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Multiple, bilateral renal carcinomas are a frequent occurrence in von Hippel-Lindau (VHL) disease. To elucidate the aetiological role of the VHL gene in human kidney tumorigenesis, localized and advanced tumours from 110 patients with sporadic renal carcinoma were analysed for VHL mutations and loss of heterozygosity (LOH). VHL mutations were identified in(More)
BACKGROUND Mechanical circulatory support in intractable heart failure in children has been limited to centrifugal pumps and extracorporeal membrane oxygenation: Since 1990 small adult-size pulsatile air-driven ventricular assist devices "Berlin Heart" (VAD) and, since 1992 miniaturized pediatric VAD (12, 15, 25, 30 mL pumps), have been used in our(More)
Mutational inactivation and allelic loss of the von Hippel-Lindau (VHL) gene appear to be causal events for the majority of spontaneous clear-cell renal carcinomas. We now show that hypermethylation of a normally unmethylated CpG island in the 5' region provides another potentially important mechanism for inactivation of the VHL gene in a significant(More)
BACKGROUND Controversy continues to surround determining which is the most beneficial method of complete atrioventricular septal defect repair, eg, one- versus two-patch repair, closure of mitral cleft, and the necessity of annuloplasty. METHODS Between January 1988 and November 1995, 120 patients with complete atrioventricular septal defect underwent(More)
Mammalian flap endonuclease-1 (FEN-1) is a structure-specific metalloenzyme that acts in processing of both the Okazaki fragments during lagging strand DNA synthesis and flap intermediates during DNA damage repair. We identified and cloned three open reading frames encoding a flap endonuclease from Archaeglobus fulgidus, Methanococcus jannaschii, and(More)
OBJECTIVE A modified technique for tricuspid valve repair in Ebstein's anomaly restructures the valve mechanism at the level of the true tricuspid anulus by using the most mobile leaflet for valve closure without plication of the atrialized chamber. Midterm results of this therapeutic approach for patients with Ebstein's anomaly and tricuspid valve(More)
The various techniques of preserving the chordal apparatus in mitral valve replacement applied in 1,453 cases at a single institution between 1986 and 1994 are described. The techniques include preservation of chordal attachment to the posterior mitral leaflet only and to both leaflets with five different technical variations. The specific advantages and(More)
AIMS To evaluate complications of surgical and pharmacological treatment of symptomatic patent ductus arteriosus (PDA) in very low birthweight (VLBW) infants. PATIENTS AND METHODS Of 931 VLBW infants consecutively admitted 1987-1998, a significant PDA prompted first-choice treatment by indomethacin in 101 infants, and surgery in 55 infants. PDA closed or(More)
BACKGROUND Implantation of mechanical cardiac support systems (MCSS) in patients with idiopathic dilated cardiomyopathy (IDC) may improve cardiac function and allow explantation of the device. We report of long-term effects of ventricular unloading on cardiac function, humoral anti-beta1-adrenoceptor autoantibodies (A-beta1-AABs), and myocardial fibrosis.(More)
OBJECTIVE Cardiac recovery in end-stage idiopathic dilated cardiomyopathy recently occurred after temporary support with a left ventricular assist device. We report the case histories of patients who underwent removal of the device more than 4 years ago. METHODS Since June 1994, 23 patients with end-stage idiopathic dilated cardiomyopathy who were(More)