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Results for “HEMOSIDEROSIS”

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At least 145 records · Page 8Linked to original sources
PubMed1975

Idiopathic pulmonary hemosiderosis. Electron microscopic, immunofluorescent, and iron kinetic studies.

The clinical course of a 37-year-old white man with idiopathic pulmonary hemosiderosis is presented. This patient is unusual in that he has had repeated exacerbations and remissions over a period of seven years and remains currently in spontaneous remission with no therapy. Routine sections of the lung biopsy revealed characteristec findings. Immunofluorescence staining of the lung was negative, and electron microscopic studies showed only nonspecific findings. While in remission, 51chromium-labelled red-blood-cell survival studies and 59iron kinetic studies were performed; the results were normal.

Adult↗
PubMed2004

Maternal, neonatal, and placental features associated with diffuse chorioamniotic hemosiderosis, with special reference to neonatal morbidity and mortality.

OBJECTIVE: Our purpose was to examine the significance of diffuse chorioamniotic hemosiderosis (DCH) on neonatal morbidity and mortality. METHODS: Using data from a retrospective case-control study, we analyzed 46 singleton placentas with DCH from infants who were delivered and/or admitted to the neonatal intensive care unit of Kanagawa Children's Medical Center during 1987-2001 and 92 control placentas without DCH from infants of comparable gestational age, birth weight, and duration. RESULTS: Mean and standard deviation of gestational age and infants' birth weight at delivery from the DCH group were 27 +/- 3 weeks and 939 +/- 342 g, respectively. Macroscopically, the placentas with DCH were more likely to show old peripheral blood clots (46% in the DCH group vs 8% in control group), subchorionic hematoma (20% vs 1%), and circumvallation (13% vs 1%). Histologically, amniotic necrosis was significantly more frequent in the DCH group (63% vs 24%). Of the obstetric factors, incidence of recurrent episodes of vaginal bleeding (70% vs 11%), oligohydramnios (59% vs 8%), and chronic abruption-oligohydramnios sequence (57% vs 5%) were significantly higher in the DCH group. Of the neonatal factors, persistent pulmonary hypertension of the newborn (29% vs 8%) and dry lung and/or pulmonary hypoplasia (20% vs 4%) were more common. However, respiratory distress syndrome was rare (15% vs 45%) in the DCH group. Neonatal death including stillbirth was increased in the DCH group but was not significant (24% vs 15%). Of infants who survived beyond day 28, chronic lung disease (CLD) was more frequent in the DCH group (51% vs 22%). The association of DCH, especially accompanied by amniotic necrosis, with CLD was still evident using likelihood ratio test. CONCLUSION: DCH is closely associated with preterm delivery, pulmonary hypertension of the newborn, and dry lung syndrome and is a significant risk factor for CLD.

Abruptio Placentae↗
PubMedDate not supplied

Renal hemosiderosis due to thalassemia: a light and electron microscopy study with electron probe X-ray microanalysis.

A kidney biopsy specimen with pronounced hemosiderosis from a patient with beta-thalassemia major was studied by light and electron microscopy, including X-ray microanalysis. Ferritin was absorbed from the glomerular ultrafiltrate through the parietal epithelial cells and the tubular epithelial cells and from the blood through the endothelial cells. It was transported in siderosomes into the surrounding basal lamina, where electron-dense deposits of hemosiderin were found in the outer part of the lamina densa and the reticular lamina. Fibrosis was seen as a reaction to the iron followed by severe atrophy of affected structures.

Adolescent↗
PubMed1986

Hemophagocytic syndrome with restricted organ involvement: excessive hemosiderosis and fibrosis of the spleen.

We report the case history of a 6 1/2-month-old girl with a hemophagocytic syndrome, pancytopenia, and excessive hepatosplenomegaly. Some extraordinary histological features present in this case--restricted organ involvement, excessive hemosiderosis, and fibrosis of the spleen--further contributed to the well-known problem of distinguishing between infection-associated hemophagocytic syndrome and familial hemophagocytic lymphohistiocytosis.

Diagnosis, Differential↗
PubMed1989

Renal lesions and clinical findings in thalassemia major and other chronic anemias with hemosiderosis.

Renal lesions found in 21 autopsied patients with hemosiderosis, 18 with beta-thalassemia, two with Blackfan-Diamond anemia, and one with aplastic anemia included: cellular glomeruli with increased mesangial matrix; hemosiderin deposit in visceral and parietal glomerular epithelial cells; greater hemosiderin deposit in terminal straight portions of proximal convoluted tubules and distal convoluted tubules than in connecting segments, or collecting tubules, connective tissue ferrugination; lipofuscin in tubular epithelium and vascular smooth muscle; infrequently, intimal or medial arterial thickening, and, in one patient with thalassemia, an infarct resulting from arterial thrombus. The progression of these lesions over the course of disease, and possible effects on the various lesions of high transfusion regimen, oral pancreatin, vitamin E supplementation, or treatment with intramuscular, subcutaneous, or intravenous desferrioxamine were evaluated. The results of urine and renal function studies of 4 of the autopsied patients (3 thalassemia, 1 Blackfan-Diamond anemia), and 14 patients with thalassemia and 4 with Blackfan-Diamond anemia who were not autopsied, are presented. Rarely significant until preterminal stages, the renal functional changes reflect distal more than proximal tubule dysfunction.

Adolescent↗
PubMedDate not supplied

[A case of hereditary ceruloplasmin deficiency with hemosiderosis].

We report a 49-year-old female with hereditary ceruloplasmin deficiency with hemosiderosis. There was a family history of the same symptoms; her brother showed hypoceruloplasminemia and decrease of the serum copper content. On physical examinations, dementia, dysarthria, downbeat nystagmus, sensorineural hearing disturbance, orthostatic hypotension, retinitis pigmentosa, diffuse goiter, and cerebellar ataxia were noted. Laboratory examinations disclosed leukopenia, diabetes mellitus, hypothyroidism, decrease of copper content in the serum and urine. Serum ferritin concentration was remarkably increased. Serum ceruloplasmin could not be detected. Biopsy of the liver showed that iron content in the liver was increased. On MRI study, dentate nucleus of the cerebellum, basal ganglia, and the liver showed low intensity in both T1 and T2 weighted images. A nonsense mutation in the ceruloplasmin gene was found in this patient. Systemic iron deposition and tissue damage were considered as caused by deficiency of function of ceruloplasmin as ferroxidase. To our knowledge, the characteristic combination of the clinical signs in this patient has not been reported.

Ceruloplasmin↗
PubMedDate not supplied

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.

Adrenal Cortex Hormones↗
PubMed2001

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.

Adult↗
PubMed2001

[Skeletal muscle pathology of chronic graft versus host disease accompanied with myositis, affecting predominantly respiratory and distal muscles, and hemosiderosis].

We report the muscle pathology in a 43-year-old woman who died of chronic graft versus host disease (GVHD) complicated by myositis and systemic transfusional hemosiderosis, after an allogeneic bone marrow transplantation and a donor leukocyte transfusion for acute myelogenous leukemia. Despite cyclosporin A treatment, fatal ventilatory failure progressed while she was still ambulant. Autopsy revealed the presence of chronic GVHD mildly involving the liver, skin, pericardium, pancreas, and salivary glands, in addition to skeletal muscles. Myopathic changes with mild inflammation and prominent iron deposition were found in the tibialis anterior muscle and, to a lesser degree, in the diaphragm and the intercostal muscle. There were iron deposits in both macrophages and sarcoplasm in the tibialis anterior. The iliopsoas and pectoralis major muscles showed prominent type 2 fiber atrophy; inflammation and iron deposition were minimal in the iliopsoas, but none in the pectoralis. Although we ascribed respiratory failure largely to GVHD myositis, weakness of the lower leg appeared to be aggravated by iron deposition superimposing the underlying GVHD myositis.

Adult↗
PubMedDate not supplied

Severe iron deficiency anemia in a child with idiopathic pulmonary hemosiderosis: a case report.

We report a case of idiopathic pulmonary hemosiderosis (IPH) in a three-year-old male patient who presented with severe iron deficiency anemia. The child had been diagnosed with iron deficiency anemia nine months earlier and had received multiple blood transfusions, but the cause of his anemia had not been established. The diagnosis of IPH was made after a biopsy of the left lung showed large numbers of hemosiderin-filled macrophages in the alveoli. He did not respond to standard dose corticosteroid (CS) treatment (2 mg/kg/d). However, high-dose short-term CS treatment was successful in two episodes of acute respiratory hemorrhage in this patient. We conclude that IPH should always be considered when investigating the cause of iron deficiency anemia. A more rapid diagnosis in this case could have prevented unnecessary investigations and blood transfusions. We also suggest that high-dose short-term CS treatment should be kept in mind, especially in patients who do not respond to a standard dose.

Anemia, Iron-Deficiency↗
PubMedDate not supplied

[Pulmonary hemosiderosis: a case report].

A case of pulmonary hemosiderosis was reported at the Eliseo Noel Caamaño Pediatric Teaching Hospital in Matanzas City. It was a three-year-old patient, with clinical picture and complementary investigations. A review was made of the literature regarding the disease, which is unusual in children, especially with such a clear cause. Because of its high morbidity and frequent mortality, it will be imperative to carry out early examination and adequate therapy. Emphasis is placed on specific nursing procedures for the management of this disease.

Child, Preschool↗
PubMed2004

Anemia as the sole presenting symptom of idiopathic pulmonary hemosiderosis: report of two cases.

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease in children and has an unknown etiology. It is characterized by the triad of hemoptysis, pulmonary infiltrates on chest radiograph (CXR) and iron deficiency anemia. We report two young children, aged 3 and 4 years, were admitted due to pale-looking appearance but without hemoptysis or other respiratory symptoms. Pallor was the sole presenting feature in these 2 children with IPH and which was unusual. CXR obtained on admission led to the suspicion of pulmonary hemorrhage. The diagnosis of IPH was confirmed based on the presence of many hemosiderin-laden macrophages in bronchoalveolar lavage fluid obtained by flexible bronchoscopy. Steroid was initiated after the diagnosis of IPH was established; the both of them have been well and received regular follow-up in our outpatient department. IPH may not be diagnosed because of difficulty in diagnosis. Anemia may be the only presenting feature of IPH, which was due to occult pulmonary hemorrhage. Initial treatment with corticosteroids has been successful in our patients for a period of 6 and 8 months of follow up respectively.

Adrenal Cortex Hormones↗
PubMed1992

[Hemosiderosis in thalassemia major. Quantitative study with magnetic resonance (MR)].

Hemosiderosis, which results from transfusional therapy and from increased absorption of dietary iron, is a severe complication of thalassemia major. The complication is also very difficult to manage. An accurate determination of the total amount and of the distribution of body iron stores is essential for prognostic purposes and to evaluate the efficacy of chelation therapy. Therefore, a suitable tool for the noninvasive depiction of iron overload is to be hoped for. MR imaging has been usefully employed, of late, to this purpose. Sixteen thalassemic patients have been studied to evaluate iron depositions using a 1.5 T magnet. Signal intensities from liver, pancreas, spleen, bone marrow, and heart were considered. The signals were dramatically lower than the normal values obtained in a sex- and age-matched group; the differences were due to the iron. Iron distribution appeared inhomogeneous, with the highest overload in liver and bone marrow. Since cardiomyopathy represents the first cause of death in thalassemic patients, the relationship between cardiac function and iron overload in the heart has been thoroughly investigated. Finally, the authors conclude that MR will become a widely employed method for assessing iron overload and will contribute to improve the management of thalassemia major.

Adolescent↗
PubMedDate not supplied

[Hydroxychloroquine in pediatric idiopathic pulmonary hemosiderosis--a case report].

The authors present the case of a child with severe idiopathic pulmonary hemosiderosis who after having begun treatment with hydroxychloroquine had a significant and lasting improvement. The efficacy of this therapeutic is pointed out. They also report the occurrence of clinical exacerbation, with hemoptysis, after receiving an influenza vaccine.

Child↗
PubMedDate not supplied

[Idiopathic pulmonary hemosiderosis (2 clinical cases)].

The authors present two children with idiopathic pulmonary hemosiderosis, in whom the diagnosis was very difficult on the basis of evolution by stages, hypochromic anemia, hemoptoic sputum containing siderophages, cloudy pulmonary shadow and microscopical skin and lung examination that evidenced marked alterations of the elastic system and siderophages on pulmonary tissue preparations. All the clinical, hematologic and radiological signs were considerably improved by corticoid therapy. The necessity is stressed of such investigations in children with anemia.

Age Factors↗
PubMed1990

[Generalized immune-complex vasculitis combined with pulmonary hemosiderosis and dilated cardiomyopathy].

The paper is concerned with observations over 3 patients in whom unusual vasculitis lay at the basis of the clinicopathological manifestations. All the patients were men of the young age. The disease debut was marked by fever, weakness, dyspnea, palpitation, cough, hemoptysis, the articulation syndrome. In two cases, there was hemorrhagic rash on the leg skin. All the patients manifested liver and spleen enlargement, two patients had lymphoadenopathy. The leading clinical symptoms included dilated cardiomyopathy, complete blockade of the inferior peduncle of His bundle and reduction of myocardial contractility. Anemia belonged to iron deficient one. The clinical examples provided indicate that immunocomplex vasculitis with evident lesions of the lungs and myocardium, not going into criteria for the known diseases, is not likely to be a casuistic rarity. Those syndromes may be associated with more or less pronounced hemosiderosis of the lungs (and, probably, of the lymph nodes, spleen and liver), with transitory or steady derangements of myocardial conduction, which attests to diffuse lesions of the myocardium possibly with both immune complexes and hemosiderin. The pathology requires further studies.

Adult↗
PubMedDate not supplied

[Idiopathic pulmonary hemosiderosis in the adult: a case report].

The case of idiopathic pulmonary hemosiderosis in a 20 year old male is presented. Iron deficiency anemia, recurrent hemoptysis and micronodular infiltrate in lower lung areas were the most important signs observed. The transbronchial biopsy showed alveolar and interstitial hemorrhage and hemosiderin in the pulmonary macrophages. Treatment was started with prednisone, but the addition of immunosuppressive drugs (cyclophosphamide) was needed, followed by a clinical remission.

Adult↗