scholarly journals A population-based study to estimate survival and standardized mortality of tuberous sclerosis complex (TSC) in Taiwan

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Jui-Hui Peng ◽  
Hung-Pin Tu ◽  
Chien-Hui Hong
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Survival Rates ◽  
Population Based ◽  
Standardized Mortality Ratio ◽  
Significant Prognostic Factor ◽  
Research Database ◽  
Mortality And Morbidity ◽  
Asian Patients ◽  
Diagnosis Age

Abstract Background Tuberous sclerosis complex (TSC) is an autosomal dominant disease with systemic manifestations, which can cause significant mortality and morbidity. Population-based epidemiological studies on TSC mortality and survival remain scarce, though several recent studies provide evidence that TSC survival rates are high and disease prognosis is fair for most patients. This study aims to estimate the life expectancy and mortality statistics in Taiwanese TSC patients, investigate prognosis and associations of TSC mortality based on demographic variables, and compare these results to past literature, especially for Asian patients. Methods Taiwanese National Health Insurance (NHI) insurees can obtain Catastrophic Illness Certificates (CIC) for certain eligible diseases to waive copayments after diagnosis by two independent physicians. CIC holders for TSC during 1997–2010 were identified from the NHI Research Database. Queries on enrollment (CIC acquisition) age, endpoint (end of query period or death) age, sex, and comorbidities were obtained. Patients were separated into cohorts (endpoint age, sex, and age of diagnosis), and analyzed accordingly. Results 471 patients (232 male, 239 female) were identified, of which 14 died. Compared to literature, patients showed similar demographics (age range, diagnosis age, sex distribution); similar manifestations and prevalence (epilepsy, intellectual disability, renal disease); lower disease prevalence (1 in 63,290); lower mortality (0.21% per year); and near-identical standardized mortality ratio (4.99). A cumulative mortality of 4.08% was found over 14 years, though mortality plateaued at 7 years post-enrollment, suggesting a good overall survival rate; comparable with previous studies in Asian patients. Enrollment age was a significant prognostic factor, with late-enrollment (age > 18) patients at higher risk for all-cause mortality (Hazard ratio = 6.54). Average remaining lifetime was significantly lower than the general population, and decreased with age. Conclusions This study reports a population-based disease database, highlights the importance of diagnosis age in prognosis prediction, and suggests the role of renal manifestations in mortality. Furthermore, it corroborates recent TSC studies in the Asian population in terms of survival. Overall, physician vigilance, early diagnosis, and careful monitoring are beneficial for disease outcome and patient survival.

EPID-10. GLIOMA INCIDENCE AND SURVIVAL BY INCOME LEVEL IN THE UNITED STATES GENERAL POPULATION AGES AND ABOVE FROM 2000-2018

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi87-vi88
Author(s):  
Jennifer Murillo ◽  
Elizabeth Anyanda ◽  
Jason Huang
Keyword(s):  
United States ◽  
General Population ◽  
Survival Data ◽  
Survival Rates ◽  
The United States ◽  
Income Level ◽  
Population Based ◽  
Research Database ◽  
Income Levels ◽  
Average Survival

Abstract Gliomas are the most common primary malignant brain tumor in the United States with previous studies showing the incidence varied by age, sex, and race or ethnicity. Survival after diagnosis has also been shown to vary by these factors. Also, socioeconomic status and its association with various cancers have also been studied at length over time. PURPOSE: The purpose of our research was to quantify the differences in incidence and survival rates of gliomas in 15 years and older by income level. METHODS: This population-based study obtained incidence and survival data from the Incidence-SEER Research Database the general population. Average age incidence were generated by glioma groups and grouped by income levels. Survival rates were generated by overall glioma diagnosis grouped by observed survival at 12, 24, 36, 48 and 60 months and by again by income levels. The analysis included 94,207 patients with glioma diagnosed in those aged 15 years or older. RESULTS: Overall, 94, 207 patients diagnosed with glioma were analyzed. Of these, 1,089 (1.16%) fell into the < $35k group, 1,684 (1.79%) in the $35k-$40k group, 3,473 (3.69%) in the $40k-$45k group, 5,647 (5.99%) in the $45k-$50k group, 7,138 (7.58%) in the $50k-$55k group, 6,468 (6.87%) in the $55k-$60k group, 15,348 (16.29%) in the $60k-$65k group, 13,216 (14.03%) in the $65k-$70k group, 9,035 (9.59%) in the $70k-$75k group, and 31,109 (33.02%) fell in > $75k group. The data was also broken further down into survivability showing average survival. CONCLUSION: Incidence of glioma and 12, 24, 36, 48 and 60 month survival rates after diagnosis vary significantly by income level with higher income level greater than $75,000+ having higher incidence and higher survival rates compared with lower income levels. Further research is needed to help determine risk factors and barriers to care to help reveal health disparities.

scholarly journals Mortality and Vascular Outcomes in Patients Treated for Thyroid Dysfunction

2006 ◽  
Vol 91 (6) ◽  
pp. 2159-2164 ◽  
Author(s):  
R. W. V. Flynn ◽  
T. M. MacDonald ◽  
R. T. Jung ◽  
A. D. Morris ◽  
G. P. Leese
Keyword(s):  
General Population ◽  
Thyroid Dysfunction ◽  
Population Based ◽  
Primary Hypothyroidism ◽  
Secondary Outcome ◽  
Standardized Mortality Ratio ◽  
Vascular Events ◽  
Mortality And Morbidity ◽  
Increased Risk ◽  
All Cause Mortality

Abstract Context: There are limited studies describing mortality and morbidity in patients treated for hyperthyroidism, and no data exist for people with treated hypothyroidism. Objective: The objective of the study was to describe all-cause mortality and vascular mortality and morbidity in patients after treatment for hyperthyroidism and hypothyroidism. Design: This was a population-based cohort study from 1994 to 2001. Setting: The study was conducted in Tayside, Scotland. Patients: All patients were treated for thyroid dysfunction. Intervention(s): Event rates among patients with thyroid dysfunction were compared with rates in the general population. We measured standardized mortality ratio and standardized incidence ratio (SIR). Main Outcome Measure(s): The primary outcome was all-cause mortality. The secondary outcome was serious vascular event, the composite end point of nonfatal myocardial infarction, nonfatal stroke, or vascular death. Results: There were 15,889 primary hypothyroid and 3,888 hyperthyroid patients. There were 3,116,719 patient-years of follow-up in 524,152 subjects in the general population. No increase was found in all-cause mortality or serious vascular events in patients with treated hypothyroidism or hyperthyroidism. Nonfatal ischemic heart disease [SIR 1.23, 95% confidence interval (CI) 1.10–1.36] and dysrhythmias (SIR 1.32, 95% CI 1.11–1.57) were increased in treated hypothyroidism when adjusted for age, sex, diabetic status, and previous vascular disease. In treated stabilized hyperthyroidism, only the risk of dysrhythmias was increased (SIR 2.71, 95% CI 1.63–4.24). Risk of heart failure or cerebrovascular disease was not increased in either patient group. Conclusions: We found no increase in all-cause mortality in subjects with treated thyroid disease. However, there was increased risk of cardiovascular morbidity in patients with treated primary hypothyroidism and dysrhythmias in treated hyperthyroidism.

scholarly journals All-cause mortality of patients with idiopathic pulmonary fibrosis: a nationwide population-based cohort study in Korea

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Sung Ko ◽  
Sun Mi Choi ◽  
Kyung-Do Han ◽  
Chang-Hoon Lee ◽  
Jinwoo Lee
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Cause Of Death ◽  
Normal Population ◽  
Survival Rates ◽  
Population Based ◽  
Epidemiologic Studies ◽  
Standardized Mortality Ratio ◽  
Mortality Ratio ◽  
Definition Of

AbstractMost epidemiologic studies of patients with idiopathic pulmonary fibrosis (IPF) have been conducted in North America and Europe. Moreover, there are limited data concerning the cause of death and cause-specific mortality rate of IPF patients in population-based studies. We analyzed information from the Korean National Health Insurance Service database from 2006 to 2016. Patients with a diagnosis code of IPF were extracted from the database and we included those who satisfied the narrow definition of IPF diagnosis. Age- and sex-matched controls were randomly selected at a case-to-control rate of 1:3. We included 42,777 patients newly diagnosed with IPF during the study period. Their mean age was 64.6 years, and 65.4% were male. The age-standardized mortality rates were 85.66 (95% confidence interval [CI] 84.45–86.89) per 1000 person-years. The survival rates of IPF patients 1, 2, 3, 5, and 10 years after IPF diagnosis were 84.5%, 77.4%, 71.9%, 62.9%, and 48.4%, respectively. The standardized mortality ratio of IPF patients compared to that of the normal population was 4.66. The leading cause of death in IPF patients was respiratory causes, followed by cancer. Patients with IPF in Korea showed significantly higher mortality compared to the general population.

scholarly journals P.050 NICO-assisted neuroendoscopic management of enlarging subependymal giant cell astrocytoma in tuberous sclerosis complex: a case report

2018 ◽  
Vol 45 (s2) ◽  
pp. S28-S28
Author(s):  
A Dakson ◽  
P McNeely
Keyword(s):  
Tuberous Sclerosis ◽  
Giant Cell ◽  
Tuberous Sclerosis Complex ◽  
Term Treatment ◽  
Surgical Approaches ◽  
Subependymal Giant Cell Astrocytoma ◽  
Mortality And Morbidity ◽  
Long Term Treatment ◽  
Causes Of Mortality ◽  
Giant Cell Astrocytoma

Background: Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome classically associated with mental disability, seizure disorder and adenoma sebaceum, among other anomalies. One of the major causes of mortality and morbidity in adults is the exclusive occurrence of subependymal giant cell astrocytoma (SEGA) which responds in at least 35% of cases to everolimus, mTOR inhibitor. However, drug treatment is associated with 33% rate of adverse events and requires long-term treatment Methods: In this report, we present a case of 49-year old female with TSC and a left enlarging SEGA that was approached endoscopically in order to minimize morbidity associated with open surgical approaches. Results: The use of NICO Myriad system is described in this case to achieve successful tumor debulking without post-operative neurologic morbidity. Conclusions: This report reveals the value of minimally invasive neuroendoscopic techniques in the management of challenging intraventricular tumors while avoiding injury to crucial deep venous structures.

Effect of Chronic Pancreatitis on Complications and Mortality in DM Patients: A 10-year Nationwide Cohort Study

2020 ◽  
Vol 105 (3) ◽  
pp. e739-e745 ◽  
Author(s):  
Cheng-Heng Lin ◽  
Nai-Cheng Yeh ◽  
Jhi-Joung Wang ◽  
Chung-Han Ho ◽  
Shwu-Huey Her ◽  
...  
Keyword(s):  
Cohort Study ◽  
Chronic Pancreatitis ◽  
Survival Rates ◽  
Population Based ◽  
Rank Test ◽  
Research Database ◽  
Hyperglycemic Hyperosmolar State ◽  
Increase Risk ◽  
Patients With Diabetes

Abstract Context Chronic pancreatitis (CP), is a long-term inflammation of the pancreatic parenchyma, and might increase risk of a hyperglycemia crisis or hypoglycemia in patients with diabetes mellitus (DM); however, the relationship has not been previously investigated. Objective To investigate the risk of diabetic ketoacidosis (DKA), hyperglycemic hyperosmolar state (HHS), hypoglycemia, and long-term outcomes in DM patients with CP. Design A population-based cohort study. Setting and Participants Tapping Taiwan’s National Health Insurance Research Database, we identified 506 DM patients with newly diagnosed CP from 1999 to 2010 and created a control cohort consisting of 5060 age- and sex-matched DM patients without CP from the same time period. We followed those 2 cohorts from the index date to occurrence of outcomes, the date of death or 31 December 2012. Main Outcome Measures DKA, HHS, hypoglycemia and mortality. Results DM patients with CP, who were predominantly male (88%) and younger (60% < 45 years old), had a 9.5-, 5.0-, and 3.0-fold higher risk for DKA (95% confidence interval [CI]: 6.51–13.91), HHS (95% CI: 2.85–8.62), and hypoglycemia (95% CI: 2.23–4.08), respectively. They also had lower 1-, 5-, and 10-year cumulative survival rates (98.4% vs 99.0%, 87.7% vs 96.6%, and 78.7% vs 93.6%, respectively) (log-rank test: P < .001), and a 2.43-fold higher risk for death (HR: 2.43, 95% CI: 1.82–3.27). Conclusions In Taiwan, DM patients with CP have a higher incidence of DKA, HHS, hypoglycemia, and mortality. More attention is needed for preventing hyperglycemia crisis and hypoglycemia prevention in this population.

scholarly journals Subsequent Dyslipidemia and Factors Associated with Mortality in Schizophrenia: A Population-Based Study in Taiwan

Healthcare ◽  
2021 ◽  
Vol 9 (5) ◽  
pp. 545
Author(s):  
Mei-Chi Hsu ◽  
Wen-Chen Ouyang
Keyword(s):  
Survival Rates ◽  
Population Based ◽  
Research Database ◽  
Factors Associated ◽  
Risk Of Death ◽  
Hazard Ratios ◽  
Risk Of Mortality ◽  
Taiwan National Health Insurance ◽  
Persons With Schizophrenia

Background: Persons with schizophrenia are at greater risk of developing subsequent medical conditions. To date, few studies have examined comprehensively the risks, mortality and survival rates in schizophrenia and subsequent dyslipidemia over different time periods. The objective of this study was to evaluate the occurrence of subsequent dyslipidemia after the diagnosis of schizophrenia, and factors associated with mortality and survival rate in patients with schizophrenia. Methods: We used a population-based cohort from Taiwan National Health Insurance Research Database, to investigate in patients whom were first diagnosed with schizophrenia during the period from 1997 through 2009, the risk of subsequent dyslipidemia during follow-up. Cumulative incidences and hazard ratios after adjusting for competing mortality risks were calculated. Results: A total of 20,964 eligible patients were included. Risks (i.e., comorbidity) and protective factors (i.e., statin use) have significant impacts on mortality. The mortality exhibits a U-shaped pattern by age. After 50, the risk of death increases with age. Risk of mortality before 50 increases with a decrease in age. Risks differed by the duration time to subsequent dyslipidemia after schizophrenia. Mean duration was 63.55 months in the survive group, and 43.19 months in the deceased group. The 5-, 10-, and 15-year survival rates for patients with schizophrenia and subsequent dyslipidemia were 97.5, 90, and 79.18%, respectively. Conclusion: Early occurrence of subsequent dyslipidemia is associated with increased overall mortality in patients with schizophrenia.

Subependymal nodules, giant cell astrocytomas and the tuberous sclerosis complex: a population-based study

2008 ◽  
Vol 93 (9) ◽  
pp. 751-754 ◽  
Author(s):  
F J K O'Callaghan ◽  
C N Martyn ◽  
S Renowden ◽  
M Noakes ◽  
D Presdee ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Giant Cell ◽  
Tuberous Sclerosis Complex ◽  
Population Based ◽  
Population Based Study ◽  
Subependymal Nodules

scholarly journals Intracranial aneurysms in patients with tuberous sclerosis complex: a systematic review

2019 ◽  
Vol 24 (2) ◽  
pp. 174-183
Author(s):  
Mehdi Chihi ◽  
Oliver Gembruch ◽  
Marvin Darkwah Oppong ◽  
Bixia Chen ◽  
Thiemo Florin Dinger ◽  
...  
Keyword(s):  
Systematic Review ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Intracranial Aneurysms ◽  
Developmental Disorders ◽  
Internal Carotid ◽  
Large Population ◽  
Population Based ◽  
Healthy Population ◽  
Male Patients

OBJECTIVETuberous sclerosis complex (TSC) is a rare multisystem genetic disease. Arterial wall developmental disorders, such as aneurysms, in association with TSC have been well described for extracranial vasculature. The characteristics of intracranial aneurysms (IAs) in TSC have not previously been addressed in the literature. This systematic review was performed to identify and assess the distinct characteristics of IAs in patients with TSC.METHODSThe authors searched PubMed, Scopus, and Web of Science for publications describing cases of TSC and IA reported before August 7, 2018. They also report 2 cases of IAs in TSC patients treated at their own institution.RESULTSThirty-three TSC patients with a total of 42 IAs were included in this review. Three individuals presented with subarachnoid hemorrhage. The IAs were large or giant in 57.1% and fusiform in 45.2% of the cases. Most of the IAs (61.9%, 26 of 42) originated from the internal carotid artery. There was a higher prevalence of pediatric cases (66.7%) and male patients (63.6%, 21 of 32 individuals with known sex) among the collected series.CONCLUSIONSTSC patients with IAs are characterized with a higher proportion of large/giant and fusiform IAs and young age, suggesting rapid aneurysmal growth. Furthermore, there is a distinct location pattern of IAs and an inverse sex ratio than in the healthy population. Large population-based patient registers are required to improve the understanding of epidemiology and pathophysiology of IA formation in TSC.

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