1- Interna de Anestesiologia, Centro Hospitalar de Lisboa Central
2- Interna de Anestesiologia, Centro Hospitalar do Porto
3 - Assistente Hospitalar, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central
4 - Directora do Serviço de Anestesiologia, Centro Hospitalar de Lisboa Central
- Euroanaesthesia 2016, Londres (Poster)
Resumo: Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder associated with deficiencies in 21-,11-, or 17-hydroxylase (OHD). More than 90% of CAH is caused by 21OHD. Clinical manifestations are mainly due to adrenocortical insufficiency, hyperandrogenism and adverse effects of glucocorticoids supplementation. Anesthetic management surrounds the complications of ACTH suppression due to steroid therapy, mainly hypertension, hyperglycemia, electrolyte abnormalities, adrenal and pituitary suppression and immune suppression. Children with CAH can be managed successfully during anesthesia after understading the patophysiology of disease with proper history and perioperative steroid supplementation. Hemodynamic and metabolic stability, as well a good pain control are major concerns in the anesthetic management of this patients.
Palavras Chave: Congenital adrenal hyperplasia, glucocorticoids