Noonan syndrome is characterized by typical facies, congenital heart defect, and some clinical features similar to Turner syndrome, but with normal chromosomes. The most commonly associated cardiac defects are pulmonary valvular stenosis and strial septal defect. We experienced a case of Nonan syndrome associated with pulmonay valve stenosis with double-chambered right ventricle and atrial septal defect and cryptorchidism. Pulmonary valvotomy was done through transannular incision. Hypertrophied muscle bundles were excised. Atrial septal defect was closed directly. RVOT was reconstructed with pericardial transannular patch. Orchiopexy was performed simultaneously without any problem.
Constriction, Pathologic ; Cryptorchidism ; Facies ; Heart Defects, Congenital ; Heart Septal Defects, Atrial* ; Heart Ventricles* ; Male ; Noonan Syndrome* ; Orchiopexy ; Turner Syndrome

No abstract available.
Dexamethasone* ; Prognosis*

Malignant Rhabdoid tumor is a rare malignant neoplasm and is morphologically similar to rhabdomyosarcoma, but is different immunohistochemically. Most malignant rhabdoid tumors occur in the kidney of infants, and are rarely reported at extrarenal sites. Since Frierson and his collagues first described the malignant extrarenal rhabdoid tumor(MRT) in children in 1985, it has rarely been reported. The neoplasm is histologically and immunohistochemically identical to a renal malignant rhabdoid tumor. There have been no previous reports of this neoplasm in Korea. An MRT of the sacroiliac bone in a 11-year-old girl is described. Five months after the initial presentation, the patient is still alive with partial response to combination chemotherapy and radiotherapy. The authors review previous reports of extrarenal MRT, and discuss the pathologic characteristics, differential diagnosis, and treatment of this rare neoplasm.
Child ; Diagnosis, Differential ; Drug Therapy, Combination ; Female ; Humans ; Infant ; Kidney ; Korea ; Radiotherapy ; Rhabdoid Tumor* ; Rhabdomyosarcoma

Myocardial free wall rupture is the most serious complication of acute myocardial infarction. Although it is not uncommon, it is difficult to treat successfully. We report a case of acute inferior myocardial infarction complicated with left ventricular free wall rupture that occurred 8 hours after onset of chest pain. In this case, progression of mild pericardial effusion to cardiac tamponade was monitored by transhtoracic echocardiography. Pericardiocentesis and draninage failed to treat cardiac tamponade, and surgical repair was performed successfully. The patient discharged uneventfully on 28th day and followed regularly at the outpatient department.
Cardiac Tamponade ; Chest Pain ; Echocardiography ; Heart Rupture* ; Humans ; Inferior Wall Myocardial Infarction* ; Myocardial Infarction ; Outpatients ; Pericardial Effusion ; Pericardiocentesis ; Rupture