824 sitive groups. Harvey’s comparison of "mean liver-cadmium concentration" of 11 p.p.m. with very much higher values of up to 260 p.p.m. in over 600 industrially exposed workers may be misleading. It is not correct to compare a mean value with an upper value of a range. Furthermore the ratio for cadmium concentrations in liver to kidney will increase with increasing exposure, as is seen in industrial workers. The Shipham incident is the first in the general population outside Japan where concentrations of cadmium have been reported in a range which may well give rise to symptoms. We do not yet know whether symptoms have occurred. In certain age-groups there was a high prevalence of increased excretion of &bgr;2-microglobulin in urine. Not enough information is provided about the sampling of the villagers studied to permit firm conclusions. However, the cadmium levels reported would fit with the possibility of early signs of tubular dysfunction. I agree that detailed studies in Shipham are very important-not only for the sake of the villagers themselves but also because such an investigation might serve as a model example for future environmental problems. Department of Environmental Hygiene, Karolinska Institute,
EXCHANGE TRANSFUSION WITH HEPARINISED FRESH BLOOD IN NECROTISING ENTEROCOLITIS
SIR,-Dr Seger and colleagues (Jan. 6, p. 48) have observed premature baby with fulminant necrotising enterocolitis (N.E.C.) and Clostridium perfringens bacteraemia. Some clostridia produce neuraminidase which can expose the ThomsenFriedenreich (T) antigen. Because of T activation the patient’s red blood-cells were agglutinated by normal human sera as well as by peanut agglutinin (anti-T lectin from Arachis hypogœa). T-activated red cells can react with T agglutinin of sera of blood-donors. Seger et al. suggest giving washed red bloodcells, if blood-transfusions are needed in such cases, and they advise against exchange transfusions with whole blood, because of the theoretical risk of microemboli due to agglutinated red blood-cells. In our experience, however, these potential hazards of exchange transfusion with heparinised fresh blood in patients with T-activation have not been realised. We have seen 24 children with T activation in vivo (see table). 7 had haemolytic uraemic syndrome (5 died) and 4 had
a
RESULTS IN CHILDREN WITH T ACTIVATION IN VIVO
LARS FRIBERG
S-104 01 Stockholm 60, Sweden
(1970-78)*
PLATELET TAURINE UPTAKE IN RETINITIS
PIGMENTOSA
SIR,-In their paper on platelet uptake of taurine in retinitis pigmentosa (R.P.) Dr Airaksinen and colleagues, like so many earlier workers doing research of L.R.P., fail to separate their patients by genetic history. As Bird’ has pointed out, R.P. is very probably a heterogeneous group of conditions with similar clinical pictures but differing in pathogenesis. Information about the genetic history of the patients and breakdown of the data would make Airaksinen’s paper more informative. If Airaksinen et al. have shown an abnormality of platelet taurine uptake in patients with genetically distinct varieties of R.P., this would make their results even more interesting. Departments of Ophthalmology and Medical Genetics, University of British Columbia, Vancouver, British Columbia, Canada V5Z 3N9
JEAN D. A. CARRUTHERS
** This letter has been shown to DrAiraksinen and Dr P. Sihvola, whose
reply follows.-ED.L. SIR,-All but one of the patients studied had no known relatives with the same disease. Thus eleven of our twelve patients probably have the recessive (or sex-linked) form of retinitis pigmentosa. One female patient whose father has the same disease may have the dominantly inherited form of the disease, and she also showed diminished uptake of taurine into her platelets compared with the daily control value. None of our patients had any other defects such as hearing defects or convulsions, and they all were mentally normal. Department of Pharmacology, University of Kuopio, 70101 Kuopio 10, Finland
Ophthalmic Clinic, University Central Hospital, Kuopio
*Age 2 days to 61 months (mean 22 months). t3 not requiring blood-transfusions.
(3 died). Exchange transfusion with heparinised fresh done in 4 children. No complications during or after exchange transfusion were seen. On the contrary, impressive beneficial effects were noticed. 1 newborn baby had milk-plug syndrome, and peritonitis and severe haemolysis due to C. perfringens infection developed after surgical intervention.’ By an immunofluorescent technique, with purified peanut agglutinin and fluorescein-labelled anti-peanut globulin serum of rabbits,2 we determined the proportion of T-activated red cells. After exchange transfusion with three times the blood volume of the baby the proportion of T-activated red blood-cells fell N.E.C.
blood
from 100% to 2%. This is the same percentage as that seen after exchange transfusion for non-antibody-mediated hyperbilirubinxmia. During exchange transfusion no increased destruction of T-activated patient’s red blood-cells was detectable. Possibly, heparinisation of the donor blood prevents hxmolysis of T-activated cells, even in the presence of T agglutinin. Thus, we could not substantiate the theoretically based warnof Seger et al. In our experience exchange transfusion with heparinised fresh blood is of benefit in patients with T-activation as well as in patients with septicxmia of other
ings
origin.3,4 E. M. AIRAKSINEN
P. SIHVOLA
*** We apologise to Dr Airaksinen and colleagues for an editorial in the Discussion in their paper. The fourth sentence should read: "The mechanism responsible for binding taurine to plasma membranes in platelets’" and synaptosomes" is
Clostridia produce not only neuraminidase but also other toxins which may be more dangerous than neuraminidase itself. Besides red-cell alteration, neuraminidase (and, probably, other bacterial toxins) can lead to dysfunction of several 1.
error
poorly known."-ED.L. 1.
Bird, A. C. Trans. ophthal Soc. N.Z. 1977, 29, 51.
was
Poschmann, 18.
A. in Praktische Transfusionsmedizin
(edited by H. Zöckler , p.
Gräfelfing, 1977.
2.
Poschmann, A., Fischer, K., Reuther, K., Myllylä, G. Vox Sang. 1973. 24,
3.
Belohradsky, B. H., Muntean, W., Riegel, K., Marget, heilk. 1977, 125, 590.
4.
Pohlandt, F., Töllner, U., Fried, F., Hennchs, I. in Pädiatrische Intensivmedizin (edited by P. Emmnch); p. 205. Stuttgart, 1975.
489.
W. Mschr. Kinder-
EXCHANGE TRANSFUSION WITH HEPARINISED FRESH BLOOD IN NECROTISING ENTEROCOLITIS
SIR,-Dr Seger and colleagues (Jan. 6, p. 48) have observed premature baby with fulminant necrotising enterocolitis (N.E.C.) and Clostridium perfringens bacteraemia. Some clostridia produce neuraminidase which can expose the ThomsenFriedenreich (T) antigen. Because of T activation the patient’s red blood-cells were agglutinated by normal human sera as well as by peanut agglutinin (anti-T lectin from Arachis hypogœa). T-activated red cells can react with T agglutinin of sera of blood-donors. Seger et al. suggest giving washed red bloodcells, if blood-transfusions are needed in such cases, and they advise against exchange transfusions with whole blood, because of the theoretical risk of microemboli due to agglutinated red blood-cells. In our experience, however, these potential hazards of exchange transfusion with heparinised fresh blood in patients with T-activation have not been realised. We have seen 24 children with T activation in vivo (see table). 7 had haemolytic uraemic syndrome (5 died) and 4 had
a
RESULTS IN CHILDREN WITH T ACTIVATION IN VIVO
LARS FRIBERG
S-104 01 Stockholm 60, Sweden
(1970-78)*
PLATELET TAURINE UPTAKE IN RETINITIS
PIGMENTOSA
SIR,-In their paper on platelet uptake of taurine in retinitis pigmentosa (R.P.) Dr Airaksinen and colleagues, like so many earlier workers doing research of L.R.P., fail to separate their patients by genetic history. As Bird’ has pointed out, R.P. is very probably a heterogeneous group of conditions with similar clinical pictures but differing in pathogenesis. Information about the genetic history of the patients and breakdown of the data would make Airaksinen’s paper more informative. If Airaksinen et al. have shown an abnormality of platelet taurine uptake in patients with genetically distinct varieties of R.P., this would make their results even more interesting. Departments of Ophthalmology and Medical Genetics, University of British Columbia, Vancouver, British Columbia, Canada V5Z 3N9
JEAN D. A. CARRUTHERS
** This letter has been shown to DrAiraksinen and Dr P. Sihvola, whose
reply follows.-ED.L. SIR,-All but one of the patients studied had no known relatives with the same disease. Thus eleven of our twelve patients probably have the recessive (or sex-linked) form of retinitis pigmentosa. One female patient whose father has the same disease may have the dominantly inherited form of the disease, and she also showed diminished uptake of taurine into her platelets compared with the daily control value. None of our patients had any other defects such as hearing defects or convulsions, and they all were mentally normal. Department of Pharmacology, University of Kuopio, 70101 Kuopio 10, Finland
Ophthalmic Clinic, University Central Hospital, Kuopio
*Age 2 days to 61 months (mean 22 months). t3 not requiring blood-transfusions.
(3 died). Exchange transfusion with heparinised fresh done in 4 children. No complications during or after exchange transfusion were seen. On the contrary, impressive beneficial effects were noticed. 1 newborn baby had milk-plug syndrome, and peritonitis and severe haemolysis due to C. perfringens infection developed after surgical intervention.’ By an immunofluorescent technique, with purified peanut agglutinin and fluorescein-labelled anti-peanut globulin serum of rabbits,2 we determined the proportion of T-activated red cells. After exchange transfusion with three times the blood volume of the baby the proportion of T-activated red blood-cells fell N.E.C.
blood
from 100% to 2%. This is the same percentage as that seen after exchange transfusion for non-antibody-mediated hyperbilirubinxmia. During exchange transfusion no increased destruction of T-activated patient’s red blood-cells was detectable. Possibly, heparinisation of the donor blood prevents hxmolysis of T-activated cells, even in the presence of T agglutinin. Thus, we could not substantiate the theoretically based warnof Seger et al. In our experience exchange transfusion with heparinised fresh blood is of benefit in patients with T-activation as well as in patients with septicxmia of other
ings
origin.3,4 E. M. AIRAKSINEN
P. SIHVOLA
*** We apologise to Dr Airaksinen and colleagues for an editorial in the Discussion in their paper. The fourth sentence should read: "The mechanism responsible for binding taurine to plasma membranes in platelets’" and synaptosomes" is
Clostridia produce not only neuraminidase but also other toxins which may be more dangerous than neuraminidase itself. Besides red-cell alteration, neuraminidase (and, probably, other bacterial toxins) can lead to dysfunction of several 1.
error
poorly known."-ED.L. 1.
Bird, A. C. Trans. ophthal Soc. N.Z. 1977, 29, 51.
was
Poschmann, 18.
A. in Praktische Transfusionsmedizin
(edited by H. Zöckler , p.
Gräfelfing, 1977.
2.
Poschmann, A., Fischer, K., Reuther, K., Myllylä, G. Vox Sang. 1973. 24,
3.
Belohradsky, B. H., Muntean, W., Riegel, K., Marget, heilk. 1977, 125, 590.
4.
Pohlandt, F., Töllner, U., Fried, F., Hennchs, I. in Pädiatrische Intensivmedizin (edited by P. Emmnch); p. 205. Stuttgart, 1975.
489.
W. Mschr. Kinder-
