CEROID-LIPOFUSCINOSIS. Report of Two Autopsy Cases

Light and electron microscopic examinations were performed on two autopsy cases of ceroid-lipofuscinosis of the juvenile (Case 1) and late infantile (Case 2) types. Much ceroid-lipofuscin (CL) was found in nerve cells throughout the nervous system. In Case 1, CL had also accumulated in thyroid follicular cells, glomerular podocytes, and epithelial cells of the ductus epididymidis, and in the endothelium and smooth muscle of vessels. Electron microscopy showed CL in 5% of peripheral lymphocytes sampled when the patient was alive. In Case 2, an accumulation of CL was found in the vascular endothelial cells of the cerebrum, and Kupffer cells and sinusoidal endothelial cells of the liver. The CL was autofluorescent, and was seen to be composed of electron-dense granules, lipid droplets, lamellar structures, and curvilinear bodies by electron microscopy. Limiting membranes were often found surrounding CL granules. The dolichol level in the cerebral cortex was high in Case 1. Accumulation of CL was found in cells other than nerve cells, although the main signs and symptoms were caused by the involvement of nerve cells. The CL showed various ultrastructural features. ACTA PATHOL JPN 38: 1191∼1203, 1988.     

Two Cases of Mushroom Poisoning by Podostroma Cornu-Damae

Podostroma cornu-damae is a rare fungus that houses a fatal toxin in its fruit body. In this case report, two patients collected and boiled the wild fungus in water, which they drank for one month. One patient died, presenting with desquamation of the palms and soles, pancytopenia, severe sepsis and multiple organ failure. The other patient recovered after one month of conservative care after admission. We found a piece of Podostroma cornu-damae in the remaining clusters of mushrooms. Mushroom poisoning by Podostroma cornu-damae has never been previously reported in Korea.     

Acute Aortic Dissection Due to Giant Cell Arteritis Report of Two Autopsy Cases

Two autopsy cases of giant cell arteritis with aortic dissection are reported. One patient was an 87 year old man, and the other was an 80 year old woman. In both cases the patient died suddenly, and fatal pericardial bleeding due to the aortic dissection was revealed at autopsy. Histologically, marked inflammation with giant cells was observed in the wall of the aorta and in other arteries including the cranial arteries. The authors therefore consider that giant cell arteritis was one of the preceding lesions that culminated in aortic dissection. Acta Pathol Jpn 39: 821 826, 1989.     

Two Autopsy Cases of Primary Leiomyosarcoma of the Liver Superiority of Muscle-specific Actin Immunoreactivity in Diagnosis

Two autopsy cases of leiomyosarcoma of the liver in a 49-year old female and 63-year-old male are reported. Both of the liver tumors showed electron microscopically dense patches in the cytoplasm and intermediate junctions between the tumor cells, suggesting a smooth muscle cell origin, irrespective of their different histological features. The nature of both tumors was confirmed by positive immunoreactivity for muscle-specific actin in the tumor cells, whereas desmin immunoreactivity was labile in both cases, showing a higher diagnostic value of the former compared with the latter in these leiomyosarcomas. Both cases, showed extensive distant metastases in spite of an evident difference in their mitotic indices, indicating that this index is not reliable for judging the metastatic potential of these tumors. Acta Pathol Jpn 41: 461–465, 1991.     

NEOPLASTIC ANGIOENDOTHELIOMATOSIS Report of Two Autopsy Cases with Special Reference to the Origin of Atypical Cells

Two autopsy cases of neoplastic angioendotheliomatosls (NAE) were presented. Both patients were elderly woman, characterized by an ascending progression of transverse myelopathy and abnormal brain shadows similar to metastatic tumor in computer tomographic examination in Case 1, and by typical clinical features of cerebral infarction in Case 2. Postmortem examinations of both cases revealed an exclusive intravascular distribution of large atypical cells in generalized organs, especially in the central nervous system, and associated multiple cerebral infarcts and, in Case 1, widespread demyelina-tion of the spinal cord. There were no distinct lesions suggestive of a primary focus. In both cases only a few atypical cells were immunohistochemically positive for factor VHI-related antigen, likely due to non-specific absorption of serum factor VIII into the cells. On the other hand, almost all of the atypical cells were immunoreactive for LN-1, LN-2, and leukocyte common antigen, suggestive of lymphocytic (B cell) origin.     

燃煤型砷中毒患者的球结膜微循环改变

探讨燃煤型砷中毒病人的微循环变化及其临床意义。方法应用LWB -Ⅰ型微循环仪对162例砷中毒病人及68例正常人作双眼球结膜微循环观测。结果不同程度砷中毒病人 ,其球结膜微循环变化也不尽相同。轻度砷中毒病人以球结膜微血管数目减少及血管周围渗出为主 ;中度砷中毒病人以血管粗细不均 ,血流速度缓慢较为显著 ;重度砷中毒病人的各项指标的异常发生率逐渐升高 ,尤以血球重度聚集及缺血区的出现更为突出。结论球结膜微循环障碍是慢性砷中毒病人的重要表现 ,监测其变化 ,有助于砷中毒病因的探讨、病情的判断及指导治疗     

PRIMARY PULMONARY ARTERY SARCOMA. Report of Two Autopsy Cases Studied by Immunohistochemistry and Electron Microscopy, and Review of 110 Cases Reported in the Literature

Two cases of primary pulmonary artery sarcoma are reported. The patient in the first case was a 61-year-old male with a two-year history of cough and exertional dyspnea, who died of intractable cardiac failure two months after admission without establishment of a diagnosis related to the etiology of cardiac failure. Autopsy revealed a sessile tumor within the pulmonary trunk and a solitary metastatic lesion in the lung. Histologic, immunohistocyto-chemical and electron microscopic studies were performed and a diagnosis of malignant mesenchymoma was made. The patient in the second case was a 32-year-old male complaining of exertional dyspnea and back pain. Radiologic studies indicated a mediastinal tumor involving the pulmonary artery. Exploratory thoracotomy revealed that the mediastinal mass arose from the left pulmonary artery. He died of respiratory failure 26 months after onset of his initial symptoms. Histologic, immunocytochemical and electron microscopic studies of both surgical and autopsy materials revealed a malignant fibrous histiocytoma. One hundred ten previously reported cases of this tumor are reviewed, and its clinicopathologic and morphologic features and probable histogenesis are discussed. ACTA PATHOL JPN 38: 883∼896, 1988.     

急性有机磷农药中毒纳洛酮治疗120例临床观察

目的比较有机磷农药中毒常规治疗组和加用纳洛酮治疗组,以探讨纳洛酮能否提高抢救成功率,降低死亡率。方法120例有机磷农药中毒患者随机分为常规治疗组（对照组）和加用纳洛酮治疗组,比较其在中毒程度、治愈、死亡、阿托品中毒、反跳、中间综合征等方面的区别。结果纳洛酮治疗组治愈率明显高于对照组,差异有统计学意义（P〈0.05）,纳洛酮治疗组治疗中毒昏迷清醒时间较常规治疗组清醒时间短,两组比较差异有统计学意义（P〈0.05）。结论治疗有机磷农药中毒患者加用纳洛酮可明显降低病死率,缩短中毒患者清醒时间,并减少阿托品中毒发生。从而提示纳洛酮可提高抢救成功率。