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proteinuria in children

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https://www.readbyqxmd.com/read/28339709/clinicopathologic-correlations-of-renal-pathology-in-adult-population-of-poland
#1
Agnieszka Perkowska-Ptasinska, Artur Bartczak, Malgorzata Wagrowska-Danilewicz, Agnieszka Halon, Krzysztof Okon, Aldona Wozniak, Marian Danilewicz, Henryk Karkoszka, Andrzej Marszalek, Jolanta Kowalewska, Andrzej Mroz, Agnieszka Korolczuk, Andrzej Oko, Alicja Debska-Slizien, Beata Naumnik, Zbigniew Hruby, Marian Klinger, Kazimierz Ciechanowski, Marek Myslak, Wladyslaw Sulowicz, Andrzej Rydzewski, Andrzej Wiecek, Jacek Manitius, Tadeusz Gregorczyk, Stanislaw Niemczyk, Michal Nowicki, Ryszard Gellert, Tomasz Stompor, Monika Wieliczko, Krzysztof Marczewski, Leszek Paczek, Olga Rostkowska, Dominika Deborska-Materkowska, Grazyna Bogdanowicz, Andrzej Milkowski, Magdalena Durlik
Background: This is the first report on the epidemiology of biopsy-proven kidney diseases in Poland. Methods: The Polish Registry of Renal Biopsies has collected information on all (n = 9394) native renal biopsies performed in Poland from 2009 to 2014. Patients' clinical data collected at the time of biopsy, and histopathological diagnoses were used for epidemiological and clinicopathologic analysis. Results: There was a gradual increase in the number of native renal biopsies performed per million people (PMP) per year in Poland in 2009-14, starting from 36 PMP in 2009 to 44 PMP in 2014...
February 11, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28336816/glomerular-density-and-volume-in-renal-biopsy-specimens-of-children-with-proteinuria-relative-to-preterm-birth-and-gestational-age
#2
Kentaro Koike, Yohei Ikezumi, Nobuo Tsuboi, Go Kanzaki, Kotaro Haruhara, Yusuke Okabayashi, Takaya Sasaki, Makoto Ogura, Akihiko Saitoh, Takashi Yokoo
BACKGROUND AND OBJECTIVES: A low total nephron number, which is associated with low birth weight (LBW), may indicate increased susceptibility to early-onset renal diseases in children. However, few studies have assessed renal biopsy findings in LBW children. We examined the relationship between LBW and glomerular density (GD) and/or glomerular volume (GV) in renal biopsy samples as a surrogate for total nephron number. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Renal biopsy findings of children of LBW were compared with those of age-matched control subjects of normal birth weight (NBW) who were histopathologically diagnosed with FSGS or minimal change nephrotic syndrome (MCNS) from 1995 to 2011...
March 23, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28332779/intrafamilial-phenotypic-variability-in-a-polish-family-with-sensenbrenner-syndrome-and-biallelic-wdr35-mutations
#3
Joanna Walczak-Sztulpa, Anna Wawrocka, Agata Sobierajewicz, Lukasz Kuszel, Jan Zawadzki, Ryszard Grenda, Anna Swiader-Lesniak, Beata Kocyla-Karczmarewicz, Anna Wnuk, Anna Latos-Bielenska, Krystyna H Chrzanowska
Sensenbrenner syndrome (cranioectodermal dysplasia, CED) is a very rare autosomal recessive ciliopathy. Cranioectodermal dysplasia is characterized by craniofacial, skeletal, and ectodermal abnormalities. About 50 patients have been described to date. Sensenbrenner syndrome belongs to a group of ciliary chondrodysplasias and is a genetically heterogeneous disorder. Mutations in five genes: IFT122, WDR35, IFT43, WDR19, and IFT52 have been associated with CED. All known genes encode proteins that are part of the intraflagellar transport complex, which plays an important role in the assembly and maintenance of cilia...
March 23, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28329394/intermediate-follow-up-of-pediatric-patients-with-hemolytic-uremic-syndrome-during-the-2011-outbreak-caused-by-e-coli-o104-h4
#4
Sebastian Loos, Wiebke Aulbert, Bernd Hoppe, Thurid Ahlenstiel-Grunow, Birgitta Kranz, Charlotte Wahl, Hagen Staude, Alexander Humberg, Kerstin Benz, Martin Krause, Martin Pohl, Max C Liebau, Raphael Schild, Johanna Lemke, Ortraud Beringer, Dominik Müller, Christoph Härtel, Marianne Wigger, Udo Vester, Martin Konrad, Dieter Haffner, Lars Pape, Jun Oh, Markus J Kemper
Background.: In 2011 Escherichia coli O104:H4 caused an outbreak with over 800 cases of hemolytic uremic syndrome (HUS) in Germany, including 90 children. Data on the intermediate outcome in children after HUS due to E. coli O104:H4 have been lacking. Methods.: Follow-up data were gathered retrospectively from the medical records of patients who had been included in the German Pediatric HUS Registry during the 2011 outbreak. Results.: A total of 72/89 (81%) of the patients was included after a median follow-up of 3...
March 13, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28314744/randomised-controlled-trial-comparing-ofatumumab-to-rituximab-in-children-with-steroid-dependent-and-calcineurin-inhibitor-dependent-idiopathic-nephrotic-syndrome-study-protocol
#5
Pietro Ravani, Alice Bonanni, Gian Marco Ghiggeri
INTRODUCTION: Oral steroids induce remission in about 90% of children with idiopathic nephrotic syndrome (INS), which is characterised by severe proteinuria and hypoalbuminaemia. Some children become steroid-dependent (SD) and require addition of calcineurin inhibitors (CNI) to maintain remission. Since these oral agents are toxic, alternative interventions are needed for long-term treatment. The anti-CD20 antibody rituximab has shown promising steroid-sparing properties in clinical trials, but benefits are less convincing in complicated forms of SD-INS...
March 17, 2017: BMJ Open
https://www.readbyqxmd.com/read/28299461/efficacy-of-higher-dose-levamisole-in-maintaining-remission-in-steroid-dependant-nephrotic-syndrome
#6
Asiri S Abeyagunawardena, Umeshi Karunadasa, Heshan Jayaweera, Shenal Thalgahagoda, Sampath Tennakoon, Shamali Abeyagunawardena
OBJECTIVE: Levamisole (LEV) has been used successfully on an alternate-day regime of 2.5 mg/kg in steroid-dependant nephrotic syndrome (SDNS) to maintain remission. This pilot study was carried out between 2010 and 2015 at a single center in Sri Lanka to evaluate the efficacy of LEV prescribed at 2.5 mg/kg daily, which is double the alternate-day dose. METHODS: Sequential children with SDNS, relapsing more than twice in the preceding 12 months and previously treated with LEV and low-dose alternate-day prednisolone (0...
March 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28298181/early-onset-of-adck4-glomerulopathy-with-renal-failure-a-case-report
#7
Ksenija Lolin, Benedetta D Chiodini, Elise Hennaut, Brigitte Adams, Karin Dahan, Khalid Ismaili
BACKGROUND: We present a rare early presentation of a ADCK4-related glomerulopathy. This case is of interest as potentially treatable if genetic results are timely obtained. CASE PRESENTATION: We report the case of a 5-year-old boy who was identified with significant proteinuria by a urinary routine screening program for school children. Physical examination revealed dysplastic ears and abnormal folded pinna. Albumin level was 41 g/L (39-53 g/L), and urine proteins/creatinine ratio was 2...
March 16, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28290633/proteinuria-in-children-evaluation-and-differential-diagnosis
#8
Alexander K C Leung, Alex H C Wong, Stefani S N Barg
Although proteinuria is usually benign in the form of transient or orthostatic proteinuria, persistent proteinuria may be associated with more serious renal diseases. Proteinuria may be an independent risk factor for the progression of chronic kidney disease in children. Mechanisms of proteinuria can be categorized as glomerular, tubular, secretory, or overflow. A history, a physical examination, and laboratory tests help determine the cause. Transient (functional) proteinuria is temporary. It can occur with fever, exercise, stress, or cold exposure, and it resolves when the inciting factor is removed...
February 15, 2017: American Family Physician
https://www.readbyqxmd.com/read/28257644/a-single-center-analysis-of-henoch-schonlein-purpura-nephritis-with-nephrotic-proteinuria-in-children
#9
Dan Feng, Wen-Yan Huang, Sheng Hao, Xiao-Ling Niu, Ping Wang, Ying Wu, Guang-Hua Zhu
BACKGROUND: In children with Henoch-Schonlein purpura nephritis (HSPN), the degree of proteinuria has been proven to be not only a sign of kidney damage, but also an accelerator of kidney disease progression. Nephrotic proteinuria at disease onset has been proposed as a predictor of a poor renal outcome. This study aims to assess the clinical and pathological features of HSPN with nephrotic proteinuria in a single center. METHODS: One hundred thirty-seven patients with HSPN who visited Shanghai Children's Hospital from January 2009 to December 2013 were retrospectively reviewed...
March 4, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28257282/wt1-mutation-associated-nephropathy-a%C3%A2-single-center-experience%C3%A2
#10
Zhihui Yue, Haiyan Wang, Hongrong Lin, Juan Yang, Ting Liu, Yulin Liu, Huamu Chen, Liangzhong Sun
This study explored Wilms' tumor 1 (WT1) mutations in children with, or suspected of having, steroid-resistant nephrotic syndrome (SRNS), referred to or treated in our hospital in the past 6 years as well as the correlation between genotype and phenotype in WT1 mutation-associated nephropathy in Chinese patients. In total, 76 patients participated in the study. WT1 mutations were identified in 15 patients, 5 of whom harbored splice-site mutations in intron 9. Four of these 5 patients exhibited early onset of nephropathy and rapid deterioration of renal function...
March 3, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28245398/-clinical-significance-of-serum-midkine-in-children-with-henoch-schonlein-purpura
#11
Mo-Min Li, Ni Liu, Miao Zhagn, Hai-Rong Xue
OBJECTIVE: To investigate the clinical significance of serum midkine (MK) in children with henoch-schnlein purpura(HSP). METHODS: One hundred and six children with HSP admitted in our hospital from March 2013 to March 2016 were enrolled in HSP group, but then 80 healthy volunteers were used as control. MK, interleukin-2 (IL-2), IL-4, IL-6, IL-10, tumor necrosis factor (TNF), interferon γ (IFN-γ) and IL-17 in peripheral blood were measured by ELISA, biochemical indicators including white blood cell count (WBC), platelet (Plt), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), D-dimer, immunoglobulin A (IgA), IgE, IgG, IgM and other clinical data were recorded and analyzed...
February 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28209267/assessment-of-volume-status-and-appropriate-fluid-replenishment-in-the-setting-of-nephrotic-syndrome
#12
Pierluigi Marzuillo, Stefano Guarino, Andrea Apicella, Rosaria Marotta, Vincenzo Tipo, Laura Perrone, Angela La Manna, Giovanni Montini
BACKGROUND: When the permeability of the glomerular filtration barrier increases, leading to proteinuria, nephrotic syndrome (NS) occurs. First episodes or relapses of NS can be concurrent with acute gastroenteritis (AGE) infections. This condition can cause further deterioration of the hypovolemic state, as intravascular water is lost through both AGE-related vomiting/diarrhea and NS-related fluid shifting into the interstitium. In this case report, we wish to raise the issues about the difficult management of children presenting with both NS and AGE...
April 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28204946/clinical-outcomes-in-children-with-henoch-sch%C3%A3-nlein-purpura-nephritis-without-crescents
#13
Jean Daniel Delbet, Julien Hogan, Bilal Aoun, Iulia Stoica, Rémi Salomon, Stéphane Decramer, Isabelle Brocheriou, Georges Deschênes, Tim Ulinski
BACKGROUND: Henoch-Schönlein purpura is the most common vasculitis in children. Its long-term prognosis depends on renal involvement. The management of Henoch-Schönlein purpura nephritis (HSPN) remains controversial. This study reports the prognosis of children with HSPN presenting with class 2 International Study of Kidney Disease in Children (ISKDC) nephritis. METHODS: All children with HSPN class 2 diagnosed between 1995 and 2015 in four pediatric nephrology centers were included, and clinical and biological data were collected from the medical files...
February 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28204945/spectrum-of-mutations-in-chinese-children-with-steroid-resistant-nephrotic-syndrome
#14
Fang Wang, Yanqin Zhang, Jianhua Mao, Zihua Yu, Zhuwen Yi, Li Yu, Jun Sun, Xiuxiu Wei, Fangrui Ding, Hongwen Zhang, Huijie Xiao, Yong Yao, Weizhen Tan, Svjetlana Lovric, Jie Ding, Friedhelm Hildebrandt
BACKGROUND: The aim of this study was to elucidate whether genetic screening test results of pediatric patients with steroid-resistant nephrotic syndrome (SRNS) vary with ethnicity. METHODS: Using high-throughput DNA sequencing, 28 nephrotic syndrome-related genes were analyzed in 110 chil-dren affected by SRNS and 10 children with isolated proteinuria enrolled by 5 centers in China (67 boys, 53 girls). Their age at disease onset ranged from 1 day to 208 months (median, 48...
February 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28188437/clinical-utility-of-spot-urine-protein-to-creatinine-ratio-modified-by-estimated-daily-creatinine-excretion-in-children
#15
Eun Mi Yang, Bo Ae Yoon, Soo Wan Kim, Chan Jong Kim
BACKGROUND: The spot urine protein-to-creatinine ratio (UPCR) is widely used to predict 24-h urine protein (24-h UP) excretion. In patients with low daily urine creatinine excretion (UCr), however, the UPCR may overestimate 24-h UP. The aim of this study was to predict 24-h UP using UPCR adjusted by estimated 24-h UCr in children. METHODS: This study included 442 children whose 24-h UP and spot UPCR were measured concomitantly. Estimated 24-h UCr was calculated using three previously existing equations...
February 10, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28175986/does-zika-virus-infection-induce-prolonged-remissions-in-children-with-idiopathic-nephrotic-syndrome
#16
Carolina Peralta-Aros, Víctor García-Nieto
BACKGROUND: Zika is an emerging mosquito-borne flavivirus. We report two pediatric patients diagnosed with idiopathic nephrotic syndrome who achieved complete remission of the disease after suffering Zika virus (ZIKV) infection. CASE DIAGNOSIS/TREATMENT: The first patient was a young girl aged 2.5 years with steroid-dependent nephrotic syndrome who was subsequently diagnosed with ZIKV infection. Following the infection, the steroid dose could be reduced until complete withdrawal...
February 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28174414/a-case-of-henoch-schonlein-purpura-associated-with-rotavirus-infection-in-an-elderly-asian-male-and-review-of-the-literature
#17
REVIEW
Chen Tang, Daphne Scaramangas-Plumley, Cynthia C Nast, Zab Mosenifar, Marc A Edelstein, Michael Weisman
BACKGROUND Henoch-Schönlein purpura (HSP), a small vessel vasculitis mediated by deposition of immune-complexes containing IgA in the skin, gut, and glomeruli, often presents with abdominal pain, purpuric rash in the lower extremities and buttocks, joint pain, and hematuria. The disease most commonly targets children but can affect adults who tend to have a worse prognosis. CASE REPORT We discuss a case of HSP in an elderly Chinese male who presented with severe proximal bowel inflammation, vasculitic rash, and proteinuria; he was found to have positive stool rotavirus and giardia...
February 8, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28141565/repeated-administrations-of-rituximab-along-with-steroids-and-immunosuppressive-agents-in-refractory-steroid-resistant-nephrotic-syndrome
#18
Shuichiro Fujinaga, Koji Sakuraya
BACKGROUND: A recent randomized control trial in children with steroid-resistant nephrotic syndrome revealed that two doses of rituximab did not reduce proteinuria. CASE CHARACTERISTICS: A 14-month-old boy developed refractory steroid-resistant nephrotic syndrome due to focal segmental glomerulosclerosis. OBSERVATION: The patient achieved complete remission 11 months after disease onset following eight doses of rituximab combined with steroids and cyclosporine...
January 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28133685/impact-of-acute-kidney-injury-on-long-term-mortality-and-progression-to-chronic-kidney-disease-among-critically-ill-children
#19
Najlaa G Al-Otaibi, Maryam Zeinelabdin, Mohamed A Shalaby, Norah Khathlan, Ghadi D Mashat, Amal A Zahrani, Sundus Mw NoorSaeed, Nora M Shalabi, Khalid A Alhasan, Sara N Sharief, Amr S Albanna, Jameela A Kari
To determine the 2-year outcome of acute kidney injury (AKI) following admission to pediatric critical care units (PICU). Methods: A retrospective cohort study was conducted between January 2012 and December 2013. We followed 131 children admitted to PICU, King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia with a diagnosis of AKI, based on pRIFLE (pediatric risk, injury, failure, loss, and end-stage renal disease), for 2 years. During the study period, 46 children died and 38 of survivors completed the follow-up...
February 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28130969/the-oxford-classification-predictors-of-chronic-kidney-disease-in-pediatric-patients-with-iga-nephropathy
#20
Rafaela C G Fabiano, Stanley A Araújo, Eduardo A Bambirra, Eduardo A Oliveira, Ana Cristina Simões E Silva, Sérgio V B Pinheiro
OBJECTIVE: The Oxford Classification for Immunoglobulin A nephropathy (IgAN) identifies pathological variables that may predict the decline of renal function. This study aimed to evaluate the Oxford Classification variables as predictors of renal dysfunction in a cohort of Brazilian children and adolescents with IgAN. METHODS: A total of 54 patients with IgAN biopsied from 1982 to 2010 were assessed. Biopsies were re-evaluated and classified according to the Oxford Classification...
January 27, 2017: Jornal de Pediatria
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