Conhecimento, atitudes e práticas dos profissionais da enfermagem acerca da síndrome de Berardinelli-Seip no Hospital Universitário Onofre Lopes

Abstract

Berardinelli-Seip Syndrome (BSCL), also known as Generalized Congenital Lipodystrophy (LGG), is a rare autosomal recessive genetic disease of low prevalence with 1: 10,000,000 people. LGC patients have a very characteristic phenotype with acromegaloid facial appearance, prognathism, atrophic cheeks, prominent musculature, acanthosis nigricans, phlebomegaly, umbilical potency, among others. The aim of the research was to evaluate the level of knowledge, attitudes and practices of nurses at the Hospital Universitário Onofre Lopes - HUOL on Berardinelli-Seip Syndrome. This is a transverse, descriptive and exploratory study of a quantitative approach, in which 74 nurses from HUOL were interviewed, located in the city of Natal, Northeastern Brazil. In this research, it was possible to observe that the majority of nurses are unaware of the aspects of BSCL regarding morphology, physiology, genetic causes, treatment and importance of multiprofessional treatment and assistance to patients with the syndrome. According to the above, due to the fact that the nurse is the health professional who has more contact with his patients, his unrestricted importance in the care of patients with BSCL is evidenced. Finally, it is necessary to report the poor knowledge of nurses regarding the follow-up and care in the hospital context of people with BSCL and the extreme need for periodic training in this field.