Rochester Epidemiology Project Research Articles

S0193 Outcomes of Pediatric Hospital and Community-Associated Clostridioides difficile Infection

INTRODUCTION: Incidence of Clostridioides difficile infection (CDI) in pediatric population is increasing, with paucity of data on CDI outcomes in this population, particularly in community-associated cases. METHODS: A population-based cohort study of the pediatric age group (< 18 years) from 2006 to 2017 was conducted using the Rochester Epidemiology Project. CDI was diagnosed with watery diarrhea and a positive stool assay, after exclusion of other causes of diarrhea. CDI was defined as healthcare-associated (HA) if symptoms occurred >48 hours after admission to or < =4 weeks of hospital discharge; community-acquired (CA) if there was no discharge from a hospital in the past 12 weeks; rest were indeterminate. CDI was considered severe if WBC ≥15000/µL or creatinine ≥1.5mg/dL, fulminant if there was ileus, megacolon, ICU admission, colectomy, hypotension or sepsis. Recurrence was defined with recurrent diarrhea within 8 weeks of the last episode, with positive stool assay and interim symptom resolution. Chi-square/Fischer’s exact tests were used for comparison of baseline characteristics and outcomes. P < 0.05 was considered statistically significant. RESULTS: From 2006 to 2017, 157 pediatric patients were identified with median age 3.6 years (range, 0.2–17.9), 54% (85) were female. Overall 12 patients with indeterminate CDI were excluded. Of the remaining patients, majority [67.6% (98)] had CA-CDI. Baseline characteristics of CA and HA CDI are outlined in Table 1; 48.3% (70) patients had antibiotic exposure, more in HA than CA CDI. Overall, 2.1% patients (3) had severe CDI, 9.6% (14) had fulminant CDI; 6.9% (10) required ICU admission. Overall, 8.3% (12) patients needed a change in treatment due to non-response or adverse events. Recurrence occurred in 20.1% (29) patients. Patients with CA-CDI were less likely to have fulminant disease and ICU admission compared to HA-CDI (Table 2). There were no CDI-related deaths. CONCLUSION: Community-acquired infection accounts for a majority of CDI in the pediatric age group, and is associated with better outcomes compared to healthcare-associated CDI.Table 1.: Baseline characteristics by mode of acquisition of CDI* *data presented as n (%) for categorical and median (min-max) for continuous variables) ^risk factor exposure within 90 days prior to CDI is reportedTable 2.: Outcome of CDI by mode of acquisition

Incidence and Outcomes of Home Parenteral Nutrition in Patients With Crohn Disease in Olmsted County, Minnesota.

BackgroundWe sought to estimate the incidence of home parenteral nutrition (HPN) use in a population-based cohort of patients with Crohn disease (CD), and to assess clinical outcomes and complications associated with HPN.MethodsWe used the Rochester Epidemiology Project (REP) to identify residents of Olmsted County, who were diagnosed with CD between 1970 and 2011, and required HPN.ResultsFourteen out of 429 patients (3.3%) with CD received HPN (86% female). Eleven patients (79%) had moderate–severe CD and 12 patients (86%) had fistulizing disease. Thirteen patients (93%) underwent surgery, primarily due to obstruction. Among CD incidence cases, the cumulative incidence of HPN from the date of CD diagnosis was 0% at 1 year, 0.5% at 5 years, 0.8% at 10 years, and 2.4% at 20 years. Indications for HPN included short bowel syndrome in 64%, malnutrition in 29%, and bowel rest in 21%. The median duration of HPN was 2.5 years. There was an average weight gain of 1.2 kg at 6 months, an average weight loss of 1.4 kg at 1 year, and a further weight loss of 2.2 kg at 2 years from the start of HPN. Patients were hospitalized a mean of 5 times after the start of HPN, mainly due to catheter-related bloodstream infections and thrombosis.ConclusionsLess than 4% of patients with CD need HPN. Most have moderate to severe disease with short bowel syndrome or malnutrition. Possible reasons for the patients’ weight loss could be noncompliance, and increased metabolic needs because of active disease.

RISK FOR OBSTRUCTIVE LUNG DISEASE DEVELOPMENT AFTER BILATERAL OOPHORECTOMY

SESSION TITLE: Obstructive Lung Disease Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: October 18-21, 2020 PURPOSE: Obstructive lung diseases (OLD) affect women disproportionately with higher risk of disease development and greater symptom burden. Female reproductive hormones, namely estrogen and progesterone, have been implicated as mediators in lung physiology as women advance in their reproductive lifespan. However, conflicting evidence exists on the effects of hormonal changes during menopause on lung function. We therefore studied the effect of bilateral oophorectomy (BO) on the development of OLD. METHODS: The Rochester Epidemiology Project (REP) medical records-linkage system was used to identify all premenopausal women who underwent BO for benign indications before age 50, between 1988 and 2007 in Olmsted County, Minnesota (N=1,653). Each woman was randomly matched to a referent woman born in the same year (+/-1) who had not undergone BO (N=1,653). OLD diagnostic codes were obtained from REP electronic indexes. Medical records were reviewed through 12/31/2018. Global Initiative for Chronic OLD (GOLD) and Global Initiative for Asthma (GINA) 2019 definitions were used to classify OLD on pulmonary function testing. Radiologist-interpreted reports were used to evaluate changes on computed tomography. Each woman could have more than one type of OLD. Hazard ratios were derived from Cox proportional hazards models adjusted for chronic conditions and other possible confounders at the index date, separately for each type of OLD (asthma, chronic obstructive pulmonary disease [COPD], emphysema without documented airflow limitation, or chronic bronchitis). RESULTS: Over a median of 18 years of follow up, de novo OLD was identified in 259 women who underwent BO (219 asthma, 15 COPD, 19 emphysema, 60 chronic bronchitis) and 202 referent women (168 asthma, 15 COPD, 13 emphysema, 36 chronic bronchitis). Women who underwent BO had a higher risk of asthma overall (HR 1.23, 95% CI 1.01-1.51). This was most notable in younger women age ≤45 years at BO (HR 1.54, 95% CI 1.19-1.99), especially those who did not receive estrogen treatment (ET) (HR 2.49, 95% CI 1.08-5.75). No significant risk of COPD (HR 1.01, 95% CI 0.49-2.10), emphysema (HR 1.29, 95% CI 0.66-2.53), or chronic bronchitis (HR 1.41, 95% 0.94-2.13) was found. CONCLUSIONS: Women who underwent BO, especially young women ≤45 years of age and those who did not receive ET, had an increased risk of asthma. CLINICAL IMPLICATIONS: Some studies suggest that as sex hormones wane in natural menopause, there is a decreased risk of asthma, and that ET may increase the risk. However, other studies suggest that menopause is associated with lower lung function and worse asthma symptom severity. This study suggests that age of onset of menopause and ET may influence the risks of asthma. Although a clear sexual dimorphism in airway pathology exists, further investigation is warranted to understand the impact of hormonal changes on lung pathology. DISCLOSURES: No relevant relationships by Megan Dulohery Scrodin, source=Web Response No relevant relationships by Trinh Nguyen, source=Web Response No relevant relationships by Walter Rocca, source=Web Response No relevant relationships by Carin Smith, source=Web Response Recipient of grant funding relationship with Bristol Myers Squibb Please note: >$100000 Added 03/23/2020 by Robert Vassallo, source=Web Response, value=Grant/Research Support Recipient of grant funding relationship with Sun Pharmaceuticals Please note: >$100000 Added 03/23/2020 by Robert Vassallo, source=Web Response, value=Grant/Research Support Recipient of grant funding relationship with Pfizer Please note: >$100000 Added 03/23/2020 by Robert Vassallo, source=Web Response, value=Grant/Research Support

Androgen Deprivation Therapy Use and Risk of Mild Cognitive Impairment in Prostate Cancer Patients.

We examined the association between androgen deprivation therapy (ADT) use and the risk of mild cognitive impairment (MCI) among prostate cancer patients. We included 241 cognitively unimpaired men, aged 70 to 90, with a history of prostate cancer before enrollment in the population-based Mayo Clinic Study of Aging. Using the Rochester Epidemiology Project medical records-linkage system, ADT use and length of exposure were abstracted. Follow-up visits occurred every 15 months and MCI diagnoses were made based on clinical consensus. Cox proportional hazards models, with age as the timescale, were used to examine the association between ADT use (yes/no) and length of exposure with the risk of MCI adjusting for education, apolipoprotein E, depression, and the Charlson Index score. There was no association between any ADT use (27.8% of participants) and the risk of MCI in the multivariable model [hazard ratio (HR), 1.25; 95% confidence interval (CI), 0.75-2.10]. Although not significant, there was an ADT dose-response relationship for risk of MCI: <5 years versus no use (HR, 1.08; 95% CI, 0.60-1.96) and ≥5 years versus not use (HR, 1.89; 95% CI, 0.83-4.27). ADT use among prostate cancer patients was not associated with an increased risk of developing MCI.

Colorectal cancer screening completion: An examination of differences by screening modality.

Average-risk colorectal cancer (CRC) screening is broadly recommended, using one of several endorsed test options. However, CRC screening participation rates remain below national goals. To gain further insights regarding recent, population-based patterns in overall and test-specific CRC screening participation, we conducted a retrospective study of adults, ages 50–75 years, utilizing comprehensive data resources from the Rochester Epidemiology Project (REP).Among residents of Olmsted County, MN eligible and due for CRC screening, we identified 5818 residents across three annual cohorts who completed screening between 1/1/2016 and 12/31/2018. We summarized CRC screening rates as incidence per 1000 population and used Poisson regression to test for overall and mode-specific CRC trends. We also analyzed rates of follow-up colonoscopy within 6-months after a positive stool-based screening result.While no significant differences over time were observed in overall CRC screening incidence rates among those due for screening, we observed a statistically significant increase in mt-sDNA test and statistically significant decreases in screening colonoscopy and FIT/FOBT test completion rates; differences in screening overall and by modality were observed by age, sex, and race/ethnicity. The diagnostic colonoscopy follow-up rate within six months after a positive stool-based test was significantly higher following mt-sDNA (84.9%) compared to FIT/FOBT (42.6%).In this retrospective, population-based study, overall CRC screening incidence rates remained stable from 2016 to 2018, while test-specific rates for mt-sDNA significantly increased and decreased for colonoscopy and FIT/FOBT. Adherence with follow-up colonoscopy after a positive stool-based test was significantly higher among patients who underwent mt-sDNA screening compared to FIT/FOBT.

WOMEN WITH A HISTORY OF PRIMARY INFERTILITY HAVE INCREASED RISK OF FUTURE HYSTERECTOMY AND OOPHORECTOMY

To evaluate the incidence of future hysterectomy and oophorectomy for women with a history of primary infertility compared to age-matched referent women from the same community. A population-based retrospective cohort study. A random sample of 300 women first diagnosed with primary infertility between 1980 and 1999 while a resident of Olmsted County, Minnesota, were identified through the Rochester Epidemiology Project (REP) records-linkage system (https://rochesterproject.org/). Records were manually reviewed to confirm the date (index date) and diagnosis of primary infertility. Each woman was 1:1 age-matched (±1 y) to a referent woman residing in the county at the time of the index date who had not been diagnosed with primary infertility or had a prior hysterectomy. The occurrence of hysterectomy and/or oophorectomy both prior to and after index date was collected by manual chart review. Cox proportional hazards models were fit to estimate the hazard ratio (HR) and 95% confidence interval to compare the long-term risk of hysterectomy and oophorectomy, respectively, between infertility cases and referents. The mean (SD) age at the index date was 29.9 (4.6) and 30.0 (4.6) for 300 infertility cases and 300 referents, respectively. At the time of the index date, 1 infertility case and 1 referent each had a prior bilateral salpingo-oophorectomy (BSO) and 4 infertility cases and 4 referents had a prior unilateral oophorectomy. The median duration of follow-up after the index date was 21.5 years (interquartile range (IQR), 13.0-25.5) and 20.9 years (IQR, 4.9-24.9) for the infertility cases and referents, respectively. Among those without a prior BSO at the index date, risk of subsequent oophorectomy was 2-fold higher in infertility cases compared to referent women (HR 2.06, 95% CI 1.20-3.53) (43 infertility cases and 19 referents). Infertility cases also had a significantly increased risk of hysterectomy (HR 1.56, 95% CI 1.06-2.29) (69 infertility cases and 41 referents). The three most common indications for hysterectomy among both groups (infertility cases vs. referents) were similar: fibroids (25% vs 29%), heavy menstrual bleeding (23% vs. 27%), and endometriosis (17% vs. 15%). Ten of 69 (14%) infertility cases had a hysterectomy for a gynecologic malignancy compared to 3 of 41 (7%) referents. Women with primary infertility are at increased risk of subsequent hysterectomy and oophorectomy compared to age-matched referents. Although indication for hysterectomy was similar among groups, more women with a history of primary infertility underwent hysterectomy for treatment of gynecologic malignancy. These findings are significant, as oophorectomy and hysterectomy have been previously demonstrated to negatively impact long-term health.

LONG-TERM PSYCHIATRIC ILLNESS IN WOMEN WITH PRIMARY INFERTILITY

To evaluate the long-term risk of de novo psychiatric illness in women with a history of primary infertility compared to age-matched referent women. Population-based retrospective cohort study. A random sample of 300 female residents of Olmsted County, MN, diagnosed with primary infertility from 1980-1999 were identified through the Rochester Epidemiology Project (REP) records-linkage system. Medical records were manually reviewed to confirm primary infertility diagnosis and date of diagnosis became the index date. Each woman was 1:1 age-matched (±1 y) to a referent woman residing in the county who had not been diagnosed with primary infertility or undergone hysterectomy prior to the index date. Diagnosis codes for depression, anxiety and bipolar disorder were electronically obtained for each pair using standard REP protocols. Diagnoses made prior to or up to 3 months after the index date were considered prevalent disorders. Each of the psychiatric disorders was evaluated separately; women with a prevalent disorder at index date were excluded from the estimation of risk for the other newly diagnosed disorders (de novo). Cox proportional hazards models were fit to estimate the hazard ratio (HR) and 95% confidence interval to compare the long-term risk between infertility cases and referents. Live-birth was handled as a time-dependent covariate in Cox models for each outcome. The mean ± SD age at index date was 29.9 ± 4.6 and 30.0 ± 4.6 for 300 infertility cases and 300 referents, respectively. There was no difference in prevalence of depression, anxiety, and bipolar disorder with 11.7%, 4.3% and 0.3% among infertility cases compared to 10.7%, 3.7%, and 0.7% among referents. The median duration of follow-up after index date was 21.5 years (interquartile range (IQR), 13.0-25.5) and 20.9 years (IQR, 4.9-24.9) for the infertility cases and referents, respectively. The risk of de novo bipolar disorder was 3.6 times higher for women with primary infertility (HR 3.63, 95% CI 0.77-17.09), however, this was not statistically significant as only 10 de novo diagnoses (8 infertility cases and 2 referents) were identified. We did not identify an increased risk of de novo depression (HR 1.07, 95% CI 0.79-1.45) or de novo anxiety (HR 1.25, 95% CI 0.88-1.76). Among women with primary infertility, we found no significant association between live birth and de novo bipolar disorder (HR 0.68, 95% CI 0.16-2.89), depression (HR 1.47, 95% CI 0.91-2.39), or anxiety (HR 1.48, 95% CI 0.88-2.47). We found no increased rate of anxiety or depression in women with primary infertility compared to age matched referent women. However, a trend toward higher risk of de novo bipolar disorder was identified. Live birth after diagnosis of primary infertility showed a trend toward decreased incidence of bipolar disorder but increased rates of anxiety and depression. These findings suggest a need for additional studies to evaluate patient characteristics associated with development of de novo bipolar disorder or other psychiatric disorders that may benefit from additional psychiatric evaluation.

Incidence and natural history of isolated abdominal aortic dissection: A population-based assessment from 1995 to 2015

BackgroundIsolated abdominal dissection (IAD) is an uncommon clinical problem that is less well-understood than thoracic aortic dissection (AD). We performed a population-based assessment of the incidence, natural history, and treatment outcomes of IAD to better characterize this disease. MethodsWe used the Rochester Epidemiology Project to identify all Olmsted County, MN residents with a diagnosis of AD, intramural hematoma or penetrating ulcer (1995-2015). Diagnostic imaging of all patients was reviewed to confirm the diagnosis of IAD for inclusion. Presentation, treatment, and outcomes were reviewed. Survival of IAD patients was compared to age- and sex-matched population controls 3:1. ResultsOf 133 residents with aortic syndrome (AD, intramural hematoma, or penetrating ulcer), 23 were initially diagnosed with IAD. Nine were reclassified as having a penetrating aortic ulcer and were excluded, leaving 14 patients for review (10 male [71%]; mean age, 71 years). Three patients (21%) were symptomatic (abdominal pain, back pain, hypertension) and none had malperfusion or rupture. Prior aortic dilatation was present in eight patients (57%) and Marfan syndrome in one (7%). Two patients (14%) had iatrogenic IAD. Initial management was medical in 13 and endovascular aneurysm repair in one (symptomatic subacute, infrarenal dissection with small aneurysm). The median clinical and imaging follow-up was 6.7 years (range, 0-17 years). An abdominal aortic aneurysm occurred in eight (six at the time of IAD diagnosis, one at 2.9 years, and one at 5.2 years after diagnosis). The average growth in the entire cohort was 0.9 ± 0.4 cm, which translated to an average growth rate of 0.09 cm/year. Subsequent intervention was performed in two patients; for severe aortic stenosis with claudication in one (infrarenal aortic stenting) and increasing aortic size in one (open repair). One patient required reintervention (thrombolysis and stenting for endovascular aneurysm repair limb thrombosis). Survival for IAD at 1, 3, and 5 years was 93%, 85%, and 76%, respectively, compared with population controls at 98%, 85%, and 71%, respectively (long rank P = .38). Mortality was due to cardiovascular causes in three patients (21%) and no deaths were aortic related. Major adverse cardiac events occurred in five patients (36%) owing to heart failure. ConclusionsIAD is rare. The initial management for asymptomatic patients is medical. The aortic growth rate is slow, with no aortic-related mortality and a low rate of aortic intervention. The overall mortality is similar to population controls. Heart failure and cardiac-related death are prevalent, suggesting that close cardiovascular care is needed in this patient population.

Utility of HAS-BLED and CHA2DS2-VASc Scores Among Patients With Atrial Fibrillation and Imaging Evidence of Cerebral Amyloid Angiopathy

ObjectiveTo determine the utility of the HAS-BLED (Hypertension, Abnormal renal/liver function, Stroke, Bleeding history or predisposition, Labile international normalized ratio, Elderly, Drugs/alcohol concomitantly) and CHA2DS2-VASc (Congestive heart failure, Hypertension, Age, Diabetes, previous Stroke/transient ischemic attack–VAScular disease) scores among patients on anticoagulation (AC) therapy for atrial fibrillation (AF) who have evidence of cerebral amyloid angiopathy (CAA). Patients and MethodsPatients older than 55 years with a diagnosis of AF who had a nontraumatic intracerebral hemorrhage (ICH) while on AC therapy between 1995 and 2016 were identified using the Rochester Epidemiology Project Database. Medical records were reviewed, including imaging of the brain, to identify baseline characteristics, AC use, and outcomes. ResultsA total of 65 patients were identified (mean age, 81.3 years); 35 (53.8%) had evidence of possible/probable CAA. Mean HAS-BLED score in the CAA group was significantly lower (2.1) than that of the non-CAA group (2.9; P<.001). Mortality after ICH, adjusted for HAS-BLED scores, was not significantly different among patients with and without CAA. Sixteen patients restarted on AC therapy after ICH; CHA2DS2-VASc scores were no different between this group and those who were not restarted. Among patients with CAA, the overall rate of ICH recurrence was 8.6% over 93.5 person-years of follow-up. Among patients with CAA, the rate of ICH recurrence was 3.2 per 100 patient-years, higher than their HAS-BLED scores would predict (1.9 bleeds/100 patient-years). ConclusionHAS-BLED scores were lower in patients who had evidence of CAA compared with those without, suggesting underestimation of ICH risk in patients with CAA. CHA2DS2-VASc scores did not affect resumption of AC therapy. ICH recurrence was higher in patients with CAA than their HAS-BLED scores predicted. Current risk assessment scoring systems do not accurately account for CAA in patients with AF on AC.

Connecting community-delivered evidence-based programs and the healthcare system: Piloting a learning "wellcare" system.

IntroductionMany evidence‐based programs (EBPs) have been determined in randomized controlled trials to be effective, but few studies explore the real‐world effectiveness of EBPs implemented in the natural community setting. Our study evaluated whether a novel linked infrastructure would enable such insights and continuous improvement as part of a learning healthcare‐community bridged “wellcare” ecosystem.MethodsWe created a secure, web‐based data entry and storage platform with a network of Minnesota community‐based organizations to record EBP participants' demographics and attendance, and program details. We then linked participant's information to their Rochester Epidemiology Project (REP) medical records. With this infrastructure, we conducted a proof of concept, retrospective cohort study by matching EBP participants to REP controls and comparing medical record‐documented outcomes over 1 year follow‐up.ResultsWe successfully linked EBP participant records with medical records in 77.6% of cases, and the infrastructure proved feasible and scalable. Still, key challenges remain in obtaining participant consent for data sharing. Upfront resource investments and the availability of REP‐like warehouses limit generalizability. Optimal learning will be improved by enhancements that better track program fidelity. Our pilot study established a proof‐of‐concept, but sample sizes (n = 99 for falls prevention and n = 97 chronic disease/pain management EBP completers) were too small to detect significant differences in hospital admittance as compared to matched controls for either EBP group, (OR = 0.66[0.36, 1.19]) and (OR = 0.81[0.43, 1.54]), respectively. Events were too rare to gather meaningful information about effects on fall rates.ConclusionsOur pilot demonstrates the feasibility of developing an online infrastructure that connects information from community leaders with medical record documented health outcomes, bridging the knowledge gap between community programs and the health care system. Insights gleaned from our infrastructure can be used to continuously shape community program delivery to reduce the need for formal health care services.

The Rise and Fall of Otosclerosis: A Population-based Study of Disease Incidence Spanning 70 Years.

Although it is commonly held that otosclerosis has become increasingly uncommon over recent decades, no population-based data exist to characterize this trend. Moreover, because most large epidemiologic databases within the United States primarily include cancer data, even the modern incidence of otosclerosis is unknown. The chief objective of the current work was to characterize the trend in the incidence of otosclerosis over 70 years using the unique resources of the Rochester Epidemiology Project. Population-based study. Residents of Olmsted County, Minnesota diagnosed with otosclerosis. Disease incidence from 1950 to 2017. From 1950 to 2017, 614 incident cases of otosclerosis were identified. The incidence rose from 8.9 per 100,000 person-years in the 1950s to a peak of 18.5 from 1970 to 1974. From this peak, the incidence significantly declined to 6.2 per 100,000 person-years by the early-1990s and reached a nadir of 3.2 from 2015 to 2017 (p<0.001). From 1970 to 2017, age at diagnosis (p = 0.23) and the proportion of bilateral cases (p = 0.16) did not significantly change; pure-tone average at diagnosis did not clinically appreciably change over the study period (median difference <5 dB across decades, p = 0.034). The incidence of otosclerosis drastically declined since the early-1970s. Historically considered one of the most common causes of acquired hearing loss, the low modern incidence of otosclerosis renders it legally a "rare disease" within the United States. These trends require consideration when determining trainee case requirements and developing practice guidelines.

Epidemiology of autosomal-dominant polycystic liver disease in Olmsted county.

Background & AimsIsolated autosomal-dominant polycystic liver disease (ADPLD) is generally considered a rare disease. However, the frequency of truncating mutations to ADPLD genes in large, population sequencing databases is 1:496. With the increasing use of abdominal imaging, incidental detection of hepatic cysts and ADPLD has become more frequent. The present study was performed to ascertain the incidence and point prevalence of ADPLD in Olmsted County, MN, USA, and how these are impacted by the increasing utilisation of abdominal imaging.MethodsThe Rochester Epidemiology Project and radiology databases of Mayo Clinic and Olmsted Medical Center were searched to identify all subjects meeting diagnostic criteria for definite, likely, or possible ADPLD. Annual incidence rates were calculated using incident cases during 1980–2016 as numerator, and age- and sex-specific estimates of the population of Olmsted County as denominator. Point prevalence was calculated using prevalence cases as numerator, and age- and sex-specific estimates of the population of Olmsted County on 1 January 2010 as denominator.ResultsThe incidence rate and point prevalence of combined definite and likely ADPLD were 1.01 per 100,000 person-years and 9.5 per 100,000 population, respectively. Only 15 of 35 definite and likely incident ADPLD cases had received a diagnostic code, and only 8 had clinically significant hepatomegaly. The incidence rates were much higher when adding possible cases, mainly identified through radiology databases, particularly in recent years and in older patients because of the increased utilisation of imaging studies.ConclusionsClinically significant isolated ADPLD is a rare disease with a prevalence <1:10,000 population. The overall prevalence of ADPLD, however, to a large extent not clinically significant, is likely much higher and closer to the reported genetic prevalence.Lay summaryIsolated autosomal-dominant polycystic liver disease (ADPLD) is generally considered a rare disease. However, we demonstrate that it is a relatively common disease, which is rarely (<1:10,000 population) clinically significant.

Burden and causes of readmissions following initial discharge after aortic syndromes

BackgroundAortic syndromes, including aortic dissection (AD), intramural hematoma (IMH), and penetrating aortic ulcer (PAU), carry significant morbidity and mortality; few data exist regarding burden and causes of related rehospitalizations following initial discharge. MethodsThe study was conducted using the Rochester Epidemiology Project. All adult residents (age ≥18 years) with an incident diagnosis of AD, IMH, and PAU (1995-2015) were identified from the Rochester Epidemiology Project using the International Classification of Diseases, Ninth Revision and Tenth Revision codes and Hospital Adaptation of the International Classification of Diseases, second edition, codes. Assessment of any-cause (aortic and cardiovascular), aorta-related, or cardiovascular-related readmissions was determined following date of hospital discharge or diagnosis date (ie, the index event). ResultsA total of 117 patients of 130 cases of AD, IMH, and PAU included in the initial study population survived the index event and were evaluated. The median age of diagnosis was 74 years, and 70 (60%) were male. A total of 79 patients (68%) experienced at least one readmission. The median time to first any-cause, cardiovascular, and aortic readmission was 143, 861, and 171 days, respectively. The cumulative incidence of any-cause readmissions at 2, 4, and 10 years was 45%, 55%, and 69%, respectively. The cumulative incidence of cardiovascular readmissions at 2, 4, and 10 years was 15%, 20%, and 28%, respectively. The cumulative incidence of aortic readmissions at 2, 4, and 10 years was 38%, 46%, and 59%, respectively. Overall survival for the entire cohort at 2, 4, and 10 years was 84%, 75%, and 50%, respectively. ConclusionsReadmissions following initial discharge after diagnosis of aortic syndrome are common and not different across specific disease types. Whereas aorta-related rehospitalizations occur in more than half of patients but tend to be earlier, cardiovascular-related rehospitalizations tend to happen later in about one-third of patients. This may suggest the need for early follow-up focused on aortic complications, whereas later follow-up should address cardiovascular events.

High Mortality Rates Among Patients With Non‐Traumatic Intracerebral Hemorrhage and Atrial Fibrillation on Antithrombotic Therapy Are Independent of the Presence of Cerebral Amyloid Angiopathy: Insights From a Population‐Based Study

BackgroundIntracerebral hemorrhage (ICH) risk is higher in elderly patients with atrial fibrillation on antithrombotic therapy as well as those with cerebral amyloid angiopathy (CAA). We investigated if mortality among patients with atrial fibrillation on antithrombotic therapy presenting with non‐traumatic ICH was influenced by underlying CAA.Methods and ResultsWe used the Rochester Epidemiology Project to identify 6045 patients with atrial fibrillation aged >55 years on anticoagulation or antiplatelet therapy from 1995 to 2016. Seventy‐four patients in this cohort presented with non‐traumatic ICH. Medical records including imaging data were reviewed to identify those with CAA and record baseline variables and outcomes of interest; 38 of our 74 patients (51.4%) (mean age 81.5 years) met Modified Boston Criteria for possible or probable CAA. Twenty‐six of 74 patients (35%) died during the first 30 days while 56 of the 74 (76%) patients died by 10 years follow‐up after index ICH. Overall mortality was not significantly different between the CAA and non‐CAA groups at any point of time during follow‐up (P=0.89) even amongst patients restarted on anticoagulation +/− antiplatelet (n=19) (P=0.46) or those patients restarted only on antiplatelet therapy (n=22) (P=0.66). Three of the 41 patients who restarted on antithrombotic therapy had a recurrent ICH; these 3 patients met criteria for possible or probable CAA.ConclusionsAlthough more than half of our patients with atrial fibrillation on antithrombotic therapy and non‐traumatic ICH met Modified Boston Criteria for CAA, CAA did not significantly influence the high mortality seen in this cohort.

Self-injurious and suicidal behavior in young adults, teens, and children with epilepsy: A population-based study.

Whereas studies in adult epilepsy patients have shown higher rates of suicidal ideation and attempt, such studies in children are limited. Using the Rochester Epidemiology Project database, we compared the risk of self-injurious behavior and suicidal ideation in a population-based cohort of childhood epilepsy to controls. We studied 339 cases with epilepsy and 678 age- and sex-matched controls followed to a median age of 24.7 and 23.4years, and identified 98 subjects with self-injurious behavior or suicidal ideation (43 with epilepsy and 55 controls). All behaviors were categorized using the Columbia Suicide Severity Rating Scale. Those with epilepsy had a significantly higher rate of any self-injurious behavior and suicidal ideation (hazard ratio [HR] = 1.56, 95% confidence interval [CI] = 1.04-2.35) and tended to have an increased risk of suicidal ideation and attempt (HR = 1.48, 95% CI = 0.93-2.37). The prevalence of preceding mood and substance abuse disorders was similarly high in both cases and controls with self-injurious behavior or suicidal ideation; however, preceding attention-deficit/hyperactivity disorder was more than twice as common in the epilepsy cases. Among cases with epilepsy, we did not identify any specific epilepsy-related variable that was significantly correlated with risk of self-injurious behavior or suicidal ideation. Children, teens, and young adults with a history of childhood epilepsy are at greater risk of self-injurious behavior, highlighting the need for careful screening of mental health concerns as part of routine epilepsy care.

Population-Based Incidence of Optic Neuritis in the Era of Aquaporin-4 and Myelin Oligodendrocyte Glycoprotein Antibodies

To re-evaluate the population-based incidence of optic neuritis in the era of aquaporin-4-immunoglobulin G (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG)-IgG, which are biomarkers of optic neuritis that is distinct from multiple sclerosis (MS). Over the past 15 years, 2 new biomarkers have been discovered that allow for further characterization of the cause of atypical optic neuritis: AQP4-IgG and MOG-IgG. Retrospective, population-based cohort. population-based. all residents of Olmsted County, Minnesota, with optic neuritis diagnosed between January 1, 2000, and December 31, 2018. The Rochester Epidemiology Project database was used to identify patients. Sera were tested for AQP4-IgG and MOG-IgG by using a live-cell-based flow cytometry assay. Main outcome measurements were the incidence and cause of optic neuritis. Optic neuritis was diagnosed in 110 patients, providing an annual incidence of 3.9 per 100,000. The final diagnosis was MS in 57%, idiopathic in 29%, MOG-IgG-associated disorder in 5%, AQP4-IgG-seropositive neuromyelitis optic spectrum disorder (NMOSD) in 3%, infectious type in 2%, sarcoidosis in 2%, seronegative NMOSD in 1%, and medication-related in 1%. All 3 patients positive for AQP4-IgG had more than 1 optic neuritis attack, 2 with residual no light perception vision in at least 1 eye. Among MOG-IgG-positive patients, 4 of 6 patients had recurrent optic neuritis, and all 6 had a final visual acuity of 20/30 or better. At a population level, AQP4-IgG and MOG-IgG account for 9% of optic neuritis and are associated with recurrent attacks, but MOG-IgG optic neuritis has a better visual outcome than AQP4-IgG optic neuritis.

Outcomes of incident atrial fibrillation in heart failure with preserved or reduced ejection fraction: A community-based study.

The best management strategy for patients with atrial fibrillation (AF) with heart failure (HF) and preserved left ventricular ejection fraction (LVEF) is unknown. This cohort study was conducted in Olmsted County, Minnesota, with resources of the Rochester Epidemiology Project. Patients with incident AF occurring between 2000 and 2014 with a prior or concurrent HF were included. Patients with LVEF ≥ 50% were designated as HF and preserved ejection fraction (HFpEF) and those with LVEF < 50% were designated as HF and reduced ejection fraction (HFrEF). Rhythm control in the first year after AF diagnosis was defined as prescriptions for an antiarrhythmic drug, catheter ablation, or maze procedure. The primary endpoint was all-cause mortality. The secondary endpoints were cardiovascular death, cardiovascular hospitalization, and stroke or transient ischemic attack. Of 859 patients (age, 77.2 ± 12.1 years; 49.2%, female), 447 had HFpEF-AF, and 412 had HFrEF-AF. There was no difference in all-cause mortality (10-year mortality, 83% vs 79%; p = .54) or secondary endpoints between the HFpEF-AF and HFrEF-AF, respectively. Compared with the rate control strategy, rhythm control in HFpEF-AF patients (n = 40, 15.9%) offered no survival benefits (adjusted HR, 0.70; 95% CI, 0.42-1.16; p = .16), whereas rhythm control in HFrEF-AF patients (n = 52, 22.5%) decrease cardiovascular mortality (HR, 0.38; 95% CI, 0.17-0.86; p = .02). Patients with HFpEF-AF and HFrEF-AF had similar poor prognoses. Rhythm control strategy was seldom adopted in community care in patients with HF and AF. A rhythm control strategy may provide survival benefit for patients with HFrEF-AF and the benefit of rhythm control in patients with HFpEF-AF warrants further study.

Lumbar Puncture Increases Risk of Lumbar Degenerative Disc Disease: Analysis From the Rochester Epidemiology Project.

A population-based, retrospective cohort study. To determine whether Lumbar puncture (LP) is associated with risk of degenerative disc disease (DDD) progression. Etiology of DDD is poorly understood and has been suggested to be multifactorial, with pro-inflammatory cytokine mediators playing a pathophysiological role. While it is known that LP is associated with local inflammation, a clear link has not been previously established between LP and DDD. In this study, we performed a population-based matched cohort analysis using the medical records linkage system of the Rochester Epidemiology Project to investigate this possible association. Between 2004 and 2009, a total of 950 cases who underwent LP and 1876 referent subjects (who did not undergo an LP procedure), matched by age, sex and BMI were identified. Univariate and multivariable Cox proportional hazards analyses were used to estimate the risk of DDD among patients undergoing LP. The cumulative probability of developing DDD from the time of LP procedure was 13.8% among LP cases (95% CI 11.5%-16.1%) vs. 9.4% among controls (95%CI 8.1%-10.8%) at 5 years. On multivariable Cox regression analysis, patients undergoing an LP were found to have a significantly higher risk of developing DDD (HR 1.33; 95%CI 1.10-1.60; p = 0.003). Use of fluoroscopic guidance while performing an LP was associated with a significantly lower risk of DDD (OR 0.55, 95% CI 0.30, 1.00, p value = 0.049) when compared to patients who did underwent LP without fluoroscopy. This investigation is the first to demonstrate a possible link between LP and higher risk of developing DDD. In addition, we show that using image-guided LP might attenuate that risk. 4.

Predictors of Long-term Aortic Growth and Disease Progression in Patients With Aortic Dissection, Intramural Hematoma, and Penetrating Aortic Ulcer

The objective was to investigate predictors of long-term aortic diameter change and disease progression in a population cohort of patients with newly diagnosed aortic dissection (AD), intramural hematoma (IMH), or penetrating aortic ulcer (PAU). We used the Rochester Epidemiology Project record linkage system to identify all Olmsted County, Minnesota, residents diagnosed with AD, IMH, and PAU (1995-2015). Only patients with at least two available imaging studies were included. The primary outcome was aortic diameter change. Secondary outcomes were freedom from disease progression (defined by any related intervention, aortic aneurysm, new aortic syndrome, rupture, or death) and disease resolution (defined by complete spontaneous radiologic disappearance). Linear regression models were used to assess aortic growth rate; Cox proportional hazards models were used to identify predictors of disease progression or resolution. Of 133 total incident cases, 46 ADs, 12 IMHs, and 28 PAUs with sufficient imaging data were included in the analysis. Overall median follow-up was 8.1 years. Aortic diameter increase was seen in 40 ADs (87%; median 1.0 mm/y), 5 IMHs (42%; median, 0.2 mm/y), and 14 PAUs (50%; median, 0.4 mm/y) during follow-up. A symptomatic presentation (P = .045), connective tissue disorders (P = .005), and initial maximum aortic diameter >42 mm (P = .013) were significant predictors of aortic growth rate for AD. Female sex (P = .013) and initial PAU depth >9 mm (P = .047) were significantly associated with growth rate in PAUs. No predictors of aortic growth rate were identified for IMHs. At 10 years, freedom from disease progression was 22% (95% confidence interval [CI], 12-41) for ADs, 44% (95% CI, 22-92) for IMHs, and 46% (95% CI, 27-78) for PAUs; most events occurred within 5 years (n = 34 [73%]), 9 (17%) after 5 to 10 years, and 5 (9%) >10 years from the diagnosis. Initial maximum aortic diameter was a significant predictor of IMH progression (hazard ratio, 1.4; 95% CI, 1.0-1.9; P = .037); no progression predictors were identified for ADs and PAUs. No AD spontaneously resolved; mortality-free resolution rate at 10 years was 22% (95% CI, 0-45) for IMHs and 11% (95% CI, 0-23) for PAUs. There were no predictors of disease resolution. Aortic growth is observed in most patients with AD, IMH, or PAU. Disease progression is frequent and may occur also after 10 years from the initial diagnosis, whereas a spontaneous resolution is uncommon. Follow-up imaging, particularly early after aortic events, is important in these patients. High-risk predictors of aortic growth rate and disease progression should be used to additionally tailor appropriate aortic follow-up.

The Long-term Fate of Aortic Branches in Patients With Aortic Dissection

ABSTRACT Objective Late morbidity and mortality related to aortic branches in patients with aortic dissection (AD) are not well described. We aimed to investigate the fate of aortic branches in a population cohort of patients with newly diagnosed AD. Methods We used the Rochester Epidemiology Project record linkage system to identify all Olmsted County, MN, residents diagnosed with AD from 1995 to 2015. Only patients with >30 days of available follow-up imaging were included in the analysis. The primary outcome was freedom from any branch-related event (any intervention, aneurysm, malperfusion, rupture, or death occurring after the acute phase >14 days). Secondary outcome was the diameter change of the aortic branches. Univariate and multivariable Cox proportional hazards models were used to identify predictors of branch-related events; univariate and multivariate linear regression models were used to assess aortic branches growth rate. Results Of 77 total incident AD cases, 58 patients who survived with imaging follow-up were included, 28 (48%) with type A and 30 (52%) with type B AD. The presentation was acute in 39 (67%) cases; 6 (10%) had branch malperfusion. Of 177 aortic branches involved by the AD, 81 (46%) arose from the true lumen, 33 (19%) from the false lumen, 63 (36%) from both. After the acute phase, freedom from any branch-related event at 15 years was 48% (95%CI 32-70). Thirty-one branch-related events occurred in 19 patients over 15 years: 12 interventions (76% freedom, 95%CI 63-92), 10 aneurysms (67% freedom, 95%CI 50-90), 8 malperfusions (76% freedom, 95%CI 61-94) and 1 rupture (94% freedom, 95%CI 84-100). There were no branch-related deaths. Type B AD (HR 3.5, 95%CI 1.1-10.8; P=.033), patency of the aortic false lumen (HR 6.8, 95% CI 1.1-42.2; P=.038) and malperfusion syndrome at presentation (HR 6.0, 95% CI 1.3-28.6; P=.023) were predictors of late aortic branches-related events. Overall growth rate of aortic branches was 1.3±3.0 mm/year. Patency of the aortic false lumen, initial branch diameter, and Marfan syndrome were significantly associated with diameter increase. Conclusions In patients with AD, aortic branch involvement was responsible for a significant long-term morbidity, without any related mortality. Type B AD, patency of the aortic false lumen, or malperfusion syndrome at presentation had a higher risk of branch events during the long-term follow-up. Dilatation of the aortic branches was observed in one third of cases during follow-up, in particular in case of a patent aortic false lumen or Marfan syndrome.