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Aim: The aim of the study was to determine survival probabilities and life expectancies for individuals with cerebral palsy based on data collected over a 28-year period in California., Method: We identified all individuals with cerebral palsy, aged 4 years or older, who were clients of the California Department of Developmental Services between 1983 and 2010. Kaplan-Meier survival curves were constructed for 4-year-old children, and the estimated survival probabilities were adjusted to reflect trends in mortality by calendar year. For persons aged 15, 30, 45, and 60 years, separate Poisson regression models were used to estimate age-, sex-, and disability-specific mortality rates. These mortality rates were adjusted to reflect trends of improved survival, and life expectancies were obtained using life table methods., Results: The sample comprised 16,440, 14,609, 11,735, 7023, and 2375 persons at ages 4, 15, 30, 45, and 60 years, respectively. In 1983, 50% of 4-year-old children who did not lift their heads in the prone position and were tube fed lived to age 10.9 years. By 2010, the median age at death had increased to 17.1 years. In ambulatory children the probability of survival to adulthood did not change by more than 1%. Life expectancies for adolescents and adults were lower for those with more severe limitations in motor function and feeding skills, and decreased with advancing age. Life expectancies for tube-fed adolescents and adults increased by 1 to 3 years, depending on age and pattern of disability, over the course of the study period., Interpretation: Over the past three decades in California there have been significant improvements in the survival of children with very severe disabilities. There have also been improvements to the life expectancy of tube-fed adults, though to a lesser extent than in children., (© 2014 Mac Keith Press.)

Aim: To determine whether the trend of improved survival among individuals with cerebral palsy (CP) in California during the 1980s and 1990s has continued during the most recent decade., Method: In an observational cohort study we evaluated individuals with CP, aged 4 years and older, who were clients of the California Department of Developmental Services. Medical diagnoses, functional disabilities, and special health care requirements were assessed with Client Development Evaluation Reports made between 1983 and 2010. Trends in birth cohort survival were analyzed with Kaplan-Meier curves and Cox regression. Calendar year period effects were analyzed with Poisson regression., Results: A total of 51,923 persons with CP (28,789 males [55%], 23,134 females [45%]; mean age 14y 11mo, SD 14y 1mo, range 4y 0mo to 96y 10mo) collectively contributed 662,268 years of follow-up. There were 7690 deaths for an overall mortality rate of 11.6 per 1000 persons per year. No significant birth cohort effects on survival were observed in 4-year-olds who had no severe disabilities. By contrast, children who did not lift their heads in prone position who were born in more recent years had significantly lower mortality rates (Cox hazard ratio 0.971, p<0.001) than those with comparable disabilities born earlier. With regard to calendar year period effects, we found that age-, sex-, and disability-specific mortality rates declined by 1.5% (95% CI 0.9-2.1) year-over-year from 1983 to 2010. The estimate increased to 2.5% (95% CI 1.9-3.1) per year when we additionally controlled for tube-feeding status. Mortality rates in tube fed adolescents and adults, ages 15 to 59 years, declined by 0.9% (95% CI, 0.4-1.4) per year. No improvement was observed for adolescents or adults who fed orally or for those over age 60. In fact, the ratio of age-specific mortality rates for these latter groups to those in the general population, increased by 1.7% (95% CI 1.3-2.0) per year during the study period., Interpretation: The trend toward improved survival has continued throughout the most recent decade. Declines in CP childhood mortality are comparable to the improvements observed in the United States general population (i.e. the mortality ratio in childhood has remained roughly constant over the last three decades). In contrast, the mortality ratio for most adolescents and adults with CP, relative to the general population, has increased., (© 2014 Mac Keith Press.)

Rationale: Previous studies have demonstrated that chronic obstructive pulmonary disease (COPD) causes increased mortality in the general population. But life expectancy and the years of life lost have not been reported., Objectives: To quantify mortality, examine how it varies with age, sex, and other risk factors, and determine how life expectancy is affected., Methods: We constructed mortality models using the Third National Health and Nutrition Examination Survey, adjusting for age, sex, race, and major medical conditions. We used these to compute life expectancy and the years of life lost., Measurements and Main Results: Pulmonary function testing classified patients as having Global Initiative on Obstructive Lung Disease (GOLD) stage 0, 1, 2, 3 or 4 COPD or restriction. COPD is associated with only a modest reduction in life expectancy for never smokers, but with a very large reduction for current and former smokers. At age 65, the reductions in male life expectancy for stage 1, stage 2, and stages 3 or 4 disease in current smokers are 0.3 years, 2.2 years, and 5.8 years. These are in addition to the 3.5 years lost due to smoking. In former smokers the reductions are 1.4 years and 5.6 years for stage 2 and stages 3 or 4 disease, and in never smokers they are 0.7 and 1.3 years., Conclusions: Persons with COPD have an increased risk of mortality compared to those who do not, with consequent reduction in life expectancy. The effect is most marked in current smokers, and this is further reason for smokers to quit.

This study aimed to determine the probability that a child with cerebral palsy (CP) will lose or gain ambulatory ability through adolescence and young adulthood. We analyzed retrospectively data from 1987 to 2002 on Californians with CP initially aged 10 years (SD 0.9y; n=7550 [4304 males, 3246 females]) and 25 years (SD 0.8y; n=5721 [3261 males, 2460 females]) who had varying levels of ambulatory ability (initial Gross Motor Function Classification System Levels I-IV). We used the Aalen-Johansen estimator to estimate probabilities of transition to other levels of ambulatory ability in the future. Those who walked and climbed stairs without difficulty at age 10 had only a 23% chance of decline (to requiring a handrail to manage stairs, or worse) 15 years later. Those who ambulated with some difficulty but did not use a wheelchair had a significant chance (33%) of improvement (to being able to walk unsteadily alone at least 3m or better) and only a small chance (11%) of becoming non-ambulatory. Those who used a wheelchair were more likely to lose ambulatory ability (34%) or die (6%). Those who walked and climbed stairs well at age 25 were likely to maintain that ability 15 years later (76%), while those needing support to climb stairs were more likely to lose ability. Improvement in ambulation after age 25 was unlikely. Children and young adults with CP are likely to maintain their ambulatory ability during their next 15 years. Some who ambulate with difficulty at age 10 may improve through adolescence, but those who use a wheelchair are more likely to decline. By age 25 improvement in ambulation is unlikely and decline more likely. Most, however, will not change over the next 15 years.

This study examined growth of children and adolescents with cerebral palsy (CP) who received services from the California Department of Developmental Services from 1987 to 2002. In all, 141 961 measurements of height and weight were taken from 24920 patients with CP (14103 males, 10817 females). Centiles of weight and height were determined by age, sex, and five levels of functional ability ranging from fully ambulatory to unable to walk, crawl, or feed self, and fed via gastrostomy tube. Resulting charts of height and weight centiles were compared with Centers for Disease Control and Prevention weight and height charts for the general population of the US. Centiles of height and weight of patients with CP were close to those of the general population for the highest functioning groups with CP, but lagged substantially for other groups. Presence of a feeding tube was associated with greater height and weight in the lowest functioning groups, with centiles for weight being 2 to 5 kg higher for those with gastrostomy tubes. The charts may assist in early identification of nutritional or metabolic difficulties beyond what might be expected for patients with similar functional disabilities.

Objective: To determine whether persons who incur a spinal cord injury as children are at increased risk of mortality compared with persons injured as adults given comparable current age, sex, and injury severity., Methods: A total of 25,340 persons admitted to the National Spinal Cord Injury Statistical Center database or the National Shriners Spinal Cord Injury database who were not ventilator dependent and who survived more than 2 years after injury were included in this study. These persons contributed 274,020 person-years of data, with 3844 deaths, over the 1973-2004 study period. Data were analyzed using pooled repeated observations analysis of person-years. For each person-year the outcome variable was survival/mortality, and the explanatory variables included current age, sex, race, cause of injury, severity of injury, and age at injury (the focus of the current analysis)., Results: Other factors being equal, persons who were less than 16 years of age at time of injury had a 31% (95% CI = 3%-65%) increase in the annual odds of dying compared with persons injured at older ages (P= 0.013). This increased risk did not vary significantly by current age, sex, race, injury severity, or era of injury (P > 0.05)., Conclusion: Life expectancy for persons injured as children appears to be slightly lower than that of otherwise comparably injured persons who suffered their injuries as adults. Nonetheless, persons who are injured young can enjoy relatively long life expectancies, ranging from approximately 83% of normal life expectancy for persons with minimal deficit incomplete injuries to approximately 50% of normal in high-cervical-level injuries without ventilator dependence.

Objective: To develop predictive models to estimate worklife expectancy after spinal cord injury (SCI)., Design: Inception cohort study., Setting: Model SCI Care Systems throughout the United States., Participants: 20,143 persons enrolled in the National Spinal Cord Injury Statistical Center database since 1973., Intervention: Not applicable., Main Outcome Measure: Postinjury employment rates and worklife expectancy., Results: Using logistic regression, we found a greater likelihood of being employed in any given year to be significantly associated with younger age, white race, higher education level, being married, having a nonviolent cause of injury, paraplegia, ASIA D injury, longer time postinjury, being employed at injury and during the previous postinjury year, higher general population employment rate, lower level of Social Security Disability Insurance benefits, and calendar years after the passage of the Americans with Disabilities Act., Conclusions: The likelihood of postinjury employment varies substantially among persons with SCI. Given favorable patient characteristics, worklife should be considerably higher than previous estimates.

Background/objective: Identify factors related to long-term survival, and quantify their effect on mortality and life expectancy., Setting: Model spinal cord injury systems of care across the United States., Study Design: Survival analysis of persons with traumatic spinal cord injury who are ventilator dependent at discharge from inpatient rehabilitation and who survive at least 1 year after injury., Methods: Logistic regression analysis on a data set of 1,986 person-years occurring among 319 individuals injured from 1973 through 2003., Results: The key factors related to long-term survival were age, time since injury, neurologic level, and degree of completeness of injury. The life expectancies were modestly lower than previous estimates. Pneumonia and other respiratory conditions remain the leading cause of death but account for only 31% of deaths of known causes., Conclusions: Whereas previous research has suggested a dramatic improvement in survival over the last few decades in this population, this is only the case during the critical first few years after injury. There was no evidence for such a trend in the subsequent period.

This study investigated mortality and causes of death between 1988 and 1999 in 14781 persons (6702 female) with Down syndrome in California, comparing age, sex, ethnicity, and other factors. Mean age at the start of follow-up was 14 years 8 months (SD 14y 10mo). During the study period 600 persons died. The standardized mortality ratio (SMR) for the population was 5.5. Blacks were at greater risk than whites, Hispanics, or Asians (relative risk = 1.5). Mortality declined during the period, especially for children with congenital heart defects. Leukemia (SMR = 17), respiratory illnesses (SMR = 27), congenital anomalies (SMR = 72), and circulatory diseases (SMR = 5.3) accounted for most of the excess mortality. With the exception of leukemia, cancer mortality was not different from that of the general population.

Objectives: The study was done to evaluate whether ethnic differences exist in the prevalence of coronary artery calcification (CAC), and to determine whether differences in calcification correlate with the degree of coronary obstruction., Background: Electron beam tomography (EBT) can be used to quantitate the amount of CAC and assist in prognostication of future cardiac events. It is unclear whether ethnic differences in coronary mortality are related to differences in the prevalence of coronary obstruction and CAC., Methods: A total of 782 symptomatic subjects underwent both EBT and angiography. A 50% luminal narrowing defined an angiographic obstruction., Results: We observed substantial ethnic differences in prevalence of both CAC and angiographic stenosis. In whites (n = 453), prevalence of CAC (score >0) was 84%, and significant obstruction on angiogram was 71%. Compared with whites, blacks (n = 108) had a significantly lower prevalence of CAC (62%, p < 0.001) and angiographic disease (49%, p < 0.01). Hispanics (n = 177) also had a lower prevalence of CAC (71%, p < 0.001) and angiographic obstruction (58%, p < 0.01). Asians (n = 44) were not significantly different in regard to CAC (73%, p = 0.06) or angiographic stenosis (64%, p = 0.30). These ethnic differences remained after simultaneously controlling (by use of multiple logistic regression) for age, gender and cardiac risk factors., Conclusions: As compared with whites, blacks and Hispanics had significantly lower prevalence of CAC and obstructive coronary disease. Ethnic differences in risk-factor profiles do not explain these differences. This study demonstrated that whites have a higher atherosclerotic burden than blacks and Hispanics, independent of risk-factor differences among symptomatic patients referred for angiography.

Objectives: This study compared coronary artery calcium (CC) as detected by electron beam computed tomography (EBCT) with conventional stress testing in the evaluation of patients with symptoms suggestive of coronary artery disease (CAD)., Background: Exercise electrocardiogram treadmill stress testing (treadmill-ECG) is limited by its requirement of a normal resting ECG and the ability of the patient to exercise adequately. The addition of myocardial imaging agents such as technetium improves the sensitivity and specificity but substantially increases the cost and prolongs the testing time. The use of EBCT provides a noninvasive and rapid method for identifying the presence and amount of CC, which has been shown to be related to atherosclerosis, and may provide additional information in combination with more traditional noninvasive testing methods., Methods: A total of 97 patients underwent technetium stress testing (technetium-stress), treadmill-ECG, and EBCT coronary scanning within three months of coronary angiography for the evaluation of chest pain., Results: The relative risk (RR) of obstructive angiographic CAD for an abnormal test was higher for EBCT (4.53) than either treadmill-ECG (1.72) or technetium-stress (1.96). The low specificity of EBCT (47%) was improved by the addition of treadmill-ECG (83%, p < 0.05)., Conclusions: Electron beam computed tomography has a higher diagnostic ability than either treadmill-ECG or technetium-stress for the detection of obstructive angiographic CAD. Electron beam computed tomography is an accurate and noninvasive alternative to traditional stress testing for the detection of obstructive CAD in symptomatic patients.

Background: Prognostic risk models have had limited success in predicting coronary events in subjects with multiple risk factors. We and others have proposed an alternative approach using radiographically detectable coronary calcium. We evaluated and compared the predictive value of these 2 approaches for determining coronary event risk in asymptomatic adults with multiple coronary risk factors. In addition, we assessed the predictive value of a risk model that included calcium score and cardiac risk-factor data., Methods and Results: We recruited 1196 asymptomatic high-coronary-risk subjects who then underwent risk-factor assessment and cardiac electron-beam CT (EBCT) scanning and were followed up for 41 months with a 99% success rate. We applied the Framingham model and our data-derived risk model to determine the 3-year likelihood of a coronary event. The mean age of our cohort was 66 years, and mean 3-year Framingham risk was 3.3+/-3.6%. Sixty-eight percent (818 subjects) had detectable coronary calcium. There were 17 coronary deaths (1.4%) and 29 nonfatal infarctions (2. 4%). The receiver operating characteristic (ROC) curve areas calculated from the Framingham model, our data-derived risk model, and the calcium score were 0.69+/-0.05, 0.68+/-0.05, and 0.64+/-0.05, respectively (P=NS). When calcium score was included as a variable in the data-derived model, the ROC area did not change significantly (0.68+/-0.05 to 0.71+/-0.04; P=NS)., Conclusions: Neither risk-factor assessment nor EBCT calcium is an accurate event predictor in high-risk asymptomatic adults. EBCT calcium score does not add significant incremental information to risk factors, and its use in clinical screening is not justified at this time.

Background: Blacks have been found to have lower amounts of coronary calcium as well as higher levels of the osteoregulatory steroid 1,25-dihydroxyvitamin D3 [1,25(OH)2D3] than whites. We sought to determine if racial differences in coronary calcium mass could be explained by differences in serum levels of 1,25(OH)2D3., Methods and Results: We evaluated standard coronary risk factors, quantified coronary calcium mass with electron-beam computed tomography (EBCT), and measured serum 1,25(OH)2D3 with radioimmunoassay in 283 high-risk subjects (51 [180%] black, 232 [82%] white). Black subjects had lower masses of coronary calcium than whites (14 versus 47 mg; P=.003). Serum 1,25(OH)2D3 levels were slightly higher in blacks (41 versus 38 pg/mL; P=.05). Log 1,25(OH)2D3 levels were inversely proportional to log-transformed calcium mass (r=-.19; P=.001) in both races. Multivariate linear regression demonstrated that both black race (P=.02) and 1,25(OH)2D3 levels (P=.007) contributed inversely and independently to coronary calcium mass. However, an interaction term of racex1,25(OH)2D3 did not significantly contribute to coronary calcium mass, indicating that other undetermined factors in addition to 1,25(OH)2D3 are responsible for ethnic differences in coronary calcium mass., Conclusions: Both black race and serum levels of 1,25(OH)2D3 are independent negative determinants of coronary calcium mass. Nevertheless, diminished amounts of coronary calcium in blacks are not accounted for by higher 1,25(OH)2D3 levels.