1339
analyses of chromosomal banding-patterns
are
in progress.
Dr Martin R. Klemperer referred this patient for cytogenetic Carol Goodman and Linda Osowski gave valuable studies. technical assistance.
This work was supported in part from the National Cancer Institute. University of Rochester and Monroe Community Hospital, Rochester, N.Y. 14603, U.S.A.
by
research grant CA-14876
KONG-OO GOH.
primarily caused by dietary factors. It can be induced experimentally by diets high in carbohydrate but low in protein and fat, and the incidence can also be influenced by environmental factors such as fasting, temperature, and stress. The incidence of the disorder can be reduced by increasing fat and protein levels in the diet, and the disease can be totally prevented by increasing the dietary levels of biotin. However, classical symptoms of biotin deficiency are not observed in the affected birds. Hepatic gluconeogenesis activity is severely depressed in F.L.K.S., but recovery to almost normal levels is obtained by biotin both in vivo and in vitro. IS In both conditions hypoglycsemia is thought to be an important factor in causing death. In view of the established role of biotin in gluconeogenesis and its success in preventing the avian disease, some consideration could be given to the role of dietary cofactors in the treatment and prevention of Reye’s syndrome. In any event, F.L.K.S. may be a suitable model for Reye’s syndrome, whether the latter has a primary nutritional cause or results from the interference of other aetiological agents in a similar metabolic pathway.
treatment
REYE’S SYNDROME OF CHILDREN AND THE FATTY-LIVER-AND-KIDNEY SYNDROME OF CHICKENS SIR,-It has been brought to our attention (Prof. J. B. Cavanagh, personal communication) that Reye’s syndrome (encephalopathy and fatty degeneration of the viscera) has many features in common with a condition we are studying in the fowl called the fatty-liver-and-kidney syndrome (F.L.K.S.). Reye’s disease occurs sporadically in many countries, and it is reported to be a leading cause of childhood deaths in Thailand.l Its aetiology is obscure and its pathogenesis poorly understood.2,3 It has been suggested3 that an understanding of this disorder would be aided by further study in animals. F.L.K.S., as a naturally occurring disease which is also experimentally reproducible, may provide a model for the human disorder. Various papers on F.L.K.S. deal with pathology,4-7 biochemistry,8-1o
experimental induction,11.12 and prevention. 13. 14 Both diseases affect the young, causing acute disturbances of consciousness, including coma; the mortality-rate is high. In both there is striking hypoglycaemia, raised blood free-fatty-acid and pyruvate levels, reduced hepatic glycogen, and excessive accumulation of lipid (mainly triglyceride) in the liver, proximal tubules of the kidneys, and myocardium (usually unaccompanied by cellular degeneration or inflammatory reactions). No specific
P. A. L. WIGHT W. G. SILLER A. J. EVANS D. W. BANNISTER C. C. WHITEHEAD R. BLAIR.
Agricultural Research Council’s Poultry Research Centre, King’s Buildings, West Mains Road, EH9 3JS.
Edinburgh
PLATELETS IN HUMAN URINE ?
SIR,-Most of us are concerned with the platelet in blood, but there are no reports about the platelet in urine. One of us (N. K.) has examined urinary sediment from normal and sick persons and has observed a small body
infective or toxic agent has been identified in either the human or the avian disease. There are, however, dissimilarities. Cerebral oedema, which occurs in Reye’s syndrome, is not seen in F.L.K.S. although an unusual deposition of lipid involves the astrocytes. Dyspnoea and pulmonary congestion are also features of Reye’s syndrome which do not occur in F.L.K.S. Serum-aspartate-aminotransferase levels are raised in Reye’s syndrome; this has not been tested in F.L.K.S., but in affected birds isocitric dehydrogenase, leucine aminopeptidase, and cholinesterase levels are within normal limits. Most investigators agree that Reye’s syndrome is a clinical and pathophysiological entity, but the suspected causes are multiple and varied, as indicated by the different virus diseases and agents, such as mycotoxins, which have been associated with it. No prodromal illness occurs in F.L.K.S., which has been found to be a metabolic disorder 1. Olson, L., Bourgeois, C. H., Cotton, R. B., Harikul, S., Grossman, R. A., Smith, T. J. Pediatrics, Springfield, 1971, 47, 707. 2. Schubert, W. K., Partin, J. C., Partin, J. S. Progr. Liver Dis. 1972, 4, 489. 3. Mowat, A. P. Archs Dis. Childh. 1973, 48, 411. 4. Wight, P. A. L., Siller, W. G. Res. vet. Sci. (in the press). 5. Siller, W. G., Wight, P. A. L. ibid. 6. Wight, P. A. L. Neuropath. appl. Neurobiol. (in the press). 7. Wight, P. A. L. J. comp. Path. Ther. (in the press). 8. Whitehead, C. C. Res. vet. Sci. 1975, 18, 32. 9. Evans, A. J., Bannister, D. W., Whitehead, C. C. ibid. p. 26. 10. Bannister, D. W., Evans, A. J., Whitehead, C. C. ibid. p. 149. 11. Whitehead, C. C., Blair, R. ibid. 1974, 17, 86. 12. Whitehead, C. C., Bannister, D. W., Blair, R., Evans, A. J. ibid. p. 222. 13. Whitehead, C. C., Blair, R., Bannister, D. W., Evans, A. J., Jones, R. M. ibid. (in the press). 14. Payne, C. G., Gilchrist, P., Pearson, J. A., Hemsley, L. A. Br. Poult. Sci. 1974, 15, 489.
Scanning electron microscopic findings The upper small
body
is
of urine sediment.
dendritic, the lower is discoid.
( x 2500.) __________
resembling
a
platelet in size,
appearance, and
granular
content.
We have compared the platelet-like body in urine with normal platelets in blood and also with platelets in normal urine supernatant to which had been added platelet-rich 15. Bannister, D. W. Comp. Biochem. Physiol. (in the press).
analyses of chromosomal banding-patterns
are
in progress.
Dr Martin R. Klemperer referred this patient for cytogenetic Carol Goodman and Linda Osowski gave valuable studies. technical assistance.
This work was supported in part from the National Cancer Institute. University of Rochester and Monroe Community Hospital, Rochester, N.Y. 14603, U.S.A.
by
research grant CA-14876
KONG-OO GOH.
primarily caused by dietary factors. It can be induced experimentally by diets high in carbohydrate but low in protein and fat, and the incidence can also be influenced by environmental factors such as fasting, temperature, and stress. The incidence of the disorder can be reduced by increasing fat and protein levels in the diet, and the disease can be totally prevented by increasing the dietary levels of biotin. However, classical symptoms of biotin deficiency are not observed in the affected birds. Hepatic gluconeogenesis activity is severely depressed in F.L.K.S., but recovery to almost normal levels is obtained by biotin both in vivo and in vitro. IS In both conditions hypoglycsemia is thought to be an important factor in causing death. In view of the established role of biotin in gluconeogenesis and its success in preventing the avian disease, some consideration could be given to the role of dietary cofactors in the treatment and prevention of Reye’s syndrome. In any event, F.L.K.S. may be a suitable model for Reye’s syndrome, whether the latter has a primary nutritional cause or results from the interference of other aetiological agents in a similar metabolic pathway.
treatment
REYE’S SYNDROME OF CHILDREN AND THE FATTY-LIVER-AND-KIDNEY SYNDROME OF CHICKENS SIR,-It has been brought to our attention (Prof. J. B. Cavanagh, personal communication) that Reye’s syndrome (encephalopathy and fatty degeneration of the viscera) has many features in common with a condition we are studying in the fowl called the fatty-liver-and-kidney syndrome (F.L.K.S.). Reye’s disease occurs sporadically in many countries, and it is reported to be a leading cause of childhood deaths in Thailand.l Its aetiology is obscure and its pathogenesis poorly understood.2,3 It has been suggested3 that an understanding of this disorder would be aided by further study in animals. F.L.K.S., as a naturally occurring disease which is also experimentally reproducible, may provide a model for the human disorder. Various papers on F.L.K.S. deal with pathology,4-7 biochemistry,8-1o
experimental induction,11.12 and prevention. 13. 14 Both diseases affect the young, causing acute disturbances of consciousness, including coma; the mortality-rate is high. In both there is striking hypoglycaemia, raised blood free-fatty-acid and pyruvate levels, reduced hepatic glycogen, and excessive accumulation of lipid (mainly triglyceride) in the liver, proximal tubules of the kidneys, and myocardium (usually unaccompanied by cellular degeneration or inflammatory reactions). No specific
P. A. L. WIGHT W. G. SILLER A. J. EVANS D. W. BANNISTER C. C. WHITEHEAD R. BLAIR.
Agricultural Research Council’s Poultry Research Centre, King’s Buildings, West Mains Road, EH9 3JS.
Edinburgh
PLATELETS IN HUMAN URINE ?
SIR,-Most of us are concerned with the platelet in blood, but there are no reports about the platelet in urine. One of us (N. K.) has examined urinary sediment from normal and sick persons and has observed a small body
infective or toxic agent has been identified in either the human or the avian disease. There are, however, dissimilarities. Cerebral oedema, which occurs in Reye’s syndrome, is not seen in F.L.K.S. although an unusual deposition of lipid involves the astrocytes. Dyspnoea and pulmonary congestion are also features of Reye’s syndrome which do not occur in F.L.K.S. Serum-aspartate-aminotransferase levels are raised in Reye’s syndrome; this has not been tested in F.L.K.S., but in affected birds isocitric dehydrogenase, leucine aminopeptidase, and cholinesterase levels are within normal limits. Most investigators agree that Reye’s syndrome is a clinical and pathophysiological entity, but the suspected causes are multiple and varied, as indicated by the different virus diseases and agents, such as mycotoxins, which have been associated with it. No prodromal illness occurs in F.L.K.S., which has been found to be a metabolic disorder 1. Olson, L., Bourgeois, C. H., Cotton, R. B., Harikul, S., Grossman, R. A., Smith, T. J. Pediatrics, Springfield, 1971, 47, 707. 2. Schubert, W. K., Partin, J. C., Partin, J. S. Progr. Liver Dis. 1972, 4, 489. 3. Mowat, A. P. Archs Dis. Childh. 1973, 48, 411. 4. Wight, P. A. L., Siller, W. G. Res. vet. Sci. (in the press). 5. Siller, W. G., Wight, P. A. L. ibid. 6. Wight, P. A. L. Neuropath. appl. Neurobiol. (in the press). 7. Wight, P. A. L. J. comp. Path. Ther. (in the press). 8. Whitehead, C. C. Res. vet. Sci. 1975, 18, 32. 9. Evans, A. J., Bannister, D. W., Whitehead, C. C. ibid. p. 26. 10. Bannister, D. W., Evans, A. J., Whitehead, C. C. ibid. p. 149. 11. Whitehead, C. C., Blair, R. ibid. 1974, 17, 86. 12. Whitehead, C. C., Bannister, D. W., Blair, R., Evans, A. J. ibid. p. 222. 13. Whitehead, C. C., Blair, R., Bannister, D. W., Evans, A. J., Jones, R. M. ibid. (in the press). 14. Payne, C. G., Gilchrist, P., Pearson, J. A., Hemsley, L. A. Br. Poult. Sci. 1974, 15, 489.
Scanning electron microscopic findings The upper small
body
is
of urine sediment.
dendritic, the lower is discoid.
( x 2500.) __________
resembling
a
platelet in size,
appearance, and
granular
content.
We have compared the platelet-like body in urine with normal platelets in blood and also with platelets in normal urine supernatant to which had been added platelet-rich 15. Bannister, D. W. Comp. Biochem. Physiol. (in the press).
