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Background Neonatal acute liver failure (NALF) is a rare and life-threatening condition. It causes bilirubin to accumulate to a dangerous level in the body, causing permanent damage to vital organs such as the brain and lungs. In many cases, the etiology of NALF remains unknown. Case presentation We described a case of an 8-day-old baby girl who presented with poor oral intake, lethargy, and jaundice. Her clinical condition rapidly deteriorated with progression to multi-organ failure, and despite intensive resuscitation efforts, she expired. At autopsy, the most significant findings were liver necrosis, yellow hyaline membrane deposition in the lungs, and bilirubin deposition in the brain (kernicterus). Conclusions NALF is a rare and potentially fatal condition necessitating prompt recognition and disease-specific treatment approaches. Toxic accumulation of bilirubin in the lungs can lead to hypoxia and precipitate further ischemic injury to the liver.

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Background: Kernicterus or bilirubin encephalopathy is a condition rarely observed in animal characterized by a yellowish discoloration of the central nervous system. It is a potentially fatal condition due to bilirubin neurotoxic effects caused by the increase of non-conjugated bilirubin pigment, which passes blood brain barrier and has been attributed to an imbalance between albumin and bilirubin levels. Intracellular bilirubin is toxic for cells and can cause decrease in protein synthesis, specially albumin, depression of cell respiration and cellular death. This paper describes kernicterus in a 2-year-old Great Dane female dog.Case: Clinically, the animal showed apathy, lethargy, weight loss and jaundice, which progressed to vomiting and neurological signs characterized by loss of consciousness and eventually coma. Blood parameters were within normal range, except for high levels of alanine aminotransferase (523 U/L), suggesting a liver lesion. The animal was submitted to euthanasia due to the poor prognosis, and at post-mortem examination it showed dehydration and severe jaundice, especially oral, vaginal and ocular mucosas, subcutaneous tissue and blood vessels intima surface. The liver had an accentuated lobular pattern, and the stomach mucosa was reddened. Multiple petechiae were observed in the epicardium, as well as icterus in the blood vessels of the heart. Furthermore, the brain and cerebellum cortex, thalamic region and nuclei region of brainstem showed extensive icteric areas. Microscopically, the liver presented a mononuclear portal hepatitis, centrilobular necrosis and presence of yellowish pigments. The brain had neuronal necrosis, mild vacuolization of the white matter, perineuronal edema and Alzheimer type II astrocytes, while cerebellum showed Purkinje cells necrosis. Hepatic cooper measurement was within range values, and direct imunofluorescence for the detection of Leptospira sp. was negative.[...](AU)

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Background: Kernicterus or bilirubin encephalopathy is a condition rarely observed in animal characterized by a yellowish discoloration of the central nervous system. It is a potentially fatal condition due to bilirubin neurotoxic effects caused by the increase of non-conjugated bilirubin pigment, which passes blood brain barrier and has been attributed to an imbalance between albumin and bilirubin levels. Intracellular bilirubin is toxic for cells and can cause decrease in protein synthesis, specially albumin, depression of cell respiration and cellular death. This paper describes kernicterus in a 2-year-old Great Dane female dog.Case: Clinically, the animal showed apathy, lethargy, weight loss and jaundice, which progressed to vomiting and neurological signs characterized by loss of consciousness and eventually coma. Blood parameters were within normal range, except for high levels of alanine aminotransferase (523 U/L), suggesting a liver lesion. The animal was submitted to euthanasia due to the poor prognosis, and at post-mortem examination it showed dehydration and severe jaundice, especially oral, vaginal and ocular mucosas, subcutaneous tissue and blood vessels intima surface. The liver had an accentuated lobular pattern, and the stomach mucosa was reddened. Multiple petechiae were observed in the epicardium, as well as icterus in the blood vessels of the heart. Furthermore, the brain and cerebellum cortex, thalamic region and nuclei region of brainstem showed extensive icteric areas. Microscopically, the liver presented a mononuclear portal hepatitis, centrilobular necrosis and presence of yellowish pigments. The brain had neuronal necrosis, mild vacuolization of the white matter, perineuronal edema and Alzheimer type II astrocytes, while cerebellum showed Purkinje cells necrosis. Hepatic cooper measurement was within range values, and direct imunofluorescence for the detection of Leptospira sp. was negative.[...]

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Infants with chronic bilirubin encephalopathy often demonstrate abnormal bilateral, symmetric, high-signal intensity on T2-weighted magnetic resonance imaging of the globus pallidus and subthalamic nucleus, consistent with the neuropathology of kernicterus. Early magnetic resonance imaging of at-risk infants, while frequently showing increased T1-signal in these regions, may give false-positive findings due to the presence of myelin in these structures. Advanced magnetic resonance imaging including diffusion-weighted imaging, magnetic resonance spectroscopy, and diffusion tensor imaging with tractography may shed new insights into the pathogenesis of bilirubin-induced brain injury and the neural basis of long-term disability in infants and children with chronic bilirubin encephalopathy.

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A 6-day-old female newborn, readmitted for extreme hyperbilirubinemia with bilirubin encephalopathy, died despite 2 double-volume exchange transfusions. On autopsy examination the basal ganglia and hippocampus were selectively stained deep yellow. The infant was heterozygous for both the glucose-6-phosphate dehydrogenase Mediterranean mutation and for the (TA)(6)/(TA)(7) promoter polymorphism for the gene encoding the bilirubin conjugating enzyme uridine diphosphate-glucuronosyltransferase 1A1 (UGT1A1*28, associated with Gilbert syndrome). No additional mutations of the UGT1A1 were detected. Seemingly innocuous, heterozygotic mutations may interact synergistically to result in serious and even fatal outcomes.

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None of experimental models used to study the toxic effect of unconjugated bilirubin brain accumulation, reproduce the conditions in which the hyperbilirubinemia is a consequence of a hemolytic process, i.e. when important amounts of bilirubin and iron are released. The aim was to develop an animal model to determine the role of bilirubin and iron, in the encephalopathy secondary to a hemolytic disease. Male Wistar rats 7 days old (n=30) were treated with phenylhydrazine as hemolytic at 75 mg/kg body weight intraperitoneally for 2 days and euthanized 24 h after the last dose. Hemoglobin, hematocrit, serum and brain bilirubin, serum iron and lipoperoxidation products, as well as neuronal damage and iron positive staining were evaluated and compared among treated and untreated (n=10) animals. The animals with induced hemolysis showed significant reduction in hemoglobin and hematocrit, increased concentration of total and conjugated bilirubin, as well as of serum iron and lipid peroxidation products. The neuronal damage in treated animals included the presence of altered neurons spread out among normal cells, as well as of iron-staining positive cells. With the use of appropriated pharmacological procedures, the characteristics of the model can be useful to dissect the participation of both bilirubin and iron, on the bilirubin encephalopathy secondary to hemolysis.

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La ictericia neonatal es un dilema diagnóstico y terapéutico muy común. Las decisiones basadas en mediciones seriadas de bilirrubina sérica se asocian con dolor en el niño, tiempo de espera y ansiedad por los resultados. La bilirrubinometría transcutánea puede ser una alternativa confiable si se demuestra su validez para poblaciones e instituciones particulares. Establecer el grado de correlación entre bilirrubina sérica total (BST) y bilirrubina transcutánea (BTC) en neonatos estratificados por edad gestacional. Estudio de diseño transversal. Cien recién nacidos ictéricos, 50 de término y 50 pretérmino, fueron analizados. A cada niño se le realizó una medición simultánea de BST y BTC. Luego se calcularon los coeficientes de variación de las medias, las diferencias de las medidas, la sensibilidad y el valor predictivo positivo de la BTC, los coeficientes de correlación, la ecuación de la línea de regresión, y la equivalencia de ambas mediciones para predecir el riesgo de hiperbilirrubinemia. El coeficiente de variación de las medias fue similar para ambas mediciones (BST: 3,09 por ciento; BTC: 3,24 por ciento). Las mediciones de BST y BTC fueron diferentes en 95 niños, e iguales en 5 de ellos. En los 95 neonatos con lecturas divergentes, la BTC subestimó la BST en 59 y la sobreestimó en 36, con una diferencia promedio de 1,39 mg/dl (DE 0,58 mg/dl). La sensibilidad de la BTC para identificar una BST >12 mg/dl fue 87 por ciento, con un valor predictivo positivo de 94 por ciento. El coeficiente de correlación de toda la muestra fue 0,88; en los neonatos de término fue 0,92 y en los pretérmino fue 0,84. El 82 por ciento de los neonatos fueron correctamente clasificados por la BTC en los percentiles de riesgo para hiperbilirrubinemia significativa. La precisión de las medidas de BST y BTC es similar en nuestra institución. Aunque hay una correlación aceptable entre ambos métodos de valoración, independientemente de la edad gestacional, la técnica de la BTC...

Newborn jaundice with its potential for producing brain damage remains a continuing problem for pediatricians. Therapeutic decisions based on serial measurements of serum bilirubin (TSB) are time-consuming and associated with stress for the child, parents and practitioners. Transcutaneous bilirubinometry (TCB) may be an alternative method if its reliability is proven for a particular institution and population. To establish the degree of correlation between TSB and TCB in newborn infants classified by gestational age. Cross-sectional study. 100 jaundiced infants, stratified into two groups of 50 preterm and 50 full-term newborns, were analyzed by simultaneous samples of TSB and TCB. Statistical analysis included estimates of differences between the two measurements, coefficients of variation of means, correlation coefficients, sensitivity and positive predictive value of TCB, and accuracy of TCB to predict significant hyperbilirubinemia. The coefficient of variation of means was similar for both methods (TSB: 3.09 percent; TCB: 3.24 percent). Measurements of TSB and TCB were different in 95 infants and equivalent in 5. TCB underestimated TSB in 59 measurements and overestimated it in 36 of them, with a mean difference of 1.39 mg/dl (SD 0.58 mg/dl). Sensitivity of TCB to identify a TSB >12 mg/dl was 87 percent, with a positive predictive value of 94 percent. The global correlation coefficient was 0.88 (term infants: 0.92; preterm infants: 0.84). 82 percent of the infants were correctly classified by the TCB within the percentiles of risk for significant hyperbilirubinemia. Precision of measurements of TSB and TCB is similar in our institution. Despite a good correlation between the two methods, regardless the gestational age, TCB tends to underestimate TSB when bilirubin values go beyond 12 mg/dl. The TCB technique can accurately predict the risk of extreme hyperbilirubinemia, so it can be cautiously used prior to dischange to reduce this risk and to avoid...

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The case histories and autopsy reports of 64 neonates who died from kernicterus were reviewed. The most outstanding findings were: high incidence of kernicterus with serum indirect bilirubin less than 15 mg.; different clinical picture of the premature in comparison with term neonate; predisposing factors such as respiratory distress and intrauterine malnutrition were often associated in the premature. Preventive, educational and therapeutic measures were discussed at the end.

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The case history and autopsy findings of an infant with the "bronze baby" syndrome are presented. These findings substantiate that kernicterus occurs in term infants receiving phototherapy for concentrations of serum indirect bilirubin below 20 mg/dl. The findings at autopsy suggest that the photodecomposed pigmented products of bilirubin are unable to pass the blood-brain barrier. The need for establishing the cause of jaundice prior to initiation of phototherapy is stressed.

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