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

Copyright © 1999 EDIZIONI MINERVA MEDICA

language: Italian

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


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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.

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