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Lamotrigine adjunctive therapy among children and adolescents with primary generalized tonic-clonic seizures.

CONTEXT AND OBJECTIVE: Primary generalized tonic-clonic seizures are relatively more common among children than among adults. Primary generalized tonic-clonic seizures are associated with increased risk of injury and death. Therefore, effective control of primary generalized tonic-clonic seizures is necessary to reduce epilepsy-related morbidity and mortality. Lamotrigine has demonstrated efficacy from published randomized clinical trials for childhood partial seizures, absence seizures, and for the generalized seizures associated with Lennox-Gastaut syndrome. A randomized, blinded, placebo-controlled study was conducted to assess the efficacy and tolerability of adjunctive therapy with lamotrigine in the treatment of primary generalized tonic-clonic seizures among patients > or = 2 years of age; we report the data from children and adolescents 2 to 20 years of age from this randomized clinical trial. This is the first published analysis of data from a randomized, double-blind, controlled clinical trial of primary generalized tonic-clonic seizures focusing on children and adolescents. PATIENTS AND METHODS: We randomly assigned (1:1) 117 patients, aged 2 to 55 years, with primary generalized tonic-clonic seizures inadequately controlled on 1 to 2 current antiepileptic drugs and with evidence of primary generalized tonic-clonic seizures on electroencephalogram and no historical or electroencephalogram evidence of partial seizures to either lamotrigine or placebo in a double-blind parallel group clinical trial from 2001 through 2004. We analyzed the subgroup of children and adolescents, aged 2 to 20 years (n = 45), from this randomized clinical trial. Patients having > or = 3 primary generalized tonic-clonic seizures over an 8-week baseline were randomly assigned (1:1) to receive either lamotrigine or placebo. The treatment period consisted of an escalation phase (12 weeks for patients 2-12 years; 7 weeks for patients > 12 years) and a maintenance phase (12 weeks). The study had 4 phases: screening phase, baseline phase, escalation phase, and maintenance phase. During the screening phase, baseline medical examinations and seizure type and seizure frequency assessments were performed. During the 8-week baseline phase, the number and dosages of concomitant antiepileptic drugs were maintained while seizure frequency was assessed. The assessment of primary generalized tonic-clonic seizure frequency was determined during the 8-week baseline phase. Patients eligible for random assignment experienced > or =3 primary generalized tonic-clonic seizures during the baseline phase and > or = 1 primary generalized tonic-clonic seizure in the 8 weeks before the baseline phase. Lamotrigine was introduced and titrated using a schedule based on the patients' age and concurrent antiepileptic drug regimen. During the escalation phase, the number and doses of concomitant antiepileptic drugs were not changed. The escalation phase was followed by a 12-week maintenance phase during which time the lamotrigine dose was maintained at a specific dose defined by the patients' age and concomitant antiepileptic drugs, whereas the doses of concurrent antiepileptic drugs were maintained at a constant dose. Concurrent antiepileptic drugs could not be discontinued or added during the maintenance phase. The primary efficacy end point measure was the median reduction in the frequency of primary generalized tonic-clonic seizures from baseline; seizure counts were recorded prospectively in standardized daily seizure diaries. Other efficacy end point data for analysis were as follows: the median seizure counts, the median percentage change from the baseline phase in average monthly seizure frequency for other generalized seizure types, and the percentage of patients with a reduction of > or = 25%, > or = 50%, > or = 75%, or 100% in frequencies of primary generalized tonic-clonic seizures and all generalized seizures during the escalation phase and/or maintenance phase relative to the baseline phase. Accurate counts of absence seizure frequency require electroencephalogram-video monitoring; absence seizure frequency was not an outcome measure for this analysis. RESULTS: Forty-five (21 lamotrigine and 24 placebo) patients 2 to 19 years of age were randomly assigned and received study drug. Eight patients (3 lamotrigine and 5 placebo) had a combination of clinical (myoclonus and/or absence seizures) and electroencephalogram findings that were consistent with juvenile myoclonic epilepsy. Among the 45 children randomly assigned, 74% had generalized spike, polyspike, and/or generalized spike and wave discharges on routine electroencephalogram recordings; the remaining 26% of children had no electroencephalogram findings suggestive of partial epilepsy and a clear history consistent with primary generalized tonic-clonic seizures. Electroencephalogram findings were not significantly different between the lamotrigine and the placebo treatment groups. The median percentage decrease from baseline in primary generalized tonic-clonic seizures during the entire treatment period was 77% in the lamotrigine group and 40% in the placebo group (P = .044). The median primary generalized tonic-clonic seizure counts per month were 0.7 in the lamotrigine group and 3.6 in the placebo group during escalation (P = .008), 0.3 in the lamotrigine group and 2.0 in the placebo group during maintenance (P = .005), and 0.4 in the lamotrigine group and 2.5 in the placebo group during the entire treatment period (P = .007). Trends were noted during escalation and maintenance with a median percentage decrease in primary generalized tonic-clonic seizures during escalation of 72% in the lamotrigine group and 30% in the placebo group (P = .059), and 83% in the lamotrigine group and 42% in the placebo group during maintenance (P = .058). During the maintenance phase, 48% of lamotrigine patients were seizure free compared with 17% treated with placebo (P = .051). One patient from each treatment group discontinued from the study because of an adverse event; 1 patient who received lamotrigine experienced "disorientation"; and 1 patient who received placebo had a convulsion with apnea. No rashes occurred among patients taking lamotrigine or placebo. No patient experienced worsening of the intensity or frequency of myoclonus. CONCLUSIONS: Adjunctive lamotrigine therapy seems effective in controlling primary generalized tonic-clonic seizures among patients 2 to 20 years of age.

Adolescent↗

Contribution of general practitioners to hospital intrapartum care in maternity units in England and Wales in 1988.

OBJECTIVE: To ascertain the contribution of general practitioners to hospital intrapartum care in 1988. DESIGN: Confidential postal questionnaire. SETTING: All maternity units in England and Wales. MAIN OUTCOME MEASURES: Type of general practitioner unit (if any); number of bookings, transfers, and deliveries by general practitioners; participation of general practitioners in the policy and audit of the unit. RESULTS: 277 (93%) of 297 units replied. Of 611,644 deliveries, 36,043 (5.9%) were under general practitioner care. In all, 228 units permitted general practitioners to book women under their sole care: 65 were isolated, 29 alongside, and 134 integrated general practitioner units. Alongside units had significantly more bookings (568), antenatal transfers (69), intrapartum transfers (86), and deliveries (387) compared with isolated units (185, 18, 16, and 125, respectively) and integrated units (106, 18, 18, and 52) (p less than 0.001 for all differences). The percentage of women booked by general practitioners transferred either before or during labour was independent of both the type of unit and the number of general practitioner bookings. General practitioners in consultant units were significantly less likely to attend meetings reviewing perinatal mortality (p less than 0.01), and these units were less likely to have any form of general practitioner-consultant liaison committee (p less than 0.001) compared with general practitioner units as a whole. Compared with those in isolated and alongside units, general practitioners in integrated units were less likely to have taken part in deciding the unit's booking policy (p less than 0.01) and consultants more likely to be the final determinant of whether a general practitioner should be permitted to practice within the unit (p less than 0.001). CONCLUSIONS: Both the number of deliveries booked by general practitioners and the number of isolated general practitioner units have fallen. Transfer from general practitioner to consultant care was independent of the general practitioner unit's caseload or the type of unit. General practitioner units differ from consultant units in important ways and differ among themselves as well. Except in remote areas, alongside units may be the ideal type of unit to encourage general practitioners to continue to provide intrapartum care.

Delivery Rooms↗

GP recruitment and retention: a qualitative analysis of doctors' comments about training for and working in general practice.

BACKGROUND AND AIMS: General practice in the UK is experiencing difficulty with medical staff recruitment and retention, with reduced numbers choosing careers in general practice or entering principalships, and increases in less-than-full-time working, career breaks, early retirement and locum employment. Information is scarce about the reasons for these changes and factors that could increase recruitment and retention. The UK Medical Careers Research Group (UKMCRG) regularly surveys cohorts of UK medical graduates to determine their career choices and progression. We also invite written comments from respondents about their careers and the factors that influence them. Most respondents report high levels of job satisfaction. A noteworthy minority, however, make critical comments about general practice. Although their views may not represent those of all general practitioners (GPs), they nonetheless indicate a range of concerns that deserve to be understood. This paper reports on respondents' comments about general practice. ANALYSIS OF DOCTORS' COMMENTS: Training Greater exposure to general practice at undergraduate level could help to promote general practice careers and better inform career decisions. Postgraduate general practice training in hospital-based posts was seen as poor quality, irrelevant and run as if it were of secondary importance to service commitments. In contrast, general practice-based postgraduate training was widely praised for good formal teaching that met educational needs. The quality of vocational training was dependent upon the skills and enthusiasm of individual trainers. Recruitment problems Perceived deterrents to choosing general practice were its portrayal, by some hospital-based teachers, as a second class career compared to hospital medicine, and a perception of low morale amongst current GPs. The choice of a career in general practice was commonly made for lifestyle reasons rather than professional aspirations. Some GPs had encountered difficulties in obtaining posts in general practice suited to their needs, while others perceived discrimination. Newly qualified GPs often sought work as non-principals because they felt too inexperienced for partnership or because their domestic situation prevented them from settling in a particular area. Changes to general practice The 1990 National Health Service (NHS) reforms were largely viewed unfavourably, partly because they had led to a substantial increase in GPs' workloads that was compounded by growing public expectations, and partly because the two-tier system of fund-holding was considered unfair. Fund-holding and, more recently, GP commissioning threatened the GP's role as patient advocate by shifting the responsibility for rationing of health care from government to GPs. Some concerns were also expressed about the introduction of primary care groups (PCGs) and trusts (PCTs). Together, increased workload and the continual process of change had, for some, resulted in work-related stress, low morale, reduced job satisfaction and quality of life. These problems had been partially alleviated by the formation of GP co-operatives. Retention difficulties Loss of GPs' time from the NHS workforce occurs in four ways: reduced working hours, temporary career breaks, leaving the NHS to work elsewhere and early retirement. Child rearing and a desire to pursue interests outside medicine were cited as reasons for seeking shorter working hours or career breaks. A desire to reduce pressure of work was a common reason for seeking shorter working hours, taking career breaks, early retirement or leaving NHS general practice. Other reasons for leaving NHS general practice, temporarily or permanently, were difficulty in finding a GP post suited to individual needs and a desire to work abroad. CONCLUSIONS: A cultural change amongst medical educationalists is needed to promote general practice as a career choice that is equally attractive as hospital practice. The introduction of Pre-Registration House Officer (PRHO) placements in general practice and improved flexibility of GP vocational training schemes, together with plans to improve the quality of Senior House Officer (SHO) training in the future, are welcome developments and should address some of the concerns about poor quality GP training raised by our respondents. The reluctance of newly qualified GPs to enter principalships, and the increasing demand from experienced GPs for less-than-full-time work, indicates a need for a greater variety of contractual arrangements to reflect doctors' desires for more flexible patterns of working in general practice.

Attitude of Health Personnel↗

Calculating the workforce in general surgery.

OBJECTIVE: To provide a reasonable estimate of the patient care and resident physician workforce practicing general surgery in 1994. DESIGN: Data regarding general surgical residents and practicing general surgeons were obtained from four sources and compared with previously published numbers. DATA SOURCES: Information was derived from the American College of Surgeons' Longitudinal Study of Surgical Residents: 1992-1993; the American Medical Association's Physician Characteristics and Distribution in the United States, 1994 Edition; the American Board of Medical Specialties' database on general surgeons; and the American Board of Surgery recertification data from the files of diplomates since 1968. Each of these sources was analyzed separately to derive a count of graduates from general surgery residency programs and an estimate of fully trained general surgeons engaged in patient care activities. RESULTS: We found that approximately 600 graduates of general surgery residency programs enter the practice of general surgery each year, and we found a close correlation between maximum and minimum estimates of the number of fully trained general surgeons engaged in active patient care, certified general surgeons who are not retired, and currently certified general surgeons. This number (17,289 to 23,502) is approximately half that commonly used in calculations of the general surgery workforce (38,239). The larger number includes surgeons with subspecialty training beyond general surgery, surgical residents, and surgeons not engaged in patient care. CONCLUSIONS: Estimations of the workforce in general surgery and predictions of future needs for general surgeons must be derived from the appropriate number of general surgery residents and practicing general surgeons.

Career Choice↗

Evaluation of endoscopic retrograde cholangiopancreatography under conscious sedation and general anesthesia.

BACKGROUND AND STUDY AIMS: In adults, general anesthesia is usually only provided during endoscopic retrograde cholangiopancreatography (ERCP) when prior attempts using conscious sedation have failed. It was hypothesized that in our hospital, other factors might be associated with general anesthesia for ERCP. The aim of this study was therefore to assess the indications for ERCP under general anesthesia, and to evaluate the underlying diseases, type, and efficacy of ERCP under general anesthesia in comparison with conscious sedation. PATIENTS AND METHODS: We retrospectively analyzed 1,056 ERCPs that had been carried out with the patients under general anesthesia or conscious sedation. The indications for general anesthesia were recorded, and the underlying diseases, the type and success of the interventions, and the causes of premature ERCP termination in both groups were assessed. RESULTS: Eighteen percent of the ERCPs were performed under general anesthesia and 82% under conscious sedation. The indications for general anesthesia were related to the type of procedure planned (46%), premature termination of ERCP under conscious sedation (28%), and other reasons. Patients with primary sclerosing cholangitis and liver transplant recipients received general anesthesia more frequently (general anesthesia vs. conscious sedation, 36% vs. 16%, P < 0.0001 and 22% vs. 13%, P = 0.003). Conscious sedation was provided more frequently in patients with neoplasms and cholelithiasis (21% vs. 12%, P = 0.004 and 13% vs. 3%, P < 0.001). Painful dilations were performed more frequently with the patients under general anesthesia (60% vs. 19%, P < 0.001), whereas major papillotomies were preferably performed with conscious sedation (34% vs. 21%, P = 0.006). More interventions per ERCP were performed with the patient under general anesthesia compared to conscious sedation (P < 0.001), during the same time (51 +/- 28 min vs. 52 +/- 26 min, P = 0.39). With conscious sedation, the ERCP failure rate was double that with general anesthesia (7% vs. 14%, P = 0.012), mainly due to inadequate conscious sedation (61%). CONCLUSIONS: The frequent use of general anesthesia for ERCP at our institution is related to the underlying diseases, which are frequently treated with complex and painful ERCP procedures. The efficacy of ERCP with general anesthesia supports a continued preference for general anesthesia rather than conscious sedation when complex and painful interventional ERCP procedures are planned.

Anesthesia, General↗

The clinical and educational experiences of pre-registration house officers in general practice.

AIMS: To describe aspects of the clinical experience and educational supervision gained by pre-registration house officers (PRHOs) in general practice, and to relate these to the current General Medical Council (GMC) aims for general clinical training in general practice. DESIGN: Qualitative evaluation, part of which involved semistructured interviews with 12 PRHOs who were experiencing a general practice rotation. Interviews were conducted at the beginning and the end of the pre-registration year, and following return to hospital work after completion of the general practice placement. SETTINGS: Three teaching hospitals, two district general hospitals and six general practices in south-east England. PARTICIPANTS: 12 PRHOs who were involved in rotations incorporating a general practice placement. RESULTS: To varying degrees, the GMC aims for training in general practice were met for all the participants. All PRHOs recognized the value of the clinical experience and educational supervision they received in general practice. They particularly valued aspects such as having an individual training programme based on their own needs, and the interlinking of theory and practice, which aided learning. Most felt that having responsibility for their own patients acted as an important incentive for learning, and in general, PRHOs appreciated having the time to learn which general practice allowed. CONCLUSIONS: For the majority of PRHOs, the time spent in general practice was seen as a positive clinical and educational experience. In a variety of ways, the general practice placement encouraged PRHOs to develop the self-directed learning skills seen as essential to the lifelong learning advocated by the GMC. A number of recommendations are made to help improve the integration of the hospital and general practice components of these rotations.

Clinical Competence↗

Recruitment, retention, and time commitment change of general practitioners in England and Wales, 1990-4: a retrospective study.

OBJECTIVES: To describe the recruitment and retention of general practitioners and changes in their time commitment from 1 October 1990 to 1 October 1994. DESIGN: Retrospective analysis of yearly data. SETTING: England and Wales. SUBJECTS: General practitioners in unrestricted practice. MAIN OUTCOME MEASURES: Numbers of general practitioners moving into and out of general practice; proportion of general practitioners practising less than full time; proportion of general practitioners having unchanged time commitment over the study period; and proportion of general practitioners leaving general practice in 1991 who were subsequently practising in 1994. RESULTS: Numbers of general practitioners entering general practice (1565 in 1990, 1400 in 1994) fell over the study period as did the numbers leaving general practice (1488 in 1990, 1115 in 1994). The net effect was an increase in both the total and full time equivalent general practitioners practising from 1 October 1990 (26,757 full time equivalents) to 1 October 1994 (27,063 full time equivalents). Numbers of general practitioners practising full time were decreasing whereas part time practice was increasing; women were more likely to practise part time. 35.5% (43/121) of women practising full time and 17.8% (24/135) of men practising full time who left practice in 1991 were practising again in 1994. CONCLUSION: Simply using total numbers of general practitioners or net increase to describe workforce trends masks much movement in and out of general practice and between differing time commitments. Recruitment and retention issues need to be separated if reasonable policies are to be developed to assure the necessary general practitioner workforce for a primary care led NHS.

Adult↗

Patients with urinary tract infection: proposed management strategies of general practitioners, microbiologists and urologists.

BACKGROUND: It is difficult to implement change in general practice. It is not known how best to conduct effective continuing medical education in general practice. General practitioners' criteria for good clinical practice vary and it is unknown whether systematic education by hospital specialists could be expected to reduce variation between general practitioners. AIM: A study was undertaken to describe general practitioners', microbiologists' and urologists' strategies for diagnosis, treatment, and follow up of female patients with symptoms of urinary tract infection, a common reason for consultation in general practice. The findings of the study were to be used as a base upon which to discuss the advantages and disadvantages of using hospital specialists as a resource in general practitioners' peer group based continuing medical education. METHOD: Three vignettes together with several proposals for diagnosis, treatment and follow up were presented in a questionnaire to general practitioners, microbiologists and urologists in Denmark. The case histories concerned three female patients (aged 10, 30 and 60 years) who consulted their general practitioner for advice. The female patients were otherwise healthy and well known to the practice. General practitioners', microbiologists' and urologists' recommendations for good clinical practice were compared. RESULTS: A total of 154 general practitioners (77%), 45 microbiologists (51%) and 54 urologists (61%) who were eligible for the study responded to the questionnaire. There was considerable variation in the management strategies proposed by doctors within each specialty and between the specialties. Microbiologists and urologists were more likely to suggest treating the 30-year-old woman by giving advice and a prescription by telephone compared with their general practitioner colleagues. Conversely, the microbiologists and urologists were more likely to suggest asking the 10- and 60-year-old patients to attend the clinic for examination compared with the general practitioners. The general practitioners reported asking the patients to return for follow up more commonly than the hospital specialists. CONCLUSION: Large variation in suggested strategies for diagnosis, treatment and follow up was shown both within and between specialties. Continuing medical education of general practitioners based on small peer group discussions using hospital specialists as a group resource would not necessarily reduce variation in clinical practice between general practitioners. A need for evidence-based rather than consensus-directed guidelines would be needed in order to reduce variation in clinical practice between doctors.

Adult↗

Phenytoin versus valproate monotherapy for partial onset seizures and generalized onset tonic-clonic seizures.

BACKGROUND: Phenytoin and valproate are commonly used antiepileptic drugs. It is generally believed that phenytoin is more effective for partial onset (simple partial, complex partial and secondary generalized tonic-clonic seizures) seizures whilst valproate is more effective in generalized onset seizures (generalized tonic-clonic seizures, absence, myoclonus) although there is no evidence from randomized controlled trials to support this belief. The use of individual patient data meta-analysis enabled us to examine time to event outcomes which are important in epilepsy monotherapy trials, and also to examine treatment-covariate interactions. OBJECTIVES: To review the best evidence comparing phenytoin and valproate when used as monotherapy in subjects with partial onset seizures, or generalized onset tonic-clonic seizures with or without other generalized seizure types. SEARCH STRATEGY: Our search strategy included: (i) the Cochrane Epilepsy Group trial register, (ii) MEDLINE 1966-2000, (iii) hand-searching relevant journals, (iv) the pharmaceutical industry, and (v) researchers in the field. SELECTION CRITERIA: Randomized controlled trials in children or adults with partial onset seizures or generalized onset tonic-clonic seizures. Trials must have included a comparison of phenytoin monotherapy with valproate monotherapy. DATA COLLECTION AND ANALYSIS: This was an individual patient data review. Outcomes were time to (i) withdrawal of allocated treatment, (ii) 12 month remission, (iii) six month remission, and (iv) first seizure post randomization. Data were analysed using stratified logrank analysis with results expressed as hazard ratios (HR) and 95% confidence intervals (95% CI), where a HR>1 indicates an event is more likely on phenytoin. A test for interaction between treatment and seizure type (partial onset versus generalized onset) was also undertaken for each outcome. MAIN RESULTS: Data were available for 669 subjects from five trials, representing 60% of the subjects recruited into the eleven trials that met our inclusion criteria. One important limitation of these data is that in four of the five trials, for patients classified as having generalized onset seizures, tonic-clonic seizures were the only seizure types recorded at follow up, despite the fact that some patients will have been experiencing other generalized seizure types such as absence or myoclonus. Their results for the generalized seizures therefore relate only to generalized onset tonic-clonic seizures. The main overall results were as follows (HR(95% CI), HR>1 indicates a clinical advantage for phenytoin for both remission outcomes and a clinical advantage for valproate for the outcomes time to withdrawal and time to first seizure) (i) time to withdrawal of allocated treatment 1.10(0.79-1.54), (ii) time to 12 month remission 1.04(0.78-1.38), (iii) time to six month remission 0.89(0.71-1.11), and (iv) time to first seizure 0.92(0.74-1.14). The results suggest no overall difference between drugs for these outcomes. The test for an interaction between treatment and seizure type (generalized versus partial onset) was non significant for all outcomes. REVIEWER'S CONCLUSIONS: We have not found evidence that a significant difference exists between phenytoin and valproate for the outcomes examined in this review. As generalized seizures such as absence and myoclonus were counted in only one trial, results do not address the treatment of these seizure types. We found no unequivocal evidence to overthrow or support the policy of using valproate in generalized onset tonic-clonic seizures and phenytoin in partial onset seizures.

Anticonvulsants↗

Changing relationships: attitudes and opinions of general practitioners and pharmacists regarding the role of the community pharmacist.

BACKGROUND: Relationship between general practitioners and pharmacists. AIM: To explore similarities and differences in opinions between general practitioners and pharmacists about the pharmacist's role. To identify factors which determine the attitude of the general practitioner towards the role of the pharmacist as a care provider. DESIGN: Cross-sectional questionnaire survey. SETTING: The Netherlands, 2001. PARTICIPANTS: 926 non-dispensing general practitioners, 93 dispensing general practitioners and 328 community pharmacists. METHODS: Statements about the pharmacist's position in primary care were formulated. The attitude towards the pharmacist's care-providing function was determined by adding the scores to the statements. Data were collected on age, gender, professional experience, trainership, type and size of practice, electronic communication, urbanisation level, part-time work, work pressure, postgraduate training, pharmacotherapy audit meetings (PTAMs), the mutual relationship and the desired role of the pharmacist in pharmacotherapy. RESULTS: No significant differences in opinions were found between pharmacists and non-dispending general practitioners with respect to a number of the pharmacist's signalling tasks. Eighty-six percent (CI 83-89) of non-dispensing general practitioners and eighty-two percent (CI 76-87) of pharmacists shared the opinion that pharmacists need to provide the general practitioner with feedback on prescription figures. Furthermore, 85% (CI 82-88) of the non-dispensing general practitioners and 80% (CI 75-86) of the pharmacists believed that pharmacists should suggest items for the PTAM agenda, based on prescription patterns they notice in their pharmacy. The attitude of general practitioners towards the care-providing function of the community pharmacist correlates significantly with the relationship between general practitioners and pharmacists (P < 0.001). There is also a significant difference (P < 0.001) in the attitude score between dispensing and non-dispensing general practitioners: dispensing general practitioners scored lower on the attitude scale. CONCLUSION: Pharmacists and general practitioners largely agree on the pharmacotherapeutic signalling role that a pharmacist should fulfill. A good relationship benefits the attitude of general practitioners towards the pharmacist's care-providing function.

Attitude of Health Personnel↗

Generalized pustular psoriasis in Japan: two distinct groups formed by differences in symptoms and genetic background.

A multi-center study for investigating generalized pustular psoriasis was carried out in Japan in order to clarify the prevalence, the etiology, and the standard therapy. Through questionnaires, 541 cases of patients with generalized pustular psoriasis and related disorders were collected, and the history, the precipitating factors, the symptoms and the laboratory findings were analyzed from medical records. The statistic analysis revealed that 902 (+/- 102.2) patients with generalized pustular psoriasis were expected at the time, and that the prevalence of generalized pustular psoriasis in Japan was 7.46/million. Of 541 patients with generalized pustular psoriasis, 208 cases had the recurrent episodes of symptoms with generalized pustules which had been referred to von Zumbusch type pustular psoriasis or to the acute form of generalized pustular psoriasis. Two hundred and eight patients were further subdivided into two groups: one with a preceding history of ordinary psoriasis (pso+ generalized pustular psoriasis; 65 cases) and another without a psoriasis history (pso- generalized pustular psoriasis; 143 cases). Subdivision into the two groups defined that the onset of pustular outbreak was earlier in the pso- generalized pustular psoriasis group. It was also pointed out that the precipitating factors were different, as the pso- generalized pustular psoriasis group was more frequently affected after infections, and that the pso+ generalized pustular psoriasis group was more frequently affected by preceding corticosterold therapy. HLA analysis of 92 cases with von Zumbusch type generalized pustular psoriasis confirmed that A2, B14, B35 phenotypes were weakly correlated, and when 92 cases were subdivided into the two groups, the pso+ generalized pustular psoriasis group revealed a statistically significant correlation with A1 (p < 0.01), B37 (p < 0.02) and DRw10 (p < 0.05), which was closely related with psoriasis vulgaris patients in Japan. Both clinical surveillance and genetic analysis have disclosed the heterogeneity of von Zumbusch type generalized pustular psoriasis, as there exist two types; one is closely related to psoriasis and the other not.

Adolescent↗

General health status measures for people with cognitive impairment: learning disability and acquired brain injury.

BACKGROUND: Currently there is a wide range of health status measures that aim to assess general health status in people with cognitive impairment. However, the validity and/or applicability to this patient group are largely unknown. This has implications for the assessment of treatment outcomes and rehabilitation, for prognostic purposes, for planning services, and for determining the benefits and adverse effects of health technologies targeted at these patient groups. OBJECTIVES: (1) To identify the general health status measures that have been validated in patients with cognitive impairment. (2) To assess the extent to which these measures have been validated. (3) To draw out the implications of the findings for the use of existing measures and for future primary research in this area. METHODS. SELECTION CRITERIA: Studies that assessed general health status in people with cognitive impairment due to acquired brain injury (traumatic brain injury, cerebro-vascular accident or multiple sclerosis (MS)) or learning disability (LD) were included in the review. Studies that used general health status instruments measuring only one general health dimension, and studies that only featured participants with cognitive impairment due to dementia were excluded. METHODS. SEARCH STRATEGY: A wide range of relevant databases were searched for studies on cognitive impairment, general health status measures, and validation of health status measures. A handsearch of general health status bibliographies was also conducted. Data were collected on the general health status measure used, the population characteristics, aims of the study, validity details, and conclusions. RESULTS: The review includes data from 71 studies, reported in 83 separate publications. In total 34 different general health status measures were described in the 83 publications, with the Sickness Impact Profile (SIP) and the Short Form-36 (SF-36) the most frequently used measures (20 and 19 studies, respectively). These studies included a total of 98 instrument validations, 52 of which definitely or probably included people with cognitive impairment. Six measures were extensively validated (quality scores ranged from 0.25 to 0.5, on a scale from 0 to 1) in studies in which more than 50% of the respondents were people with cognitive impairment. A further three measures were also validated in studies in which more than 50% of the respondents were people with cognitive impairment, but their level of validation was more limited (quality scores ranged from 0.1 to 0.2). Five measures were validated in studies in which 20-50% of the respondents were cognitively impaired, which may limit their relevance to participants with cognitive impairment (quality scores ranged from 0.1 to 0.6). The SF-36 was also validated in two studies in which 20-50% of the respondents were cognitively impaired and the quality score was 0.3. Finally, nine of the measures were only validated in studies in which less than 20% of the respondents were cognitively impaired. For these measures it was unclear whether the findings applied to people with cognitive impairment. CONCLUSIONS: Very few measures have been validated specifically for cognitively impaired respondents. Studies where at least 50% of the respondents were cognitively impaired generally showed poorer validity results compared with studies with fewer cognitively impaired persons, indicating that general health status measures designed for the general population are not automatically suitable for people with cognitive impairment. The few measures that were specifically developed for people with cognitive impairment also reported poor validity results. Therefore, there are no validated instruments available for use in cognitively impaired respondents; existing measures, specifically designed for use in these populations, should be used with caution. The most promising measure is the MS-Quality of Life Interview (MS-QLI) for MS patients. The MS-QLI was thoroughly validated in 300 MS patients and the results were good, except for the 'social function' subscale. However, only 20-50% of the respondents in this study had cognitive impairment. Most information on the validity of general health status measures was found in studies among people with LD. For these patients, six measures were found that have been validated in a populations where more than 50% of the respondents were cognitively impaired LD patients. CONCLUSIONS: (1) Existing general health status measures should be used with caution in individuals with cognitive impairments. (2) There is no evidence to indicate the most suitable general health status measure for use in economic evaluations of cognitive impairment. (3) There is little evidence to support the validity of proxy assessments in cognitively impaired populations. (ABSTRACT TRUNCATED)

Brain Injuries↗

[Prescription of high dose buprenorphine by general practitioners].

BACKGROUND: Since 1996, prescribing buprenorphine in high dosage as a drug maintenance treatment has been allowing French general practitioners to undertake drug addicts with a pharmacological support. In France, buprenorphine prescriptions seem to spread over general practitioners (buprenorphine was given to 74,300 patients in 2001). This paper assesses the different factors associated with buprenorphine prescription by general practitioners and with the different degrees of general practitioners' commitment in actually caring drug addicts with the help of buprenorphine. METHODS: Two representative samples of private general practitioners, either prescribing buprenorphine (345 over a population of 876) or not prescribing (355 over a population of 1380) have been questioned through a phone interview in the department of South-Eastern France in November and December 2002. Samples have been constituted with the help of a random stratified survey according to sex, age and volume of services (acceptance rate: 65.5%). Survey data have been completed with general practitioners' activity data from Health Insurance and local socioeconomic data from Insee. RESULTS: 37.5% of general practitioners have at least once prescribed buprenorphine during the considered period, but only 26% of the prescribers treated 75% of patients. Prescribers are most often men, younger than 49 years, working in the fixed fees sector and having group practice. They are also most often members of a health care network, trained for drug maintenance treatments and, from an individual point of view, have relatives suffering cancer, or having HIV, or hepatitis C, or who are drug addicts. Low socioeconomic status of the area where GPs exert seems to be particularly associated with general practitioners' prescription of buprenorphine. CONCLUSION: Data handled in this paper show that supply of substitutive treatments is concentrated among a reduced number of general practitioners and in particularly deprived geographic areas. Workload in deprived areas combined to great professional commitment in maintenance treatment reveals unexpected and unwanted specialisation behaviours by general practitioners, as well as more isolated behaviours by general practitioners who do not ask for particular training program or help by colleagues. These results question the consistency of the general framework of support to general practitioners proposed by health authorities with general practitioners actual practice and needs.

Analgesics, Opioid↗

Setting priorities and identifying barriers for general practice research in Europe. Results from an EGPRW meeting.

BACKGROUND: In spring 2002, WONCA Europe, the European Society of General Practice/Family Medicine and its Network organizations reached consensus on a 'new' European definition of general practice. Subsequently, the European General Practice Research Workshop (EGPRW) started working on a European General Practice Research Agenda. This topic was addressed during the 2002 EGPRW autumn meeting. OBJECTIVE: Our aim was to explore the views of European general practice researchers on needs and priorities as well as barriers for general practice research in Europe. METHODS: In seven discussion groups, 43 general practice researchers from 18 European countries had to answer the following questions. (i) What major topics should be included in a research agenda for general practice in your country? (ii) What are the barriers to adequate implementation of general practice research in your country? Group answers were listed and subsequently categorized by two authors. RESULTS: Research on 'clinical issues' (common diseases, chronic diseases, etc.), including diagnostic strategies, was considered to be the core content of general practice research, with primary care-based morbidity registration essential for surveillance of disease, clinical research and teaching in general practice. There was also consensus on the need for research on education and teaching. 'Insufficient funding opportunities' was perceived to be the major barrier to the development of general practice research. CONCLUSIONS: These findings could be used as a basis for national checklists of 'content of' and 'conditions for' general practice research. European general practice research training programmes should be developed further.

Communication Barriers↗