Multi‐modality cardiac imaging in advanced chronic kidney disease

Cardiovascular disease (CVD) is the leading cause of death worldwide and is particularly frequent among those with severe renal impairment. Early diagnosis and therapeutic intervention may help alleviate the burden of cardiovascular complication within this population. In the last years, advances have been made toward developing noninvasive imaging techniques that could offer better insight into the cardiac involvement in end‐stage renal disease (ESRD). Conventional transthoracic echocardiography remains the first‐line investigation used to assess cardiac function, but encompassing in our daily practice, the newer approaches such as speckle‐tracking imaging, cardiac computed tomography, or cardiac magnetic resonance can guide us to a more comprehensive understanding of CVD in ESRD. Given that patients with chronic kidney disease may not present with typical CVD symptoms, the amount of information brought by newer imaging techniques is crucial for an accurate diagnosis, risk stratification, and further management. The purpose of this review is to briefly summarize the specific applications of standard cardiac imaging techniques in patients with ESRD and to offer insight into the novel imaging modalities, highlighting the newest research in this field. By doing so, we aim to identify the most important imaging predictors of clinical outcomes in this population.     

A Day in the Life of MRI: The Variety and Appropriateness of Exams Being Performed in Canada

Purpose

This study aimed to determine the volumes and types of magnetic resonance imaging exams being performed across Canada, common indications for the exams, and exam appropriateness using multiple evaluation tools.

Neuroblastoma among children in Southern and Eastern European cancer registries: Variations in incidence and temporal trends compared to US

Neuroblastoma comprises the most common neoplasm during infancy (first year of life). Our study describes incidence of neuroblastoma in Southern–Eastern Europe (SEE), including – for the first time – the Nationwide Registry for Childhood Hematological Malignancies and Solid Tumors (NARECHEM‐ST)/Greece, compared to the US population, while controlling for human development index (HDI). Age‐adjusted incidence rates (AIR) were calculated for 1,859 childhood (0–14 years) neuroblastoma cases, retrieved from 13 collaborating SEE registries (1990–2016), and were compared to those of SEER/US (N = 3,166; 1990–2012); temporal trends were assessed using Poisson regression and Joinpoint analyses. The overall AIR was significantly lower in SEE (10.1/million) compared to SEER (11.7 per million); the difference was maximum during infancy (43.7 vs. 53.3 per million, respectively), when approximately one‐third of cases were diagnosed. Incidence rates of neuroblastoma at ages <1 and 1–4 years were positively associated with HDI, whereas lower median age at diagnosis was correlated with higher overall AIR. Distribution of primary site and histology was similar in SEE and SEER. Neuroblastoma was slightly more common among males compared to females (male‐to‐female ratio: 1.1), mainly among SEE infants. Incidence trends decreased in infants in Slovenia, Cyprus and SEER and increased in Ukraine and Belarus. The lower incidence in SEE compared to SEER, especially in infants living in low HDI countries possibly indicates a lower level of overdiagnosis in SEE. Hence, increases in incidence rates in infancy noted in some subpopulations should be carefully monitored to avoid the unnecessary costs health impacts of tumors that could potentially spontaneously regress.     

Comprehensive characterization of a Canadian cohort of von Hippel-Lindau disease patients

Von Hippel-Lindau disease (VHL) is a heritable condition caused by pathogenic variants in VHL and is characterized by benign and malignant lesions in the central nervous system (CNS) and abdominal viscera. Due to its variable expressivity, existing efforts to collate VHL patient data do not adequately capture all VHL manifestations. We developed a comprehensive and standardized VHL database in the web-based application, REDCap, that thoroughly captures all VHL manifestation data. As an initial trial, information from 86 VHL patients from the University Health Network/Hospital for Sick Children was populated into the database. Analysis of this cohort showed missense variants occurring with the greatest frequency, with all variants localizing to the α- or β-domains of VHL. The most prevalent manifestations were central nervous system (CNS), renal, and retinal neoplasms, which were associated with frameshift variants and large deletions. We observed greater age-related penetrance for CNS hemangioblastomas with truncating variants compared to missense, while the reverse was true for pheochromocytomas. We demonstrate the utility of a comprehensive VHL database, which supports the standardized collection of clinical and genetic data specific to this patient population. Importantly, we expect that its web-based design will facilitate broader international collaboration and lead to a better understanding of VHL.