Pro-Arg(II), Lys-Pro-Arg-Thr-Lys-Pro-Arg(III) and Thr-Lys-Pro-Arg-Thr-Lys-Pro-Arg(IV) by classical and solid-phase methods are described. The obtained peptides were tested for their biological activity: restoration of the phagocytosis of defected granulocytes from blood of children with acute lymphoblastic leukemia (ALL).