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Neonatal hepatitis

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https://www.readbyqxmd.com/read/28337030/prenatal-steroid-administration-leads-to-adult-pericardial-and-hepatic-steatosis-in-male-baboons
#1
A H Kuo, J Li, C Li, H F Huber, M Schwab, P W Nathanielsz, G D Clarke
Developmental programming studies indicate that glucocorticoids modify fetal development. We hypothesized that administration of the synthetic glucocorticoid (sGC) betamethasone to pregnant baboons at doses and stages of fetal life equivalent to human obstetric practice to decrease premature offspring morbidity and mortality, programs lipid metabolism. In 10-year-old male baboons (human equivalent 40) exposed in fetal life to betamethasone or saline, we quantified pericardial fat and hepatic lipid content with magnetic resonance imaging and spectroscopy...
March 24, 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/28334898/changing-knowledge-and-practices-of-mothers-on-newborn-care-through-mother-class-an-intervention-study-in-indonesia
#2
Narila Mutia Nasir, Yuli Amran, Yasuhide Nakamura
Poor knowledge and practices of newborn care may contribute to high neonatal mortality. This study aims to analyze the effect of prenatal education, namely 'mother class', on knowledge and practices of newborn care among mothers in Tangerang Selatan District, Indonesia. An intervention study with a quasi-experimental design was conducted. Using a questionnaire, 427 mothers were interviewed: an intervention group (n = 214) that attended mother class and a control group (n = 213) that had not. A home visit followed the intervention to assess newborn care practices...
March 3, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/28332100/expression-of-programmed-death-1-and-its-ligands-in-the-liver-of-biliary-atresia
#3
Pan-Liang Wang, Jun Wang, Ying Zhou, Xiao-Song Chen, Ke-Jun Zhou, Jie Wen, Jian-Jun Zhang, Wei Cai
BACKGROUND: An aberrant immune response is the predominant pathogenetic factor in biliary atresia (BA). Programmed death-1 (PD-1) and its two ligands, programmed death ligand-1 and programmed death ligand-2 (PD-L1 and PD-L2, respectively) play an important inhibitory role in immune reactions. We aimed to illustrate the expression of these molecules in BA. METHODS: Liver specimens were obtained from infants with BA during the Kasai procedure (early BA) and liver transplantation (late BA)...
March 22, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28330995/hepatic-fcrn-regulates-albumin-homeostasis-and-susceptibility-to-liver-injury
#4
Michal Pyzik, Timo Rath, Timothy T Kuo, Sanda Win, Kristi Baker, Jonathan J Hubbard, Rosa Grenha, Amit Gandhi, Thomas D Krämer, Adam R Mezo, Zachary S Taylor, Kevin McDonnell, Vicki Nienaber, Jan Terje Andersen, Atsushi Mizoguchi, Laurence Blumberg, Shalaka Purohit, Susan D Jones, Greg Christianson, Wayne I Lencer, Inger Sandlie, Neil Kaplowitz, Derry C Roopenian, Richard S Blumberg
The neonatal crystallizable fragment receptor (FcRn) is responsible for maintaining the long half-life and high levels of the two most abundant circulating proteins, albumin and IgG. In the latter case, the protective mechanism derives from FcRn binding to IgG in the weakly acidic environment contained within endosomes of hematopoietic and parenchymal cells, whereupon IgG is diverted from degradation in lysosomes and is recycled. The cellular location and mechanism by which FcRn protects albumin are partially understood...
March 22, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28324053/hepatic-insulin-resistance-and-altered-gluconeogenic-pathway-in-premature-baboons
#5
Lisa McGill-Vargas, Amalia Gastaldelli, Hanyu Liang, Diana Anzueto Guerra, Teresa Johnson-Pais, Steven Seidner, Donald McCurnin, Giovanna Muscogiuri, Ralph DeFronzo, Nicolas Musi, Cynthia Blanco
Premature infants have altered glucose regulation early in life and increased risk for diabetes in adulthood. Although prematurity leads to an increased risk of diabetes and metabolic syndrome in adult life, the role of hepatic glucose regulation and adaptation to an early extra-uterine environment in preterm infants remain unknown. The purpose of this study was to investigate developmental differences in glucose metabolism, hepatic protein content and gene expression of key insulin signaling/gluconeogenic molecules...
January 17, 2017: Endocrinology
https://www.readbyqxmd.com/read/28318185/neonatal-acute-liver-failure-a-diagnosis-challenge
#6
Mirta Ciocca, Fernando Álvarez
Neonatal acute liver failure is a rare, very severe disease with a high rate of mortality. It is clinically and etiologically different from acute liver failure seen in older children and adults. Coagulopathy with an international normalized ratio ≥ 3 is the critical parameter that defines it. The most common causes are fetal alloimmune hepatitis, previously called neonatal hemochromatosis, viral infections, metabolic disorders, and hemophagocytic lymphohistiocytosis. There is a group of treatable diseases that require a very early diagnosis for the prescription of an adequate treatment...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28317045/fibrolamellar-variant-of-hepatocellular-carcinoma-presenting-during-pregnancy-management-dilemmas
#7
Nagalapuram Vishnu, Aditya V Kulkarni, Sreenivasan Vidhyalakshmi, Swaminathan Sambandam, Prerna Garg, Venkatakrishnan LeelaKrishnan, Krishnaveni Janarthan, Gursharan Singh, Maninder Kaur, T V Chitra, Biku Joseph John
The Fibrolamellar variant of Hepatocellular Carcinoma (FLHCC) is a rare form of liver cancer that presents in the 3(rd) decade of life, is rarely associated with cirrhosis or chronic Hepatitis B/C virus infection, and usually presents with normal serum alpha-fetoprotein (AFP) levels. FLHCC presenting during pregnancy is extremely rare, with only 4 cases reported. We present a case of FLHCC in pregnancy and discuss the dilemmas in management. A 26 year-old primigravida, 26 weeks of gestation presented with a month's history of obstructive jaundice secondary...
February 2017: Ann Hepatobiliary Pancreat Surg
https://www.readbyqxmd.com/read/28299817/altered-expression-of-the-olr59-ethe1-and-slc10a2-genes-in-the-liver-of-f344-rats-by-neonatal-thyroid-hormone-disruption
#8
Kana Matsubara, Naoki Nakamura, Seigo Sanoh, Shigeru Ohta, Shigeyuki Kitamura, Naoto Uramaru, Shinichi Miyagawa, Taisen Iguchi, Nariaki Fujimoto
Many concerns have been expressed regarding the possible adverse effects of thyroid hormone-disrupting chemicals in the environment. The disruption of thyroid hormones in the neonatal period may lead to permanent effects on thyroid hormone homeostasis as well as related developmental disorders, as thyroid hormones are essential for regulating the growth and differentiation of many tissues. To understand the long-term alteration in gene expressions by neonatal administration of thyroid hormone-like chemicals in general, we identified genes whose expression was altered in the liver, an important component of the thyroid hormone axis, by neonatal exposure to triiodothyronine (T3)...
March 16, 2017: Journal of Applied Toxicology: JAT
https://www.readbyqxmd.com/read/28291718/cystathionine-beta-synthase-deficiency-alters-hepatic-phospholipid-and-choline-metabolism-post-translational-repression-of-phosphatidylethanolamine-n-methyltransferase-is-a-consequence-rather-than-a-cause-of-liver-injury-in-homocystinuria
#9
René L Jacobs, Hua Jiang, John P Kennelly, David J Orlicky, Robert H Allen, Sally P Stabler, Kenneth N Maclean
Classical homocystinuria (HCU) due to inactivating mutation of cystathionine β-synthase (CBS) is a poorly understood life-threatening inborn error of sulfur metabolism. A previously described cbs-/- mouse model exhibits a semi-lethal phenotype due to neonatal liver failure. The transgenic HO mouse model of HCU exhibits only mild liver injury and recapitulates multiple aspects of the disease as it occurs in humans. Disruption of the methionine cycle in HCU has the potential to impact multiple aspect of phospholipid (PL) metabolism by disruption of both the Kennedy pathway and phosphatidylethanolamine N-methyltransferase (PEMT) mediated synthesis of phosphatidylcholine (PC)...
March 2, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28289704/preferential-tnf%C3%AE-signaling-via-tnfr2-regulates-epithelial-injury-and-duct-obstruction-in-experimental-biliary-atresia
#10
Pranavkumar Shivakumar, Tatsuki Mizuochi, Reena Mourya, Sridevi Gutta, Li Yang, Zhenhua Luo, Jorge A Bezerra
Biliary atresia is an obstructive cholangiopathy of infancy that progresses to end-stage cirrhosis. Although the pathogenesis of the disease is not completely understood, previous reports link TNFα to apoptosis of the bile duct epithelium in the presence of IFNγ. Here, we investigate if TNFα signaling regulates pathogenic mechanisms of biliary atresia. First, we quantified the expression of TNFA and its receptors TNFR1 and TNFR2 in human livers and found an increased expression of the receptors at the time of diagnosis...
March 9, 2017: JCI Insight
https://www.readbyqxmd.com/read/28289498/intracardiac-fungal-mass-in-a-term-neonate
#11
Magdy M El-Sayed Ahmed, Mustafa Kurkluoglu, Conor F Hynes, Darren Klugman, Elena Puscasiu, Dilip S Nath
Systemic fungal infections pose insidious challenges in neonatal intensive care settings. We present the case of a 9-day-old male term neonate admitted for polymicrobial sepsis and hepatic dysfunction who later developed candidemia superinfection. Despite broad antifungal therapy, the fungemia was complicated by progressive growth of a fungus ball in the right ventricular outflow tract that threatened cardiac function. Surgical excision of the mass was undertaken by right atriotomy and histologic examination confirmed Candida albicans...
October 2016: Methodist DeBakey Cardiovascular Journal
https://www.readbyqxmd.com/read/28266016/clinical-and-mutation-analysis-of-24-chinese-patients-with-ornithine-transcarbamylase-deficiency
#12
Yongxian Shao, Minyan Jiang, Yunting Lin, Huifen Mei, Wen Zhang, Yanna Cai, Xueying Shu, Hao Hu, Xiuzhen Li, Li Liu
The principal aim of this study was to examine the clinical manifestations, biochemical features, and molecular genetic characteristics of Chinese patients with ornithine transcarbamylase deficiency (OTCD) at a single medical center. We retrospectively analyzed 24 patients (17 males and 7 females) diagnosed with OTCD between 2006 and 2015. Five male patients had a neonatal presentation; 12 male patients had late onset disease and 7 female patients presented as symptomatic. Patients with a neonatal presentation had the highest peak plasma ammonia and glutamine levels at diagnosis with a high mortality (80% versus 16% in late onset disease)...
March 7, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28253873/extended-clinical-features-associated-with-novel-glis3-mutation-a-case-report
#13
K A Alghamdi, A B Alsaedi, A Aljasser, A Altawil, Naglaa M Kamal
BACKGROUND: Mutations in the GLI-similar 3 (GLIS3) gene encoding the transcription factor GLIS3 are a rare cause of neonatal diabetes and congenital hypothyroidism with 12 reported patients to date. Additional features, previously described, include congenital glaucoma, hepatic fibrosis, polycystic kidneys, developmental delay, facial dysmorphism, osteopenia, sensorineural deafness, choanal atresia, craniosynostosis and pancreatic exocrine insufficiency. CASE PRESENTATION: We report a new case for consanguineous parents with homozygous novel mutation in GLIS3 gene who presented with neonatal diabetes mellitus, severe resistant congenital hypothyroidism, cholestatic liver disease, bilateral congenital glaucoma and facial dysmorphism...
March 2, 2017: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/28248119/familial-hemophagocytic-lymphohistiocytosis-from-autopsy-to-prenatal-diagnosis-report-of-a%C3%A2-case
#14
Marta Ježová, Renata Gaillyová
Hemophagocytic lymphohistiocytosis is a rare immunologic disorder affecting small children. It is characterized by an excessive and injurious immune response which turns rapidly fatal unless promptly and effectively treated. The main clinical signs are prolonged fever, hepatosplenomegaly, bleeding and laboratory findings of pancytopenia, increased serum transaminases, hypertriglyceridemia and hypofibrinogenemia. Four genes responsible for familiar hemophagocytic lymphohistiocytosis, which is inherited in autosomal recessive manner, have been identified so far...
2017: Ceskoslovenská Patologie
https://www.readbyqxmd.com/read/28246672/electronic-alerts-improve-immunization-rates-in-two-month-old-premature-infants-hospitalized-in-the-neonatal-intensive-care-unit
#15
Kimberly D Ernst
OBJECTIVE: To determine if an electronic alert improves 2 month immunization rates in infants remaining hospitalized in the neonatal intensive care unit. METHODS: Institutional Review Board-approved retrospective chart review of 261 infants with birth weights <2 kg and still hospitalized at ≥ 58 days. Charts were reviewed between 2009 and 2013, before and after the 2011 electronic alert was instituted in the electronic medical record from days 56 to 67 to remind providers that immunizations were due...
March 1, 2017: Applied Clinical Informatics
https://www.readbyqxmd.com/read/28239322/intestinal-barrier-integrity-and-function-in-infants-with-cholestasis
#16
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/28231660/-effect-of-telbivudine-on-infants-born-to-hbsag-positive-mothers-with-non-hypo-response-to-hepatitis-b-vaccine-during-their-second-and-third-trimesters-of-pregnancy
#17
X X Xu, B Wang, X F Wang, H X Wen, F Zhang, Z Q Yang, H Y Hao, T Wang, X H Shi, Z D Fu, B Wang, S P Wang
Objective: To explore the effect of telbivudine treatment in a prevention program on infants born to HBsAg-positive mothers with non-/hypo-responsiveness to hepatitis B vaccine. Methods: A retrospective cohort study with a total of 321 HBsAg-positive pregnant women and their infants enrolled, was conducted. The mothers were recruited from the Third People' s Hospital of Taiyuan, from July 2011 to January 2013. According to the situation of telbivudine intake in second and third trimesters of pregnancy, the participants were divided into two groups: with telbivudine-treated or as control...
February 10, 2017: Zhonghua Liu Xing Bing Xue za Zhi, Zhonghua Liuxingbingxue Zazhi
https://www.readbyqxmd.com/read/28222150/polymorphisms-and-features-of-cytomegalovirus-ul144-and-ul146-in-congenitally-infected-neonates-with-hepatic-involvement
#18
Gangqiang Guo, Liang Zhang, Sisi Ye, Yingying Hu, Baoqing Li, Xiangwei Sun, Chenchen Mao, Jianfeng Xu, Yiping Chen, Lifang Zhang, Xiangyang Xue
Human cytomegalovirus is a significant agent of hepatic involvement in neonates. In this study, we investigated the polymorphisms and features of the viral genes UL144 and UL146 as well as their significance to congenital hepatic involvement. In 79 neonates with congenital cytomegalovirus infection and hepatic involvement, full length UL144 and UL146 were successfully amplified in 73.42% and 60.76% of cases, respectively. Sequencing indicated that both genes were hypervariable. Notably, UL144 genotype B was highly associated with aspartate aminotransferase (P = 0...
2017: PloS One
https://www.readbyqxmd.com/read/28209492/reproductive-considerations-in-the-setting-of%C3%A2-chronic-viral-illness
#19
REVIEW
Brent M Hanson, Jessie A Dorais
Special considerations must be taken when patients with human immunodeficiency virus (HIV), hepatitis B, or hepatitis C desire to become pregnant. Patients with chronic viral illnesses desire to have children at rates similar to the general population, and options are available to decrease both vertical transmission and viral transmission between partners. Preconception counseling or consultation with fertility specialists is imperative in patients with HIV, hepatitis B, and hepatitis C so that reproductive goals can be addressed and optimized...
February 14, 2017: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28203327/a-rapid-screening-test-on-dried-blood-for-the-neonatal-diagnosis-of-tyrosinemia-type-i
#20
Farahnaz Bodaghkhan, Bita Geramizadeh, Abbas Abdollah Rajeh, Mahmoud Haghighat, Mohsen Dehghani, Naser Honar, Mojgan Zahmatkeshan, Mohammad-Hadi Imanieh
BACKGROUND: Tyrosinemia is an inherited metabolic disorder characterized by elevated levels of tyrosine and its metabolites in plasma. Without treatment, the disease will progress to hepatic and renal failure, so that without liver transplantation will cause death in less than 10 years of age. So, early diagnosis and treatment can be life saving and crucial. It means that with early treatment starting in the neonatal period, the patient can have normal life with very few restrictions in diets containing tyrosine and phenylalanine...
October 2016: Iranian Journal of Pediatrics
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