Coronary artery dilation associated with anti-synthetase syndrome in an adolescent

Abstract Background Idiopathic inflammatory myopathies (IIM) are a group of systemic autoimmune disorders primarily affecting skeletal muscle. Pediatric coronary artery dilation is frequently discussed in Kawasaki disease. However, it has yet to be reported in the IIMs or antisynthetase syndrome. We report a unique case of a patient with IIM, antisynthetase syndrome and coronary artery dilation. Case presentation We report an adolescent presenting with joint symptoms, fever, and eye swelling with a clinical diagnosis of Juvenile Dermatomyositis. He subsequently developed diastolic hypotension with evidence of coronary artery dilation. He received steroids and immunoglobulin and followed by immunosuppressants with mild improvement in his symptoms. The adolescent later developed dyspnea and cough with CT lungs evident for cystic changes; lung biopsy showed interstitial fibrosis and inflammation, and muscle biopsy was abnormal as well. The anti-pl-12 antibody was positive. Following several weeks of treatment, an echocardiogram showed improvement in coronary artery dilation. His joint symptoms, muscle strength and respiratory symptoms have also improved. Conclusions Coronary artery dilation is not well understood in IIMs or antisynthetase syndrome. Pathobiology of coronary artery involvement, its treatment and prognosis, and association with IIM and antisynthetase syndrome needs further exploration

A Differential Approach to an Uncommon Case of Acute Anemia in a Child With Sickle Cell Disease

Introduction: Hyperhemolytic crisis is a rare and dangerous complication of sickle cell disease where the hemoglobin level drops rapidly. This can quickly lead to organ failure and death. In the literature, most cases of hyperhemolysis in sickle cell patients followed a red cell transfusion. Case Summary : In this article, we report a case of a 6-year-old African American boy with sickle cell disease who presented with fever, increased work of breathing, and consolidation in the left lower lobe of the lung on chest X-ray. He initially improved with oxygen, fluids, and antibiotics but his hemoglobin acutely dropped from 7.6 to 6 g/dL the next day of admission. He was not previously transfused, and his reticulocyte count remained high. Subsequent transfusion recovered his hemoglobin. Conclusion : This case demonstrates that in the background of the chronic hemolysis of sickle cell disease, an acute anemia should warrant exploration of aplastic crisis (parvovirus infection), immune hemolytic anemia, hepatic sequestration crisis, splenic sequestration crisis, and hyperhemolytic crisis as possible etiologies. Ongoing reticulocytosis and a source of infection may direct suspicion especially toward hyperhemolytic crisis even without preceding red cell transfusion. We propose that the optimum management should include full supportive care (including transfusions if necessary) and treatment of the underlying cause of hemolysis (such as infections or drug exposure)

Population-level SDOH and Pediatric Asthma Health Care Utilization: A Systematic Review

CONTEXT: Spatial analysis is a population health methodology that can determine geographic distributions of asthma outcomes and examine their relationship to place-based social determinants of health (SDOH). OBJECTIVES: To systematically review US-based studies analyzing associations between SDOH and asthma health care utilization by geographic entities. DATA SOURCES: Pubmed, Medline, Web of Science, Scopus, and Cumulative Index to Nursing and Allied Health Literature. STUDY SELECTION: Empirical, observational US-based studies were included if (1) outcomes included asthma-related emergency department visits or revisits, and hospitalizations or rehospitalizations; (2) exposures were ≥1 SDOH described by the Healthy People (HP) SDOH framework; (3) analysis occurred at the population-level using a geographic entity (eg, census-tract); (4) results were reported separately for children ≤18 years. DATA EXTRACTION: Two reviewers collected data on study information, demographics, geographic entities, SDOH exposures, and asthma outcomes. We used the HP SDOH framework\u27s 5 domains to organize and synthesize study findings. RESULTS: The initial search identified 815 studies; 40 met inclusion criteria. Zip-code tabulation areas (n = 16) and census-tracts (n = 9) were frequently used geographic entities. Ten SDOH were evaluated across all HP domains. Most studies (n = 37) found significant associations between ≥1 SDOH and asthma health care utilization. Poverty and environmental conditions were the most often studied SDOH. Eight SDOH-poverty, higher education enrollment, health care access, primary care access, discrimination, environmental conditions, housing quality, and crime - had consistent significant associations with asthma health care utilization. CONCLUSIONS: Population-level SDOH are associated with asthma health care utilization when evaluated by geographic entities. Future work using similar methodology may improve this research\u27s quality and utility

Social Determinants of Health and At-Risk Rates for Pediatric Asthma Morbidity

BACKGROUND AND OBJECTIVES: Compared with population-based rates, at-risk rates (ARRs) account for underlying variations of asthma prevalence. When applied with geospatial analysis, ARRs may facilitate more accurate evaluations of the contribution of place-based social determinants of health (SDOH) to pediatric asthma morbidity. Our objectives were to calculate ARRs for pediatric asthma-related emergency department (ED) encounters and hospitalizations by census-tract in Washington, the District of Columbia (DC) and evaluate their associations with SDOH. METHODS: This population-based, cross-sectional study identified children with asthma, 2 to 17 years old, living in DC, and included in the DC Pediatric Asthma Registry from January 2018 to December 2019. ED encounter and hospitalization ARRs (outcomes) were calculated for each DC census-tract. Five census-tract variables (exposures) were selected by using the Healthy People 2030 SDOH framework: educational attainment, vacant housing, violent crime, limited English proficiency, and families living in poverty. RESULTS: During the study period, 4321 children had 7515 ED encounters; 1182 children had 1588 hospitalizations. ARRs varied 10-fold across census-tracts for both ED encounters (64-728 per 1000 children with asthma) and hospitalizations (20-240 per 1000 children with asthma). In adjusted analyses, decreased educational attainment was significantly associated with ARRs for ED encounters (estimate 12.1, 95% confidence interval [CI] 8.4 to 15.8, P \u3c.001) and hospitalizations (estimate 1.2, 95% CI 0.2 to 2.2, P = .016). Violent crime was significantly associated with ARRs for ED encounters (estimate 35.3, 95% CI 10.2 to 60.4, P = .006). CONCLUSION: Place-based interventions addressing SDOH may be an opportunity to reduce asthma morbidity among children with asthma

Developing an effective inpatient learning climate

12 month embargo; published online: 11 December 2019This item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]