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  • 1
    ISSN: 1432-1440
    Keywords: Hyperoxaluria Type I ; Chronic renal failure ; Kidney and liver transplantation ; Childhood
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The difficulties of biochemical diagnosis in children and in chronic renal failure are discussed in detail, as well as the development of diagnostic and therapeutic possibilities in recent years, exemplified by 4 cases. Excretion of oxalate (and glycolate) may be incorrectly assumed to be normal with: a) uncritical application of the method of measurement, b) disregard of the clearly lower oxalate excretion in children (values should be referred to m2 of body surface), c) disregard of a decreased glomerular filtration rate (values should be referred to the creatinine clearance). With compromised renal function the excretion of oxalate and glycolate in primary oxalosis drops to “normal” whereas plasma values increase considerably. In this case the biochemical diagnosis is possible only by measurement of plasma values of glycolate and oxalate. Consequently, extensive extrarenal deposition of calcium oxalate crystals will, as a rule, become clinically manifest only after chronic renal failure has turned irreversible. In recent years, several therapeutic procedures, have been developed. They are of therapeutic significance for the early stages of the disease as well. Observing especially conditions renal transplantation or combined hepatorenal transplantation can be managed with a successful outcome. As the perioxisomal enzyme is activated only in the liver cells, an early liver transplantation as a definitive treatment by enzyme replacement may be the successful therapy in the future.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Langenbeck's archives of surgery 372 (1987), S. 860-860 
    ISSN: 1435-2451
    Keywords: Dubious histopathological diagnosis ; Extent of surgery ; Prognosis ; Zweifelhafte Dignität ; Radikaloperation ; Prognose
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Das Hämangiopericytom ist ein sehr seltener Gefässtumor, der von den Pericyten ausgeht und deshalb im gesamten Organismus auftreten kann. Pathohistologisch ist eine sichere Aussage über die Dignität nicht möglich. Mindestens 50% der Patienten haben einen malignen Verlauf. Bei 12 Patienten in unserer Klinik mit histologisch malignem Hämangiopericytom hatten nur diejenigen eine Überlebenszeit von mehr als 2 Jahren, bei denen der Tumor radikal im Gesunden entfernt werden konnte. Wir folgern aus unseren Erfahrungen, dass dieser Tumor immer als potentiell maligne angesehen und dementsprechend operiert werden muss.
    Notes: Summary The haemangiopericytoma is a very rare vascular tumor, which is produced by the pericytes and can therefore arise anywhere in the entire organism. It is impossible to assess the malignancy with histopathological methods. At least 50% of the patients suffer a malignant growth in the followup. Of 12 patients with a histologically malignant haemangiopericytoma, only those whose tumor could be resected radically survived more than 2 years. We conclude from our experience that this kind of tumor should always be regarded as malignant and should be treated accordingly.
    Type of Medium: Electronic Resource
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