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neonatal cholestasis

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https://www.readbyqxmd.com/read/28324003/biliary-anomalies-in-patients-with-hnf1b-diabetes
#1
Jarno Lt Kettunen, Helka Parviainen, Päivi J Miettinen, Martti Färkkilä, Marjo Tamminen, Pia Salonen, Eila Lantto, Tiinamaija Tuomi
Context: The clinical spectrum of organogenetic anomalies associated with HNF1B mutations is heterogeneous. Besides cystic kidney disease, diabetes, and various other manifestations, odd cases of mainly neonatal and post-transplant cholestasis have been described. The biliary phenotype is incompletely defined. Objective: To systematically characterize HNF1B-related anomalies in the bile ducts by imaging with MRI or MRCP. Setting and Patients: Fourteen patients with HNF1B mutations in the catchment area of the Helsinki University hospital were evaluated with upper abdominal MRI and MRCP...
March 17, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28321310/neonatal-cholestasis-mimicking-biliary-atresia-could-it-be-urinary-tract-infection
#2
Noella Maria Delia Pereira, Ira Shah
Cholestasis can occur in newborns due to infections. However, the manifestations of the underlying infections usually dominate the presentation. We present a 2-month-old infant who presented with jaundice and no fever or signs of systemic illness. Liver biopsy was suggestive of cholangitis. He was subsequently detected to have urinary tract infection with Klebsiella pneumoniae. The child was treated with appropriate antibiotics for 2 weeks following which the cholestasis resolved. Thus, neonatal cholestasis due to infections can also occur in the post-neonatal period without clinical manifestations of an underlying infection...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28314860/a-newborn-screening-method-for-cerebrotendinous-xanthomatosis-using-bile-alcohol-glucuronides-and-metabolite-ratios
#3
Frédéric M Vaz, Albert H Bootsma, Willem Kulik, Aad Verrips, Ron A Wevers, Peter C Schielen, Andrea E DeBarber, Hidde H Huidekoper
Cerebrotendinous xanthomatosis (CTX) is a treatable neurodegenerative metabolic disorder of bile acid synthesis where symptoms can be prevented if treatment with chenodeoxycholic acid supplementation is initiated early in life, making CTX an excellent candidate for newborn screening. We developed a new dried blood spot screening assay for this disorder based on different ratios between the accumulating cholestanetetrol glucuronide (tetrol) and specific bile acids/bile acid intermediates, without the need for derivatization...
March 17, 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/28302210/-risk-factors-for-neonatal-pulmonary-hemorrhage-in-the-neonatal-intensive-care-unit-of-a-municipal-hospital
#4
Jie Fan, Ming-Yan Hei, Xi-Lin Huang, Xiao-Ping Li
OBJECTIVE: To investigate the risk factors for neonatal pulmonary hemorrhage (NPH) in the neonatal intensive care unit (NICU) of a municipal hospital, and to provide a basis for the early identification and treatment of NPH. METHODS: A total of 112 neonates who were admitted to the NICU of Shaoyang Central Hospital of Hunan Province and diagnosed with NPH were enrolled as the case group. A nested case-control method was used to select, as a control group (n=224), the neonates who underwent the treatment with an assisted mechanical ventilator and did not experience pulmonary hemorrhage...
March 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28283263/impact-of-oocyte-donation-on%C3%A2-perinatal-outcome-in-twin-pregnancies
#5
Lucie Guilbaud, Pietro Santulli, Eva Studer, Vanessa Gayet, François Goffinet, Camille Le Ray
OBJECTIVE: To compare perinatal outcomes of twin pregnancies after oocyte donation (OD), in vitro fertilization (IVF) with autologous oocyte (AO), and non-IVF conception. DESIGN: Five-year retrospective cohort study. SETTING: Tertiary university medical center. PATIENT(S): All patients with twin pregnancies who gave birth after 24 weeks of gestation. The outcomes of 102 OD twin pregnancies were compared with those of 201 AO and 369 non-IVF twin pregnancies...
March 7, 2017: Fertility and Sterility
https://www.readbyqxmd.com/read/28239322/intestinal-barrier-integrity-and-function-in-infants-with-cholestasis
#6
Nagla H Abu Faddan, Tahra M K Sherif, Omnia A Mohammed, Khalid A Nasif, Ebtesam M El Gezawy
BACKGROUND/AIMS: The safety of the human body is maintained by effective monitoring of the mucosal surface integrity and protection against potentially harmful compounds. This function of the gut called intestinal barrier function can be affected by cholestasis and the absence of bile in the intestinal lumen. We aimed to determine whether the gut barrier integrity is impaired in infants with cholestasis by evaluation of the intestinal fatty acid binding proteins (I-FABP) and ileal bile acid binding protein (I-BABP) as markers of intestinal epithelial cell damage and plasma D-lactate level as a marker of gut wall permeability...
January 2017: Intestinal Research
https://www.readbyqxmd.com/read/28135431/retrospective-dual-center-study-of-parenteral-nutrition-associated-cholestasis-in-premature-neonates
#7
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
#8
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
#9
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
#10
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
#11
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...
April 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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