- Email: [email protected]
HOMOZYGOUS CASE OF HEREDITARY COPROPORPHYRIA
1348
by political attitudes, would prove a far more potent force in expanding private practice than any contractual change could be. Last week’s Lancet editorial goes on to spell out the sources of consultant discontent, mentioning specifically that "even last summer an 80% increase was needed to bring a wholetimer back, in real terms, to his 1974 salary". This disastrous situation has been achieved by operation of the existing contract. All consultants must ask themselves whether they can see any prospect, under any conceivable circumstances, of this degree of degradation of their financial position being corrected under the existing contract. For one so clearly committed to the future well-being of, at least, the whole-time consultant, you are curiously anxious to convince Government that it cannot allow consultants to achieve any financial advantage from the new contract. Clearly, it will be for the Review Body to assess the situation and to make what award it thinks fit and for the consultants’ representatives to discuss with Government a system of implementation which is fair to the consultants and to the national economy. The responsible, elected representatives of the consultants are trying to find a way to correct the acknowledged injustices to which their colleagues are subject by rational argument and negotiation and, despite the great difficulties involved, the word "sanctions" has never been mentioned. It is odd that the first veiled threat of such action should have been found, of all places, in the columns of The Lancet, a journal from which we would have expected support for an honest attempt to achieve a peaceful resolution of our difficulties. Finally, there appears a "plug" for the N.H.S. Consultants Association-presumably concern for the N.H.S. is confined to its tiny and unrepresentative membership-mentioning various proposals common to that organisation and the C.C.H.M.S. but studiously avoiding any reference to suggestions in their latest publication on contract such as the one that any consultant engaging in private practice should be limited to a maximum of seven N.H.S. sessions. "Above all" they say, "Mr Ennals must stick to the principle of financial recognition of N.H.S. commitment". The C.C.H.M.S., the elected body representing all consultants, the Scottish C.H.M.S., with its large whole-time element, the Negotiating Subcommittee and the Contract Working Party, both containing almost exactly equal numbers of whole-time and part-time consultants, all disagree with this view. Our junior colleagues, who will have no option, on attaining consultant status, but to accept the new contract if it is introduced, unequivocally support the view that consultants should be free to use their noncontractual time as they choose. The new contract offers many immediate financial advantages and the prospect of further progress in the future but it also holds out the prospect of uniting the consultants into a single body by ending the present artificial division between whole and part time. Such an objective is certainly worth pursuing and one which all consultants can support: those who do not support such a development can, of course, keep their present contracts. Meanwhile, it is greatly to be hoped that the consultant dog will not allow himself to be wagged by this particular academic tail. ever
DAVID British Medical Association, Tavistock Square, London WC1H 9JP
BOLT,
Chairman of the Negotiating Subcommittee of C.C.H.M.S. and of the Staff Side of the Contract Working Party
SIR,-The thinking of the N.H.S. Consultants Association, if the letter from Dr MacDonald (Dec. 10, p. 1234) is typical, seems deluded. In my first year of part-time work, having formerly been a full-time consultant, my accountant and I were able, with difficulty, to obtain an allowance to be set against tax of k296 for all private-practice motoring expenses. With tax at 50% this meant in real terms ,E148 for me (except that I believe the actual expenses were greater). During that year my gross receipts (certified in my private-practice- accounts) were rather less than my drop in salary when I changed from
part time. But that year I did more work for the N.H.S. on an extra outpatient clinic at Darlington. From these gross receipts the cost of stationery, secretarial services, rent, and so on had to be found. Dr MacDonald’s statement that "The automatic tax benefits enjoyed by the part-timer, especially in respect of car depreciation and petrol, mean that in real terms he will be more than 1000 better off per annum than his full-time colleague before he earns a penny in private practice" is nonfull
to
I took
as
sense.
North
Ormesby Hospital, Middlesbrough, Cleveland
TS3
6HJ
ROGER HOLE
SiR,-You have given space in your letters pages to the views of the N.H.S. Consultants Association, the function of which appears to be to limit the earnings of those consultants who can earn a little more than the minimum. The argument is that of the Yorkshire miners, and in that industry productivity deals, leading to increased earnings of those who are able to give more service, have been accepted by the nation with a sigh of relief. Now in the medical industry there are also those who do not wish to relate service to pay, and who, like the N.H.S. Consultants Association, wish to impose their own ethic on the rest of the profession. The reply surely must be that if the members of the N.H.S. Consultants Association wish to provide extra service for no extra pay, they should be allowed to do soindeed there is nothing to stop them doing it now. The rest of us will move with the times and have their contracts closed. Interference in a closed contract will be subject to the kind of "unreasonable restraint of trade" High Court ruling given by Mr Justice Slade in the Packer cricket circus case. Dr Clark (Nov. 10, p. 1234) says of the insurance-based services in West Germany that most people cannot afford the premiums. I have recently spent a year there treating penniless Germans, Turks, Italians, and Spaniards. Their treatment was paid for by employers’ contributions. Such is the credibility of the N.H.S. Consultants Association. Joyce Green Hospital, Dartford, Kent DA1 5PL
FELIX E. WEALE
SIR,-Dr MacDonald believes that a part-time consultant, before he earns a penny in private practice, is 1000 annum better off than his full-time colleague. Perhaps Dr MacDonald should try the experiment of converting his contract from whole-time to part-time in order to discover how much more money he will have.
per
Kettering and District Hospital, Kettering, Northamptonshire NN16 8UZ
G. F. BAINES
HOMOZYGOUS CASE OF HEREDITARY COPROPORPHYRIA
SiR,-Hereditary coproporphyria (H.c.)) is a hepatic porphyria which is inherited as an autosomal dominant;’ clinically it resembles two other forms of hepatic porphyria, intermittent acute porphyria and porphyria variegata.2 Excessive excretion of coproporphyrin III in faeces is the most striking biochemical abnormality. A deficiency of 50% of coproporphyrinogen III oxidase (c.P.G. oxidase) activity has been demonstrated in skin fibroblasts3 and blood lympho1.
Berger, H., Goldberg, A. Br. med. J. 1955, ii, 85. Schmid, R. in The Metabolic Basis of Inherited Disease (edited by J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson); p. 1087. New York, 1972. Elder, G. H., Evans, J. O., Thomas, N., Cox, R., Brodie, M. J., Moore, M. R., Goldberg, A. Lancet, 1976, ii, 1217.
2. Marver, H. S.,
3.
1349 TABLE I-BIOCHEMICAL FINDINGS
*per g creatinine. tper g dry weight. A.L.A.=8-aminolaEvuHnic acid; Copro=coproporphyrin; P.B.G.=Porphobilinogen; Proto=protoporphyrin; Uro=uroporphyrin.
cytes4.s
as
the
primary
defect. We report here the first
case
of
TABLE II-ACTIVITY OF LYMPHOCYTE C.P.G.OXIDASE
homozygous H.c. The patient was
born in 1957 after a normal pregnancy. When she was 4 years old her mother noted hypertrichosis and skin pigmentation affecting the face and the dorsum of both hands. At 10 years, she was admitted to hospital for persistent vomiting, abdominal pain, and constipation, followed by diarrhcea:6 her plasma-sodium was very low (113 mmol/1). Her urine was dark, and porphyrin screening was done, revealing H.c.: urine and faeces contained very large amounts of coproporphyrin and raised concentrations of precursors and uroporphyrin. After few weeks she improved rapidly and remained in remission for 10 years. In 1977 she became pregnant, and was soon acutely ill with vomiting. Physical examination was normal, except for short stature (142 cm) and tachycardia. Nordmann, Y., Grandchamp, B., Phung, N., de Verneuil, H., Grelier, M., Noiré, J. ibid. 1977, i, 140. 5. Grandchamp, B., Nordmann, Y. Biochem. Biophys. Res. Comm., 1977, 74, 4.
1089.
6.
Gajdos, A., Weil, J., Gajdos-Török, M., Coupry, A. Rev. Fr. Étud. Clin. Biol. 1969, 14, 279.
*pmol protoporphyrin/mgprotein/h at 37 °C; done in duplicate.
Family screening at the time of diagnosis (1967) uncovered only two latent cases (mother and stepsister, see figure and table i). In 1977 we measureds the propositus’s lymphocyte C.P.G. oxidase activity and found it to be only 2% of normal, instead of 50% as expected. When we reinvestigated the family by measuring C.P.G. oxidase activity we found that not only the mother but also the father were latent cases, with only 50% of normal C.P.G. oxidase activity (table u). Furthermore father and mother were first cousins (see figure). The patient is undoubtedly homozygous. Table ii shows also the enzyme results obtained with one of the patient’s sisters and with both children of her stepsister, who are normal. To our knowledge only one homozygous case of H.c. has previously been suspected;l this patient was the son of first cousins who were latent cases of H.c. As with our patient, this boy was very small, excreted a tremendous amount of coproporphyrin in urine and faeces and did not show any anaemia, a striking feature (our patient was never anaemic, except during the late crisis associated with pregnancy). It seems therefore that mammalian cells have the potential for overproduction of hsem, allowing synthesis of sufficient haem despite drastic enzyme deficiency. Measurement of the C.P.G. oxidase activity of the patient described by Berger and Goldberg might confirm the genetic hypothesis. Department of Biochemistry, University of Paris VII, Hôpital Louis Mourier, Colombes 92701, France
BERNARD GRANDCHAMP NHU PHUNG YVES NORDMANN
SERUM ANTIBODIES 9 YEARS AFTER CENDEHILL RUBELLA IMMUNISATION
SIR,-Natural rubella infection gives life-long protection clinical disease, but the duration of the immunity after
against
rubella vaccination is not known.1,2
Family tree.
1. Herrmann, K. L., Halstead, S. B., Brandling-Bennett, A. D., Witte, J. J. Wiebenga, N. H., Eddins, D. L. J. Am. med. Ass. 1976, 235, 2201. 2. Black, F. L., Lamm, S. H., Emmons, J. E., Pinheiro, P. F. J. infect. Dis.
1976, 133, 393.
by political attitudes, would prove a far more potent force in expanding private practice than any contractual change could be. Last week’s Lancet editorial goes on to spell out the sources of consultant discontent, mentioning specifically that "even last summer an 80% increase was needed to bring a wholetimer back, in real terms, to his 1974 salary". This disastrous situation has been achieved by operation of the existing contract. All consultants must ask themselves whether they can see any prospect, under any conceivable circumstances, of this degree of degradation of their financial position being corrected under the existing contract. For one so clearly committed to the future well-being of, at least, the whole-time consultant, you are curiously anxious to convince Government that it cannot allow consultants to achieve any financial advantage from the new contract. Clearly, it will be for the Review Body to assess the situation and to make what award it thinks fit and for the consultants’ representatives to discuss with Government a system of implementation which is fair to the consultants and to the national economy. The responsible, elected representatives of the consultants are trying to find a way to correct the acknowledged injustices to which their colleagues are subject by rational argument and negotiation and, despite the great difficulties involved, the word "sanctions" has never been mentioned. It is odd that the first veiled threat of such action should have been found, of all places, in the columns of The Lancet, a journal from which we would have expected support for an honest attempt to achieve a peaceful resolution of our difficulties. Finally, there appears a "plug" for the N.H.S. Consultants Association-presumably concern for the N.H.S. is confined to its tiny and unrepresentative membership-mentioning various proposals common to that organisation and the C.C.H.M.S. but studiously avoiding any reference to suggestions in their latest publication on contract such as the one that any consultant engaging in private practice should be limited to a maximum of seven N.H.S. sessions. "Above all" they say, "Mr Ennals must stick to the principle of financial recognition of N.H.S. commitment". The C.C.H.M.S., the elected body representing all consultants, the Scottish C.H.M.S., with its large whole-time element, the Negotiating Subcommittee and the Contract Working Party, both containing almost exactly equal numbers of whole-time and part-time consultants, all disagree with this view. Our junior colleagues, who will have no option, on attaining consultant status, but to accept the new contract if it is introduced, unequivocally support the view that consultants should be free to use their noncontractual time as they choose. The new contract offers many immediate financial advantages and the prospect of further progress in the future but it also holds out the prospect of uniting the consultants into a single body by ending the present artificial division between whole and part time. Such an objective is certainly worth pursuing and one which all consultants can support: those who do not support such a development can, of course, keep their present contracts. Meanwhile, it is greatly to be hoped that the consultant dog will not allow himself to be wagged by this particular academic tail. ever
DAVID British Medical Association, Tavistock Square, London WC1H 9JP
BOLT,
Chairman of the Negotiating Subcommittee of C.C.H.M.S. and of the Staff Side of the Contract Working Party
SIR,-The thinking of the N.H.S. Consultants Association, if the letter from Dr MacDonald (Dec. 10, p. 1234) is typical, seems deluded. In my first year of part-time work, having formerly been a full-time consultant, my accountant and I were able, with difficulty, to obtain an allowance to be set against tax of k296 for all private-practice motoring expenses. With tax at 50% this meant in real terms ,E148 for me (except that I believe the actual expenses were greater). During that year my gross receipts (certified in my private-practice- accounts) were rather less than my drop in salary when I changed from
part time. But that year I did more work for the N.H.S. on an extra outpatient clinic at Darlington. From these gross receipts the cost of stationery, secretarial services, rent, and so on had to be found. Dr MacDonald’s statement that "The automatic tax benefits enjoyed by the part-timer, especially in respect of car depreciation and petrol, mean that in real terms he will be more than 1000 better off per annum than his full-time colleague before he earns a penny in private practice" is nonfull
to
I took
as
sense.
North
Ormesby Hospital, Middlesbrough, Cleveland
TS3
6HJ
ROGER HOLE
SiR,-You have given space in your letters pages to the views of the N.H.S. Consultants Association, the function of which appears to be to limit the earnings of those consultants who can earn a little more than the minimum. The argument is that of the Yorkshire miners, and in that industry productivity deals, leading to increased earnings of those who are able to give more service, have been accepted by the nation with a sigh of relief. Now in the medical industry there are also those who do not wish to relate service to pay, and who, like the N.H.S. Consultants Association, wish to impose their own ethic on the rest of the profession. The reply surely must be that if the members of the N.H.S. Consultants Association wish to provide extra service for no extra pay, they should be allowed to do soindeed there is nothing to stop them doing it now. The rest of us will move with the times and have their contracts closed. Interference in a closed contract will be subject to the kind of "unreasonable restraint of trade" High Court ruling given by Mr Justice Slade in the Packer cricket circus case. Dr Clark (Nov. 10, p. 1234) says of the insurance-based services in West Germany that most people cannot afford the premiums. I have recently spent a year there treating penniless Germans, Turks, Italians, and Spaniards. Their treatment was paid for by employers’ contributions. Such is the credibility of the N.H.S. Consultants Association. Joyce Green Hospital, Dartford, Kent DA1 5PL
FELIX E. WEALE
SIR,-Dr MacDonald believes that a part-time consultant, before he earns a penny in private practice, is 1000 annum better off than his full-time colleague. Perhaps Dr MacDonald should try the experiment of converting his contract from whole-time to part-time in order to discover how much more money he will have.
per
Kettering and District Hospital, Kettering, Northamptonshire NN16 8UZ
G. F. BAINES
HOMOZYGOUS CASE OF HEREDITARY COPROPORPHYRIA
SiR,-Hereditary coproporphyria (H.c.)) is a hepatic porphyria which is inherited as an autosomal dominant;’ clinically it resembles two other forms of hepatic porphyria, intermittent acute porphyria and porphyria variegata.2 Excessive excretion of coproporphyrin III in faeces is the most striking biochemical abnormality. A deficiency of 50% of coproporphyrinogen III oxidase (c.P.G. oxidase) activity has been demonstrated in skin fibroblasts3 and blood lympho1.
Berger, H., Goldberg, A. Br. med. J. 1955, ii, 85. Schmid, R. in The Metabolic Basis of Inherited Disease (edited by J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson); p. 1087. New York, 1972. Elder, G. H., Evans, J. O., Thomas, N., Cox, R., Brodie, M. J., Moore, M. R., Goldberg, A. Lancet, 1976, ii, 1217.
2. Marver, H. S.,
3.
1349 TABLE I-BIOCHEMICAL FINDINGS
*per g creatinine. tper g dry weight. A.L.A.=8-aminolaEvuHnic acid; Copro=coproporphyrin; P.B.G.=Porphobilinogen; Proto=protoporphyrin; Uro=uroporphyrin.
cytes4.s
as
the
primary
defect. We report here the first
case
of
TABLE II-ACTIVITY OF LYMPHOCYTE C.P.G.OXIDASE
homozygous H.c. The patient was
born in 1957 after a normal pregnancy. When she was 4 years old her mother noted hypertrichosis and skin pigmentation affecting the face and the dorsum of both hands. At 10 years, she was admitted to hospital for persistent vomiting, abdominal pain, and constipation, followed by diarrhcea:6 her plasma-sodium was very low (113 mmol/1). Her urine was dark, and porphyrin screening was done, revealing H.c.: urine and faeces contained very large amounts of coproporphyrin and raised concentrations of precursors and uroporphyrin. After few weeks she improved rapidly and remained in remission for 10 years. In 1977 she became pregnant, and was soon acutely ill with vomiting. Physical examination was normal, except for short stature (142 cm) and tachycardia. Nordmann, Y., Grandchamp, B., Phung, N., de Verneuil, H., Grelier, M., Noiré, J. ibid. 1977, i, 140. 5. Grandchamp, B., Nordmann, Y. Biochem. Biophys. Res. Comm., 1977, 74, 4.
1089.
6.
Gajdos, A., Weil, J., Gajdos-Török, M., Coupry, A. Rev. Fr. Étud. Clin. Biol. 1969, 14, 279.
*pmol protoporphyrin/mgprotein/h at 37 °C; done in duplicate.
Family screening at the time of diagnosis (1967) uncovered only two latent cases (mother and stepsister, see figure and table i). In 1977 we measureds the propositus’s lymphocyte C.P.G. oxidase activity and found it to be only 2% of normal, instead of 50% as expected. When we reinvestigated the family by measuring C.P.G. oxidase activity we found that not only the mother but also the father were latent cases, with only 50% of normal C.P.G. oxidase activity (table u). Furthermore father and mother were first cousins (see figure). The patient is undoubtedly homozygous. Table ii shows also the enzyme results obtained with one of the patient’s sisters and with both children of her stepsister, who are normal. To our knowledge only one homozygous case of H.c. has previously been suspected;l this patient was the son of first cousins who were latent cases of H.c. As with our patient, this boy was very small, excreted a tremendous amount of coproporphyrin in urine and faeces and did not show any anaemia, a striking feature (our patient was never anaemic, except during the late crisis associated with pregnancy). It seems therefore that mammalian cells have the potential for overproduction of hsem, allowing synthesis of sufficient haem despite drastic enzyme deficiency. Measurement of the C.P.G. oxidase activity of the patient described by Berger and Goldberg might confirm the genetic hypothesis. Department of Biochemistry, University of Paris VII, Hôpital Louis Mourier, Colombes 92701, France
BERNARD GRANDCHAMP NHU PHUNG YVES NORDMANN
SERUM ANTIBODIES 9 YEARS AFTER CENDEHILL RUBELLA IMMUNISATION
SIR,-Natural rubella infection gives life-long protection clinical disease, but the duration of the immunity after
against
rubella vaccination is not known.1,2
Family tree.
1. Herrmann, K. L., Halstead, S. B., Brandling-Bennett, A. D., Witte, J. J. Wiebenga, N. H., Eddins, D. L. J. Am. med. Ass. 1976, 235, 2201. 2. Black, F. L., Lamm, S. H., Emmons, J. E., Pinheiro, P. F. J. infect. Dis.
1976, 133, 393.