Correlates of 1-Year Change in Quality of Life in Patients with Urologic Chronic Pelvic Pain Syndrome: Findings from the Multidisciplinary Approach to the Study of Chronic Pelvic Pain (MAPP) Research Network.

PURPOSE: We evaluated and identified baseline factors associated with change in health related quality of life among patients with interstitial cystitis/bladder pain syndrome and chronic prostatitis/chronic pelvic pain syndrome. MATERIALS AND METHODS: A total of 191 men and 233 women with interstitial cystitis/bladder pain syndrome or chronic prostatitis/chronic pelvic pain syndrome (collectively referred to as urologic chronic pelvic pain syndrome) were followed for 12 months with bimonthly completion of the Short Form 12 to assess general mental and physical health related quality of life, and with biweekly assessment of condition specific health related quality of life using the Genitourinary Pain Index. A functional clustering algorithm was used to classify participants as improved, stable or worsened for each health related quality of life measure. Ordinal logistic regression was used to determine baseline factors associated with change. RESULTS: Physical health related quality of life improved in 22% of the participants, mental health related quality of life improved in 25% and condition specific health related quality of life improved in 47%. Better baseline physical health related quality of life, older age and the presence of nonurological symptoms were associated with lower likelihood of improvement in physical health related quality of life. Better baseline mental health related quality of life, female sex, and greater baseline depression and stress were associated with a lower likelihood of improvement in mental health related quality of life. Better baseline condition specific health related quality of life and more severe baseline urologic chronic pelvic pain syndrome pain symptoms were associated with a lower likelihood of improvement in condition specific health related quality of life. CONCLUSIONS: While several nonurologic chronic pelvic pain syndrome factors influenced the trajectory of general health related quality of life over time, only condition specific baseline health related quality of life and urologic chronic pelvic pain syndrome symptoms were associated with urologic chronic pelvic pain syndrome specific health related quality of life change. Significant differences in how urologic chronic pelvic pain syndrome impacts various aspects of health related quality of life suggest a multidisciplinary approach to assessment and treatment of these patients.

Reliability of the sourcil method of acetabular index measurement in developmental dysplasia of the hip.

PURPOSE: The ability to monitor and study developmental dysplasia of the hip (DDH) requires validated radiographic outcome measures. The sourcil method of acetabular index measurement (AI-S) has not yet been shown to be a reliable measure of acetabular dysplasia in a DDH population, despite its widespread use. The aims of this study were to test the reliability of the AI-S method in a DDH population, and to compare the reliability of the AI-S method with that of the classic lateral edge method (AI-L). METHODS: From an institutional database, standardized anteroposterior hip radiographs were obtained from a cohort of 35 female patients (70 hips) at two and five years of age who had been treated nonoperatively for DDH. Three observers independently measured the acetabular index using the AI-L and AI-S methods on all 70 hips at two time points, four weeks apart. RESULTS: The inter-rater reliability intraclass correlation coefficient (ICC) for the AI-L and AI-S methods was between good and excellent at 0.94 (confidence interval (CI) 0.89 to 0.96) and 0.91 (CI 0.87 to 0.94), respectively. The ICCs for intra-rater reliability for the AI-L method were excellent at 0.93 (CI 0.90 to 0.95), 0.95 (CI 0.93 to 0.97) and 0.95 (CI 0.94 to 0.97) for raters 1, 2 and 3, respectively. The ICCs for intra-rater reliability for the AI-S method were between good and excellent at 0.91 (CI 0.87 to 0.93), 0.93 (CI 0.90 to 0.95) and 0.90 (CI 0.86 to 0.93) for raters 1, 2 and 3 respectively. CONCLUSION: Both AI-S and AI-L methods are equally reliable radiographic measures of DDH. LEVEL OF EVIDENCE: Level III (diagnostic).

Expert-Based Consensus on the Principles of Pavlik Harness Management of Developmental Dysplasia of the Hip.

Developmental dysplasia of the hip (DDH) is the most common orthopaedic disorder in newborns. While the Pavlik harness is one of the most frequently used treatments for DDH, there is immense variability in treatment parameters reported in the literature and in clinical practice, leading to difficulties in standardizing teaching and comparing outcomes. In the absence of definitive quantitative evidence for the optimal Pavlik harness management strategy for DDH, we addressed this problem by obtaining international expert-based consensus on the subject. METHODS: An initial list of items relevant to Pavlik harness treatment was derived by a review of the literature. Delphi methodology was used to guide serial rounds of surveying and obtaining feedback from content matter experts from the International Hip Dysplasia Institute (IHDI), which continued in the same manner until consensus based on standard statistical analysis was reached. This was followed by a corroboration of face validity to derive the final set of management principles. RESULTS: Four rounds of structured surveying were required to reach consensus. Following 2 rounds of peer review, and from an initial list of 66 items in 8 categories, we were able to derive 2 simplified, yet comprehensive, print-friendly tables consisting of 28 items in 8 categories to assist clinicians in managing DDH with a Pavlik harness. The tables contain principles of treatment initiation, application and follow-up of the harness, complications, weaning, and end-of-treatment decision-making as well as specific criteria based on the severity of the DDH. Furthermore, highly contentious items were identified as important areas of future study. CONCLUSIONS: We developed a comprehensive set of principles based on expert consensus to assist clinicians in the management of DDH using the Pavlik harness. This study also generated a list of the most controversial areas in the nonoperative management of DDH, which should be considered high priority for future study to further refine and optimize outcomes. LEVEL OF EVIDENCE: Therapeutic Level V. See Instructions for Authors for a complete description of levels of evidence.

The incidence of avascular necrosis and the radiographic outcome following medial open reduction in children with developmental dysplasia of the hip: a systematic review.

The incidence of clinically significant avascular necrosis (AVN) following medial open reduction of the dislocated hip in children with developmental dysplasia of the hip (DDH) remains unknown. We performed a systematic review of the literature to identify all clinical studies reporting the results of medial open reduction surgery. A total of 14 papers reporting 734 hips met the inclusion criteria. The mean follow-up was 10.9 years (2 to 28). The rate of clinically significant AVN (types 2 to 4) was 20% (149/734). From these papers 221 hips in 174 children had sufficient information to permit more detailed analysis. The rate of AVN increased with the length of follow-up to 24% at skeletal maturity, with type 2 AVN predominating in hips after five years' follow-up. The presence of AVN resulted in a higher incidence of an unsatisfactory outcome at skeletal maturity (55% vs 20% in hips with no AVN; p < 0.001). A higher rate of AVN was identified when surgery was performed in children aged < 12 months, and when hips were immobilised in ≥ 60° of abduction post-operatively. Multivariate analysis showed that younger age at operation, need for further surgery and post-operative hip abduction of ≥ 60° increased the risk of the development of clinically significant AVN.

Pelvic support, pelvic symptoms, and patient satisfaction after colpocleisis.

The objective was to study the effect of colpocleisis on pelvic support, symptoms, and quality of life and report-associated morbidity and postoperative satisfaction. Women undergoing colpocleisis for treatment of pelvic organ prolapse (POP) were recruited at six centers. Baseline measures included physical examination, responses to the Pelvic Floor Distress Inventory, and Pelvic Floor Impact Questionnaire. Three and 12 months after surgery we repeated baseline measures. Of 152 patients with mean age 79 (+/-6) years, 132 (87%) completed 1 year follow-up. Three and 12 months after surgery, 90/110 (82%) and 75/103 (73%) patients following up had POP stage < or = 1. All pelvic symptom scores and related bother significantly improved at 3 and 12 months, and 125 (95%) patients said they were either 'very satisfied' or 'satisfied' with the outcome of their surgery. Colpocleisis was effective in resolving prolapse and pelvic symptoms and was associated with high patient satisfaction.

Comparing two measures of quality of life for children with haemophilia: the CHO-KLAT and the Haemo-QoL.

Disease-specific measures of quality of life (QoL) for children with haemophilia are now available for use in clinical studies [Haemophilia, 10, 2004, 9-16]. One of these measures, the Canadian Haemophilia Outcomes - Kids' Life Assessment Tool (CHO-KLAT), was developed in Canada with emphasis on the perspectives of children [Pediatr Blood Cancer, 47, 2006, 305-11; Haemophilia, 10, 2004, 34-43]. Another, the Haemo-QoL, was developed in Europe, with emphasis on the perspectives of clinicians [Haemophilia, 8, 2002, 47-54; Haemophilia, 10, 2004, 17-25]. While these two measures are unique and independent, researchers from both studies were collaboratively linked throughout development and testing. This study presents the results of a joint assessment of the two measures with respect to their strengths, limitations and unique contributions. The primary questions addressed were: 1 What is the relationship between the CHO-KLAT and the Haemo-QoL in terms of summary scores and item content? 2 What are the methodological strengths, limitations and unique contributions of each measure? We conducted a retrospective analysis of data from field testing of both measures. The analysis included a comparative assessment of the basic validity, reliability and items used in each measure. Overall, the CHO-KLAT and the Haemo-QoL are promising and valuable measures of QoL for children with haemophilia. Our analyses confirmed the basic psychometric properties of both tools, but identified some discrepancies between them. Additional data will allow for greater understanding of these discrepancies and lend clarity to how the tools should be used in clinical studies (separately or merged). The present recommendation is that the measures be run independently, but preferably concurrently in studies of children with haemophilia.

Hemophilia joint health score reliability study.

UNLABELLED: Measurement of joint health is critically important when assessing children with haemophilia. Few measures exist; they lack sensitivity to small changes, don't account for normal development and were never formally validated. To address these concerns, the Hemophilia Joint Health Score (HJHS) was developed by modifying existing scores. OBJECTIVE: To test the inter-observer and test-retest reliability of the HJHS. METHODS: Using a fully factorial design, four physiotherapists (from Canada, the United States and Sweden) examined eight boys with severe haemophilia A on two consecutive days using the HJHS. The boys ranged in age from 4-12 years and presented with variable joint damage. Six index joints (elbows, knees and ankles) were assessed on 11 impairment items including swelling, flexion and extension loss and gait. Concordance was measured by the intra-class correlation co-efficient. RESULTS: Reliability of the HJHS was excellent with an inter-observer co-efficient of 0.83 and a test-retest of 0.89. CONCLUSION: This study is the first in a series to assess the psychometric properties of the HJHS, a promising new measure of joint health in boys with haemophilia.

Physical therapy and imaging outcome measures in a haemophilia population treated with factor prophylaxis: current status and future directions.

Routine infusions of factor VIII to prevent bleeding, known as prophylaxis, and other intensive therapies are being more broadly applied to patients with haemophilia. These therapies differ widely in replacement product usage, cost, frequency of venous access and parental effort. In order to address residual issues relating to recommendations, implementation, and evaluations of prophylaxis therapy in persons with haemophila, a multinational working group was formed and called the International Prophylaxis Study Group (IPSG). The group was comprised of haemophilia treaters actively involved in studies of prophylaxis from North America and Europe. Two expert committees, the Physical Therapy (PT) Working Group and the Magnetic Resonance Imaging (MRI) Working Group were organized to critically assess existing tools for assessment of joint outcome. These two committees independently concluded that the WFH Physical Examination Scale (WFH PE Scale) and the WFH X-ray Scale (WFH XR Scale) were inadequately sensitive to detect early changes in joints. New scales were developed based on suggested modifications of the existing scales and called the Haemophilia Joint Health Score (HJHS) and the International MRI Scales. The new scales were piloted. Concordance was measured by the intra-class correlation coefficient of variation. Reliability of the HJHS was excellent with an inter-observer co-efficient of 0.83 and a test-retest value of 0.89. The MRI study was conducted using both Denver and European scoring approaches; inter-reader reliability using the two approaches was 0.88 and 0.87; test-retest reliability was 0.92 and 0.93. These new PT and MRI scales promise to improve outcome assessment in children on early preventive treatment regimens.

Development of a health-related quality of life measure for boys with haemophilia: the Canadian Haemophilia Outcomes--Kids Life Assessment Tool (CHO-KLAT).

Several measures of quality of life (QoL) are available for children with haemophilia. However, most are not disease-specific and few focus on children's perspectives. The purpose of this study was to develop a psychometrically sound measure of QoL that included the perspectives of boys with haemophilia. A list of potential items was developed from the literature, other measures, and input from five discussion sessions with adults with haemophilia, children with haemophilia and their parents and haemophilia nurses. The list was augmented with items generated by three focus groups with children and three focus groups with parents. These groups also prioritized items and recommended a domain structure. Supplemental information was gathered by surveying haematologists. Data from all sources were analysed to reduce the number of items using a two-step approach, based on rules that weighted the children's priorities most heavily. The remaining items were compiled into a questionnaire that was pilot tested with 10 children and their parents. The total item pool contained 228 potential items. Of these, 33 were removed based on three focus groups and survey responses, 72 were removed after the completion of all focus groups and 46 were removed due to redundancy. This resulted in a 77-item version of the CHO-KLAT. Pilot testing identified the need to subdivide two items, resulting in a 79-item CHO-KLAT. The CHO-KLAT is a promising disease-specific measure of QoL that reflects children's unique perspectives. This child-centric focus distinguishes the CHO-KLAT from alternative measures of QoL. Further research will assess the measurement properties of the CHO-KLAT.

Endometrial adenocarcinoma with trophoblastic differentiation.

OBJECTIVE: To report a case of stage IIIc poorly differentiated endometrial adenocarcinoma with trophoblastic differentiation and to review previously reported cases. METHODS: The clinical course and histopathology of the case were reviewed, and a literature search for other reported cases was performed. RESULTS: The tumor contained syncytiotrophoblast-like giant cells that stained positively for the beta subunit of human chorionic gonadotropin (beta-hCG), and the patient's serum beta-hCG level was elevated (95 mIU/ml), but became undetectable after treatment. Beta-hCG was used as a tumor marker during further therapy. At 16 months' survival, she remains without evidence of disease and with a beta-hCG (level < 5 mIU/ml). Nine other cases of trophoblastic differentiation in gynecologic nontrophoblastic tumors have been reported, five in endometrial carcinomas which we review. CONCLUSIONS: Trophoblastic differentiation in gynecologic nontrophoblastic tumors is rare. Beta-hCG may be useful as a tumor marker in these cases. The clinical behavior of these tumors has been aggressive, with advanced stages at diagnosis, early widespread metastases or recurrences and poor patient outcomes. The patient presented in this report, however, remains without evidence of disease 16 months following diagnosis and may be the longest survivor with this tumor reported to date.