D. M. Cooper

Directed neutrophil migration to IL-8 is increased in cystic fibrosis: a study of the effect of erythromycin

BACKGROUND The aim of this study was to compare neutrophil migration in cystic fibrosis (CF) and non-CF populations and to investigate the effect of erythromycin on directed migration of neutrophils (PMNs) in CF. METHODS PMNs were isolated and their migratory capacity in response to interleukin-8 (IL-8) or f-Met-Leu-Phe (fMLP) in the presence or absence of erythromycin (1–100 μg/ml) was assessed. RESULTS CF derived PMNs showed significantly increased migration to IL-8 but not to fMLP compared with non-CF PMNs. Erythromycin had no significant effect on migration responses to IL-8 and in vitro exposure of PMNs to erythromycin had no effect. CONCLUSIONS CF derived PMNs show higher migratory responsiveness to IL-8 but not to fMLP, suggesting that CF PMNs may be “primed” to IL-8 which is significantly increased in CF serum compared with non-CF serum. Treatment with erythromycin had no significant effect on PMN migration in vitro.

Parental asthma knowledge: its association with readmission of children to hospital.

Objectives: To assess whether poor parental knowledge about asthma was a risk factor for readmission of their children to hospital.

Respiratory and body movements as indicators of sleep stage and wakefulness in infants and young children.

Conventional polysomnographic (PSG) sleep staging to sleep staging based on a static‐charge‐sensitive bed (SCSB) recording in infants and young children was compared. The study consisted of whole‐night clinical sleep studies in 22 children at 24 weeks (SD 24, range 1–79 weeks) of age. Most of the children presented with respiratory disturbances during sleep. From the SCSB record, sleep stages were differentiated according to regularity of breathing, presence of body movements, and most important, presence of high‐frequency components of breathing (SCSB spikes). With both methods, three sleep/wake stages were distinguished: rapid eye movement (REM) sleep, non‐rapid eye movement (NREM) sleep and wakefulness. The average inter‐scorer reliability of the PSG sleep staging controlled in nine subjects was 88%. The average concordance between the two methods ranged from 82 to 85%, depending on the criteria used for scoring the SCSB. The mean sensitivity of the SCSB to detect NREM sleep ranged from 77 to 90% and the mean sensitivity to detect REM sleep ranged from 61 to 86%. The mean positive predictive value was 89–96% for NREM sleep and 54–67% for REM sleep. In conclusion, REM sleep is characterized by irregular breathing with superimposed fast respiratory movements. These changes are specific enough to allow distinction between episodes of NREM sleep, REM sleep and wakefulness with the non‐invasive SCSB method in infants and young children. Incomplete concordance between PSG and SCSB score was most frequently observed during sleep stage transition periods, where the behavioural state and electrophysiological criteria disagreed. When combined with the PSG, the SCSB provides complementary information about the behavioural state of child.

Hydroxychloroquine therapy of diffuse pulmonary sarcoidosis in two Australian male children

Abstract Sarcoidosis is a chronic granulomatous disease, seen infrequently in children,1 with the capacity to affect multiple systems. The incidence and prevalence of the disease in children is largely unknown but is dependent on many factors including: age, race and geographical location.2 There have been very few studies of sarcoidosis in Australian (Aboriginal or white) children. Diffuse pulmonary sarcoidosis is very rare in non‐Scandinavian white Caucasian children, yet in the winter of 1992 two Caucasian males, aged 11.1 and 10.5 years were seen at the John Hunter Hospital in Newcastle. Both boys had pulmonary sarcoidosis and after an initial trial of treatment with steroids responded well to hydroxychloroquine therapy.

Genotype of the cystic fibrosis population of the Hunter Region of New South Wales

OBJECTIVE To determine the genotype of patients attending the cystic fibrosis clinic at John Hunter Hospital, Newcastle, Australia. METHODOLOGY Seventy-five of the 76 patients attending the clinic over a 6 month period had blood collected for genetic analysis of 17 of the cystic fibrosis (CF) gene mutations. RESULTS Sixty-one per cent of the patients were homozygous for the delta F508 mutation and all except one child had at least one delta F508 mutation. DISCUSSION Nearly 80% of the CF genes were the delta F508 mutation. This prevalence suggests that the obligatory false negative rate of a newborn screening programme for CF based on a combination of immunoreactive trypsin and the delta F508 gene may be as low as 4-5%.