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Biomedical subjects

L Monnens

Publications and source records attributed to L Monnens.

At least 91 records · Page 5Linked to original sources
PubMed1980

Disturbances in bile acid metabolism of infants with the Zellweger (cerebro-hepato-renal) syndrome.

The bile acid pattern in bile and serum from two infants with the cerebro-hepato-renal syndrome of Zellweger was severely disturbed. An increased concentration particularly of trihydroxycoprostanic acid and also of dihydroxycoprostanic acid could be demonstrated. A generalized mitochondrial defect could explain these increased concentrations. This hypothesis is supported by the abnormal structure of the mitochondria in the liver biopsy of one of our patients. It is possible that the abnormal bile acids contribute to the liver damage of infants with Zellweger syndrome.

Bile Acids and Salts↗
PubMed1980

The complement system in hemolytic-uremic syndrome in childhood.

A dynamic estimation of the complement system was obtained by immunochemical estimation of C3, C4, C5, C1q, C3b + C3c, C3d, Ba in children with hemolytic-uremic syndrome. The presence of increased breakdown products of C3 (C3b + C3c, C3d) and of factor B (Ba) suggests an activation of the complement system possibly by the alternative pathway. No definite explanation for these complement abnormalities can be given.

Child↗
PubMed1979

Development of glomerular fibrinolytic activity in the rat.

Fibrinolytic activity of the glomeruli was studied in rats of different age using the fibrin-slide technic. In normal rats glomerular fibrinolytic activity (GFA) was relatively low. After a single injection of liquoid (sodium polyanethol sulfonate), a pronounced increase of GFA was evident. The superficial nephrons had a significantly higher GFA than the juxtamedullary nephrons. Compared with rats 2 months of age, rats 2 weeks of age showed a greater GFA in all the glomeruli.

Aging↗
PubMed1976

Functional and morphologic maturation of superficial and juxtamedullary nephrons in the rat.

The development of single nephron glomerular filtration rate (SNGFER) was studied in both superficial and juxtamedullary nephrons in rats in relation to concomitant morphologic maturation. These experiments were carried out in rats between 23 and 91 days of age (between 36 nad 275 g body weight) with the [14C]ferrocyanide infusion technique. 2. SNGFR of the superficial and juxtamedullary nephrons increased with body weight, glomerular volume and proximal tubular length. 3. The ratio SNGFR of the superficial (S) nephrons/SNGFR of the juxtamedullary (JM) nephrons rose from 0-60 in the 40-60 g rats to 0-84 in the adult rats, demonstrating the centrifugal functional maturation of the nephrons. 4. The S/JM ratio for both glomerular volume and tubular length was constant and averaged 0-72+/--0-12 and 0-81+/-0-05, respectively, indicating that while the increase in SNGFR was greater for S than for JM nephrons, this was not accompanied by concomitant disproportionate increases of glomerular volume and/or proximal tubular length between these nephron categories during development in the rat.

Age Factors↗
PubMed1976

Chemical properties of glomerular basement membrane in congenital nephrotic syndrome.

Glomerular basement membranes have been isolated from kidneys of three children presenting with a congenital nephrotic syndrome. The histology of renal tissue was characterized by an increase of the mesangial area mainly due to matrix. Immunofluorescent studies showed no positive staining. After isolation, the glomerular basement membranes contained no more cellular contaminants than normal membranes. Isolated abnormal membranes exhibited, as normal preparations, less than 1% DNA, RNA, phospholipids or glycosaminoglycans. Since the protein content of both membrane preparations was comparable, significant contamination by non-proteic material did not seem likely. Although the amino acid and carbohydrate composition of both normal and abnormal membranes was very similar, the folowing statistically significant differences were observed: diseased membranes exhibited an increased number of hydroxylysine, 3- and 4-hydroxyproline residues, and an increased content of glucosyl-galactosyl-hydroxlysine units. The data suggest that, in the congenital nephrotic syndrome, the production of disaccharide-rich glomerular basement membrane subunits is enhanced, which could interfere with the packing of the peptide chains and therefore account for the altered permeability of the membrane observed in this disease.

Amino Acids↗