We present a case of generalized congenital ichthyosiform dermatosis associated with neurosensory hearing loss, neovascularization of corneas, partial alopecia and abnormalities of nails and teeth on a 26 years old female patient. This case is strictly similar to the previously reported cases. The combination of these abnormalities was stuggested to be a new syndrome by some authors. This case is the first reported case in Korea, although in the literature we could find several cascs with similar patterns of malformations.
Adult ; Alopecia ; Cornea ; Deafness* ; Female ; Hearing Loss ; Humans ; Keratitis* ; Korea ; Skin Diseases* ; Tooth

No abstract available.
Ear, Middle*

This study was performed to establish a radiological indication and contraindication of indirect decompression in the thoracolumbar burst fracture as well as to clarify an acceptable degree of the canal constriction to enhance neurologic recovery and to prevent the spinal stenosis. The canal diameter ratio (CDR) of the constricted level was determined using pre-and post-operative CAT images of 31 thoracolumbar burst fractures, decompressed indirectly. The acceptable CDR was decided by the lower limit of the 95% confidence interval of the post-operative CDR in cases without neurologic deficit associated with the canal constriction. A radiological indication and contraindication of indirect decompression was establish based on the calculation of the discriminant equation and linear regression equation respectively. The results were as followings. 1. The acceptable CDR was 46% at the cauda equina level and 37% at the conus medullaris level. 2. According to the result of calculation of the regression equation, the radiological contraindication of indirect decompression was the pre-operative CDR of 13% or less at the cauda equina level and 27% or less at the conus medullaris level. In conclusion, the radiological indication of indirect decompression was the pre-operative CDR of 34% at the cauda eqbina level and 42% at the conus medullaris.
Animals ; Cats ; Cauda Equina ; Constriction ; Conus Snail ; Decompression* ; Linear Models ; Neurologic Manifestations ; Spinal Stenosis

No abstract available.
Humans ; Infant, Newborn* ; Mothers* ; Thrombocytopenia

Nevus Iipomatosus cutaneous superficialis (NLCS) is a very rare skin disease which usually is present at birth. The lesion consists of grouped papules and nodules which on histological examination show the presence of ectopic fatty tissue in the corium. The authors observed a 17year old female with NLCS. She noticed soft papules and nodules on the coccygeal area since 8 years old. We confirmed this case by clinically and histopathological findings which showed the strands or islands of mature fatty tissue within papillay, mid and lower dermis.
Adipose Tissue ; Child ; Dermis ; Female ; Humans ; Islands ; Nevus* ; Parturition ; Skin Diseases

Adenomyoepithelioma is a rare benign tumor which occurs mainly in the skin, salivary gland and very rarely in the breast, in case of malignant adenomyoepithelioma of the breast is a rare lesion characterized by malignant proliferation of epithelial and myoepithelial cells that show characteristic histology and immunohistochemical features. We report two cases of adenomyoepithelioma, one was benign occuring in the outer upper quadrant of the right breast (48-year-old female), and the other was malignant adenomyoepithelioma occuring in the peri-areolar of the left breast (46-years-old female). We confirmed with both H&E stain and immunohistochemistry. We report benign and malignant adenomyoepithelioma of the breast occurred in middle aged women.
Adenomyoepithelioma* ; Breast* ; Female ; Humans ; Immunohistochemistry ; Middle Aged ; Salivary Glands ; Skin

Shy-Drager syndrome is a multisystem degenerative disorder of the central nervous system in which progressive autonomic failure such as orthostatic hypotension, urinary or fecal incontinence, impotence and anhidrosis is a main feature. The nonautonomic neurological abnormalities include parkinsonia, cerebellar and pyramidal signs. In 1984 we met 3 such cases at Seoul National University Hospital and present them here with a brief review of literatures.
Central Nervous System ; Erectile Dysfunction ; Fecal Incontinence ; Hypohidrosis ; Hypotension, Orthostatic ; Male ; Seoul ; Shy-Drager Syndrome*

No abstract available.
Antibodies* ; Chungcheongnam-do* ; Diagnosis* ; Immunoglobulin G* ; Immunoglobulin M* ; Orientia tsutsugamushi* ; Rickettsia* ; Scrub Typhus*

No abstract available.
Antibodies* ; Chungcheongnam-do* ; Diagnosis* ; Immunoglobulin G* ; Immunoglobulin M* ; Orientia tsutsugamushi* ; Rickettsia* ; Scrub Typhus*

No abstract available.
Hand* ; Humans