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neonatal cholestasis

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https://www.readbyqxmd.com/read/29925342/ursodeoxycholic-acid-versus-phenobarbital-for-cholestasis-in-the-neonatal-intensive-care-unit
#1
Tamorah Lewis, Simisola Kuye, Ashley Sherman
BACKGROUND: Although neonates and young infants with cholestasis are commonly treated with either phenobarbital or ursodeoxycholic acid (ursodiol), there is no evidence that phenobarbital is effective for this indication. Our objective was to compare the effectiveness of ursodiol and phenobarbital for the treatment of cholestasis in a diverse NICU population. METHODS: This is a retrospective cohort study including infants with cholestasis who were admitted to a Level IV NICU between January 2010 and December 2015...
June 20, 2018: BMC Pediatrics
https://www.readbyqxmd.com/read/29904725/serum-level-of-interleukin-13-receptor-alpha-2-in-infants-with-biliary-atresia-is-it-of-value
#2
Nermin Adawy, Hanaa El-Araby, Alif Allam, Soha Elshenawy, Mohammed Khedr, Yasmine Ibrahim, Haidy M Zakaria
Aim of the study: We aimed to assess the utility of serum level IL-13Rα2 receptors as a non-invasive marker for early diagnosis of biliary atresia (BA) and selection of BA patients indicated for Kasai portoenterostomy. Material and methods: The study included 60 infants with neonatal cholestasis in three groups; early BA group ( n = 20), delayed BA group ( n = 20) and non-BA cholestasis group ( n = 20). A fourth group of 20 healthy neonates ( n = 20) served as controls...
June 2018: Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29893700/correlation-of-apri-index-with-metavir-index-in-children-with-neonatal-cholestasis-without-biliary-atresia
#3
Ira Shah, Nikita Madgum
INTRODUCTION AND AIM: Neonatal cholestasis constitutes for 19 to 33% of all chronic liver disease in India. Cholestasis leads to fibrosis of liver and ultimately cirrhosis. There are various methods of diagnosis of fibrosis of liver like fibroscan, APRI index, FIB-4, fibro index, forns index, heap score, magnetic elastography. Here we are comparing APRI index with METAVIR index in patients with neonatal cholestasis without biliary atresia and determining whether APRI index can be used as a tool to determine fibrosis in these patients...
July 2018: Annals of Hepatology
https://www.readbyqxmd.com/read/29886774/labor-induction-in-indicated-moderate-to-late-preterm-birth
#4
Charline Bertholdt, Olivier Morel, Matthieu Dap, Marion Choserot, Hélène Minebois
INTRODUCTION: The primary objective of the study was to evaluate the success of labor induction for indicated moderate and late preterm birth. As secondary objectives, the mode of delivery was assessed. MATERIAL AND METHODS: This is an observational study conducted in a tertiary care unit between 2013 and 2015. All patients who underwent labor induction for indicated preterm birth between 32 + 0 and 36 + 6 weeks of gestation (as premature rupture of membranes, preeclampsia, intrauterine growth restriction, fetomaternal alloimmunization, or intrahepatic cholestasis) were included...
June 10, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29881821/age-dependent-glycosylation-of-the-sodium-taurocholate-cotransporter-polypeptide-from-fetal-to-adult-human-livers
#5
Camillo Sargiacomo, Hoda El-Kehdy, Guillaume Pourcher, Bruno Stieger, Mustapha Najimi, Etienne Sokal
Sodium taurocholate cotransporter polypeptide (NTCP), mainly expressed on the sinusoidal membrane of hepatocytes, is one of the major transporters responsible for liver bile acid (BA) re-uptake. NTCP transports conjugated BA from the blood into hepatocytes and is crucial for correct enterohepatic circulation. Studies have shown that insufficient hepatic clearance of BA correlates with elevated serum BA in infants younger than 1 year of age. In the current study, we investigated human NTCP messenger RNA and protein expression by using reverse-transcription quantitative polymerase chain reaction and immunoblotting in isolated and cryopreserved human hepatocytes from two different age groups, below and above 1 year of age...
June 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29809326/-bronze-baby-syndrome-an-unpredictable-complication-of-phototherapy-a-case-report
#6
Juan Sebastián Peinado-Acevedo, Estephanía Chacón-Valenzuela, Laura Liliana Rodríguez-Moncada
The bronze baby syndrome is an infrequent dyschromia resulting from phototherapy in newborn babies with neonatal jaundice. Even though the common phenotype has been described in patients with direct neonatal hyperbilirubinemia secondary to cholestasis, several cases of patients with indirect neonatal hyperbilirubinemia who have managed to reverse it have been reported, as well as patients with isolated hyperbilirubinemia.Currently, the physiopathology of this condition is still a subject of controversy and, therefore, there is a lack of clear conducts for its correct diagnosis and treatment...
May 1, 2018: Biomédica: Revista del Instituto Nacional de Salud
https://www.readbyqxmd.com/read/29807952/clinical-and-biochemical-characteristics-of-infants-with-prolonged-neonatal-jaundice
#7
S L Siu, L W Chan, A N Kwong
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. To study the diagnostic yield of this approach, we carried out a retrospective study of all infants referred for prolonged jaundice...
May 25, 2018: Hong Kong Medical Journal, Xianggang Yi Xue za Zhi
https://www.readbyqxmd.com/read/29740092/bile-acids-and-their-respective-conjugates-elicit-different-responses-in-neonatal-cardiomyocytes-role-of-gi-protein-muscarinic-receptors-and-tgr5
#8
Effendi Ibrahim, Ivan Diakonov, Dulasi Arunthavarajah, Teresa Swift, Mary Goodwin, Saraid McIlvride, Vanya Nikolova, Catherine Williamson, Julia Gorelik
Bile acids are recognised as bioactive signalling molecules. While they are known to influence arrhythmia susceptibility in cholestasis, there is limited knowledge about the underlying mechanisms. To delineate mechanisms underlying fetal heart rhythm disturbances in cholestatic pregnancy, we used FRET microscopy to monitor cAMP release and contraction measurements in isolated rodent neonatal cardiomyocytes. The unconjugated bile acids CDCA, DCA and UDCA and, to a lesser extent, CA were found to be relatively potent agonists for the GPBAR1 (TGR5) receptor and elicit cAMP release, whereas all glyco- and tauro- conjugated bile acids are weak agonists...
May 8, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29727438/germline-hemizygous-deletion-of-gene-hnf1b-associated-with-a-case-of-severe-neonatal-cholestasis-and-hepatocarcinoma
#9
Cécile de Leusse, André Maues De Paula, Audrey Ascherod, Chistelle Parache, Hery Geraldine, Mathilde Cailliez, Chantal Missirian, Alexandre Fabre
No abstract text is available yet for this article.
April 27, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29709288/is-the-bishop-koop-procedure-useful-in-severe-jejunoileal-atresia
#10
Yanfen Peng, Haiqing Zheng, Qiuming He, Zhe Wang, Hong Zhang, Puja Bk Chaudhari, Wei Zhong, Jiakang Yu
PURPOSE: The aim of this study was to report our experience using the Bishop-Koop procedure for the treatment of various surgical problems of jejunoileal atresia including luminal discrepancy, complex meconium peritonitis, type IIIb and type IV atresia which we defined as severe jejunoileal atresia. METHODS: This retrospective study was performed on the patients with severe jejunoileal atresia who underwent Bishop-Koop procedure at a tertiary center in China over a five year of period...
April 5, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29659898/cholesterol-metabolism-is-enhanced-in-the-liver-and-brain-of-children-with-citrin-deficiency
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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...
July 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
#19
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...
May 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29480924/intrahepatic-cholestasis-of-pregnancy-a-review-of-diagnosis-and-management
#20
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
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