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  • 1
    Electronic Resource
    Electronic Resource
    Lipidmetabolstörung der Leber durch parenteral verabreichtes Desoxycholat bei der Ratte (1973)
    Springer
    Journal of molecular medicine 51 (1973), S. 39-40 
    Details
    ISSN: 1432-1440
    Keywords: Liver damage ; deoxycholate ; lipid metabolism ; rat ; Leberschädigung ; Desoxycholat ; Lipidmetabolismus ; Ratte
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Einmalige intraperitoneale Applikation von 5,75–23 mg Desoxycholat (Na-Salz) an ∼200 g schwere, mannliche Ratten erzeugt innerhalb von 6–8 h schwere Leberschädigungen, nachweisbar an klinischen Allgemeinsymptomen, ultrastrukturellen Alterationen, insbesondere der Mitochondrien, und analytisch durch eine Zunahme der Aktivitäten von GOT, GPT, SDH, GLDH und der freien Fettsäuren im Serum sowie durch die Abnahme an Neutralfetten, freien Fettsäuren und Phospholipiden mit Ausnahme von Lecithin und Phosphatidyläthanolamin in der Leber. Diese Metabolaberrationen zeigen charakteristische Verlaufsformen, von denen die Quotienten Lecithin/Lysolecithin sowie Phophatidyläthanolamin/Lysophosphatidyläthanolamin biphasisch mit einer geringen zeitlichen Phasenverschiebung verlaufen. Die Metabolaberrationen dürften primär durch gestörte Membrantransporte unter Desoxycholat bedingt sein.
    Notes: Summary Single intraperitoneal application of 5.75–23 mg deoxycholate (sodium salt) in ∼200 g heavy male rats produces within 6–8 h severe liver injury, manifested in general clinical symptoms, ultrastructural alterations, especially on mitochondria, analytical evidence by increase of activities of GOT, GPT, SDH, GLDH, and of serum free fatty acids and also by the decrease of neutral fat, free fatty acids and phospholipids, except for lecithin and phosphatidyl ethanolamine in the liver. These metabolic aberrations show a typical course, of which the quotients lecithin/lysolecithin and phosphatidyl ethanolamine/lysophosphatidyl ethanolamine are biphasic with a low periodic fluctuation. With deoxycholate, aberrations of metabolism may be primarily dependent on disturbed membrane transports.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01484142
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  • 2
    Electronic Resource
    Electronic Resource
    Ultrastructure of heart muscle in generalized gangliosidosis GM1 (1971)
    Springer
    European journal of pediatrics 110 (1971), S. 104-114 
    Details
    ISSN: 1432-1076
    Keywords: Inborn Errors of Metabolism ; Generalized Gangliosidosis GM1 ; Ultrastructure of Heart Muscle
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Die generalisierte Gangliosidose GM1 ist durch einen erheblichen bzw. vollständigen Mangel des Enzyms β-Galactosidase gekennzeichnet. Die Spaltung der endständigen Galactosemoleküle vom Gangliosid GM1 ist deshalb blockiert. Es werden die Veränderungen der Herzmuskelzellen, der Capillarendothelien und der vegetativen Nervenfasern beschrieben. Große Vacuolen mit einem feinflockigen Inhalt und Einschlußkörper stellen die wesentlichen Befunde dar.
    Notes: Abstract Generalized gangliosidosis GM1 is characterized by the almost complete deficiency of the enzyme β-galactosidase, therefore the cleavage of the terminal galactose from the gangliosid GM1 is impaired. Typical alterations could be observed in generalized gangliosidosis GM1 in heart muscle cells as well as in capillary endothelium, and nerve fibers. Large vacuoles, containing granular or flocculent material and inclusion bodies, were obvious.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442285
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  • 3
    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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  • 4
    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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  • 5
    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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  • 6
    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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  • 7
    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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  • 8
    Electronic Resource
    Electronic Resource
    Cytoplasmic fusions between liver parenchymal cells and infiltrating cells in chronic aggressive hepatitis (1973)
    Springer
    Virchows Archiv 359 (1973), S. 309-313 
    Details
    ISSN: 1432-2307
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Lymphocytes and plasma cells have been demonstrated in the peripheral zones of lobules in liver biopsies from patients suffering from chronic aggressive hepatitis. In the region of contact between lymphocytes and parenchymal cells, the cell membranes are frequently not demonstrable so that an impression is created of cytoplasmic fusion. However, when the goniometer is used, the cell membranes in this region can still be demonstrated at a certain tilt angle. It can therefore be concluded that the presumed cytoplasmic fusions are frequently the result of superposition effects in projection.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00548602
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  • 9
    Electronic Resource
    Electronic Resource
    73. Erfahrungen bei der orthotopen Xenotransplantation der Hundeleber auf das Schwein (1971)
    Springer
    Langenbeck's archives of surgery 329 (1971), S. 222-223 
    Details
    ISSN: 1435-2451
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wurde bei 11 Schweinen eine orthotope Xenotransplantation der Hundeleber durchgeführt. Die Überlebensdauer war durchschnittlich 4,3 ± 1,5 Std. Die Todesursachen waren generalisierte Blutungen. Um die hyperakute Abstoßungsreaktion zu bremsen, wurden vor der Verpflanzung der Hundeleber bei 5 Fällen Hundeniere und Hundemilz je 20 min und bei 5 anderen Fällen eine zweite Hundeleber am Femoralkreislauf eingeschaltet. Wegen der stark aufgetretenen Kreislaufreaktion (Tachykardie und Hypotonie) war die Überlebensdauer noch kürzer. Aus gleichen Gründen wurden hohe Dosen Prednisolon intraund postoperativ bei 5 Fällen verabreicht. Die Überlebensdauer von 4 Schweinen war 9,0 :L 2,2 Std. Ein Schwein überlebte 5 Tage. Bei diesem Schwein wurde Hundeproteinsynthese beobachtet.
    Notes: Summary Orthotopic xeno-transplantations of dog livers into 11 pigs were carried out. The survival time was on average 4.3 ± 1.5 h. The cause of death was generalized haemorrhage. To inhibit the hyperacute rejection, before trans-planting the dog liver we connected dog kidneys and dog spleens in 5 cases and in another 5 cases a second dog liver to the femoral circulation, 20 min, before the transplant, in each case. In view of the very strong circulatory reaction (tachycardia and hypotension) the survival time was even shorter. For the same reason high doses of prednisolone were administered intra- and postoperatively in 5 cases. The survival time of 4 pigs was 9.0 ± 2.2 h. One pig survived for 5 days, and in this animal we were able to observe dog-protein synthesis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01770490
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  • 10
    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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