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2.
Cardiol Young ; 28(11): 1361-1362, 2018 Nov.
Article in English | MEDLINE | ID: mdl-30152304

ABSTRACT

TandemHeart, an adult ventricular assist device, is also being used in children for mechanical circulatory support. In this case series, we describe our experience using TandemHeart ventricular assist device with a modified circuit to provide mechanical circulatory support in three neonates for multiple indications. TandemHeart ventricular assist device with a modified circuit can be used successfully to provide extracorporeal support to neonates with complex CHD.


Subject(s)
Extracorporeal Membrane Oxygenation/methods , Heart Defects, Congenital/therapy , Heart Ventricles/physiopathology , Heart-Assist Devices , Hemodynamics/physiology , Heart Defects, Congenital/physiopathology , Humans , Infant, Newborn , Male , Prosthesis Design
3.
J Pediatr Intensive Care ; 7(1): 59-61, 2018 Mar.
Article in English | MEDLINE | ID: mdl-31073470

ABSTRACT

A cardiac papillary fibroelastoma is a benign, pedunculated tumor with strand-like projections arising from the endocardium. While often discovered incidentally, these tumors can embolize resulting in major neurologic events. There is a dearth of pediatric literature describing the necessity and optimal timing of surgical intervention for this rare primary cardiac tumor after a cerebrovascular event. We report an interesting case and the subsequent management dilemma in a teenage patient presenting with acute embolic strokes secondary to an aortic valve papillary fibroelastoma. Despite its rarity, a high index of suspicion should be maintained for such tumors in previously healthy children presenting with acute stroke.

4.
J Pediatr Intensive Care ; 7(2): 97-101, 2018 Jun.
Article in English | MEDLINE | ID: mdl-31073479

ABSTRACT

Invasive community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) infections disproportionately affect children, but there are few pediatric reports of pericarditis and mediastinitis caused by CA-MRSA in previously healthy children. Here we report a severe case of CA-MRSA pericarditis with extension to the mediastinum and carotid sheath in a previously healthy 8-month-old infant who was successfully treated with surgical interventions and with a combination of daptomycin and vancomycin. The relatively indolent clinical course in this patient was notable given the significant extent of infection. This case highlights the potential virulence of CA-MRSA in previously healthy children and the importance of early diagnosis, prompt drainage, and appropriate antibiotic coverage.

5.
J Pediatr Intensive Care ; 6(3): 221-224, 2017 Sep.
Article in English | MEDLINE | ID: mdl-31073452

ABSTRACT

Takayasu arteritis (TA) is the third most common vasculitis in childhood, peaking in the second to third decades of life but affecting patients as young as 6 months of age. It often presents with nonspecific systemic symptoms, although at late stages, it may present with cardiac, renal, or focal neurologic sequelae of vascular compromise. In this case, we describe a 15-year-old patient who presented acutely with stroke. In the absence of more classic rheumatological symptoms and significant laboratory abnormalities on initial testing, the diagnosis of TA was only reached through extensive vascular imaging following consultation with multiple subspecialty teams. This case demonstrates the need to maintain a high index of suspicion for vasculitis in pediatric patients presenting with new onset stroke in the absence of known predisposing factors. Doing so may reduce the time to diagnosis, hasten treatment, and optimize outcomes.

7.
Acta Crystallogr D Biol Crystallogr ; 59(Pt 1): 73-6, 2003 Jan.
Article in English | MEDLINE | ID: mdl-12499542

ABSTRACT

The 2.0 A crystal structure has been determined for Escherichia coli uridine phosphorylase (UP), an essential enzyme in nucleotide biosynthesis that catalyzes the phosphorolytic cleavage of the C-N glycosidic bond of uridine to ribose-1-phosphate and uracil. The structure determination of two independent monomers in the asymmetric unit revealed the residue composition and atomic details of the apo configurations of each active site. The native hexameric UP enzyme was revealed by applying threefold crystallographic symmetry to the contents of the asymmetric unit. The 2.0 A model reveals a closer structural relationship to other nucleotide phosphorylase enzymes than was previously appreciated.


Subject(s)
Escherichia coli/enzymology , Uridine Phosphorylase/chemistry , Apoenzymes/chemistry , Binding Sites , Crystallography, X-Ray/methods , Models, Molecular , Protein Conformation
8.
J Struct Funct Genomics ; 2(3): 129-34, 2002.
Article in English | MEDLINE | ID: mdl-12836703

ABSTRACT

The New York Structural Genomics Research Consortium has targeted highly conserved but uncharacterized enzyme families for structure determination. As part of this effort, the 2.65-A crystal structure has been determined for Saccharomyces cerevisiae myo-inositol 1-phosphate synthase (MIP), an essential enzyme that catalyzes critical steps in inositol biosynthesis. The structure determination of four independent monomers in the asymmetric unit (240 kDa) reveals atomic details and residue composition for the partially closed NAD-containing active sites in apo-configuration. The structure further reveals extensive interactions involved in tetrameric assembly of the enzyme complex.


Subject(s)
Myo-Inositol-1-Phosphate Synthase/chemistry , Saccharomyces cerevisiae Proteins/chemistry , Saccharomyces cerevisiae/enzymology , Binding Sites , Crystallography, X-Ray , Inositol Phosphates/biosynthesis , Models, Molecular , Myo-Inositol-1-Phosphate Synthase/isolation & purification , Myo-Inositol-1-Phosphate Synthase/metabolism , NAD/chemistry , Protein Conformation , Saccharomyces cerevisiae Proteins/isolation & purification , Saccharomyces cerevisiae Proteins/metabolism , Structure-Activity Relationship
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