Cryoablation of a Nodoventricular Mahaim Fiber

An 11-year old female presented with paroxysmal tachycardia and was diagnosed with a Mahaim fiber during electrophysiologic study. A preexcited tachycardia and the typical variety of AV nodal reentry tachycardia were induced at different times. During preexcited tachycardia, the His bundle electrogram followed the ventricular electrogram, and, introduction of atrial premature beats at different coupling intervals, advanced the peri-AV nodal atrial tissue, with no change in the ventricular cycle length, leading to a diagnosis of an antidromic tachycardia due to a nodoventricular fiber. Cryoablation at a mid-septal location under three-dimensional guidance successfully eliminated both tachycardias without detrimental effects to the AV node.     

Primary spinal cord oligodendroglioma: case report and review of the literature

Objects The objectives were to present a case of pediatric spinal oligodendroglioma and review the existing literature written in English on the subject of human spinal oligodendrogliomas. A comparison of the clinical, radiologic, and pathologic characteristics, as they relate to those already described in similar cases, was also attempted.Methods Thorough evaluation of the patients clinical course was undertaken. Presenting symptoms and signs are reported. The perioperative radiologic features of the case are presented and the intraoperative details as well as the pathologoanatomic findings and follow-up history are provided. We subsequently performed a thorough search in the literature focusing on the number, characteristics, treatment modalities, and prognosis of patients with spinal cord oligodendrogliomas.Conclusions Spinal oligodendrogliomas are a distinctly rare type of nervous system tumor, especially in the pediatric population. An international registry addressing all of their clinical and pathobiological characteristics would be of great benefit to patients harboring these rare tumors.     

White coat effect in treated versus untreated hypertensive individuals: a case-control study using ambulatory and home blood pressure monitoring

BACKGROUND: Some studies have shown a significant white coat effect (WCE) (i.e., difference between clinic blood pressure [CBP] and awake ambulatory blood pressure [ABP]) to be present not only in untreated but also in treated hypertensive individuals. This study aims to assess 1) the prevalence and the magnitude of the WCE in treated versus untreated hypertensive persons, and 2) the usefulness of home blood pressure (HBP) versus ABP in the detection of this phenomenon. METHODS: A case-control study was conducted in 138 treated hypertensive patients and same number of sex- and age-matched untreated hypertensive subjects who had measurements of CBP (at least three visits), HBP, and ABP. Subjects with a WCE of >20/10 mm Hg (systolic/diastolic) were classified as clinic reactors. RESULTS: There was a trend for a larger WCE assessed by ABP monitoring in the untreated group (mean difference in systolic WCE, 1.8 +/- 22.2 mm Hg, 95% CI -2.0 to 5.5; diastolic 1.8 +/- 11.9 mm Hg, 95% CI -0.2 to 3.8) and for more untreated clinic reactors (27% untreated v 20% treated, odds ratio 1.5, 95% CI 0.9 to 2.7). The sensitivity, specificity, and positive and negative predictive values of HBP to detect clinic reactors correctly were 56%/62% (treated/untreated), 87%/84%, 52%/59%, and 89%/86%, respectively, with moderate agreement between HBP and ABP (kappa 0.42/0.46). CONCLUSIONS: In treated hypertensive patients, WCE seems to be reduced compared with that in untreated hypertensive persons but is not eliminated. In both untreated and treated hypertensive individuals HBP monitoring appears to be useful in the detection of the WCE, but it may not be appropriate as an alternative to the ABP method.     

Bietti's crystalline corneoretinal dystrophy: a cross-sectional study

OBJECTIVE: To evaluate the prevalence of Bietti's corneoretinal dystrophy (BCD) in a reference population of retinitis pigmentosa (RP), and to document the disease course clinically and angiographically by a cross-sectional approach. METHODS: Two hundred seven consecutive RP patients were ascertained within a 3-year period. All patients had a complete eye examination, including fluorescein and indocyanine green angiography (FA, ICG). Blood samples were collected for electron microscopy evaluation of circulating lymphocytes of BCD patients. RESULTS: Six patients were diagnosed with BCD. Prevalence was 3% of nonsyndromic RP cases. Limbal corneal and intraretinal crystals were found in all cases. In the early phase of BCD, ICG revealed focal lobular areas of choriocapillary atrophy at the equator, with concomitant retinal pigment epithelium (RPE) changes at the posterior pole on FA. These lesions gradually progressed both anteriorly and posteriorly, sparing an incomplete peripapillary ring and macular island until late in the disease. Progressive sclerosis of ciliary and choroidal arteries was noted upon ICG. The presence of lysosomal crystals was documented in a subpopulation of circulating lymphocytes that seemed to increase with age. CONCLUSION: The present study is the first to assess BCD prevalence in a population of retinitis pigmentosa patients, to describe clinically and angiographically its evolution from first symptom to legal blindness, and to localize the first fundus alterations not only at the posterior pole but also at the equator.