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PubMed2001

Kernicterus in full-term infants--United States, 1994-1998.

Kernicterus is a preventable life-long neurologic syndrome caused by severe and untreated hyperbilirubinemia during the neonatal period. High levels of bilirubin are toxic to the developing newborn. In full-term infants, hyperbilirubinemia symptoms include severe jaundice, lethargy, and poorfeeding. Features of kernicterus may include choreoathetoid cerebral palsy, mental retardation, sensorineural hearing loss, and gaze paresis. Kernicterus is not a reportable condition in the United States, and its prevalence is unknown; however, a pilot registry at a Pennsylvania hospital documented 90 cases in 21 states from 1984 to June 2001 (L. Johnson, Pennsylvania Hospital, Philadelphia, personal communication, 2001). This report summarizes case histories of four full-term, healthy infants who developed kernicterus and underscores that to prevent kernicterus, newborns must be screened and promptly treated for hyperbilirubinemia.

Humans↗
PubMedDate not supplied

1H MR spectroscopic characteristics of kernicterus: a possible metabolic signature.

BACKGROUND AND PURPOSE: MR examination of infants with kernicterus shows abnormal changes in signal intensity in various parts of the brain, including the globus pallidus and subthalamic regions. 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. METHODS: Six patients aged 3 days to 3 weeks, with hyperbilirubinemia and symptoms of kernicterus underwent MR imaging and MR spectroscopy with a single-voxel point-resolved spectroscopic sequence. The voxel of interest was centered in the basal ganglia with CSF excluded. Quantitative analysis was done by using LCModel. Between-subject comparisons were based on metabolite ratios relative to creatine. RESULTS: Ratios of taurine, glutamate and glutamine, and myoinositol relative to creatine were significantly elevated (P < .001), whereas the ratio of choline to creatine was significantly decreased (P < .001) compared with normal values published for this age group. Lactate levels were not significantly elevated. CONCLUSION: Kernicterus has a characteristic signature that is detectable on (1)H-MR spectroscopy. Our results are generally in agreement with what is known about bilirubin pathology, and the finding of increased glutamate and glutamine and decreased choline ratios indicates a possible link between hepatic encephalopathy and kernicterus. This observation may help in elucidating the pathophysiology of bilirubin neurotoxicity.

Female↗
PubMed1994

Kernicterus in premature infants: current prevalence and relationship to NICHD Phototherapy Study exchange criteria.

OBJECTIVE: This study was undertaken to determine the current prevalence of kernicterus in premature neonates and to relate the occurrence of kernicterus to 1) the categorization of the infant as "at risk" by National Institute of Child Health and Human Development (NICHD) Phototherapy Study exchange transfusion criteria, and 2) the clinical management of the infant's hyperbilirubinemia. DESIGN: Retrospective review of postmortem and clinical records. SETTING: Magee-Womens Hospital, a University of Pittsburgh Medical Center affiliated hospital with approximately 10,000 deliveries per year and a Level III Neonatal Intensive Care Unit with about 1400 admissions annually. SUBJECTS: All neonates autopsied between January 1, 1984 and June 30, 1993 who were < 34 weeks gestation and who lived at least 48 hours; a total of 81 infants. RESULTS: Three infants had kernicterus resulting in a prevalence rate of 4%. These cases included: 1) a 33-week newborn with nonimmune hydrops and a peak bilirubin of 26 mg/dl; 2) a 25-week newborn with asphyxia, hyaline membrane disease, grade IV intraventricular hemorrhage, necrotizing enterocolitis, meconium peritonitis, sepsis, prolonged acidosis, and a peak bilirubin of 11.3 mg/dl; and 3) a 24-week newborn with asphyxia, hyaline membrane disease, grade III intraventricular hemorrhage, and a peak serum bilirubin of 18.5 mg/dl. Of the remaining 78 infants who did not have kernicterus, peak bilirubin ranged from 3.6 to 22.5 mg/dl and 56% had bilirubin levels greater than that suggested as a criterion for exchange transfusion by NICHD Phototherapy Study guidelines; yet all but three were managed with phototherapy alone. CONCLUSIONS: We conclude that kernicterus is currently an uncommon event in preterm infants, even when bilirubin levels are allowed to rise above those previously thought to place the premature infant at risk.

Bilirubin↗
PubMed1997

Bilirubin metabolism and kernicterus.

Neonatal jaundice continues to be a common problem. Kernicterus, although rare, continues to be a very real concern in both full-term and preterm infants. The diagnosis of kernicterus requires not only bilirubin staining in a characteristic pattern in the brain but also neuronal damage. With careful pathologic evaluation, kernicterus should be distinguishable from the brain damage associated with asphyxia and hypoxia. Early hospital discharge is a risk factor for the development of kernicterus. Combining the use of traditional phototherapy from above and a fiberoptic blanket from below has improved the effectiveness of phototherapy. Clinical trials with SnMP as an inhibitor of heme oxygenase appear encouraging; no adverse effects were noted, except for mild, occasional photosensitization manifest by erythema in babies receiving phototherapy. One theoretical toxicity of inhibitors of heme oxygenase involves the recent observation that carbon monoxide (CO) is a neurotransmitter in certain regions of the brain, possibly comparable to nitric oxide (NO), and the consequences of such inhibition are unknown. More research is needed to improve our understanding about the entry of bilirubin into the brain, the predilection of bilirubin for certain brain regions, and the cytotoxicity of bilirubin. In the United States, there is currently no generally accepted method to predict hyperbilirubinemia or kernicterus. Brain stem auditory evoked responses and MRI can both be used effectively to monitor the effects of severe hyperbilirubinemia.

Bilirubin↗
PubMed2004

Kernicterus in sick and preterm infants (1999-2002): a need for an effective preventive approach.

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.

Bilirubin↗
PubMed1991

Risk factors associated with kernicterus in the newborn infant: importance of benzyl alcohol exposure.

The prevalence of kernicterus in our neonatal intensive care unit (NICU) decreased from more than 2/1000 live births in 1980 to none in 1984. To clarify predictors of kernicterus, we examined the medical records of infants born during that time who died between 2 and 28 days of age. Infants were divided into three groups: those with kernicterus, all born before June 10, 1982 (n = 29); contemporaneous controls (n = 28); and infants born after June 10, 1982 (n = 32). Benzyl alcohol was not used after June 10, 1982. Kernicteric infants were more likely than contemporaneous controls to have seizures (p less than 0.001). Indices of pulmonary disease, arterial partial pressure of carbon dioxide and end-expiratory pressure on the ventilator were higher after 1982. However, exposure to benzyl alcohol was not different in kernicteric infants and contemporaneous controls, suggesting that benzyl alcohol exposure was not the explanation for the decrease in the prevalence of kernicterus in our NICU.

Analysis of Variance↗
PubMed1984

Value and limitations of bilirubin binding capacity in predicting the development of kernicterus.

The serum bilirubin (SBR) and the bilirubin binding capacity (BBC) were determined on hospital admission in 181 consecutive neonates with neonatal jaundice. Twenty-three babies were less than 2500 g and two were of birth weight less than 2000 g. Fifteen babies were preterm. There were eight cases of kernicterus. Although the serum bilirubin was generally higher in infants with a BBC less than 34 mumol/l, there was no correlation between the bilirubin binding capacity and the presence of kernicterus (r = 0.28, P greater than 0.05). However, when both the serum bilirubin and the bilirubin binding capacity were used, a low value of BBC less than 34 mumol/l increased the risk of kernicterus. Higher values of BBC, on the other hand, did not mean that there was less risk of kernicterus, other factors being equal. This, while the bilirubin binding capacity may provide an additional source of information, its limitations should always be borne in mind.

Bilirubin↗
PubMed1992

Determination of serum unbound bilirubin for prediction of kernicterus in low birthweight infants.

Serum unbound bilirubin concentrations (UBC) and serum total bilirubin concentrations (TBC) were measured serially in 138 low birthweight (LBW) infants treated with phototherapy for non-hemolytic hyperbilirubinemia. We attempted to assign the suitable critical UBC levels for predicting bilirubin encephalopathy into two different birthweight groups: a very low birthweight (VLBW) group (birthweight < 1,500 g) and an LBW group (birthweight between 1,500 g and 2,499 g). Twelve infants were diagnosed as 'at risk' for kernicterus, of whom 11 had signs of acute bilirubin encephalopathy and received exchange transfusion. One VLBW infant had neurological sequelae at a 3 year follow-up, although exchange transfusion was not carried out because of low TBC. Sensitivity and specificity for predicting kernicterus were calculated at different UBC levels between 0.6 microgram/dl and 1.5 micrograms/dl and TBC levels between 8 mg/dl and 26 mg/dl. The receiver-operating characteristic (ROC) curves plotted for UBC as a predictor of kernicterus were clearly shifted up and to the left compared with the curves for TBC in the VLBW and LBW groups. Thus, the UBC measurement may well provide a more rational basis for evaluating the risk of kernicterus in LBW infants. The optimal cut-off points were derived from these curves. In the VLBW group, the sensitivity was 100% and the specificity was 96% for a UBC of 0.8 microgram/dl, and 80% and 64% for a TBC of 11 mg/dl. In the LBW group, the sensitivity was 100% and the specificity was 98% for a UBC of 1.0 microgram/dl and 71% and 78% for a TBC of 16 mg/dl.(ABSTRACT TRUNCATED AT 250 WORDS)

Bilirubin↗
PubMed2005

Bronze baby syndrome and the risk of kernicterus.

AIM: The problem of kernicterus in infants with bronze baby syndrome (BBS) has been reviewed on the basis of cases reported in the literature. In addition, a new case concerning an infant with severe Rh haemolytic disease, who presented with BBS and who has developed neurological manifestations of kernicterus with magnetic resonance images showing basal ganglia abnormalities, is presented. In this patient, the total serum bilirubin (TSB) concentration ranged from 18.0 to 22.8 mg/dl (306 to 388 micromol/l) and the bilirubin/albumin (B/A) ratio was 6.0 (mg/g) (6.8 is the value at which an exchange transfusion should be considered). The case presented is important due to the fact that kernicterus appeared after an exchange transfusion was performed when the TSB level reached 22.8 mg/dl (388 micromol/l) on 6th day of life while the haematocrit was 30%. From this case and from other cases reported in the literature, we must stress that, even if the level at which hyperbilirubinemia poses a threat remains undefined, BBS may constitute an additional risk of developing kernicterus. CONCLUSION: The possible strategies for implementing an approach to the management of hyperbilirubinemia (especially the haemolytic kind) in the presence of BBS may include an exchange transfusion carried out at lower TSB concentration than previously recommended or an early administration of Sn-mesoporphyrin.

Basal Ganglia↗
PubMed1998

Current drug treatment options in neonatal hyperbilirubinaemia and the prevention of kernicterus.

Neonatal jaundice is a frequent problem in neonatology, but the advent of phototherapy which has simplified its treatment, it no longer represents a major concern. Early hospital discharge of neonates has now resulted in a re-emergence of kernicterus. Neonatal jaundice is principally the result of a transient deficiency of bilirubin conjugation, of a partial deficiency of hepatic bilirubin uptake and intracellular transport and of an increased enterohepatic circulation of the pigment. The fact that bilirubin production in the neonate is 2 or more times greater than in the adult per kilogram of bodyweight represents the mainstay of this condition. 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. The daily evaluation of transcutaneous bilirubin measurement gives additional information on the rise of serum bilirubin level, and can help to distinguish physiological from nonphysiological hyperbilirubinaemia. A significant hyperbilirubinaemia is more frequent in infants born before term, and in neonates who do not feed well and lose more than 10% of bodyweight. In preterm infants the typical clinical feature of kernicterus is seen very rarely, and kernicterus is now a very infrequent postmortem observation. Since it is very difficult to distinguish the effects of bilirubin from other potentially toxic factors, it is difficult to give guidelines for the treatment of jaundice in very low birthweight infants other than to keep the serum bilirubin levels to a lower level than in full term infant (e.g. 10 mg/dl lower than in full term babies). The intramuscular administration of a single dose of Sn-mesoporphyrin (6 mumol/kg bodyweight) in healthy term or near-term infants seems to be a promising treatment modality for controlling hyperbilirubinaemia.

Breast Feeding↗
PubMed2006

[Incidence of kernicterus in newborn infants in Denmark].

INTRODUCTION: Kernicterus is an infrequent but serious complication of neonatal hyperbilirubinaemia. The purpose of this investigation was to describe the incidence of the condition in Denmark, its causes and its prevention. A follow-up was done to illustrate the serious consequences of this disease. METHODS: The study included all infants in Denmark born term or near term, with symptoms of classical kernicterus, in the period from 1977 to 2003. RESULTS: From 1977 to 1993, no cases of kernicterus were reported. In the following period, from 1994 to 2002, eight patients were registered. The maximum plasma total bilirubin concentration was 531-745 mol/l. The aetiology was determined in two infants: spherocytosis and galactosaemia. Most likely, ABO blood type immunization was the reason in four cases. In two cases, the aetiology was unknown. Seven infants had symptoms of chronic bilirubin encephalopathy. One child had only minor signs of this condition. Seven were boys, and six infants were of Caucasian descent. Two of the infants died. CONCLUSION: The incidence of kernicterus in Denmark is increasing, as in the rest of the Western world; this after a period of at least 20 years in which no cases were reported. A change in the assessment of the risk and inadequate knowledge of the serious consequences of bilirubin encephalopathy may be explanations. Through information and education of health personnel, it is possible to provide sufficient information to parents, perform screening procedures and commence therapy at the appropriate time. This could possibly contribute to a reduction in the incidence of the disease and its serious consequences.

Denmark↗
PubMed1989

Relationship of benzyl alcohol to kernicterus, intraventricular hemorrhage, and mortality in preterm infants.

Intraventricular hemorrhage and death in preterm neonates has been associated with the use of fluid containing benzyl alcohol, a bacteriostatic agent, to flush intravascular catheters. The hospital and autopsy records of infants admitted to a nursery during the last 18 months that benzyl alcohol was in use (218 patients) were reviewed and compared with those of infants admitted in the first 18 months after benzyl alcohol was withdrawn (232 patients). The volume of flush solution administered to each patient was estimated. Exposure to benzyl alcohol was significantly associated with the development of kernicterus (P less than .005), and intraventricular hemorrhage (P less than .000,000,5). Kernicterus did not develop in any patient after benzyl alcohol was withdrawn. Many patients with kernicterus or intraventricular hemorrhages received small daily volumes of fluid containing benzyl alcohol. Withdrawal of benzyl alcohol from clinical use had no demonstrable effect on mortality. Medications intended for neonatal use should not contain benzyl alcohol. Our data indicate that patients not exposed to benzyl alcohol have a greatly reduced risk of kernicterus. If this finding is confirmed by other investigators, present indications for exchange transfusions in preterm infants with moderate elevations of serum bilirubin should be reconsidered.

Benzyl Alcohol↗
PubMed1982

Unbound bilirubin and kernicterus in low-birth-weight infants.

Unbound bilirubin, bilirubin binding capacity, and bilirubin binding affinity were determined by the horseradish peroxidase method at the time of maximum hyperbilirubinemia and/or before exchange transfusions in 13 preterm infants who later died and had autopsies performed. Five of the 13 infants had kernicterus at autopsy. There were no significant differences in weight, gestational age, highest indirect bilirubin level, albumin concentration, severity of acidosis, use of assisted ventilation, sepsis, or other major clinical complications between the five infants with kernicterus and the eight infants without kernicterus. Compared with the eight nonkernicteric infants, the five kernicteric infants had significantly higher unbound bilirubin concentrations (13 +/- vs 27 +/- 9 nmoles/liter, respectively, P less than .05) and significantly lower bilirubin binding capacity and affinity. The data suggest an association between low bilirubin binding capacity and affinity, increased unbound bilirubin, and kernicterus in preterm infants with severe clinical complications.

Bilirubin↗
PubMed1980

The association of kernicterus with bacterial infection in the newborn.

A total population of 29,395 neonates cared for in the six-year period from 1971 to 1976 was reviewed for evidence of autopsy-proven kernicterus. A total of 327 neonates died and 232 were autopsied. The only cases of kernicterus occurred in four near-term infants with antemortem proven sepsis. All four of these infants weighed more than 2,200 gm and were delivered after gestations of either 36 or 37 weeks. These cases of kernicterus occurred during a period when more aggressive management of hyperbilirubinemia in low-birth-weight infants had apparently eliminated immaturity as a predisposing factor in the development of kernicterus, uncovering bacterial infection as the major remaining etiologic co-factor.

Bacterial Infections↗
PubMed2004

Hyperintense globus pallidus on T1-weighted MR imaging in acute kernicterus: is it common or rare?

Globus pallidus involvement is a well-known magnetic resonance (MR) imaging finding of acute kernicterus. However, it is not clear how early the involvement of globus pallidus occurs and whether or not it is seen in every case. Therefore, we aimed to investigate the globus pallidus involvement in 13 neonates with acute kernicterus by MR imaging. Thirteen neonates who were admitted with jaundice, encephalopathy and indirect hyperbilirubinemia (mean, 37.0 mg/dl) were prospectively evaluated with cranial MR imaging. Pathological signal changes were noted concerning the globus pallidus. Eight of the 13 patients demonstrated bilateral, symmetric increased signal intensity in the globus pallidus on T1-weighted MR imaging. These lesions were not apparent on T2-weighted images. Multiple parenchymal punctuate T1 hyperintense lesions were detected in one patient without globus pallidus involvement. This appearance was consistent with hemorrhage. The MR imaging findings of the other four patients showed no evidence of abnormality. The symmetric involvement of globus pallidus seen as hyperintense on T1-weighted MR imaging is a common and characteristic finding of acute kernicterus.

Acute Disease↗
PubMed1979

Auditory brain stem responses of kernicterus infants.

Auditory brainstem evoked responses (ABRs) and behavioral audiometry in 25 infants with kernicterus were studied to determine the level of the lesion causing their hearing disorders. ABR thresholds, peak latencies of wave I and V, and interwave latency of wave V-I were measured; behavioral audiometric thresholds were determined through conditioned orientation reflex audiometry (COR). Eighty-eight per cent of infants with kernicterus who showed ABR threshold elevation with respect to age-matched normals, were found to show ABR abnormalities associated with peripheral hearing loss, and 84% of these were found to have COR threshold elevation. The ABR abnormalities were threshold elevation of wave V, prolonged latency of wave I and V, and the absence of ABRs. However, no brainstem lesion pattern was found in our cases. Our results suggested that at least some lesions which produce hearing disorders in kernicterus occur in the cochlea or auditory nerve.

Adolescent↗
PubMed2006

Single photon emission computed tomography and serial MRI in preterm infants with kernicterus.

Single photon emission computed tomography was performed in three preterm infants with athetoid cerebral palsy due to kernicterus. No clinical signs and symptoms of kernicterus, or ultrasonographic abnormalities were seen during the neonatal period in any patients. Although MRI during infancy revealed high intensity areas in bilateral globi pallidi in all of them, MRI abnormalities were mild in two of them. On later MRI, subtle high intensity areas in the globi pallidi were recognized in only one of them. Single photon emission computed tomography demonstrated hypoperfusion in the basal ganglia regions in all patients. Regions of interest analyses showed decreased blood flow in the basal ganglia related to the cortical areas. Single photon emission computed tomography will be useful for the diagnosis of kernicterus, whereas MRI abnormalities become less clear beyond infancy.

Basal Ganglia↗
PubMed2006

Hyperbilirubinemia and kernicterus.

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.

Bilirubin↗