Predictors of non-adherence to prescribed prophylactic clotting-factor treatment regimens among adolescent and young adults with a bleeding disorder.

INTRODUCTION: Adherence to clotting-factor treatment regimens, especially among adolescents and young adults (AYAs), is under-researched. AIM: We determined factors associated with better adherence to prophylaxis. METHODS: From April through December 2012, a convenience sample of AYA (aged 13-25 years) persons with haemophilia or von Willebrand disease (VWD) completed an online survey that assessed adherence to prescribed prophylactic treatment regimens [Validated Haemophilia Regimen Treatment Adherence Scale (VERITAS)-Pro]. Logistic regression analysis assessed demographic and clinical factors related to non-adherence (VERITAS-Pro≥57). RESULTS: Seventy-three prophylactically treating AYAs participated. Of which, 88%, 8% and 4% had haemophilia A, B and VWD respectively. Almost all (90%) had severe disease and 58% had never developed an inhibitor. Most were aged 13-17 years (56%), white (78%), non-Hispanic (88%), never married (94%) and had some type of health insurance (96%). Median VERITAS-Pro score was 48 (range = 25-78) and 22 (30%) participants were non-adherent to prophylaxis (VERITAS-Pro≥57). Final logistic regression modelling suggested that, compared to those aged 13-17 years, participants aged 18-25 years were 6.2 (95% CI: 1.8-21.0; P < 0.01) times more likely to be non-adherent. Compared to respondents whose mother had at least a Bachelor's degree, respondents whose mother did not were 3.8 (95% CI: 1.0-14.3; P = 0.05) times more likely to be non-adherent. CONCLUSIONS: Results suggest that adherence efforts should be especially targeted to young adults as they transition from adolescence (i.e. parental supervision) and assume primary responsibility for their bleeding disorder care. Healthcare providers should be mindful of AYAs whose mothers have less formal education and ensure that adequate time and resources are dedicated to family adherence education.

Better adherence to prescribed treatment regimen is related to less chronic pain among adolescents and young adults with moderate or severe haemophilia.

Little data exist, especially for adolescent and young adult (AYA) persons with haemophilia (PWH), about the relationship between adherence to prescribed treatment regimen and chronic pain. We examined this relationship among PWH (moderate or severe) aged 13-25 via cross-sectional survey. Adherence was assessed using the Validated Hemophilia Regimen Treatment Adherence Scale (VERITAS)-Pro and VERITAS-PRN for prophylactic and on-demand participants respectively. VERITAS scores range from 24 (most adherent) to 120 (least adherent). Chronic pain was measured using the FPS-R and was dichotomized as high for FPS-R scores ≥4 and low for <4. Logistic regression models were constructed to assess factors associated with having high (vs. low) chronic pain. Of 80 AYA respondents (79 men), most had severe disease (91%), infused prophylactically (86%) and had haemophilia A (91%). Fifty-one per cent were aged 13-17 and most were white (76%), non-Hispanic (88%) and never married (93%). Chronic pain was reported as high for 35% of respondents. Mean VERITAS-Pro scores for those with high and low chronic pain were 53.6 ± 12.3 vs. 47.4 ± 12.9, P = 0.05. VERITAS-PRN scores were similar across chronic pain status. Logistic regression revealed that for each 10-point reduction (i.e. increase in adherence) in the combined VERITAS (Pro and PRN) and VERITAS-Pro scores there was a 35% (OR = 0.65; 95% CI = 0.44, 0.96; P = 0.03) and 39% (OR = 0.61; 95%CI = 0.39, 0.96; P = 0.03) reduction in odds of having high chronic pain respectively. Among AYA PWHs, better adherence was associated with significantly lower odds of having high chronic pain. Moreover, non-whites were >4 times as likely as whites to report high chronic pain.

Current prescription of prophylactic factor infusions and perceived adherence for children and adolescents with haemophilia: a survey of haemophilia healthcare professionals in the United States.

The primary goal of prophylaxis in patients with severe haemophilia is to convert the phenotype from severe to moderate and to prevent the development of chronic arthropathy. Prior studies have demonstrated that prophylaxis decreases episodes of joint bleeds and chronic arthropathy. Effectiveness depends on prescription of prophylaxis and adherence to the prescribed regimen. The aim of this study was to determine if prescription of prophylaxis for children with haemophilia and perceptions of adherence to prophylaxis have changed since publication of the Joint Outcome Study (JOS). A questionnaire was sent, in electronic and written formats, to health professionals who provide care to children with haemophilia at US haemophilia treatment centres (HTCs). The response rate was 56 of 128 (44%) of the targeted HTCs. There were a few missing data and denominators are provided. All responses agreed with the results of the JOS and 30/55 (55%) reported the JOS increased their prescription of prophylaxis. Nineteen of 56 (34%) physicians or HTC staff reported that they had not prescribed prophylaxis within the last year due to concerns about adherence, and 19/56 (34%) reported they had stopped prophylaxis due to concerns about adherence within the last year. Predicted adherence decreased with increasing age. Prescription of prophylaxis appears to be increasing since publication of the JOS. Strategies to improve adherence may increase the likelihood of physician prescription of prophylaxis and make prophylaxis easier to implement for individual patients, thereby improving the clinical outcome of children and adults with haemophilia.

Transition of care for pediatric and adult patients with venous thromboembolism: A National Quality Improvement Project from the American Thrombosis and Hemostasis Network (ATHN).

BACKGROUND: Transition of care (TOC) for management of anticoagulation from inpatient to outpatient setting for patients with acute venous thromboembolism (VTE) poses serious safety concerns. We implemented a national quality improvement educational initiative to address this issue. METHODS: Pediatric and adult patients admitted for their first VTE were prospectively enrolled at 16 centers from January 2016 to December 2018. Patient demographics, VTE diagnosis, risk factors, and treatment characteristics were collected. There were two phases: pre-intervention (PI) and quality intervention (QI). The PI phase assessed the quality and patient understanding and satisfaction of anticoagulation instructions given at hospital discharge and adherence to these instructions via a patient and/or caregiver feedback questionnaire (PFQ) and a patient knowledge questionnaire (PKQ) at 30 days. The QI phase provided patient and/or caregiver enhanced education regarding anticoagulation therapy and VTE at hospital discharge using a comprehensive discharge instruction module and a phone call follow-up at one week. Patient and/or caregiver knowledge at 7 and 30 days was assessed with the same PFQ and PKQ and compared to the PI baseline measures. RESULTS: Of the 409 study patients, 210 (51%) were adults, 218 (53%) females, and 316 (77%) White. Deep vein thrombosis (62.8%) and pulmonary embolism (47.9%) were the most common VTE in children and adults, respectively. Day 30 PFQ scores were significantly higher in the QI phase compared to the PI phase by 11% (p < 0.01). Day 30 PKQ demonstrated enhanced teaching (93.7% vs. 83.5%, p-value 0.004) and disease recognition (89.6% vs. 84.6% p = 0.03) in the QI phase than the PI phase. CONCLUSION: Comprehensive VTE discharge instructions followed by a 1-week post-discharge phone call strengthen patient and caregiver knowledge, satisfaction of education given and care provided, and disease recognition.

Operative intervention for carotid restenosis is safe and effective.

Carotid stenting has been proposed as an alternative to reoperative carotid endarterectomy (rCEA) for recurrent carotid stenosis. The purpose of this study is to prove the safety, effectiveness and durability of reoperation in long term follow up of 18 years in a community hospital setting. From March 1988 to April 2005 80 patients, 46 men and 34 women (mean age: 64.1 years) underwent a total of 83 operations. Symptomatic recurrent stenosis (>70%) was the indication in 32, asymptomatic high-grade stenosis (>80%) in 49, intimal flap in one and fibromuscular dysplasia (F.M.D), in one. The initial operation was carotid endarterectomy with primary closure in 60 and prosthetic patch in 23. The mean recurrences were at 23.3 months in 33 with myointimal hyperplasia, 105.4 months in 29 with recurrent atherosclerosis, 61.4 months in 19 with both hyperplasia and atherosclerosis, 2 months in one with intimal flap and 8 months in one with F.M.D bands. Reoperation utilized primary closure (3), vein patch (14), prosthetic patch (55), Gore-Tex interposition grafts (7), vein interposition grafts (3) and intraoperative dilation (1). No perioperative strokes or deaths occurred. One patient died from cardiac complications following combined rCEA and coronary artery bypass grafting. Operative morbidity consisted of reversible nerve injury (5), irreversible recurrent laryngeal nerve injury (1) and hematoma requiring evacuation (3). During follow up (3-153 months; mean: 50.9) carotid occlusion resulted in mild ipsilateral stroke in one patient, and one non-hemispheric stroke. There were 26 late deaths due to all causes, one due to CVA. Eight patients required reoperation (mean 53.4 months). Seven of these were hypertensive. Kaplan-Meier analysis of long-term follow up shows relatively high stroke free rates; at 153 months (12.75 years) the hemispheric stroke free rate was 98.67% and the all-stroke free rate was 95.85%. The survival estimate following redo surgery was 69.97% at 5 years and 40.23% at 10 years. We found that individuals on statin therapy (p-value=0.0042), and those on combination of statin and aspirin (p-value=0.0320), had significantly increased interval between primary and secondary operation. Increased age was correlated to a decreased time to redo surgery (p-value=<0.0001). We conclude that reoperation for recurrent carotid stenosis using standard vascular techniques is safe, effective, durable and cost effective. It should continue to be the mainstay of treatment when secondary intervention is required. Statins have a salutary effect on durability of the procedure and should be used when indicated.

Characteristic retinal atrophy with secondary "inverse" optic atrophy identifies vigabatrin toxicity in children.

OBJECTIVE: To describe the clinical pattern of retinal atrophy in children caused by the anticonvulsant vigabatrin. DESIGN: An interventional case series report. PARTICIPANTS: One hundred thirty-eight patients, mainly infants, were evaluated regularly for evidence of possible vigabatrin toxicity in the Eye and Neurology clinics at the Hospital for Sick Children, Toronto. METHOD: Sequential clinical and electroretinographic (International Society for Clinical Electrophysiology of Vision standards) evaluations every 6 months. MAIN OUTCOME MEASURES: Presence of recognizable retinal and optic atrophy in the presence of abnormal electroretinogram (ERG) and other clinical findings. RESULTS: Three children being treated for seizures with vigabatrin showed definite clinical findings of peripheral retinal nerve fiber layer atrophy, with relative sparing of the central or macular portion of the retina and relative nasal optic nerve atrophic changes. Some macular wrinkling was evident in 1 case. Progressive ERG changes showing decreased responses, especially the 30-Hz flicker response, supported the presence of decreased retinal function. CONCLUSIONS: A recognizable and characteristic form of peripheral retinal atrophy and nasal or "inverse" optic disc atrophy can occur in a small number of children being treated with vigabatrin. The changes in superficial light reflexes of the retina in children facilitate the clinical recognition of nerve fiber layer atrophy. The macula is relatively spared, although superficial retinal light reflexes indicating wrinkling of the innermost retina suggest early macular toxicity as well. Because these changes are accompanied by electrophysiologic evidence of retinal dysfunction, discontinuation of vigabatrin should be strongly considered.

A canine model of paired graft thrombosis.

A novel canine model for reliably producing graft thrombosis is described. Two unilateral aortoiliac bypasses are performed while preserving the integrity of the native vascular tree. These grafts are briefly mechanically occluded at the time of the initial operation, and they remain occluded postoperatively because flow is preferentially directed through the larger-caliber, thrombo-resistant native circulation. Percutaneous temporary intra-arterial balloon occlusion of the native vessels redirects flow into the occluded graft segments, which can be used in experimental protocols. This new model for studying graft thrombosis requires only a single, simple operative procedure, and easily allows secondary percutaneous graft manipulation.

Recurrent sympathetic dystrophy: successful treatment by contralateral sympathectomy.

Reflex sympathetic dystrophy comprises a group of disorders characterized by disabling pain and vasomotor disturbances. Sympathectomy has become a mainstay of therapy. Recurrent symptoms following sympathectomy have been attributed to incorrect diagnosis, delayed treatment, or secondary gains. This report concerns a case of lower-extremity reflex sympathetic dystrophy that recurred following an initially curative sympathectomy and that responded to treatment with contralateral sympathectomy. We suggest that these recurrent symptoms may be due to reinnervation from the contralateral lumbar sympathetic chain.

Ruptured abdominal aneurysm subsequent to cystic medial necrosis: a case report.

The majority of abdominal aortic aneurysms are the result of atherosclerosis. Cystic medial necrosis is a pathologic finding classically associated with thoracic aortic dissection, particularly in patients with Marfan's syndrome. The presence of cystic medial necrosis in other segments of the aorta or its branches is unusual. We report a case of a ruptured abdominal aortic aneurysm in a 38-year-old woman without Marfan's syndrome.

Calcified pancreatic pseudocysts.

Extensive calcification of a pancreatic pseudocyst that permits visualization on plain abdominal radiographs is unusual. When such x-ray findings are encountered, a broad differential diagnosis can be made, which includes tumors, cysts, abscesses, or malformations of the adjacent structures. Two cases of calcified pancreatic pseudocysts are discussed. Calcified pancreatic pseudocysts may be a potential source of complications such as pain, bleeding, or infection. Because the cyst wall is mature and spontaneous resolution is unlikely, proper treatment of calcified pseudocysts consists of timely resection or internal drainage.

Contralateral effects of unilateral resistance training: a meta-analysis.

It is often claimed that strength training of one limb increases the strength of the contralateral limb, but this has not been demonstrated consistently, particularly in well-controlled studies. The aim was to quantitatively combine the results of other studies on the effects of unilateral training on contralateral strength in humans to provide an answer to this physiological question. We analyzed all randomized controlled studies of voluntary unilateral resistance training that used training intensities of at least 50% of maximal voluntary strength for a minimum of 2 wk. Studies were identified by computerized and hand searches of the literature. Data on changes in strength of contralateral and control limbs were extracted and statistically pooled in a meta-analysis. This approach allows conclusions to be based on a statistically meaningful sample size, which might be difficult to achieve in other ways. Seventeen studies met the inclusion criteria, and 13 provided enough data for statistical pooling. The contralateral effects of strength training reported in individual studies varied from -2.7 to 21.6% of initial strength. The pooled estimate of the effect of unilateral resistance training on the maximal voluntary strength of the contralateral limb was 7.8% (95% confidence interval: 4.1-11.6%). This was 35.1% (95% confidence interval: 20.9-49.3%) of the effect on the trained limb. Pooling of all available data shows that unilateral strength training produces modest increases in contralateral strength.

Simultaneous treatment of chronic pancreatitis and pancreatic pseudocyst.

Records from 87 consecutive patients undergoing lateral pancreaticojejunostomy (LPJ) for chronic pancreatitis were reviewed to determine the incidence of pseudocyst and the safety of combined pancreatic duct and pseudocyst drainage. Twelve patients had undergone previous pancreatic pseudocyst drainage; four of them also had pancreatic pseudocysts present at the time of LPJ. In addition, 22 patients had pseudocysts identified preoperatively and/or confirmed at operation. The overall incidence of pseudocyst was 39%. Twenty-six patients (group 1) underwent pancreaticojejunostomy combined with pseudocyst drainage. Sixty-one patients (group 2) underwent pancreaticojejunostomy only. Operative morbidity and mortality results (19% and 8%, respectively, in group 1; 18% and 2%, respectively, in group 2) were similar. Patient outcome was also similar in the two groups (81% and 84% of patients obtained pain relief in groups 1 and 2, respectively). There were no pseudocyst recurrences in either group. Thus, there is a high incidence (39%) of pancreatic pseudocyst in patients undergoing LPJ for chronic pancreatitis. Combined drainage of the pancreatic duct and pseudocyst is safe and effective.

Routine completion study during carotid endarterectomy is not necessary.

The results of many studies have suggested the need for a completion study during carotid endarterectomy (CE). This paper describes our experience not routinely using completion studies. We retrospectively reviewed the charts of 417 patients who underwent 455 CEs. Demographic features, risk factors, ipsilateral neurologic events during the first 30 days, and mortality data were identified. There were 14 neurologic events and 4 deaths. No technical defects were found in 13 patients; 1 patient did not have exploratory surgery after an occlusion. Long-term follow-up shows 10 of the 14 arteries are open. Two patients were lost to follow-up, 1 patient died, and 1 artery was not explored. We conclude that CE may be carried out without routinely using a completion study, with an acceptable postoperative neurologic complication rate. Careful technique is mandatory.

Long-term follow-up of bypasses to the brachial artery across the shoulder joint.

BACKGROUND: Most atherosclerotic lesions in the subclavian artery are successfully treated with carotid to subclavian bypass. The need to bypass to the brachial artery (BA) is rare. We reviewed our experience with this bypass. METHODS: Over a 10-year period, we have performed 13 bypasses to the BA originating from an artery proximal to the shoulder joint. In this retrospective study, the demographic and clinical risk factors were evaluated. Long-term results were analyzed. RESULTS: Thirteen operations were performed in 10 patients, aged 47 to 80 years. The operations were carried out for acute severe ischemia in 1 limb, effort discomfort in 9, and rest pain in 3 limbs. Donor arteries were axillary (7), carotid (4), and subclavian (2). All bypasses were to the BA proximal to the elbow joints. Life-table analysis showed 100% patency in the first 3 years and 88% at 7 years. There were 2 deaths in follow-up. Average preoperative brachial to brachial index was 0.59 and postoperative index was 1.1. In patients with bilateral occlusions, mean preoperative brachial artery pressure was 62 mm Hg, which improved to 142 mm Hg postoperatively. There were no neurological complications and no 30-day mortality. CONCLUSIONS: Bypass across the shoulder joint to the BA using expanded polytetrafluoroethylene (ePTFE) or vein is a safe operation with excellent long-term patency. The carotid artery can be used as a donor vessel without complications. Hypertension and female gender appear to be risk factors for extensive disease in proximal upper extremity arteries.

Retroperitoneal versus transperitoneal approach for repair of abdominal aortic aneurysms.

We, as well as other authors, believe that the retroperitoneal approach is an excellent alternative to the transperitoneal route for the repair of abdominal aortic aneurysms. This approach is associated with a significant decrease in pulmonary and cardiac complications and therefore can be used in selected high-risk patients with expanding aneurysms. A well-controlled randomized multicenter trial should answer the question: "Is this approach the surgical access of choice for the elective repair of abdominal aortic aneurysms?"

Needle biopsy of nodular thyroid disease.

To evaluate nodular thyroid disease, 150 patients underwent 169 fine needle biopsies (FNB) and recently 28 have also had core needle biopsies (CNB). Multiple biopsies were required in 19 patients because of multiple lesions, reaccumulation of cysts, follow-up of nodules failing to regress, or inadequate material. Adequate material was obtained in 97 per cent of FNB and 92 per cent of CNB. FNB and CNB agreed in 20 of 28 cases. There were no complications with FNB and one patient (4%) hemorrhaged with CNB. Forty nine patients underwent thyroidectomy. Postoperative diagnoses include papillary carcinoma (9), follicular carcinoma (2), lymphoma (2), medullary carcinoma (1), metastatic carcinoma (1), benign nodular goiter (14), follicular neoplasm (15), and thyroiditis (5). Sixty per cent of patients avoided surgery, 61 per cent of operated specimens contained neoplasia and 31 per cent contained malignancy. Eighty seven per cent of malignancies were identified at the initial evaluation. FNB had 86 per cent sensitivity for neoplasia and 44 per cent specificity for neoplasm (94% for papillary carcinoma). CNB had 89 per cent sensitivity and 67 per cent specificity for neoplasm. FNB and CNB are useful means of assessing thyroid nodules for the presence of cancer. They can decrease the need for diagnostic thyroidectomy. However, clinical evaluation must still be used in conjunction when determining the need for thyroidectomy.

Ileal perforation due to arteriovenous malformation in a premature infant.

Gastrointestinal arteriovenous malformations (AVM) in children are most commonly associated with bleeding. Although not previously reported, we present a case of an AVM associated with intestinal perforation in a premature infant.

The impact of angiodynography on infrainguinal bypass graft surveillance.

We have followed 64 lower extremity in situ grafts and 56 selected vein or prosthetic grafts with serial angiodynography since 1988 (Quantum Color-flow Duplex) and ankle/brachial indices (ABI). Each graft can be scanned in 20 minutes. Scan results affected clinical decision making in 26 cases. There were ten graft stenoses, 10 distal stenoses, 5 large arteriovenous fistulas (AFV), and one limb with a stenosis and AFV. Four other limbs had incompressible vessels and normal angiodynograms. There were no false positives. Twenty-eight graft revisions were performed in 24 patients. Seven stenoses detected by angiodynogram were not accompanied by changes in ABI. Cumulative patency of revised grafts were 61% at 3 years. The detection of graft or distal arterial stenoses by color flow changes is accurate and rapid. Scanning is recommended for all lower extremity bypass grafts. ABI alone is not sufficient. Scanning helps in planning the surgical incision and may obviate arteriography in selected cases.

Selective non-operative management of pseudoaneurysms and arteriovenous fistulae complicating femoral artery catheterization.

Femoral arterial pseudoaneurysms or arteriovenous fistulae may sometimes complicate percutaneous femoral artery catheterization procedures. Most surgeons recommend prompt operative repair because of the unfavorable natural history of pseudoaneurysms or arteriovenous fistulae secondary to violent or accidental arterial trauma. However, the natural history of catheterization-induced pseudoaneurysms and arteriovenous fistulae has not been well documented. Accordingly, we prospectively studied the natural history of 22 pseudoaneurysms, 8 arteriovenous fistulae, and 3 combined lesions, identified by duplex scan in 32 patients following trans-femoral cardiac, peripheral vascular, or vascular access arterial catheterization procedures. Angiographic procedures were performed with the use of 5-8F introducer sheaths. A femoral artery complication was significantly more likely to result from coronary balloon angioplasty (9/304; 3.0%) than from diagnostic cardiac catheterization (21/2476; 0.8%) (p less than 0.003; chi square). Fourteen patients (13 pseudoaneurysms, 1 combined pseudoaneurysm/fistulae) underwent surgical repair. Pain and/or enlarging hematoma resulted in repair within two days of the diagnosis in 8 patients. The need for chronic anticoagulation prompted elective repair in 2 patients. A pseudoaneurysm was repaired in one patient five days following catheterization when it became painful. In three stable patients, asymptomatic pseudoaneurysms were repaired electively during another surgical procedure. There were no operative deaths. One patients (7%) developed a wound infection postoperatively. Eighteen patients (19 arterial lesions: 9 pseudoaneurysms, 8 arteriovenous fistulae, 2 combined pseudoaneurysms/arteriovenous fistulae) with improving symptoms and stable physical signs were followed by serial clinical evaluation and duplex scans. Seventeen of 19 (89%) of these lesions resolved spontaneously within 5-90 days (mean 30.7 days).(ABSTRACT TRUNCATED AT 250 WORDS)

Passive moment about the hip in straight leg raising.

OBJECTIVE: The purpose of this examine is to study the load-deformation characteristics of the hip in straight leg raising. DESIGN: An experimental study in which passive moment about the hip was determined as a function of hip angle. BACKGROUND: Straight leg raising is widely employed in clinical examination, and there is little information on its mechanical characteristics. METHODS: Fourteen healthy volunteers were recruited for this study. Three trials of straight leg raise tests were performed while subjects lay supine on a plinth that was fitted with load cells. An electrogoniometer was employed to measure hip flexion during the test. Resistive moment at the hip was determined using a dynamic biomechanical model. RESULTS: The present experimental method was shown to be highly reliable. The moment-angle curves of all subjects were shown to follow an exponential function. CONCLUSION: Stiffness and strain energy of posterior hip tissues could be derived from the moment-angle curves. Evaluation of such elastic properties is clinically important as they may be altered with injuries of the tissues. RELEVANCE: Clinically, contracture of hamstring muscles and other posterior hip tissues is evaluated by measuring the available range of hip flexion in straight leg raising. However, this does not provide any information on the elastic properties of the tissues. The present study reports a reliable method of evaluating such properties.