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NONLINEAR ELECTROSTRICTIVE PROPERTIES OF PbZrO_(3):Sn SINGLE CRYSTALS WITH ANTIFERROELECTRIC PHASE TRANSITIONS

Journal of Advanced Dielectrics 2011年第2期1卷 223-227页

作者： irena jankowska-sumara KRYSTIAN ROLEDER A.MAJCHROWSKI J.ŻMIJA Institute of Physics Pedagogical University ul.Podchorazych 230-084 KrakowPoland Institute of Physics University of Silesia ul.Uniwersytecka 440-007 KatowicePoland Institute of Applied Physics Military University of Technology ul.Kaliskiego 200-908 WarszawPoland

The electrostrictive properties above Te max represented by the field-related and polarization-related M and Q coefficients have been measured for PbZr_(1-x)Sn_(x)O_(3)single crystals with ani-ferroelectric phase *** investigations presented in this paper have proved that the M_(11)(T)and Q_(11)(T)runs bring direct information of the pre-transitional effects connected with the co-existence of local polar objects with nonpolar *** to this co-existence,nonlinear Q_(11)(T)dependence in the neighborhood of T_(C)has been *** even in a wide tem-perature above T_(C),this co-existence is a natural feature of the material with ABO_(3)perovskite structure that undergoes structural transition to po lar phase.

关键词： Antiferroelectric phase transitions electrostriction polar regions

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Progressive familial intrahepatic cholestasis—farnesoid X receptor deficiency due to NR1H4 mutation:A case report

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World Journal of Clinical Cases 2021年第15期9卷 3631-3636页

作者： Piotr Czubkowski Richard J Thompson irena jankowska A S Knisely Milton Finegold Pamela Parsons Joanna Cielecka-Kuszyk Sandra Strautnieks Joanna Pawłowska Laura N Bull Department of Gastroenterology HepatologyNutritional Disorders and PediatricsThe Children’s Memorial Health InstituteWarsaw 04-730Poland Institute of Liver Studies King's College London HospitalLondon SE59RSUnited Kingdom Institut für Pathologie Medizinische Universität GrazGraz 8010Austria Department of Pathology and Immunology Baylor College of MedicineHoustonTX 77030United States Texas Children’s Hospital HoustonTX 77030United States Department of Pathology The Children’s Memorial Health InstituteWarsaw 04-730Poland Department of Medicine and Institute for Human Genetics UCSF Liver Center LaboratoryUniversity of California San FranciscoSan FranciscoCA 94143United States

BACKGROUND Functioning farnesoid X receptor(FXR;encoded by NR1H4)is key to normal bile acid *** mutations in NR1H4 are reported in a few children with intrahepatic *** describe a boy with progressive familial intrahepatic cholestasis and homozygous mutation in *** SUMMARY A boy had severe neonatal cholestasis with moderate hypercholanemia and persistently elevated *** medical treatment,coagulopathy was uncontrollable,prompting liver transplantation at age 8 mo with incidental *** patient experienced catch-up growth with good liver function and did not develop allograft ***,1 year after transplant,he died from an acute infection,considered secondary to immunosuppression and asplenia.A homozygous protein-truncating mutation,c.547C>T,p.(Arg183Ter),was subsequently identified in NR1H4,and both parents were shown to be heterozygous *** of FXR and of bile salt export pump expression was confirmed by immunostaining of explanted *** Severe cholestasis with persistently high alpha-fetoprotein and modest elevation of serum bile acid levels may suggest FXR *** patients with FXR deficiency may not develop allograft steatosis and may respond well to liver transplantation.

关键词： Neonatal cholestasis Progressive familial intrahepatic cholestasis Bile salt export pump Liver transplantation Alpha-fetoprotein Case report

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