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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
#22
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
#23
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
#24
Soumya Patil, Mahantesh V Patil, Apoorva Bagalkotkar, Shashikala Wali
BACKGROUND: Primary membranous nephropathy is a rare presentation in children. Patients unresponsive to steroids and experiencing frequent relapse are considered steroid-resistant. They often require complex treatment regimens consisting of immunosuppressants like cyclophosphamide, tacrolimus, and cyclosporin A. CASE: In the present case, a 5-year-old child was suffering from steroid-resistant nephrotic syndrome for the past 10 months. He was initially treated with prednisolone 20mg but was subsequently found to be steroid-resistant...
January 9, 2024: Current Drug Safety
#25
JOURNAL ARTICLE
Mengyu Wu, Wen Zhou, Shengnan Hu, Fangrong Peng, Fang Yang, Liuyi Zhang
PURPOSE: To investigate the level of readiness for discharge among parents of children with primary nephrotic syndrome and to explore the mediating role of parenting self-efficacy and parenting stress between perceived social support and readiness for discharge. DESIGN AND METHODS: A cross-sectional study was conducted in five large tertiary general hospitals in Hunan, China. Data related to demographics, perceived social support, parenting self-efficacy, parenting stress, and readiness for discharge were collected from 350 parents of children diagnosed with primary nephrotic syndrome...
January 8, 2024: Journal of Pediatric Nursing
#26
JOURNAL ARTICLE
Lasanthi Weerasooriya, Alexander J Howie, Matthew P Wakeman, Susan Cavanagh, David V Milford
BACKGROUND: Diabetic nephropathy may begin in childhood, but clinical kidney disease ascribable to this is uncommon in children with type 1 (insulin dependent) diabetes mellitus. METHODS: We reviewed our experience of kidney biopsies in children with type 1 diabetes mellitus. RESULTS: Between 1995 and 2022, there were biopsies in 17 children, with various clinical indications for kidney biopsy, making this the largest series of biopsies in diabetic children with clinical kidney abnormalities...
December 21, 2023: Pediatric Nephrology
#27
JOURNAL ARTICLE
William Wong, Chanel Prestidge, Jonathan Zwi, Dug Yeo Han
BACKGROUND: Acute post-streptococcal glomerulonephritis (APSGN) is a common cause of acute kidney injury (AKI) in children; however, in a small subgroup, the presentation is one of rapidly progressive glomerulonephritis (RPGN) deteriorating kidney function associated with severe oligo-anuria or a mixed nephritic-nephrotic picture. This study reviewed potential clinical and laboratory factors which may assist the treating clinician to identify patients at high risk of severe disease. METHODS: All kidney biopsies for APSGN performed between 1996 and 2020 were obtained from a departmental biopsy database...
December 20, 2023: Pediatric Nephrology
#28
JOURNAL ARTICLE
Amal A Al-Eisa, Maysoun Al Rushood, Sumedha Kashyap, Mohammad Z Haider
OBJECTIVES: TNF-α is a pro-inflammatory cytokine that has been implicated in many inflammatory diseases, but its association with idiopathic nephrotic syndrome (INS) is poorly understood. This study looked for an association of TNF-α gene polymorphisms with INS, as well as its effect on steroid responsiveness among Kuwaiti Arab children. METHODS: Genotypes of the TNF-a gene polymorphisms were analyzed using polymerase chain reaction-restriction fragment length polymorphism in 151 INS Kuwaiti Arab patients and 64 age and sex-matched controls...
December 6, 2023: Journal of Tropical Pediatrics
#29
JOURNAL ARTICLE
Keishiro Furuie, Shohei Kuraoka, Hideki Ban, Yuko Hidaka, Hiroko Nagata, Hiroshi Tamura, Koji Nagano, Tomoyasu Kawano, Akio Furuse, Hitoshi Nakazato, Kimitoshi Nakamura
BACKGROUND: Childhood-onset glomerular disease often requires ongoing treatment and follow-up into adulthood. However, few studies have analyzed the associated impact and distress experienced by patients with this condition during the transition from childhood to adolescence and adulthood. METHODS: At three facilities, we recruited patients who developed idiopathic nephrotic syndrome or IgA nephropathy during childhood and were at least 18 years old at the time of study entry...
December 19, 2023: Pediatric Nephrology
#30
JOURNAL ARTICLE
Khalid Alhasan, Amro Attaf Alsalmi, Weiam Almaiman, Adi J Al Herbish, Afrah Farhat, Ibrahim Sandokji, Majed Aloufi, Hassan Yahya Faqeehi, Naif Abdulmajeed, Abdulkarim Alanazi, Abdulaziz AlHassan, Abdulaziz Alshathri, Abeer Mohammad Almalki, Afaf Alawi Bafageeh, Ali M Aldajani, Ashraf AlMuzain, Faten Sudan Almuteri, Haydar Hassan Nasser, Khalid Al Alsheikh, Khamisa Mohamed Almokali, Magbul Maghfuri, Mohamed Waleed Abukhatwah, Muawia Abdulla Mohamed Ahmed, Naeima Fatani, Naffaa Al-Harbi, Rezqah Fajor AlDhaferi, Sulaiman Amohaimeed, Zahra Hussain AlSannaa, Mohamed A Shalaby, Rupesh Raina, Dieter Clemens Broering, Jameela A Kari, Mohamad-Hani Temsah
BACKGROUND: This study aimed to determine the prevalence and etiology of kidney failure (KF) among children below 15 years of age receiving chronic dialysis in Saudi Arabia and describe their dialysis modalities. METHODS: This cross-sectional descriptive study was conducted on 8 August 2022, encompassing all 23 pediatric dialysis centers in Saudi Arabia. Data gathered comprised patient demographics, causes of KF, and the dialysis methods employed. Collected data underwent analysis to determine prevalence of children undergoing chronic dialysis, discern underlying causes of KF, and evaluate distribution of patients across different dialysis modalities...
December 13, 2023: Pediatric Nephrology
#31
JOURNAL ARTICLE
Dahai Wang, Chunrong Shan, Tingting Gao, Jia Liu, Ranran Zhang, Qiuye Zhang, Hong Chang, Yi Lin
OBJECTIVE: To explore the clinical characteristics and genetic etiology for a child with atypical Hemolytic uremic syndrome (aHUS) in conjunct with nephrotic level proteinuria. METHODS: A child patient who had visited the Affiliated Hospital of Qingdao University on June 25, 2020 was selected as the study subject. Clinical data of the patient was collected. Whole exome sequencing (WES) was carried out for the child, and candidate variant was verified by Sanger sequencing of the child and his parents...
December 10, 2023: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
#32
JOURNAL ARTICLE
Bobbity Deepthi, Ramge Ramachandran Sivakumar, Sudarsan Krishnasamy, Debasis Gochhait, Kausik Mandal, Sriram Krishnamurthy
A two-and-a-half-month-old female infant presented with generalized edema for 10 days. At presentation, she had periorbital puffiness, moderate ascites, and pedal edema. Laboratory investigations revealed serum albumin 1.3 g/dL, spot urine protein to creatinine ratio (Up:Uc) 20.87 mg/mg, total cholesterol 380 mg/dL, and serum creatinine 0.31 mg/dL. Exome sequencing revealed compound heterozygous variants in LAMA5 gene (NM_005560.6). There was a heterozygous likely pathogenic missense variant in exon 2: LAMA5: c...
November 20, 2023: Pediatric Nephrology
#33
JOURNAL ARTICLE
Priyanka S Amonkar, Suhas N Kharche, Amol A Madave, Jeetendra B Gavhane
Although presentation of multisystem inflammatory syndrome (MIS-C) in children is typically with fever and symptoms like diarrhea, vomiting, rash, conjunctival injection, or shock, the spectrum of associated multisystem involvement is wide. Here, we present an account of patients with MIS-C that presented at a tertiary hospital in Navi Mumbai, India in the latter half of the pandemic from October 2020 to January 2021. We retrospectively studied the clinical features of 12 patients satisfying World Health Organization criteria for MIS-C...
December 2023: Journal of Pediatric Intensive Care
#34
JOURNAL ARTICLE
Paraselli Saiteja, Bobbity Deepthi, Sudarsan Krishnasamy, Madhileti Sravani, Sriram Krishnamurthy
BACKGROUND: In pediatric steroid-resistant nephrotic syndrome (SRNS), calcineurin inhibitors (CNIs) are recommended as first-line therapy, with efficacy ranging between 60 and 80%, implying a substantial proportion will exhibit CNI resistance. Which alternate immunosuppressive therapy should be used in non-genetic pediatric SRNS exhibiting CNI resistance is especially relevant in low- to middle-income countries (LMIC), where the prohibitive costs of certain drugs such as monoclonal antibodies often determine therapy choice...
April 2024: Pediatric Nephrology
#35
Pankaj V Deshpande
Renal involvement in systemic lupus erythematosus (SLE) is well known. We present a 16-year-old boy, who was in remission on treatment for SLE for the past three years and now presented with nephrotic syndrome. The kidney biopsy was normal with the immunofluorescence revealing no deposits. He went into remission by day 9 of treatment and completed the course of treatment with prednisolone alone with no relapses. The clinical picture along with the histology and autoimmune markers for SLE indicate that he developed minimal change nephrotic syndrome that was responsive to prednisolone...
2023: Indian Journal of Nephrology
#36
Zhuo Deng, Qi Zhou, Tai-Guang Zhou
BACKGROUND: Historically, due to the lack of distinct clinical symptoms, Alport syndrome, a hereditary kidney disease prevalent in children and a leading cause of kidney failure, has often been misdiagnosed as other kidney conditions. CASE DESCRIPTION: This article presents a comprehensive review and analysis of clinical data concerning a child diagnosed with Alport syndrome, where nephrotic syndrome served as the primary manifestation. The male child in this case exhibited symptoms starting at the age of 6, initially diagnosed as nephrotic syndrome...
October 20, 2023: Transplant Immunology
#37
JOURNAL ARTICLE
Zhara Pouransiri, Farahnak Assadi, Masoumeh Mohkam, Nakysa Hooman, Zahra Rostami, Mojgan Mazaheri, Anoush Azarfar, Fatemeh Ghane Sharbaf
OBJECTIVE: This systematic review and meta-analysis aimed to explore rituximab (RTX) associated infectious complications in children with glomerular disease. METHODS: We performed an electronic search of PubMed, International Scientific Information (ISI), Scopus, and EMBASE between January 2010 and July 2021. Infection rates and total drug-related adverse events were the outcomes. Statistical heterogeneity was evaluated by using the I2 statistic. When there was statistical evidence of heterogeneity ( I 2 > 50%, p > 0...
2023: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
#38
JOURNAL ARTICLE
Jonathan S Chawla, Joseph R Angelo, Michael C Braun
BACKGROUND: Current guidelines note a gap in high-quality evidence regarding utility of kidney ultrasonography (KUS) during initial evaluation of nephrotic syndrome (NS) due to presumed minimal change disease (pMCD). However, KUS is frequently obtained at our institution. This retrospective chart review assessed incidence and impact of abnormal sonographic findings in these patients. METHODS: Patients 1-18 years, newly diagnosed at our institution with NS from pMCD between 2011 and 2021, were identified...
October 4, 2023: Pediatric Nephrology
#39
JOURNAL ARTICLE
Rui Gu, Qin Wang, Peipei Shi, Yingying Zhang, Daojing Ying, Yuanzhao Zhi, Jianjiang Zhang
BACKGROUND: Pathogenesis and relapse of steroid-sensitive nephrotic syndrome (SSNS) are primarily associated with infection. Dental caries is the most common chronic progressive oral infection in children. However, clinical studies of SSNS combined with dental caries in children are rare. METHODS: In our retrospective cohort study from January 2021 to June 2022, 145 children with SSNS were included in the baseline analysis and 105 in the follow-up analysis. The follow-up period was 1 year...
October 2, 2023: Pediatric Nephrology
#40
JOURNAL ARTICLE
Tian Chang, Zhi Han, Xiao Liu, Panpan Wang
OBJECTIVE: To investigate the clinical features and genetic variant of a child with X-linked dominant Alport syndrome (XLAS). METHODS: A child who had presented at the First Affiliated Hospital of Zhengzhou University in May 2019 was selected as the study subject. Clinical data of the child was collected. Next generation sequencing (NGS) was carried out for the child. Candidate variants were validated by Sanger sequencing of his family members. RESULTS: The child, a 12-year-old boy, had mainly manifested gross hematuria, proteinuria, nephrotic syndrome, and progressive renal impairment in conjunct with hearing loss...
October 10, 2023: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
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