PURPOSE: We evaluated the capability of MR in the diagnosis of anomalous pulmonary venous connection (APVC). MATERIALS AND METHODS: The patient group consisted of 11 total APVC and 8 partial APVC diagnosed with MR. Echocardiography was performed in all cases, cardiac angiography in 12 cases and operation in 12 cases. We compared MR findings with those of operation, echocard iography and cardiac angiography. RESULTS: In surgically proven 12 cases, diagnostic accuracy of preoperative MR, echocardiography and cardiac angiography was 100%, 67% and 63%, respectively. In the remaining cases, MR findings well correlated with those of echocardiography or cardiac angiography. Stenosis of common pulmonary vein or superior vena cava was identified in 4 cases. In one patient, MR depicted associated cor triatriatum clearly. CONCLUSION: MR is an effective modallty in depicting anomalous pulmonary venous con nections.
Angiography ; Constriction, Pathologic ; Cor Triatriatum ; Diagnosis ; Echocardiography ; Humans ; Pulmonary Veins ; Vena Cava, Superior

No abstract available.
Diabetes Mellitus, Type 1* ; Echocardiography*

The pleomorphic adenoma is the most common neoplasm involving both the major and minor salivary glands. It is a benign, slowgrowing tumor, but local recurrences can occur. The pleomorphic adenoma gene 1 (PLAG1), which is a novel zinc finger gene, is frequently activated by reciprocal chromosomal translocations involving 8q12 in a subset of salivary gland pleomorphic adenomas. This experimental study was preformed to observe the translocation patterns between PLAG1 gene and the three translocation partner genes. We also have analyzed the presence of PLAG1 transcripts by RT-PCR. CTNNB1/PLAG1 gene fusion was observed in three of nine pleomorphic adnomas. However, LIFR/PLAG1 and SII/PLAG1 gene fusions were not detectable. All of three gene fusions was not detectable in one Warthin's tumor and three inflammatory salivary gland tissues. PLAG1 transcripts were expressed in all inflammatory salivary gland tissues and tumors except for three pleomorphic adenomas. Of particular one pleomorphic adenoma showing CTNNB1/P AG1 gene fusion did not express PLAG1 transcipt. Our data indicate that gene fusion involving PLAG1 is a frequent event in pleomorphic adenoma, but correlation between gene fusion involving PLAG1 and PLAG1 transcription is not definite.
Adenoma, Pleomorphic* ; Gene Fusion ; Recurrence ; Salivary Glands* ; Salivary Glands, Minor ; Translocation, Genetic ; Zinc Fingers

PURPOSE: Forward pulmonary blood flow may be absent in some neonates with Ebstein's anomaly by anatomical or functional pulmonary atresia in association with the elevated pulmonary vascular resistance, patent ductus arteriosus and tricuspid regurgitation. We reviewed the presentation and outcomes of symptomatic neonates with Ebstein's anomaly focusing on the pulmonary atresia. METHODS: Clinical presentation and outcome of 15 symptomatic neonates with Ebstein's anomaly seen at Asan medical center from 1998 to 2004 were reviewed. RESULTS: Ten (67%) of 15 patients showed no forward pulmonary blood flow and 6 of them had functional pulmonary atresia. O2 saturation and pH were lower and cardiothoracic (CT) ratio in chest radiography was more increased in the patients with pulmonary atresia than in the patients without pulmonary atresia (P< 0.05). pH and CT ratio were not different between the anatomical and functional pulmonary atresia group, but O2 saturation was lower in functional atresia group (P< 0.05). 13 patients (87%) were managed with PGE1. 4 of 6 patients with functional pulmonary atresia were treated with inhaled nitric oxide. Surgery was performed in 1 of 5 patients without pulmonary atresia and in 8 of 10 patients with pulmonary atresia during follow-up period (mean 37 months). 3 patients (20%) died and none of patients without pulmonary atresia died. CONCLUSION: We found that most symptomatic neonates with Ebstein's anomaly had functional or anatomical pulmonary atresia. The neonates with Ebstein's anomaly who had no forward pulmonary blood flow were more symptomatic and needed surgery earlier. Further studies will be needed to distinguish effectively functional and anatomical pulmonary atresia and to manage appropriately neonates with functional atresia.
Infant, Newborn ; Humans

No abstract available.
Aged* ; Dementia* ; Gyeongsangbuk-do* ; Humans ; Prevalence*

No abstract available.
Aged* ; Dementia* ; Gyeongsangbuk-do* ; Humans ; Prevalence*

Kawasaki disease (KD) is an immune-mediated disease which is a leading cause of acquired cardiovascular disease in developed country. Recently, tumor necrosis factor-alpha (TNF-alpha) blocker, infliximab has been considered a promising option for patients with refractory KD. Although chronic use of a TNF-alpha blocker could increase risk of opportunistic infections, a few studies have documented that use of infliximab was safe without serious adverse effects in patients with KD. We observed serious bacterial infection after infliximab treatment in an infant with refractory KD. Our patient was a 5-month-old male infant diagnosed with KD who did not respond to repeated doses of intravenous immunoglobulin. We effectively treated him with a single infusion of infliximab (5 mg/kg), but gram-negative (Acinetobacter lwoffii) septicemia developed after infliximab infusion. Therefore, we report a case of serious septicemia after treatment with infliximab, and suggest considering the risk of severe infection when deciding whether to prescribe infliximab to an infant with refractory KD.
Bacterial Infections ; Cardiovascular Diseases ; Developed Countries ; Humans ; Immunoglobulins ; Infant* ; Male ; Mucocutaneous Lymph Node Syndrome* ; Opportunistic Infections ; Sepsis* ; Tumor Necrosis Factor-alpha ; Infliximab

Hemangioblastomas comprise 1 to 2% of all intracranial neoplasm, and 8 to 12% of tumors within the posterior fossa. They are composed of admixtures of three different cell types; endothelial cells, pericytes and stromal cells. Although most hemangioblastomas arise sporadically, they are associated with von Hippel-Lindau disease in about 20% cases. We have experienced a case of multiple hemangioblastomas occuiing in the cerebellum, medulla oblongata and cervical spinal cord simultaneously in a 55-year-old male. He had complained of headache, dizziness, generalized weakness and gait disturbance for 2 weeks. The patient had neither specific family history nor increased hematocrit. MRI showed a nonenhancing cystic lesion with an enhancing mural nodule in the right cerebellar hemisphere and two separate enhancing nodules in the medulla oblongata and dorsal cervical spinal cord at the 5-6th. Grossly, the excised mass of the cerebellum, 2.5 x 2 x 1.8cm, was solid to partly cystic, and that of spinal cord, lcm in diameter, was mostly solid. Microscopically, the tumor was composed of thin-walled blood vessels in variable size and interspersed stromal cells. The stromal cells revealed dimorphic cytoplasm that were either homogeneous and eosinophilic, or clear and vacuolated. Immunohistochemically, the endothelial cells reacted positively for glial fibrfllaty acidic protein(GFAP) and vimentin. The stromal cells reacted diffusely positively for vimentin, focally positively for GFAP and S-100 protein near the periphery of the tumor, focally positivel for neuro specipic enolase(NSE), and negatively for lysozyme, desmin and chromogranin. Ultrastructurally, the stromal cells contained numerous microfilaments and lipid droplets.

Hemangioblastomas comprise 1 to 2% of all intracranial neoplasm, and 8 to 12% of tumors within the posterior fossa. They are composed of admixtures of three different cell types; endothelial cells, pericytes and stromal cells. Although most hemangioblastomas arise sporadically, they are associated with von Hippel-Lindau disease in about 20% cases. We have experienced a case of multiple hemangioblastomas occuiing in the cerebellum, medulla oblongata and cervical spinal cord simultaneously in a 55-year-old male. He had complained of headache, dizziness, generalized weakness and gait disturbance for 2 weeks. The patient had neither specific family history nor increased hematocrit. MRI showed a nonenhancing cystic lesion with an enhancing mural nodule in the right cerebellar hemisphere and two separate enhancing nodules in the medulla oblongata and dorsal cervical spinal cord at the 5-6th. Grossly, the excised mass of the cerebellum, 2.5 x 2 x 1.8cm, was solid to partly cystic, and that of spinal cord, lcm in diameter, was mostly solid. Microscopically, the tumor was composed of thin-walled blood vessels in variable size and interspersed stromal cells. The stromal cells revealed dimorphic cytoplasm that were either homogeneous and eosinophilic, or clear and vacuolated. Immunohistochemically, the endothelial cells reacted positively for glial fibrfllaty acidic protein(GFAP) and vimentin. The stromal cells reacted diffusely positively for vimentin, focally positively for GFAP and S-100 protein near the periphery of the tumor, focally positivel for neuro specipic enolase(NSE), and negatively for lysozyme, desmin and chromogranin. Ultrastructurally, the stromal cells contained numerous microfilaments and lipid droplets.

The infantile polycystic kidney disease is rare fetal urinary tract anomaly. It is inherited with an autosomal recessive pattern and recurrence rate is 25%. The gene locus is on chromosome 6p. The pathogenesis of infantile polycystic kidney is the primary defect of the collecting ducts. The ultrasonographic finding of infantile polycystic kidney is oligohydramnios, bilaterally symmetrical enlarged kidneys with maintenance of their reinform shape. The differential diagnosis with adult polycystic kidney disease is important. The massive enlargement of the kidneys is rarely seen in adult polycystic kidney disease and the examination of the parents and other members of the family is helpful to confirm the adult polycystic kidney disease. If there is severe renal involvements, stillbirth or neonatal death secondary to pulmonary hypoplasia would be developed. If it were diagnosed before viability, termination of pregnancy is recommended. In a fetus at risk, diagnosed after viability, pregnancy termination is also recommended since this condition is uniformly fatal. We present a case of infantile polycystic kidney.
Diagnosis, Differential ; Female ; Fetus ; Humans ; Kidney ; Oligohydramnios ; Parents ; Polycystic Kidney Diseases* ; Polycystic Kidney, Autosomal Dominant ; Pregnancy ; Recurrence ; Stillbirth ; Urinary Tract