Disease relevance of Siderosis

• The pedigree study described here offers support for the hypothesis that a single allele for HLA-linked hereditary hemochromatosis is responsible for the hepatic siderosis in sporadic porphyria cutanea tarda [1].
• Fetal liver disease may precede extrahepatic siderosis in neonatal hemochromatosis [2].
• Deferiprone monotherapy was significantly more effective than deferoxamine over 1 year in improving asymptomatic myocardial siderosis in beta-thalassemia major [3].
• It is concluded: that chemical measurement of hepatic iron concentration, when corrected for the age of the subject, reliably distinguishes early hemochromatosis from alcoholic siderosis, and, that there appears to be a threshold level of hepatic iron above which there is a high risk of fibrosis [4].
• The younger daughter had vitreal deposits, sensorimotor polyneuropathy, and acute sensorineural hearing but no evidence of siderosis on cranial MRI [5].

High impact information on Siderosis

• Constant subcutaneous deferoxamine administration may prove to be an effective and practical means of eliminating large quantities of iron in siderosis [6].
• 4-Dimethylaminoazobenzene was fed to Wistar-derived, male, albino rats after hepatic siderosis had been induced by including ferric citrate in the diet [7].
• These findings indicate that in some cases of neonatal hemochromatosis, extrahepatic siderosis may be caused by hepatic injury rather than primarily due to excessive transport of iron from mother to fetus and support speculation that in some instances an infective agent may be responsible [2].
• Subnormal activity of hepatic uroporphyrinogen decarboxylase is responsible for the derangement of porphyrin biosynthesis in both sporadic and familial porphyria cutanea tarda, but the enzymatic defect is not clinically expressed in the absence of hepatic siderosis [1].
• Heterozygosity for HLA-linked hemochromatosis as a likely cause of the hepatic siderosis associated with sporadic porphyria cutanea tarda [1].

Chemical compound and disease context of Siderosis

• The primary end point was the change in myocardial siderosis (myocardial T2(*)) over 1 year in patients maintained on subcutaneous deferoxamine or those switched to oral deferiprone monotherapy [3].
• Hepatocellular altered foci were induced in rat liver by cycles of feeding of N-2-fluorenylacetamide and were distinguished by their resistance to iron accumulation following production of hepatic siderosis by dietary administration of 8-hydroxyquinoline and ferrous gluconate [8].
• At lower, predominantly hepatocellular liver siderosis, the breath ethane exhalation was dependent on dietary vitamin E (VitE) supplements (onset of ethane exhalation at liver-Fe > 2 mg/g w wt on vitE-restricted diet; > 5 mg Fe per gram on VitE-replete diet) [9].
• Uroporphyria in the uroporphyrinogen decarboxylase-deficient mouse: Interplay with siderosis and polychlorinated biphenyl exposure [10].
• Steroid responsiveness of the associated pulmonary siderosis suggests that it had an immune basis [11].

Biological context of Siderosis

• Most heterozygotes for URO-D mutations do not express a porphyric phenotype unless hepatic siderosis is present [12].
• Multivariate analysis showed that TNF-alpha polymorphism was independently associated with ALT values (P =.0008 and P =.045, respectively, in homozygotes and nonhomozygotes for the C282Y mutation) and siderosis in nonhomozygotes (P =.047) [13].
• In CH-C, hepatic siderosis and lipid peroxidation are improved with successful IFN treatment [14].
• Studies on Bantu siderosis, hemochromatosis and other overload pathologies also provide insight into the intake and storage of this metal [15].
• OBJECTIVE : To identify the most appropriate testing strategy for genetic haemochromatosis in a liver clinic population by determining the ethnic distribution of the HFE mutations and the relationship between serum iron markers, hepatic siderosis and HFE genotype [16].

Anatomical context of Siderosis

• Central nervous system superficial siderosis, headache, and epilepsy [17].
• We suggest that the increased capacity of transferrin deficient in sialic acid to selectively deposit iron in the hepatocyte may be of significance for the development of the hepatic siderosis observed in alcoholism [18].
• The effect of ethanol on the uptake, binding, and desialylation of transferrin by rat liver endothelium: implications in the pathogenesis of alcohol-associated hepatic siderosis [19].
• 3'-azido-3'-deoxythymidine (AZT), the first chemotherapeutic drug approved by FDA for treatment of HIV-infected patients and still used in combination therapy, has been shown to induce, upon prolonged exposure, severe bone marrow toxicity manifested as anemia, neutropenia and siderosis [20].
• Lymph node siderosis in trypan blue treated rats [21].

Gene context of Siderosis

• There was no correlation between the duodenal expression of DMT1 and Ireg1 and the degree of hepatic siderosis [22].
• Among patients with hepatic iron overload, the distinction between hereditary hemochromatosis (HH), a common yet treatable genetic disease, and other causes of siderosis remains problematic [23].
• Porphyria cutanea tarda (PCT), a disorder characterized by a photosensitive dermatosis and hepatic siderosis, is caused by a decreased activity of the hepatic enzyme uroporphyrinogen decarboxylase (UROD) [24].
• This indicates that, as already suggested by our previous data in experimental siderosis, iron maintains the ability to induce transferrin gene activity even when cellular iron content is significantly increased [25].
• Regulation of hepatic transferrin, transferrin receptor and ferritin genes in human siderosis [25].

Analytical, diagnostic and therapeutic context of Siderosis

• Rapid development of hepatocellular siderosis after liver transplantation for neonatal hemochromatosis [26].
• We discuss the possibility that hemochromatosis recurred in the liver allograft and review possible factors contributing to the siderosis [26].
• BACKGROUND: Cardiac siderosis from transfused iron remains the major cause of death in thalassaemia major, despite iron chelation therapy with desferrioxamine [27].

References