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Charles A. Stanley - One of the best experts on this subject based on the ideXlab platform.
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Neonatal Hypoglycemia Studies — Is There a Sweet Story of Success Yet?
The New England journal of medicine, 2015Co-Authors: Rebecca A. Simmons, Charles A. StanleyAbstract:For over half a century, Neonatal Hypoglycemia has been a contentious topic.1 Although there is agreement that recurrent severe Hypoglycemia causes brain injury, there have been few high-quality studies providing data that inform the management or report the neurodevelopmental outcomes of transient Neonatal Hypoglycemia. Much of the controversy about Neonatal Hypoglycemia has focused on the question of number — that is, What glucose level should be used to “define” Hypoglycemia in neonates? Because the validity of statistical definitions of Neonatal Hypoglycemia has been appropriately criticized, an alternative approach has been to evaluate long-term outcomes, such as those in the recent . . .
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Neonatal Hypoglycemia studies is there a sweet story of success yet
The New England Journal of Medicine, 2015Co-Authors: Rebecca A. Simmons, Charles A. StanleyAbstract:For over half a century, Neonatal Hypoglycemia has been a contentious topic.1 Although there is agreement that recurrent severe Hypoglycemia causes brain injury, there have been few high-quality studies providing data that inform the management or report the neurodevelopmental outcomes of transient Neonatal Hypoglycemia. Much of the controversy about Neonatal Hypoglycemia has focused on the question of number — that is, What glucose level should be used to “define” Hypoglycemia in neonates? Because the validity of statistical definitions of Neonatal Hypoglycemia has been appropriately criticized, an alternative approach has been to evaluate long-term outcomes, such as those in the recent . . .
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Re-evaluating “Transitional Neonatal Hypoglycemia”: Mechanism and implications for management
The Journal of pediatrics, 2015Co-Authors: Charles A. Stanley, Lynne L Levitsky, Khalid Hussain, Paul J. Rozance, Paul S. Thornton, Diva D. De León, Deborah L. Harris, Morey W. Haymond, Mohammad Hassan Murad, Rebecca A. SimmonsAbstract:A Committee of the Pediatric Endocrine Society was recently asked by xxx to develop guidelines for evaluation and management of Hypoglycemia in neonates, infants, and children. To aid in formulating recommendations for neonates, in this review, we analyzed available data on the brief period of Hypoglycemia which commonly is observed in normal newborns during the transition from fetal to extrauterine life, hereafter referred to as transitional Neonatal Hypoglycemia in normal newborns. The goal was to better understand the mechanism underlying this phenomenon in order to formulate recommendations for recognizing neonates requiring diagnosis and treatment during the first days of life for disorders causing severe and persistent Hypoglycemia. It has long been known that plasma glucose concentrations are lower in the first 1–3 days of life in normal newborn infants than at later ages. Not until the 1960s was it appreciated that Hypoglycemia in neonates could sometimes be symptomatic and, as in older infants and children, cause seizures or permanent brain damage (1, 2). Although studies in laboratory animals have demonstrated postnatal developmental changes in specific enzymes involved in hepatic gluconeogenesis and ketogenesis (3, 4), it is unclear that such changes adequately explain transitional Neonatal Hypoglycemia in human newborns or if other mechanisms may be involved (5, 6). A National Institutes of Health conference outlined many of the “gaps in knowledge” about Neonatal Hypoglycemia and lamented the lack of a rational basis for defining Hypoglycemia in neonates (7). For this re-evaluation of transitional Neonatal Hypoglycemia in normal newborns, we used the strategy routinely employed by pediatric endocrinologists for evaluation of Hypoglycemia in older infants and children. This strategy, based on an examination of the major metabolic fuel and hormone responses to Hypoglycemia, makes it possible to discover the mechanism of Hypoglycemia and to make a specific diagnosis of the underlying cause (Figure; available at www.jpeds.com) (8). We reviewed published data in normal newborns on metabolic fuel and hormone responses during the period of transitional Neonatal Hypoglycemia. We focused on mean responses as being most likely representative of normal newborns, recognizing the possibility of heterogeneity, particularly with regard to peri-partum stresses and feeding practices. We found that transitional Neonatal Hypoglycemia most closely resembles known genetic forms of congenital hyperinsulinism, which cause a lowering of the plasma glucose threshold for suppression of insulin secretion. This conclusion is based on strong evidence supported by two or more independent reports and provides a novel perspective on both the diagnosis and management of Hypoglycemia in the first several days after birth. Figure Hypoglycemia diagnosis based on plasma metabolic fuel responses. Measurement of major fuels (lactate as a gluconeogenic substrate, FFA from adipose tissue lipolysis, and beta-hydroxybutyrate as the major ketone from hepatic ketogenesis) at a time of Hypoglycemia ...
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The causes of Neonatal Hypoglycemia.
The New England journal of medicine, 1999Co-Authors: Charles A. Stanley, Lester BakerAbstract:The risk of Hypoglycemia in neonates is high. If their first feeding is delayed for as little as three to six hours after birth, 10 percent of normal neonates cannot maintain plasma glucose concentrations above 30 mg per deciliter (1.7 mmol per liter).1 The risk is even higher among premature infants and those who are small or large for gestational age. After 12 hours of life, the risk of Hypoglycemia in normal neonates falls, but it remains substantial throughout the newborn period, especially in infants with birth asphyxia or low birth weight. Although Neonatal Hypoglycemia can cause seizures and permanent . . .
Kaeko Ogura - One of the best experts on this subject based on the ideXlab platform.
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long term prognosis of symptomatic occipital lobe epilepsy secondary to Neonatal Hypoglycemia
Epilepsy Research, 2010Co-Authors: Hesham Montassir, Yoshihiro Maegaki, Kousaku Ohno, Kaeko OguraAbstract:Summary Purpose To report on long-term clinical course in patients with symptomatic occipital lobe epilepsy secondary to Neonatal Hypoglycemia. Methods Six patients with Neonatal Hypoglycemia and symptomatic occipital lobe epilepsy were studied in our hospital through reviewing their medical records retrospectively. Results The median onset age of epilepsy was 2 years 8 months and median follow-up period was 12 years and 4 months. Initial seizure types were generalized convulsions in 4 patients, hemiconvulsion in 1, and infantile spasms in 1. Ictal manifestations of main seizures were identical to occipital lobe seizures, such as eye deviation, eye blinking, ictal vomiting, and visual hallucination. Seizure frequency was maximum during infancy and early childhood and decreased thereafter with no seizure in 2 patients, a few seizures a year in 3, and once a month in 1. All patients had status epilepticus in the early course of epilepsy. EEGs showed parieto-occipital spikes in all patients. MRI revealed cortical atrophy and T2 prolongation parieto-occipitally in 4 patients, hippocampal atrophy in 1, and unremarkable in 1. Conclusion This study indicates that epilepsy secondary to Neonatal Hypoglycemia is intractable during infancy and early childhood with frequent status epilepticus but tends to decrease in older age.
Mary B. Connolly - One of the best experts on this subject based on the ideXlab platform.
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Profound Neonatal Hypoglycemia and Lactic Acidosis Caused by Pyridoxine-Dependent Epilepsy
Pediatrics, 2012Co-Authors: Saadet Mercimek-mahmutoglu, Paula J Waters, Eduard A. Struys, Sylvia Stockler-ipsiroglu, Cornelis Jakobs, Gabriella Horvath, Marion B. Coulter-mackie, Tanya N. Nelson, Michael A. Sargent, Mary B. ConnollyAbstract:Pyridoxine-dependent epilepsy (PDE) was first described in 1954. The ALDH7A1 gene mutations resulting in α-aminoadipic semialdehyde dehydrogenase deficiency as a cause of PDE was identified only in 2005. Neonatal epileptic encephalopathy is the presenting feature in >50% of patients with classic PDE. We report the case of a 13-month-old girl with profound Neonatal Hypoglycemia (0.6 mmol/L; reference range >2.4), lactic acidosis (11 mmol/L; reference range A (p.Val278Val), and a novel putative pathogenic missense mutation c.1192G>C (p.Gly398Arg) in the ALDH7A1 gene. She has been seizure-free since 1.5 months of age on treatment with pyridoxine alone. She has motor delay and central hypotonia but normal language and social development at the age of 13 months. This case is the first description of a patient with PDE due to mutations in the ALDH7A1 gene who presented with profound Neonatal Hypoglycemia and lactic acidosis masquerading as a Neonatal-onset gluconeogenesis defect. PDE should be included in the differential diagnosis of Hypoglycemia and lactic acidosis in addition to medically refractory Neonatal seizures.
Rebecca A. Simmons - One of the best experts on this subject based on the ideXlab platform.
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Neonatal Hypoglycemia Studies — Is There a Sweet Story of Success Yet?
The New England journal of medicine, 2015Co-Authors: Rebecca A. Simmons, Charles A. StanleyAbstract:For over half a century, Neonatal Hypoglycemia has been a contentious topic.1 Although there is agreement that recurrent severe Hypoglycemia causes brain injury, there have been few high-quality studies providing data that inform the management or report the neurodevelopmental outcomes of transient Neonatal Hypoglycemia. Much of the controversy about Neonatal Hypoglycemia has focused on the question of number — that is, What glucose level should be used to “define” Hypoglycemia in neonates? Because the validity of statistical definitions of Neonatal Hypoglycemia has been appropriately criticized, an alternative approach has been to evaluate long-term outcomes, such as those in the recent . . .
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Neonatal Hypoglycemia studies is there a sweet story of success yet
The New England Journal of Medicine, 2015Co-Authors: Rebecca A. Simmons, Charles A. StanleyAbstract:For over half a century, Neonatal Hypoglycemia has been a contentious topic.1 Although there is agreement that recurrent severe Hypoglycemia causes brain injury, there have been few high-quality studies providing data that inform the management or report the neurodevelopmental outcomes of transient Neonatal Hypoglycemia. Much of the controversy about Neonatal Hypoglycemia has focused on the question of number — that is, What glucose level should be used to “define” Hypoglycemia in neonates? Because the validity of statistical definitions of Neonatal Hypoglycemia has been appropriately criticized, an alternative approach has been to evaluate long-term outcomes, such as those in the recent . . .
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Re-evaluating “Transitional Neonatal Hypoglycemia”: Mechanism and implications for management
The Journal of pediatrics, 2015Co-Authors: Charles A. Stanley, Lynne L Levitsky, Khalid Hussain, Paul J. Rozance, Paul S. Thornton, Diva D. De León, Deborah L. Harris, Morey W. Haymond, Mohammad Hassan Murad, Rebecca A. SimmonsAbstract:A Committee of the Pediatric Endocrine Society was recently asked by xxx to develop guidelines for evaluation and management of Hypoglycemia in neonates, infants, and children. To aid in formulating recommendations for neonates, in this review, we analyzed available data on the brief period of Hypoglycemia which commonly is observed in normal newborns during the transition from fetal to extrauterine life, hereafter referred to as transitional Neonatal Hypoglycemia in normal newborns. The goal was to better understand the mechanism underlying this phenomenon in order to formulate recommendations for recognizing neonates requiring diagnosis and treatment during the first days of life for disorders causing severe and persistent Hypoglycemia. It has long been known that plasma glucose concentrations are lower in the first 1–3 days of life in normal newborn infants than at later ages. Not until the 1960s was it appreciated that Hypoglycemia in neonates could sometimes be symptomatic and, as in older infants and children, cause seizures or permanent brain damage (1, 2). Although studies in laboratory animals have demonstrated postnatal developmental changes in specific enzymes involved in hepatic gluconeogenesis and ketogenesis (3, 4), it is unclear that such changes adequately explain transitional Neonatal Hypoglycemia in human newborns or if other mechanisms may be involved (5, 6). A National Institutes of Health conference outlined many of the “gaps in knowledge” about Neonatal Hypoglycemia and lamented the lack of a rational basis for defining Hypoglycemia in neonates (7). For this re-evaluation of transitional Neonatal Hypoglycemia in normal newborns, we used the strategy routinely employed by pediatric endocrinologists for evaluation of Hypoglycemia in older infants and children. This strategy, based on an examination of the major metabolic fuel and hormone responses to Hypoglycemia, makes it possible to discover the mechanism of Hypoglycemia and to make a specific diagnosis of the underlying cause (Figure; available at www.jpeds.com) (8). We reviewed published data in normal newborns on metabolic fuel and hormone responses during the period of transitional Neonatal Hypoglycemia. We focused on mean responses as being most likely representative of normal newborns, recognizing the possibility of heterogeneity, particularly with regard to peri-partum stresses and feeding practices. We found that transitional Neonatal Hypoglycemia most closely resembles known genetic forms of congenital hyperinsulinism, which cause a lowering of the plasma glucose threshold for suppression of insulin secretion. This conclusion is based on strong evidence supported by two or more independent reports and provides a novel perspective on both the diagnosis and management of Hypoglycemia in the first several days after birth. Figure Hypoglycemia diagnosis based on plasma metabolic fuel responses. Measurement of major fuels (lactate as a gluconeogenic substrate, FFA from adipose tissue lipolysis, and beta-hydroxybutyrate as the major ketone from hepatic ketogenesis) at a time of Hypoglycemia ...
Hesham Montassir - One of the best experts on this subject based on the ideXlab platform.
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long term prognosis of symptomatic occipital lobe epilepsy secondary to Neonatal Hypoglycemia
Epilepsy Research, 2010Co-Authors: Hesham Montassir, Yoshihiro Maegaki, Kousaku Ohno, Kaeko OguraAbstract:Summary Purpose To report on long-term clinical course in patients with symptomatic occipital lobe epilepsy secondary to Neonatal Hypoglycemia. Methods Six patients with Neonatal Hypoglycemia and symptomatic occipital lobe epilepsy were studied in our hospital through reviewing their medical records retrospectively. Results The median onset age of epilepsy was 2 years 8 months and median follow-up period was 12 years and 4 months. Initial seizure types were generalized convulsions in 4 patients, hemiconvulsion in 1, and infantile spasms in 1. Ictal manifestations of main seizures were identical to occipital lobe seizures, such as eye deviation, eye blinking, ictal vomiting, and visual hallucination. Seizure frequency was maximum during infancy and early childhood and decreased thereafter with no seizure in 2 patients, a few seizures a year in 3, and once a month in 1. All patients had status epilepticus in the early course of epilepsy. EEGs showed parieto-occipital spikes in all patients. MRI revealed cortical atrophy and T2 prolongation parieto-occipitally in 4 patients, hippocampal atrophy in 1, and unremarkable in 1. Conclusion This study indicates that epilepsy secondary to Neonatal Hypoglycemia is intractable during infancy and early childhood with frequent status epilepticus but tends to decrease in older age.
