|3.||Glucosephosphate Dehydrogenase Deficiency
|1.||Bhutani, Vinod K: 13 articles (02/2015 - 05/2003)|
|2.||Brites, Dora: 12 articles (01/2016 - 01/2002)|
|3.||Shapiro, Steven M: 10 articles (06/2011 - 11/2002)|
|4.||Watchko, Jon F: 8 articles (11/2014 - 10/2003)|
|5.||Tiribelli, Claudio: 8 articles (03/2014 - 07/2003)|
|6.||Fernandes, Adelaide: 6 articles (01/2016 - 09/2007)|
|7.||Kaplan, Michael: 6 articles (06/2011 - 04/2002)|
|8.||Ahlfors, Charles E: 6 articles (04/2010 - 05/2003)|
|9.||Slusher, Tina M: 5 articles (01/2015 - 06/2004)|
|10.||Wennberg, Richard P: 5 articles (11/2014 - 10/2004)|
10/01/1985 - "Thus, in the undamaged rat brain, bilirubin is rapidly cleared, in contrast to its persistence in autopsy-proven human kernicterus. "
07/07/1998 - "We suggest that the unstable UGT1A1 polymorphism may serve to "fine-tune" the plasma bilirubin level within population groups, maintaining it at a high enough level to provide protection against oxidative damage, but at a level that is sufficiently low to prevent kernicterus in infants."
01/01/2006 - "Lapses in care have been attributed as root causes for kernicterus in an era when there should be no barriers to safe and effective bilirubin reduction strategies. "
11/01/2000 - "He received bilirubin adsorption therapy several times, and the bilirubin encephalopathy improved in response to the fall in the serum level of bilirubin. "
09/01/2015 - "This study aimed to evaluate peak serum total bilirubin (TB) and unbound bilirubin (UB) levels in preterm infants with clinical kernicterus (KI) who were diagnosed by clinical findings during infancy. "
|2.||Glucosephosphate Dehydrogenase (Glucose 6 Phosphate Dehydrogenase)IBA
05/01/2007 - "We aimed to investigate the rate of kernicterus, and physical and laboratory examination findings in hyperbilirubinemic infants with glucose-6-phosphate dehydrogenase (G-6-PD) deficiency. "
04/01/2002 - "Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is a commonly occurring enzyme defect that can lead to severe neonatal hyperbilirubinaemia and kernicterus. "
07/01/1998 - "Prevention of kernicterus in full term infants is based on the detection of neonates at risk for developing hyperbilirubinaemia, and can be accomplished with simple tests performed on umbilical cord blood such as blood type, Rh, Coombs' test and glucose-6-phosphate dehydrogenase, in order to detect haemolytic diseases. "
01/01/1988 - "A simplified method for the detection of glucose-6-phosphate dehydrogenase activity in hair follicles that would allow health service workers in the field to determine the carrier status of pregnant women might form the basis for a future kernicterus prevention programme."
08/01/1973 - "An Iraqi Jewish family with a new red cell glucose-6-phosphate dehydrogenase variant (GD-Bagdad) and kernicterus."
|3.||bilirubin glucuronoside glucuronosyltransferaseIBA
12/01/2001 - "Because the liver architecture is not disturbed in CN-1 and partial correction of bilirubin-UDP-glucuronosyltransferase (UGT1A1) activity is expected to be sufficient for protection against kernicterus, cell and gene therapies are being developed using the Gunn rat as an animal model of the disease. "
05/01/1995 - "Crigler-Najjar syndrome type I (CN-I) is a congenital hepatic metabolic deficiency in bilirubin UDP-glucuronosyltransferase activity which leads to profound jaundice and death from kernicterus. "
01/01/1984 - "For example, in Type I Crigler-Najjar syndrome, absence of hepatic bilirubin glucuronyl transferase activity causes severe unconjugated hyperbilirubinemia which invariably leads to death from kernicterus. "
|4.||Phenobarbital (Luminal)FDA Link
09/01/1987 - "After an episode of kernicterus in childhood he was treated with phenobarbital with a resultant marked decrease in his serum bilirubin concentration. "
01/01/1969 - "Effect of sodium phenobarbital on bilirubin metabolism in an infant with congenital, nonhemolytic, unconjugated hyperbilirubinemia, and kernicterus."
01/01/1969 - "Sodium phenobarbital and various hormones, compounds capable of hepatic enzyme induction, were given to an infant boy with congenital, nonhemolytic, unconjugated, hyperbilirubinemia and severe kernicterus for prolonged periods between the ages of 2 and 25 months to determine their effect on serum bilirubin concentrations. "
01/01/2003 - "1. To determine the efficacy of metalloporphyrins in reducing bilirubin levels, reducing the need for phototherapy or exchange transfusion and reducing the incidence of bilirubin encephalopathy in neonates with unconjugated hyperbilirubinemia when compared to placebo, phototherapy or exchange transfusion. "
08/01/1982 - "When kernicterus was induced in jaundiced rats by an injection of bucolome, the NSE level in cerebrospinal fluid was elevated up to more than 30-fold the control, together with a significantly higher level of alpha gamma form in blood plasma are helpful in detecting neuronal damage in the central nervous system."
10/01/2008 - "The purpose of this study was to evaluate the diagnostic value of conventional magnetic resonance imaging (MRI), proton magnetic resonance spectroscopy ((1)H-MRS), and diffusion-weighted imaging (DWI) for neonatal bilirubin encephalopathy. "
02/01/2012 - "Proton magnetic resonance spectroscopic images in preterm infants with bilirubin encephalopathy."
06/01/2005 - "The purpose of this study was to retrospectively analyze proton ((1)H) MR spectroscopic data to see if the MR spectroscopic profiles of infants with hyperbilirubinemia and symptoms of kernicterus provide new insights into the pathophysiology of bilirubin neurotoxicity. "
|8.||Carrier Proteins (Binding Protein)IBA
08/01/1986 - "Experimental bilirubin encephalopathy: importance of total bilirubin, protein binding, and blood-brain barrier."
11/01/1995 - "This paper reviews previously published studies that were instrumental in identifying the role of hypoxia, acidosis, hemolytic disease, intracranial hemorrhage and protein binding in bilirubin encephalopathy and identifies two key variables which contribute to bilirubin flux-free bilirubin concentration and time. "
01/01/1993 - "A study was made of the effect of berberine, the major ingredient of the Chinese herb huanglian (coptis chinensis) reported to pose some risk for kernicterus among jaundiced newborn Chinese infants, on the protein binding of bilirubin, using the peroxidase kinetic method. "
|9.||Sulfisoxazole (Sulfafurazole)FDA Link
04/01/2014 - "Previous clinical studies with sulfisoxazole have demonstrated occurrence of kernicterus in neonates. "
02/01/1989 - "At therapeutic serum levels, mephenamate and LMOX may possess the potential for displacing bilirubin from albumin and increasing the risk of bilirubin encephalopathy, in a manner similar to sulfisoxazole."
03/01/2004 - "Ibuprofen interferes with bilirubin-albumin binding and increases the unbound bilirubin in pooled newborn plasma to levels similar to those produced by sulfisoxazole, a drug that causes kernicterus in premature newborn infants."
10/01/2000 - "Sulfisoxazole at 12 mg/dL and benzoate at 10 mmol/L are associated with kernicterus at total bilirubins near 12 and 10 mg/dL, respectively. "
10/01/2000 - "To determine the unbound bilirubin concentration (UBC) associated with kernicterus with the use of clinical data from clusters of kernicterus after sulfisoxazole and benzyl alcohol administration. "
03/01/1979 - "Acute bilirubin encephalopathy induced with sulfadimethoxine in Gunn rats."
01/01/1971 - "Electron microscopic observations on acute bilirubin encephalopathy in Gunn rats induced by sulfadimethoxine."
11/01/2012 - "At peak postnatal hyperbilirubinemia, j/j Gunn rat pups were dosed with sulfadimethoxine to induce bilirubin encephalopathy. "
06/15/2009 - "Spontaneously jaundiced Gunn rats exposed to sulfadimethoxine develop bilirubin encephalopathy (kernicterus) with hearing loss and dystonia, closely resembling the human syndrome. "
02/01/2009 - "The objective of this study was to compare calculated central nervous system (CNS) B(F) levels in Gunn rat pups during (i) peak postnatal hyperbilirubinemia and (ii) sulfadimethoxine-induced acute bilirubin encephalopathy (ABE) previously reported from our laboratory with those predicted in human neonates with peak total serum bilirubin (TSB) levels of 35 mg per 100 ml (599 micromol l(-1)), a clinical cohort that often evidence moderate-to-severe adverse post-icteric neurodevelopmental sequelae. "
|1.||Phototherapy (Light Therapy)
04/01/2013 - "Universal access to Rh immunoprophylaxis, coordinated perinatal-neonatal care, and effective phototherapy has virtually eliminated the risk of kernicterus in many countries. "
01/01/1982 - "It is concluded that infusion of both HSA preparations during phototherapy provides an immediate protection against bilirubin encephalopathy. "
06/01/2011 - "In addition, effective phototherapy is crucial if we are to make kernicterus a "never-event." Finally it is essential that we conduct appropriate population-based studies to accurately elucidate the magnitude of the problem. "
02/01/2009 - "Including only cases with a procedure code for phototherapy or exchange transfusion resulted in 2.7 per 100000 diagnosed with kernicterus over the entire study period. "
06/01/1994 - "Kernicterus in premature infants: current prevalence and relationship to NICHD Phototherapy Study exchange criteria."
08/01/1996 - "In 2 patients, ages 22 and 23 years, early signs of bilirubin encephalopathy could be reversed, in 1 by prompt medical intervention followed by liver transplantation and in the other by prompt liver transplantation. "
03/15/2005 - "Current therapy relies on phototherapy to prevent kernicterus, but liver transplantation presently is the only permanent cure. "
05/01/2006 - "While patients await liver transplantation for CND, hyperbilirubinemia can be managed safely and effectively to prevent kernicterus. "
11/01/2000 - "Bilirubin adsorption therapy and subsequent liver transplantation cured severe bilirubin encephalopathy in a long-term survival patient with Crigler-Najjar disease type I."
11/01/2000 - "To save patients with the acute onset type of bilirubin encephalopathy, sufficient bilirubin adsorption followed by liver transplantation appears to be the most recommended therapeutic approach."
|4.||Intrauterine Blood Transfusion
01/01/2013 - ", none of the review's secondary outcomes were reported in the included study: the need for increased surveillance for suspected fetal blood sampling and fetal transfusions in subsequent pregnancies, neonatal morbidity such as neonatal anaemia, jaundice, bilirubin encephalopathy, erythroblastosis, prematurity, hypoglycaemia (low blood sugar) in subsequent pregnancies, maternal adverse events of anti-D administration including anaphylactic reaction and blood-borne infections.The"
|5.||Drug Therapy (Chemotherapy)
11/02/1973 - "[Letter: Drug therapy of newborns and bilirubin encephalopathy. "
10/19/1973 - "[Drug therapy in newborn infants and bilirubin encephalopathy]."
08/31/1973 - "[Drug therapy in newborn infants and bilirubin encephalopathy]."
06/22/1973 - "[Does drug therapy increase the dangers of bilirubin encephalopathy during the newborn period?]."
06/01/1965 - "KERNICTERUS ASSOCIATED WITH PERINATAL ASPHYXIA AND DRUG THERAPY."