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Biomedical subjects

J Pearn

Publications and source records attributed to J Pearn.

At least 127 records · Page 7Linked to original sources
PubMed1980

Classification of spinal muscular atrophies.

Clinical heterogeneity within the spinal muscular atrophies (SMA) has long been a source of confusion for questions of prognosis and genetic counselling. Comprehensive clinical and genetic analyses of 240 consecutive index cases from two English centres (The English SMA Study) have enabled some nosological questions to be resolved. The different SMA syndromes can be discriminated by (a) age at the first clinical signs of the disease, (b) pattern of muscle involvement, (c) age at death of other patients within an affected kindred, and (d) genetic evidence. Seven different SMA syndromes can be defined clinically and genetically; thirteen mutant genes are incriminated. Prevalence and incidence figures are presented. SMA type I (Werdnig-Hoffman disease) and chronic childhood SMA together comprise 74% of all SMA cases. The classification of the spinal muscular atrophies presented also provides the differential diagnosis for newly presenting cases.

Adolescent↗
PubMed1980

Dog bite injuries to children: potential rabies threat to Australia.

A consecutive series of 800 mammalian bites is reported from The Canberra Hospital. Of these, 66% were dog bites; 119 children were bitten by dogs (requiring hospital attention) over a 30-month period, giving an average dog-bite rate for the total population of 184 per 100,000 per year. One dog in 38 causes an injury requiring hospital attention. Thirty-seven per cent of the 119 children were bitten on the face and head, the modal age for this type of injury being three years. Ten per cent of cases required sutures. Each year, one person in 540 suffers from a dog-bite requiring hospital attention. If rabies became endemic, 26,000 antirabies immunization regimens would have to be undertaken annually for dog-bite alone, and a total of 38,000 annually for mammalian bites of all types.

Animals↗
PubMed1980

Who accepts first aid training?

The percentage of individuals trained in first aid skills in the general community is inadequate. We report here a study to investigate factors which influence motivation to accept voluntary training in first aid. A group of 700 randomly selected owners of inground swimming pools (a parental high-risk group) was offered a course of formal first aid instruction. Nine per cent attended the offered training course. The time commitment involved in traditional courses (eight training nights spread over four weeks) is not a deterrent, the same percentage accepting such courses as that who accept a course of one night's instruction. Cost is an important deterrent factor, consumer resistance rising over 15 cost units (one cost unit = the price of a loaf of bread). The level of competent first aid training within the community can be raised by (a) keeping to traditional course content, but (b) by ensuring a higher acceptance rate of first aid courses by a new approach to publicity campaigns, to convince prospective students of the real worth of first aid training. Questions concerning who should be taught first aid, and factors influencing motivation, are discussed.

Adult↗
PubMed1979

Bee-sting anaphylaxis in childhood.

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.

Aerosols↗
PubMed1979

Oleander poisoning.

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.

Antidotes↗
PubMed1979

Saltwater drowning and near-drowning accidents involving children. A five-year total population study in south-east Queensland.

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.

Accidents↗
PubMed1979

Blood selenium in chronic spinal muscular atrophy.

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.

Adolescent↗
PubMed1979

Distal spinal muscular atrophy. A clinical and genetic study of 8 kindreds.

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.

Adolescent↗
PubMed1978

Drowning risks to epileptic children: a study from Hawaii.

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.

Adolescent↗
PubMed1978

"Ring the bell and win a cigar". Some early experiments on the measurement of human strength in Port Jackson and Van Diemen's Land.

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.

Anthropometry↗
PubMed1978

Anterior-horn cell degeneration and gross calf hypertrophy with adolescent onset. A new spinal muscular atrophy syndrome.

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.

Adolescent↗