摘要:

Neuropathological and enzyme‐histochemical studies were performed on brindled mouse hemizygotes (BMs) and normal littermates at the age of 2 days, 7 days, 11 days and 14 days, together with an investigation of their tissue cop per levels. A greatly increased copper concentration was confirmed in the kidney and intestine and a greatly reduced concentration in the liver and brain of BMs. The copper concentration in the brain increased gradually with age in the normal littermates, whereas this did not occur in BMs. There was no significant difference in the tissue copper concentration between the cerebrum and the cerebellum brainstem in BMs or in normal littermates. Light and electron microscopy of the BM brain revealed progressive neuronal degeneration in association with increased mito‐chondrial changes (ballooning and crista disintegration). Enzyme histochemical examinations demonstrated a progressive comparative decrease (i.e., an increased difference from normal) of cytochrome oxidase activity in the BM brain. These data suggest that progressive degeneration of the brain in Menkes’disease is attributable to mitochon‐drial degeneration caused by a comparative decrease of both copper concentration and cytochrome oxidase activity in th

ISSN:1320-5463    

DOI:10.1111/j.1440-1827.1990.tb01577.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Lethally irradiated SJL/J mice were reconstituted with B10 bone marrow cells, and the process of thymic reconstitution by donor derived cells positive for I‐ A or Vβ8 molecules was investigated. The donor‐derived la+cells appeared in the medulla on day 7 after reconstitution. The la+ cells became confluent up to day 14, and the cellularity in the medulla on day 17 was almost the same as that in the normal thymus. Dull Vβ8+thymocytes were first recognized in the cortex on day 10 and were identifiable in the medulla by day 14. The Vβ8+cells seemed to be mainly CD4+8+double‐positive. Furthermore, most of the Vg8’cells in the medulla of chimeras given cyclosporin A for 3 weeks after reconstitution appeared to be CD4+8+. The present findings demonstrate that CD4 8+ thymocytes which bear a low concentration of TCR exist in the thymic medulla at a relatively early stage when donor‐derived la+cells have already settled there. The coincidental appearance and coexistence of la+cells and TCR+thymocytes in the medulla suggest that these histological characteristics may be related to the selection of thymocytes

ISSN:1320-5463    

DOI:10.1111/j.1440-1827.1990.tb01578.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

The development and regression of right ventricular hypertrophy was investigated in 12 pigs with special reference to changes in ventricular function and myocardial fiber orientation. Nine ventricles were pressure ‐loaded by banding the pulmonary artery for 28–81 days, and four of them were then released from the load by removing the band. Right ventricular systolic pressure (RVSP), end‐diastolic pressure (RVEDP) and end systolic volume index (ESVI) increased significantly during banding and decreased after debanding. End diastolic volume index (EDVI) and stroke volume index (SVI) showed no significant change during banding and after debanding. The weight of the right ventricle relative to both ventricles (RV/TV) and the thickness of muscle fibers were increased significantly in the loaded ventricles, and reduced again to the control level in ventricles released from the load. The intramyocardial distribution of angles (θ) of inclination of muscle fibers from the transverse plane of the outflow tract was estimated histometrically. There was a significantly larger proportion of circularly oriented fibers (|θ|≦30) in the pressure loaded ventricles than in the control, whereas these fibers decreased again to the control level after removal of the pressure load. The present findings indicates that 1) the right ventricular hypertrophy induced by pressure loading is characterized not only by an increase in ventricular weight and muscle fiber thickness, but also by a change in intramyocardial fiber orientation, and 2) the hypertrophic right ventricle can regress both functionally and morphologically to a normal state after removal of the pres

ISSN:1320-5463    

DOI:10.1111/j.1440-1827.1990.tb01579.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

We studied the histologic and histometric features of the aortic media in cases of dissecting aneurysm in order to clarify the pathologic background of this condition. The results were then compared with cases of true aneurysm and control specimens of “normal” aging aorta. Most cases of dissecting aneurysm and true aneurysm, as well as a number of control specimens, showed severe cystic medial necrosis, and therefore this feature does not appear to be specific to dissecting aneurysm. Fibrosis was noted in the process of repair of medionecrosis in dissecting aneurysm, true aneurysm, and controls. The main pathologic features of the aortic media in dissecting aneurysm included a higher grade of elastin fragmentation (offensive factor) and less fibrosis (defensive factor). Our findings indicate that the pathologic balance between these offensive and defensive factors is an important consideration when evaluating the pathogenesis of dissecting aneur

ISSN:1320-5463    

DOI:10.1111/j.1440-1827.1990.tb01580.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

A pathological study was conducted on four patients with nephroblastoma associated with aniridia. The age at diagnosis was one year in three cases and 4 years in one case. Chromosomal analysis was performed in three cases, and showed 11p13 deletion in all. Each nephroblastoma consisted of metanephric blastemal, epithelial, and mesenchymal cells. All the tumors were centrilobar, and in one case an additional polypoid tumor grew into the renal pelvis. Histological examination showed conspicuous heterotopic cells, such as striated muscle and adipose tissue. Striated muscle was seen in all four cases, while both adipose tissue and smooth muscle were seen in two cases. Squamous epithelium was present in two cases. A literature survey revealed a high incidence of bilateral occurrence in cases of nephroblastoma associated with aniridia. lntralobar nephroblastomatosis was observed in all four cases, and it is proposed that this is the precursor lesion of nephroblastoma associated with aniridia.

ISSN:1320-5463    

DOI:10.1111/j.1440-1827.1990.tb01581.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

A case of type I Gaucher's disease in a 39 year old male is reported. Autopsy showed marked enlargement of the spleen (3,070g) and infiltration of typical Gaucher's cells in the spleen, liver, bone marrow, gastrointestinal tract, lymph nodes and adrenal glands. The diagnosis of Gaucher's disease was ascertained by the very low β‐glucosidase activity of cultured subcutaneous fibroblasts and the high content of glucocerebroside in the spleen tissue. A peculiar finding in this case was prominent deposition of brown pigment in endothelial cells of the spleen and smooth muscle fibers of the gastrointestinal tract, urinary bladder and prostate. Histochemical examination revealed that the granules in endothelial cells and smooth muscle fibers were ceroid. Such deposition of ceroid has never been reported previously in Gaucher's disease. Ceroid deposition in generalized smooth muscle fibers is known as brown bowel syndrome, and is highly suggestive of severe vitamin E deficiency. Although other symptoms of vitamin E deficiency were not noticed in this case, some malnutritional condition might play a role in prominent deposition of ceroid in lysosomes, possibly together with deficient activity of a Jysosomal enzy

ISSN:1320-5463    

DOI:10.1111/j.1440-1827.1990.tb01582.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

We report a rare case of apocrine neoplasm with malignant potential. The patient, a 29 year‐old man, had a nodule 1 cm in diameter on his left upper eyelid which had been growing slowly for several years. It was a cystic lesion, consisting of neoplastic cells of probable apocrine gland or Moll's gland origin. This opinion was based on the histological characteristics, which included eosinophilic cytoplasm accompanied with decapitation secretion, iron granules, and granular depositions which were stained positively with periodic acid‐Schiff, with and without diastase digestion. Ferritin was found in their cytoplasm, a feature that has not been reported. It was uncertain whether the neoplasm was benign or malignant, because the cells showed nuclear atypia, characterized by variation in size and hyper‐chromasia, but lacked the histological features of malignancy, including infiltration into the adjacent tissue and mi

ISSN:1320-5463    

DOI:10.1111/j.1440-1827.1990.tb01583.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Subdermal metastatic nodules in a 62 year old male patient with esophageal carcinoma contained both car‐cinomatous and chondroid areas. The carcinomatous areas showed the histology of poorly differentiated squamous cell carcinoma, and light microscopically an apparent transition could be traced from carcinomatous cells to chondroid cells. In the chondroid cells, the characteristics of chondrocytes were demonstrated by light microscopic, electron microscopic, histochemical and immunohistochemical studies, although nuclear atypism was evident, suggesting their malignancy. Furthermore, immunohistochemical studies showed that some chondroid cells contained both keratin proteins and squamous cell carcinoma antigen, which were also found in the carcinomatous cells. These findings together with the light microscopic observations suggest that chondroid cells are derived from squamous cell carcinoma cell

ISSN:1320-5463    

DOI:10.1111/j.1440-1827.1990.tb01584.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

An autopsy case of chronic pancreatitis associated with unusual chronic thyroiditis in a 54 year‐old woman is presented. Microscopically, the pancreas was densely infiltrated by lymphocytes and its exocrine parenchyma was completely replaced by sclerotic tissue. The thyroid gland was also infiltrated by lymphocytes, but no lymphoid follicles were observed. These morphological changes are rare findings with respect to the severity of inflammation and the association of the affected organs. Further findings suggested involvement of an autoimmune mechanism in the pathogenesis of these lesions. Using the avidin‐biotin conjugate technique and antibodies (Abs) against T lymphocyte and HLA‐DR antigens (Ags), im‐munological aspects of the lesions were studied. Most of these infiltrating lymphocytes were revealed to be T lymphocytes, and HLA DR Ags were observed on the epithelial cells of the pancreatic ducts and thyroid follicles. As a control, 45 surgical specimens of pancreas and thyroid gland were studied for detection of HLA DR expression on the epithelial cells. One case of chronic pancreatitis was revealed to express HLA‐DR Ag on the epithelium. The patient was a 44 year‐old woman who had silently developed pancreatic cyst due to chronic inflammation. This finding also suggests a role of autoimmunity in the pathogenesis of chronic idiopathic p

ISSN:1320-5463    

DOI:10.1111/j.1440-1827.1990.tb01585.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

A 41 year old Japanese woman with lipoprotein glomerulopathy is reported. The patient presented with nephrotic syndrome, hyperlipoproteinemia and an increased level of plasma Apo E. The damaged glomeruli showed various degrees of capillary dilatation and accumulation of some material often showing stratification, along with mesangial proliferation and mesangial interposition. The accumulated material was stained positive for β‐ lipoprotein by the indirect immunoperoxidase technique. Capillary dilatation resulted from gradual accumulation of the lipoprotein as well as destruction of the mesangium, as if being torn off, due to increased intraluminal pressure. The patient's eldest sister also suffers from the same renal disease, suggesting a hereditary predispositi

ISSN:1320-5463    

DOI:10.1111/j.1440-1827.1990.tb01586.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY