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Kutty S.,University of Nebraska Medical Center | Delaney J.W.,University of Nebraska Medical Center | Latson L.A.,Joe DiMaggio Childrens Hospital | Danford D.A.,University of Nebraska Medical Center
Journal of the American Society of Echocardiography | Year: 2013

The rapid proliferation of catheter-mediated treatments for congenital heart defects has brought with it a critical need for cooperation and communication among the numerous physicians supporting these new and complex procedures. New interdependencies between physicians in specialties including cardiac imaging, interventional cardiology, pediatric cardiology, anesthesia, cardiothoracic surgery, and radiology have become apparent, as centers have strived to develop the best systems to foster success. Best practices for congenital heart disease interventions mandate confident and timely input from an individual with excellent adjunctive imaging skills and a thorough understanding of the devices and procedures being used. The imager and interventionalist must share an understanding of what each offers for the procedure, use a common terminology and spatial orientation system, and convey concise and accurate information about what is needed, what is seen, and what cannot be seen. The goal of this article is to review how the cardiovascular imaging specialists and interventionalists can work together effectively to plan and execute catheter interventions for congenital heart disease. Copyright 2013 by the American Society of Echocardiography. Source

White J.G.,University of Minnesota | Pakzad K.,Pathology Consultants of South Broward | Meister L.,Joe DiMaggio Childrens Hospital
Platelets | Year: 2013

The present report describes a fourth patient with platelet pathological features identical to those found in the first three cases with the York platelet syndrome (YPS), as well as other findings that suggest he may be a variant. His platelets contain the same giant opaque and target organelles found earlier, as well as enlarged organelles with a gray appearing matrix. It is possible that the giant structures have the same source, but are at different stages of development. The fourth patient has platelet pathology suggestive of other thrombocyte disorders. He has many large platelets and normal sized thrombocytes nearly devoid of alpha granules. As a result, he was originally thought to have the gray platelet syndrome. He also has significant numbers of platelets attached to platelets and platelets in platelets as seen in patients with the X-linked GATA-1 mutation. Some of the fourth YPS patient's platelets contained massive alpha granules suggesting the possibility of the Paris Trousseau Jacobson Syndrome. Yet, none of these other platelet disorders had giant dense organelles like those found in YPS thrombocytes. As a result, it is reasonable to include this child with the other three, and diagnose him as a patient with the YPS. © 2013 Informa UK Ltd All rights reserved: reproduction in whole or part not permitted. © 2013 Informa UK Ltd. Source

Schanler R.J.,New York University | Schanler R.J.,Yeshiva University | Fraley J.K.,Baylor College of Medicine | Lau C.,Baylor College of Medicine | And 3 more authors.
Journal of Perinatology | Year: 2011

Objective:As expressed mother's milk (MM) is known to be colonized by microbial species, it is occasionally considered as a source of infection in premature infants, prompting some clinicians to obtain milk bacterial culture results before infant feeding. To determine whether serial microbial cultures of MM predict infection in premature infants. Study Design:Milk microbial flora was determined by plate counts from aliquots of MM obtained from 161 mothers of infants born <30 weeks gestation (n=209). Pathogens isolated from the same infant were tabulated. Result:Milk samples (n=813) yielded 1963 isolates. There were no relationships between microbial counts and maternal age, ethnicity, education, skin-to-skin contact and infant infection. In 64 infants, milk and pathological isolates had presumptively the same Gram-positive organism, yet the odds of infection before or after exposure to milk containing that Gram-positive organism were not significant (1.18; 95% confidence interval=0.51, 2.76). In eight infants, milk and pathological isolates had presumptively the same Gram-negative organism, which appeared sporadically in milk, either before or after isolation in the infant. Conclusion:Results of initial milk cultures do not predict subsequent culture results. Random milk cultures, even if obtained at any time during hospitalization, are not predictive of infection in premature infants. The sporadic nature of the appearance of certain isolates, however, suggests common exposure of both mother and infant. Routine milk cultures do not provide sufficient data to be useful in clinical management. © 2011 Nature America, Inc. All rights reserved. Source

Qureshi M.Y.,University of Miami | Ratnasamy C.,Arkansas Childrens Hospital | Sokoloski M.,University of Miami | Young M.-L.,Joe DiMaggio Childrens Hospital
PACE - Pacing and Clinical Electrophysiology | Year: 2013

Background Cryoablation is an alternative to radiofrequency ablation in treating atrioventricular nodal reentrant tachycardia (AVNRT). However, its long-term effectiveness is in question when compared to radiofrequency ablation. We reviewed the results of cryoablation in children with AVNRT at our institute. Methods We performed a retrospective single-center chart review of consecutive patients ≤18 years of age with AVNRT who underwent cryoablation between January 2007 and August 2009. During cryoablation, a 6-mm-tip cryocatheter was used with temperature set to -80°C. Test lesions were performed at the presumed slow pathway location based on combined anatomic and electrophysiologic approach. If successful, ablation was then continued with triple freeze-thaw cycles (FTC) of 4 minutes each. Results A total of 53 patients (age range: 6.1-18.4 years, mean: 13.6 years, median: 13.2 years) underwent slow pathway modification with cryoablation. Acute success was achieved in 51 (96.2%) cases. Transient atrioventricular block was seen in 19 cases. The block occurred during FTC in eight patients (15%). The number of FTC was three in 47 (92.2%) patients. Less than three FTC were given in two patients due to transient heart block and four FTC were given in two patients with suspected catheter movement. Procedure duration was 177 ± 56 minutes; fluoroscopic time was 14 ± 11 minutes. Mean follow-up was 30.7 ± 10 (range 12-52, median 31) months. Recurrence of supraventricular tachycardia was seen in only one (1.96%) patient. Conclusions Triple FTC cryoablation lesions resulted in a low recurrence rate comparable to RF ablation in treating AVNRT without increased complications. © 2012 Wiley Periodicals, Inc. Source

Kino T.,U.S. National Institutes of Health | Pavlatou M.G.,U.S. National Institutes of Health | Moraitis A.G.,U.S. National Institutes of Health | Nemery R.L.,Joe DiMaggio Childrens Hospital | And 2 more authors.
Journal of Clinical Endocrinology and Metabolism | Year: 2012

Context: Nuclear hormone receptors exert their transcriptional effects through shared cofactor molecules; thus, defects in such intermediate proteins may be associated with multiple hormone resistance. Microdeletion of small chromosomal segments results in hereditary or sporadic diseases by affecting expression of residing genes. Objectives: We describe a 7-yr-old boy with partial resistance to glucocorticoids, thyroid hormones, and possibly androgens. He was diagnosed as being in the autism spectrum disorder and had developmental delay and several facial morphological manifestations. We explored genes responsible for multiple hormone resistance of this case. Results: We found in this patient an approximately 1.1-Mb heterozygous 16p11.2 microdeletion, which included an approximately 500-kb unique deletion along with the common, previously reported approximately 600-kb 16p11.2 microdeletion. The small interfering RNA-based screening revealed that knockdown of ZNF764, which is located in the deleted segment unique to our case, significantly reduced glucocorticoid-, androgen-, and thyroid hormone-induced transcriptional activity of their responsive genes in HeLa cells, whereas its overexpression enhanced their transcriptional activity. The activities of the estrogen and progesterone receptors, cAMP response element-binding protein, and p53 were not affected in these cells. ZNF764 (zinc finger protein 764) expression was reduced in the patient's peripheral blood mononuclear cells, whereas exogenously supplemented ZNF764 recovered responsiveness to glucocorticoids in the patient's Epstein-Barr virus-transformed lymphocytes. The effect of ZNF764 on the glucocorticoid receptor transcriptional activity was mediated through cooperation with a general nuclear hormone receptor coactivator, transcriptional intermediary factor 1. Conclusions: ZNF764 haploinsufficiency caused by microdeletion maybe responsible for the partial multiple hormone resistance observed in our patient. ZNF764 appears to be involved in glucocorticoid, androgen, and thyroid hormone action. Copyright © 2012 by The Endocrine Society. Source

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