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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European archives of oto-rhino-laryngology and head & neck 217 (1977), S. 475-485 
    ISSN: 1434-4726
    Keywords: Tangier disease ; Disorder of fat metabolism ; Yellow-orange enlargement of tonsills and adenoids ; Cholesteryl-ester storage in histiocytes ; Morbus Tangier ; Fettstoffwechselstörung ; Orange-gelbliche Verfärbung der Tonsillen ; Cholesterinesterspeicherung in Histiocyten
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über die ersten in Deutschland entdeckten Patienten mit einer Hypo-α-Lipoproteinämie — Tangier disease — berichtet. Diese Fettstoffwechselstörung zeichnet sich durch eine Verminderung der HDL-Lipoproteine im Serum sowie durch Cholesterinspeicherung in Histiocyten aus. Hervorstechendes Merkmal ist eine orange-gelbliche Verfärbung des lymphatischen Rachenringes; die weiteren Symptome können vielgestaltig sein, der Verlauf der Erkrankung ist wenig typisch. Als Ausdruck der wahrscheinlich autosomal rezessiv vererbten Fettstoffwechselstörung werden hauptsächlich Cholesterinester im Reticuloendothelialen System (z. T. in kristalliner Form mit Doppelbrechung) gespeichert, in geringerem Maße auch in glatten Muskelzellen, Pericyten und Schwannschen Zellen. Der Morbus Tangier kann durch laborchemische und ultrastrukturelle Kriterien von anderen Fettstoffwechselstörungen abgegrenzt werden. Eine causale Therapie ist nicht bekannt, der HNO-Arzt kann die sicher häufiger vorkommende Krankheit frühzeitig erkennen.
    Notes: Summary The report deals with the first three cases of Tangier disease (Hypo-α-Lipoproteinaemia) observed in Germany. This rare metabolic disorder is distinguished by a diminution of HDL-Lipoproteins in serum and a lipid storage in histiocytes. The prominent symptom is the orange yellow discolouration of hyperplastic tonsills and adenoids; further symptoms of the disease generally are various, the development is little typical. The morphological substrat of the deranged fat metabolism is cholesteryl ester storage in the reticuloendothelial system, smooth muscle cells, pericytes and Schwann cells of peripheral nerves. M. Tangier can be differentiated from other lysosomal defects by ultrastructural and chemical parameters, it seems to have a autosomal recessiv inheritance. It is the ENT-specialist, who can establish an early diagnosis of this disease, provided he is aware of its clinical features.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European archives of oto-rhino-laryngology and head & neck 216 (1977), S. 623-624 
    ISSN: 1434-4726
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In order to induce a serous otitis media in cats, the pharyngeal opening of the Eustachian tube was cauterized and obstructed on one side only through transpalatal approach; the other side remaind intact and served as control. On tympanic membrane examination and puncture 11 months later, there was fluid in the middle ear in three ears, no fluid in two ears, a tympanic membrane perforation in one ear and a red tympanic membrane in one ear. The middle ear was then ventilated through a plastic tube in two ears with and without fluid respectively. In all other ears, no tube was inserted. After another 7 months, the animals were intravitally perfused, and the middle ear mucosa biopsyed. The histological results of the light and electron microscopy showed chronic changes of the middle ear mucosa following experimental tubal obstruction.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neurochirurgica 37 (1977), S. 75-91 
    ISSN: 0942-0940
    Keywords: Supratentorial astrocytomas ; Recurrences of astrocytomas ; Classification of gliomas ; Recurrence intervals ; Radiotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report 137 recurrent supratentorial astrocytomas. The primary tumours diagnosed on the basis of a grading system with three stages were 72 astrocytomas I and 65 astrocytomas II. In the first group 14% of the recurrences were not changed, 55.5% became astrocytomas II, and 30.5% became glioblastomas. In the second group 55.4% were unchanged, and 44.6% became glioblastomas. The postoperative intervals until reintervention or death were statistically examined. It seems that the recurrence time chiefly depends on the nature of the primary tumour. The transformation of an astrocytoma I to a glioblastoma takes longer than the transformation of an astrocytoma II into a glioblastoma. In about two thirds of all astrocytomas an increase of malignancy is to be expected. From the histological picture it is not possible in an individual case to predict the likelihood or speed of malignant change. With regard to the effect of irradiation the authors conclude that radiotherapy most probably does not produce malignancy.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 0942-0940
    Keywords: Supratentorial oligodendrogliomas ; Recurrences of oligodendrogliomas ; Classification of gliomas ; Recurrence intervals ; Radiotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary On the basis of a three stage grading system we report 23 stage one recurrent oligodendrogliomas (O 1), and 29 stage two recurrent oligodendrogliomas (O 2). In the O 1 group after the first interval 15 became O 2 and 2 became glioblastomas. Twenty tumours of the O 2 group after the first interval were not changed, three became oligodendroglioma-astrocytomas stage 2, and six became glioblastomas. The time relation for the recurrent phase in the primary O 1 group is calculated as 42 months, and in the primary O 2 group as 22 months, but this is without significance. For the development of malignancy, especially for the change to glioblastoma, a prominent participation by transformed local astrocytes seems to be essential. Postoperative irradiation most probably does not favour malignant change. A prolongation of the expectation of life by radiotherapy is not noticed.
    Type of Medium: Electronic Resource
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