Interpreting genetic risks.

Prof. Peter Beighton has given a professional lifetime to helping patients and their families who have been afflicted by inherited disease. His clinical skills have brought certainty, confidence and support to those confronted with some of the most difficult decisions in life's progress. Prof. Beighton's research has led to the discovery of new syndromes and the elucidation of accurate genetic risks in many diseases. This in turn has empowered patients and their families to make informed decisions and has provided doctors with the scientific knowledge to help patients. On the occasion of this festschrift, I join with so many members of Peter's international professional family to pay tribute to his leadership and service - not only in medical genetics - but also in the broadest domains of healthcare.

Dr William Wilson Ingram (1888-1982): doctor-soldier, physician and Antarctic expeditioner.

Dr William Wilson Ingram (1888-1982), a Scottish-born physician, contributed significantly to the health and heritage of Australia, his adopted land. Born on Speyside and educated in Aberdeen, he was a doctor-soldier in two World Wars and decorated with the Military Cross. Ingram was a Foundation Fellow (1938) of the Royal Australasian College of Physicians and established one of the first specialist diabetic clinics in Australia, in Sydney in 1928. As an arachnologist, he published clinical descriptions of both surviving and fatal cases of envenomation by the Sydney funnel web spider, Atrax robustus. He founded the Kolling Institute of Medical Research at the Royal North Shore Hospital in Sydney where for two generations he was a leader in both undergraduate and postgraduate medical education. The international significance of his life's work relates to his service as the medical officer and biologist on the two British, Australian and New Zealand Antarctic Research Expeditions (BANZARE) of 1929-1931, for which service he was awarded the Polar Medal and subsequent Clasp. Those expeditions secured, for the British Crown, what was to become the Australian Antarctic Territory, ceded to Australia by a British Order in Council of 24 August 1936. Sir Douglas Mawson, polar expeditioner and the leader of BANZARE, described Ingram as 'an ideal medical officer', one who in addition to his clinical skills and judgement, manifested courage and 'physical endurance and a full measure of camaraderie'. Ingram has no published obituary or biography. This précis records some details of his extraordinary life.

Enduring symbols of dentistry: international metaphors of dental science.

Dentists' contributions to science and society extend beyond the practice of clinical dentistry and preventive oral health. Such service encompasses contributions to biology specifically and more generally to societal good works for which dentists are particularly esteemed. The profession of dentistry promotes the history and heritage of its craft and those who practise it. Enduring symbols of dentistry take many forms. These include metonymic emblems such as those of Cadmus and Saint Apollonia and the portrayal of effigies of twentieth century dentists on eponymous medals. Other enduring symbols are to be found in the names of streets and towns (eg Normanville in Australia) which commemorate dentists; and in the worldwide scientific names of plants and animal species which perpetuate singular contributions of dentists to biological science. Such include the scientific names of grasses (Deyeuxia rodwayi) after the Tasmanian dentist, Dr Leonard Rodway (1853-1936); seaweeds (Jeannerettia sp.) after a seaweed of the Pacific and Southern Oceans, after Dr Henry Jeannerett (1802-1886); gastropods (Typhina yatesi) after Dr Lorenzo Yates (1837-1909); copepods (eg Mimocalanus heronae) named to commemorate the life and works of Gayle Ann Heron (b.1923), a dental hygienist of the University of Washington, USA; and crabs (eg Cancer bellianus) named to commemorate the life and works of the leading British dentist of his day, Thomas Bell (1792-1880). This paper explores this theme of the creation and promotion of enduring symbols of dental science - enshrined in the civic, numismatic and taxonomic record.

Baden-Powell on teeth: a centenary perspective of a pioneer of preventive dental health.

In the era when dental care, particularly preventive dental health, did not enjoy a high public profile, Lieut-General (later Lord) Robert Baden-Powell (1857-1941) was an influential advocate for the care of the teeth. He was a pioneer in a targeted outreach to youth, specifically boys and young men, emphasising the importance of dental health as an essential part of total body health and fitness. In his book, Scouting for boys, first published on 1 May 1908, he described personal accounts of the consequences of the neglect of oral hygiene and presented advice on how to make an effective 'camp tooth-brush' in order that dental hygiene would not be compromised even under the exigencies of conditions away from home. Baden-Powell wrote explicitly that daily dental hygiene was the single most important 'one civilised thing [teenage youths] could do', irrespective of one's physical circumstances. Scouting for boys was for more than five decades the world's best seller in English, after the Bible. It has run to, and now surpasses, 60 million copies in 30 languages and has been published in 35 editions. It is believed that Baden-Powell's frank and direct exhortations to preserve the teeth, with simple and direct advice on food and what today would be called oral hygiene, have been read by 350 million people throughout the world. His advocacy reached out to boys and young men as it does today to youths of both sexes in that 'window of opportunity' when life-long habits of healthcare are being inculcated and when important components of secondary dentition are forming. This paper is a centenary perspective of Baden-Powell's pioneering advocacy of modern preventive dental health.

Idiopathic necrotizing dermatitis: current management.

OBJECTIVES: To identify and demonstrate necrotizing dermatitis in infancy; an uncommon, puzzling syndrome, in which anecdotal reporting and personal experience indicates that one third of cases may require skin grafting. Much informed discussion about the pathogenesis of this distressing syndrome centres on the role of spider envenomation; and in particular on the speculative role of the Australian White-tailed spider, Lampona cylindrata. METHODS: We present here six cases of necrotizing dermatitis treated surgically at the Royal Children's Hospital and Mater Children's Hospital in Brisbane over the period from 1991 to 1999. Clinical history, surgical details and pathological investigations were reviewed in each case. Microbiological investigation of necrotic ulcers included standard aerobic and anaerobic culture. RESULT: Nocardia and Staphylococcus were cultured in two cases, but no positive bites were witnessed and no spiders were identified by either the children or their parents. All cases were treated with silver sulphadiazine creme. Two of the infants required general anaesthesia, excision debridement and split skin grafting. The White-tailed spider, Lampona cylindrata, does not occur in Queensland, but Lampona murina does; neither species has necrotizing components in its venom. Circumstantial evidence is consistent with this syndrome being due to invertebrate envenomation, possibly following arachnid bites. CONCLUSION: In our experience there is insufficient evidence to impute a specific genus as the cause, at this stage of scientific knowledge. If the offending creature is a spider, we calculate that the syndrome of necrotizing dermatitis occurs in less than 1 in 5000 spider bites.

Dr James George Beaney (1828-1891): a pioneer Australian paediatrician and paediatric surgeon.

Dr James George Beaney (1828-1891) was a flamboyant and controversial Melbourne surgeon and paediatrician. He was the first in Australia, in 1859, to publish a medical textbook; and the first, in 1873, to publish a paediatric text, Children: their treatment in health and disease. An analysis of four of his published works relating to paediatrics and paediatric surgery establishes his place as a true pioneer in the chronology of children's medicine and welfare in his adopted land. He undertook heroic yet conservative surgery on children, was the first to write in detail about paediatric anaesthesia, and was the pioneer of family planning in Australia. In Children: their treatment in health and disease, he described in detail the supreme importance of breastfeeding, detailed clear practical concepts for the weaning of infants and discussed the diagnosis and management of diseases of the mouth, ears, eyes and teeth of infants. Beaney was shunned by much of the established medical profession because of his self-promoting flamboyance and his egotism. However, an audit of surviving archives and of his published works affords him a place as another, hitherto unacknowledged true pioneer of Australian paediatrics.

The Cotard syndrome. Report of two patients: with a review of the extended spectrum of 'délire des négations'.

The Cotard syndrome is characterized by the delusion where an individual insists that he has died or part of his body has decayed. Although described classically in schizophrenia and bipolar disorder, physical disorders including migraine, tumour and trauma have also been associated with the syndrome. Two new cases are described here, the one associated with arteriovenous malformations and the other with probable multiple sclerosis. The delusion has been embarrassing to each patient. Study of such cases may have wider implications for the understanding of the psychotic interpretation of body image, for example that occurring in anorexia nervosa.

Children and war.

Children bear disproportionate consequences of armed conflict. The 21st century continues to see patterns of children enmeshed in international violence between opposing combatant forces, as victims of terrorist warfare, and, perhaps most tragically of all, as victims of civil wars. Innocent children so often are the victims of high-energy wounding from military ordinance. They sustain high-energy tissue damage and massive burns - injuries that are not commonly seen in civilian populations. Children have also been deliberately targeted victims in genocidal civil wars in Africa in the past decade, and hundreds of thousands have been killed and maimed in the context of close-quarter, hand-to-hand assaults of great ferocity. Paediatricians serve as uniformed military surgeons and as civilian doctors in both international and civil wars, and have a significant strategic role to play as advocates for the rights and welfare of children in the context of the evolving 'Laws of War'. One chronic legacy of contemporary warfare is blast injury to children from landmines. Such blasts leave children without feet or lower limbs, with genital injuries, blindness and deafness. This pattern of injury has become one of the post-civil war syndromes encountered by all intensivists and surgeons serving in four of the world's continents. The continued advocacy for the international ban on the manufacture, commerce and military use of antipersonnel landmines is a part of all paediatricians' obligation to promote the ethos of the Laws of War. Post-traumatic stress disorder remains an undertreated legacy of children who have been trapped in the shot and shell of battle as well as those displaced as refugees. An urgent, unfocused and unmet challenge has been the increase in, and plight of, child soldiers themselves. A new class of combatant comprises these children, who also become enmeshed in the triad of anarchic civil war, light-weight weaponry and drug or alcohol addiction. The International Criminal Court has outlawed as a War Crime, the conscription of children under 15 years of age. Nevertheless, there remain more than 300000 child soldiers active and enmeshed in psychopathic violence as part of both civil and international warfare. The typical profile of a child soldier is of a boy between the ages of 8 and 18 years, bonded into a group of armed peers, almost always an orphan, drug or alcohol addicted, amoral, merciless, illiterate and dangerous. Paediatricians have much to do to protect such war-enmeshed children, irrespective of the accident of their place of birth. Only by such vigorous and maintained advocacy can the world's children be better protected from the scourge of future wars.

Jules Cotard (1840-1889): his life and the unique syndrome which bears his name.

Dr. Jules Cotard (1840-1889) was a Parisian neurologist who first described the délire des négations. Cotard's syndrome or Cotard's delusion comprises any one of a series of delusions ranging from the fixed and unshakable belief that one has lost organs, blood, or body parts to believing that one has lost one's soul or is dead. In its most profound form, the delusion takes the form of a professed belief that one does not exist. Encountered primarily in psychoses such as schizophrenia and bipolar disorder, Cotard's syndrome has also been described in organic lesions of the nondominant temporoparietal cortex as well as in migraine. Cotard's delusion is the only self-certifiable syndrome of delusional psychosis. Jules Cotard, a Parisian neurologist and psychiatrist and former military surgeon, was one of the first to induce cerebral atrophy by the experimental embolization of cerebral arteries in animals and a pioneer in studies of the clinicopathologic correlates of cerebral atrophy secondary to perinatal and postnatal pathologic changes. He was the first to record that unilateral cerebral atrophy in infancy does not necessarily lead to aphasia and was also the pioneer of studies of altered conscious states in diabetic hyperglycemia.

Forced emigration, favourable outcomes.

The discipline of public health and preventive medicine in Australia and New Zealand had its genesis in the advocacy of 18th and 19th century military pioneers. Military (Royal Navy and British Army) surgeons were posted to Australia as part of their non-discretionary duty. Civilian doctors emigrated variously for adventure, escapism and gold fever. One group, a particularly influential group disproportionate to their numbers, came in one sense as forced emigrants because of chronic respiratory disease in general, and tuberculosis in particular. Tuberculosis was an occupational hazard of 19th century medical and surgical practice throughout western Europe. This paper analyses six examples of such emigration which had, perhaps unforeseen at the time, significant results in the advancement of public health. Such emigration was in one sense voluntary, but in another was forced upon the victims in their quest for personal survival. In Australia, such medical individuals became leading advocates and successful catalysts for change in such diverse fields as social welfare, public health, the preventive aspects of medical practice, child health, nutrition and medical education. A number of such public health pioneers today have no physical memorials; but their influence is to be seen in the ethos of medical practice in Australia and New Zealand today. Their memory is further perpetuated in the names of Australian native wildflowers and trees that symbolise not only a healthy environment but the long-term investment, accrued with interest, of the institution of public health measures for which their advocacy achieved much success.