Background
Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of renal failure. For several decades, ADPKD was regarded as an adult-onset disease. In the past decade, it has become more widely appreciated that the disease course begins in childhood. However, evidence-based guidelines on how to manage and approach children diagnosed with or at risk of ADPKD are lacking. Also, scoring systems to stratify patients into risk categories have been established only for adults. Overall, there are insufficient data on the clinical course during childhood. We therefore initiated the global ADPedKD project to establish a large international pediatric ADPKD cohort for deep characterization.
Methods
Global ADPedKD is an international multicenter observational study focusing on childhood-diagnosed ADPKD. This collaborative project is based on interoperable Web-based databases, comprising 7 regional and independent but uniformly organized chapters, namely Africa, Asia, Australia, Europe, North America, South America, and the United Kingdom. In the database, a detailed basic data questionnaire, including genetics, is used in combination with data entry from follow-up visits, to provide both retrospective and prospective longitudinal data on clinical, radiologic, and laboratory findings, as well as therapeutic interventions.
Discussion
The global ADPedKD initiative aims to characterize in detail the most extensive international pediatric ADPKD cohort reported to date, providing evidence for the development of unified diagnostic, follow-up, and treatment recommendations regarding modifiable disease factors. Moreover, this registry will serve as a platform for the development of clinical and/or biochemical markers predicting the risk of early and progressive disease.
6 2 3 WEDNESDAY Supplement to Transplantation July 27, 2008, Volume 86 Number 2S sensitized with panel reactive antibody greater than ninety-five percent. She remained on dialysis for four years. After pre-transplant desensitization with high dose IVIG (2g/kg once a month for four months) and Rituximab 750mg/m2 for two doses, she received a cross-match negative, deceased donor transplantation. She was induced with anti-thymocyte globulin and continued on prednisone, mycophenolate mofetiel and tacrolimus. She maintained good allograft function until post-operative day ten when she presented with an acute rise in creatinine and anuria. A biopsy revealed acute cellular type 2 and antibody-mediated rejection diagnosed by complement 4d (C4d) positive staining. She was treated with plasmapheresis and IVIG. Serial donor specific HLA antibody measurements remained negative. An extended search for a non-HLA antibody detected donor specific *012 MICA antibody. Donor specificity was confirmed by MICA genotyping of the patient and donor. Serial anti-MICA antibody titers were measured using luminex technology using median fluorescence intensity. (See Figure one) Results: Pre-transplant serum revealed the existence of pre-formed *012 MICA antibody, with an elevated level at pre-transplantation and at the time of rejection. Resolution of rejection and return of patient's graft function correlated well with the decline in MICA antibody titer following treatment with plasmapheresis and IVIG (see fi gure one). Interestingly, the MICA antibody titer increased during an episode of acute gastroenteritis, but continued to decline following the resolution of the illness and continuation of treatment with plasmapheresis and IVIG.
Conclusions:Our case emphasizes the importance of screening for donor specifi c MICA antibodies in re-transplant recipients, specifi cally when donor-specifi c anti-HLA antibodies are not detected during rejection. Current desensitization protocols using high dose IVIG and Rituximab may not effectively decrease MICA antibodies. Therefore, preformed MICA antibodies can lead to both acute antibody mediated and acute cellular rejection (type 2). Plasmapheresis followed by high dose IVIG may be an effective treatment of acute rejection caused by MICA antibodies. Background: The recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation has a potentially detrimental impact leading to the loss of renal function. Although, plasmapheresis (PF) and rituximab are commonly recommended the treatment is still a matter of debate. Aim: We report our single-center experience to assess the outcome of the renal transplantation in children with FSGS. Patients and methods: Medical records of 10 (F/M: 4/6) renal transplanted patients with FSGS were evaluated. Among 10 grafts 7 were from living related and 3 from deceased donor. The original diagnosis of FSGS as well as recurrences was biopsy-proven in all patients. All patients treated with calcineurin-based immunosuppressive therapy. PF was done at days -3...
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