Evidence Suggests a Decrease in the Incidence of Kernicterus in California.

We identified children diagnosed with kernicterus in the California Department of Developmental Services and estimated an incidence of 0.42 per 100 000 births from 1988 to 2014, significantly decreasing to 0.04 per 100 000 births after 2009. We also examined national infant kernicterus mortality from 1979 to 2016 using CDC data. It did not decrease significantly.

Life expectancy in multiple sclerosis by EDSS score.

The median survival time of newly-diagnosed MS patients without severe disabilities is approximately 30-35 years. The prognosis after the onset of severe disability has not been reported. Based on Harding et al.'s 2018 study of the Southeast Wales MS registry, we calculated life expectancies according to the Expanded Disability Status Scale (EDSS). Upon loss of independent ambulation (EDSS 6-6.5; mean age 51.2) life expectancy was 13.3 additional years. At EDSS 9-9.5 (mean age 70.8) life expectancy was 1.1 additional years. These figures provide an empirical basis for discussions of advanced MS care planning.

Life Expectancy after Liver Transplantation for NASH.

Introduction: Non-Alcoholic Steatohepatitis is an increasing reason for liver transplantation in the western world. Knowledge of recipient life expectancy may assist in prudent allocation of a relatively scarce supply of donor livers. Research Questions: We calculated life expectancies for Non-alcoholic steatohepatitis (NASH) patients both at time of transplant and one year later, stratified by key risk factors, and examined whether survival has improved in recent years. Design: Data on 6635 NASH patients who underwent liver transplantation in the MELD era (2002-2018) from the United States OPTN database were analyzed using the Cox proportional hazards regression model and life table methods. Results: Factors related to survival were age, presence of diabetes or hepatic encephalopathy (HE), and whether the patient required dialysis in the week prior to transplant. Other important factors were whether the patient was working, hospitalization prior to transplant, ventilator support, and length of hospital stay (LOS). Survival improved over the study period at roughly 4.5% per calendar year during the first year posttransplant, though no improvement was observed in those who had survived one year. Conclusion: Life expectancy in NASH transplant patients was much reduced from normal, and varied according to age, medical factors, status at transplant, and post transplant course. Over the 17-year study period, patient survival improved markedly during the first year posttransplant, though not thereafter. The results given here may prove helpful in medical decision-making regarding treatment for both liver disease and other medical conditions, as they provide both clinicians and their patients with evidence-based information on prognosis.

Life Expectancy of 1-Year Survivors of Traumatic Brain Injury, 1988-2019: Updated Results From the TBI Model Systems.

OBJECTIVE: To update the life expectancy estimates according to age, sex, mobility, and feeding skills reported in the 2015 study of Brooks et al. To examine trends in survival over the past decade. DESIGN: Observational cohort study. SETTING: Poisson regression and life table analysis applied to long-term follow-up data on United States (US) Traumatic Brain Injury (TBI) Model Systems patients recorded in the national database. Functional mobility and feeding skills were assessed with FIM. PARTICIPANTS: A total of 14,803 persons with TBI during the years 1988-2019 who underwent inpatient rehabilitation and provided at least 1 long-term assessment of functional skills 1 year or more postinjury (N=14,803). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Survival, mortality rates, and life expectancy. RESULTS: Life expectancy was lower than that of the age- and sex-matched general population. Older age and severity of functional impairments were risk factors for mortality (both P<.0001 in regression models). Among ambulatory individuals, mortality was 51% (95% confidence interval, 35%-69%) higher in men than women. Life expectancy of 20-year-old women who walked well (FIM ambulation score 7) was 55 (SE=0.8) additional years to age 75, representing a reduction of 6.9 years from the normal general population figure. For 20-year-old men who walked well, the life expectancy was 49 (SE=0.5) additional years, representing a reduction of 8.1 years from normal. Life expectancies for men and women who did not walk and were fed by others were much lower. There was no significant change in mortality rates during the study period (hazard ratio, 1.008; P=.07). CONCLUSIONS: There has been no significant change in the long-term survival of persons with TBI in the US since the late 1980s. The life expectancies reported here are similar to those reported in the 2015 study of Brooks et al, although they are more precise because of the larger sample size and longer follow-up.

Life Expectancy After Liver Transplantation for Alcoholic Cirrhosis.

BACKGROUND: Alcohol-associated liver disease is the leading cause of liver transplantation in the western world. For these patients we calculated life expectancies both at time of transplant and several years later, stratified by key risk factors, and determined if survival has improved in recent years. METHODS: Data on 14 962 patients with alcohol-associated liver disease who underwent liver transplantation in the MELD era (2002-2018) from the United States Organ Procurement and Transplantation Network database were analyzed using the Cox proportional hazards regression model and life table methods. RESULTS: Demographic and past medical history factors related to survival were patient age, presence of diabetes or severe hepatic encephalopathy, and length of hospital stay. Survival improved over the study period, at roughly 3% per calendar year during the first 5 years posttransplant and 1% per year thereafter. CONCLUSIONS: Life expectancy in transplanted patients with alcohol-associated liver disease was much reduced from normal, and varied according to age, medical risk factors, and functional status. Survival improved modestly over the study period. Information on patient longevity can be helpful in making treatment decisions.

Life Expectancy after Liver Transplantation for Non-Cirrhotic Hepatocellular Carcinoma.

BACKGROUND: Hepatocelluar carcinoma typically occurs with underlying cirrhosis. However roughly 20% of cases arise in a non-cirrhotic liver. There is limited literature that addresses the long-term survival of the narrow subgroup who received transplantation. For such patients we sought to calculate life expectancies both at time of transplant and several years later, stratified by key risk factors, and to determine if survival has improved in recent years. Such information can be helpful in making treatment decisions. METHODS: Data on 4,373 non-cirrhotic HCC patients who underwent liver transplantation in the MELD era (2002-2018) from the United States OPTN database were analyzed using the Cox proportional hazards regression model and life table methods. RESULTS: Demographic and past medical history factors related to survival were patient age, donor age over 20, and the presence of ascites or severe hepatic encephalopathy. Survival did not vary by race or sex. HCC-specific factors significantly related to survival were the total number of tumors, extrahepatic spread, lymph node involvement, satellite lesions, micro- or macrovascular invasion, tumor differentiation (grade), and pre-transplant treatment. Survival improved over the study period, at 4% per calendar year during the first 5 years post transplant and 1% per year thereafter. CONCLUSIONS: Life expectancy in non-cirrhotic HCC transplant patients is much reduced from normal, and varies according to age and tumor-related factors. Survival improved modestly over the study period.

Life Expectancy after Stroke Based On Age, Sex, and Rankin Grade of Disability: A Synthesis.

BACKGROUND: Stroke is a leading cause of death and disability in the developed world. The major factor affecting long term survival (other than age) is known to be the severity of disability. Yet to our knowledge there are no studies reporting life expectancies stratified by both age and severity. Remaining life expectancy is a key measure of health. METHODS: We identified 11 long-term follow-up studies of stroke patients that reported the multivariate effects of age, sex, the modified Rankin Scale (mRS) grade of disability, and other factors. From these we computed the composite effects of these factors on survival, then used these to calculate age-, sex-, and mRS-specific mortality rates. Finally we used the rates to construct life tables, and hence obtain life expectancies. RESULTS: Life expectancy varies by age, sex, and mRS. The life expectancies of males age 70, for example, were 13, 13, 11, 8, 6, and 5 years for Rankin Grades 0-5, respectively, representing reductions of 1, 1, 3, 6, 8, and 9 years from the corresponding general population figure. CONCLUSIONS: These figures demonstrate the importance of rehabilitation following stroke, and can be used in discussion of public policy and benchmarking of future results.

Life expectancy in pancreatic neuroendocrine cancer.

BACKGROUND: The prognoses widely reported for pancreatic cancer reflect the very poor survival associated with the most common histological type, exocrine adenocarcinoma. We calculated life expectancies for patients with less common pancreatic neuroendocrine tumors (PNETs), and also for the subsets of these patients who survive 1 and 5 years post-diagnosis, all of which carry a significantly better prognosis. Results for 1- and 5-year PNET survivors appear not to have been previously reported, nor have life expectancies (average long-term survival times) been given. METHODS: We identified 5287 cases of PNET in the SEER US national database, 1973-2013. The Kaplan-Meier estimator was used to compute empirical survival probabilities and median survival times for functioning (n = 279) and non-functioning PNET (n = 5008) cases. The Cox proportional hazards regression model was used to examine univariate associations of survival with covariates including patient age, sex, race, cancer stage, tumor grade, surgical treatment, and calendar year. A multivariate multiplicative hazard Poisson regression model estimated mortality rates for all combinations of the covariates. The rates were used to construct actuarial life tables, which gave life expectancies for male and female patients according to age, cancer stage, tumor grade, histology (functioning versus non-functioning), surgical treatment status, and time since diagnosis. These life expectancies were compared with age- and sex-specific figures from the US general population. RESULTS: Life expectancy in PNET is lower than that of the US general population and varies significantly according to patient age, cancer stage, tumor grade, mode of treatment, and time since diagnosis. For example, it is near normal for persons aged 70 and older who undergo surgical resection of localized well-differentiated (i.e., grade I) tumors. By contrast, persons with metastatic high-grade tumors not amenable to surgery have life expectancies of only 1 to 4 years depending on patient age. Functioning PNETs were associated with somewhat lower mortality than non-functioning within the first few years after diagnosis, though no major differences were observed long-term. Positive factors for survival were younger age, localized stage, low tumor grade, and surgical treatment. Survival improved over the 1973-2013 study period: on average mortality rates fell by 1.2% per year after controlling for changes in the patient population. Life expectancy increased markedly with time since diagnosis: those surviving 1 and 5 years post-diagnosis had longer additional life expectancies. CONCLUSIONS: Life expectancies of patients with PNETs may be markedly reduced from normal, but even in the worst cases their prognoses remain significantly better than that of patients with the more common pancreatic adenocarcinomas. In some very favorable cases, the life expectancy is near-normal, especially amongst 1- and 5-year survivors. This information can be used to counsel patients.

An update on survival after anoxic brain injury in adolescents and young adults.

While much is known about long-term survival after traumatic brain injury, less is known about survival after anoxic/hypoxic brain injury. We previously compared the two and found no significant difference (hazard ratio [HR] 0.97, p=0.92) after controlling for age, sex, and severity of disability. The present study updates this, based on 1,802 patients with TBI and 380 with anoxic, aged 15 to 25, evaluated in 1986 or later, and who survived one year post injury. The anoxic group had higher mortality (HR = 1.13), though again the difference was not statistically significant (p=0.58).

A note on survival after anoxic brain injury in adolescents and young adults.

BACKGROUND: Much is known about survival after traumatic brain injury (TBI), yet relatively little about survival after anoxic brain injury (ABI). OBJECTIVE: To determine whether long-term survival after ABI is comparable to that after TBI. METHODS: We identified 237 patients with ABI and 1,620 with TBI in California who were aged 15 to 35, survived at least 1 year post injury, and were injured in 1986 or later. We analyzed the long-term follow-up data using the Cox Proportional Hazards Regression Model, controlling for age, sex, and severity of disability. RESULTS: After adjustment for risk factors, no significant differences in long-term survival between ABI and TBI were found (hazard ratio = 0.97; 95% c.i. 0.57-1.65). CONCLUSIONS: In adolescents and young adults, long-term survival after ABI appears to be similar to that after TBI.

Long-term survival after traumatic brain injury part I: external validity of prognostic models.

OBJECTIVES: To develop prognostic models for long-term survival in adults with traumatic brain injury (TBI) and to assess their external validity in 2 independent populations. DESIGN: Survival analysis. SETTING: Post-discharge from rehabilitation units and long-term follow-up at regional centers. PARTICIPANTS: Two cohorts of long-term survivors of TBI (N=12,481): the Traumatic Brain Injury Model Systems (TBIMS) cohort comprised 7365 persons who were admitted to a TBIMS facility and were assessed at ≥1 years postinjury, and the California Department of Developmental Services (CDDS) cohort comprised 5116 persons who sustained a TBI and received long-term services from the CDDS. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Survival/mortality. RESULTS: Older age, male sex, and severity of disability in walking and feeding were significant predictors of increased long-term mortality rates (all P<.05, both databases). The CDDS model predicted 623 deaths for persons in the TBIMS cohort, with an observed-to-expected ratio of .94 (95% confidence interval [CI], 0.87-1.02). The TBIMS model predicted a total of 525 deaths for persons in the CDDS cohort, with an observed-to-expected ratio of 1.08 (95% CI, 0.99-1.17). Regression calibration statistics were satisfactory, and both models ranked survival times well from shortest to longest (TBIMS: C index, .78; 95% CI, .76-.80; CDDS: C index, .80; 95% CI, .78-.82). CONCLUSIONS: Long-term survival prognosis in TBI is related to age, sex, and severity of disability. When compared on the basis of these factors, the survival estimates derived from the TBIMS and CDDS cohorts are found to be similar. The close agreement between model predictions and actual mortality rates confirm the external validity of the prognostic models presented herein.

Long-Term Survival After Traumatic Brain Injury Part II: Life Expectancy.

OBJECTIVES: To compute the life expectancy of persons with traumatic brain injury (TBI) based on validated prognostic models from 2 cohorts, to compare mortality and life expectancy of persons with TBI with those of the U.S. general population, and to investigate trends toward improved survival over the last 2 decades. DESIGN: Survival analysis. SETTING: Postdischarge from rehabilitation units and long-term follow-up at regional centers. PARTICIPANTS: Two cohorts of long-term survivors of TBI (N=12,481): the Traumatic Brain Injury Model Systems (TBIMS) cohort comprised 7365 persons who were admitted to a TBIMS facility with moderate to severe TBI and were assessed at ≥1 years postinjury, and the California Department of Developmental Services (CDDS) cohort comprised 5116 persons who sustained a TBI and received long-term services from the CDDS. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Life expectancy. RESULTS: The estimates of age-, sex-, and disability-specific life expectancy of persons with TBI derived from the CDDS and TBIMS were similar. The estimates of age- and sex-specific life expectancy were lower than those of the U.S. general population. Mortality rates of persons with TBI were higher than those of the U.S. general population. Mortality rates did not improve and the standardized mortality ratio increased over the study period from 1988 to 2010. CONCLUSIONS: Life expectancy of persons with TBI is lower than that of the general population and depends on age, sex, and severity of disability. When compared, the survival outcomes in the TBIMS and CDDS cohorts are remarkably similar. Because there have been no marked trends in the last 20 years, the life expectancies presented in this article may remain valid in the future.

Improvements in long-term survival after spinal cord injury?

OBJECTIVE: To investigate whether there have been improvements in long-term survival after spinal cord injury in recent decades. DESIGN: Survival analysis using time-varying covariates. The outcome variable was survival or mortality, and the explanatory variables were age, sex, level and grade of injury, and calendar year. The data were analyzed using the logistic regression model, Poisson regression model with comparison to the general population, and the computation of standardized mortality ratios for various groups. SETTING: National Spinal Cord Injury Model Systems facilities. PARTICIPANTS: Persons (N=31,531) who survived 2 years postinjury, were older than 10 years, and who did not require ventilator support. These persons contributed 484,979 person-years of data, with 8536 deaths over the 1973 to 2012 study period. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Survival; survival relative to the general population; life expectancy. RESULTS: After adjustment for age, sex, race, etiology of injury, time since injury, and level and grade of injury, mortality in persons with spinal cord injury was higher in the 2005 to 2012 period than in 1990 to 2004 or 1980 to 1989, the odds ratios for these 3 periods were .857, .826, and .802 as compared with the 1970 to 1979 reference period. CONCLUSIONS: There was no evidence of improvement. Long-term survival has not changed over the past 30 years.

Mobility, continence, and life expectancy in persons with Asia Impairment Scale Grade D spinal cord injuries.

OBJECTIVE: Previous research on the life expectancy of persons with American Spinal Injury Association (ASIA) Impairment Scale Grade D spinal cord injury has considered them as a large homogenous group, making no functional or medical distinctions. This study sought to (1) determine how survival in this group depends on ambulatory function and the extent of bowel or bladder dysfunction, (2) compute life expectancies for various subgroups, and (3) examine whether survival has improved over time. DESIGN: Data were from 8,206 adults with ASIA Impairment Scale Grade D spinal cord injury in the Spinal Cord Injury Model Systems database who were not ventilator dependent and who survived more than 1 yr after injury. There were a total of 114,739 person-years of follow-up and 1,730 deaths during the 1970-2011 study period. Empirical age- and sex-specific mortality rates were computed. Regression analysis of survival data with time-dependent covariates was used to determine the effect of risk factors, to test for a time trend, and to estimate mortality rates for subgroups. Life expectancies were obtained from life tables constructed for each subgroup. RESULTS: The ability to walk, whether independently or with an assistive device, was associated with longer survival than wheelchair dependence. The need for an indwelling catheter, and to a lesser extent intermittent catheterization, was associated with increased mortality risk. Persons who walked unaided and who did not require catheterization had life expectancies roughly 90% of normal. Those who required a wheelchair for locomotion had life expectancies comparable with that in paraplegia, less than 75% of normal. No time trend in survival was found. CONCLUSIONS: Life expectancy of persons with ASIA Impairment Scale D spinal cord injury depends strongly on the ability to walk and the need for catheterization.

Angioedema deaths in the United States, 1979-2010.

BACKGROUND: Hospital admission data indicate that the angioedema incidence has increased during the past several decades. Little is known about mortality trends. OBJECTIVES: To count the number of deaths associated with angioedema in the United States, investigate correlations with age, sex, race, and other contributory causes, and analyze trends from 1979 to 2010. METHODS: All US death certificates in which angioedema was listed as an underlying or contributing cause of death during 1979 to 2010 were analyzed. Age-adjusted mortality rates were analyzed by age, sex, and race. Other conditions designated as the underlying cause of death were investigated. RESULTS: From 1979 to 2010, there were 5,758 deaths in which angioedema was listed as a contributing cause. The age-adjusted death rate for hereditary angioedema decreased from 0.28 (95% confidence interval [CI] 0.25-0.32) to 0.06 (95% CI 0.05-0.08) per million persons per year. Conversely, mortality for angioedema increased from 0.24 (95% CI 0.21-0.27) to 0.34 (95% CI 0.31-0.37) per million. Blacks constituted 55% of angioedema deaths that were associated with use of angiotensin-converting enzyme inhibitors. On death certificates that listed hereditary angioedema as the underlying cause of death, cancer (frequently lymphoma or leukemia) was the second most commonly listed cause. CONCLUSION: Angioedema-associated deaths were very rare from 1979 to 2010. Hereditary angioedema deaths became even more so, whereas nonhereditary angioedema deaths increased. Risks associated with angiotensin-converting enzyme inhibitors were higher in blacks. Lack of specific coding for acquired angioedema most likely explains the observed association between cancer and hereditary angioedema. In the future, more granular coding systems may help distinguish hereditary from acquired angioedema.

Recent trends in cerebral palsy survival. Part II: individual survival prognosis.

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.

Recent trends in cerebral palsy survival. Part I: period and cohort effects.

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.

Comparative mortality of persons with intellectual disability in California: an update (2000-2010).

This paper updates our 2003 study on the effect of intellectual disability (ID) on mortality in persons with no significant physical disability. As previously, we used the California Department of Developmental Services database to compute mortality rates by age, sex, and severity of ID. There were 64,207 subjects age 5 and older, who contributed 386,000 person-years of follow-up and 1514 deaths during the 2000 to 2010 study period. The excess death rates increased with age, ranging from 0.1 to 6.8 per 1000 in mild/moderate ID, and 3.4 to 6.7 in severe/profound.