Impaired phosphorylation of JAK2-STAT5b signaling in fibroblasts from uremic children.
Pediatric Nephrology 31(6):965 (2016)
Chronic kidney disease (CKD) in children is characterized by severe growth failure. The growth hormone/insulin-like growth factor-1 (GH/IGF-1) axis in uremic animals shows a post-receptor impaired phosphorylation of Janus kinase 2/signal transducer and activator of transcription (JAK-STAT) prote...
Pathogenesis of minimal change nephrotic syndrome: an immunological concept.
Korean Journal of Pediatrics 59(5):205 (2016)
Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia. Minimal change nephrotic syndrome (MCNS) is the most common form of INS in children. The pathogenesis of MCNS still remains unclear, however, several hypotheses have been recently propose...
Nephrin Contributes to Insulin Secretion and Affects Mammalian Target of Rapamycin Signaling Independently of Insulin Receptor.
Journal of the American Society of Nephrology 27(4):1029 (2016)
Nephrin belongs to a family of highly conserved proteins with a well characterized function as modulators of cell adhesion and guidance, and nephrin may have a role in metabolic pathways linked to podocyte and pancreatic β-cell survival. However, this role is incompletely characterized. In this ...
Infection-Related Focal Segmental Glomerulosclerosis in Children.
BioMed Research International 2016:7351964 (2016)
Focal segmental glomerulosclerosis (FSGS) is the most common cause of steroid resistant nephrotic syndrome in children. It describes a unique histological picture of glomerular damage resulting from several causes. In the majority of patients the causing agent is still unknown, but in some cases...
Immunologic Changes Implicated in the Pathogenesis of Focal Segmental Glomerulosclerosis.
BioMed Research International 2016:2150451 (2016)
Focal segmental glomerulosclerosis is a histological pattern on renal biopsy caused by diverse mechanisms. In its primary form, a circulatory factor is implicated in disease onset and recurrence. The natural history of primary FSGS is unpredictable, since some patients are unresponsive towards i...
Focal Segmental Glomerulosclerosis: Genetics, Mechanism, and Therapies.
BioMed Research International 2016:9643785 (2016)
Response to cyclosporine in steroid-resistant nephrotic syndrome: discontinuation is possible.
Pediatric Nephrology 30(9):1477 (2015)
Steroid-resistant nephrotic syndrome (SRNS) is still regarded as a serious disease although treatment with cyclosporine (CSA) has improved outcome. However, the duration of treatment in responders is unclear, and treatment of patients with genetic causes is a matter of debate.
Clinical and molecular characterization of patients with heterozygous mutations in wilms tumor suppressor gene 1.
Clinical Journal of the American Society of Nep... 10(5):825 (2015)
The Wilms tumor suppressor gene 1 (WT1) plays an essential role in urogenital and kidney development. Genotype/phenotype correlations of WT1 mutations with renal function and proteinuria have been observed in world-wide cohorts with nephrotic syndrome or Wilms tumor (WT). This study analyzed mid...
Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort.
Clinical Journal of the American Society of Nep... 10(4):592 (2015)
Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function. The rare nature, heterogeneity, and slow evolution of the disorder are major obstacles to systematic genotype-phenotype, intervention, and out...
Successful detoxification and liver transplantation in a severe poisoning with a chemical wood preservative containing chromium, copper, and arsenic.
Transplantation 99(4):e29 (2015)
Alterations in the ubiquitin proteasome system in persistent but not reversible proteinuric diseases.
Journal of the American Society of Nephrology 25(11):2511 (2014)
Podocytes are the key cells affected in nephrotic glomerular kidney diseases, and they respond uniformly to injury with cytoskeletal rearrangement. In nephrotic diseases, such as membranous nephropathy and FSGS, persistent injury often leads to irreversible structural damage, whereas in minimal ...
Gender differences in solitary functioning kidney: do they affect renal outcome? Response.
Pediatric Nephrology 29(11):2245 (2014)
Co-incident BK and Epstein-Barr virus replication in a 3-year-old immunocompetent boy.
Clinical Nephrology 82(4):278 (2014)
We present a unique case of infectious mononucleosis attended with transient asymptomatic BK virus (BKV) manifestation in the urine of an immunocompetent caucasian boy without kidney dysfunction. The urine sediment showed abundant decoy cells initially misdiagnosed as malignant cancer cells. Thi...
Neurological involvement in children with E. coli O104:H4-induced hemolytic uremic syndrome.
Pediatric Nephrology 29(9):1607 (2014)
The aim of this study was to analyze the neurological involvement and outcome in pediatric patients with hemolytic uremic syndrome (HUS) during the 2011 epidemic caused by Escherichia coli O104:H4.
Clinical data and data from magnetic resonance imaging (MRI) scans and electroencephalography (EEG...
Myeloperoxidase deficiency ameliorates progression of chronic kidney disease in mice.
American Journal of Physiology: Renal, Fluid & ... 307(4):F407 (2014)
Myeloperoxidase (MPO) is an enzyme expressed in neutrophils and monocytes/macrophages. Beside its well-defined role in innate immune defence, it may also be responsible for tissue damage. To identify the role of MPO in the progression of chronic kidney disease (CKD), we investigated CKD in a mod...
Is rituximab effective in childhood nephrotic syndrome? Yes and no.
Pediatric Nephrology 29(8):1305 (2014)
The idiopathic nephrotic syndrome (i.e. MCNS and FSGS) in children has been regarded as a disorder of T-cell function. Recent studies, however, also describe abnormalities of B-cell function. This supports the use of B-cell modulating treatment for idiopathic nephrotic syndrome (INS), especially...
Protection of human podocytes from shiga toxin 2-induced phosphorylation of mitogen-activated protein kinases and apoptosis by human serum amyloid P component.
Infection and Immunity 82(5):1872 (2014)
Hemolytic uremic syndrome (HUS) is mainly induced by Shiga toxin 2 (Stx2)-producing Escherichia coli. Proteinuria can occur in the early phase of the disease, and its persistence determines the renal prognosis. Stx2 may injure podocytes and induce proteinuria. Human serum amyloid P component (SA...
Abatacept in B7-1-positive proteinuric kidney disease.
New England Journal of Medicine 369(25):2416 (2013)
Abatacept (cytotoxic T-lymphocyte-associated antigen 4-immunoglobulin fusion protein [CTLA-4-Ig]) is a costimulatory inhibitor that targets B7-1 (CD80). The present report describes five patients who had focal segmental glomerulosclerosis (FSGS) (four with recurrent FSGS after transplantation an...
Protective effects of the mTOR inhibitor everolimus on cytoskeletal injury in human podocytes are mediated by RhoA signaling.
PLoS ONE 8(2):e55980 (2013)
Podocytes are highly differentiated kidney cells playing an important role in maintaining the glomerular filtration barrier. Particularly, the integrity of the actin cytoskeleton is crucial as cytoskeletal damage associated with foot process effacement and loss of slit diaphragms constitutes a m...
The lectin-like domain of thrombomodulin ameliorates diabetic glomerulopathy via complement inhibition.
Thrombosis and Haemostasis 108(6):1141 (2012)
Coagulation and complement regulators belong to two interactive systems constituting emerging mechanisms of diabetic nephropathy. Thrombomodulin (TM) regulates both coagulation and complement activation, in part through discrete domains. TM's lectin like domain dampens complement activation, whi...