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Biomedical subjects

M Brozovic

Publications and source records attributed to M Brozovic.

At least 55 records · Page 3Linked to original sources

Incidence of overt and covert neurological damage in children with sickle cell disease.

We studied 25 patients with sickle cell disease (15 with HbSS; 8 with HbSC and 2 with HbS beta degree) using neurological examination as a marker of overt and computed tomographic (CT) scan as a marker of covert neurological damage. Of the 25 patients, 5 (20%) had evidence of cerebral infarcts on CT scan, but only 4 of these 5 patients had neurological signs. Neurological problems were only seen in patients with HbSS. We could not identify any haematological risk factors, but in this small group of HbSS patients a crisis rate greater than 1 per year of follow up was associated with a significant risk of neurological damage.

Adolescent↗

Haemostatic function and ischaemic heart disease: principal results of the Northwick Park Heart Study.

The Northwick Park Heart Study (NPHS) has investigated the thrombotic component of ischaemic heart disease (IHD) by the inclusion of measures of haemostatic function. Among 1511 white men aged between 40 and 64 at the time of recruitment, 109 subsequently experienced first major events of IHD. High levels of factor VII coagulant activity and of plasma fibrinogen were associated with increased risk, especially for events occurring within 5 years of recruitment. These associations seemed to be stronger than for cholesterol, elevations of one standard deviation in factor VII activity, fibrinogen, and cholesterol being associated with increases in the risk of an episode of IHD within 5 years of 62%, 84%, and 43% respectively. Multiple regression analyses indicated independent associations between each of the clotting factor measures and IHD but not between the blood cholesterol level and IHD incidence. The risk of IHD in those with high fibrinogen levels was greater in younger than in older men. Much of the association between smoking and IHD may be mediated through the plasma fibrinogen level. The biochemical disturbance leading to IHD may lie at least as much in the coagulation system as in the metabolism of cholesterol.

Adult↗

Red cell alloimmunization in sickle cell disease.

Alloimmunization to red cell antigens contributes to morbidity in transfused patients. It has been recommended that blood for sickle cell patients need not be matched for antigens other than ABO and Rh(D), as there is no greater incidence of antibody production than in other multitransfused patient populations. Post transfusion alloimmunization was studied in a group of 34 sickle cell disease patients attending a U.K. haemoglobinopathy clinic. Red cell antibodies were formed in 17.6% of the transfused patients and Rhesus and Kell antibodies accounted for 66% of this total. In order to reduce alloimmunization, a policy of performing extended red cell phenotyping on the patients, and providing blood matched for Kell, and in certain circumstances the Rhesus antigens other than Rh(D), is recommended.

Adult↗

An unusual variant of T-CLL: evidence for the existence of a hitherto unrecognized T cell subset.

A case of T-cell chronic lymphocytic leukaemia (T-CLL) with an unusual mature membrane phenotype: E+, CD3+, CD4+, CD8-, M1+, Leu-15+, Fc gamma+, is described. The cells were large granular lymphocytes with slight immature features. Functionally these cells lacked helper, suppressor and NK activity but possessed normal levels of K activity. These findings demonstrate several features not previously described in T-CLL: the coexpression of the antigens detected by T4, M1 and Leu-15 the presence of Fc gamma receptors on CD4+ lymphocytes and the lack of NK activity in M1+, Fc gamma+ cells. This study broadens the known heterogeneity of T-CLL and suggests the existence of a hitherto unrecognized normal T-lymphocyte subset with the same functional and phenotypic characteristics as in the case described here.

Antibodies, Monoclonal↗

Neoplastic kappa and lambda cells in a B-PLL with chromosome translocations of both light chain gene regions.

We describe a chromosome translocation t(2;13) (p11-12;p11) involving the kappa (kappa) light chain gene region (2p11-12), and a subsequent translocation t(11;22) (q23;11) involving the lambda (lambda) light chain gene region (22q11) within the same clone, in a patient with B-cell prolymphocytic leukaemia (B-PLL), of whose peripheral mononuclear cells 82% expressed (kappa) chains and 8% expressed (lambda) chains. Electron microscope (EM) studies using the immunogold method showed that both kappa- and lambda-producing cells were prolymphocytes. Immunoglobulin (Ig) gene analysis by Southern blotting demonstrated rearrangement of both Ig heavy (H) chain genes and one kappa gene. Although a lambda gene rearrangement corresponding to the minor lambda-positive population was not detected, a small monoclonal (M) band of IgM-lambda was present in the serum. The chromosome translocations and the pattern of light chain expression, particularly the lambda-producing cells, are discussed in the light of the restricted light chain expression observed in Burkitt's lymphomas with variant translocations involving the light chain genes.

B-Lymphocytes↗

Screening cord blood for sickle haemoglobinopathies in Brent.

Between 1981 and 1983, 3165 consecutive specimens of cord blood were tested at the Central Middlesex Hospital for the presence of an abnormal haemoglobin: the incidence of sickle cell trait was 2.8%, of HbC trait 0.9%, and the overall incidence of an abnormal haemoglobin at birth was 6.9%. Five babies with homozygous sickle cell disease, three with HbSC, and three with either HbCC or HbC beta thalassaemia were detected. Twenty two per cent of the mothers were of Afro-Caribbean origin. The cost of the test was 30p. An H6000 blood count was carried out on 1000 consecutive cord blood samples. The mean red cell volume was 97.95 (SD 3.67) fl. Thirteen cord blood samples had a mean cell volume below 85 fl, and all contained Hb Barts. In addition, six samples with a mean cell volume between 86 and 92 fl also showed Hb Barts on electrophoresis. The overall incidence of Hb Barts was 2.1%. These results indicate that the incidence of HbSS and HbSC on neonatal screening in Brent is similar to that found in the urban areas of North America and that the number may be predicted from the number of births to mothers of Afro-Caribbean origin.

Anemia, Sickle Cell↗

Acute chest syndrome in sickle-cell disease.

25 consecutive episodes of acute chest syndrome in 13 adult patients with sickle-cell disease were studied retrospectively. Chest symptoms were present on admission in 23 of 25 episodes. Abnormal chest signs and an abnormal chest X-ray were present on admission in only 11 and 9 episodes, respectively, but developed later in the remainder. The 9 episodes with bilateral radiological changes were associated with higher pulse rates, longer-lasting fever, more profound arterial hypoxaemia, and greater falls in haemoglobin than the 16 unilateral episodes. 2 patients with bilateral disease died; both had platelet counts less than 100 X 10(9)/litre. In 12 episodes (6 bilateral, 6 unilateral) exchange transfusion was required and produced striking improvement in 11. Despite intensive microbiological investigation, infection was found in only 2 episodes--1 mycoplasma and 1 evidence of Escherichia coli. Pulmonary intravascular sickling may account for much of the clinical picture.

Acute Disease↗

Effect of blood transfusion on iron status in sickle cell anaemia.

The iron status of 31 patients with sickle cell anaemia (Hb SS) and balanced globin chain synthesis was studied. Twelve patients (group I) had never been transfused; 14 had received up to 4 units of blood in the past (group II) and five had been hypertransfused for 6 months to 2 years (group III). The hypertransfused group had significantly higher MCV and MCH than the nontransfused one, and significantly lower total iron binding capacity and higher serum ferritin concentration than either groups I or II. The serum ferritin concentration was lower than normal in eight patients (five in group I and three in group II), and higher than normal in seven patients (four in group III and three in group II). The remaining 16 patients had normal serum ferritin concentrations. Our results indicate that iron overload is uncommon in adults with Hb SS who have not been transfused, and that a proportion of patients have lower than normal serum ferritin concentrations. Transfused patients, even 6 months after the last transfusion, show increased haemoglobin content of red cells, which may have an adverse effect on the frequency and severity of sickle crises.

Adult↗

Haemostatic changes following surgery.

Changes in factors V, VII and VIII and in fibrinogen were studied in 32 patients undergoing major abdominal surgery. Mean levels of factors V and VII were similar to population-based values preoperatively. Factor V fell following elective surgery and then rose above the mean pre-operative level before returning to it by the tenth post-operative day. Factor VII fell following both elective and emergency surgery and tended to remain depressed throughout the post-operative period. Pre-operative values of factor VIII and fibrinogen were higher than population-based values and higher in the emergency than in waiting-list patients. Both factor VIII and fibrinogen rose following elective surgery but no statistically significant change was seen following emergency surgery. The uncomplicated conditions leading to elective surgery, the acute complications leading to emergency surgery, and surgery itself may each have contributed to increases in factor VIII and fibrinogen levels, whereas the fall in factors V and VII was largely related to surgery itself. The findings may help in the interpretation of associations between clotting factors and thrombotic disease, particularly in the case of factor VII.

Aged↗

Clinical staging and immunological findings in chronic lymphocytic leukemia.

Several immunological markers were tested in 52 untreated cases of chronic lymphocyte leukemia (CLL) to see whether their frequency differed according to the clinical stage in Rai's system. The leukemic cells in all cases had B-cell features as shown by monoclonal immunoglobulins on the cell surface (SmIg) and/or a high percentage of mouse RBC (M)-rosettes. Of the two B-cell markers, the M-rosette test was the more consistently positive. The frequency of these markers did not correlate with clinical staging. The percentage of T-lymphocytes, low in all cases, was found to correlate inversely with the lymphocyte counts, which were higher in advanced stages. The absolute number of T-lymphocytes was above normal in most cases, but did not relat to staging. At least one of the serum Ig, most commonly IgA, was decreased in 87% of cases. Low Ig were slightly less common in Stages 0-I than in advanced stages (II-IV). The above features were also examined in two groups of CLL patients: with stable (9) or progressive (16) disease. The only difference observed between the two groups was that surface IgM only was present in 1 of the 9 stable cases as compared to 9 of the 16 progressive ones. Our findings do not support the suggestions that surface IgM is a feature of a benign form of CLL or that the absolute number of T-lymphocytes correlates with prognosis.

Adult↗

Relationship of air temperature to various chemical, haematological, and haemostatic variables.

Results for biochemical and haematological variables have been correlated with data on atmospheric temperature in order to identify possible mechanisms through which low environmental temperature may increase mortality from myocardial infarction and cerebrovascular disease. With the exception of cholesterol, there were no associations in the case of several clinical chemistry variables, or of haemoglobin and related indices. With varying degrees of consistency among the sex and age groups studied, temperature was positively correlated with factor VII, antithrombin III, and cholesterol, and negatively correlated with fibrinolytic activity. The correlations were all low but may offer some clues to mechanisms whereby air temperature influences ischaemic heart and cerebrovascular disease mortality.

Adolescent↗

Self-inflicted bleeding and bruising.

Of 8 psychiatrically referred patients with bruises and bleeding, self-injury was admitted in 2 cases and the means of self-injury were discovered in 6. All the patients were fairly young, single or unhappily married females, with unresolved emotional problems, most commonly sexual. 22 of 43 female haematology outpatients showed no haemostatic abnormality and were though to be seeking attention.

Adolescent↗