Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “HEMOSIDEROSIS”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,675 records · Page 93Linked to original sources
PubMed1998

Idiopathic pulmonary haemosiderosis. Epidemiology, pathogenic aspects and diagnosis.

Idiopathic pulmonary haemosiderosis (IPH) is a rare clinical entity characterized by recurrent episodes of diffuse alveolar haemorrhage, often presenting with haemoptysis. Many patients have iron deficiency anaemia due to deposition of haemosiderin iron in the alveoli, and eventually develop moderate pulmonary fibrosis. Typically, intensive search for an aetiology ends up negative. There is no evidence of pulmonary vasculitis or capillaritis. The aetiology is obscure, but may be an immunological or toxic mechanism causing a defect in the basement membrane of the pulmonary capillary. IPH affects both children and adults. During an acute episode, a chest X-ray demonstrates bilateral, alveolar infiltrates. Sputum examination discloses haemosiderin-laden alveolar macrophages. Diagnosis is established by lung biopsy (fiber-optic or thoracoscopic), showing large numbers of haemosiderin-laden macrophages in the alveoli and without evidence of capillaritis or deposition of immunoglobulins. Corticosteroids and/or immunosuppressive drugs may be effective during an acute bleeding episode, and may in some patients improve symptoms and prognosis on the long-term, but the response to treatment displays great interindividual variation.

Adrenal Cortex Hormones↗
PubMed1998

Endothelial activation in patients with chronic venous disease.

OBJECTIVES: Leukocyte trapping due to leukocyte-endothelial activation has been implicated as the cause of lipodermatosclerosis and ulceration in patients with chronic venous disease. We investigated endothelial activity in normal controls and patients subjected to short-term venous hypertension. METHODS: Twenty-five normal volunteers and 30 patients with chronic venous disease divided into two groups: varicose veins with skin changes (LDS, n = 15); and varicose veins without skin changes (VVs, n = 15) were studied. Blood samples were taken from a foot vein before and after experimental venous hypertension. Plasma levels of ELAM-1 (endothelial leukocyte adhesion molecule-1), ICAM-1 (intercellular adhesion molecule-1), VCAM-1 (vascular cell adhesion molecule-1), and von Willebrand factor (vWf) was measured by an ELISA. RESULTS: There was a significant rise in the plasma concentration of ELAM-1, ICAM-1 and VCAM-1 in patients and normal controls in response to venous hypertension. Basal levels of plasma VCAM-1 and vWf were higher in patients with LDS compared to patients with VVs. The magnitude of rise of VCAM-1 was greater in patients with LDS compared to patients with VVs (p = 0.01, Mann-Whitney U-test). There was no difference in the basal levels or in the magnitude of change in plasma ICAM-1 and ELAM-1 between the two patient groups. CONCLUSION: Venous hypertension results in endothelial activation which may aid endothelial-leukocyte adhesion. Patients with LDS exhibit increased VCAM-1, which is a counterligand for receptors expressed by monocytes and lymphocytes signifying that these cells may be more important in the development of skin changes.

Adult↗
PubMed2004

[Persistent hemoptysis secondary to extensive epithelioid angiosarcoma].

Pulmonary angiosarcoma is an unusual cause of diffuse pulmonary hemorrhage. Angiosarcomas are rare malignant vascular tumors accounting for 1% to 2% of all sarcomas. Angiosarcomas have been detected in nearly all organs, but lung involvement is unusual, accounting for less than 7%. The literature describes approximately 10 isolated cases of primary pulmonary angiosarcoma as opposed to the more common metastatic type. Given that primary and metastatic types are clinicopathologically similar, the presence of a distant primary sarcoma must be ruled out before a diagnosis of primary pulmonary angiosarcoma can be made. A pathological diagnosis requires a finding of polygonal or oval cells with atypical irregular nuclei and vascular spaces lined with such cells surrounded by hemorrhagic phenomena. Immunohistochemical analysis is positive for specific endothelial cell markers such as CD31 and factor VIII, and coexpression of keratin is a frequent finding.

Aged↗
PubMed1998

Idiopathic neonatal hepatitis presenting as neonatal hepatic siderosis and steatosis.

Idiopathic neonatal hepatitis (INH) is a heterogeneous disease of undetermined cause. We report a retrospective histologic reevaluation of INH. Sixty patients with INH were reviewed along with 32 biliary atresia (BA) patients. Histologic findings, iron and fat deposits, giant cell transformation, portal fibrosis, and bile duct proliferation were semiquantitatively graded from 0 to 4+. Significant histologic findings were defined as > or =2+. Frequencies of patients with significant histologic findings in the INH group were compared with those of the BA group. Among the patients with significant histologic findings, those in the INH group had significantly less iron deposits (P < 0.01), portal fibrosis (P < 0.01), and bile duct proliferation (P < 0.01) than those of the BA group. A combination of significant hepatic macrovesicular steatosis and siderosis was observed in 10 INH patients but not in any BA patient (10/60 vs 0/32, P < 0.05). Without extensive treatment, the 10 INH patients all recovered, and hepatic abnormalities normalized by the age of 12 months. In conclusion, the present study showed that the recognition of hepatic siderosis is helpful to distinguish BA from INH and that in a subset of INH patients hepatic macrovesicular steatosis and siderosis occurs.

Biliary Atresia↗
PubMed2003

Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches.

OBJECTIVE: Superficial siderosis of the central nervous system (CNS) is a rare chronic progressive disorder caused by chronic subarachnoid hemorrhage. We present four patients with superficial siderosis of the CNS to describe the characteristic symptoms, and to discuss the pathogenetic heterogeneity and possible new therapeutic approaches. RESULTS: The causes of chronic subarachnoid bleeding in superficial siderosis were different. In two patients surgical treatment of ependymoma or cerebral cavernomas were the underlying diseases. No cause was detected in one patient. For the first time, we present one patient with vasculitis of the central nervous system associated with systemic hemochromatosis in superficial siderosis. Therapeutic approaches included exstirpation of cavernomas as the source of chronic bleeding in one patient, immunosuppressive therapy and venupunctures in the patient with vasculitis and hemochromatosis, and symptomatic treatment with chelating agents and antioxidants. The patients remained clinically stable for the follow-up period of up to 2 years. CONCLUSIONS: Our cases underline the pathogenetic heterogeneity of superficial siderosis and favor the early diagnosis for prompt initiation of therapy. Besides treatment of the underlying condition, antioxidants and radical scavengers may be effective in halting the progression of the disease.

Adult↗
PubMed2001

Utility of HHV8 RNA detection for differentiating Kaposi's sarcoma from its mimics.

BACKGROUND: The diagnostic distinction of atypical vascular lesion or angiomatoid/hemosiderotic dermatofibroma and Kaposi's sarcoma can be difficult, especially in AIDS patients. Given the strong association between human herpes virus 8 (HHV8) and Kaposi's sarcoma, this study attempts to determine if HHV8 RNA detection by reverse transcription (RT) in situ polymerase chain reaction (PCR) could help in differentiating these entities. METHODS: Twenty-three Kaposi's sarcoma cases, 13 dermatofibromas, including 7 angiomatoid or hemosiderotic variants and eight atypical vascular lesions were tested for HHV8 by RT in situ PCR; five of the patients in the latter two groups were known to have AIDS. RESULTS: HHV8 RNA was detected in each of the 23 cases considered on histologic grounds to represent Kaposi's sarcoma; viral RNA and DNA localized to the majority of the endothelial and stromal spindle cells. None of the cases of dermatofibroma were HHV8 positive, whereas two of the atypical vascular lesions were viral positive, leading to a final diagnosis of Kaposi's sarcoma. CONCLUSIONS: RT in situ PCR for HHV8 RNA is a useful tool in the differential diagnosis between Kaposi's sarcoma and its mimics.

Acquired Immunodeficiency Syndrome↗
PubMed2000

Hemosiderotic fibrohistiocytic lipomatous lesion: ten cases of a previously undescribed fatty lesion of the foot/ankle.

We address the clinicopathologic features of a previously undescribed heavily-pigmented spindle cell proliferation within a circumscribed benign lipomatous lesion that occurs mainly in the ankle region of older females. Patients with "lipoma with fibrohistiocytic proliferation" were retrieved from our files. Slides and clinical information were reviewed, and immunohistochemistry was performed (n = 5). Ten patients with hemosiderotic fibrohistiocytic lipomatous lesions were identified. All cases demonstrated a well-circumscribed fatty lesion with random focal proliferations of plump, slightly pleomorphic spindled cells, scattered inflammatory cells, and abundant iron pigment. The spindled cells had vesicular nuclei with indistinct nucleoli; occasional hyperchromatism was observed. No nuclear cytoplasmic inclusions were identified. The spindled component had a reactive appearance. In most cases, the fatty component, with homogeneously sized adipocytes, predominated. The lesions occurred in the foot/ankle region (8/10, one each cheek and hand) of primarily females (8/10) with a mean age of 50.6 years (range 42-63 years), size of 7.7 cm (range 2.5-17 cm), and prior duration of 3.1 years. Seven of eight patients had a history of prior trauma. The spindled component was positive for vimentin, calponin, CD34, and occasionally KP-1 or lysozyme and negative for caldesmon, S100, and desmin. Follow-up on eight patients revealed four with recurrences or residual disease over three years, requiring re-excision. No cases metastasized or caused patient death (mean 12 years, range 1-23 years). We describe a predominantly fatty lesion that is hemosiderin rich with a "fibrohistiocytic" proliferation, composed of histiocytes, myofibroblasts, and C34-positive fibroblasts, which occurs predominantly in the ankle region of middle-aged females. We believe that this is a reactive process due to antecedent trauma, the inflammatory cells, hemosiderin, mixed spindled cells, and homogeneous non-neoplastic appearance of the fat. HFLL can be distinguished from previously described lesions. Correct identification of hemosiderotic fibrohistiocytic lipomatous lesion is important, as it may locally recur.

Adult↗
PubMed2006

Overcoming barriers that inhibit proper treatment of anemia.

Intravenous (i.v.) iron and recombinant human erythropoietin (EPO), like all other medications, are associated with the risk of adverse events. Historically, the primary concern with iron therapy has been the possibility of iron overload, which exposes the individual to the effects associated with nontransferrin-bound iron. Experience with EPO use has demonstrated an association with hypertension and with the upregulation of a number of markers of inflammation. The impact of these potential adverse effects merits careful analysis, given that both i.v. iron and EPO are designed for long-term use in a patient population at high risk for infection and cardiovascular disease. However, the incidence of iron overload and the risks associated with nontransferrin-bound iron have dramatically been reduced since the introduction of EPO therapy, and no data exist that demonstrate a definitive association between i.v. iron and an increased risk of morbidity related to infection or cardiovascular disease. On the other hand, EPO use is associated with hypertension, endothelial dysfunction, and prothrombotic and inflammatory states in hemodialysis patients. Risks associated with hypertension can be minimized by using the lowest effective EPO dose, which may be achieved through the regular use of i.v. iron. Judicious use of both i.v. iron and EPO may optimize cardiovascular outcomes.

Anemia, Iron-Deficiency↗
PubMed2006

Iron indices: what do they really mean?

Routine monitoring of body iron stores is an essential component of overall management for the patient on hemodialysis. Adequate iron levels are important for the prevention and treatment of iron-deficiency anemia, which is associated with reduced physical functioning, cardiovascular disease, and poor quality of life. Hemodialysis patients are at especially high risk for iron-deficiency anemia, owing to continuous blood losses and supraphysiologic levels of erythropoiesis driven by recombinant human erythropoietin therapy. Unfortunately, the accurate determination of iron status in these patients can be a challenging task, which is made more difficult by inflammation, infections, and the large number of comorbid conditions that can affect commonly used indices of body iron stores. Despite their limitations, transferrin saturation (TSAT) and serum ferritin remain the cornerstones of iron status assessment. Because these values can be altered by a number of non-iron-related factors, it is necessary to go beyond these measures and draw upon additional sources of information to determine the patient's iron status. Other important factors to consider when assessing the need for iron therapy include evidence of underlying inflammatory processes that may block iron mobilization and distort the standard iron indices, the results of alternative iron indices, and the patient's recent history of iron administration. Frequently, the response to a gram of intravenous (i.v.) iron is a safe and effective way to determine the role of iron deficiency in the anemia of the problematic patient. The chronic inflammatory state associated with malnutrition and clinical or subclinical infections substantially increases the risk of misdiagnosing the patient with iron overload and may place the patient at risk of iron deficiency owing to inappropriate withdrawal of i.v. iron therapy. To avoid the risks of withholding iron therapy, the nephrologist must keep this relationship in mind whenever serum ferritin testing suggests replete iron stores, whereas TSAT testing suggests insufficient iron availability.

Anemia, Iron-Deficiency↗