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  • 1
    Electronic Resource
    Electronic Resource
    Thromboembolisehe Komplikationen und Thrombocytenanomalien bei Homocystinurie (1972)
    Springer
    European journal of pediatrics 112 (1972), S. 309-320 
    Details
    ISSN: 1432-1076
    Keywords: Homocystinuria ; Thromboembolism ; Platelets
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung In der vorliegenden Arbeit wird über 7 Patienten mit Homocystinurie berichtet. Bei 4 von ihnen traten thromboembolische Komplikationen auf, die zweimal Todesursache waren. Autoptisch konnten in diesen beiden Fällen Sinus- und Gehirnvenenthrombosen sowie Hirnerweichungsherde nachgewiesen werden. Bei 6 Patienten mit Homocystinurie wurden die Thrombocyten isoliert und elektronenoptisch untersucht. Als konstanter befund zeigte sich eine erhebliche Vacuolisierung im Cytoplasma der Thrombocyten. Diese Veränderung werden als Hinweis für die thrombocytogene Natur, der Gerinnungsstörung bei Homocystinurie angesehen.
    Notes: Abstract 7 patients with homocystinuria are reported. In 4 of them thromboembolic complications occurred, resulting in death of 2 patients. The postmortem examination in both of these cases showed thromboses of sinus and cerebral veins as well as encephalomalacic lesions. In 6 patients with homocystinuria platelets were isolated and investigated by electron microscopy. A constant finding was a distinct vacuolization of the platelet cytoplasm. These changes were regarded as an indication of the thrombocytogenic nature of the coagulation disorder in homocystinuria.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00438625
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  • 2
    Electronic Resource
    Electronic Resource
    Vacuolated lymphocytes in type II glycogenosis —A diagnostic approach? (1977)
    Springer
    European journal of pediatrics 127 (1977), S. 1-7 
    Details
    ISSN: 1432-1076
    Keywords: Glycogenosis type II ; Lymphocytes ; Electron microscopic examination ; Diagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Using electron microscopy, glycogen-filled lysosomes were found in peripheral lymphocytes in 5 cases of the infantile form of glycogenosis type II. In two infants whose blood smears were available, the ultrastructural demonstration of this pathognomonic storage corresponded to well-delineated vacuoles detected by routine light microscopy. Detection of such vacuoles in peripheral lymphocytes by light microscopy and demonstration of glycogenfilled lysosomes by electron microscopy could be a simple and harmless tool for diagnosing the classical form of type II glycogenosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00465559
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    The clinical findings in a patient with nonketotic hyperglycinemia (1973)
    Springer
    European journal of pediatrics 114 (1973), S. 191-204 
    Details
    ISSN: 1432-1076
    Keywords: Nonketotic hyperglycinemia ; Crystalline inclusions in lysosomes of liver parenchymal cells ; Glycine and serin free diet
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über einen weiteren Fall der nichtketotischen Form der Hyperglycinämie berichtet. Hyperglycinämie, Hyperglycinurie, mentale Retardierung, das Fehlen von Neutropenie, Thrombocytopenie, Acidose und Ketose sowie In vitro-Bestimmung der Glycin-Serin-Konversion im Lebergewebe bestätigen die Diagnose. Elektronmikroskopisch konnten in den Lysosomen der Leberparenchymzellen osmiophobe, kristalline Einschlüsse gefunden werden, die möglicherweise durch gesteigerte Glycinkonjugation entstandene Hippursäure sein könnten. Nach Einstellung auf eine glycin- und serinfreie Diät besserte sich das pathologisch gestörte EEG. Krämpfe wurden nicht mehr festgestellt. Der Glycinspiegel im Serum senkte sich deutlich. Die zusätzliche Gabe von Cholin und Benzoesäure bewirkte keine weitere Senkung des Glycinspiegels.
    Notes: Abstract A new case of nonketotic hyperglycinemia is reported. Hyperglycinemia, hyperglycinuria and mental retardation without neutropenia, thrombocytopenia, acidosis, ketosis, and the in vitro study of glycine-serine conversion in liver homogenate confirmed the diagnosis. Using electronmicroscopy osmiophobe, crystalline inclusions were found in lysosomes of liver parenchymal cells. These inclusions could be hippuric acid produced by increased conjugation of glycine. Under a glycine and serine free diet the pathologic EEG improved. Seizures did not recur. Glycine level in serum decreased. Administration of cholin and benzoic acid did not lower the glycine level in serum.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00497423
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  • 4
    Electronic Resource
    Electronic Resource
    Klinische, pathologische und biochemische Untersuchungen in einem Fall von infantiler generalisierter Gangliosidose (GM1-Mucolipidose) (1972)
    Springer
    European journal of pediatrics 112 (1972), S. 23-42 
    Details
    ISSN: 1432-1076
    Keywords: Gangliosidosis ; Absence of β-galactosidase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Schilderung eines Falles von infantiler generalisierter Gangliosidose infolge Fehlens der β-Galaktosidase. Dabei werden das Gangliosid GM1 und ein Keratan-ähnliches Mucopolysaccharid in Ganglienzellen, Leber, Milz, Niere und Knochenmark gespeichert. Die Eltern des Kindes sind blutsverwandt. Bei einem unter der Geburt verstorbenen Geschwisterkind konnten zwar die gleichen morphologischen Speicherphänomene, nicht jedoch der Enzymdefekt nachgewiesen werden. Auffällig ist die Steigerung der Aktivität der β-N-Acetyl-Hexosaminidase bei dem Geschwisterkind und in Fibroblasten der Eltern.
    Notes: Abstract Report on a case of infantile generalized gangliosidosis due to deficiency of β-galactosidase. There is accumulation of ganglioside GM1 and a Keratanlike mucopolysaccharide in the brain and viscera. The parents are consanguineous. In a sibling who died during delivery the same morphological phenomena of storage were found but no enzyme deficiency. The activity of β-N-acetyl-hexosaminidase was elevated in the sibling and in fibroblasts of both parents.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00491963
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  • 5
    Electronic Resource
    Electronic Resource
    A metabolic disorder similar to Zellweger syndrome with hepatic acatalasia and absence of peroxisomes, altered content and redox state of cytochromes, and infantile cirrhosis with hemosiderosis (1977)
    Springer
    European journal of pediatrics 124 (1977), S. 261-275 
    Details
    ISSN: 1432-1076
    Keywords: Acatalasia ; Cerebro-hepato-renal syndrome ; Cirrhosis ; Hemochromatosis ; Cytochrome abnormalities ; Peroxisomes ; Mitochondria ; Oxidative phosphorylation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A patient with a cerebro-hepato-renal syndrome was investigated. The visceral manifestations were those of the Zellweger syndrome (ZS); however, the child exhibited muscular hypertonia and survived into the 2nd year of life. Ultramicroscopically, hepatocytes were lacking peroxisomes, but, contrary to findings in one patient with ZS [2], contained smooth endoplasmic reticulum. No catalase was found by histochemistry or spectroscopy. Mitochondria showed normal succinate and glutamate respiration, and normal coupling of respiration to the phosphorylation potential. The cytochrome (cyt) content was diminished to one-third with an abnormally inversed redox patterns of the respiratory chain in the controlled state, cyt b being 5%, cyt c 23% reduced. The oxygen affinity of cyt a 3 was normal. These findings exclude a defect in the nonheme iron protein region of the respiratory chain as described in ZS [2], but point to a functional abnormality of cyt b in our patient.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441934
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  • 6
    Electronic Resource
    Electronic Resource
    Feinstrukturelle Aspekte der membranösen Glomerulonephritis bei nephrotischem Syndrom (1971)
    Springer
    Virchows Archiv 353 (1971), S. 248-260 
    Details
    ISSN: 1432-2307
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung 52 Nierenpunktate von Erwachsenen aus Deutschland und Japan wurden elektronenmikroskopisch untersucht. Während bei frischen Fällen charakteristische elektronendichte Ablagerungen im Epimembraneum nachgewiesen werden, sind diese Ablagerungen bei länger verlaufender Erkrankung in der epithelialen Seite der unregelmäßig verdickten Basalmembran lokalisiert. Die älteren Ablagerungen sind von geringerer Elektronendichte. Zwischen diesen Ablagerungen und den Podocyten liegt eine neu gebildete Basalmembran. Die Häufigkeit der membranösen Glomerulonephritis bei nephrotischem Syndrom ist in unserem Untersuchungsgut geringer als allgemein angenommen. Unterschiede zwischen deutschen und japanischen Patienten sind qualitativ und quantitativ nicht feststellbar. Der Mischtyp der membranösen proliferativen Glomerulonephritis und sein klinisches und feinstrukturelles Verhalten wird diskutiert.
    Notes: Summary The electron microscopic findings in membranous glomerulonephritis from 27 German and 25 Japanese adult patients with nephrotic syndrome have been described. At the beginning of the disease massive deposits were seen in the epimembraneum of the capillary loops. In chronic cases these deposits were found at the epithelial side of the basement membrane, which also showed marked but irregular thickening. The older deposits become less electron dense or even translucent while new basement membrane was synthetized between the deposits and the podocytes. No qualitative relationship was found to exist between the thickening of the basement membrane due to the deposition of dense materials and clinical data of the patients. The frequency of membranous glomerulonephritis in nephrotic syndrome was less than had been expected. There were no differences qualitatively and quantitatively between German and Japanese cases. The mixed type of membranous and proliferative glomerulonephritis and its clinicopathological behaviour are discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00545733
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  • 7
    Electronic Resource
    Electronic Resource
    216. Operationsausmass und Prognose beim Hämangiopericytom (1987)
    Springer
    Langenbeck's archives of surgery 372 (1987), S. 860-860 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF01298000
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