The 4Ds of ectopic ACTH syndrome: diagnostic dilemmas of a difficult disease
Arch. endocrinol. metab. (Online)
;
63(2): 175-181, Mar.-Apr. 2019. tab, graf
Article
in English
| LILACS
| ID: biblio-1001221
ABSTRACT
SUMMARY Cushing's syndrome (CS) is an uncommon condition that leads to high morbidity and mortality. The majority of endogenous CS is caused by excessive ACTH secretion, mainly due to a pituitary tumor - the so-called Cushing's disease (CD) - followed by ectopic ACTH syndrome (EAS), an extra-pituitary tumor that produces ACTH; adrenal causes of CS are even rarer. Several methods are used to differentiate the two main etiologies specific laboratory tests and imaging procedures, and bilateral inferior petrosal sinus sampling (BIPSS) for ACTH determination; however, identification of the source of ACTH overproduction is often a challenge. We report the case of a 28-year-old woman with clinical and laboratory findings consistent with ACTH-dependent CS. All tests were mostly definite, but several confounding factors provoked an extended delay in identifying the origin of ACTH secretion, prompting a worsening of her clinical condition, with difficulty controlling hyperglycemia, hypokalemia, and hypertension. During this period, clinical treatment was decisive, and measurement of morning salivary cortisol was a differential for monitoring cortisol levels. This report shows that clinical reasoning, experience and use of recent methods of nuclear medicine were decisive for the elucidation of the case.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
ACTH Syndrome, Ectopic
/
Carcinoma, Neuroendocrine
/
Lung Neoplasms
Type of study:
Diagnostic study
/
Etiology study
/
Prognostic study
Limits:
Adult
/
Female
/
Humans
Language:
English
Journal:
Arch. endocrinol. metab. (Online)
Journal subject:
Endocrinology
/
Metabolism
Year:
2019
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Universidade Federal de São Paulo/BR
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