Abstract:
:Germline GATA2 mutations have been associated with a vast array of clinical manifestations, as well as hematological deficiencies and a propensity to AML or MDS. We present two cases of pediatric AML/MDS with underlying GATA2 mutations who underwent a successful umbilical cord hematopoietic stem cell transplantation using two different conditioning regimens. These cases illustrate the importance of recognizing the clinical features associated with GATA2 mutations and performing the appropriate molecular testing. Diagnosis of heritable gene mutations associated with familial AML/MDS has significant clinical implication for the patients and affected families.
journal_name
Pediatr Transplantjournal_title
Pediatric transplantationauthors
Mallhi K,Dix DB,Niederhoffer KY,Armstrong L,Rozmus Jdoi
10.1111/petr.12764subject
Has Abstractpub_date
2016-11-01 00:00:00pages
1004-1007issue
7eissn
1397-3142issn
1399-3046journal_volume
20pub_type
杂志文章,评审abstract::Activation of the RAS has a crucial role in the progression of ischemia/reperfusion-associated CAD. The regulation of RAS differs in the two genders. However, the extent of gender differences and locations of renin production have not been revealed yet. We investigated in vivo the local renin production in the two gen...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12712
更新日期:2016-08-01 00:00:00
abstract::Recipient lymphocytes are crucial for direct and indirect pathways of allorecognition. We proposed that the administration of alemtuzumab several weeks pretransplantation could eradicate peripheral lymphatic cells and promote donor-specific acceptance. This was a single-center, retrospective review of 101 consecutive ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12941
更新日期:2017-08-01 00:00:00
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::Laparoscopic donor nephrectomy (LDN) is rapidly becoming the preferred technique for the procurement of living donor kidneys. An association of this technique with delayed graft function and higher risk for rejection has been reported in pediatric recipients. We reviewed our experience of 17 pediatric patients who rec...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2005.00478.x
更新日期:2006-05-01 00:00:00
abstract::BK DNAemia in renal transplant recipients is a significant cause of allograft dysfunction and can lead to graft loss due to BK polyomavirus-associated nephropathy or to graft rejection due to immunosuppression reduction. Currently, the first-line treatment for BK DNAemia is immunosuppression reduction. Second-line tre...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13600
更新日期:2020-02-01 00:00:00
abstract::IHHE as the most common vascular tumor of the liver in infancy can present with acute postnatal liver and congestive heart failure. LTx may be a lifesaving option, but can be complicated by extrahepatic involvement and bleeding complications, especially in neonates. Here we discuss the benefit of LTx in cases of acute...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2008.01039.x
更新日期:2009-08-01 00:00:00
abstract::Clinical grading of GI involvement during acute GVHD remains a challenging issue, especially in children. Plasma citrulline, a non-protein amino acid selectively produced and released by enterocytes, is a suitable surrogate endpoint for small intestinal epithelial cell mass, irrespective of the underlying cause of cel...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12128
更新日期:2013-11-01 00:00:00
abstract::The aim of the study was to demonstrate clinical course of the first reported cases of PLS in pediatric kidney transplantation and therapeutic outcome for such condition using a combination of high-dose corticosteroid and tacrolimus. We report a single case (a nine-year-old Thai boy) with end-stage kidney disease seco...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2006.00560.x
更新日期:2006-09-01 00:00:00
abstract::An increasing number of individuals with end-stage renal disease have become sensitized to human leukocyte antigens (HLA). Sensitization can have a profound impact on the likelihood of obtaining a requisite negative crossmatch (-XM) with a potential donor. Technologic breakthroughs in our ability to diagnose antibody-...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2004.00214.x
更新日期:2004-12-01 00:00:00
abstract:BACKGROUND:Of the 600 pediatric candidates added to the liver waiting list annually, 100 will remain waiting while over 100 liver allografts are discarded, often for subjective reasons. METHODS:We created a risk index to predict discard to better optimize donor supply. We used the UNOS database to retrospectively anal...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13963
更新日期:2021-01-06 00:00:00
abstract:BACKGROUND:The hybrid stage 1 palliation for hypoplastic left heart syndrome (HLHS) was first described in 1993 as a bridge to heart transplant for HLHS. There are limited data on this strategy as primary heart transplantation for HLHS has become less common. METHODS:This is an observational, single-center study compa...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13307
更新日期:2018-12-01 00:00:00
abstract::Biliary atresia is defined as partial or total obliteration of the extra-hepatic bile ducts. In advanced cases, liver transplantation (LTx) is considered the most appropriate treatment. This report describes a female patient whose biliary atresia and subsequent cirrhosis required LTx at 1 yr of age. Macroscopic inspec...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.2001.t01-1-00020.x
更新日期:2001-02-01 00:00:00
abstract::PTLD is a major complication after transplantation. Treatment options for PTLD are not standardized, usually sequential, starting with reduction in immunosuppression. Recently, we have used a dual combination of rituximab and reduced dose chemotherapy (R/C) directly after failed RI. We retrospectively identified 30 pe...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2010.01370.x
更新日期:2010-11-01 00:00:00
abstract::We report a 10-yr-old boy who developed CIPS after a second allogeneic BMT for severe aplastic anemia. He received the second BMT from the same HLA-matched sibling donor 16 months after the first BMT due to secondary graft failure. The preparative regimen for the second BMT consisted of fludarabine, cyclophosphamide, ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2008.01009.x
更新日期:2009-08-01 00:00:00
abstract::Alternate donor HSCT for thalassemia major from a matched unrelated donor or haploidentical family donor is a feasible therapeutic option in children with no matched family donor. Aggressive pretransplant immunosuppression, reduced toxicity conditioning, and PTCY result in excellent thalassemia-free survival. We descr...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13893
更新日期:2020-10-27 00:00:00
abstract::Success after solid organ transplantation is dependent on the proper balance of immunosuppression to prevent rejection of the allograft while limiting the risk of developing infections and malignancy. We present a 9-year-old girl, remote from transplant, who presented with airway plaque after a change in immunosuppres...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13046
更新日期:2017-11-01 00:00:00
abstract::Pediatric solid organ transplant recipients have long-standing malnutrition concerns related to their pretransplant medical status. The targeted nutrition therapy utilized pre-, peri-, and post-transplantation may have the adverse effect of impeding normally developing feeding skills, particularly in very young childr...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13389
更新日期:2019-05-01 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::Although TEG directs effective resuscitation in adult surgical patients, pediatric data are lacking. We performed a retrospective comparative review of the effect of TEG on blood product utilization and outcomes following pediatric liver transplantation in 38 patients between 2008 and 2014. Diagnoses, laboratory value...
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::The role of unrelated donor HSCT for children with de novo AML in CR1 is controversial. We performed this study to investigate the feasibility of unrelated donor HSCT who initially had intermediate- or high-risk cytogenetics. We retrospectively reviewed medical records of patients with AML who received unrelated HSCT ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13397
更新日期:2019-06-01 00:00:00
abstract::Medical cannabis is now legal in over half of the United States. As more patients adopt this unconventional therapy, it is inevitable that potential transplant recipients will disclose their cannabis use during transplant evaluation. Transplant teams are tasked with the decision to utilize a pressure resource, often w...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13467
更新日期:2019-08-01 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::DD kidney allocation protocols may influence timing of transplantation and graft quality for pediatric recipients. This study aimed to evaluate the effects of these protocols, including pediatric priority, on waiting time on dialysis, transplant type, donor age, and HLA matching according to state of transplant in Aus...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12544
更新日期:2015-09-01 00:00:00
abstract::RVIs are a significant cause of morbidity and mortality in immunocompromised children. We analyzed the characteristics and outcomes of infection by four respiratory viruses (RSV, adenovirus, influenza, and parainfluenza) treated at a pediatric tertiary care hospital in a retrospective cohort of patients who had receiv...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12022
更新日期:2013-03-01 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::Rabbit antithymocyte globulin (ATG-F) is an extensively used induction agent. To our knowledge, no study to date has assessed reduced ATG-F dosage in children undergoing renal transplantation. This was a retrospective analysis of pediatric renal recipients in the Department of Kidney Transplantation, The First Affilia...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12224
更新日期:2014-05-01 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::We assessed our long-term experience with regards to the safety and efficacy of MMF in our pediatric renal transplant population and compared it retrospectively to our previous non-MMF immunosuppressive regimen. Forty-seven pediatric renal transplants received MMF as part of their immunosuppressive protocol in the per...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2009.01179.x
更新日期:2011-05-01 00:00:00
abstract::Cryptosporidium is an intracellular protozoa that can cause gastroenteritis in humans. In immunocompromised hosts, infection can be severe, leading to life-threatening persistent diarrhea. There is limited experience in treating this infection in solid organ transplants. Although newer drugs active against Cryptospori...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/j.1399-3046.2006.00593.x
更新日期:2007-02-01 00:00:00
abstract::DTI indices have been associated with cellular rejection in adult heart transplant recipients, but their predictive value in pediatric recipients is unknown. The purpose of this study was to evaluate DTI measures in the detection of cellular and AMR in pediatric heart transplant recipients. One hundred and forty-eight...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2007.00812.x
更新日期:2008-03-01 00:00:00