Transthyretin-related familial amyloidotic polyneuropathy—Progress in Kumamoto, Japan (1967–2010)—

The authors reviewed contribution of Kumamoto University group to the progress of the studies on transthyretin (TTR)-related familial amyloidotic polyneuropathy (TTR-related FAP) for 42 years (from 1967 to 2009). Andrade (1952) first described a large group of patients with FAP in Portugal and Araki et al. (1967) in second discovered similar FAP patients in Arao, Kumamoto, Japan. Owing to progress in biochemical and molecular genetic analyses, FAP is now believed to occur worldwide. As of today, reports of about 100 different points of single or two mutations, or a deletion in the transthyretin (TTR) gene, have been published. The authors’ group has made pioneer works for study of FAP in the world. The focus on therapy in amylodosis will increase sharply as an impetus in near future, and successful treatments are expected

Effect of Rust Inhibitor in Brine on Corrosion Properties of Copper

In this study, the effects of rust inhibitors in brine on corrosion behaviors of copper were investigated by measurement of cathode and anode polarization curves and an immersion test. For rust inhibitors, benzotriazole, sodium benzoate and sodium nitrite were prepared. From measurement results of cathode and anode polarization curves, it was found that the corrosion rate of copper in the benzotriazole solution is low and a stable passive film with excellent corrosion resistance generates on the surface of copper in the solution. In the case of the sodium benzoate solution, the corrosion resistance of the passive film was inferior to that in the benzotriazole solution although the passive film generated on the surface of copper. In contrast, the passive film scarcely generated on the surface of copper in the sodium nitrite solution. The result shows that the rust preventive effect of the solution to copper is weak. Furthermore, the immersion test revealed that the benzotriazole solution has the rust preventive effect to copper. In contrast, the effect of the sodium benzoate solution is weak and that of the sodium nitrite solution is scarcely expected

Mechanism of furosemide resistance in analbuminemic rats and hypoalbuminemic patients

Mechanism of furosemide resistance in analbuminemic rats and hypoalbuminemic patients. To elucidate the mechanism of resistance of hypoalbuminemic patients to furosemide, the effect of this diuretic on urine volume of normal and analbuminemic rats (NAR) and of hypoalbuminemic patients was studied. Intravenous administration of furosemide rapidly enhanced sodium diuresis in normal rats but not in NAR. Total plasma clearance and distribution volume of furosemide were much larger in NAR than in normal rats, while no significant difference in these pharmacokinetic parameters was observed for the unbound fraction of the diuretic between the two animal groups. In contrast, urinary secretion of furosemide was significantly lower in NAR than in normal rats. Injected furosemide bound to albumin markedly promoted diuresis in NAR, while the same dose of albumin alone had no effect, indicating that binding to albumin is essential for the delivery of furosemide to the kidney, the site for its action. Injection of the complex rapidly increased the urine volume of hypoalbuminemic patients who showed a marked resistance to this diuretic. Thus, the resistance to furosemide in both NAR and hypoalbuminemic patients may be explained on the same basis

カゾクセイ アミロイド ポリニューロパチー FAP ノ イデンシ シンダン ガイドライン サクセイ ニ ムケテ

We conducted research into literature on living donor liver transplantation and genetic diagnosis taking into consideration our present clinical situation at the Familial Amyloidotic Polyneuropathy (FAP) group at the Kumamoto University Hospital. Based on this research, we drafted genetic diagnosis guidelines (self-regulations)