Complete occlusion of the left circumflex coronary artery after radiofrequency catheter ablation in an infant

Radiofrequency catheter ablation of a left lateral accessory atrioventricular pathway was performed in a 5-week-old infant with drug-refractory supraventricular tachycardia. Energy application via a 5-French mapping and ablation catheter in the temperature-controlled mode (60 degrees C, 30 W) at the atrial aspect of the mitral valve annulus repeatedly resulted in termination of the tachycardia by conduction block within the pathway. Tachycardia remained inducible subsequently. After a safety energy application during sinus rhythm, significant ST-segment elevation in the inferior, mid precordial, and left lateral leads was noted. Selective left coronary angiography revealed complete occlusion of the circumflex coronary artery. Moderate-to-severe mitral valve regurgitation developed, finally requiring mitral valve replacement.     

Management of skin,mucosa and joint involvement of Behçet’s syndrome: A systematic review for update of the EULAR recommendations for the management of Behçet’s syndrome

Objectives

The aim of this systematic review was to inform the update of European League Against Rheumatism (EULAR) Recommendations for the management of Behçet’s syndrome (BS), on the evidence for the treatment of skin, mucosa and joint involvement of BS.

The Influence of Iron Loading and Iron Chelation on the Proliferation and Telomerase Activity of Human Peripheral Blood Mononuclear Cells

Background: Iron is an essential trace element in cell proliferation. Several investigations demonstrate that iron deprivation inhibits cell proliferation. However, the impact of iron on telomerase activity of activated lymphocytes remains unexplained to date. Objective: In this study, the effect of iron on the proliferation and telomerase activity of lymphocytes stimulated by phytohemagglutinin (PHA) were investigated. Methods: Iron loading was performed by incubating peripheral blood mononuclear cells in 500μM FeSO4.7H2O for 24 h and iron chelation was done by exposing cells to desferrioxamine, a potent iron chelator. The effects of silymarin, a flavonoid with both antioxidant and iron chelating activities, on the proliferation and telomerase activity of PHAactivated lymphocytes were also compared with desferrioxamine. Proliferation and telomerase activity were assessed using BrdU incorporation assay and Telomeric Repeat Amplification Protocol (TRAP), respectively. Results: The proliferations of lymphocytes were significantly inhibited by 10 and 20 μg/ml desferrioxamine in a dose dependent manner, while iron loading recovered suppressed cell proliferation to the normal level. Silymarin at 20 μg/ml significantly increased the proliferation of lymphocytes in both normal and iron-treated conditions. Telomerase activity of lymphocytes was markedly increased by iron treatment and suppressed by desferrioxamine. Conversely, iron treatment had no effect on the telomerase activity of lymphocytes incubated with silymarin. Conclusion: Iron plays a significant role in the proliferation and telomerase activity of lymphocytes. The effects of silymarin on the proliferation and telomerase activity of lymphocytes were completely different from those of desferrioxamine, suggesting that the immunomodulatory effect of silymarin is probably not associated with its iron chelating activity.     

First Report of a Dominantly Inherited β-Thalassemia Caused by a Novel Elongated β-Globin Chain

A distinct set of mutations on the β-globin gene leads to dominantly inherited β-thalassemia (β-thal) that is associated with a disease phenotype in a single mutant copy. We described molecular and hematological characteristics of a novel elongated β-globin chain in combination with a known hemoglobin (Hb) variant (N-Baltimore or HBB: c.286A>G) in cis. The highly unstable Hb variant caused typical features of β-thal major (β-TM) or β-thal intermedia (β-TI) in two members of a family depending on their α-globin genotypes. The β mutant allele of the mother was transmitted in an autosomal dominant fashion to her daughter. They resemble severe forms of β-thal due to ineffective erythropoiesis. Taken together with previously published data, this result indicates that a dominant form of β-thal should be regarded as a phenotypic term of hemoglobinopathies caused by β chain variants that are highly unstable.     

Prognostic value of positive T wave in lead aVR in patients with non‐ST segment myocardial infarction

Background

Lead aVR provides prognostic information in various settings in patients with ischemia. We aim to investigate the role of a positive T wave in lead aVR in non‐ST segment myocardial infarction (NSTEMI).

Methods

In a prospective cohort study, we included 400 patients with NSTEMI. Presentation electrocardiogram (ECG) was investigated for presence of a positive T wave as well as ST segment elevation (STE) in aVR and study variables were compared. Predictors of primary outcome defined as hospital major adverse cardiovascular events (MACE) and secondary outcome, defined as three‐vessel coronary disease and/or left main coronary artery stenosis (3VD/LMCA) stenosis in angiography, were determined in multivariate logistic regression analysis.

Results

Patients with a positive T wave in aVR were significantly older and were more likely to be female. Left ventricular ejection fraction was significantly lower in patients of positive T group. Positive T group was more likely to have 3VD/LMCA stenosis (58.3% vs. 19.8%, p < .001). The prevalence of a positive T wave in aVR was significantly higher in MACE group (54.9 % vs. 24.8%, p < .001). However, in multivariate analysis, it was not an independent predictor of MACE (OR: 1.083 95% CI: [0.496–2.365], p: .841). Though, it was independently associated with presence of 3VD/LMCA stenosis (OR: 3.747 95% CI: [2.058–6.822], p < .001).

Conclusion

Though positive T wave in lead aVR was more common in patients with MACE; it was not an independent predictor. Additionally, a positive T wave in aVR was an independent predictor of 3VD/LMCA stenosis in NSTEMI.

     

Proposed outcome measures for prospective clinical trials in juvenile idiopathic arthritis–associated uveitis: A consensus effort from the multinational interdisciplinary working group for uveitis in childhood

Objective

To develop a set of core outcome measures for use in randomized controlled trials (RCTs) and longitudinal observational studies in juvenile idiopathic arthritis (JIA)–associated uveitis.

Methods

The literature relating to outcome measures used in studies of uveitis in childhood and adolescence was reviewed. A set of core outcomes and domains was established using the Delphi process. This was reviewed by a representative multinational interdisciplinary working group. Nominal group technique consensus was reached on face and content validity of the range and content of the domains. The outcomes and the appropriate instruments for uveitis trials were adapted to the age ranges of patients with JIA‐associated uveitis.

Results

Consensus was reached that data should be reported at defined time points in longitudinal studies with patients stratified by prognostic markers. Visual acuity testing should be age appropriate. The severity of uveitis (measured as anterior chamber cell grade) and duration of active inflammation should be documented. Visually significant structural complications should be recorded and quantified with standard measures. The responses to treatment and corticosteroid‐sparing effects of treatment should be documented. Patient‐reported disease activity and age‐specific uveitis‐related quality of life should be reported using appropriate questionnaires.

Conclusion

The proposed outcome measures in JIA‐associated uveitis should aid in the standardization and comparison of future RCTs of the treatment regimens for this disease. The proposed outcome measures will be verified in a prospective validation study.     

Evaluation of rabbit antibody response against 8 and 16 kDa recombinant subunits of antigen B from Echinococcus granulosus

ObjectiveTo immunize rabbits with 12 and 16 kDa recombinant subunits of antigen B from Echinococcus granulosus (E. granulosus) and measuring polyclonal antibody and humoral immune response using ELISA and gel diffusion.MethodsTwo mentioned antigens were cloned and expressed in expression vector and purified by affinity chromatography. Four young rabbits were selected and challenged intradermally with yielded recombinant antigens. Rabbits' sera were collected post infection and were tested using ELISA and gel diffusion for polyclonal antibody detection 10 days after last injection.ResultsThe specific antibody against the recombinant peptides was efficiently produced within 4 weeks post infection.ConclusionsProduced recombinants proteins could induce the immune response of the rabbits successfully. This process might improve the clarification of diagnosis and vaccination as regards hydatidosis.     

Surgical management of cataract and posterior chamber intraocular lens implantation in Fuchs‘ heterochromic iridocyclitis

Background: To determine the inflammatory course and level of visual rehabilitation after cataract extraction and posterior chamber lens implantation in patients withFuchs‘ heterochromic iridocyclitis complicated by cataract. Methods: In a clinical trial, 32 eyes (visual acuity of 20/160 or worse) of 30 patients underwent extracapsular cataract extraction (19 eyes) or lensectomy(13 eyes) accompanied by posterior chamber intraocular lens implantation. Indirect ophthalmoscopy was performed intraoperatively prior to intraocular lens implantation and the extent of vitreous haze was assessed. If vitreous haziness was 3+ or more, core vitrectomy (two eyes) or three-port pars plana deep vitrectomy (four eyes) was performed. Results: After an average follow up of14.4 months (6 to 24 months), there was no statistically significant increase in cell and flare in the anterior chamber and vitreous or in keratic precipitates compared with the preoperative status of the eyes. However, 12%of the eyes developed synechiae (anterior and/or posterior) in comparison to preoperative condition (p< 0.05). Eighty-seven percent of the eyes gained visual acuity of 20/40 or better (P < 0.005). Using the logistic regression model, a higher level of preoperative inflammation was associated with reduced likelihood of gaining visual acuity of 20/25 or more (OR = 0.25, 95%CI 0.049, 1.255). The complications of surgery were synechiae, 12%; opaque posterior capsule, 12%; vitreous loss, 3%; chronic glaucoma, 3%; and retinal detachment,3%. Conclusions: Implantation of a posterior chamber intraocular lens can be well tolerated in patients with Fuchs‘ heterochromic iridocyclitis. Vitrectomy, whenever indicated in addition to cataract extraction, improves visual rehabilitation. This revised version was published online in August 2006 with corrections to the Cover Date.     

The role of MSX1 in tooth agenesis in Iranians

INTRODUCTION: MSX1 gene has a critical role in craniofacial development, the aim of this case-control study is to test the hypothesis that MSX1 mutation contributes to congenital tooth agenesis in Iranians. MATERIALS AND METHODS: The study group consisted of 20 affected individuals with tooth agenesis of lower second premolars or upper lateral incisors with mean age of 24.6. The control group consisted of 20 unaffected individuals. DNA was extracted from all 40 individuals; the polymerase chain reaction (PCR) for MSX1 was carried out with Phenol: Chloroform: Isoamylalchol (PCI) extraction method. Ban II restriction digest and agarose gel electrophoresis of the 20 affected individuals verified the presence of mutation in all 20 affected individuals. The unaffected controls did not show any mutation. Statistical analysis performed by the chi-squared method. RESULTS: Ban II did not digest PCR product (DNA) in the control group (195 bp band on electrophoresis gel) but digested the affected allele (106 bp and 89 bp bands). There is a statistically significant correlation between tooth agenesis and MSX1 mutation (P < 0.001). CONCLUSION: The results indicated that MSX1 gene mutation contributes to tooth agenesis in Iranian individuals. As the timing of tooth calcification can vary, radiographic finding of congenital tooth agenesis can be confirmed by this molecular method during different dental ages to achieve certainty.