THE JOURNAL OF PEDIATRICS
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43. Hack M, Taylor HG, Drotar D, Schluchter M, Cartar L, WilsonCostello D, et al. Poor predictive validity of the Bayley Scales of Infant Development for cognitive function of extremely low birth weight children at school age. Pediatrics 2005;116:333-41. 44. Schmidt B, Anderson PJ, Doyle LW, Dewey D, Grunau RE, Asztalos EV, et al. Survival without disability to age 5 years after neonatal caffeine therapy for apnea of prematurity. JAMA 2012; 307:275-82.
Vol. 164, No. 6 45. Msall ME. Neurodevelopmental surveillance in the first 2 years after extremely preterm birth: evidence, challenges, and guidelines. Early Hum Dev 2006;82:157-66. 46. Koller H, Lawson K, Rose SA, Wallace I, McCarton C. Patterns of cognitive development in very low birth weight children during the first six years of life. Pediatrics 1997;99:383-9. 47. Aylward GP. Neurodevelopmental outcomes of infants born prematurely. J Dev Behav Pediatr 2005;26:427-40.
50 Years Ago in THE JOURNAL OF PEDIATRICS Ketotic Hypoglycemia Colle E, Ulstrom RA. J Pediatr 1964;64:632-51
C
olle and Ulstrom described 8 children with episodic hypoglycemia associated with ketonemia and ketonuria, most of whom presented with early morning seizures. The authors proposed that the cause of ketotic hypoglycemia was impaired adaptation to fat metabolism during diminished carbohydrate supplies. They also proposed a management plan for these children, emphasizing early identification of ketosis and carbohydrate intake during illness, recommendations that are still in use today. This was a remarkable study at a time where the etiologies of pediatric hypoglycemia were in the early stages of being unraveled. In characterizing this group of patients with ketotic hypoglycemia, Drs Colle and Ulstrom provided a challenge for future investigators to identify the etiology. In the 50 years since this article was published, there has been little progress in our understanding of ketotic hypoglycemia. Whether it represents a specific disorder of fasting adaptation or is simply the lower end of the normal distribution of fasting tolerance in young children is unresolved. Ketotic hypoglycemia remains a diagnosis of exclusion and may represent a heterogeneous group of disorders. Hepatic glycogenoses might play a role in some of these patients, where ketotic hypoglycemia is the presenting clinical sign. Because hepatomegaly is not seen in glycogen synthase deficiency, this condition has been proposed as a common cause of ketotic hypoglycemia.1 Iatrogenic adrenal insufficiency from topical or inhaled steroids is more common in children with ketotic hypoglycemia than generally appreciated. Thorough evaluation of children with ketotic hypoglycemia, with active exclusion of alternative diagnoses, remains the cornerstone of management. Although our understanding of this heterogeneous group of conditions has improved since this article was published, a large proportion of cases continue to carry the unsatisfactory diagnosis of “idiopathic ketotic hypoglycemia.” Colin P. Hawkes, MD Charles A. Stanley, MD Division of Endocrinology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania
Reference
http://dx.doi.org/10.1016/j.jpeds.2013.11.028
1. Weinstein DA, Correia CE, Saunders AC, Wolfsdorf JI. Hepatic glycogen synthase deficiency: an infrequently recognized cause of ketotic hypoglycemia. Mol Genet Metab 2006;87:284-8.
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DeMauro et al
www.jpeds.com
43. Hack M, Taylor HG, Drotar D, Schluchter M, Cartar L, WilsonCostello D, et al. Poor predictive validity of the Bayley Scales of Infant Development for cognitive function of extremely low birth weight children at school age. Pediatrics 2005;116:333-41. 44. Schmidt B, Anderson PJ, Doyle LW, Dewey D, Grunau RE, Asztalos EV, et al. Survival without disability to age 5 years after neonatal caffeine therapy for apnea of prematurity. JAMA 2012; 307:275-82.
Vol. 164, No. 6 45. Msall ME. Neurodevelopmental surveillance in the first 2 years after extremely preterm birth: evidence, challenges, and guidelines. Early Hum Dev 2006;82:157-66. 46. Koller H, Lawson K, Rose SA, Wallace I, McCarton C. Patterns of cognitive development in very low birth weight children during the first six years of life. Pediatrics 1997;99:383-9. 47. Aylward GP. Neurodevelopmental outcomes of infants born prematurely. J Dev Behav Pediatr 2005;26:427-40.
50 Years Ago in THE JOURNAL OF PEDIATRICS Ketotic Hypoglycemia Colle E, Ulstrom RA. J Pediatr 1964;64:632-51
C
olle and Ulstrom described 8 children with episodic hypoglycemia associated with ketonemia and ketonuria, most of whom presented with early morning seizures. The authors proposed that the cause of ketotic hypoglycemia was impaired adaptation to fat metabolism during diminished carbohydrate supplies. They also proposed a management plan for these children, emphasizing early identification of ketosis and carbohydrate intake during illness, recommendations that are still in use today. This was a remarkable study at a time where the etiologies of pediatric hypoglycemia were in the early stages of being unraveled. In characterizing this group of patients with ketotic hypoglycemia, Drs Colle and Ulstrom provided a challenge for future investigators to identify the etiology. In the 50 years since this article was published, there has been little progress in our understanding of ketotic hypoglycemia. Whether it represents a specific disorder of fasting adaptation or is simply the lower end of the normal distribution of fasting tolerance in young children is unresolved. Ketotic hypoglycemia remains a diagnosis of exclusion and may represent a heterogeneous group of disorders. Hepatic glycogenoses might play a role in some of these patients, where ketotic hypoglycemia is the presenting clinical sign. Because hepatomegaly is not seen in glycogen synthase deficiency, this condition has been proposed as a common cause of ketotic hypoglycemia.1 Iatrogenic adrenal insufficiency from topical or inhaled steroids is more common in children with ketotic hypoglycemia than generally appreciated. Thorough evaluation of children with ketotic hypoglycemia, with active exclusion of alternative diagnoses, remains the cornerstone of management. Although our understanding of this heterogeneous group of conditions has improved since this article was published, a large proportion of cases continue to carry the unsatisfactory diagnosis of “idiopathic ketotic hypoglycemia.” Colin P. Hawkes, MD Charles A. Stanley, MD Division of Endocrinology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania
Reference
http://dx.doi.org/10.1016/j.jpeds.2013.11.028
1. Weinstein DA, Correia CE, Saunders AC, Wolfsdorf JI. Hepatic glycogen synthase deficiency: an infrequently recognized cause of ketotic hypoglycemia. Mol Genet Metab 2006;87:284-8.
1310
DeMauro et al
