Congenital mesoblastic nephroma(CMN) is an important differential diagnosis of a renal mass occurring in the newborn or in early childhood. It was first described by Bolande as a separate disease entity distinct form Wilms' tumor. In 1974, Beckwith has predicted that this tumor has a pathologic spectrum with classic congenital mesoblastic nephroma at one extreme, unequivocally mallignant spindle cell sarcomas at the other, and intermediate "gray zone" lesions of indeterminate biologic significanse. In 1986, Joshi has described "atypical mesoblastic nephroma" as a potentially aggressive variant of CMN, which shows atypical gross and microscopic features such as hemorrhage, necrosis, high cellularity, and mitotic index. We report of a case of atypical mesoblastic nephroma presenting in a 38 days-old male infant. Grossly, the tumor involved the upper and midportion of the left kidney. On section, the cut surface was fleshy, grayish-white, and homogeneous. Microscopically, the tumorshowed high degree of cellularity and arrangement of fusiform cells in sheets and vague interlacing bundles. The individual tumor cells showed fusiform to oval nuclei, indistinct scanty pale-eosinophilic cytoplasm and many mitotic figures.
Infant ; Child ; Male ; Female ; Infant, Newborn ; Humans ; Diagnosis, Differential

Turner's syndrome results from complete or partial monosomy of the X chromosome and is characterized by hypogonadism or related other congenital anomalies in phenotypic females. In these patients, there are failure to develop normal secondary sex characteristics, amenorrhea, or short stature at puberty and the ovaries are reduced to atrophic fibrous strands devoid of ova and follicles(streak gonads). Individuals with this condition are particularly prone to the development of gonadoblastoma. For this reason, the gonads should be early removed and supplemental estrogen therapy given. We experienced a case of Turner's syndrome, 45, XO/46, XY karyotype in a 20-year-old phenotypic female complained an amenorrhea. On the exploratory laparotomy, the right gonadal mass is sevearly adhered to the adjacent organs and measures 8 x 5 x 5 cm in dimension and 75gm in weight and shows multiple foci of hemorrhage with necrosis. The left streak gonad measures 3.5 x 2 x 1.5 cm in dimension and shows multiple foci of calcification. Microscopically, the right gonadal mass reveals malignant mixed germ cell tumor, composed of endodermal sinus tumor, composed of endodermal sinus tumor with dysgerminoma and gonadoblastoma. The left streak gonad consists of mainly dense fibrous connective tissue and shows some foci of calcification associated with gonadoblastoma. On immunohistochemical and special stainings, the cytoplasm and hyalin droplets of the endodermal sinus tumor component reveal strong positivity to the a-fetoprotein and PAS. After removal of both gonads, the serum level of the a-fetoprotein is markedly down from 1742ng/ml to 2.6 ng/ml.
Female ; Humans

The authors analyzed 30 patients whose celiac artery or its branches were dissected during the procedure of transcatheter arterial embolization (TAE) for hepatocellular carcinoma (HCC) and were followed up angiographically. The incidence of arterial dissection was 1.25%. The dissection occurred most frequently in the celiac artery (40% 12/30) and the proper hepatic artery(28% 7/30). The frequency of arterial dissection was affected by the status of the vessel and the experience of the operator. The follow-up angiography revealed complete recanalization in 40% (12/30), irregularity and narrowing of the lumen in 23% (7/30), pseudoaneurysm formation in 23%(7/30), and complete obstruction in 13% (4/30) of the cases. The rate of recanalization in the celiac artery was lower than that of any other arteries in our series. Recamalization to a certain degree without therapeutic intervention was observed in 72% of the dissected arteries enabling and contributing to subsequent successful TAE within 2 months in 78% of the patients with the dissection.
Aneurysm, False ; Angiography ; Arteries ; Carcinoma, Hepatocellular ; Celiac Artery* ; Follow-Up Studies* ; Humans ; Incidence

No abstract available.
Female ; Pregnancy ; Pregnancy, Ectopic* ; Sterilization, Tubal*

The authors encountered 2 siblings who showed progressive ophthalmoplegia and bulbar palsy. The patients were studied with various laboratory aids and thought to be as oculopharyngeal muscular dystrophy. The oculopharyngeal muscular dystrophy is a rare form of muscular dystrophy. The authors present the cases as familial oculopharyngeal muscular dystrophy.
Bulbar Palsy, Progressive ; Humans ; Muscular Dystrophies ; Muscular Dystrophy, Oculopharyngeal* ; Ophthalmoplegia ; Siblings

In order to study the clinical efficacy of the metallic stent of the treatment of iliac arterial stenosis, the clinical and arteriographic findings of the 8 patients were retrospectively reviewed. All 8 patients were males with an age of 51 to 79. The Fontaine class for the functional status of lower extremities was II in 4 patients, III in 3 patients and IV in 1 patients. Self expandable Gianturco stent was inserted in 7 iliac arteries and balloon expandable Palmaz stent was inserted in 2 iliac arteries in the 8 patients. The indications for the metallic stent application were localized dissection with significant residual stenosis in 6sites, recoiling due to calcification in one case and eccentricity of the stenotic lesion in 2 sites. The deployment of the metallic stent was successful in all the cases to maintain the patency of iliac arteries with residual stenosis less than 30%. The Fontaine class was improved to I in 6 patients, II a in 1 patients IIb in another one. During the follow-up period of 3 to 14 months, none except one developed recurrence of the symptom. On the basis of our experience, we believe that metallic stent is safe and effective for the treatment of iliac arterial stenosis. However, we think that it is a complementary measure to the percutaneous transluminal angioplasty. The comparative study between different types of metallic stent and the long-term effect should be investigated further.
Angioplasty ; Constriction, Pathologic* ; Follow-Up Studies ; Humans ; Iliac Artery ; Lower Extremity ; Male ; Recurrence ; Retrospective Studies ; Stents* ; Treatment Outcome

In order to study the clinical efficacy of the metallic stent of the treatment of iliac arterial stenosis, the clinical and arteriographic findings of the 8 patients were retrospectively reviewed. All 8 patients were males with an age of 51 to 79. The Fontaine class for the functional status of lower extremities was II in 4 patients, III in 3 patients and IV in 1 patients. Self expandable Gianturco stent was inserted in 7 iliac arteries and balloon expandable Palmaz stent was inserted in 2 iliac arteries in the 8 patients. The indications for the metallic stent application were localized dissection with significant residual stenosis in 6sites, recoiling due to calcification in one case and eccentricity of the stenotic lesion in 2 sites. The deployment of the metallic stent was successful in all the cases to maintain the patency of iliac arteries with residual stenosis less than 30%. The Fontaine class was improved to I in 6 patients, II a in 1 patients IIb in another one. During the follow-up period of 3 to 14 months, none except one developed recurrence of the symptom. On the basis of our experience, we believe that metallic stent is safe and effective for the treatment of iliac arterial stenosis. However, we think that it is a complementary measure to the percutaneous transluminal angioplasty. The comparative study between different types of metallic stent and the long-term effect should be investigated further.
Angioplasty ; Constriction, Pathologic* ; Follow-Up Studies ; Humans ; Iliac Artery ; Lower Extremity ; Male ; Recurrence ; Retrospective Studies ; Stents* ; Treatment Outcome

Ocular neuromyotonia (ONM) is an episodic involuntary contraction of one or more extraocular muscles, resulting from spontaneous neural discharges of ocular motor nerves. Previous radiation therapy to pituitary or other juxtasellar tumor and vascular compressions are the most common reported causes of ONM. We report one unique case of ONM involving the abducens nerve without any other organic brain lesion and prior radiation therapy.
Abducens Nerve ; Brain ; Isaacs Syndrome* ; Muscles

PURPOSE: This study reviews the feasibility of a total colectomy with ileo-rectal anastomosis (TCIRA) and the functional outcome following the operation. METHODS: The cases of a total of 50 patients (31 men and 19 women) with a median age of 61 who underwent a TCIRA were reviewed retrospectively. The median follow-up time was 28 months (4~72). The clinical records were reviewed to analyze the postoperative complications and bowel function. The clinical outcomes were examined directly from patients' scoring. RESULTS: The indications of TCIRA were metachronous or synchronous colorectal cancer (34 percent), multiple polypoid lesions (22 percent), malignant colon obstruction (24 percent), ischemic colitis (2 percent), Crohn's disease (6 percent), and tuberculosis colitis (2 percent). The overall mortality and morbidity rates were 0 and 31 percent, respectively. The morbidity included postoperative bleeding, obstruction, intra-abdominal abscess formation, pneumonia, and wound complications. We used the CCIS index to evaluate postoperative functional bowel habit change. The CCIS index evaluation revealed perfect continence in 57 percent of the patients with short-term follow up (<6 months) and in 83 percent of the patients who had undergone a TCIRA more than 2 years ago. CONCLUSIONS: Most patients were satisfied with their bowel function on long-term follow up, and we think the TCIRA is a safe operation, and the clinical outcomes are relatively satisfactory.
Abdominal Abscess ; Colectomy* ; Colitis ; Colitis, Ischemic ; Colon ; Colorectal Neoplasms ; Crohn Disease ; Follow-Up Studies ; Hemorrhage ; Humans ; Male ; Mortality* ; Pneumonia ; Postoperative Complications ; Retrospective Studies ; Tuberculosis ; Wounds and Injuries

BACKGROUND: Until the early 1990's in Korea, treatment outcomes of patients with pulmonary tuberculosis in the private sector were reported to be inferior to those of the public health center under the National Tuberculosis Programme. The purpose of this study was to analyze the clinical characteristics and the treatment efficacy of the patients with pulmonary tuberculosis recently diagnosed at a private general hospital. MATERIALS and METHODS: The study included all pulmonary tuberculosis patients diagnosed at Samsung Medical Center and notified to the public health center from August 2000 to January 2001. RESULTS: Of the 232 patients included in the study during a 6-month period, 188 were (81.0%) initial treatment cases and 44 (19.0%) were re-treatment cases. Eighty-three (35.8%) patients had smear-positive sputa, and 27 (11.6%) had smear-negative, culture-positive sputa. Initial sputum examinations were not performed in 47 (20.3%) patients. A six-month, short-course treatment using isoniazid, rifampin, ethambutol and pyrazinamide was prescribed for 31.4% of the patients under the initial treatment. The success rate (cured plus treatment completed) of the initial treatment for the smear-positive patients was 69.1%. Eleven (13.3%) of the 83 patients with smear-positive pulmonary tuberculosis discontinued their treatment without notice. CONCLUSIONS: To improve the treatment efficacy and decrease the default rates of the patients with pulmonary tuberculosis in the private sector, further efforts are required in line with the Korean Academy of Tuberculosis and Respiratory Disease Treatment Guidelines and in the implementation of an appropriate model of public-private mix for tuberculosis control in Korea.
Diagnosis ; Ethambutol ; Hospitals, General* ; Humans ; Isoniazid ; Korea ; Private Practice ; Private Sector ; Public Health ; Pyrazinamide ; Rifampin ; Sputum ; Treatment Outcome ; Tuberculosis ; Tuberculosis, Pulmonary*