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childhood nephrotic

M Mizerska-Wasiak, J Małdyk, A Turczyn, K Cichoń-Kawa, A Rybi-Szumińska, A Wasilewska, B Bieniaś, M Zajączkowska, M Miklaszewska, J Pietrzyk, U Demkow, M Roszkowska-Blaim, M Pańczyk-Tomaszewska
The aim of this retrospective study was to assess the usefulness of potential predictors of poor prognosis in IgA nephropathy in children. The study population consisted of 55 children aged 11 ± 4 years, diagnosed on the basis of the Oxford classification and MEST score of kidney biopsy findings. Proteinuria, glomerular filtration rate (GFR), and the IgA/C3 serum ratio were assessed in all patients twice: at onset and at follow-up. The patients were treated with steroids, immunosuppressive drugs, and/or angiotensin-converting enzyme inhibitors...
October 8, 2016: Advances in Experimental Medicine and Biology
Elisa Adele Colombo, Luigina Spaccini, Ludovica Volpi, Gloria Negri, Davide Cittaro, Dejan Lazarevic, Salvatore Zirpoli, Andrea Farolfi, Cristina Gervasini, Maria Vittoria Cubellis, Lidia Larizza
BACKGROUND: Integrin α3 (ITGA3) gene mutations are associated with Interstitial Lung disease, Nephrotic syndrome and Epidermolysis bullosa (ILNEB syndrome). To date only six patients are reported: all carried homozygous ITGA3 mutations and presented a dramatically severe phenotype leading to death before age 2 years, from multi-organ failure due to interstitial lung disease and congenital nephrotic syndrome. The involvement of skin and cutaneous adnexa was variable with sparse hair and nail dysplasia combined or not to skin lesions ranging from skin fragility to epidermolysis bullosa-like blistering...
October 7, 2016: Orphanet Journal of Rare Diseases
Yasser Gamal, Ahlam Badawy, Salwa Swelam, Mostafa S K Tawfeek, Eman Fathalla Gad
Glucocorticoids are primary therapy of idiopathic nephrotic syndrome (INS). However, not all children respond to steroid therapy. We assessed glomerular glucocorticoid receptor expression in fifty-one children with INS and its relation to response to steroid therapy and to histopathological type. Clinical, laboratory and glomerular expression of glucocorticoid receptors were compared between groups with different steroid response. Glomerular glucocorticoid expression was slightly higher in controls than in minimal change early responders, which in turn was significantly higher than in minimal change late responders...
October 3, 2016: Fetal and Pediatric Pathology
Manel Jellouli, Sonia Fitouhi, Kamel Abidi, Yousra Hammi, Ouns Naija, Chokri Zarrouk, Tahar Gargah
OBJECTIVES: To establish the efficacy of mycophenolate mofetil (MMF) in steroid dependent nephrotic syndrome and to determine the predictive factors for a good response. METHODS: retrospective hospital-based cohort study in the department of pediatric of Charles Nicolle hospital, between 2005 and 2012 included 30 children with steroid-dependent nephritic syndrome who were treated with MMF. RESULTS: A total of 30 patients (20 boys and 10 girls) were included...
March 2016: La Tunisie Médicale
Amal A Al-Eisa, Mohammad Z Haider
BACKGROUND: Vitamin D activity is controlled by vitamin D receptors (VDRs), which are affected by different genetic polymorphisms, including TaqI and Apal restriction fragment length polymorphisms (RFLPs), which have been reported to be associated with several diseases. The aim of this study was to determine the frequency and the association of VDR gene polymorphisms with idiopathic nephrotic syndrome (INS) and steroid responsiveness in Kuwaiti children. SUBJECTS AND METHODS: Genotypes of the VDR TaqI gene polymorphism and the Apal gene polymorphism were analyzed using polymerase chain reaction-RFLP in 78 INS patients and 56 matched controls...
2016: International Journal of Nephrology and Renovascular Disease
Jiun-Nong Lin, Cheng-Li Lin, Chi-Hui Yang, Ming-Chia Lin, Chung-Hsu Lai, Hsi-Hsun Lin, Chia-Hung Kao
PURPOSE: Nephrotic syndrome is a common chronic illness encountered during childhood. Infections have been identified as a cause of nephrotic syndrome. The aim of this study was to evaluate the association between enteroviral infection and nephrotic syndrome. METHODS: A nationwide retrospective cohort study was conducted by analyzing data from the National Health Insurance Research Database in Taiwan. Children aged <18 years with enteroviral infection were enrolled...
2016: PloS One
Shuji Sai, Masaki Yamamoto, Rie Yamaguchi, Karen E Chapman, Teruaki Hongo
Childhood nephrotic syndrome, in which steroid-dependence occurs concurrently with steroid-resistance, requires aggressive therapy to prevent relapse. Predictive biomarkers that can be used to stratify treatment are urgently needed. Here we report that reciprocal regulation of the glucocorticoid metabolizing enzymes, 11β-hydroxysteroid dehydrogenase types 1 and 2, is associated with steroid-responsiveness and disease remission in childhood nephrotic syndrome, potentially providing a marker to identify patients in which aggressive therapy is required...
September 2016: Pediatrics
Syed Sajid Hussain Shah, Farkhanda Hafeez
OBJECTIVE: To compare the efficacy of tacrolimus versus cyclosporine (Calcineurin Inhibitors) in the management of childhood steroid-resistant nephritic syndrome (SRNS). STUDY DESIGN: Quasi-experimental study. PLACE AND DURATION OF STUDY: Department of Paediatric Nephrology at The Children's Hospital and Institute of Child Health, Lahore, from August 2014 to September 2015. METHODOLOGY: Patients of either gender aged 1 - 12 years, with the diagnosis of mesangioproliferative glomerulonephritis (MesangioPGN), focal segmental glomerulosclerosis (FSGS) or minimal-change disease (MCD) were included...
July 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Hai-Dong Fu, Gu-Ling Qian, Zheng-Yang Jiang
Although, most patients respond initially to therapy for nephrotic syndrome, about 70% of patients have a relapse. Currently, there is no consensus about the most appropriate second-line agent in children who continue to suffer a relapse. This network meta-analysis was designed to compare the efficacy and safety of the commonly used immunosuppressive agents in second-line therapeutic agents (ie, cyclophosphamide, cyclosporine, tacrolimus and mycophenolate mofetil) for refractory childhood nephrotic syndrome...
August 3, 2016: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
Alana M Karp, Rasheed A Gbadegesin
The pathogenesis of childhood-onset nephrotic syndrome (NS), disparity in incidence of NS among races, and variable responses to therapies in children with NS have defied explanation to date. In the last 20 years over 50 genetic causes of steroid-resistant nephrotic syndrome (SRNS) have been identified, and at least two disease loci for two pathologic variants of SRNS (focal segmental glomerulosclerosis and membranous nephropathy) have been defined. However, the genetic causes and risk loci for steroid-sensitive nephrotic syndrome (SSNS) remain elusive, partly because SSNS is relatively rare and also because cases of SSNS vary widely in phenotypic expression over time...
July 29, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Tonny H M Banh, Neesha Hussain-Shamsy, Viral Patel, Jovanka Vasilevska-Ristovska, Karlota Borges, Cathryn Sibbald, Deborah Lipszyc, Josefina Brooke, Denis Geary, Valerie Langlois, Michele Reddon, Rachel Pearl, Leo Levin, Monica Piekut, Christoph P B Licht, Seetha Radhakrishnan, Kimberly Aitken-Menezes, Elizabeth Harvey, Diane Hebert, Tino D Piscione, Rulan S Parekh
BACKGROUND AND OBJECTIVES: Ethnic differences in outcomes among children with nephrotic syndrome are unknown. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We conducted a longitudinal study at a single regional pediatric center comparing ethnic differences in incidence from 2001 to 2011 census data and longitudinal outcomes, including relapse rates, time to first relapse, frequently relapsing disease, and use of cyclophosphamide. Among 711 children, 24% were European, 33% were South Asian, 10% were East/Southeast Asian, and 33% were of other origins...
July 21, 2016: Clinical Journal of the American Society of Nephrology: CJASN
Kazumoto Iijima, Mayumi Sako, Kandai Nozu
Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. At least 20 % of children with this syndrome show frequent relapses and/or steroid dependence during or after immunosuppressive therapies, a condition defined as complicated frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS). Approximately 1-3 % of children with idiopathic nephrotic syndrome are resistant to steroids and all immunosuppressive agents, a condition defined as refractory steroid-resistant nephrotic syndrome (SRNS); these SRNS children have a high risk of end-stage renal failure...
July 15, 2016: Clinical and Experimental Nephrology
Dario Maratea, Monica Bettio, Maria Grazia Corti, Giovanni Montini, Francesca Venturini
BACKGROUND: Nephrotic syndrome is a disorder characterized by proteinuria, hypoalbuminemia and dyslipidemia. Low-dose alternate-day steroid regimen is the standard of care. In case of relapse or significant adverse events, steroid-sparing agents may be used. This analysis was aimed at assessing the efficacy and safety of rituximab for the treatment of children with nephrotic syndrome. RESULTS: Four studies were included in the final meta-analysis. The end-point of our analysis was the percentage of patients in remission at 6 months...
2016: Italian Journal of Pediatrics
Marcin Tkaczyk, Monika Miklaszewska, Jolanta Lukamowicz, Wojciech Fendler
BACKGROUND: The role of idiopathic nephrotic syndrome (INS) in the pathogenesis of atherosclerosis in childhood has not been clearly elucidated. However, antioxidative defense in INS is thought to be imbalanced. This study aimed to assess the changes of plasma concentration of selected aminothiols in the blood of children with INS at various stages of the disease. METHODS: This cross-sectional study was conducted in 125 children aged 2-18 years. The children were divided into 4 groups: group A, early relapse (n=37); group B, early remission for 4-6 weeks from the onset (n=37); group C, late steroid-free remission (n=31); and group D, long-term remission for 2-5 years (n=20)...
August 2016: World Journal of Pediatrics: WJP
Kevin V Lemley, Christian Faul, Karla Schramm, Kevin Meyers, Frederick Kaskel, Katherine M Dell, Debbie S Gipson, Keisha Gibson, Howard Trachtman
Case reports have linked childhood nephrotic syndrome to food sensitivity, including gluten. We report our experience with 8 children (6 boys, 2 girls; age at implementation of special diet 2-14 years) with difficult-to-manage nephrotic syndrome who were placed on a gluten-free diet for 3.4 ± 4.3 years (range, 0.6-14 years) and who had clinical improvement enabling reduction or discontinuation in steroid dosage.
July 2016: Pediatrics
Yo Han Ahn, Eu Jin Park, Hee Gyung Kang, Seong Heon Kim, Hee Yeon Cho, Jae Il Shin, Joo Hoon Lee, Young Seo Park, Kyo Sun Kim, Il-Soo Ha, Hae Il Cheong
BACKGROUND: WT1 is one of the genes commonly reported as mutated in children with steroid-resistant nephrotic syndrome (SRNS). We analyzed genotype-phenotype correlations in pediatric SRNS patients with WT1 mutations. METHODS: From 2001 to 2015, WT1 mutations were detected in 21 out of 354 children with SRNS by genetic screening (5.9 %). The patients were grouped into missense (n = 11) and KTS splicing (n = 10) mutation groups. RESULTS: Nine (82 %) patients with missense mutations presented with congenital/infantile nephrotic syndrome, while 8 (80 %) with KTS splicing mutations presented with childhood-onset SRNS...
June 14, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Laurène Dehoux, Julien Hogan, Claire Dossier, Marc Fila, Olivier Niel, Anne Maisin, Marie Alice Macher, Thérésa Kwon, Véronique Baudouin, Georges Deschênes
BACKGROUND: Prospective studies have established the mycophenolate mofetil (MMF) efficiency in childhood idiopathic nephrotic syndrome (INS) but reports on the long-term outcome are lacking. Moreover, the search for factors influencing its efficiency would be useful to define its place among the other treatments. METHODS: We performed a monocentric retrospective study including 96 children with steroid-dependent INS followed for 4.7 years (median) (IQ 3-6) after the onset of MMF treatment...
November 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Rebecca Hjorten, Zohra Anwar, Kimberly Jean Reidy
There are limited studies on long-term outcomes of childhood onset nephrotic syndrome (NS). A majority of children with NS have steroid-sensitive nephrotic syndrome (SSNS). Steroid-resistant nephrotic syndrome (SRNS) is associated with a high risk of developing end-stage renal disease. Biomarkers and analysis of genetic mutations may provide new information for prognosis in SRNS. Frequently relapsing and steroid-dependent NS is associated with long-term complications, including dyslipidemia, cataracts, osteoporosis and fractures, obesity, impaired growth, and infertility...
2016: Frontiers in Pediatrics
Michelle R Denburg
PURPOSE OF REVIEW: This review summarizes recent findings on musculoskeletal health in three chronic renal conditions of childhood: chronic kidney disease stages 2-5D, nephrotic syndrome, and urolithiasis. Findings with important clinical implications warranting further investigation are highlighted. RECENT FINDINGS: Recent cohort studies have demonstrated a high burden of fracture and progressive deficits of cortical bone in children with chronic kidney disease...
July 2016: Current Opinion in Nephrology and Hypertension
Francesca Becherucci, Benedetta Mazzinghi, Aldesia Provenzano, Luisa Murer, Sabrina Giglio, Paola Romagnani
Primitive nephrotic syndrome is one of the most common glomerular diseases in childhood and represents the clinical manifestation of various pathologic changes in the kidney. In children, nephrotic syndrome is classified based on the initial response to empiric corticosteroid treatment, which is considered as the best predictor of patients' final outcome. The advent of next-generation sequencing technology showed that genetic alterations in structural genes of the podocyte can be recognized in a significant proportion of not only familial or syndromic patients with steroid-resistant nephrotic syndrome (SRNS), but also of sporadic cases, raising the question of whether it is time to update current protocols of patient care...
August 2016: Journal of Nephrology
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