Behçet's disease (BD) is a systemic vasculitis that may involve a variety of organs. We describe a girl with oculocutaneous albinism (OCA) who, on initial evaluation for popliteal artery occlusion, was noted to have multiple cardiac thrombi. She later developed hemoptysis, which was the result of bilateral pulmonary artery aneurysms. Her clinical picture was consistent with BD. She was initially treated with prednisone and cyclophosphamide, followed by maintenance therapy with infliximab. This case describes a unique patient with OCA who developed BD with large pulmonary aneurysms and has remained stable for over 1 year while receiving infliximab.

Herein we present the largest retrospective case-control series of deep sedation in patients with Rett syndrome, including discussion of the unique aspects of Rett syndrome that make these patients at high risk for sedation. Twenty-one patients with Rett syndrome and 21 control patients who received propofol for deep sedation to facilitate lumbar puncture were compared. Patients with Rett syndrome required significantly less propofol than control patients when standardized for weight and the duration of the procedure (P = .004). Seven of the 21 patients with Rett syndrome compared with none of the control patients experienced a serious adverse event, most of which were due to prolonged apnea (P = .004). All adverse events were transient, and all patients returned to their baseline after the procedure was completed. Sedation of patients with Rett syndrome is associated with a relatively high rate of complications and should not be done without appropriate personnel available who recognize the risks of sedating this unique population.

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Herein we present the largest retrospective case-control series of deep sedation in patients with Rett syndrome, including discussion of the unique aspects of Rett syndrome that make these patients at high risk of sedation. Twenty-one patients with Rett syndrome and 21 control patients who received propofol for deep sedation to facilitate lumbar puncture were compared. Patients with Rett syndrome required significantly less propofol than control patients when standardized for weight and the duration of the procedure ( P = .004). Seven of the 21 patients with Rett syndrome compared with none of the control patients experienced a serious adverse event, most of which were due to prolonged apnea ( P = .004). All adverse events were transient, and all patients returned to their baseline after the procedure was completed. Sedation of patients with Rett syndrome is associated with a relatively high rate of complications and should not be done without appropriate personnel available who recognize the risks of sedating this unique population. ( J Child Neurol 2006;21:210—213; DOI 10.2310/7010.2006.00051).

This study aimed to create a pediatric sedation scoring system independent of the American Society of Anesthesiology Physical Status (ASA-PS) classification that is predictive of adverse events, facilitates objective stratification, and resource allocation. Multivariable regression and machine learning algorithm analysis of 134,973 sedation encounters logged in to the Pediatric Sedation Research Consortium (PSRC) database between July 2007 and June 2011. Patient and procedure variables were correlated with adverse events with resultant β-regression coefficients used to assign point values to each variable. Point values were then summed to create a risk assessment score. Validation of the model was performed with the 2011 to 2013 PSRC database followed by calculation of ROC curves and positive predictive values. Factors identified and resultant point values are as follows: 1 point: age ≤ 6 months, cardiac diagnosis, asthma, weight less than 5th percentile or greater than 95th, and computed tomography (CT) scan; 2 points: magnetic resonance cholangiopancreatography (MRCP) and weight greater than 99th percentile; 4 points: magnetic resonance imaging (MRI); 5 points: trisomy 21 and esophagogastroduodenoscopy (EGD); 7 points: cough at the time of examination; and 18 points: bronchoscopy. Sum of patient and procedural values produced total risk assessment scores. Total risk assessment score of 5 had a sensitivity of 82.69% and a specificity of 26.22%, while risk assessment score of 11 had a sensitivity of 12.70% but a specificity of 95.29%. Inclusion of ASA-PS value did not improve model sensitivity or specificity and was thus excluded. Higher risk assessment scores predicted increased likelihood of adverse events during sedation. The score can be used to triage patients independent of ASA-PS with site-specific cut-off values used to determine appropriate sedation resource allocation.