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Biomedical subjects

G Lubec

Publications and source records attributed to G Lubec.

At least 289 records · Page 16Linked to original sources
PubMed1979

[Excretion of acid mucopolysaccharides in the urine and sweat in hereditary bullous epidermolysis].

Urine samples of five patients with epidermolysis bullosa hereditaria were examined for the presence of acid glycosaminoglycans (GAG) and compared to 10 healthy subjects. A significant increase (p less than 0.0005) could be detected in patients with epidermolysis (t = 6.66, means: 49.7 mg/day, standard deviation: +/- 18.3). Additionally, acid glycosaminoglycan concentration in sweat of patients and healthy controls was determined but statistical calculations showed no significant difference (p less than 0.15). On grounds of the increased mucopolysaccharide excretion and the previous studies of collagen-glycosaminoglycan interactions, we strongly suggest that acid GAG are involved in the pathogenesis of that disease with the molecular defect of disturbed fibril formation due to the altered skin collagen-glycosaminoglycan equilibrium.

Adolescent↗
PubMed1979

Intracellular phenylalanine and tyrosine concentrations in 19 heterozygotes for phenylketonuria (PKU) and 26 normals. Do the higher values in heterozygotes explain their lowered intellectual level?

Intracellular phenylalanine and tyrosine was determined in lymphocytes of 10 heterozygotes (parents) for PKU and in 26 randomly collected apparently normal persons. In cells from the heterozygotes the concentrations of both phenylalanine and tyrosine were higher than in those from the normals, the difference being statistically highly significant. It is argued that this could be responsible for the slight, though statistically significant, intellectual inferiority of heterozygotes for PKU.

Female↗
PubMed1979

[Significance of alpha 1-acid glycoprotein for the diagnosis of stomach cancer (author's transl)].

In 41 patients with benignant and malignant diseases of the stomach the appearance of alpha 1-acid glycoprotein (alpha1sGP) in the gastric juice was recorded quantitatively by radial immunodiffusion and compared with normal serum alpha1sGP by immunelectrophoretic analyzation and by immunodiffusion after after Ouchterlony. In 80.0% of the patients with carcinoma, in 11.8% of patients with gastric or duodenal ulcer and in 28.6% of the patients with gastritis the presence of alpha1sGP could be evidenced in the gastric juice. The appearance of this glycoprotein significantly differed in benignant and malignant diseases of the stomach. A polymorphism of the stomach alpha1sGP compared with normal serum alpha1sGP could not be established by the applied methods. Therefore it is unprobable that the gastric alpha1sGP is originated by the malignoma.

Diagnosis, Differential↗
PubMed1979

In vitro evidence of cellular and humoral immune responses following lung allotransplantation in canine recipients with and without immunosuppressive treatment.

Using donor lung antigens as targets, two test methods, a rosette-forming cell test (RFC) and indirect immunofluorescence test (indir. IF), were applied to focus on the first set lung allograft rejection in canine recipients with and without immunosuppressive treatment. In both groups the first sign of humoral or cellular sensitization became evident on the 4th postoperative day, which might answer some unsolved problems in early lung graft damage, recently discussed as a manifestation of certain immunologic reactions. The tests used, indir. IF and RFC, revealed a steadily climbing tendency of immunization in untreated recipients and more variable and reversible positive results in the immunosuppressively treated group. No correlation between cellular and humoral immunity could be proven by our tests. The fact that we found no incorrect positive results in RFC suggests that this test provides important hints for the diagnosis of lung allograft rejection.

Animals↗
PubMed1979

Collagenase activity of rat kidney with glomerulonephritis is inhibited by erythromycin.

Collagenolytic activity of rat kidney with glomerulonephritis (GN) of Masugi's type was determined by a series of biological experiments. The determination was carried out during the heterologous phase of the disease together with inhibition studies in vivo and in vitro. Erythromycin has been tested in vivo for its inhibitory activity and an activity of collagenase was found in untreated rats with GN only, the panel treated with erythromycin did not show any collagenolysis. Statistical calculations indicate the significance of the inhibition (p less than 0.001). In addition, kidneys of test animals with GN have been investigated for their enzymatic activity and the inhibition by erythromycin in vitro where the kidneys failed to show collagenolytic activity. It can be concluded that erythromycin is able to inhibit in vivo and in vitro the activity of the collagenolytic enzyme produced by polymorphonuclear leukocytes during the heterologous phase of the nephritis, an effect which leads to the recovery of the physiological equilibrium of the protease and its inhibitor.

Animals↗
PubMed1979

Donor- and organ-specific evaluation of antibodies eluted from canine lung allografts rejected by immunosuppressively treated and untreated recipients.

Using elution techniques, the humoral lung allograft rejection in immunosuppressively treated versus untreated recipients is analyzed at the donor organ specific level. The antibodies were evaluated quantitatively and qualitatively by elution of lung graft bound immunoglobulins and by testing the eluates against donor lung antigen using passive hemagglutination and indirect immunofluorescence. By correlating the results in those assays, a considerable amount of humoral anti-donor lung antibodies could be proved only in dogs not treated immunosuppressively, and there was no accordance with the results of direct immunofluorescence by quantification. The difference in the organ-bound antibodies between immunosuppressively treated and untreated lung grafts seems to be remarkable because of a similar mononuclear infiltration. Thus, enabling a specific improvement of some previous speculations about the lung-specific humoral alloimmune reaction, this type of rejection seems to be similar to rather stereotypical allograft rejection, but may be modified by a standard immunosuppression with methylprednisolone and azathioprine.

Animals↗
PubMed1979

[5-fluorouracil inhibits bacterial collagenase and the collagenolytic system of a human rhabdomyosarcoma].

Protease inhibitory activity of eight cytostatic drugs on 5 proteases was tested by applying an immunoelectrophoretic system, a rhabdomyosarcoma's collagenolytic activity was investigated and inhibition studies were performed. We found 5-fluorouracil stopping collagenolytic activity of clostridial origin and the enzyme released by the tumor. No other cytostatic drug showed protease inhibition. The collagenolysis of the rhabdomyosarcoma was examined for its origin. Negative inhibition studies with ethylene-diamine-tetraacetate make the leukocyte origin highly improbable so that one can suggest that the hydrolytic enzyme derived from the tumor. Our findings could contribute to characterize the enzyme released by the tumor. We suggest its role as a mediator of invasion and metastasis and on the other hand we detected its relatively specific inhibitor, 5-fluorouracil, which could influence tumorous and normal growth, regeneration and the intermediary protein metabolism.

Cells, Cultured↗
PubMed1979

[Enzyme induction by Epstein-Barr-virus-producing infection of lymphoblastoid cell lines].

By means of biological in vitro assay collagenolytic activity of normal peripheral lymphocytes was compared to that of EBV-infected lymphoblastoid cell lines: the EBV-producing P3HR1 cells and the non-productive Raji cells. While normal lymphocytes and Raji cells revealed only traces of activity, significantly increased (p less than 0.001) collagenolytic activity was detected in samples of P3HR1, the cell-free EBV-suspension alone showed no collagenolysis. The described enzymatic activity was fully inhibited by 0-phenanthroline and EDTA indicating collagenase action. As main explanations for the phenomenon we suggest the possibility of viral enzyme induction, increased release of protease and thirdly that EBV carries a gene for synthesis of a collagenase.

Burkitt Lymphoma↗
PubMed1979

Immunochemical characterization of mature and immature glomerular basement membrane.

20 kidneys from premature infants (27th to 38th weeks of gestation), 5 kidneys of mature newborns and 5 kidneys of children between 5 and 15 years of age were obtained at necropsy and glomerular basement membranes (GBM) isolated. The isolated GBMs were degraded by papain and the degradation products were characterized by immunoelectrophoresis applying an antihuman GBM antiserum from the rabbit. GBMs of children between 5 and 15 years showed in each case 3 precipitation lines distributed from the alpha-gamma-zone. The examined newborns and 17 premature infants presented two precipitation lines only, moving with alpha 1-mobility and beta-gamma-inter-region. 3 premature infants showed a pattern with 2--4 different precipitation lines of different mobility, maybe interpretable as a result of bacterial digestion. On the grounds of these findings we postulate that the GBM is, from an immunochemical point of view, immature at birth and in the late fetal life becoming mature in the child with about 5.

Adolescent↗
PubMed1979

Urinary excretion of glomerular basement membrane antigens in premature infants and the newborn.

25 premature infants, 8 mature newborns and 25 children between 5 and 15 years of age were examined for urinary excretion of glomerular basement membrane (GBM) antigens. For the characterization of the excreted GBM antigen, immunoelectrophoresis was applied. In the group of 25 premature infants 23 showed alpha-1-mobility, in the group of 8 mature newborns all showed alpha-1-mobility, and in the group of the 25 children aged 5--15 years 24 showed migration into the alpha-2-zone. Differentiating, whether the difference between the immature and mature GBM is quantitative or qualitative, the immunoelectrophoretical difference points to the interpretation that the premature GMB shows a unique chemical composition.

Adolescent↗
PubMed1979

[The effect of collagenase inhibition on bullous eruption and healing process in epidermolysis bullosa].

The collagenase inhibiting effect of erythromycin already observed in vitro was demonstrated also after oral administration of the drug in vivo in one child with epidermolysis bullosa letalis and one child with epidermolysis bullosa dystrophica. Despite inhibition of skin collagenase activity during administration of the drug the frequency of bullous eruptions and healing process of affected skin areas remained unchanged. This suggests no direct causal relation existing between skin collagenase activity and epidermolysis bullosa. Whether increased collagenase activity reflects a secondary reaction of the organism cannot be concluded from this study.

Child↗
PubMed1978

[In-vitro inhibition of collagenase activity in epidermolysis bullosa hereditaria dystrophica].

By means of a biological collagenase assay we estimated collagenolytic activity of the vesicular fluid of patients with epidermolysis bullosa hereditaria dystrophica. All the 5 examined patients showed increased collagenolysis. Inhibition studies were performed applying ethylendiaminetetraacetate inhibition 11,15%, normal human serum in 12.15% (means). o-Phenanthroline as well as erythromycine in an aequimolar ratio stopped the collagenolytic activity completely in the 5 specimen. It was the aim of our study to indicate that erythromycine, in contrast to other collagenase inhibitors relatively atoxic, is able to inhibit collagenase activity which is suggested to play a pathogenetic role in that disease.

Adult↗
PubMed1978

[Mucopolysaccharidosis V (Ullrich-Scheie syndrome) (author's transl)].

Mucopolysaccharidosis V (Scheie's syndrome, MPS-IS) is a very rare, autosomal recessively inherited metabolic disease. The degradation of dermatan sulphate and heparan sulphate is disturbed due to alpha-L-iduronidase deficiency, leading to intracellular storage and excessive urinary secretion of these substances. The characteristic clinical features are contractures (claw-like flexion of the fingers), umbilical and inguinal herniae, corneal opacity, hepatomegaly, myocardiopathy and minor skeletal malformations. A patient with Scheie's syndrome is now reported for the first time in Austria; the results of the clinical, biochemical, chromosomal, dermatoglyphic and electron optical investigations are described and discussed.

Adolescent↗
PubMed1978

Complement in cystic fibrosis.

Complement components C3, C4, and C3A were estimated in 30 patients with cystic fibrosis aged 1 to 21 years (M:F = 16:14) and were compared with results in 40 healthy, age-matched subjects. The influences of the clinical score, sputum microbiology, and the patients' sex were also investigated. In contrast to most previous communications, this paper shows that, compared to the control group, a significant decrease of C3 (P less than 0.001) and C4 (P less than 0.02) was observed whereas C3A levels were not altered. There were no increases in complement. Shwachman-scores above or below 70 did not influence the complement levels, nor did exacerbations of the disease change the levels. No influence of the patients' sex could be shown. Pseudomonas aer. in the sputum was clearly associated with complement defects (14/18). Alternative-pathway involvement of complement activation could be demonstrated in 32%. The results make complement activation due to pulmonary infection most likely. The defects observed probably represent secondary changes.

Adolescent↗
PubMed1978

Collagenase activity of rat kidney with immune complex glomerulonephritis.

By means of a biological assay the collagenolytic activity of kidneys from rats with experimental immune complex glomerulonephritis and control animals was tested. We detected collagenolysis in both panels but found quantitative differences in the collagenase activity. The enzymatic collagenolysis was significantly increased (P less than 0.001) in the nephritic kidneys. From the fact that the chelating agent EDTA inhibited potentially the released enzyme it can be concluded that the origin is the polymorphonuclear granules.

Animals↗
PubMed1978

Urinary excretion of glomerular basement membrane antigens in Alport's syndrome. A new diagnostic approach.

Alport's syndrome is defined by the combination of hereditary nephropathy and neurosensory deafness, and is diagnosed from the family history combined with renal electron microscopy. Immunoelectrophoresis of the urine of 8 of 12 children suspected of Alport's syndrome showed a precipitation line moving into the beta-zone, applying an antiglomerular basement membrane antibody derived from an immunised rabbit. All patients who showed the typical pattern of Alport's syndrome on renal electron microscopy were among the 8 cases whose urine gave this immunoelectrophoresis pattern. Additionally, 5 of the mothers of the 8 children excreted the same antigen in their urine. The urine of 30 healthy children and of 10 patients with the idiopathic nephrotic syndrome did not show the presence of this antigen. This characteristic sign of Alport's syndrome may therefore be useful for its detection.

Adolescent↗
PubMed1978

[5-fluorouracil inhibits the collagenolytic activity of invasive colonic adenocarcinomas in vitro].

By means of a biological assay for the enzyme collagenase the activity of this protease was examined in 12 invasive adenocarcinomas of the colon. In spite of considerable quantitative differences collagenolytic activity in the dimensions of 10(-3) units/mm theta tissue could be revealed in all cases. EDTA inhibited the reaction by 19%, normal human serum by 23% in the average, whereas 1,10-0-phenanthroline, D-penicillamine as well as the cytostatic 5-fluorouracil resulted in nearly total inhibition. It can be suggested that the activity of the colonic carcinoma is not derived from granulocytes, serum, or normal mucosa, but from the tumor itself. Contrasting to the limited inhibition by normal serum inhibitors, the enzyme is nearly fully inhibited by 5-fluorouracil.

Adenocarcinoma↗