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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Abdominal imaging 25 (2000), S. 286-289 
    ISSN: 1432-0509
    Keywords: Key words: Gaucher disease—Spleen in Gaucher disease—Splenic nodules—Computed tomography—Magnetic resonance imaging.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We present a 26-year-old male patient with Gaucher disease who presented with epigastric pain and a palpable mass in the left abdomen. Ultrasound, abdominal computed tomography, and magnetic resonance imaging showed massive splenomegaly with multiple splenic nodules up to 7 cm in diameter. Splenic nodules should be included in the differential diagnosis of splenic masses. Follow-up is necessary because of the increased incidence of hematologic malignancies in Gaucher disease.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1084
    Keywords: Key words: MR imaging – Cardiac MRI – Volume measurement
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. The aim of this study was to determine the accuracy in quantifying right and left ventricular volumes using a 1.0-T system and commercially available, standard equipment. For exact comparison of MRI measurements and real volumes we used an animal heart model ex vivo. Eight pig hearts were explanted and prepared by removal of the atria. Aorta and pulmonary truncus were cannulated. Definable volumes were injected into the ventricles. Magnetic resonance imaging was performed at 1.0 T (Gyroscan T10 NT, Philips, Eindhoven, The Netherlands); sequence: fast field echo–echo planar (multishot EPI); body coil; MR software: Cardiac Application Package (Philips). Statistical analysis correlated the real volumes and MR measurements separately for both ventricles and two investigators (SAS, ANOVA). For both ventricles and both investigators the correlation between real volumes and MR measurements was greater than 0.99. There was no significant systematic false estimation for both ventricles. Magnetic resonance imaging at 1.0 T using standard hardware and software equipment enables the quantification of right and left ventricular volumes with high approximation to the real volumes in vitro. There is a clear restriction in translating these data into a clinical application because under experimental conditions no motion-induced artifacts existed.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1084
    Keywords: Key words: Gaucher's disease type I – Skeletal disease – Bone marrow imaging – MRI
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Gaucher's disease type I is the most prevalent lysosomal storage disorder caused by an autosomal-recessive inherited deficiency of glucocerebrosidase activity with secondary accumulation of glucocerebrosides within the lysosomes of macrophages. The storage disorder produces a multisystem disease characterized by progressive visceral enlargement and gradual replacement of bone marrow with lipid-laden macrophages. Skeletal disease is a major source of disability in Gaucher's disease. Extraosseous extension of Gaucher cells is an extremely rare manifestation of skeletal Gaucher's disease. This is a report on the MRI and histopathological findings of an extraosseous Gaucher-cell extension into the midface in a patient with Gaucher's disease.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-2102
    Keywords: Schlüsselwörter ; Herz ; Herz-MR ; Volumen ; Cine MR ; Key words ; Heart ; Cardiac MRI ; Volume ; Cine MR
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Purpose: To evaluate method- and user effects as well as the required time in the determination of left- and right ventricular ejection fractions in comparing manual and semiautomatic border detection of the endocardium. Methods: In 22 patients with varying cardiac diseases endocardial borders were detected either manually or semiautomatically (seeded ROI) and subsequently the right and left ventricular volume ejection fractions were computer-calculated. Two investigators defined the endocardial borders for both ventricles using both methods. For statistical analysis two-factorial analyses of variance were performed for the four variables: VEF right ventricle, VEF left ventricle, required time right ventricle, required time left ventricle (SAS, Anova). Results: There was no user or method effect detectable for both ventricles. The required time for both ventricles also did not reveal a user effect, but there was a dramatic method effect concerning a reduction of the required time for analysis for both ventricles 〉50%. Conclusions: The semiautomatic mode for detecting the endocardial borders in determining right and left ventricular ejection fractions did not demonstrate results differing from the results of the manual method, but is significantly superior concerning the time required for analysing ejection fractions of both ventricles with a reduction of over 50%. This might significantly reduce the expenditure for personnel.
    Notes: Zusammenfassung Fragestellung: Die manuelle und semiautomatische Endokarddetektion zur Bestimmung links- und rechtsventrikulärer Volumenejektionsfraktionen (VEF) werden verglichen. Ziel dieser Arbeit ist die Evaluierung des Methoden- und Anwendereffekts sowie der Bearbeitungszeit beider Verfahren. Methode: An 22 Patienten mit unterschiedlichen kardialen Erkrankungen wurden die Endokardgrenzen manuell und mit einem semiautomatischen Verfahren (Seeded-ROI) ermittelt und anschließend die rechts- und linksventrikulären Volumenejektionsfraktionen automatisch berechnet. In allen Fällen wurden von 2 Untersuchern die Endokardgrenzen des rechten und linken Ventrikels manuell und semiautomatisch bestimmt. Zur statistischen Analyse wurden 2-faktorielle Varianzanalysen (Methoden- und Anwendereffekt einschließlich Wechselwirkungen) für die 4 Variablen durchgeführt: VEF rechtsventrikulär, VEF linksventrikulär, Zeitaufwand rechtsventrikulär, Zeitaufwand linksventrikulär (SAS, Anova). Ergebnisse: Bezüglich der Bestimmungen der rechts- und linksventrikulären VEF war weder ein Methoden- (p=0,9613) noch ein Anwendereffekt (p=0,3095) nachweisbar. Bezogen auf die benötigte Bearbeitungszeit fand sich ebenfalls kein Anwendereffekt, aber ein massiver Methodeneffekt (p〈0,0001) im Sinn einer Reduzierung der Bearbeitungszeit um 〉50% sowohl für den rechten als auch für den linken Ventrikel. Schlussfolgerung: Nach unseren Erfahrungen liefert das semiautomatische Seeding-Verfahren zur Festlegung der Endokardgrenzen bei der Bestimmung der links- und rechtsventrikulären Ejektionsfraktionen keine von der manuellen Methode abweichenden Ergebnisse, ist aber bezüglich der Bearbeitungszeit deutlich überlegen. Die über 50%ige Zeitersparnis reduziert den Personalaufwand.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-2161
    Keywords: Key words Gaucher disease ; Bone disease ; Extraosseous Gaucher disease ; Bone marrow imaging ; MRI
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Objective. To investigate the frequency and morphology of extraosseous extension in patients with Gaucher disease type I. Design and patients. MRI examinations of the lower extremities were analyzed in 70 patients with Gaucher disease type I. Additionally, the thoracic spine and the midface were investigated on MRI in two patients. Results. Four cases are presented in which patients with Gaucher disease type I and severe skeletal involvement developed destruction or protrusion of the cortex with extraosseous extension into soft tissues. In one patient, Gaucher cell deposits destroyed the cortex of the mandible and extended into the masseter muscle. In the second patient, multiple paravertebral masses with localized destruction of the cortex were apparent in the thoracic spine. In the third and fourth patient, cortical destruction with extraosseous tissue extending into soft tissues was seen in the lower limbs. Conclusions. Extraosseous extension is a rare manifestation of Gaucher bone disease. While an increased risk of cancer, especially hematopoietic in origin, is known in patients with Gaucher disease, these extraosseous benign manifestations that may mimic malignant processes should be considered in the differential diagnosis of extraosseous extension into soft tissues. A narrow neck of tissue was apparent in all cases connecting bone and extraosseous extensions.
    Type of Medium: Electronic Resource
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