Development and assessment of a low literacy, pictographic asthma action plan with clinical automation to enhance guideline-concordant care for children with asthma.

OBJECTIVE: Asthma is characterized by reversible pulmonary symptoms, frequent hospitalizations, poor quality of life, and varied treatment. Parents with low health literacy (HL) is linked to poor asthma outcomes in children. Recent practice updates recommended inhaled corticosteroids for the management of persistent asthma, but guideline-concordant care is suboptimal. Our aim was to develop and assess an Asthma Action Plan (AAP) that could serve as an individualized plan for low HL families and facilitate guideline-concordant care for clinicians. METHODS: We followed the National Institute of Health 5-step "Clear & Simple" approach to develop the Uniformed Services AAP. Our AAP included symptom pictographs (dyspnea, cough, sleep, activity) and guideline-concordant clinical automation tools. Caregivers assessed the pictograms for validity (transparency of ≥ 85%; translucency score ≥ 5; and ≥ 85% recall). Readability was assessed using 7 formulas. (<6th Grade was acceptable). Comprehensibility, design quality, and usefulness was assessed by caregivers using the Consumer Information Rating Form (CIRF) (>80% was acceptable). Understandability and actionability was assessed by medical librarians using the Patient Education Materials Assessment Tool-Printable (>80% was acceptable). Suitability was assessed by clinicians using the modified Suitability Assessment of Materials (SAM) instrument (>70% was superior). RESULTS: All 12 pictograms were validated (N = 118 respondents). Readability demonstrated a 4th grade level. Overall CIRF percentile score = 80.4%. Understandability and Actionability = 100%. Suitability score = 75%. CONCLUSIONS: Our AAP was formally endorsed by the Allergy & Asthma Network. The Uniformed Services AAP is a novel tool with embedded clinical automation that can address low HL and enhance guideline-concordant care.

Survival after lung transplantation of cystic fibrosis patients infected with Burkholderia dolosa (genomovar VI).

Cystic fibrosis (CF) with severe lung disease is a well-recognized indication for lung transplantation. Colonization with various organisms in CF patients may impact post-transplant morbidity and mortality. Burkholderia cepacia complex (BCC) is made up of distinct genomovars with significant morbidity and mortality associated with B. cenocepacia (genomovar III) following lung transplant. The outcomes of patients infected with genomovar B. dolosa (genomovar VI) have yet to be described in the literature. We performed a retrospective chart review of all cystic fibrosis patients colonized with B. dolosa from our center who underwent lung transplantation (n = 11) at various medical centers across the US between 2000 and 2014. Survival rates were 73%, 53%, and 30% for 1, 3, and 5 years, respectively. Median survival was 44 months (95% CI = 11.1-76.8). CF patients with B. dolosa that have undergone lung transplantation have decreased one-year survival when compared to all patients transplanted with cystic fibrosis. Conditional 5-year survival for B. dolosa-infected patients was 43% in patients that survived the first year post-transplant, suggesting that this first year is crucial in managing the infection. Importantly, the survival of the B. dolosa patients was higher than compared to previously reported survival rates of B. cenocepacia patients post-transplant.

Visual diagnosis: 6-month-old boy with leg pain.

Pediatricians play an important role in diagnosing OI as a cause of fracture and may be asked to differentiate this uncommon genetic diagnosis from intentional trauma and other causes of fracture. Early referral to a pediatric endocrinologist, physical therapist, and orthopedic surgeon for the evaluation and treatment of low bone mass and recurrent fractures is important because early medical and surgical intervention may help to minimize the rate of future fractures, even within the first year of life. Continued follow-up with physical and occupational therapy, audiology testing, and regular evaluations of dental health are all essential for children with OI.

Pulmonary hemorrhage in infancy: A 10-year single-center experience.

AIM: Pulmonary hemorrhage in infancy is rare, with challenges in determining its incidence, causes, and outcomes across diverse groups. Our aim was to better understand the incidence and identified causes. We further analyzed the subgroup of patients meeting criteria for acute idiopathic pulmonary hemorrhage of infancy (AIPHI) to determine recurrence, mortality, and treatment. METHODS: We performed a 10-year retrospective cohort study of infants with pulmonary hemorrhage in a large tertiary care center. One-hundred fifty-seven patients overall were identified. RESULTS: The most common diagnoses in infants with pulmonary hemorrhage were congenital heart disease (36.6%), prematurity/premature lung disease (34.6%), congenital or acquired lung disorders (15.0%), and congenital or acquired coagulopathies (13.7%). Nonaccidental trauma (NAT; n = 3) was also an important cause of pulmonary hemorrhage. All patients diagnosed with NAT had normal retinal examinations and skeletal surveys. Only four patients were identified with AIPHI. There was no mortality in this group of infants. One of four patients with AIPHI had a recurrence. Steroids were the consistent treatment for AIPHI, with a large range of treatment duration. CONCLUSION: Diagnostic studies should focus on identifying non-pulmonary sources of bleeding, infection, underlying lung disease, congenital heart defects, coagulopathies, infection, and NAT, as these were the most frequently identified causes of bleeding. NAT is not adequately identified with ophthalmology exam and skeletal survey. Overall, we found AIPHI to be a rare diagnosis. All of the patients with idiopathic hemorrhage received systemic steroids with varying doses and lengths of treatment.