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Sudden cardiac death (SCD) is one of the most common causes of death in developed countries. In Italy, an annual incidence of 0.7 per 1000 inhabitants per year can be estimated. SCD represents the main cause of sudden death in children, adolescents and young adults and often occurs in young and previously asymptomatic patients. This issue has acquired even greater relevance since implantable cardioverter-defibrillators have proved to be highly effective in preventing sudden death in high-risk subjects. Autopsy findings of young SCD victims include inherited cardiac disorders with a defined morphologic substrate but also hearts without any identifiable structural abnormalities (sudden unexplained death). The potential heritability of the underlying disorder makes surviving relatives at risk of sudden death. A cardiological workup in these families may allow identification of cardiac disease and may unmask affected surviving relatives in whom the disease had remained unrecognized. Cardiological and genetic assessment of relatives of SCD victims based on current literature is reported in this review as well as our experience on SCD in young people in the Lazio Region (Italy) between 2001 and 2008.

Aim: The aim of this prospective study was to evaluate a new surgical approach for the immediate rehabilitation of fully edentulous maxilla without any type of bone grafting., Methods: The technique named ''V-II-V'' consists of an immediately loaded full-arch bridge supported by six implants. Two distal implants engage the posterior wall of the maxillary sinus, being tilted 30-45 degrees relative to the occlusal plane. Two other tilted implants engage the anterior wall of the sinus, and finally two axial implants are inserted in the anterior maxilla. An acrylic provisional restoration is delivered within 4 hours of implant placement. At each follow-up, plaque and bleeding indexes were scored, marginal bone level change around tilted and axial implants were evaluated, and a questionnaire was compiled to assess patient's satisfaction for function and aesthetics., Results: Twenty-one patients were rehabilitated. A total of 126 implants were inserted. Nineteen prostheses were in function for more than 12 months. The average follow-up was 20 months (range 4-35 months). No implant failure occurred to date, leading to 100% survival rate and 100% prosthesis success. No complication was recorded. Peri-implant bone loss at axial and tilted fixtures was similar at one year, being 0.8 mm (SD 0.4, N.=28) and.0.9 mm (SD 0.5 mm, N.=56) respectively (P>0.05)., Results: The ''V-II-V'' technique is a viable treatment modality for the immediate rehabilitation of the edentulous maxilla, avoiding bone grafting or sinus augmentation. Long-term evaluation is needed to confirm these encouraging preliminary

Aim: Near infrared spectroscopy (NIRS) is a non invasive optical technique to assess the monitoring of oxygenation and cerebral hemodynamics. Aim of our study was to value cerebral hemodynamics during major surgery to reduce the period of possible modifications of cerebral oxygenation., Methods: Twenty-five newborns which underwent surgical intervention (8 diaphragmatic hernia, 8 esophageal atresia, 1 neck lymphangioma, 8 intestinal malformation) were studied during surgery by means of NIRS (NIRO 300), using an electrode applied to the scalp in the frontoparietal region. We monitored the Tissue Oxygenation Index (TOI) as well as the changes in concentration of total haemoglobin (tHb), oxygenated haemoglobin (O2Hb) and deoxygenated haemoglobin (HHb). The changes have been expressed as difference from the basal value recorded at the beginning of surgery., Results: During the surgical intervention O2Hb, tHb and TOI decreased (DeltaO2Hb=-11.4+/-6.5 microM; P<0.001; DeltatHb=- 7.54+/-4.3 microM, P<0.05; microTOI=-12.5+/-5.5%, P<0.001), and HHb increased (DeltaHHb=+4.80+/-2.30 microM, P<0.001); the greatest changes occurred when the viscera were positioned into the abdomen (in diaphragmatic hernia and intestinal malformation)., Conclusion: The present study suggests that NIRS, during major surgery, is able to monitor oxygenation and cerebral hemodynamics thus allowing a real time evaluation of some intraoperative procedure aftereffects that, if timely modified, could reduce cerebral hypoxia risks.

Aim: The aim of this study was to assess the impact and the peculiarities of cerebral palsy (CP) in children discharged from our neonatal intensive care unit (NICU) from January 1998 to April 2004., Methods: A total of 2 303 children were discharged from our NICU during this period and 1 912 were followed up for 1 year through neurological examination (traditional, Brazelton, general movements) and cranial ultrasound (US); high-risk newborns were evaluated with brain magnetic resonance imaging (MRI) too., Results: In 65 children (3.4% of the follow-up group) were diagnosed CP, and classified as follows: 21 (32%) diplegia, 19 (29%) quadriplegia, 20 (31%) hemiplegia, 4 (6%) double hemiplegia, 1 (2%) dyskinetic form. In diplegia and quadriplegia prevailed low birth weight infants (less than or equal to 2,500 g) and preterm infants, while in hemiplegia prevailed normal birthweight infants (greater than 2,500 g) and infants at term. The main MRI findings were: in diplegia 82% periventricular white matter lesions; in quadriplegia 94% periventricular and/or subcortical white matter lesions; in hemiplegia 95% bilateral periventricular or subcortical white matter lesions, predominating on contralateral cerebral hemisphere; in double hemiplegia 100% periventricular and/or subcortical white matter lesions, 100% enlargement of subarachnoid spaces; in dyskinetic form 100% basal ganglia lesions., Conclusions: The impact of CP in children discharged from our NICU, in agreement with the literature, is higher than in the total population of newborns, thus it is very important to evaluate carefully high-risk newborns during hospitalization and follow-up, through neurological examination and radiologic imaging (US, MRI), for an accurate and early treatment.

Three patients with neonatal thrombosis of the middle cerebral artery are described. In two patients the thrombosis was secondary to neonatal asphyxia. The third patient had a porencephalic cyst twenty days after birth suggesting a prenatal origin of the thrombotic event. The follow-up was carried out for two years. In the first year, two patients had infantile spasms and hyparrhythmic pattern of EEG. This type of epilepsy has never been previously reported. Futhermore, all three patients presented hemiplegia and reduction of cranial circumference. The neuroradiologic procedures showed to be very useful for the diagnosis and long term follow-up. These patients must be followed carefully since the development of a subsequent epilepsy is reported to be approx 11%.

The vein of Galen aneurysmal malformation (VGAM) is a rare cerebro-vascular disorder in neonates. It is characterized by an abnormal direct communication between one or several cerebral arteries and the vein of Galen. It may appear in the neonatal period or afterwards. Three cases of patients affected by VGAM with different clinical expression are presented. Two of them were treated successfully with endovascular embolization. It was not possible to provide the same treatment to the third patient for an intractable congestive heart failure already existing when VGAM was diagnosed.

Objectives: The aim of this clinical study was to verify the therapeutic activity of cumarinic extract of Melilotus officinalis (CEMO) in patients with chronic lymphedema of the upper arm caused by lymphadenectomy for breast cancer. Cumarine, in fact, has antiedemic properties due to macrophagic action that stimulates proteolysis in the tissues affected by chronic lymphedema., Patients and Methods: In an open clinical study we enrolled 24 patients with chronic upper arm lymphedema due to post-lymphadenectomy of the axilla for breast cancer. 21 patients were eligible to receive 400 mg of CEMO containing 8 mg of cumarine in a sole daily administration for 6 months. We measure the circumference of the upper arm at 3 and 6 months from treatment. We evaluated the symptoms and tolerability through a questionnaire given to the patients at every clinical control., Results: Of the 21 (87.5%) patients eligible, only 14 (66.6%) were treated with CEMO according to protocol. Of these 11 patients (52.3%) had a reduction of the circumference of the affected arm of 5% with respect to base values. Three patients (14.2%) had no change. In 12 patients (57.1%) symptoms improved. As for tolerability: 3 patients (14.2%) had transitory gastrointestinal side-effects. There was worsening of lymphedema and symptoms in 4 patients (19%) that did not receive CEMO and were followed as controls. Three patients (14.2%) were not evaluable because they were lost to follow-up., Conclusions: Cumarinic extract of Melilotus officinalis (CEMO) was effective in reducing lymphedema in 79% of the pts treated for a period of six months. The median reduction of the upper arm circumference was modest (5% with respect to initial values) but statistically significant (p = 0.048). Treatment with CEMO for lymphedema could be associated to the physiotherapy given to these patients such as manual lymph drainage (MLD).

An assessment was made of the fourth heart sound as a non-invasive clinical and instrumental aid in the diagnosis of latent cardiac insufficiency. The sound was recorded in various decubitus positions in a series of healthy subjects and patients with recent and past infarction, under basal conditions, after exercise on the cycle ergometer, and after digitalis i.v. Persistence of the fourth sound in the standing position was noted in nearly all infarct patients, while its incidence decreased significantly after digitalis. It is felt that this finding can be interpreted as evidence of the non-existence of latent heart failure. Its further investigation and correlation with other invasive and non-invasive instrumental examinations is proposed.

A group of heart patients and another group of healthy subjects have been studied and their systolic intervals (PEP-LVET - PEP/LVET ratio and the telediastolic index) evaluated using the mechanocardiographic system. In both long-standing and recent heart patients, the parameters studied showed significant deviations from the norm. These were even more marked after exercise on the ergonometer bicycle. Acute doses of digitalis reduced deviations from the norm observed under basal conditions and after exercise. Polycardiography, it is concluded, assists in the gathering of extremely valuable information about cardiac performance in relation to latent cardiac insufficiency. Especially useful information can be deduced from PEP indications and from the PEP/LVET ratio. The use of digitalis is suggested for physically active post-infarction subjects in order to give greater tolerance of physical exercise. The concept of long-term polygraphic studies as part of out-patient follow-ups on such patients is outlined.

Implantation of pure cryopreserved epithelial cells obtained by enzymatic digestion of human amnion was successfully carried out in one patient affected by Niemann-Pick disease type B. No host-versus-graft reaction was recorded after implantation. The clinical improvement observed in this patient is supposed to be effect of the documented increase of sphingomyelinase activity in his leukocytes after implantation, confirming the possibility that an effective release of sphingomyelinase from amniotic epithelial cells and enzyme uptake by deficient cells can occur. Separation and cryopreservation of human amnion epithelial cells markedly improve the procedures of implantation and may represent a further step beyond in the enzymatic therapy of many lysosomal storage disorders.