THE INHERITED AMYLOIDOSES

1
220 eight years out of dental fees alone. Free bone fluoride- filters which can be fitted to taps might be offered to fanatical anti-fluoridators, although I have never yet heard of one who was logical enough to filter the fluoride off his or her tea. I can think of no happier way of ending a historic Parliament. Beckley, Rye. C. G. LEAROYD. THE INHERITED AMYLOIDOSES A. M. G. CAMPBELL. SIR,-I was very interested -in the paper by Dr. Gafni and his colleagues (Jan. 11). Their description of these cases bears considerable resem- blance to a family which Clifton and I described in 1950,1 with neurological disturbances which we possibly wrongly attributed to adult toxoplasmosis. In retrospect the rash, joint pains, nerve-deafness, and enlarged spleen appear to suggest amyloid disease; but our cases had chronic meningoencephalitis, eosinophilia of the blood and cerebrospinal fluid, optic atrophy, hydrocephalus, and (in one at least) calcification of the brain. Although toxoplasmosis was never proved, the cytoplasm modifying test, serum complement-fixation test, and toxo- plasmin test were positive in all three cases, which does not prove necessarily that this disease had anything to do with the etiology but is worth considering. It might be of interest to consider toxoplasmosis in the amyloid cases described by Gafni, particularly if it was an immunity disturbance related to toxoplasmosis which produced the amyloid infiltration. Undoubtedly the cases described by Muckle and Wells 2 are somewhat similar, and possibly the cases that Clifton and I described were a variant of this familial amyloidosis which at the time we were not aware existed. The exact cause of amyloid infiltration still remains a mystery. 01 Bristol SCHOOL HEALTH SERVICE AND FAMILY DOCTOR A. L. SMALLWOOD. Central Health Clinic, Tower Hill, Bristol, 2. SIR,-Dr. Didsbury (Jan. 11) reminds us that many local-authority medical officers are still working in an outdated fashion. However, his criticism of the School Health Service glosses over many long unsolved problems. 1. Dr. Didsbury wishes to educate the parents to assume their health responsibilities. Who is to do this and how ? Our experience is that those parents in most need are the most difficult to approach, let alone teach. 2. There is a distinction between health problems which have an educational content, as being the province of the School Health Service, and those which have not, which are the province of the general practitioner. Would Dr. Didsbury care to say which health problems have no effect on education ? 3. Many people agree that conferences between teacher, doctor, and nurse about health problems are desirable. Does Dr. Didsbury feel that, in every school, the persons concerned would be equally able and willing to act in this way ? If he has found some sure way of expediting the process, perhaps he would let us into the secret ? 4. He makes great play with the unprofitable use of the school medical officer’s time in examining children who wish to take part-time employment. Is he not aware, whatever P.S.M.o.s may think of the need, that many local education authorities make a medical certificate a condition of employ- ment, and that this is almost bound to be so in view of the wording of Section 59 of the Education Act, 1944 ? Incidentally, if he proposes to select some children for examination in this way, who is going to select them and what criteria are to be used ? 5. A table is given of defects found to require treatment, based on national figures from " The Health of the School Child ", 1960-61, with the purpose of showing what a small proportion of the child population has defects. He has omitted to mention that the next table in the same publication lists twice as manv defects found and requiring observation. It 1. Campbell, A. M. G., Clifton, F. Brain, 1950, 73, 281. 2. Muckle, T. J., Wells, M. Quart. J. Med. 1962, 31, 235. might well be agreed, for example, that a psychological defect would be more likely to be recorded in the observation than in the treatment table; and, in fact, this particular defect is four times more numerous in the former than the latter. 6. Does Dr. Didsbury think the general practitioner would have enough time, inclination, and familiarity to deal with minor defects, such as defective colour vision, speech defect, minor hearing-loss, enuresis, and plantar warts, all of which may affect the child’s educational advancement ? We all agree that the general practitioner must increas- ingly concern himself with health problems which have a bearing on the child’s progress at school. As the standard of the health of children continues to improve, general practitioners will have time to concern themselves less with the problems of disease, and more with the promo- tion of individual and community health of school- children. This is always to suppose that the geriatric problem will not have first claim on their attention. HYPOXÆMIA DUE TO ATROPINE SIR,-Dr. Buxton Hopkin (Jan. 11) is mistaken. During the past thirty years atropine has been found responsible for a wide variety of complications before, during, and after surgery. As long ago as 1922 Levy 1 condemned its routine use before the administration of chloroform, because of the danger of increased ventricular irritability; and in 1948 Gillies,2 in a survey of 800,000 patients given chloroform, stated that the incidence of cardiac arrest was twice as great in those patients premedicated with atropine as in those not given atropine. Furthermore, it has been shown that the intravenous injection of atropine during anaesthesia with ether, cyclopropane, halo- thane, and chloroform 3-5 will often provoke ventricular extrasystoles. It has also been known to produce ventricular fibrillation and cardiac arrest.3 Its use as premedication has led to pulmonary oedema in patients with mitral stenosis,6 and to hyperpyrexia and even convulsions, especially in children. In patients with hyper- thyroidism its use is definitely contraindicated because of its tendency to aggravate tachycardia and to increase the metabolic rate. Postoperatively, pulmonary collapse, paralytic ileus, paro- titis,9 and acute glaucoma In have all been attributed to atropine; combined with neostigmine it has produced fatal cardiac arrest." Dr. Buxton Hopkin is equally mistaken about hypoxxmia. Severe hypoxaemia can exist without any clinical signs,12 13 and it is therefore not surprising that he has failed to notice it in his patients. It is true that a fall in oxygen tension of 15 mm. Hg is unlikely to lead to any grave consequences in healthy patients, but there are many whose pre-existing desaturation is such that a slight fall in tension cannot be tolerated. It was for their protec- tion that we recommended that the routine use of atropine should be abandoned. We do not deny that there are specific indications for atropine premedication-e.g., before induction with ether, before electroconvulsive therapy, or before giving 1. Levy, A. G. Chloroform Anæsthesia; p. 36. London, 1922. 2. Gillies, J. Anœsthesia, 1948, 3, 45. 3. Jones, R. E., Deutsch, S., Turndorf, H. Anesthesiology, 1961, 22, 67. 4. Payne, J. P. Proc. R. Soc. Med. 1963, 56, 92. 5. Payne, J. P., Conway, C. M. Brit. J. Anœsth. 1963, 35, 588. 6. Sellick, B. A. Proceedings of World Congress of Anesthesiology, 1956, p. 58. 7. Goodman, L. S., Gilman, A. Pharmacological Basis of Therapeutics; p. 550. New York, 1955. 8. Hewer, C. L. Recent Advances in Anæsthesia and Analgesia; p. 21. London, 1944. 9. Holt, A. T. Lancet, 1962, ii, 984. 10. Gartner, S., Billet, E. Amer. J. Ophthal. 1958, 45, 668. 11. Bain, W. A., Broadbent, K. L. Brit. med. J. 1949, i, 1137. 12. Comroe, J. H., Botelho, S. Amer. J. med. Sci. 1947, 214, 1. 13. Nunn, J. F., Payne, J. P. Lancet, 1962, ii, 632.

Transcript of THE INHERITED AMYLOIDOSES

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eight years out of dental fees alone. Free bone fluoride-filters which can be fitted to taps might be offered tofanatical anti-fluoridators, although I have never yet heardof one who was logical enough to filter the fluoride off hisor her tea. I can think of no happier way of ending ahistoric Parliament.

Beckley, Rye. C. G. LEAROYD.

THE INHERITED AMYLOIDOSES

A. M. G. CAMPBELL.

SIR,-I was very interested -in the paper by Dr. Gafniand his colleagues (Jan. 11).

Their description of these cases bears considerable resem-blance to a family which Clifton and I described in 1950,1 withneurological disturbances which we possibly wrongly attributedto adult toxoplasmosis. In retrospect the rash, joint pains,nerve-deafness, and enlarged spleen appear to suggest amyloiddisease; but our cases had chronic meningoencephalitis,eosinophilia of the blood and cerebrospinal fluid, optic atrophy,hydrocephalus, and (in one at least) calcification of the brain.Although toxoplasmosis was never proved, the cytoplasmmodifying test, serum complement-fixation test, and toxo-

plasmin test were positive in all three cases, which does notprove necessarily that this disease had anything to do with theetiology but is worth considering. It might be of interest toconsider toxoplasmosis in the amyloid cases described byGafni, particularly if it was an immunity disturbance relatedto toxoplasmosis which produced the amyloid infiltration.

Undoubtedly the cases described by Muckle and Wells 2 aresomewhat similar, and possibly the cases that Clifton and Idescribed were a variant of this familial amyloidosis which atthe time we were not aware existed. The exact cause of

amyloid infiltration still remains a mystery.01

Bristol

SCHOOL HEALTH SERVICE AND

FAMILY DOCTOR

A. L. SMALLWOOD.Central Health Clinic,Tower Hill, Bristol, 2.

SIR,-Dr. Didsbury (Jan. 11) reminds us that manylocal-authority medical officers are still working in anoutdated fashion. However, his criticism of the SchoolHealth Service glosses over many long unsolved problems.

1. Dr. Didsbury wishes to educate the parents to assumetheir health responsibilities. Who is to do this and how ? Ourexperience is that those parents in most need are the mostdifficult to approach, let alone teach.

2. There is a distinction between health problems whichhave an educational content, as being the province of the SchoolHealth Service, and those which have not, which are the

province of the general practitioner. Would Dr. Didsbury careto say which health problems have no effect on education ?

3. Many people agree that conferences between teacher,doctor, and nurse about health problems are desirable. DoesDr. Didsbury feel that, in every school, the persons concernedwould be equally able and willing to act in this way ? If he hasfound some sure way of expediting the process, perhaps hewould let us into the secret ?

4. He makes great play with the unprofitable use of theschool medical officer’s time in examining children who wishto take part-time employment. Is he not aware, whateverP.S.M.o.s may think of the need, that many local educationauthorities make a medical certificate a condition of employ-ment, and that this is almost bound to be so in view of the

wording of Section 59 of the Education Act, 1944 ? Incidentally,if he proposes to select some children for examination in this

way, who is going to select them and what criteria are to beused ?

5. A table is given of defects found to require treatment,based on national figures from " The Health of the SchoolChild ", 1960-61, with the purpose of showing what a smallproportion of the child population has defects. He has omittedto mention that the next table in the same publication liststwice as manv defects found and requiring observation. It

1. Campbell, A. M. G., Clifton, F. Brain, 1950, 73, 281.2. Muckle, T. J., Wells, M. Quart. J. Med. 1962, 31, 235.

might well be agreed, for example, that a psychological defectwould be more likely to be recorded in the observation than inthe treatment table; and, in fact, this particular defect is fourtimes more numerous in the former than the latter.

6. Does Dr. Didsbury think the general practitioner wouldhave enough time, inclination, and familiarity to deal withminor defects, such as defective colour vision, speech defect,minor hearing-loss, enuresis, and plantar warts, all of which

may affect the child’s educational advancement ?

We all agree that the general practitioner must increas-ingly concern himself with health problems which have abearing on the child’s progress at school. As the standardof the health of children continues to improve, generalpractitioners will have time to concern themselves lesswith the problems of disease, and more with the promo-tion of individual and community health of school-children. This is always to suppose that the geriatricproblem will not have first claim on their attention.

HYPOXÆMIA DUE TO ATROPINE

SIR,-Dr. Buxton Hopkin (Jan. 11) is mistaken. Duringthe past thirty years atropine has been found responsiblefor a wide variety of complications before, during, andafter surgery.As long ago as 1922 Levy 1 condemned its routine use before

the administration of chloroform, because of the danger ofincreased ventricular irritability; and in 1948 Gillies,2 in asurvey of 800,000 patients given chloroform, stated that theincidence of cardiac arrest was twice as great in those patientspremedicated with atropine as in those not given atropine.Furthermore, it has been shown that the intravenous injectionof atropine during anaesthesia with ether, cyclopropane, halo-thane, and chloroform 3-5 will often provoke ventricular

extrasystoles. It has also been known to produce ventricularfibrillation and cardiac arrest.3

Its use as premedication has led to pulmonary oedema inpatients with mitral stenosis,6 and to hyperpyrexia and evenconvulsions, especially in children. In patients with hyper-thyroidism its use is definitely contraindicated because of itstendency to aggravate tachycardia and to increase the metabolicrate.

Postoperatively, pulmonary collapse, paralytic ileus, paro-titis,9 and acute glaucoma In have all been attributed to atropine;combined with neostigmine it has produced fatal cardiacarrest."

Dr. Buxton Hopkin is equally mistaken about

hypoxxmia. Severe hypoxaemia can exist without anyclinical signs,12 13 and it is therefore not surprising that hehas failed to notice it in his patients. It is true that a fallin oxygen tension of 15 mm. Hg is unlikely to lead to anygrave consequences in healthy patients, but there are

many whose pre-existing desaturation is such that a slightfall in tension cannot be tolerated. It was for their protec-tion that we recommended that the routine use of atropineshould be abandoned.We do not deny that there are specific indications for

atropine premedication-e.g., before induction with ether,before electroconvulsive therapy, or before giving1. Levy, A. G. Chloroform Anæsthesia; p. 36. London, 1922.2. Gillies, J. Anœsthesia, 1948, 3, 45.3. Jones, R. E., Deutsch, S., Turndorf, H. Anesthesiology, 1961, 22, 67.4. Payne, J. P. Proc. R. Soc. Med. 1963, 56, 92.5. Payne, J. P., Conway, C. M. Brit. J. Anœsth. 1963, 35, 588.6. Sellick, B. A. Proceedings of World Congress of Anesthesiology, 1956,

p. 58.7. Goodman, L. S., Gilman, A. Pharmacological Basis of Therapeutics;

p. 550. New York, 1955.8. Hewer, C. L. Recent Advances in Anæsthesia and Analgesia; p. 21.

London, 1944.9. Holt, A. T. Lancet, 1962, ii, 984.

10. Gartner, S., Billet, E. Amer. J. Ophthal. 1958, 45, 668.11. Bain, W. A., Broadbent, K. L. Brit. med. J. 1949, i, 1137.12. Comroe, J. H., Botelho, S. Amer. J. med. Sci. 1947, 214, 1.

13. Nunn, J. F., Payne, J. P. Lancet, 1962, ii, 632.