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Palmino Sacco

Publications and source records attributed to Palmino Sacco.

4 recordsLinked to original sources

Global developmental delay, osteopenia and ectodermal defect: a new syndrome.

UNLABELLED: Global developmental delay is a serious social problem. It is often unrecognized and the phenotypes are inadequately studied. To investigate the phenotypes of children with aspecific central nervous system (CNS) impairment (poor speech, maladaptive behavioral symptoms such as temper tantrums, aggressiveness, poor concentration and attention, impulsiveness, and mental retardation). SETTING: Tertiary care hospital. PATIENTS: Three children (two male siblings, and one unrelated girl). METHODS: We used the results from clinical neurological evaluations; imaging and electrodiagnostic studies; metabolic and genetic tests; skin biopsies and bone mineral densitometry. All three children suffered from (A) global developmental delay, (B) osteopenia, and (C) identical skin defects. The skin ultrastructural abnormalities were abnormal keratin differentiation, consisting of hyperkeratosis and granular layer thickening; sweat gland abnormalities, consisting of focal, cytoplasmic clear changes in eccrine secretory cells; and melanocyte abnormalities, with both morphological changes (reduced number and size without evident dendritic processes), and functional changes (defects in the migration of melanosomes in the keratinocytes). These patients present a previously unrecognized syndrome. We retain useful to report this new association, to be recognized, in the next future, as a specific key-sign of a well-defined genetic defect.

Biopsy↗

PACS implementation in a university hospital in Tuscany.

We describe cost/benefit analysis for the implementation of a picture archiving and communication system in the Department of radiology of University of Siena, Italy. We also highlight technical and functional innovations provided by information of several department units: cost conversion, workflow optimisation and operator development. We analysed: operator costs, paper costs, film costs, chemical costs, costs of optical disks and location rent for hardware and software of RIS/PACS. The results show that advantages provided by PACS implementation derive from a workflow optimization and saving of human resources rather than from a reduction in films and chemicals. Moreover, better management of radiological unit provides improved handling of clinical information, resulting in reduced time to initiate clinical action, with reduction in average length of patient stay and improvements in overall health outcomes.

Cost-Benefit Analysis↗

[Radiotherapy of malignant gliomas: results from conventional treatment methods and the prospects of advanced techniques].

PURPOSE: The results of post-operative radiation therapy of malignant gliomas are disappointing, with mean survival time (MST) of 16-70 weeks and 2-year survival rates ranging from 8.5% to 25% in the literature. A slightly more favourable prognosis is found in the following cases: in anaplastic astrocytomas with respect to glioblastoma multiforme; in younger patients with respect to the more elderly; the longer the duration of symptoms before diagnosis; and in the event in which surgery has been macroscopically radical. An improvement in treatment outcome is foreseeable with the use of advanced volume definition techniques for radiation therapy. MATERIALS AND METHODS AND RESULTS: Our experience with conventional radiation treatment shows therapeutic results in agreement with other institutions. In the overall 134 cases MST was 50 weeks and the 2-year survival rate was 10%. In patients affected by anaplastic astrocytoma MST was 58% and 2-year survival rate was 17%, whereas the figures for glioblastoma multiforme were 47 weeks and 8% (p>0.05, not statistically significant, probably due to the small number of cases). Patients of sixty years of age or less showed a more statistically favourable prognosis: MST was 59 weeks and 2-year survival rate was 16%, compared with 44 weeks and 4% in patients above 60 years of age (p<0.05). The duration of symptoms of 6 months or less had a less favourable prognosis with respect to symptom onset of greater than 6 months: in the former MST was 49 weeks and 2-year survival was 7%, and in the latter the figures were 68 weeks and 40% (P<0.05). Lastly, the presence of residual neoplastic tissue after surgery is an unfavourable element: in this case MST was 41 weeks and 2-year survival was 7%, compared with 68 weeks and 13% (P<0.05) after macroscopically radical surgery. DISCUSSION AND CONCLUSIONS: Computed tomography (CT) is still today an indispensable technique for radiation therapy planning. Magnetic Resonance (MR) imaging, nonetheless, provides greater definition of the neoplastic extension. The possibility of combining CT and MR neuroimaging data together with stereotactic radiotherapy techniques enables the optimal development of the three-dimensional treatment plane. This translates into high dose delivery to the neoplastic volumes without affecting the regions of the brain with no tumour involvement. Furthermore, a real improvement in the prognosis of malignant gliomas must also consider the results from research in the fields of tumour biology and functional neuroimaging.

Adolescent↗