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[A case of rapid development of arc welder's lung during the course of a year].

A 57-year-old man who had worked as a welder for about 40 years was admitted to our hospital. Every year he had had medical examinations in his workplace. On March 17, 1999, there was no finding of pneumoconiosis, but he had worked in a tunnel on a special arc welding project from December 20, 1999 to January 10, 2000. On admission, chest radiography revealed reticular shadows in the middle and lower lung fields. CT scans showed ground-glass opacities in both lungs. Serum iron was high (231 micrograms/dl) and serum ferritin was extremely high (2,309 ng/ml). Many iron particles were detected in the alveoli of the transbronchial biopsy specimen, and also in the sputum. A pathological diagnosis of siderosis was therefore made, and, considering the patient's occupation, the condition was termed arc welder's lung. This rapidly developing case was characterized by reticular shadows on chest radiography.

Disease Progression↗

An unusual intaocular foreign body.

An unusual foreign body in a 60-year-old man with ocular siderosis is described. A surgical needle was found in the eye after magnetic resonance imaging was performed for cerebral ischemia. As a general precaution in patients who have undergone any kind of surgery, it might be better to perform a plain x-ray before performing magnetic resonance imaging to identify such foreign bodies.

Eye↗

[Delayed discovery of a metallic intraocular foreign body: diagnostic and therapeutic stakes].

Ocular siderosis is a serious and rare complication when a penetrating ocular injury with a metallic foreign body goes unnoticed. The foreign body must be urgently removed from the eye. If it is located on the ciliary body or the zonule, it may be difficult to visualize clinically. In this case, high-frequency ultrasound biomicroscopy can pinpoint its precise location and guide the surgical intervention.

Adult↗

[Abnormalities of the erythrocyte membrane ultrastructure and the membrane proteins in a patient with HEMPAS, alpha thalassemia and complicated diabetes].

OBJECTIVE: Diagnosis of a case with congenital dyserythropoietic anemia (CDA). METHODS: Routine tests for hemolysis were carried out. The activities of erythrocyte enzymes were measured according to the methods recommended by international committee for standardization in hematology (ICSH). The quantity and quality of erythrocyte membrane proteins were analyzed with 4% - 15% sodium dodecyl sulfate-polyacrylamide gradient gel electrophoresis (SDS-PAGE). The membrane ultrastructure of erythrocyte from bone marrow was observed under transmission electron microscope (TEM). RESULTS: The main results were: (1) Bone marrow morphology revealed erythroid hyperplasia and 0.10 symmetrically binucleated late erythroblasts. The erythrocytes in peripheral blood showed anisocytosis and hypochromia. (2) The intracellular iron was 0.98 and the storage iron was strongly positive in bone marrow. The serum ferritin was 1607 micro g/L. The content of blood sugar was 27.5 mmol/L. (3) Ham test was negative in his own acidified serum but positive in the group-compatible sera. (4) A quick mobile H band was seen in hemoglobin electrophoresis. H inclusion test was positive. (5) SDS-PAGE demonstrated that the migration of band 3 protein of erythrocyte membrane in an electric field was faster (110%) than that of normal controls and the relative contents of band 1, band 3, band 4.1 were reduced to various extent. (6) "Double membrane" with gap and shedding was observes under TEM. CONCLUSIONS: The final diagnosis of the case was CDAII, also called HEMPAS (hereditary erythroblastic multinuclearity with positive acidified serum), accompanied with alpha thalassemia and secondary siderosis and diabetes.

Adult↗

[Prevalence, causes and effects of increased iron storage in patients with kidney transplantation].

Patients on chronic hemodialysis often need blood transfusions due to erythropoietin deficiency. Even after successful kidney transplantation iron overload may persist. Former histological studies have revealed siderosis of the liver in 69% of all patients whose serum ferritin was above 1100 ng/ml. The aim of the present study was to evaluate the influence of iron overload on liver function. In 146 symptom free patients with renal allografts serum ferritin was determined to detect possible iron overload. Serum ferritin between 4 and 5480 ng/ml were found (women: 358.7 +/- 105.3; men 282.4 +/- 63.3 ng/ml; x +/- SEM). Twelve patients (8.1%) had ferritin levels higher than 1100 ng/ml. These twelve patients as well as another group of eight patients with renal allografts whose serum ferritin was known to be higher than 1100 ng/ml were included for further evaluation. Their data were matched and compared with those of a control group also patients with renal allograft (same age and sex) whose serum ferritin was lower than 1100 ng/ml. Transaminases (SGPT 22.6 +/- 3.6 vs. 15.4 +/- 6.0 U/l; SGOT 14.7 +/- 2.0 vs. 13.0 +/- 4.8 U/l) and plasma glucose (90.5 +/- 7.1 vs. 76.8 +/- 3.7 mg/dl) were found to be significantly higher (p less than 0.05) in patients with serum ferritin levels above 1100 ng/ml. Elevated transaminases were significantly more frequent in patients with high serum ferritin (9 vs. 2; p less than 0.02) as compared with the control. Ferritin levels significantly correlated with the number of preceding blood transfusions (p less than 0.002). Hbs-persistence was detected in six out of 20 patients with high ferritin levels but only in one out of 20 in the control group (p less than 0.05) whereas anti-Hbs prevalence was not different in the two groups. These data indicate that chronic iron overload should be considered as a possible cause of chronic liver disease in patients with renal allografts.

Adolescent↗

The Montgomery Lecture, 1979. Ocular metallosis.

'Redox processes' and complex biochemical formations offer a simple key to understanding metallotic damage to the eye. Various factors determine the timing and severity of this damage. Siderosis is a process with a chronic course. In a large group of patients with copper-containing foreign bodies the majority showed a more acute course. In the past, the relatively rare cases of chronic chalcosis without fibrotic reactions in the vitreous determined the text book descriptions familiar to our fathers. In our series of 282 cases of copper-containing foreign bodies in the vitreous, the chalcosis took a subacute, acute, or fulminating course in 68 per cent, and only half of the other 32 per cent represented time chronic chalcosis. In 18 per cent the injury was recent and the cellular reaction was only just beginning. Cellular reactions, especially fibrosis, are the chief characteristics of acute chalcosis. Vitrectomy permits the surgical treatment of eyes with severe vitreous fibrosis and retinal detachment, but because these eyes are in the pre-phthisic stage, a fair number of successes is counterbalanced by an increasing number of enucleations.

Acute Disease↗

Yersiniae and iron. A study in host-parasite relationships.

Most enterobacteria obtain the iron they require for growth by producing low-molecular-weight high-affinity iron ligands known as siderophores. These substances chelate and solubilize iron making it available to bacteria. The pathogenic Yersiniae produce no detectable siderophores; thus, they proliferate poorly or not at all under conditions of iron limitation. Most systemic infections with Yersinia enterocolitica occur in patients who are overloaded with iron. This may be due to the presence of excess iron in the tissues of such patients, but the adverse effects of excess iron on immune responsiveness may also be partly responsible. Many patients with iron overload receive treatment with desferrioxamine B, a bacterial siderophore which promotes growth of Y. enterocolitica in vitro and in vivo. Thus, desferrioxamine B may add to the risk of systemic yersiniosis developing in patients with siderosis. Some strains of Yersinia frederiksenii, Yersinia intermedia and Yersinia kristensenii produce the hydroxamate siderophore aerobactin, but, paradoxically, they appear to be unable to proliferate in tissues.

Deferoxamine↗

[The role of ultrasonography in the diagnosis of diffuse liver disease].

131 patients thought to have diffuse liver disease underwent ultrasonography and percutaneous liver biopsy. The ultrasonographic criteria examined were hepatic echogenicity compared to that of the renal cortex, homogeneity of hepatic parenchyma, and regularity of hepatic outline. On the basis of histologic examination of liver biopsies, several groups of pathologic lesions (not diagnostic entities) were established. Evaluation of ultrasound and histology was double blind. When the lobular architecture of the liver was respected histologically (normal liver, granulomatosis, siderosis, hepatitis), the ultrasound was normal in 86% of cases. The sensitivity of ultrasound was 0.9 for detection of fatty liver and 0.6 for cirrhosis. An abnormal ultrasound predicted structural modifications or a fatty liver in 93% of cases. Ultrasound proved incapable of differentiating between fatty liver and cirrhosis.

Biopsy↗

[A fatal disease of the heart in primary and secondary hemochromatosis].

In a 53 year-old man suffering from idiopathic hemochromatosis a massive myocardial siderosis occurred with disperse necroses and fibroses especially in subepicardial zone of the left ventricle. A similar picture was observed at secondary hemochromatosis in a 33 year-old woman with sideroblastic anemia after repeated blood transfusions. Both patients died of the failure of hypertrophic heart.

Adult↗

[Toxic retinopathies].

Among the drugs, which are topically administered, only epinephrine may produce retinal changes (macular oedema) in aphakic patients. Among the drugs, which are systemically administered, some immunodepressors, ethambutol, indomethacine, digitalis, but particularly chlorpromazine, thioridazine, quinine and chloroquine may produce toxic retinopathies. Concerning the intraocular metallic foreign bodies, siderosis is much more toxic for the retina than chalcosis.

Chloroquine↗

[Bioptic liver changes in brucellosis (author's transl)].

Histomorphological changes of the liver are demonstrated in 120 biopsy samples of 104 patients with clinically and serologically proven Bang's disease. A granulomatous hepatitis was found in 1/3 of the patients in addition to a non-specific mobilisation of the active mesenchyma and a reactive hepatitis of changing intensity. The nodules are not looking like tuberculous granulomas, but consist of a disseminated accumulation of small lympho-histiocytic cells. Furthermore, the relatively high percentage of steatosis of the parenchyma (50%), lipofuscinosis (40%), and circumscribed hepatocellular siderosis (26%) can be considered as important lesions of parenchymal cells.

Adolescent↗

Staining of minerals and solubility of iron in tissues.

Iron deposits in ethionine induced aortic siderosis of rats, in splenic deposits in sickle cell anemia and siderocalcific vessels in cerebral arteriosclerosis were completely removed by exposure to 20 percent hydrochloric acid for 30 min. This contrasted with idiopathic hemochromatosis and idiopathic pulmonary hemosiderosis in which the iron containing organs had to be exposed to 40 percent hydrochloric acid for two hours. The more soluble iron appeared colorless in unstained tissues, purplish blue with hematoxylin and eosin, turquoise blue with Perls' stain, violet blue with gallocyanin and dark-drown with sodium rhodizonate. The less soluble iron was golden yellow in unstained tissues. It appeared golden yellow with hematoxylin and eosin and sodium rhodizonate, but it stained greenish blue with Perls' method and dark brown with gallocyanin. Lead and copper were capable of deposition in some tissues in vitro in the presence of iron and/or calcium but not when these minerals were removed. This phenomenon may be of importance in certain pathological conditions, e.g. hemochromatosis, where on preexisting tissue-iron-complexes there is a secondary deposition of copper.

Anemia, Sickle Cell↗

Dissociation between hepatosplenic and marrow iron in liver cirrhosis.

Histochemical study of tissue iron in the various parts of the reticuloendothelial system in 15 subjects with cirrhosis was performed. Stainable iron in the liver and spleen sections, generally in large quantities, was found in 13 of 15 cases. Paradoxical association of hepatosplenic siderosis with depletion of marrow iron reserves was observed in seven subjects. In all seven, gross gastrointestinal blood loss had occurred during life and the source of bleeding from one or more anatomic lesions was identified at the time of autopsy. Depletion of marrow iron reserves in these seven subjects can be attributed to two factors: first, the available marrow iron stores were used for erythropoiesis; second, the ample hepatosplenic iron deposits could not be mobilized for transport to the bone marrow. Thus, hepatosplenic iron in cases of cirrhosis seems to be sequestered from its normal metabolic pathways.

Adult↗

The ocular effects of intralenticular iron foreign bodies in rabbits.

Optimal treatment of intralenticular metallic foreign-body injuries remains controversial, especially in patients with clear lenses and good vision. Using 20 rabbit eyes, we examined the cataractogenic potential of intralenticular iron and its effects on the retina. Iron wire implanted into the lenses of 12 rabbit eyes caused cataracts in every eye within 4 weeks. However, after 4 1/2 weeks, none of these eyes showed evidence of retinal siderosis. No cataracts developed in five eyes with intralenticular glass, while three with intravitreal iron showed marked retinal toxicity. These results suggest that as long as the lenses remain clear, conservative management of these injuries carries minimal risk of permanent retinal damage.

Animals↗

[Pathology of experimental hypocobaltosis in goats].

Histological changes in chronic experimental hypocobaltosis manifest as atrophic-degenerative alterations and restructuring of endocrine organs as well as a decrease in systemic resistance (accidental transformation of the thymus, duodenitis and sigmoiditis in some animals). Pronounced fat hepatosis and signs of iron metabolism damage (cheilitis, spleen siderosis) are found as well.

Animals↗

[Presence of aluminum and magnesium in the cerebral arteries and parenchyma of patients with striatonigral syndrome: study by Castaing's microprobe].

Electron probe microanalysis demonstrates the presence of aluminium, magnesium, iron, calcium, phosphorus in and around blood vessels in the pallidum (vascular siderosis) and in the putaminal parenchyma in five out of six cases of striatonigral degeneration, associated with orthostatic hypotension in two of these cases. These results suggest that striatonigral degeneration could be the result of a vascular disease, the result of an elemental intoxication of unknown cause.

Aluminum↗

[A case of acute pyrimethamine poisoning].

Intoxication by pyrimethamine is rare compare to these of patients who ingest a massive dose of amino-4-quinoleine. Clinic manifestations can be delayed and they are specially based on haematologic disorder as shown by literature data. Others organs can be concerned. The authors would like to present the case of a patient with a severe pancytopenia accompanied with: ocular blow like hyperhaemia of conjunctiva then iris siderosis. skin attack as Gũnther's disease. Etiologic treatment by folinic acid is the only deduction. Recovery is complete.

Acute Disease↗

Selective damage to the cerebellar vermis in chronic alcoholism: a contribution from neurotoxicology to an old problem of selective vulnerability.

The curiously consistent localization of cerebellar cortical damage in chronic alcoholism is re-evaluated in the light of selective damage, with a similar topography in the cerebellar vermal region, in superficial siderosis in man and in experimental animals exposed to certain toxic substances. Attention is drawn to the capacity for Purkinje cell dendrites and Bergmann glia to extract materials from the CSF, and to the close anatomical relationships of the susceptible lobules I-II, IX and X to the roof of the IVth ventricle and to the cistern of the great cerebral veins. This restriction of damage to vermis and paravermis may reflect some compartmentalization of CSF flow within leptomeninges, consistently increasing exposure of these cerebellar surfaces to materials circulating in the CSF. In other circumstances when this pattern of damage is encountered it raises the question as to whether other environmental agents, gaining access to the CSF, may be similarly distributed.

Alcoholism↗