Surgical treatment of children and adolescents with Wolff-Parkinson-White syndrome.
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Biomedical subjects
Publications and source records attributed to W H Fleming.
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The effect of human GH (hGH) on c-myc gene expression and cell proliferation in the Nb2 lymphoma cell line was examined. The addition of hormone to stationary cultures of Nb2 cells resulted in an increased accumulation of c-myc transcripts that was detectable within 15 min, reached a maximum induction of 25-fold in 3 h, and then gradually declined. Although maximal accumulation of c-myc transcripts occurred within 3 h after hGH addition, removal of the hormone even after 4 h of treatment resulted in a failure of the cells to proliferate. This loss of proliferative capacity following hormone removal was accompanied by a 20-fold reduction in the level of c-myc transcripts. These results indicate that hGH induced proliferation of Nb2 cells is associated with the induction and maintenance of c-myc gene expression.
A 16-year-old boy developed marked calcific stenosis of an Ionescu-Shiley valve that was implanted in the aortic position 42 months before his sudden death. Renal hemosiderosis caused by intravascular hemolysis was quantitated by atomic absorption spectrophotometry. The accelerated calcification of the bovine pericardial valve in our patient supports the view that, in general, xenografts should not be used in adolescents. Frequent follow-up evaluations are imperative in young patients who receive these valves to avoid the irrevocable progression of valvular stenosis and hemolytic anemia.
Prostaglandins and ductal formalin infiltration, singly and together, have been used in efforts to improve pulmonary flow in very ill newborn infants with right ventricular outflow tract obstruction. To evaluate the efficacy of concurrent use of prostaglandins and ductal formalin infiltration, we have reviewed our experience with 25 infants with right ventricular outflow tract obstruction and prostaglandin-ductal formalin infiltration therapy. Prostaglandin therapy was begun 22 +/- 21 hours (range 20 to 93 hours) before and was continued 20 +/- 18 hours (range 0 to 62 hours) following ductal formalin infiltration; prostaglandin administration was initiated at a dose of 0.05 to 0.1 microgram/kg/min and tapered postoperatively. Clinical cyanosis was diminished in 20 of 25 infants (80%) postoperatively. Systemic arterial pH and oxygen saturation both improved following prostaglandin-ductal formalin infiltration therapy from 7.35 to 7.41 (p less than 0.001) and from 35.7 to 50.3 (p less than 0.001), respectively. Persistent ductal patency (mean 219 +/- 191 days) was observed in 17 survivors of the early postoperative period (more than 14 days). Two of five infants who died within 14 days of operation had a widely patent ductus with resultant progressive congestive heart failure. The other three infants died as a result of operative technical problems, dysrhythmias, and thrombotic ductal closure, respectively. No correlation was observed between duration of ductus patency and operatively determined size of ductus, total prostaglandin dose, or duration of prostaglandin infusion. Secondary operative intervention was delayed by 92 +/- 74 days with prostaglandin-ductal formalin infiltration therapy; thus prostaglandin-ductal formalin infiltration therapy may have a role in selected neonates with right ventricular outflow tract obstruction.
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Prostaglandin (PGE1) may be used to maintain ductal patency in the infant with cyanotic congenital heart disease, but the risk of infection may be increased. Between October, 1976 and December, 1982, 38 neonates with complex cyanotic congenital heart disease required operations creating systemic-to-pulmonary artery shunts. Of 13 patients who did not receive PGE1 therapy, none developed a wound infection. Of 25 patients who did receive PGE1 therapy, four (16 percent) developed a significant wound infection. The two patient groups were similar when compared by age and weight at operation, by severity of heart disease and by the presence of other congenital anomalies. Pathogenic Staphylococcus epidermidis was recovered from all infected wounds, all of which responded favorably over a period of two to four weeks with a short course of antibiotics and wound debridement.
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Ventricular arrhythmia originating from the outflow tract of the right ventricle is a presumed cause of late sudden death in patients after repair of tetralogy of Fallot. Exercise testing has been shown to enhance detection, and phenytoin has been shown to control ventricular arrhythmias in these patients. This study reports new findings in 3 patients who underwent electrophysiologic studies at postoperative cardiac catheterization; in each, sustained ventricular tachycardia was induced and found to originate from the inflow-septal area of the right ventricle. Using serial studies, the same sustained ventricular tachycardia was induced during therapeutic serum concentrations of phenytoin but not after propranolol. No patient had ventricular arrhythmia during a 24-hour electrocardiogram or during exercise. Although no patient had normal hemodynamic function, only 1 patient had right ventricular pressure greater than two-thirds systemic pressure. Each patient had undergone initial intracardiac repair at a relatively late age (3, 9, and 9 years).
Three children with a pacemaker sustained similar lead fractures within a two-year period. Each fracture occurred in the corkscrew portion of a Medtronic Model 6917 epicardial lead. All patients were boys. The leads had been in place for 12, 45, and 43 months prior to fracture. None of the patients had sustained major trauma. Fracture of the corkscrew lead has been considered an uncommon complication. However, three lead fractures in the corkscrew area in a population of 60 children followed at this institution indicate that this may be a more common cause of pacemaker-system malfunction than previous data suggest. A plea is made for reporting all pacing failure to the manufacturer.
The aortic arch anatomy of the premature infant may not be as simple to determine as the anatomy of an older child. A case of a premature infant who had inadvertent ligation of the left pulmonary artery during attempted ligation of a patent ductus arteriosus is described. On the fourth day following the initial operation, the patient underwent successful surgery to remove the pulmonary artery ligature and to ligate the persistent patent ductus arteriosus. A lung scan on the seventh day following removal of the pulmonary artery ligature showed normal perfusion of both lungs. Six months later, the child's growth, development, and chest roentgenogram were normal.
Left ventricular apical-abdominal aortic valved conduit has been a successful procedure for relief of severe left ventricular outlet obstruction. Postoperatively, unusual deformities of the gastric fundus and cardia may be seen on the upper gastrointestinal series and should not be mistaken for pathologic mass lesions. Three deformities are characteristic: anterior impression on the gastric fundus, medial impression on the gastric fundus, and narrowing of the distal esophagus. Occasional gastrointestinal complications including dysphagia and gastric erosion may occur.
Blast cells in acute leukemia and lymphoma appear to be "frozen" at various stages of lymphoid cell differentiation. The enzymatic and antigenic phenotypes expressed by these cells often correspond to the gene products of their normal precursors. We have used various immunocytochemical and enzymatic techniques to identify membrane, nuclear, and cytoplasmic markers associated with the prolactin-dependent Nb2 lymphoma cell line. The Nb2 cells, whether stationary or in log-phase growth, did not express any surface immunoglobulin. However, 100% of the Nb2 cells bound both a monoclonal antibody raised to rat thymocyte W3/25-HLK, which specifically binds an antigenic determinant on rat T-helper cells, and second monoclonal antibody OX8-HL, which identifies rat nonhelper T-cells. Transmission electron microscopy showed no evidence of phagocytic vacuoles, and activity of the lysosomal enzyme muramidase was also absent. There was no evidence of the DNA polymerase enzyme terminal deoxynucleotidyl transferase. alpha-Naphthyl acetate esterase activity was indicated in about 50% of the Nb2 cells by a faint particulate cytoplasmic staining similar to that found in thymocytes. Rosette formation with guinea pig erythrocytes, a property of mature rat thymocytes, was not observed with Nb2 cells. The data suggest that the Nb2 tumor may have arisen from a thymocyte at an intermediate stage of differentiation. The presence of Thy-like alpha-naphthyl acetate esterase pattern and the binding of both W3/25-HLK and OX8-HL support the thymic origin and relative immaturity of these lymphoid cells. It is becoming increasingly apparent that a significant proportion of lymphomas and leukemias also originate in undifferentiated thymic cels.
Permanent pacemakers were implanted in 50 children. Indications were symptomatic sinus node dysfunction in 34 (68%), surgical block in 9 (18%), and congenital block in 7 (14%). Twenty-three (68%) of the 34 children with sinus node dysfunction had undergone prior cardiac operations. Only 4 of the 50 patients (8%) had electrode problems after a mean pacing time of 29.5 months (range, 1 to 96 months). All 35 of the mercury-cell pulse generators used in 28 patients ceased to function after an average useful life of 20.8 months (range, 1 to 51 months). The lithium-powered units in the 45 survivors all show satisfactory pacing after 5 to 44 months (mean, 28.1 months). With improved pacemaker technology, longer survival after complex repairs, and better monitoring techniques, the indications for cardiac pacing in children have broadened. Surgical block now is an indication in only a small fraction of the pediatric pacemaker population. Sinus node dysfunction accounts for an ever-increasing majority of the pacemakers we currently implant in children.
Recently it was demonstrated that certain fungal pathogens could be rapidly and accurately identified on the basis of their exoantigens. Mycelial-form cultures of the Histoplasma species consistently appear to produce either H or M antigens, or both, whereas cultures of Coccidioides immitis produce tube precipitinogen, heat-labile precipitinogen, or heat-labile F antigens. Three laboratories each evaluated 48 cultures by the exoantigen procedure for the identification of Histoplasma spp. and C. immitis. One hundred forty-four cultures were tested, and 143 (99.3%) were correctly identified as Histoplama spp. or C. immitis, or neither. The simple and rapid exoantigen test appears to be a sensitive and specific method for identification of these fungi.
Twenty-three infants less than age 3 months (mean age 31 days) underwent patch aortoplasty for relief of coarctation of the aorta. All had intractable congestive heart failure, despite aggressive medical therapy. Each infant had other cardiac anomalies, including patent ductus arteriosus (83 percent) and ventricular septal defect (74 percent). All patients underwent closure of the ductus arteriosus and patch angioplasty of the aorta to produce a luminal diameter of at least 16 mm. In addition, 9 of the 17 patients (53 percent) with a large shunt ventricular septal defect underwent pulmonary arterial banding. There was one hospital death 42 days after operation secondary to bowel perforation and sepsis. Hospitalization beyond 21 days postoperatively was always due to other unrepaired cardiac lesions. The three late deaths at 3, 9 and 18 months after operation were associated with additional major anomalies. Fourteen patients have had postoperative catheterization. No gradient was found across the site of coarctation repair, but one patient had a gradient between the left carotid and left subclavian arteries. Surgical repair of critical coarctation of the aorta in infants can safely be offered despite the presence of other cardiac anomalies.
Between May, 1975, and June, 1977, we surgically repaired an infracardiac total anomalous pulmonary venous return in 4 infants under deep hypothermic cardiac arrest. All patients had pulmonary hypertension and a patent ductus arteriosus. All survived operation and required positive end-expiratory pressure with mechanical ventilation. Late complications included patent ductus arteriosus not visualized at initial catheterization, breakdown of an oversewn atrial septal defect repair, and pulmonary venous obstruction despite an anastomosis diamter of 1.5 to 2.5 cm. At reoperation, there was scarring and contraction at the junction of the pulmonary veins and left atrium. One infant with these complications died post-operatively. In another patient, there was kinking of the left lower pulmonary vein at its juncture with the left atrium. In view of these complications we currently recommend the following: ligation of the ductus arteriosus regardless of catheterization findings; a right-sided approach to avoid pulmonary vein kinking; prosthetic patch closure of the atrial septal defect to avoid excessive tension on suture lines and to maintain maximum left atrial size; stellate anastomosis to provide the largest possible drainage channel; awareness that an unusually high positive end-expiratory pressure may be required postoperatively; and early recatheterization.
In order to improve the isolation and identification of yeasts in a cancer research hospital, a protocol was developed utilizing an improved blood culture methodology and a four-test schema for rapid yeast identification. The blood culturing technique, based upon centrifugation, has shown a ten-fold increase in isolation of fungi from blood and has provided for: quantitation or organisms, unlimited selection of media and atmospheres for primary culturing, and a 1:200 dilution of microorganisms away from serum antimicrobial factors and antibiotics. The four-test schema, which may be adapted for the identification of any unknown yeast in pure culture, consists of a dye pour plate auxanogram (DPPA), Tween 80-Oxgall-Caffeic acid (TOC), a rapid nitrate-reductase test (swab test) and Urea 'R' Broth. Using this protocol, over 95% of the clinical isolates received were correctly identified within 24 hours and 100% by 48 hours. By using DPPA, a 14 sugar assimilation pattern for each isolate was determined within 12 to 16 hours; and in some cases, as little as 6 hours. Growth on TOC yielded one of the following results: (1) Candida albicans and Candida stellatoidea sequentially produced germ tubes and chlamydospores in 3 hours and 24 hours, respectively; (2) Cryptococcus neoformans produced a brown pigment specific for its identification in 12 hours or less. The swab test gave results on nitrate utilization in less than 15 minutes and urease was detected within 4 hours.