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Polycystic liver

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https://www.readbyqxmd.com/read/29643536/-genetic-diagnosis-of-caroli-syndrome-with-autosomal-recessive-polycystic-kidney-disease-a-case-report-and-literature-review
#1
X Y Yang, L P Zhu, X Q Liu, C Y Zhang, Y Yao, Y Wu
This case report is about one genetically specified diagnosed infant case of Caroli syndrome with autosomal recessive polycystic kidney disease (ARPKD) in China. The patient in this case report was an eight-month infant boy with an atypical onset and the main clinical manifestation was non-symptomatic enlargement of the liver and kidneys. The imaging study demonstrated a diffused cystic dilatation of intrahepatic bile ducts as well as polycystic changes in bilateral kidneys. The basic blood biochemical tests indicated a normal hepatorenal function...
April 18, 2018: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29622386/long-term-outcome-of-transjugular-intrahepatic-portosystemic-shunt-for-portal-hypertension-in-autosomal-recessive-polycystic-kidney-disease
#2
Sarah Verbeeck, Djalila Mekhali, David Cassiman, Geert Maleux, Peter Witters
BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) with congenital hepatic fibrosis (CHF) causes portal hypertension and its complications. A transjugular intrahepatic portosystemic shunt (TIPSS) could serve as a symptomatic treatment for portal hypertension-related symptoms in these children. AIMS: To study the effect of TIPSS on portal hypertension, liver and kidney function and the long term complications. MATERIALS AND METHODS: We report on 5 children with CHF treated with a TIPSS to manage severe portal hypertension related symptoms...
March 15, 2018: Digestive and Liver Disease
https://www.readbyqxmd.com/read/29618028/hepatic-production-of-fibroblast-growth-factor-23-in-autosomal-dominant-polycystic-kidney-disease
#3
Frank Bienaimé, Ariane Ambolet, Béatrice Aussilhou, François Brazier, Marie Fouchard, Amandine Viau, Pauline Barre, Anne-Marie Tissier, Jean-Michel Correas, Valérie Paradis, Fabiola Terzi, Gérard Friedlander, Bertrand Knebelmann, Dominique Joly, Dominique Prié
Context: the bone-derived hormone fibroblast growth factor 23 (FGF23) controls phosphate homeostasis and urinary phosphate excretion. FGF23 plasma levels increase at the early step of renal insufficiency to prevent hyperphosphatemia. Recent evidence suggests that this increase has off-target effects on cardiac and immune cells that compromises patients' health. Subjects with autosomal dominant polycystic kidney disease (ADPKD) have been reported to display higher FGF23 concentrations than non-ADPKD patients with similar renal function...
March 29, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29606941/a-surgically-resected-pancreatic-schwannoma-with-obstructive-jaundice-with-special-reference-to-differential-diagnosis-from-other-cystic-lesions-in-the-pancreas
#4
Takayoshi Watanabe, Kenichiro Araki, Norihiro Ishii, Takamichi Igarashi, Akira Watanabe, Norio Kubo, Hiroyuki Kuwano, Ken Shirabe
Pancreatic schwannomas are uncommon. About 60% of pancreatic schwannomas develop cystic lesions, and the differential diagnosis from other cystic pancreatic tumors is difficult. A 43-year-old man presented for evaluation of liver dysfunction detected during a medical checkup. Blood testing detected obstructive jaundice. A computed tomography scan revealed a well-defined polycystic tumor of about 5 cm at the pancreatic head. We performed surgical resection to treat the patient's symptoms and facilitate long-term management...
January 2018: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/29590099/polycystic-ovary-syndrome-androgen-excess-and-the-risk-of-nonalcoholic-fatty-liver-disease-in-women-a-longitudinal-study-based-on-a-united-kingdom-primary-care-database
#5
Balachandran Kumarendran, Michael W O'Reilly, Konstantinos N Manolopoulos, Konstantinos A Toulis, Krishna M Gokhale, Alice J Sitch, Chandrika N Wijeyaratne, Arri Coomarasamy, Wiebke Arlt, Krishnarajah Nirantharakumar
BACKGROUND: Androgen excess is a defining feature of polycystic ovary syndrome (PCOS), which affects 10% of women and represents a lifelong metabolic disorder, with increased risk of type 2 diabetes, hypertension, and cardiovascular events. Previous studies have suggested an increased risk of nonalcoholic fatty liver disease (NAFLD) in individuals with PCOS and implicated androgen excess as a potential driver. METHODS AND FINDINGS: We carried out a retrospective longitudinal cohort study utilizing a large primary care database in the United Kingdom, evaluating NAFLD rates in 63,120 women with PCOS and 121,064 age-, body mass index (BMI)-, and location-matched control women registered from January 2000 to May 2016...
March 2018: PLoS Medicine
https://www.readbyqxmd.com/read/29587769/circulating-osteopontin-and-its-association-with-liver-fat-content-in-non-obese-women-with-polycystic-ovary-syndrome-a-case-control-study
#6
Yuying Wang, Wei Zhou, Chunhua Wu, Yi Zhang, Tzuchun Lin, Yun Sun, Wei Liu, Tao Tao
BACKGROUND: Osteopontin (OPN) plays an important role in inflammatory processes and insulin resistance. Polycystic ovary syndrome (PCOS) is a reproductive metabolic disease associated with insulin resistance and metabolic abnormalities, including high levels of liver fat content (LFC). The objective of this study was to explore whether circulating OPN independently contributes to elevated LFC in non-obese PCOS patients. METHODS: This study included 61 non-obese PCOS patients and 56 age-matched healthy women from Shanghai, China...
March 27, 2018: Reproductive Biology and Endocrinology: RB&E
https://www.readbyqxmd.com/read/29587557/bile-acids-as-potential-biomarkers-to-assess-liver-impairment-in-polycystic-kidney-disease
#7
William J Brock, James J Beaudoin, Jason R Slizgi, Mingming Su, Wei Jia, Sharin E Roth, Kim L R Brouwer
Polycystic kidney disease is characterized by the progressive development of kidney cysts and declining renal function with frequent development of cysts in other organs including the liver. The polycystic kidney (PCK) rat is a rodent model of polycystic liver disease that has been used to study hepatorenal disease progression and evaluate pharmacotherapeutic interventions. Biomarkers that describe the cyst progression, liver impairment, and/or hepatic cyst burden could provide clinical utility for this disease...
March 2018: International Journal of Toxicology
https://www.readbyqxmd.com/read/29579867/multicentric-study-of-the-andalusian-experience-in-polycystic-liver-disease-as-indication-for-liver-transplantation
#8
S Dios-Barbeito, M Domínguez-Bastante, A Moreno-Navas, F J León-Díaz, Y Fundora-Suárez, F J Briceño-Delgado, M Pitarch-Martínez, M Á Gómez-Bravo
BACKGROUND: The purpose of this study was to determine the morbidity and survival in patients with polycystic liver disease (PLD) undergoing liver transplantation (LT) in 4 Spanish hospitals. METHODS: A multicentric retrospective study using a prospective database was designed including 19 LTs after PLD diagnosis performed from January 1, 1990, to December 31, 2016. Pediatric patients were excluded from the analysis. RESULTS: Of the included patients, 63...
March 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29565498/minimally-invasive-management-of-hepatic-cysts-indications-and-complications
#9
D Vardakostas, C Damaskos, N Garmpis, E A Antoniou, K Kontzoglou, G Kouraklis, D Dimitroulis
OBJECTIVE: Liver cysts are divided into congenital and acquired. Congenital cystic lesions include polycystic liver disease, simple cysts, duct related and ciliated hepatic foregut cysts. Acquired cystic lesions are divided into infectious and non-infectious. The infectious cysts are the hydatid cyst, the amoebic abscess, and the pyogenic abscess, whereas the non-infectious cysts are neoplastic cysts and false cysts. While modern medicine provides a lot of minimally invasive therapeutic modalities, there has emerged a pressing need for understanding the various types of liver cysts, the possible minimal therapeutic options along with their indications and complications...
March 2018: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29557942/development-and-validation-of-a-patient-reported-outcome-measurement-for-symptom-assessment-in-cirrhotic-ascites
#10
Myrte Neijenhuis, Tom J G Gevers, Thomas D Atwell, Tim J Gunneson, Amanda C Schimek, Wietske Kievit, Joost P H Drenth, Patrick S Kamath
OBJECTIVES: As current treatments of cirrhotic ascites are not associated with survival benefit, symptom relief is the major therapeutic end point. We developed a questionnaire (Ascites-Q; modified polycystic liver disease questionnaire) and assessed validity and responsiveness for symptom assessment in cirrhotic ascites. METHODS: Ascites-Q was compared with Functional Assessment of Chronic Illness Therapy-Ascites Index (FACIT-AI; developed for malignant ascites) and Japanese Ascites Symptom Inventory-7 (ASI-7) in cirrhotics undergoing large-volume paracentesis...
March 20, 2018: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/29543047/a-case-report-of-an-angiomyxoma-in-the-liver
#11
A K Malik, R Filobbos, A Manoharan, N Harvey, D A O'Reilly, N de Liguori Carino
Angiomyxomas are uncommon myxoid tumours arising most commonly from the pelvis. A 46-year-old woman with a history of polycystic kidney disease presented asymptomatically for surveillance ultrasonography; changes were noted in the size and morphology of her liver cysts. Subsequent radiological assessment displayed features suspicious of malignancy and a right hemihepatectomy was performed with curative intent. Pathological examination of the resected specimen found histology consistent with an angiomyxoma arising primarily from the liver parenchyma...
March 15, 2018: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/29538364/quantitative-magnetic-resonance-imaging-mri-assessments-of-autosomal-recessive-polycystic-kidney-disease-arpkd-progression-and-response-to-therapy-in-an-animal-model
#12
Bernadette O Erokwu, Christian E Anderson, Chris A Flask, Katherine M Dell
BACKGROUND: Autosomal Recessive Polycystic Kidney Disease (ARPKD) is associated with significant mortality and morbidity and there are currently no disease-specific treatments available for ARPKD patients. One major limitation in establishing new therapies for ARPKD is a lack of sensitive measures of kidney disease progression. Magnetic Resonance Imaging (MRI) can provide multiple quantitative assessments of disease. METHODS: We applied quantitative image analysis of high resolution (non-contrast) T2-weighted MRI techniques to study cystic kidney disease progression and response to therapy in the PCK rat model of ARPKD...
March 14, 2018: Pediatric Research
https://www.readbyqxmd.com/read/29527983/preoperative-echocardiographic-differences-and-transplant-outcomes-among-patients-receiving-simultaneous-liver-kidney-versus-liver-transplant-alone
#13
Nik R Mazumder, Stuart D Russell, Aliaksei Pustavoitau, Matthews Chacko, Nicole Rizkalla, Behnam Saberi, Benjamin Philosophe, Andrew M Cameron, Ahmet Gurakar, Fizza F Naqvi
OBJECTIVES: Liver transplant and simultaneous liver-kidney transplant are major surgeries performed on high-risk individuals with end-stage liver disease and end-stage renal disease. We sought to examine the relationship between pretransplant echocardiographic parameters and outcomes in our simultaneous liver-kidney transplant and liver transplant-alone populations. MATERIALS AND METHODS: In our retrospective analysis, we included adult patients who underwent index transplant from January 1, 2010 to December 31, 2015 at Johns Hopkins Comprehensive Transplant Center...
March 2018: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/29520754/kidney-enlargement-and-multiple-liver-cyst-formation-implicate-mutations-in-pkd1-2-in-adult-sporadic-polycystic-kidney-disease
#14
T Fujimaru, T Mori, A Sekine, S Mandai, M Chiga, H Kikuchi, F Ando, Y Mori, N Nomura, S Iimori, S Naito, T Okado, T Rai, J Hoshino, Y Ubara, S Uchida, E Sohara
Distinguishing autosomal dominant polycystic kidney disease (ADPKD) from other inherited renal cystic diseases in patients with adult polycystic kidney disease and no family history is critical for correct treatment and appropriate genetic counseling. However, for patients with no family history, there are no definitive imaging findings that provide an unequivocal ADPKD diagnosis. We analyzed 53 adult polycystic kidney disease patients with no family history. Comprehensive genetic testing was performed using capture-based next-generation sequencing for 69 genes currently known to cause hereditary renal cystic diseases including ADPKD...
March 9, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29520518/scanning-electron-microscopy-of-polycystic-liver-disease
#15
Kazutaka Kojima, Masaji Hashimoto, Yoshifumi Ubara
Resected specimens of PCLD by laparoscopic fenestration surgery were evaluated by scanning electron microscopy. Epithelium lining the largest cyst (26 cm in size) showed prominent villous proliferation with positivity of Ki-67, while the epithelium of the small cyst (3 cm in size) showed slight proliferation (smooth) with small positivity of Ki-67.
March 8, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29518665/sharp-liver-excision-under-hepatic-vascular-exclusion-in-case-of-liver-transplant-for-large-polycystic-disease-case-report-of-a-new-surgical-technique
#16
Filip Thieme, Jiri Fronek
INTRODUCTION: Polycystic liver disease is observed in 75-90% of patients with autosomal dominant polycystic kidney disease (ADPKD). ADPKD has a high prevalence of 1/1000. Hepatomegaly severely reduces quality of life and liver transplantation seems to be method of choice for many patients. Because of the rarity of this disease and the small number of symptomatic patients with massive hepatomegaly indicated for the transplantation, there is no standard approach for explantation of the liver...
January 8, 2018: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29508162/elevation-of-the-serum-liver-enzyme-levels-during-tolvaptan-treatment-in-patients-with-autosomal-dominant-polycystic-kidney-disease-adpkd
#17
Shiho Makabe, Toshio Mochizuki, Michihiro Mitobe, Yumi Aoyama, Hiroshi Kataoka, Ken Tsuchiya, Kosaku Nitta
BACKGROUND: In 2014, tolvaptan, a vasopressin receptor antagonist, was approved for the treatment of autosomal dominant polycystic kidney disease (ADPKD) in Japan. Clinical trials of tolvaptan revealed frequent occurrence of the liver function abnormality. According to the package insert in Japan, liver function tests should be performed once a month in patients receiving tolvaptan. Furthermore, immediate discontinuation of tolvaptan is recommended in the appearance of liver function abnormalities...
March 5, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29487756/liver-transplant-for-unusually-large-polycystic-liver-disease-challenges-and-pitfalls
#18
Pablo Serrano Rodriguez, Alfred Sidney Barritt Iv, David Allen Gerber, Chirag Sureshchandra Desai
Patients with polycystic liver disease are described in the literature as both recipient and donor for liver transplant. Due to well-preserved liver function, it is often difficult for these patients to receive an organ. Livers of these patients are often large and heavier than a normal organ. We describe two cases who had exceedingly large livers, weighing 14 and 19 kg. To the best of our knowledge and search, these are some of the heaviest explanted livers, and one of the patients incidentally received a liver from a donor with ADPKD...
2018: Case Reports in Transplantation
https://www.readbyqxmd.com/read/29485445/fdg-pet-ct-findings-of-polymorphic-posttransplant-lymphoproliferative-disorders-in-a-transplanted-kidney
#19
Yuanyuan Yang, Wei Wang, Ying Kan, Jie Liu, Jigang Yang
A 55-year-old woman underwent simultaneous liver-kidney transplantation because of polycystic liver and kidney. Six months later, FDG PET/CT was acquired to evaluate possible lesions in the renal graft, which demonstrated abnormal F-FDG accumulation in the renal pelvic lesions without other abnormality. The subsequent pathology examination after biopsy demonstrated the polymorphic posttransplant lymphoproliferative disorders.
February 27, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29473360/-exploration-of-the-meridian-differentiation-law-in-polycystic-ovarian-syndrome-of-hirsutism-based-on-data-mining-technology
#20
Lingyu Shen, Yu Xing, Qiudan Lu, Cuimei Liang, Wenjin Yang, Hui Hu
OBJECTIVE: To analyze the distribution characteristics of hirsutism and the law of meridian differentiation in polycystic ovarian syndrome (PCOS). METHODS: For the patients of PCOS, the modified Ferriman-Gallwey score (mF-G score) was adopted to evaluate the hirsutism distribution. The clementine data mining software was used to analyze the distribution region of hirsutism and the correlation with meridian. RESULTS: A total of 141 patients of PCOS participated in the study...
February 12, 2018: Zhongguo Zhen Jiu, Chinese Acupuncture & Moxibustion
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