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The bone marrow (BM) in myelodysplastic syndromes (MDS) undergoes pathobiological changes that mimic an inflammatory process, and hence, an infectious etiology was suspected in these disorders. In the present report, we examined the bone marrow mononuclear cells (BMMNC) of 19 MDS patients and seven normal donors for the expression of one latency-related (Latency membrane protein 1 (LMP-1) and immediate early protein (IEP)) and one activation-related (BZLF and DNA-Pol) m-RNA each for two herpes viruses, Epstein-Barr virus (EBV) and cytomegalovirus (CMV), respectively. Reverse transcriptase polymerase chain reaction was used for this purpose. The latency-related transcripts (EBV-LMP-1 and CMV-IEP) were present in all the MDS and normal specimens. Intriguingly, 10/19 MDS specimens ( approximately 53%) and 2/7 normal donors ( approximately 28%) were positive for active EBV-BZLF (P=0.0067), while 2/19 MDS specimens ( approximately 11%) with 1/7 normal ( approximately 14%) showed active CMV-DNA-Pol (P=0.1588). Later, from another set of MDS patients (n=7) and normal donors (n=4), BM stromal cultures were established, which, at a 75% confluency, were overlaid with cord blood mononuclear cells (CBMNC). IEP was detectable in the CBMNC before and after co-incubation with MDS, as well as normal stroma. So, it was also present both in MDS and normal stromal cells. The other three were absent both in MDS and normal stromal layers. In CBMNC though, active EBV-BZLF and CMV-DNA-Pol m-RNA were detectable in one of seven MDS co-cultures each, albeit from different patients. None of the normal co-cultures showed active virus, either in stroma or CBMNC. Thus, the present report demonstrates, for the first time, the presence of active herpes viruses in the BMMNC of MDS patients and reveals the ability of the MDS stroma to support the viral activation.

Background: Le Fort III distraction osteogenesis improves midface form and dental relationships in patients with syndromic craniosynostosis, but its effect on the upper airway is not well documented. Methods: A retrospective review was conducted of patients with syndromic craniosynostosis undergoing Le Fort III distraction osteogenesis from 2000 to 2006 (n = 20). Changes in velar angle and nasopharyngeal, velopharyngeal, oropharyngeal, and hypopharyngeal spaces were measured cephalometrically. Three-dimensional airway casts were created from computed tomographic data to ascertain circumferential airspace changes. Patients with the preoperative diagnosis of severe obstructive sleep apnea or a tracheostomy were designated as having significant airway compromise. Cephalometric differences in the preoperative superior airspace were compared between patients with and without significant airway compromise. Improvement in the symptoms of obstructive sleep apnea was studied. Results: Cephalometric analysis revealed an increase in the velar angle (121 degrees to 148 degrees; p < 0.001) and an increase in the nasopharyngeal (3.9 mm to 13.0 mm; p < 0.001) and velopharyngeal airspaces (2.0 mm to 5.9 mm; p< 0.01). Three-dimensional computed tomographic analysis confirmed these findings. Comparison between preoperative cephalograms of patients with (n = 10) and without significant airway compromise (n = 10) revealed smaller nasopharyngeal (2.2 mm versus 5.7 mm; p < 0.05) and velopharyngeal airspaces (0.9 mm versus 3.0 mm; p= 0.05). Nine of 10 patients with significant airway compromise experienced improvement in their symptoms of obstructive sleep apnea or had their tracheostomy removed. Conclusions: Le Fort III distraction osteogenesis significantly increases nasopharyngeal and velopharyngeal airspaces in patients with syndromic craniosynostosis. Midface distraction improves but does not resolve all causes of obstructive sleep apnea in this patient population.