Predictors of acquired lipodystrophy in juvenile-onset dermatomyositis and a gradient of severity

We describe the clinical features of 28 patients with juvenile dermatomyositis (JDM) and 1 patient with adult-onset dermatomyositis (DM), all of whom developed lipodystrophy (LD) that could be categorized into 1 of 3 phenotypes, generalized, partial, or focal, based on the pattern of fat loss distribution. LD onset was often delayed, beginning a median of 4.6 years after diagnosis of DM. Calcinosis, muscle atrophy, joint contractures, and facial rash were DM disease features found to be associated with LD. Panniculitis was associated with focal lipoatrophy while the anti-p155 autoantibody, a newly described myositis-associated autoantibody, was more associated with generalized LD. Specific LD features such as acanthosis nigricans, hirsutism, fat redistribution, and steatosis/nonalcoholic steatohepatitis were frequent in patients with LD, in a gradient of frequency and severity among the 3 sub-phenotypes. Metabolic studies frequently revealed insulin resistance and hypertriglyceridemia in patients with generalized and partial LD. Regional fat loss from the thighs, with relative sparing of fat loss from the medial thighs, was more frequent in generalized than in partial LD and absent from DM patients without LD. Cytokine polymorphisms, the C3 nephritic factor, insulin receptor antibodies, and lamin mutations did not appear to play a pathogenic role in the development of LD in our patients. LD is an under-recognized sequela of JDM, and certain DM patients with a severe, prolonged clinical course and a high frequency of calcinosis appear to be at greater risk for the development of this complication. High-risk JDM patients should be screened for metabolic abnormalities, which are common in generalized and partial LD and result in much of the LD-associated morbidity. Further study is warranted to investigate the pathogenesis of acquired LD in patients with DM.     

Detection of inadvertent catheter movement into a pulmonary vein during radiofrequency catheter ablation by real-time impedance monitoring

Introduction: During radiofrequency ablation to encircle or isolate the pulmonary veins (PVs), applications of radiofrequency energy within a PV may result in stenosis. The aim of this study was to determine whether monitoring of real-time impedance facilitates detection of inadvertent catheter movement into a PV.
Methods and Results: In 30 consecutive patients (mean age 53 ± 11 years) who underwent a left atrial ablation procedure, the three-dimensional geometry of the left atrium, the PVs, and their ostia were reconstructed using an electroanatomic mapping system. The PV ostia were identified based on venography, changes in electrogram morphology, and manual and fluoroscopic feedback as the catheter was withdrawn from the PV into the left atrium. Real-time impedance was measured at the ostium, inside the PV at approximately 1 and 3 cm from the ostium, in the left atrial appendage, and at the posterior left atrial wall. There was an impedance gradient from the distal PV (127 ± 30 Ω) to the proximal PV (108 ± 15 Ω) to the ostium (98 ± 11 Ω) in each PV (P < 0.01). There was no significant impedance difference between the ostial and left atrial sites. During applications of radiofrequency energy, movement of the ablation catheter into a PV was accurately detected in 80% of the cases (20) when there was an abrupt increase of ≥4 Ω in real-time impedance.
Conclusion: There is a significant impedance gradient from the distal PV to the left atrium. Continuous monitoring of the real-time impedance facilitates detection of inadvertent catheter movement into a PV during applications of radiofrequency energy. (J Cardiovasc Electrophysiol, Vol. 15, pp. 1-5, June 2004)     

Pulmonary vein isolation as an end point for left atrial circumferential ablation of atrial fibrillation.

OBJECTIVES: We sought to determine whether elimination of pulmonary vein (PV) arrhythmogenicity is necessary for the efficacy of left atrial circumferential ablation (LACA) for atrial fibrillation (AF). BACKGROUND: The PVs often provide triggers or drivers of AF. It has been shown that LACA is more effective than PV isolation in eliminating paroxysmal AF. However, it is not clear whether complete PV isolation is necessary for the efficacy of LACA. METHODS: In 60 consecutive patients with paroxysmal (n = 39) or chronic (n = 21) AF (mean age 53 +/- 12 years), LACA to encircle the left- and right-sided PVs, with additional lines in the posterior left atrium and along the mitral isthmus, was performed under the guidance of an electroanatomic navigation system. The PVs were mapped with a decapolar ring catheter before and after LACA. If PV isolation was incomplete, no attempts at complete isolation were made. RESULTS: After LACA, there was incomplete electrical isolation of one or more PVs in 48 (80%) of the 60 patients. The prevalence of PV tachycardias was 82% before and 8% after LACA (p < 0.001). At 11 +/- 1 months of follow-up, 10 (83%) of the 12 patients with complete and 39 (81%) of 48 patients with incomplete PV isolation were free from recurrent AF without antiarrhythmic drug therapy (p = 1.0). A successful outcome was not related to the number of completely isolated PVs per patient (p = 0.6). CONCLUSIONS: Left atrial circumferential ablation modifies the arrhythmogenic substrate within the PVs. Complete electrical isolation of the PVs is not a requirement for a successful outcome after LACA.     

Mechanism of recurrence after radiofrequency catheter ablation of atrial fibrillation guided by complex fractionated atrial electrograms

Background A better understanding of the mechanisms of recurrent atrial fibrillation (AF) after radiofrequency ablation of complex, fractionated atrial electrograms (CFAEs) may be helpful for refining AF ablation strategies. Methods and results Electrogram-guided ablation (EGA) was repeated in 30 consecutive patients (mean age = 59 ± 8 years) for recurrent paroxysmal AF, 10 ± 4 months after the first ablation. During the first procedure, CFAEs were targeted without isolating all pulmonary veins (PVs). During repeat ablation, all PVs and the superior vena cava (SVC) were mapped with a circular catheter and the left atrium was mapped for CFAEs. EGA was performed until AF was rendered noninducible or all identified CFAEs were eliminated. During repeat ablation, ≥1 PV tachycardia was found in 83 PVs in 29 of the 30 patients (97%). Among these 83 PVs, 63 (76%) had not been completely isolated previously. During repeat ablation, drivers originating in a PV or PV antrum were identified only after infusion of isoproterenol (20 μg/min) in 12 patients (40%). At 9 ± 4 months of follow-up after the repeat ablation procedure, 21 of the 30 patients (70%) were free from recurrent AF and flutter without antiarrhythmic drugs. Conclusions Recurrence of AF after EGA is usually due to PV tachycardias. Therefore, it may be preferable to systematically map and isolate all PVs during the first procedure. High-dose isoproterenol may be helpful to identify AF drivers.     

Clinical features and outcomes of 76 patients with COVID-19-related multi-system inflammatory syndrome in children

Clinical Rheumatology - Multi-system inflammatory syndrome in children (MIS-C) is a less understood and a rare complication of coronavirus disease-2019 (COVID-19). Given the scarce data regarding...     

Efficacy and safety of ciprofloxacin oral suspension versus trimethoprim-sulfamethoxazole oral suspension for treatment of older women with acute urinary tract infection

OBJECTIVES: To compare the efficacy and safety of ciprofloxacin (CIP) oral suspension to trimethoprim/sulfamethoxazole (TMP/SMX) oral suspension among older women with acute urinary tract infections (UTIs). DESIGN: Prospective, randomized, open-label, multicenter study of older women (age 65 and older). SETTING: Community and nursing home. PARTICIPANTS: A total of 261 older women were evaluable for safety. Of these, 172 (86 community, 86 nursing home) were evaluable for clinical and bacteriological efficacy. INTERVENTION: Patients were randomized to a 10-day regimen of either CIP (250 mg/5 mL twice daily) or TMP/SMX (160/800 mg/20 mL twice daily). MEASUREMENTS: Clinical response 4 to 10 days posttherapy. RESULTS: For the efficacy-valid population, posttherapy clinical resolution was statistically superior following CIP (97%) versus TMP/SMX (85%) (95% CI=2.0-21.3; P= .009). Eradication of pretreatment bacterial isolates posttherapy was also higher following CIP (95%) versus TMP/SMX (84%) (95% CI=2.7-21.3; P= .019). For the intent-to-treat population, posttherapy clinical resolution was significantly higher in the CIP group (96%) than in the TMP/SMX group (87%) (95% CI=0.2-16.7; P= .025). Safety was assessed in the intent-to-treat population and the incidence of drug-related adverse events were significantly lower following CIP (17%) than following TMP/SMX (27%) (P= .047). Premature discontinuation due to these events was also less prevalent with CIP than with TMP/SMX (2% vs 11%, respectively) (P= .004). CONCLUSION: CIP suspension showed higher clinical success and bacteriological eradication rates than did TMP/SMX for both community-based and nursing home-residing older women with acute UTIs. Furthermore, CIP suspension was associated with significantly lower rates of adverse events and premature discontinuations compared with TMP/SMX suspension.     

Accessory pathway associated with an anomalous coronary sinus

Congenital coronary sinus anomalies are unusual and they rarely coexist with accessory atrioventricular pathways. These anomalies are generally asymptomatic, however they can cause difficulty in mapping. The association between accessory pathway and coronary sinus anomalies may suggest an embryologic link. In this case, we report a male patient with permanent form of reciprocating tachycardia coexistent with anomalous coronary sinus.