Utilizing RENAL nephrometry in pediatric patients undergoing nephron-sparing surgery for renal tumors: A single-institutional cohort.

INTRODUCTION: RENAL Nephrometry is a complexity score validated in adults with renal tumors and describes the likelihood of complication after partial nephrectomy (PN). Utilization in pediatrics has been limited. Thus, our goal is to quantify inter-rater agreement as well as determine how scores correlate with outcomes. We hypothesize that the RENAL Nephrometry Score is reproducible in children with renal tumors and is related to perioperative and post-operative complications. METHODS: All pediatric patients who underwent PN for a renal mass from 2006 to 2019 were identified. Patient data, operative details, and outcomes were aggregated. Pre-operative CT/MR imaging was anonymized and scored by 2 pediatric radiologists and 2 pediatric urologists using RENAL Nephrometry metrics. Statistical analysis utilized Fleiss' kappa and the intraclass correlation coefficient (ICC). Comparative analyses were performed based on Nephrometry Score <9 and ≥ 9. RESULTS: 28 patients undergoing 33 PN were identified. Median age at surgery was 3.2 years (IQR 1.8-4.0). There is moderate-good agreement across scorers on the domains of RENAL Nephrometry Score, with the lowest agreement noted for anterior vs posterior tumors. Comparing patients with scores <9 and ≥ 9, there was increased operative time (357 vs 267 min, p = 0.003) and LOS for those with a higher score, but no difference in the incidence of 30-day complications. CONCLUSION: RENAL Nephrometry Score is an easily reproducible complexity score for renal tumors in pediatric patients. Higher scores are associated with increased length of stay and estimated blood loss but not complications. Reporting of nephrometry scores in future publications on pediatric renal tumors should become standard in the literature.

Cognitive functioning in children with atopic dermatitis: Pilot observations.

Atopic dermatitis (AD) is a common and chronic inflammatory skin disease that can adversely affect quality of life and carry significant burdens on physical, emotional, and social health. Recent evidence suggests that AD may also impair cognition, including attention and memory. In a pilot study of six children with AD, we administered a comprehensive battery of assessments to evaluate cognition and behavior and found that this approach was feasible and practical, which will enable the conduct of future larger-scale studies to characterize the impact of AD on cognitive function.

Cutaneous mastocytosis in a child with a de novo GNB1 mutation.

In the last few years, de novo mutations in the GNB1 gene have been found to cause a neurodevelopmental disorder typically characterized by global developmental delay and hypotonia. Only 4 cases of maculopapular cutaneous mastocytosis in children with GNB1 mutations have been reported to date. Here, we describe another case of the condition with concomitant cutaneous mastocytosis.