Generalized histiocytosis X in the elderly: a light and electron microscope and monoclonal antibody study

Two elderly female patients are described with generalized histiocytosis X (Letterer-Siwe disease). In each case, a definitive diagnosis was not established until ultrastructural and immunoperoxidase investigations had been performed. The histopathological findings in skin biopsies from each patient were similar. Light microscopy demonstrated a bandlike epidermotrophic cellular infiltrate which included large atypical cells (histiocytosis X cells). Electron microscopy showed that these cells contained Birbeck granules. Monoclonal antibody studies demonstrated the presence of T6, T4 and HLA-DR surface antigens. Lysozyme and alpha-I-antitrypsin were absent from the cells. The associated cellular infiltrate included T4 and T8 positive lymphocytes. It is possible that more cases of generalized histiocytosis X in adults will be identified with the increasing use of specialized histopathological techniques and that the disease is more common than currently believed.     

Substance P activation of bronchoalveolar macrophages from asthmatic patients and normal subjects

The role of substance P in the pathogenesis of asthma is unclear. Animal studies suggest that it may be important, whereas human studies do not confirm this. Alveolar macrophages can be recovered easily by bronchoalveolar lavage (BAL) and stimulated in vitro. To assess the role of substance P in humans, we tested its ability to stimulate alveolar macrophages from six normal subjects and seven asthmatic patients. BAL cells were separated by adherence and alveolar macrophages constituted 95% of the adherent cell population. Four concentrations of substance P were used (10(-7), 10(-6), 10(-5), 10(-4) M). To assess the non-specific activation of alveolar macrophages we used three concentrations of lipopolysaccharides (LPS) (5, 10, 20 micrograms/ml). The stimulation of alveolar macrophages was assessed by the release of thromboxane B2 by radioimmunoassay. This study indicates that alveolar macrophages are stimulated by LPS but are poorly activated or not at all by substance P.     

Right Ventricular Dysplasia: A Report of 24 Adult Cases

Right ventricular dysplasia is characterized by an abnormality in the development of part of the right ventricular musculature. Patients with right ventricular dysplasia may present with ventricular tachycardia, suprgventricular arrhythmias, right-heart failure or asymptomatic cardlomegaly. Twenty-two adult patients with right ventricular dysplasia who had recurrent ventricular tachycardia were seen during a 7-year period. The male/female ratio was 2.7:1. The mean age at the time of hospitalization was 39 years. All but one of the patients had ventricular tachycardia of a left bundle branch block configuration. With few exceptions, the T waves were inverted over the right precordial leads. The heart was usually enlarged and the pulmonary vasculaturc was usually normal. In six patients who had two-dimensional echocardiograms, all showed increased right ventricular diastolic dimensions. All patients had right ventricular angiography; the diagnosis of right ventricular dysplasia was substantiated during surgery in 12 patients and at autopsy in another. Two other patients who did not have arrhythmias had right ventricular dysplasia diagnosed by right- and left-heart angiography. Our unique experience, when combined with a literature review of 34 adult cases, permits a composite clinical profile of this condition in the adult.     

Tobacco smoking, an independent determinant for unhealthy diet?: A cross-sectional study of Norwegian workers on platforms in the North Sea

The objective of the study was to examine whether male tobaccosmokers had a more unhealthy diet than non-smokers. Data onsmoking and other variables were collected by questionnaireinterviews and food intake by one 24 h recall. The setting was2 oll platforms. The participants were 310 healthy men workingon 2 platforms in the Norwegian sector of the North Sea. Themean ages were 37 and 39 years for non-smokers and smokers.The prevalence of smoking was 50.3%. Smoking decreased withincreasing level of education, but increased with degree ofurbanization. Smokers consumed less vegetables, fruit and fish,but more meat, soft drinks containing sugar and more coffeethan non-smokers. Smokers had a higher intake of fat and a lowerintake of carbohydrate, vitamins A, D and C and dietary fibre.Smoking was an independent predictor for increasing the consumptionof coffee and high intake of energy, energy from fat and cholesteroland a decreasing consumption of vegetables, fruit, energy fromcarbohydrates, dietary fibre and vitamins A and C. It is concludedthat smokers had a more unhealthy diet than non-smokers.     

Congenital anomalies in children with cerebral palsy: a population‐based record linkage study

Aim Our aim was to determine the proportion of children with cerebral palsy (CP) who have a congenital anomaly (CA) in three regions (Isère Region, French Alps; Funen County, Denmark; Northern Region, England) where population‐based CP and CA registries exist, and to classify the children according to CA subtype. Method Data for children born between 1991 and 1999 were linked using electronic matching of cases. All potential matches were checked manually by each centre and verified as true matches. Results A total of 1104 children with CP were born during the study period (663 males, 441 females). Of these, 166 (15%; 95% Confidence Interval [CI] 13.0–17.3) children with CP had a CA: 8.8% had a cerebral anomaly, 4.8% had a non‐cerebral anomaly, and 1.4% had a non‐cerebral‐related syndrome or a chromosomal/genetic anomaly. Interpretation The prevalence of cerebral anomaly was highest in children with ataxic CP (41.7%) and lowest in those with dyskinetic CP (2.1%). Cerebral anomalies were found in 8.4% and 7% of children with bilateral and unilateral spastic CP respectively. The most frequent cerebral anomalies were primary microcephaly (26.5%) and congenital hydrocephalus (17.3%). The most common non‐cerebral anomalies recorded were cardiac (12.6% of children with CP and CA), urinary (5.4%), and musculoskeletal (5.4%). The prevalence of cerebral anomalies was higher among children born at term (13%) than among those born preterm (3.8%). Associated sensorineural or intellectual impairments occurred more often in children with CP and cerebral anomalies. We concluded that cerebral and non‐cerebral CA prevalence was higher among the CP population than in the general population of live births.     

Phonological development in very‐low‐birthweight children: an exploratory study

Aim Very‐low‐birthweight (VLBW; birthweight <1500g and/or gestational age <32wks) children are at risk for speech problems. However, there are few studies on speech development in VLBW children at an early age. The aim of this study was to investigate phonological development in 2‐year‐old VLBW children. Method Twenty VLBW children without major neurosensory impairment (7 males, 13 females; mean birthweight 971g, SD 315; mean gestational age 28wks, SD 1.81) and 20 term children (7 males, 13 females; mean birthweight 3503g, SD 416; mean gestational age 40wks, SD 1.26) were compared on measures of phonological development derived from 20‐minute spontaneous speech samples of standardized mother–child play interaction as well as on standardized tests of cognitive and psychomotor development, language, and behaviour. Results VLBW children had significantly fewer acquired consonants (median 9, p=0.02) and a significantly lower phonological mean length of utterance (pMLU; median 4.1, p<0.01) than term children (median acquired consonants 10, median pMLU 5.0). Interpretation This study provides evidence for poor phonological development in even healthy VLBW children, compared with term‐matched children, independent of their cognitive, psychomotor, and language development, and their behavioural functioning.     

EMOTIONS AND EATING IN EVERYDAY LIFE: APPLICATION OF THE EXPERIENCE-SAMPLING METHOD

Previous research emphasized relationships between negative emotions and eating. This study explored associations of both positive and negative emotions with eating by experience-sampling in the natural environment. Eight healthy volunteers (6 female, 2 male) were beeped at random intervals, 10 times daily on 7 consecutive days. Upon each signal, participants rated their emotional state and indicated whether they had eaten during the previous 15 minutes. In total, 485 situations were sampled. Emotional states reported in these situations were classified by cluster analysis. In 145 situations subjects reported having eaten. Thirty-seven percent of eating bouts occurred in situations classified as emotionally positive; 30% occurred in situations classified as emotionally negative. Participants rated that eating influenced their emotional state to a higher degree in emotionally positive situations than in emotionally negative ones. Positive emotions may play a stronger role in the relationship between emotions and eating than has been generally acknowledged.