The effect of surgical time on perioperative complications in adolescent idiopathic scoliosis cases. A propensity score analysis.

BACKGROUND: Posterior spinal instrumentation and fusion (PSIF) for adolescent idiopathic scoliosis (AIS) can be lengthy and complication-ridden. The aim of this study was to evaluate the effect of surgical time on perioperative complications in this procedure when controlling for confounding variables with propensity score analysis. METHODS: This was an IRB-approved review of electronic health records from 2010 to 2019 at a single tertiary care children's hospital. Patients undergoing PSIF were grouped into "short" (< 6 h) or "long" (≥ 6 h) surgical time groups. Outcome measures were estimated blood loss (EBL), cell saver transfusions, packed red blood cell (pRBC) transfusions, length of stay (LOS), intraoperative monitoring (IOM) alerts, hematocrit, ICU transfer, neurologic loss, surgical site infection, and 90-day readmissions. We controlled for age, sex, BMI, curve severity, number of segments fused, and surgeon factors. RESULTS: After propensity score matching there were 113 patients in each group. The short surgical time group had lower EBL (median 715, IQR 550-900 vs median 875, IQR 650-1100 cc; p < 0.001), received less cell saver blood (median 120, IQR 60-168 vs median 160, IQR 97-225 cc; p = 0.001), received less intraoperative pRBCs (median 0, IQR 0-0 vs median 0, IQR 0-320, p = 0.002), had shorter average LOS (4.8 ± 1.7 vs 5.4 ± 2.5 days; p = 0.039), and fewer IOM alerts (4.3% vs 18%, p = 0.003). CONCLUSIONS: Patients with shorter surgical times had less blood loss, received less transfused blood, had a shorter LOS, and fewer IOM alerts compared to patients with longer surgical times. Surgical times < 6 h may have safety and efficacy advantages over longer times. LEVEL OF EVIDENCE: III.

Prevalence of CYP2D6 structural variation in large retrospective study.

CYP2D6 is a highly polymorphic gene with clinically important structural variations. Commonly, only exon 9 is assayed on clinical pharmacogenomics panels, as it allows for accurate functional characterization even in the presence of a CYP2D6::CYP2D7 conversion. However, this method does not capture CYP2D7::CYP2D6 (CYP2D6*13) conversions, possibly leading to inaccurate phenotype assignment. The study's purpose was to determine the frequency of structural variations in CYP2D6 utilizing multiple copy number variation (CNV) assay locations to quantify the potential impact on clinical phenotype classification. A retrospective analysis was conducted of de-identified pharmacogenomics data submitted through the Translational Software, Inc. platform. Samples with CYP2D6 CNV data for exon 9 and at least one additional CNV location (5'UTR, exon 1, intron 2, exon 5 or intron 6) were included. CYP2D7::CYP2D6 and CYP2D6::CYP2D7 conversions were classified according to PharmVar nomenclature. The CYP2D6 copies were capped at four total copies to account for assay limitations in detecting more than four copies. A total of 106,474 samples were included for analysis. CYP2D7::CYP2D6 conversions were present in approximately 2.44% of samples, and 5.84% of samples had CYP2D6::CYP2D7 conversions. Many samples did not have a CYP2D7 conversion detected (91.5%; 97,462/106,474). A full gene deletion was detected in 0.15%, and 5.98% had a duplication or multiplication present. This retrospective study underscores the importance of testing more than one CNV site for CYP2D6 . Over 2% of patients were found to have a CYP2D7::CYP2D6 conversion. This translates into potentially misclassified phenotype classification and incongruent clinical recommendations.

Nudging During Pediatric Intensive Care Conferences With Family Members: Retrospective Analysis of Transcripts From a Single-Center, 2015-2019.

OBJECTIVES: Nudging, a behavioral economics concept, subtly influences decision-making without coercion or limiting choice. Despite its frequent use, the specific application of nudging techniques by clinicians in shared decision-making (SDM) is understudied. Our aim was to analyze clinicians' use of nudging in a curated dataset of family care conferences in the PICU. DESIGN: Between 2019 and 2020, we retrospectively studied and coded 70 previously recorded care conference transcripts that involved physicians and families from 2015 to 2019. We focused on decision-making discussions examining instances of nudging, namely salience, framing, options, default, endowment, commission, omission, recommend, expert opinion, certainty, and social norms. Nudging instances were categorized by decision type, including tracheostomy, goals of care, or procedures. SETTING: Single-center quaternary pediatric facility with general and cardiac ICUs. PATIENTS: None. INTERVENTIONS: None. MEASUREMENTS: We assessed the pattern and frequency of nudges in each transcript. MAIN RESULTS: Sixty-three of the 70 transcripts contained SDM episodes. These episodes represented a total of 11 decision categories based on the subject matter of nudging instances, with 308 decision episodes across all transcripts (median [interquartile range] 5 [4-6] per conference). Tracheostomy was the most frequently discussed decision. A total of 1096 nudging instances were identified across the conferences, with 8 (6-10) nudge types per conference. The most frequent nudging strategy used was gain frame (203/1096 [18.5%]), followed by loss frame (150/1096 [13.7%]). CONCLUSIONS: Nudging is routinely employed by clinicians to guide decision-making, primarily through gain or loss framing. This retrospective analysis aids in understanding nudging in care conferences: it offers insight into potential risks and benefits of these techniques; it highlights ways in which their application has been used by caregivers; and it may be a resource for future trainee curriculum development.

Relationship Between Obesity and Global Longitudinal Strain in the Pediatric Single Ventricle Fontan Population Across Ventricular Morphologies.

BACKGROUND: Obesity is associated with diminished myocardial function as measured by strain echocardiography in children and young adults with normal cardiac anatomy. Data are lacking about the effect of obesity on myocardial strain in patients with a single ventricle. In this study, the relationship between body mass index (BMI) and single ventricle myocardial strain in the Fontan population was assessed. METHODS AND RESULTS: Thirty-eight abnormal BMI Fontan cases (21 overweight and 17 obese) and 30 normal BMI Fontan controls matched based on single ventricular morphology, age, and sex were included in the study. Ventricular morphology was categorized as single right ventricle, single left ventricle, or biventricular. Single ventricle global longitudinal peak systolic strain (GLS) and other echocardiographic measurements were performed and compared between groups, with a P≤0.05 defined as significant. The abnormal BMI group demonstrated diminished GLS (-15.7±3.6% versus -17.2±3.2%, [P=0.03]) and elevated systolic blood pressure (P=0.04) compared with the normal BMI group. On subgroup analysis of those with single right ventricle morphology, the abnormal BMI group demonstrated diminished GLS compared with controls. There was no significant difference in GLS between the abnormal BMI and control groups in the single left ventricle and biventricular subgroups. Analyzed by ventricular morphology, no other variables were statistically different in the abnormal BMI group including systolic blood pressure. Inter-reader reproducibility for GLS and strain rate were excellent for both measures. CONCLUSIONS: Obesity has an adverse relationship with myocardial strain in the young Fontan population, with the most maladaptive response seen in the single right ventricle.

A Review of an Interfacility Transport Program Pediatric Stroke Clinical Practice Guideline.

BACKGROUND: Pediatric acute ischemic stroke is a rare diagnosis that requires timely recognition and definitive management to prevent morbidity and mortality. Children often present to primary care offices, urgent care clinics, and adult emergency departments for evaluation of symptoms that may be signs and symptoms of stroke. Currently, there are no published prehospital or transport protocols specific to pediatric acute ischemic stroke. The Children's Mercy Hospital Critical Care Transport Team (CMCCT) created a pediatric-specific clinical practice guideline (CPG) for suspected acute ischemic stroke. METHODS: This retrospective, descriptive study reports pediatric patients aged younger than 18 years who met criteria for the pediatric stroke CPG and required interfacility transport by CMCCT over a 4- year period. Large vessel occlusion (LVO) scores used in adults were calculated retrospectively. RESULTS: Seventeen patients met inclusion criteria. Four (24%) of 17 had radiographic evidence of acute thrombus, 3 of whom received alteplase and/or endovascular clot retrieval. Median age of confirmed stroke was 83 months (interquartile range, 65-148) compared with 177 months for nonstroke (interquartile range, 169-191), P = 0.126. The most common presenting symptom was hemiplegia in the confirmed stroke group. The confirmed stroke group scored significantly lower on the Glasgow Coma Scale (median of 8 vs 15, P = 0.014), significantly higher on the Los Angeles Motor Scale LVO score (median 4 vs 0, P = 0.021), and significantly higher on the Rapid Arterial Occlusion Evaluation LVO (median 4 vs 0, P = 0.036). CONCLUSIONS: To our knowledge, the CMCCT CPG is the first pediatric transport protocol aimed at recognition and management of pediatric stroke described in the literature. Retrospective calculation of LVO scores show that they may be helpful in application to pediatric patients.

Progression of comorbidities in youth with overweight or obesity during the COVID-19 pandemic.

BACKGROUND: Childhood obesity rates have continued to increase with the COVID-19 pandemic. However, data are limited on the impact of increasing obesity on associated comorbidities. METHODS: We evaluated the progression of overweight- or obesity-associated comorbidities by investigating change in laboratory results pre-COVID-19 pandemic and post-COVID-19 pandemic onset in youth with overweight or obesity. We defined progression of comorbidities based on increase in category rather than absolute change in value. RESULTS: HbA1c progression was seen in 19%, and LDL cholesterol progression was seen in 26%, as defined by categories. HbA1c progression and LDL cholesterol progression were significantly correlated. HbA1c and LDL cholesterol progression were significantly associated with older age and Hispanics, respectively. CONCLUSION: The results indicate youths with overweight or obesity have experienced progression of comorbidities during the COVID-19 pandemic. This study emphasizes the importance of early detection of comorbidities among a high-risk pediatric population.

Examining diagnostic variability among pediatric subspecialists using case examples of infant head injury.

BACKGROUND: Although there is research regarding the diagnosis and timing of abusive head trauma, there remains practice variation among pediatric subspecialists. OBJECTIVES: To examine diagnostic variability among pediatric subspecialists using case examples of infant head injury. Secondary objectives were timing variability among subspecialists, and diagnostic and timing variability among Child Abuse Pediatricians (CAPs). PARTICIPANTS AND SETTING: Pediatric subspecialists were recruited from Child Abuse Pediatrics, Hospital Medicine, Emergency Medicine, Critical Care, and Neurosurgery to complete a research instrument. Participants qualified for the study if they evaluated at least 1 case of possible abusive head trauma during their career. METHODS: This multi-institutional, mixed-methods study used a research instrument with 4 case examples of infant head injury: severe retinal hemorrhages (RH), mass-effect subdural hemorrhage (SDH), SDH membrane formation, and sepsis. The response selected by most CAPs was reference and compared across subspecialties and among CAPs using Chi-square or Fisher's exact tests. A Bonferroni correction (p < 0.01) was used for subspecialty comparisons. RESULTS: There were 288 participants who completed at least 1 case example. Diagnostic variability was observed in all case examples. Significantly fewer Hospital Medicine (34.9 % vs. 57.9 %, p < 0.01), Emergency Medicine (28.0 % vs. 57.9 %, p < 0.0001), and Neurosurgery (24.0 % vs. 57.9 %, p < 0.01) participants selected the reference response for the mass-effect SDH case example. Timing variability was statistically significant for all case examples (p < 0.01). Significantly fewer CAPs aged 44-64 years selected the reference response for timing (p < 0.01) for the severe RH case example. CONCLUSIONS: Additional peer review processes and consensus guidelines for challenging issues in abusive head trauma may be beneficial.

Diagnostic role of fractional exhaled nitric oxide in pediatric eosinophilic esophagitis, relationship with gastric and duodenal eosinophils.

BACKGROUND: Eosinophilic esophagitis (EoE) is an eosinophilic-predominant inflammation of the esophagus diagnosed by upper endoscopy and biopsies. A non-invasive and cost-effective alternative for management of EoE is being researched. Previous studies assessing utility of fractional exhaled nitric oxide (FeNO) in EoE were low powered. None investigated the contribution of eosinophilic inflammation of the stomach and duodenum to FeNO. AIM: To assess the utility of FeNO as a non-invasive biomarker of esophageal eosinophilic inflammation for monitoring disease activity. METHODS: Patients aged 6-21 years undergoing scheduled upper endoscopy with biopsy for suspected EoE were recruited in our observational study. Patients on steroids and with persistent asthma requiring daily controller medication were excluded. FeNO measurements were obtained in duplicate using a chemiluminescence nitric oxide analyzer (NIOX MINO, Aerocrine, Inc.; Stockholm, Sweden) prior to endoscopy. Based on the esophageal peak eosinophil count (PEC)/high power field on biopsy, patients were classified as EoE (PEC ≥ 15) or control (PEC ≤ 14). Mean FeNO levels were correlated with presence or absence of EoE, eosinophil counts on esophageal biopsy, and abnormal downstream eosinophilia in the stomach (PEC ≥ 10) and duodenum (PEC ≥ 20). Wilcoxon rank-sum test, Spearman correlation, and logistic regression were used for analysis. P value < 0.05 was considered significant. RESULTS: We recruited a total of 134 patients, of which 45 were diagnosed with EoE by histopathology. The median interquartile range FeNO level was 17 parts per billion (11-37, range: 7-81) in the EoE group and 12 parts per billion (8-19, range: 5-71) in the control group. After adjusting for atopic diseases, EoE patients had significantly higher FeNO levels as compared to patients without EoE (Z = 3.33, P < 0.001). A weak yet statistically significant positive association was found between the number of esophageal eosinophils and FeNO levels (r = 0.30, P < 0.005). On subgroup analysis within the EoE cohort, higher FeNO levels were noted in patients with abnormal gastric (n = 23, 18 vs 15) and duodenal eosinophilia (n = 28, 21 vs 14); however, the difference was not statistically significant. CONCLUSION: After ruling out atopy as possible confounder, we found significantly higher FeNO levels in the EoE cohort than in the control group.

Comparative healthcare use by adolescents screening positive for sexual exploitation.

BACKGROUND: Although screens for commercial sexual exploitation of children (CSEC) have been developed, little is known about how adolescents at high risk for, or victims of, CSEC compared to non-CSEC adolescents in healthcare utilization as previous studies have not included a control group. OBJECTIVE: Identify where and how often CSEC adolescents presented to medical care in 12 months prior to being identified as compared to non-CSEC adolescents. PARTICIPANTS AND SETTING: Adolescents between 12 and 18 years seen in a tertiary pediatric health care system in a Midwestern city with a metropolitan population of >2 million. METHODS: This was a 46-month retrospective case-control study. Cases included adolescents who screened high risk or positive for CSEC. Control group 1 included adolescents who screened negative for CSEC. Control group 2 were adolescents who were not screened for CSEC, matched to cases and to control group 1. The three study groups were compared for frequency of, location of, and diagnosis given for medical visits. RESULTS: There were 119 CSEC adolescents, 310 CSEC negative, and 429 unscreened adolescents. Compared to the controls, CSEC positive adolescents sought care less frequently (p < 0.001) and were more likely to present to an acute care setting (p < 0.0001). CSEC cases sought medical care in the acute setting more commonly for inflicted injuries (p < 0.001), mental health (p < 0.001), and reproductive health (p = 0.003). In primary care, CSEC adolescents were more commonly seen for reproductive health (p = 0.002) and mental health (p = 0.006). CONCLUSIONS: CSEC adolescents differ from non-CSEC adolescents in frequency, location, and reasons for seeking healthcare.

Impact of Duodenal Pathology on Oral Drug Bioavailability and Disease Outcomes in Pediatric Crohn's Disease.

BACKGROUND: Crohn's disease with upper gastrointestinal tract involvement occurs more often in children than adults and has the potential to interfere with oral drug absorption. We aimed to compare disease outcomes in children receiving oral azathioprine for the treatment of Crohn's disease with (DP) and without (NDP) duodenal pathology at diagnosis. METHODS: Duodenal villous length, body mass index (BMI), and laboratory studies were compared in DP vs. NDP during the first year post-diagnosis, using parametric/nonparametric tests and regression analysis (SAS v9.4); the data are reported as the median (interquartile range) or the mean ± standard deviation. Thiopurine metabolite concentration (pmol/8 × 108 erythrocytes) 230-400 was considered therapeutic for 6-thioguanine nucleotides (6-TGN), and >5700 was considered hepatotoxic for 6-methylmercaptopurine (6-MMPN). RESULTS: Twenty-six of the fifty-eight children enrolled (29 DP, 29 NDP) started azathioprine for standard medical care, including nine DP and ten NDP who had normal thiopurine methyltransferase activity. Duodenal villous length was significantly shorter in DP vs. NDP (342 ± 153 vs. 460 ± 85 µm; p < 0.001) at diagnosis; age, sex, hemoglobin, and BMI were comparable between groups. A trend toward lower 6-TGN was observed in the DP vs. NDP subset receiving azathioprine (164 (117, 271) vs. 272 (187, 331); p = 0.15). Compared to NDP, DP received significantly higher azathioprine doses (2.5 (2.3, 2.6) vs. 2.2 (2.0, 2.2) mg/kg/day; p = 0.01) and had an increased relative risk of sub-therapeutic 6-TGN. At 9 months post-diagnosis, children with DP had significantly lower hemoglobin (12.5 (11.7, 12.6) vs. 13.1 (12.7, 13.3) g/dL; p = 0.01) and BMI z-scores (-0.29 (-0.93, -0.11) vs. 0.88 (0.53, 0.99); p = 0.02) than children with NDP. CONCLUSION: For children with Crohn's disease, duodenal pathology, marked by villous blunting, increased the risk of sub-therapeutic 6-TGN levels, despite higher azathioprine dosing during the first year post-diagnosis. Lower hemoglobin and BMI z-scores at 9 months post-diagnosis suggest the impaired absorption/bioavailability of nutrients, as well as oral drugs, in children with duodenal disease.

The Perfect Storm: Perceptions of Influencing Adults Regarding Latino Teen Pregnancy in Rural Communities.

INTRODUCTION: Recognition of the importance of adolescents' environments in influencing their sexual and reproductive health (SRH) decision-making necessitates a deeper understanding of the role that community stakeholders play in shaping Adolescent and Young Adults (AYA) access to SRH education and care. We describe community stakeholders' knowledge, beliefs, and attitudes about AYA's SRH needs in three rural Latino communities in Kansas. METHODS: Key stakeholders completed a written survey incorporating the theory of Planned Behavior to assess attitudes, norms, and intentions to support AYA's SRH education and access to care. RESULTS: Across three rural immigrant community settings, respondents (N = 55) included 8 community health workers, 9 health care providers, 7 public health officials, 19 school health officials, and 12 community members. More than half self-identified as Latino (55%). Six (11%) participants, half of whom were in the health sector, thought SRH education would increase the likelihood that teens would engage in sexual activities. In contrast, other stakeholders thought that providing condoms (17, 30.9%), contraception other than condoms (14, 25.5%), and providing HPV immunization (5, 9.6%) would increase the likelihood of engaging in sexual activity. Ambivalence regarding support for SRH education and service provision prevailed across sectors, reflected even in the endorsement of the distribution of less effective contraceptive methods. Obstacles to care include immigration status, discrimination, lack of confidential services, and transportation. CONCLUSION: Key stakeholders living in rural communities revealed misconceptions, negative attitudes, and ambivalent beliefs related to the delivery of SRH education and services, potentially creating barriers to AYA's successful access to care.

Little is known about the knowledge, beliefs and attitudes held by key stakeholders in rural immigrant Latino communities regarding their level of support for AYA's SRH needs. Key stakeholders may harbor influential and ambivalent opinions regarding AYA's sexuality considering existing cultural norms, therefore, stigmatizing access to SRH education and care. We examined key community stakeholders' attitudes and norms as measures of intentions to support AYA's SRH education and care. We identify actionable steps needed to eliminate existing barriers to AYA's access to SRH education and care among at-risk immigrant Latino communities in the rural Midwest.

Surgeon and SterEOS agreement of spinal sagittal plane parameters in patients with adolescent idiopathic scoliosis.

PURPOSE: To compare agreement between surgeons and sterEOS sagittal plane measurements. METHODS: EOS radiographs of 74 patients with adolescent idiopathic scoliosis were reviewed. The measurements were generated by two surgeons and compared to sterEOS. Intraclass correlations (ICC) were calculated. Agreement was also analyzed for the following subgroups: Cobb angle < 70° vs ≥ 70°, lumbar modifier A vs B/C, and BMI of < 24.5 kg/m2 vs ≥ 24.5 kg/m2. Agreement was poor if the ICC was < 0.5, moderate if 0.5-0.75, good if 0.75-0.9, and excellent if > 0.9. Paired t tests were performed to compare the surgeon's and sterEOS means. RESULTS: For the surgeons, agreement was good (0.75-0.89) except for pelvic tilt (PT) and sacral slope (SS), which were excellent (0.91-0.92). Agreement between the surgeons and sterEOS were good (0.78-0.9) except PT and SS, which were excellent (0.91-0.93). Agreement was negatively affected for T4-T12 kyphosis, PI, and SS in the ≥ 70°group, LL when BMI was ≥ 24.5 kg/m2, and LL, PI, and SS in the lumbar modifier B/C group. The ICCs overlapped with the 95% confidence intervals (95% CI). Paired t-test showed a significant difference for T4-T12 kyphosis (p < 0.001). This was also true in the < 70° group (p < 0.001), the ≥ 70° group (p = 0.04), and the BMI < 24.5 kg/m2 group. PT was significantly different for the ≥ 70° group. CONCLUSIONS: There was good to excellent agreement between the surgeons and surgeons and sterEOS. Some variables may affect agreement. The surgeons overestimated T4-T12 kyphosis.

Single ventricular strain measures correlate with peak oxygen consumption in children and adolescents with Fontan circulation.

INTRODUCTION: Children with a single ventricle post-Fontan palliation are at increased risk of poor outcomes with peak oxygen consumption acting as a surrogate outcome marker. The purpose of this study is to evaluate the relationship between peak oxygen consumption and echocardiographic measures of ventricular function and deformation, including ventricular global longitudinal strain and dyssynchrony, in children and adolescents following Fontan palliation. METHODS: Patients (age 8-21 years) with single ventricle post-Fontan palliation were prospectively recruited and participated in an echocardiogram, including views optimised for two-dimensional speckle tracking, and a cardiopulmonary exercise test on a cycle ergometer to maximal volitional fatigue. RESULTS: Thirty-eight patients (mean age 13.7 ± 2.3 years) post-Fontan palliation had either a single left ventricular (n = 20), single right ventricular (n = 14), or biventricular (n = 4) morphology. Peak oxygen consumption (24.9 ± 5.6 ml/kg/minute) was correlated with global longitudinal strain (r = -0.435, p = 0.007), a strain discoordination time to peak index (r = -0.48, p = 0.003), and the presence of an electro-mechanical dyssynchrony strain pattern (p = 0.008). On multivariate regression modelling, these three variables were associated with peak oxygen consumption independently of age and sex. The single right ventricular group had evidence of possible diastolic dysfunction by E/e' compared to the single left ventricular and biventricular groups (p = 0.001). CONCLUSIONS: Strain analysis measures are correlated with peak oxygen consumption in this cohort of children, adolescents, and young adults following Fontan palliation, suggesting that ventricular mechanics may influence the efficiency of the Fontan circulation.

The influence of somatic maturity on the relationship between the triglyceride/high-density lipoprotein ratio and vascular health in children and adolescents with dyslipidemia.

INTRODUCTION: Both the triglyceride to HDL cholesterol (TG/HDL) ratio and timing of pubertal maturation have been identified as independent contributors to the development of atherosclerosis. OBJECTIVE: The purpose of our study was to determine the relationship between the TG/HDL ratio and measures of vascular health in children and adolescents with dyslipidemia stratified by somatic maturity. We hypothesized that somatic maturity would have a significant interaction with TG/HDL ratio and vascular health. METHODS: This was a longitudinal analysis of 120 children and adolescents (age 8-14 years) with dyslipidemia recruited from a pediatric preventive cardiology clinic. At baseline and each follow-up visit, a non-fasting serum lipid panel was collected and vascular health (carotid artery intima--media thickness, pulse wave velocity, augmentation index) was assessed. Peak height velocity (PHV) was calculated at each visit, and participants were stratified into groups by maturity offset (pre-PHV, mid-PHV, post-PHV). A mixed model design permitted baseline and follow-up visits to be classified as discrete data points. RESULTS: Of the n = 235 data points (pre-PHV = 23%, mid-PHV = 19%, and post-PHV = 58%), we identified no significant interaction between TG/HDL ratio, maturity offset, and measures of vascular structure or function. There was also no significant relationship found between TG/HDL and maturity group. Within the mid-pubertal group, there was weak relationship found between TG/HDL and augmentation index. CONCLUSION: Despite the well-described relationship between early pubertal maturation and development of cardiovascular risk factors in adulthood, we found that vascular damage resulting from an elevated TG/HDL ratio is not independently associated with somatic maturity.

Gender diversity in adolescents with polycystic ovary syndrome.

OBJECTIVES: The objective of our study was to describe the prevalence of gender diverse (GD) youth  among adolescents with polycystic ovary syndrome (PCOS). METHODS: We conducted a retrospective chart review on patients who met NIH criteria for PCOS in our Multidisciplinary Adolescent PCOS Program (MAPP). We compared those with PCOS to MAPP patients who did not meet PCOS criteria as well as to non-PCOS patients from the Adolescent Specialty Clinic (ASC). Variables analyzed included gender identity, androgen levels, hirsutism scores, and mood disorders. We used chi-square, Fisher's exact, t-tests, and Wilcoxon rank sum tests to compare groups.  Gender identities self-reported as male, fluid/both or nonbinary  were pooled into the GD category. RESULTS: Within the MAPP, 7.6% (n=12) of PCOS youth self-identified as GD compared to 1.8% (n=3) of non PCOS youth (p=0.01, chi-square). When compared to non-PCOS GD adolescents from ASC (4.4%; n=3), the difference to PCOS youth was no longer significant (p=0.56). Among MAPP patients, gender diversity was associated with higher hirsutism scores (p<0.01), but not higher androgen levels. In PCOS, depression/anxiety was higher in GD vs cisgender youth (100% vs. 37.6%, p<0.01 and 77.8% vs. 35.8%, p=0.03 respectively). CONCLUSIONS: Gender diversity was observed more commonly in those meeting PCOS criteria. PCOS GD youth were more hirsute and reported more depression/anxiety. Routine screening for differences in gender identity in comprehensive adolescent PCOS programs could benefit these patients, as alternate treatment approaches may be desired to support a transmasculine identity.

The effect of neuropsychiatric medication on pediatric nonalcoholic fatty liver disease.

Obese and overweight children are at risk of developing nonalcoholic fatty liver disease (NAFLD), which can lead to steatohepatitis, cirrhosis, and liver transplantation. Neuropsychiatric conditions affect an increasing proportion of children and often require neuropsychiatric medications (NPMs) that are associated with weight gain and/or drug-induced liver injury. We sought to evaluate the role that the extended use of NPMs play in pediatric NAFLD. Medical chart review was conducted for 260 patients with NAFLD (NPM = 77, non-NPM = 183) seen in the Liver Care Center at Children's Mercy Hospital between 2000 and 2016. Outcome measures included body mass index (BMI) percentile, BMI z-score, aspartate aminotransferase (AST), alanine aminotransferase (ALT), total bilirubin, and gamma glutamyltransferase, and were collected at diagnosis, 6-18 month follow-up, and 18-36 months. Controlling for race and metformin, there was a significant increase over time in BMI z-score (p < 0.01) and total bilirubin (p = 0.03), with only initial decreases in ALT (p < 0.01) and AST (p < 0.01). Except for higher total bilirubin in the non-NPM group, no main effect of group or interaction effect was found. Similar patterns remained when subjects were analyzed by NPM drug class. Further study is needed to confirm these findings and to evaluate the effects of NPM dose and duration of exposure, by drug class, on pediatric NAFLD outcomes.

The Frequency and Outcomes of Nerve Palsies in Operatively Treated Supracondylar Humerus Fractures.

BACKGROUND: A high index of suspicion for nerve palsy is essential in the setting of a displaced supracondylar humerus fracture (SCHF) with careful attention to the examination. We hypothesize that nerve injuries are more prevalent in higher energy type III and flexion SCHFs compared with type II fractures. METHODS: A retrospective review was performed of 1085 operatively treated SCHFs in pediatric patients, aged 0 to 14 years, between January 1, 2015 and December 31, 2018. There were 979 patients eligible for analysis. Exclusion criteria included follow-up <3 weeks, polytrauma, pathologic fracture, and prior fracture of the ipsilateral elbow. RESULTS: The overall rate of nerve injury was 10.6% (104/979). A significant difference in the rate of nerve palsy was identified between fracture types: 0.9% type II, 19.3% type III, and 10.7% flexion type SCHFs ( P <0.001). Median nerve palsy was the most common (n=65). An increased rate of ulnar nerve palsy was observed in flexion type SCHFs. The rate of nerve palsy was higher in the medial pin group (14.9%, P =0.035) but no significant association with iatrogenic ulnar nerve palsy was present. In type III and flexion SCHFs, operative time >60 minutes ( P =0.023) and the need for open reduction ( P =0.012) were significantly associated with postoperative nerve palsy. Referral to therapy was required in 30.8% of patients with a nerve palsy compared with 7.9% of patients without ( P <0.001). CONCLUSION: Longer operative times, need for open reduction, and significantly higher rates of nerve palsy among type III and flexion SCHFs are best explained by the unstable nature of the fracture and greater degree of soft tissue trauma. Given prolonged duration of follow-up and more frequent need for therapy demonstrated in patients with a nerve palsy, these data can be used to improve counseling for families and patients after displaced SCHF. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

IV Sotalol Use in Pediatric and Congenital Heart Patients: A Multicenter Registry Study.

Background There is limited information regarding the clinical use and effectiveness of IV sotalol in pediatric patients and patients with congenital heart disease, including those with severe myocardial dysfunction. A multicenter registry study was designed to evaluate the safety, efficacy, and dosing of IV sotalol. Methods and Results A total of 85 patients (age 1 day-36 years) received IV sotalol, of whom 45 (53%) had additional congenital cardiac diagnoses and 4 (5%) were greater than 18 years of age. In 79 patients (93%), IV sotalol was used to treat supraventricular tachycardia and 4 (5%) received it to treat ventricular arrhythmias. Severely decreased cardiac function by echocardiography was seen before IV sotalol in 7 (9%). The average dose was 1 mg/kg (range 0.5-1.8 mg/kg/dose) over a median of 60 minutes (range 30-300 minutes). Successful arrhythmia termination occurred in 31 patients (49%, 95% CI [37%-62%]) with improvement in rhythm control defined as rate reduction permitting overdrive pacing in an additional 18 patients (30%, 95% CI [19%-41%]). Eleven patients (16%) had significant QTc prolongation to >465 milliseconds after the infusion, with 3 (4%) to >500 milliseconds. There were 2 patients (2%) for whom the infusion was terminated early. Conclusions IV sotalol was safe and effective for termination or improvement of tachyarrhythmias in 79% of pediatric patients and patients with congenital heart disease, including those with severely depressed cardiac function. The most common dose, for both acute and maintenance dosing, was 1 mg/kg over ~60 minutes with rare serious complications.

Demographics in Children Presenting With Acute Neurologic Deficits Concerning for Stroke: An Evaluation of the Stroke Alert Process.

Objective: To assess the demographics and clinical characteristics of children presenting with acute neurologic symptoms concerning for stroke. Background: Stroke leads to significant morbidity in the pediatric population. Stroke protocols were created as a means to help identify and triage these children. Despite implementation of these protocols, there have been few population-based studies evaluating the demographics and clinical features of children presenting with acute strokelike symptoms. Methods: A retrospective chart review of patients for whom the stroke alert process was activated from September 2016 through August 2018 at Children's Mercy Hospital. Results: There were a total of 61 activations. Acute ischemic stroke or transient ischemic attack comprised 18% and was the second leading diagnoses after seizure with postictal (Todd) paralysis (20%). Two activations were candidates for mechanical thrombectomy, and none received tissue plasminogen activator (tPA). Children with acute ischemic stroke / transient ischemic attack were likely to be younger in age (median 4 years, interquartile range [IQR] 3-9) compared to those with nonischemic stroke diagnoses (median 12.5 years, IQR 7-15.3) (P = .010). The anatomical location of acute ischemic stroke was widespread, including both anterior and posterior circulations. Past medical history, family history, racial demographics, sex, and initial presenting symptoms were not predictive of the diagnosis of acute ischemic stroke / transient ischemic attack. 38% of activations with diagnoses other than transient ischemic attack / acute ischemic stroke required urgent treatment, with 16% requiring intensive care unit admission. Conclusion: Acute ischemic stroke / transient ischemic attack comprised nearly one-fifth of all pediatric stroke activations, highlighting the importance of developing protocols for early recognition and evaluation of children who present with symptoms concerning for stroke.

Current practices in delaying testing for IgE-mediated food allergy after anaphylaxis.

Background: General recommendations for delaying allergy testing, especially skin testing, in the weeks following anaphylaxis have been proposed owing to concern for false-negative results. However, no specific guidelines exist to aid clinicians in the decision regarding when to perform postanaphylactic food allergy testing and which test to use. The current practices of allergists regarding a delay in testing for food allergy after anaphylaxis are unknown. Objective: We aimed to quantify the use of different approaches to postanaphylactic food allergy testing among allergists across the United States. Methods: A clinical scenario was presented to 82 allergy and immunology fellowship program directors via e-mail survey. They were asked whether they avoid skin prick and/or blood-specific IgE testing in a child in the 6 weeks following suspected food-induced anaphylaxis owing to concern for false-negative results. Results: The survey response rate was 33%. Of the 27 respondents, 4 (14.8%) avoid both blood and skin testing, 7 (25.9%) avoid skin testing only, and 16 (59.3%) do not avoid any testing. Conclusion: Given the paucity of published evidence, a spectrum of opinions exists regarding testing in the weeks following food-induced anaphylaxis. About 40% of the allergy and immunology fellowship program directors in the United States who responded to the survey in this study avoid some allergy testing in the first 6 weeks after food-induced anaphylaxis owing to concern for false-negative results.