Turkish J Pediatr
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Case Reports
Anesthetic management of a patient with hereditary fructose intolerance and phenylketonuria.
This is a report of a five-year-old girl with phenylketonuria (PKU) and hereditary fructose intolerance (HFI) who underwent elective strabismus surgery. PKU and HFI are two inborn errors of metabolism which have an autosomal recessive mode of inheritance. This case report describes the anesthetic features of a patient with PKU and HFI, each defect requiring specific anesthetic management.
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Twenty-five infants with hypernatremic dehydration due to acute gastroenteritis were given oral rehydration therapy (ORT). The patients received a glucose-electrolyte solution (such as that recommended by the World Health Organization) over six hours (2:1 rotating method). Twenty-three patients were successfully rehydrated within 48 hours after onset of therapy, while the two remaining patients attained normal serum Na+ levels within 72 hours. Acidosis was noted in 10 patients which disappeared in 24 hours.
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A four-month-old boy with beta-ketothiolase deficiency is described in this report. Presenting symptoms and signs were vomiting, irritability and acidotic respiration. ⋯ Subsequent urinary GC-MS analysis of the patient's urine sample showed the typical pattern of beta-ketothiolase deficiency. Our experience with this case indicates that accurate diagnosis and early treatment of inborn errors might be lifesaving.
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The human being is a homeotherm. Homeothermy is a result of thermoregulation which includes many physiological processes. Thermoregulation maintains an equilibrium between heat production (thermogenesis) and heat loss (thermolysis). ⋯ Even in the very small premature baby, we find that metabolic and vasomotor control responses are developed. To protect the newborn from stress resulting from hypo or hyperthermia, one should take into consideration the concept of the neutral temperature range which is also called the "Thermoneutral Zone" in (TNZ) or "Thermal Neutrality". Curves, proposed in 1971 by Hey are essential for keeping newborns in the TNZ.