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neonatal cholestasis

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https://www.readbyqxmd.com/read/28135431/retrospective-dual-center-study-of-parenteral-nutrition-associated-cholestasis-in-premature-neonates
#1
Weihui Yan, Li Hong, Ying Wang, Yi Feng, Lina Lu, Yijing Tao, Jiang Wu, Huijuan Ruan, Qingya Tang, Wei Cai
BACKGROUND: The pathogenesis of parenteral nutrition-associated cholestasis (PNAC) has not been clarified. The objective of this study was to explore the incidence of PNAC in premature infants without surgery and to identify associated risk factors. MATERIALS AND METHODS: Premature neonates who received parenteral nutrition (PN) at least 14 days were included in a retrospective, dual-center study. Cholestasis was diagnosed as conjugated bilirubin ≥2 mg/dL. Infants with metabolic liver disease, cyanotic congenital heart disease, congenital syphilis, hepadnaviridae infection, and those who underwent surgery were excluded...
January 1, 2017: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/28125097/utility-of-measuring-direct-bilirubin-at-first-72%C3%A2-h-of-age-in-neonates-admitted-to-the-neonatal-intensive-care-unit
#2
A J Sloane, U S Nawab, D Carola, Z H Aghai
OBJECTIVE: To assess the utility of measuring direct bilirubin (DB) during the first 72 h of life in infants admitted to the neonatal intensive care unit (NICU). STUDY DESIGN: Infants born between May 2006 and June 2013, and admitted to the NICU were included. Abnormal DB was defined as: DB level⩾1 mg dl(-1) with a corresponding TB of ⩽5 mg dl(-1) or DB level >20% of the corresponding TB>5 mg dl(-1). RESULTS: The DB levels were measured in 3715 infants during the first 72 h of life...
January 26, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28076608/dental-chromatic-alteration-caused-by-neonatal-cholestasis
#3
Yasmin Etienne Albuquerque, Camila Maria Bullio Fragelli, Josimeri Hebling, Elisa Maria Aparecida Giro
No abstract text is available yet for this article.
October 2016: Einstein
https://www.readbyqxmd.com/read/28051047/evaluation-of-the-use-of-laparoscopic-guided-cholecystocholangiography-and-liver-biopsy-in-definitive-diagnosis-of-neonatal-cholestatic-jaundice
#4
Khalid Shreef, Abdullah Alhelal
BACKGROUND: Once it is established that a jaundiced infant has direct hyperbilirubinemia, the principal diagnostic concern is to differentiate hepatocellular from obstructive cholestasis. Traditional tests such as ultrasonography, percutaneous liver biopsy and technetium 99 m hepatobiliary iminodiacetic acid (HIDA) scan are often not sufficiently discriminating. Definitive exclusion of biliary atresia (BA) in the infant with cholestatic jaundice usually requires mini-laparotomy and intra-operative cholangiography...
October 2016: African Journal of Paediatric Surgery: AJPS
https://www.readbyqxmd.com/read/28028560/pathophysiology-prevention-treatment-and-outcomes-of-intestinal-failure-associated-liver-disease
#5
REVIEW
Noora H Al-Shahwani, David L Sigalet
BACKGROUND: Intestinal failure-associated liver disease (IFALD) remains a serious problem in the treatment of infants with nutritional problems and short bowel syndrome. METHODS: A review of the recent literature from 2010 to 2016, concentrating on articles related to the pathophysiology of IFALD and to outcomes of novel nutritional and pharmacological therapies for neonatal cholestasis in the post-surgical neonate. RESULTS: The pathophysiology of IFALD relates to an increase sensitivity of the neonatal liver to cholestasis in the non-fed state; prolonged cholestasis almost inevitably results in liver damage which will progress from fibrosis to cirrhosis...
December 27, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#6
Güzide Doğan, Fatih Düzgün, Serdar Tarhan, Yeliz Çağan Appak, Erhun Kasırga
Congenital intrahepatic portosystemic shunts are rare in children. Portosystemic venous malformations are characterized by extreme clinical variability. We report a full-term 33-day-old male infant presenting with neonatal jaundice. On physical examination, he had generalized icterus and the liver was palpable 3.5 cm below the right costal margin. He had no other symptoms. Laboratory tests showed AST 632 U/L, ALT 198 U/L, total bilirubin 12.1 mg/dL, conjugated bilirubin 10.2 mg/dL, alkaline phosphatase 753 U/L, GGT 47 U/L and glucose 67 U/L...
December 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27975109/cholestasis-in-infancy
#7
Melissa G Andrianov, Ruba K Azzam
Jaundice is a key manifestation of hepatobiliary disease in all age groups. Jaundice is a common finding in the first 2 weeks after birth, occurring in 2.4% to 15% of newborns. The neonatal liver is at increased susceptibility to cholestasis, with an incidence ranging from 1 in 2,500 to 1 in 5,000 live births. Etiologies vary, but the most common is biliary atresia. In 2004, the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition published guidelines for the evaluation of cholestasis that clearly stated any infant with jaundice persisting beyond age 2 weeks (3 weeks in breast-fed infants with an otherwise normal history and physical examination) should be evaluated with a fractionated serum bilirubin level...
December 1, 2016: Pediatric Annals
https://www.readbyqxmd.com/read/27938561/-value-of-albumin-in-diagnosis-of-neonatal-intrahepatic-cholestasis-caused-by-citrin-deficiency
#8
J Zhang, X H Wang, Y Z Ye, X B Xie, Y Lu, L J Ye, H Yu
Objective: To investigate the clinical value of albumin (Alb) in the diagnosis of neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). Methods: A retrospective analysis was performed for the clinical data of 90 children with NICCD who visited Children's Hospital of Fudan University from January 2007 to December 2014, and according to the content of Alb, these children were divided into Alb < 30 g/L (LA) group with 20 children and Alb ≥30 g/L (NA) group with 70 children. The clinical manifestations, results of laboratory examination, results of blood tandem mass spectrometry and urine gas chromatography-mass spectrometry, and gene detection results were compared between the two groups...
October 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/27936482/intrahepatic-cholestasis-of-pregnancy-icp-case-report-and-review-of-the-literature
#9
V Keitel, C Dröge, S Stepanow, T Fehm, E Mayatepek, K Köhrer, D Häussinger
Intrahepatic cholestasis of pregnancy (ICP) represents the most common pregnancy-related liver disease in women. Women frequently present in the third trimester with pruritus and elevated serum bile acid and/or alanine transaminase levels. Clinical symptoms quickly resolve after delivery; however, recurrence in subsequent pregnancies has to be expected. Intrahepatic cholestasis of pregnancy is associated with increased perinatal complications, such as premature delivery, meconium staining of the amniotic fluid, respiratory distress, low Apgar scores, and even stillbirth...
December 2016: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/27933591/charcoal-hemoperfusion-in-the-treatment-of-medically-refractory-pruritus-in-cholestatic-liver-disease
#10
Wonngarm Kittanamongkolchai, Ziad M El-Zoghby, J Eileen Hay, Russell H Wiesner, Patrick S Kamath, Nicholas F LaRusso, Kymberly D Watt, Carl H Cramer, Nelson Leung
BACKGROUND: Pruritus is a distressing symptom in a considerable proportion of cholestatic patients and a few of them do not respond to conventional treatment. Charcoal hemoperfusion (CH) is an extracorporeal technique that is effective in eliminating protein-bound substances which may have accumulated during cholestasis. Several case reports have shown significant reduction of bilirubin in mechanical jaundice and neonatal hemolytic jaundice. However, the published data of CH for the treatment of refractory pruritus in cholestatic patients are scarce...
December 8, 2016: Hepatology International
https://www.readbyqxmd.com/read/27931577/liver-transplantation-in-polish-children-with-%C3%AE-1-antitrypsin-deficiency-a-single-center-experience
#11
A Bakula, J Pawlowska, O Niewiadomska, I Jankowska, M Teisseyre, P Kaliciński, P Socha
BACKGROUND: α1-Antitrypsin deficiency (ATD) is the most common genetic cause of liver injury in young children. Asymptomatic hepatitis is observed in most patients. However, the course of liver disease due to ATD is unpredictable, and some children develop liver cirrhosis. Liver transplantation (Ltx) dramatically improves their outcome and in some cases is required in the first years of life. The aim of the study was to evaluate the course of the disease in children with ATD treated with Ltx in a single center...
December 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27916444/influence-of-weight-at-enterostomy-reversal-on-surgical-outcomes-in-infants-after-emergent-neonatal-stoma-creation
#12
Lindsay J Talbot, Robert D Sinyard, Kristy L Rialon, Brian R Englum, Elizabeth T Tracy, Henry E Rice, Obinna O Adibe
PURPOSE: Neonates after emergent enterostomy creation frequently require reversal at low weight because of complications including cholestasis, dehydration, dumping, failure to thrive, and failure to achieve enteral independence. We investigated whether stoma reversal at low weight (< 2.5kg) is associated with poor surgical outcomes. METHODS: Patients who underwent enterostomy reversal from 2005 to 2013 at less than 6months old were identified in our institutional database...
January 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27891590/bi-allelic-iars-mutations-in-a-child-with-intra-uterine-growth-retardation-neonatal-cholestasis-and-mild-developmental-delay
#13
Naama Orenstein, Karin Weiss, Stephanie N Oprescu, Rivka Shapira, Dvora Kidron, Lina Vanagaite-Basel, Anthony Antonellis, Maximilian Muenke
Recently, bi-allelic mutations in cytosolic isoleucyl-tRNA synthetase (IARS) have been described in three individuals with growth delay, hepatic dysfunction, and neurodevelopmental disabilities. Here we report an additional subject with this condition identified by whole-exome sequencing. Our findings support the association between this disorder and neonatal cholestasis with distinct liver pathology. Furthermore, we provide functional data on two novel missense substitutions and expand the phenotype to include mild developmental delay, skin hyper-elasticity, and hypervitaminosis D...
November 28, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/27891271/intrahepatic-cholestasis-of-pregnancy-with-severe-elevation-of-bile-acids-in-the-setting-of-acute-hepatitis-c-infection
#14
Megan L Lawlor, Agatha S Critchfield
Intrahepatic cholestasis of pregnancy (ICP) is a complication of pregnancy resulting in elevation of serum bile acid levels. ICP is often associated with underlying liver disease, including hepatitis C. Bile acids in relationship to the acute infection of hepatitis C virus have not yet been delineated in the literature. A 26-year-old gravida 4 para 2103 with dichorionic, diamniotic twin gestation and history of intravenous drug abuse developed ICP in the setting of acute hepatitis C infection. In addition to clinical symptoms of pruritus and right upper quadrant pain, she developed severe elevation in bile acids, 239 micromol/L, and transaminitis aspartate aminotransferase 1033 U/L, and alanine aminotransferase 448 U/L...
2016: Case Reports in Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27890981/neonatal-cholestasis-single-centre-experience-in-central-india
#15
Mayank Jain, Sagar Adkar, Chandrashekhar Waghmare, Jenisha Jain, Shikhar Jain, Kamna Jain, Gouri Rao Passi, Rashmi Shad Vinay, M K Soni
BACKGROUND: Neonatal cholestasis syndrome (NCS) is a major cause of morbidity and mortality in infants. The disorder has rarely been studied in centers from Central India. OBJECTIVES: To study the prevalence, clinical presentation and etiology of NCS at a tertiary referral center in Central India. MATERIALS AND METHODS: The study was carried out at a tertiary referral center in Central India. The study is a descriptive study. The records of all patients with suspected NCS treated in the Department of Pediatrics from 2007-2012 were analyzed...
October 2016: Indian Journal of Community Medicine
https://www.readbyqxmd.com/read/27882439/obstetric-and-perinatal-outcome-of-babies-born-from-sperm-selected-by-macs-from-a-randomized-controlled-trial
#16
Laura Romany, Nicolas Garrido, Ana Cobo, Belen Aparicio-Ruiz, Vicente Serra, Marcos Meseguer
PURPOSE: The purpose of this study is to assess outcomes after magnetic-activated cell sorting (MACS) technology on obstetric and perinatal outcomes compared with those achieved after swim up from randomized controlled trial. METHODS: This is a two-arm, unicentric, prospective, randomized, and triple-blinded trial and has a total of 237 infertile couples, between October 2010 and January 2013. A total of 65 and 66 newborns from MACS and control group, respectively, were described...
November 23, 2016: Journal of Assisted Reproduction and Genetics
https://www.readbyqxmd.com/read/27843908/anterior-chamber-pathology-in-alagille-syndrome
#17
Derek K Ho, Alex V Levin, William V Anninger, David A Piccoli, Ralph C Eagle
BACKGROUND: Alagille syndrome is an autosomal dominant disorder characterized by neonatal cholestasis, characteristic facies, and cardiac abnormalities. Ocular abnormalities include posterior embryotoxon, mosaic pattern of iris stromal hypoplasia, microcornea, optic disc drusen, and pigmentary retinopathy. We present the second report of ocular pathology in two cases of Alagille syndrome. METHODS: Gross and histologic preparations of four eyes of two patients. RESULTS: Posterior embryotoxon is seen in both cases, with iris processes extending to the embryotoxon in case 1...
October 2016: Ocular Oncology and Pathology
https://www.readbyqxmd.com/read/27829683/biochemical-and-molecular-characteristics-of-citrin-deficiency-in-korean-children
#18
Seak Hee Oh, Beom Hee Lee, Gu-Hwan Kim, Jin-Ho Choi, Kyung Mo Kim, Han-Wook Yoo
Mutations in SLC25A13 cause citrin deficiency, which has three phenotypes: neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), failure to thrive and dyslipidemia caused by citrin deficiency (FTTDCD) and adult-onset type 2 citrullinemia (CTLN2). The purpose of this study was to determine the mutation spectrum and the clinical and biochemical characteristics of citrin deficiency in Korean patients. Thirty-four patients were diagnosed with citrin deficiency based on mutations in SLC25A13, as verified by direct sequencing and long PCR screening of a large transposon insertion...
November 10, 2016: Journal of Human Genetics
https://www.readbyqxmd.com/read/27806795/-infantile-cholestasis-caused-by-cftr-mutation-case-report-and-literature-review
#19
L Li, N L Wang, J Y Gong, J S Wang
Objective: To study the clinical presentation, biochemical features and genetic analysis of an infant with cholestasis related to the CFTR mutations. Method: The clinical presentation, laboratory investigations and management of a case with infantile cholestasis caused by CFTR mutations were summarized and the relevant literature was reviewed. Result: (1) The patient was a 5 months old boy with cholestasis which developed in neonatal period with delayed meconium exclusion.The laparoscopic exploration was performed to exclude biliary atresia because of acholic stool when he was two months old...
November 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27781254/-application-of-bishop-koop-stoma-in-refractory-congenital-intestinal-atresia
#20
Hong Zhang, Wei Zhong, Jing Sun, Qiuming He, Yong Wang, Le Li, Jiakang Yu, Zhe Wang, Zhihua Ye, Kailin Tang, Huimin Xia
OBJECTIVE: To explore the feasibility and safety of Bishop-Koop stoma procedure in the treatment of neonates with refractory congenital intestinal atresia. METHODS: Clinical and follow-up data of 25 neonates with refractory congenital intestinal atresia undergoing Bishop-Koop stoma procedure in our center from January 2011 to December 2014 were retrospectively analyzed. Of 25 neonates, 13 (52%) were male, 12(48%) were female, the birth weight was 1600-3800 g (mean 2920 g), the age of admission was 10 hours to 20 days, and the age of operation was 1-58 d (mean 7 d)...
October 25, 2016: Zhonghua Wei Chang Wai Ke za Zhi, Chinese Journal of Gastrointestinal Surgery
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