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Results for “HEMOSIDEROSIS”

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PubMed2005

Variability in hepatic iron concentration in percutaneous needle biopsy specimens from patients with transfusional hemosiderosis.

In patients with sickle cell disease or beta-thalassemia receiving RBC transfusions for a long period, a precise knowledge of the liver iron concentration (LIC) is essential for treatment. Patients underwent LIC and liver pathology assessment by duplicate biopsies in 2 passes from the same local liver site. Fresh tissue cores in trace element-free containers and tissues from dissolved paraffin-embedded cores were analyzed. LIC measurements in each of 2 paraffin-embedded cores did not differ significantly (median, 12,455 vs 12,153 microg/g dry weight; n = 29). A significant difference was observed when 1 fresh tissue sample and 1 paraffin-embedded core were analyzed (median, 11,716 vs 12,864 microg/g dry weight; n = 16; P < .001) with a median disagreement between LIC measurements of 23.0%. We found high agreement in LICs between liver biopsy specimens processed by the paraffin-embedding technique but overestimation of LICs in comparison with desiccated fresh tissue samples.

Biopsy, Needle↗
PubMed1999

Prognosis in pediatric idiopathic pulmonary hemosiderosis.

STUDY OBJECTIVES: Previously, IPH patients have been reported to have an average survival of 2.5 years. However, at our institution, many IPH patients have survived longer than that. Therefore, we conducted this study to determine the clinical course and current mortality of pediatric IPH patients treated with immunosuppressants. DESIGN: Retrospective chart review. SETTING: Children's hospital. PARTICIPANTS: Seventeen patients in whom IPH was diagnosed between 1972 and 1998. MEASUREMENTS AND RESULTS: Mean age at diagnosis was 4.5 +/- 3.5 years, and 12 patients were female. At diagnosis, all patients had anemia and pulmonary infiltrates; 85% had hypoxemia, 65% had hemoptysis, and 70% had fever. The diagnosis was made by open lung biopsy in 13 patients (76%), hemosiderin-laden macrophages in BAL fluid in 1 patient (6%), hemosiderin-laden macrophages in gastric aspirate in 2 patients (12%), or by clinical presentation alone in 1 patient (6%). The mean duration of follow-up for all patients was 3.6 +/- 3.4 years (range, 0.7 to 10.2). Initial treatment consisted of prednisone only in 14 patients (82%), and prednisone and hydroxychloroquine in two patients (12%). Thirteen patients (76%) required long-term corticosteroids because of recurrent hemoptysis. Eight patients (47%) required other immunosuppressants (hydroxychloroquine or azathioprine) in addition to prednisone to control their hemoptysis. One patient who was not treated with prednisone remained asymptomatic for 1.8 years. Three patients (17%) died of acute massive pulmonary hemorrhage (4.1 +/- 5.0 years postdiagnosis). CONCLUSION: Five-year survival for IPH patients in our study was 86% (by Kaplan-Meier method). We conclude that these IPH patients who received long-term treatment had a better outcome than those previously reported who were not treated with extended courses of immunosuppressive therapy. We speculate that long-term immunosuppression therapy may improve the prognosis in IPH.

Adolescent↗
PubMed1991

Long-term clinical follow-up of adult idiopathic pulmonary hemosiderosis and celiac disease.

A 22-year-old man with a history of repeated whole blood transfusions since the age of 7, was admitted to the hospital for dyspnea and blood-stained sputum. A complete blood cell count, a test for reticulin antibodies, an x-ray film of the chest, a transbronchial biopsy and a biopsy of the small bowel were performed and the results confirmed the diagnosis of IPH, associated with CD. The patient has been asymptomatic for four years on a gluten-free diet. In cases of IPH, it may be important to investigate the coexistence of CD because treatment of the latter could lead to a better prognosis.

Adult↗
PubMed1999

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.

Air Microbiology↗
PubMed1980

Experimental hemosiderosis: relationship between skin pigmentation and hemosiderin.

Iron (totalling 7.5 mg per mouse in three doses) was injected into hairless mice to determine the relationship between skin pigmentation and hemosiderin deposition. The skin color reached its maximum 24 to 48 hours after the last injection and then gradually faded over the subsequent 8 months. In the skin, hemosiderin granules were present extracellularly between collagen bundles as well as within dermal macrophages, Langerhans cells and indeterminate dendritic cells of the epidermis. A larger amount of iron was deposited in the facial than in the dorsal skin, resulting in darker pigmentation of the former. This study suggests that brownish discoloration of skin in hemochromatosis might be attributable in some degree to accumulation of hemosiderin and that pronounced hyperpigmentation of the face in hemochromatosis might be due to increased activation of melanocytes by a high content of hemosiderin.

Animals↗