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ADDICTION NEW STYLE
1433 In our view, most forms of idiopathic kidney failure, liver disease, and heart-disease that call for organ transplantation are likely to be autoaggressive (or so-called" disturbed-tolerance autoimmune ") in aetiology. Organ failure results from the
formation and proliferation of one or more " forbidden clones " of cells synthesising mutant m.c.p.s complementary to self T.C.F.S. Suppose antisera specific for cells producing mutant Ht.c.P.s could be prepared. Such antisera might then be used to supplement the endogenous defence mechanism, and to produce a remission of the primary disease.10 This form of anti-forbidden-clone therapy may well prove to be simpler and more efficacious than suppressing multiple M.c.p.s complementary to not-self T.C.F.S of a homograft. In other words, rational treatment for the original disease, and suppression of homograft rejection, both encounter analogous immunological
problems. The General Infirmary at Leeds. The Royal National Orthopædic Hospital, London W.1.
P. R. J. BURCH N. R. ROWELL.
mind while examining and eye anomalies.
mentally
Complete case-reports will be which Prof. C. E. Dent has kindly Ophthalmic Department, Ipswich and East Suffolk Hospital, Ipswich,
retarded children with bone
published after agreed to do for
Suffolk.
enzyme
studies,
us.
S. K. TALEGAONKAR.
TABLE-TOP FITTING FOR WALKING-AID SIR,-Wards for the care of elderly patients are often crowded, and the shortage of space may be made worse by the active policy of nursing patients out of bed and getting them walking. Chairs, walking-aids, and tables all add to the general
overcrowding. In an effort M. Hurry has
to
reduce the space taken by equipment, Sister a table-top which fits on a standard
developed
R. G. BURWELL.
ADDICTION NEW STYLE
SIR,-Professor Hamilton’s view (May 11, p. 1033) that the physician’s " ethical principles dictate that the patient should be taken off the drug [heroin] as soon as possible " is commendable. Unfortunately the reality situation is such that if an addict is forcibly treated (i.e., withdrawn from his drug willynilly), he will have an overpowering compulsion to seek his supplies elsewhere. This we do not want to happen. We do not want to go through the unfortunate American experience of the burgeoning of a vicious criminal underworld. Furthermore, when we examine the alternative to giving the addict intravenous heroin
we
find that he has
injection six-hourly, out of the question.
seven
Cane Hill Hospital, Coulsdon, Surrey CR3 3YL.
to
days
attend for an intramuscular a week, and this of course is H. DALE BECKETT.
HOMOCYSTINURIA SIR,--Homocystinuria was first described in 1962 by Carson and Neill 12 and Gerritsen et a1.13 and nearly 50 cases have now been reported.14-laThe salient clinical features include dislocation of the lens, mental retardation, skeletal anomalies, and the presence of excessive amounts of homocystine in the urine, owing to deficiencies of cystathionine synthetase, an enzyme responsible for the conversion of homocysteine to cystathionine. Many patients get thromboembolic attacks which are precipitated by surgery. I screened the urine of patients with ectopio lentis attending this department by the nitroprusside test. When this was positive, cysteine and homocysteine were distinguished by chromatography. Mental, ocular, and skeletal evaluations were done and information from previous clinical studies was assembled. Parents and sibs of the affected children were subjected to similar examinations. The diagnosis of homocystinuria was established in 3 patients. All had ectopio lentis, mental retardation, and skeletal anomalies. 1 patient had successful lens extraction with good visual results. 2 of these 3 cases showed curious skeletal anomalies like epiphyseal dysplasia, and bone cysts with pathological fractures. I feel that this disease is not as rare as the number of published reports suggests, since I could find 3 cases in a hospital with an average yearly attendance (ophthalmic cases) of 10,395. If other eye departments undertake a similar study, many more cases may be found. Moreover, paediatricians and orthopa:dic surgeons should also keep the possibility of homocystinuria in 12. Carson, N. A., Neill, D. W. Archs Dis. Childn. 1962, 37, 505. 13.
Gerritsen, T., Vaughan, J. G., Waisman, H. A. Biochem. biophys. Res. Comm. 1962, 9, 493. 14. Komrower, G. M., Wilson, V. K. Proc. R. Soc. Med. 1963, 56, 996. 15. White, H. H., Thompson, H. L., Rowland, L. P. Trans. Am. neurol. Ass. 1964, 89, 24. 16. Arnott, E. J., Greaves, D. P. Br. J. Ophthal. 1964, 48, 688. 17. Carson, N. A. J., Dent, C. E., Field, C. M. J. Pediat. 1965, 66, 565. 18. Schimke, R. N., McKusick, V. A., Huang, T., Pollack, A. D. J. Am. med. Ass. 1965, 193, 711.
Walking-aid table-top.
Zimmer walking-aid. This obviates the need for a separate table. It has the disadvantage of having to be removed before the patient can walk with the aid, but this presents no greater inconvenience than is experienced with the table adjustment for a geriatric chair. This table-top for a walking-aid can be made up easily in an
occupational therapy department. Greenwich District Hospital, London S.E.10.
R. V. BOYD.
METACHROMASIA IN FIBROBLASTS SiR,-We have followed with interest the reports by Danes and Bearn on the detection of metachromasia in fibroblasts from patients with Hurler’s and Hunter’s diseases,l and more recently from patients with cystic fibrosis.2 We wish to confirm their observations and report preliminary results on tissue-cultures from bone-marrow aspirates in which metachromasia may be demonstrated at the time of the first subculture, within two weeks of the initiation of cell cultures. Several drops of bone-marrow (obtained from iliac crest) are transferred directly from the syringe into 30 ml. plastic tissueculture flasks containing 3 ml. of McCoy’s medium supplemented with 5% carbon dioxide. Within 24 hours occasional fibroblasts are present, and within 3 days rapid proliferation of cells accompanied by active phagocytosis of degenerating red blood-cells may be observed. The medium is changed first between 24 and 48 hours, and every 3-5 days subsequently. (If necessary, the cultures may be washed several times with balanced salt solution to remove excess red blood-cells before fresh medium is added.) Cells are ready to be subcultured at 1-2 weeks. At this time, Leighton-tube coverslip cultures are also initiated. Coverslips are removed for staining with toluidine blue by the method of Danes and Bearn.1 Metachromatically staining granules and vesicles were observed in cultures from patients with Hurler’s disease and cystic fibrosis. At the time of the first and second subcultures, we have not found differences in the nature of the metachromatically staining material in the two conditions. Although these cytological observations are based on a few patients with each disorder, we wish to emphasise the ease of 1. 2.
Danes, B. S., Beam, A. G. J. exp. Med. 1966, 123, 1. Danes, B. S., Beam, A. G. Lancet, May 18, 1968, p. 1061.
formation and proliferation of one or more " forbidden clones " of cells synthesising mutant m.c.p.s complementary to self T.C.F.S. Suppose antisera specific for cells producing mutant Ht.c.P.s could be prepared. Such antisera might then be used to supplement the endogenous defence mechanism, and to produce a remission of the primary disease.10 This form of anti-forbidden-clone therapy may well prove to be simpler and more efficacious than suppressing multiple M.c.p.s complementary to not-self T.C.F.S of a homograft. In other words, rational treatment for the original disease, and suppression of homograft rejection, both encounter analogous immunological
problems. The General Infirmary at Leeds. The Royal National Orthopædic Hospital, London W.1.
P. R. J. BURCH N. R. ROWELL.
mind while examining and eye anomalies.
mentally
Complete case-reports will be which Prof. C. E. Dent has kindly Ophthalmic Department, Ipswich and East Suffolk Hospital, Ipswich,
retarded children with bone
published after agreed to do for
Suffolk.
enzyme
studies,
us.
S. K. TALEGAONKAR.
TABLE-TOP FITTING FOR WALKING-AID SIR,-Wards for the care of elderly patients are often crowded, and the shortage of space may be made worse by the active policy of nursing patients out of bed and getting them walking. Chairs, walking-aids, and tables all add to the general
overcrowding. In an effort M. Hurry has
to
reduce the space taken by equipment, Sister a table-top which fits on a standard
developed
R. G. BURWELL.
ADDICTION NEW STYLE
SIR,-Professor Hamilton’s view (May 11, p. 1033) that the physician’s " ethical principles dictate that the patient should be taken off the drug [heroin] as soon as possible " is commendable. Unfortunately the reality situation is such that if an addict is forcibly treated (i.e., withdrawn from his drug willynilly), he will have an overpowering compulsion to seek his supplies elsewhere. This we do not want to happen. We do not want to go through the unfortunate American experience of the burgeoning of a vicious criminal underworld. Furthermore, when we examine the alternative to giving the addict intravenous heroin
we
find that he has
injection six-hourly, out of the question.
seven
Cane Hill Hospital, Coulsdon, Surrey CR3 3YL.
to
days
attend for an intramuscular a week, and this of course is H. DALE BECKETT.
HOMOCYSTINURIA SIR,--Homocystinuria was first described in 1962 by Carson and Neill 12 and Gerritsen et a1.13 and nearly 50 cases have now been reported.14-laThe salient clinical features include dislocation of the lens, mental retardation, skeletal anomalies, and the presence of excessive amounts of homocystine in the urine, owing to deficiencies of cystathionine synthetase, an enzyme responsible for the conversion of homocysteine to cystathionine. Many patients get thromboembolic attacks which are precipitated by surgery. I screened the urine of patients with ectopio lentis attending this department by the nitroprusside test. When this was positive, cysteine and homocysteine were distinguished by chromatography. Mental, ocular, and skeletal evaluations were done and information from previous clinical studies was assembled. Parents and sibs of the affected children were subjected to similar examinations. The diagnosis of homocystinuria was established in 3 patients. All had ectopio lentis, mental retardation, and skeletal anomalies. 1 patient had successful lens extraction with good visual results. 2 of these 3 cases showed curious skeletal anomalies like epiphyseal dysplasia, and bone cysts with pathological fractures. I feel that this disease is not as rare as the number of published reports suggests, since I could find 3 cases in a hospital with an average yearly attendance (ophthalmic cases) of 10,395. If other eye departments undertake a similar study, many more cases may be found. Moreover, paediatricians and orthopa:dic surgeons should also keep the possibility of homocystinuria in 12. Carson, N. A., Neill, D. W. Archs Dis. Childn. 1962, 37, 505. 13.
Gerritsen, T., Vaughan, J. G., Waisman, H. A. Biochem. biophys. Res. Comm. 1962, 9, 493. 14. Komrower, G. M., Wilson, V. K. Proc. R. Soc. Med. 1963, 56, 996. 15. White, H. H., Thompson, H. L., Rowland, L. P. Trans. Am. neurol. Ass. 1964, 89, 24. 16. Arnott, E. J., Greaves, D. P. Br. J. Ophthal. 1964, 48, 688. 17. Carson, N. A. J., Dent, C. E., Field, C. M. J. Pediat. 1965, 66, 565. 18. Schimke, R. N., McKusick, V. A., Huang, T., Pollack, A. D. J. Am. med. Ass. 1965, 193, 711.
Walking-aid table-top.
Zimmer walking-aid. This obviates the need for a separate table. It has the disadvantage of having to be removed before the patient can walk with the aid, but this presents no greater inconvenience than is experienced with the table adjustment for a geriatric chair. This table-top for a walking-aid can be made up easily in an
occupational therapy department. Greenwich District Hospital, London S.E.10.
R. V. BOYD.
METACHROMASIA IN FIBROBLASTS SiR,-We have followed with interest the reports by Danes and Bearn on the detection of metachromasia in fibroblasts from patients with Hurler’s and Hunter’s diseases,l and more recently from patients with cystic fibrosis.2 We wish to confirm their observations and report preliminary results on tissue-cultures from bone-marrow aspirates in which metachromasia may be demonstrated at the time of the first subculture, within two weeks of the initiation of cell cultures. Several drops of bone-marrow (obtained from iliac crest) are transferred directly from the syringe into 30 ml. plastic tissueculture flasks containing 3 ml. of McCoy’s medium supplemented with 5% carbon dioxide. Within 24 hours occasional fibroblasts are present, and within 3 days rapid proliferation of cells accompanied by active phagocytosis of degenerating red blood-cells may be observed. The medium is changed first between 24 and 48 hours, and every 3-5 days subsequently. (If necessary, the cultures may be washed several times with balanced salt solution to remove excess red blood-cells before fresh medium is added.) Cells are ready to be subcultured at 1-2 weeks. At this time, Leighton-tube coverslip cultures are also initiated. Coverslips are removed for staining with toluidine blue by the method of Danes and Bearn.1 Metachromatically staining granules and vesicles were observed in cultures from patients with Hurler’s disease and cystic fibrosis. At the time of the first and second subcultures, we have not found differences in the nature of the metachromatically staining material in the two conditions. Although these cytological observations are based on a few patients with each disorder, we wish to emphasise the ease of 1. 2.
Danes, B. S., Beam, A. G. J. exp. Med. 1966, 123, 1. Danes, B. S., Beam, A. G. Lancet, May 18, 1968, p. 1061.