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Indication for biopsy in nephrotic syndrome

V Muthu, R Ramachandran, R Nada, V Kumar, M Rathi, H S Kohli, V Jha, K L Gupta, V Sakhuja
The spectrum of biopsy-proven glomerular disease was studied from a single center in Northwestern India, among adolescents aged 13-19 years. From January 2009 to December 2012, a total of 177 patients with biopsy-proven glomerular disease were studied. The same pathologist reported all the biopsy specimens after subjecting to light, immunofluorescence, and electron microscopy. The clinical profile and laboratory findings of the patients were correlated with the histopathological spectrum of glomerular diseases...
January 2018: Indian Journal of Nephrology
Jarcy Zee, Jeffrey B Hodgin, Laura H Mariani, Joseph P Gaut, Matthew B Palmer, Serena M Bagnasco, Avi Z Rosenberg, Stephen M Hewitt, Lawrence B Holzman, Brenda W Gillespie, Laura Barisoni
CONTEXT: - Testing reproducibility is critical for the development of methodologies for morphologic assessment. Our previous study using the descriptor-based Nephrotic Syndrome Study Network Digital Pathology Scoring System (NDPSS) on glomerular images revealed variable reproducibility. OBJECTIVE: - To test reproducibility and feasibility of alternative scoring strategies for digital morphologic assessment of glomeruli and explore use of alternative agreement statistics...
February 19, 2018: Archives of Pathology & Laboratory Medicine
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
Nina A van de Lest, Malu Zandbergen, Daphne H T IJpelaar, Ron Wolterbeek, Jan A Bruijn, Ingeborg M Bajema, Marion Scharpfenecker
Introduction: Minimal change disease is a common cause of nephrotic syndrome. In general, patients with minimal change disease respond to corticosteroids and have excellent long-term renal survival. However, some patients have less favorable outcome. These patients are often thought to have progressed to focal segmental glomerulosclerosis. We previously reported that a segmental loss of podocyte markers is present before the development of focal segmental glomerulosclerosis in a rat model...
January 2018: KI Reports
Bojana Šimunov, Mihaela Gunjača, Branislav Čingel, Dinko Škegro, Mladen Knotek
BACKGROUND: Kidney biopsy is frequently performed in our centre as an outpatient procedure. The aim of this study was to evaluate the safety of biopsy in the outpatient setting. METHODS: We analysed kidney biopsies performed from March 2013 to February 2017. Seven hundred twenty-five biopsies performed in the outpatient setting were identified: There were 592 transplant and 133 native biopsies including 3 solitary kidney biopsies. All were performed under ultrasound guidance using a 16G or 18G needle, with freehand technique...
January 4, 2018: Nephron
Yeawon Kim, Sun-Ji Park, Scott R Manson, Carlos Af Molina, Kendrah Kidd, Heather Thiessen-Philbrook, Rebecca J Perry, Helen Liapis, Stanislav Kmoch, Chirag R Parikh, Anthony J Bleyer, Ying Maggie Chen
ER stress has emerged as a signaling platform underlying the pathogenesis of various kidney diseases. Thus, there is an urgent need to develop ER stress biomarkers in the incipient stages of ER stress-mediated kidney disease, when a kidney biopsy is not yet clinically indicated, for early therapeutic intervention. Cysteine-rich with EGF-like domains 2 (CRELD2) is a newly identified protein that is induced and secreted under ER stress. For the first time to our knowledge, we demonstrate that CRELD2 can serve as a sensitive urinary biomarker for detecting ER stress in podocytes or renal tubular cells in murine models of podocyte ER stress-induced nephrotic syndrome and tunicamycin- or ischemia-reperfusion-induced acute kidney injury (AKI), respectively...
December 7, 2017: JCI Insight
Rebecca Preston, Helen M Stuart, Rachel Lennon
Steroid-resistant nephrotic syndrome (SRNS) is a common cause of chronic kidney disease in childhood and has a significant risk of rapid progression to end-stage renal disease. The identification of over 50 monogenic causes of SRNS has revealed dysfunction in podocyte-associated proteins in the pathogenesis of proteinuria, highlighting their essential role in glomerular function. Recent technological advances in high-throughput sequencing have enabled indication-driven genetic panel testing for patients with SRNS...
November 27, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
O Boyer, V Baudouin, E Bérard, C Dossier, V Audard, V Guigonis, I Vrillon
Nephrotic syndrome (NS) is defined by massive proteinuria and hypoalbuminemia, with resulting hyperlipidemia and edema. The most common cause of NS in children is idiopathic nephrotic syndrome (INS), also called nephrosis. Its annual incidence has been estimated to 1-4 per 100,000 children and varies with age, race, and geography. Many agents or conditions have been reported to be associated with INS such as infectious diseases, drugs, allergy, vaccinations, and malignancies. The disease may occur during the 1st year of life, but it usually starts between the ages of 2 and 7 years...
December 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Emel Isiyel, Kibriya Fidan, Bahar Buyukkaragoz, Meltem Akcaboy, Yasar Kandur, Ipek Isik Gonul, Necla Buyan, Sevcan Bakkaloglu, Oguz Soylemezoglu
Renal biopsy is an important diagnostic procedure in pediatric nephrology. This study retrospectively investigates the indications, results and complications in a single tertiary children's hospital in Turkey. We evaluated the native and transplant kidney biopsies in Gazi University Pediatric Nephrology Department between 2001 and 2015. A total of 196 biopsies (144 natives and 52 transplants) were included into the study. The mean age of the patients was respectively 10.8 ± 3.5, 13.9 ± 1.5 years...
November 2017: Renal Failure
Chunlei Lu, Ke Zuo, Yinghui Lu, Shaoshan Liang, Xianghua Huang, Caihong Zeng, Jiong Zhang, Yu An, Jinquan Wang
RATIONALE: Apolipoprotein A-1 (ApoA-1)-related amyloidosis is characterized by the deposition of ApoA-1 in various organs and can be either hereditary or nonhereditary. It is rare and easily misdiagnosed. Renal involvement is common in hereditary ApoA-1 amyloidosis, but rare in the nonhereditary form. PATIENT CONCERNS: We reported two cases with ApoA-1 amyloidosis, a 64-year-old man suffering from nephrotic syndrome and a 40-year-old man with nephrotic syndrome and splenomegaly...
September 2017: Medicine (Baltimore)
Anjali Mohapatra, Shailesh Kakde, Vellaichamy M Annapandian, Anna T Valson, Neelaveni Duhli, Anila Korula, Smita Mary Matthai, Anna B Pulimood, Vinoi G David, Suceena Alexander, Shibu Jacob, Santosh Varughese, Basu Gopal, Tamilarasi Veerasamy, George T John
AIM: We report findings from a large single centre paediatric renal biopsy cohort in South Asia. METHODS: We analyzed all renal biopsies performed on children aged ≤18 years between 1996 and 2015 at our centre. The clinical characteristics and histological diagnosis pertaining to each case, distribution of renal diseases in children with various clinical presentations, and changes in the pattern of kidney disease during the study period were analyzed. RESULTS: A total of 1740 paediatric kidney biopsies were performed during the study period...
August 28, 2017: Nephrology
Ho Sik Shin, Dae Hyeon Cho, Soo Kyoung Kang, Hyun Jeong Kim, Soo Young Kim, Joung Wook Yang, Gyong Hoon Kang, Ye Na Kim, Yeonsoon Jung, Bong-Kwon Cheon, Hark Rim
BACKGROUND: Several registries and centers have reported the results of renal biopsies from different parts of the world. As there are few data regarding the epidemiology of glomerulonephritis (GN) in South Korea, we conducted this study on renal biopsy findings during the last 20 years from a single center. METHODS: Data for 818 patients who underwent renal biopsy at our center between 1992 and 2011 were collected retrospectively. All kidney specimens were examined with light microscopy (LM) and immunofluorescent microscopy (IF)...
November 2017: Renal Failure
Amar H Kelkar, Anushi A Shah, Sherri L Yong, Zohair Ahmed
RATIONALE: In the adult patient, hemophagocytic lymphohistiocytosis (HLH) is uncommon and frequently difficult to diagnose due to its nonspecific presentation and numerous complications. PATIENT CONCERNS: Herein, we present the case of a 25-year-old female who initially presented for evaluation of persistent fevers and fatigue. She was found to have splenomegaly, generalized lymphadenopathy, pancytopenia, and acute hepatic failure. DIAGNOSES, INTERVENTIONS, AND OUTCOMES: Her course was further complicated by the development of nephrotic syndrome and autoimmune hemolytic anemia (AIHA)...
July 2017: Medicine (Baltimore)
Yosr Chaabouni, Rahma Guesmi, Yosr Hentati, Khaoula Kammoun, Mohamed Ben Hmida, Zeineb Mnif, Tahya Boudawara, Jamil Hachicha
The horseshoe kidney is a frequent urological birth defect. The most frequent complications are urinary tract infections, stones and hydronephrosis. The occurrence of glomerular disease in horseshoe kidney is rare. Therefore, we report the first case of minimal change disease occurring in a patient with horseshoe kidney in literature. A 22-year-old Caucasian man without personal or family medical history admitted to the pneumology department for a pulmonary artery embolism. In presence of a generalized oedema, a biological assessment was performed yielding intense nephrotic syndrome with urine protein excretion 22g/day...
2017: Pan African Medical Journal
Shigeo Hara, Hiroyasu Tsukaguchi, Tastufumi Oka, Makiko Kusabe, Masayuki Mizui, Kensuke Joh
We herein report the case of a 64-year-old male who presented with progressive glomerulonephritis notable for organized and striated ultra-substructures. The patient was diagnosed with hypertension and proteinuria 3 years prior to admission and subsequently developed nephrotic syndrome and impairment of renal function. Laboratory tests did not reveal any evidence of infections or autoimmune diseases. Monoclonal gammopathy was not detected in serum or urine, although a small population of abnormal plasma cell clones was detected by flow cytometry...
July 2017: Ultrastructural Pathology
Samuel N Uwaezuoke
Two histological subtypes of idiopathic nephrotic syndrome are commonly recognized in children, namely minimal change nephropathy and focal segmental glomerulosclerosis. Children with minimal change nephropathy (the majority of whom are steroid-sensitive) and focal segmental glomerulosclerosis (the majority of whom are steroid-resistant) require early identification in order to ensure appropriate therapeutic intervention and better outcome. Although renal biopsy and histology remain the ideal diagnostic steps to identify these histological subtypes, reports indicate that serum and urinary biomarkers are now being utilized in the investigation of childhood idiopathic nephrotic syndrome...
2017: International Journal of Nephrology and Renovascular Disease
Agnes Trautmann, Sven Schnaidt, Beata S Lipska-Ziętkiewicz, Monica Bodria, Fatih Ozaltin, Francesco Emma, Ali Anarat, Anette Melk, Marta Azocar, Jun Oh, Bassam Saeed, Alaleh Gheisari, Salim Caliskan, Jutta Gellermann, Lina Maria Serna Higuita, Augustina Jankauskiene, Dorota Drozdz, Sevgi Mir, Ayse Balat, Maria Szczepanska, Dusan Paripovic, Alexandra Zurowska, Radovan Bogdanovic, Alev Yilmaz, Bruno Ranchin, Esra Baskin, Ozlem Erdogan, Giuseppe Remuzzi, Agnieszka Firszt-Adamczyk, Elzbieta Kuzma-Mroczkowska, Mieczyslaw Litwin, Luisa Murer, Marcin Tkaczyk, Helena Jardim, Anna Wasilewska, Nikoleta Printza, Kibriya Fidan, Eva Simkova, Halina Borzecka, Hagen Staude, Katharina Hees, Franz Schaefer
We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models...
October 2017: Journal of the American Society of Nephrology: JASN
William G Couser
Membranous nephropathy (MN) is a unique glomerular lesion that is the most common cause of idiopathic nephrotic syndrome in nondiabetic white adults. About 80% of cases are renal limited (primary MN, PMN) and 20% are associated with other systemic diseases or exposures (secondary MN). This review focuses only on PMN. Most cases of PMN have circulating IgG4 autoantibody to the podocyte membrane antigen PLA2R (70%), biopsy evidence PLA2R staining indicating recent immunologic disease activity despite negative serum antibody levels (15%), or serum anti-THSD7A (3%-5%)...
June 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
Alanoud Alshami, Abishek Roshan, Marisa Catapang, Jasper J Jöbsis, Trevor Kwok, Nonnie Polderman, Jennifer Sibley, Matt Sibley, Cherry Mammen, Douglas G Matsell
BACKGROUND: Most cases of childhood nephrotic syndrome (NS) are due to minimal change disease (MCD), while a minority of children have focal segmental glomerulosclerosis (FSGS) and an unfavorable clinical course, requiring a kidney biopsy to confirm diagnosis. We hypothesized that clinical characteristics at diagnosis and initial response to corticosteroid treatment accurately predict FSGS and can be used to guide consistent practice in the indications for kidney biopsy. METHODS: This was a case control study (1990-2012)...
May 24, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Shimon Storch, Nadav Willner, Aurora Toubi, Simona Croitoru, Vladimir Wolfson, Ibrahim Matar, Elinor Grushka, Majed Odeh, Efrat Wolfovits, Elad Schiff, Yitzhak Rosner, Elias Toubi, Aharon Kessel, Ofer Ben Izhak, Boaz Moskovitz, Ofer Nativ
INTRODUCTION: Little is known about the prevalence of kidney diseases according to renal biopsy in Israel. Since updated literature worldwide emphasizes changing etiologies of chronic kidney disease, it is crucial to research and define the epidemiology and pathology of kidney disease in Israel. Hereby, we introduce an original review of the prevalence of kidney diseases in our study population, which we believe reflects the prevalence of kidney diseases in the population of Israel. AIMS: To investigate the prevalence of kidney diseases diagnosed by renal biopsy, according to age, gender, race and clinical symptoms...
September 2016: Harefuah
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