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JOURNAL ARTICLE
Cal H Robinson, Nowrin Aman, Tonny Banh, Josefina Brooke, Rahul Chanchlani, Vaneet Dhillon, Valerie Langlois, Leo Levin, Christoph Licht, Ashlene McKay, Damien Noone, Alisha Parikh, Rachel Pearl, Seetha Radhakrishnan, Veronique Rowley, Chia Wei Teoh, Jovanka Vasilevska-Ristovska, Rulan S Parekh
BACKGROUND: Children with nephrotic syndrome are at risk of obesity and growth impairment from repeated steroid treatment. However, incidence and risk factors for obesity and short stature remain uncertain, which is a barrier to preventative care. Our aim was to determine risk, timing, and predictors of obesity and short stature among children with nephrotic syndrome. METHODS: We evaluated obesity and longitudinal growth among children (1-18 years) enrolled in Insight into Nephrotic Syndrome: Investigating Genes, Health, and Therapeutics...
April 18, 2024: Pediatric Nephrology
#2
JOURNAL ARTICLE
Gioacchino Andrea Rotulo, Claudia Campanello, Marcella Battaglini, Marta Bassi, Carlotta Pastorino, Andrea Angeletti, Giacomo Brisca, Sara Signa, Roberta Caorsi, Gian Marco Ghiggeri
Allopurinol-induced drug reaction syndrome with eosinophilia and systemic symptoms (A-DRESS) is a well-described condition in adults, whereas it is uncommon among children. We describe a case of A-DRESS in a 16-year-old male with steroid-dependent nephrotic syndrome. He presented a life-threatening clinical course with persisting fever, skin rash, eosinophilia, lymphadenopathy, distributive shock, and herpesvirus 6 detection. The withdrawal of allopurinol and a combination of intravenous immunoglobulins (IVIGs) and systemic corticosteroids led to the patient's recovery without sequelae...
April 2024: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
#3
JOURNAL ARTICLE
Patrícia Sousa, Catarina Brás, Catarina Menezes, Ramon Vizcaino, Teresa Costa, Maria Sameiro Faria, Conceição Mota
INTRODUCTION: Percutaneous kidney biopsy (KB) is crucial to the diagnosis and management of several renal pathologies. National data on native KB in pediatric patients are scarce. We aimed to review the demographic and clinical characteristics and histopathological patterns in children who underwent native percutaneous KB over 24 years. METHODS: Retrospective observational study of patients undergoing native percutaneous KB in a pediatric nephrology unit between 1998 and 2021, comparing 3 periods: period 1 (1998-2005), period 2 (2006-2013), and period 3 (2014-2021)...
2024: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
#4
JOURNAL ARTICLE
Habib Qaiser, Irshad Bajeer, Sabeeta Khatri, Seema Hashmi, Ali Asghar Lanewala
OBJECTIVE: To determine the clinico-pathological features and long-term outcome of secondary steroid-resistant nephrotic syndrome treated with steroids and calcineurin inhibitors. METHODS: The retrospective cohort study was conducted at the Sindh Institute of Urology and Transplant, Karachi, in June and July 2023, and comprised data from January 1, 2008, to December 31, 2020, of children aged 1-18 years who developed steroid resistance after initial sensitivity to steroids with at least 1-year of follow-up...
March 2024: JPMA. the Journal of the Pakistan Medical Association
#5
JOURNAL ARTICLE
Rajiv Sinha, Subhankar Sarkar, Sushmita Banerjee, Shakil Akhtar, Sanjukta Poddar, Deblina Dasgupta, Rana Saha, Jayati Sengupta, Mita Mandal, Yincent Tse, Amitava Pahari
BACKGROUND: Levamisole is a commonly used steroid-sparing agent (SSA), but the reported incidence of antineutrophil cytoplasmic antibody (ANCA) positivity has been concerning. METHODS: Observational cross-sectional study wherein children aged 2 to 18 years with frequently relapsing/steroid dependent nephrotic syndrome (FRNS/SDNS) on levamisole for ≥ 12 months were tested for ANCA. RESULTS: A total of 210 children (33% female), median age of 7...
April 8, 2024: Pediatric Nephrology
#6
JOURNAL ARTICLE
Toru Nagata, Kenji Nakagawa, Fumitoshi Tsurumi, Ken Watanabe, Tomomi Endo, Atsuko Hata
Nuclear factor kappa B (NF-κB) family plays a central role in the human immune system. Heterozygous variants in NFKB2 typically cause immunodeficiency with various degrees of central adrenal insufficiency, autoimmunity, and ectodermal dysplasia. No reported case has presented kidney failure as an initial symptom. Moreover, documentation of kidney involvement of this disease is limited. CASE DIAGNOSIS: A 2-year-old female who presented with dyspnea and hypertensive emergency in the setting of new-onset nephrotic syndrome with acute-on chronic kidney injury with resultant chronic kidney disease (CKD) was found to have a novel heterozygous N-terminal variant in NFKB2 (c...
April 8, 2024: Pediatric Nephrology
#7
JOURNAL ARTICLE
Hala Wannous
BACKGROUND: Idiopathic nephrotic syndrome (INS) is the most common glomerular disease in children. We performed this study to report histopathological findings, the correlation between clinical and histopathological features, and the response to steroids and other immunosuppressive drugs and outcomes in Syrian children with INS. METHODS: A single-center retrospective observational cohort study was conducted at Children's University Hospital in Damascus, and included all patients aged 1-14 years, admitted from January 2013 to December 2022, with INS and who underwent kidney biopsy...
April 8, 2024: Pediatric Nephrology
#8
JOURNAL ARTICLE
Fatima S Naim, Yassir M Bakhiet, Mohmmed A Mohmmedahmed, Bashir A Yousef
BACKGROUND: Nephrotic syndrome (NS) is an essential chronic disease in children that has a major impact on a child's health-related quality of life (HRQoL). This study aimed to evaluate the HRQoL of Sudanese children with NS and clinical parameters that can influence their HRQoL. METHODS: This study was a descriptive cross-sectional of children with NS conducted in Khartoum state hospitals. A standardized PedsQLTM 4.0 Scale Score evaluated the HRQoL of the participants...
2024: Pediatric Health, Medicine and Therapeutics
#9
JOURNAL ARTICLE
Shuai Tang, Yang Yang, Xiang Li, Bing-Yang Bie, Jian Zhang
OBJECTIVES: To observe the correlation between growth impairment induced by long-term oral glucocorticoids (GC) therapy and the ratio of FGF23/Klotho in children with primary nephrotic syndrome (PNS). METHODS: A prospective study was conducted on 56 children with GC-sensitive PNS who had discontinued GC therapy for more than 3 months and revisited the Department of Pediatrics of the First Affiliated Hospital of Henan University of Traditional Chinese Medicine between June 2022 and December 2022...
March 15, 2024: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
#10
JOURNAL ARTICLE
Sirmen Kızılcan Çetin, Elif Özsu, Zeynep Şıklar, Hasan Fatih Çakmaklı, Gizem Şenyazar, Zehra Aycan, Serdar Ceylaner, Merih Berberoğlu
MIRAGE syndrome is a rare multisystemic disorder characterized by various manifestations, such as myelodysplasia, susceptibility to infections, growth retardation, adrenal hypoplasia, genital anomalies, and enteropathy. In the literature, there have been rare cases of dysautonomia. We present a 6.5-year-old girl, who was first admitted to our department with short stature. On follow up, she exhibited multiple endocrinological issues, including transient hypothyroidism, primary hypoparathyroidism and dysautonomia, along with multisystem involvement...
March 26, 2024: Journal of Clinical Research in Pediatric Endocrinology
#11
Kyle N Goodman, Pongpratch Puapatanakul, Kevin T Barton, Mai He, Jeffrey H Miner, Joseph P Gaut
Congenital nephrotic syndrome is an autosomal recessive inherited disorder that manifests as steroid-resistant massive proteinuria in the first three months of life. Defects in the glomerular filtration mechanism are the primary etiology. We present a child who developed severe nephrotic syndrome at two weeks of age and eventually required a bilateral nephrectomy. Genetic testing revealed compound heterozygous variants in NPHS1 including a known pathogenic variant and a missense variant of uncertain significance...
2024: Case Reports in Nephrology
#12
JOURNAL ARTICLE
Kinnari Vala, Kanisha Shah, Shahenaz Kapadia, Mahipal Khandelwal, Amit Jojera, Shailesh Soni, Ashka Prajapati, Anshuman Saha
Lipoprotein glomerulopathy (LPG) is a rare condition of renal lipidosis characterized by lipoprotein thrombi in glomeruli, an abnormal plasma lipoprotein profile, and a marked increase in serum apolipoprotein E (apo E) levels. It is a monogenic disorder with autosomal dominant inheritance and the average age of presentation is 32 years (4-69 years). It is rare in children. The presentation can be nephrotic syndrome, hematuria, or progressive renal failure. Here we report the first described case of LPG in an Indian 7...
March 4, 2024: CEN Case Reports
#13
JOURNAL ARTICLE
Hamsa Vasantha Shekar Reddy, Divya S Nagabushana, Deepthi Kattamanchi, Ahish Dakappa
We report a child with steroid-dependent nephrotic syndrome presenting with excessive irritability, double vision and inability to walk for 5 days. On examination, the child was irritable with Glasgow coma sccale (GCS of 12/15, had bilateral convergent squint (R>L), vertical nystagmus, ataxia without any focal neurological deficits and normal fundus. MRI brain with venogram showed bilateral symmetric FLAIR hyperintensity in the medial thalamus and periaqueductal grey matter showing diffuse restriction with normal venogram...
February 27, 2024: BMJ Case Reports
#14
MULTICENTER STUDY
Zhen Wang, Nan Wang, Ruyue Chen, Hanyun Tang, Qiang Lin, Xiaozhong Li
OBJECTIVE: To analyze the clinical effect of urokinase on the prevention of thrombosis in children with primary nephrotic syndrome. METHODS: A total of 370 children diagnosed with primary nephrotic syndrome (PNS) in the Children's Hospital of Soochow University and Zibo Maternal and Child Health Hospital from January 2018 to December 2022 were selected as the research objects. The patients were divided into a urokinase adjuvant therapy group and non-urokinase adjuvant therapy group according to the application of drugs...
February 26, 2024: BMC Nephrology
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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