Clinical management of angioneurotic oedema patient post-orthognathic surgery

Angioneurotic oedema is an acute swelling involving the submucosal or subcutaneous tissues; it is most often located in the oral and maxillofacial region, which can result in upper airway obstruction. Its aetiology is frequently associated with immunoglobulin-mediated hypersensitivity reactions that elicit a heightened inflammatory response. The objective of this study was to report the case of a patient who developed an episode of angioneurotic oedema following combined orthognathic surgery. Pharmacological and nonpharmacological interventions used in the treatment of this important clinical condition are described and discussed.     

Successful medical treatment of EBV smooth muscle tumor in a renal transplant recipient

Belingheri M, Comoli P, Locatelli F, Baldanti F, Martina V, Giani M, Ferraresso M, Cro L, Edefonti A, Ghio L. Successful medical treatment of EBV smooth muscle tumor in a renal transplant recipient.
Pediatr Transplantation 2010: 14:E101–E104. © 2009 John Wiley & Sons A/S. Abstract: EBV is associated with various malignancies in patients with acquired or induced immune impairment. EBV‐SMT is very uncommon in immunocompromised patients, and a kidney localization has been described only anecdotally. We report the case of a 17‐yr‐old kidney transplant recipient diagnosed as having an EBV‐SMT inside the renal graft, which was successfully managed by minimizing isolated immunosuppression.     

Isolated bone marrow occurrence of classic Hodgkin's lymphoma in an HIV-negative patient

Classic Hodgkin's Lymphoma is a disease usually characterized by lymph nodal presentation. Rare cases with extra-nodal first clinical manifestation may occur, and primary bone marrow involvement may be part of them: isolated bone marrow forms have been recently described in the context of HIV infection. We herein report a case of isolated bone marrow Hodgkin's Lymphoma occurrence in a HIV-negative, elderly patient. The impacts of such unusual Hodgkin's Lymphoma manifestation on clinical practice as well as the related diagnostic and therapeutic implications are discussed.     

Initial fixation strength of a new hybrid technique for femoral ACL graft fixation: the bone wedge technique

Background Aperture fixation with interference screws matching the diameter of the tunnel is associated with the risk of graft laceration and graft rotation. Hypothesis A hybrid fixation technique (extracortical and aperture fixation) with undersized interference screw placed behind a bone wedge provides a higher fixation strength as aperture fixation with a screw alone matching the size of the tunnel. Study design Experimental laboratory study. Methods We evaluated the initial fixation strength (single cycle and cyclic loading tests) of hybrid and interference screw aperture fixation using different sized interference screws in porcine knees. Results Analysis of yield load, maximum load and stiffness in the single cycle loading test showed no statistically significant differences for hybrid fixation with a 1 mm undersized screw and aperture fixation with a screw matching the size of the tunnel. The use of an undersized screw alone resulted in low fixation strength. Conclusion The initial fixation strength of the hybrid technique with undersized screws is comparable to that of interference screw fixation matching the size. Clinical relevance The new “bone wedge fixation” is an alternative for ACL graft fixation without the risk of graft laceration and graft rotation.     

The prevalence and clinical implications of c-kit expression in plasma cell myeloma

AIM: To evaluate the clinical implications of c-kit (CD117) expression in plasma cell myeloma (PCM). METHODS AND RESULTS: We first evaluated the reliability of immunohistochemistry in assessing c-kit expression by comparing the results with those obtained by flow cytometry and gene expression arrays in 22 PCM and in 10 PCM cell lines. Immunohistochemical results showed a perfect concordance with those of flow cytometry; likewise, immunohistochemical and gene expression data were also concordant in all but one PCM and cell lines analysed. Then, we investigated the clinical implications of c-kit immunoreactivity in bone marrow biopsies of 85 PCM patients with a mean follow-up of 41 months. C-kit immunoreactivity was detected in 24 (28.2%) of the 85 cases and it was significantly associated with a high microvessel density, but not with traditional clinicopathological characteristics or with survival. CONCLUSIONS: Our findings suggest that immunohistochemistry is a reliable indicator of c-kit gene expression and reinforce the notion that approximately one-third of PCM express high levels of c-kit. The lack of association with traditional clinicopathological parameters and patient survival suggests that c-kit expression may not be an adjunct in predicting the clinical course of the disease.     

Ex vivo gene therapy with lentiviral vectors rescues adenosine deaminase (ADA)-deficient mice and corrects their immune and metabolic defects

Adenosine deaminase (ADA) deficiency is caused by a purine metabolic dysfunction, leading to severe combined immunodeficiency (SCID) and multiple organ damage. To investigate the efficacy of ex vivo gene therapy with self-inactivating lentiviral vectors (LVs) in correcting this complex phenotype, we used an ADA(-/-) mouse model characterized by early postnatal lethality. LV-mediated ADA gene transfer into bone marrow cells combined with low-dose irradiation rescued mice from lethality and restored their growth, as did transplantation of wild-type bone marrow. Mixed chimerism with multilineage engraftment of transduced cells was detected in the long term in animals that underwent transplantation. ADA activity was normalized in lymphocytes and partially corrected in red blood cells (RBCs), resulting in full metabolic detoxification and prevention of severe pulmonary insufficiency. Moreover, gene therapy restored normal lymphoid differentiation and immune functions, including antigen-specific antibody production. Similar degrees of detoxification and immune reconstitution were obtained in mice treated early after birth or after 1 month of enzyme-replacement therapy, mimicking 2 potential applications for ADA-SCID. Overall, this study demonstrates the efficacy of LV gene transfer in correcting both the immunological and metabolic phenotypes of ADA-SCID and supports the future clinical use of this approach.     

The Olive Leaves Extract Has Anti-Tumor Effects against Neuroblastoma through Inhibition of Cell Proliferation and Induction of Apoptosis

Neuroblastoma (NB) is the most common extra-cranial solid tumor of pediatric age. The prognosis for high-risk NB patients remains poor, and new treatment strategies are desirable. The olive leaf extract (OLE) is constituted by phenolic compounds, whose health beneficial effects were reported. Here, the anti-tumor effects of OLE were investigated in vitro on a panel of NB cell lines in terms of (i) reduction of cell viability; (ii) inhibition of cell proliferation through cell cycle arrest; (iii) induction of apoptosis; and (iv) inhibition of cell migration. Furthermore, cytotoxicity experiments, by combining OLE with the chemotherapeutic topotecan, were also performed. OLE reduced the cell viability of NB cells in a time- and dose-dependent manner in 2D and 3D models. NB cells exposed to OLE underwent inhibition of cell proliferation, which was characterized by an arrest of the cell cycle progression in G0/G1 phase and by the accumulation of cells in the sub-G0 phase, which is peculiar of apoptotic death. This was confirmed by a dose-dependent increase of Annexin V+ cells (peculiar of apoptosis) and upregulation of caspases 3 and 7 protein levels. Moreover, OLE inhibited the migration of NB cells. Finally, the anti-tumor efficacy of the chemotherapeutic topotecan, in terms of cell viability reduction, was greatly enhanced by its combination with OLE. In conclusion, OLE has anti-tumor activity against NB by inhibiting cell proliferation and migration and by inducing apoptosis.