Banca de QUALIFICAÇÃO: VINICIUS RODRIGUES DE OLIVEIRA

Uma banca de QUALIFICAÇÃO de DOUTORADO foi cadastrada pelo programa.
STUDENT : VINICIUS RODRIGUES DE OLIVEIRA
DATE: 28/08/2024
TIME: 10:00
LOCAL: Plataforma Google Meet
TITLE:

Construction and validation of a nursing care bundle to assist
children and adolescents with mucopolysaccharidosis type II.

KEY WORDS:

Mucopolysaccharidosis II. Nursing Care. Child. Adolescent. Validation Study.

PAGES: 51
BIG AREA: Ciências da Saúde
AREA: Enfermagem
SUMMARY:

Mucopolysaccharidosis type II or Hunter syndrome is a rare genetic disease that occurs
due to the absence of the enzyme iduronate 2-sulfatase, making the lysosomal cellular
mechanism incompetent to degrade glycosaminoglycans: dermatan sulfate and heparan
sulfate. The classic symptoms of the disease include problems in the central nervous
system (in severe cases), hepatosplenomegaly, recurrent ear infections, valvular,
respiratory, musculoskeletal complications and the appearance of hernias. Children and
adolescents with this disease require a broad network of care provided by an
interprofessional team, which must include a nurse. Although the relevance of this
professional in the care of people with mucopolysaccharidosis type II is recognized,
guidelines, protocols and other instruments that direct nursing care to this population are
scarce. Based on this context, the present study aims to construct and validate a nursing
care bundle to guide the care provided to children and adolescents with
mucopolysaccharidosis type II. A methodological study will be developed based on the
theoretical aspects of Pasquali's Psychological Instrument Development Model. The
study will be operationalized in four stages, namely: (1) Scoping Review, prepared
according to the guidelines of the JBI Manual for Evidence Synthesis (Edition 2024),
with the aim of mapping the main signs and symptoms of mucopolysaccharidosis type
II, in addition to relating them to possible nursing diagnoses and interventions; (2)
Mixed method study with convergent parallel design (QUAN + QUAL), to consist of
qualitative research based on Grounded Theory and quantitative research through cross-
sectional research, to understand the role of nurses in the care of children and
adolescents with mucopolysaccharidosis type II. Data collection for both studies will be
carried out remotely throughout the country with nurses working in specialized services
or referral centers for rare diseases; (3) Construction of the instrument based on the data
collected in the previous stages; (4) Validation of the bundle content, to be carried out
with expert judges, via Google forms and using the Delphi technique. Quantitative data,
referring to the judges' evaluation, will be analyzed based on the calculation of the
Content Validity Index and Cronbach's alpha coefficient. The study's ethical aspects are
supported by Resolution No. 466/2012 and will be submitted to the Research Ethics
Committee of the Federal University of Rio Grande do Norte.

COMMITTEE MEMBERS:
Interna - 1552864 - ALLYNE FORTES VITOR
Presidente - 2696718 - JONAS SAMI ALBUQUERQUE DE OLIVEIRA
Externo à Instituição - JOSÉ LUÍS GUEDES DOS SANTOS
Externa à Instituição - Rosângela Aparecida Pimenta Ferrari
Interna - 1214075 - SORAYA MARIA DE MEDEIROS