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Intrahepatic Cholestasis

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https://www.readbyqxmd.com/read/28074213/-intrahepatic-cholestasis-of-pregnancy-rare-but-important
#1
REVIEW
A E Kremer, K Wolf, S Ständer
Intrahepatic cholestasis of pregnancy (ICP) is a liver-specific disorder occurring in approximately 0.5-2.0% of all pregnancies with a considerable variation in certain ethnic groups. ICP usually runs a benign course for the mother and is characterized by maternal pruritus mainly in the third trimester, elevated transaminases and fasting total serum bile salts and increased fetal adverse events. The etiology of ICP is only partially understood but seems to be multifactorial. Cholestasis-inducing effects of certain female sex hormones and their metabolites play an important role in genetically susceptible women...
January 10, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28074212/-specific-dermatoses-of-pregnancy
#2
REVIEW
C M Ambros-Rudolph, M Sticherling
The specific dermatoses of pregnancy represent a heterogeneous group of inflammatory skin diseases related to pregnancy and/or the postpartum period. A clinically relevant classification has been well established over the past 10 years and includes pemphigoid gestationis, polymorphic eruption of pregnancy, intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. The hallmark of all four entities is severe pruritus that is accompanied by characteristic skin changes. While some of these dermatoses are distressing only to the mother because of pruritus, others may be associated with significant fetal risks...
January 10, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28073941/prognostic-roles-of-tetrahydroxy-bile-acids-in-infantile-intrahepatic-cholestasis
#3
Chee-Seng Lee, Akihiko Kimura, Jia-Feng Wu, Yen-Hsuan Ni, Hong-Yuan Hsu, Mei-Hwei Chang, Hiroshi Nittono, Huey-Ling Chen
Tetrahydroxy bile acids (THBAs) are hydrophilic and are present at minimal or undetectable levels in healthy human adults but are present at high levels in bile salt export pump (abcb11)-knockout mice. The roles of THBAs in human cholestatic diseases are unclear. We aimed to investigate the presence of THBAs in patients with infantile intrahepatic cholestasis and its correlation with outcome. Urinary bile acids were analysed by GC-MS. Data were compared between good (N=21) (disease-free before 1 year old) and poor prognosis groups (N=19)...
January 10, 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/28064265/a-novel-truncation-mutation-in-atp8b1-gene-in-progressive-familial-intrahepatic-cholestasis
#4
Anjali Sharma, Ujjal Poddar, Shikha Agnihotry, Rakesh Aggarwal
BACKGROUND: Progressive familial intrahepatic cholestasis has been only infrequently reported from India. CASE CHARACTERISTICS: An Indian girl with progressive cholestatic liver disease beginning during infancy, normal gamma-glutamyl transpeptidase levels, parental consanguinity, positive family history and a fatal outcome. OBSERVATION: A novel, homozygous mutation (c.[589_592inv;592_593insA]) in ATP8B1 gene, with a markedly truncated protein (p...
December 15, 2016: Indian Pediatrics
https://www.readbyqxmd.com/read/28055006/biliary-tract-instillation-of-a-smac-mimetic-induces-trail-dependent-acute-sclerosing-cholangitis-like-injury-in-mice
#5
Maria Eugenia Guicciardi, Anuradha Krishnan, Steven F Bronk, Petra Hirsova, Thomas S Griffith, Gregory J Gores
Primary sclerosing cholangitis (PSC) is a cholestatic liver disease of unknown etiopathogenesis characterized by fibrous cholangiopathy of large and small bile ducts. Systemic administration of a murine TNF-related apoptosis-inducing ligand (TRAIL) receptor agonist induces a sclerosing cholangitis injury in C57BL/6 mice, suggesting endogenous TRAIL may contribute to sclerosing cholangitis syndromes. Cellular inhibitor of apoptosis proteins (cIAP-1 and cIAP-2) are negative regulators of inflammation and TRAIL receptor signaling...
January 5, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28052628/management-of-cholestatic-disease-in-2017
#6
REVIEW
Elsemieke de Vries, Ulrich Beuers
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most frequent chronic cholestatic liver diseases and serve as model diseases to discuss the management of cholestasis in 2017 in the lecture that is summarized in this report. PBC and PSC are characterized by inflammation and fibrosis of small intrahepatic (PBC) or larger intra- and/or extrahepatic (PSC) bile ducts. Bile duct damage leads to cholestasis and can progress to liver fibrosis and even cirrhosis. Various genetic, environmental and endogenous factors may contribute to the development of chronic cholestatic liver diseases, but the exact pathogenesis of PBC and PSC has not been clarified...
January 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28049946/ursodeoxycholic-acid-ameliorates-intrahepatic-cholestasis-independent-of-biliary-bicarbonate-secretion-in-vil2-kd-kd-mice
#7
Ryo Hatano, Kotoku Kawaguchi, Fumitaka Togashi, Masato Sugata, Shizuka Masuda, Shinji Asano
Ursodeoxycholic acid (UDCA) is a hydrophilic bile acid that possesses many pharmacological effects, including increasing bile flow, changing the hydrophobicity of the bile acid pool, and modulation of the immune response. UDCA has been approved for treating cholestatic liver disease, such as primary biliary cholangitis. However, several unanticipated severe side effects of UDCA are observed in cholestatic patients, and its pharmacological benefits remain controversial. We reported that ezrin-knockdown (Vil2(kd/kd)) mice exhibited severe hepatic injury because of a functional disorder in bile duct fluidity and alkalinity regulation, resembling human intrahepatic cholestatic disease...
2017: Biological & Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/28045770/clinical-variability-following-partial-external-biliary-diversion-in-familial-intrahepatic-cholestasis-1-deficiency
#8
James E Squires, Neslihan Celik, Amy Morris, Kyle Soltys, George Mazariegos, Benjamin Shneider, Robert H Squires
OBJECTIVES: Familial intrahepatic cholestasis 1 (FIC1) deficiency is caused by a mutation in the ATP8B1 gene. Partial external biliary diversion (PEBD) is pursued to improve pruritus and arrest disease progression. Our aim is to describe clinical variability after PEBD in FIC1 disease. METHODS: We performed a single-center, retrospective review of genetically confirmed FIC1 deficient patients who received PEBD. Clinical outcomes after PEBD were cholestasis, pruritus, fat-soluble vitamin supplementation, growth, and markers of disease progression that included splenomegaly and aspartate aminotransferase-to-platelet ratio index...
December 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28039538/maternal-liver-elasticity-determined-by-acoustic-radiation-force-impulse-elastosonography-in-intrahepatic-cholestasis-of-pregnancy
#9
Orkun Cetin, Erbil Karaman, Harun Arslan, Ibrahim Akbudak, Recep Yildizhan, Ali Kolusari
PURPOSE: Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-specific liver disorder characterized by maternal pruritus and impaired liver function. The objective of the study was to evaluate maternal liver elasticity by acoustic radiation force impulse (ARFI) elastosonography in ICP and to compare it with that in healthy pregnant women. METHODS: This descriptive, case-control study consisted of 33 women with healthy pregnancies and 22 women with ICP in the third trimester of gestation...
December 30, 2016: Journal of Medical Ultrasonics
https://www.readbyqxmd.com/read/28028430/a-child-with-debilitating-pruritus
#10
Nikhil Sonthalia, Samit S Jain, Vinay B Pawar, Vinay G Zanwar, Ravindra G Surude, Pravin M Rathi, Kshitij K Munde, Sandeep Bavdekar
We describe a case of two-year-old boy presenting with debilitating pruritus, patchy alopecia and jaundice since the age of 6 months. On evaluation he had intrahepatic cholestasis with persistently raised serum alkaline phosphatase, normal Gamma glutamyl transferase and raised serum bile acid levels. His liver biopsy showed bland cholestasis and electron microscopy showed granular bile suggestive of progressive familial intrahepatic cholestasis type I. Medical therapy with ursodeoxycholic acid, cholestyramine, rifampicin with nutritional modification was successful in alleviating the symptoms and correcting the nutritional status...
October 24, 2016: Clinics and Practice
https://www.readbyqxmd.com/read/28027587/analysis-of-surgical-interruption-of-the-enterohepatic-circulation-as-a-treatment-for-pediatric-cholestasis
#11
Kasper S Wang, Greg Tiao, Lee M Bass, Paula M Hertel, Douglas Mogul, Nanda Kerkar, Matthew Clifton, Colleen Azen, Laura Bull, Philip Rosenthal, Dylan Stewart, Riccardo Superina, Ronen Arnon, Molly Bozic, Mary L Brandt, Patrick A Dillon, Annie Fecteau, Kishore Iyer, Binita Kamath, Saul Karpen, Frederick Karrer, Kathleen M Loomes, Cara Mack, Peter Mattei, Alexander Miethke, Kyle Soltys, Yumirle P Turmelle, Karen West, Jessica Zagory, Cat Goodhue, Benjamin L Shneider
: To evaluate the efficacy of non-transplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 Alagille syndrome (ALGS), 16 Familial Intrahepatic Cholestasis-1 (FIC1), 18 Bile Salt Export Pump (BSEP) disease, & 4 others with low γ-glutamyl transpeptidase disease (levels <100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBD), 11 ileal exclusions (IE) and 7 gallbladder-to-colon diversion (GBC)...
December 27, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28012258/functional-defect-of-variants-in-the-adenosine-triphosphate-binding-sites-of-abcb4-and-their-rescue-by-the-cystic-fibrosis-transmembrane-conductance-regulator-potentiator-ivacaftor-vx-770
#12
Jean-Louis Delaunay, Alix Bruneau, Brice Hoffmann, Anne-Marie Durand-Schneider, Véronique Barbu, Emmanuel Jacquemin, Michèle Maurice, Chantal Housset, Isabelle Callebaut, Tounsia Aït-Slimane
: ABCB4 (MDR3) is an adenosine triphosphate (ATP)-binding cassette (ABC) transporter expressed at the canalicular membrane of hepatocytes, where it mediates phosphatidylcholine (PC) secretion. Variations in the ABCB4 gene are responsible for several biliary diseases, including progressive familial intrahepatic cholestasis type 3 (PFIC3), a rare disease that can be lethal in the absence of liver transplantation. In this study, we investigated the effect and potential rescue of ABCB4 missense variations that reside in the highly conserved motifs of ABC transporters, involved in ATP binding...
November 5, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28009332/effect-of-intrahepatic-cholestasis-of%C3%A2-pregnancy-on-maternal-serum-screening%C3%A2-tests
#13
G G Türkmen, H Timur, Z Yilmaz, A Kirbas, K Daglar, A Tokmak, D Uygur, N Danişman
OBJECTIVE: In this study, we aimed to evaluate whether the changes in the first and second trimester maternal serum biochemical markers used for prenatal screening are associated with euploid pregnancies complicated by intrahepatic cholestasis of pregnancy (ICP). METHODS: A total of 94 pregnant women were included in this retrospective comparative study. Thirty-seven women whose pregnancy was complicated with ICP constituted the study group whereas 57 of them constituted the control group...
2016: Journal of Neonatal-perinatal Medicine
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#14
Güzide Doğan, Fatih Düzgün, Serdar Tarhan, Yeliz Çağan Appak, Erhun Kasırga
Congenital intrahepatic portosystemic shunts are rare in children. Portosystemic venous malformations are characterized by extreme clinical variability. We report a full-term 33-day-old male infant presenting with neonatal jaundice. On physical examination, he had generalized icterus and the liver was palpable 3.5 cm below the right costal margin. He had no other symptoms. Laboratory tests showed AST 632 U/L, ALT 198 U/L, total bilirubin 12.1 mg/dL, conjugated bilirubin 10.2 mg/dL, alkaline phosphatase 753 U/L, GGT 47 U/L and glucose 67 U/L...
December 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/28003028/worsening-cholestasis-and-possible-cefuroxime-induced-liver-injury-following-successful-therapeutic-endoscopic-retrograde-cholangiopancreatography-for-a-distal-common-bile-duct-stone-a-case-report
#15
Madunil Anuk Niriella, Ravindu Sujeewa Kumarasena, Anuradha Supun Dassanayake, Aloka Pathirana, Janaki de Silva Hewavisenthi, Hithanadura Janaka de Silva
BACKGROUND: Cefuroxime very rarely causes drug-induced liver injury. We present a case of a patient with paradoxical worsening of jaundice caused by cefuroxime-induced cholestasis following therapeutic endoscopic retrograde cholangiopancreatography for a distal common bile duct stone. CASE PRESENTATION: A 51-year-old, previously healthy Sri Lankan man presented to our hospital with obstructive jaundice caused by a distal common bile duct stone. Endoscopic retrograde cholangiopancreatography with stone extraction, common bile duct clearance, and stenting failed to improve the cholestasis, with paradoxical worsening of his jaundice...
December 21, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27998400/-factors-related-to-the-influence-on-congenital-malformation-of-body-surface-in-shaanxi-province
#16
J Ying, Q Lei, B W Qin, P F Qu, F L Lei, S N Dang, L X Zeng, H Yan
Objective: To investigate the influencing factors on congenital malformation of body surface. Methods: Multi-stage stratified random sampling method was adopted. A questionnaire survey was conducted among the childbearing aged women who experienced more than 28 weeks pregnancy or having definite pregnancy outcomes in Shaanxi, during 2010-2013. Results: Results from the logistic regression model showed that factors as: intrahepatic cholestasis of pregnancy (OR=21.76, 95%CI: 4.46-106.25), histories with abnormal pregnancy or reproduction (OR=11...
December 10, 2016: Zhonghua Liu Xing Bing Xue za Zhi, Zhonghua Liuxingbingxue Zazhi
https://www.readbyqxmd.com/read/27958341/downregulation-of-peroxiredoxin-3-by-hydrophobic-bile-acid-induces-mitochondrial-dysfunction-and-cellular-senescence-in-human-trophoblasts
#17
Wei-Bin Wu, Ramkumar Menon, Yue-Ying Xu, Jiu-Ru Zhao, Yan-Lin Wang, Yuan Liu, Hui-Juan Zhang
Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-specific disorder characterised by raised bile acids in foetal-maternal circulation, which threatens perinatal health. During the progression of ICP, the effect of oxidative stress is underscored. Peroxiredoxin-3 (PRDX3) is a mitochondrial antioxidant enzyme that is crucial to balance intracellular oxidative stress. However, the role of PRDX3 in placental trophoblast cells under ICP is not fully understood. We demonstrated that the level of PRDX3 was downregulated in ICP placentas as well as bile acids-treated trophoblast cells and villous explant in vitro...
December 13, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27938561/-value-of-albumin-in-diagnosis-of-neonatal-intrahepatic-cholestasis-caused-by-citrin-deficiency
#18
J Zhang, X H Wang, Y Z Ye, X B Xie, Y Lu, L J Ye, H Yu
Objective: To investigate the clinical value of albumin (Alb) in the diagnosis of neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). Methods: A retrospective analysis was performed for the clinical data of 90 children with NICCD who visited Children's Hospital of Fudan University from January 2007 to December 2014, and according to the content of Alb, these children were divided into Alb < 30 g/L (LA) group with 20 children and Alb ≥30 g/L (NA) group with 70 children. The clinical manifestations, results of laboratory examination, results of blood tandem mass spectrometry and urine gas chromatography-mass spectrometry, and gene detection results were compared between the two groups...
October 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/27936482/intrahepatic-cholestasis-of-pregnancy-icp-case-report-and-review-of-the-literature
#19
V Keitel, C Dröge, S Stepanow, T Fehm, E Mayatepek, K Köhrer, D Häussinger
Intrahepatic cholestasis of pregnancy (ICP) represents the most common pregnancy-related liver disease in women. Women frequently present in the third trimester with pruritus and elevated serum bile acid and/or alanine transaminase levels. Clinical symptoms quickly resolve after delivery; however, recurrence in subsequent pregnancies has to be expected. Intrahepatic cholestasis of pregnancy is associated with increased perinatal complications, such as premature delivery, meconium staining of the amniotic fluid, respiratory distress, low Apgar scores, and even stillbirth...
December 2016: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/27932171/recurrence-of-progressive-familial-intrahepatic-cholestasis-type-2-phenotype-after-living-donor-liver-transplantation-a-case-report
#20
K Masahata, S Uehara, S Ibuka, K Nakahata, Y Hasegawa, H Kondou, R Kubitz, T Ueno
BACKGROUND: Progressive familial intrahepatic cholestasis 2 (PFIC2) is the result of mutations in the ABCB11, which encodes for bile salt export pump (BSEP). An absence of BSEP in the canalicular membrane causes cholestasis and leads to the development of end-stage liver disease in the first decade of life. Liver transplantation (LT) has been considered curative for BSEP disease. However, patients with PFIC2 having undergone LT have recently been reported to develop recurrence of cholestasis together with the clinical and histological features of primary BSEP disease...
November 2016: Transplantation Proceedings
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