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Chem Biodivers 2021 Jun 3. Epub 2021 Jun 3.

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

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

Clin Pediatr (Phila) 2021 May;60(4-5):221-225

Rambam Health Care Campus, Haifa, Israel.

JPEN J Parenter Enteral Nutr 2021 Apr 1. Epub 2021 Apr 1.

Intestinal Rehabilitation Program, Division of Gastroenterology, Hepatology, and Nutrition, Children's National Hospital, Washington, DC, USA.

Our aim was to analyze the outcomes in children with short-bowel syndrome (SBS), parenteral nutrition dependence (PND), and intestinal failure-associated liver disease (IFALD) treated in our Intestinal Rehabilitation Program (IRP) during 2007-2018. We retrospectively reviewed charts of 135 patients with SBS-PND at the time of enrollment in IRP; of these, 89 (66%) had IFALD, defined as conjugated bilirubin (CB) of ≥2 mg/dl at enrollment and/or abnormal liver biopsy showing stage 2-4 fibrosis. Outcomes included resolution of CB, enteral autonomy, laboratory parameters (platelets, aspartate aminotransferase to platelet ratio index), growth trends, transplant rates, and mortality. Read More

J Nucl Med Technol 2021 Jun 15;49(2):193-194. Epub 2021 Mar 15.

Third Department of Pediatrics, Attikon University Hospital, National and Kapodistrian University of Athens, Athens, Greece.

Rotor syndrome (RS) is a benign, inherited, commonly misdiagnosed cause of conjugated hyperbilirubinemia whose identification prevents unnecessary invasive investigations. We present the case of a 3-y-old boy with phenotypic and laboratory findings of RS but negative genetic test results, whose diagnosis was confirmed by hepatobiliary scintigraphy. Read More

Rev Esp Enferm Dig 2021 Mar 4. Epub 2021 Mar 4.

Cirugía General, Hospital Garcia Orcoyen.

We present the case of a 35-year-old woman with a history of polycystic ovary syndrome, treated with oral contraceptives who was under study for 9 months evolution pain in the right iliac fossa, associated with hyporexia and mild hyperbilirubinemia with a predominance of the conjugated fraction (total Bi 3.7 mg/dL, conjugated Bi 2.9 mg/dL). Read More

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

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

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

World J Clin Cases 2021 Feb;9(4):878-885

Department of Infectious Diseases, The Affiliated Hospital of Guizhou Medical University, Guiyang 550001, Guizhou Province, China.

Background: Dubin-Johnson syndrome (DJS) is a benign autosomal recessive liver disease involving mutations of the gene. It is characterized by chronic or intermittent conjugated hyperbilirubinemia, with chronic idiopathic jaundice as the main clinical manifestation. Genetic alterations of the gene are commonly used for diagnosing DJS; however, the causative point mutation in Chinese patients remains unknown. Read More

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

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

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

Pediatr Dev Pathol 2021 Mar-Apr;24(2):154-158. Epub 2021 Jan 20.

Department of Pediatrics, UT Southwestern Medical Center/Children's Medical Center, Dallas, Texas.

Dubin-Johnson syndrome (DJS) is a rare autosomal recessive disorder that typically manifests in young adulthood as jaundice with conjugated hyperbilirubinemia. We report a case presenting as neonatal cholestasis with the unexpected histologic finding of paucity of interlobular bile ducts, a feature that is not typically seen in DJS. The diagnosis was confirmed by absent canalicular multidrug-resistance-associated protein 2 (MRP2) immunohistochemical staining on liver biopsy tissue and molecular genetic testing that demonstrated heterozygous mutations in the ATP-Binding Cassette Subfamily C Member 2 ( gene, including a novel missense mutation. Read More

Eur J Pediatr 2021 May 19;180(5):1653-1657. Epub 2021 Jan 19.

Division of Endocrinology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, 555 University AvenueM5G1X8, Toronto, Ontario, Canada.

From clinical practice, we noted that a subset of neonates with hyperinsulinism develop conjugated hyperbilirubinemia. A relationship between these two conditions has not been previously described. We aimed to assess the incidence of cholestasis in a cohort of neonates with hyperinsulinism and describe their clinical characteristics. Read More

Indian J Gastroenterol 2021 Feb 11;40(1):72-76. Epub 2021 Jan 11.

Gastroenterology and Advanced G.I Endoscopy, Cochin Gastroenterology Group, Ernakulam Medical Center, Kochi, 682 028, India.

Dubin-Johnson syndrome (DJS), an autosomal recessive disorder presenting with conjugated hyperbilirubinemia, is not associated with progression to chronic liver disease (CLD). Next-generation sequencing, application of bioinformatics pipeline, and segregation analysis were performed on 8 members of a consanguineous family with DJS and CLD. A novel variant, c. Read More

World J Gastrointest Endosc 2020 Nov;12(11):493-499

Gastrointestinal Endoscopy Unit, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo 05403-000, Brazil.

Background: Pancreaticoduodenectomy is a technically demanding operation, with reported morbidity rates of approximately 40%-50%. A novel idea is to use endoscopic vacuum therapy (EVT) in a preemptive setting to prevent anastomotic leakage and pancreatic fistulas. In a recent case series, EVT was proven to be effective in preventing leaks in patients with anastomotic ischemia. Read More

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

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

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

J Pediatr Gastroenterol Nutr 2020 11;71(5):e138-e141

The Center for Pediatric Liver Diseases, Children's Hospital of Fudan University, Department of Pediatrics, Shanghai Medical College of Fudan University.

Objectives: The aim of the study was to explore the significance of sodium taurocholate cotransporting polypeptide (NTCP) deficiency and its clinical features in Chinese children presenting with isolated persistent hypercholanemia.

Methods: The exon and adjacent regions of SLC10A1, the gene encoding NTCP, were sequenced in 33 Chinese children presenting with isolated hypercholanemia. Clinical history and medical data were reviewed. Read More

Case Rep Med 2020 22;2020:6504260. Epub 2020 Sep 22.

Department of Surgery, Dr. Soetomo General Hospital, Universitas Airlangga, Surabaya, Indonesia.

Generalized peritonitis caused by spontaneous gall bladder perforation is rare and difficult to diagnose preoperatively. The increased absorption of the spilling conjugated bile from the gall bladder by the inflamed peritoneum causes jaundice and direct hyperbilirubinemia. A 56-year-old male patient with septic generalized peritonitis and obstructive-type hyperbilirubinemia came to our hospital. Read More

Tidsskr Nor Laegeforen 2020 09 20;140(12). Epub 2020 Aug 20.

In cases of infants with yellow colouration, both the sclerae and the skin should be examined. The top priority is to rule out conjugated hyperbilirubinaemia, which may be a symptom of biliary atresia. Children with this condition will first develop yellow sclerae, and will have jaundice that continues beyond the first two weeks of life. Read More

Lab Med 2021 May;52(3):226-231

Department of Cardiology, University of Health Sciences, Van Education and Research Hospital, Van, Turkey.

Objective: Hyperbilirubinemia is associated with protection against various oxidative stress-mediated diseases. We aimed to investigate the association between bilirubin and novel electrocardiography (ECG)-based ventricular repolarization parameters.

Methods: We enrolled 201 healthy men with mild hyperbilirubinemia (group 1) and 219 healthy men with normal bilirubin levels (group 2). Read More

Semin Pediatr Surg 2020 Aug 23;29(4):150945. Epub 2020 Jul 23.

Pediatric Liver Center, Digestive Health Institute, Children's Hospital Colorado, Section of Pediatric Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine, Box B290, 13123 E. 16th Ave., Aurora, CO 80045, USA; Colorado Clinical and Translational Sciences Institute, University of Colorado Anschutz Medical Campus, Aurora, CO, USA. Electronic address:

Neonatal cholestasis is characterized by conjugated hyperbilirubinemia in the newborn and young infant and is a sign common to over 100 hepatobiliary and/or metabolic disorders. A timely evaluation for its etiology is critical in order to quickly identify treatable causes such as biliary atresia, many of which benefit from early therapy. An expanding group of molecularly defined disorders involving bile formation, canalicular transporters, tight junction proteins and inborn errors of metabolism are being continuously discovered because of advances in genetic testing and bioinformatics. Read More

Pan Afr Med J 2020 19;36:106. Epub 2020 Jun 19.

Department of Paediatrics, Babcock University Teaching Hospital, Ilishan, Ogun State, Nigeria.

Infantile cholestasis has numerous causes and diagnosis can be difficult, especially in low-income countries where essential laboratory facilities are not readily available. This is a report of a baby who had severe conjugated neonatal hyperbilirubinaemia and deranged liver function tests, which posed a diagnostic dilemma before a diagnosis of congenital cytomegalovirus (CMV) infection was made. He was treated with Ganciclovir and responded well to treatment. Read More

Pharmaceutics 2020 Aug 11;12(8). Epub 2020 Aug 11.

Solvo Biotechnology, Science Park, Building B1, 4-20 Irinyi József utca, H-1117 Budapest, Hungary.

Bilirubin, the end product of heme catabolism, is produced continuously in the body and may reach toxic levels if accumulates in the serum and tissues; therefore, a highly efficient mechanism evolved for its disposition. Normally, unconjugated bilirubin enters hepatocytes through the uptake transporters organic anion transporting polypeptide (OATP) 1B1 and 1B3, undergoes glucuronidation by the Phase II enzyme UDP glucuronosyltransferase 1A1 (UGT1A1), and conjugated forms are excreted into the bile by the canalicular export pump multidrug resistance protein 2 (MRP2). Any remaining conjugated bilirubin is transported back to the blood by MRP3 and passed on for uptake and excretion by downstream hepatocytes or the kidney. Read More

Nutrients 2020 Jul 17;12(7). Epub 2020 Jul 17.

Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Grattan Street, Melbourne, VIC 3010, Australia.

This study aimed to evaluate jaundice outcomes of low-birthweight premature infants commenced on earlier versus later nutrient supplementation (80 mL/kg/day vs. 160 mL/kg/day; total fluid intake, F80 vs. F160). Read More

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

Toxicol Appl Pharmacol 2020 07 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

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

Pediatr Transplant 2020 06 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

Clin Radiol 2020 11 16;75(11):815-821. Epub 2020 Apr 16.

Department of Radiology, Leeds Children's Hospital, Clarendon Wing, Leeds, LS1 3EX, UK. Electronic address:

Infants with persistent conjugated jaundice, lasting longer than 14 days of age in a term infant and 21 days in a preterm infant, should be referred to a national paediatric liver unit for investigation of the cause. This paper reviews the ultrasound findings in such cases with a particular emphasis upon the diagnosis of biliary atresia. Read More

Indian Pediatr 2020 04;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