Congenital subglottic stenosis of the larynx is one of the most common cause of chronic airway obstruction im infancy and childhood. It is defined as narrowing of the space bounded inferiorly by the inferior margin of the cricoid cartilage amd superiorly by the insertion of the fibers of the conus elasticus into the true vocal cords. In case we experienced was a female full-term baby delivered by Cesarean section. The stenosis was believed by hypertrophy of stromal soft tissue and cricoid cartilage in the subglottic area. The lesion was associated with tracheoesophageal fistula of H1 type. A brief review of the literature was done.
Child ; Male ; Female ; Humans

A case of desmoplastic fibroma of mandible in a 18 years old woman is presented. She had complained progressive swelling of right mandible for 4 years. Radiographically, a multilocular radiolucent of right hemimandibulectomy showed multinodular external surface without cortical destruction. Cut surfaces revealed grayish white, fibrous homogeneous appearance with firm consistency, sparing head portion. The maximum diameter of the tumor was 13 cm. Microscopically, the tumor was composed of interlacing bundles of monomorphic spindle-shaped cells with abundant intercellular collagen. Ultrastructurally, most tumor cells were fibroblastic-like cells with abundant RER and cytoplasmic fibrils, but a few disclosed transition to myofibroblasts. However, no fully developed myofibroblasts were seen.
Female ; Humans

A case of desmoplastic fibroma of mandible in a 18 years old woman is presented. She had complained progressive swelling of right mandible for 4 years. Radiographically, a multilocular radiolucent of right hemimandibulectomy showed multinodular external surface without cortical destruction. Cut surfaces revealed grayish white, fibrous homogeneous appearance with firm consistency, sparing head portion. The maximum diameter of the tumor was 13 cm. Microscopically, the tumor was composed of interlacing bundles of monomorphic spindle-shaped cells with abundant intercellular collagen. Ultrastructurally, most tumor cells were fibroblastic-like cells with abundant RER and cytoplasmic fibrils, but a few disclosed transition to myofibroblasts. However, no fully developed myofibroblasts were seen.
Female ; Humans

Malignant mixed mesodermal tumor (MMMT) of the ovary is endometrioid tumor containing epithelial and mesenchymal components. The mesenchymal component may be homologous, in which case the term carcinosarcoma is often used, or heterologous, containing cartilage, striated muscle, osteoid, bone, or fat, or combination of these elements. MMMT usually afflicts the postmenopausal woman and occurs more often in the nulliparous female. The disease is usually advanced (stage III) when diagnosed. Cliniopathologic data were presented for 3 cases of MMMT of the ovary with review of literatures. Clinical history substantiated previous reports in postmenopausal women wit this tumor. Symptoms were the same as for ovarian malignancy in general. All the patients presented wit stage II disease, according to the International Federation fo Gynecology and Obstetrics (FIGO) classification. Two patients died of tumor 4 and 10 months after operation, respectively. One patient had been alive at 2 months after operation. None of 3 cases demonstrate evidence of endometriosis in the remaining ovaries. Microscopically, periodic acid-Schiff (PAS)-positive, diastase-resistant hyaline droplets were found frequently in undifferentiated mesenchymal stroma and epithelial structures. The clinical significance and origin of the hyaline droplets in MMMT should be further explored.
Female ; Humans

Because neuroblastoma is predominantly a tumor of childhood, patholgists and clinicians tend to exclude it from differential diagnosis when the patient is an adult. The diagnosis of neuroblastoma by light microscopy can be difficult because of similarities between neuroblasts and cells of other round cell neoplasms. The fine structural features of neuroblastoma is distinctive and is valuable in its diagnosis. We present 2 cases of neuroblastoma and a case of ganglioneuroblastoma arising in adults with their ultrastructural findings.
Child ; Adult ; Male ; Female ; Humans ; Diagnosis, Differential ; Neuroblastoma

Electron microscopic study of two malignant fibrous histiocytomas confirmed the presence of previously described tumor cells in the literature. In addition, there existed intermediate cells with morphologic features of both myofibroblasts and histiocytes, or both histiocytes and smooth muscle cells. Our result supported the idea that malignant fibrous histiocytoma may be derived from the undifferentiated mesenchymal cells that differentiate primarily along a fibroblastic and histiocytic cells.

OBJECTIVES: The purpose of this study was to assess the objective and universal attitudes toward mentally-disabled defendants. The authors examined the criminal characteristics of the mentally-disabled defendants and clarified the differences between psychiatric opinions about the criminal responsibilities of mental patients and the final judicial decisions. METHODS: The subjects consisted of 75 mentally-disabled defendants. They had been referred to Seoul Eunpung Municipal hospital during the period of Jan. 1990 to Mar. 1997. We examined them through psychiatric interview, clinical psychological tests and other tests. The data obtained were analyzed in several aspects: demographic factors, characteristics of crime, psychiatric opinions and legal opinions. RESULTS: Regarding the demographic background, most commonly met subjects were males, single, in their twenties and thirties, with educational background of about 11 years, unemployed, and living in urban districts. Approximately one third(37.5%) of all subjects were diagnosed as schizophrenia, 10.8% an mood disorder and 9.3% as personality disorder. The most frequent criminal act was assault and battery(28.0) and the mental states at the time of the criminal act could be inferred as delusion/hallucination(52.0%) and paroxysmal emotional uncontrollable state(33.3%),etc. About forty perecnt(39.4%) of victims were strangers, 25.3% were public servants 21.3% were family members. According to the psychiatric report out of 75 defenddants, defendants were classified as followes:44 defendants were evaluated to be totally free of criminal responsibility, 22 defendants to be partially responsible, and 5 defendants to be fully responsible. However, the psychiatric evaluation was found to be in contrast with the final judicial decisions by the court:16 defendants were totally free of criminal responsibility, 44 of restricted criminal responsibility and 15 fully responsible. In case of no responsibility, the agreement rate between the judge's opinions and the psychiatric ones was 36.3%, for partial responsibility it was 73.0%, and for full responsibility it was 100%. DISCUSSION: The judges do not completely agree to psychiatric opinions. Therefore, the authors suggest that more facilitated communication between psychiatrists and judges should be necessary for the benefiit of mentally-disabled defendants.
Crime ; Criminals ; Demography ; Disability Evaluation* ; Hospitals, Municipal ; Humans ; Male ; Mentally Ill Persons ; Mood Disorders ; Personality Disorders ; Psychiatry ; Psychological Tests ; Schizophrenia ; Seoul

To our knowledge, MR findings of neuroshistosomiasis has not been reported in Korea. A case of neuroshistosomiasis involving cerebellum and presumably spinal cord is reported. A 40 year old man who had lived in Middle East complained of low back pain and progressive paraparesis for 6 months, and subsequently developed headache, dizziness and diplopia. On cervical spine MRI, there was diffuse enlargement of cervical cord with increased signal intensity on T2 weighted image and nodular heterogenous enhancement after Gd-DTP^ administration. Brain MR imaging obtained 4 months later showed ill-defined, irregularly enhancing heterogenous mass in cerebellar vermis which was proved to be a granulomatous lesion containing shistosomiasis mansoni ova.
Adult ; Brain ; Cerebellum ; Diplopia ; Dizziness ; Granuloma* ; Headache ; Humans ; Korea ; Low Back Pain ; Magnetic Resonance Imaging* ; Middle East ; Myelitis, Transverse* ; Neuroschistosomiasis* ; Ovum ; Paraparesis ; Spinal Cord ; Spine

Congenital lipoid adrenal hyperplasia, the most severe form of congenital adrenal hyperplasia, is caused by mutations in the steroidogenic acute regulatory protien (StAR). It is characterized by failure of synthesis of all three classes of adrenal steroids and massive accumulation of lipids and cholesterol in the adrenal cortex. The computed tomography (CT) unequivocally delineated massively enlarged adrenal glands of fat-tissue attenuation, enabling early diagnosis and replacement therapy. We report a case of congenital lipoid adrenal hyperplasia, in which CT established that lipoid deposition at the adrenal cortex disappeared after the adrenal hormone replacement therapy.
Adrenal Cortex ; Adrenal Glands ; Adrenal Hyperplasia, Congenital ; Cholesterol ; Early Diagnosis* ; Hormone Replacement Therapy ; Hyperplasia* ; Steroids

No abstract available.
Leukodystrophy, Metachromatic*