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Iron overload (IOL) is a frequently reported complication following hematopoietic stem cell transplantation (HSCT) that has been investigated extensively in the field of hemoglobinopathies but has not been thoroughly characterized after HSCT in pediatric malignancies. Our aim was to assess prevalence, severity, risk factors, and management of IOL, as defined using biochemical (serum ferritin) and radiologic tools (T2*-weighted magnetic resonance imaging [MRI]), in a cohort of pediatric patients who underwent HSCT for either malignant or benign diseases. This monocentric, retrospective, observational study included all the 163 patients alive and in continuous remission at 24 months post-HSCT out of the 219 consecutive children and adolescents who underwent HSCT at our institution between 2012 and 2018, were included in the study. IOL was classified into 4 categories: absent, mild, moderate, and severe. Among the 163 patients, 73% had some degree of IOL (mild in 37%, moderate in 29%, and severe in 7%). Moderate/severe IOL was more frequent among patients diagnosed with a malignant disease versus those with a benign disease (43% versus 19%; P =.0065). Trend lines for serum ferritin showed a “bell-shaped” distribution, with the highest levels recorded during the first 6 months post-HSCT, followed by a spontaneous reduction. Both pre-HSCT (1659 ng/mL versus 617 ng/mL; P

Endometriosis can be associated with adverse pregnancy outcomes. We report six cases of endometriosis-related spontaneous hemoperitoneum diagnosed in pregnant and postpartum women over 13 years. Spontaneous hemoperitoneum in pregnancy mainly occurred in the second half of gestation. All women presented with acute abdominal pain; four of them needed an emergent surgery, two were managed expectantly. The median estimated blood loss was 4250 ml, four women required massive transfusion. Three out of six women had a known history of endometriosis, all of them had histologically confirmed endometriosis after surgery. No maternal or perinatal deaths occurred. In one case, reticence to perform a computed tomography scan led to delayed diagnosis. Since delay can lead to lethal consequences, high levels of suspicion for spontaneous hemoperitoneum should be maintained in cases of severe abdominal pain, even with a woman's negative history of endometriosis. Improved knowledge and regular interdisciplinary meetings are pivotal to ameliorate outcomes.

Background & Aims: Inhalation of welding fume may cause pulmonary disease known as welder's lung. At our centre we came across a number of welders with systemic iron overload and prolonged occupational history and we aimed at characterizing this novel clinical form of iron overload. Methods: After exclusion of other known causes of iron overload, 20 welders were fully evaluated for working history, hepatic, metabolic and iron status. MRI iron assessment was performed in 19 patients and liver biopsy in 12. We included 40 HFE-HH patients and 24 healthy controls for comparison. Results: 75% of patients showed lung HRCT alterations; 90% had s-FERR > 1000 ng/mL and 60% had TSAT > 45%. Liver iron overload was mild in 8 and moderate-severe in 12. The median iron removed was 7.8 g. Welders showed significantly lower TSAT and higher SIS and SIS/TIS ratio than HFE-HH patients. Serum hepcidin was significantly higher in welders than in HFE-HH patients and healthy controls. At liver biopsy, 50% showed liver fibrosis that was mild in four, and moderate-severe in two. Liver staging correlated with liver iron overload. Conclusions: Welders with prolonged fume exposure can develop severe liver iron overload. The mechanism of liver iron accumulation is quite different to that of HFE-HH suggesting that reticuloendothelial cells may be the initial site of deposition. We recommend routine measurement of serum iron indices in welders to provide adequate diagnosis and therapy, and the inclusion of prolonged welding fume exposure in the list of acquired causes of hyperferritinemia and iron overload.

Rationale: In the exploratory Phase II STEM-AMI (Stem Cells Mobilization in Acute Myocardial Infarction) trial, we reported that early administration of G-CSF (granulocyte colony-stimulating factor), in patients with anterior ST-segment–elevation myocardial infarction and left ventricular (LV) dysfunction after successful percutaneous coronary intervention, had the potential to significantly attenuate LV adverse remodeling in the long-term. Objective: The STEM-AMI OUTCOME CMR (Stem Cells Mobilization in Acute Myocardial Infarction Outcome Cardiac Magnetic Resonance) Substudy was adequately powered to evaluate, in a population showing LV ejection fraction ≤45% after percutaneous coronary intervention for extensive ST-segment–elevation myocardial infarction, the effects of early administration of G-CSF in terms of LV remodeling and function, infarct size assessed by late gadolinium enhancement, and myocardial strain. Methods and Results: Within the Italian, multicenter, prospective, randomized, Phase III STEM-AMI OUTCOME trial, 161 ST-segment–elevation myocardial infarction patients were enrolled in the CMR Substudy and assigned to standard of care (SOC) plus G-CSF or SOC alone. In 119 patients (61 G-CSF and 58 SOC, respectively), CMR was available at baseline and 6-month follow-up. Paired imaging data were independently analyzed by 2 blinded experts in a core CMR lab. The 2 groups were similar for clinical characteristics, cardiovascular risk factors, and pharmacological treatment, except for a trend towards a larger infarct size and longer symptom-to-balloon time in G-CSF patients. ANCOVA showed that the improvement of LV ejection fraction from baseline to 6 months was 5.1% higher in G-CSF patients versus SOC ( P =0.01); concurrently, there was a significant between-group difference of 6.7 mL/m 2 in the change of indexed LV end-systolic volume in favor of G-CSF group ( P =0.02). Indexed late gadolinium enhancement significantly decreased in G-CSF group only ( P =0.04). Moreover, over time improvement of global longitudinal strain was 2.4% higher in G-CSF patients versus SOC ( P =0.04). Global circumferential strain significantly improved in G-CSF group only ( P =0.006). Conclusions: Early administration of G-CSF exerted a beneficial effect on top of SOC in patients with LV dysfunction after extensive ST-segment–elevation myocardial infarction in terms of global systolic function, adverse remodeling, scar size, and myocardial strain. Clinical Trial Registration: URL: https://www.clinicaltrials.gov . Unique identifier: NCT01969890.

Purpose: To evaluate the image quality and radiation dose exposure of low-dose coronary CTA (cCTA) study, reconstructed with the new model-based iterative reconstruction algorithm (IMR), compared with standard hybrid-iterative reconstruction (iDose4) cCTA in patients with suspected coronary artery disease. Materials and methods: Ninety-eight patients with an indication for coronary CT study were prospectively enrolled. Fifty-two patients (study group) underwent 256-MDCT low-dose cCTA (80 kV; automated-mAs; 60 mL of CM, 350 mgL/mL) with prospective ECG-triggering acquisition and IMR. A control group of 46 patients underwent 256-MDCT standard prospective ECG-gated protocol (100 kV; automated-mAs; 70 mL of CM, 400 mgL/mL; iDose4). Subjective and objective image quality (attenuation value, SD, SNR and CNR) were evaluated by two radiologists subjectively. Radiation dose exposure was quantified as DLP, CTDIvol and ED. Results: Mean values of mAs were significantly lower for IMR-cCTA (167 ± 62 mAs) compared to iDose-cCTA (278 ± 55 mAs), p < 0.001. With a significant reduction of 38% in radiation dose exposure (DLP: IMR-cCTA 91.7 ± 26 mGy cm vs. iDose-cCTA 148.6 ± 35 mGy cm; p value < 0.001), despite the use of different CM, we found higher mean attenuation values of the coronary arteries in IMR group compared to iDose4 (mean density in LAD: 491HU IMR-cCTA vs. 443HU iDose-cCTA; p = 0.03). We observed a significant higher value of SNR and CNR in study group due to a lower noise level. Qualitative analysis did not reveal any significant differences between the two groups (p = 0.23). Conclusions: Low-dose cCTA study combined with IMR reconstruction allows to correctly evaluate coronary arteries disease, offering high-quality images and significant radiation dose exposure reduction (38%), as compared to standard cCTA protocol.

Objective. Hyperferritinemia is frequent in chronic liver diseases of any cause, but the extent to which ferritin truly reflects iron stores is variable. In these patients, both liver iron and fat are found in variable amount and association. Liver biopsy is often required to quantify liver fat and iron, but sampling variability and invasiveness limit its use. We aimed to assess single breath-hold multiecho magnetic resonance imaging (MRI) for the simultaneous lipid and iron quantification in patients with hyperferritinemia. Material and methods. We compared MRI results for both iron and fat with their respective gold standards - liver iron concentration and computer-assisted image analysis for steatosis on biopsy. We prospectively studied 67 patients with hyperferritinemia and other 10 consecutive patients were used for validation. We estimated two linear calibration equations for the prediction of iron and fat based on MRI. The agreement between MRI and biopsy was evaluated. Results. MRI showed good performances in both the training and validation samples. MRI information was almost completely in line with that obtained from liver biopsy. Conclusion. Single breath-hold multiecho MRI is an accurate method to obtain a valuable measure of both liver iron and steatosis in patients with hyperferritinemia.

Objective To assess whether granulocyte colony-stimulating factor (G-CSF) treatment induces a sustained benefit on adverse remodelling in patients with large anterior ST-elevation myocardial infarction (STEMI) and left ventricular (LV) dysfunction after successful reperfusion. Methods The STEM-AMI Trial was a prospective, placebo-controlled, multicentre study. Sixty consecutive patients with a first anterior STEMI, who underwent primary percutaneous coronary intervention 2–12 h after symptom onset, with LV ejection fraction (LVEF) ≤45% measured by echocardiography within 12 h after successful revascularisation (TIMI flow score ≥2), were randomised 1:1 to G-CSF (5 µg/Kg body weight b.i.d . ) or placebo. Clinical events and Major Adverse Cardiac and Cerebrovascular Event (MACCE) were monitored, and LVEF, LV end-diastolic (LVEDV) and end-systolic (LVESV) volumes, and infarct size were evaluated by MRI at the final 3-year follow-up. Results Fifty-four patients completed the study, of whom 35 with MRI. No significant differences were found in mortality and MACCE between G-CSF and placebo-treated groups. The 3-year infarct size was not different between groups, whereas LVEDV was significantly lower in G-CSF (n=20) than in placebo (n=15) patients (170.1±8.1 vs 197.2±8.9 mL, respectively; p=0.033 at analysis of covariance). A significant inverse correlation was detected in G-CSF patients between the number of circulating CD34 cells at 30 days after reperfusion and the 3-year absolute and indexed LVEDV (ρ=−0.71, 95% CI −0.90 to −0.30, and ρ=−0.62, −0.86 to −0.14, respectively), or their change over time (r=−0.59, −0.85 to −0.11, and r=−0.55, −0.83 to −0.06, respectively). Conclusions G-CSF therapy may be beneficial in attenuating ventricular remodelling subsequent to a large anterior STEMI in the long term. No differences have been detected in clinical outcome.