the new generation magnetic iron detector to measure the iron overload in the human liver

Abstract Background: The knowledge of liver iron overload is essential for the diagnosis and the therapy of diseases which induce systemic iron overload in the body. The liver needle biopsy is a well-known invasiv

SARS-CoV-2 infection in beta thalassemia: Preliminary data from the Italian experience

Patients with pre\u2010existent chronic morbidities are likely to be more severely affected by SARS\u2010Cov2 infection, but no data are available regarding Thalassemic Syndromes (TS). Note, TS and hemoglobin variants represent, according to WHO, one of the most frequent causes of anemia, affecting more than 7% of the world population.1 Thalassemic Syndromes are classified in either transfusion\u2010dependent thalassemia (TDT) or non\u2010transfusion\u2010dependent thalassemia (NTDT). Infectious complications, mainly from bacteria, constitute a common cause of mortality and morbidity in TS. Stress erythropoiesis, iron overload, splenectomy and adrenal insufficiency among others may contribute to increase susceptibility to infection.2 To verify the impact of SARS\u2010CoV\u20102 infection on TS, we set\u2010up a specific survey by electronic Case Report Form (eCRF).3 Inclusion criteria require at least 15\u2009days of follow\u2010up from either the onset of symptoms or SARS\u2010CoV2 positivity. The survey was approved by Ethics Committee and eCRF was shared with the Centers of Italian Hemoglobinopathies Network. The \u201cSociet\ue0 Italiana Talassemie ed Emoglobinopatie\u201d (SITE), has estimated the presence in Italy of approximately 5000 TDT and 1900 NTDT patients.3 As of 10 April 2020, 11 cases of TS and COVID\u201019 have been collected (see supplementary information). All the reported patients are in Northern Italy, where the rate of infection is higher, reflecting the national epidemiology. The mean age is 44\u2009\ub1\u200911\u2009years (range 31\u201061\u2009years) and 55% (6/11) are females. Ten patients are TDT, and one is NTDT. All the patients have thalassemia associated comorbidities, eight are splenectomized, and one patient (#9 in the supplementary table) has pulmonary hypertension treated with sildenafil. The likely source of infection has been detected in 55% (6/11) of cases: two had contacts with COVID\u201019 positive subjects, and four had occupational exposure (three are nurses working in hospital or assisted living facilities). Three patients were asymptomatic. One patient (#3 in supplementary information) was admitted for high fever and bone marrow hypoplasia, lymphopenia, and agranulocytosis (on treatment with deferiprone) and tested positive at the third swab. Six out of 11 were hospitalized, but no one required mechanical ventilation. The patient with more severe symptoms who required more intensive ventilation support with continuous positive airway pressure (CPAP) has a history of diffuse large B\u2010cell lymphoma, treated with chemotherapy in the previous year, currently in complete remission. Of the six people admitted to the hospital, only three received supposedly specific treatment for COVID\u201019: one hydroxychloroquine (HCQ), one HCQ plus ritonavir/darunavir, and one HCQ plus anakinra. Patient #3 did not receive HCQ due to concomitant therapy with amiodarone and an increased risk of life\u2010threatening arrhythmia. The clinical course ranged from 10 to 29\u2009days. Ten patients have clinically recovered and are on a daily remote phone call follow\u2010up. Splenectomy which was present in 8/11 patients did not seem to affect the clinical course. Of note, except for the patient with myelosuppression, no increase in blood requirement was observed. When luspatercept treatment was halted in the NTDT patient, hemoglobin fell from 110 to 82 g/L, a value similar to the pre\u2010luspatercept period. Neither death nor severe SARS or signs of cytokines storm were observed in these 11 subjects, which may be surprising, taking into account the mean age and the presence of severe comorbidities. Our data, although preliminary, do not indicate increased severity of COVID\u201019 in TS. A larger number of cases needs to be collected to define the impact of this new infection and its outcome in these fragile patients

Safety and efficacy of ketorolac continuous infusion for multimodal analgesia of vaso-occlusive crisis in patients with sickle cell disease

Pain is an hallmark of sickle-cell-related acute clinical manifestations as part of acute vaso-occlusive crisis (VOC). In SCD pain has different origins such as vascular or neuropathic pain, which requires multimodal analgesia. This is based on the administration of drugs with different pharmacological mechanisms of action, maximizing analgesia and minimizing their adverse events and the risk of drug-addition in patients experiencing acute-recurrent pain events as in SCD. Ketorolac is a potent non-narcotic analgesic, being relatively safe and effective during pain-management in children and adults. Up to now, there is a lack of safety information on continuous infusion ketorolac as used to control acute pain in patients with SCD, and the benefits/risks ratio needs to be investigated. Here, we report for the first time the safety profile of ketorolac in the special population of patients with SCD. We confirmed that ketorolac in combination with tramadol, an opioid like molecule, is effective in pain control of adult patients with SCD experiencing acute severe VOCs defined by pain visual analog scale. Our study shows that short term (72 h) continuous infusion of ketorolac plus tramadol is not associated with adverse events such as liver or kidney acute disfunction or abnormalities in coagulation parameters during patients' hospitalization and within 30 days after patients discharge. This is extremely important for patients with SCD, who should have access to multimodal therapy to control recurrent acute pain crisis in order to limit central sensitization a fearsome issue of undertreated recurrent acute pain and of chronic pain

Vector valued regression for iron overload estimation

In this work we present and discuss in detail a novel vector-valued regression technique: our approach allows for an all-at-once estimation, as opposed to solve a number of scalar-valued regression tasks. Despite its general purpose nature, the method has been designed to solve a delicate medical issue: a reliable and noninvasive assessment of body-iron overload. The Magnetic Iron Detector (MID) measures the magnetic track of a person, which depends on the anthropometric characteristics and the body-iron burden. We aim to provide an estimate of this signal in absence of iron overload. We show how this question can be formulated as the estimation of a vector-valued function which encompasses the prior knowledge on the shape of the magnetic track. This is accomplished by designing an appropriate vector-valued feature map. We successfully applied the method on a dataset of 84 volunteers.

Lack of correlation between heart, liver and pancreas MRI-R2*: Results from long term follow-up in a cohort of adult \u3b2-thalassemia major patients

No available; Lette

Daily alternating deferasirox and deferiprone therapy successfully controls iron accumulation in untreatable transfusion dependent thalassemia patients

No abstract is available for this article. This article is protected by copyright. All rights reserved

Whole Liver Iron Overload Measurement by Magnetic Iron Detector (MID). A Non Cryogenic Bio-Susceptometer.

Accurate assessment of body-iron accumulation is essential for diagnosis and therapy of iron-overload in diseases such as thalassemia, hereditary hemochromatosis and other forms of severe congenital or acquired anemias. The susceptometer presented herein, named Magnetic Iron Detector (MID), measures the iron overload in the whole liver. In 2 patients, affected by Congenital Hemocromatosis, we correlated the LIC measurement by MID with the assessment of the expected iron depletion obtained with the phlebotomy therapy R 0.94 (Fig 2). All the measurements were correlated with the serum-ferritin concentration values R 0.72. We obtained correlation with the LIC measurement by liver biopsy in 7 patients R 0.89, further measures are in progress. . In conclusion the data obtained shows that MID is a reliable instrument for the diagnosis of the liver iron overload and for the follow-up of the chelation therapy. It is simpler to operate being manageable directly in the Clinical Center and more affordable than competing techniques