PROMs in total knee replacement: analysis of negative outcomes.

INTRODUCTION: NHS England uses the Oxford Knee Score (OKS) as part of patient-reported outcome measures (PROMs) to evaluate 'health gains' following total knee replacement. Policy makers use this to guide healthcare funding and resource allocation. Our study aims to undertake a qualitative and quantitative analysis of OKS among patients who experienced a negative outcome after a total knee replacement at our centre. MATERIALS AND METHODS: Between April 2017-March 2018, 19 of 189 (10%) patients had a worsened OKS at our centre. We retrospectively and prospectively reviewed 14 of these patients. Structured telephone interviews with a repeat OKS were carried out in September 2019 (18-29 months post-operation). RESULTS: Eight patients were female and the total age range was 57-95, mean average 75.6 (SD 9.9 years). Of 48 (higher scores meaning better outcomes), the average preoperative OKS was 24.2 and the average postoperative OKS at 6 months was 19.4 (decrease of 20%). The average postoperative OKS at 18-29 months was 35.6 (an increase of 83.5% from 6 months). DISCUSSION: The OKS was developed and validated over 20 years ago in Oxford. In our study, four patients asked for clarification of questions 4, 6 and 10 owing to ambiguous language. All 14 patients who had negative OKS outcomes had positive outcomes when retested after 18 months, depicting 'health gains' not conveyed in PROMs analysis. CONCLUSION: The OKS needs to be revalidated on current patient groups for accurate and reliable data. Further prospective studies should be undertaken on larger cohorts to understand the recovery course and whether PROMs should be carried out later.

The dissemination of the French School of Psychiatry and its impact in the world.

The French School of Psychiatry has characteristics which are proper to it, and it conveys many notions related to health care, in addition to the accompaniment and the comprehension of mentally ill people and mental illness. These notions are specific to the French culture. Thus, famous French psychiatrists have described many syndromes and discovered the first neuroleptic, chlorpromazine. Among these psychiatrists: Pinel, Esquirol, Janet, Ey, Delay and Deniker. Furthermore, the first World Psychiatry Congress was held in Paris in 1950. It was a major congress for many specialists from all the world have participated and strongly influenced the future of psychiatry in the world. We will be describing the French School of Psychiatry's impact in the world (South America) and mainly in the French-speaking world (Romania, Africa, Lebanon and Quebec). We will also be discussing the tools, associations and publications which participate in the dissemination of this school of thought's knowledge.

Isolated language impairment as the primary presentation of sporadic Creutzfeldt Jakob Disease.

OBJECTIVES: Sporadic Creutzfeldt Jakob Disease (sCJD) is a neurodegenerative disorder that typically presents as a rapidly progressive encephalopathy associated with various neurological features, culminating in akinetic mutism and death. Atypical cases, presenting with an isolated focal may cause diagnostic confusion. We described a series of patients with sCJD presenting with isolated language impairment. MATERIALS & METHODS: We report a patient with sCJD referred to the NCJDRSU, who presented with isolated language impairment and subsequently identified all cases of sporadic CJD on the NCJDRSU database (covering the years 1990-2012) with an isolated language impairment presentation. RESULTS: Nineteen patients (11 females) with sCJD (1.19% of all patients) had an isolated language disorder of at least 2 weeks duration as the first neurological symptom pattern. Mean age at onset was 68.28 years. No specific pattern of language affection was seen in these patients. Further progression usually affected more than one neurological domain, with all patients eventually developing cognitive decline and myoclonic jerks. The median duration of illness was 4 months. CSF 14.3.3 was positive and S100b level was elevated in all patients in whom it was performed. EEG and MRI showed typical features of sCJD in six patients each. Most patients showed MM genotype of PRNP codon 129. CONCLUSION: This study highlights the fact that isolated aphasia can be the first neurological symptom approximately in 1% of patients with sCJD. The diagnosis is usually made with appearance of other clinical features and investigation results, but in a small minority, these may not be apparent for relatively long periods.

Risk of rabies exposure among travellers.

BACKGROUND: In recent years, requests for rabies immunoglobulin have increased at Amsterdam's Academic Medical Center's travel clinic. Travellers who received rabies pre-exposure prophylaxis (PrEP) before travel departure have immunological memory that can quickly be activated by timely booster vaccinations after possible exposure to rabies. PrEP alleviates the need for costly and scarcely available rabies immunoglobulin in case of exposure. This study describes which travellers are at risk of rabies exposure and would benefit from PrEP. The secondary aim was to specify which factors influence decision-making on taking PrEP. METHODS: We reviewed electronic patient files of travellers attending our clinic for rabies post-exposure prophylaxis between January 2009 and February 2014. Demographic and travel characteristics were compared with a sample of patients who were seen for pre-travel advice at our clinic. To assess which factors had influenced the decision to take PrEP, a questionnaire survey was conducted. RESULTS: A total of 161 travellers experienced animal-associated injury. Compared with travellers from the pre-travel database, more people travelled to Southeast Asia (49.5% vs. 30.9%, p = 0.035) for comparable time periods (median 21 vs. 21 days, p = 0.083). Transcutaneous injuries (type III) were common (73.9%), most often inflicted by dogs (45%). Only ten travellers (6.2%) had received PrEP. Barriers for PrEP were high costs and a short time interval between consultation and travel departure. CONCLUSION: Travellers t o Southeast Asia should particularly be informed about rabies and the possibility of PrEP. Long-term travel was not associated with a higher risk of rabies exposure.

Effect of Rare-Earth Gd Incorporation on the Characteristics of ZnO Thin Film.

The doping of oxide-based semiconductors with rare-earth ions may serve as an alternative to transition metal doping with the possibility of room-temperature ferromagnetism. Here, highly transparent thin films of ZnO doped with a rare-earth element, Gd, were prepared via a sol-gel-derived precursor. A series of Gd-doped ZnO films with different Gd concentrations (0 to 8 at.%) were deposited onto glass substrates. XRD analysis revealed that the FWHM of the diffraction peaks increased with the increase of Gd composition which may indicate that the Gd incorporation decreased the crystallinity. However no new peaks associated with the formation of secondary phases such as GdO and Gd metal clusters were detected. The MFM images were seen to be more pronounced with the incorporation of Gd ions where we could clearly observe the magnetic signals at room temperature especially for the samples with higher Gd doping of 4 and 8 at.% Gd. Evidently, the magnetic domain size exhibited a significant increase with the increase of Gd composition in contrast to the undoped sample.

A novel mutation in the MFSD8 gene in late infantile neuronal ceroid lipofuscinosis.

Neuronal ceroid lipofuscinoses (NCL) are lysosomal storage disorders and constitute the most common group of progressive neurodegenerative diseases in childhood. Most NCLs are inherited in a recessive manner and are clinically characterised by a variable age at onset, epileptic seizures, psychomotor decline, visual impairment and premature death. To date, eight causative genes have been identified to underlie various clinical forms of NCL. We performed a genome-wide linkage analysis followed by sequencing the recently described NCL gene MFSD8 in three affected and three unaffected members of a consanguineous Egyptian family with an autosomal recessively inherited progressive neurodegenerative disorder. The clinical picture of the patients was compatible with a late infantile NCL (LINCL); however, impairment of the visual system was not a cardinal symptom in the respective family. By linkage analysis, we identified two putative loci on chromosome 1p36.11-p35.1 and 4q28.1-q28.2. The latter locus (4q28.1-q28.2) contained the MFSD8 gene, comprising a novel homozygous missense mutation in exon 5 (c.362a>g /p.Tyr121Cys), which segregated with the disease in the three affected sibs. We describe a novel mutation in the previously identified MFSD8 gene in a family with a common phenotype of LINCL, but no clinical report of vision loss. Our results enlarge the mutational and perhaps the nosological spectrum of one of the recently identified subtypes of NCL, called CLN7.

Use of a double rhomboid transposition flap in the treatment of extensive complex pilonidal sinus disease.

OBJECTIVE: Rhomboid transposition flaps are safe and successful in treating recurrent, complex pilonidal disease. There are little data on treatment of very large (> 12 cm) pilonidal sinuses. We describe a new technique using two simultaneous rhomboid flaps to achieve primary closure following extensive wide-excision. METHOD: With antibiotic prophylaxis, the sinus is excised en-bloc as a parallelogram (two adjacent rhomboids). Two rhomboid fasciocutaneous flaps are transposed to close this defect over suction-drains. Sutures are removed after 14 days. RESULTS: Eight patients (seven males) were treated with this technique (median age 26 years; range 22-35 years). All had very extensive and recurrent disease, having had multiple previous procedures. Median drain-duration was 2 days (range 1-5 days) and postoperative stay was 2 days (range 0-5 days). One complication arose: a wound infection with partial dehiscence which healed with conservative treatment. No recurrence or further complication arose in a 33-month follow-up (range 8-41 months). CONCLUSION: This is the first description of the use of two simultaneous rhomboid flaps for very large recurrent pilonidal disease. It is a relatively simple and safe alternative to major plastic reconstruction which is often resorted to in such large-scale disease.

Effects of dexamethasone on G protein levels and adenylyl cyclase activity in rat vascular smooth muscle cells.

Dexamethasone administration in vitro has been shown to increase adenylyl cyclase activity in vascular smooth muscle cells (VSMC) from renal arteries and in non-vascular cell lines. To investigate whether G proteins are involved in this response, cultured VSMC from mesenteric arteries of Sprague-Dawley rats were incubated in the presence and absence of 10 nM dexamethasone for 24 and 48 h. Basal and stimulated adenylyl cyclase activities were increased by approximately 50% after treatment with dexamethasone. The changes were neither specifically associated with ligands which stimulate adenylyl cyclase catalytic unit via Gs (isoproterenol and prostaglandin E1) nor with guanylylimidodiphosphate (0.1 nM), which inhibits the catalytic unit via Gi. This suggests that dexamethasone enhances adenylyl cyclase activity through changes at the level of the catalytic unit, rather than through the G proteins which modulate its activity. No differences were seen in immunoblotting studies of the levels of Gi alpha 2, Gs alpha, Gi alpha 3 and beta subunits. Similarly, dexamethasone had no effect on the expression of mRNA for Gi alpha 2 and Gs alpha. The results indicate that glucocorticoid-induced increases of adenylyl cyclase activity are due to changes at the level of the adenylyl cyclase catalytic unit rather than alteration of the levels or turnover of Gs alpha, Gi alpha 2, Gi alpha 3 and beta subunits in the membranes of VSMC.

[A new scale of quantitative and qualitative evaluation of anxiety: the Ferrerri Anxiety Rating Diagram (FARD)]. / Une nouvelle échelle d'évaluation quantitative et qualitative de l'anxiété: le F.A.R.D. (Ferreri anxiety rating diagram).

This new instrument to evaluate anxiety, the F.A.R.D. (Ferreri anxiety rating diagram - 1987) has been conceived to appreciate the repercussions of anxiety on the activity of the subject. It realizes a special image of anxiety. It has been studied, by both psychiatrists and general practitioners in 320 patients included on generalised anxiety disorders criteria (following DMS III). The validation has been realised in a study of prazepam for 81 patients. The F.A.R.D. is based upon 12 items. The factor analysis has retained 4 factors explaining 66% of the whole variance. These 4 factors composed by 3 items each are: relational, somatic, vigilance, cognitive. This new instrument, the F.A.R.D.: evaluates the intensity of anxiety, precision, the shape of anxiety, specifies sub-groups of anxiety, allows a following of evolution, appreciates the pharmaco-clinical profile of the anxiolytic drug prescribed.

[Construction and preliminary validation of an anxiety scale, the FARD (Ferreri anxiety rating diagram)]. / Construction et validation préliminaire d'une échelle d'anxiété, le FARD (Ferreri-Anxiety Rating Diagram).

The complexity of the anxious disease does not facilitate the clinical approach. That the reason why the authors have developed a rating scale based on a descriptive symptomatology to find out a diagnosis, to rate the severity of illness and to facilitate the pharmaceutical prescription. To develop a skill needed by general practitioners as well as specialists, Prof. M. Ferreri worked with pools of medical doctors to isolate the more frequent items in the anxious symptomatology. So they pointed out a first group of 14 items. A first statistical approach was to compare the new scale with the well-known Hamilton anxiety scale by the way of a sample of 81 ratings. After a first factor analysis, a sub-group of 12 items were definitely retained. These items were split in 4 factors which permitted to present the factorial results in diagram [Acta psychiat. belg., 87, 704-713 (1987)].