We experienced two cases of congenital adrenal hyperplasia(CAH), complicated by true precocious puberty with early maturation of the hypothalamic-pituitary-gonadal axis in a boy and a girl. The boy was diagnosed as CAH at 8 years of age, when he showed pseudoprecocity with adrenal rest tumor. The girl was diagnosed at neonate, when she showed ambiguous genitalia and salt-losing symptom. They developed precocious puberty during steroid treatment. Clinically they showed precocity, advanced bone age, and decreased predicted adult height, even though they showed partial gonadotrophin response to luteinizing hormone releasing hormone (LHRH) stimulation test. They were treated with LHRH analogue(leuprolide depot) resulting no further progression of precocity.
Adrenal Hyperplasia, Congenital* ; Adrenal Rest Tumor ; Adult ; Axis, Cervical Vertebra ; Disorders of Sex Development ; Female ; Gonadotropin-Releasing Hormone ; Humans ; Infant, Newborn ; Male ; Puberty, Precocious*

We performed a histopathologic and immunohistochemical study of 23 cases of surgically resected large cell undifferentiated carcinoma(LCUC) of the lung. The relative incidence of LCUC was 7.6% among the total resected cases of primary lung cancer over 7 years(1987-1993). The mean age of the patients was 56 years and 21 cases were male. The mean size of the mass was 5 cm and 11 cases were located peripherally. According to the histologic features, LCUC could be divided into three groups: squamous cell carcinoma-like(6 cases), adenocarcinoma-like(13 cases), and small cell carcinoma-like(4 cases) groups. The histologic differences were related to the variations of the immunohistochemical properties, but there were no differences in prognosis among these groups. Immunoreactivity to cytokeratin(CAM 5.2) was demonstrated in 22/23(96%). Carcinoembryonic antigen was positive in 13/23(57%). Neuron specific enolase and chromogranin were positive in 11/23(48%) and 5/23(22%), respectively. Vimentin was seen in 11/23(48%). From these observations, we could subclassify them by their immunologic phenotypes; exocrine features in 6/23(26%), neuroendocrine(NE) features in 4/23(17%), both exocrine and NE phenotypes in 7/23(30%), and 6 cases(26%) showed neither phenotype. The group with NE features showed a worse prognosis(P<0.05) and immunoreactivity for vimentin was also related to a worse prognosis(P<0.05). These findings imply that the immunohistochemical properties of LCUC are closely related to the histopathologic features. The groups, subdivided by histology and immunoreactivity, showed no prognostic difference except for the NE differentiation and reaction for vimentin.
Incidence ; Lung Neoplasms

Decubitus ulcer is often seen in the skin and underlying tissue of debilitated or immobilized patients as the result of prolonged pressure and impaired circulation. It manifests chiefly as an ulcer over bony prominences, but tumefaction is an extremely unusual presentation. A 53-year old male, a paralytic of the lower extremity for 18 years, developed a recurrent decubital ulcer despite repeated surgical repair, from which a rapidly growing, large fungating mass grew within a month. The last resected mass was bosselated and measured 15x9x3 cm with a major area of cicatrix-like induration, interdigitated with skeletal muscle bundles at the central area. Microscopically, the mass was composed of an upper half of active granulation tissue layer and a deeper half of dense, poorly cellular, fibrocollagenous bundles admixed with florid proliferation of atypical fibroblasts, but the absence of mitosis and the multifocal admixture of active inflammatory process-granulation tissue formation seemed to help exclude genuine fibromatosis, nodular fasciitis or proliferative myositis. We assume that this rapidly growing pseudofibromatosis is an additional manifestation of a prolonged decubitus ulcer, possibly related to the modified reparative process of decubitus ulcer following repeated excisions, for which we propose a term of decubitoma.

HIV-1 p24 was cloned into multiple cloning site of pMV261, extrachromosomal expression vectors carrying BCG replication origin and BCG-specific heat-shock promoter, and then introduced into BCG and E. coli. Western blot experiments showed that the p24 efficiently expressed in recombinant BCG (rBCG), but not in E. coli. Recombinant p24 expression induced by a single heat-shock of rBCG was maintained longer than 3 weeks. Immunoblot experiments with intact rBCG did not show any distinctive positive signal, suggesting that the recombinant protein was not secreted or exposed at the surface of BCG. The guinea pigs immunized with live rBCG showed delayed type hypersensitivity (DTH) by the systemic area as well as an effective humoral immunity, suggesting that tbis rBCG is believed to elicit eKcient immune responses against p24, even though the expression is restricted only in the cytoplasm as reported previously with other antigen. These results demonstrate that BCG can be developed as a live recombinant vaccine vector against a broad spectrum of infectious disease.
Animals* ; Blotting, Western ; Clone Cells ; Cloning, Organism ; Communicable Diseases ; Cytoplasm ; Guinea Pigs ; HIV* ; HIV-1 ; Hypersensitivity ; Immunity, Humoral ; Mycobacterium bovis* ; Replication Origin

No abstract available.
Meningioma* ; Neoplasm Metastasis*

Purpose: The diagnostic usefulness of fetal echocardiography in congenital heart disease is well known. One of the indications for fetal echocardiography is presence of extracardiac anomalies and such coexistent congenital heart disease may have important implications for obstetric and neonatal management. MATERIALS AND METHODS: 496 women with singleton pegnancies with amgenital anomalies that underwent penatal care and follow-up in Severance Hospital for 5 years from 1991 to 1995 were evaluated for the incidence of congenital heart disease. RESULTS: The incidence of congenital heart disease in fetuses with the associated extracardiac anomalies were as follows; central nervous system 6.9%(11/160), gastro-intestinal system 10.7%(8/75), genito-urinary sysem 4.7%(4/85), others 5.6%(4/72). Thirty-one of eighty-ane fetuses with congenital heart disease were found to have the associated extracardidac anomalies and ten of them chenoscenal abnormalities. CONCLUSION: We recommend that fetuses with congenital extracardiac anomalies sbould be checked for the presence of congenital heart disease and chmmosomal abnormalities, if needed.
Central Nervous System ; Echocardiography ; Female ; Fetus ; Follow-Up Studies ; Heart Defects, Congenital* ; Humans ; Incidence

BACKGROUND AND OBJECTIVES: Although many cochlear implant (CI) recipients receive significant benefits from using the implants, speech perception ability is still known to vary among CI users, and the variability is closely related to the ability of users to process temporal information. As one of the important temporal cues for speech perception in CI users, voice onset time (VOT) can be used to examine behavioral perception and neural correlates of temporal processing. In this study, we measured categorical perception and N1-P2 cortical auditory evoked potentials (CAEPs) using a /ba/-/pa/ VOT continuum in CI users as well as in normal-hearing (NH) listeners.SUBJECTS AND METHOD: Ten CI subjects and 11 NH controls participated in syllable identification tasks and in electroencephalography recording. CI subjects were divided into two groups, which were ‘good’ and ‘poor’ performers based on their speech perception scores. The stimuli used in both experiments were a synthetic /ba/-/pa/ VOT continuum. The identification boundary and N1-P2 peak-to-peak amplitude were measured for each subject group. RESULTS: Behavioral results revealed that poor CI performers had a delayed identification boundary compared to NH listeners and good CI performers. However, N1-P2 peak-to-peak amplitudes in both good and poor CI performers were significantly smaller than those in NH group. CONCLUSION N1-P2 amplitudes reflected decreased auditory cortical activity of temporal cues in CI users. The categorical perception of good CI performers was similar to that of NH listeners, whereas their cortical responses were decreased to the level similar to that of poor CI performers. This finding indicates that CAEPs would be more sensitive to altered temporal processing of CI users than to behavioral measures.

BACKGROUND AND OBJECTIVES: Although many cochlear implant (CI) recipients receive significant benefits from using the implants, speech perception ability is still known to vary among CI users, and the variability is closely related to the ability of users to process temporal information. As one of the important temporal cues for speech perception in CI users, voice onset time (VOT) can be used to examine behavioral perception and neural correlates of temporal processing. In this study, we measured categorical perception and N1-P2 cortical auditory evoked potentials (CAEPs) using a /ba/-/pa/ VOT continuum in CI users as well as in normal-hearing (NH) listeners. SUBJECTS AND METHOD: Ten CI subjects and 11 NH controls participated in syllable identification tasks and in electroencephalography recording. CI subjects were divided into two groups, which were ‘good’ and ‘poor’ performers based on their speech perception scores. The stimuli used in both experiments were a synthetic /ba/-/pa/ VOT continuum. The identification boundary and N1-P2 peak-to-peak amplitude were measured for each subject group. RESULTS: Behavioral results revealed that poor CI performers had a delayed identification boundary compared to NH listeners and good CI performers. However, N1-P2 peak-to-peak amplitudes in both good and poor CI performers were significantly smaller than those in NH group. CONCLUSION: N1-P2 amplitudes reflected decreased auditory cortical activity of temporal cues in CI users. The categorical perception of good CI performers was similar to that of NH listeners, whereas their cortical responses were decreased to the level similar to that of poor CI performers. This finding indicates that CAEPs would be more sensitive to altered temporal processing of CI users than to behavioral measures.
Cochlear Implants ; Cues ; Electroencephalography ; Evoked Potentials, Auditory ; Methods ; Speech Perception ; Voice

PURPOSE:Autonomic neuropathy in adult diabetics is known to be common and poor in prognosis. Cardiac autonomic neuropathy increases sudden cardiac death and is present before apparant cardiac symptoms. Subclinical diabetic peripheral neuropathy is common with type 1 diabetes. In children, few studies for subclinical peripheral neuropathy and cardiac autonomic neuropathy were reported but the results were inconsistent. The authors investigated subclinical cardiac and peripheral neuropaties and evaluated correlation between nerve conduction velocity(NCV) and cardiac autonomic function(CAF) test in type 1 diabetic children and adolescents METHODS:Over 5 years from 1997 to 2002, 27 IDDM without clinical neuropathy were registered. Total 57 CAF and NCV were tested every 2 or 3 years. Duration of diabetes, average HbA1c during 1 year prior to the test, and urinary microalbumin excretion for 24 hours were assessed. Diabetic autonomic nervous function was evaluated by cardiovascular reflex test:falling systolic blood pressure in response to standing, heart rate changes in response to standing, beat-to-beat rate variation during deep breathing, RR interval change during and after Valsalva maneuver. Subclinical peripheral neuropathy was evaluated by NCV in both upper and lower extremities. Correlation of parameters according to NCV abnormality and microalbuminuria were analyzed. RESULTS:The age, duration of diabetes and microalbuminuria were not statistically different between children with normal and abnormal NCV. The level of HbA1c was associated with NCV abnormality. In a multivariate logistic model for NCV abnormality, level of HbA1c showed statistical significance after controlling the effects of age, duration of diabetes, and urinary microalbumin level, and the odd ratio was 1.532. The degree of CAF abnormality was marginally significant in the logistic model. Children with microalbuminuria showed older age and longer duration of diabetes than those without microalbuminuria. However, mean HbA1c level was not significantly different between the two groups. Degree of CAF abnormality was not significantly associated with microalbuminuria, either. In a general linear model including urinary albumin level as the dependent variable, none of independent variables was statistically significant. CONCLUSION: Therapy against neuropathy should be considered in patients with high HbA1c and abnormal CAF test even without clinically apparent neuropathy.
Adolescent ; Adult ; Blood Pressure ; Child* ; Death, Sudden, Cardiac ; Diabetes Mellitus, Type 1 ; Diabetic Neuropathies ; Heart Rate ; Humans ; Linear Models ; Logistic Models ; Lower Extremity ; Neural Conduction ; Peripheral Nervous System Diseases ; Prognosis ; Reflex ; Respiration ; Valsalva Maneuver

Acquired tufted angioma is a benign, slowly progressive angioma with a typical histological pattern that was first described by Wilson-Jones in 1976. We report a case of acquired tufted angioma in a 19 year old female who had erythematous papules and plaques on the right thigh. Histopathological findings showed multiple capillary lobules in a cannonball arrangement scattered throughout dermis, which was diagnostic of acquired tufted angioma.
Capillaries ; Dermis ; Female ; Hemangioma* ; Humans ; Thigh ; Young Adult