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JOURNAL ARTICLE
Eun Mi Yang, Jayoun Kim, Eujin Park, Kyoung Hee Han, Seong Heon Kim, Heeyeon Cho, Jae Il Shin, Min Hyun Cho, Joo Hoon Lee, Ji Hyun Kim, Hee Gyung Kang, Il-Soo Ha, Yo Han Ahn
BACKGROUND: The natural course of chronic kidney disease (CKD) progression in children varies according to their underlying conditions. This study aims to identify different patterns of subsequent decline in kidney function and investigate factors associated with different patterns of estimated glomerular filtration rate (eGFR) trajectories. METHODS: We analyzed data from the KNOW-Ped CKD (KoreaN cohort study for Outcomes in patients With Pediatric Chronic Kidney Disease), which is a longitudinal, prospective cohort study...
February 23, 2024: Kidney Research and Clinical Practice
#22
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
#23
Min Song Jeong, Yun Young Choi, Yo Han Ahn, Kyeonghun Lee, Ji Soo Park, Dong In Suh
Toxic epidermal necrolysis (TEN) is a life-threatening mucocutaneous disorder commonly caused by drugs. TEN is often treated with corticosteroids, intravenous immunoglobulin (IVIG), or cyclosporine; however, the efficacy of these treatments is controversial. Etanercept (a TNF-α antagonist) was proven to decrease skin-healing time in a randomized clinical trial. Herein, we report the case of a 44-month-old boy who developed TEN due to deflazacort as the probable culprit drug and was successfully treated with etanercept...
2024: Frontiers in Immunology
#24
JOURNAL ARTICLE
Mercedes Lopez-Gonzalez, Gema Ariceta
Historically, specific mutations in WT1 gene have been associated with distinct syndromes based on phenotypic characteristics, including Denys-Drash syndrome (DDS), Frasier syndrome (FS), Meacham syndrome, and WAGR syndrome. DDS is classically defined by the triad of steroid-resistant nephrotic syndrome (SRNS) onset in the first year of life, disorders of sex development (DSD), and a predisposition to Wilms tumor (WT). Currently, a paradigm shift acknowledges a diverse spectrum of presentations beyond traditional syndromic definitions...
February 7, 2024: Pediatric Nephrology
#25
JOURNAL ARTICLE
Y Z Zhi, L Cao, D J Ying, W J Dou, R Gu, J J Zhang
Objective: To investigate the incidence and influencing factors of hypogammaglobulinemia (HGG) in children with steroid-dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS) treated with rituximab (RTX), and its relationship with the risk of severe infections. Methods: The clinical data of children with SDNS/FRNS treated with RTX at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University from December 2020 to January 2023 were retrospectively analyzed. RTX treatment was performed using a B-cell-guided regimen (a single dose of 375 mg/m2 , a maximum of 500 mg/dose, and an additional one dose when reassessment of peripheral blood CD19+ B cells≥1%)...
February 6, 2024: Zhonghua Yi Xue za Zhi [Chinese medical journal]
#26
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
#27
JOURNAL ARTICLE
Johnathon Carboni, Elizabeth Thomas, Debbie S Gipson, Tammy M Brady, Tarak Srivastava, David T Selewski, Larry A Greenbaum, Chia-Shi Wang, Katherine M Dell, Frederick Kaskel, Susan Massengill, Kimberly Reidy, Cheryl L Tran, Howard Trachtman, Richard Lafayette, Salem Almaani, Sangeeta Hingorani, Rasheed Gbadegesin, Keisha L Gibson, Christine B Sethna
BACKGROUND: In the current study, longitudinal BP and lipid measurements were examined in a NEPTUNE cohort of children with newly diagnosed nephrotic syndrome (cNEPTUNE). We hypothesized that hypertensive BP and dyslipidemia would persist in children with nephrotic syndrome, regardless of steroid treatment response. METHODS: A multi-center longitudinal observational analysis of data obtained from children < 19 years of age with new onset nephrotic syndrome enrolled in the Nephrotic Syndrome Study Network (cNEPTUNE) was conducted...
February 6, 2024: Pediatric Nephrology
#28
JOURNAL ARTICLE
Shoichi Shimizu, Hiroshi Saito, Shori Takahashi, Tamaki Morohashi, Riku Hamada, Hiroshi Hataya, Yoshiaki Kondo, Ichiro Morioka
BACKGROUND: The urine protein to creatinine ratio (UPCR) correlates well with the 24-h urine protein test (24-h UPT) and is a reliable indicator of proteinuria. However, in nephrotic syndrome, the correlation between the UPCR and the 24-h UPT tends to decrease. To address this, we introduced the fractional excretion of total protein (FETP), which reflects serum total protein and creatinine levels because severe hypoproteinemia and/or elevated serum creatinine levels tend to occur under these conditions...
2024: Pediatrics International: Official Journal of the Japan Pediatric Society
#29
JOURNAL ARTICLE
Itamar Oren, Violeta Temper, Ayelet Michael-Gayego, Yair Motro, Oded Volovelsky, Jacob Moran-Gilad, Oren Gordon
Helicobacter cinaedi is known to cause invasive infections in immunocompromised adults. Here we report the first case of H. cinaedi bacteremia in a child with nephrotic syndrome. The patient presented with a mild transient febrile illness that resolved spontaneously. We discuss the diagnostic challenges associated with this case and the microbiologic approach, including genomic analysis. Furthermore, we review the current case together with all previous pediatric cases (n = 6). Notably, all cases involved neonates or otherwise immunocompromised individuals and were characterized by severe disease with complicated infections (eg, meningitis, cholangitis and arthritis)...
January 31, 2024: Pediatric Infectious Disease Journal
#30
JOURNAL ARTICLE
Jyoti Sharma, Anshuman Saha, Alpana Ohri, Vaishali More, Fagun Shah, Jalpa Dave, Brinda Panchal Jain, Manoj Matnani, K Sathe, Pankaj Bhansali, Puneet Chhajed, Pawan Deore, Nivedita Pande, Chintan Shah, Vala Kinnari, Jyoti Singhal, Nisha Krishnamurthy, Meenal Agarwal, Uma Ali
BACKGROUND: Eighty-five percent of infants with congenital nephrotic syndrome (CNS) and 66% with infantile NS (INS) are likely to have a monogenic etiology. There exists a significant genetic variability between different regions and ethnic groups. This study aimed to determine the genetic defects in children with CNS and INS by establishing a registry in western India. METHODS: In this cross-sectional study, pediatric nephrologists from 13 private and government institutions shared relevant clinical data and details of the genetic evaluation of children presenting with NS within the first year of life...
January 31, 2024: Pediatric Nephrology
#31
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
#32
JOURNAL ARTICLE
Ross Tollaksen, Randall D Craver, Ihor V Yosypiv
Steroid resistant nephrotic syndrome (SRNS) accounts for 30% of all cases of nephrotic syndrome (NS) in children and frequently leads to end stage kidney disease (ESKD). About 30% of children with SRNS demonstrate causative mutations in podocyte- associated genes. Early identification of genetic forms of SRNS is critical to avoid potentially harmful immunosuppressive therapy. A 2-year-old male patient with NS and no family history of renal disease did not respond to 4-week steroid treatment. Kidney biopsy demonstrated mesangial proliferative glomerulopathy with basement membrane dysmorphism...
January 30, 2024: Pediatric and Developmental Pathology
#33
JOURNAL ARTICLE
Yoko Sobue, Kentaro Nishi, Koichi Kamei, Yuta Inoki, Kei Osaka, Tomoya Kaneda, Misaki Akiyama, Mai Sato, Masao Ogura, Kenji Ishikura, Akira Ishiguro, Shuichi Ito
BACKGROUND: Despite adverse events associated with the long-term use of immunosuppressants, their long-term discontinuation remains challenging in children with idiopathic nephrotic syndrome. Relapse and resumption of immunosuppressants after discontinuation and associated risk factors were analyzed. METHODS: This single-center retrospective cohort study included children with frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS) or steroid-resistant nephrotic syndrome (SRNS) who initiated immunosuppressant treatment between 2010 and 2020...
January 25, 2024: Pediatric Nephrology
#34
JOURNAL ARTICLE
Yuta Inoki, Kentaro Nishi, Kei Osaka, Tomoya Kaneda, Misaki Akiyama, Mai Sato, Masao Ogura, Koichi Kamei
BACKGROUND: Nephrotic syndrome relapse within 6 months is a known risk factor for steroid-dependent nephrotic syndrome/frequently relapsing nephrotic syndrome (SDNS/FRNS), but the risk of early development of SDNS/FRNS and initiation of immunosuppression therapy remains unknown. METHODS: Patients with childhood-onset idiopathic nephrotic syndrome who had the first relapse within 6 months were enrolled. We analyzed the relationship between the time of the first relapse or the time of initial remission and incidence of SDNS/FRNS or initiation of immunosuppression therapy...
January 25, 2024: Pediatric Nephrology
#35
JOURNAL ARTICLE
Abir Boussetta, Khouloud Ben Njima, Manel Jellouli, Taher Gargah
INTRODUCTION: Focal segmental glomerulosclerosis is a histopathological entity. AIM: To analyze the epidemiological, clinical and histological profile of primary focal segmental glomerulosclerosis in children, as well as their prognostic factors. METHODS: This was a retrospective cross-sectional study over a period of 20 years (2001-2020), conducted in the Department of Pediatrics at Charles Nicolle Hospital in Tunis, which included children followed for primary focal segmental glomerulosclerosis...
March 5, 2023: La Tunisie Médicale
#36
JOURNAL ARTICLE
Deepthi Avvaru, M Santhosh Reddy, Shinaj Azar Ms, Shashikala Wali, Mahantesh V Patil, Ramesh Bhandari, M S Ganachari
BACKGROUND: Nephrotic syndrome (NS) is a commonly encountered chronic kidney disease in pediatric populations, with South Asian children being at high risk and requiring long-term pharmacological management. Thus, identifying medication discrepancies and evaluating the appropriateness of therapy and its economic burden are vital for inpatient management. The aim of the study was to assess medication reconciliation, medication appropriateness, and cost analysis in NS cases. METHODS: An ambispective cohort observational study was carried out with 150 NS patients where medication discrepancies were identified retrospectively and prospectively using the best possible medication history and following up patients correspondingly...
January 22, 2024: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
#37
JOURNAL ARTICLE
Ganesh M Krishna, Aashima Dabas, Mukta Mantan, Akshay Kumar M, Binita Goswami
BACKGROUND: Children with nephrotic syndrome are exposed to alternate day steroids for prolonged periods and this poses the need for evaluation of adrenocortical suppression using the adrenocorticotropic hormone (ACTH) stimulation test. METHODS: This cross-sectional study enrolled children (2-18 years) both with steroid sensitive nephrotic syndrome (SSNS) (n = 27) and steroid resistant (SRNS) (n = 25); those on daily prednisolone or having serious bacterial infections or hospitalized were excluded...
January 23, 2024: Pediatric Nephrology
#38
JOURNAL ARTICLE
Howard Trachtman, Hailey Desmond, Amanda L Williams, Laura H Mariani, Sean Eddy, Wenjun Ju, Laura Barisoni, Heather K Ascani, Wendy R Uhlmann, Cathie Spino, Lawrence B Holzman, John R Sedor, Crystal Gadegbeku, Lalita Subramanian, Chrysta C Lienczewski, Tina Manieri, Scott J Roberts, Debbie S Gipson, Matthias Kretzler
Glomerular diseases are classified using a descriptive taxonomy that is not reflective of the heterogeneous underlying molecular drivers. This limits not only diagnostic and therapeutic patient management, but also impacts clinical trials evaluating targeted interventions. The Nephrotic Syndrome Study Network (NEPTUNE) is poised to address these challenges. The study has enrolled >850 pediatric and adult patients with proteinuric glomerular diseases who have contributed to deep clinical, histologic, genetic, and molecular profiles linked to long-term outcomes...
February 2024: Kidney International
#39
JOURNAL ARTICLE
Tanatuch Tiratrakoonseree, Suwanat Charoenpichitnun, Rungrote Natesirinilkul, Natsaruth Songthawee, Patcharee Komvilaisak, Pongpak Pongphitcha, Jarin Vaewpanich, Nongnuch Sirachainan
INTRODUCTION: The diagnosis of pediatric pulmonary embolism (PE) is often delayed due to non-specific symptoms, and clinical prediction tools designed for adults are unsuitable for children. This study aimed to create a PE predictive model and to evaluate the reported tools in the Thai pediatric population. MATERIALS AND METHODS: A multi-center retrospective study from 4 university hospitals included children ≤18 years of age undergoing computed tomography pulmonary angiogram from 2000 to 2020 with the suspicion of PE...
February 2024: Thrombosis Research
#40
REVIEW
Zubin J Modi, Yan Zhai, Jennifer Yee, Hailey Desmond, Wei Hao, Matthew G Sampson, Christine B Sethna, Chia-Shi Wang, Debbie S Gipson, Howard Trachtman, Matthias Kretzler
Primary glomerular diseases are rare entities. This has hampered efforts to better understand the underlying pathobiology and to develop novel safe and effective therapies. NEPTUNE is a rare disease network that is focused on patients of all ages with minimal change disease, focal segmental glomerulosclerosis, and membranous nephropathy. It is a longitudinal cohort study that collects detailed demographic, clinical, histopathologic, genomic, transcriptomic, and metabolomic data. The goal is to develop a molecular classification for these disorders that supersedes the traditional pathological features-based schema...
January 18, 2024: Pediatric Nephrology
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