Autoimmune polyglandular syndrome with shock and high anion gap metabolic acidosis.
Am J Med Sci
; 368(2): 162-166, 2024 08.
Article
en En
| MEDLINE
| ID: mdl-38801948
ABSTRACT
Autoimmune polyglandular syndrome (APS) is a rare group of immune-mediated disorders, which are typically, but not exclusively, related to the presence of endocrine abnormalities. APS type 2 is the most common subtype of the syndrome, more often observed in adulthood, with a characteristic clinical triad, which includes adrenal insufficiency, autoimmune thyroiditis and diabetes mellitus type 1. Adrenal insufficiency is an essential and necessary clinical manifestation of the syndrome, as it is observed in 100 % of the cases, while it can be accompanied by hyperchloremic metabolic acidosis. Herein, we present a 23 years-old patient with adrenal insufficiency in the context of autoimmune polyglandular syndrome type 2 with coexisting autoimmune thyroiditis and metabolic acidosis with an increased anion gap attributed to prolonged malnutrition. Additionally, we analyze the main clinical features of adrenal insufficiency, which is a central component of autoimmune polyglandular syndrome; highlight characteristics that differentiate the major APS subtypes.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Acidosis
/
Poliendocrinopatías Autoinmunes
/
Insuficiencia Suprarrenal
Límite:
Adult
/
Humans
Idioma:
En
Revista:
Am J Med Sci
Año:
2024
Tipo del documento:
Article
Pais de publicación:
Estados Unidos