Case report presenting the diagnostic challenges in a patient with recurrent acquired angioedema, antiphospholipid antibodies and undetectable C2 levels.

BACKGROUND: Angioedema secondary to acquired C1 inhibitor deficiency (AAE) is a rare disease. It usually is associated with lymphoproliferative disorders. We present a case of AAE in a patient with antiphospholipid syndrome (APS), a non-Hodgkin lymphoproliferative disorder (NHL) with undetectable levels of C2, C4, and an undetectable CH50. The co-existence of AAE, APS, and NHL, with an undetectable C2 level, to the best of our knowledge, has never before reported together in the same patient. CASE PRESENTATION: A patient with a recent history of thrombosis presented with recurrent episodes of angioedema. The workup revealed undetectable levels of C2, C4 and undetectable CH50. Quantitative levels of C1 inhibitor and C1q were low. C1 inhibitor function was less than 40%. Anti-cardiolipin antibodies were found. The patient was initially treated on demand with intravenous plasma-derived human C1-INH concentrates, (Cinryze® Shire). Later the patient received prophylactic therapy with danazol. She was diagnosed with lymphoma 3 years after her first episode of angioedema. Single agent therapy with rituximab was not only effective in treating her lymphoma but also preventing further episodes of angioedema. Anti-cardiolipin antibody titers also declined. Additionally, marked early primary pathway complement component abnormalities and CH50 also corrected, although incomplete normalization of C4 proved to be due to a heterozygous C4 deficiency. CONCLUSION: This case shows the unique association of AAE, APS and NHL in a patient with undetectable levels of early complement components. Additionally, this case also shows for the first time the effectiveness of rituximab therapy in all three disease states while co-existing simultaneously in the same patient.

Routinely sleeping away from home and the association with child asthma readmission.

The increased prevalence of transitions between households may have implications for child asthma morbidity. We, therefore, sought to enumerate the prevalence of regularly spending nights sleeping away from home among children admitted to the hospital for asthma and to examine the relationship of nights away to asthma-related readmission. This was a population-based, prospective cohort of 774 children, aged 1-16 years, who were admitted with asthma or bronchodilator-responsive wheezing and enrolled in the Greater Cincinnati Asthma Risks Study. The study took place at Cincinnati Children's Hospital Medical Center, an urban, academic children's hospital in the Midwest. The primary exposure was regularly spending nights away from home. Selected covariates included caregiver marital status, shift work, child's race, income, psychological distress, and running out of/not having medications on hand. The primary outcome was asthma-related readmission within 12 months. A total of 19 % were readmitted within 12 months. The 33 % of children that spent ≥1 night away from home per week were significantly more likely to be readmitted than those who spent no nights away (25 % vs. 16 %, p = 0.002). Spending nights away from home [adjusted relative risk (aRR) 1.5, 95 % confidence interval (CI) 1.2-2.0] and lower income (aRR 2.6, 95 % CI 1.1-6.4) were the strongest independent predictors of readmission after adjusting for child age, gender, and race, and caregiver marital status, shift work, risk of psychological distress, and running out of meds. Increased awareness of the multiple settings in which children with asthma live may help shape more comprehensive approaches to asthma care.

Role of financial and social hardships in asthma racial disparities.

BACKGROUND AND OBJECTIVES: Health care reform offers a new opportunity to address child health disparities. This study sought to characterize racial differences in pediatric asthma readmissions with a focus on the potential explanatory role of hardships that might be addressed in future patient care models. METHODS: We enrolled 774 children, aged 1 to 16 years, admitted for asthma or bronchodilator-responsive wheezing in a population-based prospective observational cohort. The outcome was time to readmission. Child race, socioeconomic status (measured by lower income and caregiver educational attainment), and hardship (caregivers looking for work, having no one to borrow money from, not owning a car or home, and being single/never married) were recorded. Analyses used Cox proportional hazards. RESULTS: The cohort was 57% African American, 33% white, and 10% multiracial/other; 19% were readmitted within 12 months. After adjustment for asthma severity classification, African Americans were twice as likely to be readmitted as whites (hazard ratio: 1.98; 95% confidence interval: 1.42 to 2.77). Compared with whites, African American caregivers were significantly more likely to report lower income and educational attainment, difficulty finding work, having no one to borrow money from, not owning a car or home, and being single/never married (all P ≤ .01). Hardships explained 41% of the observed racial disparity in readmission; jointly, socioeconomic status and hardship explained 49%. CONCLUSIONS: African American children were twice as likely to be readmitted as white children; hardships explained >40% of this disparity. Additional factors (eg, pollution, tobacco exposure, housing quality) may explain residual disparities. Targeted interventions could help achieve greater child health equity.

Single parent households and increased child asthma morbidity.

OBJECTIVES: To characterize whether single parent households are associated with pediatric asthma-related repeat healthcare utilization and to examine family-level psychosocial variables that may explain this relationship. METHODS: We analyzed a prospective cohort of 526 children aged 1-16 years hospitalized for asthma or bronchodilator-responsive wheezing whose caregivers self-reported their marital status. Those reporting being "single" were considered the at-risk category. The outcome was repeat asthma-related utilization (emergency room (ER) revisit or hospital readmission) within 12 months. We assessed, a priori, four psychosocial variables (household income, caregiver risk of psychological distress, ratio of in-home children to adults, and regular attendance at childcare or a secondary home). RESULTS: Among all children enrolled in the cohort, 40% returned to the ER or hospital for asthma within 12 months. Of all caregivers, 59% self-identified as single. Single status was significantly associated with each psychosocial variable. Children in households with lower incomes and higher ratios of children to adults were both more likely to return to the ER or hospital than children with higher incomes and lower ratios, respectively (each p < 0.05). Patients in single parent households were significantly more likely to reutilize than those in married parent households (OR 1.44, 95% CI 1.00-2.07, p < 0.05). When adjusted for income, the relationship between single parent households and reutilization became non-significant. CONCLUSIONS: Children admitted for asthma from single parent households were more likely to have asthma-related reutilization within 12 months than children from homes with married parents. This was driven, in large part, by underlying differences in household income.

Inequalities in neighborhood child asthma admission rates and underlying community characteristics in one US county.

OBJECTIVES: To characterize variation and inequalities in neighborhood child asthma admission rates and to identify associated community factors within one US county. STUDY DESIGN: This population-based prospective, observational cohort study consisted of 862 sequential child asthma admissions among 167 653 eligible children ages 1-16 years in Hamilton County, Ohio. Admissions occurred at a tertiary-care pediatric hospital and accounted for nearly 95% of in-county asthma admissions. Neighborhood admission rates were assessed by geocoding addresses to city- and county-defined neighborhoods. The 2010 US Census provided denominator data. Neighborhood admission distribution inequality was assessed by the use of Gini and Robin Hood indices. Associations between neighborhood rates and socioeconomic and environmental factors were assessed using ANOVA and linear regression. RESULTS: The county admission rate was 5.1 per 1000 children. Neighborhood rates varied significantly by quintile: 17.6, 7.7, 4.9, 2.2, and 0.2 admissions per 1000 children (P < .0001). Fifteen neighborhoods containing 8% of the population had zero admissions. The Gini index of 0.52 and Robin Hood index of 0.38 indicated significant inequality. Neighborhood-level educational attainment, car access, and population density best explained variation in neighborhood admission rates (R(2) = 0.55). CONCLUSION: In a single year, asthma admission rates varied 88-fold across neighborhood quintiles in one county; a reduction of the county-wide admission rate to that of the bottom quintile would decrease annual admissions from 862 to 34. A rate of zero was present in 15 neighborhoods, which is evidence of what may be attainable.