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At least 1,027 records · Page 57Linked to original sources
PubMed1986

Hepatobiliary pathology in hemodialysis patients: an autopsy study of 78 cases.

Necropsy findings of hepatobiliary system from 78 patients with end-stage renal disease maintained on hemodialysis are reported. Ninety percent of the patients exhibited some abnormalities. Multiple abnormalities often coexisted in each patient. Hepatomegaly was found in 50% of the patients and could be attributed to a discernible cause in all but two of the affected patients who had isolated hepatomegaly. Hepatic congestion was also prevalent and was complicated by fibrosis, cardiac cirrhosis, and centrilobular necrosis and hemorrhage in some patients. This was associated with chronic fluid overload, hypertension, and/or cardiovascular disease in the affected patients indicating the importance of adequate control of these factors. Mild periportal hepatic fibrosis, fatty metamorphosis, triaditis, hemosiderosis, and cystic changes were also seen with some frequency--the latter were associated with polycystic kidney disease and were complicated by massive intracystic hemorrhage and abscess formation, each in one patient. Chronic active hepatitis was found in three patients and was associated with chronic HBs antigenemia in one patient and presumed non-A, non-B infection in two. Nearly 22% of the patients showed either cholelithiasis at autopsy or before cholecystectomy due to complications. Significant negative findings included lack of acute viral hepatitis, silicone hepatosis, and recently described focal anoxic lesions associated with erythrocyte sludging. In conclusion, the present study has demonstrated the spectrum of hepatobiliary pathology in a large group of patients with end-stage renal disease maintained on hemodialysis.

Adult↗
PubMed1979

Pulmonary alveolar microlithiasis. A case report with a discussion of differential diagnosis.

The rare disease of pulmonary alveolar microlithiasis is characterized by innumerable microscopic stones, mainly of tribasic calcium phosphate, within the pulmonary alveoli. In a 13-year-old boy an earlier radiological examination showed diffuse lung opacity. The main differential diagnoses taken into consideration were sarcoidosis, idiopathic hemosiderosis, diffuse interstitial fibrosis and alveolar proteinosis. The correct diagnosis was made after a lung biopsy which showed intra-alveolar microliths. The radiological findings are in agreement with the reports in the literature. The specific X-ray features of pulmonary alveolar microlithiasis are calcified opacities, nodules, hilifugal trabeculations and pleural striae. These radiological signs are sufficiently diagnostic to restrict lung biopsy to particularly difficult cases. The disease is resistant to all therapeutic measures.

Adolescent↗
PubMed1979

[Results of lung biopsy in interstitial pneumopathies in children. A report on 100 cases (author's transl)].

Needle biopsy was performed in 100 children with diffuse interstitial pneumopathy. Severe histological lesions were noted in the biopsy sections in 4 out 5 cases. Those most frequently observed (57 cases) were non-specific lesions of diffuse interstitial pneumopathy (DIP) or idiopathic fibrosis, the course of which was followed, in some cases, by repeating the biopsy. Specific lesions, especially idiopathic pulmonary hemosiderosis (8 cases), were noted in 22 children, either associated with interstitial fibrosis or apparently better tolerated. The other 16 cases included the lipoidoses (Niemann-Pick, histiocytosis X), alveolar microlithiasis, and some specific inflammatory diseases (tuberculosis, syphilis) or those related to inhalation of a foreign body.

Adolescent↗
PubMedDate not supplied

Staining of minerals and solubility of iron in tissues.

Iron deposits in ethionine induced aortic siderosis of rats, in splenic deposits in sickle cell anemia and siderocalcific vessels in cerebral arteriosclerosis were completely removed by exposure to 20 percent hydrochloric acid for 30 min. This contrasted with idiopathic hemochromatosis and idiopathic pulmonary hemosiderosis in which the iron containing organs had to be exposed to 40 percent hydrochloric acid for two hours. The more soluble iron appeared colorless in unstained tissues, purplish blue with hematoxylin and eosin, turquoise blue with Perls' stain, violet blue with gallocyanin and dark-drown with sodium rhodizonate. The less soluble iron was golden yellow in unstained tissues. It appeared golden yellow with hematoxylin and eosin and sodium rhodizonate, but it stained greenish blue with Perls' method and dark brown with gallocyanin. Lead and copper were capable of deposition in some tissues in vitro in the presence of iron and/or calcium but not when these minerals were removed. This phenomenon may be of importance in certain pathological conditions, e.g. hemochromatosis, where on preexisting tissue-iron-complexes there is a secondary deposition of copper.

Anemia, Sickle Cell↗
PubMed1984

Alveolar hemorrhage syndromes: diffuse microvascular lung hemorrhage in immune and idiopathic disorders.

We have reviewed the alveolar hemorrhage (AH) syndromes, defined as immune or idiopathic disorders associated with diffuse microvascular hemorrhage into the acinar portion of the lung. The disorders that are most often associated with AH include antibasement membrane antibodies (ABMA) disease, idiopathic pulmonary hemosiderosis, systemic lupus erythematosus, systemic vasculitides, and idiopathic rapidly progressive glomerulonephritis. An approach to the recognition, diagnosis, and treatment of the AH syndromes has been outlined and several illustrative case studies have been presented. Recognition of AH is not usually difficult, but does require a high index of suspicion, since many disease processes may give rise to hemoptysis with infiltrates on chest roentgenogram. Recognition of AH is aided by careful clinical and laboratory assessment for evidence of extrapulmonary disease; simple hematologic studies such as sequential hemoglobins and iron studies; and measurement of carbon monoxide uptake by the lungs. Early recognition of AH may decrease the likelihood of respiratory failure and end-stage renal disease. The specific etiology of AH is usually determined by clinical examination, serologic assay for ABMA, and percutaneous renal biopsy by immunofluorescence. Open-lung biopsy is required in a minority of cases. High-dose pulse methylprednisolone appears to effectively control AH of diverse etiology. Combined plasma exchange and immunosuppression controls AH in ABMA disease and is the treatment of choice in this disorder. Cyclophosphamide is used for Wegener's granulomatosis, and sometimes in systemic necrotizing vasculitis, in an attempt to prevent irreversible damage to the kidneys.

Adult↗
PubMed1980

Pulmonary manifestations of juvenile rheumatoid arthritis. A report of eight cases and review.

Pleuropulmonary disease was seen in 4 per cent of patients with juvenile rheumatoid arthritis. Roentgenographic abnormalities seen in association with juvenile rheumatoid arthritis include: transient pneumonitis, interstitial reticular and nodular infiltrates, pleural and pericardial effusions, and patchy pleural infiltrates. Pathologic abnormalities seen in association with juvenile rheumatoid arthritis include pulmonary hemosiderosis, lymphoid follicular bronchiolitis, and lymphocytic interstitial pneumonitis. Patients with juvenile rheumatoid arthritis and pleural disease recover fully. In children with parenchymal disease, residual abnormalities include roentgenographic evidence of interstitial fibrosis and minimal abnormalities of pulmonary function.

Arthritis, Juvenile↗
PubMed1982

[Anergic miliary tuberculosis, with principally splenic localization simulating hemolymphopathy. Report of 4 cases].

Four cases of disseminated tuberculosis with prevailing spleen involvement and lack of tuberculin reactivity are described. The atypical clinical picture justified, at the beginning of the disease, the suspect of a lymphoreticular disorder (malignant lymphoma in 3 cases) or of a pulmonary hemosiderosis (in 1 case). The splenectomy and the following anti-tubercular chemotherapy were fully successful in all 4 patients and the skin reactivity was restored. The Authors discuss the pathogenesis of the observed features and the differential diagnosis of the cases of tuberculosis with only extrapulmonary involvement. These cases represent today about 1/6 of the patients with postprimary tuberculosis.

Adolescent↗
PubMed1979

The selection and evaluation of new chelating agents for the treatment of iron overload.

A large-scale systematic evaluation of potential iron chelators for the treatment of hemosiderosis was conducted. The compounds were identified and evaluated using a hypertransfused mouse screen in which deferrioxamine B was a standard. This screen was designed to measure iron depletion in the tissues as well as iron excretion. Groups of 10 previously hypertransfused BDF1 male mice received a single daily i.p. injection of either vehicle, standard, or test compound for 7 days. Iron in daily urine pools and individual spleen and liver homogenates was determined by atomic absorption. More than 70 chelators were evaluated, including natural and synthetic hydroxamic acids, phenols, catechols and tropolones known to have a high affinity for iron (III) in vitro. Ethylenediamine-N,N'-bis(2-hydroxyphenylacetic acid) was shown to be considerably more effective than deferrioxamine B (i.p.) and, in addition, was orally active. Factors determining the efficacy of this and other chelating agents are discussed.

Animals↗
PubMed1993

[Nuclear spin tomographic findings in compensated chronic hemolysis. A case report of a hereditary spherocytosis].

A case report of a 58-year-old patient with hereditary spherocytosis and large paravertebral masses in the thorax and abdomen is presented MRI detects the origin of the masses with typical signal intensities of the masses and the bone marrow in T1 and T2 weighted sequences as extramedullary hematopoiesis. In this special case there is bleeding into these masses and, as often, liver hemosiderosis and splenomegaly.

Chronic Disease↗
PubMed1996

[Vitamins and metals: possible hazards for humans].

Administration of vitamins or metals may cause severe side effects. Retinoids (derivatives of vitamin A) used for the treatment of various skin disorders are teratogenic, hepatotoxic and may induce a substantial increase in serum lipids. A case report demonstrates that vitamin D supplementation in a patient under total parenteral nutrition can cause hypercalcemia. The isolated administration of vitamin B1, without concomitant vitamin B6 and nicotinamide may precipitate potentially life-threatening pellagra encephalopathy. Repeat blood transfusions may produce clinically overt organ hemosiderosis, e.g. cirrhosis of the liver, diabetes mellitus or myocardiopathy. The literature contains reports on a few cases of sarcoma associated with orthopedic metal implants. The controversial issue of the potential dangers of dental amalgams is briefly mentioned.

Adult↗
PubMed2001

Etiology and management of pediatric hemoptysis.

OBJECTIVE: To review the diagnostic and treatment strategies of hemoptysis in children. DESIGN: Retrospective analysis of patients evaluated between January 1, 1995, and August 31, 1999. SETTING: Tertiary pediatric referral center. PATIENTS: Nineteen consecutive children presenting with hemoptysis to the otolaryngology service. RESULTS: Chest radiography and bronchoscopy established the correct etiology in 15 patients. Infection and tracheostomy-related complications were the most common underlying problems. Other causes included congenital heart disease, pulmonary hemosiderosis, inflammatory bronchial mass, cystic fibrosis, factitious hemoptysis, and esophagitis. Appropriate management, ranging from antibiotics to emergency embolization, resulted in control of hemoptysis in all patients. CONCLUSIONS: Hemoptysis is a rare but potentially life-threatening symptom of underlying respiratory tract abnormality in children. An efficient systematic evaluation is imperative to identifying the underlying etiology; aggressive management is important because of the potential severity of the problem. The otolaryngologist plays a pivotal role in the diagnosis and management, by flexible endoscopy of the nose, nasopharynx, and larynx, and through the use of rigid bronchoscopy, especially in cases of massive hemoptysis.

Adolescent↗
PubMed1979

Endocrine abnormalities in thalassemia major.

Endocrine function evaluations in 16 patients with beta-thalassemia indicate that hypogonadotropic hypogonadism, hypoparathyroidism, and reduced adrenocorticotropic hormone reserve occur frequently, whereas reduced growth hormone and thyroid reserve are less common manifestations. Hemosiderosis-induced damage of the endocrine glands seems to be the main cause for endocrine dysfunction in the patients studied.

Adolescent↗
PubMed1996

Familial sideroblastic anemia with emergence of monosomy 5 and myelodysplastic syndrome.

The case history of two sisters with pyridoxine-refractory familial sideroblastic anemia (FSA) is presented in which one developed a myelodysplastic syndrome (MDS) with monosomy for chromosome 5. Bone marrow examination of both patients at diagnosis showed erythroid hyperplasia with more than 50% ring sideroblasts. Karyotypic analysis initially showed a normal 46, XX karyotype in both of the children. Therapeutic trials with pyridoxine, prednisone, and erythropoietin were unsuccessful. The first patient required regular transfusions and developed a significant hemosiderosis. At the age of 9 years, 7.5 years after the diagnosis of FSA, refractory anemia with excess of blasts (RAEB) was diagnosed. Bone marrow cytogenetic analysis revealed a clone with monosomy for chromosome 5. Her sister's illness was detected at the age of 12 years. She has a more benign course of disease, remains largely transfusion independent and until now shows no signs of myelodysplasia. To our knowledge this is the first observation of a transition of FSA to MDS accompanied by the appearance of a chromosomal abnormality. FSA might be another type of bone marrow failure syndrome, therefore close follow-up of these patients may be necessary.

Anemia, Refractory, with Excess of Blasts↗
PubMed1998

Topographical localization of iron in brains of the aged fat-tailed dwarf lemur (Cheirogaleus medius) and gray lesser mouse lemur (Microcebus murinus).

Iron deposits in the human brain are characteristic of normal aging but have also been implicated in various neurodegenerative diseases. Among nonhuman primates, strepsirhines are of particular interest because hemosiderosis has been consistently observed in captive aged animals. In particular, the cheirogaleids, because of their small size, rapid maturity, fecundity, and relatively short life expectancy, are a useful model system for the study of normal and pathological cerebral aging. This study was therefore undertaken to explore iron localization in the brain of aged cheirogaleids (mouse and dwarf lemurs) with histochemistry and magnetic resonance microscopy. Results obtained with both techniques were comparable. There was no difference between old animals in the two species. The young animals (3 years old) showed no iron deposits. In the old animals (8-15 years old), iron pigments were mainly localized in the globus pallidus, the substantia nigra, the neocortical and cerebellar white matter, and anterior forebrain structures, including the nucleus basalis of Meynert. This distribution agrees with previous findings in monkeys and humans. In addition, we observed iron in the thalamus of these aged non-human primates. Microscopic NMR images clearly reveal many features seen with the histochemical procedure, and magnetic resonance microscopy is a powerful method for visualizing age-related changes in brain iron.

Aging↗
PubMed1997

Evaluation of 13-week inhalation toxicity study on methyl t-butyl ether (MTBE) in Fischer 344 rats.

Methyl-t-butyl ether (MTBE) is widely used as an octane enhancing agent in gasoline. A 13-week inhalation study was conducted in Fischer 344 rats to provide information on potential target organs and toxicity of MTBE, and to ascertain a no-observed-adverse-effect level (NOAEL) for MTBE. Male and female Fischer 344 rats were exposed to target doses of MTBE vapor of 0, 800, 4000 and 8000 ppm for 6 h a day, 5 days per week for 13 weeks: MTBE produced no mortalities. At 8000 ppm, males and females showed a decrease in body weights compared to controls. The only notable effect on clinical observation was ataxia at 8000 ppm, which was apparent during the first 4 weeks of treatment. Mild hematological and clinical chemistry changes were observed in the 8000 ppm group. At 8000 ppm, animals showed increased serum levels of corticosteroids, which suggest some stress-like effect. At necropsy, there were no treatment-related gross lesions. Absolute and relative organ weights (liver, adrenals and kidneys) were increased in both sexes at 4000 and 8000 ppm, but there were no microscopic lesions in these tissues with the exception of the kidney. Microscopic examination of other tissues revealed no effects with the exception that at 8000 ppm, male rats showed: mild increased size of hyaline droplets within the kidney, mild increase in hemosiderosis in the spleen and higher incidence of hyperplasia in the lymph nodes. The highest NOAEL was judged at 800 ppm.

Administration, Inhalation↗
PubMed1976

Pulmonary lymphangiomyomatosis: three new cases studied with electron microscopy.

Three cases of pulmonary lymphangiomyomatosis are described, with emphasis on the ultrastructural changes. The clinicopathologic features corresponded to those previously described. Each patient was a female in the reproductive years; breathlessness and recurrent pneumothoraces were the predominant clinical features. Histologically, the lungs showed a focal interstitial infiltrate of short, spindle-shaped mononuclear cells compatible with primitive smooth muscle, which was associated with irregular emphysema and hemosiderosis. Electron microscopy confirmed the smooth muscle nature of the pulmonary infiltrate and showed the presence of cells intermediate between smooth muscle and fibroblasts. Abnormalities were also noted in the pulmonary connective tissue that are possibly related to the fragility of the lung in this condition.

Adult↗
PubMed1979

Non-Hodgkin's lymphoma: a study of the evolution of the disease based upon 92 autopsied cases.

The evolution of the disease process in 92 patients with non-Hodgkin's lymphoma has been analyzed by comparing the initial histopathologic material with that obtained at autopsy. The distribution of lymphoma did not differ substantially among the subtypes, but the gross appearance of the lesions was different among certain subtypes, particularly between the "small cell" and "large cell" types. The diffuse lymphomas showed a remarkable constancy of subtype in the same patient, whereas the nodular lymphomas showed a high frequency of change to a diffuse pattern and/or a larger cell type. Diffuse histiocytic lymphomas with plasmacytoid features showed a striking propensity for involvement of the gastrointestinal tract. The occurrence and significance of bizarre pleomorphic giant cells, epithelioid histiocytes, a "starry-sky" pattern, erythrophagocytosis, hemosiderosis and extramedullary hematopoiesis are reported. All patients received anti-lymphomatous chemotherapy and/or radiotherapy. A second malignancy was observed in 8 patients. Thirteen patients showed no residual lymphoma at autopsy. The cause of death was considered to be related to lymphoma in all 92 patients; 67 had infections identified.

Adolescent↗