A Case Report of Kidney-Only Transplantation in Primary Hyperoxaluria Type 1: A Novel Approach with the Use of Nedosiran.

The primary hyperoxalurias (PHs) are a group of diseases characterized by kidney stones, nephrocalcinosis, and chronic kidney disease. At stages of advanced kidney disease, glomerular filtration of oxalate becomes insufficient, plasma levels increase, and tissue deposition may occur. Hemodialysis is often unable to overcome the excess hepatic oxalate production. The current surgical management of primary hyperoxaluria type 1 (PH1) is combined liver kidney transplantation. In a subset of PH1 patients who respond to pyridoxine, kidney-only transplantation has been successfully performed. Recently, kidney-only transplantation has also been performed in PH1 patients receiving a small interfering RNA therapy called lumasiran. This drug targets the hepatic overproduction of oxalate, making kidney-only transplantation a potentially practical novel approach for managing PH1 patients with advanced kidney disease. It is unknown if similar effects could be seen with a different small interfering RNA agent called nedosiran. This article will briefly review PH1, describe the small interfering RNA therapies being used to treat PH, summarize the reported cases of kidney-only transplantation performed with lumasiran, and detail a case of kidney-only transplantation performed in a PH1 patient receiving nedosiran.

Standardizing Blood Pressure Measurements in Pediatric Hemodialysis: a Collaborative Journey.

PURPOSE OF REVIEW: This manuscript details the development and execution of a quality improvement (QI) initiative aimed at standardizing blood pressure (BP) measurement practices in pediatric hemodialysis (HD) units across a national dialysis collaborative. RECENT FINDINGS: Although there are recommendations for the detection and treatment of hypertension in the pediatric population, currently there is no data or recommendations specific to the methodology of measuring blood pressure in a pediatric hemodialysis setting. In 2016, the Standardizing Care to Improve Outcomes in Pediatric End Stage Kidney Disease (SCOPE) Collaborative assembled a dedicated working group to thoroughly examine BP measurement practices across participating pediatric HD centers and, drawing from current research, to establish a standardized best practice for BP measurement in pediatric HD patients both in-center and at home. Employing QI methodology, the working group devised a standardized "BP Bundle" and implemented it throughout the SCOPE Collaborative. This work led to successful practice improvement by establishing a consistent approach to BP measurement in pediatric HD patients cared for in SCOPE centers. With a standard best practice now in place and over 85% compliance with the BP Bundle across the SCOPE Collaborative, researchers and healthcare professionals can more accurately study and ultimately enhance the cardiovascular health of pediatric HD patients.

Abdominal Applications of Pediatric Body MR Angiography: Tailored Optimization for Successful Outcome.

OBJECTIVE. The purpose of this article is to summarize current common techniques and indications for pediatric abdominopelvic MR angiography and strategies for optimizing them to achieve successful outcomes. We also discuss newer MR angiography techniques, including whole-body imaging and blood pool contrast agents, as well as various approaches to reducing the need for anesthesia in pediatric MRI. CONCLUSION. Pediatric body vascular imaging presents a unique set of challenges that require a tailored approach. Emerging pediatric abdominopelvic MR angiography techniques hold promise for continued improvement in pediatric body MR angiography.

De novo leukocyte chemotactic factor 2 amyloidosis in a pediatric renal allograft, 15 years post-transplant.

Leukocyte chemotactic factor 2 amyloidosis (ALECT2) is a recently described form of systemic amyloidosis, which most commonly affects the kidney and liver. The LECT2 protein is produced during inflammatory processes, but its precise function in renal diseases in unclear. ALECT2, however, is known to be a relatively common form of renal amyloidosis, after amyloid light chain and serum amyloid A types and is most often seen in patients of Hispanic ethnicity. ALECT2 can occur de novo or as recurrent disease in kidney transplants. We present the first case, to our knowledge, of de novo ALECT2 in a pediatric kidney transplant patient, 15 years post-transplant.

Pediatric renal transplant biopsy with ultrasound guidance: the 'core' essentials.

This review provides a comprehensive and practical approach to pediatric percutaneous renal transplant biopsies, highlighting techniques and strategies to optimize adequate sample yield and ensure patient safety. In children with end-stage renal disease, transplantation is the preferred choice of therapy, providing for overall lower long-term morbidity and mortality compared with dialysis. In the ongoing management of renal transplant patients, core tissue sampling via a percutaneous renal biopsy remains the gold standard when transplant dysfunction is suspected. Indications for renal transplant biopsy and techniques/tools for adequate sample yield are discussed. Strategies for common challenges such as poor visualization and renal transplant mobility are addressed. We discuss the clinical signs, techniques and imaging findings for common complications including hematomas, arteriovenous fistulas and pseudoaneurysms. Although the percutaneous renal transplant biopsy procedure is generally safe with rare complications, care must be taken to ensure major complications are promptly recognized and treated. Adequate tissue samples obtained via renal biopsy are imperative to promptly identify transplant rejection to provide valuable information for patient diagnosis, treatment and outcomes. Radiologist and nephrologist attention to proper ultrasound techniques and optimal biopsy tools are critical to ensure tissue adequacy and minimize complications.

Intravoxel incoherent motion analysis of renal allograft diffusion with clinical and histopathological correlation in pediatric kidney transplant patients: A preliminary cross-sectional observational study.

The purpose of this study was to compare IVIM values in pediatric renal transplants with histopathology and clinical management change. Fifteen pediatric renal transplant recipients (mean 15.7±2.9 years) were prospectively scanned on a 3T MR scanner with multi-b DTI, prior to same-day transplant biopsy. IVIM maps from 14 subjects were analyzed (one excluded due to motion). Mean values were computed from cortical ROIs and medullary ROIs corresponding to the biopsy site. Subjects were also grouped according to whether or not the biopsy resulted in a change in clinical management. Cortico-medullary IVIM estimates and histopathologic Banff scores were correlated with KT. Cortico-medullary IVIM differences between the "change" and "no change" groups was compared with Mann-Whitney U test. Cortical Dp showed significant moderate negative correlation with Banff t and ci scores (KT=-0.497, P=.035 and KT=-0.46, P=.046) and moderate positive correlation with Banff i score (KT=0.527, P=.028). Cortical Pf showed significant moderate correlation with ci and ct scores (KT=0.489, P=.035 and KT=0.457, P=.043). Tissue diffusivity, Dt , estimated with IVIM was significantly different between the "change" and "no change" groups in medullary ROIs (U=6, P=.021). IVIM analysis has potential as a noninvasive biomarker in assessment of pediatric renal allograft pathology.

Pilot Study of Renal Diffusion Tensor Imaging as a Correlate to Histopathology in Pediatric Renal Allografts.

OBJECTIVE: Fractional anisotropy (FA) is a measure of molecular motion obtained from diffusion tensor imaging (DTI). The objective of this study was to assess the use of FA as a noninvasive correlate of renal allograft histopathology. SUBJECTS AND METHODS: Sixteen pediatric renal allograft recipients were imaged using DTI in a prospective study, between October 2014 and January 2016, before a same-day renal allograft biopsy. The Kendall tau correlation coefficient was used to assess the relationship between cortical and medullary FA values and several clinically important Banff renal allograft histopathology scores. The Mann-Whitney U test was also used to compare cortical and medullary FA values in the region of biopsy in patients whose biopsy results did and in those whose biopsy results did not change clinical management. RESULTS: Medullary FA values had direct inverse correlation with several histopathology scores: tubulitis (designated "t" score in Banff pathologic classification, p < 0.04), interstitial inflammation (i score, p < 0.005), tubular atrophy (ct score, p < 0.002), and interstitial fibrosis (ci score, p < 0.007). Cortical FA values inversely correlated with peritubular capillaritis (ptc score, p < 0.02). Neither medullary nor cortical FA values correlated with glomerulitis (g score). At a b value of 800 s/mm2, medullary FA values of pediatric renal allograft recipients whose renal biopsies prompted a change in clinical management (mean ± SD at a b value of 800 s/mm2 = 0.262 ± 0.07; n = 9) were statistically different compared with the group whose biopsy results did not change clinical management (mean ± SD at a b value of 800 s/mm2 = 0.333 ± 0.06; n = 7) (p < 0.006). CONCLUSION: FA is a noninvasive correlate of several important renal allograft histopathology scores and a potential noninvasive method of assessing renal allograft health in pediatric allograft recipients.

Challenges managing end-stage renal disease and kidney transplantation in a child with MTFMT mutation and moyamoya disease.

Moyamoya disease is a chronic cerebrovascular disorder with progressive stenosis. We describe a four-yr-old female with features of moyamoya disease referred to our center for kidney transplant evaluation with ESRD secondary to presumed renal dysplasia along with concern for cerebral vascular anomalies. With her constellation of organ involvement, a genetic workup revealed a homozygous, frameshift mutation in the mitochondrial methionyl-tRNA formyltransferase gene. Given her vascular anomalies and evidence of prior infarcts seen on cerebral imaging, it was felt that her risk of future stroke events was high and that hypotension or intravascular volume depletion would further exacerbate this risk. In hopes of improving her tenuous cerebral perfusion, she underwent a bilateral temporal craniotomy for superficial temporal artery to middle cerebral artery bypass. We highlight the challenges faced in a child with ESRD and kidney transplantation when cerebral vasculature is compromised. A multidisciplinary approach is critical in determining the need for a revascularization procedure prior to transplant and to help reduce the risk of ischemic or hemorrhagic events in this patient population.

Cathinone preservation in khat evidence via drying.

A primary concern with the forensic analysis of the khat plant (Catha edulis) has been the need to preserve the principle psychoactive component, cathinone, which converts to the less-active substance, cathine, after harvesting. The loss of cathinone has serious legal implications since it is a Schedule I controlled substance under federal regulations in the United States, while cathine is Schedule IV. A common misconception is that cathinone is highly unstable once the plant is harvested, and may be undetectable upon drying and prolonged storage. However, drying the plant material will preserve cathinone. Numerous seizures of a dried form of khat (referred to as "graba" in the United States) have been made in recent years, suggesting that drying the plant material is a viable approach to preserve khat evidence for both storage and reanalysis. A qualitative and quantitative study of the composition of khat samples seized as dried plant material has found the khat alkaloids to be relatively stable for a monitored period of 3 years, and cathinone has remained identifiable while stored at room temperature for over 10 years. Studies of green khat (received moist) have also determined that drying the moist leaves at either room temperature or by the application of heat are suitable methods to preserve cathinone in the dried material. These findings demonstrate that cathinone persists in dried khat for a time frame of several years, and simple drying techniques are an effective means to preserve seized khat evidence for long-term storage.

Humming is as effective as Valsalva's maneuver and Trendelenburg's position for ultrasonographic visualization of the jugular venous system and common femoral veins.

STUDY OBJECTIVE: The purpose of this study is to compare ultrasonographic visualization of the jugular and common femoral veins by using a novel technique (humming) and 2 conventional techniques (Valsalva's maneuver and Trendelenburg's position). The Valsalva's maneuver and Trendelenburg's position are common methods for producing venous distention, aiding ultrasonographically guided identification and cannulation of the jugular and common femoral veins. We hypothesize that humming is as effective as either Valsalva's maneuver or Trendelenburg's position for distention and ultrasonographic visualization of these procedurally important blood vessels. Herein, we investigate a new method of venous distension that may aid in the placement of central venous catheters by ultrasonographic guidance. METHODS: Healthy, normal volunteers aged 28 to 67 years were enrolled. Each subject's internal jugular, external jugular, and common femoral veins were measured in cross-section by ultrasonograph during rest (baseline), humming, Valsalva's maneuver, and Trendelenburg's position. Three measurements were recorded per observation in each position. Subjects were used as their own controls, and measurements were normalized to percentage increase in diameter during each maneuver or position for later comparison. RESULTS: The study population consisted of 7 subjects, with a mean age of 47 years. Cross-sectional area was calculated for each vessel in 3 groups: baseline/control, Valsalva, Trendelenburg, and humming. The mean percentage change (+/-SD) relative to baseline cross-sectional area of the jugular vessels for each subject were external jugular vein: humming 134%+/-25% (95% confidence interval [CI] 124.9% to 146.9%), Valsalva 136%+/-23% (95% CI 121.3% to 147.5%), Trendelenburg 137%+/-32% (95% CI 120.7% to 156.9%); internal jugular vein: humming 137%+/-27% (95% CI 119.4% to 148.2%), Valsalva 139%+/-24% (95% CI 122.4% to 148.7%), Trendelenburg 141%+/-35% (95% CI 116.5% to 156.5%); common femoral vein: humming 131%+/-15% (95% CI 120.4% to 139.1%), Valsalva 139%+/-18% (95% CI 127.9% to 150.4%), Trendelenburg 132%+/-24% (95% CI 113.3% to 142.9%). CONCLUSION: All 3 maneuvers distended the external jugular, internal jugular, and common femoral veins compared to baseline. There was no important difference in magnitude of cross-sectional area between any of the 3 maneuvers when compared with one another. Humming shares many physiologic similarities to Valsalva's maneuver and may be more familiar and easier to perform during procedures such as ultrasonographically guided central venous catheter placement and insertion of external jugular intravenous catheters.