Abstract:
:LAD is a rare and fatal congenital disorder in which there is a defect of the leukocyte adhesion molecule (CD18) on neutrophils. Severe LAD results in frequent and potentially fatal infections. Although allogeneic HSCT is the only curative treatment for severe LAD, strategies for HSCT remain to be established since a high engraftment failure rate and severe persistent infection are still seen. We report a four-month-old boy with LAD who was successfully treated with allogeneic HSCT from an HLA-complete matched sibling following a conditioning regimen of pharmacokinetically adjusted individual busulfan doses.
journal_name
Pediatr Transplantjournal_title
Pediatric transplantationauthors
Yoshimoto A,Hashii Y,Kashiwagi H,Koizumi M,Tokimasa S,Fujisaki H,Ohta H,Hanai N,Ozono K,Hara Jdoi
10.1111/j.1399-3046.2007.00723.xsubject
Has Abstractpub_date
2007-06-01 00:00:00pages
453-5issue
4eissn
1397-3142issn
1399-3046pii
PTR723journal_volume
11pub_type
杂志文章abstract::Controversy exists over whether PHTN in heart transplant candidates increases post-transplant mortality. We performed analysis of data reported to UNOS for children who underwent primary heart transplantation for cardiomyopathy from January 1994 to June 2010. Patients were divided into two groups depending on their pr...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2012.01678.x
更新日期:2012-06-01 00:00:00
abstract::eHAT is one of the most dreaded post-LT complication. Treatment approaches include retransplantation, revascularization, or observation. Systemic thrombolytic therapy is used in pediatric patients with thromboembolic events. However, there is no previous study reporting on the use of systemic r-tPA to treat eHAT. The ...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13902
更新日期:2020-10-27 00:00:00
abstract::While 24-h ambulatory blood pressure monitoring (ABPM) is an established tool for monitoring antihypertensive therapy in adults, data in children are scarce. We retrospectively analysed whether office blood pressure (BP) is reliable for the diagnosis of BP control in 26 treated hypertensive paediatric renal transplant...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2006.00595.x
更新日期:2007-02-01 00:00:00
abstract::Nonalcoholic steatohepatitis (NASH) is the most severe form of non-alcoholic fatty liver disease (NAFLD). The aim of our study was to highlight NASH as a rare but possible problem in children. We present a case of 13-yr-boy with a well-established diagnosis of liver cirrhosis secondary to NASH, who underwent orthotopi...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2007.00767.x
更新日期:2007-11-01 00:00:00
abstract::The adjusted relative risk (aRR) for development of post-transplant lymphoproliferative disorder (PTLD) is higher in kidney transplant recipients receiving monoclonal antibody induction therapy, but the aRR between the different available polyclonal agents has not been investigated in detail. We analyzed data from the...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2005.00361.x
更新日期:2005-10-01 00:00:00
abstract::Recurrent FSGS is a major challenge in the field of nephrology. To clarify the role of NPHS2 defects in the pathogenesis of FSGS recurrence, we sequenced all eight exons of NPHS2 in 11 Japanese pediatric FSGS patients with or without post-transplant recurrence. All patients had biopsy-proven primary FSGS, had no famil...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2007.00752.x
更新日期:2008-05-01 00:00:00
abstract::The high discard rate of pediatric donor hearts presents a major challenge for children awaiting heart transplantation. Recent literature identifies several factors that contribute to the disparities in pediatric donor heart usage, including regulatory oversight, the absence of guidelines on pediatric donor heart acce...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/petr.13655
更新日期:2020-05-01 00:00:00
abstract::To evaluate social development of pediatric heart transplant (tx) recipients who have lived 15 or more years after transplantation. Among 498 pediatric patients, age less than 18 years, who underwent heart transplantation, at a single institution, 337 were performed between 1985 and 1998. We identified all who survive...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12853
更新日期:2017-03-01 00:00:00
abstract::Ingestion of rare-earth magnet beads in children has been a public health concern. The potential risk of swallowing multiple magnets is related to magnet attraction to each other, resulting in serious gastrointestinal complications, such as entero-enteric fistula formation, peritonitis, bowel ischemia or necrosis, bow...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13915
更新日期:2020-11-20 00:00:00
abstract::We report a case of a patient who received a bilateral lung transplant for end-stage lung disease secondary to Gauchers type-1 disease with no evidence of recurrence of the disease in the transplanted lung. ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2005.00251.x
更新日期:2005-04-01 00:00:00
abstract::Pediatric cardiac transplantation is currently an accepted option for end-stage heart disease and congenital cardiac malformations. This report focuses on the anesthetic perioperative management in 12 yr. From 1988 to 2001 we performed 90 heart transplantations in 88 children, infants and neonates. The pediatric heart...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2004.00155.x
更新日期:2004-06-01 00:00:00
abstract:PURPOSE:The understanding of the HRQOL issues for parent donors of children who underwent LDLT is lacking. We evaluated the HRQOL of donor and non-donor parents, described their subjective experiences and identified factors associated with lower HRQOL post-donation. METHODS:This is a cross-sectional study of parent do...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13337
更新日期:2019-03-01 00:00:00
abstract::The risk of adverse outcomes for pediatric renal transplant patients is highest during the transition from pediatric to adult care. While there have been many studies focus on graft failure and death, loss to follow-up likely plays a large role in patient outcomes. We hypothesize patients are lost to follow-up during ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13547
更新日期:2019-11-01 00:00:00
abstract::Studies are increasingly recognizing health-related quality of life (HRQOL) as a key pediatric outcome in both clinical and research settings and an essential health outcome measure to assess the effectiveness of medical treatment. However, it has not yet been studied among the healthy siblings of kidney transplant re...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13734
更新日期:2020-08-01 00:00:00
abstract::Thrombosis after kidney transplantation may result in catastrophic outcomes, including graft loss. Thrombophilia has been implicated in post-transplant thrombosis; data, however, are inconclusive on the impact of acquired and inherited thrombophilia and resultant thrombosis in renal graft recipients. We aimed to evalu...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13314
更新日期:2019-02-01 00:00:00
abstract::This systematic review was designed to find out optimal tacrolimus dose in pediatrics according to their CYP3A5*1 genotype by performing meta-analysis. PubMed, Scopus, ISI web of Science, ProQuest, Cochrane library, and clinicaltrail.gov were systematically searched to find studies in which tacrolimus dose and/or bloo...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/petr.13248
更新日期:2018-06-19 00:00:00
abstract::PMF of infancy is a recently described autosomal recessive disorder presenting with severe bone marrow failure, accelerated neutrophil apoptosis, and significant platelet dysfunction, caused by a mutation in the VPS45 gene. In this study, we update our group of patients with PMF, highlighting different aspects of this...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12169
更新日期:2013-12-01 00:00:00
abstract:BACKGROUND:The objective of the study was to analyze the profile of infections in children with BMF following alloHCT. METHODS:Data of 169 consecutive children with inherited and acquired BMF treated with alloHCT between 2012 and 2017 in Polish pediatric transplant departments were analyzed in registry-based retrospec...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13592
更新日期:2019-12-01 00:00:00
abstract::ADV is frequently seen in our pediatric SOT population. It presents in a variety of clinical presentation and can cause severe disease. In this population, there are very few studies to determine the safety of CDV as a potential therapeutic agent. We present the findings of our retrospective study evaluating the effic...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13231
更新日期:2018-06-03 00:00:00
abstract::Drowning, a common cause of death in the pediatric population, is a potentially large donor pool for OLT. Anecdotally, transplant centers have deemed these organs high risk over concerns for infection and graft dysfunction. We theorized drowned donor liver allografts do not portend worse outcomes and therefore should ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13009
更新日期:2017-09-01 00:00:00
abstract::Hypogammaglobulinemia is common after pediatric SCT and IgG replacement is recommended. Some children will have a prolonged hypogammaglobulinemia but may have poor venous access which impedes further iv IgG replacement. In primary antibody deficiencies, sc IgG replacement, performed by parents at home, has been shown ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2012.01783.x
更新日期:2012-12-01 00:00:00
abstract:: FSGS has a high recurrence rate after renal transplantation. To examine the effects of the use of preemptive and post-transplant PP on recurrence and graft outcome, we conducted a retrospective study on 34 pediatric patients (mean age 13±5 yr) with biopsy-proven pretransplant FSGS and who underwent a renal transplan...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2011.01478.x
更新日期:2011-08-01 00:00:00
abstract::Cystic fibrosis (CF) is associated with varying degrees of pleural inflammatory reaction that occurs as a result of chronic pulmonary infections and intervention to pleural space. The amount of pleural reaction is associated with the difficulty involved when performing the pneumonectomy at the time of lung replacement...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:
更新日期:1998-11-01 00:00:00
abstract::Much is still unknown about LUT function after receiving renal graft. Graft function was the main focus of different studies discussing the same issue. However, these studies ignored the effects of the graft on lower tract function and more demand for bladder cycling and growth of the child. Therefore, we aimed at eva...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13735
更新日期:2020-09-01 00:00:00
abstract::The aim of this study was to re-evaluate the indications and timing of LT for WD. From 2000 to 2009, eight patients with WD who had been referred to our institution for LT were enrolled in this study. The mean patient age was 15.9 yr (range, 7-37 yr). Four patients could not receive LT, because there were no available...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12085
更新日期:2013-06-01 00:00:00
abstract:BACKGROUND AND AIM:The aim of the study was to assess efficacy and safety of endoscopic treatment in BS after pediatric LTx. METHODS:We retrospectively reviewed data of patients with DDA who developed BS and underwent ERCP. RESULTS:Of 189 transplanted patients with DDA, strictures developed in 30 (16%). In this subgr...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13271
更新日期:2018-11-01 00:00:00
abstract::Recurrence of hemolytic uremic syndrome (HUS) after kidney transplantation is frequent, occurring almost exclusively in patients with atypical HUS, which is not caused by Escherichia coli gastroenteritis and in which diarrhea is absent. Calcineurin inhibitors are associated with recurrence of HUS. In two children who ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.2002.t01-1-00002.x
更新日期:2002-08-01 00:00:00
abstract::Nephrotic syndrome is characterized by proteinuria, hypoalbuminemia, and general edema. These symptoms may persist in children who reach ESRD, which is unfavorable for the patient's allograft outcome. In addition, this may hamper early diagnosis of a relapse after transplantation. Surgical bilateral nephrectomy is oft...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13703
更新日期:2020-06-01 00:00:00
abstract::FH is a hereditary inherited disorder of cholesterol metabolism. Homozygous form of the disease associates severe form of atherosclerotic disease. Clinicians have been tried to inhibit the progression of the homozygous FH with medical and surgical treatment. We here present three siblings with homozygous FH who were s...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2010.01469.x
更新日期:2011-05-01 00:00:00
abstract:BACKGROUND:Urinary diversion using catheterizable channel among posterior urethral valve (PUV) patients may help to alleviate the functional concerns of the bladder on the allograft. Herein, we review our series of PUV patients undergoing renal transplants at a single institution to determine outcomes between those wit...
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