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Objective. In recent years, continuous intravenous propofol infusion has been widely used in pediatric intensive care units. Several case reports have raised concerns about its safety. The objective of this study was to report our experience with continuous intravenous propofol in consecutive patients during an 18-month period. Methods. The study design was a retrospective review of a case series. Case was defined as a critically ill child who was treated with continuous intravenous propofol. The attending physician staff agreed to prescribe propofol via continuous intravenous infusion at a dose not to exceed 50/ [micro]g/kg/min. The protocol allowed for each patient to receive an additional intravenous bolus of propofol at a dose of 1 mg/kg no more than once per hour. The study entailed data collection from consecutive patients who were prescribed a continuous infusion of propofol in either the pediatric intensive care unit or bone marrow transplant unit. Results. Data from 142 patients were analyzed. Each patient enrolled was adequately sedated. Administration of propofol via continuous intravenous infusion was not associated with metabolic acidosis or hemodynamic compromise. No patient in the study group was inadvertently extubated or had a central venous catheter accidentally discontinued. Conclusions. Propofol can be safely and effectively used to provide sedation to critically ill infants and children. We speculate that continuous infusion of propofol for extended periods of time should not exceed 67 [micro]g/kg/min. Pediatrics 2002;110:1177-1181; sedation, metabolic acidosis, mechanical ventilation., ABBREVIATIONS. PICU, pediatric intensive care unit; BMT, bone marrow transplantation; FDA, US Food and Drug Administration. Providing effective and safe sedation for young children in the pediatric intensive care unit [...]

Inhaled low-dose nitric oxide in babies with persistent pulmonary hypertension of the newborn (PPHN) may not be beneficial and may even be harmful. PPHN is a lung disease that causes oxygen levels to decrease. In a study of 38 newborn babies, inhaled low-dose nitric oxide did not substantially increase oxygen levels in those who received it. High-dose nitric oxide improved oxygen levels, but only in babies who had not received low-dose nitric oxide. Nitric oxide relaxes blood vessels, and when it is inhaled this could improve lung oxygenation., Recent reports indicate that inhaled nitric oxide (iNO) causes selective pulmonary vasodilation, increases arterial oxygen tension, and may decrease the use of extracorporeal membrane oxygenation (ECMO) in infants with persistent pulmonary hypertension of the newborn (PPHN). Despite these reports, the optimal dose and timing of iNO administration in PPHN remains unclear. Objectives. To test the hypotheses that in PPHN 1) iNO at 2 parts per million (ppm) is effective at acutely increasing oxygenation as measured by oxygenation index (OI); 2) early use of 2 ppm of iNO is more effective than control (0 ppm) in preventing clinical deterioration and need for iNO at 20 ppm; and 3) for those infants who fail the initial treatment protocol (0 or 2 ppm) iNO at 20 ppm is effective at acutely decreasing OI. Study Design. A randomized, controlled trial of iNO in 3 nurseries in a single metropolitan area. Thirty-eight children, average gestational age of 37.3 weeks and average age [is less than] 1 day were enrolled. Thirty-five of 38 infants had echocardiographic evidence of pulmonary hypertension. On enrollment, median OI in the control group, iNO at 0 ppm, (n = 23) was 33.1, compared with 36.9 in the 2-ppm iNO group (n = 15). Results. Initial treatment with iNO at 2 ppm for an average of 1 hour was not associated with a significant decrease in OI. Twenty of 23 (87%) control patients and 14 of 15 (92%) of the low-dose iNO group demonstrated clinical deterioration and were treated with iNO at 20 ppm. In the control group, treatment with iNO at 20 ppm decreased the median OI from 42.6 to 23.8, whereas in the 2-ppm iNO group with a change in iNO from 2 to 20 ppm, the median OI did not change (42.6 to 42.0). Five of 15 patients in the low-dose nitric oxide group required ECMO and 2 died, compared with 7 of 23 requiring ECMO and 5 deaths in the control group. Conclusion. In infants with PPHN, iNO 1): at 2 ppm does not acutely improve oxygenation or prevent clinical deterioration, but does attenuate the rate of clinical deterioration; and 2) at 20 ppm acutely improves oxygenation in infants initially treated with 0 ppm, but not in infants previously treated with iNO at 2 ppm. Initial treatment with a subtherapeutic dose of iNO may diminish the clinical response to 20 ppm of iNO and have adverse clinical sequelae. Pediatrics 1999;104:1089-1094; persistent pulmonary hypertension of the newborn, extracorporeal membrane oxygenation, hypoxemia, respiratory failure, pulmonary vascular reactivity., ABBREVIATIONS. PPHN, persistent pulmonary hypertension of the newborn; iNO, inhaled nitric oxide; ECMO, extracorporeal membrane oxygenation; OI, oxygenation index; P/F, arterial partial pressure of oxygen/fraction of inspired oxygen; a/A, arterial/ [...]

Treatment, prognosis, and quality of life for people with cystic fibrosis (CF) have improved steadily since the initial description of the disease, but most dramatically in the past decade. In 2021, the median predicted survival increased to 53 years, compared with 17 years in 1970. The recent improvement in outcomes is attributable to the advent of cystic fibrosis transmembrane regulator (CFTR) modulators, small molecules that enhance the function of defective CFTR protein. The first CFTR modulator, ivacaftor, received Food and Drug Administration approval in 2011 to treat a single CFTR variant, comprising only 4% of those affected by CF. With the demonstration of efficacy, drug approval has been expanded to other variants. Multiple CFTR modulators used in combination with ivacaftor augment efficacy and increase the number of CFTR variants amenable to therapy. Approval of elexecaftor/tezecaftor/ivacaftor in 2019 increased the number of individuals who could benefit from highly effective modulator therapy (HEMT) to ∼90% of the CF population in the United States. HEMT has been dramatically effective, with overall improvements in lung function, quality of life, nutritional status, and, in women, increased fertility. HEMT may delay the onset of other CF-related comorbidities. Although off-target effects, including hepatotoxicity, drug-drug interactions, and putative mental health issues can complicate use, modulator therapy has been generally well tolerated. Ten percent of people with CF have variants that are not amenable to modulator treatment. HEMT, despite its great cost and limited global access, has brought legitimate hope and changed the lives of a significant majority of individuals and families affected by CF in North America., (Copyright © 2023 by the American Academy of Pediatrics.)

Background and Objective: Sexual assault is a major cause of injury, unplanned pregnancy, HIV infection, and mental health problems worldwide. In parts of sub-Saharan Africa, sexual assault has reached epidemic proportions. This study evaluated the efficacy of an empowerment and self-defense intervention for adolescent girls to decrease the incidence of sexual assault and harassment in Nairobi's large informal settlements., Methods: A prospective cohort of 1978 adolescents from 4 neighborhoods near Nairobi were taught empowerment, deescalation, and self-defense skills in six 2-hour sessions. The standard-of-care (SOC) group (n = 428) received a life skills class. Self-reported, anonymous survey data were collected at baseline and 10.5 months after intervention., Results: Annual sexual assault rates decreased from 17.9/100 person-years at baseline to 11.1 at follow-up (rate ratio = 1.61; 95% confidence interval [CI], 1.26-1.86; P < .001); there was no significant change in the SOC group (14.3 to 14.0, rate ratio = 1.02; 95% CI, 0.67-1.57, P = .92). Sexual assault disclosure in the intervention group increased from 56% to 75% (P = .006), compared with a constant incidence of disclosure (53%) in the SOC group. The majority (52.3%) of adolescents in the intervention group reported using skills learned to stop an assault., Conclusions: This intervention decreased sexual assault rates among adolescent girls in Kenya. The intervention was also associated with an increase in the disclosure of assaults, thereby enabling survivors to seek care and support and possibly leading to the identification and prosecution of perpetrators. This model should be adaptable to other settings both in Africa and globally., (Copyright © 2014 by the American Academy of Pediatrics.)

Objectives: We hypothesized that a checklist enhanced by the electronic medical record and a unit-wide dashboard would improve compliance with an evidence-based, pediatric-specific catheter care bundle and decrease central line-associated bloodstream infections (CLABSI)., Methods: We performed a cohort study with historical controls that included all patients with a central venous catheter in a 24-bed PICU in an academic children's hospital. Postintervention CLABSI rates, compliance with bundle elements, and staff perceptions of communication were evaluated and compared with preintervention data., Results: CLABSI rates decreased from 2.6 CLABSIs per 1000 line-days before intervention to 0.7 CLABSIs per 1000 line-days after intervention. Analysis of specific bundle elements demonstrated increased daily documentation of line necessity from 30% to 73% (P < .001), increased compliance with dressing changes from 87% to 90% (P = .003), increased compliance with cap changes from 87% to 93% (P < .001), increased compliance with port needle changes from 69% to 95% (P < .001), but decreased compliance with insertion bundle documentation from 67% to 62% (P = .001). Changes in the care plan were made during review of the electronic medical record checklist on 39% of patient rounds episodes., Conclusions: Use of an electronic medical record-enhanced CLABSI prevention checklist coupled with a unit-wide real-time display of adherence was associated with increased compliance with evidence-based catheter care and sustained decrease in CLABSI rates. These data underscore the potential for computerized interventions to promote compliance with proven best practices and prevent patient harm.

Objective: Timely provision of evidence-based recommendations through computerized physician order entry with clinical decision support may improve use of red blood cell transfusions (RBCTs)., Methods: We performed a cohort study with historical controls including inpatients admitted between February 1, 2008, and January 31, 2010. A clinical decision-support alert for RBCTs was constructed by using current evidence. RBCT orders resulted in assessment of the patient's medical record with prescriber notification if parameters were not within recommended ranges. Primary end points included the average pretransfusion hemoglobin level and the rate of RBCTs per patient-day., Results: In total, 3293 control discharges and 3492 study discharges were evaluated. The mean (SD) control pretransfusion hemoglobin level in the PICU was 9.83 (2.63) g/dL (95% confidence interval [CI]: 9.65-10.01) compared with the study value of 8.75 (2.05) g/dL (95% CI: 8.59-8.90) (P < .0001). The wards' control value was 7.56 (0.93) g/dL (95% CI: 7.47-7.65), the study value was 7.14 (1.01) g/dL (95% CI: 6.99-7.28) (P < .0001). The control PICU rate of RBCTs per patient-day was 0.20 (0.11) (95% CI: 0.13-0.27), the study rate was 0.14 (0.04) (95% CI: 0.11-0.17) (P = .12). The PICU's control rate was 0.033 (0.01) (95% CI: 0.02-0.04), and the study rate was 0.017 (0.007) (95% CI: 0.01-0.02) (P < .0001). There was no difference in mortality rates across all cohorts., Conclusions: Implementation of clinical decision-support alerts was associated with a decrease in RBCTs, which suggests improved adoption of evidence-based recommendations. This strategy might be widely applied to promote timely adoption of scientific evidence.