Br.J. Anaesth. (1976), 48, 707
HYPERSENSITIVITY TO SUXAMETHONIUM IN A SUFFOLK FAMILY F. M. McK.
DEWAR AND G. WAKEFIELD SUMMARY
Suxamethonium apnoea developed in a patient who was a homozygote for the atypical gene. Fifty-five members of the patient's family were investigated. Seven further homozygotes were found. Suxamethonium apnoea had been demonstrated previously in one of these. Thirty-two members of the family were heterozygotes.
tone 350 mg. Suxamethonium 50 mg was given and an 8.0-mm cuffed endotracheal tube was introduced into the trachea. Spontaneous respiration failed to return following operation and the patient was ventilated artificially with halothane 0.5%, nitrous oxide and oxygen. Apnoea was present for 55 min and was followed by a 15-min period of respiratory insufficiency. Blood analysis revealed:
Pseudocholinesterase 10 units 16 Dibucaine No. 25 Fluoride No. 84 Butanol No. METHODS DD Genotype The serum cholinesterase concentrations were deterHomozygote for the atypical gene mined by the micromanometric method of Ammon After surgery the patient said that one of her (1933). One unit of pseudocholinesterase enzyme activity is defined as that which hydrolyses 1 micro- sisters (II 8 , table I) had experienced a similar episode mole of acetylcholine per minute per ml of serum at under anaesthesia on February 2, 1972, when she had undergone tubal ligation in the same hospital. 37 °C. The dibucaine numbers were determined by the Our hospital records revealed that the sister's dimethod of Kalow and Genest (1957), the fluoride bucaine number was 17.5. Thus she was a homonumbers by the method of Harris and Whittaker zygote for the atypical gene. Unfortunately, no fur(1961) and the butanol numbers by the method of ther investigations of the family had been carried out at that time. Whittaker (1968). Following this case (II 6 , table I), full family investigations were undertaken. The genetic abnormality CASE REPORT On September 11, 1974, a fit 38-year-old female was explained to each member of the family and they patient (II 6 , table I) underwent an operation for were asked to attend for a blood test. The results of the enzyme assays and inhibition dilatation, curettage and tubal h'gation. Premedication with diazepam 20 mg was administered orally studies on four generations of the family are shown 1£ h before operation. Following the injection of atro- in table I. A family tree showing the genetic type is pine 0.6 mg, anaesthesia was induced with thiopen- shown in figure 1. The parents of the two sisters mentioned above were heterozygotes for the normal and atypical gene FLORA M. M C K . DEWAR, M.B., CH.B. (EDIN.), D.A., F.F.A.R.C.S., Department of Anaesthesia, The Ipswich and of their 14 children, six were homozygotes for the Hospital, Anglesea Road, Ipswich, Suffolk IP1 3PY. atypical gene, four were heterozygotes for the normal GRETA WAKEFIELD, A.I.S.T., M.R.C. Abnormal Haemoand atypical gene and four were normal homozygotes. globin Unit, University Department of Clinical BioThe third generation produced two homozygotes chemistry, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QR. for the atypical gene and 24 heterozygotes for the
Downloaded from http://bja.oxfordjournals.org/ at Carleton University on July 9, 2015
The short action of suxamethonium is a result of its hydrolysis by plasma cholinesterase, a dose of 20100 mg normally producing 3-4 min muscular paralysis. Soon after the introduction of suxamethonium, prolonged apnoea was reported by several anaesthetists (Bourne, Collier and Somers, 1952; Evans et al., 1952). In patients with a genetically inherited abnormality of plasma cholinesterase, apnoea is prolonged considerably. This report presents a patient with suxamethonium "apnoea" and details the results of the subsequent investigations of the patient's family.
708
BRITISH JOURNAL OF ANAESTHESIA
L A L AIL L i 1
j:
i
6 I
*> 22 23 2« 25 2 6 .
111
27 21 29
121
30
13
31
11
32 33
31
5
ICTODZYGOIE H l £
TABLE I. Laboratory data for the subjects studied. Normal values in brackets
Pseudocholin- Dibuesterase caine Fluoride Butanol No. No. No. Geno(units) Subject (60-120) (77-83) (57-68) (180-220) type
HI. Ill, III. Ill, nil.
66 74 69 55 23 84 15 10 134 20 117 118 66 18 98 28 105 47 101 67 88 88 86 60 48 71
TTT
Q-2
III 1 2 IHl.
44 13
Ii Ii Hi Hi
II. II4
II.
II. II7
II. II, H» II11 "1. Hi.
II14
nix III 2
in, in* in.
57 63 67 52 20 67 22 16 82 14 87 81 63 16 83 14 83 67 80 81 77 76 55 51 63 82
45 52 57 45 28 54 28 25 65 28 68 64 52 21 67 25 61 58 58 66 58 68 52 54 53 64
70 /y
JO
57
18
eo
48 29
155 137 175 160 86 158 67 84 199 84 192 187 140 81 213 94 193 171 204 228 226 209 163 155 151 188 1
Q^
161 89
ND
ND ND ND DD ND DD DD NN
DD
Pseudocholin- Dibucaine Fluoride Butanol esterase No. No. No. (units) Subject (60-120) (77-83) (57-68) (180-220) III14 IIIi. nil. nil, nil. nil.
in.. in.1 111.1
in..
NN NN ND DD NN
m« in» m 26 111,7
DD/DS
His,
NN ND NN NN NN NN ND ND ND NN
III30 III.1
IIIjS
m in..32 11134
IVx
iv 2 IV 3 IV 4 IV.
22 24 42 44 78 48 55 70 82 87 67 103 80 55 90 51 77 70 61 67 73 64 108 61 74 84
16 60 61 60 64 68 67 61 61 61 62 60 59 52 79 59 59 65 58 63 61 65 83 58 57 85
27 45 52 42 52 54 57 51 53 43 47 55 54 48 64 45 51 49 55 53 52 44 70 54 51 64
95 155 153 158 148 142 181 168 151 167 180 150 137 152 187 150 179 157 178 150 140 126 206 174 169 222
Genotype
DD/DS ND ND ND ND ND ND ND ND ND ND ND ND ND NN ND ND ND ND ND ND ND NN ND ND NN
VTXT
IN IN
ND DD/DS
N = normal cholinesterase gene;D =: dibucaine resistant gene; S == silent gene.
Downloaded from http://bja.oxfordjournals.org/ at Carleton University on July 9, 2015
D
FIG. 1. Family tree showing genetic type.
709
HYPERSENSITIVITY TO SUXAMETHONIUM normal and atypical gene. In the fourth generation, so far, three heterozygotes have been produced. It is of interest that, in the case of the second son, one of the children by his first wife was a heterozygote and two of the children by his second wife were homozygotes for the atypical gene. Examination of this second wife revealed that she too was a heterozygote for the normal and atypical genes. DISCUSSION
ACKNOWLEDGEMENTS
We would like to thank Dr G. Mair who obtained several of the blood samples for the family investigation and for permitting the use of his surgery, and also Miss Pat Leathers for secretarial assistance, Mrs Teresa Milner for technical assistance, and Professor H. Lehmann and Dr Clive Jolly for their advice and encouragement. REFERENCES
Ammon, R. (1933). Die fermentative Spaltung des Acetycholins. Pfluegers Arch., 233, 480. Bauld, H. W., Gibson, P. F. 3 Jebson, P. J., and Brown, S. S. (1974). Aetiology of prolonged apnoea after suxamethonium. Br.J. Anaesth., 46,273. Bourne, J. G., Collier, H. O., and Somers, G. F. (1952). Succinylcholine (succinoylcholine): muscle relaxant of short action. Lancet, 1, 1225. Editorial (1967). Suxamethonium apnoea. Br. J. Anaesth., 39, 837. Evans, F. T., Gray, P. M. S., Lehmann, H., and Silk, E. (1952). Sensitivity to succinylcholine in relation to serum cholinesterase. Lancet, 1, 1229. Harris, H., and Whittaker, M. (1961). Differential inhibition of human serum cholinesterase with fluoride: recognition of two phenotypes. Nature (Land.), 191, 496. Kalow, W., and Genest, K. (1957). A method for the detection of atypical forms of human cholinesterase: determination of dibucaine numbers. Can. J. Biochem. Physiol., 35, 339. Whittaker, M. (1968). Differential inhibition of human serum cholinesterase with n-butyl alcohol: recognition of new phenotypes. Acta Genet. Med. Gemellol. (Roma), 18, 335. Vickers, M. D. (1968). Suxamethonium apnoea. Br.J. Anaesth., 40, 393.
HYPERSENSIBILITE AU SUXAMETHONIUM D'UNE FAMILLE DU SUFFOLK RESUME
Une apnee causee par le suxamethonium est apparue chez un patient qui etait homozygote pour le gene atypique. On a effectue des recherches sur 55 membres de la famille du patient en question. On a trouve sept autres homozygotes. II avait ete prouve anterieurement que l'un de ceux-ci avait recemment souffert d'apnee provoquee par le suxamethonium. Trente-deux membres de cette famille etaient heterozygotes.
Downloaded from http://bja.oxfordjournals.org/ at Carleton University on July 9, 2015
This investigation is unique in that the whole of a large family has been traced and tested. In many of the Suffolk villages intermarriage has occurred, and this probably accounts for the fact that, in one family, there are two occasions when two heterozygotes have married and produced several homozygote children. In 1967 the importance of investigating families of affected patients was emphasized (Editorial, 1967) and, in 1968, suggestions were made that these investigations should be carried out on a national basis (Whittaker and Vickers, 1968). The value of screening the relatives of patients with cholinesterase variants has been shown in a Regional Study in S.E. Scotland (Bauld et al., 1974). The present investigation of a family supports this study. If this family had been screened in 1972 when the patient (II 8 , table I) suffered prolonged apnoea, then the difficulty with the later patient (II 6 , table I) could have been avoided. The likelihood of prolonged apnoea occurring among heterozygotes for the normal and atypical gene is of great interest to anaesthetists. Of the 32 heterozygotes in this family only eight had received general anaesthetics. The majority of these heterozygotes appeared in the third and fourth generations and 53% of all the heterozygotes are under the age of 15 years. Only one of these patients (II 4 , table I) has received an anaesthetic in which suxamethonium was administered, and he did not react abnormally. It has been suggested that investigations of this kind are unnecessary, time consuming and upsetting to the patient concerned. It has been our experience in Ipswich that all patients realize that it is in their best interest, and all have been extremely co-operative. Family screening is time consuming, but the avoidance of a 1-2 h delay in an operating list and the avoidance of an unnecessarily long anaesthetic for the patient, more than compensate for this. It is now the policy of the Anaesthetic Department in this hospital to investigate, as far as possible, all members of the family when an affected patient is found. Cards are then issued to all patients affected
stating their hypersensitivity to suxamethonium, the dibucaine number and the genotype. In addition a register is kept, in the Anaesthetic Department, of all patients hypersensitive to suxamethonium. Another "affected" member of this family has received a general anaesthetic in this hospital, subsequent to this investigation. Because of her investigation as part of this family and her presentation of her suxamethonium hypersensitivity card on her admission, it was possible to avoid the administration of suxamethonium.
710
BRITISH JOURNAL OF ANAESTHESIA
HYPERSENSITIVITAT AUF SUXAMETHONIUM BEI EINER FAMILIE IN SUFFOLK
HIPERSENSIBILIDAD AL SUXAMETONIO EN UNA FAMILIA DE SUFFOLK
ZUSAMMENFASSUNG
SUMARIO
Eine Suxamethonium-Apnoe entwickelte sich in einem Patienten, einem Homozygoten fiir die atypischen Gene. Funfundfunfzig Mitglieder der Familie dieses Patienten wurden untersucht, wobei sieben weitere Homozygoten festgestellt wurden. Bei einem davon war schon fruher eine Suxamethonium-Apnoe festgestellt worden. Zweiunddreissig Familienmitglieder waren Heterozygoten.
Se desarrollo apnea por suxametonio (succinilcolina) en un paciente homocigoto para el gen atipico. Cincuenta y cinco miembros de la familia del paciente fueron investigados, hallandose otros siete homocigotos. En uno de estos se habia demostrado previamente ese tipo de apnea. Treinta y dos miembros de la familia era heterocigotos.
Downloaded from http://bja.oxfordjournals.org/ at Carleton University on July 9, 2015
THE YORKSHIRE SOCIETY OF ANAESTHETISTS
13th October, 1976 "Anaesthesia & Politics"—Dr. Brian Lewis 10th November, 1976 "Curare & Charles Waterton"—Dr. K. Bryn Thomas 8th December, 1976 "The British Journal of Anaesthesia"—Dr. Alastair Spence
ROYAL COLLEGE OF SURGEONS OF ENGLAND
The 1976 Annual Meeting of Fellows and Members will take place at the University of Leeds on Friday, 24th and Saturday, 25th September 1976. The symposia on topics of interest to surgeons, dental surgeons, anaesthetists and others are open to all medical and dental practitioners, whether or not they are diplomates of the College.
Full particulars may be obtained from the Secretary, Royal College of Surgeons of England, 35/43 Lincoln's Inn Fields, London WC2A 3PN. (Tel: 01-405 3473 ext. 163).
Downloaded from http://bja.oxfordjournals.org/ at Carleton University on July 9, 2015
Hon. Secretary: Dr. R. S. Edmondson, Chalforde House, Hall Drive, Bramhope, Leeds LS16 9JE Tel: Arthington 2478
When rapid, reliable, sedative cover is needed.
The gentle persuaderIntravenous Valium Roche For minor surgery and unpleasant diagnostic and therapeutic procedures an intravenous injection of Valium Roche gives rapid sedation and relief of anxiety with useful muscle relaxation.
Dosage can be adjusted to individual needs by observing the development of ptosis. Keep intravenous Valium Roche in mindand at hand. Valium is the trade mark tor Roche pharmaceutical preparations containing dia^cpam. Full prescribing information is available. Roche Products Limited, PO Box 2LE, 15 Manchester Square, London WIA 2LE.
Canad.AnaeMh.Soc.J..io.?i,;«,i66 Brit.med.J..iO72,j,278 Brit.J.Anaesth.,1973.4.!.1150 Pracmioner.tu?}..?/ 1.805 Dundee.J.W.andWvant.GM . " Intravenous Anaesthesia,1" Churchill 1 jvingslone. Edinburgh and L.ond
HYPERSENSITIVITY TO SUXAMETHONIUM IN A SUFFOLK FAMILY F. M. McK.
DEWAR AND G. WAKEFIELD SUMMARY
Suxamethonium apnoea developed in a patient who was a homozygote for the atypical gene. Fifty-five members of the patient's family were investigated. Seven further homozygotes were found. Suxamethonium apnoea had been demonstrated previously in one of these. Thirty-two members of the family were heterozygotes.
tone 350 mg. Suxamethonium 50 mg was given and an 8.0-mm cuffed endotracheal tube was introduced into the trachea. Spontaneous respiration failed to return following operation and the patient was ventilated artificially with halothane 0.5%, nitrous oxide and oxygen. Apnoea was present for 55 min and was followed by a 15-min period of respiratory insufficiency. Blood analysis revealed:
Pseudocholinesterase 10 units 16 Dibucaine No. 25 Fluoride No. 84 Butanol No. METHODS DD Genotype The serum cholinesterase concentrations were deterHomozygote for the atypical gene mined by the micromanometric method of Ammon After surgery the patient said that one of her (1933). One unit of pseudocholinesterase enzyme activity is defined as that which hydrolyses 1 micro- sisters (II 8 , table I) had experienced a similar episode mole of acetylcholine per minute per ml of serum at under anaesthesia on February 2, 1972, when she had undergone tubal ligation in the same hospital. 37 °C. The dibucaine numbers were determined by the Our hospital records revealed that the sister's dimethod of Kalow and Genest (1957), the fluoride bucaine number was 17.5. Thus she was a homonumbers by the method of Harris and Whittaker zygote for the atypical gene. Unfortunately, no fur(1961) and the butanol numbers by the method of ther investigations of the family had been carried out at that time. Whittaker (1968). Following this case (II 6 , table I), full family investigations were undertaken. The genetic abnormality CASE REPORT On September 11, 1974, a fit 38-year-old female was explained to each member of the family and they patient (II 6 , table I) underwent an operation for were asked to attend for a blood test. The results of the enzyme assays and inhibition dilatation, curettage and tubal h'gation. Premedication with diazepam 20 mg was administered orally studies on four generations of the family are shown 1£ h before operation. Following the injection of atro- in table I. A family tree showing the genetic type is pine 0.6 mg, anaesthesia was induced with thiopen- shown in figure 1. The parents of the two sisters mentioned above were heterozygotes for the normal and atypical gene FLORA M. M C K . DEWAR, M.B., CH.B. (EDIN.), D.A., F.F.A.R.C.S., Department of Anaesthesia, The Ipswich and of their 14 children, six were homozygotes for the Hospital, Anglesea Road, Ipswich, Suffolk IP1 3PY. atypical gene, four were heterozygotes for the normal GRETA WAKEFIELD, A.I.S.T., M.R.C. Abnormal Haemoand atypical gene and four were normal homozygotes. globin Unit, University Department of Clinical BioThe third generation produced two homozygotes chemistry, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QR. for the atypical gene and 24 heterozygotes for the
Downloaded from http://bja.oxfordjournals.org/ at Carleton University on July 9, 2015
The short action of suxamethonium is a result of its hydrolysis by plasma cholinesterase, a dose of 20100 mg normally producing 3-4 min muscular paralysis. Soon after the introduction of suxamethonium, prolonged apnoea was reported by several anaesthetists (Bourne, Collier and Somers, 1952; Evans et al., 1952). In patients with a genetically inherited abnormality of plasma cholinesterase, apnoea is prolonged considerably. This report presents a patient with suxamethonium "apnoea" and details the results of the subsequent investigations of the patient's family.
708
BRITISH JOURNAL OF ANAESTHESIA
L A L AIL L i 1
j:
i
6 I
*> 22 23 2« 25 2 6 .
111
27 21 29
121
30
13
31
11
32 33
31
5
ICTODZYGOIE H l £
TABLE I. Laboratory data for the subjects studied. Normal values in brackets
Pseudocholin- Dibuesterase caine Fluoride Butanol No. No. No. Geno(units) Subject (60-120) (77-83) (57-68) (180-220) type
HI. Ill, III. Ill, nil.
66 74 69 55 23 84 15 10 134 20 117 118 66 18 98 28 105 47 101 67 88 88 86 60 48 71
TTT
Q-2
III 1 2 IHl.
44 13
Ii Ii Hi Hi
II. II4
II.
II. II7
II. II, H» II11 "1. Hi.
II14
nix III 2
in, in* in.
57 63 67 52 20 67 22 16 82 14 87 81 63 16 83 14 83 67 80 81 77 76 55 51 63 82
45 52 57 45 28 54 28 25 65 28 68 64 52 21 67 25 61 58 58 66 58 68 52 54 53 64
70 /y
JO
57
18
eo
48 29
155 137 175 160 86 158 67 84 199 84 192 187 140 81 213 94 193 171 204 228 226 209 163 155 151 188 1
Q^
161 89
ND
ND ND ND DD ND DD DD NN
DD
Pseudocholin- Dibucaine Fluoride Butanol esterase No. No. No. (units) Subject (60-120) (77-83) (57-68) (180-220) III14 IIIi. nil. nil, nil. nil.
in.. in.1 111.1
in..
NN NN ND DD NN
m« in» m 26 111,7
DD/DS
His,
NN ND NN NN NN NN ND ND ND NN
III30 III.1
IIIjS
m in..32 11134
IVx
iv 2 IV 3 IV 4 IV.
22 24 42 44 78 48 55 70 82 87 67 103 80 55 90 51 77 70 61 67 73 64 108 61 74 84
16 60 61 60 64 68 67 61 61 61 62 60 59 52 79 59 59 65 58 63 61 65 83 58 57 85
27 45 52 42 52 54 57 51 53 43 47 55 54 48 64 45 51 49 55 53 52 44 70 54 51 64
95 155 153 158 148 142 181 168 151 167 180 150 137 152 187 150 179 157 178 150 140 126 206 174 169 222
Genotype
DD/DS ND ND ND ND ND ND ND ND ND ND ND ND ND NN ND ND ND ND ND ND ND NN ND ND NN
VTXT
IN IN
ND DD/DS
N = normal cholinesterase gene;D =: dibucaine resistant gene; S == silent gene.
Downloaded from http://bja.oxfordjournals.org/ at Carleton University on July 9, 2015
D
FIG. 1. Family tree showing genetic type.
709
HYPERSENSITIVITY TO SUXAMETHONIUM normal and atypical gene. In the fourth generation, so far, three heterozygotes have been produced. It is of interest that, in the case of the second son, one of the children by his first wife was a heterozygote and two of the children by his second wife were homozygotes for the atypical gene. Examination of this second wife revealed that she too was a heterozygote for the normal and atypical genes. DISCUSSION
ACKNOWLEDGEMENTS
We would like to thank Dr G. Mair who obtained several of the blood samples for the family investigation and for permitting the use of his surgery, and also Miss Pat Leathers for secretarial assistance, Mrs Teresa Milner for technical assistance, and Professor H. Lehmann and Dr Clive Jolly for their advice and encouragement. REFERENCES
Ammon, R. (1933). Die fermentative Spaltung des Acetycholins. Pfluegers Arch., 233, 480. Bauld, H. W., Gibson, P. F. 3 Jebson, P. J., and Brown, S. S. (1974). Aetiology of prolonged apnoea after suxamethonium. Br.J. Anaesth., 46,273. Bourne, J. G., Collier, H. O., and Somers, G. F. (1952). Succinylcholine (succinoylcholine): muscle relaxant of short action. Lancet, 1, 1225. Editorial (1967). Suxamethonium apnoea. Br. J. Anaesth., 39, 837. Evans, F. T., Gray, P. M. S., Lehmann, H., and Silk, E. (1952). Sensitivity to succinylcholine in relation to serum cholinesterase. Lancet, 1, 1229. Harris, H., and Whittaker, M. (1961). Differential inhibition of human serum cholinesterase with fluoride: recognition of two phenotypes. Nature (Land.), 191, 496. Kalow, W., and Genest, K. (1957). A method for the detection of atypical forms of human cholinesterase: determination of dibucaine numbers. Can. J. Biochem. Physiol., 35, 339. Whittaker, M. (1968). Differential inhibition of human serum cholinesterase with n-butyl alcohol: recognition of new phenotypes. Acta Genet. Med. Gemellol. (Roma), 18, 335. Vickers, M. D. (1968). Suxamethonium apnoea. Br.J. Anaesth., 40, 393.
HYPERSENSIBILITE AU SUXAMETHONIUM D'UNE FAMILLE DU SUFFOLK RESUME
Une apnee causee par le suxamethonium est apparue chez un patient qui etait homozygote pour le gene atypique. On a effectue des recherches sur 55 membres de la famille du patient en question. On a trouve sept autres homozygotes. II avait ete prouve anterieurement que l'un de ceux-ci avait recemment souffert d'apnee provoquee par le suxamethonium. Trente-deux membres de cette famille etaient heterozygotes.
Downloaded from http://bja.oxfordjournals.org/ at Carleton University on July 9, 2015
This investigation is unique in that the whole of a large family has been traced and tested. In many of the Suffolk villages intermarriage has occurred, and this probably accounts for the fact that, in one family, there are two occasions when two heterozygotes have married and produced several homozygote children. In 1967 the importance of investigating families of affected patients was emphasized (Editorial, 1967) and, in 1968, suggestions were made that these investigations should be carried out on a national basis (Whittaker and Vickers, 1968). The value of screening the relatives of patients with cholinesterase variants has been shown in a Regional Study in S.E. Scotland (Bauld et al., 1974). The present investigation of a family supports this study. If this family had been screened in 1972 when the patient (II 8 , table I) suffered prolonged apnoea, then the difficulty with the later patient (II 6 , table I) could have been avoided. The likelihood of prolonged apnoea occurring among heterozygotes for the normal and atypical gene is of great interest to anaesthetists. Of the 32 heterozygotes in this family only eight had received general anaesthetics. The majority of these heterozygotes appeared in the third and fourth generations and 53% of all the heterozygotes are under the age of 15 years. Only one of these patients (II 4 , table I) has received an anaesthetic in which suxamethonium was administered, and he did not react abnormally. It has been suggested that investigations of this kind are unnecessary, time consuming and upsetting to the patient concerned. It has been our experience in Ipswich that all patients realize that it is in their best interest, and all have been extremely co-operative. Family screening is time consuming, but the avoidance of a 1-2 h delay in an operating list and the avoidance of an unnecessarily long anaesthetic for the patient, more than compensate for this. It is now the policy of the Anaesthetic Department in this hospital to investigate, as far as possible, all members of the family when an affected patient is found. Cards are then issued to all patients affected
stating their hypersensitivity to suxamethonium, the dibucaine number and the genotype. In addition a register is kept, in the Anaesthetic Department, of all patients hypersensitive to suxamethonium. Another "affected" member of this family has received a general anaesthetic in this hospital, subsequent to this investigation. Because of her investigation as part of this family and her presentation of her suxamethonium hypersensitivity card on her admission, it was possible to avoid the administration of suxamethonium.
710
BRITISH JOURNAL OF ANAESTHESIA
HYPERSENSITIVITAT AUF SUXAMETHONIUM BEI EINER FAMILIE IN SUFFOLK
HIPERSENSIBILIDAD AL SUXAMETONIO EN UNA FAMILIA DE SUFFOLK
ZUSAMMENFASSUNG
SUMARIO
Eine Suxamethonium-Apnoe entwickelte sich in einem Patienten, einem Homozygoten fiir die atypischen Gene. Funfundfunfzig Mitglieder der Familie dieses Patienten wurden untersucht, wobei sieben weitere Homozygoten festgestellt wurden. Bei einem davon war schon fruher eine Suxamethonium-Apnoe festgestellt worden. Zweiunddreissig Familienmitglieder waren Heterozygoten.
Se desarrollo apnea por suxametonio (succinilcolina) en un paciente homocigoto para el gen atipico. Cincuenta y cinco miembros de la familia del paciente fueron investigados, hallandose otros siete homocigotos. En uno de estos se habia demostrado previamente ese tipo de apnea. Treinta y dos miembros de la familia era heterocigotos.
Downloaded from http://bja.oxfordjournals.org/ at Carleton University on July 9, 2015
THE YORKSHIRE SOCIETY OF ANAESTHETISTS
13th October, 1976 "Anaesthesia & Politics"—Dr. Brian Lewis 10th November, 1976 "Curare & Charles Waterton"—Dr. K. Bryn Thomas 8th December, 1976 "The British Journal of Anaesthesia"—Dr. Alastair Spence
ROYAL COLLEGE OF SURGEONS OF ENGLAND
The 1976 Annual Meeting of Fellows and Members will take place at the University of Leeds on Friday, 24th and Saturday, 25th September 1976. The symposia on topics of interest to surgeons, dental surgeons, anaesthetists and others are open to all medical and dental practitioners, whether or not they are diplomates of the College.
Full particulars may be obtained from the Secretary, Royal College of Surgeons of England, 35/43 Lincoln's Inn Fields, London WC2A 3PN. (Tel: 01-405 3473 ext. 163).
Downloaded from http://bja.oxfordjournals.org/ at Carleton University on July 9, 2015
Hon. Secretary: Dr. R. S. Edmondson, Chalforde House, Hall Drive, Bramhope, Leeds LS16 9JE Tel: Arthington 2478
When rapid, reliable, sedative cover is needed.
The gentle persuaderIntravenous Valium Roche For minor surgery and unpleasant diagnostic and therapeutic procedures an intravenous injection of Valium Roche gives rapid sedation and relief of anxiety with useful muscle relaxation.
Dosage can be adjusted to individual needs by observing the development of ptosis. Keep intravenous Valium Roche in mindand at hand. Valium is the trade mark tor Roche pharmaceutical preparations containing dia^cpam. Full prescribing information is available. Roche Products Limited, PO Box 2LE, 15 Manchester Square, London WIA 2LE.
Canad.AnaeMh.Soc.J..io.?i,;«,i66 Brit.med.J..iO72,j,278 Brit.J.Anaesth.,1973.4.!.1150 Pracmioner.tu?}..?/ 1.805 Dundee.J.W.andWvant.GM . " Intravenous Anaesthesia,1" Churchill 1 jvingslone. Edinburgh and L.ond
