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Polycystic liver

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https://www.readbyqxmd.com/read/29453607/semaphorin-7a-in-circulating-regulatory-t-cells-is-increased-in-autosomal-dominant-polycystic-kidney-disease-and-decreases-with-tolvaptan-treatment
#1
Yashang Lee, Katrina Lehmann Blount, Feng Dai, Siobhan Thompson, Jonathan Kaufman Scher, Sherrie Bitterman, Madeline Droher, Erica L Herzog, Gilbert Moeckel, Anil Karihaloo, Neera K Dahl
BACKGROUND: Semaphorin 7A (SEMA7A) is an immunomodulating protein implicated in lung and liver fibrosis. In autosomal-dominant polycystic kidney disease (ADPKD), the progressive expansion of renal cysts, inflammation, and subsequent renal fibrosis leads to end-stage renal disease (ESRD). SEMA7A may play a role in renal fibrosis and in ADPKD. METHODS: We evaluated Sema7a in a mouse model of renal fibrosis and determined the expression of SEMA7A in human ADPKD kidney...
February 16, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29435317/impact-of-liver-volume-on-polycystic-liver-disease-related-symptoms-and-quality-of-life
#2
Myrte K Neijenhuis, Wietske Kievit, Stef Mh Verheesen, Hedwig M D'Agnolo, Tom Jg Gevers, Joost Ph Drenth
Background: Symptoms in polycystic liver disease (PLD) are thought to be caused by compression of organs and structures by the enlarged liver. Aim: The aim of this article is to assess the impact of liver volume on symptoms and quality of life (QoL) in PLD. Methods: We included PLD patients from two prospective studies that used the PLD-questionnaire (PLD-Q) for symptom assessment. QoL was assessed through SF-36, summarized in a physical (PCS) and mental (MCS) component score...
February 2018: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/29432207/prevalence-of-comorbid-conditions-pre-existing-and-diagnosed-at-a-tertiary-care-pediatric-weight-management-clinic
#3
Griselle Leon, Elizabeth de Klerk, Josephine Ho, Michelle Jackman, Raylene A Reimer, Kimberly E Connors, Paola Luca
BACKGROUND: Childhood obesity places individuals at risk for a multitude of physical and mental health problems. The aim of this study was to assess the prevalence of obesity related comorbidities diagnosed prior to and after attending a tertiary care pediatric weight management clinic. METHODS: A cross sectional retrospective chart review of patients 2-17 years old seen in the weight management clinic at Alberta Children's Hospital from May 2012 to May 2014. RESULTS: A total of 199 patients were included in the review...
February 13, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29408736/diagnostic-evaluation-comorbidity-screening-and-treatment-of-polycystic-ovary-syndrome-in-adolescents-in-three-specialty-clinics
#4
Meghna R Sebastian, Constance M Wiemann, Fida Bacha, Sharonda J Alston-Taylor
STUDY OBJECTIVE: A polycystic ovary syndrome (PCOS) diagnosis in adolescence can have significant long-term health implications. The criteria for its diagnosis in adolescents have been subject to much debate. This study aimed to characterize the variability in diagnosis and management among different pediatric specialties. DESIGN, SETTINGS, PARTICIPANTS, AND INTERVENTIONS: This is a retrospective review of electronic medical records of females (11 - 21 years old) who presented to three specialties (adolescent medicine (AM), pediatric endocrinology (ENDO), and gynecology (GYN)), with a post-visit diagnosis of PCOS, menstrual disorders, or hirsutism, at a large tertiary care center, from November 1, 2011- October 31, 2012...
January 31, 2018: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/29406938/metabolic-syndrome-systems-thinking-in-heart-disease
#5
REVIEW
Ron Dommermuth, Kristine Ewing
Metabolic syndrome (MetS) is a cluster of cardiometabolic risk factors. MetS is associated with approximately 4-fold increase in the likelihood of developing type 2 diabetes mellitus (T2DM) and a 2-fold increase in the incidence of cardiovascular disease complications. MetS is a progressive, proinflammatory, prothrombotic condition that manifests itself along a broad spectrum of disease. It is associated with hypertension, obstructive sleep apnea, fatty liver disease, gout, and polycystic ovarian syndrome. Intervening in and reversing the pathologic process become more difficult as the disease progresses, highlighting the needs for increased individual and community surveillance and primary prevention...
March 2018: Primary Care
https://www.readbyqxmd.com/read/29395568/-pulmonary-granuloma-in-an-immunodepressed-patient
#6
F Gadby, L Quaesaet, C Eveilleau, A Payet-Charneau, F Couturaud, C Tromeur
INTRODUCTION: Pulmonary alveolar echinococcosis is a rare but potentially severe condition. CASE REPORT: We report the case of a 50-year-old woman suffering from pulmonary alveolar echinococcosis who had had a renal transplant for polycystic liver and kidney disease. A lung opacity was identified radiologically in May 2013. Both broncho-alveolar lavage and bronchial biopsy were uninformative. In January 2014, a follow up CT-scan showed the opacity to be enlarging...
January 27, 2018: Revue des Maladies Respiratoires
https://www.readbyqxmd.com/read/29379816/hepatopleural-fistula-with-empyema-thoracis-a-rare-complication-of-autosomal-dominant-polycystic-kidney-disease
#7
Jeerawat Maytapa, Kessarin Thanapirom, Sombat Treeprasertsuk, Piyawat Komolmit, Bundit Chaopathomkul, Pinit Kullavanijaya
We report a 70-year-old man with autosomal dominant polycystic kidney disease (ADPKD) who presented with right-sided extended-spectrum beta-lactamases Escherichia coli empyema thoracis. Chest and abdominal computed tomography showed hepatopleural fistula. The patient refused a surgical operation and was treated with tube thoracotomy, percutaneous drainage of dominant liver cyst, and intravenous antibiotics. His symptoms improved after 2 months of nonsurgical treatment.
2018: ACG Case Reports Journal
https://www.readbyqxmd.com/read/29366679/the-combination-of-an-hdac6-inhibitor-and-a-somatostatin-receptor-agonist-synergistically-reduces-hepato-renal-cystogenesis-in-an-animal-model-of-polycystic-liver-disease
#8
Maria Lorenzo Pisarello, Tatyana V Masyuk, Sergio A Gradilone, Anatoliy I Masyuk, Jingyi Francess Ding, Pui-Yuen Lee, Nicholas F LaRusso
Hepatic cystogenesis in polycystic liver disease (PLD) is associated with abnormalities in multiple cellular processes, including elevated cAMP and over-expression of histone deacetylase 6 (HDAC6). Disease progression in PCK rats (an animal model of PLD) is attenuated by inhibition of either cAMP production or HDAC6. Therefore, we hypothesized that concurrent targeting of HDAC6 and cAMP would synergistically reduce cyst growth. Changes in hepato-renal cystogenesis were examined in PCK rats treated with: i) a pan-HDAC inhibitor, panobinostat; ii) three specific HDAC6 inhibitors, ACY-1215, ACY-738, and ACY-241; and iii) a combination of ACY-1215 and the somatostatin receptor analog, pasireotide...
January 20, 2018: American Journal of Pathology
https://www.readbyqxmd.com/read/29351485/insulin-signaling-displayed-a-differential-tissue-specific-response-to-low-dose-dht-in-female-mice
#9
Stanley Andrisse, Katelyn Billings, Ping Xue, Sheng Wu
Hyperandrogenemia and hyperinsulinemia are believed to play prominent roles in polycystic ovarian syndrome (PCOS). We explored the effects of low dose dihydrotestosterone (DHT), a PCOS model, on insulin signaling in metabolic and re-productive tissues in a female mouse model. Insulin resistance in the energy storage tissues is associated with type 2 diabetes. Insulin signaling in the ovaries and pituitary either directly or indirectly stimulates androgen production. Energy storage and reproductive tissues were isolated and molecular assays were performed...
December 19, 2017: American Journal of Physiology. Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29329621/first-parasitological-histopathological-and-molecular-characterization-of-echinococcus-vogeli-rausch-and-bernstein-1972-from-cuniculus-paca-linnaeus-1766-in-the-cerrado-biome-mato-grosso-do-sul-brazil
#10
Fernanda Bittencourt-Oliveira, Paulo Teixeira, Alba Alencar, Rodrigo Menezes, Christiane Corrêa, Leandro Neves, Fernanda Almeida, Daniel Daipert-Garcia, José Roberto Machado-Silva, Rosângela Rodrigues-Silva
Polycystic echinococcosis (PE) is caused by Echinococcus vogeli metacestodes (larval stage) in Neotropical countries. E. vogeli is trophically-transmitted between predators bush dogs (Speothos venaticus) and prey pacas (Cuniculus paca). In Brazil, reported PE cases are restricted to the Amazon biome. In this study, metacestodes from a paca hunted in Mato Grosso do Sul state (Cerrado biome) were identified morphological and histopathological techniques and further confirmed by molecular testing (sequencing of cytochrome C oxidase subunit I (cox1) gene) for the first time...
January 30, 2018: Veterinary Parasitology
https://www.readbyqxmd.com/read/29322327/combined-and-sequential-liver-kidney-transplantation-in-children
#11
Ryszard Grenda, Piotr Kaliciński
Combined and sequential liver-kidney transplantation (CLKT and SLKT) is a definitive treatment in children with end-stage organ failure. There are two major indications: - terminal insufficiency of both organs, or - need for transplanting new liver as a source of lacking enzyme or specific regulator of the immune system in a patient with renal failure. A third (uncommon) option is secondary end-stage renal failure in liver transplant recipients. These three clinical settings use distinct qualification algorithms...
January 10, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29322175/association-of-psoriasis-with-comorbidity-development-in-children-with-psoriasis
#12
Megha M Tollefson, Holly K Van Houten, Dennis Asante, Xiaoxi Yao, Hilal Maradit Kremers
Importance: Children with psoriasis are at increased risk for comorbidities. Many children with psoriasis are also overweight or obese; it is unknown whether the increased risk of comorbidities in these children is independent of obesity. Objective: To determine the risk of elevated lipid levels (hyperlipidemia/hypertriglyceridemia), hypertension, metabolic syndrome, polycystic ovarian syndrome, diabetes, nonalcoholic liver disease, and elevated liver enzyme levels in children with and without psoriasis, after accounting for obesity...
January 10, 2018: JAMA Dermatology
https://www.readbyqxmd.com/read/29321346/mutational-screening-of-pkd2-gene-in-the-north-indian-polycystic-kidney-disease-patients-revealed-28-genetic-variations
#13
Sonam Raj, Rana Gopal Singh, Parimal Das
Polycystic kidney disease (PKD) is a systemic disorder which adds majority of renal patients to end stage renal disease. Autosomal dominant polycystic kidney disease (ADPKD) is more prevalent and leading cause of dialysis and kidney transplant. Linkage analysis revealed some closely linked loci, two of which are identified as PKD1, PKD2 and an unidentified locus to ADPKD. This study was performed using PCR and automated DNA sequencing in 84 cases and 80 controls to test potential candidature of PKD2 as underlying cause of PKD by in silico and statistical analyses...
December 2017: Journal of Genetics
https://www.readbyqxmd.com/read/29318424/pasireotide-does-not-improve-efficacy-of-aspiration-sclerotherapy-in-patients-with-large-hepatic-cysts-a-randomized-controlled-trial
#14
Titus F M Wijnands, Tom J G Gevers, Marten A Lantinga, René H Te Morsche, Leo J Schultze Kool, Joost P H Drenth
OBJECTIVES: We tested whether complementary use of the somatostatin analogue pasireotide would augment efficacy of aspiration sclerotherapy of hepatic cysts. METHODS: We conducted a double-blind, placebo-controlled trial in patients who underwent aspiration sclerotherapy of a large (>5 cm) symptomatic hepatic cyst. Patients were randomized to either intramuscular injections of pasireotide 60 mg long-acting release (n = 17) or placebo (sodium chloride 0.9 %, n = 17)...
January 9, 2018: European Radiology
https://www.readbyqxmd.com/read/29307164/primary-disseminated-hydatid-cysts-in-a-14-year-old-girl-a-case-report
#15
Nazanin Fallah, Soghra Rabiee, Mohammad Fallah
Hydatid cysts in humans usually located in the liver or lungs primarily and some case in the other organs. Sometimes, because the trauma or medical manipulating, the cyst may be rupture and protoscoleces spillage resulted in secondary hydatid cyst. Primary dissemination of cyst to multiple organs is a rare phenomenon. Herein, we reported a 14-year-old teenage female patient with symptoms of abdominal pain with an unusual ultrasonographic and computed tomographic presentation mimicking polycystic ovarian syndrome PCOS...
November 2017: Acta Medica Iranica
https://www.readbyqxmd.com/read/29279078/-isolated-polycystic-liver-disease
#16
Rita Seara Costa, Bruno Gonçalves, Ana Isabel Rebelo
No abstract text is available yet for this article.
November 29, 2017: Acta Médica Portuguesa
https://www.readbyqxmd.com/read/29243290/a-non-coding-variant-in-ganab-explains-isolated-polycystic-liver-disease-pcld-in-a-large-family
#17
Whitney Besse, Jungmin Choi, Dina Ahram, Shrikant Mane, Simone Sanna-Cherchi, Vicente Torres, Stefan Somlo
Expanded mutation detection and novel gene discovery for isolated polycystic liver disease (PCLD) are necessary as 50% of cases do not have identified mutations in the seven published disease genes. We investigated a family with 5 affected siblings for which no loss of function variants were identified by whole exome sequencing analysis. SNP genotyping and linkage analysis narrowed the candidate regions to ∼8% of the genome, which included two published PCLD genes in close proximity to each other, GANAB and LRP5...
December 15, 2017: Human Mutation
https://www.readbyqxmd.com/read/29224111/prenatal-testosterone-programming-of-insulin-resistance-in-the%C3%A2-female-sheep
#18
Muraly Puttabyatappa, Vasantha Padmanabhan
Insulin resistance, a common feature of metabolic disorders such as obesity, nonalcoholic fatty liver disease, metabolic syndrome, and polycystic ovary syndrome, is a risk factor for development of diabetes. Because sex hormones orchestrate the establishment of sex-specific behavioral, reproductive, and metabolic differences, a role for them in the developmental origin of insulin resistance is also to be expected. Female sheep exposed to male levels of testosterone during fetal life serve as an excellent translational model for delineating programming of insulin resistance...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29211938/polycystic-liver-disease-the-interplay-of-genes-causative-for-hepatic-and-renal-cystogenesis
#19
Tatyana V Masyuk, Anatoliy I Masyuk, Nicholas F LaRusso
No abstract text is available yet for this article.
December 6, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29209995/early-clinical-expressions-of-insulin-resistance-the-real-enemy-to-look-for
#20
René Rodríguez-Gutiérrez, Alejandro Salcido-Montenegro, José Gerardo González-González
The type 2 diabetes mellitus epidemic threatens public healthcare systems worldwide. Efforts to prevent chronic complications of diabetes and reduce their associated mortality have been ineffective. Hence, early prevention of type 2 diabetes mellitus and cardiovascular disease needs to be prioritized. This strategy, however, must be centered not on an approach based on hyperglycemia but on early pathophysiologic mechanisms, such as insulin resistance. Non-alcoholic fatty liver disease, androgenic alopecia, acanthosis nigricans, and polycystic ovarian syndrome are all well-accepted early clinical manifestations of insulin resistance that represent, in themselves, a risk for further development of type 2 diabetes and that appear years before hyperglycemia...
December 5, 2017: Diabetes Therapy: Research, Treatment and Education of Diabetes and related Disorders
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