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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 64 (1986), S. 342-347 
    ISSN: 1432-1440
    Keywords: Myoadenylate deaminase deficiency ; Histochemistry ; Biochemistry ; Metabolic myopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary This report concerns two unrelated males; one had sarcoidosis, sarcoid myopathy and muscle weakness, and the other had exercise-induced weakness and myalgia. Both patients had a lack of ammonia rise in their serum after an ischemic work test, minimal histochemical activity of myoadenylate deaminase in repeated muscle biopsies, and less than 5% of normal biochemical activity of myoadenylate deaminase in their skeletal muscles. These three criteria establish primary myoadenylate deaminase deficiency as a separate primary metabolic muscle disease which merits differential diagnostic consideration when patients complain of muscle weakness and cramps.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1440
    Keywords: PAH ; PAH-metabolism ; N-acetylation ; renal artery stenosis ; essential hypertension ; PAH ; PAH-Metabolismus ; N-Acetylierung ; Nierenarterien-Stenose ; essentielle Hypertonie
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Routinemäßige PAH-Clearance-Untersuchungen wurden durchgeführt bei Patienten mit a) Glomerulonephritis, b) sekundärer Beeinträchtigung der Nierenfunktion, c) essentieller Hypertonie und d) bei Patienten mit einseitiger Nierenarterien-Stenose. Die renale Ausscheidung von N-Acetyl-PAH war signifikant erniedrigt bei Patienten mit Nierenarterien-Stenose oder mit essentieller Hypertonie, während die anderen Gruppen keine Störung des PAH-Metabolismus aufwiesen. Die Bedeutung dieser Befunde wird diskutiert.
    Notes: Summary Routine PAH-clearance studies were carried out on patients with (a) glomerulonephritis, (b) secondary impairment of renal function, (c) essential hypertension, and (d) patients with unilateral renal artery stenosis. Renal excretion of N-acetyl-PAH was significantly lower in patients with unilateral artery stenosis or with essential hypertension, while the other groups showed no impairment of the PAH-metabolism. The implications of these findings are discussed.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1434-3916
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Eine 33jährige Dialysepatientin litt an einer Osteomyelitis und einer cervikalen Myelopathie bei Luxation des Dens axis. Bei der Patientin war in der Vergangenheit dreimal ein anaphylaktischer Schock aufgetreten, nach Behandlung mit Dialysatoren, die mit Ethylenoxid sterilisiert worden waren. Eine Ethylenoxid-Allergie mit Nachweis von IgE-Antikörpern gegen Komplexe aus Humanalbumin und Ethylenoxid konnte mit der Radioallergosorbent-Technik (RAST) gesichert werden. Unmittelbar im Anschluß an eine operative Stabilisierung der Halswirbelsäule mit Knochenzement (Palacos R) kam es zu einem Quincke-Ödem mit massiven Schwellungen des Larynx, Pharynx und der Zunge. Palacos wird mit Ethylenoxid sterilisiert. Wir schließen daraus, daß restliches Ethylenoxid im Knochenzement bei sensibilisierten Patienten zu akuten allergischen Reaktionen führen kann. Dialysepatienten sind als besondere Risikogruppe für solche anaphylaktischen Reaktionen anzusehen, da die Inzidenz einer Ethylenoxid-Allergie in dieser Patientengruppe bei etwa 10% liegt.
    Notes: Summary A 33-year-old female dialysis patient suffered from osteomyelitis and luxation of the dens axis with cervical myelopathy. In the past she had had three attacks of anaphylaxis after treatment with dialyzers that had been sterilized with ethylene oxide. IgE-type antibodies directed against human serum albumin-ethylene oxide complexes could be demonstrated in the patient's serum by radioallergosorbent techniques. Immediately after an operation in which acrylic bone cement (Palacos-R) sterilized with ethylene oxide was implanted for stabilization of the cervical spine, the patient developed massive edema of the larynx, pharynx, and tongue, suggesting Quincke's edema. It is concluded that ethylene oxide present in acrylic bone cement may induce acute allergic reactions in sensitized patients. Dialysis patients may be at special risk, since the incidence of ethylene oxide allergy in this patient population is about 10%.
    Type of Medium: Electronic Resource
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