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25 papers 0 to 25 followers Congenital Anomalies of the Kidney and Urinary Tract
By P O Pediatrics, Nephrology
Annelies M C Mavinkurve-Groothuis, Frank van de Kracht, Rik Westland, Joanna A E van Wijk, Jacqueline J Loonen, Michiel F Schreuder
BACKGROUND: Children with unilateral Wilms tumor (WT) treated with chemotherapy and/or radiotherapy and nephrectomy have excellent survival rates. A solitary functioning kidney (SFK) is associated with progressive renal injury. This study aims to investigate the additional effect of Wilms tumor treatment on renal function compared with children with an SFK for non-oncological reasons. METHODS: A single-center retrospective cohort study on the renal injury markers of 79 survivors of unilateral WT was performed and compared with a matched group of children with an SFK for non-oncological reasons...
March 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Elke Wühl, Karlijn J van Stralen, Enrico Verrina, Anna Bjerre, Christoph Wanner, James Goya Heaf, Oscar Zurriaga, Andries Hoitsma, Patrick Niaudet, Runolfur Palsson, Pietro Ravani, Kitty J Jager, Franz Schaefer
BACKGROUND AND OBJECTIVES: Congenital anomalies of the kidney and urinary tract (CAKUT) are the leading cause of ESRD in children, but the proportion of patients with individual CAKUT entities progressing to ESRD during adulthood and their long-term clinical outcomes are unknown. This study assessed the age at onset of renal replacement therapy (RRT) and patient and renal graft survival in patients with CAKUT across the entire age range. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Patients with CAKUT were compared with age-matched patients who were undergoing RRT for other renal disorders on the basis of data from the European Renal Association-European Dialysis and Transplant Association Registry...
January 2013: Clinical Journal of the American Society of Nephrology: CJASN
Rui-Yun Chen, Han Chang
Renal dysplasia is an aberrant developmental disease usually diagnosed during the perinatal and childhood years. Prevalence is estimated at 0.1% of infants (via ultrasound screening) and 4% of fetuses and infants (via autopsy study). Occurrences may be combined with abnormalities in the collecting system or associated with complex syndromes. Histopathology shows primitive tubules surrounded by a fibromuscular collar. The differential diagnosis includes renal dysplasia, hypoplasia, and renal atrophy. Immunohistochemical expression of the paired box genes 2 and 8 (PAX2/8) and Wilms tumor 1 (WT1) is increased in the primitive ducts and fibromuscular collar, respectively...
April 2015: Archives of Pathology & Laboratory Medicine
Yugeesh R Lankadeva, Reetu R Singh, Marianne Tare, Karen M Moritz, Kate M Denton
Epidemiological studies reveal that children born with a solitary functioning kidney (SFK) have a greater predisposition to develop renal insufficiency and hypertension in early adulthood. A congenital SFK is present in patients with unilateral renal agenesis or unilateral multicystic kidney dysplasia, leading to both structural and functional adaptations in the remaining kidney, which act to mitigate the reductions in glomerular filtration rate and sodium excretion that would otherwise ensue. To understand the mechanisms underlying the early development of renal insufficiency in children born with a SFK, we established a model of fetal uninephrectomy (uni-x) in sheep, a species that similar to humans complete nephrogenesis before birth...
April 15, 2014: American Journal of Physiology. Renal Physiology
Kenji Ishikura, Osamu Uemura, Yuko Hamasaki, Hideo Nakai, Shuichi Ito, Ryoko Harada, Motoshi Hattori, Yasuo Ohashi, Ryojiro Tanaka, Koichi Nakanishi, Tetsuji Kaneko, Kazumoto Iijima, Masataka Honda
BACKGROUND: Vesicoureteral reflux (VUR) is associated with an increased risk of kidney disorders. It is unclear whether VUR is associated with progression from chronic kidney disease (CKD) to end-stage kidney disease (ESKD) in children with congenital anomalies of the kidney and urinary tract (CAKUT). METHODS: We conducted a 3-year follow-up survey of a cohort of 447 children with CKD (stage 3-5). Rates of and risk factors for progression to ESKD were determined using the Kaplan-Meier method and Cox regression respectively...
January 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Kalyani R Vats, Chandra Ishwad, Ish Singla, Ashutosh Vats, Robert Ferrell, Demetrius Ellis, Michael Moritz, Urvashi Surti, Parul Jayakar, Donald R Frederick, Abhay N Vats
Congenital anomalies of kidney and urinary tract (CAKUT), including vesico-ureteric reflux (VUR), are major causes of ESRD in childhood. Herein is reported evidence for a locus on 13q33q34 associated with CAKUT. Deletion mapping of chromosome 13q was performed in four children with CAKUT using 31 microsatellite markers on peripheral blood genomic DNA that was obtained from the patients and their parents. mRNA expression of the positional candidate genes was compared with sequences in electronic databases in silico and also studied in adult and fetal mouse kidneys using reverse transcription-PCR...
April 2006: Journal of the American Society of Nephrology: JASN
Simone Sanna-Cherchi, Pietro Ravani, Valentina Corbani, Stefano Parodi, Riccardo Haupt, Giorgio Piaggio, Maria L Degli Innocenti, Danio Somenzi, Antonella Trivelli, Gianluca Caridi, Claudia Izzi, Francesco Scolari, Girolamo Mattioli, Landino Allegri, Gian Marco Ghiggeri
Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) are a major cause of morbidity in children. We measured the risk of progression to end-stage renal disease in 312 patients with CAKUT preselected for the presence of anomalies in kidney number or size. A model of dialysis-free survival from birth was established as a function of the renal CAKUT categories of solitary kidney; unilateral and bilateral hypodysplasia; renal hypodysplasia associated with posterior urethral valves; and multicystic and horseshoe kidney...
September 2009: Kidney International
Robert L Chevalier
Most renal failure in children results from congenital anomalies of the kidney and urinary tract (CAKUTs). Sanna-Cherchi et al. predict that by 30 years of age, nearly 50% of patients with a solitary kidney would be receiving dialysis. This outcome differs markedly from that of renal-transplant donors, who have no increased risk for renal failure. Because morbidity from CAKUTs may not develop until adulthood, these patients should be closely followed throughout life.
September 2009: Kidney International
Renfang Song, Ihor V Yosypiv
Congenital anomalies of the kidney and urinary tract (CAKUT) occur in 1 in 500 births and are a major cause of morbidity in children. Notably, CAKUT account for the most cases of pediatric end-stage renal disease and predispose the individual to hypertension and cardiovascular disease throughout life. Although some forms of CAKUT are a part of a syndrome or are associated with a positive family history, most cases of renal system anomalies are sporadic and isolated to the urinary tract. Broad phenotypic spectrum of CAKUT and variability in genotype-phenotype correlation indicate that pathogenesis of CAKUT is a complex process that depends on interplay of many factors...
March 2011: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Kirsten Y Renkema, Paul J Winyard, Ilya N Skovorodkin, Elena Levtchenko, An Hindryckx, Cécile Jeanpierre, Stefanie Weber, Rémi Salomon, Corinne Antignac, Seppo Vainio, Andreas Schedl, Franz Schaefer, Nine V A M Knoers, Ernie M H F Bongers
Congenital anomalies of the kidney and urinary tract (CAKUT) are the commonest cause of chronic kidney disease in children. Structural anomalies within the CAKUT spectrum include renal agenesis, kidney hypo-/dysplasia, multicystic kidney dysplasia, duplex collecting system, posterior urethral valves and ureter abnormalities. While most CAKUT cases are sporadic, familial clustering of CAKUT is common, emphasizing a strong genetic contribution to CAKUT origin. Animal experiments demonstrate that alterations in genes crucial for kidney development can cause experimental CAKUT, while expression studies implicate mislocalization and/or aberrant levels of the encoded proteins in human CAKUT...
December 2011: Nephrology, Dialysis, Transplantation
Isabel G Quirino, Jose Silverio S Diniz, Maria Candida F Bouzada, Alamanda K Pereira, Thais J Lopes, Gabriela M Paixão, Natalia N Barros, Luisa C Figueiredo, Antonio Carlos V Cabral, Ana Cristina Simões e Silva, Eduardo A Oliveira
BACKGROUND AND OBJECTIVES: With the advent of fetal screening ultrasonography, the detection of congenital anomalies of the kidney and urinary tract (CAKUT) in utero has permitted early management of these conditions. This study aims to describe the clinical course of a large cohort of patients with prenatally detected nephrouropathies. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: In this retrospective cohort study, 822 patients were prenatally diagnosed with CAKUT and systematically followed up at a tertiary Renal Unit for a median time of 43 months...
March 2012: Clinical Journal of the American Society of Nephrology: CJASN
Renfang Song, Ihor V Yosypiv
The mature renal medulla, the inner part of the kidney, consists of the medullary collecting ducts, loops of Henle, vasa recta and the interstitium. The unique spatial arrangement of these components is essential for the regulation of urine concentration and other specialized kidney functions. Thus, the proper and timely assembly of medulla constituents is a crucial morphogenetic event leading to the formation of a functioning metanephric kidney. Mechanisms that direct renal medulla formation are poorly understood...
January 2012: Organogenesis
Mélanie Paces-Fessy, Mélanie Fabre, Céline Lesaulnier, Silvia Cereghini
The transcription factors HNF1B and Pax2, co-expressed in the Wolffian duct and ureteric bud epithelia, play essential roles during the early steps of mouse kidney development. In humans, heterozygous mutations in these genes display a number of common kidney phenotypes, including hypoplasia and multicystic hypoplastic kidneys. Moreover, a high prevalence of mutations either in HNF1B or PAX2 has been observed in children with renal hypodysplasia. To gain a better understanding of Hnf1b and Pax2 interactions in vivo, we generated compound heterozygous mice for Hnf1b and Pax2 null alleles...
July 15, 2012: Human Molecular Genetics
Albertien M van Eerde, Karen Duran, Els van Riel, Carolien G F de Kovel, Bobby P C Koeleman, Nine V A M Knoers, Kirsten Y Renkema, Henricus J R van der Horst, Arend Bökenkamp, Johanna M van Hagen, Leonard H van den Berg, Katja P Wolffenbuttel, Joop van den Hoek, Wouter F Feitz, Tom P V M de Jong, Jacques C Giltay, Cisca Wijmenga
Vesico-ureteral reflux (VUR) is the retrograde passage of urine from the bladder to the urinary tract and causes 8.5% of end-stage renal disease in children. It is a complex genetic developmental disorder, in which ectopic embryonal ureteric budding is implicated in the pathogenesis. VUR is part of the spectrum of Congenital Anomalies of the Kidney and Urinary Tract (CAKUT). We performed an extensive association study for primary VUR using a two-stage, case-control design, investigating 44 candidate genes in the ureteric budding pathway in 409 Dutch VUR patients...
2012: PloS One
Ihor V Yosypiv
Congenital anomalies of the kidney and urinary tract (CAKUTs) occur in 3-6 per 1000 live births, account for the most cases of pediatric end-stage kidney disease (ESKD), and predispose an individual to hypertension and cardiovascular disease throughout life. Although CAKUTs are a part of many known syndromes, only few single-candidate causative genes have been implicated so far in nonsyndromic cases of human CAKUT. Evidence from mouse models supports the hypothesis that non-syndromic human CAKUT may be caused by single-gene defects...
2012: International Journal of Nephrology
Rik Westland, Roel A J Kurvers, Joanna A E van Wijk, Michiel F Schreuder
OBJECTIVE: The hyperfiltration hypothesis implies that children with a solitary functioning kidney are at risk to develop hypertension, proteinuria, and chronic kidney disease. We sought to determine the presenting age of renal injury and identify risk factors for children with a solitary functioning kidney. METHODS: We evaluated 407 patients for signs of renal injury, defined as hypertension, proteinuria, an impaired glomerular filtration rate, and/or the use of renoprotective medication...
February 2013: Pediatrics
Rik Westland, Michiel F Schreuder, Johannes C F Ket, Joanna A E van Wijk
BACKGROUND: Unilateral renal agenesis (URA) is associated with other congenital anomalies of the kidney and urinary tract (CAKUT) and extra-renal anomalies. However, the reported prevalences of these anomalies are highly variable. We estimated the prevalence of associated CAKUT and extra-renal anomalies in patients with URA. Furthermore, we determined the prevalence of renal injury in URA patients. METHODS: We conducted a systematic review of English and non-English articles using PubMed and Embase...
July 2013: Nephrology, Dialysis, Transplantation
Paloma Parvex, Chistophe Combescure, Maria Rodriguez, Jacques Birraux, Eric Girardin
BACKGROUND: Congenital anomalies of the kidney and urinary tract (CAKUT) is a main cause leading to endstage renal disease (ESRD) during childhood occurring at a frequency of 1 in every 500 pregnancies. No early predictive markers of long-term renal function (RF) are validated in these neonates. The aim of this study was to compare CysC and creatinine (creat) as markers of RF from birth to 2 years and to identify factors of RF progression. METHODS: The 56 patients included in this study were followed for a median of 235 days (137 - 739)...
May 2014: Clinical Nephrology
Joshua Blake, Norman D Rosenblum
The human kidney is composed of an arborized network of collecting ducts, calyces and urinary pelvis that facilitate urine excretion and regulate urine composition. The renal collecting system is formed in utero, completed by the 34th week of gestation in humans, and dictates final nephron complement. The renal collecting system arises from the ureteric bud, a derivative of the intermediate-mesoderm derived nephric duct that responds to inductive signals from adjacent tissues via a process termed ureteric induction...
December 2014: Seminars in Cell & Developmental Biology
Maria M Rodriguez
This article reviews the majority of Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) with emphasis in Pediatric Pathology describing and illustrating lesions as varied as ureteral duplications, ureteropelvic junction obstruction, horseshoe kidney, posterior urethral valve and prune belly syndrome, obstructive renal dysplasia, nonmotile ciliopathies and several syndromes associated with renal malformations (Meckel-Joubert, short rib, Bardet-Biedl, asplenia/polysplenia, hereditary renal adysplasia, Zellweger, trisomies, VACTER-L, Potter, caudal dysplasia, and sirenomelia), as well as ADPK, and ARPK...
October 2014: Fetal and Pediatric Pathology
2015-09-26 16:31:00
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