Richer's syndrome is a development of a high grade malignant lymphoma in a patient with preexisting chronic 1ymphocytic leukemia, small lymphocytic lymphoma or Waldenstrom's macroglobulinemia. A rare case of Richer's syndrome arising in the spleen of a 35-year-old-man was studied by morphology, immunohistochemistry and gene rearrangement study. He has had weight loss and night sweat for last 6 months. Hepatosplenomegaly and abdominal lymphadenopathy were noted on CT scanning. Especially an ovoid radiolucent mass was found within the image of splenomegaly. Lymph nodes and liver biopsy, bone marrow aspiration and splenectomy were done. In the lymph nodes, liver and bone marrow, well differentiated small lymphocytic infiltrations were found but, in the spleen, pleomorphic, large cells with occasional multinucleated giant cells formed a nodular mass surrounded by diffuse, extensive infiltration of small well differentiated lymphocytes. The two distinctive areas in the spleen had positive staining for B-cell marker (HLA-DR and L26), negative staining for T-cell marker (UCLH1), and positive staining for IgM heavy chain and kappa light chain by immuohistochemical study. so this case was diagnosed as a diffuse large cell 1ymphoma transformed from small lymphocytic lymphoma. We made an another effort to clarify their clonality. Gene rearrangement method usingcomplementarity.determining region 3(CDR3) of immunoglobulin heavy chain (IgH) gene and T-cell receptor gamma (TCRgamma) gene by polymerase chain reaction (PCR) technique was done. The two lymphomas in the spleen demonstrated the same rearrangement pattern in both IgH and TCRgamma gene. We think these findings strongly suggest that the large cell lymphoma has the same clonality with that of the small lymphocytic lymphoma.
Male ; Humans

No abstract available.
Cartilage* ; Fascia* ; Transplants* ; Tympanoplasty*

BACKGROUND: Carcinoembryonic antigen (CEA) is a glycoprotein on cellular surface, which is highly condensed in embryonic tissue and tumor of various kinds. Previous study found out that CEA may grow with various cancer or other diseases other than cancer as well. Besides, it is widely known that smoking also influences the rise in CEA. Among the same smokers, some of them show high CEA figures in serum when others remain in normal range. There are those whose pulmonary function is not influenced by smoking when that of others are susceptible to it. Therefore, this study was undertaken with an aim to study the relationship between serum CEA and pulmonary function by investigating how the change in pulmonary function caused by smoking influences serum CEA. METHODS: From Nov, 1997 to Feb, 2001, this study carried out tests on adult male smokers ages 35 to 64 who visited a hospital located in Kang Nung city. The subjects were divided into two groups: one group of 29 subjects with high CEA with over 6.0 ng/ml with normal colon study; the other group, which is the CEA normal group, consisted of 58 subjects selected through age adjusted random sampling. Data on personal information, smoking and clinical history was collected from a questionnaire. CEA was tested using radioimmunoassay of Abott. Pulmonary function was examined using Analyzer assembly Vmax 20C from Sensormedics Company. These examinations was limited to those who have been screened not to have cancer by chest X-ray, abdominal ultrasonography, and duodenofibroscopy. RESULTS: Smoking per day for the group with high serum CEA was 1.3 pack ( 0.4 pack), which was found to be significantly higher compared to that of normal group (P<0.01). Pack-years with high serum CEA group was 32.6 13.5 which was also comparatively higher than that of the normal group with 22.4 10.9 (P<0.01). Pulmonary function test indicated that FEV1 for the group with high serum CEA was 3.0 0.5 L, which marked lower than that of the normal group with 3.4 0.5 L (P<0.05). After compensating for age and pack years, FEV1 decreased in proportion to the rise in CEA. CONCLUSION: This study has established a link between serum CEA and daily smoking, pack years, and pulmonary function and found that FEV1 was inversely proportionate to the rise in CEA regardless of corrected pack years and daily smoking. Consequently, serum CEA alone is thought to be related to the pulmonary function. Therefore, it is advised that smokers with high serum CEA need to take heed of the influence on pulmonary function.
Adult ; Carcinoembryonic Antigen* ; Colon ; Glycoproteins ; Humans ; Male* ; Radioimmunoassay ; Reference Values ; Respiratory Function Tests ; Smoke ; Smoking ; Thorax ; Ultrasonography ; Surveys and Questionnaires

PURPOSE: The purpose of this study was to describe technological development, caring attributes and professional self-concept as perceived by nurses in YanBian. METHOD: Data were collected using an instrument containing 137 Likert items was administered to 477 RN's working in general hospitals in YanBian. The instrument contained sections which examined technological influences questionnaire(TIQ), caring attributes questionnaire(CAQ), and professional self-concept nursing inventory(PSCNI). RESULT: Descriptive and inferential statistics revealed by marital status and position. Married, working special ward nurses reported a higher TIQ score than that of unmarried and working general ward and OPD. PSCNI and CAQ score of head or supervisor nurses were higher than that of staff nurses. Subjects revealed very low score of CAQ, while PSCNI score was higher than that of other Asian countries such as Korea, Beijing China, HongKong China and Japan as proved in former study. CONCLUSION: Useful information for educators and nurse administrators is provided from this results. Further study needs to be done to discuss in the light of cultural and environmental differences between YanBian(Korean-Chinese) and Korean nurses.

Antihypertensive effect of angiotensin converting enzyme(ACE) inhibitor Captopril was studied in 34 cases of essential hypertension. A single oral dose of 50mg Captopril was administered daily and blood pressure was followed every 2 weeks. Diuretics were added to patients who responded inadequately after 2 weeks of Captopril single treatment. Alpha-blocker, beta-blocker or calcium channel-blocker was added to patients who responded inadequately after another 2 weeks of Captopril and diuretics combined treatment. In 5 cases, Captopril was raised to 100mg and further antihypertensives were added to unresponded 3 cases. The resuts were as follows; 1) In 15 patients, blood pressure dropped from 170.3+/-10.5mmHg/108.7+/-6.1mmHg to 148.3+/-4.4mmHg/93.3+/-3.7mmHg after 8 weeks of Captopril 50mg single therapy. 2) Hydrochlorothiazide 25mg was added to non-responders, and blood pressure dropped from 180+/-6.7mmHg/111.1+/-6.2mmHg to 155.0+/-15.0mmHg/106.2+/-8.7mmHg in 9 of 19 patients after 8 weeks of combined treatment. 3) Alpha-blocker, Beta-blocker or calcium channel blocker was added to 10 non-responders to Captopril-hyprochlorothiazide combination therapy, and blood pressure dropped from 189.0+/-27mmHg/116+/-10mmHg to 137.8+/-15.5mmHg/88.5+/-10.2mmHg after 8 weeks. 4) Increase of captopril from 50mg to 100mg in 5 random nonresponder cases of Captopril single treatment lowered blood pressure from 168.0+/-13.6mmHg/107.1+/-6.4mmHg to 161+/-15.2mmHg/99+/-8.8mmHg after 2 weeks. 5) Heart rate, and serum creatinine, electrolytes and lipid levels showed no significant interval change. 6) Six patients complained of dry cough and one patient complained of poor appetite but no other clinically significant complications were noted during Captopril treatment.
Angiotensins ; Antihypertensive Agents ; Appetite ; Blood Pressure ; Calcium ; Calcium Channels ; Captopril* ; Cough ; Creatinine ; Diuretics ; Electrolytes ; Heart Rate ; Humans ; Hydrochlorothiazide ; Hypertension

Purpose: To evaluate the findings and frequencies of retinal microvascular abnormalities observed in patients with type 1 neurofibromatosis. Methods: Fundus photographs of 61 patients with type 1 neurofibromatosis and 61 controls without systemic disease or ophthalmic abnormalities were retrospectively compared and analyzed. The presence or absence of retinal microvascular abnormalities in the form of simple vascular tortuosity, corkscrew retinal vessels, and moyamoya-like patterns was confirmed, and the diagnostic sensitivity, diagnostic specificity, positive predictive value, negative predictive value, and diagnostic accuracy for type 1 neurofibromatosis were analyzed. Results: Retinal microvascular abnormalities were found in 19.7% (12 patients) of the patient group, There was no cases in the control group, thus. The difference between the patient group and the control group was significant (p = 0.0003). Of the 12 patients with abnormalities, 10 exhibited simple vascular tortuosity, one had corkscrew retinal vessels, and one exhibited both findings. The diagnostic sensitivity of retinal microvascular abnormalities for type 1 neurofibromatosis was 23.53%, the diagnostic specificity was 100%, the positive predictive value was 100%, and the negative predictive value was 61%. The diagnostic accuracy was 65.18%, which was slightly lower than the 79.5% diagnostic accuracy for the Lisch nodule, but the diagnostic accuracy was comparable to that of neurofibroma (68.03%). Conclusion Retinal microvascular abnormalities were observed in 19.7% of type 1 neurofibromatosis patients, of which simple vascular tortuosity was the most common. Considering that retinal microvascular abnormalities were not observed at all in the control group, and the diagnostic accuracy was 65.18%, this type of abnormality could be included as a new ophthalmic clinical feature of type 1 neurofibromatosis.

Purpose: Dominant optic atrophy is one of the most common hereditary optic neuropathies, causing progressive bilateral vision loss that begins early in life. Optic atrophy 1 (OPA1) gene mutation brings about mitochondrial dysfunction, which results in clinical manifestations of dominant optic atrophy. Here, we report a case of dominant optic atrophy caused by the c.1334G>A mutation of the OPA1 gene, the first known case in Korea to our knowledge.Case summary: A 12-year-old female patient with no specific medical history or systemic symptoms visited our clinic complaining of a progressive decrease in vision in either eye. Slit-lamp microscopy, intraocular pressure, ocular motility, and pupil reflex were normal. However, her best-corrected visual acuity in both eyes was 20/100, and her color vision was reduced to 8/12 in Ishihara’s test. Fundus examination showed temporal pallor of the optic nerve head in both eyes, and a corresponding cecocentral scotoma was observed on Goldmann visual field examination. Optical coherence tomography revealed significant thinning of the peripapillary retinal fiber layer and macular ganglion cell layer in both eyes. Genetic examination confirmed the c.1334G>A mutation of the OPA1 gene. Conclusions We report a case of dominant optic nerve atrophy caused by c.1334G>A mutation of the OPA1 gene and its clinical manifestations.

BACKGROUND: Core needle biopsy (CNB) is widely used as the initial sampling method for breast cancer. And because frozen section (FS) diagnosis is rapid and reliable, we studied the diagnostic agreement between the diagnosis of FS of CNB and final diagnosis after surgery to evaluate the diagnostic accuracy of the FS of CNB. METHODS: Of 409 patients who were preoperatively diagnosed by FS of CNB and who underwent final surgery from 1996 through 2000, 24 cases were found to be ductal carcinoma in situ (DCIS) and 385 cases invasive carcinoma (IC). The diagnoses of FS of CNB were compared with final diagnoses. RESULTS: The diagnostic accuracy of carcinoma is 63.6% for DCIS and 86.9% for invasive carcinoma. Five cases (1.2%) could not be diagnosed because of material insufficiency for diagnosis. Twenty two cases (5.4%) were diagnosed as benign on FS, among which 20 (90.9%) were misdiagnosed by sampling error. Twenty seven cases (6.7%) were deferred on FS, 4 of these cases were DCIS, 5 were invasive lobular carcinoma (ILC), the rest displayed low nuclear grades or marked freezing artifacts. CONCLUSIONS: The diagnostic accuracy of FS of CNB is very high except for cases of ILC and low grade DCIS. Considering the advantage of rapid evaluation, more definitive diagnosis, familiarity by pathologists and availability of ancillary study, FS of CNB is very useful method as the preoperative evaluation.
Animals ; Artifacts ; Biopsy, Large-Core Needle* ; Biopsy, Needle ; Breast Neoplasms* ; Breast* ; Carcinoma, Intraductal, Noninfiltrating ; Carcinoma, Lobular ; Diagnosis ; Freezing ; Frozen Sections* ; Humans ; Mammary Neoplasms, Animal ; Recognition (Psychology) ; Selection Bias

Four cases of primary transitional cell carcinoma (TCC) arising in the ovary (3 cases) and the parovarium (1 case) were collected for clinicopathologic analysis. The mean age was 46.2 years (range, 39-57 years). Two patients complained abdominal discomfort and vaginal discharge, respectively. Other 2 cases were incidentally found from routine check. Grossly, the tumors were solid and cystic (2 cases), solid (1 case) and surface papillary growth on capsule (1 case). Microscopically, the tumor showed almostly same to the histologic features of TCC of urinary bladder. Three cases were pure TCC, and one was mixed TCC and serous carcinoma. FIGO stage were 1 IIa, 2 IIc, and 1 IIIc. Treatment was surgery with adjuvant chemotherapy. Two patients are alive with no evidence of disease, and two have lung or brain metastasis.
Brain ; Carcinoma, Transitional Cell* ; Chemotherapy, Adjuvant ; Female ; Humans ; Lung ; Neoplasm Metastasis ; Ovary ; Urinary Bladder ; Vaginal Discharge

Telomerase is a ribonucleoprotein enzyme that synthesizes telomeric DNA onto the ends of chromosomes. thereby preventing the replication-dependent shortening of these ends. Telomerase activity is detected in a wide range of cancers of various tissues, and its expression may be a critical step in tumor progression. Our objective was to determine if detection of telomerase activity may be an indicator for diagnosis of breast cancer and any association between telomerase activity and prognostic factors of breast cancer. Using a polymerase chain reaction-based telomerase activity assay, we examined telomerase activity in 30 breast cancer specimens (2 ductal carcinoma in situ, 28 invasive ductal carcinoma), 25 benign lesions (14 fibroadenomas, 11 fibrocystic diseases) and 24 normal breast tissues (13 adjacent to malignancy, 11 adjacent to benign lesion). Among surgically resected samples, telomerase activity was detected in 23 (77%) of 30 breast cancers. While telomerase activity was not detected in any of 11 specimens of fibrocystic disease and 11 adjacent normal tissues to benign lesion, surprisingly low levels of telomerase activity were detected in 5 (36%) of 14 fiboadenomas and 1 (7%) of 13 adjacent normal tissues to malignancy. There was no significant difference in expression of telomerase among prognostic factors of breast cancer. In summary, telomerase activity in breast cancer may be useful in diagnosis of breast cancer. We found no correlation between telomerase activity and stage, tumor size or LN status. Mechanisms of telomerase expression are still under investigation; therefore, the significance of telomerase expression in malignant tumors and their progression remains to be determined.
Breast Neoplasms* ; Breast* ; Carcinoma, Intraductal, Noninfiltrating ; Diagnosis ; DNA ; Fibroadenoma ; Humans* ; Ribonucleoproteins ; Telomerase*