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S Kircher

Publications and source records attributed to S Kircher.

14 recordsLinked to original sources
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↗
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

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↗
PubMed1978

[Results of screening for risk factors in a rural community (author's transl)].

515 women and 372 men from a rural community of 2862 inhabitants over 14 years volunteered to participate in a screening test for risk factors. We found an exceptionally high percentage of hypertension and hypercholesterolaemia as well as overweight. Women were more frequently affected than men except for smoking, which occurred 3,6 times more often in men than in women. Since the life expectancy of men in this community was clearly lower than that of women, smoking seems to have the most weight as a risk factor for cardiovascular diseases.

Cardiovascular Diseases↗
PubMed1977

Aide orientation.

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Inservice Training↗
PubMed1977

[Angiographic studies in fluoroscopically detected coronary calcification (author's transl)].

We performed coronary angiography in eighty subjects with radiographically diagnosed coronary calcification. Radiographically proven coronary calcification is highly specific for coronary heart disease. Within certain limitations it helps in locating stenoses and is present more frequently in multiple vessel disease. It sometimes detects coronary artery disease missed by other non-invasive methods.

Adult↗