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https://www.readbyqxmd.com/read/28098112/autosomal-dominant-polycystic-kidney-disease-study-of-clinical-characteristics-in-an-indian-population
#1
Sanjay Vikrant, Anupam Parashar
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary form of kidney disease. Clinical data on this multisystem disorder are scarce from developing countries. We conducted a prospective observational study of the clinical profile of ADPKD patients at a single center over a period of six years. A total of 208 patients were studied. Majority were male (60.6%) and the mean age was 45.8 ± 14.5 years. About 61.5% had early stage (Stages 1-3) of chronic kidney disease (CKD) and 38.5% had advanced CKD (Stages 4 and 5)...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28093231/the-effects-of-6-gingerol-on-reproductive-improvement-liver-functioning-and-cyclooxygenase-2-gene-expression-in-estradiol-valerate-induced-polycystic-ovary-syndrome-in-wistar-rats
#2
Parisa Sadat Pournaderi, Parichehreh Yaghmaei, Hamidreza Khodaei, Zahra Noormohammadi, Seyed Hossein Hejazi
6- gingerol is the major pungent ingredient of ginger with anti-inflammatory and antioxidant properties. In this study, we evaluate the effects of 6- gingerol on the biochemical parameters and ovarian histological improvements in estradiol valerate (EV) induced PCOS rats. Thirty six female Wistar rats were divided into 4 groups: control, received normal diet, PCOS control, received 4 mg/kg EV injection for 28 days and two experimental groups, received an EV injection for 28 days and followed by 6- gingerol (200 μg/kg and 400 μg/kg) for 14 days...
January 13, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28088190/total-kidney-and-liver-volume-is-a-major-risk-factor-for-malnutrition-in-ambulatory-patients-with-autosomal-dominant-polycystic-kidney-disease
#3
Hyunjin Ryu, Hyosang Kim, Hayne Cho Park, Hyunsuk Kim, Eun Jin Cho, Kyu-Beck Lee, Wookyung Chung, Kook-Hwan Oh, Jeong Yeon Cho, Young-Hwan Hwang, Curie Ahn
BACKGROUND: In patients with autosomal dominant polycystic kidney disease (ADPKD), malnutrition may develop as renal function declines and the abdominal organs become enlarged. We investigated the relationship of intra-abdominal mass with nutritional status. METHODS: This cross-sectional study was performed at a tertiary hospital outpatient clinic. Anthropometric and laboratory data including serum creatinine, albumin, and cholesterol were collected, and kidney and liver volumes were measured...
January 14, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28052462/hepatobiliary-and-pancreatic-a-unique-case-of-hpv-related-metastatic-schneiderian-carcinoma-presenting-as-polycystic-liver-disease
#4
K R Bhamidimarri, D C Lanza, N D Kashikar
No abstract text is available yet for this article.
January 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28050373/histopathologic-findings-in-autopsies-with-emphasis-on-interesting-and-incidental-findings-a-pathologist-s-perspective
#5
Sapna Patel, B R Rajalakshmi, G V Manjunath
INTRODUCTION: Autopsy aids to the knowledge of pathology by unveiling the rare lesions which are a source of learning from a pathologist's perspective Some of them are only diagnosed at autopsy as they do not cause any functional derangement. This study emphasizes the various incidental lesions which otherwise would have been unnoticed during a person's life. AIM: The aim of this study was to determine the spectrum of histopathological findings including neoplastic lesions related or unrelated to the cause of death...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28024168/carbohydrate-antigen-19-9-is-significantly-elevated-in-autosomal-dominant-polycystic-kidney-disease
#6
Hirotaka Fukasawa, Mai Kaneko, Hiroki Niwa, Hideo Yasuda, Hiromichi Kumagai, Ryuichi Furuya
AIM: Liver cysts are the most common extrarenal manifestation in patients with autosomal dominant polycystic kidney disease (ADPKD). Carbohydrate antigen 19-9 (CA19-9) is generally used as a marker for biliopancreatic malignancies, although CA19-9 levels in patients with ADPKD are largely unknown. METHODS: A prospective observational study of 53 ADPKD patients and 83 non-ADPKD control subjects was performed. The serum levels of CA19-9 were studied to evaluate the association with clinical parameters and liver cysts...
December 26, 2016: Nephrology
https://www.readbyqxmd.com/read/28018103/portal-hypertension-in-polycystic-liver-disease-patients-does-not-affect-wait-list-or-immediate-post-liver-transplantation-outcomes
#7
Neil Rajoriya, Dhiraj Tripathi, Joanna A Leithead, Bridget K Gunson, Sophie Lord, James W Ferguson, Gideon M Hirschfield
AIM: To establish the impact of portal hypertension (PH) on wait-list/post-transplant outcomes in patients with polycystic liver disease (PCLD) listed for liver transplantation. METHODS: A retrospective single-centre case controlled study of consecutive patients listed for liver transplantation over 12 years was performed from our centre. PH in the PCLD cohort was defined by the one or more of following parameters: (1) presence of radiological or endoscopic documented varices from our own centre or the referral centre; (2) splenomegaly (> 11 cm) on radiology in absence of splenic cysts accounting for increased imaging size; (3) thrombocytopenia (platelets < 150 × 10(9)/L); or (4) ascites without radiological evidence of hepatic venous outflow obstruction from a single cyst...
December 7, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27994919/frequency-of-nerve-root-sleeve-cysts-in-autosomal-dominant-polycystic-kidney-disease
#8
Murat Aşık, Fatih Tufan, Timur Selçuk Akpınar, Nilgül Akalın, Elvan Ceyhan, Necmeddin Tunç, Zehra Işık Hasıloğlu, Mehmet Rıza Altıparmak, Tevfik Ecder, Sait Albayram
BACKGROUND: There is sporadic data about the occurrence of spinal meningeal cysts in patients with autosomal dominant polycystic kidney disease (ADPKD). We suggest that there is a relationship with the frequency and size of spinal meningeal cysts and headache, intracranial aneurysms, and cerebrospinal fluid leakage in patients with ADPKD. AIM: To investigate the relationship with spinal meningeal cyst, cerebrospinal fluid leakage, and headache in patients with ADPKD...
November 2016: Balkan Medical Journal
https://www.readbyqxmd.com/read/27967242/low-dose-dihydrotestosterone-drives-metabolic-dysfunction-via-cytosolic-and-nuclear-hepatic-androgen-receptor-mechanisms
#9
Stanley Andrisse, Shameka Childress, Yaping Ma, Katelyn Billings, Yi Chen, Ping Xue, Ashley Stewart, Momodou L Sonko, Andrew Wolfe, Sheng Wu
Androgen excess in women is associated with metabolic dysfunction (obesity, hyperinsulinemia, insulin resistance, and increased risk of type 2 diabetes (T2D)) and reproductive dysfunction (polycystic ovaries, amenorrhea, dysregulated gonadotropin release, and infertility). We sought to identify the effects of androgen excess on glucose metabolic dysfunction and the specific mechanisms of action by which androgens are inducing pathology. We developed a mouse model that displayed pathophysiological serum androgen levels with normal body mass/composition to ensure that the phenotypes were directly from androgens and not an indirect consequence of obesity...
December 14, 2016: Endocrinology
https://www.readbyqxmd.com/read/27956812/pediatric-living-donor-liver-transplantation-for-congenital-hepatic-fibrosis-using-a-mother-s-graft-with-von-meyenburg-complex-a-case-report
#10
Naoya Yamada, Yukihiro Sanada, Takumi Katano, Masahisa Tashiro, Yuta Hirata, Noriki Okada, Yoshiyuki Ihara, Atsushi Miki, Hideki Sasanuma, Taizen Urahashi, Yasunaru Sakuma, Koichi Mizuta
This is the first report of living donor liver transplantation (LDLT) for congenital hepatic fibrosis (CHF) using a mother's graft with von Meyenburg complex. A 6-year-old girl with CHF, who suffered from recurrent gastrointestinal bleeding, was referred to our hospital for liver transplantation. Her 38-year-old mother was investigated as a living donor and multiple biliary hamartoma were seen on her computed tomography and magnetic resonance imaging scan. The mother's liver function tests were normal and she did not have any organ abnormality, including polycystic kidney disease...
November 28, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27938556/-hereditary-polycystic-kidney-disease-a-neglected-etiology-of-liver-cirrhosis
#11
Y J Wu, H G Ding
There are two common types of hereditary polycystic kidney diseases, autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease. Congenital hepatic fibrosis is an autosomal recessive disorder and can occur in hereditary polycystic kidney disease. Therefore, hereditary polycystic kidney disease is one of the causes of unexplained liver fibrosis and liver cirrhosis.
October 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/27919367/familial-partial-lipodystrophy-presenting-as-metabolic-syndrome
#12
Darwin Chan, Adam D McIntyre, Robert A Hegele, Andrew C Don-Wauchope
We report the first described case of a heterozygous p.R545H (c.1634 G > A) missense mutation in the LMNA gene with clinical features compatible with Dunnigan-type 2 familial partial lipodystrophy (FPLD2). The case presented as metabolic syndrome to a specialist clinical service and highlights the overlap between FPLD2 and the metabolic syndrome. The associations with type 2 diabetes mellitus, fatty liver disease, polycystic ovarian syndrome, and hypertriglyceridemia are highlighted. The importance of evaluating patients for these associated conditions is discussed, and the potential mechanisms of disease are briefly outlined...
November 2016: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/27891514/evidence-for-a-pathogenic-triumvirate-in-congenital-hepatic-fibrosis-in-autosomal-recessive-polycystic-kidney-disease
#13
REVIEW
Lu Jiang, Pingping Fang, James L Weemhoff, Udayan Apte, Michele T Pritchard
Autosomal recessive polycystic kidney disease (ARPKD) is a severe monogenic disorder that occurs due to mutations in the PKHD1 gene. Congenital hepatic fibrosis (CHF) associated with ARPKD is characterized by the presence of hepatic cysts derived from dilated bile ducts and a robust, pericystic fibrosis. Cyst growth, due to cyst wall epithelial cell hyperproliferation and fluid secretion, is thought to be the driving force behind disease progression. Liver fibrosis is a wound healing response in which collagen accumulates in the liver due to an imbalance between extracellular matrix synthesis and degradation...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27862900/long-term-renal-allograft-survival-after-sequential-liver-kidney-transplantation-from-a-single-living-donor
#14
Kumiko Kitajima, Yuichi Ogawa, Katsuyuki Miki, Kotaro Kai, Akihito Sannomiya, Kazuhiro Iwadoh, Toru Murakami, Ichiro Koyama, Ichiro Nakajima, Shohei Fuchinoue
Background Combined liver-kidney transplantation (CLKT) is well-established as a definitive therapy with the potential to provide complete recovery for certain liver-kidney diseases, although the results might be contingent on the cause of transplantation. The purposes of the present study were to review the long-term outcome of renal allografts in CLKT patients from single living donors and to investigate the beneficial factors, compared with solitary renal transplantation. Materials and Methods Thirteen patients underwent sequential liver (LTx) and kidney (KTx) transplantations from single living donors...
November 10, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/27829402/intracystic-magnetic-resonance-imaging-in-patients-with-autosomal-dominant-polycystic-kidney-disease-features-of-severe-cyst-infection-in-a-case-control-study
#15
Tatsuya Suwabe, Yoshifumi Ubara, Toshiharu Ueno, Noriko Hayami, Junichi Hoshino, Aya Imafuku, Masahiro Kawada, Rikako Hiramatsu, Eiko Hasegawa, Naoki Sawa, Satoshi Saitoh, Itsuko Okuda, Kenmei Takaichi
BACKGROUND: The purpose of this study was to investigate the usefulness of intracystic MRI features for detection of severe cyst infection that is usually refractory to antibiotic therapy alone in patients with autosomal dominant polycystic kidney disease. METHODS: Seventy-six patients (88 episodes) with positive cyst cultures treated from January 2006 to December 2013 were enrolled as the cases for this case-control study, while 147 patients who continued to attend our hospital from January 2011 to December 2013 and did not have cyst infection diagnosed during that period were enrolled as the controls...
November 9, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27826057/adenylyl-cyclase-5-links-changes-in-calcium-homeostasis-to-camp-dependent-cyst-growth-in-polycystic-liver-disease
#16
Carlo Spirli, Valeria Mariotti, Ambra Villani, Luca Fabris, Romina Fiorotto, Mario Strazzabosco
BACKGROUND & AIMS: Genetic defects in polycystin-1 or -2 (PC1 or PC2) cause polycystic liver disease associated with autosomal dominant polycystic kidney disease (PLD-ADPKD). Progressive cyst growth is sustained by a cAMP-dependent Ras/ERK/HIFα pathway, leading to increased vascular endothelial growth factor A (VEGF-A) signaling. In PC2-defective cholangiocytes, cAMP production in response to [Ca(2+)]ER depletion is increased, while store-operated Ca(2+) entry (SOCE), intracellular and endoplasmic reticulum [Ca(2+)]ER levels are reduced...
November 5, 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/27823607/metformin-from-research-to-clinical-practice
#17
REVIEW
Meng H Tan, Hussain Alquraini, Kara Mizokami-Stout, Mark MacEachern
Metformin is the recommended first-line oral glucose-lowering drug initiated to control hyperglycemia in type 2 diabetes mellitus. It acts in the liver, small intestines, and skeletal muscles with its major effect on decreasing hepatic gluconeogenesis. It is safe, inexpensive, and weight neutral and can be associated with weight loss. It can reduce microvascular complication risk and its use is associated with a lower cardiovascular mortality compared with sulfonylurea therapy. It is also used to delay the onset of type 2 diabetes mellitus, in treating gestational diabetes, and in women with polycystic ovary syndrome...
December 2016: Endocrinology and Metabolism Clinics of North America
https://www.readbyqxmd.com/read/27804265/hepatic-steatosis-is-common-in-adolescents-with-obesity-and-pcos-and-relates-to-de-novo-lipogenesis-but-not-insulin-resistance
#18
Melanie Cree-Green, Bryan C Bergman, Gregory V Coe, Lindsey Newnes, Amy D Baumgartner, Samantha Bacon, Ann Sherzinger, Laura Pyle, Kristen J Nadeau
OBJECTIVE: Increased liver fat and type 2 diabetes are prevalent in women with polycystic ovarian syndrome (PCOS) and cause excess mortality, yet little is known about their development during adolescence. The objective of this study was to measure hepatic steatosis and related metabolic contributors in girls with obesity, with and without PCOS. METHODS: Nondiabetic adolescents with obesity, 41 with PCOS (PCOS; age 15.0 [13.0-16.0] years, BMI 35.2 ± 0.61 kg/m(2) ) and 30 without PCOS (OB; age 14...
November 2016: Obesity
https://www.readbyqxmd.com/read/27798284/assessment-of-growth-and-metabolism-characteristics-in-offspring-of-dehydroepiandrosterone-induced-polycystic-ovary-syndrome-adults
#19
Ying Huang, Jiang-Man Gao, Chun-Mei Zhang, Hong-Cui Zhao, Yue Zhao, Rong Li, Yang Yu, Jie Qiao
Polycystic ovary syndrome (PCOS) is a common reproductive disorder that has many characteristic features including hyperandrogenemia, insulin resistance and obesity, which may have significant implications for pregnancy outcomes and long-term health of women. Daughters born to PCOS mothers constitute a high-risk group for metabolic and reproductive derangements, but no report has described potential growth and metabolic risk factors for such female offspring. Hence, we used a mouse model of dehydroepiandrosterone (DHEA)-induced PCOS to study the mechanisms underlying the pathology of PCOS by investigating the growth, developmental characteristics, metabolic indexes and expression profiles of key genes of offspring born to the models...
December 2016: Reproduction: the Official Journal of the Society for the Study of Fertility
https://www.readbyqxmd.com/read/27796023/-clinical-manifestations-in-autosomal-dominant-polycystic-kidney-disease-adpkd
#20
Giovanni Piscopo, Giovanna Capolongo
ADPKD is a systemic disorder, associated with numerous extrarenal manifestations, including polycystic liver disease (PCLD) and other gastrointestinal manifestations, as well as pancreatic cysts, diverticular disease, inguinal and ventral hernias which play a significant role in disease burden, particularly in the advanced stage of ADPKD. In most cases the natural history of ADPKD goes through a long period of stability followed by a progressive decline in renal function. The coexistence of hypertension, cyst infections and nephrolithiasis can influence and accelerate the progression of kidney failure...
September 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
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