Confidential enquiry into deaths due to prematurity.

The aim of this study was to audit the management of neonatal respiratory distress syndrome (RDS) in a geographically defined population using a retrospective peer review of case notes. The subjects were 49 infants of 24-36 wk gestation with a birthweight >499 g, and dying as a consequence of prematurity at <1 y of age in Wales during 1996. Forty-four infants (90%) were delivered in a unit with staff experienced in the management of preterm birth. Of the 30 infants <30 weeks' gestation, 29 (97%) received neonatal intensive care on a (sub)regional unit. Predelivery corticosteroids were indicated in 34 cases and administered in 31 (91%). Resuscitation at birth was indicated in 47 infants and conducted satisfactorily in 42 (89%). Temperature on admission to the neonatal unit was not recorded in 7 infants; in the other 42 it was >35.5 degrees C in 21 (50%). Early surfactant therapy was administered to 31/34 (91%) infants still intubated 120 min after birth, but was given within 30 min to only 8 (24%). Mechanical ventilation was assessed in 41 infants and considered to be good in 23 (56%). Cardiovascular therapy was evaluated in 40 infants requiring active support and considered to be good in 31 (78%). We concluded that neonatal RDS was generally well managed, thermal care during resuscitation was poor, surfactant should be administered more promptly, and deficiencies in the management of ventilation were common and related mainly to poor anticipation and a slow response to problems.

Depressive symptoms and self-concept in young people with spina bifida.

Examined self-reported depressive symptoms in 72 young people with spina bifida, ages 9 to 18:11, and matched able-bodied comparison subjects, using the Dimensions of Depression Profile for Children and Adolescents (Harter & Nowakowski, 1987). Independent variables included gender, self-perceptions (including physical appearance), and perceived social support. Young people with spina bifida were at greater risk of depressive mood, low self-worth, and suicidal ideation. Girls, independent of disability, were at greater risk of depressive mood, low self-worth, and self-blame. Multiple regression analyses suggest that global self-worth serves as a mediating variable for the effect of physical appearance self-concept on depressed mood (particularly in young people with spina bifida), and that perceived parental social support has a direct effect on depressed mood (particularly in girls).

Impact of functional severity on self concept in young people with spina bifida.

This study examines the relationship between medical and functional severity of disability and levels of self esteem and self concept in 79 young people with spina bifida. Greater feelings of global self worth and of self esteem in physical appearance were associated with greater severity of disability. This was only in part an effect of lower IQ among the most disabled young people. Many of the least disabled had marked impairment of self esteem. Analysis of the impact of individual aspects of disability confirmed the association between increased self esteem in physical appearance and global self worth, and diminished functional ability. Academic self ratings, however, were higher in the less disabled. Hydrocephalus and continence appeared to have minimal effect on self esteem. The relationship between severity of disability in spina bifida and self concept is complex and mediated by a range of factors. It is incorrect to assume that the psychological impact is less in the mildly disabled young person.

The self-concept of young people with spina bifida: a population-based study.

Seventy-nine young people with spina bifida were given a psychological, medical, carer and occupational therapy assessment. 79 matched able-bodied young people received the psychosocial interview. The disabled group felt themselves to be less competent in academic, athletic and social aspects of self-concept, less supported by classmates, equally supported by parents and friends and more supported by teachers than the able-bodied group. Disabled subjects did not discount the importance of any area of personal-social functioning, and experienced greater discrepancies between competence and importance in most academic, athletic, social and physical appearance aspects of self-concept. Disabled girls assigned very high importance to physical appearance. Physical appearance was more strongly associated with general self-esteem than any other area of self-concept.

Models of parent partnership and child development centres.

Four models (and an emergent fifth model) of parent partnership are described and their relevance to UK child development centres is explored. Clinical, management and service development issues are drawn out.

Does health education prevent childhood accidents?

In an attempt to test the assumption that health education directed at parents and children can reduce childhood accidents, a controlled study was carried out in Ely , one area of Cardiff. Using conventional health education techniques, the campaign was carried out in June and July 1981 and monitored by the numbers of injured children attending the local Accident and Emergency Department. Comparison of accident numbers in Ely between 1980 and 1981 and between Ely and the whole of Cardiff in 1981 showed no significant change. A slight increase in trivial injuries suggested an increased willingness to attend hospital. There was no change in the age distribution of victims. The benefits of health education and alternative methods of accident prevention are discussed and the need for further research is emphasized.

Febrile convulsions: electroencephalographic changes related to rectal temperature.

Nine children had simultaneous continuous electroencephalographic monitoring and temperature recording immediately after febrile convulsions. Background electroencephalographic rhythms did not alter with temperature and with one exception, seizure discharges occurred only during sustained high pyrexia.

Fatal infantile mitochondrial myopathy due to cytochrome c oxidase deficiency.

A case of cytochrome c oxidase deficiency primarily affecting skeletal muscle is described. The child was admitted at 4 weeks due to failure to thrive and examination at that time revealed weakness and hypotonia. His condition deteriorated until at 11 weeks respiratory arrest necessitated artificial ventilation and death occurred at 14 weeks. Biochemical investigation showed lactic acidaemia and generalised aminoaciduria. Histochemical examination of muscle obtained at biopsy showed strong reactions for some oxidative enzymes, but by contrast cytochrome c oxidase could not be detected. Cytochrome c oxidase activity was less than 5% of control values in an extract of fresh muscle. The reduced-minus oxidised absorption spectra of muscle mitochondrial fractions prepared from post-mortem tissue showed an absence of cytochrome aa3 and a partial deficiency of cytochrome b. Ultra-structural examination showed abnormal mitochondria with loss of cristae and an abnormal granular matrix. The family history suggests autosomal recessive inheritance.