The Subclinical Cardiomyopathy of Friedreich's Ataxia in a Pediatric Population. (Record no. 2766)

MARC details
000 -LEADER
fixed length control field 03495nam a22005537a 4500
008 - FIXED-LENGTH DATA ELEMENTS--GENERAL INFORMATION
fixed length control field 171017s20182018 xxu||||| |||| 00| 0 eng d
022 ## - INTERNATIONAL STANDARD SERIAL NUMBER
International Standard Serial Number 1071-9164
040 ## - CATALOGING SOURCE
Original cataloging agency Ovid MEDLINE(R)
099 ## - LOCAL FREE-TEXT CALL NUMBER (OCLC)
PMID 28986271
245 ## - TITLE STATEMENT
Title The Subclinical Cardiomyopathy of Friedreich's Ataxia in a Pediatric Population.
251 ## - Source
Source Journal of Cardiac Failure. 24(10):672-679, 2018 10.
252 ## - Abbreviated Source
Abbreviated source J Card Fail. 24(10):672-679, 2018 10.
253 ## - Journal Name
Journal name Journal of cardiac failure
260 ## - PUBLICATION, DISTRIBUTION, ETC.
Year 2018
260 ## - PUBLICATION, DISTRIBUTION, ETC.
Manufacturer FY2019
266 ## - Date added to catalog
Date added to catalog 2017-10-17
269 ## - Original dates
Original fiscal year FY2018
501 ## - WITH NOTE
Local holdings Available online from MWHC library: 1995 - present
520 ## - SUMMARY, ETC.
Abstract BACKGROUND: Identification of a subclinical cardiomyopathy in a pediatric patients with Friedreich's ataxia (FA) has not been well-described.
520 ## - SUMMARY, ETC.
Abstract CONCLUSIONS: A subclinical hypertrophic cardiomyopathy is common in pediatric FA patients and CH is associated with both diastolic and systolic dysfunction. Copyright (c) 2017. Published by Elsevier Inc.
520 ## - SUMMARY, ETC.
Abstract METHODS: We performed echocardiography (echo), cardiac magnetic resonance imaging (cMRI) and neurologic assessment in a cross-sectional analysis of 48 genetically-confirmed FA subjects aged 9-17 years with moderate neurologic impairment but without a cardiovascular history. Echo and cMRI-determined left ventricular mass were indexed to height in g/m2.7 (LVMI). LV remodeling was categorized as concentric remodeling (CR), concentric hypertrophy (CH) or eccentric hypertrophy based upon echo-determined relative LV wall thickness.
520 ## - SUMMARY, ETC.
Abstract RESULTS: Echo LVMI exceeded age-based normal values in 85% of subjects and cMRI-determined LVMI correlated with depression of both diastolic and systolic tissue Doppler velocity (E': r= -0.65, p<0.001, S': r=-0.46, p<0.001) as well as increased early diastolic Doppler flow velocity/tissue velocity ratio (r=0.55, p<0.001), a marker of elevated LV filling pressure. Similar associations were found with echo LV mass. Depressed LV relaxation and increased LV stiffness were observed in 88% and 71% of subjects despite a normal LV ejection fraction in almost all cases (mean = 60 + 7%). CR and CH were present in 40% and 44% of the study group though significant depressions of E' and S' were observed only in subjects with CH (p<0.005).
546 ## - LANGUAGE NOTE
Language note English
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element *Cardiomyopathy, Hypertrophic/et [Etiology]
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element *Echocardiography, Doppler/mt [Methods]
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element *Friedreich Ataxia/co [Complications]
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element *Heart Ventricles/dg [Diagnostic Imaging]
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element *Magnetic Resonance Imaging, Cine/mt [Methods]
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element *Ventricular Function, Left/ph [Physiology]
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Adolescent
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Cardiomyopathy, Hypertrophic/di [Diagnosis]
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Cardiomyopathy, Hypertrophic/pp [Physiopathology]
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Child
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Cross-Sectional Studies
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Disease Progression
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Double-Blind Method
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Female
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Follow-Up Studies
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Heart Ventricles/pp [Physiopathology]
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Humans
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Male
650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM
Topical term or geographic name entry element Ventricular Remodeling
651 ## - SUBJECT ADDED ENTRY--GEOGRAPHIC NAME
Institution MedStar Health Research Institute
657 ## - INDEX TERM--FUNCTION
Medline publication type Journal Article
700 ## - ADDED ENTRY--PERSONAL NAME
Local Authors Horton, Kenneth D
790 ## - Authors
All authors Di Prospero NA, Drinkard BE, Ernst I, Hasbani K, Horton KD, Plehn JF
856 ## - ELECTRONIC LOCATION AND ACCESS
DOI <a href="https://dx.doi.org/10.1016/j.cardfail.2017.09.012">https://dx.doi.org/10.1016/j.cardfail.2017.09.012</a>
Public note https://dx.doi.org/10.1016/j.cardfail.2017.09.012
942 ## - ADDED ENTRY ELEMENTS (KOHA)
Koha item type Journal Article
Item type description Article
Holdings
Withdrawn status Lost status Damaged status Not for loan Collection Home library Current library Date acquired Total Checkouts Full call number Barcode Date last seen Price effective from Koha item type
          MedStar Authors Catalog MedStar Authors Catalog 10/17/2017   28986271 28986271 10/17/2017 10/17/2017 Journal Article

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