Respiratory concerns in children with medical complexity.

Children with medical complexity (CMC) are a diverse group of children affected by a spectrum of chronic disorders and physiologic abnormalities. Due to their underlying disorders, they disproportionately experience respiratory comorbidities and complications. Respiratory-related complications that arise in this population often begin subtly and progress over time and can result in lung injury, respiratory insufficiency, and eventual chronic respiratory failure. CMC make up a substantial proportion of pediatric hospitalizations and healthcare expenditures, with respiratory-related illness being one of the leading causes of admission. It is, therefore, important for the practitioner caring for these children to have knowledge of the common respiratory concerns in this population as well as prevention and management strategies. This review will describe the most common respiratory concerns in CMC and will provide an overview of diagnosis and management.

Identifying Clinical and Research Priorities in Sickle Cell Lung Disease. An Official American Thoracic Society Workshop Report.

Background: Pulmonary complications of sickle cell disease (SCD) are diverse and encompass acute and chronic disease. The understanding of the natural history of pulmonary complications of SCD is limited, no specific therapies exist, and these complications are a primary cause of morbidity and mortality.Methods: We gathered a multidisciplinary group of pediatric and adult hematologists, pulmonologists, and emergency medicine physicians with expertise in SCD-related lung disease along with an SCD patient advocate for an American Thoracic Society-sponsored workshop to review the literature and identify key unanswered clinical and research questions. Participants were divided into four subcommittees on the basis of expertise: 1) acute chest syndrome, 2) lower airways disease and pulmonary function, 3) sleep-disordered breathing and hypoxia, and 4) pulmonary vascular complications of SCD. Before the workshop, a comprehensive literature review of each subtopic was conducted. Clinically important questions were developed after literature review and were finalized by group discussion and consensus.Results: Current knowledge is based on small, predominantly observational studies, few multicenter longitudinal studies, and even fewer high-quality interventional trials specifically targeting the pulmonary complications of SCD. Each subcommittee identified the three or four most important unanswered questions in their topic area for researchers to direct the next steps of clinical investigation.Conclusions: Important and clinically relevant questions regarding sickle cell lung disease remain unanswered. High-quality, multicenter, longitudinal studies and randomized clinical trials designed and implemented by teams of multidisciplinary clinician-investigators are needed to improve the care of individuals with SCD.

Acetazolamide therapy for metabolic alkalosis in critically ill pediatric patients.

OBJECTIVE: Despite a paucity of supporting literature, acetazolamide is commonly used in critically ill children with metabolic alkalosis (elevated plasma bicarbonate [pHco-3] and pH). The objective of this study was to assess the change in 18 hours after initiation of acetazolamide therapy. DESIGN: Retrospective study. SETTING: PICU of an urban, tertiary-care children's hospital. PATIENTS: Mechanically ventilated children (≤ 17 yr) with metabolic alkalosis (pHco-3 ≥ 35 mmol/L). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of 153 consecutively screened patients, 61 patients (29 female patients) were enrolled: 18 cardiac patients (after congenital heart disease repair) and 43 noncardiac patients. The cardiac patients were younger than the noncardiac patients (median [interquartile range] age, 0.6 mo [0.3-2.5 mo] vs 7.4 mo [2.8-39.9 mo]; p < 0.00001) and had higher preacetazolamide baseline diuretic scores and urine output. The pHco-3 levels 18 hours after initiation of acetazolamide were reduced in the cohort as a whole (40.2 ± 4.8 to 36.2 ± 5.6 mmol/L; p < 0.001) and in the noncardiac patients, but they were unchanged in the cardiac patients. The PCO2 remained unchanged after acetazolamide in both subgroups. Because young age and presence of cardiac disease were potential confounders, the 20 noncardiac patients who are 6 months old or younger were compared with the cardiac subgroup and demonstrated reduced pHco-3 after acetazolamide and lower preacetazolamide baseline diuretic score and urine output. CONCLUSION: Acetazolamide reduces pHco-3 concentration in critically ill, mechanically ventilated children overall, but it did not do so in cardiac patients in our cohort, even in comparison with noncardiac patients of a similar age. These findings do not support the current use of acetazolamide for metabolic alkalosis in critically ill children with congenital heart disease. Further study is required to determine why these cardiac patients respond differently to acetazolamide than noncardiac patients and whether this response impacts important clinical outcomes, for example, weaning mechanical ventilation.