Beckwith-Widemann Macroglossia: The Role of Surgical Tongue Reduction.

Unlabelled: This review was conducted to define the natural history of unoperated Beckwith-Wiedemann syndrome (BWS) macroglossia and the effect of tongue reduction surgery upon breathing, eating, speaking and dentoskeletal development in individuals having BWS. This is a retrospective study of medical records.

Setting: All patients were evaluated and treated in one of two Children's Hospitals by an ACPA approved Craniofacial Team.

Ear Abnormalities.

Congenital ear abnormalities present an aesthetic and psychosocial concern for pediatric patients and their parents. Diagnosis of external ear deformities is based on clinical examination and is facilitated by an understanding of normal ear anatomy. Ear anomalies can be categorized as malformations or deformations.

Reduction Glossectomy for Macroglossia in Beckwith-Wiedemann Syndrome: Is Post-Op Intubation Necessary?

Objective: Macroglossia is a characteristic feature of Beckwith-Wiedemann syndrome (BWS), commonly treated with reduction glossectomy to restore form and function. There exists no consensus on the perioperative management of these patients undergoing tongue reduction surgery, including anecdotal information regarding how long postoperative intubation should be maintained. The aim of this study is to evaluate the necessity of prolonged postoperative intubation in patients receiving tongue reduction surgery via the surgical and anesthetic management methods at our center.

Galectin-9 bridges human B cells to vascular endothelium while programming regulatory pathways.

Humoral immunity is reliant on efficient recruitment of circulating naïve B cells from blood into peripheral lymph nodes (LN) and timely transition of naive B cells to high affinity antibody (Ab)-producing cells. Current understanding of factor(s) coordinating B cell adhesion, activation and differentiation within LN, however, is incomplete. Prior studies on naïve B cells reveal remarkably strong binding to putative immunoregulator, galectin (Gal)-9, that attenuates BCR activation and signaling, implicating Gal-9 as a negative regulator in B cell biology.

Crouzon syndrome mouse model exhibits cartilage hyperproliferation and defective segmentation in the developing trachea.

Crouzon syndrome is the result of a gain-of-function point mutation in FGFR2. Mimicking the human mutation, a mouse model of Crouzon syndrome (Fgfr) recapitulates patient deformities, including failed tracheal cartilage segmentation, resulting in a cartilaginous sleeve in the homozygous mutants. We found that the Fgfr2 mutants exhibited an increase in chondrocytes prior to segmentation.