Although the definition of male pseudohermaphroditism remains controversial, male pseudohermaphrodites will be defined as chromatin-negative individuals who have testis with the failure of normal development of the normal male. Also included are those individuals who may have more than one cell line, but at least one cell line, containing a Y chromosome and no cell line having two X chromosomes. Gonadal histologic findings may be either testicular or streak, but not ovarian. Male pseudohermaphroditism can result as a consequence of 1) absent M(llerian regression, 2) Inadequate synthesis of testosterone, 3) inadequate synthesis of dihydrotestosterone and 4) androgen receptor deficiency. We have seen 5 cases of male pseudohermaphroditism with manifestations of penoscrotal or perineal hypospadias, pubertal virilization, cryptorchism or atrophied testes, and feminization. Four cases had been reared as male and one case as female. All revealed chromatin-negative pattern in buccal smear and testes. After the plausible discussion, two cases were decided to be reared as female and three were as male.
46, XY Disorders of Sex Development* ; Androgen-Insensitivity Syndrome ; Cell Line ; Cryptorchidism ; Dihydrotestosterone ; Female ; Feminization ; Gonads ; Humans ; Hypospadias ; Male* ; Testis ; Testosterone ; Virilism ; X Chromosome ; Y Chromosome

We report herein a case of smooth muscle hamartoma associated with Beckers nevus in a 5-year-old female. The child presented with a slight.ly brownish. 2 x 3 cm sized, pigmentecl patch with follicular accentuation on the right forearm, witch had been observecl since age of 2. Histopathologic findings showed the epidermal changes most consistent with Becker's nevus and the presence of irregularly arranged hyperplastic smooth muscle hundless in the dermis.
Child ; Child, Preschool ; Dermis ; Female ; Forearm ; Hamartoma* ; Humans ; Muscle, Smooth* ; Nevus*

Pulmonary edema is a rare adverse reaction to the iodinated contrast medium. Complaining of huge abdominal mass, a 52 years-old female was admitted to the Hangang Sungsim Hospital. On physical examination, the patient appeared to be healthy. She had stable vital Signs, i.e. BP: 120/80 mmHg, pulse rate: 80/min. etc. An adult head sized mass was palpated in the left mid and lower abdomen. Otherwise nonspecific. On laboratory studies, the positive findings were 8-10 WBC/HPF in urine, 25.6 mg/dl for BUN and PVC in EKG. It was negative for urine protein, serum creatinine and liver function test. We injected 100ml and 30 ml for Urografin 60 through the abdominal aortadividing 3 times and major branches of the abdominal aorta, respectively. Immediately after completing angiography, interstitial pulmonary edema was found, showing blurring of the vascular margins, perivascular haziness and thickening of the interiobular septal lines in the both lower lung fields. The blood pressure was dropped to 80/60 mmHg, but pulse rate was normal. She did not complian of dyspnea, and cyanosis was not developed. The urine volume was normally maintained. She was treated for pulmonary edema, which was completely absorbed after 20 hours. And the blood pressure was also normalized. We has experienced a case of pulmonary edema developed after intraarterial injection of the iodinated contrast medium without underlying cardiac, renal and hepatic problems, and reviewed the literatures on mechanisms of pulmonary edema caused by intravascular injection of the iodinated contrast materials.
Abdomen ; Adult ; Angiography ; Aorta, Abdominal ; Blood Pressure ; Contrast Media ; Creatinine ; Cyanosis ; Diatrizoate Meglumine ; Dyspnea ; Electrocardiography ; Female ; Head ; Heart Rate ; Humans ; Injections, Intra-Arterial ; Liver Function Tests ; Lung ; Physical Examination ; Pulmonary Edema ; Vital Signs

No abstract available.
Sinusitis*

BACKGROUND: Low levels of serum vitamin D is associated with several lung diseases. The production and activation of matrix metalloproteinases (MMPs) may play an important role in the pathogenesis of emphysema. The aim of the current study therefore is to investigate if vitamin D modulates the expression and activation of MMP-2 and MMP-9 in human lung fibroblasts (HFL-1) cells. METHODS: HFL-1 cells were cast into three-dimensional collagen gels and stimulated with or without interleukin-1beta (IL-1beta) in the presence or absence of 100 nM 25-hydroxyvitamin D (25(OH)D) or 1,25-dihydroxyvitamin D (1,25(OH)2D) for 48 hours. Trypsin was then added into the culture medium in order to activate MMPs. To investigate the activity of MMP-2 and MMP-9, gelatin zymography was performed. The expression of the tissue inhibitor of metalloproteinase (TIMP-1, TIMP-2) was measured by enzyme-linked immunosorbent assay. Expression of MMP-9 mRNA and TIMP-1, TIMP-2 mRNA was quantified by real time reverse transcription polymerase chain reaction. RESULTS: IL-1beta significantly stimulated MMP-9 production and mRNA expression. Trypsin converted latent MMP-2 and MMP-9 into their active forms of MMP-2 (66 kDa) and MMP-9 (82 kDa) within 24 hours. This conversion was significantly inhibited by 25(OH)D (100 nM) and 1,25(OH)2D (100 nM). The expression of MMP-9 mRNA was also significantly inhibited by 25(OH)D and 1,25(OH)2D. CONCLUSION: Vitamin D, 25(OH)D, and 1,25(OH)2D play a role in regulating human lung fibroblast functions in wound repair and tissue remodeling through not only inhibiting IL-1beta stimulated MMP-9 production and conversion to its active form but also inhibiting IL-1beta inhibition on TIMP-1 and TIMP-2 production.
Collagen ; Emphysema ; Enzyme-Linked Immunosorbent Assay ; Fibroblasts* ; Gelatin ; Gels ; Humans ; Interleukin-1beta ; Lung Diseases ; Lung* ; Matrix Metalloproteinase 9 ; Matrix Metalloproteinases ; Polymerase Chain Reaction ; Reverse Transcription ; RNA, Messenger ; Tissue Inhibitor of Metalloproteinase-1 ; Tissue Inhibitor of Metalloproteinase-2 ; Trypsin ; Vitamin D* ; Wounds and Injuries

OBJECTIVES: This study is to investigate clinicopathologic characteristics, survival and prognostic factors in patients with Krukenberg tumor of the ovary. MATERIAL & METHODS: From Jan. 1991 to Dec. 2000, 42 patients with Krukenberg tumor of the ovary were investigated with clinical profiles, such as age, stage, primary sites, clinical symptoms, and survival, retrospectively. RESULTS: A mean age of 42 patients was 44.8 years (range 27-77). Stomach was the most frequent primary site (30/42, 71.4%), followed by colon (7/42, 16.7%) and gallbladder (1/42, 2.4%). In 38 patients, primary sites diagnosed before or after 1 month of diagnosis of Krukenberg tumor of ovary (36/38 cases, 94.7%). The most common feature of patients with Krukenberg tumor of ovary was bilateral abdominal mass. 5-year survival rate of patients with Krukenberg tumor of ovary was 8.94% (95% CI=3.33-14.55) and median survival time was 11 months. Age, bilaterality of tumor, time of diagnosis, presence of ascites and the primary site did not affect the survival. The patients who received post-operative adjuvant chemotherapy had better 3-year survival than those who did not (17.28% vs 10% p=0.03). CONCLUSION: Krukenberg tumor of the ovary is an aggressive tumor with poor prognosis. Post-operative adjuvant chemotherapy may increase the survival of patients with Krukenberg tumor of the ovary. Further prospective studies for the role of surgery and chemotherapy are needed.
Ascites ; Chemotherapy, Adjuvant ; Colon ; Diagnosis ; Drug Therapy ; Female ; Gallbladder ; Humans ; Krukenberg Tumor* ; Ovary* ; Prognosis ; Retrospective Studies ; Stomach ; Survival Rate

OBJECTIVES: This study is to investigate clinicopathologic characteristics, survival and prognostic factors in patients with Krukenberg tumor of the ovary. MATERIAL & METHODS: From Jan. 1991 to Dec. 2000, 42 patients with Krukenberg tumor of the ovary were investigated with clinical profiles, such as age, stage, primary sites, clinical symptoms, and survival, retrospectively. RESULTS: A mean age of 42 patients was 44.8 years (range 27-77). Stomach was the most frequent primary site (30/42, 71.4%), followed by colon (7/42, 16.7%) and gallbladder (1/42, 2.4%). In 38 patients, primary sites diagnosed before or after 1 month of diagnosis of Krukenberg tumor of ovary (36/38 cases, 94.7%). The most common feature of patients with Krukenberg tumor of ovary was bilateral abdominal mass. 5-year survival rate of patients with Krukenberg tumor of ovary was 8.94% (95% CI=3.33-14.55) and median survival time was 11 months. Age, bilaterality of tumor, time of diagnosis, presence of ascites and the primary site did not affect the survival. The patients who received post-operative adjuvant chemotherapy had better 3-year survival than those who did not (17.28% vs 10% p=0.03). CONCLUSION: Krukenberg tumor of the ovary is an aggressive tumor with poor prognosis. Post-operative adjuvant chemotherapy may increase the survival of patients with Krukenberg tumor of the ovary. Further prospective studies for the role of surgery and chemotherapy are needed.
Ascites ; Chemotherapy, Adjuvant ; Colon ; Diagnosis ; Drug Therapy ; Female ; Gallbladder ; Humans ; Krukenberg Tumor* ; Ovary* ; Prognosis ; Retrospective Studies ; Stomach ; Survival Rate

BACKGROUND: The presence of cardiac metastasis from cervical cancer is very rare. Due to the rarity of this condition, clinical characteristics of the patients with cardiac metastasis from cervical carcinoma is not well known. This study is to investigate the clinical profiles and outcomes of patients with cardiac metastasis from the carcinoma of the uterine cervix. MATERIALS AND METHODS: From Jan. 1990 to May 1999, 4 patients with cardiac metastasis from cervical cancer during the course of the disease were registered in Korea Cancer Center Hospital. The clinical characteristics of these patients were reviewed retrospectively. RESULTS: Mean age of patients was 49 years, and median interval from initial diagnosis to recurrence was 27 months. Histologically, all 4 cases were squamous cell type carcinoma. The stage distribution is two patients with FIGO stage II, one with stage I and one with stage III. Clinical symptoms of patients were dyspnea and facial edema in 2 patients, facial edema in one patients, and dyspneas only in one patient. All patients were confirmed histologically by pericardiocentesis. Synchronous metastatic lesions were supraclavicular lymph node, lung parenchyme and paraaortic, mediastinal lymph node. Following pericardiocentesis or pericardiodesis, all patients were improved clinically and survived for mean interval of 5.3 months. CONCLUSIONS: The patients with pericardial metastasis from cervical carcinoma showed a short survival time, but could be managed by pericardiocentesis or pericardiodesis with improvement of clinical symptoms.
Cervix Uteri ; Diagnosis ; Dyspnea ; Edema ; Female ; Humans ; Korea ; Lung ; Lymph Nodes ; Neoplasm Metastasis* ; Pericardiocentesis ; Recurrence ; Retrospective Studies ; Uterine Cervical Neoplasms*

OBJECTIVES: This non-randomized retrospective study was to investigate the clinical characteristics and to evaluate the actual 5-year survival rate of the patients with invasive cancer of the cervix. METHODS: 489 evaluable patients with invasive cancer of the cervix were treated at Korea Cancer Center Hospital from January to December 1992. In this retrospective study, we studied the clinico-pathologic characteristics(age, FIGO stage, histologic type, nodal metastasis) and treatment modalities by the review of medical records. Especially, the survival was confirmed by the support of the police and government office. RESULTS: The most common subsets of patients were found in the group of FIGO stage IIb(32.5%) and age between 51 and 60(33%). Surgery was the main treatment in stage Ib/IIa(65%) and radiation in stage IIb or more(97%). Nodal metastasis were surgically identified in 6% of stage Ib, 29% of stage IIa and 36% of stage IIb. Overall actual 5-year survival rate was 72.2%; stage Ia(100%), Ib(94%), IIa(82%), IIb(63%), IIIa(36%), IIIb(47%), and IV(0%). The five-year survival rate according to LN status in surgically confirmed FIGO stage Ib-II patients were 91.9% in negative patients and 73.1% in positive patients respectively. Five-year survival rate was significantly different according to stage(P < 0.02) and nodal metastasis(p < 0.01). However, age and histologic type did not show any significant differences in survival. CONCLUSION: Overall actual five-year survival rate of 489 evaluable patients with invasive cancer of the cervix who were treated at Korea Cancer Center Hospital from January to December 1992 was 72.2%. Five-year survival rate was different according to stage and nodal metastasis.
Humans ; Korea ; Medical Records ; Neoplasm Metastasis ; Police ; Retrospective Studies ; Survival Rate ; Uterine Cervical Neoplasms*

OBJECTIVES: To investigate clinicopathologic findings of patients with small cell carcinoma of uterine cervix, and to evaluate the recurrence pattern and prognosis of patients with small cell carcinoma of the uterine cervix. Methods: From Jan. 1990, to Dec. 1997, 23 patients with small cell carcinoma of the uterine cervix were registered and followed-up at Korea Cancer Center Hospital. Clinical characteristics, survival of these patients were studied retrospectively. RESULTS: Among the 23 cases of small cell carcinoma of uterine cervix, 17 cases(74%) were of the neuroendocrine type (NE group), and 6 cases(26.0%) of the squamous cell type (SCC group). The median age, FIGO stage, and treatment modality were not significant difference between two groups. Pelvic lymph node metastases were found 53% in NE group, and 33% in SCC group, but there were not significant difference between two groups(p>0.05). Three patients showed distant metastases in NE group(bone 18%, bladder 9%), but there was no distant metastasis in SCC group. The 3 year survival rate was 50.0% in SCC group and 32.1% in NE group, but there were not statistical significance(p>0.05). Six patients showed recurrence after treatment (4/17 cases in NE group, 2/6 cases in SCC group). Recurrence sites were liver (3/6, 50%), and lung (2/6, 33%), brain (2/6, 33%), retroperitoneum (1/6, 17%), and axillae lymph node (1/6, 17%). CONCLUSION: This study showed neuroendocrine small cell carcinoma may have more aggressive than squamous small cell carcinoma, but there were not significant difference in prognosis between the two groups. Because of limitation of number of patients, further large scaled multicenter studies are needed.
Axilla ; Brain ; Carcinoma, Small Cell* ; Cervix Uteri* ; Female ; Humans ; Korea ; Liver ; Lung ; Lymph Nodes ; Neoplasm Metastasis ; Prognosis ; Recurrence ; Retrospective Studies ; Survival Rate ; Urinary Bladder ; Uterine Cervical Neoplasms