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Meyer R.E.,State Center for Health StatisticsRaleigh | Liu G.,Albany State University | Gilboa S.M.,National Center on Birth Defects and Developmental DisabilitiesCenters for Disease Control and PreventionAtlanta | Ethen M.K.,Texas | And 6 more authors.
American Journal of Medical Genetics, Part A

Trisomy 13 (T13) and trisomy 18 (T18) are among the most prevalent autosomal trisomies. Both are associated with a very high risk of mortality. Numerous instances, however, of long-term survival of children with T13 or T18 have prompted some clinicians to pursue aggressive treatment instead of the traditional approach of palliative care. The purpose of this study is to assess current mortality data for these conditions. This multi-state, population-based study examined data obtained from birth defect surveillance programs in nine states on live-born infants delivered during 1999-2007 with T13 or T18. Information on children's vital status and selected maternal and infant risk factors were obtained using matched birth and death certificates and other data sources. The Kaplan-Meier method and Cox proportional hazards models were used to estimate age-specific survival probabilities and predictors of survival up to age five. There were 693 children with T13 and 1,113 children with T18 identified from the participating states. Among children with T13, 5-year survival was 9.7%; among children with T18, it was 12.3%. For both trisomies, gestational age was the strongest predictor of mortality. Females and children of non-Hispanic black mothers had the lowest mortality. Omphalocele and congenital heart defects were associated with an increased risk of death for children with T18 but not T13. This study found survival among children with T13 and T18 to be somewhat higher than those previously reported in the literature, consistent with recent studies reporting improved survival following more aggressive medical intervention for these children. © 2015 Wiley Periodicals, Inc. Source

Abbas A.E.,Cardiovascular Systems | Franey L.M.,Cardiovascular Systems | Lester S.,Arizona | Raff G.,Cardiovascular Systems | And 4 more authors.

In patients with aortic stenosis (AS) and eccentric transaortic flow, greater pressure loss occurs as the jet collides with the aortic wall together with delayed and diminished pressure recovery. This leads to the elevated transaortic valve pressure gradients noted on both Doppler and cardiac catheterization. Such situations may present a diagnostic dilemma where traditional measures of stenosis severity indicate severe AS, while imaging modalities of the aortic valve geometric aortic valve area (GOA) suggest less than severe stenosis. In this study, we present a series of cases exemplifying this clinical dilemma and demonstrate how color M-mode, 2D and 3D transthoracic (TTE) and transesophageal (TEE) echocardiography, cardiac computed tomography angiography (CTA), and magnetic resonance imaging (MRI), may be used to resolve such discrepancies. © 2014, Wiley Periodicals, Inc. Source

Green D.M.,Tennessee | Breslow N.E.,BiostatisticsUniversity of WashingtonSeattle | D'Angio G.J.,University of Pennsylvania | Malogolowkin M.H.,Midwest Childrens Cancer Center at Childrens Hospital of WisconsinMilwaukee | And 9 more authors.
Pediatric Blood and Cancer

Background: Intra-operative tumor spill increases the risk of local recurrence of Wilms tumor, and adversely impacts relapse-free (RFS) and overall survival (OS) rates. Methods: Surgical checklists, operative notes, institutional pathology reports, central pathology review and flow sheets of 602 patients registered between August 1986 and September 1994 on National Wilms Tumor Study-4 as randomized, followed or switched and coded as Final Stage II, favorable histology (FH) were reviewed. RFS and OS were estimated using the Kaplan-Meier method. Hazard ratios (HRs) were estimated using the Cox model and tested for statistical significance by the log-rank test. Results: Four hundred ninety-nine patients were found after review to have Stage II, FH Wilms tumor. The 8-year RFS percentages were 85.0% (95% confidence interval (CI): 81.1%, 88.1%) for those with no spill compared to 75.7% (65.8%, 83.2%) for those with spill. The 8-year OS percentages were 95.6% (93.1%, 97.3%) for those with no spill compared to 90.3% (82.2%, 94.9%) for those with spill. The HR for relapse among those with spill was 1.55 ((95%CI: 0.97,2.51), P=0.067) and the HR for death was 1.94 ((0.92,4.09), P=0.077). Conclusions: RFS and OS were lower for patients who had intra-operative tumor spill. The majority of NWTS Stage II, FH patients with intra-operative tumor spill have an overall excellent outcome when treated with two drug chemotherapy (vincristine and actinomycin D) and no abdominal irradiation. © 2013 Wiley Periodicals, Inc. Source

Agarwal R.,Indiana University | Agarwal R.,Richard udebush Va Medical Center | Michael Bouldin J.,Indiana University | Light R.P.,Indiana University | Garg A.,Arizona
Clinical Journal of the American Society of Nephrology

Background and objectives Hypervolemia is an important and modifiable cause of hypertension. Hypertension improves with probing dry weight, but its effect on echocardiographic measures of volume is unknown. Design, setting, participants, & measurements Shortly after dialysis, echocardiograms were obtained at baseline and longitudinally every 4 weeks on two occasions. Among 100 patients in the additional ultrafiltration group, 198 echocardiograms were performed; among 50 patients in the control group, 104 echocardiograms were performed. Results Baseline inferior vena cava (IVC)insp diameter was approximately 5.1 mm/m2; with ultrafiltration, change in IVCinsp diameter was -0.95 mm/m2 more compared with the control group at 4 weeks and -1.18 mm/m2 more compared with the control group at 8 weeks. From baseline IVCexp diameter of approximately 8.2 mm/m2, ultrafiltration-induced change at 4 weeks was -1.06 mm/m2 more and at 8 weeks was -1.07 mm/m2 more (P = 0.044). From a baseline left atrial diameter of 2.1 cm/m2, ultrafiltration-induced change at 4 weeks was -0.14 cm/m2 more and at 8 weeks was -0.15 cm/m2 more. At baseline, there was no relationship between interdialytic ambulatory BP and echocardiographic parameters of volume. The reduction in interdialytic ambulatory BP was also independent of change in the echocardiographic volume parameters. Conclusions The inferior vena cava and left atrial diameters are echocardiographic parameters that are responsive to probing dry weight; thus, they reflect excess volume. However, echocardiographic volume parameters are poor determinants of interdialytic BP, and their change does not predict the BP response to probing dry weight. © 2011 by the American Society of Nephrology. Source

Objectives/Hypothesis: To report two patients with a history of microvascular decompression (MVD) for hemifacial spasm who presented with Teflon granulomas (TG) mimicking cerebellopontine angle (CPA) tumors and to perform a systematic review of the English-language literature. Study Design: Case series at a single tertiary academic referral center and systematic review. Methods: Retrospective chart review with analysis of clinical, radiological, and histopathological findings. Systematic review using PubMed, Embase, MEDLINE, and Web of Science databases. Results: Two patients with large skull base TGs mimicking CPA tumors clinically and radiographically were managed at the authors' institution. The first presented 4 years after MVD with asymmetrical sensorineural hearing loss, multiple progressive cranial neuropathies, and brainstem edema due to a growing TG. Reoperation with resection of the granuloma confirmed a foreign-body reaction consisting of multinucleated giant cells containing intracytoplasmic Teflon particles. The second patient presented 11 years after MVD with asymmetrical sensorineural hearing loss and recurrent hemifacial spasm. No growth was noted over 2 years, and the patient has been managed expectantly. Only one prior case of TG after MVD for hemifacial spasm has been reported in the English literature. Conclusions: TG is a rare complication of MVD for hemifacial spasm. The diagnosis should be suspected in patients presenting with a new-onset enhancing mass of the CPA after MVD, even when performed decades earlier. A thorough clinical and surgical history is critical toward establishing an accurate diagnosis to guide management and prevent unnecessary morbidity. Surgical intervention is not required unless progressive neurologic complications ensue. © 2016 The American Laryngological, Rhinological and Otological Society, Inc. Source

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