1,240 results match your criteria Hyperbilirubinemia Unconjugated

Ursodeoxycholic acid as adjuvant treatment to phototherapy for neonatal hyperbilirubinemia: a systematic review and meta-analysis.

World J Pediatr 2022 Jun 11. Epub 2022 Jun 11.

Institute of Clinical Medicine, Department of Pediatrics, University of Eastern Finland, Kuopio, Finland.

Background: Neonatal hyperbilirubinemia is observed in most newborns, and 5-15% of neonates require phototherapy. Phototherapy is effective but often prolongs hospitalization and has both short-term and potential long-term harms. The aim of this systematic review and meta-analysis was to evaluate the role of ursodeoxycholic acid (UDCA) combined with phototherapy in neonatal hyperbilirubinemia. Read More

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Vascular network expansion, integrity of blood-brain interfaces, and cerebrospinal fluid cytokine concentration during postnatal development in the normal and jaundiced rat.

Fluids Barriers CNS 2022 Jun 7;19(1):47. Epub 2022 Jun 7.

BIP Facility, Lyon Neurosciences Research Center, Bron, France.

Background: Severe neonatal jaundice resulting from elevated levels of unconjugated bilirubin in the blood induces dramatic neurological impairment. Central oxidative stress and an inflammatory response have been associated with the pathophysiological mechanism. Cells forming the blood-brain barrier and the choroidal blood-CSF barrier are the first CNS cells exposed to increased plasma levels of unconjugated bilirubin. Read More

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[Hepatic manifestations of hematological diseases].

Zhonghua Gan Zang Bing Za Zhi 2022 Apr;30(4):347-351

Liver Research Center, Beijing Friendship Hospital, Capital Medical University; Beijing Key Laboratory of Translational Medicine on Liver Cirrhosis & National Clinical Research Center for Digestive Diseases, Beijing 100050, China.

Liver involvement is often observed in hematological disorders, resulting in liver abnormality, including unconjugated hyperbilirubinemia, monoclonal hyperglobulinemia, portal vein, or hepatic vein thrombosis or portal hypertension, hepatosplenomegaly, or iron accumulation in the liver. Here we summarize the major hematological diseases that often affect the liver: hemolytic anemia, defect in coagulation or anti-coagulation factors, myeloproliferative neoplasm, hemophagocytic lymphohistiocytosis, multiple myeloma, leukemia, and lymphoma. We hope this review will help clinicians diagnose and manage the patients with liver involvement by hematological disorders. Read More

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Novel combined UGT1A1 mutations in Crigler Najjar Syndrome type I.

J Clin Lab Anal 2022 Jun 9;36(6):e24482. Epub 2022 May 9.

Genomics of Signalopathies at the service of Medicine, Medical University of Sfax, Sfax, Tunisia.

Background: Uridine diphosphate-glucuronosyl transferase 1A1 (UGT1A1), which is the major UGT1 gene product, is located on chromosome 2q37. The expression of UGT1A1 is relatively managed by a polymorphic dinucleotide repeat inside the promoter TATA box consisting of 5-8 copies of a TA repeat. A (TA) 6TAA is considered as the wild type. Read More

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Gilbert or Crigler-Najjar syndrome? Neonatal severe unconjugated hyperbilirubinemia with P364L UGT1A1 homozygosity.

Ital J Pediatr 2022 Apr 18;48(1):59. Epub 2022 Apr 18.

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Pediatric Liver Unit, Milan, Italy.

Background: Several mutations of bilirubin uridine diphosphate-glucuronosyltransferase gene (UGT1A1) have been reported in patients with unconjugated hyperbilirubinemia. Few reports are available about the p.Pro364Leu mutation (P364L, c. Read More

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Light-Emitting Diode (LED) Phototherapy Versus Non-LED Phototherapy Devices for Hyperbilirubinemia in Neonates: A Systematic Review and Meta-Analysis.

Am J Perinatol 2022 Jun 7. Epub 2022 Jun 7.

Unidad de Análisis y Generación de Evidencias en Salud Pública (UNAGESP), Instituto Nacional de Salud, Ministerio de Salud Lima, Lima, Peru.

Objective:  This review was conducted to evaluate the efficacy of light-emitting diode (LED) phototherapy as compared with the conventional phototherapy in neonates with unconjugated hyperbilirubinemia and their adverse effects.

Study Design:  We searched the following databases right from their inception till April, 2021: MEDLINE, EMBASE, Cochrane Library, and LILACS. Randomized clinical trials (RCTs) comparing the LED phototherapy with other light sources, which enrolled newborns (term and preterm) with unconjugated hyperbilirubinemia were included. Read More

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Genetic testing of UGT1A1 in the diagnosis of Gilbert syndrome: The discovery of seven novel variants in the Chinese population.

Mol Genet Genomic Med 2022 Apr 14:e1958. Epub 2022 Apr 14.

Research Laboratory of Clinical Virology, Department of Infectious Diseases, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

Background: Genetic testing of UGT1A1 was used to facilitate the diagnosis of Gilbert syndrome, and analyze the distribution features of pathogenic variants in the Chinese population.

Methods: DNA was extracted from whole blood samples of patients with unconjugated hyperbilirubinemia, and sequencing of the UGT1A1 gene was performed after PCR amplification. After alignment with reference sequences, the known pathogenic variants were identified, the variant spectrum was analyzed, and the pathogenicity of novel variants was predicted using online mutation prediction tools. Read More

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Effect of the genetic mutant G71R in uridine diphosphate-glucuronosyltransferase 1A1 on the conjugation of bilirubin.

Open Life Sci 2022 18;17(1):221-229. Epub 2022 Mar 18.

Department of Pediatrics, The First Affiliated Hospital of Guangxi Medical University, No. 6 Shuangyong Road, Nanning 530021, Guangxi, China.

We aimed to investigate the effect of the genetic mutant G71R (c. 211G > A) in uridine diphosphate (UDP)-glucuronosyltransferase 1A1 (UGT1A1) on the glucuronidation of unconjugated bilirubin. The UGT1A1 wild-type and mutant G71R gene sequences were inserted into the lentiviral vector GV358 plasmid and then transfected into COS-7 cells. Read More

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Successful haploidentical hematopoietic stem cell transplantation (HSCT) and durable engraftment by repeated donor lymphocyte infusions for a Chinese patient with transfusion-dependent hemoglobin (Hb) Hammersmith and massive splenomegaly.

Pediatr Transplant 2022 Apr 9:e14278. Epub 2022 Apr 9.

Department of Pediatrics and Adolescent Medicine, Hong Kong Children's Hospital, Hong Kong Special Administrative Region, China.

Background: Hemoglobin (Hb) Hammersmith is a rare form of unstable β-chain hemoglobinopathy causing hemolytic anemia. This rare event led to a more serious transfusion-dependent phenotype in a patient. It was successfully cured by haploidentical hematopoietic stem cell transplantation (HSCT). Read More

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Can serum albumin level affect the transcutaneous bilirubinometry in term neonates?

D Kumar D Kumar

J Neonatal Perinatal Med 2022 Mar 25. Epub 2022 Mar 25.

Department of Pediatrics, Uttar Pradesh University of Medical Sciences, Saifai, Etawah, India.

Background: Jaundice is the quite common benign condition in neonates, but due to its potential toxicity, neonates must be monitored. This study was aimed to evaluate the effect of serum albumin level on the transcutaneous bilirubin (TcB) measurements in term neonates with unconjugated hyperbilirubinemia.

Methods: Serum albumin and total serum bilirubin (TSB) of 252 jaundiced term neonates were estimated and simultaneously TcB was measured over sternum and the effect of serum albumin on TcB measurements was evaluated. Read More

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Periodic change of body position under phototherapy in term and preterm neonates with hyperbilirubinaemia.

Cochrane Database Syst Rev 2022 03 2;3:CD011997. Epub 2022 Mar 2.

Department of Neonatology, Government Medical College and Hospital, Chandigarh, India.

Background: Phototherapy is the mainstay of treatment of neonatal hyperbilirubinaemia. Periodic change in position of the neonate under phototherapy (from supine to prone or lateral positions) may improve the efficiency of phototherapy by hastening the access of phototherapy light to bilirubin deposited in different parts of the skin and subcutaneous tissue.

Objectives: To evaluate the effects of periodic change of body position during phototherapy as compared to no prescribed change in body position, on serum total bilirubin level and duration of treatment in neonates with unconjugated hyperbilirubinaemia during the first 28 days of life. Read More

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Sexual dimorphism: increased sterol excretion leads to hypocholesterolaemia in female hyperbilirubinaemic Gunn rats.

J Physiol 2022 04 1;600(8):1889-1911. Epub 2022 Mar 1.

School of Pharmacy and Medical Sciences, Griffith University, Gold Coast, Queensland, Australia.

Circulating bilirubin is associated with reduced serum cholesterol concentrations in humans and in hyperbilirubinaemic Gunn rats. However, mechanisms contributing to hypocholesterolaemia remain unknown. Therefore, this study aimed to investigate cholesterol synthesis, transport and excretion in mutant Gunn rats. Read More

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Intravenous Fluid Supplementation for Preterm Infants With Hyperbilirubinemia: A Retrospective Cohort Study.

Adv Neonatal Care 2022 Feb 2. Epub 2022 Feb 2.

Department of Pediatrics, College of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia (Ms Al-Wassia); and Department of Pediatrics, King Fahad Specialist Hospital, Dammam, Saudi Arabia (Mr Garrada).

Background: Hyperbilirubinemia is one of the most frequently occurring problems in the neonatal period, and phototherapy has remained the primary treatment of choice. Fluid supplementation has been proposed to reduce serum bilirubin levels.

Purpose: To assess the risks and benefits of fluid supplementation compared with standard fluid management in preterm infants with unconjugated hyperbilirubinemia under conventional phototherapy. Read More

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February 2022

Pharmaceutical strategies for preventing toxicity and promoting antioxidant and anti-inflammatory actions of bilirubin.

J Enzyme Inhib Med Chem 2022 Dec;37(1):487-501

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

Bilirubin (BR) is the final product of haem catabolism. Disruptions along BR metabolic/transport pathways resulting from inherited disorders can increase plasma BR concentration (hyperbilirubinaemia). Unconjugated hyperbilirubinemia may induce BR accumulation in brain, potentially causing irreversible neurological damage, a condition known as BR encephalopathy or kernicterus, to which newborns are especially vulnerable. Read More

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December 2022

A rare etiology of persistent jaundice in type 1 autoimmune hepatitis.

Arch Clin Cases 2021 29;8(3):37-41. Epub 2021 Dec 29.

Department of Medical Gastroenterology, All India Institute of Medical Sciences, Rishikesh, India.

A 24-year-old male without previously known comorbidities presented with progressive jaundice and hepatomegaly. Autoimmune serology and liver biopsy revealed features of autoimmune hepatitis. A treatment by oral prednisolone and azathioprine was initiated, after which he achieved remission. Read More

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December 2021

Clinical and Genetic Etiologies of Neonatal Unconjugated Hyperbilirubinemia in the China Neonatal Genomes Project.

J Pediatr 2022 04 23;243:53-60.e9. Epub 2021 Dec 23.

Department of Neonatology, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai, China; Key Laboratory of Birth Defects, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai, China; Clinical Genetic Center, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai, China. Electronic address:

Objective: To investigate the clinical and genetic causes of neonatal unconjugated hyperbilirubinemia.

Study Design: We included 1412 neonates diagnosed with unconjugated hyperbilirubinemia (total serum bilirubin >95 percentile for age), from the China Neonatal Genomes Project between August 2016 and September 2019, in the current study. Clinical data and targeted panel sequencing data on 2742 genes including known unconjugated hyperbilirubinemia genes were analyzed. Read More

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Long-Term Effects of Biliverdin Reductase a Deficiency in Mice: Impact on Redox Status and Metabolism.

Antioxidants (Basel) 2021 Dec 20;10(12). Epub 2021 Dec 20.

Tytgat Institute for Liver and Intestinal Research, Amsterdam Gastroenterology and Metabolism, Amsterdam University Medical Centers, Location AMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.

Accumulation of neurotoxic bilirubin due to a transient neonatal or persistent inherited deficiency of bilirubin glucuronidation activity can cause irreversible brain damage and death. Strategies to inhibit bilirubin production and prevent neurotoxicity in neonatal and adult settings seem promising. We evaluated the impact of deficiency in neonatal and aged mice, in a background of unconjugated hyperbilirubinemia, by abolishing bilirubin production. Read More

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December 2021

Case Report: Bilateral Valsalva Retinopathy in an Acutely Anemic Patient.

Optom Vis Sci 2022 03;99(3):303-307

Significance: The Valsalva maneuver is known to cause pre-retinal hemorrhages, which have a predilection to affect the macula. Certain conditions may increase the risk of developing Valsalva retinopathy including anemia. Knowing these associations can aid in uncovering underlying systemic conditions for which a patient may not otherwise be aware. Read More

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Neonatal hyperbilirubinemia: recommendations for diagnosis and management in the emergency department.

Pediatr Emerg Med Pract 2022 Jan 2;19(1):1-24. Epub 2022 Jan 2.

Health Science Clinical Professor, Pediatric Emergency Medicine, University of California Riverside, Department of Emergency Medicine, Riverside, CA.

Hyperbilirubinemia is one the most common reasons for emergency department visits for the neonate. Most cases of unconjugated hyperbilirubinemia are benign. Although rare, unrecognized or untreated pathologic unconjugated hyperbilirubinemia can lead to the development of acute bilirubin encephalopathy and, ultimately, kernicterus. Read More

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January 2022

Abnormal liver enzymes: A review for clinicians.

World J Hepatol 2021 Nov;13(11):1688-1698

Department of Medicine, Texas A&M University, College Station, TX 77843, United States.

Liver biochemical tests are some of the most commonly ordered routine tests in the inpatient and outpatient setting, especially with the automatization of testing in this technological era. These tests include aminotransferases, alkaline phosphatase, gamma-glutamyl transferase, bilirubin, albumin, prothrombin time and international normalized ratio (INR). Abnormal liver biochemical tests can be categorized based on the pattern and the magnitude of aminotransferases elevation. Read More

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November 2021

Neonatal Eye Shielding during Phototherapy: What Protects the Eye Better?

J Trop Pediatr 2021 12;67(6)

Department of Pediatrics, University of Minnesota & Hennepin Healthcare, Minneapolis, MN 55415, USA.

Background: Phototherapy remains an important component of the management of unconjugated neonatal jaundice, a major cause of newborn morbidity. During phototherapy the toxic unconjugated form of bilirubin is converted to nontoxic form through action of light delivered by phototherapy. An important possible complication of phototherapy is retinal damage and therefore eye protective measures are taken during therapy to prevent this from occurring. Read More

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December 2021

Transient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus D prophylaxis: a case report.

J Med Case Rep 2021 Nov 26;15(1):573. Epub 2021 Nov 26.

Institute of Clinical Research, University of Southern Denmark, Odense, Denmark.

Background: In neonates, rhesus D alloimmunization despite anti-D immunoglobulin prophylaxis is rare and often unexplained. Rhesus D alloimmunization can lead to hemolytic disease of the newborn with anemia and unconjugated hyperbilirubinemia. In past reports, transient congenital hyperinsulinism has been described as a rare complication of rhesus D alloimmunization. Read More

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November 2021

[Gilbert's syndrome: hyperbilirubinemia enemy or friend].

Zhonghua Gan Zang Bing Za Zhi 2021 Oct;29(10):1024-1027

Department of Elderly Gastroenterology, The First Affiliated Hospital of China Medical University, Shenyang 110001, China.

Gilbert's syndrome is a kind of benign inherited disease of bilirubin binding disorder, mainly due to the homozygous polymorphism A(TA)7TAA in the promoter of the gene for uridine diphosphate -glucuronosyltransferase 1A1 (UGT1A1), which is a TA insertion into the promoter, designated as UGT1A1*28, with UGT activity reduction to 30% of the normal value. Therefore, circulating fat-soluble unconjugated bilirubin cannot be converted into water-soluble conjugated bilirubin, leading to unconjugated hyperbilirubinemia. Bilirubin has a strong affinity for erythrocyte phospholipids, which interferes with membrane composition and dynamics, resulting in increased erythrocytes fragility, easy rupture, and gradual shortening of survival time. Read More

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October 2021

Irradiance footprint of phototherapy devices: a comparative study.

Pediatr Res 2021 Nov 2. Epub 2021 Nov 2.

Neonatology Department, UMCG, Groningen, The Netherlands.

Background: Phototherapy (PT) is the standard treatment of neonatal unconjugated hyperbilirubinemia. The irradiance footprint, i.e. Read More

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November 2021

Bilirubin represents a negative regulator of ILC2 in allergic airway inflammation.

Mucosal Immunol 2022 02 22;15(2):314-326. Epub 2021 Oct 22.

Joint Program in Immunology, Department of Internal Medicine, Guangzhou Women and Children's Medical Center, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, China.

Group 2 innate lymphoid cells (ILC2s) play an important role in allergic airway inflammation. Despite recent advances in defining molecular mechanisms that control ILC2 development and function, the role of endogenous metabolites in the regulation of ILC2s remains poorly understood. Herein, we demonstrated that bilirubin, an end product of heme catabolism, was a potent negative regulator of ILC2s. Read More

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February 2022

Bile Duct Ligation Upregulates Expression and Function of L-Amino Acid Transporter 1 at Blood-Brain Barrier of Rats via Activation of Aryl Hydrocarbon Receptor by Bilirubin.

Biomedicines 2021 Sep 26;9(10). Epub 2021 Sep 26.

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

Liver failure is associated with increased levels of brain aromatic amino acids (AAAs), whose transport across the blood-brain barrier (BBB) is mainly mediated by L-amino acid transporter 1 (LAT1). We aimed to investigate whether liver failure induced by bile duct ligation (BDL) increases levels of brain AAAs by affecting the expression and function of LAT1. The LAT1 function was assessed using the brain distribution of gabapentin. Read More

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September 2021

Severe Presentation of Congenital Hemolytic Anemias in the Neonatal Age: Diagnostic and Therapeutic Issues.

Diagnostics (Basel) 2021 Aug 26;11(9). Epub 2021 Aug 26.

Neonatal Intensive Care Unit, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy.

Congenital hemolytic anemias (CHAs) are a group of diseases characterized by premature destruction of erythrocytes as a consequence of intrinsic red blood cells abnormalities. Suggestive features of CHAs are anemia and hemolysis, with high reticulocyte count, unconjugated hyperbilirubinemia, increased lactate dehydrogenase (LDH), and reduced haptoglobin. The peripheral blood smear can help the differential diagnosis. Read More

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Factors Affecting Prevalence of Urinary Tract Infection in Neonates with Unexplained Hyperbilirubinemia: A Systematic Review and Meta-Analysis Study in Iran.

Iran J Public Health 2021 Jul;50(7):1311-1323

Urology and Nephrology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Background: The prevalence and risk factors of urinary tract infection (UTI) in neonates with unexplained hyperbilirubinemia are not studied thoroughly. Since the prevalence of UTI is highly variable in different areas and countries, this study aimed to review the existing data of Iranian neonates with UTI presented with unexplained hyperbilirubinemia.

Methods: This study is a meta-analysis of Iranian newborns with unexplained hyperbilirubinemia. Read More

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Management of Crigler-Najjar syndrome.

Med Pharm Rep 2021 Aug 10;94(Suppl No 1):S64-S67. Epub 2021 Aug 10.

Department of Internal Medicine, Nicolae Testemitanu State University of Medicine and Pharmacy, Chisinau, Republic of Moldova.

Crigler-Najjar syndrome is a rare autosomal recessive inherited non-hemolytic unconjugated hyperbilirubinemia caused by UDP-glucuronosyltransferase deficiency. There are two forms of this disorder. Type 1 disease is associated with severe jaundice and neurologic impairment due to bilirubin encephalopathy that can result in permanent neurologic sequelae. Read More

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