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2.
Br J Dermatol ; 191(2): 177-186, 2024 Jul 16.
Artigo em Inglês | MEDLINE | ID: mdl-38863109

RESUMO

BACKGROUND: Therapeutic patient education (TPE) is recommended for children with atopic dermatitis (AD), but no consensus has been reached on the optimal tailoring of delivery. While repeated multidisciplinary group education sessions have shown effectiveness, the benefits of one-on-one educational interventions led by nurses for children with AD have not yet been assessed. OBJECTIVES: To assess the benefits of additional, well-structured, 1-h nurse-led individual TPE interventions in children with AD and their families compared with standard care alone. METHODS: Children with moderate-to-severe AD and their parents were randomized to receive a 1-h nurse-led education session in addition to standard care vs. standard care alone. The primary outcome was the area under the curve (AUC) of the SCORing of Atopic Dermatitis index (SCORAD) from baseline to week 24 (lower AUC values represent better long-term control of the disease). RESULTS: In our study, 176 patients were randomized across 11 centres, and 153 were included in the full analysis set. The mean (SD) age was 4.47 (4.57) years. By week 24, there were no significant differences in the AUCs of the SCORAD between the two groups (P = 0.3). Secondary outcomes including patient-reported severity and quality of life [AUCs of the patient-oriented SCORAD (PO-SCORAD) and Infants' Dermatitis Quality of Life Index (IDLQI), Children's Dermatitis Quality of Life Index (CDLQI) and Family Dermatitis Quality of Life Index (FDLQI)] were not significantly different between the two groups. The only significant change observed in the intervention group, when compared with the one receiving standard care, was a decrease in topical steroid phobia, as assessed by the topical corticosteroid phobia (TOPICOP) score. Prespecified subgroup analyses showed that disease severity in the intervention group was significantly lower throughout the study, compared with the standard-care group when participants had moderate AD at baseline (n = 47); while participants with severe AD at baseline (n = 106) did not show benefit from the intervention. Participants showed no additional benefit from the intervention regardless of age group. CONCLUSIONS: This study did not show any additional effectiveness, in long-term severity control, of a 1-h nurse-led TPE intervention in children with AD treated with standard care, compared with those treated with standard care alone. However, it should be noted that the intervention reduced the fear of using topical steroids and may be beneficial for patients in the subgroup with moderate AD.


Atopic dermatitis (AD), also known as atopic eczema, is a chronic relapsing disease that affects 7­15% of children worldwide. Therapeutic patient education (TPE) is recommended for children with AD, but no agreement has been reached on the best way to tailor delivery. While repeated multidisciplinary group education sessions in a hospital setting have been found effective, this type of intervention requires a lot of resources and is time-consuming. To assess the benefits of TPE in children with AD, researchers in France carried out this study with children with moderate-to-severe AD, to compare a 1-hour nurse-led education session in addition to standard care vs. standard care alone. The main aim of this research was to assess the effectiveness of a TPE intervention over a period of 6 months, using a measurement tool called the SCORAD (SCORing of Atopic Dermatitis index). We found no additional benefits in terms of long-term severity control and quality of life at 6 months of a 1-hour nurse-led education intervention in children with AD treated with standard care. However, it should be noted that the intervention reduced the fear of using topical steroids and may be beneficial for people in the moderate AD subgroup.


Assuntos
Dermatite Atópica , Educação de Pacientes como Assunto , Humanos , Dermatite Atópica/terapia , Dermatite Atópica/enfermagem , Masculino , Feminino , Pré-Escolar , Criança , Resultado do Tratamento , Qualidade de Vida , Pais/educação , Lactente
3.
J Eur Acad Dermatol Venereol ; 38(9): 1818-1827, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-38595321

RESUMO

BACKGROUND: Data on dermatological manifestations of Costello syndrome (CS) remain heterogeneous and lack in validated description. OBJECTIVES: To describe the dermatological manifestations of CS; compare them with the literature findings; assess those discriminating CS from other RASopathies, including cardiofaciocutaneous syndrome (CFCS) and the main types of Noonan syndrome (NS); and test for dermatological phenotype-genotype correlations. METHODS: We performed a 10-year, large, prospective, multicentric, collaborative dermatological and genetic study. RESULTS: Thirty-one patients were enrolled. Hair abnormalities were ubiquitous, including wavy or curly hair and excessive eyebrows, respectively in 68% and 56%. Acral excessive skin (AES), papillomas and keratotic papules (PKP), acanthosis nigricans (AN), palmoplantar hyperkeratosis (PPHK) and 'cobblestone' papillomatous papules of the upper lip (CPPUL), were noted respectively in 84%, 61%, 65%, 55% and 32%. Excessive eyebrows, PKP, AN, CCPUL and AES best differentiated CS from CFCS and NS. Multiple melanocytic naevi (>50) may constitute a new marker of attenuated CS associated with intragenic duplication in HRAS. Oral acitretin may be highly beneficial for therapeutic management of PPHK. No significant dermatological phenotype-genotype correlation was determined between patients with and without HRAS c.34G>A (p.G12S). CONCLUSIONS AND RELEVANCE: This validated phenotypic characterization of a large number of patients with CS will allow future researchers to make a positive diagnosis, and to differentiate CS from CFCS and NS.


Assuntos
Síndrome de Costello , Proteínas Proto-Oncogênicas p21(ras) , Humanos , Síndrome de Costello/genética , Síndrome de Costello/complicações , Estudos Prospectivos , Feminino , Masculino , Criança , Proteínas Proto-Oncogênicas p21(ras)/genética , Adolescente , Pré-Escolar , Adulto , Adulto Jovem , Displasia Ectodérmica/genética , Síndrome de Noonan/genética , Síndrome de Noonan/complicações , Acantose Nigricans/genética , Diagnóstico Diferencial , Ceratodermia Palmar e Plantar/genética , Cardiopatias Congênitas/genética , Cardiopatias Congênitas/complicações , Fenótipo , Papiloma/genética , Papiloma/patologia , Acitretina/uso terapêutico , Sobrancelhas/anormalidades , Sobrancelhas/patologia , Insuficiência de Crescimento/genética , Insuficiência de Crescimento/etiologia , Lactente , Ceratolíticos/uso terapêutico , Fácies
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