845 results match your criteria Hyperbilirubinemia Conjugated


Prolonged cholestasis after acute viral hepatitis: successfully treated with oral steroid.

BMJ Case Rep 2020 May 21;13(5). Epub 2020 May 21.

Pediatrics, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Cholestasis is a rare distinct complication of hepatitis A infection. Usually it runs a long indolent course with significant pruritus and malabsorption lasting for few months. A 9-year-old boy presented with yellowish discolouration of eye for 1 month. Read More

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http://dx.doi.org/10.1136/bcr-2020-234430DOI Listing

Elucidation of OATP1B1 and 1B3 transporter function using transgenic rodent models and commonly known single nucleotide polymorphisms.

Toxicol Appl Pharmacol 2020 Jul 11;399:115039. Epub 2020 May 11.

Department of Pharmacology, Toxicology and Therapeutics, School of Medicine, University of Kansas, Kansas City, KS, United States of America.

The clearance of many drugs from the blood into the liver, such as the statins, is dependent on the organic anion transporting polypeptides (OATPs). Patients with *5 and *15 polymorphisms of OATP1B1 remove less of the statin as it traverses the liver and thus more reaches the rest of the body, including the skeletal muscle where it can cause myalgia, myopathy, and rhabdomyolysis. OATP1B1 polymorphisms also affect the pharmacokinetics of anticancer drugs (methotrexate, taxanes, and doxorubicin) and numerous anti-hypertensive drugs. Read More

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http://dx.doi.org/10.1016/j.taap.2020.115039DOI Listing

Risk factors and outcomes for hyperbilirubinaemia after heart surgery in children.

Cardiol Young 2020 Jun 5;30(6):761-768. Epub 2020 May 5.

Nicklaus Children's Hospital, Miami, FL, USA.

Introduction: Liver dysfunction, associated with morbidity and mortality, is common in patients with CHD. We investigate risk factors for and outcomes of hyperbilirubinaemia in neonates and infants after cardiac surgery.

Materials And Methods: In a retrospective analysis of neonates and infants undergoing cardiac surgery at our institution between January 2013 and December 2017, we identified those with post-operative conjugated hyperbilirubinaemia. Read More

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http://dx.doi.org/10.1017/S1047951120000967DOI Listing

Liver transplantation for Gaucher disease presenting as neonatal cholestasis: Case report and literature review.

Pediatr Transplant 2020 Jun 23;24(4):e13718. Epub 2020 Apr 23.

Department of Pediatrics, Kingston Health Sciences Center, Queen's University, Kingston, ON, Canada.

Background: We present a rare case of neonatal cholestasis in a female infant with Gaucher Disease (GD), who received liver transplantation. We review the relevant literature on similar disease presentations.

Methods: A chart review of the index case was performed. Read More

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http://dx.doi.org/10.1111/petr.13718DOI Listing

An Infant with Severe Anemia and Hypoalbuminemia.

Indian Pediatr 2020 Apr;57(4):349-355

Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

We discuss the case of a two-month-old girl admitted with complaints of progressive pallor, generalized body swelling and pale colored stool since the neonatal period. On examination, severe pallor, chubby cheeks and moderate hepatomegaly were noted. Investigations revealed isolated anemia, transaminitis, conjugated hyperbilirubinemia, prolonged prothrombin time and hyperlipidemia. Read More

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April 2020
1.014 Impact Factor

Acute liver failure due to DGUOK deficiency-is liver transplantation justified?

Clin Res Hepatol Gastroenterol 2020 Apr 8. Epub 2020 Apr 8.

Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, Children's Memorial Health Institute, Al. Dzieci Polskich 20, Warsaw, Poland.

Background: Deoxyguanosine kinase (DGUOK) deficiency is one of the causes of the hepatocerebral form of mitochondrial depletion syndrome (MDS). It is characterized by an early onset of liver failure with concomitant neurological deterioration. In the current literature, there are only few reports regarding long-term observation of children with DGUOK deficiency. Read More

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http://dx.doi.org/10.1016/j.clinre.2020.02.018DOI Listing

Rapid Identification of Biallelic Mutation in a Neonate with Severe Congenital Hemolytic Anemia and Liver Failure.

Mol Syndromol 2020 Feb 1;11(1):50-55. Epub 2020 Feb 1.

Victorian Clinical Genetics Services, Murdoch Children's Research Institute, Melbourne, Australia.

Heterozygous pathogenic variants in cause autosomal dominant hereditary spherocytosis, an important cause of neonatal nonimmune hemolytic anemia. Biallelic mutations are rarely reported, all with severe neonatal presentation. We describe rapid (68 h) genomic diagnosis of homozygous β-spectrin deficiency in a newborn with severe transfusion-dependent hemolytic anemia, conjugated hyperbilirubinemia, and progressive liver failure. Read More

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http://dx.doi.org/10.1159/000505886DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7109415PMC
February 2020

Vancomycin prevents fermentable fiber-induced liver cancer in mice with dysbiotic gut microbiota.

Gut Microbes 2020 Jul 30;11(4):1077-1091. Epub 2020 Mar 30.

Microbiome Consortium, Center for Hypertension and Precision Medicine, University of Toledo College of Medicine and Life Sciences , Toledo, OH, USA.

Owing to their health benefits, dietary fermentable fibers, such as refined inulin, are increasingly fortified in processed foods to enhance their nutritional value. However, we previously demonstrated that when inulin was fed to Toll-like receptor 5 deficient (T5KO) mice susceptible to dysbiosis, a subset of them developed cholestasis and subsequently liver cancer in a gut microbiota-dependent manner. Therefore, we hypothesized that clearance of bacterial taxa, and thereby gut metabolites, involved in the onset and progression to liver cancer could abate the disease in these mice. Read More

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http://dx.doi.org/10.1080/19490976.2020.1743492DOI Listing

Co-existence of ABCB11 and DCDC2 disease: Infantile cholestasis requires both next-generation sequencing and clinical-histopathologic correlation.

Eur J Hum Genet 2020 Jun 20;28(6):840-844. Epub 2020 Mar 20.

Department of Pediatrics, Medical University of Innsbruck, Innsbruck, Austria.

A boy exhibiting conjugated hyperbilirubinemia from birth, with elevated serum gamma-glutamyl transpeptidase activity (GGT), developed liver failure unusually early (7mo); GGT concomitantly normalized. ABCB4 disease was suspected, but no ABCB4 lesion was found. The boy was instead homozygous for ABCB11 variant c. Read More

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http://dx.doi.org/10.1038/s41431-020-0613-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7253416PMC

A recurrent ABCC2 p.G693R mutation resulting in loss of function of MRP2 and hyperbilirubinemia in Dubin-Johnson syndrome in China.

Orphanet J Rare Dis 2020 Mar 18;15(1):74. Epub 2020 Mar 18.

Liver Research Center, Experimental Center, Beijing Friendship Hospital, Capital Medical University, 95 Yong-An Road, Beijing, 100050, China.

Background: Dubin-Johnson syndrome (DJS) is a rare autosomal recessive disorder characterized by predominantly conjugated hyperbilirubinemia that is caused by pathogenic mutations in the adenosine triphosphate-binding cassette subfamily C member 2 (ABCC2) gene, which encodes multidrug resistance-associated protein 2 (MRP2). However, little is known about the causative mutation of DJS in China. Recently, we have reported ABCC2 p. Read More

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http://dx.doi.org/10.1186/s13023-020-1346-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7079413PMC

Insertion of LINE-1 Retrotransposon Inducing Exon Inversion Causes a Rotor Syndrome Phenotype.

Front Genet 2019 31;10:1399. Epub 2020 Jan 31.

Experimental Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China.

Rotor syndrome, a rare autosomal-recessive genetic disorder characterized by conjugated hyperbilirubinemia, is caused by biallelic pathogenic variants in both and genes. Long interspersed nuclear elements (LINEs) make up about 17% of the human genome and insertion of LINE-1 in genes can result in genetic diseases. In the current study, we examined and genes in two Chinese patients diagnosed with Rotor syndrome based on laboratory tests. Read More

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http://dx.doi.org/10.3389/fgene.2019.01399DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7005217PMC
January 2020

MicroRNA 122 Reflects Liver Injury in Children with Intestinal Failure-Associated Liver Disease Treated with Intravenous Fish Oil.

J Nutr 2020 May;150(5):1144-1150

Department of Pediatrics, Neonatal Research Center of the UCLA Children's Discovery and Innovation Institute, David Geffen School of Medicine UCLA, and UCLA Mattel Children's Hospital, Los Angeles, CA, USA.

Background: There is evidence that microRNA (MIR) 122 is a biomarker for various liver diseases in adults and children. To date, MIR122 has not been explored in children with intestinal failure-associated liver disease (IFALD, or hyperbilirubinemia associated with prolonged parenteral nutrition).

Objectives: This study's purpose was to investigate changes in plasma miR-122, correlate miR-122 with serum liver function tests and enzymes, and investigate changes in whole blood transcripts including miR-122 targets in a group of children with IFALD who received pure intravenous fish oil (FO) as a treatment for cholestasis. Read More

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http://dx.doi.org/10.1093/jn/nxaa001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7198298PMC

Evaluation of Home Phototherapy for Neonatal Hyperbilirubinemia.

J Pediatr 2020 May 14;220:80-85. Epub 2020 Feb 14.

Department of Pediatrics, University of Washington, Seattle, WA.

Objective: To characterize home phototherapy treatment for neonatal hyperbilirubinemia and assess the risk factors associated with the need for hospital admission during or after home phototherapy.

Study Design: This was a retrospective study of newborn infants born at ≥35 weeks of gestation who underwent comprehensive home phototherapy (that included daily in-home lactation support and blood draws) over an 18-month period. We excluded infants who lacked a recorded birth date or time, started treatment at age >14 days, or had a conjugated serum bilirubin level of ≥2 mg/dL (≥34. Read More

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http://dx.doi.org/10.1016/j.jpeds.2020.01.004DOI Listing

Dubin-Johnson Syndrome Presenting During Cardiac Transplantation Evaluation.

Cureus 2020 Jan 8;12(1):e6594. Epub 2020 Jan 8.

Internal Medicine: Gastroenterology, Tufts Medical Center, Boston, USA.

Dubin-Johnson syndrome is a rare, benign disorder that results in conjugated hyperbilirubinemia. The disease manifests as intermittent jaundice without long-term hepatic or other clinical complications. This article reports a case of Dubin-Johnson syndrome, which was identified during cardiac transplant evaluation for cardiomyopathy secondary to a polyglycogen storage disease. Read More

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http://dx.doi.org/10.7759/cureus.6594DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7003716PMC
January 2020

Case 2: Conjugated Hyperbilirubinemia in a Late Preterm Neonate.

Neoreviews 2020 02;21(2):e123-e126

Division of Neonatology, Department of Pediatrics, LAC+USC Medical Center & Children's Hospital Los Angeles, Keck School of Medicine of USC, Los Angeles, CA.

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http://dx.doi.org/10.1542/neo.21-2-e123DOI Listing
February 2020

A novel timesaving and semiquantitative method for radionuclide hepatobiliary scintigraphy for suspected biliary atresia.

Ann Palliat Med 2020 Jan;9(1):63-69

Department of Nuclear Medicine, the First Affiliated Hospital of Guangxi Medical University, Nanning 530021, China.

Background: To optimize the performance of the hepatobiliary scintigraphy (HS) for suspected biliary atresia (BA) using a timesaving and semiquantitative method without a loss in diagnostic accuracy.

Methods: A retrospective analysis of 185 patients with persistent jaundice who underwent surgery were included. According to the surgical evaluation and pathological diagnosis, patients were divided into a BA group (99 cases) and an infant hepatitis syndrome (IHS) group (86 cases). Read More

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http://dx.doi.org/10.21037/apm.2019.12.07DOI Listing
January 2020

[Consensus on hyperbilirubinemia of the first trimester of life].

Authors:

Arch Argent Pediatr 2020 02;118(1):S12-S49

Neonatal jaundice may be due to different causes, ranging from physiological conditions to severe diseases. In term neonates with persistent jaundice beyond 14 days of life, it should be determined whether hyperbilirubinemia is unconjugated or conjugated, in order to study the etiology and start early treatment. In the majority of cases, conjugated hyperbilirubinemia (cholestasis) is a sign of liver dysfunction possibly associated with alterations in the bile flow secondary to structural or molecular abnormalities of the liver and/or the biliary tract. Read More

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http://dx.doi.org/10.5546/aap.2020.S12DOI Listing
February 2020

Neurocognitive Functioning in Early School-age Children With Intestinal Failure.

J Pediatr Gastroenterol Nutr 2020 Feb;70(2):225-231

Transplant and Regenerative Medicine Centre.

Objectives: Little is known about school-age functioning in children with intestinal failure (IF). This study examines neurocognitive outcomes of children with IF at ages 4 to 8 years treated at a single centre, along with relevant medical and demographic variables.

Methods: Between 2012 and 2016, neurocognitive assessments were administered to 28 children receiving treatment in our IF rehabilitation program, and included measures of intelligence, academics, learning/memory, language, visual-motor integration, and fine-motor dexterity. Read More

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http://dx.doi.org/10.1097/MPG.0000000000002500DOI Listing
February 2020

Individualized Asparaginase Dosing in Childhood Acute Lymphoblastic Leukemia.

J Clin Oncol 2020 Mar 10;38(7):715-724. Epub 2020 Jan 10.

Pediatric Oncology and Hematology, Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands.

Purpose: In the DCOG ALL-11 protocol, polyethylene glycol-conjugated asparaginase (PEGasparaginase) and asparaginase treatment of pediatric acute lymphoblastic leukemia are individualized with therapeutic drug monitoring (TDM). The efficacy of TDM and its effect on asparaginase-associated toxicity are reported.

Patients And Methods: After induction with 3 fixed intravenous doses of 1,500 IU/m PEGasparaginase, medium-risk patients (n = 243) received 14 individualized doses that targeted trough levels of 100-250 IU/L, standard-risk patients (n = 108) received 1 individualized dose, and high-risk patients (n = 18) received 2-5 fixed administrations (1,500 IU/m). Read More

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http://dx.doi.org/10.1200/JCO.19.02292DOI Listing

Extreme neonatal hyperbilirubinemia and kernicterus spectrum disorder in Denmark during the years 2000-2015.

J Perinatol 2020 02 6;40(2):194-202. Epub 2020 Jan 6.

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

Objective: To determine the incidence and etiology of extreme neonatal hyperbilirubinemia, defined as total serum bilirubin (TSB) ≥450 µmol/L, and kernicterus spectrum disorder (KSD) in Denmark between 2000 and 2015.

Study Design: We identified all infants born between 01.01. Read More

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http://dx.doi.org/10.1038/s41372-019-0566-8DOI Listing
February 2020

Interference of hemolysis, hyperlipidemia, and icterus on plasma infrared spectral profile.

Anal Bioanal Chem 2020 Feb 19;412(4):805-810. Epub 2019 Dec 19.

Université de Reims Champagne-Ardenne, BioSpecT EA7506, UFR de Pharmacie, 51097, Reims, France.

Recently, pre-analytical, analytical, and post-analytical issues have been addressed to implement biofluid FTIR spectroscopy as a novel diagnostic tool in the clinical setting. Although hemolysis, icterus, and hyperlipidemia are known to interfere with colorimetric and turbidimetric biochemical methods, there are no data on their impact on serum/plasma FTIR spectra. This study aimed at investigating the impact of hemoglobin, bilirubin, and triglycerides concentrations on plasma spectral analysis. Read More

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http://dx.doi.org/10.1007/s00216-019-02312-0DOI Listing
February 2020

Neonatal cholestasis, hyperferritinemia, hypoglycemia and deafness: a diagnostic challenge.

BMJ Case Rep 2019 Dec 1;12(11). Epub 2019 Dec 1.

Pediatrics, Maastricht University Medical Centre+, Maastricht, Limburg, The Netherlands

Neonatal conjugated hyperbilirubinemia is a diagnostic challenge. A full term, small for gestational age boy presented with cholestasis, hypoglycemia, hyperferritinemia and severe bilateral deafness. Diagnostic work-up revealed two hereditary diseases: alpha-1-antitrypsin deficiency (PI*ZZ genotype) and autosomal recessive deafness type 3 (compound heterozygous MYO15A gene mutation). Read More

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http://dx.doi.org/10.1136/bcr-2019-231978DOI Listing
December 2019

Embryonal rhabdomyosarcoma of the biliary tree in a paediatric patient - A rare cause of obstructive jaundice.

SA J Radiol 2019 10;23(1):1662. Epub 2019 Jan 10.

Department of Radiology, University of the Witwatersrand, South Africa.

Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in the paediatric age group, ranking fourth in frequency after central nervous system tumours, neuroblastomas and nephroblastomas. Embryonal RMS of the biliary tree is considered a rare entity, with the most common clinical presentation being that of obstructive jaundice. We present the case of a 4-year-old boy who presented with hepatomegaly and obstructive jaundice. Read More

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http://dx.doi.org/10.4102/sajr.v23i1.1662DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6837790PMC
January 2019

The impact of bilirubin ditaurate on platelet quality during storage.

Platelets 2019 Nov 20:1-13. Epub 2019 Nov 20.

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

Bilirubin ditaurate (BRT), a conjugated bilirubin analogue, has demonstrated anti-platelet characteristics following acute exposure. Scavenging of mitochondrial superoxide and attenuation of granule exocytosis suggested a potential benefit for including BRT for storage. With no reports of cytotoxicity following acute exposure, the impact of 35µM BRT on platelet function was investigated, in clinically suppled units, for up to seven days. Read More

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http://dx.doi.org/10.1080/09537104.2019.1693038DOI Listing
November 2019

Incidence and predictors of treatment-related conjugated hyperbilirubinemia during early treatment phases for children with acute lymphoblastic leukemia.

Pediatr Blood Cancer 2020 02 17;67(2):e28063. Epub 2019 Nov 17.

Section of Hematology and Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.

Conjugated hyperbilirubinemia (CHB) and liver transaminase elevation are known complications of acute lymphoblastic leukemia (ALL) therapy, but host risk factors are poorly understood. Among 373 children diagnosed with ALL between 2011 and 2016, clinically significant CHB and transaminase elevation were observed in 15 (4.0%) and 12 (3. Read More

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http://dx.doi.org/10.1002/pbc.28063DOI Listing
February 2020
2.562 Impact Factor

A Triplication of 6q24 and Meconium Pseudocyst: A Case Report.

Neonatal Netw 2019 Nov;38(6):329-335

With the rise in genetic screening both pre- and postnatally, new variances in genes are being recognized. Some are of unknown significance, while other known genetic expressions have obvious phenotypical expressions. Transient neonatal diabetes mellitus is a result of the duplication of chromosome 6q24, but little is known about the phenotypic expression of a triplication of chromosome 6q24. Read More

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http://dx.doi.org/10.1891/0730-0832.38.6.329DOI Listing
November 2019

Primary Pancreatic Neuroblastoma in an Infant.

J Pediatr Hematol Oncol 2019 Oct 29. Epub 2019 Oct 29.

Marshfield Medical Center.

A 2-month-old girl with conjugated hyperbilirubinemia was found at the surgery and by computed tomography to have a large mass originating in the pancreas. Histopathology, molecular testing, and staging evaluations showed this to be a stage 3, MYCN unamplified, intermediate-risk neuroblastoma. The patient had a partial response to risk-stratified chemotherapy. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001629DOI Listing
October 2019
1 Read

A Comparison of Smoflipid® and Intralipid® in the Early Management of Infants with Intestinal Failure.

J Pediatr Surg 2020 Jan 25;55(1):153-157. Epub 2019 Oct 25.

Division of Pediatric Surgery, University of British Columbia/BC Children's Hospital, Vancouver, BC, Canada. Electronic address:

Purpose: Cholestasis is problematic for infants with intestinal failure (IF). The soy-based lipid Intralipid® (IL) has been implicated. An alternative, Smoflipid® (SMOF), is increasingly used. Read More

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http://dx.doi.org/10.1016/j.jpedsurg.2019.09.073DOI Listing
January 2020

Reductive effect of ursodeoxycholic acid on bilirubin levels in neonates on phototherapy.

Clin Exp Gastroenterol 2019 29;12:349-354. Epub 2019 Jul 29.

Department of Paediatrics, University of Nigeria Teaching Hospital, Enugu, Nigeria.

Background: Phototherapy is paramount in the management of high total serum bilirubin (TSB). Whether its effectiveness can be improved with ursodeoxycholic acid (UDCA) has not been evaluated among newborns of African descent.

Methods: A double-blind-controlled study was used to evaluate the effect of UDCA on the management of high TSB in neonates. Read More

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http://dx.doi.org/10.2147/CEG.S207523DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6681162PMC
July 2019
2 Reads

Transient Myeloproliferative Disorder: An Update for Neonatal Nurses.

Neonatal Netw 2019 May 20;38(3):144-150. Epub 2019 May 20.

Down syndrome (DS) is a well-known genetic disorder that affects 700-1,000 infants per year. One particular comorbidity of DS is transient myeloproliferative disorder (TMD), a disease characterized by leukocytosis with elevated blast counts. Approximately 10 percent of DS infants develop TMD, which usually manifests during the first week of life and can lead to an extended hospitalization in a NICU. Read More

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http://dx.doi.org/10.1891/0730-0832.38.3.144DOI Listing

A practical approach to the pathology of neonatal cholestatic liver disease.

Semin Diagn Pathol 2019 Nov 26;36(6):375-388. Epub 2019 Jul 26.

Department of Pathology, University of California San Francisco, San Francisco, CA United States. Electronic address:

Navigating the complexities of interpreting a liver biopsy performed on a neonate with conjugated/direct hyperbilirubinemia can be an arduous task given these biopsies are infrequently encountered. The list of entities is long and yet there are only a few histologic patterns of liver injury. The first step for the pathologist is to determine the histologic pattern, which will guide further inquiry into the useful clinical information to have while evaluating the biopsy. Read More

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http://dx.doi.org/10.1053/j.semdp.2019.07.004DOI Listing
November 2019
1 Read

Mevalonate kinase deficiency masked by cytomegalovirus infection and obscure liver disease.

Clin Chim Acta 2019 Nov 17;498:122-125. Epub 2019 Aug 17.

Department of Chemical Pathology, Faculty of Health Sciences, University of Pretoria and National Health Laboratory Service (NHLS) Tshwane Academic Division, Pretoria, South Africa; Division of Chemical Pathology, University of Cape Town, South Africa. Electronic address:

Background: Chronic liver disease with conjugated hyperbilirubinaemia and failure to thrive can have multifactorial aetiologies. Investigations can be complex and difficult especially when obscured by a viral infection affecting liver function.

Methods: A 5 month old male infant was referred for investigation of chronic liver disease and a history of jaundice with multiple febrile episodes. Read More

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http://dx.doi.org/10.1016/j.cca.2019.08.014DOI Listing
November 2019
2 Reads

Circadian Clock Gene Bmal1 Regulates Bilirubin Detoxification: A Potential Mechanism of Feedback Control of Hyperbilirubinemia.

Theranostics 2019 9;9(18):5122-5133. Epub 2019 Jul 9.

Reserach Center for Biopharmaceutics and Pharmacokinetics, College of Pharmacy, Jinan University, 601 Huangpu Avenue West, Guangzhou 510632, China.

Controlling bilirubin to a low level is necessary in physiology because of its severe neurotoxicity. Therefore, it is of great interest to understand the regulatory mechanisms for bilirubin homeostasis. In this study, we uncover a critical role for circadian clock in regulation of bilirubin detoxification and homeostasis. Read More

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http://www.thno.org/v09p5122.htm
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http://dx.doi.org/10.7150/thno.35773DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6691581PMC
July 2019
1 Read
7.827 Impact Factor

Impact of Cytokine Adsorption Treatment in Liver Failure.

Transplant Proc 2019 Sep 9;51(7):2420-2424. Epub 2019 Aug 9.

Department of Anesthesiology and Reanimation, Surgical Intensive Care Unit, Şişli Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey.

Sepsis causes life-threatening organ dysfunction and is the leading cause of morbidity and mortality in critically ill patients worldwide. Mortality rate of sepsis is close to 30% to 50% despite better understanding of the pathophysiology of sepsis, and advances in antimicrobial therapy, resuscitation strategies, and mechanical ventilation. Liver failure is characterized by accumulation of potentially toxic substances in the systemic circulation of the patient. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00411345183171
Publisher Site
http://dx.doi.org/10.1016/j.transproceed.2019.01.167DOI Listing
September 2019
4 Reads

Amount of Cycloserine Emanating from Terizidone Metabolism and Relationship with Hepatic Function in Patients with Drug-Resistant Tuberculosis.

Drugs R D 2019 Sep;19(3):289-296

School of Pharmacy, University of the Western Cape, Private Bag X17, Bellville, Cape Town, 7535, South Africa.

Background And Objectives: The dosing of cycloserine and terizidone is the same, as both drugs are considered equivalent or used interchangeably. Nevertheless, it is not certain from the literature that these drugs are interchangeable. Therefore, the amount of cycloserine resulting from the metabolism of terizidone and the relationship with hepatic function were determined. Read More

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http://dx.doi.org/10.1007/s40268-019-00281-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6738357PMC
September 2019
4 Reads

[An infant with prolonged jaundice].

Ned Tijdschr Geneeskd 2019 07 23;163. Epub 2019 Jul 23.

Amsterdam UMC, afd. Kindermaag-, darm- en leverziekten, Amsterdam.

Background: In every neonate presenting with prolonged jaundice persisting beyond day 21 of life, neonatal cholestasis should always be excluded even if the infant is breast fed. Pale stools are an alarm symptom and additional tests for neonatal cholestasis should be carried out directly.

Case Description: We describe the case of a five-week-old girl of Chilean origin who was referred with conjugated hyperbilirubinaemia. Read More

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July 2019
6 Reads

Nineteenth-Century Homeopathic Repertories Predict Increased Urinary Excretion of Bile in Cholestasis but Not in Non-Cholestatic Infant Jaundice.

Authors:
Janice Block

Homeopathy 2019 11 22;108(4):270-276. Epub 2019 Jul 22.

Merkaz HaBriut, Center for Integrative Medicine, Ramat Beit Shemesh, Israel.

Introduction: There are two types of bilirubin: conjugated bilirubin, prevalent in cholestatic jaundice, and unconjugated bilirubin, prevalent in hematologic jaundice. Conjugated bilirubin is water soluble and is excreted in urine, whereas unconjugated bilirubin is neither water soluble nor excreted in urine. Homeopathic repertories published prior to the discovery of the two types of bilirubin in 1913 present an opportunity to test the reliability of homeopathic repertories and associated materia medica. Read More

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http://dx.doi.org/10.1055/s-0039-1691835DOI Listing
November 2019
2 Reads

Biliary atresia combined with progressive familial intrahepatic cholestasis type 3: A case report and review of the literature.

Medicine (Baltimore) 2019 May;98(19):e15593

Department of Pediatrics.

Rationale: Neonatal cholestasis is one of the most serious diseases in infancy. Progressive familial intrahepatic cholestasis (PFIC) is a disease that leads to intrahepatic cholestasis. It is one of the common causes of neonatal cholestasis in addition to biliary atresia (BA). Read More

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http://dx.doi.org/10.1097/MD.0000000000015593DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6531222PMC
May 2019
19 Reads

A Case of Dubin-Johnson Syndrome in Pregnancy.

Cureus 2019 Feb 11;11(2):e4048. Epub 2019 Feb 11.

Obstetrics & Gynecology, Maulana Azad Medical College, New Delhi, IND.

Dubin-Johnson syndrome is an autosomal recessive condition characterized by recurrent episodes of jaundice and conjugated hyperbilirubinemia. It exacerbates during pregnancy and needs to be differentiated from other causes of jaundice. A 30-year-old patient presented to us with jaundice in her fourth pregnancy. Read More

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http://dx.doi.org/10.7759/cureus.4048DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6464460PMC
February 2019
7 Reads

Beyond an Obvious Cause of Cholestasis in a Toddler: Compound Heterozygosity for Mutations.

Pediatrics 2019 05;143(5)

Institut für Pathologie, Medizinische Universität Graz, Graz, Austria.

A 27-month-old girl presented with a short history of jaundice initially attributed to drug-induced liver injury. During the preceding 20 days, she had received a 10-day course of cefprozil and 2 doses of a homeopathic preparation of cantharidin for cystitis. Severe conjugated hyperbilirubinemia was present with normal γ-glutamyl transpeptidase activity. Read More

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http://dx.doi.org/10.1542/peds.2018-2146DOI Listing
May 2019
4 Reads

Hemoadsorption in isolated conjugated hyperbilirubinemia after extracorporeal membrane oxygenation support. Cholestasis of sepsis: A case report and review of the literature on differential causes of jaundice in ICU patient.

Int J Artif Organs 2019 May 28;42(5):263-268. Epub 2019 Mar 28.

1 II Department of Anaesthesiology and Intensive Care, Medical University of Lublin, Lublin, Poland.

Hyperbilirubinemia occurs in up to 40% of critically ill. In ICU, hyperbilirubinemia is an independent factor that influences patients' morbidity and mortality. Jaundice can reflect the course of disease or be caused by treatment (e. Read More

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http://dx.doi.org/10.1177/0391398819834012DOI Listing
May 2019
32 Reads

Neonatal cholestasis: emerging molecular diagnostics and potential novel therapeutics.

Nat Rev Gastroenterol Hepatol 2019 06;16(6):346-360

Pediatric Liver Center, Digestive Health Institute, Children's Hospital Colorado, Section of Pediatric Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine, Aurora, CO, USA.

Neonatal cholestasis is a group of rare disorders of impaired bile flow characterized by conjugated hyperbilirubinaemia in the newborn and young infant. Neonatal cholestasis is never physiological but rather is a sign of hepatobiliary and/or metabolic disorders, some of which might be fatal if not identified and treated rapidly. A step-wise timely evaluation is essential to quickly identify those causes amenable to treatment and to offer accurate prognosis. Read More

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http://www.nature.com/articles/s41575-019-0132-z
Publisher Site
http://dx.doi.org/10.1038/s41575-019-0132-zDOI Listing
June 2019
17 Reads

Severe Fetal Hemolysis and Cholestasis Due to High-Titer Maternal IgG Anti-A Antibodies.

Pediatrics 2019 04 14;143(4). Epub 2019 Mar 14.

Department of Experimental Immunohematology, Sanquin, Amsterdam, Netherlands.

ABO blood group incompatibility between mother and fetus can lead to hemolytic disease of the fetus and newborn (HDFN). We present the first case of severe O/A HDFN associated with extremely high-titer (1:32 000) immunoglobulin G anti-A antibodies in a Cameroon mother. Cord blood analysis revealed severe fetal hemolytic anemia and conjugated hyperbilirubinemia. Read More

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http://pediatrics.aappublications.org/lookup/doi/10.1542/ped
Publisher Site
http://dx.doi.org/10.1542/peds.2018-2859DOI Listing
April 2019
23 Reads

Case report: Cholecystoduodenostomy for cholestatic liver disease in a premature infant with cystic fibrosis and short gut syndrome.

BMC Pediatr 2019 03 11;19(1):78. Epub 2019 Mar 11.

Discipline of Paediatrics, School of Women's and Children's Health, Medicine, University of New South Wales, Sydney, NSW, Australia.

Background: Cholecystoduodenostomy is a surgical procedure that bypasses the extrahepatic biliary tree and connects the gallbladder directly to the duodenum. This case describes the successful use of this procedure in a novel situation.

Case Presentation: A premature (34 weeks gestation) female infant with cystic fibrosis required a laparotomy on day 1 of life due to an intrauterine small bowel perforation. Read More

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http://dx.doi.org/10.1186/s12887-019-1443-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6410495PMC
March 2019
8 Reads

Tissue variations of mosaic genome-wide paternal uniparental disomy and phenotype of multi-syndromal congenital hyperinsulinism.

Eur J Med Genet 2020 Jan 21;63(1):103632. Epub 2019 Feb 21.

Odense Pancreas Center (OPAC), Odense University Hospital, Odense, Denmark; Institute of Clinical Research, University of Southern Denmark Odense, Odense, Denmark; Dept. of Pathology, Odense University Hospital, Odense, Denmark.

Mosaic genome-wide paternal uniparental disomy (GW-pUPD) is a rarely recognised disorder. The phenotypic manifestations of multilocus imprinting defects (MLIDs) remain unclear. We report of an apparently non-syndromic infant with severe congenital hyperinsulinism (CHI) and diffuse pancreatic labelling by 18F*-DOPA-PET/CT leading to near-total pancreatectomy. Read More

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http://dx.doi.org/10.1016/j.ejmg.2019.02.004DOI Listing
January 2020
16 Reads

Neonatal Jaundice.

Indian J Pediatr 2019 09 21;86(9):830-841. 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
Publisher Site
http://dx.doi.org/10.1007/s12098-019-02856-0DOI Listing
September 2019
15 Reads