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Chronic kidney polycystic

Funda Sarı, Arzu Didem Yalçın, Gizem Esra Genç, Metin Sarıkaya, Atıl Bisgin, Ramazan Çetinkaya, Saadet Gümüşlü
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by multiple, large renal cysts and impaired kidney function. Although the reason for the development of kidney cysts is unknown, ADPKD is associated with cell cycle arrest and abundant apoptosis of renal tubular epithelial cells. AIMS: We asked whether serum-soluble TNF-related apoptosis-inducing ligand (sTRAIL) might underlie ADPKD. STUDY DESIGN: Case-control study...
September 2016: Balkan Medical Journal
Gnanasambandan Ramanathan, Santu Ghosh, Ramprasad Elumalai, Soundararajan Periyasamy, Bhaskar V K S Lakkakula
BACKGROUND & OBJECTIVES: Autosomal dominant polycystic kidney disease (ADPKD) is an inherited systemic disorder, characterized by the fluid filled cysts in the kidneys leading to end stage renal failure in later years of life. Hypertension is one of the major factors independently contributing to the chronic kidney disease (CKD) progression. The renin-angiotensin aldosterone system (RAAS) genes have been extensively studied as hypertension candidate genes. The aim of the present study was to investigate the role of angiotensin converting enzyme tagging - single nucleotide polymorphisms (ACE tag-SNPs) in progression of CKD in patients with ADPKD...
June 2016: Indian Journal of Medical Research
Funda Sari, Ayca Inci, Suleyman Dolu, Hamit Yasar Ellidag, Ramazan Cetinkaya, Fettah Fevzi Ersoy
This study aims to determine fibroblast growth factor-23 and soluble α-Klotho levels in patients with autosomal dominant polycystic kidney disease. A total of 76 patients with autosomal dominant polycystic kidney disease and 32 healthy volunteers were included in the study. Serum fibroblast growth factor-23 and soluble α-Klotho levels were measured with ELISA kits. Parathyroid hormone, phosphate, calcium, creatinine, 25-hydroxyvitamin D3 levels, urinary protein to creatinine ratio and estimated glomerular filtration rate were also measured or calculated...
October 12, 2016: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
Jason Misurac
Neonatal chronic kidney disease (CKD) occurs with an estimated incidence of 1 in 10,000 live births, whereas the incidence of neonatal end-stage renal disease (ESRD) is about 7.1 per million age-related population. The most frequent etiologies are renal hypoplasia/dysplasia, posterior urethral valves, and other congenital anomalies of the kidney and urinary tract. Other etiologies include polycystic kidney disease, cortical necrosis, and renal vascular thrombosis. Management of CKD focuses primarily on replacing renal functions such as erythropoietin, 1,25-hydroxylation of vitamin D, electrolyte homeostasis/excretion, and, in ESRD, waste product removal...
October 9, 2016: Seminars in Fetal & Neonatal Medicine
Olga Zegarra-Moran, Luis J V Galietta
CFTR protein is an ion channel regulated by cAMP-dependent phosphorylation and expressed in many types of epithelial cells. CFTR-mediated chloride and bicarbonate secretion play an important role in the respiratory and gastrointestinal systems. Pharmacological modulators of CFTR represent promising drugs for a variety of diseases. In particular, correctors and potentiators may restore the activity of CFTR in cystic fibrosis patients. Potentiators are also potentially useful to improve mucociliary clearance in patients with chronic obstructive pulmonary disease...
October 4, 2016: Cellular and Molecular Life Sciences: CMLS
Salahuddin Khan, Tariq Hussain, Najma Salahuddin, Salahuddin Mehreen
AIM: The basic aim of this study was to discover the association of End Stage Renal Disease (ESRD) with various risk factors. End Stage Renal Failure is the last stage of the chronic renal failure in which kidneys become completely fail to function. MATERIALS AND METHODS: The data were collected from the patients of renal diseases from three major hospitals in Peshawar, Pakistan. Odds ratio analysis was performed to examine the relationship of ESRD (a binary response variable) with various risk factors: Gender, Diabetic, Hypertension, Glomerulonephritis, Obstructive Nephropathy, Polycystic kidney disease, Myeloma, SLE Nephritis, Heredity, Hepatitis, Excess use of Drugs, heart problem and Anemia...
September 15, 2016: Open Access Macedonian Journal of Medical Sciences
Oscar Swift, Enric Vilar, Belinda Rahman, Lucy Side, Daniel P Gale
AIMS: No recommendations currently exist regarding implementation of both prenatal diagnosis and preimplantation genetic diagnosis (PGD) for autosomal dominant polycystic kidney disease (ADPKD). This study evaluated attitudes in ADPKD patients with either chronic kidney disease (CKD) stages I-IV or end-stage renal failure (ESRF) toward prenatal diagnosis and PGD. METHODS: Ninety-six ADPKD patients were recruited from an outpatient clinic, wards, and dialysis units...
September 30, 2016: Genetic Testing and Molecular Biomarkers
Fareed B Kamar, Bikaramjit Mann, Gregory Kline
BACKGROUND: Tumoral calcinosis is a rare manifestation of extraskeletal calcification, featuring large calcified cystic masses in the periarticular regions of large joints. In chronic kidney disease (CKD), this disorder is thought to evolve through a chronically elevated calcium-phosphorus solubility product leading to calcium precipitation in soft tissue. Treating tumoral calcinosis in these patients involves interventions to lower the calcium-phosphorus product such as reduction in vitamin D therapy and intensive hemodialysis regimens...
September 29, 2016: BMC Nephrology
Daniel Eriksson, Linda Karlsson, Oskar Eklund, Hans Dieperink, Eero Honkanen, Jan Melin, Kristian Selvig, Johan Lundberg
BACKGROUND: A limited number of studies have assessed health-related quality of life (HRQoL) in autosomal dominant polycystic kidney disease (ADPKD). Results to date have been conflicting and studies have generally focused on patients with later stages of the disease. This study aimed to assess HRQoL in ADPKD across all stages of the disease, from patients with early chronic kidney disease (CKD) to patients with end-stage renal disease. METHODS: A study involving cross-sectional patient-reported outcomes and retrospective clinical data was undertaken April-December 2014 in Denmark, Finland, Norway and Sweden...
September 23, 2016: Nephrology, Dialysis, Transplantation
Sheena Sharma, Jennifer M Kalish, Ethan M Goldberg, Francis Jeshira Reynoso, Madhura Pradhan
Background. Oral-facial-digital syndrome type 1 (OFD1) is a rare condition with X-linked dominant inheritance caused by mutations in the Cxorf5 (OFD1) gene. This gene encodes the OFD1 protein located within centrosomes and basal bodies of primary cilia. Approximately 15-50% of patients with OFD1 progress to end-stage kidney disease following development of polycystic changes within the kidneys. This condition almost always causes intrauterine lethality in males. Description of Case Diagnosis and Treatment. A Caucasian male aged 9 years and 9 months presented with increased urinary frequency, increased thirst, and decreased appetite...
2016: Case Reports in Nephrology
Frank Strutz
Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenetic kidney disease. The clinical course is highly variable, association to certain genetic mutations only weak. Great progress has been made in recent years in determining the pathophysiology of the disease. Diagnosis of ADPKD is almost always possible by ultrasound, genetic examination is confined to selected cases. In addition, imaging is important for prognosis where MRI and computer tomography are superior for determination of total kidney volume...
September 2016: Deutsche Medizinische Wochenschrift
Sreenithya Ravindran, Vinitha Kuruvilla, Kerry Wilbur, Shankar Munusamy
Metformin, a well-known anti-diabetic agent, is very effective in lowering blood glucose in patients with type 2 diabetes with minimal side-effects. Metformin is also being recommended in the treatment of obesity and polycystic ovary syndrome. Metformin elicits its therapeutic effects mainly via activation of AMP-activated kinase (AMPK) pathway. Renal cells under hyperglycemic or proteinuric conditions exhibit inactivation of cell defense mechanisms such as AMPK and autophagy, and activation of pathologic pathways such as mammalian target of rapamycin (mTOR), endoplasmic reticulum (ER) stress, epithelial-to-mesenchymal transition (EMT), oxidative stress, and hypoxia...
September 14, 2016: Journal of Cellular Physiology
Erum A Hartung, Katherine M Dell, Matthew Matheson, Bradley A Warady, Susan L Furth
BACKGROUND: Previous studies have suggested that some children with autosomal recessive polycystic kidney disease (ARPKD) have growth impairment out of proportion to their degree of chronic kidney disease (CKD). The objective of this study was to systematically compare growth parameters in children with ARPKD to those with other congenital causes of CKD in the chronic kidney disease in Children (CKiD) prospective cohort study. METHODS: Height SD scores (z-scores), proportion of children with severe short stature (z-score < -1...
2016: Frontiers in Pediatrics
Tung-Min Yu, Ya-Wen Chuang, Mei-Ching Yu, Cheng-Hsu Chen, Cheng-Kuang Yang, Shih-Ting Huang, Cheng-Li Lin, Kuo-Hsiung Shu, Chia-Hung Kao
BACKGROUND: Data for the risk of any solid cancer in patients with polycystic kidney disease are scarce. Therefore, we did a nationwide cohort study in Taiwan to establish the risk of cancer in patients with polycystic kidney disease without either chronic kidney disease or end-stage renal disease. METHODS: From inpatient claims of the Taiwan National Health Insurance Research Database, we included patients aged 20 years and older and diagnosed with polycystic kidney disease between January, 1998 and December, 2010, in the polycystic kidney disease cohort...
October 2016: Lancet Oncology
Bruno Moulin
Cardiorenal syndromes refer to clinical and metabolic consequences of acute and chronic heart failure or kidney disease on other organ. Recent studies have further clarified the pathophysiological mechanisms behind the different types of cardiorenal syndromes and propose a new classification. The cardiorenal syndrome type 1 corresponds to an acute heart failure (cardiogenic shock, acute decompensated congestive heart failure) which induces acute renal dysfunction. In the cardiorenal syndrome type 2 heart failure is chronic (congestive heart failure) and induces chronic kidney damages in the long-term...
June 2016: La Revue du Praticien
Julia Volkmann, Johannes Nordlohne, Roland Schmitt, Sibylle von Vietinghoff
Multicystic back masses can be of infectious, metastatic, or local pre- or malignant origin. We present a case of a rapidly evolving mass in a hemodialysis patient with severe "chronic kidney disease-associated mineral bone disease" (CKD-MBD), that also highlights limitations of chest x-ray for diagnosis of bone disease.
August 2016: Clinical Case Reports
Mohd Ashraf, Virender Kumar, Rifat Ara Bano, Khursheed Ahmed Wani, Javed Ahmed, Kaisar Ahmed
INTRODUCTION: Definite paucity of data pertaining to spectrum of renal and urinary tract diseases in our state and in various parts of India forms the basis of this study. Available data has emphasized more on specific clinical syndromes and chronic renal diseases rather than over all spectrums of renal and urinary tract diseases, that too in adult population. AIM: The present study a retrospective analysis, forms one of the basic data of paediatric nephrology and urology related disorders in our state...
June 2016: Journal of Clinical and Diagnostic Research: JCDR
Ana Konvalinka, Ihor Batruch, Tomas Tokar, Apostolos Dimitromanolakis, Shelby Reid, Xuewen Song, York Pei, Andrei P Drabovich, Eleftherios P Diamandis, Igor Jurisica, James W Scholey
BACKGROUND: Angiotensin-II (Ang II) mediates progression of autosomal-dominant polycystic kidney disease (ADPKD) and other chronic kidney diseases (CKD). However, markers of kidney Ang II activity are lacking. We previously defined 83 Ang II-regulated proteins in vitro, which reflected kidney Ang II activity in vivo. METHODS: In this study, we developed selected reaction monitoring (SRM) assays for quantification of Ang II-regulated proteins in urine of ADPKD and CKD patients...
2016: Clinical Proteomics
Valentina Corradi, Francesca Martino, Fiorella Gastaldon, Elisa Scalzotto, Carlotta Caprara, Antonio Fortunato, Giulia Pinaffo, Cristina Marchetti, Francesca Fabbi, Davide Giavarina, Fiorenza Ferrari, Mitchell H Rosner, Claudio Ronco
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. Studies have suggested a possible prognostic role of copeptin in determining the rate of progressive kidney function decline in ADPKD patients. However, it remains unresolved whether the changes in copeptin levels are specific for ADPKD or merely reflect a decline in glomerular filtration rate (GFR) regardless of the etiology of chronic kidney disease (CKD). METHODS: We performed a case-control study in ADPKD and non-ADPKD (control) patients...
September 2016: Clinical Nephrology
Meltem Gursu, Sami Uzun, Derya Topcuoğlu, Leyli Kadriye Koc, Lamiye Yucel, Abdullah Sumnu, Egemen Cebeci, Oktay Ozkan, Ahmet Behlul, Leyla Koc, Savas Ozturk, Rumeyza Kazancioglu
AIM: To examine all skin changes in peritoneal dialysis (PD) patients followed up in our unit. METHODS: Patients on PD program for at least three months without any known chronic skin disease were included in the study. Patients with already diagnosed skin disease, those who have systemic diseases that may cause skin lesions, patients with malignancies and those who did not give informed consent were excluded from the study. All patients were examined by the same predetermined dermatologist with all findings recorded...
July 6, 2016: World Journal of Nephrology
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