The Journal of pediatrics
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The Journal of pediatrics · Sep 1990
Comparative StudyGlucose therapy for glycogenosis type 1 in infants: comparison of intermittent uncooked cornstarch and continuous overnight glucose feedings.
This study was undertaken to test the glycemic response of five infants with glycogen storage disease type 1, aged 0.7 to 1.5 years, to uncooked cornstarch under various dietary conditions, and to evaluate the long-term effects of a dietary regimen consisting of uncooked cornstarch in milk every 4 hours, in addition to three meals daily, on biochemical values and physical growth. The results were compared with previous experience in treating six infants with continuous overnight glucose infusion via gastrostomy plus multiple daily feedings containing an adequate source of glucose. A test dose of cornstarch (1.6 to 1.8 gm/kg) providing four times the calculated hourly glucose production rate, when given in water 15 to 30 minutes after a continuous overnight intragastric glucose infusion was stopped, did not maintain normoglycemia. ⋯ Overnight blood glucose levels were comparable to those achieved with continuous intragastric glucose infusion. With this regimen the five infants have maintained linear growth rates normal for their age and genetic potential; the mean percentage of ideal body weight for length percentile did not change significantly, although two of the five patients were overweight (123% and 124% of ideal body weight respectively) after 3 years of treatment. We conclude that a trial of uncooked cornstarch in feedings of milk every 4 hours should be attempted as soon as a more frequent feeding schedule with dextrose-containing formulas proves ineffective, because the former has the potential to provide the continuous glucose required by infants with glycogen storage disease type 1 in a safer and less invasive fashion than continuous intragastric glucose infusion.
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The Journal of pediatrics · Aug 1990
Effect of hypothermia and cardiac arrest on outcome of near-drowning accidents in children.
We conducted a retrospective review of 55 near-drowning victims (mean age 4.75 years) admitted to the intensive care unit during a 5-year period, to determine the factors that may influence survival both before and after hospital admission. All patients who remained comatose after resuscitation received ventilation for an initial 24 hour period, after which an assessment of central nervous system injury was made. Intracranial pressure was not monitored, and barbiturate therapy was used only for seizure control. ⋯ All patients with a detectable pulse, regardless of temperature, survived without neurologic sequelae. The 58% intact survival rate in this series compares favorably with the 50% we reported previously when high-dose barbiturate therapy and hypothermia were used to control intracranial pressure; at the same time, the number of survivors with a persistent vegetative state has been reduced by 50%. We conclude that prolonged in-hospital resuscitation and aggressive treatment of near-drowning victims who initially have absence of vital signs and are not hypothermic either results in eventual death or increases the number of survivors with a persistent vegetative state.
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To determine the frequency of eye and auditory complications and their relationship to drug dosage and iron stores in patients receiving deferoxamine, we studied 52 regularly transfused patients who received deferoxamine by subcutaneous or intravenous infusion in doses from 26 to 136 mg/kg/day, and whose serum ferritin levels of 185 to 17,775 micrograms/L reflected a wide range of iron stores. Forty-nine patients (94%) had no evidence of drug-induced visual or auditory abnormalities. Symptomatic loss of vision and hearing developed in one patient; both problems improved when chelation therapy was stopped. ⋯ Eye and ear abnormalities in the symptom-free patients did not progress despite continuation or resumption of chelation therapy at the same dosage. Patients with ophthalmologic and audiologic abnormalities did not receive higher doses of deferoxamine and did not have lower serum ferritin levels than patients without such abnormalities. These findings demonstrate that eye and ear abnormalities during chelation therapy with deferoxamine may not occur uniformly at as high a frequency as previously reported, even in patients who receive large doses of the chelating agent or who have only modest amounts of excessive iron.