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Kernicterus

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https://www.readbyqxmd.com/read/29892033/extreme-neonatal-hyperbilirubinemia-acute-bilirubin-encephalopathy-and-kernicterus-spectrum-disorder-in-children-with-galactosemia
#1
Laura F Bech, Mette Line Donneborg, Allan M Lund, Finn Ebbesen
BACKGROUND: Galactosemia has not been recognized as a cause of extreme neonatal hyperbilirubinemia, although growing evidence supports this association. METHODS: In a retrospective cohort study, we identified children with galactosemia due to GALT deficiency using the Danish Metabolic Laboratory Database. Among these, we identified children with extreme neonatal hyperbilirubinemia or symptoms of ABE. Extreme neonatal hyperbilirubinemia was defined as maximum total serum bilirubin (TSBmax) ) level ≥450 µmol/L and a ratio of conjugated serum bilirubin/TSB <0...
May 31, 2018: Pediatric Research
https://www.readbyqxmd.com/read/29887684/b-l-basal-ganglia-lesions-in-a-child-leading-to-a-diagnosis-of-glucose-6-phosphate-dehydrogenase-deficiency
#2
Nidhi Prabhakar, Chirag K Ahuja, Niranjan Khandelwal
Bilateral basal ganglia lesions are a common non-specific finding seen in many diseases. One of the differential diagnoses for it, in a child, is kernicterus occurring due to hyperbilirubinemia. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common cause of severe hyperbilirubinemia. A 1-year old child presented to the hospital with history of generalized dystonia in the previous 3 days. MRI showed evidence of symmetrical lesions in bilateral globus pallidus, which were hyperintense on T2/FLAIR and isointense on T1...
April 2018: Annals of Neurosciences
https://www.readbyqxmd.com/read/29845869/short-term-development-and-fate-of-mge-like-neural-progenitor-cells-in-jaundiced-and-non-jaundiced-rat-brain
#3
Fu-Chen Yang, Julia Draper, Peter G Smith, Jay L Vivian, Steven M Shapiro, John A Stanford
Neonatal hyperbilirubinemia targets specific brain regions and can lead to kernicterus. One of the most debilitating symptoms of kernicterus is dystonia, which results from bilirubin toxicity to the globus pallidus (GP). Stem cell transplantation into the GP to replace lost neurons and restore basal ganglia circuits function is a potential therapeutic strategy to treat dystonia in kernicterus. In this study we transplanted human medial ganglionic eminence (MGE)-like neural progenitor cells (NPCs) that we differentiated into a primarily gamma-aminobutyric acid (GABA)ergic phenotype, into the GP of non-immunosuppressed jaundiced (jj) and non-jaundiced (Nj) rats...
January 1, 2018: Cell Transplantation
https://www.readbyqxmd.com/read/29802103/learning-from-claims-hyperbilirubinaemia-and-kernicterus
#4
Janet M Rennie, Jeanette Beer, Michele Upton
We examined claims made against the National Health Service (NHS) involving neonatal jaundice in order to determine whether there were lessons that could be learnt from common themes.This was a retrospective anonymised study using information from the NHS Resolution database for 2001-2011.Twenty cases (16 males) had sufficient information for analysis. Fifteen had confirmed cerebral palsy and two young children had damage to the globus pallidus without confirmed CP. In three cases, the outcome was uncertain...
May 25, 2018: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/29781686/the-prevalence-of-neonatal-jaundice-and-risk-factors-in-healthy-term-neonates-at-national-district-hospital-in-bloemfontein
#5
Hanneke Brits, Jeanie Adendorff, Dyanti Huisamen, Dahne Beukes, Kristian Botha, Hanre Herbst, Gina Joubert
BACKGROUND:  Neonatal jaundice affects one in two infants globally. The jaundice is the result of an accumulation of bilirubin as foetal haemoglobin is metabolised by the immature liver. High serum levels of bilirubin result in lethargy, poor feeding and kernicterus of the infant. AIM:  The main aim of this article was to determine the prevalence of neonatal jaundice and secondly to explore its risk factors in healthy term neonates. SETTING:  Maternity ward, National District Hospital, Bloemfontein, South Africa...
April 12, 2018: African Journal of Primary Health Care & Family Medicine
https://www.readbyqxmd.com/read/29778298/a-case-report-of-kernicterus-in-a-neonate-with-hemolytic-disease-of-newborn-lessons-to-learn
#6
Maria F Saavedra, Praveen Kumar
No abstract text is available yet for this article.
May 16, 2018: Journal of Pediatric Health Care
https://www.readbyqxmd.com/read/29672616/adherence-to-hyperbilirubinemia-guidelines-by-midwives-general-practitioners-and-pediatricians-in-indonesia
#7
Mahendra T A Sampurna, Kinanti A Ratnasari, Risa Etika, Christian V Hulzebos, Peter H Dijk, Arend F Bos, Pieter J J Sauer
Severe hyperbilirubinemia, which may result in kernicterus, is seen more frequently in low and middle-income countries, such as Indonesia, than in high-income countries. In Indonesia midwives, general practitioners (GPs), and pediatricians are involved in the care of jaundiced newborn infants. It is unknown whether the high incidence of severe hyperbilirubinemia in this country is related to a lack of awareness of existing hyperbilirubinemia guidelines issued by, for example, the World Health Organization, the American Academy of Pediatrics, or the Indonesian Health Ministry, or to a lack of adherence to such guidelines...
2018: PloS One
https://www.readbyqxmd.com/read/29593357/patterns-of-acute-bilirubin-encephalopathy-in-nigeria-a-multicenter-pre-intervention-study
#8
Udochukwu M Diala, Richard P Wennberg, Isa Abdulkadir, Zubaida L Farouk, Carlos D Coda Zabetta, Efe Omoyibo, Abieyuwa Emokpae, Aleksandr Aravkin, Bose Toma, Stephen Oguche, Tina Slusher
BACKGROUND: Acute bilirubin encephalopathy (ABE) is an important cause of neonatal morbidity in Nigeria, accounting for 5-14% of neonatal deaths. Most newborns with severe ABE have irreversible damage before receiving treatment emphasizing the need for timely pre-admission monitoring and referral. There is limited evidence that educational interventions targeting mothers and health care providers will reduce delayed care. OBJECTIVE: To provide baseline data on the incidence of ABE and associated pre-admission risk factors in five centers of Nigeria in order to evaluate the effect of subsequent educational interventions on outcome...
March 28, 2018: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/29551014/immunoglobulin-for-alloimmune-hemolytic-disease-in-neonates
#9
REVIEW
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
https://www.readbyqxmd.com/read/29534860/corrigendum-to-a-new-rat-model-of-neonatal-bilirubin-encephalopathy-kernicterus-j-pharmacol-toxicol-methods-84-2017-44-50
#10
Naser Amini, Nasim Vousooghi, Mansoureh Soleimani, Ali Samadikuchaksaraei, Mehdi Akbari, Hosein Safakheil, Pezhman Atafimanesh, Ali Shahbazi, Peiman Brouki Milan, Sara Ramezani, Masoud Mozafari, Mohammad Taghi Joghataei
No abstract text is available yet for this article.
March 11, 2018: Journal of Pharmacological and Toxicological Methods
https://www.readbyqxmd.com/read/29510380/the-neuroprotective-effects-of-hypothermia-on-bilirubin-induced-neurotoxicity-in-vitro
#11
Nazli Kuter, Nese Aysit-Altuncu, Gurkan Ozturk, Eren Ozek
BACKGROUND: In high-risk newborns indirect hyperbilirubinemia can lead to acute bilirubin encephalopathy and kernicterus. Despite the current therapeutic modalities, preventing or reversing the neurotoxicity cannot be achieved in all infants. OBJECTIVE: To investigate the neuroprotective effects of hypothermia on bilirubin-induced toxicity in primary mouse neuronal cell cultures. METHODS: Hippocampal cell cultures, isolated from newborn mouse brains, were incubated with unconjugated bilirubin (UCB) at 3 days in vitro (DIV) and immediately exposed to varying degrees of hypothermia...
March 6, 2018: Neonatology
https://www.readbyqxmd.com/read/29472193/outcomes-of-neonatal-jaundice-in-taiwan
#12
Pei-Chen Tsao, Hsin-Ling Yeh, Yen-Chen Chang, Po-Huang Chiang, Yu-Shih Shiau, Szu-Hui Chiang, Wen-Jue Soong, Mei-Jy Jeng, Kwang-Jen Hsiao
OBJECTIVE: To investigate the burden of clinically significant neonatal jaundice (SNJ) in Taiwan, 2000 - 2010. STUDY DESIGN: The nationwide, population-based health insurance database in Taiwan was used to investigate the incidence, kernicterus rate and mortality rates of SNJ cohort born between 2000 and 2010. RESULTS: From 2000 to 2010, up to 242 546 patients admitted with neonatal jaundice (NJ) were identified. The incidence of SNJ was 5...
February 22, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29458193/attenuation-of-neuro-inflammation-improves-survival-and-neurodegeneration-in-a-mouse-model-of-severe-neonatal-hyperbilirubinemia
#13
Simone Vodret, Giulia Bortolussi, Alessandra Iaconcig, Elena Martinelli, Claudio Tiribelli, Andrés F Muro
All pre-term newborns and a high proportion of term newborns develop neonatal jaundice. Neonatal jaundice is usually a benign condition and self-resolves within few days after birth. However, a combination of unfavorable complications may lead to acute hyperbilirubinemia. Excessive hyperbilirubinemia may be toxic for the developing nervous system leading to severe neurological damage and death by kernicterus. Survivors show irreversible neurological deficits such as motor, sensitive and cognitive abnormalities...
February 16, 2018: Brain, Behavior, and Immunity
https://www.readbyqxmd.com/read/29419612/effectiveness-of-universal-hyperbilirubinemia-screening-on-newborn-health-a-systematic-review-protocol
#14
Faiza Khurshid, Jennifer Medves
The objective of this review is to assess the effectiveness of the universal hyperbilirubinemia screening program on common newborn health outcomes.Specifically, the review will assess: the incidence of severe hyperbilirubinemia/kernicterus/exchange transfusion, rate of readmission due to jaundice, length of hospital stay on birth admission, rate and utilization of phototherapy during birth hospitalization, and jaundice related emergency visits.
February 2018: JBI Database of Systematic Reviews and Implementation Reports
https://www.readbyqxmd.com/read/29410540/abo-hemolytic-disease-of-the-fetus-and-newborn-thirteen-years-of-data-after-implementing-a-universal-bilirubin-screening-and-management-program
#15
R D Christensen, V L Baer, B C MacQueen, E A O'Brien, S J Ilstrup
OBJECTIVE: ABO hemolytic disease occurs among neonates with blood groups A or B delivered to group O women. Extreme neonatal hyperbilirubinemia due to ABO disease has been reported, but its frequency is not well known. We sought to determine the odds of developing severe ABO hemolytic disease in the 13 years since adopting universal bilirubin screening/management in the Intermountain Healthcare system. STUDY DESIGN: We conducted a retrospective analysis of neonates born between 2004 and 2016, defining "severe hemolytic disease" as; (1) total serum bilirubin (TSB) >25 mg/dL, or (2) hospital readmission for jaundice, or (3) bilirubin encephalopathy...
February 6, 2018: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/29399656/severe-neonatal-hyperbilirubinemia-in-crigler-najjar-syndrome-model-mice-can-be-reversed-with-zinc-protoporphyrin
#16
Ryoichi Fujiwara, Ryo Mitsugi, Asuka Uemura, Tomoo Itoh, Robert H Tukey
Neurotoxic bilirubin is solely conjugated by UDP-glucuronosyltransferase (UGT) 1A1. Due to an inadequate function of UGT1A1, human neonates develop mild to severe physiological hyperbilirubinemia. Accumulation of bilirubin in the brain leads to the onset of irreversible brain damage called kernicterus. Breastfeeding is one of the most significant factors that increase the risk of developing kernicterus in infants. Why does the most natural way of feeding increase the risk of brain damage or even death? This question leads to the hypothesis that breast milk-induced neonatal hyperbilirubinemia might bring certain benefits to the body...
October 2017: Hepatology Communications
https://www.readbyqxmd.com/read/29302043/heme-oxygenase-1-genetic-variants-and-the-conundrum-of-hyperbilirubinemia-in-african-american-newborns
#17
David L Schutzman, Erica Gatien, Samuel Ajayi, Ronald J Wong
BACKGROUND: African-American (AA) infants are known to have, overall, lower bilirubin levels than infants of other ethnicities during their birth hospitalization. However, they are known to have a higher incidence of severe hyperbilirubinemia and are over represented in the US Kernicterus Registry. Heme oxygenase-1 (HO) is the rate limiting enzyme in heme metabolism leading to the equimolar production of bilirubin, carbon monoxide (CO) and free iron (Fe). Short (S) (GT)n repeats (<25) in the promoter region of the gene encoding the inducible HO-1 isozyme augment its expression, while long (L) repeats (>33) lead to an attenuation, modulating the production of bilirubin and CO...
January 4, 2018: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/29240507/neonatal-jaundice-aetiology-diagnosis-and-treatment
#18
Subhabrata Mitra, Janet Rennie
A significant proportion of term and preterm infants develop neonatal jaundice. Jaundice in an otherwise healthy term infant is the most common reason for readmission to hospital. Jaundice is caused by an increase in serum bilirubin levels, largely as a result of breakdown of red blood cells. Bilirubin is conveyed in the blood as 'unconjugated' bilirubin, largely bound to albumin. The liver converts bilirubin into a conjugated form which is excreted in the bile. Very high levels of unconjugated bilirubin are neurotoxic...
December 2, 2017: British Journal of Hospital Medicine
https://www.readbyqxmd.com/read/29180541/neonatal-brain-injuries-in-england-population-based-incidence-derived-from-routinely-recorded-clinical-data-held-in-the-national-neonatal-research-database
#19
Chris Gale, Yevgeniy Statnikov, Sena Jawad, Sabita N Uthaya, Neena Modi
OBJECTIVE: In 2015, the Department of Health in England announced an ambition to reduce 'brain injuries occurring during or soon after birth'. We describe the development of a pragmatic case definition and present annual incidence rates. DESIGN: Retrospective cohort study using data held in the National Neonatal Research Database (NNRD) extracted from neonatal electronic patient records from all National Health Service (NHS) neonatal units in England, Wales and Scotland...
October 22, 2017: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/29166181/sensitivity-specificity-and-accuracy-of-kramer-examination-of-neonatal-jaundice-comparison-with-total-bilirubin-serum
#20
Zesi Aprillia, Dewi Gayatri, Fajar Tri Waluyanti
Neonatal jaundice often occurs in the first week after birth. Early detection of neonatal jaundice can prevent kernicterus as a complication of high levels of bilirubin in neonates. Visual assessment is one of the examinations introduced by Kramer to assess the presence of neonatal jaundice. The purpose of this study was to determine the sensitivity, specificity, and accuracy of Kramer's visual assessment in the examination of neonatal jaundice. This study used cross-sectional design to examine 102 infants, with the sample selected by the convenience sampling method...
2017: Comprehensive Child and Adolescent Nursing
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