Kidney transplantation in children with bladder augmentation or ileal conduit diversion.

INTRODUCTION: Various congenital and acquired diseases of the lower urinary tract can lead to chronic renal failure requiring renal replacement therapy. AIM: The aim of the study was to assess problems and results of kidney transplantation in children with significant lower urinary tract dysfunction. MATERIALS AND METHODS: Between 1984 and 2007, there were 33 kidney transplantations in children with end-stage renal disease and severe lower tract dysfunction out of 539 kidney transplantations performed in our department. The patients were 23 males and 10 females. Thirty patients received a kidney from a deceased donor, the remaining 3 from a living related donor. The age at transplantation ranged from 2.25 years to 19 years. In 26 patients an ileal conduit modo Bricker was created (in 21 patients at transplant operation). Bladder augmentation was performed in 6 patients and a continent urinary reservoir was created in 1. RESULTS: Post-transplant follow-up ranged from 7 to 88 months (mean 32 months). Overall patient survival is 100% and graft survival is 97%. Creatinine concentrations ranged from 0.3 to 3.4 mg% (mean 0.92 mg%). Surgical complications were diagnosed in 16 patients. All surgical complications were treated successfully and none of them caused graft loss. Urinary tract infections (UTI) were the most commonly observed complication, occurring in 26/33 (78%) patients, but the majority of these UTI were asymptomatic and had no influence on graft function. CONCLUSIONS: Kidney transplantation in children with lower urinary tract dysfunction and end-stage renal failure offers excellent medium term results in our experience, despite the creation of non-standard urinary drainage. Recurrent urinary tract infections are the most common complications in these patients, but in the majority of cases this does not lead to impairment of graft function.

[Familial, recurring plasma infusion dependent thrombotic thrombocytopenic purpura/hemolytic uremic syndrome]. / Rodzinna, nawracajaca, zalezna od wlewów osocza, zakrzepowa plamica maloplytkowa z zespolem hemolityczno-mocznicowym.

Two siblings with chronic relapsing TTP/HUS from early childhood are presented. An onset of the disease was probably neonatal period and it did not involved the organ until puberty. During puberty the course of the disease aggravated with renal involvement and became fulminant in a girl who died disseminated TTP. Her older brother had episode of severe atypical HUS responding to plasma therapy. Now, his renal function is normal but the symptoms are depend an regular, prophylactic plasma infusions.

Primary vascular thrombosis after renal transplantation in children.

The aim of the study was to assess the incidence, causes, diagnostic and treatment modalities, and outcome of vascular thrombosis after kidney transplantation in children. Between 1984 and 1995 we performed 176 kidney transplants in pediatric recipients aged 1 to 18 years. Vascular thrombosis followed 7 transplants, 4 were renal vein and 3 arterial thromboses. Venous thromboses occurred 2 to 12 days after transplantation. All of the patients with a renal vein thrombosis lost their grafts. Arterial thrombosis developed in 2 cases of double renal arteries which were separately anastomosed into the recipient vessels. One graft was lost, but the other was saved by thrombolytic therapy (streptokinase). One child experienced intrarenal segmental artery thrombosis during acute vascular rejection, which resolved following combined anti-rejection and thrombolytic (intra-arterial streptokinase) treatment with full recovery of graft function. In all, vascular thrombosis complicated 7 out of 176 transplants (4.0%), and was the cause of 5 graft losses (2.8%). The incidence of vascular thrombosis was not increased in grafts with vascular anomalies (3/34 v. 4/142; p>0.05, chi sq.). We conclude that acute tubular necrosis, rejection and unstable volemia may predispose to vascular thrombosis. In selected cases, early diagnosis of vascular thrombosis may enable graft salvage by surgical or thrombolytic treatment.