A proposed methodology for uncertainty extraction and verification in priority setting partnerships with the James Lind Alliance: an example from the Common Conditions Affecting the Hand and Wrist Priority Setting Partnership.

BACKGROUND: To report our recommended methodology for extracting and then confirming research uncertainties - areas where research has failed to answer a research question - derived from previously published literature during a broad scope Priority Setting Partnership (PSP) with the James Lind Alliance (JLA). METHODS: This process was completed in the UK as part of the PSP for "Common Conditions Affecting the Hand and Wrist", comprising of health professionals, patients and carers and reports the data (uncertainty) extraction phase of this. The PSP followed the robust methodology dictated by the JLA and sought to identify knowledge gaps, termed "uncertainties" by the JLA. Published Cochrane Systematic Reviews, Guidelines and Protocols, NICE (National Institute for Health and Care Excellence) Guidelines, and SIGN (Scottish Intercollegiate Guidelines Network) Guidelines were screened for documented "uncertainties". A robust method of screening, internally verifying and then checking uncertainties was adopted. This included independent screening and data extraction by multiple researchers and use of a PRISMA flowchart, alongside steering group consensus processes. Selection of research uncertainties was guided by the scope of the Common Conditions Affecting the Hand and Wrist PSP which focused on "common" hand conditions routinely treated by hand specialists, including hand surgeons and hand therapists limited to identifying questions concerning the results of intervention, and not the basic science or epidemiology behind disease. RESULTS: Of the 2358 records identified (after removal of duplicates) which entered the screening process, 186 records were presented to the PSP steering group for eligibility assessment; 79 were deemed within scope and included for the purpose of research uncertainty extraction (45 full Cochrane Reviews, 18 Cochrane Review protocols, 16 Guidelines). These yielded 89 research uncertainties, which were compared to the stakeholder survey, and added to the longlist where necessary; before derived uncertainties were checked against non-Cochrane published systematic reviews. CONCLUSIONS: In carrying out this work, beyond reporting on output of the Common Conditions Affecting the Hand and Wrist PSP, we detail the methodology and processes we hope can inform and facilitate the work of future PSPs and other evidence reviews, especially those with a broader scope beyond a single disease or condition.

A randomised feasibility trial comparing needle fasciotomy with limited fasciectomy treatment for Dupuytren's contractures.

PURPOSE: The purpose of this study is to assess the feasibility of conducting a large, multicentre randomised controlled trial (RCT) comparing needle fasciotomy with limited fasciectomy for treatment of Dupuytren's contractures. DESIGN: The design of this study is a parallel, two-arm, multicentre, randomised feasibility trial with embedded QuinteT Recruitment Intervention. PARTICIPANTS: Patients aged 18 years or over who were referred from primary to secondary care for treatment of a hand with Dupuytren's contractures of one or more fingers of more than 30° at the metacarpophalangeal (MCP) and/or proximal interphalangeal (PIP) joints and well-defined cord(s). Patients were excluded if they had undergone previous Dupuytren's contracture surgery on the same hand. METHODS: Potential participants were screened for eligibility. Recruited participants randomised (1:1) to treatment with either needle fasciotomy or limited fasciectomy and followed-up for up to 6 months after treatment. Data on recruitment rates, completion of follow-up, and procedure costs were collected. Four patient reported outcome measures (PROMs) and objective outcome measures were collected before intervention and 6 weeks and 6 months afterwards. RESULTS: One hundred and fifty-three of 267 (57%) primary-care referrals for Dupuytren's contractures met the eligibility criteria for the study. Seventy-one of the 153 (46%) agreed to participate and were randomly allocated to treatment with needle fasciotomy or limited fasciectomy. Sixty-seven of these underwent their allocated treatment, two were crossovers from limited fasciectomy to needle fasciotomy, and two (both allocated limited fasciectomy) received no treatment. Fifty-nine participants (85%) completed 6-month follow-up PROMs. Participants felt the MYMOP, PEM and URAM PROMs allowed them to better describe how their treatment affected their hand function than the DASH PROM. The estimated costs of limited fasciectomy (in an operating theatre) and needle fasciotomy (in a clinic room) were £777 and £111 respectively. CONCLUSION: A large RCT comparing treatment of Dupuytren's contractures by needle fasciotomy and limited fasciectomy is feasible. Data from this study will help determine the number of sites and duration of recruitment required to complete an adequately powered RCT and will assist the selection of PROMs in future studies on the treatment of Dupuytren's contractures. (Level 1 feasibility study). TRIAL REGISTRATION: Trial registered with ISRCTN (registration number: ISRCTN11164292), date assigned - 28/08/2015.

Hereditary vitamin D-resistant rickets in Greek children: genotype, phenotype, and long-term response to treatment.

We describe four patients (two pairs of children from two unrelated kindreds) from a Greek island, suffering from hereditary vitamin D-resistant rickets (HVDRR) with alopecia. There were two different homozygous mutations in the vitamin D receptor (VDR) gene of the affected members of the two kindreds that resulted in a truncated or missing receptor. The disorder began in early infancy with similar clinical, biochemical and radiological findings in all four patients, namely, alopecia (which provided the initial diagnostic evidence for HVDRR), rachitic deformities, hypocalcemia, hypophosphatemia, secondary hyperparathyroidism, and elevated serum levels of 1,25-dihydroxyvitamin D; however, the patients of kindred B had a more severe clinical expression. Treatment options include oral or intravenous calcium and active vitamin D metabolites. The response varies widely in different cases. Our patients were initially treated with high doses of 1alpha(OH)D3 and oral calcium supplementation. Kindred A patients had a satisfactory response to this regimen, while kindred B patients presented clinical and biochemical improvement when 1alpha(OH)D3 was changed to 1,25(OH)2D3. In the older patients of each kindred, treatment requirements gradually decreased during puberty, and therapy was finally discontinued before adulthood.

The effects of Scaphoid and Colles casts on hand function.

Although necessary for bone healing, immobilisation temporarily prevents hand function and may necessitate corrective physiotherapy later. Scaphoid and Colles casts are both commonly used to immobilize scaphoid fractures. Non-union rates are comparable with both casts. The Scaphoid cast incorporates the thumb, whereas the Colles cast leaves the thumb free. We compared the effect of the two casts on hand function in 20 healthy right-hand-dominant volunteers using the Jebsen-Taylor Hand Function Test. Data were obtained through a mixed between and within subject design. Both casts prolonged the time taken to complete the hand function test compared to controls. Testing in the Scaphoid cast took significantly longer than in the Colles cast.

Cost-effectiveness of volar locking plate versus percutaneous fixation for distal radial fractures: Economic evaluation alongside a randomised clinical trial.

This study compares the cost-effectiveness of treating dorsally displaced distal radial fractures with a volar locking plate and percutaneous fixation. It was performed from the perspective of the National Health Service (NHS) using data from a single-centre randomised controlled trial. In total 130 patients (18 to 73 years of age) with a dorsally displaced distal radial fracture were randomised to treatment with either a volar locking plate (n = 66) or percutaneous fixation (n = 64). The methodology was according to National Institute for Health and Care Excellence guidance for technology appraisals. . There were no significant differences in quality of life scores between groups at any time point in the study. Both groups returned to baseline one year post-operatively. NHS costs for the plate group were significantly higher (p < 0.001, 95% confidence interval 497 to 930). For an additional £713, fixation with a volar locking plate offered 0.0178 additional quality-adjusted life years in the year after surgery. The incremental cost-effectiveness ratio (ICER) for plate fixation relative to percutaneous fixation at list price was £40 068. When adjusting the prices of the implants for a 20% hospital discount, the ICER was £31 898. Patients who underwent plate fixation did not return to work earlier. We found no evidence to support the cost-effectiveness, from the perspective of the NHS, of fixation using a volar locking plate over percutaneous fixation for the operative treatment of a dorsally displaced radial fracture.

Extra-articular fractures of the distal radius--a European view point.

There is no unified consensus view on the management of distal radius fractures within Europe. This is partially because of the failure of clinical studies to demonstrate superiority of one treatment technique over the others. Nonclinical factors, such as cost and operating room availability, also contribute to the decision making regarding treatment, and there remains uncertainty as to the criteria that need to be fulfilled to achieve a good functional result. This article therefore does not describe a unified European viewpoint, but the viewpoint of two Europeans working within the health care system of one European country.

Epidemiology and outcome of fracture of the hip in women aged 65 years and under: a cohort study.

We examined prospectively collected data from 6782 consecutive hip fractures and identified 327 fractures in 315 women aged ≤65 years. We report on their demographic characteristics, treatment and outcome and compare them with a cohort of 4810 hip fractures in 4542 women aged > 65 years. The first significant increase in age-related incidence of hip fracture was at 45, rather than 50, which is when screening by the osteoporosis service starts in most health areas. Hip fractures in younger women are sustained by a population at risk as a result of underlying disease. Mortality of younger women with hip fracture was 46 times the background mortality of the female population. Smoking had a strong influence on the relative risk of 'early' (≤ 65 years of age) fracture. Lag screw fixation was the most common method of operative treatment. General complication rates were low, as were re-operation rates for cemented prostheses. Kaplan-Meier implant survivorship of displaced intracapsular fractures treated by reduction and lag screw fixation was 71% (95% confidence interval 56 to 81) at five years. The best form of treatment remains controversial.

Total ankle replacement in patients with significant pre-operative deformity of the hindfoot.

We carried out 123 consecutive total ankle replacements in 111 patients with a mean follow-up of four years (2 to 8). Patients with a hindfoot deformity of up to 10 degrees (group A, 91 ankles) were compared with those with a deformity of 11 degrees to 30 degrees (group B, 32 ankles). There were 18 failures (14.6%), with no significant difference in survival between groups A and B. The clinical outcome as measured by the post-operative American Orthopaedic Foot and Ankle Surgeons score was significantly better in group B (p = 0.036). There was no difference between the groups regarding the post-operative range of movement and complications. Correction of the hindfoot deformity was achieved to within 5 degrees of neutral in 27 ankles (84%) of group B patients. However, gross instability was the most common mode of failure in group B. This was not adequately corrected by reconstruction of the lateral ligament. Total ankle replacement can safely be performed in patients with a hindfoot deformity of up to 30 degrees . The importance of adequate correction of alignment and instability is highlighted.

A revolution in the management of fractures of the distal radius?

The recent development of locking-plate technology has led to a potential revolution in the management of fractures of the distal radius. This review examines the evidence for pursuing anatomical restoration of the distal radius and the possible advantages and pitfalls of using volar locking plates to achieve this goal. The available evidence for adopting volar locking plates is presented and a number of important and, as yet unanswered, questions are highlighted.

Laparoscopic proctocolectomy with restorative ileal-anal pouch.

OBJECTIVE: The aim of the study was to analyse the outcome of restorative proctocolectomy carried out by laparoscopic surgery. METHODS: A prospectively collected electronic database of all colorectal laparoscopic procedures performed between April 2001 and July 2003 has been used to identify surgical outcomes in 14 consecutive patients who have undergone laparoscopic RPC. RESULTS: Fourteen patients (5 male), median BMI 24 kg/m(2) have undergone restorative laparoscopic proctocolectomy over a two year period: 13 (ulcerative colitis, one with cancer) and 1 (FAP). The median operation time was 260 min; time has not decreased with experience. There were no intra-operative surgical complications or deaths. Patient controlled analgesia continued for a median of 36 h. The median time to diet was 48 h and median hospital stay 7 days; three patients required nasogastric aspiration for delayed gastric emptying. Eighteen regional lymph nodes were retrieved local to the carcinoma. There was one anastomotic leak. All covering stomas were closed by 6 months (12 by eight weeks). All 14 patients are fully continent, able to suppress urgency and have a median pouch frequency of 4/24 h. None admit to having problems with potency, orgasm sensation, ejaculation, micturition. One lady reports dysparunia. All are highly satisfied with functional outcome and cosmesis. CONCLUSION: We are encouraged to continue to offer our patients the option of a laparoscopic resection.

Unusual absence of neurologic symptoms in a six-year old girl with ataxia-telangiectasia.

Ataxia-telangiectasia (A-T) is a rare multisystem, neurodegenerative genetic disorder. We present a case of a 6-year-old girl who had a history of frequent respiratory infections and also had ocular and immunological features of this syndrome. The absence of neurological symptoms, which is very unusual for a patient of this age, raised many difficulties in the diagnosis of the disease. It is concluded that a normal neurological assessment must not exclude the diagnosis of A-T and delay the proper interventional measures.

Childhood acute rheumatic fever in Greece: experience of the past 18 years.

UNLABELLED: This study reports on the authors' experience with acute rheumatic fever (ARF) during the years 1980-1997. The objectives were to estimate the incidence of the disease an area of Greece to characterize its epidemiology, to determine the frequency of the antecedent symptoms and to describe its clinical presentation. The medical records of 66 confirmed cases admitted to the First Department of Pediatrics, "Aghia Sophia" Children's Hospital, were reviewed. Two outbreaks occurred during this period. In contrast to the 3-4 cases seen every year, 14 cases were diagnosed during the 6 mo period from October 1989 to March 1990. An additional 10 cases were diagnosed in 1993. Most of the children (76%) were between 8 and 14 y old. The children were predominantly from middle-class families with ready access to medical care. Carditis, evident by auscultation, and arthritis were the dominant major manifestations in 70% and 68% of the cases, respectively. Mild carditis was present in 54% of children with valvular disease. CONCLUSION: ARF exists in the paediatric Greek population with exacerbations and remissions, but the cardiac manifestations appear mild or moderate.