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JOURNAL ARTICLE
Matthew Schuchman, Tammy M Brady, Dorey A Glenn, Katherine R Tuttle, Gabriel Cara-Fuentes, Rebecca V Levy, Agustin Gonzalez-Vicente, Fadhl M Alakwaa, Tarak Srivastava, Christine B Sethna
INTRODUCTION: The prevalence of mental health disorders including anxiety and depression is increasing and is linked to hypertension in healthy individuals. However, the relationship of psychosocial patient-reported outcomes on blood pressure (BP) in primary proteinuric glomerulopathies is not well characterized. This study explored longitudinal relationships between psychosocial patient-reported outcomes and BP status among individuals with proteinuric glomerulopathies. METHODS: An observational cohort study was performed using data from 745 adults and children enrolled in the Nephrotic Syndrome Study Network (NEPTUNE)...
March 21, 2024: Journal of Nephrology
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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
#3
JOURNAL ARTICLE
Min-Hua Tseng, Shih-Hua Lin, Wen-Lang Fan, Ta-Wei Wu, Shuan-Pei Lin, Jhao-Jhuang Ding, I-Jung Tsai, Jeng-Daw Tsai
BACKGROUND/PURPOSE: Congenital nephrotic syndrome (CNS) is one of the important causes of end-stage kidney disease in children. Studies on the genotype, phenotype, and clinical outcome in infants with CNS caused by genetic mutations are scarce. METHODS: We analyzed the genetic background, clinical manifestations, treatment response, and prognosis of pediatric patients with CNS in Taiwan. RESULTS: Fifteen infants with CNS were enrolled, and 11 patients of median age 21 (interquartile range 3∼44) days caused by genetic mutations from 10 unrelated families were included in the study...
October 14, 2023: Journal of the Formosan Medical Association
#4
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
#5
JOURNAL ARTICLE
Yingchao Peng, Tao Ju, Chunlin Gao, Zhengkun Xia, Meiqiu Wang, Xiaoyi Sun, Ren Wang, Xiaojie Li, Yaqin Wei, Lili Jia, Huangyu Chen
BACKGROUND: C1q nephropathy is a relatively rare glomerulonephritis characterized by dominant mesangial deposition of C1q. Even though C1q nephropathy has been described for more than three decades, the clinicopathological features and renal outcomes remain unclear. C1q nephropathy may present diverse morphological patterns, including focal segmental glomerulosclerosis and, the notion of C1q nephropathy as a separate disease entity is still debated. This study aimed to describe the clinical and prognostic relevance of C1q nephropathy in children with primary focal segmental glomerulosclerosis...
July 10, 2023: Journal of Nephrology
#6
REVIEW
Leong Tung Ong
Acute post-streptococcal glomerulonephritis (APSGN) is the major cause of acute glomerulonephritis among children, especially in low- and middle-income countries. APSGN commonly occurs following pharyngitis due to the activation of antibodies and complements proteins against streptococcal antigens through the immune-complex-mediated mechanism. APSGN can be presented as acute nephritic syndrome, nephrotic syndrome, and rapidly progressive glomerulonephritis, or it may be subclinical. The management of APSGN is mainly supportive in nature with fluid restriction, anti-hypertensives, diuretics, and renal replacement therapy with dialysis, when necessary, as the disease is self-limiting...
September 25, 2022: World Journal of Nephrology
#7
REVIEW
Khemchand N Moorani, Madiha Aziz, Farhana Amanullah
Rapidly progressive glomerulonephritis (RPGN), characterized by a rapid development of nephritis with loss of kidney function in days or weeks, is typically associated histologically, with crescents in most glomeruli; and is a challenging problem, particularly in low resource settings. RPGN is a diagnostic and therapeutic emergency requiring prompt evaluation and treatment to prevent poor outcomes. Histopathologically, RPGN consists of four major categories, anti-glomerular basement membrane (GBM) disease, immune complex mediated, pauci-immune disorders and idiopathic /overlap disorders...
January 2022: Pakistan Journal of Medical Sciences Quarterly
#8
JOURNAL ARTICLE
Sudarsan Krishnasamy, Mukta Mantan, Kirtisudha Mishra, Kanika Kapoor, Megha Brijwal, Manish Kumar, Shobha Sharma, Swarnim Swarnim, Rajni Gaind, Priyanka Khandelwal, Pankaj Hari, Aditi Sinha, Arvind Bagga
BACKGROUND: Information on the course of SARS-CoV-2 infection in children with chronic kidney disease (CKD) is limited. METHODS: We retrospectively reviewed the presentation and outcomes of SARS-CoV-2 infection in patients with CKD followed at any of the four pediatric nephrology centers in New Delhi from April 2020 to June 2021. Outcomes, including cardiopulmonary and renal complications, were reported in relation to underlying disease category and illness severity at presentation...
April 2022: Pediatric Nephrology
#9
JOURNAL ARTICLE
Jackson Londeree, Courtney E McCracken, Larry A Greenbaum, Evan J Anderson, Laura C Plantinga, Scott E Gillespie, Chia-Shi Wang
BACKGROUND: Epidemiological data on childhood idiopathic nephrotic syndrome (INS) are limited. We estimated childhood INS incidence in a racially and ethnically diverse U.S. population and performed a meta-analysis of published reports to examine differences by race, ethnicity, and time. METHODS: One hundred seventy-five children aged 1-17 years living in the Atlanta Metropolitan Statistical Area (MSA) between 2013 and 2018 were identified by retrospective chart review...
March 2022: Journal of Nephrology
#10
JOURNAL ARTICLE
Bahriye Atmis, Aysun K Bayazit, Derya Cevizli, Deniz Kor, Hatice Busra Fidan, Atil Bisgin, Sebile Kilavuz, Ilker Unal, Kivilcim Eren Erdogan, Engin Melek, Gulfiliz Gonlusen, Ali Anarat, Neslihan Onenli Mungan
BACKGROUND: Cystinosis is a lysosomal storage disease that affects many tissues. Its prognosis depends predominantly on kidney involvement. Cystinosis has three clinical forms: nephropathic infantile, nephropathic juvenile and non-nephropathic adult. Proximal tubular dysfunction is prominent in the infantile form, whereas a combination of glomerular and tubular alterations are observed in the juvenile form. METHODS: Thirty-six children with nephropathic cystinosis were included in the study...
April 2022: Journal of Nephrology
#11
JOURNAL ARTICLE
Matko Marlais, Tanja Wlodkowski, Samhar Al-Akash, Petr Ananin, Varun Kumar Bandi, Veronique Baudouin, Olivia Boyer, Luciola Vásquez, Sukanya Govindan, Nakysa Hooman, Iftikhar Ijaz, Reyner Loza, Marta Melgosa, Nivedita Pande, Lars Pape, Anshuman Saha, Dmitry Samsonov, Michiel F Schreuder, Jyoti Sharma, Sahar Siddiqui, Rajiv Sinha, Heather Stewart, Velibor Tasic, Burkhard Tönshoff, Katherine Twombley, Kiran Upadhyay, Marina Vivarelli, Donald J Weaver, Robert Woroniecki, Franz Schaefer, Kjell Tullus
BACKGROUND: Children are recognised as at lower risk of severe COVID-19 compared with adults, but the impact of immunosuppression is yet to be determined. This study aims to describe the clinical course of COVID-19 in children with kidney disease taking immunosuppressive medication and to assess disease severity. METHODS: Cross-sectional study hosted by the European Rare Kidney Disease Reference Network and supported by the European, Asian and International paediatric nephrology societies...
December 21, 2020: Archives of Disease in Childhood
#12
JOURNAL ARTICLE
Ayşe Seda Pınarbaşı, Ismail Dursun, Ibrahim Gokce, Elif Çomak, Seha Saygılı, Meral Torun Bayram, Osman Donmez, Engin Melek, Demet Tekcan, Neslihan Çiçek, Dilek Yılmaz, Yılmaz Tabel, Zeynep Y Yıldırım, Elif Bahat, Mustafa Koyun, Alper Soylu, Nur Canpolat, Bağdagül Aksu, Mehtap Ezel Çelakıl, Mehmet Taşdemir, Meryem Benzer, Gül Özçelik, Sevcan A Bakkaloğlu, Ruhan Düşünsel
BACKGROUND: C3 glomerulopathy (C3G) is characterized by heterogeneous clinical presentation, outcome, and predominant C3 accumulation in glomeruli without significant IgG. There is scarce outcome data regarding childhood C3G. We describe clinical and pathological features, treatment and outcomes, and risk factors for progression to chronic kidney disease stage 5 (CKD5) in the largest pediatric series with biopsy-proven C3G. METHODS: Sixty pediatric patients with C3G from 21 referral centers in Turkey were included in this retrospective study...
May 2021: Pediatric Nephrology
#13
JOURNAL ARTICLE
Mohammed Alsuhaibani, Egab Aldosari, Khawla A Rahim, Saeed Alzabli, Dayel Alshahrani
BACKGROUND: Fungal peritonitis (FP) is an infrequent but serious complication in children undergoing peritoneal dialysis (PD). This study aimed to explore the risk factors, clinical manifestations, causative organisms, fungal susceptibility findings, and outcomes of FP in children from Saudi Arabia. METHODS: In this case-control study, the medical records and laboratory results of paediatric patients aged 0-14 years who underwent PD were reviewed for FP episodes...
September 16, 2020: BMC Nephrology
#14
JOURNAL ARTICLE
O Abumregha, E Naicker, C Connolly, R Bhimma
BACKGROUND: The outcome and response of idiopathic nephrotic syndrome (NS) to steroids have been linked to race. OBJECTIVES: To determine the age of presentation, sex, race, histopathology, kidney function and disease status at the last hospital visit and correlate these with steroid response in Indian and black African children with idiopathic NS. METHODS: This is a retrospective review of 231 children aged 1 - 14 years, who were seen at Inkosi Albert Luthuli Central Hospital, Durban, South Africa (SA) from 2003 to 2018...
July 7, 2020: South African Medical Journal
#15
JOURNAL ARTICLE
Yuko Hamasaki, Riku Hamada, Masaki Muramatsu, Shinsuke Matsumoto, Kunihiko Aya, Kenji Ishikura, Tetsuji Kaneko, Kazumoto Iijima
BACKGROUND: Congenital nephrotic syndrome (CNS) and infantile nephrotic syndrome (INS) cause substantial morbidity and mortality. In Japan, there is a lack of knowledge regarding the characteristics of CNS and INS. This study aimed to clarify the characteristics of CNS and INS in Japan. METHODS: This cross-sectional nationwide survey obtained data from 44 institutions in Japan managing 92 patients with CNS or INS, by means of two survey questionnaires sent by postal mail...
August 24, 2020: BMC Nephrology
#16
REVIEW
Olivia Boyer, Sandra Bérody
The management of infants with congenital nephrotic syndrome (CNS) is very challenging as they are prone to severe complications such as hemodynamic disturbances, infections, thromboses, and impaired growth, and most will develop end-stage kidney disease (ESKD) within a few years. Since the seventies, an "aggressive" approach, including daily albumin infusions, early nephrectomies, dialysis, and transplantation, has dramatically improved survival and morbidity. More recent case-note reviews have reported successful conservative treatment (using optimized nutrition, complication prophylaxis, and delayed renal replacement therapy), which led to similarly good outcomes and low complication rates...
October 2020: Pediatric Nephrology
#17
JOURNAL ARTICLE
Eefke Vos, Linda Koster-Kamphuis, Nicole C A J van de Kar, Charlotte M H H T Bootsma-Robroeks, Elisabeth A M Cornelissen, Michiel F Schreuder
Nephrotic syndrome is characterized by proteinuria, hypoalbuminemia, and general edema. These symptoms may persist in children who reach ESRD, which is unfavorable for the patient's allograft outcome. In addition, this may hamper early diagnosis of a relapse after transplantation. Surgical bilateral nephrectomy is often considered for that reason, but medical nephrectomy may be a less invasive alternative. In this retrospective single-center case series, we identified all children on dialysis with ESRD due to nephrotic syndrome in which a medical nephrectomy was attempted before kidney transplantation between 2013 and 2018...
June 2020: Pediatric Transplantation
#18
JOURNAL ARTICLE
Fatma El-Husseiny Moustafa, Riham Eid, Nashwa Hamdy
BACKGROUND: Hematuria is a common problem in pediatric practice and necessitates exhausting studies to detect etiology and establish proper management and counselling. SUBJECTS AND METHODS: We reviewed the clinical and pathological findings in 95 children presented between 2013 and 2019 with gross or microscopic hematuria with or without proteinuria in whom non-glomerular causes were excluded. In addition, a reference range for normal glomerular basement membrane thickness (GBMT) is introduced based on the assessment of biopsies of 20 steroid-resistant nephrotic syndrome cases aged 3-15 years, and with minimal change pathology...
July 2020: Clinical and Experimental Nephrology
#19
JOURNAL ARTICLE
Neus Roca, Marina Muñoz, Alejandro Cruz, Ramon Vilalta, Enrique Lara, Gema Ariceta
BACKGROUND: Denys-Drash syndrome (DDS) is a rare disease caused by mutations in exons 8 and 9 of the WT1 gene. It is characterized by the association of early onset steroid-resistant nephrotic syndrome (SRNS), Wilms' tumour and, in some patients, intersex disorders, with increasing risk of gonadoblastoma. There are few published data concerning the long-term outcome of patients with DDS. The aim of this study was to report our experience. METHODS: Data were collected from five children (three boys) with confirmed DDS diagnosed from 1996 to 2017...
December 2019: Clinical Kidney Journal
#20
MULTICENTER STUDY
Stephanie Dufek, Elisa Ylinen, Agnes Trautmann, Harika Alpay, Gema Ariceta, Christoph Aufricht, Justine Bacchetta, Sevcan Bakkaloglu, Aysun Bayazit, Salim Caliskan, Maria do Sameiro Faria, Ismail Dursun, Mesiha Ekim, Augustina Jankauskiene, Günter Klaus, Fabio Paglialonga, Andrea Pasini, Nikoleta Printza, Valerie Said Conti, Claus Peter Schmitt, Constantinos Stefanidis, Enrico Verrina, Enrico Vidal, Hazel Webb, Argyroula Zampetoglou, Alberto Edefonti, Tuula Holtta, Rukshana Shroff
BACKGROUND: Children with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in infancy and require dialysis, but little is known about the complications and outcomes of dialysis in these children. METHODS: We conducted a retrospective case note review across members of the European Society for Pediatric Nephrology Dialysis Working Group to evaluate dialysis management, complications of dialysis, and outcomes in children with CNS. RESULTS: Eighty children (50% male) with CNS were identified form 17 centers over a 6-year period...
April 2019: Pediatric Nephrology
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