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neonatal cholestasis

Linling Zhu, Yu Zhang, Yifeng Liu, Runjv Zhang, Yiqing Wu, Yun Huang, Feng Liu, Meigen Li, Saijun Sun, Lanfeng Xing, Yimin Zhu, Yiyi Chen, Li Xu, Liangbi Zhou, Hefeng Huang, Dan Zhang
This study was carried out to explore associations between assisted reproductive technology (ART) and maternal and neonatal outcomes compared with similar outcomes following spontaneously conceived births. We conducted a retrospective cohort study of pregnancies conceived by ART (N = 2641) during 2006-2014 compared to naturally conceived pregnancies (N = 5282) after matching for maternal age and birth year. Pregnancy complications, perinatal complications and neonatal outcomes of enrolled subjects were investigated and analysed by multivariate logistic regression...
October 20, 2016: Scientific Reports
Xiang Kong, Yan Kong, Fangyuan Zhang, Tingting Wang, Jin Yan
BACKGROUND: Intrahepatic cholestasis of pregnancy (ICP) is a specific pregnancy-related disorder without standard medical therapies. Ursodeoxycholic acid (UDCA) is the most used medicine, but the efficacy and safety of UDCA remain uncertain. Several meta-analyses had been made to assess the effects of UDCA in ICP. However, the samples were not large enough to convince obstetricians to use UDCA. We conducted a meta-analysis to evaluate the effects and safety of UDCA in patients with ICP, which included only randomized controlled trials (RCTs)...
October 2016: Medicine (Baltimore)
Sajan Agarwal, Bikrant Bihari Lal, Dinesh Rawat, Archana Rastogi, Kishore G S Bharathy, Seema Alam
OBJECTIVE: To study the clinical and laboratory profile of children with progressive familial intrahepatic cholestasis (PFIC) and evaluate their outcome. METHODS: The study is a retrospective review of all cases diagnosed with PFIC between January 2011 and July 2015. All children underwent histopathological examination and immunostaining. Management was done as per institute's protocol. RESULTS: There were a total of 24 PFIC cases (PFIC 1-2, PFIC 2-19, PFIC 3-3)...
September 2016: Journal of Clinical and Experimental Hepatology
Andreas Repa, Ruth Lochmann, Lukas Unterasinger, Michael Weber, Angelika Berger, Nadja Haiden
BACKGROUND: Parenteral nutrition associated cholestasis (PNAC) is a frequently observed pathology in extremely low birth weight (ELBW) infants. Its pathogenesis is determined by the composition and duration of parenteral nutrition (PN) as well as the tolerance of enteral feeds (EF). "Aggressive" nutrition is increasingly used in ELBW infants to improve postnatal growth. Little is known about the effect of "aggressive" nutrition on the incidence of PNAC. We analyzed the influence of implementing an "aggressive" nutritional regimen on the incidence of PNAC and growth in a cohort of ELBW infants...
2016: PeerJ
Eugene Ct Lau, Adrian Ch Fung, Kenneth Ky Wong, Paul Kh Tam
BACKGROUND: Necrotizing enterocolitis in premature neonates often results in bowel resection and stoma formation. One way to promote bowel adaptation before stoma closure is to introduce proximal loop effluents into the mucous fistula. In this study, we reviewed our experience with distal loop refeeding with respect to control group. METHODS: All patients with necrotizing enterocolitis between 2000 and 2014 necessitating initial diverting enterostomies and subsequent stoma closure in a tertiary referral center were included...
September 15, 2016: Journal of Pediatric Surgery
Danielle Usatin, Melissa Fernandes, Isabel E Allen, Emily R Perito, James Ostroff, Melvin B Heyman
OBJECTIVES: To systematically review risks and summarize reported complication rates associated with the performance of endoscopic retrograde cholangiopancreatography (ERCP) in children during the past 2 decades. STUDY DESIGN: A systematic literature search of MEDLINE, Embase, and Web of Science from January 1995 to January 2016 was conducted for observational studies published in English. Studies reporting ERCP complications in patients <21 years without history of liver transplant or cholecystectomy were included...
September 20, 2016: Journal of Pediatrics
Nalini Sharma, S Panda, Ahanthem Santa Singh
OBJECTIVE: To study the incidence, maternal, and perinatal outcomes in intrahepatic cholestasis of pregnancy with active management. MATERIALS AND METHODS: This observational study included 48 cases diagnosed as intrahepatic cholestasis of pregnancy. Medical treatment and active management (fetal surveillance and termination of pregnancy at 37-38 weeks) were offered to all. Maternal and perinatal outcomes are studied. RESULTS: Incidence of ICP was 2...
October 2016: Journal of Obstetrics and Gynaecology of India
Abdelmoneim Em Kheir, Wisal Ma Ahmed, Israa Gaber, Sara Ma Gafer, Badreldin M Yousif
Cholestasis in early infancy represents a diagnostic dilemma and most of these infants suffer either from extrahepatic biliary atresia or idiopathic neonatal hepatitis. Differentiation between the two conditions may be extremely difficult both clinically and biochemically, and a diagnostic liver biopsy is usually required. We report on a Sudanese infant who presented at the age of 4 weeks with prolonged cholestatic jaundice, abdominal ultrasound was inconclusive, HIDA scan was suggestive of extrahepatic biliary atresia and the diagnosis of idiopathic neonatal hepatitis was only reached by liver biopsy...
2016: Sudan J Paediatr
Gurleen Wander, Francesa Neuberger, Mandish K Dhanjal, Catherine Nelson-Piercy, May Ching Soh
Most published cases of cytomegalovirus infection in pregnancy relate to congenital abnormalities in neonates infected in early pregnancy, while the mother remains asymptomatic. We describe a diagnostically challenging case of an immunosuppressed woman with scleroderma who developed deranged liver function tests attributed to intrahepatic cholestasis of pregnancy and haemolysis, elevated liver enzymes and low platelets syndrome but was ultimately found to have disseminated cytomegalovirus. Cytomegalovirus can present in a myriad of ways...
September 2016: Obstetric Medicine
Colin D Gause, Madoka Hayashi, Courtney Haney, Daniel Rhee, Omar Karim, Brian W Weir, Dylan Stewart, Jeffrey Lukish, Henry Lau, Fizan Abdullah, Estelle Gauda, Howard I Pryor
BACKGROUND/PURPOSE: Premature neonates can develop intraabdominal conditions requiring emergent bowel resection and enterostomy. Parenteral nutrition (PN) is often required, but results in cholestasis. Mucous fistula refeeding allows for functional restoration of continuity. We sought to determine the effect of refeeding on nutrition intake, PN dependence, and PN associated hepatotoxicity while evaluating the safety of this practice. METHODS: A retrospective review of neonates who underwent bowel resection and small bowel enterostomy with or without mucous fistula over 2years was undertaken...
November 2016: Journal of Pediatric Surgery
Viara Shoumnalieva-Ivanova, Ivan Tanev, Yani Zdravkov, Simeon Monov, Russka Shumnalieva
Aagenaes syndrome, also called lymphoedema cholestasis syndrome 1 (LSC1), is characterized by neonatal intrahepatic cholestasis, often lessening and becoming intermittent with age and severe chronic lymphoedema, mainly affecting the lower extremities. The condition is autosomal recessively inherited, and the gene is located on chromosome 15q. The locus, LCS1, was mapped to a 6.6 cM region on chromosome 15. Angioid streaks are visible irregular crack-like dehiscences in bruch's membrane that are associated with atrophic degeneration of the overlying retinal pigment epithelium...
September 10, 2016: International Ophthalmology
Ying-Hsien Huang, Hsiang-Hung Shih, Mao-Meng Tiao, Chao-Cheng Huang, Kuang-Che Kuo, Fu-Chen Huang, Ya-Ling Yang, Jiin-Haur Chuang
BACKGROUND/PURPOSE: Viral infections and innate immunity signaling, especially Toll-like receptor 7 (TLR7) have been implicated in the pathogenesis of biliary atresia (BA). Administration of rhesus rotavirus-type A to newborn Balb/c mice produces inflammatory obstruction of bile ducts, which resembles human BA. However, whether activation of TLR7 signaling plays a role in neonatal hepatobiliary injury remains to be investigated. METHODS: TLR7 agonist, imiquimod (R837), was intraperitoneally administered to Balb/c mice within 24 hours of birth and then every other day...
August 3, 2016: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
Sangeethapriya Duraiswamy, Jeanne S Sheffield, Donald Mcintire, Kenneth Leveno, Marlyn J Mayo
BACKGROUND: The most common cause of jaundice during pregnancy in the United States (US) is still attributed to viral hepatitis, despite the dramatic drop in incidence of viral hepatitis in the US. OBJECTIVE: We hypothesized that viral hepatitis is no longer a frequent etiology of jaundice among the pregnant population in the US and sought to identify the contemporary causes of elevated bilirubin during pregnancy as well as to quantify the associated risk to the mother and fetus...
September 1, 2016: Digestive Diseases and Sciences
Ling Wang, Xinran Cheng, Li Yan, Yan Wei, Fang Tang, Xin Dong, Yanjiao Yuan, Yanmei Xie
OBJECTIVE: To analyze the clinical features and potential mutations of the SLC25A13 gene in a boy affected with neonatal intrahepatic cholestasis. METHODS: Clinical data and peripheral venous blood sample of the child, and peripheral venous blood samples of both parents, were collected. All coding exons of the SLC25A13 gene were amplified with PCR and subjected to direct DNA sequencing. RESULTS: The boy was found to be a compound heterozygote carrying c...
October 2016: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Virginia Mirra, Sergio Bernasconi
This review focuses key advances in different pediatric fields that were published in Italian Journal of Pediatrics and in international journals in 2015. Weaning studies continue to show promise for preventing food allergy. New diagnostic tools are available for identifying the allergic origin of allergic-like symptoms. Advances have been reported in obesity, short stature and autoimmune endocrine disorders. New molecules are offered to reduce weight gain and insulin-resistance in obese children. Regional investigations may provide suggestions for preventing short stature...
August 27, 2016: Italian Journal of Pediatrics
Qi Lu, Shupeng Cheng, Min Zhou, Jialin Yu
BACKGROUND: Necrotizing enterocolitis (NEC) in neonates is devastating, and risk-factor identification is crucial. This study aimed to evaluate risk factors for NEC in different gestational age (GA) groups. METHODS: Risk factors associated with NEC were investigated using a retrospective case-control design. Patients with Bell's Stage NEC≥II were divided into three groups based on GA: I, <34 weeks; II, ≥34 weeks but <37 weeks; III, ≥37 weeks. Each case was paired with two GA- and weight-matched controls...
June 22, 2016: Pediatrics and Neonatology
Mahdi Alsaleem, Lina Saadeh, Amrit Misra, Shailender Madani
Isolated ACTH deficiency (IAD) is a rare cause of neonatal cholestasis and hypoglycaemia. This diagnosis has a 20% mortality potential if unrecognised. We describe a case of an infant presenting with cholestatic jaundice and hypoglycaemia. The patient had laboratory findings suggestive of IAD, which was later confirmed with molecular genetic testing. One of the mutations this patient had is a new finding. The patient was started on glucocorticoid replacement therapy after which his bilirubin and glucose levels normalised...
2016: BMJ Case Reports
Nina Bögershausen, Umut Altunoglu, Filippo Beleggia, Gökhan Yigit, Hülya Kayserili, Peter Nürnberg, Yun Li, Janine Altmüller, Bernd Wollnik
Kabuki syndrome (KS) is a rare developmental disorder characterized by multiple congenital malformations, postnatal growth retardation, intellectual disability, and recognizable facial features. It is mainly caused by mutations in either KMT2D or KDM6A. We describe a 14-year-old boy with KS presenting with an unusual combination of bilateral microphthalmia with orbital cystic venous lymphatic malformation and neonatal cholestasis with bile duct paucity, in addition to the typical clinical features of KS. We identified the novel KMT2D mutation c...
August 17, 2016: American Journal of Medical Genetics. Part A
K P Srikanth, Inusha Panigrahi, Babu Ram Thapa, Kim Vaiphei
We present a rare presentation of cystic fibrosis with neonatal cholestasis. Histological features of mucoviscidosis were present in liver involving the biliary tract, intestinal mucosa, pancreas, and lung. Besides, there was a rare association with autosomal dominant type of polycystic renal disease.
July 2016: Indian Journal of Pathology & Microbiology
Ajay Kumar Jain, Abhineet Sharma, Sumit Arora, Keith Blomenkamp, Ik Chan Jun, Robert Luong, David John Westrich, Aayush Mittal, Paula M Buchanan, Miguel A Guzman, John Long, Brent A Neuschwander-Tetri, Jeffery Teckman
BACKGROUND: Parenteral nutrition (PN) is a lifesaving therapy but is associated with gut atrophy and cholestasis. While bile acids (BAs) can modulate intestinal growth via gut receptors, the gut microbiome likely influences gut proliferation and inflammation. BAs also regulate the bile salt export pump (BSEP) involved in cholestasis. We hypothesized that the BA receptor agonist oleanolic acid (OA) regulates gut TGR5 receptor and modulates gut microbiota to prevent PN-associated injury...
August 8, 2016: JPEN. Journal of Parenteral and Enteral Nutrition
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