Ziele: Evaluierung der Knochenmarkveränderungen beim Morbus Gaucher und MR-morphologischer Befunde, die Patienten mit einem erhöhten Risiko schwerer Knochenkomplikationen wie die avaskuläre Hüftkopfnekrose detektieren können. Methode: Es wurden 63 erwachsene Typ I Gaucher Patienten in der MRT untersucht. Coronare T1- und T2-gewichtete Sequenzen der unteren Extremitäten wurden akquiriert. Die Infiltration des Knochenmarkes durch Gaucher Zell Ablagerungen wurde mit einem semiquantiativen MR-Score (Düsseldorfer Gaucher Score, DGS) analysiert. Die Morphologie der Knochenmarkinfiltration wurde differenziert in Typ A (homogener Befall) versus Typ B (inhomogener Befall). Ein aktiver Knochenmarkprozess mit Knochenödem auf T2-gewichteten Sequenzen und die avaskuläre Hüftkopfnekrose wurden evaluiert. Ergebnis: Die Knochenmarkinfiltration fand sich in den femoralen häufiger als in den tibialen Anteilen der unteren Extremitäten. Ein hoher DGS war signifikant assoziiert mit der Typ B Morphologie und der Hüftkopfnekrose (p<0.0001). Splenektomierte Patienten und Patienten mit Morphologie Typ B zeigten einen signifikant höheren DGS als nicht-splenektomierte Patienten (p<0.05). Eine Hüftkopfnekrose wurde bei 46% der Patienten mit Morphologie Typ B und bei 3% der Patienten mit Morphologie Typ A detektiert (p<0.0001). DGS und Morphologie der Knochenmarkinfiltration waren nicht signifikant mit einem aktiven Knochenmarkprozess assoziiert. Schlussfolgerung: Ein morphologisch inhomogener Knochenmarkbefall und extensive Knochenmarkinfiltrationen waren signifikant mit der Hüftkopfnekrose assoziiert (beide p<0.0001). Diese Parameter haben einen prädiktiven Stellenwert und sollten in das klinische Behandlungskonzept einfliessen um notwendige therapeutische Massnahmen zu ergreifen.

Die nichtokklusive Ischämie (NOI) des Kolons ist eine häufig verkannte Ursache ischämischer Kolitiden.

Background. Nonocclusive disease (NOD) is known to be a common cause of ischemic colitis, which is frequently underestimated. Material and methods. A computer-assisted search of radiological reports at our institute over a period of 18 months, describing ischemic colitis of the ascending colon with an unimpared perfusion of the superior mesenteric artery, was performed. Results. A retrospective analysis of the clinical and radiological data of 14 patients was performed. In ten cases colonic ischemia was confirmed clinically or intraoperatively. Most of our patients needed intravenous catecholamines due to severe hypotension. However, no significant radiographic predictors could be identified. Conclusion. Awareness of NOD seems to be crucial. Especially in cases of acute abdominal pain associated with severe hypotension, renal insufficiency, or pancreatitis, one should include NOD as a differential diagnosis at an early stage.

We report the case of a 58-year-old man with end-stage non-ischemic cardiomyopathy. Baseline transthoracic echocardiography (TTE) and cardiac magnetic resonance (cMRI) revealed a markedly depressed left ventricle systolic function. He underwent autologous CD133+ BM-derived cell transplantation through a minimally invasive approach. During surgery 19 x 10(6) BM-derived stem cells were injected by the transepimyocardial route. Six months after the operation TTE and cMRI showed a clear improvement in left ventricular contractility.

PURPOSE:Development of a feasible and reliable method for determining abdominal fat using breath-hold T1-weighted magnetic resonance imaging.MATERIALS AND METHODS:The high image contrast of T1-weighted gradient echo MR sequences makes it possible to differentiate between abdominal fat and non-fat tissue. To obtain a high signal-to-noise ratio, the measurements are usually performed using phased array surface coils. Inhomogeneity of the coil sensitivity leads to inhomogeneity of the image intensities. Therefore, to examine the volume of abdominal fat, an automatic algorithm for intensity correction must be implemented. The analysis of the image histogram results in a threshold to separate fat from other tissue. Automatic segmentation using this threshold results directly in the fat volumes. The separation of intraabdominal and subcutaneous fat is performed by interactive selection in a last step.RESULTS:The described correction of inhomogeneity allows for the segmentation of the images using a global threshold. The use of semiautomatic interactive volumetry makes the analysis more subjective. The variance of volumetry between observers was 4.6 %. The mean time for image analysis of a T1-weighted investigation lasted less than 6 minutes.CONCLUSION:The described method facilitates reliable determination of abdominal fat within a reasonable period of time. Using breath-hold MR sequences, the time of examination is less than 5 minutes per patient.

Purpose: Development of a feasible and reliable method for determining abdominal fat using breath-hold T1-weighted magnetic resonance imaging. Materials and Methods: The high image contrast of T1-weighted gradient echo MR sequences makes it possible to differentiate between abdominal fat and non-fat tissue. To obtain a high signal-to-noise ratio, the measurements are usually performed using phased array surface coils. Inhomogeneity of the coil sensitivity leads to inhomogeneity of the image intensities. Therefore, to examine the volume of abdominal fat, an automatic algorithm for intensity correction must be implemented. The analysis of the image histogram results in a threshold to separate fat from other tissue. Automatic segmentation using this threshold results directly in the fat volumes. The separation of intraabdominal and subcutaneous fat is performed by interactive selection in a last step. Results: The described correction of inhomogeneity allows for the segmentation of the images using a global threshold. The use of semiautomatic interactive volumetry makes the analysis more subjective. The variance of volumetry between observers was 4.6%. The mean time for image analysis of a T1-weighted investigation lasted less than 6 minutes. Conclusion: The described method facilitates reliable determination of abdominal fat within a reasonable period of time. Using breath-hold MR sequences, the time of examination is less than 5 minutes per patient.

M. Gaucher ist die häufigste lysosomale Speichererkrankung: unbehandelt führt sie zu Anämie, Thrombozytopenie, Hepatosplenomegalie und Destruktion des Knochens. Standardtherapie ist die Enzymersatztherapie mit Imiglucerase, die zu einer Normalisierung des Blutbildes, der Hepatosplenomegalie und der Knochenmanifestationen führt. Umstritten ist die adäquate Dosierung der teuren Enzymersatztherapie, die je nach Land und verfügbaren Ressourcen zwischen 7.5 und 120 U/kg KG/2 Wochen i.v. schwankt. Ziel der Untersuchung war, die hämatologischen, viszeralen und ossären Ansprechraten bei unterschiedlich dosierten Kollektiven zu vergleichen. Hierzu wurden das Düsseldorfer (UKD) Patientenkollektiv (n=69) mit dem Amsterdamer (AMC) Patientenkollektiv verglichen (AMC, n=50). Die Patientengruppen waren im Hinblick auf Alter, Geschlecht, Schweregrad sowie Dauer der Enzymersatztherapie vergleichbar (Tab. 1). Während die Düsseldorfer Patienten mit einer Initialdosis von 40 U/kg KG/2 wks. begannen, lag die initiale Dosierung der niederländischen Patienten bei 7.5 U/kg KG/2 wks.. Verglichen wurden die klinischen Ansprechraten über einen Zeitraum von fast 10 Jahren. Es zeigte sich, dass eine Normalisierung des Hb-Werts bei den UKD-Patienten nach durchschnittlich 8, bei den AMC-Patienten nach 12 Monaten erreicht wurde. Die Thrombozyten hatten sich in beiden Gruppen nach etwa 16 Monaten normalisiert. Ein Rückgang der Leber- und Milzgröße auf 50% des Ausgangswerts wurde nach durchschnittlich 24 (UKD) bzw. 30 Monaten (AMC) erreicht. Die Chitotriosidase, die initial massiv erhöht war und wichtigster Kontrollparameter der Erkrankung ist, fiel bei den UKD-Patienten nach durchschnittlich 16, bei den AMC-Patienten erst nach 48 Monaten auf Werte unter 5000 nmol/ml/h ab (p<0,02). Eine Verringerung des BMB (bone marrow burden score) im MRT des Knochens um 2 Punkte wiesen die UKD-Patienten nach durchschnittlich 48 Monaten, die AMC-Patienten erst nach 79 Monaten auf (p<0,03). 30% der niederländischen Patienten, aber nur 5% der deutschen Patienten zeigten keine messbare Verbesserung des Knochenbefalls. Diese Langzeit-Studie zeigt zum ersten Mal, dass die Dosierung von Imiglucerase nur die Normalisierung der Chitotriosidase und der ossären Manifestationen beeinflusst.

PURPOSETo evaluate the ability of contrast-enhanced MRI with SHU 555 A to provide additional information for characterization of focal liver tumors compared with non-enhanced MRI and multislice spiral CT.MATERIALS AND METHODSIn a prospective manner the images of 45 patients who underwent multislice spiral CT, unenhanced MRI alone and unenhanced and SHU 555 A-enhanced MRI including dynamic imaging at a field strength of 1.0 T were analyzed in a blinded reading. The readers had to determine on a scale from 1 to 5 whether a tumor was benign or malignant. Furthermore, the readers had to give a definitive diagnosis for each lesion. A true cut needle biopsy served as gold standard against which all imaging procedures were compared.RESULTSThe sensitivity for differentiation malignant vs. benign lesion was 77 % with spiral CT, 72 % with unenhanced MRI and 94 % with SHU 555 A-enhanced MRI, respectively (p < 0.05). The specificity for spiral CT was 73 %, for unenhanced MRI 83 % and for contrast-enhanced MRI 83 %, respectively (n. s.). Compared with the histopathologic results, the correct diagnosis was made with spiral CT in 25/45 (56 %), unenhanced MRI in 16/45 (36 %) and contrast-enhanced MRI in 32/45 (71 %) of the patients (p < 0.05). For the subgroup of patients with liver cirrhosis, the correct diagnosis was established with spiral CT in 16/23 (70 %), unenhanced MRI in 9/23 (39 %) and contrast-enhanced MRI in 19/23 (83 %) of the patients (p < 0.05).CONCLUSIONContrast-enhanced MRI with SHU 555 A has the ability to improve the differential diagnosis of focal liver tumors compared with unenhanced MRI and multislice spiral CT.

To improve tissue regeneration of ischemic myocardium, autologous bone marrow-derived stem cells have been injected intramyocardially in 10 patients with end-stage ischemic heart disease. Approx. 60 – 380 ml of bone marrow were harvested from the posterior iliac crest and processed in the operating room under GMP conditions using the automated cell selection device CliniMACS. By using the Duesseldorf protocol, the intraoperative isolation of CD133+-stem cells (1.9−10.0 x 106 cells; purity up to 97%) was achieved in less than 3 hours. Following isolation, autologous CD133+-stem cells were injected in a predefined pattern into the myocardium. Cardiac function was assessed by cardiac MRI and echocardiography three, six, and nine months postoperatively. A significant improvement of cardiac function could be documented in 7 out of 10 patients: ejection fraction (EF) before treatment: 10–22% - after 3 months: 18–30% - after 6 months: 19–30%, after 9 months: 21–31%; left ventricular enddiastolic volume (LVEDV) before treatment: 210 ± 123 ml, after 3 months: 169 ± 80 ml, after 6 months: 162 ± 82 ml, after 9 months: 175 ± 70 ml; left ventricular enddiastolic diameter (LVEDD) before treatment: 79.2 ± 7 mm, after 3 months: 57.4 ± 3 mm, after 6 months: 59.4 ± 4 mm, after 9 months: 56.2 ± 5 mm, respectively. In two patients cardiac function improved only temporarily over the first three months, 1 patient died one month after surgery due to a non-cardiac cause. In conclusion, the sole intramyocardial injection of autologous CD133+-cells proved to be safe and led to a significant gain in heart function in 7 of 10 patients, thereby avoiding or postponing (“bridging”) alternative therapies such as heart transplantaton. The benefial effects of intramyocardially injected bone marrow-derived stem cells might be explained by direcct cellular effects including neovascularization and indirect effects including the formation of growth factors promoting tissue repair.

We report 2 cases in which patients with coronary heart disease not amenable for conventional revascularization underwent transmyocardial laser revascularization (TMLR) and implantation of AC133+ bone-marrow stem cells. The reason for using TMLR in combination with stem cell application is to take advantage of the synergistic angiogenic effect. The local inflammatory reaction induced by TMLR should serve as an informational platform for stem cells and may trigger their angiogenic differentiation. Functional analysis of myocardial performance after treatment in these 2 cases revealed dramatic improvement of the wall motion at the site of the TMLR and stem cell application. Because TMLR does not enhance myocardial contractility and there was no angiographic evidence of major collaterals to the ischemic region in either patient, we assume that the synergistic effect of stem cells and TMLR-induced angiogenesis occurred; however, our assumption is of a speculative nature. We think that TMLR in combination with stem cell transplantation might become a novel revascularization therapy for ischemic myocardium.

To improve tissue regeneration of ischemic myocardium, autologous bone marrow-derived stem cells have been injected intramyocardially in five patients undergoing coronary artery bypass grafting and transmyocardial laser revascularization. We have established an innovative method for the intraoperative isolation of CD133+-stem cells in less than 3 hours. After induction of general anesthesia, approx. 60 – 240 ml of bone marrow were harvested from the posterior iliac crest and processed in the operating room under GMP conditions using the automated cell selection device CliniMACS. Following standard coronary artery bypass grafting, laser channels were shot in predefined areas within the hibernating myocardium. Subsequently, autologous CD133+-stem cells (1.9–9.7 x 106 cells; purity up to 97%) were injected in a predefined pattern around the laser channels (0.5–1.0x106 cells/channel). Three months postoperatively, significant improvement of cardiac function as assessed by transthoracal echocardiography (ejection fraction before treatment: 25–30% - after treatment: 38–46%; left ventricular enddiastolic volume before treatment: 58 – 64 mm - after treatment: 40–44 mm; endsystolic wall thickness before treatment: 5–7 mm - after treatment 9–13 mm) and improvement of cardiac wall motion (electrocardiographically-triggered magnetic resonance imaging) were documented. This significant gain in heart function might be partly explained through the synergistic angiogenic effect of transmyocardial laser revascularization leading to a localized inflammatory response and the cellular effects of intramyocardially injected pluripotent bone marrow-derived stem cells promoting tissue repair. Through the intraoperative isolation of CD133+-cells, this effective treatment of ischemic myocardium can be applied to patients scheduled both for elective and for emergency revascularisation procedures.

Ziel: Messung von MRT-Perfusionsparametern der LWS bei Patienten mit myelodysplastischen Syndromen (MDS) zur Beurteilung der Vaskularisation und des antiangiogenetischen Effektes einer Thalidomidtherapie. Material und Methode: Bei 20 gesunden Normalpersonen und 28 MDS-Patienten wurde eine kontrastmittelunterstützte dynamische MRT (d-MRT) der LWS durchgeführt. Bei 24 der 28 MDS-Patienten wurde nach der initialen d-MRT-Untersuchung eine anti-angiogenetische Therapie mit Thalidomid begonnen. Durchschnittlich 4,2 Monate nach Therapiebeginn erfolgte bei 9 dieser Patienten eine d-MRT-Verlaufsuntersuchung. Anhand der Signalintensitäts-Zeit-Kurven der d-MRT wurden die Amplitude und Austauschratenkonstante berechnet und ein statistischer Vergleich der Werte zwischen Probanden und Patienten sowie eine Korrelation der klinischen Verlaufsparameter der MDS-Patienten mit den d-MRT-Ergebnissen durchgeführt. Ergebnisse: Bei den 28 MDS-Patienten wurden im Vergleich zu den Normalpersonen durchschnittlich höhere Amplituden (Normalpersonen: 14,4 ± 5,2, MDS: 24,8 ± 8,1) und Austauschratenkonstanten (Normalpersonen: 0,124 ± 0,042, MDS: 0,136 ± 0,036) gemessen. Bei 7 der 9 MDS-Patienten konnte unter Thalidomidtherapie eine Reduktion der Amplitude und Austauschratenkonstante in den d-MRT-Verlaufsuntersuchungen nachgewiesen werden. Klinisch zeigten diese Patienten ein Therapieansprechen mit kompletter oder partieller Krankheitsremission. Schlussfolgerungen: Bei MDS-Patienten lassen sich signifkant höhere d-MRT-Parameter als bei Normalpersonen nachweisen. Unter anti-angiogenetischer Therapie kommt es im Falle eines Therapieansprechens zum Abfall dieser Werte. Die d-MRT scheint somit zur Beurteilung anti-angiogenetischer Therapieeffekte geeignet.

Bei einer asymptomatischen Patientin ist eine zervikale Raumforderung aufgetreten, die computertomographisch und kernspintomographisch benigne Kriterien aufwies. Es wird exemplarisch die Differenzialdiagnose eines zystischen zervikalen Tumors dargestellt.

A 33-year-old woman with type 1 Gaucher's disease developed painful swelling of her right tibia. Initial diagnostics suggested a typical bone crisis. However, clinical course and subsequent imaging pointed to malignant disease, which was specified as high-grade lymphoma. Chemotherapy was applied together with enzyme replacement and resulted in complete remission of the lymphoma. We conclude that osseous lesions, although suggestive of common manifestations of Gaucher's disease, should be discriminated very carefully from neoplastic infiltration to maintain curative treatment options.

PURPOSE:To assess radiation exposure of patients undergoing Multi-Row Detector CT (Multi-Slice CT, MSCT) of the heart.MATERIAL AND METHODS:Four different cardiac MSCT protocols with changing slice collimation (4 x 1, and 4 x 2.5 mm), and pitch-factor (1.5, 1.8, and 4) were examined. An anthropomorphic Alderson-Rando phantom was equipped with LiF-thermoluminescent dosimeters at several organ sites, and effective doses were calculated using ICRP-weighting factors. These data were compared to data from standard MSCT of chest and abdomen.RESULTS:Effective dose in different protocols for cardiac MSCT varies from 2.8 to 10.3 mSv (male), and from 3.6 to 12.7 mSv (female). In protocols with thin collimation and low pitch or a combination of several heart examinations, radiation exposure may be comparable to the effective dose of standard MSCT of the chest (male: 11.9 mSv, female: 12.9 mSv) or the abdomen (male: 16.1 mSv, female: 15.7 mSv). Highest organ doses were found for the female breast (up to 46.6 mGy), and the lungs (up to 36.4 mGy) with surface doses as high as 54.3 mGy.CONCLUSIONS:Cardiac MSCT adds significantly to the radiation exposure of patients and can reach the effective dose applied by standard MSCT of chest or abdomen.

Reduction of the defibrillation energy requirement offers the opportunity to decrease implantable cardioverter defibrillator (ICD) size and to increase device longevity. Therefore, the purpose of this prospective study was to obtain confirmed defibrillation thresholds (DFTs) of < or = 15 J in each patient with an endocardial dual-coil lead system incorporating an active pectoral pulse generator (TRIAD lead system: RV- --> SVC+ + CAN+). According to our previous clinical and experimental studies, we tried to lower DFTs that were > 15 J by repositioning the distal coil of the endocardial lead system in the right ventricle. A total of 190 consecutive patients requiring ICDs for ventricular fibrillation and/or recurrent ventricular tachycardia were investigated at the time of ICD implantation (42 women, 148 men; mean age 61.9 +/- 12.0 years; mean left ventricular ejection fraction 42.7 +/- 16.6%). Coronary artery disease was present in 139 patients; nonischemic dilated cardiomyopathy in 34 patients; and other etiologies in 17 patients; 47 patients had undergone previous cardiac surgery. Regardless of optimal pacing and sensing parameters, for patients having DFTs > 15, we repositioned the distal coil of the endocardial lead system toward the intraventricular septum to include this part of both ventricles within the electrical defibrillating field. In 177 of 190 patients, induced ventricular fibrillation was successfully terminated with < or = 15 J (group I) using the initial lead position. Repositioning of the endocardial lead was necessary in 13 patients whose DFT(plus) (DFT(plus) = second additional success at lowest energy level) were > 15 J (group II). In all patients, repositioning was successful within a 15 J energy level (100% success). The mean DFT(plus) was 7.3 +/- 3.5 J (group I) and 11.0 +/- 4.5 J (group II; p<0.005). The mean DFT(plus) of all patients enrolled in the study was 7.6 +/- 3.7 J (range: 2 to 15 J). In 87% of all patients, DFT(plus) of < or = 10 J was achieved. Repositioning of the endocardial lead in the right ventricle is a simple and effective method to reduce intraoperative high DFTs. As a result of this procedure, ICDs with a 20 J output should be sufficient for the vast majority (87%) of our patients. Furthermore, we were able to avoid additional subcutaneous or epicardial electrodes in all patients.

ZusammenfassungDer vorliegende Fall verdeutlicht, daß auch bei nicht hochgradig immunsupprimierten Patienten eine invasive Aspergillusinfektion differentialdiagnostisch in Betracht gezogen werden muß. Ein persistierender Fokus kann auch ohne offensichtlichen Auslöser reaktiviert werden und zu einer schwerwiegenden Erkrankung führen. Eine Aspergillusendokarditis kann sich atypisch präsentieren und muß als seltene Differentialdiagnose bei Reizleitungsstörungen oder dem Verdacht auf eine ischämische Herzerkrankung in Betracht gezogen werden.