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In patients with rheumatoid arthritis (RA) with cardiovascular disease risk, it is unknown whether exercises are safe, improve cardiorespiratory fitness and reduce disease-related symptoms and cardiovascular-disease (CVD) risk factors. We aimed to investigate in RA patients with CVD risk: (1) safety of medium to high-intensity aerobic exercises, (2) potential changes of cardiorespiratory fitness and (3) disease activity and CVD risk factors in response to the exercises.Single-arm pilot-exercise intervention study including 26 consecutive patients (21 women) with 4% 10-year risk of CVD mortality according to the Dutch Systematic Coronary Risk Evaluation. Aerobic exercises consisted of two supervised-sessions and five home-sessions per week for 12 weeks. Patients were required to exercise at intensities between 65 and 85% of their maximum heart rate. To assess safety, we recorded exercise related adverse events. Before and after the exercises, cardiorespiratory fitness was assessed with a graded maximal oxygen-uptake exercise test, while disease activity was evaluated via the Disease Activity Score-28 (DAS28) using the erythrocyte segmentation rate (ESR). Resting blood pressure, ESR and total cholesterol were assessed as CVD risk factors.Twenty out of 26 patients performed the 12-week exercises without any adverse events. According to patients, withdrawals were unrelated to the exercises. Exercises increased cardiorespiratory fitness (pre: 15.91 vs. post: 18.15 ml.kgA 12-week exercise intervention seems to be safe and improves cardiorespiratory fitness and disease activity in patients with RA with a high risk for cardiovascular diseases. Key Points 1. Rheumatoid arthritis patients with high cardiovascular disease risk were able to perform a maximum exercise test and a 12-week aerobic-based medium-to-high intensity exercise intervention. 2. The exercise intervention improved cardiorespiratory fitness and disease activity in rheumatoid arthritis patients with high cardiovascular disease risk. 3. Cardiorespiratory fitness levels were still low post-exercise intervention (i.e. 18.15 ml.kg-1min-1 compared to the 20.9 ml.kg-1min-1 baseline mean of the RA patients without CVD risk).

BACKGROUND To date, there is no evidence to support the optimal competition strategy for success in powerlifting competitions. The purpose of this study was to analyse Powerlifting (PL) competition data to assess the relationship between squat attempts, the success of each lift attempt, and weight increase between attempts, with winning. METHODS 10,672 individual competition entries (males: n = 6617, females: n = 4,055) were included within the analysis from 'raw' Powerlifting Australia sanctioned competitions held between 2008 and 2019. We reported Cohen's d, statistical significance, 95% confidence intervals and the univariate odds of winning an event. Factors were assessed by separate simple logistic regression and reported as an odds ratio. RESULTS Overall, first squat attempt weight for those who won was on average 7.0kg greater (P

Latella, C, Teo, W-P, Spathis, J, and van den Hoek, D. Long-term strength adaptation: A 15-year analysis of powerlifting athletes. J Strength Cond Res 34(9): 2412–2418, 2020—Strength is a fundamental component of athletic performance and development. This investigation examined the long-term strength development of powerlifting (PL) athletes. The rate of strength gain/day was assessed in 1897 PL athletes (F = 626, M = 1,271) over a 15-year period (2003–2018). Independent T-tests explored sex differences in baseline absolute (kg) and relative strength (kg·body mass−1 [bm]) recorded from the first competition, and strength gain/day (kg·d−1). Analyses based on initial strength quartiles were conducted using one-way analysis of variances with significance set at p < 0.05. Bivariate correlational analysis tested for relationships between strength gain/day and baseline strength, the number of competitions, and mean days between competitions. Males had greater absolute (M: 513.3 ± 99.8 kg, F: 289.4 ± 55.7 kg, p < 0.001) and relative (M: 5.89 ± 1.04 kg·bm−1, F: 4.27 ± 0.85 kg·bm−1, p < 0.001) strength at baseline. Overall, strength gain/day (F: 0.12 ± 0.69 kg·d−1, M: 0.15 ± 0.44 kg·d−1, p = 0.318) was similar between sexes. However, the strongest males showed a lower rate of strength improvement (0.102 kg·d−1) compared with least strong males (0.211 kg·d−1), p = 0.010. No differences were observed across quartiles for females. Correlational analyses revealed significant but weak negative relationships between strength gain/day and the mean days between competitions for females (r2 = −0.120, p = 0.003) and males (r2 = −0.190, p < 0.001). Similar relationships were observed for baseline strength (r2 = −0.073, p = 0.009) and the number of competitions (r2 = −0.111, p < 0.001) for males. The results suggest similar strength adaptation between sexes. The strongest males improve more slowly, possibly due to a ceiling effect. Collectively, the findings provide novel evidence of real-world long-term strength adaptations that may be particularly useful to understand athlete development, to aid periodized programming, and to benchmark strength over time.

Pearson, J, Spathis, JG, van den Hoek, DJ, Owen, PJ, Weakley, J, and Latella, C. Effect of competition frequency on strength performance of powerlifting athletes. J Strength Cond Res 34(5): 1213-1219, 2020-Powerlifting (PL) requires athletes to achieve the highest possible "total" weight lifted across squat, bench press, and deadlift. Athletes compete multiple times per year; however, it is not well understood how often PL athletes should compete to facilitate maximal strength performance. This study investigated the effect of competition frequency on strength (relative and absolute) in PL athletes over a 12-month period. Results across all male (n = 563, mean ± SD; age; 28 ± 10 years, body mass; 89.3 ± 19.3 kg) and female (n = 437, age; 31 ± 11 years, body mass; 70.1 ± 15.8 kg) PL athletes were collated. Total competition scores were used to calculate absolute and relative strength for each competition. Linear mixed models with random effects, and effect sizes ± 95% confidence intervals compared competition frequency and total score for (a) all, (b) male, and (c) female competition entries, respectively. The association between total score at each competition was assessed with Pearson's correlation coefficient for the same independent variables. Results demonstrate greater absolute strength at competition 2 for all athletes (5.1%: p = 0.043: d = 0.16) and males (2.9%: p = 0.049: d = 0.15). For females, absolute strength was greater at competition 5 compared to 1 (12.0%: p = 0.001: d = 0.65) and 2 (9.6%: p = 0.007: d = 0.50). Weak positive correlations for relative strength and number of times competed for males were evident between competitions 1 to 4 (r = 0.070-0.085, p = 0.003-0.043). For females, 3 competitions weakly correlated with absolute strength (r = 0.106, p = 0.016). PL athletes who compete multiple times per year are more likely to achieve higher totals; however, there is an upper limit to the number of competitions (4 per year) that seem to allow a performance increase.

Latella, C, van den Hoek, D, and Teo, WP. Differences in strength performance between novice and elite athletes: Evidence from powerlifters. J Strength Cond Res 33(7S): S103-S112, 2019-Strength forms an integral part of many sports. In particular, powerlifting success is determined solely by maximal strength, providing a unique opportunity to investigate the differences and potential factors influencing novice and elite competitors. We evaluated performance from 2,137 competitors between local (LOC), national (NAT), and international (INT) competitions. Results were analyzed by using the total (TOT) competition score within weight classes and age categories. Cohen's d effect sizes and 95% confidence intervals were used to detect differences within categories between LOC, NAT, and INT competitions. The coefficient of variation (CV) was used to determine the absolute variability. A moderate to large increase in performance was observed for all weight classes between LOC and NAT (men; d = 0.76, women; d = 1.09). No meaningful differences were observed between LOC and NAT, and NAT and INT when compared using age. No meaningful differences were observed between NAT to INT competitions when compared using weight classes. The CV was not different across competition level (CV = 17.4-22.9%) categories. Several internal (athlete) and external (environmental) factors are likely to explain these findings. Therefore, factors such as training experience, performance variability, body composition, anthropometric characteristics, and competition pressure that may influence strength performance should also be considered in both training phases and during competition. Collectively, the results offer novel information regarding the difference in strength performance between novice, subelite, and elite strength athletes. Strength and conditioning professionals should consider these factors when working with various athletes where maximal strength is an important determinant of success.

Lower cardiorespiratory fitness (CRF) and physical activity (PA) associate with higher cardiovascular disease (CVD) risk, but the relationship between CRF and PA in people who have rheumatoid arthritis (RA) at an increased CVD risk (CVD-RA) is not known. The objectives of this study were to determine the levels of CRF and PA in people who have CVD-RA and to investigate the association of CRF with PA in people who have CVD-RA. A total of 24 consecutive patients (19 women) with CVD-RA ( 4% for 10-year risk of fatal CVD development as calculated using the Systematic Coronary Risk Evaluation)-were included in the study. CRF was assessed with a graded maximal exercise test determining maximal oxygen uptake (VO

Introduction: To determine whether combined exercise training with an energy-restricted diet leads to improved physical fitness and body composition when compared to energy restriction alone in free-living premenopausal women with clinically severe obesity. Methods: Sixty premenopausal women (BMI of 40.4 ± 6.7) were randomised to energy restriction only (ER) or to exercise plus energy restriction (EXER) for 12 months. Body composition and fitness were measured at baseline, 3, 6 and 12 months. Results: VO2 peak improved more for EXER compared to ER at 3 (mean difference ± SEM 2.5 ± 0.9 mL ∙ kg–1 ∙ min–1, p = 0.006) and 6 (3.1 ± 1.2 mL ∙ kg–1 ∙ min–1, p = 0.007) but not 12 months (2.3 ± 1.6 mL ∙ kg–1 ∙ min–1, p = 0.15). Muscle strength improved more for EXER compared to ER at all time points. No differences between groups for lean mass were observed at 12 months. Conclusion: Combining exercise training with an energy-restricted diet did not lead to greater aerobic power, total body mass, fat mass or limit lean body mass loss at 12 months when compared to energy restriction alone for premenopausal women with clinically severe obesity in free-living situations. Future research should aim to determine an effective lifestyle approach which can be applied in the community setting for this high-risk group.

Objective Obesity is associated with a decline in health-related quality of life (HRQOL), while weight loss and exercise training have a positive influence. The aim of this systematic review was to compare the effects of energy restriction (ER) alone intervention to diet and exercise intervention on HRQOL. Methods MEDLINE, CINAHL and PsycINFO databases were searched for randomised controlled trials examining HRQOL through lifestyle interventions which examined ER and energy restriction plus exercise in obese adults. Results Nine hundred and fifty-two papers were assessed for inclusion in this review with nine being deemed suitable. This review indicates that four studies provide evidence to support the role of exercise in addition to ER to improve HRQOL in adults with obesity. The findings of this review are limited due to the limited number of studies as well as substantial heterogeneity in ER, exercise prescription variables and outcome measures utilised within studies. Conclusions A definitive conclusion regarding the capacity of exercise to facilitate greater improvement in HRQOL than diet alone, or, to formulate an exercise prescription for obese adults to address HRQOL is not feasible based on the existing evidence. Future studies should utilise comparable HRQOL assessment tools along with ensuring full reporting of results.

Summary Introduction Achieving continence in children with neurogenic sphincteric incompetence is a challenge. Awareness of the long-term outcome in this young patient population is important. In the past 25 years, the study institution has built experience in bladder outlet procedures such as bladder neck sling and bladder neck reconstructions. Objective The objective of this study was to evaluate the long-term outcome on continence and re-intervention rate of bladder outlet procedures in children with neurogenic urinary incontinence at the study institution. Design All children who underwent a bladder neck procedure between 1992 and 2017 at the study institution were retrospectively reviewed. Continence at the end of follow-up was the primary endpoint, defined as ‘dry’ when there was an interval of a minimum of 4 h without urinary leakage. Non-parametric tests were used for statistical analysis. Results During this 25-year period, a total of 60 children underwent a bladder outlet procedure, either a bladder neck sling (n = 43) or a bladder neck reconstruction (n = 17). The median age at surgery was 11.6 years (interquartile range [IQR] 7.8–13.9). Concomitant surgery consisted of bladder augmentation in 80% and continent catheterizable urinary channel in 97% of children. Dry rate within 1 year was 38%. After a median follow-up of 10.4 years (IQR 6.5–15.5), 77% of all children were dry. Twenty-five children (42%) needed one or more re-interventions, including redo of the bladder outlet procedure, other type of outlet procedure, bulking agents, bladder augmentation, and bladder neck closure. Discussion This study confirms that achieving continence is a challenge. The inconsistent use of the definition of urinary continence creates confusion in the literature and makes comparison of outcome with other studies difficult. Openness of (long-term) results in achieving urinary continence is important and helpful for future patients. Conclusion On the long term, the majority of children with neurogenic urinary incontinence were dry after a bladder outlet procedure, but a considerable number of patients had a re-intervention. The initial outcome on continence was slightly disappointing. Reporting long-term results is essential and helpful for patient counseling. Summary Table . Gender and continence outcome presented in number (%). Total n = 60 BNS n = 43 BNR n = 17 Difference between BNS and BNR Gender Female 35 (58%) 31 (72%) 4 (23.5%) Male 25 (42%) 12 (28%) 13 (77.5%) P a Continence outcome Dry rate 23 (38%) 15 (35%) 8 (47%) P = 0.382 a Dry rate > 1 yr follow-up 46 (77%) 33 (77%) 13 (77%) P = 0.982 a BNS, bladder neck sling; BNR, bladder neck reconstruction. a Chi-squared test.

Background Accelerometry is the method of choice for objectively assessing physical activity in older adults. Many studies have used an accelerometer count cut point corresponding to 3 metabolic equivalents (METs) derived in young adults during treadmill walking and running with a resting metabolic rate (RMR) assumed at 3.5 mL · kg−1 · min−1 (corresponding to 1 MET). RMR is lower in older adults; therefore, their 3 MET level occurs at a lower absolute energy expenditure making the cut point derived from young adults inappropriate for this population. The few studies determining older adult specific moderate-to-vigorous intensity physical activity (MVPA) cut points had methodological limitations, such as not measuring RMR and using treadmill walking. Methods This study determined a MVPA hip-worn accelerometer cut point for older adults using measured RMR and overground walking. Following determination of RMR, 45 older adults (mean age 70.2 ± 7 years, range 60–87.6 years) undertook an outdoor, overground walking protocol with accelerometer count and energy expenditure determined at five walking speeds. Results Mean RMR was 2.8 ± 0.6 mL · kg−1 · min−1. The MVPA cut points (95% CI) determined using linear mixed models were: vertical axis 1013 (734, 1292) counts · min−1; vector magnitude 1924 (1657, 2192) counts · min−1; and walking speed 2.5 (2.2, 2.8) km · hr−1. High levels of inter-individual variability in cut points were found. Conclusions These MVPA accelerometer and speed cut points for walking, the most popular physical activity in older adults, were lower than those for younger adults. Using cut points determined in younger adults for older adult population studies is likely to underestimate time spent engaged in MVPA. In addition, prescription of walking speed based on the adult cut point is likely to result in older adults working at a higher intensity than intended.

Background: Clinical practice guidelines globally recommend lifestyle modification including diet and exercise training as first-line treatment for obesity. The clinical benefits of exercise training in adults with obesity is well-documented; however, there is no strong evidence for the effectiveness of exercise training for weight loss in class II and class III obesity. The purpose of the randomised controlled trial described in this protocol article is to examine the effect of exercise training, in addition to a very low energy diet (VLED), in clinically severe obese women for changes in body composition, physical function, quality of life, and markers of cardiometabolic risk. Methods/Design: Sixty women, aged 18–50 years with a body mass index (BMI) greater than 34.9 kg.m2 and at least one obesity-related co-morbidity, will be recruited for this 12-month study. Participants will be randomised to either exercise plus energy restriction (n = 30), or energy restriction alone (n = 30). All participants will follow an energy-restricted individualised diet incorporating a VLED component. The exercise intervention group will also receive exercise by supervised aerobic and resistance training and a home-based exercise programme totalling 300 minutes per week. Primary outcome measures include body composition and aerobic fitness. Secondary outcome measures include: physical function, cardiometabolic risk factors, quality of life, physical activity, and mental health. All outcome measures will be conducted at baseline, 3, 6 and 12 months. Discussion: Previous research demonstrates various health benefits of including exercise training as part of a healthy lifestyle at all BMI ranges. Although clinical practice guidelines recommend exercise training as part of first-line treatment for overweight and obesity, there are few studies that demonstrate the effectiveness of exercise in class II and class III obesity. The study aims to determine whether the addition of exercise training to a VLED provides more favourable improvements in body composition, physical function, quality of life, and markers of cardiometabolic risk for women with clinically severe obesity, compared to VLED alone. Trial registration: Australian New Zealand Clinical Trials Registry (ACTRN12611000694910). Date registered: 4 July 2011

Patients with rheumatoid arthritis (RA) have a significantly increased risk of mortality compared with the general population. One of the most important predictors for mortality is somatic comorbidity. Moreover, studies have demonstrated that comorbid depression is associated with mortality. Which specific comorbidities are associated with mortality is less investigated. The purpose of this study was to investigate the association of a wide range of comorbidities with mortality in patients with RA.Longitudinal data over a 14-year period were collected from 882 patients with RA. Data were assessed with questionnaires. The mortality status was obtained from the Statistics Netherlands for the period 1996-2011 for 99% of the patients. Somatic comorbidity was assessed in 1997, 1998, 1999, and 2008 and measured by a national population-based questionnaire including 20 chronic diseases. Comorbid depression was assessed in 1997, 1998, and 1999 and measured with the Center for Epidemiologic Studies Depression Scale. Cox regression was used to study the relationship between comorbidity and mortality.At baseline, 72% of the patients were women. The mean ± SD age was 59.3 ± 14.8 years, and the median (interquartile range) disease duration was 5.0 (2.0-14.0) years. A total of 345 patients died during the study period. Comorbidities that were associated with mortality were circulatory conditions (hazard ratio [HR] 1.60 [95% confidence interval (95% CI) 1.15-2.22]), respiratory conditions (HR 1.43 [95% CI 1.09-1.89]), cancer (HR 2.00 [95% CI 1.28-3.12]), and depression (HR 1.35 [95% CI 1.06-1.72]).Comorbid circulatory conditions, respiratory conditions, cancer, and depression are associated with mortality among patients with RA. Careful monitoring of these comorbidities during the course of the disease and adequate referral may improve health outcomes and chances of surviving.

Introduction An increased activity of the external urethral sphincter or pelvic floor muscles during voluntary voiding leads to dysfunctional voiding. Frequently reported symptoms are urinary incontinence, urinary tract infections and high post-void residuals. Dysfunctional voiding is a common problem in school-aged children and despite various treatment options, 10-40% of the children remain therapy-refractory. Objective The aim of this study is to evaluate the effectiveness of Onabotulinum toxin-A (BTX-A) injections in the external urethral sphincter in children with therapy-refractory dysfunctional voiding. Patients and methods Patients with therapy-refractory dysfunctional voiding who have received BTX-A injections in the external urethral sphincter from 2010 to 2013 were analysed. Children with known neuropsychiatric disorders were excluded. All children had abnormal flow patterns and increased pelvic floor tone during uroflowmetry/EMG studies. They had received at least five sessions of urotherapy and two sessions of pelvic floor physical therapy prior to treatment. A total of 100 IU of BTX-A was injected in the external urethral sphincter at the 3, 9 and 12 o'clock positions. Our main outcome measures were urinary incontinence, recurrent urinary tract infections and post-void residual. Results A total of twenty patients, of whom 16 girls, with a median age of 9 years (range 5-14) were treated with BTX-A. The median follow-up was 13 months (range 5-34). Post-void residual decreased by 75% after BTX-A, from a median of 47.5 ml (16.3-88.5 ml) to 0 ml (0.0-28.0 ml) (p = 0.001) Six patients had a post-void residual < 20 ml prior to treatment. After BTX-A sixteen patients had a post-void residual < 20 ml (Figure). No significant changes in uroflowmetry results was seen. Sixteen children are no longer daily incontinent, of whom 9 became completely dry (p = 0.0001). Eleven patients suffered from recurrent urinary tract infections prior to treatment. After BTX-A five children remained infection free, while the other six experienced only one urinary tract infection during follow-up (p = 0.003). Fourteen patients received additional urotherapy after BTX-A. Repeat injections were necessary in four patients after initial satisfactory results, with repeated good clinical responses. Two children showed no improvement after first BTX-A injection. No serious adverse events were reported. Discussion The results in this homogenous group of patients confirm the conclusions of previous studies in opting BTX-A in the external urethral sphincter to be a viable treatment option for the therapy-refractory group of patients with dysfunctional voiding. What is new, is that in most of our patients post-injection urotherapy was used to amplify the BTX-A effect. During our long-term follow-up the satisfactory results were sustained, similar to the results of the long-term follow-up presented by Vricella et al. [1]. The retrospective character and relative small sample size are limitations of this study. Conclusions This study shows safe and persistent satisfactory results during our average 13-month follow-up in 90% of our patients with therapy-refractory dysfunctional voiding. A prospective study using validated and standardized measurements will be performed to affirm our results and evaluate the exact role of post-injection urotherapy.

Diseases in childhood have an impact on growth. The influence of Langerhans cell histiocytosis (LCH) on growth has never been studied well. Recently a patient with LCH was treated with human growth hormone (GH) because of severe GH deficiency due to LCH involvement of both the hypothalamus and pituitary. This led us to review our charts from 1971 onward for evaluation of the growth patterns in patients with LCH. Here the long-term growth of 22 patients with LCH is reported, the median follow up being 7 years and 1 month. The height data were converted into standard deviation scores (SDS). At diagnosis the mean SDS of patients with isolated LCH at diagnosis was 0.04 and -0.37 in patients with disseminated LCH. Of the total group, 12 patients did not show any influence from the LCH or therapy on their growth. The remaining 10 patients reached, after a minimum of 3 years, a percentile clearly higher than that at diagnosis. However all the ten above mentioned patients, either isolated or disseminated LCH, had a lesion in the facial side of the skull. CONCLUSION. GH deficiency is not a common manifestation of LCH in childhood and GH provocation tests are only indicated when there is a poor or decelerating growth rate. In our patients the number of organs involved and/or the treatment modality did not influence the growth in all but one.

To describe long-term physical functioning and its association with somatic comorbidity and comorbid depression in patients with established rheumatoid arthritis (RA).Longitudinal data over a period of 11 years were collected from 882 patients with RA at study inclusion. Patient-reported outcomes were collected in 1997, 1998, 1999, 2002, and 2008. Physical functioning was measured with the Health Assessment Questionnaire and the physical component summary score of the Short Form 36 health survey. Somatic comorbidity was measured by a questionnaire including 12 chronic diseases. Comorbid depression was measured with the Center for Epidemiologic Studies Depression Scale. We distinguished 4 groups of patients based on comorbidity at baseline.Seventy-two percent of the patients at baseline were women. The mean ± SD age was 59.3 ± 14.8 years and the median disease duration was 5.0 years (interquartile range 2.0-14.0 years). For the total group of patients with RA, physical functioning improved over time. Patients with somatic comorbidity, comorbid depression, or both demonstrated worse physical functioning than patients without comorbidity at all data collection points. Both groups with comorbid depression had the lowest scores. Only patients with both somatic comorbidity and comorbid depression showed significantly less improvement in physical functioning over time.Both somatic comorbidity and comorbid depression were negatively associated with physical functioning during an 11-year followup period. Furthermore, their combination seems to be especially detrimental to physical functioning over time. These results emphasize the need to take somatic comorbidity and comorbid depression into account in the screening and treatment of patients with RA.

We devised a technique for phalloplasty in a female-to-male transsexual combining the lateral upper arm free flap with a bladder mucosa graft, to address the ideal requirements of phalloplasty in the best possible way. The surgical techniques are described. Healing of the phallus has been uneventful, but postoperative bladder spasms, meatal stenosis, and an intraurethral valve were encountered. The cosmetic result of the phalloplasty is pleasing. Sensibility is still increasing after a 14-month follow-up. Scarring of the upper arm donor site can be camouflaged easily. Although the actual phalloplasty as described in this article is a one-stage microsurgical procedure that appears to be reproducible, the construction of a phallus meeting all requirements still implies more than one stage.

Contains fulltext : 79985.pdf (Publisher’s version ) (Closed access) Chromosome analysis in two young patients with multiple congenital anomalies revealed a de novo interstitial deletion of 8q that has not been reported before. The deletions were overlapping by 8.35 Mb (8q24.21q24.23). The clinical features shared by our patients were coloboma, VSD, digital abnormalities, congenital dislocation of a hip, feeding problems, psychomotor delay and convulsions. The deletion included the region for Langer-Giedion syndrome (TRPS1 and EXT1) in the girl only. However, she is too young to present features of this syndrome, apart from dysmorphic features like a bulbous nose and notched alae nasi. Several genes are present in the commonly deleted region, including genes with unknown function, and genes for which haploinsufficiency is known to have no phenotypic effect in mice (Wnt1). A gene that might play a role in the convulsions of our patients is KCNQ3.

Heart failure is a life-threatening complication of fulminant meningococcal septic shock (MSS). Depression of left ventricular function, in particular, is thought to be due to circulating meningococcal endotoxin. Myocardial failure leads to ventricular dilation expressed by an increased left-ventricle end-diastolic diameter (LVED). With ultrasonography, LVED can be accurately measured as well as the shortening fraction (SF). In an evaluative study we investigated the accuracy of the SF and compared it to the accuracy of the Glasgow meningococcal septicemia prognostic score (GMSPS) in the prediction of mortality in children with fulminant MSS. In 27 children admitted in a 4-year period with a presumptive clinical diagnosis of fulminant MSS, hypotension persisted for more than 1 h despite volume loading and inotropic therapy. Seven of these children died (26%); all had an SF or =10 (the sensitivity of both scores was 100%). Positive predictive values of the SF and GMSPS were 41% and 58% respectively. CONCLUSIONS: SF can be used in addition to other severity scores in clinical decision-making and contribute to the selection of children with the worst prospects for inclusion in experimental treatment studies

Newly developed IRMAs to measure the plasma concentrations of renin and prorenin were validated for clinical use and compared with a classical enzyme kinetic assay. The IRMAs involve two monoclonal antibodies, one that reacts equally well with renin and prorenin and one that recognizes renin well but prorenin only minimally. Prorenin reactivity with the second antibody was enhanced by adding the renin inhibitor, Remikiren, to plasma. The complex of prorenin with this active-site ligand undergoes a conformational change, whereby prorenin is converted into a form that cannot be differentiated from renin by the IRMA. The linear working range of the assay was 4.0-3000 mU/L. The concentration of prorenin was calculated by subtracting the assay result obtained without Remikiren (i.e., renin) from the result obtained with Remikiren (i.e., renin plus prorenin). No more than 2% of prorenin present in plasma was detected as renin. The interassay CVs for renin quantification were 18%, 13%, and 8% at low, medium, and high concentrations, respectively. The interassay CV for calculated prorenin was 8% at both low and high concentrations. The IRMA results were highly correlated with those of an enzyme kinetic assay in healthy subjects; in patients with such conditions as primary hyperaldosteronism, renovascular hypertension, and low-, medium-, and high-renin essential hypertension; and in women undergoing gonadotropin stimulation.

We present a case report of an adult female patient with bilateral duplex system ureteroceles, containing multiple small stones. There was no history of urinary tract infections or stone disease. Treatment consisted of right upper pole heminephrectomy because of non-function and transurethral incision of the left ureterocele, resulting in almost complete removal of the calculi. Furthermore no vesicoureteral reflux was seen 3 months postoperatively. Transurethral incision is a safe and effective treatment in removing stones in ureteroceles without necessitating further treatment because of vesicoureteral reflux.

To determine trends in commercial sexual risk behaviour among drug-using prostitutes in Amsterdam after local and national prevention campaigns began in 1987.A subgroup of 281 drug-using prostitutes was selected from a comprehensive, open cohort study of drug users in Amsterdam.Cross-sectional (at intake) and within-person trends in sexual behaviour were evaluated. Person-time analysis was used to determine trends in incidence of sexually transmitted disease (STD).From 1986 to 1992, 'always' use of condoms in the 6 months preceding intake increased from 21 to 58% (P0.001) and the number of commercial contacts declined from 84 to 64 per month (P = 0.06). Consequently, the mean number of unprotected commercial contacts declined from 26 to nine per month (P0.001). Temporal trends within individual prostitutes from visits 1 to 8 (mean interval between visits, 4 months) showed a somewhat larger risk reduction than the cross-sectional trends. From 1989 to 1992, the STD incidence declined from 61 to 40 episodes per 100 person-years (P = 0.06). Prostitutes residing in Amsterdam for shorter time periods reported more STD and a relatively small core group reported the majority of STD episodes. At intake, when10% of all participants knew their HIV serostatus, HIV-positive prostitutes reported significantly lower levels of condom use and more STD than HIV-negatives, while, after learning their serostatus, levels of condom use and the STD-incidence were comparable. Prevalent and incident HIV infections were not observed among non-injecting prostitutes.Drug-using prostitutes have reduced sexual risk behaviour. There is evidence for the effectiveness of HIV testing and counseling. At present, new infections among clients of drug-using prostitutes are expected to occur, although not on a large scale. Since the incidence of STD continues to be high, it is important to monitor the spread of HIV among heterosexuals, including prostitutes and their clients, closely.

To study markers of progression in a cohort of HIV-infected intravenous drug users (IDU).A prospective epidemiologic study.We studied progression of HIV infection among 126 IDU attending the Municipal Health Service in Amsterdam.Progression was defined as a decline of the CD4 cell count to200 x 10(6)/l on two consecutive follow-up visits or AIDS.Using Cox modelling, the following baseline variables were predictive of progression. Enhanced progression was associated with: age30 years [relative hazard (RH), 7.7 [95% confidence intervals (CI), 1.7-36.0]], core antibody negativity [RH, 5.3 (95% CI, 1.6-17.6)], CD4 cell count [for CD4 cells 350-500 x 10(6)/l, RH, 1.38 (95% CI, 0.37-5.16); for CD4 cells 200-350 x 10(6)/l, RH, 9.20 (95% CI, 2.73-31.05) compared with a CD4 count500 x 10(6)/l]. A lower rate of progression was associated with borrowing used injecting equipment. IDU who reported borrowing injecting equipment between 1980 and baseline 10-99 times or99 times had a RH of 0.44 (95% CI, 0.22-0.88) and 0.19 (95% CI, 0.03-0.37), respectively, compared with IDU who had borrowed10 times. p24 antigen positivity was more predictive than core antibody negativity in a model with time-dependent variables, the relative risk for p24 antigen-positive participants was 3.5 (95% CI, 1.3-9.3). Additional analysis of progression to AIDS in a larger group of IDU showed comparable results with regard to the effect of borrowing on progression.Our observation that those IDU who reported borrowing injecting equipment most frequently appeared to have the lowest rate of progression, corrected for some sources of potential confounding, requires further epidemiologic confirmation and extended laboratory studies since other sources of bias might have been present. Baseline CD4 count, age and core antibody or p24 antigen were predictive of progression in IDU. We wish to emphasize that our results do not imply that borrowing should be encouraged, but may have implications for our understanding of HIV pathogenesis.

Two case reports of children with ectopic ureteroceles are presented. The embryologic development, symptoms, diagnosis and treatment are discussed. Hemi-nephroureterectomy is the treatment of choice. In instances, such as serious urinary tract infections, massive vesicoureteral reflux in other ureters and in single system ureteroceles, endoscopic incision of the ureterocele can be effective in terms of control of infection and decompression of other kidney parts. Definite surgery can be deferred until the child is in a better condition.

In connection with 4 cases of megaureter in children, the diagnosis and the therapy of this condition are described. The term megaureter describes a large wide ureter. It may be associated with reflux or obstruction or be present without reflux and/or obstruction. The diagnosis of obstruction by renography may be difficult because of diminished glomerular filtration rate, either physiological or due to a decreased number of glomeruli, a high compliance of the collecting system, diminished urinary output or a filled bladder. Clear cases of obstruction and/or reflux should be treated operatively. In the asymptomatic child with a primary obstructive megaureter and a good relative function conservative treatment may result in reducing the dilatation without loss of kidney function.

Deposition of radiocaesium from the Chernobyl reactor accident on the Netherlands made it possible to collect contaminated fresh grass and first cut wilted grass silage. These contaminated roughages were used in transfer experiments with lactating dairy cows to determine transfer coefficients and half-lives for Cs-137 in milk. The experimental design was based on three consecutive periods: a preliminary period to determine the background concentration of the isotope in milk, a contamination period to determine the magnitude of accumulation and finally a depletion period to measure the rate at which the activity concentration of Cs-137 in milk declined after continuous feeding. The average transfer coefficient (Fmilk) for cows fed on contaminated dried grass under steady-state conditions was 0.002 d/kg and for cows fed on slightly contaminated second cut fresh grass 0.006 d/kg. The highest transfer coefficients were obtained for cows fed on contaminated grass silage for 119 days, which also included the dry period of about two months. For the first five days after calving the Fmilk values varied from 0.0066 to 0.0091 d/kg. There were no significant differences in transfer coefficients between cows in early lactation (third month of lactation), cows in late lactation (the last month of the lactation period) and cows fed on both contaminated grass silage and uncontaminated maize silage simultaneously. Half-life values for the rate of decline of the isotope in milk during the depletion period were estimated on the basis of a mathematical model with two exponential components. These components were characterized by half-lives of 0.5 to 3.5 days and 10 to 46 days.

Two lactating cows were given tritiated hay containing organically bound tritium (OBT) only for about 4 weeks. Tritium activity was determined in milk fat, casein, lactose, milk water, and whole milk. In one cow, milk was sampled for approximately 450 days, covering two lactation periods. At steady state, specific tritium activities in casein, lactose, and milk water were 58, 10, and 11%, respectively, of those in milk fat. Some OBT was converted into THO during catabolism and entered the body water pool. This 3H source accounted for nearly 40% of tritium in lactose, but in casein and milk fat about 97% of tritium was derived from ingested OBT. Comparison of the specific activity of milk constituents with the specific activity of ingested hay showed the following values: 0.84 for milk fat, 0.49 for casein, 0.05 for lactose, 0.10 for milk water. About one-half of the tritium transferred to milk was found in organic milk constituents and the other half in milk water. Decrease of tritium activity with time could be represented by three components with different half-lives for the organic milk constituents. Those for milk fat and casein were quite similar, with a slow component of nearly 3 months.

Two lactating Friesian cows received tritiated water (19 nCi of tritium per ml) to drink for 25 days. Urine and milk samples were collected and analyzed for tritium content. Plateau levels in milk water, and in milk fat, lactose and casein were reached in about 18 days. Tritium concentration in milk water and urine water was about 82% that in drinking water, indicating dilution with water from food and metabolic processes, and as a result of exchange through lungs and skin. At steady state, tritium activity in milk fat, lactose and casein was about 30, 28 and 15% of that in drinking water on a weight basis; at this time, about 1.5% of the daily ingested tritium appeared in one litre of milk in both animals. Comparison of the specific activity of the various milk constituents with the specific activity of the body water showed that the highest tritium incorporation occurred in lactose (0.58), followed by milk fat (0.36) and casein (0.27). Tritium incorporation in milk dry matter (0.45) is considerably higher than in most tissue components of several mammalian species after continuous ingestion of THO as reported in the literature. Tritium levels fell rapidly in milk and urine after the administration of THO was stopped, with half-times of around 4 days for the rapid components in all milk constituents. A slow component of 44 days, 225 days and 24 days half-time was found in milk water, milk fat and casein respectively.