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Polycystic liver

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https://www.readbyqxmd.com/read/28814334/abernethy-malformation-associated-with-caroli-s-syndrome-in-a-patient-with-a-pkhd1-mutation-a-case-report
#1
Xiao-Xiao Mi, Xiao-Guang Li, Zi-Rong Wang, Ling Lin, Chun-Hai Xu, Jun-Ping Shi
BACKGROUND: Abernethy malformation is a rare congenital anomaly characterised by the partial or complete absence of the portal vein and the subsequent development of an extrahepatic portosystemic shunt. Caroli's disease is a rare congenital condition characterised by non-obstructive saccular intrahepatic bile duct dilation. Caroli's disease combined with congenital hepatic fibrosis and/or renal cystic disease is referred to - Caroli's syndrome. The combination of Abernethy malformation and Caroli's syndrome has not been reported previously...
August 16, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28813771/hepatic-adenoma-a-case-report
#2
Hasan H Turaihi, Thavam C Thambi-Pillai
Although liver lesions in the young population are relatively rare, clinicians can benefit from being familiar with a subset of common benign liver lesions which include hepatic adenoma, hepatic hemangioma, and focal nodular hyperplasia. This a case report of a 25-year-old Jehovah's Witness female on chronic oral contraception for polycystic ovarian syndrome who presented with progressive right upper quadrant abdominal pain. Ultrasound and MRI findings were consistent with hepatic adenoma. A description of her clinical work up followed by a brief description of the surgical intervention is discussed...
March 2017: South Dakota Medicine: the Journal of the South Dakota State Medical Association
https://www.readbyqxmd.com/read/28808352/the-effect-of-androgen-excess-on-maternal-metabolism-placental-function-and-fetal-growth-in-obese-dams
#3
Romina Fornes, Manuel Maliqueo, Min Hu, Laila Hadi, Juan M Jimenez-Andrade, Kerstin Ebefors, Jenny Nyström, Fernand Labrie, Thomas Jansson, Anna Benrick, Elisabet Stener-Victorin
Pregnant women with polycystic ovary syndrome (PCOS) are often overweight or obese. To study the effects of maternal androgen excess in obese dams on metabolism, placental function and fetal growth, female C57Bl6J mice were fed a control (CD) or a high fat/high sucrose (HF/HS) diet for 4-10 weeks, and then mated. On gestational day (GD) 15.5-17.5, dams were injected with dihydrotestosterone (CD-DHT, HF/HS-DHT) or a vehicle (CD-Veh, HF/HS-Veh). HF/HS dams had higher fat content, both before mating and on GD18...
August 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28798345/nedd4-family-e3-ligase-dysfunction-due-to-pkhd1-pkhd1-defects-suggests-a-mechanistic-model-for-arpkd-pathobiology
#4
Jun-Ya Kaimori, Cheng-Chao Lin, Patricia Outeda, Miguel A Garcia-Gonzalez, Luis F Menezes, Erum A Hartung, Ao Li, Guanqing Wu, Hideaki Fujita, Yasunori Sato, Yasuni Nakanuma, Satoko Yamamoto, Naotsugu Ichimaru, Shiro Takahara, Yoshitaka Isaka, Terry Watnick, Luiz F Onuchic, Lisa M Guay-Woodford, Gregory G Germino
Autosomal recessive polycystic kidney disease (ARPKD) is an important childhood nephropathy, occurring 1 in 20,000 live births. The major clinical phenotypes are expressed in the kidney with dilatation of the collecting ducts, systemic hypertension, and progressive renal insufficiency, and in the liver with biliary dysgenesis, portal tract fibrosis, and portal hypertension. The systemic hypertension has been attributed to enhanced distal sodium reabsorption in the kidney, the structural defects have been ascribed to altered cellular morphology, and fibrosis to increased TGF-β signaling in the kidney and biliary tract, respectively...
August 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28782656/genetics-and-mechanisms-of-hepatic-cystogenesis
#5
REVIEW
L F M van de Laarschot, J P H Drenth
Polycystic liver disease (PLD) is a heterogeneous genetic condition. PKD1 and PKD2 germline mutations are found in patients with autosomal dominant polycystic kidney disease (ADPKD). Autosomal dominant polycystic liver disease (ADPLD) is associated with germline mutations in PRKCSH, SEC63, LRP5, and recently ALG8, and SEC61. GANAB mutations are found in both patient groups. Loss of heterozygosity of PLD-genes in cyst epithelium contributes to the development of hepatic cysts. A genetic interaction network is implied in hepatic cystogenesis that connects the endoplasmic glycoprotein control mechanisms and polycystin expression and localization...
August 4, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28776546/transarterial-embolization-for-treatment-of-symptomatic-polycystic-liver-disease-more-than-2-year-follow-up
#6
Jin-Long Zhang, Kai Yuan, Mao-Qiang Wang, Jie-Yu Yan, Hai-Nan Xin, Yan Wang, Feng-Yong Liu, Yan-Hua Bai, Zhi-Jun Wang, Feng Duan, Jin-Xin Fu
BACKGROUND: Currently, treatment of symptomatic polycystic liver disease (PLD) is still a challenging problem, especially for these patients who are not feasible for surgery. Minimally invasive options such as laparoscopic fenestration and percutaneous cyst aspiration with sclerotherapy demonstrated disappointing results due to multiple lesions. Because the cysts in PLD are mostly supplied from hepatic arteries but not from portal veins, transcatheter arterial embolization (TAE) of the hepatic artery branches that supply the major hepatic cysts can lead to shrinkage of the cyst and liver size, relieve symptoms, and improve nutritional status...
August 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28768225/complex-liver-cysts-in-autosomal-dominant-polycystic-kidney-disease
#7
Zerwa Farooq, Ashkan Heshmatzadeh Behzadi, Jon D Blumenfeld, Yize Zhao, Martin R Prince
PURPOSE: To determine prevalence of complex liver cysts in Autosomal Dominant Polycystic Kidney Disease (ADPKD). METHODS: Abdominal MRI in 186 ADPKD subjects were evaluated by two independent observers to determine prevalence of complex liver cysts. RESULTS: 23 (12%) of subjects, had at least 1 complex cyst. Only 8 (4%) were reported to have a complex cyst prospectively, representing an under-reporting rate of 65%. Median total cyst volume was 66-times greater for subjects with complex cysts compared to subjects without (p<0...
July 25, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28748913/d-chiro-inositol-effectively-attenuates-cholestasis-in-bile-duct-ligated-rats-by-improving-bile-acid-secretion-and-attenuating-oxidative-stress
#8
Shuang-Shuang Zhao, Na-Ren Li, Wu-Li Zhao, Hong Liu, Mao-Xu Ge, Yi-Xuan Zhang, Long-Yin Zhao, Xue-Fu You, Hong-Wei He, Rong-Guang Shao
Cholestatic liver diseases are important causes of liver cirrhosis and liver transplantation, but few drugs are available for treatment. D-chiro-inositol (DCI), an isomer of inositol found in many Leguminosae plants and in animal viscera, is used clinically for the treatment of polycystic ovary syndrome (PCOS) and diabetes mellitus. In this study, we investigated whether DCI exerted an anti-cholestatic effect and its underlying mechanisms. A cholestatic rat model was established via bile duct ligation (BDL)...
July 27, 2017: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/28729032/a-novel-model-of-autosomal-recessive-polycystic-kidney-questions-the-role-of-the-fibrocystin-c-terminus-in-disease-mechanism
#9
Patricia Outeda, Luis Menezes, Erum A Hartung, Stacey Bridges, Fang Zhou, Xianjun Zhu, Hangxue Xu, Qiong Huang, Qin Yao, Feng Qian, Gregory G Germino, Terry Watnick
Autosomal recessive polycystic kidney disease (OMIM 263200) is a serious condition of the kidney and liver caused by mutations in a single gene, PKHD1. This gene encodes fibrocystin/polyductin (FPC, PD1), a large protein shown by in vitro studies to undergo Notch-like processing. Its cytoplasmic tail, reported to include a ciliary targeting sequence, a nuclear localization signal, and a polycystin-2 binding domain, is thought to traffic to the nucleus after cleavage. We now report a novel mouse line with a triple HA-epitope "knocked-in" to the C-terminus along with lox P sites flanking exon 67, which encodes most of the C-terminus (Pkhd1(Flox67HA))...
July 17, 2017: Kidney International
https://www.readbyqxmd.com/read/28714645/-extra-renal-manifestations-of-the-autosomal-dominant-polycystic-kidney-disease
#10
Aurélie Butscher, Olivier Phan, Olivier Bonny
Extra-renal complications of autosomal dominant polycystic kidney disease (ADPKD) are often unrecognized. Liver cysts are usually benign, but may cause chronic pain, may interfere with nutrition and may necessitate partial liver resection or even liver transplant. Somatostatin analogs may be an alternative therapy. Cerebral aneurisms are rare, but devastating. They must be investigated in case of positive familial history. Cardiac valvulopathies will be searched for by echocardiography. Abdominal wall herniae are often identified during the physical exam in ADPKD patients...
February 22, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28711597/micrornas-and-extracellular-vesicles-in-cholangiopathies
#11
REVIEW
P Olaizola, P Y Lee-Law, A Arbelaiz, A Lapitz, M J Perugorria, L Bujanda, J M Banales
Cholangiopathies encompass a heterogeneous group of disorders affecting biliary epithelial cells (i.e. cholangiocytes). Early diagnosis, prognosis and treatment still remain clinically challenging for most of these diseases and are critical for adequate patient care. In the past decade, extensive research has emphasized microRNAs (miRs) as potential non-invasive biomarkers and tools to accurately identify, predict and treat cholangiopathies. MiRs can be released extracellularly conjugated with lipoproteins or encapsulated in extracellular vesicles (EVs)...
July 12, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28705567/%C3%A2-%C3%A2-le-service-de-p%C3%A3-diatrie-%C3%A3-tait-notre-deuxi%C3%A3-me-maison%C3%A2-%C3%A2
#12
Emmanuelle Barsky
"THE PAEDIATRICS DEPARTMENT WAS OUR SECOND HOME".: At the age of a few months, Yakub was diagnosed with congenital liver fibrosis then polycystic kidney disease. He has been monitored regularly since then, and has had to be readmitted on several occasions due to complications. Now, aged 15, he is waiting for a liver transplant and is under the care of the adult hepatology department. The transition from paediatrics to the adult services was prepared, in advance, with him and his family. Yakub and his mother's account...
July 2017: Soins. Pédiatrie, Puériculture
https://www.readbyqxmd.com/read/28705172/endocannabinoid-receptor-blockade-reduces-alanine-aminotransferase-in-polycystic-ovary-syndrome-independent-of-weight-loss
#13
Alison J Dawson, Eric S Kilpatrick, Anne-Marie Coady, Abeer M M Elshewehy, Youssra Dakroury, Lina Ahmed, Stephen L Atkin, Thozhukat Sathyapalan
BACKGROUND: Evidence suggests that endocannabinoid system activation through the cannabinoid receptor 1 (CB1) is associated with enhanced liver injury, and CB1 antagonism may be beneficial. The aim of this study was to determine the impact of rimonabant (CB1 antagonist) on alanine aminotransferase (ALT), a hepatocellular injury marker, and a hepatic inflammatory cytokine profile. METHODS: Post hoc review of 2 studies involving 50 obese women with PCOS and well matched for weight, randomised to weight reducing therapy; rimonabant (20 mg od) or orlistat (120 mg tds), or to insulin sensitising therapy metformin, (500 mg tds), or pioglitazone (45 mg od)...
July 14, 2017: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/28694246/management-of-endocrine-disease-polycystic-ovary-syndrome-and-nonalcoholic-fatty-liver-disease
#14
REVIEW
Djuro Macut, Ivana Božić-Antić, Jelica Bjekić-Macut, Konstantinos Tziomalos
Polycystic ovary syndrome (PCOS) is a frequent endocrine disease in women, with a number of metabolic and reproductive consequences. Obesity, insulin resistance (IR) and type 2 diabetes are prominent metabolic characteristics of PCOS and common factors affecting liver function and generating nonalcoholic fatty liver disease (NAFLD). Multiple genes involved in the synthesis of androgens, cytokines and IR, as well as acquired factors, such as endocrine disruptors, could associate the etiopathogenesis of PCOS and NAFLD...
September 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28682033/-adpkd-and-heart
#15
Francesca Liut, Claudia Izzi, Nadia Dallera, Francesco Scolari
Autosomal Polycystic Kidney Disease ( ADPKD) is the most common inherited renal disease. ADPKD is caused by mutations in PKD1 and PKD2, encoding polycystin 1 and 2, respectively. ADPKD is a systemic disease, with renal and extrarenal involvement. Renal disease is characterized by formation and growth of cysts, with progressive destruction of renal parenchyma and development of End Stage Renal Disease (ESRD) in about 50% of affected individuals at the age of 60 years. Extrarenal disease usually involves the liver, heart and vasculature...
March 2017: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/28681988/effect-of-liraglutide-on-ectopic-fat-in-polycystic-ovary-syndrome-a-randomized-clinical-trial
#16
Signe Frøssing, Malin Nylander, Elizaveta Chabanova, Jan Frystyk, Jens Juul Holst, Caroline Kistorp, Sven O Skouby, Jens Faber
Women with polycystic ovary syndrome (PCOS) were treated with the GLP-1 receptor agonist liraglutide to investigate the effect on liver fat content, visceral adipose tissue (VAT), and the prevalence of nonalcoholic fatty liver disease (NAFLD). In a double-blind, placebo-controlled, randomized clinical trial 72 women with PCOS and BMI >25 kg/m(2) and/or insulin resistance were treated with liraglutide or placebo 1.8 mg/day (2:1) for 26 weeks. Liver fat content was assessed by (1) HMR spectroscopy, VAT by MRI, body composition by DXA, and glucose metabolism by oral glucose tolerance test...
July 6, 2017: Diabetes, Obesity & Metabolism
https://www.readbyqxmd.com/read/28669735/prenatal-diagnosis-of-caroli-disease-associated-with-autosomal-recessive-polycystic-kidney-disease-by-3-d-ultrasound-and-magnetic-resonance-imaging
#17
Pedro Teixeira Castro, Ana Paula Pinho Matos, Heron Werner, Pedro Daltro, Tatiana Fazecas, Renata Nogueira, Edward Araujo Júnior
BACKGROUND: Caroli disease is a very rare congenital anomaly characterized by non-obstructive saccular or fusiform dilatation of the intrahepatic bile ducts. It is associated with bile stagnation and hepatolithiasis, which explain the recurrent cholangitis and portal hypertension as a consequence of congenital liver fibrosis. Although there are several reports of diagnosis in childhood and adult life, the prenatal diagnosis using conventional 2-D ultrasound is rare, with few reports in the literature...
June 29, 2017: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
https://www.readbyqxmd.com/read/28664236/alterations-in-biomechanical-properties-of-the-cornea-among-patients-with-polycystic-kidney-disease
#18
Kubra Serefoglu Cabuk, Bennur Esen, Kursat Atalay, Ahmet Kirgiz, Rukiye Aydin
PURPOSE: The aim of this study was to evaluate the corneal biomechanical features in polycystic kidney disease (PKD) patients and compare them with the healthy individuals. METHODS: Totally 81 patients with a mean age of 48.46 ± 14.51 years and 60 control cases with a mean age of 44.68 ± 12.69 years were included in the study. All of the subjects underwent a complete ophthalmological examination, including visual acuity testing, biomicroscopic anterior and posterior segment examinations...
June 29, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28650098/disparities-in-eurotransplant-liver-transplantation-waitlist-outcome-between-patients-with-and-without-exceptional-meld
#19
Andreas Umgelter, Alexander Hapfelmeier, Wouter Kopp, Marieke van Rosmalen, Xavier Rogiers, Markus Guba
BACKGROUND: The sickest-first principle in donor-liver allocation can be implemented by allocating organs to cirrhotic patients with the highest Model of End-stage Liver Disease (MELD) scores. For patients with other risk factors, standard (SE) and non-standard exceptions (NSE) have been developed. We investigated whether this system of matched MELD scores achieves similar outcome on the liver transplant waiting-list for various diagnostic groups in Eurotransplant (ET) countries with MELD-based individual allocation (Belgium, The Netherlands and Germany)...
June 26, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28638817/a-challenging-case-of-hepatoblastoma-concomitant-with-autosomal-recessive-polycystic-kidney-disease-and-caroli-syndrome-review-of-the-literature
#20
Nevil Kadakia, Steven J Lobritto, Nadia Ovchinsky, Helen E Remotti, Darrell J Yamashiro, Jean C Emond, Mercedes Martinez
We report a rare case of an 18-month-old female with autosomal recessive polycystic kidney disease, Caroli syndrome, and pure fetal type hepatoblastoma. The liver tumor was surgically resected with no chemotherapy given. Now 9 years post resection she demonstrates no local or distant recurrence and stable renal function.
2017: Frontiers in Pediatrics
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