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Thrombocytosis associated with the mucocutaneous lymph node syndrome
688
Letters to the Editor
findings of Smith and associates' suggest that in vitro insulin abolishes the stimulatory effect of cortisol on lecithin synthesis. o n e is tempted to speculate, as the authors do, that "'these observations are of potential clinical relevance since the incidence of the respiratory distress syndrome is greater in the infant of the diabetic mother than in the normal population." However, one is left without an apparent explanation for the observation that the most severe forms o f maternal diabetes (classes DEF) are associated with lecithin/sphingomyelin ratio in fetal lung which is markedly accelerated as compared to those of normal pregnancies? Either the in vitro biochemical results do not find their clinical analogue in the infant of diabetic mother or factor(s) associated with the angiopathy of DEF diabetic mothers (increased cortisol production?) counteract the suppressive effect of insulin on lecithin synthesis. It would therefore be of interest to investigate in the setting of Smith and associates' whether a further increase of cortisol concentration in the medium would overcome the unfavorable effect of insulin on the lecithin synthesis. Dimitris A nagnostakis, M.D. Neonatal Unit Catherine Dacou- Voutetakis, M.D. Endocrine Unit First Department of Pediatrics of A thens University Athens 608 Greece REFERENCES l.
2. 3.
Smith BT, Giroud CJP, Robert M, and Avery ME: Insulin antagonism of cortisol action on lecithin synthesis by cultured fetal lung cells, J PEDIATR 87:953, 1975. Avery ME, and Frantz I III, Commentary: Intrauterine developmental retardation, J PEDIATR 87:956, 1975. Cluck L, and Kulovich M: Fetallung development. Current concepts, Pediatr Clin North Am 20:367, 1973.
Thrombocytosis associated with the mucocutaneous lymph node syndrome To the Editor: We wish to add an additional case of mucocutaneous lymph node syndrome (MLNS) seen in the continental United States to the three cases recently reported by Brown and associates.' Our patient also demonstrated a thrombocytosis during the convalescent stage o f his illness which has not previously been reported with this syndrome, CASE REPORT
An 8-year-old black boy was admitted with a history and, physical findings as described by Kawasaki and associatesY Laboratory data were compatible with a diagnosis of mucocuta-
The Journal of Pediatrics October 1976
neous lymph node syndrome;-' specific tests for infectious diseases or collagen disorders were negative. Exfoliation of the exanthem began on the twelfth hospital day at which time the temperature returned to normal. The child was discharged on the sixteenth hospital day without symptoms or abnormal physical findings. A progressive increase in platelet count, beginning on the fifth hospital day, reached a peak of 910,000/mm ~ on the fourteenth and decreased to 724,000/mm:' on the sixteenth day. One week after discharge, the platelet count was 380,000/mm ~. DISCUSSION Thrombocytosis, to our knowledge, has not previously been associated with MLNS, and this finding is of interest in that MLNS has an estimated mortality rate of 1 to 2%~ due to thrombosis of the coronary artery? Vasculitis of the coronary arteries has been documented in MLNS ~ but an associated thrombocytosis might predispose or contribute to thrombus formation. We wonder if Dr. Brown and his co-workers or others have found a significant elevation in platelet count in their patients with MLNS. We agree with Dr. Brown that physicians should be made aware that this syndrome can occur in the United States and, hopefully, with increasing reports, more can be learned about its true incidence, etiology, and treatment. Miroslav Kovacevic, M.D. H. Garry Gardner, M.D. Department of Pediatrics Loyola University Stritch School of Medicine Maywood, Ill. 60153 REFERENCES
1.
2.
3.
Brown JS, Billmeier GJ, Cox F, Ibrahim M, Stepp WP, and Gibson R: Mucocutaneous lymph node syndrome in the continental United States, J PEDIATR 88:81, 1976. Kawasaki T, Kosaki F, Okawa S, Shigematsu I, and Yanagawa H: A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan, Pediatrics 54:271, 1974. Yanagisawa M, Kobayashi N, and Matsuya S: Myocardial infarction due to coronary thromboa/teritis, following acute febrile mucocutaneous lymph node syndrome (MLNS) in an infant, Pediatrics 54:277, 1974.
Dr. Brown was given an opportunity to answer the question posed in this letter; he has not replied. Editor
On genetic variants in fucosidosis To the Editor: We read with interest the article "Fucosidosis detection of carrier state in peripheral blood leukocytes" from Beratis and associates.' Our findings on a-L-fucosidase activity in leukocytes
Letters to the Editor
findings of Smith and associates' suggest that in vitro insulin abolishes the stimulatory effect of cortisol on lecithin synthesis. o n e is tempted to speculate, as the authors do, that "'these observations are of potential clinical relevance since the incidence of the respiratory distress syndrome is greater in the infant of the diabetic mother than in the normal population." However, one is left without an apparent explanation for the observation that the most severe forms o f maternal diabetes (classes DEF) are associated with lecithin/sphingomyelin ratio in fetal lung which is markedly accelerated as compared to those of normal pregnancies? Either the in vitro biochemical results do not find their clinical analogue in the infant of diabetic mother or factor(s) associated with the angiopathy of DEF diabetic mothers (increased cortisol production?) counteract the suppressive effect of insulin on lecithin synthesis. It would therefore be of interest to investigate in the setting of Smith and associates' whether a further increase of cortisol concentration in the medium would overcome the unfavorable effect of insulin on the lecithin synthesis. Dimitris A nagnostakis, M.D. Neonatal Unit Catherine Dacou- Voutetakis, M.D. Endocrine Unit First Department of Pediatrics of A thens University Athens 608 Greece REFERENCES l.
2. 3.
Smith BT, Giroud CJP, Robert M, and Avery ME: Insulin antagonism of cortisol action on lecithin synthesis by cultured fetal lung cells, J PEDIATR 87:953, 1975. Avery ME, and Frantz I III, Commentary: Intrauterine developmental retardation, J PEDIATR 87:956, 1975. Cluck L, and Kulovich M: Fetallung development. Current concepts, Pediatr Clin North Am 20:367, 1973.
Thrombocytosis associated with the mucocutaneous lymph node syndrome To the Editor: We wish to add an additional case of mucocutaneous lymph node syndrome (MLNS) seen in the continental United States to the three cases recently reported by Brown and associates.' Our patient also demonstrated a thrombocytosis during the convalescent stage o f his illness which has not previously been reported with this syndrome, CASE REPORT
An 8-year-old black boy was admitted with a history and, physical findings as described by Kawasaki and associatesY Laboratory data were compatible with a diagnosis of mucocuta-
The Journal of Pediatrics October 1976
neous lymph node syndrome;-' specific tests for infectious diseases or collagen disorders were negative. Exfoliation of the exanthem began on the twelfth hospital day at which time the temperature returned to normal. The child was discharged on the sixteenth hospital day without symptoms or abnormal physical findings. A progressive increase in platelet count, beginning on the fifth hospital day, reached a peak of 910,000/mm ~ on the fourteenth and decreased to 724,000/mm:' on the sixteenth day. One week after discharge, the platelet count was 380,000/mm ~. DISCUSSION Thrombocytosis, to our knowledge, has not previously been associated with MLNS, and this finding is of interest in that MLNS has an estimated mortality rate of 1 to 2%~ due to thrombosis of the coronary artery? Vasculitis of the coronary arteries has been documented in MLNS ~ but an associated thrombocytosis might predispose or contribute to thrombus formation. We wonder if Dr. Brown and his co-workers or others have found a significant elevation in platelet count in their patients with MLNS. We agree with Dr. Brown that physicians should be made aware that this syndrome can occur in the United States and, hopefully, with increasing reports, more can be learned about its true incidence, etiology, and treatment. Miroslav Kovacevic, M.D. H. Garry Gardner, M.D. Department of Pediatrics Loyola University Stritch School of Medicine Maywood, Ill. 60153 REFERENCES
1.
2.
3.
Brown JS, Billmeier GJ, Cox F, Ibrahim M, Stepp WP, and Gibson R: Mucocutaneous lymph node syndrome in the continental United States, J PEDIATR 88:81, 1976. Kawasaki T, Kosaki F, Okawa S, Shigematsu I, and Yanagawa H: A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan, Pediatrics 54:271, 1974. Yanagisawa M, Kobayashi N, and Matsuya S: Myocardial infarction due to coronary thromboa/teritis, following acute febrile mucocutaneous lymph node syndrome (MLNS) in an infant, Pediatrics 54:277, 1974.
Dr. Brown was given an opportunity to answer the question posed in this letter; he has not replied. Editor
On genetic variants in fucosidosis To the Editor: We read with interest the article "Fucosidosis detection of carrier state in peripheral blood leukocytes" from Beratis and associates.' Our findings on a-L-fucosidase activity in leukocytes