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PubMedDate not supplied

[Disulone and hepatosiderosis].

BACKGROUND: Disulone (dapsone + iron oxalate) is a sulfone used in the treatment of numerous skin diseases. We report two cases of hepatosiderosis secondary to long-term administration of Disulone. PATIENTS AND METHODS: Case n degrees 1. A 51-year-old man was treated with Disulone for a neutrophilic skin disease. After 17 years of treatment, elevated serum ferritin and free iron with hemolysis were found. Liver biopsy confirmed hepatosiderosis. A diagnosis of genetic hemochromatosis was ruled out by the absence of C282Y mutation of the HFE gene. Case n degrees 2. A 52-year-old man receiving Disulone for dermatitis herpetiformis for 25 years presented elevated serum ferritin and free iron with hemolysis. Hepatic iron overload was confirmed by liver biopsy. The absence of C282Y mutation (HFE gene) ruled out a diagnosis of genetic hemochromatosis. DISCUSSION: In our two cases, hepatosiderosis was noted after long-term administration of Disulone. This complication has been reported only rarely. In murine models, a relationship was found between prolonged administration of dapsone and hepatic iron overload as revealed by hemolysis. Although it is difficult to extrapolate this relationship to humans with any certainty, our patients had also chronic hemolysis and iron overload secondary to administration of Disulone. Moreover in France, dapsone is marketed in combination with iron oxalate, with the attendant risk of iron overload. These cases raise the question of the need for serum ferritin analysis during Disulone therapy.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1983

Corneal epithelial iron deposition.

Different patterns of iron deposition in the epithelium in 96 of 120 corneal buttons that had superficial irregularities of different causes, as examined after staining grossly with Prussian blue by Gomori's method were detected. These observations were confirmed by light microscopy in 86 cases. The deposition was either diffuse or it formed a linear pattern within the epithelium adjacent to elevated areas of corneal scarring. The authors believe that the tear film is the source of iron in these cases, and that relative pooling of tears on an irregular surface is the mechanism by which iron lines are formed.

Cornea↗
PubMed2002

Hydroxychloroquine ototoxicity in a child with idiopathic pulmonary haemosiderosis.

Hydroxychloroquine, a quinoline compound, rarely causes ototoxicity. According to the few reports in existence, hydroxychloroquine-induced ototoxicity occurred following prolonged therapy in adult patients with rheumatoid arthritis and lupus erythematosus. We report a case of unilateral sensorineural hearing loss in a 7-year-old girl with idiopathic pulmonary haemosiderosis, after 2 years of hydroxychloroquine treatment. Sensorineural hearing loss has previously been reported with hydroxychloroquine treatment, but this is the first report in a child and associated to idiopathic pulmonary haemosiderosis and has the characteristic of being unilateral.

Acoustic Impedance Tests↗
PubMed1990

Recombinant human erythropoietin and phlebotomy in the treatment of iron overload in chronic hemodialysis patients.

Five long-term hemodialysis patients with clinical iron overload were treated with 300 U/kg of recombinant human erythropoietin (rHuEPO) intravenously (IV) after each hemodialysis. The patients were phlebotomized after each hemodialysis at any time the predialysis hematocrit was 35% or greater. Over a period of 1 year, the average phlebotomy rate varied from 0.5 to 1.1 U/wk with a mean phlebotomy rate of 45.8 +/- 5.6 U/yr (range, 27 to 57 U). The mean serum ferritin decreased from 8,412 +/- 1,599 micrograms/L (ng/mL) to 3,007 +/- 1,129 micrograms/L (ng/mL), and the mean iron removal over this period was 9.5 g. Liver iron deposition, as measured by density on computed tomographic (CT) scan, improved, while skin color lightened significantly. Patients tolerated phlebotomy with no major symptoms or complications and exhibited no change in the hemogram or serum chemistries. In patients with severe iron overload, changes in serum ferritin with erythropoietin treatment alone may not reflect true change in iron burden. Use of high-dose erythropoietin and phlebotomy is an effective and safe (at least for 1 year) method of reducing iron overload in long-term hemodialysis patients.

Adult↗
PubMed1991

Pulmonary hemorrhage and necrotizing glomerulonephritis without glomerular immune deposits: report of two cases.

Two children with a syndrome of pulmonary hemorrhage and necrotizing, nonimmune glomerulonephritis are reported. A boy and girl, both of East Indian descent, developed recurrent lung infiltrates from the age of 3 months and 2 years, respectively. Both subsequently presented with pulmonary hemorrhage, fever, arthritis, hematuria, and nephrotic range proteinuria at 1.5 and 6 years of age, respectively. Renal biopsy in each case showed acute, severe, focal and segmental, necrotizing and crescentic glomerulonephritis without immune deposits. Subsequent renal biopsies revealed severe glomerular and tubulointerstitial scarring. No vasculitis or granulomas were seen on renal, skin, or lung biopsies. Antineutrophil cytoplasmic antibodies (ANCA) were not detected in sera taken 1.5 years in the boy and 3.5 years in the girl after the onset of renal disease. The boy was treated with prednisone, azathioprine, and plasmapheresis, but developed progressive renal impairment and commenced dialysis within 4 years. Subsequent to renal transplantation, he developed an immunoblastic lymphoma and died. The girl was treated initially with prednisone and later with cyclophosphamide. After 4 years, she had a normal glomerular filtration rate (GFR). While necrotizing nonimmune glomerulonephritis associated with pulmonary hemorrhage is rare, and cases are characteristically difficult to classify because of many overlapping features, it is possible that these children had a unique illness.

Child↗
PubMed1987

Iron overload and mobilization in long-term hemodialysis patients.

Iron overload from repeated transfusions of RBCs in long-term hemodialysis patients is a problem of increasing clinical significance. We report on the prevalence of and diagnostic criteria for identification of hemodialysis patients with iron overload. In 150 unselected hemodialysis patients, 62 (41%) had ferritin levels greater than 2,000 ng/mL (normal = 10 to 360 ng/mL). In 16 of these patients, accurate transfusion histories were obtained and ferritin levels correlated with calculated transfusional iron burden (r = 0.553, P less than .05). These patients could be divided into two distinct groups on the basis of their response to a single dose (2 g, IV) of deferoxamine: "high" responders had twice the level of feroxamine (the chelated product of deferoxamine and iron) of the "low" responders (P less than .001). High responders also had significantly higher prevalence of the "hemochromatosis" alleles A3, B7, and B14 than a large group of dialysis patients awaiting transplantation (71% v 37%, P less than .001). In two patients with iron overload and clinically significant bone disease, bone histology revealed prominent iron staining at the calcification front. We conclude that transfusional iron overload is a significant clinical problem in long-term hemodialysis patients, that may also be associated with bone pathology.

Adult↗
PubMedDate not supplied

Serum histamine-releasing activity in a patient with idiopathic pulmonary haemosiderosis.

BACKGROUND: idiopathic pulmonary haemosiderosis (IPH) is a rare disorder characterized by intermittent, diffuse alveolar bleeding. The pathogenesis of the diseases is unclear, although an association with milk or gluten hypersensitivity has been described, and an immune-mediated damage of alveolar capillaries has been suggested. A previous report showed the release of histamine after cow's milk intake in a newborn with cow's milk intolerance and IPH. METHODS AND RESULTS: here, we report the detection of serum histamine-releasing activity (HRA) in a 30-year-old woman with IPH. The serum taken during an active phase of the disease induced histamine release from basophils of two normal donors; conversely, when the patient was receiving prednisone and azathioprine, and the disease was in remission, the serum HRA was reduced. Serum fractions with a MW lower than 100 kDa displayed an enhanced HRA; in contrast, serum fractions with MW above 100 kDa were not able to induce histamine release, suggesting that the activity was due to a cytokine and not to an immunoglobulin. CONCLUSIONS: the detection of serum HRA provides further evidence that the immune system is activated in the course of IPH and supports an immunologic basis for the alveolar capillary damage, which is responsible for alveolar bleeding.

Adult↗
PubMed1997

Supratentorial cavernous malformations and epilepsy: seizure outcome after lesionectomy on a series of 35 patients.

Epilepsy is the most frequent presenting sign in patients with cavernous angiomas and is the major cause of morbility. Persistence of seizures after surgical treatment prompted many authors to examine the possibility of removing the cavernoma and the surrounding tissue. In our series of 53 cavernous angiomas, all the 35 patients with preoperative seizures underwent surgery by means of lesionectomy alone. One hundred percent of patients with less than five preoperative seizures and/or an history under 12 months was seizure free, while only 62.5% of patients with more than five seizures and/or an history longer than 12 months was seizure free. Number and duration of seizures before surgery seems to be the most important factor in the seizure outcome after surgical treatment.

Adolescent↗
PubMed1997

[Repeated rupture of the extensor tendons of the hand due to fluoroquinolones. Apropos of a case].

Fluoroquinolone toxicity on cartilages and tendons has been well known since 1983. Tendon inflammation or rupture has been described. Achilles tendon rupture is the most frequent complication but many other sites of tendon injuries have been reported. This article presents a case of rupture of extensor tendons of the hand in an elderly woman treated by fluoroquinolones. As far as we know, this site of tendon lesion has never been previously described. Histological examination of tendon injuries was possible after surgical treatment. Histological structures were similar to the classical description but had specific features. Like other authors, we think that the mechanism of the disease involves vascular disorders as well as direct toxicity. The histological lesions seem to be different in chronic and acute forms.

Acute Disease↗
PubMed1988

Sicca syndrome with iron deposition in the salivary glands.

A case of iron deposition in the labial accessory salivary glands associated with prominent xerostomy and xerophtalmy is reported. Suffering from a myelodysplastic syndrome with refractory anaemia, this patient received multiple transfusions over the past 7 years. A transfusional haemosiderosis gradually developed. Histopathologic examination of accessory salivary glands demonstrated haemosiderin deposition in the serous alveoli and in the epithelial cells of intercalated and interlobular ducts. A relationship between the iron deposition in the salivary glands and the development of the sicca syndrome is suggested. The literature related to iron deposition in salivary glands and sicca syndrome is reviewed.

Anemia, Refractory↗
PubMed1994

Clinical manifestations and therapy of transfusional haemosiderosis.

Long-term blood transfusions lead to the accumulation of iron that in the absence of chelation therapy causes complications such as liver cirrhosis, growth failure, hypogonadism, hypothyroidism, hypoparathyroidism, diabetes and myocardiopathy. The last still represents the most frequent cause of death in haemosiderotic transfusion-dependent patients. At the moment the only chelator widely used is desferrioxamine (DFX). The drug works best when administered as a continuous infusion, mainly by the subcutaneous route. To patients with severe iron overload, impending organ failure, or poor compliance to chelation, DFX can be administered intravenously, through an externalized central catheter or, preferably, a subcutaneous port. Several studies have shown the effectiveness of DFX in reducing the iron burden, thus preventing the complications, once considered inevitable, of iron overload, and even in reverting some, but not all, of the iron-induced dysfunctions. Practical and psychological support are necessary to ensure satisfactory compliance with a therapy that is cumbersome and difficult. Toxic effects of DFX such as growth failure, hearing impairment and bone abnormalities seem to occur mainly in patients who have received high doses of DFX despite a low iron burden. Visual loss and renal and pulmonary toxicities, on the contrary, seem to be more directly related to high DFX peak doses administered irrespective of the patient's amount of iron overload. After bone marrow transplantation, phlebotomy or erythrocytoapheresis might be necessary to reduce further the iron accumulated during years of transfusions.

Adolescent↗
PubMed1989

Results of long-term subcutaneous desferrioxamine therapy.

Regular subcutaneous desferrioxamine therapy has prolonged the life and reduced the incidence of cardiac, endocrine and liver complications of iron overload in patients with thalassaemia major and other refractory transfusion-dependent anaemias. Its full impact will be apparent only when patients who began this treatment as children 10 years ago reach adult life. The major problems with s.c. DF therapy are its cost and the difficulty of patient compliance with a painful and cumbersome method of administration. Attempts to maintain normal iron stores in these patients has resulted in significant toxicity caused by the excessive use of DF. Although recent studies have shown that long-term subcutaneous calcium diethylene triamine penta-acetic acid (Ca-DTPA) can be used as an effective alternative to s.c. DF (Wonke et al, 1988), this treatment presents additional potential problems of zinc depletion which can be prevented only by careful management. The prospects of an inexpensive, orally active iron chelator are now real (Kontoghiorghes and Hoffbrand, 1988) and it is to be hoped that long-term results at least as good as those with s.c. DF will be achievable. Such oral chelators may be available to a far wider group of patients, than s.c. DF, and the problems of cost and compliance may be diminished.

Deferoxamine↗
PubMed1989

Diagnosis and clinical evaluation of iron overload.

Diagnostic evaluation of the various forms of iron overload requires information about the total amount and distribution of iron stores. Direct information on the quantity of storage iron can be obtained only by its mobilization in response to repeated phlebotomy or after dilution of a labelled iron test dose in the total body iron pool. Both approaches are cumbersome and time-consuming and are suitable only for research purposes. Detailed information on the amount and distribution of tissue iron in iron overload can be obtained from biopsy specimens of the major iron storage organs such as the liver and bone marrow. However, the invasive nature of these procedures limits their clinical usefulness. Indirect measures, such as serum iron concentration, TIBC saturation, serum ferritin, chelate-induced urinary iron excretion or intestinal iron absorption, and ferrokinetic measurements may provide useful information on the amount of total body iron reserve. However, they all have important limitations in their diagnostic use for evaluating iron overload. The most suitable indirect storage iron index among these methods is the ferritin assay, which has a well established place in the diagnosis of iron overload and monitoring of the effect of therapy. Recent developments in physical methods such as CT, SQUID and NMR have significantly improved the applicability of these techniques for non-invasive measurement of liver iron. It is expected that quantitative measurement of hepatic iron stores will soon be integrated into the diagnostic procedures available by imaging techniques such as CT and NMR. In combination with screening parameters such as serum ferritin and TIBC saturation these new but expensive diagnostic tools may simplify and shorten the diagnostic process and may also be useful for monitoring the treatment of iron overload by phlebotomy or chelating drugs.

Biopsy↗
PubMed1989

The clinical manifestations of chronic iron overload.

Primary or genetic haemochromatosis is an inherited disease characterized by an inappropriate degree of iron absorption with accumulation of excessive amounts of tissue iron. Parenchymal iron accumulation results in the typical clinical features of the disease including hepatic cirrhosis, diabetes, testicular atrophy and skin pigmentation. The disease is inherited in an autosomal recessive manner. The gene for the disease has not been identified but is tightly linked to the A locus of the histocompatibility complex on chromosome 6. The approximate homozygote frequency in Caucasians is 0.3% with an equal sex ratio. Excessive body iron stores have been described in a number of other conditions, particularly alcoholic liver disease. There is increasing evidence that many of these individuals are in fact also suffering from genetic haemochromatosis. Diagnostic tests including serum iron, transferrin saturation, serum ferritin and liver iron concentration make it possible to detect sufferers of the disease. Screening relatives of affected individuals with these tests allows a diagnosis to be made before permanent tissue damage has occurred. Removal of excess iron stores by repeated phlebotomy is the primary treatment. If iron is removed before significant tissue damage has occurred, the complications and natural course of the disease will be prevented provided reaccumulation of iron does not occur. Excessive iron accumulation with resultant organ damage also occurs in anaemias associated with ineffective erythropoiesis and after excessive parenteral iron administration or repeated blood transfusions. Similar clinical features may be seen. Chelation therapy is the mainstay of treatment in these cases where long-term venesection is not possible.

Bloodletting↗
PubMed1989

Ultrastructural pathology of iron overload.

The study of the ultrastructural changes in the iron-laden organism has indicated the presence of a number of 'defensive' features, best understood if examined according to a concept of biological levels. At the molecular level, the main features are: the increased capacity of cells to bind toxic, inorganic iron to a specific storage protein, apoferritin, which becomes visible due to its iron-containing, electron-opaque core; since iron itself is involved in the de-repression of apoferritin synthesis, the number of assembled ferritin molecules depends on the amount of unbound iron present in the cell; there is a maximal, cell line-specific concentration of cytosolic ferritin; ferritin particles have a variable iron content, with richer molecules having a tendency to form clusters. At the cellular level, the transport of ferritin into the lysosomal compartment with formation of ferritin and haemosiderin-containing siderosomes enables further segregation of iron and permits cell survival even in the new steady-state of cytosiderosis. When siderosomes increase beyond a cell line-specific concentration, signs of organelle alteration followed by cellular death are noted. At the tissue level, the contributory ultrastructural observations include finding of early intercellular fibrosis, atypical (amorphous) iron deposition, as well as the accessibility of a detailed assessment of iron distribution in various cell types, i.e. endothelium, parenchymal cells, RE cells. The ultrastructural study of material obtained from human subjects with various stages of iron overload and of experimental animals facilitates the understanding of the process of iron overload itself and of the ensuing cellular damage. The recent emphasis on iron as a putative contributory factor in infections, as well as its role in neoplasia, has provided new directions for research, both clinical and experimental. Ultrastructural observations, combined with biochemical, immunological and biophysical investigations, are mandatory for providing answers to the numerous pending questions related to iron metabolism. Sequential electron microscopic studies of iron-laden cells enable the evaluation of chelating agents in either clinical or experimental conditions.

Ferritins↗