Extracorporeal fetal support: a new animal model with preservation of the placenta.

Background: Previous models of support for premature sheep fetuses have consisted of cesarean delivery followed by catheterization of umbilical or central vessels and support with extracorporeal membrane oxygenation (ECMO). The limitations of these models have been insufficient blood flow, significant fetal edema, and hemorrhage related to anticoagulation.

Methods: We performed a gravid hysterectomy on 13 ewes between 135 and 145days gestational age.

An early presenting esophageal schwannoma.

Esophageal schwannoma is a rare diagnosis and historically has been a tumor of middle-aged females. We report a case of a 22-year-old male presenting initially with dyspnea secondary to tracheal compression from an 8 × 6 × 3.0 cm esophageal schwannoma.

Benchmarking the quality of care of infants with low-risk gastroschisis using a novel risk stratification index.

Background: The nationwide mortality of neonates with gastroschisis was compared to determine whether significant variations in outcome occurred at the hospital level.

Methods: Utilizing a previously developed risk-stratification index, low-risk neonates with gastroschisis were identified by a score of < or = 2. Only hospitals that had a record of treating >25 low-risk neonates were included in the analysis.

Mutations in TRPV4 cause Charcot-Marie-Tooth disease type 2C.

Charcot-Marie-Tooth disease type 2C (CMT2C) is an autosomal dominant neuropathy characterized by limb, diaphragm and laryngeal muscle weakness. Two unrelated families with CMT2C showed significant linkage to chromosome 12q24.11.

Differential regulation of TRPV1, TRPV3, and TRPV4 sensitivity through a conserved binding site on the ankyrin repeat domain.

Transient receptor potential vanilloid (TRPV) channels, which include the thermosensitive TRPV1-V4, have large cytoplasmic regions flanking the transmembrane domain, including an N-terminal ankyrin repeat domain. We show that a multiligand binding site for ATP and calmodulin previously identified in the TRPV1 ankyrin repeat domain is conserved in TRPV3 and TRPV4, but not TRPV2. Accordingly, TRPV2 is insensitive to intracellular ATP, while, as previously observed with TRPV1, a sensitizing effect of ATP on TRPV4 required an intact binding site.