300
fusion dependent, but some of the explanation may be that the enzyme method is related to the mass of muscle involved whereas the isotope method is more related to the area of infarcted muscle. The scintillation camera can only view a three-dimensional object in a two-dimensional plane. The field of view and resolution of the camera vary with depth, and the attenuation of the gamma radiation in tissues allows only a qualitative relationship between image and the distribution of activity in the myocardium.2 Originally the main aim of infarct size was to determine whether treatment in the first few hours could limit subsequent damage, but positive scans develop too slowly for this. Moreover, the long half-life of 99mTC (6 h) limits the frequency of repeat studies. Myocardial scintigraphy can be used to localise the site of infarction, particularly when electrocardiographic abnormalities such as left-bundlebranch block make diagnosis difficult. It may be helpful in reinfarction or infarction after cardiac surgery. Right-ventricular infarction can be diagnosed more easily by scintigraphy than by any other method.19 In most other patients the diagnosis of myocardial infarction is easily confirmed from E.C.G. and serum-enzyme changes. Myocardial scintigraphy does not yet provide information which is essential for management of patients with infarction. However, it has contributed to understanding of the pathogenesis of ischsemia and infarction. Radio-iodinated antibody specific for cardiac myosin,2o neutrally charged liposomes,21 three-dimensional imaging with positron emitters,22 and advances in computerised axial tomography23 may all enable us to assess much more accurately the extent and location of myocardial
damage.
Water with
Sugar and Salt
discovery that sodium transport and glucose transport are coupled in the small intestine, so that glucose accelerates absorption of solute and water, was potentially the most important medical advance this century.It opened the way to oral hydration treatment for severe diarrhœa—the main cause of infant death in the developing world. HIRSCHHORN and others2 showed that an oral gluTHE
Sharpe, D. N., Botvinick, E. H., Shames, D. M., Schiller, N. B., Massie, B. M., Chatterjee, K., Parmley, W. W. Circulation, 1978, 57, 483. 20. Khaw, B. A., Beller, G. A., Haber, E. ibid. p. 743. 21. Caride, V. J., Zaret, B. L. Clin Res. 1977, 25, 211A. 22. Weiss, E. S., Ahmed, S. A., Welsh, M. J., Williamson, J. R., Ter-Pogossian, M. M., Sobel, B. E. Circulation, 1977, 55, 66. 23. Powell, W. J. Jr., Wittenberg, J., Maturi, R. A., Dinsmore, R. E., Miller, S. W. ibid. p. 99. 1. See Lancet, 1975, i, 79. 2. Hirschhorn, N., Kinzie, J. L., Sachar, D. B., Northrup, R. S., Taylor, J. O., Ahmed, S. F., Phillips, R. A. New Engl. J. Med. 1968, 279, 176. 19.
cose electrolyte solution could replace intravenous fluids for cholera, and similar solutions have proved equally useful in enterotoxigenic Escherichia coli enteritis and other forms of infective diarrhoea.3Three years ago the main obstacles to wide application seemed to be practical. How might these oral glucose electrolyte preparations be brought into remote homes and health centres? But there were other questions: would a single fluid do for all forms of diarrhoea in all age-groups? For maintenance of hydration, as opposed to rehydration, would it be permissible to leave out the potassium and bicarbonate (the most perishable ingredients) and treat the patient simply with salt and glucose-or even better, salt and sucrose? Some answers have emerged. Controlled trials in London have shown that, in mild diarrhoeal disease, oral sucrose electrolyte solution is as effective as oral glucose electrolyte solution. 4 5 probably many of these patients were infected with rotaviruses.In piglets DAVIDSON and others detected abnormalities of carbohydrate handling in viral diarrhoea -which are absent in toxigenic diarrhoea. Could these interfere with sugar absorption and with the splitting of sucrose into glucose and fructose, rendering the oral solution less effective? Dr SACK and his co-workers address this question on p. 280 this week, and the answer is broadly no. In Bangladeshi children with rotavirus diarrhoea, oral sucrose was as good as oral glucose. What is more, despite the lower sodium losses in rotavirus infection than in toxigenic diarrhoeas, no patient became hypernatraemic on the standard World Health Organisation-style electrolyte regimen (perhaps because additional fluids were allowed). On p. 277 Dr NALIN and his co-workers report another comparison of glucose and sucrose in oral solutions, this time in various kinds of diarrhoea in Costa Ricans. Glucose had a distinct edge, but not a crucial one. Ideally, every household would have a few packets of glucose electrolyte powder and a measuring vessel for mixing it with water. This ideal is far from reality. Apart from the expense and perishability of existing preparations (such as W.H.O. glucose electrolyte powder), there is great difficulty in getting them into people’s homes. Health centres can cope with them, or can stock and measure out the separate ingredients.8 But the first object is to
3. Hirschhorn, N., McCarthy, B. J., Ranney, B., Hirschhorn, M. A., Woodward, S. T., Lacapa, A., Cash, R. A., Woodward, W. E. J. pediat. 1973,
5.
83, 562. Rahilly, P. M., Shepherd, R., Challis, D., Walker-Smith, J. A., Manly, J. Archs Dis. Childh. 1976, 51, 152. Hutchins, P. M., Matthews, T., Lawrie, B., Manly, J., Walker-Smith, J. A.
6.
Bishop,
4.
Lancet, 1978, i, 1211. R.
F., Davidson, G. P., Holmes, I. H., Ruck, B. J. ibid. 1973, ii,
1497.
7. 8.
Davidson, G. P., Gall, D. G., Petrii, M., Butler, clin. Invest. 1977, 60, 1402. Morley, D., King, M. Lancet, 1978, i, 53.
D.
G., Hamilton, J. R. J.
301
prevent dehydration, and with early
in the home refinements may prove unnecessary. What is the risk of acidosis9 and hypokalaemia if potassium and bicarbonate are omitted? More information is needed on this. The best means of bringing oral therapy to most of the people may well be salt, sugar, some special spoons,and a plastic bag10 in every home. To prevent accidents a
good maxim is "taste it, salty than tears".
and don’t
treatment
give anything
more
(V.U.R.
I.R.R.) + U.T.I. C.P.N. IN 1974 an editorial" entitled V.U.R.+I.R.R.=C.P.N.? +
=
important concept, based on clinical and experimental work,12 13 that the segmental scar of chronic pyelonephritis is related to calico-tubular backflow (intrarenal reflux) during vesico-ureteric reflux. Ransley and Risdon14 had just reported their work in pigs indicating a morphological explanation for I.R.R. Renal scars, they proposed, arise only in those areas of the kidney with compound papillae shaped in such a way as to allow intrarenal reflux. These early observations have been amplified15-17 and confirmed;18 and now Ransley and Risdon’9 report a long-term study in pigs which may well complete the equation. Their work suggests, firstly, that the segmental scars of reflux nephropathy arise exclusively in relation to the refluxing papillae and, furthermore, that this happens only when the urine is infected. There was no scarring in any of the animals with intra-
discussed the
renal reflux of sterile urine. This is a controversial observation since some workers still doubt the importance of urinary infection in the alone be genesis of renal scarring; might not sterile i.R.R. sufficient to produce a pyelonephritic scar?12 20 21 There is great difficulty in acquiring reliable clinical evidence on this point. Most patients with reflux nephropathy already have renal scars when first seen and the finding of a sterile urine at this time does not mean that infection has never occurred. Radiological progression of an established scar in the presence of sterile urine may simply reflect scar contraction with hypertrophy or normal growth of the surrounding parenchyma. Increase in pelvi-calyceal dilatation of the generalised "back-pressure" type is an entirely separate entity and should not be lumped together with the true segmental lesion. The of a completely new scar in a previously norkidney with continuously sterile urine has not been reported. The results of Ransley and Risdon’s carefully controlled study now argue strongly in favour of the prime importance of infected I.R.R. in the pathogenesis of reflux nephropathy. They propose a new hypothesis
occurrence
mal
9. Mosley, W. H. ibid. p. 991. 10. Nalin, D. R. ibid. July 29, 1978, p. 264. 11. Lancet, 1974, ii, 1120. 12. Rolleston, G. L., Maling, T. M. J., Hodson, C. J. Archs. Dis. Childh. 1974,
that infection alone
alone will not and v.u.R. Infection produce pyelonephritic is intrarenal if there but reflux. do only together will so, that up to two Analyses of papillary morphology suggest one or more children’s kidneys out of three will contain papillae which allow I.R.R.,16 18 and in the appropriate circumstances of v.u.R. plus infection the extent of scarring
which
states
or v.u.R.
scars.
depend on the innate papillary population. Conversely, one-third of kidneys do not possess any papillae will
which would allow I.R.R. and therefore should remain unscarred despite the combined insult of v.u.R. and urinary infection. Sometimes scarring seems to take place remarkably quickly,12 19 and in a child with vesico-ureteric reflux and a susceptible kidney the kidney may well be damaged by the first infection. If so, then the prospects for preventive treatment are extremely limited. This outline does not account for those very rare but undoubted cases where scars arise in response to repeated urinary infection.22 Nor may scar contraction and parenchymal growth account entirely for apparently new scars or scar progression; an additional mechanism must operate. Ransley and Risdon have provided evidence that infected reflux may so modify the morphology of some apparently non-refluxing papillae that they allow I.R.R. and so permit progressive scarring-a notion which has had radiological support from Hodson.23 This papillary transformation may account for the rare occurrence of new segmental scars, or sequential scarring of an already scarred kidney. But the risk of late additional scarring will usually be too small to justify reimplantation of the ureter simply because a kidney is scarred.
SPASMODIC TORTICOLLIS Mon pauvre corps est raccourci, Et j’ai la tete sur l’oreille; Mais cela me sied a merveille, Et parmi les torticolis Je passe pour des plus jolis.1
SPASMODIC torticollis (wry neck) is a rare disorder characterised by involuntary movements of the cervical musculature resulting in tonic deviation of the head and neck. Ancient writings referred to the caput obstipum, probably an early observation of torticollis, while dystonic twisting of the neck has also been described in fossil dinosaurs.2 Rabelais is believed to have first used the term "torty colly". This disorder, beginning insidiously in the fourth or fifth decades of life, presents with jerking movements of the head and painful spasm of the neck muscles. Both sexes are equally affected.3 Familial cases occasionally occur4 and an association with both benign essential tremor5 and Parkinson’s diseasehas been noted. Spasmodic torticollis is now generally regarded as a variety of segmental dystonia,’often
49, 531. 13. Hodson, C. J., Maling, T. M. J., McManamon, P. J., Lewis, M. G. Radiol. 1975, suppl. 13. 14. Ransley, P. G., Risdon, R. A. Lancet, 1974, ii, 1114. 15. Ransley, P. G., Risdon, R. A. Urol. Res. 1975, 3, 105. 16. Ransley, P. G., Risdon, R. A. ibid. p. 111. 17. Ransley, P. G. ibid. 1977, 5, 61. 18. Tamminen, T. E., Kaprio, E. A. Br. J. Urol. 1977, 49, 345. 19. Ransley, P. G., Risdon, R. A. Br. J. Radiol. 1978, suppl. 14. 20. Bailey, R. R. Clin. Nephrol. 1973, 1, 132. 21. Bailey, R. R. Archs Dis. Childh. 1977, 52, 804.
Br. J. 22. Smellie, J. M., Normand, I. C. S. ibid. 1975, 50, 581. 23. Hodson, C. J. I.P.N.A. meeting, Helsinki, 1977. 1. 17th century poet, quoted by S. A. K. Wilson, Brain, 1907, 30, 398. 2. Kaiser, H. E. Zentbl. allg. Path. Anat. 1954, 91, 196. 3. Herz, E., Glaser, G. H. Archs Neurol. Psychiat. 1949, 61, 227. 4. Gilbert, G. J. Neurology, 1977, 27, 11. 5. Couch, J. R. Trans. Am. neurol. Ass. 1975, 100, 181. 6. Wilson, S. A. K. in Neurology; p. 132. London, 1954. 7. Cooper, I. S. New Engl. J. Med. 1964, 270, 967.
fusion dependent, but some of the explanation may be that the enzyme method is related to the mass of muscle involved whereas the isotope method is more related to the area of infarcted muscle. The scintillation camera can only view a three-dimensional object in a two-dimensional plane. The field of view and resolution of the camera vary with depth, and the attenuation of the gamma radiation in tissues allows only a qualitative relationship between image and the distribution of activity in the myocardium.2 Originally the main aim of infarct size was to determine whether treatment in the first few hours could limit subsequent damage, but positive scans develop too slowly for this. Moreover, the long half-life of 99mTC (6 h) limits the frequency of repeat studies. Myocardial scintigraphy can be used to localise the site of infarction, particularly when electrocardiographic abnormalities such as left-bundlebranch block make diagnosis difficult. It may be helpful in reinfarction or infarction after cardiac surgery. Right-ventricular infarction can be diagnosed more easily by scintigraphy than by any other method.19 In most other patients the diagnosis of myocardial infarction is easily confirmed from E.C.G. and serum-enzyme changes. Myocardial scintigraphy does not yet provide information which is essential for management of patients with infarction. However, it has contributed to understanding of the pathogenesis of ischsemia and infarction. Radio-iodinated antibody specific for cardiac myosin,2o neutrally charged liposomes,21 three-dimensional imaging with positron emitters,22 and advances in computerised axial tomography23 may all enable us to assess much more accurately the extent and location of myocardial
damage.
Water with
Sugar and Salt
discovery that sodium transport and glucose transport are coupled in the small intestine, so that glucose accelerates absorption of solute and water, was potentially the most important medical advance this century.It opened the way to oral hydration treatment for severe diarrhœa—the main cause of infant death in the developing world. HIRSCHHORN and others2 showed that an oral gluTHE
Sharpe, D. N., Botvinick, E. H., Shames, D. M., Schiller, N. B., Massie, B. M., Chatterjee, K., Parmley, W. W. Circulation, 1978, 57, 483. 20. Khaw, B. A., Beller, G. A., Haber, E. ibid. p. 743. 21. Caride, V. J., Zaret, B. L. Clin Res. 1977, 25, 211A. 22. Weiss, E. S., Ahmed, S. A., Welsh, M. J., Williamson, J. R., Ter-Pogossian, M. M., Sobel, B. E. Circulation, 1977, 55, 66. 23. Powell, W. J. Jr., Wittenberg, J., Maturi, R. A., Dinsmore, R. E., Miller, S. W. ibid. p. 99. 1. See Lancet, 1975, i, 79. 2. Hirschhorn, N., Kinzie, J. L., Sachar, D. B., Northrup, R. S., Taylor, J. O., Ahmed, S. F., Phillips, R. A. New Engl. J. Med. 1968, 279, 176. 19.
cose electrolyte solution could replace intravenous fluids for cholera, and similar solutions have proved equally useful in enterotoxigenic Escherichia coli enteritis and other forms of infective diarrhoea.3Three years ago the main obstacles to wide application seemed to be practical. How might these oral glucose electrolyte preparations be brought into remote homes and health centres? But there were other questions: would a single fluid do for all forms of diarrhoea in all age-groups? For maintenance of hydration, as opposed to rehydration, would it be permissible to leave out the potassium and bicarbonate (the most perishable ingredients) and treat the patient simply with salt and glucose-or even better, salt and sucrose? Some answers have emerged. Controlled trials in London have shown that, in mild diarrhoeal disease, oral sucrose electrolyte solution is as effective as oral glucose electrolyte solution. 4 5 probably many of these patients were infected with rotaviruses.In piglets DAVIDSON and others detected abnormalities of carbohydrate handling in viral diarrhoea -which are absent in toxigenic diarrhoea. Could these interfere with sugar absorption and with the splitting of sucrose into glucose and fructose, rendering the oral solution less effective? Dr SACK and his co-workers address this question on p. 280 this week, and the answer is broadly no. In Bangladeshi children with rotavirus diarrhoea, oral sucrose was as good as oral glucose. What is more, despite the lower sodium losses in rotavirus infection than in toxigenic diarrhoeas, no patient became hypernatraemic on the standard World Health Organisation-style electrolyte regimen (perhaps because additional fluids were allowed). On p. 277 Dr NALIN and his co-workers report another comparison of glucose and sucrose in oral solutions, this time in various kinds of diarrhoea in Costa Ricans. Glucose had a distinct edge, but not a crucial one. Ideally, every household would have a few packets of glucose electrolyte powder and a measuring vessel for mixing it with water. This ideal is far from reality. Apart from the expense and perishability of existing preparations (such as W.H.O. glucose electrolyte powder), there is great difficulty in getting them into people’s homes. Health centres can cope with them, or can stock and measure out the separate ingredients.8 But the first object is to
3. Hirschhorn, N., McCarthy, B. J., Ranney, B., Hirschhorn, M. A., Woodward, S. T., Lacapa, A., Cash, R. A., Woodward, W. E. J. pediat. 1973,
5.
83, 562. Rahilly, P. M., Shepherd, R., Challis, D., Walker-Smith, J. A., Manly, J. Archs Dis. Childh. 1976, 51, 152. Hutchins, P. M., Matthews, T., Lawrie, B., Manly, J., Walker-Smith, J. A.
6.
Bishop,
4.
Lancet, 1978, i, 1211. R.
F., Davidson, G. P., Holmes, I. H., Ruck, B. J. ibid. 1973, ii,
1497.
7. 8.
Davidson, G. P., Gall, D. G., Petrii, M., Butler, clin. Invest. 1977, 60, 1402. Morley, D., King, M. Lancet, 1978, i, 53.
D.
G., Hamilton, J. R. J.
301
prevent dehydration, and with early
in the home refinements may prove unnecessary. What is the risk of acidosis9 and hypokalaemia if potassium and bicarbonate are omitted? More information is needed on this. The best means of bringing oral therapy to most of the people may well be salt, sugar, some special spoons,and a plastic bag10 in every home. To prevent accidents a
good maxim is "taste it, salty than tears".
and don’t
treatment
give anything
more
(V.U.R.
I.R.R.) + U.T.I. C.P.N. IN 1974 an editorial" entitled V.U.R.+I.R.R.=C.P.N.? +
=
important concept, based on clinical and experimental work,12 13 that the segmental scar of chronic pyelonephritis is related to calico-tubular backflow (intrarenal reflux) during vesico-ureteric reflux. Ransley and Risdon14 had just reported their work in pigs indicating a morphological explanation for I.R.R. Renal scars, they proposed, arise only in those areas of the kidney with compound papillae shaped in such a way as to allow intrarenal reflux. These early observations have been amplified15-17 and confirmed;18 and now Ransley and Risdon’9 report a long-term study in pigs which may well complete the equation. Their work suggests, firstly, that the segmental scars of reflux nephropathy arise exclusively in relation to the refluxing papillae and, furthermore, that this happens only when the urine is infected. There was no scarring in any of the animals with intra-
discussed the
renal reflux of sterile urine. This is a controversial observation since some workers still doubt the importance of urinary infection in the alone be genesis of renal scarring; might not sterile i.R.R. sufficient to produce a pyelonephritic scar?12 20 21 There is great difficulty in acquiring reliable clinical evidence on this point. Most patients with reflux nephropathy already have renal scars when first seen and the finding of a sterile urine at this time does not mean that infection has never occurred. Radiological progression of an established scar in the presence of sterile urine may simply reflect scar contraction with hypertrophy or normal growth of the surrounding parenchyma. Increase in pelvi-calyceal dilatation of the generalised "back-pressure" type is an entirely separate entity and should not be lumped together with the true segmental lesion. The of a completely new scar in a previously norkidney with continuously sterile urine has not been reported. The results of Ransley and Risdon’s carefully controlled study now argue strongly in favour of the prime importance of infected I.R.R. in the pathogenesis of reflux nephropathy. They propose a new hypothesis
occurrence
mal
9. Mosley, W. H. ibid. p. 991. 10. Nalin, D. R. ibid. July 29, 1978, p. 264. 11. Lancet, 1974, ii, 1120. 12. Rolleston, G. L., Maling, T. M. J., Hodson, C. J. Archs. Dis. Childh. 1974,
that infection alone
alone will not and v.u.R. Infection produce pyelonephritic is intrarenal if there but reflux. do only together will so, that up to two Analyses of papillary morphology suggest one or more children’s kidneys out of three will contain papillae which allow I.R.R.,16 18 and in the appropriate circumstances of v.u.R. plus infection the extent of scarring
which
states
or v.u.R.
scars.
depend on the innate papillary population. Conversely, one-third of kidneys do not possess any papillae will
which would allow I.R.R. and therefore should remain unscarred despite the combined insult of v.u.R. and urinary infection. Sometimes scarring seems to take place remarkably quickly,12 19 and in a child with vesico-ureteric reflux and a susceptible kidney the kidney may well be damaged by the first infection. If so, then the prospects for preventive treatment are extremely limited. This outline does not account for those very rare but undoubted cases where scars arise in response to repeated urinary infection.22 Nor may scar contraction and parenchymal growth account entirely for apparently new scars or scar progression; an additional mechanism must operate. Ransley and Risdon have provided evidence that infected reflux may so modify the morphology of some apparently non-refluxing papillae that they allow I.R.R. and so permit progressive scarring-a notion which has had radiological support from Hodson.23 This papillary transformation may account for the rare occurrence of new segmental scars, or sequential scarring of an already scarred kidney. But the risk of late additional scarring will usually be too small to justify reimplantation of the ureter simply because a kidney is scarred.
SPASMODIC TORTICOLLIS Mon pauvre corps est raccourci, Et j’ai la tete sur l’oreille; Mais cela me sied a merveille, Et parmi les torticolis Je passe pour des plus jolis.1
SPASMODIC torticollis (wry neck) is a rare disorder characterised by involuntary movements of the cervical musculature resulting in tonic deviation of the head and neck. Ancient writings referred to the caput obstipum, probably an early observation of torticollis, while dystonic twisting of the neck has also been described in fossil dinosaurs.2 Rabelais is believed to have first used the term "torty colly". This disorder, beginning insidiously in the fourth or fifth decades of life, presents with jerking movements of the head and painful spasm of the neck muscles. Both sexes are equally affected.3 Familial cases occasionally occur4 and an association with both benign essential tremor5 and Parkinson’s diseasehas been noted. Spasmodic torticollis is now generally regarded as a variety of segmental dystonia,’often
49, 531. 13. Hodson, C. J., Maling, T. M. J., McManamon, P. J., Lewis, M. G. Radiol. 1975, suppl. 13. 14. Ransley, P. G., Risdon, R. A. Lancet, 1974, ii, 1114. 15. Ransley, P. G., Risdon, R. A. Urol. Res. 1975, 3, 105. 16. Ransley, P. G., Risdon, R. A. ibid. p. 111. 17. Ransley, P. G. ibid. 1977, 5, 61. 18. Tamminen, T. E., Kaprio, E. A. Br. J. Urol. 1977, 49, 345. 19. Ransley, P. G., Risdon, R. A. Br. J. Radiol. 1978, suppl. 14. 20. Bailey, R. R. Clin. Nephrol. 1973, 1, 132. 21. Bailey, R. R. Archs Dis. Childh. 1977, 52, 804.
Br. J. 22. Smellie, J. M., Normand, I. C. S. ibid. 1975, 50, 581. 23. Hodson, C. J. I.P.N.A. meeting, Helsinki, 1977. 1. 17th century poet, quoted by S. A. K. Wilson, Brain, 1907, 30, 398. 2. Kaiser, H. E. Zentbl. allg. Path. Anat. 1954, 91, 196. 3. Herz, E., Glaser, G. H. Archs Neurol. Psychiat. 1949, 61, 227. 4. Gilbert, G. J. Neurology, 1977, 27, 11. 5. Couch, J. R. Trans. Am. neurol. Ass. 1975, 100, 181. 6. Wilson, S. A. K. in Neurology; p. 132. London, 1954. 7. Cooper, I. S. New Engl. J. Med. 1964, 270, 967.
