Last data update: Apr 22, 2024. (Total: 46599 publications since 2009)
Records 1-25 (of 25 Records) |
Query Trace: Maddox RA [original query] |
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Suicides in National Hormone Pituitary Program recipients of cadaver-derived human growth hormone
Abrams JY , Mills JL , Schonberger LB , Chang D , Maddox RA , Belay ED , Leschek EW . J Endocr Soc 2023 7 (12) bvad130 CONTEXT: Numerous reports of suicide among individuals who received cadaver-derived human growth hormone (c-hGH) through the National Hormone Pituitary Program (NHPP) raised the alarm for potentially increased suicide risk. OBJECTIVE: We conducted a study to assess suicide risk in the NHPP cohort and identify contributing factors to facilitate early recognition and intervention. METHODS: The study population consisted of patients receiving NHPP c-hGH starting from 1957, and cohort deaths with an ICD code consistent with suicide or possible suicide through 2020 were evaluated. Descriptive data were extracted from medical records. Standardized mortality ratios (SMRs) to compare the observed number of suicide deaths in the cohort to the expected number were calculated using general population suicide rates by sex, age group, and time period. RESULTS: Among 6272 patients there were 1200 all-cause cohort deaths, of which 55 (52 male, 3 female) were attributed to suicide. Of these, 47 were identified by ICD code alone compared to an expected count of 37.8 (SMR = 1.25, 95% CI 0.91-1.66). Among male cohort members, the SMR was 1.33 (95% CI 0.97-1.78). Elevated risk of suicide was detected for cohort members aged 25-34 (SMR = 1.79, 95% CI 1.06-2.83) and during the period from September 19, 1985, to December 31, 1998 (SMR = 1.70, 95% CI 1.02-2.65). CONCLUSION: Overall, the observed number of suicides among NHPP c-hGH recipients was not significantly higher than expected. However, certain subgroups may be at elevated risk of suicide. Studies are needed to better understand the nature and magnitude of suicide risk among c-hGH recipients to facilitate early intervention to prevent suicide deaths. |
First-line corticosteroids for Kawasaki disease: Pulse versus multiple dose
Abrams JY , Ae R , Maddox RA , Schonberger LB , Nakamura Y , Belay ED . Pediatr Int 2022 64 (1) e15112 BACKGROUND: Kawasaki disease (KD) can result in severe coronary artery abnormalities (CAAs). Corticosteroids added to initial standard intravenous immunoglobulin (IVIG) treatment may decrease the risk for these complications. Different corticosteroid regimens (single-day high dose pulse vs multiple lower doses) may contribute to the discrepant results of prior studies. METHODS: Using data from the 22nd, 23(rd) , and 24th Japanese nationwide KD surveys (2011-2016), we identified KD patients who did not have CAAs at first presentation and who were treated with either pulse or multiple-dose corticosteroids as part of their initial treatment. Occurrence of subsequent CAAs and treatment failure were compared between the treatment regimens and adjusted odds ratios were calculated controlling for sex, age group, illness day at first treatment, survey, and recurrent KD. RESULTS: There were 782 KD patients who received pulse corticosteroid treatment and 4,817 who received multiple dose treatment. Patients receiving multiple dose treatment were less likely to develop CAAs (5.5% vs 8.3%, OR 0.64; 95% CI: 0.48-0.85) or treatment failure (21.4% vs 41.6%; OR: 0.38; 95% CI: 0.33-0.45). Adjusted analyses showed similar protective effects of multiple-dose treatment against CAAs (OR: 0.67, 95% CI: 0.51-0.90) and treatment failure (OR: 0.39, 95% CI: 0.33-0.46). CONCLUSIONS: Multiple-dose corticosteroid combination treatment resulted in substantially improved outcomes in KD patients compared to pulse treatment. For patients who may be at elevated risk of treatment failure or CAA, use of multiple-dose corticosteroids in conjunction with IVIG is likely to provide considerable clinical benefit. |
Kawasaki Disease With Coronary Artery Lesions Detected at Initial Echocardiography
Ae R , Maddox RA , Abrams JY , Schonberger LB , Nakamura Y , Kuwabara M , Makino N , Kosami K , Matsubara Y , Matsubara D , Sasahara T , Belay ED . J Am Heart Assoc 2021 10 (7) e019853 Background Detection of coronary artery lesions (CALs) at initial echocardiography can aid in diagnosing Kawasaki disease (KD) and inform primary adjunctive treatments. We aimed to characterize patients with KD with CALs detected at initial echocardiography. Methods and Results We analyzed data from the nationwide Japanese KD survey that contained information on 103 222 population-based patients diagnosed with KD across Japan during 2011 to 2018. Patients with CALs detected at initial echocardiography were assessed by age, day of illness, and number of principal KD signs (≥3). Multivariable logistic regression analysis was performed to evaluate factors independently associated with CAL detection. Overall, 3707 (3.6%) patients had CALs detected at initial echocardiography. Patients aged <12 and ≥60 months were associated with CAL detection (adjusted odds ratio [95% CI], 1.28 [1.18‒1.39] and 1.32 [1.20‒1.45], respectively; reference, 12‒59 months). Patients with delayed hospital visits were increasingly at higher risk for CAL detection (days 7‒8, 1.84 [1.63‒2.08]; days 9-10, 4.30 [3.58-5.15]; and days ≥11, 9.12 [7.63‒10.90]; reference, days 1-4). Patients with 3 or 4 principal KD signs were independently associated with CAL detection (1.75 [1.63‒1.88]). These patients were significantly more likely to be aged <12 months but were not associated with delayed hospital visit. Younger patients visited at earlier days of illness. Conclusions Timely diagnosis could be beneficial for patients with KD. However, even when the hospital visit occurred early in the course of illness, patients with 3 or 4 principal KD signs, especially younger patients, were at higher risk of CAL detection at initial echocardiography. |
Toxic shock syndrome in patients younger than 21 years of age, United States, 2006-2018
Leung J , Abrams JY , Maddox RA , Godfred-Cato S , Schonberger LB , Belay ED . Pediatr Infect Dis J 2021 40 (3) e125-e128 We examined the incidence of toxic shock syndrome in the United States during 2006-2018 among persons <21 years old with commercial or Medicaid-insurance using administrative data. There were 1008 commercially-insured and 481 Medicaid-insured toxic shock syndrome cases. The annual rate was 1 per 100,000 and stable over time. Rates were even lower in children <5 years old and stable over time. |
Kawasaki disease and Kawasaki disease shock syndrome hospitalization rates in the United States, 2006-2018
Maddox RA , Person MK , Kennedy JL , Leung J , Abrams JY , Haberling DL , Schonberger LB , Belay ED . Pediatr Infect Dis J 2020 40 (4) 284-288 BACKGROUND: Kawasaki disease (KD) is a febrile illness of unknown etiology. Patients with Kawasaki disease shock syndrome (KDSS) may present with clinical signs of poor perfusion and systolic hypotension in addition to typical KD features. The United States Centers for Disease Control and Prevention analyzes and interprets large hospitalization databases as a mechanism for conducting national KD surveillance. METHODS: The Kids' Inpatient Database (KID), the National (Nationwide) Inpatient Sample (NIS), and the IBM MarketScan Commercial (MSC) and MarketScan Medicaid (MSM) databases were analyzed to determine KD-associated hospitalization rates and trends from 2006 to the most recent year of available data. KD and potential KDSS hospitalizations were defined using International Classification of Disease-Clinical Modification codes. RESULTS: For the most recent year, the KD-associated hospitalization rates for children <5 years of age were 19.8 (95% CI: 17.2-22.3, KID: 2016), 19.6 (95% CI: 16.8-22.4, NIS: 2017), 19.3 (MSC: 2018), and 18.4 (MSM: 2018) per 100,000. There was no indication of an increase in KD rates over the time period. Rates of potential KDSS among children <18 years of age, ranging from 0.0 to 0.7 per 100,000, increased; coding indicated potential KDSS for approximately 2.8%-5.3% of KD hospitalizations. CONCLUSIONS: Analyses of these large, national databases produced consistent KD-associated hospitalization rates, with no increase over time detected; however, the percentage of KD hospitalizations with potential KDSS increased. Given reports of increasing incidence elsewhere and the recent identification of a novel virus-associated syndrome with possible Kawasaki-like features, continued national surveillance is important to detect changes in disease epidemiology. |
Human prion disease surveillance in Washington state, 2006-2017
Sánchez-González L , Maddox RA , Lewis LC , Blevins JE , Harker EJ , Appleby BS , Person MK , Schonberger LB , Belay ED , DeBolt C , Lofy KH . JAMA Netw Open 2020 3 (10) e2020690 IMPORTANCE: Human prion disease surveillance is critical to detect possible cases of variant Creutzfeldt-Jakob disease and other acquired forms of prion disease in the United States. Results are presented here that describe 12 years of surveillance in Washington, the only US state that has reported the presence of classic bovine spongiform encephalopathy, an animal prion disease that has been shown to transmit to humans. OBJECTIVE: To describe the current prion disease surveillance system in Washington and the epidemiological and clinical results of surveillance from 2006 through 2017. DESIGN, SETTING, AND PARTICIPANTS: This cross-sectional study reports findings from the human prion disease surveillance system in place in Washington state from January 1, 2006, through December 31, 2017. Participants included Washington residents with a clinical suspicion of human prion disease or suggestive test results from the National Prion Disease Pathology Surveillance Center or with prion disease listed as a cause of death on the death certificate. Data for this report were analyzed from June 1, 2016, to July 1, 2020. EXPOSURE: Human prion disease diagnosis. MAIN OUTCOMES AND MEASURES: The main outcome was incidence of human prion disease cases, including identification of variant Creutzfeldt-Jakob disease. RESULTS: A total of 143 human prion disease cases were detected during the study period, none of which met criteria for a variant Creutzfeldt-Jakob disease diagnosis. Among 137 definite or probable cases, 123 (89.8%) occurred in persons aged 55 years or older, with a median age at death of 66 years (range, 38-84 years). Most patients were White (124 [92.5%] among 134 with reported race), and slightly over half were male (70 [51.1%]). The average annual age-adjusted prion disease incidence was 1.5 per million population per year, slightly higher than the national rate of 1.2 per million. A total of 99 cases (69.2%) were confirmed by neuropathology. Sporadic prion disease was the most common diagnosis, in 134 cases (93.7%), followed by familial prion disease in 8 cases (5.6%). One iatrogenic prion disease case (0.7%) was also reported. CONCLUSIONS AND RELEVANCE: The findings of this cross-sectional study suggest that demographic characteristics of patients with prion disease in Washington are consistent with national findings. The slightly higher incidence rate may be due to the state's enhanced surveillance activities, including close collaboration with key partners and educational efforts targeted toward health care providers. Results indicate that surveillance will continue to be beneficial for monitoring epidemiological trends, facilitating accurate diagnoses, and detecting variant Creutzfeldt-Jakob disease or other emerging human prion disease cases. |
Corticosteroids added to initial intravenous immunoglobulin treatment for the prevention of coronary artery abnormalities in high-risk patients with Kawasaki disease
Ae R , Abrams JY , Maddox RA , Schonberger LB , Nakamura Y , Kuwabara M , Makino N , Matsubara Y , Kosami K , Sasahara T , Belay ED . J Am Heart Assoc 2020 9 (17) e015308 BACKGROUND Randomized controlled trials previously provided different conclusions about the superiority of adding corticosteroids to initial intravenous immunoglobulin treatment for the prevention of coronary artery abnormalities in patients with Kawasaki disease (KD). To further assess this issue, we analyzed large-scale data from nationwide KD surveys in Japan, where combination treatment (corticosteroids added to initial standard intravenous immunoglobulin treatment) has become commonly used for patients at high risk for KD. METHODS AND RESULTS Standard intravenous immunoglobulin treatment and combination treatment were compared using data from time periods with and without combination treatment. Outcome measures were coronary artery abnormalities and initial intravenous immunoglobulin treatment failure. Hospitals where ≥20% of patients received combination treatment were identified, and treatment and control groups were selected via matching by age, sex, illness day at initial treatment, and KD recurrence. Matched group selection and subsequent analyses were conducted 1000 times to minimize sampling bias and potential confounders (bootstrapping). From 115 hospitals, 1593 patients with KD in the treatment group and 1593 controls were selected for each of the 1000 sample iterations. The median proportion of patients who developed coronary artery abnormalities among the treatment group and controls were 4.6% (95% CI, 3.8%-5.8%) and 8.8% (95% CI, 7.5%-10.0%), respectively: an estimated risk ratio of 0.53 (0.41-0.67). A median of 14.1% (95% CI, 12.4%-15.9%) of the patients in the treatment group and 21.7% (95% CI, 19.8%-23.4%) in the controls had treatment failure: an estimated risk ratio of 0.65 (0.56-0.75). CONCLUSIONS Combination treatment reduced coronary artery abnormality risk by an estimated 47% and treatment failure by 35%. Multiple-dose corticosteroids may provide benefit in selected patients at high risk for KD. |
Diagnosis of prion diseases by RT-QuIC results in improved surveillance
Rhoads DD , Wrona A , Foutz A , Blevins J , Glisic K , Person M , Maddox RA , Belay ED , Schonberger LB , Tatsuoka C , Cohen ML , Appleby BS . Neurology 2020 95 (8) e1017-e1026 OBJECTIVE: We present the National Prion Disease Pathology Surveillance Center's (NPDPSC) experience using cerebrospinal fluid (CSF) real time quaking induced conversion (RT-QuIC) as a diagnostic test, examine factors associated with false negative RT-QuIC results, and investigate RT-QuIC's impact on prion disease surveillance. METHODS: Between May 2015-April 2018, the NPDPSC received 10,498 CSF specimens that were included in the study. Sensitivity and specificity analyses were performed using 567 autopsy verified cases. Prion disease type, demographic characteristics, specimen color, and time variables were examined for association with RT-QuIC results. The effect of including positive RT-QuIC cases in prion disease surveillance was examined. RESULTS: The diagnostic sensitivity and specificity of RT-QuIC across all prion diseases was 90.3% and 98.5%, respectively. Diagnostic sensitivity was lower for fatal familial insomnia, Gerstmann-Straussler-Scheinker disease, sporadic fatal insomnia, variably protease sensitive prionopathy, and the VV1 and MM2 subtypes of sCJD. Individuals with prion disease and negative RT-QuIC results were younger, had elevated tau levels, and non-elevated 14-3-3 levels compared to RT-QuIC positive cases. Sensitivity was high throughout the disease course. Some cases that initially tested RT-QuIC negative had a subsequent specimen test positive. Including positive RT-QuIC cases in surveillance statistics increased laboratory-based case ascertainment of prion disease by 90% over autopsy alone. CONCLUSIONS: RT-QuIC has high sensitivity and specificity for diagnosing prion diseases. Sensitivity limitations are associated with prion disease type, age, and related CSF diagnostic results. RT-QuIC greatly improves laboratory-based prion disease ascertainment for surveillance purposes. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that 2(nd) generation real time quaking-induced conversion (RT-QuIC) identifies prion disease with sensitivity of 90.3% and specificity of 98.5%, among patients being screened for these diseases due to concerning symptoms. |
Outcomes in Kawasaki disease patients with coronary artery abnormalities at admission
Ae R , Abrams JY , Maddox RA , Schonberger LB , Nakamura Y , Kuwabara M , Makino N , Matsubara Y , Matsubara D , Kosami K , Sasahara T , Belay ED . Am Heart J 2020 225 120-128 BACKGROUND: Previous studies demonstrated that coronary artery lesions (CALs) resulting from Kawasaki disease (KD) can improve over time. However, limited information is available on sub-acute outcomes of CALs detected at admission during KD illness. METHODS: The nationwide Japanese KD survey contained substantial information on KD patients with CALs detected at admission and who received standard IVIG treatment within 10 days of disease onset. Coronary outcomes were evaluated by changes in CALs from admission to the first assessment at 30 days from disease onset in three categories: improved, unchanged, and progressed. Ordinal logistic regression analysis was performed to evaluate factors associated with the outcomes. RESULTS: Of 2024 patients with CALs detected at admission, improved, unchanged, and progressed outcomes were found in 1548 (76.5%), 390 (19.3%), and 86 (4.2%), respectively. Over 80% of patients with coronary artery (CA) dilatations had improved outcome. Independent factors associated with worse outcomes were larger-size CALs (adjusted ORs [95% CIs]: CA aneurysm=5.13 [3.65-7.22] and giant CA aneurysms=7.49 [3.56-15.72] compared with CA dilatation, respectively), age>/=60 months (1.45 [1.08-1.94] compared with 12-59 months), recurrent KD (1.57 [1.07-2.29]), parental history of KD (2.23 [1.02-4.85]), and delayed admission (8-10 days from disease onset: 1.76 [1.21-2.57] compared with 1-4 days). CONCLUSIONS: KD patients with larger CALs, >/=60 months old, and with recurrent status or parental history may require more rigorous treatment. In addition, delayed admission may result in worse coronary outcome, indicating that prompt diagnosis and treatment are required. |
COVID-19 Among Workers in Meat and Poultry Processing Facilities - 19 States, April 2020.
Dyal JW , Grant MP , Broadwater K , Bjork A , Waltenburg MA , Gibbins JD , Hale C , Silver M , Fischer M , Steinberg J , Basler CA , Jacobs JR , Kennedy ED , Tomasi S , Trout D , Hornsby-Myers J , Oussayef NL , Delaney LJ , Patel K , Shetty V , Kline KE , Schroeder B , Herlihy RK , House J , Jervis R , Clayton JL , Ortbahn D , Austin C , Berl E , Moore Z , Buss BF , Stover D , Westergaard R , Pray I , DeBolt M , Person A , Gabel J , Kittle TS , Hendren P , Rhea C , Holsinger C , Dunn J , Turabelidze G , Ahmed FS , deFijter S , Pedati CS , Rattay K , Smith EE , Luna-Pinto C , Cooley LA , Saydah S , Preacely ND , Maddox RA , Lundeen E , Goodwin B , Karpathy SE , Griffing S , Jenkins MM , Lowry G , Schwarz RD , Yoder J , Peacock G , Walke HT , Rose DA , Honein MA . MMWR Morb Mortal Wkly Rep 2020 69 (18) Congregate work and residential locations are at increased risk for infectious disease transmission including respiratory illness outbreaks. SARS-CoV-2, the virus that causes coronavirus disease 2019 (COVID-19), is primarily spread person to person through respiratory droplets. Nationwide, the meat and poultry processing industry, an essential component of the U.S. food infrastructure, employs approximately 500,000 persons, many of whom work in proximity to other workers (1). Because of reports of initial cases of COVID-19, in some meat processing facilities, states were asked to provide aggregated data concerning the number of meat and poultry processing facilities affected by COVID-19 and the number of workers with COVID-19 in these facilities, including COVID-19-related deaths. Qualitative data gathered by CDC during on-site and remote assessments were analyzed and summarized. During April 9-27, aggregate data on COVID-19 cases among 115 meat or poultry processing facilities in 19 states were reported to CDC. Among these facilities, COVID-19 was diagnosed in 4,913 (approximately 3%) workers, and 20 COVID-19-related deaths were reported. Facility barriers to effective prevention and control of COVID-19 included difficulty distancing workers at least 6 feet (2 meters) from one another (2) and in implementing COVID-19-specific disinfection guidelines.* Among workers, socioeconomic challenges might contribute to working while feeling ill, particularly if there are management practices such as bonuses that incentivize attendance. Methods to decrease transmission within the facility include worker symptom screening programs, policies to discourage working while experiencing symptoms compatible with COVID-19, and social distancing by workers. Source control measures (e.g., the use of cloth face covers) as well as increased disinfection of high-touch surfaces are also important means of preventing SARS-CoV-2 exposure. Mitigation efforts to reduce transmission in the community should also be considered. Many of these measures might also reduce asymptomatic and presymptomatic transmission (3). Implementation of these public health strategies will help protect workers from COVID-19 in this industry and assist in preserving the critical meat and poultry production infrastructure (4). |
Platelet count variation and risk for coronary artery abnormalities in Kawasaki disease
Ae R , Abrams JY , Maddox RA , Schonberger LB , Nakamura Y , Shindo A , Kuwabara M , Makino N , Matsubara Y , Kosami K , Sasahara T , Belay ED . Pediatr Infect Dis J 2019 39 (3) 197-203 BACKGROUND: Platelet count is considered as a biomarker for the development of coronary artery abnormalities (CAAs) among Kawasaki disease (KD) patients. However, previous studies have reported inconsistent results. We addressed the controversial association of platelet count with CAAs using a large-scale dataset. METHODS: A retrospective cohort study was conducted using KD survey data from Japan (2015-2016; n = 25,448). Classifying patients by intravenous immunoglobulin (IVIG) responsiveness, we described the trends in platelet count using the lowest and highest values along with the specific illness days. Multivariate logistic regression analysis was performed to evaluate the association between platelet count and CAAs, adjusting for relevant factors. RESULTS: Platelet counts rapidly decreased from admission, reached the lowest count at 6-7 days, and peaked after 10 days. Platelet counts in IVIG non-responders decreased with a lower minimum value than IVIG responders, but subsequently rebounded toward a higher maximum. Compared with patients with normal platelet counts (150-450 x 10/L), patients with abnormally high platelet counts (>450 x 10/L) were more likely to have CAAs at admission (adjusted odds ratio: IVIG responders, 1.50 [95% confidence interval 1.20-1.87] and non-responders, 1.46 [1.01-2.12]). By contrast, IVIG non-responding patients whose counts were below normal (<150 x 10/L) after hospitalization were at higher risk for developing CAAs (2.27 [1.44-3.58]). CONCLUSIONS: Platelet count varied widely by illness day and was confounded by IVIG responsiveness, which might have contributed to previous inconsistent findings. KD patients with abnormally high platelet counts at admission or abnormally low counts after hospitalization were at higher risk for CAAs. |
Prion disease incidence in the United States, 2003-2015
Maddox RA , Person MK , Blevins JE , Abrams JY , Appleby BS , Schonberger LB , Belay ED . Neurology 2019 94 (2) e153-e157 OBJECTIVE: To report the incidence of prion disease in the United States. METHODS: Prion disease decedents were retrospectively identified from the US national multiple cause-of-death data for 2003-2015 and matched with decedents in the National Prion Disease Pathology Surveillance Center (NPDPSC) database through comparison of demographic variables. NPDPSC decedents with neuropathologic or genetic test results positive for prion disease for whom no match was found in the multiple cause-of-death data were added as cases for incidence calculations; those with cause-of-death data indicating prion disease but with negative neuropathology results were removed. Age-specific and age-adjusted average annual incidence rates were then calculated. RESULTS: A total of 5,212 decedents were identified as having prion disease, for an age-adjusted average annual incidence of 1.2 cases per million population (range 1.0 per million [2004 and 2006] to 1.4 per million [2013]). The median age at death was 67 years. Ten decedents were <30 years of age (average annual incidence of 6.2 per billion); only 2 of these very young cases were sporadic forms of prion disease. Average annual incidence among those >/=65 years of age was 5.9 per million. CONCLUSIONS: Prion disease incidence can be estimated by augmenting mortality data with the results of neuropathologic and genetic testing. Cases <30 years of age were extremely rare, and most could be attributed to exogenous factors or the presence of a genetic mutation. Continued vigilance for prion diseases in all age groups remains prudent. |
Iatrogenic Creutzfeldt-Jakob disease with amyloid-beta pathology: an international study
Cali I , Cohen ML , Hasmall yi Ukrainiank S , Parchi P , Giaccone G , Collins SJ , Kofskey D , Wang H , McLean CA , Brandel JP , Privat N , Sazdovitch V , Duyckaerts C , Kitamoto T , Belay ED , Maddox RA , Tagliavini F , Pocchiari M , Leschek E , Appleby BS , Safar JG , Schonberger LB , Gambetti P . Acta Neuropathol Commun 2018 6 (1) 5 The presence of pathology related to the deposition of amyloid-beta (Abeta) has been recently reported in iatrogenic Creutzfeldt-Jakob disease (iCJD) acquired from inoculation of growth hormone (GH) extracted from human cadaveric pituitary gland or use of cadaveric dura mater (DM) grafts.To investigate this phenomenon further, a cohort of 27 iCJD cases - 21 with adequate number of histopathological sections - originating from Australia, France, Italy, and the Unites States, were examined by immunohistochemistry, amyloid staining, and Western blot analysis of the scrapie prion protein (PrP(Sc)), and compared with age-group matched cases of sporadic CJD (sCJD), Alzheimer disease (AD) or free of neurodegenerative diseases (non-ND).Cases of iCJD and sCJD shared similar profiles of proteinase K-resistant PrP(Sc) with the exception of iCJD harboring the "MMi" phenotype. Cerebral amyloid angiopathy (CAA), either associated with, or free of, Thioflavin S-positive amyloid core plaques (CP), was observed in 52% of 21 cases of iCJD, which comprised 37.5% and 61.5% of the cases of GH- and DM-iCJD, respectively. If only cases younger than 54 years were considered, Abeta pathology affected 41%, 2% and 0% of iCJD, sCJD and non-ND, respectively. Despite the patients' younger age CAA was more severe in iCJD than sCJD, while Abeta diffuse plaques, in absence of Abeta CP, populated one third of sCJD. Abeta pathology was by far most severe in AD. Tau pathology was scanty in iCJD and sCJD.In conclusion, (i) despite the divergences in the use of cadaveric GH and DM products, our cases combined with previous studies showed remarkably similar iCJD and Abeta phenotypes indicating that the occurrence of Abeta pathology in iCJD is a widespread phenomenon, (ii) CAA emerges as the hallmark of the Abeta phenotype in iCJD since it is observed in nearly 90% of all iCJD with Abeta pathology reported to date including ours, and it is shared by GH- and DM-iCJD, (iii) although the contributions to Abeta pathology of other factors, including GH deficiency, cannot be discounted, our findings increase the mounting evidence that this pathology is acquired by a mechanism resembling that of prion diseases. |
Increased Kawasaki disease incidence associated with higher precipitation and lower temperatures, Japan, 1991-2004
Abrams JY , Blase JL , Belay EB , Uehara R , Maddox RA , Schonberger LB , Nakamura Y . Pediatr Infect Dis J 2017 37 (6) 526-530 BACKGROUND: Kawasaki disease (KD) is an acute febrile vasculitis which primarily affects children. The etiology of KD is unknown: while certain characteristics of the disease suggest an infectious origin, genetic or environmental factors may also be important. Seasonal patterns of KD incidence are well-documented, but it is unclear whether these patterns are caused by changes in climate or by other unknown seasonal effects. METHODS: The relationship between KD incidence and deviations from expected temperature and precipitation were analyzed using KD incidence data from Japanese nationwide epidemiological surveys (1991-2004) and climate data from 136 weather stations of the Japan Meteorological Agency. Seven separate Poisson-distributed generalized linear regression models were run to examine the effects of temperature and precipitation on KD incidence in the same month as KD onset and the previous 1, 2, 3, 4, 5, and 6 months, controlling for geography as well as seasonal and long-term trends in KD incidence. RESULTS: KD incidence was negatively associated with temperature in the previous 2, 3, 4, and 5 months and positively associated with precipitation in the previous 1 and 2 months. The model that best predicted variations in KD incidence used climate data from the previous 2 months. An increase in total monthly precipitation by 100mm was associated with increased KD incidence (rate ratio=1.012, 95% confidence interval=1.005-1.019) and an increase of monthly mean temperature by 1 degrees C was associated with decreased KD incidence (RR=0.984, 95% CI=0.978-0.990). CONCLUSIONS: KD incidence was significantly affected by temperature and precipitation in previous months independent of other unknown seasonal factors. Climate data from the previous 2 months best predicted variations in KD incidence. Although fairly minor, the effect of temperature and precipitation independent of season may provide additional clues to the etiology of KD. |
Cardiac complications, earlier treatment, and initial disease severity in Kawasaki disease
Abrams JY , Belay ED , Uehara R , Maddox RA , Schonberger LB , Nakamura Y . J Pediatr 2017 188 64-69 OBJECTIVES: To assess if observed higher observed risks of cardiac complications for patients with Kawasaki disease (KD) treated earlier may reflect bias due to confounding from initial disease severity, as opposed to any negative effect of earlier treatment. STUDY DESIGN: We used data from Japanese nationwide KD surveys from 1997 to 2004. Receipt of additional intravenous immunoglobulin (IVIG) (data available all years) or any additional treatment (available for 2003-2004) were assessed as proxies for initial disease severity. We determined associations between earlier or later IVIG treatment (defined as receipt of IVIG on days 1-4 vs days 5-10 of illness) and cardiac complications by stratifying by receipt of additional treatment or by using logistic modeling to control for the effect of receiving additional treatment. RESULTS: A total of 48 310 patients with KD were included in the analysis. In unadjusted analysis, earlier IVIG treatment was associated with a higher risk for 4 categories of cardiac complications, including all major cardiac complications (risk ratio, 1.10; 95% CI, 1.06-1.15). Stratifying by receipt of additional treatment removed this association, and earlier IVIG treatment became protective against all major cardiac complications when controlling for any additional treatment in logistic regressions (OR, 0.90; 95% CI, 0.80-1.00). CONCLUSIONS: Observed higher risks of cardiac complications among patients with KD receiving IVIG treatment on days 1-4 of the illness are most likely due to underlying higher initial disease severity, and patients with KD should continue to be treated with IVIG as early as possible. |
Intracranial procedures and expected frequency of Creutzfeldt-Jakob disease
Abrams JY , Maddox RA , Schonberger LB , Belay ED . Neuroepidemiology 2015 46 (1) 1-8 BACKGROUND/AIMS: To assess the frequency and characteristics of intracranial procedures (ICPs) performed and the number of US residents living with a history of ICP. These data are used to calculate the expected annual number of sporadic Creutzfeldt-Jakob disease (CJD) cases among US residents with a history of ICP. METHODS: The Nationwide Inpatient Sample provided data on the frequency and types of ICPs, and data from the National Center for Health Statistics was used to produce age-adjusted mortality rates. A model was constructed, which estimated long-term survival and sporadic CJD rates among ICP patients based on procedure type and age. RESULTS: There were an estimated 2,070,488 ICPs in the United States from 1998 to 2007, an average of over 200,000 per year. There were an estimated 2,023,726 US residents in 2013 with a history of ICP in the previous 30 years. In 2013, there was expected to be 4.1 sporadic CJD cases (95% CI 1-8) among people with a history of ICP in the past 30 years. CONCLUSIONS: The considerable proportion of US residents living with a history of ICP is important information for retrospective assessments of CJD or any other suspected long-term outcome of ICPs. |
Clinically unsuspected prion disease among patients with dementia diagnoses in an Alzheimer's disease database
Maddox RA , Blase JL , Mercaldo ND , Harvey AR , Schonberger LB , Kukull WA , Belay ED . Am J Alzheimers Dis Other Demen 2015 30 (8) 752-5 BACKGROUND: Brain tissue analysis is necessary to confirm prion diseases. Clinically unsuspected cases may be identified through neuropathologic testing. METHODS: National Alzheimer's Coordinating Center (NACC) Minimum and Neuropathologic Data Set for 1984 to 2005 were reviewed. Eligible patients had dementia, underwent autopsy, had available neuropathologic data, belonged to a currently funded Alzheimer's Disease Center (ADC), and were coded as having an Alzheimer's disease clinical diagnosis or a nonprion disease etiology. For the eligible patients with neuropathology indicating prion disease, further clinical information, collected from the reporting ADC, determined whether prion disease was considered before autopsy. RESULTS: Of 6000 eligible patients in the NACC database, 7 (0.12%) were clinically unsuspected but autopsy-confirmed prion disease cases. CONCLUSION: The proportion of patients with dementia with clinically unrecognized but autopsy-confirmed prion disease was small. Besides confirming clinically suspected cases, neuropathology is useful to identify unsuspected clinically atypical cases of prion disease. |
Re: examining the association between Kawasaki disease and pertussis
Abrams JY , Schonberger LB , Maddox RA , Belay ED . Epidemiology 2015 26 (5) e56-7 The March 2014 issue of EPIDEMIOLOGY included an article by Pellegrino et al. describing a strong correlation between the annual number of hospitalizations for Kawasaki disease and pertussis in the United States.1 Kawasaki disease is a potentially serious vasculitis that occurs most commonly in young children, and its etiology is still unknown. Previous studies have reported that Kawasaki disease cases are more likely than controls to have had respiratory illness in the previous 30 days.2–4 Pellegrino et al. used data from the National Inpatient Sample, a roughly 20% sample of US hospitals, to conduct an exploratory analysis assessing any possible correlation between Kawasaki disease and a large number of respiratory diseases; pertussis was the only disease strongly correlated with Kawasaki disease (r = 0.77).1 | To assess whether the reported preliminary correlation between pertussis and Kawasaki disease reflected a chance finding or a signal of a causal relation, we examined whether trends in pertussis and Kawasaki disease are occurring (1) in the same geographic locations, (2) in the same age group, and (3) in either the same time frame or with Kawasaki disease trends immediately following pertussis trends. We conducted a Poisson regression with the outcome being monthly number of hospitalizations for Kawasaki disease from 2000 to 2011. The main predictor was the monthly number of pertussis cases; to assess temporal associations, we also separately ran regressions using the number of pertussis cases 1 month to 12 months prior. We controlled for year (linear trend) and geographic region (the four US census regions). Hospitalizations account for a smaller proportion of overall pertussis cases than for Kawasaki disease cases. Therefore, we analyzed hospitalized and nonhospitalized pertussis cases reported to the US National Notifiable Diseases Surveillance System. For both pertussis and Kawasaki disease, we limited the data to children less than 5 years old. |
Recurrent Kawasaki disease, United States and Japan
Maddox RA , Holman RC , Uehara R , Callinan LS , Guest JL , Schonberger LB , Nakamura Y , Yashiro M , Belay ED . Pediatr Int 2015 57 (6) 1116-20 BACKGROUND: Descriptive epidemiologic studies of recurrent and non-recurrent Kawasaki disease (KD) may identify other potentially important differences between these illnesses. METHODS: Data from the United States and Japan, the Centers for Disease Control and Prevention (CDC) national KD surveillance(1984-2008) and the 17th Japanese nationwide survey (2001-2002), respectively, were analyzed to examine recurrent KD patients <18 years of age meeting the CDC KD case or atypical KD case definition. These patients were compared to non-recurrent KD patients. RESULTS: Of the 5557 US KD patients <18 years of age during 1984-2008, 97 (1.7%) were identified as having had recurrent KD. Among the US Asian/Pacific IslanderKDpatients, 3.5% had recurrent KD, which was similar to the percentage identified among KD patients (3.5%) in the Japanese survey. Compared to non-recurrent KDpatients, KD patients experiencing a recurrent KDepisode were more likely to be older, fulfill the atypical KD case definition, and have coronary artery abnormalities (CAA) despite IVIG treatment. CONCLUSIONS: Differences in the age, race, and frequency of CAA exist between recurrent and non-recurrent KD patients. The increased association of CAA with recurrent KD suggests that more aggressive treatment strategies in conjunction with IVIG may be indicated for the second episode of KD. |
Recent US case of variant Creutzfeldt-Jakob disease - global implications
Maheshwari A , Fischer M , Gambetti P , Parker A , Ram A , Soto C , Concha-Marambio L , Cohen Y , Belay ED , Maddox RA , Mead S , Goodman C , Kass JS , Schonberger LB , Hussein HM . Emerg Infect Dis 2015 21 (5) 750-9 Variant Creutzfeldt-Jakob disease (vCJD) is a rare, fatal prion disease resulting from transmission to humans of the infectious agent of bovine spongiform encephalopathy. We describe the clinical presentation of a recent case of vCJD in the United States and provide an update on diagnostic testing. The location of this patient's exposure is less clear than those in the 3 previously reported US cases, but strong evidence indicates that exposure to contaminated beef occurred outside the United States more than a decade before illness onset. This case exemplifies the persistent risk for vCJD acquired in unsuspected geographic locations and highlights the need for continued global surveillance and awareness to prevent further dissemination of vCJD. |
Sporadic fatal insomnia in an adolescent
Blase JL , Cracco L , Schonberger LB , Maddox RA , Cohen Y , Cali I , Belay ED . Pediatrics 2014 133 (3) e766-70 The occurrence of sporadic prion disease among adolescents is extremely rare. A prion disease was confirmed in an adolescent with disease onset at 13 years of age. Genetic, neuropathologic, and biochemical analyses of the patient's autopsy brain tissue were consistent with sporadic fatal insomnia, a type of sporadic prion disease. There was no evidence of an environmental source of infection, and this patient represents the youngest documented case of sporadic prion disease. Although rare, a prion disease diagnosis should not be discounted in adolescents exhibiting neurologic signs. Brain tissue testing is necessary for disease confirmation and is particularly beneficial in cases with an unusual clinical presentation. |
Kawasaki syndrome and factors associated with coronary artery abnormalities, California
Callinan LS , Tabnak F , Holman RC , Maddox RA , Kim JJ , Schonberger LB , Vugia DJ , Belay ED . Pediatr Infect Dis J 2012 31 (9) 894-8 BACKGROUND: Kawasaki syndrome (KS) occurs in children less than 18 years of age and is the leading cause of acquired heart disease among children in the United States. Understanding the epidemiology of KS and factors associated with coronary artery abnormalities (CAA) may lead to timely diagnosis and treatment of KS and could limit CAA. METHODS: Epidemiologic characteristics, including risk factors for the development of CAA, among KS and incomplete KS patients less than 18 years of age with onset during 2000-2009 reported by the California Department of Public Health to the Centers for Disease Control and Prevention's national KS surveillance system were analyzed. RESULTS: A total of 2056 KS and incomplete KS patients less than 18 years of age were reported for 2000-2009. The median age of patients was 2 years; 60% of patients were male. Of 1818 patients with race information reported, 56% were white and 28% were Asian/Pacific Islander. Ninety-eight percent of patients received intravenous immunoglobulin (IVIG). Of 1843 patients with information on cardiac complications, 89 (5%) had coronary artery aneurysms and 341 (19%) had CAA. Characteristics associated with the occurrence of CAA in KS patients were male sex, Asian/Pacific Islander race, age less than 1 year or 9-17 years, and receiving IVIG treatment on or after the fifth day of illness. CONCLUSIONS: This study suggests that IVIG treatment before the fifth day of illness may reduce CAA among KS patients. Timely diagnosis and treatment of KS continue to be important in reducing the occurrence of cardiac complications. |
Lower risk of Creutzfeldt-Jakob disease in pituitary growth hormone recipients initiating treatment after 1977
Abrams JY , Schonberger LB , Belay ED , Maddox RA , Leschek EW , Mills JL , Wysowski DK , Fradkin JE . J Clin Endocrinol Metab 2011 96 (10) E1666-9 CONTEXT: Creutzfeldt-Jakob disease (CJD) caused by contaminated cadaveric pituitary-derived human GH (hGH) has been responsible for hundreds of deaths worldwide. Studies of U.S. National Hormone and Pituitary Program (NHPP) hGH recipients have found CJD only in patients treated before 1977, when a new purification procedure with column chromatography was implemented for hGH extraction. OBJECTIVE: Our objective was to provide updated information on transmission of CJD to NHPP hGH recipients and determine whether recipients of hGH produced after 1977 had a significantly lower CJD risk than pre-1977 recipients. PATIENTS: A total of 5570 NHPP hGH recipients were included in the study: 2099 in the pre-1977 cohort and 3471 in the post-1977 cohort. MAIN OUTCOME MEASURE: We used probability distribution functions to determine whether the observed number of CJD cases in the post-1977 cohort was significantly fewer than expected if the CJD risk was equal to that of the pre-1977 cohort, controlling for treatment duration and follow-up time. RESULTS: All 22 CJD cases (diagnosed from 1984-2009) occurred in the pre-1977 hGH recipients. Almost half (47.9%) of pre-1977 recipients had a treatment duration of at least 5 yr compared with only 13.8% for post-1977 recipients. Based on the rates present in the pre-1977 cohort, the probability of observing no cases in the post-1977 cohort by chance alone was low (P = 0.0019). CONCLUSIONS: Risk of acquiring CJD was significantly lower for post-1977 NHPP hGH recipients than for pre-1977 recipients, suggesting that the new purification procedure in 1977 may have greatly reduced or eliminated CJD agent in hGH. |
Travel history, hunting, and venison consumption related to prion disease exposure, 2006-2007 FoodNet population survey
Abrams JY , Maddox RA , Harvey AR , Schonberger LB , Belay ED . J Am Diet Assoc 2011 111 (6) 858-63 The transmission of bovine spongiform encephalopathy (BSE) to human beings and the spread of chronic wasting disease (CWD) among cervids have prompted concerns about zoonotic transmission of prion diseases. Travel to the United Kingdom and other European countries, hunting for deer or elk, and venison consumption could result in the exposure of US residents to the agents that cause BSE and CWD. The Foodborne Diseases Active Surveillance Network 2006-2007 population survey was used to assess the prevalence of these behaviors among residents of 10 catchment areas across the United States. Of 17,372 survey respondents, 19.4% reported travel to the United Kingdom since 1980, and 29.5% reported travel to any of the nine European countries considered to be BSE-endemic since 1980. The proportion of respondents who had ever hunted deer or elk was 18.5%, and 1.2% had hunted deer or elk in a CWD-endemic area. More than two thirds (67.4%) reported having ever eaten deer or elk meat. Respondents who traveled spent more time in the United Kingdom (median 14 days) than in any other BSE-endemic country. Of the 11,635 respondents who had consumed venison, 59.8% ate venison at most one to two times during their year of highest consumption, and 88.6% had obtained all of their meat from the wild. The survey results were useful in determining the prevalence and frequency of behaviors that could be important factors for foodborne prion transmission. |
Human prion diseases in the United States
Holman RC , Belay ED , Christensen KY , Maddox RA , Minino AM , Folkema AM , Haberling DL , Hammett TA , Kochanek KD , Sejvar JJ , Schonberger LB . PLoS One 2010 5 (1) e8521 BACKGROUND: Prion diseases are a family of rare, progressive, neurodegenerative disorders that affect humans and animals. The most common form of human prion disease, Creutzfeldt-Jakob disease (CJD), occurs worldwide. Variant CJD (vCJD), a recently emerged human prion disease, is a zoonotic foodborne disorder that occurs almost exclusively in countries with outbreaks of bovine spongiform encephalopathy. This study describes the occurrence and epidemiology of CJD and vCJD in the United States. METHODOLOGY/PRINCIPAL FINDINGS: Analysis of CJD and vCJD deaths using death certificates of US residents for 1979-2006, and those identified through other surveillance mechanisms during 1996-2008. Since CJD is invariably fatal and illness duration is usually less than one year, the CJD incidence is estimated as the death rate. During 1979 through 2006, an estimated 6,917 deaths with CJD as a cause of death were reported in the United States, an annual average of approximately 247 deaths (range 172-304 deaths). The average annual age-adjusted incidence for CJD was 0.97 per 1,000,000 persons. Most (61.8%) of the CJD deaths occurred among persons >or=65 years of age for an average annual incidence of 4.8 per 1,000,000 persons in this population. Most deaths were among whites (94.6%); the age-adjusted incidence for whites was 2.7 times higher than that for blacks (1.04 and 0.40, respectively). Three patients who died since 2004 were reported with vCJD; epidemiologic evidence indicated that their infection was acquired outside of the United States. CONCLUSION/SIGNIFICANCE: Surveillance continues to show an annual CJD incidence rate of about 1 case per 1,000,000 persons and marked differences in CJD rates by age and race in the United States. Ongoing surveillance remains important for monitoring the stability of the CJD incidence rates, and detecting occurrences of vCJD and possibly other novel prion diseases in the United States. |
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