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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 135 (1996), S. 0 
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary Cutaneous macroglobulinosis is a rare cutaneous manifestatiou of Waldenstrom's disease. Lesions are thought to result from accumulation of macroglobulin in the dermis and are therefore called IgM storage papules. Ultrastructural findings in the previously reported cases were contradictory and the nature of the deposits was not established by electron microscopy. The purpose of this study was to analyse such deposits by the use of immunoelectron microscopy. A 60-year-old woman had multiple erythemalous papules for 1 year. The histopathological changes consisted of plasmocytic infiltration of the dermis and eosinophiiic deposits. The skin changes and other investigations led to a diagnosis of Waldenstrom's disease. Samples from normal and diseased, skin were analysed by electron microscopy and by immunogold labelling with anti-IgM antibodies, after Lowicryl K4M embedding. An extracellular electron dense granular and filamentous material was observed in the mid- and upper dermis, between and within the collagen bundles. No periodicity was noted and no deposits were seen at the dermoepidermal junction. Immunoelectron mieroscopy showed a positive labelling located only on these deposits, in both normal-appearing skin and in lesions. In this patient, immunoelectron microscopy clearly demonstrated the presence of large amounts of IgM in the dermis, which were found in the lesions of cutaneous macroglobulinosis and in normal skin. These results suggest that the IgM storage papules result from a greater density of deposits rather than a site-specitic accumulation.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 2 (1978), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: A case of a 28-month-old boy with chondroectodermal dysplasia (Ellis-van Creveld syndrome) is reported. Besides polydactyly, ectodermal dysplasia, acromelic dwarfism and congenital heart defect, which are characteristic morphologic features of the syndrome, additional dysplastic developmental defects were discovered in the kidneys, liver, and lungs. Detailed histopathologic studies of the growth plates of tibia, femur and ribs disclosed an irregular, partly hyperplastic, partly dystrophic appearance of the epiphyseal cartilage, which was not resorbed properly by the invading blood vessels. Focal areas of necrosis occurred and barriers of tongue-shaped cartilaginous peninsulas persisted. Regular enchondral ossification was hindered and compensatory membrane ossification was found in the fibrosing metaphyseal bone marrow adjacent to the cartilage. Dysplasia and fibrosis of the renal medulla plus interstitial fibrosing nephritis in the cortex resulted in kidney contraction and renal failure. Hepatomegaly, portal fibrosis and bile duct hyperplasia and dysplasia were detected at autopsy, but did not have any clinical significance. These findings once again emphasize that derivatives of all three germ layers are involved in the Ellis-van Creveld syndrome. The possibility that a single metabolic or structural abnormality of the mesenchymal tissues could be responsible for the various organ dysplasias is discussed.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Journal of Steroid Biochemistry 16 (1982), S. 339-342 
    ISSN: 0022-4731
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Journal of Steroid Biochemistry 20 (1984), S. 663-670 
    ISSN: 0022-4731
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 398 (1982), S. 229-235 
    ISSN: 1432-2307
    Keywords: Birth injury ; Breech presentation ; Brain tissue embolism ; Neonatal myocardial infarction ; Neonatal pulmonary embolism ; Shock
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Brain tissue embolism in the coronary, leptomeningeal and pulmonary arteries was discovered microscopically following the autopsy of a female newborn. Death occurred 8 h after breech delivery, which had been complicated by both arms being turned up beside the head. The dislodged brain tissue originated from the left cerebellar hemisphere and had entered the venous blood stream through a rupture of the left sinus transversus. Both “paradoxical” systemic and pulmonary artery embolization ensued. The baby died from the combined effects of cerebral haemorrhage, pulmonary embolism, myocardial infarction and shock.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Alveoläres Adenom ; Immunhistochemie ; Surfactantapoproteine B und C ; Alveozyten Typ II ; Key words Alveolar adenoma ; Immunohistochemistry ; Surfactant apoproteins B and C ; Alveocytes type II
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The alveolar adenoma of the lung is a rare benign tumor in which the normal parenchymal architectur is imitated by a proliferation of both the alveolar epithelial cells and the mesenchymal septal cells. The first description, based on six cases, was published in 1986 by Yousem and Hochholzer. From their ultrastructural findings they presumed a type II pneumocytes differentiation of the epithelial cells. We investigated an alveolar adenoma of the lung immunhistochemical by means of antibodies against apoprotein B and C of human surfactant. Both the lining cells and the macrophages in the alveolar-like spaces were stained. The septal connection tissue cells did not react. These findings confirm the expression of surfactant constituants and, hence, the differentiation into type II pneumocytes of the epithelial cells of the alveolar adenoma.
    Notes: Zusammenfassung Das alveoläre Adenom der Lunge ist ein seltener gutartiger Tumor, der als kombinierte Proliferation alveolärer epihelialer Zellen und septalen Mesenchyms angesehen wird und normale Lungenparenchymarchitektur nachahmt. Es wurde 1986 zum ersten Mal anhand von 6 Fällen von Yousem u. Hochholzer [11] beschrieben. Die von den Autoren an 2 Fällen durchgeführten ultrastrukturellen Untersuchungen wiesen auf eine Differenzierung der epithelialen Komponente in Richtung Alveozyten Typ II hin. Wir konnten an einem Fall eines alveolären Adenoms immunhistochemische Untersuchungen mit einem Antikörper gegen die Apoproteine B und C des humanen Surfactant durchführen (zur Verfügung gestellt von der Firma Thomae). Dabei zeigten die Epithelzellen, die alveolenähnliche Hohlräume auskleiden sowie in diesen Hohlräumen liegende Makrophagen eine positive Reaktion, wohingegen die Zellen der Bindegewebssepten negativ reagierten. Der immunhistochemische Nachweis der Surfactantapoproteine B und C im Zytoplasma der epithelialen Tumorzellen bestätigen deren Differenzierung in Richtung Alveozyten Typ II.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Cellular and molecular life sciences 38 (1982), S. 1253-1254 
    ISSN: 1420-9071
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Bromocriptine treatment on either prooestrus or oestrus in female rats did not affect luteal function on the day of dioestrus 1.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1076
    Keywords: Glutaric aciduria type II ; Multiple acyl-CoA dehydrogenase deficiency ; Organic aciduria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two male siblings suffering from a severe form of glutaric aciduria type II were studied. One patient died within one hour after birth, the other at the age of five days. Both patients presented with respiratory distress soon after birth. They had a variety of congenital morphologic abnormalities. One patient's outstanding “sweaty-feet” odour on the second day of life led to organic acid analysis of urine revealing massive lactic, glutaric, and ethylmalonic aciduria along with a high excretion of various other carboxylic acids and glycine conjugates of the branched chain carboxylic acids. The pattern of metabolites in serum and urine as well as results of degradation studies with various substrates in cultured fibroblasts were consistent with a defect in multiple acyl-CoA dehydrogenation. The morphological abnormalities are presented in a subsequent paper.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1076
    Keywords: Multiple acyl-CoA-dehydrogenation deficiency ; Glutaric aciduria type II ; Congenital polycystic kidneys ; Fatty liver ; Cerebrocortical dysplasia ; Fetopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The post mortem and microscopic findings of two newborn male sibling of Turkish origin suffering from multiple acyl-CoA-dehydrogenation deficiency (glutaric aciduria type II) are reported in detail for the first time. The morphological disease pattern was strikingly identical in both siblings: enlarged bilateral polycystic kidneys, symmetric warty dysplasia of the cerebral cortex, and bile duct hypoplasia, cholestasis, siderosis and fatty degeneration of the liver were found in both infants. In addition, features of Potter syndrome (pulmonary hypoplasia and ‘Potter face’) were observed only in sibling I, and focal hypoplasia and dysplasia of pancreatic ducts only in sibling II. It appears to be a rather remote chance that the rare metabolic disorder accidently coincided with the equally rare developmental abnormality in both siblings. We believe it to be more likely that both conditions are pathogenetically related in that the accumulation of large quantities of carboxylic acids exerted their effect already in intrauterine life, probably leading to cellular damage and secondary developmental defects of the fetal kidneys, liver, pancreas and brain. From the nature of the observed morphological alterations we speculate that the injury did not occur until after cessation of organogenesis during the fetal period of intrauterine development.
    Type of Medium: Electronic Resource
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