Validity and reliability of “Shriners Hospital for Children Upper Extremity Evaluation” in children with rheumatic diseases

Clinical Rheumatology - The aim of this study was to investigate the validity and reliability of “Shriners Hospital for Children Upper Extremity Evaluation (SHUEE)” for children with...     

Metabolic syndrome is a predictor for an ECG sign of no-reflow after primary PCI in patients with acute ST-elevation myocardial infarction

Background and aimThe purpose of this study was to evaluate both the predictive value of metabolic syndrome (MS) on no-reflow phenomenon and 30-day clinical outcomes on patients undergoing primary percutaneous coronary intervention (PCI) for acute ST-elevation myocardial infarction (STEMI).Methods and resultsOne hundred and twelve consecutive patients (mean age 57 ± 11 years, 94 male) with acute STEMI treated with primary PCI were analysed prospectively. Sum of ST-segment elevation was obtained immediately before and 60 min after the restoration of TIMI-3 flow. The difference between two measurements was accepted as the amount of ST-segment resolution and was expressed as ∑STR. ∑STR < 50% was accepted as ECG sign of no-reflow phenomenon. Metabolic syndrome was defined based on Adult Treatment Panel-III criteria.The no-reflow was found in 22.3% of the entire group and was significantly higher in patients with MS than those without MS (43.7% vs. 13.7%, p < 0.001). There was no significant difference in no-reflow between patients who had both MS and diabetes mellitus (DM) and patients who had MS but not DM (42.5% vs. 50%, respectively; p > 0.05).ConclusionThe presence of MS may play an important role in the occurrence of no- reflow in STEMI treated with primary PCI.     

The effects of thyroxine replacement on the levels of serum asymmetric dimethylarginine (ADMA) and other biochemical cardiovascular risk markers in patients with subclinical hypothyroidism

BACKGROUND: The relationship between subclinical hypothyroidism (SH) and cardiovascular disease (CVD) is still under debate. The purpose of the present study was to evaluate plasma total homocysteine (tHcy), high sensitive C-reactive protein (hsCRP), small dense low-density lipoprotein (sdLDL), L-arginine and asymmetric dimethylarginine (ADMA) concentrations and their relationship to nitric oxide (NO) production, measured as plasma nitrite-plus-nitrate (NO(x)) concentration, in patients with SH before and after thyroxine replacement therapy and compared with control group values. DESIGN: Eighty-four women with SH and 33 healthy women as controls matched to the patient group for sex, age and body mass index (BMI), were enrolled in this study. Lipoprotein profile, tHcy, hsCRP, sdLDL, ADMA, L-arginine and NO(x) were measured in pre- and post-treatment blood samples. RESULTS: The pretreatment total cholesterol (TC), LDL-C, hsCRP, ADMA and L-arginine levels were significantly higher and NO(x) levels were lower than in the control group. After treatment, hsCRP, ADMA and L-arginine levels were significantly reduced and sdLDL and NO(x) levels were significantly increased. CONCLUSION: The present study demonstrated an elevation of hsCRP and ADMA plasma levels of patients with SH associated with a reduction in NO production, which may contribute to some cardiovascular alterations. The elevated ADMA and hsCRP levels were reduced after thyroxine replacement. Also the sdLDL levels of SH patients were found to be lower than the control group values whereas TC and LDL were elevated. Even though we found an elevation in sdLDL levels after treatment, those values were still not higher than in the control group.     

Death rituals,religious beliefs,and grief of Turkish women

Grief following a death loss is a common experience that all individuals face at some point in life. There, however, are only a few in-depth studies regarding grief in cultures around the world and specific roles that rituals and beliefs related to death may have in the grieving process. Results of interview data from eight grieving Turkish women revealed three themes: (a) metaphors of loss, (b) funeral rituals, and (c) rituals in relation to control and personal factors. Overall, participants’ sense of control appeared to influence their grief experiences and perceptions of rituals.     

Is it safe to use anti-TNF-α agents for tuberculosis in children suffering with chronic rheumatic disease?

To determine the incidence of latent tuberculosis infection and evaluate the follow-up protocol of the patients diagnosed with juvenile idiopathic arthritis (JIA) and other chronic rheumatologic diseases treated with anti-TNF-α treatment (etanercept, infliximab, adalimumab) in Turkey, 144 patients were evaluated retrospectively for the development of tuberculosis. Patients were evaluated every 6?months for tuberculosis using history, physical examination, tuberculin skin test (TST), chest radiographs, and, when required, examination of sputum/early morning gastric aspirates for acid-fast bacilli and chest tomography. A tuberculin skin test over 10?mm induration was interpreted as positive. Patients were diagnosed with JIA (n?=?132), enthesitis-related arthritis (ERA; n?=?14), juvenile psoriatic arthritis (JPsA; n?=?4), chronic idiopathic uveitis (n?=?4), and chronic arthritis related to FMF (n?=?8). Mean age was 12.25?±?3.96?years (4.08–19.41?years), mean duration of illness was 5.86?±?3.77?years (0.66–15?years), and the mean duration of anti-TNF-α treatment was 2.41?±?1.47?years (0.6–7?years). Anti-TNF-α agents prescribed were etanercept (n?=?133), infliximab (n?=?30), and adalimumab (n?=?6). When unresponsive to one anti-TNF-α therapy, patients were switched to another. There was no history of contact with individuals having tuberculosis. During follow-up, seven patients (4.8%) with positive TST were given INH prophylaxis. One oligoarticular JIA patient (0.69%) diagnosed with secondary uveitis who had been followed for 5?years and had been using infliximab for 2?years, developed a positive Quantiferon-TB test while on INH prophylaxis. He was started on an anti-tuberculosis drug regimen. In conclusion, anti-TNF-α treatment in children with chronic inflammatory disease is safe. Follow-up every 6?months of children on anti-TNF-α treatment with respect to tuberculosis by the pediatric infectious disease department is important to prevent possible complications.     

Nemaline myopathy in a newborn infant: a rare muscle disorder

Nemaline myopathy (NM) is a genetically and clinically heterogeneous muscle disorder, defined by the presence of characteristic nemaline bodies on muscle biopsy. The disease has a wide spectrum of phenotypes, ranging from forms with neonatal onset and fatal outcome to asymptomatic forms. The neonatal form is severe and usually fatal. The clinical variability, with differing age of onset and severity of symptoms makes the diagnosis difficult during infancy. There is no curative treatment. L-tyrosine may prevent aspiration by reducing pharyngeal secretions and drooling. Most of the patients die from respiratory and cardiac failure.This article discusses a newborn infant who presented with generalized weakness and respiratory failure. Partial response to L-tyrosine treatment was noted. The case is worth presenting to remind clinicians of congenital myopathies in the differential diagnosis of floppy infant during neonatal period and to emphasize the importance of muscle biopsy in diagnosis.     

Sensorimotor polyneuropathy in patients with SMA type‐1: Electroneuromyographic findings

Introduction: Generally, spinal muscular atrophy (SMA) is believed to be a pure motor neuron disease. We retrospectively evaluated our electrodiagnostic findings in SMA type 1 patients to demonstrate co‐existence of sensorimotor neuropathies. Methods: Electroneuromyographic (ENMG) studies in 15 patients (11 boys, 4 girls) were reviewed independently by 2 neurophysiologists. Upper extremity findings were compared with normal right arm controls. Results: Patient ages ranged from 1.5 to 26 months. Four SMA patients (26.7%) had decreased sensory nerve action potentials (SNAPs) or sensory nerve conduction velocities. Of them, median SNAPs could not be elicited in 3, and sural SNAPs could not be elicited in 2. Compound muscle action potential amplitudes were severely decreased in 14 (93.3%) and normal in 1. Conclusions: Survival motor neuron 1 (SMN1) gene analysis should be considered if clinical features are consistent with SMA, even if pathological or electrophysiological findings demonstrate peripheral sensorimotor polyneuropathies. Muscle Nerve, 2013