No central adrenal insufficiency found in patients with Prader-Willi syndrome with an overnight metyrapone test

2018 ◽  
Vol 31 (7) ◽  
pp. 809-814 ◽  
Author(s):  
Kathryn S. Obrynba ◽  
Robert P. Hoffman ◽  
David R. Repaske ◽  
Kathryn Anglin ◽  
Manmohan K. Kamboj
Keyword(s):  
Adrenal Insufficiency ◽  
Low Dose ◽  
False Positive Rate ◽  
Standard Test ◽  
Prader Willi Syndrome ◽  
Acth Stimulation Test ◽  
Acth Stimulation ◽  
Positive Rate ◽  
Central Adrenal Insufficiency ◽  
Method Of Assessment

Abstract Background Individuals with Prader-Willi syndrome (PWS) have hypothalamic dysfunction and may have central adrenal insufficiency (CAI). The prevalence of CAI in PWS remains unknown. Methods Twenty-one subjects with PWS aged 4–53 years underwent a low dose adrenocorticotropic hormone (ACTH) stimulation test (LDAST) (1 μg/m2, maximum 1 μg) followed by an overnight metyrapone test (OMT). Metyrapone (30 mg/kg, maximum 3 g) was administered at 2400 h. Cortisol, 11-deoxycortisol (11-DOC) and ACTH levels were collected the following morning at 0800 h. OMT was the standard test for comparison. Peak cortisol ≥15.5 μg/dL (427.6 nmol/L) on LDAST and 0800 h 11-DOC ≥7 μg/dL (200 nmol/L) on OMT were classified as adrenal sufficiency. Results Twenty subjects had 0800 h 11-DOC values ≥7 μg/dL on OMT indicating adrenal sufficiency. One subject had an inconclusive OMT result. Six of the 21 (29%) subjects had peak cortisol <15.5 μg/dL on LDAST. Conclusions We found no evidence of CAI based on OMT, yet 29% of our PWS population failed the LDAST. This suggests that the LDAST may have a high false positive rate in diagnosing CAI in individuals with PWS. OMT may be the preferred method of assessment for CAI in patients with PWS.

scholarly journals Multidrug-Resistant Tuberculosis and Its Association with Adrenal Insufficiency: Assessment with the Low-Dose ACTH Stimulation Test

2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
René Rodríguez-Gutiérrez ◽  
Adrian Rendon ◽  
Maximiliano Barrera-Sánchez ◽  
Kevin Erick Gabriel Carlos-Reyna ◽  
Neri Alejandro Álvarez-Villalobos ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Low Dose ◽  
Multidrug Resistant ◽  
Stimulation Test ◽  
Acth Stimulation Test ◽  
Acth Stimulation ◽  
Resistant Tuberculosis ◽  
Multidrug Resistant Tuberculosis ◽  
Antituberculosis Treatment ◽  
Mdr Tb

Background. Multidrug-resistant tuberculosis (MDR-TB) is a major public health care concern that affects the life of millions of people around the world. The association of tuberculosis and adrenal insufficiency is well known; however, it is thought to be less prevalent every time. A spike in TB incidence and a lack of evidence of this association in patients with MDR-TB call for reassessment of an illness (adrenal dysfunction) that if not diagnosed could seriously jeopardize patients’ health.Objective. To determine the prevalence of adrenocortical insufficiency in patients with MDR-TB using the low-dose (1 μg) ACTH stimulation test at baseline and at 6–12 months of follow-up after antituberculosis treatment and culture conversion.Methods. A total of 48 men or women, aged ≥18 years (HIV-negative patients diagnosed with pulmonary MDR-TB) were included in this prospective observational study. Blood samples for serum cortisol were taken at baseline and 30 and 60 minutes after 1 μg ACTH stimulation at our tertiary level university hospital before and after antituberculosis treatment.Results. Forty-seven percent of subjects had primary MDR-TB; 43.8% had type 2 diabetes; none were HIV-positive. We found at enrollment 2 cases (4.2%) of adrenal insufficiency taking 500 nmol/L as the standard cutoff point value and 4 cases (8.3%) alternatively, using 550 nmol/L. After antituberculosis intensive phase drug-treatment and a negative mycobacterial culture (10.2±3.6months) adrenocortical function was restored in all cases.Conclusions. In patients with MDR-TB, using the low-dose ACTH stimulation test, a low prevalence of mild adrenal insufficiency was observed. After antituberculosis treatment adrenal function was restored in all cases. Given the increasing and worrying epidemic of MDR-TB these findings have important clinical implications that may help clinicians and patients make better decisions when deciding to test for adrenocortical dysfunction or treat insufficient stimulated cortisol levels in the setting of MDR-TB.

scholarly journals Accuracy of the Low-Dose ACTH Stimulation Test for Adrenal Insufficiency Diagnosis: A Re-Assessment of the Cut-Off Value

2019 ◽  
Vol 8 (6) ◽  
pp. 806 ◽  
Author(s):  
Laura Maria Mongioì ◽  
Rosita Angela Condorelli ◽  
Federica Barbagallo ◽  
Rossella Cannarella ◽  
Sandro La Vignera ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Roc Curve ◽  
False Positive ◽  
Low Dose ◽  
Stimulation Test ◽  
High Rate ◽  
Acth Stimulation Test ◽  
Roc Curve Analysis ◽  
Acth Stimulation ◽  
Predictive Values

Background: The clinical practice shows that many low-dose ACTH-stimulation tests have a false positive result. The aim of the study was to determine the diagnostic accuracy of a low-dose ACTH-stimulation test in the diagnosis of adrenal insufficiency and to define its optimal cut-off. Methods: We analyzed data from 103 patients undergoing 1 µg ACTH-stimulation test. Four patients had adrenal insufficiency (AI) upon follow up: Two primary, and two secondary AI. Cortisol serum levels were evaluated at time 0, 20’, and 30’ after the injection of 1 µg i.v. of ACTH. The sensitivity, specificity, accuracy, and positive and negative predictive values of the test were calculated for both 20’ and 30’ sampling. The receiver operating characteristic (ROC) curve was obtained to assess the sensitivity and specificity of low-dose ACTH-stimulation test in the diagnosis of adrenal insufficiency at different cut-off values. Results: Considering 500 nmol/L as the standard cut-off value, low-dose ACTH stimulation test showed a 100% sensitivity and a 67.3% specificity, with a high rate of false positive results. ROC curve analysis showed that the cut-off of 401.5 nmol/L is the best compromise between sensitivity (100%) and specificity (93.9%). Conclusions: By using a cut-off value of 401.5 nmol/L for the low-dose ACTH stimulation test, the number of false positive patients decreased significantly, but the sensitivity remained high.

scholarly journals THE ICET-A SURVEY ON CURRENT CRITERIA USED BY CLINICIANS FOR THE ASSESSMENT OF CENTRAL ADRENAL INSUFFICIENCY IN THALASSEMIA: ANALYSIS OF RESULTS AND RECOMMENDATIONS

2016 ◽  
Vol 8 ◽  
pp. 2016034
Author(s):  
Vincenzo De Sanctis
Keyword(s):  
Adrenal Insufficiency ◽  
Predictive Value ◽  
Serum Cortisol ◽  
Stimulation Test ◽  
Serum Cortisol Level ◽  
Second Step ◽  
Acth Stimulation Test ◽  
Acth Stimulation ◽  
Basal Serum ◽  
Central Adrenal Insufficiency

Introduction: In March 2015, the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) implemented a two-step survey on central adrenal insufficiency (CAI) assessment in TM patients and after analysis of the collected data,  recommendations for the assessment of hypothalamic-pituitary- adrenal (HPA) axis in clinical practice were defined.Methods: To ascertain the current practice for assessment of CAI in thalassemia, the Coordinator of ICET-A sent two questionnaires by email: i) The first to evaluate the current interpretation of basal serum cortisol level (first step) and ii) The second to evaluate the current usage of ACTH test and the variability in practice" (second step). Based on the surveys the core ICET-A group prepared the recommendations for the assessment of suspected CAI in thalassemia (third step).Results: A total of 19 thalassemologists/endocrinologists have participated in the first survey and 35 specialists participated in the second step questionnaire. The survey demonstrated a considerable variability in almost all aspects of relevant current criteria used for the diagnosis of CAI.  A ROC analysis using peak value > 20 μg/dl (> 550 nmol/L), after ACTH stimulation test, was performed with the aim of identifying the optimal basal serum cortisol cut-off. The optimal threshold that maximizes sensitivity plus specificity for morning basal cortisol against peak post-ACTH value >20 μg/dl (>550 nmol/L) was 10 μg/dl (275 nmol/L).,Furthermore, the values associated with highest negative predictive value (NPV) and highest, positive predictive value (PPV) were 4.20 (115 nmol/L) and 18.45 μg/dl (510 nmol/L), respectively.Surprisingly, 20 specialists in thalassemia working in blood bank, thalassemia centres (day hospital), internal medicine, hematology and onco-hematology had poor knowledge and experience in testing for CAI and stopped filling the questionnaire after the second question. In contrast, 9 endocrinologists (8 pediatricians) and 6 hematologists working in collaboration with endocrinologists completed the questionnaire.Conclusions: While waiting more extensive adequately powered and targeted studies, physicians should adopt an acceptable policy for accurate assessment of HPA in TM patients. Regular surveillance, early diagnosis, treatment and follow-up in a multi-disciplinary specialized setting are also recommended. The ICET-A recommendations are reported in order to facilitate for interested physicians the approach to successful assessment of adrenal function in thalassemia. Key words: Thalassemia, adrenal insufficiency, pitfall in the diagnosis, ACTH stimulation test, guidelines.

No central adrenal insufficiency found in adults with prader-willi syndrome tested by multiple dose metyrapone test

2019 ◽  
Author(s):  
Anna Rosenberg ◽  
Karlijn Pellikaan ◽  
Kirsten Davidse ◽  
Stephany Donze ◽  
Anita Hokken-Koelega ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Multiple Dose ◽  
Prader Willi Syndrome ◽  
Central Adrenal Insufficiency

Cortisol Response to Low-Dose ACTH Stimulation Test in Non-Stressed Pediatric Patients

2011 ◽  
pp. P2-741-P2-741
Author(s):  
Joran Sequeira ◽  
Richard A Noto ◽  
Qiuhu Shi ◽  
Mamatha Sandu ◽  
Figen Altunkaya ◽  
...  
Keyword(s):  
Low Dose ◽  
Pediatric Patients ◽  
Stimulation Test ◽  
Cortisol Response ◽  
Acth Stimulation Test ◽  
Acth Stimulation

scholarly journals Low-dose ACTH test for the diagnosis of non-classical form of congenital adrenocortical dysfunction

2010 ◽  
Vol 56 (2) ◽  
pp. 10-14
Author(s):  
N B Chagaĭ ◽  
V V Fadeev ◽  
E G Bakulina
Keyword(s):  
Low Dose ◽  
Stimulation Test ◽  
Acth Test ◽  
Acth Stimulation Test ◽  
Acth Stimulation ◽  
Hydroxylase Deficiency ◽  
Classical Form ◽  
21 Hydroxylase Deficiency

The possibilities to diagnose the non-classical form of 21-hydroxylase deficiency using the low-dose (5 mcg) 1-24 ACTH stimulation test are considered.

scholarly journals OR25-04 Over 14% of Inpatients with Euvolemic Hyponatremia Have Undiagnosed Adrenal Insufficiency

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Amit Kumar ◽  
Maria Ghosh ◽  
Jubbin Jagan Jacob
Keyword(s):  
Adrenal Insufficiency ◽  
Hormone Secretion ◽  
Stimulation Test ◽  
Acth Stimulation Test ◽  
Sheehan’S Syndrome ◽  
Acth Stimulation ◽  
Steroid Use ◽  
Inappropriate Antidiuretic Hormone Secretion ◽  
Inhaled Steroids ◽  
Sheehan's Syndrome

Abstract Background- The commonest cause of euvolemic hyponatremia (EvHNa) is the syndrome of inappropriate antidiuretic hormone secretion (SIADH). The diagnosis of SIADH requires the exclusion of secondary adrenal insufficiency (AI) and untreated hypothyroidism. Studies have suggested about 4% of unselected patients presenting to the emergency room with EvHNa have undiagnosed SAI.1 Among patients admitted to specialized endocrine units this prevalence maybe as high as 20%.2Objective- To study the prevalence of undiagnosed AI among inpatients with EvHNa admitted to general medical wards. Methods- This was a prospective, single centre observational study conducted among inpatients with EvHNa. EvHNa was defined as patients with a serum sodium (Na) &lt;135 mEq/L, with no clinical evidence of dehydration or fluid excess, and a urine spot Na &gt;30mmol/L. In addition patients with recent vomiting, renal failure, recent diuretic use, uncontrolled hyperglycemia and patients with history of use of oral or parenteral steroids in the last 6 months were excluded. Adrenal functions were assessed by a modified porcine ACTH stimulation test which has been described recently by Nair et al. A cut off cortisol value of &lt;18mg/dl after 60 minutes of ACTH injection was used to diagnose AI.3Results- One hundred and forty one (141) patients were included after informed consent and all underwent a modified ACTH stimulation test. They had a mean age of 58 years and 52.3% (n=74) were males. Modified ACTH stimulation testing suggested 20/141 (14.2%) had undiagnosed AI. The mean age among those with AI was 55.2 years. In only 25% (5/20) AI was suspected based on clinical presentation by the treating physician. Despite excluding patients with documented steroid use, the commonest cause of AI (9/20) was secondary AI due to exogenous steroid use including high potency inhaled steroids (5/9) and the use of undocumented steroids or steroid containing medicaments by alternative practitioners (4/9). Hypopituitarism was diagnosed as the cause of AI in 5 patients, which included unsuspected Sheehan’s syndrome in post menopausal women (3/5), non functioning pituitary adenoma (1/5) and lymphocytic hypophysitis (1/5). Despite primary AI not commonly presenting as EvHNa, 3/20 patients had primary AI and in the remaining 3 patients the aetiology of AI remained unclear. Conclusions- Undiagnosed AI is much more common in our country among inpatients presenting with EvHNa to medical units. This increase is primarily driven by inhaled and undocumented exogenous steroid use and undiagnosed Sheehan’s syndrome. An assessment of the hypothalamic-pituitary-adrenal axis is mandatory before making a diagnosis of SIADH. References -(1) Diederich et al. Eur J Endocrinol 2003; 148: 609-617. (2) Cuesta et al. Clin Endocrinol (Oxf) 2016; 85: 836-844. (3) Nair A et al. Eur J Endocrinol. 2019 Oct 1. pii: EJE-19-0558.R2.

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