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Polycystic liver

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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
#1
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
https://www.readbyqxmd.com/read/28615245/the-regulatory-1%C3%AE-subunit-of-protein-kinase-a-modulates-renal-cystogenesis
#2
Hong Ye, Xiaofang Wang, Megan M Constans, Caroline R Sussman, Fouad Chebib, Maria V Irazabal, William F Young, Peter C Harris, Lawrence S Kirschner, Vicente E Torres
The failure of the polycystins (PCs) to function in primary cilia is thought to be responsible for Autosomal Dominant Polycystic Kidney Disease (ADPKD). Primary cilia integrate multiple cellular signaling pathways, including calcium, cAMP, Wnt, and Hedgehog, which control cell proliferation and differentiation. It has been proposed that mutated PCs result in reduced intracellular calcium, which in turn upregulates cAMP, protein kinase A (PKA) signaling, and subsequently other proliferative signaling pathways...
June 14, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28612285/non-alcoholic-fatty-liver-disease-in-women-with-polycystic-ovary-syndrome-systematic-review-and-meta-analysis
#3
REVIEW
A L L Rocha, L C Faria, T C M Guimarães, G V Moreira, A L Cândido, C A Couto, F M Reis
PURPOSE: Non-alcoholic fatty liver disease (NAFLD) is an insidious pathologic condition that can manifest from simple steatosis to steatohepatitis (NASH) with potential progression to cirrhosis. Like the polycystic ovary syndrome (PCOS), NAFLD is associated with obesity, diabetes mellitus, insulin resistance and metabolic syndrome. PCOS women have an increased risk of NAFLD, but it is debatable which features of PCOS, either specific (androgen excess) or unspecific (metabolic derangements) affect the NAFLD risk...
June 13, 2017: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/28611971/diagnosis-and-management-of-hepatobiliary-complications-in-autosomal-recessive-polycystic-kidney-disease
#4
REVIEW
Andrew Wehrman, Alyssa Kriegermeier, Jessica Wen
Autosomal recessive polycystic kidney disease (ARPKD) is a congenital hepatorenal fibrocystic disease. The hepatic manifestations of ARPKD can range from asymptomatic to portal hypertension and massively dilated biliary system that results in liver transplantation. Hepatic complications of ARPKD typically present with signs of portal hypertension (splenomegaly and thrombocytopenia) or cholangitis. Liver disease in ARPKD does not always correlate with severity of renal disease. Management of ARPKD-related liver disease is largely treating specific symptoms, such as antibiotics for cholangitis or endoscopic treatment for variceal bleeding...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28611533/functional-budd-chiari-syndrome-associated-with-severe-polycystic-liver-disease
#5
Precil Diego Miranda de Menezes Neves, Bruno Eduardo Pedroso Balbo, Elieser Hitoshi Watanabe, Vinicius Rocha-Santos, Wellington Andraus, Luiz Augusto Carneiro D'Albuquerque, Luiz Fernando Onuchic
A 50-year-old woman with end-stage renal disease secondary to autosomal dominant polycystic kidney disease was referred to a quaternary care center due to significantly increased abdominal girth. Her physical examination revealed tense ascites and abdominal collateral veins. A 10-L paracentesis improved abdominal discomfort and disclosed a transudate, suggestive of portal hypertension. A computed tomographic scan revealed massive hepatomegaly caused by multiple cysts of variable sizes, distributed throughout all hepatic segments...
2017: Clinical Medicine Insights. Gastroenterology
https://www.readbyqxmd.com/read/28565035/clinical-experience-with-wbc-pet-ct-in-adpkd-patients-with-suspected-cyst-infection-a-prospective-case-series
#6
Hyunsuk Kim, Yun Kyu Oh, Hayne Cho Park, Seokwoo Park, Soojin Lee, Ho-Young Lee, Young-Hwan Hwang, Curie Ahn
AIMS: Cyst infection (CI) is a common problem in patients with autosomal dominant polycystic kidney disease (ADPKD). Localization is of great importance in CI. We describe the clinical experience with [18F] FDG-labeled white-blood cell (WBC) PET/CT in detecting CI in ADPKD. METHODS: 19 ADPKD patients (M:F = 7:12) suspected of having CI were enrolled in this prospective study. All underwent WBC-PET/CT and MRI or CT. The degree of their WBC accumulation was evaluated from the maximal standardized uptake value of cystic wall...
May 31, 2017: Nephrology
https://www.readbyqxmd.com/read/28560483/conditional-loss-of-hepatocellular-hedgehog-signaling-in-female-mice-leads-to-the-persistence-of-hepatic-steroidogenesis-androgenization-and-infertility
#7
Christiane Rennert, Franziska Eplinius, Ute Hofmann, Janina Johänning, Franziska Rolfs, Wolfgang Schmidt-Heck, Reinhardt Guthke, Rolf Gebhardt, Albert M Ricken, Madlen Matz-Soja
The Hedgehog signaling pathway is known to be involved in embryogenesis, tissue remodeling, and carcinogenesis. Because of its involvement in carcinogenesis, it seems an interesting target for cancer therapy. Indeed, Sonidegib, an approved inhibitor of the Hedgehog receptor Smoothened (Smo), is highly active against diverse carcinomas, but its use is also reported to be associated with several systemic side effects. Our former work in adult mice demonstrated hepatic Hedgehog signaling to play a key role in the insulin-like growth factor axis and lipid metabolism...
May 30, 2017: Archives of Toxicology
https://www.readbyqxmd.com/read/28559591/genetics-novel-causative-genes-for-polycystic-liver-disease
#8
María J Perugorria, Jesús M Banales
No abstract text is available yet for this article.
May 31, 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28555180/risk-factors-for-neurocognitive-functioning-in-children-with-autosomal-recessive-polycystic-kidney-disease
#9
REVIEW
Stephen R Hooper
This mini review provides an overview of the issues and challenges inherent in autosomal recessive polycystic kidney disease (ARPKD), with a particular focus on the neurological factors and neurocognitive functioning of this population. ARPKD typically is discovered at the end of pregnancy or during the neonatal developmental period and occurs in approximately 1 in 20,000 live births. During the neonatal period, there is a relatively high risk of death, with many infants dying from respiratory failure. As the child ages, they experience progressive kidney disease and become increasingly vulnerable to liver disease, with many individuals eventually requiring dual organ transplants...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28545401/the-effect-of-disease-severity-markers-on-quality-of-life-in-autosomal-dominant-polycystic-kidney-disease-a-systematic-review-meta-analysis-and-meta-regression
#10
Myrte K Neijenhuis, Wietske Kievit, Ronald D Perrone, Jeff A Sloan, Patricia Erwin, Mohammad Hassan Murad, Tom J G Gevers, Marie C Hogan, Joost P H Drenth
BACKGROUND: Little is known about determinants of quality of life (QoL) in autosomal dominant polycystic kidney disease (ADPKD). Recent studies suggest that QoL in ADPKD is determined by more factors than mere renal function. We investigated the effect of ADPKD on QoL and evaluated how Qol is affected by disease severity markers renal function, kidney volume and liver volume. METHODS: We performed a systematic review, meta-analysis and meta-regression analyses of cohort studies and randomized controlled trials investigating patient-reported QoL in adult patients with ADPKD not yet on dialysis...
May 25, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28543567/tgr5-contributes-to-hepatic-cystogenesis-in-rodents-with-polycystic-liver-diseases-via-camp-g%C3%AE-s-signaling
#11
Tatyana V Masyuk, Anatoliy I Masyuk, Maria Lorenzo Pisarello, Brynn N Howard, Bing Q Huang, Pui-Yuen Lee, Xavier Fung, Eduard Sergienko, Robert J Ardesky, Thomas Dy Chung, Anthony B Pinkerton, Nicholas F LaRusso
Hepatic cystogenesis in Polycystic Liver Disease (PLD) is associated with increased levels of cAMP in cholangiocytes lining liver cysts. TGR5, a G protein-coupled bile acid receptor, is linked to cAMP and expressed in cholangiocytes. Therefore, we hypothesized that TGR5 might contribute to disease progression. We examined expression of TGR5 and Gα proteins in cultured cholangiocytes and in livers of animal models and humans with PLD. In vitro, we assessed cholangiocyte proliferation, cAMP levels, and cyst growth in response to: (i) TGR5 agonists [taurolithocholic acid (TLCA), oleanolic acid (OA) and two synthetic compounds]; (ii) a novel TGR5 antagonist (SBI-115); and (iii) a combination of SBI-115 and pasireotide, a somatostatin receptor (SSTR) analog...
May 23, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28515418/automatic-segmentation-of-kidneys-using-deep-learning-for-total-kidney-volume-quantification-in-autosomal-dominant-polycystic-kidney-disease
#12
Kanishka Sharma, Christian Rupprecht, Anna Caroli, Maria Carolina Aparicio, Andrea Remuzzi, Maximilian Baust, Nassir Navab
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common inherited disorder of the kidneys. It is characterized by enlargement of the kidneys caused by progressive development of renal cysts, and thus assessment of total kidney volume (TKV) is crucial for studying disease progression in ADPKD. However, automatic segmentation of polycystic kidneys is a challenging task due to severe alteration in the morphology caused by non-uniform cyst formation and presence of adjacent liver cysts. In this study, an automated segmentation method based on deep learning has been proposed for TKV computation on computed tomography (CT) dataset of ADPKD patients exhibiting mild to moderate or severe renal insufficiency...
May 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28509136/primary-hepatic-leiomyosarcoma-in-a-patient-with-autosomal-dominant-polycystic-kidney-disease
#13
Takashi Iida, Tamaki Maeda, Yoshifumi Amari, Takatomi Yurugi, Yoshitane Tsukamoto, Fumitaka Nakajima
Primary hepatic leiomyosarcoma is an extremely rare tumor. The diagnosis is difficult, and its etiologic factors have not been clarified. A 63-year-old woman with numerous cysts in her kidneys and liver was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). Several members of her family also had ADPKD. She underwent treatment with tolvaptan to inhibit cyst growth and slow the decline in kidney function. Eight months after the start of the therapy, she was hospitalized with fatigue and fever of unknown origin...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508983/effect-of-tolvaptan-in-a-patient-with-autosomal-dominant-polycystic-kidney-disease-after-living-donor-liver-transplantation
#14
Kiyotaka Uchiyama, Kazuya Honda, Ryochi Yoshida, Yuka Kamijo, Mai Yanagi, Mineo Nakatsuka, Yoshitaka Ishibashi
Recently, a large randomized placebo-controlled trial indicated a beneficial effect of tolvaptan on the progression of autosomal dominant polycystic kidney disease (ADPKD) with near-normal kidney function. Meanwhile, the evidence of tolvaptan's efficacy in ADPKD with severe renal insufficiency was limited and higher frequency of liver enzyme elevations were observed in patients taking tolvaptan. Liver transplantation (LT) is the only curative treatment for patients with severe polycystic liver disease associated with ADPKD, but considering that liver injuries should be avoided particularly in patients who underwent LT, we must be careful to start tolvaptan in post-LT ADPKD patients...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28487551/high-free-androgen-index-is-associated-with-increased-risk-of-non-alcoholic-fatty-liver-disease-in-women-with-polycystic-ovary-syndrome-independently-of-obesity-and-insulin-resistance
#15
C Jie, W Chunhua, Z Yi, W Yuying, X Wendi, L Tzuchun, L Shengxian, W Lihua, Z Jun, S Yun, L Wei, T Tao
BACKGROUND/OBJECTIVECentral obesity and insulin resistance are common conditions in women with PCOS and in subjects with non-alcoholic fatty liver disease (NAFLD). However, few studies have addressed the association between hyperandrogenism (HA) and NAFLD. We aimed to determine whether variations in the free androgen index (FAI) might be associated with NAFLD prevalence.SUBJECTS/METHODSA cross-sectional study was performed including 400 Chinese women with PCOS and 100 age and body mass index (BMI)-matched women...
May 10, 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/28465378/biliary-tract-and-liver-complications-in-polycystic-kidney-disease
#16
Parminder K Judge, Charlie H S Harper, Benjamin C Storey, Richard Haynes, Martin J Wilcock, Natalie Staplin, Raph Goldacre, Colin Baigent, Jane Collier, Michael Goldacre, Martin J Landray, Christopher G Winearls, William G Herrington
Polycystic liver disease is a well described manifestation of autosomal dominant polycystic kidney disease (ADPKD). Biliary tract complications are less well recognized. We report a 50-year single-center experience of 1007 patients, which raised a hypothesis that ADPKD is associated with biliary tract disease. We tested this hypothesis using all England Hospital Episode Statistics data (1998-2012), within which we identified 23,454 people with ADPKD and 6,412,754 hospital controls. Hospitalization rates for biliary tract disease, serious liver complications, and a range of other known ADPKD manifestations were adjusted for potential confounders...
May 2, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28460625/effect-of-statin-therapy-on-the-progression-of-autosomal-dominant-polycystic-kidney-disease-a-secondary-analysis-of-the-halt-pkd-trials
#17
Godela Brosnahan, Kaleab Z Abebe, Frederic F Rahbari-Oskoui, Charity G Patterson, Kyongtae T Bae, Robert W Schrier, William E Braun, Arlene B Chapman, Michael F Flessner, Peter C Harris, Ronald D Perrone, Theodore I Steinman, Vicente E Torres, The Halt Pkd Investigators
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) commonly results in end-stage renal disease (ESRD), yet a long-term treatment that is well tolerated is still lacking. In a small randomized trial in children and adolescents pravastatin administration for 3 years was associated with reduced renal cyst growth, but no large trial has tested the effect of statins in adults. METHODS: We performed a post-hoc analysis of the HALT PKD trials to compare outcomes of participants who never used statins with those who used statin for at least 3 years...
April 27, 2017: Current Hypertension Reviews
https://www.readbyqxmd.com/read/28457031/prepubertal-childhood-onset-type-2-diabetes-mellitus-four-case-reports
#18
Anandakumar Amutha, Ranjit Unnikrishnan, Ranjit Mohan Anjana, Viswanathan Mohan
BACKGROUND: The prevalence of childhood onset type 2 diabetes (T2D) is increasing, but prepubertal T2D is still unusual. METHODS: We report four cases of T2D with onset at or below 10 years of age registered at a tertiary diabetes centre in southern India.T2D was diagnosed based on the absence of ketosis, good beta cell reserve as shown by the C peptide assay, absence of GAD antibodies and pancreatic calculi, and response to oral hypoglycemic agents. RESULTS: All four patients were female, obese and had acanthosis nigricans...
February 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28416225/b-type-natriuretic-peptide-overexpression-ameliorates-hepatorenal-fibrocystic-disease-in%C3%A2-a%C3%A2-rat%C3%A2-model-of-polycystic-kidney-disease
#19
Sara J Holditch, Claire A Schreiber, Peter C Harris, Nicholas F LaRusso, Marina Ramirez-Alvarado, Alessandro Cataliotti, Vicente E Torres, Yasuhiro Ikeda
Polycystic kidney disease (PKD) involves progressive hepatorenal cyst expansion and fibrosis, frequently leading to end-stage renal disease. Increased vasopressin and cAMP signaling, dysregulated calcium homeostasis, and hypertension play major roles in PKD progression. The guanylyl cyclase A agonist, B-type natriuretic peptide (BNP), stimulates cGMP and shows anti-fibrotic, anti-hypertensive, and vasopressin-suppressive effects, potentially counteracting PKD pathogenesis. Here, we assessed the impacts of guanylyl cyclase A activation on PKD progression in a rat model of PKD...
April 14, 2017: Kidney International
https://www.readbyqxmd.com/read/28386465/transgastric-removal-of-a-polycystic-liver-disease-using-mini-laparoscopic-excision
#20
REVIEW
Fatih Sumer, Cuneyt Kayaalp, Yılmaz Polat, Ismail Ertugrul, Servet Karagul
Surgical treatment is often necessary for patients with symptomatic or complicated polycystic liver diseases (PLD). In this paper, we describe a 52-year-old female with symptomatic PLD that had resulted in the formation of liver cysts, the largest of which was 23 cm in diameter. The patient underwent mini-laparoscopic fenestration through 5-mm abdominal trocars. The walls of the cysts were unroofed using a harmonic scalpel. Four thickened rubber-like pieces of specimens (sizes ranged between 9 × 6 × 0...
June 1, 2016: Interventional Medicine & Applied Science
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