Siderosis

BackgroundIn thalassemia major (TM), severe cardiac siderosis can be treated by continuous parenteral deferoxamine, but poor compliance, complications and deaths occur. Combined chelation therapy with deferiprone and deferoxamine is effective for moderate myocardial siderosis, but has not been prospectively examined in severe myocardial siderosis.MethodsT2* cardiovascular magnetic resonance (CMR) was performed in 167 TM patients receiving standard subcutaneous deferoxamine monotherapy, and 22 had severe myocardial siderosis (T2* ResultsAt baseline, deferoxamine was prescribed at 38 +/- 10.2 mg/kg for 5.3 days/week, and deferiprone at 73.9 +/- 4.0 mg/kg/day. All patients continued both deferiprone and deferoxamine for 12 months. There were no deaths or new cardiovascular complications. The myocardial T2* improved (5.7 +/- 0.98 ms to 7.9 +/- 2.47 ms; p = 0.010), with concomitant improvement in LV ejection fraction (51.2 +/- 10.9% to 65.6 +/- 6.7%; p ConclusionIn patients with severe myocardial siderosis and impaired LV function, combined chelation therapy with subcutaneous deferoxamine and oral deferiprone reduces myocardial iron and improves cardiac function. This treatment is considerably less onerous for the patient than conventional high dose continuous subcutaneous or intravenous deferoxamine monotherapy, and may be considered as an alternative. Very prolonged tailored treatment with iron chelation is necessary to clear myocardial iron, and alterations in chelation must be guided by repeated myocardial T2* scans.Trial registrationThis trial is registered as NCT00103753.

PurposeThe purpose of this paper is to provide a meaningful literature review about the epidemiology, pathogenesis, clinical signs, imaging and treatment of ocular siderosis (OS).MethodsA computerized search from inception up to March 2020 of the online electronic database PubMed was performed using the following search strings: "ocular siderosis" and "siderosis bulbi". The reference list in each article was analysed for additional relevant publications.ResultsOS is an uncommon cause of visual loss due to a retained ferrous intraocular foreign body (IOFB). It may develop from 18 days to years after a penetrating trauma that usually occurs during hammering. On average, patients are 22-25 years old, and the vast majority are male. The most common cause of OS development is delayed presentation by the patient or missed diagnosis of IOFB after trauma. The pathophysiology is not fully understood; nevertheless, iron deposition causes hydroxyl radical formation, which damages photoreceptors and retinal pigment epithelium. Moreover, iron damages retinal vessels with consequent inner retinal layers degeneration. The most frequent signs are iris heterochromia, pupillary mydriasis, cataract development and retinal arteriolar narrowing with pigmentary retinal degeneration. Electroretinogram signs, in particular, b-wave amplitude reduction, arise earlier than clinical signs. Orbital CT scans and ultrasonography play an essential role in detecting IOFBs. Treatment depends on the IOFB location and OS development. However, it is crucial to remove the IOFB after OS development because visual acuity and clinical signs may improve. Anterior segment IOFBs can be dislodged using an intraocular magnet (IOM) or forceps through limbal paracentesis. In contrast, posterior segment IOFBs require a pars plana vitrectomy and IOM or forceps to be removed through an enlarged sclerotomy or the limbus.ConclusionRecommending the usage of protective glasses and spreading knowledge about OS may further benefit patient care.

BackgroundMyocardial black blood (BB) T2* relaxometry at 1.5T provides robust, reproducible and calibrated non-invasive assessment of cardiac iron burden. In vitro data has shown that like T2*, novel native Modified Look-Locker Inversion recovery (MOLLI) T1 shortens with increasing tissue iron. The relative merits of T1 and T2* are largely unexplored. We compared the established 1.5T BB T2* technique against native T1 values at 1.5T and 3T in iron overload patients and in normal volunteers.MethodsA total of 73 subjects (42 male) were recruited, comprising 20 healthy volunteers (controls) and 53 patients (thalassemia major 22, sickle cell disease 9, hereditary hemochromatosis 9, other iron overload conditions 13). Single mid-ventricular short axis slices were acquired for BB T2* at 1.5T and MOLLI T1 quantification at 1.5T and 3T.ResultsIn healthy volunteers, median T1 was 1014 ms (full range 939-1059 ms) at 1.5T and modestly increased to 1165ms (full range 1056-1224 ms) at 3T. All patients with significant cardiac iron overload (1.5T T2* values ConclusionsT1 mapping at 1.5T and at 3T can identify individuals with significant iron loading as defined by the current gold standard T2* at 1.5T. However, there is significant scatter between results which may reflect measurement error, but it is also possible that T1 interacts with T2*, or is differentially sensitive to aspects of iron chemistry or other biology. Hurdles to clinical implementation of T1 include the lack of calibration against human myocardial iron concentration, no demonstrated relation to cardiac outcomes, and variation in absolute T1 values between scanners, which makes inter-centre comparisons difficult. The relative merits of T1 at 3T versus T2* at 3T require further consideration.

The role of inflammation in cortical superficial siderosis (cSS), a marker of cerebral amyloid angiopathy (CAA) linked to high hemorrhage risk, is unclear. We examined 15 patients with cSS using 3 T post-contrast vessel wall MRI (VWI) and CSF analysis. Although only 27% met current CAA-ri criteria, 93% showed vessel wall enhancement or sulcal hyperintensities near cSS, frequently extending beyond. Seven patients with follow-up VWI demonstrated corticosteroid-responsive regression of inflammation. CSF albumin quotients, indicating blood-brain barrier dysfunction, correlated with MRI inflammation scores. These findings reveal subclinical meningovascular inflammation in cSS and support VWI for detecting a broader CAA-related inflammation spectrum.