Neuro-ventilatory efficiency during weaning from mechanical ventilation using neurally adjusted ventilatory assist.

BACKGROUND: Neuro-ventilatory efficiency (NVE), defined as the tidal volume to electrical diaphragm-activity ratio (VT/EAdi) at the beginning and end of the weaning process after acute hypoxaemic respiratory failure, may provide valuable information about patient recovery. METHODS: This observational study included 12 patients breathing with neurally adjusted ventilatory assist (NAVA). When a spontaneous breathing trial (SBT) with pressure support of 7 cm H2O and PEEP was unsuccessful, NAVA was used and the level was adjusted to obtain an EAdi of ∼60% of maximal EAdi during SBT. VT and EAdi were recorded continuously. We compared changes in NVE between NAVA and SBT at the first failed and first successful SBT. RESULTS: When patients were switched from NAVA to SBT, NVE was significantly reduced during both unsuccessful and successful SBT (-56 and -38%, respectively); however, this reduction was significantly lower when SBT was successful (P=0.01). Between the first and last day of weaning, we observed that NVE decreased with NAVA [40.6 (27.7-89.5) vs 28.8 (18.6-46.7); P=0.002] with a significant decrease in NAVA level, whereas it remained unchanged during SBT [15.4 (10.7-39.1) vs 19.5 (11.6-29.6); P=0.50] with significant increases in both EAdi and VT and no difference in respiratory rhythm. CONCLUSIONS: These results suggest that in patients after respiratory failure and prolonged mechanical ventilation, changes in VT and NVE, between SBTs are indicative of patient recovery. Larger clinical trials are needed to clarify whether changes in NVE reliably predict weaning in patients ventilated with NAVA.

Tooth developmental anomalies in severe combined immunodeficiency disease and juvenile myelomonocytic leukemia: common clinical features and treatment outcomes.

AIM: Human Severe Combined Immunodeficiency (SCID) is a prenatal disorder of T lymphocyte development that depends on the expression of numerous genes. Juvenile myelomonocytic leukemia (JMML), previously known as juvenile chronic myeloid leukemia (JCML), is a rare, myelodysplastic/myeloproliferative disease typically presenting in early childhood. CASE REPORTS: Two cases are described of immunodeficiency disorders, both treated with chemotherapeutic drugs (Busulfan plus cyclophosphamide) before bone marrow transplantation. After treatment, these two different cases showed several similar oral lesions: microdontia, root alterations, numerous tooth ageneses, incomplete calcification, enamel hypoplasia, premature apexification and hypodontia. Both subjects underwent dental and orthodontic treatment. The first phase comprised orthopaedic treatment using a removable appliance (Interim-G®) followed by rapid palatal expansion; in the second phase patients underwent tooth extraction and were treated using fixed appliances for 19 and 26 months, respectively (mean 2 years) to obtain final alignment and maximum intercuspation. In the third and final phase, reconstruction of malformed teeth was completed, and implant-supported protheses were applied. CONCLUSION: The difficulties of managing and treating these diseases are discussed, with particular focus on tooth anomalies and malocclusion disorders. Collaboration between dentist and paediatrician in dealing with patients with a variety of oral lesions and tooth anomalies is important in order to prevent any other possible tooth lesions and ensure correct jaw development.