Search PubMed⌕ Search

Biomedical subjects

M Brozović

Publications and source records attributed to M Brozović.

At least 19 recordsLinked to original sources
PubMed1989

The presentation, management and prophylaxis of sickle cell disease.

Sickle cell disease (SCD) is encountered in all parts of the world where plasmodium falciparum has been endemic and has spread by migration to the temperate countries of the world resulting in a heavy caseload in Northern Europe and the United States. These patients in the temperate climates manifest most of the clinical problems associated with SCD in Tropical Africa and the West Indies. There are, however, differences between the groups in both the frequency and presentations of the clinical syndromes. The clinical management of SCD is discussed with particular reference to the potentially fatal sequestration syndromes: splenic, hepatic, 'the girdle syndrome' and 'the chest syndrome'. In all clinical situations encountered in SCD blood transfusions should be by isovolaemic exchange unless there is a marked fall in haematocrit (less than 5 g/dl) as may occur with sequestration and aplasia. The criteria for exchange transfusion in the chest syndrome are a pAO2 of less than 60 mm Hg while breathing air or a rapidly deteriorating clinical picture. Analgesia for vaso-occlusive sickle pain should be adequate and freely available which often requires the parental administration of opiates. We have delineated a small sub-group (6%) of SCD patients, 'the non-copers', who manifest a high demand for analgesia. The prospects for cure, and the techniques and issues for antenatal diagnosis are reviewed demonstrating the importance of education and counselling. The significant reduction in mortality and morbidity of children with SCD taking penicillin prophylaxis is emphasised with the need for its early institution.

Adolescent↗
PubMed1988

Counselling for prenatal diagnosis of sickle cell disease and beta thalassaemia major: a four year experience.

A non-directive programme of prenatal counselling was used during a four year period. Forty-three couples at risk for having a baby with a haemoglobinopathy were identified. Prenatal diagnosis was offered in 19 pregnancies to 14 couples at risk of having a baby with sickle cell anaemia and in two pregnancies in two couples at risk of a baby with beta thalassaemia major, who presented before the 18th week of pregnancy. Six couples at risk for sickle cell anaemia accepted prenatal diagnosis in 10 pregnancies, as did both couples at risk for thalassaemia. Couples who were eligible for prenatal diagnosis but refused it tended not to have been informed about sickle cell disease before counselling, one partner was more frequently absent at the time of the initial counselling session, or they either had no children with sickle cell disease or the children were not severely affected. Other factors influencing their decision included a poor obstetric history and rejection of abortion, mainly on moral grounds. The approximately 50% uptake of prenatal diagnosis in this initial study highlights the complex issues involved. Our experience indicates that with systematic screening and counselling in the antenatal clinic, and with increased awareness of the haemoglobinopathies, couples at risk will be in a better position to make informed decisions.

Anemia, Sickle Cell↗
PubMed1987

Acute admissions of patients with sickle cell disease who live in Britain.

All acute admissions of patients with sickle cell disease who lived in the London borough of Brent and attended this hospital were analysed for a period of one year. Sixty three of the 211 patients who were followed up by the haematology department required 161 acute admissions during the year. Most admissions (126) were for the 42 patients with homozygous sickle cell disease; 147 (91%) were for vaso-occlusive episodes, 142 of which were for painful crises, three for cerebrovascular accidents, and two for renal papillary necrosis. Preschool children with sickle cell disease were admitted predominantly with limb pain, whereas in schoolchildren and adults the incidence of trunk pain was higher. Twenty four of the 93 episodes of trunk pain culminated in an episode of severe visceral sequestration usually affecting the lungs, the liver, or the mesenteric circulation. Two patients died: an 18 month old baby with an acute splenic sequestration crisis and a 19 year old man with a severe girdle syndrome (sickling in the mesenteric circulation, liver, and lungs). Infective episodes were rare (11 episodes) but severe: one haemophilus meningitis, two salmonella infections, and three aplastic crises due to parvovirus infections. The average duration of the hospital stay was 7.4 days per admission. It is concluded that because sickle cell disease causes appreciable morbidity in older children, adolescents, and adults a systematic approach to management is needed to deal with acute episodes such as sequestration syndromes.

Adolescent↗
PubMed1986

Serum ferritin concentration in sickle cell crisis.

Serum ferritin, aspartate aminotransferase (AST), alkaline phosphatase and hydroxybutyrate dehydrogenase (HBD) were studied during 21 vaso-occlusive crises in 12 adults with sickle cell disease (11 SS, 1 S beta degrees). The patients comprised three groups: those who had been untransfused (4), those who had received occasional exchange transfusion in crisis (3), and those who had been multiply transfused (5). Serum ferritin concentrations in crisis were compared with those of the steady state value. Rises in serum ferritin concentrations occurred in all crises in all groups. Although AST, alkaline phosphatase, and HBD rose, there was no correlation between these and log ferritin concentrations. The clinical impression was that the degree of rise in ferritin related to the severity of the particular crisis, and the above results showed that haemolysis and liver damage were not causally related to this rise. An estimate of serum ferritin cannot be used to assess the state of iron balance in sickle cell disease unless the patient is in the steady state. The considerable rise in serum ferritin concentration found in crisis, however, may be a useful marker of the extent of vaso-occlusion and tissue damage.

Adolescent↗
PubMed1984

Sickle cell disease in Britain.

Sickel cell disease is common in urban areas of Britain and it is estimated that in London alone there are nearly 2000 patients. One hundred and eighty four patients with sickle cell disease are known to the Central Middlesex Hospital, and 155 of those attend the sickle cell clinic regularly. The commonest cause for admission to hospital is acute painful or vaso-occlusive crisis, which accounts for 80% of all acute admissions; 12% of admissions are for acute chest syndrome. Comparison of clinical features in Brent and in Jamaica shows that the Brent patients with homozygous sickle cell anaemia are admitted with painful crises more frequently than Jamaican patients. However, the frequency of admissions for chest syndrome and priapism, and the incidence of splenomegaly are similar. Leg ulcers are uncommon in Brent. Patients with sickle cell haemoglobin C disease appeared more severely affected in Jamaica than in Brent. During the past two years 3165 newborn babies have been screened for sickle cell disease at the Central Middlesex Hospital: five babies with homozygous sickle cell anaemia and three babies with sickle cell haemoglobin C disease were detected. The overall incidence of sickle cell trait was 3.2% and of haemoglobin C trait 0.8%. A significant number of babies with sickle cell disease are born in London every year. It is essential that such babies are detected at birth and offered prophylaxis against pneumococcal infection, which is one of the major causes of infant mortality. Sickle cell disease is becoming an important blood disease in Britain and firm guidelines for the management of acute and chronic complications are required.

Adolescent↗
PubMed1983

Iron deficiency in sickle cell anaemia.

Thirty-seven patients with SCD were studied: 24 were diagnosed as homozygous Hb S on the basis of their haematological findings, and alpha:non-alpha globin chain ratios were found to be balanced in all. Thirteen patients were thought to have alpha or beta thalassaemia interaction with Hb S on the basis of low MCV and MCH, family history and/or presence of Hb A on electrophoresis. Six of them had abnormal alpha:non-alpha ratio (one had a ratio of 0.72 suggestive of alpha thalassaemia, and five had ratios between 1.4 and 1.9, compatible with beta thalassaemia interaction). The remaining seven patients with microcytosis had balanced globin chain synthesis and five were found to be iron deficient. Five additional patients (3 with Hb SS and 2 with Hb S/beta thalassaemia) had lower than normal serum ferritin concentration. The analysis of case histories disclosed that peptic ulceration, recurrent epistaxis and multiple pregnancies could account for iron loss in seven patients. These findings indicate that iron deficiency may be common in SCD and should be excluded as a cause of microcytosis. Microcytosis, in the absence of conclusive family studies and/or presence of Hb A on electrophoresis, is an unreliable indicator of alpha or beta thalassaemia interaction with Hb S.

Adolescent↗
PubMed1981

Sickle-cell disease in a British urban community.

Seventy cases of sickle-cell disease were identified in the London Borough of Brent from records dating back to 1962. All but three were still alive and, with one exception, were recalled for confirmation of the diagnosis and to provide personal and family histories. The group consisted of 22 individuals with homozygous sickle-cell anaemia (Hb SS), 12 with sickle-cell/beta-thalassaemia double heterozygosity, 34 with sickle-cell/haemoglobin C disease (Hb SC), and two with the combination of haemoglobin S and hereditary persistence of fetal haemoglobin. They were predominantly of West Indian origin, more than half had been born in Britain, and most were aged under 25. The records for 304 patient admissions between 1962 and 1979 were analysed. There were 199 sickle-cell-disease-related admissions, 61 unrelated to sickle-cell disease, and 44 for pregnancy or its complications. Admissions per patient-year averaged less than one, except for children with Hb SS under the age of 5 years, who were admitted more frequently. The commonest reasons for admission were painful crises (74% of all admissions) and the "chest syndrome" (21%). There were four pneumococcal infections, all in children with Hb SS under the age of 8 years; all recovered. Three patients, aged 10, 15, and 50 years, died. The two children with Hb SS died in their sleep without gross evidence of sickling at necropsy. Multiple brain infarcts were found at necropsy in the 50-year-old woman with Hb SC who, having survived nine uneventful pregnancies, succumbed to an infection after cryosurgery to the cervix. Obstetric records were available for 18 term pregnancies in 11 women. Three antenatal sickling crises and three postpartum thromboembolic complications were encountered. There were no maternal or perinatal deaths. Fifteen asymptomatic individuals with sickle-cell disease were diagnosed as a result of routine screening procedures. There are likely to be many such individuals currently undiagnosed in the community. They urgently need identification because of their increased risks from pregnancy, surgery, and infection.

Adolescent↗
PubMed1980

Antithrombin III in patients on long-term oral anticoagulants.

The antithrombin III (AT III) concentration in plasma was measured in 63 patients on oral anticoagulant treatment (mean age 57.7 years), 26 healthy laboratory controls (mean age 28 years), and 21 patients attending the hypertensive clinic who had never been on oral anticoagulants (mean age 50 years). Three methods were used to measure AT III: a coagulation assay, a chromogenic substrate assay, and an immunological assay. In patients on oral anticoagulants, the mean values for AT III in the three assays were: 124%, 107%, and 96% respectively. The mean AT III concentration in laboratory staff was 103.4%, 94%, and 104.1% for the three assays; patients attending the hypertensive clinic had AT III concentrations indistinguishable from those in patients on oral anticoagulants: 117.9%, 110.5%, and 93.9%. The difference between both patient groups and laboratory staff was statistically highly significant, but no difference was demonstrated between patients on anticoagulant treatment and those not receiving it. Our results show that the increase in the functional AT III concentration (measured by coagulation and chromogenic assays) observed in patients on oral anticoagulants is probably due to the effects of age and underlying disease rather than to the anticoagulant treatment itself.

Adult↗