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Biomedical subjects

G Asboe-Hansen

Publications and source records attributed to G Asboe-Hansen.

At least 19 recordsLinked to original sources
PubMed1979

Effect of hydralazine and dihydralazine on connective tissue and binding to serum protein.

Hydralazine and dihydralazine, chelators of Fe2+, Fe3+ and Mn2+ ions, inhibited collagen biosynthesis in organ culture of chicken embryo tibiae. Both substances inhibited the in vitro hydroxylation of [14C]Pro-labelled protocollagen by protocollagen proline hydroxylase. A decreased incorporation of [14C]-D-glucosamine into glyco- and/or muco-proteins was found under the effect of the two drugs. Dihydralazine, a cationic compound, was bound to DNA and acid mucopolysaccharides (glycosaminoglycans), forming an insoluble complex. Both substances were bound to serum alpha 2-globulin. Subcutaneous injections of dihydralazine in rats produced a local as well as a general toxic effect.

Animals↗
PubMed1979

[The dermoepidermal junction in skin diseases].

Dermatological conditions characterized by dermo-epidermal separation, basal lamina discontinuity, multiplication, and thickness variability, and/or irregularity of the subepidermal space are discribed. Pathological changes of the dermo-epidermal junction are considered to be destructive or reproductive. Both may appear in combination. Destructive processes may be relfected by dermo-epidermal separation. Epidermal cells and/or dermal connective tissue appear degenerated. Thickening of the lamina may occur by reactive hyperproduction or by precipitation of pathological materials. Reproductive processes of the junction originate in the epidermal cells and are reflected in multilayering of the basal lamina. Interruption of the lamina and irregularity of the subepidermal space often precede these phenomena.

Dermatitis↗
PubMed1979

Urinary proline to hydroxyproline ratio varies with age.

Urinary excretion of proline and hydroxyproline was studied in five groups of subjects, viz. controls, pituitary dwarfs, familial dwarfs, patients with fibrodysplasia ossificans progressive and patients with generalized scleroderma. The ratio Pro/Hyp in the urinary fraction precipitated with 5 parts of acetone (1 + 5 fraction) was close to one in children and youngsters in all the groups, while in adults it was higher than two. Total Pro/Hyp does not give an accurate index of age.

Adolescent↗
PubMed1979

Ultrastructure of skin in primary systemic amyloidosis.

Amyloid masses were found in the dermis of two brothers suffering from primary amyloidosis. The masses consisted of fibrils, composed in turn of twin hollow filaments of amyloid. An amyloid filament appeared as a 3 nm thick lucent core with a 2 nm thick wall. Occasionally 4--6 filaments were packed together in one fibril. The twin filaments were slightly twisted, with a twisting angle of 2.5 degrees and a coiling pitch of 1 160 nm. Wavy shapes of amyloid fibrils were also seen in elastic fibres. Amyloid fibrils were found in elastic fibres, under the basal lamina of the epidermis, sweat gland epithelium and Schwann cells; also around perineural cells, perivascular cells and, in one of the brothers, in collagen fibril bundles. No amyloid fibrils were found under the endothelial basal lamina. It would appear that amyloid fibrils are pathological fibrils belonging to the elastic fibre-basal lamina system.

Aged↗
PubMed1979

Treatment of generalized scleroderma: updated results.

Long-term treatment of patients with generalized progressive scleroderma by means of inhibitors of connective-tissue biosynthesis brings about total or subtotal regression of dermal sclerosis in 40.8%, partial regression in 33.1%, arrest of progression without regression in 14.8%, while in 11.3% it had no effect whatsoever. The drugs used were D-penicillamine, benzyl-penicillin-diethyl-aminoethylesterhydro-iodide, glutamine, hydralazine, chlorpromazine, L-dopa, diphenylhydantoin, and corticosteroids. Disease activity before, during and after treatment was indicated by the urinary fractions of high-molecular hydroxyproline and hydroxylysine containing peptides and of uronic acid, break-down products of collagen and acid glycosaminoglycans of connective-tissue ground substance. The prospects were better for young patients than for old, for those with a short history than for the longstanding disease cases, and for those having a large total dose than for those who had less. If left untreated, scleroderma progresses inexorably.

Chlorpromazine↗
PubMed1978

Fibrodysplasia (myositis) ossificans progressiva treated with disodium etidronate.

Two patients with fibrodysplasia ossificans progressiva (FOP) were treated with disodium etidronate, a disodium salt of ethane-1-hydroxy-1, 1-diphosphonic acid (EHPD) for two years; both had multiple ectopic ossifications of connective tissue. Regular follow-up showed no improvement in joint movement whilst laboratory examination revealed an increase in the serum phosphorus level. Serum calcium and urinary phosphorus excretion remained within normal limits. The urinary calcium excretion varied with the dose of EHDP. X-ray examination showed significant reduction in size and density of the ectopic ossifications, while the density of bones during and after EHDP therapy remained normal. Development of new ossification was inhibited in the active phase of the disease. Suspected side-effects of the drug were occult blood in stools, skin petechiae, and amenorrhoea. Previous reports on the EHDP treatment of disorders with ectopic ossification are summarised.

Adult↗
PubMed1978

Fibrodysplasia ossificans progressiva. Biochemical changes in blood serum, urine, skin, bone, and ectopic ossification.

Increased urinary output of total hydroxyproline, hydroxylysine and uronic acid was found in two patients suffering from fibrodysplasia ossificans progressiva. Before treatment of one of the patients, the high molecular weight peptide fraction deriving from newly synthesised collagen was particularly increased. Treatment with disodium etidronate (diphosphonate) reduced the values. The urinary values of sodium, potassium and calcium were also depressed during treatment, and, in both patients, serum phosphate was high, while serum calcium was normal. The hydroxyproline and hydroxylysine contents in skin and bone of FOP patients did not differ from controls, while ectopic ossifications showed a considerable increase in hydroxylysine in relation to normal bone.

Adolescent↗
PubMed1978

Effects of some connective-tissue active drugs on protocollagen proline hydroxylase activity.

The effect on protocollagen proline hydroxylase of drugs reported to be capable of inducing the lupus erythematosus syndrome and in some way acting on connective tissue and inflammation was studied in an in vitro system for the hydroxylation of 14C-Pro-labelled protocollagen. Some derivatives were also studied. It was found that benzoic acid and phenothiazine derivatives, hydroxyphenols, (+)catechin, beta-amino propionitrile, certain B vitamins, dihydrazinophthalazine, cysteine and dimethylcysteine were inhibitors of PPH. The chelation of Fe2+ions by the compounds mentioned is suggested to be essential.

Animals↗
PubMed1978

Effect of (+)catechin on connective tissue.

The effect of (+)catechin on connective tissue was studied in organ cultures of chick embryo tibiae and after in vivo injection in rats and mice. Collagen biosynthesis and hydroxylation of 14C-Pro-labelled protocollagen in vitro by protocollagen proline hydroxylase (PPH) were inhibited. In vivo, a slight decrease in the biosynthesis of collagen and in the PPH activity in skin of rats and mice was noticed as an effect of (+)catechin. No effect of the drug was observed on the incorporation of 14C-D-glucosamine by chick embryo tibiae. Increased urinary excretion of acid mucopolysaccharide metabolites by the mice injected with (+)catechin was observed.

Aminopropionitrile↗
PubMed1978

Abnormal skin collagen in scleroderma.

A significant decrease in the content of hydroxyproline and hydroxylysine was found in the skin of patients with generalized scleroderma (acrosclerosis), the lowering of Hyp being more marked than that of Hyl. The production of an abnormal collagen or a change from one collagen type to another is suggested to take place.

Collagen↗
PubMed1978

Hydroxyproline to hydroxylysine molar ratio indicates collagen type.

By using the molar ratio Hyp to Hyl, types I and II of collagen can be differentiated in 0.5 to 10 mg of dried, defatted tissue. Analyses on human skin, tendon, bone, aorta, cartilage, as well as nucleus pulposus, and annulus fibrosus of intervertebral discs are reported. Analyses of collagen of mesenchymal tissues of other vertebrates are also reported. From amino acid analysis of purified collagen samples published in the literature, the molar ratio Hyp/Hyl was calculated. Type I and type III collagen were differentiated from type II, and the latter was differentiated from type IV collagen. The molar ratios obtained with our analyses followed closely the values from previously reported amino acid analyses on purified collagen.

Amino Acids↗
PubMed1977

Fibrodysplasia ossificants progressiva.

Two cases of fibrodysplasia ossificans progressiva are reported. Both patients were females and had suffered from the disease since birth. The characteristic anomalies of great toes and thumbs associated with multiple ectopic ossifications of the connective tissue are described. Fasciae, tendons, ligaments, and joint capsules of the proximal parts of the extremities and the dorsal aspect of the trunk were involved. The muscle atrophy was probably a secondary phenomenon.

Adult↗