The fetal porcine aorta and mesenteric acellular matrix as small-caliber tissue engineering vessels and microvasculature scaffold.

Background: The extracellular matrix (ECM) is characterized by not only well-preserved scaffolds of organs and vascularized tissues, but also by extremely low immunogenicity during allo- or xeno-implantation. This study aimed to establish a model of a composite microvasculature network scaffold with a small-caliber-dominant vascular pedicle by decellularizing fetal porcine aorta and the conterminous mesentery.

Methods: The aorta and the conterminous mesenteric vascular system originating from the inferior mesenteric artery were harvested from fetal pigs at late gestation.

The role of Frizzled-4 mutations in familial exudative vitreoretinopathy and Coats disease.

Aim: The aim of this study is to assess the role of Frizzled-4 (FZD4) in familial exudative vitreoretinopathy (FEVR) and Coats disease.

Methods: Tissue samples were collected for DNA extraction and automated DNA sequencing of the two coding exons of FZD4 in both directions. Cases carrying a FZD4 mutation and demonstrating extreme disease severity were selected for direct automated sequencing of all coding exons of LRP5, NDP and TSPAN12.

Severe retinopathy of prematurity associated with FZD4 mutations.

Purpose: To determine whether mutations in the FZD4 gene are a risk factor for developing severe ROP.

Methods: Three Canadian tertiary care centers recruited premature infants prospectively and retrospectively, and assigned affectation status based on the maximum degree of severity of ROP recorded in both eyes. Mutation screening of the FZD4 gene was performed using direct sequencing.

Phenotypic overlap of familial exudative vitreoretinopathy (FEVR) with persistent fetal vasculature (PFV) caused by FZD4 mutations in two distinct pedigrees.

Purpose: To describe a severe familial exudative vitreoretinopathy (FEVR) phenotype seen in infancy that resembles persistent fetal vasculature (PFV) caused by mutations in the FZD4 gene in two pedigrees with high intrafamilial variability.

Methods: Three infants presented with features compatible with bilateral PFV. Eye examinations from the affected children and their relatives were reviewed retrospectively (follow-up:18 months-9 years).

Mutant frizzled-4 disrupts retinal angiogenesis in familial exudative vitreoretinopathy.

Familial exudative vitreoretinopathy (FEVR) is a hereditary ocular disorder characterized by a failure of peripheral retinal vascularization. Loci associated with FEVR map to 11q13-q23 (EVR1; OMIM 133780, ref. 1), Xp11.