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TURNER'S SYNDROME AND CONGENITAL ERYTHROID HYPERPLASIA
449 dissolved in xylocaine, a plain xylocaine control being used. A gross erythematous reaction developed at the phenylbutazone patch site on the forearm in about 4 hours and spread to involve the whole limb, proximal to the test area and the face. The arm became very swollen. The patient complained of severe " tingling " in the region of the ipsilateral epitrochlear and axillary nodes, which she said was exactly the same as the initial reaction on previous occasions. Since she also developed pronounced periorbital oedema, the reaction was stopped with chlorpheniramine maleate before the development of overt
lymphadenopathy. In the two previous reports of lymphadenopathy in phenylbutazone sensitivity this reaction seems to have been localised to the cervical nodes. The severe and generalised nature of the reaction in this case is in contrast to the previous findings. Our thanks are due reference facilities.
Harrogate
Royal Bath Yorkshire.
to
30-60 V alternating current applied directly to the right ventricle. On 10 separate occasions in 4 dogs defibrillation was accomplished successfully. Aconitine-induced ventricular fibrillation, therefore, has a persistent focus, which cannot be reverted by a D.c. discharge. The D.c. defibrillator (supplied by Short Bros & Harland Ltd., Belfast) used in this study was fairly cheap, compact, and portable, and a further modification has resulted in an even lighter and smaller model weighing 15.7 kg. and measuring
by
44 x 24 x 18 cm. Department of Physiology, The Queen’s University, Belfast. Cardiac Department, Belfast City Hospital, Belfast.
PETER F. BINNION R. J. MCFARLAND. E. FLETCHER.
Dr. P. D. Fowler of Geigy, Ltd., for
Hospital,
T. G. PLUNKETT D. I. HASLOCK J. R. GOLDING.
Medicine and the Law As Others See Us
TURNER’S SYNDROME AND CONGENITAL ERYTHROID HYPERPLASIA the SIR,-For past 15 years we have observed the course of a girl with congenital erythroid hypoplasia associated with severe growth retardation, absence of sexual maturation, and radiological evidence of a horseshoe-shaped kidney. Her growth-hormone is normal. Chromosomal analysis shows an XO karyotype. In the light of a report on chromosomal breakage in some patients with Fanconi’s ansemia/ as well as a similar report on a patient with hypoplastic anxmia and hypocalcoemiawe undertook a survey of the simultaneous occurrence of Turner’s syndrome and congenital erythroid hypoplasia among the major medical centres of the United States and Canada. A questionary to 70 poediatric haematologists resulted in a 96% return and only two positive responses: a girl who died before the era of karyotyping,3 and a so-called " male Turner’s syndrome " with an XY karyotype. A third possibility was a female with the clinical stigmata of Turner’s syndrome but with a normal chromosomal analysis.5 Statistically, the evidence overwhelmingly supports the chance occurrence of congenital erythroid hypoplasia with Turner’s syndrome, but we wonder whether any of your readers have observed a similarly proven association. Department of Pediatrics, ARTHUR J. NEWMAN Western Reserve University, SAMUEL GROSS. Cleveland, Ohio 44106.
ACONITINE-INDUCED FIBRILLATION
SIR,-We have studied the action of a new defibrillator on ventricular fibrillation induced by the local application of aconitine to the myocardium. Dogs anaesthetised with sodium pentobarbitone (30 mg. per kg. intravenously) were used, and through a right-sided thoracotomy the pericardium was incised and the right ventricle exposed. A diluted solution of aconitine in chloroform (0-010-1%) was applied directly to the wall of the right ventricle and ventricular fibrillation was produced within a few minutes. Direct-current (D.c.) discharges of 40-225 joules had no effect on this arrhythmia in 7 dogs. Since the defibrillator was new, it was not clear whether this failure to revert ventricular fibrillation was a fault of the machine or was due to persistence of the aconitine focus. In order to resolve this point ventricular fibrillation was induced 1.
2.
Tartaglia, A. P., Propp, S., Amarose, A. P., Propp, R. P., Hall, C. A. Am. J. Med. 1966, 41, 990. Bloom, G. E., Warner, S., Gerald, P. S., Diamond, L. K. New Engl. J. Med. 1966, 274, 8.
3. Diamond, L. K. Personal communication. 4. McElfresh, W. M. Personal communication. 5. Mauer, A. M. Personal communication.
FROM A MEDICOLEGAL CORRESPONDENT
AN editorial in the Law Guardian1 suggests that there is something like a conspiracy of silence among the medical profession when a member is asked for reasoned comment or evidence on the alleged negligence of a colleague. Conspiracy is too strong a word, but it is certainly true that such comment is very difficult to obtain. The doctor’s attitude to the courts is the result of his " upbringing and his experience. He is brought up in a no speaking " tradition; he is taught by consultants who make no secret of their dislike and disapproval of proceedings for negligence; he is often embittered by experience in the witness-box, where he does the answering and not the questioning. He reflects how nearly, if not how exactly, his own experience has duplicated that of his unfortunate colleague; he considers the difficulty of persuading patients and lawyers that medicine is not an exact science; that cortisone and leeches, cupping and laminectomy, can coexist in the clinical armamentarium and a place be found for all four. For all these reasons, and they are not discreditable, the doctor is often a reluctant witness. But his basic duty is to his patient. If disability is found to have been caused by medical treatment, or by failure to prescribe it, the doctor’s duty is to tell his patient, to advise him what he should do, and to help him, in cooperation with his legal advisers, in any action that may ensue.
When the doctor’s advice is sought over patients other than his own, the position is less clearcut, and he may be even more reluctant to give evidence or advice. Here I believe he is justified in making plain his dislike, if any, of attending courts of law and his own limitations of experience and specialisation. But if he can help, he should do so by giving a temperate and reasoned opinion to which he is prepared to swear. It is, of course, his duty to keep before those consulting him not only the aspects favourable to their plea, but also the unfavourable, for these will certainly be elicited in cross-examination. Many actions are now brought only because the solicitor for the plaintiffs, probably supported by legal aid, is unable to obtain a medical opinion until the defendant communicates with him through his protection society. Greater willingness on the doctor’s part to cooperate in the process of litigation would help both professions. 1. Law
Guardian, February,
1967.
lymphadenopathy. In the two previous reports of lymphadenopathy in phenylbutazone sensitivity this reaction seems to have been localised to the cervical nodes. The severe and generalised nature of the reaction in this case is in contrast to the previous findings. Our thanks are due reference facilities.
Harrogate
Royal Bath Yorkshire.
to
30-60 V alternating current applied directly to the right ventricle. On 10 separate occasions in 4 dogs defibrillation was accomplished successfully. Aconitine-induced ventricular fibrillation, therefore, has a persistent focus, which cannot be reverted by a D.c. discharge. The D.c. defibrillator (supplied by Short Bros & Harland Ltd., Belfast) used in this study was fairly cheap, compact, and portable, and a further modification has resulted in an even lighter and smaller model weighing 15.7 kg. and measuring
by
44 x 24 x 18 cm. Department of Physiology, The Queen’s University, Belfast. Cardiac Department, Belfast City Hospital, Belfast.
PETER F. BINNION R. J. MCFARLAND. E. FLETCHER.
Dr. P. D. Fowler of Geigy, Ltd., for
Hospital,
T. G. PLUNKETT D. I. HASLOCK J. R. GOLDING.
Medicine and the Law As Others See Us
TURNER’S SYNDROME AND CONGENITAL ERYTHROID HYPERPLASIA the SIR,-For past 15 years we have observed the course of a girl with congenital erythroid hypoplasia associated with severe growth retardation, absence of sexual maturation, and radiological evidence of a horseshoe-shaped kidney. Her growth-hormone is normal. Chromosomal analysis shows an XO karyotype. In the light of a report on chromosomal breakage in some patients with Fanconi’s ansemia/ as well as a similar report on a patient with hypoplastic anxmia and hypocalcoemiawe undertook a survey of the simultaneous occurrence of Turner’s syndrome and congenital erythroid hypoplasia among the major medical centres of the United States and Canada. A questionary to 70 poediatric haematologists resulted in a 96% return and only two positive responses: a girl who died before the era of karyotyping,3 and a so-called " male Turner’s syndrome " with an XY karyotype. A third possibility was a female with the clinical stigmata of Turner’s syndrome but with a normal chromosomal analysis.5 Statistically, the evidence overwhelmingly supports the chance occurrence of congenital erythroid hypoplasia with Turner’s syndrome, but we wonder whether any of your readers have observed a similarly proven association. Department of Pediatrics, ARTHUR J. NEWMAN Western Reserve University, SAMUEL GROSS. Cleveland, Ohio 44106.
ACONITINE-INDUCED FIBRILLATION
SIR,-We have studied the action of a new defibrillator on ventricular fibrillation induced by the local application of aconitine to the myocardium. Dogs anaesthetised with sodium pentobarbitone (30 mg. per kg. intravenously) were used, and through a right-sided thoracotomy the pericardium was incised and the right ventricle exposed. A diluted solution of aconitine in chloroform (0-010-1%) was applied directly to the wall of the right ventricle and ventricular fibrillation was produced within a few minutes. Direct-current (D.c.) discharges of 40-225 joules had no effect on this arrhythmia in 7 dogs. Since the defibrillator was new, it was not clear whether this failure to revert ventricular fibrillation was a fault of the machine or was due to persistence of the aconitine focus. In order to resolve this point ventricular fibrillation was induced 1.
2.
Tartaglia, A. P., Propp, S., Amarose, A. P., Propp, R. P., Hall, C. A. Am. J. Med. 1966, 41, 990. Bloom, G. E., Warner, S., Gerald, P. S., Diamond, L. K. New Engl. J. Med. 1966, 274, 8.
3. Diamond, L. K. Personal communication. 4. McElfresh, W. M. Personal communication. 5. Mauer, A. M. Personal communication.
FROM A MEDICOLEGAL CORRESPONDENT
AN editorial in the Law Guardian1 suggests that there is something like a conspiracy of silence among the medical profession when a member is asked for reasoned comment or evidence on the alleged negligence of a colleague. Conspiracy is too strong a word, but it is certainly true that such comment is very difficult to obtain. The doctor’s attitude to the courts is the result of his " upbringing and his experience. He is brought up in a no speaking " tradition; he is taught by consultants who make no secret of their dislike and disapproval of proceedings for negligence; he is often embittered by experience in the witness-box, where he does the answering and not the questioning. He reflects how nearly, if not how exactly, his own experience has duplicated that of his unfortunate colleague; he considers the difficulty of persuading patients and lawyers that medicine is not an exact science; that cortisone and leeches, cupping and laminectomy, can coexist in the clinical armamentarium and a place be found for all four. For all these reasons, and they are not discreditable, the doctor is often a reluctant witness. But his basic duty is to his patient. If disability is found to have been caused by medical treatment, or by failure to prescribe it, the doctor’s duty is to tell his patient, to advise him what he should do, and to help him, in cooperation with his legal advisers, in any action that may ensue.
When the doctor’s advice is sought over patients other than his own, the position is less clearcut, and he may be even more reluctant to give evidence or advice. Here I believe he is justified in making plain his dislike, if any, of attending courts of law and his own limitations of experience and specialisation. But if he can help, he should do so by giving a temperate and reasoned opinion to which he is prepared to swear. It is, of course, his duty to keep before those consulting him not only the aspects favourable to their plea, but also the unfavourable, for these will certainly be elicited in cross-examination. Many actions are now brought only because the solicitor for the plaintiffs, probably supported by legal aid, is unable to obtain a medical opinion until the defendant communicates with him through his protection society. Greater willingness on the doctor’s part to cooperate in the process of litigation would help both professions. 1. Law
Guardian, February,
1967.