Unoccupied electron band structure of Na overlayers on Al(111).
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Biomedical subjects
Publications and source records attributed to K Frank.
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Sonography and computed tomography were the first imaging methods enabling the visualization of intramurally growing tumours of the stomach. The article reports on a rare case of a diffuse leiomyoma that was diagnosed by means of computed tomography.
Inherited via the X chromosome, adrenomyeloneuropathy is a rare cause of primary adrenocortical insufficiency. Neurological signs are of central and peripheral demyelinization, while endocrinologically it is characterized by Addison's disease and primary testicular insufficiency. In two patients with this condition the metabolic defect in the breakdown of long-chain fatty acids was confirmed by an increased hexakosan (C 26) blood level. One patient had an isolated failure of the zona fasciculata; in the other there was clinically manifest complete adrenocortical insufficiency. Both patients had incipient hypogonadism. In the second case, neurological symptoms preceded the endocrinological ones, while in the first both the family history and the adrenocortical insufficiency led to the diagnosis. In peripheral neuropathy in a young male, attention should always be given to signs of incipient adrenocortical insufficiency.
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A three-generation family with hereditary high thyroxine-binding globulin (TBG) serum levels has been studied. All patients were clinically euthyroid and did not have a goiter. Four females of the eleven members of the kindred had elevated TBG (range: 34-56 micrograms/ml), total thyroxine (T4) and total triiodothyronine (T3) levels, but normal free T4 (fT4) and normal response of thyroid stimulating hormone (TSH) to intravenous application of thyrotropin-releasing hormone (TRH). Microheterogeneity of TBG studied by isoelectric focusing showed a normal pattern. No variations of the four main bands as seen in other causes of TBG excess like liver diseases, estrogen therapy or pregnancy could be measured. The changes in TBG concentration seem to be due to an abnormal gene expression controlling synthesis of TBG.
Sonography can rapidly and reliably help to determine the size by the spleen. To standardise the sonographic dimensions of the healthy spleen, we effected sonographic examination of 793 female and male patients aged between 17 and 82 years with a healthy spleen. In 95% of the patients the length of the spleen was less than 11 cm, the breadth below 7 cm and the thickness less than 5 cm. The weight of the spleen as determined via the rotation ellipsoid formula was below 190 g in 95% of the patients. Whether or not a spleen larger than standard signals an acute disease can be clinically assessed only on the basis of a synopsis of all findings.
The audiometric data of 41 patients with Paget's disease of the bone show that hearing loss exceeds the age-related presbycusis in patients with deformities of the temporal bone as demonstrated by X-ray (27%). The usual type of hearing loss is a sensorineural one. 80% of these patients showed hair cell damage, 32% of them a retrocochlear lesion, depending on the degree of deformity of the temporal bone. Conductive hearing loss of the otosclerosis type was found in only 20%. The Paget-related hearing loss can be regarded as a consequence of the deformation occurring both at the bone surrounding the cochlea and at the internal auditory canal.
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Three patients with a surgically confirmed and 1 with suspected pheochromocytoma as part of the syndrome of multiple endocrine neoplasia (MEN) type II are presented. Pheochromocytoma and medullary thyroid carcinoma (MTC) as well as other tumors are part of this heritable entity. Three cases were members of a MEN IIa family and 1 patient has suffered from a sporadic form of MEN IIb. Pheochromocytoma was diagnosed by screening procedures (catecholamine screening, computerized tomography, sonography or selective venous catheterization). In 2 of the patients there was no clinical manifestation of the pheochromocytoma. On three occasions MTC was diagnosed first, the thyroid tumor occurred prior to the pheochromocytoma by 7, 6, and 1/2 years. Pheochromocytoma occurred bilaterally in 2 patients, in 1 it is suspected in both adrenals.
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In a 31 year old patient with adrenocortical gland insufficiency and minor clinical signs of involvement of peripheral and central nervous system an adrenoleucodystrophy was diagnosed, based upon the characteristic elevation of hexacosanoic acid in plasma. The motor and sensory nerve conduction velocities in upper and lower extremities were markedly reduced; in accordance, the somatosensory evoked potentials showed distinct reduction in the peripheral part. In contrast, the interpeak latencies N 20 - NSEP 3 (median nerve) and N 22 - P 40 (tibial nerve) were in the upper normal range, so that a damage of the central sensory system could not be evaluated. The visual and brainstem acoustic evoked potentials were a little prolonged. The problems in differential diagnosis of this form of adrenoleucodystrophy, which especially involves the peripheral nervous system, are discussed.
Ejaculated bull spermatozoa separated from seminal plasma by repeated washing were used to study the effects of various substrates on respiration, mitochondrial energization, cellular ATP/ADP ratios, and motility. The respiratory rate in the presence of lactate, pyruvate, acetate or glycerol was about two to three times higher compared to cells oxidizing amino acids, fatty acids, or endogenous substrates. When well oxidizable substrates were present, the uncoupler FCCP produced a marked increase in respiration (two to three times at optimal FCCP concentrations). Mitochondrial energization was monitored in intact spermatozoa by spectrophotometry with the dye safranin. Lactate and pyruvate yielded more pronounced mitochondrial energization than did alanine or endogenous substrates. Cellular ATP/ADP ratios, with lactate, pyruvate, acetate or glycerol, were found to range from 6 to 10, whereas endogenous substrates and amino acids were able to establish ATP/ADP quotients as low as about 1 only. The capacity to oxidize various substrates was clearly reflected by the amount of motile cells. Motility in the presence of lactate, acetate or glycerol was seen in more than 60% of spermatozoa, while spermatozoa in the presence of alanine or butyrate showed a motility not above the endogenous level (approx. 30% motile cells). The experiments demonstrated that cellular performance is highly dependent on the energy state as made possible by the capacity of substrate utilization.
Three patients with a sporadic form of multiple endocrine neoplasia (MEN) IIb are presented. MEN IIb is the association of medullary carcinoma of the thyroid (MTC), pheochromocytoma and multiple mucosal neuromata. The age at diagnosis and primary therapy of MTC was relatively late (8, 16, 18 years) although thyroid enlargement (two cases), typical marfanoid habitus (two cases) or multiple mucosal neuromata (one case) had been evident since early childhood. The diagnosis was confirmed by measurements of tumour markers like calcitonin (CT) and carcinoembryonic antigen (CEA) for MTC, measurements of vanillylmandelic acid for pheochromocytoma, evaluation of the thyroid gland by sonography, scintigraphy and fine needle biopsy, and investigation of the adrenals by computer tomography and meta-(I-131) iodobenzylguanidine (I-131-MIBG) scintigraphy. After surgical treatment determinations of tumour markers confirmed relapses of MTC in all cases. Early diagnosis of MEN IIb, which could be made by recognition of the typical clinical appearance, is of special importance because of the poor prognosis of MTC.
This report deals with the results of post-mortem sonographic examination of the spleen of 36 patients. The goal of the study was to gain direct information on the precision of the sonographic organometric method we use. It becomes evident that - depending on the position of the spleen in the dome of the diaphragm - the real length of the organ is underestimated when it is measured as the direct line between the two poles. For this reason the length should be taken as the (curved) median line obtained when performing a longitudinal section of the spleen at sonography. Under these conditions there is a good correlation of the sonographic and anatomic data obtained by autopsy. The mean deviation is maximally 7 mm. For the approximate calculation of the organ weight from the sonographically obtained data the simple ellipsoid formula is sufficient. In order to obtain a normogram for sonographic weight estimation a larger statistical sample is necessary.
In a prospective study, the authors screened a total of 2.070 patients. Of these, 213 showed unexpected sonographic findings. 96 of these patients were women. Gynaecological diseases or changes in the minor pelvis were least represented. This proportion is so small that routine sonography does not appear worth while as far as gynaecology is concerned. For, if we take the incidence of adnexal tumours into account, relevant chance findings can be expected in only 2.8 cases out of one million, which makes the screening process uneconomical.