Medulloblastomas with metastases outside the nervous system.
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The various patterns observed by generating Fourier transforms of the fixed normal corneal stroma are described. The patterns are analysed in terms of the dimensions of the stromal fibers, the dimensional and directional order of the fiber distribution and the order of the fiber-fiber functions by proteoglycans.
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A case of familial deficiency in alpha-1-antitrypsin connected with Pi-0Z gene led to the finding of a new Gcler variant partly deficient, the electrofocusing pattern of which, located between that of G and I variants, was modified after neuraminidase digestion. A study of three generations shows that Gcler variant is transmitter according to an autosomally codominant mode. Moreover serum trypsin inhibitory capacity and concentration of ten proteins have been measured in this family. Except the known relation between serum alpha-1-antitrypsin level and trypsin inhibitory, capacity, only serum IgA showed a significant correlation with serum alpha-1-antitrypsin in the deficient group with Pi-Z allele.
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Childhood (3 to 9 years) is a calm period of constant growth throughout which social contacts are discovered and intelligence and discretion take place. Consequently, most diseases are infectious. Microrelief was found twice shallower and skin (epidermis + dermis) twice thinner than in adults. Intervals between furrows were reduced parallel to total body surface, thus ruling out the possible existence of special shelters favouring bacterial growth. Sweating is lower, as are skin surface lipid levels, in contrast to the post-natal period, inducing dryness of skin. Although total skin stiffness in vivo was found to be identical to young adult skin, skin chapping is much more frequent. Since detergents as well as extended hot baths may reduce the stratum corneum water binding capacity, they should be used with discretion. Hence, skin care in this period of age should be based upon different criteria than in adults.
Among 120 cases of cerebral metastases, 62 primary sites were found. The diagnosis of the primary site was confirmed: --after the first investigations in 48 cases (in 90% of cases by anamnestic inquiry, clinical examination or chest X ray); --by the evolution of the disease in 5 cases; --by autopsy in 9 cases. The most frequent primary sites were lung (45%), digestive tract (17.7%), melanoma (8%) and ovary (6.5%). Among the identified cases, 25 received treatment for their primary cancer, but only 4 had a surgical resection. There was no significant difference in mean survival between the 2 groups (defined as primary site known or unknown); over all mean survival was 8 months. The early discovery of cerebral metastases suggests the advanced state of the disease. Therefore, investigations which cause the patient to suffer do not seem justified merely for research purposes.
Between 1975 and 1979, 137.526 annual X-ray examinations of the chest were performed among the employees of a large Paris administration. Fifty-four cases of tuberculosis, 28 cases of sarcoidosis, 10 cases of bronchial carcinoma and 26 miscellaneous chest diseases were detected. This study confirms the inadequacy of radiological examination for detecting chronic obstructive bronchopulmonary conditions. The small proportion of tuberculosis (0.39 p.1000) and bronchial carcinoma (0.07 p.1000) revealed by this method suggests that it should be reserved to high risk patients.
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The haptoglobin (Hp) polymorphism is investigated in 11 African groups living in an area from the Algerian Sahara to Central Africa. More than 4,000 samples were examined. In the Saharian samples, the Hp1 gene frequency is higher than in any other African group. From north to south, a decrease in the Hp1 gene frequency is observed; in the Pygmy sample only, this frequency is lower than the frequency of the Hp2 gene. By means of a sensitive radioimmunoelectrophoresis, the presence of a residual Hp in Hp O sera in which the Hp polymorphism can also be determined can be revealed. Absence of Hp 1-1 and significant excess of Hp 2-2 individuals were observed. More Hp 2-1M phenotypes were detected in the Hp O population than in the non-Hp O population examined. In the Hp O samples, the influence of the phenotype distribution on the Hp gene frequencies is discussed. The heavy polymers of the Hp related to the presence of the alpha 2 chain (Hp2 gene product) are involved only in the biological mechanisms responsible for the presence of Hp O and Hp 2-1 M phenotypes among African groups.
Two chronic alcoholic patients developed severe encephalopathy while receiving high doses of parenteral thiamine and pyridoxine. Both presented with unusually marked hypertonia of the gegenhalten type, myoclonias and fluctuating mental impairment with memory disturbances and hallucinations. Later on, one patient went into stupor and coma and died ; the other recovered after parenteral niacin treatment. No skin lesions or diarrhoea were observed. Post-mortem examination of the first patient confirmed the presence of a pellagra-like encephalopathy characterized by widespread neuronal chromatolysis. There were no changes suggesting an associated alcoholic encephalopathy.
The therapeutic problems raised by recurrences are different from those which apply to the initial malignant glioma. Firstly, the histological diagnosis is known (by excision or biopsy) and secondly the clinical period which has elapsed before the recurrence represents a factor in the assessment of progression and/or malignancy. The management of treatment must take into account various parameters of an anatomical and clinical nature, but although those relating the various forms of treatment used already. Although the methods used are the same: surgery, radiotherapy, chemotherapy, the strategy will differ according to indications of final recourse or reoperation will be discussed after initial surgery followed by radiotherapy, irradiation in those cases in which radiotherapy has not already been used and chemotherapy, Corticosteroids are also widely employed. Whilst such palliative treatment has the merit of ensuring prolongation of survival, the principal aims must be concerned with the quality and conditions of this survival.
Plasma cholenesterase is classified among blood markers whose genetic variation in man was discovered during past years. To look broadly at the characteristics of this system two main fields are now well established: geographical hematology and pharmacogenetics. In the present work the results of the study of that enzyme are reported in 2 400 individuals belonging to distinct Pyrenean communities. On geographical hematology the data obtained show the existence of a genetic polymorphism, the extent of which has a significant variation in the different areas. The E1a gene reaches the highest frequency (7.7%) in the central part of the Pyrénées (Lux Saint-Sauveur Valley): the rate decreases in Toulouse (3%) and in the Basques (2.3%). The E1s allele was found in the Basques with a frequency of 2.13% value which gives a peculiar feature to this group when compared to other Pyrenean populations. The E1f gene was seldom discovered in the samples studied. These data fill a gap in mapping the distribution of blood markers in France. On pharmacogenetics, the gene frequencies observed at the E1 locus were used for the likelihood of findings genotypes with a sensitivity to suxamethonium or other chemicals. Such a sensitivity cannot be underestimated in some groups (central part of Pyrénées, Basques). In medical practice a molecular abnormality of cholinesterase may be detected after apnea due to suxamethonium administration during anesthesia or in some cases of toxic syndromes induced by chemical compounds (agricultural or industrial products), inhibition of Cholinesterase.
One hundred and fifty patients admitted consecutively to the same neurosurgical service in Paris were reviewed retrospectively. There is no definite difference between intracranial tumors in mental patients and those encountered in a normal population in terms of: frequency, anatomic localisation and histological distribution. All the patients had been referred previously to psychiatric hospitals for: confusional states, disturbed behaviour or pseudo-depression in 60% of the cases. Neurological localizing signs were present in 83% of the cases; EEG was abnormal in 96% of the patients recorded during their stay in psychiatric wards. Diagnosis of brain tumors in mental patients appears feasible if not always simple; it is based upon elementary neurological examination and routine investigations. In a near future, isotopic scan and CT scan should become a great help to reduce excessive diagnostic delays in order to improve the prognosis which remains poor.
In 34 patients with peripheral occlusive arterial disease, circulating levels of endothelial cell markers were compared with the ischemic status. Unlike tissue plasminogen activator and plasminogen activator inhibitor, plasma levels of thrombomodulin were closely related to both transcutaneous oxygen pressure (p = 0.01) and the graded clinical stages of disease (p = 0.02). Levels of von Willebrand factor were correlated only with the transcutaneous oxygen pressure (p = 0.04). Since thrombomodulin and von Willebrand factor constitute markers of endothelial cell damage, the extent of endothelial injury would appear to be determined by the ischemic status.
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