[MRI bone marrow findings in 63 patients with type I Gaucher disease]. / MRT der Knochenmarkverändungen bei 63 Patienten mit Morbus Gaucher Typ I.

PURPOSE: To determine whether MR bone marrow findings in Gaucher patients may help to identify patients at high risk of developing severe Gaucher bone complications exemplified by avascular necrosis (AVN) of the femoral head. MATERIALS AND METHODS: MR images were obtained in 63 Type I Gaucher patients through a standard protocol using coronal T 1 and T 2-weighted sequences of the lower extremities. The location and extent of infiltrated marrow was established using a semi-quantitative MRI scoring method (Düsseldorf Gaucher score, DGS) and the morphological pattern of bone marrow involvement determined (whether homogeneous type A or non-homogeneous type B). The active marrow process with bone edema and AVN of the femoral head were also analyzed. RESULTS: Bone marrow involvement was observed in femoral sites more than in tibial sites. A high DGS was significantly correlated with type B morphology and femoral AVN (both p < 0.0001). Splenectomized patients showed a significantly higher Düsseldorf Gaucher score and type B morphology than non-splenectomized patients (both p < 0.05). AVN was seen in 46 % of patients with type B morphology versus 3 % in type A morphology (p < 0.0001). DGS and morphology of bone marrow involvement were not significantly correlated with active marrow processes. CONCLUSION: Type B marrow morphology and extensive marrow packing were significantly associated with AVN of the femoral head (both p < 0.0001). These patterns are considered predictive and may be employed in a disease management context to alert physicians to the need for urgent therapeutic measures.

Necrotizing fasciitis: microbiological characteristics and predictors of postoperative outcome.

OBJECTIVE: Necrotizing fasciitis is a life threatening soft-tissue infection with a high morbidity and mortality. Prompt treatment based on extensive surgical debridement and antibiotic therapies are the therapeutic principles. METHODS: The medical records of patients with necrotizing fasciitis (n = 26) from 1996 to 2005 were retrospectively analyzed. RESULTS: The localization of necrotizing fasciitis was most commonly the trunk (42.3 %). Type I polymicrobial infection was the dominating infection. The involvement of anaerobic bacteria was associated with an increase in the number of surgical revisions (p = 0.005). Length of postoperative intensive care unit stay, duration of postoperative ventilation and mortality were significantly increased in the ASA IV-V group. Computed tomography displayed only a limited significance as diagnostic tool for initial diagnosis. CONCLUSIONS: In severe cases the combination of necrotic skin and soft tissue gas facilitates the correct diagnosis, which should than be followed by immediate - and most often - repeated debridement. If anaerobes are isolated an early and aggressive second look is necessary.

Different dose-dependent correction of MIP-1beta and chitotriosidase during initial enzyme replacement therapy.

In tissue lesions of type I Gaucher patients, characteristic lipid-laden macrophages, 'Gaucher cells', are surrounded by inflammatory phagocytes. Gaucher cells secrete the elevated plasma chitotriosidase. The elevated plasma MIP-1beta in Gaucher patients stems from the phagocytes surrounding the Gaucher cells. Plasma chitotriosidase and MIP-1beta decrease upon successful enzyme replacement therapy (ERT) with mannose-terminated recombinant glucocerebrosidase (alglucerase). Previous histochemical analysis of Gaucher spleens revealed that Gaucher cells express little mannose receptor, in contrast to surrounding phagocytes. We therefore investigated the corrective effects of ERT on plasma MIP-1beta and chitotriosidase in more detail. We also compared effects of one year of treatment with a relatively low dose and a relatively high dose of ERT. A more rapid correction in plasma MIP-1beta, compared to chitotriosidase, was observed in most patients on low-dose ERT. Correction of plasma MIP-1beta and chitotriosidase levels was more pronounced in the higher-dosed patient group. Upon prolonged treatment, differences in the effects of enzyme dose were no longer significant. Normalization of plasma MIP-1beta and chitotriosidase levels was attained in the majority of patients. In conclusion, ERT with mannose-terminated gluocerebrosidase results in prominent corrections of plasma chitotriosidase, a marker of Gaucher cells, and in particular of plasma MIP-1beta, a marker of inflammatory phagocytes. The sharper response in plasma MIP-1beta to ERT is in line with the observation that especially phagocytes surrounding Gaucher cells express mannose-receptors.

"Silent" bone stress injuries in the feet of diabetic patients with polyneuropathy: a report on 12 cases.

BACKGROUND: Bone stress injuries are rarely being diagnosed in patients with sensory neuropathy, most likely because they may be silent in terms of pain. Load-related pain is considered a key feature of any bone stress injury, a symptom, which may be partially or completely absent in subjects with sensory neuropathy (loss of protective sensation). We evaluated the clinical course of bone stress injuries in insensitive feet in diabetic patients with polyneuropathy. METHODS: We investigated 12 consecutive diabetic patients with bone stress injuries of the foot (bone marrow edema, bone bruise and microtrabecular fractures, on magnetic resonance imaging MRI), which were undetectable on plain X-ray. All patients suffered from diabetic polyneuropathy, none of them had an active foot ulcer. RESULTS: The patients presented with a swollen foot, which was only mildly painful and did not prevent them from walking. Complaints were related to the swelling, which increased during load-bearing. In seven cases, a traumatic event preceding the onset of symptoms could be ascertained. MRI disclosed stress injuries in 2.5 (1-8) [median (range)] bones per foot. In 11 patients, treatment was started immediately by off-loading with total contact cast for 17 (8-52) weeks, followed by gradual increase in weight bearing. One patient unfortunately received off-loading treatment only after deforming fractures had developed. All bone injuries healed uneventuelly in eight patients, and with residual mild osteoarthrosis in three patients without gross deformities. However, the untreated patient developed severe Charcot foot deformity. CONCLUSIONS: In diabetic patients with polyneuropathy, symptoms of bone stress injuries of the foot are atypical, in that there is load-related swelling rather than load-related pain. Immediate diagnosis, and treatment with off-loading, leads to a restitutio ad integrum like in non-neuropathic patients. Delayed cessation of overuse, however, may cause irreversible joint and bone damage (Charcot foot).

[Nonocclusive ischemia of the right colon]. / Nichtokklusive Ischämie (NOI) des rechtsseitigen Kolons.

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 unimpaired 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.

Intramyocardial implantation of CD133+ stem cells improved cardiac function without bypass surgery.

INTRODUCTION: Cell transplantation for myocardial regeneration has been shown to have beneficial effects on cardiac function after myocardial infarction. Most clinical studies of intramyocardial cell transplantation were performed in combination with coronary artery bypass grafting (CABG). The contribution of implanted stem cells could yet not be clearly distinguished from the effect of the CABG surgery. Our current phase 1 clinical study has focused on the safety and feasibility of CD133+-enriched stem cell transplantation without CABG and its potential beneficial effect on cardiac function. METHOD AND RESULTS: Ten patients with end-stage chronic ischemic cardiomyopathy (ejection fraction <22%) were enrolled in the study. Bone marrow (up to 380 mL) was harvested from the iliac crest. CD133+ cells were purified from bone marrow cells using the CliniMACS device with purities up to 99%. Autologous bone marrow CD133+ cells (1.5-9.7 X 106 cells) were injected into predefined regions. Cardiac functions prior to and 3, 6, and 9 months after cell transplantation were assessed by cardiac magnetic resonance imaging. Stem cell transplantation typically improved the heart function stage from New York Heart Association/Canadian Cardiovascular Society class III-IV to I-II. The mean preoperative and postoperative ventricular ejection fractions were 15.8 +/- 5% and 24.8 +/- 5%, respectively. CONCLUSION: CD133+ injection into ischemic myocardium was feasible and safe. Stem cell transplantation alone improved cardiac function in all patients. This technique might hold promise as an alternative to medical management in patients with severe ischemic heart failure who are ineligible for conventional revascularization.

Evaluation of the diabetic charcot foot by MR imaging or plain radiography--an observational study.

Magnetic resonance (MR) images and concomitant plain radiographs of 26 Charcot feet of different stages (stage 0 to III) were reviewed in retrospect for discordant findings. Bone and joint abnormalities were evaluated qualitatively, and quantitatively guided by the AO integral classification of injuries of the foot. MR images revealed traumatic bone and joint injuries (bone oedema, occult fractures, and joint effusion) already in stage 0, when X-ray still showed normal bone and joint anatomy (p = 0.02). Moreover, MR images revealed bone oedema, joint effusion and soft tissue oedema in addition to fractures and calluses in stage I (bone dissolution), stage II (bone coalescence), and stage III (bone remodeling), i.e., in stages with overt radiographic pathology. According to this observational study, MR imaging, in addition to radiography, provides important information as to the extent and the natural course of bone injury in the diabetic Charcot foot. MR imaging, thus, may improve disease staging, as well as treatment monitoring.

Radiation exposure of patients in comprehensive computed tomography of the head in acute stroke.

BACKGROUND AND PURPOSE: To assess patient radiation exposure in comprehensive stroke imaging using multidetector row CT (MDCT) combining standard CT of the head, cerebral perfusion (CTP), and CT angiography (CTA) studies. METHODS: Examination protocols for CT and CTA of cerebral and cervical vessels, as well as CTP were simulated using a Somatom Sensation Cardiac 64. Effective doses were derived from measurements with the use of lithium-fluoride thermoluminescent dosimeters (LiF-TLD) at several organ sites using an Alderson-Rando phantom. RESULTS: LiF-TLD measurements resulted in effective doses of 1.7 mSv for CT, 1.9 mSv for CTA of intracranial vessels, and 2.8 mSv for CTA of cervical vessels, respectively. Depending on examination parameters, effective doses varied between 1.1 and 5.0 mSv for cerebral CTP. For CTP, local doses in the area of the primary beam ranged between 114 and 444 mGy. CONCLUSIONS: Comprehensive stroke imaging may result in up to 9.5 mSv with possible local doses of 490 mGy. Although critical doses for organ damage (eg, cataract formation or hair loss) are not reached, physicians need to be aware of possible radiation induced sequelae particularly in repetitive examinations.

[Interactive thresholded volumetry of abdominal fat using breath-hold t1-weighted magnetic resonance imaging]. / Interaktive Schwellenwert-basierte Volumetrie des abdominalen Fettgehaltes mittels atemgehaltener T1-gewichteter Magnetresonanztomographie.

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.

Autologous transplantation of CD133+ BM-derived stem cells as a therapeutic option for dilatative cardiomyopathy.

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.

Imaging characteristics of hepatocellular carcinoma using the hepatobiliary contrast agent Gd-EOB-DTPA.

PURPOSE: To evaluate the ability of contrast-enhanced magnetic resonance imaging (MRI) with Gd-EOB-DTPA in comparison with non-enhanced imaging and spiral computed tomography (CT) to provide additional information for classification and characterization of hepatocellular carcinoma. MATERIAL AND METHODS: Forty patients with histopathology-proven hepatocellular carcinoma were selected for this subgroup analysis from a phase-III multicenter study in 235 patients with known or suspected liver lesions. The primary analysis was comparison of the proportion of hepatocellular carcinoma correctly classified and characterized by combined pre-/post-contrast MRI compared with pre-contrast MRI alone or with spiral CT. All images were evaluated on site, and in a blinded reading by three independent readers off site. RESULTS: In the on-site evaluation, the lesions were correctly classified as a malignant tumor with combined MRI in 90.3%, with pre-contrast imaging alone in 82.9% and with spiral CT in 87.8% (n.s.). The proportion of correct characterization (lesion type diagnosis) with combined MRI was 85.4%, 75.6% for pre-contrast imaging, and 77.5% for spiral CT (n.s.), respectively. In the blinded reading, one reader showed a significant increase in the proportion of correctly characterized lesions by 27% (P<0.05). The other two readers showed a reduction in the proportion of correct characterization by 12% and 15%, respectively (n.s.). CONCLUSION: With regard to lesion classification, no difference was found between combined pre-/post-contrast MRI and spiral CT. A non-significant trend in favor of Gd-EOB-DTPA-enhanced MRI with regard to characterization of hepatocellular carcinoma was found, although the CT scans were not optimized as the MRI scans.

Volatile compounds of Soumbala, a fermented African locust bean (Parkia biglobosa) food condiment.

AIMS: To determine the profile of volatile compounds responsible for the aroma of Soumbala produced spontaneously and with pure and mixed cultures of Bacillus subtilis and Bacillus pumilus. METHODS AND RESULTS: Traditional and controlled fermentation trials of African locust bean with pure and mixed starter cultures of B. subtilis (B7, B9 and B15) and B. pumilus (B10) were performed. Aroma volatiles were analysed using Likens-Nikerson method coupled with gas chromatography and mass spectrophotometry. Sensory analysis of Soumbala as well as rice dishes prepared with each type of Soumbala were carried out by 10 panellists. In total 116 compounds were identified. They included pyrazines, aldehydes, ketones, esters, alcohols, acids, alkanes, alkenes, amines, pyridines, benzenes, phenols, sulphurs, furans and other compounds. Using principal component analysis for comparison, the aroma profiles of the Soumbala samples could be separated into three groups. The sensory evaluation showed variable acceptability. However, it was noticed that Soumbala samples produced with starter cultures were scored higher than traditionally prepared Soumbala. CONCLUSIONS: Aroma volatiles and organoleptic properties of Soumbala vary according to the Bacillus isolates involved in the fermentation. SIGNIFICANCE AND IMPACT OF THE STUDY: This study contributes to the selection of Bacillus starter cultures for controlled production of Soumbala.

[Differential diagnosis of focal liver lesions using contrast-enhanced MRI with SHU 555 A in comparison with unenhanced MRI and multidetector spiral-CT]. / Dignitätsbeurteilung fokaler Leberläsionen mit der kontrastverstärkten MRT mit SHU 555 A im Vergleich zur nativen MRT und zur Mehrzeilen-Detektor-Spiral-CT.

PURPOSE: To 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 METHODS: In 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. RESULTS: The 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). CONCLUSION: Contrast-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.

Effect of soybean fortification on Ghanaian fermented maize dough aroma.

The effect of soy fortification on the development of aroma compounds, dough acidity and growth of the predominant microorganisms were investigated in Ghanaian maize dough fermented spontaneously over a period of 72 h. The fortified maize dough was prepared from a milled mixture of soaked maize grains and pre-soaked, blanched and dehulled soybeans added at 20% replacement level. Extracts of volatiles from the soy-fortified and unfortified dough samples were obtained by Likens-Nickerson simultaneous distillation and extraction method analysed by gas chromatography-mass spectrometry and gas chromatography-olfactometry (gas chromatography-sniffing). Major aroma compound groups identified in the fermented dough samples were carbonyls, alcohols, esters and volatile organic acids. Twenty-nine carbonyls were found in soy-fortified maize dough compared with 21 in the unfortified dough. Although the same alcohols were found in both dough samples, the total concentration of alcohols was slightly different, being less for the soy-fortified sample. Total concentration of esters in soy-fortified dough decreased after 72 h while levels in the unfortified dough increased. Increases in the production of lactic and acetic acids occurred in soy-fortified samples, but the maximum concentrations of acetic acid achieved did not differ significantly between the two types of dough. Twenty-three compounds were perceived to be contributing to the typical aroma of soy-fortified maize dough by the method of gas chromatography-sniffing. These included 12 carbonyls, six alcohols, two esters, two acids and one furan.

[Mycotic visceral artery aneurysm: clinical picture, diagnosis, differential therapy, and discussion of the literature]. / Das mykotische Viszeralarterienaneurysma. Klinik, Diagnostik und Differenzialtherapie mit Darstellung der Literatur.

Bacterially infected arterial aneurysms were named mycotic aneurysms by William Osler in 1885 due to their morphology. This rare vascular disease is mainly localized in the femoral artery but also occurs in the aorta and visceral arteries. After the first surgically treated mycotic visceral aneurysm in 1949, we found 36 casuistics in the literature. We report on two patients treated in our department with mycotic visceral aneurysms and discuss the literature concerning topography, differential diagnosis, and surgical management.

Autologous bone marrow-derived stem cell therapy in combination with TMLR. A novel therapeutic option for endstage coronary heart disease: report on 2 cases.

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.

Volatile compounds produced by Lactobacillus fermentum, Saccharomyces cerevisiae and Candida krusei in single starter culture fermentations of Ghanaian maize dough.

AIMS: To identify and compare the volatile compounds associated with maize dough samples prepared by spontaneous fermentation and by the use of added starter cultures in Ghana. METHODS AND RESULTS: The starter cultures examined were Lactobacillus fermentum, Saccharomyces cerevisiae and Candida krusei. For identification of aroma volatiles, extracts by the Likens-Nickerson simultaneous distillation and extraction technique were analysed by gas chromatography-mass spectrometry (GC-MS) and using a trained panel of four judges by GC-Olfactometry (GC-sniffing). Compounds identified by GC-MS in maize dough samples after 72 h of fermentation included 20 alcohols, 22 carbonyls, 11 esters, seven acids, a furan and three phenolic compounds. Of the total 64 volatile compounds, 51 were detected by GC-sniffing as contributing to the aroma of the different fermented dough samples. Spontaneously fermented maize dough was characterized by higher levels of carbonyl compounds while fermentations with added L. fermentum recorded the highest concentration of acetic acid. S. cerevisiae produced higher amounts of fusel alcohols and increasing levels of esters with fermentation time and C. krusei showed similarity to L. fermentum with lower levels of most volatiles identified. CONCLUSION: The present study has given a detailed picture of the aroma compounds in fermented maize and demonstrated that the predominant micro-organisms in fermented maize dough can be used as starter cultures to modify the aroma of fermented maize dough. SIGNIFICANCE AND IMPACT OF THE STUDY: The study has documented the advantage of using starter cultures in African traditional food processing and provided a scientific background for introducing better controlled fermentations.

Dose reduction in multi-slice CT of the heart by use of ECG-controlled tube current modulation ("ECG pulsing"): phantom measurements.

PURPOSE: To evaluate the effect of ECG-controlled tube current modulation on radiation exposure in retrospectively-ECG-gated multislice CT (MSCT) of the heart. MATERIAL AND METHODS: Three different cardiac MSCT protocols with different slice collimation (4 x 1, and 4 x 2.5mm), and a pitch-factor of 1.5 and 1.8 were investigated at a multi-slice CT scanner Somatom Volume Zoom, Siemens. An anthropomorphic Alderson-Rando phantom was equipped with LiF-thermoluminescence dosimeters at several organ sites, and effective doses were calculated using ICRP-weighting factors. Scan protocols were performed with ECG-controlled tube current modulation ("ECG pulsing") at two different heart rates (60 and 80 bpm). These data were compared to previous data from MSCT of the heart without use of "ECG pulsing". RESULTS: Radiation exposure with (60 bpm) and without tube current modulation using a 2.5 mm collimation was 1.8 mSv and 2.9 mSv for females, and 1.5 mSv and 2.4 mSv for males, respectively. For protocols using a 1 mm collimation with a pitch-factor of 1.5 (1.8), radiation exposure with and without tube current modulation was 5.6 (6.3) mSv and 9.5 (11.2) mSv for females, and 4.6 (5.2) mSv and 7.7 (9.2) mSv for males, respectively. At higher heart rates (80 bpm) radiation exposure is increased from 1.5 - 1.8 mSv to 1.8 - 2.1 mSv, using the 2.5 mm collimation, and from 4.6 - 5.6 mSv to 5.9 - 7.2 mSv, for protocols using 1 mm collimation. CONCLUSIONS: The ECG-controlled tube current modulation allows a dose reduction of 37 % to 44 % when retrospectively ECG-gated MSCT of the heart is performed. The tube current - as a function over time - and therefore the radiation exposure is dependent on the heart rate.