Perda de peso inesperada na distrofia muscular de Duchenne
Reino Unido – ganho de peso é comum na distrofia muscular de Duchenne pela inatividade física e pelo uso de corticóides. Algumas pessoas perdem peso em alguns episódios e os autores estudaram estes pacientes com o objetivo de identificar as causas. Nesta população estudada as causas mais comumente encontradas foram: deterioração respiratória, a deterioração cardiovascular, infecções, os problemas de deterioração combinados respiratório, cardiovascular e gastroenterológica, agravamento de escoliose, após a cirurgia e perda de peso intencional. Nenhuma causa identificada em sete episódios. Os autores concluem que complicações cardio-respiratórias e digestórias são com maior frequência a causa de perda de peso em Duchenne, podendo indicar uma deterioração de órgãos em potencial e sempre deve ser investigada.
O resumo em inglês pode ser lido abaixo:
(Developmental Medicine & Child Neurology, 2013) Audit of unexpected weight loss in patients with Duchenne muscular dystrophy (also presented at UK Neuromuscular Translational Conference 2012)
R KULSHRESTHA, N SWIDERSKA, ,H STUART,S SPINTY – UK
Background: Significant proportion of individuals affected by Duchenne muscular dystrophy (DMD) are overweight, a number
are at risk of weight loss with disease progression. Poor nutrition can potentially have a negative effect on every organ system and
can contribute to reduced life expectancy. Objective: To identify the causes for weight loss in this patient group as a potential marker for deterioration in organ systems that might be amenable to targeted intervention. Method: Included in this retrospective observational study were individuals with DMD who were 10 years of age or older, who experienced weight loss in last 5 years. Descriptive statistics used to analyse the data in view of small numbers. Results: We identified 19/77 (24.7%) patients (age 11–17yr) who experienced 30 episodes of weight loss. The average weight loss was 6.29% (1.1–31%). Weight loss was attributed to various reasons: respiratory deterioration (3), cardiovascular deterioration (2), infections (2), combined problems of respiratory, cardiovascular and gastroenterological deterioration (9), worsening scoliosis (1), post surgery (4) and deliberate weight loss (2). No cause iden-tified in seven episodes (23.3%). Paired data were available for left ventricular fractional shortening and sitting forced vital capacity (FVC) 6 months prior and at the time of weight loss in 10 and 14 episodes respectively. 6/14 (43%) had reduction of FVC by an average of 7.7%. There was no change in fractional shortening. The average time to regain weight was 7.6 months (2–12m). 13 (43%) episodes had weight loss of >5% and 9 (47%) took longer than 6 months to regain weight.
Conclusion: Our study shows unexpected weight loss in patients with DMD might be more frequent than expected. Combined respiratory, cardiovascular and gastroenterological causes were responsible for weight loss in majority. Unexpected weight loss can be a marker for potential organ deterioration that should prompt a detailed history, examination, targeted investigations and supportive management.