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)

A microcomputer program (BCDE) has been developed to analyze automated blood cell counts and differentials' similarity to normal values or to 36 disease categories. In 50 normal subjects, the analytic program listed the correct diagnosis as the first diagnosis in 49 cases (the only diagnosis in 44) and second of two diagnoses in one case. In 182 subjects with known hematologic disorders, the correct diagnosis was listed first in 134 and second or third in an additional 40. Subjects with iron deficiency, heterozygous thalassemia, immune thrombocytopenia, anemia of chronic disease, reactive thrombocytosis, acute infection, and chronic leukemia had the disorder identified as the most likely one by the analytic program with both sensitivity greater than 80% and specificity greater than 98%. Subjects with acute leukemia, folate deficiency, sickle cell anemia, cytotoxic chemotherapy, and chronic liver disease had the disorder identified as most likely by the program with a sensitivity less than 80%. In a different 11 cases with known hematologic status, a panel of 37 physicians identified the disorder(s) or normality only 72% of the time, whereas the analytic program listed the correct diagnosis first in 10 of 11 (91%). The analytic program appears useful for both triage of normal from abnormal data and for the initial differential analysis of abnormal data.
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PMID:Evaluation of BCDE, a microcomputer program to analyze automated blood counts and differentials. 330 76

We studied 29 patients with thalassaemia major who had received intensive chelation for between 6.2 and 8.8 years. All patients had normal oral glucose tolerance tests before subcutaneous chelation therapy was introduced and 22 of 29 patients had normal liver function tests. At the end of the period of study 12 patients still had normal oral glucose tolerance (7 with normal liver function tests and 5 with chronic active hepatitis). On the other hand, 11 patients had developed impaired glucose tolerance tests (3 patients had normal liver function tests, 5 with chronic active hepatitis and 3 with cirrhosis), and 6 patients had developed frank diabetes mellitus (one with chronic active hepatitis and 5 with cirrhosis). Patients with chronic active hepatitis showed 91% positivity for one or more hepatitis B markers whilst all patients with cirrhosis were positive. Ferritin levels before subcutaneous chelation in patients with normal oral glucose tolerance tests were lower than in those patients with abnormal oral glucose tolerance or diabetes (P less than 0.05) but none had normal serum ferritin levels. In addition, a positive correlation was found between glucose area under the curve after chelation therapy and serum ferritin levels (r = 0.47, P less than 0.01). It is apparent that long term chelation therapy does not prevent the development of abnormal oral glucose tolerance in chronically transfused patients. More intensive chelation therapy is needed to prevent tissue damage. Chronic liver disease may have an important role to play in the deterioration of glucose tolerance.
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PMID:The development of diabetes mellitus and chronic liver disease in long term chelated beta thalassaemic patients. 354 13

Monoclonal antibodies (OKT series) have been used to investigate possible modifications of T lymphocytes and T lymphocyte subsets in 65 multiply transfused beta-thalassemia patients. No significant difference was observed in percentage and absolute number of OKT3-, OKT4-, and OKT8-positive cells when compared to controls. A subgroup of patients (10 patients, 15.3%), however, could be selected who showed a reversal of OKT4/OKT8 ratio. These patients did not differ from the others as to age, number of transfusions, frequency of splenectomy, ferritin levels, hepatitis B markers, chronic liver disease incidence, and numbers of B lymphocytes and natural killer cells. The features distinguishing this group from the remaining patients were: decreased mitogen responsiveness; early age when first transfused; high incidence of males (90%). Immunological investigation was done in 2 occasions, 1 year apart, but no significant modification was observed in these patients. These findings suggest that in beta-thalassemia patients transfusion therapy started very early in life may be responsible for persistent immunological modifications. The susceptibility to such modifications might be greater in males.
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PMID:Altered T cell subsets and function in polytransfused beta-thalassemia patients: correlation with sex and age at first transfusion. 387 36

Superoxide anion production in resting and PMA- or zymosan-stimulated neutrophils was evaluated in 21 beta-thalassemia patients. The results were correlated with ferritin values, hepatitis B virus serum markers, liver pathology, immunoglobulin levels and T-cell subsets. Superoxide anion generation from resting or PMA-stimulated neutrophils was significantly higher in patients than in controls. On the contrary, zymosan-stimulated neutrophils showed reduced superoxide anion production. Increased superoxide anion production in resting neutrophils showed a significant correlation to the values of ferritin. In addition, patients with biopsy-proven chronic liver disease showed significantly increased ferritin levels and superoxide anion production as compared to the remaining patients. No correlation was observed between superoxide anion production and the presence or the absence of hepatitis B virus serum markers, immunoglobulin levels and T-cell subsets. A possible role of interreactions between iron and oxygen radicals in determining liver damage in beta-thalassemia patients is suggested.
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PMID:Changes in superoxide anion production in neutrophils from multitransfused beta-thalassemia patients: correlation with ferritin levels and liver damage. 633 Oct 44

IgE values obtained in 117 beta-thalassaemia patients were significantly higher than in age matched normal subjects. In 31 patients (26.5%) IgE levels were above 2 s.d. of normal values for age, but the frequency of IgE with reaginic activity was lower in patients (5.1%) than in controls (11.9%). The highest values were observed in splenectomized patients who were also positive for one or more serological markers of hepatitis B virus infection. The increase of IgE levels was directly correlated with the number of years after splenectomy, and patients with biopsy proven chronic liver disease had higher IgE levels than those without evidence of liver damage. On the other hand, IgE levels were not correlated with the number of transfusions, age, IgG, IgA, IgM levels or T cell subsets and mitogen responsiveness. These results show that beta-thalassaemia patients develop elevated IgE levels to which splenectomy and hepatitis B virus infection contribute in a synergistic manner.
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PMID:Raised IgE levels in beta-thalassaemia: correlation with splenectomy and hepatitis B virus infection. 633 94

The inactivated hepatitis B vaccine that was licensed in November 1981 will be distributed for general use later this year. Extensive studies have confirmed the safety, immunogenicity, and remarkable efficacy of this vaccine for the prevention of acute hepatitis B disease, asymptomatic infection, and the chronic hepatitis B carrier state. The vaccine is recommended for immunization of infants, children, and adults who are considered to be at increased risk of contracting hepatitis B infection. These population groups include medical, dental, laboratory, and other health care personnel, selected patients (eg, hemodialysis, thalassemia), clients and staff of institutions for the mentally disabled, homosexually active males, intimate contacts of carriers, users of illicit drugs, infants in high-risk areas, and other high-risk groups. The availability and appropriate use of the newly licensed hepatitis B vaccine should enable physicians to prevent a serious cause of acute and chronic liver disease.
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PMID:The newly licensed hepatitis B vaccine. Characteristics and indications for use. 703 74

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

Vibrio vulnificus infection, which is a rare and fatal disease, can be categorized clinically as either primary septicemia or wound infection. The clinical presentation of patients with primary septicemia can vary from fever alone to a more severe illness including high-grade bullous lesions, hypotension, and shock. Wound infection typically results from either injury to the skin in a marine environment or contact of a preexisting wound with sea water. We reported eight cases with Vibrio vulnificus infection in Chang gung Memorial Hospital and reviewed ten other cases previously reported with details in Taiwan. Fourteen patients presented with primary septicemia, and four with wound infection. Thirteen patients had alcoholism or chronic liver disease, two had peptic ulcer disease, one was steroids abuser, and one patient had thalassemia and chronic liver disease. Overall mortality was 55.6% (ten patients). Patients with hypotension within 48 hours of admission had higher mortality than normotensive patients (77% vs. 0%, P = 0.007). Patients with chronic liver disease or liver cirrhosis also had tendency to a higher mortality than not (64% vs. 25%, P = 0.274). Chronic liver diseases and liver cirrhosis are common disease in Taiwan. They take a high risk for Vibrio vulnificus infection. Clinician should keep in mind of this potentially fatal infection in these patients reporting a history of recent raw oyster consumption and presented with sepsis and characterized skin lesions. Prompt empirical antibiotics treatment and aggressive surgical treatment may be lifesaving for this acute and fatal disease.
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PMID:Vibrio vulnificus infection--report of 8 cases and review of cases in Taiwan. 785 Jun 49

We studied the prevalence of HCV antibody seropositivity and serum alanine concentrations in a random sample of healthy Egyptian children (n = 110) as well as in four high risk groups of children. Group 1 included 18 children with thalassemia major, group 2 included 17 children with insulin-dependent diabetes mellitus (IDDM), group 3 included 21 children with schistosomal hepatic fibrosis (SHF), and group 4 included 20 children with chronic rheumatic heart disease (RHD). The prevalence rate of HCV seropositivity was 12 per cent in normal children, 44 per cent in thalassemic children, 29 per cent in children with IDDM, 38 per cent in children with SHF and 0 per cent in patients with RHD. The liver size was significantly larger in HCV seropositive normal children as well as in HCV seropositive children with thalassemia and SHF compared to the seronegative children in each group respectively (P < 0.05). In all groups serum alanine transferase concentrations were significantly higher in HCV seropositive v. seronegative children. This pointed out to the high risk of continuous parenchymal hepatic damage in these children following acute HCV infection. In summary, our data revealed a relatively high prevalence of HCV antibody seropositivity in healthy Egyptian children compared to reports from other countries, and a significantly high prevalence of HCV seropositivity in children with thalassemia, IDDM, and SHF which carries a considerably high risk for development of chronic liver disease in these patients.
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PMID:Prevalence of hepatitis-C antibody seropositivity in healthy Egyptian children and four high risk groups. 860 41

The study group screened for anti-HCV comprised 789 subjects of hepatitis, renal failure, thalassaemia and healthy voluntary blood donors coming from Central India during July 1992 to November 1995. The prevalence of HCV was low (4.85%) among 103 patients of acute viral hepatitis (AVH) while it was higher (25.64%) among 117 patients of chronic liver disease (CLD) with the highest rate of 31.57 percent in 57 patients of cirrhosis. The anti-HCV positivity among 101 patients with hepatic failure was around 10 percent. High risk groups such as chronic renal failure (CRF) patients mainly on haemodialysis and thalassaemics receiving multiple blood transfusions showed the prevalence of anti-HCV in 41.9 and 25.45 percent respectively. Only 1.78 percent of the 280 voluntary blood donors showed positivity for anti-HCV. Comparison of the data on HCV in the present study with data from other parts of India showed a wide variation in the different centers. The higher prevalence of HCV among CRF patients and thalassaemics indicates the need for screening of the blood units for anti-HCV before transfusion to these high risk patients.
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PMID:Prevalence of anti-HCV antibodies in central India. 884 Jun 56


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