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Biomedical subjects

R G Weintraub

Publications and source records attributed to R G Weintraub.

At least 19 recordsLinked to original sources

Mechanisms and management of gingival overgrowth in paediatric transplant recipients: a review.

UNLABELLED: Increasing numbers of children are receiving solid organ transplants namely kidney, liver, heart and lung. Patient survival rates following such transplants are essentially good with much of the success attributable to the development of Cyclosporine A (CyA), an immunosuppressive drug, that minimizes organ rejection. However the gingival overgrowth (GO) associated with the use of CyA is not only disfiguring but in paediatric recipients, may interfere with normal oral development and function. OBJECTIVE: The aim of this review is to summarize current knowledge concerning the aetiology, pathogenesis and management of gingival overgrowth. METHODS: Literature pertaining to gingival overgrowth is reviewed with particular reference to the paediatric population. Emphasis is placed on summarizing the evidence pertaining to the effectiveness of intervention. CONCLUSION: CyA undoubtedly causes gingival overgrowth, the effects and levels of which appears to be more severe in younger patients. There is conflicting evidence as to the effectiveness of oral hygiene regimes, antibiotics and surgery in reducing overgrowth. The introduction of an alternative immunosuppressive agent (Tacrolimus) offers potential as it does not appear to cause overgrowth, although research to date is limited by the small sample size of many of the studies. This is an area in which multicentre studies would be of great value.

Child↗

Long term somatic growth after repair of tetralogy of Fallot: evidence for restoration of genetic growth potential.

OBJECTIVE: To compare actual with predicted long term growth after early repair of tetralogy of Fallot (TOF). DESIGN: Serial preoperative and postoperative anthropometric data were converted with z scores. The presence of restrictive physiology was assessed by echocardiography. PATIENTS: 45 otherwise healthy patients who underwent repair at median age 1.6 years (range 0.2-4.9) were studied. Predicted height was determined from mid-parental height corrected for sex. RESULTS: Mean (SD) weight and height z scores at the time of surgery were significantly depressed (-1.04 (0.82) and -0.93 (0.95), respectively; p < 0.0001 for both). At latest follow up at a median age of 14.2 years (range 11-20.5), mean weight and height z scores were 0.16 (1.1) and -0.05 (0.81) (p = 0.32 and p = 0.41, respectively). The improvement between surgical and late weight and height z scores was significant (p < 0.0001 for each comparison). Catch up growth was largely complete within two years. Age at correction, duration of follow up, and prior surgical procedures were unrelated to growth. Mean current height z scores were similar to those predicted by mid-parental height. Patients with restrictive right ventricular physiology (n = 24) had a significantly greater late z score for weight (0.49 v -0.34; p = 0.01), with a similar trend for height. Low birth weight patients experienced comparable catch up growth but remained shorter than patients with normal birth weight (mean height z score -0.64 v 0.06; p = 0.03). CONCLUSIONS: Early repair of TOF results in significant acceleration of weight and height, with normalisation of long term growth and fulfilment of genetic growth potential.

Adolescent↗

Clinical, electrocardiographic, and histologic correlations in children with dilated cardiomyopathy.

OBJECTIVE: To determine whether presenting electrocardiography is related to histologic findings and clinical outcomes in children with dilated cardiomyopathy. BACKGROUND: Lymphocytic myocarditis is an important cause of childhood dilated cardiomyopathy, the outcome of which is unclear. The results of non-invasive investigations are often used to infer the presence or absence of lymphocytic myocarditis. METHODS: Thirty-four children, presenting acutely with dilated cardiomyopathy, underwent both early electrocardiography and endomyocardial biopsy. The parameters examined included heart rate, PR, QRS, and corrected QT intervals, R-wave voltages in Leads V(1) and V(6), S-wave voltages in Leads V(1) and V(6), and sum of SV(1) and RV(6). We expressed measurements as Z scores, based on published normal values for age and gender. RESULTS: A total of 15 patients had lymphocytic myocarditis on endomyocardial biopsy (Group I), and 19 had non-specific histologic findings (Group II). We did not distinguish the 2 groups by age, time to endomyocardial biopsy, or duration of follow-up. Group I patients had significantly smaller R-wave Z scores in Leads V(1) and V(6), and combined S in V(1) and R in V(6) Z scores (p < 0.02 for each). The positive and negative predictive values of an R-wave amplitude in V(6) < 5th percentile were 75% and 65%, respectively, for the diagnosis of lymphocytic myocarditis. An R-wave amplitude in V(6) > 95th percentile had a positive and negative predictive value of 80% and 63%, respectively, for the diagnosis of idiopathic dilated cardiomyopathy. Survival and freedom from late cardiac dysfunction were more common among Group I patients compared with Group II (p <or= 0.02 for both). CONCLUSION: Myocardial histology cannot reliably be inferred from the presenting electrocardiogram. Survival and outcome for children with lymphocytic myocarditis is better than for those with non-specific histology.

Biopsy↗

Pulmonary vascular resistance and reactivity in children with end-stage cardiomyopathy.

Pulmonary vascular resistance (PVR) and reactivity were compared in 63 children with end-stage cardiomyopathy (CM) referred for cardiac transplantation. Diagnostic category of CM was the sole determinant of PVR. Compared with other patients, children with restrictive CM were younger at diagnosis and had a significantly higher pulmonary vascular resistance index (PVRI). Children with a baseline PVRI of up to 11.8 units per meter squared (U.m(2)) who showed reactivity underwent successful orthotopic cardiac transplantation.

Adolescent↗

Persistent truncus arteriosus in monozygotic twins: case report and literature review.

We report on a pair of monozygotic twins with persistent truncus arteriosus. They had no evident clinical signs of DiGeorge syndrome. Pathologic examination of the placenta and DNA analysis in chromosomes 7, 8, and 15 was consistent with monozygosity. Fluorescence in situ hybridization test was negative for chromosome 22q11 microdeletion. Family history revealed a female cousin with tetralogy of Fallot. The isolated presence of this conotruncal abnormality in monozygotic twins is extremely rare. The genetic considerations are discussed.

Diseases in Twins↗

Relationship of the dimension of cardiac structures to body size: an echocardiographic study in normal infants and children.

Normalization of the dimensions of cardiac structures to the size of the body, using so-called Z scores, is becoming increasingly common in the management of infants and children with congenital heart disease. Current published nomograms for the ascertainment of Z scores for cardiac structures in childhood are based largely on normal data obtained in formalin-fixed hearts. Since decisions concerning management are frequently based on the findings of cross-sectional echocardiograms, the dimensions of 15 cardiac structures were measured using cross-sectional echocardiography in 125 normal infants and children. Regression equations were derived relating cardiac dimensions to the size of the body. The expression of size with the highest correlation to cardiac dimensions was body surface area. Nomograms were then developed from which the Z score of a cardiac structure could be estimated from a knowledge of the body surface area and the echocardiographically derived measurement.

Adolescent↗

Hypoxic-ischaemic encephalopathy is associated with regional changes in cerebral blood flow velocity and alterations in cardiovascular function.

Doppler-derived indices of cerebral blood flow velocity (CBFV) and echocardiographic parameters of left ventricular function were measured in 18 patients with hypoxic-ischaemic encephalopathy HIE (group I) and in 28 normal controls (group II). Group-I infants had a subnormal distribution of CBFV values increasing over the first 85 h postnatally. CBFV values were constantly higher in the internal carotid than in the anterior cerebral artery. During the first 24 h postnatally, pulsatility and resistance indices of cerebral blood flow were significantly higher in group-I patients. From 30 to 85 h after birth, resistance indices were lower in group-I infants with severe HIE. Depressed left ventricular function and/or hypotension was documented in 50% of group-I patients.

Asphyxia Neonatorum↗

Anatomic features and surgical strategies in double-outlet right ventricle.

BACKGROUND: The objective of this study was to review anatomic features and surgical strategies in children with double-outlet right ventricle (DORV) and to assess risk factors for early mortality. METHODS AND RESULTS: Records were reviewed of all children with DORV undergoing surgery between 1978 and 1993. Noncomplex patients (group 1) had atrioventricular (AV) concordance, a single ventricular septal defect (VSD), balanced ventricles, no straddling AV valves, and no major pulmonary artery anomaly. Group 2 (complex) comprised all remaining patients. Independent risk factors analyzed included location of the main VSD, presence of additional VSDs, coarctation, ventricular outflow obstruction, ventricular hypoplasia, age at operation, operation before 1985, previous palliation, and type of definitive operation. Of 193 patients, 117 were in group 1 and 76 in group 2. In 148 patients, biventricular repair was undertaken, including 111 of 117 group 1 patients and 37 of 76 group 2 patients. Early mortality was higher among group 2 patients undergoing biventricular repair than among group 1 patients (8 of 37 versus 4 of 111, P<.005) and higher than group 2 patients undergoing a Fontan procedure (none of 29, P<.01). Aortic arch obstruction, operation before 1985, and multiple VSDs were significant risk factors for mortality. Age <1 month (P<.05) and multiple VSDs (P<.005) were independent risk factors after definitive repair. Up-to-date follow-up is available on 144 surviving patients, with 127 (88%) in New York Heart Association class I and the remaining 17 in class II. Overall 10-year survival probability was 81%, whereas probability of survival, free from reoperation (after definitive surgery), was 65% at 10 years. CONCLUSIONS: Biventricular repair can be achieved in most patients with DORV with low risk. In complex DORV, a Fontan procedure is associated with a lower surgical mortality.

Adolescent↗

Senning plus arterial switch operation for discordant (congenitally corrected) transposition.

BACKGROUND: Congenitally corrected transposition of the great arteries is a complex cardiac lesion, usually associated with ventricular septal defect, left ventricular outflow tract obstruction, and tricuspid valve abnormalities. A subset of patients without left ventricular outflow tract obstruction have undergone Senning plus arterial switch operation in an attempt to place the left ventricle in the systemic circuit and the right ventricle in the pulmonary circuit. METHODS: Fourteen patients have had the operation performed since July 1989. Age and weight medians were 12 months (range, 0.5 to 120 months) and 8.2 kg (range, 3.2 to 34 kg). All but 1 patient had a left ventricular to right ventricular pressure ratio greater than 0.7, due to a large ventricular septal defect (with or without a previous pulmonary artery band), severe congestive heart failure caused by right ventricular dysfunction and tricuspid insufficiency, or a pulmonary artery band for left ventricular retraining. At least 10 patients had strong contraindications to "classic" repair, including right ventricular hypoplasia (n = 2), moderate to severe right ventricular dysfunction (n = 5), or moderate to severe tricuspid insufficiency (n = 9). RESULTS: There was one hospital death, occurring in a neonate (7%; 95% confidence interval = 0% to 34%). Actuarial survival beyond 10 months is 81% (95% confidence interval = 42% to 95%), currently with 389 patient-months of total follow-up time. The median grade of tricuspid insufficiency fell from 3/4 preoperatively to 1/4 postoperatively (p = 0.003). Right ventricular function is normal in 11/12 current survivors, all but 1 of whom are in New York Heart Association class I or II. CONCLUSIONS: Senning plus arterial switch operation is a good option for selected patients with congenitally corrected transposition of the great arteries with a similar or lower early risk (as compared with classic repairs). Some of the long-term problems associated with congenitally corrected transposition of the great arteries may be avoided with this strategy.

Cardiac Surgical Procedures↗

Gingival overgrowth in pediatric heart and heart-lung transplant recipients.

BACKGROUND: Gingival overgrowth is a recognized side effect of cyclosporine therapy with cosmetic and functional sequelae. This study examines the incidence and severity of gingival overgrowth in pediatric heart and heart-lung transplant recipients. METHODS: Thirty-one pediatric heart and heart-lung transplant recipients underwent a comprehensive dental evaluation. The severity of gingival overgrowth was scored by use of dental plaster casts. Parameters of dental hygiene in each patient included both a plaque index and a gingival inflammation index. The mean cyclosporine level and daily dose (mg/kg/day) at 3 and 12 months after transplantation and at latest follow-up were determined. RESULTS: The mean (+/- SD) patient age at transplantation was 10.5 (+/- 5.5) years, and the mean duration of posttransplantation follow-up was 3.2 (+/- 2.1) years. In all 30/31 (97%) of the cohort had some degree of overgrowth, with children aged less than 10 years at time of transplantation the most severely affected. By univariate analysis gingival overgrowth was inversely related to age at time of transplantation (r = -0.67, p < 0.001). With multiple regression analysis, only age at transplantation was significantly related to gingival overgrowth. CONCLUSIONS: Gingival overgrowth occurs in most pediatric heart and heart-lung transplant recipients treated with cyclosporine and is most severe in the younger patients. Attention to oral hygiene may improve gingival health parameters; however, the daily weight-adjusted dose of cyclosporine is not related to the severity of overgrowth.

Age Factors↗

Myocarditis in children with dilated cardiomyopathy: incidence and outcome after dual therapy immunosuppression.

BACKGROUND: The true incidence and prognosis of myocarditis in children with acute dilated cardiomyopathy (DCM) at presentation remains uncertain. This study examines the incidence of lymphocytic myocarditis in a consecutive cohort of children with acute DCM at presentation and outcome after dual therapy immunosuppression with cyclosporine and steroids. METHODS: Twenty-nine consecutive children with acute DCM underwent early endomyocardial biopsy. Children with "definite" myocarditis comprised group I (n = 9) and were treated with cyclosporine and prednisolone. Group II (n = 2) had "borderline" myocarditis, and group III (n = 18) nonspecific histologic findings. Outcome was assessed by echocardiographic measurement of left ventricular end-diastolic dimension and fractional shortening, with follow-up endomyocardial biopsy in group I subjects. RESULTS: Myocardial inflammation with or without myocardial necrosis (groups I and II) was present in 38% of all cases. There were no initial clinical, electrocardiographic, or echocardiographic features to distinguish patients in group I from patients in group III. At presentation, the mean +/- SEM left ventricular end-diastolic dimension and fractional score-Z scores of group I patients were 4.6 +/- 1.7 and -5.1 +/- 0.8, respectively, compared with 0.8 +/- 0.3 and -0.9 +/- 0.4, respectively, at withdrawal of immunosuppression (p < 0.001 for both). Both of these parameters did not differ significantly from normal controls at least follow up. Two group I patients had a biopsy-proven relapse after withdrawal of therapy that responded to reinstitution of immunosuppression. At latest follow-up, all nine group I patients had regained normal left ventricular function compared with four of 18 group III patients (p < 0.001). CONCLUSION: Lymphocytic myocarditis is frequent in children with dilated cardiomyopathy and cannot be predicted from noninvasive investigations. The use of cyclosporine and steroids is associated with a favorable outcome, and a controlled trial of dual therapy immunosuppression in children is therefore warranted.

Anti-Inflammatory Agents↗

Surgical commissurotomy of the aortic valve: outcome of open valvotomy in neonates with critical aortic stenosis.

Early intervention is necessary in neonates with critical aortic stenosis. The advent of alternate therapy, particularly balloon aortic valvuloplasty, requires a reappraisal of the traditional surgical approach, including the efficacy of initial transvalvar gradient reduction and freedom from recurrence of obstruction in the longer term. This report describes a series of 33 consecutive infants who underwent surgical aortic valvotomy in the first month of life. The hospital mortality was 18 percent with a 5-year probability of survival of 66 percent (90 percent CI, 50 percent to 79 percent). Fourteen reinterventions, nine reoperations and five balloon dilatations, were required at a median age of 0.8 years (range 9 days to 6 years). Three patients died after reintervention (one early and two late). The median follow-up time was 5.8 years (range 0.2 to 14 years). At last follow-up Doppler investigation the average Doppler mean and peak gradients were 34 mm Hg and 18 mm Hg, respectively. Open valvotomy in neonatal aortic valve stenosis allows the use of an appropriate surgical approach with low initial mortality and satisfactory 5-year freedom from reintervention.

Aortic Valve Stenosis↗

Heart transplantation for chyloptysis after Fontan operation.

Chyloptysis is a rare clinical problem, fewer than 10 patients having been reported in the literature. We report a patient with intractable chyloptysis associated with a Fontan procedure, who was palliated by heart transplantation.

Bronchial Fistula↗

Fetal echocardiography.

Fetal echocardiography has provided a means for the detailed assessment of cardiac structure and function from early prenatal life. Indications for a fetal echocardiographic examination and the potential implications for individuals caring for newborns with cardiac malformations are discussed.

Echocardiography↗

Long-term growth following neonatal anatomic repair of transposition of the great arteries.

Despite generally normal prenatal growth, surviving infants with transposition of the great arteries (TGA) frequently develop severe and progressive growth impairment which is not always fully reversed by elective atrial repair within the first year of life. This study was undertaken to determine the effect of neonatal anatomic repair of TGA on long-term growth. Twenty-three children with uncomplicated TGA were followed for a mean of 60 (12-90) months after anatomic repair at a mean age of 11 (1-40) days. Standardized measurements of weight, height, and head circumference for both patients and normal siblings were expressed as percentiles as well as in Z scores (in standard deviations from the mean for age and sex) based on internationally recognized standards. At latest follow-up, 22 (96%) of the patients were above the 3rd percentile for weight and 21 (91%) for both height and head circumference, with 13 (57%), 11 (48%), and 13 (57%) above the 50th percentile for each respective parameter. The mean Z scores (+/- SD) for weight, height, and head circumference for the patient group were -0.1 +/- 1.2, -0.2 +/- 1.3, and -0.1 +/- 1.1, respectively, and did not differ significantly from those of the reference population (p > 0.05 for each comparison). Paired comparisons of mean Z scores for each growth parameter with those of 35 normal siblings demonstrated no significant difference for weight or height and a small but significant difference for head circumference. Age at surgical repair (within the first 6 weeks of life), duration of follow-up and the development of moderate supravalvar pulmonary stenosis were not statistically related to long-term growth.(ABSTRACT TRUNCATED AT 250 WORDS)

Birth Weight↗

Accuracy of flow convergence estimates of mitral regurgitant flow rates obtained by use of multiple color flow Doppler M-mode aliasing boundaries: an experimental animal study.

The proximal flow convergence method of multiplying color Doppler aliasing velocity by flow convergence surface area has yielded a new means of quantifying flow rate by noninvasively derived measurements. Unlike previous methods of visualizing the turbulent jet of mitral regurgitation on color flow Doppler mapping, flow convergence methods are less influenced by machine factors because of the systematic structure of the laminar flow convergence region. However, recent studies have demonstrated that the flow rate calculated from the first aliasing boundary of color flow Doppler imaging is dependent on orifice size, flow rate, aliasing velocity and therefore on the distance from the orifice chosen for measurement. In this study we calculated the regurgitant flow rates acquired by use of multiple proximal aliasing boundaries on color Doppler M-mode traces and assessed the effect of distances of measurement and aliasing velocities on the calculated regurgitant flow rate. Six sheep with surgically induced mitral regurgitation were studied. The distances from the mitral valve leaflet M-mode line to the first, second, and third sequential aliasing boundaries on color Doppler M-mode traces were measured and converted to the regurgitant flow rates calculated by applying the hemispheric flow equation and averaging instantaneous flow rates throughout systole. The flow rates that were calculated from the first, second, and third aliasing boundaries correlated well with the actual regurgitant flow rates (r = 0.91 to 0.96). The mean percentage error from the actual flow rates were 151% for the first aliasing boundary, 7% for the second aliasing boundary, and -43% for the third aliasing boundary; and the association between aliasing velocities and calculated flow rates indicates an inverse relationship, which suggests that in this model, there were limited velocity-distance combinations that fit with a hemispheric assumption for flow convergence geometry. The second aliasing boundary with an aliasing velocity, of 102 cm/sec, (which was achieved by use of a 4 kHz pulse repetition frequency, a 3.75 MHz transducer, and no color baseline shift), provided the closest fit to the actual regurgitant flow rates (r = 0.99; y = 0.95x + 0.07). The averaged calculated flow rates from all aliasing velocities also resulted in excellent correlation (r = 0.97; y = 0.99x + 0.5). A hemispheric flow convergence method that is based on color Doppler M-mode echocardiography is a feasible and automatable method for quantifying mitral regurgitant rate.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗