1,102 results match your criteria Hyperbilirubinemia Unconjugated


Screening and treatment to reduce severe hyperbilirubinaemia in infants in primary care (STARSHIP): a factorial stepped-wedge cluster randomised controlled trial protocol.

BMJ Open 2019 Apr 20;9(4):e028270. Epub 2019 Apr 20.

Division of Neonatology, Department of Paediatrics, Erasmus MC - Sophia Children's Hospital, University Medical Centre Rotterdam, Rotterdam, Netherlands.

Introduction: Jaundice caused by hyperbilirubinaemia is a physiological phenomenon in the neonatal period. However, severe hyperbilirubinaemia, when left untreated, may cause kernicterus, a severe condition resulting in lifelong neurological disabilities. Although commonly applied, visual inspection is ineffective in identifying severe hyperbilirubinaemia. Read More

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http://dx.doi.org/10.1136/bmjopen-2018-028270DOI Listing

Unconjugated bilirubin and schizophrenia: a systematic review.

CNS Spectr 2019 Mar 27:1-12. Epub 2019 Mar 27.

Clínica Universitária de Psiquiatria e Psicologia Médica, Faculdade de Medicina,Universidade de Lisboa,Lisboa,Portugal.

Schizophrenia is a complex syndrome of unknown etiology and difficult to manage. Unconjugated bilirubin has been researched as a potential biological marker of this syndrome. The objective of this review article was to gather the studies published to date on the relationship between this molecule and schizophrenia. Read More

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http://dx.doi.org/10.1017/S109285291800161XDOI Listing
March 2019
1 Read

A 12-week rescue therapy by PrOD-based regimen for advanced fibrotic genotype-1 CHC patients who failed to pegylated interferon plus ribavirin.

J Chin Med Assoc 2019 Mar;82(3):186-190

Division of Gastroenterology and Hepatology, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.

Background: Treatment of chronic hepatitis C (CHC) evolved rapidly due to the invention of interferon-free direct antiviral agents. Previous clinical trials showed combination therapy with paritaprevir/ritonavir, ombitasvir, and dasabuvir (PrOD) with or without ribavirin (RBV) can cure over 95% of genotype 1 CHC patients, regardless with cirrhosis or not. However, real-world data regarding the efficacy and safety of PrOD-based therapy in Asian HCV genotype 1 CHC patients are limited, especially for advanced-fibrotic patients who failed previous therapy with pegylated interferon (PEG-IFN) plus RBV. Read More

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http://dx.doi.org/10.1097/JCMA.0000000000000069DOI Listing
March 2019
1 Read

Easy Diagnosis of Jaundice: A Smartphone-Based Nanosensor Bioplatform Using Photoluminescent Bacterial Nanopaper for Point-of-Care Diagnosis of Hyperbilirubinemia.

ACS Sens 2019 Mar 29. Epub 2019 Mar 29.

Chemistry and Chemical Engineering Research Center of Iran , 14335-186 , Tehran , Iran.

One of the concerns of parents in the first days of their baby's birth is the baby's risk of jaundice/hyperbilirubinemia. This is because more than 60% of babies are born with jaundice that, if not timely diagnosed and subsequently treated, can lead to serious damage to their health. On the other hand, despite recent progress in sensor technology for clinical applications, the development of easy-to-use, cost-effective, sensitive, specific, and portable diagnostic devices, which use nontoxic and biodegradable materials in their design and fabrication, is still in high demand. Read More

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http://dx.doi.org/10.1021/acssensors.9b00275DOI Listing
March 2019
5 Reads

A case of accidental neonatal paracetamol overdose with prolonged half-life and measured metabolites.

Clin Toxicol (Phila) 2019 Mar 11:1-3. Epub 2019 Mar 11.

a Monash Toxicology Unit and Emergency Medicine Service, Monash Health , Victoria , Australia.

Introduction: Limited data exist regarding paracetamol metabolism after overdose in the neonate. We report a case of repeated supratherapeutic overdose in a neonate with paracetamol metabolite concentrations.

Case Report: A 10-day-old male neonate presented to hospital after repeated supratherapeutic dosing of paracetamol. Read More

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http://dx.doi.org/10.1080/15563650.2019.1587450DOI Listing
March 2019
2 Reads
3.122 Impact Factor

LED-phototherapy does not induce oxidative DNA damage in hyperbilirubinemic Gunn rats.

Pediatr Res 2019 Mar 9. Epub 2019 Mar 9.

Section of Molecular Metabolism and Nutrition, Department of Pediatrics, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.

Background: Phototherapy (PT) is the standard treatment of neonatal unconjugated hyperbilirubinemia. Fluorescent tube (FT)-emitted PT light is known to induce oxidative DNA damage in neonates. Nowadays, however, FTs have largely been replaced by light-emitting diodes (LEDs) for delivering PT. Read More

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http://dx.doi.org/10.1038/s41390-019-0367-yDOI Listing
March 2019
3 Reads

Impact of preterm birth and low birth weight on medical conditions, medication use and mortality among neonates: a prospective observational cohort study.

World J Pediatr 2019 Mar 4. Epub 2019 Mar 4.

Department of Pediatrics, JSS Medical College and Hospital, JSS Academy of Higher Education and Research, Mahatma Gandhi Road, Mysuru, Karnataka 570 004, India.

Background: There is a paucity of studies conducted in India on neonatal outcomes of preterm birth and low birth weight. Hence, we aimed to assess the impact of preterm birth and low birth weight on medical conditions, medication use and mortality among neonates.

Methods: A prospective observational cohort study was conducted at the neonatal intensive care unit (NICU) over a period of 9 months. Read More

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http://dx.doi.org/10.1007/s12519-019-00239-1DOI Listing
March 2019
7 Reads

Significance of *28 Genotype in Patients with Advanced Liver Injury Caused By Chronic Hepatitis C.

J Med Biochem 2019 Mar 1;38(1):45-52. Epub 2019 Mar 1.

Clinic for Infectious and Tropical Diseases, Clinical Centre of Serbia, Belgrade, Serbia.

Background: Chronic hepatitis C (CHC) is a significant cause of liver related morbidity and mortality worldwide. The role of genetics in the host response to hepatitis C virus is not elucidated. Genetic variations in gene are the most common cause of hereditary unconjugated hyperbilirubinemia-Gilbert syndrome. Read More

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http://dx.doi.org/10.2478/jomb-2018-0015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6298452PMC
March 2019
1 Read

Neonatal Jaundice.

Indian J Pediatr 2019 Feb 21. Epub 2019 Feb 21.

Department of Radio-Diagnosis, All India Institute of Medical Sciences, New Delhi, India.

Hyperbilirubinemia is a common occurrence in neonates; it may be physiological or pathological. Conjugated hyperbilirubinemia may result from medical or surgical causes, and can result in irreversible liver damage if untreated. The aim of imaging is the timely diagnosis of surgical conditions like biliary atresia and choledochal cysts. Read More

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http://link.springer.com/10.1007/s12098-019-02856-0
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http://dx.doi.org/10.1007/s12098-019-02856-0DOI Listing
February 2019
4 Reads

The effect of intestinal flora on the neural development of severe hyperbilirubinemia neonates.

Eur Rev Med Pharmacol Sci 2019 Feb;23(3):1291-1295

Department of Pediatric, Dezhou People's Hospital, Dezhou, P.R. China.

Objective: To investigate the effect of intestinal flora on the neural development of severe hyperbilirubinemia neonates.

Patients And Methods: The clinical data of 108 severe hyperbilirubinemia neonates admitted to the Dezhou People's Hospital from January 2015 through January 2018 were analyzed, and all newborns had a serum total bilirubin level > 342 μmol/L. Based on whether they suffered from neural development abnormalities, the neonatal patients were divided into the neural abnormality group (n=52) and the non-neural abnormality group (n=56). Read More

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http://dx.doi.org/10.26355/eurrev_201902_17024DOI Listing
February 2019
1 Read

The frequency, clinical course, and health related quality of life in adults with Gilbert's syndrome: a longitudinal study.

BMC Gastroenterol 2019 Feb 4;19(1):22. Epub 2019 Feb 4.

Faculty of Medicine, Misr University for Science and Technology, Cairo, Egypt.

Background: Gilbert syndrome (GS) is an autosomal recessive inherited disorder of bilirubin glucuronidation which has not been investigated in Egypt. This longitudinal study investigated the frequency, clinical course, genetic profile and health related quality of life in Egyptian adults.

Methods: An initial cross-sectional study was conducted to assess the frequency of Gilbert syndrome among Egyptian adults. Read More

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http://dx.doi.org/10.1186/s12876-019-0931-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360704PMC
February 2019
3 Reads

[Study on spectrum of UGT1A1 mutations in connection with inherited non-hemolytic unconjugated hyperbilirubinemia].

Zhonghua Gan Zang Bing Za Zhi 2018 Dec;26(12):898-902

Liver Disease Department, Nanjing Second Hospital, Affiliated to Nanjing University of Chinese Medicine, Nanjing 210003, China.

To compare and analyze patient's general condition, changes in laboratory parameters, and the spectrum of UGT1A1 mutations in patients with inherited non-hemolytic unconjugated hyperbilirubinemia. A retrospective study was conducted at Nanjing Second Hospital from January 2015 to July 2018 and patients' demographic characteristics, liver function test, and UGT1A1 gene were analyzed. The categorical variable data were compared by (2) test. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1007-3418.2018.12.005DOI Listing
December 2018
11 Reads

Phototherapy for Neonatal Unconjugated Hyperbilirubinemia: Examining Outcomes by Level of Care.

Hosp Pediatr 2019 Feb 3;9(2):115-120. Epub 2019 Jan 3.

Department of Health Systems Science, Kaiser Permanente School of Medicine, Pasadena, California.

Objectives: Newborns hospitalized with unconjugated hyperbilirubinemia without critical comorbidities may receive intensive phototherapy (IP) in non-ICU levels of care, such as a mother-newborn unit, or ICU levels of care. Our aim was to compare outcomes between each level.

Methods: Using hospital discharge data from 2005 to 2011 in New York's State Inpatient Database, we performed multivariate analyses to compare outcomes that included total cost of hospitalization, length of stay, 30-day readmission rate after IP, and the number of cases of death, exchange transfusion, and γ globulin infusion. Read More

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http://dx.doi.org/10.1542/hpeds.2018-0136DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6350264PMC
February 2019
1 Read

Bilirubin-Induced Oxidative Stress Leads to DNA Damage in the Cerebellum of Hyperbilirubinemic Neonatal Mice and Activates DNA Double-Strand Break Repair Pathways in Human Cells.

Oxid Med Cell Longev 2018 26;2018:1801243. Epub 2018 Nov 26.

International Centre for Genetic Engineering and Biotechnology (ICGEB), Padriciano 99, 34149 Trieste, Italy.

Unconjugated bilirubin is considered a potent antioxidant when present at moderate levels. However, at high concentrations, it produces severe neurological damage and death associated with kernicterus due to oxidative stress and other mechanisms. While it is widely recognized that oxidative stress by different toxic insults results in severe damage to cellular macromolecules, especially to DNA, no data are available either on DNA damage in the brain triggered by hyperbilirubinemia during the neonatal period or on the activation of DNA repair mechanisms. Read More

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http://dx.doi.org/10.1155/2018/1801243DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287157PMC
February 2019
6 Reads

UGT1A1 genotypes and unconjugated hyperbilirubinemia phenotypes in post-neonatal Chinese children: A retrospective analysis and quantitative correlation.

Medicine (Baltimore) 2018 Dec;97(49):e13576

Department of Pediatrics, Jinshan Hospital of Fudan University, Shanghai, China.

To retrospectively analyze and quantitatively correlate UGT1A1 (bilirubin UDP- glucuronosyltransferase gene) genotypes and unconjugated hyperbilirubinemia (UCH) phenotypes among Chinese children.We retrospectively reviewed UCH patients, quantitatively analyzed genotype-phenotype correlation by comparing with healthy controls. Pfam database, SWISS-model, and Pymol were used for UGT1A1 protein domain analysis and protein modeling for assessing the effect of novel missense variants on protein structure. Read More

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http://dx.doi.org/10.1097/MD.0000000000013576DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6310575PMC
December 2018
2 Reads

High unbound bilirubin for age: a neurotoxin with major effects on the developing brain.

Pediatr Res 2019 Jan 5;85(2):183-190. Epub 2018 Dec 5.

Keck School of Medicine of University of Southern California, Division of Neonatology, Department of Pediatrics, LAC+USC Medical Center, Los Angeles, CA, USA.

Neonatal hyperbilirubinemia is one of the most frequent diagnoses made in neonates. A high level of unconjugated bilirubin that is unbound to albumin is neurotoxic when the level exceeds age-specific thresholds or at lower levels in neonates with neurotoxic risk factors. Lower range of unbound bilirubin results in apoptosis, while moderate-to-high levels result in neuronal necrosis. Read More

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http://www.nature.com/articles/s41390-018-0224-4
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http://dx.doi.org/10.1038/s41390-018-0224-4DOI Listing
January 2019
3 Reads

(TA) Promoter Genotype: Diagnostic and Population Pharmacogenetic Marker in Serbia.

Balkan J Med Genet 2018 Jun 29;21(1):59-68. Epub 2018 Oct 29.

Laboratory for Molecular Biomedicine, Institute of Molecular Genetics and Genetic Engineering, University of Belgrade, Belgrade, Serbia.

The UGT1A1 enzyme is involved in the metabolism of bilirubin and numerous medications. Unconjugated hyperbilirubinemia, commonly presented as Gilbert syndrome (GS), is a result of decreased activity of the UGT1A1 enzyme, variable number of TA repeats in the promoter of the gene affects enzyme activity. Seven and eight TA repeats cause a decrease of UGT1A1 activity and risk GS alleles, while six TA repeats contribute to normal UGT1A1 activity and non-risk GS allele. Read More

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http://dx.doi.org/10.2478/bjmg-2018-0012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6231317PMC
June 2018
3 Reads

Neonatal Cholestasis: A Primer of Selected Etiologies.

Authors:
Ranjani Ananth

Pediatr Ann 2018 Nov;47(11):e433-e439

Cholestasis refers to impairment in formation or excretion of bile. This can be due to defects in intrahepatic production of bile, defects in the transmembrane transport of bile, or mechanical obstruction to bile flow. Clinical features of cholestasis reflect the retention of components of bile (bilirubin, bile acids, cholesterol) in the body. Read More

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https://www.healio.com/doiresolver?doi=10.3928/19382359-2018
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http://dx.doi.org/10.3928/19382359-20181018-01DOI Listing
November 2018
17 Reads

Pamidronate decreases bilirubin-impaired cell death and improves dentinogenic dysfunction of stem cells from human deciduous teeth.

Stem Cell Res Ther 2018 11 8;9(1):303. Epub 2018 Nov 8.

Department of Molecular Cell Biology and Oral Anatomy, Division of Oral Biological Sciences, Kyushu University Graduate School of Dental Science, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.

Background: Hyperbilirubinemia that occurs in pediatric liver diseases such as biliary atresia can result in the development of not only jaundice in the brain, eyes, and skin, but also tooth abnormalities including green pigmentation and dentin hypoplasia in the developing teeth. However, hyperbilirubinemia-induced tooth impairments remain after liver transplantation. No effective dental management to prevent hyperbilirubinemia-induced tooth impairments has been established. Read More

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https://stemcellres.biomedcentral.com/articles/10.1186/s1328
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http://dx.doi.org/10.1186/s13287-018-1042-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6225573PMC
November 2018
31 Reads

Hyperbilirubinemia and Follow-up Auditory Brainstem Responses in Preterm Infants.

Clin Exp Otorhinolaryngol 2019 May 9;12(2):163-168. Epub 2018 Nov 9.

Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Korea.

Objectives: Neonatal hyperbilirubinemia is considered one of the most common causative factors of hearing loss. Preterm infants are more vulnerable to neuronal damage caused by hyperbilirubinemia. This study aimed to evaluate the effect of hyperbilirubinemia on hearing threshold and auditory pathway in preterm infants by serial auditory brainstem response (ABR). Read More

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http://www.e-ceo.org/journal/view.php?doi=10.21053/ceo.2018.
Publisher Site
http://dx.doi.org/10.21053/ceo.2018.00899DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6453789PMC
May 2019
28 Reads

Developmental influence of unconjugated hyperbilirubinemia and neurobehavioral disorders.

Pediatr Res 2019 Jan 23;85(2):191-197. Epub 2018 Oct 23.

Communication Sciences and Disorders, James Madison University, Harrisonburg, VA, USA.

Bilirubin-induced brain injury in the neonatal period has detrimental effects on neurodevelopment that persist into childhood and adulthood, contributing to childhood developmental disorders. Unconjugated bilirubin is a potent antioxidant that may be useful for protecting against oxidative injuries, but it becomes a potent neurotoxin once it crosses the blood brain barrier. Because bilirubin toxicity involves a myriad of pathological mechanisms, can damage most types of brain cells, and affects brain circuits or loops that influence cognition, learning, behavior, sensory, and language, the clinical effects of bilirubin-induced neurotoxicity are likely to be manifold. Read More

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http://www.nature.com/articles/s41390-018-0216-4
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http://dx.doi.org/10.1038/s41390-018-0216-4DOI Listing
January 2019
18 Reads

A Novel Pathogenic Variant in a Sudanese Child with Type 1 Crigler-Najjar Syndrome.

Drug Metab Dispos 2019 01 1;47(1):45-48. Epub 2018 Nov 1.

Department of Pediatrics, Milton S. Hershey Penn State Medical Center, Hershey, Pennsylvania (W.E.); Division of Pharmaceutical Chemistry and Technology, University of Helsinki, Helsinki, Finland (E.J., J.M., M.F.); Pediatric Genomics Discovery Program, Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut (W.J., A.G.S., M.K., S.A.L.); Departments of Pathology and Cell Biology (A.C.I.) and Surgery (S.J.L.), Columbia University Medical Center, New York, New York; and Department of Pediatrics, University of Rochester Medical Center, Rochester, New York (A.C.)

Uridine diphosphate glucuronosyltransferases (UGTs) are key enzymes responsible for the body's ability to process a variety of endogenous and exogenous compounds. Significant gains in understanding UGT function have come from the analysis of variants seen in patients. We cared for a Sudanese child who showed clinical features of type 1 Crigler-Najjar syndrome (CN-1), namely severe unconjugated hyperbilirubinemia leading to liver transplantation. Read More

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http://dmd.aspetjournals.org/lookup/doi/10.1124/dmd.118.0843
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http://dx.doi.org/10.1124/dmd.118.084368DOI Listing
January 2019
5 Reads

Identification of Genetic Risk Factors for Neonatal Hyperbilirubinemia in Fujian Province, Southeastern China: A Case-Control Study.

Biomed Res Int 2018 12;2018:7803175. Epub 2018 Sep 12.

Center of Neonatal Screening, Fujian Provincial Maternity and Children's Hospital, Affiliated Hospital of Fujian Medical University, Fuzhou 350001, Fujian Province, China.

To date, the genetic risk factors for neonatal hyperbilirubinemia remain unknown in Southeastern China. This case-control study aimed to identify the genetic risk factors for neonatal hyperbilirubinemia in Fujian, Southeastern China. A total of 286 hyperbilirubinemic newborns were enrolled as a case group, and 250 randomly selected newborns without jaundice or with a bilirubin level that was lower than the threshold required for phototherapy served as controls. Read More

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http://dx.doi.org/10.1155/2018/7803175DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6157199PMC
January 2019
3 Reads

Case report: multiple UGT1A1 gene variants in a patient with Crigler-Najjar syndrome.

BMC Pediatr 2018 Oct 3;18(1):317. Epub 2018 Oct 3.

Scientific Laboratory of Molecular Genetics, Riga Stradiņš University, Dzirciema Street 16, Riga, LV 1007, Latvia.

Background: Inherited unconjugated hyperbilirubinemia is caused by variants in the gene UGT1A1 leading to Gilbert's syndrome and Crigler-Najjar syndrome types I and II. These syndromes are differentiated on the basis of UGT1A1 residual enzymatic activity and its affected bilirubin levels and responsiveness to phenobarbital treatment.

Case Presentation: In this report, we present a boy with Crigler-Najjar syndrome type II with high unconjugated bilirubin levels that decreased after phenobarbital treatment but increased in adolescence. Read More

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https://bmcpediatr.biomedcentral.com/articles/10.1186/s12887
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http://dx.doi.org/10.1186/s12887-018-1285-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6169020PMC
October 2018
5 Reads

Whole-exome sequencing enables correct diagnosis and surgical management of rare inherited childhood anemia.

Cold Spring Harb Mol Case Stud 2018 10 1;4(5). Epub 2018 Oct 1.

Riley Hospital for Children at IU Health, Indianapolis, Indiana 46202, USA.

Correct diagnosis of inherited bone marrow failure syndromes is a challenge because of the significant overlap in clinical presentation of these disorders. Establishing right genetic diagnosis is crucial for patients' optimal clinical management and family counseling. A nondysmorphic infant reported here developed severe transfusion-dependent anemia and met clinical criteria for diagnosis of Diamond-Blackfan anemia (DBA). Read More

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http://dx.doi.org/10.1101/mcs.a003152DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6169821PMC
October 2018
7 Reads

Development of new methods for determination of bilirubin.

J Pharm Biomed Anal 2019 Jan 18;162:272-285. Epub 2018 Sep 18.

Department of Biosciences and Bioengineering, Indian Institute of Technology Guwahati, Guwahati, Assam, 781039, India. Electronic address:

The ever-increasing demand for a sensitive, rapid and reliable method for determination of serum bilirubin level has been inciting the interest of the researchers to develop new methods for both laboratory set up and point of care applications. These efforts embrace measurement of different forms of bilirubin, such as, unconjugated (free and albumin bound) bilirubin, conjugated (direct) bilirubin, and total (both conjugated and unconjugated) bilirubin in the serum that may provide critical information useful for diagnosis of many diseases and metabolic disorders. Herein, an effort has been made to provide a broad overview on the subject starting from the conventional spectroscopy based analytical methods widely practiced in the laboratory setup along with the sophisticated instrument based sensitive methods suitable for determination of different forms of bilirubin to various portable low cost systems applicable in point of care (POC) settings. Read More

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http://dx.doi.org/10.1016/j.jpba.2018.09.034DOI Listing
January 2019
4 Reads

Crigler-Najjar Syndrome Type II Diagnosed in a Patient with Jaundice Since Birth.

J Coll Physicians Surg Pak 2018 Oct;28(10):806-808

Department of Medicine, King Edward Medical University, Lahore, Pakistan.

Crigler-Najjar syndrome type II is caused by mutations in the UGT1A1 gene resulting in severely reduced hepatic activity of UDP-glucoronyltransferase - an enzyme required to convert bilirubin into a more soluble form that can then be removed from the body. Absence or severe deficiency of this enzyme can lead to bilirubin accumulation in the body resulting in yellow skin and eyes (jaundice). The earliest signs of this disease can be apparent in the neonatal period. Read More

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http://dx.doi.org/3028DOI Listing
October 2018
6 Reads
0.318 Impact Factor

Liver Cirrhosis in a Patient with Crigler Najjar Syndrome.

Fetal Pediatr Pathol 2018 Aug 27;37(4):301-306. Epub 2018 Sep 27.

c Department of Pathology , Baskent University Faculty of Medicine , Ankara , Turkey.

Introduction: Crigler Najjar (CN) disease is a genetic disorder which results in increased unconjugated bilirubin level. Liver parenchyma was previously considered structurally normal. Recent reports describe significant fibrosis in the liver parenchyma of patients with CN syndrome. Read More

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http://dx.doi.org/10.1080/15513815.2018.1492053DOI Listing
August 2018
4 Reads

Hemolytic Anemia: Evaluation and Differential Diagnosis.

Am Fam Physician 2018 Sep;98(6):354-361

Womack Army Medical Center, Fort Bragg, NC, USA.

Hemolytic anemia is defined by the premature destruction of red blood cells, and can be chronic or life-threatening. It should be part of the differential diagnosis for any normocytic or macrocytic anemia. Hemolysis may occur intravascularly, extravascularly in the reticuloendothelial system, or both. Read More

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September 2018
4 Reads

Neuroinflammation and ER-stress are key mechanisms of acute bilirubin toxicity and hearing loss in a mouse model.

PLoS One 2018 14;13(8):e0201022. Epub 2018 Aug 14.

Department Neuroscience, Psychology & Behaviour, University of Leicester, Leicester, Leicestershire, United Kingdom.

Hyperbilirubinemia (jaundice) is caused by raised levels of unconjugated bilirubin in the blood. When severe, susceptible brain regions including the cerebellum and auditory brainstem are damaged causing neurological sequelae such as ataxia, hearing loss and kernicterus. The mechanism(s) by which bilirubin exerts its toxic effect have not been completely understood to date. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0201022PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6091913PMC
February 2019
14 Reads

A New Variant of PKLR Gene Associated With Mild Hemolysis may be Responsible for the Misdiagnosis in Pyruvate Kinase Deficiency.

J Pediatr Hematol Oncol 2019 Jan;41(1):e1-e2

Division of Pediatric Hematology, Dr. Behçet Uz Children's Hospital, Izmir, Turkey.

Pyruvate kinase deficiency (PKD) is the most common glycolytic defect leading to hemolytic anemia. PKD is caused by the mutations in the PKLR gene; however, the detection of a decreased PK activity should be first measured for rapid diagnosis. We report here the case of a 1-year-old girl with mild hemolysis and PKD. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001254DOI Listing
January 2019
29 Reads

Clinical evaluation of severe neonatal Hyperbilirubinaemia in a resource-limited setting: a 4-year longitudinal study in south-East Nigeria.

BMC Pediatr 2018 06 23;18(1):202. Epub 2018 Jun 23.

Department of Paediatrics, Enugu State University of Science and Technology, Enugu, Enugu State, Nigeria.

Background: Neonatal hyperbilirubinaemia is one of the commonest causes of hospital visit in the neonatal period. When severe, it is a leading cause of irreversible neurological and musculoskeletal disability. Prompt recognition and timely interventions are imperative for a drastic reduction in complications associated with severe hyperbilirubinaemia in newborns. Read More

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http://dx.doi.org/10.1186/s12887-018-1174-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6015658PMC
June 2018
7 Reads
1.920 Impact Factor

Extreme neonatal hyperbilirubinemia, acute bilirubin encephalopathy, and kernicterus spectrum disorder in children with galactosemia.

Pediatr Res 2018 08 31;84(2):228-232. Epub 2018 May 31.

Department of Pediatrics, Aalborg University Hospital, Aalborg, Denmark.

Background: Galactosemia has not been recognized as a cause of extreme neonatal hyperbilirubinemia, although growing evidence supports this association.

Methods: In a retrospective cohort study, we identified children with galactosemia due to GALT deficiency using the Danish Metabolic Laboratory Database. Among these, we identified children with extreme neonatal hyperbilirubinemia or symptoms of ABE. Read More

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http://dx.doi.org/10.1038/s41390-018-0066-0DOI Listing
August 2018
9 Reads

A Perspective on a Possible Relation Between the Psychopathology of the Schizophrenia/Schizoaffective Spectrum and Unconjugated Bilirubin: A Longitudinal Protocol Study.

Front Psychiatry 2018 23;9:146. Epub 2018 Apr 23.

Clínica de Saúde Mental do Porto, Porto, Portugal.

Some authors suggest a relation between Unconjugated Bilirubin (UCB) plasma high levels and schizophrenia, as schizophrenia patients have been showing higher UCB levels when compared with other psychiatric patients and general population. These higher UCB levels have been already correlated with acute psychotic states, positive symptoms, and poor outcome in patients with schizophrenia. Schizophrenia and schizoaffective disorders share common symptoms but there aren't yet accepted biomarkers for their distinction. Read More

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http://dx.doi.org/10.3389/fpsyt.2018.00146DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5924810PMC
April 2018
2 Reads

Successful Liver Transplants for Liver Failure Associated With Pyruvate Kinase Deficiency.

Pediatrics 2018 04;141(Suppl 5):S385-S389

Division of Gastroenterology, Hepatology, and Nutrition, Centre Hospitalier Universitaire Sainte-Justine, Université de Montréal, Montreal, Quebec, Canada;

Pyruvate kinase deficiency (PKD) is the most common cause of congenital nonspherocytic chronic hemolytic anemia, and patients normally present with mild to severe anemia, unconjugated hyperbilirubinemia, and splenomegaly. Only a few reports of PKD have documented its association with severe, progressive liver failure. In all those cases, the patients died before liver transplant (LT) or immediately after transplant. Read More

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http://dx.doi.org/10.1542/peds.2016-3896DOI Listing
April 2018
6 Reads

Unconjugated hyperbilirubinemia in a blood donor: Chance finding due to unusual plasma discoloration.

Asian J Transfus Sci 2018 Jan-Jun;12(1):8-9

Department of Transfusion Medicine, SGPGIMS, Lucknow, Uttar Pradesh, India.

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http://dx.doi.org/10.4103/ajts.AJTS_34_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5850704PMC
March 2018
1 Read

UGT1A1 gene and neonatal hyperbilirubinemia: a preliminary study from Bengkulu, Indonesia.

BMC Res Notes 2018 Mar 13;11(1):172. Epub 2018 Mar 13.

Division of Pharmaceutical Microbiology and Biotechnology, Faculty of Pharmacy, Universitas Indonesia, UI Depok Campus, Depok, West Java, 16424, Indonesia.

Objective: The genetic involvement in unconjugated neonatal hyperbilirubinemia has been extensively studied. Despite the high incidence of hyperbilirubinemia in Indonesia, studies are lacking. The objective of this study is to elucidate the role of polymorphism in the UGT1A1 in Neonatal Hyperbilirubinemia in Bengkulu, Indonesia. Read More

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http://dx.doi.org/10.1186/s13104-018-3284-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5851072PMC
March 2018
4 Reads

The Neuroprotective Effects of Hypothermia on Bilirubin-Induced Neurotoxicity in vitro.

Neonatology 2018 6;113(4):360-365. Epub 2018 Mar 6.

Division of Neonatology, Department of Pediatrics, School of Medicine, Marmara University, Istanbul, Turkey.

Background: In high-risk newborns indirect hyperbilirubinemia can lead to acute bilirubin encephalopathy and kernicterus. Despite the current therapeutic modalities, preventing or reversing the neurotoxicity cannot be achieved in all infants.

Objective: To investigate the neuroprotective effects of hypothermia on bilirubin-induced toxicity in primary mouse neuronal cell cultures. Read More

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http://dx.doi.org/10.1159/000487221DOI Listing
March 2018
2 Reads

Attenuation of neuro-inflammation improves survival and neurodegeneration in a mouse model of severe neonatal hyperbilirubinemia.

Brain Behav Immun 2018 05 16;70:166-178. Epub 2018 Feb 16.

International Centre for Genetic Engineering and Biotechnology (ICGEB), Padriciano, 99, 34149 Trieste, Italy. Electronic address:

All pre-term newborns and a high proportion of term newborns develop neonatal jaundice. Neonatal jaundice is usually a benign condition and self-resolves within few days after birth. However, a combination of unfavorable complications may lead to acute hyperbilirubinemia. Read More

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http://dx.doi.org/10.1016/j.bbi.2018.02.011DOI Listing
May 2018
20 Reads

Diagnostic criteria and contributors to Gilbert's syndrome.

Crit Rev Clin Lab Sci 2018 03 1;55(2):129-139. Epub 2018 Feb 1.

b School of Medical Science and Menzies Health Institute Queensland , Griffith University , Gold Coast , Australia.

Hyperbilirubinemia is a well-known condition in the clinical setting; however, the causes of elevated serum bilirubin are diverse, as are the clinical ramifications of this condition. For example, diagnoses of individuals vary depending on whether they exhibit an unconjugated or conjugated hyperbilirubinemia. Diagnoses can include conditions of disordered bilirubin metabolism (Gilbert's, Crigler-Najjar, Rotor, or Dubin-Johnson syndromes) or an acquired disease, including alcoholic/non-alcoholic fatty liver disease, hepatotropic hepatitis, cirrhosis, or hepato-biliary malignancy. Read More

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http://dx.doi.org/10.1080/10408363.2018.1428526DOI Listing
March 2018
11 Reads

"Gilbert's-like" syndrome as part of a spectrum of persistent unconjugated hyperbilirubinemia in post-chronic hepatitis patients.

Sci Rep 2018 01 31;8(1):2008. Epub 2018 Jan 31.

Division of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 430022, Wuhan, China.

Gilbert's syndrome (GS) patients present with remittent unconjugated hyperbilirubinemia. In this study, we investigated the correlation between polymorphisms in the gene encoding UDP-glucuronosyltransferase, UGT1A1, and the development of unconjugated hyperbilirubinemia in clinical GS and post-hepatitis hyperbilirubinemia. Blood samples were collected from 285 patients, including 85 patients who were clinically diagnosed with GS, 70 patients who had indirect hyperbilirubinemia during the recovery period of chronic liver diseases, 109 patients with normal hepatic function and 21 chronic active hepatitis patients. Read More

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http://dx.doi.org/10.1038/s41598-018-19847-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5792633PMC
January 2018
12 Reads
5.080 Impact Factor

Unconjugated bilirubin induces pyroptosis in cultured rat cortical astrocytes.

J Neuroinflammation 2018 Jan 22;15(1):23. Epub 2018 Jan 22.

Department of Neonatology, Children's Hospital of Chongqing Medical University, Chongqing, 400014, China.

Background: Bilirubin-induced neurological dysfunction (BIND), a severe complication of extreme neonatal hyperbilirubinemia, could develop into permanent neurodevelopmental impairments. Several studies have demonstrated that inflammation and nerve cell death play important roles in bilirubin-induced neurotoxicity; however, the underlying mechanism remains unidentified.

Methods: The present study was intended to investigate whether pyroptosis, a highly inflammatory form of programmed cell death, participated in the bilirubin-mediated toxicity on cultured rat cortical astrocytes. Read More

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http://dx.doi.org/10.1186/s12974-018-1064-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5776766PMC
January 2018
9 Reads

Caffeine prevents bilirubin-induced cytotoxicity in cultured newborn rat astrocytes.

J Matern Fetal Neonatal Med 2019 Jun 2;32(11):1813-1819. Epub 2018 Jan 2.

c Department of Anatomy, Division of Neonatology, Faculty of Medicine , Pamukkale University , Denizli , Turkey.

Objective: Unconjugated bilirubin (UCB) may cause neurotoxicity in preterm neonates due to immaturity of UGT1A1 leading to bilirubin accumulation in the brain. Caffeine used in the treatment of apnea of prematurity was reported to decrease mechanical ventilation requirement, the frequencies of bronchopulmonary dysplasia, patent ductus arteriosus, cerebral palsy and neurodevelopmental disorders in very low birth weight infants. However, the effect of caffeine on hyperbilirubinemia was not yet clarified. Read More

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http://dx.doi.org/10.1080/14767058.2017.1419175DOI Listing
June 2019
5 Reads

Mild hyperbilirubinaemia as an endogenous mitigator of overweight and obesity: Implications for improved metabolic health.

Atherosclerosis 2018 02 14;269:306-311. Epub 2017 Dec 14.

University of Vienna, Faculty of Life Sciences, Department of Nutritional Sciences, Althanstraße 14 (UZA2), 1090, Vienna, Austria; Research Platform Active Ageing, University of Vienna, Faculty of Life Sciences, Althanstraße 14 (UZA2), 1090, Vienna, Austria. Electronic address:

Background And Aims: Mild endogenous elevation of unconjugated bilirubin (UCB) as seen in Gilbert's syndrome (GS), might mitigate cardiovascular disease (CVD) risk factors including overweight/obesity. This study aimed to determine whether hyperbilirubinaemia is linked to improved anthropometric data and lipid profile.

Methods: Our study considered GS and age-/gender-matched healthy controls (n = 248). Read More

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http://dx.doi.org/10.1016/j.atherosclerosis.2017.12.021DOI Listing
February 2018
16 Reads

Point-of-care device to diagnose and monitor neonatal jaundice in low-resource settings.

Proc Natl Acad Sci U S A 2017 12 4;114(51):E10965-E10971. Epub 2017 Dec 4.

Department of Bioengineering, Rice University, Houston, TX 77030;

Newborns are at increased risk of jaundice, a condition in which excess bilirubin accumulates in blood. Left untreated, jaundice can lead to neurological impairment and death. Jaundice resulting from unconjugated hyperbilirubinemia is easily treated with exposure to blue light, and phototherapy systems have been developed for low-resource settings; however, there are no appropriate solutions to diagnose and monitor jaundice in these settings. Read More

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http://dx.doi.org/10.1073/pnas.1714020114DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5754796PMC
December 2017
12 Reads

An Infant With Unusually High Unconjugated Hyperbilirubinemia Due to Coexistence of Hereditary Spherocytosis and Gilbert Syndrome.

J Pediatr Hematol Oncol 2018 03;40(2):e127-e128

Clinical Hospital Center Rijeka, Rijeka, Croatia.

Hereditary spherocytosis is the most frequent congenital hemolytic anemia and is characterized with variable degree of anemia, jaundice, and splenomegaly. In the case of severe hyperbilirubinemia out of proportion with hemolysis, other causes of hyperbilirubinemia must be considered. Gilbert syndrome (GS) is an autosomal dominant disorder characterized with intermittent hyperbilirubinemia without any other sign and symptom of liver disease as a result of reduced activity of uridine diphosphate-glucuronyl transferase 1A1. Read More

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http://Insights.ovid.com/crossref?an=00043426-201803000-0003
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http://dx.doi.org/10.1097/MPH.0000000000001025DOI Listing
March 2018
13 Reads

Identification of pathogens for differential diagnosis of fever with jaundice in the Central African Republic: a retrospective assessment, 2008-2010.

BMC Infect Dis 2017 11 29;17(1):735. Epub 2017 Nov 29.

Institut Pasteur of Bangui, Virology Department, PO Box 923, Bangui, Central African Republic.

Background: Febrile jaundice results clinically in generalized yellow coloration of the teguments and mucous membranes due to excess plasma bilirubin, accompanied by fever. Two types are found: conjugated and unconjugated bilirubin jaundice. Jaundice is a sign in several diseases due to viruses (viral hepatitis and arbovirus), parasites (malaria) and bacteria (leptospirosis). Read More

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http://dx.doi.org/10.1186/s12879-017-2840-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5707826PMC
November 2017
21 Reads

Hepatic Parenchymal Injury in Crigler-Najjar Type I.

J Pediatr Gastroenterol Nutr 2018 04;66(4):588-594

Division of Gastroenterology, Hepatology and Nutrition.

Background: Crigler-Najjar syndrome type I (CNI) arises from biallelic variants of UGT1A1 that abrogate uridine diphosphate glucuronosyltransferase (UGT1A1) activity resulting in unconjugated hyperbilirubinemia. Historically, liver parenchyma in CNI was considered structurally and histologically normal. Recent review of CNI liver explants revealed fibrosis. Read More

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http://dx.doi.org/10.1097/MPG.0000000000001843DOI Listing
April 2018
7 Reads