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Vinod K. Bhutani - One of the best experts on this subject based on the ideXlab platform.
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a global need for affordable neonatal jaundice technologies
Seminars in Perinatology, 2011Co-Authors: Tina Marye Slusher, Alvin Zipursky, Vinod K. BhutaniAbstract:Globally, health care providers worldwide recognize that severe neonatal jaundice is a “silent” cause of significant neonatal morbidity and mortality. Untreated neonatal jaundice can lead to death in the neonatal period and to Kernicterus, a major cause of neurologic disability (choreo-athetoid cerebral palsy, deafness, language difficulty) in children who survive this largely preventable neonatal tragedy. Appropriate technologies are urgently needed. These include tools to promote and enhance visual assessment of the degree of jaundice, such as simpler transcutaneous bilirubin measurements and readily available serum bilirubin measurements that could be incorporated into routine treatment and follow-up. Widespread screening for glucose-6-phoshate dehydrogenase deficiency is needed because this is often a major cause of neonatal jaundice and Kernicterus worldwide. Recognition and treatment of Rh hemolytic disease, another known preventable cause of Kernicterus, is critical. 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. However, knowledge alone is not sufficient. If we are to implement these and other programs and technologies to relegate severe neonatal jaundice and its sequelae to the history books, screening and interventions must be low cost and technologically appropriate for low and middle income nations.
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Synopsis report from the pilot USA Kernicterus Registry.
Journal of perinatology : official journal of the California Perinatal Association, 2009Co-Authors: Vinod K. Bhutani, Lois H. JohnsonAbstract:Breakdown in systems for safe newborn health-care delivery accounts for the majority of Kernicterus cases encountered in the United States. Traditional epidemiologic investigations do not track the national incidence of severe hyperbilirubinemia and Kernicterus or recognize its recent surge. Innovative investigative strategies are needed to seek more sensitive surrogates for Kernicterus (often diagnosed late in infancy) and to overcome the limitations of retrospective continuity of adverse neonatal experiences because of severe hyperbilirubinemia. Root cause analysis of a cohort of infants who manifested Kernicterus in the past two decades attests to some of the clinical and health-service barriers encountered by families as they negotiate health care from multiple providers at multiple sites during the first week after birth. Clinicians, health-care organizations, parents, and payors and purchasers of health care were often unaware of the ongoing patterns of care that may have obstructed preventive care. Now, partly based on these analyses, key recommendations have led to clinical usable guidelines for practitioners and have contributed to systems-oriented national guidelines for evidence-based safer management of newborn jaundice.(1) Clinician- and family-oriented tool kits have been made available, based on the report presented in this study, to facilitate effective implementation and thus optimize and institutionalize these guidelines (http://www.cdc.gov/jaundice). An informed partnership of parents and clinicians seems to be the most effective strategy to prevent severe neonatal hyperbilirubinemia and 'near-miss' cases of Kernicterus in the United States.
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Kernicterus in the 21st century: frequently asked questions.
Journal of perinatology : official journal of the California Perinatal Association, 2009Co-Authors: Vinod K. Bhutani, Lois H. JohnsonAbstract:Acute Kernicterus remains a clinical emergency and its delayed management represents an easily preventable neonatal brain injury. Yet, practitioners encounter recurrent questions regarding the risk and timing of bilirubin-related neurotoxicity. These include the following: does bilirubin damage the brain of healthy infants? Is there a re-emergence of Kernicterus in the United States? Was Kernicterus previously prevented in the United States? What was the public health impact of 1994 American Academy of Pediatrics Guidelines? What is the current incidence of Kernicterus and severe neonatal hyperbilirubinemia? What is the estimated risk of Kernicterus in infants with excessive hyperbilirubinemia? Is there a specific bilirubin threshold total serum bilirubin (TSB) value for neurotoxicity? Are there sequelae of severe or prolonged moderate hyperbilirubinemia in the absence of recognized acute bilirubin encephalopathy? Can we define a bilirubin level that is safe in newborns? We address these questions in the context of available data and evidence, and estimate the current risk of chronic Kernicterus is about one in seven in infants with TSB >30 mg per 100 ml (513 micromol l(-1)).
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Hyperbilirubinemia and Kernicterus.
Clinics in perinatology, 2006Co-Authors: Steven M. Shapiro, Vinod K. Bhutani, Lois JohnsonAbstract:This article describes new findings concerning the basic science of bilirubin neurotoxicity, new considerations of the definition of clinical Kernicterus, and new and useful tools to diagnose Kernicterus in older children, and discusses treatments for Kernicterus beyond the newborn period and why proper diagnosis is important.
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Kernicterus: a preventable neonatal brain injury
J Arab Neonatal Forum, 2005Co-Authors: Vinod K. Bhutani, Lois H. JohnsonAbstract:Kernicterus, one of the most easily preventable causes of brain injury from severe neonatal jaundice, has re-emerged in the United States and other nations with well developed healthcare systems as a public and societal health concern. Kernicterus, in its usually recognized form, causes devastating disabilities including athetoid cerebral palsy (CP) and speech and hearing impairment. It represents the severe manifestation of bilirubin induced neurologic dysfunction (BIND) syndrome. This condition not only ranks amongst the highest cost per new case (according to the Center of Disease Control’s Financial Burden of Disability study in 1992), but also results in profound and uncompromising grief for the family and loss to siblings of healthy, talkative playmates. And for the child with Kernicterus (usually remarkably intelligent, but trapped in an uncontrollable body), grief and frustration are enormous. In 2001 national healthcare organizations, including Centers for Disease Control (CDC), the Joint Commission for the Accreditation of Healthcare Organizations (JACHO) and the American Academy of Pediatrics (AAP) issued alerts to all accredited hospitals and public health professionals in the United States and affiliated organizations that all healthy infants are at potential risk of Kernicterus if their newborn jaundice is unmonitored and inadequately treated. Evidentiary analysis of 125 cases, from an informal Pilot Kernicterus Registry, documents that unmonitored or inadequately treated severe hyperbilirubinemia results in Kernicterus in otherwise healthy term and near term infants. On the basis of this empirical evidence, the lapses in care and root causes have been associated with the occurrence of Kernicterus in these infants. The re-emergence of Kernicterus in the United States is the result of interacting phenomena including a) early hospital discharge (before extent of jaundice is known and signs of impending brain damage have appeared); b) lack of adequate concern for the risks of severe jaundice in healthy term and near term newborns; c) an increase in breast feeding without adequate instruction, monitoring and support; d) medical care cost constraints leading to early discharge with loss of supervision; e) paucity of educational materials to enable parents to participate in safeguarding their newborns; and f) limitations within the healthcare systems to provide continuity of care. The current resources for clinical interventions that can drastically and efficiently reduce the increased bilirubin load, intensive phototherapy and exchange transfusions, are available for use in those infants with excessive hyperbilirubinemia. However, these interventions leave a very narrow margin of safety for babies who have rapid or unrecognized increases in their bilirubin load. Because most babies are discharged before the hyperbilirubinemia reaches its peak during the first week of life, preventive and system-based strategies offer a safer, kinder and gentler means to prevent BIND including Kernicterus.
John M. Tilford - One of the best experts on this subject based on the ideXlab platform.
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trends in hospitalizations for neonatal jaundice and Kernicterus in the united states 1988 2005
Pediatrics, 2009Co-Authors: Bryan L. Burke, James M. Robbins, Charlotte A. Hobbs, Clare Nesmith, Mac T Bird, John M. TilfordAbstract:Context and objective Recent reports have raised global concerns about a reemergence of Kernicterus. Accurate information on the incidence of Kernicterus is unavailable because of the rarity of the condition and the lack of a systematic surveillance strategy. We used nationally representative hospital discharge data to evaluate trends in the diagnosis and management of neonatal jaundice and the incidence of Kernicterus in relation to the American Academy of Pediatrics hyperbilirubinemia clinical practice guideline. Patients and methods The data came from the Healthcare Cost and Utilization Project family of databases. The Nationwide Inpatient Sample and the Kids' Inpatient Database were combined to generate trend data for the years 1988-2005. All neonatal discharges with primary or secondary International Classification of Diseases, Ninth Revision, Clinical Modification diagnoses codes for jaundice or Kernicterus occurring within the first 30 days of life were selected with population incidence rates calculated from estimates of term and preterm newborn hospitalizations derived from the Healthcare Cost and Utilization Project data. To increase the reliability of identified Kernicterus hospitalizations, newborns with a diagnosis of Kernicterus and a procedure code for phototherapy or exchange transfusion were included as cases. Results Hospital diagnosis codes for Kernicterus likely included a substantial number of rule-out cases, because approximately 70% did not include a procedure code for phototherapy or exchange transfusion. 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. A diagnosis code for jaundice was recorded for 15.6% of newborns. The diagnosis of jaundice and Kernicterus differed according to race and gender. Rates also were elevated in preterm relative to term infants for both jaundice and Kernicterus. Trends in diagnosis for newborn jaundice were u-shaped, with rates falling in the years before the initial American Academy of Pediatrics guideline (1988-1993) and increasing in the years after publication of the guideline (1997-2005). In contrast, the number of newborn hospitalizations with a diagnosis of Kernicterus generally declined throughout the study period. Most of the decline in hospitalizations for term infants with a diagnosis of Kernicterus occurred before and immediately after publication of the 1994 guideline, going from 5.1 per 100000 in 1988 to 1.5 per 100000 in the years from 1994 to 1996 and has since remained constant. Conclusions Nationally representative hospital data indicate a declining incidence of hospitalizations with a diagnosis of Kernicterus in newborn infants over the period 1988-2005. The decline occurred before and immediately after publication of the 1994 American Academy of Pediatrics guideline on hyperbilirubinemia. Epidemiologic findings were mostly consistent with other studies. Healthcare Cost and Utilization Project data provide an important system for monitoring hospitalizations of uncommon newborn conditions such as Kernicterus.
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Trends in Hospitalizations for Neonatal Jaundice and Kernicterus in the United States, 1988–2005
Pediatrics, 2009Co-Authors: Bryan L. Burke, James M. Robbins, T. Mac Bird, Charlotte A. Hobbs, Clare Nesmith, John M. TilfordAbstract:Context and objective Recent reports have raised global concerns about a reemergence of Kernicterus. Accurate information on the incidence of Kernicterus is unavailable because of the rarity of the condition and the lack of a systematic surveillance strategy. We used nationally representative hospital discharge data to evaluate trends in the diagnosis and management of neonatal jaundice and the incidence of Kernicterus in relation to the American Academy of Pediatrics hyperbilirubinemia clinical practice guideline. Patients and methods The data came from the Healthcare Cost and Utilization Project family of databases. The Nationwide Inpatient Sample and the Kids' Inpatient Database were combined to generate trend data for the years 1988-2005. All neonatal discharges with primary or secondary International Classification of Diseases, Ninth Revision, Clinical Modification diagnoses codes for jaundice or Kernicterus occurring within the first 30 days of life were selected with population incidence rates calculated from estimates of term and preterm newborn hospitalizations derived from the Healthcare Cost and Utilization Project data. To increase the reliability of identified Kernicterus hospitalizations, newborns with a diagnosis of Kernicterus and a procedure code for phototherapy or exchange transfusion were included as cases. Results Hospital diagnosis codes for Kernicterus likely included a substantial number of rule-out cases, because approximately 70% did not include a procedure code for phototherapy or exchange transfusion. 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. A diagnosis code for jaundice was recorded for 15.6% of newborns. The diagnosis of jaundice and Kernicterus differed according to race and gender. Rates also were elevated in preterm relative to term infants for both jaundice and Kernicterus. Trends in diagnosis for newborn jaundice were u-shaped, with rates falling in the years before the initial American Academy of Pediatrics guideline (1988-1993) and increasing in the years after publication of the guideline (1997-2005). In contrast, the number of newborn hospitalizations with a diagnosis of Kernicterus generally declined throughout the study period. Most of the decline in hospitalizations for term infants with a diagnosis of Kernicterus occurred before and immediately after publication of the 1994 guideline, going from 5.1 per 100000 in 1988 to 1.5 per 100000 in the years from 1994 to 1996 and has since remained constant. Conclusions Nationally representative hospital data indicate a declining incidence of hospitalizations with a diagnosis of Kernicterus in newborn infants over the period 1988-2005. The decline occurred before and immediately after publication of the 1994 American Academy of Pediatrics guideline on hyperbilirubinemia. Epidemiologic findings were mostly consistent with other studies. Healthcare Cost and Utilization Project data provide an important system for monitoring hospitalizations of uncommon newborn conditions such as Kernicterus.
Jon F. Watchko - One of the best experts on this subject based on the ideXlab platform.
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Combating the Hidden Health Disparity of Kernicterus in Black Infants: A Review.
JAMA pediatrics, 2020Co-Authors: Francesca Okolie, Jeannette E. South-paul, Jon F. WatchkoAbstract:Importance Kernicterus is a devastating, permanently disabling neurologic condition resulting from bilirubin neurotoxicity. Black neonates account for more than 25% of Kernicterus cases in the US, despite making up only approximately 14% of all births. This is a largely overlooked health disparity. Observations The black Kernicterus health disparity exists despite a lower overall incidence of clinically significant hyperbilirubinemia among black neonates, a paradox recently explained by a previously unrecognized risk for hazardous hyperbilirubinemia. Aligned with national and global health initiatives to reduce or eliminate health disparities, this review highlights the multiple biologic and nonbiologic factors contributing to Kernicterus risk in black infants and approaches to reduce this health disparity. This includes both parent-level and clinician-level Kernicterus prevention strategies, with an emphasis on improving parental health literacy on neonatal jaundice and acute bilirubin encephalopathy and clinician awareness of the key factors that contribute to hazardous hyperbilirubinemia risk in this vulnerable group. Parent-level prevention strategies include efforts to improve their health literacy on neonatal jaundice and acute bilirubin encephalopathy and empower care seeking for jaundice. Clinician-level prevention strategies include efforts to eliminate community and institutional barriers that impede access to care, heighten clinician awareness of the factors that contribute to Kernicterus risk in this vulnerable patient group, and strengthen newborn hyperbilirubinemia management and bilirubin surveillance. Conclusions and Relevance There are multiple opportunities for intervention to reduce black Kernicterus risk. Although Kernicterus is a rare disorder, the incidence among black infants is not a trivial matter nor are efforts to prevent Kernicterus. While the multiple interacting biologic and nonbiologic contributors to increased Kernicterus risk among black infants pose a considerable challenge to clinicians, there are opportunities for intervention to reduce this risk and health disparity. Continued study is imperative to understand the current scope of Kernicterus and its occurrence in black neonates.
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Maternal Empowerment - An Underutilized Strategy to Prevent Kernicterus?
Current pediatric reviews, 2017Co-Authors: Richard Wennberg, Jon F. Watchko, Steven M. ShapiroAbstract:BACKGROUND Kernicterus is a common cause of death and morbidity in many Low- Middle-income Countries (LMICs) and still occurs in affluent nations. In either case, the immediate cause is delayed treatment of severe hyperbilirubinemia. In the West, a provider driven "systems approach" has been widely adopted to identify babies at risk prior to discharge from birthing centers with follow up monitoring based on the serum bilirubin level at time of discharge. The situation is more complicated in regions of the world where Kernicterus is endemic, especially in LMICs where Glucose-6-phosphate Dehydrogenase Deficiency (G6PDd) is common and the system of jaundice management is often fragmented. OBJECTIVE To examine reasons for errors in jaundice management leading to Kernicterus and the potential beneficial role of enlisting more parental participation in management decisions. METHOD We searched world literature related to pitfalls in jaundice management including deficiencies in providers' and parents' knowledge and behavior. Perspectives from mothers of children with Kernicterus supplemented the literature review. RESULT System failures contributing to Kernicterus in affluent countries include a lack of follow up planning, bad advice by providers, and a delay in care seeking by parents. In many LMICs, the majority of births occur at home with unskilled attendants. Traditional practices potentiate hemolysis in G6PDd babies. The danger of severe jaundice is frequently underestimated both by parents and care providers, and cultural and economic barriers as well as ineffective therapies delay care seeking. The failure to provide parents information about identifying severe jaundice and knowledge about the risks and treatment of hyperbilirubinemia has contributed to delayed treatment in both affluent and low-middle-income countries. A recent non-randomized clinical trial, supports teaching all parents skills to monitor jaundice, signs of early neurotoxicity, the importance of breast feeding, avoidance of ineffective or dangerous practices, and when/where to seek help. CONCLUSION Empowering parents allow them to participate more fully in care decisions and to confront obstacles to care when provider services fail.
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the neurological sequelae of neonatal hyperbilirubinemia definitions diagnosis and treatment of the Kernicterus spectrum disorders ksds
Current Pediatric Reviews, 2017Co-Authors: Jeanbaptiste Le Pichon, Jon F. Watchko, Sean M Riordan, Steven M. ShapiroAbstract:Background Despite its lengthy history, the study of 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. For example, the term Bilirubin-induced Neurological Dysfunction (BIND) has been used to refer to all neurological sequelae caused by exposure to high levels of bilirubin, to only mild neurological sequelae, or to scoring systems that quantitate the progressive stages of Acute Bilirubin Encephalopathy (ABE). Objective We seek to clarify and simplify terminology by introducing, defining, and proposing new terms and diagnostic criteria for Kernicterus. Methods We propose a systematic nomenclature based on pathophysiological and clinical criteria, presenting a logical argument for each term. Acknowledging observations that Kernicterus is symptomatically broad and diverse, we propose the use of the overarching term Kernicterus Spectrum Disorders (KSDs) to encompass all the neurological sequelae of bilirubin neurotoxicity including Acute Bilirubin Neurotoxicity (ABE). We further suggest subclassification of KSDs based on the principal disabling features of Kernicterus (motor, auditory). Finally, we suggest the term subtle KSD to designate a child with a history of significant bilirubin neurotoxicity with mild or subtle developmental delays. Results and conclusion We conclude with a brief description of the limited treatments currently available for KSD, thereby underscoring the importance of further research. We believe that adopting a systematic nomenclature for the spectrum of clinical consequences of hyperbilirubinemia will help unify the field and promote more effective research in both prevention and treatment of KSDs.
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Refractory Causes of Kernicterus in Developed Countries: Can We Eradicate G6PD Deficiency Triggered and Low-Bilirubin Kernicterus?
Current pediatric reviews, 2017Co-Authors: Jon F. WatchkoAbstract:BACKGROUND Glucose-6-phosphate dehydrogenase (G6PD) deficiency triggered and low-bilirubin Kernicterus persist despite current prevention strategies. OBJECTIVE Review efforts to eradicate bilirubin induced brain injury in these two conditions including novel approaches to risk assessment and hyperbilirubinemia evaluation. RESULT AND CONCLUSION In the case of G6PD deficiency, a heightened awareness of populations at risk and an expanded Kernicterus prevention strategy focused on intensified parental engagement, education and counselling on neonatal jaundice is needed. In the case of low-bilirubin Kernicterus, a renewed focus on identifying infants with hypoalbuminemia and implementation of hyperbilirubinemia treatment thresholds based on the bilirubin/albumin ratio is needed. Bilirubin binding panels when commercially available will prove valuable.
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bilirubin induced apoptosis in vitro insights for Kernicterus commentary on the article by hanko et al on page 179
Pediatric Research, 2005Co-Authors: Jon F. WatchkoAbstract:Bilirubin Induced Apoptosis In Vitro : Insights for Kernicterus: Commentary on the article by Hanko et al. on page 179
Steven M. Shapiro - One of the best experts on this subject based on the ideXlab platform.
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Maternal Empowerment - An Underutilized Strategy to Prevent Kernicterus?
Current pediatric reviews, 2017Co-Authors: Richard Wennberg, Jon F. Watchko, Steven M. ShapiroAbstract:BACKGROUND Kernicterus is a common cause of death and morbidity in many Low- Middle-income Countries (LMICs) and still occurs in affluent nations. In either case, the immediate cause is delayed treatment of severe hyperbilirubinemia. In the West, a provider driven "systems approach" has been widely adopted to identify babies at risk prior to discharge from birthing centers with follow up monitoring based on the serum bilirubin level at time of discharge. The situation is more complicated in regions of the world where Kernicterus is endemic, especially in LMICs where Glucose-6-phosphate Dehydrogenase Deficiency (G6PDd) is common and the system of jaundice management is often fragmented. OBJECTIVE To examine reasons for errors in jaundice management leading to Kernicterus and the potential beneficial role of enlisting more parental participation in management decisions. METHOD We searched world literature related to pitfalls in jaundice management including deficiencies in providers' and parents' knowledge and behavior. Perspectives from mothers of children with Kernicterus supplemented the literature review. RESULT System failures contributing to Kernicterus in affluent countries include a lack of follow up planning, bad advice by providers, and a delay in care seeking by parents. In many LMICs, the majority of births occur at home with unskilled attendants. Traditional practices potentiate hemolysis in G6PDd babies. The danger of severe jaundice is frequently underestimated both by parents and care providers, and cultural and economic barriers as well as ineffective therapies delay care seeking. The failure to provide parents information about identifying severe jaundice and knowledge about the risks and treatment of hyperbilirubinemia has contributed to delayed treatment in both affluent and low-middle-income countries. A recent non-randomized clinical trial, supports teaching all parents skills to monitor jaundice, signs of early neurotoxicity, the importance of breast feeding, avoidance of ineffective or dangerous practices, and when/where to seek help. CONCLUSION Empowering parents allow them to participate more fully in care decisions and to confront obstacles to care when provider services fail.
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the neurological sequelae of neonatal hyperbilirubinemia definitions diagnosis and treatment of the Kernicterus spectrum disorders ksds
Current Pediatric Reviews, 2017Co-Authors: Jeanbaptiste Le Pichon, Jon F. Watchko, Sean M Riordan, Steven M. ShapiroAbstract:Background Despite its lengthy history, the study of 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. For example, the term Bilirubin-induced Neurological Dysfunction (BIND) has been used to refer to all neurological sequelae caused by exposure to high levels of bilirubin, to only mild neurological sequelae, or to scoring systems that quantitate the progressive stages of Acute Bilirubin Encephalopathy (ABE). Objective We seek to clarify and simplify terminology by introducing, defining, and proposing new terms and diagnostic criteria for Kernicterus. Methods We propose a systematic nomenclature based on pathophysiological and clinical criteria, presenting a logical argument for each term. Acknowledging observations that Kernicterus is symptomatically broad and diverse, we propose the use of the overarching term Kernicterus Spectrum Disorders (KSDs) to encompass all the neurological sequelae of bilirubin neurotoxicity including Acute Bilirubin Neurotoxicity (ABE). We further suggest subclassification of KSDs based on the principal disabling features of Kernicterus (motor, auditory). Finally, we suggest the term subtle KSD to designate a child with a history of significant bilirubin neurotoxicity with mild or subtle developmental delays. Results and conclusion We conclude with a brief description of the limited treatments currently available for KSD, thereby underscoring the importance of further research. We believe that adopting a systematic nomenclature for the spectrum of clinical consequences of hyperbilirubinemia will help unify the field and promote more effective research in both prevention and treatment of KSDs.
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Hyperbilirubinemia and Kernicterus.
Clinics in perinatology, 2006Co-Authors: Steven M. Shapiro, Vinod K. Bhutani, Lois JohnsonAbstract:This article describes new findings concerning the basic science of bilirubin neurotoxicity, new considerations of the definition of clinical Kernicterus, and new and useful tools to diagnose Kernicterus in older children, and discusses treatments for Kernicterus beyond the newborn period and why proper diagnosis is important.
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Definition of the clinical spectrum of Kernicterus and bilirubin-induced neurologic dysfunction (BIND).
Journal of perinatology : official journal of the California Perinatal Association, 2004Co-Authors: Steven M. ShapiroAbstract:Kernicterus, currently used to describe both the neuropathology of bilirubin-induced brain injury and its associated clinical findings, is a complex syndrome. The neurobiology of Kernicterus, including the determinants and mechanisms of neuronal injury, is discussed along with traditional and evolving definitions ranging from classical Kernicterus with athetoid cerebral palsy, impaired upward gaze and deafness, to isolated conditions, for example, auditory neuropathy or dys-synchrony (AN/AD), and subtle bilirubin-induced neurological dysfunction (BIND). The clinical expression of BIND varies with location, severity, and time of assessment, influenced by the amount, duration and developmental age of exposure to excessive free bilirubin. Although total serum bilirubin (TSB) is important, Kernicterus cannot be defined based solely on TSB. For study purposes Kernicterus may be defined in term and near-term infants with TSB > or = 20 mg/dl using abnormal muscle tone on examination, auditory testing diagnostic of AN/AD, and magnetic resonance imaging showing bilateral lesions of globus pallidus+/-subthalamic nucleus.
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Kernicterus in sick and preterm infants 1999 2002 a need for an effective preventive approach
Seminars in Perinatology, 2004Co-Authors: Vinod K. Bhutani, Lois Johnson, Steven M. ShapiroAbstract:Kernicterus in sick and preterm infants is a rarity. Universal availability of phototherapy and concerted clinical efforts to identify, effectively manage and establish clinical guidelines have been instrumental in preventing Kernicterus in US intensive care nurseries. However, in sick and preterm infants the absence of precise data on prevalence of bilirubin induced neurologic injury, the lack of proven predictive indices and the absence of evidence-based studies that clearly demonstrate the actual risk of Kernicterus. These leave questions regarding the basis for clinical strategies and recommendations for the management of neonatal jaundice in this select population. This article reviews 6 preterm infants selected from the Pilot Kernicterus Registry who had recovered from life-threatening neonatal illnesses, briefly discusses current indices used to ascertain risk, and offers an initial bilirubin level based identification of infants while future directions and studies are conducted to supplement our presently incomplete knowledge for safer clinical practice.
Lois Johnson - One of the best experts on this subject based on the ideXlab platform.
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Hyperbilirubinemia and Kernicterus.
Clinics in perinatology, 2006Co-Authors: Steven M. Shapiro, Vinod K. Bhutani, Lois JohnsonAbstract:This article describes new findings concerning the basic science of bilirubin neurotoxicity, new considerations of the definition of clinical Kernicterus, and new and useful tools to diagnose Kernicterus in older children, and discusses treatments for Kernicterus beyond the newborn period and why proper diagnosis is important.
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Kernicterus in sick and preterm infants 1999 2002 a need for an effective preventive approach
Seminars in Perinatology, 2004Co-Authors: Vinod K. Bhutani, Lois Johnson, Steven M. ShapiroAbstract:Kernicterus in sick and preterm infants is a rarity. Universal availability of phototherapy and concerted clinical efforts to identify, effectively manage and establish clinical guidelines have been instrumental in preventing Kernicterus in US intensive care nurseries. However, in sick and preterm infants the absence of precise data on prevalence of bilirubin induced neurologic injury, the lack of proven predictive indices and the absence of evidence-based studies that clearly demonstrate the actual risk of Kernicterus. These leave questions regarding the basis for clinical strategies and recommendations for the management of neonatal jaundice in this select population. This article reviews 6 preterm infants selected from the Pilot Kernicterus Registry who had recovered from life-threatening neonatal illnesses, briefly discusses current indices used to ascertain risk, and offers an initial bilirubin level based identification of infants while future directions and studies are conducted to supplement our presently incomplete knowledge for safer clinical practice.
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urgent clinical need for accurate and precise bilirubin measurements in the united states to prevent Kernicterus
Clinical Chemistry, 2004Co-Authors: Vinod K. Bhutani, Lois JohnsonAbstract:Kernicterus, a preventable brain injury resulting from severe neonatal jaundice, has reemerged in the US (1)(2)(3). Newborn jaundice, a usually benign condition that typically resolves with supervision and appropriate nutritional intake, can progress to severe hyperbilirubinemia in ∼8–10% of healthy newborn infants. Severe hyperbilirubinemia may need treatment with phototherapy. Some newborns discharged as healthy have developed severe hyperbilirubinemia after discharge and succumbed to serious and often irreversible posticteric sequelae. Kernicterus, as described in neonates, refers to the icteric (yellow) staining of the basal ganglia, specifically the globus pallidus (4). The voluntary Pilot Kernicterus Registry now recognizes a syndrome of bilirubin-induced neurologic dysfunction (BIND), which includes Kernicterus in its most severe acute and chronic forms. Using the Registry eligibility criteria, Johnson et al. (1) have documented the reemergence of Kernicterus in a population of term and near-term “healthy” infants after its near eradication following prevention of Rh sensitization and widespread availability of phototherapy. The common insult in all cases of BIND results from a total serum bilirubin (TSB) concentration that exceeds the infant’s neuroprotective defenses and leads to neuronal injury, primarily in the basal ganglia, central and peripheral auditory pathways, hippocampus, diencephalon, subthalamic nuclei, midbrain, cerebellum and pontine and brain-stem nuclei for oculomotor function and for respiratory, neurohumoral, and electrolyte control. The manifestations of acute bilirubin encephalopathy and chronic kernicteric sequelae may be minimal to severe and occur as various combinations (or possibly, isolated findings) of extrapyramidal disorders, neuromotor abnormalities, sensorineural hearing loss, and visual disability. Although not yet demonstrated, some experts believe that milder and subtler neurologic manifestations of BIND exist. The current reemergence of Kernicterus in babies discharged as healthy from US hospitals represents a crisis of credibility and calls into question our ability to measure TSB with accuracy and precision. There is a need …
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Kernicterus: Lessons for the Future From a Current Tragedy
NeoReviews, 2003Co-Authors: Vinod K. Bhutani, Lois JohnsonAbstract:Kernicterus, a preventable injury to the brain due to severe neonatal jaundice, has re-emerged in the United States as a health and societal concern. In its usually recognized form, Kernicterus causes devastating disabilities, including athetoid cerebral palsy and speech and hearing impairments. In response to disturbing reports of Kernicterus in term and near-term infants discharged as “healthy” from their place of birth, A. K. Brown and colleagues inaugurated a Pilot Kernicterus Registry in May, 1992 at the 10th Annual Kernicterus Symposium. Drs. Lois Johnson and Audrey Brown maintained a confidential manual database of acute and chronic bilirubin encephalopathies for voluntary reporting of cases of Kernicterus (considered by many to be an “underground” disease). Usually referring to the icteric (yellow) staining of the basal ganglia and lesions of the extrapyramidal nervous system, Kernicterus is detected at autopsy. Now, the Pilot Kernicterus Registry has offered formal clinical definitions of bilirubin-induced neurologic dysfunction (BIND) and Kernicterus. (1) Audrey K. Brown, the “grande dame of bilirubin,” helped us to understand bilirubin conjugation and to respect the dangers of excessive total serum bilirubin (TSB) load. She was a leader in the campaign to prevent Kernicterus. Her historical review (see accompanying special article) chronicles the achievements of the researchers of Kernicterus. Her research reminds us of the universality of neonatal bilirubinemia. Sadly, she also chronicled both the fall and rise of Kernicterus. Dr. Brown and other “ancestral” researchers imparted a legacy to maintain vigilance for the perpetual and ominous specter of Kernicterus that can threaten humanity now and for generations to come. The current re-emergence of Kernicterus in babies discharged as healthy from United States hospitals represents a crisis of pediatrician credibility, a societal demand for patient safety, and a disease that needs to be controlled by public health policy. The actual incidence of Kernicterus …
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Newborn jaundice and Kernicterus--health and societal perspectives.
Indian journal of pediatrics, 2003Co-Authors: Vinod K. Bhutani, Lois JohnsonAbstract:Kernicterus, a preventable injury to the brain from severe neonatal jaundice, has re-emerged in the United States as a public and societal health concern. Kernicterus, in its usually recognized form, causes devastating disabilities, including athetoid cerebral palsy and speech and hearing impairment. This condition not only ranks amongst the highest cost per new case (per CDC’s Financial Burden of Disability study, 1992), but also results in profound and uncompromising grief for the family and loss to siblings of healthy, talkative playmates. And for the child with Kernicterus (usually remarkably intelligent, but trapped in an uncontrollable body), grief and frustration are enormous. In 2001 national healthcare organizations, including Centers for Disease Control (CDC), the Joint Commission for the Accreditation of Healthcare Organizations (JACHO) and the American Academy of Pediatrics (AAP) issued alerts to all accredited hospitals and public health professionals in the United States that all healthy infants are at potential risk of Kernicterus if their newborn jaundice is unmonitored and inadequately treated. The re-emergence of Kernicterus in the United States is the result of interacting phenomena including (a) Early hospital discharge (before extent of jaundice is known and signs of impending brain damage have appeared); (b) Lack of adequate concern for the risks of severe jaundice in healthy term and near newborns; (c) An increase in breast feeding; (d) Medical care cost constraints; (e) Paucity of educational materials to enable parents to participate in safeguarding their newborns; and (f) Limitations within in healthcare systems to monitor the outpatient progression of jaundice. A multidisciplinary approach that encompasses both healthcare and societal needs should be evaluated at a national level for practical and easy to implement strategies. An approach that is based on principles of evidence-based medicine, patient-safety and family centeredness is presented in this article. These strategies should also be based on public awareness campaign such that the healthcare providers can attempt to achieve a “Zero Tolerance of Kernicterus” and thereby decrease both childhood disabilities and infant mortality within the community.
