Abstract:
:DIDS is a unique form of combined immune deficiency characterized by an unusual susceptibility to cutaneous viral infections, severe allergies with eosinophilia and elevated immunoglobulin E titers, autoimmunity, and cancer. HSCT is considered the standard of care for this deadly disease. We have retrospectively analyzed the outcome of allogeneic HSCT from unrelated donors in patients with DIDS. Data from four patients, with five transplants, are presented. All patients received transplants from unrelated donors' BM, except for one patient who received a cord blood transplant. The conditioning regimens were based on myeloablative protocols for BM derived transplants; a NM regimen was pursued for the patient who received a cord blood transplant, which resulted in graft rejection. Although recurrent pneumonia and skin infections resolved immediately after transplantation, all patients subsequently developed human herpesvirus infection, including cutaneous herpetic lesions, cytomegalovirus reactivation, and zona zoster, which could be attributed to the use of ATG. Despite the presence of serious morbidities prior to transplantation, all patients recovered successfully. DIDS can be successfully treated with allogeneic HSCT from unrelated donors following a myeloablative conditioning regimen, with a reasonable safety profile.
journal_name
Pediatr Transplantjournal_title
Pediatric transplantationauthors
Uygun DFK,Uygun V,Reisli İ,Keleş S,Özen A,Yılmaz M,Sayar EH,Daloğlu H,Öztürkmen SI,Çakı S,Karasu GT,Yeşilipek Adoi
10.1111/petr.13015subject
Has Abstractpub_date
2017-11-01 00:00:00issue
7eissn
1397-3142issn
1399-3046journal_volume
21pub_type
杂志文章abstract::To evaluate the effects of gender on HRQOL and overall health status in our pediatric kidney transplants. We performed a cross-sectional study in 77 children who received living renal allo-transplants in our center between 1981 and 2003. The patients were given a questionnaire at a post-transplant visit. After complet...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2009.01174.x
更新日期:2010-03-01 00:00:00
abstract::Castleman disease is a rare hematologic disorder, closely linked to the HHV-8, and most commonly observed in immunocompromised individuals. Thirteen months following a liver transplant for CPS-1 defect, a 15-month-old boy presented with fevers, anemia, and growth retardation. Abdominal CT scan showed splenomegaly and ...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/j.1399-3046.2011.01570.x
更新日期:2012-09-01 00:00:00
abstract::Adjusted survival outcomes following hepatic resection and transplantation for pediatric liver tumors have not been compared. To address this question, we conducted a retrospective cohort study using the SEER registry. While SEER lacks certain specifics regarding staging, chemotherapy, comorbidities, and recurrence, i...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12144
更新日期:2013-12-01 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::SAB assays have increased the sensitivity and specificity to detect HLA alloantibodies, but there is uncertainty about the clinical relevance of SAB-positive alloantibodies when the FCXM is negative. We performed a retrospective study to evaluate the clinical significance of SAB-detected DSA in 82 pediatric recipients...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2009.01281.x
更新日期:2010-09-01 00:00:00
abstract::Maintaining fluid balance, pre- and post-MA-HCT is essential and usually requires frequent administration of diuretics. Hepatic sinusoidal obstructive syndrome is potentially life-threatening, especially when associated with AKI and MOF. This study describes six patients who developed AKI-associated SOS and diuretic-r...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13139
更新日期:2018-03-01 00:00:00
abstract:BACKGROUND:Cryptosporidium enteritis can be devastating in the immunocompromised host. In pediatric liver transplant recipients, infection may be complicated by prolonged carriage of the parasite, rejection, and biliary tree damage and fibrosis. Herein, we report on six patients and their long-term outcomes following c...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13807
更新日期:2020-12-01 00:00:00
abstract:INTRODUCTION:Myeloablative conditioning before allogeneic HSCT during childhood exposes to serious long-term complications, especially gonadal dysfunction. Pubertal issues are less described than other post-HSCT sequelae in childhood. METHODS:Pubertal development and biological gonadal parameters were assessed in a re...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13773
更新日期:2020-09-01 00:00:00
abstract::Live viral vaccines have historically been avoided in children after solid organ transplantation. Multiple reports of safety and immunogenicity, largely in the pediatric liver transplant population, have led to a reconsideration of this recommendation. Here, we report the case of a 4-year-old boy who inadvertently rec...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13667
更新日期:2020-06-01 00:00:00
abstract::SR-aGVHD remains a significant cause of morbidity and mortality in allogeneic HCT recipients. Alemtuzumab has been used with success in adult patients but has not been studied in the pediatric setting. To estimate the effectiveness of alemtuzumab for the treatment of SR-aGVHD in pediatric patients, we retrospectively ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12183
更新日期:2014-02-01 00:00:00
abstract::The clinical significance of parvovirus B19 infection in pediatric solid-organ and bone marrow transplanted patients is unclear. The overall prevalence of parvovirus B19 infection in these patients is about 1-2% during the first year after transplantation. The most common symptom is anemia, but leukopenia and thromboc...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1034/j.1399-3046.2001.00035.x
更新日期:2001-10-01 00:00:00
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 prec...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2009.01227.x
更新日期:2010-05-01 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::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::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::HBL and HCC are the most common hepatic malignancies in children. The role of OLT in children with HCC is still a matter of debate. The aim of this study was to review our experience of OLT for HCC. Medical records of patients (<18 yr) who underwent OLT for HCC were reviewed and compared to children who underwent OLT ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2007.00751.x
更新日期:2007-09-01 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
abstract::CHS is a rare immunodeficiency syndrome with defects in the functions of cytotoxic cells and neutrophils. Approximately 85% of patients with CHS undergo an AP within the first decade, which is similar to FHLH. Chemotherapy could induce transient remission, but only allogeneic HCT could correct the underlying genetic d...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13014
更新日期:2017-11-01 00:00:00
abstract::Although de novo DSA are associated with inferior graft survival, there are no effective strategies to prevent their formation. Underexposure to MPA (prodrug: MMF) also contributes to rejection rates early after transplantation, but the effect of this phenomenon on the formation of DSA long-term post-transplantation i...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12637
更新日期:2016-02-01 00:00:00
abstract::Obscure gastrointestinal (GI) bleeding is defined as bleeding from the GI tract that persists or recurs, with no obvious etiology, after esophagogastroduodenoscopy (EGD), colonoscopy, and radiologic evaluation of the small bowel. We present the case of a 17-yr-old girl who for two years had been suffering from recurre...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2010.01425.x
更新日期:2012-05-01 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::Cardiac transplantation for children with end-stage cardiac disease with no other medical or surgical options is now standard. The number of children in need of cardiac transplant continues to exceed the number of donors considered "acceptable." Therefore, there is an urgent need to understand which recipients are in ...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/petr.13671
更新日期:2020-05-01 00:00:00
abstract::Despite significant interest by pediatric transplant patients in meeting others who have undergone transplantation, geographic distances combined with their daily routines make this difficult. This mixed-method study describes the use of Zora, a Web-based virtual community designed to create a support system for these...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2010.01271.x
更新日期:2010-03-01 00:00:00
abstract::Despite advances in therapy for acute leukemia, relapse continues to be the major cause of treatment failure. Hematopoietic stem cell transplant can rescue some patients after relapse, but the ability to escalate the intensity of preparative regimens is limited by toxicity to normal organs. Radiolabeled monoclonal ant...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1034/j.1399-3046.7.s3.14.x
更新日期:2003-01-01 00:00:00
abstract::Cystic fibrosis (CF) is an inherited disease with a defect in epithelial chloride transport that results in a multisystem disease. Although pulmonary disease remains the primary cause of morbidity and mortality, focal biliary cirrhosis and portal hypertension may develop in up to 8% of these patients. Liver transplant...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12157
更新日期:2014-02-01 00:00:00
abstract::Despite the introduction of a variety of new immunosuppressive agents, cyclosporine A (CsA) has maintained a strong position in pediatric transplantation (Tx). Post-Tx dosing with CsA is a challenging task because of the narrow therapeutic window of the drug, the great individual variability of metabolism and the lack...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.2003.00025.x
更新日期:2003-04-01 00:00:00
abstract::The aim of this study was to study the incidence of chronic renal dysfunction in patients with more than 5 yr of follow-up following liver transplantation and to evaluate the benefit of decreasing cyclosporine A (CsA) dose combined with mycophenolate mofetil (MMF) on renal function and immune response in these patient...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2004.00172.x
更新日期:2004-10-01 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::Children receiving HCT loose protective immunity to vaccines received pre-HCT. Therefore, revaccination post-HCT is of major importance. In Denmark, a vaccination schedule with fewer doses post-HCT has been used, including two doses for diphtheria, tetanus, polio, measles, mumps, and rubella, and one dose only for Hae...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13599
更新日期:2020-02-01 00:00:00
abstract::This study examined patient-reported sleep quality in a single-center cross-sectional sample of adolescents with solid organ transplants and evaluated associations between sleep quality, psychosocial functioning (ie, depression/anxiety symptoms), and HRQOL. Health disparities associated with minority race/ethnicity an...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13577
更新日期:2019-12-01 00:00:00