Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Idiopathic nephrotic syndrome in children may be complicated by resistance to steroids with constant proteinuria in diffuse mesangial proliferation (DMP) and focal segmental glomerulosclerosis (FSGS). In our observation, sometimes in children with steroid-resistant nephrotic syndrome, the presence of immature renal glomeruli can be detected (hypercellularity and presence of a constant layer of cubical epithelial cells on the surface of glomerular tufts, without sclerosis, resembling M-stage of glomerulo-genesis). The aim of this study was immunohistochemical analysis of the podo-cyte-associated proteins, particularly ezrin, podocalyxin, synaptopodin and nephrin in glomeruli with and without signs of immaturity in children. In DMP with signs of immaturity podo-cytes situated in the central region of the glomerulus were immunohistochemically negative. The positive reaction was observed exclusively in the most superficial continuous 'layer' of podo-cytes. The unfavourable clinical course of nephrotic syndrome with signs of glomerular immaturity may be a consequence of decreased immunohistochemical expression of cytoskeleton-specific proteins.
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PMID:[Immunohistochemical analysis of podocytopathy with immature glomeruli and glomerulosclerosis in children with nephrotic syndrome]. 1689 1

Our aim was to correlate an immunohistochemical pattern of selected podocyte cytoskeleton-associated proteins in children diagnosed with focal segmental glomerulosclerosis (FSGS) and diffuse mesangial proliferation accompanied by glomerular immaturity (Im-DMP) with the clinical courses of both diseases. The material included 33 renal biopsies obtained from children diagnosed with DMP with or without signs of glomerular immaturity (ten and 15 participants, respectively) or FSGS (eight patients). Ezrin, podocalyxin, synaptopodin and nephrin expression was evaluated by immunohistochemical assay. A positive reaction for ezrin, podocalyxin, synaptopodin and nephrin was observed in the most superficial, continuous 'layer' of podocytes in Im-DMP patients. This distribution closely mimicked the immunohistochemical pattern observed in FSGS. The severe initial course of Im-DMP was transient. Resistance to steroids (six children) and renal insufficiency (two patients) in these subjects subsided, whilst, in the FSGS patients, the resistance to steroids recognized in all the children and the renal insufficiency diagnosed in three of them were still present. Mimicry between the immunohistochemical pattern of glomerular immaturity in DMP and focal segmental glomerulosclerosis might explain the severe initial course of this nephrotic syndrome in children. The transient clinical character of the former may also indicate that it is not a variant of FSGS.
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PMID:Is mesangial hypercellularity with glomerular immaturity a variant of glomerulosclerosis? 1723 49