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A near-term female neonate with total serum bilirubin levels not exceeding the exchange transfusion level with hypoalbuminaemia developed abnormal movements while recovering from sepsis. Investigations showed an evidence of kernicterus on brain imaging and bilateral auditory neuropathic changes.
- Newborn Screening for G6PD Deficiency- Why is it Important for India? [JOURNAL ARTICLE]
- Indian J Pediatr 2013 Apr 19.
Neonatal screening, the most important preventive public health programme of the 21st century, is implemented in majority of the developed countries. The Asia-Pacific region has a long history-the late Emeritus Professor Wong Hock Boon in 1965 initiated cord blood G6PD screening in Singapore, which virtually eliminated kernicterus. In India currently there is no government funded neonatal screening programme for the masses, but most private hospitals have started screening for disorders which the pediatrician there thinks is relevant in that part of the country. Indian Council of Medical Research has established a task force to look into these and there have been numerous updates from them including an updated website where a pediatrician or a hospital can download information. The authors present this study, which reveals that G6PD in India is a high priority for public screening.
- Raltegravir In Vitro Effect on Bilirubin Binding. [JOURNAL ARTICLE]
- Pediatr Infect Dis J 2013 Mar 6.
Drugs that displace bilirubin from albumin may increase the risk of kernicterus in neonates. We evaluated the effect of raltegravir on bilirubin-albumin binding in pooled neonatal serum using the peroxidase method. Raltegravir had minimal effect on bilirubin-albumin binding at concentrations of 5 and 10 µM, caused a small but statistically significant increase in unbound bilirubin at 100 µM, and caused potentially harmful increases at 500 and 1000 µM. Our data suggest that the effect of raltegravir on neonatal bilirubin binding is unlikely to be clinically significant at typical peak concentrations reached with usual dosing.
- Successful plasmapheresis for acute and severe unconjugated hyperbilirubinemia in a child with crigler najjar type I syndrome. [Journal Article]
- JIMD Rep 2012.:33-6.
Crigler-Najjar syndrome type I (CN-I, MIM #218800) is a rare and severe autosomal disorder. It is caused by deficiency of the liver enzyme responsible for bilirubin elimination, the uridine diphosphate glucuronosyltransferase 1A1 (UGT1A1; EC 22.214.171.124). Biologically, the disease manifests itself with severe and persistent unconjugated hyperbilirubinemia. Kernicterus is a well-known complication of severe unconjugated hyperbilirubinemia in infants and young children, especially in patients with CN-I.Few articles have shown the efficiency of plasmapheresis for extreme hyperbilirubinemia.In this report, we describe the efficiency of plasmapheresis for a rapid control of acute and severe unconjugated hyperbilirubinemia in a 6-year-old CN-I patient who had previously developed kernicterus in the neonatal period. In spite of intensification of phototherapy, the patient developed severe hyperbilirubinemia (up to 830 μmol/l, with bilirubin/albumin ratio at 1.2). With two plasmapheresis procedures, bilirubin serum concentration decreased to 420 μmol/ and bilirubin/albumin ratio to 0.55. Following the acute episode of very severe unconjugated hyperbilirubinemia, the child recovered and neurological examination was unchanged, thus suggesting that plasmapheresis possibly prevented further worsening of kernicterus.
- Should we screen newborns for glucose-6-phosphate dehydrogenase deficiency in the United States? [JOURNAL ARTICLE]
- J Perinatol 2013 Feb 21.
Glucose-6-phosphate dehydrogenase (G6PD) deficiency, a common X-linked enzymopathy can lead to severe hyperbilirubinemia, acute bilirubin encephalopathy and kernicterus in the United States. Neonatal testing for G6PD deficiency is not yet routine and the American Academy of Pediatrics recommends testing only in jaundiced newborns who are receiving phototherapy whose family history, ethnicity, or geographic origin suggest risk for the condition, or for infants whose response to phototherapy is poor. Screening tests for G6PD deficiency are available, are suitable for use in newborns and have been used in birth hospitals. However, US birth hospitals experience is limited and no national consensus has emerged regarding the need for newborn G6PD testing, its effectiveness or the best approach. Our review of current state of G6PD deficiency screening highlights research gaps and informs specific operational challenges to implement universal newborn G6PD testing concurrent to bilirubin screening in the United States.Journal of Perinatology advance online publication, 21 February 2013; doi:10.1038/jp.2013.14.
- Black race is not protective against hazardous bilirubin levels. [Journal Article]
- J Pediatr 2013 May; 162(5):1068-9.
Although black race is considered protective against hyperbilirubinemia, black infants appear at increased risk of kernicterus. We found that although black infants have a lower risk of developing total serum bilirubin levels ≥20 mg/dL than white infants, they appear at greater risk of developing levels ≥30 mg/dL.
- [Symptomatic psychosis--to create new things by taking lessons from the past]. [English Abstract, Journal Article, Review]
- Seishin Shinkeigaku Zasshi 2012; 114(11):1330-6.
Symptomatic psychosis is one of the central problems in research psychosomatic correlational research. My topic forthis lecture is on the research of symptomatic psychosis, which could be called one of the central problems in the field of clinical psychiatry. It is true that if a person is not physically stable, their "brain" and/or "mind" will not be calm. The opposite is equally true. 1. Are delusions of theft symptomatic psychosis In the elderly, there are some physical disease cases which developed into mental illness. For example, delusions of theft were triggered by physical diseases such as knee osteoarthritis, high blood pressure and glaucoma. I think it is possible to position these patients group as having symptomatic psychosis. 2. "Schizophrenia" is symptomatic psychosis We are thinking that there is a group that the biological material (bilirubin) in body fluid by way of hepatic failure did play a role leading to the expression of schizophrenia. Therefore I propose the following hypothesis: "there is a schizophrenia group that is an expression of a very mild kernicterus". This research started from our experiences having patients who had Gilbert's syndrome which has a high indirect (unconjugated) bilirubin. The patients also had schizophrenia. The psychological symptoms of schizophrenia fluctuated depending on the indirect (unconjugated) bilirubin levels. Also, we clarified that the frequency of patients with schizophrenia coexisting with GS is significantly higher than with other psychiatric disorders.
- Hyperbilirubinemia: subcortical mechanisms of cognitive and behavioral dysfunction. [Journal Article]
- Pediatr Neurol 2013 Jan; 48(1):3-13.
Although development of the full syndrome of kernicterus is relatively rare, neonatal jaundice continues to occur frequently. Controversy remains concerning whether or not infants with moderate elevations in bilirubin are at risk for neurodevelopmental disorders in later childhood. Sites of brain pathology associated with bilirubin neurotoxicity are identified and well established. Based on these regions of brain involvement, we apply neuroscientific principles of brain-behavior relationships to predict types of cognitive features that may accompany hyperbilirubinemia. We address a range of neurodevelopmental abnormalities that can arise as a function of elevated neonatal bilirubin levels affecting these brain regions, even in the absence of full kernicterus syndrome. Moreover, we explain the neuropathologic mechanisms that would drive these abnormalities. We thus attempt to establish a blueprint for future investigations of these conditions, to improve neurodevelopmental outcomes.
- Cost-effectiveness analysis of a system-based approach for managing neonatal jaundice and preventing kernicterus in Ontario. [Journal Article]
- Paediatr Child Health 2012 Jan; 17(1):11-6.
To evaluate the incremental cost-effectiveness of a system-based approach for the management of neonatal jaundice and the prevention of kernicterus in term and late-preterm (≥35 weeks) infants, compared with the traditional practice based on visual inspection and selected bilirubin testing.Two hypothetical cohorts of 150,000 term and late-preterm neonates were used to compare the costs and outcomes associated with the use of a system-based or traditional practice approach. Data for the evaluation were obtained from the case costing centre at a large teaching hospital in Ontario, supplemented by data from the literature.The per child cost for the system-based approach cohort was $176, compared with $173 in the traditional practice cohort. The higher cost associated with the system-based cohort reflects increased costs for predischarge screening and treatment and increased postdischarge follow-up visits. These costs are partially offset by reduced costs from fewer emergency room visits, hospital readmissions and kernicterus cases. Compared with the traditional approach, the cost to prevent one kernicterus case using the system-based approach was $570,496, the cost per life year gained was $26,279, and the cost per quality-adjusted life year gained was $65,698.The cost to prevent one kernicterus case using the system-based approach is much lower than previously reported in the literature.
- Use of the extended parallel processing model to evaluate culturally relevant kernicterus messages. [Journal Article, Research Support, U.S. Gov't, P.H.S.]
- J Pediatr Health Care 2013 Jan; 27(1):33-40.
Kernicterus is a serious but easily preventable disease in newborns that is not well-known even by some health care professionals. This study evaluated a parent guide and poster on kernicterus awareness and prevention generated by the Centers for Disease Control and Prevention. The extended parallel processing model was used as a framework for creating the interview protocol and analyzing the results.In-depth interviews were conducted with four parents and six health care personnel of different ethnicities to evaluate the materials. Content for the parent guide and poster was held constant, but photos were varied according to the ethnicity of the baby (white, African American, or Hispanic) and the language in which the interviews were conducted (English and Spanish).The parent guide was evaluated positively, but reactions to the poster were varied. The consensus was that the poster drew more attention than the pocket guide but lacked sufficient information about what jaundice is or how to treat it, while the pocket guide provided information, especially with regard to efficacy. The extended parallel processing model claims that when efficacy is equal to or higher than perceived threat, respondents should engage in recommended responses, which was the general finding from these interviews.Recommendations for improvements of the materials are presented. The focus on different ethnicities in the materials was perceived as unnecessary and potentially counter-productive. Both parents and health care professionals mentioned the lack of information regarding treatment. Providing information on the length and effectiveness of treatment for jaundice and kernicterus might increase efficacy in averting the threat in both conditions.