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Cartilage cells of human trachea contain a large quantity of fat droplets, which become smaller and less regular with ageing. Yellowishbrown pigmentation develops with ageing, especially to a marked extent after the age of 50. Histochemically, this pigmentation is compatible to a ceroid-like substance, which electron microscopically consists of alternate lamellar arrangement of layers with high and low electron density, altogether forming a fingerprint-like or lattice-like pattern. This substance is present surrounding fragmented, tiny fat granules. The ultramicroscopic crystalloid structure of the substance may be of great significance in pigmentation of cartilage. Although the ceroid-like substance detected in this study may be classified histochemically into the category of ceroid, it is reasonable to designate it as “paraceroid”, since it is unstable in structure.

In order to clarify the present state of opportunistic fungal infections increasing in incidence in autopsy cases, all autopsy cases from 1966 to 1975 reported in the Annual of Pathological Autopsy Cases in Japan were reviewed. Of the total 233,130 autopsy cases, mycoses were present in 4,340 (1.86%). The incidence of mycoses has strikingly increased during the recent five-year period. In Japan, the mycoses most frequently occurring in autopsy cases were candidiasis (32.28%), aspergillosis (23.08%), cryptococcosis (9.63%), and mucormycosis (2.90%). These occurred more frequently in younger persons and were most commonly secondary and deep-seated infections (95.78%). Among the primary diseases associated with mycoses, aplastic anemia (14.36%), leukemia (9.89%), malignant lymphoma (5.73%), multiple myeloma (4.68%), and systemic lupus erythematosus (4.62%) were most frequent. The incidence of the primary diseases associated with mycoses is increasing extraordinarily, and this seems to be strongly related to the modern therapy of using high doses of anticancer or immunosuppressive agents.

Summary The lesions of this disease are seen mainly in the trunks of larger and medium sized arteries where focal coagulation necrosis and granuloma consisting of epithelioid cells and giant cells of Langhans type occur primarily, and these focal changes extend to the surrounding area forming irregular scar tissues and in turn cause a diffuse productive inflammation of the arterial walls. Such lesions seem to begin centering at the vasa vasorum in adventitia and media, and changes in the intima or formation of thrombi are interpreted to be secondary. It is important to note that marked thickening of the arterial wall in some portions is found immediately after branching off, while no changes are visible proximal to the branching. From these findings it is proposed that the preferable designation for this disease is Trunco-arteritis Diffusa (or Granulomatosa) Productiva. In this case, marked stenosis due to thickening of the wall of the left subclavian artery causes an absence of radial pulsation of this side, that of the renal arteries brings about degenerative changes in both kidneys, and marked stenosis of the short gastric arteries causes an ischemic necrosis of the stomach wall, through which perforations occur. Atypical tubercle bacilli of viral type are suspected as the etiologic factor of this disease. In has been said that this disease occurs most frequently among women in puberty, and therefore, this is the first autopsy report of a boy in the first decade such as 5 years of age.

The second autopsy case of microlithiasis alveolaris pulmonum in our country was discussed. This case had no familial back-ground or genetic predisposition for the disease and followed an almost asymptomatic course for 13 years since abnormal shadow of chest was revealed radio-logically. Of interest in this case in the deposition observed microscopically of the same substance in alveolar walls or in the interstitium as the ground substance of intra-alveolar microlith, which was probably responsible for the irregular elastosis.

VII Summary . 1. Degeneration products of so-called basophilic (mucoid) degeneration show the properties of glycogen or glycogen-protein complex in the initial stage, followed by formation of neutral and acid mucopolysaccharides. Judging from the steps taken in the occurrence of degeneration and from the histochemical characteristics, it is designated as mucopolysaccharide (MPS) degeneration. 2. Having discovered a new type of degeneration that forms crystalloid bodies, what is considered to be in the absorption stage of MPS degeneration, it was named as crystalloid degeneration. Histochemically in this degeneration characteristics of neutral mucopolysaccharide are demonstrated more frequently, but in a later stage some also reveal properties of hyaline protein or lipoprotein. 3. MPS degeneration and crystalloid degeneration set in from abnormal metabolism of sugars, especially of glycogen, and as the products resulting from misguided synthesis caused by disturbed enzyme systems there are formed various complexes of substances that chemically represent residual radicals of sugars, and of proteins. Therefore, it may be appropriate to designate these degeneration types taken together as glycoprotein (GP) degeneration. 4. Products of GP degeneration of heart muscles are not direct deposits of substances that infiltrated into the muscle fiber from outside but they are endogenous autochthonous metabolites. Moreover, this degeneration is closely associated with aging phenomena, and it develops, as a rule, not from specific nature of any particular disease but rather it sets in proportionately to underlying wasting conditions of the body. 5. Mentions were made on 3 cases of myoclonic epilepsy and on one case each of myeloma and X-ray irradiated cancer of esophagus, as examples that developed markedly multiple GP degeneration in heart muscle as to present a classical state of myodegeneratio cordis. 6. GP degeneration develops not only in heart muscle but also in skeletal muscle and smooth muscle. In skeletal muscle it occurs at the time when the muscle is injured and also in myogenous cells during subsequent degeneration phase in the “abortive regeneration”. 7. There could be recognized intermediary forms between crystalloid degeneration substance to which lipoid was adsorbed and coarse lipofuscin clusters, suggesting that the formation of lipofuscin has a close relationship with GP degeneration. 8. GP degeneration of heart muscle was experimentally induced in rabbits. Namely, by injection of desoxycorticosterone acetate (DOCA) GP degeneration was developed approximately parallel to the degree of dysprotei'nemia in 8 of 11 animals. However, since the degeneration occurs even by repeated heart punctures, dysproteinemia is not an indispensable factor in the development of this degeneration. GP degeneration was observed in 6 of the 8 rabbits in experimental starvation and in 14 of the 26 rabbits exposed to a large dose of X-ray irradiation. However, GP degeneration did not develop in the animals with experimental hypo- and hyperthyroidism. 9. In the experimental GP degeneration induced acutely within a short period of time, MPS-type degeneration is found more frequently but formation of acid mucopolysaccharide is less than that in the human heart muscle. In addition, experimental GP degeneration induced chronically over a long period of time tends to be mostly of crystalloid-type degeneration. 10. From these results it can be said that glycoprotein degeneration of muscle fibers represents energetic-chemical insufficiency of the muscle function.

This report presents the first autopsy case in Japan of idiopathic retroperitoneal fibrosis which had developed in a 71-year-old man. The clinical course was of extremely long duration of more than 10 years. Malignancy was suspected on biopsy, and at one stage chylous ascites developed. The cause of this retroperitoneal fibrosis could not be determined, but it is considered that the vicious circle of inflammatory processes such as cellulitis and lymphangitis in the retroperitoneum and lymphedema as well as lymphorrhage attributable to mechanical lymphatic obstruction brought about the retroperitoneal fibrosis.

Chronic granulomatous disease (CGD) is based on the dysfunction of phagocytes and characterized by a comparatively uniform granulomatous lesion caused by organisms which do not produce hydrogen peroxide and which are catalase-positive. This report describes two autopsy cases in children, a nine year-two month-old boy and a ten month-old girl, with the clinical manifestations and autopsy findings of CGD and a brief review of all autopsy cases consistent with CGD reported in Japan. In these cases, in addition to the usual CGD lesions, there was a markedly different type of granuloma due to Aspergillus sp., which consisted of multinuclear giant cells alone, or a caseous center surrounded by giant cells. This peculiar type of aspergillosis may correspond to a primary infection, described as pseudotuberculosis aspergillina. It appears that in some CGD patients, macrophages may function normally and sometimes be more activated by these infections than in the normal body. The histochemical and electron microscopic examination of the present cases and a review of the previous cases suggest that the yellowish brown pigment found seems to resemble a ceroid. It is a product of the degradation of leukocytes and tissue elements with subsequent accumulation in phagocytic histiocytes and is not necessarily peculiar to CGD.