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Nephrotic Syndrome in children

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https://www.readbyqxmd.com/read/28921387/analysis-of-24-genes-reveals-a-monogenic-cause-in-11-1-of-cases-with-steroid-resistant-nephrotic-syndrome-at-a-single-center
#1
Weizhen Tan, Svjetlana Lovric, Shazia Ashraf, Jia Rao, David Schapiro, Merlin Airik, Shirlee Shril, Heon Yung Gee, Michelle Baum, Ghaleb Daouk, Michael A Ferguson, Nancy Rodig, Michael J G Somers, Deborah R Stein, Asaf Vivante, Jillian K Warejko, Eugen Widmeier, Friedhelm Hildebrandt
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is the second most frequent cause of end-stage renal disease (ESRD) among patients manifesting at under 25 years of age. We performed mutation analysis using a high-throughput PCR-based microfluidic technology in 24 single-gene causes of SRNS in a cohort of 72 families, who presented with SRNS before the age of 25 years. METHODS: Within an 18-month interval, we obtained DNA samples, pedigree information, and clinical information from 77 consecutive children with SRNS from 72 different families seen at Boston Children's Hospital (BCH)...
September 18, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28915908/advances-in-paediatrics-in-2016-current-practices-and-challenges-in-allergy-autoimmune-diseases-cardiology-endocrinology-gastroenterology-infectious-diseases-neonatology-nephrology-neurology-nutrition-pulmonology
#2
REVIEW
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Silvia Montella, Sergio Bernasconi
This review reports main progresses in various pediatric issues published in Italian Journal of Pediatrics and in international journals in 2016. New insights in clinical features or complications of several disorders may be useful for our better understanding. They comprise severe asthma, changing features of lupus erythematosus from birth to adolescence, celiac disease, functional gastrointestinal disorders, Moebius syndrome, recurrent pneumonia. Risk factors for congenital heart defects, Kawasaki disease have been widely investigated...
September 16, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28914167/nephrotic-syndrome-in-infants-and-children-pathophysiology-and-management
#3
Mallory L Downie, Claire Gallibois, Rulan S Parekh, Damien G Noone
Nephrotic syndrome is defined by nephrotic-range proteinuria (≥40 mg/m(2)/hour or urine protein/creatinine ratio ≥200 mg/mL or 3+ protein on urine dipstick), hypoalbuminaemia (<25 g/L) and oedema. This review focuses on the classification, epidemiology, pathophysiology, management strategies and prognosis of idiopathic nephrotic syndrome of childhood, and includes a brief overview of the congenital forms.
September 15, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28904877/long-term-repeated-rituximab-treatment-for-childhood-steroid-dependent-nephrotic-syndrome
#4
Ji Hyun Kim, Eujin Park, Hye Sun Hyun, Myung Hyun Cho, Yo Han Ahn, Hyun Jin Choi, Hee Gyung Kang, Il-Soo Ha, Hae Il Cheong
BACKGROUND: Rituximab (RTX) can be used as a rescue therapy for steroid-dependent nephrotic syndrome (SDNS). However, the efficacy and safety of long-term, repeated use of RTX are not established. This study was conducted to assess the efficacy and safety of long-term, repeated RTX treatment in children. METHODS: Eighteen consecutive child patients with SDNS who were treated with three or more cycles of RTX for one year or longer were recruited, and their medical records were retrospectively reviewed...
September 2017: Kidney Research and Clinical Practice
https://www.readbyqxmd.com/read/28904681/clinicopathological-findings-and-outcome-of-lupus-nephritis-in-tunisian-children-a-review-of-43-patients
#5
Hela Jebali, Meriam Hajji, Lamia Rais, Fethi Ben Hamida, Soumaya Beji, Mohammed Karim Zouaghi
We report clinical and renal histological data, treatment modalities and outcome of 43 Tunisian children with biopsy-proven lupus nephritis seen over a 23-year period. There were 39 girls and 4 boys with a mean age of 12.5 years at diagnosis of lupus nephritis and followed for a mean period of 77 months. Renal symptoms included urinary abnormalities in all patients, hypertension in 40% of cases, nephrotic syndrome in 60% of cases and renal failure in 25% of cases. Class IV and class III nephritis were observed in 48...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28888057/glucocorticoid-treatment-earlier-in-childhood-and-adolescence-show-dose-response-associations-with-diurnal-cortisol-levels
#6
Martin Vestergaard, Sara K Holm, Peter Uldall, Hartwig R Siebner, Olaf B Paulson, William F C Baaré, Kathrine S Madsen
Heightened levels of glucocorticoids in children and adolescents have previously been linked to prolonged changes in the diurnal regulation of the stress-hormone cortisol, a glucocorticoid regulated by the hypothalamic-pituitary-adrenal-axis (HPA-axis). To address this question, we examined the salivary cortisol awakening response (CAR) and daily cortisol output in 36 children and adolescents (25 girls/11 boys) aged 7-16 years previously treated with glucocorticoids for nephrotic syndrome or rheumatic disorder and 36 healthy controls...
September 9, 2017: Developmental Psychobiology
https://www.readbyqxmd.com/read/28884421/assessing-responsiveness-over-time-of-the-promis-%C3%A2-pediatric-symptom-and-function-measures-in-cancer-nephrotic-syndrome-and-sickle-cell-disease
#7
Bryce B Reeve, Lloyd J Edwards, Byron C Jaeger, Pamela S Hinds, Carlton Dampier, Debbie S Gipson, David T Selewski, Jonathan P Troost, David Thissen, Vaughn Barry, Heather E Gross, Darren A DeWalt
PURPOSE: Previous studies provided evidence for the validity of the PROMIS Pediatric measures in cross-sectional studies. This study evaluated the ability of the PROMIS Pediatric measures to detect change over time in children and adolescents with cancer, nephrotic syndrome (NS), or sickle cell disease (SCD). METHODS: Participants (8-17 years) completed measures of fatigue, pain interference, anger, anxiety, depressive symptoms, mobility, upper extremity, and peer relationships at three or four time points (T1-T4)...
September 7, 2017: Quality of Life Research
https://www.readbyqxmd.com/read/28879428/difficult-to-treat-idiopathic-nephrotic-syndrome-established-drugs-open-questions-and-future-options
#8
REVIEW
Markus J Kemper, Lisa Valentin, Michael van Husen
The idiopathic nephrotic syndrome in childhood can be classified according to the International Study of Kidney Disease in Children (ISKDC) based on the response to steroids. Typically, steroid-sensitive nephrotic syndrome (SSNS) is characterised by minimal changes in disease (MCD) histology, whereas in steroid-resistant nephrotic syndrome (SRNS) focal segmental glomerulosclerosis (FSGS) is the most prevalent lesion. Patients with SSNS may develop frequent relapses and/or steroid dependency, which can be difficult to treat...
September 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28878838/correlation-between-idiopathic-nephrotic-syndrome-and-atopy-in-children-short-review
#9
Elena Camelia Berghea, Mihaela Balgradean, Ionela-Loredana Popa
The idiopathic nephrotic syndrome is a common chronic kidney diseases in children defined by the association of massive proteinuria and hypoalbuminemia in a relapsing/remission course, with histological aspect of minimal changes (also called minimal change disease) in the majority of the cases, but its pathogenesis remains not very well known. Clinical and immunological studies have consistently shown a relationship between atopic diathesis, immunoglobulin E and cytokines involved in immunoglobulin E synthesis and idiopathic nephrotic syndrome...
January 2017: Mædica
https://www.readbyqxmd.com/read/28870123/bayesian-treatment-comparison-using-parametric-mixture-priors-computed-from-elicited-histograms
#10
Peter F Thall, Moreno Ursino, Véronique Baudouin, Corinne Alberti, Sarah Zohar
A Bayesian methodology is proposed for constructing a parametric prior on two treatment effect parameters, based on graphical information elicited from a group of expert physicians. The motivating application is a 70-patient randomized trial to compare two treatments for idiopathic nephrotic syndrome in children. The methodology relies on histograms of the treatment parameters constructed manually by each physician, applying the method of Johnson et al. (2010). For each physician, a marginal prior for each treatment parameter characterized by location and precision hyperparameters is fit to the elicited histogram...
January 1, 2017: Statistical Methods in Medical Research
https://www.readbyqxmd.com/read/28868299/tubuloreticular-inclusions-in-the-absence-of-systemic-lupus-erythematosus-and-hiv-infection-a-report-of-three-pediatric-cases
#11
Ayah Elmaghrabi, Elizabeth Brown, Ei Khin, Jared Hassler, Allen R Hendricks
Tubuloreticular inclusions (TRIs) are subcellular structures located within the cisternae of endoplasmic reticulum. Formation of TRIs has been linked to the exposure of excess interferon (IFN), either from endogenous or exogenous sources. In renal disease, TRIs have been most commonly associated with systemic lupus erythematosus (SLE), and human immunodeficiency virus-associated nephropathy (HIVAN). Case reports of patients with renal biopsies showing TRIs without underlying SLE or HIV are infrequent in adults, and to our knowledge none have been reported in children...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28864927/cost-analysis-on-the-use-of-rituximab-and-calcineurin-inhibitors-in-children-and-adolescents-with-steroid-dependent-nephrotic-syndrome
#12
Franca Iorember, Diego Aviles, Mahmoud Kallash, Oluwatoyin Bamgbola
BACKGROUND: Rituximab (RTX) is increasingly being used in place of calcineurin inhibitors (CNI) in pediatric patients with steroid-dependent nephrotic syndrome (SDNS). However, despite its favorable safety profile, its unit cost is prohibitive. We therefore compared the healthcare costs associated with the use of both agents in a retrospective cohort. METHODS: This study was a retrospective analysis of data retrieved from the medical charts and electronic databases of pediatric patients (age range 2-18 years) with SDNS who were treated with either CNI or RTX from January 2008 to December 2012 at Children's Hospital of New Orleans, Louisiana...
September 1, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28859624/living-kidney-transplantation-between-brothers-with-unrecognized-renal-amyloidosis-as-the-first-manifestation-of-familial-mediterranean-fever-a-case-report
#13
Ramón Peces, Sara Afonso, Carlos Peces, Julián Nevado, Rafael Selgas
BACKGROUND: Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent episodes of fever and polyserositis and by the onset of reactive amyloid-associated amyloidosis. Amyloidosis due to familial Mediterranean fever can lead to end-stage renal disease, culminating in kidney transplantation for some patients. In this study, we report the clinical outcome of two brothers with familial Mediterranean fever who were the inadvertent donor and recipient, respectively, of a kidney...
August 31, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28856214/the-roche-total-mycophenolic-acid%C3%A2-assay-an-application-protocol-for-the-abx-pentra-400-analyzer-and-comparison-with-lc-ms-in-children-with-idiopathic-nephrotic-syndrome
#14
François Parant, Bruno Ranchin, Marie-Claude Gagnieu
BACKGROUND: For TDM of mycophenolate acid (MPA), the Roche Total Mycophenolic Acid® assay based on the inhibition of recombinant inosine monophosphate dehydrogenase (IMPDH) has been shown to be a simple and reliable alternative to chromatographic methods. We have adapted this assay on the ABX Pentra 400 analyzer (HORIBA). OBJECTIVE: To investigate the analytical performances of the Roche Total Mycophenolic Acid® assay on the ABX Pentra 400 and to compare it to an LC-MS method using samples from children with nephrotic syndrome treated with mycophenolate mofetil (MMF)...
April 2017: Pract Lab Med
https://www.readbyqxmd.com/read/28852159/loss-of-the-podocyte-glucocorticoid-receptor-exacerbates-proteinuria-after-injury
#15
Han Zhou, Xuefei Tian, Alda Tufro, Gilbert Moeckel, Shuta Ishibe, Julie Goodwin
Nephrotic syndrome is a common disorder in adults and children whose etiology is largely unknown. Glucocorticoids remain the mainstay of therapy in most cases, though their mechanism of action remains poorly understood. Emerging evidence suggests that immunomodulatory therapies used in nephrotic syndrome directly target the podocytes. To study how steroids directly affect the podocytes in the treatment of proteinuria, we created a mouse model with podocyte-specific deletion of the glucocorticoid receptor. The podocyte-specific glucocorticoid receptor (GR) knockout mice had similar renal function and protein excretion compared to wild type...
August 29, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28846194/spectrum-of-biopsy-proven-renal-disease-in-south-asian-children-two-decades-at-a-tropical-tertiary-care-centre
#16
Anjali Mohapatra, Shailesh Kakde, Vellaichamy M Annapandian, Anna T Valson, Neelaveni Duhli, Anila Korula, Smita Mary Matthai, Anna B Pulimood, Vinoi G David, Suceena Alexander, Shibu Jacob, Santosh Varughese, Basu Gopal, Tamilarasi Veerasamy, George T John
AIM: We report findings from a large single centre paediatric renal biopsy cohort in South Asia. METHODS: We analyzed all renal biopsies performed on children aged ≤18 years between 1996 and 2015 at our centre. The clinical characteristics and histological diagnosis pertaining to each case, distribution of renal diseases in children with various clinical presentations, and changes in the pattern of kidney disease during the study period were analyzed. RESULTS: A total of 1740 paediatric kidney biopsies were performed during the study period...
August 28, 2017: Nephrology
https://www.readbyqxmd.com/read/28835233/responsiveness-of-the-promis%C3%A2-measures-to-changes-in-disease-status-among-pediatric-nephrotic-syndrome-patients-a-midwest-pediatric-nephrology-consortium-study
#17
David T Selewski, Jonathan P Troost, Danyelle Cummings, Susan F Massengill, Rasheed A Gbadegesin, Larry A Greenbaum, Ibrahim F Shatat, Yi Cai, Gaurav Kapur, Diane Hebert, Michael J Somers, Howard Trachtman, Priya Pais, Michael E Seifert, Jens Goebel, Christine B Sethna, John D Mahan, Heather E Gross, Emily Herreshoff, Yang Liu, Noelle E Carlozzi, Bryce B Reeve, Darren A DeWalt, Debbie S Gipson
BACKGROUND: Nephrotic syndrome represents a condition in pediatric nephrology typified by a relapsing and remitting course, proteinuria and the presence of edema. The PROMIS measures have previously been studied and validated in cross-sectional studies of children with nephrotic syndrome. This study was designed to longitudinally validate the PROMIS measures in pediatric nephrotic syndrome. METHODS: One hundred twenty seven children with nephrotic syndrome between the ages of 8 and 17 years participated in this prospective cohort study...
August 23, 2017: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/28827494/urinary-tract-infection-in-children-with-nephrotic-syndrome
#18
Upma Narain, Arvind Gupta
BACKGROUND: The occurrence of urinary tract infection (UTI) in nephrotic syndrome has been reported. However, the only causes for the infection reported to date are bacteria and prior reported series did not identify yeast or fungi as causative organisms. METHODS: A retrospective study of 2880 children with nephrotic syndrome was made to determine the incidence of UTI, predisposing factor and bacterial and fungal etiologies. RESULTS: Between January 2000 and November 2016 we identified that 15% of the children developed urinary tract infection...
August 17, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28822445/-progress-in-the-treatment-of-nephrotic-syndrome-in-children
#19
N Guan
No abstract text is available yet for this article.
August 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28819780/preliminary-research-on-syndrome-types-of-chinese-medicine-in-children-with-primary-nephrotic-syndrome
#20
Wen Sun, Jian Yu, Gu-Lan Zeng, Bing-Feng Zhang
OBJECTIVE: To provide an objective reference for the syndrome types of Chinese medicine (CM) associated with pediatric primary nephrotic syndrome (PNS). METHODS: A cross-sectional study was performed. Data on clinical symptoms, CM syndrome types, biochemical indices, and medications used were collected from 98 children with PNS. Then, the correlation between CM syndromes and biochemical indices, as well as medications used, was analyzed. RESULTS: The four most common symptoms in children with PNS were brown urine, red tongue, excessive sweating, and swelling of the face and limbs...
August 17, 2017: Chinese Journal of Integrative Medicine
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