Redefining the Pulvinar Sign in Fabry Disease.

BACKGROUND AND PURPOSE: The pulvinar sign refers to exclusive T1WI hyperintensity of the lateral pulvinar. Long considered a common sign of Fabry disease, the pulvinar sign has been reported in many pathologic conditions. The exact incidence of the pulvinar sign has never been tested in representative cohorts of patients with Fabry disease. The aim of this study was to assess the prevalence of the pulvinar sign in Fabry disease by analyzing T1WI in a large Fabry disease cohort, determining whether relaxometry changes could be detected in this region independent of the pulvinar sign positivity. MATERIALS AND METHODS: We retrospectively analyzed brain MR imaging of 133 patients with Fabry disease recruited through specialized care clinics. A subgroup of 26 patients underwent a scan including 2 FLASH sequences for relaxometry that were compared with MRI scans of 34 healthy controls. RESULTS: The pulvinar sign was detected in 4 of 133 patients with Fabry disease (3.0%). These 4 subjects were all adult men (4 of 53, 7.5% of the entire male population) with renal failure and under enzyme replacement therapy. When we tested for discrepancies between Fabry disease and healthy controls in quantitative susceptibility mapping and relaxometry maps, no significant difference emerged for any of the tested variables. CONCLUSIONS: The pulvinar sign has a significantly lower incidence in Fabry disease than previously described. This finding, coupled with a lack of significant differences in quantitative MR imaging, allows hypothesizing that selective involvement of the pulvinar is a rare neuroradiologic sign of Fabry disease.

The kidney in Fabry's disease.

Fabry disease (FD) is an X-linked disease in which mutations of the GLA gene result in a deficiency of the enzyme α-galactosidase A and subsequent progressive, intralysosomal deposition of undegraded glycosphingolipid products, primarily globotriaosylceramide, in multiple organs. Progressive nephropathy is one of the main features of FD and is marked by an insidious development, with an overall rate of progression of chronic kidney disease (CKD) very similar to diabetic nephropathy. Untreated patients usually develop end stage renal disease in their 50s. The decline in renal function in FD is adversely affected by male gender, advanced CKD, hypertension and, in particular, severe proteinuria. Enzyme replacement therapy (ERT) has been shown to slow the progression of Fabry nephropathy. The current consensus is that ERT should be started in all men and women with signs of renal involvement.

Coronary artery calcification and outcomes in diabetic patients with and without chronic kidney disease.

BACKGROUND/AIMS: Presence and progression of coronary artery calcification (CAC) indicate severe atherosclerosis and predict cardiovascular events (CVE) in dialysis (ESRD patients) and nondialysis patients (CKD patients). This study aimed at evaluating the prevalence and progression of CAC and CVE in CKD patients with diabetes in whom atherosclerosis of coronary arteries is the leading cause of CVE. METHODS: This was a retrospective study conducted in both out- and in-patients with chronic kidney disease, stage 2-5. CAC were assessed by the total calcium (TC) score. CT scans were performed at the entry and at the end of the study. RESULTS: Patients (n = 341) were divided into nondiabetic (n = 281) and diabetic patients (n = 60). CAC prevalence and TC score were higher in diabetics and associated with age, diabetes mellitus and pulse pressure. Diabetics with CKD presented a higher percentage of CAC progression and CVE. CONCLUSION: Prevalence, extent, progression of CAC and CVE are higher in diabetics with concomitant CKD. These findings may promote a more aggressive protocol of screening and care in type 2 diabetics with concomitant CKD.

Effects of switching from agalsidase Beta to agalsidase alfa in 10 patients with anderson-fabry disease.

Anderson-Fabry disease (AFD) is a multiorgan X-linked lysosomal storage disease that particularly affects the heart, kidneys, and cerebrovascular system. Current treatment is enzyme replacement therapy (ERT) with agalsidase beta (Fabrazyme(®), Genzyme Corporation, Cambridge, MA, USA) or agalsidase alfa (Replagal(®), Shire Human Genetic Therapies AB, Lund, Sweden). It was recommended that patients switch to agalsidase alfa due to a manufacturing shortage of agalsidase beta beginning in June 2009. This study assessed the effect of switching to agalsidase alfa on clinical outcomes in patients with AFD previously treated with agalsidase beta. Ten patients (seven male, three female) with genetically confirmed AFD and at least 48 months' continuous data collected during treatment with agalsidase beta 1 mg/kg every other week were switched to agalsidase alfa 0.2 mg/kg every other week for at least 20 months, with prospective clinical evaluations every 6 months. Pre-switch data was collected retrospectively from patient charts. Cardiac functional parameters were assessed using magnetic resonance imaging. Results showed that renal function was normal (estimated glomerular filtration rate ≥90 mL/min/1.73 m(2)) in 8 of 10 patients prior to agalsidase alfa and generally remained stable after the switch. Cardiac mass decreased significantly (p < 0.05 vs pre-ERT) after agalsidase beta and remained unchanged after switching to agalsidase alfa. Symptoms of pain and health status scores did not deteriorate during agalsidase alfa therapy. Adverse events were mostly mild and infusion related. In conclusion, switching to agalsidase alfa was relatively well tolerated and associated with stable clinical status and preserved renal and cardiac function.

Role of cardiac MRI in evaluating patients with Anderson-Fabry disease: assessing cardiac effects of long-term enzyme replacement therapy.

PURPOSE: Anderson-Fabry disease is a multisystemic disorder of lipid metabolism secondary to X-chromosome alterations and is frequently associated with cardiac manifestations such as left ventricular (LV) hypertrophy, gradually leading to an alteration in cardiac performance. The purpose of this study was to monitor, using magnetic resonance imaging (MRI), any changes produced by enzyme replacement therapy with agalsidase beta at the cardiac level in patients with Anderson-Fabry disease. MATERIALS AND METHODS: Sixteen (ten men, six women) patients with genetically confirmed Anderson-Fabry disease underwent cardiac MRI before starting enzyme replacement therapy (baseline study) and after 48 months of treatment with agalsidase beta at the dose of 1 mg/kg (follow-up study). RESULTS: After 48 months of treatment, a significant reduction in LV mass and wall thickness was observed: 187±59 g vs. 149±44 g, and 16±3 mm vs. 13±3 mm, respectively. A significant reduction in T2 relaxation time was noted at the level of the interventricular septum (81±3 ms vs. 67±7 ms), at the apical level (80±8 ms vs. 63±6 ms) and at the level of the lateral wall (82±8 ms vs. 63±10 ms) (p<0.05). No significant variation was observed in ejection fraction between the two studies (65±3% vs. 64±2%; p>0.05) (mean bias 1.0); however, an improvement was noted in the New York Heart Association (NYHA) class of the majority of patients (12/16) (p<0.05). CONCLUSIONS: In patients with Anderson-Fabry disease undergoing enzyme replacement therapy with agalsidase beta, MRI documented a significant reduction in myocardial T2 relaxation time, a significant decrease in maximal myocardial thickness and in total LV mass. MRI did not reveal significant improvements in LV global systolic function; however, improvement in NYHA functional class was noted, consistent with improved diastolic function.

Cardiac magnetic resonance imaging illustrating Anderson-Fabry disease progression.

Anderson-Fabry disease is an X-linked lysosomal storage disorder resulting from a deficiency of the enzyme α-galactosidase A (α-Gal A) and subsequent cellular storage of the enzyme's substrate globotriaosylceramide (Gb3) and related glycosphingolipids. We report a case of Anderson-Fabry disease with cardiac involvement evaluated with cardiovascular MRI. Disease progression was observed despite enzyme replacement therapy.

Comparison between multislice CT and MR imaging in the diagnostic evaluation of patients with pancreatic masses.

PURPOSE: This study compared the results of multislice computed tomography (MSCT) and high-field magnetic resonance imaging (MRI) in the diagnostic evaluation of pancreatic masses. MATERIALS AND METHODS: Forty patients with clinical and ultrasonographic evidence of pancreatic masses underwent MSCT and MRI. The majority of patients (31/40, 78%) had proven malignant pancreatic tumours (24 ductal adenocarcinoma, six mucinous cystadenocarcinoma, one intraductal papillary mucinous carcinoma), whereas the remaining patients (9/40, 22%) were found to have benign lesions (eight chronic pancreatitis, one serous cystadenoma). Results of the imaging studies were compared with biopsy (n=33) and/or histology (n=7) findings to calculate sensitivity, specificity, accuracy and positive (PPV) and negative (NPV) predictive value for correct identification of tumours and evaluation of resectability of malignancies. RESULTS: Both for tumour identification and resectability, MSCT and MRI had comparable diagnostic accuracy, with no statistically significant differences between them. Tumour identification CT/MRI: accuracy 98/98%, sensitivity 100/100%, specificity 88/88%, PPV 97/97%, NPV 100/100%; tumour resectability CT/MRI: accuracy 94/90%, sensitivity 92/88%, specificity 100/100%, PPV 100/100%, NPV 78/70%. CONCLUSIONS: MRI represents a valid diagnostic alternative to CT in the evaluation of patients with pancreatic masses, both for correct identification and characterisation of primary lesions and to establish resectability in the case of malignancies. New high-field MRI equipment allows optimal imaging quality with good contrast resolution in evaluating the upper abdomen.

Dual-time-point [18F]-FDG PET/CT in the diagnostic evaluation of suspicious breast lesions.

PURPOSE: The authors sought to evaluate whether the reacquisition of images 3 h after administration of radiotracer improves the sensitivity of fluorine-18 fluorodeoxyglucose positron emission tomography computed tomography ([(18)F]-FDG PET/CT) in patients with suspicious breast lesions. MATERIALS AND METHODS: Forty-eight patients with 59 breast lesions underwent an [(18)F]-FDG PET/CT study in the prone position with a dual-time-point acquisition performed in the early phase 1 h after FDG administration (PET-1) and in the delayed phase 3 h after FDG administration (PET-2). Both examinations were evaluated qualitatively and semiquantitatively with calculation of the mean percentage variation of the standard uptake values (Delta% SUV(max)) between PET-1 and PET-2. All lesions with an SUV(max) >or=2.5 at PET-1 or a reduction in SUV between PET-1 and PET-2 were considered benign. The definitive histopathological diagnosis was available for all patients included in the study. RESULTS: The dual-time-point acquisition of [(18)F]-FDG PET/CT displayed an accuracy of 85% for lesions with an SUV(max) >or=2.5 and/or positive Delta% SUV(max), with sensitivity and specificity values of 81% and 100% compared with 69%, 63% (both p<0.001) and 100% (p=n.s.), respectively, for the single-time-point acquisition. Malignant lesions showed an increase in FDG uptake between PET-1 and PET-2, with a Delta% SUV(max) of 10+/-7 (p<0.04). In contrast, benign lesions showed a decrease in SUV between PET-1 and PET-2, with a Delta% SUV(max) of -21+/-7 (p<0.001). CONCLUSIONS: The delayed repeat acquisition of PET images improves the accuracy of [(18)F]-FDG PET/CT in patients with suspicious breast lesions with respect to the single-time-point acquisition. In addition, malignant breast lesions displayed an increase in FDG uptake over time, whereas benign lesions showed a reduction. These variations in FDG uptake between PET-1 and PET-2 are a reliable parameter that can be used for differentiating between benign and malignant breast lesions.

Assessment of left ventricular volumes with cardiac MRI: comparison between two semiautomated quantitative software packages.

PURPOSE: This study compared two quantitative semiautomated software packages for volumetric analysis of the left ventricle (LV) by magnetic resonance (MR) imaging using two-dimensional (2D) cine balanced steady-state free precession (b-SSFP) sequences. MATERIALS AND METHODS: We included 46 consecutive patients who underwent cardiac MR imaging for various indications. Two-dimensional cine b-SSFP sequences were used to assess the LV. Data sets were evaluated with two dedicated software packages: ViewForum, version 4.2, and Argus, version Va60C. Results were compared with Student's t test for paired samples, Pearson's r correlation coefficient and R2 coefficient of determination; ejection fraction differences were assessed with Bland-Altman analysis. The time required for analysis was also recorded. RESULTS: We observed very high levels of concordance and reproducibility. High correlation was observed for ejection fraction (p>0.05; r=0.9; R (2)=0.82). The time required for analysis was 7.6+/-2.78 min vs. 7.52+/-2.4 min (p>0.05; r=0.85; R (2)=0.73). Intraobserver and interobserver variability did not show significant differences. CONCLUSIONS: LV volume evaluation is an integral part of cardiac MR imaging. In our experience, there is no significant difference between the commonly used software packages in either quantitative output or time required for analysis.

Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study.

BACKGROUND: Anderson-Fabry disease is a multisystem X linked disorder of lipid metabolism frequently associated with cardiac symptoms, including left ventricular (LV) hypertrophy gradually impairing cardiac function. Evidence showing that enzyme-replacement therapy (ERT) can be effective in reducing LV hypertrophy and improving myocardial function in the long term is limited. OBJECTIVE: This study aimed to assess the long-term effects of ERT with recombinant alpha-galactosidase A (agalsidase beta, Fabrazyme) on LV function and myocardial signal intensity in 11 patients with Anderson-Fabry disease. PATIENTS: Eleven patients (eight males, three females) with varying stages of genetically confirmed Anderson-Fabry disease were examined by means of physical examination and magnetic resonance imaging before ERT with agalsidase beta at 1 mg/kg every other week (study 1) and after a mean treatment duration of 45 months (study 2). RESULTS: At 45 months of treatment, LV mass and LV wall thickness had significantly reduced: 188 (SD 60) g versus 153 (47) g, and 16 (4) mm versus 14 (4) mm, respectively. Furthermore, a significant reduction in myocardial T2 relaxation times was noted in all myocardial regions, that is, interventricular septum 80 (5) ms versus 66 (8) ms, apex 79 (10) ms versus 64 (10) ms, and lateral wall 80 (8) ms versus 65 (16) ms. Changes in LV ejection fraction were not significant. Amelioration of clinical symptoms was observed in all patients. CONCLUSIONS: Long-term therapy with agalsidase beta at 1 mg/kg every 2 weeks was effective in significantly reducing LV hypertrophy, improving overall cardiac performance and ameliorating clinical symptoms in patients with Anderson-Fabry disease.

Comparative diagnostic evaluation with MR cholangiopancreatography, ultrasonography and CT in patients with pancreatobiliary disease.

PURPOSE: The aim of this study was to directly compare the results of magnetic resonance cholangiopancreatography (MRCP) with those of ultrasonography (US) and multislice computed tomography (MSCT) in the diagnosis of pancreaticobiliary diseases. MATERIALS AND METHODS: A total of 70 patients (41 men, 29 women) aged 22-89 years were studied either before (n=59) or after cholecystectomy (n=11) for biliary lithiasis. Clinical signs and symptoms were jaundice (n=15), abdominal pain (n=37) and proven biliary lithiasis (n=18). MRCP was performed in all patients, whereas abdominal US was performed in 55 (group 1) and MSCT in 37 (group 2) patients. A regional evaluation of the main structures of the pancreaticobiliary system was performed: gallbladder and cystic duct, intra- and extrahepatic bile ducts and main pancreatic duct. Histology (n=27), biopsy (n=5), endoscopic retrograde cholangiopancreatography (ERCP) (n=28) and/or clinical-imaging follow-up (n=10) were considered standards of reference. In particular, patients were classified as showing benign (n=47) or malignant (n=12) lesions or normal biliary anatomy (n=11). RESULTS: In group 1, the results of MRCP and US were concordant in the majority (92%) of cases; however, statistically significant discordance (p<0.01) was found in the evaluation of the extrahepatic ducts, with nine cases (16%) of middle-distal common bile duct stones being detected on MRCP only. In group 2, the results of MRCP and MSCT were also concordant in most cases (87%). However, findings were significantly discordant when the intra- and extrahepatic ducts were analysed, with seven (19%) and six (16%) cases, respectively, of lithiasis being detected on MRCP only (p<0.01 for both). CONCLUSIONS: The results of our study confirm the diagnostic potential of MRCP in the study of the pancreaticobiliary duct system. In particular, the comparison between MRCP and US and MSCT indicates the superiority of MRCP in evaluating bile ducts and detecting stones in the common bile duct.

Cardiac performance during exercise in patients with Fabry's disease.

BACKGROUND: Fatigability and dyspnoea on effort are present in many patients with Fabry's disease. We assessed the determinants of cardiac performance during exercise in patients with Fabry's disease and preserved left ventricular ejection fraction at rest. MATERIALS AND METHODS: Sixteen patients with Fabry's disease and 16 control subjects underwent radionuclide angiography at rest and during exercise, tissue Doppler echocardiography and magnetic resonance imaging at rest. RESULTS: The exercise-induced change in stroke volume was +25 +/- 14% in controls and +5.8 +/- 19% in patients with Fabry's disease (P < 0.001). In 10 patients (group 1), the stroke volume increased (+19 +/- 10%), and in 6 patients (group 2) it decreased (-16 +/- 9%) with exercise. Patients of group 2 were older, had worse renal function, higher left ventricular mass and impaired diastolic function compared to group 1. The abnormal stroke volume response to exercise in group 2 was associated with a decrease in end-diastolic volume (P < 0.001) and a lack of reduction of end-systolic volume (P < 0.01) compared with both controls and group 1. The ratio of peak early-diastolic velocity from mitral filling to peak early-diastolic mitral annulus velocity was the only independent predictor of exercise-induced change in stroke volume (B -0.44; SE 0.119; beta-0.70; P < 0.005). CONCLUSIONS: The majority of patients with Fabry's disease were able to augment stroke volume during exercise by increasing end-diastolic volume, whereas patients with more advanced cardiac involvement may experience the inability to increase cardiac output by the Frank Starling mechanism.

Pathogenesis of hypertrophic cardiomyopathy. Impact of growth factors on left ventricular anatomy.

AIM: The aim of this study was to evaluate the effect of insulin-like growth factor 1 (IGF1) and transforming growth factor beta-1 (TGFbeta-1) on collagen turnover, left ventricular (LV) hypertrophy and on passive diastolic function of the LV in hypertrophic cardiomyopathy (HCM). METHODS: This study group comprised 34 patients with non-dilated HCM. Procollagen I amino-terminal propeptide (PINP) and collagen I carboxy-terminal telopeptide (ICTP) were measured by radioimmunoassay. Matrix metalloproteinase 9 (MMP 9), IGF1 and TGFalfa-1 were determined by enzyme-linked immunosorbent assay. The difference in duration between transmitral forward (A) and pulmonary venous retrograde (Ar) waves, was considered as an estimate of passive diastolic function; the ratio between the peak flow velocity at rapid filling at the mitral level (E) and E' measured by tissue Doppler was considered an estimate of active diastolic function. LV mass was measured and normalized to body surface area (LVMi) by cardiac magnetic resonance imaging. RESULTS: LVMi correlates to E/E' (r=0.597, P=0.019 ) and is inversely related to A-Ar (r=0.453, P=0.015). TGFbeta-1 is directly related to active MMP 9 (r=0.439, P=0.012 ). IGF1 is directly related to PICP-ICTP (r=0.347, P=0.501), that expresses the balance between collagen I synthesis and its degradation. CONCLUSION: The study demonstrated that in HCM, LVMi influences active and passive diastolic dysfunction and that IGF1 stimulates collagen synthesis and TGFbeta-1 is related to LV hypertrophy.

Difficult vascular conditions in kidney transplantation.

The new dialytic and medical therapies have improved the survival of uremic patients and their preservation of a efficacious clinical condition so as to warrant suitability for transplantation, even after a long period of dialysis. In addition, today the use of a "marginal donor" and "marginal kidney" are often used to increase the pool of available organs, so that the surgeon must face more technical difficulties than in the past; anomalies of the donor kidney, harvesting and bench surgery damages, as well as vascular pathologies in the recipient. A review of our 151 renal transplantations from January 1999 to May 2003 showed that it was often possible to overcome these technical difficulties yielding good results. This work sought to demonstrate that neither "marginal donor"/"marginal kidney" used to expand donor pool nor recipient vascular pathologies should be considered transplant contraindications. Knowledge of various technical options and the ability to put them rapidly into practice are necessary to use any organ.

Diagnostic accuracy of chemical-shift MR imaging to differentiate between adrenal adenomas and non adenoma adrenal lesions.

PURPOSE: The objective of this study was to evaluate the diagnostic accuracy of chemical-shift (CS) magnetic resonance (MR) imaging in the differential diagnosis of adenoma and nonadenoma adrenal masses. MATERIALS AND METHODS: We enrolled 36 patients (9 men, 27 women, mean age 51.3+14.4 years) with unilateral (n=31) or bilateral (n=5) adrenal masses incidentally discovered during imaging examinations [ultrasound (US), computed tomography (CT)] performed for other indications. A total of 41 adrenal lesions were evaluated (mean diameter 3.0+2.2 cm). Histology (n=19), biopsy (n=3) or clinical-imaging follow-up (n=19) demonstrated 29 adenomas, five pheochromocytomas, three cysts and four carcinomas. MR imaging was performed using the following breath-hold sequences: T1-fast field echo (FFE) [repetition time (TR)/echo time (TE)=236/4.6 ms], T2-turbo spin echo-single shot (TSE-SSh) (TR/TE=831/80 ms), T1-DUAL-FFE (TR=236, double TE=4.6/2.3 ms in phase and out of phase) and T1-FFE after gadolinium-DTPA (Gd). Axial and coronal imaging planes were used, with a slice thickness of 3-5 mm. MR images were qualitatively assessed for signal intensity of the adrenal mass relative to the liver on T1, T2, CS and T1-Gd scans; diagnostic criteria for adenomas were isointensity or hypointensity on both T1 and T2 scans, out-of-phase CS signal loss and mild transient enhancement after Gd. RESULTS: Analysis of T1-T2 signal intensity showed diagnostic accuracy, sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of 80%, 72%, 100%, 100% and 60%, respectively. In contrast, analysis of CS and T1-Gd signal intensity showed diagnostic accuracy, sensitivity, specificity, PPV and NPV for both sequences of 93%, 90% (p<0.05 vs. T1-T2 analysis), 100%, 100% and 80% (p<0.05 vs. T1-T2 analysis), respectively. CONCLUSIONS: CS MR imaging significantly improves characterization of adrenal masses compared with conventional T1-T2-weighted images, providing accuracy similar to that of the T1 sequence after Gd. Therefore, the CS sequence is strongly recommended for MR study of adrenal masses, and its use might obviate the need for Gd administration.

Multislice CT with single-phase technique in patients with suspected pancreatic cancer.

PURPOSE: The purpose of this study was to evaluate the role of multislice computed tomography (MSCT) with a single-phase technique in patients with suspected pancreatic cancer (PC). MATERIALS AND METHODS: Seventy-eight patients underwent MSCT with the following technical parameters: collimation: 4x1 mm; pitch 1; 120 kVp; 260 mAs. The pre-contrast scan was followed by a single acquisition phase in the caudocranial direction from the inferior hepatic margin to the diaphragm with a 60-s delay after IV administration of 150 ml of iodinated contrast material at a rate of 3 ml/s. Two radiologists assessed the single images independently. Receiver operating characteristics (ROC) curves were obtained for each of the two observers. RESULTS: The final diagnosis was pancreatic cancer in 46 cases and chronic pancreatitis in 32 cases. Areas under the curve (AZ) for diagnosis and evaluation of disease resectability were 0.97 and 0.93 for the first observer (p=ns), and 0.97 and 0.90 for the second observer (p=ns). The mean difference in tissue attenuation values between the cancer and normal pancreas was 72 +/- 3 Hounsfield units (HU). No statistically significant differences were observed in the degree of opacification between the peripancreatic arteries and veins. CONCLUSIONS: MSCT with a single-phase technique is an accurate and reproducible method for diagnosis and evaluation of disease resectability in patients with suspected PC, ensuring optimal tumour-to-pancreas contrast and maximal opacification of the main peripancreatic arterial and venous structures.

Triphasic helical CT in Budd-Chiari syndrome: patterns of enhancement in acute, subacute and chronic disease.

AIM: To retrospectively evaluate helical computed tomography (CT) findings in a series of consecutive patients with Budd-Chiari syndrome. METHODS: Patterns of enhancement observed at contrast-enhanced helical CT in 10 consecutive patients (six women, four men; aged 27-51 years) with either acute, subacute or chronic Budd-Chiari syndrome were retrospectively evaluated along with the status of the hepatic veins. All patients underwent triphasic helical CT (10 mm beam collimation, 7 mm rec. intervals, 120 kV, 200-250 mA, pitch = 1.0) performed at 20-25, 70-75 and 300 s after i.v. bolus (3 ml/s) injection of 150 ml iodinated non-ionic contrast media. RESULTS: Abnormal patterns of enhancement were identified in eight patients. In all patients with acute Budd-Chiari disease (3/3) abnormal arterial enhancement of the caudate lobe, the so-called "fan-shaped pattern" was observed, whereas visible venous thrombosis was only depicted in two. Conversely, a "patchy pattern" of enhancement was observed in five out of seven patients with either sub-acute (2) or chronic Budd-Chiari disease (5) along with a strip-like appearance or lack of visualization of hepatic veins. CONCLUSIONS: The "fan-shaped" pattern of enhancement represent a characteristic finding of acute Budd-Chiari disease, and it may help to suggest the correct diagnosis even in absence of visible venous thrombosis.

A study on two different CAD systems for mammography as an aid to radiological diagnosis in the search of microcalcification clusters.

OBJECTIVE: The aim of the present study was to evaluate the efficacy of two different computer aided detection (CAD) systems for mammography in improving radiological diagnosis in the search of microcalcification clusters. The CAD systems used are: the SecondLooktrade mark (CADx Medical Systems, Canada) commercial system and the CALMA (computer assisted library in MAmmography) research CAD system. Three radiologists were asked to read mammographic images with and without the support of the CAD systems. MATERIAL AND METHODS: Three radiologists with respectively 3, 5 and 7 years of practice in mammogram reading in an Italian public hospital analysed a dataset composed of 120 digitized mammograms of healthy subjects with no lesion (proven by a radiological follow up of at least 3 years) and 70 images of patients with malignant cluster of microcalcification (proven by histopathological examination) both with no CAD support as well as with the help of the SecondLooktrade mark system. After 3 months they were asked to observe the same digitized mammograms with the assistance of the CALMA system. The radiologists worked independently and were unaware of the final diagnosis. The values of the area A(z) under the ROC curve, diagnostic sensitivity, specificity, positive and negative predictive values, and diagnostic accuracy were evaluated with and without the support of the CAD systems. The reading time and qualitative evaluations of each radiologist were also reported. RESULTS: With the support of the two CAD systems an improvement in A(z) area was obtained ranging from 0.01 to 0.04. Sensitivity increased from +8.6 to +15.7% and specificity decreased from 0.8 to 4.2%. CONCLUSION: In our study, not conditioned by the dataset, the CAD systems as second reader produced an increase in overall sensitivity of up to 15.7%, with a little decrease in specificity of up to 4.2%. Based on these results both CAD systems might be used in the current practise to improve the sensitivity values of conventional reading (radiologist alone). The results of this study show that no significant differences exist in term of A(z), sensitivity and specificity between CALMA and CADx.

The many ways to myocardial perfusion imaging.

Myocardial perfusion imaging (MPI) is important for the management of patients with suspected or known coronary artery disease (CAD). Nuclear cardiology is the most widely used noninvasive approach for the assessment of myocardial perfusion. The available single-photon emission computed tomography (SPECT) flow agents are characterized by a rapid myocardial extraction and by a cardiac uptake proportional to blood flow. In addition, different positron emission tomography (PET) tracers may be used for the quantitative measurement of myocardial blood flow and coronary flow reserve. The decrease in blood flow, determined by coronary artery stenosis, produces myocardial ischemia leading to perfusion abnormalities detectable by SPECT or PET in the early phase of ischemia. Other imaging techniques, such as contrast echocardiography and magnetic resonance imaging (MRI) have been more recently proposed as alternative methods for the evaluation of myocardial perfusion. Although several technical aspects have to be better defined to use contrast echocardiography in clinical practice, this approach appears promising for the evaluation of myocardial perfusion. MRI has also been proposed for the assessment of myocardial perfusion by measuring the alteration of regional myocardial magnetic properties after the intravenous injection of contrast agents. Due to the high contrast and spatial resolution of the technique, MRI allows differentiating sub-endocardial and sub-epicardial perfusion, emerging as a potential alternative non-ionizing technique to evaluate myocardial perfusion. This review illustrates the noninvasive imaging modalities for the evaluation of myocardial perfusion, underlying advantages and disadvantages of each technique.