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https://www.readbyqxmd.com/read/28108282/hyperfiltration-associated-biomechanical-forces-in-glomerular-injury-and-response-potential-role-for-eicosanoids
#1
REVIEW
Mukut Sharma, Ram Sharma, Ellen T McCarthy, Virginia J Savin, Tarak Srivastava
Hyperfiltration is a well-known risk factor in progressive loss of renal function in chronic kidney disease (CKD) secondary to various diseases. A reduced number of functional nephrons due to congenital or acquired cause(s) results in hyperfiltration in the remnant kidney. Hyperfiltration-associated increase in biomechanical forces namely pressure-induced tensile stress and fluid flow-induced shear stress (FFSS) determine cellular injury and response. We believe the current treatment of CKD yields limited success because it largely attenuates pressure-induced tensile stress changes but not the effect of FFSS on podocytes...
January 17, 2017: Prostaglandins & Other Lipid Mediators
https://www.readbyqxmd.com/read/28100158/double-inferior-vena-cava-ivc-with-intrahepatic-interruption-hemiazygos-vein-continuation-and-intrahepatic-venous-shunt
#2
Hilal Sahin, Yeliz Pekcevik, Ramazan Aslaner
The duplication of the inferior vena cava (IVC) is a rare congenital anomaly, which also has some variations regarding the complex embryological development of the IVC. In the typical form, infrarenal IVC segments are duplicated and the left IVC joins the left renal vein, which crosses anterior to the aorta in the normal fashion to join the right IVC. In variant forms, the interruption of the intrahepatic segment of the IVC, azygos or hemiazygos continuation, or retroaortic course of the renal vein may be seen...
January 2017: Vascular and Endovascular Surgery
https://www.readbyqxmd.com/read/28099405/insights-in-transplanting-complex-paediatric-renal-recipients-with-vascular-anomalies
#3
Pankaj Chandak, Nicos Kessaris, Chris J Callaghan, Francis Calder, Jelena Stojanovic, Jonathon Olsburgh, Martin Drage, Helen Hume-Smith, Zubir Ahmed, Anna Adamusiak, Derek Roebuck, Colin Forman, Stephen D Marks, Nizam Mamode
BACKGROUND: Children with end-stage kidney disease may have co-existing iatrogenic or congenital vascular anomalies making transplantation difficult. We describe our approach in 5 recipients with vascular anomalies and significant co-morbidities, including one case of blood group incompatibility. METHODS: Five children aged 3 - 17 (median 7) years, weighing 14 - 34 (median 18) kg of whom 4 had occluded inferior vena cava or iliac veins and 2 had previous complex vascular reconstructions prior to transplantation for mid-aortic syndrome and multiple aortic aneurysms, respectively underwent renal transplantation...
January 17, 2017: Transplantation
https://www.readbyqxmd.com/read/28078995/sphingolipids-in-genetic-and-acquired-forms-of-chronic-kidney-diseases
#4
Norishi Ueda
Sphingolipids (SLs) regulate apoptosis, proliferation, and stress response. SLs, including ceramide, glycosphingolipids (glucosylceramide, lactosylceramide, and gangliosides) and sphingosine-1-phosphate (S1P), play a role in the pathogenesis and progression of genetic (lysosomal storage disease, congenital nephrotic syndrome and polycystic kidney disease) and non-genetic forms of chronic kidney diseases (CKDs). SLs metabolism defects promote complications (cardiovascular events, etc.) via oxidant stress in CKDs...
January 12, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28076021/radiologic-assessment-of-native-renal-vasculature-a-multimodality-review
#5
Sayf Al-Katib, Monisha Shetty, Syed Mohammad A Jafri, Syed Zafar H Jafri
A wide range of clinically important anatomic variants and pathologic conditions may affect the renal vasculature, and radiologists have a pivotal role in the diagnosis and management of these processes. Because many of these entities may not be suspected clinically, renal artery and vein assessment is an essential application of all imaging modalities. An understanding of the normal vascular anatomy is essential for recognizing clinically important anatomic variants. An understanding of the protocols used to optimize imaging modalities also is necessary...
January 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28068786/topical-and-low-dose-intravenous-tranexamic-acid-in-cyanotic-cardiac-surgery
#6
Jigar Patel, Mrugesh Prajapati, Hardik Patel, Hemang Gandhi, Shilpa Deodhar, Himani Pandya
Background Coagulopathy is a major problem in surgery for cyanotic congenital heart disease. Tranexamic acid has been used both topically and systemically and plays a vital role in pediatric cardiac surgery by reducing blood loss and blood product requirement. We aimed to determine the anti-fibrinolytic effectiveness of low-dose systemic or topical tranexamic acid or a combination of both. Methods Seventy-five patients were divided in 3 groups of 25. Group A patients were given tranexamic acid 20 mg kg(-1) intravenously after sternotomy and 20 mg kg(-1) after heparin reversal...
January 1, 2017: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/28060690/-peritoneal-dialysis-in-the-first-two-years-of-life-experience-of-a-nephrology-and-renal-transplantation-pediatric-unit
#7
Sofia Deuchande, Tânia Mano, Cristina Novais, Rute Machado, Rosário Stone, Margarida Almeida
INTRODUCTION: Peritoneal dialysis is the dialytic method of choice in chronic end-stage renal disease in children. This study main purpose was to characterize the long-term survival of a pediatric population who began peritoneal dialysis within the first two years of life. MATERIAL AND METHODS: A descriptive and retrospective study was performed in a portuguese nephrology and renal transplantation pediatric unit, between January 1991 and August 2014. End-stage renal disease etiology, mortality, comorbidities and complications of peritoneal dialysis and end-stage renal disease, growth and psychomotor development were evaluated...
September 2016: Acta Médica Portuguesa
https://www.readbyqxmd.com/read/28060152/an-elevated-low-cardiac-output-syndrome-score-is-associated-with-morbidity-in-infants-after-congenital-heart-surgery
#8
Kalia P Ulate, Ofer Yanay, Howard Jeffries, Harris Baden, Jane L Di Gennaro, Jerry Zimmerman
OBJECTIVES: To evaluate an empirically derived Low Cardiac Output Syndrome Score as a clinical assessment tool for the presence and severity of Low Cardiac Output Syndrome and to examine its association with clinical outcomes in infants who underwent surgical repair or palliation of congenital heart defects. DESIGN: Prospective observational cohort study. SETTING: Cardiac ICU at Seattle Children's Hospital. PATIENTS: Infants undergoing surgical repair or palliation of congenital heart defects...
January 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28058212/complement-related-kidney-diseases-recurrence-after-transplantation
#9
REVIEW
Maurizio Salvadori, Elisabetta Bertoni
The recurrence of renal disease after renal transplantation is becoming one of the main causes of graft loss after kidney transplantation. This principally concerns some of the original diseases as the atypical hemolytic uremic syndrome (HUS), the membranoproliferative glomerulonephritis (MPGN), in particular the MPGN now called C3 glomerulopathy. Both this groups of renal diseases are characterized by congenital (genetic) or acquired (auto-antibodies) modifications of the alternative pathway of complement...
December 24, 2016: World Journal of Transplantation
https://www.readbyqxmd.com/read/28054444/heterozygous-pathogenic-variant-in-dact1-causes-an-autosomal-dominant-syndrome-with-features-overlapping-townes-brocks-syndrome
#10
Bryn D Webb, Sanjeeva Metikala, Patricia G Wheeler, Mingma D Sherpa, Sander M Houten, Marko E Horb, Eric E Schadt
A heterozygous nonsense variant was identified in dapper, antagonist of beta-catenin, 1 (DACT1) via whole exome sequencing in family members with imperforate anus, structural renal abnormalities, genitourinary anomalies, and/or ear anomalies. The DACT1 c.1256G>A;p.Trp419* variant segregated appropriately in the family consistent with an autosomal dominant mode of inheritance. DACT1 is a member of the Wnt signaling pathway, and mice homozygous for null alleles display multiple congenital anomalies including absent anus with blind-ending colon and genitourinary malformations...
January 5, 2017: Human Mutation
https://www.readbyqxmd.com/read/28043466/increasing-complexity-of-heart-transplantation-in-patients-with-congenital-heart-disease
#11
William Y Shi, Pankaj Saxena, Matthew S Yong, Silvana F Marasco, David C McGiffin, Anne Shipp, Robert G Weintraub, Yves d'Udekem, Christian P Brizard, Igor E Konstantinov
Owing to improved surgical results, there is a growing population of patients with repaired congenital heart disease (CHD) requiring heart transplantation. The objective of the study was to review our experience in these patients. A retrospective review of the outcomes of heart transplantation in patients with CHD (n = 77) between 1988 and 2014 was performed. Outcomes of early (1988-1999) and late (2000-2014) eras were compared. In results, the mean age was 18 ± 14 years (range: 16 days-58 years). Seventy (91%) patients underwent a mean of 2...
July 2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28027277/multi-organ-involvement-confounding-the-diagnosis-of-bartonella-henselae-infective-endocarditis-in-children-with-congenital-heart-disease
#12
Christopher P Ouellette, Sarita Joshi, Karen Texter, Preeti Jaggi
Two children with congenital heart disease status-post surgical correction presented with prolonged constitutional symptoms, hepatosplenomegaly and pancytopenia. Concern for malignancy prompted bone marrow biopsies that were without evidence thereof. In case #1, echocardiography identified a multilobulated vegetation on the conduit valve. In case #2, transthoracic, transesophageal and intracardiac echocardiography were performed and were without evidence of cardiac vegetations, however pulmonic emboli raised concern for infective endocarditis...
December 23, 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28018145/epigenetics-of-renal-development-and-disease
#13
REVIEW
Sylvia A Hilliard, Samir S El-Dahr
An understanding of epigenetics is indispensable to our understanding of gene regulation under normal and pathological states. This knowledge will help with designing better therapeutic approaches in regenerative tissue medicine. Epigenetics allows us to parse out the mechanisms by which transcriptional regulators gain access to specific gene loci thereby imprinting epigenetic information affecting chromatin function. This epigenetic memory forms the basis of cell lineage specification in multicellular organisms...
December 2016: Yale Journal of Biology and Medicine
https://www.readbyqxmd.com/read/28009797/what-s-new-in-congenital-scoliosis
#14
Joshua M Pahys, James T Guille
BACKGROUND: Congenital scoliosis is a failure of vertebral formation, segmentation, or a combination of the 2 arising from abnormal vertebral development during weeks 4 to 6 of gestation. The associated spinal deformity can be of varying severity and result in a stable or progressive deformity based on the type and location of the anomalous vertebra(e). Bracing for congenital scoliosis is rarely indicated, while recent reports have demonstrated the utility of serial derotational casting for longer curves with multiple anomalous vertebrae as an effective "time buying strategy" to delay the need for surgery...
December 22, 2016: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/28007064/the-society-of-thoracic-surgeons-congenital-heart-surgery-database-public-reporting-initiative
#15
REVIEW
Jeffrey P Jacobs
Three basic principles provide the rationale for the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database (CHSD) public reporting initiative: (1) Variation in congenital and pediatric cardiac surgical outcomes exist. (2) Patients and their families have the right to know the outcomes of the treatments that they will receive. (3). It is our professional responsibility to share this information with them in a format they can understand. The STS CHSD public reporting initiative facilitates the voluntary transparent public reporting of congenital and pediatric cardiac surgical outcomes using the STS CHSD Mortality Risk Model...
January 2017: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
https://www.readbyqxmd.com/read/28002851/prenatal-ultrasonographic-diagnosis-of-cataract-in-utero-manifestations-of-cryptic-disease
#16
Eran Ashwal, Asaf Achiron, Yinon Gilboa, Michal Berkenstadt, Mordecai Rosner, Reuven Achiron
Purpose To report and review our experience with antenatal evaluation for fetuses diagnosed with congenital cataract. Materials and Methods We retrospectively identified pregnancies diagnosed with fetal cataract during antenatal ultrasound. Evaluation of fetal eyes included intraocular anatomy and biometry. Data on fetal malformations, serology and fetal karyotype were collected. Results 8 cases, identified over the course of 10 years, were reviewed. Week 15 was the mean time for diagnosis (range 11 - 34)...
December 21, 2016: Ultraschall in der Medizin
https://www.readbyqxmd.com/read/28000646/renal-cell-carcinoma-in-a-horseshoe-kidney-report-of-a-rare-disease
#17
Kehinde Habeeb Tijani, Rufus Wale Ojewola, Dubem E Orakwe, Abisola E Oliyide
A horseshoe kidney (HSK) is the most common congenital renal fusion anomaly. HSKs are more likely than normal kidneys to have associated problems of stones, ureteropelvic junction obstruction, stasis and infection. However, they do not have an increased incidence of renal cell carcinoma when compared to normal kidneys. Due to its rarity, accurate diagnosis may be difficult. Of similar significance is the fact that problems may arise during surgery on these kidneys due to altered anatomy and aberrant blood supply...
October 2016: Nigerian Postgraduate Medical Journal
https://www.readbyqxmd.com/read/27993715/appearance-of-pyelitis-emphysematosa-on-computed-tomography
#18
Todd S Yecies, Brian T Kadow, Stephen V Jackman
Pyelitis emphysematosa is a gas-forming infection characterized by gas located within the wall of the collecting system and renal pelvis. There are only 2 reported cases of pyelitis emphysematosa in the literature, neither of which occurred in the era of cross-sectional imaging. Here we present a case of pyelitis emphysematosa occurring in an elderly female with congenital left renal atrophy and chronic right hydronephrosis secondary to ureteropelvic junction obstruction.
December 16, 2016: Urology
https://www.readbyqxmd.com/read/27992675/readmissions-after-adult-congenital-heart-surgery-frequency-and-risk-factors
#19
Yuli Y Kim, Wei He, Thomas E MacGillivray, Oscar J Benavidez
OBJECTIVE: Despite their clinical importance, 30-day readmission after adult congenital heart surgery has been understudied. They sought to determine the frequency of unplanned readmissions after adult congenital heart surgery and to identify any potential associated risk factors. DESIGN: Retrospective cohort study using State Inpatient Databases for Washington, New York, Florida, and California from 2009 to 2011. SETTING: Federal and nonfederal acute care hospitals...
December 19, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27964749/truncating-de-novo-mutations-in-the-kr%C3%A3-ppel-type-zinc-finger-gene-znf148-in-patients-with-corpus-callosum-defects-developmental-delay-short-stature-and-dysmorphisms
#20
Servi J C Stevens, Anthonie J van Essen, Conny M A van Ravenswaaij, Abdallah F Elias, Jaclyn A Haven, Stefan H Lelieveld, Rolph Pfundt, Willy M Nillesen, Helger G Yntema, Kees van Roozendaal, Alexander P Stegmann, Christian Gilissen, Han G Brunner
BACKGROUND: Krüppel-type zinc finger genes (ZNF) constitute a large yet relatively poorly characterized gene family. ZNF genes encode proteins that recognize specific DNA motifs in gene promotors. They act as transcriptional co-activators or -repressors via interaction with chromatin remodeling proteins and other transcription factors. Only few ZNF genes are currently linked to human disorders and identification of ZNF gene-associated human diseases may help understand their function...
December 13, 2016: Genome Medicine
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