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10.1007/8904_2016_562 http://scihub22266oqcxt.onion/10.1007/8904_2016_562 C5362560!5362560!27334896     free     free     free Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       JIMD+Rep 2017 ; 32 (ä): 101-4 Nephropedia Template TP {{tp|p=27334896|t=2017. Rare Case of Hepatic Gaucheroma in a Child on Enzyme Replacement Therapy |pdf=|usr=}}{{27334896}} gab.com Text Rare Case of Hepatic Gaucheroma in a Child on Enzyme Replacement Therapy JO: JIMD Rep http://www.kidney.de/pget.php?&tw=1&pmid=27334896 #FOAvip Background: We present a 6 year old boy with type I Gaucher treated from 16 months with ERT, developing focal Gaucheroma in the liver at 3.5 years.Case: The subject presented at 13 months of age with anaemia, thrombocytopenia and hepatosplenomegaly. Gaucher disease was confirmed by leucocyte enzyme assay. A homozygous change: c.1193G>A (p.Arg398Gln) in the GBA gene was identified. He had normal neurology with normal saccades. Imiglucerase was administered at 60 IU/kg/fortnight from 15 months as per Australian regulations with good clinical response. At 3.5 years hepatic ultrasound demonstrated a nodular cystic lesion measuring 7?×?5.3?×?5.1 cm in the right lobe of liver, confirmed on MRI. Biopsy demonstrated acellular hyaline necrosis, portal?portal bridging fibrosis and nodules of Gaucher cells. Cystic fluid comprised necrotic debris and Gaucher cells. Further evaluation over 18 months including repeat MRI, biopsy, alpha-fetoprotein monitoring and whole-body FDG-Pet scan demonstrate no malignancy.Conclusion: GD is the most common lysosomal storage disorder. The aetiology, natural history and optimal management strategy of rare Gaucheroma in paediatric cases has not been defined particularly in regards to malignancy risk. Twit Text FOAVip Rare Case of Hepatic Gaucheroma in a Child on Enzyme Replacement Therapy ????JIMD Rep http://www.kidney.de/pget.php?&tw=1&pmid=27334896 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=27334896 #FOAvip English Wikipedia <ref>{{Cite pmid|27334896}}</ref> Rare Case of Hepatic Gaucheroma in a Child on Enzyme Replacement Therapy #MMPMID27334896 Korula S; Owens P; Charlton A; Bhattacharya K; Baumgartner MR; Patterson M; Rahman S; Peters V; Morava E; Zschocke J JIMD Rep 2017[]; 32 (ä): 101-4 PMID27334896show ga Background: We present a 6 year old boy with type I Gaucher treated from 16 months with ERT, developing focal Gaucheroma in the liver at 3.5 years.Case: The subject presented at 13 months of age with anaemia, thrombocytopenia and hepatosplenomegaly. Gaucher disease was confirmed by leucocyte enzyme assay. A homozygous change: c.1193G>A (p.Arg398Gln) in the GBA gene was identified. He had normal neurology with normal saccades. Imiglucerase was administered at 60 IU/kg/fortnight from 15 months as per Australian regulations with good clinical response. At 3.5 years hepatic ultrasound demonstrated a nodular cystic lesion measuring 7?×?5.3?×?5.1 cm in the right lobe of liver, confirmed on MRI. Biopsy demonstrated acellular hyaline necrosis, portal?portal bridging fibrosis and nodules of Gaucher cells. Cystic fluid comprised necrotic debris and Gaucher cells. Further evaluation over 18 months including repeat MRI, biopsy, alpha-fetoprotein monitoring and whole-body FDG-Pet scan demonstrate no malignancy.Conclusion: GD is the most common lysosomal storage disorder. The aetiology, natural history and optimal management strategy of rare Gaucheroma in paediatric cases has not been defined particularly in regards to malignancy risk. äRare Case of Hepatic Gaucheroma in a Child on Enzyme Replacement Thera(epmc).pdf DeepDyve Pubget Overpricing