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Home > Journals > Minerva Anestesiologica > Past Issues > Minerva Anestesiologica 1998 November;64(11) > Minerva Anestesiologica 1998 November;64(11):529-34



A Journal on Anesthesiology, Resuscitation, Analgesia and Intensive Care

Official Journal of the Italian Society of Anesthesiology, Analgesia, Resuscitation and Intensive Care
Indexed/Abstracted in: Current Contents/Clinical Medicine, EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 2,036

Frequency: Monthly

ISSN 0375-9393

Online ISSN 1827-1596


Minerva Anestesiologica 1998 November;64(11):529-34


Therapeutical goals and therapeutic strategies in NBIA 1 (Hallervorden-Spatz syndrome)

Bruscoli F., Corsi A., Cavicchi C., Carloni R., Ferioli I., Gemmani A., Crociati M., Pompili A.

AUSL Rimini - Presidio Ospedaliero di Rimini, Servizio di Anestesia, Rianimazione e Terapia Antalgica

A 10 years old male patient, DG, was admitted in the ICU because of continuous uncontrolled movements due to a neurologycal degenerative disease (Hallervorden-Spatz syndrome) able to determine reduction of spontaneous breathing efficacy. At admission he presented acute ventilatory failure, because of a Staphy-lococcus aureus broncopneumonia, so he had a tracheal tube and mechanical ventilation (pressure support). During hospitalization (4 months in ICU and 2 months in Pediatric Department) DG received tracheotomy and percutaneous gastrostomy, to obtain adeguate spontaneous ventilation and artificial enteral nutrition; a satisfactory pharmacological control of choreo-athetosic movements, with not great interference with original sleep-awake cycle, was obtained. Actually DG is living in his family (9 months follow-up); he has tracheotomy and percutaneous gastrostomy; he can relate with the environment; in a few months, he’ll go to school again. He need 30 daily administrations of 8 different drugs; family, supported by an integrated multidisciplinary équipe, takes care of him. The role of Intensivist is essential not only in the management of acute phases in cronic diseases, but also in the long-term management of a homely care.

language: Italian


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