We report an autopsy case of congenital monoblastic leukemia that developed in monozygotic twins. The twin presented with progressive hepatosplenomegaly at 4 weeks after birth. One twin died of massive bleeding and hypovolemic shock before the treatment started. At autopsy, the liver was diffusely enlarged and showed a diffuse whitish discoloration except for the subcapsular and perivenular areas. Microscopic examination disclosed infiltration of histiocyte-like atypical cells along the sinusoids and portal areas of the liver. Spleen, lymph nodes and choroid plexus were also infiltrated by the tumor cells. However, bone marrow involvement of the tumor was minimal although multifocal. On immunohistochemical staining, these atypical cells were reactive for CD68 (PGM-1) and lysozyme, suggesting that the tumor cells might have been derived from mono- histiocyte. Cytogenetic study revealed 9;11 translocation, which is frequently associated with acute monoblastic leukemia. To the best of our knowledge, this is the first report of congenital monoblastic leukemia of monozygotic twins in Korea.
*Chromosomes, Human, Pair 11 ; *Chromosomes, Human, Pair 9 ; *Diseases in Twins/genetics ; Fatal Outcome ; Female ; Hepatomegaly/complications/genetics/pathology ; Human ; Infant, Newborn ; Leukemia, Monocytic, Acute/complications/*congenital/genetics/pathology ; Liver/pathology ; Splenomegaly/complications/genetics/pathology ; *Translocation (Genetics) ; *Twins, Monozygotic/genetics

To investigate the interaction of stimulatory GTP binding protein (G(s)) pathways with others, we overexpressed wild type alpha subunit of G(s) (G(s) alpha), constitutively activated R201E G(s) alpha, and dominant negative G226A G(s) alpha in COS-1 cells by transfection with DEAE-dextran, respectively, The expression of various G proteins in the transfected cells was analyzed after 72 h by quantitative Western blots, and cAMP production by stimulation with isoproterenol and forskolin was quantitated using cAMP binding proteins, The expression of Gs alpha increased about 5-fold in the transfected cells, with concomitant increase in the small forms. However, there was no significant alteration the in the level of the alpha subunit of inhibitory G protein (G(i)) and G(q), and the beta subunits of G proteins. The cAMP level without stimulation increased in the cells transfected with G(s) alpha regardless to the type of mutation, Treatment with either isoproterenol or forskolin resulted in comparable increase of the cAMP level in all the transfected cells, though the ratio to its respective basal level was smaller in the G(s) alpha-transfected cells, From this experiment, we found that the expression of the other G proteins and the signaling pathway producing cAMP did not change significantly by transiently expressing wild type, constitutively activated type, and dominant negative type of G(s) alpha. Analysis of the effects of long-term expression of Gs alpha would contribute to better understanding on how the G(s) alpha signaling system interacts with other signaling pathways and how it adapts to the changed environments.
Animals ; Blotting, Western ; Carrier Proteins ; Colforsin ; COS Cells* ; Cyclic AMP ; DEAE-Dextran ; GTP-Binding Proteins* ; Isoproterenol ; Protein Engineering ; Recombinant Proteins ; Transfection

We Studied the sport injuries of 135 Korean dissbled athletes who were going to Seoul Paralympics from 15th September 1988 to 30th September 1988. The results were as follows.1. The mean age was 23.3years, male and female ratio was 7 to l. 2. The type of disability were as follows ; Poliomyelitis, Cerebral Palsy and Blindness and transition rate of below 5 years of age was 71.6%. 3. The most common age of begining exercise of the disabled athletes were 16-20 years and composed of 77.4%. 4. The most common cause and process of athletic injuries were heavy training and excessive exercise. 5. The most common athletic injury was soft tissue injury and the most common site was hand and the most common type of injury was blindness and the most common causative sport was baseball and fencing. 6. The protects were used in 41% of athletes and they were globes and glassed as follows. 7. Over 77% of the disabled athletes were answered as “Necessary” to the question about scientification of disabled sport.
Athletes ; Athletic Injuries ; Baseball ; Blindness ; Cerebral Palsy ; Female ; Glass ; Hand ; Humans ; Male ; Poliomyelitis ; Seoul ; Soft Tissue Injuries ; Sports

Rhabdomyosarcoma is the most common sarcoma of children and adolescents. Rhabdomyosarcoma is a highly malignant soft tissue sarcoma that can arise in any site of the body containing striated muscle or its mesenchymal analogue. The common primary sites were head and neck including orbit, extremities, genitourinary tract, trunk, retroperitoneum, gastrointestinal tract, and thorax. There are basically three types of rhabdomyosarcoma: embryonal, alveolar, and pleomorphic; these categories may overlap histologically. Retroperitoneal rhabdomyosarcoma of embryonal type is rarely reported in an adult. Since we experienced a case of 25 X 20 X 12cm-sized retroperitoneal rhabdomyosarcoma of embryonal type, we report this with a review of literatures.
Adolescent ; Adult* ; Child ; Extremities ; Gastrointestinal Tract ; Head ; Humans ; Muscle, Striated ; Neck ; Orbit ; Rhabdomyosarcoma* ; Sarcoma ; Thorax

Acute multifocal bacterial nephritis is a severe form of acute renal infection in which a heavy leukocytic infiltrate occurs throughout the kidney with intrarenal abscess formation. It is also early phase of renal corticomedullary abscess. It is imperative that a prompt acute diagnosis be made in most of these conditions, for these patients may be or may rapidly become septic and suddenly deteriorate if not treated appropriately and in a timely manner. Clinically, acute multifocal bacterial nephritis present as acute pyelonephritis and could be diagnosed by radiologic grounds including abdominal computerized tomography(CT) which demonstrates poorly defined wedge-shaped area of decreased contrast enhancement identical to those found in acute focal bacterial nephritis. This case was a women with high fever, pyuria and severe leukocytosis. It was diagnosed by abdominal computerized tomography which show multiple wedge-shaped area of decreased attenuation and by computerized tomography guide aspiration biopsy which demonstrates E. coli. We report one case of acute multifocal bacterial nephritis with the reviews of literatures which showed involvement of both kidneys
Abscess ; Biopsy, Needle ; Diagnosis ; Female ; Fever ; Humans ; Kidney ; Leukocytosis ; Nephritis* ; Pyelonephritis ; Pyuria

Mesenchymal hamartoma (MH) of the liver is an uncommon benign lesion related to ductal plate malformation. It is usually cystic and mainly composed of myxoid mesenchymal tissue with tortuous or cystic bile ducts. In order to characterize the clinicopathological features of MH, the Korean Gastrointestinal Pathology Study Group collected a total of 17 MH cases diagnosed in 7 hospitals from 1992 to 2002 and compared the clinicopathologic findings of cystic MH with those of solid variant. Among the 17 cases, 7 (41%) were solid. The solid form showed a higher serum level of alpha-fetoprotein (AFP), the smaller bile ducts, and more frequent proliferation of vessels. Serum AFP level was related to the amount of hepatocytes. Two of seven solid cases harbored a larger amount of evenly distributed hepatocytes and proliferation of small duct with focal hepatocyte-bile duct transition. These histologic findings are similar to those of mixed hamartoma. Therefore, the mixed hamartoma and the MH of both solid and cystic types could be the variants of one disease spectrum. And hepatocytes within MH might be rather a genuine tumor component than entrapped into the tumor. In conclusion, MH can show various clinicopathological features and recognition of these features will facilitate accurate diagnosis of MH.
Adult ; Aged ; Child ; Child, Preschool ; Comparative Study ; Cysts/pathology ; Female ; Hamartoma/*pathology ; Humans ; Infant ; Liver/pathology ; Liver Diseases/*pathology ; Male

Multiple lymphomatous polyposis(MLP) is an interesting clinical entity of non-Hodgkin's lymphoma(NHL) and is defined as B-cell lymphoma characterized by the presence of multiple lymphomatous polyps along the gastrointestinal tract. Recently MLP has been considered as a variant form of mantle cell lymphoma(MCL). The median survival of patients with MCL is only 3 years, and none of the available conventional chemotherapy regimens appears curative. Encouraging results have been reported with high dose chemotherapy with autoSCT and alloSCT for its treatment. We introduce 4 cases of MLP diagnosed as MCL by morphologic and immunologic method. The common clinical findings of these cases were splenomegaly (4/4), multiple intraabdominal lymphadenopathy (4/4), and advanced stage (3/4) at presentation. The overall remission duration was relatively short (5-27 months) and three of four cases relapsed after conventional chemotherapy or autologous stem cell transplant. Our report suggests that MCL presented as MLP is a high risk subgroup of NHL and more aggressive approach may be needed for cure.
Drug Therapy ; Gastrointestinal Tract ; Humans ; Lymphatic Diseases ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Mantle-Cell* ; Polyps ; Splenomegaly ; Stem Cells

PURPOSE: Despite extracardiac conduit Fontan had many advantages, long-term results related to longevity of conduit, and anticoagulation were not proven. This study was to evaluate the long-term outcome of hospital survivors with extracardiac Fontan circulation. METHODS: Between 1996 and 2006, 200 patients underwent extracardiac conduit Fontan operation. Median age at the Fontan operation was 3.4 years (range:16 months-35.7 years). All patients (89.5 %) except 21 patients of one stage Fontan operation underwent bi-directional cavopulmonary shunt. Fenestration was required in 85 patients (42.5%). RESULTS: There has been 6 hospital mortalities (3.0%), and 7 late mortalities (3.6%) at a mean follow-up of 52.4+/-32.2 months (range; 18days-120 months). Overall 10-years survival was 92.4 2.1%. Multivariate analysis identified severe infection at early postoperative periods (hazard ratio =12.439, P=0.001), and high pulmonary arterial pressure at preoperative period (hazard ratio=3.445, P=0.038) as risk factors for mortality. Reoperation was performed in 24 patients (12.0%), and freedom from reoperation was 82.4+/-4.1% at 10 years. Arrhythmia occurred in 32 patients (16.0%) after Fontan operation, and freedom from arrhythmia was 85.14.4% at 10 years. Risk factors for arrhythmia were heterotaxy syndrome (P=0.001), Follow up duration (P=0.027) and the age at Fontan operation (P=0.001). Freedom from thromboembolism was 92.91.9% at 10 years. The conduit cross- sectional area decreased by 14%, and the extent of decrease of the conduit cross-sectional remained stable irrespective of the follow-up duration. 95.2% of patients had New York Heart Association class I. CONCLUSIONS: After 10 years of follow-up, the overall survival, and the functional status of survivors of the extracardiac Fontan procedure are satisfactory. Fenestration has a beneficial effect on the results of high-risk Fontan patients. The incidence of late deaths, reoperations, obstructions of the cavopulmonary pathway, arrhythmias and thromboembolisms is low.
Arrhythmias, Cardiac ; Arterial Pressure ; Follow-Up Studies ; Fontan Procedure* ; Freedom ; Heart ; Heart Bypass, Right ; Heterotaxy Syndrome ; Hospital Mortality ; Humans ; Incidence ; Korea* ; Longevity ; Mortality ; Multivariate Analysis ; Postoperative Period ; Preoperative Period ; Reoperation ; Risk Factors ; Survivors ; Thromboembolism

BACKGROUND: Once the diagnosis of operable breast cancer has been made, the single most predictive factor is lymph node status. Although patients with lymph node initially reported as histologically negative have a relatively good prognosis, the relapse rate is still considerable. In an effort to detect micrometastases in the axillary nodes, various antibodies have been used that recognize membrane and cytokeratin antigens. Cytokeratin antigens are expressed by epithelial tumors and are not expressed by normal lymphoid tissues. This study uses an immunohistochemical method to examine the incidence and the prognostic significance of such micrometastases in a series of patients with "node-negative" breast cancer. METHODS: The study population consisted of a retrospective series of 150 patient who were treated at Soonchunhyang University Hospital for breast cancer between March 1992 and February 1998. Based on the original pathologic examination, patients had negative axillary nodes. Pan-cytokeratin, a cocktail of monoclonal antibodies to cytokeratin (58 kd, 56 kd, 52 kd, 45 kd), and the avidin-biotin-peroxidase- complex technique were used to detect micrometastases in paraffin embedded lymph nodes. RESULTS: Micrometastases were detected in 16 (10.7%) patients. The recurrence rate for patients with micrometastases was 6.3% (1/16), and the recurrence rate for patients without micrometastases was 1.5% (2/134). Micrometastases correlated with the histological type (P=0.026) and were seen more frequently with larger tumor size, higher anaplastic nuclear grade, and overexpression of p53.
Antibodies ; Antibodies, Monoclonal ; Breast Neoplasms* ; Breast* ; Diagnosis ; Fibrinogen ; Humans ; Incidence ; Keratins ; Lymph Nodes* ; Lymphoid Tissue ; Membranes ; Neoplasm Micrometastasis* ; Paraffin ; Prognosis ; Recurrence ; Retrospective Studies

BACKGROUND: Acute ischemic stroke secondary to aortic dissection (AoD) is challenging in the era of thrombolysis owing to the diagnostic difficulty within a narrow time window and the high risk of complications. CASE REPORT: A 64-year-old woman with middle cerebral artery occlusion syndrome admitted to the emergency room within intravenous recombinant tissue plasminogen activator (rt-PA) time window. Her neurological symptoms improved during thrombolysis, but chest and abdominal pain developed. Repeated history-taking, physical examination, and imaging studies led to the timely diagnosis and surgical treatment of AoD, which produced a successful outcome. CONCLUSIONS: Clinical suspicion is invaluable for the diagnosis of this rare cause of stroke. Considering the stroke mechanism and complications, the risks of thrombolysis might outweigh its benefits.
Abdominal Pain ; Emergencies ; Female ; Humans ; Infarction, Middle Cerebral Artery ; Middle Aged ; Physical Examination ; Stroke ; Thorax ; Tissue Plasminogen Activator