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Chicago Ridge, IL, United States

Everitt M.D.,University of Utah | Sleeper L.A.,New England Research Institutes, Inc. | Lu M.,New England Research Institutes, Inc. | Canter C.E.,Washington University in St. Louis | And 13 more authors.
Journal of the American College of Cardiology | Year: 2014

Objectives This study sought to determine the incidence and predictors of recovery of normal echocardiographic function among children with idiopathic dilated cardiomyopathy (DCM). Background Most children with idiopathic DCM have poor outcomes; however, some improve. Methods We studied children <18 years of age from the Pediatric Cardiomyopathy Registry who had both depressed left ventricular (LV) function (fractional shortening or ejection fraction z-score <-2) and LV dilation (end-diastolic dimension [LVEDD] z-score >2) at diagnosis and who had at least 1 follow-up echocardiogram 30 days to 2 years from the initial echocardiogram. We estimated the cumulative incidence and predictors of normalization. Results Among 868 children who met the inclusion criteria, 741 (85%) had both echocardiograms. At 2 years, 22% had recovered normal LV function and size; 51% had died or undergone heart transplantation (median, 3.2 months), and 27% had persistently abnormal echocardiograms. Younger age (hazard ratio [HR]: 0.92; 95% confidence interval [CI]: 0.88 to 0.97) and lower LVEDD z-score (HR: 0.78; 95% CI: 0.70 to 0.87) independently predicted normalization. Nine children (9%) with normal LV function and size within 2 years of diagnosis later underwent heart transplantation or died. Conclusions Despite marked LV dilation and depressed function initially, children with idiopathic DCM can recover normal LV size and function, particularly those younger and with less LV dilation at diagnosis. Investigations related to predictors of recovery, such as genetic associations, serum markers, and the impact of medical therapy or ventricular unloading with assist devices are important next steps. Longer follow-up after normalization is warranted as cardiac failure can recur. (Pediatric Cardiomyopathy Registry; NCT00005391).

Schumacher K.R.,University of Michigan | Gossett J.,Ann and Robert H. Lurie Childrens Hospital | Guleserian K.,Childrens Medical Center Dallas | Naftel D.C.,University of Alabama at Birmingham | And 7 more authors.
Journal of Heart and Lung Transplantation | Year: 2015

Background Post-Fontan protein-losing enteropathy (PLE) is associated with significant morbidity and mortality. Although heart transplantation (HTx) can be curative, PLE may increase the risk of morbidity before and after HTx. This study analyzed the influence of PLE influence on waiting list and post-HTx outcomes in a pediatric cohort. Methods Fontan patients listed for HTx and enrolled in the Pediatric Heart Transplant Study from 1999 to 2012 were stratified by a diagnosis of PLE, and the association of PLE with waiting list and post-HTx mortality, rejection, and infection was analyzed. Results Compared with non-PLE Fontan patients (n = 260), PLE patients listed for HTx (n = 96) were older (11.9 years vs 7.6 years; p = 0.003), had a larger body surface area (1.1 m2 vs 0.9 m2; p = 0.0001), had lower serum bilirubin (0.5 vs 0.9 mg/dl; p = 0.01), lower B-type natriuretic peptide (59 vs 227 pg/ml; p = 0.006), and were less likely to be on a ventilator (3% vs 13%; p = 0.006). PLE patients had lower waiting list mortality than non-PLE Fontan patients (p < 0.0001). There were no intergroup differences for post-HTx survival or times to the first infection or rejection. PLE was not independently associated with increased post-HTx mortality at any time point. Conclusions In this multicenter cohort, the diagnosis of PLE alone was not associated with increased waiting list mortality or post-HTx morbidity or mortality. Given the limitations of our data, this analysis suggests that PLE patients in the pediatric age group have outcomes similar to their non-PLE counterparts. Additional multicenter studies of PLE patients with targeted collection of PLE-specific information will be necessary to fully delineate the risks conferred by PLE for HTx. © 2015 International Society for Heart and Lung Transplantation.

Phillips G.,Northwestern University | Kuhns L.M.,Ann and Robert H. Lurie Childrens Hospital | Kuhns L.M.,Northwestern University | Garofalo R.,Ann and Robert H. Lurie Childrens Hospital | And 2 more authors.
Journal of Epidemiology and Community Health | Year: 2014

Background: To generate unbiased estimates for data collected using respondent-driven sampling (RDS), a number of assumptions need to be met: individuals recruit randomly from their social network and people can accurately report their eligible network size. However, research has shown that these assumptions are often violated. Methods: This study used baseline data from Crew 450, a longitudinal study of young men who have sex with men in Chicago who were recruited via a modified form of RDS and its network substudy, in which a subset of 175 participants reported details on the composition and characteristics of their social network at either 1 or 2 years postbaseline. Results: Nearly two-thirds of participants reported giving coupons to at least one alter (64%), and 56.3% believed their alter(s) used the coupons. Frequency of communication, closeness and type of relationship played a major role in determining coupon distribution. Participants whose alters used coupons were significantly less likely to describe the strength of their relationship as 'not at all close' (OR=0.08; 95% CI 0.02 to 0.36) compared with 'very close' and to communicate weekly (OR=0.20; 95% CI 0.08 to 0.49) or 1-6 times in the past 6 months (OR=0.18; 95% CI 0.06 to 0.59). Conclusions: Contrary to RDS assumptions, we found that relationship characteristics played a significant role when individuals decided to whom they would give coupons.

Shearer W.T.,Baylor College of Medicine | Fleisher T.A.,U.S. National Institutes of Health | Buckley R.H.,Duke University | Ballas Z.,University of Iowa | And 23 more authors.
Journal of Allergy and Clinical Immunology | Year: 2014

The present uncertainty of which live viral or bacterial vaccines can be given to immunodeficient patients and the growing neglect of societal adherence to routine immunizations has prompted the Medical Advisory Committee of the Immune Deficiency Foundation to issue recommendations based on published literature and the collective experience of the committee members. These recommendations address the concern for immunodeficient patients acquiring infections from healthy subjects who have not been immunized or who are shedding live vaccine-derived viral or bacterial organisms. Such transmission of infectious agents can occur within the hospital, clinic, or home or at any public gathering. Collectively, we define this type of transmission as close-contact spread of infectious disease that is particularly relevant in patients with impaired immunity who might have an infection when exposed to subjects carrying vaccine-preventable infectious diseases or who have recently received a live vaccine. Immunodeficient patients who have received therapeutic hematopoietic stem transplantation are also at risk during the time when immune reconstitution is incomplete or while they are receiving immunosuppressive agents to prevent or treat graft-versus-host disease. This review recommends the general education of what is known about vaccine-preventable or vaccine-derived diseases being spread to immunodeficient patients at risk for close-contact spread of infection and describes the relative risks for a child with severe immunodeficiency. The review also recommends a balance between the need to protect vulnerable subjects and their social needs to integrate into society, attend school, and benefit from peer education. © 2013 American Academy of Allergy, Asthma & Immunology.

Webb C.L.,University of Michigan Congenital Heart Center | Waugh C.L.,Ann and Robert H. Lurie Childrens Hospital | Grigsby J.,University of Colorado at Denver | Busenbark D.,University of Colorado at Denver | And 3 more authors.
Journal of the American Society of Echocardiography | Year: 2013

Background Previous single-center studies have shown that telemedicine improves care in newborns with suspected heart disease. The aim of this study was to test the hypothesis that telemedicine would shorten time to diagnosis, prevent unnecessary transports, reduce length of stay, and decrease exposure to invasive treatments. Methods Nine pediatric cardiology centers entered data prospectively on patients aged <6 weeks, matched by gestational age, weight, and diagnosis. Subjects born at hospitals with and without access to telemedicine constituted the study group and control groups, respectively. Data from patients with mild or no heart disease were analyzed. Results Data were obtained for 337 matched pairs with mild or no heart disease. Transport to a tertiary care center (4% [n = 15] vs 10% [n = 32], P =.01), mean time to diagnosis (100 vs 147 min, P <.001), mean length of stay (1.0 vs 26 days, P =.005) and length of intensive care unit stay (0.96 vs 2.5 days, P =.024) were significantly less in the telemedicine group. Telemedicine patients were significantly farther from tertiary care hospitals than control subjects. The use of inotropic support and indomethacin was significantly less in the telemedicine group. By multivariate analysis, telemedicine patients were less likely to be transported (odds ratio, 0.44; 95% confidence interval, 0.23-0.83) and less likely to be placed on inotropic support (odds ratio, 0.16; 95% confidence interval, 0.10-0.28). Conclusions Telemedicine shortened the time to diagnosis and significantly decreased the need for transport of infants with mild or no heart disease. The length of hospitalization and intensive care stay and use of indomethacin and inotropic support were less in telemedicine patients.

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