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Nephrotic syndrome management in children

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https://www.readbyqxmd.com/read/28914167/nephrotic-syndrome-in-infants-and-children-pathophysiology-and-management
#1
Mallory L Downie, Claire Gallibois, Rulan S Parekh, Damien G Noone
Nephrotic syndrome is defined by nephrotic-range proteinuria (≥40 mg/m(2)/hour or urine protein/creatinine ratio ≥200 mg/mL or 3+ protein on urine dipstick), hypoalbuminaemia (<25 g/L) and oedema. This review focuses on the classification, epidemiology, pathophysiology, management strategies and prognosis of idiopathic nephrotic syndrome of childhood, and includes a brief overview of the congenital forms.
September 15, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28798906/cerebral-sinovenous-thrombosis
#2
REVIEW
Rebecca Ichord
Cerebral sinovenous thrombosis (CSVT) is a rare but serious cerebrovascular disorder affecting children from the newborn period through childhood and adolescence. The incidence is estimated at 0.6/100,000/year, with 30-50% occurring in newborns. Causes are diverse and are highly age dependent. Acute systemic illness is the dominant risk factor among newborns. In childhood CSVT, acute infections of the head and neck such as mastoiditis are most common, followed by chronic underlying diseases such as nephrotic syndrome, cancer, and inflammatory bowel disease...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28751734/glucocorticoid-therapy-regulates-podocyte-motility-by-inhibition-of-rac1
#3
James C McCaffrey, Nicholas J Webb, Toryn M Poolman, Maryline Fresquet, Cressida Moxey, Leo A H Zeef, Ian J Donaldson, David W Ray, Rachel Lennon
Nephrotic syndrome (NS) occurs when the glomerular filtration barrier becomes excessively permeable leading to massive proteinuria. In childhood NS, immune system dysregulation has been implicated and increasing evidence points to the central role of podocytes in the pathogenesis. Children with NS are typically treated with an empiric course of glucocorticoid (Gc) therapy; a class of steroids that are activating ligands for the glucocorticoid receptor (GR) transcription factor. Although Gc-therapy has been the cornerstone of NS management for decades, the mechanism of action, and target cell, remain poorly understood...
July 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28751054/the-role-of-bioelectrical-impedance-analysis-nt-probnp-and-inferior-vena-cava-sonography-in-the-assessment-of-body-fluid-volume-in-children-with-nephrotic-syndrome
#4
Hulya Nalcacioglu, Ozan Ozkaya, Kemal Baysal, Hassan Candas Kafali, Bahattin Avci, Demet Tekcan, Gurkan Genc
BACKGROUND: Assessment of volume status and differentiating "underfill" and "overfill" edema is essential in the management of patients with nephrotic syndrome (NS). OBJECTIVES: Our aim was to evaluate the volume status of NS patients by using different methods and to investigate the utility of bioelectrical impedance analysis (BIA) in children with NS. METHODS: The hydration status of 19 patients with NS (before treatment of NS and at remission) and 25 healthy controls was assessed by multifrequency BIA, serum N-terminal-pro-brain natriuretic peptide (NT-proBNP) levels, inferior vena cava (IVC) diameter, left atrium diameter (LAD) and vasoactive hormones...
July 24, 2017: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/28717938/rituximab-in-steroid-sensitive-nephrotic-syndrome-lessons-from-clinical-trials
#5
REVIEW
Kazumoto Iijima, Mayumi Sako, Koichi Kamei, Kandai Nozu
Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. A total of 80-90% of patients with childhood idiopathic nephrotic syndrome achieve remission with steroid therapy [steroid-sensitive nephrotic syndrome (SSNS)]. However, approximately 50% of children with SSNS develop frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS). Children with FRNS or SDNS are usually treated with immunosuppressive agents, but 10-20% of children receiving immunosuppressive agents still show frequent relapses or steroid dependence during or after treatment, defined as complicated FRNS or SDNS...
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28717937/clinical-presentation-and-outcomes-of-childhood-onset-membranous-lupus-nephritis
#6
Maria Pereira, Eyal Muscal, Karen Eldin, M John Hicks, Anna Carmela P Sagcal-Gironella, Marietta DeGuzman, Scott E Wenderfer
BACKGROUND: Best practices for managing childhood-onset membranous lupus nephritis (MLN) are not yet established. Most studies involve primarily or exclusively adult cohorts or pediatric cohorts with combinations of pure or mixed membranous and proliferative nephritis. METHODS: We performed a single-center cohort study of consecutively diagnosed children with pure MLN from 1990 and 2016. Patients received care in Houston, Texas, one of the most diverse metropolitan areas in North America...
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28646993/improving-the-evidence-for-the-management-of-childhood-nephrotic-syndrome
#7
Brendan D Crawford, Debbie S Gipson
Management of idiopathic nephrotic syndrome in children is based on a series of clinical trials. The trial by Sinha and colleagues in this issue is 1 of many needed to improve the evidence base for induction and maintenance therapies in this population. While key questions remain about identifying the appropriate therapy for each patient, clinical trials provide an opportunity to extend evidence-based practice that minimizes toxicity and optimizes patient health.
July 2017: Kidney International
https://www.readbyqxmd.com/read/28547746/the-support-needs-of-parents-having-a-child-with-a-chronic-kidney-disease-a-focus-group-study
#8
W W Geense, B G I van Gaal, J L Knoll, E A M Cornelissen, T van Achterberg
BACKGROUND: Parents of children with a chronic kidney disease (CKD) have a crucial role in the management of their child's disease. The burden on parents is high: they are often exhausted, depressed and experience high levels of stress and a low quality of life, which could have a negative impact on their child's health outcomes. Support aiming at preventing and reducing parental stress is essential. Therefore, it is necessary to have insight in the problems and support needs among these parents...
May 25, 2017: Child: Care, Health and Development
https://www.readbyqxmd.com/read/28480100/the-burden-and-outcome-of-pediatric-renal-admissions-at-the-federal-teaching-hospital-abakaliki-a-3-year-review-2011-2013
#9
V U Muoneke, A F Una, C B Eke, O U Anyanwu
BACKGROUND: Renal diseases are important causes of morbidity and mortality in children worldwide particularly in the resource-poor countries of sub-Saharan Africa. Adequate data on these diseases in children in our setting are limited as a result of late/nonpresentation. AIM: The aim of the study is to review the pattern and outcome of pediatric renal admissions at the Federal Teaching Hospital (FETH) Abakaliki over a 3-year period. SUBJECTS AND METHODS: This was a retrospective observational review of all childhood renal admissions in FETH, Abakaliki, Ebonyi state between 2011 and 2013...
July 2016: Annals of Medical and Health Sciences Research
https://www.readbyqxmd.com/read/28474581/presentations-and-management-of-different-causes-of-chylothorax-in-children-one-medical-center-s-experience
#10
Chien-Heng Lin, Wei-Ching Lin, Jeng-Sheng Chang
BACKGROUND: Chylothorax in children is a relatively rare cause of pleural effusion. However, it is usually a common complication of cardiothoracic operations like open-heart surgery. Other etiologies for chylothorax, such as trauma or malignancy, occur more common in adults and rare in children. To explore the etiologies of chylothorax in children, this study analyzed the pediatric patients that were admitted in to onea medical center. METHODS: We retrospectively reviewed the medical records of the pediatric patients that were admitted to this tertiary transfer center with a diagnosis of chylothorax during the period of 1995 to 2005...
March 2017: BioMedicine
https://www.readbyqxmd.com/read/28466615/angiographic-embolization-in-pediatric-abdominal-trauma
#11
Yechiel Sweed, Jonathan Singer-Jordan, Sorin Papura, Norman Loberant, Alon Yulevich
BACKGROUND: Trauma is the leading cause of childhood morbidity and mortality. Abdominal bleeding is one of the common causes of mortality due to trauma. Angiography and embolization are well recognized as the primary treatments in certain cases of acute traumatic hemorrhage in adults; however, evidence is lacking in the pediatric population. OBJECTIVES: To assess the safety and efficacy of transcatheter arterial embolization (TAE) for blunt and penetrating abdominal and pelvic trauma in the pediatric age group...
November 2016: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28427453/the-italian-society-for-pediatric-nephrology-sinepe-consensus-document-on-the-management-of-nephrotic-syndrome-in-children-part-i-diagnosis-and-treatment-of-the-first-episode-and-the-first-relapse
#12
REVIEW
Andrea Pasini, Elisa Benetti, Giovanni Conti, Luciana Ghio, Marta Lepore, Laura Massella, Daniela Molino, Licia Peruzzi, Francesco Emma, Carmelo Fede, Antonella Trivelli, Silvio Maringhini, Marco Materassi, Giovanni Messina, Giovanni Montini, Luisa Murer, Carmine Pecoraro, Marco Pennesi
This consensus document is aimed at providing an updated, multidisciplinary overview on the diagnosis and treatment of pediatric nephrotic syndrome (NS) at first presentation. It is the first consensus document of its kind to be produced by all the pediatric nephrology centres in Italy, in line with what is already present in other countries such as France, Germany and the USA. It is based on the current knowledge surrounding the symptomatic and steroid treatment of NS, with a view to providing the basis for a separate consensus document on the treatment of relapses...
April 21, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28391342/learning-to-live-with-nephrotic-syndrome-experiences-of-adult-patients-and-parents-of-children-with-nephrotic-syndrome
#13
Heather Beanlands, Maria Maione, Caroline Poulton, Emily Herreshoff, Michelle A Hladunewich, Marilyn Hailperin, Mary Margaret Modes, Lawrence An, Julie Wright Nunes, Howard Trachtman, Patrick Nachman, Debbie S Gipson
Background: People living with nephrotic syndrome (NS) need to develop an in-depth understanding of their condition in order to participate in treatment decisions, develop self-management skills and integrate illness into daily life. However, the learning needs of adult patients and parents of children with NS are unknown. We therefore explored patient and parent perspectives on learning needs related to NS as part of a larger study to develop a shared learning tool for NS. Methods: Qualitative data were collected using semistructured focus groups and individual interviews with adult patients (n = 22) and parents of children with NS (n = 25)...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28391337/managing-glomerular-disease-in-pregnancy
#14
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
#15
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/28335748/steroid-resistant-nephrotic-syndrome-as-the-initial-presentation-of-nail-patella-syndrome-a-case-of-a-de-novo-lmx1b-mutation
#16
Tomohiro Nakata, Ryo Ishida, Yuu Mihara, Atsuko Fujii, Yoshimoto Inoue, Tetsuro Kusaba, Tsuyoshi Isojima, Yutaka Harita, Chiaki Kanda, Sachiko Kitanaka, Keiichi Tamagaki
BACKGROUND: Nail-patella syndrome (NPS) is an autosomal dominant disorder caused by mutations in the LMX1B gene and is characterized by nail dysplasia, skeletal abnormalities, and nephropathy. We herein report a case of steroid-resistant nephrotic syndrome (SRNS) prior to overt orthopedic symptoms in a patient with NPS. CASE PRESENTATION: A 24-year-old woman presented to our hospital with knee pain. She had poorly developed nails, hypoplastic patellas, dislocation of the elbows, and iliac horns in the pelvis...
March 23, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28270642/kidney-disease-profile-of-syrian-refugee-children
#17
Mehtap Akbalik Kara, Beltinge Demircioglu Kilic, Nilgun Col, Ayse Aysima Ozcelik, Mithat Buyukcelik, Ayse Balat
INTRODUCTION: Although preventative nephrology is the effective management of childhood kidney diseases, it is hard to provide it in this undesirable conditions. In this study, we aimed to document the kidney disease profile of Syrian refugee children admitted to our hospital. MATERIALS AND METHODS: One hundred and thirty Syrian refugee children were admitted to the Pediatric Nephrology Department of the University of Gaziantep from September 2012 to January 2015...
March 2017: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/28254584/outcomes-and-risk-factors-for-graft-loss-lessons-learned-from-1-056-pediatric-kidney-transplants-at-the-university-of-minnesota
#18
Srinath Chinnakotla, Priya Verghese, Blanche Chavers, Michelle N Rheault, Varvara Kirchner, Ty Dunn, Clifford Kashtan, Thomas Nevins, Michael Mauer, Timothy Pruett
BACKGROUND: Advances in immunosuppression, surgical techniques, and management of infections in children receiving kidney transplants have affected outcomes. STUDY DESIGN: We analyzed a prospectively maintained database of pediatric kidney transplantations. RESULTS: From June 1963 through October 2016, we performed 1,056 pediatric kidney transplantations. Of these, 129 were in children less than 2 years old. The most common indications for transplant were congenital anomalies (dysplastic kidneys), obstructive uropathy, and congenital nephrotic syndrome...
April 2017: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/28213606/parental-health-literacy-and-outcomes-of-childhood-nephrotic-syndrome
#19
Karlota Borges, Cathryn Sibbald, Neesha Hussain-Shamsy, Jovanka Vasilevska-Ristovska, Tonny Banh, Viral Patel, Josefina Brooke, Monica Piekut, Michele Reddon, Kimberly Aitken-Menezes, Ashley McNaughton, Rachel J Pearl, Valerie Langlois, Seetha Radhakrishnan, Christoph P B Licht, Tino D Piscione, Leo Levin, Damien Noone, Diane Hebert, Rulan S Parekh
OBJECTIVE: Determine the association of parental health literacy with treatment response among children with nephrotic syndrome. METHODS: This was a cohort study of children aged 1-18 with nephrotic syndrome and their parent. Health literacy was measured using the validated Short Test of Functional Health Literacy in Adults assessing reading comprehension and numeracy. Outcomes included initial relapse-free period, frequently relapsing disease, relapse rate, second-line medication use, and complete remission after therapy...
March 2017: Pediatrics
https://www.readbyqxmd.com/read/28209267/assessment-of-volume-status-and-appropriate-fluid-replenishment-in-the-setting-of-nephrotic-syndrome
#20
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
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