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cystic kidney disease

Luciane M Silva, Damon T Jacobs, Bailey A Allard, Timothy A Fields, Madhulika Sharma, Darren P Wallace, Pamela V Tran
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is caused by mutation of PKD1 or PKD2, which encode polycystin 1 and 2, respectively. The polycystins localize to primary cilia and the functional loss of the polycystin complex leads to the formation and progressive growth of fluid-filled cysts in the kidney. The pathogenesis of ADPKD is complex and molecular mechanisms connecting ciliary dysfunction to renal cystogenesis are unclear. Primary cilia mediate Hedgehog signaling, which modulates cell proliferation and differentiation in a tissue-dependent manner...
March 21, 2018: Scientific Reports
Jianjun Chang, Yan Ding, Zhiyu Zhou, Hong-Guang Nie, Hong-Long Ji
Transepithelial fluid and salt re-absorption in epithelial tissues play an important role in fluid and salt homeostasis. In absorptive epithelium, fluid and salt flux is controlled by machinery mainly composed of epithelial sodium channels (ENaC), cystic fibrosis transmembrane conductance regulator (CFTR), Na⁺/H⁺ exchanger (NHE), aquaporin, and sodium potassium adenosine triphosphatase (Na⁺/K⁺-ATPase). Dysregulation of fluid and salt transport across epithelium contributes to the pathogenesis of many diseases, such as pulmonary edema and cystic fibrosis...
March 16, 2018: International Journal of Molecular Sciences
Moataz M Hassan, Zied Gaifer, Ibrahim S Al-Zakwani
Background Colistin is used to treat gram-negative infections but it's highly associated with nephrotoxicity. Objectives To determine the incidence and risk factors as well as mortality in patients on colistin. Setting Sultan Qaboos University Hospital, Muscat, Oman. Methods This was a retrospective cohort study of patients admitted and who received colistin for ≥ 48 h. The exclusion criteria included inhaled colistin therapy, cystic fibrosis, or pregnancy. The study period was from January 2010 to June 2016...
March 14, 2018: International Journal of Clinical Pharmacy
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
Dechao Xu, Jiayi Lv, Liangliang He, Lili Fu, Ruikun Hu, Ying Cao, Changlin Mei
Polarity complexes, including the PAR (Partitioning-defective), CRB (Crumbs) and SCRIB (Scribble) complexes, are required for the physiologic establishment, stabilization, and maintenance of a functional apical-basolateral polarity. Inactivation of some of the polarity complexes results in cystic kidneys, and apical-basolateral polarity defects are commonly observed in autosomal-dominant polycystic kidney disease (ADPKD); however, little is known about the role that polarity complexes play in ADPKD. Here, we demonstrate that Scribble, a core protein of the SCRIB complex, is down-regulated in ADPKD cell lines and the zebrafish model of this disease ( pkd2 morphants)...
March 12, 2018: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
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
Ying Jing, Ming Wu, Di Zhang, Dongping Chen, Ming Yang, Shuqin Mei, Liangliang He, Junhui Gu, Na Qi, Lili Fu, Lin Li, Changlin Mei
OBJECTIVES: The aim of our current study was to investigate the long-term effect and the mechanism of triptolide in an adult non-orthologous rat model of polycystic kidney disease (PKD), and study the effect of triptolide on the mitogenic JAK2/STAT3 pathway.. METHODS: Male wild type (WT+/+) and Cy/+ cystic Han:SPRD rats were treated with vehicle or triptolide from week 44 weeks to week 16 weeks of age. Rats were sacrificed at 16 weeks of age 16 for the blood, urine and organ collection...
March 7, 2018: American Journal of Physiology. Renal Physiology
Erin E Olsan, Jonathan D West, Jacob A Torres, Nicholas Doerr, Thomas Weimbs
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a life-threatening, highly prevalent monogenic disease caused by mutations in polycystin-1 (PC1) in 85% of patients. We have previously identified a C-terminal cleavage fragment of PC1, PC1-p30, that interacts with the transcription factor STAT6 to promote transcription. STAT6 is aberrantly active in PKD mouse models and human ADPKD, and genetic removal or pharmacological inhibition of STAT6 attenuates disease progression. High levels of interleukin-13 (IL13), a STAT6 activating cytokine, are found in the cyst fluid of PKD mouse models and increased IL13 receptors in ADPKD patient tissue suggesting that a positive feedback loop between IL13 and STAT6 is activated in cystic epithelial cells and contributes to disease progression...
March 7, 2018: American Journal of Physiology. Renal Physiology
Qinghua Zhao, Jin Luo, Qin Zhang, Tianyan Leng, Lihua Yang
RATIONALE: Cystic echinococcosis (CE) is a parasitic zoonosis caused by echinococcus larvae. Manifestations of the disease include a severe damage to the liver and lung. Damages to the mesentery, omentum, spleen, brain, heart, bone, thyroid, kidney, and uterus are rarely observed. Moreover, primary ovarian and retroperitoneal hydatid disease is extremely rare, and is easily ignored or misdiagnosed. PATIENT CONCERNS: We present a case of CE in a 34-year-old female who presented with an adnexal mass detected by B-ultrasound...
January 2018: Medicine (Baltimore)
Jens Christian König, Andrea Titieni, Martin Konrad
Hereditary cystic kidney diseases comprise a complex group of genetic disorders representing one of the most common causes of end-stage renal failure in childhood. The main representatives are autosomal recessive polycystic kidney disease, nephronophthisis, Bardet-Biedl syndrome, and hepatocyte nuclear factor-1beta nephropathy. Within the last years, genetic efforts have brought tremendous progress for the molecular understanding of hereditary cystic kidney diseases identifying more than 70 genes. Yet, genetic heterogeneity, phenotypic variability, a lack of reliable genotype-phenotype correlations and the absence of disease-specific biomarkers remain major challenges for physicians treating children with cystic kidney diseases...
2018: Frontiers in Pediatrics
Mittul Gulati, Justin Cheng, Jerry T Loo, Matt Skalski, Harshawn Malhi, Vinay Duddalwar
Ultrasound (US) is the first-line imaging modality for evaluating azotemic patients for urinary obstruction and renal size. US is also valuable for distinguishing congenital variants and simple cystic lesions from renal masses. Doppler US is effective in detection of renal calculi and evaluation of vascular pathology. Unfortunately, renal US is limited in distinguishing causes of medical renal disease. The kidneys have a complex internal architecture with a highly variable appearance on US. This article illustrates non-neoplastic renal conditions, including normal and embryological variants, parenchymal, cystic, and vascular diseases...
February 16, 2018: Clinical Imaging
Vandana Dua Niyyar, W Charles O'Neill
Sonography is increasingly being performed by clinicians and has applications throughout the spectrum of nephrology, including acute and chronic renal failure, urinary obstruction, cystic disease, pain, hematuria, transplantation, kidney biopsy, temporary and permanent vascular access, and assessment of fluid status. The skill is relatively easily acquired, expedites patient care, and enhances the practice of nephrology. However, the lack of exposure in most training programs remains a major obstacle.
February 22, 2018: Kidney International
Yubin Shin, Do Yeon Kim, Je Yeong Ko, Yu Mi Woo, Jong Hoon Park
Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common inherited disorders. ADPKD is caused by mutations in the gene encoding either polycystic kidney disease 1 ( PKD1) or polycystic kidney disease 2 ( PKD2). Patients with ADPKD show progressive growth of cystic fluid-filled renal cysts. Here, we used Pkd2f/f control mice and Pkd2f/f :HoxB7-Cre experimental mice, which are bred to have a conditional deletion of Pkd2 in the collecting ducts, and analyzed the expression pattern of microRNAs (miRNAs) of kidney tissues from Pkd2f/f and Pkd2f/f :HoxB7-Cre mice...
February 16, 2018: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
Rashid A Barnawi, Rahaf Z Attar, Sultan S Alfaer, Osama Y Safdar
Autosomal dominant polycystic kidney disease (ADPKD) causes pathological cystic changes to the kidney and is characterized by numerous renal and systemic manifestations. ADPKD is the fourth most common renal disease requiring renal replacement therapy. In this report, we present a detailed review of ADPKD, with a particular focus on its major economic, psychological, and social burden in affected patients. Treatment of this disease has been based on prophylactic and supportive measures. However, in recent years, new drugs have emerged as promising agents that may retard the progression of ADPKD, such as tolvaptan...
2018: International Journal of Nephrology and Renovascular Disease
Oliver Oey, Padmashree Rao, Magdalena Luciuk, Carly Mannix, Natasha M Rogers, Priyanka Sagar, Annette Wong, Gopala Rangan
Dimethyl fumarate is an FDA-approved oral immunomodulatory drug with anti-inflammatory properties that induces the upregulation of the anti-oxidant transcription factor, nuclear factor erythroid-derived factor 2. The aim of this study was to determine the efficacy of dimethyl fumarate on interstitial inflammation and renal cyst growth in a preclinical model of nephronophthisis. Four-week-old female Lewis polycystic kidney disease (a genetic ortholog of human nephronophthisis-9) rats received vehicle (V), 10 mg/kg (D10) or 30 mg/kg (D30) ( n = 8-9 each) dimethyl fumarate in drinking water for eight weeks...
January 1, 2018: Experimental Biology and Medicine
Jia Hu, Lu Jin, Yifan Li, Tao He, Jiaju Liu, Bentao Shi, Shangqi Yang, Yaoting Gui, Xiangming Mao, Yongqing Lai, Liangchao Ni
Multilocular cystic renal cell carcinoma (MCRCC), which exhibits low-stage and low-grade characteristics, is a special type of RCC. MCRCC is extremely rare and generally develops at ages >50 years. We herein report a case of MCRCC in a 28-year-old man, which, to the best of our knowledge, is the youngest case reported worldwide to date. The patient presented with irritative bladder symptoms for 1 year. Dynamic enhanced computed tomography (CT) imaging revealed a mass with inhomogeneous enhancement in the left kidney, with a rich blood supply...
February 2018: Molecular and Clinical Oncology
Roman-Ulrich Müller, Christian S Haas, John A Sayer
Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease worldwide. The renal phenotype is characterized by progressive cystic enlargement of the kidneys leading to a decline in renal function, hypertension and often end-stage renal disease (ESRD). Supportive care with blood pressure control and management of pain, urinary infections and renal stone disease has, until recently, been the mainstay of treatment. With the recent approval of tolvaptan for use in ADPKD, the disease progression may now be targeted specifically...
February 2018: Clinical Kidney Journal
Asadur Rahman, Daisuke Yamazaki, Abu Sufiun, Kento Kitada, Hirofumi Hitomi, Daisuke Nakano, Akira Nishiyama
To date, good experimental animal models of renal anemia are not available. Therefore, the purpose of this study was to establish a novel approach to induce chronic kidney disease (CKD) with severe anemia by oral administration of adenine in rodents. Adenine was administered to 6-week-old male C57BL/6 mice (25 and 50 mg/kg body weight) by oral gavage daily for 28 days. Serum creatinine and BUN as well as hematocrit, hemoglobin (Hb) and plasma erythropoietin (EPO) levels were monitored to assess renal function and anemia, respectively...
2018: PloS One
Osamu Ichii, Teppei Nakamura, Takao Irie, Hirokazu Kouguchi, Kozue Sotozaki, Taro Horino, Yuji Sunden, Yaser Hosny Ali Elewa, Yasuhiro Kon
Cotton rat ( Sigmodon hispidus) is a useful experimental rodent for the study of human infectious diseases. We previously clarified that cotton rats, particularly females, developed chronic kidney disease characterized by cystic lesions, inflammation, and fibrosis. The present study investigated female-associated factors for chronic kidney disease development in cotton rats. Notably, female cotton rats developed separation of the pelvic symphysis and hypertrophy in the vaginal parts of the cervix with age, which strongly associated with pyometra...
January 1, 2018: Experimental Biology and Medicine
Anna S Nikonova, Alexander Y Deneka, Anna A Kiseleva, Vladislav Korobeynikov, Anna Gaponova, Ilya G Serebriiskii, Meghan C Kopp, Harvey H Hensley, Tamina N Seeger-Nukpezah, Stefan Somlo, David A Proia, Erica A Golemis
Autosomal-dominant polycystic kidney disease (ADPKD) is associated with progressive formation of renal cysts, kidney enlargement, hypertension, and typically end-stage renal disease. In ADPKD, inherited mutations disrupt function of the polycystins (encoded by PKD1 and PKD2), thus causing loss of a cyst-repressive signal emanating from the renal cilium. Genetic studies have suggested ciliary maintenance is essential for ADPKD pathogenesis. Heat shock protein 90 (HSP90) clients include multiple proteins linked to ciliary maintenance...
January 10, 2018: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
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