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Cholestasis

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https://www.readbyqxmd.com/read/28324003/biliary-anomalies-in-patients-with-hnf1b-diabetes
#1
Jarno Lt Kettunen, Helka Parviainen, Päivi J Miettinen, Martti Färkkilä, Marjo Tamminen, Pia Salonen, Eila Lantto, Tiinamaija Tuomi
Context: The clinical spectrum of organogenetic anomalies associated with HNF1B mutations is heterogeneous. Besides cystic kidney disease, diabetes, and various other manifestations, odd cases of mainly neonatal and post-transplant cholestasis have been described. The biliary phenotype is incompletely defined. Objective: To systematically characterize HNF1B-related anomalies in the bile ducts by imaging with MRI or MRCP. Setting and Patients: Fourteen patients with HNF1B mutations in the catchment area of the Helsinki University hospital were evaluated with upper abdominal MRI and MRCP...
March 17, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28319428/two-cases-of-first-onset-intrahepatic-cholestasis-of-pregnancy-associated-with-moderate-ovarian-hyperstimulation-syndrome-after-ivf-treatment-and-review-of-the-literature
#2
Mehmet Firat Mutlu, Koray Aslan, Ismail Guler, Ilknur Mutlu, Mehmet Erdem, Nuray Bozkurt, Ahmet Erdem
Intrahepatic cholestasis of pregnancy (ICP) is an uncommon disorder, which generally occurs in the second and third trimester of pregnancy with symptoms of pruritus. The cause of ICP is unknown but genetic, hormonal and environmental factors contribute to its pathogenesis. The aetiology of ICP is unclear but elevation in oestrogen levels thought to cause ICP is typically seen in the third trimester of pregnancy, and for this reason it is not usually considered in the differential diagnosis of pruritus and liver function disorders in the first trimester of the pregnancy...
March 20, 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28314860/a-newborn-screening-method-for-cerebrotendinous-xanthomatosis-using-bile-alcohol-glucuronides-and-metabolite-ratios
#3
Frédéric M Vaz, Albert H Bootsma, Willem Kulik, Aad Verrips, Ron A Wevers, Peter C Schielen, Andrea E DeBarber, Hidde H Huidekoper
Cerebrotendinous xanthomatosis (CTX) is a treatable neurodegenerative metabolic disorder of bile acid synthesis where symptoms can be prevented if treatment with chenodeoxycholic acid supplementation is initiated early in life, making CTX an excellent candidate for newborn screening. We developed a new dried blood spot screening assay for this disorder based on different ratios between the accumulating cholestanetetrol glucuronide (tetrol) and specific bile acids/bile acid intermediates, without the need for derivatization...
March 17, 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/28302210/-risk-factors-for-neonatal-pulmonary-hemorrhage-in-the-neonatal-intensive-care-unit-of-a-municipal-hospital
#4
Jie Fan, Ming-Yan Hei, Xi-Lin Huang, Xiao-Ping Li
OBJECTIVE: To investigate the risk factors for neonatal pulmonary hemorrhage (NPH) in the neonatal intensive care unit (NICU) of a municipal hospital, and to provide a basis for the early identification and treatment of NPH. METHODS: A total of 112 neonates who were admitted to the NICU of Shaoyang Central Hospital of Hunan Province and diagnosed with NPH were enrolled as the case group. A nested case-control method was used to select, as a control group (n=224), the neonates who underwent the treatment with an assisted mechanical ventilator and did not experience pulmonary hemorrhage...
March 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28298162/expression-and-significance-of-dendritic-cells-and-th17-treg-in-serum-and-placental-tissues-of-patients-with-intrahepatic-cholestasis-of-pregnancy
#5
Xiang Kong, Yan Kong, Fangyuan Zhang, Tingting Wang, Xiaotong Zhu
PURPOSE: Dendritic cells (DCs) are involved in immune system, which can also regulate the differentiation of T helper 17 (Th17) and regulatory T cells (Treg). DCs and Th17/Treg participate in preeclampsia and recurrent spontaneous abortion (RSA), but there is still lack of research in intrahepatic cholestasis of pregnancy (ICP). The aim was to evaluate the expression and significance of CD83(+)DCs, CD1a(+)DCs, interleukin-17 (IL-17) and IL-35 in serum and placental tissues of patients with ICP...
March 15, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28292174/extrahepatic-manifestations-of-primary-biliary-cholangitis
#6
REVIEW
Sara L Chalifoux, Peter G Konyn, Gina Choi, Sammy Saab
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogren's syndrome, thyroid dysfunction and systemic sclerosis. It is thought that patients with PBC are at increased risk of developing these extrahepatic manifestations, almost all of which are autoimmune, because patients with autoimmune disease are at higher risk of developing another autoimmune condition...
March 16, 2017: Gut and Liver
https://www.readbyqxmd.com/read/28289714/bile-acids-initiate-cholestatic-liver-injury-by-triggering-a-hepatocyte-specific-inflammatory-response
#7
Shi-Ying Cai, Xinshou Ouyang, Yonglin Chen, Carol J Soroka, Juxian Wang, Albert Mennone, Yucheng Wang, Wajahat Z Mehal, Dhanpat Jain, James L Boyer
Mechanisms of bile acid-induced (BA-induced) liver injury in cholestasis are controversial, limiting development of new therapies. We examined how BAs initiate liver injury using isolated liver cells from humans and mice and in-vivo mouse models. At pathophysiologic concentrations, BAs induced proinflammatory cytokine expression in mouse and human hepatocytes, but not in nonparenchymal cells or cholangiocytes. These hepatocyte-specific cytokines stimulated neutrophil chemotaxis. Inflammatory injury was mitigated in Ccl2(-/-) mice treated with BA or after bile duct ligation, where less hepatic infiltration of neutrophils was detected...
March 9, 2017: JCI Insight
https://www.readbyqxmd.com/read/28286628/hemophagocytic-lymphohistiocytosis-an-unclear-nosologic-entity-case-report-of-an-adult-man-with-rising-of-amylase-and-lipase-and-spinal-cord-infiltration
#8
Moris Sangineto, Antonio Perrone, Pasquale Agosti, Viera Boccuti, Anna Campobasso, Carlo Sabbà
Here we present the case of a 57-years old patient affected by hemophagocytic lymphohistiocytosis (HLH), a rare disease characterized by an uncontrolled immune activation, resulting in clinical and biochemical manifestations of extreme inflammation. In a previous hospitalization, the patient showed fever, hepato-splenomegaly, pancytopenia, hyperferrtitinemia, lymphadenopathy and cholestasis. No diagnosis was done, however, he totally recovered after splenectomy. Eight months later, he relapsed, showing also hypofibrinogenemia, hypertriglyceridemia, hemophagocytic signs in bone marrow, cholestatic jaundice, high LDH and high PT-INR...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28283263/impact-of-oocyte-donation-on%C3%A2-perinatal-outcome-in-twin-pregnancies
#9
Lucie Guilbaud, Pietro Santulli, Eva Studer, Vanessa Gayet, François Goffinet, Camille Le Ray
OBJECTIVE: To compare perinatal outcomes of twin pregnancies after oocyte donation (OD), in vitro fertilization (IVF) with autologous oocyte (AO), and non-IVF conception. DESIGN: Five-year retrospective cohort study. SETTING: Tertiary university medical center. PATIENT(S): All patients with twin pregnancies who gave birth after 24 weeks of gestation. The outcomes of 102 OD twin pregnancies were compared with those of 201 AO and 369 non-IVF twin pregnancies...
March 7, 2017: Fertility and Sterility
https://www.readbyqxmd.com/read/28279123/a-pioneering-study-oral-clarithromycin-treatment-for-feeding-intolerance-in-very-low-birth-weight-preterm-infants
#10
Selim Sancak, Tugba Gursoy, Abdulhamit Tuten, Didem Arman, Guner Karatekin, Fahri Ovali
PURPOSE: To examine the prokinetic effect of clarithromycin in very low birth weight (VLBW) preterm infants. MATERIALS AND METHODS: VLBW preterm infants who have not achieved half of the full enteral feeding in the second week of life were enrolled in the study. The infants enrolled in the study were randomized. 20 infants received oral clarithromycin (7.5 mg/kg, twice a day) and 20 control infants did not receive any treatment. RESULTS: Full enteral feeding was attained earlier in the clarithromycin group than in the control group [7 (6-9) vs 9 (9-11) days, respectively; p < 0...
March 9, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28277061/%C3%AE-granule-biogenesis-from-disease-to-discovery
#11
Chang Hua Chen, Richard W Lo, Denisa Urban, Fred G Pluthero, Walter H A Kahr
Platelets are critical to hemostasis and thrombosis. Upon detecting injury, platelets show a range of responses including the release of protein cargo from α-granules. This cargo is synthesized by platelet precursor megakaryocytes or endocytosed by megakaryocytes and/or platelets. Insights into α-granule biogenesis have come from studies of hereditary conditions where these granules are immature, deficient or absent. Studies of Arthrogryposis, Renal dysfunction, and Cholestasis (ARC) syndrome identified the first proteins essential to α-granule biogenesis: VPS33B and VPS16B...
March 2017: Platelets
https://www.readbyqxmd.com/read/28276873/the-hidden-niemann-pick-type-c-patient-clinical-niches-for-a-rare-inherited-metabolic-disease
#12
Christian J Hendriksz, Mathieu Anheim, Peter Bauer, Olivier Bonnot, Anupam Chakrapani, Jean-Christophe Corvol, Tom J de Koning, Anna Degtyareva, Carlo Dionisi-Vici, Sarah Doss, Thomas Duning, Paola Giunti, Rosa Iodice, Tracy Johnston, Dierdre Kelly, Hans-Hermann Klünemann, Stefan Lorenzl, Alessandro Padovani, Miguel Pocovi, Matthis Synofzik, Alta Terblanche, Florian Then Bergh, Meral Topçu, Christine Tranchant, Mark Walterfang, Christian Velten, Stefan A Kolb
BACKGROUND: Niemann-Pick disease type C (NP-C) is a rare, inherited neurodegenerative disease of impaired intracellular lipid trafficking. Clinical symptoms are highly heterogeneous, including neurological, visceral, or psychiatric manifestations. The incidence of NP-C is under-estimated due to under-recognition or misdiagnosis across a wide range of medical fields. New screening and diagnostic methods provide an opportunity to improve detection of unrecognized cases in clinical sub-populations associated with a higher risk of NP-C...
March 2, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28275692/endoplasmic-reticulum-stress-regulates-hepatic-bile-acid-metabolism-in-mice
#13
Anne S Henkel, Brian LeCuyer, Shantel Olivares, Richard M Green
BACKGROUND & AIMS: Cholestasis promotes endoplasmic reticulum (ER) stress in the liver, however, the effect of ER stress on hepatic bile acid metabolism is unknown. We aim to determine the effect of ER stress on hepatic bile acid synthesis and transport in mice. METHODS: ER stress was induced pharmacologically in C57BL/6J mice and human hepatoma (HepG2) cells. The hepatic expression of genes controlling bile acid synthesis and transport was determined. To measure the activity of the primary bile acid synthetic pathway, the concentration of 7α-hydroxy-4-cholesten-3-1 was measured in plasma...
March 2017: Cellular and Molecular Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28275221/magnetic-resonance-mr-cholangiopancreatography-demonstration-of-the-cystic-duct-entering-the-right-hepatic-duct
#14
Tommaso D'Angelo, Sergio Racchiusa, Silvio Mazziotti, Giuseppe Cicero
BACKGROUND MR cholangiopancreatography is widely performed before laparoscopic cholecystectomy to rule out choledocholithiasis and to avoid iatrogenic injuries that may be related to the high frequency of anatomical variations of the biliary tree. Although most of these variants have already been demonstrated surgically and by endoscopic retrograde cholangiopancreatography and CT cholangiography, there are no references in which MR cholangiopancreatography has shown a cystic duct draining into the right hepatic biliary duct...
March 9, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28273180/synthesis-in-vitro-and-in-vivo-evaluation-of-3%C3%AE-18f-fluorocholic-acid-for-the-detection-of-drug-induced-cholestasis-in-mice
#15
Stef De Lombaerde, Sara Neyt, Ken Kersemans, Jeroen Verhoeven, Lindsey Devisscher, Hans Van Vlierberghe, Christian Vanhove, Filip De Vos
INTRODUCTION: Drug-induced cholestasis is a liver disorder that might be caused by interference of drugs with the hepatobiliary bile acid transporters. It is important to identify this interference early on in drug development. In this work, Positron Emission Tomography (PET)-imaging with a 18F labeled bile acid analogue was introduced to detect disturbed hepatobiliary transport of bile acids. METHODS: 3β-[18F]fluorocholic acid ([18F]FCA) was prepared by nucleophilic substitution of a mesylated precursor with [18F]fluoride, followed by deprotection with sodium hydroxide...
2017: PloS One
https://www.readbyqxmd.com/read/28272341/liver-effects-of-clinical-drugs-differentiated-in-human-liver-slices
#16
Alison E M Vickers, Anatoly V Ulyanov, Robyn L Fisher
Drugs with clinical adverse effects are compared in an ex vivo 3-dimensional multi-cellular human liver slice model. Functional markers of oxidative stress and mitochondrial function, glutathione GSH and ATP levels, were affected by acetaminophen (APAP, 1 mM), diclofenac (DCF, 1 mM) and etomoxir (ETM, 100 μM). Drugs targeting mitochondria more than GSH were dantrolene (DTL, 10 μM) and cyclosporin A (CSA, 10 μM), while GSH was affected more than ATP by methimazole (MMI, 500 μM), terbinafine (TBF, 100 μM), and carbamazepine (CBZ 100 μM)...
March 7, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28271527/the-role-of-lncrna-h19-in-gender-disparity-of-cholestatic-liver-injury-in-mdr2-mice
#17
Xiaojiaoyang Li, Runping Liu, Jing Yang, Lixin Sun, Luyong Zhang, Zhenzhou Jiang, Puneet Puri, Emily C Gurley, Guanhua Lai, Yuping Tang, Zhiming Huang, William M Pandak, Phillip B Hylemon, Huiping Zhou
The multi-drug resistance 2 knockout (Mdr2(-/-) ) mouse is a well-established model of cholestatic cholangiopathies. Female Mdr2(-/-) mice develop more severe hepatobiliary damage than male Mdr2(-/-) mice, which is correlated with a higher proportion of taurocholate (TCA) in bile. Although estrogen has been identified as an important player in intrahepatic cholestasis, the underlying molecular mechanisms of gender-based disparity of cholestatic injury remain unclear. The long non-coding RNA H19 is an imprinted, maternally expressed and estrogen-targeted gene, which is significantly induced in human fibrotic/cirrhotic liver and bile duct ligated mouse liver...
March 8, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28270873/predictors-of-fetal-and-maternal-outcome-in-the-crucible-of-hepatic-dysfunction-during-pregnancy
#18
Indrajit Suresh, Vijaykumar Tr, Nandeesh Hp
BACKGROUND: Hepatic dysfunction during pregnancy places both the mother and the fetus at risk. Investigations which are efficient, cost effective and easily available for prognostication are required to tackle this global problem. We studied the etiologies and evaluated investigations for predictive efficiency. METHODS: One hundred ninety-seven pregnant women with hepatic dysfunction during pregnancy were identified. All patients were followed up till 8 weeks after termination of pregnancy or death...
February 2017: Gastroenterology Research
https://www.readbyqxmd.com/read/28267673/paeoniflorin-attenuates-anit-induced-cholestasis-by-inhibiting-apoptosis-in-vivo-via-mitochondria-dependent-pathway
#19
Hou-Qin Zhou, Wei Liu, Jian Wang, Yin-Qiu Huang, Peng-Yan Li, Yun Zhu, Jia-Bo Wang, Xiao Ma, Rui-Sheng Li, Shi-Zhang Wei, Kun Li, Hao-Tian Li, Jian-Yu Li, Xiao-He Xiao, Yan-Ling Zhao
Apoptosis induced by the bile acids in the liver is considered to play a pivotal role in the pathogenesis of cholestatic disease. Increasing evidence has demonstrated that Paeoniflorin (PF) exerts therapeutic effect on severe cholestatic liver diseases. However, whether PF could protect against alpha-naphthylisothiocyanate (ANIT)-induced cholestasis by inhibiting apoptosis remains unclear. In this study, we mainly investigated the effect and anti-apoptosis mechanism of PF on cholestasis. Experimental results indicated that PF pretreatment could attenuate liver damage and cholestasis by ANIT in rats, lift the biliary excretion in addition to decrease serum indices (ALT, AST, DBIL, TBIL, TBA, ALP and ϒ-GT) and conspicuous neutrophil infiltration and cell apoptosis in liver evidenced by TUNEL staining...
March 3, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28262241/hemostatic-issues-in-pregnancy-induced-liver-disease
#20
Ton Lisman, William Bernal
Liver diseases may be accompanied by profound changes in the hemostatic system including thrombocytopenia, decreased plasma levels of pro- and anticoagulants, and alterations in plasma levels of fibrinolysis. The net effect of the hemostatic changes in chronic and acute liver diseases is a hemostatic system that is in relative balance due to the simultaneous decline in pro- and antihemostatic drivers. A unique category of liver diseases are those induced by pregnancy. In acute fatty liver of pregnancy, profound hemostatic changes occur, which may be caused by a combination of liver failure and disseminated intravascular coagulation...
March 2017: Thrombosis Research
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