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proteinuria in children

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https://www.readbyqxmd.com/read/28630858/pulsed-vincristine-therapy-in-steroid-resistant-nephrotic-syndrome
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
https://www.readbyqxmd.com/read/28533882/-idiopathic-nephrotic-syndrome-ins-in-children-in-dakar-about-40-cases
#7
Younoussa Keita, Ahmed Tall Lemrabott, Assane Sylla, Babacar Niang, El Hadji Fary Ka, Chérif Mohamed Dial, Aliou Abdoulaye Ndongo, Amadou Sow, Claude Moreira, Abdou Niang, Ousmane Ndiaye, Boucar Diouf, Mouhamadou Guélaye Sall
INTRODUCTION: This study aimed to analyze the diagnostic, therapeutic, and evolutionary features of nephrosis in children in a pediatric department in Dakar. METHODS: The study was carried out in the Department of Pediatrics at the Aristide Le Dantec Hospital. We conducted a retrospective study over a period of 3 years from 1 January 2012 to 31 December 2014. All patients aged 2-12 years with idiopathic nephrotic syndrome were included in the study. RESULTS: Forty cases of nephrosis were collected, that is to say a prevalence of 23% among patients with kidney disease treated in the Department of Pediatrics...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28515415/evaluation-of-mycophenolate-mofetil-and-low-dose-steroid-combined-therapy-in-moderately-severe-henoch-sch%C3%A3-nlein-purpura-nephritis
#8
Zhihong Lu, Junfeng Song, Jianhua Mao, Yonghui Xia, Caiyun Wang
BACKGROUND The most appropriate management of Henoch-Schönlein Purpura (HSP) nephritis with nephrotic-range proteinuria remains uncertain. The aim of this study was to evaluate the clinical therapeutic effects of mycophenolate mofetil and low-dose steroid in Henoch-Schönlein purpura nephritis (HSPN) with nephrotic-range proteinuria and pathological classification less than IV in children. MATERIAL AND METHODS The clinical effects of MMF and low-dose steroid therapy were studied in children with Henoch-Schönlein purpura nephritis manifested with nephrotic-range proteinuria, normal kidney function, and <50% crescents or sclerosing lesions on renal biopsy...
May 18, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28499374/clinical-improvement-of-renal-amyloidosis-in-a-patient-with-systemic-onset-juvenile-idiopathic-arthritis-who-received-tocilizumab-treatment-a-case-report-and-literature-review
#9
Songkiat Chantarogh, Soamarat Vilaiyuk, Thipwimol Tim-Aroon, Suchin Worawichawong
BACKGROUND: Juvenile idiopathic arthritis (JIA) is a common rheumatic disease in children and adolescents. Although JIA may cause secondary amyloidosis, this is a rare complication in patients with JIA and other rheumatic diseases. Many previous studies have revealed that common heterozygous or homozygous mutations in the MEFV gene are associated with systemic-onset JIA (SJIA). CASE PRESENTATION: We herein report a case involving a 19-year-old female patient with difficult-to-control SJIA...
May 12, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28496353/early-manifestations-of-renal-disease-in-patients-with-tuberous-sclerosis-complex
#10
Laura Malaga-Dieguez, Robert Spencer, Laura J Pehrson, Suzanne Vento, Kimberly Menzer, Orrin Devinsky, Howard Trachtman
OBJECTIVES: Renal manifestations are the second most significant cause of morbidity and mortality in patients with tuberous sclerosis complex (TSC), and include renal cysts, angiomyolipomas, fat-poor lesions, and malignant tumors. These lesions begin in childhood and often lead to chronic kidney disease (CKD). Little is known on the incidence of early modifiable risk factors of CKD, such as proteinuria and hypertension, or subtle decreases in glomerular filtration rate that correspond to the early stages of CKD in children with TSC...
2017: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/28477260/atypical-thymic-carcinoid-manifesting-with-nephrotic-range-proteinuria-in-a-7-year-old-boy
#11
Jolanta Soltysiak, Danuta Ostalska-Nowicka, Katarzyna Zaorska, Karolina Sterzyńska, Katarzyna Derwich, Jacek Zachwieja
BACKGROUND: Nephrotic-range proteinuria as a paraneoplastic syndrome (PNS) is an exceptional presentation, especially in children. It is usually associated with hematologic malignancies. Solid tumors are very rare causes of proteinuria. CASE-DIAGNOSIS/TREATMENT: We present the case of a 7-year-old boy with an extremely rare atypical thymic carcinoid accompanied by nephrotic-range proteinuria as PNS. The kidney biopsy was consistent with minimal change disease (MCD)...
May 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28463080/lupus-glomerulonephritis-in-788-chinese-children-a-multi-centre-clinical-and-histopathological-analysis-based-on-549-renal-biopsies
#12
Si-Yan Jin, Dan-Lin Huang, Xi-Qiang Dang, Zhu-Wen Yi
BACKGROUND: System lupus erythematosus (SLE) is a severe multisystem autoimmune disease. OBJECTIVE: To describe the clinical and pathological features, treatment, and renal outcome in children under 18 years with lupus nephritis (LN). METHODS: The study was undertaken by a questionnaire completed in 26 Grade 3A hospitals' paediatric renal units in China. The study comprised 788 children (619 girls, 169 boys) diagnosed with SLE by the American College of Rheumatology criteria (1997) during 2005-2010...
May 2, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28449720/evolution-of-blood-pressure-in-children-with-congenital-and-acquired-solitary-functioning-kidney
#13
Riccardo Lubrano, Isotta Gentile, Raffaele Falsaperla, Giovanna Vitaliti, Alessia Marcellino, Marco Elli
BACKGROUND: It is not yet clear if blood pressure and renal function changes evolve differently in children with a congenital or acquired solitary functioning kidney. This study aims to assess if there are any differences between these two types of solitary kidney patients. METHODS: Current research is a retrospective study assessing the evolution of glomerular filtration rate, proteinuria, and blood pressure in clinical records of 55 children with a solitary functioning kidney (37 congenital and 18 acquired)...
April 27, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28441404/angiopoietin-like-4-and-minimal-change-disease
#14
Gabriel Cara-Fuentes, Alfons Segarra, Cecilia Silva-Sanchez, Heiman Wang, Miguel A Lanaspa, Richard J Johnson, Eduardo H Garin
BACKGROUND: Minimal Change Disease (MCD) is the most common type of nephrotic syndrome in children. Angiopoietin-like-4 (Angplt4) has been proposed as mediator of proteinuria in MCD. The aim of this study was to evaluate the role of Angptl4 as a biomarker in MCD. METHODS: Patients with biopsy-proven primary MCD, focal segmental glomerulosclerosis, membranous nephropathy (60, 52 and 52 respectively) and 18 control subjects had urinary and serum Angptl4 measured by Elisa...
2017: PloS One
https://www.readbyqxmd.com/read/28436233/cyclosporine-based-immunosuppressive-therapy-for-patients-with-steroid-resistant-focal-segmental-glomerulosclerosis-a-meta-analysis
#15
Yuan-Yow Chiou, Yi-Che Lee, Mei-Ju Chen
OBJECTIVE: Focal segmental glomerulosclerosis (FSGS) is a leading cause of end-stage kidney disease that requires immunosuppressive treatment as therapy. Few studies have been specifically designed to assess the efficacy of cyclosporine (CSA) in patients with steroid-resistant FSGS. This study investigated the efficacy of CSA-based therapy in steroid-resistant FSGS. METHODS: Medline, Cochrane, EMBASE, and Google Scholar databases were searched through April 30, 2014 using the keywords "cyclosporine", "steroid-resistant", "focal segmental glomerulosclerosis", and "FSGS"...
May 31, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28407818/-clinical-and-pathological-features-and-the-misdiagnosis-of-childhood-alport-syndrome-a-retrospective-analysis-of-91-cases
#16
Yan-Zhen Chen, Liang-Zhong Sun, Hai-Yan Wang, Xiao-Yun Jiang, Ying Mo, Zhi-Hui Yue, Hua-Mu Chen, Ting Liu, Hong-Rong Lin
OBJECTIVE: To explore the clinical and pathological features and the diagnosis of childhood Alport syndrome (AS). METHODS: A retrospective analysis was performed on clinical data of 91 children with AS. RESULTS: Hematuria was observed in all 91 patients, of whom 86 were accompanied with proteinuria. Sixty-one children with X-Linked AS (XL-AS) had positive family history. Renal biopsy was performed on 82 children. Mild to moderate mesangial proliferation was observed in 74 cases...
April 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28406083/ambulatory-arterial-stiffness-index-blood-pressure-variability-and-nocturnal-blood-pressure-dip-in-children-with-iga-and-henoch-sch%C3%A3-nlein-nephropathy%C3%A2
#17
Piotr Skrzypczyk, Małgorzata Mizerska-Wasiak, Barbara Jerszow, Paweł Ruszczykowski, Małgorzata Pańczyk-Tomaszewska
AIM: Evaluation of mean blood pressure values, ambulatory arterial stiffness index (AASI), pulse pressure (PP), blood pressure variability (BPV), and circadian blood pressure rhythm using ambulatory blood pressure monitoring (ABPM) in children with IgA nephropathy and Henoch-Schönlein nephropathy (IgAN/HSN). MATERIAL AND METHODS: In 48 children (29 with IgAN, 19 with HSN) aged 14.04 ± 3.76 years, we evaluated retrospectively 24-hour systolic and diastolic BP (24hSBP, 24hDBP), AASI, PP, systolic and diastolic BPV, and nocturnal BP dip by ABPM, body mass index (BMI) Z-score, medications, and biochemical parameters...
April 13, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28405891/renalase-in-children-with-glomerular-kidney-diseases
#18
Piotr Skrzypczyk, Joanna Przychodzień, Małgorzata Mizerska-Wasiak, Elżbieta Kuźma-Mroczkowska, Magdalena Okarska-Napierała, Elżbieta Górska, Anna Stelmaszczyk-Emmel, Urszula Demkow, Małgorzata Pańczyk-Tomaszewska
Studies suggest that renalase, a renal catecholamine-inactivating enzyme, plays a major role in the pathogenesis of kidney and cardiovascular diseases in adults. This study seeks to determine the role of renalase in children with glomerular kidney diseases. We evaluated the serum renalase, arterial stiffness, intima-media thickness, blood pressure, and clinical and biochemical parameters in 78 children (11.9 ± 4.6 years of age) with glomerulopathies such as idiopathic nephrotic syndrome (40 cases), IgA nephropathy (12 cases), Henoch-Schönlein nephropathy (12 cases), and other glomerulopathies (14 cases)...
April 13, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28405841/focal-segmental-glomerulosclerosis-and-medullary-nephrocalcinosis-in-children-with-adck4-mutations
#19
Eujin Park, Hee Gyung Kang, Young Hun Choi, Kyoung Bun Lee, Kyung Chul Moon, Hyeon Joo Jeong, Michio Nagata, Hae Il Cheong
BACKGROUND: Mutations in the AarF domain containing kinase 4 gene (ADCK4), one of the novel genes causing steroid-resistant nephrotic syndrome (SRNS), usually manifest as isolated adolescent-onset focal segmental glomerulosclerosis (FSGS). ADCK4 interacts with components of the coenzyme Q10 (CoQ10) biosynthesis pathway. METHODS: The incidence and phenotypes of patients with ADCK4 mutations were investigated in a cohort of Korean pediatric patients with SRNS. RESULTS: Among the 53 patients enrolled in the study the incidence of ADCK4-associated FSGS was 7...
April 12, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28391337/managing-glomerular-disease-in-pregnancy
#20
Michelle A Hladunewich, Kate Bramham, Belinda Jim, Sharon Maynard
Glomerular disease, especially lupus nephritis, is common in young women of childbearing age. As such, practicing nephrologists must have adequate knowledge and expertise to prepare these women for pregnancy, to diagnose and manage the numerous pregnancy-associated complications that are common in this vulnerable patient population and finally to support these young women who often struggle to manage both their young children along with an often onerous chronic disease. In this article we will review the pre-pregnancy counseling these women should ideally receive to allow them to make an informed decision about proceeding with a pregnancy, an approach to the diagnosis of pregnancy-associated worsening of kidney function and proteinuria as well as pregnancy-safe management strategies, including immunosuppression, antihypertensive agents and other medications necessary for the management of nephrotic syndrome...
January 1, 2017: Nephrology, Dialysis, Transplantation
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