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https://www.readbyqxmd.com/read/28809621/cardiovascular-risk-factors-and-mortality-in-children-with-chronic-kidney-disease
#1
Abdullahi Mudi, Caroline Dickens, Cecil Levy, Daynia Ballot
BACKGROUND: Cardiovascular disease (CVD) begins early in children with chronic kidney disease (CKD), and its progression is determined by the presence of single or multiple cardiovascular risk factors (CVRFs). OBJECTIVE: To determine the prevalence of CVRFs in children with CKD and their association with mortality in children on chronic dialysis. METHODS: This comparative cross-sectional study recruited children aged 5 - 18 years with all stages of CKD...
July 28, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/28796785/low-renal-but-high-extrarenal-phenotype-variability-in-schimke-immuno-osseous-dysplasia
#2
Beata S Lipska-Ziętkiewicz, Jutta Gellermann, Olivia Boyer, Olivier Gribouval, Szymon Ziętkiewicz, Jameela A Kari, Mohamed A Shalaby, Fatih Ozaltin, Jiri Dusek, Anette Melk, Aysun K Bayazit, Laura Massella, Lidia Hyla-Klekot, Sandra Habbig, Astrid Godron, Maria Szczepańska, Beata Bieniaś, Dorota Drożdż, Rasha Odeh, Wioletta Jarmużek, Katarzyna Zachwieja, Agnes Trautmann, Corinne Antignac, Franz Schaefer
Schimke immuno-osseous dysplasia (SIOD) is a rare multisystem disorder with early mortality and steroid-resistant nephrotic syndrome (SRNS) progressing to end-stage kidney disease. We hypothesized that next-generation gene panel sequencing may unsurface oligosymptomatic cases of SIOD with potentially milder disease courses. We analyzed the renal and extrarenal phenotypic spectrum and genotype-phenotype associations in 34 patients from 28 families, the largest SMARCAL1-associated nephropathy cohort to date. In 11 patients the diagnosis was made unsuspectedly through SRNS gene panel testing...
2017: PloS One
https://www.readbyqxmd.com/read/28794651/hypertension-in-pediatric-patients-with-chronic-kidney-disease-management-challenges
#3
REVIEW
Claire M Gallibois, Natasha A Jawa, Damien G Noone
In contrast to adults where hypertension is a leading cause of chronic kidney disease, in pediatrics, hypertension is predominantly a sequela, however, an important one that, like in adults, is likely associated with a more rapid decline in kidney function or progression of chronic kidney disease to end stage. There is a significant issue with unrecognized, or masked, hypertension in childhood chronic kidney disease. Recent evidence and, therefore, guidelines now suggest targeting a blood pressure of <50th percentile for age, sex, and height in children with proteinuria and chronic kidney disease...
2017: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/28780657/combination-therapy-of-rituximab-and-mycophenolate-mofetil-in-childhood-lupus-nephritis
#4
Julien Hogan, Astrid Godron, Véronique Baudouin, Theresa Kwon, Jérôme Harambat, Georges Deschênes, Olivier Niel
BACKGROUND: In clinical trials, the addition of rituximab (RTX) to the combination therapeutic regimen of mycophenolate mofetil (MMF) and corticosteroids failed to improve outcome in lupus nephritis (LN). However, recent data suggest that RTX may have steroid-sparing beneficial effects with an efficacy similar to that of conventional regimens. We report our experience with RTX in the treatment of children with LN. METHODS: Patients treated with RTX for first occurrence of LN class III to V were enrolled in the study...
August 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28776307/genetic-basis-of-adult-onset-nephrotic-syndrome-and-focal-segmental-glomerulosclerosis
#5
REVIEW
Jian Liu, Weiming Wang
Nephrotic syndrome (NS) is one of the most common glomerular diseases with signs of nephrosis, heavy proteinuria, hypoalbuminemia, and edema. Dysfunction of glomerular filtration barrier causes protein loss through the kidneys. Focal segmental glomerulosclerosis (FSGS) accounts for nearly 20% of NS among children and adults. Adult-onset FSGS/NS is often associated with low response to steroid treatment and immunosuppressive medication and poor renal survival. Several genes involved in NS and FSGS have been identified by linkage analysis and next-generation sequencing...
August 3, 2017: Frontiers of Medicine
https://www.readbyqxmd.com/read/28759637/pituitary-adenylate-cyclase-activating-polypeptide-pacap-in-zebrafish-models-of-nephrotic-syndrome
#6
Benedicte Eneman, Mohamed A Elmonem, Lambertus P van den Heuvel, Laleh Khodaparast, Ladan Khodaparast, Chris van Geet, Kathleen Freson, Elena Levtchenko
Pituitary adenylate cyclase-activating polypeptide (PACAP) is an inhibitor of megakaryopoiesis and platelet function. Recently, PACAP deficiency was observed in children with nephrotic syndrome (NS), associated with increased platelet count and aggregability and increased risk of thrombosis. To further study PACAP deficiency in NS, we used transgenic Tg(cd41:EGFP) zebrafish with GFP-labeled thrombocytes. We generated two models for congenital NS, a morpholino injected model targeting nphs1 (nephrin), which is mutated in the Finnish-type congenital NS...
2017: PloS One
https://www.readbyqxmd.com/read/28752734/elevated-baseline-adiponectin-level-predicting-an-increased-risk-of-disease-activity-in-idiopathic-nephrotic-syndrome-and-chronic-kidney-disease-in-children
#7
Wafaa M Abo El Fotoh, Ghada M El Mashad
BACKGROUND: Adiponectin, an adipocyte-derived protein, is present abundantly in circulation and has anti-atherogenic and anti-inflammatory effects, so we aimed to assess the role of adiponectin in idiopathic nephrotic syndrome and chronic kidney disease in children. METHODS: Overall of 290 children, 95 patients with the steroid-responsive idiopathic nephrotic syndrome (50 in remission and 45 in relapse).Another 95 patients with chronic kidney disease (50 on hemodialysis and 45 on conservative therapies) and 100 apparently healthy matched children were enrolled into the study...
July 27, 2017: Minerva Pediatrica
https://www.readbyqxmd.com/read/28751734/glucocorticoid-therapy-regulates-podocyte-motility-by-inhibition-of-rac1
#8
James C McCaffrey, Nicholas J Webb, Toryn M Poolman, Maryline Fresquet, Cressida Moxey, Leo A H Zeef, Ian J Donaldson, David W Ray, Rachel Lennon
Nephrotic syndrome (NS) occurs when the glomerular filtration barrier becomes excessively permeable leading to massive proteinuria. In childhood NS, immune system dysregulation has been implicated and increasing evidence points to the central role of podocytes in the pathogenesis. Children with NS are typically treated with an empiric course of glucocorticoid (Gc) therapy; a class of steroids that are activating ligands for the glucocorticoid receptor (GR) transcription factor. Although Gc-therapy has been the cornerstone of NS management for decades, the mechanism of action, and target cell, remain poorly understood...
July 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28711069/preterm-birth-and-its-impact-on-renal-health
#9
Valerie A Luyckx
Preterm birth occurs in approximately 10% of all births worldwide. Preterm infants have reduced nephron numbers at birth in proportion to gestational age, and are at increased risk of neonatal acute kidney injury as well as higher blood pressure, proteinuria, and chronic kidney disease later in life. Rapid catch-up growth in preterm infants, especially if resulting in obesity, is a risk factor for end-stage kidney disease among children with proteinuric renal disease. Preterm birth, however, is a risk factor not only for the infant because mothers who deliver preterm have an increased risk of having subsequent preterm deliveries as well as hypertension, cardiovascular disease, and renal disease later in life...
July 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28700526/urinary-macrophage-migration-inhibitory-factor-as-a-noninvasive-biomarker-in-pediatric-henoch-sch%C3%A3-nlein-purpura-nephritis
#10
Jiapei Wang, Yunyan Li, Yuanling Chen, Xiahua Dai, Yazhen Di, Mengjiao Shen, Qianqian Ying, Shiwei Fu, Yinjie Li
PURPOSES: The aims of this study were to investigate urinary macrophage migration inhibitory factor (MIF) levels and their clinical significance in Henoch-Schönlein purpura (HSP) children with or without nephritis (N) and to assess the influence of steroid treatment on the urine MIF levels of HSPN patients. METHODS: Group I comprised 35 children with HSPN who were examined twice (A before treatment and B after steroid treatment). Group II comprised 41 children with HSP...
August 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28697831/-adverse-effects-of-high-dose-methotrexate-therapy
#11
Qing Sun, Yao Xie, Wei-Hong Zhao, Ying Hua, Peng-Hui Wu, Shuo Li, Xin-Tian Lu
OBJECTIVE: To investigate the adverse effects of high-dose methotrexate (HDMTX) therapy, and to provide a theoretical basis for optimizing clinical treatment. METHODS: A retrospective analysis was performed for the clinical data of 120 children with acute lymphoblastic leukemia or non-Hodgkin lymphoma who underwent 601 times of HDMTX therapy. The adverse effects of various systems were analyzed according to the WHO criteria for the classification of adverse effects of anticancer drugs...
July 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28673073/primary-monosymptomatic-nocturnal-enuresis-and-associated-factors-in-a-referral-continence-clinic-of-abu-dhabi
#12
Salvatore Arena, Mario Patricolo
The aim of the present study was to investigate the prevalence of primary monosymptomatic nocturnal enuresis (PMNE) and its associated factors in a major referral centre for nocturnal enuresis in the City of Abu Dhabi. Children referred to the Pediatric Continence Clinic of Department of Pediatric and Urology Surgery at Al Noor Hospital, Abu Dhabi (UAE), between January 2014 and January 2016 for the suspected diagnosis of NE were considered. The inclusion criteria of our study were: age 5-14 years; full medical history and physical examination; urine dipstick to exclude glycosuria and proteinuria; completion of diagnostic urological work-up; final diagnosis of PMNE...
June 23, 2017: La Pediatria Medica e Chirurgica: Medical and Surgical Pediatrics
https://www.readbyqxmd.com/read/28672087/interventions-for-chronic-kidney-disease-in-people-with-sickle-cell-disease
#13
REVIEW
Noemi Ba Roy, Patricia M Fortin, Katherine R Bull, Carolyn Doree, Marialena Trivella, Sally Hopewell, Lise J Estcourt
BACKGROUND: Sickle cell disease (SCD) is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta-globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Kidney disease is a frequent and potentially severe complication in people with SCD.Chronic kidney disease is defined as abnormalities of kidney structure or function, present for more than three months. Sickle cell nephropathy refers to the spectrum of kidney complications in SCD...
July 3, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28670635/variability-of-urine-parameters-in-children-infected-with-schistosoma-haematobium-in-ukawu-community-onicha-local-government-area-ebonyi-state-nigeria
#14
Juliana Eze Elom, Oliver O Odikamnoro, Agwu Ulu Nnachi, Ifeanyi Ikeh, John O Nkwuda
BACKGROUND: Schistosomiasis, a chronic, debilitating and neglected tropical and sub-tropical water-borne ailment, is highly endemic in Nigeria, especially among primary school children in rural communities. The study on the variability of urine parameters in children infected with Schistosomahaematobium in Ukawu community, Onicha Local Government Area of Ebonyi State, was undertaken. MATERIALS AND METHODS: Urine samples were aseptically collected from 400 primary school children in the community and analysed using chemical reagent strips and sedimentation techniques...
2017: African Journal of Infectious Diseases
https://www.readbyqxmd.com/read/28630858/pulsed-vincristine-therapy-in-steroid-resistant-nephrotic-syndrome
#15
Shenal Thalgahagoda, Shamali Abeyagunawardena, Heshan Jayaweera, Umeshi Ishanthika Karunadasa, Asiri Samantha Abeyagunawardena
Steroid-resistant nephrotic syndrome (SRNS) poses a therapeutic challenge for the paediatric nephrologist. As relentless progression to renal failure occurs with continued proteinuria, such patients will be treated with different cytotoxic medications with variable success rates and side-effects. We present here our findings on administering the anticancer drug vincristine for SRNS patients at a single centre in Sri Lanka. Methods. Between 2002 and 2007, fifty-four children presenting with steroid and cyclophosphamide resistance were treated with vincristine at 1...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28624980/emerging-biomarkers-of-chronic-kidney-disease-in-children
#16
REVIEW
Jason H Greenberg, Aadil Kakajiwala, Chirag R Parikh, Susan Furth
Chronic kidney disease (CKD) has become a significant public health concern, as it is associated with substantial morbidity. Prior research has evaluated multiple novel CKD biomarkers to supplement serum creatinine and proteinuria. The ultimate goal of this research is to find biomarkers that can be used to accurately predict CKD progression and to better time outpatient follow-up, and referral for transplant. Also, an optimal panel of biomarkers can augment the predictive value of proteinuria and serum creatinine by enriching patient enrollment in clinical trials...
June 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28593446/c3-glomerulonephritis-with-a-severe-crescentic-phenotype
#17
Aishwarya Ravindran, Fernando C Fervenza, Richard J H Smith, Sanjeev Sethi
BACKGROUND: C3 glomerulopathy (C3G) is rare type of glomerulonephritis resulting from the glomerular deposition of C3 due to dysregulation of the alternative pathway of complement. It is further subdivided into C3 glomerulonephritis (C3GN) and dense deposit disease (DDD), depending on the ultrastructural features. C3GN usually presents with a membranoproliferative pattern of injury. Crescents may or may not be present. However, we have noted a severe necrotizing and crescentic glomerulonephritis in a small subset of C3GN patients...
June 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28566477/long-term-outcome-of-steroid-resistant-nephrotic-syndrome-in-children
#18
Agnes Trautmann, Sven Schnaidt, Beata S Lipska-Ziętkiewicz, Monica Bodria, Fatih Ozaltin, Francesco Emma, Ali Anarat, Anette Melk, Marta Azocar, Jun Oh, Bassam Saeed, Alaleh Gheisari, Salim Caliskan, Jutta Gellermann, Lina Maria Serna Higuita, Augustina Jankauskiene, Dorota Drozdz, Sevgi Mir, Ayse Balat, Maria Szczepanska, Dusan Paripovic, Alexandra Zurowska, Radovan Bogdanovic, Alev Yilmaz, Bruno Ranchin, Esra Baskin, Ozlem Erdogan, Giuseppe Remuzzi, Agnieszka Firszt-Adamczyk, Elzbieta Kuzma-Mroczkowska, Mieczyslaw Litwin, Luisa Murer, Marcin Tkaczyk, Helena Jardim, Anna Wasilewska, Nikoleta Printza, Kibriya Fidan, Eva Simkova, Halina Borzecka, Hagen Staude, Katharina Hees, Franz Schaefer
We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models...
May 31, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28546440/albuminuria-proteinuria-and-renal-disease-progression-in-children-with-ckd
#19
Dana Y Fuhrman, Michael F Schneider, Katherine M Dell, Tom D Blydt-Hansen, Robert Mak, Jeffrey M Saland, Susan L Furth, Bradley A Warady, Marva M Moxey-Mims, George J Schwartz
BACKGROUND AND OBJECTIVES: The role of albuminuria as an indicator of progression has not been investigated in children with CKD in the absence of diabetes. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Children were enrolled from 49 centers of the CKD in Children study between January of 2005 and March of 2014. Cross-sectional multivariable linear regression (n=647) was used to examine the relationship between urine protein-to-creatinine (UP/C [milligrams per milligram]) and albumin-to-creatinine (ACR [milligrams per gram]) with eGFR (milliliters per minute per 1...
June 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28544686/regulatory-t-cells-and-ctla-4-in-idiopathic-nephrotic-syndrome
#20
Shoji Tsuji, Takahisa Kimata, Sohsaku Yamanouchi, Tetsuya Kitao, Jiro Kino, Chikushi Suruda, Kazunari Kaneko
The pathogenesis of idiopathic nephrotic syndrome (INS) remains unknown. Recently, it was postulated that suppression of regulatory T cells (Treg) leads to massive proteinuria in INS, although there is some controversy. Considering the important role of cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) in Treg-mediated immune suppression, the aim of this study was therefore to clarify the involvement of Treg and CTLA-4 in the pathogenesis of INS. Fifteen patients with INS were enrolled. Their blood was sampled twice, once at onset and once at remission induced by glucocorticoid...
May 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
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