Pulmonary hypertension due to multiple emboli.
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Biomedical subjects
Publications and source records attributed to K Aterman.
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Disseminated peritoneal leiomyomatosis is an unusual condition characterized by the development of numerous nodules in the peritoneal cavity which histologically have the appearance of smooth muscle tumors. The ninth proven case of this ill-understood disorder is presented here, with comments on some of the other published instances. Most reported cases were diagnosed in pregnant women, and there is good reason to believe that endocrine factors are of importance in the development of this condition. Attention is drawn to experimental studies in which an apparently similar condition has been produced in guinea pigs by endocrine manipulation. Clinically, the condition appears to be benign and the correct diagnosis is, therefore, of great importance. In the case presented here histological involution of the leiomyomatous peritoneal nodules could be demonstrated for the first time by two biopsies at an interval of 4 months--in the absence of any therapy apart from the termination of pregnancy.
In recent years large numbers of the so-called "mucocutaneous lymph node syndrome" or "Kawasaki's disease" have been described by Japanese workers, but instances of this disorder are only now being reported as isolated cases by European or North American physicians. The disease has, therefore, been considered to be a new entity. One of its most striking features is the development of aneurysms of the coronary arteries in infants or children, which may lead to sudden death. Aneurysms of the coronary arteries in childhood are rare, and hence it was considered relevant to report six such cases, and to examine their possible relationship to Kawasaki's disease. The pathological changes underlying the latter disorder are not well known; they are considered to be indistinguishable from infantile polyarteritis nodosa. A diagnosis of polyarteritis nodosa was also thought to be most likely to apply in the cases presented here, particularly in view of the frequency with which aneurysms of the coronary arteries have been found in this disorder. In the absence of valid pathological distinctions between Kawasaki's disease and infantile polyarteritis nodosa, the question arises whether these entities are, in fact, different, and whether Kawasaki's disease is the new entity it is assumed to be.
A myopathy restricted to the diaphragm was found in an infant who died at 3 months of age of respiratory failure due to immobility of the diaphragm. Grossly, the diaphragm was thin and translucent. On microscopical examination few muscle bundles were seen. Most muscle fibers were small, and numerous necrotic fibers with large sarcolemmal nuclei, focal cytoplasmic basophilia, and hyaline or homogeneous eosinophilic degeneration in the center of the fibers were seen.
The historical development of the iodine-sulphuric acid reaction for amyloid is described. The reaction dates back to 1814 when Colin and Gaultier de Claubry, and independently Stromeyer, introduced the iodine reaction for starch. A variant of the acidified iodine reaction appears to have been used for printing paper by Gmelin in 1829, and in 1838 Schleiden used the iodine-sulphuric acid test on plants to demonstrate what he considered to be a transformation of the plant material into starch. Shortly afterwards Payen (1839) defined "cellulose", and the iodine-sulphuric acid reaction became a standard procedure used by botanists to demonstrate this plant component. In 1853 Virchow used Harting's (1847) procedure to demonstrate the reaction of Purkynĕ's corpora amylacea to this test, on the assumption that they might be cellulose derivatives, and applied it to what appeared to be similar corpuscles in a "waxy" spleen. The first histochemical reaction for amyloidosis had thus been introduced into pathology, and continued to exert from that time on an important influence on amyloid research, whose impact is felt to the present day.
Letterer-Siwe's disease was diagnosed from clinical appearance and initial assessment of a skin biopsy in a child with a 2-month history of skin rash. Fine erythematous papules were scattered on the trunk. The biopsy showed epidermal thickening and an inflammatory infiltrate chiefly in the upper layers of the dermis; deeper in the dermis the infiltrate was perivascular and periappendicular, histiocytes predominating in some areas and lymphocytes in others. A diagnosis of scabies was made after burrows were demonstrated on palms and soles and the mite of scabies was isolated from them.
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75 male rats were given toxic dosage of the hepatotoxin N-nitrosomorpholine (NNM) using varied concentrations over varied time intervals. During and after the toxic dosings the kidneys were examined by light and electron microscopy in order to decide, whether the kidneys are also damaged by NNM. Our studies reveal that under the influence of a low concentration of NNM a distinct thickening of the GBM and an increase of the mesangial matrix occurs (changes referred by us as glomerulosclerosis). When a high concentration of NNM was given, toxic lesions of the mesangial and epithelial cells of the glomeruli were found, but a glomerulosclerosis was not observed during the intoxication. After this toxic dose was stopped, however, a progressive glomerulosclerosis did develop, which at first was accompanied by a transient proliferation of the mesangial cells. The glomerular changes found in the course of poisoning with NNM were interpreted as a direct effect of the NNM. From studies of the formal pathogenesis of the glomerulosclerosis presented here one can conclude that the poisoning leads to a decrease in breakdown of the components of the basement membrane and the mesangial matrix, thus causing the widening of the GBM and the augmentation of the mesangial matrix.
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Three brothers are described who had a severe connective-tissue disorder and died in infancy. They were the offspring of a consanguineous union and members of a kindred in which other paternal relatives were found to have the benign hypermobile form of Ehlers-Danios syndrome. The findings in the three brothers were not clearly representative of any known connective-tissue disorder. The authors feel that the brothers' phenotype is best explained as a result of genetic interaction between the Ehlers-Danios gene and another single unspecified gene in double dose.