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pediatric nephrotic syndrome

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https://www.readbyqxmd.com/read/27900972/failure-to-thrive-and-nephrocalcinosis-due-to-distal-renal-tubular-acidosis-a-rare-presentation-of-pediatric-lupus-nephritis
#1
Madhumita Nandi, Mrinal Kanti Das, Sukanta Nandi
A 9-year-old female child was initially diagnosed of having nephrocalcinosis with distal renal tubular acidosis (dRTA) while investigating for short stature. She later on developed features of nephrotic syndrome (NS) while on treatment for RTA. Investigation for the cause of NS revealed very strong serological evidence in favor of systemic lupus erythematosus (SLE). Histopathological confirmation could not be done due to bilateral severely contracted kidneys. There are a few case reports of dRTA as the presentation of SLE, but nephrocalcinosis with dRTA with subsequent manifestation of SLE has hitherto not been reported in literature...
November 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27821253/bone-mineral-density-in-children-with-idiopathic-nephrotic-syndrome
#2
Ghada Mohamed El-Mashad, Mahmoud Ahmed El-Hawy, Sally Mohamed El-Hefnawy, Sanaa Mansour Mohamed
OBJECTIVES: To assess bone mineral density (BMD) in children with idiopathic nephrotic syndrome (NS) and normal glomerular filtration rate (GFR). METHODS: Cross-sectional case-control study carried out on 50 children: 25 cases of NS (16 steroid-sensitive [SSNS] and nine steroid-resistant [SRNS] under follow up in the pediatric nephrology unit of Menoufia University Hospital, which is tertiary care center, were compared to 25 healthy controls with matched age and sex...
November 5, 2016: Jornal de Pediatria
https://www.readbyqxmd.com/read/27801486/focal-segmental-glomerulosclerosis-in-children
#3
Manel Jellouli, Kamel Abidi, Mouna Askri, Meriem Ferjani, Ouns Naija, Yousra Hammi, Rym Goucha, Tahar Gargah
Background Focal segmental glomerulosclerosis (FSGS) represents 20% of nephrotic syndrome in children. The clinical course and prognosis is heterogeneous in children. The aim of this study was to analyze treatment and outcome of children with FSGS. Methods This retrospective study was conducted in the Department of Pediatrics in Charles Nicolle Hospital during a 15-year period (1996-2010). Results There were 30 children, 16 boys and 14 girls. The mean age was 7 ± 4 years. Nephrotic syndrome was observed in 26 patients, hematuria was noticed in 2 patients and renal insufficiency was detected in 2 patients at presentation...
May 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/27795625/a-prospective-audit-of-complications-in-100-consecutive-pediatric-percutaneous-renal-biopsies-done-under-real-time-ultrasound-guidance
#4
R Sinha, B Maji, B Sarkar, S Meur
Despite being a common procedure, percutaneous renal biopsy (PRB) carries the potential for complications. The British Association of Paediatric Nephrologist (BAPN) has published standards for pediatric PRB. As Indian data are scarce, we conducted a prospective audit of 100 consecutive pediatric renal biopsies (60% males) under real-time ultrasound guidance. Nephrotic syndrome was the most common indication for PRB (68%) with minimal change disease (30%) and focal segmental glomerulosclerosis (25%) being the most common histopathological lesions...
September 2016: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/27783158/steroid-resistant-nephrotic-syndrome-a-persistent-challenge-for-pediatric-nephrology
#5
Samriti Dogra, Frederick Kaskel
Steroid-resistant nephrotic syndrome remains a challenge to treat, but various efforts are underway to better understand the pathogenesis and improve patient outcomes. This review provides an update on the newer advances in understanding the molecular etiologies for a variety of podocyte abnormalities, potential circulating factors that may initiate and sustain the steroid-resistant state, genetic mutations, and precision medicine treatment modalities in this continuously perplexing disorder.
October 26, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27761562/diverse-etiology-of-hyperlipidemia-among-hospitalized-children-in-western-region-of-saudi-arabia
#6
Abdulmoein E Al-Agha, Abrar M Alnawab, Tala M Hejazi
To determine the various etiologies of primary and secondary hyperlipidemia among children visiting the pediatric endocrine clinic. Methods: This is a retrospective, cross-sectional, cohort study conducted at King Abdulaziz University Hospital (KAUH), Jeddah, Kingdom of Saudi Arabia from January 2010 to 2015 that included 253 children aged from birth to 12 years old. Data were obtained by reviewing medical reports of patients who presented with hyperlipidemia to the clinic, and their laboratory investigation results using KAUH electronic "Phoenix" system...
November 2016: Saudi Medical Journal
https://www.readbyqxmd.com/read/27714465/iga-nephropathy-with-presentation-of-nephrotic-syndrome-at-onset-in-children
#7
Yuko Shima, Koichi Nakanishi, Masashi Sato, Taketsugu Hama, Hironobu Mukaiyama, Hiroko Togawa, Ryojiro Tanaka, Kandai Nozu, Mayumi Sako, Kazumoto Iijima, Hiroyuki Suzuki, Norishige Yoshikawa
BACKGROUND: Despite a low incidence, nephrotic syndrome (NS) can present with IgA nephropathy (IgAN). The clinical characteristics and long-term outcomes of pediatric patients with IgAN presenting with NS (NS-IgAN) at onset have not been fully elucidated. METHODS: We retrospectively analyzed 426 patients, and compared clinical and pathological (Oxford) findings between those with NS-IgAN and those with non-NS-IgAN. RESULTS: Among 426 patients, 30 (7...
October 6, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27687621/ofatumumab-in-two-pediatric-nephrotic-syndrome-patients-allergic-to-rituximab
#8
Marina Vivarelli, Manuela Colucci, Alice Bonanni, Martina Verzani, Jessica Serafinelli, Francesco Emma, Gianmarco Ghiggeri
BACKGROUND: Rituximab, a chimeric anti-CD20 monoclonal antibody, is an effective treatment in steroid-dependent nephrotic syndrome (SDNS). However, some patients develop adverse reactions. CASE-DIAGNOSIS/TREATMENT: Patient 1, a 14-year-old boy with SDNS since the age of 2, was treated with oral prednisone, cyclosporine A (CsA) and mycophenolate mofetil. A first infusion of rituximab at age 12 years was well tolerated, but this was followed by a prolonged relapse unresponsive to oral prednisone, mycophenolate mofetil and CsA...
September 29, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27617138/the-genetics-of-nephrotic-syndrome
#9
REVIEW
Michelle N Rheault, Rasheed A Gbadegesin
Nephrotic syndrome (NS) is a common pediatric kidney disease and is defined as massive proteinuria, hypoalbuminemia, and edema. Dysfunction of the glomerular filtration barrier, which is made up of endothelial cells, glomerular basement membrane, and visceral epithelial cells known as podocytes, is evident in children with NS. While most children have steroid-responsive nephrotic syndrome (SSNS), approximately 20% have steroid-resistant nephrotic syndrome (SRNS) and are at risk for progressive kidney dysfunction...
March 2016: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/27590892/high-incidence-of-idiopathic-nephrotic-syndrome-in-east-asian-children-a-nationwide-survey-in-japan-jp-shine-study
#10
Kaori Kikunaga, Kenji Ishikura, Chikako Terano, Mai Sato, Fumiyo Komaki, Yuko Hamasaki, Satoshi Sasaki, Kazumoto Iijima, Norishige Yoshikawa, Koichi Nakanishi, Hitoshi Nakazato, Takeshi Matsuyama, Takashi Ando, Shuichi Ito, Masataka Honda
BACKGROUND: Little is known regarding the epidemiology of idiopathic nephrotic syndrome (INS) in East Asia. Previous studies have suggested higher incidence of INS in Asian children, though decreasing trend of its incidence has also been shown. METHODS: We conducted a nationwide study of Japanese children aged 6 months to 15 years with INS. Children who were newly diagnosed with INS between 1 January 2010 and 31 December 2012 were eligible. Children with congenital nephrotic syndrome or nephrotic syndrome secondary to nephritis were excluded...
September 2, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27575507/mycophenolate-mofetil-in-treatment-of-childhood-steroid-dependent-nephrotic-syndrome
#11
Manel Jellouli, Sonia Fitouhi, Kamel Abidi, Yousra Hammi, Ouns Naija, Chokri Zarrouk, Tahar Gargah
OBJECTIVES: To establish the efficacy of mycophenolate mofetil (MMF) in steroid dependent nephrotic syndrome and to determine the predictive factors for a good response. METHODS: retrospective hospital-based cohort study in the department of pediatric of Charles Nicolle hospital, between 2005 and 2012 included 30 children with steroid-dependent nephritic syndrome who were treated with MMF. RESULTS: A total of 30 patients (20 boys and 10 girls) were included...
March 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/27566421/progress-in-pediatrics-in-2015-choices-in-allergy-endocrinology-gastroenterology-genetics-haematology-infectious-diseases-neonatology-nephrology-neurology-nutrition-oncology-and-pulmonology
#12
EDITORIAL
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Virginia Mirra, Sergio Bernasconi
This review focuses key advances in different pediatric fields that were published in Italian Journal of Pediatrics and in international journals in 2015. Weaning studies continue to show promise for preventing food allergy. New diagnostic tools are available for identifying the allergic origin of allergic-like symptoms. Advances have been reported in obesity, short stature and autoimmune endocrine disorders. New molecules are offered to reduce weight gain and insulin-resistance in obese children. Regional investigations may provide suggestions for preventing short stature...
August 27, 2016: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/27539155/pediatric-thromboembolism-a-national-survey-in-japan
#13
Akira Ishiguro, Chibueze Chioma Ezinne, Nobuaki Michihata, Hisaya Nakadate, Atsushi Manabe, Masashi Taki, Midori Shima
Thromboembolism is being detected at increasing rates in pediatric tertiary care hospitals. The incidence of pediatric thrombophilia differs across countries, and is unknown in Japan. We sent a survey to 520 pediatric department heads and 629 specialists, requesting details on patients who developed symptomatic thromboembolism between 2006 and 2010. Of 280 eligible cases, congenital thrombophilia and other conditions were reported. Congenital thrombophilia (n = 54, 19.3 %) comprised defects in protein C (27), protein S (9), and antithrombin (7)...
August 18, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27528553/the-relatively-poor-correlation-between-random-and%C3%A2-24-hour-urine-protein-excretion-in-patients-with%C3%A2-biopsy-proven-glomerular-diseases
#14
Marie C Hogan, Heather N Reich, Peter J Nelson, Sharon G Adler, Daniel C Cattran, Gerald B Appel, Debbie S Gipson, Matthias Kretzler, Jonathan P Troost, John C Lieske
Random urine protein creatinine ratios are used to estimate 24-hour urine protein excretion, which is considered a diagnostic gold standard. However, few studies are available of the sensitivity and specificity of this estimation in patients with glomerular proteinuria. To clarify this, we measured the urine protein and creatinine centrally in random and 24-hour urine collections at biopsy and longitudinally every 6 months in individuals participating in the Nephrotic Syndrome Study Network (NEPTUNE) cohort with glomerular disease...
November 2016: Kidney International
https://www.readbyqxmd.com/read/27468526/post-infectious-glomerulonephritis-in-pediatric-patients-over-two-decades-severity-associated-features
#15
Rona Dagan, Roxana Cleper, Miriam Davidovits, Levana Sinai-Trieman, Irit Krause
BACKGROUND: The incidence of post-infectious glomerulonephritis (PIGN) has decreased over the last decades. As a result, recent epidemiological data from industrialized countries are scarce. OBJECTIVES: To evaluate patterns of PIGN in children and detect possible predictors of disease severity. METHODS: We collected clinical and laboratory data of patients with PIGN admitted to Schneider Children's Medical Center during 1994-2011. Diagnostic criteria included presence of hematuria with/without other features of nephritic syndrome along with hypocomplementemia and/or microbiological/serological evidence of streptococcal infection...
June 2016: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/27445165/ethnic-differences-in-incidence-and-outcomes-of-childhood-nephrotic-syndrome
#16
Tonny H M Banh, Neesha Hussain-Shamsy, Viral Patel, Jovanka Vasilevska-Ristovska, Karlota Borges, Cathryn Sibbald, Deborah Lipszyc, Josefina Brooke, Denis Geary, Valerie Langlois, Michele Reddon, Rachel Pearl, Leo Levin, Monica Piekut, Christoph P B Licht, Seetha Radhakrishnan, Kimberly Aitken-Menezes, Elizabeth Harvey, Diane Hebert, Tino D Piscione, Rulan S Parekh
BACKGROUND AND OBJECTIVES: Ethnic differences in outcomes among children with nephrotic syndrome are unknown. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We conducted a longitudinal study at a single regional pediatric center comparing ethnic differences in incidence from 2001 to 2011 census data and longitudinal outcomes, including relapse rates, time to first relapse, frequently relapsing disease, and use of cyclophosphamide. Among 711 children, 24% were European, 33% were South Asian, 10% were East/Southeast Asian, and 33% were of other origins...
July 21, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27442391/steroid-resistant-nephrotic-syndrome-genetic-consideration
#17
REVIEW
Velibor Tasic, Zoran Gucev, Momir Polenakovic
Nephrotic syndrome is defined as the association of massive proteinuria, hypoalbuminaemia, edema, and hyperlipidemia. It is separated to steroid-sensitive or steroid-resistant (SRNS) forms in respect to the response to intensive steroid therapy. SRNS usually progresses to end-stage renal failure. According to the North American Pediatric Renal Trials and Collaborative Studies SRNS constitutes the second most frequent cause of ESRD in the first two decades of life. Unfortunately, there is no curative treatment for majority of patients...
2015: Prilozi (Makedonska Akademija Na Naukite i Umetnostite. Oddelenie za Medicinski Nauki)
https://www.readbyqxmd.com/read/27422620/rituximab-for-nephrotic-syndrome-in-children
#18
Kazumoto Iijima, Mayumi Sako, Kandai Nozu
Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. At least 20 % of children with this syndrome show frequent relapses and/or steroid dependence during or after immunosuppressive therapies, a condition defined as complicated frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS). Approximately 1-3 % of children with idiopathic nephrotic syndrome are resistant to steroids and all immunosuppressive agents, a condition defined as refractory steroid-resistant nephrotic syndrome (SRNS); these SRNS children have a high risk of end-stage renal failure...
July 15, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27377607/sxr-rs3842689-a-prognostic-factor-for-steroid-sensitivity-or-resistance-in-pediatric-idiopathic-nephrotic-syndrome
#19
Stefano Turolo, Alberto Edefonti, Marta Lepore, Luciana Ghio, Eva Cuzzoni, Giulia De Corti, Andrea Pasini, Marco Materassi, Cristina Malaventura, Fabrizio Pugliese, Giovanni Montini
AIM: of the study was to analyse the impact of SXR rs3842689 polymorphism on the response to corticosteroids in pediatric idiopathic nephrotic syndrome. PATIENTS & METHODS: 66 children (56 steroid-sensitive, ten steroid-resistant) were studied for SXR gene polymorphism distribution. RESULTS: Steroid sensitive patients accounted for 96% of cases with In/In polymorphism, but only for 53% of cases with Del/Del polymorphism At odds ratio analysis, Del/Del represented a clear risk factor of steroid resistance (OR: 20...
July 5, 2016: Pharmacogenomics
https://www.readbyqxmd.com/read/27375752/neurological-manifestations-of-renal-diseases-in-children-in-qazvin-iran
#20
Reza Dalirani, Abolfazl Mahyar, Parviz Ayazi, Ghazaleh Ahmadi
OBJECTIVE: Renal diseases are one of the most common causes of referrals and admissions of children, hence it is important to know their neurological presentations. This study aimed to determine neurological presentations of renal diseases in children. MATERIAL & METHODS: A total of 634 children with renal diseases, admitted to Qazvin Pediatric Hospital, Qazvin, central Iran from 2011 to 2013 were studied. Neurological presentations of patients were established and the results were analyzed using statistical tests...
2016: Iranian Journal of Child Neurology
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