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Hyperintensity of the globus pallidus on T1-weighted magnetic resonance imaging (MRI) has been reported in patients with chronic liver disease. This abnormality has been associated with the severity of liver disease and tremor, but its cause is unknown. Similar MRI signal abnormalities have been reported in experimental models of manganese neurotoxicity.(More)
OBJECTIVE Previous reports suggest an association between inflammatory bowel disease (IBD) and immune thrombocytopenic purpura (ITP) in adults. To date, only five children with both diseases have been described. The aim of the study was to describe the characteristics of children with IBD and ITP. METHODS Cases were obtained from the pediatric(More)
Transferrin has been proposed as the mobilization protein for iron and manganese. To better understand the role of transferrin in the transport of these metals, we studied the tissue distribution of injected iron 59 and manganese 54 in the hypotransferrinemic (Hp) mouse mutant. The Hp mouse has a mutation in the transferrin gene and produces < 1% of normal(More)
The metals iron (Fe) and manganese (Mn) are essential for normal functioning of the brain. This review focuses on recent developments in the literature pertaining to Fe and Mn transport. These metals are treated together because they appear to share several transport mechanisms. In addition, several neurological diseases such as Alzheimer's Disease,(More)
Hypotransferrinemia (hpx/hpx) is a genetic defect in mice resulting in <1% of normal plasma transferrin (Tf) concentrations; heterozygotes for this mutation (+/hpx) have low circulating Tf concentrations. These mice provide a unique opportunity to examine the role of Tf in Fe and Mn transport in the brain. Twenty weanling wild-type BALB/cJ mice, 15 +/hpx(More)
Hyperintense symmetric pallidal lesions have been described in chronic hepatic failure. Similar lesions are reported in experimental models of manganese neurotoxicity. We describe an 8-year-old girl with chronic hepatic failure and dystonia in association with an elevated whole blood manganese level and symmetric hyperintense pallidal lesions on magnetic(More)
A 17-year-old male patient with a 3-year history of hidradenitis suppurativa presented to our dermatology clinic for more effective treatment. Areas of involvement included the medial aspect of the thighs, scrotum, buttocks, and inframammary folds. His axillae were spared. He also had a pilonidal cyst and nodulocystic acne. A diagnosis of hidradenitis(More)
The hyperintense signal in the globus pallidus of cirrhotic patients on T1-weighted magnetic resonance (MR) imaging has been postulated to arise from deposition of paramagnetic manganese2+ (Mn). Intestinal absorption of both iron and Mn are increased in iron deficiency; iron deficiency may therefore increase susceptibility to Mn neurotoxicity. To(More)
Hypotransferrinemia is a genetic defect in mice resultingin 1% of normal plasma transferrin (Tf) concen-trations;heterozygotes for thismutation (+/hpx) have low circulating Tf concentrations. These mice providea unique opportunity toexamine the developmental pattern and response of Tf to iron-deficient diets, andfurthermore,to address the controversial role(More)
Transferrin (Tf) is accepted as the iron mobilization protein, but its role in transport of other metals is controversial. In this study, we used mixed glial cultures from hypotransferrinemic (Hp) mice to determine the dependence of these cells on transferrin for iron and manganese delivery and release. Hp mice have a splicing defect in the transferrin (Tf)(More)