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, …

To estimate the prevalence of gout and to examine its risk factors among Taiwan's Aborigines compared with non-Aborigines.Data were collected from persons older than 40 years living in 3 aboriginal and 2 non-aboriginal districts in Taiwan by a community survey. Cases of gout were identified from self-reporting of a doctor's diagnosis based on clinical criteria. Baseline variables and biochemical data were examined as risk factors for the development of gout.The prevalence of gout history was found to be 15.2% (25/165) and 4.8% (11/231) among aboriginal men and women, respectively, compared with a prevalence rate of 0.3% among non-Aborigines. A logistic regression model showed that aboriginal men older than 60 years with hyperuricemia were more severely affected by gout than any other group (p0.05).A high prevalence of gout among Taiwanese Aborigines was observed in this study and race was the most significant risk factor associated with the disease.

Inversion of chromosome 16, inv(16)(p13q22), is characteristic of acute myeloid leukaemia (AML) with eosinophilia and is rarely found in the myelodysplastic syndrome (MDS). We report three cases of MDS in which inv(16) was observed. They were classified to FAB subtypes RA, RARS and RAEBT; eosinophilia or abnormal eosinophils were not observed. The disease appeared to be stable in all three patients. MDS with inv(16) without eosinophilia may be a rare subgroup associated with a good prognosis.

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.

A 34-year-old patient was transplanted from an HLA-identical sister for high grade non-Hodgkin's lymphoma in first complete remission. One month post-transplant, he developed hepatitis and haemorrhagic cystitis. He died 2 months post-transplant from fulminant hepatic failure. Adenovirus type 5 was cultured from urine, and characteristic adenovirus inclusions were seen in the liver. Striking paracrystalline arrays of adenoviruses were seen in the liver on electron microscopy. Reactivation of adenovirus infection is increasingly recognized post-BMT, but this complication of type 5 infection is unusual, and we describe in detail this second reported case.

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.

A number of factors were identified which influence the determination of red blood cell glucose 6-phosphate dehydrogenase (G6PD). White blood cells were effectively removed from whole blood by filtration, resulting in a mean reduction of G6PD activity of greater than 1.0 U/g Hb. Optimal enzyme activity was found using haemolysates with a Hb concentration in the range 0.4-1.2 g/dl. At a constant temperature of 30 degrees C, maximum linear rate of activity was achieved between the 10th and 20th min after initiation of the reaction. As a result of these findings, modifications to standardize the assay system were introduced. The new method exhibits excellent precision (CV less than 2.0%) with normal samples, and 95% reference ranges of 7.00-11.15 U/g Hb for adult females and 7.05-10.75 U/g Hb for adult males.

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

The ratios of the diameter of NaCl solution droplets to the diameter of NaCl particles from which the droplets have grown at various humidities are computed. Graphs of the ratio versus particle diameter at various relative humidities and the ratio versus the relative humidity at various particle diameters are presented and discussed.