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Kernicterus

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https://www.readbyqxmd.com/read/28919619/comparison-of-serum-bilirubin-with-transcutaneous-bilirubinometry-in-late-preterm-and-term-newborn
#1
N Nahar, M A Mannan, A C Dey, F Ahmed, K A Khan, I Jahan, S K Dey, M Shahidullah
Neonatal jaundice or hyperbilirubinemia is a common occurrence in newborns. It can progress to develop kernicterus unless intervention is initiated. Severity and decision for management are usually based on serum bilirubin which needs blood sampling. Transcutaneous bilirubin measurement is a noninvasive technique and correlates closely with serum bilirubin. This Cross sectional study was done in the Department of Neonatology, Bangabandhu Sheikh Mujib Medical University from March 2013 to August 2014 to evaluate the transcutaneous bilirubin in comparison to serum bilirubin...
July 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28919246/filtered-sunlight-solar-powered-phototherapy-and-other-strategies-for-managing-neonatal-jaundice-in-low-resource-settings
#2
Tina M Slusher, Louise Tina Day, Tolulope Ogundele, Nick Woolfield, Joseph Aderinsola Owa
Challenges in treating severe neonatal jaundice in low and middle-income country settings still exist at many levels. These include: a lack of awareness of causes and prevention by families, communities and even sometimes health care professionals; insufficient, ineffective, high quality affordable diagnostic and therapeutic options; limited availability of rehabilitation provision for kernicterus. Collectively these challenges lead to an unacceptably high global morbidity and mortality from severe neonatal jaundice...
September 15, 2017: Early Human Development
https://www.readbyqxmd.com/read/28815739/kernicterus-in-a-boy-with-ornithine-transcarbamylase-deficiency-a-case-report
#3
Eduardo López-Corella, Isabel Ibarra-González, Cynthia Fernández-Lainez, Miguel Á Rodríguez-Weber, Sara Guillén-Lopez, Leticia Belmont-Martínez, David Agüero-Linares, Marcela Vela-Amieva
Ornithine transcarbamylase deficiency (OTCD) is an X-linked urea cycle defect associated with severe and usually fatal hyperammonemia. This study describes a patient with early onset lethal OTCD due to a known pathogenic variant (c.298+1G>A), as well as the novel autopsy finding of kernicterus with relatively low blood concentration of unconjugated bilirubin (UCB) (11.55 mg/dL). The patient was a full-term male with a family history of two previous male siblings who died as newborns after acute neurologic deterioration...
August 16, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28814249/the-neurological-sequelae-of-neonatal-hyperbilirubinemia-definitions-diagnosis-and-treatment-of-the-kernicterus-spectrum-disorders-ksds
#4
Steven Shapiro, Jean Baptiste Le Pichon, Sean M Riordan, Jon Watchkoe
Despite its lengthy history the study of benign jaundice, hyperbilirubinemia and kernicterus suffers from a lack of clarity and consistency in the key terms used to describe both the clinical and pathophysiological nature of these conditions. This has led to multiple terms being used to describe similar conditions (i.e. kernicterus and chronic bilirubin encephalopathy) or the same term being used to describe different concepts (i.e. BIND as a descriptor of the signs of bilirubin neurotoxicity, mild kernicterus, or as a scale to assess bilirubin toxicity)...
August 14, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28762235/fluid-supplementation-for-neonatal-unconjugated-hyperbilirubinaemia
#5
REVIEW
Nai Ming Lai, Azanna Ahmad Kamar, Yao Mun Choo, Juin Yee Kong, Chin Fang Ngim
BACKGROUND: Neonatal hyperbilirubinaemia is a common problem which carries a risk of neurotoxicity. Certain infants who have hyperbilirubinaemia develop bilirubin encephalopathy and kernicterus which may lead to long-term disability. Phototherapy is currently the mainstay of treatment for neonatal hyperbilirubinaemia. Among the adjunctive measures to compliment the effects of phototherapy, fluid supplementation has been proposed to reduce serum bilirubin levels. The mechanism of action proposed includes direct dilutional effects of intravenous (IV) fluids, or enhancement of peristalsis to reduce enterohepatic circulation by oral fluid supplementation...
August 1, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28754279/-g6pd-deficiency-in-females-with-neonatal-revelation-report-of-four-cases
#6
A Renault, D Mitanchez, A Cortey
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human erythrocyte enzyme defect, estimated to affect approximately 4 million people worldwide. It is associated with severe neonatal hyperbilirubinemia, which may lead to bilirubin encephalopathy and kernicterus, and with hemolytic crisis. G6PD deficiency is an X-linked enzymopathy affecting hemizygous males, homozygous females, and also a subset of heterozygous females via chromosome X inactivation. We report four cases of female newborns with neonatal hyperbilirubinemia related to a G6PD deficiency and followed by the Centre national de référence en hémobiologie périnatale (CNRHP) from November 2013 to July 2014...
July 25, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28730953/an-update-on-predicting-severe-hyperbilirubinemia-and-bilirubin-neurotoxicity-risks-in-neonates
#7
Tom Newman, M Jeffrey Maisels
Extreme hyperbilirubinemia and kernicterus, though rare, continue to occur despite the adoption of universal screening. Unless they are known to have glucose-6-phosphate dehydrogenase deficiency, infants who currently develop kernicterus in high resource countries are often otherwise healthy newborns discharged from the well-baby nursery. In this review, we highlight risk factors that increase the risk of a newborn ≥35 weeks gestational age developing severe hyperbilirubinemia, as well as the risk factors that increase the hyperbilirubinemic infant's risk of kernicterus...
July 20, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28721814/refractory-causes-of-kernicterus-in-developed-countries-can-we-eradicate-g6pd-deficiency-triggered-and-low-bilirubin-kernicterus
#8
Jon Freeman Watchko
BACKGROUND: Glucose-6-phosphate dehydrogenase (G6PD) deficiency triggered and low-bilirubin kernicterus persist despite current prevention strategies. OBJECTIVE: Efforts to eradicate bilirubin induced brain injury in these two conditions will require novel approaches to riskassessment and hyperbilirubinemia evaluation. METHOD: In the case of G6PD deficency, a heightenedawareness of populations at risk on who expanded kernicterus preventio strategies can befocused including intensified parental engagement, education and counselling on neonataljaundice...
July 18, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28605485/follow-up-of-children-with-kernicterus-in-kano-nigeria
#9
Zubaida Ladan Farouk, Abdussalam Muhammed, Safiya Gambo, Maria Mukhtar-Yola, Shehu Umar Abdullahi, Tina M Slusher
Introduction: Acute bilirubin encephalopathy (ABE) is associated with long-term sequelae (kernicterus). It continues to be a significant issue in our region of Nigeria, accounting for much morbidity and mortality. Herein we report the outcome of neonates with ABE seen at our centre. Methodology: We established a surveillance of children who had ABE and returned to follow-up from prospective cases of ABE (2012-2014). ABE was diagnosed based on a bilirubin-induced neurologic dysfunction score of ≥ 1...
June 12, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/28605161/the-use-of-therapeutic-plasma-exchange-to-reduce-serum-bilirubin-in-a-dog-with-kernicterus
#10
Tricia Tovar, Sarah Deitschel, Christine Guenther
OBJECTIVE: To describe the use of a manual method of therapeutic plasma exchange to reduce total serum bilirubin, manage kernicterus, and halt progression of neurological dysfunction in a dog with immune-mediated hemolytic anemia (IMHA). CASE SUMMARY: A 5-year-old male neutered Lhasa Apso diagnosed with IMHA developed acute onset neurologic signs consistent with kernicterus. Manual therapeutic plasma exchange was performed in an attempt to reduce total serum bilirubin...
July 2017: Journal of Veterinary Emergency and Critical Care
https://www.readbyqxmd.com/read/28590786/liver-fibrosis-associated-with-crigler-najjar-syndrome-in-a-compound-heterozygote
#11
Cynthia R Fata, Lynette A Gillis, M Cristina Pacheco
Crigler-Najjar syndrome is a hereditary unconjugated hyperbilirubinemia. Two forms of the disease are recognized. Type I is more severe and results in kernicterus if left untreated, and Type II is less severe and responds to phenobarbital. While Crigler-Najjar syndrome is thought by many to have normal liver histology, few reports of the liver pathology exist. Herein, we present a 19-year-old patient with Crigler-Najjar who underwent liver transplantation. The liver showed marked canalicular cholestasis with portal and variable, delicate, bridging fibrosis...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28554938/term-admissions-to-neonatal-units-in-england-a-role-for-transitional-care-a-retrospective-cohort-study
#12
Cheryl Battersby, Stephanie Michaelides, Michele Upton, Janet M Rennie
OBJECTIVE: To identify the primary reasons for term admissions to neonatal units in England, to determine risk factors for admissions for jaundice and to estimate the proportion who can be cared for in a transitional setting without separation of mother and baby. DESIGN: Retrospective observational study using neonatal unit admission data from the National Neonatal Research Database and data of live births in England from the Office for National Statistics. SETTING: All 163 neonatal units in England 2011-2013...
May 29, 2017: BMJ Open
https://www.readbyqxmd.com/read/28503958/neonatal-management-and-outcome-in-alloimmune-hemolytic-disease
#13
REVIEW
Isabelle M C Ree, Vivianne E H J Smits-Wintjens, Johanna G van der Bom, Jeanine M M van Klink, Dick Oepkes, Enrico Lopriore
Hemolytic disease of the fetus and newborn (HDFN) occurs when fetal and neonatal erythroid cells are destroyed by maternal erythrocyte alloantibodies, it leads to anemia and hydrops in the fetus, and hyperbilirubinemia and kernicterus in the newborn. Postnatal care consists of intensive phototherapy and exchange transfusions to treat severe hyperbilirubinemia and top-up transfusions to treat early and late anemia. Other postnatal complications have been reported such as thrombocytopenia, iron overload and cholestasis requiring specific management...
July 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28461823/neonatal-indirect-hyperbilirubinemia-and-glucose-6-phosphate-dehydrogenase-deficiency
#14
Hasan M Isa, Masooma S Mohamed, Afaf M Mohamed, Adel Abdulla, Fuad Abdulla
PURPOSE: This study aimed to determine the prevalence of glucose-6-phosphate dehydrogenase (G6PD) deficiency among infants with neonatal indirect hyperbilirubinemia (NIH); compare G6PD-deficient and G6PD-normal patients regarding hyperbilirubinemia and need for exchange transfusions (ET); and assess risk factors for ET and kernicterus. METHODS: This is a case-control retrospective study. Medical records of NIH patients admitted to the Pediatric Department, Salmaniya Medical Complex, Bahrain, between January 2007 and June 2010 were reviewed...
April 2017: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28409762/readmission-for-neonatal-hyperbilirubinemia-in-an-area-with-a-high-prevalence-of-glucose-6-phosphate-dehydrogenase-deficiency-a-hospital-based-retrospective-study
#15
A Al-Omran, S Al-Abdi, Z Al-Salam
BACKGROUND: Hyperbilirubinemia is one of the most common causes of neonatal readmission to hospital. AIMS: To assess risk factors for hyperbilirubinemia among neonates readmitted for this condition and the ratio of the mean corpuscular hemoglobin concentration (MCHC) to the mean corpuscular volume (MCV). METHODS: We retrospectively studied the clinical and laboratory findings, management and possible risk factors for hyperbilirubinemia in 301 neonates born at ≥35 weeks gestation and readmitted to hospital owing to hyperbilirubinemia over five years...
2017: Journal of Neonatal-perinatal Medicine
https://www.readbyqxmd.com/read/28340583/inflammatory-signature-of-cerebellar-neurodegeneration-during-neonatal-hyperbilirubinemia-in-ugt1-mouse-model
#16
Simone Vodret, Giulia Bortolussi, Jana Jašprová, Libor Vitek, Andrés F Muro
BACKGROUND: Severe hyperbilirubinemia is toxic during central nervous system development. Prolonged and uncontrolled high levels of unconjugated bilirubin lead to bilirubin-induced neurological damage and eventually death by kernicterus. Bilirubin neurotoxicity is characterized by a wide array of neurological deficits, including irreversible abnormalities in motor, sensitive and cognitive functions, due to bilirubin accumulation in the brain. Despite the abundant literature documenting the in vitro and in vivo toxic effects of bilirubin, it is unclear which molecular and cellular events actually characterize bilirubin-induced neurodegeneration in vivo...
March 24, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28283555/developmental-genetic-dietary-and-xenobiotic-influences-on-neonatal-hyperbilirubinemia
#17
REVIEW
Mei-Fei Yueh, Shujuan Chen, Nghia Nguyen, Robert H Tukey
Hyperbilirubinemia, caused by the accumulation of unconjugated bilirubin, is one of the most common clinical diagnoses in both premature and term newborns. Owing to the fact that bilirubin is metabolized solely through glucuronidation by UDP-glucuronosyltransferase (UGT) 1A1, it is now known that immaturity of UGT1A1, in combination with the overproduction of bilirubin during the developmental stage, acts as a bottleneck to bilirubin elimination and predisposes the infant to high total serum bilirubin levels...
May 2017: Molecular Pharmacology
https://www.readbyqxmd.com/read/28251113/rh-isoimmunized-pregnancy-managed-noninvasively-a-report-of-two-cases
#18
Deepti Jain
Pregnancy with Rh isoimmunization is a grave situation. Two women with indirect Coombs test (ICT) positive were managed conservatively with a favorable outcome. Peak systolic velocity (PSV) of middle cerebral artery (MCA) was measured every 2 weeks, and pregnancy continued till the value remained <1.5 mean of median. In one woman, the pregnancy could be prolonged to 37 weeks when delivery was induced and the neonate did not develop hyperbilirubinemia. In the second woman with bad obstetric history, when a highly positive ICT was reported, intravenous immunoglobulin (IVIG) was given in a single dose of 5 g every 2 weeks starting at 27 weeks, for a total of three doses, along with measurement of PSV of MCA...
January 2017: International Journal of Applied and Basic Medical Research
https://www.readbyqxmd.com/read/28167773/intestinal-ncor1-a-regulator-of-epithelial-cell-maturation-controls-neonatal-hyperbilirubinemia
#19
Shujuan Chen, Wenqi Lu, Mei-Fei Yueh, Eva Rettenmeier, Miao Liu, Johan Auwerx, Ruth T Yu, Ronald M Evans, Kepeng Wang, Michael Karin, Robert H Tukey
Severe neonatal hyperbilirubinemia (SNH) and the onset of bilirubin encephalopathy and kernicterus result in part from delayed expression of UDP-glucuronosyltransferase 1A1 (UGT1A1) and the inability to metabolize bilirubin. Although there is a good understanding of the early events after birth that lead to the rapid increase in serum bilirubin, the events that control delayed expression of UGT1A1 during development remain a mystery. Humanized UGT1 (hUGT1) mice develop SNH spontaneously, which is linked to repression of both liver and intestinal UGT1A1...
February 21, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28155818/fate-of-neural-progenitor-cells-transplanted-into-jaundiced-and-nonjaundiced-rat-brains
#20
Fu-Chen Yang, Sean M Riordan, Michelle Winter, Li Gan, Peter G Smith, Jay L Vivian, Steven M Shapiro, John A Stanford
High levels of bilirubin in infants can cause kernicterus, which includes basal ganglia damage and dystonia. Stem cell transplantation may be an effective treatment for this disease. In this study, we transplanted human neural progenitor cells differentiated toward propriospinal interneurons into the striatum of 20-day-old spontaneously jaundiced (jj) Gunn rats and nonjaundiced (Nj) littermates. Using immunohistochemical methods, we found that grafted cells survived and grew fibers in jj and Nj brains 3 weeks after transplantation...
April 13, 2017: Cell Transplantation
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