Sympathetic paraganglioma in a patient with unrepaired tetralogy of Fallot: a case report and review of the literature.

Context: Paragangliomas are a type of neuroendocrine tumor that has been reported to be present in patients with cyanotic congenital heart disease. This report documents the first case of a patient with successful resection of a sympathetic paraganglioma in the setting of unrepaired tetralogy of Fallot, the most common cause of cyanotic heart disease, with pulmonary atresia.

Objective: We present a 33-yr-old woman with hypertensive crises from a paraganglioma who presented for surgical resection.

Review of ambulatory pulmonary artery catheterization in the management of advanced heart failure.

The use of pulmonary artery catheterization (PAC) has declined secondary to associated complications and lack of demonstrable efficacy in the inpatient setting. Few studies have been published on the use of PAC in nonacute heart failure (HF) patients. The purpose of this study was to review the use of PAC in guiding advanced therapy in nonacute ambulatory HF patients.

Hey2 functions in parallel with Hes1 and Hes5 for mammalian auditory sensory organ development.

Background: During mouse development, the precursor cells that give rise to the auditory sensory organ, the organ of Corti, are specified prior to embryonic day 14.5 (E14.5).

Wnt5a functions in planar cell polarity regulation in mice.

Planar cell polarity (PCP) refers to the polarization of cells within the plane of a cell sheet. A distinctive epithelial PCP in vertebrates is the uniform orientation of stereociliary bundles of the sensory hair cells in the mammalian cochlea. In addition to establishing epithelial PCP, planar polarization is also required for convergent extension (CE); a polarized cellular movement that occurs during neural tube closure and cochlear extension.

Dishevelled genes mediate a conserved mammalian PCP pathway to regulate convergent extension during neurulation.

The planar cell polarity (PCP) pathway is conserved throughout evolution, but it mediates distinct developmental processes. In Drosophila, members of the PCP pathway localize in a polarized fashion to specify the cellular polarity within the plane of the epithelium, perpendicular to the apicobasal axis of the cell. In Xenopus and zebrafish, several homologs of the components of the fly PCP pathway control convergent extension.

Regulation of polarized extension and planar cell polarity in the cochlea by the vertebrate PCP pathway.

The mammalian auditory sensory organ, the organ of Corti, consists of sensory hair cells with uniformly oriented stereocilia on the apical surfaces and has a distinct planar cell polarity (PCP) parallel to the sensory epithelium. It is not certain how this polarity is achieved during differentiation. Here we show that the organ of Corti is formed from a thicker and shorter postmitotic primordium through unidirectional extension, characteristic of cellular intercalation known as convergent extension.