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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
Rossana Malatesta-Muncher, Karen W Eldin, Laurence H Beck, Mini Michael
BACKGROUND: Idiopathic membranous nephropathy is an uncommon cause of nephrotic syndrome in children and can present treatment challenges. The current treatment options of steroids and cyclophosphamide, cyclosporine, or mycophenolate require prolonged treatment durations and the associated side effects may result in nonadherence in children, especially in adolescents. CASE-DIAGNOSIS: We report two adolescent patients with idiopathic membranous nephropathy with nephrotic range proteinuria and elevated anti-phospholipase A2 receptor levels who did not achieve remission with steroids and were later treated with rituximab...
March 15, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Ryo Hatano, Ai Takeda, Yukiko Abe, Kotoku Kawaguchi, Itsuro Kazama, Mitsunobu Matsubara, Shinji Asano
Ezrin is highly expressed in glomerular podocytes and is reported to form a multi-protein complex with scaffold protein Na+ /H+ exchanger regulatory factor 2 (NHERF2) and podocalyxin, a major sialoprotein. Podocalyxin-knockout mice died within 24 h of birth with anuric renal failure, whereas NHERF2-knockout mice show no apparent changes in the glomerular functions. However, the physiological roles of ezrin in glomerular podocytes remain unclear. Here, we investigated the importance of ezrin in the regulation of glomerular podocyte function using ezrin-knockdown mice (Vil2kd/kd )...
March 14, 2018: Scientific Reports
Yu Meng, FangGen Lu, Lin Shi, MeiChu Cheng, Jie Zhang
RATIONALE: The use of anticoagulants is a contributor to gastrointestinal (GI) bleeding. Most bleeding patients on anticoagulant therapy such as warfarin commonly have basic lesions existing in their GI mucosa. PATIENT CONCERNS: We report a case of major GI bleeding following the use of anticoagulants in a patient with hookworm infection. DIAGNOSES: The patient was diagnosed with nephrotic syndrome with pulmonary embolism. INTERVENTIONS: He was treated with anticoagulants and suffered from acute major GI bleeding during the treatment...
March 2018: Medicine (Baltimore)
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
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...
March 13, 2018: American Journal of Nephrology
Janice Crespo-Salgado, Tyrus Stewart, Diego H Aviles
No abstract text is available yet for this article.
March 13, 2018: American Journal of Nephrology
Fei Liu, Jian-Hua Mao
BACKGROUND: Calcineurin inhibitors (CNIs) are commonly given to transplant recipients of kidneys and other solid organs and to patients with immune disorders, such as steroid-resistant nephrotic syndrome, steroid-dependent nephrotic syndrome, and frequent relapse nephrotic syndrome. Although CNIs remain the most effective available immunosuppressant agent, there is clinical concern regarding possible long-term nephrotoxicity. This concern is especially significant in children who have a longer life expectancy and greater growth rate...
March 12, 2018: World Journal of Pediatrics: WJP
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
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
Ilan Rozenberg, Andres Kotliroff, Tania Zahavi, Sydney Benchetrit
BACKGROUND: Idiopathic membranous nephropathy (IMN) is one of the most common causes of nephrotic syndrome (NS) in Caucasian adults. Most patients have good renal prognosis, but 30-40% may progress to end stage renal disease (ESRD). OBJECTIVES: To evaluate the efficacy and safety of immunosuppressive treatment (IST) in high-risk patients. METHODS: All IMN patients diagnosed by kidney biopsy from 2004-2010 were included. Clinical and laboratory data were collected at each follow-up visit...
March 2018: Israel Medical Association Journal: IMAJ
Eunjin Bae, Sung Woo Lee, Seokwoo Park, Dong Ki Kim, Hajeong Lee, Hyuk Huh, Ho Jun Chin, Shina Lee, Dong-Ryeol Ryu, Ji In Park, Sejoong Kim, Dong Jun Park, Shin-Wook Kang, Yon Su Kim, Yun Kuy Oh, Yong Chul Kim, Chun Soo Lim, Jung Tak Park, Jung Pyo Lee
Idiopathic membranous nephropathy (MN) is the most common glomerulonephritis in elderly patients showing nephrotic syndrome. However, little is known about its treatment options and outcomes in elderly MN patients at long term follow-up. We retrospectively enrolled patients with biopsy-proven MN between April 1990 and December 2015 from eight tertiary hospitals in Korea. Among them, we excluded patients who had secondary causes of MN and subnephrotic-range proteinuria. We evaluated the presenting features and clinical outcomes and analyzed the all-cause mortality, renal outcomes, infection, and remission with respect to age...
March 3, 2018: Archives of Gerontology and Geriatrics
Satish Maharaj, Karan Seegobin, Stephen Chrzanowski, Simone Chang
Streptococcus anginosus is a clinically important pathogen that is emerging globally but remains poorly investigated. Here, we report the first case of acute glomerulonephritis resulting from infection with S. anginosus Glomerulonephritis is typically caused by S. pyogenes and reports secondary to other strains including S. zooepidemicus and S. constellatus exist. Infection with S. anginosus in this patient was associated with acute nephritis (haematuria, oedema and hypertension), nephrotic syndrome and progressive azotemia...
March 9, 2018: BMJ Case Reports
Sanda Mrabet, Narjess Ben Aicha, Nihed Abdessayed, Moncef Mokni, Abdellatif Achour
BACKGROUND: Membranous nephropathy (MN), the leading cause of nephrotic syndrome in adults, is characterized by the deposition of subepithelial immune deposits. Most of the cases are primary, while only approximately 25% of the cases are secondary to some known diseases. Recently, MN has been considered to be a possible presentation of chronic graft-versus-host disease (GVHD) of the kidney in allogeneic hematopoietic stem cell transplantation (HSCT) patients. In autologous HSCT populations, there have been scarce reports of associated MN, as a result of immune dysregulation leading to systemic autoimmunity and miming chronic GVHD...
March 9, 2018: BMC Nephrology
Y F Dong, L W Sun, B Zhang, X Y Kuang, X L Niu, Y L Kang, S Hao, P Wang, Z Li, G H Zhu, W Y Huang, Y Wu
Objective: To explore the clinical features and expression of PLA(2)R in renal tissue of children with idiopathic membranous nephropathy. Methods: Retrospective study was performed in patients with membranous nephropathy diagnosed through renal biopsy and the follow-up time was at least half a year in Shanghai Children ' s Hospital from January 2010 to February 2017. We compared their clinicopathological and pathological findings of IMN. Indirect immunofluorescence assay was used to detect glomerular PLA(2)R expression...
March 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
A J Scheen, N Paquot, P Lancellotti, J M Krzesinski
Differential diagnosis of lower limb oedema is a common exercise in clinical practice. Taking the example of a patient presenting with such clinical picture in the presence of type 2 diabetes and arterial hypertension with coronary heart disease and chronic kidney disease as comorbidities, we discuss here the respective contributions of congestive heart failure, renal impairment (and possibly nephrotic syndrome) and liver disease in the development of lower limb oedema. The focus is made on a careful patient history and a meticulous clinical examination, two crucial steps that should allow prescribing well selected simple complementary procedures and rapidly make the final diagnosis...
February 2018: Revue Médicale de Liège
I N Aboobacker, A Krishnakumar, S Narayanan, B Hafeeque, J C Gopinathan, F Aziz
Nail-Patella syndrome (NPS) is an inherited disease with characteristic nail, limb, and renal anomalies. While almost all patients manifest classical skeletal and nail abnormalities, renal involvement is seen in only 40% of patients. Asymptomatic proteinuria is the most common renal presentation. Although a substantial number of patients can progress to nephrotic range proteinuria, development of end-stage kidney disease is rare. Pathological abnormalities are appreciable only by electron microscopy. Our patient who had not been diagnosed with NPS previously presented with new-onset proteinuria during the second trimester of her first pregnancy...
January 2018: Indian Journal of Nephrology
N Prasad, R Manjunath, D Rangaswamy, A Jaiswal, V Agarwal, D Bhadauria, A Kaul, R Sharma, A Gupta
Calcineurin inhibitors (CNIs) are the preferred drugs for treatment of childhood steroid-resistant nephrotic syndrome (SRNS) who are also resistant to cyclophosphamide (CYC). Although few studies have shown a benefit of one over the other, efficacy and safety of either CNIs (tacrolimus [TAC] or cyclosporine [CSA]) in this special population remained to be assessed in long-term studies. Forty-five children with SRNS who were also resistant to CYC (CYC-SRNS) from January 2006 to June 2011, were included in the study...
January 2018: Indian Journal of Nephrology
M Sahay, Y Saivani, K Ismal, P S Vali
Systemic lupus erythematosus is common in our country, and renal involvement is an important cause of chronic kidney disease. This study was aimed at comparing the three regimens, i.e., cyclophosphamide-based regimes (low dose and high dose) and mycophenolate mofetil (MMF)-based regime and determining if cyclophosphamide (CPM)-based regime can be an effective, safe, and cheap alternative to MMF-based regime in a resource-limited setting. Out of 144 patients, females constituted 89%. Nephrotic nephritic presentation was the most common...
January 2018: Indian Journal of Nephrology
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
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