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Takayasu's arteritis(TA) is a nonspecific areritis of unknown etiology affecting segmentally the aorta and its main branches, which result in stenosis, occlusion or aneurysm of involved arteries. The clinical manifestations present with a variety of symptoms such as headache, dyspnea on exertion, pain and weakness of extremities, pulse deficit, and hypertension according to involves arteries. Usually it can be managed by medical or surgical treatment, and recently by percutaneous transluminal balloon angioplasty. The type III classified by Lupi-Herrena and associates is the most frequent variety of TA. However the case of type III involving both subclavin arteries and both renal arteries has rarely been reported. We experienced a case of TA involving both subcalvian arteries, and both renal arteries presented with paroxysmal hypertension and right flank pain, in which the stenosis of both subclavian arteries were managed by percutaneous transluminal balloon angioplasty and the stenosis and occlusion of both renal arteries were successfully managed by aorto-renal bypass surgery with autogenous right iliac artery and synthetic vessel(Gortex). The patient was discharged uneventfully.
Aneurysm ; Angioplasty, Balloon ; Aorta ; Arteries* ; Constriction, Pathologic* ; Dyspnea ; Extremities ; Flank Pain ; Headache ; Humans ; Hypertension ; Iliac Artery ; Renal Artery* ; Subclavian Artery ; Takayasu Arteritis*

The enzyme complex 3beta-hydroxysteroid dehydrogenase (3beta-HSD) is involed in the biosynthesis of all classes of active steroids. It has been known that the enzymatic activity of 3beta-HSD is present not only in the classical steroido-genic tissues, but also in many peripheral tissues. This study was performed to investigated of 3beta-HSD immunore-activity in the rat cardiovascular tissues such as the ventricle, atrium, aortic arch, and abdominal aorta. Immunoblot analyses and immunohistochemical studies were performed using polyclonal antibodies raised against purified human placental 3beta-HSD. We identified 43 and 37KDa bands in the ventricle and atrum, whereas only 37KDa band was observed in the aortic arch and abdominal aorta. Immunostaining for 3beta-HSD was detected in the ventricular and atrial cardiocytes. The intensity of staining was much higher in the atrial cardiocytes than in the ventricular cardiocytes. Immunostaining was also found in the smooth muscles of aortic arch and abdominal aorta.
Animals ; Antibodies ; Aorta, Abdominal ; Aorta, Thoracic ; Cardiovascular System* ; Humans ; Muscle, Smooth ; Oxidoreductases ; Rats* ; Steroids

Transitional cell carcinoma of the ovary is extremely rare tumor and the category of transitional cell carcinoma has been proposed for those tumors in which definite urothelial features are present but no benign, metaplastic, and/or proliferating Brenner tumor is identified. We report a case of primary transitonal cell carcinoma of the ovary in 48-year old woman who presented with low abdominal pain and dyspepsia. Brief literature was reviewed about the features of its behavioral aggressiveness and characteristic chemosensitivity.
Abdominal Pain ; Brenner Tumor ; Carcinoma, Transitional Cell* ; Dyspepsia ; Female ; Humans ; Middle Aged ; Ovary*

Cowden syndrome is very rare clinical condition with characteristic mucocutaneous lesions associated with abnormalities of the breast, thyroid, and gastrointestinal tract. Here, the case of a 16-year-old girl with diverse gastrointestinal polyposis with breast and thyroid lesions is reported as a definite case of Cowden syndrome. During follow up for 4 years, changes in the characteristic lesions were observed; Esophageal acanthosis and oropharyngeal polyps were newly developed. Gastric, duodenal, rectal and ileal polyps were noted at the first visit when she was 12 years of age and revealed histologically hyperplastic polyps. Mucocutaneous lesions, the pathognomonic finding of Cowden syndrome, were not noted at the first visit or during the follow up period. Breast and thyroid masses were noted at the first visit. Breast aspiration showed highly cellular ductal epithelial fragments and fibroblastic stromal cell fragments, and a thyroid biopsy shows proliferation of irregular sized follicles, with variable colloid contents. Macrocephaly was also noted at the first visit. The characteristic lesions of Cowden syndrome change with the patient's age, and the long-term follow up is recommended in cases suspected with this syndrome.
Adolescent ; Biopsy ; Breast ; Colloids ; Female ; Fibroblasts ; Follow-Up Studies* ; Gastrointestinal Tract ; Hamartoma Syndrome, Multiple* ; Humans ; Macrocephaly ; Polyps ; Stromal Cells ; Thyroid Gland

PURPOSE: Phyllodes tumor is a rare fibroepithelial tumor of the breast, first described by Johannes Muller in 1838. Much has been written about phyllodes tumor, but very few widely accepted conclusions about its clinical behavior, treatment modality, and prognosis have been reached. This study aims to analyze the clinical, radiological, and pathological characteristics of phyllodes tumor of the breast. METHODS: The medical records of 41 patients with phyllodes tumor who had been treated between February 1982 and August 1998 at the Department of Surgery, Seoul National University Hospital, were retrospectively reviewed for clinical, radiological and pathological findings, treatment modalities, and follow-up results. RESULTS: Of these 41 cases, there were 28 cases (68.3%) of benign tumors and 13 cases (31.7%) of malignanat tumors. All patients were females, and the mean ages of onset were 33.2 years for benign tumors and 40.8 years for malignant tumors. Most patients, 100% of benign and 92.3% of malignant, presented with a papable mass in the breast. The median duration of illness was 2 months for malignant tumors and 8 months for benign tumors. The tumor size was greater than 10 cm in diameter in 5 cases (38.5%) of malignant tumors and in 3 cases (10.7%) of benign tumors. Only 4 cases were preoperatively diagnosed as having a phyllodes tumor by using radiological and fine needle aspiration cytology. Out of the 10 malignant cases reviewed, 5 cases were confirmed as malignant, and 5 cases were confirmed as borderline phyllodes tumors. Cellularity was moderate or above in all 5 malignant and 5 borderline cases. Atypism above moderate degree was found in 4 of 5 (80%) malignant tumors, in 3 of 5 (60%) borderline tumors, and in 4 of 24 (16.7%) benign tumors. Mitotic counts in all 5 malignant cases were 5 or more per 10 high power field while those in the 5 borderline tumors were 2-5 mitoses per 10 high power field. The most commonly performed operative procedures were a simple mastectomy (50%) for malignant tumors and a simple excision (64.3%) for benign tumors. Post-operative adjuvant therapy was done for 4 cases; out of these, 1 case had been initially diagnosed as a malignant phyllodes tumor, but the diagnosis was changed to a benign phyllodes tumor upon review. Of the 27 follow-up cases, recurrences developed in 3 cases (16.7%) of benign tumors. CONCLUSION: From the above results, there were no specific clinical features for differentiating benign from malignant phyllodes tumor preoperatively; therefore, we cannot help depending on the pathologic findings. Pathologic reviews showed that among several criteria, atypism, cellularity, and mitotic count were the most definite pathologic characteristics in differentiating benign from malignant phyllodes tumor. But much more experience and long-term follow-up may be needed to define optimal treatments and to analyze the prognosis for phyllodes tumors of the breast.
Biopsy, Fine-Needle ; Breast* ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Mastectomy, Simple ; Medical Records ; Mitosis ; Phyllodes Tumor* ; Prognosis ; Recurrence ; Retrospective Studies ; Seoul ; Surgical Procedures, Operative

Steroid 21 hydroxylase deficiency is a major cause of congenital adrenal hyperplasia(CAH) and is caused by genetic impairment (CYP21B) of this enzyme. In the human genome, CYP21B is located within MHC class III region on the short arm of chromosome 6. Most of the genes in this region are highly polymorphic and crowded. Also the CYP21B gene is accompanied by its pseudogene (CYP21A) and tandemly arranged with two genes of fourth component of complement. This highly complex gene arrangement in this area may predispose genetic unstability of CYP21 genes,i.e. mutations. In the current study, we tried to investigate the frequency of duplication and deletion of CYP21 genes and pattern of the genetic alteration of these genes by RFLPs. We also compared the genetic alteration of CYP21 in normal subjects with those of the CAH patients. According to our study, 15% of the normal Korean population have duplication or deletion of CYP21. There was one normal subject with heterozygous deletion of CYP21B gene. Of the 5 CAH patients examined, we found abnormal patterns in 2 patients. One was a large scale gene conversion and the other was a deletion of CYP21B and C4 locus II genes with gene conversion. These results suggest that high frequency of duplication and deletion of CYP21 and C4 in the general population may provide the genetic pool of instable CYP21 genes and these duplicated or deleted genes may result in gene conversions between CYP21A(pseudogene) and CYP21B(true gene) by preventing the normal recombination event.
Adrenogenital Syndrome* ; Arm ; Chromosomes, Human, Pair 6 ; Complement System Proteins ; Gene Conversion ; Gene Order ; Genome, Human ; Humans ; Polymorphism, Restriction Fragment Length ; Pseudogenes ; Recombination, Genetic ; Steroid 21-Hydroxylase*

PURPOSE: The aim of this study was to identify the clinical features and natural history of dietary protein induced proctocolitis (DPIPC) and to detect the causative foods of DPIPC, and to evaluate the effect of elimination of the foods on the course of the disease. METHODS: Between March 2003 and July 2004, data from 30 consecutive patients with DPIPC who were followed for over 6 months, was reviewed. The diagnostic criterion used for DPIPC was an increase in the number of eosinophils in the lamina propria (> or =60 per 10 high-power fields). In breast feeding mothers, 5 highly allergenic foods were eliminated from the maternal diet for 7 days, namely, allergenic food groups such as dairy products, eggs, nuts and soybean, fish and shellfish, and wheat and buckwheat. We observed the disappearance or appearance of hematochezia after elimination or challenge with the offending foods. RESULTS: Before diagnosis infants were breast-fed (93.3%) or formula-fed (6.7%). Mean age at symptom onset was 11.5+/-5.1 (5~24) weeks, and mean age at diagnosis was 17.8+/-9.5 (8~56) weeks. Duration from symptom onset to diagnosis was 6.3+/-6.7 (0~36) weeks. Mean peripheral blood eosinophil count was 478+/-320 (40~1,790)/mm3 and eosinophilia (>250/mm3) was observedin 90.0% of patients. None of patients were found to have an increased serum IgE level. Of 15 patients that received sigmoidoscopy, nodular hyperplasia with erosion was observed in 93.3%. Of 27 patients whose mother ate the diet eliminated the 5 food groups, hematochezia diappeared in 74.1% of patients. Offending foods were identified as dairy products (37.5%), wheat and buckwheat (27.5%), fish and shellfish (20.0%), nuts and soybean (7.5%) and eggs (7.5%). A free maternal diet without patient's clinical symptoms was achieved at 29.4+/-8.7 (9~44) weeks of patient's age, and a free baby diet without blood in stools was achieved at 37.5+/-9.7 (12~56) weeks of age. CONCLUSION: DPIPC commonly occurs in exclusively breast-fed babies. Elimination of the above-mentioned 5 hyper-allergenic food groups from the maternal diet for 7days enables the detection of the offending foods. DPIPC is a transient disorder and 96.0% of patients can tolerate the offending foods at 12 months of age.
Breast Feeding ; Dairy Products ; Diagnosis ; Diet* ; Dietary Proteins* ; Eggs ; Eosinophilia ; Eosinophils ; Fagopyrum ; Gastrointestinal Hemorrhage ; Humans ; Hyperplasia ; Immunoglobulin E ; Infant ; Mothers ; Mucous Membrane ; Natural History* ; Nuts ; Ovum ; Proctocolitis* ; Shellfish ; Sigmoidoscopy ; Soybeans ; Triticum

PURPOSE: Although papillary thyroid carcinomas are known to have a good prognosis, invasive papillary thyroid carcinomas have different outcomes. There are many studies on the prognostic factors for thyroid carcinomas, but few studies have been performed for invasive papillary thyroid carcinomas. We performed this study to investigate the prognosis and the prognostic factors for invasive papillary thyroid carcinomas. METHODS: We analyzed 184 patients with papillary thyroid carcinomas who had undergone a thyroidectomy between 1985 and 1990, especially for the clinicopathologic entity of an invasive papillary thyroid carcinoma, and made univariate and multivariate analyses for various clinical and pathological factors to evaluate whether they would be of value in estimating the prognosis in papillary thyroid carcinoma patients. RESULTS: In the univariate analysis of the 10-year disease-free survival rate of the overall cases, sex, tumor size, and lymph-node metastasis were confirmed to be significant prognostic factors. In the multivariate analysis, all of these factors were independent significant prognostic factors. Invasive papillary thyroid carcinoma patients, compared to non-invasive papillary thyroid carcinoma patients, were older and more prone to metastasis to cervical lymph nodes. In the univariate analysis of the 10-year disease-free survival rate of patients with an invasive papillary thyroid carcinoma, sex, tumor size, and lymph-node metastasis were significant prognostic factors. However, in the multivariate analysis, sex and tumor size appeared to be independent significant prognostic factors. CONCLUSION: We conclude that invasive papillary thyroid carcinomas have a the tendency to occur in elderly patients and are more prone to metastasis to cervical lymph nodes. The male sex, a tumor size more than 5 cm, and positive cervical-lymph-node metastasis present the worse prognosis. However, a randomized prospective study may be needed to better understand how to manage invasive papillary thyroid carcinomas.
Aged ; Disease-Free Survival ; Humans ; Lymph Nodes ; Male ; Multivariate Analysis ; Neoplasm Metastasis ; Prognosis ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy

We recently experienced a case of endometrial cancer 5 years after the diagnosis of breast cancer in a patient with a mutation in the BRCA2 gene. A 55-year-old Korean woman who had a past history of breast cancer in her 50s underwent an operation for endometrial cancer. Final pathology confirmed stage Ia, and no adjuvant treatment was performed. After surgery, considering her history of sequential cancer occurrence, genetic counseling was offered. The result showed the BRCA2 variation of unknown significance mutation. This is the first case report of sequential cancers (endometrial and breast) in a patient with a BRCA2 mutation among a Korean population.
Breast Neoplasms* ; Diagnosis ; Endometrial Neoplasms* ; Female ; Genes, BRCA2 ; Genetic Counseling ; Humans ; Middle Aged ; Pathology