Case report, case study
https://doi.org/10.21857/9xn31cdoly
Preoperative assessment of patient with APECED syndrome
Anja Mandarić
orcid.org/0000-0002-0344-9028
; University Hospital Centre Zagreb, Department of Anesthesiology, ICU and Pain Therapy, Zagreb, Croatia
*
Nataša Piljek - Margaretić
; University Hospital Centre Zagreb, Department of Anesthesiology, ICU and Pain Therapy, Zagreb, Croatia
Zrinka Orešković
; University Hospital Centre Zagreb, Department of Anesthesiology, ICU and Pain Therapy, Zagreb, Croatia
Sandra Morović
; Aviva Medical Centre
* Corresponding author.
Abstract
Autoimmune polyendocrinopathy-candidiasis-ectodermal-dystrophy (APECED) or autoimmune polyglandular syndrome type 1 (APS-1) is a rare autosomal recessive genetic disease due to mutations in the AIRE (AutoImmune REgulator) gene. A 40 year old female patient who suffers from the above mentioned condition was admitted to the hospital due to ureterolithiasis and hydronephrosis of the left kidney. When a patient suffers from an autoimmune disease such as APECED syndrome, preoperative preparation and assessment is of key importance in inducing and maintaining safe anaesthesia. Regarding the major role that the adrenal gland plays in adaptive response to stress, a careful evaluation of corticoid therapy, both chronic and supplemental, was performed. In patients with primary adrenal insufficiency, the major clinical features of adrenal insufficiency are consequences of cortisol deficiency and also volume depletion and hypotension, resulting mainly from mineralocorticoid deficiency as well. Therefore, when patients with impaired hypothalamo-pituitary-adrenal (HPA) axis undergo a surgical procedure, a state of enhanced stress, preoperative preparation must be done in terms of administration of high doses of hydrocortisone to enable the patients organism to cope with very high stress as surgical stimuli as well as maintaining euvolemic and electrolyte balance.
This case has shown that comprehensive preoperative preparation and assessment of patients with primary immune deficiencies can minimize surgical complications and optimize patient outcomes. It is of key importance to predict and address potential metabolic disturbances when people with this or similar conditions that include impairment in HPA axis undergo surgical procedure because lack of preoperative optimization can lead to severe complications during surgery as well as in postoperative
period.
Keywords
APECED; autoimmune disease; anaesthesia; preoperative
Hrčak ID:
312751
URI
Publication date:
22.12.2023.
Visits: 634 *