Severe congenital ichthyosiform dermatosis in CHIME syndrome successfully treated with ixekizumab.

Coloboma, congenital heart disease, ichthyosiform dermatosis, intellectual disability, conductive hearing loss, and epilepsy (CHIME) syndrome is a rare autosomal recessive neuroectodermal disorder caused by PIGL gene mutations. There is emerging literature to support the use of interleukin-17 (IL-17) antagonists in the treatment of certain ichthyosiform dermatoses. Here, we report a case of severe ichthyosiform dermatosis in a child with CHIME syndrome who was recalcitrant to multiple topical medications and dupilumab. This is the first reported case of successful treatment of congenital ichthyosiform dermatosis in a CHIME syndrome patient with ixekizumab, an IL-17A antagonist.

Bioactive borate glass triggers phenotypic changes in adipose stem cells.

A bioactive borate glass, 13-93B3 (B3), has been used successfully in the clinic to treat chronic, nonhealing wounds without scarring. However, the mechanism by which B3 stimulates wound healing is poorly understood. Because adipose stem cells (ASCs) have been shown to have multiple roles in wound repair, we hypothesized that B3 triggers ASCs. In this study, we evaluate the effects of B3 on ASC survival, migration, differentiation, and protein secretion in vitro. In concentrations ≤10 mg/ml, B3 did not affect ASC viability under static conditions. B3 promoted the migration of ASCs but did not increase differentiation into bone or fat. B3 also decreased ASCs secretion of collagen I, PAI-1, MCP-1, DR6, DKK-1, angiogenin, IL-1, IGFBP-6, VEGF, and TIMP-2; increased expression of IL-1R and E-selectin; had a transient decrease in IL-6 secretion; and had a transient increase in bFGF secretion. Together, these results show that B3 alters the protein secretion of ASCs.

Adult stem cell response to doped bioactive borate glass.

Bioactive glasses have transformed healthcare due to their versatility. Bioactive borate glass, in particular, has shown remarkable healing properties for both hard and soft tissues. Incorporating dopants into the composition of bioactive glass helps to control mechanical properties, and it increases their usefulness for clinical applications. Using a bioactive borate glass, 13-93B3 (B3), we investigated eleven dopants on the viability and migration potential of adipose stem cells (ASCs), a therapeutic source of cells used in tissue engineering and cell therapy. Our results show that under standard cell culture conditions, only Cu-doped B3 decreased cell viability, while only Y-doped B3 attracted ASCs as it dissolved in cell culture media. Using a transwell invasion assay, priming ASCs with Co, Fe, Ga, I, Sr, or Zn-doped B3 increased their homing capacity. Because there is widespread interest in optimizing and enhancing the homing efficiency of ASCs and other therapeutic cells, we then tested if priming bone marrow mesenchymal stem cells (BMSCs) with dopants also increased their homing capacity. In the case of BMSCs, there was a significant increase in invasion when cells were primed with any of the doped-B3 glasses. This work shows that incorporating dopants into borate glasses can provide a platform for a safe and efficient method that stimulates endogenous cells and healing mechanisms.