Spur cell anaemia and hepatic iron stores in patients with alcoholic liver disease undergoing orthotopic liver transplantation.
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BACKGROUND: Following orthotopic liver transplantation (OLT) histological examination of explant livers from patients with alcoholic liver disease (ALD) sometimes shows extensive iron deposits in a distribution suggestive of homozygous haemochromatosis. AIMS: To use haemochromatosis gene (HFE) assays to distinguish between ALD with notable siderosis and hereditary haemochromatosis. To evaluate the possible influence of spur cell haemolytic anaemia on hepatic iron loading. PATIENTS: Thirty seven patients with ALD were abstinent for at least six months prior to OLT. Twenty three patients had transferrin saturations greater than 55%, 16 also had increased serum ferritin (>350 micrograms/l). Eight of 37 (22%) explant livers had grade 3 or 4 hepatic iron deposition, predominantly in hepatocytes. Of these, four had a hepatic iron index greater than 1. 9 and most seemed to have spur cell haemolytic anaemia. METHODS: Mutation analysis for C282Y and H63D mutations was performed on DNA extracts from peripheral blood or explant liver. Spur cell haemolytic anaemia was diagnosed when the haemoglobin was 105 g/l in the presence of notable acanthocytosis. RESULTS: None of the eight patients with grade 3 or 4 hepatic iron had evidence of the C282Y mutation. Two of the eight were heterozygous for H63D. None of the remaining 28 patients tested showed homozygous HFE mutations. Spur cell anaemia was present in six of the eight patients with heavy iron deposition and only one of the remaining patients. CONCLUSIONS: The HFE mutation was not present in these patients with advanced ALD and heavy iron loading. Spur cell haemolytic anaemia provides an alternative potential mechanism for the heavy iron loading. (+info)
Superficial siderosis of the CNS associated with multiple cavernous malformations.
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Superficial siderosis of the CNS due to chronic, recurrent subarachnoid hemorrhage is an uncommon and potentially debilitating disorder. The classic clinical manifestation is progressive bilateral sensorineural hearing loss (SNHL), although ataxia and pyramidal signs also are observed frequently. Cavernous malformations rarely present with subarachnoid hemorrhage. We describe an unusual case of a young patient who presented with progressive, bilateral SNHL who was found to have superficial CNS siderosis associated with multiple cavernous malformations. (+info)
Increased hepatic iron deposition resulting from treatment of chronic hepatitis C with ribavirin.
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Increased levels of hepatic iron may impair the response of patients with chronic hepatitis C to treatment with interferon-alfa, but combination therapy with ribavirin has demonstrated efficacy in the treatment of hepatitis C. When used alone or with interferon-alfa, ribavirin may cause a dose-dependent reversible hemolytic anemia. We compared the extent and cellular localization of iron deposition in liver tissue from biopsy specimens obtained before and after 36 weeks of therapy with ribavirin or placebo for 59 patients with chronic hepatitis C. Paired slides were available for review from 26 ribavirin and 27 placebo recipients. Iron deposition was assessed using coded slides stained with Perls Prussian blue and was semi-quantitated in hepatocytes, Kupffer cells, and areas of fibrosis. The overall iron score fell by 0.96 in the placebo group and increased 1.69 in the ribavirin recipients. Iron was deposited mainly in hepatocytes; the hepatocyte iron score increased from 2.19 to 3.81 in the ribavirin group. The amount of iron staining in Kupffer cells declined in the placebo group and increased slightly in the ribavirin group. Iron changes in areas of fibrosis were minor and did not differ between groups. Increased total hepatic iron deposition occurred during a 9-month course of ribavirin. Ribavirin-associated hemolysis deposits iron preferentially in hepatocytes. This increased deposition of hepatic iron does not seem to affect the biochemical or histologic response to ribavirin therapy but may have implications for hepatocyte susceptibility to future injury. (+info)
Diminished injury in hypotransferrinemic mice after exposure to a metal-rich particle.
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Using the hypotransferrinemic (Hp) mouse model, we studied the effect of altered iron homeostasis on the defense of the lung against a catalytically active metal. The homozygotic (hpx/hpx) Hp mice had greatly diminished concentrations of both serum and lavage fluid transferrin relative to wild-type mice and heterozygotes. Fifty micrograms of a particle containing abundant concentrations of metals (a residual oil fly ash) was instilled into wild-type mice and heterozygotic and homozygotic Hp animals. There was an oxidative stress associated with particle exposure as manifested by decreased lavage fluid concentrations of ascorbate. However, rather than an increase in lung injury, diminished transferrin concentrations in homozygotic Hp mice were associated with decreased indexes of damage, including concentrations of relevant cytokines, inflammatory cell influx, lavage fluid protein, and lavage fluid lactate dehydrogenase. Comparable to other organs in the homozygotic Hp mouse, siderosis of the lung was evident, with elevated concentrations of lavage fluid and tissue iron. Consequent to these increased concentrations of iron, proteins to store and transport iron, ferritin, and lactoferrin, respectively, were increased when assayed by immunoprecipitation and immunohistochemistry. We conclude that the lack of transferrin in Hp mice did not predispose the animals to lung injury after exposure to a particle abundant in metals. Rather, these mice demonstrated a diminished injury that was associated with an increase in the metal storage and transport proteins. (+info)
Marginal siderosis and degenerative myelopathy: a manifestation of chronic subarachnoid hemorrhage in a horse with a myxopapillary ependymoma.
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Marginal siderosis is recognized in humans as an uncommon clinicopathologic entity characterized by degeneration of neural tissue at the surface of the brain and spinal cord, in association with the accumulation of hemosiderin, and resulting from chronic subarachnoid hemorrhage. The sources of hemorrhage are various and include neoplasms, malformations, cysts, and vasculopathy. Marginal siderosis of the spinal cord due to a myxopapillary ependymoma was diagnosed in a 19-year-old Dutch Warm Blood horse with clinical signs of myelopathy. There is only one previous report of marginal siderosis in the veterinary literature, also in a horse with clinical myelopathy. (+info)
Two remarkable events in the field of intraocular foreign body: (1) The reversal of siderosis bulbi. (2) The spontaneous extrusion of an intraocular copper foreign body.
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Two unusual events concerning intraocular foreign bodies are presented. The first patient had an occult or unsuspected intraocular foreign body. He showed iridoplegia with mydriasis, siderosis iridis, and an intraocular piece of iron lying posteriorly near the retina. The foreign body was removed and the patient regained normal iris color and pupillary activity. His vision remains 20/15 six years postoperatively dispite ensuing retinal detachment one year after removal of the foreign body. The second patient was a young boy injured by a blasting cap explosion. He lost one eye from the injury and had a piece of intraocular brass in his left eye. In spite of the development of chalcosis and a mature cataract the lens gradually shrank in the pupillary space permitting a clear aphakic area and 20/25 vision. The brass fragment migrated forward and inferiorly and was finally extruded under the conjunctiva five years later, where it was removed and chemically analyzed by x-ray diffraction. (+info)
MRI findings in a case of a superficial siderosis associated with an ependymoma.
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We present the imaging findings of superficial siderosis of the central nervous system associated with an ependymoma of the posterior fossa in a patient who presented with progressive bilateral sensorineural hearing loss and cerebellar ataxia. The ependymoma was a homogeneous well-defined mass of the fourth ventricle without hydrocephalus. Secondary siderosis due to chronic bleeding from the ependymoma appeared as linear hypointensity delineating the surface of the cortex, thin and subtle on spin-echo T2-weighted images, thick and obvious on gradient-echo T2-weighted images. (+info)
Welder's pneumoconiosis: diagnostic usefulness of high-resolution computed tomography and ferritin determinations in bronchoalveolar lavage fluid.
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OBJECTIVE: We assessed the usefulness of high-resolution computed tomography (HRCT) and ferritin determinations in bronchoalveolar lavage (BAL) fluid for diagnosis of welder's pneumoconiosis. PATIENTS AND METHODS: We investigated 11 patients with welder's pneumoconiosis who were 34 to 67 years old and had been welding for 17 to 45 years. Ten patients were current smokers. We performed chest radiography and HRCT, and measured serum ferritin on all 11 patients. We obtained BAL fluid in 9 cases and transbronchial lung biopsy (TBLB) specimens in 7. Ferritin concentrations in BAL fluid were compared with those in welders without pneumoconiosis and other pneumoconiosis cases. RESULTS: HRCT revealed small centrilobular nodules in 9 cases, mild fibrotic changes in 3, and emphysematous changes in 3. Serum ferritin concentrations were elevated (>240 ng/ml) in 10 cases. Ferritin concentrations in BAL fluid were higher in welder's pneumoconiosis than in the occupational control group. CONCLUSION: In welder's pneumoconiosis, small centrilobular nodules are frequently seen on HRCT, and ferritin shows elevations in serum and/or BAL fluid. Such ferritin determinations are of value in diagnosis. (+info)