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We evaluated frequency, pattern, and associations of renal iron accumulation in sickle/β-thalassemia. Thirty-three sickle/β-thalassemia patients (36.5 ± 14.7 years; 13 females), 14 homozygous sickle cell disease (SCD) patients, and 71 thalassemia major (TM) patients, enrolled in the E-MIOT Network, underwent magnetic resonance imaging. Iron overload (IO) was quantified by the T2* technique. Sickle/β-thalassemia patients had a significantly lower frequency of renal IO (T2* 31 ms) than homozygous SCD patients (9.1% vs. 57.1%; P = 0.001), besides having similar hepatic, cardiac and pancreatic IO. Kidney T2* values were comparable between regularly transfused sickle/β-thalassemia and TM patients but were significantly lower in regularly transfused homozygous SCD patients than in the other two groups. In sickle/β-thalassemia patients, global renal T2* values were not associated with age, gender, splenectomy, and presence of regular transfusions or chelation. No correlation was detected between renal T2* values and serum ferritin levels or iron load in the other organs. Global renal T2* values were not associated with serum creatinine levels but showed a significant inverse correlation with serum lactate dehydrogenase (R = - 0.709; P 0.0001) and indirect bilirubin (R = - 0.462; P = 0.012). Renal IO is not common in sickle/β-thalassemia patients, with a prevalence significantly lower compared to that of homozygous SCD patients, but with a similar underlying mechanism due to the chronic hemolysis.

Evidence about the cross-talk between iron, glucose metabolism, and cardiac disease is increasing. We aimed to explore the link of pancreatic iron by Magnetic Resonance Imaging (MRI) with glucose metabolism and cardiac complications (CC) in sickle cell disease (SCD) patients.We considered 70 SCD patients consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network. Iron overload was quantified by R2* technique and biventricular function by cine images. Macroscopic myocardial fibrosis was evaluated by late gadolinium enhancement technique. Glucose metabolism was assessed by the oral glucose tolerance test.Patients with an altered glucose metabolism showed a significantly higher pancreas R2* than patients with normal glucose metabolism. Pancreatic siderosis emerged as a risk factor for the development of metabolic alterations (OddsRatio 8.25, 95%confidence intervals 1.51-45.1; p = .015). Global pancreas R2* values were directly correlated with mean serum ferritin levels and liver iron concentration. Global pancreas R2* was not significantly associated with global heart R2* and macroscopic myocardial fibrosis. Patients with history of CC showed a significantly higher global pancreas R2* than patients with no CC.Our findings support the evaluation of pancreatic R2* by MRI in SCD patients to prevent the development of metabolic and cardiac disorders.

In thalassemia major, pancreatic iron was demonstrated as a powerful predictor not only for the alterations of glucose metabolism but also for cardiac iron, fibrosis, and complications, supporting a profound link between pancreatic iron and heart disease. We determined for the first time the prevalence of pancreatic iron overload (IO) in thalassemia intermedia (TI) and systematically explored the link between pancreas T2* values and glucose metabolism and cardiac outcomes. We considered 221 beta-TI patients (53.2% females, 42.95 ± 13.74 years) consecutively enrolled in the Extension–Myocardial Iron Overload in Thalassemia project. Magnetic Resonance Imaging was used to quantify IO (T2* technique) and biventricular function and to detect replacement myocardial fibrosis. The glucose metabolism was assessed by the oral glucose tolerance test (OGTT). Pancreatic IO was more frequent in regularly transfused (N = 145) than in nontransfused patients (67.6% vs. 31.6%; p < 0.0001). In the regular transfused group, splenectomy and hepatitis C virus infection were both associated with high pancreatic siderosis. Patients with normal glucose metabolism showed significantly higher global pancreas T2* values than patients with altered OGTT. A pancreas T2* < 17.9 ms predicted an abnormal OGTT. A normal pancreas T2* value showed a 100% negative predictive value for cardiac iron. Pancreas T2* values were not associated to biventricular function, replacement myocardial fibrosis, or cardiac complications. Our findings suggest that in the presence of pancreatic IO, it would be prudent to initiate or intensify iron chelation therapy to prospectively prevent both disturbances of glucose metabolism and cardiac iron accumulation.

We determined the prevalence of incidental extracardiac findings (IEF) at Magnetic Resonance Imaging (MRI) potentially related to anemia and hypoxia in age- and sex-matched populations (N = 318) with thalassemia major (TM) and thalassemia intermedia (TI) enrolled in the Myocardial Iron Overload in Thalassemia network. Overall, IEFs were detected in 33.3% and 25.8% of patients with TI and TM, respectively (P = 0.114). TI and TM patients had elevated but comparable prevalence of renal, splenic and liver cysts, and vertebral hemangiomas while TI patients had a significant higher frequency of extramedullary hematopoiesis (EMH) (15.1% vs 4.4%; P = 0.002). The prevalence of total IEFs increased with advancing age. TI non-transfusion-dependent patients had a significantly lower frequency of renal cysts than TI transfusion-dependent patients (8.8% vs 26.4%; P = 0.005). The prevalence of renal cysts in the thalassemic population was significantly higher than that in the general population (19.2% vs 1.9%; P

Background: Chronically transfused homozygous sickle cell disease (HbSS) patients were shown to have higher kidney iron deposition than thalassemia major patients, not associated to total body iron and mainly caused by chronic hemolysis. Kidney iron deposition has not been explored in sickle beta-thalassemia (Sβ-thal), resulting from the inheritance of both sickle cell and beta-thalssemia genes. Aim: This multi center study aimed to study frequency, pattern, and associations of renal iron accumulation in sickle beta-thalassemia. Methods: Thirty-three Sβ-thal patients (36.49±14.72 years; 13 females) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia (E-MIOT) network were considered. Moreover, 20 healthy subjects, 14 HbSS patients and 71 thalassemia major (TM) patients were included as comparison groups. Hepatic, cardiac, pancreatic, and renal iron overload was quantified by the gradient-echo T2* technique. In each kidney T2* was measured in anterior, posterolateral, and posteromedial parenchymal regions and the global T2* value was calculated as the average of the two kidneys T2* values. Results. Global renal T2* were significantly higher in healthy subjects versus both Sβ-thal patients (49.68±10.09 ms vs 43.19±8.07 ms; P=0.013) and HbSS patients (49.68±10.09 ms vs 26.21±17.07 ms; P Sβ-thal patients showed comparable age, sex, frequency of regular transfusion, hematochemical parameters, and hepatic, cardiac and pancreatic iron load than HbSS patients, but they had a significant lower frequency of renal iron overload (global renal T2* Regularly transfused patients (16 Sβ-thal and 10 HbSS) were compared with TM patients, homogeneous for age and sex, but TM started regular transfusions significantly earlier and they were more frequently chelated. No significant difference was detected in terms of hepatic and cardiac iron levels, but TM patients had significantly lower pancreas T2* values than both the other two groups and significantly higher global renal T2* values than HbSS patients (42.87±9.43 ms vs 24.39±15.74 ms; P=0.001). In Sβ-thal patients no significant difference was detected between T2* values in left and right kidneys, and global renal T2* values were not associated to age, gender, splenectomy, and they were comparable between regularly transfused and non transfused patients. No correlation was detected between renal T2* values and serum ferritin levels or iron load in the other organs. Global renal T2* values were not associated with serum creatinine levels but showed a significant inverse correlation with serum lactate dehydrogenase (Figure 1). Conclusion. Renal iron deposition is not common in Sβ-thal patients, with a prevalence significantly lower compared to that of HbSS patients, but with a similar underlying mechanism due to the chronic hemolysis. Figure 1 Figure 1. Disclosures Pepe: Bayer S.p.A.: Other: no profit support; Chiesi Farmaceutici S.p.A: Other: no profit support. Maggio: Novartis: Membership on an entity's Board of Directors or advisory committees; Celgene Corp: Membership on an entity's Board of Directors or advisory committees; Bluebird Bio: Membership on an entity's Board of Directors or advisory committees.

Here, we report the case of a young patient admitted to the emergency department because of abdominal pain. Computed tomography revealed a mass within her right heart. Through serial multimodality imaging testing, including computed tomography, three-dimensional (2D)- and three-dimensional echocardiography, as well as cardiac magnetic resonance, the diagnosis of cardiac involvement in the course of Echinococcus granulosus infection was hypothesized.

Introduction. A prospective magnetic resonance imaging (MRI) study demonstrated a good control of myocardial iron overload (MIO) in terms of prevention and treatment in children with thalassemia major (TM). The aim of the present study was to evaluate if changes in MIO were related to baseline hepatic iron or changes in hepatic iron overload (HIO). Methods. We considered 68 TM patients enrolled in the MIOT (Myocardial Iron Overload in Thalassemia) project with less than 18 years at the first MRI scan and who performed a follow-up (FU) study at 18±3 months. Myocardial and hepatic iron burdens were quantified by the T2* technique. The value of 20 ms was used as conservative normal value for the global T2* value. Liver T2* values were converted into liver iron concentration (LIC) values. A LIC Results. Thirty-six patients were females and mean age at the time of the baseline MRI was 13.74±3.09 years. Baseline global heart T2* values were 29.72±11.21 ms and 16 (23.5%) patients showed significant baseline MIO. The percentage changes in global heart T2* values per month in the whole patient population were 0.66±1.70 and they resulted significantly higher in the 16 patients with significant baseline MIO versus the patients with no baseline MIO (1.99±2.53% vs 0.25±1.09% ms; P=0.002). Percentage changes in global heart T2* values per month were not influenced by initial MRI LIC values (R=0.048; P=0.695) (Figure 1A) and were comparable among the 4 groups of patients identified on the basis of baseline MRI LIC values (14 no HIO: 0.29±1.12% vs 21 mild HIO: 0.75±1.56% vs 15 moderate HIO: 0.82±2.03% vs 18 severe HIO: 0.71±2.00%; P=0.876). Percentage changes in global heart T2* values per month were not associated to final MRI LIC values (R=-0.134; P=0.277) (Figure 1B). The correlation between % changes in global heart T2* and MRI LIC values did not reach the statistical significance (R=-0.244; P=0.067) (Figure 1C). In patients with baseline MIO no correlation was found between % changes in global heart T2* values per month and initial MRI LIC values (R=-0.325; P=0.219) or % changes in MRI LIC values per month (R=-0.353; P=0.180). Conclusion. In pediatric TM patients changes in cardiac iron are not correlated to baseline MRI LIC values and changes in hepatic iron. So, our data seem not supporting the hypothesis for which it is necessary to clean the liver before removing iron from the heart. Figure 1 Disclosures Pepe: Chiesi Farmaceutici S.p.A., ApoPharma Inc., and Bayer: Other: No profit support.

To compare the sensitivity and specificity of diffusion-weighted (DW) magnetic resonance (MR) imaging for identifying liver metastases from neuroendocrine tumor (NET) to those of T2-weighted fast spin-echo (FSE) and three-dimensional dynamic gadolinium-enhanced MR imaging, with surgical and histopathologic findings as the reference standard.This retrospective study was approved by institutional review board, and informed consent was waived. Fifty-nine patients with NETs (41 patients with 162 liver metastases, and 18 control subjects with no liver metastases) underwent MR imaging that included DW, T2-weighted FSE, and dynamic gadolinium-enhanced MR sequences. Images were retrospectively reviewed by two abdominal radiologists, independently, for the detection and characterization of liver metastases. MR findings were compared with histopathologic and intraoperative ultrasonography findings for metastasis on a lesion-by-lesion basis to determine the sensitivity of each MR sequence alone and combined. Specificity was calculated by using the control population. Interreader agreement for each MR sequence and McNemar test were also calculated.There was excellent agreement between observers 1 and 2 for characterizing liver metastases at per-lesion analysis (κ coefficient: 0.86-1.00). DW MR was more sensitive (observer 1: sensitivity, 71.6% [116 of 162], 95% confidence interval [CI]: 64.2%, 78.0%; observer 2: sensitivity, 71.0% [115 of 162], 95% CI: 63.6%, 77.4%) than T2-weighted FSE (observer 1: sensitivity, 55.6% [90 of 162], 95% CI: 47.9%, 63.0%; observer 2: sensitivity, 55.6% [90 of 162], 95% CI: 47.9%, 63.0%) and dynamic gadolinium-enhanced MR (observer 1: sensitivity, 47.5% [77 of 162], 95% CI: 34.0%, 55.2%; observer 2: sensitivity, 48.1% [78 of 162], 95% CI: 40.6%, 55.8%) (P.001 for both, McNemar test). The specificity of these sequences ranged from 88.9% to 100% (DW MR vs T2-weighted FSE MR: P.99, DW MR vs dynamic gadolinium-enhanced MR: P = .61, and T2-weighted FSE MR vs dynamic gadolinium-enhanced MR: P = .61, McNemar test).DW MR imaging was more sensitive for the detection and characterization of liver metastases from NETs than T2-weighted FSE and dynamic gadolinium-enhanced MR imaging and should be systematically performed.

The aim of this work was to compare the quality and noise of true nonenhanced (TNE) and virtual nonenhanced (VNE) images in patients undergoing dualenergy computed tomography (DECT) of the liver. Twenty consecutive patients (mean age 54.7±19.9 years) prospectively underwent abdominal DECT to assess the liver using a triphasic protocol consisting of precontrast, arterial-phase and portal-phase acquisitions. Exclusion criteria were allergy to iodinated contrast material, impaired renal function and a body mass index (BMI) >35 kg/m2. The DE portal-phase acquisition was performed with automatic dose modulation (CARE Dose 4D). Nonionic iodinated contrast material (Iomeron 400) was administered at 0.625 gI/kg with a flow rate of 3.5 ml/s. Axial VNE images were reconstructed based on the portal data set using a collimation and an increment of 5 mm and were compared with TNE images reconstructed with the same parameters. The average image quality and noise were analysed by two radiologists in separate reading sessions. No statistically significant difference (p>0.05) in image quality was observed between VNE (4.00±0.85) and TNE images (4.35±0.58). A sufficient diagnostic quality was found in 95.0% (19/20) of VNE images and in 100% of TNE images. No statistically significant difference (p

WehavereadwithgreatinterestthearticlebyScanagattaand colleagues [1] reporting the case of a huge hepatocarci-noma (HCC) with intrathoracic extension. The authorsaddressed the diagnostic challenge to recognise tumourorigin and possible pitfalls with intrathoracic neoplasm; theyalso advocated the use of magnetic resonance imaging (MRI)to better characterise lesion relationships.Aswe candeduce from Fig. 1(c), itseemsa case ofHCC onnormal liver, which is infrequent and presents severaldiagnostic pitfalls. HCCs are hypervascular tumours anddemonstrate a strong arterial enhancement [2]; for thisreason, the finding of a single esofitic mass that extensivelyinfiltrates liver parenchyma with a strong arterial enhance-ment should be addressed in the first instance as HCC; inFig. 1(b) and (c), we can observe an arterial acquisition.Nevertheless, the tumour does not seem to present a strongarterial enhancement; this could be related to the extensiveintra-tumoral necrosis. However, we wonder if the authorshave used a bolus-tracking technique to obtain a correctarterial phase and also which acquisition delay has beenapplied?In Fig. 1(c), it also seems to appreciate a perihepaticeffusion without significant pleural effusion, which is aninfrequent finding for an extensive intrathoracic tumour.By our experience, we do not think that MRI couldbe useful to better define, in this case, tumour originand relationships. This is essentially because MRI has a lowerspatial resolution (3-mm slice thickness using volumetricsequences) compared with multidetector computed tomo-graphy (MDCT) (0.6-mm slice thickness) [3]. The realadvantages of MRI in characterisation of a liver lesion arerepresented by its intrinsic contrast, which offers thepossibility to characterise the different components ofthe lesion (haemorrhage, fat and iron) [4] and also by thepossibility to administrate hepatobiliary-specific mediumcontrast, which permits to distinguish lesions from function-ing hepatocytes or Kupffer cells [5].We conclude that, in this case, MDCTrepresents the besttechnique in the attempt to characterise the lesion and itsspatial relationships, and to permit a complete oncologicalstaging with whole body examination.