Search PubMed⌕ Search

Biomedical subjects

G Lubec

Publications and source records attributed to G Lubec.

At least 217 records · Page 12Linked to original sources
PubMed1989

[Treatment of diaper rash with Parfenac lipid ointment (bufexamac). A study by Austrian pediatricians].

447 children between 0 and 24 months with diaper dermatitis were treated by topical application of Parfenac-Fettsalbe (Bufexamac). This panel was selected out of 844 patients which were originally in the protocol. The Parfenac-Fettsalbe was well tolerated, 7 children presented local side effects. 62.71% of treated girls showed very good results, whereas 59.89% of boys showed this outcome. Good results were obtained in 24.35% of girls and 21.93% of boys. Very good (no efflorescences remaining) and good results (some remaining efflorescences) were thus found in more than 80% of patients treated: with respect to corticosteroid advoidance this can be regarded as an effective antiphlogistic medication for diaper dermatitis. Non responders might have been superinfected with microorganisms as candida and staphylococci. In addition, epidemiological considerations are reported in that study.

Bufexamac↗
PubMed1988

Structural and functional changes in lung tissue of mice fed with beta-aminopropionitrile fumarate, L-3-cis-hydroxyproline, and L-4-cis-hydroxyproline.

Structural and functional changes in lung tissue of mice fed with beta-aminopropionitrile fumarate, L-3-cis-hydroxyproline, and L-4-cis-hydroxyproline. We fed 0.1% solutions of 3-cis-hydroxyproline (3cisHP), 4-cis-hydroxyproline (4cisHP) or beta-aminopropionitrile fumarate (beta APN) to 5-week-old mice for 1 month and studied the effect of each of these substances on the lung function and structure. Compared to control animals the compliance of the respiratory system in the mean was increased by 3-15% in the mice fed beta APN or 3cisHP, and decreased by 2-8% in the mice fed 4cisHP. On electron microscopial examination no overt morphological changes were detectable although as proven by biochemical analysis 3cisHP and 4cisHP were incorporated into the collagen of the mice who received these substances. We conclude that the effect of substances interfering with the normal production of collagen molecules depends on the kind of collagen affected and on the rate of collagen turnover.

Aminopropionitrile↗
PubMed1988

The determination of urinary 3-trans-hydroxyproline (3 OHP). II. Normal values in neonates, infants and preschool children.

3-trans-hydroxyproline is a hydroxyproline isomer present in collagens. It is more abundant in basement membrane collagen (collagen type IV) where it can be found in a relation of 12 per 1000 amino acid residues. This implicates its probable use as a marker substance for collagen type IV metabolism. Up to now only hydrolyzed urine samples were examined for the presence of this amino acid, thus indicating total urinary excretion. We are reporting the free urinary 3 OHP content of neonates, infants and preschool children. Neonates (n = 23) showed mean excretion of 0.363 micrograms/mg creatinine (SD +/- 0.127), SEM 0.026, min. 0.197, max. 0.598. Infants (n = 11) showed mean excretion of 1.501 micrograms/mg creatinine (SD +/- 0.468), SEM 0.141, min. 0.908, max. 2.553. Preschool children (n = 16) had a mean excretion of 1.442 micrograms/mg creatinine (SD +/- 0.637), SEM 0.159, min. 0.318, max. 2.342. As determined by statistical calculations, neonates differed from infants and preschool children significantly (p less than 0.0005). Infants did not differ from preschool children, however (p less than 0.4). The reason for the low urinary excretion of 3 OHP in the neonatal period could be explained by the low enzyme activities which are physiologically observed in this developmental period in general and in the special case of a presumably low activity of the 3-prolyl-hydroxylase. Infants and preschool children presented levels 5 times higher than their neonatal mates, probably reflecting the increased 3-prolyl-hydroxylase activity which in turn is a marker of collagen synthesis.

Child Development↗
PubMed1988

[Phenotype of mucopolysaccharidoses. A warning: mucopolysaccharidosis is not called gargoylism!].

In medical practice only mucopolysaccharidosis syndromes presenting with gargoylism are being diagnosed. It must however be mentioned that the most frequent mucopolysaccharidosis, Sanfilippo's disease, and other forms of mucopolysaccharidoses (MPSoses) are not showing gargoylism. Besides normal phenomenology "anthropoid" appearance is the prominent and most common--pithecoid--clinical feature. It is the aim of this publication to point to the fact that the appearance of children is in most of the cases with MPSoses nonpathognomonic and only few of them are undergoing further diagnostic steps concerning specific MPSoses diagnostic. This lead to the postulation that even in the presence of few clinical signs of mucopolysaccharidoses as psychomotor retardation, or anthropoid appearance, or hair changes, a screening must be performed. For this purpose the application of the toluidine blue test is not sufficient, as only quantitative changes are found by the use of this test. At least one dimensional or, better, two dimensional electrophoretic separation of urinary mucopolysaccharides should be performed. This procedure, if giving positive results, must be followed by enzyme studies. Following this scheme seems to be the only possibility to establish early and fair genetic counselling in order to cope with these fatal diseases.

Child↗
PubMed1988

Infrared spectroscopy studies on the conformation of human hair.

10 hair samples of healthy subjects and 8 hair samples of patients with mucopolysaccharidosis III A were examined by two techniques in the native state and hydrolyzed. For the hydrolyzed samples transmission spectroscopy was used, for the studies on native hair we applied the attenuated reflection technique. The main differences between native and hydrolyzed hair were at 1450, 2337, 2362, 2850, 2870, 2917, 2930, 3080 cm-1. Those wave numbers seem to reflect the supramolecular structure expressed by hydrogen bonding and intramolecular hydrogen bonds. Only the native hair showed those peaks. Other differences, but of quantitative nature only could be found in the infrared spectra. There were no differences between transmission and ATR spectra in patients with mucopolysaccharidose III A and healthy subjects, only the additional peak of the storage material was detectable. In this paper the supramolecular structure of hair on infrared spectroscopy is characterized, giving the basis for futural studies on the alterations in pathology and variations of human hair.

Child↗
PubMed1988

The determination of urinary 3-trans-hydroxyproline (3 OHP). I. Normal values in school children and adults.

3-trans-hydroxyproline is a hydroxyproline isomer present in collagens. It is more abundant in basement membrane collagen (collagen type IV) where it can be found in a relation of 12 residues per 1000 amino acid residues. This implicates its probable use as a marker substance for collagen type IV metabolism. Up to now only hydrolyzed urine samples were examined for the presence of this amino acid, thus indicating total urinary excretion. We are reporting the free urinary 3 OHP content of adults and school children. School children (age group 8-18 years) showed mean excretion of 1.066 micrograms/mg creatinine (SD +/- 0.376), SEM 0.054, min. 0.516; max. 2.397, range: 1.881. Adults (age 19-29 years) showed mean excretion of 0.618 micrograms/ml creatinine (SD +/- 0.465), SEM 0.134, min. 0.055; max. 1.634, range: 1.579. As given by the statistical calculation of unpaired t-test, adults showed significantly lower urinary 3 OHP levels: t = 3.518 and p = less than 0.0005. This can be explained by increased collagen metabolism of the growing organism and is in congruence with data reported in literature for total urinary 3 OHP excretion.

Adolescent↗
PubMed1987

Acrodermatitis chronica atrophicans in association with lichen sclerosus et atrophicans: tubulo-interstitial nephritis and urinary excretion of spirochete-like organisms.

We report about a 38-year-old male patient with coexisting acrodermatitis chronica atrophicans, lichen sclerosus et atrophicans and recurrent diabetic metabolic disorders since 9 years. Serologically IgG antibodies against Borrelia burgdorferi could be detected. Moveless winded structures, morphologically resembling borreliae could be demonstrated in the urine sediment by dark field microscopy. Additionally a tubulo-interstitial nephritis was diagnosed by the presence of a dysmorphic hematuria, a pathological polyacrylamide gel electrophoresis and raised alpha 1- and beta 2-microglobulin in the urine. We suggest that the excreted spirochete-like structures are borreliae. They may be the putative infectious agent for the development of lichen sclerosus et atrophicans in the genital area.

Acrodermatitis↗
PubMed1987

Determination of urinary low molecular weight proteins for the diagnosis of tubular damage.

The low molecular weight proteins alpha-1-microglobulin, beta-2-microglobulin and retinol binding protein were estimated in 32 healthy children and compared to 81 urine samples of children with tubular damage. Tubular damage was diagnosed clinically and established on polyacrylamide gel electrophoresis. Means for healthy children were: beta-2-microglobulin (b2m) 109 +/- 77 micrograms/l, alpha-1-microglobulin (alpha-1-m) 0.39 +/- 0 mg/dl, retinol binding protein (rbp) 0.49 +/- 0 mg/dl. Means for children with tubular damage were: b2m 16952 +/- 20057 micrograms/l, alpha-1-m 3.51 +/- 2.09 mg/dl and rbp 2.75 +/- 2.83 mg/dl. Comparing the groups using Student's t test revealed significant differences between healthy children and those with tubular disorders for each of the methods applied (b2m: t = 4.73, p = less than 0.0001, alpha-1-m: t = 8.43, p = less than 0.05, rbp: t = 4.5, p = less than 0.0001). The three methods did not correlate significantly to each other. Alpha-1-microglobulin showed tubular damage in 71 out of 81 patients, beta-2-microglobulin in 64 out of 81 and retinol binding protein in 41 out of 81 children with tubular disorders. The determination of b2m and alpha-1-m is useful for screening and diagnosis of tubular disorders in contrast to the estimation of the retinol binding protein. Cost/time effectiveness is being discussed.

Acidosis, Renal Tubular↗
PubMed1987

Value of SDS-polyacrylamide gel electrophoresis, small molecular weight proteins and alpha-1-acid glycoprotein for the diagnosis of tubular damage.

The low molecular weight proteins alpha-1-microglobulin, beta-2-microglobulin, retinol binding protein, alpha-1-acid glycoprotein and the SDS-polyacrylamide gel electrophoresis (SDS-PAGE) were estimated in 31 healthy children and compared to 81 urine samples of children with tubular damage (TD). The tubular damage was diagnosed clinically and established by beta-2-microglobulin determination. Means for healthy children were: beta-2-microglobulin 109 +/- 77 micrograms/l, alpha-1-microglobulin 0.39 +/- 0.0 mg/dl, retinol binding protein 0.49 +/- 0.0 mg/dl, alpha-1-acid glycoprotein 0.69 +/- 0.0 mg/dl. Means for children with TD were: beta-2-microglobulin 8530 +/- 14693, alpha-1-microglobulin 2.98 +/- 2.28 mg/dl, retinol binding protein 1.68 +/- 2.44 mg/dl and alpha-1-acid glycoprotein 2.79 +/- 3.44 mg/dl. Comparing the panel of healthy children with sick children (TD) we found the following: b2m t = 3.2, p = less than 0.0005, a1m t = 6.5, p = less than 0.0005, rbp t = 2.65, p = less than 0.0005, a-1-agp t = 3.4, p = less than 0.005. a1m revealed tubular damage in 77 out of 81 tubular disorders, the rbp in 25 out of 81 and alpha-1-acid glycoprotein showed tissue damage or inflammation in 33 out of 81 patients. SDS-PAGE revealed tubular damage in 46 out of 81 patients with TD. We can assay that alpha-1-microglobulin is a useful marker for tubular damage. The determination of retinol binding protein and alpha-1-acid glycoprotein is useless for this purpose. The SDS-PAGE, though a potent tool for the differential diagnosis between glomerular or tubular damage, alone is not a reliable assay for the diagnosis of TD.

Acidosis, Renal Tubular↗
PubMed1987

Chronobiology of urinary acid glycosaminoglycan excretion.

Acid glycosaminoglycans (AGAG, mucopolysaccharides) are major constituents of the extracellular matrix. They play a role in filtration and permeability processes, isolation and scaffolding. In childhood no circadian rhythms have been reported up to now. We examined 11 children testing the parameters of: urine volume, creatinine concentration and -excretion, glycosaminoglycan concentration and -excretion, the glycosaminoglycan/creatinine ratio, chondroitin 4 sulfate (Ch4S) in % of total AGAG, Ch4S in mg/2 hrs, heparan sulfate (HS) in % of total AGAG and HS in mg/2 hrs. The biorhythmicity of these data were processed by the cosinor system. We found significant circadian rhythmus for urine volume (acrophase at 1704 hrs, p = 0.05), creatinine concentration (acrophase at 0512 hrs, p = 0.01) and glycosaminoglycan concentration (acrophase at 0358 hrs, p = 0.005). The data presented can form the physiological basis for studies of biorhythmicity in several pathological states as e.g. diabetes and mucopolysaccaridoses.

Adolescent↗
PubMed1986

Noninvasive diagnosis of tubular damage by the use of the urinary chondroitin-4-sulfate/heparan sulfate ratio.

20 healthy children, 20 children with tubulointerstitial nephritis (TIN) and 20 children with glomerulonephritis in the active state were examined. Polyacrylamide gel electrophoresis of urinary proteins, beta-2-microglobulin excretion and acid glycosaminoglycan electrophoresis were performed. Polyacrylamide-gel electrophoresis showed in all the cases with TIN low molecular weight proteinuria, no bands were observed in healthy children and in all the cases of glomerulonephritis high molecular weight proteinuria. Beta-2-microglobulin determination showed no differences between healthy children and children with glomerulonephritis, but showed high levels in the group of children with TIN. Acid glycosaminoglycan electrophoresis showed in the group of children with healthy children a mean chondroitin-4-sulfate-/heparan sulfate ratio (CS/HS) of 3.8 +/- 0.4. Children with TIN presented a low ratio of 1.5 +/- 0.5. Patients with glomerulonephritis showed a mean ratio of 3.7 +/- 0.3. Our results clearly show that tubular damage can be revealed by a low quotient of chondroitin-4-sulfate to heparan sulfate.

Child↗
PubMed1986

Physicochemical hair conformation of patients with Sanfilippo disease type IIIA.

Sanfilippo disease type IIIA is an inborn error of metabolism with a deficiency in the heparan sulfamidase. Besides severe psychomotor retardation hair changes are obligatory. Hair is found to be coarse like a brush. We applied X-ray diffraction and infrared spectroscopy to characterize the conformation of hair samples of Sanfilippo patients. In healthy subjects as well as in the affected hair samples we found the wave numbers of structural relevance 1450, 1500, 1630, 1730, the pair 2337 and 2362, the quadruplet 2850, 2870, 2917, 2930 and 3080 cm-1. Also on X-ray diffraction analysis no differences could be detected. Though morphological-macroscopically and microscopically-changes were described for Sanfilippo hair samples, we could not find any change in supramolecular structure. The physical properties of coarseness of those hair specimen seems to be due to differences in the structural assembly of hair fibres and storage of heparan sulfate.

Child↗
PubMed1986

Interaction between laminin, fibronectin and the light chain of the H2 complex. Attachment of glomerular cells to basement membranes by linking the endoskeleton to the exoskeleton by a transmembrane protein.

At present the detailed mechanism for transmembrane interactions is not known and a protein linked to the endoskeleton as well as to the exoskeleton has not been described as yet. The H2 complex, a transmembrane protein, consists of heavy and light chains, the latter is named beta-2-microglobulin. In order to look for an association of beta-2-microglobulin with an exoskeleton protein, we examined the extracellular matrix proteins, collagen type I, type IV, fibronectin, amyloid P, the solubilized glomerular basement membrane and laminin in respect to their interaction with the light chain. The heavy chain is known to bind strongly to the endoskeleton protein actin. Only laminin and the glomerular basement membrane bound firmly to the beta-2-microglobulin; 3 M urea was necessary to dissociate the formed complex. Incubation with beta-2-microglobulin antibody prevented binding of beta-2-microglobulin to laminin and the glomerular basement membrane on affinity chromatography columns. Antiserum to the glomerular basement membrane in turn prevented binding of beta-2-microglobulin to the glomerular basement membrane, whereas antibodies against the basement membrane collagen type IV failed to inhibit this binding to the glomerular basement membrane. Beta-2-microglobulin also bound to fibronectin but this complex was dissociated with 1 M urea. In a rosette assay beta-2-microglobulin antibody and antiserum to the glomerular basement membrane reduced attachment of glomerular cells to beads coupled with laminin and solubilized glomerular cells to beads coupled with laminin and solubilized glomerular basement membrane.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1986

Periodicity of urinary beta-2-microglobulin excretion.

Using the cosinor system we examined the chronobiology of urinary beta-2-microglobulin excretion. The following dimensions were studied: urinary volume, creatinine concentration, creatinine excretion, beta-2-microglobulin concentration, beta-2-microglobulin excretion and the beta-2-microglobulin/creatinine ratio. Urinary volume showed an acrophase at 1704 hours (p = less than 0.01), creatinine concentration showed an acrophase at 0512 hrs (p = less than 0.006) and the beta-2-microglobulin/creatinine ratio presented an acrophase at 1736 hrs (p = less than 0.03). Data were related to a tau = 24 hrs. The data clearly reveal that the beta-2-microglobulin/creatinine ratio shows a circadian rhythm in urinary excretion.

Adolescent↗
PubMed1985

Structural changes of hair after incorporation of the proline analogue L-azetidine-2-carboxylic acid. A model of hair disease by alteration of primary structure.

In order to correlate biochemical changes of the hair with physical properties we present a model for the examination techniques. L-azetidine was incorporated into the hair keratin complex and the resulting mechanoelastic properties were determined using the ultramicrohardness testing system on scanning electron microscopy. Structure was investigated by X-ray diffraction and incorporation of L-azetidine was detected by thin-layer chromatography. This system could possibly be introduced for examination of hair changes in humans. 8 white mice, 3 weeks of age, were given L-azetidine-2-carboxylic acid in water (0.1 g/100 ml) as only source of fluid over a period of 5 weeks. They had free access to dry mouse cake only. 8 animals of the same strain, who had free access to tap water and mouse cake and were kept under the same conditions, served as controls. After 5 weeks, the animals were sacrificed and hair was obtained for analyses. 2 dimensional thin-layer chromatography of hair hydrolyzed with 6N HCl at 105 degrees C for 12 hours revealed 2 additional spots in the chromatographic pattern in the experimental animals in comparison with the control group. 1 of the spots was identified as L-azetidine-2-carboxylic acid, while the second spot was possibly a degradation product of L-azetidine on acid hydrolysis at a high temperature. Hair of the animals was put into Mark capillaries and subjected to X-ray diffraction, which showed a markedly disordered orientation of keratin. Impression studies using scanning electron microscopy revealed a remarkably reduced elasticity of hair with incorporated L-azetidine. These findings may be explained on the basis of qualitative or quantitative changes in the helical structure of the keratin complex of hair, which is responsible for the elastic properties, whereas the globular matrix is responsible for the firmness of the hair.

Animals↗