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nephritic syndrome pediatric

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
Y Han, S Q DU, H J Xiao, Y Zhou, J Ding, J J Ding, Y M Cui
OBJECTIVE: Tacrolimus prolonged-release(PR) formulation is a new once-daily formulation of the calcineurin inhibitor tacrolimus, which is currently used in adult liver or kidney transplant patients,and is also gradually widely used in children with nephrotic syndrome.The present study was undertaken to preliminarily investigate the pharmacokinetic characteristics of tacrolimus PR in pediatric nephrotic syndrome recipients. METHODS: This single-center open-label prospective study was performed in pediatric nephrotic syndrome recipients...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
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...
December 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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
M Mizerska-Wasiak, A Turczyn, A Such, K Cichoń-Kawa, J Małdyk, M Miklaszewska, J Pietrzyk, A Rybi-Szumińska, A Wasilewska, A Firszt-Adamczyk, R Stankiewicz, M Szczepańska, B Bieniaś, M Zajączkowska, A Pukajło-Marczyk, D Zwolińska, K Siniewicz-Luzeńczyk, M Tkaczyk, K Gadomska-Prokop, R Grenda, U Demkow, M Pańczyk-Tomaszewska
IgA nephropathy (IgAN) is the most common form of glomerulonephritis in pediatric population. The clinical presentation of the disease in children ranges from microscopic hematuria to end-stage kidney disease. The aim of the study was to retrospectively assess clinical and kidney biopsy features in children with IgAN. We assessed a cohort of 140 children, 88 boys, 52 girls with the diagnosis of IgAN in the period of 2000-2015, entered into the national Polish pediatric IgAN registry. The assessment included the following: proteinuria, hematuria, glomerular filtration rate (GFR), arterial blood pressure, and the renal pathological changes according to the Oxford classification and crescents formation, as modifiable and unmodifiable risk factors...
2016: Advances in Experimental Medicine and Biology
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
S Imtiaz, K Nasir, M F Drohlia, B Salman, A Ahmad
Kidney biopsy occupies a fundamental position in the management of kidney diseases. There are very few renal pathology studies available in the literature from developing world. This study scrutinized the frequency and clinicopathological relationship of kidney biopsies done at the kidney center from 1997 to 2013 amongst pediatric patients. Kidney allograft biopsy were excluded. The specimen was examined under light microscopy and immunofluorescence while electron microscopy was not done. The study includes 423 patients, mean age was 10...
May 2016: Indian Journal of Nephrology
Ronny Lehmann, Benjamin Hanebeck, Stephan Oberle, Anke Simon, Daniela Choukair, Burkhard Tönshoff, Sören Huwendiek
AIM: Virtual patients (VPs) are a one-of-a-kind e-learning resource, fostering clinical reasoning skills through clinical case examples. The combination with face-to-face teaching is important for their successful integration, which is referred to as "blended learning". So far little is known about the use of VPs in the field of continuing medical education and residency training. The pilot study presented here inquired the application of VPs in the framework of a pediatric residency revision course...
2015: GMS Zeitschrift Für Medizinische Ausbildung
Kamal V Kanodia, Aruna V Vanikar, Lovelesh K Nigam, Rashmi D Patel, Kamlesh S Suthar, Dinesh N Gera, Hargovind L Trivedi
BACKGROUND: Renal biopsy is a well-established diagnostic modality for the assessment of kidney diseases in children. It can provide diagnostic precision and prognostic value and guide in therapeutic options for many renal diseases. OBJECTIVES: This report describes the indication, histopathological patterns, and epidemiology of renal diseases in children in India. PATIENTS AND METHODS: This is a single-center study on renal biopsies performed between January 2008 and December 2013 in 346 children (age ≤ 14 years)...
July 2015: Nephro-urology Monthly
Michiel J S Oosterveld, Mark R Garrelfs, Bernd Hoppe, Sandrine Florquin, Joris J T H Roelofs, L P van den Heuvel, Kerstin Amann, Jean-Claude Davin, Antonia H M Bouts, Pietrik J Schriemer, Jaap W Groothoff
BACKGROUND AND OBJECTIVES: Dense deposit disease (DDD), a subtype of C3 glomerulopathy, is a rare disease affecting mostly children. Treatment options are limited. Debate exists whether eculizumab, a monoclonal antibody against complement factor C5, is effective in DDD. Reported data are scarce, especially in children. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: The authors analyzed clinical and histologic data of five pediatric patients with a native kidney biopsy diagnosis of DDD...
October 7, 2015: Clinical Journal of the American Society of Nephrology: CJASN
Mengjie Jiang, Xiaoyun Jiang, Liping Rong, Yuanyuan Xu, Lizhi Chen, Zeting Qiu, Ying Mo
Immunoglobulin A nephropathy (IgAN) is an immunopathologic diagnosis based on a renal biopsy, it is characterized by deposits of IgA-containing immune complexes in the mesangium. Adults with IgAN have a galactose-deficient IgA1 in the circulation and glomerular deposition. There are few studies on the glycosylation of serum IgA1 in children with IgAN. To measure the serum levels of galactose-deficient IgA1 in pediatric patients with IgAN, 72 biopsy-proven IgAN children were divided into 3 groups based on the clinical features: isolated hematuria group (24 patients), hematuria and proteinuria group (22 patients), and nephritic syndrome group (26 patients)...
2015: International Journal of Clinical and Experimental Medicine
Hiroaki Kanai, Emi Sawanobori, Keiichi Koizumi, Ryuji Ohashi, Kosuke Higashida
Post-streptococcal glomerulonephritis (PSGN) generally has a good renal prognosis, and immunosuppressive therapies are not needed. However, a few patients present with severe acute kidney injury and extensive crescent formations. The etiology of such patients is not well known, and involvement of anti-neutrophil cytoplasmic antibodies is rarely reported. A 9-year-old girl with rapidly progressive nephritic syndrome was diagnosed with PSGN. A biopsy showed diffuse crescentic glomerulonephritis with immunoglobulin G and C3 deposits; moreover, humps were observed on electron microscopy...
April 2015: Clinical Nephrology
(no author information available yet)
OBJECTIVE: Nephrotic syndrome (NS) was recognized as a clinical syndrome caused by massive proteinuria, leading to hypoalbuminemia and edema. Primary nephrotic syndrome, accounted for about 90% of NS, seems to be one of common urologic-kidney diseases in childhood. The diagnosis and treatment of NS had so far failed to be unified nationwide. The purpose of this research was to survey the diagnosis and treatment status of steroid-sensitive, relaping/steroid-dependent nephrotic syndrome (SSNS, RNS/SDNS) in Chinese children and to evaluate the applicability and promotion of the evidence-based guideline on diagnosis and treatment of SSNS, RNS/SDNS (for trial implementation) conducted by The Subspecialty Group of Nephrology, the Society of Pediatrics, Chinese Medical Association in 2009...
March 2014: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Mariana Luna, Victoria Bocanegra, Patricia G Vallés
Granulomatosis with polyangiitis (GPA) is associated with a broad range of clinical manifestations including renal disease. It is a systemic vasculitis that is rarely encountered in children. We present a 14-year-old girl who suffered from pharyngitis 1 week before admittance to hospital. She was admitted for macroscopic hematuria and oliguria, under the possibility of nephritic syndrome. Renal failure with rapidly progressive glomerulonephritis occurred within 24 hours. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies (c-ANCA with antiproteinase 3 specificity) and renal biopsy revealed pauci-immune crescentic focal necrotizing glomerulonephritis...
2014: International Journal of Nephrology and Renovascular Disease
O Naija, J Bouzaraa, R Goucha-Louzir, T Gargah
AIM: To describe epidemiological, clinical, histological aspects, treatment and outcome of Henoch-Schönlein nephrits in children. METHODS: A retrospective study was conducted on medical data of 34 patients with Henoch-Schönlein nephritis From January 1, 1996 to December 31, 2010 in the Pediatric Department of Charles Nicolle Hospital. RESULTS: Nephritis occured in 68,7%. The average patient age was 7 years 2 months and sex ratio was 0,6...
December 2013: La Tunisie Médicale
Katarzyna Michaud, Silke Grabherr, Kebede Shiferaw, Franceso Doenz, Marc Augsburger, Patrice Mangin
Cocaine is a well known trigger of acute coronary syndromes. Over the last 10 years levamisole, a veterinary anthelminthic drug has been increasingly used as an adulterant of cocaine. Levamisole was used to treat pediatric nephritic syndrome and rheumatoid arthritis before being withdrawn from the market due to its significant toxicity, i.e. hematological complications and vasculitis. The major complications of levamisole-adultered cocaine reported up to now are hematological and dermatological. The case reported here is of a 25 year old man with a history of cocaine abuse who died at home after complaining of retrosternal pain...
January 2014: Journal of Forensic and Legal Medicine
Xiao-Ling Yin, Min-Shu Zou, Yu Zhang, Jia Wang, Tong-Lin Liu, Jin-Hui Tang, Li-Ru Qiu, Yu Chen, Hui-Qing Yuan, Jian-Hua Zhou
BACKGROUND: The aim of this study was to investigate the clinical and pathological characteristics as well as their associations with trends for diseases in 1,579 pediatric renal biopsies from 1989 to 2012. METHODS: The clinical and pathological data were retrospectively analyzed for children undergoing renal biopsy from 1989 to 2012 in our hospital. RESULTS: Primary glomerulonephritis (PGN) accounted for 60.1% of total cases, followed by secondary glomerulonephritis (SGN) (31...
July 2013: Journal of Nephrology
Hüseyin Per, Mehmet Canpolat, Hakan Gümüş, Hatice Gamze Poyrazoğlu, Ali Yıkılmaz, Sarper Karaküçük, Hakkı Doğan, Sefer Kumandaş
OBJECTIVE: Pseudotumor cerebri (PTC) is a clinical condition characterized by signs and symptoms of increased intracranial pressure, such as headache and papilledema. Our aim was to investigate the etiological and clinical features of pseudotumor cerebri (PTC) in children. MATERIALS AND METHOD: We performed a comprehensive analysis of epidemiology, diagnostic work-up, therapy, and clinical follow-up in 42 consecutive patients. RESULTS: Totally 42 patients diagnosed with PTC [27 (64...
June 2013: Brain & Development
Layla Saleh Abdullah
To study the clinical indications and histopathological pattern of renal biopsies and renal resection specimens in the pediatric age group (birth to 17 years) in a large academic center in western Saudi Arabia. A retrospective review of the computerized database of the Pathology Department at King Abdul Aziz University Hospital (KAUH) was carried out on the final pathology reports of all children of the age of 17 years and below who either had ultrasound-guided renal biopsies or partial or total renal resections during the period between January 1995 and December 2008...
March 2012: Saudi Journal of Kidney Diseases and Transplantation
Mirije Begolli, Ilir Begolli, Xhenane Gojani, Fatime Arenliu-Qosaj, Merita Berisha
The aim of this case is to present a case of a two month old female with congenital nephritic syndrome, which is very rare. On admission, the baby showed marked edema and distended abdomen. She was diagnosed and treated with daily albumin infusions, antibiotics, diuretic, gamma globulin replacement, ACEI and NSAIDs. Parents were informed about the nature of the disease, prognosis, and advised for further medical care in a more advanced kidney transplantation centre. This was the first treatment of this condition in the Pediatric Clinic in Kosovo and it presented a challenge for us...
2011: Medicinski Arhiv
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