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Selected AbstractsNew patients, new lessons, new thinking in inflammatory bowel disease: World Congress of Gastroenterology Symposium, Montreal, Canada, September 2005COLORECTAL DISEASE, Issue 2006D. P. Jewell No abstract is available for this article. [source] Changing pattern of care of boys with haemophilia in western European centresHAEMOPHILIA, Issue 2 2005H. Chambost Summary., Haemophilia management is not uniform among countries, even within western Europe, that have close economic, social and cultural relationship. The European Paediatric Network PedNet aims to share experiences in the field of the care of boys with haemophilia. In 1998, a PedNet survey has shown significant disparities in 20 centres from 16 countries, particularly as regards the implementation of prophylaxis regimen. This survey has been updated in 2003 to describe the current status of haemophilia management in 22 centres and the changing pattern of care of boys with severe haemophilia in western Europe. Regular, continuous long-term prophylaxis is provided in all PedNet centres, more than 50% and 80,100% of boys being treated this way in 20/22 and 15/22 centres respectively. Twenty of the 22 centres (91%) recommend continuous prophylaxis (primary or secondary A) for a new patient. The use of recombinant factor VIII concentrates was already widespread in 1998 and a further expansion of recombinant products has been observed over the last 5 years. Recombinant FVIII is now used exclusively in nine centres and for more than 80% of boys with haemophilia A in nine other centres. The use of recombinant and plasma derived FIX is more balanced: among 18 centres where boys with haemophilia B are treated, 14 use recombinant FIX, and nine administer it to a majority of patients. Other modifications of practice have been stressed in this survey, such as more targeted use of central venous devices in the youngest boys and more extensive characterisation of genetic mutations. [source] Prediction of survival in patients with head and neck cancerHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 9 2001Robert Jan Baatenburg de Jong PhD Abstract Background In patients with head and neck squamous cell carcinoma (HNSCC) the estimated prognosis is usually based on the TNM classification. The relative weight of the three contributing parameters is often not completely clear. Moreover, the impact of other important clinical variables such as age, gender, prior malignancies, etc is very difficult to substantiate in daily clinical practice. The Cox-regression model allows us to estimate the effect of different variables simultaneously. The purpose of this study was to design a model for application in new HNSCC patients. In our historical data-base of patients with HNSCC, patient, treatment, and follow-up data are stored by trained oncological data managers. With these hospital-based data, we developed a statistical model for risk assessment and prediction of overall survival. This model serves in clinical decision making and appropriate counseling of patients with HNSCC. Patients and Methods All patients with HNSCC of the oral cavity, the pharynx, and the larynx diagnosed in our hospital between 1981 and 1998 were included. In these 1396 patients, the prognostic value of site of the primary tumor, age at diagnosis, gender, T-, N-, and M-stage, and prior malignancies were studied univariately by Kaplan-Meier curves and the log-rank test. The Cox-regression model was used to investigate the effect of these variables simultaneously on overall survival and to develop a prediction model for individual patients. Results In the univariate analyses, all variables except gender contributed significantly to overall survival. Their contribution remained significant in the multivariate Cox model. Based on the relative risks and the baseline survival curve, the expected survival for a new HNSCC patient can be calculated. Conclusions It is possible to predict survival probabilities in a new patient with HNSCC based on historical results from a data-set analyzed with the Cox-regression model. The model is supplied with hospital-based data. Our model can be extended by other prognostic factors such as co-morbidity, histological data, molecular biology markers, etc. The results of the Cox-regression may be used in patient counseling, clinical decision making, and quality maintenance. © 2001 John Wiley & Sons, Inc. Head Neck 23: 718,724, 2001. [source] Comparison of Commonly Used Assays for the Detection of MicroalbuminuriaJOURNAL OF CLINICAL HYPERTENSION, Issue 2004Douglas E. Busby MD There are a variety of methods for assessing urinary albumin excretion, extending from the very low-range microalbuminuria to higher ranges extending into macroalbuminuria or proteinuria. The recommendation for the initial screening of a new patient is to use a urine dipstick to assess for microalbuminuria. If positive, a spot urine for albumin:creatinine should be measured and reassessed annually. All patients with kidney disease, diabetes, or hypertension and metabolic syndrome should be screened for albuminuria. New methodologies using high-performance liquid chromatography are much more sensitive and specific when compared with older methods of detection and may prove very useful for earlier identification of high-risk patients. This is important since studies have shown that albuminuria levels below the microalbuminuria range, determined by conventional methodologies in uncomplicated essential hypertensive men, are associated with an adverse cardiovascular and metabolic risk profile. High performance liquid chromatography methodology, in contrast to older studies, detects all intact albumin and enables clinicians to assess disease severity and monitor therapeutic effectiveness with confidence in the accuracy of the microalbuminuria data reported to them. [source] Measuring the sensations of urge and bladder filling during cystometry in urge incontinence and the effects of neuromodulationNEUROUROLOGY AND URODYNAMICS, Issue 1 2003Sarah Oliver Abstract Aims: As urge and urgency contribute greatly to a patient's symptoms, it follows that sensory evaluation combined with noninvasive neuromodulation during urodynamics may provide new criteria for improving patient selection for an implantable stimulator. The purpose of this research was to develop and validate an objective measure of bladder sensations during filling cystometry and then to apply this technique to evaluate the effects of neuromodulation on the sensations of urge measured in this way. Methods: In study 1 a new patient-activated keypad device was tested during urodynamics to measure bladder sensations according to a 0,4 scale and validated by using a technique adapted from a standard psychophysical sensory threshold testing method. In study 2 the effects of pudendal afferent nerve stimulation on measured sensations of urge were assessed during cystometry with patients as their own controls. Forty-three patients diagnosed with idiopathic detrusor instability were studied; 10 participated in study 1 and 35 in study 2. Results: The new device gave reliable and repeatable measures of sensations with statistically significant differences in bladder volume at each of the urge levels tested (Wilcoxon matched pairs test). Neuromodulation suppressed urinary urge in 89% of the 35 patients. This effect was associated with a statistically significant increase in bladder volume at all urge levels. Conclusions: A new patient operated key-pad device provided a reliably objective measure of sensations of urge during urodynamics without the need for prompting. Neuromodulation using noninvasive pudendal afferent stimulation suppressed these sensations whilst increasing bladder volume. Neurourol. Urodynam. 22:7,16, 2003. © 2003 Wiley-Liss, Inc. [source] Gastric fundic gland polyps: a clinico-pathological study from North West TasmaniaANZ JOURNAL OF SURGERY, Issue 6 2009Inian Samarasam Abstract Background:, Fundic gland polyps (FGPs) of the stomach were originally described in association with familial polyposis syndromes. It is now known that the majority of these polyps occur in the sporadic setting and are incidentally seen in up to 1.9% of routine upper gastrointestinal endoscopes. The aim of this study was to look at the clinico-pathological features of the FGPs and to analyse their relationship to Helicobacter pylori infection, proton pump inhibitor treatment, colonic polyps and malignancy. Methods:, A search of the histopathology records for a period of 10 years from 1997 to 2006 identified 120 patients with a histologically confirmed diagnosis of FGPs. The clinical history, upper gastrointestinal endoscopy findings, histopathology and colonoscopy findings were recorded from the medical records and analysed. Results:, FGPs were seen in 3.2% of patients undergoing routine upper gastrointestinal endoscopes. There was a definite association with long-term proton pump inhibitor treatment. There was a strikingly low incidence of H. pylori infection in the study population. Although there was no dysplasia or malignancy in any of these polyps, one patient had concomitant adenocarcinoma of the stomach. In the subgroup of patients who also had colonoscopy during the study period, 19% had associated colonic polyps and 6% had associated colonic malignancies. Conclusions:, Every new patient diagnosed with FGPs should have a thorough clinico-pathological study to see if the polyps are part of a sporadic or syndromic setting. A long-term follow-up study of patients with FGPs and its association with colonic polyps may be warranted. [source] Migrating Partial Seizures in Infancy: Expanding the Phenotype of a Rare Seizure SyndromeEPILEPSIA, Issue 4 2005Eric Marsh Summary:,Purpose: The constellation of early-onset, unprovoked, alternating electroclinical seizures and neurodevelopmental devastation was first described by Coppola et al. We report six new patients and the prospect of a more optimistic developmental outcome. Methods: Retrospective chart reviews were performed on six infants evaluated at the Children's Hospital of Philadelphia (five patients) and at Hershey Medical Center (one patient) who had electroclinically alternating seizures before age 6 months of age. Electroclinical characteristics and long-term follow-up were recorded. Results: All had unprovoked, early-onset (range, 1 day to 3 months; mean, 25 days) intractable electroclinical seizures that alternated between the two hemispheres. Each patient underwent comprehensive brain imaging and neurometabolic workups, which were unrevealing. In all patients, subsequently intractable partial seizures developed and often a progressive decline of head circumference percentile occurred with age. Three demonstrated severe developmental delay and hypotonia. All survived, and 7-year follow-up on one patient was quite favorable. Conclusions: Our patients satisfied the seven major diagnostic criteria first described by Coppola et al. The prognosis of this rare neonatal-onset epilepsy syndrome from the original description and subsequent case reports was very poor, with 28% mortality, and the majority of survivors were profoundly retarded and nonambulatory. Our patient data validate the diagnostic criteria of this syndrome and further quantify a previously described observation of progressive decline of head circumference percentiles with age. Our data also suggest that the prognosis of this syndrome, although poor, is not as uniformly grim as the cases reported previously in the literature. [source] Physical assessment of patients with anorexia nervosa and bulimia nervosa: an international comparisonEUROPEAN EATING DISORDERS REVIEW, Issue 6 2003D. Kovacs Abstract Objective: A questionnaire study was carried out to determine which investigations were carried out routinely on patients with anorexia nervosa and bulimia nervosa. Method: A specially designed questionnaire was sent to 168 clinicians working in the field of eating disorders in 25 countries. Respondents were asked to supply information about how often they carry out specific investigations on new patients with AN and BN. The questionnaire covered the use of physical examination, biochemical and haematological tests and cardiac investigations. Results: 71,(42.3,per cent) questionnaires were returned. Biochemical investigations and full blood counts were carried out frequently. Significant differences were found between AN patients and BN patients in the measurement of calcium, phosphate and magnesium levels. In some cases, patients with BN were not routinely assessed for hypokalaemia. Micronutrient levels were measured rarely and only 40,per cent of respondents carried out routine electrocardiograms (ECGs) in AN. Discussion: Measurement of serum potassium should be routine in BN and other electrolytes should probably measured more often in both disorders. Detection of treatable micronutrient deficiencies should be given more emphasis and the ECG should become a routine investigation in AN. Copyright © 2003 John Wiley & Sons, Ltd and Eating Disorders Association. [source] A collaborative approach to embedding graduate primary care mental health workers in the UK National Health ServiceHEALTH & SOCIAL CARE IN THE COMMUNITY, Issue 5 2008Janine Fletcher MSc Abstract The UK National Health Service (NHS) workforce has recently seen the arrival of the Graduate Mental Health Worker (GMHW) in primary care. We established a Quality Improvement Collaborative to assist in embedding this new workforce in one Strategic Health Authority Area of England. The intervention utilised ,collaborative' technology which involves bringing together groups of practitioners from different organizations to work in a structured way to improve the quality of their service. The process was evaluated by an action research project in which all stakeholders participated. Data collection was primarily qualitative. During the project, there was an increase in throughput of new patients seen by the GMHWs and increased workforce satisfaction with a sense that the collaborative aided the change process within the organizations. Involvement of managers and commissioners from the Primary Care Trusts where the GMHWs were employed appeared to be important in achieving change. This was not, however, sufficient to combat significant attrition of the first cohort of workers. The project identified several barriers to the successful implementation of a new workforce for mental health problems in primary care, including widespread variation in the level and quality of supervision and in payment and terms of service of workers. A collaborative approach can be used to support the development of new roles in health care; however, full engagement from management is particularly necessary for success in implementation. The problems faced by GMHWs reflect those faced by other new workers in healthcare settings, yet in some ways are even more disturbing given the lack of governance arrangements put in place to oversee these developments and the apparent use of relatively unsupported and inexperienced novices as agents of change in the NHS. [source] Mucopolysaccharidosis type IIID: 12 new patients and 15 novel mutations,HUMAN MUTATION, Issue 5 2010Marlies J. Valstar Abstract Mucopolysaccharidosis III D (Sanfilippo disease type D, MPS IIID) is a rare autosomal recessive lysosomal storage disorder previously described in only 20 patients. MPS IIID is caused by a deficiency of N-acetylglucosamine-6-sulphate sulphatase (GNS), one of the enzymes required for the degradation of heparan sulphate. So far only seven mutations in the GNS gene have been reported. The clinical phenotype of 12 new MPS IIID patients from 10 families was studied. Mutation analysis of GNS was performed in 16 patients (14 index cases). Clinical signs and symptoms of the MPS IIID patients appeared to be similar to previously described patients with MPS III. Early development was normal with onset of behavioral problems around the age of 4 years, followed by developmental stagnation, deterioration of verbal communication and subsequent deterioration of motor functions. Sequence analysis of the coding regions of the gene encoding GNS (GNS) resulted in the identification of 15 novel mutations: 3 missense mutations, 1 nonsense mutation, 4 splice site mutations, 3 frame shift mutations, 3 large deletions and 1 in-frame small deletion. They include the first missense mutations and a relatively high proportion of large rearrangements, which warrants the inclusion of quantitative techniques in routine mutation screening of the GNS gene. © 2010 Wiley-Liss, Inc. [source] Screening of ARHSP-TCC patients expands the spectrum of SPG11 mutations and includes a large scale gene deletion,HUMAN MUTATION, Issue 3 2009Paola S. Denora Abstract Autosomal recessive spastic paraplegia with thinning of corpus callosum (ARHSP-TCC) is a complex form of HSP initially described in Japan but subsequently reported to have a worldwide distribution with a particular high frequency in multiple families from the Mediterranean basin. We recently showed that ARHSP-TCC is commonly associated with mutations in SPG11/KIAA1840 on chromosome 15q. We have now screened a collection of new patients mainly originating from Italy and Brazil, in order to further ascertain the spectrum of mutations in SPG11, enlarge the ethnic origin of SPG11 patients, determine the relative frequency at the level of single Countries (i.e., Italy), and establish whether there is one or more common mutation. In 25 index cases we identified 32 mutations; 22 are novel, including 9 nonsense, 3 small deletions, 4 insertions, 1 in/del, 1 small duplication, 1 missense, 2 splice-site, and for the first time a large genomic rearrangement. This brings the total number of SPG11 mutated patients in the SPATAX collection to 111 cases in 44 families and in 17 isolated cases, from 16 Countries, all assessed using homogeneous clinical criteria. While expanding the spectrum of mutations in SPG11, this larger series also corroborated the notion that even within apparently homogeneous population a molecular diagnosis cannot be achieved without full gene sequencing. © 2008 Wiley-Liss, Inc. [source] Periapical status and quality of root fillings and coronal restorations in an adult Spanish populationINTERNATIONAL ENDODONTIC JOURNAL, Issue 8 2004J. J. Segura-Egea Abstract Aim, To investigate the quality of root fillings and coronal restorations and their association with periapical status in an adult Spanish population. Methodology, A total of 180 subjects, aged 37.1 ± 15.7 years, who presented as new patients at the Faculty of Dentistry, Seville, Spain, were examined. All participants underwent a full-mouth radiographic survey incorporating 14 periapical radiographs. The periapical region of all root filled teeth, excluding third molars, were examined. The technical quality of root fillings was evaluated in terms of length in relation to the root apex and lateral adaptation to the canal wall. Radiographic signs of overhang or open margins associated with coronal restorations were also evaluated. Periapical status was assessed using the Periapical Index score. Statistical analyses were conducted using the Cohen's , test and logistic regression. Results, The total number of root filled teeth was 93, and 60 (64.5%) had apical periodontitis (AP). Presence of AP in root filled teeth was associated with inadequate adaptation of the filling (OR = 2.29; P = 0.06), inadequate length of the root filling (OR = 2.44; P = 0.048), and with poor radiographic quality of the coronal restoration (OR = 2.38; P = 0.054). Only 34.4% of the root fillings were adequate from a technical perspective. When both root fillings and coronal restorations were adequate the incidence of AP decreased to 31.3% (OR = 5.50; P < 0.01). Conclusions, The incidence of AP in root filled teeth was high. Many root fillings were technically unsatisfactory. Adequate root fillings and coronal restorations were associated with a lower incidence of AP; an adequate root filling had a more substantial impact on the outcome of treatment than the quality of the coronal restoration. [source] Prevalence of apical periodontitis and frequency of root-filled teeth in an adult Spanish populationINTERNATIONAL ENDODONTIC JOURNAL, Issue 3 2004A. Jiménez-Pinzón Abstract Aim, To estimate the prevalence of teeth with apical periodontitis (AP) and root-filled teeth in an adult Spanish population. Methodology, A total of 180 subjects, aged 37.1 ± 15.7 years, presenting as new patients to the Faculty of Dentistry, Seville, Spain, were examined. All participants underwent a full-mouth radiographic survey (14 periapical radiographs). The frequency of root canal treatment and the periapical status of all teeth, using the periapical index (PAI) score, were assessed. An intraobserver agreement test on PAI scores produced a Cohen's kappa of 0.77 (substantial agreement). Results were analysed statistically using the Chi-square test. Results, Apical periodontitis in one or several teeth was found in 110 subjects (61.1% prevalence), and 73 (40.6% prevalence) had at least one root-filled tooth. Among subjects with root-filled teeth, 48 (65.8%) had AP affecting at least one root-filled tooth. A total of 4453 teeth were examined, of these 186 (4.2%) had AP. The total number of root-filled teeth was 93 (2.1%), of which 60 (64.5%) had AP. Among non-root filled teeth, only 2.9% had AP. The prevalence of AP in connection with molar teeth was higher (5.5%) than for premolar (4.5%) and anterior teeth (3.2%; P < 0.01). More premolar and molar teeth were root-filled (2.8 and 2.7%, respectively) than anterior teeth (1.3%; P < 0.01). The prevalence of AP increased with age. Conclusions, The prevalence of AP in root-filled and untreated teeth, and the frequency of root-filled teeth were comparable to those reported in previous similar studies carried out in European countries. The prevalence of root-filled teeth with AP was found to be higher compared to that demonstrated in other epidemiological studies. [source] Change in pattern of skin disease in Kaduna, north-central NigeriaINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 9 2007Husain Yahya MSc Background, We report our experience on the pattern of skin disease in Kaduna, north-central Nigeria over a 6-year period, and compare it with a similar survey conducted in the same area 30 years ago and with surveys from Nigeria and from other parts of Africa. Methods, The medical records of new patients attending the dermatology outpatient clinics of Barau Dikko Specialist Hospital and Habbat Medical Center from March 2000 to December 2005 were retrieved. Demographic data (age and sex) and the diagnoses of skin disease were extracted and analyzed. Results, A total of 5982 cases was seen. Forty-nine per cent were males and 51% were females. One-third of the patients were aged under 20 years, and three quarters were aged below 40 years. Eczematous dermatitis was the most common skin disorder seen, making up 35% of cases, and had replaced dermatophyte infections and scabies, which were the most dominant skin diseases 30 years previously (now constituting 6% and 1.4% of cases, respectively). Atopic dermatitis had more than doubled in frequency (13.8% vs. 5.2%), and contact dermatitis had tripled in frequency (5.8% vs. 1.8%). Acne vulgaris (6.7%), pigmentary disorders (3.9%), urticaria (3.6%), papular urticaria (3.6%), hair disorders (3.3%), lichen simplex chronicus (3%), viral warts (2.9%), and drug eruptions (2.7%) had also increased. Human immunodeficiency virus-related skin disease constituted 4.3% of cases, with pruritic papular eruption being the most common condition. Conclusion, These changes in skin disease can be attributed mainly to an increase in urbanization and improved socio-economic conditions. [source] Males assessed by a specialized adult eating disorders service: Patterns over time and comparisons with femalesINTERNATIONAL JOURNAL OF EATING DISORDERS, Issue 8 2008Eric Button PhD Abstract Objective: In view of previous inconsistencies and the limited literature on males with eating disorders, we aimed to examine changes in presentation rates over time and any differences between males and females. Method: In a cohort of 2,554 new patients assessed by a specialized service for adults over a 21-year period, we examined rates by gender over time. We also carried out a detailed comparison of selected clinical and demographic variables on a series of 65 males and females matched by diagnosis and date of assessment. Results: Approximately 5% of patients were male and there was no evidence of a change in presentation rate by gender over time. Males were more likely to be diagnosed as not having a clinical eating disorder and less likely to abuse laxatives, but otherwise there was little difference in clinical presentation. Conclusion: Eating disorders continue to present predominantly in females and the proportion of males remains broadly stable. © 2008 by Wiley Periodicals, Inc. Int J Eat Disord 2008 [source] Lip cancer in Northern Finland: changing incidence and clinical characteristicsJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 7 2000Olli-Pekka Alho Abstract: A population-based survey was conducted in 35 municipalities in Northern Finland to assess the incidence of lip cancer as well as the patient and tumour characteristics in cases diagnosed between 1983 and 1997. A total of 96 new patients emerged. The age-standardised incidence (per 100,000 years) of lip cancer in men decreased from 4.8 in 1983,1987 to 1.4 in 1993,1997. The incidences in women were 0.30 to 0.36, respectively. The median age of the patients increased from 66 to 73 years through the years. Overall, 90% of the patients had at least one of the known risk factors, namely rural domicile, outdoor occupation or smoking. The median duration of symptoms also remained the same, as did the median size and location of the tumour at diagnosis. In contrast, spread to regional lymph nodes became rare during the period. [source] Depression and diffuse physical symptoms in southern Chinese with temporomandibular disordersJOURNAL OF ORAL REHABILITATION, Issue 6 2009A. S. MCMILLAN Summary, The study investigated the experience of depressive symptoms and the relationship with diffuse physical symptoms reporting in southern Chinese seeking professional care for temporomandibular disorders (TMD) in Hong Kong. Eighty-seven new patients [77 females/10 males; mean age 39·3 years (SD 12·7)] referred to the specialist TMD clinic at the Prince Philip Dental Hospital, Hong Kong participated in this study. The Research Diagnostic Criteria (RDC)/TMD history questionnaire was used to derive Axis II psychological data. Psychological status was assessed through depression and non-specific physical symptoms (NPS) scores (pain items included and excluded) measured with RDC/TMD Axis II instruments; 42·5% of patients experienced moderate/severe depression symptoms; 59·8% and 57·5% had moderate/severe NPS scores when pain items were included and excluded, respectively. Strong, positive and statistically significant correlations were noted between depression scores and the NPS scores that included pain items (r = 0·80) and those that did not (r = 0·80). The correlations remained consistent and were of similar magnitude when male patients were excluded from the computation and also when the possible effect of patient age was controlled. While taking into account the modest patient sample which was related to a low rate of treatment seeking, depressive symptoms were common and similar to other western and Chinese patient groups. NPS reports were higher than in Singapore Chinese patients. There appeared to be a clear association between depression and diffuse physical symptoms. The findings should be considered in the holistic care of Chinese people with TMD. [source] Nail Changes in Childhood Psoriasis: A Study from KuwaitPEDIATRIC DERMATOLOGY, Issue 1 2007FRCPC, Nawaf Al-Mutairi M.D. Childhood psoriasis is a distinct entity and the literature focused on nail changes associated with childhood psoriasis is scant. Our objectives were to evaluate the frequency of nail involvement in childhood psoriatic patients, assess the types of nail changes in childhood psoriasis, and compare our clinical findings with the few reports available in the literature. Two hundred and one consecutive new patients with childhood (age , 16 years) psoriasis of both sexes were selected for the study of nail changes. The diagnosis of psoriasis was made on clinical grounds. Each patient underwent a thorough dermatologic examination with special attention paid to the nail changes. If a clinical suspicion of fungal infection of the nails existed, further mycologic investigations were performed. We found the prevalence of nail changes to be 37.81% (boys > girls) in children who had psoriasis. Nail pitting was found to be the most common manifestation (61.84%) followed by onycholysis (30.26%), subungual hyperkeratosis (13.16%), and discoloration of the nail plate (7.90%). Nail involvement had no relationship to the type of psoriasis, patient's sex, or duration or extent of disease. [source] Incidence of Kawasaki disease in Japan: the nationwide surveys of 1999,2002PEDIATRICS INTERNATIONAL, Issue 4 2006HIROSHI YANAGAWA Abstract Objective: The purpose of the present study was to describe the results of nationwide epidemiologic surveys of Kawasaki disease for the 4 year period 1999,2002. Methods: The design is a retrospective incidence survey. The patients reported in these two surveys are all new patients who were reported during the two study periods (1999,2000 and 2001,2002), although the data were collected retrospectively. A questionnaire was sent to all pediatric departments of hospitals with 100 beds or more throughout Japan, requesting data on patients with Kawasaki disease, such as sex, age, date of first hospital visit, recurrence, and cardiac lesions. Results: The total number of patients reported in the 4 year period 1999,2002 was 32 266 (18 604 male, 13 662 female), with an average annual incidence of 137.7 per 100 000 children younger than 5 years old. The male/female ratio was 1.30. The incidence peaked at 9,11 months of age, and the proportion of patients under 1 year of age was 26%. The monthly distribution had a high peak in January and a gradual increase in summer. Geographically, the high-incidence areas were limited to certain prefectures and moved from year to year. The cardiac lesions at acute stage and cardiac sequelae occurred more in children under 1 year and older than 4 years. Among the principal symptoms, fever persisting ,5 days occurred most commonly, followed by conjunctival congestion, changes in lips and oral cavity, polymorphous exanthema, and changes of extremities. Cervical lymphadenopathy occurred less. Conclusion: More than 32 000 patients with Kawasaki disease during the 4 year period 1999,2002 were reported to the nationwide incidence surveys. The number of patients is steadily increasing despite the decrease of children. The seasonal variation, geographical distribution, and age-specific distribution support the infection theory for the etiology of Kawasaki disease. [source] Constructing a patient education system: A performance technology projectPERFORMANCE IMPROVEMENT, Issue 4 2009Edith E. Bell The purpose of the patient education system described here was to distribute patient education material to and within medical practices managed by a small medical practice management company. The belief was that patient education opportunities improved health care outcomes and increased patient participation in health care decisions and compliance with health care plans. This tool reinforced medical practices' commitment to having patients participate actively in their treatment, differentiated them from other practices, and contributed to the generation of new patients. [source] Holoprosencephaly and agnathia spectrum: Presentation of two new patients and review of the literature,,AMERICAN JOURNAL OF MEDICAL GENETICS, Issue 1 2010Emily F. Kauvar§ Abstract Holoprosencephaly (HPE), the most common developmental disorder of the human forebrain, is occasionally associated with the spectrum of agnathia, or virtual absence of the mandible. This condition results in a constellation of structural cerebral and craniofacial abnormalities. Here we present two new patients and review 30 patients from the literature with HPE and variants of agnathia. The majority of these patients are female and have the most severe forms of HPE, with cyclopia present more frequently than is usually observed in cohorts of patients with HPE. Also, many patients have additional clinical findings not typical in patients with classic HPE, particularly situs abnormalities. Recent animal studies suggest that the association of HPE and agnathia may relate to alterations in signaling from forebrain and foregut endoderm organizing centers and subsequent first pharyngeal arch development, although present models are inadequate to explain all of the clinical findings of this enigmatic human syndrome. Further research is required to better elucidate the causal and pathogenic basis of this association. Published 2010 Wiley-Liss, Inc. [source] Holoprosencephaly and ectrodactyly: Report of three new patients and review of the literature,,AMERICAN JOURNAL OF MEDICAL GENETICS, Issue 1 2010Amelia A. Keaton§ Abstract Holoprosencephaly (HPE) and ectrodactyly represent congenital malformations of the developing forebrain and developing digits, respectively. The combination of these conditions is rare, with only 15 cases known to date (12 previously reported, and 3 new cases described here). While the findings in these patients overlap with previously described genetic conditions, the similarity in phenotypes among these patients has led to the establishment of a at least one distinct syndrome: HPE, ectrodactyly, and bilateral cleft lip-palate syndrome (OMIM 300571). There has been great interest in identifying a genetic cause for the findings in patients with HPE and ectrodactyly; however the cause(s) of this rare association still remain unknown. Published 2010 Wiley-Liss, Inc. [source] FGFR3 protein expression and its relationship to mutation status and prognostic variables in bladder cancer,THE JOURNAL OF PATHOLOGY, Issue 1 2007DC Tomlinson Abstract FGFR3 is frequently activated by mutation in urothelial carcinoma (UC) and represents a potential target for therapy. In multiple myeloma, both over-expression and mutation of FGFR3 contribute to tumour development. To define the population of UC patients who may benefit from FGFR-targeted therapy, we assessed both mutation and receptor over-expression in primary UCs from a population of new patients. Manual or laser capture microdissection was used to isolate pure tumour cell populations. Where present, non-invasive and invasive components in the same section were microdissected. A screen of the region of the highest tumour stage in each sample yielded a mutation frequency of 42%. Mutations comprised 61 single and five double mutations, all in hotspot codons previously identified in UC. There was a significant association of mutation with low tumour grade and stage. Subsequently, non-invasive areas from the 43 tumours with both non-invasive and invasive components were analysed separately; 18 of these had mutation in at least one region, including nine with mutation in all regions examined, eight with mutation in only the non-invasive component and one with different mutations in different regions. Of the eight with mutation in only the non-invasive component, six were predicted to represent a single tumour and two showed morphological dissimilarity of fragments within the block, indicating the possible presence of distinct tumour clones. Immunohistochemistry showed over-expression of FGFR3 protein in many tumours compared to normal bladder and ureteric controls. Increased expression was associated with mutation (85% of mutant tumours showed high-level expression). Overall, 42% of tumours with no detectable mutation showed over-expression, including many muscle-invasive tumours. This may represent a non-mutant subset of tumours in which FGFR3 signalling contributes to the transformed phenotype and which may benefit from FGFR-targeted therapies. Copyright © 2007 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. [source] The Vermont Model for Rural HIV Care Delivery: Eleven Years of Outcome Data Comparing Urban and Rural ClinicsTHE JOURNAL OF RURAL HEALTH, Issue 2 2010Christopher Grace MD Abstract Context: Provision of human immunodeficiency virus (HIV) care in rural areas has encountered unique barriers. Purpose: To compare medical outcomes of care provided at 3 HIV specialty clinics in rural Vermont with that provided at an urban HIV specialty clinic. Methods: This was a retrospective cohort study. Findings: Over an 11-year period 363 new patients received care, including 223 in the urban clinic and 140 in the rural clinics. Patients in the 2 cohorts were demographically similar and had similar initial CD4 counts and viral loads. There was no difference between the urban and rural clinic patients receiving Pneumocystis carinii prophylaxis (83.5% vs 86%, P= .38) or antiretroviral therapy (96.8% vs 97.5%, P= .79). Both rural and urban cohorts had similar decreases in median viral load from 1996 to 2006 (3,876 copies/mL to <50 copies/mL vs 8,331 copies/mL to <50 copies/mL) and change in percent of patients suppressed to <400 copies/mL (21.4%-69.3% vs 16%-71.4%, P= .11). Rural and urban cohorts had similar increases in median CD4 counts (275/mm3 -350/mm3 vs 182 cells/mm3 -379/mm3). A repeated measures regression analysis showed that neither fall in viral load (P= .91) nor rise in CD4 count (P= .64) were associated with urban versus rural site of care. Survival times, using a Cox proportional hazards model, were similar for urban and rural patients (hazard ratio for urban = 0.80 [95% CI, 0.39-1.61; P= .53]). Conclusions: This urban outreach model provides similar quality of care to persons receiving care in rural areas of Vermont as compared to those receiving care in the urban center. [source] Eczema workshops reduce severity of childhood atopic eczemaAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 2 2009Elizabeth J Moore ABSTRACT An intervention study was conducted to assess the effectiveness of a nurse-led eczema workshop in reducing the severity of atopic eczema in infants, children and adolescents. Ninety-nine new patients referred to the Dermatology Department of The Royal Children's Hospital in Melbourne, Australia, for the management of atopic eczema were randomized to receive care from an eczema workshop or a dermatologist-led clinic. Patients were followed-up 4 weeks after the intervention. The primary outcome was the severity of eczema as determined by scores obtained using the Scoring of Atopic Dermatitis (SCORAD) index at a 4-week follow-up visit. The secondary outcome was a comparison of treatments used in both clinics. At the 4-week review the mean improvement in SCORAD was significantly greater in those patients attending the eczema workshop than those attending the dermatologist-led clinic (,9.93, 95% confidence interval ,14.57 to ,5.29, P < 0.001). Significantly more patients from the eczema workshop improved from moderate severity eczema at baseline to mild at review. There was greater adherence to eczema management in the eczema workshop compared with the dermatologist-led clinic. In this study, patients attending the eczema workshop had a greater improvement in eczema severity thanpatients attending a dermatologist-led clinic, supporting collaborative models of service provision. [source] Developing a strategy to reduce the high morbidity of patients with long-term urinary catheters: the BioMed catheter research clinicBJU INTERNATIONAL, Issue 6 2007Azhar A. Khan OBJECTIVE To assess the idea of managing patients having problems with long-term catheterization (LTC, normally used when all other methods of bladder management have failed or are unsuitable) in a dedicated clinic, to present a prospective analysis of consecutive new patients attending between February 2002 and October 2006, and to establish the incidence of bladder stones in patients who have recurrent catheter encrustation and blockage. PATIENTS AND METHODS Patients treated with LTC are a large heterogeneous group, mainly consisting of elderly people who have chronic disabilities, and catheter-associated complications occur in > 70% of them. In all, 260 consecutive new patients having problems with LTC were assessed; the evaluation consisted of basic demographics, a detailed history, clinical examination, urine analysis and flexible cystoscopy (FC) via the catheterization route. Patients with bladder stones were screened with FC for recurrence of stones at 3, 6 and 12 months after treatment. RESULTS In all, 117 men and 143 women (mean age 67.7 years, range 23,97) were assessed; 147 (55.5%) had catheter encrustation. FC showed that 66 of the 147 patients (45%) had bladder stones. Forty-eight patients (73%) were successfully treated at the same clinic appointment and their stones were removed with the help of a tip-less stone basket. Eighteen patients (27%) were referred for inpatient treatment of bladder stones under general anaesthesia. Twenty of 66 patients with bladder stones (30%) formed recurrent bladder stones at a mean (range) follow-up of 8.1 (3,18 months). In addition, 36 patients had successful insertion of suprapubic catheter (SPC) under local anaesthetic in the clinic, and 11 were referred for SPC insertion under general anaesthesia. Two patients were diagnosed with bladder transitional cell carcinoma. CONCLUSION The introduction of a dedicated catheter clinic, equipped with facilities such as FC and a hoist, enables patients to be treated in an environment that meets their needs and potentially reduces the risk of more complex stone removal and catheter problems at a later date. It can also act as a potential source of data for use in research and development. A significant proportion (45%) of patients with catheter encrustation and blockage had formed bladder stones. Our study provides a rationale for FC of all such patients to detect and remove stones. [source] Dermatology outpatient case-mix survey for all Welsh Trusts, 2007BRITISH JOURNAL OF DERMATOLOGY, Issue 1 2010G.M. Hill Summary Background, In 2006 a U.K. government White Paper recommended making NHS care in England more accessible by shifting services from secondary care into community settings. There is a shortage of contemporary activity data for U.K. dermatology units to allow benchmarking for service development. This study will not only provide useful comparative data for the future in Wales, but will also serve to highlight the impact of changes made in England. Objective, To provide an overview of 1 week's dermatology outpatient activity for the whole of Wales. Methods, All dermatology units in Wales collected data for 1 week in early 2007. The case mix, appropriateness of referral, requirement for surgery or second-line therapies and follow-up requirements were all determined. Results, A total of 2142 patients were seen. Of new patients, 21% had skin cancer. Seventeen per cent of skin cancers had no diagnosis suggested by the general practitioner (GP) and 10% of basal cell carcinomas, 33% of squamous cell carcinomas and 17% of malignant melanomas were inappropriately diagnosed. In all, 26% of new patients had benign lesions, and this group caused the greatest diagnostic difficulty for GPs. Seventy-one per cent of these patients were diagnosed, reassured and discharged at their first visit without the need for biopsy or surgery. Thirty-seven per cent of new patients required surgery, of which 21% required complex intervention. Twenty-six per cent of follow-up patients were receiving second-line therapies. The new to follow-up ratio varied considerably according to diagnosis, the mean ratio being 1 : 0·21 for benign lesions through to 1 : 5·53 for psoriasis. This highlights the inappropriate nature of a ,one fits all' ratio. The majority of follow-up patients in secondary care required this level of input for monitoring of cancer, complex second-line therapies or surgery. Conclusions, This study provides evidence to support logical planning of dermatological services and to assess the impact of proposed changes on different healthcare systems in the U.K. [source] The challenge of follow-up in narrowband ultraviolet B phototherapyBRITISH JOURNAL OF DERMATOLOGY, Issue 2 2007B.L. Diffey Summary Background, The use of narrowband ultraviolet (UV) B phototherapy to treat psoriasis and other disorders has increased markedly since the TL-01 lamps were introduced in the 1980s. While broadband UVB phototherapy has generally been considered to be a relatively safe treatment, some concern has been raised about the potential increased skin cancer risk with narrowband UVB. Objectives, The likelihood of a patient who is free of nonmelanoma skin cancer (NMSC) at the start of phototherapy developing a malignancy after a certain follow-up period will be dependent not only on the carcinogenic potential of the treatment but also on the age-conditional probability of natural occurrence. We were interested to explore the potential difficulty of designing studies to separate these two events. Methods, Mathematical models were developed that combined age-conditional probabilities of developing NMSC due to natural causes with the risk of inducing these cancers from narrowband UVB phototherapy in order to estimate the excess number of cancers resulting from this therapeutic intervention in a cohort of patients. Results, Within-department studies will be most unlikely to demonstrate that the number of NMSCs observed in follow-up studies is significantly different from that expected in an untreated population, even for a follow-up period of 20 years. Conclusions, Determination of the carcinogenic potential associated with narrowband UVB will require large multicentre studies typically involving several thousand new patients per year and followed up for 10 years or more. [source] Prevalence of familial malignancy in a prospectively screened cohort of patients with lymphoproliferative disordersBRITISH JOURNAL OF HAEMATOLOGY, Issue 3 2008Jennifer R. Brown Summary Increasing evidence points to a heritable contribution in the development of lymphoma. The goal of this study was to determine the rate of familial lymphoproliferative malignancy among consecutive lymphoma patients presenting to a tertiary care center and to enrol families with multiple affected first-degree relatives on a data and tissue collection study. Beginning in 2004 all new patients presenting to the Dana-Farber Cancer Institute with non-Hodgkin (NHL) or Hodgkin lymphoma (HL) or chronic lymphocytic leukaemia (CLL) were asked to complete a one-page self-administered family history questionnaire. 55·4% of 1948 evaluable patients reported a first-degree relative with a malignancy, with the highest rate among CLL probands. Lymphoid malignancies were particularly common, with 9·4% of all probands reporting a first-degree relative with a related lymphoproliferative disorder (LPD). This frequency was again highest for CLL, at 13·3% of CLL probands, compared to 8·8% of NHL probands and 5·9% of HL probands (P = 0·002). The prevalence of CLL was significantly increased in parents of CLL probands (P < 0·05), and a greater risk of NHL was seen in fathers of NHL probands than in mothers (P = 0·026). We conclude that familial aggregation of LPDs is common among newly diagnosed patients, varies significantly by diagnosis and contributes meaningfully to the population disease burden. [source] Low-dose cyclophosphamide conditioning for haematopoietic cell transplantation from HLA-matched related donors in patients with Fanconi anaemiaBRITISH JOURNAL OF HAEMATOLOGY, Issue 1 2005J. Zanis-Neto Summary Allogeneic haematopoietic cell transplantation (HCT) is effective therapy for Fanconi anaemia (FA). FA patients do not tolerate conditioning with 200 mg/kg of cyclophosphamide (Cy), typically used in aplastic anaemia. We previously published results of studies in which Cy doses were gradually reduced from 200 to 100 mg/kg. Here we update results of the initial studies and report data on 30 new patients conditioned with Cy either at 80 mg/kg (n = 7) or at 60 mg/kg (n = 23), given over 4 days before HCT from human leucocyte antigen-matched related donors. Methotrexate and cyclosporine were given for graft- versus -host disease (GVHD) prophylaxis. All seven patients given Cy at 80 mg/kg and 21 of 23 given Cy at 60 mg/kg had sustained engraftment, while two patients, both with clonal cytogenetics abnormalities, experienced graft failure. Grades 2,3 acute GVHD rates were 57% and 14% for patients given the higher and lower Cy doses, respectively (P = 0·001). Four patients given Cy at 80 mg/kg and 22 given Cy at 60 mg/kg were alive at a median of 47 (44,58) months and 16 (3,52) months, respectively. Cy at 60 mg/kg has acceptable toxicities, low rates of GVHD, and is sufficient for engraftment of related grafts in most FA patients. [source] | ||||||||||||||||||||||||||||||||||