Objective To determine factors associated with acute respiratory failure after bone marrow transplantation which can be identified before the onset of lung disease. Design Population-based, retrospective study. Setting A referral-based pediatric intensive care unit and bone marrow transplant center. Patients Thirty-nine patients with lung disease (abnormal chest radiograph or a need for supplemental oxygen) were identified from a group of 318 pediatric bone marrow transplant patients from 1978 to 1988. Thirty-four of 39 patients with complete data were further classified into patients with mild lung disease (recovery without needing endotracheal intubation, n = 16) and patients with acute respiratory failure (requirement for endotracheal intubation, n = 18). Interventions Regression analyses were performed to define risk factors for development of respiratory failure (multivariate logistic regression) and for a shortened interval between the identification of lung disease and respiratory failure (Cox proportional hazards analysis). Measurements and Main Results Ninety-three percent (15/16) of patients with mild lung disease survived. Conversely, only 9% (2/23) of patients with respiratory failure survived. Predictors of respiratory failure included graft vs. host disease (odds ratio 28.3, 95% confidence interval 1.9–421, p = .015), a prelung disease (baseline) circulating creatinine concentration of >1.5 mg/dL (>132.6 μmol/L) (odds ratio 28.4, 95% confidence interval 1.4–577, p = .029), and male gender (odds ratio 14.6, 95% confidence interval 1–210, p = .049). Predictors of a shortened time to onset of respiratory failure included baseline serum creatinine value of >1.5 mg/dL (>132.6 μmol/L) (hazard ratio 6.2, 95% confidence interval 1.5–26.5, p = .013) and baseline total bilirubin concentration >1.4 mg/dL (>23.9 μmol/L) (hazard ratio 4.5, 95% confidence interval 0.98–20.7, p = .053). The median time to onset of respiratory failure was 4 days in patients with baseline creatinine values ≥1.5 mg/dL (>132.6 μmol/L) and 5 days in patients with baseline bilirubin concentrations ≥;1.4 mg/dL (>23.9 μmol/L) vs. >26 days in patients with creatinine <1.5 mg/dL (<132.6 μmol/L) and >29 days in patients with bilirubin <1.4 mg/dL (<23.9 μmol/L) (Kaplan-Meier analysis). Conclusions Renal and liver dysfunction preceded clinical evidence of lung disease in bone marrow transplant patients who developed respiratory failure. Lung disease leading to respiratory failure and adult respiratory distress syndrome appears to develop as one component of the multiple organ failure syndrome in pediatric bone marrow transplant patients.
Congenital heart disease (CHD) is the most common congenital anomaly affecting 0.7% to 0.8% of live births. Although the incidence of CHD has remained stable worldwide, the age standardized mortality rate of CHD in developed countries has declined substantially over the past three decades. This chapter describes the common pediatric cardiovascular conditions and recognizes the pathophysiology of common cardiovascular conditions from anatomic/structure, electrophysiological, and myocardial muscle perspectives. Due to innovations in surgery, anesthesia, and cardiopulmonary bypass, 85% of all children born with congenital heart defects are now long-term survivors resulting in many patients with repaired or palliated heart anomalies present for non-cardiac surgery. Except for the simplest of completely repaired lesions (atrial septal defect, ventricular septal defect, and patent ductus arteriosus) these patients carry a burden of residual cardiac pathophysiology and decreased cardiovascular reserve that increases anesthesia risk.
Abstract Williams syndrome is a multisystem, congenital disorder which is commonly associated with arterial stenoses: supravalvar aortic stenosis and peripheral pulmonary artery stenosis. Venous abnormalities have not been previously reported in children with Williams syndrome. We present a case of a 3-year-old girl with Williams syndrome and diffuse venous ectasia as detected by MRI.
