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Biomedical subjects

J Herrmann

Publications and source records attributed to J Herrmann.

At least 289 records · Page 16Linked to original sources

Simpson-Golabi-Behmel syndrome: follow-up of the Michigan family.

Here we report a follow-up on a boy born in 1983 into a family with presumed Simpson-Golabi-Behmel syndrome and first reported as patient 3 by Opitz [1984] under the designation "Golabi-Rosen" syndrome. The patient died at 25 months without having attained any measure of psychomotor development or maturation and with a neurologic picture of irritability, increased muscle tone, seizures, deafness and possible cortical blindness. He had a striking facial appearance similar to that of severely affected individuals in the family reported by Golabi and Rosen [1984], with mild hepatosplenomegaly, unusual skin, normal growth, decelerating OFC, and on autopsy a spongiform degeneration of brain stem and cerebrum. Results of all biochemical studies, including those pertaining to GM3 gangliosidosis, were normal.

Abnormalities, Multiple↗

Hepatic thyroid hormone levels following chronic alcohol consumption: direct experimental evidence in rats against the existence of a hyperthyroid hepatic state.

To study the effect of chronic alcohol consumption on hepatic levels of thyroid hormones, female Sprague-Dawley rats (n = 24) were pair-fed nutritionally adequate liquid diets containing either ethanol (36% of total calories) or isocaloric carbohydrates for 21 days. Compared to controls, chronic alcohol consumption failed to result in a significant change of hepatic thyroid hormone levels (thyroxine: 14.7 +/- 1.81 ng per gm of liver wet weight vs. 15.0 +/- 1.59; triiodothyronine: 2.60 +/- 0.16 ng per gm of liver wet weight vs. 2.66 +/- 0.18). Similar results were obtained when the hepatic levels of thyroid hormones were expressed per total liver, per gram of liver protein or per 100 gm of body weight. Moreover, prolonged alcohol ingestion led to a significant reduction of serum total thyroxine by 31.6% (p less than 0.001), free thyroxine by 38.9% (p less than 0.02), total triiodothyronine by 40.2% (p less than 0.001) and free triiodothyronine by 56.1% (p less than 0.001) when compared to their pair-fed controls, whereas thyroid-stimulating hormone levels remained virtually unchanged. These data, therefore, clearly show that chronic alcohol consumption is incapable of creating a hyperthyroid hepatic state in rats, and limit the rationale for antithyroid treatment in patients with alcoholic liver disease.

Alcoholism↗

Prevalence of hypothyroidism in the elderly in Germany. A pilot study.

In 2579 senior German citizens studied in nursing homes and in- and outpatient clinics overt hypothyroidism was found in only 1%. This figure is clearly lower than that reported for Great Britain, New Zealand and North America (2-3%). Subclinical primary hypothyroidism on the other hand was detected in almost 4% on the basis of an exaggerated TSH response to TRH. Measurement of the basal TSH levels did not significantly increase the diagnostic sensitivity for hypothyroidism, since among the 768 subjects screened by primary TRH test only 16 of the 29 cases classified as subclinical hypothyroidism had a slightly elevated basal TSH. Basal TSH was elevated in only 2.4% of the cases. Again this figure is lower than that found in other countries (6-14%). In view of the low prevalence of subjects with overt hypothyroidism who would benefit from subsequent treatment, a large scale screening program can be recommended only with reserve for the federal republic of Germany.

Aged↗

Inhibition of peripheral deiodination of 3, 5, 3'-triiodothyronine: an adverse effect of propylthiouracil in the treatment of T3-thyrotoxicosis.

An inhibition of peripheral conversion of T4 to T3 is thought to be of benefit in the treatment of thyrotoxicosis. Therefore, propylthiouracil (PTU) has been considered to be more effective in the therapy of hyperthyroidism than methimazole, since the former has the additional peripheral effect of decreasing the conversion of T4 to T3, From in vitro studies PTU is known, however, to inhibit the deiodination at the 5' as well as at the 5 position of the iodothyronine molecule. To study if PTU blocks degradation in T3 in vivo as well, the effect of PTU on thyroid hormone concentrations in serum and liver tissue during a constant and high administration of T4 or T3 to rats was followed. It was shown that PTU clearly inhibits T4 and reverse T3 degradation. Moreover, simultaneous treatment of the rats with T3 and PTU resulted in a significantly higher increase of T3 concentration in liver tissue (11.5 ng/g liver vs 5.6 ng/g liver) and serum (615 ng/di vs 345 ng/dl) than with T3 alone. This effect may be explained by an inhibition of the T3 degradation by PTU in vivo as well. Provided the results obtained from these animal experiments can be applied to the situation in man, the inhibition of peripheral deiodination could have an adverse effect at least in the treatment of T3-thyrotoxicosis.

Animals↗

Effects of lipoic acid in hexacarbon-induced neuropathy.

The effects of lipoic acid on hexacarbon neurotoxicity in rats were investigated. Rats were exposed by inhalation to n-hexane for 24 hours/day, 7 days/week, up to a total period of 9 weeks. Eight animals were exposed to 700 ppm n-hexane only, and eight animals were exposed to 700 ppm n-hexane and additionally received 100 mumol/kg lipoic acid PO daily. Clinical status of the animals was evaluated by examination of general condition, motor performance tests and neurophysiological measurements of caudal nerve motor conduction velocity. Results showed that animals exposed to 700 ppm n-hexane developed severe motor neuropathy leading to paralysis by the 6th week. Motor distal latencies of these animals were severely prolonged. In contrast, in animals treated with lipoic acid the onset of motor neuropathy was delayed for approximately 3 weeks as could be demonstrated by motor performance tests and measurements of motor distal latencies.

Action Potentials↗

Structural basis for binding of Smac/DIABLO to the XIAP BIR3 domain.

The inhibitor-of-apoptosis proteins (IAPs) regulate programmed cell death by inhibiting members of the caspase family of enzymes. Recently, a mammalian protein called Smac (also named DIABLO) was identified that binds to the IAPs and promotes caspase activation. Although undefined in the X-ray structure, the amino-terminal residues of Smac are critical for its function. To understand the structural basis for molecular recognition between Smac and the IAPs, we determined the solution structure of the BIR3 domain of X-linked IAP (XIAP) complexed with a functionally active nine-residue peptide derived from the N terminus of Smac. The peptide binds across the third beta-strand of the BIR3 domain in an extended conformation with only the first four residues contacting the protein. The complex is stabilized by four intermolecular hydrogen bonds, an electrostatic interaction involving the N terminus of the peptide, and several hydrophobic interactions. This structural information, along with the binding data from BIR3 and Smac peptide mutants reported here, should aid in the design of small molecules that may be used for the treatment of cancers that overexpress IAPs.

Amino Acid Sequence↗

[Clinical relevance of the early detection of arthrosis].

In the years 1989 to 1992 615 local persons underwent yearly examinations for analysis of osteoarthrosis of the hip and knee by means of comprehensive documentation of orthopaedic health history and clinical findings. Of special interest in our investigation were the Altman ACR criteria for osteoarthrosis of the hip and knee over the years. We can show, that finding the diagnosis is as accurate with the ACR criteria as well as the far more extensive Lequesne and Tegner-Lysholm score. Analysis of the investigations over the years revealed clearly different results in the frequency of osteoarthrosis. The reason is the nature of osteoarthrosis changing between silent and active phases especially during time of onset. Our investigations show, that valuable criteria exists for detection of early osteoarthrosis, however apparent are deficits for observing its course.

Adult↗

The endothelium: dysfunction and beyond.

Since its anatomic discovery in the 19th century, the endothelium was considered to fulfill no other purpose than that of a physical barrier between blood and tissue, until Furchgott and colleagues defined endothelium-dependent vasoreactivity in the late 1970s. Henceforth, a functional paradigm defined the balance between endothelium-derived relaxing factors and endothelium-derived contracting factors as the hallmark of endothelial cell integrity. As a consequence, any reflection of a deviation from this state was defined as endothelial dysfunction, most notably the impairment of vasorelaxation in response to pharmacologic stimuli such as acetylcholine or nonpharmacologic stimuli such as shear stress and cold pressor. Within the coronary artery tree these alterations have been recognized before the development of obstructive coronary artery disease, affecting the microcirculation before the epicardial conduit vessel. Furthermore, recent clinical trials outlined the prognostic significance of these changes, whereby impairment of increase in coronary blood flow in response to endothelium-dependent stimuli seemed to be of utmost importance. Thus it is intriguing to speculate on the evolving role of myocardial perfusion imaging in combination with pharmacologic and nonpharmacologic stimuli for the noninvasive assessment of coronary endothelial dysfunction in patients at risk for future adverse events. At a minimum, this review aims to put endothelial dysfunction into an imaging perspective beyond the scope of the conventional approach.

Animals↗

[Methods of resolution for haptic assistance during catheterization].

During catheterization navigation within the patient is mainly dependent on a live x-ray image on the screen. Although methods for 3D visualisation and remote navigation of the catheter are discussed and tested still precise positioning is merely the result of intense training and a high skill and level of training of the performing surgeon. This article refers to a system which can be considered as an add-on for existing procedures of catheterization. It compromises of a miniaturised force sensor located at the tip of guide-wires whose prototype is shown here. The measured forces will be presented to the surgeon amplified by an external actuator described in this article. As a result a haptic perception of the forces between the tip of the guide-wire and the vessels walls will be available and enable the surgeon to gain an impression which is comparable to palpation of living vessels from the inside

Catheterization↗

The hospital response.

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Contract Services↗