Rett syndrome (RTT) is a pervasive neurodevelopmental disorder mainly linked to mutations in the gene encoding the methyl-CpG-binding protein 2 (MeCP2). Respiratory dysfunction, historically credited to brainstem immaturity, represents a major challenge in RTT. Our aim was to characterize the relationships between pulmonary gas exchange abnormality (GEA), upper airway obstruction, and redox status in patients with typical RTT (n = 228) and to examine lung histology in a Mecp2-null mouse model of the disease. GEA was detectable in ~80% (184/228) of patients versus ~18% of healthy controls, with “high” (39.8%) and “low” (34.8%) patterns dominating over “mixed” (19.6%) and “simple mismatch” (5.9%) types. Increased plasma levels of non-protein-bound iron (NPBI), F2-isoprostanes (F2-IsoPs), intraerythrocyte NPBI (IE-NPBI), and reduced and oxidized glutathione (i.e., GSH and GSSG) were evidenced in RTT with consequently decreased GSH/GSSG ratios. Apnea frequency/severity was positively correlated with IE-NPBI, F2-IsoPs, and GSSG and negatively with GSH/GSSG ratio. A diffuse inflammatory infiltrate of the terminal bronchioles and alveoli was evidenced in half of the examined Mecp2-mutant mice, well fitting with the radiological findings previously observed in RTT patients. Our findings indicate that GEA is a key feature of RTT and that terminal bronchioles are a likely major target of the disease.
Most of research in regenerative oral surgery describes materials or techniques for increasing volumetric results for implant-supported prosthesis. The use of bio-materials in alveolar ridge preservation after tooth extraction commonly leads to a delayed recovery. Bromelain is an enzyme that belongs to a family of proteolytic enzymes derived from the stem of the pineapple plant (Ananas comosus) with effectiveness in decreasing the inflammation development and swelling. The present paper reports a prospective comparative study performed in order to test the possible use of oral bromelain 40 mg in alveolar ridge preservation. Evaluations were performed at three time points after the surgery: after 2 days (t1), after 7 days (t2) and after 14 days (t3). A statistically significant difference among patients that used bromelain and patients that used placebo resulted among the use of bromelain and lower Visual Analogue Scale (VAS) at t1 (r = −0.75, p = 0.0067), t2 (r = −0.90, p = 0.0001) and t3 (r = −0.8566, p = 0.0008). Bromelain therapy reported a statistically significant difference among patients that used bromelain and patients that used placebo even with regards to the use of bromelain and postoperative swelling at t1 (r = −0.79, p = 0.0034), t2 (r = −0.81, p = 0.0020) but not at t3 (r = −0.34, p = 0.2967). With the result of the present paper, and the poorness of contraindication of the investigated drug, bromelain may be suggested to be used for patients that undergo to alveolar ridge preservation after tooth extraction.
We assume there is the responsibility of the expression of lymphocytes, not only type but also as quantity, in determining RL or WL manifestation of OLP. Circulating lymphocytes may have a role, too.
Results of this study showed an uneventful recovery for all patients with our approach and reduced healing time of bone graft. So extraoral approach with submental incision and calvarial graft is a reliable method in reconstruction of atrophic mandibles, and staged implant surgery is suggested.
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