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Background The COVID-19 pandemic has brought substantial challenges for current practices in treating hereditary neuromuscular disorders (hNMDs). However, this infection has not been the only concern for these patients. Social distancing has compromised multidisciplinary assistance and physical activity, and has brought about several mental health issues. We presented a follow-up on 363 patients with hNMDs at a Brazilian tertiary center during the peak of the COVID-19 pandemic. Objective We aimed to show the frequency and severity of SARS-CoV-2 infection among hNMD patients and to demonstrate the effects of the pandemic on life habits, disease progression and multidisciplinary supportive care status. Methods Three hundred and sixty-three patients (58% male and 42% female) were followed for three months through three teleconsultations during the peak of the COVID-19 pandemic in Brazil. Results There were decreases in the numbers of patients who underwent physical, respiratory and speech therapies. For several patients, their appetite (33%) and sleep habits (25%) changed. Physical exercises and therapies were interrupted for most of the patients. They reported new onset/worsening of fatigue (17%), pain (17%), contractions (14%) and scoliosis (7%). Irritability and sleep, weight and appetite changes, and especially diminished appetite and weight loss, were more frequent in the group that reported disease worsening. There was a low COVID-19 contamination rate (0.8%), and all infected patients had a mild presentation. Conclusion The isolation by itself was protective from a COVID-19 infection perspective. However, this isolation might also trigger a complex scenario with life habit changes that are associated with an unfavorable course for the NMD. RESUMO Antecedentes: A Pandemia por COVID-19 tem trazido desafios subtanciais para a prática clínica no tratamento das doenças neuromusculares hereditárias (DNMh). A infecção não tem sido a única preocupação para os pacientes. O distanciamento social tem comprometido a assistência multidisciplinar, atividade física e tem trazido problemas mentais em decorrência do próprio isolamento. Nós apresentamos aqui um seguimento de 363 pacientes com DNMh de um centro terciário Brasileiro durante o pico da Pandemia de Covid-19. Objetivos: Mostrar a frequência e gravidade da infecção por Sars-Cov-2 em pacientes com DNMh e demonstrar os efeitos da pandemia nos hábitos de vida, na progressão da doença e no cuidado multidisciplinary. Métodos Trezentos e sessenta e três pacientes (58% homens and 42% mulheres) foram acompanhados por 3 meses através de 3 teleconsultas durante o pico da Pandemia de Covid-19 no Brasil. Resultados Houve um decréscimo no número de pacientes que faziam terapia física, respiratória e fonoaudiológica. Em muitos pacientes, o apetite (33%) e hábitos do sono (25%) se alteraram. Exercícios físicos e terapias foram interrompidas pela maioria dos pacientes. Physical exercises and therapies were interrupted for most of the patients. Eles relataram piora ou aparecimento de fadiga (17%), dor (17%), retrações (14%), e escoliose (7%). Irritabilidade, mudanças no sono, peso e apetite, sendo principalmente diminuição do apetite e peso foram mais frequentemente encontrados em pacientes que apresentaram piora clinica da doença. Houve uma baixa taxa de contaminação por Covid-19 (0.8%), e todos os pacientes infectado apresentaram quadro clinico leve. Conclusão O isolamento por si só se mostrou protetor na perspectiva de infecção por Covid-19, mas pode desencadear um cenário complexo com mudanças nos hábitos de vida e curso desfavorável da doença de base.

BACKGROUND: Nonalcoholic fatty liver disease is the leading cause of liver pathology. The mainstay of management is weight loss. Our aim was to evaluate responses to nutritional counseling in long-term patients with this condition. METHODS: A prospective cohort study with consecutive inclusion of 105 subjects with nonalcoholic fatty liver disease who received individualized low-calories diet counseling (1400 to 1600 kcal/day according to gender) every three months for 24 months. Weight loss of 5% or more was considered as a therapeutic response. RESULTS: Out of 105 patients, 45 (42.9%) did not return for a second evaluation. Mean age was 55 ± 9 years, 81.6% were women and mean body mass index was 31.9 (23.8-44.9) kg/m2. Follow-up time was 6.5 (3.2-26.9) months and median appointment number was 3 (2-11). Metabolic syndrome and hypercholesterolemia were more common in women. The number of subjects who lost more than 5% weight was: 5/20 (25%) at 6-months; 3/15 (33%) at 12 months; 3/18 (17%) at 18 months and 4/13 (31%) at the end of follow up. The median body weight loss at 6, 12, 18 and 24 months decreased significantly. CONCLUSIONS: Adherence to nutritional counseling is poor in patients with nonalcoholic fatty liver disease. Only a very small proportion of patients reached the targeted body loss of weight on long term. RESUMO OBJETIVOS: A doença hepática gordurosa não alcoólica éa principal causa de patologia hepática. Essencial para seu manejo éa perda de peso. Nosso objetivo foi avaliar as respostas a aconselhamento nutricional em pacientes crônicos com esta condição. METODOS: Estudo prospectivo de coorte com inclusão consecutiva de 105 indivíduos com doenca hepática gordurosa não alcoólica que receberam dieta individualizada de baixa caloria (1400-1600kcal/dia, de acordo com o sexo) e aconselhamento a cada 3 meses, durante 24 meses. A perda de peso de 5% ou mais foi considerada como resposta terapêutica adequada. RESULTADOS: Dos 105 pacientes, 45 (42,9%) não voltaram para uma segunda avaliação. A média de idade foi de 55 ± 9 anos, 81,6% eram mulheres e o índice de massa corporal foi de 31,9 (23,8-44,9) kg/m2. O tempo de seguimento foi de 6,5 (3,2-26,9) meses e número médio de entrevistas foi de 3 (2-11). A síndrome metabólica e a hipercolesterolemia foram mais comuns em mulheres. O número de indivíduos que perderam mais de 5% em peso foi: 5/20 (25%) em 6 meses; 3/15 (33%) aos 12 meses; 3/18 (17%) e aos 18 meses 4/13 (31%) no final do seguimento. A perda de peso corporal média aos 6, 12, 18 e 24 meses diminuiu significativamente. CONCLUSÕES: A adesão ao aconselhamento nutricional épobre em pacientes com doenca hepática gordurosa não alcoólica. Apenas uma pequena proporcão de pacientes que atingiu a perda de peso corporal programada a longo prazo.

BACKGROUND: Nonalcoholic fatty liver disease is the leading cause of liver pathology. The mainstay of management is weight loss. Our aim was to evaluate responses to nutritional counseling in long-term patients with this condition. METHODS: A prospective cohort study with consecutive inclusion of 105 subjects with nonalcoholic fatty liver disease who received individualized low-calories diet counseling (1400 to 1600 kcal/day according to gender) every three months for 24 months. Weight loss of 5% or more was considered as a therapeutic response. RESULTS: Out of 105 patients, 45 (42.9%) did not return for a second evaluation. Mean age was 55 ± 9 years, 81.6% were women and mean body mass index was 31.9 (23.8-44.9) kg/m2. Follow-up time was 6.5 (3.2-26.9) months and median appointment number was 3 (2-11). Metabolic syndrome and hypercholesterolemia were more common in women. The number of subjects who lost more than 5% weight was: 5/20 (25%) at 6-months; 3/15 (33%) at 12 months; 3/18 (17%) at 18 months and 4/13 (31%) at the end of follow up. The median body weight loss at 6, 12, 18 and 24 months decreased significantly. CONCLUSIONS: Adherence to nutritional counseling is poor in patients with nonalcoholic fatty liver disease. Only a very small proportion of patients reached the targeted body loss of weight on long term.