|1.||beta-Thalassemia (Cooley's Anemia)
|2.||Sensorineural Hearing Loss
|4.||Cardiac Arrhythmias (Arrythmia)
|5.||Syringomyelia (Morvan's Disease)
|1.||Pennell, Dudley J: 14 articles (03/2015 - 05/2006)|
|2.||Porter, John B: 7 articles (06/2015 - 03/2010)|
|3.||Aydinok, Yesim: 6 articles (06/2015 - 03/2010)|
|4.||Cappellini, Maria Domenica: 6 articles (11/2014 - 02/2002)|
|5.||Galanello, Renzo: 6 articles (06/2013 - 05/2006)|
|6.||Habr, Dany: 5 articles (06/2015 - 03/2010)|
|7.||Kattamis, Antonis: 5 articles (06/2015 - 01/2005)|
|8.||El-Beshlawy, Amal: 5 articles (06/2015 - 03/2010)|
|9.||Piga, Antonio: 5 articles (02/2015 - 05/2006)|
|10.||Musallam, Khaled M: 5 articles (02/2015 - 01/2011)|
10/01/2010 - "Patients with mild to moderate liver siderosis improved their cardiac T2* while more severely siderotic patients did not, regardless of initial cardiac iron burden. "
11/01/1990 - "Since there were no statistical correlations between the degree of hepatic siderosis and porphyrinuria, and since we repeatedly found remission of the PCT in spite of the continued existence of hepatic siderosis, the removal of iron cannot be regarded as the only principle of action in CQ therapy. "
08/01/2013 - "We looked at the change in cardiac T2*, liver iron concentration (LIC) and left ventricular ejection fraction (LVEF) at years 3 and 5. In patients with severe cardiac siderosis, cardiac T2* (mean ± SD) improved from 6.9 ± 1.6 at baseline to 13.6 ± 10.0 by year 5, mean ΔT2* = 6.7 (P = 0.04). "
08/01/2010 - "Recent technologic advances in the documentation of organ-specific siderosis and the improved efficiency of iron chelating programs resulted in a spectacular improvement in the prevention of iron-induced end-organ failure and improved survival in thalassemic patients."
12/01/2007 - "In several studies the MRI parameters of liver siderosis have failed to correlate with those of other affected organs, underlining the necessity for MRI iron evaluation in individual organs. "
01/01/2009 - "Therapy combining deferiprone with DFO has proved effective in the management of severe cardiac siderosis. "
01/01/2013 - "Further studies are required to assess the clinical efficacy of deferiprone in superficial siderosis. "
01/01/2012 - "Prospectively designed efficacy studies are necessary to determine the clinical efficacy of deferiprone in superficial siderosis."
01/01/2012 - "Pilot safety trial of deferiprone in 10 subjects with superficial siderosis."
01/01/2013 - "The proposed deferiprone-based therapy has provided a paradigm for treating regional types of siderosis without affecting hematological parameters and systemic functions. "
09/01/1995 - "Electron microscopic studies of unstained sections showed early parenchymal cell siderosis, with accumulation of numerous ferritin particles and clusters in the cytosol, as well as ferritin and haemosiderin in lysosomes (siderosomes). "
10/01/2015 - "Renal siderosis and serum ferritin."
03/01/2015 - "Serum ferritin and CMR were performed to assess the cardiac siderosis (T2* < 20 ms). "
03/01/2014 - "In this group of severe cardiac siderosis, T2* was also not correlated to age (P = 0.5), LVEF (P = 0.14), LIC (P = 0.97), or serum ferritin (P = 0.82). "
03/01/2014 - "The mean serum ferritin was 4,510 ± 2,847 ng/ml (533 to 22,360 ng/ml), and in 83.2 %, the serum ferritin was >2,500 ng/ml. The prevalence of myocardial siderosis (T2* of <20 ms) was 24.7 % (mean age 20.9 ± 7.5 years), with mean T2* of 12.7 ± 4.4 ms, mean LVEF of 68.6 ±5.8 %, mean LIC of 30.9 ± 13 mg Fe/g dw, and median serum ferritin of 4,996 ng/ml. There was no correlation between T2* and age, LVEF, LIC, and serum ferritin (P = 0.65, P = 0.085, P = 0.99, and P = 0.63, respectively). "
|4.||Glucose (Dextrose)FDA LinkGeneric
01/01/2010 - "Significant reduction in signal intensity ratio (SIR) of the liver and the pancreas was shown in thalassemic patients compared to controls (P < 0.0001), Thalassemic patients with abnormal glucose tolerance; including diabetics and thalassemics with impaired glucose tolerance; displayed a higher degree of pancreatic and hepatic siderosis compared to thalassemics with normal glucose tolerance or controls (P < 0.001, P < 0.0001). "
01/01/2010 - "pancreatic siderosis can be detected by T2* gradient-echo MRI since childhood in thalassemic patients, and is more evident in patients with abnormal glucose tolerance. "
06/01/2007 - "The study aims at describing the MR features of pancreas in beta-thalassemia major, investigating the relations between MR findings and glucose disturbances and between hepatic and pancreatic siderosis. "
01/01/2013 - "All patients showed significant reduction in the signal intensity of the liver and the pancreas on T2(∗)GRD compared to controls, thalassemic patients who had abnormal glucose tolerance; diabetic and impaired glucose tolerance patients displayed a higher degree of pancreatic and hepatic siderosis and more T2(∗) drop in their signal intensity than those with normal blood sugar level. "
03/01/2010 - "Iron accumulation predominantly in hepatocyes was associated with a 1.7-fold higher risk of a fibrosis stage greater than 1 (95% confidence interval [CI]: 1.2-2.3), compared with the absence of siderosis (after adjustment for age, body mass index, glucose tolerance status, and alanine aminotransferase level). "
|5.||Deferoxamine (Desferal)FDA LinkGeneric
06/18/2015 - "HYPERION, an open-label single-arm prospective phase 2 study, evaluated combination DFX-deferoxamine (DFO) in patients with severe transfusional myocardial siderosis (myocardial [m] T2* 5-<10 ms; left ventricular ejection fraction [LVEF] ≥56%) followed by optional switch to DFX monotherapy when achieving mT2* >10 ms. Mean dose was 30.5 mg/kg per day DFX and 36.3 mg/kg per day DFO on a 5-day regimen. "
01/01/1965 - "[LATEST CONTRIBUTION TO THE STUDY OF THE USE OF DESFERRIOXAMINE B AS A TEST FOR DETERMINATION OF STATES OF SIDEROSIS]."
01/01/2008 - "T2* cardiovascular magnetic resonance (CMR) was performed in 167 TM patients receiving standard subcutaneous deferoxamine monotherapy, and 22 had severe myocardial siderosis (T2* < 8 ms) with impaired left ventricular (LV) function. "
08/23/2002 - "However, complete regression of organ siderosis was achieved by deferoxamine administration. "
01/01/1987 - "Thus, desferrioxamine B may add to the risk of systemic yersiniosis developing in patients with siderosis. "
05/01/2001 - "Magnetic resonance imaging studies revealed hemosiderin deposition, characteristic of superficial siderosis, and multiple cavernous malformations in all three cases. "
10/16/2014 - "Diffuse superficial siderosis of the central nervous system (CNS) is a rare condition due to hemosiderin deposits in the subpial layers of the brain and spinal cord. "
07/01/2013 - "Superficial siderosis (SS) is a rare condition in which hemosiderin is deposited on the pial surface of the brain and/or spinal cord. "
09/01/2012 - "His serial magnetic resonance imaging (MRIs) showed a progressive deposition of hemosiderin in the cerebellar folia and around the brainstem, confirming a diagnosis of superficial siderosis. "
09/01/2012 - "Superficial siderosis of the central nervous system results from deposition of hemosiderin in the subpial layers of the brain and spinal cord. "
|7.||Isoflurophate (DFP)FDA Link
05/01/2007 - "Clinical studies using this method indicated superiority of DFP compared to DFO with respect to the treatment of myocardial siderosis. "
01/01/2009 - "In retrospective and prospective studies, DFP monotherapy was significantly more effective than DFO in the treatment of myocardial siderosis in thalassemia major. "
01/01/2005 - "Long-term combined therapy with DFO with DFP results in considerable reduction of both ferritin levels and liver iron concentration as well as significant improvement in cardiac siderosis and function. "
|8.||Interferon-alpha (Interferon Alfa)FDA Link
|9.||Biological Markers (Surrogate Marker)IBA
10/15/2008 - "Here we present the first report of a patient with superficial CNS siderosis in whom cerebrospinal fluid biomarkers of brain damage were assessed. "
07/01/2014 - "CSF biomarkers in superficial siderosis: a new tool for diagnosis and evaluation of therapeutic efficacy of deferiprone--a case report."
01/01/2012 - "The biomarkers include (1) greater occipital uptake on amyloid positron emission tomography imaging and a decrease of cerebrospinal fluid Aβ40 levels as markers suggestive of CAA, and (2) symptomatic lobar ICH, lobar microhemorrhages, focal subarachnoidal hemorrhages/superficial siderosis, cortical microinfarcts, and subacute encephalopathy (caused by CAA-related inflammation or angiitis) as imaging findings of CAA-related ICH and other disorders. "
|10.||tau Proteins (tau Protein)IBA
|1.||Chelation Therapy (Therapy, Chelation)
01/01/2008 - "Combined chelation therapy in thalassemia major for the treatment of severe myocardial siderosis with left ventricular dysfunction."
01/01/2014 - "We review the high quality randomized controlled trials in this area for iron chelation therapy in the management of cardiac siderosis. "
01/01/2013 - "Regional siderosis: a new challenge for iron chelation therapy."
01/01/2013 - "Treatment of superficial siderosis with iron chelation therapy."
01/01/2013 - "We report a patient who developed superficial siderosis following neurosurgery for syringomyelia and who had an improvement in his hearing and mobility following treatment with a new iron chelation therapy that can penetrate the blood-brain barrier. "
12/01/2014 - "The purpose of this study is to report an additional case of cochlear implantation in a patient with superficial siderosis and to contribute to the current literature available on outcomes of implantation in these individuals. "
09/01/2010 - "The purpose of this study is to present follow-up on a previously reported case of successful cochlear implantation in a patient with superficial siderosis. "
08/01/2015 - "To date, there have been less than 30 cases of cochlear implantation (CI) in patients with superficial siderosis (SS) reported in the literature. "
08/01/2015 - "Cochlear Implantation in Patients With Superficial Siderosis: Seven Cases and Systematic Review of the Literature."
12/01/2014 - "Rapid deterioration in hearing thresholds and speech discrimination before implantation may be an indicator of an actively progressing case of superficial siderosis, which may suggest possible limited benefit from cochlear implantation."
|3.||Gastric Bypass (Roux-en-Y Gastric Bypass)
09/01/1975 - "Gastrojejunostomy inhibits postshunt siderosis."
09/01/1975 - "Thus continued iron absorption appears to account for the development of postshunt siderosis and the addition of gastrojejunostomy ameliorates postshunt siderosis by diminishing iron absorption."
09/01/1975 - "Thirteen of fourteen dogs developed marked increases in hepatic hemosiderin (postshunt siderosis) after construction of portacaval shunt, whereas only three of 12 dogs developed such siderosis after construction of a portacaval shunt and end-to-side gastrojejunostomy with duodenal exclusion. "
|5.||Cochlear Implants (Cochlear Implant)
12/01/2012 - "Only 10 studies have described the use of cochlear implants in superficial siderosis and results have been variable. "
12/01/2014 - "One cochlear implant user with superficial siderosis in a tertiary referral center. "
05/01/2012 - "Programming peculiarities in two cochlear implant users with superficial siderosis of the central nervous system."
09/01/2010 - "Benefit from cochlear implants in patients with superficial siderosis is variable and may not be long standing."
06/01/2009 - "Cochlear implant patients with superficial siderosis."