Professional Skills and Competence for Safe and Effective Procedural Sedation in Children: Recommendations Based on a Systematic Review of the Literature

Objectives. To investigate which skills and competence are imperative to assure optimal effectiveness and safety of procedural sedation (PS) in children and to analyze the underlying levels of evidence. Study Design and methods. Systematic review of literature published between 1993 and March 2009. Selected papers were classified according to their methodological quality and summarized in evidence-based conclusions. Next, conclusions were used to formulate recommendations. Results. Although the safety profiles vary among PS drugs, the possibility of potentially serious adverse events and the predictability of depth and duration of sedation define the imperative skills and competence necessary for a timely recognition and appropriate management. The level of effectiveness is mainly determined by the ability to apply titratable PS, including deep sedation using short-acting anesthetics for invasive procedures and nitrous oxide for minor painful procedures, and the implementation of non-pharmacological techniques. Conclusions. PS related safety and effectiveness are determined by the circumstances and professional skills rather than by specific pharmacologic characteristics. Evidence based recommendations regarding necessary skills and competence should be used to set up training programs and to define which professionals can and cannot be credentialed for PS in children

Fatal cerebral edema associated with serine deficiency in CSF

Two young girls without a notable medical history except for asthma presented with an acute toxic encephalopathy with very low serine concentrations both in plasma and cerebrospinal fluid (CSF) comparable to patients with 3-phosphoglycerate dehydrogenase (3-PGDH) deficiency. Clinical symptoms and enzyme measurement (in one patient) excluded 3-PGDH deficiency. Deficiencies in other serine biosynthesis enzymes were highly unlikely on clinical grounds. On basis of the fasting state, ketone bodies and lactate in plasma, urine and CSF, we speculate that reduced serine levels were due to its use as gluconeogenic substrate, conversion to pyruvate by brain serine racemase or decreased L-serine production because of a lack of glucose. These are the first strikingly similar cases of patients with a clear secondary serine deficiency associated with a toxic encephalopathy