Roberta Poletti presented new imaging techniques for the early diagnosis of fibrosis in the heart of laminopathic patients.
Figure 3. Flow chart for diagnosis and follow-up of cardiac laminopathy. Progeroid laminopathies and familial partial lipodystrophy. Future trial developments The new perspectives for the treatment of laminopathies affecting adipose #PXD101 keyword# tissue and/or causing premature ageing have been presented. Paolo Sbraccia presented the clinical features and the outcome of the first clinical trial performed in MADA (16) by the use of statins and bisphosphonates. Alessandra Gambineri presented the flow-chart for diagnosis and follow-up of familial partial lipodystrophy (Fig. 4) and the efficacy of pioglitazone treatment in patients with Inhibitors,research,lifescience,medical metabolic disturbancies. Emanuela Scarano presented the flow-chart for diagnosis and follow-up of patients affected by HGPS (Fig. 5) and the clinical outcome in a patient undergoing a clinical trial using
statins and bisphosphonates. Giovanna Lattanzi presented new experimental therapeutical approaches for laminopathies affecting bone, such as MADA and HGPS, by the use of drugs limiting the levels of cytochines (TGFbeta 2 and osteoprotegerin) (17), and the rationale of therapies based on the use of statins and bisphosphonates. Further, recent data showing decline Inhibitors,research,lifescience,medical of IGF1 levels in models of progeroid laminopathies (18) have been reported as a suggestion for new pathogenetic mechanisms and/or new therapeutic perspectives. Figure
Inhibitors,research,lifescience,medical 4. Flow chart for diagnosis and follow-up of FPLD2. Figure 5. Flow-chart for the follow-up of metabolic laminopathies. Patients’ contribution Patients, affected by progeria or EDMD2, have presented their experiences and their point of view on diagnosis, follow-up and treatment of diseases. Inhibitors,research,lifescience,medical They suggested closer interplay among clinicians, researchers and patients and were in favour of the website as a way of information for family doctors and patients to improve diagnostic approach and follow-up. They also encouraged the research activity. Conclusions Animate discussions during this meeting clarified different points of view, and constructively resulted in a proposal for specific guidelines and flow-charts in laminopathies. The inter-disciplinary Astemizole approach to laminopathic disorders was highly encouraged. This was an enjoyable and fruitful workshop that will lead to new collaborations into the network (https://www.igm.cnr.it/index.php?id=383) and will contribute significantly to the improvement of future therapeutic perspectives in laminopathies. List of participants Enrico Bertini – Unit of Molecular Medicine for Neuromuscular and Neurodegenerative Diseases, Children’s Hospital and Research Institute “Bambino Gesù”, Rome. Elena Biagini – Institute of Cardiology, Policlinico S.Orsola-Malpighi, University of Bologna. Giuseppe Boriani – Institute of Cardiology, Policlinico “S. Orsola-Malpighi”, University of Bologna.