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| 1 | Hepatic phenotypes of HNF1B gene mutations:A case of neonatal cholestasis requiring portoenterostomy and literature review显示文摘Hepatocyte nuclear factor 1-β(HNF1B)defects cause renal cysts and diabetes syndrome(RCAD),or HNF1B-maturity-onset diabetes of the young.However,the hepatic phenotype of HNF1B variants is not well studied.We present a female neonate born small for her gestational age[birth weight 2360 g;-2.02standard deviations(SD)and birth length 45 cm;-2.40 SD at the 38th gestational week].She developed neonatal cholestasis due to biliary atresia and required surgical intervention(portoenterostomy)when 32-d old.Following the operation,icterus resolved,but laboratory signs of liver dysfunction persisted.She had hyperechogenic kidneys prenatally with bilateral renal cysts and pancreatic hypoplasia postnatally that led to the diagnosis of an HNF1B deletion.This represents the most severe hepatic phenotype of an HNF1B variant recognized thus far.A review of 12 published cases with hepatic phenotypes of HNF1B defects allowed us to distinguish three severity levels,ranging from neonatal cholestasis through adult-onset cholestasis to noncholestatic liver impairment,all of these are associated with congenital renal cysts and mostly with diabetes later in life.We conclude that to detect HNF1B variants,neonates with cholestasis should be checked for the presence of renal cysts,with special focus on those who are born small for their gestational age.Additionally,patients with diabetes and renal cysts at any age who develop cholestasis and/or exocrine pancreatic insufficiency should be tested for HNF1B variants as the true etiological factor of all disease components.Further observations are needed to confirm the potential reversibility of cholestasis in infancy in HNF1B mutation/deletion carriers. | Radana Kotalova Petra Dusatkova Ondrej Cinek Lenka Dusatkova Tomas Dedic Tomas Seeman Jan Lebl Stepanka Pruhova | 2015 | World Journal of Gastroenterology2015，21，8： | 7 |
| 2 | ON CONJUGATE SYMPLECTICITY OF MULTI-STEP METHODS显示文摘In this paper, we solve a problem on the existence of conjugate symplecticity of linear multi-step methods (LMSM), the negative result is obtained, | Yi-fa Tang (LSEC, Institute of Computational Mathematics and Scientific/Engineering Computing, Academy of Mathematics and System Sciences, Chinese Academy of Sciences, Beijing 100080, China) Dedicated to Feng Kang on his 80th birthday | 2000 | Journal of Computational Mathematics2000，18，4： | 2 |
| 3 | Complementation analy- sis of triple gene block of Potato virus S (PVS) revealed its capability to support systemic infection and aphid transmissibility of recombinant Potato virus X 显示文摘 | Matousek J Schubert J Dedic P | 2009 | Virus Research2009，146，12： | 1 |
| 4 | Application of a diode laser in the reduction of targeted periodontal pathogens显示文摘 | Gojkov-Vukelic M Hadzic S Dedic A | 2013 | Acta InformMed2013，21，4： | 1 |
| 5 | Art v 1,the major allergen of mugwort pollen,is a modular glycoprotein with a defensin-like and a hydroxyproline-rich domain 显示文摘 | HIMLY M JAHN-SCHMID B DEDIC A | 2003 | FASEB J2003，17，1： | 1 |
| 6 | Application of adiode laser in the reduction of targeted periodontal pathogens显示文摘 | Gojkov-Vukelic M Hadzic S Dedic A | 2013 | Acta Inform Med2013，21，4： | 1 |
| 7 | A GNERAL BIJECTIVE ALGORITHM FOR INCREASING TREES显示文摘The main concern of this paper is with a bijective approach to various classes ofincreasing trees. We discover an increasing tree counterpart of the decomposition algorithmfor Schroder trees. This bijection has probably reached its full generality for decomposingincreasing trees. As a special case of our algorithm, we provide a solution to a problemconcerning the enumeration of plane trees by the net number of inversions. | (Dedicated to Professor Wu Wentsun on the occasion of his 80-th birthday)William Y. C. CHEN(Center for Combinatorics, Nankai University, Tianjin 300071, Chinaand T-7, Mail Stop B 284, Los Alamos National Laboratory,Los Alamos, New Mexico 87545, USA) | 1999 | Systems Science and Mathematical Sciences1999，12，3： | 1 |
| 8 | Nanofabrication in Polymer Solutions显示文摘This review covers recent advances in fabrication of nanomaterials in polymer solutions with emphasis on using the self assembled amphiphilic block copolymers in solution to fabricate organic/inorganic composites with nanoscale modifications. The phase behavior of block copolymers in water and the use of templates to form ordered nanostructures are reviewed in detail. Modern physical techniques for nanoscale characterization are also introduced. The authors suggested that this approach should provide new routes to create materials with interesting morphologies for many different applications. | Tianbo LIU, Li Zhi LIU, Benjamin CHU *(Department of Chemistry,State University of New York at Stony Brook, Stony Brook,NY 11790 3400,USA) Dedicated to the Occasion of the 80th Birthday of Prof.HUANG Bao Tong and Prof.FENG Zhi Liu | 2001 | 应用化学2001，18，5： | 1 |
| 9 | A broad variability of potato virus S (PVS) revealed by analysis of virus sequences amplified by reverse transcriptase-polymerase chain reaction显示文摘 | Matousek J Schubert J Dedic P | 2000 | Plant Pathol2000，22，： | 1 |
| 10 | Fkbp52 heterozygosity alters behavioral, endocrine and neurogenetic parameters under basal and chronic stress conditions in mice 显示文摘 | Hartmann J Wagner KV Dedic N | 2012 | Psychoneuroendocrinology2012，37，12： | 1 |
| 11 | Art V 1, the major allergen of mllgwort pollen, is a modular glycoprotein with a defensin -like and a hydroxyproline -rich domain显示文摘 | Himly M Jahn - Schmid B Dedic A et el | 2003 | FASEBJ2003，17，1： | 1 |
| 12 | Some aspects and comparisons of microwave drying of beech (Fagus moesiaca) and fir wood (Abies alba)显示文摘 | Dedic A Z | 2001 | Holz als Roh- und Werkstoff2001，59，4： | 1 |
| 13 | Soldier's social adaptation during the military service显示文摘 | Dedic G | 2004 | Vojnosanit Pregl2004，61，6： | 1 |
| 14 | Time-resolved 1 and singlet oxygen formation after illumination of the hypericin-low- density lipoprotein complex 显示文摘 | Gbur P Dedic R Miskovsky P | 2009 | Photoehem Photobiol2009，85，3： | 1 |
| 15 | Complementation analy- sis of triple gene block of potato virus S (PVS) revealed its capability to support systemic infection and aphid transmissibility of recombinant potato virus X 显示文摘 | Matousek J Schubert J Dedic P | 2009 | Virus Res2009，146，： | 1 |
| 16 | Parathyroid Hormone (PTH)/PTH-related Peptide Type 1 Receptor (PPR) Signaling in Osteocytes Regulates Anabolic and Catabolic Skeletal Responses to PTH显示文摘 | Vaibhav Saini Dean A. Marengi Kevin J. Barry Keertik S. Fulzele Erica Heiden Xiaolong Liu Christopher Dedic Akira Maeda Sutada Lotinun Roland Baron Paola Divieti Pajevic | 2013 | Journal of Biological Chemistry2013，，28： | 1 |
| 17 | Phase Transitions and Structures of Poly(ethylene-co-chlorotrifluoroethylene)显示文摘Poly(ethylene- co -chlorotrifluoroethylene) with a 1∶1 molar ratio of ethylene and chlorotrifluoroethylene in composition, PECTFE, is a statistically alternating copolymer widely used in cable coating industry. PECTFE has been commonly characterized as a semicrystalline polymer with crystals melting at 238 ℃. Differential scanning calorimetry, thermal mechanical analysis and dynamic mechanical analysis indicated that PECTFE undergoes a reversible solid-solid transition during cooling and heating in the temperature range between 100 ℃ and 200 ℃. Based on detailed structural analysis via wide angle X-ray diffraction and electron diffraction techniques, we proposed that PECTFE possesses a hexagonal phase above 200 ℃( a =0 60 nm, c =0 49 nm) and an orthorhombic phase( a =0 99 nm, b =0 57 nm, c =0 49 nm) at room temperature. | J.M.DEITZEL, Bojie WANG, Ping HUANG, L.Lawrence CHAPOY, Stephen Z.D.CHENG * (The Maurice Morton Institute and Department of Polymer Science, The University of Akron,Akron Ohio 44325-3909,U.S.A.) Dedicated to the Occasion of the 80th Birthday of P | 2001 | 应用化学2001，18，5： | 1 |
| 18 | Parathyroid Hormone (PTH)/PTH-related Peptide Type 1 Receptor (PPR) Signaling in Osteocytes Regulates Anabolic and Catabolic Skeletal Responses to PTH显示文摘 | Vaibhav Saini Dean A. Marengi Kevin J. Barry Keertik S. Fulzele Erica Heiden Xiaolong Liu Christopher Dedic Akira Maeda Sutada Lotinun Roland Baron Paola Divieti Pajevic | 2013 | Journal of Biological Chemistry2013，，28： | 1 |
| 19 | DNA microarray: parallel detection of potato viruses 显示文摘 | Bystricka D Lenz O Mraz I Dedic P Sip M | 2003 | Acta Virol2003，47，1： | 1 |
| 20 | Diagnostic accuracy of 128-slice dual-source CT coronary angiography: a randomized comparison of different acquisition protocols显示文摘 | Lisan A. Neefjes Alexia Rossi Tessa S. S. Genders Koen Nieman Stella L. Papadopoulou Anoeshka S. Dharampal Carl J. Schultz Annick C. Weustink Marcel L. Dijkshoorn Gert-Jan R. Kate Admir Dedic Marcel Straten Filippo Cademartiri M. G. Myriam Hunink Gabri?l | 2013 | European Radiology2013，，3： | 1 |
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