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Nephrotic syndrome

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https://www.readbyqxmd.com/read/27913276/association-of-genetic-polymorphisms-of-angiopoietin-like-4-with-severity-of-posttransplant-proteinuria-in-kidney-allograft-recipients
#1
Youngil Chang, Tariq Shah, Jaewook Yang, David I Min
BACKGROUND: Proteinuria is a hallmark of glomerular injury, and persistent proteinuria is associated with graft failure in kidney transplant patients. Recently, it is known that the level of circulating angiopoietin-like 4 (ANGPTL4) is elevated in the patients with human nephrotic syndrome, in which ANGPTL4 is responsible for relieving proteinuria. PURPOSE: The purpose of this study is to determine effects of clinical factors and genetic polymorphism of ANGPTL4 on proteinuria after kidney transplantation...
November 29, 2016: Transplant Immunology
https://www.readbyqxmd.com/read/27904864/nephrotic-range-proteinuria-and-peripheral-edema-in-a-child-not-only-idiopathic-nephrotic-syndrome
#2
Valentina Dolcemascolo, Marina Vivarelli, Manuela Colucci, Francesca Diomedi-Camassei, Rossella Piras, Marta Alberti, Francesco Emma
Hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy (TMA) mainly occurring in renal and cerebral microvessels. Although the most common cause of HUS in children is Shiga toxin-producing Escherichia coli, atypical forms in which Shiga toxin is not the trigger may occur. Research over the last few years has shown that complement dysregulation secondary to mutations of genes coding for proteins involved in the regulation of the alternative pathway of complement account for most forms of atypical HUS (aHUS)...
September 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27904828/dent-s-disease-complicated-by-nephrotic-syndrome-a-case-report
#3
Guohua He, Hongwen Zhang, Shanshan Cao, Huijie Xiao, Yong Yao
Dent's disease is an X-linked recessive proximal tubular disorder that mostly affects male patients in childhood or early adult life. The condition is caused by mutations in the CLCN5 (Dent disease 1) or OCRL (Dent disease 2) genes located on chromosome Xp11.22 and Xq25, respectively. In most male patients, proteinuria is subnephrotic but may reach nephrotic levels. Here, we report the first case of Dent's disease complicated by nephrotic syndrome. Dent's disease should be considered in the differential diagnosis of nephrotic syndrome, and especially in male patients with early onset of nephrotic syndrome...
November 2016: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/27904002/diffuse-mesangial-sclerosis-in-a-child-with-dyskeratosis-congenita-leading-to-end-stage-renal-disease
#4
Abidi Kamel, Taha Sayari, Manel Jellouli, Yousra Hammi, Rim Ghoucha Louzir, Tahar Gargah
Dyskeratosis congenita (DC) is a very rare inherited disorder. It is caused by dysfunction of telomere maintenance. It involves RNA telomerase components relevant to various mutations leading to a classic triad of physical findings consisting of nail dystrophy of the hands and feet, mucosal leukoplakia, and reticular pigmentation of the skin, most commonly on the head, neck, and trunk. Bone marrow failure along with pulmonary complications and malignancies are all common causes of premature death in patients with DC as well as other abnormalities...
November 2016: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/27900977/unconventional-strategies-in-the-battle-of-focal-and-segmental-glomerulosclerosis
#5
Satish Mendonca, R S V Kumar, Devika Gupta, Pooja Gupta, Satish Barki, M L Sharma
A 24-year-old male presented with classic features of the nephrotic syndrome. An initial renal biopsy revealed minimal change disease and thereafter, a second biopsy showed features of focal and segmental glomerulosclerosis. There was no response to conventional immunosuppression, and the patient had to be given rituximab; in spite of this, he went on to develop end-stage renal disease. He continued to have heavy proteinuria leading to severe hypoalbuminemia, thrombosis, infections, and malnutrition, placing the patient in a life-threatening situation...
November 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27900972/failure-to-thrive-and-nephrocalcinosis-due-to-distal-renal-tubular-acidosis-a-rare-presentation-of-pediatric-lupus-nephritis
#6
Madhumita Nandi, Mrinal Kanti Das, Sukanta Nandi
A 9-year-old female child was initially diagnosed of having nephrocalcinosis with distal renal tubular acidosis (dRTA) while investigating for short stature. She later on developed features of nephrotic syndrome (NS) while on treatment for RTA. Investigation for the cause of NS revealed very strong serological evidence in favor of systemic lupus erythematosus (SLE). Histopathological confirmation could not be done due to bilateral severely contracted kidneys. There are a few case reports of dRTA as the presentation of SLE, but nephrocalcinosis with dRTA with subsequent manifestation of SLE has hitherto not been reported in literature...
November 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27890680/the-putative-role-of-maldi-msi-in-the-study-of-membranous-nephropathy
#7
Andrew Smith, Vincenzo L'Imperio, Elena Ajello, Franco Ferrario, Niccolò Mosele, Martina Stella, Manuel Galli, Clizia Chinello, Federico Pieruzzi, Goce Spasovski, Fabio Pagni, Fulvio Magni
Membranous Nephropathy (MN) is an immunocomplex mediated renal disease that represents the leading cause of nephrotic syndrome in adults and is one of the most frequent glomerulopathies worldwide. This glomerular disease can manifest as primary (idiopathic) or secondary and this distinction is crucial when choosing the most appropriate management of patients. In secondary cases, the best strategy consists in treating the underlying disease whereas in primary forms, the possible identification of confirmatory markers of the idiopathic etiology underlining the process is requested by clinicians...
November 23, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27885584/wt1-and-nphs2-gene-mutation-analysis-and-clinical-management-of-steroid-resistant-nephrotic-syndrome
#8
Aravind Selvin Kumar Ramanathan, Murali Vijayan, Srilakshmi Rajagopal, Padmaraj Rajendiran, Prabha Senguttuvan
Nephrotic syndrome (NS) is a kidney disease predominantly present in children with idiopathic condition; final stage of the disease progresses into end-stage renal disease. Generally, NS is treated using standard steroid therapy, however; most of the children are steroid sensitive and about 15-20% are non-responders (SRNS). Non-responsiveness of these children would be a risk with the possibility of mutational changes in podocyte genes (NPHS1, NPHS2, WT1, PLCE1). The mutation in podocyte genes is associated with SRNS...
November 25, 2016: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/27884537/-nephrotic-syndrome-complicated-by-chylous-ascites-in-a-girl-of-2%C3%A2-years-and-8%C3%A2-months-old
#9
J R Mabiala Babela, L C Ollandzobo Ikobo, R Loumingou, E R Nika, A Mouko
We report on a case of nephrotic syndrome with focal and segmental hyalinosis complicated by chylous ascites in a girl of 2 years and 8 months old. This pure nephrotic syndrome in its early stage was initially treated with intensive steroid treatment at 2mg/kg/day orally for 2 months, followed by a bolus of methylprednisolone. The persistence of proteinuria meant corticosteroid resistance. Renal biopsy then revealed focal and segmental hyalinosis. A recurrence of the edema-ascites syndrome was associated with macroscopic hematuria...
November 21, 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/27882743/congenital-nephrotic-syndrome-with-a-novel-nphs1-mutation
#10
Chikage Yoshizawa, Yasuko Kobayashi, Yuka Ikeuchi, Masahiko Tashiro, Satoko Kakegawa, Toshio Watanabe, Yoshimitsu Goto, Koichi Nakanishi, Norishige Yoshikawa, Hirokazu Arakawa
Congenital nephrotic syndrome of the Finnish type (CNF) is a rare autosomal recessive disorder. The incidence of CNF is relatively high in Finland but considerably lower in other countries. We encountered a male newborn with CNF, associated with compound heterozygous mutations in nephrosis 1, congenital, Finnish type (NPHS1). The patient was admitted to hospital as a preterm infant. Physical and laboratory findings fulfilled the diagnostic criteria of nephrotic syndrome, and were compatible with a diagnosis of CNF, but there was no family history of the disease...
November 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27872827/zinc-supplementation-as-an-adjunct-to-standard-therapy-in-childhood-nephrotic-syndrome-a-systematic-review
#11
Girish Chandra Bhatt, Shikha Jain, Rashmi Ranjan Das
AIM: To evaluate the role of zinc as add on treatment to the "recommended treatment" of nephrotic syndrome (NS) in children. METHODS: All the published literature through the major databases including Medline/Pubmed, Embase, and Google Scholar were searched till 31(st) December 2015. Reference lists from the articles were reviewed to identify additional pertinent articles. Retrieved papers concerning the role of zinc in childhood NS were reviewed by the authors, and the data were extracted using a standardized data collection tool...
November 8, 2016: World Journal of Clinical Pediatrics
https://www.readbyqxmd.com/read/27864708/thyroid-dysfunction-and-kidney-disease-an-update
#12
REVIEW
Pedro Iglesias, María Auxiliadora Bajo, Rafael Selgas, Juan José Díez
Thyroid hormones influence renal development, kidney hemodynamics, glomerular filtration rate and sodium and water homeostasis. Hypothyroidism and hyperthyroidism affect renal function by direct renal effects as well as systemic hemodynamic, metabolic and cardiovascular effects. Hypothyroidism has been associated with increased serum creatinine and decreased glomerular filtration rate. The reverse effects have been reported in thyrotoxicosis. Most of renal manifestations of thyroid dysfunction are reversible with treatment...
November 18, 2016: Reviews in Endocrine & Metabolic Disorders
https://www.readbyqxmd.com/read/27862787/two-diseases-one-remedy-systemic-amyloidosis-secondary-to-hidradenitis-suppurativa-treatment-with-infliximab
#13
İlkay Özer, Cengiz Karaçin, Esra Adışen, Galip Güz, Mehmet Ali Gürer
Hidradenitis suppurativa, known as acne inversa, is a relapsing and chronic inflammatory skin disease affecting the skin folds. During the chronic course of the disease many local complications like fistulae to other tissues or systemic complications including anemia, secondary amyloidosis, lymphedema, nephrotic syndrome, artropathy may take place. Amyloid A amyloidosis is a rare complication of hidradenitis suppurativa, which has been described in a limited number of case reports. Herein, we present such a patient that had developed AA amyloidosis during the course of hidradenitis suppurativa...
November 12, 2016: Dermatologic Therapy
https://www.readbyqxmd.com/read/27861367/impact-of-acute-kidney-injury-on-coagulation-in-adult-minimal-change-nephropathy
#14
Meng-Jie Huang, Ri-Bao Wei, Ting-Yu Su, Yang Wang, Qing-Ping Li, Xi Yang, Xiao-Meng Lv, Xiang-Mei Chen
A hypercoagulable state exists in patients with nephrotic syndrome (NS), which more easily leads to venous thromboembolism (VTE). However, whether acute kidney injury (AKI), a common complication of NS, affects the hypercoagulable state and VTE has rarely been elucidated. In this study, we aimed to explore coagulation changes and analyze relevant influencing factors in NS-AKI patients.A total of 269 consecutive NS patients with minimal change disease (MCD) between 2011 and 2016 were included in this observational study...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27855638/alteration-of-histone-h3k4-methylation-in-glomerular-podocytes-associated-with-proteinuria-in-patients-with-membranous-nephropathy
#15
Takayuki Fujino, Naoyuki Hasebe
BACKGROUND: Histone H3K4 trimethylation (H3K4 me3) is found in active euchromatic regions and plays an important role in podocyte function in which actin filaments are abundant in the foot processes. The pathogenesis of membranous nephropathy (MN), the most prevalent cause of primary nephrotic syndrome in the middle-aged and elderly, is podocyte dysfunction. METHODS: We investigated the role of H3K4 me3 in podocyte dysfunction in nephrotic syndrome using cultured podocytes and a mouse proteinuria model induced by LPS...
November 17, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27853075/minimal-change-nephrotic-syndrome-sequentially-complicated-by-acute-kidney-injury-and-painful-skin-ulcers-due-to-calciphylaxis
#16
Ryuta Sato, Tetsu Akimoto, Toshimi Imai, Saki Nakagawa, Mari Okada, Atsushi Miki, Shinichi Takeda, Hisashi Yamamoto, Osamu Saito, Shigeaki Muto, Eiji Kusano, Daisuke Nagata
Calciphylaxis is rare cutaneous manifestation associated with painful skin ulceration and necrosis. It primarily occurs in patients with end-stage chronic kidney disease. In this report, we would like to show our experience with a male patient presenting with minimal change nephrotic syndrome that was sequentially complicated by acute kidney injury and painful ulcerative cutaneous lesions due to calciphylaxis. There seemed to be several contributing factors, including a disturbance of the patient's mineral metabolism and the systemic use of glucocorticoids and warfarin...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27847659/interferon-induced-focal-segmental-glomerulosclerosis
#17
Yusuf Kayar, Nuket Bayram Kayar, Nadir Alpay, Jamshid Hamdard, Iskender Ekinci, Sebnem Emegil, Rabia Bag Soydas, Birol Baysal
Behçet's disease is an inflammatory disease of unknown etiology which involves recurring oral and genital aphthous ulcers and ocular lesions as well as articular, vascular, and nervous system involvement. Focal segmental glomerulosclerosis (FSGS) is usually seen in viral infections, immune deficiency syndrome, sickle cell anemia, and hyperfiltration and secondary to interferon therapy. Here, we present a case of FSGS identified with kidney biopsy in a patient who had been diagnosed with Behçet's disease and received interferon-alpha treatment for uveitis and presented with acute renal failure and nephrotic syndrome associated with interferon...
2016: Case Reports in Nephrology
https://www.readbyqxmd.com/read/27833453/benign-acute-childhood-myositis-complicating-influenza-b-infection-in-a-boy-with-idiopathic-nephrotic-syndrome
#18
Piotr Skrzypczyk, Joanna Przychodzień, Małgorzata Pańczyk-Tomaszewska
INTRODUCTION: Benign acute childhood myositis (BACM) is an acute complication of an infection characterized by calf pain, limitation of lower limb mobility, an increase in serum creatine kinase, and a self-limiting course. No reports of BACM in children with idiopathic nephrotic syndrome (INS) can be found in the literature. CASE REPORT: A 5-year-old boy with steroid-sensitive INS presented with fever, leg pain, and problems with walking. Physical examination showed pharyngeal erythema, preserved movements in all joints, and weakness of leg muscles...
2016: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/27833441/lupus-nephritis-in-children-10-years-experience
#19
Hanna Szymanik-Grzelak, Elżbieta Kuźma-Mroczkowska, Jadwiga Małdyk, Małgorzata Pańczyk-Tomaszewska
Systemic lupus erythematosus (SLE) in children is usually more severe than it is in adults and there is a higher incidence of renal involvement. We described 18 children (16 girls, 2 boys) with lupus nephritis (LN), whose average age was 14.4 ±1.81 years. Disease activity was assessed according to SLEDAI (SLE Disease Activity Index). Renal biopsy was classified according to the INS/RPS (International Society of Nephrology/Renal Pathology Society). The patients were treated with steroids (100%) and pulses of cyclophosphamide (88...
2016: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/27833440/levamisole-therapy-in-children-with-frequently-relapsing-and-steroid-dependent-nephrotic-syndrome-a-single-center-experience
#20
Elżbieta Kuźma-Mroczkowska, Piotr Skrzypczyk, Małgorzata Pańczyk-Tomaszewska
INTRODUCTION: Numerous studies suggest that levamisole, an antihelmintic agent with an immunomodulatory effect, reduces the number of relapses in children with frequently relapsing and steroid-dependent nephrotic syndrome (FRNS/SDNS). The aim of the study was to present a single center's experience in treatment of FRNS and SDNS with levamisole. MATERIAL AND METHODS: Among 72 children with FRNS/SDNS treated in our department with levamisole in the years 1984-2011 we studied in detail 53 patients (mean age: 6...
2016: Central-European Journal of Immunology
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