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Pulmonary hemosiderosis and immune thrombocytopenia. Initial manifestations of collagen-vascular disease.

Severe iron deficiency anemia, pulmonary infiltrates, and cutaneous hemorrhage associated with thrombocytopenia developed in a 7-year-old girl. A typical clinical course and the presence of abundant hemosiderinladen macrophages in the gastric juice and alveoli suggested a diagnosis of idiopathic pulmonary hemosiderosis (PH). Investigation of the marked thrombocytopenia, not previously reported as a finding in IPH, resulted in the demonstration of antiplatelet antibody in the patient's serum and on her platelets. Response to corticosteroid administration and splenectomy was consistent with idiopathic thrombocytopenic purpura (ITP). The IPH and ITP may have occurred coincidentally in this patient, but certain pathophysiological similarities between these two disorders and ultimate development of a poorly defined fatal diffuse connective tissue disorder suggest that thrombocytopenia and intrapulmonary hemorrhage were related.

Anemia, Hypochromic↗

[Fahr disease and idiopathic pulmonary hemosiderosis in a 10 year old patient (author's transl)].

A 10 year old patient with acute respiratory insufficiency, dispnea, cough, temperature, cyanosis and hemoptisis is presented. Cranial X-rays and scanning revealed basal ganglia calcifications. Intense hypocalcemia and hyperphosphoremia were found as well as response to parathyroid hormone administration. A diagnosis of primary hypoparathyroidism and idiopathic pulmonary hemosiderosis was made. These two diseases are associated and related because of the autoimmune nature of both of them.

Calcinosis↗

[Cor triatriatum--a rare cause of pulmonary hemosiderosis].

Pneumological examinations including open lung biopsy performed on a male patient of 30 years of age suffering from severe respiratory distress that disabled him, as well as from massive recurring attacks of hemoptysis, resulted in suspicion of idiopathic pulmonary hemosiderosis (also known as Ceelen-Gellerstedt's syndrome). Diagnosis of cor triatriatum followed by surgery was arrived at only after a pulmonary oedema had developed and after other rare cardiac diseases had been considered. This rare congenital malformation--which occasionally becomes clinically manifest only in the adult--should be suspected in differential diagnosis of respiratory distress and a sometimes also life-threatening hemoptysis. Echocardiography is the diagnostic method of choice in this regard.

Adult↗

Pulmonary hemosiderosis and immune complex glomerulonephritis.

Two children with a syndrome of pulmonary hemorrhage and immune complex nephritis are reported. Clinical history suggests that pulmonary lesions precede renal abnormalities. Necrotizing glomerulonephritis with granular immune deposits along the glomerular basement membrane was found. Although the etiology of this disease complex is still unknown, the clinical and pathological findings in these patients suggest that immune complex glomerulonephritis is an unusual complication of idiopathic pulmonary hemosiderosis.

Anti-Glomerular Basement Membrane Disease↗

Decrease in serum ferritin level in a patient with HCV hepatitis and liver hemosiderosis by interferon-alpha.

We describe here a patient with chronic hepatitis C and liver hemosiderosis whose serum ferritin level was notably reduced by long-term interferon-alpha (IFN alpha) therapy. The decrease of the elevated serum ferritin level was considered to have been mostly obtained by the improvement of liver dysfunction. However, at the beginning of the therapy, in spite of alanine aminotransferase (ALT) improvement, his serum ferritin level increased transiently, and after cessation of IFN alpha therapy, the serum ALT increased again, but the serum ferritin had not increased. This indicates that IFN alpha has an effect on the iron-related measurement, partly due to improvement of hepatic status.

Alanine Transaminase↗

Hepatic hemosiderosis and Klebsiella bacteremia in a green aracari (Pteroglossus viridis).

A green aracari (Pteroglossus viridis) was presented for necropsy after being found dead; no previous clinical signs had been noted. Microscopic examination revealed multifocal necrosis of the liver, spleen, and lung compatible with an acute bacteremia. Klebsiella pneumoniae was isolated from the liver, kidney, and intestine. Histopathological and toxicological findings also reflected a concurrent hepatopathy due to excess iron accumulation. Hepatic hemosiderosis has been reported in mynahs, birds of paradise, and quetzals but has not previously been reported in the green aracari.

Animals↗

[A case of idiopathic pulmonary hemosiderosis of adult onset].

A 69-year-old woman suddenly suffered massive hemoptysis and was admitted to the hospital. The test of anti-glomerular basement membrane antibodies was negative. Chest radiograph showed diffuse infiltrative shadows similar to those of lung edema in the both lung fields. The patient's condition worsened gradually during the next 3 weeks, with repeated massive hemoptysis. Steroid pulse therapy had limited effects on the progressive respiratory failure, and the patient died. Autopsy showed alveolar hemorrhage and macrophages containing haemosiderin. Immunofluoresence microscopy showed no deposits of immunoglobulin in the kidney. Idiopathic pulmonary hemosiderosis of adult onset with acute respiratory failure is rare in Japan.

Age of Onset↗

Superficial hemosiderosis of the central nervous system. A case report.

A case of idiopathic superficial hemosiderosis (SH) of the central nervous system and a review of the literature are presented. The patient suffered from progressive cerebellar ataxia, hearing loss, anosmia, spastic paraparesis, but no mental deterioration. The diagnosis was made with brain and spinal MRI, that showed in T2 weighted images superficial hypointensity of spinal cord, medulla oblungata, pons, mesencephalon, cerebellum and cerebral hemispheres, images that are considered pathognomonic of SH. Repeated spinal fluid examinations were negative, suggesting that evidence of overt subarachnoidal bleeding is not essential in the diagnosis. In patients with SH of unknown etiology no valid therapy is yet available.

Aged↗

[Idiopathic pulmonary hemosiderosis, celiac disease and cardiomyopathy].

UNLABELLED: BACKGROUND--Idiopathic pulmonary hemosiderosis (IPH), a rare and possibly immune disease, is sometimes associated with coeliac disease and myocardiopathy. CASE REPORTS: CASE NO 1--A 2 year-old boy with IPH was investigated because he suffered from frequent, soft stools. Small bowel biopsy showed partial villous atrophy. Circulating gliadin antibodies were present. The patient was placed on a gluten-free diet. CASE NO 2--An 8 year-old girl was admitted because she suffered from severe anemia (Hb: 4 g/100 ml). She was found to have IPH and myocardiopathy. She had no manifestation, but a systematic search for coeliac disease was positive (total villous atrophy; presence of circulating gliadin and alveolar basement membrane antibodies). The patient was placed on a gluten-free diet, prednisone and diuretics, but she died during a relapse 2 months later. CONCLUSION--It is worthwhile checking for coeliac disease in all patients with IPH. The presence of myocardiopathy is a negative prognosis.

Cardiomyopathies↗

[Idiopathic pulmonary hemosiderosis with good course in the adult: an atypical form?].

We present the cases of two adult patients hospitalized due to alveolar hemorrhage. In the absence of data regarding the affection of other organs and after a follow-up of 20 months and 4 years respectively, they were diagnosed as idiopathic pulmonary hemosiderosis (IPH). These patients represent two opposed extremes in the clinical spectrum of the disease, yet none of them needed therapy once they overcame the acute episode, remaining asymptomatic. Although the usual clinical course of IPH involves a short survival, given the evolution of our cases and of others previously described in the literature, one may think that at least a subgroup of adult patients have just one alveolar hemorrhagic episode as the result of the exposition to an unknown inhaled stimulus.

Adult↗

[A case of superficial hemosiderosis of the central nervous system with normal CSF findings and unknown source of bleeding].

A 65-year-old woman had suffered from slowly progressive hearing loss for one year. She had neither repeated episodes of headache or vomiting nor a past history of neurosurgical operation. Neurologic examination revealed moderate diminution in hearing, pyramidal tract sign and cerebellar ataxia without dementia. CSF was under normal pressure, clear and colorless, with total protein 35 mg/dl, glucose 59 mg/dl and a cell count of 2 WBC/mm3. T2-weighted images (TR200/TE80) of high-field MRI demonstrated marginal hypointensity of the brainstem, the Sylvian fissures and the entire spinal cord. Angiography of the cerebral vessels failed to identify the source of bleeding. To our knowledge, this is the first report of superficial hemosiderosis of the central nervous system with normal CSF findings and an unknown source of bleeding confirmed by MRI.

Aged↗

[Idiopathic pulmonary hemosiderosis].

We report a case of a 23 year-old man admitted to hospital with cough, fever and recurrent episodes of haemoptysis. Laboratory findings, which included a pulmonary biopsy, established the diagnosis of Idiophatic Pulmonary Hemosiderosis (IPH). Despite a number of morphologic, immunologic and ultrastructural studies, the etiology and pathogenesis of this disease remain indetermined. To diagnose an IPH all the other causes of pulmonary hemorrhage must be excluded. This is a rare disease, even more rare in adults. Based on these facts the authors publish this article, which includes the case report and a literature review.

Adult↗

[Idiopathic pulmonary hemosiderosis. Clinical and radiological assessment of re-exacerbation].

Predominant characteristics of idiopathic pulmonary hemosiderosis (IPH), a rare pathology of unknown etiology, are recurrent alveolar hemorrhage, hemoptysis and iron deficiency anemia. No evidence of vascular disorders, infections, cancer, pulmonary embolus, veno-occlusive diseases must also be considered for the diagnosis. A case of chronic IPH with long asymptomatic periods and stages of riacutization with severe dyspnoea, high fever, cough with rusty coloured spitting, asthenia and serious respiratory insufficiency is described. The patient adds to our understanding in one of such riacutization in that she agreed to high-resolution computed tomography (HRCT) testing, in addition to common routine testing. It became possible to underline the importance of HRCT both in the diagnosis of IPH without hemoptysis, awaiting invasive investigations like fiberoptic bronchoscopy and lung biopsy, and in the clinical evaluation of the riacutization. Such analysis leads to forwarding the installment of the most appropriate therapy and to the limitation of fibrotic evolution, when possible.

Acute Disease↗

Hemosiderosis in a patient on regular hemodialysis: treatment by desferrioxamine.

Hemosiderosis following regular administration of parenteral iron was observed in a patient receiving maintenance hemodialysis. Infusions of desferrioxamine in doses of 2,3 and 4 g each resulted in the removal of approximately 45 mg of iron during dialysis. Desferrioxamine 2 g was infused thrice weekly during dialysis for twelve months. Body iron stores, as judged by liver iron and serum ferritin concentrations, fell by about half. This agrees well with the result calculated from the amount of iron administered and the amount removed during dialysis.

Deferoxamine↗

[Idiopathic primary pulmonary hemosiderosis. Report of three cases (author's transl)].

Three cases of idiopathic primary pulmonary hemosiderosis are reported. Hemosiderin was demonstrated in macrophages (siderophages) in all three cases. In one of them, isotopic examination with radioiron and in an other one, a pulmonary biopsy were performed. All the patients received corticosteroids. The first case had a good evolution in spite of five years without therapy. The second case showed a bad therapeutic response and required immunosuppressive drugs. The third case with a short follow-up is doing well. Literature is reviewed, and considerations about etiopathogeny, clinical features, roentgenographic and therapeutic aspects of the disease are made.

Biopsy↗

Unusual combination of total occlusion of left main coronary artery and heart failure with pulmonary hemosiderosis: case report.

A case of total occlusion of the left main coronary artery, congestive heart failure, and pulmonary hemosiderosis in a 54-year-old man is reported. Cardiac catheterization showed total occlusion of the left main coronary artery, subtotal occlusion of the right coronary artery, severely deranged hemodynamics, and an akinetic left ventricle except for a hypokinetic posterobasal segment. A radionuclide left ventricular performance study revealed an ejection fraction of 0.16 with diffuse biventricular hypokinesis and dilatation. Despite all the risk factors, the patient underwent a total of six saphenous vein grafts without perioperative or immediate postoperative complications.

Journal Article↗