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MEASURING BLOOD-LOSS
1218 CLINICAL FEATURES OF FAMILIAL DYSAUTONOMIA
frequent familial incidence of the disease suggests that this is due to a single gene; and according to modern genetic concepts this must be due to a specific block in some essential metabolic pathway, possibly related to the absence of an enzyme and possibly situated within the central
nervous
system.
Summary
sympathetic and sympathetic nervous systems. These have been summarised in the table. All of them, save those classified as " miscellaneous ", could be related to an overactivity or overproduction of acetylcholine at the nerve-endings. The effects of acetylcholine on glands and on viscera, such as the urinary bladder and gut, are too well known to require discussion here, but some comment on its less familiar effects on blood-vessels and skeletal muscles may be necessary. All parasympathetic preganglionic and postganglionic fibres, the sympathetic postganglionic fibres to the sweatglands, as well as those responsible for vasodilation, and also the sympathetic preganglionic fibres are cholinergic. It is clear that overactivity of these various cholinergic fibres could explain such features of familial dysautonomia as excess sweating, areas of vasodilation in the skin, and postural hypotension. Furthermore, Koelle (1955) found that there were some cholinergic fibres in almost all predominantly adrenergic and sensory nerves. He suggested that the terms " cholinergic " and " adrenergic " might refer to the predominant, although not necessarily the exclusive, transmitting agents of the respective nervefibres. Burn and Rand (1959, 1960) also demonstrated the presence of some cholinergic fibres in sympathetic nerves. Moreover, they showed that by means of a nicotine-like action exerted at the periphery, acetylcholine could produce sympathetic effects by releasing noradrenaline at the nerve-endings. These histochemical studies support the contention that the variations in bloodpressure of familial dysautonomia could be attributed to the overproduction of substances with an action like acetylcholine. The chemical transmitting agent at the motor end-plate is, of course, acetylcholine; and muscular contraction is accompanied by depolarisation of the muscle-fibre. It is well recognised that repolarisation is prevented by excess and this phenQmenon of acetylcholine acetylcholine, " poisoning " could account for the muscle weakness, diminished tendon reflexes, motor incoordination, and difficulty in swallowing seen in our patients. The tracings in fig. 3 indicate that the spread of the nerve impulse through the muscle was impeded by groups of musclefibres in a depolarised state. A nerve-plexus in the skin from case 1 obtained during life, and stained specifically for acetylcholinesterase, showed this enzyme in excess of
normal (fig. 4). It is accepted by MacIntosh (1941),
Feldberg and Vogt (1948), and Burgen and Chipman (1951) that in the peripheral nerves the concentrations of acetylcholine, acetylcholinesterase, and choline acetylase are generally parallel. Thus apart from the miscellaneous group of clinical signs (see table) which are at present unexplained, the other features of familial dysautonomia can be explained on the hypothesis that there is an overactivity of the cholinergic nerve-fibres throughout the body. The
Two siblings with familial dysautonomia are described (one in detail). They were entirely of Scottish descent, and non-Jewish. Muscle testing in one patient showed normal nerve conduction, but inhibition of the spread of the impulse through the muscle. This is attributed to prolonged depolarisation of the muscle-fibres as a consequence of excess production of acetylcholine at the motor nerve-
endings. By a special staining technique excess production of acetylcholinesterase was demonstrated in a nerve-plexus of the skin in the same patient. The clinical features of familial dysautonomia may be largely due to an excess production of acetylcholine. indebted to Dr. G. B. S. Roberts for the histochemical to Dr. A. Scott for help with its interpretation. We are also grateful to Miss M. Fitzgerald for the muscle-testing, and to Mr. J. Devlin for the photographs. We
are
preparation;
REFERENCES
Burn, J. H., Rand, M. J. (1959) Nature, Lond. 184, 163. (1960) Brit. J. Pharmacol. 15, 56. Burgen, A. S. V., Chipman, L. M. (1951) J. Physiol. 114, 296. Feldberg, W., Vogt, M. (1948) ibid. 107, 372. Grossman, H. J., Limosani, M. A., Shore, M. (1956) J. Pediat. 49, 289. Harris, J. R., Gall, H., Wasser, S. (1955) Pediatrics, 16, 842. Koelle, G. B. (1955) J. Pharmacol. 114, 167. Linde, L. M. (1955) J. Pediat. 46, 453. MacIntosh, F. C. (1941) J. Physiol. 99, 436. McKendrick, T. (1958) Arch. Dis. Childh. 33, 465. Riley, C. M., Day, R. L., Greeley, D. McL., Langford, W. S. (1949) Pediatrics, 3, 468. — Freedman, A. M., Langford, W. S. (1954) ibid. 14, 475. Scott, A. (1958) Brit. J. Derm. 70, 1. St. Martin, D. A. (1953) Clin. Proc. Child. Hosp., Wash. 9, 81. Stradler, H. E. (1958) J. Pediat. 53, 481. -
-
New Inventions MEASURING BLOOD-LOSS DURING major operations it has become standard practice to weigh the swabs to give the surgeon and anaesthetist an estimate of the blood-loss. At first we used ordinary scales and subtracted the weight of
1219 the dry swabs from that of the used swabs. This involved much arithmetic, and also tended to become more and more inthe need for accuracy increased. There is an electronic " washing machine " for measuring blood-loss; but it is complex, the surgeon cannot use saline with it, and it interferes with swab-counting. For some time now we have used a simple balance with two plastic pails to measure blood-loss (fig. 1). For every used swab placed in one pail we put an identical dry one in the other; the dial reading then gives the blood-loss. The lids reduce evaporation, and all swabs are left in the pails. Those used for counter-balancing are coloured to prevent any confusion with When the blood-loss reading reaches the the count. maximum shown on the scale-in our case 500 g.-this is
Reviews of Books
accurate as
recorded, and swabs. The
be
a
500 g. weight is added to the coloured then returns to 0, and the process can
pointer
repeated.
This method has proved so simple and successful that we have adopted it for measuring blood-loss by suction. The balance is essentially the same, but with it is incorporated a Matburn double-bottle suction apparatus (fig. 2). Each bottle is used independently, so that when one is full it can be disconnected, emptied, and replaced without interrupting either the blood-loss reading or the suction. While a bottle is being emptied, a weight equal to that of the empty bottle is used to compensate. We
are
Hospital,
grateful to Dr. D. D. C. Howat, anxsthetist to St. George’s for his interest; and to Mr. J. G. Russell, of Messrs.
W. & T. Avery, for his advice and enthusiastic cooperation in adapting the balance for use with the suction apparatus.
A. M. MATTHIAS Theatre Superindendent St.
George’s Hospital, London, S.W.1
M. J. PENFOLD Deputy Theatre Superintendent
The British National Health Service DONALD McI. JOHNSON, M.B., Barrister-at-Law. Johnson Publications Ltd. 1962. Pp. 234. 21s.
London:
THIS is a very readable book by a Member of Parliament who has been a general practitioner both before and after the advent of the National Health Service. The early chapters contain an objective historical review of the health services of England from the turn of the century, and they are followed by an account of the negotiations immediately preceding the Act of 1946. The bulk of the book is a criticism of the service, with particular emphasis on the general practitioner’s loss of independence and his lack of incentive through payment by capitation. Dr. Johnsonjustly condemns the tripartite arrangement whereby general practitioners, hospitals, and preventive services are under separate managements, and this arrangement undoubtedly has grave disadvantages-though, in separating the practitioner from the hospital, it does at any rate ensure that he is in full charge of his own practice. One difficulty in uniting the three parts of the service has been to devise an administrative unit which is not too large for the general-practitioner and preventive services and too small for the hospitals. But many of the ills Dr. Johnson so graphically describes are not attributable to the N.H.S. Since the war there has been a scientific and social revolution. High-powered salesmanship of new drugs would have happened anyway. For better or worse, people are much more health conscious and are no longer satisfied with a platitude and placebo. By 1946 the voluntary hospitals were bankrupt and needed large Government subventions to keep them open-which led inexorably to a large degree of national control. Dr. Johnson is right in saying that there is a large fund of good will available locally; but we for our part do not believe that this could be translated into the millions of money needed to modernise the hospitals and build health centres for practitioners, which is what he suggests. Other proposals are the abolition of regional hospital boards (which many regard as one of the outstanding successes of the service) and the creation in each county and county borough of a semiautonomous local health board, administering all three branches of the service. The majority of the members would be elected representatives of the family and hospital doctors, the nurses, the ancillary professions, the " friends " of the hospitals, and other groups. Though this sounds attractive, anyone who has worked with such a body of delegates will know that each individual seldom takes interest in matters outside his own specialty and few are capable of viewing the service as a whole. The author gives the impression of trying hard to be objective, but he too often questions the motives of those who have a good word for the service.
Diseases of the Intervertebral Disc and its Tissues
Surrounding
REUBEN RABINOVITCH, M.sc., M.D., assistant professor in neurology and neurosurgery, McGill University, Montreal. Springfield, Ill.: Charles C. Thomas. Oxford: Blackwell Scientific Publications. 1961. Pp. 152. 68s.
THE interest in intervertebral disc protrusions is reflected in the rate at which publications on the subject have multiplied. The Index Medicus for 1929 listed only 5 papers dealing with it: by 1960 some 2500 had appeared, and the number is increasing by about 130 annually. This immense literature deals very adequately, as Dr. Rabinovitch points out, with the diagnosis and treatment of the condition. Very little, however, has been added to what is known of its aetiology. Some lesions are obviously due to injury, and trauma is often blamed for many more. The researches which Dr. Rabinovitch now reports were designed to determine whether some physical or chemical alteration in the composition of a disc predisposes to its protrusion. A simple but very effective differential staining method, which is to likely to be widely adopted, was devised and
applied
frequent familial incidence of the disease suggests that this is due to a single gene; and according to modern genetic concepts this must be due to a specific block in some essential metabolic pathway, possibly related to the absence of an enzyme and possibly situated within the central
nervous
system.
Summary
sympathetic and sympathetic nervous systems. These have been summarised in the table. All of them, save those classified as " miscellaneous ", could be related to an overactivity or overproduction of acetylcholine at the nerve-endings. The effects of acetylcholine on glands and on viscera, such as the urinary bladder and gut, are too well known to require discussion here, but some comment on its less familiar effects on blood-vessels and skeletal muscles may be necessary. All parasympathetic preganglionic and postganglionic fibres, the sympathetic postganglionic fibres to the sweatglands, as well as those responsible for vasodilation, and also the sympathetic preganglionic fibres are cholinergic. It is clear that overactivity of these various cholinergic fibres could explain such features of familial dysautonomia as excess sweating, areas of vasodilation in the skin, and postural hypotension. Furthermore, Koelle (1955) found that there were some cholinergic fibres in almost all predominantly adrenergic and sensory nerves. He suggested that the terms " cholinergic " and " adrenergic " might refer to the predominant, although not necessarily the exclusive, transmitting agents of the respective nervefibres. Burn and Rand (1959, 1960) also demonstrated the presence of some cholinergic fibres in sympathetic nerves. Moreover, they showed that by means of a nicotine-like action exerted at the periphery, acetylcholine could produce sympathetic effects by releasing noradrenaline at the nerve-endings. These histochemical studies support the contention that the variations in bloodpressure of familial dysautonomia could be attributed to the overproduction of substances with an action like acetylcholine. The chemical transmitting agent at the motor end-plate is, of course, acetylcholine; and muscular contraction is accompanied by depolarisation of the muscle-fibre. It is well recognised that repolarisation is prevented by excess and this phenQmenon of acetylcholine acetylcholine, " poisoning " could account for the muscle weakness, diminished tendon reflexes, motor incoordination, and difficulty in swallowing seen in our patients. The tracings in fig. 3 indicate that the spread of the nerve impulse through the muscle was impeded by groups of musclefibres in a depolarised state. A nerve-plexus in the skin from case 1 obtained during life, and stained specifically for acetylcholinesterase, showed this enzyme in excess of
normal (fig. 4). It is accepted by MacIntosh (1941),
Feldberg and Vogt (1948), and Burgen and Chipman (1951) that in the peripheral nerves the concentrations of acetylcholine, acetylcholinesterase, and choline acetylase are generally parallel. Thus apart from the miscellaneous group of clinical signs (see table) which are at present unexplained, the other features of familial dysautonomia can be explained on the hypothesis that there is an overactivity of the cholinergic nerve-fibres throughout the body. The
Two siblings with familial dysautonomia are described (one in detail). They were entirely of Scottish descent, and non-Jewish. Muscle testing in one patient showed normal nerve conduction, but inhibition of the spread of the impulse through the muscle. This is attributed to prolonged depolarisation of the muscle-fibres as a consequence of excess production of acetylcholine at the motor nerve-
endings. By a special staining technique excess production of acetylcholinesterase was demonstrated in a nerve-plexus of the skin in the same patient. The clinical features of familial dysautonomia may be largely due to an excess production of acetylcholine. indebted to Dr. G. B. S. Roberts for the histochemical to Dr. A. Scott for help with its interpretation. We are also grateful to Miss M. Fitzgerald for the muscle-testing, and to Mr. J. Devlin for the photographs. We
are
preparation;
REFERENCES
Burn, J. H., Rand, M. J. (1959) Nature, Lond. 184, 163. (1960) Brit. J. Pharmacol. 15, 56. Burgen, A. S. V., Chipman, L. M. (1951) J. Physiol. 114, 296. Feldberg, W., Vogt, M. (1948) ibid. 107, 372. Grossman, H. J., Limosani, M. A., Shore, M. (1956) J. Pediat. 49, 289. Harris, J. R., Gall, H., Wasser, S. (1955) Pediatrics, 16, 842. Koelle, G. B. (1955) J. Pharmacol. 114, 167. Linde, L. M. (1955) J. Pediat. 46, 453. MacIntosh, F. C. (1941) J. Physiol. 99, 436. McKendrick, T. (1958) Arch. Dis. Childh. 33, 465. Riley, C. M., Day, R. L., Greeley, D. McL., Langford, W. S. (1949) Pediatrics, 3, 468. — Freedman, A. M., Langford, W. S. (1954) ibid. 14, 475. Scott, A. (1958) Brit. J. Derm. 70, 1. St. Martin, D. A. (1953) Clin. Proc. Child. Hosp., Wash. 9, 81. Stradler, H. E. (1958) J. Pediat. 53, 481. -
-
New Inventions MEASURING BLOOD-LOSS DURING major operations it has become standard practice to weigh the swabs to give the surgeon and anaesthetist an estimate of the blood-loss. At first we used ordinary scales and subtracted the weight of
1219 the dry swabs from that of the used swabs. This involved much arithmetic, and also tended to become more and more inthe need for accuracy increased. There is an electronic " washing machine " for measuring blood-loss; but it is complex, the surgeon cannot use saline with it, and it interferes with swab-counting. For some time now we have used a simple balance with two plastic pails to measure blood-loss (fig. 1). For every used swab placed in one pail we put an identical dry one in the other; the dial reading then gives the blood-loss. The lids reduce evaporation, and all swabs are left in the pails. Those used for counter-balancing are coloured to prevent any confusion with When the blood-loss reading reaches the the count. maximum shown on the scale-in our case 500 g.-this is
Reviews of Books
accurate as
recorded, and swabs. The
be
a
500 g. weight is added to the coloured then returns to 0, and the process can
pointer
repeated.
This method has proved so simple and successful that we have adopted it for measuring blood-loss by suction. The balance is essentially the same, but with it is incorporated a Matburn double-bottle suction apparatus (fig. 2). Each bottle is used independently, so that when one is full it can be disconnected, emptied, and replaced without interrupting either the blood-loss reading or the suction. While a bottle is being emptied, a weight equal to that of the empty bottle is used to compensate. We
are
Hospital,
grateful to Dr. D. D. C. Howat, anxsthetist to St. George’s for his interest; and to Mr. J. G. Russell, of Messrs.
W. & T. Avery, for his advice and enthusiastic cooperation in adapting the balance for use with the suction apparatus.
A. M. MATTHIAS Theatre Superindendent St.
George’s Hospital, London, S.W.1
M. J. PENFOLD Deputy Theatre Superintendent
The British National Health Service DONALD McI. JOHNSON, M.B., Barrister-at-Law. Johnson Publications Ltd. 1962. Pp. 234. 21s.
London:
THIS is a very readable book by a Member of Parliament who has been a general practitioner both before and after the advent of the National Health Service. The early chapters contain an objective historical review of the health services of England from the turn of the century, and they are followed by an account of the negotiations immediately preceding the Act of 1946. The bulk of the book is a criticism of the service, with particular emphasis on the general practitioner’s loss of independence and his lack of incentive through payment by capitation. Dr. Johnsonjustly condemns the tripartite arrangement whereby general practitioners, hospitals, and preventive services are under separate managements, and this arrangement undoubtedly has grave disadvantages-though, in separating the practitioner from the hospital, it does at any rate ensure that he is in full charge of his own practice. One difficulty in uniting the three parts of the service has been to devise an administrative unit which is not too large for the general-practitioner and preventive services and too small for the hospitals. But many of the ills Dr. Johnson so graphically describes are not attributable to the N.H.S. Since the war there has been a scientific and social revolution. High-powered salesmanship of new drugs would have happened anyway. For better or worse, people are much more health conscious and are no longer satisfied with a platitude and placebo. By 1946 the voluntary hospitals were bankrupt and needed large Government subventions to keep them open-which led inexorably to a large degree of national control. Dr. Johnson is right in saying that there is a large fund of good will available locally; but we for our part do not believe that this could be translated into the millions of money needed to modernise the hospitals and build health centres for practitioners, which is what he suggests. Other proposals are the abolition of regional hospital boards (which many regard as one of the outstanding successes of the service) and the creation in each county and county borough of a semiautonomous local health board, administering all three branches of the service. The majority of the members would be elected representatives of the family and hospital doctors, the nurses, the ancillary professions, the " friends " of the hospitals, and other groups. Though this sounds attractive, anyone who has worked with such a body of delegates will know that each individual seldom takes interest in matters outside his own specialty and few are capable of viewing the service as a whole. The author gives the impression of trying hard to be objective, but he too often questions the motives of those who have a good word for the service.
Diseases of the Intervertebral Disc and its Tissues
Surrounding
REUBEN RABINOVITCH, M.sc., M.D., assistant professor in neurology and neurosurgery, McGill University, Montreal. Springfield, Ill.: Charles C. Thomas. Oxford: Blackwell Scientific Publications. 1961. Pp. 152. 68s.
THE interest in intervertebral disc protrusions is reflected in the rate at which publications on the subject have multiplied. The Index Medicus for 1929 listed only 5 papers dealing with it: by 1960 some 2500 had appeared, and the number is increasing by about 130 annually. This immense literature deals very adequately, as Dr. Rabinovitch points out, with the diagnosis and treatment of the condition. Very little, however, has been added to what is known of its aetiology. Some lesions are obviously due to injury, and trauma is often blamed for many more. The researches which Dr. Rabinovitch now reports were designed to determine whether some physical or chemical alteration in the composition of a disc predisposes to its protrusion. A simple but very effective differential staining method, which is to likely to be widely adopted, was devised and
applied