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  • Electronic Resource  (6)
  • Glucosephosphate isomerase  (4)
  • Cerebral aneurysm  (2)
  • 1
    Electronic Resource
    Electronic Resource
    Zur Frage gewebespezifischer Isoenzyme der Glucosephosphatisomerase des Menschen (1974)
    Springer
    Journal of molecular medicine 52 (1974), S. 149-151 
    Details
    ISSN: 1432-1440
    Keywords: Glucosephosphate isomerase ; Isoenzymes ; Tissue distribution ; Glucosephosphat ; Isomerase ; Isoenzyme-Gewebsverteilung
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei angeborenen Enzymdefekten ist das Vorhandensein von organspezifischen Isoenzymen des betreffenden Enzyms wichtig für die Ausbreitung der Funktionsstörung im Organismus. Die Ergebnisse der Elektrophorese, der Bestimmung der Substrataffinität und der Thermoinaktivierung der GPI in menschlichen Organextrakten lieferten keinen Anhaltspunkt für ein organspezifisches Isoenzymsystem. Die Verteilung der GPI-Aktivität in verschiedenen postmortal und intravital entnommenen Gewebsproben ist relativ gleichförmig mit Ausnahme der Erythrocyten.
    Notes: Summary The occurrence of tissue-specific isoenzymes is important for the distribution of the disease in enzyme deficiencies. In the case of GPI our results of starch gel electrophoresis, substrate affinity measurements and thermoinactivation gave no evidence for the occurrence of tissue-specific isoenzymes of GPI in man. GPI activity was in the same order of magnitude in various postmortal and intravital samples of human tissues, except in erythrocytes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01620756
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Early management in poor grade aneurysm patients (1994)
    Springer
    Acta neurochirurgica 126 (1994), S. 33-37 
    Details
    ISSN: 0942-0940
    Keywords: Cerebral aneurysm ; poor grade aneurysm ; early management ; outcome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Aneurysm surgery began in Lübeck only in 1986 when the department was completely reorganized. Early operation in the good grade patients (I–III, according to Hunt and Hess) was performed. In every case we also discussed the feasibility of operating on the poor grade patients (Hunt and Hess IV and V). During a five-year period (1986–1991) a total of 277 SAH patients were admitted to the department. 109 (39%) patients arrived in a poor grade (Hunt and Hess IV or V), 12 of these patients died within hours of admission. 25 patients, who presented with a large intracerebral and/or subdural haematoma, were urgently operated upon by haematoma evacuation and aneurysm clipping. An external ventricular drainage was performed on 72 patients. Of the ventriculostomy group 33 patients improved and 27 were operated upon. In 17 of the 39 patients without improvement after CSF-drainage we decided to operate. Overall 69 patients were surgically treated (craniotomy, aneurysm clipping) and 40 were not. The mortality rate in the surgical cases was 16 (23%) compared with 30 (75%) without operation. It is concluded that poor grade aneurysm patients can achieve a better outcome with active treatment based on immediate ventriculostomy and optimal haemodynamic parameters after haematoma evacuation and early occlusion of the aneurysm.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01476491
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Acute subdural haematoma from ruptured intracranial aneurysms (1995)
    Springer
    Acta neurochirurgica 136 (1995), S. 163-167 
    Details
    ISSN: 0942-0940
    Keywords: Cerebral aneurysm ; acute subdural haematoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Acute subdural haematoma (SDH) secondary to a raptured intracranial aneurysm is a rare event. Out of a total of 292 patients with a verified aneurysm (period 1986–1992) in five cases SDH was the diagnosis on CT-evaluation. One patient was in such a bad condition that no treatment was indicated. The remaining four patients were operated on: craniotomy and haematoma evacuation in two cases, craniotomy for haematoma evacuation and aneurysm-clipping in the other two cases. Two patients died and two achieved a good outcome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01410620
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  • 4
    Electronic Resource
    Electronic Resource
    Inherited glucosephosphate isomerase deficiency (1979)
    Springer
    Annals of hematology 39 (1979), S. 405-417 
    Details
    ISSN: 1432-0584
    Keywords: Glucosephosphat-Isomerase ; Enzymdefekt ; HÄmolytische AnÄmie ; Erythrozytenstoffwechsel ; Glucosephosphate isomerase ; Enzyme deficiency ; Hemolytic anemia ; Red cell metabolism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Since the first report of GPI deficiency in 1967 many patients from all over the world have been described. The patients suffer from a typical nonspherocytic hemolytic anemia with hemolytic crises during acute infections. The disease is inherited as an autosomal recessive, half of the patients are homozygotic, the others are double heterozygotes. The biochemical properties of the deficient enzymes vary widely. Thus, many well characterized enzymes have been designated as different variants. The modification of physicochemical properties surpasses kinetic aberrations. All defective variants are more or less unstable. The activity diminishes progressively, leading to a rise in G6P concentration and in red cells after aging in vitro to a dramatic impairment of glycolysis and concomittant hemolysis. The cause of the metabolic block is the diminished GPI activity itself and not an inhibition of hexokinase by the high G6P.
    Notes: Zusammenfassung Seit der Erstbeschreibung des GPI-Mangels 1967 wurden zahlreiche Patienten aus allen Teilen der Welt mitgeteilt. Die Patienten leiden an einer typischen nicht-sphÄrozytÄren hÄmolytischen AnÄmie mit krisenhafter Verschlechterung wÄhrend akuter Infektionen. Die Erkrankung wird autosomal rezessiv vererbt, die HÄlfte der Patienten ist homozygot, die anderen doppelt-heterozygot. Die biochemischen Eigenschaften der defekten Enzyme variieren stark, so da\ verschiedene Varianten beschrieben wurden. Die physiko-chemischen Eigenschaften sind hÄufiger verÄndert als die kinetischen. Alle Defekt-Varianten sind mehr oder weniger instabil. Die wÄhrend der Zellalterung fortschreitende Verminderung der GPI-AktivitÄt führt zu einem Anstieg des G6P, und bei in vitro gealterten Erythrozyten zu einem dramatischen Abfall der Glycolyserate und gleichzeitiger HÄmolyse der Zellen. Die Ursache der GlycolyseeinschrÄnkung scheint die Verminderung der GPI-AktivitÄt selbst und nicht etwa eine Hemmung der Hexokinase durch das erhöhte G6P zu sein.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01008661
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  • 5
    Electronic Resource
    Electronic Resource
    Glucosephosphate isomerase deficiency type Liège: A new variant with congenital nonspherocytic hemolytic anemia (1977)
    Springer
    Annals of hematology 35 (1977), S. 187-193 
    Details
    ISSN: 1432-0584
    Keywords: Hemolytic anemia ; Enzyme deficiency ; Glucosephosphate isomerase ; Hämolytische Anämie ; Enzymdefekt ; Glukosephosphat-Isomerase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei einem 3jährigen Mädchen marokkanischer Abstammung, das seit Geburt an einer hämolytischen Anämie leidet, wurde ein GPI-Defekt der Erythroxyten festgestellt. Das defekte Enzym ist sehr thermolabil und zeigt in der Stärkegelelektrophorese eine einzelne Bande mit einer Wanderungsgeschwindigkeit von 96 % der normalen Hauptbande. Nach einer 16000fachen Anreicherung mit einer Ausbeute von 35% war das gereinigte Enzym außergewöhnlich instabil sogar bei niedrigen Temperaturen. Die kinetischen Konstanten der Vor- und Rückreaktion sowie die Inhibitorkonstanten für 2,3-DPG und 6-PG wichen nicht signifikant von den Werten des Wildtyps ab. Die biochemischen Eigenschaften des Enzyms sprechen für eine neue Defektvariante, die nach dem Geburtsort der Patientin Typ Liège genannt wurde.
    Notes: Summary GPI deficiency was detected in a three year old girl of Morrocan origin suffering, since birth, from hemolytic anemia. The defective GPI is very thermolabile and migrates on starch gel electrophoresis as a single band with a mobility of 96 % of the normal main band. The purification of the patient's GPI resulted in a 16000-fold enriched preparation, free of any other enzyme activity. The yield was 35 %. The purified enzyme was very unstable even at low temperature. The kinetic constants of the forward and backward reaction as well as the inhibitory constants of 2,3-DPG and 6-PG do not significantly differ from normal values. The bio-chemical properties of the patient's GPI indicate a new variant designated type Liège.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00999459
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  • 6
    Electronic Resource
    Electronic Resource
    Augsburg-type glucosephosphate isomerase deficiency (1980)
    Springer
    Annals of hematology 40 (1980), S. 107-115 
    Details
    ISSN: 1432-0584
    Keywords: Hemolytic anemia ; Enzyme deficiency ; Glucosephosphate isomerase ; Hämolytische Anämie ; Enzymdefekt ; Glucosephosphat-Isomerase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei einem einjährigen deutschen Jungen wurde als Ursache für eine chronische hämolytische Anämie mit rezidivierenden hämolytischen Krisen ein GPI-Defekt der Erythrozyten entdeckt. Bei Konsanguinität der Eltern handelt es sich um einen homozygoten Defektträger. Die Untersuchung der biochemischen Eigenschaften des Defektenzymes ergab ein verändertes elektrophoretisches Muster, eine deutliche Thermolabilität, eine erhöhte Affinität für G6P und für den kompetitiven Inhibitor 6-PG und leicht zum alkalischen verschobene pH-Optima für beide Substrate. Von der Elektrophorese nach Einfrieren und Auftauen des Hämolysates kann geschlossen werden, daß die genetische Modifikation der GPI-Untereinheit hauptsächlich den Mechanismus betrifft, der beim normalen Enzym die Hauptbande in die Sekundärbanden überführt. Die einmaligen Eigenschaften des Defektenzyms sprechen für eine neue Variante, für die nach dem Geburtsort des Patienten der Name GPI Augsburg vorgeschlagen wird.
    Notes: Summary In a 1-year-old German boy a GPI deficiency was found to be the cause of a chronic nonspherocytic hemolytic anemia with recurrent hemolytic crises. Because of consanguinity of the parents, the patient is true homozygote. The investigation of the biochemical properties of the deficient enzyme revealed an altered electrophoretic behavior, pronounced thermolability, an increased affinity for G6P, an increased affinity for the competitive inhibitor 6-PG, and slightly changed pH optima for both substrates. Electrophoresis after freezing and thawing the hemolysate indicates that the genetic modification of the subunit involves the mechanism of transforming the main band into the secondary bands. The properties of the new deficient GPI indicate a new variant designated GPI Augsburg.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01013693
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