Prolonged neonatal jaundice: a manifestation of heterozygote state for Crigler--Najjar syndrome?

Abstract:

:A prolonged but transient neonatal jaundice due to unconjugated hyperbilirubinemia was observed in two unrelated babies who were siblings of patients with Crigler--Najjar disease (Arias type I). The bilirubin-UDP-glucuronyl transferase activity measured in the liver of both patients, at the age of 6 and 7 months, respectively, was found to be low, suggesting a permanent deficiency in bilirubin glucuronidation, which might be the expression of a heterozygote state.

authors

Odièvre M,Luzeau R,Alagille D

subject

Has Abstract

pub_date

1982-01-01 00:00:00

pages

239-41

issue

2

eissn

0277-2116

issn

1536-4801

journal_volume

1

pub_type

杂志文章
  • Gastric emptying in children: what is the best acquisition method?

    abstract:OBJECTIVES:The geometric mean is the recommended method for calculating gastric emptying in adults to correct for variable attenuation during the study. In children, it has been reported that a single posterior acquisition is sufficient. Our objective was to evaluate the relation between the values of gastric emptying ...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/MPG.0b013e31824ee2fa

    authors: Drubach LA,Kourmouzi V,Cao X,Zurakowski D,Fahey FH

    更新日期:2012-08-01 00:00:00

  • Effect of pH on fecal recovery of energy derived from volatile fatty acids.

    abstract::We assessed the effect of pH on volatilization of short-chain fatty acids during lyophilization. Acetic, propionic, valeric, and butyric acids were added to a fecal homogenate in amounts sufficient to raise the energy density by 18-27%. Fecal homogenate samples were either acidified (pH 2.8-3.2), alkalinized (pH 7.9-8...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:

    authors: Kien CL,Liechty EA

    更新日期:1987-11-01 00:00:00

  • Hepatic abscess as a complication of Crohn's disease.

    abstract::A 17-year-old male with Crohn's disease presented with persistent fever and right upper quadrant tenderness. Ultrasonography and abdominal computerized axial tomography (CAT scan) showed two large loculated abscess cavities in the right lobe of the liver. Surgical drainage and antibiotic therapy led to resolution of b...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/00005176-198302010-00021

    authors: Weinberg RJ,Klish WJ,Brown MR,Smalley JR,Emmens RW

    更新日期:1983-01-01 00:00:00

  • Pharmacokinetics and safety of a new solution of vitamin K1(20) in children with cholestasis.

    abstract::In cholestatic diseases, the absorption of fat-soluble compounds, including vitamin K1(20), is low and periodic administration of vitamin K1(20) is often necessary. Due to the low absorption of vitamin K1(20) from the Konakion formulation, late hemorrhagic disease of the newborn also occurs especially after oral vitam...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/00005176-199202000-00007

    authors: Amédée-Manesme O,Lambert WE,Alagille D,De Leenheer AP

    更新日期:1992-02-01 00:00:00

  • γ-Glutamyl transpeptidase level as a screening marker among diverse etiologies of infantile intrahepatic cholestasis.

    abstract:OBJECTIVES:Low γ-glutamyl transpeptidase (GGT) level is an important marker for progressive familial intrahepatic cholestasis, yet the cutoff level and clinical application is not well defined. This study aimed to evaluate the role of GGT as a screening marker among diverse etiologies of infantile cholestasis. METHODS...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/MPG.0000000000000538

    authors: Lu FT,Wu JF,Hsu HY,Ni YH,Chang MH,Chao CI,Chen HL

    更新日期:2014-12-01 00:00:00

  • Increasing incidence of paediatric inflammatory bowel disease in northern Stockholm County, 2002-2007.

    abstract:OBJECTIVES:A sharp increase in paediatric (younger than 16 years) inflammatory bowel disease (IBD) incidence was observed in northern Stockholm County, Sweden, in 1990-2001. The increasing incidence was primarily explained by a rising incidence of Crohn disease (CD). Here, we present an update on the trends in incidenc...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/MPG.0b013e31828f21b4

    authors: Malmborg P,Grahnquist L,Lindholm J,Montgomery S,Hildebrand H

    更新日期:2013-07-01 00:00:00

  • Folic acid absorption determined by a single stool sample test--a double-isotope technique. The folic acid absorption capacity in children.

    abstract::The fractional folic acid absorption (FAFol) was determined in 66 patients with various gastrointestinal diseases by a double-isotope technique, employing a single stool sample test (SSST) as well as a complete stool collection. The age of the patients ranged from 2.5 months to 16.8 years (mean 6.3 years). The test do...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/00005176-198910000-00012

    authors: Hjelt K

    更新日期:1989-10-01 00:00:00

  • North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome.

    abstract::Cyclic vomiting syndrome (CVS) is a disorder noted for its unique intensity of vomiting, repeated emergency department visits and hospitalizations, and reduced quality of life. It is often misdiagnosed due to the unappreciated pattern of recurrence and lack of confirmatory testing. Because no accepted approach to mana...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 共识发展会议,杂志文章

    doi:10.1097/MPG.0b013e318173ed39

    authors: Li BU,Lefevre F,Chelimsky GG,Boles RG,Nelson SP,Lewis DW,Linder SL,Issenman RM,Rudolph CD,North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.

    更新日期:2008-09-01 00:00:00

  • Results of extremely-low-birth-weight infants randomized to receive extra enteral calcium supply.

    abstract:BACKGROUND AND OBJECTIVE:Bone mineral deficiency continues to occur in extremely-low-birth-weight (ELBW) infants despite formulas enriched in calcium (Ca) and phosphorus (P). This study tested whether extra enteral Ca supplementation increases bone mineral content (BMC) and prevents dolichocephalic head flattening and ...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章,随机对照试验

    doi:10.1097/MPG.0b013e3182187ecd

    authors: Carroll WF,Fabres J,Nagy TR,Frazier M,Roane C,Pohlandt F,Carlo WA,Thome UH

    更新日期:2011-09-01 00:00:00

  • Pediatric Gastrojejunostomy Tube Replacement: Effects of Communication on the Need for After-Hours Procedures.

    abstract:OBJECTIVES:The aim of the study was to determine whether embedding into the radiology report a patient-specific plan in the event of gastrojejunostomy (GJ) tube dysfunction reduces the need for after-hours utilization of pediatric interventional radiology resources for the replacement of GJ tubes. MATERIALS AND METHOD...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/MPG.0000000000001267

    authors: Sharafinski ME Jr,Sehnert E,Moe D,Zhang L,Simpson P,Vo NJ

    更新日期:2016-09-01 00:00:00

  • Influence of esophageal pH recording on physical activity in children.

    abstract:OBJECTIVES:Twenty-four-hour pH-metry represents the gold standard for the diagnosis of gastroesophageal reflux. Ambulatory esophageal pH recording provides a better reflection of occurrences under normal domestic living conditions, but it may influence physical activity. The aim of this study was to assess the influenc...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/mpg.0b013e31817f2827

    authors: Michaud L,Troadec F,Béghin L,El Rifai N,Guimber D,Turck D,Gottrand F

    更新日期:2009-04-01 00:00:00

  • Clinical features of interleukin 10 receptor gene mutations in children with very early-onset inflammatory bowel disease.

    abstract:OBJECTIVES:In the present study, we studied a cohort of patients with very early onset inflammatory bowel disease (IBD) to determine the frequency of mutations in the interleukin 10 (IL10) receptor genes as a cause of early-onset IBD. METHODS:Sanger sequencing was performed to determine the presence of IL10 and/or IL1...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/MPG.0000000000000621

    authors: Beser OF,Conde CD,Serwas NK,Cokugras FC,Kutlu T,Boztug K,Erkan T

    更新日期:2015-03-01 00:00:00

  • Treatment of acute diarrhea with Saccharomyces boulardii in infants.

    abstract:OBJECTIVE:The aim of the study was to determine whether an oral treatment with a commercial pharmaceutical product containing Saccharomyces boulardii would reduce the duration of diarrhea in infants with acute diarrhea. PATIENTS AND METHODS:In the present double-blind, placebo-controlled study, 186 infants, 6 to 48 mo...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章,随机对照试验

    doi:10.1097/MPG.0b013e31822b7ab0

    authors: Corrêa NB,Penna FJ,Lima FM,Nicoli JR,Filho LA

    更新日期:2011-11-01 00:00:00

  • "Let there be bile"--understanding hepatic injury in cholestasis.

    abstract::Cholestatic liver disorders account for a large proportion of chronic liver ailments in adults, children and infants, and are among the leading indications for liver transplantation in all age groups. Recent studies have begun to characterize the cellular and molecular mechanisms of hepatocyte injury caused by the ret...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章,评审

    doi:10.1097/01.mpg.0000226384.71859.16

    authors: Sokol RJ,Devereaux M,Dahl R,Gumpricht E

    更新日期:2006-07-01 00:00:00

  • Impact of early dietary intake and blood lipid composition of long-chain polyunsaturated fatty acids on later visual development.

    abstract:BACKGROUND:In contrast to human milk, current infant formulas in the United States do not contain omega3 and omega6 long-chain polyunsaturated fatty acids. This may lead to suboptimal blood lipid fatty acid profiles and to a measurable diminution of visual function in developing term infants. The need for docosahexaeno...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1097/00005176-200011000-00016

    authors: Hoffman DR,Birch EE,Birch DG,Uauy R,Castañeda YS,Lapus MG,Wheaton DH

    更新日期:2000-11-01 00:00:00

  • Pediatric Intestinal Pseudo-obstruction: Impact of Neonatal and Later Onset on Clinical and Nutritional Outcomes.

    abstract:OBJECTIVE:The aim of the study was to evaluate long-term nutritional outcomes and clinical characteristics in a cohort of children with pediatric intestinal pseudo-obstruction (PIPO) at neonatal-onset (NO-PIPO) and at later-onset (LO-PIPO). METHODS:All children fulfilling new PIPO criteria over a 30-year period were r...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/MPG.0000000000002373

    authors: Diamanti A,Fusaro F,Caldaro T,Capriati T,Candusso M,Nobili V,Borrelli O

    更新日期:2019-08-01 00:00:00

  • Alkaline phosphatase activity in fresh intestinal mucosa and cultured mucosal explants.

    abstract::Alkaline phosphatase (AP) activity is an accepted marker of epithelial cell differentiation and integrity in intestinal mucosal explant systems. In experiments involving AP it is usually expressed as activity per unit protein in tissue culture. It has been previously demonstrated that during organ culture a considerab...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/00005176-198403000-00007

    authors: Ashkenazi A,Idar D,Maimon M,Hegesh E,Frank E,Hahn T,Wolman Y,Levin S

    更新日期:1984-03-01 00:00:00

  • Cholesteryl ester storage disease: protean presentations of lysosomal acid lipase deficiency.

    abstract:OBJECTIVE:LIPA gene mutations result in deficiency of lysosomal acid lipase and present phenotypically as Wolman disease or cholesteryl ester storage disease (CESD) depending on the level of deficiency. Patients with CESD may often be misdiagnosed because symptoms may be nonspecific. Symptoms may present in infancy if ...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章,评审

    doi:10.1097/MPG.0b013e31828b36ac

    authors: Zhang B,Porto AF

    更新日期:2013-06-01 00:00:00

  • Parenteral nutrition for infants: effect of high versus low calcium and phosphorus content.

    abstract::Calcium (Ca) and phosphorus (P) homeostasis were determined in 18 infants (birth weight, 2,810 +/- 135 g; gestational age, 37.4 +/- 0.5 weeks; mean +/- SEM) who received high or low Ca and P content (Ca, P) parenteral nutrition (PN) with a fixed, low dose of vitamin D (25 IU/dl). Nine infants were randomized into low ...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:

    authors: Koo WW,Tsang RC,Steichen JJ,Succop P,Babcock D,Oestreich AE,Noseworthy J,Horn J,Farrell MK

    更新日期:1987-01-01 00:00:00

  • Evaluation of oral rehydration solution by whole-gut perfusion in rats: effect of osmolarity, sodium concentration and resistant starch.

    abstract:BACKGROUND:Reduced osmolarity oral rehydration solution (ORS) improved small bowel absorption of fluid and electrolytes in segmental perfusion in experimental animals; this was borne out in clinical practice. Adding amylase-resistant starch (RS) to ORS is expected to increase colonic fluid absorption. This study used c...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/01.mpg.0000239998.43141.b2

    authors: Subramanya S,Ramakrishna BS,Binder HJ,Farthing MJ,Young GP

    更新日期:2006-11-01 00:00:00

  • Apolipoprotein A-I:B ratio and B screening: a preliminary study of 10- and 11-year-old children.

    abstract::The apolipoprotein (apo) A-I:B ratio and the apo B concentration were determined by radial immunodiffusion in dried blood spot samples from 1,767 10- and 11-year-old children. Children with either apo A-I:B ratios below the first percentile or apo B levels above the 99th were recalled and plasma lipid and apolipoprote...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:

    authors: Sveger T,Fex G,Flodmark CE,Kjellström T,Borgfors N

    更新日期:1990-02-01 00:00:00

  • Persistent morbidity and mortality of protein calorie malnutrition in young infants with CF.

    abstract::Young infants with cystic fibrosis (CF) are at risk for developing symptomatic protein-calorie malnutrition (PCM) characterized by hypoalbuminemia, edema, and anemia. We reviewed the hospital charts of all infants less than 12 months of age referred to our CF clinic between 1979 and 1982 and found nine patients with P...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/00005176-198605000-00010

    authors: Abman SH,Accurso FJ,Bowman CM

    更新日期:1986-05-01 00:00:00

  • Total Bile Acid Concentration in Duodenal Fluid Is a Useful Preoperative Screening Marker to Rule Out Biliary Atresia.

    abstract:OBJECTIVES:Duodenal tube test (DTT) is used as a preoperative screening to rule out biliary atresia (BA). In previous reports, DTT was assessed by the color of the duodenal fluid, but there were no quantitative criteria. The aim of this study was to examine the efficacy of DTT based on the total bile acid (TBA) concent...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/MPG.0000000000002037

    authors: Fukuoka T,Bessho K,Tachibana M,Satomura Y,Konishi A,Yasuda K,Kimura T,Hasegawa Y,Ueno T,Miyoshi Y,Ozono K

    更新日期:2018-09-01 00:00:00

  • Linear growth after colectomy for ulcerative colitis in childhood.

    abstract::Eighteen children and adolescents, of a total of 90 treated in our unit between 1982 and 1992, underwent colectomy for ulceractive colitis. Six (6.7%) of the 90 patients had short stature, i.e., a height below the 3rd centile, and 8 of the 18 who had surgery had a subnormal height velocity, i.e., below the 25th centil...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/00005176-199507000-00014

    authors: Nicholls S,Vieira MC,Majrowski WH,Shand WS,Savage MO,Walker-Smith JA

    更新日期:1995-07-01 00:00:00

  • Randomized Controlled Trial of Oropharyngeal Colostrum Administration in Very-low-birth-weight Preterm Infants.

    abstract:OBJECTIVE:The purpose of this study was to evaluate the effects of oropharyngeal colostrum administration in the incidence of late-onset clinical and proven sepsis and in concentrations of immunoglobulin A (IgA) in very-low-birth-weight (VLBW) infants. METHODS:We conducted a double-blinded, randomized, placebo-control...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章,随机对照试验

    doi:10.1097/MPG.0000000000002356

    authors: Ferreira DMLM,Oliveira AMM,de Leves DV,de Bem ÉB,Fatureto GG,Navarro NF,Afonso NG,Santiago FM,Mineo JR,Sopelete MC,Martinez FE,Bernardino Neto M,Abdallah VOS

    更新日期:2019-07-01 00:00:00

  • Impact of Breast Milk, Respiratory Insufficiency, and Gastroesophageal Reflux Disease on Enteral Feeding in Infants With Omphalocele.

    abstract:OBJECTIVES:The aim of this study was to document the process of achieving full enteral feeding in infants with omphalocele and to identify factors that affect feeding success. METHODS:After institutional review board approval (study no. 5100169), 123 infants with omphalocele, born between 1993 and 2011 were reviewed. ...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/MPG.0000000000001463

    authors: Haug S,St Peter S,Ramlogan S,Goff D,Thorpe D,Hopper A,Baerg J

    更新日期:2019-06-01 00:00:00

  • Evaluation of endoscopic sclerotherapy of esophageal varices in children.

    abstract::Sixteen children (3.5-14.7 years) with portal hypertension and variceal hemorrhage were treated by direct endoscopic variceal sclerotherapy. Follow-up clinical and endoscopic evaluations have been carried out over a 1-6-year period (mean, 2.3 years). Prior to sclerotherapy, three patients had undergone unsuccessful su...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/00005176-198609000-00005

    authors: Donovan TJ,Ward M,Shepherd RW

    更新日期:1986-09-01 00:00:00

  • Incidence, clinical presentation and location at diagnosis of pediatric inflammatory bowel disease: a prospective population-based study in northern France (1988-1999).

    abstract:OBJECTIVE:To assess the incidence and location at diagnosis of inflammatory bowel disease in children and adolescents in northern France between 1988 and 1999. METHODS:A 12-year prospective population-based study was conducted by gastroenterologists and pediatric gastroenterologists of northern France (1,312,141 child...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/01.mpg.0000162479.74277.86

    authors: Auvin S,Molinié F,Gower-Rousseau C,Brazier F,Merle V,Grandbastien B,Marti R,Lerebours E,Dupas JL,Colombel JF,Salomez JL,Cortot A,Turck D

    更新日期:2005-07-01 00:00:00

  • Reduced bile output with chronic enteral and parenteral infusion of amino acids, glucose, and fat emulsion in rabbits.

    abstract::To assess the effect of chronic administration of amino acids, glucose, and fat on hepatic excretory function, bile flow and bile salt secretion were directly measured in adult rabbits alimented either intravenously or intragastrically. Five groups of animals were studied after 9-11 days on different nutritional regim...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/00005176-198510000-00023

    authors: Zahavi I,Shaffer EA,Kelly J,Gall DG

    更新日期:1985-10-01 00:00:00

  • Measurement of the rate of entry of intact colon-derived lactose into the circulation: a model for assessing gut uptake of molecules not endogenously synthesized.

    abstract:BACKGROUND:Results of in vitro studies have documented colonic absorption of lactose in the newborn. A stable isotope model was developed for assessing the entry rate of intact lactose into the portal circulation in newborn piglets. METHODS:In experiment 1, unlabeled and [D-1-(13C)]-lactose were infused into two separ...

    journal_title:Journal of pediatric gastroenterology and nutrition

    pub_type: 杂志文章

    doi:10.1097/00005176-199707000-00011

    authors: Kien CL,Murray RD,Ailabouni AH,Habash DL,Powers PA

    更新日期:1997-07-01 00:00:00