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neonatal cholestasis

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https://www.readbyqxmd.com/read/28818034/utility-of-hepatobiliary-scintigraphy-in-diagnosing-or-excluding-biliary-atresia-in-premature-neonates-and-full-term-infants-with-conjugated-hyperbilirubinemia-who-received-parenteral-nutrition
#1
Karine Barseghyan, Rangasamy Ramanathan, Thomas Chavez, Susan Harlan, Chuan-Hao Lin, Tania Mitsinikos, Claire McLean
PURPOSE: Hepatobiliary scintigraphy (HBS) is used to aid in the diagnosis of Biliary Atresia in full-term infants with conjugated hyperbilirubinemia. There is little information on the utility of the HBS in premature infants with conjugated hyperbilirubinemia and infants with parenteral nutrition associated cholestasis (PNAC). The purpose of this study is to assess the utility of HBS in differentiating Biliary Atresia and PNAC in premature neonates and full-term infants who received parenteral nutrition (PN)...
August 17, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28816798/solid-liver-lesions-in-an-infant-with-neonatal-cholestasis-is-it-always-malignant
#2
Zeren Bariş, Pelin Börcek, Kemal Murat Haberal, Figen Özçay
In this report we describe a patient with neonatal cholestasis who was found to have a liver lesion with suspicious imaging features, although ultimately it was histologically proved to be a pseudotumor. We discuss the characteristic features and imaging findings of macroregenerative nodules of the liver.
August 14, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28798896/pregnancy-outcomes-in-hiv-infected-women-experience-from-a-tertiary-care-center-in-india
#3
Vatsla Dadhwal, Aparna Sharma, Kavita Khoiwal, Dipika Deka, Plaboni Sarkar, P Vanamail
BACKGROUND AND OBJECTIVES: There is conflicting data on the effect of HIV infection as well as antiretroviral therapy (ART) on pregnancy outcome. The objectives of this study were to compare pregnancy outcomes in women with and without HIV infection, and to evaluate the effect of HAART on pregnancy in HIV-infected women. METHODS: This is a prospective case record analysis of 212 HIV-infected women delivering between 2002 and 2015, in a tertiary health care center in India...
2017: International Journal of MCH and AIDS
https://www.readbyqxmd.com/read/28780310/outcome-of-neonates-with-gastroschisis-at-different-gestational-ages-using-a-national-database
#4
Ruby Gupta, Erwin T Cabacungan
BACKGROUND/PURPOSE: The optimal time for delivery of neonates with a prenatal diagnosis of gastroschisis (GS) is controversial. We compared the outcomes for GS at three different gestational ages (GAs), 33-34 weeks, 35-36 weeks, and ≥ 37 weeks. METHODS: We analyze hospital discharge data of neonates with GS using the 2006, 2009 and 2012 Healthcare Cost and Utilization Project Kids' Inpatient Database (HCUPKIDS). Multivariable analysis was used to compare the association between GS outcomes and the three GAs...
July 22, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28763485/cxcr2-signaling-and-the-microbiome-suppress-inflammation-bile-duct-injury-and-the-phenotype-of-experimental-biliary-atresia
#5
Junbae Jee, Reena Mourya, Pranavkumar Shivakumar, Lin Fei, Michael Wagner, Jorge A Bezerra
Biliary atresia is progressive fibro-inflammatory cholangiopathy of young children. Central to pathogenic mechanisms of injury is the tissue targeting by the innate and adaptive immune cells. Among these cells, neutrophils and the IL-8/Cxcl-8 signaling via its Cxcr2 receptor have been linked to bile duct injury. Here, we aimed to investigate whether the intestinal microbiome modulates Cxcr2-dependent bile duct injury and obstruction. Adult wild-type (WT) and Cxcr2-/- mice were fed a diet supplemented with sulfamethoxazole/trimethoprim (SMZ/TMP) during pregnancy and lactation, and their pups were injected intraperitoneally with rhesus rotavirus (RRV) within 24 hours of life to induce experimental biliary atresia...
2017: PloS One
https://www.readbyqxmd.com/read/28745070/management-options-for-cholestatic-liver-disease-in-children
#6
Andrea Catzola, Pietro Vajro
Due to a peculiar age-dependent increased susceptibility, neonatal cholestasis affects the liver of approximately 1 in every 2500 term infants. A high index of suspicion is the key to an early diagnosis, and to implement timely, often life-saving treatments. Even when specific treatment is not available or curative, prompt medical management and optimization of nutrition are of paramount importance to survival and avoidance of complications. Areas covered: The present article will prominently focus on a series of newer diagnostic and therapeutic options of cholestasis in neonates and infants blended with consolidated established paradigms...
July 28, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28737145/congenital-hepatic-fibrosis-with-polycystic-kidney-disease-an-unusual-cause-of-neonatal-cholestasis
#7
Vani Bharani, G Vybhav Venkatesh, Uma Nahar Saikia, B R Thapa
Congenital hepatic fibrosis is characterized by hepatic fibrosis, portal hypertension, and renal cystic disease. Typical presentation of congenital hepatic fibrosis is in the form of portal hypertension, in adolescents and young adults. We present an unusual case of neonatal cholestasis with rapid deterioration within first 4 months of life, who was diagnosed to have congenital hepatic fibrosis with polycystic kidney disease on autopsy.
July 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28730136/early-diagnosis-of-abcb11-spectrum-liver-disorders-by-next-generation-sequencing
#8
Su Jeong Lee, Jung Eun Kim, Byung-Ho Choe, An Na Seo, Han-Ik Bae, Su-Kyeong Hwang
PURPOSE: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. METHODS: Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic diseases or ABCB11 spectrum liver disorders. Two families with proven ABCB11 spectrum liver disorders were subjected to genetic analyses to confirm the diagnosis and were provided genetic counseling...
June 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28699603/infants-with-extrahepatic-biliary-atresia-effect-of-follow-up-on-the-survival-rate-at-ege-university-medical-school-transplantation-center
#9
Miray Karakoyun, Maşallah Baran, Caner Turan, Murat Kılıç, Orkan Ergun, Sema Aydoğdu
BACKGROUND/AIMS: Biliary atresia (BA) is the main cause of neonatal cholestasis and the primary reason for infant liver transplants worldwide. It is an obliterative cholangiopathy observed only in children and caused by progressive inflammation and fibrosis of the bile duct. We collaborated with a liver transplantation center to investigate the effects of follow-up in patients with BA. MATERIALS AND METHODS: Medical records of 99 patients who were diagnosed with BA and monitored at our center from 1990 to 2002 (27 patients) and from 2003 to 2015 (72 patients) were analyzed retrospectively...
July 2017: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/28687948/role-of-hepatobiliary-scintigraphy-and-preoperative-liver-biopsy-for-exclusion-of-biliary-atresia-in-neonatal-cholestasis-syndrome
#10
Ankur Mandelia, Richa Lal, Nijagal Mutt
All diagnostic algorithms for Neonatal Cholestasis Syndrome (NCS) focus on differentiating numerous medical causes from Biliary Atresia (BA). No preoperative diagnostic algorithm has 100% diagnostic accuracy for BA and yet, timely diagnosis is crucial to optimize surgical outcome. Markers for high index of clinical suspicion for BA are: a "usually" well thriving infant with conjugated hyperbilirubinemia, raised gamma glutamyl transpeptidase, persistently "acholic" stools, firm hepatomegaly with dysmorphic, hypoplastic gall bladder...
July 8, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28665446/first-experience-on-bilirubin-removal-with-a-hemoadsorption-column-lixelle%C3%A2-in-a-child-with-cardiogenic-liver-injury
#11
Gabriella Bottari, Andrea Moscatelli, Enrico E Verrina, Franco Lerzo, Fabio S Taccone
INTRODUCTION: Hyperbilirubinemia may have deleterious effects on many organs, even after the neonatal age. Blood purification is effective in the treatment of hyperbilirubinemia. Recently some reports suggest the potential role of hemoadsorption columns in this setting. METHODS: We present the case of a 6-year-old child with severe hyperbilirubinemia due to congestive liver dysfunction, complicated by persistent inflammation, immunosuppression and catabolism syndrome (PICS)...
June 20, 2017: International Journal of Artificial Organs
https://www.readbyqxmd.com/read/28603204/a-6-year-old-girl-diagnosed-with-mevalonate-kinase-deficiency-who-had-hydrops-fetalis-and-neonatal-onset-cholestasis
#12
Yuriko Yamashita, Shinsuke Matsumoto, Ryugo Hiramoto, Isao Komori, Takayuki Tanaka, Ryuta Nishikomori, Toshio Heike, Shuichiro Umetsu, Ayano Inui
  We experienced a 6-year-old girl diagnosed with mevalonate kinase deficiency (MKD) who had cholestasis, anemia, and elevated inflammatory markers in neonatal period. She was admitted to our hospital because of fever and elevated inflammatory markers at 5 years 11months of age. Without using antibiotics, the fever and the inflammatory markers were spontaneously resolved. MKD was suspected from elevated serum IgD level and the recurrent febrile attacks. The genetic test revealed heterozygous mutation of p...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28600664/optimizing-care-and-outcome-of-neonatal-cholestasis-are-we-on-the-right-track
#13
REVIEW
Anshu Srivastava
No abstract text is available yet for this article.
August 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28588613/preoperative-serum-il-12p40-is-a-potential-predictor-of-kasai-portoenterostomy-outcome-in-infants-with-biliary-atresia
#14
Shaimaa Samy Goda, Mohamed Ahmed Khedr, Soha Zaki Elshenawy, Tarek Mohamed Ibrahim, Hanaa Ahmed El-Araby, Mostafa Mohamed Sira
The standard-of-care treatment for biliary atresia (BA) is surgical restoration of bile flow by Kasai portoenterostomy. We aimed to study serum interleukin- (IL-) 12p40, a natural antagonist for the proinflammatory IL-12p70, and its relation to surgical outcomes of BA. The study included 75 infants with neonatal cholestasis: BA group (n = 25), non-BA cholestasis group (n = 30), and neglected BA group (n = 20), in addition to thirty healthy neonates serving as controls. IL-12p40 was measured by ELISA in all individuals and a second assessment was performed 3 months postoperatively in the BA group...
2017: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/28587329/mosapride-combined-with-probiotics-on-gastrointestinal-function-and-growth-in-premature-infants
#15
Ai-Mei Zhang, Zhi-Qun Sun, Li-Ming Zhang
The aim of this study was to investigate the effects of mosapride combined with probiotics on gastrointestinal function and growth and development in premature infants. A total of 240 premature infants treated at Weifang People's Hospital between June 2012 and May 2015 who matched our criteria were randomly divided into three groups of 80 cases each. Group A received routine treatment, group B received routine treatment combined with live B. subtilis and E. faecium granules with multivitamins (Medilac-Vita), and group C received routine treatment and Saccharomyces boulardii sachets (Bioflor)...
June 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28583322/ocular-findings-in-patients-with-cholestatic-disorders-of-infancy-a-single-centre-experience
#16
Hanaa El-Karaksy, Dalia Hamed, Hanan Fouad, Engy Mogahed, Heba Helmy, Fotouh Hasanain
BACKGROUND AND STUDY AIMS: Neonatal cholestasis can be associated with ocular findings that might aid in its diagnosis, e.g., Alagille syndrome (AGS) and Niemann Pick disease (NPD). We aimed to investigate the frequency of ocular manifestations in infants with cholestasis. PATIENTS AND METHODS: This cross-sectional study included cholestatic infants presenting to the Paediatric Hepatology Unit, Cairo University Paediatric Hospital, Cairo, Egypt. All infants underwent examination of lid, ocular motility, anterior and posterior segments and measurement of intraocular pressure, cycloplegic refraction, ocular ultrasonography and vision...
June 2, 2017: Arab Journal of Gastroenterology: the Official Publication of the Pan-Arab Association of Gastroenterology
https://www.readbyqxmd.com/read/28581354/perinatal-outcomes-associated-with-intrahepatic-cholestasis-of-pregnancy
#17
Christina Annette Herrera, Tracy A Manuck, Gregory J Stoddard, Michael W Varner, Sean Esplin, Erin A S Clark, Robert M Silver, Alexandra G Eller
OBJECTIVE: The objective of this study is to examine perinatal outcomes associated with cholestasis of pregnancy according to bile acid level and antenatal testing practice. STUDY DESIGN: Retrospective cohort study of women with symptoms and bile acid testing from 2005 to 2014. Women were stratified by bile acid level: no cholestasis (<10 μmol/L), mild (10-39 μmol/L), moderate (40-99 μmol/L), and severe (≥100 μmol/L). The primary outcome was composite neonatal morbidity (hypoxic ischemic encephalopathy, severe intraventricular hemorrhage, bronchopulmonary dysplasia, necrotizing enterocolitis, or death)...
June 5, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28574590/pregnancy-outcomes-after-liver-transplantation-in-finland
#18
Mirjami Mattila, Helena Kemppainen, Helena Isoniemi, Päivi Polo-Kantola
INTRODUCTION: Pregnancy after liver transplantation is possible but associated with increased risk of obstetrical complications. We report here for the first time the pregnancy outcomes after liver transplantation in Finland. MATERIAL AND METHODS: All of the 25 pregnancies ending in deliveries after liver transplantation in Finland in 1998-2015 were analyzed. The data were collected from the mothers' medical records. The main outcome measures included pregnancy complications and the mode of delivery...
September 2017: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/28571223/neonatal-hyperthyroidism-with-fulminant-liver-failure-a-case-report
#19
Mohammed Hasosah, Khalid Alsaleem, Mansour Qurashi, Abdullah Alzaben
Neonatal hyperthyroidism is a rare disease that is seen in infants born to mothers with Graves' disease. Hepatic manifestation of neonatal hyperthyroidism is extremely rare. We describe a neonate with fulminant liver failure secondary to neonatal hyperthyroidism caused by maternal Graves's disease. The baby was admitted with low birth weight and hepatosplenomegaly. At day 2 of life, the baby was irritable and he developed respiratory distress and fulminant hepatic failure which required mechanical ventilation...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28565794/novel-two-step-derivation-method-for-the-synchronous-analysis-of-inherited-metabolic-disorders-using-urine
#20
Xiao-Qi Sheng, Yi-Chao Wang
The aim of the present study was to conduct preliminary clinical screening and monitoring using a novel two-step derivatization process of urine in five categories of inherited metabolic disease (IMD). Urine samples (100 µl, containing 2.5 mmol/l creatinine) were taken from patients with IMDs. The collected urine was then treated using a two-step derivatization method (with oximation and silylation at room temperature), where urea and protein were removed. In the first step of the derivatization, α-ketoacids and α-aldehyde acids were prepared by oximation using novel oximation reagents...
May 2017: Experimental and Therapeutic Medicine
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