813 TABLE I-PLASMA BRANCHED-CHAIN-AMINOACID LEVELS
Letters
to
the Editor
FOLLOWING HOSPITAL ADMISSION
FACULTY OF COMMUNITY MEDICINE
SiR,-The Report
of the
Royal
Commission
on
Medical
Education (1965-68) recommended that an organisation should be formed which would have a major role in the
training of those who practise in the field of community *Peritoneal
medicine.
dialysis commenced f Samples taken post mortem.
of the
of
A working-party, composed representatives Royal College of Physicians of London, the Royal College of Physicians of Edinburgh, and the Royal College of Physicians and Surgeons of Glasgow, and of doctors drawn from the Society of Medical Officers of Health and its
TABLE II-PLASMA POTASSIUM AND ACID-BASE STATUS FOLLOWING HOSPITAL ADMISSION
Scottish branch, the senior administrative medical officers
regional hospital boards in England and the Welsh Hospital Board, the Society for Social Medicine, the Scottish Association of Medical Administrators, medical members of the staffs of the Department of Health and Social Security, and the Scottish Home and Health Department, has been meeting over the past twelve months. As the result of these meetings the working-party has recommended to the Colleges that they should combine in the formation of a Faculty of Community Medicine
of the
which will be part of their own structure. Community medicine is that branch of medicine which deals with populations or groups rather than with individual patients. In the context of a national system of medical care, it therefore comprises those doctors who try to measure accurately the needs of the population both sick and well. It will require to bring to this study special knowledge of the principles of epidemiology, of the organisation and evaluation of medical-care systems, of the medical aspects of the administration of health services, and of the techniques of health education and rehabilitation which are comprised within the field of social and preventive medicine. Community medicine thus brings together within the one discipline those who are engaged in the practice of public health, in the administration of the health service, whether in hospital, local authority, or central government, in relevant research, and those responsible for undergraduate and postgraduate education in the university departments of social medicine. It is hoped that the inaugural meeting of the Faculty will take place in March, 1972, and we would like to draw attention to the invitation, which appears on another page (advertisements p. 16), to those eligible to apply for Foundation Membership of the Faculty. President, Royal College of Physicians of Edinburgh.
J. HALLIDAY CROOM.
President, Royal College of Physicians and Surgeons of Glasgow.
E. M. MCGIRR.
President, Royal College of Physicians of London. ROSENHEIM.
stormy neonatal course, she made satisfactory progress. She was maintained on a reduced branched-chain-aminoacid intake, with appropriate vitamin and mineral supplements. At the age of 21 months her development was assessed to be at the 18-month level. 4 days before her last admission she developed mild diarrhoea, but remained generally well until immediately before being admitted, when she began to vomit. After admission she became increasingly drowsy and irritable. The levels of branchedchain aminoacids are shown in table I, and plasma potassium and acid-base readings in table n. An intravenous infusion was commenced with 10% dextrose alternating with 20% fructose, which gave an intake of 150 C. per kg. bodyweight. The acidosis was corrected with 60 meq. 8-4% sodium bicarbonate solution and she was also given 10 ml. ofParentrovite’, which contains 500 mg. of thiamine. Thirty-six hours after admission she was semicomatose and hypokalxmic despite potassium supplementation of 3 meq. per kg. body-weight. By forty-eight hours she was unconscious and convulsing, and there was a strong odour of maple syrup in her urine. Peritoneal dialysis was commenced usingDialaflex No. 61’ in cycles of 500 ml. hourly. This lowered the plasma-levels of her branchedchain aminoacids, but made little difference to her clinical state. The hypokalaemia persisted despite the addition of 12 meq. potassium to each 500 ml. of dialysing fluid, and intravenous potassium chloride at 4-5 meq. per kg. bodyweight. Terminally, the acidosis recurred and could not be corrected by intravenous sodium bicarbonate, and her blood-pressure fell. At one stage it became unrecordable, but rose following 75 ml. of plasma. Sixty-four hours after admission she had a cardiac arrest and died. No postmortem examination was obtained.
Comment In this child
INFECTION IN MAPLE-SYRUP-URINE DISEASE
SIR,-With early diagnosis and dietary control, children maple-syrup-urine disease (M.S.U.D.) can thrive. Recently, a 2-year-old patient of the late Dr. Dennis
with
Cottom was admitted to the ward with severe metabolic acidosis precipitated by a trivial infection, and she died
despite our attempts at control. I report the details of this case in the hope that others faced with this complication may have
more success.
Case-report The patient was born in July, 1969, and the diagnosis of M.S.U.D. was made on the 7th day of life. Following a
a severe
metabolic disturbance followed
presumed gastroenteritis-other members of the family had been similarly affected. She became hypercatabolic, and, in the absence of adequate oral nutrition, began to break down her own body protein with subsequent increase in branched-chain-aminoacid levels in her plasma. The accumulation of the ketoacid derivatives caused a metabolic acidosis. Many children with M.S.U.D. who survive the neonatal period subsequently succumb during the course of an intercurrent infection.1 This seems to happen particularly during the 2nd year of life. Our patient’s death despite 1.
Dickinson, J. P., Holton, J. B., Lewis, G. M., Littlewood, J. M., Steel, A. E. Acta pœdiat. scand. 1969, 58, 341.