Serologic response to Epstein-Barr virus antigens in patients with systemic lupus erythematosus: a controlled study

Previous studies showed a link between systemic lupus erythematosus (SLE) and Epstein-Barr virus (EBV) infection. We sought to determine the features of serologic response to EBV in SLE patients and whether this response differs from those of systemic sclerosis (SSc) and primary antiphospholipid syndrome (PAPS) patients as well as healthy individuals. Sera from 198 consecutive SLE patients have been tested to detect IgG antibodies to EA/D, EBNA-1, VCA P18 and for comparison, cytomegalovirus (CMV) using commercially available ELISA kits (Trinity Biotech, USA). Forty-six SSc patients and 38 PAPS patients were enrolled as diseased control groups and sixty-five individuals as healthy controls. Significantly more SLE (54%, P?=?0.001, OR 5.77, 95% CI 2.8?C11.6), SSc (41.3%, P?=?0.005, OR 3.4, 95% CI 1.4?C8.2) and PAPS sera (36.8%, P?=?0.023, OR 2.86, 95% CI 1.14?C7.22) reacted against EA/D than healthy controls (16.9%). The mean age of anti-EA/D-positive SLE patients was significantly higher, and their disease duration was longer compared to anti-EA/D-negative SLE patients (41 ± 14 vs. 33.8 ± 10.8?years, P?<?0.001 and 100 ± 73 vs. 71 ± 62?months, P?=?0.003). In SLE patients, EA/D reactivity was associated with Raynaud??s phenomenon and the presence of any anti-ENA antibodies. Although it did not reach a statistical significance, anti-EBNA-1 reactivity was slightly lower in patients with SLE. The frequency of anti-CMV Ig G positivity was found significantly higher in SLE patients (100%) when compared to patients with SSc (95.7%), PAPS (94.7%) and healthy controls (95.4%) (P?=?0.035, P?=?0.025 and P?=?0.015 respectively). Our results support the proposed link between EBV and SLE. The finding that SSc and PAPS patients also have increased frequency of anti-EA/D response has revealed that this immune interaction may not be unique to patients with SLE, and there may be a common mechanism involving EBV in these autoimmune diseases.     

Incidence of Appropriate Shock in Implantable Cardioverter-Defibrillator Patients With Improved Ejection Fraction

BackgroundHeart failure with recovered ejection fraction (EF) is a recently described clinical entity. There is insufficient information on the management of implantable-cardioverter defibrillator (ICD) patients with improved EF at generator replacement.Methods and ResultsWe examined the incidence of appropriate shocks in 91 consecutive patients with ICDs for primary prevention of sudden death who underwent generator replacement. Improved EF was defined as both EF >35% at generator replacement and increase in EF by ≥10% since original implantation. Patients were 70 ± 11 years old, and 76% had ischemic cardiomyopathy. At generator replacement, 25 patients (27%) had improved EF (0.49 ± 0.08 vs 0.31 ± 0.07 at baseline; P < .0001). Over 6.2 ± 2.2 years of follow-up after original implantation, 9 patients (36%) with improved EF versus 19 (29%) with unchanged EF had appropriate ICD shocks (P = .51). Incidence of appropriate ICD shocks was similar between the two groups before (P = .90) and after (P = .97) generator replacement. Of the 9 improved EF patients with appropriate shock, 4 had shocks before generator replacement, 2 had shocks before and after generator replacement, and 3 patients, who never had shocks before, had their first shock after generator replacement.ConclusionsSome ICD patients whose EF improves to >35% at generator replacement remain at risk for appropriate ICD shocks.     

The influence of sarcoidosis on quality of life

In the follow up of chronic illnesses like sarcoidosis, the psychological and social dimensions are getting more important in addition to the clinical parameters. It is the quality of life (QoL) that brings psychological and social dimensions in to the clinic. In this study we aimed to investigate the QoL of patients suffering from sarcoidosis. Study Short Form (SF-36) QoL scala was applied to 70 sarcoidosis patients who have been followed up in our center at least for a year. Among these patients 49 were females, 21 were males and the mean age was 43.27 +/- 11.97, mean disease duration was 3.55 +/- 1.80 (1-8 years). In our study, we found that variables belonging to sarcoidosis influence the QoL as well as the patient herself/himself. While age and female gender influenced almost all parameters of QoL, in cases with symptoms physical component had been influenced most. Diffusing capacity (DLCO) showed positive correlation with many of the parameters of QoL, whereas disease stage did negative. Patients' income and their to be treatment did not affect QoL. In conclusion, we suggest that QoL may help to follow up and decide on the treatment of sarcoidosis along with the other treatment criteria.     

Effect of Valsalva maneuver on surface electrocardiographic P-wave dispersion in paroxysmal atrial fibrillation

The purpose of this study was to investigate the effect of the Valsalva maneuver on P-wave durations and dispersion. After the Valsalva maneuver, we found that maximum P-wave duration increased, minimum P-wave duration decreased, and P-wave dispersion increased in controls, whereas the opposite was true for unselected patients with paroxysmal AF. It was concluded that patients with paroxysmal AF performing the Valsalva maneuver normalized their P-wave dispersion, thereby correcting the inhomogeneous intra-atrial conduction.     

Novel Βeta (β)-Thalassemia Mutation in Turkish Children

Beta (β)-thalassemia is the most frequently observed hereditary blood disorder in the world. It is characterized by deficiency of hemoglobin β-globin gene and is also a profoundly heterogeneous both at the molecular and clinical level. In the case of β-thalassemia, there is reduced (β⁺ type) or absent (β^o type) synthesis of the beta chains of hemoglobin. β-Thalassemia clinically occurs in three main forms: major, intermedia and minor according to requirement of transfusion. The objective of this study was to evaluate β-thalassemia mutations in 89 patients ranging from 2 months to 16 years of age, who enrolled to Medical School Research and Training Hospital, Gaziantep University. The direct DNA sequence analysis was performed for mutation scanning of β-globin gene. 89 children with β-Thalassemia including all types were analyzed, 16 different β-thalassemia mutations were detected. We have also identified a novel mutation (HBB.c.-80delT, rs397509430) in the promoter region (−30 TATA box) of β-globin gene, and clinical data of patient having novel mutation was given. The β-Thalassemia mutations were determined as β-Thalassemia major type in 42 patients (47.19 %), β-Thalassemia intermedia in 4 (4.49 %), β-Thalassemia minor in 43, (48.31 %) patients. The most frequent mutation was IVS I-110 G>A, followed by IVS I-1 G>A, IVS I-6 T>C, IVS II-1 G>A, respectively.     

Clinicopathological characteristics and treatment outcomes of adult patients with paratesticular rhabdomyosarcoma (PRMS): A 10-year single-centre experience

Introduction:

We report our experience with 8 consecutive adults treated for paratesticular rhabdomyosarcoma (RMS) at a single institution between 2000 and 2010.

Methods:

After primary surgical excision, 7 patients were classified into group I according to the Intergroup Rhabdomyosarcoma Study Group (IRSG) Postsurgical Grouping Classification, and 1 patient into group IIB. Retroperitoneal node dissection was not a required staging procedure. Adjuvant chemotherapy was administered to 7 of the 8 patients. No additional radiotherapy was administered.

Results:

The median age at diagnosis was 24 years (range: 18–60). Embryonal histology was the most common (75%) subtype. During follow-up, 3 patients experienced local relapse and 5 distant relapse. The median progression-free and overall survival times were 17.0 ± 9.9 months (range: 5–31) and 27.3 ± 1.3 months (range: 16–58), respectively.

Conclusion:

Paratesticular RMS is an uncommon malignancy in adults. We confirm that patients with localized paratesticular RMS may have different prognoses. Retroperitoneal lymphadenectomy can be avoided as a treatment for paratesticular RMS after radical inguinal orchiectomy.