Psy34 - Hemophilia B - Treatment Patterns and Epidemiology Insights From the German Hospital Quality Reports

Abstract

were used to estimate the age-adjusted national prevalence of diagnosed HCU and PKU patients. RESULTS: Among 97.3 million patients in the MSN, 6,613 (0.068 per 1,000) had a diagnosis of HCU between 1/1/2010 and 12/31/2016, compared to 5,120 (0.053 per 1,000) with a PKU diagnosis. Of the HCU cases, 0.51% were ages 0e11 years and 80% were 45 years or older at the time of the first recorded diagnosis during the study period. For PKU cases, 53% were ages 0e11 years and 13% were 45 years or older. The estimated age-adjusted prevalence of diagnosed HCU in the US for 2017 was~0.01% (31,162 cases) vs. 0.005% (16,615 cases) for PKU. CONCLUSIONS: As expected, the highest proportion of diagnosed PKU was observed in the youngest age group (ages 0e11 years), likely due to infants being diagnosed through mandatory newborn screening. Conversely, the proportion of diagnosed HCU cases in the younger age group was approximately ten times smaller, implying that HCU patients are not diagnosed primarily at birth or during early childhood. This suggests that current newborn screening tests fail to capture the vast majority of HCU cases, with patients diagnosed at later ages, even into adulthood, when they present with symptoms or comorbid conditions indicative of HCU.OBJECTIVES: Approximately 1 in 200,000e335,000 people worldwide have homocystinuria (HCU) but it may be underdiagnosed. We examined incidence and prevalence of homocysteine (tHcy) testing and the frequency of tHcy levels >30 mmoles/L. METHODS: In the MarketScan© Lab Database (MSN), we identified commercially insured patients with claims for a tHcy test (1/1/2006e3/31/2016). Frequency of elevated tHcy test result >30 mmoles/L and medications prescribed were examined. Frequencies of select comorbidities were assessed, using International Classification of Diseases (ICD-9 and ICD10) codes. Incidence of first-time tHcy testing and incidence and prevalence of tHcy >30 mmoles/L were tabulated. RESULTS: Of 8.2 million MSN patients in the Lab database, 1.9 million were enrolled throughout 4/1/15e3/31/16. Of those, 0.8% (15,012) had a tHcy test since 2006 (mean±SD, 10.8 ±15.1 mmoles/L). Of the 15,012 patients, 223 (1.5%) had tHcy >30 (96.0±174.1, median 39.9). Of the 223, 45% were diagnosed with hypertension, 44% hyperlipidemia, 17% hypothyroidism, 15% vitamin D deficiency and 12% renal disease, with only 10% (22) diagnosed with HCU or a sulfur amino acid metabolism disorder. After a tHcy >30 result, 11% were prescribed antihypertensives, 10% lipidlowering drugs, 9% anxiolytics or antidepressants, 7% thyroid hormone and 2% vitamin D. Annual incidence of a first tHcy test result was 0.8e2.3 (mean 1.5) tests/ 1000 person years in 2009e2015. The incidence of a first tHcy result >30 was 0.01e0.06 (mean 0.03)/1000. Estimated point prevalence of tHcy >30 in the U.S. on 4/ 1/2016 was~0.010%e 0.014% (33,562e43,706). CONCLUSIONS: This study is one of the first to estimate the prevalence of tHcy levels in the range of classical HCU in the U.S. Of patients having many symptoms associated wit...

A Systematic Review of International Affective Picture System (IAPS) around the World

“…Neuropsychological instruments used for participant evaluation in the reviewed articles. Abbreviations: 20-item Center for Epidemiologic Studies-Depression Scale (CES-D) [119], Alcohol Use Disorders Identification Test Consumption (AUDIT-C) [120], Animal Naming Task (ANT) [121], Antisocial Process Screening Device (APSD) [122], Beck Depression Inventory (BDI) [123], Clock Drawing Test (CDT) [124], Diagnostic and Statistical Manual of Mental Disorders (DSM) [125], Drug Abuse Screening Test (DAST-10) [126], Experienced Attention Deficits Self-Rating Inventory (FEDA) [127], Five Factor Personality Inventory (FFPI) [128], Future Time Limit (FTP) [129], Geriatric Depression Scale (GDS) [130], Hachinski Ischemic Score (HIS) [131], Impulsiveness, Venturesomeness and Empathy (IVE) [132], Instrumental Activities of Daily Living (IADL) [133], Korean Wahler Physical Symptoms Inventory (K-WPSI) [134], Korean Wechsler Adult Intelligence Scale (K-WAIS) [135], Mini-Mental State Examination (MMSE) [136], Patient Health Questionnaire for Depression-9 (PHQ-9) [137], Primary Care Post-Traumatic Stress Disorder Screen (PC-PTSD) [138], Positive and Negative Affect Schedule (PANAS) [139], expanded version of the Positive and Negative Affect Schedule (PANAS-X) [140], Self Depression Scale (SDS) [141], Sensitivity to Punishment and Sensitivity to Reward Questionnaire (SPSRQ) [142], Short Portable Mental Status Questionnaire (SHPMSQ) [143], State Trait Anxiety Inventory (STAI) [144], Strengths and Difficulties Questionnaire (SDQ) [145], Waterloo Handedness Questionnaire (WHD) [146], Wechsler Adult Intelligence Scale (WAIS) [147], Wortschatztest [Vocabulary test] (WST) [148], and Zuckerman-Kuhlman Personality Questionnaire (ZKPQ) [149].…”