Search

Objective To define the incidence, risk factors and complications of priapism in a large population of patients with sickle-cell anaemia in five centres in the UK and Nigeria, as priapism is common among these patients, but the precise characteristics of the condition in this population are poorly documented. Patients and methods A questionnaire was developed and administered to patients with sickle-cell disease. Questions were designed to define the incidence, nature, precipitants, duration, treatment and complications of priapism. A distinction was made between acute (severe) priapism and the recurrent, ‘stuttering’ type. Results The questionnaire was completed by 130 patients (mean age 25 years, sd 11, range 4–66) from the five centres; 102 (78%) were homozygous Hb SS genotype, 19 (15%) were Hb SC genotype and two (1.5%) were Hb Sα−thalassaemia. Of the patients, 46 (35%) reported a history of priapism, and of these, 33 (72%) had a history of stuttering priapism, while 24 (52%) had had an acute episode of priapism. The mean age of onset of priapism was 15 years, with 75% of patients having the first episode before their 20th birthday. Sexual activity was the most frequent precipitating factor, with fever and/or dehydration being the next most common. Of the 46 patients, 10 (21%) with a history of priapism reported having erectile dysfunction. A similar proportion reported dissatisfaction with sexual intercourse, including a fear of engaging in sexual activity. Conclusion The incidence of priapism among patients with sickle-cell anaemia is high (35%). The implications of priapism for erectile and sexual function are significant and documented in this large series. The treatment of this condition in these patients remains unstandardised. This study highlights the need for an increased awareness of the problems associated with priapism among patients, families and medical professionals.

The objective of this study was to determine the frequency of ethnic groups within the antenatal population in central Manchester and thereby ensure that the haemoglobinopathy service was targeting the correct population and their needs. Ethnic data collection records of 6 718 patients were analysed over a 7 month period. Of these 62,3% stated that they were White. 1 3.2% Asian. 7.9% Black, 3.8% Chinese or 'other ethnic groups' and 12. 7% gave no information about their ethnic background. A subset of 1144 patients were screened for haemoglobinopathies over a 1 month period. The incidence of haemoglobinopathies within the screened population was 2.62%, and comprised 0.69% beta thalassaemia trait, 1.22% sickle cell trait, 0.43% haemoglobin C trait and 0.26% haemoglobin D trait. The total incidence of haemoglobinopathies was highest within the Black population (1 8.2%), followed by the no information group (5. 6%), Asian (3,35%) and white (0.26%), The high proportion of ethnic minorities and the significant carrier frequency in the no information group. support our view that non-selective screening should be offered to the antenatal population of central Manchester.

We have collected data on the cancer deaths of children and adolescents 0-19 yr old living in a residential area near 3 large petroleum and petrochemical complexes in and near Kaohsiung city (petrochemical industrial districts, PIDs) in the period of 1971-1990 and compared these with the cancer deaths of children and adolescents 0-19 yr old among the entire population of Taiwan (national reference) and among the residents of 26 administrative districts, comprising all of Kaohsiung city and Kaohsiung county (local reference), except for 8 sparsely populated, rural districts. Having scrutinized all cancer death certificates, we have identified various statistically significant excess deaths, as compared with the national and local reference, due to cancers at all sites. Cancer of the bone, brain, and bladder in boys and girls 0-9 yr and 10-19 yr of age in the 1981-1990 decade that followed the establishment of petrochemical production in the PIDs was studied. However, excess cancer deaths seemed to have clustered in the 10-19 yr age group, who had been potentially exposed to the petrochemical pollutants for the longest period of time from the youngest age. Almost all bone, brain, and bladder cancer deaths registered were within 3 km of the 3 complexes. Bone and brain cancers in particular occurred in girls in the PIDs more frequently than in boys, even though these are believed to occur more in males than females elsewhere.

EDITOR—We welcome the launch of a European register of patients with sickle cell disease treated with hydroxyurea1 and will certainly offer anonymised details of our treated patients. However, Davies and Roberts-Harwood would be the first to agree that a register collecting data retrospectively cannot address research questions in the way that formal prospective controlled studies can. We agree that the results of Charache et al's study of hydroxyurea2 were convincing. However, there is wide clinical heterogeneity in this condition, …

After reports of the successful use of mithramycin and hydroxyurea in the myeloid blast phase of chronic granulocytic leukemia, we treated nine patients according to the protocol devised by Koller and Miller (1986). There were no complete responses, but one patient had a partial response with a transient return to the chronic phase. Of the remaining eight patients, two experienced lessening of bone pains, and one a reduction in spleen size, but without hematological improvement. The regimen was associated with significant toxicity, and no overall survival advantage. We present a review of published data regarding the use of mithramycin in chronic granulocytic leukemia which supports the results in our series. The combination of mithramycin and hydroxyurea is largely ineffective in the blast phase of chronic granulocytic leukemia, but may be of value in the accelerated phase.

Sickle cell disease is the commonest inherited haemoglobinopathy in Britain and affects over 9000 people.1 Clinical severity varies considerably, but patients with the most severe disease have a life expectancy of just over 40 years. Conventional management of the disease is largely supportive, highlighting a pressing need for approaches that can alter the course of the disease. Trials in America have suggested that hydroxyurea can have a significant impact on the course of the disease, but in Britain it is proving virtually impossible to recruit patients into trials to confirm these results in a British population. The fact that the clinical severity of the sickle cell disease varies even within groups of patients with the same β globin genotype2 has led to the concept that the disease is a multigene disorder, with inheritance of α thalassemia and genes controlling the concentration of fetal haemoglobin, among others, modulating disease expression. …

A 28 year old patient with primary thrombocythaemia, who had had two stillbirths in the third trimester, is reported. She was successfully treated with hydroxyurea and delivered a healthy 6lb boy by elective caesarean section. The ease with which this treatment can be given, its high level of tolerance among patients, and its low cost are likely to increase its use as a safe and effective treatment in pregnant patients with primary thrombocythaemia.

Routine full blood counts and serum ferritin determinations were carried out after admission to hospital in 112 children which included a white caucasian group (n = 65) and two ethnic minority groups of West Indians (n = 24) and Asians (n = 23). In these 3 groups those children between the ages of 1 week and 6 months were found to have similar haemoglobin, mean corpuscular volume, and ferritin levels. In the remaining children (aged from 7 months to 14 years 5 months) serum ferritin levels were lower in the ethnic minority groups than in white caucasians, but the haemoglobin and mean corpuscular volume were not significantly different. Children with lower height centiles had reduced ferritin levels, irrespective of their ethnic origins. A nutritional survey between the ages of 7 months and 14 years 5 months showed that mean daily dietary intakes of energy, protein, and iron in white caucasions were similar to those in West Indian and Asian children. The differences noted were in larger phytate and fibre intakes in the ethnic minority groups. Asian diets appeared to differ in containing meat less often as a source of iron, while pulses and chapattis provided more phytate and fibre. It is suggested that dietary intakes of phytate and fibre are important in causing lower ferritin levels by reducing iron absorption.

The natural history of 45 cases of smouldering leukaemia has been studied. Males and females were equally represented, with a median age of 60.5. The median survival of the whole group was only 20 months, but rare cases lived 10 years or longer. 38% developed acute leukaemia; the remainder usually died of the results of marrow failure. Although it was possible to divide these marrow dysplasias morphologically into 3 major subgroups (refractory anaemia with excess of myeloblasts, chronic myelomonocytic leukaemia and chronic erythraemic myelosis), several displayed transitional features. Many showed refractory macrocytosis at diagnosis. The survival of the 3 groups was similar, though patients with high monocyte counts tended to present with less anaemia and fared rather better than the others. Statistical analysis suggests that increasing age, severe anaemia, thrombocytopenia and hepatomegaly are associated with a poor prognosis. Chemotherapy, when attempted, was usually unsuccessful.

During immersion in water to the neck, seven seated resting normal subjects showed, without exception in 14 trials, an increase in diffusing capacity of the lung (DlCO) which averaged 16.2 ± 0.79 sd % of the control (unimmersed) values (P < 0.001). At an intermediate depth of immersion at which the calculated hydrostatic pressure (gauge) was approximately halved, the rise in DlCO was also halved. The hemodynamic readjustment to external pressure was completed within a few minutes, since no further change in DlCO occurred during continuous immersion to the neck for as long as 90 min. Immersion produced a rise in “permeability” of the lung (Kco) which was on the average 5.8% greater than that in DlCO. In three subjects the pulmonary capillary blood volume (Vc) rose on the average 47% at the deeper level of immersion, suggesting that, as in the pressure suit, the rise in DlCO was due to pulmonary vascular engorgement. pulmonary vascular engorgement Submitted on July 6, 1964

One hundred and sixty two people working in various departments of cotton spinning and weaving mills measured and recorded their own peak expiratory flow rate (PEFR) at two hourly intervals during Monday, Wednesday, and Thursday of the same work week, from waking in the morning throughout the day until going to bed and a last time the following morning after waking. The circadian rhythm in PEFR was computed by the Halberg program. The mean amplitude of the rhythm in the group was found to be 3.3% and the acrophase fell approximately in the middle of the waking hours. Older workers and those claiming to suffer from symptoms of chronic bronchitis were found to have an amplitude significantly higher (4.1% and 3.9% respectively) than their younger or symptom free counterparts (2.6% and 2.9% respectively; p less than 0.03). The amplitude of cardroom workers (2.4%), workers with byssinosis (2.7%), and those with much exposure to airborne cotton dust (3.3%) and bacteria (2.9%) tended to be lower than that of less exposed groups such as office staff (3.9%), though the difference was significant only in the case of cardroom workers (p less than 0.04). This may be due to airborne contaminants in the working environment.

A respiratory symptoms questionnaire was completed for 4656 volunteers employed in 31 textile factories engaged in spinning or weaving manmade fibre or cotton of various qualities. Sets of airborne dust and bacteria samples were collected in workzones and personal breathing zones in the workrooms where the volunteers were employed. A total of 182 people indicated experiencing byssinotic symptoms, mainly in opening and carding rooms or in spinning and winding rooms where medium to coarse cotton was being processed. This represents a significant decline in the prevalence of byssinotic symptoms over the years, due possibly to lower concentrations of airborne contaminants, especially of bacteria, as cleaner raw materials are being used. According to a multiple, logistic regression model, the prevalence of byssinotic symptoms was found to be statistically significantly related to years worked in the cotton industry, exposure to dust, quality of cotton used, workroom of employment, ethnic origin, and smoking habits. Symptoms of chronic bronchitis were found to be significantly related to smoking habit and to factors connected with occupation, such as exposure to dust, workroom, and the quality of fibre processed.

The concentration of airborne microbes, their endotoxins and the prevalence of byssinotic symptoms among workers were measured in the cardrooms of seven cotton spinning, a wool spinning and two cotton waste mills and in a dusty workroom of a group of five willowing mills, a tea-packing plant and a pipe tobacco factory. The concentration of microbes cultured on endoagar plates were found to correlate with byssinosis prevalence (r greater than 0.95, P less than 0.001). The concentration of nutrient agar microbes correlated with prevalence less well (r greater than 0.77, P less than 0.01) and that of fungi and endotoxins not at all.