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Bilirubin adsorption therapy and subsequent liver transplantation cured severe bilirubin encephalopathy in a long-term survival patient with Crigler-Najjar disease type I.

Crigler-Najjar disease (CN) type I is characterized by persistent unconjugated hyperbilirubinemia from birth. The male patient here was diagnosed with this disease as a neonate and had been treated by phototherapy. At age 16 he suddenly developed generalized convulsions, followed by impaired cognitive function. The serum level of bilirubin was extremely high (total bilirubin: 41.7 mg/dl) and there were no other detectable causes responsible for the metabolic encephalopathy. He received bilirubin adsorption therapy several times, and the bilirubin encephalopathy improved in response to the fall

High resolution melting curve analysis enables rapid and reliable detection of G6PD variants in heterozygous females.

BACKGROUND: Like glucose-6-phosphate dehydrogenase (G6PD) deficient hemizygous males and homozygous females, heterozygous females could also manifest hemolytic crisis, neonatal hyperbilirubinemia or kernicterus upon exposure to oxidative stress induced by certain foods such as fava beans, drugs or infections. Although hemizygous males and homozygous females are easily detected by conventional G6PD enzyme assay method, the heterozygous state could be missed by the conventional methods as the mosaic population of both normal and deficient RBCs circulates in the blood. Thus the present study aime…

Computer-aided diagnosis of neonatal acute bilirubin encephalopathy with multi-modal MRI images and convolutional neural networks.

Acute bilirubin encephalopathy (ABE) is a serious complication of hyperbilirubinemia (HB). Efficiently detecting ABE in non-ABE neonates with HB by magnetic resonance imaging (MRI) techniques remains a great challenge in clinical practice, as both groups may exhibit similar MRI features of T1 hyperintensity in specific brain regions. To this end, a computer-aided (CAD) diagnosis system based on multi-modal MRI images and convolutional neural networks (CNNs) was proposed in this study. A total of 150 patients were included in the study, half of whom were ABE neonates, and the other half were no

Assessing jaundice in infants of 35-week gestation and greater.

PURPOSE OF REVIEW: In 2004, the American Academy of Pediatrics officially recommended universal predischarge risk assessment for severe neonatal hyperbilirubinemia with the goal of minimizing subsequent risk of chronic bilirubin encephalopathy (formerly known as kernicterus). In this article, we review recent research regarding jaundice predischarge risk assessment, current expert recommendations for universal predischarge bilirubin screening, and concerns expressed in the literature regarding these recommendations. RECENT FINDINGS: A group of experts have recently recommended universal predi…

Experience with Sephadex gel filtration in assessing the risk of bilirubin encephalopathy in neonatal jaundice.

Clinical experience with Sephadex gel filtration for evaluation of the bilirubin binding affinity of serum in neonatal jaundice is reported. In serum specimens from 362 jaundiced neonates the results of the test were in most cases in good agreement with the independently determined decision to perform exchange transfusion. In all relevant cases of the 24 with bilirubin encephalopathy the Sephadex test was strongly positive. The test was positive at lower bilirubin levels and bilirubin:albumin molar ratios in preterm as compared with term neonates, especially those in poor clinical conditions.

Пренебрежение двойной ролью билирубина в неврологических болезнях: всеобъемлющее исследование его нейрозащитного и нейротоксичного воздействия.

Нейродегенеративные расстройства характеризуются постепенной потерей нейронов, что приводит к значительным сбоям в работе двигателей и когнитивных систем. Bilirubin — побочный продукт гема желтого цвета, существующий в двух первичных изоформах, а именно в неокончательных и спаренных, в то время как первоначально произведенный неокончательный изомер является липофильным и цитотоксичным по своей природе. На физиологическом уровне билирубин играет важную роль в функционировании мозга, выступая в качестве мощного антиоксиданта, предотвращая окислительное повреждение тканей головного мозга путем ус

Совпадение в аннотации оригинала
Neurodegenerative disorders are characterized by a progressive loss of neurons, causing substantial deficits in motor and cognitive function … orrhagic stroke, traumatic brain injury (TBI), multiple sclerosis (MS), epilepsy, schizophrenia and kernicterus spectrum disorder (KSD), which causes neuronal damage, especially in regions like the basal ganglia and cerebellum, which causes movement abnormalities and cognitive deficits. The aim of this article is to explore the dual role of bilirubin as neuro …

Efficacy of double versus single phototherapy in treatment of neonatal jaundice: a meta-analysis.

To assess the efficacy of double phototherapy in managing neonatal jaundice compared to single phototherapy in infants with different birth … irth weights with the most important finding regarding preterm infants, who are more susceptible to kernicterus.What is Known:• Double phototherapy has shown to be more efficacious than single phototherapy in treating neonatal jaundice.• Double phototherapy efficacy on neonates with different gestational ages and birth weights still remain ambiguous in treati …

Unbound (free) bilirubin: improving the paradigm for evaluating neonatal jaundice.

BACKGROUND: The serum or plasma total bilirubin concentration (B(T)) has long been the standard clinical laboratory test for evaluating neonatal jaundice, despite studies showing that B(T) correlates poorly with acute bilirubin encephalopathy (ABE) and its sequelae including death, classical kernicterus, or bilirubin-induced neurological dysfunction (BIND). The poor correlation between B(T) and ABE is commonly attributed to the confounding effects of comorbidities such as hemolytic diseases, prematurity, asphyxia, or infection. Mounting evidence suggests, however, that B(T) inherently performs…

Metalloporphyrins for treatment of unconjugated hyperbilirubinemia in neonates.

BACKGROUND: Metalloporphyrins are heme analogues that inhibit heme oxygenase, the rate-limiting enzyme in the catabolism of heme to bilirubi … ransfusion in the two studies that described this outcome. None of the studies reported on neonatal kernicterus, death, long-term neurodevelopmental outcomes or iron deficiency anemia. Though a small number of metalloporphyrin-treated as well as control infants developed a photosensitivity rash, the trials were too small to rule out an increase in the risk of …

Comparison of prognosis between hyperbilirubinemic infants with and without hypernatremia.

OBJECTIVES: Hypernatremia may facilitate the diffusion of bilirubin through the blood-brain barrier and increase the risk of bilirubin encephalopathy. This study was conducted to compare the prognosis of jaundice infants with those with jaundice and hypernatremia. METHODS: A total of 615 term infants with idiopathic jaundice with or without hypernatremia were enrolled in this cohort study with 24-months follow-up at Ghaem Hospital, Mashhad, Iran, between 2010 and 2022. An in-house questionnaire including the laboratory evaluation and neonatal characteristics was used as the data collection to

Magnetic resonance spectroscopy and auditory brain-stem response audiometry as predictors of bilirubin-induced neurologic dysfunction in full-term jaundiced neonates.

The purpose of this research was to define the functions of MRS and ABR as predictors of bilirubin-induced neurologic dysfunction (BIND) in full-term neonates who required intervention (phototherapy and/or exchange transfusion). This prospective cohort study was done at the NICU of Tanta University Hospitals over a 2-year duration. Fifty-six full-term neonates with pathological unconjugated hyperbilirubinemia were divided according to MRS and ABR findings into 2 groups: group (1) included 26 cases with mild acute bilirubin encephalopathy (BIND-M score 1-4). Group (2) included 30 cases with neo