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Introduction and Objectives Intra-articular corticosteroid injections are standard of care for managing joint pain secondary to osteoarthritis or rheumatoid arthritis but are rarely used in haemophilic arthropathy. We have introduced and evaluated the efficacy and safety of ultrasound-guided corticosteroid injections for pain relief in patients with haemophilic arthropathy. Patients and methods Ultrasound-guided intra-articular injections performed on haemophilia patients at UCSD between March 2012 and January 2016 were analysed. Needle placement and injection (40 mg triamcinolone; 3–5 mL lidocaine) were performed with musculoskeletal ultrasound and Power Doppler. Analysis included patient demographics, joint-specific parameters such as tissue hypervascularity and effusions, pain relief, and procedure-associated complications. Results Forty-five injections (14 ankles, 13 elbows, 18 knees) were administered in 25 patients. Advanced arthropathy with hypervascularity and/or effusions was present in 91% and 61% of joints, respectively. Ninety-one per cent of injections resulted in pain relief which was significant in 84% (>30% reduction). Median pain score was reduced from 7 of 10 to 1 of 10 (P < 0.001), usually within 24 h. Median duration of pain relief was 8 weeks (range 1–16 weeks). Haemophilia B patients experienced longer periods of relief, and high Pettersson scores were associated with shorter duration of relief. There were no procedure-associated complications. Repeat ultrasound of eight joints within 4 weeks of injection demonstrated nearly complete resolution of hypervascularity. Conclusions Point-of-care ultrasound enabled intra-articular corticosteroid injections that provided highly effective, safe, and relatively long-lasting pain relief in haemophilic arthropathy. This approach should be used to improve pain management in haemophilic arthropathy.

Objective It is unclear if vascular remodeling in hemophilic joints perpetuates bleeding because it is difficult to gauge leakiness in the setting of low clotting factor levels. Two patients provided the unique opportunity to demonstrate that vascular changes directly contribute to joint bleeding. Approach and Results Two patients had severe hemophilic arthropathies; joint vascularity and bleeding were studied during periods of normalized plasma clotting factor activities with MSKUS and PD. One patient was “cured” of hemophilia with liver transplantation. Abnormal elbow vascularization persisted despite normalization of coagulation status and was associated with severe bleeding years after transplant. Dynamic vascular changes were detected prior to and during bleeding; angiography revealed enlarged, thickened vessels consistent with remodeling, and required arterial embolization. The second patient had continued knee bleeding and fluctuating vascular changes for months following knee replacement, unresponsive to intense daily factor treatment. Subsequently, new bleeds developed in other joints associated with similar vascular changes despite continued factor replacement. Conclusions Vascular remodeling contributes to perpetuated hemophilic joint bleeding and therefore contributes to progressive arthropathy. Intra-articular or systemic administration of anti-angiogenic drugs, such as vascular endothelial growth factor antagonists, might be of benefit in such patients, but requires study.

Papaverine has been associated with transient cranial nerve dysfunction after topical application during craniotomy. The authors report similar dysfunction after the use of papaverine affected brainstem structures. Two patients undergoing craniotomy for clipping of an aneurysm experienced bilateral depression of cortical somatosensory evoked potentials to both median and tibial nerve stimulation after administration of papaverine. Arterial blood gas analysis, hemodynamic parameters, and anesthetic levels remained constant throughout these somatosensory evoked potential changes. In addition, intraoperative angiography and immediate postoperative CT imaging showed intact blood flow with complete exclusion of the aneurysm. Both patients recovered within 1–2 hours and had normal neurological examination findings after extubation. Topical papaverine use may be associated with direct effects on brainstem structures. The transient nature of those changes suggests that aggressive intervention may not be needed. Maneuvers to limit the spread of papaverine to basal cisterns should be considered.

Objective. In 1996, the Residency Review Committee–Pediatrics recommended doubling time in continuity clinic to 2 half days per week. It has yet to be demonstrated that increased time in clinic yields greater continuity of care. The objective of this study was to determine whether increasing the number of half days spent in clinic improves continuity of care for residents.Methods. We reviewed computerized encounter records for all Medical University of South Carolina pediatric residents from 1982 to 1998. Depending on the year and the resident’s training level, house staff spent varying amounts of time in continuity clinic. Time in clinic was estimated from grants and materials generated in the residency program. We calculated continuity of care from the resident’s perspective for each individual resident per year using the Continuity for Physician (PHY) formula.Results. Continuity for 488 resident-years (200 residents) was evaluated. Residents spent from 10% to 30% of their time per year in clinic. Mean PHY was 57% (interns), 62% (second-year residents), and 52% (third-year residents). The correlation coefficient (R) between PHY and percentage of time in clinic was .22. In multivariable modeling, percentage of time in clinic, training level, and year predicted continuity. An increase of 1 half day in clinic was associated with an 11% increase in physician continuity. When analyses were limited to sick visits, R improved to .58. The effect size remained 11%. However, training level and academic year were no longer significant.Conclusion. Increasing time spent in clinic improves continuity and may indeed enhance the quality of this fundamental experience.

The objective of this study was to determine rates of blood pressure (BP) screening at well-child visits as recommended by the Task Force on Blood Pressure Control in Children. The 1985 and 1996 National Ambulatory Medical Care Surveys were analyzed for changes in proportion of well visits for children aged 3-18 years at which BP was checked. Patient and physician demo-graphics are described. BP screening increased from 50% in 1985 to 61 % in 1996. For pediatricians, the estimates were 50% (95% CI, 43-57) and 60% (95% CI, 53-68). For family/general medicine the estimates were 51% (95% CI, 34-69) and 58% (95% CI, 43-74). Age, geographic location, and length of a visit were significant in predicting BP screening. Gender, race, ethnicity, or urban location were not. A stepwise logistic regression confirmed these results. Rates of screening BP at well-child visits have increased but fall short of current recommendations. High-risk children are not screened at a rate different from their lower risk peers. Clin Pediatr. 2003;42:627-634

Background. There is widespread agreement among pediatric educators that continuity (following a panel of patients on a first contact basis for all their health care) is an important part of the education of pediatricians.Objective. To measure continuity in a pediatric residency practice and to compare this continuity with 2 nearby private general pediatric group practices. We also examined measures of continuity suggested in the literature.Design. Visit data were obtained from the practice billing system for a resident continuity practice and 2 nearby private practices for the 3-year period from July 1, 1992, to June 30, 1995. Visit data used were restricted to patients seen in the office of the practices during regular office hours. Continuity was measured using 5 different indices: 1) the usual provider of care index, visits by the usual clinician/total visits, 2) continuity for patient, the average proportion of visits that an individual patient was seen by his or her own physician, 3) continuity for physician (PHY), the average proportion of visits that an individual physician saw his or her own patients, 4) Continuity of Care Index (COC), and 5) the Modified, Modified Continuity Index. During the period examined, pediatric residents were present in the continuity practice for 1 half-day each week. The resident continuity practice (RCP) had 57 residents and saw 3386 patients for 18 955 visits. Private practice 1 (PP1) had 4 pediatricians who saw 4968 patients for 33 537 visits. Private practice 2 (PP2) had 5 pediatricians who saw 11 953 patients for 75 778 visits.Results. For all visit types, continuity in the RCP was not as high as in the private practices, PHY-RCP versus PP1, PP2; 53% versus 70%, 77%. However, continuity in RCP was greater than 50% for all measures except the COC index, which precipitously decreases as the number of clinicians seen increases. Examining continuity for health maintenance visits (PHY-RCP, PP2 vs PP1; 96%, 96% vs 82%) RCP was equal to the best of the private practices. The percentage of patients not seen for a health maintenance visit during the study period was lowest in the resident practice (RCP/PP1/PP2, 15/22/30).Conclusion. Although continuity for all visits in this RCP was less than in private practice, it was surprisingly high, considering the limited time residents spend in clinic. In a particularly important area for continuity, health maintenance visits, continuity was identical to one and superior to the other private practice.

Background: Hemophilia is a bleeding disorder associated with frequent hemarthroses and ensuing debilitating arthropathies. Patients with hemophilia (PWH) are encouraged to participate in low impact physical activities to improve joint health, mobility, and quality of life (QoL). However, activities such as walking, swimming or physical therapy are often perceived as "boring", which results in participation in high risk activities that may cause injury or bleeding. Indoor therapeutic rock climbing is practiced successfully to improve physical and psychological well-being in patients with neuromuscular disorders, and may be a "fun" alternative for PWH. The aim of this study was to investigate the safety of therapeutic rock climbing and its effects on joint health for PWH with arthropathies. Methods: Twelve adult male patients (median age 31 years, IQR=24,41) with moderate to severe hemophilia A and arthropathies (defined by decreased normative range of motion (ROM)) were recruited from the Hemophilia Treatment Centers at University of California, San Diego, USA (UCSD) and Ludwig Maximilians University, Munich, Germany (LMU)). All participants completed 12 sessions of individually tailored indoor top rope rock climbing, instructed by a climbing coach and physical therapist. Functional and clinical joint status including ROM, Hemophilia Joint Health Score (HJHS) for elbows, knees, and ankles (n=12), climbing skills (UCSD: Yosemite Decimal Scale; LMU: Union Internationale des Associations d'Alpinisme scale), QoL measures (UCSD: Haem-A-Qol, Hep-Test-Q; LMU: Hemo-Qol-A), annual bleed rate (ABR), and clotting factor consumption were assessed in both cohorts (UCSD n=6; LMU n=6) pre and post climbing. Additionally, effects on cartilage health, joint inflammation and soft tissue hypertrophy were assessed by musculoskeletal ultrasound and power doppler (MSKUS/PD) in the UCSD cohort. Descriptive statistics and Wilcoxon matched-pairs signed-rank tests were used for data analysis. Data are expressed as median and inter-quartile range; p-values ≤ 0.05 were considered significant. Results: Compared to baseline, HJHS improved significantly after completion of the program (16.5 [IQR=6.0, 28.5] post vs 17.5 [6.0, 35.0] pre; n=12; p = 0.03). A significant increase in dorsiflexion was evident in arthropathic ankles (0 degrees [IQR= -4, 4] post vs -4 [IQR-10, -3] pre; n = 9; p Conclusions:Therapeutic rock climbing is not only safe but improves joint health and well-being in PWH with advanced arthropathies without triggering excessive clotting factor consumption. "Hemophilia Vertical" is an innovative athletic concept without the "boring" stigma of other low impact sports, and should be promoted to advance athletic hemophilia care concepts. Disclosures von Drygalski: Pfizer: Consultancy, Honoraria, Speakers Bureau; Biogen: Consultancy, Honoraria, Speakers Bureau; CSL-Behring: Consultancy, Honoraria, Speakers Bureau; Novo Nordisk: Consultancy, Honoraria, Speakers Bureau; Bayer: Consultancy, Honoraria, Speakers Bureau; Baxalta/Shire: Consultancy, Honoraria, Speakers Bureau; Hematherix LLC: Membership on an entity's Board of Directors or advisory committees.

Neoangiogenesis and vascular remodeling in the hypertrophic synovium of arthropathic hemophilic joints may disturb vascular integrity and contribute to repeated bleeding (A von Drygalski, et al. Vascular Remodeling Underlies Re-Bleeding in Hemophilic Arthropathy; ASH 2015; Abstract ID 78677). However, it is difficult to distinguish the importance of the contribution of abnormal vascular structures to hemophilic joint bleeding from the contribution of low clotting factor levels. This is because most prophylactic clotting factor replacement strategies are designed to avoid trough levels below 1-5%, but not to fully correct the factor deficiency. This report reveals the importance of aberrant angiogenesis for joint bleeding in two hemophilia patients whose clotting factor activity levels were constantly normal or near normal. The first patient had severe Hemophilia A with normalized Factor (f)VIII activity levels after liver transplantation 6 years previously. Abnormal vascularization of elbows, knees and ankles (absent bleeding) was detected by musculoskeletal ultrasound and Power Doppler (PD). Three months after the baseline exam the patient experienced severe elbow bleeding associated with new vascular changes on PD. These changes were characterized by pronounced confluent, patchy vascular signals and numerous enlarged and thickened vessels by angiography consistent with acute and chronic vascular remodeling. Bleeding could not be controlled by increasing fVIII activity to supra-normal levels, and ultimately required synovial embolization. The second patient has severe Hemophilia B and presented shortly after left knee joint replacement with fluctuating knee bleeding for many months associated with pronounced dynamic vascular changes on PD. Neither bleeding nor vascular changes were prevented by daily fIX treatment which maintained near normal plasma fIX activity levels. Within one year, additional chronic bleeding with vascular changes developed in the left elbow and left ankle and remained unresponsive to intense factor replacement. These cases provide several insights. First, vascular changes in hemophilic arthropathy persist and evolve in dynamic fashion even in the presence of normal clotting factor activities. Second, vascular disturbance and leakiness contributed to bleeding independent of clotting factor activity levels as exemplified by the first patient and as suggested by the second patient. Third, the sequence of bleeding events in the second patient suggests that at least some of the observed vascular abnormalities are mediated systemically. These observations provide clinical evidence in support of the hypothesis that neoangiogenesis and vascular remodeling contribute to hemophilic joint bleeding and should lead to new studies of the pathophysiology of vascular remodeling in such joints. Targeting angiogenesis may emerge as a new treatment strategy to enhance the effectiveness of clotting factor replacement hemophilic arthropathy. Disclosures Chang: Biogen: Research Funding. Rose:Sirtex Medical: Equity Ownership, Honoraria; XLSciTech: Consultancy; Surefire Medical: Consultancy, Equity Ownership, Membership on an entity's Board of Directors or advisory committees; Embolx: Consultancy. von Drygalski:Baxalta: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Biogen: Honoraria, Research Funding; Hematherix Inc: Equity Ownership, Membership on an entity's Board of Directors or advisory committees, Patents & Royalties; Pfizer: Honoraria, Membership on an entity's Board of Directors or advisory committees; CSL Behring: Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Novo Nordisk: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Bayer: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding.

Papaverine has been associated with transient cranial nerve dysfunction after topical application during craniotomy. The authors report similar dysfunction after the use of papaverine affected brainstem structures. Two patients undergoing craniotomy for clipping of an aneurysm experienced bilateral depression of cortical somatosensory evoked potentials to both median and tibial nerve stimulation after administration of papaverine. Arterial blood gas analysis, hemodynamic parameters, and anesthetic levels remained constant throughout these somatosensory evoked potential changes. In addition, intraoperative angiography and immediate postoperative CT imaging showed intact blood flow with complete exclusion of the aneurysm. Both patients recovered within 1-2 hours and had normal neurological examination findings after extubation. Topical papaverine use may be associated with direct effects on brainstem structures. The transient nature of those changes suggests that aggressive intervention may not be needed. Maneuvers to limit the spread of papaverine to basal cisterns should be considered.