- Email: [email protected]
CURIOUS CURD
1430 in subjects with normal renal function. Accordingly, dosage intervals for procainamide can be adjusted individually and some patients can be maintained satisfactorily with intervals exceeding 4 hours. Department of Pharmacology, Cornell University Medical College, New York, N.Y. 10021,
MARCUS M. REIDENBERG.
U.S.A.
D-PENICILLAMINE AND HÆMOLYTIC ANÆMIA
SIR,-We have seen a patient with lichenoid eruptions after D-penicillamine.l This side-effect is not surprising, since many drugs used for arthritis therapy can induce lichenoid eruptions. The same drugs also produce, hsemolytic anaemias, especially in patients with a deficiency of erythrocytic glucose-6-phosphate dehydrogenase (G.-6P.D.). This may be due to the inhibition of G.-6-P.D.2,3 which would be aggravated in individuals with a prior deficiency of this enzyme. Our experience with the patient with lichenoid eruptions after penicillamine prompted us to test the effect of this drug on purified G.-6-P.D. in vitro.1 We found that it was a strong non-competitive inhibitor with a K1 of 3-5 x 10-4M. Consequently it is possible to explain why one of the side-effects of penicillamine therapy is haemolytic anxmia, as noted in your editorial (May 17, p. 1123). Thus, in our opinion, penicillamine would be contraindicated in any patients with a G.-6-P.D. deficiency. Department of Dermatology, Sint Radboudziekenhuis,
Javastraat 104,
Nijmegen, Netherlands.
W. B. J. M. VAN DER STAAK D. W. K. COTTON.
CURIOUS CURD
SIR,— We wish that
seems to
to
have
describe no
unusual paediatric case in the experience of our
an
parallel
colleagues. A boy of 2 years 8 months had a whooping-cough type of illness for one month. Before he went to bed one night, his mother gave him an 8 oz. (220 ml.) drink of warm Jersey milk mixed with approximately 15 ml. of honey to soothe the cough. In the small hours of the next morning she was woken by the sound of his violent coughing, and found him lying face down and cyanosed, with a white object just protruding from his mouth. This was extricated with immediate improvement in the child’s condition. The " object" (see accompanying figure) had the appearance, odour, and consistency of curdled milk, and two similar but
much smaller curds were produced subsequently, each time after drinking milk to which, on these occasions, a teaspoonful of sugar had been added. Examination a few hours after the first episode revealed no abnormalities except otitis media, and he has had a barium meal and follow-through which was normal. His past history is unremarkable, immunisations are complete to date, and he now remains in good health.
Inspissated formula milk causing intestinal obstruction der Staak, W. B. J. M., Cotton, D. W. K., Jonckheer-Vanneste, M. M. H. Dermatologica (in the press). 2. Cotton, D. W. K., Sutorius, A. H. M. Nature, 1971, 233, 197. 3. Cotton, D. W. K., van den Hurk, J. J. M. A., van der Staak, W. B. J. M. Br. J. Derm. 1972, 87, 341. 1.
van
is
a
well-recognised problem in the premature infant,1
but a search of the literature has revealed no similar cases to this one. Majd and Lopresti2 described an infant, and reviewed 4 others, in whom over-concentrated powdered milk and water produced a radiologically visible coagulum, " a lactobezoar ", in the stomach; but in our case dairy milk was given to an older child. The illustration shows that the curd could be a cast of the duodenum or oesophagus, but how it formed and precisely where perplexes us. Do any of your readers know of a similar phenomenon ? Schopwick, Elstree, PETER BENNETT. Herts. WD6 3EX. Central Middlesex Hospital, Acton Lane, London NW10 7NS
STEPHEN HERMAN.
FACTOR-VIII COMPLEX AND ENDOTHELIAL DAMAGE SIR,-We agree with Dr Ekberg and Professor Nilsson (May 17, p. 1111) that the level of factor VIII seems to be a pointer to the severity of vessel damage in glomerulonephritis. The levels of factor VIII antigen (Laurell’s immunoelectrophoresis 3) and VIII w (quantitative ristocetin assay 4) were studied by us in several groups of patients with clinical conditions of endothelial injury:
Group 1.-60 healthy controls. Group II.—42 patients with arteritis of the lower limbs, subdivided into ua (23 patients with minor segmentary damage) and lib (19 patients with extensive injury). The classification into (a) or (b) was made by the surgeon without knowledge of the biological findings, after angiographic examination. Group III.—34 diabetics subdivided into ilia (17 patients without clinical angiopathy) and IIIb (17 patients with obvious vascular manifestations). Group IV.—23 patients with gram-negative sepsis and thrombocytopenia, a clinical condition known to produce endothelial injury.1i The results summarised in the table show MEANS
(AND 95%
CONFIDENCE
INTERVALS)
FOR VIII
a
significant
Ag
AND VIII W
0001, t P < 0.01, p < 005 for comparison with group I by analysis of variance. increase in VIII Ag and VIII w in all the groups of patients in comparison with the controls. The level of VIII Ag seems linked to the severity of endothelial damage: significant differences between groups lia and lib (p < 0-001) and between groups ilia and IIIb (p < 0001). VIII Ag is synthesised by endothelial cells 6; it plays a key role in platelet adhesion to subendothelial structures, and, therefore, in the first stage of arterial thrombosis. Thus if VIII Ag is an indicator of endothelial damage, it may be possibly a contributory factor of thrombosis in *P <
arterial disease. Laboratoire d’Hémostase, Centre de Transfusion Sanguine, 31052 Toulouse Cedex, France.
B. BONEU M. ABBAL
J. PLANTE R. BIERME.
1. Br. med. J. 1969, iv, 633. 2. Majd, M., Lopresti, J. M. Am. J. Roentgenol. Radium Ther. nucl. Med. 1972, 116, 575. 3. Laurell, C. B. Analyt. Biochem. 1966, 15, 45. 4. Weiss, H. J., Hoyer, L. W., Rickles, F. R., Varma, A., Roger, J. J. clin. Invest. 1973, 52, 2708. 5. Bouvier, C. A., Gaynor, E., Cintron, J. R., Bernhardt, B., Spaet, Th., Thromb. Diath. Hœmorrh. 1970, 40. suppl. 163. 6. Jaffe, E. A., Hoyer, L. W., Nachman, R. L. J. clin. Invest. 1973, 52, 2757.
,
MARCUS M. REIDENBERG.
U.S.A.
D-PENICILLAMINE AND HÆMOLYTIC ANÆMIA
SIR,-We have seen a patient with lichenoid eruptions after D-penicillamine.l This side-effect is not surprising, since many drugs used for arthritis therapy can induce lichenoid eruptions. The same drugs also produce, hsemolytic anaemias, especially in patients with a deficiency of erythrocytic glucose-6-phosphate dehydrogenase (G.-6P.D.). This may be due to the inhibition of G.-6-P.D.2,3 which would be aggravated in individuals with a prior deficiency of this enzyme. Our experience with the patient with lichenoid eruptions after penicillamine prompted us to test the effect of this drug on purified G.-6-P.D. in vitro.1 We found that it was a strong non-competitive inhibitor with a K1 of 3-5 x 10-4M. Consequently it is possible to explain why one of the side-effects of penicillamine therapy is haemolytic anxmia, as noted in your editorial (May 17, p. 1123). Thus, in our opinion, penicillamine would be contraindicated in any patients with a G.-6-P.D. deficiency. Department of Dermatology, Sint Radboudziekenhuis,
Javastraat 104,
Nijmegen, Netherlands.
W. B. J. M. VAN DER STAAK D. W. K. COTTON.
CURIOUS CURD
SIR,— We wish that
seems to
to
have
describe no
unusual paediatric case in the experience of our
an
parallel
colleagues. A boy of 2 years 8 months had a whooping-cough type of illness for one month. Before he went to bed one night, his mother gave him an 8 oz. (220 ml.) drink of warm Jersey milk mixed with approximately 15 ml. of honey to soothe the cough. In the small hours of the next morning she was woken by the sound of his violent coughing, and found him lying face down and cyanosed, with a white object just protruding from his mouth. This was extricated with immediate improvement in the child’s condition. The " object" (see accompanying figure) had the appearance, odour, and consistency of curdled milk, and two similar but
much smaller curds were produced subsequently, each time after drinking milk to which, on these occasions, a teaspoonful of sugar had been added. Examination a few hours after the first episode revealed no abnormalities except otitis media, and he has had a barium meal and follow-through which was normal. His past history is unremarkable, immunisations are complete to date, and he now remains in good health.
Inspissated formula milk causing intestinal obstruction der Staak, W. B. J. M., Cotton, D. W. K., Jonckheer-Vanneste, M. M. H. Dermatologica (in the press). 2. Cotton, D. W. K., Sutorius, A. H. M. Nature, 1971, 233, 197. 3. Cotton, D. W. K., van den Hurk, J. J. M. A., van der Staak, W. B. J. M. Br. J. Derm. 1972, 87, 341. 1.
van
is
a
well-recognised problem in the premature infant,1
but a search of the literature has revealed no similar cases to this one. Majd and Lopresti2 described an infant, and reviewed 4 others, in whom over-concentrated powdered milk and water produced a radiologically visible coagulum, " a lactobezoar ", in the stomach; but in our case dairy milk was given to an older child. The illustration shows that the curd could be a cast of the duodenum or oesophagus, but how it formed and precisely where perplexes us. Do any of your readers know of a similar phenomenon ? Schopwick, Elstree, PETER BENNETT. Herts. WD6 3EX. Central Middlesex Hospital, Acton Lane, London NW10 7NS
STEPHEN HERMAN.
FACTOR-VIII COMPLEX AND ENDOTHELIAL DAMAGE SIR,-We agree with Dr Ekberg and Professor Nilsson (May 17, p. 1111) that the level of factor VIII seems to be a pointer to the severity of vessel damage in glomerulonephritis. The levels of factor VIII antigen (Laurell’s immunoelectrophoresis 3) and VIII w (quantitative ristocetin assay 4) were studied by us in several groups of patients with clinical conditions of endothelial injury:
Group 1.-60 healthy controls. Group II.—42 patients with arteritis of the lower limbs, subdivided into ua (23 patients with minor segmentary damage) and lib (19 patients with extensive injury). The classification into (a) or (b) was made by the surgeon without knowledge of the biological findings, after angiographic examination. Group III.—34 diabetics subdivided into ilia (17 patients without clinical angiopathy) and IIIb (17 patients with obvious vascular manifestations). Group IV.—23 patients with gram-negative sepsis and thrombocytopenia, a clinical condition known to produce endothelial injury.1i The results summarised in the table show MEANS
(AND 95%
CONFIDENCE
INTERVALS)
FOR VIII
a
significant
Ag
AND VIII W
0001, t P < 0.01, p < 005 for comparison with group I by analysis of variance. increase in VIII Ag and VIII w in all the groups of patients in comparison with the controls. The level of VIII Ag seems linked to the severity of endothelial damage: significant differences between groups lia and lib (p < 0-001) and between groups ilia and IIIb (p < 0001). VIII Ag is synthesised by endothelial cells 6; it plays a key role in platelet adhesion to subendothelial structures, and, therefore, in the first stage of arterial thrombosis. Thus if VIII Ag is an indicator of endothelial damage, it may be possibly a contributory factor of thrombosis in *P <
arterial disease. Laboratoire d’Hémostase, Centre de Transfusion Sanguine, 31052 Toulouse Cedex, France.
B. BONEU M. ABBAL
J. PLANTE R. BIERME.
1. Br. med. J. 1969, iv, 633. 2. Majd, M., Lopresti, J. M. Am. J. Roentgenol. Radium Ther. nucl. Med. 1972, 116, 575. 3. Laurell, C. B. Analyt. Biochem. 1966, 15, 45. 4. Weiss, H. J., Hoyer, L. W., Rickles, F. R., Varma, A., Roger, J. J. clin. Invest. 1973, 52, 2708. 5. Bouvier, C. A., Gaynor, E., Cintron, J. R., Bernhardt, B., Spaet, Th., Thromb. Diath. Hœmorrh. 1970, 40. suppl. 163. 6. Jaffe, E. A., Hoyer, L. W., Nachman, R. L. J. clin. Invest. 1973, 52, 2757.
,