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pediatric proteinuria

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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/29729114/evaluation-of-clinical-and-laboratory-findings-in-pediatric-post-streptococcal-glomerulonephritis
#2
Beltinge Demircioglu Kılıc, Mehtap Akbalık Kara, Mithat Buyukcelik, Ayse Balat
BACKGROUND: Acute post-streptococcal glomerulonephritis (APSGN) is the most common postinfectious glomerulonephritis in childhood. In this study, we aimed to determine the possible risk factor(s), responsible for decreased glomerular filtration rate (GFR) in APSGN. METHODS: The data of patients followed up with the diagnosis of APSGN in the Pediatric Nephrology Clinic of Gaziantep University Hospital between October 2014 and October 2016 were retrospectively evaluated...
May 5, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29693796/a-phase-1-study-of-cabozantinib-in-children-and-adolescents-with-recurrent-or-refractory-solid-tumors-including-cns-tumors-trial-advl1211-a-report-from-the-children-s-oncology-group
#3
Meredith K Chuk, Brigitte C Widemann, Charles G Minard, Xiaowei Liu, AeRang Kim, Melanie Brooke Bernhardt, Rachel A Kudgus, Joel M Reid, Stephan D Voss, Susan Blaney, Elizabeth Fox, Brenda J Weigel
BACKGROUND: We conducted a phase 1 trial to determine the maximum tolerated dose (MTD), toxicity profile, pharmacokinetics (PK), pharmacodynamics (PD), and preliminary activity of cabozantinib in children with refractory or relapsed solid tumors. METHODS: Patients received cabozantinib tablets on a continuous dosing schedule in a rolling-six escalating phase 1 trial design. PK and PD studies were performed. RESULTS: Forty-one patients, median (range) age 13 (4-18) years, received cabozantinib to achieve a weekly cumulative dose equivalent to 30 (n = 6), 40 (n = 23)...
April 25, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29671499/poststreptococcal-illness-recognition-and-management
#4
David L Maness, Michael Martin, Gregg Mitchell
Group A beta-hemolytic streptococcus can cause several postinfectious, nonsuppurative immune- mediated diseases including acute rheumatic fever, poststreptococcal reactive arthritis, pediatric autoimmune neuropsychiatric disorders, and poststreptococcal glomerulonephritis. Except for sporadic outbreaks, poststreptococcal autoimmune syndromes occur most commonly in sub-Saharan Africa, India, Australia, and New Zealand. Children younger than three years are rarely affected by group A streptococcus pharyngitis or rheumatic fever, and usually do not require testing...
April 15, 2018: American Family Physician
https://www.readbyqxmd.com/read/29625558/predominant-but-silent-c1q-deposits-in-mesangium-on-transplanted-kidneys-long-term-observational-study
#5
Takahiro Kanai, Yuko Akioka, Kenichiro Miura, Masataka Hisano, Junki Koike, Yutaka Yamaguchi, Motoshi Hattori
BACKGROUND: C1q nephropathy (C1qN) was first described as glomerular disease characterized by predominant meangial C1q deposits in patients with proteinuria and no evidence of systemic lupus erythematosus. Several studies, however, revealed the clinical heterogeneity of C1qN, showing some cases with normal urinalysis. To confirm the existence of cases with predominant mesangial C1q deposits and negative or mild proteinuria and/or hematuria, we investigated renal graft biopsy specimens showing negative to mild proteinuria (less than or equal to 1+ by dip stick test) and/or hematuria...
April 6, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29569812/ofatumumab-in-post-transplantation-recurrence-of-a-pediatric-steroid-resistant-idiopathic-nephrotic-syndrome
#6
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
#7
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
#8
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/29552446/the-ratio-of-urinary-%C3%AE-1-microglobulin-to-microalbumin-can-be-used-as-a-diagnostic-criterion-for-tubuloproteinuria
#9
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/29546930/-acute-renal-damage-secondary-to-acute-tubulointerstitial-nephritis-drug-use-case-report
#10
Niki Oikonomopoulou, Ana Belén Martínez López, Javier Urbano Villaescusa, María Del Carmen Molina Molina, Laura Butragueño Laiseca, Daniel Barraca Nuñez, Olalla Álvarez Blanco
INTRODUCTION: Acute tubulointerstitial nephritis (ATIN) is a rare entity in the pediatric age. It is de fined by the infiltration of the renal parenchyma by mononuclear and/or polynuclear cells with se condary involvement of the tubules, without glomerular injury. It can be triggered by infections or immunological diseases, drugs like NSAIDs or be of idiopathic origin. OBJECTIVE: To raise awareness among pediatricians about the prescription of NSAIDs, especially to patients of less than a year old, since they can provoke renal damage...
December 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/29534211/genetic-variants-in-the-lama5-gene-in-pediatric-nephrotic-syndrome
#11
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/29533929/atypical-hemolytic-uremic-syndrome-in-children-aged-2-years
#12
Nilgun Çakar, Z Birsin Ozcakar, Fatih Ozaltin, Mustafa Koyun, Banu Celikel Acar, Elif Bahat, Bora Gulhan, Emine Korkmaz, Ayşe Yurt, Songül Yılmaz, Oğuz Soylemezoglu, Fatoş Yalcinkaya
BACKGROUND: There are limited data on infants with atypical hemolytic uremic syndrome (aHUS). The aim of this study was to determine the clinical and laboratory features, and to evaluate treatment modalities and outcomes in infants with aHUS. MATERIALS AND METHODS: Relevant data on patients with onset of aHUS at age <2 years were obtained from the Turkish Pediatric aHUS Registry. RESULTS: Among the 146 patients included in the Registry, 53 (36%) (23 male and 30 female) were enrolled for the study...
March 13, 2018: Nephron
https://www.readbyqxmd.com/read/29523959/the-light-at-the-end-of-the-tunnel-an-unusual-case-of-acute-kidney-injury-in-a-pediatric-patient-answers
#13
Miriam Steinberg, Joseph P Gaut, Stanley Paul Hmiel, Aadil Kakajiwala
Monoclonal gammopathies are a rare diagnosis in pediatric patients. A 19-year-old female patient with past medical history of hypogammaglobulinemia and natural killer cell deficiency and stage III follicular lymphoma, in remission, presented with a right-sided pneumonia, noted to have acute kidney injury and proteinuria. Complement C3 and C4 levels were normal. Anti-double-stranded DNA antibodies, antinuclear antibodies, anti-extractable nuclear antigen antibodies, and antineutrophil cytoplasmic antibodies were negative...
March 9, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29520517/initial-treatment-with-pulse-methylprednisolone-followed-by-short-term-prednisolone-and-tonsillectomy-for-childhood-iga-nephropathy
#14
Akifumi Yamada, Shuichiro Fujinaga, Koji Sakuraya, Asanuma Satoshi, Daishi Hirano
BACKGROUND: Although a combination therapy, comprising 2-year high-dose oral prednisolone (PSL), is effective for treating childhood immunoglobulin A nephropathy (IgAN), severe adverse effects and residual proteinuria occur in some patients after the therapy. METHODS: To clarify the efficacy of intravenous pulse methylprednisolone (IVMP; 15-20 mg/kg; maximum 600 mg/day; for 3 consecutive days/week for 3 weeks) followed by short-term reduced-dose PSL (initially 1 mg/kg; maximum 30 mg on alternate days; tapered off within approximately 12 months) and tonsillectomy as an initial treatment, we retrospectively reviewed the clinical courses of 54 consecutive children with IgAN (32 boys; mean age at onset, 12...
March 8, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29515298/a-study-of-clinical-presentation-and-correlative-histopathological-patterns-in-renal-parenchymal-disease
#15
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
#16
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/29457021/difficult-renal-pathological-classification-in-a-case-of-pediatric-nephrotic-syndrome
#17
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/29452889/anthropometric-and-biochemical-determinants-of-estimated-glomerular-filtration-rate-in-a-large-cohort-of-obese-children
#18
Pierluigi Marzuillo, Anna Grandone, Anna Di Sessa, Stefano Guarino, Mario Diplomatico, Giuseppina Rosaria Umano, Cesare Polito, Angela La Manna, Laura Perrone, Emanuele Miraglia Del Giudice
OBJECTIVE: We aimed to investigate which clinical and metabolic factors could influence the estimated glomerular filtration rate (eGFR) levels, evaluating a large population of obese children without suspect of primary kidney disease. DESIGN: Retrospective, cross-sectional study. SETTING: Pediatric university department. SUBJECTS: We enrolled 2,957 obese children and adolescents consecutively attending our department between January 2000 and 2017...
February 13, 2018: Journal of Renal Nutrition
https://www.readbyqxmd.com/read/29429752/patterns-of-clinical-response-to-eculizumab-in-patients-with-c3-glomerulopathy
#19
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
#20
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
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