757
excessive dosage.15 Phytomenadione is (’Konakion’) a synthetic preparation of vitamin K, for oral and parenteral use, the recommended dose for newborns being 1 mg by intramuscular injection. Its effectiveness in correcting a vitamin-Kdependent coagulation-factor deficiency in a matter of hours is beyond dispute but arguments about its prophylactic use in all babies at birth will continue while the incidence of haemorrhagic disease remains low. An aspect commonly overlooked is that of cost, this one small item of routine prevention costing about 100 000 in materials alone in the U.K. A rational compromise would be to ensure selective prevention of hsemorrhagic disease in all those at risk-namely, preterm and low-birthweight infants, those who have had difficult births, those destined for early discharge or to be wholly breast fed, those with inadequate intakes or on antibiotics, those with evidence of bruising or bleeding, and those needing surgery. A programme of selective vitamin-K prophylaxis should appeal to those clinicians who rightly question the wisdom of injecting the majority of normal babies unnecesdue
tainly
to
sarily. SELF-MONITORING OF BLOOD-GLUCOSE IN
an era
of elaborate electronic and mechanical
devices, revolutionary yet simple techniques may be under-rated. Such was the case with ’Dextrostix’, the glucose-oxidase-based reagent strip for measuring bloodglucose launched by Ames in 1964; it caused hardly a stir. To ease reading of the colour of the strip, Ames then introduced a reflectance meter, but reaction was again muted. Critics complained of inaccuracy and instability, so this device was replaced in 1974 by the more accurate but less portable ’Eyetone’ meter; a similar combination of reagent strips and meter, the ’Reflotest’ system, was introduced by Boehringer. Yet, despite the simplicity, cheapness, speed, and accuracy of these methods-they measure a blood-glucose within 90 seconds, can be as accurate as standard laboratory methods, yet cost less than lOp a time-they have not become a popular method of blood-glucose analysis. Few general practitioners do their own measurements, though the advantages to patient and health service are obvious. This week’s Lancet has two papers about an application of these sticks and meters which is perhaps even more useful-self-monitoring of blood-glucose by diabetic patients as a means to better diabetic control. Diabetics have traditionally been taught to "balance" their insulin dose by measurement of urine glucose, but the renal threshold for glucose has made this difficult. The average diabetic has been faithfully testing and charting his urine for years, mainly to please his doctor and partly, perhaps, because he believes that this is the punishment he is given for contracting diabetes. In reality he, like the patient cited by Dr Sonksen and his colleagues, finds that regulating his insulin dose on the T. C., Angus, J. Archs Dis. Childh. 1956, 31, 212. 1. West, T. E. T., Judd, S. L., Sönksen, P. H. Diabete Metab. 1977, 3, 165.
15. Meyer,
is like driving a car in a 30 m.p.h. restricted area when its speedometer does not read below 70 m.p.h. How can urine testing possibly be useful? If the blood-glucose is to be kept anywhere near the normal range there will be no glycosuria. How then is the diabetic best able to tell whether his blood-glucose is 1 or 10 mmol/1? Obviously by measuring it rather than by guessing. The St. Thomas’ and Nottingham groups both found that self-monitoring of blood-glucose enabled patients to adjust the balance of short and intermediate insulins to reduce or eliminate swings in bloodsugar. The day profiles collected by the Nottingham workers indicate that postprandial peaks of hyperglycaemia can virtually be eliminated on conventional regimens of twice-daily soluble and isophane insulin. Even though the average blood-sugars were lower, hypoglycaemic attacks were less frequent-presumably because swings of blood-sugar were less. 70% of patients preferred blood tests to urine tests. If one accepts that good diabetic control is not only a "good thing" but can delay or prevent diabetic complications-and the experimental evidence is now strongly in favour of this-then patients should wherever possible be taught to adjust their insulin dose on the basis of blood rather than urine glucose measurements. This means that reagent strips must be made available to patients on prescription from their general practitioner, and a suitable meter produced which is cheaper and more appropriate for home use than the existing two mains-operated machines.
results of his urine
tests
LIPID-STORAGE MYOPATHIES THE lipid-storage disorders, an indispensible part of every medical student’s list of differential diagnoses, involve the accumulation in lysosomes of complex lipids
such as sphingomyelin (in Niemann-Pick disease) or glucocerebroside (in Gaucher’s disease). In each case a hydrolytic enzyme is lacking, required for further metabolism. A new group of lipid-storage disorders has lately been recognised which involves cellular accumulation of neutral fat, the common triglyceride.1,2 Skeletal muscle is the tissue most obviously affected. The material accumulates not in any cell organelle but in cytoplasmic vacuoles, and these vacuoles take up dyes such as oilred-O which are soluble in neutral fat. The patients are usually children or young adults. Of 13 cases 8 were women and 5 men, and the commonest complaint was of muscle weakness in the arms and legs and easy fatiguability ; some had muscle pain or cramps.l,3-14 At least 4 patients had myoglobinuria.4,s.lo Serum-creatine-phosphokinase was abnormal in less than half the patients Engel, A. G., Siekert, R. G. Archs Neurol. 1972, 27, 174. Chanarin, I., Patel, A., Slavin, G., Wills, E. J., Andrews, T. M., Stewart, G. Br. med. J. 1975, i, 553. 3. Bradley, W. G., Hudgson, P., Gardner-Medwin, D., Walton, J. N. Lancet, 1. 2.
1969, i, 495. W. K., Vick, N. A., Glueck, C. J., Levy, R. L. New Engl. J. Med. 1970, 282, 697. 5. Dimauro, S., Dimauro, P. M. M. Science, 1973, 182, 929. 6. Johnson, M. A., Fulthorpe, J. J., Hudgson, P. Acta neuropath. 1973, 24, 97. 7. Engel, A. G., Angelini, C. Science, 1973, 179, 899. 8. Jerusalem, F., Spiess, H., Baumgartner, G. J. neurol. Sci. 1975, 24, 273. 9. Markesbery, W. R., McQuillen, P., Procopis, P. G., Harrison, A. R., Engel, A. G. Archs Neurol. 1974, 31, 320. 10. Bank, W. J., DiMauro, S., Bonilla, E., Capuzzi, D. M., Rowland, L. P. New Engl. J. Med. 1975, 292, 443. 4.
Engel,
excessive dosage.15 Phytomenadione is (’Konakion’) a synthetic preparation of vitamin K, for oral and parenteral use, the recommended dose for newborns being 1 mg by intramuscular injection. Its effectiveness in correcting a vitamin-Kdependent coagulation-factor deficiency in a matter of hours is beyond dispute but arguments about its prophylactic use in all babies at birth will continue while the incidence of haemorrhagic disease remains low. An aspect commonly overlooked is that of cost, this one small item of routine prevention costing about 100 000 in materials alone in the U.K. A rational compromise would be to ensure selective prevention of hsemorrhagic disease in all those at risk-namely, preterm and low-birthweight infants, those who have had difficult births, those destined for early discharge or to be wholly breast fed, those with inadequate intakes or on antibiotics, those with evidence of bruising or bleeding, and those needing surgery. A programme of selective vitamin-K prophylaxis should appeal to those clinicians who rightly question the wisdom of injecting the majority of normal babies unnecesdue
tainly
to
sarily. SELF-MONITORING OF BLOOD-GLUCOSE IN
an era
of elaborate electronic and mechanical
devices, revolutionary yet simple techniques may be under-rated. Such was the case with ’Dextrostix’, the glucose-oxidase-based reagent strip for measuring bloodglucose launched by Ames in 1964; it caused hardly a stir. To ease reading of the colour of the strip, Ames then introduced a reflectance meter, but reaction was again muted. Critics complained of inaccuracy and instability, so this device was replaced in 1974 by the more accurate but less portable ’Eyetone’ meter; a similar combination of reagent strips and meter, the ’Reflotest’ system, was introduced by Boehringer. Yet, despite the simplicity, cheapness, speed, and accuracy of these methods-they measure a blood-glucose within 90 seconds, can be as accurate as standard laboratory methods, yet cost less than lOp a time-they have not become a popular method of blood-glucose analysis. Few general practitioners do their own measurements, though the advantages to patient and health service are obvious. This week’s Lancet has two papers about an application of these sticks and meters which is perhaps even more useful-self-monitoring of blood-glucose by diabetic patients as a means to better diabetic control. Diabetics have traditionally been taught to "balance" their insulin dose by measurement of urine glucose, but the renal threshold for glucose has made this difficult. The average diabetic has been faithfully testing and charting his urine for years, mainly to please his doctor and partly, perhaps, because he believes that this is the punishment he is given for contracting diabetes. In reality he, like the patient cited by Dr Sonksen and his colleagues, finds that regulating his insulin dose on the T. C., Angus, J. Archs Dis. Childh. 1956, 31, 212. 1. West, T. E. T., Judd, S. L., Sönksen, P. H. Diabete Metab. 1977, 3, 165.
15. Meyer,
is like driving a car in a 30 m.p.h. restricted area when its speedometer does not read below 70 m.p.h. How can urine testing possibly be useful? If the blood-glucose is to be kept anywhere near the normal range there will be no glycosuria. How then is the diabetic best able to tell whether his blood-glucose is 1 or 10 mmol/1? Obviously by measuring it rather than by guessing. The St. Thomas’ and Nottingham groups both found that self-monitoring of blood-glucose enabled patients to adjust the balance of short and intermediate insulins to reduce or eliminate swings in bloodsugar. The day profiles collected by the Nottingham workers indicate that postprandial peaks of hyperglycaemia can virtually be eliminated on conventional regimens of twice-daily soluble and isophane insulin. Even though the average blood-sugars were lower, hypoglycaemic attacks were less frequent-presumably because swings of blood-sugar were less. 70% of patients preferred blood tests to urine tests. If one accepts that good diabetic control is not only a "good thing" but can delay or prevent diabetic complications-and the experimental evidence is now strongly in favour of this-then patients should wherever possible be taught to adjust their insulin dose on the basis of blood rather than urine glucose measurements. This means that reagent strips must be made available to patients on prescription from their general practitioner, and a suitable meter produced which is cheaper and more appropriate for home use than the existing two mains-operated machines.
results of his urine
tests
LIPID-STORAGE MYOPATHIES THE lipid-storage disorders, an indispensible part of every medical student’s list of differential diagnoses, involve the accumulation in lysosomes of complex lipids
such as sphingomyelin (in Niemann-Pick disease) or glucocerebroside (in Gaucher’s disease). In each case a hydrolytic enzyme is lacking, required for further metabolism. A new group of lipid-storage disorders has lately been recognised which involves cellular accumulation of neutral fat, the common triglyceride.1,2 Skeletal muscle is the tissue most obviously affected. The material accumulates not in any cell organelle but in cytoplasmic vacuoles, and these vacuoles take up dyes such as oilred-O which are soluble in neutral fat. The patients are usually children or young adults. Of 13 cases 8 were women and 5 men, and the commonest complaint was of muscle weakness in the arms and legs and easy fatiguability ; some had muscle pain or cramps.l,3-14 At least 4 patients had myoglobinuria.4,s.lo Serum-creatine-phosphokinase was abnormal in less than half the patients Engel, A. G., Siekert, R. G. Archs Neurol. 1972, 27, 174. Chanarin, I., Patel, A., Slavin, G., Wills, E. J., Andrews, T. M., Stewart, G. Br. med. J. 1975, i, 553. 3. Bradley, W. G., Hudgson, P., Gardner-Medwin, D., Walton, J. N. Lancet, 1. 2.
1969, i, 495. W. K., Vick, N. A., Glueck, C. J., Levy, R. L. New Engl. J. Med. 1970, 282, 697. 5. Dimauro, S., Dimauro, P. M. M. Science, 1973, 182, 929. 6. Johnson, M. A., Fulthorpe, J. J., Hudgson, P. Acta neuropath. 1973, 24, 97. 7. Engel, A. G., Angelini, C. Science, 1973, 179, 899. 8. Jerusalem, F., Spiess, H., Baumgartner, G. J. neurol. Sci. 1975, 24, 273. 9. Markesbery, W. R., McQuillen, P., Procopis, P. G., Harrison, A. R., Engel, A. G. Archs Neurol. 1974, 31, 320. 10. Bank, W. J., DiMauro, S., Bonilla, E., Capuzzi, D. M., Rowland, L. P. New Engl. J. Med. 1975, 292, 443. 4.
Engel,
