- Email: [email protected]
More on factors related to intellectual development of children with Down syndrome
Volume 90 Number 6
DISCUSSION Two points came to light in the above observations: (1) Continuous low-dose infusion of insulin is effective in rapidly lowering serum glucose level (up to 100 mg/dl/hour using O.1 U / kg/hour). Frequent monitoring of glucose levels is mandatory to prevent overcorrection of hyperglycemia. (2) The correction of ketoacidosis lags far behind the improvement of hyperglycemia in this isolated case. Normoglycemia has been attained 20 hours ahead of the regaining of normal pH. This is an indication for the use of.alkali solution in cases of severe ketoacidosis. With the anticipation of a widespread use of continuous lowdose infusion of insulin for the treatment of diabetic ketoacidosis, our concern shared by others ~ should serve as a note of caution for those contemplating the use of this method. Physicians should familiarize themselves with the possible problems of this treatment regimen and also be prepared for the possibility of a failure in response to this approach as suggested in the recent editorial in New England Journal of Medicine.3 Wah Jun Tze, M.D., FR.C.P.(C). Kee Sheen Leong-Sit, M.D. Children's Hospital 250 West 59th Ave. Vancouver, BC, Canada V5X 1X2 REFERENCES
Letters to the Editor
hour instead of being discontinued, and 5% glucose administration started at a rate of 5 ml/kg/hour to maintain normal glucose concentration as outlined in our article, the patient would have been well by 10 instead of 27 hours. Alternatively, fractional coverage and glucose administration should have begun when insulin infusion was discontinued, not five hours later. That the decrease in serum acetoacetate and beta hydroxybutyrate concentrations may lag behind the fall in blood glucose for a significant period of time has long been known and is unrelated to the mode of insulin administration. As noted in the studies of Sulway and Malins, ~ elevation of ketone bodies may persist for more than 24 hours after institution of adequate therapy. Malcolm M. Martin, M.D., F.R.CP., F.A.C.P., F.A.A.P. : Arline L. A. Martin, M.A., M.D. Department of Pediatrics Georgetown University School of Medicine Georgetown University Medical Center Washington, D.C. 20007 REFERENCES Martin MM, and Martin ALA: Continuous low-dose infusion of insulin, J P~DtA'rR 89:560, 1976. 2. Page et al.: Treatment of diabetic ketoacidosis, N Engl J Med 294:1183, 1976. 3. Sulway M J, and Malins JM: Acetone in diabetic ketoacidosis, Lancet 2:736, 1970.
:l.
1. Martin MM, and Martin ALA: Continuous low-dose infusion of insulin, J PEDIATR 89:560, 1976. 2. Schultz TA, Verkleeren JL, and Blevins RD: Treatment of diabetic ketoacidosis, N Engl J Med 294:1183, 1976. 3. Madison LL: Low-dose insulin: A plea for caution, N Engl J Med 294:393, 1976.
Reply To the Editor: We appreciate the letter by Drs. Tze and Leong-Sit and their support of continuous low-dose insulin infusion as an effective tool in the management of diabetic ketoacidosis in children. 1 We agree that this method of treatment is still relatively new. It must be emphasized, however, that it has now been used in over lO0 patients by competent investigators with excellent results and without complications, z The difficulty Drs. Tze and Leong-Sit experienced with their patient appears to have been the result of discontinuing the intravenous insulin infusion, rather than because of any inherent dangers or problems with it. As their table shows, the response to the continuous infusion of insulin was most gratifying. Serum glucose concentration dropped from 556 to 145 mg/dl and pH increased from 7.08 to 7.22. At five hours, however, insulin infusion was discontinued, resulting in an increase in serum glucose concentration to 390 mg/dl, a decrease in pH to 7.20 and in bicarbonate to 5.0 mEq/l over the next five hours. Had the insulin infusion been adjusted to between 0.02 and 0.06 U / k g /
10 4 5
More on factors related to intellectual development of children with Down syndrome To the Editor'." Stephan and associates' reviewed macrocephaly with cutaneous anglomatoses in which they reported 3 cases of KlippelTrenaunay-Weber syndrome with macrocephaly. The possible cause of macrocephaly in these cases was not determined. We wish to report the brain autopsy results on a recent patient of ours.
CASE REPORT Patient B. N. was a term male infant born to a gravida 3, para 1, abortus 1 woman. He weighed 5,700 gm ( > 97th percentile). He had persistent cyanosis, poor respiratory effort that required ventilatory assistance, and died approximately 12 to 13 hours post delivery. Physical findings were compatible with KlippelTrenaunay-Weber syndrome. At postmortem examination the brain weighed 720 gm. The macrencephaly was asymmetric. There was hypertrophy of the right cerebral hemisphere and right cerebellar hemisphere. The cerebral enlargement appeared to be due to a proportionate increase in neural and glial elements and not to any vascular malformation. The enlargement of the cerebellum could be partially attributed to heterotopic gray matter. The ventricular system was not dilated. The cerebral
10 4 6
Letters to the Editor
aqueduct was patent. Therefore, from this case it seems as if the macrocephaly was secondary to a true macrencephaly.
Cynthia Trevenen, M.D. Department of Pediatric Pathology John T. Martso(f, M.D. Division of Genetics ChiMren's Centre Health Sciences Centre 685 Bannatyne A re. Winnipeg, Manitoba, Canada R3E 0 W1 REFERENCE
1. Stephan M J, Hall BD, Smith DW, and Cohen MM Jr: Macrocephaly in association with unusual cutaneous angiomatosis, J PEDIATR 87:353, 1975.
The Journal of Pediatrics June 1977
Reply To the Editor: The findings of true macrencephaly as the cause for the large head in this instance of Klippel-Trenaunay-Weber syndrome is of great practical relevance and tends to support our clinical recommendation of nonintervention by pneumoencephalography and/or angiography in this disorder unless there are indications beyond macrocephaly per se.
David W. Smith, M.D. Dysmorphology Unit Department of Pediatrics University of Washington School of Medicine Seattle, WA 98195
DISCUSSION Two points came to light in the above observations: (1) Continuous low-dose infusion of insulin is effective in rapidly lowering serum glucose level (up to 100 mg/dl/hour using O.1 U / kg/hour). Frequent monitoring of glucose levels is mandatory to prevent overcorrection of hyperglycemia. (2) The correction of ketoacidosis lags far behind the improvement of hyperglycemia in this isolated case. Normoglycemia has been attained 20 hours ahead of the regaining of normal pH. This is an indication for the use of.alkali solution in cases of severe ketoacidosis. With the anticipation of a widespread use of continuous lowdose infusion of insulin for the treatment of diabetic ketoacidosis, our concern shared by others ~ should serve as a note of caution for those contemplating the use of this method. Physicians should familiarize themselves with the possible problems of this treatment regimen and also be prepared for the possibility of a failure in response to this approach as suggested in the recent editorial in New England Journal of Medicine.3 Wah Jun Tze, M.D., FR.C.P.(C). Kee Sheen Leong-Sit, M.D. Children's Hospital 250 West 59th Ave. Vancouver, BC, Canada V5X 1X2 REFERENCES
Letters to the Editor
hour instead of being discontinued, and 5% glucose administration started at a rate of 5 ml/kg/hour to maintain normal glucose concentration as outlined in our article, the patient would have been well by 10 instead of 27 hours. Alternatively, fractional coverage and glucose administration should have begun when insulin infusion was discontinued, not five hours later. That the decrease in serum acetoacetate and beta hydroxybutyrate concentrations may lag behind the fall in blood glucose for a significant period of time has long been known and is unrelated to the mode of insulin administration. As noted in the studies of Sulway and Malins, ~ elevation of ketone bodies may persist for more than 24 hours after institution of adequate therapy. Malcolm M. Martin, M.D., F.R.CP., F.A.C.P., F.A.A.P. : Arline L. A. Martin, M.A., M.D. Department of Pediatrics Georgetown University School of Medicine Georgetown University Medical Center Washington, D.C. 20007 REFERENCES Martin MM, and Martin ALA: Continuous low-dose infusion of insulin, J P~DtA'rR 89:560, 1976. 2. Page et al.: Treatment of diabetic ketoacidosis, N Engl J Med 294:1183, 1976. 3. Sulway M J, and Malins JM: Acetone in diabetic ketoacidosis, Lancet 2:736, 1970.
:l.
1. Martin MM, and Martin ALA: Continuous low-dose infusion of insulin, J PEDIATR 89:560, 1976. 2. Schultz TA, Verkleeren JL, and Blevins RD: Treatment of diabetic ketoacidosis, N Engl J Med 294:1183, 1976. 3. Madison LL: Low-dose insulin: A plea for caution, N Engl J Med 294:393, 1976.
Reply To the Editor: We appreciate the letter by Drs. Tze and Leong-Sit and their support of continuous low-dose insulin infusion as an effective tool in the management of diabetic ketoacidosis in children. 1 We agree that this method of treatment is still relatively new. It must be emphasized, however, that it has now been used in over lO0 patients by competent investigators with excellent results and without complications, z The difficulty Drs. Tze and Leong-Sit experienced with their patient appears to have been the result of discontinuing the intravenous insulin infusion, rather than because of any inherent dangers or problems with it. As their table shows, the response to the continuous infusion of insulin was most gratifying. Serum glucose concentration dropped from 556 to 145 mg/dl and pH increased from 7.08 to 7.22. At five hours, however, insulin infusion was discontinued, resulting in an increase in serum glucose concentration to 390 mg/dl, a decrease in pH to 7.20 and in bicarbonate to 5.0 mEq/l over the next five hours. Had the insulin infusion been adjusted to between 0.02 and 0.06 U / k g /
10 4 5
More on factors related to intellectual development of children with Down syndrome To the Editor'." Stephan and associates' reviewed macrocephaly with cutaneous anglomatoses in which they reported 3 cases of KlippelTrenaunay-Weber syndrome with macrocephaly. The possible cause of macrocephaly in these cases was not determined. We wish to report the brain autopsy results on a recent patient of ours.
CASE REPORT Patient B. N. was a term male infant born to a gravida 3, para 1, abortus 1 woman. He weighed 5,700 gm ( > 97th percentile). He had persistent cyanosis, poor respiratory effort that required ventilatory assistance, and died approximately 12 to 13 hours post delivery. Physical findings were compatible with KlippelTrenaunay-Weber syndrome. At postmortem examination the brain weighed 720 gm. The macrencephaly was asymmetric. There was hypertrophy of the right cerebral hemisphere and right cerebellar hemisphere. The cerebral enlargement appeared to be due to a proportionate increase in neural and glial elements and not to any vascular malformation. The enlargement of the cerebellum could be partially attributed to heterotopic gray matter. The ventricular system was not dilated. The cerebral
10 4 6
Letters to the Editor
aqueduct was patent. Therefore, from this case it seems as if the macrocephaly was secondary to a true macrencephaly.
Cynthia Trevenen, M.D. Department of Pediatric Pathology John T. Martso(f, M.D. Division of Genetics ChiMren's Centre Health Sciences Centre 685 Bannatyne A re. Winnipeg, Manitoba, Canada R3E 0 W1 REFERENCE
1. Stephan M J, Hall BD, Smith DW, and Cohen MM Jr: Macrocephaly in association with unusual cutaneous angiomatosis, J PEDIATR 87:353, 1975.
The Journal of Pediatrics June 1977
Reply To the Editor: The findings of true macrencephaly as the cause for the large head in this instance of Klippel-Trenaunay-Weber syndrome is of great practical relevance and tends to support our clinical recommendation of nonintervention by pneumoencephalography and/or angiography in this disorder unless there are indications beyond macrocephaly per se.
David W. Smith, M.D. Dysmorphology Unit Department of Pediatrics University of Washington School of Medicine Seattle, WA 98195