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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 | 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 |
| 5 | 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 |
| 6 | Microinvasive carcinoma of the vagina:a distinct clinical entity?显示文摘 | Peters Ⅲ WA Kumar NB Morley GW | 1985 | Am J Obstet Gynecol1985，153，5： | 1 |
| 7 | 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 |
| 8 | Microinvasive carcinoma of the vagina a distinct clinical entity 显示文摘 | Peters WA 3rd Kumar NB Morley GW | 1985 | Am J Obstet Gyneco1985，153，5： | 1 |
| 9 | Primary sarcoma of the adult vagina:a clinicopathologic study显示文摘 | Peters WA Kumar NB Andersen WA | 1985 | Obstet Gynecal1985，65，： | 1 |
| 10 | Webvise:Browser and Proxy Support for Open Hypermedia Structuring Mechanisms on the World Wide Web显示文摘 | Kaj Grφnb(ae)k Lennert Sloth Peterφrb(ae)k | 1999 | Computer Networks1999，，31： | 1 |
| 11 | Electrophysiological evidence that drug cues have greater salience than other affective stimuli in opiate addiction显示文摘 | Lubman DI Allen NB Peters LA | 2008 | J Psychopharmacol2008，22，8： | 1 |
| 12 | Electrophysiological evidence of the motivational salience of drug cues in opiate addiction 显示文摘 | Lubman DI Allen NB Peters LA | 2007 | Psychol Med2007，37，8： | 1 |
| 13 | Theory-assisted determination of absolute stereochemistry for complex natural products via computation of molar rotation angles 显示文摘 | Rama KK Peter W David NB | 1998 | J Am Chem Soc1998，120，9： | 1 |
| 14 | Electrophysiological evidence of the motivational salience of drug cues in opiate addiction 显示文摘 | Lubman DI Allen NB Peters LA | 2007 | Psychol Med2007，37，： | 1 |
| 15 | Carcinoma of the vagina Factors influencing treatment outcome显示文摘 | Peters WA III Kumar NB Morley GW | | 0，，04： | 1 |
| 16 | Harmonic imaging adds to ultrasound capabilities显示文摘 | Peter NB | | 0，，： | 1 |
| 17 | Postprandial hyperglycaemia and vascular damagethe benefits of acarbose显示文摘 | Suad E | 2006 | Diabetes Vasc Dis Res2006，3，2： | 1 |
| 18 | Stellio sacra (Smith 1935) a distinct Species of Asiatic rock agamid fromTibet 显示文摘 | Ananjeva NB Peters G Macey Papenfuss T | 1990 | Asiatic Herpetological Research1990，，3： | 1 |
| 19 | Focal hepatic masses:enhancement patterns with SHU 508A and pulse-inversion US显示文摘 | Marcus JDM Peter NB Korosh K | 2002 | Radiology2002，222，1： | 1 |
| 20 | Electrophysiological evidence of the motivational salience of drug cues in opiate addiction显示文摘 | Allen NB Peters LA | 2007 | Psychol Med2007，37，： | 1 |
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