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proteinuria in children

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https://www.readbyqxmd.com/read/28515415/evaluation-of-mycophenolate-mofetil-and-low-dose-steroid-combined-therapy-in-moderately-severe-henoch-sch%C3%A3-nlein-purpura-nephritis
#1
Zhihong Lu, Junfeng Song, Jianhua Mao, Yonghui Xia, Caiyun Wang
BACKGROUND The most appropriate management of Henoch-Schönlein Purpura (HSP) nephritis with nephrotic-range proteinuria remains uncertain. The aim of this study was to evaluate the clinical therapeutic effects of mycophenolate mofetil and low-dose steroid in Henoch-Schönlein purpura nephritis (HSPN) with nephrotic-range proteinuria and pathological classification less than IV in children. MATERIAL AND METHODS The clinical effects of MMF and low-dose steroid therapy were studied in children with Henoch-Schönlein purpura nephritis manifested with nephrotic-range proteinuria, normal kidney function, and <50% crescents or sclerosing lesions on renal biopsy...
May 18, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28499374/clinical-improvement-of-renal-amyloidosis-in-a-patient-with-systemic-onset-juvenile-idiopathic-arthritis-who-received-tocilizumab-treatment-a-case-report-and-literature-review
#2
Songkiat Chantarogh, Soamarat Vilaiyuk, Thipwimol Tim-Aroon, Suchin Worawichawong
BACKGROUND: Juvenile idiopathic arthritis (JIA) is a common rheumatic disease in children and adolescents. Although JIA may cause secondary amyloidosis, this is a rare complication in patients with JIA and other rheumatic diseases. Many previous studies have revealed that common heterozygous or homozygous mutations in the MEFV gene are associated with systemic-onset JIA (SJIA). CASE PRESENTATION: We herein report a case involving a 19-year-old female patient with difficult-to-control SJIA...
May 12, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28496353/early-manifestations-of-renal-disease-in-patients-with-tuberous-sclerosis-complex
#3
Laura Malaga-Dieguez, Robert Spencer, Laura J Pehrson, Suzanne Vento, Kimberly Menzer, Orrin Devinsky, Howard Trachtman
OBJECTIVES: Renal manifestations are the second most significant cause of morbidity and mortality in patients with tuberous sclerosis complex (TSC), and include renal cysts, angiomyolipomas, fat-poor lesions, and malignant tumors. These lesions begin in childhood and often lead to chronic kidney disease (CKD). Little is known on the incidence of early modifiable risk factors of CKD, such as proteinuria and hypertension, or subtle decreases in glomerular filtration rate that correspond to the early stages of CKD in children with TSC...
2017: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/28477260/atypical-thymic-carcinoid-manifesting-with-nephrotic-range-proteinuria-in-a-7-year-old-boy
#4
Jolanta Soltysiak, Danuta Ostalska-Nowicka, Katarzyna Zaorska, Karolina Sterzyńska, Katarzyna Derwich, Jacek Zachwieja
BACKGROUND: Nephrotic-range proteinuria as a paraneoplastic syndrome (PNS) is an exceptional presentation, especially in children. It is usually associated with hematologic malignancies. Solid tumors are very rare causes of proteinuria. CASE-DIAGNOSIS/TREATMENT: We present the case of a 7-year-old boy with an extremely rare atypical thymic carcinoid accompanied by nephrotic-range proteinuria as PNS. The kidney biopsy was consistent with minimal change disease (MCD)...
May 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28463080/lupus-glomerulonephritis-in-788-chinese-children-a-multi-centre-clinical-and-histopathological-analysis-based-on-549-renal-biopsies
#5
Si-Yan Jin, Dan-Lin Huang, Xi-Qiang Dang, Zhu-Wen Yi
BACKGROUND: System lupus erythematosus (SLE) is a severe multisystem autoimmune disease. OBJECTIVE: To describe the clinical and pathological features, treatment, and renal outcome in children under 18 years with lupus nephritis (LN). METHODS: The study was undertaken by a questionnaire completed in 26 Grade 3A hospitals' paediatric renal units in China. The study comprised 788 children (619 girls, 169 boys) diagnosed with SLE by the American College of Rheumatology criteria (1997) during 2005-2010...
May 2, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28449720/evolution-of-blood-pressure-in-children-with-congenital-and-acquired-solitary-functioning-kidney
#6
Riccardo Lubrano, Isotta Gentile, Raffaele Falsaperla, Giovanna Vitaliti, Alessia Marcellino, Marco Elli
BACKGROUND: It is not yet clear if blood pressure and renal function changes evolve differently in children with a congenital or acquired solitary functioning kidney. This study aims to assess if there are any differences between these two types of solitary kidney patients. METHODS: Current research is a retrospective study assessing the evolution of glomerular filtration rate, proteinuria, and blood pressure in clinical records of 55 children with a solitary functioning kidney (37 congenital and 18 acquired)...
April 27, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28441404/angiopoietin-like-4-and-minimal-change-disease
#7
Gabriel Cara-Fuentes, Alfons Segarra, Cecilia Silva-Sanchez, Heiman Wang, Miguel A Lanaspa, Richard J Johnson, Eduardo H Garin
BACKGROUND: Minimal Change Disease (MCD) is the most common type of nephrotic syndrome in children. Angiopoietin-like-4 (Angplt4) has been proposed as mediator of proteinuria in MCD. The aim of this study was to evaluate the role of Angptl4 as a biomarker in MCD. METHODS: Patients with biopsy-proven primary MCD, focal segmental glomerulosclerosis, membranous nephropathy (60, 52 and 52 respectively) and 18 control subjects had urinary and serum Angptl4 measured by Elisa...
2017: PloS One
https://www.readbyqxmd.com/read/28436233/cyclosporine-based-immunosuppressive-therapy-for-patients-with-steroid-resistant-focal-segmental-glomerulosclerosis-a-meta-analysis
#8
Yuan-Yow Chiou, Yi-Che Lee, Mei-Ju Chen
OBJECTIVE: Focal segmental glomerulosclerosis (FSGS) is a leading cause of end-stage kidney disease that requires immunosuppressive treatment as therapy. Few studies have been specifically designed to assess the efficacy of cyclosporine (CSA) in patients with steroid-resistant FSGS. This study investigated the efficacy of CSA-based therapy in steroid-resistant FSGS. METHODS: Medline, Cochrane, EMBASE, and Google Scholar databases were searched through April 30, 2014 using the keywords "cyclosporine," "steroid-resistant," "focal segmental glomerulosclerosis," and "FSGS...
April 22, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28407818/-clinical-and-pathological-features-and-the-misdiagnosis-of-childhood-alport-syndrome-a-retrospective-analysis-of-91-cases
#9
Yan-Zhen Chen, Liang-Zhong Sun, Hai-Yan Wang, Xiao-Yun Jiang, Ying Mo, Zhi-Hui Yue, Hua-Mu Chen, Ting Liu, Hong-Rong Lin
OBJECTIVE: To explore the clinical and pathological features and the diagnosis of childhood Alport syndrome (AS). METHODS: A retrospective analysis was performed on clinical data of 91 children with AS. RESULTS: Hematuria was observed in all 91 patients, of whom 86 were accompanied with proteinuria. Sixty-one children with X-Linked AS (XL-AS) had positive family history. Renal biopsy was performed on 82 children. Mild to moderate mesangial proliferation was observed in 74 cases...
April 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28406083/ambulatory-arterial-stiffness-index-blood-pressure-variability-and-nocturnal-blood-pressure-dip-in-children-with-iga-and-henoch-sch%C3%A3-nlein-nephropathy%C3%A2
#10
Piotr Skrzypczyk, Małgorzata Mizerska-Wasiak, Barbara Jerszow, Paweł Ruszczykowski, Małgorzata Pańczyk-Tomaszewska
AIM: Evaluation of mean blood pressure values, ambulatory arterial stiffness index (AASI), pulse pressure (PP), blood pressure variability (BPV), and circadian blood pressure rhythm using ambulatory blood pressure monitoring (ABPM) in children with IgA nephropathy and Henoch-Schönlein nephropathy (IgAN/HSN). MATERIAL AND METHODS: In 48 children (29 with IgAN, 19 with HSN) aged 14.04 ± 3.76 years, we evaluated retrospectively 24-hour systolic and diastolic BP (24hSBP, 24hDBP), AASI, PP, systolic and diastolic BPV, and nocturnal BP dip by ABPM, body mass index (BMI) Z-score, medications, and biochemical parameters...
April 13, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28405891/renalase-in-children-with-glomerular-kidney-diseases
#11
Piotr Skrzypczyk, Joanna Przychodzień, Małgorzata Mizerska-Wasiak, Elżbieta Kuźma-Mroczkowska, Magdalena Okarska-Napierała, Elżbieta Górska, Anna Stelmaszczyk-Emmel, Urszula Demkow, Małgorzata Pańczyk-Tomaszewska
Studies suggest that renalase, a renal catecholamine-inactivating enzyme, plays a major role in the pathogenesis of kidney and cardiovascular diseases in adults. This study seeks to determine the role of renalase in children with glomerular kidney diseases. We evaluated the serum renalase, arterial stiffness, intima-media thickness, blood pressure, and clinical and biochemical parameters in 78 children (11.9 ± 4.6 years of age) with glomerulopathies such as idiopathic nephrotic syndrome (40 cases), IgA nephropathy (12 cases), Henoch-Schönlein nephropathy (12 cases), and other glomerulopathies (14 cases)...
April 13, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28405841/focal-segmental-glomerulosclerosis-and-medullary-nephrocalcinosis-in-children-with-adck4-mutations
#12
Eujin Park, Hee Gyung Kang, Young Hun Choi, Kyoung Bun Lee, Kyung Chul Moon, Hyeon Joo Jeong, Michio Nagata, Hae Il Cheong
BACKGROUND: Mutations in the AarF domain containing kinase 4 gene (ADCK4), one of the novel genes causing steroid-resistant nephrotic syndrome (SRNS), usually manifest as isolated adolescent-onset focal segmental glomerulosclerosis (FSGS). ADCK4 interacts with components of the coenzyme Q10 (CoQ10) biosynthesis pathway. METHODS: The incidence and phenotypes of patients with ADCK4 mutations were investigated in a cohort of Korean pediatric patients with SRNS. RESULTS: Among the 53 patients enrolled in the study the incidence of ADCK4-associated FSGS was 7...
April 12, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28391337/managing-glomerular-disease-in-pregnancy
#13
Michelle A Hladunewich, Kate Bramham, Belinda Jim, Sharon Maynard
Glomerular disease, especially lupus nephritis, is common in young women of childbearing age. As such, practicing nephrologists must have adequate knowledge and expertise to prepare these women for pregnancy, to diagnose and manage the numerous pregnancy-associated complications that are common in this vulnerable patient population and finally to support these young women who often struggle to manage both their young children along with an often onerous chronic disease. In this article we will review the pre-pregnancy counseling these women should ideally receive to allow them to make an informed decision about proceeding with a pregnancy, an approach to the diagnosis of pregnancy-associated worsening of kidney function and proteinuria as well as pregnancy-safe management strategies, including immunosuppression, antihypertensive agents and other medications necessary for the management of nephrotic syndrome...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28385484/genetic-mutation-in-egyptian-children-with-steroid-resistant-nephrotic-syndrome
#14
Manal Micheal Thomas, Mohamed S Abdel-Hamid, Nermine Nabil Mahfouz, Emad Emil Ghobrial
BACKGROUND/PURPOSE: Nephrotic syndrome is the commonest etiology of proteinuria in children. Steroid-resistant nephrotic syndrome (SRNS) is defined by resistance to standard steroid therapy, and it continues to be one of the most intractable etiologies of renal failure. Molecular studies discovered specialized molecules in podocytes that play a role in proteinuria. Mutations in NPHS2 that encodes for podocin constitute a frequent cause of SRNS worldwide. This study aimed to screen for podocin mutations in SRNS Egyptian children and their parents...
April 3, 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28339709/clinicopathologic-correlations-of-renal-pathology-in-the-adult-population-of-poland
#15
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...
April 1, 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
#16
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...
April 3, 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
#17
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...
May 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
#18
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
#19
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
#20
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
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