Since it had not been possible to find any formal report on this disease in Korea, we had chance to examine and to make accurate diagnosis, so that we would like to report 10 calles among 3 families. Throughout the literature, the age of onset, the incidence, the mode of heredity, the etiology, the clinical picture, the prognosis, and the treatment were discussed. The results of this survey are as followings. 1. There were 5 males and 5 females in this material. This sex incidence showed rather similar with Japanese's than European's and American's. 2. As to the incidence of age of onset, Case I and III were between 15-22 years old, but Case II were between 6-8 years old. 3. As to the visual acuity, most patients had from H.M. to 0.1 (20/200), and most patients were who associated with the color disturbance. 4. The ophthalmoscopic finding revealed only total or temporal pallor on the disc, otherwise normal macula and retinal vessels. 5. Visual field changes showed central scotoma in all cases which were examined. 6. Various laboratory examinations, X-ray examinations for skull and orbit, neurological examination, etc., showed no significant pathological finding in cases which were examined. 7. As to the mode of heredity, those three families revealed not a typical sex-linked recessive form, as far as we could observe in two generations of the pedigree.
Age of Onset ; Diagnosis ; Family Characteristics ; Female ; Heredity ; Humans ; Incidence ; Korea ; Male ; Neurologic Examination ; Orbit ; Pallor ; Pedigree ; Prognosis ; Retinal Vessels ; Scotoma ; Skull ; Visual Acuity ; Visual Fields

No Abstract Available.
Alopecia*

The E-cadherin, alpha-catenin, and beta-catenin expressions were immunohistochemically investigated in paraffin-embedded materials of 80 cases of colorectal adenocarcinomas. The staining similar to normal colorectal mucosa with preserved strong membranous staining pattern was considered normal or preserved expression. The X2 test was used to analyse the statistical correlation of cadherin/catenin expression with clinicopathologic parameters and the Breslow test for the correlation with survival length. Normal colorectal mucosa showed strong membranous expression of cadherin/catenin complex. The reduced E-cadherin, alpha-catenin, and beta-catenin expression were found in 53/80 (66.3%), 46/80 (57.5%), and 44/80 (55.5%) cases of colorectal cancers examined, respectively. There were significant correlations between E- cadherin and alpha -catenin (p=0.035), and between alpha-catenin and beta-catenin (p=0.013). The reduced E-cadherin expression was associated with histologic dedifferentiation, tumor depth, lymph node metastasis, clinical stage (p<0.05), poor clinical outcome in stage II (p=0.016) and the reduced alpha-catenin expression with lymph node metastasis and clinical stage (p<0.05). Reduced expression of two or more proteins was correlated with lymph node matastasis, histologic dedifferentiation, clinical stage, and survival (p<0.05). The present study demonstrates a significant down-regulation of E-cadherin and alpha-catenin expression in colorectal cancer is associated with tumor invasiveness, histologic dedifferentiation, lymph node metastasis, and clinical stage. These results suggest that E-cadherin and alpha-catenin may be useful markers of invasiveness, lymph node metastatic potential, and clinical stage and of value as prognostic markers in the earlier stage. Further studies are needed to confirm the prognostic value of these cadherin/catenin complex.
Adenocarcinoma* ; alpha Catenin* ; beta Catenin* ; Cadherins* ; Colorectal Neoplasms ; Down-Regulation ; Lymph Nodes ; Mucous Membrane ; Neoplasm Metastasis

PURPOSE: To estimate and standardize horizontal ocular movement of normal Korean people and, to adapt correlations among three Methods for clinical approach. METHODS: This study investigated the amounts of horizontal ocular movement in normal Korean people whose age was ranged from 20 to 79 (50 persons in each age decades) by the lateral version light reflex Method, the limbus test of motility and the imaginary vertical line Method, and compared relationships among the amounts measured with above three Methods by Spearman correlation coefficient. The study subjects were three hundred people who visited Dept. of Ophthalmology at Gyeongsang National University Hospital, whose best corrected visual acuity was no less than 20/50 Snellen visual acuity chart, who had no history of ophthalmic operation and diseases, whose age was ranged from 20 years to 79 years (50 persons in each age decades). RESULTS: In imaginary vertical line Method, the excessive ocular movements were more frequent in younger people than in elder people. With age the proportion of the excessive ocular movement was gradually decreased and that of the normal ocular movement was increased until 6th decades. With other two Methods, the amounts of horizontal ocular movement was higher in younger ages than in elder ages and they decreased with aging. There were significantly moderate correlation (rs = - 0.54 to - 0.64) between age and ocular movement, and high correlation (rs = 0.79 to 0.86) among ocular amounts measured with imaginary vertical line Method, the lateral version light reflex test, and the limbus test of motility of Kestenbaum. CONCLUSIONS: This study suggests that any Method of three is reliable clinically.
Aging ; Humans ; Ophthalmology ; Reflex ; Visual Acuity

The surgical release of flexor tendons from their restricting adhesions has historically been a somewhat controversial procedure. Especially clinical efficacy of tenolysis is dependent on early active digital motion. The administration of long acting anesthetics (Bupivacaine) through indwelling catheter after tenolysis relieved pain and so achieved early active digital motion in 12 cases. The times for tenolysis following tendon repair and the followup period were 3 months and 6 months on an average. According to flexor zones classification, in 4 patients the lesion was in zone II, 5 in zone III, 1 in zone IV and 2 in zone V. The results were as follows; 1. The functional results after tenolysis showed up 7 excellent, 4 good and 1 fair. 2. The subjective results of the postoperative pain relief showed up 8 excellent and 4 good out of 12 cases.
Anesthetics ; Catheters, Indwelling ; Classification ; Follow-Up Studies ; Humans ; Pain, Postoperative ; Tendons ; Treatment Outcome

N,N-Diethylnitrosamine (DEN) has been proved to have carcinogenic potential in the initiation or promotion stage and the transformed cells proliferate to form preneoplastic nodules which are positive for placental form of glutathione S-transferase (GST-P). E-Cadherin, a member of the cadherin family, is expressed in epithelial cells. To evaluate the role of adhesion molecules (E-Cadherin, alpha-catenin, and beta-catenin), which have not been well understood in carcinogenesis, we investigated the changes of E-cadherin, alpha-Catenin and beta-Catenins by immunohistochemistry and immunoblotting in DEN-induced hepatocarcinogenesis of rat liver. In addition, the sequential analysis of histopathology and the expression of GST-P were also examined. Immunoreactive areas for GST-P were gradually increased from early period of carcinogenesis and strong GST-P positive foci were noted in various lesions, especially in the clear cell and eosinophilic cell nodules. Immunohistochemically, the E-Cadherin expression was increased in DEN-treated preneoplastic nodules in 4 and 10 weeks and hepatocellular carcinomas displayed relatively reduced expression compared with the hyperplastic nodules. But alpha- and beta-catenin expression was increased in hyperplastic nodules and hepatocellular carcinomas. Immunoblotting studies revealed that the level of alpha-catenin (cytosol and membranous fraction) was overexpressed in hyperplastic nodules as well as hepatocellular carcinomas, which showed markedly increased expression. The membranous fraction of beta-catenin was markedly increased in 10 weeks of DEN treatment and slightly reduced in hepatocellular carcinomas. These findings suggest that during DEN-induced hepatocarcinogenesis, the clear cell and eosinophilic cell nodules expressing GST-P in their cytoplasm are early transformed cell nodules. The altered expression of E-Cadherin and catenins is closely related with tumor propagation. Loss or reduced expression of E-cadherin may play a role in the progression of late hyperplastic nodule to hepatocellular carcinoma in DEN-induced rat hepato carcinogenesis.
alpha Catenin ; Animals ; beta Catenin ; Cadherins* ; Carcinogenesis ; Carcinoma, Hepatocellular ; Catenins* ; Cytoplasm ; Eosinophils ; Epithelial Cells ; Glutathione Transferase* ; Glutathione* ; Humans ; Immunoblotting ; Immunohistochemistry ; Liver* ; Rats*

PURPOSE: MDM-2 is an oncoprotein that inhibits p53 tumor-suppressor protein. These abnor malities have a role in tumorigenesis through inactivation of p53 function. To determine the clini copathological and prognostic value of MDM2 abnormalities in gastric adendegrees Carcinoma, MDM-2& p53 protein expression were analysed in surgically resected materials of gastric adendegrees Carcinoma. MATERIALS AND METHODS: Fifty cases which had got follow-up after surgical resection were immunohistdegrees Chemically studied with p53 and MDM-2 antibodies. We defined variable clinico pathologic factors for expression of p53 and MDM-2 protein and analysed their relationships. RESULTS: Immunohistdegrees Chemical stain revealed expression of MDM-2 protein as a 52.0% (26/50) and p53 protein 20.0% (10/50), respectively. But their expressions were not assdegrees Ciated with clinicopathological factors such as T-factor, N-factor, stage, histology and differentiation. Overall, p53-negative patients seemed to have a better prognosis regardless of MDM-2 protein status (P= 0.057). MDM-2 protein status was considered to have no play as a prognostic factor. CONCLUSION: In the gastric adendegrees Carcinoma, p53 protein expression seemed to have a inverse relationship with clinical outcomes but MDM-2 protein expression, which was observed more frequently than those of p53, seemed not to be prognostic indicator.
Antibodies ; Carcinogenesis ; Follow-Up Studies ; Humans ; Prognosis ; Proto-Oncogene Proteins c-mdm2 ; Stomach Neoplasms

Small follicular cysts are common findings in the ovaries of prepubertal girls, and in most cases, they are of no clinical importance. Ocassionally these cysts may enlarge and continue to produce estrogen, resulting in signs of precocious sexual development and vaginal bleeding. We have experienced a case of a precocious pseudopuberty causing ovarian follicular cyst which was treated by exploratory laparotomy. we present this case with a brief review of literatures
Estrogens ; Female ; Follicular Cyst* ; Humans ; Laparotomy ; Ovary ; Sexual Development ; Uterine Hemorrhage

We report a case of multiple Bowen's disease of probable arsenic origin associated with internal malignancies. A 63-year-old man who had a history of arsenicals injections showed pruritic erythematous 11 8 cm sized psoriasiform plaque on the right flank and multiple keratotic plaques on the trunk and both extremities. He had operation on his bladder due to transitional cell carcinoma. He was found to have a bronchogenic carcinoma by chest X-ray and CT scan. We report this as an interesting case of atypical multiple Bowen's disease manifestating a psoriasiform large plaque associated with internal malignancies of lung and bladder.
Arsenic ; Arsenicals ; Bowen's Disease* ; Carcinoma, Bronchogenic ; Carcinoma, Transitional Cell ; Extremities ; Humans ; Lung ; Middle Aged ; Thorax ; Tomography, X-Ray Computed ; Urinary Bladder

Primary amenorrhea due to intrasella arachnoid cyst is a very rare disease and require careful and frequent evaluation because may produce intracranial hemorrhage, elevated intracranial pressure and rapid expansion. Surgical intervention is needed only when visual disturbance, hypopituitarism or enlarging lesion is shown. Thus, we present a case of primary amenorrhea due to intrasella arachnoid cyst which was resected through the transsphenoidal approach.
Amenorrhea ; Arachnoid* ; Female ; Hypogonadism* ; Hypopituitarism ; Intracranial Hemorrhages ; Intracranial Hypertension ; Rare Diseases