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nephrotic syndrome, pediatric

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https://www.readbyqxmd.com/read/28792718/mdr-1-and-cyp3a5-polymorphisms-in-pediatric-idiopathic-nephrotic-syndrome-impact-on-susceptibility-and-response-to-steroids-preliminary-results
#1
Amira Moussa, Sameh Mabrouk, Haithem Hamdouni, Maroua Ajmi, Miniar Tfifha, Asma Omezzine, Saoussen Abroug, Ali Bouslama
BACKGROUND: Oral steroid treatment is the first line of therapy for childhood nephrotic syndrome (NS). However, resistance to this treatment has been observed in some patients. Here, we investigated the association of two steroid metabolism-related genes with susceptibility to childhood NS and the steroid response. METHODS: We genotyped the single nucleotide polymorphisms (SNP) of MDR-1 [C1236T (rs1128503), G2677T/A (rs2032582), and C3435T (rs1045642)] and the CYP3A5 gene (A6986G) in 63 NS patients and 110 age and gender matched controls by PCR-RFLP...
July 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28774288/the-impact-of-igm-deposits-on-the-outcome-of-nephrotic-syndrome-in-children
#2
Sandra Juozapaite, Rimante Cerkauskiene, Arvydas Laurinavicius, Augustina Jankauskiene
BACKGROUND: The significance of IgM deposits in glomerular mesangium has been controversial since they were first described due to the variations in the both the definitions used and described impact on clinical outcome. The aim of our study was to evaluate the significance of the IgM deposits in the glomerular mesangium for outcomes of nephrotic syndrome (NS) in children. METHODS: Forty-five children with NS who underwent renal biopsy at tertiary pediatric hospital from January 1st, 2000 to December 31st, 2015 and the pathology diagnosis of minimal change disease, focal segmental glomerulosclerosis and mesangial hypercellularity (MH) were retrospectively analyzed...
August 3, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28748888/childhood-idiopathic-steroid-resistant-nephrotic-syndrome-at-a-single-center-in-khartoum
#3
El-Tigani M A Ali, Hanna F K Makki, Mohamed B Abdelraheem, Salwa O Makke, Rashid A Allidir
Prevalence, clinicopathological features, and outcome of childhood idiopathic steroid-resistant nephrotic syndrome (ISRNS) vary in different countries. We report on these parameters in a single center in Khartoum. We retrospectively reviewed all the records of children with idiopathic nephrotic syndrome (INS) followed up in the pediatric renal unit, Soba Hospital, Khartoum between 2001 and 2012. ISRNS was defined as no remission within four weeks of daily prednisolone at a dose of 60 mg/m2. In 430 children with INS 130 (28%) had SRNS with a mean age of 7...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28725977/the-effect-of-vitamin-d-and-calcium-supplementation-in-pediatric-steroid-sensitive-nephrotic-syndrome
#4
Sushmita Banerjee, Surupa Basu, Ananda Sen, Jayati Sengupta
BACKGROUND: Low serum levels of total 25-hydroxycholecalciferol (25(OH)D) occur in nephrotic syndrome (NS). We aimed to assess the effects of vitamin D3 and calcium supplementation on 25(OH)D levels, bone mineralization, and NS relapse rate in children with steroid-sensitive NS. METHODS: A randomized controlled trial (RCT) was performed in children with steroid-sensitive NS. The treatment group received vitamin D3 (60,000 IU orally, weekly for 4 weeks) and calcium supplements (500 to 1,000 mg/day for 3 months) after achieving NS remission...
July 19, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28724397/partial-uniparental-isodisomy-of-chromosome-16-unmasks-a-deleterious-biallelic-mutation-in-ift140-that-causes-mainzer-saldino-syndrome
#5
Benjamin M Helm, Jason R Willer, Azita Sadeghpour, Christelle Golzio, Eric Crouch, Samantha Schrier Vergano, Nicholas Katsanis, Erica E Davis
BACKGROUND: The ciliopathies represent an umbrella group of >50 clinical entities that share both clinical features and molecular etiology underscored by structural and functional defects of the primary cilium. Despite the advances in gene discovery, this group of entities continues to pose a diagnostic challenge, in part due to significant genetic and phenotypic heterogeneity and variability. We consulted a pediatric case from asymptomatic, non-consanguineous parents who presented as a suspected ciliopathy due to a constellation of retinal, renal, and skeletal findings...
July 19, 2017: Human Genomics
https://www.readbyqxmd.com/read/28717937/clinical-presentation-and-outcomes-of-childhood-onset-membranous-lupus-nephritis
#6
Maria Pereira, Eyal Muscal, Karen Eldin, M John Hicks, Anna Carmela P Sagcal-Gironella, Marietta DeGuzman, Scott E Wenderfer
BACKGROUND: Best practices for managing childhood-onset membranous lupus nephritis (MLN) are not yet established. Most studies involve primarily or exclusively adult cohorts or pediatric cohorts with combinations of pure or mixed membranous and proliferative nephritis. METHODS: We performed a single-center cohort study of consecutively diagnosed children with pure MLN from 1990 and 2016. Patients received care in Houston, Texas, one of the most diverse metropolitan areas in North America...
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28699616/levamisole-in-frequently-relapsing-and-steroid-dependent-nephrotic-syndrome
#7
Evangeline Mary Kiruba Samuel, Sriram Krishnamurthy, Singanamalla Bhanudeep, Sravani Muske
OBJECTIVE: To evaluate the efficacy of levamisole in children with frequently relapsing nephrotic syndrome (FRNS) and steroid dependent nephrotic syndrome (SDNS) when administered on an alternate day ('initial therapy' in all cases) or daily basis ('rescue therapy' in whom alternate day therapy failed). METHODS: The records of 95 children (age 1-18y) with FRNS and SDNS, who were treated at the Pediatric nephrology clinic, and received levamisole therapy (maximum 2 years duration, between 2010-2013) with a follow-up period of minimum1 year, were included...
July 11, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28699031/effectiveness-and-nephrotoxicity-of-a-2-year-medium-dose-of-cyclosporine-in-pediatric-patients-with-steroid-dependent-nephrotic-syndrome-determination-of-the-need-for-follow-up-kidney-biopsy
#8
Yoshiyuki Kuroyanagi, Yoshimitsu Gotoh, Katsuaki Kasahara, China Nagano, Naoya Fujita, Satoshi Yamakawa, Masaki Yamamoto, Asami Takeda, Osamu Uemura
BACKGROUND: High dose of cyclosporine (CyA) for ≥2 years in children with steroid-dependent nephrotic syndrome (SDNS) increases the risk for nephropathy. Considering this, risk can be lowered with lower doses of CyA; we evaluated the effects of a medium dose of CyA, with target serum level, C2, of 450 ng/ml, over a 2-year period of observation, to determine the need for follow-up kidney biopsy. METHODS: We retrospectively evaluated C2 levels in 38 patients (17 males, 5...
July 11, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28613279/nox-mediated-impairment-of-pdgf-induced-dna-synthesis-in-peripheral-blood-lymphocytes-of-children-with-idiopathic-nephrotic-syndrome
#9
Amal Al-Eisa, Gursev S Dhaunsi
BackgroundCellular oxidative stress, inflammatory responses, and immunogenic events are involved in pathogenesis of idiopathic nephrotic syndrome (INS); however, the exact mechanism remains unknown. We examined NADPH oxidase (NOX) activity and platelet-derived growth factor (PDGF)-induced DNA synthesis in peripheral blood lymphocytes (PBL) of patients with INS.MethodsPBL from 15 patients with INS and 15 age- and gender-matched controls were isolated, and enzyme activities of NOX, catalase, and superoxide dismutase (SOD) were measured along with the assay of malondialdehyde levels and bromo-deoxyuridine incorporation...
June 14, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28607687/glomerular-filtration-rate-trends-during-follow-up-in-children-with-steroid-sensitive-nephrotic-syndrome
#10
Sulaiman Alsaidi, Daniel Wagner, Silviu Grisaru, Julian Midgley, Lorraine Hamiwka, Andrew Wade, Alberto Nettel-Aguirre, Susan Samuel
BACKGROUND: Overall prognosis of children with steroid-sensitive nephrotic syndrome (SSNS) is regarded as generally favorable. However, only a few recent studies have evaluated changes in kidney function and blood pressure over time in children with SSNS. OBJECTIVES: We describe clinical features of SSNS patients and characterize changes in calculated estimated glomerular filtration rate (eGFR) and use of antihypertensive medications during follow-up. DESIGN: This is a retrospective cohort study...
2017: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/28600697/neurotoxicity-of-cyclosporine%C3%A2-a-in-children-with-steroid-resistant-nephrotic-syndrome-is-cytotoxic-edema-really-an-unfavorable-predictor-of-permanent-neurological-damage
#11
Danica Batinić, Danko Milošević, Boris Filipović-Grčić, Marija Topalović-Grković, Nina Barišić, Daniel Turudić
BACKGROUND: Cyclosporine A-associated neurotoxicity has been reported mainly after organ transplantation. Only a small number of children with steroid-resistant nephrotic syndrome and cyclosporine A-associated neurotoxicity have been reported. PATIENTS: We report three children, aged 4, 11, and 15, with steroid-resistant nephrotic syndrome and cyclosporine A-associated neurotoxicity. In two of the patients, primary diagnosis was idiopathic nephrotic syndrome, and in one it was IgA nephropathy...
June 9, 2017: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/28592406/factors-influencing-practice-variation-in-the-management-of-nephrotic-syndrome-a-qualitative-study-of-pediatric-nephrology-care-providers
#12
Susan M Samuel, Rachel Flynn, Michael Zappitelli, Allison Dart, Rulan Parekh, Maury Pinsk, Cherry Mammen, Andrew Wade, Shannon D Scott
BACKGROUND: Treatment protocols for childhood nephrotic syndrome are highly variable between providers and care centres. We conducted a qualitative study to understand the complex multilevel processes that lead to practice variation and influence provider management of nephrotic syndrome. METHODS: Focus groups with multidisciplinary pediatric nephrology care providers (n = 67) from 10 Canadian pediatric nephrology centres that had more than 1 pediatric nephrologist were conducted between September 2013 and April 2015...
June 7, 2017: CMAJ Open
https://www.readbyqxmd.com/read/28540898/assessment-of-quality-of-life-in-children-with-nephrotic-syndrome-at-a-teaching-hospital-in-south-india
#13
Sonia Agrawal, Sriram Krishnamurthy, Bijaya Nanda Naik
This study was conducted to assess the quality of life (QOL) in children between 2 and 18 years of age with primary idiopathic nephrotic syndrome (NS) using Pediatric Quality of Life Inventory (PedsQL 4.0 Generic Core Scales). This cross-sectional comparative study was conducted at a tertiary care hospital in South India between December 2014 and February 2015. In this questionnaire-based study, 50 children with primary idiopathic NS and an equal number of age-matched controls with other chronic ailments were recruited...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28540445/indications-for-kidney-biopsy-in-idiopathic-childhood-nephrotic-syndrome
#14
Alanoud Alshami, Abishek Roshan, Marisa Catapang, Jasper J Jöbsis, Trevor Kwok, Nonnie Polderman, Jennifer Sibley, Matt Sibley, Cherry Mammen, Douglas G Matsell
BACKGROUND: Most cases of childhood nephrotic syndrome (NS) are due to minimal change disease (MCD), while a minority of children have focal segmental glomerulosclerosis (FSGS) and an unfavorable clinical course, requiring a kidney biopsy to confirm diagnosis. We hypothesized that clinical characteristics at diagnosis and initial response to corticosteroid treatment accurately predict FSGS and can be used to guide consistent practice in the indications for kidney biopsy. METHODS: This was a case control study (1990-2012)...
May 24, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28533882/-idiopathic-nephrotic-syndrome-ins-in-children-in-dakar-about-40-cases
#15
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/28480100/the-burden-and-outcome-of-pediatric-renal-admissions-at-the-federal-teaching-hospital-abakaliki-a-3-year-review-2011-2013
#16
V U Muoneke, A F Una, C B Eke, O U Anyanwu
BACKGROUND: Renal diseases are important causes of morbidity and mortality in children worldwide particularly in the resource-poor countries of sub-Saharan Africa. Adequate data on these diseases in children in our setting are limited as a result of late/nonpresentation. AIM: The aim of the study is to review the pattern and outcome of pediatric renal admissions at the Federal Teaching Hospital (FETH) Abakaliki over a 3-year period. SUBJECTS AND METHODS: This was a retrospective observational review of all childhood renal admissions in FETH, Abakaliki, Ebonyi state between 2011 and 2013...
July 2016: Annals of Medical and Health Sciences Research
https://www.readbyqxmd.com/read/28474581/presentations-and-management-of-different-causes-of-chylothorax-in-children-one-medical-center-s-experience
#17
Chien-Heng Lin, Wei-Ching Lin, Jeng-Sheng Chang
BACKGROUND: Chylothorax in children is a relatively rare cause of pleural effusion. However, it is usually a common complication of cardiothoracic operations like open-heart surgery. Other etiologies for chylothorax, such as trauma or malignancy, occur more common in adults and rare in children. To explore the etiologies of chylothorax in children, this study analyzed the pediatric patients that were admitted in to onea medical center. METHODS: We retrospectively reviewed the medical records of the pediatric patients that were admitted to this tertiary transfer center with a diagnosis of chylothorax during the period of 1995 to 2005...
March 2017: BioMedicine
https://www.readbyqxmd.com/read/28469399/a-novel-biomarker-panel-to-identify-steroid-resistance-in-childhood-idiopathic-nephrotic-syndrome
#18
Michael R Bennett, LaTawnya Pleasant, Christopher Haffner, Qing Ma, Wendy D Haffey, Jun Ying, Michael Wagner, Kenneth D Greis, Prasad Devarajan
Idiopathic nephrotic syndrome (NS) is the most common glomerular disorder of childhood. Response to initial treatment with corticosteroids is an indicator of prognosis, as resistant patients often present more progressive disease. In this cross-sectional pilot study, we set out to discover a panel of noninvasive biomarkers that could distinguish steroid-resistant nephrotic syndrome (SRNS) from steroid-sensitive nephrotic syndrome (SSNS). Information gleaned from such a panel could yield more individualized treatment plans and prevent unnecessary steroid exposure in patients unlikely to respond...
2017: Biomarker Insights
https://www.readbyqxmd.com/read/28466615/angiographic-embolization-in-pediatric-abdominal-trauma
#19
Yechiel Sweed, Jonathan Singer-Jordan, Sorin Papura, Norman Loberant, Alon Yulevich
BACKGROUND: Trauma is the leading cause of childhood morbidity and mortality. Abdominal bleeding is one of the common causes of mortality due to trauma. Angiography and embolization are well recognized as the primary treatments in certain cases of acute traumatic hemorrhage in adults; however, evidence is lacking in the pediatric population. OBJECTIVES: To assess the safety and efficacy of transcatheter arterial embolization (TAE) for blunt and penetrating abdominal and pelvic trauma in the pediatric age group...
November 2016: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28465799/immunoglobulin-g-and-m-levels-in-childhood-nephrotic-syndrome-two-centers-egyptian-study
#20
Ghada Mohamed El Mashad, Soha Abd El Hady Ibrahim, Sameh Abd Allah Abdelnaby
INTRODUCTION: Idiopathic nephrotic syndrome (INS) is the most common glomerular disease in children. Immune cell subsets may play a role in pathogenesis of INS. We aimed to assess immunoglobulin G (IgG) and immunoglobulin M (IgM) levels in children with nephrotic syndrome (NS) to predict prognosis of the disease and response to treatment. METHODS: This prospective case control study was done in Pediatric Nephrology Units at Minoufia and Benha University Hospitals, during the period from 1st March 2014 to 30th June 2015...
February 2017: Electronic Physician
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