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Objective: The aim was to clarify the role of serotonin (5-hydroxytryptamine, 5-HT) in monocrotaline induced pulmonary hypertension. Methods: Plasma 5-HT levels, pulmonary capillary platelet count, and vascular responsiveness to 5-HT were evaluated in the model. The effects of the selective 5-HT2 receptor antagonist, DV- 7028, on the development of pulmonary hypertension were also investigated. Results: Plasma 5-HT was raised 12 h to 3 d after monocrotaline administration (60 mg·kg−1), coinciding with accumulation of platelets in the pulmonary circulation. Isolated pulmonary arteries showed hyperreactivity to 5-HT at 14 and 21 d after monocrotaline. Administration of DV-7028 (20 mg·kg−1·d−1) attenuated the increase in pulmonary arterial pressure, right ventricular hypertrophy, and medial thickening of the pulmonary arteries. Conclusions: The present study suggests that 5-HT released from platelets contributes to the initiation and progression of monocrotaline induced pulmonary hypertension. Cardiovascular Research 1993; 27 :1619-1623

A large, poorly demarcated, elevated lesion over the area ranging from the lower end of the esophagus to the lesser curvature of the cardiac region was examined in a 19 year old woman, and demonstrated an irregular and highly infiltrative proliferation of spindle cells over the full thickness of the gastric wall. Although the spindle cells grew mainly in the submucosa, they penetrated the muscularis propria, leaving intact muscle tissue that formed an irregular, island-like pattern, and reached the subserosa. The cells were identified as myofibroblasts by light microscopy, immuno-histochemistry and electron microscopy. The present case is considered to be of an inflammatory pseudotumor due to extremely infiltrative growth of myofibroblasts. This lesion differed from inflammatory fibroid polyp (similarly consisting of proliferating myofibroblasts accompanied by inflammatory cells) in terms of age at onset, tumor size, pattern of cell proliferation and main inflammatory cell component. This report also discusses the relationship between this lesion and a very recently advocated new entity, inflammatory fibrosarcoma of the mesentery and retroperito-neum, which is a tumour closely simulating inflammatory pseudotumor.

Background. Papillary carcinoma of the thyroid frequently occurs in young women. Four cases of papillary carcinoma were found in women in their 20s, and the disease was characterized by unusual morphologic and clinical features. Methods. Four cases were analyzed clinically, grossly, microscopically, ultrastructurally, and immuno-histochemically and compared with control cases of papillary carcinoma. Results. The tumors histologically showed various appearances with trabecular, solid cribriform, follicular, and papillary patterns. A striking finding was that stratified cancer cell nests with prominent nuclear clearings were scattered in the tumors. Ultrastructurally, the nuclear clearings were composed of fine thread-like fibrils, and they were bound unselectively to various antibodies against hormones and intermediate filaments. The four patients also had common clinicopathologic features as follows: they were all women in their 20s and their preop-erative serum thyroglobulin levels were all normal despite the large size of their thyroid tumors. The tumors were grossly well encapsulated, and there was no lymph node metastasis, except in one patient who had only one positive node. Conclusions. The peculiar nuclear features were distinct from intranuclear cytoplasmic inclusions or ground-glass nuclei, and these have not been reported previously to the authors' knowledge in conventional thyroid neoplasms. These tumors might represent another subtype of papillary thyroid carcinoma.

The distribution of elastic fibers and elastin mRNA was studied in the uvea of 19-day-old chick embryos by light and electron microscopy and in situ hybridization techniques using a 35S-labeled RNA probe. Light and electron microscopic examination revealed that elastic fibers were present in the uveal portion of the ciliary body, but not in the posterior choroid. Electron microscopically, elastic fibers were also found in Bruch's membrane. Abundant amounts of elastin mRNA were strictly localized in the fibroblasts in the uveal portion of the ciliary body, and no elastin mRNA was found in any of the cells in the posterior choroid by in situ hybridization. Cultured uveal tissue obtained from the ciliary body was found to produce large amounts of tropoelastin as compared with cultured posterior choroid. Using V8 protease peptide mapping and Northern blot analysis, the tropoelastin and its mRNA produced by the ciliary body were identical to those produced by the smooth muscle cells in the aorta. The results suggest that elastin is synthesized in the ciliary body, acting as a supporting substance in this area.

Vestibular sensory epithelia were studied histologically and ultrastructurally in 17 cases with acoustic neurinoma (AN). The superior vestibular nerve (SVN) near the fundus was also histologically studied in S of these 17 cases. Histologically, severe fibrotic change of the vestibular sensory epithelia was found in 1 case, and severe fibrotic change of the SVN was also found in this case. Intra-epithelial cysts were found at the edge of the utricular sensory epithelia in 2 cases. The cysts consisted of the transitional epithelium and were filled with the darkly stained substances. Ultrastructurally, abnormal accumulation of darkly stained masses within the nerve ending and abnormal accumulation of the fibrillar material below the normal basal lamina were frequently observed. These morphological changes described above were regarded as the pathological changes due to AN. In addition, the relationship among the histologic changes, ultrastructural changes and clinical data were fully investigated, and some histologic and ultrastructural changes were regarded as artifacts or age-related changes.

A 7 year old boy with giant cell fibroblastoma (GCF) of the skin and subcutaneous tissue of the right chest wall is described. To date, the histogenesis of GCF has not been clarified. The reason for the diversity of immunohistochemical data among various authors may be because the specimens studied were from only part of the lesion, or reduction of antigenicity through the preparation process. However, our findings based on studies of many specimens from various parts of the tumor for accurate immunohistochemical evaluation suggest that GCF may be a myofibro histiocytic tumor. Recently, the suggestion that GCF is a juvenile form of dermatofibrosarcoma protuberans (DFSP) has been reported. In addition to the present case, we performed immunohistochemical examination of 10cases of definitely diagnosed DFSP for comparison. The immunohistochemical characteristics of these two neoplasms were concordant. However, from clinical and morphological viewpoints, it seems premature to recognize GCF as a juvenile form of DFSP. Acta Pathol Jpn 41: 552–560, 1991.

We present a case of acute lymphoblastic leukemia (ALL) in which the leukemic cells had cytoplasmic azurophilic granules. Surface marker studies revealed that the leukemic cells expressed CD10 (CALLA), CD19, CD20, and HLA-DR antigens. Cytochemical studies by light microscopy revealed that the blasts were negative for myeloperoxidase, PAS staining, and double esterase staining, supporting the diagnosis of ALL. However, an ultrastructural study demonstrated that some of the cytoplasmic granules were myeloperoxidase (MPO) positive. Our findings suggested that leukemic transformation in this case may have taken place at a stage ontogenetically close to the pluripotent stem cell. Furthermore, the present case indicates the existence of a new form of ALL is characterized by MPO-positive granules detectable by ultracytochemistry and lymphoid-associated surface markers.

A case of acute suppurative thyroiditis following a perithyroidal abscess, which was thought to have resulted from infection of a cervical thymic cyst, is reported herein. The patient was an 8 year old asthmatic Japanese boy who originally presented with tender swelling of the left anterior neck in July, 1986. Although pharyngography could not clearly demonstrate the pyriform sinus fistula, a hypoechoic area around the left lobe of the thyroid gland was noted on ultrasonography. Incisional drainage revealed Streptococcus milleri. A diagnosis of acute suppurative thyroiditis was established, and a cystic tubular mass was surgically resected from the left perithyroidal space some time later. Histopathological examination of the specimen revealed partly cystic thymic tissue along with parathyroid tissue. These observations suggested that acute suppurative thyroiditis in this case was caused by a perithyroidal abscess and that a perithyroidal abscess may also result from infection of a cervical thymic cyst.

Unilateral muscular thickening (ULT) was observed in the wall of the fetal veins on the placental surface beneath the amnion. The maternal facing wall of the fetal veins was thin, and protruded into the amniotic sac. The ratio of the thickness of the maternal facing to the fetal facing wall was 4:1 to 3:1 in the fetal veins. ULT was found to develop after 17 weeks of gestation and was not observed in the small veins less than 1.0 mm in diameter or in the veins located in the deep portion of the chorionic plate. The intimal thickening was not recognized and there was no lipid deposition in ULT. ULT was found in every one of 110 mature placentae and was assumed to be a normal histological finding.

An autopsy case of an elderly man with Takayasu's arteritis and atherosclerosis is presented. Ischemic signs and symptoms including anginal attack were aggravated with activity of arteritis despite administration of prednisolone throughout the entire course. The autopsy revealed severe atherosclerosis over the aorta and its branches, and arteritis in the fibrotic stage in localized area. Vessels of vital organs including heart and kidneys, did not show Takayasu's arteritis but were atherosclerotic. This case highlights the numerous problems encountered in the treatment of elderly patients with vasculitis such as Takayasu's arteritis.

Two cases of Japanese girls with congenital cutis laxa associated with cardiovascular abnormalities are described. Case 1: A 12-year-old girl has been under our observation from the age of 6 months. Cardioangiogram revealed dilatation of the ascending aorta, meandering of the descending aorta and the coronary arteries, coiling of the carotid and innominate (brachiocephalic) arteries, and hypoplasia of the pulmonary arteries. Case 2: A 2.8/12-year-old girl died after our follow-up from the age of 3 months. The cause of death was congestive heart failure secondary to peripheral stenosis of the pulmonary arteries. In both cases, skin biopsy revealed a decreased number of elastic fibers and an increased amount of acidic mucopolysaccharides. The same histological features were observed in the pulmonary arteries and other arteries as well. Electron microscopic findings were diffuse thinning of elastic fibers and reduced elastic content. The high blood level of elastase (167.8 micrograms/l) in case 1 may cast a light on the unknown etiology of the disease.

72歳7回経妊経産婦が淡血性帯下および下腹部重圧感を主訴として来院した. 術前反復細胞診では異型細胞は検出されなかったが, 内診所見およびCT-scan像などにより卵巣癌を疑い, 開腹手術を施行した. 術後の病理学的検索にて原発性卵管癌であることが判明した症例を経験したので, その病変部捺印細胞所見・組織所見・電顕所見を報告するとともに若干の考察を加えた.1) 従来よりいわれているごとく, 腺癌細胞の同定は可能であったが, 卵管癌の特徴的な細胞所見はみいだせなかった.2) 進行例にもかかわらず, 卵管癌の特徴所見といわれている樹枝状構築を示す乳頭状発育を認めたことから, 卵管癌を疑った. その連続切片より, 悪性との移行像ともいうべき所見と病変部が卵巣部よりも卵管部が広範囲であること, 卵管より卵巣への連続性が確認されたことなどにより, 原発性卵管癌と診断した.3) 卵管癌の超微形態学的特徴所見とされているultramicroscopic alveolar spaceをみいだした.4) 本症例のoriginとして, ciliaおよびsecretory granuleの存在をみいだし得なかったことから, peg cellないしはindifferent cellより発生したものと推察した.

The autopsy findings in a 29-year-old male with Werner's syndrome are presented, with a review of similar data on 17 autopsy cases reported in the literature. The pathologic findings in this new case were scleroderma-like skin atrophy, arteriosclerosis, and atrophy of the endocrine glands (including the genital organs). In the reported cases of Werner's syndrome, as in the present example, the authors were unable to identify any responsible organ or system. However, they suggest that connective tissue, widely distributed throughout the entire body, may play an important role. The possible abnormal metabolism of connective tissue in Werner's syndrome deserves further study.

In recent years, both a numerical and relative increase in the morbidity of cardiovascular and cerebral vascular diseases has been noted in Japanese diabetics. The trend of macroangiopathy in the period between 1953 and 1980 was studied in 210 autopsy cases (Group A: 1953-62, 27 cases, Group B: 1963-72, 43 cases and Group C: 1973-80, 140 cases). The incidence of myocardial infarction and cerebral infarction as a primary cause of death was 25. 9% in Group A, 23.3% in Group B and 37.9% in Group C. From the autopsy findings, the morbidity of cardiovascular disease was 14.8%, 30.2% and 34.3% respectively. In order to elucidate the clinical risk factors, the age at death, duration of diabetes at the time of death, and incidence of hypertension and serum cholesterol in the past history were studied. The percentages of those aged over 70 at death was 18.5%, 23.3% and 42.8% respectively, and those of the cases with durations of more than 10 years was 25.9%, 40.5% and 51.8% respectively. The prevalence of hypertension and hypercholesterolemia was almost identical in each group. From the above, the previous increase in the death-rate due to coronary heart disease and cerebrovascular disease in Japanese diabetics is thought to be attributable to the increasing number of elderly diabetics of long duration.

A characterisation of the biological behaviour of gastric carcinoma was attempted by relating the histological classification to the mean number of metastases for each case (metastatic index). The material was chosen from a consecutive series of 294 gastric cancer autopsies in which only conservative therapy had been given or in which the cancer was first diagnosed at autopsy. According to the extent of metastasis it was possible to divide the nine histological types into two groups of limited invasiveness and generalised invasiveness. Unfavourable biological behaviour of the cancer was determined by the presence of the latter group of histological types in the primary site even in cases where this histological type formed the minor component of the tumour. The variation in histology within the primary tumour and between the primary site and the metastatic sites was related both to histological affinities and to the metastatic index associated with the primary site histology. It is suggested that this dual classification system adds significantly to our understanding of the natural history of gastric cancer.

肝臓および腸間膜相互の循環は, 門脈を介して, 相互に影響を受けるが, 両臓器はそれぞれ独特の血行動態を有するため, 梗塞発生はまれである. 著者らは, 84歳の心疾患負荷をもつ老女性に, 肝梗塞および空腸の非閉塞性梗塞による出血性壊死性腸症の合併を経験した.剖検: 肝両葉には, それぞれ大きさ3～4cmの新鮮凝固壊死巣を2ケずつ認めた. 細胞反応には乏しく, 新鮮な貧血性梗塞の像を示していた. 肝動脈右葉枝本幹, 左葉枝分枝に血栓塞栓と思われる血栓を認めた. 他部の肝には高度の小葉中心性うっ血を認めた.空腸上部には長さ5～6cmの出血, 壊死を認めたが, 細胞反応には乏しかった. 腸間膜動静脈は硬化性ではあったが, 血栓は認められなかった.心臓は500gで左室肥大を呈し, 両心房, 心室は拡張性で, 僧帽弁には石灰化を伴う線維性心内膜炎を認めた.その他, 腎梗塞, 新鮮消化性胃潰瘍などを認めた.これら剖検所見から, 心疾患を基盤とする大循環系障害のみならず, 心不全による肝うっ血が, 一方では, 肝梗塞, 他方では腸管梗塞を発生せしめたと推察され, 本例にみられた肝梗塞および末期性出血性壊死性腸症は, 門脈を介した局所循環障害が加味されて, 発生したものと考えられた.

PSSの腎病変で,ネフローゼ症候群を呈した症例は少ない.また強皮症状を欠くPSS sine sclerodermaの報告例もまれである.多発性関節炎およびレイノー現象で発症し,強皮症状を示さず, 9年の経過後に,イレウス手術時の生検でPSS sine sclerodermaと診断され,その後ネフローゼ症候群を呈し,尿細管acidosisを疑つた1剖検例を報告した.症例はPSSとして治療中であつた32才の女性で, 3カ月前から高度の蛋白尿となり,ネフローゼ症候群を呈して入院したが,高窒素血症は明らかでなかつた.腎性糖尿,低カリウム血症,高クロール血症,代謝性acidosisが認められ,約2カ月後に細菌性肺炎を併発し死亡した.剖検では,全身諸臓器の線維化と筋層の萎縮が明らかで, PSSが確認され,また膜性増殖性糸球体腎炎が認められた.本例の腎病変とPSSとの関連につき考察を加えた.

Prognosis of well-differentiated carcinoma of the thyroid gland is generally favorable, while that of anaplastic carcinoma, extremely poor. Well-differentiated carcinoma may sometimes be fatal; the most common underlying cause is considered to be due to anaplastic transformation of the original well-differentiated carcinoma to a less differentiated form. We studied 27 consecutive autopsy cases of fatal thyroid cancer treated at the Ito Hospital, Tokyo, during a five-year period, 1969-1973. We found uniform histological features of anaplastic carcinoma in 10 cases and of well differentiated carcinoma in four cases. In addition, co-existence of well-differentiated and anaplastic carcinomas was observed in nine cases and well differentiated and squamous cell carcinomas in four. Circumstantial evidence strongly suggests that malignant transformation is a part of the natural history of thyroid carcinoma, from well-differentiated carcinoma to less differentiated forms, either squamous cell or anaplastic carcinoma.

A case of hepatic infarction associated with terminal hemorrhagic necrotizing enteropathy (THNE) in an 84-year-old woman with cardiac failure is presented. The autopsy showed hepatic infarction, THNE, fibrous endocarditis, generalized arteriosclerosis, myocardial hypertrophy, liver congestion, and other findings as described. Both rare infarctions (hepatic and intestinal) seemed to have originated not only as a result of impaired systemic circulation due to cardiac failure, but also from liver congestion due to peripheral circulatory deficiency through the portal vein. The circulatory correlation between these infarcts of the liver and intestine is discussed.

HE staining, Victoria blue staining and Factor VIII staining were performed and compared in order to precisely check the degree of blood vessel invasion of follicular cacinoma of the thyroid gland.Forty-eight surgical treated cases were reviewed, covering a ten-year span, taken from specimens stored at the First Department of Surgery of Yokohama City University School of Medicine and Ito Hospital. In order to decide what comprises blood vessel invasion, criteria of a cluster of tumor cells present inside vessels and preservation of all the blood vessel endothelium were chosen.When Factor VIII staining was performed, blood vessel invasion was found in 31 of 48 cases (64.6%). With Victoria blue and HE staining, the corresponding figures were 20.8% and 8.3%, respectively neither of which reached the level found with Factor VIII staining.When the relationship between blood vessel invasion and tumor diameter was investigated, in t1 and t2 cases, blood vessel invasion amounted to only slightly less than 50%, whereas in t3 and t4 cases there was more than 80% invasion, the difference being statistically significant. Moreover, there was a significantly higher average age level for patients with blood vessel invasion as compared with those without invasion.

It is not unusual for normal-looking thyroid tissues to be found within cervical lymph nodes when thoroughly examined histologically following neck dissection. This has long been called lateral aberrant thyroid, which is now regarded as metastasis of thyroid carcinoma. On occasions, however, some exceptional cases are encountered which are difficult to interpret as metastatic foci. The present case is a 14-year-old girl who presented with goiter in the left thyroid lobe and ipsilateral lymph node swelling. With the diagnosis of thyroid carcinoma (JT2N1M0), left thyroid lobectomy was carried out along with left modified radical neck dissection. Postoperative histological examination revealed that the primary lesion was adenomatous goiter without any malignant findings. Thyroid tissue within the cervical lymph nodes also showed the same findings as the main lesion. After a thorough histological examination, only one benign follicle was noted in the small lymphatic vessels next to the primary lesion. From these findings it is suggested that this was a case of benign metastasizing adenomatous goiter.

This study was designed to identify 11 different antigens including calcitonin (CT), calcitonin gene-related peptide (CGRP), gastrin-releasing peptide (GRP), and carcinoembryonic antigen (CEA), in the tumors of 36 patients with medullary thyroid carcinoma (MTC) using immunoperoxidase staining techniques. In addition, clinical features of MTC patients were compared with the immunohistochemical findings to establish factors influencing prognosis. MTC was found to contain various products in many patients and CT and CEA were positive in all patients. CGRP and GRP showed positive staining in 96.6% and 82.9% of MTC patients, respectively, suggesting that CGRP and GRP are novel tumor markers for MTC. In tumor cells, CT, CGRP, and GRP were often revealed in identical cells. Familial patients showed more multiple substances than sporadic patients. In the 2 inoperable patients with extremely aggressive progression, tumors showed undifferentiated histology and poor staining for peptide hormones, suggesting that specific qualities such as neuroendocrine tumor had been lost. These 2 patients particularly revealed an inverse relationship between CT and CEA distribution such that small amounts of CT were present in cells which have homogenous staining for CEA. This study suggests that CGRP and GRP, in addition to CT and CEA, may be a histologically potential tumor marker for MTC. CT and CEA may be possible markers for differentiating patients with high malignancy from those with ordinary malignancy.

We reported three cases of Graves' disease which showed unusual histological findings featuring solid follicles, multinucleated giant cells and diffuse infiltration of histiocytes as well as lymphocytes in the whole section of the resected thyroid. Characteristics of these three cases were as follows: (1) Clinically, longer duration of the disease and exophthalmos were their prevailing findings. (2) On laboratory data, antimicrosomal antibody showed extremely high titers with 100,000 to 400,000. (3) Their operative findings were different from ordinary Graves' goiters in that colors of the goiter were yellow-red or gray-red, surface was rough and coarse, consistency was firm, and adhesions with the adjacent connective tissue were noted. (4) Postoperative clinical outcome was quite similar to that of ordinary Graves' patients. From these findings, these three cases were considered to be different from either so-called Hashitoxicosis or silent thyroiditis or Graves' disease with granulomatous foci, and it was suggested that these three cases might be a subgroup of Graves' disease or another hyperthyroidism than ordinary Graves' disease. Further accumulation and analysis of such cases will be necessary in order to answer this question.

Urinary calculi found in 4 patients on chronic hemodialysis or continuous ambulatory peritoneal dialysis (CAPD) were identified as protein calculi by infrared spectroscopic analysis. Positive Congo red staining and immunological assessment revealed that the calculi were composed of amyloid protein derived from beta 2-microglobulin. A comparison of the patients who excreted calculi with 10 patients on chronic dialysis without urinary calculi showed no significant differences in the urinary and serum levels of beta 2-microglobulin. The mechanism of amyloid calculus formation may involve factors independent of the concentration of beta 2-microglobulin in urine or serum. Urinary calculi found in patients on chronic hemodialysis or CAPD were composed of amyloid protein derived from beta 2-microglobulin.

常識的に考えて長寿は遺伝と環境との相互作用によって得られるものと考えられる. 本論文は長寿の達成に遺伝子が関与しているか否かを解明することを目的とし, ことに遺伝に関する一連の研究のうち家族歴についての case controlled study を行ったものである. 現在まで長寿の遺伝に関する論文は数多くあるが, それらは単に事実について発表しているものであって, 長寿は長寿家系より生ずることを積極的に証明しているものは, 1974年に Abbott が発表した論文が唯一のものである. しかし彼の論文では, 対照群の選択にバイアス因子が混入していると考えられる. 我々は厳重な条件を設定した. 即ち, 百寿者と同一部落に生れ育ち, 死亡した人で, 同胞の末子, または末より2番目であり, 統計処理上自殺, 他殺, 原因不明死, 事故死, 戦争関係死を除く病死者の同胞が3名以上いることを必要とした. また彼らは調査時点の沖繩の平均寿命より年長であり, さらに彼らの長兄ないし長姉が調査時点で生きていれば100歳から105歳である人とした. さらに彼らから充分満足できる解答がえられることが必要条件であった. 以上の結果, 対照として選ばれた者は65歳以上の同部落老人2,270人中11人であった. これらの両家系について寿命に関する各種の指標を算出して, 父親群・母親群・男同胞・女同胞・全同胞・全家族の5群別に比較検討した.推計学的に有意差を示したものは平均寿命では全家系群, 80↑/65↓と90↑/65↓では女同胞と全家族群, 80歳達成率では全同胞と全家族群, 90歳達成率では女同胞・全同胞と全家族群, 65歳以下若年死の平均死亡年齢では母親群・男同胞・全同胞・全家族群, R90では女同胞・全同胞と全家族群であった. これらの結果から長寿への遺伝の関与が推察された. それらは主として女性を介するように考えられたが男性を介するものも認められた.

A case of marked chronic mature eosinophilia associated with multiple thrombosis in 30-year-old male is presented. Hitherto this sort of the disease has been categorized as DECD. Reviewing the autopsied cases of DECD, including our own case, DECD should not be categorized as a collagen disease because of the absent connective tissue degeneration and absent connective tissue degeneration and absent serological abnormalities. We rather noticed a certain relation between marked chronic mature eosinophilia and thrombus formation. Therefore, we propose here the term hypereosinophilic multiple thrombosis to express the pathomorphogenesis more precisely.

The results of treatment were analyzed in relation to serum microsomal antibody (MCAb) titre before treatment in 1185 patients with Graves' disease. The percentage of patients who had ablative therapy because of poor response to antithyroid drug treatment was significantly greater in those with MCAb haemagglutination test (MCHA) titres greater than 1:25 000. With 131I treatment, the patients with MCHA titres greater than 1:6400 responded significantly less to therapy, although the analysis was done in 146 selected patients with certain defined radiation doses and small goitres. With surgical treatment, the percentage of the patients entering into remission was significantly smaller for patients with MCHA titres greater than 1:25 000, because of an increase in both hypothyroidism and relapses. The incidence of hypothyroidism was significantly higher in patients with marked lymphocyte infiltration and/or lymphoid follicles. The degree of these histological findings in Graves' disease was not marked in spite of high MCAb titre and it was significantly different from that in Hashimoto's disease when analyzed in relation to the MCHA titre. These data indicate that in Graves' patients with high MCAb titre, remission is difficult to obtain by treatment, and suggest that the significance of MCAb is different in Graves' disease and Hashimoto's disease. The titre in Graves' disease may be one expression of the activity of this disease.

老化過程研究の好個のモデルと考えられる Werner 症候群の1剖検例を報告した. 患者は男性で, 思春期前後より漸次, 嗄声, 足底部皮膚硬化, 両側若年性白内障を来たし, 高血圧にて入院. 既往歴も勘案し本症の診断を受けて加療中, 29歳時に心, 腎不全にて死亡. 家族歴で, 両親はいとこ同士の結婚であるが, 同胞には本症を疑わせる者はいなかった. 検査所見では, 心, 腎不全を思わせる以外に, 内分泌機能も含め, 特記すべき事はなかった.剖検所見は外見上, 禿頭, 白髪, 顔面皮膚の萎縮のため, 年齢よりはるかに老けてみえた. 下腹, 下肢には, 色素沈着を伴う皮膚萎縮, 足底部皮膚硬化, 左眼義眼装着, 右眼白内障術後状態, 腋毛, 恥毛脱落, 精巣および陰茎萎縮を認めた. 心血管系は心肥大 (450g), 年齢に比して高度の大動脈粥状硬化症, 全身の高度の動脈および細小動脈硬化症をみた. ことに, 脳, 膵, 前立腺では, pseudocalcification および Monckeberg 型動脈硬化症を呈した. 腎は高度の細小動脈硬化性萎縮腎 (左84, 右77g) の他, 悪性腎硬化症を思わせる所見もみた. その他, 内分泌臓器の萎縮 (下垂体0.5g. 甲状腺8g, 副腎左右各6g, 膵52g, 精巣左8, 右9g), 前立腺萎縮をみた. は, 骨梁の細小化, 声帯の萎縮, 胸腺退縮を認めた. なお, 悪性腫瘍の合併はなかった.既剖検報告を通覧すると, 全般的に強皮症様皮膚萎縮, 動脈硬化症, 内分泌・生殖器臓器の萎縮が認められ, 本例でも同様の変化をみた. 特に本例の細小動脈硬化症は特徴的であった. また, 既報告中, 甲状腺腺腫および類縁腫瘍の高頻度な事も指摘したい. 本症は, 特定臓器の欠陥に起因すると考えられる組織学的ならびに諸種検査上の所見に乏しく, 形態学的な変化は, 本症の本態を示すものではなく, 2次的表現と考えられる. 特に, 眼, 皮膚, 血管, 骨, 声帯, 内臓諸臓器とともに, 常に存在する結合織に着目したい. 就中, 従来より知られる血清, 尿中のムコ多糖, 培養線維芽細胞の態度などより, subcellular な障害が考えられ, 結合織代謝と老化という点から, なお追求されるべきであろう.

A morphological and biochemical study of granulation tissue in wound healing was reported, using the circular excised wound of the dorsal skin of male adult mice.Argyrophilic fibrils appeared first on the 6th day after the excision. After the 8th and subsequent days, prominent fibrogenesis was marked. Ribonucleic acid was most intensely demonstrated on the 8th and the 10th day. Alkaline phosphatase was observed only in the fibrotic area after the 8th day.Neutral salt soluble collagen may not integrate in the newly deposited argyrophilic collagen fibrils in the 8th day granulation tissue. The neutral salt soluble collagen would exist in a dispersed or submicroscopic fibrillar state in the granulation tissue.Acid soluble collagen might represent the bulk of newly deposited col-lagen fibrils.PAS positivity of collagen fibers gradually decreased with maturation of fibers.Biochemical study revealed that the neutral salt soluble collagen already appeared on the 6th day in considerable amounts. All collagen fractions gradually increased thereafter, and remarkable accumulation of insoluble collagen was recognized on the 10th and the 12th day. The ratio of neutral salt soluble and insoluble collagen to the total collagen changed inversely. Acid soluble collagen showed no remarkable change.The content of desoxyribo- and ribonucleic acid increased up to the 8th day and then gradually decreased in the granulation tissue.It has been shown that the incorporation of radioactive phosphorus into the nucleic acids made two peaks. The first was noted on the 4th day, when the leucocytic infiltration was prominent, and the second was on the 8th and the 10th day, when the active proliferation of fibroblasts and synthesis of collagen precursor were remarkable.The possible significance of these results is discussed.

In order to study the pathogenesis of dissecting aortic aneurysm, an attempt was made to demonstrate aortic medial vasa vasorum by infusion of India ink into 25 weanling rats that had been administered β-aminopropionitrile fumarate for as long as 30 days. More than half of the rats developed gross or microscopic dissecting aneurysms of the aorta, with the frequent occurrence of hemothorax due to rupture. India ink was present in the dissected lumen of the aortic wall but not within intact portions of the media. Capillary networks containing India ink were noted in periadventitial and adventitial layers and in periadventitial granulation tissue covering dissection, but were not found within the media. Infusion of India ink in 18 normal rats showed abundant capillary networks in periadventitial and adventitial layers but failed to reveal capillary vascular structure within the media of aorta. The results of this study indicate that in experimental dissecting aortic aneurysm in rats induced by β-aminopropionitrile fumarate the vasa vasorum do not play a role in its pathogenesis.

Transport of fatty acids was investigated in human aorta, aiming to analyze the esterification of cholesterol and the structure of glycerides. PC was found to be a source of supplying fatty acids to cholesterol through the participation of phospholipase. LCAT seemed to be less active in this aspect. Structure of TG was different especially at 1 and 3 position in between plasma and atheromatous plaque. This could indicate that deposited TG was modified either by transferring FFA in various position or by the biosynthesis in situ.

Two autopsy cases of granulomatous aortitis associated with necrotizing angiitis and glomerulonephritis were reported. Case 1 was a 31 year-old female and a diagnosis of typical Takayasu's arteritis was made. Case 2 was a 64-year-old female. In the central portion of the aotlc media, granulomatous inflammation surrounding coagulation necrosis was observed. This aortitis is different from classical Takayasu's arteritis and is diagnosed as “unclassified” aortitis. The significance and inter-relationship of these lesions were discussed. Clinical and anatomical features of these two cases suggest some common background of Immune abnormalities.

A case of annulo-aortic ectasia was reported. The patient was a 44 year old Japanese female who expired shortly after the operative intervention against aortic aneurysm and aortic insufficiency. This is the first case of annulo-aortic ectasia reported in Japan. Histological findings of the resected aneurysm revealed pronounced cystic medial necrosis. At autopsy, similar medial necrosis was found in the remaining aorta, pulmonary artery and some of the major branches of the aorta. The aortic valve cusps also showed myxomatous accumulation of acid mucopolysaccharides and hypoplasia of annular connective tissue. The aortic ring was dilated with evidence of regurgitation. Problems related to pathologic findings were discussed with special reference to a forme fruste manifestation of Marfan's syndrome. We believe cases, such as presented here, should not be considered as a forme fruste of Marfan's syndrome with the present knowledge of the disease, in spite of the morphologic similarity of lesions in our case with those described in classical Marfan's syndrome.

Familial occurrence of differentiated, nonmedullary thyroid carcinoma in 23 patients from 11 families is reported. Five patients were male and 18 were female. The familial relationship of patients was “parent and child” in 12 cases from 6 families, and “siblings” in 11 cases from 5 families. Carcinoma of other organs was noted in other members in 8 families. Histological examination revealed 18 papillary, 2 follicular, and 2 anaplastic carcinomas (the 2 anaplastic carcinomas were considered to be transformed from preexisting differentiated carcinoma). In 1 case, the histological type was unknown. The average diameter of the primary lesion was 29.9 mm. Cervical lymph node metastasis was found in 77.8% and local recurrence in 28.6% of the patients. Solid and invasive growth was dominant. On HLA typing, phenotypes of B7 and DR1 were significantly redominant in familial patients compared with nonfamilial patients and normal Japanese. Moreover, the haplotype of B7-Cw7-DR1 was observed in 5 of 13 patients tested. It is suggested from these observations that some types of differentiated, nonmedullary thyroid carcinoma may show familial occurrence and that they may have common factors with regard to the genetic and immunologic basis of the disease.

Three cases of primary band-shaped spheroidal degeneration of the cornea from two consanguineous families are reported. In all cases vision deteriorated slowly, the deterioration beginning in childhood. Photophobia in childhood was also a common symptom, and two patients suffered from intermittent painful sensations. The occupational and environmental histories had no bearing on the cases. Histochemical study of the yellow, oily appearing deposits suggested that this material has a complex composition, with staining characteristics of both protein and acid-fast lipid.

The most important disease and the most contributory cause of death were pathologically surveyed among 5106 subjects aged 80 years old and over. Bronchopneumonia, encephalomalacia, myocardial infarction, gastric cancer, generalized arteriosclerosis and myocardial hypertrophy were the most prevalent diseases in order of frequency. With advancing age, bronchopneumonia and cerebral and cardiac arterisclerotic diseases increased, while cancer decreased. This death pattern of the extreme aged has developed during the last 25 years in Japan, and is very similar to the present situation in some European countries.