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 quality of life (QOL). We report, for the first time, pre-emptive bilateral native nephrectomies and successful renal transplantation, prior to the onset of ESRD, for severe, clinically brittle, neonatal BS, resulting in correction of metabolic abnormalities and excellent graft function. We propose that fragile BS should be considered as a possible indication for early native nephrectomies and pre-emptive renal transplantation, procedures that results in a 'cure' for the underlying disease and significant improvements in patient QOL.
journal_name
Pediatr Transplantjournal_title
Pediatric transplantationauthors
Chaudhuri A,Salvatierra O Jr,Alexander SR,Sarwal MMdoi
10.1111/j.1399-3046.2005.00435.xkeywords:
subject
Has Abstractpub_date
2006-03-01 00:00:00pages
266-70issue
2eissn
1397-3142issn
1399-3046pii
PTR435journal_volume
10pub_type
杂志文章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::LT is a successful treatment for end-stage liver disease. The long-term outcome of patients transplanted in childhood has not previously been widely reported. This project assessed the long-term impact of transplantation in patients surviving >15 years. Retrospective data on growth, end-organ damage and psychosocial d...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13782
更新日期:2020-11-01 00:00:00
abstract::Myocarditis represents an important cause for acute heart failure. MYKKE, a prospective multicenter registry of pediatric patients with myocarditis, aims to gain knowledge on courses, diagnostics, and therapy of pediatric myocarditis. The role of mechanical circulatory support (MCS) in children with severe heart failu...
journal_title:Pediatric transplantation
pub_type: 杂志文章,多中心研究
doi:10.1111/petr.13548
更新日期:2019-11-01 00:00:00
abstract::HTN after renal transplantation is associated with cardiovascular morbidity. ABPM allows diagnosis of masked HTN and isolated nocturnal HTN. Longitudinal ABPM data in children post-transplant are limited. ABPM was performed in children post-transplant and repeated in 6-12 months. BP indices were used to determine the ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13710
更新日期:2020-06-01 00:00:00
abstract::There are little data on prevalence of dyslipidemia in pediatric kidney TX recipients in the modern IS era. LP profiles of 38 TX recipients receiving triple IS with MMF, prednisone, and tacrolimus were compared with those of 11 children on HD using mixed model multiple linear regression analysis of repeated measures a...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2008.00905.x
更新日期:2008-11-01 00:00:00
abstract::There are few studies on invasive pneumococcal disease in pediatric transplant recipients. Given this fact plus the advent of pneumococcal conjugate vaccines, we conducted a retrospective study at a major pediatric transplant center. The objectives were to determine the incidence and outcomes of invasive pneumococcal ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2005.00275.x
更新日期:2005-04-01 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::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::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::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::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::Administration of hematopoietic growth factors, with or without chemotherapy, can augment progenitor cell numbers available for collection. The dose of granulocyte colony stimulating factor (G-CSF) used for mobilization of peripheral blood progenitor cells (PBPC) is controversial, and doses between 5 and 32 microg/kg/...
journal_title:Pediatric transplantation
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:1998-05-01 00:00:00
abstract::Surgical techniques that have been used during liver transplantation (LT) together with patient's coagulation profile and institutional practices are reported to have an effect on transfusion requirements. The aim of this study is to evaluate the transfusion requirement in both cadaveric (CDLT, n = 22) and living dono...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2005.00284.x
更新日期:2005-06-01 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::Few studies have identified the psychosocial characteristics of those children and their families associated with future non-adherence to immunosuppressive medications following a heart or lung transplant. UNOS data and medical records information were used to test the association between patient and family psychosoci...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12899
更新日期:2017-06-01 00:00:00
abstract::This 18-month prospective investigation sought to examine changes in HRQOL over time for adolescent solid organ transplant recipients. Additionally, this study examined the relationship between adolescent and parent report of HRQOL and compared parent report of HRQOL to published normative data. Forty-eight adolescent...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2010.01392.x
更新日期:2010-12-01 00:00:00
abstract:AIMS:We investigated interrelations between cognitive abilities, behavioural problems, quality of life and disease-related variables of children after LTX. METHODS:Our sample consisted of 25 children. They were 8.5/2.8 (M/SD) years old and had received the transplant 5.5/3.1 years previously. For assessment we used we...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2009.01257.x
更新日期:2010-06-01 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::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::Early GF is a frequent complication following hematopoietic stem cell transplantation for patients with thalassemia. We report the outcome of double-unit CBT in three patients who developed early GF after CBT. The initial conditioning regimen consisted of i.v. Bu 14 mg/kg (day -9 to -6), i.v. Cy 200 mg/kg (day -5 to -...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2008.01021.x
更新日期:2009-09-01 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::The literature regarding the etiology and incidence of short and long-term renal functional impairment in pediatric liver allograft recipients was reviewed. Most of the reports include recipients receiving cyclosporine as the primary immunosuppressant. Using calculated glomerular filtration rate (cGFR), creatinine cle...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/j.1399-3046.2005.00381.x
更新日期:2005-10-01 00:00:00
abstract::Non-adherence to immunosuppressant medications is an important risk factor for graft dysfunction. To evaluate the effectiveness of adherence-enhancing interventions, we reviewed adherence intervention studies in solid organ transplant recipients (all ages). Using the following databases: PsycINFO, PubMed, Scopus, and ...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/petr.13086
更新日期:2018-02-01 00:00:00
abstract::LDLT is a well-established treatment for most terminal liver diseases in children. Survival rates have improved, yet few studies have considered HRQoL or sleep problems in LDLT recipients. In this cross-sectional study, we enrolled 51 children who had undergone LDLT in Renji Hospital. PedsQL(™) 4.0 Generic Core Scales...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12532
更新日期:2015-08-01 00:00:00
abstract::Children with cholestatic liver disease have been thought to develop hepatic osteodystrophy resulting from vitamin D and calcium malabsorption, resulting in secondary hyperparathyroidism and osteomalacia or rickets. However, treatment with vitamin D has not always proven successful in improving the bone disturbance. T...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.2002.01060.x
更新日期:2002-04-01 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 safe observation time following pediatric renal transplant biopsy is unknown. To predict the safety of day-care pediatric renal transplant biopsy, we retrospectively evaluated the timing, incidence and severity of post-biopsy complications in children observed overnight. Biopsies were performed under real time ult...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2005.00433.x
更新日期:2006-03-01 00:00:00
abstract::To analyze the clinical profile and outcome of pediatric patients who had undergone a liver and/or RT at our center over a five yr period, case records of all the patients who had undergone a liver or RT were analyzed retrospectively. One hundred solid organ transplants were performed at our center between January 200...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12324
更新日期:2014-11-01 00:00:00
abstract::Thrombosis is a major postoperative complication in pediatric liver transplantation. There is marked heterogeneity in prophylactic antithrombotic therapies used, without established guidelines. This review summarizes current worldwide incidence of thrombotic events and compares antithrombotic therapies in children pos...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/petr.13420
更新日期:2019-06-01 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