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RENAL LESIONS IN WILSON'S DISEASE
1178
conditioning of calm. An exercise which controls breathing favourably influences the psychiatric regimen. Sanatorium, Tokyo.
National Musashi
M. HIGASHI.
RENAL LESIONS IN WILSON’S DISEASE
SiR,ńDr. Hall (May 2) asks whether the histological changes reported in my paperwere due to autolysis. The tissue was not obtained in the first hour after death, it is true; but control tissues from patients with other diseases, similarly handled, were always examined simultaneously, and these changes were not seen. Moreover, the finding that the areas of histological changes frequently corresponded to the areas showing copper deposition histochemically suggests that these two observations are related. Again, these same areas were frequently surrounded by normal tissue. If autolysis had been responsible for the changes, it would have been selective autolysis, and its appearance in the areas where the copper was deposited would have been fortuitous.
I believe this is highly unlikely. All the slides were examined by two eminent pathologists, who agreed that the changes were definite and probably secondary to the disease. National Institute of Allergy and Infectious Diseases, Public Health Service, Bethesda, Maryland, U.S.A.
SHELDON M. WOLFF.
CARDIAC PUNCTURE
SIR,-We must take exception to certain statements in your leading article (Nov. 7). You state that "it is doubtful whether this procedure [atrial septal puncture] is significantly safer than some of the others ". We are in the process of publishing our experience with this approach to the left side of the heart. In our series of 508 transseptal punctures there were 2 deaths (under 0-4%). This experiaccords with that of other workers who have used this method. Brockenborogh et al. reported 450 septal punctures without a death and Miller and Meddreported 65 cases with no mortality. These figures show that the mortality from this procedure is no greater than that from other major diagnostic procedures, such as cerebral angiography, which are now accepted as routine. You also state that percutaneous needle puncture of the left ventricle for assessing aortic stenosis is " simple, the successrate is high, and the risk is acceptable in competent hands ". In the original series of 26 cases reported by Brock et al.4 there were no deaths. In an attempt to obtain the aortic gradient, this method was then extended to include catheterisation of the aorta by means of a catheter passed through the needle. Later these workersreported a series of 115 cases which included the original 26 cases. There were 2 deaths (1-6%). You do not " state what you mean by success-rate ", but, if this means success in obtaining a gradient across the aortic valve by passing the catheter into the aorta, then these workers were able to do so in 28 out of 36 attempts-a success-rate of 78%. In our series it was possible to enter the left ventricle using a fine catheter passed through the trans-septal needle in 84% of patients with disease restricted to the aortic valve, the aortic gradient being obtained by simultaneously recording the brachial arterial pressure. Brockenborogh et al.2 entered the left ventricle in 95 % of 450 cases. Gray and Joshipura6 entered the left ventricle from the aorta in 21 out of 25 cases of aortic stenosis-a success-rate of 84%. The cavity of the left ventricle in aortic stenosis is very small. This makes needle puncture much more hazardous, since many stabs may be required to ence
1. 2. 3. 4. 5.
6.
Wolff, S. M. Lancet, 1964, i, 843. Brockenborogh, E. C., Braunwald, E., Ross, J., Jr. Circulation, 1962, 25, 15. Miller, B. L., Medd, W. E. Brit. Heart J. 1964, 26, 33. Brock, R., Milstein, B. B., Ross, D. N. Thorax, 1956, 11, 63. Fleming, H. A., Hancock, E. W., Milstein, B. B., Ross, D. N. ibid. 1958, 13, 97. Gray, I. R., Joshipura, C. S. Brit. Heart J. 1964, 26, 199.
introduce the needle cleanly into the cavity. This is in clear contrast to mitral incompetence, where the dilated cavity can be entered with ease and safety. We have punctured the left ventricle without a fatality in 85 patients with mitral incompetence, some of whom also had aortic stenosis. The evidence shows that aortic stenosis can be assessed with equal success by several methods other than ventricular puncture. Percutaneous needle puncture of the ventricle provides only very limited information and is in no sense an alternative diagnostic method to trans-septal catheterisation. Even that limited information may be obtained with as much success and far greater safety by means of the trans-septal technique. Charing Cross Hospital, London, W.C.2.
H. IKRAM P. G. F. NIXON.
ORAL CONTRACEPTIVES AND ACUTE INTERMITTENT PORPHYRIA
SIR,-Acute intermittent porphyria is twice as common in women as in men: the attacks sometimes come on in connection with the menarche and menstruation, and those affected often have amenorrhoea or other menstrual Severe attacks have been observed after disorders. hysterectomy and bilateral salpingo-oophorectomy or
during pregnancy. Haeger-Aronsen2 reported a case which improved afterAnovlar’ (4 mg. norethisterone and 0-05 mg. ethinylcestradiol per tablet). The following case also suggests that acute intermittent porphyria in women may be connected with endocrine factors. An unmarried woman, aged 26, who had never been pregnant, admitted to our department with an attack of acute intermittent porphyria. She had already had twenty-three attacks, on account of which she had spent altogether 341 days in hospital, the last time in December, 1963. In May, 1964, she began taking ’Lyndiol ’ (5 mg. ethinylcestradiol and 0’15 mg. mestranol per tablet) for dysmenorrhoea. In the first month after she began taking the tablets she had nausea and vomiting, and abdominal pain of a kind she had never had before. During the summer she became apprehensive and agitated, and had attacks of anxiety associated with epigastric pain. Laboratory analysis in mid-July revealed high serumwas
transaminases :
serum-glutamic-oxaloacetic-transaminase
385 Sigma-Frankel units, serum-glutamic-pyruvictransaminase (S.G.P.T.) 450 Sigma-Frankel units. At the end of August severe abdominal pain and constipation set in, and the patient’s arms and legs began to show signs of paralysis. On admission on Sept. 1, both knee-jerks were absent; the patient was depressed, and she had difficulty in concentrating and showed uneven intellectual abilities, as well as incipient mental confusion. Laboratory data were as follows :
(S.G.O.T.)
Serum-bilirubin, 0’3 mg. per 100 ml.; thymol turbidity, 8’6 Maclagan units; alkaline phosphatase, 2-8 Bessey-Lowry units; protracted bromsulphthalein-excretion rate, concentration half-time 47 minutes (as against a normal range of between 13 and 25 in our laboratory). s.G.o.T. 70 units and S.G.P.T. 74 units. Heamoglobin 77%, white blood-cells 6700 per c.mm., reticulocytes 1’2%. Urinary porphobilinogen 144 mg. per day, and urinary a aminolaevulinic acid 59 mg. a day. In view of a report of hepatic dysfunction in postmenopausal women treated with oral contraceptives,3 the lyndiol was stopped. The patient was then given adenosine monophosphoric acid4 250 mg. intramuscularly daily. After this she improved distinctly, her reflexes 1. 2. 3. 4.
Goldberg, A., Rimington, C. Diseases of Porphyrin Metabolism; p. 75. Springfield, Ill., 1962. Haeger-Aronsen, B. S. Afr. J. Lab. clin. Med. 1963, 9, 288. Eisalo, A., Jarvinen, P. A., Luukkainen, T. Brit. med. J. 1963, ii, 426. Gajdos, A., Gajdos-Török, M. Lancet, 1961, ii, 175.
conditioning of calm. An exercise which controls breathing favourably influences the psychiatric regimen. Sanatorium, Tokyo.
National Musashi
M. HIGASHI.
RENAL LESIONS IN WILSON’S DISEASE
SiR,ńDr. Hall (May 2) asks whether the histological changes reported in my paperwere due to autolysis. The tissue was not obtained in the first hour after death, it is true; but control tissues from patients with other diseases, similarly handled, were always examined simultaneously, and these changes were not seen. Moreover, the finding that the areas of histological changes frequently corresponded to the areas showing copper deposition histochemically suggests that these two observations are related. Again, these same areas were frequently surrounded by normal tissue. If autolysis had been responsible for the changes, it would have been selective autolysis, and its appearance in the areas where the copper was deposited would have been fortuitous.
I believe this is highly unlikely. All the slides were examined by two eminent pathologists, who agreed that the changes were definite and probably secondary to the disease. National Institute of Allergy and Infectious Diseases, Public Health Service, Bethesda, Maryland, U.S.A.
SHELDON M. WOLFF.
CARDIAC PUNCTURE
SIR,-We must take exception to certain statements in your leading article (Nov. 7). You state that "it is doubtful whether this procedure [atrial septal puncture] is significantly safer than some of the others ". We are in the process of publishing our experience with this approach to the left side of the heart. In our series of 508 transseptal punctures there were 2 deaths (under 0-4%). This experiaccords with that of other workers who have used this method. Brockenborogh et al. reported 450 septal punctures without a death and Miller and Meddreported 65 cases with no mortality. These figures show that the mortality from this procedure is no greater than that from other major diagnostic procedures, such as cerebral angiography, which are now accepted as routine. You also state that percutaneous needle puncture of the left ventricle for assessing aortic stenosis is " simple, the successrate is high, and the risk is acceptable in competent hands ". In the original series of 26 cases reported by Brock et al.4 there were no deaths. In an attempt to obtain the aortic gradient, this method was then extended to include catheterisation of the aorta by means of a catheter passed through the needle. Later these workersreported a series of 115 cases which included the original 26 cases. There were 2 deaths (1-6%). You do not " state what you mean by success-rate ", but, if this means success in obtaining a gradient across the aortic valve by passing the catheter into the aorta, then these workers were able to do so in 28 out of 36 attempts-a success-rate of 78%. In our series it was possible to enter the left ventricle using a fine catheter passed through the trans-septal needle in 84% of patients with disease restricted to the aortic valve, the aortic gradient being obtained by simultaneously recording the brachial arterial pressure. Brockenborogh et al.2 entered the left ventricle in 95 % of 450 cases. Gray and Joshipura6 entered the left ventricle from the aorta in 21 out of 25 cases of aortic stenosis-a success-rate of 84%. The cavity of the left ventricle in aortic stenosis is very small. This makes needle puncture much more hazardous, since many stabs may be required to ence
1. 2. 3. 4. 5.
6.
Wolff, S. M. Lancet, 1964, i, 843. Brockenborogh, E. C., Braunwald, E., Ross, J., Jr. Circulation, 1962, 25, 15. Miller, B. L., Medd, W. E. Brit. Heart J. 1964, 26, 33. Brock, R., Milstein, B. B., Ross, D. N. Thorax, 1956, 11, 63. Fleming, H. A., Hancock, E. W., Milstein, B. B., Ross, D. N. ibid. 1958, 13, 97. Gray, I. R., Joshipura, C. S. Brit. Heart J. 1964, 26, 199.
introduce the needle cleanly into the cavity. This is in clear contrast to mitral incompetence, where the dilated cavity can be entered with ease and safety. We have punctured the left ventricle without a fatality in 85 patients with mitral incompetence, some of whom also had aortic stenosis. The evidence shows that aortic stenosis can be assessed with equal success by several methods other than ventricular puncture. Percutaneous needle puncture of the ventricle provides only very limited information and is in no sense an alternative diagnostic method to trans-septal catheterisation. Even that limited information may be obtained with as much success and far greater safety by means of the trans-septal technique. Charing Cross Hospital, London, W.C.2.
H. IKRAM P. G. F. NIXON.
ORAL CONTRACEPTIVES AND ACUTE INTERMITTENT PORPHYRIA
SIR,-Acute intermittent porphyria is twice as common in women as in men: the attacks sometimes come on in connection with the menarche and menstruation, and those affected often have amenorrhoea or other menstrual Severe attacks have been observed after disorders. hysterectomy and bilateral salpingo-oophorectomy or
during pregnancy. Haeger-Aronsen2 reported a case which improved afterAnovlar’ (4 mg. norethisterone and 0-05 mg. ethinylcestradiol per tablet). The following case also suggests that acute intermittent porphyria in women may be connected with endocrine factors. An unmarried woman, aged 26, who had never been pregnant, admitted to our department with an attack of acute intermittent porphyria. She had already had twenty-three attacks, on account of which she had spent altogether 341 days in hospital, the last time in December, 1963. In May, 1964, she began taking ’Lyndiol ’ (5 mg. ethinylcestradiol and 0’15 mg. mestranol per tablet) for dysmenorrhoea. In the first month after she began taking the tablets she had nausea and vomiting, and abdominal pain of a kind she had never had before. During the summer she became apprehensive and agitated, and had attacks of anxiety associated with epigastric pain. Laboratory analysis in mid-July revealed high serumwas
transaminases :
serum-glutamic-oxaloacetic-transaminase
385 Sigma-Frankel units, serum-glutamic-pyruvictransaminase (S.G.P.T.) 450 Sigma-Frankel units. At the end of August severe abdominal pain and constipation set in, and the patient’s arms and legs began to show signs of paralysis. On admission on Sept. 1, both knee-jerks were absent; the patient was depressed, and she had difficulty in concentrating and showed uneven intellectual abilities, as well as incipient mental confusion. Laboratory data were as follows :
(S.G.O.T.)
Serum-bilirubin, 0’3 mg. per 100 ml.; thymol turbidity, 8’6 Maclagan units; alkaline phosphatase, 2-8 Bessey-Lowry units; protracted bromsulphthalein-excretion rate, concentration half-time 47 minutes (as against a normal range of between 13 and 25 in our laboratory). s.G.o.T. 70 units and S.G.P.T. 74 units. Heamoglobin 77%, white blood-cells 6700 per c.mm., reticulocytes 1’2%. Urinary porphobilinogen 144 mg. per day, and urinary a aminolaevulinic acid 59 mg. a day. In view of a report of hepatic dysfunction in postmenopausal women treated with oral contraceptives,3 the lyndiol was stopped. The patient was then given adenosine monophosphoric acid4 250 mg. intramuscularly daily. After this she improved distinctly, her reflexes 1. 2. 3. 4.
Goldberg, A., Rimington, C. Diseases of Porphyrin Metabolism; p. 75. Springfield, Ill., 1962. Haeger-Aronsen, B. S. Afr. J. Lab. clin. Med. 1963, 9, 288. Eisalo, A., Jarvinen, P. A., Luukkainen, T. Brit. med. J. 1963, ii, 426. Gajdos, A., Gajdos-Török, M. Lancet, 1961, ii, 175.