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LT for PFIC type 1 is often complicated by postoperative diarrhea and recurrent graft steatosis. A 26-month-old female child with cholestatic jaundice, pruritus, diarrhea, and growth retardation revealed total bilirubin 9.1 mg/dL, gamma-glutamyl transpeptidase 64 IU/L, and TBA 295.8 μmol/L. Genetic analysis confirmed ATP8B1 defects. A LT (segment 2, 3 graft) from the heterozygous father was performed. Biliary diversion was performed by a 35-cm jejunum conduit between the graft hepatic duct and the mid-transverse colon. Stools became pigmented immediately. Follow-up at 138 days revealed resolution of jaundice and pruritus and soft-to-hard stools (6-8 daily). Radioisotope hepato-biliary scintigraphy (days 26, 68, and 139) confirmed unobstructed bile drainage into the colon (t1/2 34, 27, and 19 minutes, respectively). Contrast meal follow-through at day 62 confirmed the absence of any colo-jejuno-hepatic reflux. At 140 days, contrast follow-through via the biliary stent revealed patent jejuno-colonic anastomosis and satisfactory transit. Graft biopsy at LT, 138 days, and 9 months follow-up revealed comparable grades of macrovesicular steatosis (<20%). TIBD during LT may be a clinically effective stoma-free biliary diversion and may prevent recurrent graft steatosis following LT for PFIC type 1. [ABSTRACT FROM AUTHOR]

We assessed the serological response to pertussis vaccines administered pre- and post-liver transplantation in 58 pediatric patients at a children's hospital in Japan. A high rate of pertussis vaccine failure was observed, 44.8% against the pertussis toxin and 69.0% against filamentous hemagglutinin, with no difference in the seropositivity rate with respect to the timing of the vaccination during the peritransplant period. [ABSTRACT FROM AUTHOR]

The article discusses research on the expression of various skin autoantigens and autoimmune regulator (AIRE) in thymoma in a patient with pemphigus foliaceus (PF) and in other thymic tumours. Topics covered include the unique antigen expression showed by PF thymoma, the histopathology of the tumour, and the expression of AIRE in all tumours by immunoblotting (IB), immunohistochemistry (IHC) and reverse transcription polymerase chain reaction (RT-PCR).

Background Hepatic arterial reconstruction during living donor liver transplantation ( LDLT) is a very delicate and technically complicated procedure. Post- LDLT hepatic arterial complications are associated with significant morbidity and mortality. Methods We retrospectively analyzed the details of post-operative hepatic arterial complications in 673 consecutive adult LDLT recipients between January 1996 and September 2009. Results Hepatic arterial complications occurred in 43 of 673 adult recipients (6.4%) within a median of 13 post-transplant days (range, 1-63). These included hepatic artery thrombosis (including anastomotic stenosis) in 33 cases, anastomotic bleeding in seven cases, and rupture of anastomotic aneurysm in three cases. To treat these complications, surgical re-anastomosis was performed in 26 cases, while the other 17 cases underwent conservative therapies, including four angioplasties by interventional radiology. Biliary complications after hepatic arterial complications occurred in 17 cases. The overall survival rate after LDLT was significantly lower in the hepatic arterial complication group compared with that in the non-complication group (60.7% vs. 80.1% at one yr, 44.3% vs. 74.2% at five yr, respectively; p < 0.001). Multivariate analysis showed that the extra-anatomical anastomosis (p = 0.011) was the only independent risk factor for hepatic arterial complications. Conclusion Because hepatic arterial complications after LDLT are associated with poor patient survival, early diagnosis and immediate treatment are crucial. The anatomical anastomosis may be the first choice for the hepatic arterial reconstruction to the extent possible. [ABSTRACT FROM AUTHOR]

Aims/hypothesis: Hyperlipidaemia is an independent risk factor for the progression of diabetic nephropathy, but its molecular mechanism remains elusive. We investigated in mice how diabetes and hyperlipidaemia cause renal lesions separately and in combination, and the involvement of Toll-like receptor 4 (TLR4) in the process. Methods: Diabetes was induced in wild-type (WT) and Tlr4 knockout (KO) mice by intraperitoneal injection of streptozotocin (STZ). At 2 weeks after STZ injection, normal diet was substituted with a high-fat diet (HFD). Functional and histological analyses were carried out 6 weeks later. Results: Compared with treatment with STZ or HFD alone, treatment of WT mice with both STZ and HFD markedly aggravated nephropathy, as indicated by an increase in albuminuria, mesangial expansion, infiltration of macrophages and upregulation of pro-inflammatory and extracellular-matrix-associated gene expression in glomeruli. In Tlr4 KO mice, the addition of an HFD to STZ had almost no effects on the variables measured. Production of protein S100 calcium binding protein A8 (calgranulin A; S100A8), a potent ligand for TLR4, was observed in abundance in macrophages infiltrating STZ-HFD WT glomeruli and in glomeruli of diabetic nephropathy patients. High-glucose and fatty acid treatment synergistically upregulated S100a8 gene expression in macrophages from WT mice, but not from KO mice. As putative downstream targets of TLR4, phosphorylation of interferon regulatory factor 3 (IRF3) was enhanced in kidneys of WT mice co-treated with STZ and HFD. Conclusions/interpretation: Activation of S100A8/TLR4 signalling was elucidated in an animal model of diabetic glomerular injury accompanied with hyperlipidaemia, which may provide novel therapeutic targets in progressive diabetic nephropathy. [ABSTRACT FROM AUTHOR]

The present tectonics of Northeast Asia has been extensively investigated during the last 12 yr by using GPS techniques. Nevertheless, crustal velocity field of the southeast of Russia near the northeastern boundaries of the hypothesized Amurian microplate has not been defined yet. The GPS data collected between 1997 February and 2009 April at sites of the regional geodynamic network were used to estimate the recent geodynamic activity of this area. The calculated GPS velocities indicate almost internal (between network sites) and external (with respect to the Eurasian tectonic plate) stability of the investigated region. We have not found clear evidences of any notable present-day tectonic activity of the Central Sikhote-Alin Fault as a whole. This fault is the main tectonic unit that determines the geological structure of the investigated region. The obtained results speak in favour of the existence of a few separate blocks and a more sophisticated structure of the proposed Amurian microplate in comparison with an indivisible plate approach. [ABSTRACT FROM AUTHOR]

Overproduction of corticotrophin-releasing factor (CRF), the major mediator of the stress response, has been linked to anxiety, depression and addiction. CRF excess results in increased arousal, anxiety and altered cognition in rodents. The ability to adapt to a potentially threatening stimulus is crucial for survival, and impaired adaptation may underlie stress-related psychiatric disorders. Therefore, we examined the effects of chronic transgenic neural CRF overproduction on behavioural adaptation to repeated exposure to a non-home cage environment. We report that CRF transgenic mice show impaired adaptation in locomotor response to the novel open field. In contrast to wild-type (WT) mice, anxiety-related behaviour of CRF transgenic mice does not change during repeated exposure to the same environment over the period of 7 days or at retest 1 week later. We found that locomotor response to novelty correlates significantly with total locomotor activity and activity in the centre at the last day of testing and at retest in WT but not in CRF transgenic mice. Mice were divided into low responders and high responders on the basis of their initial locomotor response to novelty. We found that differences in habituation and re-exposure response are related to individual differences in locomotor response to novelty. In summary, these results show that CRF transgenic mice are fundamentally different from WT in their ability to adapt to an environmental stressor. This may be related to individual differences in stress reactivity. These findings have implications for our understanding of the role of CRF overproduction in behavioural maladaptation and stress-related psychiatric disorders. [ABSTRACT FROM AUTHOR]

Brain natriuretic peptide (BNP) is a potent vasorelaxing and natriuretic peptide that is secreted from the heart and has cardioprotective properties. We have previously generated hypotensive transgenic mice (BNP-Tg mice) that overproduce BNP in the liver, which is released into the circulation. Using this animal model, we successfully demonstrated the amelioration of renal injury after renal ablation and in proliferative glomerulonephritis. Glomerular hyperfiltration is an early haemodynamic derangement, representing one of the key mechanisms of the pathogenesis of diabetic nephropathy. Based on the suggested involvement of increased endogenous natriuretic peptides, the aim of this study was to investigate their role in the development and progression of diabetic nephropathy. We evaluated the progression of renal injury and fibrogenesis in BNP-Tg mice with diabetes induced by streptozotocin. We also investigated the effect of BNP on high glucose-induced signalling abnormalities in mesangial cells. After induction of diabetes, control mice exhibited progressively increased urinary albumin excretion with impaired renal function, whereas these changes were significantly ameliorated in BNP-Tg mice. Notably, diabetic BNP-Tg mice revealed minimal mesangial fibrogenesis with virtually no glomerular hypertrophy. Glomerular upregulation of extracellular signal-regulated kinase, TGF-β and extracellular matrix proteins was also significantly inhibited in diabetic BNP-Tg mice. In cultured mesangial cells, activation of the above cascade under high glucose was abrogated by the addition of BNP. Chronic excess of BNP prevents glomerular injury in the setting of diabetes, suggesting that renoprotective effects of natriuretic peptides may be therapeutically applicable in preventing the progression of diabetic nephropathy. [ABSTRACT FROM AUTHOR]

The parameters of split S waves from local weak earthquakes along eastern Hokkaido Island are studied over the period of 2003, including the strong Tokachi-oki September 26, 2003 earthquake ( M = 8.0). Earthquake records of five stations belonging to the ISV seismological network were used. The studies of the split S wave parameters showed that they vary in space and time along Hokkaido Island. The zones of the Hidaka Mountains (ERM, MYR), Tokachi Plain (IWN, URH), and Kushiro Plain (AKK) are distinguished along Hokkaido. The anisotropy coefficients beneath the ERM, MYR, IWN, URH, and AKK stations attain 10.5, 10, 5, 3.5, and 6.5%, respectively. Beneath ERM, azimuths of the fast S wave (ϕ) are predominantly in the N-S direction until July and in the E-W direction from July (parallel and normal to the Japan trench strike). By the time of the Tokachi-oki earthquake, the ϕ directions were oriented SE in agreement with the direction of the Pacific plate motion. The ϕ directions on the northern side of the Hidaka Range (MYR) are predominantly orthogonal to those beneath ERM, which can be evidence for differences in the direction of deformations on opposite sides of the range. Higher seismicity, the variation of S wave parameters, and a high anisotropy of the medium point to an intense development of deformation (dilatancy) processes in the area of the Hidaka Mountains. The fast wave azimuths beneath AKK are predominantly 50°–70°, and this orientation is consistent with the direction of migration of the Kurile arc front along the trench. Beneath IWN, the azimuths ϕ are oriented along the N-NE directions, and beneath URH, along the direction of the Pacific plate motion (100°–150°). Strengthening of mechanical properties of the medium and development and accumulation of shear deformations in a subhorizontal plane are supposed to take place in the Tokachi Plain area. [ABSTRACT FROM AUTHOR]

The effects of crown ethers were examined for the intramolecular two-site jumping rates of sodium cations in 2,5-di- tert -1,4-benzoquinone ion pairs using a high-pressure electron spin resonance (ESR) technique. The activation volumes for the intramolecular migration of the sodium cation (Δ V 1 ‡ ) and the inclusion complex of the sodium cation with crown ethers (Δ V 2 ‡ ) were estimated to be -7.4 and 9.5-13.8 cm 3 mol -1 , respectively. Further, the reaction volumes (Δ V ) for the Na + /crown-ether complex formation were determined as 15.6-17.5 cm 3 mol -1 in 1,2-dimethoxyethane. Based on the results, the mechanism for intramolecular sodium cation migration in the presence of crown ethers is discussed from the perspective of volume. [ABSTRACT FROM AUTHOR]

Studies the application of proton-induced x-ray emission to the characterization of size-segregated single raindrops. Key issues of interest; Analysis of pertinent issues and relevant topics; Implications on proton-induced x-ray emission analysis.