Differential in Kidney Graft Years on the Basis of Solitary Kidney, Simultaneous Liver-Kidney, and Kidney-after-Liver Transplants.

BACKGROUND: The number of simultaneous liver-kidney (SLK) transplants has significantly increased in the United States. There has also been an increase in kidney-after-liver transplants associated with 2017 policy revisions aimed to fairly allocate kidneys after livers. SLK and kidney-after-liver candidates are prioritized in allocation policy for kidney offers ahead of kidney-alone candidates. METHODS: We compared kidney graft outcomes of kidney-alone transplant recipients with SLK and kidney-after-liver transplants using paired kidney models to mitigate differences among donor risk factors. We evaluated recipient characteristics between transplant types and calculated differential graft years using restricted mean survival estimates. RESULTS: We evaluated 3053 paired donors to kidney-alone and SLK recipients and 516 paired donors to kidney-alone and kidney-after-liver recipients from August 2017 to August 2022. Kidney-alone recipients were younger, more likely on dialysis, and Black race. One-year and 3-year post-transplant kidney graft survival for kidney-alone recipients was 94% and 86% versus SLK recipients 89% and 80%, respectively, P < 0.001. One-year and 3-year kidney graft survival for kidney-alone recipients was 94% and 84% versus kidney-after-liver recipients 93% and 87%, respectively, P = 0.53. The additional kidney graft years for kidney-alone versus SLK transplants was 21 graft years/100 transplants (SEM=5.0) within 4 years post-transplantation, with no significant difference between kidney-after-liver and kidney-alone transplants. CONCLUSIONS: Over a 5-year period in the United States, SLK transplantation was associated with significantly lower kidney graft survival compared with paired kidney-alone transplants. Most differences in graft survival between SLK and kidney-alone transplants occurred within the first year post-transplantation. By contrast, kidney-after-liver transplants had comparable graft survival with paired kidney-alone transplants.

A comparison of cytoreductive and non-cytoreductive management strategies for nephron-sparing approaches to tumours in solitary kidneys.

INTRODUCTION: Tumours in a solitary kidney pose challenges in management. Metastatic tumours and those in difficult locations complicate treatment further. The advent of immunotherapy has shed new light on the management of such tumours. We present a series of three cases treated with nephron-sparing surgery following neoadjuvant immunotherapy and compare the outcomes with patients who underwent robotic partial nephrectomy in a solitary kidney. METHODS: We present the outcomes of three patients with solitary kidney tumours who underwent delayed nephron-sparing surgery following good response to immunotherapy. All patients had solitary kidney following a previous nephrectomy, two of which were nonmetastatic but, due to size/location, not amenable to primary treatment; the third patient had metastatic disease and responded to immunotherapy. Two patients underwent robotic partial nephrectomy and one opted for cryotherapy. We compared the preoperative, intraoperative and postoperative parameters of the two patients who underwent robotic cytoreductive partial with patients who underwent robotic partial nephrectomy in a solitary kidney. RESULTS: Out of 231 partial nephrectomy patients in our centre, 2 underwent cytoreductive partial nephrectomy and 5 underwent solitary partial nephrectomy. There was no statistically significant difference in the patient demographics in the two groups. Patients in both groups had comparable operative time, warm ischaemia time, blood loss and length of stay. Two of the five patients in the non-cytoreductive robotic partial nephrectomy had Clavien Dindo 1 complications compared with one patient in the robotic cytoreductive partial nephrectomy group. This was not statically significant. CONCLUSION: Neoadjuvant immunotherapy can play a valuable role in shrinking renal tumours in solitary kidneys to facilitate robotic partial nephrectomies. There were no significant differences in the intra- and postoperative parameters in patients who underwent cytoreductive partial nephrectomy when compared with patients undergoing robotic solitary partial nephrectomy.

Maternal PKU: Defining phenylalanine tolerance and its variation during pregnancy, according to genetic background.

BACKGROUND AND AIMS: Phenylketonuria (PKU)-affected women may become pregnant, and dietary phenylalanine (Phe) intake must be adjusted according to Phe tolerance. We report our experience with maternal PKU in relation to genotype PKU heterogeneity. METHODS AND RESULTS: A total of 10 pregnancies in 7 PKU women (7 different genotypes) were followed up as part of personalized care. Phe tolerance during preconception and pregnancy was assessed by strict dietary control and weekly Phe measurement (blood spots) in relation to genotype. Most women had stopped PKU diet during childhood or adolescence and six pregnancies were unplanned; a phenylalanine-restricted diet was reinstituted soon after conception. Women were classified according to their Phe levels at birth screening and genotype. Phe tolerance increased systematically in the course of pregnancy in all cases, but the increase was different in subjects with classic PKU (cPKU) when compared with cases with mild hyperphenylalaninemia (mHPA), both on average (+297 mg/day in cPKU vs. 597 in mHPA; P = 0.017) and as percentage (+107% in cPKU vs. +17% in mHPA). Notably, Phe tolerance also varied in the same women in the course of different pregnancies, when body weight gain was also different. Two newborns from the same cPKU mother (unplanned pregnancies on free diet) were affected by congenital alterations. CONCLUSIONS: Several factors influence metabolic phenotype in maternal PKU, to an unpredictable extent even in the same woman. The number of maternal PKU cases is growing in dedicated Nutrition Units, and the burden associated with careful management of this condition for the health care system should be adequately considered.

Towards adulthood with a solitary kidney.

Around 1/1000 people have a solitary kidney. Congenital conditions mainly include multicystic dysplastic kidney and unilateral renal aplasia/agenesis; acquired conditions are secondary to nephrectomy performed because of urologic structural abnormalities, severe parenchymal infection, renal trauma, and renal or pararenal tumors. Children born with congenital solitary kidney have a better long-term glomerular filtration rate than those with solitary kidney secondary to nephrectomy later in life. Acute and chronic adaptation processes lead to hyperfiltration followed by fibrosis in the remnant kidney, with further risk of albuminuria, arterial hypertension, and impaired renal function. Protective measures rely on non-pharmacological renoprotection (controlled protein and sodium intake, avoidance/limitation of nephrotoxic agents, keeping normal body mass index, and limitation of tobacco exposure). Lifelong monitoring should include blood pressure and albuminuria assessment, completed by glomerular filtration rate (GFR) estimation in case of abnormal values. In the absence of additional risk factors to solitary kidney, such assessment can be proposed every 5 years. There is no current consensus for indication and timing of pharmacological intervention.

Pure large cell neuroendocrine carcinoma of the bladder without urological symptoms.

Neuroendocrine carcinoma is one of the uncommonly seen pathologies of the urinary bladder. Macroscopic hematuria is frequently encountered symptom in patients with neuroendocrine carcinoma. We report a 45-year-old man with left solitary kidney and oliguria for five days the development of acute renal failure (ARF) with the impaired general condition. The underlying cause being identified as pure type large-cell neuroendocrine carcinoma of the bladder. Large-cell neuroendocrine carcinoma of the bladder is an uncommon fatal tumor. No macroscopic hematuria or urological symptom was observed in our case. Advanced ectasia was not observed in the kidney, and the patient's clinical status was complicated with ARF. It must not be forgotten that in some bladder tumors, the patient's general condition may be impaired without urological symptoms.

Robot-assisted partial nephrectomy: systematic review of functional results.

PURPOSE OF REVIEW: Various ischemia type during partial nephrectomy for renal cell cancer (RCC) resulted in different postoperative functional outcomes. Our objective was to systematically review the contemporary literature on robot-assisted partial nephrectomy (RPN) and investigate the association of ischemia type and tumor complexity with postoperative functional outcomes of the operated kidney and overall. RECENT FINDINGS: Forty-five of the 99 reports identified were selected for qualitative analysis. All included studies were observational and nonrandomized. Overall, we found that patients undergoing RPN with zero ischemia and selective artery clamping had a lower decrease in glomerular filtration rates of the operated kidney in comparison to both warm and cold ischemia. This association seems also to play a role in patients with bilateral kidneys harboring complex tumors. SUMMARY: Zero ischemia and selective artery clamping provide the best functional outcomes following robotic partial nephrectomy. This seems to be of particular relevance in patients with single kidney or tumors of high complexity. Whether these changes are statistically or clinically significant cannot be determined within this systematic review.

Renal function up to the 5th decade of life after nephrectomy in childhood: A literature review.

The aim of the present study was to find out if in children ablation of 50% of renal mass may be associated with a progressive renal damage (Brenner's hypothesis). We collected 1035 adult or adolescent survivors who underwent nephrectomy for unilateral oncological or non-oncological causes during childhood. Stratification of all survivors for age revealed that the number of subjects with blood hypertension and/or renal dysfunction (glomerular filtration rate < 90 mL/min per 1.73 m2 ) to be significantly higher in survivors ≥30 years old in comparison with younger patients. Available data on long-term renal function after nephrectomy during childhood support the Brenner's hypothesis.

Congenital unilateral renal agenesis: Prevalence, prenatal diagnosis, associated anomalies. Data from two birth-defect registries.

BACKGROUND: The different mechanisms leading to a solitary kidney should be differentiated because the long-term outcome might be different. The fetal period is the best moment to make a true diagnosis of congenital unilateral renal agenesis (URA). The objective was to determine the prevalence of URA at birth. The secondary objectives were to describe the evolution of sensitivity of prenatal diagnosis over time and the different forms of URA (isolated and associated with other malformations) detected up to 1 year. METHODS: The cases were retrospectively identified through two French population-based birth defect registries (Auvergne and Bas-Rhin) between 1995 and 2013. Stillbirths and fetuses up to 22 weeks of gestation and infants up to 1 year old with URA were included. RESULTS: A total of 177 cases of URA were registered. The prevalence at birth was 4.0/10,000. The overall prenatal prevalence was 3.6/10,000 (isolated URA: 2.8/10,000). URA were isolated (59.9%), associated with isolated contralateral congenital anomaly of kidney or urinary tract (CAKUT) (7.3%) and with other extra-renal anomalies (32.8%). The total proportion of contralateral CAKUT was 15%. Only three cases presented an aneuploidy, prenatally detected and conducting to a termination of pregnancy. The sensitivity of prenatal diagnosis improved over time (from 54.2% in 1995 to 1997 to 95.8% in 2010 to 2013; p = 0.002). CONCLUSION: Our study provides estimates of prevalence of URA at birth. A longitudinal cohort from the antenatal period to puberty should be performed to determine the prognosis of the contralateral kidney among these children with isolated, associated with contralateral CAKUT and URA with extra-renal anomalies. Birth Defects Research 109:1204-1211, 2017. © 2017 Wiley Periodicals, Inc.