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10.1182/blood-2016-08-697110 http://scihub22266oqcxt.onion/10.1182/blood-2016-08-697110 C5345731!5345731!28057638     free     free     free Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       Blood 2017 ; 129 (10): 1251-9 Nephropedia Template TP {{tp|p=28057638|t=2017. An international registry of survivors with Hb Bart s hydrops fetalis syndrome |pdf=|usr=}}{{28057638}} gab.com Text An international registry of survivors with Hb Bart s hydrops fetalis syndrome JO: Blood http://www.kidney.de/pget.php?&tw=1&pmid=28057638 #FOAvip Hemoglobin (Hb) Bart's hydrops fetalis syndrome (BHFS) resulting from ?0-thalassemia is considered a universally fatal disorder. However, over the last 3 decades, improvements in intrauterine interventions and perinatal intensive care have resulted in increasing numbers of BHFS survivors. We have initiated an international registry containing information on 69 patients, of which 31 are previously unpublished. In this perspective, we analyze the available clinical information to document the natural history of BHFS. In the future, once we have accrued sufficient cases, we aim to build on this study and provide information to allow counseling of at-risk couples. To date, 39 patients have survived beyond the age of 5 years, 18 of whom are now older than 10 years. Based on the available cases, we find evidence to suggest that intrauterine therapy provides benefits during the perinatal and neonatal period; however, it may not provide additional benefits to long-term growth and neurodevelopmental outcomes. Growth retardation is a major adverse long-term outcome among BHFS patients with ?40% being severely affected in terms of weight and ?50% in terms of height. There is also an increased risk of neurodevelopmental delay as we find 20% (11/55) of BHFS survivors suffer from a serious delay of ?6 months. Most patients in the registry require lifelong transfusion and often have associated congenital abnormalities and comorbidities. This perspective is a first step in gathering information to allow provision of informed counseling on the predicted outcomes of affected babies. Twit Text FOAVip An international registry of survivors with Hb Bart s hydrops fetalis syndrome ????Blood http://www.kidney.de/pget.php?&tw=1&pmid=28057638 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=28057638 #FOAvip English Wikipedia <ref>{{Cite pmid|28057638}}</ref> An international registry of survivors with Hb Bart s hydrops fetalis syndrome #MMPMID28057638 Songdej D; Babbs C; Higgs DR Blood 2017[Mar]; 129 (10): 1251-9 PMID28057638show ga Hemoglobin (Hb) Bart's hydrops fetalis syndrome (BHFS) resulting from ?0-thalassemia is considered a universally fatal disorder. However, over the last 3 decades, improvements in intrauterine interventions and perinatal intensive care have resulted in increasing numbers of BHFS survivors. We have initiated an international registry containing information on 69 patients, of which 31 are previously unpublished. In this perspective, we analyze the available clinical information to document the natural history of BHFS. In the future, once we have accrued sufficient cases, we aim to build on this study and provide information to allow counseling of at-risk couples. To date, 39 patients have survived beyond the age of 5 years, 18 of whom are now older than 10 years. Based on the available cases, we find evidence to suggest that intrauterine therapy provides benefits during the perinatal and neonatal period; however, it may not provide additional benefits to long-term growth and neurodevelopmental outcomes. Growth retardation is a major adverse long-term outcome among BHFS patients with ?40% being severely affected in terms of weight and ?50% in terms of height. There is also an increased risk of neurodevelopmental delay as we find 20% (11/55) of BHFS survivors suffer from a serious delay of ?6 months. Most patients in the registry require lifelong transfusion and often have associated congenital abnormalities and comorbidities. This perspective is a first step in gathering information to allow provision of informed counseling on the predicted outcomes of affected babies. äAn international registry of survivors with Hb Bart s hydrops fetalis (epmc).pdf DeepDyve Pubget Overpricing