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| 1 | New tight junction protein 2 variant causing progressive familial intrahepatic cholestasis type 4 in adults: A case report显示文摘BACKGROUND Progressive familial intrahepatic cholestasis(PFIC)encompasses a group of autosomal recessive disorders with high morbidity and mortality.Variants in the gene encoding tight junction protein-2(TJP2)have been linked to PFIC type 4(PFIC4),which predominantly presents in childhood.However,there are only limited data from adults with TJP2-related PFIC4.We report a family with an autosomal recessive disorder with a novel variant in the TJP2 gene in adults with very variable expression of PFIC4.CASE SUMMARY The index patient presented at 19 years old with liver cirrhosis and variceal bleeding and was treated with endoscopic banding and beta-blockers.In 2018,he developed primary liver cancer that was treated with radiofrequency ablation followed by liver transplantation in 2019.Genetic testing revealed a novel homozygous TJP2 variant causing PFIC4(TJP2([NM_004817.3]:c.[3334C>T];[3334C>T])).The consanguineous family consists of the father and mother(both heterozygous)and their 12 children,of which five carry the variant in a homozygous state;however,these five siblings have highly variable expression of PFIC4.Two homozygous brothers had cirrhosis and portal hypertension at diagnosis at the ages of 19 and 36.Two other homozygous brothers,age 23 and 19,and the homozygous sister,age 21,have elevated liver enzymes but presently no cirrhosis,which may suggest an age-dependent penetrance.In addition,five sisters had severe and mild intrahepatic cholestasis of pregnancy and carry the TJP2 variant in a homozygous and heterozygous state,respectively.CONCLUSION This novel TJP2 variant is associated with PFIC4 causing severe liver disease with cirrhosis and primary liver cancer in adolescents/adults. | Chun-Shan Wei Naja Becher Jenny Blechingberg Friis Peter Ott Ida Vogel Henning Grønbæk | 2020 | World Journal of Gastroenterology2020，26，5： | 6 |
| 2 | Treatment of Budd-Chiari syndrome with a focus on transjugular intrahepatic portosystemic shunt显示文摘AIM:To evaluate long-term complications and survival in patients with Budd-Chiari syndrome (BCS) referred to a Danish transjugular intrahepatic portosystemic shunt (TIPS) centre.METHODS:Twenty-one consecutive patients from 1997-2008 were retrospectively included [15 women and 6 men,median age 40 years (range 17-66 years)].Eighteen Danish patients came from the 1.8 million catchment population of Aarhus University Hospital and three patients were referred from Scandinavian hospitals.Management consisted of tests for underlying haematological,endocrinological,or hypercoagulative disorders parallel to initiation of specific treatment of BCS.RESULTS:BCS was mainly caused by thrombophilic (33%) or myeloproliferative (19%) disorders.Fortythree percents had symptoms for less than one week with ascites as the most prevalent finding.Fourteen (67%) were treated with TIPS and 7 (33%) were manageable with treatment of the underlying condition and diuretics.The median follow-up time for the TIPS-treated patients was 50 mo (range 15-117 mo),and none required subsequent liver transplantation.Ascites control was achieved in all TIPS patients with a marked reduction in the dose of diuretics.A total of 14 TIPS revisions were needed,mostly of uncovered stents.Two died during follow-up:One non-TIPS patient worsened after 6 mo and died in relation to transplantation,and one TIPS patient died 4 years after the TIPS-procedure,unrelated to BCS.CONCLUSION:In our BCS cohort TIPS-treated patients have near-complete survival,reduced need for diuretics and compared to historical data a reduced need for liver transplantation. | Anders Bay Neumann Stine Degn Andersen Dennis Tφnner Nielsen Peter Holland-Fischer Hendrik Vilstrup Henning Grφnbk | 2013 | World Journal of Hepatology2013，5，1： | 5 |
| 3 | Autoimmune hepatitis in Denmark: Incidence, prevalence, prognosis, and causes of death. A nationwide registry-based cohort study显示文摘 | Lisbet Gr?nb?k Hendrik Vilstrup Peter Jepsen | 2013 | Journal of Hepatology2013，，： | 3 |
| 4 | Empirical estimates of regional carbon budgets imply reduced global soil heterotrophic respiration显示文摘Resolving regional carbon budgets is critical for informing land-based mitigation policy.For nine regions covering nearly the whole globe,we collected inventory estimates of carbon-stock changes complemented by satellite estimates of biomass changes where inventory data are missing.The net land–atmospheric carbon exchange(NEE)was calculated by taking the sum of the carbon-stock change and lateral carbon fluxes from crop and wood trade,and riverine-carbon export to the ocean.Summing up NEE from all regions,we obtained a global‘bottom-up'NEE for net land anthropogenic CO_(2)uptake of–2.2±0.6 Pg C yr^(-1)consistent with the independent top-down NEE from the global atmospheric carbon budget during 2000–2009.This estimate is so far the most comprehensive global bottom-up carbon budget accounting,which set up an important milestone for global carbon-cycle studies.By decomposing NEE into component fluxes,we found that global soil heterotrophic respiration amounts to a source of CO_(2)of 39 Pg C yr^(-1)with an interquartile of 33–46 Pg C yr^(-1)—a much smaller portion of net primary productivity than previously reported. | Philippe Ciais Yitong Yao Thomas Gasser Alessandro Baccini Yilong Wang Ronny Lauerwald Shushi Peng Ana Bastos Wei Li Peter A.Raymond Josep G.Canadell Glen P.Peters Rob J.Andres Jinfeng Chang Chao Yue A.Johannes Dolman Vanessa Haverd Jens Hartmann Goulven Laruelle Alexandra G.Konings Anthony W.King Yi Liu Sebastiaan Luyssaert Fabienne Maignan Prabir K.Patra Anna Peregon Pierre Regnier Julia Pongratz Benjamin Poulter Anatoly Shvidenko Riccardo Valentini Rong Wang Grégoire Broquet Yi Yin Jakob Zscheischler Bertrand Guenet Daniel SGoll Ashley-P.Ballantyne Hui Yang Chunjing Qiu Dan Zhu | 2021 | National Science Review2021，8，2： | 3 |
| 5 | Body composition changes after transjugular intrahepatic portosystemic shunt in patients with cirrhosis显示文摘AIM:To investigate the effect of transjugular intra-hepatic porto-systemic shunt (TIPS) on malnutrition in portal hypertensive cirrhotic patients.METHODS: Twenty-one patients with liver cirrhosis and clinical indications for TIPS insertion were investigated before and 1, 4, 12, 52 wk after TIPS. For each patient we assayed body composition parameters [dry lean mass, fat mass, total body water (TBW)], routine liver and kidney function tests, and free fatty acids (FFA). Glucose and insulin were measured for the calculation of the homeostasis model assessment insulin resistance (HOMA-IR); liver function was measured by the galactose elimination capacity (GEC); the severity of liver disease was graded by model for end-stage liver disease (MELD).RESULTS: Porto-systemic gradient decreased after TIPS (6.0±2.1 mmHg vs 15.8±4.8 mmHg, P<0.001). Patients were divided in two groups according to initial body mass index. After TIPS, normal weight patients had an increase in dry lean mass (from 10.9±5.9 kg to 12.7±5.6 kg, P=0.031) and TBW (from 34.5±7.6 L to 40.2±10.8 L,P=0.007), as well as insulin (from 88.9±49.2 pmol/L to 164.7±107.0 pmol/L,P=0.009) and HOMA-IR (from 3.36%±2.18% to 6.18%±4.82%,P=0.023). In overweight patients only FFA increased significantly (from 0.59±0.24 mmol/L to 0.93±0.34 mmol/L, P=0.023).CONCLUSION: TIPS procedure is effective in lowering portal pressure in patients with portal hypertension and improves body composition without significant changes in metabolic parameters. | Jonathan Montomoli Peter Holland-Fischer Giampaolo Bianchi Henning GrФnbk Hendrik Vilstrup Giulio Marchesini Marco Zoli | 2010 | World Journal of Gastroenterology2010，16，3： | 3 |
| 6 | Long-Term Follow-Up of Biopsy-Proven Viral Myocarditis显示文摘 | Stefan Grün Julia Schumm Simon Greulich Anja Wagner Steffen Schneider Oliver Bruder Eva-Maria Kispert Stephan Hill Peter Ong Karin Klingel Reinhardt Kandolf Udo Sechtem Heiko Mahrholdt | 2012 | Journal of the American College of Cardiology2012，，18： | 2 |
| 7 | Gap filling strategies for long term energy flux data sets显示文摘 | Eva Falge Dennis Baldocchi Richard Olson Peter Anthoni Marc Aubinet Christian Bernhofer George Burba Reinhart Ceulemans Robert Clement Han Dolman André Granier Patrick Gross Thomas Grünwald David Hollinger Niels-Otto Jensen Gabriel Katul Petri Keronen And | 2001 | Agricultural and Forest Meteorology2001，，1： | 2 |
| 8 | Shot-noise limited, supercontinuum-based optical coherence tomography显示文摘We present the first demonstration of shot-noise limited supercontinuum-based spectral domain optical coherence tomography(SD-OCT)with an axial resolution of 5.9μm at a center wavelength of 1370 nm.Current supercontinuumbased SD-OCT systems cannot be operated in the shot-noise limited detection regime because of severe pulse-topulse relative intensity noise of the supercontinuum source.To overcome this disadvantage,we have developed a low-noise supercontinuum source based on an all-normal dispersion(ANDi)fiber,pumped by a femtosecond laser.The noise performance of our 90 MHz ANDi fiber-based supercontinuum source is compared to that of two commercial sources operating at 80 and 320 MHz repetition rate.We show that the low-noise of the ANDi fiber-based supercontinuum source improves the OCT images significantly in terms of both higher contrast,better sensitivity,and improved penetration.From SD-OCT imaging of skin,retina,and multilayer stacks we conclude that supercontinuumbased SD-OCT can enter the domain of shot-noise limited detection. | Shreesha Rao D.S. Mikkel Jensen Lars Grüner-Nielsen Jesper Toft Olsen Peter Heiduschka Björn Kemper Jürgen Schnekenburger Martin Glud Mette Mogensen Niels Møller Israelsen Ole Bang | 2021 | Light(Science & Applications)2021，10，7： | 2 |
| 9 | CMR Imaging Predicts Death and Other Adverse Events in Suspected Cardiac Sarcoidosis显示文摘 | Simon Greulich Claudia Christina Deluigi Steffen Gloekler Andreas Wahl Christine Zürn Ulrich Kramer Detlev Nothnagel Helmut Bültel Julia Schumm Stefan Grün Peter Ong Anja Wagner Steffen Schneider Kai Nassenstein Meinrad Gawaz Udo Sechtem Oliver Bruder Hei | 2013 | JACC: Cardiovascular Imaging2013，，： | 2 |
| 10 | Examples of high-frequency EPR studies in bioinorganic chemistry显示文摘 | K. Kristoffer Andersson Peter P. Schmidt Bettina Katterle Kari R. Strand Amy E. Palmer Sang-Kyu Lee Edward I. Solomon Astrid Gr?slund Anne-Laure Barra | 2003 | JBIC Journal of Biological Inorganic Chemistry2003，，3： | 1 |
| 11 | Information technology:Efficient restructuring and the productivity puzzle显示文摘 | Hans Peter Grüner | | 0，，03： | 1 |
| 12 | Bank cooperation and banking policy in a monetary union: A political-economy perspective on EMU显示文摘 | Hans Peter Grüner Carsten Hefeker | 1996 | Open Economies Review1996，，3： | 1 |
| 13 | Effects and safety of natriuretic peptides as treatment of cirrhotic ascites:A systematic review and meta-analysis显示文摘BACKGROUND Natriuretic peptides are involved in the cascade of pathophysiological events occurring in liver cirrhosis,counterbalancing vasoconstriction and anti-natriuretic factors.The effects of natriuretic peptides as treatment of cirrhotic ascites have been investigated only in small studies,and definitive results are lacking.AIM To examine the effects and safety of natriuretic peptides in cirrhosis patients with ascites.METHODS We searched MEDLINE,Web of Science,Scopus,Cochrane Library and Embase for all available studies applying intravenous administration of any natriuretic peptide to patients suffering from cirrhotic ascites.Inclusion was not limited by treatment duration or dose,or by follow-up duration.Both randomised controlled trials and non-randomised studies were eligible for inclusion.The primary outcome was change in renal sodium excretion.Secondary outcomes included safety measures and changes in renal water excretion,plasma aldosterone concentration,and plasma renin activity.RESULTS Twenty-two studies were included.Atrial natriuretic peptide(ANP)was the only intensively studied treatment.Sodium excretion increased in response to continuous ANP infusion and was more pronounced when infusion rates of>30 ng/kg/min were administered compared with≤30 ng/kg/min(P<0.01).Moreover,natriuresis was significantly higher in study subgroups with mild/moderate ascites compared with moderate/severe and refractory ascites(P<0.01).ANP infusions increased renal water excretion,although without reaching a statistically significant dose-response gradient.Plasma aldosterone concentration and plasma renin activity were significantly lower at baseline in study subgroups achieving a negative sodium balance in response to an ANP administration compared with treatment non-responders(P<0.01).Blood pressure decreases occurred less frequently when ANP doses≤30 ng/kg/min were applied.The quality of evidence for a natriuretic response to ANP was low,mainly due to small sample sizes and considerable between-study heterogeneity.Data were sparse for the other natriuretic peptides;B-type natriuretic peptide and urodilatin.CONCLUSION Intravenous ANP infusions increase sodium excretion in patients with cirrhotic ascites.Continuous infusion rates>30 ng/kg/min are the most effective.However,safety increases with infusion rates≤30 ng/kg/min. | Rasmus Hvidbjerg Gantzel Mikkel Breinholt Kjær Peter Jepsen Niels Kristian Aagaard Hugh Watson Lise Lotte Gluud Henning Grønbæk | 2022 | World Journal of Hepatology2022，14，4： | 1 |
| 14 | Detection of Activated Parietal Epithelial Cells on the Glomerular Tuft Distinguishes Early Focal Segmental Glomerulosclerosis from Minimal Change Disease显示文摘 | Bart Smeets Fabien Stucker Jack Wetzels Isabelle Brocheriou Pierre Ronco Hermann-Josef Gr?ne Vivette D’Agati Agnes B. Fogo Toin H. van Kuppevelt Hans-Peter Fischer Peter Boor Jürgen Floege Tammo Ostendorf Marcus J. Moeller | 2014 | The American Journal of Pathology2014，，： | 1 |
| 15 | Quantitative histological assessment of hepatic ischemia-reperfusion injuries following ischemic pre- and post-conditioning in the rat liver显示文摘 | Anders R. Knudsen Anne-Sofie Kannerup Henning Gr?nb?k Stephen H. Dutoit Jens R. Nyengaard Peter Funch-Jensen Frank V. Mortensen | 2012 | Journal of Surgical Research2012，，： | 1 |
| 16 | Changes in human fetal oxygenation during maternal hyperoxia as estimated by BOLD MRI显示文摘 | Anne S?rensen David Peters Carsten Simonsen Michael Pedersen Brian Stausb?l‐Gr?n Ole Bjarne Christiansen G?ran Lingman Niels Uldbjerg | 2012 | Prenat Diagn2012，，2： | 1 |
| 17 | Prognostic Value of Renal Vein and Inferior Vena Cava Involvement in Renal Cell Carcinoma显示文摘 | Bernd Wagner Jean-Jacques Patard Arnaud Méjean Karim Bensalah Grégory Verhoest Richard Zigeuner Vincenzo Ficarra Jacques Tostain Peter Mulders Denis Chautard Jean-Luc Descotes Alexandre de la Taille Laurent Salomon Tommaso Prayer-Galetti Luca Cindolo Anto | 2008 | European Urology2008，，2： | 1 |
| 18 | Pilot study: peripheral biomarkers for diagnosing sporadic Parkinson’s disease显示文摘 | Edna Grünblatt Sonja Zehetmayer Christian P. Jacob Thomas Müller Wolfgang H. Jost Peter Riederer | 2010 | Journal of Neural Transmission2010，，12： | 1 |
| 19 | Preputial Plasty: A good alternative to circumcision显示文摘 | Peter M Cukow GR | 1994 | J Pediatr Surg1994，29，： | 1 |
| 20 | Catching up faster by switching sooner:a predictive ap-proach to adaptive estimation with an application to theAIC–BIC dilemma显示文摘 | Tim van Erven Peter Grünwald and Steven de Rooij | 2012 | Journal of the Royal Statistical So-ciety:Series B(Statistical Methodology)2012，74，36： | 1 |
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