Abstract:
:Methylmalonic acidemia with complete mutase deficiency (mut(0) type) is an inborn error of metabolism with high mortality and morbidity. LT has been suggested to be a solution to this disease, but elevation of urinary and blood MMA was still observed after LT. In this study, we measured dry blood spot MMA and its precursor propionyl-carnitine (C3-carnitine) for mut(0) patients. The results revealed that when C3-carnitine rose during metabolic stress, MMA rose exponentially (up to 1000 micromol/L) in patients who did not undergo LT. In patients who underwent LT, MMA rose to 100-200 micromol/L when C3-carnitine reached 10-20 micromol/L. However, when C3-carnitine rose further to 40-50 micromol/L, MMA levels just stayed put. Therefore, LT stabilized blood MMA level, though there might be a threshold for blood MMA clearance by the donor liver. This finding should be critical to understand the long-term outcome for LT in methylmalonic acidemia.
journal_name
Pediatr Transplantjournal_title
Pediatric transplantationauthors
Chen PW,Hwu WL,Ho MC,Lee NC,Chien YH,Ni YH,Lee PHdoi
10.1111/j.1399-3046.2009.01227.xsubject
Has Abstractpub_date
2010-05-01 00:00:00pages
337-41issue
3eissn
1397-3142issn
1399-3046pii
PTR1227journal_volume
14pub_type
杂志文章abstract::BM remains an important source of stem cells. The BM characteristics change with age but the estimation of CD34 calculation of one CD34+ cell per 100 nucleated cells is used for all donors including pediatric donors in the operating room before getting the actual CD34 count. In order to see whether this formula is app...
journal_title:Pediatric transplantation
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abstract::PV thrombosis following pediatric LT is a serious complication that may lead to graft loss. LDLT poses limitations with regard to the availability of vein grafts for complex PV reconstructions. We herein report a unique reconstruction of the PV inflow in a one-yr-old boy with situs inversus undergoing re-LDLT. The inf...
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doi:10.1111/petr.13271
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journal_title:Pediatric transplantation
pub_type: 杂志文章,多中心研究
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更新日期:1997-11-01 00:00:00
abstract::HSCT has substantially improved pediatric acquired SAA patients' outcomes. Retrospectively, we attempted to assess the outcome of MRD HSCT in 65 pediatric patients referred to a single center from 1992 to 2012. We were particularly interested to find out whether source of SC (PB, n = 40 and BM, n = 25) significantly i...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12458
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abstract::Post-transplant anemia is a widespread problem among pediatric renal transplant recipients. Many clinicians treat post-transplant anemia in a manner similar to treatment of anemia in dialysis patients, including the use of intravenous iron, such as sodium ferric gluconate (SFG). Data on pediatric dosing of SFG are lim...
journal_title:Pediatric transplantation
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doi:10.1111/j.1399-3046.2005.00258.x
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journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12067
更新日期:2013-05-01 00:00:00
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journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13798
更新日期:2020-12-01 00:00:00
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journal_title:Pediatric transplantation
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pub_type: 杂志文章
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journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
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更新日期:2007-02-01 00:00:00
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journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12804
更新日期:2016-12-01 00:00:00
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journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12198
更新日期:2014-02-01 00:00:00
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journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2004.00180.x
更新日期:2004-08-01 00:00:00
abstract::NH is the most common identifiable cause of ALF in the neonate. LT is the definitive treatment for neonates with NH who have failed medical therapy. Our aim was to determine the outcomes of LT in infants with NH. Patients (less than one yr of age) with NH who were listed for LT and patients who underwent LT between 19...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12418
更新日期:2015-03-01 00:00:00
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journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13176
更新日期:2018-06-01 00:00:00
abstract::This single-centre, retrospective, observational pilot study was performed to evaluate the safety and efficacy of intravenous and oral itraconazole prophylaxis in paediatric haematopoietic stem cell transplantation (HCT). Study end-points were proven invasive fungal infection (IFI), survival, adverse reactions and gra...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2006.00643.x
更新日期:2007-05-01 00:00:00
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journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12117
更新日期:2013-09-01 00:00:00
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journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.2001.005004297.x
更新日期:2001-08-01 00:00:00
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journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2007.00855.x
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journal_title:Pediatric transplantation
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doi:10.1111/j.1399-3046.2007.00871.x
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更新日期:2015-02-01 00:00:00
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journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:
更新日期:1998-02-01 00:00:00
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pub_type: 杂志文章
doi:10.1111/petr.13683
更新日期:2020-05-01 00:00:00
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journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2009.01213.x
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