Solving the Autism Puzzle: Creating a Broad-Spectrum Tool to Improve Care Delivery in the Clinical Setting.

PURPOSE: The purpose of this quality improvement (QI) project was to develop and implement an assessment tool to identify a patient's specific needs due to autism spectrum disorder (ASD). The use of an individualized plan of care related to sensory and behavioral differences correlates with improved experiences in the perioperative setting for patients with ASD. DESIGN: Mixed methods, pre-post survey, retrospective data comparison. METHODS: Metrics planned to evaluate intervention outcomes included: (1) Comparison of pre and postsurvey data obtained from perioperative staff members following ASD education, (2) Evaluation of the number of behavior response team calls made compared to retrospective data, and (3) Survey response data from families assessing the perceived experience of perioperative stay. FINDINGS: Two hundred and fifty staff members responded to the learning needs survey; 164 in the preperiod and 86 in the postperiod. The perioperative process for these patients improved from the pre- to the postperiod in its ability to meet the needs of patients with autism (P < .001). Overall, respondents rated the sensory aids and the behavioral and sensory assessment tool as very useful (Median = 5, IQR = 2) and stated that they are likely to continue to use the tools in the future when caring for patients with autism (Median = 5, IQR = 1). CONCLUSIONS: The caregivers of study patients felt they had a high level of satisfaction with their surgery or procedure experience. Health care providers also reported increased confidence working with individuals with ASD in the perioperative environment and satisfaction with the intervention program.

Serum Hormone Concentrations in Transgender Youth Receiving Estradiol.

OBJECTIVE: This study aimed to evaluate the serum estradiol levels in gender-diverse youth to compare the efficacy of different estradiol routes in achieving therapeutic blood levels and suppressing serum testosterone levels. METHODS: This was a retrospective chart review of patients who initiated estradiol at an adolescent gender clinic between 2010 and 2019. Data on the route of estradiol administration and antiandrogen use (spironolactone or gonadotropin-releasing hormone agonist) were collected, and laboratory data were analyzed. Scatterplots were used to visualize the relationship between the estradiol dose and testosterone and estradiol laboratory values. RESULTS: A total of 118 patients were included, with a mean (standard deviation [SD]) age of 17.2 (1.6) years. The most common route of estradiol administration was oral only (62.7%), followed by transdermal only (23.7%), multiple routes excluding subcutaneous (8.5%), and any subcutaneous (5.1%). Notable variability was observed in the serum estradiol levels, with means (SDs) of 131.9 (120.4) pg/mL for those on oral estrogen 6 to 8 mg per day, 62.6 (40.3) pg/mL for those on transdermal estrogen 0.1 to 0.15 mg every 24 hours, and 53.6 (42.4) pg/mL for those on subcutaneous estradiol. In patients who received spironolactone, transdermal estradiol was associated with lower testosterone levels than estradiol administered orally or subcutaneously. CONCLUSION: Oral, transdermal, and subcutaneous administrations of estrogen all lead to increased serum estradiol levels and are effective for use in gender-affirming care for youth. Patients on transdermal estrogen tended to have lower serum estradiol levels but also had more suppression of serum testosterone levels.

Association between iron deficit repletion with ferric carboxymaltose relative to iron sucrose in children with inflammatory bowel disease: A retrospective cohort study.

BACKGROUND: Iron deficiency and iron deficiency anemia are common in pediatric inflammatory bowel disease and often require supplementation with iron. There is a paucity of literature regarding optimal iron formulation. The aim of this study is to compare outcomes in pediatric patients with inflammatory bowel disease receiving either iron sucrose or ferric carboxymaltose during inpatient hospitalizations. METHODS: This was a single-center retrospective study of pediatric patients with inflammatory bowel disease admitted for newly diagnosed disease or flare who received either iron sucrose or ferric carboxymaltose. Linear regression was used to assess differences in iron repletion. Longitudinal linear mixed-effects models and generalized estimating equations compared hematologic and iron outcomes 6 months post-iron repletion. RESULTS: Thirty patients received ferric carboxymaltose. Sixty-nine patients received iron sucrose. Baseline hemoglobin and iron deficits were similar in both groups. A larger percentage of iron deficit was repleted in the ferric carboxymaltose group (81.4%) compared with iron sucrose (25.9%) (P < 0.001) with fewer infusions. Cumulative doses of ferric carboxymaltose administered (18.7 mg/kg) were higher than iron sucrose (6.1 mg/kg) (P < 0.001). Hemoglobin increased more quickly with ferric carboxymaltose compared with iron sucrose (P = 0.04 and P = 0.02, respectively). Total iron binding capacity and red cell distribution width levels decreased more over time with ferric carboxymaltose vs iron sucrose (P < 0.01 and P = 0.01, respectively). No adverse effects were seen. CONCLUSIONS: Hematologic and iron parameters responded more quickly with fewer infusions in patients who received ferric carboxymaltose vs iron sucrose. Patients who received ferric carboxymaltose achieved a higher percentage of iron deficit repleted.

Effect of Race and Geography on Patient- and Parent-Reported Quality of Life for Children With Congenital Upper Limb Differences.

PURPOSE: Patient beliefs about health and disability are shaped by many social factors and are a key determinant in their ultimate outcome. We hypothesized that pediatric and parent-reported outcome measures regarding a child's congenital upper limb difference will be affected by geographic location, parent education, sex, ethnicity, race, age, and presence of additional medical comorbidities. METHODS: Patients enrolled in the multicenter Congenital Upper Limb Difference registry were included. Age, sex, race, ethnicity, medical comorbidities, highest level of parental education, area deprivation index, and geographic region were recorded. Patient-Reported Outcomes Measurement Information System (PROMIS) in the pediatric and parent-reported domains of upper extremity, anxiety, pain interference, peer relationships, and depressive symptoms were collected. RESULTS: The only difference between geographic regions in the United States in pediatric and parent-reported PROMIS was that parents in the Midwest reported higher upper extremity function scores in children with upper limb differences than the West. Black patients demonstrated higher scores in parent and child-reported domains of depression, pain, and anxiety, and lower scores in upper extremity function than White and Asian peers. Additionally, children with medical comorbidities also demonstrated worse outcomes in multiple PROMIS domains. There was no difference in scores based on sex, parent education, and ethnicity. CONCLUSIONS: In children with congenital upper limb differences, race and additional medical comorbidities have an impact on patient- and parent-reported PROMIS outcome measures in multiple domains, with Black children and those with additional medical comorbidities scoring lower than their peers. CLINICAL RELEVANCE: As we strive to develop a health care system that provides equitable care to all patients, providers who care for children with upper limb differences should be aware that race and additional medical comorbidities can negatively affect patient- and parent-reported PROMIS outcome measures.

Risk factors for severe acute kidney injury after pediatric hematopoietic cell transplantation.

BACKGROUND: Acute kidney injury (AKI) is common after hematopoietic cell transplantation (HCT) and is associated with poorer outcomes. Risk factors for AKI after pediatric HCT are not fully understood. The study objective was to assess unique risk factors for AKI in the HCT population and evaluate post-HCT AKI patterns. METHODS: We conducted a retrospective cohort study of patients < 21 years of age who underwent HCT at Seattle Children's Hospital/Fred Hutchinson Cancer Center from September 2008 to July 2017 (n = 484). We defined AKI using KDIGO criteria. We collected demographics, baseline HCT characteristics, post-HCT complications, and mortality. Multinomial logistic regression was used to estimate association between AKI and potential risk factors. We used adjusted Cox proportional hazard ratios to evaluate differences in mortality. RESULTS: One hundred and eighty-six patients (38%) developed AKI. Seventy-nine (42%) had severe AKI and 27 (15%) required kidney replacement therapy. Fluid overload was common in all groups and 67% of those with severe AKI had > 10% fluid overload. Nephrology was consulted in less than 50% of those with severe AKI. In multivariable analysis, risk of severe AKI was lower in those taking a calcineurin inhibitor (CNI). Risk of death was higher in severe AKI compared to no AKI (RR 4.6, 95% CI 2.6-8.1). CONCLUSIONS: AKI and fluid overload are common in pediatric patients after HCT. Severe AKI occurred less often with CNI use and was associated with higher mortality. Future interventions to reduce AKI and its associated complications such as fluid overload are approaches to reducing morbidity and mortality after HCT. A higher resolution version of the Graphical abstract is available as Supplementary information.

Analysis of transition from intravenous iron sucrose to ferric carboxymaltose infusions in pediatric patients with intestinal failure.

BACKGROUND: Iron supplementation is required for pediatric patients with intestinal failure (IF). There is a paucity of literature on optimal iron formulation and outcomes in this patient population that requires ongoing supplementation. The aim of this study was to assess outcomes in pediatric patients with IF receiving iron sucrose (IS) vs ferric carboxymaltose (FCM) iron infusions. METHODS: This was a single-center observational cohort study of pediatric patients with IF requiring ongoing intravenous iron supplementation. Patients were transitioned from IS to FCM as iron therapy. Longitudinal linear mixed-effects models and generalized estimating equations were used to compare outcomes, including hematologic, iron, and growth parameters for 12-month treatment duration on each iron formulation. Adverse effects were descriptively summarized. RESULTS: Twenty-three patients were included. Sixteen received IS and later switched to FCM, five received IS only, and two received FCM only. Most patients' etiology of IF was short bowel syndrome (FCM: 81%, IS: 83%). No differences were seen over time for iron, hematologic, and growth metrics between IS and FCM. The median number of infusions over 12 months for those taking IS was 15 (interquartile range [IQR] = 13-26) and 2 for FCM (IQR = 1-2). Asymptomatic hypophosphatemia was noted in both groups. Similar central line-associated bloodstream infection rates were noted. CONCLUSION: IS and FCM infusions both maintained hematologic and iron parameters with no significant difference noted between the two types of iron, though the number of FCM infusions was significantly less. No significant adverse effects were noted.

Risk Factors and Inadequacy of Screening for Sleep-Disordered Breathing in Children with Marfan Syndrome.

The prevalence of obstructive sleep apnea (OSA) is increased in children and adults with Marfan syndrome (MFS) compared to the general population and has been shown to be associated with rapid aortic root dilation and dissection in adults. Early diagnosis and treatment of OSA may decrease long-term cardiac morbidity. We therefore studied the utility of noninvasive OSA screening tools in children with MFS. We hypothesized that youth with MFS would have higher OSA screening scores than the general pediatric population. Subjects with confirmed MFS were recruited from a single pediatric center. Data collected included cardiac history, retrospective polysomnogram (PSG) data, and prospectively collected Pediatric Sleep Questionnaire (SRBD-PSQ) and Epworth Sleepiness Scale (ESS-CHAD) scores. Fifty-one individuals aged 2-21 years old were identified. Nineteen subjects completed the surveys, 53% female, median age 16 years. Of those that completed the survey, mean SRBD-PSQ score was 0.24 ± 0.21 and mean ESS-CHAD was 6.4 ± 3.7. Comparatively, published normative data for pediatric control subjects were 0.24 ± 0.21 for SRBD-PSQ and 5.4 ± 3.7 for ESS-CHAD. In conclusions, youth with MFS had similar OSA screening scores compared to published pediatric controls. Given these findings and high prevalence of OSA in MFS youth, standard questionnaires may not be an appropriate tool for identifying children at risk for OSA in this population. In the absence of evidence-based guidelines, physicians caring for children with MFS should consider referral for PSG, even in the absence of classic symptoms.

Decreased Incidence of Hepatic Artery Thrombosis in Pediatric Liver Transplantation Using Technical Variant Grafts: Report of the Society of Pediatric Liver Transplantation Experience.

OBJECTIVE: To evaluate risk factors for hepatic artery thrombosis (HAT) and examine the long-term outcomes of graft and patient survival after HAT in pediatric recipients of liver transplantation. STUDY DESIGN: Using multicenter data from the Society of Pediatric Liver Transplantation, Kaplan-Meier and Cox regression analyses were performed on first-time pediatric (aged <18 years) liver transplant recipients (n = 3801) in the US and Canada between 1995 and 2016. RESULTS: Of children undergoing their first liver transplantation, 7.4% developed HAT within the first 90 days of transplantation and, of those who were retransplanted, 20.7% developed recurrent HAT. Prolonged warm ischemia times increased the odds of developing HAT (OR, 1.11; P = .02). Adolescents aged 11-17 years (OR, 0.53; P = .03) and recipients with split, reduced, or living donor grafts had decreased odds of HAT (OR, 0.59; P < .001 compared with whole grafts). Fifty percent of children who developed HAT developed graft failure within the first 90 days of transplantation (adjusted hazard ratio, 11.87; 95% CI, 9.02-15.62) and had a significantly higher post-transplant mortality within the first 90 days after transplantation (adjusted hazard ratio, 6.18; 95% CI, 4.01-9.53). CONCLUSIONS: These data from an international registry demonstrate poorer long-term graft and patient survival in pediatric recipients whose post-transplant course is complicated by HAT. Notably, recipients of technical variant grafts had lower odds of HAT compared with whole liver grafts.

Hepatic Histology in Treatment-naïve Children With Chronic Hepatitis B Infection Living in the United States and Canada.

OBJECTIVES: Chronic hepatitis B virus infection is a major cause of morbidity and mortality. The aim of the study is to describe the hepatic histology in children chronically infected with hepatitis B virus living in the United States and Canada. METHODS: Liver biopsies of 134 treatment-naïve children with chronic hepatitis B virus infection were scored for inflammation, fibrosis, and other histological features, and correlated with clinical and laboratory data. RESULTS: Sixty percentage of subjects acquired the infection vertically, 51% were male, and 69% were hepatitis B e antigen-positive at the time of the biopsy. Hepatitis B DNA levels were generally high (mean 7.70 log IU/mL), as was serum alanine aminotransferase (median 120 U/L). Using the Ishak-modified histology activity index scoring system, interface hepatitis was mild in 31%, moderate in 61%, and severe in 6%. Lobular inflammation was mild in 54%, moderate in 29%, and marked in 7%. Portal inflammation was mild in 38% and moderate in 62% of subjects. Eighteen percentage had no fibrosis, 59% had portal expansion without bridging fibrosis, 19% had bridging fibrosis, and 4% had cirrhosis. Alanine aminotransferase positively correlated with inflammation and fibrosis. Neither age, duration of infection, nor Hepatitis B virus DNA levels correlated with fibrosis. Fibrosis-4 index did not correlate with fibrosis but correlated with inflammation. Aspartate aminotransferase/platelet ratio index correlated with both inflammation and fibrosis. CONCLUSIONS: Chronic hepatitis B virus infection results in significant inflammation and fibrosis during childhood. Serum alanine aminotransferase is a strong indicator of the severity and extent of hepatic inflammation and fibrosis.

Implementation of the m-ISHAPED Tool for Nursing Interdepartmental Handoffs.

BACKGROUND: Handoffs can pose risks to patients. LOCAL PROBLEM: Nurses throughout a children's hospital understood patient handoffs differently, resulting in handoff errors (failures) across departments. METHODS: Lean principles were used to modify and implement the ISHAPED (Introduction, Story, History, Assessment, Plan, Error Prevention, and Dialogue) tool. INTERVENTIONS: The team implemented a modified ISHAPED (m-ISHAPED) tool to align the content shared and the expectations for interdepartmental handoff. RESULTS: Improvements were seen in reported patient safety events from 6.84 to 1.57 per 100 patient days (P < .001) and nurse satisfaction from 81.1% to 90.6% (P < .001). CONCLUSIONS: A standardized process for interdepartmental nursing handoff was successfully implemented.

Analysis of rabbit anti-thymocyte globulin vs basiliximab induction in pediatric liver transplant recipients.

Literature is limited comparing induction immunosuppression in pediatric liver transplant (LTx) recipients. This is a single-center, retrospective cohort study of primary pediatric liver transplants at our center between 2005 and 2016 who received either basiliximab (BSX) or rabbit anti-thymocyte globulin (rATG) induction. Maintenance immunosuppression consisted of tacrolimus ± a corticosteroid taper. Exclusions included receipt of an ABO-incompatible graft, retransplantation, and multi-organ transplantation. Primary outcomes were incidence of treated biopsy-proven acute rejection (tBPAR) and PTLD within the first year and infections within 90 days of LTx. Secondary outcomes included graft and patient survival, time to first tBPAR, and incidence of steroid-resistant rejection (SRR) within the first year post-LTx. A total of 136 patients were included in the final analysis of which 57 patients (42%) received BSX induction. Patients who received rATG induction with or without a 2-week corticosteroid taper experienced significantly more tBPAR compared to those who received BSX induction with a 6-month corticosteroid taper (55.7% vs 33.3%, P = .01). There were no differences in the incidence of PTLD, infections, SRR, graft or patient survival, or time to first tBPAR between the two groups. Induction with rATG either with or without a short corticosteroid taper was associated with significantly more tBPAR in primary pediatric LTx recipients when compared to BSX induction with a prolonged corticosteroid taper in the setting of maintenance immunosuppression with tacrolimus.

Blood Pressure Measurement Error in Children: Lessons in Measurement Reliability.

BACKGROUND AND PURPOSE: Blood pressure measurement represents the pressure exerted during heart ejection and filling. There are several ways to measure blood pressure and a valid measure is essential. The purpose of this study was to evaluate the approach to noninvasive blood pressure measurement in children. METHODS: Blood pressure measurements were taken using the automatic Phillips MP30 monitor and compared against Welch Allyn blood pressure cuffs with Medline manual sphygmomanometers. RESULTS: A total of 492 measurements were taken on 82 subjects, and they demonstrated comparability between automatic and manual devices. CONCLUSIONS: Although our study indicated acceptable agreement between automatic and manual blood pressure measurement, it also revealed measurement error remains a concern, with sample size, study protocol, training, and environment all playing a role.

Hepatic Venous Pressure Gradient Measurements in Children: Correlation With Hepatic Histology and Clinical Indicators of Portal Hypertension.

OBJECTIVES: In adults, elevated hepatic venous pressure gradients (HVPGs) are correlated with the degree of liver fibrosis on histopathology and predict worse outcomes including variceal bleeding and death. We aimed to examine the association between HVPG measurements, histopathologic findings, and clinical indicators of portal hypertension in children. METHODS: Utilizing retrospective data from 2 pediatric centers between 2006 and 2015, we identified children who underwent simultaneous HVPG measurement and transjugular liver biopsy. Medical charts were reviewed for histopathology, imaging, endoscopic, and clinical data. RESULTS: Forty-one children (median age 11 years) were included in the analysis with diagnoses of acute hepatitis (n = 15), chronic liver disease (n = 12), hepatic noncirrhotic portal hypertension (n = 4), acute liver failure (n = 3), and nonhepatic causes of portal hypertension (n = 7). Elevated mean HVPG measurements were found in children with acute liver failure (10 mmHg, range 4-12) and chronic liver disease (7 mmHg, range 1-12). HVPG measurements did not correlate with the histological severity of fibrosis (ρ = 0.23, P = 0.14) or portal inflammation (ρ = 0.24, P = 0.29), and no difference was found in HVPG when comparing children with and without a history of variceal bleeding (P = 0.43). CONCLUSIONS: HVPG measurements do not correlate significantly with the degree of hepatic fibrosis on biopsy. Furthermore, HVPG measurements are not associated with the presence of varices or history of variceal bleeding, suggesting the possibility of intrahepatic shunting in children with advanced liver disease. Therefore, unlike in adults, HVPG measurements may not accurately predict children who are at risk of complications from portal hypertension.

Human Herpesvirus-6B Reactivation Is a Risk Factor for Grades II to IV Acute Graft-versus-Host Disease after Hematopoietic Stem Cell Transplantation: A Systematic Review and Meta-Analysis.

Graft-versus-host disease (GVHD) is an important cause of morbidity and mortality after allogeneic hematopoietic cell transplantation (HCT). Many studies have suggested that human herpesvirus-6B (HHV-6B) plays a role in acute GVHD (aGVHD) after HCT. Our objective was to systematically summarize and analyze evidence regarding HHV-6B reactivation and development of aGVHD. PubMed and EMBASE databases were searched using terms for HHV-6, HCT, and aGVHD, yielding 865 unique results. Case reports, reviews, articles focusing on inherited chromosomally integrated HHV-6, poster presentations, and articles not published in English were excluded. The remaining 467 articles were reviewed for the following requirements: a statistical analysis of HHV-6B reactivation and aGVHD was described, HHV-6B reactivation was defined by PCR, and blood (plasma, serum, or peripheral blood mononuclear cells) was used for HHV-6B PCR. Data were abstracted from publications that met these criteria (n = 33). Publications were assigned to 1 of 3 groups: (1) HHV-6B reactivation was analyzed as a time-dependent risk factor for subsequent aGVHD (n = 14), (2) aGVHD was analyzed as a time-dependent risk factor for subsequent HHV-6B reactivation (n = 1), and (3) analysis without temporal specification (n = 18). A statistically significant association (P < .05) between HHV-6B reactivation and aGVHD was observed in 10 of 14 studies (71%) in group 1, 0 of 1 study (0%) in Group 2, and 8 of 18 studies (44.4%) in Group 3. Of the 14 studies that analyzed HHV-6B as a risk factor for subsequent aGVHD, 11 performed a multivariate analysis and reported a hazard ratio, which reached statistical significance in 9 of these studies. Meta-analysis of these 11 studies demonstrated a statistically significant association between HHV-6B and subsequent grades II to IV aGVHD (hazard ratio, 2.65; 95% confidence interval, 1.89 to 3.72; P < .001). HHV-6B reactivation is associated with aGVHD, and when studies have a temporal component to their design, HHV-6B reactivation is associated with subsequent aGVHD. Further research is needed to investigate whether antiviral prophylaxis reduces incidence or severity of aGVHD.

A clinical pathway for the postoperative management of hypocalcemia after pediatric thyroidectomy reduces blood draws.

OBJECTIVES: Postoperative calcium management is challenging following pediatric thyroidectomy given potential limitations in self-reporting symptoms and compliance with phlebotomy. A protocol was created at our tertiary children's institution utilizing intraoperative parathyroid hormone (PTH) levels to guide electrolyte management during hospitalization. The objective of this study was to determine the effect of a new thyroidectomy postoperative management protocol on two primary outcomes: (1) the number of postoperative calcium blood draws and (2) the length of hospital stay. STUDY DESIGN: Institutional review board approved retrospective study (2010-2016). METHODS: Consecutive pediatric total thyroidectomy and completion thyroidectomy ±â€¯neck dissection cases from 1/1/2010 through 8/5/2016 at a single tertiary children's institution were retrospectively reviewed before and after initiation of a new management protocol. All cases after 2/1/2014 comprised the experimental group (post-protocol implementation). The pre-protocol control group consisted of cases prior to 2/1/2014. Multivariable linear and Poisson regression models were used to compare the control and experimental groups for outcome measure of number of calcium lab draws and hospital length of stay. RESULTS: 53 patients were included (n = 23, control group; n = 30 experimental group). The median age was 15 years. 41 patients (77.4%) were female. Postoperative calcium draws decreased from a mean of 5.2 to 3.6 per day post-protocol implementation (Rate Ratio = 0.70, p < .001), adjusting for covariates. The mean number of total inpatient calcium draws before protocol initiation was 13.3 (±13.20) compared to 7.2 (±4.25) in the post-protocol implementation group. Length of stay was 2.1 days in the control group and 1.8 days post-protocol implementation (p = .29). Patients who underwent concurrent neck dissection had a longer mean length of stay of 2.32 days compared to 1.66 days in those patients who did not undergo a neck dissection (p = .02). Hypocalcemia was also associated with a longer mean length of stay of 2.41 days compared to 1.60 days in patients who did not develop hypocalcemia (p < .01). CONCLUSIONS: The number of calcium blood draws was significantly reduced after introduction of a standardized protocol based on intraoperative PTH levels. The hospital length of stay did not change. Adoption of a standardized postoperative protocol based on intraoperative PTH levels may reduce the number of blood draws in children undergoing thyroidectomy.

Pediatric airway study: Endoscopic grading system for quantifying tonsillar size in comparison to standard adenotonsillar grading systems.

SIGNIFICANCE: Current grading systems may not allow clinicians to reliably document and communicate adenotonsillar size in the clinical setting. A validated endoscopic grading system may be useful for reporting tonsillar size in future clinical outcome studies. This is especially important as tonsillar enlargement is the cause of a substantial health care burden on children. OBJECTIVE: To propose and validate an easy-to-use flexible fiberoptic endoscopic grading system that provides physicians with a more accurate sense of the three-dimensional relationship of the tonsillar fossa to the upper-airway. METHODS: 50 consecutive pediatric patients were prospectively recruited between February 2015 and February 2016 at a pediatric otolaryngology outpatient clinic. The patients had no major craniofacial abnormalities and were aged 1 to 16years. Each patient had data regarding BMI, Friedman palate position, OSA-18 survey results collected. For each child, digital video clips of fiberoptic nasopharyngeal, oropharyngeal and laryngeal exams were presented to 2 examiners. Examiners were asked to independently use the proposed Endoscopic tonsillar grading system, the Brodsky tonsillar grading scale, the Modified Brodsky tonsillar grading scale with a tongue depressor, and the Parikh adenoid grading system to rate adenotonsillar hypertrophy. Cohen's Kappa and weighted Kappa scores were used to assess interrater reliability for each of the four grading scales. The Spearman correlation was used to test the associations between each scale and OSA-18 scores, as well as Body Mass Index (BMI). RESULTS: 50 pediatric patients were included in this study (mean age 6.1years, range of 1year to 16years). The average BMI was 20. The average OSA-18 score was 61.7. The average Friedman palate position score was 1.34. Twelve percent of the patients had a Friedman palate position score≥3, which made traditional Brodsky grading of their tonsils impossible without a tongue depressor. All four scales showed strong agreement between the two raters. The weighted Kappa was 0.83 for the Modified Brodsky scale, 0.89 for the Brodsky scale, 0.94 for the Parikh scale to 0.98 for the Endoscopic scale (almost perfect agreement). The Endoscopic scale showed the most consistent agreement between the raters during the study. There was a moderate association between the Parikh adenoid grading system with OSA-18 scores (Spearman's ρ=0.58, p<0.001) compared to a low association of the tonsillar grading systems with OSA- 18 scores. None of the scales correlated with patient BMI. CONCLUSIONS: The proposed Endoscopic tonsillar grading system is as reliable of a method of grading tonsillar size as conventional grading systems. It offers the advantage of allowing for critical evaluation of the tonsils without any anatomic distortion which may occur with the use of a tongue blade. This new validated endoscopic grading system provides a tool for communicating the degree of airway obstruction at the level of the oropharynx regardless of Friedman palate position and may be used in future outcomes projects.