Conditional deletion of AP-2ß in mouse cranial neural crest results in anterior segment dysgenesis and early-onset glaucoma.

Anterior segment dysgenesis (ASD) encompasses a group of developmental disorders in which a closed angle phenotype in the anterior chamber of the eye can occur and 50% of patients develop glaucoma. Many ASDs are thought to involve an inappropriate patterning and migration of the periocular mesenchyme (POM), which is derived from cranial neural crest cells (NCCs) and mesoderm. Although, the mechanism of this disruption is not well understood, a number of transcriptional regulatory molecules have previously been implicated in ASDs. Here, we investigate the function of the transcription factor AP-2ß, encoded by Tfap2b, which is expressed in NCCs and their derivatives. Wnt1-Cre-mediated conditional deletion of Tfap2b in NCCs resulted in post-natal ocular defects typified by opacity. Histological data revealed that the conditional AP-2ß NCC knockout (KO) mutants exhibited dysgenesis of multiple structures in the anterior segment of the eye including defects in the corneal endothelium, corneal stroma, ciliary body and disruption in the iridocorneal angle with adherence of the iris to the cornea. We further show that this phenotype leads to a significant increase in intraocular pressure and a subsequent loss of retinal ganglion cells and optic nerve degeneration, features indicative of glaucoma. Overall, our findings demonstrate that AP-2ß is required in the POM for normal development of the anterior segment of the eye and that the AP-2ß NCC KO mice might serve as a new and exciting model of ASD and glaucoma that is fully penetrant and with early post-natal onset.

Infant teething information on the world wide web: taking a byte out of the search.

INTRODUCTION: The purpose of this study was to describe and evaluate the quality of infant teething information on selected popular parenting Web sites. METHODS: Two checklists were used to evaluate the quality of the 16 parenting sites and infant teething-specific content included on each site. FINDINGS: Three of the 16 parenting sites did not contain teething-specific articles. Teething-specific content found on 13 of the 16 sites supported a connection between the process of teething and nonspecific symptoms with a perception that management is required. Popular management strategies included chewing on chilled objects, gingival massage, and the use of over-the-counter medications. Information about possible adverse effects of administering medications for infant teething was not found on the majority of sites. Eleven of the 16 sites advised parents to contact their primary care provider if they were uncertain about management for infant teething or whether the symptoms were related to illness. IMPLICATIONS: Although infant teething has an evidence base from which parents and professionals can make safe decisions about symptoms and treatment, translating the evidence into professional practice and health-related information on the Internet remains a challenge. Parents and pediatric health providers would benefit greatly from the development of clinical practice guidelines summarizing our present-day understanding of teething symptoms and the limited evidence supporting the use of over-the-counter medications.