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 109 records · Page 6Linked to original sources
PubMed2006

Extracorporeal membrane oxygenation to rescue profound pulmonary hemorrhage due to idiopathic pulmonary hemosiderosis in a child.

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage (DAH) in pediatric patients. During the acute phase, death due to massive alveolar hemorrhage and subsequent severe respiratory failure with associated multiple organ failure often occurs. We report the case of an 11-year-old girl who developed severe hypoxemic respiratory failure and pulmonary hemorrhage. Extracorporeal membrane oxygenation (ECMO) was instituted on the next day because medical treatment and mechanical ventilation failed to maintain oxygenation. She was successfully decannulated 5 days later without ECMO-related complications. Four months later, she was intubated again and the diagnosis of IPH was made by open lung biopsy. She was treated with systemic steroid therapy and discharged smoothly. We suggest that ECMO provides a chance of recovery and survival for patients with IPH, even if accompanied by severe pulmonary hemorrhage.

Child↗
PubMed1983

Prognostic criteria in idiopathic pulmonary hemosiderosis in children.

An epidemiologic survey was undertaken of 30 children in whom idiopathic pulmonary hemosiderosis (IPH) had been identified. In determining the prognostic significance of various parameters, a clinical severity score was used. This included the year of onset of IPH, fever, difficulty in breathing, the severity of the anemia, the roentgenographic findings and therapeutic modalities such as transfusions, administration of steroids, antibiotic treatment and splenectomy. Eighteen patients had died. The results of this investigation suggest that: (1) The severity of the disease at its onset does not determine the survival; (2) Females survived longer; (3) Young age of the patients at the onset of IPH seems to carry a less favorable prognosis; (4) The common therapeutic modalities in use have not proved to be beneficial. We believe that some environmental insult to the developing lung in genetically predisposed persons may contribute to a higher morbidity in the younger patients.

Adolescent↗
PubMed1985

Evidence of subclinical extrapyramidal hemosiderosis in cystic fibrosis.

We are presenting evidence of subclinical extrapyramidal hemosiderosis in chronic cystic fibrosis (CF) by histological staining and quantitative analysis. Comparison was made between age-matched control (eight cases) and CF brains (14 cases). None of the CF patients had extrapyramidal or other focal CNS symptoms but showed an increased iron pigment, and four cases had a few dystrophic axons (DA) in pallidonigral areas. Histological stain revealed significantly increased iron pigment in pallidonigral and subthalamic nuclei (P less than 0.05, 0.01, 0.01, respectively). The pigment was located in astrocytes, macrophages, neuronal perikarya, and neuropils. The increase was directly proportional to age and numbers of DA in nucleus gracilis/cuneatus. As in previous reports [5, 22], the numbers of DA in nucleus gracilis/cuneatus were higher in CF (P less than 0.01) and varied directly with age (correlation coefficient 0.72). Quantitative analysis disclosed an upward trend of mean iron content in pallidonigral areas. Aluminum was detected in four cases of CF but not in the controls.

Adolescent↗
PubMed1993

Transfusional hemosiderosis in sickle cell anemia: another cause of an echogenic pancreas.

We report four sickle cell anemia patients who have received multiple blood transfusions and have been non-compliant on Desferal chelation therapy. Abdominal ultrasonography demonstrated an echogenic pancreas in all four patients. Magnetic resonance imaging in three patients revealed decreased signal intensity in all sequences in the pancreas and liver. All four patients had marked iron deposition on liver biopsy. To our knowledge, increased echogenicity of the pancreas secondary to hemosiderosis (2 degrees hemochromatosis) in sickle cell patients has not been reported in the radiologic literature.

Adolescent↗
PubMed1987

Exacerbation of idiopathic pulmonary hemosiderosis in pregnancy.

Idiopathic pulmonary hemosiderosis (IPH) is a very rare disease in adults. Its occurrence in pregnancy has been described only once. A case of a 16-yr-old patient with an exacerbation of IPH during pregnancy resulting in intrauterine fetal death in the 28th week of gestation is described. The deterioration of the IPH is most probably related to the hemodynamic alterations which are at their maximum during the seventh month of pregnancy. Although rare, the case suggests that women with IPH should be followed carefully during pregnancy and, in the case of a deterioration in the patient's condition despite immunosuppressive therapy, termination of pregnancy should be considered.

Adolescent↗
PubMed1995

Superficial hemosiderosis in a second trimester fetus: pathological and clinical manifestations.

Prenatal brain hemorrhages are associated with considerable morbidity and mortality in neonates. They appear predominantly as bleeding into periventricular germinal matrix with subsequent hemorrhages into lateral ventricles and subarachnoid space. Other patterns of brain hemorrhage are not widely documented in second trimester fetuses. We report a case of hemorrhage presenting as extensive hemosiderin deposits in leptomeninges and adjacent brain parenchyma, as observed in superficial hemosiderosis. The lesion was associated with substantial tissue damage. Clinically, it was diagnosed during the second half of second trimester by successive ultrasound examinations. If differs from germinal matrix hemorrhage and other forms of hemorrhage in origin, pathogenesis, morphology, and probably in clinical manifestations.

Adult↗
PubMed1979

Hemosiderosis of urinary bladder.

We believe this is the first case of proved hemosiderosis of the bladder to be reported. The clinical presentation, radiographic findings, and cystoscopic appearance suggested a primary bladder neoplasm.

Cystoscopy↗
PubMed1998

A novel splicing mutation in the ceruloplasmin gene responsible for hereditary ceruloplasmin deficiency with hemosiderosis.

Hereditary ceruloplasmin deficiency with hemosiderosis (aceruloplasminemia) is a newly recognized autosomal recessive disorder of copper-iron metabolism due to mutations in the ceruloplasmin (Cp) gene. We report here a novel mutation in the Cp gene in a 54-year-old Japanese woman with this disease. She showed clinical triad; diabetes mellitus, retinal degeneration and neurological disorder in her middle age. Laboratory findings were characteristic for no detectable serum ceruloplasmin and increased serum ferritin. Liver biopsy revealed excessive storage of iron in hepatocytes and magnetic resonance imaging of the brain was indicative of increased iron content in the basal ganglia, thalamus and dentate nucleus. The a-->g substitution at the splice acceptor site of the intron 6 (1209-2) caused a 8-bp deletion in Cp mRNA by defective splicing, resulting in a premature termination codon at the amino acid position 388. Truncation of Cp, even if effectively translated, may cause loss of its normal function because of drastic change in its triangular structure.

Alternative Splicing↗
PubMed1992

The use of nuclear magnetic resonance imaging in monitoring total body iron in hemodialysis patients with hemosiderosis treated with erythropoietin and phlebotomy.

Two hemodialysis patients with hemosiderosis were treated with combined erythropoietin and repeated phlebotomy. Serial nuclear magnetic resonance (NMR) imaging and serum ferritin levels were used to monitor the efficacy of treatment. This treatment modality has definite advantages over chronic deferoxamine therapy. NMR image-derived parameters offer an objective, accurate, and noninvasive indication of tissue iron stores.

Adult↗
PubMed1999

[Idiopathic pulmonary hemosiderosis in a patient of advanced age].

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology characterized by hemoptysis, diffuse pulmonary infiltrates and iron-deficiency anemia. It is typically found in children and young adults. Finding that the concentration of siderophages exceeds 20% in bronchoalveolar lavage indicates a diagnosis of IPH. We report the case of a 72-year-old man with repeated diagnoses of respiratory infection, bronchiectasis and melena. Because such a clinical picture is rare for IPH, the final diagnosis was obtained by open lung biopsy.

Aged↗
PubMed2005

[Idiopathic pulmonary hemosiderosis in adults].

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage characterized by abnormal deposition of hemosiderin iron in the macrophages of alveoli. Most cases occur in children. In adults, IPH is rare: almost 10 cases reported during the late 10 years. We report the case of a 20-year-old-girl with IPH. There was no evidence of pulmonary vasculitis or capillaritis. Long-term treatment with systemic corticosteroids was followed by clinical remission lasting 4 years.

Adult↗
PubMed1995

A mutation in the ceruloplasmin gene is associated with systemic hemosiderosis in humans.

We identified a mutation in the ceruloplasmin (Cp) gene in a Japanese family with aceruloplasminemia, some of whose members showed extrapyramidal disorders, cerebellar ataxia, and diabetes mellitus. A post-mortem study of the proband revealed excessive iron deposition mainly in the brain, liver and pancreas. The G to A transition at the splice acceptor site introduces a premature termination codon at the amino acid position 991 by defective splicing, thereby truncating the carboxyl terminus of Cp in affected individuals. We conclude that the mutation in the Cp gene is associated with systemic hemosiderosis in humans.

Alternative Splicing↗
PubMed2002

[Hemochromatosis or hemosiderosis? Initial misinterpretation of clinical symptoms and laboratory findings in a 62-year-old patient].

The exact differential diagnosis of iron overload syndromes is mandatory as important therapeutic consequences may derive from a correct diagnosis, especially when hemochromatosis is present. To facilitate diagnostic and therapeutic decisions algorithms and probabilistic calculations based on different frequencies of clinical symptoms and typical laboratory findings of the diseases in question have been proposed. Overestimation and/or underestimation of clinical symptoms and/or laboratory findings in using such calculations, however, may lead to incorrect diagnosis and therapy as demonstrated in this case. We report on a 62-year-old patient with arthralgia, pathologic glucose metabolism, brown skin pigmentation and excessively elevated ferritin and transferrin saturation levels, which initially were interpreted as signs of the assumed underlying disease (hemo-chromatosis) based on a high initial suspicion level and further corroborated by Bayesian probability analysis yielding a probability 99.0 % for the presence of hemochromatosis. Because of this high probability and the patient's wish for treatment phlebotomy was started, but stopped after having obtained negative results of genetic testing and normal quantitative liver iron values. The diagnosis of hemochromatosis had to be revised and symptoms and laboratory findings of this patient were found to be compatible with chronic fatty liver and pathologically altered iron metabolism due to chronic alcohol intake which the patient has initially concealed. The joint pain was explained in terms of chronic degenerative bone destruction, the impaired glucose tolerance seen as the consequence of obesity and the skin pigmentation was ascribed to sun exposure due to the patient's outdoor activities as a hobby farmer not evaluated during initial presentation. The implications and importance of unbiased history taking, critical interpretation of clinical symptoms and laboratory findings in using probabilistic calculations and diagnostic decision analysis are emphasized and the different mechanisms of iron metabolism in hemochromatosis and hemosiderosis are discussed.

Biopsy, Needle↗
PubMed1957

A study of hemosiderosis with the aid of electron microscopy; with observations on the relationship between hemosiderin and ferritin.

Hemosiderin deposits in rats and in man were studied and compared by means of electron and light microscopy. Typical, isotropic, iron-positive hemosiderin granules were found to contain innumerable, closely packed, electron-dense particles, embedded in matter that was much less dense to electrons. Similar dense particles were often scattered diffusely through the cytoplasmic matrix of cells containing hemosiderin granules. In cells of proximal convoluted tubules of rats given repeated intraperitoneal injections of hemoglobin the hemosiderin granules contained dense particles with a mean diameter of 55 A, and with a size-frequency distribution that indicated uniformity. These particles corresponded in size to the iron micelles of ferritin molecules. There was less uniformity of particles in hemosiderin granules situated in liver and reticulo-endothelial cells of rats that had been given a diet containing ethionine. The dense aggregates representing hemosiderin granules were often situated inside discrete cytoplasmic organelles that were bordered by membranes, and sometimes contained "cristae"; and often the membranous borders were markedly disrupted. The term "sidersomes" is proposed for these specialized cytoplasmic structures which may be derivatives of mitochondria, and apparently play a part in the formation of hemosiderin. Ferritin was crystallized from the livers and kidneys of the hemosiderotic rats with ease, but could not be crystallized from comparable quantities of liver and kidney tissue of untreated control rats. Specimens from the liver and spleen of a patient with advanced hemosiderosis, obtained at an operation, were also studied. In liver and reticulo-endothelial cells many particles with diameters of about 60 A were scattered through the cytoplasmic matrix. By contrast, hemosiderin granules in the same cells contained particles that varied considerably in size. In representative granules, examined at high resolution, the size-frequency distribution of particle diameters displayed a periodicity consistent with the presence of small, uniform subunits. Electron micrographs of ferritin, isolated from the spleen of the same patient, provided confirmation for the inferences that the dense particles observed inside cells are iron micelles, and that ferritin is probably a component of hemosiderin.

Animals↗
PubMed1999

Chronic peripheral separation of placenta. The significance of diffuse chorioamnionic hemosiderosis.

Diffuse nonmeconium-related pigment was observed in the chorioamnion of 36 of 1,023 placentas over 4 years and evaluated by iron staining. Stains were negative in 13 cases and positive in chorionic plate and membranes (diffuse chorioamnionic hemosiderosis [DCH]) in 23 cases (3/1,000 deliveries; 25/1,000 placentas). Gestational age at delivery was lower in DCH and was inversely proportional to the magnitude of iron staining. Placentas with DCH were more likely to show circumvallation, old peripheral blood clots, increased chorionic-villous macrophages, and green discoloration. To evaluate demographic, obstetric, and perinatal factors associated with DCH, 2 gestational age-matched controls were selected for each DCH case. Multiparity, smoking, and chronic vaginal bleeding all were increased significantly with DCH, while intrauterine growth retardation and oligohydramnios were increased but did not achieve statistical significance. Gestational hypertension and advanced maternal age were significantly decreased with DCH, and cocaine abuse was uncommon (3 cases). Long-term neurologic sequelae of DCH were evaluated in a separate series of gestational age-matched very-low-birth-weight infants with and without neurologic impairment at 2 years of age. No increased risk of neurologic impairment was found in patients with DCH.

Case-Control Studies↗
PubMed1999

Idiopathic pulmonary hemosiderosis.

This article discusses an investigation of two children with idiopathic pulmonary hemosiderosis (IPH) over a long period of time that included several cycles of the disease in each patient, each cycle consisting of clinical remission, a preacute or linking phase, and acute lung bleeding. The acute phase was divided into two parts; the preacute phase takes place 5 to 10 days before the onset of the acute alveolar hemorrhage. At the beginning of the preacute phase, there was neutropenia followed by increased blood eosinophilia. The lung function test showed gas trapping. Bronchoalveolar lavage during remission showed increased numbers of neutrophils; the histologic study suggested that the neutrophils are obligatory participants in the occurrence of lung bleeding. The finding of the preacute phase and the role of the neutrophils in the acute bleeding permitted development of a new hypothesis related to the physiopathology of IPH.

Acute Disease↗
PubMed2000

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.

Cysts↗
PubMed2000

Long-term outcome of idiopathic pulmonary hemosiderosis in children.

We retrospectively analyzed the long-term outcome of idiopathic pulmonary hemosiderosis (IPH) in 15 children. IPH started at a mean age of 5 years, and the mean duration of follow-up was 17.2 years (range, 10-36 yr). Four patients developed immune disorders, 3 cases of rheumatoid polyarthritis or rheumatoid polyarthritis-like diseases and 1 case of celiac disease. Respiratory outcome showed that 3 patients had severe symptoms: 2 patients developed severe pulmonary fibrosis resulting in major chronic respiratory insufficiency, and 1 patient had severe asthma. Twelve patients (80%) had mild or no respiratory problems and were able to lead a normal life. According to chest X-ray and pulmonary function test data, 4 patients had normal chest X-ray and no evidence of restrictive syndrome, 6 patients had an interstitial pattern on chest X-ray and evidence of restrictive pattern, 1 patient had an interstitial pattern but normal lung function, and 1 patient had a normal chest X-ray but evidence of mixed obstructive and restrictive pattern. Our results show that long-term survival is possible in patients with IPH. Factors of poor prognosis seem to be the presence of antineutrophil cytoplasm antibodies (ANCA) or other autoantibodies. No other clinical or biological predictive factors for prolonged survival were found.

Adolescent↗