Recognition of advanced level practice against multiprofessional capabilities: Experiences of the first radiography applicants.

INTRODUCTION: Advanced practice is well established in the health professions with multiprofessional capabilities in place in England. To recognise achievement of these capabilities an ePortfolio (supported) route was initiated in 2022. This study aimed to review the demographics and experiences of radiographers applying for recognition in the first year of operation. METHODS: The multi method evaluation consisted of quantitative data analysis of information regarding the first three cohorts of radiographers (n = 40) participating in the NHS England (NHSE) scheme. Interviews with 12 participants was undertaken with thematic analysis of the transcripts. RESULTS: Self-rated scores of expertise were significantly higher by therapeutic radiographers (n = 8) compared to their 32 diagnostic colleagues (t = 5.556; p < 0.01). Radiographers saw the ePortfolio as an opportunity to validate their experience and to evidence parity with other professions. Participants felt the process also enabled critical reflection and gave unseen insight into themselves and their roles. The support of experienced educational supervisors was felt to be vital in this process and for successful completion of portfolio. CONCLUSIONS: Several radiographers have now achieved the necessary standards to achieve NHSE recognition. The evaluation exposed that most radiographers did not have the relevant evidence to hand and the ongoing collection of evidence around capabilities and impact is critical to evidencing advanced practice capabilities. IMPLICATIONS FOR PRACTICE: Radiographers are able to achieve the capabilities expected for multiprofessional practice. Cultural change is required to normalise recording of evidence within practice including case-based discussions, clinical supervision and feedback from colleagues and patients. The support of an experienced educational supervisor aided the critical reflection on practice level.

Clinical Outcome of Exclusively Radiographer-led Delivery of Postoperative Vaginal Vault Brachytherapy for Endometrial Cancer - The Addenbrooke's Experience.

AIMS: Postoperative vaginal vault brachytherapy (VBT) reduces local recurrence in operable endometrial cancer. Radiographer-led delivery of VBT, carried out without image guidance, was implemented at Addenbrooke's in 2010 to maximise skills mix and to improve service delivery. The purpose of this study was to evaluate the safety and effectiveness of this service. MATERIALS AND METHODS: This was a single-centre retrospective study of endometrial cancer patients treated with postoperative high dose rate VBT ± external beam radiotherapy (EBRT) between January 2010 and December 2016. RESULTS: In total, 414 patients were analysed: 307 received adjuvant VBT alone and 107 patients received pelvic EBRT followed by VBT. Thirty-seven per cent of patients receiving VBT alone were high risk according to ESMO-ESGO-ESTRO criteria. After a median follow-up of 59 months (range 2-118), 9/414 (2.2%) patients had isolated vaginal recurrences, 15/414 (3.6%) had locoregional recurrence (vaginal, pelvic node or both), whereas 62/414 (15%) patients had distant recurrence. The 5-year actuarial isolated vaginal recurrence rate was 2.3% (VBT alone 2.1%, EBRT + VBT 3.0%). Grade 3 urinary or bowel toxicity occurred in 2/414 (0.6%) patients treated with EBRT and VBT. None of the patients treated with VBT alone had grade 3 complications. CONCLUSION: Radiographer-led delivery of VBT, without the use of image guidance, is a safe and effective service.

Re-raising muscle flaps: A randomised controlled animal study.

BACKGROUND: In the experience of our centre, 33% of reconstructed compound lower limb injuries will need an orthopaedic revision.1 A flap may be re-raised through numerous methods, and historically, the approach of choice has been based on the principle of protecting the vascular pedicle rather than the inset. Our aim was to determine whether a marginal versus a split approach to re-raising inferred a higher risk of flap necrosis and whether more attention should be paid to protecting the inset of the flap, particularly at the distal portion. METHOD: A pedicled pectoralis profundus muscle flap was raised in 32 Sprague-Dawley rats and transposed to the lateral chest wall. After 21 days, the flaps were randomised into one of four treatment groups according to the surgical approach and whether or not the anatomical vascular pedicle was ligated. Necrosis was assessed 48 h later, both clinically and through the analysis of digital photographs. RESULTS: The rate of necrosis in the marginal group was higher than that in the split group (63% vs 0%, p < 0.001, McNemar). More necrosis occurred in the former when the pedicle was ligated (p < 0.001, Fisher's exact test). Measured necrosis was also higher in the marginal group (18% vs 0%, p = 0.002, Wilcoxon signed-rank test). Twenty-nine percent more flap could be raised using the split approach (p = 0.001, Mann-Whitney U test). CONCLUSIONS: Splitting a muscle flap produces significantly less necrosis than incising the inset, regardless of whether the pedicle is in flow. It also offers wider exposure of structures deep to the flap. These findings provide a detailed model for human trials, which is presented as a proposed management algorithm. It also highlights conditions that must be met for translation to a human population.

Perfusion of muscle flaps independent of the anatomical vascular pedicle: Pedicle autonomy.

INTRODUCTION: Free muscle flaps are being used more commonly for complex lower limb reconstruction. Up to 33% of flaps used to reconstruct lower limb trauma will require an orthopaedic procedure after reconstruction. To date there have been only case reports detailing the variable survival of muscle flaps after actual or simulated pedicle injury and the process and timeframe of neovascularisation remains undefined. We aim to show that perfusion of a muscle flap is possible after injury to its anatomical vascular pedicle. METHODS: Pedicled muscle flaps were raised and transposed to a cutaneous inset on the chest wall in a rodent model. Each flap was subjected to simulated pedicle injury at a variable time. Allocation was by computer randomisation. Flap perfusion was assessed before and after pedicle injury followed 48 h later by sacrifice of the animal and static angiography of the flaps. RESULTS: By the 21st day after inset, all flaps survived simulated pedicle injury. Prior to this, flap survival was significantly lower (p = 0.017, Fisher's Exact Test). Clinical signs at the time of pedicle injury did not predict flap survival. Most new vessels form at the distal part of the inset (p < 0.01, ANOVA). The total number does not change with time (p = 0.82, ANOVA). New vessels anastomose preferentially with skin. The fall in perfusion after pedicle ligation was significant for all groups except the day 35 group (p = 0.53). CONCLUSIONS: Muscle flaps can perfuse after an injury to the anatomical vascular pedicle through neovascularisation at the inset. These new vessels are evident early but may not function adequately to perfuse the flap. Regional variations in neovascularisation suggest that a gradient of ischaemia drives this process. Inset at the cutaneous level is important, which has implications for buried muscle flaps. The correlation between change in flap perfusion after pedicle injury and flap necrosis suggests a role for the former in determining the capacity of a muscle flap to tolerate a pedicle injury and thereby the approach to re-raising it.

The effect of coughing at extubation on oxygenation in the post-anaesthesia care unit.

We prospectively studied 84 patients to investigate whether there is a relationship between coughing during emergence and tracheal extubation, and impaired oxygenation in the post-anaesthesia care unit. Our primary outcome measure was a change in the alveolar-arterial oxygen partial pressure gradient ((A-a)DO2 ) between time A (during general anaesthesia) and time B (1 h after extubation). Patients demonstrated a worsening of oxygenation with mean (SD) (A-a)DO2 increasing from 7.5 (5.2) kPa at time A to 13.9 (4.2) kPa at time B (p < 0.01). An overall linear regression model was not predictive for the observed change (adjusted R(2) = 0.01, p = 0.31) and nor were any of the individual predictors studied, including subjective cough score (p = 0.33), number of coughs (p = 0.95) and duration of coughing (p = 0.39). Despite the abnormal cough that occurs while tracheally intubated, we have been unable to demonstrate that coughing at extubation is associated with impaired oxygenation in the immediate postoperative period.

X-linked retinoschisis: clinical phenotype and RS1 genotype in 86 UK patients.

BACKGROUND: Inactivating mutations of the gene RS1 lead to X-linked retinoschisis, a progressive retinal dystrophy characterised by schisis within the inner layers of the neuroretina. The mutation spectrum is large and the phenotype variable. AIM: To determine whether there is a correlation between mutation type and disease severity. METHODS: We identified the causative mutation in 86 affected patients and examined each of these patients in detail. Different categories of mutation were compared for each phenotypic characteristic. RESULTS: We found a reduction in visual acuity with increasing age and worsening macular pathology in patients over 30 years old (p < or = 0.001), but there was no correlation between mutation type and severity of disease. Furthermore, we found a wide variation in phenotype even within families. CONCLUSIONS: Identifying the causative mutation in patients with X-linked retinoschisis is helpful in confirming diagnosis and in counselling of family members but cannot be used to predict prognosis for an individual patient.

Blue cone monochromatism: a phenotype and genotype assessment with evidence of progressive loss of cone function in older individuals.

AIM: To perform a detailed clinical and psychophysical assessment of the members of three British families affected with blue cone monochromatism (BCM), and to determine the molecular basis of disease in these families. METHODS: Affected and unaffected members of three families with BCM were examined clinically and underwent electrophysiological and detailed psychophysical testing. Blood samples were taken for DNA extraction. The strategy for molecular analysis was to amplify the coding regions of the long wavelength-sensitive (L) and middle wavelength-sensitive (M) cone opsin genes and the upstream locus control region by polymerase chain reaction, and to examine these fragments for mutations by direct sequencing. RESULTS: We have confirmed the reported finding of protan-like D-15 arrangements of patients with BCM. In addition, we have demonstrated that the Mollon-Reffin (MR) Minimal test is a useful colour-discrimination test to aid in the diagnosis of BCM. Affected males were shown to fail the protan and deutan axes, but retained good discrimination on the tritan axis of the MR test, a compelling evidence for residual colour vision in BCM. This residual tritan discrimination was also readily detected with HRR plates. In two families, psychophysical testing demonstrated evidence for progression of disease. In two pedigrees, BCM could be linked to unequal crossovers within the opsin gene array that resulted in a single 5'-L/M-3' hybrid gene, with an inactivating Cys203Arg mutation. The causative mutations were not identified in the third family. CONCLUSIONS: The MR test is a useful method of detecting BCM across a wide range of age groups; residual tritan colour discrimination is clearly demonstrated and allows BCM to be distinguished from rod monochromatism. BCM is usually classified as a stationary cone dysfunction syndrome; however, two of our families show evidence of progression. This is the first report of progression associated with a genotype consisting of a single 5'-L/M-3' hybrid gene carrying an inactivating mutation. We have confirmed that the Cys203Arg inactivating mutation is a common sequence change in blue cone monochromats.

Is optic nerve fibre mis-routing a feature of congenital stationary night blindness?

PURPOSE: To determine whether patients with congenital stationary night blindness (CSNB) have electrophysiological evidence of optic nerve fibre mis-routing similar to that found in patients with ocular albinism (OA). METHOD: We recorded the Pattern Onset VEP using a protocol optimised to detect mis-routing of optic nerve fibres in older children and adults. We tested 20 patients (age 15-69 yrs) with X-linked or autosomal recessive CSNB, 14 patients (age 9-56 yrs) with OA and 13 normally pigmented volunteers (age 21-66 yrs). We measured the amplitude and latency of the CI component at the occipital midline and over left and right occipital hemispheres. We also assessed the computed inter-hemispheric "difference" signal. Subjects with CSNB were classified as having the "complete" or "incomplete" phenotype on the basis of their ERG characteristics. Members of X-linked CSNB pedigrees underwent mutation screening of the NYX and CACNA1F genes. RESULTS: CI was significantly smaller over the ipsilateral hemisphere and more prominent over the contralateral hemisphere in OA patients compared with both controls and CSNB patients. In CSNB patients CI response amplitudes were not significantly different from controls but peak latency was prolonged at all three electrodes compared with controls. The inter-hemispheric "difference" signal was abnormal for the OA group but not for the CSNB group. Contralateral dominance of CI could be identified in the majority of OA patients and the "difference" signal was opposite in polarity for left compared with right eye stimulation in every patient in this group. Only 3 of 20 patients with CSNB showed significant inter-hemispheric asymmetry similar to that seen in the OA patients. All 3 CSNB patients with evidence for optic nerve fibre mis-routing had X-linked pedigrees: 2 had an identified mutation in the NYX gene but no mutation in either the NYX or CACNA1F genes was identified in the third. VEP evidence of optic nerve fibre mis-routing was present in 3 of the 11 subjects with "complete" phenotype and none of the 9 patients with "incomplete" phenotype CSNB. CONCLUSION: Mis-routing of optic nerve fibres does occur in CSNB but we found evidence of it in only 15% of our patients.

Autonomous activity of CaMKII is only transiently increased following the induction of long-term potentiation in the rat hippocampus.

A major role has been postulated for a maintained increase in the autonomous activity of CaMKII in the expression of long-term potentiation (LTP). However, attempts to inhibit the expression of LTP with CaMKII inhibitors have yielded inconsistent results. Here we compare the changes in CaMKII autonomous activity and phosphorylation at Thr286 of alphaCaMKII in rat hippocampal slices using chemical or tetanic stimulation to produce either LTP or short-term potentiation (STP). Tetanus-induced LTP in area CA1 requires CaMKII activation and Thr286 phosphorylation of alphaCaMKII, but we did not observe an increase in autonomous activity. Next we induced LTP by 10 min exposure to 25 mM tetraethyl-ammonium (TEA) or 5 min exposure to 41 mM potassium (K) after pretreatment with calyculin A. Exposure to K alone produced STP. These protocols allowed us to monitor temporal changes in autonomous activity during and after exposure to the potentiating chemical stimulus. In chemically induced LTP, autonomous activity was maximally increased within 30 s whereas this increase was significantly delayed in STP. However, in both LTP and STP the two-fold increase in autonomous activity measured immediately after stimulation was short-lived, returning to baseline within 2-5 min after re-exposure to normal ACSF. In LTP, but not in STP, the phosphorylation of alphaCaMKII at Thr286 persisted for at least 60 min after stimulation. These results confirm that LTP is associated with a maintained increase in autophosphorylation at Thr286 but indicate that a persistent increase in the autonomous activity of CaMKII is not required for the expression of LTP.

Oligocone trichromacy: a rare and unusual cone dysfunction syndrome.

AIM: To describe the phenotype of a case series of six patients with oligocone trichromacy. METHODS: The six affected individuals underwent an ophthalmological examination, electrophysiological testing and detailed psychophysical assessment. RESULTS: All six affected patients had a history of moderately reduced visual acuity (6/12 to 6/24) from infancy, not improved by full spectacle correction. They complained of mild photophobia and they were not aware of any colour vision deficiency. They had no nystagmus and fundi were normal. Electrophysiological testing revealed either absent/profoundly reduced cone flicker responses or preserved but delayed and mildly reduced flicker responses. Colour vision was found to be within normal limits, but some patients showed mildly elevated discrimination thresholds along all axes. CONCLUSION: The largest case series to date of patients with oligocone trichromacy is presented. The electrophysiological findings suggest that there may be more than one disease mechanism. The mode of inheritance is likely to be autosomal recessive, and while previous reports have suggested that this disorder is stationary, in one of these families there is clinical evidence of progression.

Full-field ERG responses recorded with skin electrodes in paediatric patients with retinal dystrophy.

PURPOSE: Assess ERG responses recorded with skin electrodes in children with retinal dystrophies. METHOD: ERG responses were recorded using skin electrodes in 17 healthy children and 43 paediatric patients with retinal dystrophy. Subjects were aged 4-14 years. ERG responses were recorded to full-field stimuli similar to those recommended in the ISCEV standard. The type of retinal dystrophy was classified on the basis of standard clinical criteria and the ERG responses were compared with those of the age-matched controls. RESULTS: ERG responses were abnormal in every patient. The specific type of ERG abnormality was also consistent with the clinical findings in the majority of patients. Rod responses were abnormal in every patient with a rod-cone dystrophy and cone responses were also abnormal in the majority of patients. Those patients with cone dystrophy or rod monochromatism had normal or near normal rod responses but sub-normal or absent cone responses. Patients with CSNB or XLRS had a sub-normal b-wave but normal amplitude a-wave. CONCLUSION: ERGs can be recorded successfully with skin electrodes in paediatric patientsand responses can aid the diagnosis of the type of retinal dystrophy.

A role for dendritic protein synthesis in hippocampal late LTP.

The late temporal component of long-term potentiation (LTP), a putative neural mechanism for information storage in the brain, is protein synthesis-dependent, but the site of obligatory protein synthesis is not known. Here we show that when the protein synthesis inhibitor emetine is applied locally to the apical dendritic field of CA1 pyramidal cells in the murine hippocampus, late LTP is impaired at apical but not at basal dendrites, and conversely when emetine is applied locally to basal dendrites, late LTP is impaired only at basal dendrites. Thus, local protein synthesis modulates the expression of tetanically induced late LTP at Schaffer-commissural synapses on CA1 pyramidal cells.

Genotype-phenotype correlation in British families with X linked congenital stationary night blindness.

AIM: To correlate the phenotype of X linked congenital stationary night blindness (CSNBX) with genotype. METHODS: 11 CSNB families were diagnosed with the X linked form of the disease by clinical evaluation and mutation detection in either the NYX or CACNA1F gene. Phenotype of the CSNBX patients was defined by clinical examination, psychophysical, and standardised electrophysiological testing. RESULTS: Comprehensive mutation screening identified NYX gene mutations in eight families and CACNA1F gene mutations in three families. Electrophysiological and psychophysical evidence of a functioning but impaired rod system was present in subjects from each genotype group, although the responses tended to be more severely affected in subjects with NYX gene mutations. Scotopic oscillatory potentials were absent in all subjects with NYX gene mutations while subnormal OFF responses were specific to subjects with CACNA1F gene mutations. CONCLUSIONS: NYX gene mutations were a more frequent cause of CSNBX than CACNA1F gene mutations in the 11 British families studied. As evidence of a functioning rod system was identified in the majority of subjects tested, the clinical phenotypes "complete" and "incomplete" do not correlate with genotype. Instead, electrophysiological indicators of inner retinal function, specifically the characteristics of scotopic oscillatory potentials, 30 Hz flicker and the OFF response, may prove more discriminatory.

A clinical and molecular genetic study of a rare dominantly inherited syndrome (MRCS) comprising of microcornea, rod-cone dystrophy, cataract, and posterior staphyloma.

AIM: To phenotype and genetically map the disease locus in a family presenting with autosomal dominant microcornea, rod-cone dystrophy, cataract, and posterior staphyloma. METHODS: Six affected and three unaffected members of the pedigree were examined. All individuals provided a history and underwent a full clinical examination with A-scan and B-scan ultrasonography and electrophysiological testing where appropriate. PCR based microsatellite marker genotyping using a positional candidate gene approach was then performed on DNA samples extracted from venous blood provided by each subject. RESULTS: The disorder is inherited as an autosomal dominant trait with variable expressivity and has a complex phenotype. Affected individuals had bilateral microcornea, pulverulent-like lens opacities, a rod-cone dystrophy and posterior staphyloma (MRCS). Using a positional candidate gene approach, the authors have evidence suggestive of linkage of this disorder to a region on 11q13 within the nanophthalmos 1 (NNO1) genetic interval. The small family size militates against achieving a LOD score of 3, but the haplotype data and the position of the putative MRCS locus within a known nanophthalmos locus are suggestive of linkage. A candidate gene within this region (ROM1) was screened and no mutations were found in affected members of the family. CONCLUSION: This rare developmental disorder has some phenotypic similarities to nanophthalmos and possibly maps to a locus within the genetic interval encompassing the NNO1 locus. Screening of candidate genes within this region continues.

Progressive nodular histiocytosis in a child with a hypothalamic tumor.

We report a 13-year-old girl with multiple cutaneous histiocytic lesions, precocious puberty, growth hormone deficiency and a hypothalamic tumour. We conclude that she has progressive nodular histiocytosis, but this case illustrates the difficulty in differentiating the type II histiocytoses.

Idiopathic central pontine myelinolysis in childhood.

Central pontine myelinolysis (CPM) is rare in childhood with only a few cases reported in world literature. We report a 7-year-old male who presented with acute ataxia, swallowing difficulties, dysarthria, and radiological features consistent with the disorder. He improved remarkably with oral prednisolone therapy and was almost back to normal by 2 weeks. A review of the literature is also included.

Imaging the upper airways.

Radiological evaluation of the airway has been used as a screening tool and an adjunct to endoscopy for many years. It provides non-invasive data on the structure of the airway, often avoiding the risk of general anaesthesia. Standard radiographs provide some information on the intricate anatomy of the paediatric airway aided by fluoroscopy. More recently, CT and MRI are proving to have a valuable role and are approaching near endoscopic detail of airway anatomy. The purpose of this article is to highlight areas where radiology can aid in the evaluation of the airway of infants and children.