Immotile cilia syndrome is a genetically determined disorder characterized by immotility or poor motility of the cilia in the airways and elsewhere. Since its first description in 1976 determination of a ciliary abnormality has now clarified its variable expression and pleiotropism. Certain specific defects in the ciliary axoneme can be found and are pathognmoic of the syndrome. These defects include missing dynein arms, abnormally short dynein, arms, spokes with no central sheath, missing central microtubules, and displacement of one of the nine peripheral doublets. We have studied 80 cases of bronchial or nasal mucosal biopsies that were performed with the suspicion of immotile cilia syndrome. Of 80 cases only 17 cases were sampled optimally to be able to observe under transmission and scanning electron microscopes. All 17 cases had certain abnormality of the cilia. They consisted of Ia(3 cases), Ib(3 cases), Id only(3 cases) and Id+other types(6 cases) a according to Sturgess classification. Seven cases consisted of 1 solitary and 6 combined form; II+Id(1 case) and II+Id+III(5 case). All 5 cases of type III were combined with Id and II. Clinically most pronounced manifestations were cough(82%), sputum(59%), rhinorrhea(41%) and nasal stuffiness(35%), All the patients were below the age of 15 years, and there were 6 boys and 11 girls.
Biopsy

Cytomegalovirus(CMV) infection can be diagnosed by finding characteristic intranuclear inclusion body and enlargement of the cell size congenital CMV infection can be associated with various types of anomalies seen in different gestational age. These anomalies are probable due to direct virus infection of the parenchymal cell m early gestation. Based on four autopsy cases of congenital CMV infection we have studied maturation process of virus particles in parenchymal cells, with special reference to me mode of replication and transmission. Virus particles in CMV-infected cells in brain and kidney showed nucleocapsids with characteristic concentric core, that were enclosed around fibrillar network in nucleus. During replication process virions showed various morphogenic mutation that was rather consistent in different tissues and individuals. There were virions without core or with eccentric core. Occasional cores were divided into 2~5 fragments. The virus particles reached the cytoplasm through the nuclear membrane, and here the virions increased twice in size. After virions were fully matured in the cytop1asm. they showed exocytosis phenomenon through the cellular membrane to reach extracellular portion.
Humans

Many pictures must be taken for the presumption of the stereorelation of the cerebral artery, which obtained with ordinary cerebral angiography. And it is very difficult to understand the stereoimage and required many experiences. But it is able to presume the stereorelationship in only brief eye's training without the aid of the stereoscope using the prism. For the stereoview, we need the paired angiograms obtained only straight and tilting the X-ray tube. In practice, with this stereoview in cerebral angiogram, we could know the directions of the aneurysmal neck and fundus with the stereorelationship of the cerebral artery and indeed helps us greatly in operation field. In addition, we might guess the location and stereorelationships of the feeding artery and draining vein in arteriovenous malformation and other vascular tumors and it was great aid in diagnosis and operation. Now we present the methods of the pictures for the stereoview in cerebral angiogram and the methods for eye's training.
Aneurysm ; Arteries ; Arteriovenous Malformations ; Cerebral Angiography ; Cerebral Arteries ; Diagnosis ; Neck ; Veins

It is well known that intracranial pressure (ICP) and mean arterial pressure (MAP) are increased by laryngoscopy and endotracheal intubation during induction of general anesthesia, and it may be very dangerous in neurosurgical patients who had increased ICP. Therefore, this study was performed to know the range of ICP increase during induction of the conventional general anesthesia with intubation following thiopental and succinylchohne injections. Intracranial pressure and MAP were measured in 13 patients who underwent craniotomy. All the patients were monitored cerebral epidural ICP and intraarterial pressure preoperatively. The results were as follow: 1. Intracranial pressure was increased of 7.1±7.23 mmHg. 2. Arterial pressure was increased of 43.5±25.46 mmHg. 3. Cerebral perfusion pressure was increased of 33.3±27.53 mmHg. It is stressed that certain procedures are necessary to prevent from further increase of ICP due to induction of general anesthesia in patients with increased ICP.
Anesthesia, General ; Arterial Pressure ; Cerebrovascular Circulation ; Craniotomy ; Humans ; Intracranial Pressure* ; Intubation ; Intubation, Intratracheal* ; Laryngoscopy* ; Thiopental

No abstract available.
Subarachnoid Hemorrhage*

Molluscum contagiosum virus(MCV) infection occurs in both sporadic and epidemic forms, and affects children more frequently. The skin lesions are small, pinkish and wartlike tumor. MCV has not been transmitted to animals and nQt propagated in cell culture. Therefore the viruses in human lesions can only be demonstrated by electron microscopy. Histologically, characteristic intracytoplasmic inclusion bodies (called molluscum bodies) are formed in infected epithelial cells. They gradually enlarge and eventually fill the cell. Ultrastructure of molluscum bodies in infected cells showed a large number of MCV particles that are variable-sized, spherical, ellipsoidal and brick-shaped. Early viral replication stage shows small dense bodies in the nucleus and then reveal immature MCV that consist of homogeneous rounded core and separated capsidal membrane from the cytoplasm matrix In advanced stage, shrinked nucleus with irregular membrane moves into marginal area of the cell. The spherical fon-ns represented immature viruses in cytoplasm matrix, whereas the brick-sphaped forms represented mature viruses in the inclusion of cytoplasm.
Child ; Male ; Female ; Humans ; Animals

A total of 21 cases of sacrococcygeal teratomas is reviewed and analyzed histopathologically. They were 15 mature teratomas, one immature teratomas, and five endodermal sinus tumors. All but one neoplasm occurred in infants aged 3 years or younger and had visible masses at birth. Seventeen of these cases occurred in females. In mature teratomas, variable teratoid elements were observed and neuroglial tissue was particularly prominent among them. Also found were gastrointestinal mucosa, respiratory mucosa, pancreas, retinal tissue, and cartilage in order of frequency. Only one case of mature teratoma recurred and turned out to be endodermal sinus tumor with metastasis to the lungs. One immature teratoma showed undifferentiated neuroectodermal tissue in over half of the total area and the remaining portion consisted of mature neuroglial tissue and gastrointestinal mucosa. All endodermal sinus tumors were not combined with germinomatous or choriocarcinomatous elements. Three cases of endodermal sinus tumor contained mature teratoid components adjacent to the carcinomatous area. Based on the findings described above, we discussed some different histopathologic characteristics between the sacrococcygeal teratomas and gonadal teratomas, and speculated the possible origin of sacrococcygeal teratoma from the sequestrated protein in the proximal part of cloacal membrane. And this sequestration might be occurred during the process of cephalo-caudal folding in early embryonal period.
Infant ; Male ; Female ; Humans ; Neoplasm Metastasis

A human embryo obtained incidentally from a hysterectomy specimen of a 36 year old woman who was operated for chronic pelvic inflammatory disease, was serially sectioned and reconstructed with 104 section slides. This embryo was characterized by a thick corneal body with well developed Descemet's endothelium, the optic nerve having vascular canal, the cochlear turning a little more than one time, the hypophysis with threadlike stalk, the vomeronasal organ having intermediate long narrow canal, the submandibular gland with deep lumen, the kidney with short secretory tubules and few large glomeruli and a well formed osseous band in the cartilage and bone. From above findings, it was concluded that this embryo belongs to the age group XXIII of Streeter's developmental horizon.
Female ; Humans

Lymphoid lesions of the orbit and conjunctiva may be divided histologically into three groups: monomorphous and cytologically atypical malignant lymphomas, benign reactive follicular hyperplasia or inflammatory pseudotumor, and borderline lymphoid lesions mainly composed of small, non-atypical lymphocytes with or without evidence of plasmacytic differentiation or germinal centers which are difficult to clarify its nature by histologic criteria alone. From 1984 to 1992 at Seoul National University Hospital, 20 cases of orbital lymphoid infiltrates were reviewed to find out histopathologic characteristics of malignant lymphomas and try to classify them properly. Also, we sought histologic findings helpful for differential diagnosis between malignant and borderline cases. Histologic examination of malignant lesions usually revealed the features of low-grade B cell lymphomas of mucosa-associated lymphoid tissue(MALT) origin, which predominantly consist of centrocyte-like(CCL) cells. Among 13 primary orbital lymphomas, six cases were classified as MALT lymphomas of Isaacson, six were more apt to be the type of immunocytoma by kiel classification, and remaining one showed mixed pattern of both types in areas. The one case of malignant lymphoma with diffuse large cell type was confirmed by secondary involvement of intestinal lymphoma. Five cases were confirmed by immunoglobulin k-light chain monoclonality on flow cytometry and immunofluorescence study. The histologic findings such as Dutcher bodies, hemosiderin, deposits and polykaryocytes tended to be more frequently encountered in malignant lymphoma rather than indeterminate lesions and were thought to be helpful ones in making differential diagnosis between malignant and benign lesions. With above findings, we assume that the principal neoplastic cells in the low grade lymphoma arising in the orbit and conjunctiva are CCL cells, which might be originated from the localized memory B cells, and also several kinds of B lymphocytes in on-going differentiation stages such as medium-sized monocytoid B cell, lymphoplasmacytoid cell, plasma cell, and mature small lymphocyte also contribute to the tumor progression.
Diagnosis, Differential

Typical nephroblastoma arise within the renal parenchyme and have pseudocapsules composed of compressed renal tissue, renal capsule and adjacent perirenal tissue. Therefore, it is rarely possible to determine whether the tumor arose from the cortex or medulla. Many authors believe that they are probably cortical in origin and arise from nodular renal blastema just beneath the renal capsule or in the centers of columns of Bertin. We experienced a nephroblastoma supposed to arise from peripelvic region. The patient was a 7 months old male infant with an abdominal mass. Left nephrectomy was done under the impression of nephroblastoma. Received specimen was a diffusely enlarged kidney with preserved normal outline, so it looked like a hydronephrotic kidney. On section, the pelvis and calyceal system were dilated and totally filled with a gray white soft mass. Most portion of the mass was floating freely in the calyces and pelvis, and only a small portion was attached to the renal parenchyme but without invasion to it. Microscopically the mass was a nephroblastoma containing many well developed glomeruli and tubules. We presumed that the tumor arose from a nodular renal blastema in the peripelvic region, because the tumor had no connection to the renal cortex.
Infant ; Male ; Female ; Humans