Nutritional factors associated with survival following enteral tube feeding in patients with motor neurone disease.

BACKGROUND: Motor neurone disease (MND) is a progressive neurodegenerative disease leading to limb weakness, wasting and respiratory failure. Prolonged poor nutritional intake causes fatigue, weight loss and malnutrition. Consequently, disease progression requires decisions to be made regarding enteral tube feeding. The present study aimed to investigate the survival, nutritional status and complications in patients with MND treated with enteral tube feeding. METHODS: A retrospective case note review was performed to identify patients diagnosed with MND who were treated with enteral tube feeding. A total of 159 consecutive cases were identified suitable for analysis. Patients were treated with percutaneous endoscopic gastrostomy (PEG), radiologically inserted gastrostomy (RIG) or nasogastric feeding tube (NGT). Nutritional status was assessed by body mass index (BMI) and % weight loss (% WL). Serious complications arising from tube insertion and prescribed daily energy intake were both recorded. RESULTS: Median survival from disease onset was 842 days [interquartile range (IQR) 573-1263]. Median time from disease onset to feeding tube was PEG 521 days (IQR 443-1032), RIG 633 days (IQR 496-1039) and NGT 427 days (IQR 77-781) (P = 0.28). Median survival from tube placement was PEG 200 (IQR 106-546) days, RIG 216 (IQR 83-383) days and NGT 28 (IQR 14-107) days. Survival between gastrostomy and NGT treated patients was significant (P < or = 0.001). Analysis of serious complications by nutritional status was BMI (P = 0.347) and % WL (P = 0.489). CONCLUSIONS: Nutritional factors associated with reduced survival were weight loss, malnutrition and severe dysphagia. Serious complications were not related to nutritional status but to method of tube insertion. There was no difference in survival between PEG and RIG treated patients.

Natural history and clinical features of the flail arm and flail leg ALS variants.

OBJECTIVE: We sought to define the significance of brachial amyotrophic diplegia (flail arm syndrome [FA]) and the pseudopolyneuritic variant (flail leg syndrome [FL]) of amyotrophic lateral sclerosis (ALS; motor neuron disease). METHODS: We analyzed survival in clinic cohorts in London, UK (1,188 cases), and Melbourne, Australia (432 cases). Survival from disease onset was analyzed using the Kaplan- Meier method and Cox proportional hazards model. RESULTS: In the London cohort, the FA syndrome represented 11% and the FL syndrome 6% of the sample. Median survival was 35 months for limb onset and 27 months for bulbar onset ALS, whereas this was 61 months for FA syndrome (p < 0.001) and 69 months for FL syndrome (p < 0.001). Five-year survival in this cohort was 8.8% for bulbar onset, 20% for limb onset, 52% for FA syndrome, and 64% for FL syndrome. The ratio of men to women was 4:1 in the FA group compared to 2:1 in other limb onset cases. Excluding lower motor neuron FA and FL cases, progressive muscular atrophy comprised 4% of the sample and had a prognosis similar to typical limb onset ALS. In the Melbourne cohort, median survival for limb onset ALS was 31 months, bulbar onset 27 months, FA syndrome 66 months (p < 0.001), and FL syndrome 71 months (p = 0.001). CONCLUSIONS: The flail arm (FA) and flail leg (FL) syndromes had significantly better survival than typical amyotrophic lateral sclerosis (ALS) or progressive muscular atrophy cases that were not classified as FA or FL. Our findings underline the clinical and prognostic importance of the FA and FL variants of ALS.

Sodium excretion and racial differences in ambulatory blood pressure patterns.

The influence of Na+ excretion and race on casual blood pressure and ambulatory blood pressure patterns was examined in a biracial sample of healthy, normotensive children and adolescents (10-18 years; n = 140). The slopes relating 24-hour urinary Na+ excretion to systolic blood pressure were different for both black and white subjects for casual blood pressure (p less than 0.001) and blood pressure during sleep (p less than 0.03). For casual blood pressure, the slope was significant for black subjects (beta = 0.17; p less than 0.001) but not for white subjects. For blood pressure during sleep, the slope was again significant for black subjects (beta = 0.08; p less than 0.01) but not for white subjects. Na+ excretion was also related to awake levels of systolic blood pressure for black subjects (beta = 0.08, r = 0.36; p less than 0.01), although the slopes for both black and white subjects were not significantly different. Further analyses indicated the results were not due to racial differences in 24-hour urinary K+ excretion. However, plasma renin activity was marginally related to Na+ excretion in white subjects (r = 0.22; p less than 0.06) but not black subjects, a finding that is consistent with previous studies. Na+ excretion was not associated with diastolic blood pressure or heart rate in either group under any condition. The results of this study support research that has demonstrated a stronger relation between Na+ handling and casual blood pressure in black subjects and extend these findings to blood pressure while the subject is both awake and asleep.

Children's cardiovascular reactivity: stability of racial differences and relation to subsequent blood pressure over a one-year period.

After measuring blood pressure and heart rate at rest and during a video game procedure in 477 children enrolled in 3rd grade, 434 (91%) children had these measurements repeated a year later in 4th grade. Black children demonstrated greater blood pressure and heart rate reactivity than White children in both years, and an increase in heart rate reactivity from 3rd to 4th grade. Gender effects were inconsistent. Systolic and diastolic blood pressures during the video game were more highly correlated from year-to-year than were the resting measures. Regression analysis indicated that systolic reactivity was significantly related to subsequent systolic pressure at rest, particularly among Black girls. Diastolic reactivity was associated with subsequent resting diastolic pressure only among White children. Associations between reactivity and future blood pressure were independent of initial resting blood pressure. This study suggests that cardiovascular reactivity to psychological stress may be one important factor in future level of blood pressure and that the increased heart rate reactivity of Black children may be associated with the prevalence of hypertension among Black adults.

Determining efficacy of monitoring devices: evaluating new technologies.

We point out the connection between electronic monitoring and repeated screening tests, and suggest a flexible and practical method to determine the efficacy of monitoring devices and new technologies. We point out the benefits of the Mantel-Haenszel estimator of an odds ratio for determining diagnostic test indices. From estimates of sensitivities and specificities, we produce receiver operating characteristic (ROC) curves to compare the performance of two or more monitoring devices in a clinical setting.

Ambulatory blood pressure patterns in children and adolescents: influence of renin-sodium profiles.

A renin-sodium nomogram for normotensive children and adolescents was developed at our institution. The ambulatory blood pressure patterns of subjects classified by the nomogram were then compared. A biracial sample of 159 children and adolescents were classified as having a low, intermediate, or high renin-sodium profile based on the relationship between their plasma renin activity and 24-hour urinary sodium excretion. Casual (106/58 vs 107/61 vs 106/62 mm Hg) and awake (116/69 vs 117/69 vs 116/70 mm Hg) blood pressure values were comparable among subjects with low, intermediate, and high renin-sodium profiles. Subjects with high renin-sodium profiles, however, had a smaller decline in systolic blood pressure with sleep than did subjects with low renin-sodium profiles (7 vs 11 mm Hg; P less than .04), and higher diastolic blood pressure readings during sleep than subjects with intermediate renin-sodium profiles (65 vs 62 mm Hg; P less than .05). Subjects with high renin-sodium profiles also had greater variance of diastolic blood pressure readings during sleep than either subjects with low renin-sodium profiles (P less than .01) or those with intermediate renin-sodium profiles (P less than .02). The blunted nocturnal decline and increased nocturnal variance of blood pressure among subjects with high renin-sodium profiles may be a marker or mechanism for the future development of essential hypertension.

Aerobic fitness and the diurnal rhythm of blood pressure in adolescents.

We examined the effects of aerobic fitness and race on the diurnal rhythm of blood pressure of 175 healthy adolescents who performed a cycle ergometer maximal exercise test while oxygen consumption was measured. A median split of maximum oxygen consumption for boys and girls separately classified them as either "more-fit" or "less-fit" subjects. Ambulatory blood pressure recordings were also performed, and the data were analyzed for means while subjects were awake and asleep. Less-fit black boys had higher systolic pressures than more-fit black boys while awake (124 vs. 115 mm Hg; p less than 0.009) and asleep (117 vs. 108; p less than 0.001). Less-fit black boys also had higher systolic pressures than less-fit white boys while awake (114; p less than 0.002) and asleep (105; p less than 0.001), and they had higher systolic pressures than more-fit white boys while asleep (105; p less than 0.01). Less-fit black girls had higher systolic pressures than more-fit black girls while awake (116 vs. 109; p less than 0.004) and asleep (109 vs. 100; p less than 0.001). Less-fit black girls also had higher diastolic pressures than more-fit black girls while awake (71 vs. 66; p less than 0.002) and asleep (66 vs. 61; p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

The validity of children's self-reports of physical activity: a preliminary study.

Ninety-two healthy children provided self-reports of their physical activity. Self-reports were based upon posters depicting children performing typical childhood activities and were validated by measured oxygen uptake during maximal cycle ergometry. Results indicated that when children were classified as sedentary or active by their poster selection, active children had significantly greater oxygen uptake than sedentary children. These results, while preliminary, are the first to indicate that children's self-reports can provide a valid index of maximal oxygen uptake, the 'gold standard' of aerobic fitness.

Self-reports of salt intake by 10- to 18-year-olds: relationship to urinary sodium excretion.

Our data indicated that self-reports of consumption of salty foods by children and adolescents were associated with 24-hour urinary sodium excretion. Specifically, youths 10 to 18 years of age who selected a poster depicting high-sodium foods excreted significantly more sodium than youths who selected a poster depicting low-sodium foods. Future research is needed to refine simplified self-report measures, to corroborate the validity of the measures, and to extend the studies to other samples, e.g., younger children.

Race, sex, and family history of hypertension and erythrocyte sodium pump [3H]ouabain binding.

We studied the binding properties of [3H]ouabain to erythrocytes from normotensive children (n = 83) between the ages of 10 and 18 years (mean resting arterial pressure: 102/57 mm Hg) from normotensive and essential hypertensive parents. Arterial blood pressures of 101/57 and 104/57 mm Hg (subjects with normotensive and hypertensive parents, respectively) were not significantly different between the two groups. Forty-four children had normotensive parents and 39 had hypertensive parents, 51 were white and 32 were black, and 41 were girls and 42 were boys. By using the [3H]ouabain-binding technique, we determined the density of sodium pump sites and the equilibrium dissociation constants in erythrocytes from these children. Possible effects of race, sex, or parental hypertension status on pump sites and dissociation constants were tested with a three-way analysis of variance (ANOVA). Race had a major effect on the dissociation constant: blacks had a significantly higher value than did whites (p = 0.002). We also found a race by sex by parental hypertension status interaction (p = 0.04) with black girls with hypertensive parents having the highest value. There was no effect of race, sex, or status on sodium pump site density. Age, height, weight, resting arterial blood pressure, and plasma Na+ and K+ concentrations did not correlate with the dissociation constants. These data suggest that, among the groups we studied, black girls with hypertensive parents had erythrocytes with the lowest binding affinity for ouabain.(ABSTRACT TRUNCATED AT 250 WORDS)

Significance of the Doppler-derived gradient across a residual aortic coarctation.

Few data exist which address the significance of the Doppler gradient across a residual narrowing in older children who have had a coarctation repaired. Therefore, we evaluated 11 patients with repaired aortic coarctation with and without residual obstruction by Doppler echocardiography. The Doppler-derived transcoarctation pressure gradient correlated poorly with catheter-measured peak-to-peak and catheter maximal instantaneous gradients when only the maximal velocity across the repair was utilized in the simplified Bernoulli equation, [r = 0.73, standard of error of the estimate (SEE) = 5.0 mmHg and r = 0.56, SEE = 7.6 mmHg, respectively]. However, when the precoarctation velocity was included in the simplified Bernoulli equation, the correlation between Doppler-derived and catheter-measured gradients became excellent. The maximal Doppler gradient correlated well with catheter peak-to-peak gradient (r = 0.95, SEE = 2.2 mmHg) and catheter maximal instantaneous gradient (r = 0.94, SEE = 3.2 mmHg). However, the maximal Doppler gradient slightly overestimated the catheter peak-to-peak gradient and underestimated the catheter maximal instantaneous gradient. The Doppler mean gradient showed excellent correlation with the catheter mean gradient (r = 0.97, SEE = 0.85 mmHg). Precoarctation velocities were generally twofold greater than published normals due to a spatial acceleration phenomenon. All subjects had residual hypoplasia of the transverse aorta such that its transverse diameter was 29% less than, and its cross-sectional area was 50% less than, the ascending and descending aorta.(ABSTRACT TRUNCATED AT 250 WORDS)

Race and gender influence ambulatory blood pressure patterns of adolescents.

The purpose of this study was to examine ambulatory blood pressure and heart rate patterns in healthy, normotensive adolescents and to determine the influence of race and gender on these patterns. Ambulatory blood pressure recordings were performed on 199 adolescents; 42 were black males, 55 were white males, 65 were black females, and 37 were white females. The mean age (+/- SD) was 13 +/- 2 years. Blood pressure readings were obtained with an automatic, noninvasive recorder. Black adolescents and white adolescents had similar blood pressures while awake (116/69 vs. 116/69 mm Hg), with boys having higher levels of systolic blood pressure (118 vs. 114 mm Hg) and comparable levels of diastolic blood pressure (69 vs. 69 mm Hg) relative to girls. The patterns while the adolescents were asleep, however, were different. White boys (106 mm Hg), white girls (105 mm Hg), and black girls (105 mm Hg) had similar systolic blood pressures during sleep. Black boys (112 mm Hg), however, had significantly higher systolic blood pressures while asleep. Black adolescents, as a group, had significantly higher diastolic blood pressures than white adolescents while asleep (64 vs. 61 mm Hg). Changes in blood pressure from awake to asleep were not related to changes in heart rate. Results of this study indicate that both race and gender are important determinants of the diurnal pattern of blood pressure and heart rate in adolescents.

Doppler estimation of pressure gradient in pulmonary stenosis: maximal instantaneous vs peak-to-peak, vs mean catheter gradient.

We undertook a study to identify the hemodynamic significance of a Doppler-derived gradient across a stenotic pulmonary valve. Furthermore, we attempted to define the optimal plane for velocity data acquisition. A total of 17 children with valvar pulmonary stenosis were evaluated using Doppler echocardiography. Flow-velocity profiles were obtained from both the parasternal and subxiphoid windows. Ten of 17 patients were studied before and after balloon valvotomy. Therefore, 27 different transvalvar gradients were assessed by Doppler and these data were compared with the catheter-derived maximal instantaneous, peak-to-peak, and mean pressure gradients. The maximal Doppler gradient correlated well with the catheter-derived peak-to-peak pressure gradient (r = 0.95) and catheter maximal instantaneous pressure gradient (r = 0.95). Although these correlation coefficients were similar, the Doppler maximal gradient consistently overestimated the peak-to-peak catheter gradient by as much as 25%-40%. Such an overestimation was not observed when we compared the maximal Doppler gradient with the catheter-derived maximal instantaneous gradient. Moreover, the regression line of the latter comparison closely approximated the line of identity. The correlation coefficient between Doppler mean and catheter mean gradients was only 0.91. Doppler velocities were best derived when multiple transducer positions were employed to interrogate pulmonary artery velocity.

Physical fitness in children: a survey method based on parental report.

In the present study of 213 healthy children, the relation between parental reports of activity level and laboratory-determined physical fitness was examined. Results indicated that parental reports of activity level were associated with fitness for the entire sample. The association was most consistent among White children who resided in a household that was headed by their father.

Race and cardiovascular reactivity. A replication.

In a previous study, we reported that black children demonstrated greater cardiovascular reactivity than did white children to the psychological stress of a television video game. Reliance on urban volunteers and the wide age range of the children may have limited conclusions concerning the generalizability of those results. In the present study, 481 of 484 children enrolled in the third grade of the public schools of an entire rural county in Tennessee were examined with the same video game procedure used previously. Results indicated greater heart rate and blood pressure reactivity among black children than among white children. Thus, the previous results were replicated. The greater prevalence of hypertension among black adults may relate to the greater reactivity among black children; reactivity may be either a marker or a mechanism in the development of hypertension.