Nusinersen (ISIS-SMNRx or ISIS 396443) is an antisense oligonucleotide drug administered intrathecally to treat spinal muscular atrophy. We summarize lumbar puncture experience in children with spinal muscular atrophy during a phase 1 open-label study of nusinersen and its extension. During the studies, 73 lumbar punctures were performed in 28 patients 2 to 14 years of age with type 2/3 spinal muscular atrophy. No complications occurred in 50 (68%) lumbar punctures; in 23 (32%) procedures, adverse events were attributed to lumbar puncture. Most common adverse events were headache (n = 9), back pain (n = 9), and post–lumbar puncture syndrome (n = 8). In a subgroup analysis, adverse events were more frequent in older children, children with type 3 spinal muscular atrophy, and with a 21- or 22-gauge needle compared to a 24-gauge needle or smaller. Lumbar punctures were successfully performed in children with spinal muscular atrophy; lumbar puncture–related adverse event frequency was similar to that previously reported in children.

Inhaled epoprostenol reduces pulmonary pressure and improves right ventricular stroke work in patients with pulmonary hypertension undergoing cardiac surgery. A dose of 60 microg is hemodynamically safe, and its effect is completely reversed after 25 minutes. We did not observe any evidence of platelet dysfunction or an increase in surgical bleeding after administration of inhaled epoprostenol.

Inhaled PGI2 can be useful in the treatment of patients with PHT and severe hypoxia. It can however be associated with systemic side effects.

Background: Wake Up Safe, a Patient Safety Organization founded by the Society for Pediatric Anesthesia, collects data on serious adverse events along with demographic data from all pediatric patients receiving anesthesia care at participating institutions. This report reviews all events occurring between 2010 and 2015 and focuses on common adverse events that are anesthesia-related. Aims: Determine which adverse events were most common from 2010 to 2015 among participating Wake Up Safe institutions. Determine how many anesthesiarelated events were deemed to be preventable. Methods: This is a descriptive report. The Wake Up Safe registry data were queried on September 29, 2017. Institutions were included if they had complete demographic data and at least 5 adverse events per year reported. At that time, 19 out of 29 institutions had complete demographic data for events from 2010 to 2015. This study describes demographic data and adverse events from these nineteen institutions. Descriptive data were extracted, and event rate was calculated for each adverse event category. In events that were assessed as primarily related to anesthesia, further detailed analysis was performed. Results: Of all reported adverse events (2544 events), the most common were cardiac arrests (646, 31.6%), respiratory complications (598, 29.2%), and medication events (345, 16.9%). Of all anesthesia-related events (612 events), medication events were the most common (239, 31.9%), followed by respiratory complications (181, 24.1%), and cardiac arrests (139, 18.5%). Overall, 85% of anesthesia-related serious adverse events were deemed somewhat or almost certainly preventable. Conclusions: The majority of anesthesia-related serious adverse events reported to the Wake Up Safe database are preventable. Medication events are the most common anesthesia-related adverse events. Innovations aimed at decreasing medication events may be the most impactful.

General anesthetics mitigate distress and exaggerated hemodynamic responses to pain and stressful stimulation, allowing surgery and diagnostic procedures to be performed worldwide in millions of children every year. Emerging studies, mainly carried out in early postnatal laboratory animals, demonstrate widespread neuronal elimination, alteration in neuronal circuitry, and long-term neurological disabilities following exposure to all commonly used sedatives and anesthetics. These findings have raised concerns among parents, anesthesiologists, neuroscientists, and government regulators about the safety of anesthetic drugs in children, especially infants. Accumulating evidence from epidemiological studies suggests an association between surgery with anesthesia in early childhood and subsequent behavioral and cognitive abnormalities. During the Fourth Pediatric Anesthesia NeuroDevelopmental Assessment (PANDA) symposium, a meeting attended by many stakeholders, the most recent findings in the field were presented and discussed. This review summarizes the current state of clinical research into the effects of anesthetic exposure in human brain development, addresses some of the difficulties in examining the phenomenon, and introduces the most recent clinical findings presented at the PANDA symposium. The unanimous consensus among participants was that additional preclinical and clinical research efforts are urgently required to address this important concern for child health.