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COMPARATIVE STUDY
Xueting Cheng, Jiahuan Chen, Xueying Yang, Han Chan, Xia Yang, Jia Jiao, Anshuo Wang, Gaofu Zhang, Xuelan Chen, Xiaoqin Li, Mo Wang, Baohui Yang, Haiping Yang, Qiu Li
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) are monogenic in some cases, however, there are still no clear guidelines on genetic testing in the clinical practice of SRNS in children. METHODS: Three hundred thirty-two children were diagnosed with SRNS, and all children underwent genetic testing, including gene panels and/or whole-exome/genome sequencing (WES/WGS), during treatment. We analysed the relationship between clinical manifestation and genotype, and compared different genetic testing methods' detection rates and prices...
April 23, 2024: Italian Journal of Pediatrics
#2
JOURNAL ARTICLE
Floor Veltkamp, Marcel C M Pistorius, Elske M Mak-Nienhuis, Michiel F Schreuder, Antonia H M Bouts, Ron A A Mathôt
AIMS: Prednisolone is the cornerstone of treatment for idiopathic nephrotic syndrome in children, but is associated with marked side-effects. Therapeutic drug monitoring using saliva would be a patient-friendly option to monitor prednisolone treatment. To assess the feasibility of saliva monitoring, we described the pharmacokinetics (PK) of unbound prednisolone in plasma and saliva of children with first onset steroid-sensitive nephrotic syndrome (SSNS). METHODS: Children (age 2-16 years) with SSNS participating in a randomized, placebo-controlled trial with levamisole were treated with an 18-week tapering schedule of prednisolone...
April 10, 2024: British Journal of Clinical Pharmacology
#3
JOURNAL ARTICLE
Zunaira Zulfiqar, Fatema Lanewala, Sabeeta Khatri, Irshad Bajeer, Madiha Aziz, Seema Hashmi
OBJECTIVE: To screen children receiving steroids to identify ocular complications and their prevalence. METHODS: The cross-sectional study was conducted at the Paediatric Nephrology and Ophthalmology departments of the Sindh Institute of Urology and Transplantation, Karachi, from May to October 2022, and comprised patients who received at least 1500mg cumulative steroid dose for a minimum of 3 months. They were screened for steroidsensitive or steroid-resistant nephrotic syndrome...
February 2024: JPMA. the Journal of the Pakistan Medical Association
#4
JOURNAL ARTICLE
Maureen Tumwesige, Joseph Rujumba, Thereza Piloya, Judith Caroline Aujo
BACKGROUND: Nephrotic syndrome (NS) is the commonest glomerular disease among children. It is characterized by heavy proteinuria and is a risk factor for hypothyroidism in the affected children. Hypothyroidism is of concern because it affects the physical and intellectual development of children and adolescents. This study sought to establish the prevalence and factors associated with hypothyroidism among children and adolescents with NS. METHODS: A cross-sectional design was used to study 70 children and adolescents aged 1-19 years diagnosed with nephrotic syndrome and being followed up in the kidney clinic in Mulago National Referral Hospital...
February 19, 2024: BMC Pediatrics
#5
JOURNAL ARTICLE
Patrik Konopásek, Sylva Skálová, Eva Sládková, Monika Pecková, Eva Flachsová, Ivana Urbanová, Jana Laubová, Martina Samešová, Pavel Dvořák, Jakub Zieg
BACKGROUND: Several previous studies have reported a more severe course of nephrotic syndrome in children with low birth weight. PATIENTS: Cohort of 223 children with idiopathic nephrotic syndrome. METHODS: We aimed to investigate the association between course of nephrotic syndrome and low birth weight. Data from seven paediatric nephrology centres were used. RESULTS: Children with low birth weight had 3.84 times higher odds for a more severe course of steroid-sensitive nephrotic syndrome (95% CI 1...
February 6, 2024: Klinische Pädiatrie
#6
JOURNAL ARTICLE
Velibor Tasic, Vidar O Edvardsson, Evgenia Preka, Larisa Prikhodina, Constantinos J Stefanidis, Rezan Topaloglu, Diamant Shtiza, Ashot Sarkissian, Thomas Mueller-Sacherer, Rena Fataliyeva, Ina Kazyra, Elena Levtchenko, Danka Pokrajac, Dimitar Roussinov, Danko Milošević, Avraam Elia, Tomas Seeman, Mia Faerch, Inga Vainumae, Janne Kataja, Michel Tsimaratos, Irakli Rtskhiladze, Peter F Hoyer, George Reusz, Atif Awan, Danny Lotan, Licia Peruzzi, Nazim Nigmatullina, Nasira Beishebaeva, Edite Jeruma, Augustina Jankauskiene, Olivier Niel, Valerie Said-Conti, Angela Ciuntu, Snežana Pavićević, Michiel Oosterveld, Anna Bjerre, Marcin Tkaczyk, Ana Teixeira, Adrian C Lungu, Alexey Tsygin, Vesna Stojanović, Ludmila Podracka, Tanja Kersnik Levart, Mar Espino-Hernández, Per Brandström, Giuseppina Sparta, Harika Alpay, Dmytro Ivanov, Jan Dudley, Komiljon Khamzaev, Dieter Haffner, Jochen Ehrich
BACKGROUND: Primary, secondary and tertiary healthcare services in Europe create complex networks covering pediatric subspecialties, sociology, economics and politics. Two surveys of the European Society for Paediatric Nephrology (ESPN) in 1998 and 2017 revealed substantial disparities of kidney care among European countries. The purpose of the third ESPN survey is to further identify national differences in the conceptualization and organization of European pediatric kidney health care pathways during and outside normal working hours...
2024: Frontiers in Pediatrics
#7
JOURNAL ARTICLE
Katharina Rohner, Matko Marlais, Yo Han Ahn, Alaa Ali, Abrar Alsharief, Anja Blejc Novak, Marta Brambilla, Evrim Kargin Cakici, Cengiz Candan, Nur Canpolat, Eugene Yu-Hin Chan, Stéphane Decramer, Madeleine Didsbury, Filipa Durao, Anne M Durkan, Ali Düzova, Thomas Forbes, Valentina Gracchi, Tulin Güngör, Tomoko Horinouchi, Belde Kasap Demir, Yasuko Kobayashi, Mikael Koskela, Eda Didem Kurt-Sukur, Claudio La Scola, Dean Langan, Xiaozhong Li, Gabriele Malgieri, Antonio Mastrangelo, Jeesu Min, Malgorzata Mizerska-Wasiak, Nabila Moussaoui, Aytul Noyan, Matti Nuutinen, Jennifer O'Gormon, Takayuki Okamoto, Louise Oni, Michiel Oosterveld, Malgorzata Pańczyk-Tomaszewska, Gonul Parmaksiz, Andrea Pasini, Pornpimol Rianthavorn, Joris Roelofs, Yunyan Shen, Rajiv Sinha, Rezan Topaloglu, Diletta Domenica Torres, Tomohiro Udagawa, Martin Wennerström, Yok Chin Yap, Kjell Tullus
BACKGROUND AND HYPOTHESIS: IgA vasculitis with nephritis (IgAVN) is the most common vasculitis in children. Treatment recommendations are, due to a lack of evidence, based on expert opinion resulting in variation. The aim of this study was to describe clinical presentation, treatment and outcome of an extremely large cohort of children with biopsy proven IgAVN to identify prognostic risk factors and signals of treatment efficacy. METHODS: Retrospective data were collected on 1148 children with biopsy proven IgAVN between 2005 and 2019 from 41 international paediatric nephrology centres across 25 countries and analyzed using multivariate analysis...
January 11, 2024: Nephrology, Dialysis, Transplantation
#8
JOURNAL ARTICLE
Syeda Tayyaba Rehan, Eman Ali, Farea Eqbal, Muhammad Nadeem Ahsan, Muhammad Sohaib Asghar
No abstract text is available yet for this article.
January 2024: Clinical Kidney Journal
#9
JOURNAL ARTICLE
Ahmet Ünal, Emre Leventoğlu, Sevcan A Bakkaloğlu
AIM: Rituximab (RTX) is a genetically engineered chimeric monoclonal antibody which binds directly to CD20 antigen and mediates inhibition of B cell development. Although RTX has been widely used in paediatric nephrology, there is no routine protocol for its use. In this study, paediatric nephrologists in Türkiye were asked to fill out a questionnaire to understand their practice in using RTX. This study aimed to determine common practices and clarify the uncertainties regarding the use of RTX in paediatric nephrology...
December 19, 2023: Nephrology
#10
R Aliu, I Jalo, L Olatoke, I Asabe, K S Hassan, A Bello, M A Danimoh
INTRODUCTION: Children present with a spectrum of renal diseases depending on age, sex, and geographic location among other factors. With the absence of a paediatric renal registry in Nigeria, this will provide part of the regional data necessary for the Nigerian renal registry. METHODOLOGY: A retrospective study where cases of renal diseases that presented in a nephrology clinic over a 2-year period were retrieved from the nurses' and doctors' records and analysed...
November 10, 2023: West African Journal of Medicine
#11
JOURNAL ARTICLE
M Musharraf, B K Majumder, R B Chowdhury, A R Mondal, S A Albani, M A Mostakim
Minimal change nephrotic syndrome is the most common cause of the glomerular disease in children. Despite its high initial response to corticosteroids, relapses are common leading to increased morbidity. Therefore, appraisal of common triggers of relapse becomes an imperative for successful management of childhood nephrotic syndrome. The objective of the study was to identify the risk factors for frequent relapse in childhood nephrotic syndrome. This descriptive cross-sectional study was conducted in the department of Paediatrics, Rangpur Medical College Hospital, Bangladesh from January 2013 to December 2014...
October 2023: Mymensingh Medical Journal: MMJ
#12
Anna Wasilewska, Agnieszka Rybi-Szuminska, Pawel Dubiela
BACKGROUND: Although steroid therapy is a standard of care for nephrotic syndrome treatment, 15-20% of patients do not respond to it. Finding the genetic background is possible in >10% of steroid-resistant nephrotic syndrome (SRNS) cases. Variants in genes encoding nuclear pore complex proteins are a novel cause of paediatric steroid-resistant nephrotic syndrome (SRNS). Recent studies suggest NUP93 variants to be a significant cause of paediatric onset SRNS. The clinical data on certain variants and disease history are still very limited...
September 7, 2023: Journal of Clinical Medicine
#13
JOURNAL ARTICLE
Yongzheng Zhang, Lin Du, Bonan Liu, Hai Wang
Nephrotic syndrome, a common kidney disease syndrome in children, has triggered extensive clinical research to identify safe and effective treatments. Qingxin Lianzi Drink, as a traditional Chinese medicine prescription, has been paid more and more attention in the treatment of nephrotic syndrome in children. Its main ingredients include Shilotus meat, scutellaria skullcap and ground bone skin, etc. These ingredients have the effects of clearing heat and detoxifying, reducing swelling and water, and nourishing liver and kidney...
September 15, 2023: Alternative Therapies in Health and Medicine
#14
JOURNAL ARTICLE
Zivile Bekassy, Martin Lindström, Therese Rosenblad, Sunna Aradóttir, Lisa Sartz, Kjell Tullus
AIM: To investigate the need, in the Northern European setting, to perform kidney biopsy in children with steroid-sensitive nephrotic syndrome. METHODS: In this retrospective study 124 individuals aged 1-18 years with idiopathic nephrotic syndrome, followed in the paediatric hospitals in southern Sweden from 1999 to 2018, were included. RESULTS: There was a median follow-up time of 6.5 (0.2-16.8) years. The majority (92%) of children were steroid-sensitive and of them, 60...
August 29, 2023: Acta Paediatrica
#15
JOURNAL ARTICLE
P D Makanda-Charambira, H A Mujuru, I Ticklay, L Muchemwa
BACKGROUND: The pattern of paediatric kidney diseases across different regions is influenced by genetic, racial, and environmental differences. OBJECTIVES: The aim of this study was to review the current spectrum and outcome of childhood kidney diseases at Parirenyatwa Group of Hospitals and highlight the challenges of care. DESIGN: Retrospective observational study. METHODS: Data on all children below 16 years of age hospitalised for any kidney disease over an 8-month period (1 January-31 August 2022) were retrieved and retrospectively analysed...
2023: Clinical Medicine Insights. Pediatrics
#16
JOURNAL ARTICLE
Talerngsak Kanjanabuch, Suramath Isaranuwatchai, Tanawin Nopsopon, Theerachai Thammathiwat, Thitipong Pooprasert, Pongpratch Puapatanakul, Krit Pongpirul, Ratana Chawanasuntorapoj, Piyawan Kittiskulnam, Somchai Eiam-Ong, Kring Tungsanga, Saowaluck Chusil, Vuddhidej Ophascharoensuk, Supat Vanichakarn, Visith Sitprija, Vijitr Boonpucknavig
BACKGROUND: This study aims to investigate the influence of different kidney biopsy practices on the prevalence of glomerular pathologic patterns in the largest kidney biopsy registry in Thailand. METHODS: We conducted a retrospective review of kidney biopsy records from the period between 2000 and 2014. The records were obtained from 2 major institutions: King Chulalongkorn Memorial Hospital, a large university-based hospital, and the Kidney Center Bangkok Hospital, which provides pathology services to hospitals throughout Thailand...
August 2023: Nephrology
#17
Maureen Tumwesige, Joseph Rujumba, Thereza Piloya, Carol Aujo
Background Nephrotic syndrome (NS) is the commonest glomerular disease among children. It is characterized by heavy proteinuria and is a risk factor for hypothyroidism in the affected children. Hypothyroidism is of concern because it affects the physical and intellectual development of children and adolescents. This study sought to establish the prevalence and factors associated with hypothyroidism among children and adolescents with NS. Methods A cross-sectional design was used to study 70 children and adolescents aged 1-19 years diagnosed with nephrotic syndrome and being followed up in the kidney clinic in Mulago National Referral Hospital...
June 12, 2023: Research Square
#18
JOURNAL ARTICLE
Sucheta Parija, Nikita Dash, Genickson Jeyaraj
Nephrotic syndrome (NS) is a common glomerular disease characterised by massive proteinuria, hypoalbuminaemia, oedema and hyperlipidaemia. Cerebral venous sinus thrombosis (CVST) is a rare complication in children with NS. Here, we report a case of relapsing NS on steroid therapy, in a male in early childhood who presented with symptoms of headache, vomiting and double vision. On prism cover test there was 25 PD esotropia with abduction restriction in the left eye. Fundus examination showed bilateral papilloedema...
June 2, 2023: BMJ Case Reports
#19
JOURNAL ARTICLE
Floor Veltkamp, Victoire Thenot, Carlijn Mussies, Bas van Lieshout, Hessel Peters-Sengers, Jesper Kers, Djera H Khan, Julien Hogan, Sandrine Florquin, Antonia H M Bouts, Claire Dossier
BACKGROUND: The aetiology of idiopathic nephrotic syndrome (INS) remains partially unknown. Viral infections have been associated with INS onset. Since we observed fewer first onset INS cases during the Covid-19 pandemic, we hypothesised that lower INS incidence was the result of lockdown measures. Therefore, the aim of this study was to evaluate the incidence of childhood INS before and during the COVID-19 pandemic in two independent European INS cohorts. METHODS: Children with new INS in the Netherlands (2018-2021) and Paris area (2018-2021) were included...
May 16, 2023: Pediatric Nephrology
#20
JOURNAL ARTICLE
Shubham K Indurkar, Kriti Mohan, Balram Ji Omar, Swathi Chacham, Manish Kumar
BACKGROUND: Urinary tract infection (UTI) is a common infection in children with nephrotic syndrome (NS). Clinical experience suggests that childhood nephrotic syndrome is frequently diagnosed incorrectly and managed inadequately on the top of this existing UTI in the episode becomes an additive obstacle for the primary care physicians or pediatricians towards optimum management, leading to poor outcome. So, we have conducted this clinico- microbiological study of UTI in NS in children to provide the exact picture of UTI with NS so that the primary care providers can be helped in having high index of suspicion of this infection and knowing prevalent organisms and their antimicrobial sensitivity pattern...
November 2022: Journal of Family Medicine and Primary Care
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