1,189 results match your criteria Hyperbilirubinemia Unconjugated

Neonatal hemoglobin affects the accuracy of whole blood bilirubin measurement on GEM Premier 4000 blood gas analyzers.

Pract Lab Med 2021 May 27;25:e00231. Epub 2021 Apr 27.

Neonatal-Perinatal Medicine, Kingston General Hospital, 76 Stuart Street, Kingston, ON, Canada.

Objectives: Whole blood bilirubin measured on blood gas analyzers is accepted by physicians in neonatal hyperbilirubinemia management since it requires a small sample volume. The accuracy of bilirubin measurement on blood gas analyzers is instrument dependent and remains controversial.

Design And Methods: Bilirubin in adult and umbilical cord whole blood samples, spiked with an unconjugated bilirubin standard, and non-spiked adult plasma samples was measured on a blood gas analyzer (GEM 4000) and a Core Laboratory Chemistry analyzer (Architect c16000) respectively. Read More

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TaqMan real time PCR for the Detection of the Gilbert's Syndrome Markers UGT1A1*28; UGT1A1*36 and UGT1A1*37.

Mol Biol Rep 2021 Jun 4. Epub 2021 Jun 4.

Department of Public Health and Pediatrics, School of Medicine, University of Turin, Piazza Polonia 94, 10126, Turin, Italy.

Gilbert's syndrome is characterized by mild unconjugated hyperbilirubinemia. The key of this disease is a diminished activity of UDP-glucuronosyltransferase 1A1 (UGT1A1). TA insertion into the TATA box promoter region of the UGT1A1 gene on chromosome 2 is the genetic basis of Gilbert's syndrome (UGT1A1*28). Read More

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Yinzhihuang Oral Liquid Ameliorates Hyperbilirubinemia Induced by  δ-Aminolevulinic Acid and Novobiocin in Neonatal Rats.

Chem Biodivers 2021 Jun 3. Epub 2021 Jun 3.

Tianjin University, School of Chemical Engineering and Technology, State Key Laboratory of Chemical, Tianjin University, 92 Weijin Road, Nankai District, Tianjin, 300072, Tianjin, CHINA.

Yinzhihuang oral liquid (YZH) is a traditional Chinese medicine that has been widely used in Asia to prevent and treat neonatal hyperbilirubinemia, but the published preclinical studies on its anti-hyperbilirubinemia effect are conducted in adult animals, partly due to the lack of preclinical neonatal hyperbilirubinemia animal models. In the present study, we tried six reagents to induce hyperbilirubinemia in neonatal rats, and established two appropriate neonatal hyperbilirubinemia rat models by subcutaneous injection of δ-Aminolevulinic acid (ALA, 200 mg/kg) or novobiocin (NOVO, 200 mg/kg). Oral treatment of YZH (80, 160 and 320 mg/kg) significantly decreased serum conjugated bilirubin levels in ALA-treated neonatal rats and serum unconjugated bilirubin levels in NOVO-treated neonatal rats, respectively. Read More

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UGT1A1 mutation association with increased bilirubin levels and severity of unconjugated hyperbilirubinemia in ABO incompatible newborns of China.

BMC Pediatr 2021 06 1;21(1):259. Epub 2021 Jun 1.

Lab for Respiratory Disease, People's Hospital of Yangjiang, No. 42 Dongshan Road, Yangjiang, 529500, Guangdong Province, People's Republic of China.

Background: Neonatal hyperbilirubinemia causing jaundice is common in East Asian population. Uridine diphosphate glucuronosyltransferase isoenzyme (UGT1A1) glucuronidates bilirubin and converts the toxic form of bilirubin to its nontoxic form.

Method: A retrospective study was conducted to review clinical information of ABO hemolysis neonates (ABO HDN) admitted to the Department of Neonatology, referred for neonatal hyperbilirubinemia, in a large general hospital of southern China from 2011 to 2017. Read More

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Neurotoxicity of Unconjugated Bilirubin in Neonatal Hypoxic-Ischemic Brain Injury .

Front Pediatr 2021 20;9:659477. Epub 2021 Apr 20.

Department of Neurosciences, Psychology, Drug Research and Child Health, University of Florence, Florence, Italy.

The pathophysiology of bilirubin neurotoxicity in course of hypoxic-ischemic encephalopathy (HIE) in term and preterm infants is still poorly understood. We hypothesized that oxidative stress may be a common mechanism that link hyperbilirubinemia and HIE. The objective of the present study was to evaluate whether unconjugated bilirubin (UCB) may enhance the HI brain injury by increasing oxidative stress and to test pioglitazone and allopurinol as new antioxidant therapeutic drugs . Read More

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Occurrence of Recurrent Aphthous Stomatitis (RAS) as a Rare Oral Manifestation in a Patient with Gilbert's Syndrome.

Case Rep Dent 2021 16;2021:6648729. Epub 2021 Apr 16.

Department of Prosthodontics, Faculty of Stomatology, Yerevan State Medical University, Str. Koryun 2, Yerevan 0025, Armenia.

Recurrent aphthous stomatitis is an ulcerative disease of the oral cavity and can occur in isolation or as a manifestation of many systemic diseases. It is a quite common entity and may hence often be overlooked as an isolated lesion. Gilbert's syndrome is a genetic disorder where a deficiency of an enzyme associated with the conjugation of bilirubin results in unconjugated hyperbilirubinemia. Read More

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Life-Long Hyperbilirubinemia Exposure and Bilirubin Priming Prevent Metabolic Damage.

Front Pharmacol 2021 12;12:646953. Epub 2021 Mar 12.

Italian Liver Foundation (FIF), Trieste, Italy.

Unconjugated bilirubin (UCB) is more than the final product of heme catabolism. Mildly elevated systemic bilirubin concentrations, such as in Gilbert syndrome (GS), protect against various oxidative stress-mediated and metabolic diseases, including cardiovascular disease, type 2 diabetes mellitus, metabolic syndrome, cancer, and age-related disease. The Gunn rat is an animal model of hereditary hyperbilirubinemia widely used in assessing the effect of high serum bilirubin concentration in various organs. Read More

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Mitochondrial Function, Fatty Acid Metabolism, and Body Composition in the Hyperbilirubinemic Gunn Rat.

Front Pharmacol 2021 8;12:586715. Epub 2021 Mar 8.

School of Medical Science, Griffith University, Gold Coast, QLD, Australia.

Circulating bilirubin is associated with reduced adiposity in human and animal studies. A possible explanation is provided by data that demonstrates that bilirubin inhibits mitochondrial function and decreases efficient energy production. However, it remains unclear whether hyperbilirubinemic animals have similar perturbed mitochondrial function and whether this is important for regulation of energy homeostasis. Read More

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The Effects of Bilirubin and Lumirubin on Metabolic and Oxidative Stress Markers.

Front Pharmacol 2021 4;12:567001. Epub 2021 Mar 4.

Institute of Medical Biochemistry and Laboratory Diagnostics, Faculty General Hospital and 1 Faculty of Medicine, Charles University, Prague, Czechia.

For severe unconjugated hyperbilirubinemia the gold standard treatment is phototherapy with blue-green light, producing more polar photo-oxidation products, believed to be non-toxic. The aim of the present study was to compare the effects of bilirubin (BR) and lumirubin (LR), the major BR photo-oxidation product, on metabolic and oxidative stress markers. The biological activities of these pigments were investigated on several human and murine cell lines, with the focus on mitochondrial respiration, substrate metabolism, reactive oxygen species production, and the overall effects on cell viability. Read More

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Case Report: Hyperbilirubinemia in Gilbert Syndrome Attenuates Covid-19-Induced Metabolic Disturbances.

Front Cardiovasc Med 2021 17;8:642181. Epub 2021 Feb 17.

Department of Pharmacology and Therapeutics, Faculty of Veterinary Medicine, Damanhour University, Damanhour, Egypt.

Gilbert syndrome (GS) is a liver disorder characterized by non-hemolytic unconjugated hyperbilirubinemia. On the other hand, Coronavirus disease 2019 (Covid-19) is a recent viral infectious disease presented as clusters of pneumonia, triggered by the severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2). Little is known on the association between SARS-CoV-2 and GS, despite different studies have recently stated a link between hyperbilirubinemia and SARS-CoV-2 severity. Read More

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February 2021

A Rare Case Report of Crigler Najjar Syndrome Type II.

Cureus 2021 Jan 12;13(1):e12669. Epub 2021 Jan 12.

Internal Medicine, Services Institute of Medical Sciences, Lahore, PAK.

Crigler-Najjar syndrome is an inborn error of metabolism caused by a point mutation in one of the five exons of UGT1A1 gene, the product of which is responsible for elimination of bilirubin via bile. A number of hyperbilirubinemia disorders similar to Crigler-Najjar syndrome are reported, but they differ in their level of unconjugated bilirubin and responses to the treatment. Here we report a 14-year-old male patient admitted to hospital with the complaint of vomiting and frequent tonsillitis. Read More

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January 2021

Effects of bilirubin configurational photoisomers on the measurement of direct bilirubin by the vanadate oxidation method.

Ann Clin Biochem 2021 Mar 9:4563221999068. Epub 2021 Mar 9.

Department of Pediatrics, Faculty of Medicine, Kagawa University, Kagawa, Japan.

Background: Direct-reacting bilirubin concentrations measured using vanadate chemical oxidation method do not exactly match the conjugated bilirubin concentration. One of the causes is the effect of bilirubin photoisomers. However, the quantitative evaluation of the effects of these photoisomers has not been sufficiently conducted. Read More

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Hemolysis due to Alpha-Hemolytic Enterococcus Urinary Infection: A Rare Cause of Early and Severe Unconjugated Hyperbilirubinemia in a Neonate.

J Pediatr Intensive Care 2021 Mar 23;10(1):75-78. Epub 2020 Apr 23.

Division of Neonatology, Department of Pediatrics, Izmir Katip Celebi University Ataturk Training and Research Hospital, Izmir, Turkey.

The reason for reporting this case is to remind that some microorganisms may cause hemolysis leading to early and severe hyperbilirubinemia by secreting hemolysin in cases; where bilirubin levels cannot be successfully decreased despite effective phototherapy, intravenous immunoglobulin, and even exchange transfusion, or in cases of increased rebound bilirubin (although urinary tract infection is associated with increased conjugated bilirubin fraction and prolonged jaundice). The most common causes of hemolysis are ABO/Rh incompatibility and enzyme deficiencies such as glucose-6-phosphate dehydrogenase (G6PDH), pyruvate kinase (PK), and galactose-1-phosphate uridyltransferase (GALT). Our patient was a male infant, weighing 3,160 g, at 38 + 4 gestational week; he was referred to our unit with total bilirubin level of 14. Read More

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Inhibition of Lipid Accumulation in Skeletal Muscle and Liver Cells: A Protective Mechanism of Bilirubin Against Diabetes Mellitus Type 2.

Front Pharmacol 2020 25;11:636533. Epub 2021 Jan 25.

Department of Nutritional Sciences, Faculty of Life Sciences, University of Vienna, Vienna, Austria.

Ectopic lipid accumulation in skeletal muscle and liver drives the pathogenesis of diabetes mellitus type 2 (DMT2). Mild hyperbilirubinaemia has been repeatedly suggested to play a role in the prevention of DMT2 and is known for its capacity to shape an improved lipid phenotype in humans and in animals. To date, the effect of bilirubin on lipid accumulation in tissues that are prone to ectopic lipid deposition is unclear. Read More

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January 2021

Bile duct ligation causes opposite impacts on the expression and function of BCRP and P-gp in rat brain partly via affecting membrane expression of ezrin/radixin/moesin proteins.

Acta Pharmacol Sin 2021 Feb 8. Epub 2021 Feb 8.

Center of Drug Metabolism and Pharmacokinetics, School of Pharmacy, China Pharmaceutical University, Nanjing, 210009, China.

Breast cancer resistance protein (BCRP) and P-glycoprotein (P-gp) are co-located at blood-brain barrier (BBB) cells, preventing their substrates from entering brain. Accumulating evidence demonstrates that liver failure impairs P-gp and BCRP expression and function in the brain. In the current study, we investigated how liver failure influenced the expression and function of brain BCRP and P-gp in rats subjected to bile duct ligation (BDL). Read More

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February 2021

Outcome of liver transplantation and prevalence of liver fibrosis in Crigler-Najjar syndrome.

Clin Transplant 2021 Apr 22;35(4):e14219. Epub 2021 Feb 22.

Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Introduction: Crigler-Najjar syndrome (CNS) is a rare inherited disorder that is characterized by high levels of non-hemolytic, unconjugated hyperbilirubinemia leading to brain damage and even death. Liver transplantation (LT) can correct the metabolic defect, but there are little data regarding LT in this patient cohort. The liver parenchyma has been considered to be structurally normal in CNS, but there is growing evidence of clinically silent but histologically significant fibrosis in CNS patients. Read More

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Influence of UGT1A1 promoter polymorphism, α-thalassemia and β haplotype in bilirubin levels and cholelithiasis in a large sickle cell anemia cohort.

Ann Hematol 2021 Apr 1;100(4):903-911. Epub 2021 Feb 1.

Genetics Postgraduate Program, Federal University of Pernambuco, Recife, Brazil.

Hyperbilirubinemia in patients with sickle cell anemia (SCA) as a result of enhanced erythrocyte destruction, lead to cholelithiasis development in a subset of patients. Evidence suggests that hyperbilirubinemia may be related to genetic variations, such as the UGT1A1 gene promoter polymorphism, which causes Gilbert syndrome (GS). Here, we aimed to determine the frequencies of UGT1A1 promoter alleles, alpha thalassemia, and β haplotypes and analyze their association with cholelithiasis and bilirubin levels. Read More

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Efficacy of AAV8-h with Rapamycin in neonatal, suckling, and juvenile rats to model treatment in pediatric CNs patients.

Mol Ther Methods Clin Dev 2021 Mar 3;20:287-297. Epub 2020 Dec 3.

Amsterdam UMC, University of Amsterdam, Tytgat Institute for Liver and Intestinal Research, AGEM, Meibergdreef 69-71, 1105 BK Amsterdam, the Netherlands.

A clinical trial using adeno-associated virus serotype 8 (AAV8)-human uridine diphosphate glucuronosyltransferase 1A1 (h) to treat inherited severe unconjugated hyperbilirubinemia (Crigler-Najjar syndrome) is ongoing, but preclinical data suggest that long-term efficacy in children is impaired due to loss of transgene expression upon hepatocyte proliferation in a growing liver. This study aims to determine at what age long-term efficacy can be obtained in the relevant animal model and whether immune modulation allows re-treatment using the same AAV vector. Neonatal, suckling, and juvenile Ugt1a1-deficient rats received a clinically relevant dose of AAV8-h, and serum bilirubin levels and anti-AAV8 neutralizing antibodies (NAbs) in serum were monitored. Read More

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Acute Acquired Methemoglobinemia Following Nitrobenzene Poisoning.

J Nepal Health Res Counc 2021 Jan 22;18(4):798-800. Epub 2021 Jan 22.

Star Hospital, Sanepa Height, Lalitpur, Nepal.

Nitrobenzene is an aromatic compound commonly used in agricultural fertilizers. It is capable of inducing methemoglobinemia when ingested in sufficient quantities. Methemoglobinemia impairs oxygen transport by dual mechanism of impaired oxygen binding and diminished oxygen unloading. Read More

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January 2021

Effectiveness of FIBEROPTIC phototherapy compared to conventional phototherapy in treating HYPERBILIRUBINEMIA amongst term neonates: a randomized controlled trial.

BMC Pediatr 2021 01 11;21(1):32. Epub 2021 Jan 11.

Department of Paediatrics and Child Health, Kilimanjaro Christian Medical University College, P O Box 2240, Moshi, Tanzania.

Background: Neonatal jaundice is one of the most common problems in neonates. Effective treatment of jaundice requires therapeutic intervention with high quality phototherapy. Over recent years, several studies reported fiberoptic phototherapy to be less effective than conventional phototherapy in term neonates. Read More

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January 2021

Novel mutations in Uridyl-diphosphate-glucuronosyl-transferase 1A1 (UGT1A1) gene in Tunisian patients with unconjugated hyperbilirubinemia.

Eur J Med Genet 2021 Feb 7;64(2):104139. Epub 2021 Jan 7.

Université de Tunis El Manar, Institut Pasteur de Tunis, Laboratoire d'Hématologie Moléculaire et Cellulaire, Tunisie.

Introduction: Unconjugated hyperbilirubinemia (UCB) is a feature of Gilbert's syndrome (GS) and Crigler-Najjar's syndrome (CNS), which are two hereditary defects in bilirubin metabolism. Both syndromes are linked to mutations in the UGT1A1 gene, which cause either the decrease or the absence of the UGT1A1 enzymatic activity. Here, we investigated the molecular basis of the UGT1A1 gene in Tunisian patients presenting with unconjugated hyperbilirubinemia. Read More

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February 2021

Neurodevelopmental outcome of healthy term newborn with serum bilirubin >15 mg/dl at one year.

J Neonatal Perinatal Med 2020 Dec 16. Epub 2020 Dec 16.

Department of Pediatrics, Gandhi Medical College & Kamla Nehru Hospital, Bhopal, MP, India.

Background: Neonatal hyperbilirubinemia is a common medical emergency in early neonatal period. Unconjugated bilirubin is neurotoxic and can lead to lifelong neurological sequelae in survivors.

Objective: To find out the association between serum bilirubin and neurodevelopmental outcome at 1 year of age using Development Assessment Scale for Indian Infants (DASII). Read More

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December 2020

The role of microbiota in neonatal hyperbilirubinemia.

Am J Transl Res 2020 15;12(11):7459-7474. Epub 2020 Nov 15.

Department of Neonatology, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health Zhejiang, PR China.

Hyperbilirubinemia accounts for about 60% of full-term and 80% of preterm neonates globally, which is characterized by physiologically elevated unconjugated bilirubin in serum, but abnormally high levels of bilirubin have potential neurotoxic effects. Several factors contribute to the development of neonatal hyperbilirubinemia, including isoimmunization, dysregulated gut flora, genetic alteration and environmental factors. Animal studies have pinpointed the causal roles of several bacteria in bilirubin metabolism. Read More

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November 2020

The effect of ursodeoxycholic acid on indirect hyperbilirubinemia in neonates treated with phototherapy: a randomized clinical trial.

J Matern Fetal Neonatal Med 2020 Nov 22:1-6. Epub 2020 Nov 22.

Department of Epidemiology, School of Health, Arak University of Medical Sciences, Arak, Iran.

Objectives: Indirect hyperbilirubinemia during neonatal period is a common problem, and most preterm and more than half of the term neonates find this problem. Ursodeoxycholic acid (UDCA) protects the liver against oxidative stresses and prevents cellular apoptosis. In addition, it causes stimulation of bile flow, is well tolerated by the patient, and has limited side effects. Read More

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November 2020

Neonatal liver disease.

J Paediatr Child Health 2020 Nov;56(11):1760-1768

Department of Gastroenterology and James Fairfax Institute of Paediatric Nutrition, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.

Neonatal liver disease encompasses many diagnoses, including structural and genetic aetiologies. Many have significant health implications requiring long-term specialist treatment including liver transplantation. Jaundice is a common presenting feature. Read More

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November 2020

The Extent of Intracellular Accumulation of Bilirubin Determines Its Anti- or Pro-Oxidant Effect.

Int J Mol Sci 2020 Oct 30;21(21). Epub 2020 Oct 30.

Italian Liver Foundation (FIF), Bldg Q-AREA Science Park Basovizza, SS14 Km 163,5, 34149 Trieste, Italy.

Background: Severe hyperbilirubinemia can cause permanent neurological damage in particular in neonates, whereas mildly elevated serum bilirubin protects from various oxidative stress-mediated diseases. The present work aimed to establish the intracellular unconjugated bilirubin concentrations (iUCB) thresholds differentiating between anti- and pro-oxidant effects.

Methods: Hepatic (HepG2), heart endothelial (H5V), kidney tubular (HK2) and neuronal (SH-SY5Y) cell lines were exposed to increasing concentration of bilirubin. Read More

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October 2020

p.Cys223Tyr mutation causing Crigler-Najjar syndrome type II.

JGH Open 2020 Oct 16;4(5):1009-1011. Epub 2020 May 16.

Department of Liver Disease, The Second Hospital of Nanjing Nanjing University of Chinese Medicine Nanjing China.

Crigler-Najjar syndrome (CNs) is a rare hereditary unconjugated hyperbilirubinemia caused by mutations in the bilirubin Uridine (UDP) glucuronosyltransferase family 1 member A1 (UGT1A1, ENSG00000241635) gene. Two patients were clinically diagnosed with Crigler-Najjar Syndrome types II (CNs-II) can be clinically diagnosed which were based on the level of total bilirubin, efficacy of phenobarbital treatment, normal liver architecture and exclusion of hemolysis. Diagnosis was also confirmed by UGT1A1 gene mutations, which by sequencing the coding region for UGT1A1 gene mutations, which were the homozygous mutations c. Read More

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October 2020

"Fenofibrate as an adjuvant to phototherapy in pathological unconjugated hyperbilirubinemia in neonates: a randomized control trial."

J Perinatol 2021 Apr 17;41(4):865-872. Epub 2020 Oct 17.

Department of pediatrics, Mansoura University Children's Hospital, Mansoura, Egypt.

Background: Despite widespread phototherapy usage, many new-born infants remain in need of other invasive lines of therapy, such as intravenous immunoglobulins and exchange transfusions.

Objective: Assessment of the efficacy and the safety of adding fenofibrate to phototherapy for the treatment of pathological jaundice in full-term infants.

Design/methods: We conducted a double blinded randomized control study on 180 full-term infants with pathological unconjugated hyperbilirubinemia admitted to the NICU at Mansoura University Children's Hospital. Read More

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Blood Component Transfusion in Tertiary Care Neonatal Intensive Care Unit and Neonatal Intermediate Care Unit: An Audit.

Cureus 2020 Aug 23;12(8):e9952. Epub 2020 Aug 23.

Department of Pediatrics, Pramukhswami Medical College, Karamsad, IND.

Background Neonates admitted in a tertiary neonatal intensive care unit (NICU) require multiple blood transfusions because of extended NICU stay and repeated sampling. The rookie organ systems and miniature blood volumes in the neonate call for regular audits in neonatal blood transfusion practice. Sharing component usage data with the blood bank will prepare them to store components according to demand, thus limiting wastage of components as well as make banks ready to face a shortage in case of ramped up requirements. Read More

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