End-of-life opioid administration on neonatal and pediatric intensive care units: nurses' attitudes and practice.

OBJECTIVES: (1) To compare caregivers attitudes on the use of end-of-life opioid analgesia in neonatal (NICU) and pediatric (PICU) intensive care units. (2) To investigate actual opioid administration to DR (delivery room), NICU and PICU patients in various end-of-life situations. METHODS: (1) Administration of an anonymous self-report questionnaire survey to nurses of 2 level III NICUs and 3 PICUs, presenting 5 hypothetical NICU and PICU patients in end-of-life situations. (2) Retrospective chart review of all deaths at the above mentioned DRs (served by NICU staff), NICUs and PICUs during the years 2008-2009. RESULTS: There was no difference between NICU and PICU nurses in self-proclaimed opioid administration in dying NICU or PICU patients with signs of pain (about 80%) or distress (about 65%). 35.0% of NICU and 44.5% of PICU nurses favoured opioid administration with the implicit aim of active intentional ending of life. Shortening of life as an adverse effect of end-of-life opioid analgesia was acceptable for the majority of PICU (94.5%) and NICU (87.0%) nurses. The rate of dying infants who actually had received opioids was similar in NICUs (41/74, 55.4%) and PICUs (40/68, 58.8%). In contrast, none of the neonates (n=24) who died under primary comfort care in the DR received opioids. CONCLUSIONS: End-of-life opioid administration to primary comfort care patients in the DR differs fundamentally from NICU or PICU handling of dying patients. Once patients are admitted to an intensive care unit, practice and attitudes towards end-of-life opioid administration are similar in NICUs and PICUs.

Novel mutations in sarcomeric protein genes in dilated cardiomyopathy.

Mutations in sarcomeric protein genes have been reported to cause dilated cardiomyopathy (DCM). In order to detect novel mutations we screened the sarcomeric protein genes beta-myosin heavy chain (MYH7), myosin-binding protein C (MYBPC3), troponin T (TNNT2), and alpha-tropomyosin (TPM1) in 46 young patients with DCM. Mutation screening was done using single-strand conformation polymorphism (SSCP) analysis and DNA sequencing. The mutations in MYH7 were projected onto the protein data bank-structure (pdb) of myosin of striated muscle. In MYH7 two mutations (Ala223Thr and Ser642Leu) were found in two patients. Ser642Leu is part of the actin-myosin interface. Ala223Thr affects a buried residue near the ATP binding site. In MYBPC3 we found one missense mutation (Asn948Thr) in a male patient. None of the mutations were found in 88 healthy controls and in 136 patients with hypertrophic cardiomyopathy (HCM). Thus mutations in HCM causing genes are not rare in DCM and have potential for functional relevance.