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Sample records for hemosiderosis

  1. Neonatal Pulmonary Hemosiderosis

    Boris Limme

    2014-01-01

    Full Text Available Idiopathic pulmonary hemosiderosis (IPH is a rare complex entity characterized clinically by acute or recurrent episodes of hemoptysis secondary to diffuse alveolar hemorrhage. The radiographic features are variable, including diffuse alveolar-type infiltrates, and interstitial reticular and micronodular patterns. We describe a 3-week-old infant presenting with hemoptysis and moderate respiratory distress. Idiopathic pulmonary hemosiderosis was the first working diagnosis at the Emergency Department and was confirmed, 2 weeks later, by histological studies (bronchoalveolar lavage. The immunosuppressive therapy by 1 mg/kg/d prednisone was immediately started, the baby returned home on steroid therapy at a dose of 0,5 mg/kg/d. The diagnosis of idiopathic pulmonary hemosiderosis should be evocated at any age, even in the neonate, when the clinical presentation (hemoptysis and abnormal radiological chest images is strongly suggestive.

  2. [Marked hemosiderosis in myelodysplastic syndrome].

    Klinz, C

    1999-01-29

    A 68-year-old man was admitted because of symptoms of lumbar pain. He was known to have chronic anemia with ring sideroblasts and diabetes melitus and to be in heart failure. Three months before he had been given 7 units of red cell concentrate. On admission the outstanding features were brown discoloration of the skin, absent body hair, tachycardia, hepatomegaly and small testicles. He had a normocytic anemia, hyperglycemia and raised transaminases, hypogonadism and vitamin D3 deficiency. The serum levels of iron, transferrin saturation and feritin were markedly elevated. Liver iron content/g dried liver was 4.2 g (by biomagnetometer). Radiology of the lumbar vertebrae showed osteoporosis and sonography confirmed hepatomegaly. The known myelodysplastic syndrome (MDS) had fed to secondary hemosiderosis with heart failure, liver involvement, diabetes mellitus, hypogonadism and osteoporosis. Symptomatic treatment was unsuccessfully complemented by desferoxamine (up to 4 g/12 h) to release iron. But very good iron excretion was then achieved with deferiprone (3 x 1 g/d). The patient later died of the sequelae of hemosiderosis. Even when they have not required transfusions, patients with long-standing MDS should be examined regularly for the possible development of secondary hemosiderosis so that iron-chelating agents can be administered as needed.

  3. Longitudinal analysis of hearing loss in a case of hemosiderosis of the central nervous system

    Weekamp, H H; Huygen, P L M; Merx, J L; Kremer, H P H; Cremers, Cor W R J; Longridge, Neil S

    OBJECTIVE: To describe cochleovestibular aspects of superficial hemosiderosis of the central nervous system. BACKGROUND: Superficial hemosiderosis of the central nervous system is a rare disease in which cochleovestibular impairment, cerebellar ataxia, and myelopathy are the most frequent signs.

  4. Longitudinal analysis of hearing loss in a case of hemosiderosis of the central nervous system.

    Weekamp, H.; Huygen, P.L.M.; Merx, J.L.; Kremer, H.P.H.; Cremers, C.W.R.J.; Longridge, N.S.

    2003-01-01

    OBJECTIVE: To describe cochleovestibular aspects of superficial hemosiderosis of the central nervous system. BACKGROUND: Superficial hemosiderosis of the central nervous system is a rare disease in which cochleovestibular impairment, cerebellar ataxia, and myelopathy are the most frequent signs.

  5. Two Sisters with Idiopathic Pulmonary Hemosiderosis

    Mehmet Gencer

    2007-01-01

    Full Text Available Idiopathic pulmonary hemosiderosis (IPH is a rare cause of diffuse alveolar hemorrhage with unknown etiology. In the present report, the presentations of two sisters are described: one sister had IPH, eosinophilia and a high serum immunoglobulin E (IgE level; and the other had IPH, pneumothorax, eosinophilia and a high serum IgE level. Both cases had quite unusual presentations. The first patient was 23 years of age, and had suffered from dry cough and progressive dyspnea for four years. Her hemoglobin level was 60 g/L, total serum IgE level was 900 U/mL and eosinophilia was 9%. Her chest radiography revealed diffuse infiltration. She died due to respiratory failure. The second patient was 18 years of age. She had also suffered from dry cough and gradually increasing dyspnea for two years. She had partial pneumothorax in the right lung and diffuse infiltration in other pulmonary fields on chest radiography. Her hemoglobin level was 99 g/L, total serum IgE level was 1200 U/mL and eosinophilia was 8%. IPH was diagnosed by open lung biopsy. All these findings suggested that familial or allergic factors, as well as immunological factors, might have contributed to the etiology of IPH.

  6. Pulmonary hemosiderosis due to mitral valvular heart disease

    Kim, Eung Yeop; Kim, Tae Sung; Han, Joung Ho; Lee, Kyung Soo [Sungkyunkwan Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-01-01

    We report a case of biopsy-proven secondary hemosiderosis of the lung in a 58-year-old patient with mitral valvular heart disease. Both chest radiography and high-resolution CT demonstrated patchy areas of ground-glass opacity ; the former indicated that it was in both lungs, while the latter showed inter-and intralobular septal thickening. These findings were reversible when pulmonary venous hypertension was corrected.

  7. Pulmonary hemosiderosis due to mitral valvular heart disease

    Kim, Eung Yeop; Kim, Tae Sung; Han, Joung Ho; Lee, Kyung Soo

    1999-01-01

    We report a case of biopsy-proven secondary hemosiderosis of the lung in a 58-year-old patient with mitral valvular heart disease. Both chest radiography and high-resolution CT demonstrated patchy areas of ground-glass opacity ; the former indicated that it was in both lungs, while the latter showed inter-and intralobular septal thickening. These findings were reversible when pulmonary venous hypertension was corrected

  8. Nuclear scan of pulmonary hemorrhage in radiopathic pulmonary hemosiderosis

    Miller, T.; Tanaka, T.

    1979-01-01

    Idiopathic pulmonary hemosiderosis, a disease of unknown etiology most often occuring in children, is characterized by recurring episodes of alveolar consolidation. Exacerbations of pulmonary hemorrhage coincide with episodes of alveolar filling; repeated episodes lead to progressive interstitial fibrosis and eventually to corpulmonale. Serial nuclear scans of the lungs after injection of radiolabeled red blood cells should parallel the pathologic and radiographic findings. We observed the accumulation of radiolabeled red blood cells in the lungs on scan images, a finding not previously reported

  9. Celiac disease and pulmonary hemosiderosis in a patient with chronic granulomatous disease

    Hartl, Dominik; Belohradsky, Bernd H.; Griese, Matthias; Nicolai, Thomas; Krauss-Etschmann, Susanne; Roos, Dirk; Wintergerst, Uwe

    2004-01-01

    We report on a patient with the hitherto undescribed combination of chronic granulomatous disease, pulmonary hemosiderosis, and celiac disease. The hemosiderosis resolved with a gluten-free diet and glucocorticosteroid pulse therapy, but the restrictive lung function pattern remained unchanged. Lung

  10. Transfusional hemosiderosis; correlation of MR findings with clinical findings

    Park, Mi Ok; Kim, Ju Heon; Jeon, Woo Jin; Lee, Sung Moon; Kim, Hong; Suh, Soo Jhi [School of Medicine, Keimyung University, Taegu (Korea, Republic of)

    1994-08-15

    Parenchymal iron deposition occurs in hemochromatosis, while iron is deposited in reticuloendothelial cells after blood transfusions(Hemosiderosis). We studied correlation between MR finding and clinical findings(serum ferritin, TSI, LFT, disease duration) of hemosiderosis. 12 patients with chronic renal failure and one patient with aplastic anemia, who have received multiple transfusion, were performed MRI with a 2.0 Tesla unit. In all of 13 patients(17 cases), the liver revealed low signal intensity equal to background noise. In 4 of 17 cases whose serum ferritin level was below 1000 ng/ml, pancreas, gastric wall, adrenal gland were involved in 1 case. In 4 cases with serum ferritin level between 1000 and 1500, pancreas was involved in 2 cases, and other organ was involved in 1 case. In 9 cases with serum ferritin level above 1500ng/ml, pancreas was involved in 9 cases, and other origin 4 cases. The MR findings are well correlated with serum ferritin level whereas the TSI, LFT, disease duration are not correlated with involved organ on MR.

  11. Idiopathic pulmonary hemosiderosis: Alveoli are an answer to anemia

    S Bhatia

    2011-01-01

    Full Text Available Idiopathic pulmonary hemosiderosis (IPH is a rare disorder (triad of iron-deficiency anemia, hemoptysis, and alveolar infiltrates. A 3-year-old male presented with mild fever, breathlessness, dry cough, and bluish nail discoloration for 8 days. He had required five blood transfusions in the past 1 year (last transfusion was given 4 months ago. He had a respiratory rate of 58/min with respiratory distress, cyanosis, and grade III clubbing. Respiratory system examination was normal. Several previous reports of hemoglobin were as low as 3.6 g/dl with hypochromic and microcytic anemia. There were transient increases in the hemoglobin and normalization of red cell morphology with blood transfusions. Serum iron, G6PD enzyme assay, hemoglobin electrophoresis, the sickling test, Coomb′s test, stool and urine analysis, and a Meckel′s scan were normal. HIV antibody and dsDNA were negative. The chest radiograph revealed symmetrical patchy infiltrates sparing lung apices (confirmed on high-resolution computed tomography. Lung biopsy diagnosed pulmonary hemosiderosis (interstitial lung disease with hemosiderin-laden macrophages scattered in the alveoli and areas of fibrosis in the alveolar septa. The patient showed marked clinical improvement in 10 days of therapy with prednisolone. IPH should be listed in the differential diagnosis of a child presenting with unexplained hypochromic, microcytic anemia and respiratory symptoms.

  12. Magnetic resonance imaging of transfusional hemosiderosis complicating thalassemia major

    Brasch, R.C.; Wesbey, G.E.; Gooding, C.A.; Koerper, M.A.

    1984-01-01

    Tissue deposits of hemosiderin, a paramagnetic iron-protein complex, resulted in marked abnormalities of magnetic resonance (MR) spin-echo signal intensity within the viscera of three children with transfusional hemosiderosis and thalassemia major. In all patients the liver and bone marrow demonstrated abnormally low spin-echo intensities and the kidneys and muscles had abnormally high intensities. These observations correlate with in vitro MR observation of ferric (Fe +3 ) solutions, in which concentrations of ferric salts greater than 20 mmol yielded higher intensities than did water alone. MR imaging is sensitive to the tissue deposition of hemosiderin, and MR intensity appears to provide a rough measure of the amount of iron deposited

  13. Increased IgD milk antibody responses in a patient with Down's syndrome, pulmonary hemosiderosis and cor pulmonale.

    Galant, S; Nussbaum, E; Wittner, R; DeWeck, A L; Heiner, D C

    1983-10-01

    IgD antibody responses to cow's milk were investigated in a two-year-old black boy with evidence of pulmonary hemosiderosis and pulmonary hypertension. Initially a broad spectrum of immunologic responses to cow's milk were observed including IgD, IgE, and precipitin antibodies. Specific IgD antibody responses to cow's milk could be modulated in terms of challenge or elimination and correlated with the clinical course. It is possible that IgD antibodies may be important in milk-related pulmonary hemosiderosis.

  14. CT findings of transfusional hemosiderosis in patients with chronic renal failure : clinical correlation

    Park, Tae Joon; Lee, Hae Kyung; Hong, Hyun Sook; Kim, Gun Woo; Kim, Hyung Hwan; Choi, Gyo Chang; Kwon, Kui Hyang; Choi, Deuk Lin

    1997-01-01

    The purpose of this study is to evaluate whether there is any correlation between the CT features of hemosiderosis and clinical findings in patients with chronic renal failure who have received multiple blood transfusion. Among chronic renal failure patients who had undergone long-tern dialysis and received multiple blood transfusions, CT findings in 16 cases in which increased liver attenuation was seen on images obtained for other purpose, were analyzed by three radiologic specialists. The attenuation values of liver, spleen and pancreas compared with that of back muscle were correlated with the amount and duration of transfusion, and blood ferritin level. There is no correlation between the CT features of hemosiderosis and clinical findings. In patients with chronic renal failure and no clinical symptoms, the status of iron overload was relatively easily detected on CT. Close observation of CT findings is thus thought to prevent significant permanent functional deformity of organs in patients with chronic renal failure who have received multiple blood transfusions. (author). 14 refs., 1 tab., 1 fig

  15. Use of sodium chromate Cr51 in diagnosing childhood idiopathic pulmonary hemosiderosis

    Kurzweil, P.R.; Miller, D.R.; Freeman, J.E.; Reiman, R.E.; Mayer, K.

    1984-01-01

    The diagnosis of idiopathic pulmonary hemosiderosis (IPH) may be elusive. A 6-year-old boy had microcytic hypochromic anemia and a hemolytic component. Hemosiderin-laden macrophages were not found in the gastric aspirate. He had no pulmonary signs or symptoms. Extensive hematologic and roentgenologic investigations failed to reveal the cause of the anemia. Quantitative serial scintigraphic scanning showed significant (35%) pulmonary sequestration of autologous erythrocytes labeled with sodium chromate Cr51. The half-life of the RBCs was moderately decreased (half-life, 15 days; normal, 25 to 35 days). An open-lung biopsy spece Cr51. The half-life of the RBCs was moderately decreased (half-life, 15 days; normal, 25 to 35 days). An open-lung biopsy specimen confirmed the diagnosis of IPH. A diagnosis of IPH should be considered when children have iron deficiency anemia and pulmonary signs or symptoms. Organ sequestration studies may be helpful in equivocal cases

  16. Idiopathic Pulmonary Hemosiderosis Presenting as Anemia, Failure to Thrive, and Jaundice in a Toddler.

    Chen, Carol C; McManemy, Julie K; Vece, Timothy J; Cruz, Andrea T

    2016-04-01

    Idiopathic pulmonary hemosiderosis (IPH) is a rare disease characterized by the triad of hemoptysis, pulmonary infiltrates on chest radiograph, and anemia. Its diagnosis should be considered in any child presenting with moderate to severe anemia and failure to thrive of unclear etiology. Consideration of the differential diagnosis in such a child should include the review of both extravascular and intravascular causes of hemolysis. Systemic treatment of IPH with glucocorticoids has been shown to decrease morbidity, mortality, and disease progression to pulmonary fibrosis. Thus, diagnostic delays can impact prognosis. Here, we present a case of a 15-month-old boy with IPH who presented with anemia, jaundice, and failure to thrive, as well as a history of hemoptysis that was not initially elicited.

  17. Idiopathic Pulmonary Hemosiderosis in a Young Adult Patient: A Rare Case

    Abhishek Agarwal

    2018-01-01

    Full Text Available Idiopathic pulmonary hemosiderosis (IPH is often an ignored and rare cause of diffuse alveolar hemorrhage (DAH. It is characterized by triad of hemoptysis, anemia, and alveolar opacity on radiology. It is a diagnosis of exclusion, established after ruling out other causes of DAH such as Goodpasture’s syndrome, large vessel vasculitis, small vessel vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA (Wegener’s granulomatosis, Churg–Strauss syndrome, microscopic polyangiitis, immune complex-related vasculitis (collagen vascular diseases, Henoch–Schönlein purpura, mixed cryoglobulinemia drug reactions, anticoagulation and thrombocytopenia. Though it is a disease primarily affecting children, we hereby report a case of IPH in an adult patient who responded dramatically to oral corticosteroid.

  18. YERSINIA PSEUDOTUBERCULOSIS, SEROGROUP O:1A, INFECTION IN TWO AMAZON PARROTS (AMAZONA AESTIVA AND AMAZONA ORATRIX) WITH HEPATIC HEMOSIDEROSIS.

    Galosi, Livio; Farneti, Silvana; Rossi, Giacomo; Cork, Susan Catherine; Ferraro, Stefano; Magi, Gian Enrico; Petrini, Stefano; Valiani, Andrea; Cuteri, Vincenzo; Attili, Anna-Rita

    2015-09-01

    Necropsies were conducted on a female blue-fronted Amazon (Amazona aestiva) and a female yellow-headed Amazon (Amazona oratrix) that died after depression, ruffled feathers, diarrhea, and biliverdin in the urine. Gross and microscopic examinations revealed multifocal necrosis in the liver, spleen, lungs, kidneys, intestines, and heart caused by acute bacteremia. Yersinia pseudotuberculosis, serogroup O:1a, was isolated by culturing from the visceral lesions in the liver, intestines, and spleen. Virulence gene analysis showed the presence of the inv gene and the complete pathogenicity island: IS100, psn, yptE, irp1, irp2 ybtP-ybtQ, ybtX-ybtS, and int asnT-Int. Histopathologic findings and chemical analysis also demonstrated hepatic hemosiderosis. As has been demonstrated in other species, hemosiderosis may predispose Amazona spp. to systemic infection with Y. pseudotuberculosis after enteric disease.

  19. A young man with hemoptysis: Rare association of idiopathic pulmonary hemosiderosis, celiac disease and dilated cardiomyopathy

    Gopi C Khilnani

    2015-01-01

    Full Text Available Idiopathic pulmonary hemosiderosis (IPH is a rare cause of recurrent diffuse alveolar hemorrhage (DAH with no specific treatment. Herein, we discuss a case of hemoptysis, who had IPH and other rare associations. A 19-year-old man presented with recurrent hemoptysis, generalized weakness and progressive dyspnea for 3 years. Earlier, he was diagnosed with anemia and was treated with blood transfusions and hematinics. On examination he had pallor, tachycardia and was underweight. Investigations revealed low level of hemoglobin (7.8 g/dl and iron deficiency. An electrocardiography (ECG showed sinus tachycardia, interventricular conduction delay and T-wave inversion. Echocardiography revealed dilated cardiomyopathy with left ventricular dysfunction. Computed tomography of the chest demonstrated bilateral diffuse ground glass opacity suggestive of pulmonary hemorrhage. Pulmonary function tests showed restrictive pattern with increased carbon monoxide diffusion. Bronchoalveolar lavage and transbronchial lung biopsy showed hemosiderin-laden macrophages. Patient could recall recurrent episodes of diarrhea in childhood. Serum antitissue transglutamase antibodies were raised (291.66 IU/ml, normal <30 IU/ml. Duodenal biopsy showed subtotal villous atrophy consistent with celiac disease. He was started on gluten-free diet, beta blockers and diuretics. After two years of treatment, he has been showing consistent improvement. Screening for CD is important in patients with IPH. Cardiomyopathy forms rare third association. All three show improvement with gluten-free diet.

  20. Top Differential Diagnosis Should Be Microscopic Polyangiitis in ANCA-Positive Patient with Diffuse Pulmonary Hemorrhage and Hemosiderosis

    Nicholas D. Ward

    2014-01-01

    Full Text Available A rat model of antineutrophil cytoplasmic antibody (ANCA associated vasculitides reveals crescentic glomerulonephritis as seen in human renal biopsies and diffuse lung hemorrhage that is not well documented in human lung biopsies. A 64-year-old male, with shortness of breath and mild elevation of serum creatinine, was found to have a positive serum test for ANCA, but negative antiglomerular basement membrane antibody. A renal biopsy showed pauci-immune type of crescentic glomerulonephritis and focal arteritis. The prior lung wedge biopsy was retrospectively reviewed to show diffuse hemorrhage and hemosiderosis with focal giant cells. In addition, small arteries revealed subtle neutrophil aggregation, and margination along vascular endothelium, but no definitive vasculitis. The pathology of ANCA associated vasculitides results from activated neutrophils by ANCA and subsequent activation of the alternative complement cascade with endothelial injury, neutrophil aggregation and margination. Our findings, after the correlation between lung biopsy and renal biopsy, imply that the top differential diagnosis in the lung biopsy should be microscopic polyangiitis when diffuse pulmonary hemorrhage and hemosiderosis are present in this ANCA-positive patient.

  1. QUANTIFICATION OF SIDEROPHORE AND HEMOLYSIN FROM STACHYBOTRYS CHARTARUM STRAINS, INCLUDING A STRAIN ISOLATED FROM THE LUNG OF A CHILD WITH PULMONARY HEMORRHAGE AND HEMOSIDEROSIS

    A strain of Stachybotrys chartarum was recently isolated from the lung of a pulmonary hemorrhage and hemosiderosis (PH) patient in Texas (designated the Houston strain). This is the first time that S. chartarum has been isolated from the lung of a PH patient. In this study, the ...

  2. Islet inflammation, hemosiderosis, and fibrosis in intrauterine growth-restricted and high fat-fed Sprague-Dawley rats.

    Delghingaro-Augusto, Viviane; Madad, Leili; Chandra, Arin; Simeonovic, Charmaine J; Dahlstrom, Jane E; Nolan, Christopher J

    2014-05-01

    Prenatal and postnatal factors such as intrauterine growth restriction (IUGR) and high-fat (HF) diet contribute to type 2 diabetes. Our aim was to determine whether IUGR and HF diets interact in type 2 diabetes pathogenesis, with particular attention focused on pancreatic islet morphology including assessment for inflammation. A surgical model of IUGR (bilateral uterine artery ligation) in Sprague-Dawley rats with sham controls was used. Pups were fed either HF or chow diets after weaning. Serial measures of body weight and glucose tolerance were performed. At 25 weeks of age, rat pancreases were harvested for histologic assessment. The birth weight of IUGR pups was 13% lower than that of sham pups. HF diet caused excess weight gain, dyslipidemia, hyperinsulinemia, and mild glucose intolerance, however, this was not aggravated further by IUGR. Markedly abnormal islet morphology was evident in 0 of 6 sham-chow, 5 of 8 sham-HF, 4 of 8 IUGR-chow, and 8 of 9 IUGR-HF rats (chi-square, P = 0.007). Abnormal islets were characterized by larger size, irregular shape, inflammation with CD68-positive cells, marked fibrosis, and hemosiderosis. β-Cell mass was not altered by IUGR. In conclusion, HF and IUGR independently contribute to islet injury characterized by inflammation, hemosiderosis, and fibrosis. This suggests that both HF and IUGR can induce islet injury via converging pathways. The potential pathogenic or permissive role of iron in this process of islet inflammation warrants further investigation. Copyright © 2014 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

  3. The Correlation of Cardiac and Hepatic Hemosiderosis as Measured by T2*MRI Technique with Ferritin Levels and Hemochromatosis Gene Mutations in Iranian Patients with Beta Thalassemia Major

    Mohammad Soleiman Soltanpour

    2018-01-01

    Full Text Available Objectives: Organ-specific hemosiderosis and iron overload complications are more serious and more frequent in some patients with beta thalassemia major (BTM compared with others. We investigated whether coinheritance of HFE H63D or C282Y gene mutations in patients with BTM contributes to the phenotypic variation of iron overload complications and assessed the correlation of cardiac and hepatic hemosiderosis with plasma ferritin levels. Methods: We studied 60 patients with BTM with a mean age of 17.5±9.1 years from the Northwest of Iran. HFE gene mutations were analyzed using the polymerase chain reaction-restriction fragment length polymorphism method. Cardiac and hepatic hemosiderosis was assessed using T2*magnetic resonance imaging (MRI. Ferritin levels were measured using the enzyme immunoassay method. Results: Ferritin levels showed a strong inverse correlation with hepatic T2*MRI values (r = -0.631, p = 0.001 but a poor correlation with cardiac T2*MRI values (r = -0.297, p = 0.044. The correlation between cardiac T2*MRI values and hepatic T2*MRI values was poor and insignificant (r = 0.287, p = 0.058. Genotype and allele distribution of HFE H63D and C282Y mutation did not differ significantly between patients with and without hepatic or cardiac hemosiderosis (p > 0.050. However, carriers of HFE 63D allele had significantly higher ferritin levels compared with non-carriers (1 903±993 vs. 992±683, p < 0.001. Conclusions: Cardiac T2*MRI values showed a poor correlation with hepatic T2*MRI values and ferritin levels. Accurate assessment of cardiac iron overload in patients with BTM can only be done using the T2*MRI technique. Additionally, HFE H63D is a significant determinant factor for elevated ferritin levels in BTM patients.

  4. Hemossiderose pulmonar idiopática tratada com azatioprina: relato de caso em criança Idiopathic pulmonary hemosiderosis treated with azathioprine in a child

    Clemax Couto Sant`Anna

    2007-12-01

    Full Text Available A hemossiderose pulmonar idiopática (HPI, principal causa de hemossiderose pulmonar em crianças, cursa com sangramento alveolar intermitente e presença de hemossiderófagos no escarro ou no lavado gástrico. O tratamento é baseado nos corticoesteróides e citostáticos, em condições especiais. Descreve-se o caso de uma menina de sete anos com HPI, que conseguiu controle parcial da doença mediante altas doses de corticoesteróide. O tratamento, no entanto, necessitou ser suspenso gradualmente visto a paciente ter desenvolvido fácies cushingóide. Foi iniciada a associação da azatioprina ao corticóide até a substituição total por azatioprina isolada, cujo uso foi mantido por quatro anos, com ótimo resultado.Idiopathic pulmonary hemosiderosis (IPH, the main cause of pulmonary hemosiderosis in children, is characterized by intermittent alveolar bleeding and hemosiderin-laden macrophages in sputum and in gastric lavage. The treatment is based on corticosteroids and cytotoxic drugs, under special conditions. We describe the case of a 7-year-old girl with IPH who achieved partial clinical remission with high doses of corticosteroids. However, the treatment had to be discontinued because the patient developed Cushing's syndrome. Treatment was started with an azathioprine-corticosteroid combination and then changed to azathioprine alone, which was maintained for four years, with excellent results.

  5. Pulmonary hypoplasia on preterm infant associated with diffuse chorioamniotic hemosiderosis caused by intrauterine hemorrhage due to massive subchorial hematoma: report of a neonatal autopsy case.

    Yamada, Sohsuke; Marutani, Takamitsu; Hisaoka, Masanori; Tasaki, Takashi; Nabeshima, Atsunori; Shiraishi, Mika; Sasaguri, Yasuyuki

    2012-08-01

    A male infant born prematurely at 31 weeks of gestation weighed 789 g and had mildly brown-colored oral/tracheal aspirates at delivery. The amniotic fluid was also discolored, and its index was below 5. The patient died of hypoxemic respiratory and cardiac failure 2 hours after birth. The maternal profiles showed placenta previa and intrauterine growth restriction (IUGR) at 22 weeks of gestation, and revealed recurrent episodes of antenatal and substantial vaginal bleeding and oligohydramnios, indicating chronic abruption-oligohydramnios sequence. The thickened placenta, weighing 275 g, grossly displayed unevenness and diffuse opacity with green to brown discoloration in the chorioamniotic surface, and revealed chronic massive subchorial hematomas (Breus' mole) with old peripheral blood clot, circumvallation, and infarction. Microscopically, diffuse Berlin-blue staining-positive hemosiderin deposits were readily encountered in the chorioamniotic layers of the chorionic plate, consistent with diffuse chorioamniotic hemosiderosis (DCH) due to Breus' mole, accompanied by diffuse amniotic necrosis. At autopsy, an external examination showed several surface anomalies and marked pulmonary hypoplasia, 0.006 (less 0.012) of lung:body weight ratio. Since Breus' mole has a close relationship with intrauterine hemorrhage, resulting in DCH, IUGR, and/or pulmonary hypoplasia of the newborn, the present features might be typical. © 2012 The Authors. Pathology International © 2012 Japanese Society of Pathology and Blackwell Publishing Asia Pty Ltd.

  6. Associação de bronquiolite obliterante pós-infecciosa e hemossiderose pulmonar na infância Postinfectious bronchiolitis obliterans accompanied by pulmonary hemosiderosis in childhood

    Leonardo Araújo Pinto

    2006-12-01

    Full Text Available Descreve-se uma apresentação rara de bronquiolite obliterante associada a hemossiderose pulmonar, em paciente de nove anos com sintomas respiratórios persistentes iniciados após episódio de bronquiolite aguda grave aos sete meses. Após o episódio agudo, apresentou sintomas respiratórios persistentes, piorando significativamente aos sete anos, quando começou a apresentar dificuldade respiratória em pequenos esforços. A tomografia computadorizada de tórax demonstrou achados compatíveis com bronquiolite obliterante. A biópsia pulmonar a céu aberto demonstrou numerosos macrófagos corados com hemossiderina, além dos achados compatíveis com bronquiolite obliterante. O diagnóstico de hemossiderose pulmonar pode estar ocasionalmente associado a bronquiolite obliterante em crianças com seqüela pós-viral grave.In the present report, we describe an unusual presentation of post-infectious bronchiolitis obliterans accompanied by pulmonary hemosiderosis in a nine-year-old boy with persistent respiratory symptoms subsequent to an episode of acute bronchiolitis occurring at the age of seven months. After the episode, the persistent respiratory symptoms worsened significantly, and, by the age of seven, the patient began to have difficulty breathing after minimal exertion. Computed tomography of the chest presented findings consistent with bronchiolitis obliterans. Open lung biopsy revealed numerous hemosiderin-laden macrophages, as well as other findings consistent with bronchiolitis obliterans. Pulmonary hemosiderosis can occasionally be accompanied by bronchiolitis obliterans in children with severe sequelae after an episode of viral infection.

  7. Patient-reported outcomes of deferasirox (Exjade, ICL670) versus deferoxamine in sickle cell disease patients with transfusional hemosiderosis. Substudy of a randomized open-label phase II trial.

    Vichinsky, Elliott; Pakbaz, Zahra; Onyekwere, Onyinye; Porter, John; Swerdlow, Paul; Coates, Thomas; Lane, Peter; Files, Beatrice; Mueller, Brigitta U; Coïc, Lena; Forni, Gian Luca; Fischer, Roland; Marks, Peter; Rofail, Diana; Abetz, Linda; Baladi, Jean-Francois

    2008-01-01

    There is increasing evidence demonstrating the value of transfusions in sickle cell disease (SCD). However, resultant iron overload can be life threatening if untreated. Chelation therapy with deferoxamine requires parenteral infusions that can negatively impact quality of life and adherence to treatment. As part of a phase II trial, SCD patient-reported outcomes were evaluated. One hundred and ninety-five patients were randomized (2:1) to receive oral deferasirox (5-30 mg/kg/day) or deferoxamine (20-50 mg/kg, 5 days per week); 121 had previously received deferoxamine. At each time point, significantly more patients who had previously received deferoxamine were 'satisfied/very satisfied' with deferasirox, or found treatment to be 'convenient/very convenient' compared with deferoxamine (p < 0.001). In these patients, fewer hours were lost from daily activities with deferasirox than deferoxamine treatment. Most patients (77%) preferred deferasirox, and more were willing to continue taking deferasirox than deferoxamine at end-of-study (84 vs. 11%, respectively). Patients with SCD are therefore more satisfied with deferasirox, which has a lower impact on daily activities than deferoxamine. Given the high levels of satisfaction, it is likely that quality of life will be improved. These results also suggest that treatment adherence with deferasirox may be better than with deferoxamine, which should lead to improved long-term outcomes. 2008 S. Karger AG, Basel.

  8. Liver iron overloading in captive muriquis (Brachyteles spp.).

    Santos, Stéfanie V; Strefezzi, Ricardo De F; Pissinatti, Alcides; Catão-Dias, José L

    2011-04-01

    Iron accumulation was investigated qualitatively and quantitatively in the liver of 15 captive Brachyteles spp. Hepatic hemosiderosis index (HHI) was determined as the area percentage of the liver parenchyma occupied by hemosiderin and ferritin deposits, through computerized histomorphometric analysis of Prussian blue-stained histologic sections. All studied animals presented liver hemosiderosis, and HHI ranged from 0.2% to 41.7%. There were no significant differences in HHI between muriqui species or genders, and no correlations were detected among HHI and age, time in captivity or body mass. Iron deposits were accompanied by other hepatic disorders. This is the first study addressing the occurrence and consequences of iron overloading in the liver of muriquis. We propose that hemosiderosis may act as a contribute factor for the development of hepatic injuries. Further studies are advised to clarify the role of diet in the pathogenesis of hemosiderosis in these atelids. © 2010 John Wiley & Sons A/S.

  9. MR imaging of femoral marrow in treated β-thalassemia major

    Shen Jun; Liang Biling; Chen Jianyu; Zhao Jiquan; Xu Honggui; Chen Chun

    2006-01-01

    Objective: To investigate MR imaging features of femoral marrow in treated β-thalassemia major. Methods: MR imaging of the proximal femoral marrow was performed in 35 cases of β-thalassemia major and 45 age- and sex-matched normal children as control. Coronal images of femoral marrow with the techniques of spin echo and fast field echo (FFE) were obtained. On T 1 -weighted imaging the red and yellow femoral marrow were judged and marrow distribution was classified into five groups. The hemosiderosis of marrow was judged on the basis of signal intensity of marrow on FFE imaging. The marrow distribution classification and the hemosiderosis on MR imaging were correlated with clinical features. Results: On FFE, marrow hemosiderosis occurred in 15 patients with a marked hypo-intensity signal and was related to the age (P=0.032). On T 1 -weighted imaging, the femoral marrow in 35 patients was classified as group III and IV, while the marrow distribution was group I or II in all normal children, there was statistically significant difference (P<0.001). The marrow distribution correlated positively with blood transfusion (P=0.049). Conclusion: The red marrow hyperplasia and hemosiderosis could occur in the femoral marrow of the treated β-thalassemia major. The marrow hyperplasia on MR imaging was related to the blood transfusion, and the hemosiderosis related to the age. (authors)

  10. Assessment of Biochemical Liver Function Tests in Relation to Age ...

    2018-01-24

    Jan 24, 2018 ... and build upon the work non-commercially, as long as the author is credited and the new creations ... and Family Medicine General Outpatients Department clinics of ..... hepatitis, iron overload (hemosiderosis), and gallstones. Coiner et al ... this study, and therefore their role in the causation of the observed ...

  11. Iron storage disease in tapirs.

    Bonar, Christopher J; Trupkiewicz, John G; Toddes, Barbara; Lewandowski, Albert H

    2006-03-01

    Recent studies of serum iron and iron binding capacity have indicated that tapirs could be at risk of developing hemochromatosis. However, in recent surveys of pathologic findings in tapirs, hemochromatosis was not reported as a cause of death. This study reviews necropsy reports from three species of tapir (Baird's tapir [Tapirus bairdii], Malayan tapir [Tapirus indicus], and Brazilian tapir [Tapirus terrestris]) at the Philadelphia Zoological Garden between 1902 and 1994. Twelve cases of hemosiderosis, including fatal hemochromatosis in two Baird's tapirs, were found among 19 cases examined histologically. Hemochromatosis has previously been reported in the horse, rhinoceros, and in one Brazilian tapir. Dietary factors were investigated but could not be confirmed to have contributed to the incidence of hemosiderosis and hemochromatosis in the three species of tapir in the Philadelphia Zoological Garden collection.

  12. Magnetic resonance imaging of iron storage diseases

    Yoshida, Hideo; Mano, Isamu; Asai, Sae; Yashiro, Naofumi; Itai, Yuji; Iio, Masahiro.

    1985-01-01

    We presented MRI findings of four patients of iron storage diseases with hemochromatosis and hemosiderosis. We examined detectavility of iron deposits with in vitro MR and X-CT observations of ferric (Fe 3+ ) solutions. Conculusion are as follows, 1) In detection of small amount of iron deposits, MRI is much better than X-CT. 2) MRI is a unique technique to detect iron deposits in bone marrow. 3) Early estimation of iron storage diseases will be promising using MRI technique. (author)

  13. CLINICAL FEATURES OF REFRACTORY FORMS OF ANEMIA IN CHILDREN WITH CHRONIC HEPATITIS В

    F. I. Inoyаtova

    2013-01-01

    Full Text Available Examination of 125 children with chronic hepatitis В and concomitant anemia has determined the frequency of refractory forms of anemia (52,5%. The disease progressed more severely on the background of anemia, which was indicated by the prevalence of CHВ forms with severe activity (71,4%. The pathognomonic symptoms of anemic processes were revealed. Two pathogenetic variants of the anemia genesis in children with CHВ are being considered: the first is defined by veritable iron deficiency with ferrokinetic markers of iron-deficiency anemia; the second — by relocationable iron deficit that is typical for hemosiderosis and refractoriness development.

  14. Pulmón del soldador de arco

    Luciana Molinari

    2010-12-01

    Full Text Available La siderosis del soldador o neumoconiosis siderótica fue descripta por Doig y McLaughlin en 1936 como una enfermedad pulmonar causada por la inhalación crónica de polvo de hierro en soldadores de arco eléctrico. Presentamos un caso de siderosis del soldador asociada a aumento de los niveles de ferritina, sin hallazgo de depósito de hierro en otros órganos y sin causas evidentes de hemosiderosis secundaria.

  15. Visualization of the liver with sup(99m)Tc-EHDP in thalassemia major

    Valdez, V.A.; Jacobstein, J.G.

    1981-01-01

    A case is reported in which substantial liver uptake of sup(99m)Tc-hydroxyethylidene diphosphonate sup(99m)Tc-EHDP) was seen on bone scan. The patient, a 25-year-old male with the diagnosis of thalassemia major made at the age of 3 years, had received regular blood transfusions over many years. Clinical and laboratory evidence of hemosiderosis suggests that the presence of large quantities of iron in the liver of this patient may have been responsible for the hepatic uptake of the bone scanning agent. (orig.)

  16. Diabetes in Patients with ß-thalassemia or other Hemoglobinopathies - Analysis from the DPV Database.

    Warncke, K; Konrad, K; Kohne, E; Hammer, E; Ohlenschläger, U; Herrlinger, S; Jäger, A; Holl, R W

    2016-11-01

    Background: Diabetes mellitus is a common endocrinopathy in patients with thalassemia major, but the occurrence of hemoglobinopathies is rare in Germany and Western Europe. The longitudinal German-Austrian DPV (Diabetes Patienten Verlaufsdokumentation) registry allows a comprehensive characterization of this group of patients. Patients/methods: Patients from the DPV-registry agedthalassemia major or other hemoglobinopathies were compared to patients with type 1 diabetes (T1D) and type 2 diabetes (T2D) using the statistical software SAS 9.4. Results: 94 patients (0.13% of patients) with hemoglobinopathies are registered in DPV. 82.4% of 17 patients with thalassemia major, 100% of 12 patients with sickle cell disease (SCD) and >90% of 65 patients with other hemoglobinopathies receive insulin treatment. In the majority of patients with thalassemia major, hemosiderosis is documented. Patients with thalassemia major developed diabetes at a median age of 14.6 [IQR 8.4-18.0] years (9.0 years [5.3-12.5] in T1D; 18.7 years [14.2-25.6] in TD2; both pthalassemia major is probably caused by hemosiderosis due to polytransfusion, while patients with SCD/thalassemia minor are most likely affected by T1D. The high rate of hypoglycemia in patients with ß-thalassemia major may be caused by liver fibrosis and a lack of hepatic glycogen stores. © Georg Thieme Verlag KG Stuttgart · New York.

  17. Hemorragia Alveolar Difusa - a propósito de um caso clínico de Hemossiderose Pulmonar Idiopática.

    Paula Esteves

    1999-09-01

    Full Text Available RESUMO: Devido à sua raridade, a Hemorragia Alveolar Difusa (HAD é muitas vezes urn diagnóstico esquecido. Os autores apresentam uma revisião desta entidade a propósito do caso clínico de um homem de 71 anos, internado por febre, tosse seu, falência respiratória progressiva e com o diagnóstico de admissão de pneumonia da comunidade. Os exames efectuados foram compatíveis com HAD e após realização de biópsia pulmonar cirúrgica foi admitido Hemossiderose Pulmonar Idiopática como diagnóstico de exclusão.REV PORT PNEUMOL 1999; V (5: 499-505 ABSTRACT: Due to its rarity, the diffuse alveolar hemorrhage (DAH is very often a forgotten diagnosis. The authors present a revision of this entity, in relation to a clinical case of a 71 years old men, who was admitted b fever, dry cough, progressive respiratory failure and community-acquired pneumonia as admission’s diagnostic. The exams performed suggested DAH and after chirurgical pulmonary biopsy, idiopathic pulmonary hemosiderosis was established by exclusion.REV PORT PNEUMOL 1999; V (5: 499-505 Palavras chave: Hemorragia alveolar, Hemossiderose Pulmonar, Key-words: Alveolar Hemorrhage, Idiopathic Pulmonary Hemosiderosis

  18. Iron oxides in human spleen.

    Kopáni, Martin; Miglierini, Marcel; Lančok, Adriana; Dekan, Július; Čaplovicová, Mária; Jakubovský, Ján; Boča, Roman; Mrazova, Hedviga

    2015-10-01

    Iron is an essential element for fundamental cell functions and a catalyst for chemical reactions. Three samples extracted from the human spleen were investigated by scanning (SEM) and transmission electron microscopy (TEM), Mössbauer spectrometry (MS), and SQUID magnetometry. The sample with diagnosis of hemosiderosis (H) differs from that referring to hereditary spherocytosis and the reference sample. SEM reveals iron-rich micrometer-sized aggregate of various structures-tiny fibrils in hereditary spherocytosis sample and no fibrils in hemochromatosis. Hematite and magnetite particles from 2 to 6 μm in TEM with diffraction in all samples were shown. The SQUID magnetometry shows different amount of diamagnetic, paramagnetic and ferrimagnetic structures in the tissues. The MS results indicate contribution of ferromagnetically split sextets for all investigated samples. Their occurrence indicates that at least part of the sample is magnetically ordered below the critical temperature. The iron accumulation process is different in hereditary spherocytosis and hemosiderosis. This fact may be the reason of different iron crystallization.

  19. Pulmón del soldador de arco Arc welder's lung

    Luciana Molinari

    2010-12-01

    Full Text Available La siderosis del soldador o neumoconiosis siderótica fue descripta por Doig y McLaughlin en 1936 como una enfermedad pulmonar causada por la inhalación crónica de polvo de hierro en soldadores de arco eléctrico. Presentamos un caso de siderosis del soldador asociada a aumento de los niveles de ferritina, sin hallazgo de depósito de hierro en otros órganos y sin causas evidentes de hemosiderosis secundaria.Pneumoconiosis of electric arc welder or siderotic pneumoconiosis was described by Doig and McLaughlin in 1936 as a lung disease caused by chronic inhalation of iron fumes in electric arc welders. We present a case report of electric arc welder siderosis associated with high levels of ferritin, without findings of iron deposit in any other organ.

  20. Interstitial inflammatory lesions of the pulmonary allograft: a retrospective analysis of 2697 transbronchial biopsies

    Burton, C.M.; Iversen, M.; Carlsen, J.

    2008-01-01

    . The strongest histological correlations were between ACR and lymphocytic bronchiolitis (OR 5.1, Pfibrosis (OR 3.2, Ppulmonary hemosiderosis were also more likely to demonstrate the finding of interstitial fibrosis (OR 3.0 and 3.7, P..., incidence and possible associations between commonly identified inflammatory and fibrotic lesions in the pulmonary allograft. METHODS: Retrospective chart review of all transbronchial biopsies performed within the first 2 years of 299 lung-transplanted patients in the period 1996 to 2006. RESULTS: A total...... (BOOP) and interstitial pneumonitis occurred at 4 to 6 weeks, and 6 to 12 weeks, respectively. There was a steady increase in the cumulative proportion of patients with fibrosis and bronchiolitis obliterans, at each successive scheduled surveillance time point beyond 3 months posttransplantation...

  1. MR imaging of bone marrow disorders

    Yoshida, H.; Mano, I.; Yashiro, N.; Asai, S.; Lio, M.

    1986-01-01

    The author performed MR imaging in 89 patients with bone marrow disorders (29 with aplastic anemia, 20 with leukemia, 9 with postirradiation changes, 8 with hemosiderosis, 6 with primary bone tumors and metastases, and 17 with bone marrow disorders of other etiologies). They selected the thoracic and lumbar vertebral marrow as a target and used both T1-weighted spin-echo images and calculated T1 images. T1 was prolonged in bone marrow hyperplasia but shortened in hypoplasia. Bone marrow T1 values proved to depend on the number of fat cells (pathologic correlation). In aplastic anemia scattered islands of low signal intensity were seen within a background of high signal intensity in some typical cases. MR imaging patterns were used for staging aplastic anemia. T1 was prolonged in leukemia cells

  2. Imaging features of thalassemia

    Tunaci, M.; Tunaci, A.; Engin, G.; Oezkorkmaz, B.; Acunas, G.; Acunas, B. [Dept. of Radiology, Istanbul Univ. (Turkey); Dincol, G. [Dept. of Internal Medicine, Istanbul Univ. (Turkey)

    1999-07-01

    Thalassemia is a kind of chronic, inherited, microcytic anemia characterized by defective hemoglobin synthesis and ineffective erythropoiesis. In all thalassemias clinical features that result from anemia, transfusional, and absorptive iron overload are similar but vary in severity. The radiographic features of {beta}-thalassemia are due in large part to marrow hyperplasia. Markedly expanded marrow space lead to various skeletal manifestations including spine, skull, facial bones, and ribs. Extramedullary hematopoiesis (ExmH), hemosiderosis, and cholelithiasis are among the non-skeletal manifestations of thalassemia. The skeletal X-ray findings show characteristics of chronic overactivity of the marrow. In this article both skeletal and non-skeletal manifestations of thalassemia are discussed with an overview of X-ray findings, including MRI and CT findings. (orig.)

  3. Imaging features of thalassemia

    Tunaci, M.; Tunaci, A.; Engin, G.; Oezkorkmaz, B.; Acunas, G.; Acunas, B.; Dincol, G.

    1999-01-01

    Thalassemia is a kind of chronic, inherited, microcytic anemia characterized by defective hemoglobin synthesis and ineffective erythropoiesis. In all thalassemias clinical features that result from anemia, transfusional, and absorptive iron overload are similar but vary in severity. The radiographic features of β-thalassemia are due in large part to marrow hyperplasia. Markedly expanded marrow space lead to various skeletal manifestations including spine, skull, facial bones, and ribs. Extramedullary hematopoiesis (ExmH), hemosiderosis, and cholelithiasis are among the non-skeletal manifestations of thalassemia. The skeletal X-ray findings show characteristics of chronic overactivity of the marrow. In this article both skeletal and non-skeletal manifestations of thalassemia are discussed with an overview of X-ray findings, including MRI and CT findings. (orig.)

  4. Imaging findings of Copa syndrome in a 12-year-old boy

    Noorelahi, Razan; Otero, Hansel J. [George Washington University School of Medicine and Health Services, Department of Diagnostic Imaging and Radiology, Children' s National Health System, Washington, DC (United States); Perez, Geovany [George Washington University School of Medicine and Health Sciences, Pulmonary and Sleep Medicine Division, Washington, DC (United States)

    2018-02-15

    Copa syndrome is a newly described autosomal dominant autoinflammatory disease that presents as pulmonary hemosiderosis and polyarticular arthritis. Twenty-one cases from five families have been reported to date. We present chest computed tomography (CT) and temporomandibular joint magnetic resonance (MR) findings of a 12-year-old boy presenting with dyspnea on exertion, fatigue and clubbing. Additional findings included a restrictive pattern of pulmonary involvement and positive inflammatory markers and autoantibodies. Genetic testing revealed a p.W240R variant of the COPA gene confirming the diagnosis of Copa syndrome. CT of the chest showed a nonspecific interstitial pneumonia pattern distributed mainly in the lower lobes. MR of the temporomandibular joints and follow-up CT three years later are also described. (orig.)

  5. Successful treatment of idiopathic pulmonary capillaritis with intravenous cyclophosphamide.

    Flanagan, Frances

    2013-03-01

    Idiopathic pulmonary hemosiderosis (IPH), a subtype of diffuse alveolar hemorrhage is a rare condition, first described by Virchow in 1864. Historically, it manifests in children in the first decade of life with the combination of hemoptysis, iron deficiency anemia, and alveolar infiltrates on chest radiograph. More recently, diffuse alveolar hemorrhage has been classified by the absence or presence of pulmonary capillaritis (PC), the latter carrying a potential for a poorer outcome. While systemic corticosteroids remain the first line treatment option, other immune modulators have been trailed including hydroxychloroquine, azathioprine, 6-mercaptopurine, and cyclophosphamide with varying results. Our case demonstrates for the first time, the successful use of intravenous cyclophosphamide in the management of chronic idiopathic PC.

  6. Paroxysmal nocturnal hemoglobinuria: a case report of MR, CT findings

    Yang, Ik; Chung, Soo Young; Park, Hai Jung; Lee Yul; Chun, Rho Won; Noh, Jung Woo

    1995-01-01

    Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disease involving multiple hematopoietic cell lines. Characteristics of PNH are intrinsic hemolytic anemia, iron deficiency anemia and venous thrombosis. We report a case of PNH with characterostoc MR and CT findings. The signal intensity of renal cortex was lower than that of medulla on both T1-and T2-weighted MR imaging. On T2 weighted MR images, the liver showed very low signal intensity but the signal intensity of the spleen was normal. On precontrast CT the attenuation of renal cortex was higher than that of renal medulla and the attenuation of liver was higher than that of the spleen. These findings of MR imaging and CT were the result from the deposition of hemosiderin in the cells of proximal convoluted tubules and transfusional hemosiderosis of liver

  7. Gastrointestinal and hepatic complications of sickle cell disease.

    Ebert, Ellen C; Nagar, Michael; Hagspiel, Klaus D

    2010-06-01

    Sickle cell disease (SCD) is an autosomal recessive abnormality of the beta-globin chain of hemoglobin (Hb), resulting in poorly deformable sickled cells that cause microvascular occlusion and hemolytic anemia. The spleen is almost always affected by SCD, with microinfarcts within the first 36 months of life resulting in splenic atrophy. Acute liver disorders causing right-sided abdominal pain include acute vaso-occlusive crisis, liver infarction, and acute hepatic crisis. Chronic liver disease might be due to hemosiderosis and hepatitis and possibly to SCD itself if small, clinically silent microvascular occlusions occur chronically. Black pigment gallstones caused by elevated bilirubin excretion are common. Their small size permits them to travel into the common bile duct but cause only low-grade obstruction, so hyperbilirubinemia rather than bile duct dilatation is typical. Whether cholecystectomy should be done in asymptomatic individuals is controversial. The most common laboratory abnormality is an elevation of unconjugated bilirubin level. Bilirubin and lactate dehydrogenase levels correlate with one another, suggesting that chronic hemolysis and ineffective erythropoiesis, rather than liver disease, are the sources of hyperbilirubinemia. Abdominal pain is very common in SCD and is usually due to sickling, which resolves with supportive care. Computed tomography scans might be ordered for severe or unremitting pain. The liver typically shows sickled erythrocytes and Kupffer cell enlargement acutely and hemosiderosis chronically. The safety of liver biopsies has been questioned, particularly during acute sickling crisis. Treatments include blood transfusions, exchange transfusions, iron-chelating agents, hydroxyurea, and allogeneic stem-cell transplantation. Copyright 2010 AGA Institute. Published by Elsevier Inc. All rights reserved.

  8. Bone marrow MR imaging as predictors of outcome in hemopoietic stem cell transplantation

    Shen, Jun; Cheng, Li-Na; Duan, Xiao-Hui; Liang, Bi-Ling [Sun Yat-sen University, Department of Radiology, Guangzhou, Guangdong (China); Second Affiliated Hospital, Guangzhou, Guangdong (China); Griffith, James F. [Chinese University of Hong Kong, Prince of Wales Hospital, Department of Diagnostic Radiology and Organ Imaging, Shatin, Hong Kong SAR (China); Xu, Hong-Gui [Sun Yat-sen University, Department of Pediatrics, Guangzhou, Guangdong (China); Second Affiliated Hospital, Guangzhou, Guangdong (China)

    2008-09-15

    The purpose of this study is to investigate the role of femoral marrow MR imaging as predictor of outcome for hemopoietic stem cell transplantation (HSCT) in beta-thalassemia major. MR imaging of the proximal femur, including T1- and T2-weighted spin echo and short-tau inversion recovery and in-phase and out-of-phase fast field echo images, was prospectively performed in 27 thalassemia major patients being prepared for HSCT. The area of red marrow and its percentage of the proximal femur were measured, and the presence of marrow hemosiderosis was assessed. Age-adjusted multivariate logistic regression was used to determine the relationship between red marrow area percentage and marrow hemosiderosis and HSCT outcome. Red area percentage were less in patients with successful (90.25{+-}4.14%) compared to unsuccessful transplants (94.54% {+-}2.93%; p=0.01). Red marrow area percentage correlated positively with duration of symptoms(r=0.428, p=0.026) and serum ferritin (r=0.511, p=0.006). In multivariate-adjusted logistic regression analyses, red marrow area percentage was significantly inversely associated with successful HSCT (OR=1.383, 95% CI: 1.059-1.805, p=0.005). Marrow hemosidersosis and duration of sympotms and serum ferritin were not associated with HSCT outcome(p=0.174, 0.974, 0.762, respectively). Red marrow area percentage of proximal femur on MR imaging is a useful predictor of HSCT outcome. (orig.)

  9. Bone marrow MR imaging as predictors of outcome in hemopoietic stem cell transplantation

    Shen, Jun; Cheng, Li-Na; Duan, Xiao-Hui; Liang, Bi-Ling; Griffith, James F.; Xu, Hong-Gui

    2008-01-01

    The purpose of this study is to investigate the role of femoral marrow MR imaging as predictor of outcome for hemopoietic stem cell transplantation (HSCT) in beta-thalassemia major. MR imaging of the proximal femur, including T1- and T2-weighted spin echo and short-tau inversion recovery and in-phase and out-of-phase fast field echo images, was prospectively performed in 27 thalassemia major patients being prepared for HSCT. The area of red marrow and its percentage of the proximal femur were measured, and the presence of marrow hemosiderosis was assessed. Age-adjusted multivariate logistic regression was used to determine the relationship between red marrow area percentage and marrow hemosiderosis and HSCT outcome. Red area percentage were less in patients with successful (90.25±4.14%) compared to unsuccessful transplants (94.54% ±2.93%; p=0.01). Red marrow area percentage correlated positively with duration of symptoms(r=0.428, p=0.026) and serum ferritin (r=0.511, p=0.006). In multivariate-adjusted logistic regression analyses, red marrow area percentage was significantly inversely associated with successful HSCT (OR=1.383, 95% CI: 1.059-1.805, p=0.005). Marrow hemosidersosis and duration of sympotms and serum ferritin were not associated with HSCT outcome(p=0.174, 0.974, 0.762, respectively). Red marrow area percentage of proximal femur on MR imaging is a useful predictor of HSCT outcome. (orig.)

  10. Hemochromatosis and diabetes mellitus: case report and literature review

    Karina Biavatti

    2008-12-01

    Full Text Available Hemochromatosis is a disorder characterized by iron storage amended. The acquired form of the disease can be caused by iron overload, alcoholism, infection by  C virus hepatitis, non-alcoholic hepatitis and chronic liver disease. The hereditary form can be caused by different mutations, being the C282Y and H63D the most frequent, 83% of cases are homozigotous for C282Y and 4% are compound heterozygous (C282Y/H63D. Hemochromatosis is a condition that can affect several organs, including: heart, joints, liver, hypothalamus, pituitary, pancreas and gonads. The aim of this study was to report a case of hemochromatosis and review the literature, with special attention to the association of hemochromatosis and diabetes mellitus. Patient 53 years, male presented to the doctor with arthralgia metacarpophalangeal, ankles, knees, coxofemoral right, and cervical and lumbar, complaints of fatigue and weight loss. Between 3 brothers, one of them had a diagnosis of hereditary hemochromatosis, with PCR demonstrating homozygous for C282Y. Labs: GOT 128 U/L, ALT 231 U/L, alkaline phosphatase 258 U/L, abdominal ultrasound with hepatomegaly and spleen at the upper limit of normal. Liver biopsy demonstrated portal fibrosis extension with hemosiderosis intense. It also made the diagnosis of diabetes mellitus. The research confirmed the same mutation of the changing family: homozygous for C282Y

  11. Pituitary disease in childhood: utility of magnetic resonance

    Vela, A. C.; Oleaga, L.; Ibanez, A. M.; Campo, M.; Grande, D.

    2000-01-01

    To assess the utility of magnetic resonance (MR) imaging in the study of pediatric patients with clinical suspicion of pituitary disease. We studied 18 patients aged 7 to 18 years.Fifteen had hormonal disturbances, two presented amenorrhea and 1 complained of headache, fever and symptoms of polyuria and polydipsia. All the patients were examined using a Siemens SP 42 1-Tesla MRI scanner. Sagittal and coronal T1-weighted spin-echo images were obtained; in addition T2-weighted spin-echo or fast spin-echo imaging was performed in ten cases and intravenous gadolinium was administered in nine. We found 9 patients with hypothalamic-pituitary dysgenesis, 2 with germinoma, 2 cases of pituitary hemosiderosis in patients with thalassemia, 2 cases of microadenoma, one abscess, one case of idiopathic central diabetes insipidus and one of Langerhans cell histiocytosis. MR enabled us to assess pituitary structural alterations in children with hypothalamic-pituitary hormone deficiencies. In our series of patients, hypothalamic-pituitary dysgenesiss was the most frequent cause of adenohypophyseal deficiencies, and most cases of central diabetes insipidus were secondary to masses in the sellar and suprasellar region. In patients with thalassemia, T2-weighted MR images showed the amount of iron deposited in adenophypophysis. Gadolinium-enhanced studies were useful in the study of masses and when the presence of microadenoma was suspected. (Author) 26 refs

  12. Deferasirox: appraisal of safety and efficacy in long-term therapy

    Chaudhary P

    2013-08-01

    Full Text Available Preeti Chaudhary, Vinod PullarkatJane Ann Nohl Division of Hematology, University of Southern California Keck School of Medicine, Los Angeles, CA, USAAbstract: Deferasirox is a once-daily, oral iron chelator that is widely used in the management of patients with transfusional hemosiderosis. Several Phase II trials along with their respective extension studies as well as a Phase III trial have established the efficacy and safety of this novel agent in transfusion-dependent patients with β-thalassemia, sickle-cell disease and bone marrow-failure syndromes, including myelodysplastic syndrome and aplastic anemia. Data from various clinical trials show that a deferasirox dose of 20 mg/kg/day stabilizes serum ferritin levels and liver iron concentration, while a dose of 30–40 mg/kg/day reduces these parameters and achieves negative iron balance in red cell transfusion-dependent patients with iron overload. Across various pivotal clinical trials, deferasirox was well tolerated, with the most common adverse events being gastrointestinal disturbances, skin rash, nonprogressive increases in serum creatinine, and elevations in liver enzyme levels. Longer-term extension studies have also confirmed the efficacy and safety of deferasirox. However, it is essential that patients on deferasirox therapy are monitored regularly to ensure timely management for any adverse events that may occur with long-term therapy.Keywords: deferasirox, iron overload, thalassemia, sickle-cell disease, myelodysplastic syndrome

  13. Hepatic pathologies in the brackish water catfish (Chrysichthys nigrodigitatus) from contaminated locations of the Lagos lagoon complex

    Olarinmoye, O.; Taiwo, V.; Clarke, E.; Kumolu-Johnson, C.; Aderinola, O.; Adekunbi, F.

    2010-01-01

    Several toxicological studies into the effects of aquatic pollutants on the liver of teleost fish exist in literature. The focus on the liver in these studies is predicated on its central nature in the scheme of biotransformation and excretion of xenobiotics following exposure in polluted water bodies. As a consequence of the latter primary role of the liver in these processes it is regarded as a predilective site for the sub lethal effects of xenobiotics on the organism usually detectable at histological level. Hepatic histopathology recorded in livers from feral populations of the brackish water catfish Chrysichthys nigrodigitatus from locations on the Lagos lagoon complex with significant anthropogenic inputs from denizen populations and industries are presented. Liver sections from sixty specimens from two locations on the Lagos lagoon complex (Badagry lagoon: 6??24'N, 2??56'E; and Lagos lagoon: 6??29'N, 3??22'E) were analysed. Observed pathologies included hydropic degeneration (58%), portal / sinusoidal congestion (33%), hepatic necrosis (26%), hemosiderosis (12%) and foci of cellular alterations (FCA's). No obvious oncologic features were observed; the presence of the hydropic Vacuolation lesion was taken as prelude to the development of neoplasms and discussed as such. ?? 2009, Penkala Bt., Budapest, Hungary.

  14. Frequency and appearance of hemosiderin depositions after aneurysmal subarachnoid hemorrhage treated by endovascular therapy

    Falter, Bernhard; Wiesmann, Martin; Freiherr, Jessica; Nikoubashman, Omid; Mull, Michael [University Hospital Aachen RWTH, Department of Neuroradiology, Aachen (Germany)

    2015-10-15

    It is still unclear how often subarachnoid hemorrhage (SAH) leads to chronic hemosiderin depositions. In this study, we aimed to determine the frequency of chronic hemosiderin depositions after aneurysmal SAH in patients who did not undergo surgery. Furthermore, we analyzed typical MRI patterns of chronic SAH and sought to obtain information on the temporal course of MRI signal changes. We retrospectively analyzed 90 patients who had undergone endovascular treatment for acute aneurysmal SAH. In all patients, initial CT studies and at least one T2*-weighted MRI obtained 6 months or later after SAH were analyzed for the presence and anatomical distribution of SAH or chronic hemosiderin depositions. In total, 185 T2*-weighted MRI studies obtained between 2 days and 148 months after SAH were evaluated (mean follow-up 30.2 months). On MRI studies obtained later than 6 months after SAH, subpial hemosiderin depositions were found in 50 patients (55.5 %). Most frequent localizations were the parenchyma adjacent to the frontal and parietal sulci and the insular cisterns. While the appearance of hemosiderin depositions was dynamic within the first 3 months, no changes were found during subsequent follow-up. MR signal changes were not only conclusive with subarachnoid hemosiderin depositions but in many cases also resembled those that have been associated with cortical hemosiderosis. T2*-weighted MRI is an effective means of diagnosing prior SAH. Our study suggests that chronic hemosiderin depositions can be found in a considerable number of patients after a single event of subarachnoid hemorrhage. (orig.)

  15. PREVENTION OF Β THALASSEMIA IN NORTHERN ISRAEL - A COST-BENEFIT ANALYSIS.

    Ariel Koren

    2014-02-01

    Full Text Available Background:β Thalassemia major is characterized by hemolytic anemia, ineffectiveerythropoiesis and hemosiderosis. About 4 % of the world population carries a Thalassemiagene. Management includes blood transfusions and iron chelation, this treatmentis costly and population screening may be significantly more cost benefit. Purpose: Thepurpose of the current study is to analyze the cost of running a preventionprogram for β Thalassemia in Israel and compare it to the actual expensesincurred by treating Thalassemia patients. Methods: Threecost parameters were analyzed and compared: The prevention program, routinetreatment of patients and treatment of complications. An estimation of theexpenses needed to treat patients that present with complications werecalculated based on our ongoing experience in treatment of deterioratingpatients. Results andConclusions: The cost of preventing one affected newborn was $63,660 comparedto $1,971,380 for treatment of a patient during 50 years (mean annual cost:  $39,427. Thus, the prevention of 45 affectednewborns over a ten years period represents a net saving of $88.5 million tothe health budget. Even after deducting the cost of the prevention program ($413.795/yr., the program still represents abenefit of $ 76 million over ten years. Each prevented case could pay thescreening and prevention program for 4.6 ys.

  16. Advances in pediatrics in 2014: current practices and challenges in allergy, gastroenterology, infectious diseases, neonatology, nutrition, oncology and respiratory tract illnesses.

    Caffarelli, Carlo; Santamaria, Francesca; Cesari, Silvia; Sciorio, Elisa; Povesi-Dascola, Carlotta; Bernasconi, Sergio

    2015-10-31

    Major advances in the conduct of pediatric practice have been reported in the Italian Journal of Pediatrics in 2014. This review highlights developments in allergy, gastroenterology, infectious diseases, neonatology, nutrition, oncology and respiratory tract illnesses. Investigations endorse a need to better educate guardians and improve nutritional management in food allergy. Management of hyperbilirubinemia in neonates and of bronchiolitis have been improved by position statements of scientific societies. Novel treatments for infant colic and inflammatory bowel diseases have emerged. Studies suggest the diagnostic utility of ultrasonography in diagnosing community-acquired pneumonia. Progress in infectious diseases should include the universal varicella vaccination of children. Recommendations on asphyxia and respiratory distress syndrome have been highlighted in neonatology. Studies have evidenced that malnutrition remains a common underestimated problem in developing countries, while exposure to cancer risk factors in children is not negligible in Western countries. Advances in our understanding of less common diseases such as cystic fibrosis, plastic bronchitis, idiopathic pulmonary hemosiderosis facilitate diagnosis and management. Researches have led to new therapeutic approaches in patent ductus arteriosus and pediatric malignancies.

  17. Pituitary disease in childhood: utility of magnetic resonance; Patologia hipofisaria en la edad pediatrica: unidad de la resonance magnetica

    Vela, A. C.; Oleaga, L.; Ibanez, A. M.; Campo, M.; Grande, D. [Hospital de Basurto. Bilbao (Spain)

    2000-07-01

    To assess the utility of magnetic resonance (MR) imaging in the study of pediatric patients with clinical suspicion of pituitary disease. We studied 18 patients aged 7 to 18 years.Fifteen had hormonal disturbances, two presented amenorrhea and 1 complained of headache, fever and symptoms of polyuria and polydipsia. All the patients were examined using a Siemens SP 42 1-Tesla MRI scanner. Sagittal and coronal T1-weighted spin-echo images were obtained; in addition T2-weighted spin-echo or fast spin-echo imaging was performed in ten cases and intravenous gadolinium was administered in nine. We found 9 patients with hypothalamic-pituitary dysgenesis, 2 with germinoma, 2 cases of pituitary hemosiderosis in patients with thalassemia, 2 cases of microadenoma, one abscess, one case of idiopathic central diabetes insipidus and one of Langerhans cell histiocytosis. MR enabled us to assess pituitary structural alterations in children with hypothalamic-pituitary hormone deficiencies. In our series of patients, hypothalamic-pituitary dysgenesiss was the most frequent cause of adenohypophyseal deficiencies, and most cases of central diabetes insipidus were secondary to masses in the sellar and suprasellar region. In patients with thalassemia, T2-weighted MR images showed the amount of iron deposited in adenophypophysis. Gadolinium-enhanced studies were useful in the study of masses and when the presence of microadenoma was suspected. (Author) 26 refs.

  18. IRON CHELATION THERAPY IN THALASSEMIA SYNDROMES

    Paolo Cianciulli

    2009-06-01

    Full Text Available Transfusional hemosiderosis is a frequent complication in patients with transfusion dependent chronic diseases such as  thalassemias and severe type of sickle cell diseases. As there are no physiological mechanisms to excrete the iron contained in transfused red cells (1 unit of blood contains approximately 200 mg of iron the excess of iron is stored in various organs. Cardiomyopathy is the most severe complication covering more than 70% of the causes of death of thalassemic patients. Although the current reference standard iron chelator deferoxamine (DFO has been used clinically for over four decades, its effectiveness is limited by a demanding therapeutic regimen that leads to poor compliance. Despite poor compliance, because of the inconvenience of subcutaneous infusion, DFO improved considerably the survival and quality of life of patients with thalassemia. Deferiprone since 1998 and Deferasirox since 2005 were licensed for clinical use. The oral chelators have a better compliance because of oral use, a comparable efficacy to DFO in iron excretion and probably a better penetration to myocardial cells. Considerable increase in iron excretion was documented with combination therapy of DFO and Deferiprone. The proper use of the three chelators will improve the prevention and treatment of iron overload, it will reduce  complications, and improve survival and quality of life of transfused patients

  19. Pegvisomant-Induced Cholestatic Hepatitis in an Acromegalic Patient with UGT1A1 ​⁎ 28 Mutation

    Maria Susana Mallea-Gil

    2016-01-01

    Full Text Available Pegvisomant (PEGv is a growth hormone receptor antagonist approved for the treatment of acromegaly; one of its documented adverse effects is reversible elevation of hepatic enzymes. We report a 39-year-old male acromegalic patient with a pituitary macroadenoma who underwent transsphenoidal surgery. The patient’s condition improved but GH and IGF-I levels did not normalize; as a consequence, we first administered dopamine agonists and then somatostatin receptor ligands (SRLs with poor response. PEGv 15 mg every other day was added to lanreotide 120 mg monthly. The patient developed a severe hepatitis five months after starting the combination therapy. Elevated ferritin, iron, and transferrin saturation suggested probable hepatitis due to haemochromatosis. We performed a liver biopsy which showed an acute cholestatic hepatitis consistent with toxic etiology. A heterozygous genotype UGT1A1​⁎28 polymorphism associated with Gilbert’s syndrome was also found in this Argentine patient. The predominant clinical presentation resembled an acute cholestatic hepatitis associated with severe hemosiderosis, a different and new pattern of PEGv hepatotoxicity.

  20. Fetal liver iron overload: the role of MR imaging

    Cassart, Marie; Avni, Freddy Efraim; Guibaud, Laurent; Molho, Marc; D'Haene, Nicky; Paupe, Alain

    2011-01-01

    To assess the potential role of MR imaging in the diagnosis of fetal liver iron overload. We reviewed seven cases of abnormal liver signal in fetuses referred to MR imaging in a context of suspected congenital infection (n = 2), digestive tract anomalies (n = 3) and hydrops fetalis (n = 2). The average GA of the fetuses was 31 weeks. The antenatal diagnoses were compared with histological data (n = 6) and postnatal work-up (n = 1). Magnetic resonance imaging demonstrated unexpected abnormal fetal liver signal suggestive of iron overload in all cases. The iron overload was confirmed on postnatal biopsy (n = 2) and fetopathology (n = 4). The final diagnosis was hepatic hemosiderosis (haemolytic anaemia (n = 2) and syndromal anomalies (n = 2)) and congenital haemochromatosis (n = 3). In all cases, the liver appeared normal on US. Magnetic resonance is the only imaging technique able to demonstrate liver iron overload in utero. Yet, the study outlines the fundamental role of MR imaging in cases of congenital haemochromatosis. The antenatal diagnosis of such a condition may prompt ante - (in the case of recurrence) or neonatal treatment, which might improve the prognosis. (orig.)

  1. Iron overload in patients with myelodysplastic syndromes: An updated overview.

    Moukalled, Nour M; El Rassi, Fuad A; Temraz, Sally N; Taher, Ali T

    2018-06-15

    Myelodysplastic syndromes (MDS) encompass a heterogeneous group of clonal hematopoietic stem cell disorders characterized by a broad clinical spectrum related to ineffective hematopoiesis leading to unilineage or multilineage cytopenias, with a high propensity for transformation to acute myeloid leukemia. Iron overload has been recently identified as one of the important conditions complicating the management of these diverse disorders. The accumulation of iron is mainly related to chronic transfusions; however, evidence suggests a possible role for ineffective erythropoiesis and increased intestinal absorption of iron, related to altered hepcidin and growth differentiation factor-15 levels in the development of hemosiderosis in patients with MDS. In addition to its suggested role in the exacerbation of ineffective erythropoiesis, multiple reports have identified a prognostic implication for the development of iron overload in patients with MDS, with an improvement in overall survival after the initiation of iron chelation therapy. This review includes a detailed discussion of iron overload in patients with MDS whether they are undergoing supportive therapy or curative hematopoietic stem cell transplantation, with a focus on the mechanism, diagnosis, and effect on survival as well as the optimal management of this highly variable complication. Cancer 2018. © 2018 American Cancer Society. © 2018 American Cancer Society.

  2. Fetal liver iron overload: the role of MR imaging

    Cassart, Marie; Avni, Freddy Efraim [Erasme Hospital, Medical imaging, Brussels, Brabant (Belgium); Guibaud, Laurent [Hopital femme mere enfant, Imagerie Pediatrique et Foetale, Lyon-Bron (France); Molho, Marc [C.H.I Poissy/St Germain-en-Laye, Imagerie Medicale, Poissy (France); D' Haene, Nicky [Erasme Hospital, Anatomopathology Department, Brussels (Belgium); Paupe, Alain [C.H.I Poissy/St Germain-en-Laye, Pediatrie, Poissy (France)

    2011-02-15

    To assess the potential role of MR imaging in the diagnosis of fetal liver iron overload. We reviewed seven cases of abnormal liver signal in fetuses referred to MR imaging in a context of suspected congenital infection (n = 2), digestive tract anomalies (n = 3) and hydrops fetalis (n = 2). The average GA of the fetuses was 31 weeks. The antenatal diagnoses were compared with histological data (n = 6) and postnatal work-up (n = 1). Magnetic resonance imaging demonstrated unexpected abnormal fetal liver signal suggestive of iron overload in all cases. The iron overload was confirmed on postnatal biopsy (n = 2) and fetopathology (n = 4). The final diagnosis was hepatic hemosiderosis (haemolytic anaemia (n = 2) and syndromal anomalies (n = 2)) and congenital haemochromatosis (n = 3). In all cases, the liver appeared normal on US. Magnetic resonance is the only imaging technique able to demonstrate liver iron overload in utero. Yet, the study outlines the fundamental role of MR imaging in cases of congenital haemochromatosis. The antenatal diagnosis of such a condition may prompt ante - (in the case of recurrence) or neonatal treatment, which might improve the prognosis. (orig.)

  3. Iron-Induced Damage in Cardiomyopathy: Oxidative-Dependent and Independent Mechanisms

    Elena Gammella

    2015-01-01

    Full Text Available The high incidence of cardiomyopathy in patients with hemosiderosis, particularly in transfusional iron overload, strongly indicates that iron accumulation in the heart plays a major role in the process leading to heart failure. In this context, iron-mediated generation of noxious reactive oxygen species is believed to be the most important pathogenetic mechanism determining cardiomyocyte damage, the initiating event of a pathologic progression involving apoptosis, fibrosis, and ultimately cardiac dysfunction. However, recent findings suggest that additional mechanisms involving subcellular organelles and inflammatory mediators are important factors in the development of this disease. Moreover, excess iron can amplify the cardiotoxic effect of other agents or events. Finally, subcellular misdistribution of iron within cardiomyocytes may represent an additional pathway leading to cardiac injury. Recent advances in imaging techniques and chelators development remarkably improved cardiac iron overload detection and treatment, respectively. However, increased understanding of the pathogenic mechanisms of iron overload cardiomyopathy is needed to pave the way for the development of improved therapeutic strategies.

  4. Hyperferritinemia without iron overload in patients with bilateral cataracts: a case series

    Mumford Andrew

    2011-09-01

    Full Text Available Abstract Introduction Hepatologists and internists often encounter patients with unexplained high serum ferritin concentration. After exclusion of hereditary hemochromatosis and hemosiderosis, rare disorders like hereditary hyperferritinemia cataract syndrome should be considered in the differential diagnosis. This autosomal dominant syndrome, that typically presents with juvenile bilateral cataracts, was first described in 1995 and has an increasing number of recognized molecular defects within a regulatory region of the L-ferritin gene (FTL. Case presentation Two patients (32 and 49-year-old Caucasian men from our ambulatory clinic were suspected as having this syndrome and a genetic analysis was performed. In both patients, sequencing of the FTL 5' region showed previously described mutations within the iron responsive element (FTL c.33 C > A and FTL c.32G > C. Conclusion Hereditary hyperferritinemia cataract syndrome should be considered in all patients with unexplained hyperferritinemia without signs of iron overload, particularly those with juvenile bilateral cataracts. Liver biopsy and phlebotomy should be avoided in this disorder.

  5. Hydrocephalus in a yellow-headed Amazon parrot (Amazona ochrocephala oratrix).

    Keller, Krista A; Guzman, David Sanchez-Migallon; Muthuswamy, Anantharaman; Forrest, Lisa J; Steinberg, Howard; Sladky, Kurt; Petersen, Sophie

    2011-09-01

    A 37-year-old female yellow-headed Amazon parrot (Amazona ochrocephala oratrix) was presented after a 4-month-period behavior change and intermittent episodes of obtunded mentation. Clinical findings on physical examination included ataxia, a weak grasp, and reluctance to move. Results of magnetic resonance imaging were consistent with severe hydrocephalus without evidence of cerebrospinal fluid obstruction. The bird was treated with tapering dosages of prednisolone over a 4-month period, during which time the episodes did not occur. Discontinuation of treatment was attempted several times but resulted in relapse. After 3.5 years of maintenance treatment with prednisolone, the bird was presented subsequent to a 5-hour episode of obtunded mentation and worsening neurologic signs. Despite increasing the dose of prednisolone and providing additional supportive care, the bird's condition worsened, and euthanasia was elected. Necropsy findings included severe hydrocephalus with significant loss of right cerebral parenchyma and no evidence of cerebrospinal fluid obstruction. Histologic examination of the remaining cerebral parenchyma revealed a moderate, multifocal, cellular infiltrate; encephalomalacia; fibrosis; and hemosiderosis in tissue adjacent to the distended ventricles. Other findings included hepatic vacuolar degeneration. Diagnostic imaging and postmortem findings were consistent with a diagnosis of hydrocephalus ex vacuo. To our knowledge, this is the first report of hydrocephalus in an Amazon parrot as well as the first report of hydrocephalus in any avian species associated with long-term follow-up and prolonged corticosteroid treatment.

  6. Pancreatic iron stores assessed by magnetic resonance imaging (MRI) in beta thalassemic patients

    Feitosa de Assis, Reijâne Alves; Ribeiro, Andreza Alice; Kay, Fernando Uliana; Rosemberg, Laércio Alberto; Nomura, Cesar Higa; Loggetto, Sandra Regina; Araujo, Aderson S.; Fabron Junior, Antonio; Pinheiro de Almeida Veríssimo, Mônica; Baldanzi, Giorgio Roberto; Espósito, Breno Pannia; Baroni, Ronaldo Hueb; Wood, John C.; Hamerschlak, Nelson

    2012-01-01

    Purpose: To assess the correlation between MRI findings of the pancreas with those of the heart and liver in patients with beta thalassemia; to compare the pancreas T2* MRI results with glucose and ferritin levels and labile plasma iron (LPI). Materials and methods: We retrospectively evaluated chronically transfused patients, testing glucose with enzymatic tests, serum ferritin with chemiluminescence, LPI with cellular fluorescence, and T2* MRI to assess iron content in the heart, liver, and pancreas. MRI results were compared with one another and with serum glucose, ferritin, and LPI. Liver iron concentration (LIC) was determined in 11 patients’ liver biopsies by atomic absorption spectrometry. Results: 289 MRI studies were available from 115 patients during the period studied. 9.4% of patients had overt diabetes and an additional 16% of patients had impaired fasting glucose. Both pancreatic and cardiac R2* had predictive power (p 2 = 0.20, p 2 = 0.09, p 2 = 0.14, p 2 = 0.03, p 2 of 0.91. Conclusions: Pancreatic iron overload can be assessed by MRI, but siderosis in other organs did not correlate significantly with pancreatic hemosiderosis.

  7. Effects of a single high dose of 55Fe in mice

    Laissue, J.A.; Burlington, H.; Cronkite, E.P.; Heldman, B.; Reincke, U.

    1979-01-01

    High doses of 55 Fe induced cytocide in maturing erythroid cells, due to the short-range deposition of decay energy. Organ damage at the time of death was evaluated in a group of 45 female mice of the C 57 BL/6 J strain given a single i.v. injection of 2,800 μCi, 1,400 μCi or 700 μCi of 55 FeCl 3 when 10 to 14 weeks old. A corresponding amount of cold iron was given to control animals by the same route. Radioiron-treated mice died spontaneously, or were killed when moribund. Mice given 2,800 μCi died after a median survival time of 27 days with severe depletion of hemopoietic cells in bone marrow and spleen, marked atrophy of lymphoid tissues and mild liver damage. After 1,400 μCi or 700 μCi, the median survival time was 117 and 439, respectively. In contrast, median survival was 847 days in control animals allowed to survive. In the two lower 55 Fe-dose groups, there was a dose-dependent pancytopenia. Atrophy of lymphoid tissues was moderate, and signs of liver damage slight. The degree of organ hemosiderosis in experimental and control animals was slight to moderate. Organ damage associated with deposition of cold iron was not apparent in tissue sections. Morphological signs of damage to non-hemopoietic organs such as the liver were not conspicuous. Direct radiation damage, primarily to the erythroid series, and competition for stem cells between the heavily depleted erythroid and the other hemopoietic cell lines must be considered among the possible factors leading to pancytopenia. Out of 14 55 Fe-treated mice who survived longer than 300 days developed tumors of hemopoietic and lymphoid tissues, or osteosarcomas. (orig./MG) [de

  8. Rare but Lethal Hepatopathy-Sickle Cell Intrahepatic Cholestasis and Management Strategies.

    Malik, Aamir; Merchant, Chandni; Rao, Mana; Fiore, Rosemary P

    2015-11-28

    Sickle cell disease can affect the liver by way of the disease process, including sickling in hepatic sinusoids, as well as its treatment, including repeated blood transfusions leading to hemosiderosis and hepatitis. Sickle cell intrahepatic cholestasis (SCIC) is an extreme variant of sickle cell hepatopathy, and is associated with high fatality. We present the case of a 31-year-old man with past medical history of sickle cell disease and cholecystectomy who was admitted with uncomplicated vaso occlusive crisis and during the hospital stay developed fever, upper abdominal pain, and jaundice. There was an accelerated rise in total bilirubin to 50 mg/dL, direct bilirubin 38 mg/dL, and Cr 3.0 mg/dL. Hb was 6.4 g/dL, reticulocyte count 16%, ALT 40 IU/L, AST 155 IU/L, ALP 320 IU/L, and LDH 475 IU/L. Hepatitis panel was negative and MRCP showed normal caliber of the common bile duct, with no obstruction. Exchange transfusion of 9 units of packed red blood cells led to great improvement in his condition. SCIC, unlike the other sickle cell hepatopathies, requires urgent and vigorous exchange transfusion. Renal impairment in SCIC has not been well studied but usually is reversible with the hepatic impairment, as in this case. Unresolved renal impairment requires dialysis and is associated with poor outcome. There is limited data on use of hydroxyurea to prevent SCIC, and liver transplant is associated with high mortality. A timely diagnosis of SCIC and appropriate management is life-saving.

  9. Feasibility Study of NMR Based Serum Metabolomic Profiling to Animal Health Monitoring: A Case Study on Iron Storage Disease in Captive Sumatran Rhinoceros (Dicerorhinus sumatrensis).

    Watanabe, Miki; Roth, Terri L; Bauer, Stuart J; Lane, Adam; Romick-Rosendale, Lindsey E

    2016-01-01

    A variety of wildlife species maintained in captivity are susceptible to iron storage disease (ISD), or hemochromatosis, a disease resulting from the deposition of excess iron into insoluble iron clusters in soft tissue. Sumatran rhinoceros (Dicerorhinus sumatrensis) is one of the rhinoceros species that has evolutionarily adapted to a low-iron diet and is susceptible to iron overload. Hemosiderosis is reported at necropsy in many African black and Sumatran rhinoceroses but only a small number of animals reportedly die from hemochromatosis. The underlying cause and reasons for differences in susceptibility to hemochromatosis within the taxon remains unclear. Although serum ferritin concentrations have been useful in monitoring the progression of ISD in many species, there is some question regarding their value in diagnosing hemochromatosis in the Sumatran rhino. To investigate the metabolic changes during the development of hemochromatosis and possibly increase our understanding of its progression and individual susceptibility differences, the serum metabolome from a Sumatran rhinoceros was investigated by nuclear magnetic resonance (NMR)-based metabolomics. The study involved samples from female rhinoceros at the Cincinnati Zoo (n = 3), including two animals that died from liver failure caused by ISD, and the Sungai Dusun Rhinoceros Conservation Centre in Peninsular Malaysia (n = 4). Principal component analysis was performed to visually and statistically compare the metabolic profiles of the healthy animals. The results indicated that significant differences were present between the animals at the zoo and the animals in the conservation center. A comparison of the 43 serum metabolomes of three zoo rhinoceros showed two distinct groupings, healthy (n = 30) and unhealthy (n = 13). A total of eighteen altered metabolites were identified in healthy versus unhealthy samples. Results strongly suggest that NMR-based metabolomics is a valuable tool for animal health

  10. Feasibility Study of NMR Based Serum Metabolomic Profiling to Animal Health Monitoring: A Case Study on Iron Storage Disease in Captive Sumatran Rhinoceros (Dicerorhinus sumatrensis.

    Miki Watanabe

    Full Text Available A variety of wildlife species maintained in captivity are susceptible to iron storage disease (ISD, or hemochromatosis, a disease resulting from the deposition of excess iron into insoluble iron clusters in soft tissue. Sumatran rhinoceros (Dicerorhinus sumatrensis is one of the rhinoceros species that has evolutionarily adapted to a low-iron diet and is susceptible to iron overload. Hemosiderosis is reported at necropsy in many African black and Sumatran rhinoceroses but only a small number of animals reportedly die from hemochromatosis. The underlying cause and reasons for differences in susceptibility to hemochromatosis within the taxon remains unclear. Although serum ferritin concentrations have been useful in monitoring the progression of ISD in many species, there is some question regarding their value in diagnosing hemochromatosis in the Sumatran rhino. To investigate the metabolic changes during the development of hemochromatosis and possibly increase our understanding of its progression and individual susceptibility differences, the serum metabolome from a Sumatran rhinoceros was investigated by nuclear magnetic resonance (NMR-based metabolomics. The study involved samples from female rhinoceros at the Cincinnati Zoo (n = 3, including two animals that died from liver failure caused by ISD, and the Sungai Dusun Rhinoceros Conservation Centre in Peninsular Malaysia (n = 4. Principal component analysis was performed to visually and statistically compare the metabolic profiles of the healthy animals. The results indicated that significant differences were present between the animals at the zoo and the animals in the conservation center. A comparison of the 43 serum metabolomes of three zoo rhinoceros showed two distinct groupings, healthy (n = 30 and unhealthy (n = 13. A total of eighteen altered metabolites were identified in healthy versus unhealthy samples. Results strongly suggest that NMR-based metabolomics is a valuable tool for

  11. Iron as a possible aggravating factor for osteopathy in itai-itai disease, a disease associated with chronic cadmium intoxication

    Noda, M.; Yasuda, M.; Kitagawa, M.

    1991-01-01

    Itai-itai disease is thought to be the result of chronic cadmium (Cd) intoxication. We examined 23 autopsy cases of itai-itai disease and 18 cases of sudden death as controls. Urine and blood samples from 10 patients were collected before they died and revealed the presence of severe anemia and renal tubular injuries. Undecalcified sections of iliac bone were stained with Aluminon reagent, and ammonium salt of aurintricarboxylic acid, and Prussian blue reagent in all cases of itai-itai disease. These two reagents reacted at the same mineralization fronts. X-ray microanalysis revealed the presence of iron at mineralization fronts in itai-itai disease. Five patients showed evidence of hemosiderosis in the liver, spleen, and pancreas, probably as a result of post transfusion iron overload. Renal calculi and calcified aortic walls were also stained with Prussian blue reagent in several patients. Neither ferritin nor transferrin were visualized at mineralization fronts in itai-itai disease by immunohistochemical staining. These results suggest that iron is bound to calcium or to calcium phosphate by a physicochemical reaction. A marked osteomalacia was observed in 10 cases of itai-itai disease by histomorphometry. Regression analyses of data from cases of itai-itai disease suggested that an Aluminon-positive metal inhibited mineralization and that renal tubules were injured. Since bone Cd levels were increased in itai-itai disease, it is likely that renal tubules were injured by exposure to Cd. Therefore, stainable bone iron is another possible aggravating factor for osteopathy in itai-itai disease, and a synergistic effect between iron and Cd on mineralization is proposed

  12. Treatment with hydroxyurea in thalassemia intermedia with paravertebral pseudotumors of extramedullary hematopoiesis.

    Cario, H; Wegener, M; Debatin, K-M; Kohne, E

    2002-08-01

    Excessive ineffective erythropoiesis in thalassemia intermedia may cause paravertebral pseudotumors of extramedullary hematopoiesis. Due to the proximity to the spinal canal, these paravertebral masses carry the risk of severe neurological damage. Treatment strategies include hypertransfusion, radiotherapy, and laminectomy. Hydroxyurea, stimulating fetal hemoglobin synthesis, may represent an alternative therapeutic approach. We report on a 26-year-old patient suffering from thalassemia intermedia with progressive anemia symptoms and presenting multiple intrathoracic paravertebral pseudotumors of extramedullary hematopoiesis. Hypertransfusion therapy and splenectomy were followed by regular transfusion (baseline hemoglobin 10 g/dl) and chelation with desferrioxamine. With this treatment, clinical symptoms disappeared, paravertebral hematopoietic masses did not progress, but severe hemosiderosis developed within a few years. Hydroxyurea therapy was initiated to increase the efficacy of erythropoiesis, thereby reducing the required transfusion volume but suppressing concomitantly further expansion of extramedullary hematopoiesis, and finally leading to a reduction of transfusional iron load. Treatment was started with 4 mg/kg per day and stepwise increased to 12.5 mg/kg per day. The fetal hemoglobin concentration increased from 4.5 to 5.5 g/dl after 1 year and to 9.9 g/dl after 2 years of treatment. The yearly transfusion volume was halved during the 1st year of treatment. At present, after 26 months of treatment, the patient has been transfusion-independent for 10 months. Serum ferritin levels decreased from 2844 to 1335 ng/ml. Size and shape of paravertebral hematopoietic pseudotumors remained stable. No side effects of hydroxyurea have been observed. In thalassemia intermedia patients with extramedullary hematopoiesis, hydroxyurea may lead to independence from regular transfusion therapy without further expansion of ectopic hematopoietic tissue.

  13. 2-keto-4-(methylthio)butyric acid (keto analog of methionine) is a safe and efficacious precursor of L-methionine in chicks.

    Dilger, Ryan N; Kobler, Christoph; Weckbecker, Christoph; Hoehler, Dirk; Baker, David H

    2007-08-01

    Relative bioefficacy and toxicity of Met precursor compounds were investigated in young chicks. The effectiveness of DL-Met and 2-keto-4-(methylthio)butyric acid (Keto-Met) to serve as L-Met precursors was quantified using Met-deficient diets of differing composition. Efficacy was based on slope-ratio and standard-curve methodology. Using L-Met as a standard Met source added to a purified diet, DL-Met and Keto-Met were assigned relative bioefficacy values of 98.5 and 92.5%, respectively, based on weight gain. Relative bioefficacy values of 98.5 and 89.3% were assigned to DL-Met and Keto-Met, respectively, when chicks were fed a Met-deficient, corn-soybean meal-peanut meal diet. Thus, both DL-Met and Keto-Met are effective Met precursor compounds in chicks. Additionally, growth-depressing effects of L-Met, DL-Met, and Keto-Met were compared using a nutritionally adequate corn-soybean meal diet supplemented with 15 or 30 g/kg of each compound. Similar reductions in weight gain, food intake, and gain:food ratio were observed for each compound. Subjective spleen color scores, indicative of splenic hemosiderosis, increased linearly (P Keto-Met to L-Met in vivo merely requires transamination, Keto-Met may prove to be a useful supplement not only in food animal production, but also as a component of enteral and parenteral formulas for humans suffering from renal insufficiency.

  14. Iron as a possible aggravating factor for osteopathy in itai-itai disease, a disease associated with chronic cadmium intoxication

    Noda, M.; Yasuda, M.; Kitagawa, M. (Toyama Medical and Pharmaceutical Univ. (Japan))

    1991-03-01

    Itai-itai disease is thought to be the result of chronic cadmium (Cd) intoxication. We examined 23 autopsy cases of itai-itai disease and 18 cases of sudden death as controls. Urine and blood samples from 10 patients were collected before they died and revealed the presence of severe anemia and renal tubular injuries. Undecalcified sections of iliac bone were stained with Aluminon reagent, and ammonium salt of aurintricarboxylic acid, and Prussian blue reagent in all cases of itai-itai disease. These two reagents reacted at the same mineralization fronts. X-ray microanalysis revealed the presence of iron at mineralization fronts in itai-itai disease. Five patients showed evidence of hemosiderosis in the liver, spleen, and pancreas, probably as a result of post transfusion iron overload. Renal calculi and calcified aortic walls were also stained with Prussian blue reagent in several patients. Neither ferritin nor transferrin were visualized at mineralization fronts in itai-itai disease by immunohistochemical staining. These results suggest that iron is bound to calcium or to calcium phosphate by a physicochemical reaction. A marked osteomalacia was observed in 10 cases of itai-itai disease by histomorphometry. Regression analyses of data from cases of itai-itai disease suggested that an Aluminon-positive metal inhibited mineralization and that renal tubules were injured. Since bone Cd levels were increased in itai-itai disease, it is likely that renal tubules were injured by exposure to Cd. Therefore, stainable bone iron is another possible aggravating factor for osteopathy in itai-itai disease, and a synergistic effect between iron and Cd on mineralization is proposed.

  15. Experimental infection in Cavia porcellus by infected Amblyomma ovale nymphs with Rickettsia sp. (Atlantic rainforest strain).

    Brustolin, Joice Magali; da Silva Krawczak, Felipe; Alves, Marta Elena Machado; Weiller, Maria Amélia; de Souza, Camila Lopes; Rosa, Fábio Brum; Cadore, Gustavo Cauduro; Dos Anjos Lopes, Sônia Terezinha; Labruna, Marcelo Bahia; Vogel, Fernanda Silveira Flores; de Avila Botton, Sônia; Sangioni, Luís Antônio

    2018-03-01

    This study describes experimental infection of guinea pigs (Cavia porcellus) infested with naturally infected Amblyomma ovale nymphs with Rickettsia sp. (Atlantic rainforest strain), and the capacity of A. ovale nymphs to transmit this bacterium. Twenty-six guinea pigs were divided into the following groups: G1, 10 animals infested with uninfected A. ovale nymphs; G2, 10 animals infested with nymphs infected with Rickettsia sp. (Atlantic rainforest strain); and G3, 6 animals without tick infestation. Blood samples were taken 7, 14, 21, and 28 days post-infestation for serological and hematological tests. For histopathological analysis and rickettsial DNA detection, fragments of the spleen, lung, brain, and liver were harvested after euthanasia. The average feeding period for nymphs was 6.6 days for G1 and 6 days for G2. Hemolymph and PCR assays, performed to detect the causative agent in ticks, indicated that in G1, all ticks were negative, and in G2, all nymphs were positive by PCR and 80% (8/10) was positive by hemolymph tests. The only clinical change was skin scarring at the tick attachment site. Hematological parameters indicated leukopenia and total plasma protein (TPP) increased with decreased platelets in G1. In G2, leukocytosis, neutrophilia, monocytosis, an increase in platelets, and reduced TPP were observed. Only G2 guinea pigs were seroconverted (80%; 8/10). Histopathology tests indicated mild, diffuse hemosiderosis and mild, multifocal, follicular hyperplasia in the spleen. Molecular analysis did not detect Rickettsia sp. DNA in C. porcellus tissues. We demonstrated the capacity of A. ovale nymphs to transmit Rickettsia sp. (Atlantic rainforest strain) to guinea pigs.

  16. Functional MR imaging of the liver; Funktionelle MR-Tomographie der Leber

    Wibmer, A.; Nolz, R.; Ba-Ssalamah, A. [Medizinische Universitaet Wien, Allgemeines Krankenhaus der Stadt Wien, Universitaetsklinik fuer Radiodiagnostik und Nuklearmedizin, Wien (Austria); Trauner, M. [Medizinische Universitaet Wien, Universitaetsklinik fuer Innere Medizin III, Klinische Abteilung fuer Gastroenterologie und Hepatologie, Wien (Austria)

    2015-12-15

    The diagnostics of diffuse liver disease traditionally rely on liver biopsies and histopathological analysis of tissue specimens. However, a liver biopsy is invasive and carries some non-negligible risks, especially for patients with decreased liver function and those requiring repeated follow-up examinations. Over the last decades, magnetic resonance imaging (MRI) has developed into a valuable tool for the non-invasive characterization of focal liver lesions and diseases of the bile ducts. Recently, several MRI methods have been developed and clinically evaluated that also allow the diagnostics and staging of diffuse liver diseases, e. g. non-alcoholic fatty liver disease, hepatitis, hepatic fibrosis, liver cirrhosis, hemochromatosis and hemosiderosis. The sequelae of diffuse liver diseases, such as a decreased liver functional reserve or portal hypertension, can also be detected and quantified by modern MRI methods. This article provides the reader with the basic principles of functional MRI of the liver and discusses the importance in a clinical context. (orig.) [German] Die Diagnostik diffuser Lebererkrankungen stuetzt sich klassisch auf die Leberbiopsie und deren histopathologische Analyse. Dieses Verfahren ist allerdings fuer die Patienten unangenehm und schmerzhaft, birgt v. a. bei Patienten mit Lebererkrankungen ein gewisses Risiko und eignet sich daher nur sehr eingeschraenkt zur Verlaufskontrolle bei chronischen Erkrankungen. Die Magnetresonanztomographie (MRT) der Leber nimmt schon jetzt eine zentrale Stellung in der Diagnostik von Raumforderungen der Leber und von Erkrankungen der Gallenwege ein. Darueber hinaus bietet dieses nichtinvasive Verfahren Moeglichkeiten, diffuse Erkrankungen des Leberparenchyms zu diagnostizieren und ihren Schweregrad abzuschaetzen, z. B. bei nichtalkoholischer Leberverfettung, Hepatitis, Leberfibrose, Zirrhose, Haemochromatose und Siderose und anderen. Folgen einer parenchymatoesen Lebererkrankung, wie die portale

  17. Pathology and epidemiology of natural West Nile viral infection of raptors in Georgia.

    Ellis, Angela E; Mead, Daniel G; Allison, Andrew B; Stallknecht, David E; Howerth, Elizabeth W

    2007-04-01

    Carcasses from 346 raptors found between August 2001 and December 2004 were tested for West Nile virus (WNV) using virus isolation and immunohistochemistry; 40 were positive for WNV by one or both methods. Of these 40 birds, 35 had histologic lesions compatible with WNV infection, one had lesions possibly attributable to WNV, and four had no histologic evidence of WNV. The most common histologic lesions associated with WNV infection were myocardial inflammation, necrosis, and fibrosis; skeletal muscle degeneration, inflammation, and fibrosis; and lymphoplasmacytic encephalitis. Other lesions included hepatitis, lymphoid depletion in spleen and bursa, splenic and hepatic hemosiderosis, pancreatitis, and ganglioneuritis. Gross lesions included calvarial and leptomeningeal hemorrhage, myocardial pallor, and splenomegaly. Red-tailed hawks (Buteo jamaicensis) (10/56), sharp-shinned hawks (Accipiter striatus) (8/40), and Cooper's hawks (Accipiter cooperii) (10/103) were most commonly affected. Also affected were red-shouldered hawks (Buteo lineatus) (2/43), an osprey (Pandion haliaetus) (1/5), barred owls (Strix varia) (4/27), a great horned owl (Bubo virginianus) (1/18), and eastern screech owls (Megascops asio) (4/42). Although birds were examined throughout the year, positive cases occurred only during the summer and late fall (June-December). Yearly WNV mortality rates ranged from 7-15% over the four years of the study. This study indicates trends in infection rates of WNV in raptorial species over a significant time period and supports the available information regarding pathology of WNV infection in Strigiformes and Falconiformes. Although many species tested were positive for WNV infection, severity of lesions varied among species.

  18. Evolution of equine infectious anaemia in naturally infected mules with different serological reactivity patterns prior and after immune suppression.

    Autorino, Gian Luca; Eleni, Claudia; Manna, Giuseppe; Frontoso, Raffaele; Nardini, Roberto; Cocumelli, Cristiano; Rosone, Francesca; Caprioli, Andrea; Alfieri, Lavinia; Scicluna, Maria Teresa

    2016-06-30

    Information on equine infectious anaemia (EIA) in mules, including those with an equivocal reaction in agar gel immunodiffusion test (AGIDT), is scarce. For this, a study was conducted to evaluate the clinical, viral loads and pathological findings of two groups of naturally infected asymptomatic mules, respectively with a negative/equivocal and positive AGIDT reactivity, which were subjected to pharmacological immune suppression (IS). A non-infected control was included in the study that remained negative during the observation period. Throughout the whole study, even repeated episodes of recrudescence of EIA were observed in 9 infected mules, independently from their AGIDT reactivity. These events were generally characterised by mild, transient alterations, typical of the EIA acute form represented by hyperthermia and thrombocytopenia, in concomitance with viral RNA (vRNA) peaks that were higher in the Post-IS period, reaching values similar to those of horses during the clinical acute phase of EIA. Total tissue viral nucleic acid loads were greatest in animals with the major vRNA activity and in particular in those with negative/equivocal AGIDT reactivity. vRNA replication levels were around 10-1000 times lower than those reported in horses, with the animals still presenting typical alterations of EIA reactivation. Macroscopic lesions were absent in all the infected animals while histological alterations were characterised by lymphomonocyte infiltrates and moderate hemosiderosis in the cytoplasm of macrophages. On the basis of the above results, even mules with an equivocal/negative AGIDT reaction may act as EIAV reservoirs. Moreover, such animals could escape detection due to the low AGIDT sensitivity and therefore contribute to the maintenance and spread of the infection. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

  19. Evaluation of plain radiograph in mitral stenosis related to hemodynamics

    Choe, Ku Ok; Suh, Jung Ho; Park, Chang Yun; Choi, Byung So

    1973-01-01

    produced progressively smaller degrees of dilatation of main pulmonary artery and right descending pulmonary artery. Quite frequently primary pulmonary arterial segment and right descending pulmonary artery were enlarge with all degrees of pulmonary hypertension and which made enlargement of pulmonary artery as a sign but little value in determining the degree of pulmonary hypertension. 3. Correlation of pressure of hemosiderosis and intraalveolar edema to hemodynamics: Hemosiderosis was found in 8 cases among 67 cases. No relationship was observed in between the existence of hemosiderosis and the height of pulmonary venous pressure or other hemodynamic factors. Intraalveolar edema was detected in 7 cases and no significant difference of hemodynamic data was observed whether it is present or not. But the present group all had pulmonary capillary wedge pressure exceeds 19 mmHg. 4. Correlation of Kerley's B line to hemodynamic data: Distinctively present septal line was found in 18 among 65 cases (27.3%): 3 cases in right side, only one case in left side and most of them, to the existence itself or detected number of Kerley's B line. But when septal line was found distinctly, pulmonary capillary wedge pressure was all exceeded to 20 mmHg except on case. 5. Correlation of pulmonary vascular pattern to hemodynamic data: For the estimation of hemodynamics the above radiographic findings were frequently fraught with overlapping or gave us only limited information. This pulmonary vascular patterns classified in table 1 including peripheral portion as well as central were aimed to compare accordingly with hemodynamic data. These group showed relative apparent separation in each group especially in mean pulmonary artery pressure: Most of mean pulmonary artery pressure was included in the range of below 30 mmHg in group 1, from 25 to 40 mmHg in group 2, from 30 to 55 mmHg in group 3, and above 50 mmHG in group 4. The pulmonary vascular resistance was roughly included below 300 dyne

  20. Evaluation of plain radiograph in mitral stenosis related to hemodynamics

    Choe, Ku Ok; Suh, Jung Ho; Park, Chang Yun; Choi, Byung So [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    1973-04-15

    produced progressively smaller degrees of dilatation of main pulmonary artery and right descending pulmonary artery. Quite frequently primary pulmonary arterial segment and right descending pulmonary artery were enlarge with all degrees of pulmonary hypertension and which made enlargement of pulmonary artery as a sign but little value in determining the degree of pulmonary hypertension. 3. Correlation of pressure of hemosiderosis and intraalveolar edema to hemodynamics: Hemosiderosis was found in 8 cases among 67 cases. No relationship was observed in between the existence of hemosiderosis and the height of pulmonary venous pressure or other hemodynamic factors. Intraalveolar edema was detected in 7 cases and no significant difference of hemodynamic data was observed whether it is present or not. But the present group all had pulmonary capillary wedge pressure exceeds 19 mmHg. 4. Correlation of Kerley's B line to hemodynamic data: Distinctively present septal line was found in 18 among 65 cases (27.3%): 3 cases in right side, only one case in left side and most of them, to the existence itself or detected number of Kerley's B line. But when septal line was found distinctly, pulmonary capillary wedge pressure was all exceeded to 20 mmHg except on case. 5. Correlation of pulmonary vascular pattern to hemodynamic data: For the estimation of hemodynamics the above radiographic findings were frequently fraught with overlapping or gave us only limited information. This pulmonary vascular patterns classified in table 1 including peripheral portion as well as central were aimed to compare accordingly with hemodynamic data. These group showed relative apparent separation in each group especially in mean pulmonary artery pressure: Most of mean pulmonary artery pressure was included in the range of below 30 mmHg in group 1, from 25 to 40 mmHg in group 2, from 30 to 55 mmHg in group 3, and above 50 mmHG in group 4. The pulmonary vascular resistance was roughly included below 300 dyne

  1. Intoxicação experimental por cebola, Allium cepa (Liliaceae, em gatos Experimental onion Allium cepa (Liliaceae poisoning in cats

    Rafael A. Fighera

    2002-04-01

    Full Text Available A intoxicação por cebola é relatada em várias espécies animais em muitas partes do mundo. O princípio tóxico (n-propil dissulfito presente na cebola causa a transformação da hemoglobina em metemoglobina. Para estudar os achados laboratoriais, de necropsia e histopatológicos da intoxicação por cebola em gatos, cinco gatos de quatro meses de idade receberam cada um uma dose única de 10g/kg de cebola desidratada por via oral. Um outro gato de mesma idade não recebeu a refeição com cebola e serviu como controle. Todos os cinco gatos desenvolveram sinais clínicos da toxicose; um deles morreu dentro de 24 horas após a ingestão da cebola. Os sinais clínicos incluíram apatia, taquicardia, taquipnéia e cianose. Os achados laboratoriais se caracterizavam por anemia hemolítica associada a corpúsculos de Heinz e metemoglobinemia. Os principais achados de necropsia foram esplenomegalia e sangue de cor marrom. Os achados histopatológicos foram hemossiderose e hematopoese extramedular no baço e fígado.Onion poisoning is reported worlwide in several animal species. The toxic principle (n-propyl dissulfide present in onions causes the transformation of hemoglobin in methemoglobin. In order to study the laboratory, gross and histopathological findings in onion poisoning in cats, five 4-month-old cats were fed a single dose of 10g/kg of dried-onion each. Another cat of the same age did not receive the onion meal and served as control. All five cats developed clinical signs of the toxicosis; one of them died within 24 hours of the ingestion of the onion meal. Clinical signs included apathy, tachycardia, tachypnea, and cyanosis. Laboratory findings included hemolytic anemia associated with Heinz bodies and methemoglobinemia. Main necropsy findings were splenomegaly and brown discoloration of blood. Histopathological findings included splenic and hepatic hemosiderosis and multifocal extramedullary hematopoiesis.

  2. Organ damage mitigation with the Baskent Sickle Cell Medical Care Development Program (BASCARE)

    Ozdogu, Hakan; Boga, Can; Asma, Suheyl; Kozanoglu, Ilknur; Gereklioglu, Cigdem; Yeral, Mahmut; Buyukkurt, Nurhilal Turgut; Solmaz, Soner; Korur, Aslı; Aytan, Pelin; Maytalman, Erkan; Kasar, Mutlu

    2018-01-01

    Abstract The Eastern Mediterranean is among the regions where sickle cell disease (SCD) is common. The morbidity and mortality of this disease can be postponed to adulthood through therapies implemented in childhood. The present study focuses on the organ damage-reducing effects of the Baskent Sickle Cell Medical Care Development Program (BASCARE), which was developed by a team who lives in this region and has approximately 25 years of experience. The deliverables of the program included the development of an electronic health recording system (PRANA) and electronic vaccination system; the use of low citrate infusion in routine prophylactic automatic erythrocyte exchange (ARCE) programs including pregnant women; the use of leukocyte-filtered and irradiated blood for transfusion; the use of magnetic resonance imaging methods (T2∗) for the management of transfusion-related hemosiderosis; and the implementation of an allogeneic hematopoietic stem cell transplantation protocol for adult patients. The sample was composed of 376 study subjects and 249 control subjects. The hospital's Data Management System and the central population operating system were used for data collection. BASCARE enabled better analysis and interpretation of complication and mortality data. Vaccination rates against influenza and pneumococcal disease improved (21.5% vs 50.8% and 21.5% vs 49.2%, respectively). Effective and safe ARCE with low citrate infusion were maintained in 352 subjects (1003 procedures). Maternal and fetal mortality was prevented in 35 consecutive pregnant patients with ARCE. Chelating therapy rates reduced from 6.7% to 5%. Successful outcomes could be obtained in all 13 adult patients who underwent allogeneic peripheral stem cell transplantation from a fully matched, related donor. No patients died by day 100 or after the first year. Cure could be achieved without graft loss, grades III to IV acute graft versus host disease, extensive chronic graft versus host disease, or

  3. Fatal inanition in reindeer (Rangifer tarandus tarandus: Pathological findings in completely emaciated carcasses

    Mørk Torill

    2007-09-01

    Full Text Available Abstract Background In a project to determine the causes of winter mortality in reindeer in Finnmark County, northern Norway, the most frequent diagnosis turned out to be complete emaciation, despite several of the reindeer having been given silage for up to 4 weeks before they died. The present paper describes autopsy results and other findings in these animals. Methods Autopsies were made of 32 reindeer carcasses, and 28 of these were diagnosed as completely emaciated based on lack of visible fat and serous atrophy of subepicardial and bone marrow fat. Other investigations of the carcasses included histology, bacteriology, parasitology (counting of macro parasites and faecal egg counting, analysis of vitamin E and selenium in liver, chemical and botanical analysis of rumen content, analysis of lipid content in femur bone marrow and estimation of muscle atrophy by use of a muscle index. Results Main findings were: Low carcass weight, severe muscle atrophy, hemosiderosis in liver and spleen, subcutaneous oedema (18% and effusions to body cavities (18%. Two types of lipofuscin granula were identified in the liver: One type occurred in liver endothelial cells of all carcasses, while the other type occurred in hepatocytes, and prevailed in adult animals. Abomasal haemorrhages, consistent with previously described stress lesions, was present in 68% of the carcasses. Diarrhoea occurred in 2 cases, and loose faecal consistency was associated with silage feeding. Rumen content was low in crude protein. Grass dominated rumen content in silage-fed carcasses, while reindeer on natural pastures had mainly woody plants, mosses and litter in rumen. Stem dominated the grass fraction in rumens with high grass content, indicating ruminal indigestion as a cause of emaciation in silage fed animals. Some cases had heavy infestation of parasites such as warble fly larvae (Hypoderma tarandi, throat bot larvae (Cephenemyiae trompe and lung nematodes. Conclusion Lack

  4. Radiologic findings of diffuse Pulmonary hemorrhage

    Seo, Mi Ra; Song, Koun Sik; Lee, Jin Seong; Lim, Tae Hwan [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

    1998-12-01

    To describe the chest radiographic and CT findings of diffuse pulmonary hemorrhage. Two radiologists retrospectively analysed the chest radiographic and CT findings of six patients with diffuse pulmonary hemorrhage. Using open lung biopsy(n=3D2) and transbronchial lung biopsy or bronchoalveolar lavage(n=3D4), diagnosis was based on the presence of hemosiderin-laden macrophage or intra-alveolar hemorrhage. Underlying diseases were Wegener's granulomatosis(n=3D2), antiphospholipid antibody syndrome(n=3D2), Henoch-Schonlein purpura(n=3D1), and idopathic pulmonary hemosiderosis(n=3D1). In all patients, sequential chest radiographs, obtained during a one to six-month period, were available. HRCT scans were obtained in five patinets, and conventional CT scans in one. Follow-up HRCT scans were obtained in two. We also analyzed the patterns of involvement, distribution and sequential changes in the pulmonary abnormalities seen on chest radiographs and CT scans. Chest radiographs showed multifocal patchy consolidation(n=3D6), ground-glass opacity(n=3D3), and multiple granular or nodular opacity(n=3D3). These lesions were intermingled in five patients, while in one there was consolidation only. Sequential chest radiographs demonstrated the improvement of initial pulmonary abnormalities and appearance of new lesions elsewhere within 5-6 days, though within 7-25 (average, 13) days, these had almost normalized. HRCT scans showed patchy consolidation(n=3D5), multiple patchy ground-glass opacity(n=3D5), or ill-defined air space nodules(n=3D4). These lesions were intermingled in five patients, and in one, ground-glass opacity only was noted. In two patients there were interlobular septal thickening and intalobular reticular opacity. The distribution of these abnormalities was almost always bilateral, diffuse with no zonal predominancy, and spared the apex of the lung and subpleural region were less affected. Although chest radiographic and CT findings of diffuse pulmonary

  5. Radiologic findings of diffuse Pulmonary hemorrhage

    Seo, Mi Ra; Song, Koun Sik; Lee, Jin Seong; Lim, Tae Hwan

    1998-01-01

    To describe the chest radiographic and CT findings of diffuse pulmonary hemorrhage. Two radiologists retrospectively analysed the chest radiographic and CT findings of six patients with diffuse pulmonary hemorrhage. Using open lung biopsy(n=3D2) and transbronchial lung biopsy or bronchoalveolar lavage(n=3D4), diagnosis was based on the presence of hemosiderin-laden macrophage or intra-alveolar hemorrhage. Underlying diseases were Wegener's granulomatosis(n=3D2), antiphospholipid antibody syndrome(n=3D2), Henoch-Schonlein purpura(n=3D1), and idopathic pulmonary hemosiderosis(n=3D1). In all patients, sequential chest radiographs, obtained during a one to six-month period, were available. HRCT scans were obtained in five patinets, and conventional CT scans in one. Follow-up HRCT scans were obtained in two. We also analyzed the patterns of involvement, distribution and sequential changes in the pulmonary abnormalities seen on chest radiographs and CT scans. Chest radiographs showed multifocal patchy consolidation(n=3D6), ground-glass opacity(n=3D3), and multiple granular or nodular opacity(n=3D3). These lesions were intermingled in five patients, while in one there was consolidation only. Sequential chest radiographs demonstrated the improvement of initial pulmonary abnormalities and appearance of new lesions elsewhere within 5-6 days, though within 7-25 (average, 13) days, these had almost normalized. HRCT scans showed patchy consolidation(n=3D5), multiple patchy ground-glass opacity(n=3D5), or ill-defined air space nodules(n=3D4). These lesions were intermingled in five patients, and in one, ground-glass opacity only was noted. In two patients there were interlobular septal thickening and intalobular reticular opacity. The distribution of these abnormalities was almost always bilateral, diffuse with no zonal predominancy, and spared the apex of the lung and subpleural region were less affected. Although chest radiographic and CT findings of diffuse pulmonary

  6. A Study of the Modulating Action of Quercetin on Biochemical and Histological Alterations Induced by Lead Exposure in the Liver and Kidney of Rats.

    Mohammed, Ghena M.; Sedky, Azza; Elsawy, Hany

    2017-06-30

    Lead is a highly toxic metal and a very potent poison. Lead poisoning is a serious condition but can be treated. Quercetin is a flavonoid with many beneficial uses. The aim of the present study was to investigate the possible modulating action of quercetin as a model of an antioxidant against the toxic effects of lead acetate on liver and kidneys of rats. Rats were randomly divided into four groups: (i) saline group (control); (ii) lead group received i.p. lead acetate (20 mg/kg b.w.); (iii) quercetin group received i.p. quercetin (50 mg/kg b.w.); (iv) lead and quercetin group received i.p. lead acetate (20 mg/kg b.w.) followed by i.p. quercetin (50 mg/kg b.w.) for 4 weeks. The lead concentrations were determined in the liver and kidney tissues. Liver marker enzymes, bilirubin, albumin, total protein, creatinine, uric acid and urea, were assessed in the serum and light microscopic studies were performed. The results showed that lead acetate administration was associated with an increase in serum alkaline phosphatase (ALP), alanine aminotransferase (ALT), aspartate aminotransferase (AST) activities, total bilirubin, creatinine, uric acid, urea levels. Lead accumulation in kidneys and liver tissues was also found, but were associated with decrease in albumin and total protein in comparison with the respective mean values of the control. Lead acetate caused numerous histological alterations in the liver, including chronic inflammation, bilary hyperplasia, edema, congestion, Kupffer cells hyperplasia and hemosiderosis, and in the kidney, including tubular dilation, atrophy of glomerular tuft, widening of urinary space and mild fibroblast. In contrary, administration of lead acetate along with quercetin partially restored the studied parameters to normal values and improved structure of liver and kidney with significant decreases in the severity of histopathological changes when compared with the lead acetate group. In conclusion, treatment with quercetin may provide a

  7. O papel da oxigenação hiperbárica na estrutura do fígado e baço após ligadura das veias hepáticas: estudo em ratos The role of hyperbaric oxygenation in the liver and spleen structure after hepatic vein ligation: study in rats

    Ricardo Costa-Val

    2006-03-01

    and 15 minutes at the onset and end of the therapy, in a total of 120 minutes, in order to promote the gradual compression and decompression in 20 consecutive days. On the 21st preoperative day, the animals were sacrificed by ether inhalation and submitted to laparotomy and stripping of liver and spleen for histological study. The results of the histological study of livers and spleens were compared using Fisher's exact test. Statistically significant difference was considered when P < 0.05. RESULTS: The histological studies made in the livers and spleens of animals from both groups showed the following alterations: presence of thrombosis of hepatic, portal and central lobular veins in five (33.3% group 1 animals and absence in group 2 animals; very extensive necrosis of liver cells in seven (46.7% group 1 animals, and light in eight (53.3%, whereas for all group 2 animals such alteration was considered light; Kupffer cells developed and hypertrophied in 14 (93.3% group 1 animals and slightly developed and hypertrophied in all group 2 animals; high congestion of the spleen purple in six (40% and moderate in nine (60% group 1 animals, whereas all group 2 animals had moderate congestion; moderate or severe hemosiderosis in 14 (93.3% group 1 animals and mild hemosiderosis in all group 2 animals. The statistical analyses performed between both groups showed significant differences (P < 0.05 for all variables. CONCLUSIONS: The hyperbaric oxygen therapy applied in rats submitted to hepatic vein ligation mitigated its early deleterious effects on the liver and spleen, which was confirmed by the histological study.

  8. Intoxicação por larvas de Perreyia flavipes em bovinos na região sul do Rio Grande do Sul Perreyia flavipes larvae poisoning in cattle in southern Rio Grande do Sul, Brazil

    Mauro P. Soares

    2008-03-01

    enlarged with increased lobular pattern, the mesenteric lymph nodes were edematous and Peyer patches of the small gut were depressed. Petechial hemorrhages and ecchymosis were observed in the mesentery and abomasum. On histologic examination the liver showed centrolobular or massive necrosis. Hemosiderosis and necrosis of the germinative centers of lymph nodes, white pulp of the spleen and Peyer patches were also observed. The poisoning occurred probably due to an intense drought in October-December 2005, when the insect stayed as a cocoon underground. The dry conditions probably avoided a higher number of adult sawflies to emerge from the cocoons, what resulted in greater egg production. The large amount of decaying grass due to almost normal rain fall during summer seemed to have provided favorable environmental conditions for the development of larvae in winter.

  9. Opacidades em vidro fosco nas doenças pulmonares difusas: correlação da tomografia computadorizada de alta resolução com a anatomopatologia Ground-glass opacity in diffuse lung diseases: high-resolution computed tomography-pathology correlation

    Maria Lúcia de Oliveira Santos

    2003-12-01

    , association with other radiological, clinical and pathological findings must be considered for an accurate diagnostic interpretation. In this paper were reviewed 62 computed tomography examinations of patients with diffuse pulmonary diseases of 14 different etiologies in which ground-glass opacity was the only or the most remarkable finding, and correlated this findings with pathology abnormalities seen on specimens obtained from biopsies or necropsies. In pneumocystosis, ground-glass opacities correlated histologically with alveolar occupation by a foaming material containing parasites, in bronchioloalveolar cell carcinoma with thickening of the alveolar septa and occupation of the lumen by mucus and tumoral cells, in paracoccidioidomycosis with thickening of the alveolar septa, areas of fibrosis and alveolar bronchopneumonic exsudate, in sarcoidosis with fibrosis or clustering of granulomas and in idiopathic pulmonary fibrosis with alveolar septa thickening due to fibrosis. Alveolar occupation by blood was found in cases of leptospirosis, idiopathic hemosiderosis, metastatic kidney tumor and invasive aspergillosis whereas oily vacuoli were seen in lipoid pneumonia, proteinaceus and lipoproteinaceus material in silicoproteinosis and pulmonary alveolar proteinosis, and edematous fluid in cardiac failure.

  10. Cirrose hepática em cães: 80 casos (1965-2003 Hepatic cirrhosis in dogs: 80 cases (1965-2003

    Marcia C. Silva

    2007-11-01

    .8%], icterus (19/80 [23.8%], anorexia (13/80 [16.2%], neurological disturbances (12/80 [15.0%], dyspnea (12/80 [15.0%] and subcutaneous edema (10/80 [12.5%]. In 63 dogs for which there was a description of gross morphology in the necropsy reports, 76.2% had macronodular and 23.8% had micronodular cirrhosis. In 14 cases in which the histopathology of the liver was reviewed different degrees of fibrosis were observed: mild in 57.2%, moderate in 21.4%, or marked in 21.4%; in these cases there were no correlation with the degree of fibrosis and other associated histological changes such as lipidosis, biliary ducts hyperplasia, inflammation, bilestasis, hemosiderosis and random hepatocellular necrosis. Extra hepatic changes included ascites (39/63 [61.9%], icterus (19/63 [30.2%], status spongiosus (15/63 [23.8%], hydrothorax (12/63 [19.0%], subcutaneous edema (10/63 [15.9%], portosystemic shunts (11/63 [17.5%], gastric our duodenal ulceration (11/63 [17.5%] and cholemic nephrosis (4/63 [6.3%].