No abstract available.
Latex* ; Polystyrenes* ; Potassium Channels* ; Potassium* ; Vasospasm, Intracranial*

Myxofibrosarcomas (MFSs), which consist of multiple nodules with a variable cellular population in a myxoid matrix, are primarily located in subcutaneous tissue. Pleomorphic hyalinizing angiectatic tumors (PHATs) are rare soft-tissue tumors characterized by a proliferation of highly pleomorphic spindle or polygonal cells and abundant ectatic blood vessels in cellular or myxoid stroma. We present here an unusual case of a high-grade MFS with a PHAT-like appearance. A 67-year-old man presented with an asymptomatic subcutaneous mass in the right forearm. The tumor had myxoid, hypo-, and hypercellular areas with highly pleomorphic spindle or polygonal tumor cells that showed frequent mitoses and nuclear pseudoinclusions. Foci of punctuate necrosis and inflammatory infiltration were present throughout the tumor, and abundant ectatic, thick-walled vessels containing blood clots were noted. The tumor cells were immunohistochemically positive for vimentin but negative for CD34, S-100 protein, smooth muscle actin, desmin, and bcl-2.
Actins ; Aged ; Blood Vessels ; Desmin ; Forearm ; Humans ; Hyalin ; Mitosis ; Muscle, Smooth ; Necrosis ; S100 Proteins ; Sarcoma ; Subcutaneous Tissue ; Vimentin

No abstract available.

Infantile hypertrophic pyloric stenosis (IHPS) is the most common condition requiring abdominal surgery in early infancy, and is caused by hypertrophied pyloric muscle. The development of successful surgical treatment in the early 1900s by Fredet and Ramstedt made it possible for infants worldwide to survive. Modern pediatric anesthetic techniques have virtually eliminated mortality from surgical management. Atropine sulfate is a cholinergic blocking agent with potent antimuscarinic activity that decreases peristaltic contractions by relaxing smooth muscles. We treated two cases of IHPS with incomplete pyloromyotomy in 3-month-old and 5-month-old male infants by administering atropine sulfate intravenously. They were free from vomiting after 5 days of intravenous atropine sulfate treatment. In these rare cases of persistent vomiting or refractory emesis following incomplete pyloromyotomy, there may be a role for atropine sulfate.
Atropine* ; Constriction, Pathologic* ; Humans ; Infant ; Male ; Mortality ; Muscle, Smooth ; Pyloric Stenosis, Hypertrophic ; Vomiting*

OBJECTIVE: Linkage analysis is a very useful method for prenatal diagnosis of Hemophilia B, especially when a mutation was not identified. Seven polymorphic markers were studied in Korean populations to evaluate the efficiency for prenatal and carrier diagnosis. METHODS: Subjects of this study was 100 healthy Korean women (200 X-chromosomes). Polymerase chain reacton-restriction fragment length polymorphism (PCR-RFLP) method was used to detect SalI, MseI, NruI, DdeI, XmnI, TaqI and HhaI polymorphisms. RESULTS: SalI (-) allele showed the frequency of 0.355 and SalI(+) allele 0.645. MseI(-) allele was 0.645 in frequency and MseI(+) allele was 0.355. SalI and MseI polymorphisms were in complete linkage disequilibrium. And no increase was expected in overall heterozygosity with these two polymorphisms. NruI(-) allele frequency was 0.855 and NruI(+) was 0.145. There was no polymorphism of DdeI, XmnI and TaqI marker systems in Korean population. In HhaI polymorphism, allele frequencies were estimated that HhaI(-) is 0.82 and HhaI(+) is 0.18. CONCLUSION: Only SalI, NruI and HhaI polymorphisms are useful for the diagnosis of hemophilia B in Korean population. Expected heterozygosity for above 3 poylmorphic markers was estimated to be 0.723, and 71 of 100 female subjects were heterozygous for at least one marker system. Korean population showed relatively low extent of polymorphisms compared to Caucasians, Blacks and Japanese. For the effective prenatal diagnosis of hemophilia B with linkage analysis, other polymorphic markers should be evaluated.
African Continental Ancestry Group ; Alleles ; Asian Continental Ancestry Group ; Diagnosis ; Factor IX* ; Female ; Gene Frequency ; Hemophilia B ; Humans ; Linkage Disequilibrium ; Prenatal Diagnosis

No abstract available.
Causality* ; Humans ; Infant, Newborn* ; Pneumothorax*

Double outlet right ventricle, a complex of congenital cardiac anomalies in which both great arteries arise wholly or in large part from the morphologic right ventricle presents diverse clinical manifestations according to the morphological characteristics. Eighty cases with DORV were diagnosed and operated at Yonsei Cardiovascular Center from 1988 to 1992. The analysis of the morphological characteristics of this anomaly and comparative study of operative methods and mortality according to the morphological classification led to the following results: 1) The location of VSD varied to be subaortic in 40 cases, subpulmonic in 15, doubly committed in 6 and noncommitted in 19 cases. The great arteries were interrelated in D-malposition in 43 cases and L-malposition in 18 and 26 cases among above mentioned 61 cases revealed the side-by-side relationship. Ten of the cases showed normal position and the rest 9, A-malposition. 2) Pulmonary stenosis was found in 60 cases, and when associated with subpulmonic VSD, occurring less frequently. PDA and ASD secundum were associated in successional order and only 3 cases were verified to carry aortic arch anomalies such as coarctation and interruption, all with subpulmonic VSD. 3) Intraventricular tunnel repair was possible in every case associated with subaortic VSD and the postoperative prognosis was excellent. In comparison, most of the cases associated with other types of VSD in which only palliative surgery or various types of intraventricular repair were performed, the general outcome was poor. But total cavopulmonary connection, one of the physiological corrective methods, showing a better postoperative prognosis is being carefully considered for its broad adoptation in future. In conclusion, double outlet right ventricle, a pathophysiological complex of various anomalies, should be thoroughly evaluated for the morphological characteristics to decide the most appropriate types of operation and for consequent improvement of prognosis.
Aorta, Thoracic ; Arteries ; Classification ; Double Outlet Right Ventricle* ; Heart Ventricles ; Mortality ; Palliative Care ; Prognosis ; Pulmonary Valve Stenosis

Two-dimensional Doppler echocardiographic velocity profiles of four cardiac valves in a group of 85 infants and children with left to right shunt lesions(VSD, ASD and PDA) are presented. Velocities were obtained before operation and 10 days afterward. The results were as follows ; 1) VSD(type 2) Peak velocities of mitral valve were significantly decreased when compared preop. with postop. echocardiogram(n=37, preop 132+/-24, postop 98+/-16 cm/sec, p<0.0005). Peak velocities of tricuspid valve were also significantly decreased(n=30, perop 60+/-21, postop 50+/-17cm/sec, p<0.01). Peak velocities of aortic valve were decreased(n=18, perop 120+/-20, postop 108+/-26cm/sec, 0.010.05). Peak velocities of pulmonic valve were significantly decreased(n=17, perop 168+/-46, postop 104+/-51cm/sec, p<0.0005). 3) PDA Peak velocities of mitral valve were significantly decreased when compared perop. with postop. echocardiogram(n=29, perop 138+/-28, postop 111+/-19cm/sec, p<0.0005). Tricuspid valve velocities showed no significant changes between preop. and postop. echocardiogram (n=15, perop 47+/-12, postop 44+/-10cm/sec, p>0.05). Aortic valve velocities showed no significant changes (n=10, perop 134+/-23, postop 121+/-25cm/sec, p>0.05). Peak velocities of pulmonic valve were significantly decreased(n=28, perop 138+/-37, postop 107+/-27cm/sec, p<0.005). This study demonstrates that Doppler echocardiographic assessment of velovity changes are correlated with known hemodynamic data and also will be a good index in the evaluation of operation.
Aortic Valve ; Child ; Echocardiography* ; Echocardiography, Doppler ; Heart Valves* ; Hemodynamics ; Humans ; Infant ; Mitral Valve ; Tricuspid Valve

No abstract available.
Hyperpigmentation*

Bilateral coronary artery fistula is very uncommon congenital anomaly which occupy small percentage of all congenital coronary artery fistula. About 20% of the cases associated with additional congenital heart disease. And it may cause congestive heart failure, especially severely in neonate. We experienced a case of neonate who was 1 day-old-male with bilateral coronary artery-right ventricular fistula associated with atrial septal defect and congestive heart failure. The Patient was admitted because dyspnea, cyanosis since birth. 2D ecgicardiogram and cardiac catheterization revealed that a fistulous communicating ; forming a large aneurysm, was noted from bilateral coronary artery emptied into the right ventricle, and there was oxygen step-up in right ventricle.
Aneurysm ; Cardiac Catheterization ; Cardiac Catheters ; Coronary Vessels ; Cyanosis ; Dyspnea ; Fistula* ; Heart Defects, Congenital ; Heart Failure ; Heart Septal Defects, Atrial* ; Heart Ventricles ; Humans ; Infant, Newborn* ; Oxygen ; Parturition