Dirk Matthys

Expressive dimensions of pain catastrophizing: a comparative analysis of school children and children with clinical pain

&NA; We investigated the role of the child’s pain catastrophizing in explaining (1) children’s self‐reported tendency to verbally share their pain experience with others and (2) different dimensions of pain expression, as described by the mother and the father, including non‐verbal and verbal communicative pain behaviour and protective pain behaviour. Participants were school children, children with chronic or recurrent pain, and their parents. The results showed that: (1) Pain catastrophizing was associated with children’s greater self‐acknowledged tendency to verbally share their pain experience with others. (2) Mothers and fathers perceived highly catastrophizing children to be more communicative about their pain. (3) The role of pain catastrophizing in the child’s verbal sharing of pain experiences and in explaining expressive behaviour as rated by parents did not differ between the school children and children with recurrent and chronic pain. (4) Nevertheless, findings indicated marked differences between school children and the clinical sample. Children of the clinical sample experienced more severe pain, more pain catastrophizing, more protective pain behaviour, but less verbal communications about their pain. These results further corroborate the position that catastrophic thoughts about pain have interpersonal consequences. Findings are discussed in terms of the possible functions and effects upon others of pain catastrophizing and associated categories of pain behaviour.

Left ventricular outflow obstruction in rhabdomyoma of infancy: meta-analysis of the literature.

Primary heart tumors are exceptional in infants and children. Most common is the rhabdomyoma, often associated with tuberous sclerosis (Bournevilles disease). This tumor is generally believed to have no hemodynamic effects in the majority of cases. Recently, severe obstruction of the left ventricular outflow tract by a solitary tumor was diagnosed during pregnancy and emergency surgery was needed soon after birth.

Effect of branched-chain amino acids (BCAA), glucose, and glucose plus BCAA on endurance performance in rats.

PURPOSE The purpose of this study was to assess the effects of pre-exercise administration of branched-chain amino acids (BCAA), glucose, and glucose plus BCAA on time to exhaustion during treadmill exercise in rats. METHODS Wistar rats were injected intraperitoneally with 1 mL of saline (0.9% NaCl), BCAA (30 mg), glucose (100 mg), or glucose plus BCAA 5 min before either 45 min of submaximal exercise (N = 32) or running to exhaustion (N = 24). After the submaximal exercise test, blood was collected for the measurement of ammonia, BCAA, free tryptophan (free TRP), glucose, free fatty acid, and lactic acid, and muscle samples were taken from the m. soleus for determination of glycogen content. RESULTS Mean run time to exhaustion was significantly longer after BCAA administration (158+/-26 min) compared with that after saline (118+/-35 min)(P<0.05) but not compared with that after glucose administration (179+/-21 min). When glucose is administered before exercise, the supplementary administration of BCAA had no additional effect on performance (171+/-12 min). The data on blood ammonia, ratio of free TRP/BCAA, and muscle glycogen did not provide a clue for explaining the higher endurance performance after BCAA supplementation. CONCLUSION The results support the hypothesis that the effect of BCAA administration on performance could be related to carbohydrate availability during exercise.

Low-Dose Dobutamine Stress Echocardiography in Children and Young Adults

Dobutamine stress echocardiography has become widely accepted in the evaluation of adult patients with coronary heart disease. We wanted to assess the feasibility and the physiologic responses of stress echocardiography at low doses of dobutamine in a population of normal children and adults. Once achieved, we submitted a group of post-anthracycline patients to the test to assess the sensitivity of low-dose dobutamine stress echocardiography in the detection of cardiac dysfunction. Thirty-two healthy children and young adults (19 male and 13 female subjects, median age 15 years [range 6 to 26]) were studied. After the initial study of normal subjects, we submitted a cohort of 39 patients (18 female and 21 male, aged 6 to 25 years), who completed anthracycline chemotherapy, to the same protocol. Dobutamine was infused at rates of 0.5 to 2.5 and 5 microg/kg/min and echocardiographic measurements were obtained at rest and at the end of each stage. The test could be completed in 100% of the subjects without major complications. Statistically significant differences between resting echocardiographic values of systolic and diastolic function and values at 2.5 and 5 microg/kg/min of dobutamine were found. Moreover, dobutamine revealed or enhanced differences between normal subjects and the post-anthracycline patients. Thus, low-dose dobutamine stress echocardiography is feasible and safe in older children. The test is very sensitive for the detection of subclinical cardiac dysfunction in post-anthracycline patients and could possibly assess functional myocardial reserve.

Impact of oxidative stress on the endothelial dysfunction of children and adolescents with type 1 diabetes mellitus: protection by superoxide dismutase?

Diabetes mellitus is associated with endothelial dysfunction and oxidative stress (OS). We investigated whether these abnormalities are interrelated in children and adolescents with type 1 diabetes mellitus (T1DM) and if early OS markers predictive of vascular dysfunction can be identified. Thirty-five T1DM patients were matched for sex, age, height, and weight with nondiabetic subjects as healthy controls (CO). Flow-mediated dilatation (FMD), carotid intima media thickness (IMT), and OS status in fasting blood were measured. Diabetic children had impaired FMD (6.68 ± 1.98 versus 7.92 ± 1.60% in CO, p = 0.004), which was more pronounced in boys. The degree of FMD impairment was not related to the lower plasma levels of antioxidants or to the higher glucose, glycation, lipids, and peroxidation products. Erythrocyte superoxide dismutase activity, copper/zinc superoxide dismutase (Cu/Zn SOD), was higher in diabetic subjects (1008 ± 224 versus 845 ± 195 U/g Hb in CO, p = 0.003) and was positively associated with FMD. After correcting for diabetes and gender, the subgroup of children with high Cu/Zn SOD (>955 U/g Hb) had a significantly better FMD (p = 0.035). These results suggest that higher circulating Cu/Zn SOD could protect T1DM children and adolescents against endothelial dysfunction. Low Cu/Zn SOD is a potential early marker of susceptibility to diabetic vascular disease.

The role of acceptance in psychological functioning in adolescents with cystic fibrosis: A preliminary study

This study explored the role of acceptance in accounting for the heterogeneity in psychological functioning in adolescents suffering from cystic fibrosis. Thirty-four adolescents completed a battery of questionnaires assessing acceptance, anxiety, depression, and disability. Regression analyses revealed that acceptance had a significant and unique contribution in explaining adolescents’ anxiety, depression, and disability beyond the effects of demographic variables and parameters of disease severity. Forced expiratory volume, a parameter of disease severity, had a unique contribution in explaining disability, but not in explaining anxiety and depression. Our results support the idea that accepting the limitations imposed by a chronic disease and readjusting life goals has a positive effect upon psychological functioning in adolescents with cystic fibrosis. Acceptance-based therapies might prove useful in promoting well-being in adolescents with cystic fibrosis.

Acceptance and Well-Being in Adolescents and Young Adults with Cystic Fibrosis: A Prospective Study

OBJECTIVE To prospectively investigate the role of acceptance in well-being in adolescents and young adults with cystic fibrosis (CF). METHOD A total of 40 adolescents and young adults with CF (ages 14-22 years) completed questionnaires assessing acceptance, anxiety and depressive symptoms, physical functioning, role functioning, emotional functioning, and social functioning. After 6 months, 28 of them completed the questionnaires on anxiety and depressive symptoms, physical functioning, role functioning, emotional functioning, and social functioning a second time. RESULTS More acceptance (Time 1) was related to less depressive symptoms (Time 1 and 2), and to better role, emotional, and social functioning (Time 1). CONCLUSIONS Results indicate that accepting the limitations imposed by chronic disease and readjusting life goals may have a positive effect upon well-being in adolescents and young adults with CF. Further research is needed to clarify whether acceptance-based interventions are useful in promoting well-being in adolescents and young adults with CF.

Diagnostic Value of Immunostaining in Cultured Skin Fibroblasts from Patients with Oxidative Phosphorylation Defects

In the last decades, a large variety of oxidative phosphorylation (OXPHOS) defects have been reported, expressed as an increasing variety of clinical phenotypes. With the expanding number of genes and proteins involved, new screening techniques leading to more effective diagnostic routes are in ever-increasing demand. Cultured skin fibroblasts from a cohort of patients with various OXPHOS defects, previously recognized by enzyme activity studies and blue native PAGE, were investigated with an immunocytochemical technique. Cytospins of cultured fibroblasts were air dried, fixed, and stained with antibodies specifically directed against subunits of each OXPHOS complex. Control cells stained homogeneously and strongly. In fibroblasts from five out of seven patients with a severe deficiency of one of the OXPHOS complexes, a homogeneous reduction of cytoimmunoreactivity of the affected complex was observed. In five out of seven fibroblast strains harboring a mitochondrial tRNA mutation, a mosaic pattern of staining was observed for both complexes I and IV, reflecting the heteroplasmic nature of the defect. The proportion of deficient fibroblasts varied considerably between cell strains from different subjects. The method described offers a convenient and rapid approach to first-line screening of OXPHOS defects. In association with routine assays of enzyme activity, the technique is helpful in orienting molecular investigation further.

Lack of increase in stroke volume during exercise in asymptomatic adolescents in sinus rhythm after intraatrial repair for simple transposition of the great arteries

We studied stroke volume during exercise in 12 male asymptomatic patients in sinus rhythm after intraatrial repair for d-transposition of the great arteries. Data indicate that during exercise, stroke volume did not increase in the patient group, while in control subjects stroke volume increased.

Major coronary anomalies in childhood

Abstract. Major coronary artery anomalies are extremely rare in childhood. We wanted to assess the historical and diagnostic features and the therapeutic options of three distinct types of coronary artery anomalies: abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA), coronary fistula and coronary stenosis. In a retrospective study, 33 children with these types of coronary artery disease were identified, 15 with ALCAPA, 12 with fistula and six with coronary stenosis. History, physical examination, ECG, X-ray, echocardiography, angiography, therapy and outcome were reviewed. ALCAPA showed distinct typical echocardiographic characteristics. Coronary artery fistula could be identified by a typical murmur and echocardiographic evidence of coronary dilatations. Coronary stenosis should be suspected by the clinical picture in a specific context. Conclusion: rare coronary artery anomalies can be accurately diagnosed in childhood. Timely therapy yields good prognosis.