RESUMO
Individuals with epidermolysis bullosa (EB) may present with a broad spectrum of growth impairment and multiorgan disorders, with compromised nutritional status and quality of life. The provision of nutrients through a gastrostomy tube may minimize EB-related malnourishment but may also result in skin injuries and infections. In this systematic review we consider the current evidence about the effectiveness of gastrostomy in restoring nutritional status and improving quality of life in patients with EB. Seven studies (n = 146) met selection criteria and patients ranged in age from 6 weeks to 33 years of age. Although it is not a risk-free procedure, the placement of a gastrostomy tube is a feasible and safe alternative to provide nutritional support and to improve the quality of life of patients.
Assuntos
Epidermólise Bolhosa Distrófica/cirurgia , Gastrostomia , Estado Nutricional , Qualidade de Vida , Adolescente , Adulto , Criança , Pré-Escolar , Nutrição Enteral/métodos , Métodos Epidemiológicos , Epidermólise Bolhosa Distrófica/psicologia , Feminino , Humanos , Lactente , Masculino , Desnutrição/prevenção & controle , Desnutrição/psicologia , Adulto JovemRESUMO
Epidermolysis bullosa (EB) consists of a group of genetic hereditary disorders in which patients frequently present fragile skin and mucosa that form blisters following minor trauma. More than 20 subtypes of EB have been recognized in the literature. Specific genetic mutations are well characterized for most the different EB subtypes and variants. The most common oral manifestations of EB are painful blisters affecting all the oral surfaces. Dental treatment for patients with EB consists of palliative therapy for its oral manifestations along with typical restorative and periodontal procedures. The aim of this article is to describe two dental clinical treatments of recessive dystrophic EB cases and their specific clinical manifestations. The psychological intervention required during the dental treatment of these patients is also presented.
Assuntos
Epidermólise Bolhosa Distrófica/terapia , Doenças da Boca/terapia , Adolescente , Anti-Infecciosos Locais/uso terapêutico , Vesícula/psicologia , Vesícula/terapia , Pré-Escolar , Clorexidina/uso terapêutico , Placa Dentária/terapia , Restauração Dentária Permanente , Epidermólise Bolhosa Distrófica/psicologia , Feminino , Seguimentos , Gengivite/terapia , Humanos , Arcada Parcialmente Edêntula/reabilitação , Masculino , Doenças da Boca/psicologia , Úlceras Orais/terapia , Cuidados Paliativos , Planejamento de Assistência ao Paciente , Doenças Periodontais/psicologia , Doenças Periodontais/terapia , Radiografia Panorâmica , Extração Dentária , Escovação DentáriaRESUMO
Epidermolysis bullosa (EB) consists of a group of genetic hereditary disorders in which patients frequently present fragile skin and mucosa that form blisters following minor trauma. More than 20 subtypes of EB have been recognized in the literature. Specific genetic mutations are well characterized for most the different EB subtypes and variants. The most common oral manifestations of EB are painful blisters affecting all the oral surfaces. Dental treatment for patients with EB consists of palliative therapy for its oral manifestations along with typical restorative and periodontal procedures. The aim of this article is to describe two dental clinical treatments of recessive dystrophic EB cases and their specific clinical manifestations. The psychological intervention required during the dental treatment of these patients is also presented.