Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus

OBJECTIVE: To investigate potential differences between childhood-onset and adult-onset systemic lupus erythematosus (SLE). METHODS: An inception cohort with childhood-onset SLE (n = 67) was compared with an inception cohort with adult-onset SLE (n = 131), each of whom was diagnosed between 1990 and 1998 and followed up until February 1999. Prospective information included data on medications, laboratory markers, and disease activity and damage as measured by the SLE Disease Activity Index (SLEDAI) and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI), respectively. RESULTS: Eighty-five percent of patients with childhood-onset SLE and 88% of patients with adult-onset SLE were female; the mean duration of followup was 3.2 and 3.5 years, respectively. On average, the children had more-active disease than did the adults at the time of diagnosis and during followup. There was a higher incidence of renal disease in those with childhood-onset SLE (78% versus 52% in adults; P = 0.0005), and the adjusted mean SLEDAI renal score was higher in the children than in the adults (2.37 versus 0.82; P < 0.0001). Treatment with steroids (97% versus 72%; P < 0.0001) and immunosuppressive drugs (66% versus 37%; P = 0.0001) was used significantly more often in children with SLE. Four adult patients with SLE, but none of the children, died during the followup. At the end of the followup, the mean SDI scores in those with childhood-onset SLE were higher than those with adult-onset SLE (1.70 versus 0.76; P = 0.008). CONCLUSION: Children with childhood-onset SLE have more active disease at presentation and over time than do adults with SLE, especially active renal disease. Compared with adults with SLE, children receive more intensive drug therapy and accrue more damage, often related to steroid toxicity.     

Mechanical and metabolic profile of locomotion in adults with childhood-onset GH deficiency

OBJECTIVE: The aim of the present study was to evaluate the energy cost and the mechanical work of locomotion in a group of adults with childhood-onset GH deficiency (GHD). SUBJECTS: Eight males with childhood-onset GHD (mean age+/-s.d.: 31.7+/-3.6 years; mean height: 145.1+/-6.7cm) and six age-, sex- and exercise-matched normal subjects were studied. DESIGN: GHD patients and healthy controls were requested to walk and run in the speed range of 2-11km h(-1). For each condition, simultaneous mechanical and metabolic measurements were taken. METHODS: Oxygen consumption, and mechanical internal and external work of locomotion were evaluated with standard open-circuit respirometry and three-dimensional motion analysis respectively. RESULTS: External work was not significantly different between GHD patients and healthy controls, while internal work was higher for patients at all speeds. In walking, the relationships between both the mechanical energy recovery and the metabolic cost with speed were shifted towards lower speeds in patients. As a consequence, the optimal speed of walking, i.e. the speed at which the cost of locomotion is minimum, was lower for GHD patients. Stride frequency was significantly higher (11.2-11.3%) for GHD patients at all speeds of walking and running. GHD patients were unable to run at speeds higher than 8km h(-1) for the time needed to reach a metabolic steady state. CONCLUSION: It appears that both the mechanics and energetics of locomotion in short-statured adults with childhood-onset GHD are not strikingly different from those of healthy controls, thus demonstrating a substantial 'normality' in this group of GHD patients at metabolically attainable speeds. The 'harmonic' body structure and the adherence to allometric transformations in these patients do not exclude the possibility of a different metabolic role of GH in normally statured adults with childhood-onset GHD and in those with acquired GHD, taking into account the well recognized heterogeneity of the adult GHD syndrome.     

Epidemiological, endocrine and metabolic features in Turner syndrome

Turner syndrome is one of the more common genetic disorders, associated with abnormalities of the X chromosome, and occurring in about 50 per 100 000 liveborn girls. Turner syndrome is usually associated with reduced adult height, gonadal dysgenesis and thus insufficient circulating levels of female sex steroids, and infertility. A number of other signs and symptoms are seen more frequently with the syndrome. Morbidity and mortality are increased. The average intellectual performance is within the normal range. A number of recent studies have provided new insights with respect to epidemiology, cardiology, endocrinology and metabolism. Treatment with GH during childhood and adolescence allows a considerable gain in adult height, although very-long-term consequences of this treatment are not clear. Puberty has to be induced in most cases, and female sex hormone replacement therapy is given during the adult years. The proper dose of hormone replacement therapy (HRT) has not been established, and, likewise, benefits and/or drawbacks from HRT have not been thoroughly evaluated. Since the risk of cardiovascular and endocrinological disease is clearly elevated, proper care during adulthood is emphasized. In summary, Turner syndrome is a condition associated with a number of diseases and conditions which are reviewed in the present paper.     

Epidemiological, endocrine and metabolic features in Turner syndrome

Turner syndrome is one of the more common genetic disorders, associated with abnormalities of the X chromosome, and occurring in about 50 per 100,000 liveborn girls. Turner syndrome is usually associated with reduced adult height, gonadal dysgenesis, and thus insufficient circulating levels of female sex steroids, and infertility. A number of other signs and symptoms are seen more frequent with the syndrome. Morbidity and mortality is increased. The average intellectual performance is within the normal range. With respect to epidemiology, cardiology, endocrinology and metabolism a number of recent studies have allowed new insight. Treatment with GH during childhood and adolescence allows a considerable gain in adult height. Puberty has to be induced in most cases, and female sex hormone replacement therapy is given during adult years. The proper dose of HRT has not been established, and, likewise, benefits and/or drawbacks from HRT has not been thoroughly evaluated. Since the risk of cardiovascular and endocrinological disease is clearly elevated, proper care during adulthood is emphasized. In summary, Turner syndrome is a condition associated with a number of disease and conditions which are reviewed in present paper.     

Psoriasis: epidemiology, clinical features, and quality of life

Psoriasis is a common chronic, recurrent, immune mediated disease of the skin and joints. It can have a significant negative impact on the physical, emotional, and, psychosocial wellbeing of affected patients. Psoriasis is found worldwide but the prevalence varies among different ethnic groups. It has a strong genetic component but environmental factors such as infections can play an important role in the presentation of disease. There are several clinical cutaneous manifestations of psoriasis but most commonly the disease presents as chronic, symmetrical, erythematous, scaling papules and plaques. The epidemiology, clinical features, and impact on quality of life of psoriasis are reviewed.     

Prevalence and features of metabolic syndrome in childhood-onset systemic lupus erythematosus

To estimate the prevalence and features of metabolic syndrome (MetS) in childhood-onset systemic lupus erythematosus (cSLE), we performed a cross-sectional study of 76 consecutive cSLE patients and 54 healthy controls, age and sex matched. All individuals were assessed for anthropometric and MetS features according to World Health Organization (WHO), NCEP Adult Treatment Panel III (NCEP-ATP III), and International Diabetes Federation (IDF) criteria. The cSLE patients were further assessed for clinical and laboratory manifestations, disease activity (Systemic Lupus Erythematosus Disease Activity Index), cumulative damage (Systemic Lupus International Collaborating Clinics (SLICC)), and current and cumulative drug exposures. Sixty-nine (90.8%) patients were female with mean age of 16.8 years [standard deviation (SD) ±4.0 years]. Mean disease duration was 4.8 years (SD ± 4.1). Based on the WHO MetS criteria, MetS was observed in two (2.6%) cSLE patients. We observed high prevalence of the MetS in cSLE patients according to NCEP-ATP III MetS criteria (18.4%) (p = 0.002) and according to IDF MetS criteria (17.1%) (p = 0.003). We did not observe MetS in the control group. No difference in cSLE patients <18 and ≥18 years was observed. We observed an association between the presence of MetS and SLICC scores in cSLE <18 years and cumulative corticosteroid dose adjusted by weight in cSLE ≥18 years. This study showed that MetS is frequently observed in cSLE using NCEP-ATP III MetS criteria and IDF MetS criteria. The identification of MetS is important to indicate cardiovascular morbidity and mortality in cSLE.     

杭州市184例反流性食管炎的临床特点及生存质量分析

目的通过症状量表和汉化版SF-36生存质量量表研究杭州市1年内经胃镜诊断的反流性食管炎(RE)患者的临床特点和生存质量状况。方法收集2004年9月至2005年9月杭州市七家省市级医院消化科门诊就诊的RE患者184例,记录患者的临床特点和生存质量状况,并进行相关因素的分析。结果REA级和B级的患者明显多于C级和D级患者。性别、吸烟、饮酒以及RE分级对症状评分无显著影响(P>0.05),年龄对症状评分有显著影响(P<0.05)。RE患者在SF-36各个维度的积分均有下降。吸烟、饮酒、年龄、性别以及内镜下分级对生存质量量表综合得分影响无显著性意义(P>0.05)。患病时间、症状量表评分对生存质量量表综合得分影响有显著性意义(P<0.05)。结论杭州市RE患者病情多较轻,年龄对RE症状的严重程度有显著影响,症状的严重程度和患病时间是影响患者生存质量的主要因素。     

The German cystic fibrosis quality assurance project: clinical features in children and adults.

Cystic fibrosis (CF) is a complex disease which requires interdisciplinary care in specialized CF centres. In Germany, 97 paediatric and adult outpatient clinics agreed to report clinical data of their patients to a newly established registry, the Cystic Fibrosis Quality Assurance (CFQA) project. This article characterizes the design of the CFQA and the health status of the patients enrolled by the end of 1997. Data from 4,306 patients reported to the CFQA project were analysed. Nutritional status and lung function of the patients were examined as well as the use of specific therapeutic interventions. Mean age of all 4,182 patients alive by the end of 1997 was 15.7 yrs (maximum, 58 yrs), and 35.8% of patients were > 18.0 yrs of age. One-third of the CF population were treated in the nine largest centres (each caring for > 100 patients). Abnormal nutritional status (weight-for-height >90% of predicted or body mass index < 19.0 kg m2, respectively) was observed in 26.8% of children and adolescents and in 38.3% of adults. Lung function was abnormal (forced expiratory volume in one second < 80% predicted) in the majority of adults (83.9%) and in 42.5% of the younger patients. The mortality rate was 1.4 of 100 patients in 1997. No clear association of clinical status with centre size was observed. The clinical features of patients treated in German cystic fibrosis centres were generally comparable to those reported from other countries, although improvements are certainly warranted. The Cystic Fibrosis Quality Assurance project represents an important tool for future progress in the quality of cystic fibrosis care.     

Improvement in health-related quality of life with fluticasone propionate compared with budesonide or beclomethasone dipropionate in adults with severe asthma

OBJECTIVE: Changes in health-related quality of life (HRQoL) were evaluated in adults with severe asthma following inhaled corticosteroid treatment with high-dose beclomethasone dipropionate or budesonide (BDP/BUD) and compared with fluticasone propionate taken at approximately half the dose of BDP/BUD. METHODOLOGY: HRQoL was assessed as part of an open, multicentre, randomized, parallel-group study in Australia evaluating the safety and efficacy of switching to fluticasone propionate (FP) 1000-2000 micro g/day (n = 67) compared with remaining on BDP/BUD >/=1750 micro g/day (n = 66) for 6 months. Patients completed two HRQoL questionnaires, the Asthma Quality of Life Questionnaire (AQLQ) and the Medical Outcomes Study Short Form-36 (SF-36), at baseline and at weeks 12 and 24. A change in AQLQ score of >/=0.5 was considered to be clinically meaningful. RESULTS: There were significant improvements in HRQoL with FP on four of the eight dimensions on the SF-36 (i.e. physical functioning, general health, role-emotional, and mental health), while there were no significant improvements in HRQoL in the BDP/BUD group. Overall, patients in the FP group experienced significantly greater improvement (P < 0.001) in AQLQ scores at weeks 12 and 24 compared with the BDP/BUD group. On the individual domains of the AQLQ, there were significant treatment differences (P < 0.01) in favour of FP in three of the four domains (activity limitations [0.92], symptoms [0.73], and emotional function [1.02]). Mean differences between groups for overall score and these three domains were also clinically meaningful. CONCLUSION: Patients with severe asthma who received FP (at approximately half the dose of BDP/BUD) experienced statistically significant, as well as clinically meaningful, improvements in their HRQoL.     

合并代谢综合征的急性心肌梗死患者临床特征

目的 探讨合并代谢综合征(MS)的急性心肌梗死(AMI)患者的临床特征.方法 对81例合并MS的AMI患者及94例无MS的AMI患者进行回顾性研究,比较两组患者的基础临床情况、诱发因素、临床表现、梗死部位及心肌酶.结果与非 MS组相比,MS组年龄较大,女性患者所占比率较高(34.6%比17.0%,P＜0.01),吸烟和冠心病家族史比率较低(分别为43.2%比59.6%,13.6%比25.5%,均P＜0.05),饱餐后或情绪激动时发病率较高(分别为18.5%比7.4%,13.6%比4.3%,均P＜0.05),有胸痛者少(66.7%比81.9%,P＜0.05),心功能≥KillipⅡ级者较多(35.8%比22.3%,P＜0.05),住院病死率高(22.2%比10.6%.P＜0.05),前壁范围所占比率较高(51.9%比36.2%,P＜0.05),肌酸激酶同工酶较高[(147±19)U/L比(122±14)U/L,P＜0.01].结论 与无MS的AMI患者相比,合并MS的AMI患者年龄较大,女性患者较多,近期预后较差.     

Pregnancy outcomes in women with childhood-onset and adult-onset systemic lupus erythematosus: a comparative study

To compare the maternal and fetal outcomes between childhood-onset and adult-onset systemic lupus erythematosus (SLE), we reviewed the medical records of SLE pregnant women treated from January 2005 to August 2013. For comparison, patients were allocated to one of the two groups, those pregnant patients with SLE onset before 18 years of age (childhood-onset) and ≥18 years (adult-onset). The patients were evaluated at least once in each trimester and postpartum. Relevant maternal and fetal outcomes were extracted, such as lupus flare, preeclampsia/eclampsia, rate of liveborns, fetal loss (spontaneous abortion and stillbirth), term delivery, preterm birth, neonatal death, low birth weight, low birth weight at term, and congenital malformations. We studied 186 pregnancies (in 180 women), 58 of them had childhood-onset SLE, and the remaining 128 had adult-onset SLE. The rate of maternal and fetal complications was similar in both groups. Multivariate analysis showed that active SLE before pregnancy, primigravida, renal flare, preeclampsia, lupus flare, anticardiolipin antibodies, and low serum complement were associated with an increased risk of poor maternal and fetal outcomes. The diagnosis of childhood-onset had no impact on maternal–fetal outcome. The maternal and fetal outcome in women with childhood-onset SLE is similar to that reported in women with adult-onset SLE. Pregnancy in women with childhood-onset SLE should not be contraindicated if the disease is well controlled.     

Shigellosis in adults: epidemiologic, clinical, and laboratory features

One hundred and sixteen cases of adult patients hospitalized for shigellosis have been reviewed. The most common presentation included diarrhea (most frequently watery), fever, and abdominal pain, while dysentery has been less frequent. S. flexneri infection, as compared to S. sonnei, was more common in elderly patients and in males, and was characterized by a more prolonged clinical course. Abnormalities of serum electrolytes and hepatic enzymes were the most common laboratory complications, most often seen in elderly patients. Most isolates exhibited susceptibility to the common antimicrobial agents used in shigellosis therapy.