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PubMed2006

Prevalence of growth and puberty failure with respect to growth hormone and gonadotropins secretion in beta-thalassemia major.

BACKGROUND: Present transfusion protocols have increased the life expectancy of patients with beta-thalassemia major, but siderosis is a major clinical complication of the treatment. Short stature and hypogonadism are extremely frequent in patients with thalassemia. To investigate the influence of age at the onset of blood transfusion, iron chelation therapy, and serum ferritin levels on growth and pubertal development in thalassemic patients, and the prevalence of these endocrine complications with respect to pituitary somatotropic and gonadotropic functions, this study was designed. METHODS: Clinical data of 158 patients (82 females and 76 males) with thalassemia major, aged 10 - 20 years (mean age: 15.1 +/- 4.8 years) were collected from a pediatric hematology clinic in Tehran. Height was measured and stages of puberty were determined by a pediatric endocrinologist. Serum ferritin concentration, liver function tests, serum calcium and phosphorus, blood sugar, free thyroxine (FT4), TSH, FSH, LH, dehydroepiandrosterone sulfate, testosterone (in boys), estradiol (in girls), and insulin-like growth factor I were measured after overnight fasting. In patients with a height more than 2 standard deviation below the mean, the growth hormone stimulation test was considered. Bone age was determined in all patients. RESULTS: Thalassemic patients in this study showed a high prevalence of short stature (62%) and hypogonadism (69%). We found a low serum level of gonadotropins (FSH and LH) in over 14-year-old patients with impaired puberty, which indicated that hypogonadotropic hypogonadism is responsible for this complication. Results of growth hormone provocative tests and serum insulin-like growth factor I levels in short stature patients showed a reduced growth hormone response in 38% and low insulin-like growth factor I levels in 42% of thalassemic patients. Short stature and hypogonadism were more common in patients with serum ferritin levels above 2000 microg/L and these complications were significantly more frequent in patients who started treatment later than that of the patients who started treatment in the first years of life (P < 0.001). CONCLUSION: Short stature and hypogonadism are extremely frequent in our patients with thalassemia, but correct blood transfusion and appropriate iron chelation therapy can prevent or limit these complications. These data support the need for vigilant follow-up of patients with thalassemia in order to treat endocrine dysfunction at an appropriate age.

Adolescent↗

PubMed1991

The effect of human recombinant erythropoietin on iron absorption and hepatic iron in a rat model.

In order to ascertain whether recombinant human erythropoietin (EPO) therapy would result in further intestinal iron absorption in the setting of systemic iron loading, iron absorption was measured in iron-loaded rats receiving EPO therapy and a control group of iron-loaded rats. Parenteral iron dextran (100 mg) resulted in hepatic siderosis with predominantly a reticuloendothelial distribution. EPO was given by intraperitoneal injection (100 U/kg) for 10 days. Radioiron absorption was measured by total body counting. Iron absorption in EPO-treated rats was 4.8 +/- 2.0% (n = 12) and 4.5 +/- 2.2% (n = 10) in the control rats (p greater than 0.05). Mean hemoglobin in the EPO rats was 201 +/- 12.5 g/L and 140 +/- 18.2 g/L in the control rats (p less than 0.001). Mean hepatic iron concentration was 73 +/- 16 mumol/g in the control rats and 34 +/- 9.1 mumol/g in the EPO-treated rats (p less than 0.001). This study suggests that iron-loaded rats do not demonstrate an increase in intestinal iron absorption with EPO therapy despite a significant erythropoiesis. The reduction in hepatic iron concentration suggests that reticuloendothelial iron is accessible and mobilized to produce new red blood cells.

Animals↗

PubMedDate not supplied

[Pulmonary adiaspiromycosis].

A 54-yr old male patient with a history of dyspnea and cough is presented. Due to the clinical course of disease and the radiological changes in the chest a diagnosis of sarcoidosis was established. However, the open lung biopsy revealed the true nature of the pulmonary disease: pulmonary adiaspiromycosis, only secondary to asbestosis, siderosis and silicosis as due to the well known occupational exposure to asbestos and other dusts.

Chrysosporium↗

PubMed1991

[Peripheral spontaneous remission of hairy cell leukemia following transfusion-associated hepatitis C].

In a 59-year-old patient presenting in October 1981 with pancytopenia, hairy cell leukemia was diagnosed. Splenectomy, followed by treatments with oncovin, lithium, and prednisone were essentially without effect. Up to July 1984 the patient had been regularly transfused with a total of 62 unit. In June 1984 he acquired a transfusion associated hepatitis C which followed a chronic course and resulted in biopsy proven cirrhosis in 1989. The patient became independent of transfusions in July 1984. Repeated blood counts have shown a complete hematologic remission which has now lasted nearly 6 years, whereas focal leukemic infiltrates have persisted in the bone marrow. The patient has tolerated 20 courses of erythropheresis performed because of biopsy proven severe hepatic siderosis without a fall in hemoglobin. It is suggested that the spontaneous long-lasting hematologic remission of hairy cell leukemia is due to endogenous interferon produced in the course of chronic hepatitis C. Low serum levels of interferon-alpha and -gamma were detected.

Combined Modality Therapy↗

PubMed1991

Comparative iron mobilizing actions of deferoxamine, 1,2-dimethyl-3-hydroxypyrid-4-one, and pyridoxal isonicotinoyl hydrazone in iron hydroxamate-loaded mice.

A comparison was made of the actions of deferoxamine (DFX), 1,2-dimethyl-3-hydroxypyrid-4-one (L1), and pyridoxal isonicotinoyl hydrazone (PINH) in mobilizing and promoting excretion of iron in mice loaded with iron-acetohydroxamic acid complex. DFX was given ip, while L1 and PINH were given po. Each was given daily for four days at 300 mg/kg/day, and total excreta were collected 24 hr after each administration. Total iron excreted over the 4-day period, expressed as micrograms/mouse, were: Controls, 26; PINH-treated, 31; DFX-treated, 162; and L1-treated, 208. Measurements of iron in selected organs 96 hr after the last administration of each compound revealed that treatment with L1 and DFX induced significant reductions of iron concentrations in kidneys (16% and 17%, respectively) and in pancreas (18% and 19%, respectively). In addition, L1 treatment led to a significant reduction in the liver iron burden (11%), an action not seen after treatment with DFX. None of the compounds reduced iron concentrations in heart, the most critical organ for toxicity of transfusional siderosis. The synthetic routes for preparation of L1 and PINH are described in detail.

Animals↗

PubMed1990

Dietary iron overload in southern African rural blacks.

A survey conducted in rural southern African black subjects indicated that dietary iron overload remains a major health problem. A full blood count, erythrocyte sedimentation rate, serum concentrations of iron, total iron-binding capacity, ferritin, C-reactive protein (CRP), gamma-glutamyltransferase (GGT) and serological screening for hepatitis B and human immunodeficiency virus (HIV) infections were carried out in 370 subjects (214 inpatients and 156 ambulatory Mozambican refugees). The fact that the geometric mean (SD range) serum ferritin concentration was much higher in the male hospital patients than in subjects living in the community [1,581 micrograms/l (421-5,944 micrograms/l) and 448 micrograms/l (103-1,945 micrograms/l) respectively] suggested that dietary iron overload was not the only factor raising the serum ferritin concentration. The major additional factor appeared to be inflammation, since the geometric mean (SD range) serum CRP was significantly higher in male hospital patients [21 mg/l (8-53 mg/l)] than in subjects in the community [3 mg/l (1-5 mg)]. Alcohol ingestion, as judged by history and by serum GGT concentrations, was also associated with significantly raised serum ferritin concentrations. This finding was ascribed to the fact that traditional brews are not only associated with alcohol-induced hepatic damage but are also a very rich source of highly bio-available iron. The role of iron overload in the genesis of the raised serum ferritin concentrations are confirmed in the diagnostic liver biopsy study. The majority of biopsies showed heavy siderosis, with varying degrees of hepatic damage.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1990

Metal toxicity and the respiratory tract.

The type of lung disease caused by metal compounds depends on the nature of the offending agent, its physicochemical form, the dose, exposure conditions and host factors. The fumes or gaseous forms of several metals, e.g. cadmium (Cd), manganese (Mn), mercury (Hg), nickel carbonyl (Nl(CO)4, zinc chloride (ZnCl2), vanadium pentoxide (V2O5), may lead to acute chemical pneumonitis and pulmonary oedema or to acute tracheobronchitis. Metal fume fever, which may follow the inhalation of metal fumes e.g. zinc (Zn), copper (Cu) and many others, is a poorly understood influenza-like reaction, accompanied by an acute self-limiting neutrophil alveolitis. Chronic obstructive lung disease may result from occupational exposure to mineral dusts, including probably some metallic dusts, or from jobs involving the working of metal compounds, such as welding. Exposure to cadmium may lead to emphysema. Bronchial asthma may be caused by complex platinum salts, nickel, chromium or cobalt, presumably on the basis of allergic sensitization. The cause of asthma in aluminium workers is unknown. It is remarkable that asthma induced by nickel (Ni) or chromium (Cr) is apparently infrequent, considering their potency and frequent involvement as dermal sensitizers. Metallic dusts deposited in the lung may give rise to pulmonary fibrosis and functional impairment, depending on the fibrogenic potential of the agent and on poorly understood host factors. Inhalation of iron compounds causes siderosis, a pneumoconiosis with little or no fibrosis. Hard metal lung disease is a fibrosis characterized by desquamative and giant cell interstitial pneumonitis and is probably caused by cobalt, since a similar disease has been observed in workers exposed to cobalt in the absence of tungsten carbide. Chronic beryllium disease is a fibrosis with sarcoid-like epitheloid granulomas and is presumably due to a cell-mediated immune response to beryllium. Such a mechanism may be responsible for the pulmonary fibrosis occasionally found in subjects exposed to other metals e.g. aluminium (Al), titanium (Ti), rare earths. The proportion of lung cancer attributable to occupation is around 15%, with exposure to metals being frequently incriminated. Underground mining of e.g. uranium or iron is associated with a high incidence of lung cancer, as a result of exposure to radon. At least some forms of arsenic, chromium and nickel are well established lung carcinogens in humans. There is also evidence for increased lung cancer mortality in cadmium workers and in iron or steel workers.

Asthma↗

PubMed1979

The sensitivity and heterogeneity of histochemical markers for altered foci involved in liver carcinogenesis.

Subcutaneous injection of iron dextran resulted in a hepatic siderosis within 2 weeks in rats, as previously reported for mice. Hepatic carcinomas as well as neoplastic nodules in rats were entirely or mainly free of stainable iron and, thus, could be readily identified histologically. In addition, early carcinogen-induced altered foci were resistant to iron accumulation. In rats fed 0.02% N-2-fluorenylacetamide (FAA) for 13 weeks, the number of iron-resistant foci identified following iron injection was the same as that observed with dietary iron overload. Histochemical investigation of enzymatic markers that have been used to identify foci in rats revealed that foci characterized by enzymatic reactions of positive gamma-glutamyl transpeptidase and decreased adenosine triphosphatase and glucose-6-phosphatase corresponded to those characterized by resistance to iron accumulation. However, in quantitative analysis of the early carcinogen-induced foci in rats given iron dextran following a diet containing 0.02% 2-FAA for 13 weeks, more lesions were detected by resistance to iron accumulation than by any of these other properties. There was considerable phenotypic heterogeneity among foci for the enzyme markers. It is concluded that resistance to iron accumulation is a more sensitive and reliable marker for early carcinogen-induced altered hepatocellular foci than is any other histochemical property.

2-Acetylaminofluorene↗

PubMed1978

[The different effects of fe(II)- and fe(III)-ions on the hyaluronic acid of the vitreous body (author's transl)].

Fe(II)-ions reduce the viscosity of a hyaluronic acid solution but do not form a precipitate. Fe(III)-ions only slightly lower the viscosity of hyaluronic acid solutions but cause a brown, water-insoluble iron-hyaluronic acid precipitate. After adding ascorbic acid--concentrate the pH-value as in vitreous body--to the reaction solution, Fe(III)-ions reduce the viscosity too. The iron-hyalurnic acid complex is also destroyed by ascorbate. Therefore, the ascorbate of the vitreous humor plays an important role in the generation of siderosis bulbi.

Animals↗

PubMed1989

[The diagnostic value of CT in siderophilia (primary idiopathic hemochromatosis)].

CT, due to its high resolution of density, is suitable, within certain limits, to quantify iron contents in the liver. - Diagnostic value and limits of dual-energy-computed tomography after 38 examinations, 31 of them in patients with verified haemochromatosis, are described: In a progressive state of iron overload a high correlation exists between results of computed tomography and usual reference methods of iron metabolism (iron determination by absorption spectrometry and histologic examination, serum-iron-level, serum-ferritin-level, relative transferrin saturation, desferal test). - With a specificity of nearly 100% CT shows a minor or medium iron overload only with low sensitivity. - Because of its noninvasive nature CT is an alternative method of biopsy in progressive liver disease with suspected hepatic siderosis.

Fatty Liver↗

PubMed1989

[Experimental studies on the role of retinal lipid peroxidation induced by intraocular iron foreign body].

Experimental studies were made to evaluate the role of lipid peroxidation in retinal damage by intraocular iron foreign bodies. It was found that after iron particles were implanted into the vitreous bodies (1) iron concentration in the aqueous humour rose rapidly and significantly (n = 24, P less than 0.01), (2) malondialdehyde (MDA) a degradation metabolite of lipid peroxidation, in the retinas increased significantly (n = 24, P less than 0.05), and (3) superoxide dismutase (SOD) activity decreased (n = 11, P less than 0.05). These findings suggest that lipid peroxidation plays a key role in the retinal damage of siderosis bulbi, and provide a solid basis in studying the pathogenesis and guiding clinical management of the disease.

Animals↗

PubMedDate not supplied

[Interactions of alcohol and iron proteins].

The known relationship between ethanol and the two main proteins of iron metabolism, transferrin and ferritin, are reviewed. Transferrin synthesis decreases in alcoholic cirrhosis, and increases in alcoholic fatty liver. In the latter case, its turnover is accelerated. Serum desialylated transferrin increases in chronic alcoholism and could be the best marker of heavy drinking. The increased uptake of desialylated transferrin by the liver could explain the development of hepatic siderosis in some alcoholics. Serum ferritin increases in chronic alcoholism, much more because of liver damage than in relation to iron stores. It is clear in this review that few experimental studies have been interested in the investigation of these relationships.

Alcoholism↗

PubMed1989

Neonatal hemochromatosis. The regulation of transferrin-receptor and ferritin synthesis by iron in cultured fibroblastic-line cells.

The authors have investigated the hypothesis that neonatal hemochromatosis (NH), a generally fatal disease of infancy, is due to abnormalities in cellular response to ambient levels of iron. The clinical and necropsy findings in two infants with NH, the results of evaluations for iron-storage disease in their first-degree relatives, and the results of the authors' studies of ferritin and transferrin-receptor (TfR) synthesis in NH and normal fibroblasts are presented. No differences between cultured skin fibroblasts from a normal infant and similar cells from the two infants with NH were seen with respect to TfR and ferritin synthesis rates or their modulation by iron. NH and adult idiopathic hemochromatosis (AH) share a pattern of siderosis in which epithelial and mesenchymal elements contain large quantities of stainable iron, while reticuloendothelial elements contain almost none. Although no familial correlation between NH and AH has been established, and none appeared to exist in these two families, the authors' results parallel those of previous studies of various cell types from persons with AH. The abnormalities in cellular iron handling, undefined at present, that are associated with the phenotype common to NH and AH do not appear primarily to involve the regulation by iron of rates of TfR and ferritin synthesis.

Cell Line↗

PubMed1986

Lymphangioleiomyomatosis. A case report with an immunohistochemical study.

A case of pulmonary lymphangioleiomyomatosis is presented, which occurred in a 50-year-old woman suffering from severe dyspnea of 7 year's duration and with a previous history of diseases (polycystic ovaries and endometrial adenocarcinoma) usually related to hyperestrogenism. The patient showed no signs or symptoms of tuberous sclerosis, a condition often associated with pulmonary lymphangioleiomyomatosis. Both the radiological pattern and histological findings had previously been interpreted at other hospitals as consistent with either nodular interstitial fibrosis with emphysematous changes or pulmonary siderosis. Correct diagnosis was suspected on critical review of histological slides and definitely achieved by immunohistochemistry in routine sections, which showed the presence of desmin in the elements constituting the nodular lesions scattered throughout the parenchyma. The criteria for the differential diagnosis are discussed, as well as the role of immunohistochemistry in assessing the muscular nature of proliferating cells.

Desmin↗

PubMed1988

Pathologic findings in 76 autopsy cases of thalassemia.

Pathologic findings in six beta-thal major, 58 beta-thal Hb E, and 12 Hb H patients were presented. The causes of death were mainly infection in the first and second decades and cardiac failure in older patients. There was accelerated intramedullary and extramedullary erythropoiesis, with a few cases showing focal fibrosis, some with decreased cellularity and occasional atypia. Iron deposition mirrored that of thal patients in the European and American literature, apart from minimal cardiac siderosis, and the correlation between the amount of iron deposited and dysfunction of the particular organ was not always apparent. Patients with Hb H disease showed a less extensive iron deposition and organ pathology than those with the other two types of thalassemia.

Adolescent↗

PubMedDate not supplied

Sinus histiocytosis massive lymphadenopathy syndrome: histogenesis of the hepatic lesion.

Sinus histiocytosis massive lymphadenopathy (SHML) syndrome with hepatic involvement, occurring in a seven-year-old black female, is reported. Morphologic characterization of the hepatic lesion is accomplished utilizing conventional light, fluorescent and electron microscopy, and histochemical techniques and by comparing and contrasting the findings with those in cases of familial erythrophagocytic lymphohistiocytosis (FEL) and virus-associated hemophagocytic syndrome (VAHS). The histiocytic proliferation in the liver in SHML differs by showing: (a) an intralobular distribution with portal sparing; (b) marked steatosis; and (c) lipofuscinosis. The aforesaid intralobular distribution and the accompanying hypertrophy and hyperplasia of Kupffer cells, as well as commonalities of steatosis and lipofuscinosis, and, to a lesser extent, erythrophagocytosis and siderosis, suggest a histogenesis from Kupffer cells. The histochemical finding of fatty acid peroxides and both fluorescent microscopic and histochemical evidence of lipofuscin inclusions, a by-product of lipid peroxidation, in Kupffer cells provide at least a theoretical basis for both erythrophagocytosis and proliferation eventuating in intralobular histiocytosis.

Child↗

PubMed1987

[Are any drug admixtures except iron preparations required in the treatment of iron deficiency anemia?].

The aim was to estimate the efficacy of multimodality therapy of iron deficiency anemia with vitamins combined with iron salts and to compare the results of this therapy with that including iron salts only. Besides the results of therapy using iron preparations administered per os and in injections were compared. Altogether 250 women were entered in this study. Chronic posthemorrhagic iron deficiency anemia was diagnosed in 175 patients, in 75 iron deficiency anemia developed against a background of pregnancy 250 women were divided into 10 groups. The last 3 groups included pregnant women. The selection in the first 7 and last 3 groups was randomized. The rate of increment of hemoglobin, erythrocytes and serum iron did not differ in the groups of patients receiving in addition to iron vitamins B12 and B6, copper sulfate, the combination of iron with the vitamin B group and copper sulfate. In the group receiving iron for injection the rate of increment of hemoglobin did not almost differ from that in the study groups, however it was slightly higher. Similar results were obtained for the pregnant women. Subsequent therapy after hemoglobin normalization was shown to make therapeutic results more stable. A conclusion was made of inappropriateness of the use of iron combined with other preparations in view of numerous cases of side-effects and danger of the development of siderosis of internal organs as a result of erroneous drug administration. The use of iron preparations for injections was recommended only in disturbed intestinal absorption of iron.

Adolescent↗

PubMed1978

Spontaneous extrusion of intraocular foreign body after 11 years.

A case of spontaneous extrusion of intraocular foreign body after 11 years is presented. Despite being magnetic and containing mostly iron the foreign body could not be removed in repeated magnet trials. It was retained in the ciliary body region for a very long time without causing siderosis. The foreign body was spontaneously extruded without any signs of intraocular inflammation.

Adolescent↗