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Neonatal neurology

Pierpaolo Cerullo, Paola Brancaccio, Serenella Anzilotti, Antonio Vinciguerra, Ornella Cuomo, Ferdinando Fiorino, Beatrice Severino, Paola Di Vaio, Gianfranco Di Renzo, Lucio Annunziato, Giuseppe Pignataro
Hypoxic-ischemic encephalopathy (HI) accounts for the majority of developmental, motor and cognitive deficits in children, leading to life-long neurological impairments. Since the plasmamembrane sodium/calcium exchanger (NCX) plays a fundamental role in maintaining ionic homeostasis during adult brain ischemia, in the present work we aimed to demonstrate (1)the involvement of NCX in the pathophysiology of neonatal HI and (2)a possible NCX-based pharmacological intervention. HI was induced in neonatal mice at postnatal day 7(P7) by unilateral cut of the right common carotid artery, followed by 60 min exposure to 8%O2 ...
March 15, 2018: Neuropharmacology
Yangyang R Yu, Jennifer L Carpenter, Annalyn S DeMello, Sundeep G Keswani, Darrell L Cass, Oluyinka O Olutoye, Adam M Vogel, James A Thomas, Cole Burgman, Caraciolo J Fernandes, Timothy C Lee
PURPOSE: This study assesses the impact of extracorporeal membrane oxygenation (ECMO) associated morbidities on long-term quality of life (QOL) outcomes. METHODS: A single center, retrospective review of neonatal and pediatric non-cardiac ECMO survivors from 1/2005-7/2016 was performed. The 2012 Pediatric Quality of Life Inventory™ (PedsQL™) survey was administered. Clinical outcomes and QOL scores between groups were compared. RESULTS: Of 74 patients eligible, 64% (35 NICU, 12 PICU) completed the survey...
February 10, 2018: Journal of Pediatric Surgery
Carolien Zwiers, Mirjam Ea Scheffer-Rath, Enrico Lopriore, Masja de Haas, Helen G Liley
BACKGROUND: Exchange transfusion and phototherapy have traditionally been used to treat jaundice and avoid the associated neurological complications. Because of the risks and burdens of exchange transfusion, intravenous immunoglobulin (IVIg) has been suggested as an alternative therapy for alloimmune hemolytic disease of the newborn (HDN) to reduce the need for exchange transfusion. OBJECTIVES: To assess the effect and complications of IVIg in newborn infants with alloimmune HDN on the need for and number of exchange transfusions...
March 18, 2018: Cochrane Database of Systematic Reviews
Flaminia Bardanzellu, Maria Cristina Pintus, Valentina Masile, Vassilios Fanos, Maria Antonietta Marcialis
Nephrogenic syndrome of inappropriate antidiuresis (NSIAD), first described in 2005, is a rare genetic X-linked disease, presenting with hyponatremia, hyposmolarity, euvolemia, inappropriately concentrated urine, increased natriuresis, and undetectable or very low arginine-vasopressine (AVP) circulating levels. It can occur in neonates, infants, or later in life. NSIAD must be early recognized and treated to prevent severe hyponatremia, which can show a dangerous impact on neonatal outcome. In fact, it potentially leads to death or, in case of survival, neurologic sequelae...
March 15, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Mona Noureldein, Roxana Mardare, Jack Pickard, Hoi Lun Shing, Michael Eisenhut
BACKGROUND: It has been estimated that paediatric meningitis without elevated CSF white cell count (pleocytosis) accounts for 0.5-12% of all cases of bacterial meningitis. CSF protein and glucose measurements are therefore essential in management but may be neglected in clinical practice. In order to improve recognition of bacterial meningitis in neonates and to enable adequate management and audit, we investigated whether a systemic inflammatory response in the absence of meningitis is associated with elevated CSF protein and reduced CSF glucose levels...
March 14, 2018: Fluids and Barriers of the CNS
Juan Zhang, Lorelei Donovan Tucker, DongYan, Yujiao Lu, Luodan Yang, Chongyun Wu, Yong Li, Quanguang Zhang
Hypoxic-ischemic (HI) encephalopathy is a leading cause of dire mortality and morbidity in neonates. Unfortunately, no effective therapies have been developed as of yet. Oxidative stress plays a critical role in pathogenesis and progression of neonatal HI. Previously, as a Nrf2 activator, tert-butylhydroquinone (TBHQ) has been demonstrated to exert neuroprotection on brain trauma and ischemic stroke models, as well as oxidative stress-induced cytotoxicity in neurons. It is, however, still unknown whether TBHQ administration can protect against oxidative stress in neonatal HI brain injury...
March 9, 2018: Neurochemistry International
K Wegleiter, M Waltner-Romen, R Trawoeger, U Kiechl-Kohlendorfer, E Griesmaier
Fetal angiotensin II receptor antagonist exposure is associated with major complications and even death when administered during pregnancy. Neonates frequently require intensive care treatment, and mortality is high. Despite this well-known risk potential, a considerable number of women still receive angiotensin II receptor antagonists during pregnancy to treat arterial hypertension. Although clinical symptoms in the neonatal period are well described, few reports address long-term follow-up after fetal exposure to angiotensin II receptor antagonists...
2018: Case Reports in Pediatrics
Marc Beltempo, Prakesh S Shah, Xiang Y Ye, Jehier Afifi, Shoo Lee, Douglas D McMillan
OBJECTIVE: To determine the specific Score of Neonatal Acute Physiology (SNAP-II) cut-off scores associated with outcomes in extremely preterm infants, and to examine its contribution to predictive models that include nonmodifiable birth predictors. STUDY DESIGN: Retrospective observational study of 9240 infants born at 22-28 weeks' gestation and admitted to the Canadian Neonatal Network from 2010 to 2015. Outcomes included early and hospital mortality, composite of mortality/morbidity and individual morbidities...
March 12, 2018: Journal of Maternal-fetal & Neonatal Medicine
Huseyin Demirbilek, Nihal Hatipoglu, Ulku Gul, Zeynep Uzan Tatli, Sian Ellard, Sarah E Flanagan, Elisa De Franco, Selim Kurtoglu
The basic helix-loop-helix(bHLH) transcription factor, NEUROD1(also known as BETA2) is involved in the development of neural elements and endocrine pancreas. Less than 10 reports of adult-onset non-insulin dependent diabetes mellitus(NIDDM) due to heterozygous NEUROD1 mutations and two cases with permanent neonatal diabetes mellitus(PNDM) and neurological abnormalities due to homozygous NEUROD1 mutations have been published.
March 9, 2018: Pediatric Diabetes
Nina Hediger, Markus A Landolt, Carmen Diez-Fernandez, Martina Huemer, Johannes Häberle
Neonatal onset hyperammonemia in patients with urea cycle disorders (UCDs) is still associated with high morbidity and mortality. Current protocols consistently recommend emergency medical and dietary management. In case of increasing or persistent hyperammonemia, with continuous or progressive neurological signs, dialysis is performed, mostly as ultima ratio. It is presently unknown whether the currently defined ammonia threshold (e.g., at 500 μmol/L) to start dialysis is useful to improve clinical outcome...
March 8, 2018: Journal of Inherited Metabolic Disease
Marta Ivars, Ana Martin-Santiago, Eulalia Baselga, Laurent Guibaud, Juan Carlos López-Gutiérrez
Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital vascular disease associated with important morbidity and non-insignificant risk of mortality in cases of severe gastrointestinal or neurological involvement. Early diagnosis in the neonatal period can be difficult as very often skin lesions appear progressively during childhood having no correlation with gastrointestinal lesion development. The appearance of one large venous malformation (VM) in the neonatal period has suggested a characteristic finding of this syndrome...
March 8, 2018: European Journal of Pediatrics
Mohamed R El-Tahan, Samah El Kenany, Ehsan M Abdelaty, Eiad A Ramzy
BACKGROUND: Preoperative remifentanil administration blunts haemodynamic responses to tracheal intubation in parturients with severe preeclampsia. We hypothesised that the preoperative administration of low doses of remifentanil or dexmedetomidine would lead to comparable maternal neurohormonal responses and neonatal outcomes in patients with severe preeclampsia. METHODS: Parturients diagnosed with severe preeclampsia undergoing caesarean delivery were randomly allocated to receive remifentanil (0...
March 7, 2018: Minerva Anestesiologica
Jean-François Ghersi-Egea, Elodie Saudrais, Nathalie Strazielle
Drug bioavailability to the developing brain is a major concern in the treatment of neonates and infants as well as pregnant and breast-feeding women. Central adverse drug reactions can have dramatic consequences for brain development, leading to major neurological impairment. Factors setting the cerebral bioavailability of drugs include protein-unbound drug concentration in plasma, local cerebral blood flow, permeability across blood-brain interfaces, binding to neural cells, volume of cerebral fluid compartments, and cerebrospinal fluid secretion rate...
March 7, 2018: Pharmaceutical Research
Daigo Ochiai, Hirotaka Masuda, Yushi Abe, Toshimitsu Otani, Marie Fukutake, Tadashi Matsumoto, Kei Miyakoshi, Mamoru Tanaka
Mesenchymal stem cells (MSCs) have generated great interest in the fields of regenerative medicine and immunotherapy because of their unique biological properties. Among MSCs, amniotic fluid stem cells (AFS) have a number of characteristics that make them attractive candidates for tissue engineering and cell replacement strategies, particularly for perinatal medicine. If various neonatal conditions, including birth asphyxia, preterm birth, and congenital abnormalities, which result in long-lasting severe impairments, could be predicted during pregnancy, it would allow collection of small samples of amniotic fluid cells by amniocentesis...
March 6, 2018: Keio Journal of Medicine
Liv Knutzen, Christina Aye, Hannah Anderson-Knight, Elena Svirko, Lawrence Impey
INTRODUCTION: The aim of this paper was to determine if arteriovenous differences of pH and PCO2 are useful predictors of adverse neonatal outcome in acidemic neonates. MATERIAL AND METHODS: An established database of 8759 term, singleton, non-anomalous neonates with validated cord gases and outcomes (Encephalopathy (Grade 2/ 3), Apgar <7 at 5 minutes and composite neonatal outcomes of neurological and systemic involvement) was used. Analysis was of the cohort of the 520 acidemic (arterial pH <7...
March 7, 2018: Acta Obstetricia et Gynecologica Scandinavica
K J Low, K Stals, R Caswell, M Wakeling, J Clayton-Smith, A Donaldson, N Foulds, A Norman, M Splitt, K Urankar, K Vijayakumar, A Majumdar, Ddd Study, S Ellard, S F Smithson
CHN is genetically heterogeneous and its genetic basis is difficult to determine on features alone. CNTNAP1 encodes CASPR, integral in the paranodal junction high molecular mass complex. Nineteen individuals with biallelic variants have been described in association with severe congenital hypomyelinating neuropathy, respiratory compromise, profound intellectual disability and death within the first year. We report 7 additional patients ascertained through exome sequencing. We identified 9 novel CNTNAP1 variants in 6 families: three missense variants, four nonsense variants, one frameshift variant and one splice site variant...
March 6, 2018: European Journal of Human Genetics: EJHG
Lilly L Chang, James L Wynn, Marisa J Pacella, Candace C Rossignol, Felix Banadera, Neil Alviedo, Alfonso Vargas, Jeffrey Bennett, Melissa Huene, Nicole Copenhaver, Livia Sura, Kimberly Barnette, Jayne Solomon, Nikolay A Bliznyuk, Josef Neu, Michael D Weiss
BACKGROUND: Withholding enteral feedings during hypothermia lacks supporting evidence. OBJECTIVES: We aimed to determine if minimal enteral nutrition (MEN) during hypothermia in patients with hypoxic-ischemic encephalopathy was associated with a reduced duration of parenteral nutrition, time to full oral feeds, and length of stay, but would not be associated with increased systemic inflammation or feeding complications. METHODS: We performed a pilot, retrospective, matched case-control study within the Florida Neonatal Neurologic Network from December 2012 to May 2016 of patients who received MEN during hypothermia (n = 17) versus those who were not fed (n = 17)...
March 6, 2018: Neonatology
Stiljan Hoxha, Riccardo Giuseppe Abbasciano, Camilla Sandrini, Lucia Rossetti, Tiziano Menon, Luca Barozzi, Daniele Linardi, Alessio Rungatscher, Giuseppe Faggian, Giovanni Battista Luciani
Aortic arch repair in newborns and infants has traditionally been accomplished using a period of deep hypothermic circulatory arrest. To reduce neurologic and cardiac dysfunction related to circulatory arrest and myocardial ischemia during complex aortic arch surgery, an alternative and novel strategy for cerebro-myocardial protection was recently developed, where regional low-flow perfusion is combined with controlled and independent coronary perfusion. The aim of the present retrospective study was to assess short-term and mid-term results of selective and independent cerebro-myocardial perfusion in neonatal aortic arch surgery...
March 6, 2018: Artificial Organs
Ingrid Kratzer, Nathalie Strazielle, Elodie Saudrais, Kati Mönkkönen, Céline Maleval, Sandrine Blondel, Jean-François Ghersi-Egea
Exposure of the developing brain to toxins, drugs, or deleterious endogenous compounds during the perinatal period can trigger alterations in cell division, migration, differentiation, and synaptogenesis, leading to life-long neurological impairment. The brain is protected by cellular barriers acting through multiple mechanisms, some of which are still poorly explored. We used a combination of enzymatic assays, live tissue fluorescence microscopy, and an in vitro cellular model of the blood-CSF barrier to investigate an enzymatic detoxification pathway in the developing male and female rat brain...
March 5, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Tao Xiong, Yi Qu, Huiqin Wang, Hongju Chen, Jianghu Zhu, Fengyan Zhao, Rong Zou, Li Zhang, Dezhi Mu
Glycogen synthase kinase 3 beta (GSK-3β) plays an important role in neurological outcomes after brain injury. However, its roles and mechanisms in hypoxia-ischemia (HI) are unclear. Activation of mTOR complex 1 (mTORC1) has been proven to induce the synthesis of proteins associated with regeneration. We hypothesized that GSK-3β inhibition could activate the mTORC1 signaling pathway, which may reduce axonal injury and induce synaptic protein synthesis and functional recovery of synapses after HI. By analyzing a P7 rat model of cerebral HI and an in vitro ischemic (oxygen glucose deprivation) model, we found that GSK-3β inhibitors (GSK-3β siRNA or lithium chloride) activated mTORC1 signaling, leading to increased expression of synaptic proteins, including synapsin 1, PSD95, and GluR1, and the microtubule-associated protein Tau and decreased expression of the axonal injury-associated protein amyloid precursor protein...
February 28, 2018: Journal of Neuropathology and Experimental Neurology
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