Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One hundred and eighty-two patients undergoing splenectomy in infancy and childhood were followed for periods of 2 to 15 years. Serious infections occurred in 11 patients (6%) with death in 6 (3.3%). In 10 patients the infection was sepsis, and in all but one patient the infection occurred within 2 years of splenectomy. Among children over 2 years of age the risk of infection was still appreciable except when the spleen was removed incidentally or for traumatic rupture. Splenectomy for thalassemia and portal hypertension resulted in an increased risk of serious infections when compared with removal of the spleen for hereditary spherocytosis, idiopathic thrombocytopenic purpura, trauma, or for technical reasons in the course of another operation. Post-splenectomy infections tended to follow a characteristic pattern. The infecting organism was predominantly pneumococcus, the course was fulminating and the mortality high.
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PMID:The morbidity and mortality of splenectomy in childhood. 55 12

Sera from 530 children suffering from various diseases and from 64 controls were tested for smooth muscle autoantibodies (SMA) by indirect immunofluorescence. A high incidence of SMA (51-86%) was found in patients with viral and bacterial infections (viral hepatitis, infectious mononucleosis, measles, mumps, chickenpox, typhoid fever, and brucellosis), independently of liver invovlvement, and in patients with acute haemolytic anaemia due to G-6-PD deficiency (48%). By contrast, the incidence of SMA from patients with beta-thalassaemia major and idiopathic thrombocytopenic purpura was no higher than in the controls. The discrepancy in incidence in haemolytic anaemias due to different causes may reflect the effect of endogenous and extrinsic agents. In the viral infections, SMA were mainly of the IgM class and gave an 'SMA-V' staining pattern. In bacterial infections (typhoid fever and brucellosis), SMA were either IgG only or IgM and IgG, and the staining pattern was also mainly 'SMA-V'. In infections which affect or may affect the liver (viral hepatitis, infectious mononucleosis, typhoid fever, and brucellosis), SMA was present at high titres (1:80-1:320), whereas in infections not affecting the liver (measles, mumps, and chickenpox) the titres were lower (less than or equal to 1:80). In most patients SMA occurred transiently and without apparent pathogenetic significance. The antigen against which infection-induced SMA is directed is not actin; its nature has yet to be identified.
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PMID:Mechanisms of smooth muscle antibody production: a clinical study in children with infections, haemolytic syndromes, and idiopathic thrombocytopenic purpura. 57 62

Splenic cells from 10 homozygous beta-thalassemic patients were stained using cytochemical reactions: non specific esterase and acid phosphatase. Spleens from nonthalassemic subjects: a normal case who underwent gastric surgery and 5 idiopathic thrombocytopenic purpura, were also studied to serve as the control. In thalassemic spleens, no positive dot cell was shown in periarteriolar lymphocyte sheaths (PALS) when they were stained with both nonspecific esterase and acid phosphatase. In contrast, dot positive reaction was demonstrated in 92 per cent of cells from a normal spleen. These cells were presumably T lymphocytes. There were two possibilities to explain our study 1) the absence of T lymphocytes in the PALS of white pulps in homozygous thalassemia may have an impact on the immune system related to infection complication in thalassemia 2) T lymphocytes in the thalassemic spleen may be present but they do not give a positive ANAE dot reaction.
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PMID:Cytochemical reactions of homozygous beta-thalassemic spleens. 140 71

Spleens from 92 patients who underwent splenectomy for various indications were examined. These indications included hematologic disease in 38 patients and nonhematologic problems in 54. Of all the group foam cells were seen in hematoxylin and eosin (HE)-stained sections of spleens from 12 patients. This number increased to 21 (23%) when the diastase periodic acid-Schiff stain was used as a screening test. The cells in these 9 additional cases were too scanty to be observed on HE-stained sections alone. All the 21 spleens with positive foam cells were from patients with hematologic disease, specifically beta-thalassemia major, hemoglobin S/beta-thalassemia, hemoglobin AS, and idiopathic thrombocytopenic purpura. None of the spleens from the 54 nonhematologic patients showed foam cells. Factors that determine the probability of presence of foam cells were calculated. Also, certain differences in the staining reactions of foam cells were observed. The results of both, and the relation of these foam cells to the 'syndrome of the sea-blue histiocyte' are presented and discussed.
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PMID:Splenic foam cells: a clinico-pathologic analysis of 92 splenectomized patients. 168 92

The weights of the spleens of series of patients with various disorders of children dating from birth or early infancy and causing splenomegaly, with or without cirrhosis of the liver, were analyzed. The linear regression equation for spleen weight versus age in months for each disease was derived, and the rate constants from these equations were adjusted for the age range of the patients in each group. The original data of Coppoletta and Wolbach were used for normal values. The rates of splenic growth of appropriate entities for which the regression equation could be computed fell into three groups, with adjusted rate constants (growth of spleen in grams per month) of 6.53-6.95 (biliary atresia, thalassemia, and cirrhosis following neonatal hepatitis), 2.30-2.62 (cirrhosis of alpha-1-antitrypsin deficiency, infantile polycystic disease, and spherocytosis), and 1.06-1.11 (cystic fibrosis and idiopathic thrombocytopenic purpura). These classes of splenic growth rates are approximately 10, 3.7, and 1.6 times the normal growth rate (0.67 g/mo). Rate constants could not be computed for the categories cirrhosis following viral hepatitis and hemolytic anemia other than spherocytosis and sickle cell anemia, and the numbers of patients with splenic vein obstruction, cirrhosis with the cholestatic syndrome of parenteral alimentation, hypoplastic anemia with hemosiderosis, tyrosinemia, Byler's disease, congenital hepatic fibrosis, and Wilson's disease were too few for analysis. The significance of the finding of classes or "quantum groups" of splenic growth rates in disorders of children, dating from birth or early infancy and causing splenomegaly, is uncertain. Comparable data on adequate series of patients with other appropriate disorders will be necessary.
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PMID:Splenic growth rates in cirrhotic and other splenomegalic diseases of childhood. 384 62

By light and electron microscopy, we observed foamy cells in the spleens from a patient with hemolytic anemia due to red cell adenosine deaminase (ADA) overproduction, a patient with rheumatoid arthritis (RA) treated with gold, and patients with idiopathic thrombocytopenic purpura (ITP). The foamy cells associated with red cell ADA overproduction were essentially similar to Gaucher-like cells described in patients with thalassemia, and it was suggested that the accelerated destruction of red cells was one of the factors responsible for the development of foamy cells. Foamy cells in ITP and RA were closely associated with an increased destruction of platelets in the spleen. Morphologic transitions between phagocytosed platelets and myelin-like materials were traced in these disorders. In RA, however, foamy cells were heterogeneous from an ultrastructural standpoint, with different cytoplasmic inclusions. In addition to myelin-like materials, dense bodies, vacuoles with flocculent materials, and gold were noted in most of foamy cells. As gold compounds are known to inhibit lysosomal enzymes, we surmise that an acquired disturbance in lysosomal digestion is partially responsible for the accumulation of intermediate metabolites. In the pathogenesis of foamy cells associated with blood cell dyscrasia, the accelerated destruction of blood cells and/or acquired disorders in catabolic pathways within the macrophages are suggested to be the underlying mechanism of an intralysosomal accumulation of incompletely degraded cellular debris.
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PMID:Three kinds of foamy cells in the spleen: comparative histochemical and ultrastructural studies. 404 43

The influence of splenectomy on erythroid burst colony formation by peripheral blood mononuclear cells from 10 patients (four with hereditary spherocytosis, two with beta-thalassaemia major, two with Hodgkin's disease and two with idiopathic thrombocytopenic purpura) was studied. In every instance splenectomy was followed by a lowering of blood BFU-E. The post-splenectomy levels ranged from 0 to 30% of the preoperative levels. Mononuclear cells from the spleens of eight patients were cultured and found to contain numerous BFU-E. The total quantity of BFU-E in the whole blood and in the spleen of the patients was generally of the same order of magnitude. The number of splenic BFU-E did not correlate with spleen size. Splenic BFU-E differed from peripheral blood BFU-E in that they were more sensitive to erythropoietin (Ep) and in that they failed to respond to burst promoting activity (BPA) produced by preincubating the spleen mononuclear cells with phytohaemagglutinin M (PHA). In contrast, media conditioned by PHA-treated spleen cells contained BPA active on peripheral blood BFU-E from normal individuals. These data suggest that the spleen may have an influence on the numbers and functional properties of BFU-E.
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PMID:The relationship between human spleen and blood erythroid burstforming units (BFU-E). 668 75

A review of the hospital course of 82 splenectomies in children performed in the past 15 years has been carried out. Three indications have been identified: therapeutic, diagnostic, and traumatic. Thalassemia was identified as the commonest indication. Comparisons of the spleens removed showed that those from thalassaemia patients were the biggest, while those removed for idiopathic thrombocytopenic purpura (ITP), lymphoma staging, and trauma, were small. Twenty-eight per cent of patients had accessory spleens. Intrathoracic complications were frequent in the traumatic cases, whereas wound complications were commoner after splenectomies carried out for haematological reasons. No gallstones were detected. The platelet response to the various indications was studied and compared. There were two postoperative deaths.
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PMID:Splenectomy in children in Hong Kong. 693 77

Although full blood counts (FBC) are among the most commonly performed laboratory tests, the contribution of routine FBCs to the diagnosis of new problems is controversial. This study represents a unique linkage of a consultant haematology team, reviewing all abnormal blood counts, to an organization providing ambulatory health care to 350,000 patients. The objective was to establish the underlying clinical disorders responsible for all abnormal FBCs during a 2-month period, and to estimate the impact of the haematology team on the diagnostic work-up and management of newly identified problems. 572 (2.55%) of the 22,454 FBCs were abnormal. Of these, 357 showed microcytosis, caused by iron deficiency (58%), thalassaemia minor (35%), inflammation (6%) or chronic renal failure (1%). The most common causes of normocytic anaemia (25 patients) were disseminated malignancy and acute blood loss; of macrocytosis (27 patients), chronic liver disease and cancer; of erythrocytosis (16 patients), chronic hypoxia; of thrombocytopaenia (48 patients), chronic liver disease and ITP; of thrombocytosis (47 patients), iron deficiency and inflammation; of leukopaenia or pancytopaenia (20 patients), cirrhosis and disseminated malignancy; and of leukocytosis (26 patients), chronic leukaemias in the elderly and infection in children. Major new haematological abnormalities were encountered in 0.24% of all blood counts, representing about one new diagnosis per day. Routine blood counts do contribute to the health care of a population. Screening for haematological disease through a central clinical laboratory covering a large high-risk ambulatory population offers a cost-effective way of searching for serious clinical problems, alerting the primary physicians of their existence, and offering advice in continued evaluation and problem management.
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PMID:The haematologist as watchdog of community health by full blood count. 779 88

Recently there have been increasing reports of HIV infection acquired through transfusion of HIV seronegative blood in Thailand due to high incidence of HIV new infection in blood donors. Blood or blood components (BC) prepared from HIV seronegative blood donation pose significant hazards to recipients because of the risk of viremia during the "window period" of HIV infection. This paper presents the HIV seroprevalence in hematologic patients other than hemophiliacs who received multiple blood transfusion at Ramathibodi Hospital. The retrospective analysis was done on 167 patients: 132 thalassemia, 19 leukemia, 5 aplastic anemia, 5 ITP, 2 pure red cell aplasia, 2 congenital non spherocytic hemolytic anemia, 1 hereditary spherocytosis and 1 autoimmune hemolytic anemia patients, who received blood transfusion during January 1, 1987 till February 29, 1992 at the Department of Pediatrics, Ramathibodi Hospital. The number of blood or BC transfused in each patient was 1-154 units with the average of 23 units per patient per 5 years with a total 4,000 units. All were HIV sero-negative. Anti-HIV screening was performed periodically in these patients about 1-2 times per year or as necessary. The results were HIV seronegative in all cases. The reason for negative results cannot be explained clearly. It should be noted that our thalassemic patients receive leukocyte poor blood and avoid a hypertransfusion program. Patients with other blood diseases received both whole blood and BC. The HIV contaminated blood in the window period was estimated to be 1:10,000 in Thailand which showed HIV antigen positive but antibody negative. These patients may be fortunately received HIV non contaminated blood.
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PMID:HIV seroprevalence in hematologic patients other than hemophiliacs at Ramathibodi Hospital. 788 70


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