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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.

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.

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.

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.

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

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 concentrations of airborne dust and bacteria were determined in 12 flax scutching mills and in two milk processing plants in Normandy, France. A total of 308 of 340 flax workers and 111 of 113 milk processors volunteered to answer a respiratory questionnaire. Personal exposure to airborne dust in the scutching mills varied from 22.2 mg/m3 to 144 mg/m3 and areal concentrations from 8.92 mg/m3 to 47.1 mg/m3. The concentration of Gram negative bacteria ranged from 3970 (colony forming units) cfu/m3 to 67,900 cfu/m3 and that of total bacteria from 12,900 cfu/m3 to more than 600,000 cfu/m3. In all, 20% of the flax scutchers were found, on the basis of the questionnaire, to suffer from persistent cough and 25% from chronic phlegm production. The corresponding figures among milk processors were 3.6% and 4.5%. Unexpectedly, only 12.5% of the scutchers appeared to suffer from byssinotic symptoms even though they were heavily exposed to airborne dust and bacteria. The low prevalence of byssinosis might be due to self selection of the workforce or a relatively low concentration of the causative agent despite high airborne contamination.