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Pulm Pharmacol Ther 2022 06 18;73-74:102132. Epub 2022 May 18.

Clinical Trial Center, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing Key Laboratory of Drug Clinical Risk and Personalized Medication Evaluation, Beijing, China. Electronic address:

We investigated the safety, tolerability and pharmacokinetic (PK) profile of pretomanid (formerly PA-824) in healthy Chinese volunteers. This was a single-center, double-blind, placebo-controlled, phase I dose escalation study, in which healthy volunteers were consecutively allocated to increasing pretomanid dose groups (50, 100, 200, 400, 600, 800, or 1000 mg) and randomized to receive pretomanid or matching placebo. The primary objective was to evaluate the safety, tolerability and PK profile of pretomanid. Read More

Pediatr Dev Pathol 2022 May 17:10935266221103997. Epub 2022 May 17.

Gujarat Superspeciality Clinic, Ahmedabad, India.

Congenital bile acid synthesis defect type 3 is a rare metabolic liver disease with only eight patients reported in literature. We describe clinical, pathological and molecular features for a ninth patient. A 4-month-old infant presented to us with conjugated hyperbilirubinemia. Read More

Indian J Crit Care Med 2022 Mar;26(3):387-389

Department of General Medicine, Shri Ram Murti Smarak Institute of Medical Sciences, Bareilly, Uttar Pradesh, India.

We report a case of fulminant hepatic failure due to the Budd-Chiari syndrome following preservation with a tropical fever. A young lady came with fever, altered mental status, jaundice, and renal failure. Following tropical workup, it was diagnosed as a case of leptospirosis (WHO Faine's criteria) with multi-organ dysfunction. Read More

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

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

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

Paediatr Int Child Health 2022 Apr 10:1-6. Epub 2022 Apr 10.

Department of Paediatrics, Vani Vilas Hospital, Bangalore Medical College and Research Institute, Bengaluru, India.

Untreated syphilis in pregnancy can result in an adverse outcome for the fetus. A multigravida woman with a previously poor obstetric history of early neonatal death, abortion and stillbirth was admitted in labour in the 7th month of pregnancy. On admission, syphilis screening with the qualitative rapid plasma reagin (RPR) test was negative. Read More

Pediatr Infect Dis J 2022 07 7;41(7):579-586. Epub 2022 Jun 7.

School of Women's and Children's Health, University of New South Wales, Kensington.

Background: To determine the characteristics and outcomes of postnatal cytomegalovirus (pCMV) infection in preterm infants in a neonatal intensive care unit (NICU).

Methods: A retrospective, matched case-control study in a tertiary NICU. Infants born between January 2009 and December 2019, <32 weeks' gestational age (GA) and/or birth weight (BW) <1500 g with pCMV infection were matched 1:1 with cytomegalovirus-(CMV)-negative infants by year of admission, gender, GA and BW. Read More

Front Physiol 2022 21;13:859294. Epub 2022 Mar 21.

Department of Pharmacology, Faculty of Medicine in Hradec Kralove, Charles University, Hradec Kralove, Czechia.

Multidrug resistance-associated protein 2 (Mrp2) mediates biliary secretion of anionic endobiotics and xenobiotics. Genetic alteration of Mrp2 leads to conjugated hyperbilirubinemia and predisposes to the development of intrahepatic cholestasis of pregnancy (ICP), characterized by increased plasma bile acids (BAs) due to mechanisms that are incompletely understood. Therefore, this study aimed to characterize BA metabolomics during experimental Mrp2 deficiency and ICP. Read More

Arch Pediatr 2022 May 26;29(4):267-271. Epub 2022 Mar 26.

Department of Pediatric Gastroenterology, King Saud University, Riyad, Saudi Arabia.

Objectives: Dubin-Johnson syndrome (DJS) is a rare benign autosomal recessive disorder characterized by cholestasis in neonates. The aim of the present study was to describe the clinical characteristics, hepatic profiles, histopathology, gene mutations, and treatment outcomes of neonatal DJS.

Material And Methods: A multicenter retrospective study was undertaken with patients who had DJS. Read More

Dan Med J 2022 Feb 18;69(3). Epub 2022 Feb 18.

Department of Paediatrics, Randers Regional Hospital.

Introduction: The Danish Health Authority (DHA) recommends diagnostic evaluation of infants who develop prolonged jaundice and a serum conjugated bilirubin (CB) concentration ≥ 17 μmol/l. This study aimed to assess the efficacy of the programme in identifying infants with biliary atresia (BA) or other liver disease. Infants born in the Central Denmark Region from 2016 to 2021 were investigated. Read More

J Perinatol 2022 Jun 22;42(6):702-707. Epub 2022 Feb 22.

Center for Fetal Medicine, Pregnancy Care and Delivery, Women´s Health, Karolinska University Hospital, Stockholm, Sweden.

Objective: To estimate the incidence of cholestasis in neonates with hemolytic disease of the fetus and newborn (HDFN) and investigate risk factors and long-term liver disease.

Study Design: A population-based cohort study of all infants born with HDFN within the Stockholm region between 2006 and 2015. The study period was the first 90 days of life, and presence of any chronic liver disease was evaluated at two years of age. Read More

J Pediatr Endocrinol Metab 2022 May 2;35(5):681-685. Epub 2022 Feb 2.

Biochemistry Department, Gazi University, Ankara, Turkey.

Objectives: Niemann-Pick disease type C (NPC) is a lysosomal storage disease due to impaired intracellular lipid trafficking caused by biallelic pathogenic variants in or genes. NPC is classified according to the age of onset of neurological manifestations. Cholestatic liver disease can be transient or lead to liver failure. Read More

Pediatr Rev 2022 Feb;43(2):100-103

Division of Neonatology, Department of Pediatrics, Baylor Scott & White Medical Center - Temple, Temple, Texas.

Laboratory results include the following: white blood cell count, 21,600/μL (21.6 × 109/L; reference range, 9,000-30,000/μL [9-30 × 109/L]); hemoglobin, 18.2 g/dL (182 g/L; reference range, 14. Read More

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

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

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

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

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

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

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

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

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

Pediatr Clin North Am 2021 12;68(6):1333-1341

Medstar Georgetown Transplant Institute, Medstar Georgetown University Hospital, 3800 Reservoir Road, NW, Washington, DC, USA; Department of Pediatrics, Georgetown University School of Medicine, Washington, DC, USA. Electronic address:

"Biliary atresia (BA) is a common cause of jaundice in infancy. There is increasing evidence that newborn screening with direct or conjugated bilirubin leads to earlier diagnosis. Although the Kasai portoenterostomy is the primary treatment, there are scientific advances in adjuvant therapies. Read More

Case Rep Gastroenterol 2021 Sep-Dec;15(3):810-818. Epub 2021 Sep 21.

Hepatobiliary Division, Department of Internal Medicine, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo National General Hospital, Jakarta, Indonesia.

Ascites is defined as the accumulation of intra-peritoneal fluid that can be caused by several diseases. We described a 47-year-old female presenting with low serum-ascites albumin gradient (SAAG) and a markedly high level of serum globulin. Serum protein electrophoresis revealed an M spike in the gamma region. Read More

Am J Med Genet A 2022 02 1;188(2):548-555. Epub 2021 Nov 1.

Department of Pediatrics, University of Nebraska Medical Center, Omaha, Nebraska, USA.

Trisomy 13 and 18 are common chromosomal abnormalities that affect multiple organ systems. There is a paucity of published data, however, on the hepatic complications seen in these patient populations. One of the most common pathologic hepatobiliary issues seen in the newborn period is direct hyperbilirubinemia (DH). Read More

Cold Spring Harb Mol Case Stud 2021 12 9;7(6). Epub 2021 Dec 9.

Department of Medical Genetics, Haukeland University Hospital, 5021 Bergen, Norway.

Here we describe for the first time double paternal uniparental isodisomy (iUPD) 7 and 15 in a baby boy with features in the Beckwith-Wiedemann syndrome spectrum (BWSp) (placentomegaly, hyperinsulinism, enlarged viscera, hemangiomas, and earlobe creases) in addition to conjugated hyperbilirubinemia. His phenotype was also reminiscent of genome-wide paternal uniparental isodisomy. We discuss the most likely origin of the UPDs: a maternal double monosomy 7 and 15 rescued by duplication of the paternal chromosomes after fertilization. Read More

Cureus 2021 Aug 25;13(8):e17447. Epub 2021 Aug 25.

Pediatrics, Arya Care and Cure Hospital, Janakpur, NPL.

Biliary atresia (BA) is a rare disorder that usually presents with cholestatic symptoms such as jaundice, pale stool, and dark urine shortly after birth. Intracranial hemorrhage is a rare manifestation. BA may sometimes be associated with a cyst in the biliary tree. Read More

Cancer Med 2021 11 16;10(21):7551-7560. Epub 2021 Sep 16.

Cancer and Blood Disease Institute, Children's Hospital Los Angeles/University of Southern California, Los Angeles, California, USA.

Background: Pegaspargase (PEG-ASP) is an integral component of therapy for acute lymphoblastic leukemia (ALL) but is associated with hepatotoxicity that may delay or limit future therapy. Obese and adolescent and young adult (AYA) patients are at high risk. Levocarnitine has been described as potentially beneficial for the treatment or prevention of PEG-ASP-associated hepatotoxicity. Read More

BMJ Case Rep 2021 Sep 14;14(9). Epub 2021 Sep 14.

Neonatology, Bharati Vidyapeeth University, Pune, Maharashtra, India.

Neonatal hyperbilirubinaemia is a very common entity witnessed in most of the newborns. Rarely are there events where the bilirubin levels reach extreme values mandating invasive therapy. Unconjugated hyperbilirubinaemia when solely present is easy to manage and diagnose the common aetiological factors associated with it. Read More

Indian Pediatr 2022 Jan 4;59(1):43-45. Epub 2021 Sep 4.

Department of Pediatrics, All India Institute of Medical Sciences, New Delhi.

Objective: To document morbidities in adolescents with cystic fibrosis (CF) from India.

Methods: Details of children with cystic fibrosis surviving beyond 15 years of age were extracted from hospital records, and analyzed.

Results: 43 children [Median (IQR) age 18. Read More

J Pediatr Gastroenterol Nutr 2021 11;73(5):630-635

Department of Pediatrics, University of Helsinki, Helsinki, Finland.

Objectives: The prevalence and significance of liver involvement at diagnosis was studied in pediatric acute lymphoblastic (ALL) and myeloid leukemia (AML).

Methods: A population based cohort of 122 pre B-ALL, 22 T-ALL and 45 AML patients was formed from the Nordic Society of Pediatric Hematology and Oncology leukemia registries (years 2005-2017). Hepatomegaly, elevated alanine aminotransferase, high INR, hypoalbuminemia and conjugated hyperbilirubinemia at diagnosis were used as markers for liver involvement. Read More

Methods Mol Biol 2021 ;2342:695-707

Boehringer Ingelheim Pharmaceuticals Inc., Ridgefield, CT, USA.

New molecular entities (NMEs) are evaluated using a rigorous set of in vitro and in vivo studies to assess their safety and suitability for testing in humans. Regulatory health authorities require that therapeutic and supratherapeutic doses be administered, by the intended route of administration, to two nonclinical species prior to human testing. The purpose of these studies is to identify potential target organ toxicity and to determine if the effects are reversible. Read More

Can J Gastroenterol Hepatol 2021 24;2021:9969825. Epub 2021 Jun 24.

Department of Pediatric Surgery, Charles University in Prague, 2nd Faculty of Medicine, University Hospital Motol in Prague, Prague, Czech Republic.

Over a twenty-year period, we performed 255 ERCP procedures in infants aged up to 1 year. ERCP was indicated in cholestatic infants with suspicion of biliary obstruction. The most common diagnosis was biliary atresia (48%), choledochal cysts (13%), and choledocholithiasis (4%). Read More

Blood Purif 2022 17;51(3):243-250. Epub 2021 Jun 17.

Département d'Anesthésie-Réanimation, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Lyon, France.

Introduction: Liver failure is associated with hepatic and extrahepatic organ failure leading to a high short-term mortality rate. Extracorporeal albumin dialysis (ECAD) aims to reduce albumin-bound toxins accumulated during liver failure. ECAD detoxifies blood using albumin dialysis through an artificial semipermeable membrane with recirculation (molecular adsorbent recirculating system, MARS) or without (single-pass albumin dialysis, SPAD). Read More

Eur J Pediatr 2021 Aug 12;180(8):2629-2636. Epub 2021 Jun 12.

NWRH, Burnie, TAS3, Australia.

Transcutaneous measurement of bilirubin is being used for neonatal jaundice. Its utility during phototherapy in preterm babies is not established. The objective of our study was to assess the efficacy of transcutaneous bilirubin (TcB) measurement in comparison to total serum bilirubin in preterm newborns at admission and during phototherapy at the covered skin area (glabella). Read More

Chem Biodivers 2021 Aug 22;18(8):e2100222. Epub 2021 Jun 22.

School of Pharmaceutical Science and Technology, Tianjin University, 92 Weijin Road, Nankai District, Tianjin, 300072, P. R. 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 tested 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 05;60(4-5):221-225

Rambam Health Care Campus, Haifa, Israel.