Cases reported "Hemosiderosis"

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1/40. Isolation of stachybotrys from the lung of a child with pulmonary hemosiderosis.

    Recently, stachybotrys atra, a toxigenic fungus, has been implicated as a potential cause of pulmonary hemorrhage/hemosiderosis in infants living in water-damaged homes. Although epidemiologic evidence supports this association, neither the organism nor its toxic products has ever been recovered from humans. We report the first case in which stachybotrys was isolated from the bronchoalveolar lavage fluid of a child with pulmonary hemorrhage. stachybotrys was also recovered from his water-damaged home. The patient recovered completely after his immediate removal from the environment and subsequent cleaning of his home. This case provides further evidence that this fungus is capable of causing pulmonary hemorrhage in children.
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2/40. Idiopathic pulmonary hemosiderosis with cystic lesions: a rare presentation.

    This report describes a case of a 49-year-old man with cough, recurrent hemoptysis, and dyspnea during 18 months, presenting with radiological findings of alveolar infiltrate and cystic lesions in left upper lobe. Laboratory studies revealed normocytic hypochromic anemia and normal coagulation tests. c-reactive protein and mucoproteins were negative. serum protein electrophoresis and complement, urinalysis, serum creatinine, creatinine clearance, and 24-hour urine protein were normal. Tests for antineutrophil cytoplasmic antibodies and anti-glomerular-basement membrane antibodies were negative. Tests for connective tissue diseases were all negative. Histological findings were consistent with those of idiopathic pulmonary hemosiderosis. Radiological findings are discussed.
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3/40. Alveolar capillary basement membrane lesions in Goodpasture's syndrome and idiopathic pulmonary hemosiderosis.

    An ultrastructural study of lung distinguised between lesions of the alveolar capillary basement membrane in a case of Good pasture's syndrome and in three cases of idiopathic pulmonary hemosiderosis. In Goodpasture's syndrome, diffuse vascular injury with wide endothelial gaps, diffusely fragmented basement membranes and an electron dense layer on the basement membrane was found. In idiopathic pulmonary hemosiderosis, focal ruptures of the basement membrane were associated with hydropic changes in pneumocytes and, although fibroblasts were not seen, collagen deposition occurred within the basement membrane. immunofluorescent studies failed to show deposition of immunoglobulins G (IgG), A (IgA), M (IgM) or C3 in the lung in either disease. The ultrastructural lesions appear to separate these clinically similar entities.
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4/40. Idiopathic pulmonary hemosiderosis in a child: report of one case.

    Idiopathic pulmonary hemosiderosis (IPH) is uncommon in children. We report a 3-year-old girl who was presented with acute pale-looking appearance, hemoptysis, hematemesis and shortness of breath. This patient was confirmed to have pulmonary hemorrhage by the presence of hemosiderin-laden macrophages in the bronchoalveolar lavage fluid using a flexible bronchoscope. Other causes of PH including glomerular, cardiac and immunological disorder were excluded by normal laboratory studies. She was primarily treated by oral prednisolone, but due to recurrent hemoptysis, immunosuppressive agent was added for maintenance therapy. Pediatricians should consider PH in a patient who has recurrent dyspnea, hemoptysis and iron deficiency anemia.
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5/40. Massive pulmonary hemorrhage in a Saudi female with primary antiphospholipid syndrome.

    The antiphospholipid syndrome is characterized by the presence of antiphospholipid antibodies and the association of protean clinical manifestations as a result of both venous and arterial thrombosis. Because diffuse alveolar hemorrhage leading to acute respiratory failure is a rather unusual complication of antiphospholipid antibodies, this diagnosis may be overlooked or its manifestations are attributed to another disease. Presented here is a young Saudi female with primary antiphospholipid syndrome who recovered after a stormy clinical course of acute respiratory failure in the intensive care unit.
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6/40. Idiopathic pulmonary hemosiderosis in an adult. Favourable response to azathioprine.

    Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder characterised by intermittent, diffuse alveolar hemorrhage (DAH). Although an inflammatory pulmonary capillaritis can be evidenced in most patients with DAH, IPH is a distinct entity in which pulmonary inflammatory alterations are lacking. Most cases occur in children, although the disease has been exceptionally reported in adults too. Here, we, describe a case of IPH in a 30-year-old woman who was admitted to our hospital because of recurrent episodes of hemoptysis since the age of 21. IPH was diagnosed on the basis of: 1) an open lung biopsy showing focal alveolar edema and hemorrhage without parenchymal inflammatory alterations, 2) a bronchoalveolar lavage showing hemosiderin-laden macrophages, and 3) exclusion of infectious or immunologic causes of hemoptysis. prednisone administration could control the disease, but every attempt to lower the dose to less than 25 mg per day was followed by recurrence of hemoptysis. Then, azathioprine was started, and after three months prednisone was gradually tapered to the dose of 10 mg per day, without any relapse of the disease. These findings indicate that azathioprine, in combination with prednisone, may be an effective therapy for IPH and suggest that an immunologic mechanism could be involved in the pulmonary capillary damage underlying alveolar bleeding.
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keywords = alveolar
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7/40. serum histamine-releasing activity in a patient with idiopathic pulmonary haemosiderosis.

    BACKGROUND: idiopathic pulmonary haemosiderosis (IPH) is a rare disorder characterized by intermittent, diffuse alveolar bleeding. The pathogenesis of the diseases is unclear, although an association with milk or gluten hypersensitivity has been described, and an immune-mediated damage of alveolar capillaries has been suggested. A previous report showed the release of histamine after cow's milk intake in a newborn with cow's milk intolerance and IPH. methods AND RESULTS: here, we report the detection of serum histamine-releasing activity (HRA) in a 30-year-old woman with IPH. The serum taken during an active phase of the disease induced histamine release from basophils of two normal donors; conversely, when the patient was receiving prednisone and azathioprine, and the disease was in remission, the serum HRA was reduced. serum fractions with a MW lower than 100 kDa displayed an enhanced HRA; in contrast, serum fractions with MW above 100 kDa were not able to induce histamine release, suggesting that the activity was due to a cytokine and not to an immunoglobulin. CONCLUSIONS: the detection of serum HRA provides further evidence that the immune system is activated in the course of IPH and supports an immunologic basis for the alveolar capillary damage, which is responsible for alveolar bleeding.
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8/40. mitral valve prolapse with pulmonary haemosiderosis and severe anaemia: cause or association?

    A 12 years boy presented with the history of pallor for one month. Two days before hospitalisation he developed fever, cough, shortness of breath. He had past history of such episode.On examination, his heart rate was found to be 120/minute, respiratory rate 40/minute and moderate anaemia was detected. Scattered creptus was audible over mid and lower lung fields and a soft systolic murmur was auscultated at apex. On investigations, Hb was found as 4.6 g/dl and HbF was less than 2%. plasma Hb was 5 g/dl. Straight x-ray chest showed bilateral patchy opacities over mid and lower zones. His sputum was found to be blood stained and prompted the possibility of blood loss occurring in the lungs, which was confirmed by demonstrating haemosiderin laden macrophages on three consecutive sputum specimens. echocardiography revealed a systolic displacement of mitral valve leaflets into the left atrium with co-optation superior to the plane of mitral annulus. Doppler study showed a minimal late systolic regurgitation. It was decided to treat the case as idiopathic pulmonary haemosiderosis. oxygen inhalation, hypertonic saline nebulisation, i.v. hydrocortisone, packed cell transfusion followed by oral prednisolone improved the patient's condition. After 3 months of discontinuing prednisolone, he remained asymptomatic. Here one case of pulmonary haemosiderosis characterised by abnormal accumulation of haemosiderin in the lungs following repeated alveolar haemorrhages with the presence of mitral valve prolapse is reported.
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9/40. Pitfalls in the diagnosis of idiopathic pulmonary haemosiderosis.

    Idiopathic pulmonary haemosiderosis is a very rare but devastating disorder. diagnosis is sometimes difficult and the clinical course exceedingly variable, as illustrated by this report of a girl, aged 2 years 4 months, with severe iron deficiency anaemia. There was no response to iron therapy and transfusions. Sustained and striking reticulocytosis associated with low haptoglobin mimicked haemolytic anaemia. Positive faecal blood test was documented after repeated testing. There were no pulmonary symptoms. A chest radiograph showed bilateral diffuse alveolar infiltrates. bronchoalveolar lavage fluid showed numerous siderophages. High resolution computed tomography of the thorax revealed early pulmonary fibrotic changes. Recurrent reticulocytosis appeared to be a very useful sign of recurrent bleeding episodes.
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keywords = alveolar
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10/40. Clinical profile of 30 infants with acute pulmonary hemorrhage in Cleveland.

    Between 1993 and 2000, 30 infants were hospitalized with acute pulmonary hemorrhage at Rainbow Babies and Children's Hospital in Cleveland. Most infants presented with severe pulmonary symptoms requiring intensive support, but a few infants had less severe hemorrhage. Three quarters of the patients required ventilator support and blood transfusions. Eleven patients had transitory hemoglobinuria. Five patients died, but infants who survived did well. There are currently no specific treatment modalities, although we have advised moving to a different home and avoiding environmental tobacco smoke. Subsequently, rebleeding from the lower respiratory tract has decreased from 5 of 7 infants to 1 in 21. On the basis of decreased subsequent fatal hemorrhage, high dose glucocorticoids seem to be of some value. Several patients revealed continued low-grade alveolar hemorrhage for months after their initial bleed, even after removal from their original home environments.
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