Dilated cardiomyopathy associated with a mutation in the dispatched RND transporter family member 1 gene.

Dilated cardiomyopathy is the most common presentation of cardiomyopathy in children with 20-35% of patients having an identified genetic component. There are more than 30 genes implicated in the pathogenesis of dilated cardiomyopathy. We present the first report of a female infant with dilated cardiomyopathy with a genetic variant in the dispatched RND transporter family member 1 gene.

Assessment of Phenobarbital Administration to Reduce Opioid and Benzodiazepine Use in the Immediate Postoperative Period Following Stage 2 Single-Ventricle Palliation.

OBJECTIVE: The Glenn procedure may lead to the development of elevated cerebral venous pressures, which is believed to result in "Glenn headaches." This manifests as excessive irritability, often requiring significant use of opioids and benzodiazepines. This study was designed to report our experience with the use of phenobarbital in the postoperative phase after the Glenn procedure. METHODS: We performed a retrospective chart review to compare Glenn patients before and after implementation of a sedation protocol using phenobarbital. The 2 groups were compared for demographics, surgical characteristics, and cumulative sedation usage. Correlation coefficients between the preoperative catheterization variables and sedation usage were also calculated. RESULTS: Groups A (pre-phenobarbital; n = 8) and B (post-phenobarbital; n = 11) were comparable in terms of demographics, cardiac anatomy, preoperative catheterization data, and hemodynamics. Patients in Group B received a median dose of 21.8 mg/kg of phenobarbital during their ICU stay. Although there was a decreased administration of morphine equivalents (2.60 mg/kg vs 2.25 mg/kg, p = 0.38), benzodiazepine (0.1 mg/kg vs 0.074 mg/kg, p = 0.43), and dexmedetomidine (47 mcg/kg vs 37.2 mcg/kg, p = 0.53) in Group B, the differences were not statistically significant. There was also no strong correlation between preoperative hemodynamic variables and the postoperative sedation requirement, and there was no statistically significant difference in overall outcomes between the 2 groups. CONCLUSIONS: While phenobarbital may have mitigated the use of opioids, benzodiazepines, and alpha-agonist agents in some postoperative Glenn patients, the overall findings for all patients were not statistically significant. Further prospective studies are needed to ascertain the role of phenobarbital in these patients.

Improving the potential for organ donation in an inner city Muslim American community: the impact of a religious educational intervention.

OBJECTIVE: We aimed to assess the willingness of Muslim Americans to be potential organ donors, to describe potential religious barriers to organ donation, and to evaluate the efficacy of a brief religious educational intervention. METHODS: Face-to-face survey with English-, Arabic-, and Urdu-speaking Muslim American adults in places of worship and gatherings. The two-part survey included questions about demographics and organ donation. A brief educational intervention followed, which included an explanation of organ donation, along with the evidence for Islam's support for organ donation. After this intervention, the questions about organ donation and brain death were repeated. RESULTS: The response rate was 81% (231 of 285). Fifty percent of the respondents would donate their organs. Twenty-five percent changed their opinion and accepted the idea of being donors after the educational intervention. Lack of awareness of the support of Islam to organ donation and fear of disfigurement were the most common barriers cited. CONCLUSION: Muslim Americans are less likely than Caucasian Americans to be organ donors, and the perceived lack of support from Islam for organ donation is a factor. The effectiveness of our brief religious education intervention suggests that further education may improve organ donation rates among the Muslim community.

Lemierre-like syndrome caused by community-associated methicillin-resistant Staphylococcus aureus complicated by hemorrhagic pericarditis.

OBJECTIVE: To describe an infant with Lemierre-like syndrome caused by community-associated methicillin-resistant Staphylococcus aureus whose course was complicated by hemorrhagic pericarditis. DESIGN: Case report. SETTING: A 30-bed, pediatric intensive care unit at a tertiary care children's hospital. PATIENT: A 7-month-old infant presented with fever and torticollis attributable to a retropharyngeal abscess and left internal jugular venous thrombosis. He was treated with antibiotics and anticoagulation, and his course was complicated by hemorrhagic pericarditis and cardiac tamponade. INTERVENTIONS: Resuscitation of shock; video-assisted thoracoscopic drainage of bilateral empyema with pleural decortication; vancomycin and clindamycin treatment of methicillin-resistant Staphylococcus aureus; incision and drainage of retropharyngeal abscess; treatment of internal jugular venous thrombus with anticoagulation; and treatment of pericardial tamponade by insertion of pericardial drain. MEASUREMENTS AND MAIN RESULTS: Methicillin-resistant Staphylococcus aureus from blood and pleural fluid peel cultures were multi-locus sequence type 8, Panton-Valentine leukocidin-positive, and contained SCCmec IV, findings consistent with USA300 pulsotype. There was complete recovery from this life-threatening infection. CONCLUSIONS: Septic jugular venous thrombophlebitis complicating upper airway infections is a rare but serious acute medical condition. We present an infant with methicillin-resistant Staphylococcus aureus infection and clinical features that overlapped those described by Lemierre, in whom hemorrhagic pericarditis developed while receiving anticoagulation therapy. Anticoagulation, if used, should be closely monitored in this population.