[Case of chronic tubulointerstitial nephropathy associated with idiopathic ductopenia].
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Biomedical subjects
Publications and source records attributed to J Haratake.
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Malignant transformation of a mediastinal neurofibroma, presenting for many years as a mass in the superior mediastinum and arising from the right vagus nerve, was found in a patient with long-standing von Recklinghausen's disease. Surgical management of intrathoracic neurogenic tumours, even if clinically benign, is advocated.
A 59-year-old male started maintenance hemodialysis, and 80 months later died of congestive heart failure. Just before death he had no urine, and no monoclonal immunoglobulin could be demonstrated in the serum on immunoelectrophoresis. An autopsy showed deposition of periodic acid-Schiff-positive and Congo red-negative material in the interstitium and vessel walls of almost all organs and tissues. In the glomeruli there was a multifocal nodular accumulation of the material. Electron microscopy showed finely granular, not fibrillar deposits. In spite of these impressive lesions on light microscopy, the material was not stained with commercially available antisera to light chains. However, the material was positive for antisera to the variable region of the kappa1 light chain subgroup. This is the first case with light chain deposition disease that showed positive staining only with antisera to the variable region of the light chain.
A rare outbreak of acute hepatic damage in workers exposed to dichloropropanols (DCPs) was reported recently. The purpose of the present study is to examine the effects of DCPs on various organs, the dose dependency and the pathogenetic potential of DCP hepatotoxicity. A single intraperitoneal injection was given to six groups of rats with 0.2 ml of 20% ethanol (control), or 1/8 x, 1/4 x, 1/2 x, 1 x, and 2 x LD50 (0.11 ml/kg) of 1,3-dichloro, 2-propanol (DC2P) diluted in 20% ethanol. After blood samplings, all organs were subjected to histologic examinations with light and electron microscopes. Fresh liver tissues from further 4 control and 4 experimental rats sacrificed 6 hours after the injection of 20% ethanol and 1 x LD50 of DC2P were homogenized and subjected to evaluate lipid peroxidation, glutathione S-transferase activity and reduced glutathione contents in the liver. The rats administered with only ethanol or 1/8 and 1/4 LD50 of DC2P showed no serological or histopathological abnormalities. Marked elevation of serum glutamate pyruvate transaminase (SGPT) with a diffuse massive necrosis of the liver cells were noted in all rats of both the 1 x and 2 x LD50 groups, and irregular zonal necroses were found in 3 of 4 rats injected with 1/2 LD50. There were no serious toxic changes in other organs. Hepatic malondialdehyde level was significantly increased, associated with decreases of liver glutathione S-transferase activity and reduced glutathione content. It was concluded that the serious DC2P-toxicity was mainly found in the livers, dose dependently, and one of the causative mechanisms of this hepatotoxicity might be the lipid peroxidation.
Mucin-producing tumours of the pancreas have been recently reported with increasing frequency and most cases have occurred in middle-aged and elderly people. In the present report, a case of a 21-year-old man with mucinous cystadenoma of the pancreas is reported. He had a long history of recurrent pancreatitis from the age of 8. When he was aged 10, the first branch of the main pancreatic duct was shown to be enlarged on endoscopic retrograde pancreaticography (ERP). A series of ERP studies and computed tomography scans performed over a period of 11 years demonstrated continuing growth of this enlargement of the pancreatic duct. Pancreaticoduodenectomy was performed and the patient has been well without further episodes of acute pancreatitis and has been free of recurrent tumour for 1 year.
In this morphological study the authors investigated whether spinal cord cavitation, produced in young mongrel dogs that had been rendered hydrocephalic by cisternal injection of kaolin, consists of a dilated central canal or intramedullary cavities. Hydrocephalus was noted in 50 of 56 dogs treated with kaolin. Of the 50 hydrocephalic young dogs, 29 were shown to have central canal dilation that was prominent at the thoracic level and 21 to have cervical intramedullary cavities in the posterior column and/or the posterior horn. In 11 dogs from the latter group these cavities were demonstrated to have no communication with the central canal. This finding could not be explained by the hydrodynamic theory. On histopathological examination, myelomalacia and hemorrhagic infarction following ventricular shunting were noted adjacent to the cervical cavities, which suggested vascular impairment. A perfusion study revealed insufficient blood flow within the cervical cord at the level of the intramedullary cavities. A close correlation between the vascular insufficiency of the cervical cord and the pressure cone resulting from significant hydrocephalus was observed. The latter may cause cervicomedullary compression at the foramen magnum, affecting the venous drainage of the cervical cord below that level, resulting in intramedullary cavitation. Accordingly, vascular impairment was thought to play a significant role in the development of cervical syrinx formation in our kaolin model. The current results may provide a reasonable explanation for the formation of noncommunicating cervical syringomyelia in Chiari I malformation.
BACKGROUND: Ordinary craniopharyngiomas affecting sellar regions of children and preadolescents are composed of ameloblastomatous and/or squamous constituents. The authors encountered an autopsy case of a stillborn infant with a large craniopharyngioma with unusual manifestations. METHODS: The craniopharyngioma was analyzed using detailed histologic and immunohistochemical techniques. RESULTS: An autopsy revealed a large tumor arising from the pituitary gland with associated severe hydrocephalus. Histologically, the cells of the tumor showed features of ameloblastoma and organoid structures simulating tooth buds and adenohypophyseal primordia in the stage of organogenesis. Cells of the latter were shown to be immunoreactive for chromogranin A and six adenohypophyseal hormones. CONCLUSIONS: Because embryology of the adenohypophysis and the teeth indicated that all of the epithelia in this tumor emerged during the normal development of the stomodeum, it was believed that an "embryonal form of craniopharyngioma" would characterize this organoid neoplasm best. The possible morphogenesis of craniopharyngioma is also discussed.
Thirteen cases of combined hepatocellular (HCC) and cholangiocellular carcinoma (CCC) were examined. In addition to routine pathology, immunoreactivities for carcinoembryonic antigen, alpha-fetoprotein (AFP), cytokeratin (Cam 5.2 and AE1), epithelial membrane antigen (EMA) and tumor-associated glycoprotein 72 (B72.3) were also examined. The average age of the 13 cases was 64.8 years, which lay between the average ages of pure HCC and CCC cases. They were categorized as separate type (2), collision type (6), and intermingled type (5). AE1 and EMA were the best markers to differentiate the CCC from the HCC area. B72.3 immunoreactivity was detected only in CCC (46%). There were no transitional features between HCC and CCC in two cases of the separate type and two cases of the collision type. However, focal transitional features from HCC to CCC were observed in all cases of the intermingled type and in four of six cases of the collision type. In one case of the intermingled type, many cancer cells contained both bile and mucus simultaneously, and revealed dual immunoreactivities. The conclusions are: 1) the combined type is generated from two sources; one is the intrahepatic double cancer (thoroughly separate type and a part of the collision type) and another is the stem cell origin with diverse phenotypes (intermingled type and a part of the collision tumor); and 2) AE1 was the most helpful marker to differentiate the CCC area from HCC, and other markers, e.g. AFP for HCC and EMA, CEA, and B72.3 for CCC, were also supportive but somewhat limited in the differential diagnosis.
We previously reported a case of Addison's disease associated with acquired immunodeficiency syndrome (AIDS) (Endocr J, 41:13, 1994). A 46-year-old man with hemophilia B and AIDS was diagnosed as Addison's disease. The positive cytomegalovirus (CMV) antigen in urine suggested that CMV adrenalitis may have caused the adrenal insufficiency. Despite treatment with ganciclovir, the patient died one year later. Autopsy findings revealed that the typical inclusions of CMV were seen in the lung, adrenal glands (both cortex and medulla) and small intestine. Here, we describe the subsequent clinical course and postmortem findings of this case.
The chronological changes in ductular epithelium and peribiliary capillary plexuses (PBPs) after bile duct ligation are not well understood. Therefore, we examined alterations in both intrahepatic bile ductules and peribiliary microcirculation in rats after ligation of the common bile duct using immunohistochemistry, transmission electron microscopes (TEM), and scanning electron microscopes (SEM). Ductular proliferations appeared first in the peripheral areas of the portal spaces and then gradually advanced along with increasing jaundice. Distorted configuration of the hepatic parenchyma with interconnecting stroma rich in irregular ductules developed 4 weeks after the ligation. Numerous biliary cell types and cells of types intermediate between hepatocytes and biliary cells together with poorly fenestrated capillary-type vessels appeared in the periportal parenchyma, in association with an increased number of canalicular-ductular junctions on TEM. These biliary cells were often found within the lobules and apart from the stroma. SEM examination of hepatic microvascular casts using methacrylated resin showed formation of irregular portal and periportal capillary networks, partly derived from coarsened sinusoids. Direct connections between the newly formed capillary networks and the pre-existing PBPs or sinusoids were numerous, although there were few direct connections between the capillary networks and the hepatic arterial branches. Thus, these proliferated ductules and newly formed complicated capillary networks might play an important role in the effective transport of biliary materials between hepatocytes and native bile ducts or proliferated ductules through the altered microcirculation after bile duct ligation.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
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An acute phase of severe hepatic necrosis induced by dichloropropanol was examined immunohistochemically and ultrastructurally, in order to study chronological changes of sinusoidal morphology during acute hepatic injury. Male Wistar rats were injected with 1,3-dichloro-2-propanol (DC2P) and sacrificed at various intervals after the injections. DC2P-injected rats showed zonal necrosis of the centrilobular space with a peak from 24 to 48 h after the injection. Destruction of sinusoidal linings appeared at 4 h, and was gradually aggravated along the advancing hepatocytic necrosis. Monocytic influx into the necrotic areas was initiated at 6 h. At 48 h, collapsed centrilobular spaces showed a loss of most sinusoidal structures with active phagocytosis of macrophages, proliferation of perisinusoidal cells, and accumulation of collagen fibrils. At 72 h, there were many regenerating sinusoidal structures, which were composed of rather thick and less fenestrated endothelium and underlying multilayered processes of mesenchymal cells, along the regenerating hepatocytes. In these areas, occasional junctions between regenerating hepatocytes and mesenchymal cells were seen. Reconstruction of sinusoidal linings was closely related to the hepatocytic regeneration, and a hepatocytic-mesenchymal interaction might participate in this morphodynamic course of the sinusoidal reconstruction.
Male Wistar rats were exposed to two types of magnesium sulphate whiskers by inhalation for six hours a day, five days a week, for four weeks (sub-chronic study), or for one year (chronic study) to clarify the biological effects of the whiskers. There were few whiskers detected in the rat lungs even at one day after the exposure, suggesting that they are dissolved and eliminated rapidly from the lungs. To measure the clearance rate of the whiskers from the lungs, an intratracheal instillation was performed in golden hamsters. The half life of the whiskers in the lung was determined as 17.6 minutes by temporally measuring the magnesium concentration up to 80 minutes after the instillation. A histopathological examination indicated a frequent occurrence of adenoma and carcinoma in the year after chronic exposure, but it was not significantly different between exposed and control rats.
A 42-year-old man with Behçet's disease of the intestinal type and a chronic cholestatic profile is presented. He had oral aphthea, genital ulcer, erythema nodosum, thrombophlebitis and central retinitis during the clinical course. Deep ileal ulcers recurred with massive bleeding and perforation. Cholestatic laboratory findings were also noted in the clinical course. A biopsied liver specimen, taken at the second operation for the ileal ulcer, showed chronic portal inflammation associated with ductopenia and epithelial degeneration of the small interlobular bile ducts, but periductal concentric fibrosis was not found. The patient died of sepsis 7 months after the last laparotomy. Although there was no significant abnormality in the large biliary tract at autopsy, pericholangitis in the small portal tracts and cholestasis were pronounced. Sclerosing cholangitis is one of well-known complications of ulcerative colitis or Crohn's disease. We report herein another rare association of small bile duct damage resembling sclerosing cholangitis with enteritis in Behçet's disease.