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PAEDIATRIC SCURVY: OLD WINE IN NEW BOTTLES
1M. A. Anjay, 2V. Palanivel, 1R. Chaudhary, 1R. Stocks. 1James Paget University Hospital NHS Trust, Great Yarmouth, UK, 2Addenbrooke’s Hospital, Cambridge, UK
Scurvy as a paediatric disease of significant morbidity and mortality has been all but forgotten in most developed countries, including the UK. We present a case of scurvy, followed by a literature search for recent reports on scurvy and try to analyse these from a historical perspective.
Case report: A 6-year-old girl known to have cerebral palsy and hemiparesis presented with new-onset limping and painful swelling of the limbs. Subsequent evaluation revealed a poor diet, mild anaemia and some petechial spots. x-Ray and MRI studies demonstrated bone and soft tissue changes strongly suggestive of scurvy. There was dramatic response to vitamin C supplementation.
Literature search: A structured literature search was undertaken and all cases of scurvy reported from developed countries in the last 10 years were retrieved. The epidemiology of these cases was then analysed for any patterns and was then compared with historical descriptions.
Results and historical perspective: Classically, scurvy was first described in detail in sailors and the pioneering work by James Lind in this regard is well known. Towards the end of the 19th century, “infantile scurvy” was described, mostly in infants from affluent families who were given heated formula milk products. Subsequent changes in feeding practices and the fortification of milk products with vitamin C led to the virtual elimination of scurvy. It was believed to be confined to certain well defined adult groups, particularly the elderly population and alcoholics. This has led to a relative lack of knowledge among modern day paediatricians about the clinical features of this condition. Recent reports from the literature suggest that, although a rarity, paediatric scurvy still exists in developed countries. Unlike classical scurvy where infants were more affected, the disease tends to be seen in older children with neurodisability. Other risk groups include those with some form of eating disorder and those who require special diets for a prolonged period. Appreciation of this changing epidemiology and the clinical features is essential for early diagnosis and treatment. Some of the clinical features of scurvy can well be mistaken for those caused by non-accidental injury. Scurvy is a disease of antiquity, but it is not yet time to forget it.
SO NEAR AND YET SO FAR. JOHN LOCKE’S THOUGHTS CONCERNING RICKETS AND A MISSED CASE OF EBSTEIN’S ANOMALY
1A. Williams, 2N. Wilson, 3R. Sunderland. 1Virtual Academic Unit, Children’s Directorate, Northampton General Hospital, Northampton, UK, 2Department of Cardiology, Children’s Hospital, Oxford, UK, 3Birmingham Children’s Hospital, Birmingham, UK
The two pillars of Western medicine are human rights and evidence-based medicine. The physician/philosopher John Locke (1632–1704) is highly influential in both of these areas through his philosophical writings and also through his collaborative work with Thomas Sydenham (1624–89). Although Locke did not treat any identifiable patients until 1667, in 1666 his notebooks record his findings on a post mortem he performed upon an 18-month-old child who had physical signs of rickets. Locke was a medical student at this time and attributed rickets as the cause of death. However, Locke described and recognised severe cardiac abnormality and erroneously speculated on right to left inter-atrial shunting being part of rickets. Locke’s clear descriptions of the clinical history and post mortem findings are more consistent with a congenital cardiac malformation, an Ebstein’s anomaly, in addition to the rickets. Locke never considered this case being other than rickets. Locke’s notebooks are notable for their later corrections and added references. For this record, the page remains modified. This is most surprising when one considers that Locke’s own medical library contained a copy of Francis Glisson (1597–1677) De Rachidite (1650, among the earliest published treatise on rickets) a work that he frequently cited elsewhere in his writings. Also Locke and Glisson subsequently both consulted on the same case, Lord Ashley’s hydatid cyst. Locke also does not appear to have discussed the post mortem with his contemporary Richard Lower (1631–91), whose celebrated masterpiece on the heart Tractatus de Corde was published in 1669. One can only speculate why these omissions occurred. More recently, Locke’s own opinion remained unchallenged when the case report was re-presented and republished in the past half century. This paper forces a re-evaluation of 17th century understanding of infant cardiovascular physiology and pathology: Locke clearly gives one of the earliest descriptions of right to left shunting through the patent foramen ovale, although the reasons for his failure to recognise, update and communicate his findings remain unknown.
RIGHT TO LIFE OF INFANTS WITH DOWN SYNDROME: FROM EXCEPTION TO THE RULE
1M. Anderson, 2J. Anderson. 1Derbyshire Children’s Hospital, Derby, UK, 2Royal Wolverhampton Hospitals, Wolverhampton, UK
In 1981, the International Year of the Disabled Person, two landmark court rulings led to a radical change in the code of ethics of the British Medical Association: newborn “handicapped” babies were to be treated with the same respect as “normal” children. In one courtroom, a paediatrician, Dr Leonard Arthur, stood trial and was acquitted for attempted murder after withholding care from a baby born with Down syndrome whose parents did not wish them to survive. In his instructions to the jury, the judge indicated that it was lawful to sedate a baby and let them die if they were “irreversibly disabled” and “rejected by their parents.” In the other courtroom, a judge ruled that another infant with Down syndrome should undergo surgery for duodenal atresia despite the parents withholding consent, indicating that it was possible for the child to have a “happy life”, and determining that it was not for parents to decide the fate of their children. These two seemingly conflicting decisions are key points within the debate relating to the right to life of severely disabled children that perhaps commenced with the Zachary/Lorber conflict relating to “selective non-treatment” of babies with spina bifida in the 1960s and continues to the present day. Using contemporaneous press reports and opinions, they will be analysed within the context of the majority medical and social opinion of the time and their impact on current practice will be examined.
WAS BURNS CORRECT?
D. G. Young. Department of Surgical Paediatrics, Royal Hospital for Sick Children, Yorkhill, Glasgow, UK
A quarter of a millennium ago Robert Burns (1759–1856) the well-known Scottish bard was born. “To see ourselves as others see us” is one of his frequently quoted statements and it recalled some reflections on patients and doctors. Two incidents from over half a century ago stimulate me to present details of some of our predecessors and the reflections on “the caring profession” from around Burns’ time and subsequently. The first one I heard from a surgeon—ultimately Lord Brock—when I was a student and he came to Glasgow. Surgery of rheumatic heart disease was in its infancy when he gave his opinion of physicians at the time. The second was on going through the late George Macnab’s papers after his death. One of his patient’s histories entertained me before the internet approach of bringing a sheaf of papers to the consultant became the modern pattern. That the press are fickle and can change their opinion is not new, as a brief comment on the effect it has had on establishing some children’s services over the last three centuries. A number of illustrations will be given outlining how attitudes changed to doctors and those associated with the caring professions.
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