Abstract:
:Omenn's syndrome is a rare inherited variant of SCID. It is inevitably fatal, unless treated by bone marrow or stem cell transplantation. However, treatment-related complications and graft rejection are major obstacles to the success of transplantation. In this report, we describe an eight-month-old baby with Omenn's syndrome and disseminated BCGosis, who underwent allogenic BMT from his HLA-matched sister using anti-GVHD prophylaxis but without a conditioning regimen. Ten days after BMT, he developed acute GVHD involving the skin only (grade 1) with a flare of local BCGosis. Engraftment of 55% was demonstrated by FISH as early as the 11th day post-BMT. On day +48 post-BMT, he developed GVHD that was complicated by refractory pancytopenia, resistant to three doses of rituximab. Patient was re-transplanted (from the same donor) after receiving fludarabine and ATG, with successful engraftment and complete recovery from pancytopenia.
journal_name
Pediatr Transplantjournal_title
Pediatric transplantationauthors
Wahadneh A,Bin-Dahman H,Habahbeh Z,Abu-Shukear M,Ajarmeh M,Zyood R,Khaled Adoi
10.1111/j.1399-3046.2010.01413.xsubject
Has Abstractpub_date
2012-03-01 00:00:00pages
E43-8issue
2eissn
1397-3142issn
1399-3046journal_volume
16pub_type
杂志文章abstract::Heart transplantations have been performed in the Centre for Paediatric Heart Surgery at the Justus Liebig University in Giessen (Germany) since 1988. For further consultation and therapy, some affected children subsequently present at the Polyclinic for Paediatric Dentistry. In all these cases problematic oral findin...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2005.00429.x
更新日期:2006-03-01 00:00:00
abstract::There is currently no way to diagnose a rejection before a change in serum creatinine. This had led some to start doing SB, but little data exist on the utility and safety of SB in pediatric patients. There is also little known on practice patterns of pediatric nephrologists. A retrospective review of pediatric kidney...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13178
更新日期:2018-06-01 00:00:00
abstract::There are limited published data on surveillance TBB for the identification of allograft rejection in infants after lung or heart-lung transplantation. We performed a retrospective review of children under one yr of age who underwent lung or heart-lung transplant at our institution. Since 2005, four infants were trans...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12125
更新日期:2013-11-01 00:00:00
abstract::In many countries, adolescents can choose to register a deceased organ donation wish when they apply for a driver's license. They often receive education about deceased organ donation in order to make an informed choice. The objective of this review was to describe the effectiveness of school-based educational program...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/petr.12045
更新日期:2013-03-01 00:00:00
abstract::Bilateral nephrectomy prior to transplantation is indicated in some patients with end-stage renal disease. The indications for bilateral nephrectomy include persistent heavy proteinuria, refractory hypertension, and urinary tract infections. We report an eight-month-old baby with male pseudohermaphroditism and renal f...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2006.00624.x
更新日期:2007-03-01 00:00:00
abstract::T1D is an autoimmune disease, which may be caused by lack of insulin-secreting β cells due to damage of autoimmune system. Living with T1D is a challenge for the child and the family; cell transplantation is a treatment option for diabetes in children. To establish a microenvironment suitable for cell growth and proli...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12443
更新日期:2015-06-01 00:00:00
abstract::It is the practice of many pediatric renal transplant programs to 'convert' children taking cyclosporin A (CsA) to tacrolimus, although the indications for, outcome, and complications of this practice remain obscure. To better understand these aspects of tacrolimus 'conversion', a fax survey was sent to 119 North Amer...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:
更新日期:2001-12-01 00:00:00
abstract::aHUS is caused by the over-activation and dysregulation of the alternative complement pathway. Data regarding outcomes of pediatric aHUS patients after kidney transplantation are still very scarce. Accordingly, the aim of this study was to describe the clinical findings and outcomes of pediatric aHUS patients after re...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13914
更新日期:2020-11-20 00:00:00
abstract::Malnutrition adversely affects mortality and morbidity before and after liver transplantation. Outcome might be improved if liver transplant recipients were in a better nutritional state at the time of transplantation. In this review, we will examine the potential use of GH and IGF-I to improve nutritional status in p...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:
更新日期:1997-08-01 00:00:00
abstract::To evaluate the role of BAL, CTB, and OLB in the management of pulmonary infiltrates in pediatric HSCT recipients, we conducted a retrospective review of clinical records of pediatric HSCT recipients. Data were analyzed using Chi-square for dichotomous and anova for continuous variables. Logistic regression was used t...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2007.00733.x
更新日期:2007-11-01 00:00:00
abstract::LI is a subset of the heterotaxy syndrome and a rare birth defect that involves the heart and other organs. It can be combined with extracardiac abnormalities, especially BA. CHD can be associated with LI in up to 15% of cases, although it is rare in BA. Pediatric LT for a child with ESLD due to BA combined with LI an...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12364
更新日期:2014-12-01 00:00:00
abstract::Focal segmental glomerulosclerosis (FSGS) occurring in association with cytomegalovirus (CMV) infection in a renal transplant patient with no previous history of FSGS has rarely been reported. We present a case of a 16-year-old renal transplant recipient who developed acute hepatitis, leukopenia, nephrotic syndrome, a...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13538
更新日期:2019-09-01 00:00:00
abstract:BACKGROUND:Heart transplant waitlist mortality remains high in infants <1 year of age and among those with CHD. Currently, the median accepted donor-to-recipient weight percentage is approximately 130% of the recipient's weight. We hypothesized that patients with CHD may accept a larger organ using novel 3D-derived ima...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13639
更新日期:2020-02-01 00:00:00
abstract::Aspergillosis is an important cause of mortality in allogeneic HSCT. A "fungus ball" formation of Aspergillus in the bladder has seldom been reported. We report a child that underwent HSCT and developed possible disseminated aspergillosis with an intravesical "fungus ball," diagnosed by genitourinary MRI and PCR of th...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2007.00871.x
更新日期:2008-03-01 00:00:00
abstract::UCBT recipients with TM are at high risk of EF related to low number of stem cells and prior alloimmunization after multiple blood transfusions. Here, we evaluated the safety and efficacy of double-unit UCBT using TT-containing conditioning regimens in TM. Retrospective analysis of children who underwent double-unit U...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13901
更新日期:2020-11-02 00:00:00
abstract::There is a limited supply of organs for all those who need them for survival. Thus, careful decisions must be made about who is listed for transplant. Studies show that manifesting genetic disease can impact listing eligibility. What has not yet been studied is the impact genetic risks for future disease have on a pat...
journal_title:Pediatric transplantation
pub_type: 杂志文章,多中心研究
doi:10.1111/petr.13402
更新日期:2019-06-01 00:00:00
abstract::Little has been studied regarding the diets of children following LTX. The study aim was to assess and compare dietary intake and DQ of healthy children and children post-LTX. Children and adolescents (2-18 years) post-LTX (n=27) and healthy children (n=28) were studied. Anthropometric and demographic data and two 24-...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12944
更新日期:2017-09-01 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::A five-yr-old boy developed chronic liver failure and ESKD because of CHF and juvenile NPHP. He underwent sequential liver and kidney transplantation with a compatible blood type from his father, at five yr, seven months and five yr, 11 months old, respectively. Because the patient was not in ESKD, we initially perfor...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2011.01611.x
更新日期:2012-11-01 00:00:00
abstract::In their provocative paper, "Lung transplantation and survival in children with cystic fibrosis," Liou and colleagues state that "Prolongation of life by means of lung transplantation should not be expected in children with cystic fibrosis. A prospective, randomized trial is needed to clarify whether and when patients...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2008.00924.x
更新日期:2008-03-01 00:00:00
abstract::Bartter's syndrome (BS) is an incurable genetic disease, with variable response to supportive therapy relating to fluid and electrolyte management. Poor control or therapy non-compliance may result in frequent life threatening episodes of dehydration, acidosis and hypokalemia, with resultant adverse effects on patient...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2005.00435.x
更新日期:2006-03-01 00:00:00
abstract:BACKGROUND/AIM:Several PK studies have shown that most pediatric patients may require higher doses on a mg/kg basis compared to adults to attain similar therapeutic trough concentrations. The aim of this study was to compare the efficacy and safety of three times daily to twice a day dosing of tacrolimus in pediatric k...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13733
更新日期:2020-09-01 00:00:00
abstract::EBV is associated with various malignancies in patients with acquired or induced immune impairment. EBV-SMT is very uncommon in immunocompromised patients, and a kidney localization has been described only anecdotally. We report the case of a 17-yr-old kidney transplant recipient diagnosed as having an EBV-SMT inside ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2009.01213.x
更新日期:2010-12-01 00:00:00
abstract::Major neurologic complications secondary to cyclosporine are well documented and are known to include confusion, cortical blindness, seizure, spasticity, paresis, ataxia and coma. Most previous reports attribute these to white matter central nervous system (CNS) lesions or white/grey matter border lesions. Many predis...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:
更新日期:1998-02-01 00:00:00
abstract::BWS is one of the most well-known somatic overgrowth syndromes, which is characterized by macroglossia, organomegaly, abdominal wall defects, and predisposition to embryonal tumors, such as Wilms' tumor, hepatoblastoma, and adrenocortical carcinoma. We report a case of BWS in a girl with unresectable hepatoblastoma, w...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2009.01198.x
更新日期:2010-11-01 00:00:00
abstract::HHV type 6 has been reported with enhanced pathogenicity in immunocompromised patients. Herein, we report about a two-yr-old girl who experienced primary HHV 6 infection after liver transplantation. She clinically presented with graft rejection and necrotic hepatitis as well as high fever, pneumonitis with respiratory...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2010.01310.x
更新日期:2011-09-01 00:00:00
abstract::The thymus gland possesses the ability to regrow in children leading to a newly developed anterior mediastinal mass. This condition may represent a rebound phenomenon during recovery from a stressful event such as post-chemotherapy and hence was described as RTH. RTH after LT has not been well documented. We are repor...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13251
更新日期:2018-07-24 00:00:00
abstract::Renal transplantation remains the therapy of choice for children and adolescents with ESRD. Differences in graft survival are observed in kidney transplant recipients of different race and ethnicities. Data in pediatric populations are limited and confounded by disparities in access to health care. We performed a retr...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2008.01074.x
更新日期:2009-11-01 00:00:00
abstract::Recurrent and de novo disease is an increasing problem and is known to negatively impact transplant graft survival. Immunosuppressive medications have not had an impact on the prevalence of recurrent and de novo disease. Renal Allograft Disease Registry (RADR) was established to study the prevalence, impact and risk f...
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: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