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https://www.readbyqxmd.com/read/29775447/-mycophenolate-mofetil-mmf-as-the-first-choice-immunosuppressive-drug-in-treatment-of-steroid-dependent-nephrotic-syndrome-in-children
#1
Iwona Ogarek, Elżbieta Szczęsny-Choruz, Ewa Wierzchowska-Słowiaczek, Joanna Kwinta-Rybicka, Zofia Stec, Anna Moczulska, Katarzyna Wilkosz, Dorota Drożdż
Most children diagnosed with nephrotic syndrome show favourable response to corticosteroid therapy, nonetheless 30% of patients have frequent relapses or a steroid-dependent course of disease. Cyclophosphamide, cyclosporin A or MMF are being used in treatment of steroid-dependent nephrotic syndrome in search of a drug with highest long-term effectiveness and least amount of side effects. AIM: The aim of study was to assess of the efficacy of MMF as the first choice immunosuppressive drug in children with nephrotic syndrome after determining a steroid-dependency...
April 23, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29775445/the-clinical-pattern-of-nephrotic-syndrome-in-children-has-no-effect-on-the-concentration-of-soluble-urokinase-receptor-supar-in-serum-and-urine
#2
Agnieszka Ochocińska, Wioletta Jarmużek, Roman Janas
Concentration of soluble urokinase receptor (suPAR) was regarded as viable marker to differentiate the focal segmental glomerulosclerosis (FSGS) from other glomerulopathies and also as predictive parameter for progression of renal disease. AIM: The aim of this study was to evaluate serum and urine (s)(u)suPAR concentration in steroid-sensitive and steroid-resistant nephrotic children treated with different (double and triple-drug) regimens. MATERIALS AND METHODS: Overall 43 children were evaluated including 14 patients with steroid-resistant nephrotic syndrome (SRNS) aged 9±6 years and 29 with steroid-sensitive nephrotic syndrome (SSNS) aged 9±5 years, as well as control group (n=59)...
April 23, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29773874/mutations-in-six-nephrosis-genes-delineate-a-pathogenic-pathway-amenable-to-treatment
#3
Shazia Ashraf, Hiroki Kudo, Jia Rao, Atsuo Kikuchi, Eugen Widmeier, Jennifer A Lawson, Weizhen Tan, Tobias Hermle, Jillian K Warejko, Shirlee Shril, Merlin Airik, Tilman Jobst-Schwan, Svjetlana Lovric, Daniela A Braun, Heon Yung Gee, David Schapiro, Amar J Majmundar, Carolin E Sadowski, Werner L Pabst, Ankana Daga, Amelie T van der Ven, Johanna M Schmidt, Boon Chuan Low, Anjali Bansal Gupta, Brajendra K Tripathi, Jenny Wong, Kirk Campbell, Kay Metcalfe, Denny Schanze, Tetsuya Niihori, Hiroshi Kaito, Kandai Nozu, Hiroyasu Tsukaguchi, Ryojiro Tanaka, Kiyoshi Hamahira, Yasuko Kobayashi, Takumi Takizawa, Ryo Funayama, Keiko Nakayama, Yoko Aoki, Naonori Kumagai, Kazumoto Iijima, Henry Fehrenbach, Jameela A Kari, Sherif El Desoky, Sawsan Jalalah, Radovan Bogdanovic, Nataša Stajić, Hildegard Zappel, Assel Rakhmetova, Sharon-Rose Wassmer, Therese Jungraithmayr, Juergen Strehlau, Aravind Selvin Kumar, Arvind Bagga, Neveen A Soliman, Shrikant M Mane, Lewis Kaufman, Douglas R Lowy, Mohamad A Jairajpuri, Richard P Lifton, York Pei, Martin Zenker, Shigeo Kure, Friedhelm Hildebrandt
No efficient treatment exists for nephrotic syndrome (NS), a frequent cause of chronic kidney disease. Here we show mutations in six different genes (MAGI2, TNS2, DLC1, CDK20, ITSN1, ITSN2) as causing NS in 17 families with partially treatment-sensitive NS (pTSNS). These proteins interact and we delineate their roles in Rho-like small GTPase (RLSG) activity, and demonstrate deficiency for mutants of pTSNS patients. We find that CDK20 regulates DLC1. Knockdown of MAGI2, DLC1, or CDK20 in cultured podocytes reduces migration rate...
May 17, 2018: Nature Communications
https://www.readbyqxmd.com/read/29768408/a-homozygous-kat2b-variant-modulates-the-clinical-phenotype-of-add3-deficiency-in-humans-and-flies
#4
Sara Gonçalves, Julie Patat, Maria Clara Guida, Noelle Lachaussée, Christelle Arrondel, Martin Helmstädter, Olivia Boyer, Olivier Gribouval, Marie-Claire Gubler, Geraldine Mollet, Marlène Rio, Marina Charbit, Christine Bole-Feysot, Patrick Nitschke, Tobias B Huber, Patricia G Wheeler, Devon Haynes, Jane Juusola, Thierry Billette de Villemeur, Caroline Nava, Alexandra Afenjar, Boris Keren, Rolf Bodmer, Corinne Antignac, Matias Simons
Recent evidence suggests that the presence of more than one pathogenic mutation in a single patient is more common than previously anticipated. One of the challenges hereby is to dissect the contribution of each gene mutation, for which animal models such as Drosophila can provide a valuable aid. Here, we identified three families with mutations in ADD3, encoding for adducin-γ, with intellectual disability, microcephaly, cataracts and skeletal defects. In one of the families with additional cardiomyopathy and steroid-resistant nephrotic syndrome (SRNS), we found a homozygous variant in KAT2B, encoding the lysine acetyltransferase 2B, with impact on KAT2B protein levels in patient fibroblasts, suggesting that this second mutation might contribute to the increased disease spectrum...
May 16, 2018: PLoS Genetics
https://www.readbyqxmd.com/read/29750317/mycophenolate-mofetil-for-sustained-remission-in-nephrotic-syndrome
#5
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/29734156/socioeconomics-of-administering-rituximab-for-nephrotic-syndrome
#6
Tomoyuki Takura, Takashi Takei, Kosaku Nitta
Nephrotic syndrome is a type of intractable disease caused by a disorder in the kidneys, which produces swelling. Although some patients show rapid improvement and recover completely with conventional treatment, many others experience frequent recurrence (frequently relapsing nephrotic syndrome) while some remain dependent on the same high dose of steroids they were initially prescribed at the start of treatment (steroid-dependent nephrotic syndrome). In the latter cases, side effects of prolonged steroid use are a major issue...
2018: Contributions to Nephrology
https://www.readbyqxmd.com/read/29734146/rituximab-as-a-therapeutic-option-for-steroid-sensitive-minimal-change-nephrotic-syndrome-in-adults
#7
Yuko Iwabuchi, Takahito Moriyama, Mitsuyo Itabashi, Takashi Takei, Kosaku Nitta
Minimal change nephrotic syndrome (MCNS) usually responds to steroids but frequently relapses, requiring additional treatment with immunosuppressive agents. Rituximab is a chimeric murine/human monoclonal immunoglobulin G1 antibody that targets CD20, a B-cell differentiation marker. B-cell recovery begins at approximately 6 months following the completion of treatment. Rituximab has a beneficial effect, with the sustained remission or reduction of proteinuria in patients with steroid-dependent MCNS. Relapses are thought to be associated with an increase in CD19 cells...
2018: Contributions to Nephrology
https://www.readbyqxmd.com/read/29712589/management-of-intra-abdominal-biloma-in-the-setting-of-chronic-steroid-use-for-nephrotic-syndrome
#8
Gifty S Abraham, Joshua T Taylor, Marcus Tan, William O Richards
No abstract text is available yet for this article.
April 1, 2018: American Surgeon
https://www.readbyqxmd.com/read/29704630/knockdown-of-nup160-inhibits-cell-proliferation-induces-apoptosis-autophagy-and-cell-migration-and-alters-the-expression-and-localization-of-podocyte-associated-molecules-in-mouse-podocytes
#9
Ping Wang, Feng Zhao, Xiaojing Nie, Jiewei Liu, Zihua Yu
Genetic mutations in dozens of monogenic genes can lead to serious podocyte dysfunction, which is a major cause of steroid-resistant nephrotic syndrome (SRNS). The NUP160 gene is expressed in both human kidney and mouse kidney. However, whether knockdown of NUP160 impairs podocytes has not yet been established. Therefore, we knocked down NUP160 by targeted short hairpin RNA (shRNA) in conditionally immortalized mouse podocytes and observed the effect of NUP160 knockdown on the proliferation, apoptosis, autophagy and cell migration of podocytes...
April 25, 2018: Gene
https://www.readbyqxmd.com/read/29685115/acquired-modification-of-sphingosine-1-phosphate-lyase-activity-is-not-related-to-adrenal-insufficiency
#10
Gulin Sunter, Ece Oge Enver, Azad Akbarzade, Serap Turan, Pinar Vatansever, Dilek Ince Gunal, Goncagul Haklar, Abdullah Bereket, Kadriye Agan, Tulay Guran
BACKGROUND: Congenital sphingosine-1-phosphate (S1P) lyase deficiency due to biallelic mutations in SGPL1 gene has recently been described in association with primary adrenal insufficiency and steroid-resistant nephrotic syndrome. S1P lyase, on the other hand, is therapeutically inhibited by fingolimod which is an oral drug for relapsing multiple sclerosis (MS). Effects of this treatment on adrenal function has not yet been evaluated. We aimed to test adrenal function of MS patients receiving long-term fingolimod treatment...
April 23, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29680473/rituximab-in-the-management-of-pediatric-steroid-resistant-nephrotic-syndrome-a-systematic-review
#11
Manel Jellouli, Rim Charfi, Bayen Maalej, Abdelmajid Mahfoud, Sameh Trabelsi, Tahar Gargah
OBJECTIVES: To evaluate the efficacy and safety of rituximab in children with steroid-resistant nephrotic syndrome. STUDY DESIGN: A systematic review evaluating the efficacy and safety of rituximab in children with steroid-resistant nephrotic syndrome was performed. Data from studies, performed before April 2017 were collected, from MEDLINE, Cochrane Library, Scopus, and Web of Science. Study eligibility criteria included clinical trials and observational studies with a minimal sample size of 5 patients, regarding treatment with rituximab in children with steroid-resistant nephrotic syndrome...
April 18, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29668475/rare-renal-disease-in-macedonia-an-update
#12
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/29657195/evaluation-of-serum-soluble-urokinase-plasminogen-activator-receptor-as-a-marker-for-steroid-responsiveness-in-children-with-primary-nephrotic-syndrome
#13
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/29644498/the-status-quo-and-challenges-of-genetic-diagnosis-in-children-with-steroid-resistant-nephrotic-syndrome
#14
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/29614967/cardiovascular-outcomes-of-nephrotic-syndrome-in-childhood-cvons-study-a-protocol-for-prospective-cohort-study
#15
S K Patnaik, P Kumar, M Bamal, S Patel, M P Yadav, V Kumar, A Sinha, A Bagga, M Kanitkar
BACKGROUND: Nephrotic syndrome (NS) is characterized by dyslipidemia which is a well-known risk factor for atherogenesis. Atherosclerosis in childhood is mostly subclinical and endothelial dysfunction is known to precede this. Evidence for screening for endothelial dysfunction and cardiovascular risk factors and early identification of premature onset of atherosclerosis in childhood NS remains tenuous in the absence of well-designed prospective studies addressing cardiovascular comorbidity in NS...
April 3, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29594119/monogenic-causes-of-proteinuria-in-children
#16
REVIEW
Onur Cil, Farzana Perwad
Glomerular disease is a common cause for proteinuria and chronic kidney disease leading to end-stage renal disease requiring dialysis or kidney transplantation in children. Nephrotic syndrome in children is diagnosed by the presence of a triad of proteinuria, hypoalbuminemia, and edema. Minimal change disease is the most common histopathological finding in children and adolescents with nephrotic syndrome. Focal segmental sclerosis is also found in children and is the most common pathological finding in patients with monogenic causes of nephrotic syndrome...
2018: Frontiers in Medicine
https://www.readbyqxmd.com/read/29577510/successful-treatment-with-steroid-and-cyclosporine-a-in-a-patient-with-immunoglobulin-a-proliferative-glomerulonephritis-with-monoclonal-immunoglobulin-deposits
#17
Keisuke Sato, Shiho Makabe, Yuko Iwabuchi, Kaori Kojima, Masayo Sato, Hiroshi Kataoka, Takahito Moriyama, Sekiko Taneda, Ken Tsuchiya, Kosaku Nitta, Toshio Mochizuki
We report a case of glomerulonephritis with monoclonal immunoglobulin (Ig) A deposits as a form of monoclonal gammopathy of renal significance (MGRS) caused by monoclonal immunoglobulins without blood disorders in a 41-year-old woman. She developed lower leg oedema and was hospitalized because of nephrotic syndrome. Serum and urine were negative for M protein, and the free light chain κ/λ ratio was within the normal range. Renal histopathological findings included mesangial proliferation, endocapillary cell proliferation, and a double-contour appearance of the capillary walls...
March 25, 2018: Nephrology
https://www.readbyqxmd.com/read/29569812/ofatumumab-in-post-transplantation-recurrence-of-a-pediatric-steroid-resistant-idiopathic-nephrotic-syndrome
#18
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/29569147/hypertension-and-its-severity-in-children-with-steroid-sensitive-nephrotic-syndrome-during-remission
#19
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/29565146/stunting-wasting-and-mid-upper-arm-circumference-status-among-children-admitted-to-nemazee-teaching-hospital
#20
Seyed Mohsen Dehghani, Hazhir Javaherizadeh, Masoomeh Heidary, Naser Honar, Maryam Ataollahi, Homa Ilkanipour, Hossein Moravej
INTRODUCTION AND AIM: The aim of this study was to evaluate nutritional status in children without prior hospital admission or evidence of chronic disease. SUBJECTS AND METHODS: The current study is a cross-sectional and observational study which was conducted for assessing the nutritional status of children. In this study, consecutive sampling was used, with a sample size about 400 children aged 6 months to 18 years at first hospital admission. All subjects were hospitalized consecutively in the Pediatric Emergency Department of the Nemazee Teaching Hospital of Shiraz University of Medical Sciences (Shiraz, Islamic Republic of Iran)...
January 10, 2018: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
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