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New tight junction protein 2 variant causing progressive familial intrahepatic cholestasis type 4 in adults: A case report

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World journal of Gastroenterology 2020年第5期26卷 550-561页

作者： Chun-Shan Wei Naja Becher jenny Blechingberg Friis Peter Ott Ida Vogel henning Grønbæk Department of Clinical Medicine-Department of Hepatology and Gastroenterology Aarhus University HospitalAarhus N DK-8200Denmark Department of Hepatology Shenzhen Traditional Chinese Medicine HospitalThe Fourth Clinical Medical College of Guangzhou University of Chinese MedicineShenzhen 518033Guangdong ProvinceChina Department of Clinical Genetics Aarhus University HospitalAarhus N DK-8200Denmark

BACKGROUND Progressive familial intrahepatic cholestasis(PFIC)encompasses a group of autosomal recessive disorders with high morbidity and *** in the gene encoding tight junction protein-2(TjP2)have been linked to PFIC type 4(PFIC4),which predominantly presents in ***,there are only limited data from adults with TjP2-related *** report a family with an autosomal recessive disorder with a novel variant in the TjP2 gene in adults with very variable expression of *** SUMMARY The index patient presented at 19 years old with liver cirrhosis and variceal bleeding and was treated with endoscopic banding and *** 2018,he developed primary liver cancer that was treated with radiofrequency ablation followed by liver transplantation in *** 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 *** homozygous brothers had cirrhosis and portal hypertension at diagnosis at the ages of 19 and *** 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 *** addition,five sisters had severe and mild intrahepatic cholestasis of pregnancy and carry the TjP2 variant in a homozygous and heterozygous state,*** This novel TjP2 variant is associated with PFIC4 causing severe liver disease with cirrhosis and primary liver cancer in adolescents/adults.

关键词： Progressive familial intrahepatic cholestasis Tight junction protein 2 Genetic variants Liver cirrhosis Liver cancer Case report

Comparison of perioperative outcomes between laparoscopic and open partial splenectomy in children and adolescents

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World journal of Gastrointestinal Surgery 2021年第9期13卷 979-987页

作者： Mohamed Makansi Martin Hutter Till-Martin Theilen henning C Fiegel Udo Rolle Stefan Gfroerer Department of Pediatric Surgery and Pediatric Urology University Hospital FrankfurtFrankfurt am Main 60590Germany Department of Pediatric Surgery and Pediatric Urology Helios Clinic Berlin-BuchBerlin 13125Germany

BACKGROUND In order to avoid consequences of total splenectomy,partial splenectomy(PS)is increasingly *** purpose of this study was to compare perioperative outcomes of laparoscopic PS(LPS)and open PS(OPS)in children and *** To compare perioperative outcomes of patients with LPS and *** After institutional review board approval,a total of 26 patients that underwent LPS or OPS between january 2008 and july 2018 were identified from the database of our tertiary referral *** total,10 patients had LPS,and 16 patients underwent *** loss was calculated by Mercuriali’s *** scores,analgesic requirements and complications were *** Wilcoxon rank sum test was used for *** compare categorical variables,Fisher’s exact test was *** LPS was performed in 10 patients;16 patients had ***(except for body mass index and duration of follow-up),indicating primary disease,preoperative spleen size and postoperative spleen volume,perioperative hematological parameters,postoperative pain scores,analgesic requirements,adverse events according to the Clavien-Dindo classification and the comprehensive complication index,median time from operation to initiation of feeds,median time from operation to full feeds,median time from operation to mobilization and median length of hospital stay did not differ between LPS and ***(range)operative time(min)was longer in LPS compared to the OPS group[185(135-298)vs 144(112-270),respectively;P=0.048].Calculated perioperative blood loss(mL of red blood cell count)was higher in the LPS group compared to OPS[87(-45-777)vs-37(-114-553),respectively;P=0.039].CONCLUSION This is the first study that compared outcomes of LPS and *** operative approaches had comparable perioperative *** appears to be a viable alternative to OPS.

关键词： Laparoscopic vs open Laparoscopy Partial splenectomy Perioperative outcome Children Adolescents

Diagnostic procedures and classification of antisocial behavior in Norwegian inmates in preventive detention

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Open journal of Psychiatry 2012年第3期2卷 207-210页

作者： henning Vaeroy Department of Psychiatric R & D Akershus University Hospital Lorenskog Norway

In official Norwegian government reports’ prison statistics, it is claimed that the prevalence of Dissocial Personality Disorder (DPD) or Antisocial Personality Disorder (APD) among inmates in preventive detention is approximately 50%. Furthermore, previous findings have described a practice in which forensic examiners use the DSM SCID axis II for APD to confirm an ICD 10 diagnosis of DPD. Clinical investigation supported by the use of SCID Axis II for quality assurance was performed on almost half the population of inmates (46.4%) in preventive detention at a high security prison. The inmates had all committed severe violent acts including murder. All the information obtained by applying the DSM IV-TR criteria was tested against the ICD-10 Research Criteria (ICD-10-RC) for Dissocial Personality Disorder (ICD-10, DPD). It was found that all inmates met the ICD-10-RC for (DPD) and the DSM-IV-TR definition for Adult Antisocial Behavior (AAB). On the other hand, none met the DSM-IV-TR criteria for (APD). The SCID Axis II failed to identify inmates with APD because the DSM-IV-TR C-criteria, referring to symptoms of childhood Conduct Disorder (CD), were not met. These findings raise important questions since the choice of diagnostic system may influence whether a person’s clinically described antisocial behaviour should be classified as a personality disorder or not. For the inmates, a diagnosis of APD or DPD may compromise their legal rights and affect decisions on prolongation of the preventive detention. Studies have shown that combining the DSM and the ICD diagnostic systems may have consequences for the reliability of the diagnosis.

关键词： Antisocial Personality Disorder Adult Antisocial Behavior Forensic Psychiatry Prisoners Preventive Detention

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涡轮分子泵发展及使用趋势

真空 1980年第1期 68-75页

作者： j.henning 梅占先 (西德)

HG-LG Mode Conversion with Stressed 3-Mode Fibers under Polarization

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Open journal of Applied Sciences 2012年第4期2卷 224-227页

作者： henning Soller Institut für Theoretische Physik Rupprecht-Karls-Universitat HeidelbergHeidelbergGermany

We have coupled an upright HG mode into a fiber-optic waveguide and used the application of stress to generate a Laguerre-Gaussian laser mode. We have generalized previous results by McGloin et al. by using a polarized input beam, a true 3-mode fiber and by applying the stress on a stripped piece of the optical waveguide. These generalizations are necessary in order to perform quantum information experiments and obtain reliable information on the stress imposed on the optical fiber.

关键词： Mode Conversion Stress Optical Fiber Laguerre-Gaussian Laser Mode

Opening a window to skin biomarkers for diabetes stage with optoacoustic mesoscopy

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Light(Science & Applications) 2023年第11期12卷 2335-2349页

作者： Hailong He Nikolina-Alexia Fasoula Angelos Karlas Murad Omar juan Aguirre jessica Lutz Michael Kallmayer Martin Fuchtenbusch Hans-henning Eckstein Annette Ziegler Vasilis Ntziachristos Institute of Biological and Medical Imaging Helmholtz Zentrum MunchenNeuherbergGermany Chair of Biological Imaging at the Central Institute for Translational Cancer Research(TranslaTUM) School of MedicineTechnical University of MunichMunichGermany Department for Vascular and Endovascular Surgery Klinikum rechts der IsarTechnical University of Munich(TUM)MunichGermany DZHK(German Centre for Cardiovascular Research) partner site Munich Heart AllianceMunichGermany Diabetes Center at Marienplatz MunichGermany Forschergruppe Diabetes e.V. Helmholtz Zentrum MunchenNeuherbergGermany Institute of Diabetes Research Helmholtz Zentrum MunchenNeuherbergGermany

Being the largest and most accessible organ of the human body,the skin could offer a window to diabetes-related complications on the ***,skin microvasculature is typically assessed by histological analysis,which is not suited for applications to large populations or longitudinal *** introduce ultra-wideband rasterscan optoacoustic mesoscopy(RSOM)for precise,non-invasive assessment of diabetes-related changes in the dermal microvasculature and skin micro-anatomy,resolved with unprecedented sensitivity and detail without the need for contrast *** unique imaging contrast,we explored a possible role for RSOM as an investigational tool in diabetes healthcare and offer the first comprehensive study investigating the relationship between different diabetes complications and microvascular features in *** applied RSOM to scan the pretibial area of 95 participants with diabetes mellitus and 48 age-matched volunteers without diabetes,grouped according to disease complications,and extracted six label-free optoacoustic biomarkers of human skin,including dermal microvasculature density and epidermal parameters,based on a novel image-processing *** then correlated these biomarkers to disease severity and found statistically significant effects on microvasculature parameters as a function of diabetes *** discuss how label-free RSOM biomarkers can lead to a quantitative assessment of the systemic effects of diabetes and its complications,complementing the qualitative assessment allowed by current clinical metrics,possibly leading to a precise scoring system that captures the gradual evolution of the disease.

关键词： diabetes anatomy precise

TRAIL-induced apoptosis of hepatocellular carcinoma cells is augmented by targeted therapies

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World journal of Gastroenterology 2009年第47期15卷 5924-5935页

作者： Bruno Christian Koehler Toni Urbanik Binje Vick Regina johanna Boger Steffen Heeger Peter R Galle Marcus Schuchmann henning Schulze-Bergkamen First Department of Medicine Johannes Gutenberg-University Mainz55101 MainzGermany Steffen HeegerMerck KGaAMS-D Global Clinical Development Unit-Oncology64293 DarmstadtGermany National Center of Tumor Diseases Department of Medical OncologyUniversity Clinic of Heidelberg69120 HeidelbergGermany

AIM:To analyze the effect of chemotherapeutic drugs and specific kinase inhibitors,in combination with the death receptor ligand tumor necrosis factor-related apoptosis inducing ligand(TRAIL),on overcoming TRAIL resistance in hepatocellular carcinoma(HCC)and to study the efficacy of agonistic TRAIL antibodies,as well as the commitment of antiapoptotic BCL-2 proteins, in TRAIL-induced apoptosis. METHODS:Surface expression of TRAIL receptors (TRAIL-R1-4)and expression levels of the antiapoptotic BCL-2 proteins MCL-1 and BCL-xL were analyzed by flow cytometry and Western blotting,respectively. Knock-down of MCL-1 and BCL-xL was performed by transfecting specific small interfering *** cellswere treated with kinase inhibitors and chemotherapeutic *** induction and cell viability were analyzed via flow cytometry and 3-(4,5-Dimethyl-thiazol-2-yl)-2,5-diphenyltetrazolium bromide assay. RESULTS:TRAIL-R1 and-R2 were profoundly expressed on the HCC cell lines Huh7 and Hep-G2. However,treatment of Huh7 and Hep-G2 with TRAIL and agonistic antibodies only induced minor apoptosis *** resistance towards TRAIL could be considerably reduced by adding the chemotherapeutic drugs 5-fluorouracil and doxorubicin as well as the kinase inhibitors LY294002[inhibition of phosphoinositol- 3-kinase(PI3K)],AG1478(epidermal growth factor receptor kinase),PD98059(MEK1),rapamycin(mam- malian target of rapamycin)and the multi-kinase inhibitor ***,the antiapoptotic BCL-2 proteins MCL-1 and BCL-xL play a major role in TRAIL resistance:knock-down by RNA interference increased TRAIL-induced apoptosis of HCC ***, knock-down of MCL-1 and BCL-xL led to a significant sensitization of HCC cells towards inhibition of both c-jun N-terminal kinase and ***:Our data identify the blockage of survival kinases,combination with chemotherapeutic drugs and targeting of antiapoptotic BCL-2 proteins as promising ways to overcome TRAIL resistance in

关键词：肿瘤坏死因子相关凋亡诱导配体细胞凋亡基因 TRAIL PD98059 受体激酶流式细胞仪 RNA干扰酶抑制剂

Utility of endoscopic ultrasound in pancreatitis:A review

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World journal of Gastroenterology 2007年第47期13卷 6321-6326页

作者： Maged K Rizk henning Gerke University of Iowa Hospitals and Clinics Division of Gastroenterology and Hepatology Department of Internal Medicine 200 Hawkins Drive Iowa City IA 52242 United States

The close proximity of the endoscopic ultrasound probe to the pancreas results in superior spatial resolution compared to CT scan and MRI. In addition, endoscopic ultrasound (EUS) is a minimally invasive procedure that does not share the relatively high complication rate of ERCP. Due to these advantages, EUS has evolved into an important technique to assess pancreatobiliary disease. This review will discuss the role of EUS in patients with pancreatitis. The indications can be divided into acute pancreatitis and chronic pancreatitis. In acute pancreatitis, EUS is used to determine the etiology; in suspected chronic pancreatitis it is helpful to establish the diagnosis. Lastly, this review will discuss biliary pancreatitis with suspicion for persistent choledocholithiasis.

关键词：先天胰腺炎急性胰腺炎慢性胰腺炎超声检查

Herbicidal Effects of Fungicides on Arable Weeds

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American journal of Plant Sciences 2014年第1期5卷 35-41页

作者： henning Nordmeyer Tobias Koch julius Kühn-Institute Institute for Plant Protection in Field Crops and Grassland Braunschweig Germany.

In the present study, the herbicidal effects of two fungicides with the active ingredients expoxiconazole, fluxapyroxad, pyraclostrobin and fenpropimorph on the two arable weeds Lamium purpureum L. and Chenopodium album L. were investigated. The experiments were conducted in a climate chamber under defined conditions. Sowing pods were prepared and plants at the cotyledon leaf stage were pricked out in test pods. Fungicides were applied at six application rates: 0%, 12.5%, 25%, 50%, 100% and 200% of the maximum registered dose rate in Germany. Seven days after application, the first assessment was conducted, regarding growth stage, quantity of plants and visible plant damage. Fourteen days after application, the second and final assessment was conducted, regarding growth stage, quantity of plants, visible plant damage and the fresh weight of the plants. There were herbicidal effects of the fungicides, which were presumably due to the active ingredient epoxiconazole. Epoxiconazole has effects on enzymes that are dependent on cytochrome P-450 and inhibits sterol biosynthesis and probably gibberellin synthesis. By doing so, these fungicides have similar effects to plant growth regulators. Weed species showed differential sensitivities, contractions and growth inhibition and ED50-values were calculated. Notwithstanding the probably minor relevance of the present results in agricultural practice, some effects on arable weeds might result if fungicides are applied at an early growth stage in the field. In some cases, the crop-weed competition could be shifted to the benefit of the crop. Nevertheless, the results are interesting for the field of weed research and for the assessment of the ecotoxicology of fungicides.

关键词： Chenopodium album Dose Response Epoxiconazole Fungicide Treatments Lamium purpureum Nontarget Effects Plant Bioassay Weed Species