Association of GLP1R variants rs2268641 and rs6923761 with obesity and other metabolic parameters in a Polish cohort.

Introduction: Obesity is a complex disease associated with excessive fat accumulation and numerous metabolic complications. So far, many factors leading to the development of this disorder have been identified, including genetic susceptibility. Various studies linked GLP1R variants with anthropometric and metabolic parameters, suggesting the role of the variation in this gene in metabolic health. Objective: The aim of this study is to investigate the association of two single nucleotide variants of GLP1R gene, rs2268641 and rs6923761, with excessive weight, metabolic syndrome, anthropometric measurements and selected metabolic parameters. Methods: Normal-weight subjects (n= 340, control group) and subjects with excessive body mass (n = 600, study group) participated in this study. For all participants, anthropometric measurements and metabolic parameters were collected, and genotyping of the two single nucleotide variants of GLP1R gene, rs2268641 and rs6923761, was performed using the high-resolution melting curve analysis. Results: Significant differences in the genotype distribution of rs2268641 were found, where homozygous TT genotype was significantly less frequent in the study group with excessive body mass (OR=0.66; p=0.0298). For rs6923761, A allele and homozygous AA genotype were significantly more frequent in the study group with excessive weight than in the control group (OR=1.27; p=0.0239 and OR=1.69; p=0.0205, respectively). The association of studied variants with metabolic parameters was found for rs6923761. For this variant, AA carriers had higher body mass in comparison to GG carriers (p=0.0246), and AA carriers had higher glucose concentration in comparison to AG carriers (p=0.0498). We did not find an association of rs2268641 and rs6923761 with metabolic syndrome. Conclusion: In our study, AA carriers of rs6923761 had higher risk of excessive body mass, whereas TT carriers of rs2268641 had lower risk of being overweight. Moreover, homozygous carriers of the minor allele of rs6923761 had higher glucose concentration in comparison to heterozygous subjects. None of the studied variants were associated with metabolic syndrome in the studied population.

The Link between Three Single Nucleotide Variants of the GIPR Gene and Metabolic Health.

Single nucleotide variants (SNVs) of the GIPR gene have been associated with BMI and type 2 diabetes (T2D), suggesting the role of the variation in this gene in metabolic health. To increase our understanding of this relationship, we investigated the association of three GIPR SNVs, rs11672660, rs2334255 and rs10423928, with anthropometric measurements, selected metabolic parameters, and the risk of excessive body mass and metabolic syndrome (MS) in the Polish population. Normal-weight subjects (n = 340, control group) and subjects with excessive body mass (n = 600, study group) participated in this study. For all participants, anthropometric measurements and metabolic parameters were collected, and genotyping was performed using the high-resolution melting curve analysis. We did not find a significant association between rs11672660, rs2334255 and rs10423928 variants with the risk of being overweight. Differences in metabolic and anthropometric parameters were found for investigated subgroups. An association between rs11672660 and rs10423928 with MS was identified. Heterozygous CT genotype of rs11672660 and AT genotype of rs10423928 were significantly more frequent in the group with MS (OR = 1.38, 95%CI: 1.03-1.85; p = 0.0304 and OR = 1.4, 95%CI: 1.05-1.87; p = 0.0222, respectively). Moreover, TT genotype of rs10423928 was less frequent in the MS group (OR = 0.72, 95%CI: 0.54-0.95; p = 0.0221).

Hair Cortisol Concentration, Weight Loss Maintenance and Body Weight Variability: A Prospective Study Based on Data From the European NoHoW Trial.

Several cross-sectional studies have shown hair cortisol concentration to be associated with adiposity, but the relationship between hair cortisol concentration and longitudinal changes in measures of adiposity are largely unknown. We included 786 adults from the NoHoW trial, who had achieved a successful weight loss of ≥5% and had a body mass index of ≥25 kg/m2 prior to losing weight. Hair cortisol concentration (pg/mg hair) was measured at baseline and after 12 months. Body weight and body fat percentage were measured at baseline, 6-month, 12-month and 18-month visits. Participants weighed themselves at home ≥2 weekly using a Wi-Fi scale for the 18-month study duration, from which body weight variability was estimated using linear and non-linear approaches. Regression models were conducted to examine log hair cortisol concentration and change in log hair cortisol concentration as predictors of changes in body weight, change in body fat percentage and body weight variability. After adjustment for lifestyle and demographic factors, no associations between baseline log hair cortisol concentration and outcome measures were observed. Similar results were seen when analysing the association between 12-month concurrent development in log hair cortisol concentration and outcomes. However, an initial 12-month increase in log hair cortisol concentration was associated with a higher subsequent body weight variability between month 12 and 18, based on deviations from a nonlinear trend (ß: 0.02% per unit increase in log hair cortisol concentration [95% CI: 0.00, 0.04]; P=0.016). Our data suggest that an association between hair cortisol concentration and subsequent change in body weight or body fat percentage is absent or marginal, but that an increase in hair cortisol concentration during a 12-month weight loss maintenance effort may predict a slightly higher subsequent 6-months body weight variability. Clinical Trial Registration: ISRCTN registry, identifier ISRCTN88405328.

IMUMETER-A Convolution Neural Network-Based Sensor for Measurement of Aircraft Ground Performance.

The paper presents the development of the IMUMETER sensor, designed to study the dynamics of aircraft movement, in particular, to measure the ground performance of the aircraft. A motivation of this study was to develop a sensor capable of airplane motion measurement, especially for airfield performance, takeoff and landing. The IMUMETER sensor was designed on the basis of the method of artificial neural networks. The use of a neural network is justified by the fact that the automation of the measurement of the airplane's ground distance during landing based on acceleration data is possible thanks to the recognition of the touchdown and stopping points, using artificial intelligence. The hardware is based on a single-board computer that works with the inertial navigation platform and a satellite navigation sensor. In the development of the IMUMETER device, original software solutions were developed and tested. The paper describes the development of the Convolution Neural Network, including the learning process based on the measurement results during flight tests of the PZL 104 Wilga 35A aircraft. The ground distance of the test airplane during landing on a grass runway was calculated using the developed neural network model. Additionally included are exemplary measurements of the landing distance of the test airplane during landing on a grass runway. The results obtained in this study can be useful in the development of artificial intelligence-based sensors, especially those for the measurement and analysis of aircraft flight dynamics.

Incretin Hormones in Obesity and Related Cardiometabolic Disorders: The Clinical Perspective.

The prevalence of obesity continues to grow rapidly worldwide, posing many public health challenges of the 21st century. Obese subjects are at major risk for serious diet-related noncommunicable diseases, including type 2 diabetes mellitus, cardiovascular disease, and non-alcoholic fatty liver disease. Understanding the mechanisms underlying obesity pathogenesis is needed for the development of effective treatment strategies. Dysregulation of incretin secretion and actions has been observed in obesity and related metabolic disorders; therefore, incretin-based therapies have been developed to provide new therapeutic options. Incretin mimetics present glucose-lowering properties, together with a reduction of appetite and food intake, resulting in weight loss. In this review, we describe the physiology of two known incretins-glucose-dependent insulinotropic polypeptide (GIP) and glucagon-like peptide-1 (GLP-1), and their role in obesity and related cardiometabolic disorders. We also focus on the available and incoming incretin-based medications that can be used in the treatment of the above-mentioned conditions.

Wind Tunnel Testing of Plasma Actuator with Two Mesh Electrodes to Boundary Layer Control at High Angle of Attack.

The manuscript presents experimental research carried out on the wing model with the SD 7003 profile. A plasma actuator with DBD (Dielectric Barrier Discharge) discharges was placed on the wing surface to control boundary layer. The experimental tests were carried out in the AeroLab wind tunnel where the forces acting on the wing during the tests were measured. The conducted experimental research concerns the analysis of the phenomena that take place on the surface of the wing with the DBD plasma actuator turned off and on. The plasma actuator used during the experimental tests has a different structure compared to the classic plasma actuator. The commonly tested plasma actuator uses solid/impermeable electrodes, while in the research, the plasma actuator uses a new type of electrodes, two mesh electrodes separated by an impermeable Kapton dielectric. The experimental research was carried out for the angle of attack α = 15° and several air velocities V = 5-15 m/s with a step of 5 m/s for the Reynolds number Re = 87,500-262,500. The critical angle of attack at which the SD 7003 profile has the maximum lift coefficient is about 11°; during the experimental research, the angle was 15°. Despite the high angle of attack, it was possible to increase the lift coefficient. The use of a plasma actuator with two mesh electrodes allowed to increase the lift by 5%, even at a high angle of attack. During experimental research used high voltage power supply for powering the DBD plasma actuator in the voltage range from 7.5 to 15 kV.

Identification of psychological correlates of dietary misreporting under laboratory and free-living environments.

Errors inherent in self-reported measures of energy intake (EI) are substantial and well documented, but correlates of misreporting remain unclear. Therefore, potential predictors of misreporting were examined. In Study One, fifty-nine individuals (BMI = 26·1 (sd 3·8) kg/m2, age = 42·7 (sd 13·6) years, females = 29) completed a 14-d stay in a residential feeding behaviour suite where eating behaviour was continuously monitored. In Study Two, 182 individuals (BMI = 25·7 (sd 3·9) kg/m2, age = 42·4 (sd 12·2) years, females = 96) completed two consecutive days in a residential feeding suite and five consecutive days at home. Misreporting was directly quantified by comparing covertly measured laboratory weighed intakes (LWI) with self-reported EI (weighed dietary record (WDR), 24-h recall, 7-d diet history, FFQ). Personal (age, sex and %body fat) and psychological traits (personality, social desirability, body image, intelligence quotient and eating behaviour) were used as predictors of misreporting. In Study One, those with lower psychoticism (P = 0·009), openness to experience (P = 0·006) and higher agreeableness (P = 0·038) reduced EI on days participants knew EI was being measured to a greater extent than on covert days. Isolated associations existed between personality traits (psychoticism and openness to experience), eating behaviour (emotional eating) and differences between the LWI and self-reported EI, but these were inconsistent between dietary assessment techniques and typically became non-significant after accounting for multiplicity of comparisons. In Study Two, sex was associated with differences between LWI and the WDR (P = 0·009), 24-h recall (P = 0·002) and diet history (P = 0·050) in the laboratory, but not home environment. Personal and psychological correlates of misreporting identified displayed no clear pattern across studies or dietary assessment techniques and had little utility in predicting misreporting.

Consistent sleep onset and maintenance of body weight after weight loss: An analysis of data from the NoHoW trial.

BACKGROUND: Several studies have suggested that reduced sleep duration and quality are associated with an increased risk of obesity and related metabolic disorders, but the role of sleep in long-term weight loss maintenance (WLM) has not been thoroughly explored using prospective data. METHODS AND FINDINGS: The present study is an ancillary study based on data collected on participants from the Navigating to a Healthy Weight (NoHoW) trial, for which the aim was to test the efficacy of an evidence-based digital toolkit, targeting self-regulation, motivation, and emotion regulation, on WLM among 1,627 British, Danish, and Portuguese adults. Before enrolment, participants had achieved a weight loss of ≥5% and had a BMI of ≥25 kg/m2 prior to losing weight. Participants were enrolled between March 2017 and March 2018 and followed during the subsequent 12-month period for change in weight (primary trial outcome), body composition, metabolic markers, diet, physical activity, sleep, and psychological mediators/moderators of WLM (secondary trial outcomes). For the present study, a total of 967 NoHoW participants were included, of which 69.6% were women, the mean age was 45.8 years (SD 11.5), the mean baseline BMI was 29.5 kg/m2 (SD 5.1), and the mean weight loss prior to baseline assessments was 11.4 kg (SD 6.4). Objectively measured sleep was collected using the Fitbit Charge 2 (FC2), from which sleep duration, sleep duration variability, sleep onset, and sleep onset variability were assessed across 14 days close to baseline examinations. The primary outcomes were 12-month changes in body weight (BW) and body fat percentage (BF%). The secondary outcomes were 12-month changes in obesity-related metabolic markers (blood pressure, low- and high-density lipoproteins [LDL and HDL], triglycerides [TGs], and glycated haemoglobin [HbA1c]). Analysis of covariance and multivariate linear regressions were conducted with sleep-related variables as explanatory and subsequent changes in BW, BF%, and metabolic markers as response variables. We found no evidence that sleep duration, sleep duration variability, or sleep onset were associated with 12-month weight regain or change in BF%. A higher between-day variability in sleep onset, assessed using the standard deviation across all nights recorded, was associated with weight regain (0.55 kg per hour [95% CI 0.10 to 0.99]; P = 0.016) and an increase in BF% (0.41% per hour [95% CI 0.04 to 0.78]; P = 0.031). Analyses of the secondary outcomes showed that a higher between-day variability in sleep duration was associated with an increase in HbA1c (0.02% per hour [95% CI 0.00 to 0.05]; P = 0.045). Participants with a sleep onset between 19:00 and 22:00 had the greatest reduction in diastolic blood pressure (DBP) (P = 0.02) but also the most pronounced increase in TGs (P = 0.03). The main limitation of this study is the observational design. Hence, the observed associations do not necessarily reflect causal effects. CONCLUSION: Our results suggest that maintaining a consistent sleep onset is associated with improved WLM and body composition. Sleep onset and variability in sleep duration may be associated with subsequent change in different obesity-related metabolic markers, but due to multiple-testing, the secondary exploratory outcomes should be interpreted cautiously. TRIAL REGISTRATION: The trial was registered with the ISRCTN registry (ISRCTN88405328).

Association between objectively measured sleep duration, adiposity and weight loss history.

BACKGROUND: An association between sleep and obesity has been suggested in several studies, but many previous studies relied on self-reported sleep and on BMI as the only adiposity measure. Moreover, a relationship between weight loss history and attained sleep duration has not been thoroughly explored. DESIGN: The study comprised of 1202 participants of the European NoHoW trial who had achieved a weight loss of ≥5% and had a BMI of ≥25 kg/m2 prior to losing weight. Information was available on objectively measured sleep duration (collected during 14 days), adiposity measures, weight loss history and covariates. Regression models were conducted with sleep duration as the explanatory variable and BMI, fat mass index (FMI), fat-free mass index (FFMI) and waist-hip ratio (WHR) as response variables. Analyses were conducted with 12-month weight loss, frequency of prior weight loss attempts or average duration of weight maintenance after prior weight loss attempts as predictors of measured sleep duration. RESULTS: After adjusting for physical activity, perceived stress, smoking, alcohol consumption, education, sex and age, sleep duration was associated to BMI (P < 0.001), with the highest BMI observed in the group of participants sleeping <6 h a day [34.0 kg/m2 (95% CI: 31.8-36.1)]. Less difference in BMI was detected between the remaining groups, with the lowest BMI observed among participants sleeping 8-<9 h a day [29.4 kg/m2 (95% CI: 28.8-29.9)]. Similar results were found for FMI (P = 0.008) and FFMI (P < 0.001). We found no association between sleep duration and WHR. Likewise, we found no associations between weight loss history and attained sleep duration. CONCLUSION: In an overweight population who had achieved a clinically significant weight loss, short sleep duration was associated with higher BMI, with similar associations for fat and lean mass. We found no evidence of association between weight loss history and attained sleep duration.

Associations between the proportion of fat-free mass loss during weight loss, changes in appetite, and subsequent weight change: results from a randomized 2-stage dietary intervention trial.

BACKGROUND: Dynamic changes in body composition which occur during weight loss may have an influential role on subsequent energy balance behaviors and weight. OBJECTIVES: The aim of this article is to consider the effect of proportionate changes in body composition during weight loss on subsequent changes in appetite and weight outcomes at 26 wk in individuals engaged in a weight loss maintenance intervention. METHODS: A subgroup of the Diet, Obesity, and Genes (DiOGenes) study (n = 209) was recruited from 3 European countries. Participants underwent an 8-wk low-calorie diet (LCD) resulting in ≥8% body weight loss, during which changes in body composition (by DXA) and appetite (by visual analog scale appetite perceptions in response to a fixed test meal) were measured. Participants were randomly assigned into 5 weight loss maintenance diets based on protein and glycemic index content and followed up for 26 wk. We investigated associations between proportionate fat-free mass (FFM) loss (%FFML) during weight loss and 1) weight outcomes at 26 wk and 2) changes in appetite perceptions. RESULTS: During the LCD, participants lost a mean ± SD of 11.2 ± 3.5 kg, of which 30.4% was FFM. After adjustment, there was a tendency for %FFML to predict weight regain in the whole group (ß: 0.041; 95% CI: -0.001, 0.08; P = 0.055), which was significant in men (ß: 0.09; 95% CI: 0.02, 0.15; P = 0.009) but not women (ß: 0.01; 95% CI: -0.04, 0.07; P = 0.69). Associations between %FFML and change in appetite perceptions during weight loss were inconsistent. The strongest observations were in men for hunger (r = 0.69, P = 0.002) and desire to eat (r = 0.61, P = 0.009), with some tendencies in the whole group and no associations in women. CONCLUSIONS: Our results suggest that composition of weight loss may have functional importance for energy balance regulation, with greater losses of FFM potentially being associated with increased weight regain and appetite. This trial was registered at clinicaltrials.gov as NCT00390637.

Monitoring the Risk of the Electric Component Imposed on a Pilot During Light Aircraft Operations in a High-Frequency Electromagnetic Field.

High-frequency electromagnetic fields can have a negative effect on both the human body and electronic devices. The devices and systems utilized in radio communications constitute the most numerous sources of electromagnetic fields. The following research investigates values of the electric component of electromagnetic field intensification determined with the ESM 140 dosimeter during the flights of four aircrafts-Cessna C152, Cessna C172, Aero AT3 R100, and Robinson R44 Raven helicopter-from the airport in Depultycze Krolewskie near Chelm, Poland. The point of reference for the obtained results were the normative limits of the electromagnetic field that can affect a pilot in the course of a flight. The maximum value registered by the dosimeter was E = 3.307 V/m for GSM 1800 frequencies.

Application of GNSS/INS and an Optical Sensor for Determining Airplane Takeoff and Landing Performance on a Grassy Airfield.

The performance of a PZL 104 Wilga 35A airplane was determined and analyzed in this work. Takeoff and landing distances were determined by means of two different methods: one which utilized a Global Navigation Satellite System/Inertial Navigation System (GNSS/INS) sensor and another in which airplane ground speed was measured with the use of an optical non-contact sensor. Based on the airfield measurements, takeoff and landing distances as well as rolling resistance coefficients were determined for the used airplane on a grassy runway at the Radawiec airfield, located near Lublin, southeast Poland. The study was part of the "GARFIELD" project that is expected to deliver an online information system on grassy airfield conditions. It was concluded that both sensors were suitable for the aimed research. The results obtained in this study showed the effects of high grass upon the takeoff and landing performances of the test airplane. Also, the two methods were compared against each other, and the final results were compared to calculations of ground distances by means of the chosen analytical models.

Developing evidence-based behavioural strategies to overcome physiological resistance to weight loss in the general population.

Physiological and behavioural systems are tolerant of excess energy intake and responsive to energy deficits. Weight loss (WL) changes body structure, physiological function and energy balance (EB) behaviours, which resist further WL and promote subsequent weight regain. Measuring and understanding the response of EB systems to energy deficits is important for developing evidence-based behaviour change interventions for longer-term weight management. Currently, behaviour change approaches for longer-term WL show modest effect sizes. Self-regulation of EB behaviours (e.g. goal setting, action plans, self-monitoring, relapse prevention plans) and aspects of motivation are important for WL maintenance. Stress management, emotion regulation and food hedonics may also be important for relapse prevention, but the evidence is less concrete. Although much is known about the effects of WL on physiological and psychological function, little is known about the way these dynamic changes affect human EB behaviours. Key areas of future importance include (i) improved methods for detailed tracking of energy expenditure, balance and by subtraction intake, using digital technologies, (ii) how WL impacts body structure, function and subsequent EB behaviours, (iii) how behaviour change approaches can overcome physiological resistance to WL and (iv) who is likely to maintain WL or relapse. Modelling physiological and psychological moderators and mediators of EB-related behaviours is central to understanding and improving longer-term weight and health outcomes in the general population.

Determining Wheel Forces and Moments on Aircraft Landing Gear with a Dynamometer Sensor.

This paper describes airfield measurement of forces and moments that act on a landing gear wheel. For the measurement, a wheel force sensor was used. The sensor was designed and built based on strain gage technology and was embedded in the left landing gear wheel of a test aircraft. The sensor is capable of measuring simultaneously three perpendicular forces and three moments and sends data to a handheld device wirelessly. For the airfield tests, the sensor was installed on a PZL 104 Wilga 35A multipurpose aircraft. The aircraft was towed at a "marching man" speed and the measurements were performed at three driving modes: Free rolling, braking, and turning. The paper contains results obtained in the field measurements performed on a grassy runway of the Rzeszów Jasionka Aerodrome, Poland. Rolling resistance of aircraft tire, braking friction, as well as aligning moment were analyzed and discussed with respect to surface conditions.

The effect of hemodialysis on left ventricular outflow tract gradient.

BACKGROUND: The aim of the study was to assess the effect of hemodialysis (HD) on left ventricular outflow tract gradient (LVOTG) measured both in supine and upright position (provocative maneuver to unload LV cavity by rapid preload reduction). Supine/standing echocardiography was performed immediately before and immediately after HD. For additional verification of the hypothesis about preload-dependence of LVOTG, the echocardiograms after long (2-day delay HD due to weekend) versus short (usual 1-day) pause between HDs were compared. METHODS: Forty-one patients on chronic HD (mean age 44 +/- 11 years) were examined using a portable hand-carried echocardiograph. In accordance with the prestudy assumption the ultrafiltration volume was significantly greater during HD after a long pause in comparison to HD after a short pause (3707 +/- 2826 mL vs. 2665 +/- 1152 mL P < 0.05). RESULTS: After a long pause, the mean value of LVOTG at the pre-HD was mildly increased in the supine position and remained at a similar level in the upright position (13.1 +/- 6.1 vs. 13.6 +/- 9.1 mmHg). Mean LVOTG at the post-HD in the supine position was similar to pre-HD, however the orthostatic stress test induced a significant increase of LVOTG (13.9 +/- 15.2 vs. 18.2 +/- 19.9 mmHg P < 0.05). After a short pause at the pre-HD the LVOTG in the supine position and after the orthostatic provocation was very similar to measurements after long pause (13.3 +/- 9.1 vs. 13.3 +/- 10.8 mmHg). At the post-HD the mean value of LVOTG increased during upright posture but the differences were of borderline significance (13.2 +/- 6.6 vs. 17.9 +/- 18.6 mmHg P = 0.052). CONCLUSIONS: HD predisposed to standing-provoked LVOTG especially when a long pause (2 days) between HDs induced a greater weight gain and subsequently a larger volume of ultrafiltration was needed to reduce hypervolemia.

Left ventricular outflow tract gradient provoked by upright position or exercise in treated patients with hypertrophic cardiomyopathy without obstruction at rest.

OBJECTIVES: The aim of the study was to assess inducibility of left ventricular outflow tract (LVOT) gradient by change of position from supine to upright and by treadmill exercise in treated patients with hypertrophic cardiomyopathy (HCM) without obstruction at rest. METHODS: We studied 37 treated HCM patients (21 men and 16 women, mean age 44 +/- 12 years) with LVOT gradient <30 mmHg at rest in supine position. The patients were then placed in upright position and the gradient was reexamined. The patients who developed LVOT gradient >or= 30 mmHg during this maneuver were not exercised, whereas the remaining patients (nonobstructive in orthostatic position) performed moderate-intensity exercise on a treadmill, with continuous monitoring of the LVOT gradient. For comparison with resting measurements, gradients at peak exercise (in upright position) and at recovery (in supine position) were used. The resting minimal distance between the mitral valve and ventricular septum at systole was used to assess the degree of narrowing of LVOT. RESULTS: The orthostatic position provoked LVOT gradient >or= 30 mmHg in 8 of 37 patients. At peak exercise, 10 of the remaining 29 patients developed significant LVOT gradient. At recovery in supine position, this significant gradient disappeared in 6 of 10 patients, despite only a short delay in measurement. Of resting echocardiographic parameters, only systolic mitral-septal distance differentiated between the provocable and nonprovocable subgroups. Patients with provocable gradient (either by changing position or by exercise) presented with lower values of this parameter than the nonprovocable subgroup. CONCLUSIONS: In nonobstructive HCM patients under treatment, the LVOT gradient was inducible by upright position in 21.6% and by upright moderate exercise in 34.5%. The minimal septal-mitral distance may be useful to identify patients with provocable obstruction.

[Physical development in children with juvenile idiopathic arthritis]. / Ocena poziomu rozwoju fizycznego dzieci i mlodziezy z mlodzienczym idiopatycznym zapaleniem stawów.

INTRODUCTION: Juvenile chronic arthritis (JIA) is a major paediatric rheumatic disease. AIM OF THE STUDY: The aim of this study was to determine how physical development in children with JIA was affected by the subtype of the disease and by the type of therapy used. MATERIAL AND METHODS: Data were collected at the Sunbeam Center for the Diagnosis and Treatment of Childhood Illnesses in Wroclaw, Poland. The study group included 82 children from 4 to 18 years old that were diagnosed with juvenile idiopathic arthritis. The duration of the disease ranged from 3 months to 15 years, with a mean of 2.9+/-2,8 years. At the time the subjects were examined, the disease was either very mild, inactive or in remission. Because the subjects varied in terms of age, all data were standardized and recorded in terms of numbers of standard deviations away from the age-specific and sex-specific means for the reference group, which consisted of healthy children and adolescents from Warsaw, Poland. RESULTS: In the subjects in this study, height and weight were insignificantly lower than in their healthy peers. Body height and body weight were negatively affected only in children treated with corticosteroids for more than one year. Serious growth disorders were observed in 5 subjects (6%). Chest depth and chest width were significantly lower. CONCLUSIONS: Strong relationship between the growth retardation and long-term steroid therapy has been pointed out.

Acute myocardial infarction and a new ABCC6 mutation in a 16-year-old boy with pseudoxanthoma elasticum.

Pseudoxanthoma elasticum is caused by mutations of the ABCC6 gene. We hereby report a case of pseudoxanthoma elasticum with clinical features dominated by early coronary involvement in addition to typical skin and ocular abnormalities. A 16-year-old survivor of acute myocardial infarction with 3-vessel coronary artery disease exhibited compound heterozygosity for the well-known nonsense mutation (c.3421C>T; R1141X) in exon 24 and a novel missense mutation (c.3662G>A; R1221H) in exon 26 of the ABCC6 gene.