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Late-onset Cholestasis with Paucity of Portal Area Secondary 􀁗􀁒􀀃􀀫􀀱􀀩􀀔􀈕􀀃􀀧􀁈􀁉􀁌􀁆􀁌􀁈􀁑􀁆􀁜􀀃􀁌􀁑􀀃􀀤􀁇􀁘􀁏􀁗􀁋􀁒􀁒􀁇􀀝􀀃􀀤􀀃􀀦􀁄􀁖􀁈􀀃􀀵􀁈􀁓􀁒􀁕􀁗

Journal of Clinical and Translational Hepatology 2024年第3期12卷 327-331页

作者： Xuemei Zhang Kun Liu Xiaona Lu Wenlan Zheng Jia Shi Shihan Yu Hai Feng Zhuo Yu Department of Hepatopathy Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese MedicineShanghaiChina Department of Pathology Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese MedicineShanghaiChina Institute of Infectious Disease Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese MedicineShanghaiChina

Hepatocyte nuclear factor 1β(HNF1β)is essential for biliary development,while its genetic defect triggers the dysplasia of interlobular bile ducts,leading to life-threatening hepatitis and *** date,this disorder has mainly been docu-mented in ***,we report a case of cholestasis in an adult patient caused by a de novo HNF1β mutation.A liver biopsy revealed remarkable shrinkage of the portal area accompanied by a decrease or absence of interlobular bile ducts,veins,and arteries in the portal *** case showed that an HNF1β defect could induce late-onset cholestasis with paucity of the portal area in adulthood.

关键词： HNF1β Cholestasis Adult patient Paucity of the portal area renal cysts Case report

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Clinicopathological characteristics and typing of multilocular cystic renal neoplasm of low malignant potential

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World Journal of Clinical Cases 2024年第14期12卷 2332-2341页

作者： Wen-Long Gao Gang Li Dong-Sheng Zhu Yuan-Jie Niu Department of Urology The Second Hospital of Tianjin Medical UniversityTianjin 300211China Department of Pediatric Surgery The First People’s Hospital of LianyungangLianyungang 222000Jiangsu ProvinceChina

BACKGROUND Up until now,no research has been reported on the association between the cli-nical growth rate of multilocular cystic renal neoplasm of low malignant potential(MCRNLMP)and computed tomography(CT)imaging *** study sought to examine the correlation between them,with the objective of distin-guishing unique features of MCRNLMP from renal cysts and exploring effective management *** To investigate optimal management strategies of *** We retrospectively collected and analyzed data from 1520 patients,comprising 1444 with renal cysts and 76 with MCRNLMP,who underwent renal cyst decom-pression,radical nephrectomy,or nephron-sparing surgery for renal cystic disease between January 2013 and December 2021 at our *** of MC-RNLMP utilized the Bosniak classification for imaging and the 2016 World Health Organization criteria for clinical *** Our meticulous exploration has revealed compelling findings on the occurrence of ***,it comprises 1.48%of all cases involving simple renal cysts,5.26%of those with complex renal cysts,and a noteworthy 12.11%of renal tumors coexisting with renal cysts,indicating a statistically significant difference(P=0.001).Moreover,MCRNLMP constituted a significant 22.37%of the patient po-pulation whose cysts demonstrated a rapid growth rate of≥2.0 cm/year,whereas it only represented 0.66%among those with a growth rate below 2.0 cm/*** the 76 MCRNLMP cases studied,none of the nine patients who underwent subsequent nephron-sparing surgery or radical nephrectomy following renal cyst decompression experienced recurrence or *** the remaining 67 patients,who were actively monitored over a 3-year postoperative period,only one showed suspicious recurrence on CT *** MCRNLMP can be tentatively identified and categorized into three types based on CT scanning and growth rate *** treating MCRNLMP,partial nephrectomy is preferred,while rad

关键词： renal cysts Multilocular cystic renal neoplasm of low malignant potential Computed tomography Diagnosis Treatment

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Tesevatinib ameliorates progression of polycystic kidney disease in rodent models of autosomal recessive polycystic kidney disease

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World Journal of Nephrology 2017年第4期6卷 188-200页

作者： William E Sweeney Philip Frost Ellis D Avner Children’s Research Institute Children’s Hospital Health System of Wisconsin and the Medical College of WisconsinMedical College of Wisconsin Calesca Pharmaceuticals Inc.

AIMTo investigate the therapeutic potential of tesevatinib （TSV）, a unique multi-kinase inhibitor currently in Phase Ⅱ clinical trials for autosomal dominant polycystic kidney disease （ADPKD）, in well-defined rodent models of autosomal recessive polycystic kidney disease （ARPKD）. METHODSWe administered TSV in daily doses of 7.5 and 15 mg/kg per day by I.P. to the well characterized bpk model of polycystic kidney disease starting at postnatal day（PN） 4 through PN21 to assess efficacy and toxicity in neonatal mice during postnatal development and still undergoing renal maturation. We administered TSV by oral gavage in the same doses to the orthologous PCK model （from PN30 to PN90） to assess effcacy and toxicity in animals where developmental processes are complete. The following parameters were assessed： Body weight, total kidney weight; kidney weight to body weight ratios; and morphometric determination of a cystic index and a measure of hepatic disease. renal function was assessed by： Serum BUN; creatinine; and a 12 h urinary concentrating ability. Validation of reported targets including the level of angiogenesis and inhibition of angiogenesis （active VEGFR2/KDR） was assessed by Western *** study demonstrates that：（1） in vivo pharmacological inhibition of multiple kinase cascades with TSV reduced phosphorylation of key mediators of cystogenesis： EGFR, ErbB2, c-Src and KDR; and （2） this reduction of kinase activity resulted in signifcant reduction of renal and biliary disease in both bpk and PCK models of ARPKD. The amelioration of disease by TSV was not associated with any apparent *** data supports the hypothesis that this multi-kinase inhibitor TSV may provide an effective clinical therapy for human ARPKD.

关键词： Autosomal recessive Autosomal dominant Polycystic kidney disease Therapy Kinase inhibition Multi-kinase inhibitor Phosphorylation renal cysts Biliary G-protein coupled receptor

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Impact of contrast-enhanced ultrasound in patients with renal function impairment

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World Journal of Radiology 2017年第1期9卷 10-16页

作者： Rossano Girometti Tiziano Stocca Elena Serena Antonio Granata Michele Bertolotto Institute of Diagnostic Radiology Department of Medical and Biological SciencesUniversity of UdineUniversity Hospital 'S. Maria della Misericordia' S.O.C. Radiologia San Giovanni di Dio Hospital Department of Radiology University of TriesteCattinara Hospital UOC di Nefrologia-Dialisi 'San Giovanni di Dio' Hospital

AIMTo investigate the role of contrast enhanced ultrasound (CEUS) in evaluating patients with renal function impairment (RFI) showing: (1) acute renal failure (ARF) of suspicious vascular origin; or (2) suspicious renal *** retrospectively evaluated patients addressed to CEUS over an eight years period to rule-out vascular causes of ARF (first group of 50 subjects) or assess previously found suspicious renal lesions (second group of 41 subjects with acute or chronic RFI). After preliminary grey-scale and color Doppler investigation, each kidney was investigated individually with CEUS, using 1.2-2.4 mL of a sulfur hexafluoride-filled microbubble contrast agent. Image analysis was performed in consensus by two readers who reviewed digital clips of CEUS. We calculated the detection rate of vascular abnormalities in the first group and performed descriptive statistics of imaging findings for the second *** the first group, CEUS detected renal infarction or cortical ischemia in 18/50 patients (36%; 95%CI: 23.3-50.9) and 1/50 patients (2%; 95%CI: 0.1-12), respectively. The detection rate of infarction was significantly higher (P = 0.0002; McNemar test) compared to color Doppler ultrasonography (10%). No vascular causes of ARF were identified in the remaining 31/50 patients (62%). In the second group, CEUS detected 41 lesions on 39 patients, allowing differentiation between solid lesions (21/41; 51.2%) vs complex cysts (20/41; 48.8%), and properly addressing 15/39 patients to intervention when feasible based on clinical conditions (surgery and cryoablation in 13 and 2 cases, respectively). cysts were categorized Bosniak II, IIF, III and IV in 8, 5, 4 and 3 cases, respectively. In the remaining two patients, CEUS found 1 pseudolesion and 1 subcapsular *** showed high detection rate of renal perfusion abnormalities in patients with ARF, influencing the management of patients with acute or chronic RFI and renal masses throughout t

关键词： Contrast-enhanced ultrasonography renal function impairment Acute renal failure renal infarction renal lesions renal cysts Bosniak classification

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