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https://www.readbyqxmd.com/read/28630289/green-mamba-peptide-targets-type-2-vasopressin-receptor-against-polycystic-kidney-disease
#1
Justyna Ciolek, Helen Reinfrank, Loïc Quinton, Say Viengchareun, Enrico A Stura, Laura Vera, Sabrina Sigismeau, Bernard Mouillac, Hélène Orcel, Steve Peigneur, Jan Tytgat, Laura Droctové, Fabrice Beau, Jerome Nevoux, Marc Lombès, Gilles Mourier, Edwin De Pauw, Denis Servent, Christiane Mendre, Ralph Witzgall, Nicolas Gilles
Polycystic kidney diseases (PKDs) are genetic disorders that can cause renal failure and death in children and adults. Lowering cAMP in cystic tissues through the inhibition of the type-2 vasopressin receptor (V2R) constitutes a validated strategy to reduce disease progression. We identified a peptide from green mamba venom that exhibits nanomolar affinity for the V2R without any activity on 155 other G-protein-coupled receptors or on 15 ionic channels. Mambaquaretin-1 is a full antagonist of the V2R activation pathways studied: cAMP production, beta-arrestin interaction, and MAP kinase activity...
June 19, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28623191/renal-capillary-haemangioma-associated-with-renal-cell-carcinoma-and-polycythaemia-in-acquired-cystic-disease
#2
Matthew Beamer, Matthew Love, Seyed Ghasemian
Capillary haemangiomas are relatively common tumours, typically occurring in the subcutaneous tissue during childhood. However, visceral occurrence is very rare. These tumours make up a subset of vascular lesions that have previously, although rarely, been described in case reports in association with the kidney. Here we review the literature and describe a capillary haemangioma occurring in the renal hilum found to be coexistent with end-stage renal disease, renal cell carcinoma and polycythaemia. To our knowledge, this is the first case report to describe the occurrence of this tumour in the renal hilum in association with this constitution of renal pathologies...
June 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28622163/novel-roles-for-mucin-1-in-the-kidney
#3
Mohammad M Al-Bataineh, Rebecca P Hughey
PURPOSE OF REVIEW: Recent studies in the kidney have revealed that the well characterized tumor antigen mucin 1 (MUC1/Muc1) also has numerous functions in the normal and injured kidney. RECENT FINDINGS: Mucin 1 is a transmembrane mucin with a robust glycan-dependent apical targeting signal and efficient recycling from endosomes. It was recently reported that the TRPV5 calcium channel is stabilized on the cell surface by galectin-dependent cross-linking to mucin 1, providing a novel mechanism for regulation of ion channels and normal electrolyte balance...
June 15, 2017: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/28618971/inversin-correlates-with-the-malignant-phenotype-of-non-small-cell-lung-cancer-and-promotes-the-invasiveness-of-lung-cancer-cells
#4
Gui-Yang Jiang, Yong Zhang, Xiu-Peng Zhang, Xu-Yong Lin, Juan-Han Yu, En-Hua Wang
Inversin, encoded by NPHP2, is one of the 10 NPHP proteins known to be involved in nephronophthisis (an autosomal recessive cystic kidney). Although the previous reports showed that inversin played an important role in embryonic development and renal diseases, its function in cancer was not revealed clearly so far. As measured by immunohistochemical staining, inversin was highly expressed in the cytoplasm of lung cancer samples (63.4%, 161/254) compared with adjacent normal lung tissues (22.0%, 11/50, p < 0...
June 2017: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
https://www.readbyqxmd.com/read/28615245/the-regulatory-1%C3%AE-subunit-of-protein-kinase-a-modulates-renal-cystogenesis
#5
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/28604386/lysine-methyltransferase-smyd2-promotes-cyst-growth-in-autosomal-dominant-polycystic-kidney-disease
#6
Linda Xiaoyan Li, Lucy X Fan, Julie Xia Zhou, Jared J Grantham, James P Calvet, Julien Sage, Xiaogang Li
Autosomal dominant polycystic kidney disease (ADPKD) is driven by mutations in PKD1 and PKD2 genes. Recent work suggests that epigenetic modulation of gene expression and protein function may play a role in ADPKD pathogenesis. In this study, we identified SMYD2, a SET and MYND domain protein with lysine methyltransferase activity, as a regulator of renal cyst growth. SMYD2 was upregulated in renal epithelial cells and tissues from Pkd1-knockout mice as well as in ADPKD patients. SMYD2 deficiency delayed renal cyst growth in postnatal kidneys from Pkd1 mutant mice...
June 12, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28591983/-clinical-and-pathologic-analysis-of-414-cases-of-renal-angiomyolipomain-in-a-single-institution
#7
H L Bao, X Chen, Y X An, H B Sun, H Y Wang, A T Guo
Objective: To study the different clinicopathological characteristics between classic and epithelioid renal angiomyolipoma, and the relationships between clinicopathological characteristics and biological behaviors as basis for clinical treatment. Methods: The clinicopathological and follow-up data for the patients diagnosed with renal angiomyolipoma between 2004 and 2011 were retrospectively reviewed and analyzed. Results: There were 414 cases of renal angiomyolipoma diagnosed over 8 years ago, accounting for 8...
June 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28579614/cystic-kidney-disease-a-new-osmoregulatory-role-for-hnf-1%C3%AE
#8
Andrea Aguilar
No abstract text is available yet for this article.
June 5, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28577739/mitochondrial-cytopathies-and-the-kidney
#9
REVIEW
Francesco Emma, Leonardo Salviati
Mitochondrial cytopathies include a heterogeneous group of diseases that are characterized by impaired oxidative phosphorylation. Current evidence suggests that renal involvement is probably more frequent than originally suspected but remains subclinical in a significant number of patients or is underestimated due to the severity of other clinical manifestations. Until recently, these diseases were thought to develop primarily in pediatric patients but patients that become symptomatic only in adulthood are now well recognized...
April 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28575888/renal-involvement-in-2-siblings-with-cockayne-syndrome
#10
Amel Ben Chehida, Narjess Ghali, Rim Ben Abdelaziz, Fatma Ben Moussa, Neji Tebib
Renal involvement in Cockayne syndrome is rare and its pathogenesis is yet unknown. We report herein 2 cases (siblings) with Cockayne syndrome type A confirmed by biochemical and molecular assays. The first case was a 13-year-old girl who presented with nephritic syndrome and a rapidly progressive kidney failure. Her younger sister, 7 years old, exhibited hypertension, hyperfiltration, and microalbuminuria. She had hyperreninemia and hyperaldosteronemia without kidney failure or renal arterial stenosis. Renal biopsy, performed the older sister, revealed cystic focal segmental glomerulosclerosis, arteriosclerosis, tubulointerstitial fibrosis, and tubular atrophy...
May 2017: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/28565035/clinical-experience-with-wbc-pet-ct-in-adpkd-patients-with-suspected-cyst-infection-a-prospective-case-series
#11
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/28552751/-ultrasound-of-the-fetal-urinary-system-during-the-first-trimester-of-pregnancy
#12
N Hamdaoui, A Dabadie, E Lesieur, E Quarello, M Kheiri, G Hery, B Guidicelli, F Bretelle, G Gorincour
The detection of abnormalities of the fetal urinary system in the first trimester of pregnancy is constantly improving, namely owing to the improved resolution of the image, the use of the endovaginal approach and thanks to sonographers' constant training. The pathological aspects, usually detected in the second trimester of pregnancy, can be suspected early in the first trimester and range from kidneys' cavity dilation to bilateral renal agenesis, polycystic kidney disease, multi-cystic dysplasia and bladder megavessia or bladder exstrophy...
May 25, 2017: Gynecologie, Obstetrique, Fertilite & Senologie
https://www.readbyqxmd.com/read/28546813/a-case-of-urachal-carcinoma-of-the-abdominal-wall-in-a-kidney-transplant-recipient
#13
Takuya Yamazaki, Yasushi Nagaba, Yoshitaka Shimada, Yoshinori Taoka, Satoru Minamida, Dai Koguchi, Masahiro Hagiwara, Sho Watanuki, Hide Nagaba, Kazunari Yoshida, Yasuo Takeuchi
Urachal carcinoma is an extremely rare malignant tumor arising from the urachus in the fetus. We report a patient who developed urachal carcinoma 18 years after kidney transplantation. A 59-year-old man was admitted because of abdominal pain and massive ascites. He had undergone kidney transplantation 18 years earlier and had end-stage renal disease requiring dialysis. Abdominal CT showed massive ascites and an abdominal wall cystic mass separated from the peritoneal cavity. Hemodialysis was started, and paralytic ileus was diagnosed and treated...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28545714/anticystogenic-activity-of-a-small-molecule-pak4-inhibitor-may-be-a-novel-treatment-for-autosomal-dominant-polycystic-kidney-disease
#14
Vicki J Hwang, Xia Zhou, Xiaonan Chen, Josephine Trott, Omran Abu Aboud, Kyuhwan Shim, Lai Kuan Dionne, Kenneth J Chmiel, William Senapedis, Erkan Baloglu, Moe R Mahjoub, Xiaogang Li, Robert H Weiss
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a common hereditary renal disease with no currently available targeted therapies. Based on the established connection between β-catenin signaling and renal ciliopathies, and on data from our and other laboratories showing striking similarities of this disease and cancer, we evaluated the use of an orally bioavailable small molecule, KPT-9274 (a dual inhibitor of the protein kinase PAK4 and nicotinamide phosphoribosyl transferase), for treatment of ADPKD...
May 23, 2017: Kidney International
https://www.readbyqxmd.com/read/28543609/understanding-the-school-experiences-of-children-and-adolescents-with-serious-chronic-illness-a-systematic-meta-review
#15
REVIEW
A Lum, C E Wakefield, B Donnan, M A Burns, J E Fardell, G M Marshall
BACKGROUND: Serious chronic illness can have a detrimental effect on school attendance, participation and engagement, leaving affected students at risk of failing to meet their developmental potential. An improved understanding of factors that help to explain or mitigate this risk can help educators and health professionals deliver the most effective support. This meta-review critiqued the available evidence examining the link between six chronic illnesses (asthma, cancer, chronic kidney diseases, heart diseases, cystic fibrosis and gastrointestinal diseases) and children's and adolescents' school experiences and outcomes, as well as investigating the medical, school, psychosocial and sociodemographic factors that are linked to poorer or better school outcomes...
May 23, 2017: Child: Care, Health and Development
https://www.readbyqxmd.com/read/28543535/diagnosis-and-management-of-acquired-cystic-kidney-disease-and-renal-tumors-in-esrd-patients
#16
Frederic Rahbari-Oskoui, William Charles O'Neill
The incidence of renal malignancies is markedly increased in end-stage renal disease and appears to be related to the development of acquired cystic kidney disease. Identification and evaluation of simple cysts, complex cysts, and solid masses in these patients rely on imaging studies, including ultrasonography, computed tomography, and magnetic resonance imaging, each of which has advantages and disadvantages. While there are no published data to support screening of ESRD patients, this seems appropriate in at least some patients, based on patient characteristics and the presence or absence of acquired cystic kidney disease...
May 21, 2017: Seminars in Dialysis
https://www.readbyqxmd.com/read/28540891/chronic-renal-failure-an-autopsy-study
#17
Anitha Padmanabhan, Sanjay Gohil, N M Gadgil, Prerna Sachdeva
Diabetes and hypertension are at present the major causes of chronic kidney disease (CKD) and end-stage renal disease (ESRD) worldwide. The stages 0-5 of CKD are defined according to the estimated glomerular filtration rate. The term chronic renal failure (CRF) typically corresponds to CKD stages 3-5. Cardiovascular disease is the main cause of morbidity and mortality in patients of CRF and ESRD. This study was undertaken to analyze the age and sex incidence, clinical features, etiology, pathology of various organs in detail, and causes of death of CRF patients...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28540625/pituitary-dysfunction-in-granulomatosis-with-polyangiitis
#18
REVIEW
Daniela Esposito, Penelope Trimpou, Dario Giugliano, Mats Dehlin, Oskar Ragnarsson
PURPOSE: Granulomatosis with polyangiitis (GPA) is a multisystem disease, characterized by necrotizing small-vessel vasculitis, which mainly affects the respiratory tract and the kidneys. Pituitary involvement in GPA is rare, present in about 1% of all cases of GPA. To date, only case reports or small case series have been published. Herein we report clinical features, imaging findings, treatment and outcomes in three patients with GPA-related pituitary dysfunction (PD). METHODS: A retrospective analysis of three cases of GPA-related PD was conducted, followed by systematic review of the English medical literature using PubMed...
May 24, 2017: Pituitary
https://www.readbyqxmd.com/read/28536669/disparate-presentations-of-localized-cystic-disease-of-kidney-a-review-with-an-objective-of-correct-approach-for-accurate-treatment-plan
#19
Sachin Khanduri, Mriganki Chaudhary, Tushar Sabharwal, Aakshit Goyal, Gaurav Katyal
BACKGROUND: Localized cystic disease of the kidney is a rare, non-familial condition. Its imaging and clinical features are unique and need to be differentiated from autosomal dominant polycystic kidney disease and focal cystic masses such as multicystic nephroma and cystic renal cell carcinoma. It is always restricted to one kidney and is characterized by multiple cysts of varying sizes separated by residual normal renal tissue. MATERIALS AND METHODS: This study reports 12 cases of localized cystic disease of the kidney based on imaging findings and clinical histories...
April 22, 2017: Curēus
https://www.readbyqxmd.com/read/28522687/integrin-linked-kinase-signaling-promotes-cyst-growth-and-fibrosis-in-polycystic-kidney-disease
#20
Archana Raman, Gail A Reif, Yuqiao Dai, Aditi Khanna, Xiaogang Li, Lindsay Astleford, Stephen C Parnell, James P Calvet, Darren P Wallace
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by innumerous fluid-filled cysts and progressive deterioration of renal function. Previously, we showed that periostin, a matricellular protein involved in tissue repair, is markedly overexpressed by cyst epithelial cells. Periostin promotes cell proliferation, cyst growth, interstitial fibrosis, and the decline in renal function in PKD mice. Here, we investigated the regulation of these processes by the integrin-linked kinase (ILK), a scaffold protein that links the extracellular matrix to the actin cytoskeleton and is stimulated by periostin...
May 18, 2017: Journal of the American Society of Nephrology: JASN
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