The shelf-life of AVA is reported to be three years when stored between 2 °C and 8 °C (36 °F and 46 °F) and never frozen. History
Initial research and development (1954-1970)
The vaccine efficacy of AVA in humans was initially established by Philip S. Brachman of the United States Public Health Service (USPHS) in a controlled study undertaken between 1954 and 1959. The study field sites were four wool-sorting mills in the northeastern United States where employees were sometimes exposed to anthrax spores in the course of their work. Over the five years, 379 vaccinees were compared against 414 unvaccinated control subjects. There were 23 cases among controls (5 of them inhalation anthrax) compared with 3 cases among vaccinated (0 inhalation cases). The vaccine was judged to have a 92.5% vaccine efficacy against all types of anthrax experienced. Subsequently, there were no controlled clinical trials in humans of the efficacy of AVA due to the rarity of the condition (especially in the inhalational form) in humans and the ethical inadmissibility of conducting dangerous challenge studies in human subjects. Supportive animal challenge studies were done, however, showing that unvaccinated animals uniformly die whereas vaccinated animals are protected. About 95% of rhesus monkeys (62 of 65) survived challenge, as did 97% of rabbits (114 of 117). Guinea pigs (which are a poorer model for human anthrax than are monkeys or rabbits) showed a 22% protection (19 of 88).
described 4 patients (including 2 sibs, a male and a female) with clinical features very similar to those reported by Dionisi-Vici.In 2007 the renal tubular acidosis was another clinical complication described in only one case report of two brothers with Vici syndrome.In 2010 and 2012 it has also been reported a neuromuscular involvement in patients suffering from this syndrome.In 2013 Vici syndrome has been associated with mutations in the gene EPG5 (OMIM # 615068), which encodes an important regulator of the autophagy pathway, the ectopic P-granules autophagy protein 5, involved in the formation of lysosomes.In 2014 the ophthalmologic features of Vici syndrome were carefully evaluated.In 2015 the doctoral thesis entitled "Deciphering the mechanism of immune dysfunction in Vici Syndrome", University of Rome "La Sapienza" by Dr. Evangelos Axiotis, clarifies the molecular mechanisms and the role of the mutations in EPG5, all responsible for the immunodeficiency present in patients with Vici syndrome. References


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