795 results match your criteria Hyperbilirubinemia Conjugated


Is "gallbladder length-to-width ratio" useful in diagnosing biliary atresia?

J Pediatr Surg 2019 Jan 23. Epub 2019 Jan 23.

Pediatric Surgery Unit, Department of Surgery, Faculty of Medicine, Prince of Songkla University, Hat Yai, 90110, Thailand.

Background: The accurate assessment of gallbladder shape and wall abnormalities by ultrasound (US) in diagnosing biliary atresia (BA) remains a subjective determination. The objective of this study was to examine the reliability of gallbladder length-to-width ratio (LTWR) by US measurement for diagnosis of BA.

Methods: One hundred infants with conjugated hyperbilirubinemia and unknown cause of jaundice who underwent transabdominal US from February 2009 to February 2017 were enrolled. Read More

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http://dx.doi.org/10.1016/j.jpedsurg.2019.01.008DOI Listing
January 2019

[Clinical features and ABCC2 genotypic analysis of an infant with Dubin-Johnson syndrome].

Zhongguo Dang Dai Er Ke Za Zhi 2019 Jan;21(1):64-70

Department of Pediatrics, First Affiliated Hospital of Jinan University, Guangzhou 510632, China.

Dubin-Johnson syndrome (DJS) is an autosomal recessive disorder resulting from biallelic mutations of ABCC2 gene, with long-term or intermittent conjugated hyperbilirubinemia being the main clinical manifestation. This paper aims to report the clinical features and ABCC2 genotypes of an infant with DJS. A 9. Read More

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January 2019
1 Read

Developing a Novel Ambulatory Total Parenteral Nutrition-Dependent Short Bowel Syndrome Animal Model.

J Surg Res 2019 Feb 27;234:13-19. Epub 2018 Sep 27.

Department of Pediatrics, Saint Louis University School of Medicine, Saint Louis, Missouri. Electronic address:

Background: Short bowel syndrome (SBS) results from extensive bowel resection. Patients with SBS require total parenteral nutrition (TPN) for survival. Understanding mechanisms contributing to TPN-associated liver injury and gut atrophy are critical in developing SBS therapies. Read More

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http://dx.doi.org/10.1016/j.jss.2018.08.042DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6291853PMC
February 2019
12 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
12 Reads

Mutation analysis of the gene in Chinese patients with Dubin-Johnson syndrome.

Exp Ther Med 2018 Nov 3;16(5):4201-4206. Epub 2018 Sep 3.

Liver Research Center, Experimental Center, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, P.R. China.

Dubin-Johnson syndrome (DJS) is a rare, autosomal recessive disorder characterized by predominantly conjugated hyperbilirubinemia, caused by a mutation in the adenosine triphosphate-binding cassette subfamily C member 2 () gene coding the multidrug resistance-associated protein 2 (MRP2) protein. mutations have been identified in patients with DJS worldwide; however, the mutation pattern of in China is not well studied. In the present study, the mutation pattern of the gene in Chinese patients with DJS was investigated. Read More

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http://www.spandidos-publications.com/10.3892/etm.2018.6682
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http://dx.doi.org/10.3892/etm.2018.6682DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6176208PMC
November 2018
7 Reads
0.940 Impact Factor

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
3 Reads

Green Teeth Related to Bilirubin Levels.

Acta Stomatol Croat 2018 Mar;52(1):61-64

Department of Pediatric Dentistry, Bauru School of Dentistry, University of Sao Paulo, Bauru, Brazil.

Objective: To describe a form of tooth pigmentation caused by serum bilirubin deposition during dental calcification in a premature child. The bilirubin levels during the three months after birth are presented. In this period the crowns of the primary teeth are still forming. Read More

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http://dx.doi.org/10.15644/asc52/1/9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6050743PMC
March 2018
3 Reads

Clinical approach to the management of Intestinal Failure Associated Liver Disease (IFALD) in adults: A position paper from the Home Artificial Nutrition and Chronic Intestinal Failure Special Interest Group of ESPEN.

Clin Nutr 2018 Dec 9;37(6 Pt A):1794-1797. Epub 2018 Jul 9.

Gastroenterology and Clinical Nutrition, CHU of Nice, University of Nice Sophia Antipolis, Nice, France.

We recommend that intestinal failure associated liver disease (IFALD) should be diagnosed by the presence of abnormal liver function tests and/or evidence of radiological and/or histological liver abnormalities occurring in an individual with IF, in the absence of another primary parenchymal liver pathology (e.g. viral or autoimmune hepatitis), other hepatotoxic factors (e. Read More

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http://dx.doi.org/10.1016/j.clnu.2018.07.006DOI Listing
December 2018
7 Reads

A Complex Case of Cholestasis in a Patient with ABCB4 and ABCB11 Mutations.

GE Port J Gastroenterol 2018 Jun 25;25(4):189-194. Epub 2017 Nov 25.

Gastroenterology Department, Hospital Professor Doutor Fernando Fonseca, Amadora, Portugal.

The low-phospholipid-associated cholelithiasis (LPAC) syndrome is a form of symptomatic cholelithiasis occurring in young adults, characterized by recurrence of symptoms after cholecystectomy and presence of hepatolithiasis. The case refers to a healthy 39-year-old Caucasian male who presented with abdominal pain and jaundice. His blood tests showed conjugated hyperbilirubinemia and elevated liver enzymes (total bilirubin 6. Read More

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http://dx.doi.org/10.1159/000484612DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6029212PMC
June 2018
20 Reads

Reduced Hepatocellular Expression of Canalicular Transport Proteins in Infants with Neonatal Cholestasis and Congenital Hypopituitarism.

J Pediatr 2018 09 20;200:181-187. Epub 2018 Jun 20.

Paediatric Liver, GI & Nutrition Centre, King's College Hospital NHS Foundation Trust, London, United Kingdom; Institute of Liver Studies, King's College London, London, United Kingdom.

Objective: To assess whether prolonged neonatal cholestasis, described in congenital hypopituitarism and septo-optic dysplasia (SOD), is associated with altered expression of selected canalicular ectoenzymes and canalicular transport proteins.

Study Design: Children with congenital hypopituitarism (n = 21), SOD (n = 18), and cholestasis seen in our center over 26 years were reviewed. Histopathologic findings in archival liver biopsy specimens were assessed (n = 10) and in those with low/normal levels of serum γ-glutamyltransferase (GGT) activity despite conjugated hyperbilirubinemia, expression of canalicular ectoenzymes and canalicular transport proteins was evaluated immunohistochemically. Read More

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http://dx.doi.org/10.1016/j.jpeds.2018.05.009DOI Listing
September 2018
3 Reads
3.790 Impact Factor

Liquid chromatography-mass spectroscopy in the diagnosis of biliary atresia in children with hyperbilirubinemia.

J Surg Res 2018 Aug 11;228:228-237. Epub 2018 Apr 11.

Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, California; Keck School of Medicine, University of Southern California, Los Angeles, California. Electronic address:

Background: Biliary atresia (BA) is difficult to distinguish from other causes of cholestasis. We evaluated the use of liquid chromatography-mass spectroscopy (LC-MS) and bile acid profiles in the rapid, noninvasive diagnosis of BA.

Materials And Methods: Following Institutional Animal Care and Use Committee and Institutional Review Board approval, we used LC-MS to measure 26 bile acids in serum and stool samples from experimental models of BA and in urine, stool, and serum samples from non-cholestatic and cholestatic human infants. Read More

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http://dx.doi.org/10.1016/j.jss.2018.03.021DOI Listing
August 2018
4 Reads
1.940 Impact Factor

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

Pediatr Res 2018 Aug 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
8 Reads

Clinical and biochemical characteristics of infants with prolonged neonatal jaundice.

Hong Kong Med J 2018 06 25;24(3):270-276. Epub 2018 May 25.

Department of Paediatrics and Adolescent Medicine, Tuen Mun Hospital, Tuen Mun, Hong Kong.

Introduction: Protocols for investigating neonatal prolonged jaundice vary and the yield from screening has not been assessed. International guidelines recommend establishing cholestasis before proceeding to investigate the underlying pathology. However, in most hospitals administered by the Hospital Authority, full liver function is checked at the first neonatal jaundice clinic visit. Read More

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http://dx.doi.org/10.12809/hkmj176990DOI Listing
June 2018
1 Read

Liver Failure and Conjugated Hyperbilirubinemia in a Preterm Neonate: Role of Early IVIG and Exchange Transfusion.

AJP Rep 2018 Apr 14;8(2):e95-e98. Epub 2018 May 14.

Department of Pediatrics, John R. Oishei Children's Hospital, Buffalo, New York.

Neonatal liver failure (NLF) is a rare diagnosis but carries with it significant risks of mortality and morbidity. Common etiologies for NLF include metabolic causes, gestational alloimmune liver disease (GALD or neonatal hemochromatosis), and viral infections. We report a case of liver failure in a premature infant with abnormal iron profile within 48 hours of birth. Read More

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http://dx.doi.org/10.1055/s-0038-1649339DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5951787PMC
April 2018
10 Reads

Pathologic Features of Hereditary Cholestatic Diseases.

Surg Pathol Clin 2018 Jun 26;11(2):313-327. Epub 2018 Mar 26.

Faculty of Medicine, University of Queensland, Herston Road, Brisbane, Queensland 4006, Australia. Electronic address:

The inherited diseases causing conjugated hyperbilirubinemia are diverse, with variability in clinical severity, histologic appearance, and time of onset. The liver biopsy appearances can also vary depending on whether the initial presentation is in the neonatal period or later. Although many of the disorders have specific histologic features in fully developed and classic cases, biopsies taken early in the disease course may be nonspecific, showing either cholestatic hepatitis or an obstructive pattern of injury requiring close correlation with the laboratory and clinical findings to reach the correct diagnosis. Read More

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http://dx.doi.org/10.1016/j.path.2018.02.001DOI Listing
June 2018
2 Reads

EBV-related Cold Agglutinin Disease Presenting With Conjugated Hyperbilirubinemia: A Pediatric Case Report and Mini Review.

J Pediatr Hematol Oncol 2018 Apr 20. Epub 2018 Apr 20.

Departments of Pediatrics.

Hemolytic anemia occurs in only 1% to 3% of hospitalized patients with infectious mononucleosis. The authors describe an 8-year-old girl without cervical lymphadenopathy or splenomegaly, who presented with conjugated hyperbilirubinemia and was diagnosed with cold agglutinin disease caused by an immunoglobulin M autoantibody with anti-i specificity. Acute Epstein-Barr virus infection was confirmed by serological and molecular methods. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001184DOI Listing
April 2018
5 Reads

Acute Isolated Hyperbilirubinemia as a Presentation of Alcoholic Liver Disease: A Case Report and Literature Review.

Case Reports Hepatol 2018 12;2018:9403934. Epub 2018 Feb 12.

Department of Medicine, Interfaith Medical Center, Brooklyn, NY, USA.

Isolated hyperbilirubinemia as a manifestation of alcoholic liver disease without significant liver abnormalities is seen very rarely. We report such a case where a patient with chronic alcoholism presented to the ER with acute jaundice with bilirubin of 24.8 mg/dl, predominantly conjugated in nature along with mild elevation of AST (76 IU/L). Read More

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http://dx.doi.org/10.1155/2018/9403934DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5829357PMC
February 2018
6 Reads

A case report of nifedipine-induced hepatitis with jaundice.

BMC Res Notes 2018 Apr 3;11(1):228. Epub 2018 Apr 3.

Department of Medicine, Faculty of Medicine, University of British Columbia, Vancouver, BC, Canada.

Background: Nifedipine is a generic, well-known and commonly-prescribed dihydropyridine calcium channel blocker used in the treatment of hypertension and Prinzmetal's angina. A known but very rare and serious adverse effect of nifedipine is clinically-apparent hepatitis which can take months to resolve.

Case Presentation: Here we present a case of nifedipine-induced hepatitis in a 78-year-old Caucasian female with no prior history of liver or autoimmune disease. Read More

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http://dx.doi.org/10.1186/s13104-018-3322-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5883361PMC
April 2018
3 Reads

Ostomy in continuity: A novel approach for the management of children with complex short bowel syndrome.

J Pediatr Surg 2018 Oct 4;53(10):1989-1995. Epub 2018 Mar 4.

Pediatric Gastroenterology, Children's National Health System, George Washington School of Medicine, Washington, DC, United States.

Introduction: Despite medical and surgical management, a subset of children with short bowel syndrome (SBS) who have discrepancy between proximal small bowel and distal colon have persistent feeding intolerance. We propose the use of an Ostomy in Continuity (OIC) (Bishop-Koop or Santulli) as a salvage procedure to decompress the proximal bowel while still maintaining maximal intestinal length, in these patients.

Methods: A retrospective chart review of 104 SBS patients identified sixteen patients who underwent an OIC. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00223468183014
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http://dx.doi.org/10.1016/j.jpedsurg.2018.02.059DOI Listing
October 2018
17 Reads

Conjugated Hyperbilirubinemia in the Neonate and Young Infant.

Authors:

Pediatr Emerg Care 2018 Apr;34(4):284-285

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http://dx.doi.org/10.1097/01.pec.0000532237.92313.0fDOI Listing
April 2018
7 Reads

Conjugated Hyperbilirubinemia in the Neonate and Young Infant.

Pediatr Emerg Care 2018 Apr;34(4):280-283

Assistant Professor of Clinical Pediatrics (Weiss), Perelman School of Medicine at the University of Pennsylvania; Attending Physician (Weiss), Division of Emergency Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA; and Attending Physician (Vora), Pediatric Emergency Medicine, Envision Physician Services, Capital Health Medical Center, Pennington, NJ.

Cholestatic jaundice in the first few weeks of life may herald potentially life-threatening pathology. It is therefore incumbent upon the pediatric practitioner to have a high index of suspicion for severe disease when investigating jaundice in a young infant. This article outlines the epidemiology, pathophysiology, differential diagnosis, and diagnostic workup for both the most common and the most severe causes of cholestasis in the neonatal period. Read More

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http://dx.doi.org/10.1097/PEC.0000000000001467DOI Listing
April 2018
5 Reads

Outcome for biliary atresia patients treated at a low-volume centre.

Scand J Gastroenterol 2018 04 16;53(4):471-474. Epub 2018 Mar 16.

c Department of Paediatrics , Oslo University Hospital , Oslo , Norway.

Objectives: The importance of case load for treatment of extrahepatic biliary atresia (BA) is debated. The aim of this study was to register results of BA treatment in a small volume centre.

Methods: Retrospective chart review study of patients with BA treated from 2000 to 2017. Read More

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http://dx.doi.org/10.1080/00365521.2018.1439097DOI Listing
April 2018
11 Reads
2.330 Impact Factor

-associated hemophagocytic syndrome: A case report.

Exp Ther Med 2018 Feb 16;15(2):1199-1202. Epub 2017 Nov 16.

Intensive Care Unit, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, Guangdong 510405, P.R. China.

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome characterized by fever, pancytopenia and splenomegaly. The underlying hemophagocytosis occurs primarily in the bone marrow, liver and lymph nodes. Multiple microbiological agents, including cytomegalovirus, Epstein-Barr virus and , have been implicated in the pathogenesis of HLH. Read More

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http://dx.doi.org/10.3892/etm.2017.5519DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5776167PMC
February 2018
5 Reads

Severe Neonatal Hyperbilirubinemia in Crigler-Najjar Syndrome Model Mice Can Be Reversed With Zinc Protoporphyrin.

Hepatol Commun 2017 Oct 10;1(8):792-802. Epub 2017 Aug 10.

Laboratory of Environmental Toxicology, Department of Pharmacology, University of California San Diego, La Jolla, CA.

Neurotoxic bilirubin is solely conjugated by UDP-glucuronosyltransferase (UGT) 1A1. Due to an inadequate function of UGT1A1, human neonates develop mild to severe physiological hyperbilirubinemia. Accumulation of bilirubin in the brain leads to the onset of irreversible brain damage called kernicterus. Read More

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http://doi.wiley.com/10.1002/hep4.1082
Publisher Site
http://dx.doi.org/10.1002/hep4.1082DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5678921PMC
October 2017
6 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
10 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
20 Reads

Alteration of Bile Acid and Cholesterol Biosynthesis and Transport by Perfluorononanoic Acid (PFNA) in Mice.

Toxicol Sci 2018 03;162(1):225-233

Department of Pharmaceutical Sciences, College of Pharmacy and Health Sciences, St. John's University, Jamaica, New York 11439.

Perfluorochemicals produce hepatotoxic effects via activation of peroxisome proliferator-activated receptor alpha (PPARα) and constitutive androstane receptor (CAR) nuclear receptors in animals. Bile formation is one major liver function. But it remains unknown whether perfluorochemicals alter metabolism of bile acids (BAs) in liver. Read More

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http://dx.doi.org/10.1093/toxsci/kfx237DOI Listing
March 2018
14 Reads

Systemic regulation of bilirubin homeostasis: Potential benefits of hyperbilirubinemia.

Hepatology 2018 04 2;67(4):1609-1619. Epub 2018 Mar 2.

Dr. Margarete Fischer-Bosch Institute of Clinical Pharmacology, Stuttgart, Germany.

Neurotoxic bilirubin is the end product of heme catabolism in mammals. Bilirubin is solely conjugated by uridine diphospho-glucuronosyltransferase 1A1, which is a membrane-bound enzyme that catalyzes the transfer of glucuronic acid. Due to low function of hepatic and intestinal uridine diphospho-glucuronosyltransferase 1A1 during the neonatal period, human neonates develop mild to severe physiological hyperbilirubinemia. Read More

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http://dx.doi.org/10.1002/hep.29599DOI Listing
April 2018
7 Reads

Conjugated hyperbilirubinemia after surgery. A diagnosis of Dubin-Johnson syndrome confirmed by genetic testing.

Rev Esp Enferm Dig 2017 Nov;109(11):801-802

Servicio de Bioquímica Clínica, Hospital Universitario Miguel Servet, España.

Dubin-Johnson syndrome is a rare benign inherited disorder, caused by mutations in ABCC2 gen, and it is characterized by predominantly conjugated hyperbilirubinemia that can be increased by intercurrent infectious illnesses or surgical procedures. We report the case of a 10 year-old patient who showed, after a surgical procedure for peritonitis due to appendicitis, jaundice and predominantly conjugated hyperbilirubinemia, and he was diagnosed with Dubin-Johnson syndrome by genetic testing. Read More

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http://dx.doi.org/10.17235/reed.2017.4989/2017DOI Listing
November 2017
10 Reads

Conjugated hyperbilirubinemia presenting in first fourteen days in term neonates.

World J Hepatol 2017 Sep;9(26):1108-1114

Pediatric Gastroenterology, Dubai Hospital, Dubai, United Arab Emirates.

Aim: To describe the etiology and characteristics of early-onset conjugated hyperbilirubinemia (ECHB) presenting within 14 d of life in term neonates.

Methods: Retrospective review was performed of term infants up to 28-d-old who presented with conjugated hyperbilirubinemia (CHB) at a tertiary center over a 5-year period from January 2010 to December 2014. CHB is defined as conjugated bilirubin (CB) fraction greater than 15% of total bilirubin and CB greater or equal to 25 μmol/L. Read More

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http://www.wjgnet.com/1948-5182/full/v9/i26/1108.htm
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http://dx.doi.org/10.4254/wjh.v9.i26.1108DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5612842PMC
September 2017
38 Reads

Bile acid profiles in neonatal intrahepatic cholestasis caused by citrin deficiency.

Clin Chim Acta 2017 Dec 4;475:28-35. Epub 2017 Oct 4.

Department of Medical Laboratory Science and Biotechnology, College of Medicine, National Cheng Kung University, Tainan, Taiwan; Department of Pathology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan 70101, Taiwan. Electronic address:

Background: Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) is characterized by conjugated hyperbilirubinemia and increased plasma bile acid concentrations. However, the underlying mechanisms remain unclear. We established a liquid chromatography tandem mass spectrometry (LC-MS/MS) method for simultaneously quantifying plasma bile acids and examined bile acid profiles in NICCD infants. Read More

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http://dx.doi.org/10.1016/j.cca.2017.10.005DOI Listing
December 2017
34 Reads

Percutaneous US-guided Cholecystocholangiography with Microbubbles for Assessment of Infants with US Findings Equivocal for Biliary Atresia and Gallbladder Longer than 1.5 cm: A Pilot Study.

Radiology 2018 03 4;286(3):1033-1039. Epub 2017 Oct 4.

From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., S.L.C., Y.H., X.Y.X.), Department of Pediatric Surgery (H.D.C.), and Department of Anesthesiology (Y.X.Q.), the First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Rd 2, Guangzhou 510080, P.R. China; and Department of Pediatric Surgery, Guangzhou Women and Children's Medical Center, Guangzhou, P.R. China (W.Z.).

Purpose To evaluate the feasibility of ultrasonographically (US) guided percutaneous cholecystocholangiography (PCC) for early exclusion of biliary atresia (BA) in infants suspected of having BA with equivocal US findings or indeterminate type of BA and a gallbladder longer than 1.5 cm at US. Materials and Methods This study was approved by the ethics committee; written informed parental consent was obtained. Read More

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http://dx.doi.org/10.1148/radiol.2017170173DOI Listing
March 2018
12 Reads

Objectification of facial color inspection to differentiate obstructive/nonobstructive jaundice in neonates by spectrophotometer.

J Pediatr Surg 2017 Dec 5;52(12):1981-1983. Epub 2017 Sep 5.

Department of Surgery, Children's Hospital of Fudan University, Shanghai, PR China.

Purpose: The purpose of this study was to study whether color difference in facial color truly exists between neonates with obstructive and nonobstructive jaundice, and whether the color difference could be objectified by spectrophotometer.

Methods: Twelve biliary atresia patients were enrolled in an obstructive jaundice group and 15 neonates admitted for non-conjugated hyperbilirubinemia in a nonobstructive group. Nine patients with syphilis (n=6) and sacrococcygeal teratoma (n=3) were studied as control. Read More

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http://dx.doi.org/10.1016/j.jpedsurg.2017.08.069DOI Listing
December 2017
6 Reads

Neonatal Graves' Disease with Maternal Hypothyroidism.

AJP Rep 2017 Jul 22;7(3):e181-e184. Epub 2017 Sep 22.

Department of Pediatrics, Children's Mercy Kansas City, Kansas City, Missouri.

Neonatal Graves' disease presenting as conjugated hyperbilirubinemia is a diagnostic challenge because the differential includes a gamut of liver and systemic diseases. We present a unique case of neonatal Graves' disease in a premature infant with conjugated hyperbilirubinemia born to a mother with hypothyroidism during pregnancy and remote history of Graves' disease. Infant was treated with a combination of methimazole, propranolol, and potassium iodide for 4 weeks. Read More

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http://dx.doi.org/10.1055/s-0037-1606365DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5610045PMC
July 2017
4 Reads

Dubin-Johnson syndrome and intrahepatic cholestasis of pregnancy in a Sri Lankan family: a case report.

BMC Res Notes 2017 Sep 18;10(1):487. Epub 2017 Sep 18.

Laboratoire Commun de Biologie et de Génétique Moléculaires, Hôpital Saint-Antoine, 184, rue du Faubourg Saint-Antoine, 75012, Paris, France.

Background: Dubin-Johnson syndrome and intrahepatic cholestasis of pregnancy are rare chronic liver disorders. Dubin-Johnson syndrome may manifest as conjugated hyperbilirubinemia, darkly pigmented liver, presence of abnormal pigment in the parenchyma of hepatocytes and abnormal distribution of the coproporphyrin isomers I and III in the urine. Intrahepatic cholestatic jaundice of pregnancy presents as pruritus, abnormal liver biochemistry and increased serum bile acids. Read More

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http://dx.doi.org/10.1186/s13104-017-2811-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5604299PMC
September 2017
17 Reads

Rare triad of periampullary carcinoid, duodenal gastrointestinal stromal tumor and plexiform neurofibroma at hepatic hilum in neurofibromatosis type 1: a case report.

BMC Cancer 2017 Aug 29;17(1):579. Epub 2017 Aug 29.

Department of pathology, Farhat Hached Hospital, Avenue Farhat Hached, 4000, Sousse, Tunisia.

Background: Neurofibromatosis type 1 is a relatively common inherited disorder. Patients with neurofibromatosis type 1 are at high risk of developing neurogenic, neuroendocrine and mesenchymal intra-abdominal tumors. Although coexistence of multiple tumors of different types is frequent in neurofibromatosis type 1, simultaneous occurrence of abdominal tumors of three types in very rare. Read More

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http://dx.doi.org/10.1186/s12885-017-3567-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5575842PMC
August 2017
18 Reads

Utility of hepatobiliary scintigraphy in diagnosing or excluding biliary atresia in premature neonates and full-term infants with conjugated hyperbilirubinemia who received parenteral nutrition.

J Matern Fetal Neonatal Med 2018 12 31;31(24):3249-3254. Epub 2017 Aug 31.

e Division of Neonatology , Children's Hospital Los Angeles, Keck School of Medicine of University of Southern California , Los Angeles , CA , USA.

Purpose: Hepatobiliary scintigraphy (HBS) is used to aid in the diagnosis of Biliary Atresia in full-term infants with conjugated hyperbilirubinemia. There is little information on the utility of the HBS in premature infants with conjugated hyperbilirubinemia and infants with parenteral nutrition associated cholestasis (PNAC). The purpose of this study is to assess the utility of HBS in differentiating Biliary Atresia and PNAC in premature neonates and full-term infants who received parenteral nutrition (PN). Read More

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http://dx.doi.org/10.1080/14767058.2017.1368479DOI Listing
December 2018
11 Reads

I index is not an accurate indicator of icteria in conjugated hyperbilirubinemia.

Clin Chim Acta 2017 Oct 12;473:32-34. Epub 2017 Aug 12.

Department of Clinical Chemistry, Sestre milosrdnice University Hospital Center, Zagreb, Croatia.

Introduction: Serum indices have become a standard in assessing degree of endogenous interferences in serum and plasma samples. The aim of this study was to evaluate accuracy of I index in comparison with total bilirubin concentration in icteric samples with ranging amount of conjugated bilirubin.

Materials And Methods: This study retrospectively analyzed data from laboratory information system. Read More

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http://dx.doi.org/10.1016/j.cca.2017.08.013DOI Listing
October 2017
5 Reads

Eccrine Chromhidrosis in an Adolescent with Sickle Cell Disease.

Pediatr Dermatol 2017 Sep 30;34(5):e273-e274. Epub 2017 Jul 30.

School of Medicine, Duke University, Durham, North Carolina.

Eccrine chromhidrosis can occur secondary to hyperbilirubinemia. We report an adolescent with sickle cell disease who presented with eccrine chromhidrosis on his palmar and plantar surfaces. He had extremely high levels of conjugated bilirubin but no fever. Read More

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http://doi.wiley.com/10.1111/pde.13201
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http://dx.doi.org/10.1111/pde.13201DOI Listing
September 2017
8 Reads

Identification of a compound heterozygous mutation of ABCC2 in a patient with hyperbilirubinemia.

Mol Med Rep 2017 Sep 5;16(3):2830-2834. Epub 2017 Jul 5.

Department of Endocrinology, The Third Xiangya Hospital of Central South University, Changsha, Hunan 410013, P.R. China.

Bilirubin is the end product of heme catabolism, which is produced primarily from the breakdown of erythrocyte hemoglobin in the reticuloendothelial system. Hyperbilirubinemia is induced not only by increased bilirubin synthesis, but can also be caused by decreased bilirubin clearance. There are several disorders, which can contribute to hyperbilirubinemia, including Dubin‑Johnson syndrome (DJS). Read More

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http://dx.doi.org/10.3892/mmr.2017.6926DOI Listing
September 2017
43 Reads

Role of Hepatobiliary Scintigraphy and Preoperative Liver Biopsy for Exclusion of Biliary Atresia in Neonatal Cholestasis Syndrome.

Indian J Pediatr 2017 Sep 8;84(9):685-690. Epub 2017 Jul 8.

Department of Pediatric Surgical Superspecialties, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh, 226014, India.

All diagnostic algorithms for Neonatal Cholestasis Syndrome (NCS) focus on differentiating numerous medical causes from Biliary Atresia (BA). No preoperative diagnostic algorithm has 100% diagnostic accuracy for BA and yet, timely diagnosis is crucial to optimize surgical outcome. Markers for high index of clinical suspicion for BA are: a "usually" well thriving infant with conjugated hyperbilirubinemia, raised gamma glutamyl transpeptidase, persistently "acholic" stools, firm hepatomegaly with dysmorphic, hypoplastic gall bladder. Read More

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http://dx.doi.org/10.1007/s12098-017-2408-zDOI Listing
September 2017
19 Reads

Neonatal Hyperthyroidism with Fulminant Liver Failure: A Case Report.

J Clin Diagn Res 2017 Apr 1;11(4):SD01-SD02. Epub 2017 Apr 1.

Paediatric Consultant, Department of Paediatrics, King Saud Bin Abdulaziz University for Health Sciences, National Guard Hospital, Riyadh, Saudi Arabia.

Neonatal hyperthyroidism is a rare disease that is seen in infants born to mothers with Graves' disease. Hepatic manifestation of neonatal hyperthyroidism is extremely rare. We describe a neonate with fulminant liver failure secondary to neonatal hyperthyroidism caused by maternal Graves's disease. Read More

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http://dx.doi.org/10.7860/JCDR/2017/21503.9641DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5449869PMC
April 2017
17 Reads

Bile Cast Nephropathy in Cirrhotic Patients: Effects of Chronic Hyperbilirubinemia.

Am J Clin Pathol 2017 May;147(5):525-535

Department of Pathology.

Objectives: The aim of this study was to determine the prevalence of bile cast nephropathy (BCN) in autopsied cirrhotic patients and to correlate BCN with clinical and laboratory data to direct attention to this underrecognized renal complication of liver failure.

Methods: We assessed 114 autopsy cases of cirrhosis for the presence of renal intratubular bile casts using Hall stain for bile. Presence of bile casts was correlated with etiology of cirrhosis, clinical and laboratory data, and histologic findings. Read More

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http://dx.doi.org/10.1093/ajcp/aqx030DOI Listing
May 2017
15 Reads

A Challenging Case of Severe Infantile Cholestasis in Alpha-1 Antitrypsin Deficiency.

Pediatr Dev Pathol 2017 Mar-Apr;20(2):176-181. Epub 2017 Jan 25.

2 Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.

Jaundice in the newborn period can be physiologic and is often due to benign causes. Jaundice due to conjugated hyperbilirubinemia extending beyond the second week of life may be an early sign of several cholestatic or metabolic liver diseases, and it requires logical and timely analysis so that specific treatments can be initiated. Alpha-1 antitrypsin deficiency is the most common genetic cause of pediatric liver disease and transplantation, and it must be considered when evaluating cholestatic infants. Read More

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http://dx.doi.org/10.1177/1093526616686259DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4977215PMC
January 2017
9 Reads

Curated human hyperbilirubinemia data and the respective OATP1B1 and 1B3 inhibition predictions.

Data Brief 2017 Apr 10;11:204-207. Epub 2017 Feb 10.

University of Vienna, Department of Pharmaceutical Chemistry, Althanstrasse 14, 1090 Vienna, Austria.

Hyperbilirubinemia is a pathological condition, very often indicative of underlying liver condition that is characterized by excessive accumulation of conjugated or unconjugated bilirubin in sinusoidal blood. In literature there are several indications associating the inhibition of the basolateral hepatic transporters Organic anion transporting polypeptide 1B1 and 1B3 (OATP1B1 and 1B3) with hyperbilirubinemia. In this article, we present a curated human hyperbilirubinemia dataset and the respective OATP1B1 and 1B3 inhibition predictions obtained from an effort to generate a classification model for hyperbilirubinemia. Read More

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http://dx.doi.org/10.1016/j.dib.2017.02.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5320065PMC
April 2017
9 Reads

Evaluation of Jaundice in Adults.

Am Fam Physician 2017 Feb;95(3):164-168

Uniformed Services University of the Health Sciences, Bethesda, MD, USA.

Jaundice in adults can be an indicator of significant underlying disease. It is caused by elevated serum bilirubin levels in the unconjugated or conjugated form. The evaluation of jaundice relies on the history and physical examination. Read More

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February 2017
33 Reads

[Two Cases of Systemic Steroid Therapy for Hyperbilirubinemia after Right Hepatic Lobectomy of Liver Metastasis from Colorectal Cancer].

Gan To Kagaku Ryoho 2016 Nov;43(12):1739-1741

Dept. of Surgery, Japan Community Health care Organization(JCHO) Osaka Hospital.

We report 2 cases of systemic steroid therapy for hyperbilirubinemia after right hepatic lobectomy for liver metastases from colorectal cancer.One of the patients was a 65-year-old man, diagnosed with transverse colon cancer with multiple liver metastases.After surgery for the primary lesion and chemotherapy(FOLFOX plus bevacizumab, FOLFIRI plus panitumumab), he underwent right hepatic lobectomy for the liver metastasis. Read More

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November 2016
5 Reads

Liver stiffness measurements with supersonic shear wave elastography in the diagnosis of biliary atresia: a comparative study with grey-scale US.

Eur Radiol 2017 Aug 12;27(8):3474-3484. Epub 2017 Jan 12.

Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, the First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou, 510080, People's Republic of China.

Objectives: To prospectively assess the diagnostic performance of supersonic shear wave elastography (SSWE) in identifying biliary atresia (BA) among infants with conjugated hyperbilirubinaemia by comparing this approach with grey-scale ultrasonography (US).

Methods: Forty infants were analysed as the control group to determine normal liver stiffness values. The use of SSWE values for identifying BA was investigated in 172 infants suspected of having BA, and results were compared with the results obtained by grey-scale US. Read More

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http://dx.doi.org/10.1007/s00330-016-4710-yDOI Listing
August 2017
10 Reads
4.010 Impact Factor