Total Intravenous Anesthesia (TIVA) in an Infant with Werdnig-Hoffmann Disease. Case Report
نویسندگان
چکیده
منابع مشابه
Total intravenous anesthesia (TIVA) in an infant with Werdnig-Hoffmann disease. Case report.
BACKGROUND AND OBJECTIVES Werdnig-Hoffmann disease is the most common cause of hypotonia in infants and its prognosis is worse if it is present shortly after delivery. Symmetrical muscular weakness, areflexia, and fasciculations of the tongue are characteristic. The majority of the infants die before two years of age as a consequence of respiratory failure. The present report presents a case in...
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The case of an infant with Werding-Hoffmann disease, who died at the age of 4 1/2 days, is reported. At autopsy there was severe cerebellar hypoplasia, associated with degenerative changes in the brain-stem nuclei. This case supports the concept that cerebellar hypoplasia may develop as a manifestation of the neuronal abiotrophy of Werdnig-Hoffmann disease. A similarity has been noted between t...
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The prevalence of Werdnig-Hoffmann disease was found to be 0.66 per 10,000 livebirths in Hungary from 1973 to 1980. However, the true prevalence is estimated to be 1 per 10,000 livebirths, which exceeds the level of a previously published English study. There was no higher fetal death rate in previous and subsequent pregnancies of index patients' mothers. The occurrence in sibs was 32%, probabl...
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ژورنال
عنوان ژورنال: Brazilian Journal of Anesthesiology
سال: 2010
ISSN: 0034-7094
DOI: 10.1016/s0034-7094(10)70069-6