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Lymphocele is a well-known postoperative complication after kidney transplantation. The aim of this study was to analyze time trend incidence, risk factors, and outcome of post-transplant lymphocele in a large pediatric cohort. This is a retrospective single institution review of 241 pediatric kidney transplants performed from 2000 to 2013. Etiology of end-stage renal disease, recipient age and gender, transplant year, BMI percentile for age, type of dialysis, living/non-living related donor, acute rejection, and multiple transplantations were analyzed in association with lymphocele formation. Fourteen of 241 (5.81%) children developed a postoperative lymphocele. There has been a reduction in the incidence of lymphocele after 2006 (3.22% vs. 8.55%, p

Mutations in human SIX1 gene cause branchiootorenal or branchiootic syndrome. Six1 deficient mice exhibit uni- or bilateral renal hypoplasia or kidney agenesis. Furthermore a lack of Six1 gene in the ureter leads to hydroureter and hydronephrosis. These murine malformations resemble human kidney and urinary tract congenital anomalies (CAKUT), a group of diseases with a diverse anatomical spectrum which includes duplex collecting system as much as urethra kidney and ureteropelvic anomalies. Our study focuses on whether mutations or deletion of this gene may be associated with nonsyndromic CAKUT. Fifty unrelated patients (13–21 years) with nonsyndromic CAKUT were retrospectively recruited for SIX1 sequence variations analysis, and compared to three subjects without malformative nephrouropathies (controls). SIX1 coding sequence was screened by high resolution melt analysis (HRMA) and by Sanger direct sequencing. A quantitative comparative real-time polymerase chain reaction (PCR) was later performed in order to detect the presence of SIX1 gene deletion. We did not find significant differences in the HRMA melting curves for each of the SIX1 coding exons between patients and controls, as also confirmed by Sanger direct sequencing. Moreover quantitative comparative real-time PCR for SIX1 and data normalization excluded total SIX1 gene deletion in our patients. We did not find sequence variations in SIX1 coding regions or complete gene deletion in our CAKUT population. These results suggest that alterations in these sequences are unlikely to be a major cause of nonsyndromic CAKUT. Nevertheless, further studies are necessary to understand if altered SIX1 expression may play a role in human development of kidney and urinary tract congenital anomalies.

We assessed LUTS at least 12 months after RTx in patients without evidence of lower urinary tract dysfunction (non-urologic) that had been anuric for at least six months before RTx. No bladder recycling was performed before RTx. LUTS were evaluated using a questionnaire. Clinical records were also reviewed. LUTS in anuric patients were compared with those in non-anuric patients. Fourteen anuric patients fulfilled the inclusion criteria. Median age at RTx was 11 (5-21) yr, median duration of anuria before RTx 24 (7-46) months, and median post-RTx follow-up 2.7 (1.9-10.2) yr. Daytime symptoms were exceptional. Nocturia was the most common symptom (10 patients). Only one patient reported symptoms to affect her quality of life. One patient experienced a febrile UTI and none graft failure. LUTS (nocturia) proved unrelated to duration of anuria, length of follow-up, and presence of (nocturnal) polyuria. LUTS were not statistically different in patients anuric and non-anuric before RTx. Non-urologic patients suffer from long-term storage symptoms, particularly nocturia. LUTS, however, do not seem to increase the risks of urinary infections or graft failure and appear to occur irrespective of the presence of anuria before RTx. Bladder recycling before RTx seems unnecessary.

We reviewed our experience with renal transplantation (RTx) in children weighing

The three yr results of a multicenter trial in de novo pediatric KT treated with a proliferative signal inhibitor and low dose CNI are presented. Thirty-seven children (9.1 ± 5 yr old) received basiliximab, cyclosporine A (CyA C2:1400 ng/mL), (MMF C0:1.5-3 μg/mL), and prednisone. Three wk later everolimus was started (C0:5-10 ng/mL), CyA was reduced (C2:600 ng/mL after 90 days 300 ng/mL), and MMF discontinued. During the three-yr period patient and graft survivals were 96%. One patient died for causes unrelated to the immunosuppression. Cumulative acute rejection rate including protocol and indication biopsies was 21.9%. None of the patients had signs of chronic humoral rejection. Incidence of dnDSA was 5%, 11%, and 22% at one, two, and three yr post-transplant, respectively. Mean glomerular filtration rate measured at one yr and three yr post-transplant was 105.5 ± 31 and 110.7 ± 27 mL/min/1.73 m(2), respectively. A growth velocity of 7.7 ± 6.7 cm/yr was achieved with positive catch-up growth. No malignancy or post-transplant lymphoproliferative diseases were diagnosed. In conclusion, the treatment based on basiliximab induction, everolimus, low-dose cyclosporine, and low-dose prednisone leads to good long-term efficacy in de novo pediatric KT recipients.

There is mounting evidence that the quality of organs from cadaver donors may be influenced by events occurring around the time of brain death. Aim of this present study was to analyze the correlation of DGF with brain-dead donor variables in a single-center pediatric population and to evaluate DGF influence on patients- and grafts outcome. End-points of the study were DGF prevalence, DGF donor-related risk factors, graft function, patient- and graft survival rate, respectively, at six, 12, and 24 months FU. The univariate analysis showed that donor age above 15 yr and vascular cause of donor brain death represented risk factors for DGF. The multivariate analysis confirmed as independent risk factors for DGF donor age >15 yr. At six months FU, DGF showed a negative impact on graft function. In conclusion, among all considered brain-dead donor resuscitation parameters, just non-traumatic cause of death turned out to be of impact for DGF. Donor age >15 yr represented the only independent risk factor for prolonged DGF in our series of children. At two-yr FU, DGF showed a transient negative impact on six-month graft function.

Even if kidney graft survival has improved during the last decades, sensitized pediatric patients are an emerging problem. We describe a 17-yr-old male who lost his first graft due to chronic rejection becoming hyperimmunized (CDC PRA 99.61%). A desensitization protocol based on high-dose IVIG, PP, and two Mabthera(®) infusions was performed with minor response (CDC PRA post-desensitization 80%). One month after his second non-living transplant, he developed a biopsy-proven AMR; post-transplant immunological monitoring showed the presence of donor-specific anti-DQ5 antibodies (DSA, MFI 20.000). He received methylprednisolone pulses and 45 PP sessions without clinical response; eculizumab was then used to salvage a kidney undergoing severe PP-resistant rejection. A biopsy performed after the fourth eculizumab infusion showed complete resolution of AMR. Eculizumab infusions were then continued for the first year post-transplantation. Two yr after transplantation, graft function is stable. Anti-C5 therapy may represent an effective therapeutic option in pediatric patients with PP-resistant AMR.

Transplant renal artery stenosis (TRAS) is an increasingly recognised cause of post-transplant hypertension.We retrospectively analysed 216 paediatric renal recipients transplanted between 2001 and 2011 to assess TRAS prevalence and percutaneous transluminal angioplasty (PTA) efficacy. To assess risk factors, we compared children with TRAS with a propensity score-matched cohort of recipients without TRAS.Of the 216 paediatric patients who were transplanted in the study period, 44 were hypertensive (prevalence 20.3 %) and ten presented with TRAS (prevalence 4.6 %, median age at transplantation 14 years, range 6.78-17.36 years). Hypertensive patients without TRAS were prescribed one to two anti-hypertensive agents, whereas patients with TRAS required one to five medications. In the TRAS group, one recipient presented with vascular complications during surgery, and in three patients the graft had vascular abnormalities. TRAS was detected by Doppler ultrasonography (US) performed due to hypertension in nine of the patients with TRAS, but in the tenth case the TRAS was clinically silent and detected by routine Doppler-US screening. TRAS diagnosis was refined using angio-computed tomography or angio-magnetic resonance imaging. All patients underwent PTA without complications. Significant improvement after PTA was observed in the standard deviation scores for blood pressure [3.2 ± 1.4 (pre-PTA) vs. 1.04 ± 0.8 (post-PTA); p = 0.0006) and graft function [creatinine clearance: 69 ± 17.08 (pre-PTA) vs. 80.7 ± 21.5 ml/min/1.73 m(2) (post-PTA); p = 0.006] We observed no significant differences between the two cohorts for cold ischaemia time, recipient/donor weight ratio, delayed graft function, cytomegalovirus infections and acute rejection episodes.Our study reports a low but significant TRAS prevalence among the paediatric patients who were transplanted at our centre in the study period and confirms that PTA is an effective and safe therapeutic option in paediatric renal transplant recipients. Known risk factors do not appear to be related to the development of TRAS.

We retrospectively reviewed the records of 24 consecutive patients undergoing treatment for ureteral complications after RTx in the period 2001-2012 to determine the timing of presentation of the complications, and their open or endoscopic management. Three patients (12%) had a necrosis of the transplanted ureter soon after RTx. All required open urinary diversion in a native ureter. Ten cases (42%) developed ureteral obstruction. Time of presentation was variable mainly in relation to the underlying cause. Endoscopic treatment was successful in two cases with urinary stones and open surgery in two with mid-ureteral obstruction. Six patients had VUJ stenosis, three underwent open reimplantation, whereas temporary double-J stent placement was successfully performed in the remainder. Eleven patients (46%) had VUR. It seldom presented in the first year after RTx. Endoscopic treatment was attempted in all and was successful in all the six cases without vs. only one of the five with lower urinary tract pathology (p = 0.01). Endoscopic treatment is an option in patients with VUR in the absence of lower urinary tract pathology. It is an option also for the treatment of stones and can be attempted in case of VUJ stenosis. Ureteral necrosis always requires open treatment.

Near-infrared spectroscopy (NIRS) is a non-invasive technique designed to study regional oxygenation (rSO(2)) by measuring the absorption of chromophores. This study investigated the role of NIRS in the real-time monitoring of kidney graft perfusion for 72 h post-transplantation.Consecutive children undergoing living related donor (LRD) or deceased donor (DD) kidney transplantation (KTP) were prospectively enrolled between April 2010 and August 2011. Renal rSO(2) values were registered continuously for 3 days and correlated with hourly urine output, serum creatinine, and urinary neutrophil gelatinase-associated lipocalin (u-NGAL).Twenty-four children were included, 6 underwent LRD and 18 DD KTP. Median age was 12.5 years (interquartile range [IQR] 3.5-16.6) and median body weight was 37 kg (IQR 13-49.7). Four patients experienced delayed graft function (DGF). Renal Doppler ultrasound showed normal vascularization patterns in all children. Median basal renal rSO(2) value was 68.8 % (IQR 59.3-76.2), significantly lower than the end-of-period result (83.6 %; IQR 79.2-90.4; p 0.0001). Renal rSO(2) values showed significant correlation with serum creatinine (rs = -0.62; p 0.05) and estimated glomerular filtration rate (eGFR) (rs = 0.64; p 0.05). No correlation was shown between rSO(2) and diuresis. Increased rSO(2) was also found in patients who experienced DGF. u-NGAL exhibited a trend toward a decrease from baseline in both DD and LRD KTPs, with a strong negative correlation with rSO(2).rSO(2) assessed by NIRS strongly correlates with common markers of kidney graft function and perfusion, allowing continuous real-time monitoring of blood flow in renal grafts.

Heterozygous humans for PAX2 mutations show autosomal dominant papillorenal syndrome (PRS), consisting of ocular colobomas, renal hypo/dysplasia and progressive renal failure in childhood. PAX2 mutations have also been identified in patients with isolated renal hypo/dysplasia. Twenty unrelated children and young adults with kidney and urinary tract malformations and no ocular abnormalities were retrospectively recruited for PAX2 mutational analysis. All patients had undergone renal transplantation after end-stage renal disease. We identified two new sequence variations: (i) a deletion causing a frameshift (c.69delC) and (ii) a nucleotide substitution determining a splice site mutation (c.410+5 G/A) by predictive analysis. Therefore, we suggest PAX2 molecular analysis to be extended to all patients with congenital malformations of kidney and urinary tract (CAKUT).