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neonatal cholestasis

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https://www.readbyqxmd.com/read/29659898/cholesterol-metabolism-is-enhanced-in-the-liver-and-brain-of-children-with-citrin-deficiency
#1
Satoshi Hirayama, Hironori Nagasaka, Akira Honda, Haruki Komatsu, Takahiro Kodama, Ayano Inui, Ichiro Morioka, Shunsaku Kaji, Tsuyoshi Ueno, Kenji Ihara, Mariko Yagi, Zenro Kizaki, Kazuhiko Bessho, Hiroki Kondou, Tohru Yorifuji, Hirokazu Tsukahara, Kazumoto Iijima, Takashi Miida
Context: Citrin-deficient infants present neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), which resolves at 12 months. Thereafter, they have normal liver function associated with hypercholesterolemia, and a preference for lipid-rich carbohydrate-restricted diets. However, some develop adult-onset type II citrullinemia (CTLN2), which is associated with metabolic abnormalities. Objectives: To identify the causes of hypercholesterolemia in citrin-deficient children post-NICCD...
April 5, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29658448/-clinical-features-and-prognosis-of-preterm-infants-with-varying-degrees-of-bronchopulmonary-dysplasia
#2
Wen-Li Li, Fa-Lin Xu, Ming Niu, Meng-Di Liu, Hui-Fang Dong
OBJECTIVE: To study the clinical features and prognosis of preterm infants with varying degrees of bronchopulmonary dysplasia (BPD). METHODS: The clinical data of 144 preterm infants with a gestational age of <32 weeks who were admitted to the neonatal intensive care unit from March 2014 to March 2016 and were diagnosed with BPD were collected. According to the severity of BPD, these preterm infants were divided into mild group with 81 infants and moderate/severe group with 63 infants...
April 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29651749/medium-chain-triglycerides-supplement-therapy-with-a-low-carbohydrate-formula-can-supply-energy-and-enhance-ammonia-detoxification-in-the-hepatocytes-of-patients-with-adult-onset-type-ii-citrullinemia
#3
Kiyoshi Hayasaka, Chikahiko Numakura, Mitsunori Yamakawa, Tetsuo Mitsui, Hisayoshi Watanabe, Hiroaki Haga, Masahide Yazaki, Hiromasa Ohira, Yasuo Ochiai, Toshiyuki Tahara, Tamio Nakahara, Noriyo Yamashiki, Takahiro Nakayama, Takashi Kon, Hiroshi Mitsubuchi, Hiroshi Yoshida
Citrin, encoded by SLC25A13, constitutes the malate-aspartate shuttle, the main NADH-shuttle in the liver. Citrin deficiency causes neonatal intrahepatic cholestasis (NICCD) and adult-onset type II citrullinemia (CTLN2). Citrin deficiency is predicted to impair hepatic glycolysis and de novo lipogenesis, resulting in hepatic energy deficit. Secondary decrease in hepatic argininosuccinate synthetase (ASS1) expression has been considered a cause of hyperammonemia in CTLN2. We previously reported that medium-chain triglyceride (MCT) supplement therapy with a low-carbohydrate formula was effective in CTLN2 to prevent a relapse of hyperammonemic encephalopathy...
April 12, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29610156/successful-liver-transplants-for-liver-failure-associated-with-pyruvate-kinase-deficiency
#4
Marie-Eve Chartier, Lara Hart, Massimiliano Paganelli, Najma Ahmed, Marc Bilodeau, Fernando Alvarez
Pyruvate kinase deficiency (PKD) is the most common cause of congenital nonspherocytic chronic hemolytic anemia, and patients normally present with mild to severe anemia, unconjugated hyperbilirubinemia, and splenomegaly. Only a few reports of PKD have documented its association with severe, progressive liver failure. In all those cases, the patients died before liver transplant (LT) or immediately after transplant. We report 2 case patients with liver failure associated with PKD who successfully underwent LT and splenectomy: an infant who presented with neonatal cholestasis and a young adult with a severe form of PKD and having been transfusion dependent during childhood...
April 2018: Pediatrics
https://www.readbyqxmd.com/read/29601463/conjugated-hyperbilirubinemia-in-the-neonate-and-young-infant
#5
Anna K Weiss, Parag V Vora
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.
April 2018: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29594193/expression-of-intrahepatic-cd3-cd4-and-cd8-t-cells-in-biliary-atresia
#6
Behairy E Behairy, Nermine Ehsan, Magdy Anwer, Alif Allam, Ibramem El-Henawy, Nesreen Abdel Hameed, Haidy M Zakaria
Aim of the study: Assessment of the expression of cluster of differentiation (CD)3, CD4, and CD8 T cells in biliary atresia (BA) cases in comparison to neonatal cholestasis other than BA. Material and methods: This study included 79 patients: 34 patients with BA (BA group) and 35 patients with neonatal cholestasis due to causes other than BA (cholestasis group), and 10 normal liver donor as a control group. Immunohistochemical staining or CD3, CD4, and CD8 T cells in liver tissues for the 3 groups were evaluated...
March 2018: Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29507303/impacts-of-different-methods-of-conception-on-the-perinatal-outcome-of-intrahepatic-cholestasis-of-pregnancy-in-twin-pregnancies
#7
Chun Feng, Wen-Juan Li, Rong-Huan He, Xi-Wen Sun, Guirong Wang, Li-Quan Wang
Twin pregnancies have a higher prevalence of intrahepatic cholestasis of pregnancy (ICP) than single pregnancies. It is unknown whether in vitro fertilization-embryo transfer (IVF-ET) influences the fetal outcomes in twin pregnancies complicated by ICP. This study aimed to explore the impact of IVF-ET on the perinatal outcomes of ICP in twin pregnancy. Clinical data from 142 twin pregnant women complicated with ICP were retrospectively analyzed, including 51 patients who conceived through IVF-ET (IVF group) and 91 patients with spontaneous conception (SC group)...
March 5, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29505478/retrospective-analysis-of-children-with-%C3%AE-1-antitrypsin-deficiency
#8
Atakan Comba, Fatma Demirbaş, Gönül Çaltepe, Esra Eren, Ayhan G Kalayci
BACKGROUND: α-1 Antitrypsin (AAT) deficiency is the most frequently occurring genetic liver disorder. The association among classical α-1 antitrypsin deficiency (AATD), chronic liver disease, and cirrhosis is common in adult patients but rare in children. AIM: To assess the clinical characteristics of children with AATD and to compare symptoms between homozygous and heterozygous children. MATERIALS AND METHODS: The study included 20 children who were found to have mutant Pi alleles...
March 2, 2018: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29499989/clinical-pathologic-and-genetic-features-of-neonatal-dubin-johnson-syndrome-a-multicenter-study-in-japan
#9
Takao Togawa, Tatsuki Mizuochi, Tokio Sugiura, Hironori Kusano, Ken Tanikawa, Takato Sasaki, Fumio Ichinose, Seiichi Kagimoto, Takahisa Tainaka, Hiroo Uchida, Shinji Saitoh
OBJECTIVE: To clarify the clinical, pathologic, and genetic features of neonatal Dubin-Johnson syndrome. STUDY DESIGN: Ten patients with neonatal Dubin-Johnson syndrome were recruited from 6 pediatric centers in Japan between September 2013 and October 2016. Clinical and laboratory course, macroscopic and microscopic liver findings, and molecular genetic findings concerning ATP-binding cassette subfamily C member 2 (ABCC2) were retrospectively and prospectively examined...
February 28, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29480924/intrahepatic-cholestasis-of-pregnancy-a-review-of-diagnosis-and-management
#10
Amber M Wood, Elizabeth G Livingston, Brenna L Hughes, Jeffrey A Kuller
Importance: Intrahepatic cholestasis of pregnancy (ICP) complicates approximately 0.2% to 2% of pregnancies and can lead to increased fetal risks in pregnancy. Objective: This review aims to increase the knowledge of women's health care providers regarding the diagnosis, management, and fetal risks associated with ICP. Results: The diagnosis of ICP is based on symptoms of pruritus that typically include the palms and soles, as well as elevated bile acid levels...
February 2018: Obstetrical & Gynecological Survey
https://www.readbyqxmd.com/read/29476221/mri-based-decision-tree-model-for-diagnosis-of-biliary-atresia
#11
Yong Hee Kim, Myung-Joon Kim, Hyun Joo Shin, Haesung Yoon, Seok Joo Han, Hong Koh, Yun Ho Roh, Mi-Jung Lee
OBJECTIVES: To evaluate MRI findings and to generate a decision tree model for diagnosis of biliary atresia (BA) in infants with jaundice. METHODS: We retrospectively reviewed features of MRI and ultrasonography (US) performed in infants with jaundice between January 2009 and June 2016 under approval of the institutional review board, including the maximum diameter of periportal signal change on MRI (MR triangular cord thickness, MR-TCT) or US (US-TCT), visibility of common bile duct (CBD) and abnormality of gallbladder (GB)...
February 23, 2018: European Radiology
https://www.readbyqxmd.com/read/29419856/-analysis-of-slc25a13-gene-mutations-in-five-infants-with-neonatal-intrahepatic-cholestasis-caused-by-citrin-deficiency
#12
Junjie Xu, Min Gao, Yuqiang Lyu, Yunping Tang, Xuxia Wei, Lu Yang, Kaihui Zhang, Yi Liu, Zhongtao Gai
OBJECTIVE To identify potential mutations in five infants with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). METHODS The SLC25A13 gene was analyzed by next-generation sequencing. Suspected mutations were confirmed by PCR and Sanger sequencing in the probands and their parents. Impact of novel mutations was predicted with PolyPhen-2 software. RESULTS All neonates have harbored mutations of the SLC25A13 gene. Eight mutations were discovered, which included two novel mutations (c.1357A>G and c...
February 10, 2018: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/29415837/parenteral-nutrition-associated-cholestasis-and-triglyceridemia-in-surgical-term-and-near-term-neonates-a-pilot-randomized-controlled-trial-of-two-mixed-intravenous-lipid-emulsions
#13
Luís Pereira-da-Silva, Sara Nóbrega, Maria Luísa Rosa, Marta Alves, Ana Pita, Daniel Virella, Ana Luísa Papoila, Micaela Serelha, Gonçalo Cordeiro-Ferreira, Berthold Koletzko
BACKGROUND: Cholestasis is a common complication in infants receiving prolonged parenteral nutrition (PN). We studied the effects of two intravenous lipid emulsions composed with either 30% soybean oil, 30% medium-chain triglycerides (MCT), 25% olive oil, and 15% fish oil (SMOF) or with 50% MCT and 50% soybean oil n-6 (MCT/SOY) on the incidence of cholestasis in surgical term and near-term neonates. METHODS: A single-center, double-blinded, randomized controlled trial compared the incidence of cholestasis using either SMOF or MCT/SOY in neonates born at gestational age ≥34 weeks undergoing major surgery...
December 2017: Clinical Nutrition ESPEN
https://www.readbyqxmd.com/read/29406331/reduced-immunohistochemical-expression-of-hnf1%C3%AE-and-foxa2-in-liver-tissue-can-discriminate-between-biliary-atresia-and-other-causes-of-neonatal-cholestasis
#14
Usama F Shaalan, Noha L Ibrahim, Nermine A Ehsan, Mervat M Sultan, Ghada M Naser, Mohamed O Abd El-Fatah
Biliary atresia (BA) is a necroinflammatory occlusive cholangiopathy that affects infants. Genetic and environmental factors has been proposed for its occurrence. The objectives of this study was to investigate the protein expression of 2 important genes regulating ductal plate remodeling, hepatocyte nuclear factor 1-beta (Hnf1β) and the fork head box protein A2 (FoxA2) in liver tissue from patients with BA and to compare their expression with other causes of neonatal cholestasis (NC). This retrospective study included 60 pediatric patients, 30 with BA and 30 with NC...
February 5, 2018: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/29397405/early-differential-diagnosis-methods-of-biliary-atresia-a-meta-analysis
#15
REVIEW
Li Wang, Yuan Yang, Yang Chen, Jianghua Zhan
PURPOSE: To evaluate the accuracy of early differential diagnosis methods of biliary atresia in patients with infantile cholestasis. METHODS: We searched PubMed, EMBASE and the Web of Science databases for articles evaluated the early differential diagnosis methods of biliary atresia. The methodological quality of each study was assessed with version 2 of the Quality Assessment of Diagnostic Accuracy Studies tool. Two reviewers extracted data independently. Pooled sensitivity, specificity, positive likelihood ratio (LR +), negative likelihood ratio (LR -), diagnostic odds ratio (DOR) with 95% CIs were calculated to assess each diagnosis method...
April 2018: Pediatric Surgery International
https://www.readbyqxmd.com/read/29389827/detection-of-cytomegalovirus-in-liver-tissue-by-pcr-in-infants-with-neonatal-cholestasis
#16
Akshat Goel, Sneha Chaudhari, Jyoti Sutar, Gauri Bhonde, Sushmita Bhatnagar, Vainav Patel, Vikrant Bhor, Ira Shah
AIM: Cytomegalovirus (CMV) is associated with neonatal cholestasis (NC). Diagnosis of CMV infection is most often based upon either positive blood CMV IgM or CMV blood PCR. Isolation of CMV in liver tissues in patients with NC has rarely been reported. This study was undertaken to see if CMV is present in liver tissues of patients with NC and evaluate the correlation between positive CMV PCR in liver tissue with the serology and blood PCR. METHODS: This study was conducted in 31 infants with NC from June 2015-Dec 2016...
January 31, 2018: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/29388792/transcription-factor-gata6-a-novel-marker-and-putative-inducer-of-ductal-metaplasia-in-biliary-atresia
#17
Tea Soini, Marjut Pihlajoki, Noora Andersson, Jouko Lohi, Kari A Huppert, David A Rudnick, Stacey S Huppert, David B Wilson, Mikko P Pakarinen, Markku Heikinheimo
Biliary atresia (BA), a neonatal liver disease, is characterized by obstruction of extrahepatic bile ducts with subsequent cholestasis, inflammation, and progressive liver fibrosis. To gain insights into the pathophysiology of BA, we focused attention on GATA6, a transcription factor implicated in biliary development. Early in fetal development GATA6 expression is evident in cholangiocytes and hepatocytes, but by late gestation it is extinguished in hepatocytes. Utilizing a unique set of BA liver samples collected before and after successful portoenterostomy (PE), we found that GATA6 expression is markedly upregulated in hepatocytes of patients with BA compared to healthy and cholestatic disease controls...
February 1, 2018: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/29350689/safety-of-intravitreal-injection-of-ranibizumab-in-early-pregnancy-a-series-of-three-cases
#18
P Fossum, C Couret, B Briend, M Weber, L Lagarce
PurposeRanibizumab is used in the treatment of choroidal neovascularization (CNV). Although systemic exposure to ranibizumab is low after ocular administration, its mechanism of action must be regarded as potentially teratogenic and embryo-fetotoxic. Women are advised to wait 3 months after the last dose of treatment with ranibizumab before conceiving. Little is known about the fetal side-effects of this drug.MethodsThree pregnant women were treated with ranibizumab. One patient had idiopathic CNV.ResultsAfter receiving injections at 10 and 21 weeks after her last menstrual period (LMP), she gave birth to a healthy child...
April 2018: Eye
https://www.readbyqxmd.com/read/29250459/intrahepatic-cholestasis-of-pregnancy-refractory-to-multiple-medical-therapies-and-plasmapheresis
#19
Adam John Covach, William Nicholas Rose
Objectives  We report on a patient suffering from intractable itching secondary to intrahepatic cholestasis of pregnancy (ICP) unresponsive to conventional medical therapies. She was started on a regimen of therapeutic plasma exchange (TPE), which is often efficacious in relieving patient's itching from all causes of cholestasis, including ICP. Methods  We performed a retrospective review of a patient's medical record. Results  Following initial TPE, the patient reported dramatic relief of her itching and consequent insomnia...
October 2017: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/29178522/direct-hyperbilirubinemia-in-infants-with-congenital-heart-disease
#20
Jun Fujishiro, Masahiko Sugiyama, Tetsuya Ishimaru, Miho Watanabe, Kaori Sato, Noriko Hoshino, Chizue Uotani, Mai Kutsukake, Yoichiro Hirata, Akira Oka
BACKGROUND: The association between congenital heart disease (CHD) and infantile cholestasis, a key finding for the diagnosis of biliary atresia (BA), has not been previously investigated. The aim of this study was therefore to investigate the characteristics of direct hyperbilirubinemia (D-HB) in infants with CHD. METHODS: All neonates admitted to the present hospital and diagnosed with CHD in 2015 and 2016 were included. D-HB (direct bilirubin ≥ 2.0 mg/dL) at ≤60 days of age and other clinical parameters were retrospectively reviewed...
February 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
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