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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

Nat Rev Gastroenterol Hepatol 2019 Mar 22. Epub 2019 Mar 22.

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

Pediatrics 2019 Apr 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

BMC Pediatr 2019 Mar 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

Eur J Med Genet 2019 Feb 20. Epub 2019 Feb 20.

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

Indian J Pediatr 2019 Feb 21. 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

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

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

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

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

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

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

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

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

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

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

J Surg Res 2018 08 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

Pediatr Res 2018 08 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

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

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

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

Brachytherapy 2018 Jul - Aug;17(4):689-695. Epub 2018 May 7.

Department of Interventional Radiology, Zhongshan Hospital, Fudan University, Shanghai, China; Shanghai Institution of Medical Imaging, Shanghai, China.

Purpose: The purpose of this study was to analyze the long-term outcomes, including safety, efficacy, and prognostic features, of intraluminal brachytherapy with Iodine-125 (I) seed strand and stent placement for treatment of patients with malignant obstructive jaundice (MOJ).

Methods And Materials: From January 2009 to December 2013, 107 consecutive patients with MOJ were treated with intraluminal placement of I seed strands and metal stents. A retrospective evaluation of therapeutic outcomes, including overall survival (OS), stent patency rate, complications, and prognostic features, was conducted in 101 patients. Read More

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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