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nephrotic syndrome, pediatric

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https://www.readbyqxmd.com/read/29750317/mycophenolate-mofetil-for-sustained-remission-in-nephrotic-syndrome
#1
Uwe Querfeld, Lutz T Weber
The clinical application of mycophenolate mofetil (MMF) has significantly widened beyond the prophylaxis of acute and chronic rejections in solid organ transplantation. MMF has been recognized as an excellent treatment option in many immunologic glomerulopathies. For children with frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS) experiencing steroid toxicity, MMF has been recommended as a steroid-sparing drug. Uncontrolled studies in patients with FRNS and SDSN have shown that many patients can achieve sustained remission of proteinuria with MMF monotherapy...
May 11, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29680473/rituximab-in-the-management-of-pediatric-steroid-resistant-nephrotic-syndrome-a-systematic-review
#2
Manel Jellouli, Rim Charfi, Bayen Maalej, Abdelmajid Mahfoud, Sameh Trabelsi, Tahar Gargah
OBJECTIVES: To evaluate the efficacy and safety of rituximab in children with steroid-resistant nephrotic syndrome. STUDY DESIGN: A systematic review evaluating the efficacy and safety of rituximab in children with steroid-resistant nephrotic syndrome was performed. Data from studies, performed before April 2017 were collected, from MEDLINE, Cochrane Library, Scopus, and Web of Science. Study eligibility criteria included clinical trials and observational studies with a minimal sample size of 5 patients, regarding treatment with rituximab in children with steroid-resistant nephrotic syndrome...
April 18, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29569812/ofatumumab-in-post-transplantation-recurrence-of-a-pediatric-steroid-resistant-idiopathic-nephrotic-syndrome
#3
Josselin Bernard, Alexandra Bruel, Emma Allain-Launay, Jacques Dantal, Gwenaelle Roussey
Treatment of SRNS is a challenge. Antiproliferative agents and depleting antibodies have been reported to be effective. However, these agents are not always successful, and use of ofatumumab could provide a different treatment option. Our patient was diagnosed with a SRNS at 5 years of age. She developed ESRD, with FSGS. This was cause for a first renal transplantation. The NS relapsed, leading to loss of the graft, and a second renal transplantation was performed. Due to the recurrence of the NS, IAds were initiated and led to a complete remission...
March 23, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29569185/indications-and-results-of-renal-biopsy-in-children-a-36-year-experience
#4
Luisa Santangelo, Giuseppe Stefano Netti, Paolo Giordano, Vincenza Carbone, Marida Martino, Diletta Domenica Torres, Michele Rossini, Anna Maria Di Palma, Loreto Gesualdo, Mario Giordano
BACKGROUND: This study was conducted to investigate retrospectively the indications for renal biopsy (RB) in native kidneys and to analyze pathological findings in a single tertiary pediatric hospital in Southern Italy for the last 36 years. METHODS: All patients who underwent RB at our hospital from 1979 to 2014 were included. All renal tissue specimens were studied under light and immunofluorescent microscopy, while electron microscopy was performed only for specific clinical indications...
March 22, 2018: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/29569147/hypertension-and-its-severity-in-children-with-steroid-sensitive-nephrotic-syndrome-during-remission
#5
Swasti Keshri, Shobha Sharma, Neha Agrawal, Sandeep Bansal, B P Guilliani, Kailash Chandra Aggrawal
BACKGROUND AND OBJECTIVE: Hypertension is not a typical feature of steroid sensitive nephrotic syndrome (SSNS) and the presence of persistent hypertension is suggestive of significant renal lesion. There is paucity of data regarding occurrence and severity of hypertension in SSNS in pediatric population during remission and was the main objective of this study. In addition, correlation with factors like family history, BMI, and lipid profile was studied. METHODS: Cross-sectional study conducted at tertiary care center in India including 81 children of infrequent relapsing SSNS between 1 and 10 years in remission and was off steroids...
March 22, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29565146/stunting-wasting-and-mid-upper-arm-circumference-status-among-children-admitted-to-nemazee-teaching-hospital
#6
Seyed Mohsen Dehghani, Hazhir Javaherizadeh, Masoomeh Heidary, Naser Honar, Maryam Ataollahi, Homa Ilkanipour, Hossein Moravej
INTRODUCTION AND AIM: The aim of this study was to evaluate nutritional status in children without prior hospital admission or evidence of chronic disease. SUBJECTS AND METHODS: The current study is a cross-sectional and observational study which was conducted for assessing the nutritional status of children. In this study, consecutive sampling was used, with a sample size about 400 children aged 6 months to 18 years at first hospital admission. All subjects were hospitalized consecutively in the Pediatric Emergency Department of the Nemazee Teaching Hospital of Shiraz University of Medical Sciences (Shiraz, Islamic Republic of Iran)...
January 10, 2018: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/29552446/the-ratio-of-urinary-%C3%AE-1-microglobulin-to-microalbumin-can-be-used-as-a-diagnostic-criterion-for-tubuloproteinuria
#7
Hongwen Zhang, Fang Wang, Huijie Xiao, Yong Yao
Low-molecular-weight proteinuria is one of the characteristic clinical manifestations of renal tubular and interstitial diseases. Low-molecular-weight proteinuria is defined as excessive urinary loss of α1-microglobulin, β2-microglobulin, or other low-molecular-weight plasma proteins. The current study examined the ratio of urinary α1-microglobulin to microalbumin in 24 Chinese pediatric patients with renal tubular and interstitial diseases, including 10 patients with Dent disease, 2 patients with Lowe syndrome, 6 patients with acute tubulointerstitial nephritis (ATIN), 4 patients with acute tubulointerstitial nephritis with uveitis syndrome (TINU), and 2 patients with nephronophthisis (NPHP)...
February 2018: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/29534211/genetic-variants-in-the-lama5-gene-in-pediatric-nephrotic-syndrome
#8
Daniela A Braun, Jillian K Warejko, Shazia Ashraf, Weizhen Tan, Ankana Daga, Ronen Schneider, Tobias Hermle, Tilman Jobst-Schwan, Eugen Widmeier, Amar J Majmundar, Makiko Nakayama, David Schapiro, Jia Rao, Johanna Magdalena Schmidt, Charlotte A Hoogstraten, Hannah Hugo, Sevcan A Bakkaloglu, Jameela A Kari, Sherif El Desoky, Ghaleb Daouk, Shrikant Mane, Richard P Lifton, Shirlee Shril, Friedhelm Hildebrandt
Background: Nephrotic syndrome (NS), a chronic kidney disease, is characterized by significant loss of protein in the urine causing hypoalbuminemia and edema. In general, ∼15% of childhood-onset cases do not respond to steroid therapy and are classified as steroid-resistant NS (SRNS). In ∼30% of cases with SRNS, a causative mutation can be detected in one of 44 monogenic SRNS genes. The gene LAMA5 encodes laminin-α5, an essential component of the glomerular basement membrane. Mice with a hypomorphic mutation in the orthologous gene Lama5 develop proteinuria and hematuria...
March 9, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29533950/gut-microbiota-dysbiosis-in-children-with-relapsing-idiopathic-nephrotic-syndrome
#9
Shoji Tsuji, Chikushi Suruda, Masaki Hashiyada, Takahisa Kimata, Sohsaku Yamanouchi, Tetsuya Kitao, Jiro Kino, Atsushi Akane, Kazunari Kaneko
BACKGROUND: While the etiology of idiopathic nephrotic syndrome (idiopathic nephrotic syndrome [INS]; characterized by repeated relapses and comorbid allergic conditions) remains unknown, recent evidence suggests that dysfunction in regulatory T cells (Tregs) plays an important role in the development of INS as well as allergic diseases. We hypothesized that dysbiosis involving decreased butyric acid-producing gut microbiota leads to defective induction and differentiation of peripherally induced Tregs, resulting in INS relapse...
2018: American Journal of Nephrology
https://www.readbyqxmd.com/read/29532233/nephrotic-syndrome-associated-with-severe-hypertriglyceridemia-in-a-pediatric-patient-answers
#10
Beatriz Corredor-Andrés, María Teresa Muñoz-Calvo, Olga Calero, Cristina Aparicio, Jesús Argente, Miguel Calero
No abstract text is available yet for this article.
March 12, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29532231/nephrotic-syndrome-associated-with-severe-hypertriglyceridemia-in-a-pediatric-patient-questions
#11
Beatriz Corredor-Andrés, María Teresa Muñoz-Calvo, Olga Calero, Cristina Aparicio, Jesús Argente, Miguel Calero
No abstract text is available yet for this article.
March 12, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29515298/a-study-of-clinical-presentation-and-correlative-histopathological-patterns-in-renal-parenchymal-disease
#12
K Ganesh, R R Nair, N V Seethalekshmy, G Kurian, A Mathew, S Sreedharan, Z Paul
Suspicion and subsequent detection of renal disease is by an assessment of the urinalysis and renal function in the clinical context. Our attempt in this study is to correlate initial presenting features of urinalysis and renal function to the final histopathological diagnosis. A retrospective analysis of 1059 native kidney biopsies performed from January 2002 to June 2015 at Amrita Institute of Medical Sciences was conducted. Correlative patterns between urinalysis, renal function, and final histopathological diagnosis were studied...
January 2018: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/29507273/urinary-cd80-as-a-replacement-for-renal-biopsy-for-diagnosis-of-pediatric-minimal-change-disease
#13
Heba Mostafa Ahmed, Dina Ahmed Ezzat, Noha A Doudar, Mai Adel
INTRODUCTION: Early diagnosis of minimal change disease (MCD) is challenging in nephrotic children. CD80 is a protein expressed on the surface of podocytes associated with nephrotic syndrome and it is implicated in the induction of proteinuria. This study aimed to investigate the use of urinary CD80 for the diagnosis of MCD. MATERIALS AND METHODS: Urinary CD80 levels were evaluated in 36 children with nephrotic syndrome and normal glomerular filtration rate. They were divided into three groups of MCD (n = 21), focal segmental glomerulosclerosis (n = 9), and other glomerulopathies (n = 6)...
March 2018: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/29467993/pediatric-renal-transplantation-in-oman-a-single-center-experience
#14
Mohamed S Al Riyami, Sulaiman Al Saidi, Badria Al Ghaithi, Anisa Al Maskari, Sadiq Lala, Nabil Mohsin, Lekha Hirshikesan, Naifain Al Kalbani
Objectives: This study sought to report 22 years experience in pediatric kidney transplantation in Oman. Methods: Electronic charts of all Omani children below 13 years of age who received a kidney transplant from January 1994 to December 2015 were reviewed. Data collected included patient demographics, etiology of end-stage kidney disease, modality and duration of dialysis, donor type, complication of kidney transplantation (including surgical complications, infections, graft rejection) graft and patient survival, and duration of follow-up...
January 2018: Oman Medical Journal
https://www.readbyqxmd.com/read/29460503/urinary-l-fabp-level-in-children-with-nephrotic-syndrome-and-tubular-dysfunction
#15
Masashi Nishida, Hidekazu Kawakatsu, Kenji Hamaoka
BACKGROUND: Recent studies indicated the role of urinary liver-type fatty acid-binding protein (uL-FABP) as a biomarker for kidney injury. However, uL-FABP excretion in patients with relapsing nephrotic syndrome and tubular dysfunction in the pediatric population had not been reported previously. METHODS: We examined uL-FABP level in children with steroid-sensitive nephrotic syndrome (SSNS), in those with tubular dysfunction, and in control subjects. RESULTS: uL-FABP was markedly increased in patients with relapsing SSNS (12...
February 20, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29457021/difficult-renal-pathological-classification-in-a-case-of-pediatric-nephrotic-syndrome
#16
Hiroshi Yamaguchi, Atsutoshi Shiratori, Taku Nakagawa, Kyoko Kanda, Shigeo Hara, Norishige Yoshikawa, Ryojiro Tanaka
The underlying histopathology is very important in determining patient management, as the histopathology usually has direct repercussions on the treatment response and clinical course. However, the impact of the method used to assess renal biopsies, i.e., light microscopy (LM), immunofluorescence (IF), and electron microscopy (EM), on the occurrence of a difficult biopsy classification in the native kidneys of pediatric nephrotic patients is unknown. A 12-month-old Japanese boy was diagnosed with nephrotic syndrome (NS); he was administered prednisolone (60 mg/m2 /day), and a continuous albumin infusion was started...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29429831/predictors-of-poor-outcome-in-children-with-steroid-sensitive-nephrotic-syndrome
#17
Kirtisudha Mishra, Sandeep Kumar Kanwal, Sushma Veeranna Sajjan, Vikram Bhaskar, Bimbadhar Rath
BACKGROUND AND OBJECTIVE: Nearly 50% of the children with steroid sensitive nephrotic syndrome (SSNS) have a frequently relapsing (FR) or steroid dependent (SD) course, experiencing steroid toxicities and complications of immunosuppression. The study aimed to compare parameters between children with infrequent relapsing (IFR) and FR/SD nephrotic syndrome and to identify the factors associated with a FR/SD course. METHODS: A retrospective analysis of medical records from 2009 to 2014, of children with SSNS attending the pediatric nephrology clinic in a tertiary care medical college and hospital...
February 8, 2018: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/29429752/patterns-of-clinical-response-to-eculizumab-in-patients-with-c3-glomerulopathy
#18
Moglie Le Quintrec, Anne-Laure Lapeyraque, Arnaud Lionet, Anne-Laure Sellier-Leclerc, Yahsou Delmas, Véronique Baudouin, Eric Daugas, Stéphane Decramer, Leila Tricot, Mathilde Cailliez, Philippe Dubot, Aude Servais, Catherine Mourey-Epron, Franck Pourcine, Chantal Loirat, Véronique Frémeaux-Bacchi, Fadi Fakhouri
BACKGROUND: Cases reports and small series of patients with C3 glomerulopathy have reported variable efficacy of eculizumab. STUDY DESIGN: Case series of C3 glomerulopathy. SETTING & PARTICIPANTS: Pediatric and adult patients with C3 glomerulopathy treated with eculizumab between 2010 and 2016 were identified through the C3 glomerulopathy French registry database, and a questionnaire was sent to participating French pediatric and adult nephrology centers, as well as one pediatric referral center in Québec, Canada...
February 8, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29421774/efficacy-and-safety-of-rituximab-in-children-with-steroid-and-cyclosporine-resistant-and-steroid-and-cyclosporine-dependent-nephrotic-syndrome
#19
Rozita Hoseini, Kamran Sabzian, Hasan Otukesh, Nazanin Zafaranloo, Parsa Panahi, Nahid Rahimzadeh, Shahrbanoo Nakhaie, Mohsen Akhavan Sepehi
INTRODUCTION: There is evidence of the effectiveness of rituximab in treatment of nephrotic syndrome in children. The present study aimed to assess safety and the therapeutic effectiveness of rituximab in steroid- and cyclosporine-resistant pediatric nephrotic syndrome. MATERIALS AND METHODS: Forty-three children with steroid- and cyclosporine-resistant or steroid- and cyclosporine-dependent noncongenital nephrotic syndrome were included in the study to receive intravenous rituximab, 375 mg/m2/wk, for 4 weeks...
January 2018: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/29402263/effect-of-the-2015-earthquake-on-pediatric-inpatient-pattern-at-a-tertiary-care-hospital-in-nepal
#20
Bishnu Rath Giri, Ram Hari Chapagain, Samana Sharma, Sandeep Shrestha, Sunita Ghimire, P Ravi Shankar
BACKGROUND: Earthquakes impact child health in many ways. Diseases occurring immediately following an earthquake have been studied in field based hospitals but studies on the inpatient disease pattern among children without trauma in a permanent hospital setup is lacking. METHODS: We examined the diagnoses of all children without trauma, admitted to Kanti Children's Hospital, Kathmandu for fifteen-week duration (from 4th week to end of the 18th week) following the 7...
February 5, 2018: BMC Pediatrics
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