ASY-857.1: Synacthen Stimulated 17OH-progesterone ASY-857.2: Associated Documents a Synacthen Standing Order form (ref 0827/2) G:\Division\NDO\common\ETCProtocols\0827 Standing Order Synacthen 2016.pdf ASY-857.3: Distribution of Documents Copy No Number Location 1 Quality Centre 2 3.1 Endocrine Centre 3 G:\Division\NDO\common\ETCProtocols\0857 Synacthen Stimulated 17OH Progesterone test.doc ASY-857.4: Review of Document: Date Signature Next review Sign when read G:\Division\NDO\common\ETCProtocols\0857 Synacthen Stimulated 17OH Progesterone test.doc Do not photocopy Document number: Page 1 of 5
ASY-857.5: Synacthen Stimulated 17OH-progesterone ASY-825.5.1: Purpose of Synacthen Stimulated 17OH-progesterone For investigation of congenital adrenal hyperplasia (CAH) in adults. Adrenal glucocorticoid secretion is controlled by adrenocorticotrophic hormone (ACTH) released by the anterior pituitary. This test evaluates the ability of the adrenal cortex to produce cortisol after stimulation by synthetic ACTH (tetracosactide: Synacthen). In subjects with enzyme deficiency in the steroid synthetic pathway, cortisol may, or may not, be adequately secreted. However, there is excessive secretion of the precursor steroids before the defective enzyme. The commonest form of CAH is due to deficiency of 21-hydroxylase and in these subjects increased secretion of 17 OH-progesterone can be detected. ASY-825.5.2: Documentation a Inpatient: synacthen 0.25mg IV/IM to be charted on medication chart b Outpatient: standing order request form ASY-825.5.3: Patient preparation a There are no dietary restrictions for this test. b This test should be performed in the morning as there may be diurnal variations in 17 OH-progesterone. c In females this test should be performed in the follicular (preovulatory) phase of the menstrual cycle (to reduce false positives, as luteal phase 17OH-progesterone is 2 to 3 times higher than in the follicular phase). The follicular phase starts on the first day of menstruation and lasts 13 days. d Hydrocortisone should be omitted on the morning of the test. e Prednisolone should be stopped 24 hours before the test and recommenced after (unless instructed to the contrary). ASY-825.5.4: Procedure a This is a 1 hour test. b Place an IV cannula. c Take blood for 17OHprogesterone, cortisol and ACTH at baseline (pre-synacthen), and write the time on the tube (e.g. 0800h) d Check patient details with request form / medication chart. e Inject 0.25 mg synacthen IM (usually into deltoid) or IV via cannula, slowly, over 2 minutes. f If giving synacthen IV, flush the line with 5ml sodium chloride 0.9%. g Before taking 60 minute post-synacthen blood, withdraw 3ml from IV line and discard. h Take blood for 17OHprogesterone and cortisol at 60 minutes post-synacthen. Label tube with time (e.g. 0900h) and post synacthen. Do not photocopy Document number: Page 2 of 5
ASY-857.6: Interpretation ASY-825.6.1: Cortisol interpretation a A post-synacthen cortisol of <400 nmol/l (on the Roche assay) is abnormal. Endocrine review is recommended. b A post-synacthen Cortisol > 400 nmol/l suggests normal adrenocortical reserve and makes both primary and steroid-induced adrenal insufficiency extremely unlikely. However, some patients with partial or recent onset ACTH insufficiency may have a normal Synacthen response. If taking OCP, a post-synacthen cortisol below 600 nmol/l suggests adrenal insufficiency. If clinical suspicion remains high discussion with an Endocrinologist is recommended, and a short metyrapone test or insulin tolerance test may help. c The basal 0800 cortisol reference range is 170-500nmol/L. ASY-825.6.2: 17OH Progesterone interpretation a There are marked variations in 17 OH-progesterone throughout the menstrual cycle and "normal" values cannot exclude non-classical CAH. b Heterozygotes for 21 OH-deficiency should have post-acth values of 17 OHprogesterone > 35 nmol/l (guide value). There is an overlap with normals and this test is not diagnostic. c Homozygotes for non-classical CAH may have normal baseline values for 17 OHprogesterone but will have exaggerated responses to ACTH of > 60 nmol/l (guide value) d Rarely, stimulated values at 60 minutes in affected patients range between 30 nmol/l and 43 nmol/l, and this range overlaps with the carrier state; thus, genotyping may be useful if stimulated values are in this range. Do not photocopy Document number: Page 3 of 5
NB 1000ng/dL = 30nmol/L, 10000 ng/dl = 303 nmol/l Figure 1. 17-OHP nomogram for the diagnosis of steroid 21-hydroxylase deficiency (60- minute Cortrosyn stimulation test). The data for this nomogram was collected between 1982 and 1991 at the Department of Pediatrics, the New York Hospital-Cornell Medical Center, New York 1 ASY-857.7: References 1) 21-hydoxylase-Deficient Congenital Adrenal Hyperplasia Pagon RA, Adam MP, Ardinger HH, et al., editors. Seattle (WA): University of Washington, Seattle; 1993-2017. 2) L Nieman, P Merke, A Lacroix, K Martin, Diagnosis and treatment of nonclassic (lateonset) congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Uptodate. Accessed 25th January 2017 3) http://www.pathology.leedsth.nhs.uk/dnn_bilm/investigationprotocols/synacthentestsshortlon g/shortsynacthenforcongenitaladrenalh.aspx Accessed 25th January 2017 Do not photocopy Document number: Page 4 of 5
Patient information Synacthen Stimulated 17OH-progesterone The Synacthen stimulated 17OH Progesterone test assesses the ability of your adrenal glands to make the steroid hormone cortisol. Synacthen is a synthetic form of ACTH (adrenocorticotropic hormone), which is a hormone naturally produced in your body to stimulate your adrenal glands to make cortisol. Some people have a problem in manufacturing cortisol and may instead make larger amounts of different steroids such as 17OH progesterone. There are a variety of these conditions, some of which are called congenital adrenal hyperplasia, which is the main group of conditions that this test aims to help identify. The ideal time to perform this test is between 0800-1000 in the morning. This is done at Christchurch Hospital in the Endocrine s Centre located on the 2 nd Floor of the Riverside Block, next to Ward 26. Please ring 364 0934 to make an appointment Preparation: You may eat and drink as normal on the morning of the test and you may take all your normal medication except those listed below. Take the last dose of your steroid medication in the morning the day before the test. Medication to avoid prior to the test: Hydrocortisone Dexamethasone Prednisone Cortisone These medications are all steroids and should be omitted preferably for 24 hours before the test (but for at least 8 hours). If you are unsure whether you are able to omit these medications, check with your doctor first. Please bring your steroid medication with you as you can take your normal steroid dose immediately after the test has been completed. Procedure: A cannula (small plastic tube) is inserted into a vein in your arm. This reduces patient discomfort as the blood samples can be taken and the synacthen can be given via the cannula. If it is not possible to insert a cannula, blood samples are obtained as usual and synacthen is given by injection into your arm muscle. A 4ml blood sample is obtained. Synacthen hormone is given. Another 4ml blood sample is obtained 60 minutes later. Risks The test is performed on a daily basis in the Endocrine Department. Occasionally some patients experience adverse reactions such as minor pain or bruising at the injection site; nausea; vomiting and flushing. As with any medication there is a small risk of an allergic reaction. If you have previously had a synacthen test and had a bad reaction, please inform the doctor or nurse before the test is repeated. Parking Particularly since the earthquakes, parking is often difficult. Please allow plenty of time to either find a Pay&Display space, or to use the Park and Ride facilities. Issue date: 28 August 2017 Document number: Page 5 of 5