Generation and identification of thymic epithelial progenitor cells pTEC by in-vitro processing of human thymic fragments for allotransplantation.

The procedure of generation and identification of stromal progenitor cells derived from human thymic fragments (PL patent 378431) has been described in this article. Our aim was to prepare material for transplantation in elderly people. The method is based on in-vitro processing of thymic fragments to get rid of all immunogenic elements of lymphocytes, endothelial cells, macrophages, and fibroblasts. In the thymic culture process, this organ dies out in the incubation medium and epithelial cells emerge out of the organ. After about 4 weeks from the start of the culture, the population of various developmental forms of epithelial cells was generated, namely CK AE1/AE3+, SDF-1 alpha+ and a weak expression of FGF+ S-100+. Finally, we obtained approximately 3 million cells as a monolayer. The progenitor cells were experimentally transplanted into a 72-year-old volunteer in order to prove that they do not induce neither a local nor a systemic rejection response.

Chromosome 22q11.2 microdeletion in children with conotruncal heart defects: frequency, associated cardiovascular anomalies, and outcome following cardiac surgery.

The association of conotruncal heart defects with 22q11.2 chromosomal microdeletions is well established. However, it is not clear whether particular types of conotruncal malformations or additional cardiovascular anomalies are associated with microdeletions. In addition, cardiac surgery outcome in children with conotruncal defects and del22q11.2 is not well described. We prospectively enrolled 214 children with conotruncal defects: 126 with tetralogy of Fallot (TOF), 18 with pulmonary atresia-ventricular septal defect (PA-VSD), 15 with truncus arteriosus communis (TAC) type I, one with interrupted aortic arch (IAA) type B, and 54 with the transposition of great arteries, who were consecutively hospitalized at the Pediatric Cardiology Department between 2003 and 2005. 22q11.2 microdeletion was identified by fluorescence in situ hybridization. The postoperative course following cardiac surgery was compared in patients with TOF and its more severe form, PA-VSD, with/without del22q11.2 (groups A and B) and TAC with/without del22q11.2 (groups C and D). In 15 of 214 patients, 22q11.2 microdeletion was diagnosed (in 11 with TOF/PA-VSD, in three with TAC, in one with IAA type B). In patients with TOF/PA-VSD and microdeletion anatomic features that were significantly associated with 22q11.2, deletion included right aortic arch (p = 0.018), aberrant right subclavian artery (p < 0.001), and major aortopulmonary collateral arteries (p = 0.016). A complicated postoperative course was more frequent and mortality was higher in patients with conotruncal defects and with/without microdeletion. We conclude that additional cardiovascular anomalies are significantly more frequent in children with 22q11.2 microdeletion and TOF/PA-VSD. Children with conotruncal heart defects and 22q11.2 microdeletion more frequently experienced complicated postoperative course after cardiac surgery.