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Biomedical subjects

K S Kuehl

Publications and source records attributed to K S Kuehl.

At least 19 recordsLinked to original sources
PubMed2000

Insurance and education determine survival in infantile coarctation of the aorta.

Health outcomes are determined by case severity, physician decisions, and patient variables. In a population-based study between 1981 and 1989, 103 cases of infant coarctation of the aorta were diagnosed before one year of age. The goal of this study was to determine whether patient race, gender, income, and insurance status had effects on outcome of coarctation of the aorta that were distinct from the effect of case severity. Survival of infants with coarctation of the aorta, a common congenital cardiovascular malformation, is associated with greater maternal education and with having any health insurance but not with measures of severity. Infants without health insurance are 12.8 times more likely to die than infants with any health insurance. Fifty-five percent of all deaths in infant coarctation occur prior to surgical treatment. One-third of deaths occur without diagnosis. Outcome measures require knowledge of the entire population and of insurance status to inform policy.

Analysis of Variance↗
PubMed1999

Failure to diagnose congenital heart disease in infancy.

OBJECTIVE: To identify factors that predict failure to diagnose congenital heart disease in newborns. DESIGN: All fatal cases in the Baltimore-Washington Infant Study were compiled. The Baltimore-Washington Infant Study includes 4390 cases of infants with congenital cardiovascular malformations identified in a population-based study between 1981 and 1989 in the Baltimore-Washington metropolitan area. Death occurred in 800 such infants in the first year of life. In 76 of these infants, death occurred before diagnosis of heart disease. These cases were identified by community search of autopsy records. Their characteristics are compared with those of infants who died after a cardiac diagnosis was made. RESULTS: Infant characteristics (birth weight, gestational age, intrauterine growth retardation, and chromosomal anomaly) are associated with death of infants with congenital cardiovascular malformations and with death of such infants before diagnosis. Diagnoses of coarctation of the aorta, Ebstein's anomaly, atrial septal defect, and truncus arteriosus are overrepresented in infants found by community search, particularly in those infants without associated malformations. Paternal education is associated with failure to diagnose congenital heart disease in life but other sociodemographic characteristics of the infant's family are not. CONCLUSIONS: Diagnosis of congenital cardiovascular malformations requires close observation in the neonatal period. Analysis of age at death of infants with undiagnosed congenital cardiovascular malformation suggests that such infants may be at risk if discharged within the first 2 days of life.

Age Distribution↗
PubMed1997

Precipitous exit block with epicardial steroid-eluting leads.

Between January 1990 and October 1992, we implanted 16 steroid-eluting ventricular epicardial pacing leads (Medtronic 10295A and 10295B/4965) in 12 patients. There were 8 males and 4 females ranging in age from 3 months to 49 years (mean 8.7 +/- 13.2, median 6.0 years). Structural cardiac disease was present in 11 of 12 patients. Follow-up ranged from 3-73 months postimplant (mean 35.7 +/- 22.3, median 28.5 months). Lead fracture (10295A) occurred in 1 of 12 patients. Of the remaining 11 patients, 8 of 11 have very low long-term pacing thresholds. Unexpectedly, 3 patients demonstrated precipitous threshold increases from 3 months to 3.5 years postimplant. Although no deaths resulted in these exit block patients, 1 of 3 exit block patients developed marked worsening of congestive heart failure. We reviewed and analyzed the data obtained at 4 weeks postimplant for all of the 10295A and 4965 patients in the entire Medtronic clinical study database. Using the criterion of a 4 week postimplant pacing threshold > or = 0.12 ms (5 V), we found that the long-term risk of eventual exit block was 27.3% for the 10295A lead (P = 0.005) and 7.5% for the 10295B/4965 lead (P = 0.03). We, therefore, recommend that in patients implanted with the 4965 steroid-eluting epicardial lead, ventricular pacing thresholds > or = 0.12 ms (5 V) measured at 4 weeks postimplant should prompt frequent threshold testing to detect late and potentially sudden ventricular pacing threshold increases.

Adolescent↗
PubMed1993

Effective control of hypercholesterolemia in children with dietary interventions based in pediatric practice.

BACKGROUND: Based on recent recommendations, the number of hypercholesterolemic children who would require dietary therapy could overwhelm current preventive pediatric cardiology resources. No previous studies have established the efficacy of such therapy in the pediatrician's office. The purpose of this study was to evaluate two programs of office-based therapy. METHODS: We randomly assigned 295 children with hypercholesterolemia (> 185 mg/dl) two interventions: one single or four multiple 90-min sessions of family-oriented nutritional education, based in pediatric practices. We examined total cholesterol, 3-day food records, height and weight, and in the multisession group high-density lipoprotein cholesterol and triglycerides at the beginning and at intervals of 8.5-9 and 21 to 33 weeks (single-session and multisession groups, respectively). RESULTS: Total cholesterol was lowered equally in both treatment groups over the course of the study. This was accompanied by dietary changes: a decrease in calories derived from total and saturated fats, and increased intake of fiber, protein, and carbohydrate. However, more single-session patients withdrew from the program during the study. CONCLUSIONS: The two interventions were equally effective in lowering total and low-density lipoprotein cholesterol and in reducing intake of total and saturated fat. However, the higher completion rate of the multisession group suggests that this approach may be the more effective.

Adolescent↗
PubMed1992

Should a school honor a student's DNR order?

Physicians first present the case of a sixteen-year-old cardiac patient who has refused a heart transplant and cardiac resuscitation. Her family and physicians accept the decision. The family has asked the school system to honor her do not resuscitate order if she goes into cardiac arrest in school The school system has refused to do so. Following the case presentation, a lawyer, a physician/ethicist, and an educator consider the important issues raised by this ongoing case.

Adolescent↗
PubMed1989

Metabolic evaluation of obese and nonobese siblings.

OBJECTIVE: To test the hypotheses that obese adolescents have a lower resting metabolic rate and less aerobic endurance than their nonobese siblings. DESIGN: Case-referent study of obese and nonobese siblings from the same kindred. SETTING: Tertiary referral center. PARTICIPANTS: Telephone screening of community volunteers resulted in a consecutive sample of 16 kindreds. Obese and nonobese siblings were similar in age, height, and pubertal status. Significantly more female subjects were in the obese group (p less than 0.01). MEASUREMENTS AND MAIN RESULTS: Body composition studies revealed that the obese siblings had higher body fat (p less than 0.001) but that fat-free mass was similar to that of the lean siblings. Resting metabolic rates determined by indirect calorimetry for the obese and nonobese pairs did not differ. Although the obese siblings appeared less fit when maximal oxygen consumption was measured in relation to total weight, maximal oxygen consumption did not differ when values were standardized for fat-free mass. CONCLUSIONS: The obese adolescents did not have a reduced resting metabolic rate. As in adults, the relationship between resting metabolic rate and fat-free mass was similar for obese and nonobese children and adolescents. Any decreased sport participation by the obese siblings was not due to inherent reductions in aerobic capacity.

Adipose Tissue↗
PubMed1989

Sustained depression of the resting metabolic rate after massive weight loss.

To assess potential long-term effects of weight loss on resting metabolic rate (RMR), the RMRs of seven obese women were measured by indirect calorimetry before weight loss, during a protein-sparing modified fast, and for 2 mo while at a stable reduced weight. Body composition was also determined at each interval. RMR significantly decreased 22% (p less than 0.01) with initiation of the modified fast. RMR values during the modified fast and during the maintenance diet at stable reduced weight were not different and all were significantly lower than the prediet RMR. Loss of lean tissue could not account for the decrease because changes in RMR per fat-free mass paralleled the total RMR reduction. A sustained decrement in RMR accompanied weight loss and persisted for greater than or equal to 8 wk despite increased caloric consumption and body weight stabilization.

Adult↗
PubMed1989

Metabolic myopathies: evaluation by graded exercise testing.

Exertional muscle pain and fatigue are common complaints; some patients with these symptoms have a metabolic myopathy. We have performed graded exercise testing with analysis of expired ventilation on 13 individuals with various kinds of metabolic myopathies. Their results differed from normal and reflected the underlying biochemical abnormality. Patients with disorders of the mitochondrial electron transport chain demonstrated marked limitations in aerobic metabolism and a greatly reduced maximum oxygen consumption. During intense exertion, normal individuals increase carbon dioxide generation due to buffering of lactic acid. This did not occur in patients with McArdle disease, in whom the respiratory exchange ratio (carbon dioxide production/oxygen consumption) did not rise above 1.0 at maximum exercise. These results indicated a deficit in anaerobic metabolism. Pyruvate dehydrogenase complex allows pyruvate produced from carbohydrate metabolism to enter the citric acid cycle. Patients with this enzyme deficiency showed an initially normal pattern followed by an abrupt cessation in carbohydrate dependent aerobic metabolism at higher work loads. During high-intensity exercise, progressive anaerobic metabolism was not accompanied by additional oxygen consumption. Finally, results from a patient with carnitine palmitoyl transferase deficiency revealed an early dependence on carbohydrate metabolism. The ventilatory threshold occurred at a low percentage of maximal oxygen consumption, reflecting the limited availability of lipid substrates for aerobic metabolism. Detection of some muscle metabolic abnormalities can be made on small biopsy specimens. However, definitive diagnosis of the defect nearly always requires studies on fresh or frozen muscle tissue obtained by an open biopsy. The decision on how the tissue should be processed and which metabolic studies should be performed frequently needs to be made before the biopsy is obtained. Thus, a noninvasive method to initially characterize patients with potential metabolic disorders is useful. Exercise testing with expired gas analysis can indicate the presence of a metabolic myopathy and results can then be used to direct the appropriate biochemical evaluations.

Adolescent↗
PubMed1988

Does aerobic conditioning cause a sustained increase in the metabolic rate?

We measured the metabolic rate by indirect calorimetry for 90 minutes following exercise in six healthy individuals. Ten and 30 minutes of cycling at 80% of maximal intensity produced comparable increases in the resting metabolic rate, (37% and 32%, respectively) immediately after exercise. However, by 30 minutes following exertion, the metabolic rate was not different from control values. The total additional caloric use during the 90 minutes of recovery was similar for the two exercise durations, and the mean increment in recovery energy expenditure was 11.4 +/- 7.1 kcals. The majority of caloric use with exercise is during the activity. Recovery energy expenditure following usual aerobic training results in only a minor contribution to total energy use.

Adult↗
PubMed1988

Assessment of exercise intensity formulas by use of ventilatory threshold.

Guidelines for training heart rate (HR) during aerobic exercise are often determined by predictive formulas. Measurement of the heart rate at ventilatory threshold (VT) by expired gas analysis provides a direct index of the upper limits of conditioning intensity. We evaluated 115 nonsmoking, healthy adults with measurement of peak oxygen uptake to classify groups as low- (n = 45), average-(n = 45), and high (n = 25) -fitness. Heart rate at VT was compared with the approximate midpoint (77 percent) of recommended training intensity as estimated by the Karvonen equation, predicted maximal (220-age), and measured maximal HR formulas. No significant difference among the various HR formulas at 77 percent and HR at VT were found for high-fitness individuals. Among the low- and average-fitness groups, the Karvonen formula at 77 percent was significantly higher (p less than 0.001) than HR at ventilatory threshold. Predicted and measured maximal HR at 77 percent were not above the VT among the low- and average-fitness individuals and are appropriate for training intensity. However, the Karvonen formula appears to overestimate heart rate intensity among those of low and average fitness and may be excessive for these groups.

Anaerobiosis↗
PubMed1985

Focal giant cell cardiomyopathy with Beckwith-Wiedemann syndrome.

Cardiac involvement in Beckwith-Wiedemann syndrome is mostly limited to mild cardiomegaly. Although these patients have visceromegaly, macroglossia, gigantism, and adrenal cytomegaly, no significant myocardial changes have been described. An infant with dysmorphic features of this syndrome had supraventricular tachycardia since birth. Nodular lesions were present in the right atrium. Morphologically these lesions were composed of hypertrophic myocardial fibers admixed with multinucleated giant cells of myogenic origin. The exact nature of these lesions remains undetermined. It is postulated that hypertrophic myocardial cells may represent cardiac cytomegaly as a manifestation of the accelerated growth potential of cells seen with this syndrome.

Beckwith-Wiedemann Syndrome↗
PubMed1984

Repair of anomalous origin of the left coronary artery in the infant and small child.

Anomalous origin of the left coronary artery from the pulmonary artery is associated with myocardial infarction, left ventricular dysfunction, mitral valve dysfunction and, occasionally, intracardiac congenital abnormalities. A technique that utilizes a flap of the anterior wall of the pulmonary artery to serve as a neocoronary artery to direct aortic flow from a created aortopulmonary window to the pulmonary artery orifice of the anomalous left coronary artery was used in five patients aged 2.5 months to 4.75 years. Two patients were less than 4 months of age at operation. There was one death 2 days after operation and one late death. The two youngest patients required mitral valve replacement. Two of the three surviving patients are well at follow-up at 7 to 44 months. One patient has been lost to follow-up study. One patient had postoperative catheterization which showed an intact repair. The pulmonary artery neocoronary procedure is applicable to infants and small patients with anomalous origin of the left coronary artery from the pulmonary artery.

Child, Preschool↗
PubMed1983

Left ventricular false tendons in children: prevalence as detected by 2-dimensional echocardiography and clinical significance.

During a 28-month period, consecutive 2-dimensional echocardiograms were reviewed to determine the prevalence of left ventricular (LV) false tendons, their associated anomalies and clinical significance. LV false tendons were found in 31 (0.8%) of 3,847 consecutive 2-dimensional echocardiograms. Of 31 LV false tendons, 30 passed longitudinally from papillary muscle to septum and 1 went from free wall to free wall. The 31 patients were aged 1 day to 15 years. Associated heart disease, most often ventricular septal defect, bicuspid aortic valve and coarctation of the aorta, was present in 48%, of whom 73% were girls. Of those without heart disease, 69% were boys. In patients with heart disease, precordial murmurs were due to the underlying cardiac anomaly. Of those without heart disease, 15 of 16 (94%) had a precordial murmur, usually of the Still's type over the lower left sternal border. Four of 31 (13%), 1 with and 3 without heart disease, had unifocal premature ventricular contractions that were rate-dependent in the 2 patients undergoing stress testing. LV false tendons appear to occur in 0.8% of pediatric patients and usually are accompanied by a Still's type innocent murmur if unassociated with heart disease. Some LV false tendons are associated with rate-dependent premature ventricular contractions.

Adolescent↗
PubMed1983

Lipoprotein uptake by vascular smooth muscle cells of the rat cultured in 5% or 20% oxygen.

Freshly dispersed aortic smooth muscle cells of the rat were grown in either 5% or 20% oxygen. Cells proliferated more rapidly in 5% than in 20% oxygen although at confluency the protein content per cell was 20% less in the 5% than in the 20% oxygen environment. By electron microscopy, cell morphology was the same in both environments. Uptake of low-density and high-density homologous lipoprotein was unaffected by oxygen tension. Other studies, however, showed that induction of receptors for lipoprotein binding behaved variably in the low compared to the high oxygen condition. These differences were not associated with differences in lipid synthesis in the two conditions.

Animals↗