Título :	Estudo de parâmetros clínicos e metabólicos em pacientes com epidermólise bolhosa hereditária e sua relação com a densidade mineral óssea
Autor :	Alves, Natália Ribeiro de Magalhães
Orientador(es)::	Castro, Luiz Claudio Gonçalves de
Assunto::	Epidermólise bolhosa Densidade mineral óssea Vitamina D Índice de massa corporal (IMC)
Fecha de publicación :	8-mar-2018
Data de defesa::	24-nov-2017
Citación :	ALVES, Natália Ribeiro de Magalhães. Estudo de parâmetros clínicos e metabólicos em pacientes com epidermólise bolhosa hereditária e sua relação com a densidade mineral óssea. 2017. 119 f., il. Dissertação (Mestrado em Ciências da Saúde)—Universidade de Brasília, Brasília, 2017.
Abstract:	Introduction: Hereditary epidermolysis bullosa (HEB) corresponds to a group of diseases clinically characterized by fragility of the skin and mucosa, with the consequent formation of blisters after minimal traumas. Low bone mineral density (BMD) and fractures are possible complications of the severe forms of HEB and may have multifactorial etiology. Objectives: To evaluate clinical, anthropometric and metabolic variables of patients with HEB followed at the Brasilia University Hospital and to study the correlation among those variables and the areal bone mineral density (aBMD) of the patients. Methods: This was a cross-sectional observational study involving eight individuals with HEB, consisting of six females, three with EB simplex (EBS), five with recessive dystrophic EB (RDEB), mean age 16.4 } 8.3 years . A clinical history, physical examination, anthropometric evaluation, metabolic assessments and DXA of the lumbar spine (L1-L4) and of the total body in children and adolescents were performed. In individuals older than 18 years, in addition to the previous exams, DXA of the proximal femur was also performed. The aBMD of individuals with short stature was adjusted for age-stature. The possible correlations of clinical and metabolic variables with aBMD were evaluated. Results: No patient with EBS presented short stature or low body mass index (BMI). Four patients (three EBS) had normal mobility. Only patients with RDEB had anemia (mean hemoglobin 7.9 ± 2.1 g/dL), 25(OH)D insufficiency (mean 15.8 ± 2.6 ng/mL) and high C-reactive protein (CRP) (mean 5.8 ± 1.5 mg/dL). The mean interleukin (IL) -6 of patients with RDEB (38.9 ± 19.9 pg/mL) was significantly higher than that of patients with EBS (3.1 ± 3.4 pg/mL), p = 0.014. Three patients with RDEB presented low bone mass in the lumbar spine, corrected for age-stature, and no patient presented low bone mass when evaluating the total body corrected for age-stature. aBMD of L1-L4 showed a strongly positive and significant correlation with the degree of mobility of the patients (r = 0.86, p = 0.013), when an outlier was removed. There was a strong and significant correlation between lumbar spine aBMD and BMI (r = 0.79749, p = 0.0317), serum concentrations of 25(OH)D (r = 0.80474, p = 0.0290) and hemoglobin (r = 0.76887, p = 0.0434) There was a strong positive and significant correlation between the serum concentrations of IL-6 and CRP (r = 0.86, p = 0.005), but those inflammatory markers did not present statistically significant correlations with aBMD. Conclusion: In relation to patients with EBS, patients with RDEB presented greater impairment of height and nutritional status, lower concentrations of 25(OH)vitamin D and hemoglobin levels, and higher values of inflammatory parameters. In the studied group, positive and significant correlations were found between aBMD of the lumbar spine and the degree of mobility of the patient, nutritional status and serum concentrations of vitamin D and hemoglobin.
metadata.dc.description.unidade:	Faculdade de Ciências da Saúde (FS)
Descripción :	Dissertação (mestrado)—Universidade de Brasília, Faculdade de Ciências da Saúde, Programa de Pós-Graduação em Ciências da Saúde, 2017.
metadata.dc.description.ppg:	Programa de Pós-Graduação em Ciências da Saúde
Licença::	A concessão da licença deste item refere-se ao termo de autorização impresso assinado pelo autor com as seguintes condições: Na qualidade de titular dos direitos de autor da publicação, autorizo a Universidade de Brasília e o IBICT a disponibilizar por meio dos sites www.bce.unb.br, www.ibict.br, http://hercules.vtls.com/cgi-bin/ndltd/chameleon?lng=pt&skin=ndltd sem ressarcimento dos direitos autorais, de acordo com a Lei nº 9610/98, o texto integral da obra disponibilizada, conforme permissões assinaladas, para fins de leitura, impressão e/ou download, a título de divulgação da produção científica brasileira, a partir desta data.
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