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PubMed1980

Elemental content in alveolar septa in various pneumoconioses.

The elemental content of alveolar septa in various pneumoconioses was examind for a possible relationship to interstitial fibrosis. Silicon content, as well as heavy metal content, were evaluated to deterrmine possible interrelationships between the various inorganic dusts. Silicon levels were determined on the basis of silicon-sulphur ratios to accommodate for differences in tissue mass. A study of the variation of silicon-sulphur ratios in silicotics and normal individuals showed no false positives by our diagnostic criteria. A study of 11 cases of siderosis revealed that silicon was not responsible for the fibrosis as previously believed. An analysis of the lungs of 25 non-silicotic fibroses revealed that silicon-sulphur ratios were within normal limits. This technique should be useful in quantitating high exposure to certain dusts and in studying the relationship of such dusts to fibrosis.

Electron Probe Microanalysis↗

PubMed1978

[Glaucoma in the presence of chalcosis (author's transl)].

There are only few reports in the literature about the coincidence of a glaucoma with a chalcosis. After briefly presenting such a case the authors discuss the differential diagnosis; a strictly monocular chronic simple glaucoma, a contusion glaucoma or a glaucoma secondary to chalcosis. Since glaucoma and chalcosis coincide so infrequently there is no basis for assuming a true secondary glaucoma due to chalcosis existing, as is the case with siderosis.

Adult↗

PubMed1995

Effects of heterozygosity for the Rb-1t19neo allele in the mouse.

We describe the pathology of a cohort of 80 mice heterozygous for an inactive allele of the Rb-1 tumour suppressor gene. The majority of these mice developed locally invasive tumours, arising from the pituitary gland. The time of onset of overt signs of disease in mice known to have inherited their mutant allele paternally shows a small but statistically significant shift to the lower end of the spectrum, suggesting that tumorigenesis is influenced by gametic imprinting. In situ hybridisation analysis demonstrates the presence in the tumours of pro-opiomelanocortin mRNA, which is normally found both in corticotroph and melanotroph cells. Mice within this cohort also develop systemic defects. Most notably, there is increased siderosis in the spleen indicating the possibility of an abnormality in red blood cell turnover. This is consistent with the abnormalities of erythropoiesis described previously in homozygous Rb-1-deficient mice. In addition, a proportion of mice developed liver steatosis, probably representing the end organ effects of hormonal imbalance as a direct consequence of tumour presence. A significant proportion showed C cell hyperplasia in the thyroid. The spectrum of pathology in mice differs from that in the human but does provide a useful model of site-specific tumour predisposition.

Alleles↗

PubMed1995

Five liability claims involving the cornea and how they could have been prevented.

Five recent professional liability claims involving the cornea are reviewed, arising from five unusual conditions: fungal infection following corneal abrasion with a tree branch; herpes simplex-induced neurotrophic keratitis resulting from contact lens wear; corneal necrotization caused by lime particulate matter splashed into the eye; siderosis-related retinal detachment secondary to a penetrating iron foreign body; and fluctuating acuity, halos, and flare resulting from radial keratotomy. Emphasis is on how these claims might have been avoided.

Adult↗

PubMed1978

Early idiopathic hemochromatosis with absent stainable bone marrow iron stores.

Early idiopathic hemochromatosis was diagnosed in a 52-year-old woman during the course of a hospitalization for another disorder. Quantitative studies revealed massive hepatic parenchymal siderosis and absent stainable marrow iron. Additionally, intestinal absorption and hepatic sequestration of radiolabeled iron were elevated. These studies in a patient with very early hemochromatosis, before organ damage, complement observations previously made in patients with advanced disease and provide further support for the concept that this disorder may result from impaired reticuloendothelial handling of iron.

Bloodletting↗

PubMed1993

Elevated interleukin-8 serum concentrations in beta-thalassemia and graft-versus-host disease.

Neutrophil chemotactic and functional defects occur in beta-thalassemia and in patients after bone marrow transplantation (BMT). Interleukin-8 (IL-8) is a novel chemotactic and activating peptide for neutrophils and can be detected in the circulation. IL-8 serum concentrations were evaluated in 30 beta-thalassemic patients before and after BMT. Serial samples from 16 patients were also studied. Fourteen sera from healthy children, 43 patients with chronic viral hepatitis, 16 patients on chronic transfusion treatment for various hematologic disorders, and 28 healthy adults were studied as controls. IL-8 was evaluated by an enzyme-linked immunosorbent assay. Patients with beta-thalassemia had higher IL-8 concentrations than did normal controls, patients with liver disease, and patients on chronic transfusion. beta-Thalassemic patients with severe liver siderosis and fibrosis had the highest IL-8 concentrations. After BMT in patients with successful engraftment, IL-8 concentrations decreased significantly. In contrast, in patients with acute graft-versus-host disease (GVHD), IL-8 concentrations were not statistically different from the concentrations found before BMT and were higher than in patients with no complications and patients with graft rejection. IL-8 may play a part in the immune dysregulation that occurs in beta-thalassemia and may be involved in the immune mechanisms leading to GVHD.

Adolescent↗

PubMed1993

[Superficial cerebral hemosiderosis: a rare cause of ataxia or hypoacusis].

Superficial siderosis of the central nervous system (SSC) typically involves slowly progressive ataxia, hypoacusis and dementia, possibly with pyramidal signs and sphincter disturbances in combination with xanthochromic CSF with siderophages. However, there are also atypical oligosymptomatic forms. Before the era of magnetic resonance imaging (MRI), diagnosis was only possible at autopsy. Nowadays a firm diagnosis can be made during life by demonstrating the typical marginal hypointense signal in the T2-weighted images in the cerebrum, brain stem, cerebellum and spinal cord. Contrast-enhanced computed tomography may demonstrate widespread meningeal enhancement, but this sign is not specific for SSC. We present an oligosymptomatic case of SSC with slowly progressive ataxia and slight hypoacusis. The etiology, gross pathological and histopathological findings, differential diagnosis and therapy are discussed.

Aged↗

PubMed1995

The difference in liver pathology between sporadic and familial forms of porphyria cutanea tarda: the role of iron.

Porphyria cutanea tarda is a disorder of porphyrin metabolism, of which familial and sporadic forms have been described. Factors such as iron seem necessary for porphyria cutanea tarda to become clinically manifest. To study the relationship between iron and uroporphyrins in hepatocytes of patients with porphyria cutanea tarda, a morphological and morphometrical study was performed in 13 liver biopsies of patients with porphyria cutanea tarda (eight with sporadic porphyria cutanea tarda and five with familial porphyria cutanea tarda). In addition, possible differences in clinical and biochemical features and in histopathological findings between patients with sporadic porphyria cutanea tarda and familial porphyria cutanea tarda were investigated. Familial porphyria cutanea tarda patients presented at a younger age than sporadic porphyria cutanea tarda patients (42.4 +/- 5.3 vs. 57.3 +/- 8.6 years). Biochemical features were not different between sporadic porphyria cutanea tarda and familial porphyria cutanea tarda patients. Uroporphyrin crystals and a variable degree of liver siderosis were detected in the biopsies of all 13 patients. Uroporphyrin crystals were often found close to ferritin-like iron deposits. The morphometrical analysis showed that an increased mean area fraction of ferritin iron was associated with an increased mean area fraction of uroporphyrin crystals in hepatocytes of sporadic porphyria cutanea tarda and familial porphyria cutanea tarda patients. Moreover, the amount of uroporphyrin crystals was significantly higher in livers of familial porphyria cutanea tarda than sporadic porphyria cutanea tarda patients. These findings are consistent with the hypothesis that uroporphyria is precipitated by an iron-dependent process.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1995

[Changes in vitreous structure caused by oxygen free radicals].

Vitreous liquefaction in humans is considered to be part of the normal ocular aging process and is associated with vitreoretinal pathology. Because hyaluronic acid (HA), one of the main components of the vitreous gel structure, is degraded by reactive oxygen species (ROS) including oxygen free radicals, the structural changes in the vitreous may be caused by ROS. To investigate the effect of ROS on the vitreous gel structure, we treated animal vitreous with ROS, which was generated from various sources. Using riboflavin as a photosensitizer, calf vitreous was irradiated with visible light (two 15-W fluorescent lamps) and found to be considerably liquefied. The liquefaction resulted from HA depolymerization induced by ROS. Because of the small amount of riboflavin naturally present in the vitreous, a riboflavin-sensitized photochemical reaction may contribute to age-related vitreous liquefaction. Hematoporphyrin (HP), which is similar in chemical structure to heme in blood, was also used as a photosensitizer. Irradiation with HP destroyed the calf vitreous gel structure and caused liquefaction. A HP-sensitized photochemical reaction may contribute to vitreous liquefaction observed after vitreous hemorrhage. Because metal ions, including Fe2+ and Cu2+, can catalyze to generate ROS, liquefaction occurred when we treated calf vitreous with Fe2+ or Cu2+ at 4 degrees C. Adding ascorbic acid to the vitreous during the reaction increased the rate of liquefaction. Therefore, metal ion catalyzed ROS may also contribute to vitreous liquefaction, such as that found in an injured eye with siderosis. To investigate the relation between inflammatory cell mediated ROS and vitreous liquefaction, an endotoxin-induced uveitis model was created in the rabbit eye. Upon inflammation, the vitreous gel contracted and released a water-like liquid. Because superoxide dismutase can suppress the liquefaction, the destruction of the vitreous gel structure resulted from ROS generated from inflammatory cells. Although many unknown factors contribute to vitreous liquefaction, ROS may be the main cause of vitreous structure alterations. To prevent or delay the progress of vitreous liquefaction in the normal aging process or vitreous pathology, a new therapeutic procedure based on clear scientific studies is needed.

Aging↗

PubMedDate not supplied

Xanthogranuloma and xanthoma of the choroid plexus: evidence for different etiology and pathogenesis.

Xanthogranulomas of the choroid plexus (XGCP) and xanthomas of the choroid plexus (XCP) are generally held to be closely related to each other. Obtained by autopsy 17 cases with XGCP and 21 cases with XCP were investigated. Foamy cells were confirmed to be constitutional for both lesions. Immunohistochemically their antigen profile was shown to be of histiocytic type. A dense siderosis emerged as a consistent feature of XGCP but not of XCP. In no case both tumors were found associated. Analysis of clinical data revealed hyperlipidemia in 15 out of 17 cases with XCP but in only 3 out of 13 cases with XGCP (p < 0.01; in 4 cases of each sample pertinent data were pending). The male-to-female ratio was 14:3 in cases with XGCP and 13:8 in cases with XCP; when combined with data of other authors the male preponderance in XGCP became statistically significant. In conclusion, XGCP and XCP emerged as different independent lesions. XCP appeared to be closely related for example to tendon xanthomas of the skin which are well established as being diagnostic of hyperlipidemia. XGCP, on the other hand, are most likely to correspond to cholesterol granulomas of extracranial provenance. Thus, the data presented strongly recommend the replacement of the term "xanthogranuloma of choroid plexus" with the term "cholesterol granuloma of choroid plexus".

Adult↗

PubMed1996

Interactive effects of polycyclic aromatic hydrocarbons and iron oxides particles. Epidemiological and fundamental aspects.

Iron oxides are present in many occupational atmospheres mainly in iron ore mines and in steel industry. Among these workers, epidemiological studies indicated an excess of lung cancer deaths. In mines, it was difficult to involve iron oxides exposure because there are other possible causes as radon, polycyclic aromatic hydrocarbon (PAH) present in diesel exhausts, silicosis or siderosis. The contradictory results of these studies are due to the differences of exposure levels or to the presence or not of these cofactors or of a sufficient prevention. But generally the results agree with an interaction of iron oxide dusts and smoking habits. It is unclear if this interaction supports an additive or multiplicative risk of lung cancer. Experimental studies with Fe2O3 showed that these particles are able to induce lung cancers only in the presence of PAH when administered to animals. In vitro studies permitted to observe an interaction in the metabolism of benzo(a)pyrene (BaP) leading to a higher level of precursors of the ultimate carcinogen. As this metabolism of BaP is known to be enhanced during lipoperoxidation, it is possible to involve this mechanism with Fe2O3. After phagocytosis and dissolution with production of ferric ions, Fe2O3 can enhance the production of reactive oxygen species responsible of damaging both lipidic constituents and DNA. Fe3O4 and mainly FeO may be more toxic, introducing directly ferrous ions in the cells after dissolution, but the cancerogenicity of the these compounds is unknown, making necessary to develop research.

Animals↗

PubMed1997

Screening of patients with iron overload to identify hemochromatosis and porphyria cutanea tarda.

OBJECTIVE: To assess the importance of iron overload as a risk factor for porphyria cutanea tarda (PCT). DESIGN: Prospective study during a 4-month period. SETTING: Departments of emergency care, gastroenterology, and dermatology in a tertiary referral center. PATIENTS: Patients were deemed eligible for inclusion in the study if serum ferritin levels were greater than 500 micrograms/L (normal range: females, < 125 micrograms/L; males, < 325 micrograms/L). MAIN OUTCOME MEASURES: Porphyrin excretion profiles were analyzed on all patients included in the study, where clinically relevant. A diagnosis of PCT was confirmed biochemically in all cases. The HLA typing was then performed on newly diagnosed cases of PCT. RESULTS: Of 4127 patients tested, 240 patients with an elevated serum ferritin level were identified, of whom 74 had an elevated serum ferritin level of more than 500 micrograms/L. Of the latter group, 17.5% had hemochromatosis and 6.7% had PCT. The incidence of PCT in the hemochromatosis group was 23%; HLA typing revealed the presence of at least 1 of the hemochromatosis markers. CONCLUSIONS: A high serum ferritin level in the absence of evident cause should prompt investigation for both hemochromatosis and PCT. The HLA heterozygosity for hemochromatosis in some patients with PCT may be a cause of hepatic siderosis.

Adult↗

PubMed1998

Secondary iron overload disorders.

Diverse clinical disorders distinct from hereditary hemochromatosis are associated with accumulation of excess body iron in heterogeneous patterns and through various mechanisms. A deranged iron turnover somehow relates to the altered physiological barrier for iron absorption in several defined chronic anemias with ineffective erythropoiesis. Unexcretable excess iron acquired from transfusions provides a therapeutic challenge. Genetic defects of proteins essential for transport of iron into and out of cells (transferrin and ceruloplasmin) deprive the erythron of the metal and cause its accumulation in other vital organs. The hemochromatosis alleles predictably contribute to an iron burden from other causes, commonly facilitate the expression of porphyria cutanea tarda, and their clinical expression may be accelerated by hereditary hemolytic anemias. Even minimal iron excess in liver disease may contribute to the hepatocellular injury from factors such as alcohol and viruses. Uniquely localized siderosis occurs in the lung and kidney where iron cannot turn over and causes variable tissue damage. The most devastating iron overload disorder, neonatal hemochromatosis, is understood least of all.

Humans↗

PubMed1998

[Neonatal hemochromatosis].

Neonatal haemochromatosis is a disorder which affects foetuses and newborns. It is characterized by hepatocellular insufficiency, often appearing on the first day of life in the form of coagulopathy, hypoalbuminemia, hypoglycemia and jaundice. While spontaneous recovery has been reported, most of these infants die, and the diagnosis was previously often made during autopsy. With the help of MRI and salivary gland biopsies, plus increasing awareness of this disorder, the diagnosis is now often made quite early, and successful liver transplantations have been reported. Recently, there have also been encouraging preliminary reports of successful intervention with antioxidant and chelation pharmacotherapy, using a combination of selenium, vitamin E, N-acetylcysteine, deferoxamine, and prostaglandin E. We describe two patients with neonatal haemochromatosis who were both treated with this new "cocktail", one of whom died at five days of age, while the other survived, but needed a liver transplant at 2 1/2 months of age. The pathology of this condition is characterized by hepatic cirrhosis with giant cell transformation, and by siderosis of extrahepatic tissues. The prognosis is poor, and our experience with antioxidant treatment has been disappointing. Liver transplantation is a therapeutic option, but its use is limited by the scarcity of donor organs and the small size of many of the patients.

Antioxidants↗

PubMed1997

[Favorable effect of splenectomy on anemia in 3 siblings with type II congenital dyserythropoietic anemia (HEMPAS). (Ultrastructural changes in erythrocytes after splenectomy)].

Congenital dyserythropoietic anemia type II (CDA-type II) (HEMPAS) was reported in three siblings. CDA-type II was associated with marked hepatosplenomegaly and siderosis of both organs. All three sibling developed cholelithiasis with choledocholithiasis and obstructive jaundice in two of them. Anemia showing hemolytic component with sequestration of erythrocytes in the spleen was corrected after splenectomy without the need of blood transfusions during the follow up period of 20, 11 and 11 years. Ultrastructural investigation of the erythrocytes after splenectomy revealed increased number of erythrocytes showing the double membrane phenomenon.

Adult↗

PubMed1998

[Case of superficial hemosiderosis of the central nervous system treated with trientine].

A 58-year-old woman, with recurrent headaches, exhibited cerebellar alaxic gait, anosmia, deafness and a pyramidal syndrome, with a progressive onset. In cerebrospinal fluid there was erythrocytes and siderophages. MRI on T2-weighted images revealed a marginal hypo-intensity, leading to the diagnostic of superficial siderosis of the central nervous system. None haemorragic lesion was found. The patient was given Trientine. Unfortunately she worsened on later examinations.

Brain↗

PubMed1998

Ocular sequelae of BB injuries to the eye and surrounding adnexa.

BACKGROUND: BB injuries continue to be one of the most common causes of severe ocular injury among adolescent males. Extraocular and nonperforating anterior globe injuries most commonly result in favorable visual outcomes. The appropriate medical management of both perforating and nonperforating anterior globe injuries resulting from BB trauma and their potential ocular sequelae are reviewed. CASE REPORTS: Three cases illustrating typical as well as atypical anterior globe injuries caused by BBs are presented. Two cases involve nonperforating anterior globe injuries--one with intracranial and one with intraorbital involvement. The third involves a retained intraocular foreign body. The biomicroscopic and ophthalmoscopic features of these injuries are discussed, as are potential complications and management strategies associated with these types of trauma. CONCLUSIONS: Injuries associated with intraocular BBs often result in enucleation if functional vision is not salvageable. Sequelae to such injuries include endophthalmitis, traumatic hyphema, ocular siderosis, and sympathetic ophthalmia. Plain-film radiography and axial computed tomography are critical first steps in determination of the position of any intracranial or intraorbital metallic foreign body. Routine observation for the development of ocular sequelae resulting from retained intraocular, intracranial, and intraorbital BB injuries is recommended.

Adolescent↗

PubMed1998

A multicenter prospective study on the risk of acquiring liver disease in anti-hepatitis C virus negative patients affected from homozygous beta-thalassemia.

Although the risk of transfusion-transmitted hepatitis has been recently reduced, transfusion-dependent beta-thalassemia patients may still develop liver disease due to viral infection or iron overload. We assessed the frequency and causes of liver dysfunction in a cohort of anti-hepatitis C virus (HCV) negative thalassemics. Of 1,481 thalassemics enrolled in 31 centers, 219 (14.8%) tested anti-HCV- by second-generation assays; 181 completed a 3-year follow-up program consisting of alanine-aminotransferase (ALT) measurement at each transfusion and anti-HCV determination by third-generation enzyme-immunoassay (EIA-3) at the end of study. Serum ferritin levels were determined at baseline and at the end of follow-up. Ten patients were anti-HCV+ by EIA-3 at the end of follow-up. Of them, seven were already positive in 1992 to 1993 when the initial sera were retested by EIA-3, one tested indeterminate by confirmatory assay, and two had true seroconversion (incidence, 4. 27/1,000 person years; risk of infection, 1/7,100 blood units, 95% confidence interval [CI], 1 in 2,000-1 in 71,000 units). At baseline, 67 of 174 thalassemics had abnormal ALT. Of those with normal ALT, seven subsequently developed at least one episode of moderate ALT increase (incidence, 24.6/1,000 person-years). All of the 20 patients with ferritin values >/=3,000 ng/mL had clinically relevant ALT abnormalities, as compared with 53 of 151 with <3,000 ng/mL (P < .005). Hepatic dysfunction is still frequent in thalassemics. Although it is mainly attributable to siderosis and primary HCV infection, the role of undiscovered transmissible agents cannot be excluded.

Adolescent↗