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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 397 (1982), S. 183-192 
    ISSN: 1432-2307
    Keywords: Familial cushing's syndrome ; Familial cardiac myxomas ; Adrenocortical nodular dysplasia ; Adrenocortical microadenomatosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In a family with 4 children, 2 had slowly developing Cushing's syndrome and were adrenalectomized between the ages of 18 and 28 years. As in other cases with familial Cushing's syndrome, primary adrenocortical nodular dysplasia, the so-called adrenocortical adenomatosis, was demonstrated. The brother, now 39 years old, is in good health. The sister, however, died at the age of 36 years. Autopsy revealed a cardiac myxoma of the left atrium. A cardiac myxoma had been found incidentally at autopsy of these two patients' oldest brother, a boy who died at the age of 4 years. Both siblings with Cushing's syndrome presented additional Peutz-Jegher-like hyperpigmentation and myxomatous tumours of the skin. Identical observations of familial adrenocortical nodular dysplasia, Cushing's syndrome and cardiac myxomas have not been reported to date. However, the familial occurrence of cardiac myxomas and adrenocortical dysplasia in combination with bilateral large cell calcifying Sertoli cell tumours of the testis has been published recently.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 381 (1979), S. 269-281 
    ISSN: 1432-2307
    Keywords: Testis ; Testicular biopsies ; Arteriolar hyalinosis ; Morphology ; Frequency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Das häufige Vorkommen arteriolärer Hyalinosen in Hoden verstorbener Patienten ist bekannt. Gleiche Gefäßveränderungen können auch in Hodenbiopsien und operativ entfernten Hoden gefunden werden. Um uns ein Bild über die Häufigkeit machen zu können, untersuchten wir eine Serie von 2400 Hodenbiopsien und einzelnen operativ entfernten Hoden systematisch lichtmikroskopisch, teils auch histochemisch und elektronenoptisch im Hinblick auf arterioläre Hyalinosen. Dabei konnten in 3,75% der Patienten typische Herde nachgewiesen werden (Durchschnittsalter 34 Jahre). An Hodengrundkrankheiten findet man vor allem tubuläre Atrophien (58%) oder Kryptorchismus (22% der Fälle mit Gefäß Veränderungen). Die histochemischen Reaktionen lassen vermuten, daß der Hauptteil der eingelagerten Substanz aus Lipoiden, besonders Lecithinen besteht. Daneben sind auch Mukopolysaccharide abgelagert. Elektronenoptisch handelt es sich vorwiegend um fein granuläre Depots mit Vakuolen und seltenen Myelinfiguren. In späteren Stadien ist subendothelial ferner elastinartiges Material zu sehen, an der Peripherie der Herde sind Kollagenfasern nachweisbar. Die pathogenetische Bedeutung dieser arteriolären Hyalinoseherde und ihre Ursache sind unklar.
    Notes: Summary Arteriolar hyalinosis is a common post mortem finding in the testes of even young men. Identical arteriolar hyalinoses can be demonstrated in testicular biopsies of patients with infertility or in patients operated on for cryptorchidism. In a series of such biopsies from 2400 patients, the frequency of arteriolar hyalinosis was examined. In 7 cases with positive findings, histochemical studies were carried out and in an additional 14 biopsies electron microscopy was performed. Arteriolar hyalinosis was found in 3.75% of the 2,400 patients with disturbances of fertility or cryptorchidism. The mean age of these patients was 34 years. In 58% of the cases with arteriolar hyalinosis the basic testicular lesion was tubular atrophy, in 22% cryptorchidism. The arteriolar lesions were due to deposits of lipoids and mucopolysaccharides. Electron microscopy revealed a granular material with vacuoles and rarely myelin bodies below the endothelial layer and between myocytes and fibrocytes of the arteriolar wall. In later stages elastin-like material could be demonstrated in the vicinity of the endothelial cells and collagen fibers at the periphery of these deposits. The etiology of testicular arteriolar hyalinosis and its pathogenetic significance are not yet clear.
    Type of Medium: Electronic Resource
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