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Nephrotic syndrom

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https://www.readbyqxmd.com/read/29679356/regional-variations-in-immunosuppressive-therapy-in-patients-with-primary-nephrotic-syndrome-the-japan-nephrotic-syndrome-cohort-study
#1
Ryohei Yamamoto, Enyu Imai, Shoichi Maruyama, Hitoshi Yokoyama, Hitoshi Sugiyama, Kosaku Nitta, Tatsuo Tsukamoto, Shunya Uchida, Asami Takeda, Toshinobu Sato, Takashi Wada, Hiroki Hayashi, Yasuhiro Akai, Megumu Fukunaga, Kazuhiko Tsuruya, Kosuke Masutani, Tsuneo Konta, Tatsuya Shoji, Takeyuki Hiramatsu, Shunsuke Goto, Hirofumi Tamai, Saori Nishio, Arimasa Shirasaki, Kojiro Nagai, Kunihiro Yamagata, Hajime Hasegawa, Hidemo Yasuda, Shizunori Ichida, Tomohiko Naruse, Kei Fukami, Tomoya Nishino, Hiroshi Sobajima, Satoshi Tanaka, Toshiyuki Akahori, Takafumi Ito, Terada Yoshio, Ritsuko Katafuchi, Shouichi Fujimoto, Hirokazu Okada, Eiji Ishimura, Junichiro James Kazama, Keiju Hiromura, Tetsushi Mimura, Satashi Suzuki, Yosuke Saka, Tadashi Sofue, Yusuke Suzuki, Yugo Shibagaki, Kiyoki Kitagawa, Kunio Morozumi, Yoshiro Fujita, Makoto Mizutani, Takashi Shigematsu, Naoki Kashihara, Hiroshi Sato, Seiichi Matsuo, Ichiei Narita, Yoshitaka Isaka
BACKGROUND: The lack of high-quality clinical evidences hindered broad consensus on optimal therapies for primary nephrotic syndromes. The aim of the present study was to compare prevalence of immunosuppressive drug use in patients with primary nephrotic syndrome across 6 regions in Japan. METHODS: Between 2009 and 2010, 380 patients with primary nephrotic syndrome in 56 hospitals were enrolled in a prospective cohort study [Japan Nephrotic Syndrome Cohort Study (JNSCS)], including 141, 151, and 38 adult patients with minimal change disease (MCD), membranous nephropathy (MN), and focal segmental glomerulosclerosis (FSGS), respectively...
April 20, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29673759/alport-syndrome-and-pierson-syndrome-diseases-of-the-glomerular-basement-membrane
#2
REVIEW
Steven D Funk, Meei-Hua Lin, Jeffrey H Miner
The glomerular basement membrane (GBM) is an important component of the kidney's glomerular filtration barrier. Like all basement membranes, the GBM contains type IV collagen, laminin, nidogen, and heparan sulfate proteoglycan. It is flanked by the podocytes and glomerular endothelial cells that both synthesize it and adhere to it. Mutations that affect the GBM's collagen α3α4α5(IV) components cause Alport syndrome (kidney disease with variable ear and eye defects) and its variants, including thin basement membrane nephropathy...
April 16, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29670902/nephrotic-syndrome-genetics-mechanism-and-therapies
#3
EDITORIAL
Jae Il Shin, Andreas Kronbichler, Jun Oh, Björn Meijers
No abstract text is available yet for this article.
2018: BioMed Research International
https://www.readbyqxmd.com/read/29668480/the-6th-rare-disease-south-eastern-europe-see-meeting-skopje-macedonia-november-11th-2017
#4
Zoran Gucev, Velibor Tasic, Momir Polenakovic
The sixth SEE meeting on rare diseases (RDs) was held in MASA the November 10th, 2017. A block of lectures on rare renal diseases started the meeting: nephrotic syndrome, Alport syndrome, atypical HUS, hypophosphatemic rickets, CAKUT were presented in all complexities. Their molecular and genetic mechanisms were discussed. The discovery of a dozen of newly genes in CAKUT, congenital overgrowth, spodilocostal dysplasia, precocious puberty has been done with collaboration of Macedonian and foreign researchers...
December 1, 2017: Prilozi (Makedonska Akademija Na Naukite i Umetnostite. Oddelenie za Medicinski Nauki)
https://www.readbyqxmd.com/read/29668475/rare-renal-disease-in-macedonia-an-update
#5
Velibor Tasic, Zoran Gucev, Momir Polenakovic
Rare renal diseases (RRD) are an important category of rare disease (RD) as they can do great damage to the patients, families and society. The patient may undergo years even decades of numerous investigations including invasive procedures and yet not have definitive and precise diagnose and therefore, no opportunity for appropriate treatment. The great progress in molecular genetic techniques characterized many Mendelian diseases on molecular level. This gave the possibility for appropriate prevention and treatment interventions, genetic counseling and prenatal diagnosis...
December 1, 2017: Prilozi (Makedonska Akademija Na Naukite i Umetnostite. Oddelenie za Medicinski Nauki)
https://www.readbyqxmd.com/read/29665203/tolvaptan-alleviates-excessive-fluid-retention-of-nephrotic-diabetic-renal-failure-unresponsive-to-furosemide
#6
Tesshu Takada, Tsuguto Masaki, Ayako Hoshiyama, Takuya Toki, Yuji Kamata, Masayoshi Shichiri
Patients with diabetic nephropathy develop nephrotic syndrome, and may show limited response to conventional therapy. They often require earlier initiation of renal replacement therapy because they become refractory to diuretics, and experience excessive fluid retention. We aimed to investigate the efficacy of tolvaptan, an oral arginine vasopressin type 2 receptor antagonist, in a case series of 14 severe diabetic renal failure patients who were severely refractory to maximal doses of furosemide and had excessive fluid retention despite preserved cardiac function and residual renal function...
April 17, 2018: Nephrology
https://www.readbyqxmd.com/read/29663071/cyclosporine-a-responsive-congenital-nephrotic-syndrome-with-single-heterozygous-variants-in-nphs1-nphs2-and-plce1
#7
Anna Eichinger, Sabine Ponsel, Carsten Bergmann, Roman Günthner, Julia Hoefele, Kerstin Amann, Bärbel Lange-Sperandio
BACKGROUND: Congenital nephrotic syndrome (CNS) is primarily a monogenetic disease, with the majority of cases due to changes in five different genes: the nephrin (NPHS1), podocin (NPHS2), Wilms tumor 1 (WT1), laminin ß2 (LAMB2), and phospholipase C epsilon 1 (PLCE1, NPHS3) gene. Usually CNS is not responsive to immunosuppressive therapy, but treatment with ACE inhibitors, AT1 receptor blockade and/or indomethacin can reduce proteinuria. If the disease progresses to end-stage renal disease, kidney transplantation is the therapy of choice...
April 16, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29661344/clinical-significance-of-urinary-biomarkers-in-patients-with-primary-focal-segmental-glomerulosclerosis
#8
Qingyan Zhang, Chunming Jiang, Tianfeng Tang, Hengjin Wang, Yangyang Xia, Qiuyuan Shao, Miao Zhang
BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is often accompanied with tubulointerstitial lesion. This study aimed to assess the role of urinary biomarkers in predicting tubulointerstitial lesion and treatment response in FSGS patients. METHODS: Urinary neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), N-acetyl-β-d-glucosaminidase (NAG) and retinol-binding protein (RBP) were measured in 32 FSGS patients and 22 patients with minimal change nephrotic syndrome...
April 2018: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29661342/can-focal-segmental-glomerulosclerosis-be-differentiated-from-minimal-change-nephrotic-syndrome-using-biomarkers
#9
EDITORIAL
Keiko Hosohata
No abstract text is available yet for this article.
April 2018: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29657220/complete-remission-of-nephrotic-syndrome-secondary-to-amyloid-a-amyloidosis-in-patient-with-inactive-crohn-s-disease-after-treatment-by-infliximab
#10
Kawtar Hassani, Mohamed Amine Hamzi, Driss El Kabbaj
Secondary amyloidosis Amyloid A (AA) is an infrequent but a severe complication of Crohn's disease (CD). This complication results from the activity of the underlying inflammation disease to form amyloid fibril deposits in tissues. We present a case of a 34-year-old female patient with CD treated by azathioprine with inactive disease for three years and who developed a nephrotic syndrome secondary to AA amyloidosis. The treatment by infliximab for one year leads to a complete remission of the nephrotic syndrome...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29657219/paraneoplastic-glomerulopathies-associated-with-hematologic-malignancies
#11
Jeyachandran Dhanapriya, Thanigachalam Dineshkumar, Ramanathan Sakthirajan, Dhanikachalam Surendar, Natarajan Gopalakrishnan, T Balasubramaniyan
Paraneoplastic glomerulopathy (PG) includes a group of glomerular lesions that are not directly related to tumor burden, invasion, or metastasis, but induced by products from tumor cells such as hormones, growth factors, cytokines, and tumor antigens. PG was first described by Galloway in 1922, in which nephrotic syndrome (NS) secondary to Hodgkin's lymphoma was reported. They can present before, at the time of diagnosis, during remission, or at relapse of the hematological malignancies. We present three cases of paraneoplastic NS associated with hematological malignancies...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29657215/acute-gastric-dilatation-in-a-patient-with-lupus-nephritis-an-uncommon-presentation-of-lupus-mesenteric-vasculitis
#12
S Ezhilnilavan, P S Priyamvada, S Haridasan, N G Rajesh, S Parameswaran
Abdominal symptoms are common in patients with lupus nephritis and are often attributed to drugs or uremia per se. Lupus mesenteric vasculitis (LMV) or lupus enteritis is a rare entity reported in patients with active systemic lupus erythematosus. It usually occurs in patients with a long-standing history of lupus with high disease activity. Usually, small bowel is predominantly affected. The stomach and rectum are spared in view of significant collateral circulation. Here, we describe an 18-year-old boy who presented with nephrotic syndrome without any extrarenal features of lupus...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29657209/spectrum-of-biopsy-proven-renal-diseases-a-single-center-experience
#13
Lakshminarayana R Gopaliah, Indu Sudakaran, Seethalekshmy Vijayan Nalumakkal, Ranjit Narayanan, Biju Meckattuparamban Vareed
Kidney biopsy is one of the most important tools in the assessment of kidney disease as histopathological diagnosis promotes evidence-based practice in Nephrology. This study included 271 consecutive percutaneous kidney biopsies (145 males and 126 females) performed at EMS Memorial Cooperative Hospital, Perinthalmanna, Kerala, India, from September 2009 to March 2016. Among the biopsy-proven renal diseases (BPRD), primary glomerular diseases (PGD) were the most common (77.78%) followed by secondary glomerular diseases (SGD) (12...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29657208/renal-amyloidosis-in-ankylosing-spondylitis-a-monocentric-study-and-review-of-literature
#14
Samia Barbouch, Meriam Hajji, Fatima Jaziri, Raja Aoudia, Eya Fellah, Hafedh Hedri, Rym Goucha, Fethi Ben Hamida, Fethi Ben Taarit, Imen Gorsane, Taieb Ben Abdallah
Secondary renal amyloidosis (RA) is the most common type of renal involvement in ankylosing spondylitis (AS). We assessed the epidemiologic and clinico-biological profile of AS patients with RA, to analyze treatment modalities and prognostic aspects, and to determine predictive factors of RA during AS. This was a retrospective study including 13 cases of RA among 212 cases who presented with AS, during the period from 1978 to 2006. The median age of the patients at the time of diagnosing AS was 47 years (range: 19-67)...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29657195/evaluation-of-serum-soluble-urokinase-plasminogen-activator-receptor-as-a-marker-for-steroid-responsiveness-in-children-with-primary-nephrotic-syndrome
#15
Suzan O Mousa, Salah M Saleh, Hend M Aly, Maha H Amin
Nephrotic syndrome (NS) is the most common primary glomerular disease among children. It runs a relapsing course involving prolonged periods of treatment with corticosteroids and other immunosuppressive medications. Soluble urokinase plasminogen activator receptor (suPAR) has been regarded as an inflammatory as well as a permeability factor. The aim of our study was to evaluate serum suPAR levels in children with NS and its relation to steroid responsiveness. Our study was carried out on 75 children who were already diagnosed as having NS; they were classified into three groups (steroid sensitive, steroid dependent, and steroid resistant)...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29655809/acute-limb-ischemia-in-an-8-year-old-patient-case-report
#16
Young Erben, Samuel M Miller, Brandon J Sumpio, Brian J Dillon, Alfred I Lee, Peter Blume, Bauer E Sumpio, Carlos Mena-Hurtado
We report the case of an 8-year-old patient with a history of nephrotic syndrome (NS), who presented to the emergency department (ED) with right foot pain. The patient's mother described intermittent pain that woke her son from sleep and was accompanied by the foot turning purple and becoming cold to touch. Physical exam revealed capillary refill of over ten seconds in the right and less than two seconds in the left foot. Ankle-brachial indices (ABI) were 0.0 on the right and 0.96 on the left. The patient was admitted and started on therapeutic intravenous heparin...
April 12, 2018: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/29650267/virtual-touch-tissue-quantification-for-assessing-renal-pathology-in-idiopathic-nephrotic-syndrome
#17
Xue Yang, Ning Yu, Jing Yu, Hongqiao Wang, Xiumei Li
The aim of this study was to evaluate Virtual Touch tissue quantification in assessing renal interstitial fibrosis in patients with idiopathic nephrotic syndrome. Ninety patients with idiopathic nephrotic syndrome were assigned to mild, moderate and severe groups depending on the degree of renal interstitial fibrosis on histopathologic examination of renal biopsy specimens. Thirty healthy patients were also selected as the control group. Virtual Touch tissue quantification was performed to measure the shear wave velocity of the renal parenchyma...
April 9, 2018: Ultrasound in Medicine & Biology
https://www.readbyqxmd.com/read/29644498/the-status-quo-and-challenges-of-genetic-diagnosis-in-children-with-steroid-resistant-nephrotic-syndrome
#18
EDITORIAL
Yan-Yan Jin, Bing-Yu Feng, Jian-Hua Mao
No abstract text is available yet for this article.
April 11, 2018: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/29644057/clinical-and-pathological-phenotype-of-genetic-causes-of-focal-segmental-glomerulosclerosis-in-adults
#19
Nicola Lepori, Ladan Zand, Sanjeev Sethi, Gema Fernandez-Juarez, Fernando C Fervenza
Focal segmental glomerulosclerosis (FSGS) is a histologic lesion resulting from a variety of pathogenic processes that cause injury to the podocytes. Recently, mutations in more than 50 genes expressed in podocyte or glomerular basement membrane were identified as causing genetic forms of FSGS, the majority of which are characterized by onset in childhood. The prevalence of adult-onset genetic FSGS is likely to be underestimated and its clinical and histological features have not been clearly described. A small number of studies of adult-onset genetic FSGS showed that there is heterogeneity in clinical and histological findings, with a presentation ranging from sub-nephrotic proteinuria to full nephrotic syndrome...
April 2018: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29622068/-renal-venous-thrombosis-as-a-complication-of-nephrotic-syndrome
#20
Nata Kermanian, Kaj Aage Hansen
Venous thromboembolism is a very common complication of nephrotic syndrome (NS). This case report concerns a 19-year-old male, who had known NS and was admitted two times in a short period, first presenting with recurrent symptoms of NS and later with an acute abdomen, which was diagnosed as renal venous thrombosis (RVT) and pulmonary thromboembolism. The patient was treated with prednisolone and therapeutic doses of dalteparin and warfarin. Venous thrombosis, particularly RVT and pulmonary thromboembolism, should be considered in patients with NS...
April 2, 2018: Ugeskrift for Laeger
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