Yayıncı "Amer Soc Nephrology" Dahili Tıp Bilimleri Bölümü için listeleme
Toplam kayıt 16, listelenen: 1-16
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ADCK4-Associated Glomerulopathy Causes Adolescence-Onset FSGS
(Amer Soc Nephrology, 2016)Hereditary defects of coenzyme Q(10) biosynthesis cause steroid-resistant nephrotic syndrome (SRNS) as part of multiorgan involvement but may also contribute to isolated SRNS. Here, we report 26 patients from 12 families ... -
Circulating Supar In Two Cohorts Of Primary Fsgs
(Amer Soc Nephrology, 2012)Overexpression of soluble urokinase receptor (suPAR) causes pathology in animal models similar to primary FSGS, and one recent study demonstrated elevated levels of serum suPAR in patients with the disease. Here, we analyzed ... -
Dgke Variants Cause A Glomerular Microangiopathy That Mimics Membranoproliferative Gn
(Amer Soc Nephrology, 2013)Renal microangiopathies and membranoproliferative GN (MPGN) can manifest similar clinical presentations and histology, suggesting the possibility of a common underlying mechanism in some cases. Here, we performed homozygosity ... -
Genotype/Phenotype Correlation In Nephrotic Syndrome Caused By Wt1 Mutations
(Amer Soc Nephrology, 2010)Background and objectives: The risk of developing Wilms tumor (WT) can be present or absent in patients with nephrotic syndrome (NS) caused by WT1 mutations. Here, the genotype/phenotype correlation regarding the outcome ... -
Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children
(Amer Soc Nephrology, 2017)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, ... -
Management of Anemia in Children Receiving Chronic Peritoneal Dialysis
(Amer Soc Nephrology, 2013)Little information exists regarding the efficacy, modifiers, and outcomes of anemia management in children with CKD or ESRD. We assessed practices, effectors, and outcomes of anemia management in 1394 pediatric patients ... -
Mutations In Anks6 Cause A Nephronophthisis-Like Phenotype With Esrd
(Amer Soc Nephrology, 2014)Nephronophthisis (NPHP) is one of the most common genetic causes of CKD; however, the underlying genetic abnormalities have been established in <50% of patients. We performed genome-wide analysis followed by targeted ... -
Normal 25-Hydroxyvitamin D Levels Are Associated With Less Proteinuria And Attenuate Renal Failure Progression In Children With Ckd
(Amer Soc Nephrology, 2016)Angiotensin-converting enzyme inhibitors (ACEi) for renin-angiotensin-aldosterone system (RAAS) blockade are routinely used to slow CKD progression. However, vitamin D may also promote renoprotection by suppressing renin ... -
Patients with Mutations in Nphs2 (Podocin) Do not Respond To Standard Steroid Treatment of Nephrotic Syndrome
(Amer Soc Nephrology, 2004)Nephrotic syndrome (NS) represents the association of proteinuria, hypoalbuminemia, edema, and hyperlipidemia. Steroid-resistant NS (SRNS) is defined by primary resistance to standard steroid therapy. It remains one of the ... -
Peritonitis in Children Who Receive Long-Term Peritoneal Dialysis: A Prospective Evaluation of Therapeutic Guidelines
(Amer Soc Nephrology, 2007)In children who are on chronic peritoneal dialysis, peritonitis is the primary complication compromising technique survival, and the optimal therapy of peritonitis remains uncertain. An Internet-based International Pediatric ... -
Prevalence of Hypertension in Children with Early-Stage Adpkd
(Amer Soc Nephrology, 2018)Background and objectives Autosomal dominant polycystic kidney disease is the most common inheritable kidney disease, frequently thought to become symptomatic in adulthood. However, patients with autosomal dominant polycystic ... -
Six2 and Bmp4 Mutations Associate with Anomalous Kidney Development
(Amer Soc Nephrology, 2008)Renal hypodysplasia (RHD) is characterized by reduced kidney size and/or maldevelopment of the renal tissue following abnormal organogenesis. Mutations in renal developmental genes have been identified in a subset of ... -
Specific Podocin Mutations Correlate With Age Of Onset In Steroid-Resistant Nephrotic Syndrome
(Amer Soc Nephrology, 2008)Mutations in the gene encoding podocin (NPHS2) cause autosomal recessive steroid-resistant nephrotic syndrome (SRNS). For addressing the possibility of a genotype-phenotype correlation between podocin mutations and age of ... -
The Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C) Study: Objectives, Design, and Methodology
(Amer Soc Nephrology, 2010)Background and objectives: Children and adolescents with chronic kidney disease (CKD) are at high risk for cardiovascular morbidity and mortality. A systemic arteriopathy and cardiomyopathy has been characterized in pediatric ... -
The Clinical and Mutational Spectrum of Turkish Patients with Cystinosis
(Amer Soc Nephrology, 2017)Background and objectives Infantile nephropathic cystinosis is a severe disease that occurs due to mutations in the cystinosis gene, and it is characterized by progressive dysfunction of multiple organs; >100 cystinosis ... -
The Position Of The Polycystic Kidney Disease 1 (Pkd1) Gene Mutation Correlates With The Severity Of Renal Disease
(Amer Soc Nephrology, 2002)The severity of renal cystic disease in the major form of autosomal dominant polycystic kidney disease (PKD1) is highly variable. Clinical data was analyzed from 324 mutation-characterized PKD1 patients (80 families) to ...
