Dexmedetomidine-ketamine sedation during bone marrow aspirate and biopsy in a patient with duchenne muscular dystrophy.

Sedation during invasive procedures not only provides appropriate humanitarian care for patients, but also facilitates the completion of invasive procedures. Although generally safe and effective, adverse effects may occur especially in patients with co-morbid diseases. We present the successful use of a combination of dexmedetomidine and ketamine to provide sedation and analgesia in a 21-year-old patient with Duchenne muscular dystrophy (DMD) undergoing bone marrow aspiration and biopsy. Co-morbidities included both depressed myocardial function and impaired respiratory function. Dexmedetomidine was administered as a loading dose of 1 µg/kg over 5 min followed by an infusion of 1 µg/kg/h. Ketamine (20 mg) was administered along with the dexmedetomidine loading dose. An additional 10 mg of ketamine was administered to treat the pain experienced during the placement of the local anesthetic agent prior to the procedure. No clinically significant hemodynamic or respiratory changes were noted. The patient tolerated the procedure well and was discharged home. A review of previously published reports of dexmedetomidine and ketamine for procedural sedation are reviewed.

New bioinformatics approach to analyze gene expressions and signaling pathways reveals unique purine gene dysregulation profiles that distinguish between CD and UC.

BACKGROUND: Expression of purine genes is modulated by inflammation or experimental colitis and altered expression leads to disrupted gut function. We studied purine gene dysregulation profiles in inflammatory bowel disease (IBD) and determined whether they can distinguish between Crohn's disease (CD) and ulcerative colitis (UC) using Pathway Analysis and a new Comparative Analysis of Gene Expression and Selection (CAGES) method. METHODS: Raw datasets for 22 purine genes and 36 probe-sets from National Center for Biotechnology Information (NCBI) GEO (Gene Expression Omnibus) (http://www.ncbi.nlm.nih.gov/projects/geo/) were analyzed by National Cancer Institute (NCI) Biological Resources Branch (BRB) array tools for random-variance of multiple/36 t-tests in colonic mucosal biopsies or peripheral blood mononuclear cells (PBMCs) of CD, UC or control subjects. Dysregulation occurs in 59% of purine genes in IBD including ADORA3, CD73, ADORA2A, ADORA2B, ADAR, AMPD2, AMPD3, DPP4, P2RY5, P2RY6, P2RY13, P2RY14, and P2RX5. RESULTS: In CD biopsies, expression of ADORA3, AMPD3, P2RY13, and P2RY5 were negatively correlated with acute inflammatory score, Crohn's Disease Activity Index (CDAI) or disease chronicity; P2RY14 was positively correlated in UC. In mucosal biopsies or PBMCs, CD and UC were distinguished by unique patterns of dysregulation (up- or downregulation) in purine genes. Purine gene dysregulation differs between PBMCs and biopsies and possibly between sexes for each disease. Ingenuity Pathway Analysis (IPA) revealed significant associations between alterations in the expression of CD73 (upregulation) or ADORA3 (downregulation) and inflammatory or purine genes (

Primary thoracoscopic repair of diaphragmatic hernia with pericostal sutures.

The surgical management of congenital diaphragmatic hernias has traditionally been via laparotomy or thoracotomy. Although laparoscopic and thoracoscopic repairs have been described, most reports are in older infants. We describe a method for primary thoracoscopic repair applied in the immediate neonatal period when no posterolateral rim of diaphragm exists. This simple technique for placing the pericostal sutures is a useful adjunct in the thoracoscopic management of diaphragmatic hernias.

Laparoscopy in the management of antenatally detected liver masses.

Antenatally detected liver masses that are not clearly benign on postnatal investigation pose a management dilemma. Unless the diagnosis is clear, observation alone is risky. Improvements in radiological diagnosis permit confirmation of the benign nature of these masses in some instances, but it is usually difficult to distinguish them from malignant lesions. Since recent advances in ultrasound facilitate identification of liver masses during prenatal life, differential diagnosis of these masses has become a recurring issue in recent years. Laparoscopy may play a major role in the surgical management of right upper quadrant masses detected antenatally. We describe its use in a patient with an antenatally detected liver mass. No clear diagnosis could be made with radiologic investigation in the neonatal period. Definitive diagnosis was made laparoscopically: focal nodular hyperplasia was confirmed with laparoscopy and biopsy. In cases where the etiology of a liver mass remains unclear after radiologic investigation, laparoscopic intervention may prove beneficial in neonates and infants. We present an algorithm for the management of similar antenatally detected right upper quadrant lesions.

Factors influencing survival in newborns with congenital diaphragmatic hernia: the relative role of timing of surgery.

PURPOSE: Controversy persists regarding the factors influencing survival in patients with congenital diaphragmatic hernia (CDH), in particular, the role of timing of surgery. The authors therefore sought to determine such factors and to assess the relative role of timing of surgery on outcome. METHODS: All CDH newborns 1991 through 2002 (n = 111) were divided into those undergoing repair before ("early" n = 35), or after ("late" n = 76) 48 hours. A multivariate analysis was performed to determine the relative impact of various factors on survival rate. RESULTS: Overall survival rate was 64%. There was no effect on survival of heart rate, temperature, systolic blood pressure, age, extracorporeal membrane oxygenation use, mesh use, infections, or intracranial hemorrhage, and there was no difference between early (68%) or late (62%) repair (P =.2). Initial pCO2 greater than 50, pO2 less than 40, cardiac defects, or renal failure significantly decreased survival rate. CONCLUSIONS: Significant factors influencing survival rate in patients with CDH include cardiac defects, renal failure, and the initial blood gases and not the timing of surgery. CDH repair should be based on the optimization of clinical parameters as opposed to a specific time period to improve outcome.

How low can you go? Effectiveness and safety of extracorporeal membrane oxygenation in low-birth-weight neonates.

PURPOSE: Controversy exists regarding the criteria for placement of infants on extracorporeal membrane oxygenation (ECMO) at low birth weights. The authors hypothesized that ECMO is effective and safe in babies under 2 kg and sought to examine outcome and survival rate in these infants. METHODS: All patients less than 30 days old in the Extracorporeal Life Support Organization (ELSO) registry (n = 14,305) were divided into those less than 2 kg (n = 663) and more than 2 kg (n = 13,642). Multiple regression analysis determined factors that predicted survival rate and the lowest safe weight for ECMO. RESULTS: Overall survival rate was 76% and was lower in infants less than 2 kg (> or =2 kg, 77% v <2 kg, 53%, P <.0001). Survival rate was significantly lower for patients with diaphragmatic hernia (CDH), bleeding, and intracranial hemorrhage (ICH) by regression. The incidence of ICH in babies less than 2.0 kg was 6% versus 4% in those more than 2.0 kg (P <.05). Regression analysis determined that the lowest weight at which a survival rate of 40% could be achieved was 1.6 kg. CONCLUSIONS: Cannulation for ECMO may be safe and effective in babies under 2.0 kg and potentially as low as 1.6 kg. Judicious anticoagulation might limit bleeding, which occurred in a minority of these patients.