PAEDIATRIC PROTOCOLS for Malaysian Hospitals 3Rd Edition
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MRI Changes of Brain in Newborns with Hypoxic Ischemic Encephalopathy Clinical Stage Ii Or Stage Iii- a Descriptive Study
Original Research Article DOI: 10.18231/2455-6793.2017.0009 MRI changes of brain in newborns with hypoxic ischemic encephalopathy clinical stage ii or stage iii- a descriptive study Jose O1,*, Sheena V2 1Assistant Professor, 2Junior Resident, Dept. of Pediatrics, Govt. TD Medical College, Alappuzha *Corresponding Author: Email: [email protected] Abstract Objectives: The aim of the study was to estimate the proportion of MRI changes in newborns with HIE, to compare the findings of term and preterm babies and to identify if there is any clinical stage specific MRI findings Methods: After obtaining clearance from ethical committee, 30 newborns with either stage II or stage III HIE are included in the study. MRI brain was taken between one to two weeks of age once the vitals of the babies are stable & after ensuring euthermia. Results: Out of the 30 babies, 19 were male babies and 11 female babies. 16 of them were term and 14 of them preterm babies.27 of the total 30 patients had MRI changes of HIE, which accounts for 90%. 17of the 30 mothers were primi mothers which accounts for 56.7%. Most important antenatal factors associated with HIE are gestational hypertension and UTI. Gestational diabetes mellitus and placental/cord factors are also found to be important contributing factors. 33.4% had a history of UTI, 30% gestational hypertension, 23.4% gestational diabetes mellitus in the antenatal period. Conclusion: Basal ganglia and/or thalamus were affected in 50% of term babies. 87.5% of babies with periventricular leucomalacia are preterms. Intracranial hemorrhage was seen in 7.4% of the babies and all of them were preterms. -
Childhood Epilepsy: an Update on Diagnosis and Management
American Journal of Neuroscience Review Childhood Epilepsy: An Update on Diagnosis and Management Khaled Saad Department of Pediatrics, Faculty of Medicine, University of Assiut, Assiut 71516, Egypt Article history Abstract: Within the past few years there is a rapid expansion in our Received: 23-10-2014 understanding of epilepsy. The development of new anti-epileptic drugs and Revised: 25-11-2014 refinements to epilepsy surgery are widening the therapeutic options for Accepted: 12-01-2015 epilepsy. In addition, the classification of the epilepsies continues to evolve based on an increased understanding of the molecular genetics of the condition and this includes the recognition of possible novel epilepsy syndromes. This review considers some of these exciting developments, as well as addressing the essential features of the diagnosis, investigations, management and impact of epilepsy in childhood. Keywords: Children, Epilepsy, Seizure, Anti-Epileptic Drugs Introduction guidelines for optimal practices, rational therapy and counseling (Aylwad, 2008; Tamber and Mountz, 2012; Epilepsy is a common heterogeneous neurological Sharma, 2013). problem in children. It exerts a significant physical, psychological, economic and social toll on children and Definitions their caregivers. Fifty million people have epilepsies A seizure is defined as an excessive burst of abnormal globally; more than half of them are children. In the synchronized neuronal activity affecting small or large USA; between 25,000 and 40,000 children will have a neuronal networks that results in clinical manifestations first non-febrile seizure each year. The problem is that are sudden, transient and usually brief. Epilepsy is further compounded in developing countries as they add defined as a disorder of the brain characterized by any of about 75-80% of new cases of epilepsy (Guerrini, 2006; the following conditions: (1) At least two unprovoked (or Tamber and Mountz, 2012; Sharma, 2013). -
Causes of Mortality in Early Infantile Epileptic Encephalopathy: a Systematic Review
Epilepsy & Behavior 85 (2018) 32–36 Contents lists available at ScienceDirect Epilepsy & Behavior journal homepage: www.elsevier.com/locate/yebeh Review Causes of mortality in early infantile epileptic encephalopathy: A systematic review Graciane Radaelli a,b, Francisco de Souza Santos b, Wyllians Vendramini Borelli b,LeonardoPisanib, Magda Lahorgue Nunes b,d, Fulvio Alexandre Scorza c,d, Jaderson Costa da Costa b,d,⁎ a Federal University of São Paulo (UNIFESP)/Paulista School of Medicine, São Paulo, Brazil b Brain Institute of Rio Grande do Sul (BraIns), Pontifical Catholic University of Rio Grande do Sul, Porto Alegre, RS, Brazil c Laboratory of Neuroscience, Department of Neurology and Neurosurgery, Federal University of São Paulo, São Paulo, SP, Brazil d CNPq, Brazil article info abstract Article history: Introduction: Early infantile epileptic encephalopathy syndrome (EIEE), also known as Ohtahara syndrome, is an Received 6 March 2018 age-dependent epileptic encephalopathy syndrome defined by clinical features and electroencephalographic Revised 25 April 2018 findings. Epileptic disorders with refractory seizures beginning in the neonatal period and/or early infancy Accepted 5 May 2018 have a potential risk of premature mortality, including sudden death. We aimed to identify the causes of death Available online xxxx in EIEE and conducted a literature survey of fatal outcomes. Methods: We performed a literature search in MEDLINE, EMBASE, and Web of Science for data from inception Keywords: “ ”“ ”“ ”“ ” “ ” Ohtahara syndrome until September 2017. The terms death sudden, unexplained death, SUDEP, lethal, and fatal and the “ ”“ ”“ ”“ Early infantile epileptic syndrome medical subject heading terms epileptic encephalopathy, mortality, death, sudden infant death syn- Suppression burst drome,” and “human” were used in the search strategy. -
Prognostication in Neonatal Hypoxic Ischemic Encephalopathy: a Qualitative Research Study
Prognostication in Neonatal Hypoxic Ischemic Encephalopathy: A Qualitative Research Study Lisa Anne Rasmussen Department of Medicine, Division of Experimental Medicine and Biomedical Ethics Unit McGill University Montreal, Quebec, Canada November 2017 A thesis submitted to McGill University in partial fulfillment of the requirements of the degree of Master of Science in Experimental Medicine, Specialization in Biomedical Ethics ©Lisa Anne Rasmussen, 2017 Abstract Background Hypoxic ischemic encephalopathy is the most frequent cause of neonatal encephalopathy, and results in significant morbidity and mortality. From an ethical and clinical standpoint, neurological prognosis is fundamental in the care of neonates with hypoxic ischemic encephalopathy. However, accurately predicting neurodevelopmental outcomes for neonatal hypoxic ischemic encephalopathy is particular difficult, and fraught with challenges. At present, focused research in this area is limited. Objectives This thesis aims to present a review of the current literature on prognosis and the practice of prognostication in neonatal hypoxic ischemic encephalopathy, focusing on the integral challenges posed by this vulnerable group of neonates. Furthermore, this thesis incorporates an original qualitative study that explores physician perspectives about prognostication in neonatal hypoxic ischemic encephalopathy. The main objective of this thesis is to advance the current understanding of the practice of prognostication in neonatal hypoxic ischemic encephalopathy, in hopes of opening -
Neonatal Seizures Revisited
children Review Neonatal Seizures Revisited Konrad Kaminiów 1 , Sylwia Kozak 1 and Justyna Paprocka 2,* 1 Students’ Scientific Society, Department of Pediatric Neurology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, 40-752 Katowice, Poland; [email protected] (K.K.); [email protected] (S.K.) 2 Department of Pediatric Neurology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, 40-752 Katowice, Poland * Correspondence: [email protected] Abstract: Seizures are the most common neurological disorder in newborns and are most prevalent in the neonatal period. They are mostly caused by severe disorders of the central nervous system (CNS). However, they can also be a sign of the immaturity of the infant’s brain, which is characterized by the presence of specific factors that increase excitation and reduce inhibition. The most common disorders which result in acute brain damage and can manifest as seizures in neonates include hypoxic-ischemic encephalopathy (HIE), ischemic stroke, intracranial hemorrhage, infections of the CNS as well as electrolyte and biochemical disturbances. The therapeutic management of neonates and the prognosis are different depending on the etiology of the disorders that cause seizures which can lead to death or disability. Therefore, establishing a prompt diagnosis and implementing appropriate treatment are significant, as they can limit adverse long-term effects and improve outcomes. In this review paper, we present the latest reports on the etiology, pathomechanism, clinical symptoms and guidelines for the management of neonates with acute symptomatic seizures. Keywords: neonatal seizures; pathophysiology; genetics; inborn errors of metabolism Citation: Kaminiów, K.; Kozak, S.; Paprocka, J. -
235 © Springer Nature Switzerland AG 2019 G. I. Martin, W. Rosenfeld
Index A hemolytic disease, 90 Abdominal distension, 161, 166 hereditary elliptocytosis, 97 Aberrant ventricular conduction, 153, 154, 156 hereditary spherocytosis, 97 Absent uvula, 31 initial laboratory assessment, 94 Adenoviral conjunctivitis, 221 packed red blood cell transfusion, 94 Adenovirus, 77 peripheral smear, 96 Alopecia, 18 red cell indices, reference range, 90 Ambiguous genitalia reticulocyte count, 96 androgen insensitivity syndrome, 211 Rh incompatibility, 90 CAH (see Congenital adrenal hyperplasia (CAH)) rhinovirus, 97 causes of, 206, 211 signs and symptoms, 89, 94 chromosomal analysis, 205, 209 sources of blood loss, 94 DSD, 205 subgaleal hemorrhages, 92 evaluation of, 210 transfusion guidelines, 95 genetic factors for, 204 vascular access, 94 21-hydroxylase deficiency, 207 vital signs per nursery protocol, 92 incidence of, 203 Ankyloglossia, 31 pelvic sonogram, 209 Antacids, 166 sex assignment, 211 Antiarrhythmic medications, 150 sex determination and differentiation, 204 Anti-Ro and anti-La maternal antibodies, 152 46 XY karyotype and, 206 Apgar score, 183, 184 Amblyopia, 218 Aplasia cutis congenita (ACC), 44, 45 American Congress of Obstetricians and Gynecologists Arrhythmia (ACOG), 2, 55, 57 antiarrhythmic medications, 150 Amplitude electroencephalograph (aEEG), 6, 9 benign arrhythmias, 149 Androgen insensitivity syndrome (AIS), 209, 211 bradyarrhythmia Anemia atrioventricular block, 152, 153 ABO incompatibility, 90 EKG rhythm, 151 acute blood loss, 93 initial therapy, 151 blood transfusion therapy, 95, 96 management, -
(12) Patent Application Publication (10) Pub. No.: US 2010/0210567 A1 Bevec (43) Pub
US 2010O2.10567A1 (19) United States (12) Patent Application Publication (10) Pub. No.: US 2010/0210567 A1 Bevec (43) Pub. Date: Aug. 19, 2010 (54) USE OF ATUFTSINASATHERAPEUTIC Publication Classification AGENT (51) Int. Cl. A638/07 (2006.01) (76) Inventor: Dorian Bevec, Germering (DE) C07K 5/103 (2006.01) A6IP35/00 (2006.01) Correspondence Address: A6IPL/I6 (2006.01) WINSTEAD PC A6IP3L/20 (2006.01) i. 2O1 US (52) U.S. Cl. ........................................... 514/18: 530/330 9 (US) (57) ABSTRACT (21) Appl. No.: 12/677,311 The present invention is directed to the use of the peptide compound Thr-Lys-Pro-Arg-OH as a therapeutic agent for (22) PCT Filed: Sep. 9, 2008 the prophylaxis and/or treatment of cancer, autoimmune dis eases, fibrotic diseases, inflammatory diseases, neurodegen (86). PCT No.: PCT/EP2008/007470 erative diseases, infectious diseases, lung diseases, heart and vascular diseases and metabolic diseases. Moreover the S371 (c)(1), present invention relates to pharmaceutical compositions (2), (4) Date: Mar. 10, 2010 preferably inform of a lyophilisate or liquid buffersolution or artificial mother milk formulation or mother milk substitute (30) Foreign Application Priority Data containing the peptide Thr-Lys-Pro-Arg-OH optionally together with at least one pharmaceutically acceptable car Sep. 11, 2007 (EP) .................................. O7017754.8 rier, cryoprotectant, lyoprotectant, excipient and/or diluent. US 2010/0210567 A1 Aug. 19, 2010 USE OF ATUFTSNASATHERAPEUTIC ment of Hepatitis BVirus infection, diseases caused by Hepa AGENT titis B Virus infection, acute hepatitis, chronic hepatitis, full minant liver failure, liver cirrhosis, cancer associated with Hepatitis B Virus infection. 0001. The present invention is directed to the use of the Cancer, Tumors, Proliferative Diseases, Malignancies and peptide compound Thr-Lys-Pro-Arg-OH (Tuftsin) as a thera their Metastases peutic agent for the prophylaxis and/or treatment of cancer, 0008. -
Pediatric Disorders
Neurological Disorder Part 3 - Pediatric Disorders CDKL5 Disorder • Characteristics: • Rare x-linked genetic disorder • CDLK5 mutations cause deficiencies in the protein needed for normal brain development • More common in females; however males with the disorder are affected much more severely than females © Trusted Neurodiagnostics Academy CDKL5 Disorder • Characteristics • CDKL5D mutations can be found in children who have been diagnosed with infantile spasms, Lennox Gastaut syndrome, Rett Syndrome, West Syndrome and autism © Trusted Neurodiagnostics Academy CDKL5 Disorder • Symptoms: • Infantile spasms beginning the first 3 - 6 months of life • Neurodevelopmental impairment • Patients cannot walk, talk, or feed themselves • Repetitive hand movements (stereotypies) © Trusted Neurodiagnostics Academy CDKL5 Disorder • Seizures: • Early onset • Infantile spasms, myoclonic, tonic, tonic-clonic seizures • Status epilepticus and non convulsive status epilepticus can occur © Trusted Neurodiagnostics Academy CDKL5 Disorder • Diagnosis: • Genetic blood testing to confirm the change or mutation on the CDKL5 gene • EEG © Trusted Neurodiagnostics Academy CDKL5 Disorder • EEG Findings: • Early in the disorder • EEG may be normal or slightly abnormal • During progression of the disorder • Some background activity is slow and epileptic discharges can be seen in one or more areas • Burst Suppression • Atypical hypsarrhythmia © Trusted Neurodiagnostics Academy CDKL5 Disorder © Trusted Neurodiagnostics Academy CDKL5 Disorder • Treatment: • Seizures -
Perinatal Asphyxia in the Term Newborn
www.jpnim.com Open Access eISSN: 2281-0692 Journal of Pediatric and Neonatal Individualized Medicine 2014;3(2):e030269 doi: 10.7363/030269 Received: 2014 Oct 01; accepted: 2014 Oct 14; published online: 2014 Oct 21 Review Perinatal asphyxia in the term newborn Roberto Antonucci1, Annalisa Porcella1, Maria Dolores Pilloni2 1Division of Neonatology and Pediatrics, “Nostra Signora di Bonaria” Hospital, San Gavino Monreale, Italy 2Family Planning Clinic, San Gavino Monreale, ASL 6 Sanluri (VS), Italy Proceedings Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014) Cagliari (Italy) · October 25th, 2014 The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken Abstract Despite the important advances in perinatal care in the past decades, asphyxia remains a severe condition leading to significant mortality and morbidity. Perinatal asphyxia has an incidence of 1 to 6 per 1,000 live full-term births, and represents the third most common cause of neonatal death (23%) after preterm birth (28%) and severe infections (26%). Many preconceptional, antepartum and intrapartum risk factors have been shown to be associated with perinatal asphyxia. The standard for defining an intrapartum hypoxic-ischemic event as sufficient to produce moderate to severe neonatal encephalopathy which subsequently leads to cerebral palsy has been established in 3 Consensus statements. The cornerstone of all three statements is the presence of severe metabolic acidosis (pH < 7 and base deficit ≥ 12 mmol/L) at birth in a newborn exhibiting early signs of moderate or severe encephalopathy. Perinatal asphyxia may affect virtually any organ, but hypoxic-ischemic encephalopathy (HIE) is the most studied clinical condition and that is burdened with the most severe sequelae. -
Role of Thompson Score in Predicting Neurodevelopmental Outcome
ROLE OF THOMPSON SCORE IN PREDICTING NEURODEVELOPMENTAL OUTCOME Dissertation submitted to THE TAMILNADU DR.M.G.R.MEDICAL UNIVERSITY In partial fulfilment of the regulations for the award of degree of M.D DEGREE (PEDIATRICS) BRANCH VII INSTITUTE OF SOCIAL PEDIATRICS STANLEY MEDICAL COLLEGE CHENNAI – 600 001 APRIL 2016 DECLARATION I, Dr.B.RUPA , solemnly declare that the dissertation titled “ROLE OF THOMPSON SCORE IN PREDICTING NEURODEVELOPMENTAL OUTCOME”was doneby me at Government Stanley MedicalCollege during 2013- 2016 under the guidance and supervision of my chief Prof. SHANTHI M.D, D.C.H. The dissertation is submitted to The Tamil Nadu Dr.M.G.R Medical University towards the partial fulfilment of the rules and regulations for the M.D. Degree Examination - BRANCH VII - in Pediatrics . Signature of the candidate Dr.B.RUPA Place : Chennai Date: CERTIFICATE BY THE GUIDE This is to certify that the dissertation titled “ROLE OF THOMPSON SCORE IN PREDICTING NEURO DEVELOPMENTAL OUTCOME” is a bonafide research work done under my guidance by Dr.B.RUPA, Postgraduate student, Department of Pediatrics, Government Stanley medical college, The Tamil Nadu Dr.M.G.R Medical University, Chennai, in partial fulfilment of the requirement of the award for the degree of M.D PEDIATRICS - BRANCH VII. Place : Chennai Signature of the guide Date : Dr.Aravind MD, DCH Professor of NICU RSRM hospital Institute of Social Pediatrics Stanley medical college, Chennai – 600001 CERTIFICATE BY THE INSTITUTION This is to certify that the dissertation titled “ROLE OF THOMPSON SCORE IN PREDICTING NEURO DEVLOPMENTAL OUTCOME”is submitted by Dr.B.RUPA to The Tamilnadu Dr.M.G.R Medical University, Chennai in partial fulfilment of the requirement of the award for the degree of M.D BRANCH VII (PEDIATRICS) and is a bonafide work done by her under our direct supervision and guidance, during the academic year 2014 -2015 Place: Chennai DR.Aravind MD, DCH Date: Professor of NICU RSRM Hospital Institute of Social Pediatrics Stanley Medical College Chennai –600001. -
Genetic and Phenotypic Heterogeneity Suggest Therapeutic Implications in SCN2A-Related Disorders
doi:10.1093/brain/awx054 BRAIN 2017: Page 1 of 21 | 1 Genetic and phenotypic heterogeneity suggest therapeutic implications in SCN2A-related disorders Markus Wolff,1,Ã Katrine M. Johannesen,2,3,Ã Ulrike B. S. Hedrich,4,Ã Silvia Masnada,5 Guido Rubboli,2,6 Elena Gardella,2,3 Gaetan Lesca,7,8,9 Dorothe´e Ville,10 Mathieu Milh,11,12 Laurent Villard,12 Alexandra Afenjar,13 Sandra Chantot-Bastaraud,13 Cyril Mignot,14 Caroline Lardennois,15 Caroline Nava,16,17 Niklas Schwarz,4 Marion Ge´rard,18 Laurence Perrin,19 Diane Doummar,20 Ste´phane Auvin,21,22 Maria J. Miranda,23 Maja Hempel,24 Eva Brilstra,25 Nine Knoers,25 Nienke Verbeek,25 Marjan van Kempen,25 Kees P. Braun,26 Grazia Mancini,27 Saskia Biskup,28 Konstanze Ho¨rtnagel,28 Miriam Do¨cker,28 Thomas Bast,29 Tobias Loddenkemper,30 Lily Wong-Kisiel,31 Friedrich M. Baumeister,32 Walid Fazeli,33 Pasquale Striano,34 Robertino Dilena,35 Elena Fontana,36 Federico Zara,37 Gerhard Kurlemann,38 Joerg Klepper,39 Jess G. Thoene,40 Daniel H. Arndt,41 Nicolas Deconinck,42 Thomas Schmitt-Mechelke,43 Oliver Maier,44 Hiltrud Muhle,45 Beverly Wical,46 Claudio Finetti,47 Reinhard Bru¨ckner,48 Joachim Pietz,49 Gu¨nther Golla,50 Dinesh Jillella,51 Karen M. Linnet,52 Perrine Charles,53 Ute Moog,54 Eve O˜ iglane-Shlik,55 John F. Mantovani,56 Kristen Park,57 Marie Deprez,58 Damien Lederer,58 Sandrine Mary,58 Emmanuel Scalais,59 Laila Selim,60 Rudy Van Coster,61 Lieven Lagae,62 Marina Nikanorova,2 Helle Hjalgrim,2,3 G. -
Childhood Neurological Conditions and Disorders
Childhood neurological conditions and disorders Please note: We do include rare conditions. If your one is not in the list below we will still consider your survey responses. Epilepsies Autosomal dominant epilepsy with auditory features (ADEAF) Autosomal-dominant nocturnal frontal lobe epilepsy (ADNFLE) Benign epilepsy with centrotemporal spikes (BECTS) Benign familial infantile epilepsy Benign familial neonatal epilepsy (BFNE) Benign infantile epilepsy Benign neonatal seizures (BNS) Childhood absence epilepsy (CAE) Cyclin Dependent Kinase-Like 5 (CDKL5) Deficiency Disorder Dravet syndrome Early myoclonic encephalopathy (EME) Epilepsy of infancy with migrating focal seizures Epilepsy with generalized tonic–clonic seizures alone Epilepsy with myoclonic absences Epilepsy with myoclonic atonic (previously astatic) seizures Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS) Familial focal epilepsy with variable foci (childhood to adult) Febrile seizures (FS) Febrile seizures plus (FS+) (can start in infancy) Gelastic seizures with hypothalamic hamartoma Hemiconvulsion–hemiplegia–epilepsy Juvenile absence epilepsy (JAE) Juvenile myoclonic epilepsy (JME) Landau-Kleffner syndrome (LKS) Late onset childhood occipital epilepsy (Gastaut type) Lennox-Gastaut syndrome Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE with HS) Myoclonic epilepsy in infancy (MEI) Ohtahara syndrome Panayiotopoulos syndrome Photosensitive absence epilepsies including Jeavons syndrome, Sunflower syndrome Progressive myoclonus epilepsies