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Biomedical subjects

H Cordes

Publications and source records attributed to H Cordes.

9 recordsLinked to original sources

Short-term study of the uptake of PrP(Sc) by the Peyer's patches in hamsters after oral exposure to scrapie.

The disease-associated prion protein (PrP(Sc)) has been detected in the ileal Peyer's patches of lambs as early as one week after oral exposure to scrapie. In hamsters, the earliest reported time of PrP(Sc) detection in the Peyer's patches after oral exposure to scrapie is 69 days post-infection. To evaluate the acute uptake of inoculum and to investigate whether the Peyer's patches constitute the primary site of entry for scrapie after oral exposure, hamsters were each exposed orally to 1 ml of a 10% brain homogenate from hamsters in the terminal stage of infection with the 263 K strain of the scrapie agent. PrP(Sc) was demonstrated in the Peyer's patches only a few days after exposure, i.e., much earlier than previously reported. This study supports the view that the Peyer's patches constitute at least one of the primary entry sites of PrP(Sc) after oral exposure to scrapie.

Animals↗

Therapeutic plasma exchange in treatment of adrenoleukodystrophy.

Adrenoleukodystrophy (ALD) is an X-linked disorder of metabolism of very long-chain fatty acids (VLCFA) with a frequency of up to 1:20,000 in males. VLCFA C 24:0 and C 26:0 accumulate in the cholesterol ester and ganglioside fraction in plasma and red cells. Symptoms of ALD are ataxia, loss of visual and auditory functions, and cerebral convulsions. Presently, no sure therapeutic approaches are established. Efforts were reported by dietary regimens with VLCFA-restriction and glycerol trioleate and glycerol trierucate intake. In the present trial, we report on a 58-year-old white male suffering from progressive ALD with spastic paraparesis. He has a positive family history back to the 18th century. In this patient, although maximum dietary therapy was applied over a period of 60 months, no normalization of VLCFA C24:0 and C26:0 was reached, and neurological disorders were progressive. As a result, therapeutic plasma exchange (TPE) was applied from 1990 to 1994. Then, for more selective adsorption of VLCFA, dextran-sulfate adsorption (Liposorber, Kaneka, Osaka, Japan) until 1996, and after that, immunoadsorption (Therasorb, Baxter, Munchen, Germany) were used. During these periods (total, 101 months), VLCFA C 24:0 and C 26:0 levels were reduced by 55% and 50% (p < 0.001). The patient experienced a significant improvement in performance and general well-being. No further progression of neuronal disorders was documented. This anecdotal data suggest a very beneficial effect of TPE in treatment of progressive ALD.

Adrenoleukodystrophy↗

Constrained glycopeptide ligands for MPRs. Limitations of unprotected phosphorylated building blocks.

A new methodology for the synthesis of cyclic and phosphorylated glycopeptide templates was developed. First, fully protected building blocks containing mannose and mannose disaccharides with bis-trichloroethyl phosphate on Fmoc-Thr-OPfp were synthesized. These were used in solid-phase assembly through side chain anchoring of glycosylated hexa- and octa-peptides protected at the C-terminal carboxylate as the allyl ester. Selective allyl ester cleavage and head-to-tail cyclization under pseudodilution conditions gave a high yield of pure cyclic peptide templates. Unprotected phosphate in the building block was evaluated as an alternative to the problematic trichloroethyl group. It was found that one unprotected phosphate is readily incorporated, whereas the second unprotected phosphorylated building block react very slowly due to electrostatic repulsion in the solid-phase synthesis. For comparison with previous binding studies modified glycopeptide templates containing only phosphorylated mannose monosaccharides or templates modified in the peptide part were synthesized. All the structures were tested for their binding to the mannose 6-phosphate receptor, and it was found that although mannose disaccharides are required for optimal interaction, the detailed structure of the peptide template has a strong influence on binding to the receptor. The restricted conformations of the cyclic peptides decreased the binding considerably.

Chromatography, High Pressure Liquid↗

Plasmapheresis in treatment of adrenoleukodystrophy.

Adrenoleukodystrophy (ALD) is an X-linked disorder of metabolism of very long chain fatty acids (VLCFAs) with a frequency of up to 1:20,000 in males. VLCFAs C24:0 and C26:0 accumulate in the cholesterol ester and ganglioside fraction in plasma and red cells. Symptoms of ALD are ataxia, loss of visual and auditory functions, and cerebral convulsions. Up to the present, no sure therapeutic approaches have been established. Efforts were reported by dietary regimens with VLCFA restriction and glyceroltrioleate and glyceroltrierucate intake. In the present trial, we report a 55-year-old Caucasian male suffering from progressive ALD with spastic paraparesis. He has had a positive family history since the eighteenth century. In this patient treated with maximum dietary therapy over a period of 60 months, no normalization of C24:0 and C26:0 was reached, and neurological disorders were progressive. As a result, plasmapheresis was applied during the period 1990-1994 and since then for more selective adsorption of VLCFAs, dextran sulfate adsorption (Liposorber, Kaneka, Japan). During this period (64 months), C24:0 and C26:0 levels were reduced by 54.5% and 51.8%, respectively (p = 0.0001). The patient experienced a significant improvement in performance and general well-being. There has been no further progression of neuronal disorders to document.

Adrenoleukodystrophy↗

Plasmapheresis in newborns with hyperbilirubinemia.

With a miniaturized plasmapheresis system consisting of a double head pump and balancing pump, extracorporeal detoxification treatments such as plasmapheresis, hemofiltration, and hemodialysis were carried out. In total 15 premature infants and newborns with Rherythroblastosis and hyperbilirubinemia were treated using this miniaturized system. The average birth weight of the 15 patients was 2,386 g. With a main blood flow of 5.3 ml/min and a filtrate flow of 1.3 ml/min, an average of 192.4 ml of plasma per treatment was exchanged. In all patients 1 to 10 plasmapheresis treatments were necessary to reduce the mean serum bilirubin from 15.6 +/- 4.8 to 7.3 +/- 3.2 mg/dl, and after several days the serum bilirubin subsequently normalized in 12 patients, who improved. Three patients died 5 to 18 days after birth as a result of their primary disease and immaturity. For vascular access small catheters were inserted in the umbilical or the femoral vein. All treatments were well tolerated by the patients.

Acute Kidney Injury↗

[Early recognition of C-cell carcinoma by family screening (author's transl)].

All available family members of five patients with C-cell carcinoma (medullary carcinoma of the thyroid) were tested for their basal serum-calcitonin level and after pentagastrin stimulation. In two females, aged 12 and 25 years, from two different families, serum-calcitonin was elevated, but only after repeated tests or after pentagastrin stimulation, to definitely abnormal levels. Neither had palpable thyroid nodules, lymph-adenopathy or diarrhoea. The thyroid scan was normal and there were no storage defects. But because of the family history and the elevated serum-calcitonin levels total thyroidectomy was performed in both. The specimens revealed multicentric, bilateral C-cell carcinomas. The serum-calcitonin concentration became completely normal postoperatively, so that one may justifiably speak of a curative effect of the thyroidectomy in these two instances of early diagnosis.

Adult↗

[Bioclimatic consultation].

The climatological environment may effect the human state of health, disadvantageously or favourably. This knowledge, confirmed by empiric as well as scientific research can be used by e.g. changing the place of residence towards a healthy and benefical climate. For a professional climatological planning of such a project the "Deutscher Wetterdienst" renders bioclimatic consultations within the Federal Republic of Germany. For this purpose individual reactions as well as state health of the person in question have to be considered. A method will be shown how to combine medical diagnostic facts and bioclimatic consultation.

Acclimatization↗