No abstract available.
Mitochondrial Diseases*

The purpose of this study was to investigate the inhibitory role of vitamin A with respect to activation of Ito cells in fibrosis of the rat liver induced by common bile duct ligation(CBDL). The liver was examined by immunohistochemical staining for a-smooth muscle actin,the known marker of activated Ito cells, and light and electron microscopy after CBDL andCBDL with intraperitoneal injection of retinoic acid (Sigma, USA) 1 mg/Kg in 3 times per week. The results were sumrrlerized as follows: After CBDL, the bile ductules were markedly proliferated in the periportal areas extending toterminal hepatic veins. Interstitial fibrosis and inflammatory cell infiltration appeared, however,cholestasis was minimal. Retinoic acid treatment with CBDL decreased bile ductular proliferationand interstitial fibrosis compared to CBDL only. After CBDL, proliferated and activated Ito ceIs showing positive reaction in smooth muscle actin were present in the periductular andperisinusoidal areas, and areas of increased interstitial fibrosis. Activated ito cells weredecreased in number after CBDL with vitamin A treatment. Electron microscopically,intracytoplasmic fat droplets and the cytoplasmic processes of Ito cells were decreased afterCBDL. Myofibroblasts were frequently appeared in the interstitial fibrosis after CBDL. But,intracytoplasmic fat droplets of Ito cells were well preserved, and myofibroblasts were found lessfrequently after CBDL with vitamin A treatment. The results suggest that vitamin A plays an inbitory role in the activation and fibrogenesis ofIto cells after CBDL.
Rats ; Animals

Lipomas of the heart are benign neoplasms and have rarely been described. Due to the fact that they normally cause no symptoms, diagnosis is often purely accidental. Because of the rarity of these tumors, it seems worthwhile to present an example studied at autopsy. It was associated with the sudden death of a 15-year-old boy. The tumor arose from the wall of the left ventricle and occupied the pericardial cavity, measuring 13x7x6 cm in size. The tumor was whitish-yellow, translucent, and soft. Microscopically, the tumor was composed of mature adipose tissue which extended between muscle fibers. This current case, the giant cardiac lipoma is believed to produce disturbances of the conduction system and distrubances of cardiac filling.

The purpose of this study was to investigate the morphologic changes of the bile canaliculi and its associated structures of the liver induced by common bile duct ligation(CBDL) in the rat. The canalicular surface and lateral surface of the dry-fractured hepatocytes was studied with scanning electron microscopy at 1~6 weeks post ligation. The first week after CBDL, the bile canaliculi were dilated. The microvilli were increased in number and the lumens contained granular materials After 2 weeks or more, the bile canaliculi were dilated to a variable degree, and with irregularity, measuring from 1.5 to 5 micrometer in diameter, and in the advanced stage, the canaliculi showed blunting and the disappearance of microvilli. Some canaliculi had sprouting side branches. At 4~6 weeks post-ligation, the lateral surface of the hepatocytes also showed some irregularity and a tortuous appearance, and numerous small sized microvillous projections were formed. The tubular structures of the proliferated SER distributed adjacent to the lateral surface of the hepatocytes, and the direct connection of a tubular structure and the cytoplasmic membrane was observed. These results suggest that the deformity and loss of microvilli of bile canaliculi reflect the disturbance of bile secretion from the hepatocytes. And prolonged obstruction of bile flow may result in bile excretion via the lateral surface of hepatocytes.
Rats ; Animals

We describe an ovarian mucinous cystadenocarcinoma with a sarcoma-like mural nodule. In the literature, rare cases of ovarian mucinous tumors have been described which contain foci of undifferentiated carcinoma, sarcoma, and sarcoma-like nodules. The distinction between these lesions is important because of poorer prognosis of true sarcoma and anaplastic carcinoma than sarcoma-like mural nodules. This case shows different results of immunohistochemical stain for anaplastic carcinoma.

Inherited muscle diseases are heterogeneous with varying genetic etiologies and present with common symptoms and signs, including weakness, motor developmental delay, and hypotonia. To diagnose these various diseases, a meticulous family and clinical history, physical and neurological examinations, laboratory findings with electromyography, muscle biopsy, and genetic testing are needed. Here, I review several inherited muscle diseases, with a focus on muscular dystrophy in children and its genetics and general management.
Biopsy ; Child ; Electromyography ; Genetic Testing ; Humans ; Muscle Hypotonia ; Muscles ; Muscular Dystrophies ; Neurologic Examination

We report a case of plexiform neurofibroma developed under the overlying speckled lentiginous nevus, which occurred in a 20 year-old man. In this patient and his family no other signs of von Recklinghausens disease were found. Discussion is focussed on the fact that both plexiform neurofibroma and speckled lentigmous nevus, which represent a defect in the neural crest, occurred in the same area of the skin.
Humans ; Neural Crest ; Neurofibroma, Plexiform* ; Neurofibromatosis 1 ; Nevus* ; Skin

Floppy infant syndrome is a disease in which infants present with generalized hypotonia at birth or early infancy. There are many possible etiologies, which make a specific diagnosis difficult. The expanding knowledge of genetic disorders has made noninvasive genetic testing available for specific diagnoses. Therefore, it is very important for clinicians to use a systematic approach for the investigation of such children. In this chapter, I review the many possible etiologies of the floppy infant syndrome, and a systematic approach for the evaluation of this disorder will be proposed.
Child ; Genetic Testing ; Humans ; Infant ; Muscle Hypotonia ; Parturition

Tumors of the lung and bronchi containing cartilage were known by a variety of names, chondroma, adenochondroma, chondromatous hamartoma and mixed tumor. This variation in nomenclatures explain the difference of illustration on the nature of these tumor. The concept pulmonary harmatomas are benign neoplasm and not developmental malformations, has gained wide acceptance in recent years. We have experienced four cases of intrapulmonary hamartoma which were all discovered during routine chest film check up for certificate of health and evaluation of other disease. One case is added further detailed histologic examination by electron microscopy. The age at time of the detection were 53 (male), 23 (male), 39 (female), and 56 (female) years old. The mean size is 4.3x3.7x3.4 cm. The locations were three left upper lobes and one right upper lobe. Lobectomy and wedge resecions were done. Cut surface showed promiment lobular structures, papillary configuration and multiple cleft like spaces. Predominant cellular components were cartilage but fat tissue in one of the four cases. Microscopic findings showed abundant hyaline cartilages bearing lobular configuration and overlying pseudostratified ciliated columnar and cuboidal epithelium. Fibromyxoid and undifferentiated cells were seen in myxoid and fatty tissue. Electron microscopic findings revealed stellate, undifferentiated mesenchymal cells bearing collagen formation, stellate smooth muscle and transition areas between undifferentiated mesenchymal cells and mature cartilage. Epithelial components were similar to terminal bronchiole and alveolar epithelium. These findings suggest the concept that intrapulmonary hamartoma represent a histologic specturm of benign mesenchymal neoplasms, which originate in peribronchial connective tissue.
Female ; Male ; Humans ; Hamartoma

The purpose of this study is to investigate the morphologic changes of the sinusoidal endothelial cells and the associated structures of the cirrhotic rat liver induced by repeat intraperitoneal injections of N-diethylnitrosamine (DEN) (100 mg/kg/week). One day to 6 weeks later, rat livers were observed under the light, transmission and scanning electron microscopy, and immunostained with laminin antibody. Two weeks after DEN treatment, the fibrillar material in Disse's space was noted, and then a basement membrane-like structure was found at 4 weeks after treatment. Laminin was detected in perisinusoidal areas after 4 weeks. Laminin was strongly positive on the fibrous septum and in the sinusoidal wall of cirrhotic nodules after 6 weeks of treatment. The diameters and numbers of sinusoidal endothelial fenestrations did not change significantly until 2 weeks. They decreased within 4 weeks, and then the sinusoidal endothelium was poorly fenestrated at 6 weeks after DEN treatment. These results suggest that as fibrosis develops in cirrhosis, the deposit of extracellular matrix such as laminin within Disse's space is a major contributing factor in the structural alteration of sinusoidal endothelial cells, and the capillarization of the sinusoidal endothelial cells may be a contributor to impairment of the hepatic function in cirrhosis.
Rats ; Animals