Benefit of Child-to-Parent Kidney Donation

Use of child-to-parent (CTP) kidney donation may be limited because of ethical concerns as well as doubts about its effectiveness. We used the United Network for Organ Sharing database to examine the effectiveness of CTP kidney donation compared with other types of living-related (LD) kidney donation and to cadaveric kidney donation. Data from 56 873 kidney transplants performed between 1988 and 1998 showed significantly greater transplant and patient survival for CTP kidney transplants compared with cadaveric kidney transplants. The average gain in kidney transplant half-life is 3.6 years for a CTP compared with a cadaveric kidney transplant, and it is estimated that this gain for the recipient far outweighs the 1 in 3000 risk of death to the donor associated with kidney donation. We conclude that CTP kidney donation should not be discouraged, and represents a useful source of transplantable kidneys.     

Application of percutaneous nephrolithotomy combined with da Vinci Xi robot in complicated upper urinary tract repair surgery: A case report

Duplex kidney and ureter is a congenital malformation. Few patients present with hydronephrosis caused by obstruction of the ureteropelvic junction of the duplex kidney, but lower kidney calculi caused by a duplex kidney abnormality is rare. This study reports a case of a duplex kidney and ureter complicated by multiple calculi in the duplex lower kidney. Percutaneous nephrolithotomy combined with a da Vinci robot-assisted laparoscopic upper urinary tract reconstruction was performed. The lower ureter was resected, and the lower kidney was preserved. One year after the surgery, a follow-up examination reported satisfactory renal function without hydronephrosis or calculi.     

The autosomal recessive polycystic kidney disease protein is localized to primary cilia, with concentration in the basal body area

Recent evidence suggests that structural and functional abnormalities of primary cilia in kidney epithelia are associated with mouse and human autosomal dominant polycystic kidney disease. To determine whether fibrocystin/polyductin/tigmin (FPC), the protein product encoded by the PKHD1 gene that is responsible for autosomal recessive polycystic kidney disease among human subjects, is also a component of primary cilia in the kidney, antipeptide antibodies to the carboxyl-terminal intracellular domain and amino-terminal extracellular domain of FPC were generated and were characterized with immunoblotting and immuno-light and -electron microscopy. Immunolocalization in normal kidney tissue sections and cultured kidney cells demonstrated that FPC was localized to the primary cilia and concentrated on the basal bodies in both kidney tissue sections and cultured kidney cells. The FPC expression pattern was not altered in kidney cells with Pkd1 mutations. These findings suggest that FPC is a functional and/or structural component of primary cilia in kidney tubular cells. It is proposed that the pathogenesis of autosomal recessive polycystic kidney disease is linked to the dysfunction of primary cilia.     

Increased insulin-like growth factor-II tissue concentrations during compensatory kidney growth in infantile rats

Insulin-like growth factor (IGF)-I and IGF-II serum and kidney tissue concentrations were measured in compensatory kidney growth in infantile and adult rats. We hypothesized that the known switch from IGF-II in fetal life to IGF-I in adult life may be responsible for the different modes of compensatory kidney growth, which are mainly characterized by hyperplasia in infantile rats and hypertrophy in adult rats. While IGF-I serum concentrations increased with age in infantile rats, kidney tissue concentrations of IGF-I showed a similar increase in both age groups after uninephrectomy. In adult rats, serum and kidney tissue concentrations of IGF-II were unchanged by uninephrectomy. In infantile rats, however, a significant increase in both serum and kidney concentrations of IGF-II was observed with a maximum at day 5 after uninephrectomy. To investigate if compensatory kidney growth is dependent on hyperperfusion of the remnant kidney, the left renal artery was clipped in infantile rats. The clipped kidney showed growth retardation despite normal kidney tissue concentrations of IGF-I and IGF-II. The contralateral kidney was enlarged and IGF-II kidney concentrations were elevated. However, animals with one clipped kidney and nephrectomy of the contralateral kidney showed compensatory kidney growth of the clipped kidney combined with increased IGF-II kidney tissue concentrations. We conclude that IGF-II mainly promotes compensatory kidney growth in infantile rats by hyperplasia. Hyperperfusion of the remnant kidney seems to be unnecessary for initiation of compensatory kidney growth.     

Umfrage zur Überkreuznierentransplantation in Deutschland

Due to increasingly better long-term survival rates with dialysis the number of patients with renal failure constantly increases by 4% annually worldwide. Despite great progress in operative and perioperative management as well as improved immunosuppressive drugs, kidney transplantation still faces two major problems. First of all there is a huge deficit of donor organs and secondly the long-term results of the kidney grafts must be improved. One way to relieve this tense situation may be live kidney donation. In many countries not participating in Eurotransplant, especially the United States and Scandinavia, live kidney donation is performed more often than kidney transplantation from deceased donors.Germany implemented a transplantation law in December 1997. This law also regulates living donation, with exclusion of crossover transplantations. Cross-over transplantation is a special variation of live donation for couples who cannot donate/receive in their respective couple constellation. Therefore, the donor of the one couple donates his/her kidney to the recipient of the other couple and vice versa. According to German legislation this is illegal. We performed a study in order to evaluate the opinions of the German kidney transplant centers on crossover kidney transplantations.The majority of the German transplantation centers believe that crossover transplantation is acceptable with regard to ethical and medical concerns. To enable this kind of kidney transplantation the transplantation law would have to be changed. Nevertheless, nationwide polls show that live kidney donation represents only a very small portion of all transplantations taking place. Live kidney donation should be granted higher priority as dialysis triggers psychological and physical damage, especially in children. For many patients live kidney donation is the only chance for early transplantation with an excellent long-lasting kidney graft function.     

Increasing access to kidney transplantation in countries with limited resources: The Indian experience with Kidney Paired Donation

According to the Indian chronic kidney disease registry, in 2010 only 2% of end stage kidney disease patients were managed with kidney transplantation, 37% were managed with dialysis and 61% were treated conservatively without renal replacement therapy. In countries like India, where a well‐organized deceased donor kidney transplantation program is not available, living donor kidney transplantation is the major source of organs for kidney transplantation. The most common reason to decline a donor for directed living donation is ABO incompatibility, which eliminates up to one third of the potential living donor pool. Because access to transplantation with human leukocyte antigen (HLA)‐desensitization protocols and ABO incompatible transplantation is very limited due to high costs and increased risk of infections from more intense immunosuppression, kidney paired donation (KPD) promises hope to a growing number of end stage kidney disease patients. KPD is a rapidly growing and cost‐effective living donor kidney transplantation strategy for patients who are incompatible with their healthy, willing living donor. In principle, KPD is feasible for any centre that performs living donor kidney transplantation. In transplant centres with a large living donor kidney transplantation program KPD does not require extra infrastructure, decreases waiting time, avoids transplant tourism and prevents commercial trafficking. Although KPD is still underutilized in India, it has been performed more frequently in recent times. To substantially increase donor pool and transplant rates, transplant centres should work together towards a national KPD program and frame a uniform acceptable allocation policy.     

Épidémiologie de la maladie rénale chronique chez l’enfant

Major advances have been made in the management of children with chronic kidney disease over the past 30 years. However, existing epidemiology data are primarily from kidney replacement therapy registries, and information available at earlier stages of chronic kidney disease is limited. The incidence and prevalence of chronic kidney disease stages 2 to 5 remain poorly understood. However, rare population-based studies suggest that the prevalence of all-stage chronic kidney disease may be as high as 1% of the pediatric population. Congenital disorders including congenital abnormalities of the kidney and urinary tract and hereditary nephropathies account for one-half to two-thirds of pediatric chronic kidney disease cases in middle and high-income countries, whereas acquired nephropathies seem to predominate in low-income countries. The progression of chronic kidney disease is slower in children with congenital disorders than in those with acquired nephropathy, particularly glomerular disease, resulting in a lower proportion of congenital abnormalities of the kidney and urinary tract as a cause of end-stage kidney disease compared to less advanced stages of chronic kidney disease. The incidence of kidney replacement therapy in the pediatric population ranged by country from 1 to 14 per million children of the same age in 2018 (approximately 8 per million children in France) in patients younger than 20 years. The prevalence of kidney replacement therapy in children under 20 years of age in 2018 ranged from 15-30 per million children in some Eastern European and Latin American countries to 100 per million children in Finland and the United States (56 per million children in France). Most children with end-stage kidney disease initiate kidney replacement therapy with dialysis (more frequently hemodialysis than peritoneal dialysis). In about 20% of cases, the initial kidney replacement therapy modality is a pre-emptive kidney transplantation. In high-income countries, 60-80% of prevalent children with end-stage kidney disease live with a functioning transplant (75% in France). While the survival of children with chronic kidney disease has continuously improved over time, mortality remains about 30 times higher than in the general pediatric population.     

NF-κB相关信号通路在肾缺血-再灌注损伤中作用的研究进展

肾缺血-再灌注损伤（IRI）是肾移植和肾部分切除术后预后不佳的主要原因,同时也是急性肾损伤的重要病理生理过程,因此,肾IRI的防治对于改善肾移植预后具有重要意义。然而,IRI的机制较为复杂,其具体机制尚未明确。炎症反应作为IRI主要发病机制之一,在IRI导致的肾损伤中具有重要意义。核因子（NF）-κB作为一种快速反应转录因子,被证实在肾IRI中参与炎症反应的调控。因此,本文将从NF-κB的结构组成、NF-κB信号通路的激活途径及肾IRI中NF-κB上游信号通路和下游信号通路的调控机制进行综述,探讨NF-κB相关信号通路在肾IRI中的作用,为肾IRI的防治提供新的临床思路。     

Subclinical celiac disease and crystal-induced kidney disease following kidney transplant

Decreased kidney function from kidney deposition of calcium oxalate has been described previously in inflammatory bowel disease and after jejuno-ileal and Roux-en-Y gastric bypass surgeries. Although celiac disease is the most prevalent bowel abnormality associated with intestinal malabsorption, its relationship to high kidney oxalate burden and decreased kidney function has not been established. We report a case of subclinical celiac disease and hyperoxaluria that presented with loss of kidney function as a result of high oxalate load in the absence of overt diarrhea, documented intestinal fat malabsorption, and nephrolithiasis. Subclinical celiac disease is commonly overlooked and hyperoxaluria is not usually investigated in kidney patients. We propose that this entity should be suspected in patients with chronic kidney disease in which the cause of kidney damage has not been clearly established.     

7 cases of congenital multicystic kidney with special reference to its embryogenesis

We have experienced 7 cases of multicystic kidney. The latest two cases, etiologically of interest, are reported herein with special reference to its embryogenesis. Case 6: A 4-year-old girl was referred to our clinic for further evaluation of mild azotemia and nonvisualization of left kidney. Left kidney was strongly thought to be multicystic kidney from abdominal CT, whereas her contralateral kidney exhibited hydrocalycosis resembling infundibular stenosis with diminished calyceal numbers. Nephrectomy of her left kidney was performed and histological studies confirmed renal dysplasia (primitive duct, metaplastic cartilage, etc.). Case 7: A 7-year-old girl was referred to Hakodate Kyokai Hospital for the evaluation of azotemia and low stature. Her right kidney was not visualized on IVP and her left kidney exhibited hydronephrosis with diminished calyceal numbers. Her right kidney was diagnosed as multicystic kidney on CT-scan. Pyeloplasty of her left kidney was performed. Multicystic kidney is a rather rare congenital disease. Association of various anomalies in contralateral kidney has been emphasized as well as the notion that infundibulopelvic stenosis is a linked in the clinical spectrum extending from cystic dysplasia of the kidney to hydronephrosis. Our last two cases seem to be included in this category of obstruction theory. As shown by microdissection technique (Potter), however, severe ampullary inhibition early in fetal life is also an attractive hypothesis. Diminished calyceal number of contralateral kidneys seen in our recent two cases is compatible with possibility of ampullary damage. Recent experimental study also showed that renal dysplasia is not solely caused by simply mechanical obstruction to urinary drainage, even when the obstruction is imposed at an early stage of renal development.     

Kidney injury molecule-1 in kidney disease

Kidney injury molecule-1(KIM-1) is a type I membrane protein, comprising an extracellular portion and a cytoplasmic portion, which is expressed at very low levels in the normal kidney. The extracellular portion can cleave and rapidly enter tubule lumens after kidney injury, and can then be detected in the urine. It has been confirmed that the urine KIM-1 level is closely related to tissue KIM-1 level and correlated with kidney tissue damage. Not only is KIM-1 proven to be an early biomarker of acute kidney injury but it also has a potential role in predicting long-term renal outcome. This review summarizes the relationships between KIM-1 and kidney injury, especially in chronic kidney disease.     

Effect of gender and lean body mass on kidney size in healthy 10-year-old children

When evaluating renal abnormalities, kidney volume is an important parameter. Most reference materials on kidney size in children are based on data from pediatric patients examined for non-uronephrological problems. Renal size is traditionally related to body height, weight, or surface area, but not to body composition. As part of a prospective cohort study we have examined 102 healthy 10-year-old children measuring kidney volume by ultrasonography, body composition by dual energy X-ray absorptiometry, and body height and weight. Boys had significantly larger kidneys than girls. The strongest predictor of kidney volume was lean body mass, overruling height, weight, and surface area. When total kidney volume was related to lean body mass as a ratio, the gender difference in kidney size was no longer significant. A strong negative correlation was found between fat body mass and kidney volume. In conclusion, the strongest predictor of kidney volume in healthy 10-year-old children is lean body mass. The correlation is likely to reflect an association between metabolic active tissue, renal solute load, and kidney volume. We have currently no explanation for the negative correlation between fat body mass and kidney volume. Received: 10 May 2000 / Revised: 5 December 2000 / Accepted: 7 December 2000     

Characteristics of kidney donors and recipients in Iranian kidney market: Evidence from Mashhad

The Iranian model of kidney transplantation is an example of a regulated living unrelated renal donation. In this paper, we collected and analyzed a unique dataset of 436 paired kidney donors and recipients, including their characteristics and the realized price of a kidney in Mashhad. As opposed to the global picture of kidney donation, we find that women are less likely to donate and more likely to receive a kidney. Moreover, the average price of a kidney amounts less than 2 years of work with the minimum level of wage.     

Uromodulin in kidney injury: an instigator, bystander, or protector?

Uromodulin, also known as Tamm-Horsfall protein, is a glycoprotein expressed exclusively by renal tubular cells lining the thick ascending limb of the loop of Henle. Although the physiologic functions of this protein remain elusive, significant progress has been made during the last decade that highlights the importance of uromodulin in the pathophysiology of various diseases, such as medullary cystic kidney disease, urinary tract infections, and nephrolithiasis. Meanwhile, there is renewed interest in the role of uromodulin in kidney injury, both acute and chronic. In this article, we review the existing evidence that supports a role for uromodulin in acute kidney injury, chronic kidney disease, and renal inflammation. Contrary to the conventional view of uromodulin as an instigator in kidney injury, new data from uromodulin knockout mice show a protective role for this protein in acute kidney injury, possibly through downregulating interstitial inflammation. In chronic kidney disease, uromodulin excretion, when adjusted for kidney function, is increased; the significance of this is unclear. Although it has been suggested that uromodulin exacerbates progressive kidney injury, we propose that the elevation in uromodulin secretion is instead reactive to injury and reflects an increase of uromodulin in the renal parenchyma, where it slows the injury process.     

Ask-Upmark kidney with bilateral pelvi-ureteric junction obstruction – A rare entity

Renal segmental hypoplasia (Ask-Upmark kidney) is a congenital disorder, first described by Eric Ask-Upmark in 1929. Habib et al. called it “segmental hypoplasia of the kidney in 1965. Ask Upmark kidney is more in females and present with hypertension or sometimes as recurrent urinary tract infections. Usually unilateral, bilaterally asymmetrical segmental hypoplasia has also been reported. The pathogenesis of Ask-Upmark kidney is controversial, attributing to vesicoureteral reflux (VUR) with intrarenal reflux or possibility of localized developmental arrest. We report a case of two years’ male child presenting as abdominal swelling with respiratory distress. On evaluation he was found to have bilateral pelvi-ureteric junction obstruction and left non-functioning kidney for which he underwent right pyeloplasty and left nephrectomy later on. Histopathology report of left kidney suggestive of segmental renal hypoplasia (Ask-Upmark kidney).     

Ritonavir-induced acute kidney injury: kidney biopsy findings and review of literature

Ritonavir therapy is not generally considered nephrotoxic. We report a case of acute kidney injury secondary to ritonavir, with kidney biopsy demonstrating extensive acute tubular injury. This is the first report of a kidney biopsy and pathology in acute kidney injury associated with ritonavir. A review of published medical literature on the topic is also presented.     

Addressing the epidemic of chronic kidney disease in Australia

SUMMARY:   The Australia Diabetes, Obesity and Lifestyle Study (AUSDIAB) study provided, for the first time in Australia, a snapshot of the prevalence of kidney damage, reduced kidney function, hypertension and diabetes in the adult population. With this information, and the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA) registry, that has recorded kidney failure statistics for many years, the extent of the chronic kidney disease burden in Australia is being better defined. This burden is even more pronounced in the Indigenous population where the incidence of kidney disease and kidney failure is increased several-fold.
Diabetes is the second most common cause of kidney failure among Australians. The number of patients with diabetes accepted to dialysis has doubled in the last 7 years, the mean body weight of patients commencing dialysis has increased 7 kg in the past decade and the mean age at acceptance to dialysis is rising in a linear fashion (presently 60 years). These facts, together with a static transplant rate, all point to the prevalence of dialysis likely staying at or increasing beyond the present yearly growth rate of 6–7%.
The evidence shows that a large proportion of chronic kidney disease patients are dying of cardiovascular risk factors before they reach dialysis or transplantation. There are many gaps in delivering appropriate preventative treatment to these patients. A relatively small reduction in the rise in dialysis numbers that might flow from an effective prevention of progression program, could make a significant impact on the spiralling numbers and associated cost of kidney failure treatment in Australia. We now need to develop and implement a national kidney disease strategy designed to address the whole continuum of chronic kidney disease from its earliest stage right through to dialysis and transplantation.     

Renal transplantation in diabetic patients with or without simultaneous pancreatic transplantation 1986: data from the EDTA Registry

This report summarises the outcome of 90 combined kidney/pancreatic grafts performed in Europe in 1986. Data for the combined kidney/pancreas grafts were obtained by a special questionnaire. The one-year patient and kidney graft survival is compared to the results of a group of 389 patients with diabetic nephropathy on the EDTA Registry data file who received kidney grafts alone. The recipients of combined kidney-pancreas grafts were younger, whereas a greater proportion of males received kidney graft alone. Patient survival at one year after transplantation was similar: 89% in recipients of combined transplants compared to 90% in recipients of kidney grafts alone. Kidney graft survival was 78% at one year for recipients of combined grafts versus 76%. It is concluded that pancreas transplantation has little effect on the fate of concomitant kidney grafts. The procedure should-in experienced hands and in selected patients-be almost as safe as kidney grafting alone.     

Primary hyperoxaluria diagnosed after kidney transplant: A review of the literature and case report of aggressive renal replacement therapy and lumasiran to prevent allograft loss

Primary hyperoxaluria type 1 is a rare inherited disorder caused by abnormal liver glyoxalate metabolism leading to overproduction of oxalate, progressive kidney disease, and systemic oxalosis. While the disorder typically presents with nephrocalcinosis, recurrent nephrolithiasis, and/or early chronic kidney disease, the diagnosis is occasionally missed until it recurs after kidney transplant. Allograft outcomes in these cases are typically very poor, often with early graft loss. Here we present the case of a child diagnosed with primary hyperoxaluria type 1 after kidney transplant who was able to maintain kidney function, thanks to aggressive renal replacement therapy as well as initiation of a new targeted therapy for this disease. This case highlights the importance of having a high index of suspicion for primary hyperoxaluria in patients with chronic kidney disease and nephrocalcinosis/nephrolithiasis or with end stage kidney disease of uncertain etiology, as initiating therapies early on may prevent poor outcomes.     

超声造影在供肾质量评估中的应用

近年来,我国公民逝世后器官捐献数量不断增加,但每年仍有大量的终末期肾病患者等待肾移植,供需不平衡仍是目前影响临床肾移植工作开展的主要问题之一。因此,准确评估供肾质量,充分利用扩大标准供肾具有十分重要的临床意义。超声造影因其安全、便携、实时、可量化等特点,已逐步应用于各类实体器官的检测,其在供肾质量评估方面也具有良好的应用前景。本文将从当前供肾评估方法的优势与局限性及超声造影应用于供肾评估的现状及优势进行综述,探讨超声造影在供肾质量评估中的应用前景,增加供肾评估的方法和准确性,为合理利用扩大标准供肾提供参考。