Inflammatory Arthritis in a 19-month-old with Von Hippel-Lindau Disease.

Von Hippel-Lindau disease (VHL) is a rare autosomal dominant disease characterized by progressive development of cysts and tumors. Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disorder and the most common arthritis in children. Although the mechanism of pathogenesis is not fully understood, JIA is thought to be a polygenic, autoimmune-mediated disease. Inherited or acquired disorders resulting in immune dysregulation can lead to neoplastic and autoimmune disease, but very few cases of patients with VHL and concomitant autoimmune disease are reported in the literature. Herein, we describe, to the best of our knowledge, the first reported case of a child with VHL and inflammatory arthritis, and we discuss three possible pathophysiologic mechanisms that could link VHL and JIA. Understanding the shared pathophysiology and genetics of both diseases may help guide future direction of targeted therapies and lead to improved clinical outcomes.

A case of ANCA-associated vasculitis in a 16-year-old female following SARS-COV-2 infection and a systematic review of the literature.

BACKGROUND: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare form of vasculitis in children. SARS-CoV-2, the virus that causes COVID-19 infection, seems to trigger autoimmunity and new-onset autoimmune disease in pediatric and adult patients. We present a case of new-onset AAV following COVID-19 infection in an adolescent patient, and we review the literature of AAV following COVID-19 infection. CASE PRESENTATION: An adolescent female with a history of asthma was diagnosed with mild COVID-19 infection and subsequently developed persistent cough, wheezing, hearing loss, arthralgias, and rash. Her imaging and laboratory workup showed pulmonary nodules and cavitary lesions, elevated inflammatory markers, negative infectious testing, and positive ANCA. She was treated with glucocorticoids, rituximab, and mycophenolate mofetil. At six-month follow-up, she had improvement in her symptoms, pulmonary function tests, imaging findings, and laboratory markers. CONCLUSIONS: We report the second case of new-onset anti-PR3, C-ANCA vasculitis and the fourth case of pediatric-onset AAV following COVID-19 infection. A systematic review of the literature found 6 cases of new-onset AAV in adults after COVID-19 infection. Pediatric and adult patients who develop AAV post COVID-19 infection have few, if any, comorbidities, and show marked radiographic and symptomatic improvement after treatment. There is increasing evidence for COVID-19-induced autoimmunity in children and our case highlights the importance of considering AAV in a child following a recent COVID-19 infection because timely treatment may improve clinical outcomes.

Potential Impact of Initial Clinical Data on Adjustment of Pediatric Readmission Rates.

OBJECTIVE: Comparison of readmission rates requires adjustment for case-mix (ie, differences in patient populations), but previously only claims data were available for this purpose. We examined whether incorporation of relatively readily available clinical data improves prediction of pediatric readmissions and thus might enhance case-mix adjustment. METHODS: We examined 30-day readmissions using claims and electronic health record data for patients ≤18 years and 29 days of age who were admitted to 3 children's hospitals from February 2011 to February 2014. Using the Pediatric All-Condition Readmission Measure and starting with a model including age, gender, chronic conditions, and primary diagnosis, we examined whether the addition of initial vital sign and laboratory data improved model performance. We employed machine learning to evaluate the same variables, using the L2-regularized logistic regression with cost-sensitive learning and convolutional neural network. RESULTS: Controlling for the core model variables, low red blood cell count and mean corpuscular hemoglobin concentration and high red cell distribution width were associated with greater readmission risk, as were certain interactions between laboratory and chronic condition variables. However, the C-statistic (0.722 vs 0.713) and McFadden's pseudo R2 (0.085 vs 0.076) for this and the core model were similar, suggesting minimal improvement in performance. In machine learning analyses, the F-measure (harmonic mean of sensitivity and positive predictive value) was similar for the best-performing model (containing all variables) and core model (0.250 vs 0.243). CONCLUSIONS: Readily available clinical variables do not meaningfully improve the prediction of pediatric readmissions and would be unlikely to enhance case-mix adjustment unless their distributions varied widely across hospitals.

Pediatric Readmissions After Hospitalizations for Lower Respiratory Infections.

BACKGROUND AND OBJECTIVE: Lower respiratory infections (LRIs) are among the most common reasons for pediatric hospitalization and among the diagnoses with the highest number of readmissions. Characterizing LRI readmissions would help guide efforts to prevent them. We assessed variation in pediatric LRI readmission rates, risk factors for readmission, and readmission diagnoses. METHODS: We analyzed 2008-2009 Medicaid Analytic eXtract data for patients <18 years of age in 26 states. We identified LRI hospitalizations based on a primary diagnosis of bronchiolitis, influenza, or community-acquired pneumonia or a secondary diagnosis of one of these LRIs plus a primary diagnosis of asthma, respiratory failure, or sepsis/bacteremia. Readmission rates were calculated as the proportion of hospitalizations followed by ≥1 unplanned readmission within 30 days. We used logistic regression with fixed effects for patient characteristics and a hospital random intercept to case-mix adjust rates and assess risk factors. RESULTS: Of 150 590 LRI hospitalizations, 8233 (5.5%) were followed by ≥1 readmission. The median adjusted hospital readmission rate was 5.2% (interquartile range: 5.1%-5.4%), and rates varied across hospitals (P < .0001). Infants (patients <1 year of age), boys, and children with chronic conditions were more likely to be readmitted. The most common primary diagnoses on readmission were LRIs (48.2%), asthma (10.0%), fluid/electrolyte disorders (3.4%), respiratory failure (3.3%), and upper respiratory infections (2.7%). CONCLUSIONS: LRI readmissions are common and vary across hospitals. Multiple risk factors are associated with readmission, indicating potential targets for strategies to reduce readmissions. Readmission diagnoses sometimes seem related to the original LRI.

Recognizing Differences in Hospital Quality Performance for Pediatric Inpatient Care.

BACKGROUND: Hospital quality-of-care measures are publicly reported to inform consumer choice and stimulate quality improvement. The number of hospitals and states with enough pediatric hospital discharges to detect worse-than-average inpatient care remains unknown. METHODS: This study was a retrospective analysis of hospital discharges for children aged 0 to 17 years from 3974 hospitals in 44 states in the 2009 Kids' Inpatient Database. For 11 measures of all-condition or condition-specific quality, we assessed the number of hospitals and states that met a "power standard" of 80% power for a 5% level significance test to detect when care is 20% worse than average over a 3-year period. For this assessment, we approximated volume as 3 times actual 2009 admission volumes. RESULTS: For all-condition quality, 1380 hospitals (87% of all pediatric discharges) and all states met the power standard for the family experience-of-care measure; 1958 hospitals (95% of discharges) and all states met the standard for adverse drug events. For condition-specific quality measures of asthma, birth, and mental health, 203 to 482 hospitals (52%-90% of condition-specific discharges) met the power standard and 40 to 44 states met the standard. One hospital and 16 states met the standard for sickle cell disease. No hospital and ≤27 states met the standard for the remaining measures studied (appendectomy, cerebrospinal fluid shunt surgery, gastroenteritis, heart surgery, and seizure). CONCLUSIONS: Most children are admitted to hospitals in which all-condition measures of quality have adequate power to show modest differences in performance from average, but most condition-specific measures do not. Policies regarding incentives for pediatric inpatient quality should take these findings into account.

Measuring pediatric hospital readmission rates to drive quality improvement.

The Pediatric Quality Measures Program is developing readmission measures for pediatric use. We sought to describe the importance of readmissions in children and the challenges of developing readmission quality measures. We consider findings and perspectives from research studies and commentaries in the pediatric and adult literature, characterizing arguments for and against using readmission rates as measures of pediatric quality and discussing available evidence and current knowledge gaps. The major topic of debate regarding readmission rates as pediatric quality measures is the relative influence of hospital quality versus other factors within and outside of health systems on readmission risk. The complex causation of readmissions leads to disagreement, particularly when rates are publicly reported or tied to payment, about whether readmissions can be prevented and how to achieve fair comparisons of readmission performance. Despite these controversies, the policy focus on readmissions has motivated widespread efforts by hospitals and outpatient providers to evaluate and reengineer care processes. Many adult studies demonstrate a link between successful initiatives to improve quality and reductions in readmissions. More research is needed on methods to enhance adjustment of readmission rates and on how to prevent pediatric readmissions.