Device nurse intervention facilitates the patients' adaptation to cardiac shock devices in the remote monitoring era.

BACKGROUND: A substantial number of patients with shock devices (implantable cardioverter defibrillators [ICDs] or ICDs with resynchronization [CRTDs]) experience psychological distress. OBJECTIVE: We investigated the device nurse telephone intervention's effect on improving the patient's adaptation to shock devices, quality of life (QOL), and anxiety in the remote monitoring era. METHODS: The patient's adaptation to the device, health-related QOL, and anxiety were investigated by the modified Implanted Devices Adjustment-Japan score (IDAS), Short Form-36, and State-Trait Anxiety Inventory (STAI) before and 1-year after the device nurse telephone intervention, performed every 3 months. A total of 95 patients (median age 69 years and 25 females) participated. Sixty patients had ICDs and 35 CRTDs. Structural heart disease was observed in 72 patients, and idiopathic ventricular arrhythmias in the others. The mean left ventricular ejection fraction was 46% ± 15%. The median duration since the device implantation was 5.2 years. RESULTS: The total IDAS score significantly improved from 28.42 ± 7.11 at baseline to 26.77 ± 7.68 (p = 0.0076) at 1 year. Both the state and trait anxiety significantly improved (from 38.9 ± 9.6 to 35.3 ± 9.0 [<0.0001] and 38.8 ± 10.3 to 36.2±9.8 [p = 0.0044], respectively). The prevalence of patients with a state and trait anxiety of more than 40 decreased from 44 (46%) and 38 (40%) patients before the study to 27 (28 %) and 32 (34 %) at 1 year. The SF-36 mental component summary score significantly increased (50.8 ± 8.3 at baseline to 53.1 ± 7.7 at 1 year, p = 0.0031). CONCLUSIONS: The device nurse intervention facilitated the patient's adaptation to the shock device, increased the health-related QOL, and reduced the patient's anxiety.

Prospective analysis of efficacy and safety of an individualized-midazolam-dosing protocol for sedation during prolonged bronchoscopy.

BACKGROUND: Newer more advanced techniques in bronchoscopy may require longer procedure times, although a standard protocol for sedation during prolonged bronchoscopy has not yet been defined. METHODS: We designed a prospective, non-randomized, single-arm study (UMIN trial number 000003971) using patient questionnaires and vital sign monitoring to assess the efficacy and safety of a standardized midazolam dosing protocol based on gender and age for use during bronchoscopy. The loading dose of midazolam was 0.075mg/kg for men ≤65 years old and women ≤70 and 0.05mg/kg for men ≥66 years and women ≥71 years, with subsequent doses of one-half the loading dose to be administered every 20min. The primary endpoint was tolerability and secondary endpoints included anxiety and recall of procedure, willingness to undergo repeat procedure, and complications. Safety was evaluated in terms of monitored changes in blood pressures, ECG, oxygen saturation, and CO2 content in expiration during the procedure. RESULTS: A total of 204 patients were included in the study. Overall, 163 patients (79.9%) reported "no distress" during the procedure, 185 patients (90.7%) reported "no anxiety," and 175 (85.8%) replied that they would accept a repeat procedure, if necessary. The mean minimum oxygen saturation was 90.2% and the mean maximum expiratory CO2 level was 37.7mmHg. There were no serious complications related to the protocol. CONCLUSIONS: The midazolam dosing protocol examined in this study was safe and effective. It is simple, and it could easily be translated to routine clinical practice.

Echoic features of lymph nodes with sarcoidosis determined by endobronchial ultrasound.

OBJECTIVE: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive technique with a high diagnostic yield used in the investigation of mediastinal diseases including sarcoidosis. Although previous reports have discussed the echoic features of metastatic mediastinal lymph nodes in lung cancer, few have addressed those features of mediastinal lymph nodes with sarcoidosis. We therefore investigated whether the echoic features of lymph nodes with sarcoidosis are distinct when compared to those of metastatic lymph nodes in lung cancer. METHODS: This retrospective analysis was held in one university hospital between April 2007 and June 2011. EBUS-guided biopsies were performed on 219 patients, and thus resulting in sarcoidosis diagnoses in 53 patients. We quantitatively analyzed the echoic morphologic features of 42 lymph nodes from 34 sarcoidosis patients and 59 lymph nodes from 44 patients with lung cancer using digital image analyzing software. RESULTS: In patients with sarcoidosis, 64.3% of the lymph nodes had a round shape, 71.4% had a distinct margin, and 88.1% exhibited homogeneous echogenicity. A germinal center structure was observed in 71.4% of the cases. In the context of shape and margin, no significant difference could be observed between sarcoidosis and lung cancer metastasis. However, homogeneous low echogenicity and the presence of a germinal center structure were observed in sarcoidosis more frequently than in lung cancer. CONCLUSION: Homogeneous low echogenicity and the presence of a germinal central structure may be distinctive echoic features of lymph nodes with sarcoidosis. Analyzing the echogenicity of the mediastinal lymph nodes may help to distinguish sarcoidosis from lung cancer.

Aqueous fraction of Sauropus androgynus might be responsible for bronchiolitis obliterans.

BACKGROUND AND OBJECTIVE: Bronchiolitis obliterans (BO) has been reported to develop following ingestion of Sauropus androgynus (SA), a leafy shrub distributed in Southeast Asia. Little is known about direct effects of SA on airway resident cells or haematopoietic cells in vitro. Identification of the SA component responsible for the development of BO would be an important key to elucidate its mechanism. We sought to elucidate the direct effects of SA on airway resident cells or haematopoietic cells and identify the SA element responsible for the pathogenesis of BO. METHODS: SA dry powder was partitioned into fractions by solvent extraction. Human and murine monocytic cells, epithelial cells and endothelial cells were cultured with SA solution or fractions eluted from SA. We also investigated the effect of SA in vivo using a murine BO syndrome (BOS) model. RESULTS: The aqueous fraction of SA induced significant increases of inflammatory cytokine and chemokine production from monocytic lineage cells. This fraction also induced significant apoptosis of endothelial cells and enhanced intraluminal obstructive fibrosis in allogeneic trachea allograft in the murine BOS model. We found individual differences in tumour necrosis factor α (TNF-α) production from monocytes of healthy controls stimulated by this aqueous fraction of SA, whereas it induced high-level TNF-α production from monocytes of patients with SA-induced BO. CONCLUSIONS: These results suggest that an aqueous fraction of SA may be responsible for the pathogenesis of BO.

[Genetic diagnostics of pathogenic splicing abnormalities in the clinical laboratory--pitfalls and screening approaches].

In recent years, genetic diagnostics of pathogenic splicing abnormalities are increasingly recognized as critically important in the clinical genetic diagnostics. It is reported that approximately 10% of pathogenic mutations causing human inherited diseases are splicing mutations. Nonetheless, it is still difficult to identify splicing abnormalities in routine genetic diagnostic settings. Here, we studied two different kinds of cases with splicing abnormalities. The first case is a protein S deficiency. Nucleotide analyses revealed that the proband had a previously reported G to C substitution in the invariant AG dinucleotide at the splicing acceptor site of intronl/exon2, which produces multiple splicing abnormalities resulting in protein S deficiency. The second case is an antithrombin (AT) deficiency. This proband had a previously reported G to A substitution, at nucleotide position 9788 in intron 4, 14 bp in front of exon 5, which created a de novo exon 5 splice site and resulted in AT deficiency. From a practical standpoint, we discussed the pitfalls, attentions, and screening approaches in genetic diagnostics of pathogenic splicing abnormalities. Due to the difficulty with full-length sequence analysis of introns, and the lack of RNA samples, splicing mutations may escape identification. Although current genetic testing remains to be improved, to screen for splicing abnormalities more efficiently, it is significant to use an appropriate combination of various approaches such as DNA and/or RNA samples, splicing mutation databases, bioinformatic tools to detect splice sites and cis-regulatory elements, and in vitro and/or in vivo experimentally methods as needed.