Variegate porphria (VP)は,肝におけるヘム合成系の酵素異常を原因とし,神経,筋症状と共に皮膚症状を呈することが知られる.我々は四肢麻痺を示したVPの1例を経験し,肝におけるδ-aminolevurinic acid (ALA)合成活性の特異な上昇を証明した.症例は33才,女性で,第一子出産後,腹痛,嘔吐で発症し,フェノバール投与後四肢麻痺をおこし,同時に肝機能,電解質の異常を示した.皮膚の光線過敏,皮疹,および急性期に尿中ALA, porphobirinogenが上昇し,便中のcoproporphyrin, protoporpkyrinが寛解期にも高値を示したことからVPと診断された.肝性porphyriaには4型が知られるが,各病型と肝ヘム合成系の異常については不明の点が多い.そこで,本例の肝生検組織におけるALA合成活性をsuccinyl CoAを基質としたS活性とα-ketoglutarateを基質としたK活性について測定した. S活性はacute intermittent porphyria (AIP)例と同程度に上昇し, K活性はAIPやporphyria cutanea tarda例の2～5倍にあたる上昇を認めた.生検所見では肝には電顕所見で層状となつた膜様の特異な構造を認め,脂肪滴の沈着が見られた.これは従来の報告とあわせ肝性porphyriaに特異な所見と思われる.肝におけるALA合成K活性の特異な上昇は本例の多量のporphyrin体産生や,重度の神経筋症状に大きな影響を与えたと思われた. K活性はS活性より生理的なALA合成をよりよく反映すると思われ,これを中心に肝性porphyriaにおける酵素異常について考察した.

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シルクは、生体適合性に優れていいるため、古くから手術用縫合糸として使用されてきた。演者らは、このシルクの特性を人工皮膚や人工血管に応用すべく、研究開発を行っている。人工血管は、太いもので直径3～4cm、細いもので直径4～5mmのものがポリエステルなどを基材として実用化されている。しかし、直径1mm、あるいは1.5mmといった人工血管にすれば極細のものは作出が困難とされてきた。演者らは、以前直径2mmあるいは4mm程度のものを作出して報告したが¹⁾、今回極細の人工血管用基材を作出するために、フィプロイン繊維とそのバインダーとして液状フィプロインを用いたフィプロイン100%の人工血管用基材を作出し、小動物へ移植試験を行ったので、その概要を報告する。

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To study the disturbance of metabolism of plasma lipids and apoproteins in a familial lecithin: cholesterol acyltransferase deficiency, the effects of a low fat diet on plasma lipids, apoproteins and plasma enzyme activities were investigated. The plasma apoprotein A-1 and E levels were measured by a radioimmunoassay. The apoprotein B level was determined by the method of a single radial immunodiffusion. Lipoprotein lipase and hepatic triglyceride lipase activities were measured by an immunochemical method utilizing antiserum prepared against hepatic triglyceride lipase. By the restriction of fat in diet, the amount of triglyceriderich lipoproteins were decreased. Also, plasma apoprotein E and B levels were markedly decreased, while the improvements of both lipoprotein lipase and hepatic triglyceride lipase was not observed. These results suggest that high plasma apoprotein E in the familial lecithin: cholesterol acyltransferase deficiency is strongly related to the disturbed triglyceride-rich lipoprotein metabolism in the lecithin: cholesterol acyltransferase deficient state.

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福島原発事故後、放射性物質による環境汚染に、南相馬、浪江、楢葉の３つの自治体の除染と環境回復の取り組みを支援している。最新のゲノム科学をふまえ、こどもと妊婦をまもり地域の復興のため進めてきた、１１年の幼稚園の除染、１２年度コメの全品検査機の開発、１３年度常磐自動車道の開通、１４年度サカナの検査機の開発、１５年度放射能汚染廃棄物のリサイクル焼却場の建設支援など現地の状況を報告する。

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LCAT mass and its activity were measured in Japanese cases with familial LCAT deficiency. They were the menbers of the third family found in Japan.
Haptoglobin types were 2-2 in all cases. Among plasma apoproteins, A-I, A-II, B, D levels were decreased in a homozygote. In heterozygotes, plasma apoprotein levels were almost normal. Plasma cholesterol-cholesterol ester ratio was 60-70% of normal range even in heterozygotes. Plasma LCAT activity was below 10% of normal range in a homozygote and 60% in heterozygotes, these were similar to other cases previously reported. Plasma LCAT mass was 35% in a homozygote and almost normal in heterozygotes. In other reports, LCAT mass was undetectable in homozygotes and 50% in heterozygotes. In this Japanese family, homozygote has no LCAT activity but has 35% of normal LCAT mass. Heterozygotes have half-normal activity and normal mass. This suggests that deficiency of LCAT activity in this family is caused by presence of LCAT like protein which has no activity.

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Serum levels of Apo A-I and Apo A-II were determined by single radial immunodiffusion method using DAIICHI PURE CHEMICAL plates. High density lipoprotein subfractions (HDL₂, 1.063<d<1.125 and HDL₃, 1.125<d<1.21) were isolated by sequential ultracentrifugation. Blood samples were drawn from 87 cases included normal controls, hyperlipoproteinemia, liver cirrhosis, myocardial infarction and other diseases. Apo A-I correlated with HDL₂-C (r=0.461, p<0.01) and also correlated with HDL₃-C (r=0.553, p<0.01). Apo A-II did not correlated with HDL₂-C (r=0.085, N. S.) and correlated with only HDL₃-C (r=0.613, p<0.01).
Changes of apo A-II levels were more prominent than changes of apo A-I levels. Apo A-II levels were increased in hypercholesterolemia with and without hypertriglyceridemia. Liver cirrhosis showed decrease in apo A-II significantly and increase in the ratio of apo A-I/apo A-II. Liver cirrhosis showed very low levels of HDL₃-C and high ratio of HDL₂-C to HDL₃-C.
Apo A-I and apo A-II levels in myocardial infarction were not changed from the levels in control. Only HDL₂ fraction was decreased in myocardial infarction and the ratio of HDL₂-C/HDL₃-C was decreased in myocardial infarction. The ratios of HDL-C/apo A-I, HDL-C/apo A-II, HDL-PL/apo A-I, and HDL-PL/apo A-II were significantly decreased in myocardial infarction and hypertriglyceridemia. These ratios may be useful as clinical markers for discriminate risk state of atherosclerosis.

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