Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Homozygous beta-thalassemic mice show many of the features seen in human beta-thalassemia, such as decreased hemoglobin, hematocrit, and red blood cell count as well as increased reticulocyte count. They also exhibit splenomegaly and a decrease in osmotic fragility of red cells. beta-thalassemic mice were examined for spontaneous iron overload at ages ranging from 20 to 595 days. Accumulation of iron was shown to occur in the spleen, liver, and kidneys but not in the heart. Sections of spleen, liver, kidney, and heart were stained for iron and subjectively scored. Image analysis microscopy was used to examine sections of spleen and liver. Nonheme iron in the four tissues was quantitated using the bathophenanthroline sulfonate colorimetric assay. An increase in tissue iron occurred primarily in the spleen, even before weaning, despite the low iron content of milk. Iron accumulation in the absence of blood transfusion is of interest because iron overload is the major cause of death in human beta-thalassemia.
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PMID:Tissue iron deposition in untransfused beta-thalassemic mice. 271 22

This study was designed to evaluate superparamagnetic iron oxide (AMI-25) as a contrast agent for MR to distinguish normal spleens from those diffusely infiltrated by lymphoma. As diffuse splenic involvement lacks visible tumor-tissue boundaries, signal-intensity measurements of spleens were used as a diagnostic criterion in 33 patients (lymphoma, n = 8; benign splenomegaly, n = 5; normal subjects, n = 20). Unenhanced MR images were insensitive (four of eight patients) and nonspecific (20 of 25 patients) in the diagnosis of lymphoma. After injection of superparamagnetic iron oxide (40 mumol Fe/kg), lymphomatous spleens showed a significantly higher signal intensity (p less than .05) than did normal spleens or spleens enlarged by benign disease (hepatic cirrhosis, n = 4; spherocytosis, n = 1). Changes in splenic MR signal intensity unambiguously identified eight of eight lymphomatous spleens and 25 of 25 normal or enlarged spleens that did not contain lymphoma. Phagocytosis of superparamagnetic iron oxide in lymphomatous spleens is reduced because of diffuse displacement of splenic macrophages by lymphoma cells and/or by immunologic suppression of macrophage activity. Our results suggest that superparamagnetic iron oxide (AMI-25) can improve the accuracy of MR imaging in the diagnosis of splenic lymphoma. With further development, this noninvasive technique may reduce the need for diagnostic splenectomy in lymphoma patients.
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PMID:The diagnosis of splenic lymphoma by MR imaging: value of superparamagnetic iron oxide. 278 73

We have studied the mechanism of a transfusion-dependent anaemia found in a two-year-old Maltese girl with visceral leishmaniasis that was resistant to multiple courses of antimonial therapy. Major factors contributing to the anaemia were haemolysis occurring in both the massively enlarged spleen and liver and haemodilution resulting from expansion of the plasma volume. There was no evidence of significant ineffective erythropoiesis, but a reduced plasma iron in the presence of greatly increased iron stores suggested that reticuloendothelial hyperplasia was accompanied by abnormal iron retention by macrophages typical of the 'anaemia of chronic disorders'. This may limit the erythropoietic response to anaemia in chronic visceral leishmaniasis.
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PMID:Mechanism of anaemia in resistant visceral leishmaniasis. 302 61

A 39-year-old female with type II congenital dyserythropoiesis presented with iron overload. The clinical and haematologic features were an anaemia of variable severity, splenomegaly, numerous bizarre and binucleate normoblasts in the bone marrow, with prominent submembranous cisternae in the late forms, a positive Ham's acid lysis test and aberrant expression of the I and i red cell antigens. The iron overload resulted from gross ineffective erythropoiesis, with accelerated plasma iron turnover and increased absorption aggravated by inappropriate replacement therapy for past episodes of anaemia.
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PMID:Congenital dyserythropoietic anaemia (type II) presenting with haemosiderosis. 311 5

A retrospective study was designed to analyse the mode of presentation, clinical signs, haematological, biochemical and histological features in 46 Indian patients admitted with cirrhosis to R. K. Khan and King Edward VIII Hospitals, Durban, between 1977-1981. The commonest presenting feature was swelling of the body followed by pain in the right upper quadrant, most patients had hepatomegaly, jaundice and ascites, and splenomegaly was detected in one-third of cases. Biochemical investigations indicated that most patients had a high globulin and low albumin concentration. Liver function tests revealed raised bilirubin and gamma-glutamyltransferase values in most cases. On histological examination, micronodular cirrhosis predominated (95%) with a high incidence of fat and iron deposition. Changes consistent with alcoholic hepatitis were superimposed in one-third of cases while immunological and viral markers were absent. This study suggests that alcohol is the predominant cause of cirrhosis in Natal Indians.
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PMID:Patterns of cirrhosis in Natal Indians. 320 19

Inspite of its non-inclusion in the prescribed list of food colors, orange II is extensively employed to color a variety of foodstuffs. Oral LD50 value of orange II in both male and female rats was calculated to be more than 10.56 g/kg body weight. In short-term studies, animals were exposed to diets containing 0.0 (control), 0.1, 0.5 or 3.0% (w/w) of orange II, daily for 90 days. Hematological examination revealed a slight decrease in erythrocyte count and hemoglobin content, whereas leucocyte count, PCV, ESR, MCV, MCH and MCHC showed normal values. There was no change in the activities of LDH, GOT, GPT, alkaline/acid phosphatases and bioconstituents, lactic acid, cholesterol and protein in serum as well as in liver, indicating normal functioning of the liver. Histopathological examination of various body organs such as liver, heart, lung, kidney, testes, adrenal, stomach, large and small intestine presented normal appearance. Animals receiving 3.0% orange II showed marked splenomegaly and deposition of Perl's positive iron pigments. Testicular LDH, hyaluronidase and lactic acid did not reveal any deviation from controls, suggesting normal spermatogenic process. No changes in testicular cholesterol, fructose content of coagulating glands and dorso-lateral prostate, and activities of alkaline phosphatase in seminal vesicle and acid phosphatase in ventral prostate support normal androgenic status.
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PMID:Acute and short-term toxicity studies on orange II. 362 8

Three short-term studies of 7, 14, and 21 days, respectively, were made to investigate the nature of the anemia induced in rats by 3,5-dinitro-4-chloro-alpha,alpha,alpha-trifluorotoluene (DNCTT). This compound is an intermediate in the synthesis of dinitroaniline herbicides and was detected as a contaminant of a water-bearing stratum in northern Italy. DNCTT was mixed in a powdered rodent diet at a level of 2000 ppm and administered to Wistar-derived rats. DNCTT was shown to produce a hemolytic anemia of rapid onset; packed cell volume and hemoglobin concentration were decreased at all three treatment periods. Methemoglobin and reticulocyte count were increased in all the treated groups. The relative organ weights of the spleen and the liver were increased compared to those of the control groups. Spleen enlargement was also evident at the macroscopic examination, whereas the liver appearance was normal. Pearl's Prussian blue staining performed on the spleen and liver was highly positive in the spleen of treated rats, but no iron deposition was detected in the liver of treated rats.
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PMID:Hematotoxic effects of 3,5-dinitro-4-chloro-alpha,alpha,alpha-trifluorotoluene, a water contaminant. 379 63

Various haematological parameters were followed in a group of 47 Kenyan patients with visceral leishmaniasis during treatment and follow up. The WBC and platelet numbers were normal by the time of cure, the Hb level took longer to become normal. Red cells were microcytic and hypochromic. MCV and MCH increased during follow up but microcytosis persisted up to a year after cure. Low serum iron and transferrin concentration, low total iron binding capacity and normal to high serum ferritin levels were found in 10 patients and are consistent with 'anaemia of chronic inflammation'. Bone marrows of 15 patients before treatment were normo- to hypercellular with increased erythropoietic activity. Low haemosiderin content of the bone marrow was consistent with iron deficiency, but normalization of Hb without iron suppletion would argue against a major role of iron deficiency. Coagulation studies did not indicate diffuse intravascular coagulation. Splenomegaly seems the most important factor in the causation of the pancytopenia. Further studies of contributing factors and of the cause and mechanism of 'hypersplenism' are needed.
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PMID:Haematological investigations in visceral leishmaniasis. 381 Aug 41

Mice homozygous for a spontaneous mutation, in which the beta-major globin gene is deleted, have clinical symptoms of beta-thalassemia. These mice have a hypocellular, hypochromic, microcytic anemia that becomes more severe with increasing age. The defective red cell morphology, decreased osmotic fragility of erythrocytes and shortened red cell life span found in beta-thalassemic mice are similar to those observed in human beta-thalassemia. Synthesis of beta-globin is depressed but not as much as might be expected because the expression of the beta-minor globin gene is enhanced to encode two to three times more globin than in normal mice. Splenomegaly, an enlarged pool of stem cells for erythropoiesis, and iron overloading occur in older mice. The fact that these mice remain moderately healthy makes them a very suitable animal model in which to develop and test alternative techniques of gene therapy that could be successfully applied to the treatment of human thalassemia. Homozygous beta-thalassemic mice have large deposits of iron in their tissues, which might make these mice also useful for in vivo tests of the effectiveness and possible long-term side effects of newly developed iron chelators.
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PMID:Hematology of a murine beta-thalassemia: a longitudinal study. 386 Jan 41

The time course of metabolic and physiological adjustment to moderate iron deficiency anemia (MIDA, 8 g Hb/dl) and to severe iron deficiency anemia (SIDA, 4 g Hb/dl) was studied in adult, male Sprague-Dawley rats at 3, 7, 14, 30, 60, 90, 150, and 360 days, respectively. Our previous studies using the same rats used in the present study indicated that bone marrow iron was absent and plasma iron was significantly lower (p less than 0.001) in MIDA and SIDA relative to control. The following results with MIDA and SIDA rats are all expressed relative to control values. Red cell 2,3-diphosphoglycerate ranged from 45 to 146% greater in MIDA over the 360-day period and was 130% greater in SIDA at 30 days. Exhaustive run time consistently averaged 64 +/- 3% (SEM) less in MIDA over the time course and was further lowered to 18% in SIDA at 30 days. Heart rates of MIDA were elevated (p less than 0.05) at 180 days but lower (p less than 0.001) at 360 days in response to exercise. Resting heart rates of MIDA were the same at 180 and 360 days. Heart rates of SIDA were elevated (p less than 0.05) at rest and during exercise at 30 days. Organ weight/body weight changes indicated cardiomegaly in MIDA from 90 to 150 days which reverted to normal at 360 days; splenomegaly in MIDA from 90 to 360 days; and kidney atrophy in MIDA at 60 and 90 days which reverted to normal thereafter; in SIDA cardiomegaly and splenomegaly were present at 30 days. These results indicate that the onset and magnitude of physiological and metabolic adjustments occur in proportion to the severity of the anemia, and despite compensatory adjustments in parameters related to work performance, a new stable, lowered level of work tolerance is reached.
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PMID:Changes in work tolerance associated with metabolic and physiological adjustment to moderate and severe iron deficiency anemia. 629 71


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