Autoimmune hemolytic anemia associated with colon cancer.
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Adenocarcinoma↗
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Results for “HEMOSIDEROSIS”
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Hepatic pathology associated with Hodgkin's disease.
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Adult↗
Hereditary hemochromatosis and red cell aplasia.
A 63-year-old white man with myasthenia gravis and red cell aplasia was found to have hepatic iron overload. The differential diagnosis included hereditary hemochromatosis and secondary iron overload related to red cell aplasia. Family studies of siblings, including HLA typing, initially suggested a diagnosis of secondary iron overload. The investigation of a remote sibling led to the final diagnosis of hereditary hemochromatosis. The case illustrates the importance of complete family investigations in the differentiation between hereditary hemochromatosis and secondary iron overload and the potential hazard of using HLA typing as an indirect genetic test for hereditary hemochromatosis.
Diagnosis, Differential↗
Increased iron absorption in lemurs: quantitative screening and assessment of dietary prevention.
Iron storage disease (ISD) in lemurs has been reported since as early as the 1960s, and in the 1980s was demonstrated to be a consistent finding in postmortem investigations of captive lemurs. Since then this disease has consistently been diagnosed at the point of necropsy. In the current study we describe a preclinical screening procedure, as well as the quantified preventive effects of dietary intervention upon iron absorption. Twenty-three individual lemurs of four species were initially tested with the transferrin saturation test (%TS); 21 of these animals were on conventional zoo diets, and two were fed a specific diabetic diet. Initially, 20 of 21 lemurs on conventional zoo diets were demonstrated to have %TS levels above the normal range for humans; 17 of these lemurs were in the category (for humans) of excessive iron absorption. A dietary change aimed at reducing dietary iron and vitamin C levels and increasing the levels of iron-chelating tannins and/or phytates was instigated. After the animals were retested, a matched-pair comparison of %TS values before and after the diet change revealed significantly (P=0.038, n=7) lower %TS values after the diet change. All species averages were in the human hyperabsorption range on conventional zoo diets (n=21). No species averages were in that range after the dietary change (n=18). The results indicate that further investigations into the use of %TS testing in lemur husbandry, and specific preventive dietary measures, should be conducted.
Animals↗
Neuroaxonal dystrophy and hemosiderin in the central nervous system.
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Axons↗
A case report of oxidative stress in a patient with anorexia nervosa.
OBJECTIVE: Liver injury is well known as a complication of anorexia nervosa (AN), and steatosis of the liver has been thought to be the main cause of the injury. However, the precise mechanism for the liver injury is still unclear. METHOD AND RESULTS: We had a chance to examine the histological change of the liver with AN, and found that not only fat deposit but also peroxidated lipid products and irons were recognized in hepatocytes. CONCLUSION: Our present case demonstrates that oxidative stresses in hepatocytes, which might be associated with iron deposition, could be involved in the pathogenesis of liver injury in patients with AN.
Adult↗
Ultrastructural evidence for the presence of ferritin-iron in the biliary system of patients with iron overload.
Ferritin-like particles were observed in bile canaliculi of patients with iron overload. These particles have been further investigated by: a staining method enhancing the size and contrast of ferritin protein, and electron probe microanalysis detecting the presence of the elements iron and phosphorus. Morphological observation of coated vesicles in the cytoplasm adjacent to the bile canaliculi and coated pits in the canalicular membrane suggests a transport mechanism via membrane-bound organelles. Support is given to the theory that part of the iron, stored in the liver, leaves the hepatocyte by excretion of ferritin into the bile.
Anemia, Sideroblastic↗
An oral treatment for iron overload in thalassemia?
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Administration, Oral↗
Histopathology of the liver in children with chronic hepatitis C viral infection.
Although the epidemiology, natural history, and pathological aspects of chronic hepatitis C are well-defined in the adult population, little is known about the characteristics of chronic hepatitis C infection in children. Reports on the histological features and progression of hepatitis C in children are scarce, and consist primarily of multicenter studies in Japanese and European children. Given the geographic variations in viral genotype and the association of pathology with genotype, whether the Japanese and European studies can be extended to the North American populations is unclear. We report the histopathology of the liver in 40 children with chronic hepatitis C infection treated in a single North American institution. The children included 19 males and 21 females ranging in age from 2.0 to 18.6 years at the time of liver biopsy (mean +/- SD: 11.4 +/- 4.3 years). Our findings indicate that the characteristic histopathological lesions of chronic hepatitis C infection, including sinusoidal lymphocytosis, steatosis, portal lymphoid aggregates/follicles, and bile duct epithelial damage, occur with approximately the same frequencies in children as have been reported in adults. Necroinflammatory activity was generally mild. Portal fibrosis was present in 78% of the specimens, including fibrous portal expansion (26%), bridging fibrosis (22%), bridging fibrosis with architectural distortion (22%), and cirrhosis (8%). Centrilobular pericellular fibrosis, which has not been previously reported in the context of chronic hepatitis C infection in adults or children, was also a prominent feature in our series, occurring with a similar frequency as steatosis or portal lymphoid aggregates/follicles. Our data suggest that in spite of mild histological necroinflammatory activity in general, the stage of fibrosis in children can be severe in spite of relatively short duration of infection.
Adolescent↗
Clinical heterogeneity of neurodegeneration with brain iron accumulation (Hallervorden-Spatz syndrome) and pantothenate kinase-associated neurodegeneration.
Hallervorden Spatz syndrome (HSS), also referred to as neurodegeneration with brain iron accumulation (NBIA), is a rare inherited neurodegenerative disorder with childhood, adolescent, or adult onset. Patients with HSS/NBIA have a combination of motor symptoms in the form of dystonia, parkinsonism, choreoathetosis, corticospinal tract involvement, optic atrophy, pigmentary retinopathy, and cognitive impairment. After the recent identification of mutations in the PANK2 gene on chromosome 20p12.3-p13 in some patients with the HSS/NBIA phenotype, the term pantothenate kinase-associated neurodegeneration (PKAN) has been proposed for this group of disorders. To characterize clinically and genetically HSS/NBIA, we reviewed 34 affected individuals from 10 different families, who satisfied the inclusion criteria for NBIA. Relatives of patients who had clinical, magnetic resonance imaging (MRI), or pathological findings of NBIA were included in the study. Four patients were found to have mutations in the pantothenate kinase 2 (PANK2) gene. We compared the clinical features and MRI findings of those with and without PANK2 mutations. The presence of mutation in the PANK2 gene is associated with younger age at onset and a higher frequency of dystonia, dysarthria, intellectual impairment, and gait disturbance. Parkinsonism is seen predominantly in adult-onset patients whereas dystonia seems more frequent in the earlier-onset cases. The phenotypic heterogeneity observed in our patients supports the notion of genetic heterogeneity in the HSS/NBIA syndrome.
Adolescent↗
Hepatocellular carcinoma in thalassemia major.
The occurrence of hepatocellular carcinoma in a 22-year-old man with thalassemia major is reported. As a result of transfusional hemochromatosis, this patient had already developed diabetes, hypogonadism, heart failure, and the sicca syndrome; he was serum and tissue HBsAg negative. Liver iron concentration measured postmortem was found to be 50 times normal. Multiply transfused patients are at risk of developing hepatocellular carcinoma. Serial measurements of serum alpha-fetoprotein should permit early detection of the tumor and reduce mortality. Preventive measures include early immunisation against hepatitis B virus and prevention of iron accumulation by intensive use of desferrioxamine. Treatment of hemochromatosis-associated hypogonadism with androgens should be considered with caution.
Adult↗
Experimental siderosis and liver injury in the rhesus monkey.
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Animals↗
Fine structural changes in idiopathic pulmonary haemosiderosis.
Lung biopsies from four children and two adults with idiopathic pulmonary haemosiderosis have been examined by transmission electron microscopy. No qualitative differences were identified between the children and the adults but the changes were more severe in the children. In each case the major damage involved the capillary endothelium and its basement membrane. Capillary endothelial swelling was very noticeable and in one case the endothelium was attenuated but gaps between endothelial cells were very difficult to find. Capillary narrowing and platelet aggregation were common. The capillary endothelial basement membrane showed focal thickening, particularly on the thick side of the air/blood barrier, but no electron dense deposits were identified. Degenerative changes in the alveolar epithelium were not so marked as those in the capillary endothelium and the epithelial basement membrane was normal except for haemosiderin deposition. Haemosiderin was also noted on elastin and within intra-alveolar macrophages. Other secondary changes included mild interstitial oedema and fibrosis. These findings indicate that the major site of damage is the alveolar capillary, but provide no evidence of the cause of the disease.
Adolescent↗
The hypotransferrinaemic mouse: ultrastructural and laser microprobe analysis observations.
Homozygote hypotransferrinaemic mice (hpx/hpx) have cytopathological features similar to those of human congenital atransferrinaemia, genetic haemochromatosis, and neonatal haemochromatosis. These conditions all have in common high levels of cytotoxic non-transferrin-bound serum iron. This study describes the ultrastructural features of iron overload in liver, pancreas, heart, and small intestine of 2- and 12-month-old hypotransferrinaemic mice. 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). In the 12-month-old animals, iron was also found in Kupffer cells and macrophages in other tissues. In addition, there were conspicuous iron-containing compounds in the bile canaliculi, and marked iron deposition in the pancreas and heart. Laser microprobe mass analysis (LAMMA) enabled localization and relative quantitation of iron deposition in subcellular compartments providing in situ documentation of iron accumulation in siderosomes and contributed in assessing total cytosolic iron in various cell types. Moreover, it demonstrated the importance and magnitude of the biliary route for iron excretion in these animals.
Animals↗
Hemoptysis in children.
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Anti-Glomerular Basement Membrane Disease↗
Characterization of hepatic iron overload following dietary administration of dicyclopentadienyl iron (Ferrocene) to mice: cellular, biochemical, and molecular aspects.
A unique organic form of iron (dicyclopentadienyl iron; ferrocene) has been used to further elucidate specific hepatic histopathologic, biochemical, and molecular parameters associated with dietary iron overload. Male C57BL/6Ibg mice fed a diet containing 0.04-0.2% w/w ferrocene for 115 days displayed severe hepatic siderosis of hepatocytes accompanied by a 15-fold induction of nonheme iron content compared to control mice receiving a diet with normal amounts of iron. The ferrocene treatment led to significant increases in hepatocellular necrosis as measured by plasma alanine aminotransferase activity. Histological assessment of hepatic fibrosis revealed mild increases in collagen deposition localized with accumulations of hemosiderin primarily in centrilobular hepatocytes. Hepatic fibrosis was confirmed by measurement of hepatic hydroxyproline content that was increased 4-fold in ferrocene-fed animals compared to control animals not ingesting ferrocene. Hepatic siderosis was accompanied by significant increases in hepatic malondialdehyde content suggesting the ferrocene-induced iron burden initiated lipid peroxidation in vivo. Expression of the heavy-chain isoform of ferritin mRNA and protein measured in liver after ferrocene feeding was increased approximately 8- and 2-fold, respectively, compared to the appropriate controls. These results, using an organic form of iron fed to genetically well-characterized inbred mice, provide new additional insight into the specific molecular and biochemical events that occur in association with histopathologic changes initiated by iron-induced liver injury. These data support the hypothesis that peroxidation of cellular membrane lipids is an important mechanism involved in the toxicity of excess hepatic iron and possibly the initiation of liver fibrogenesis. The results presented here also provide novel in vivo evidence documenting the cellular modulation of ferritin in response to the toxic effects of hepatic iron overloading and iron-mediated oxidative stress.
Alanine Transaminase↗
Yersinia enterocolitica septicaemia.
Septicaemia from Yersinia enterocolitica carries a mortality of up to 80%. We report the successful management of a case who required intensive therapy, including inotropic and ventilatory support.
Adult↗
Concentric joint space narrowing of the hip associated with hemosiderotic synovitis (HS) including pigmented villonodular synovitis (PVNS).
Concentric joint space narrowing of the hip is an expected radiographic finding in cases of inflammatory arthritis such as rheumatoid arthritis or sepsis. However, similar joint space narrowing is associated with chronic hemorrhagic conditions that produce hemosiderotic synovitis. Hemosiderotic synovitis results from chronic intra-articular bleeding such as occurs in pigmented villonodular synovitis, generalized bleeding diathesis, synovial hemangioma, and chronic trauma. Five hips in five patients with concentric joint space narrowing not associated with inflammatory arthritis or with hemophilia were reviewed clinically, radiographically, and pathologically. All patients had a hemosiderotic synovitis. The definitive diagnosis of pigmented villonodular synovitis was made pathologically in two cases that demonstrated nodular areas of giant cell proliferation, collagen production, and lipid-laden histiocytes on histologic samples.
Adult↗