keyword
MENU ▼
Read by QxMD icon Read
search

pediatric nephrotic syndrome

keyword
https://www.readbyqxmd.com/read/28427453/the-italian-society-for-pediatric-nephrology-sinepe-consensus-document-on-the-management-of-nephrotic-syndrome-in-children-part-i-diagnosis-and-treatment-of-the-first-episode-and-the-first-relapse
#1
REVIEW
Andrea Pasini, Elisa Benetti, Giovanni Conti, Luciana Ghio, Marta Lepore, Laura Massella, Daniela Molino, Licia Peruzzi, Francesco Emma, Carmelo Fede, Antonella Trivelli, Silvio Maringhini, Marco Materassi, Giovanni Messina, Giovanni Montini, Luisa Murer, Carmine Pecoraro, Marco Pennesi
This consensus document is aimed at providing an updated, multidisciplinary overview on the diagnosis and treatment of pediatric nephrotic syndrome (NS) at first presentation. It is the first consensus document of its kind to be produced by all the pediatric nephrology centres in Italy, in line with what is already present in other countries such as France, Germany and the USA. It is based on the current knowledge surrounding the symptomatic and steroid treatment of NS, with a view to providing the basis for a separate consensus document on the treatment of relapses...
April 21, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28405841/focal-segmental-glomerulosclerosis-and-medullary-nephrocalcinosis-in-children-with-adck4-mutations
#2
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/28392951/three-novel-mutations-in-the-nphs1-gene-in-vietnamese-patients-with-congenital-nephrotic-syndrome
#3
Thi Kim Lien Nguyen, Van Dem Pham, Thu Huong Nguyen, Trung Kien Pham, Thi Quynh Huong Nguyen, Huy Hoang Nguyen
Congenital nephrotic syndrome, a rare and severe disease, is inherited as an autosomal recessive trait. The disease manifests shortly after birth and occurs predominantly in families of Finnish origin but has now been observed in all countries and races. Mutations in the NPHS1 gene, which encodes nephrin, are the main causes of congenital nephrotic syndrome in patients. In this study, we report the first mutational analysis of the NPHS1 gene in three unrelated children from three different Vietnamese families...
2017: Case Reports in Genetics
https://www.readbyqxmd.com/read/28378029/nephrotoxicity-in-children-with-frequently-relapsing-nephrotic-syndrome-receiving-long-term-cyclosporine-treatment
#4
Yuko Hamasaki, Fumiyo Komaki, Kenji Ishikura, Riku Hamada, Tomoyuki Sakai, Hiroshi Hataya, Kentaro Ogata, Takashi Ando, Masataka Honda
BACKGROUND: Steroid-sparing drugs, such as cyclosporine, are recommended as treatment for children with frequently relapsing nephrotic syndrome (FRNS) and steroid-related toxicities. We recently reported a high rate of relapsing nephrotic syndrome 2 years after discontinuation of cyclosporine treatment, suggesting that long-term treatment is necessary. Cyclosporine-associated nephrotoxicity (CAN) is a potential side effect of long-term cyclosporine treatment. METHODS: We retrospectively reviewed pediatric patients with FRNS treated with cyclosporine for ≥3 years at a single center between 1999 and 2012...
April 4, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28352006/assay-of-urinary-protein-carbonyl-content-can-predict-the-steroid-dependence-and-resistance-in-children-with-idiopathic-nephrotic-syndrome
#5
Niranjan Gopal, Bidhan Chandra Koner, Atanu Bhattacharjee, Vishnu Bhat, Sathish Babu Murugaiyan, Prakash H Muddegowda
Nephrotic syndrome in pediatric age is mostly idiopathic. Idiopathic nephrotic syndrome (INS) by default is treated with steroids from the very beginning. Some do not respond to steroids and are grouped later as either steroid-resistant (SR) or steroid-dependent (SD) cases. The protein selectivity index often fails to predict the SR and SD cases. The SD and SR cases of INS exhibit higher degrees of oxidative stress compared to steroid responders. Proteins get carbonylated when they are exposed to free radicals...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28350720/pulmonary-embolism-mimicking-infectious-pleuritis
#6
Kenichi Tetsuhara, Satoshi Tsuji, Satoko Uematsu, Koichi Kamei
The diagnosis of pulmonary thromboembolism (PE) is often delayed because it is usually misdiagnosed as pneumonia or deep vein thrombosis. We report an unusual case of PE misdiagnosed as viral pleuritis on the first arrival at the emergency department (ED) in our hospital. A 14-year-old girl with no previous significant medical history was referred to the ED with pleuritic and chest pain with low-grade fever 4 days before admission.Echography showed a small amount of left pleural effusion. A 12-lead electrocardiogram was normal...
March 27, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28326197/childhood-nephrotic-syndrome-management-and-outcome-a-single-center-retrospective-analysis
#7
Chia-Shi Wang, Jia Yan, Robert Palmer, James Bost, Mattie Feasel Wolf, Larry A Greenbaum
There is a paucity of information on outpatient management and risk factors for hospitalization and complications in childhood nephrotic syndrome (NS). We described the management, patient adherence, and inpatient and outpatient usage of 87 pediatric NS patients diagnosed between 2006 and 2012 in the Atlanta Metropolitan Statistical Area. Multivariable analyses were performed to examine the associations between patient characteristics and disease outcome. We found that 51% of the patients were treated with two or more immunosuppressants...
2017: International Journal of Nephrology
https://www.readbyqxmd.com/read/28303389/variability-of-diagnostic-criteria-and-treatment-of-idiopathic-nephrotic-syndrome-across-european-countries
#8
Georges Deschênes, Marina Vivarelli, Licia Peruzzi
The aim of the surveys conducted by the Idiopathic Nephrotic Syndrome Working Group of the ESPN was to study the possible variability of treatment in Europe at different stages of the disease by means of questionnaires sent to members of the Working Group. Four surveys have been completed: treatment of the first flare, treatment of the first relapse and the issue of steroid dependency, use of rituximab, and the management of steroid-resistant patients. A uniform treatment of the first flare was applied in only three countries, and ten additional centers have adopted one of the three main protocols...
March 16, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28270642/kidney-disease-profile-of-syrian-refugee-children
#9
Mehtap Akbalik Kara, Beltinge Demircioglu Kilic, Nilgun Col, Ayse Aysima Ozcelik, Mithat Buyukcelik, Ayse Balat
INTRODUCTION: Although preventative nephrology is the effective management of childhood kidney diseases, it is hard to provide it in this undesirable conditions. In this study, we aimed to document the kidney disease profile of Syrian refugee children admitted to our hospital. MATERIALS AND METHODS: One hundred and thirty Syrian refugee children were admitted to the Pediatric Nephrology Department of the University of Gaziantep from September 2012 to January 2015...
March 2017: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/28261934/steroid-induced-impairment-of-glucose-tolerance-prevalence-among-pediatric-patients-on-long-term-steroid-use-in-nigeria
#10
U N Chikani, M U Ibekwe, T Oguonu, L Mungai, A I Bisi-Onyemaechi, O M Ugege, I F Ogbonna, Carine de Beaufort
INTRODUCTION: Glucocorticoid (referred to from here on as simply steroid) is used for effective treatment of various inflammatory disorders since its discovery in 1940s. However, these useful drugs cause important side effects, such as impairment of glucose tolerance. We sought to determine the prevalence of steroid-induced impairment of glucose tolerance in pediatric patients on long-term steroid use. MATERIALS AND METHODS: A cross-sectional, descriptive and hospital-based study...
March 6, 2017: Pediatric Diabetes
https://www.readbyqxmd.com/read/28254584/outcomes-and-risk-factors-for-graft-loss-lessons-learned-from-1-056-pediatric-kidney-transplants-at-the-university-of-minnesota
#11
Srinath Chinnakotla, Priya Verghese, Blanche Chavers, Michelle N Rheault, Varvara Kirchner, Ty Dunn, Clifford Kashtan, Thomas Nevins, Michael Mauer, Timothy Pruett
BACKGROUND: Advances in immunosuppression, surgical techniques, and management of infections in children receiving kidney transplants have impacted outcomes. STUDY DESIGN: We analyzed a prospectively maintained database of pediatric kidney transplants. RESULTS: From June 1963 through October 2016, we performed 1,056 pediatric kidney transplants. Of these 129 were < 2 years old. The most common indications for transplant were congenital anomalies (dysplastic kidneys), obstructive uropathy and congenital nephrotic syndrome...
January 11, 2017: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/28250989/apolipoprotein-c-i-levels-are-associated-with-the-urinary-protein-urinary-creatinine-ratio-in-pediatric-idiopathic-steroid-sensitive-nephrotic-syndrome-a-case-control-study
#12
Jun Odaka, Takahiro Kanai, Takane Ito, Takashi Saito, Jun Aoyagi, Hiroyuki Betsui, Takanori Yamagata
Humoral factors may cause idiopathic steroid-sensitive nephrotic syndrome (ISSNS). In the present study, we analyzed serum proteins using mass spectrometry (MS) to identify proteins associated with the pathophysiology of pediatric ISSNS. We collected serial serum samples from 33 children during each ISSNS phase; Phase A1 is the acute phase prior to steroid treatment (STx), Phase A2 represents the remission period with STx, and Phase A3 represents the remission period after completion of STx. Children with normal urinalyses (Group B) and children with a nephrotic syndrome other than ISSNS (Group C) served as controls...
2017: International Journal of Nephrology
https://www.readbyqxmd.com/read/28248816/tdt-positive-infiltrate-in-inflamed-pediatric-kidney-a-potential-diagnostic-pitfall
#13
Jennifer B Dunlap, Michael J Cascio, Xavier Stacey, Sarah Click, Megan L Troxell
We encountered a patient with infantile nephrotic syndrome associated with a dense interstitial inflammatory infiltrate and prominent extramedullary hematopoiesis. Immunohistochemical analysis revealed numerous terminal deoxynucleotidyl transferase (TdT)-positive cells, which may raise concern for lymphoblastic lymphoma. Thus, we further characterized a group of pediatric kidneys with inflammation. TdT-positive nuclei were quantitated, and dual immunostains for TdT/CD79a, TdT/CD3, and TdT/CD43 were performed in a subset of cases; flow cytometry was performed in 1 case...
May 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28213687/ofatumumab-for-the-treatment-of-childhood-nephrotic-syndrome
#14
Chia-Shi Wang, Rochelle Schmidt Liverman, Rouba Garro, Roshan Punnoose George, Anastacia Glumova, Alana Karp, Stephanie Jernigan, Barry Warshaw
BACKGROUND: Ofatumumab is a humanized anti-CD20 monoclonal antibody that has recently garnered interest as a potential therapeutic agent for nephrotic syndrome. We report our center's experience in administering ofatumumab to five pediatric patients with idiopathic nephrotic syndrome. METHODS: Between March 2015 and November 2016, five patients were treated with ofatumumab. One patient had post-transplant recurrent focal segmental glomerulosclerosis (FSGS) which had been resistant to plasmapheresis and numerous immunosuppressive agents...
May 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28204946/clinical-outcomes-in-children-with-henoch-sch%C3%A3-nlein-purpura-nephritis-without-crescents
#15
Jean Daniel Delbet, Julien Hogan, Bilal Aoun, Iulia Stoica, Rémi Salomon, Stéphane Decramer, Isabelle Brocheriou, Georges Deschênes, Tim Ulinski
BACKGROUND: Henoch-Schönlein purpura is the most common vasculitis in children. Its long-term prognosis depends on renal involvement. The management of Henoch-Schönlein purpura nephritis (HSPN) remains controversial. This study reports the prognosis of children with HSPN presenting with class 2 International Study of Kidney Disease in Children (ISKDC) nephritis. METHODS: All children with HSPN class 2 diagnosed between 1995 and 2015 in four pediatric nephrology centers were included, and clinical and biological data were collected from the medical files...
February 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28204945/spectrum-of-mutations-in-chinese-children-with-steroid-resistant-nephrotic-syndrome
#16
Fang Wang, Yanqin Zhang, Jianhua Mao, Zihua Yu, Zhuwen Yi, Li Yu, Jun Sun, Xiuxiu Wei, Fangrui Ding, Hongwen Zhang, Huijie Xiao, Yong Yao, Weizhen Tan, Svjetlana Lovric, Jie Ding, Friedhelm Hildebrandt
BACKGROUND: The aim of this study was to elucidate whether genetic screening test results of pediatric patients with steroid-resistant nephrotic syndrome (SRNS) vary with ethnicity. METHODS: Using high-throughput DNA sequencing, 28 nephrotic syndrome-related genes were analyzed in 110 chil-dren affected by SRNS and 10 children with isolated proteinuria enrolled by 5 centers in China (67 boys, 53 girls). Their age at disease onset ranged from 1 day to 208 months (median, 48...
February 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28175986/does-zika-virus-infection-induce-prolonged-remissions-in-children-with-idiopathic-nephrotic-syndrome
#17
Carolina Peralta-Aros, Víctor García-Nieto
BACKGROUND: Zika is an emerging mosquito-borne flavivirus. We report two pediatric patients diagnosed with idiopathic nephrotic syndrome who achieved complete remission of the disease after suffering Zika virus (ZIKV) infection. CASE DIAGNOSIS/TREATMENT: The first patient was a young girl aged 2.5 years with steroid-dependent nephrotic syndrome who was subsequently diagnosed with ZIKV infection. Following the infection, the steroid dose could be reduced until complete withdrawal...
February 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28173656/-genotype-guided-individual-anticoagulation-with-warfarin-in-pediatric-nephrotic-syndrome-with-thromboembolism
#18
X H Zhong, X Q Yang, H J Xiao
No abstract text is available yet for this article.
February 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28117080/genomic-and-clinical-profiling-of-a-national-nephrotic-syndrome-cohort-advocates-a-precision-medicine-approach-to-disease-management
#19
Agnieszka Bierzynska, Hugh J McCarthy, Katrina Soderquest, Ethan S Sen, Elizabeth Colby, Wen Y Ding, Marwa M Nabhan, Larissa Kerecuk, Shivram Hegde, David Hughes, Stephen Marks, Sally Feather, Caroline Jones, Nicholas J A Webb, Milos Ognjanovic, Martin Christian, Rodney D Gilbert, Manish D Sinha, Graham M Lord, Michael Simpson, Ania B Koziell, Gavin I Welsh, Moin A Saleem
Steroid Resistant Nephrotic Syndrome (SRNS) in children and young adults has differing etiologies with monogenic disease accounting for 2.9-30% in selected series. Using whole exome sequencing we sought to stratify a national population of children with SRNS into monogenic and non-monogenic forms, and further define those groups by detailed phenotypic analysis. Pediatric patients with SRNS were identified via a national United Kingdom Renal Registry. Whole exome sequencing was performed on 187 patients, of which 12% have a positive family history with a focus on the 53 genes currently known to be associated with nephrotic syndrome...
April 2017: Kidney International
https://www.readbyqxmd.com/read/28093933/acute-kidney-injury-in-idiopathic-nephrotic-syndrome-of-childhood-is-a-major-risk-factor-for-the-development-of-chronic-kidney-disease
#20
Afshan Yaseen, Vina Tresa, Ali Asghar Lanewala, Seema Hashmi, Irshad Ali, Sabeeta Khatri, Muhammed Mubarak
BACKGROUND: Acute kidney injury (AKI) is an important complication of idiopathic nephrotic syndrome (INS) and is associated with adverse outcomes, especially the development of chronic kidney disease (CKD). We aimed to determine the clinical profile of children with INS who developed AKI and its short-term outcome. MATERIAL AND METHODS: This prospective study was conducted from March 2014 to October 2015. A total of 119 children of INS (age: 2-18 years) fulfilling the pediatric RIFLE criteria for the diagnosis of AKI were enrolled and followed up for 3 months to determine the outcome...
November 2017: Renal Failure
keyword
keyword
53750
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"