Tyler W. Buckner

Self-reported prevalence, description and management of pain in adults with haemophilia: Methods, demographics and results from the Pain, Functional Impairment, and Quality of life (P-FiQ) study

Haemophilia is characterized by frequent haemarthrosis, leading to acute/chronic joint pain.

Patterns of symptoms and functional impairments in children with cancer

Children with cancer experience multiple symptoms due to their disease and as a result of treatment. The purpose of this study was to demonstrate the feasibility and potential utility of using latent profile analysis (LPA), a type of cluster analysis, in children with cancer to identify groups of patients who experience similar levels of symptom severity and impairment of physical function.

Venous Thrombosis in Blacks

Case Presentation: A 42-year-old black woman is diagnosed with idiopathic (unprovoked) lower-extremity deep vein thrombosis (DVT) after presenting with leg swelling. She is treated with anticoagulation for 6 months without major complications and with stable international normalized ratios, and her lower-extremity symptoms completely resolve. She has sickle cell trait but no other chronic conditions. The patients mother also had an idiopathic DVT at 53 years of age. Physical examination is notable only for obesity (body mass index 33 kg/m2) and absence of lower-extremity swelling, redness, and pain. D-dimer and follow-up lower-extremity Doppler ultrasound testing are negative. Testing for antiphospholipid antibodies was negative at the time the clot was diagnosed; further tests for thrombophilia have not been performed. She wants to know whether she should continue taking warfarin. In 2008, the US Surgeon General issued The Surgeon Generals Call to Action to Prevent Deep Vein Thrombosis and Pulmonary Embolism , which highlighted the scope and impact of venous thromboembolism (VTE). In the United States alone, as many as 100 000 to 180 000 deaths occur annually due to DVT and pulmonary embolism (PE).1 Many of these events are preventable by implementation of standard prophylactic measures in certain high-risk populations (eg, hospitalized patients with reduced mobility); however, a large proportion of VTE events occur spontaneously, without clear inciting factors. Identification of novel risk factors for VTE might allow clinicians to better identify at-risk patients and modify both primary and secondary prevention strategies accordingly. The incidence of VTE depends on a complex interaction of inherited and environmental risk factors. The prevalence of inherited risk factors depends on the population being studied. To date, the genetics of VTE has been studied most frequently in patients with European …

The Autopsy in Pediatrics and Pediatric Oncology: A Single-Institution Experience

Autopsy rates and clinicopathologic correlations for pediatric autopsies and the subgroup of pediatric oncology autopsies in a large teaching hospital were studied to evaluate the utility of autopsy in these populations. Autopsy records of the University of North Carolina hospitals from 1982 to 2001 were reviewed for all patients less than 18 years of age. Autopsies performed during 1982 to 1991 (decade 1) were compared to those from 1992 to 2001 (decade 2) with respect to absolute numbers of autopsy and rates of unexpected postmortem diagnoses. Postmortem diagnostic discrepancies were subclassified into major and minor categories. The mean number of autopsies per year for decade 1 was 110 ± 24.5, compared with 77.5 ± 40.9 for decade 2 (P < 0.001), a change largely due to a decline in fetal/perinatal (patients < 7 days of age) autopsies. Of 533 pediatric autopsies, 43 were in patients with a primary diagnosis of a neoplasia. At least one antemortem misdiagnosis and/or clinically occult process was identified in 20.5% of all pediatric autopsy cases, and in 25.6% of pediatric oncology cases. These rates did not change significantly over time. In 10 of 43 pediatric oncology cases (23.3%), autopsies provided diagnostic information that was previously unknown to the clinicians. Three of these patients died shortly after presentation. Although autopsy rates for fetal/perinatal cases have declined, those for nonperinatal and pediatric oncology patients remain stable. Autopsy continues to provide diagnostic information that is unknown during the antemortem period, and remains a valuable tool for pediatricians and pediatric oncologists.

Known-group validity of patient-reported outcome instruments and hemophilia joint health score v2.1 in US adults with hemophilia: results from the Pain, Functional Impairment, and Quality of life (P-FiQ) study

Background The Pain, Functional Impairment, and Quality of Life (P-FiQ) study was an observational, cross-sectional assessment of the impact of pain on functional impairment and quality of life in adults with hemophilia in the United States who experience joint pain or bleeding. Objective To describe known-groups validity of assessment tools used in the P-FiQ study. Patients and methods Participants completed 5 patient-reported outcome (PRO) instruments (5-level EuroQoL 5-dimensional questionnaire [EQ-5D-5L] with visual analog scale [VAS], Brief Pain Inventory v2 Short Form [BPI], International Physical Activity Questionnaire [IPAQ], Short-Form Health Survey [SF-36v2], and Hemophilia Activities List [HAL]) and underwent a musculoskeletal examination (Hemophilia Joint Health Score [HJHS]) during a routine clinical visit. Results P-FiQ enrolled 381 adults with hemophilia (median age, 34 years). Participants were predominantly white/non-Hispanic (69.2%), 75% had congenital hemophilia A, and 70.5% had severe hemophilia. Most (n=310) reported bleeding within the past 6 months (mean [SD] number of bleeds, 7.1 [13.00]). All instruments discriminated between relevant known (site- or self-reported) participant groups. Domains related to pain on EQ-5D-5L, BPI, and SF-36v2 discriminated self-reported pain (acute/chronic/both; P<0.05), domains related to functional impairment on IPAQ, SF-36v2, and HAL discriminated self-reported functional impairment (restricted/unrestricted; P<0.05), and domains related to mental health on the EQ-5D-5L and SF-36v2 discriminated self-reported anxiety/depression (yes/no; P<0.01). HJHS ankle and global gait domains and global score discriminated self-reported arthritis/bone/joint problems, percentage of lifetime on prophylaxis, current treatment regimen, and hemophilia severity (P<0.01); knee and elbow domains discriminated all of these (P<0.01) except for current treatment regimen. Conclusion All assessment tools demonstrated known-group validity and may have practical applicability in evaluating adults with hemophilia in clinical and research settings in the United States.

Reliability of patient-reported outcome instruments in US adults with hemophilia: The Pain, Functional Impairment and Quality of life (P-FiQ) study

Background Hemophilia is marked by frequent joint bleeding, resulting in pain and functional impairment. Objective This study aimed to assess the reliability of five patient-reported outcome (PRO) instruments in people with hemophilia (PWH) in a non-bleeding state. Methods Adult male PWH of any severity and inhibitor status, with a history of joint pain or bleeding, completed a pain history and five PRO instruments (EQ-5D-5L, Brief Pain Inventory v2 [BPI], International Physical Activity Questionnaire [IPAQ], Short Form 36 Health Survey v2 [SF-36v2], and Hemophilia Activities List [HAL]) during their routine comprehensive care visit. Patients were approached to complete the PRO instruments again at the end of their visit while in a similar non-bleeding state. Concordance of individual questionnaire items and correlation between domain scores were assessed using intra-class correlation coefficient (ICC). Results Participants completing the retest (n=164) had a median age of 33.9 years. Median time for completion of the initial survey with PRO instruments was 36.0 minutes and for the five PRO instruments, median retest time was 21.0 minutes. The majority of participants had hemophilia A (74.4%), were white and non-Hispanic (72.6%), and self-reported arthritis/bone/joint problems (61%). Median/mean test-retest concordance was EQ-5D-5L 80.0%/79.1%, BPI 54.5%/58.9%, IPAQ 100%/100%, SF-36v2 77.8%/76.4%, and HAL 77.4%/75.9%. ICCs for test-retest reliability were EQ-5D-5L index 0.890; BPI – severity 0.950; BPI – interference 0.920; IPAQ total activity 0.940; SF-36v2 overall health 0.910; HAL total score 0.970. Conclusion All five PRO scales showed acceptable test-retest reliability in adult PWH. Therefore, the choice of instrument to be used for research or clinical care should be driven by instrument characteristics other than reliability.

Impact of pain and functional impairment in US adults with haemophilia: Patient-reported outcomes and musculoskeletal evaluation in the pain, functional impairment and quality of life (P-FiQ) study

Standardized and disease‐specific patient‐reported outcome (PRO) instruments assessing pain, functional impairment and health‐related quality of life (HRQoL) in people with haemophilia (PWH) have been used in studies, but infrequently in comprehensive care settings for individual assessment or treatment planning.

Management of US men, women, and children with hemophilia and methods and demographics of the Bridging Hemophilia B Experiences, Results and Opportunities into Solutions (B-HERO-S) study

The Bridging Hemophilia B Experiences, Results and Opportunities Into Solutions (B‐HERO‐S) initiative was launched in an effort to address specific gaps in the understanding of the psychosocial impact of mild‐moderate‐severe hemophilia B. The original Hemophilia Experiences, Results and Opportunities (HERO) qualitative study evaluated the needs of people with hemophilia A or B in multiple countries; however, a majority of participants had the more common moderate‐severe hemophilia A. The B‐HERO‐S study was designed in collaboration with the hemophilia community to evaluate the needs of adults with hemophilia B and caregivers of children with hemophilia B, including affected women and caregivers of girls with hemophilia. The report presented here describes participant demographics and comorbidities, as well as treatment regimens and access to treatment. Bleeding symptoms were reported by 27% of mothers of children with hemophilia B who participated. Women were more likely than men to self‐report arthritis and depression/anxiety as comorbidities associated with hemophilia B. More adults and children with hemophilia B were on routine treatment than on on‐demand treatment, and a high percentage of adults with moderate hemophilia B received routine treatment (86%). Many adults with hemophilia B (78%) and caregivers (69%) expressed concern about access to factor in the next 5 years, and of adults with hemophilia B, women more commonly experienced issues with access to factor in the past than did men (72% vs 44%). The findings of the B‐HERO‐S study reveal potential unmet needs of some patients with mild‐moderate hemophilia B, and the results may be leveraged to inform patient outreach by hemophilia treatment centers and education initiatives.

Factors associated with pain severity, pain interference, and perception of functional abilities independent of joint status in US adults with hemophilia: Multivariable analysis of the Pain, Functional Impairment, and Quality of Life (P-FiQ) study

Levels of pain and dysfunction appear to differ among people with hemophilia despite similar levels of joint disease.

Complete Spontaneous Remission of Diffuse Large B-Cell Lymphoma of the Maxillary Sinus After Concurrent Infections

Case Description A 67-year-old woman presented to her dentist for evaluation of painless, right-sided facial swelling that had been slowly increasing for approximately 4 weeks. The swelling did not improve after a course of oral antibiotics. One month after presentation, she was referred to an oral surgeon who extracted 2 teeth. During this procedure, a soft tissue mass that involved the right maxilla was noted, and biopsies of the mass were performed. Histologic examination of the mass revealed sheets of monomorphic, large cells with vesicular chromatin and prominent nucleoli, with admixed scattered small lymphocytes (Figure 1). Immunohistochemistry stains showed positive CD 20, CD10, Bcl-2, Bcl-6, and MUM-1 with negative CD5, consistent with diffuse large B-cell