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10.1007/s00467-007-0633-9 http://scihub22266oqcxt.onion/10.1007/s00467-007-0633-9 17968594!2753773!17968594     free     free     free       Pediatr+Nephrol 2009 ; 24 (11): 2121-8 Nephropedia Template TP {{tp|p=17968594|t=2009. Congenital nephrotic syndrome |pdf=|usr=}}{{17968594}} gab.com Text Congenital nephrotic syndrome JO: Pediatr Nephrol http://www.kidney.de/pget.php?&tw=1&pmid=17968594 #FOAvip Congenital nephrotic syndrome (CNS) is a rare kidney disorder characterized by heavy proteinuria, hypoproteinemia, and edema starting soon after birth. The majority of cases are caused by genetic defects in the components of the glomerular filtration barrier, especially nephrin and podocin. CNS may also be a part of a more generalized syndrome or caused by a perinatal infection. Immunosuppressive medication is not helpful in the genetic forms of CNS, and kidney transplantation is the only curative therapy. Before the operation, management of these infants largely depends on the magnitude of proteinuria. In severe cases, daily albumin infusions are required to prevent life-threatening edema. The therapy also includes hypercaloric diet, thyroxin and mineral substitution, prevention of thrombotic episodes, and prompt management of infectious complications. The outcome of CNS patients without major extrarenal manifestations is comparable with other patient groups after kidney transplantation. Twit Text FOAVip Congenital nephrotic syndrome ????Pediatr Nephrol http://www.kidney.de/pget.php?&tw=1&pmid=17968594 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=17968594 #FOAvip English Wikipedia <ref>{{Cite pmid|17968594}}</ref> Congenital nephrotic syndrome #MMPMID17968594 Jalanko H Pediatr Nephrol 2009[Nov]; 24 (11): 2121-8 PMID17968594show ga Congenital nephrotic syndrome (CNS) is a rare kidney disorder characterized by heavy proteinuria, hypoproteinemia, and edema starting soon after birth. The majority of cases are caused by genetic defects in the components of the glomerular filtration barrier, especially nephrin and podocin. CNS may also be a part of a more generalized syndrome or caused by a perinatal infection. Immunosuppressive medication is not helpful in the genetic forms of CNS, and kidney transplantation is the only curative therapy. Before the operation, management of these infants largely depends on the magnitude of proteinuria. In severe cases, daily albumin infusions are required to prevent life-threatening edema. The therapy also includes hypercaloric diet, thyroxin and mineral substitution, prevention of thrombotic episodes, and prompt management of infectious complications. The outcome of CNS patients without major extrarenal manifestations is comparable with other patient groups after kidney transplantation. |Humans[MESH] |Intracellular Signaling Peptides and Proteins/genetics[MESH] |Kidney Transplantation/*adverse effects[MESH] |Membrane Proteins/genetics[MESH] |Nephrotic Syndrome/*congenital[MESH]Congenital nephrotic syndrome (epmc).pdf DeepDyve Pubget Overpricing