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

Hematology results from 3800 blood samples were examined for the presence of erythrocytic microcytosis. One hundred patients with electronically measured mean corpuscular volumes of 75 cubic microns or less were reviewed to determine the cause of microcytosis and evaluate recently described simplified techniques for separating thalassemia trait from other causes of microcytosis. The discriminant function formula of England and Fraser [MCV - RBC - (5 x Hgb) - 3.4] was found to be useful in uncomplicated patients with the Mediterranean type of thalassemia trait (betadegree) but less useful in the African type (beta+) of heterozygous beta-thalassemia. Anemias of chronic disorders were found to be a major cause of microcytosis. Microcytosis of no apparent cause was found in some children. Improved techniques in hemoglobin A2 quantification remain the best approach for detecting beta-thalassemia heterozygotes.
 ...
PMID:Erythrocytic microcytosis: clinical implications in 100 patients. 87 Nov 34

A number of patients of Mediterranean and Asian origins were found to have unexplained microcytic hypochromic red blood cells. Iron deficiency and beta-thalassaemia trait were both satisfactorily excluded in all of them. The haematological indices of these patients, obtained on a Coulter Model 'S' Counter, were found to be very similar to those seen in obligatory heterozygotes for alpha-thalassaemia. It is postulated that these patients were also carriers for alpha-thalassaemia. Subsequent investigation of some of these patients showed the characteristically reduced rates of alpha-chain synthesis seen in this condition. The discriminant function of England and Fraser (1973) may be of help in diagnosing this state. alpha-Thalassaemia trait should be considered in all patients of 'high-risk' ethnic origins with a blood picture suggestive of beta-thalassaemia trait but in whom the levels of Hb A2 and Hb F are within normal limits.
 ...
PMID:Diagnosis of alpha-thalassemia trait from Coulter Counter 'S' indices. 91 18

The haematological parameters of 97 cases of beta thalassaemia trait and 40 cases of delta beta thalassaemia trait have been compared. No differences in haemoglobin, haematocrit, MCV, MCH, ferritin, % saturation or free erythrocyte protoporphyrin have been found. The RDW, however, is significantly increased in delta beta thalassaemia trait, its mean value (+/- SD) being 20 (2.05), even higher than that found in iron deficiency anaemia. The discrimination function described by England and Fraser may be of help in distinguishing these entities.
 ...
PMID:[Hematometric values in delta-beta thalassemia minor. Special importance of the erythrocyte distribution in comparison with beta thalassemia and iron deficiency]. 227 45

In order to estimate the validity of the discriminant function suggested by England e Fraser, we have determined the number of red cells (RBC) mean cell volume (MCV), hemoglobin mean concentration (MCV) and red cells distribution (RDW) in normal subjects, iron-deficient patients and uncomplicated thalassemia minor. The results obtained allow us to point out that RDW in the patients with thalassemia are higher than in normal subjects. In patients with iron-deficiency, instead, RDW medium is increased, but with a remarkable distribution of values. These results point out that anisocytosis is higher in iron-deficiency than in thalassemia.
 ...
PMID:[Differential erythrocyte parameters in thalassemia minor and hyposideremic syndromes]. 240 79

A new expert system developed on a Macintosh personal computer using a commercially available artificial intelligence shell was compared with four different discriminant functions (DFs) for the differentiation of microcytic anemia into etiologic categories. Several databases were used with a different composition but all contained at least some samples from thalassemic individuals and from patients with iron deficiency anemia. The DFs analyzed were those proposed by England and Fraser, Green and colleagues, Mentzer, and by Shine and Lal. None of the databases performed satisfactorily when used singly, whereas very high false-positive rates were obtained by one of them. The diagnostic efficiency was somewhat improved by combining several DFs. An expert system using an artificial intelligence "shell" with an "interference engine" was developed using cluster analysis and a set of learning examples. The input necessary for the system to achieve a conclusion consists of MCV, RBC, and RDW as well as a statement as to whether the patient has anemia. Based upon the values of these parameters, the expert system will give an "advice" regarding the probabilities for thalassemia, iron deficiency, and/or other probabilities such as previous transfusions, anemia of chronic disease, laboratory error, etc. In a prospective trial, the system functioned with an accuracy of better than 85%.
 ...
PMID:The use of inference strategies in the differential diagnosis of microcytic anemia. 262 97

The use of automated analysers in population screening for beta-thalassaemia has been a matter of controversy. The new fully automated haematology analyser Sysmex E-5000 (Toa Medical Electronics Co. Ltd) facilitates the discrimination of heterozygous thalassaemia from iron deficiency anaemia. In addition to haemoglobin, mean corpuscular haemoglobin and mean corpuscular volume, the red cell size-distribution width is measured. In patients with hypochromic microcytic red cells, the Sysmex data have been evaluated and compared with the indices described by England and Fraser [Lancet i, pp. 449-452, 1973], Mentzer [Lancet i, p. 882, 1973] and by Shine and Lal [Lancet i, pp. 692-694, 1977]. For the detection of beta-thalassaemia trait, the size-distribution width is superior to the previously described indices. The sensitivity is 79%, the specificity 95% and the predictive value for a positive test 94%.
 ...
PMID:Can automated haematology analysers discriminate thalassaemia from iron deficiency? 312 Apr 68

The results of a screening for beta-heterozygous thalassemia conducted on 999 school-boys aged from 11 to 13 are the following: 7.5% of the subjects were taker of the thalassemia trait; 74 subjects over 75 with beta-heterozygous thalassemia had "Mean Corpuscolar Volume" (MCV) values below 70 fl. The results of the subjects with globular volume less than or equal to 79 fl (with or without beta-thalassemia) were used to compare the diagnostic accuracy of beta-thalassemia for the hematological indexes of England-Fraser, Mentzer, Shine-Lal and the MCV estimations. This comparison has shown better results in terms of sensibility and specificity for MCV with respect to the other indexes. A more extensive application of the study could be convenient in order to evaluate if the MCV level of 70 fl is effective to discriminate between beta-thalassemia and non thalassemic microcytosis.
 ...
PMID:[Value of various hematologic indexes proposed for identifying healthy carriers of the beta-thalassemic trait]. 383 19

A small scale screening study for beta-thalassaemia trait has been carried out in the Gaza Strip, involving 1650 secondary schools healthy students, 16-18 years old and from both sexes. The results showed that the overall prevalence in the Gaza Strip of beta-thalassaemia was 4.3%. The frequency of beta-thalassaemia trait in the microcytic (defined as MCV < or = 80fl and/or MCH < or = 26 pg) subjects was 27.1%. The efficacies of some of the proposed discrimination functions in the differentiation between beta-thalassaemia trait and non-thalassemic microcytosis were evaluated. The Mentzer index, MCV of < or = 72fl, England & Fraser DF and the Shine & Lal formula were found to correctly identify 91.6%, 82.4%, 81.3% and 62.6% of the studied cases of microcytosis as having or not having the beta-thalassaemia trait. It was concluded that both beta-thalassaemia and microcytic anaemias are major health problems in the Gaza Strip. The various forms of consanguineous marriages, in addition to poor economic conditions in the Gaza Strip may have contributed to the concentration of beta-thalassaemia and the prevalence of microcytic anaemias in this population.
 ...
PMID:Screening secondary school students in the Gaza strip for beta-thalassaemia trait. 980 74

Iron deficiency anemia (IDA) and thalassemia minor are two of the most common causes of microcytic anemias worldwide. Because of similar red blood cell count parameters and blood picture, it was imperative to develop other measures that would differentially and correctly diagnose these two anemias. Several mathematical formulas and simple RBC indices have been introduced as simple, fast and inexpensive means of providing differential diagnosis for IDA and thalassemia minor. The Objective of this study was to apply and compare nine well-documented discriminant functions on a population of 153 confirmed cases of microcytic anemias (IDA n = 56, beta-thalassemia minor n = 47 and alpha-thalassemia n = 50) and to measure validity using Youden's Index. The results show that England and Fraser (E & F) Index had the highest Youden's Index value (98.2) in correctly differentiating between IDA and alpha- and beta-thalassemia minor, while Shine and Lal Index was found ineffective in differentiating between microcytic anemias in our population. E & F Index showed with great sensitivity and specificity to be the best discriminant function to differentiate between IDA and thalassemia minor cases.
 ...
PMID:Validity assessment of nine discriminant functions used for the differentiation between iron deficiency anemia and thalassemia minor. 1716 36

Beta-thalassaemia minor and iron deficiency are the most common causes of microcytosis and/or hypochromasia. The present study evaluates the diagnostic reliability of different RBC indices and formulas, as well as our proposed formula, in the differentiation of the beta-thalassaemia minor from iron deficiency in Palestinian population. Complete blood count (CBC) parameters of 2196 certainly diagnosed (1272 beta-thalassaemia minor and 924 iron deficiency) samples were used to evaluate the following indices and formulas: Bessman index (RDW), Mentzer formula (MCV/RBC), England and Fraser formula (MCV - RBC - 5 x Hb- 3.4), Shine and Lal formula (MCV2 x MCH/100), Ehsani formula (MCV-10 x RBC), Srivastava formula (MCH/RBC), Green and King formula (MCV2 x RDW/Hb x 100), red distribution width index RDWI (RDW x MCV/RBC), RDW/RBC, as well as our formula (MCV-RBC -3 x Hb). For each index and formula, the receiver operative characteristic (ROC) curve was constructed to calculate the area under the curve (AUC), in addition, sensitivity, specificity, and likelihood ratios were calculated. No significant differences were reported between our formula, Green-King formula and the RDWI (P > 0.05) in discriminating beta-thalassaemia minor from iron deficiency (AUC = 0.914, 0.909 and 0.907 respectively). However, the three indices and formula showed the highest efficiencies and they were significantly (P < 0.05) better than the others in the discrimination efficiency . It was concluded that our formula, Green-King formula and the RDWI provided the highest reliabilities in differentiating beta-thalassaemia minor from iron deficiency in Palestinian population while Bessman index was poor and ineffective for that purpose.
 ...
PMID:Evaluation of the diagnostic reliability of different RBC indices and formulas in the differentiation of the beta-thalassaemia minor from iron deficiency in Palestinian population. 1837 Oct 62


1 2 Next >>