Ectodermal dysplasia treated with one-step surgical rehabilitation: a case report.

Abstract Ectodermal dysplasia (ED) comprises a large heterogeneous group of inherited disorders that are characterized by primary defects in the skin, hair, nails, eccrine glands and teeth. The most characteristic findings are the reduced number of teeth. All rehabilitative programmes involve proper evaluation of skeletal relationships. Prosthetic-implantological treatment at the end of bony growth can be used. In this article a case of ED treated with Le Fort I for maxillary advancement, femur homografts, implants' insertion and immediate loading is described. In December 2007, a 38-year-old female was referred to the Maxillofacial Department of Galeazzi Hospital (Milan, Italy) who had a diagnosis of ED. Twelve implants were inserted in one-step surgical procedure. No implant was lost and all are stable. The occlusion is stable after 15 months of follow-up. The results indicate that the one-step oral rehabilitation can be performed in adults who are affected by ED. Also, this significantly reduces the time of oral and facial rehabilitation

Efficacy and toxicity of FLAI vs ICE for induction treatment of newly diagnosed AML patients, younger than 60 years

Acute myeloid leukaemias (AMLs) are a heterogeneous family of hemopoietic malignancies that share a high frequency and a high degree of Pgp-mediated multidrug resistance (MDR), one of the major causes of treatment failure. Induction treatment (FLAI), including 5 days administration of Idarubicin (IDA- 10mg/sqm/day), in combination with high-dose Arabinosyl Cytosine (HDAC-2g/sqm/day) and Fludarabine (FLUDA-25mg/sqm/day) was adopted as induction treatment of newly diagnosed patients with AML, except acute promyelocytic leukemia (APL). In our previous experience (Russo et al. Leuk. Lymphoma, 2001), FLAI regimen showed to be highly effective (CR rate 72%), also in AML MDR-pos patients, with a low extra-hematological toxicity. In this prospective randomized trial, FLAI was compared with ICE (IDA 10mg/sqm/day x 3 days + AC p.c 100mg/sqm/day x 10 days + VP16 100mg/sqm/day x 5 days) for induction of remission. Post-induction treatment program included: HDAC (3g/sqm/12h x 6 days), MEC (Mitoxantrone 12 mg/sqm/day x 4 days, Etoposide 100 mg/sqm/day x 4 days, Cytarabine 1 gr/sqm/day x 4 days) and allogeneic or autologous BMT. Over a period of 2 years, 118 patients were randomly assigned to FLAI (67) or ICE (51). The clinical and hematological characteristics of the two patient population were not different. In the FLAI group, the complete remission (CR) rate was 72% after the first course and 76% after HDAC; in the ICE group, the CR rate was 53% and 69%, respectively (P 1.0 x 109/L and platelets > 50 x 109/L was significantly better in FLAI arm than in ICE arm. In both groups, it was seen an approximately equal rate of FUO, Gram negative/Gram positive bacteremias and systemic fungal infections. Infections and haemorrhages caused death during induction (DDI) in 3% of patients treated with FLAI and in 10% of patients treated with ICE. Non-hematological toxicity of FLAI was mild and significantly lower than ICE. In particular, in the FLAI arm, only 3/67 pts developed a grade 3 or 4 gastro-intestinal toxicity, whereas in ICE arm 16/51 patients experienced this toxicity (p=0.0001). Other grade 3 or 4 toxicity (ipertransaminasemia, cutaneus toxicity, renal or cardiac failure) were seen in 1/67 pts in FLAI arm versus 7/51 pts in ICE arm (p=0.02). In conclusion, these preliminary results strongly suggest that FLAI, as single induction course, is an highly effective regimen with a limited non-haematologic toxicity. Furthermore, FLAI seems to be more effective than ICE to overcome Pgp-mediated multidrug resistance. 2005, The American Society of Hematolog