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REVISIÓN SISTEMÁTICA - INSULINA PARA EL TRATAMIENTO DEL SÍNDROME PHELAN MCDERMID
Authors: Sánchez Lozano, J., Martínez Pizarro, S
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Introduction: Phelan-McDermid syndrome is a rare, heterogeneous and complex genetic disorder caused by haploinsufficiency of the SHANK3 gene on chromosome 22q13.3. This syndrome is characterized by neurobehavioral symptoms. Therapeutic approaches are complex and must focus on variable physical and behavioral symptoms. In recent years, the use of intranasal insulin and insulin-like growth factor in patients with Phelan-McDermid syndrome has been proposed. The objective of this review is to evaluate the effectiveness of these treatments.
Method: A review was carried out following the PRISMA regulations. The databases of PubMed, Cinahl, PsycINFO, SPORTDiscus, Academic Search Complete, Lilacs, IBECS, CENTRAL, SciELO, and WOS were consulted. The Cochrane tool was used to assess the risk of bias and the quality of the evidence was GRADE assessed.
Results: Intranasal insulin and insulin-like growth factor are effective in patients with Phelan-McDermid syndrome. The total sample was 53 patients from a total of five studies. All studies showed safety, tolerability and no major side effects.
Conclusions: Intranasal insulin administered to patients with Phelan-McDermid syndrome improves gross and fine motor activities, cognitive functions, educational level, non-verbal communication, autonomy and social skills. On the other hand, IGF-1 is effective in improving symptoms of sensory reactivity, repetitive behaviors, hyperactivity, social isolation, and neuropsychological and behavioral symptoms in general.