Child sexual abuse. Intervention programs and the protection of children at risk.
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Biomedical subjects
Publications and source records attributed to J Pearn.
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Accidental, homicidal and suicidal drowning comprise a special challenge to the clinician and preventive medicine advocate, alike. In South-east Asia and Australasia, accidental immersion accidents rank highly among the causes of preventable child trauma. Bath-tub and bucket drownings affect infants and toddlers under the age of 12 months, and some 10 percent of fatal bucket-tub immersions affecting infants are the result of child abuse. Immersion accidents in the sea have special characteristics, not specifically as a result of differences in water osmolarity, but related to hypothermia, secondary lung complications, and immersion times. Swimming pool drownings are the major cause of preventable death affecting pre-school children in some regions of Australasia. Resuscitation of the near-drowned child is topical because, (a) of controversies about the optimality of mouth-to-nose expired air resuscitation (EAR) in infants under six months of age; (b) of controversies about the degree of brain damage among child survivors following intensive care salvage; and (c) the difficulties of having "every parent a first-aider". A major study of childhood immersions (The Brisbane Drowning Study has shown that of all survivors, some 70 percent will be completely normal, 30 percent will suffer some selective deficit (with wide disparities on sub-scale scores on formal IQ testing), and 3 percent will live in a permanent vegetative state. Vigorous preventative campaigns using the triad of (a) public media education and campaigns, (b) better safety standards and safety devices, and (c) safety legislation, can reduce both the population risk and the individual clinical severity of immersion accidents.(ABSTRACT TRUNCATED AT 250 WORDS)
The syndrome of bee-sting anaphylaxis is described. Children who have suffered crescendo reactions to previous bee stings, especially children with a history of asthma, are significantly at risk. Desensitization is required in such cases; adrenaline should be kept in the home, and parents should be trained in its emergency use. The immunological mechanisms of bee-sting anaphylaxis are described. The striking seasonal incidence of anaphylaxis suggests that pollen or plant products which are incorporated in the venom may also be important in its genesis; it suggests also that antigens prepared from either whole-body or pure-venom extracts should be prepared from bees which are collected in late spring.
The two common oleanders, Thevetia peruviana and Nerium oleander, contain a mixture of poisons including cardiac glycosides, and are extremely toxic. They are cultivated universally throughout Australia, and rank equally with mushrooms as the major cause of children's admission to hospital after accidental plant ingestions. A seven-year total population survey from south-east Queensland has revealed that, in practice, the rate of clinical poisoning due to oleander is inconsequential, and mortality is negligible. The annual age-specific admission rate for children (aged from birth to 12 years of age), for all plant ingestions is 2.33 per 100 000, and 0.62 per 100 000 specifically for oleander. Oleander ingestion causes a syndrome of combine cardiac and gastrointestinal symptoms and signs. A case series of 13 children is described, and the clinical features summarized. After accidental oleander ingestion, current experience indicates that the prognosis is excellent.
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A large total population study of childhood saltwater immersion accidents is reported. A total of 49 cases (16 fatalities, 33 survivors) occurred in the five year period from 1971 to 1975 in southeastern Queensland. As a group, more children survive a potentially fatal saltwater immersion (67%) than do those who lose consciousness in freshwater (50%). The serious saltwater accident rate (loss of consciousness or death) in childhood (from 0 to 15 years inclusive, is 3.37/100,000 children per year at risk (fatality rate 1.12). This is low; comparison with freshwater data shows that although the surf presents special hazards to children, it is very much safer than other types of water. Age-specific and site-specific accident and survival rates for saltwater immersions are presented for the first time. Toddlers are disproportionately represented (33% of all children) and their survival rates are lowest. Boating and the use of surfboards, in current practice, are negligible threats to children. The saltwater immersion rate is increasing (although the absolute risk is small) and reasons for this are discussed. Childhood saltwater immersions were unaffected by tidal state. All but one case of immersion occurred during daylight hours, and in younger children immersion occurred often on weekends.
The spinal muscular atrophies (SMA) of childhood comprise the second most common fatal recessive disease after cystic fibrosis, yet the nature of the biochemical defect causing the anterior horn cell degeneration is totally unknown. Recent reports of a cluster of adult motor neurone disease cases from a high seleniferous area in South Dakota have prompted the study of blood selenium in children with SMA in Australia. Eight children with chronic SMA were tested, in addition to 9 obligate heterozygote carriers of the gene. Blood selenium levels of patients and carriers did not differ significantly from that observed in controls. The mammalian effects of selenium toxicity are discussed.
Twelve patients (8 kindreds) with distal SMA are described, and an analysis presented of their clinical and genetic features. Distal SMA accounted for 10% of all patients with SMA in a total population survey of this disease in North-East England. The parental consanguinity rate is high, occurring in 3 of the 8 kindreds reported; the sex ratio was 1.0; the segregation ratio of sibs did not differ from 0.25. Intrafamilial concordance for clinical features of the disease is high. This current data is consistent with a suggested aetiology of two separate autosomal recessive genes. Clinical features are discussed and a review of the literature presented. The disease is only slowly progressive, but one of the genetic types may present with infantile or early juvenile onset; there is no evidence that it shortens life. 50% of cases did not have a normal gait after 4 years of age; 50% could not run after 17 years of age; and 50% could not walk unaided after 28 years of age. Details of prognosis, and principles of genetic counselling in this disease are discussed.
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The role of epileptiform seizures in causing drowning and near-drowning among children was studied by examining the case reports of all 140 childhood immersion accidents that occurred in an area of Hawaii over five years. Four of the 140 immersion accidents were caused partly by epileptiform seizures, but none were fatal. The combined results of the Hawaiian and Brisbane studies (total population studied over five years 1 600 000) showed that no epileptic children died from accidents in the sea or in swimming pools; and the 2.9% incidence of immersion accidents due to seizures in the Hawaiian study compares well with the incidence found in other series. If an epileptic child is mentally normal, well controlled with anticonvulsants, and supervised in the water then the risk of drowning is very small.
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The first medical research formally undertaken on Australian soil was a series of experiments to test different factors which might modify muscular power. The experiments were conducted by François Péron, a Parisian doctor and naturalist, who travelled with Baudin's French scientific expedition to Nouvelle Hollande and Terre de Diemen. Péron used a dynamometer, an instrument for measuring strength. He conducted his experiments at Port Jackson, and at Maria Island (in Van Diemen's Land) in 1902. The basis for the experiments, an account of the equipment used, and the results are presented together for the first time.
A new variant of spinal muscular atrophy (S.M.A.), characterised by adolescent onset, gross hypertrophy of calves, and a slowly progressive clinical course, was found in 5 patients, 3 of them in a series of 102 cases being studied in North-East England. Biopsy and electrophysiological studies indicated the presence of chronic progressive degeneration of anterior-horn cells.
A clinical and genetic study of 6 kindreds (13 patients) with autosomal dominant spinal muscular atrophy is presented. Evidence is presented to indicate that two separate autosomal dominant genes are involved. One of these causes clinical disease with onset in early childhood (birth--8 years), which is relatively benign and in which proximal selectively of muscle involvement is not marked. A separate autosomal dominant gene causes a disease with onset in adult life (median age 37 years), showing marked initial proximal selectively; this disease may be more rapid in its clinical progression. Penetrance of both genes approaches 100%. Incidence figures are presented; less than 2% of all cases of childhood onset spinal muscular atrophy, but 30% of adult onset cases, are due to an autosomal dominant gene transmitted from an affected parent. Implications for prognosis, diagnosis and genetic counselling are discussed. A review of 11 kindreds of dominant spinal muscular atrophy in the literature is presented.
A genetic study of the subacute spinal muscular atrophies (SMA) of late infancy and early childhood has been undertaken. All such patients with chronic disease (with ages at onset up to 14 years, and excluding SMA Type I) known to 2 large Neurological Centres were reassessed clinically and genetically. There were 124 index patients (67 females and 57 males) and 17 secondary cases, which formed two consecutive unselected series. To investigate the genetic composition of this group, 4 nosological approaches were used; cluster analysis of clinical features of the disease, Haldane's sib-sib analysis on familial cases, interpretation of frequency distribution histograms, and a segregation analysis. A single autosomal recessive gene accounts for over 90% of cases, causes a clinical syndrome which manifests its first clinical signs before 5 years of age and in almost all cases before two years of age, but which is compatible with life into the third decade. Moderate intrafamilial discordance for some clinical features may be observed, but no genetic heterogeneity within this group was demonstrated. A small group of cases is caused by (a) new dominant mutation(s), or (b) is composed of phenocopies, or both. This relatively uncommon form may comprise the majority of late-presenting cases, and may account for all cases which manifest the first signs after 5 years of age. The spectrum of age-at-onset of this group cannot be determined at present, but the disease may be manifest before the age of two years; it is clinically indistinguishable from SMA caused by an autosomal recessive gene. The literature has been reviewed in the light of these findings. Empirical risks for use in genetic counselling are presented.