Biomedical subjects
Publications and source records attributed to R Albrechtsen.
Granular cell myoblastomas develop most commonly in the head and neck region, but their occurrence in the salivary glands has been reported in only one case, in the submaxillary gland. The authors report a case of granular cell myoblastoma in a previously undescribed localization, the parotid gland.
Two cases are presented of malignant tumour of the parotid gland following sialography with Thorotrast, 28 and 45 years previously. Both cases were histologically established as squamous cell carcinoma and the presence of Thorotrast in the tumours was confirmed by autohistoradiography. It is suggested that the tumours may have developed from metaplastic ductal epithelium after many years of exposure to the alpha radiation from Thorotrast deposits in the gland.
A 17 year old man with a primary intracranial germ cell tumor in the pituitary region is reported. Both serum alpha-fetoprotein (AFP) and serum human chorionic gonadotropin (hCG) were found elevated preoperatively. Normal levels of serum AFP and hCG were found after operation and radiotherapy. By indirect immunofluorescence staining of the tumor tissue a slight staining for AFP was localized to the endodermal vesicles, whereas a more intensive staining for hCG could be identified in the giant cells.
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PH determinations by means of a sturdy, spear-electrode inserted in the cerebellar tissue immediately after necropsy were carried out in 60 patients suffering from different disorders and dying in hospital, and from 25 individuals killed instantly by violent accidents. A correlation was found between the presence of NGL and low pH levels in the cerebellum (average pH 5.56); subjects without NGL changes showed an average pH OF 6.08. No correlation was found between the pH levels and the time after death, or between pH and any specific disease. The so-called acute selective necrosis of the granular layer of the human cerebellar cortex (NGL), is considered from the present studies to be a phenomenon of postmortem origin. Its relationship to certain specific diseases can be explained by the fact that in these conditions there is a higher postmortem acidity, known to promote autolysis.
Acute selective necrosis of the granular layer of the cerebellum (NGL) was demonstrated in 264 patients out of a total of 1000 autopsies. The relatively high incidence of NGL was distributed evenly over the main groups of causes of death, and over all age groups, with the exception of children under the age of 10 years, in whom the incidence was lower. The lack of correlation between NGL and any particular disease makes it difficult to put forward a plausible explanation of the necrosis. In view of recent studies it is concluded that NGL develops after death.
Histochemical examination of the activity of naphthylamidase (LNAse) in the cerebellar cortex of 70 human autopsies consistantly revealed a marked activity mainly in the internal granular layer with pH optimum of 5.8. Slight enzyme activity was also localized in sites corresponding to lipofuscin deposits and areas of acid phosphatase activity in the Bergmann glial cells, Purkinje cells and in perivascular cells. The histochemical findings support the LNAse reaction as a lysosome marker. Differences in localization of LNAse and acid phosphatase could possibly be due to prior release of the latter enzyme from the internal granular layer. Significant correlation between demonstrable loss of granule cell nuclei (the so-called acute, selective necrosis of the granular layer) and low pH of the cerebellar tissue could be demonstrated in 21 cases. The present findings support the hypothesis that an enzymatic disintegration of the granule cells takes place in postmortem cerebella with low pH simulating a necrotic vital phenomenon.
A carcinoma developed in a thorotrastoma of the right parotid gland and caused facial paralysis 28 years after a sialography with radioactive thorotrast. The literature is reviewed, and another case is reported in which facial nerve paralysis occurred without evidence of malignancy.
Twelve patients with histologically verified polycystic ovary syndrome were investigated with special regard given to the effect of wedge resection on androgen status. Adrenal disorders were excluded in every case by determination of cortisol and corticosterone metabolites. Prior to and at least 6 months after surgery all patients were subjected to adrenal stimulation followed by adrenal suppression and ovarian stimulation. Comparison between pre- and postoperative studies revealed that only an insignificant reduction in the excretion of 17-ketosteroid had occurred and, although at lower levels, the stimulatory effect of hCG on ovarian androgens was still present. Clinically, 10 patients had had regular periods at followup, and 3 had become pregnant. In no case had hair growth slowed, bu the rate had declined. Seemingly, wedge resection does not significantly influence the biochemical pattern connected with the polycystic ovary syndrome.
The mechanism of neosynthesis of the human tumor-associated fetal antigen alpha-fetoprotein (AFP) in a variable percentage of patients with testicular, ovarian and extragonadal germ cell tumors has generally been considered unknown or beyond any simple explanation. Of decisive importance is the cellular basis for AFP production 1. in ontogenesis and 2. in malignancy as dependent on an exact tumor histogenesis. Based on (1) the histogenetic-embryologic classification of germ cell tumors and the concept of yolk sac tumor (or endodermal sinus tumor), (2) the available clinical and experimental observations, and (3) the immunofluorescent localization of AFP in the endodermal sinus tumor of the human testis, it is concluded that AFP synthesis in these neoplasms is explained by the fact that they contain yolk sac endoderm, which produce AFP analogous with the physiological AFP synthesis by the fetal yolk sac in early embryogenesis.
The correlation between serum alpha-foetoprotein (AFP) and the clinical pathological finding of 24 germ cell tumours arising from the testes (14 cases), the ovaries (3 cases), the mediastinum (3 cases), the retroperitoneal region (2 cases), and the sacrococcygeal region (2 cases) are presented. Irrespective of marked differences in age and sex of the patients, primary site of the tumours and clinical outcome, the 24 cases constituted a homogeneous group in fundamental histological patterns and in AFP synthesis. In all cases of endodermal sinus tumour or teratocarcinomas with a distinct vitelline component an increased serum AFP concentrations was found in the pre-operative serum samples. AFP was also demonstrated in the tumour tissue by quantitative determination of AFP in tumour homogenate (5 cases) and, by immunofluorescence technique, positive staining of the cells lining the endodermal sinuses and of the hyaline globules was found (3 cases). In 12 germ cell tumours without vitelline components in the tumour tissue sections, a normal AFP concentration below 20 mug/1 was found in preoperative serum samples.
A virilizing adrenocortical adenoma was demonstrated in a yound female. Urinary 17-ketosteroid excretion and subfractions, plasma testosterone level and urinary 17-ketogenic steroid excretion were markedly increased. Dehydroepiandrosterone was the main constituent of the androgen excess. Otherwise the adrenocortical function was found to be normal as evaluated from measurements of cortisol, corticosterone and their metabolites under basal conditions and during dynamic tests. The androgen excess showed an unexpected response to trophic hormones. Human chorionic gonadotrophin stimulation resulted in a pronounced increase in androgen production, whereas no gonadotrophin-dependency could be demonstrated by means of the oestrogen suppression test. Similarly, no corticotrophin-dependency could be demonstrated by corticotrophin stimulation and suppression tests. Removal of the tumour resulted in normalization of the androgen production and no abnormal response upon human chorionic gonadotrophin administration could now be found. The inappropriate response of tumours to trophic hormones is discussed. It is concluded that the reliability of stimulation and suppression tests in determining the site of excessive androgen production should be accepted with reservation.
Histochemical investigations of leucine aminopeptidase using LNA (L-leucyl-beta-napthylamide) as a substrate reveals a marked enzyme activity selectively localized to the granular layer with inconspicuous reaction in the stratum moleculare and the Purkinje cells in the rat cerebellum. The LNA-splitting enzyme differs from the well-known leucine aminopeptidase (LAP) by its optimum at pH 5.5. The necessary long incubation period used in the present study, and its focal precipitation of the enzyme reaction product in the same place, like acid phosphatases, in the granular layer, suggest a lysosomal localization. The functional role of the LNA-splitting enzyme has been discussed; it is considered that it is involved not only in the protein transformation for synaptic function, but may perhaps also play an important pathogenic role in necrosis, atrophy or even autolysis.
123 patients suffering from axillary hyperhidrosis have been operated on as outpatients under local anaesthesia, with radical ablation of sweat glands. All except 7 patients were satisfied with the result. There were few complications. A group of patients and controls were extensively studied including quantitative sweat testing and axillary biopsis. Apart from increased axillary sweating during the test, no differences could be found. The basic etiology of the condition is still unknown.