Translation and cross-cultural adaptation of the Charcot-Marie-Tooth disease Pediatric Scale to Brazilian Portuguese and determination of its measurement properties.

BACKGROUND: The Charcot-Marie-Tooth disease Pediatric Scale (CMTPedS) has been used to measure aspects of disability in children with all types of Charcot-Marie-Tooth disease (CMT). OBJECTIVE: To translate and cross-culturally adapt the CMTPedS into Brazilian-Portuguese and determine its reliability and validity. METHODS: The translation and cross-cultural adaptation followed international guidelines recommendations. Twenty individuals with CMT were assessed. Two examiners assessed the participants for inter-rater reliability. Face validity was assessed by eight physical therapists that judged the relevance of each test item. The Bland-Altman analysis (bias) and standard error of measurement (SEM) complemented the analysis. Furthermore, intraclass correlation coefficients (ICC), weighted kappa (k), and internal consistency (Cronbach's alpha) was determined. RESULTS: The CMTPedS was successfully translated and cross-culturally adapted. Twenty children/youth were enrolled in the study. Of these, the majority (55%) were girls with a mean age of 13.9 (range: from 6 to 18) years. Regarding face validity, the CMTPedS-Br showed relevant items for assessing children and youth with CMT. The ICC for the total score showed excellent reliability (ICC2.1  = 0.93, 95% CI = 0.84, 0.97). The most reliable items were grip, dorsiflexion and plantar flexion strength while the least reliable items were pinprick, vibration, and gait. The internal consistency was excellent (α = 0.96, 95% CI = 0.91, 0.99) and the agreement showed small variability (bias = 0.15, 95% CI= -4.28, 4.60). CONCLUSION: The CMTPedS-Br showed adequate reliability and face validity to measure disability in individuals with CMT. This tool will allow Brazil to be part of multicentered studies on such a rare but debilitating condition.

Vigilância do desenvolvimento neuropsicomotor de crianças de um programa DST/AIDS / Neurodevelopmental surveillance of children participating in the STD/AIDS program

A terapia anti-retroviral de alta potência (TARV) é uma forma eficaz de prevenção da transmissão do vírus HIV de mãe para filho. No entanto, os estudos ainda investigam os efeitos da exposição intraútero à TARV, dentre eles o atraso no desenvolvimento neuropsicomotor (DNPM). O presente estudo apresenta o relato de um projeto de extensão, cujos objetivos foram verificar o DNPM de crianças de um programa DST/AIDS, orientar as famílias considerando seu contexto socioeconômico e realizar encaminhamentos para serviços de saúde específicos. A vigilância do DNPM foi feita em três etapas: (1) avaliação em ambulatório; (2) avaliação e orientações em domicílio; (3) elaboração de relatórios aos gestores de saúde. Foram utilizados os testes DENVER II e o PEDI, além de um questionário socioeconômico. Participam do programa DST/AIDS 15 crianças, sendo 12 soro-revertidas, 1 soropositiva e 2 indefinidas. Doze crianças foram avaliadas, e os domínios mais comprometidos foram linguagem, pessoal-social e motor fino, respectivamente. Quanto ao nível econômico, 73,3% pertenciam ao nível E, e 58,3% das mães eram analfabetas ou cursaram apenas o primário. Crianças filhas de mães HIV positivo, além de fatores biológicos, geralmente estão expostas a fatores de risco ambientais que contribuem para alterações do DNPM. Desta forma, o acompanhamento por uma equipe de profissionais de saúde, em parceria com a família da criança, torna-se uma importante ferramenta para a identificação e intervenção precoce.

Highly active antiretroviral therapy (HAART) is an effective way of preventing mother-to-child transmission of HIV. However, further studies investigate the effects of short and long term exposure to HAART in-utero and its consequence on child neuropsychomotor development (NPMD). The paper presents a report and discussion of results of an extension project whose objectives were to verify the NPMD of children participating of the STD/AIDS program, to orientate families according to their socioeconomic context and make referrals to specific health services. The NPMD surveillance was divided into three parts: (1) ambulatory evaluation; (2) home evaluation and orientations; (3) reporting health managers. DENVER II and PEDI tests were used and also a socioeconomic questionnaire. Fifteen children were on the program of which 12 uninfected, 1 HIV+ and 2 indeterminate. Twelve children were evaluated and the most impaired domain were language, personal-social and fine motor, respectively. Regarding to socioeconomic status, 73,3% were E level and 58,3% of mothers were analphabet or had primary school. Children born of infected mothers, besides the biological risks, usually are exposed to environment/social risks that can affect the NPMD. Thus, monitoring by a team of health professionals, in partnership with the child's family, becomes an important tool for identification and early intervention.