Spondyloarthropathies in Japan: nationwide questionnaire survey performed by the Japan Ankylosing Spondylitis Society

OBJECTIVE: The Japan Ankylosing Spondylitis Society conducted a nationwide questionnaire survey of spondyloarthropathies (SpA) in 1990 and 1997, (1) to estimate the prevalence and incidence, and (2) to validate the criteria of Amor and the European Spondylarthropathy Study Group (ESSG) in Japan. METHODS: Japan was divided into 9 districts, to each of which a survey supervisor was assigned. According to unified criteria, each supervisor selected all the clinics and hospitals with potential for SpA patients in the district. The study population consisted of all patients with SpA seen at these institutes during a 5 year period (1985-89) for the 1st survey and a 7 year period (1990-96) for the 2nd survey. RESULTS: The 1st survey recruited 426 and the 2nd survey 638 cases, 74 of which were registered in both studies. The total number of patients with SpA identified 1985-96 was 990 (760 men, 227 women). They consisted of patients with ankylosing spondylitis (68.3%), psoriatic arthritis (12.7%), reactive arthritis (4.0%), undifferentiated SpA (5.4%), inflammatory bowel disease (2.2%), pustulosis palmaris et plantaris (4.7%), and others (polyenthesitis, etc.) (0.8%). The maximum onset number per year was 49. With the assumption that at least one-tenth of the Japanese population with SpA was recruited, incidence and prevalence were estimated not to exceed 0.48/100,000 and 9.5/100,000 person-years, respectively. The sensitivity was 84.0% for Amor criteria and 84.6 for ESSG criteria. CONCLUSION: The incidence and prevalence of SpA in Japanese were estimated to be less than 1/10 and 1/200, respectively, of those among Caucasians. The adaptability of the Amor and ESSG criteria was validated for the Japanese population.     

太原市睡眠呼吸暂停低通气综合征的流行病学调查

目的　了解太原市睡眠呼吸暂停低通气综合征 (SAHS)的患病率 ,为今后的预防诊治工作奠定基础。方法　从 1998年 1月至 2 0 0 1年 12月 ,用整群随机抽样法 ,以太原市两个城区四个居住小区的家庭常住人口为调查对象 ,进行入户问卷调查 ,总人数为 6 0 2 8名 (其中男 376 5名 ,女 2 2 6 3名 )。对所有人进行问卷Ⅰ调查 ,其中打鼾 3级以上的 10 2 4名 ,由医务人员进行再次入户问卷Ⅱ调查 ,对仍可疑 (ESS评分≥ 9)的 4 76名进行多导睡眠监测 ,从而推算出太原市SAHS的患病率。结果　实际完成调查人数为 5 12 8名 (其中男 2 90 1名 ,女 2 2 2 7名 ,问卷应答率为 85 11% ) ,确诊为SAHS患者共 179例 (男性 136例 ,女性 4 3例 ) ,患病率为 3 5 % (男性为 4 7% ,女性为 1 9% ) ,男性患病率显著高于女性 (P <0 0 1) ,其发病高峰年龄为 30～ 5 0岁。结论　此次调查推算出太原市SAHS的患病率为3 5 % ,可见SAHS是影响公众健康的疾病之一 ,应引起医务工作者的重视     

我国肺功能检测应用现状的调查和分析

目的：对肺功能检测在我国的应用现状进行首次调查。方法：对我国29个省，直辖市，自治区212家医院进行问卷调查。结果：检测回函率为81．6％，已开展肺功能检测为91．9％，其中三级医院为69．2％，肺功能检测在20世纪70年代后期开展较快，90年代产期达到高峰，国产肺功能检测仪仅为12．4％，进口检测仪为87．6％，平均每月肺功能应用次数及每病床使用数总体较低，肺功能检测项目和临床应用在三级和二级医院间有较大差异，肺功能在内，外科应用较为广泛，儿科，妇科，耳鼻喉科等应用较少，检测的基本项目是通气功能，所有已开展肺功能检测的医院都已开展，支气管扩张试验（74．2％）和激发试验（65．4％）应用也较为广泛，肺容量（60．0％），弥散（58．2％）及气道阻力（42．7％）测定分别在三级医院开展，但二级和以下医院开展较少，运动心肺功能试验在三级医院中只有16．4％的医院开展，三级以下医院均为空白，对所选用的肺功能正常预计值能明确说明的医院只有21．4％，能明确适用适于儿童正常值的医院只占开展儿科检测的16．9％，引用国人预计值的医院仅16．3％，结论：我国肺功能检测发展仍不平衡，对肺功能检测临床应用的认识有待加强，肺功能仪器国产化亟待加强，肺功能检测应更广泛的开展（对象和项目），并应重视正确的选用肺功能预计值。     

Childhood vasculitides in Turkey: a nationwide survey

Aim

The aims of this study were to evaluate the characteristics of childhood vasculitides and to establish the first registry in Turkey, an eastern Mediterranean country with a white population.

Patients and methods

A questionnaire was distributed to the main referral centers asking for the registration of the Henoch-Schönlein purpura (HSP) patients in the last calendar year only and 5 years for other vasculitides. Demographic, clinical, and laboratory data were assessed.

Results

Vasculitic diseases were registered from 15 pediatric centers. These centers had a fair representation throughout the country. In the last calendar year, incidences were as follows: HSP 81.6%, Kawasaki disease (KD) 9.0%, childhood polyarteritis nodosa (C-PAN) 5.6%, Takayasu arteritis (TA) 1.5%, Wegener’s granulomatosis 0.4%, and Behçet disease 1.9%. There was no clear gender dominance. The mean age was 11.05±4.89 years. Acute phase reactants were elevated in almost all, highest figures being in C-PAN. Renal involvement was present in 28.6% of HSP and 53% of the C-PAN patients. Abdominal aorta was involved in all TA patients. Among the C-PAN patients, 25% had microscopic PAN with necrotizing glomerulonephritis; antineutrophil cytoplasmic antibody was positive in those who were studied. Among the patients, 12.5% and 15% had classic PAN and cutaneous PAN, respectively. The remaining majority were classified as systemic C-PAN diagnosed with biopsies and/or angiograms demonstrating small to midsize artery involvement. The overall prognosis was better than reported in adult series.

Conclusion

This is the largest multicenter study defining the demographic data for childhood vasculitides. The distribution of childhood vasculitides was different in our population where KD is much less frequent, whereas HSP constitutes an overwhelming majority. C-PAN was more frequent as well.     

Blood transfusion practice: a nationwide survey in Italy

Background

Blood transfusion is a complex activity. Some of the components of this activity are implementation of standard procedures, evaluation of appropriateness of the blood use, methods for bedside identification of patients and the release of blood in emergencies, during out-of-routine hours and from hospitals lacking a Blood Centre. An overview about how these issues are managed in Italy could be of interest.

Materials and methods

A survey dealing with some issues regarding blood release was performed, using a questionnaire sent to 278 Italian Blood Centres.

Results

Out of the 278 Centres, 179 (64%) returned the questionnaire. The geographic distribution of the Centres that responded (80 in the north, 46 in the centre and 53 in the south of Italy) offers a good picture of the Italian reality. Globally there seems to be a relatively uniform application of procedures and guidelines, of methods to identify patients, and of evaluating transfusion appropriateness. The systems used to deal with emergency blood release and blood release in non-routine conditions are more variable. The use of technological resources seems to be disappointingly low.

Discussion

Although many aspects of the blood transfusion process should be improved, the picture that emerged from the survey seems to show, on the whole, a fair quality of blood transfusion practice in Italian Blood Centres.     

Surveillance colonoscopy practice in Lynch syndrome in the Netherlands： A nationwide survey

Lynch syndrome, or hereditary nonpolyposis colorectal cancer (HNPCC), is the most common genetic disorder predisposing to colorectal cancer. As regular colonoscopic surveillance has been shown to reduce the incidence of colorectal cancer, this strategy is recommended worldwide. Recently, several advances in colonoscopic techniques have improved detection rates of neoplasia in Lynch syndrome. In this nationwide survey, we evaluated current surveillance colonoscopy practices for Lynch syndrome in the Netherlands and the extent to which advanced techniques have been adopted in routine clinical practice.     

Turner's syndrome in adulthood

Turner's syndrome is the most common chromosomal abnormality in females, affecting 1:2,500 live female births. It is a result of absence of an X chromosome or the presence of a structurally abnormal X chromosome. Its most consistent clinical features are short stature and ovarian failure. However, it is becoming increasingly evident that adults with Turner's syndrome are also susceptible to a range of disorders, including osteoporosis, hypothyroidism, and renal and gastrointestinal disease. Women with Turner's syndrome have a reduced life expectancy, and recent evidence suggests that this is due to an increased risk of aortic dissection and ischemic heart disease. Up until recently, women with Turner's syndrome did not have access to focused health care, and thus quality of life was reduced in a significant number of women. All adults with Turner's syndrome should therefore be followed up by a multidisciplinary team to improve life expectancy and reduce morbidity.     

Treatment for childhood-onset Graves' disease in Japan: results of a nationwide questionnaire survey of pediatric endocrinologists and thyroidologists.

OBJECTIVE AND DESIGN: To determine the present condition of treatment of childhood-onset Graves' disease in Japan, a nationwide questionnaire survey was conducted among councilors of the Japanese Society for Pediatric Endocrinology and the Japan Thyroid Association. MAIN OUTCOME: Responses were received from 125 individuals, and the rate of collection of questionnaires was 47%. Methimazole was selected for first-line initial antithyroid drug therapy by 92% of respondents. Antithyroid drugs tended to be given at larger initial doses and over longer periods of time to childhood-onset patients than to adult patients, and these tendencies were more pronounced for pediatric endocrinologists. Combination therapy with an antithyroid drug and thyroxine was used more frequently by pediatric endocrinologists. Thyroidologists had more experience with radioiodine therapy than pediatric endocrinologists. Opinions regarding preparation of guidelines for the initial dose of methimazole in childhood-onset Graves' disease were almost equally divided among the following: the dose of methimazole should be adjusted according to the severity of disease as in adult cases, methimazole should be started at a dose of 1mg/kg per day in all patients, and the dose should be determined based on results of a randomized study. CONCLUSIONS: The present condition of treatment of childhood-onset Graves' disease in Japan was clarified.     

Risk for sleep apnea syndrome in Pakistan: a cross-sectional survey utilizing the Berlin questionnaire

Purpose  The purpose of this study was to assess the prevalence of individuals at high risk for developing obstructive sleep apnea in the Pakistani population using a validated questionnaire. Materials and methods  This is a cross-sectional survey using a pre-validated, interviewer-administered questionnaire conducted at the Aga Khan University Hospital. All healthy individuals above 18 years of age attending a seminar were included as participants after consent. Results  The percentage of people in the high-risk group was calculated to be 12.4% out of a population of 137. Presence of snoring, hypertension, and body mass index >27.5 kg/m² was found to be more prevalent in high-risk individuals. Conclusions  A significant proportion of the population is at high risk for obstructive sleep apnea syndrome.     

Subchondral insufficiency fracture with rapid collapse of the femoral head in a patient with Turner's syndrome

SIR, Primary subchondral insufficiency fracture (SIF) has beendescribed infrequently in the limited literature since 1999[1–4]. In the past it had always been misdiagnosed asosteonecrosis (ON). SIF of the femoral head characteristicallypresents as an acute painful hip disability without a historyof obvious antecedent trauma, steroid therapy or alcoholism.It has been identified in elderly obese women and renal transplantpatients [1]. We describe here a patient with Turner's syndrome(TS) and consequent generalized osteopenia whose femoral headssuccessively underwent rapid collapse within 3 months. A 31-yr-old woman visited     

Etiology of liver cirrhosis in Japan: a nationwide survey

Background  

Little is understood about worldwide changes in the epidemiological distribution of the etiology of liver cirrhosis (LC). The present study examines the etiology of liver cirrhosis in Japan using a nationwide survey.     

Predisposing factors in polymyositis-dermatomyositis: results of a nationwide survey

Utilizing case-control methods, we surveyed 322 persons who experienced the onset of polymyositis-dermatomyositis (PM-DM) during calendar years 1985 and 1986, in an effort both to test specific hypotheses, and to generate new hypotheses regarding antecedent risk associations for the disease. Data obtained by recall of events during the 12 months before disease onset in cases, and an identical time period in sex matched sibling controls, suggest that important questions for further study include the roles of antecedent heavy muscular exertion and emotional stress as risk factors for PM-DM. Furthermore, it appears that vaccinations, toxic exposures and symptoms of allergic phenomena, throat infection, and upper respiratory infection are either neutral or are negatively associated with risk of PM-DM, findings which also raise important new etiologic questions, or perhaps lay old ones to rest. Malignancy was infrequently noted.     

Musculoskeletal education in general practice: a questionnaire survey

Musculoskeletal education in primary care has previously been shown, in 1995, to be inadequate [1]. The aims of this study were to evaluate the current musculoskeletal education and skills during vocational training for general practice and to see if progress has been made. Questionnaires were sent to General Practice Registrars, in general practice attachments in June 2004. Four UK General Practice Deaneries participated (Northern, Mersey, Yorkshire and Wessex). Questionnaires were received from 251 (44 %) registrars. Of the responders, only 77 % reported receiving specific clinical rheumatology teaching at medical school and 30 % had not received any tutorials on musculoskeletal conditions during their vocational training. Of the registrars, 16 % reported having completed a rheumatology post, and an additional 19 % had been able to attend rheumatology outpatient clinics; 70 % of the registrars had injected or aspirated the knee although less than half of these (22 %) had done this in a primary care setting. Lack of experience was associated with low confidence at knowing when to perform the injection and with performing the injection itself. A significant proportion of registrars reported being pre-dominantly self-taught for performing injections (soft tissue?=?10.7 %, joint injections?=?8.7 %) and for the management of shoulder pain (20.1 %). Registrars rated their overall musculoskeletal training as inadequate. Primary care musculoskeletal education remains inadequate and needs to be improved to enable registrars to be confident in managing a significant proportion of their workload. Identifying learning needs for primary care would inform future educational interventions.     

Prevalence of metabolic syndrome and its risk factors in adult Malaysians: results of a nationwide survey

Aim

To report the national prevalence of metabolic syndrome (MetS) and its risk factors among adult Malaysians (>18 years old) based on World Health Organization (WHO), the National Cholesterol Education Program Expert Panel III (ATP III)^, International Diabetes Federation (IDF) and the ‘Harmonized’ criteria.

Methods

A multi-stage stratified sampling method was used to select 4341 subjects from Peninsular and East Malaysia. Subjects underwent physical and clinical examinations.

Results

Based on the WHO, ATP III, IDF and Harmonized definitions, the overall crude prevalences of MetS were 32.1, 34.3, 37.1 and 42.5%, respectively. Regardless of the criteria used, MetS was higher in urban areas, in females, in the Indian population and increased significantly with age. Risk factors also increased with age; abdominal obesity was most prevalent (57.4%), was higher in females (64.2%) and was highest in Indians (68.8%). Hypertension was higher in males (56.5%) and highest among Malaysians (52.2%). In contrast, the Chinese had the highest prevalence of hypertriglyceridaemia (47.4%).

Conclusions

Malaysia has a much higher prevalence of MetS compared with other Asian countries and, unless there is immediate intervention to reduce risk factors, this may pose serious implications on the country's healthcare costs and services.