{"title":"A Unifying Concept for the Role of Matrix Vesicles and Lysosomes in the Formal Pathogenesis of Diseases of Connective Tissues and Blood Vessels","authors":"U.N. Riede , J. Staubesand","doi":"10.1016/S0005-8165(77)80003-6","DOIUrl":"10.1016/S0005-8165(77)80003-6","url":null,"abstract":"<div><p>The cells of the connective tissues contain lysosomes with enzymes capable of degrading intercellular substances (collagen, elastin, proteoglycans) and release their enzymes in membrane-bound or in free form into their intercellular substance. In this way extra-cellularly located lysosomes (= matrix lysosomes) can be detected by morphological and enzymatic means as well as by their metal concentrating property. This function of such matrix-lysosomes is in connection with the two step-degradation of the connective tissue and is thought to be the main part for the fibrocytic fibrolysis, chondrocytic chondrolysis, osteocytic osteolysis and myocytic mediolyses in the vessel wall. The cells of the mesenchymal tissues thus control the turnover and transformation of their own ground substance. Inflammatory and immunologic processes are suggested to be superimposed on this lysosomal action. If the lysosomal enzyme system in the connective tissues and the vessel wall gets out of control, the consequences can be dangerous as e.g. in case of relapsing polychondritis and arterial aneurysms. In this enzyme system proteolytic activators as well as proteolytic decomposable inhibitors are present. The corresponding proteolytic processes are of lysosomal nature and are subordinated to other regulatory mechanisms.</p></div>","PeriodicalId":75583,"journal":{"name":"Beitrage zur Pathologie","volume":"160 1","pages":"Pages 3-37"},"PeriodicalIF":0.0,"publicationDate":"1977-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0005-8165(77)80003-6","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"12057127","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Mucopolysaccharidosis III A (Sanfilippo disease type A). Histochemical, electron microscopical and biochemical findings.","authors":"H Cain, E Egner, H Kresse","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>This is a report of two brothers iwth mucopolysaccharidosis. The 8- and 10-year-old boys presented the characteristic clinical symptoms of the syndrome in their entirety. Both had a highly increased excretion of heparan sulfate in urine. The elder boy died and was autopsied and diagnosed as having Sanfilippo disease Typ A by a drastic reduction of heparan sulfate sulfamidase activity in organ extracts. Histochemically, highly water-soluble, sulfate acid mucopolysaccharides were demonstrated in liver, spleen, and cerebrum of the deceased child. Chemical analyses revealed a 12-fold increase of sulfated mucopolysaccharide in the patients liver and a 4.5-fold increase in the cerebrum when compared with normal controls. The sulfated polysaccharide consisted mainly of heparan sulfate, which was of low molecular size, heterogeneous in charge, and rich in its sulfamino hexose content. In addition, the gangliocytes of cerebrum and cerebellum had accumulated glycolipids. Electron microscopically the storage cells were overloaded with lysosomal residual bodies. The mitral valve was also involved in the storage process, which is a rare manifestation of the Sanfilippo syndrome. Acid mucopolysaccharides were deposited intracellularly as well as extracellularly in the mitral valve tissue. Polarisation microscopically there was found a change from normally positive to negative birefringence in the connective tissue ground substance of the mitral valve when containing stored mucopolysaccharides.</p>","PeriodicalId":75583,"journal":{"name":"Beitrage zur Pathologie","volume":"160 1","pages":"58-72"},"PeriodicalIF":0.0,"publicationDate":"1977-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11612399","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Immunofluorescence studies on formalin-fixed and paraffin-embedded material","authors":"C. Witting","doi":"10.1016/S0005-8165(77)80084-X","DOIUrl":"10.1016/S0005-8165(77)80084-X","url":null,"abstract":"<div><p>In an attempt to apply immunomorphologic techniques to formalin-fixed material, various tissues were studied by indirect immunofluorescence. To minimize nonspecific background staining, formalin-fixed paraffin sections were treated with pronase prior to incubation with the antisera. This technique was published by Huang in 1975 to study hepatitis B core and surface antigen in formalin-fixed liver-tissue.</p><p>Our examination demonstrate that this technique might enable the morphologist to identify immunoglobulins in formalin-fixed tissues and so to get new criteria for their diagnosis.</p></div>","PeriodicalId":75583,"journal":{"name":"Beitrage zur Pathologie","volume":"161 3","pages":"Pages 288-291"},"PeriodicalIF":0.0,"publicationDate":"1977-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0005-8165(77)80084-X","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11553179","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Postmortem changes in stereological parameters of cerebral capillaries","authors":"O. Hunziker , A. Schweizer","doi":"10.1016/S0005-8165(77)80080-2","DOIUrl":"10.1016/S0005-8165(77)80080-2","url":null,"abstract":"<div><p>The present study investigated the influence of different postmortem times on stereological parameters of capillaries in the cerebral cortex. For the human investigation different brain regions of two 77 and 79 year-old subjects were examined. The animal experiment, carried out on 10 male cats, allowed a comparison between intravitam deepfrozen cerebral cortex and tissue obtained after decapitation. The parameters were diameter {ie1}, volume fraction {ie2}, surface-to-volume ratio {ie3}, mean minimal distance between capillary centers of gravity {ie4}, length per unit cortex volume {ie5} and number of fragments per measuring field {ie6}, <span>AT</span>.</p><p>Neither the cat experiment nor the human investigation yielded a noteworthy change of the capillary diameter in the postmortem cortical tissue. Nevertheless, a significantly enlarged mean minimal distance between capillaries in the cat cerebral cortex shortly after decapitation (30 sec) suggests the formation of edema which then regresses. Furthermore the experiment revealed that 22 hours after death there is a significantly (p < 0.01) diminished volume fraction and length per unit cortex volume with an augmented surfaceto-volume ratio. These changes are thought to be a consequence of water loss. In contrast to the animal experiment the human cerebral capillaries showed no changes in stereological parameters at two different postmortem times. These results encourage continuation of further stereological investigations on human brains obtained at autopsy and may contribute to the understanding of the aging process in the human cerebral cortex.</p></div><div><p>Die vorliegende quantitativ-histologische Untersuchung umfaβt ein Tierexperiment mit 10 männlichen Katzen sowie menschliches, autoptisch gewonnenes Groβhirnmaterial von zwei 77- und 79jährigen Männern. Sie dient dem Studium des Einflusses verschiedener Gewebsentnahmezeiten nach dem Tod auf stereologische Kapillarparameter der Groβhirnrinde. Das Tierexperiment erlaubte den Vergleich zwischen intra vitam eingefrorenem Groβhirnkortex mit nach Dekapitation entnommenem Gewebe. Folgende Parameter alkalischer Phosphatase-positiver Kapillaren wurden ermittelt: Durchmesser Di, Volumenfraktion Vv<sub>i</sub>, Oberflächen-Volumenverhältnis S<sub>i</sub>/V<sub>i</sub>, mittlerer minimaler Abstand zwischen kapillaren Schwerpunkten Δ<span>ab</span>, Länge pro Volumeneinheit Kortexgewebe Lv<sub>i</sub> und Anzahl Kapiüarstücke pro Meβfeld N<sub>i</sub>, <span>at</span>.</p><p>Der Kapillardurchmsser des post mortem entnommenen Rindengewebes zeigte weder experimentell noch bei der humanen Untersuchung erhebliche Veränderungen. Ein signifikant vergröβerter minimaler Kapillarabstand im Groβhirnkortex der Katze 30 sec nach Dekapitation läβt die Bildung eines ödems vermuten, das aber mit der postmortalen Zeit abnahm. Experimentell, 22 Std. post mortem festgestellte, signifikant (p < 0,01) verkleinerte Volumenfraktion und Länge pro Volumeneinh","PeriodicalId":75583,"journal":{"name":"Beitrage zur Pathologie","volume":"161 3","pages":"Pages 244-255"},"PeriodicalIF":0.0,"publicationDate":"1977-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0005-8165(77)80080-2","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11806866","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
G. Mazzocchi, G. Neri, A.S. Belloni, C. Robba, G.G. Nussdorfer
{"title":"Investigations on the turnover of adrenocortical mitochondria. XI. Effects of dexamethasone on the half-life of mitochondria from the rat zona fasciculata","authors":"G. Mazzocchi, G. Neri, A.S. Belloni, C. Robba, G.G. Nussdorfer","doi":"10.1016/S0005-8165(77)80078-4","DOIUrl":"10.1016/S0005-8165(77)80078-4","url":null,"abstract":"<div><p>The effects of dexamethasone on the half-life of rat adrenocortical mitochondria were investigated by high resolution autoradiography and liquid scintillation spectroscopy. According to the method employed, the half-life averaged 11.27 and 10.46 days. Dexamethasone was found to decrease significantly this parameter (to about 5 days). Since dexamethasone-treated rats can be regarded as pharmacologically hypophysectomized animals, the data indicates that ACTH is involved in the maintenance of the half-life of adrenocortical mitochondria.</p></div>","PeriodicalId":75583,"journal":{"name":"Beitrage zur Pathologie","volume":"161 3","pages":"Pages 221-229"},"PeriodicalIF":0.0,"publicationDate":"1977-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0005-8165(77)80078-4","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11419216","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Effect of early nutrition on the development of mouse epididymal fat pads: cellularity of adipocyte and stromal cell compartment","authors":"L. Rakow","doi":"10.1016/S0005-8165(77)80081-4","DOIUrl":"10.1016/S0005-8165(77)80081-4","url":null,"abstract":"<div><h3>Introduction</h3><p>The size of the adipose tissue depot in man and animals is ultimately dependent upon the number and size of its constituent cells. Thus, the final dimension achieved by this organ will be modified by factors that exert their effect on cell division or cellular enlargement. It has been demonstrated earlier (Rakow et al., 1971a) that obesity induced in adult animals is due to an increase in adipocyte size only, and therefore resembles a cellular hypertrophy of adipose tissue. Furthermore it could be demonstrated that the number of stromal cells (e.g., fibroblasts and cells of the wall of vessels) within the adipose tissue shows a marked increase. Considering the results obtained by other authors (Knittle et al., 1968), it was desirable to establish whether obesity induced during the suckling period of animals is accompanied by an increase in adipocyte number only, resembling a numerical hypertrophy of adipose tissue, and if this type of obesity is accompanied by an increase in stromal cell number.</p></div><div><h3>Material and methods</h3><p>In the present report we have examined the effect of early nutrition on the cellularity in the epidedymal fat pad of NMRI albino mice. Caloric intake was varied during the suckling period by manipulating litter size immediately after birth in a manner similar to that described by Parkes (1926). At birth the litters of a large number of mothers were redistributed to give some mothers a litter of four and others a litter of twelve. After weaning (21 days), all animals had free access to food. Ten animals in each experimental group were sacrificed at three and six weeks and at three and six months of age. Both epididymal fat pads were removed intact for determination of wet weight, adipocyte size and number, whole DNA-content and cytophotometric DNA-measurement of cell nuclei. One of both fat pads of each animal was used for chemical investigation. After PCA-treatment (Rakow et al, 1971b), the DNA-content (Burton, 1956) was determined in the supernatant. The remaining second fat pad was used for calculations of cell numbers (Rakow et al., 1975a). For this reason adipocytes were isolated according to Rodbell (1964). The adipocyte diameters were determined microscopically and the average mass of the adipocytes was estimated. The number of adipocytes was calculated from the wet weight of the fat pad and the average adipocyte mass. The remaining suspension of adipocytes and stromal cells was utilized for cell smears. These smears were stained with Schiffs reagent (Feulgen et al., 1924). The average relative DNA-content of single cell nuclei was measured with an integrating microdensitometer (Deeley, 1955); and the ploidy patterns were estimated. The number of stromal cells was calculated from the whole DNA-content of the fat pads and the DNA-content of the adipocytes.</p></div><div><h3>Results</h3><p>Significant elevations in body weight of the animals raised in small litters are seen at ","PeriodicalId":75583,"journal":{"name":"Beitrage zur Pathologie","volume":"161 3","pages":"Pages 256-266"},"PeriodicalIF":0.0,"publicationDate":"1977-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0005-8165(77)80081-4","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11767502","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Extremly high frecuency of infantil pulmonary thrombo-embolism","authors":"K. Brass","doi":"10.1016/S0005-8165(77)80083-8","DOIUrl":"10.1016/S0005-8165(77)80083-8","url":null,"abstract":"<div><p>Among 5 875 children with a lifetime between 24 hours to 14 years, autopsiated during period 1951-1970, were found 115 cases of naked eye pulmonary thrombo-embolism. 96 cases occurred in children with a lifetime between 24 hours and 4 years. This extremly high frequency is caused by an unusualy high number of thrombosis in renal veins and craneal sinuses, following severe diarrhetic diseases with deshydratation and haemoconcentration Severity and frequency of this enterocolitis of different aetiology, affecting especially children of poor people, are favoured by the climatic, sanitarious and socio-economical conditions of Valencia. Surprisingly, literature do not mention comparables observations about infantil pulmonary thrombo-embolism, observed in territories of similar structure.</p></div><div><p>Unter 5 875 Autopsien von Kindern im Alter zwischen 24 Stunden und 14 Jahren finden sich 115 (1,97%) makroskopisch feststellbare Lungenembolien. Im gleichen Zeitraum (1951-1970) wurden unter rund 8000 Nekropsien Erwachsener nur 544 (6,8%) Lungenembolien angetroffen. 98 der 115 infantilen Lungenembolien entfielen auf Kleinkinder bis zum Abschluβ des 4. Lebensjahres. Die von uns — vor allem an Kleinkindern — beobachtete Lungenemboliefrequenz ist die höchste jemals in einem gröβeren Kinderkollektiv nachgewiesene. Sie geht hauptsächlich zu Lasten einer ungewöhnlichen Häufung sinusaler Thrombosen, die sich im tropischen Klima Valencias nicht selten im Gefolge verschiedenartiger Durchfallkrankheiten mit allgemeiner Deshydratation und Hämokonzentration einstellen. Die betroffenen Kinder entstammen überwiegend armen Bevölkerungsschichten. Unerfahrenheit der Eltern und ungünstige sozio-ökonomische Struktur jener Bevölkerungsgruppe dürften für die hohe Lungenemboliequote unseres Materials daher mitverantwortlich sein.</p></div>","PeriodicalId":75583,"journal":{"name":"Beitrage zur Pathologie","volume":"161 3","pages":"Pages 283-287"},"PeriodicalIF":0.0,"publicationDate":"1977-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0005-8165(77)80083-8","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11806867","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Persigehl , H. Schicha , K. Kasperek , H.J. Klein
{"title":"Trace element concentration in human organs in dependence of age","authors":"M. Persigehl , H. Schicha , K. Kasperek , H.J. Klein","doi":"10.1016/S0005-8165(77)80077-2","DOIUrl":"10.1016/S0005-8165(77)80077-2","url":null,"abstract":"<div><p>There is evidence from epidemiological studies that concentration of various trace elements in human organism is not only changed by different diseases but also by the environment. Interpretation of such data requires definition of normal concentration of trace elements in the different organs.</p></div><div><h3>Material and Methods</h3><p>In 21 patients aged o to 83 years, skin, lung, heart, liver, renal medulla, renal cortex, aorta abdominalis, and brain were assayed. All samples were dried at 100° C for two days. Instrumental neutron activation analysis was used to determine the element concentration of Co, Fe, Zn, Rb, Se, Cr, Sb, Sc, Cs.</p></div><div><h3>Results</h3><p>The behaviour of several trace elements was not uniform in the different organs. The concentrations of all elements decreased in the skin apart from the non-essential element Cs. The content of all essential and non-essential elements increased in liver and kidneys after the first year of age. The behaviour of all elements was not uniform in lung, heart, aorta, and brain. The concentration of the non-essential elements Sb, Sc, and Cs were raised in nearly all organs except the skin with progressing age; only Sb showed a slight decrease in heart and aorta. Apart from the skin, the concentrations of Co and Fe increased in all organs after the first year of life. The behaviour of the elements Zn, Cr, Se, and Rb varied from organ to organ.</p></div><div><h3>Discussion</h3><p>The data indicate that the trace element concentrations in human organs are no fixed values. However, they depend on various factors. The definition of normal concentrations of some trace elements has also to regard its function of age. The different alterations of essential trace element concentrations in the varying organs indicate that they are subject to control mechanisms and not a consequence of simple storage, whereas the increasing concentrations of the non-essential elements with progressing age should be explained by storage mechanism.</p></div>","PeriodicalId":75583,"journal":{"name":"Beitrage zur Pathologie","volume":"161 3","pages":"Pages 209-220"},"PeriodicalIF":0.0,"publicationDate":"1977-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0005-8165(77)80077-2","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11807080","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
W.J. Mergner, L. Marzella, C. Mergner, M.W. Kahng, M.W. Smith, B.F. Trump
{"title":"Studies on the pathogenesis of ischemic cell injury VII. Proton gradient and respiration of renal tissue cubes, renal mitochondrial and submitochondrial particles following ischemic cell injury","authors":"W.J. Mergner, L. Marzella, C. Mergner, M.W. Kahng, M.W. Smith, B.F. Trump","doi":"10.1016/S0005-8165(77)80079-6","DOIUrl":"10.1016/S0005-8165(77)80079-6","url":null,"abstract":"<div><p>Electron transport in tissue cubes, isolated mitochondria and submitochondria particles were examined as a function of ischemie time. It was found that electron transport remains active in all systems beyond the 2 hour ischemie time interval. The NADH stimulated respiration, however, declined after 2 hours of ischemia in ASU (Ammonia-Sephadex-Urea) particles followed by respiration with matrix-located dehydrogenases tested by substrates such as glutamate, α-ketoglutarate and pyruvate plus malate. Succinate dependent respiration remains active at control levels. In contrast proton gradient reveals changes in two phases: Phase A is characterized by gradually increasing gradient without valinomycin and by a rapidly declining gradient with valinomycin in the medium. Phase B is characterized by a declining proton gradient with or without valinomycin. It is suggested that the alteration of the proton gradient between 1 and 2 hours ischemia is an important factor contributing to irreversible cell injury.</p></div>","PeriodicalId":75583,"journal":{"name":"Beitrage zur Pathologie","volume":"161 3","pages":"Pages 230-243"},"PeriodicalIF":0.0,"publicationDate":"1977-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0005-8165(77)80079-6","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"11807081","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}