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Sudden unexplained cardiac death (SUCD) can occasionally occur in nonelderly patients with epilepsy, psychiatric disorders, or no medical history. This study was conducted to aim to analyze whether values of the biomarkers for heart failure are associated with the SUCD. Serum concentrations of N-terminal probrain natriuretic peptide, high-sensitivity C-reactive protein (hs-CRP), and tumor necrosis factor α were analyzed in 57 nonelderly patients with SUCD who was diagnosed at medicolegal autopsy. The subjects were divided into 3 subgroups according to the medical history: (1) epilepsy, (2) psychiatric disorders, and (3) no specific medical history. The results showed that serum hs-CRP levels were significantly high in patients with epilepsy (P = 0.01) or psychiatric disorders (P = 0.01) as compared with the controls. Also, significantly high concentrations of hs-CRP were observed in psychiatric patients with schizophrenia, compared with the controls (P = 0.003) or the other psychiatric diseases (P = 0.01). The level of N-terminal probrain natriuretic peptide and tumor necrosis factor α did not show a significant difference between the SUCD and the controls. These results might suggest the association between high serum hs-CRP levels and the potential impairment of the cardiac function before the fatal event.

Quality management systems are the tools that document the processes and responsibilities to achieve quality policies and objectives. Recently, increasing numbers of clinical laboratories have been certified for ISO 15189, an international certification for hospital laboratories. However, our laboratory remains in the preparation process. Major obstacles in our hospital are the tight budget and the timing of introducing the ISO 15189 system because of the old equipment in our laboratory. To overcome these problems, we have made efforts including study workshops with invited lecturers with expertise, joining the prefectural work- shop for clinical technicians. We have also started to prepare standard operational procedures for each la- boratory test. As the preparation advances, it has become clear that gaining the understanding of the signifi- cance of ISO 15189 of hospital executives is indispensable, especially in respect to funding. It is our respon- sibility to achieve standardized and improved laboratory examinations, in order to contribute to high-quality medical care, research, and education. Achieving the certification for ISO 15189 offers a great opportunity to improve our skills in laboratory medicine.

Aims: Oestrogen has been speculated to play an important role in the pathogenesis of primary biliary cirrhosis (PBC), which mainly affects middle-aged and old-aged females because biliary epithelial cells (BECs) are known to express oestrogen receptors (ERs). Oestrogen-related receptors (ERRs) are constitutively active without oestrogen and competitively inhibit the ER-dependent effects of oestrogen. We clarified the effects of oestrogen and the significance of ERRs along with their association with the pathogenesis of cholangiopathy in PBC., Methods: We investigated the expression of ERs and ERRs and the apoptosis-related cell kinetics in BECs using cultured human BECs and human liver specimens., Results: Although cultured human BECs and the interlobular bile ducts in the liver expressed ERβ, in cultured BECs, oestrogen treatment did not induce significant cell proliferation but increased the expression of a negative cell proliferation regulator (14-3-3σ protein). The cultured BECs constantly expressed ERRα and ERRγ, and oestrogen downregulated the ERRγ expression. Furthermore, the ERRγ expression was determined in the intrahepatic bile ducts and was stronger in the middle-aged and old-aged females, particularly those with PBC, than in the younger females. The ERRγ ligand activated a transcription factor, SP1, and enhanced the expression of the pro-apoptotic Bcl-2 family molecules and Bcl-2 inhibitor-induced apoptosis in cultured BECs., Conclusions: Although oestrogen downregulates the ERRγ expression, the increased ERRγ expression under oestrogen-deficient conditions increases the susceptibility to Bcl-2 family-mediated apoptosis in cultured human BECs of females, particularly those with PBC. Understanding the oestrogen-mediated cell kinetics is important for elucidating the pathogenesis of cholangiopathy in PBC., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Submicroscopic deletion of the 6p subtelomere has recently been recognized as a clinically identifiable syndrome. A distinct phenotype has emerged consisting of developmental delay/mental retardation, language impairment, hearing loss, and ophthalmologic, cardiac, and craniofacial abnormalities, including hypertelorism, midface hypoplasia, small nose, and high arched palate. We describe here a patient with 6p subtelomere deletion associated with congenital glaucoma, severe mental retardation, and growth impairment. Fluorescent in situ hybridization analysis revealed only one 6p25.3 signal. Array comparative genomic hybridization assay showed 2.1 Mb deletion and 4.14 Mb duplication in the 6p25 region. Generally, developmental delay and language impairment are common findings in patients with 6p subtelomere deletion syndrome, but growth impairment is not. Compared to that, the present patient showed atypically severe developmental delay and growth impairment., (© 2013 The Authors. Pediatrics International © 2013 Japan Pediatric Society.)

We synthesized three hydantocidin derivatives and evaluated their herbicidal activity in order to elucidate the role of the spirohydantoin system at the anomeric center of hydantocidin. With application to foliage at 1000 ppm, only α-ureidoamide 14 demonstrated activity, the remaining compounds being found to be inactive.