Neuromuscular differences between boys with and without intellectual disability during squat jump

The purpose of this study was to identify the differences in vertical squat jump (SJ) between volunteers with and without intellectual disability (ID). Thirteen boys with ID (average intelligence quotient, estimated by Wisk III test: 55.6 ± 11.2) and 13 peers without disabilities performed maximal SJ on a force platform. Kinematic data were captured using a six-camera 3D motion analysis system and electromyographic (EMG) activity was recorded using surface electrodes. Unpaired T-test determined the statistical difference between the two groups. The obtained results indicated that the group with ID, jumped lower, developed lower vertical ground reaction forces, knee power output, knee angular velocity, and take-off velocity, and showed longer propulsion duration, decreased mean to maximum agonist EMG activity and higher antagonist/agonist activity ratio. The deficit in the SJ observed in individuals with ID was attributed to a deficit in the examined mechanical and neuromuscular parameters, and especially to the agonist and antagonist co-contraction.     

Sleep disorders in pediatric chronic kidney disease patients

The prevalence of sleep disorders during childhood has been estimated to range from 25 to 43 %. The aim of this review is to determine the prevalence of sleep disorders and possible associations with chronic kidney disease (CKD)-related factors and health-related quality of life (HRQOL) in children with CKD. An electronic systematic literature search for sleep disorders in children with CKD in Pubmed, Embase and the Cochrane Library Databases identified seven relevant articles for review, all of which reported an increased prevalence of sleep disorders in children with CKD. Five studies included children with CKD undergoing dialysis, and two studies included only non-dialysis patients. In all studies the presence of sleep disturbances was assessed by questionnaires; only one study compared the results of a validated questionnaire with laboratory-based polysomnography. The prevalence of any sleep disorder ranged from 77 to 85 % in dialysis patients, to 32–50 % in transplanted patients and 40–50 % in non-dialysis patients. The most commonly studied disorder was restless legs syndrome, which presented at a prevalence of 10–35 %. Three studies showed significant associations between presence of sleep disorders and HRQOL. We found consistent evidence of an increased prevalence of sleep disturbances in children with CKD, and these seemed to play a critical role in HRQOL.     

Peritonitis in children with automated peritoneal dialysis: a single-center study of a 10-year experience

Peritoneal dialysis (PD) constitutes the preferred dialysis modality for children requiring renal replacement therapy with peritonitis being one of the most common complications of PD. This study was performed to evaluate the epidemiology, microbiology, and outcomes of PD-associated peritonitis in Greek children for a 10-year period. A total of 27 patients (16 males) with a mean age 121.8?±?57.2 months were retrospective analyzed. Patients were on PD therapy for a mean duration of 45.2?±?26.1 months. We found 23 episodes of PD-associated peritonitis occurred in 9 out of 27 patients (0.23 episodes/patient-year), with four patients experienced two or more peritonitis episodes. Gram-positive bacteria were responsible for 15 (65.2%) peritonitis episodes, with Staphylococcus aureus being the predominant specie isolated in 30.4% of cases. A total of seven episodes of exit-site infections (ESIs) were identified in five patients (0.069 episodes/patient-year) with the most common bacteria isolated being S. aureus (57.4%). Initial antibiotic treatment included intraperitoneal vancomycin plus ceftazidime in the majority of cases (82.6%). At the end of study, 12 (44.4%) patients remained on PD, 11 (41.8%) underwent renal transplantation, 2 (7.4%) shifted to hemodialysis and unfortunately, two patients (7.4%) died. Conclusively, our study revealed a noticeable low peritonitis and ESIs rate as compared to international data and represents the first evaluation of the characteristics and outcomes of peritonitis in the Greek pediatric PD population.     

The ryanodine receptor agonist 4-chloro-3-ethylphenol blocks ORAI store-operated channels

BACKGROUND

Depletion of the Ca²⁺ store by ryanodine receptor (RyR) agonists induces store-operated Ca²⁺ entry (SOCE). 4-Chloro-3-ethylphenol (4-CEP) and 4-chloro-m-cresol (4-CmC) are RyR agonists commonly used as research tools and diagnostic reagents for malignant hyperthermia. Here, we investigated the effects of 4-CEP and its analogues on SOCE.

EXPERIMENTAL APPROACH

SOCE and ORAI1-3 currents were recorded by Ca²⁺ imaging and whole-cell patch recordings in rat L6 myoblasts and in HEK293 cells overexpressing STIM1/ORAI1-3.

KEY RESULTS

4-CEP induced a significant release of Ca²⁺ in rat L6 myoblasts, but inhibited SOCE. The inhibitory effect was concentration-dependent and more potent than its analogues 4-CmC and 4-chlorophenol (4-ClP). In the HEK293 T-REx cells overexpressing STIM1/ORAI1-3, 4-CEP inhibited the ORAI1, ORAI2 and ORAI3 currents evoked by thapsigargin. The 2-APB-induced ORAI3 current was also blocked by 4-CEP. This inhibitory effect was reversible and independent of the Ca²⁺ release. The two analogues, 4-CmC and 4-ClP, also inhibited the ORAI1-3 channels. Excised patch and intracellular application of 4-CEP demonstrated that the action site was located extracellularly. Moreover, 4-CEP evoked STIM1 translocation and subplasmalemmal clustering through its Ca²⁺ store-depleting effect via the activation of RyR, but no effect on STIM1 redistribution was observed in cells co-expressing STIM1/ORAI1-3.

CONCLUSION AND IMPLICATIONS

4-CEP not only acts as a RyR agonist to deplete the Ca²⁺ store and trigger STIM1 subplasmalemmal translocation and clustering, but also directly inhibits ORAI1-3 channels. These findings demonstrate a novel pharmacological property for the chlorophenol derivatives that act as RyR agonists.     

Association between TNF-alpha -308G>A polymorphism and the development of acute coronary syndromes in Greek subjects: the CARDIO2000-GENE Study.

PURPOSE: We investigated the association of a polymorphism within the promoter of TauNuF-alpha locus at the position -308 on the likelihood of having acute coronary syndromes (ACS) in Greek adults. METHODS: We studied demographic, lifestyle, and clinical information in 237 hospitalized patients (185 males) with a first event of an ACS and 237 matched by age and sex (controls) without any clinical evidence of coronary heart disease. Genotyping was performed by PCR-RFLP analysis. RESULTS: The genotype frequencies were in patients, 87% (n = 206), 12% (n = 29), and 1% (n = 2) for G/G, G/A, and A/A, and in controls, 96% (n = 227), 4% (n = 10), and 0% (n = 0) for G/G, G/A, and A/A, respectively (P = 0.04). After adjusting for age and sex, as well as various potential confounders, we observed that G/A or A/A genotypes were associated with 1.94-fold higher odds (95% CI 1.06 to 3.68) of ACS compared to G/G homozygotes. No gene to-gender or to-clinical syndrome interactions were observed. Further subgroup analysis showed that the distribution of TNF-alpha -308G>A polymorphism was associated with the presence of family history of CHD in patients, but not in controls. In particular, in G/A and A/A patients 17.2% reported family history of CHD, whereas in G/G patients, 34.5% reported family history (P = 0.036). CONCLUSIONS: Our findings may state a hypothesis of an association between the -308G>A TNF-alpha polymorphism the development of ACS and the presence of family history of CHD, in Greece.     

Outcome of pediatric patients treated with extracorporeal life support after cardiac surgery

Background

Extracorporeal life support (ECLS) has been used for over two decades in select patients after cardiac surgery. We previously described factors associated with death in this population. We now review our recent experience to reassess factors related to mortality.

Methods

All pediatric patients who received ECLS support within 7 days after surgery between July 1995 and June 2001 were examined to describe clinical features associated with survival. We compared the results with our prior report to assess changes in practice and outcome.

Results

Seventy-four patients were followed. Fifty percent survived to discharge. Hospital survival was not significantly related to patient age, cannulation site, or indication. Thirty-five percent of patients required hemofiltration while on ECLS and were significantly less likely to survive (23% vs 65%). A multivariate analysis combining all children from our prior report with the present cohort revealed that patients who received hemofiltration were five times more likely to die (odds ratio 5.01, 95% confidence interval 2.11-11.88). Children with an adequate two-ventricular repair had lower risk of death (odds ratio 0.42, 95% confidence interval 0.19-0.91) after adjusting for patient age, study period, and hours elapsed before initiation of ECLS after surgery.

Conclusions

Patients with an adequate two-ventricle repair have significantly higher hospital survival, whereas those with single ventricle physiology or need for dialysis have decreased survival.     

Endometrial injury for RIF patients undergoing IVF/ICSI: a prospective nonrandomized controlled trial

To evaluate the effect of endometrial injury on clinical outcomes in subfertile women with repeated implantation failures (RIF) undergoing assisted reproduction. In this prospective nonrandomized controlled trial, 103 subfertile women with RIF were included. Fifty-one underwent endometrial injury through hysteroscopy in the early follicular phase of the previous cycle and 52 underwent the standard protocol without any intervention. Live birth and miscarriage were the primary outcomes. Clinical and in vitro fertilization (IVF) cycle characteristics, were also compared between groups. Both groups were comparable in terms of baseline and cycle characteristics. Live birth rates were significantly higher in the study, compared with the control group (18/51 vs. 8/52, odds ratio (OR)?=?0.25; 95% confidence interval (CI)?=?0.10–0.64; p?=?0.020), although miscarriage rates were similar (7/51 vs. 10/52, OR=?0.25; 95%CI=?0.12–0.66; p?=?0.452). The rest of the outcomes parameters were comparable between groups. Logistic regression analysis revealed that endometrial injury and duration of subfertility were independent predictors of live birth after control of other variables (OR?=?2.818; 95%CI?=?1.044–7.605; p?=?0.041 and OR?=?0.674; 95%CI?=?0.461–0.985, p?=?0.042, respectively). Endometrial injury induced through office hysteroscopy in the preceding cycle in subfertile women with RIF improves live birth rates.