{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,26]],"date-time":"2025-10-26T20:54:10Z","timestamp":1761512050717},"reference-count":53,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2005,1,17]],"date-time":"2005-01-17T00:00:00Z","timestamp":1105920000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinical Endocrinology"],"published-print":{"date-parts":[[2005,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p><jats:bold>Objective\u2002<\/jats:bold> Congenital ACTH insufficiency due to disorders of the hypothalamo\u2013pituitary axis is life threatening. However, the optimal method for establishing the diagnosis remains controversial. The standard Synacthen test (SST) is safe and easy to perform in infancy, but its performance has not been well evaluated in infants and children with ACTH insufficiency.<\/jats:p><jats:p><jats:bold>Design, Patients and Measurements\u2002<\/jats:bold> We aimed to determine the value of the SST by comparing the 30\u2010min SST cortisol concentration with physiological (spontaneous) cortisol secretion (2\u2010hourly measurements for 24\u00a0h) in 28 patients (13 male; age range 0\u20135\u00b71\u00a0years) with one or a combination of optic nerve (25) midline forebrain (17) and other pituitary hormone (17) abnormalities. Spontaneous cortisol secretion (mean &lt;\u00a0145\u00a0nmol\/l) was arbitrarily used as the \u2018gold\u2010standard\u2019 in the diagnosis of ACTH insufficiency as it offered a reflection of endogenous cortisol mileu. A normal SST was defined as a 30\u2010min serum cortisol &gt;\u00a0540\u00a0nmol\/l.<\/jats:p><jats:p><jats:bold>Results\u2002<\/jats:bold> The SST basal, 30\u2010min and 60\u2010min serum cortisol concentrations were significantly (<jats:italic>P\u00a0&lt;<\/jats:italic>\u00a00\u00b7001) lower in patients with ACTH insufficiency (mean spontaneous cortisol &lt;\u00a0145\u00a0nmol\/l). 3\/13 patients without and 12\/15 patients with ACTH insufficiency failed the SST (specificity 76\u00b79%; sensitivity 80%). Two out of three patients with ACTH insufficiency who passed the test were symptomatic with fatigue, poor growth and hypoglycaemia that resolved with hydrocortisone treatment. An 08:00\u00a0h serum cortisol cut\u2010off of 175\u00a0nmol\/l provided a sensitivity of 93\u00b73% and a specificity of 92\u00b73% as compared with spontaneous cortisol secretion. The 30\u2010min increment and the 60\u2010min SST cortisol concentrations although highly specific (100%) had reduced sensitivity (40% and 60%, respectively).<\/jats:p><jats:p><jats:bold>Conclusions\u2002<\/jats:bold> A normal SST does not rule out ACTH insufficiency and measurement of physiological cortisol secretion may be required to confirm the diagnosis. A combination of 08:00\u00a0h serum cortisol &gt;\u00a0175\u00a0nmol\/l and a 30\u2010min SST serum cortisol &gt;\u00a0540\u00a0nmol\/l to exclude ACTH insufficiency (sensitivity 69%, specificity 100%) would ensure that no child with ACTH insufficiency would be missed and hence would prevent serious consequences, although it may lead to over\u2010diagnosis in some cases.<\/jats:p>","DOI":"10.1111\/j.1365-2265.2005.02215.x","type":"journal-article","created":{"date-parts":[[2005,2,24]],"date-time":"2005-02-24T10:39:36Z","timestamp":1109241576000},"page":"307-314","source":"Crossref","is-referenced-by-count":21,"title":["An update on the biochemical diagnosis of congenital ACTH insufficiency"],"prefix":"10.1111","volume":"62","author":[{"given":"Ameeta","family":"Mehta","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter 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