scholarly journals THE FREQUENCY OF ADRENAL INSUFFICIENCY IN ADOLESCENTS AND YOUNG ADULTS WITH THALASSEMIA MAJOR VERSUS THALASSEMIA INTERMEDIA IN IRAN

2014 ◽  
Vol 7 ◽  
pp. e2015005 ◽  
Author(s):  
Sara Matin ◽  
Masoud Ghanei Jahromi ◽  
Zohreh Karemizadeh ◽  
Sezaneh Haghpanah ◽  
Vincenzo De Sanctis ◽  
...  

Background: Endocrine dysfunction is not uncommon complication in patients with transfusion dependent thalassemia and is thought to occur as a consequence of excessive iron overload. The main objective of this study is to determine the frequency of adrenal insufficiency in patients with thalassemia major and thalassemia intermediate. Methods: This cross-sectional study was done at the Shiraz University of Medical Sciences, Shiraz, Southern Iran, in 2013. One hundred and ninety patients were divided into two groups; thalassemia major(TM) and thalassemia intermediate (TI) groups. We measured 8 AM serum cortisol, ACTH and ferritin concentrations in all patients. Results: The mean age of the TM and TI group were 22.5±5.7 and 23.8±6 years, respectively. 90 patients (47.4%) were splenectomized,  34 (36.2%) with TM and  56 (58.2%) with TI (p : 0.001). The mean serum ferritin levels were 3056.5±2306 and 666.2±616.5 in TM and TI respectively (p: 0.001).  Three patients with TM (1.6%) had low basal cortisol and ACTH levels. However their cortisol response to ACTH stimulation was normal. Conclusions: Low basal concentrations of cortisol and ACTH occurred in 1.6% of our adolescents young adult patients with TM suggesting a central defect of cortisol secretion at the basal state. However, cortisol response to standard – dose ACTH was normal in all patients with TM and TI.

2021 ◽  
Author(s):  
Muhammet Cuneyt Bilginer ◽  
Abbas Ali Tam ◽  
Sevgul Faki ◽  
Nagihan Bestepe ◽  
Fatma Dilek Dellal ◽  
...  

Abstract Background Patients with differentiated thyroid cancer (DTC) are exposed to subclinical exogenous hyperthyroidism for the suppression of thyroid-stimulating hormone (TSH). In this study, we aimed to evaluate the adrenal reserve in DTC patients receiving suppression therapy. Materials and Methods The study included 55 DTC patients on suppression therapy and 32 healthy volunteers. Basal serum cortisol of all participants and adrenocorticotropic hormone (ACTH) of the patient group were measured. A standard-dose ACTH test (0.25 mg) was performed in patients with a basal cortisol < 14.5 mcg/dL. Results In the patient group, TSH was lower, free thyroxine (fT4) was higher, and free triiodothyronine (fT3) was similar to those of the control group (p < 0.01, p < 0.01, p = 0.140, respectively). The serum cortisol of the patient group was significantly lower than the control group (12.14 ± 5.12 mcg/dL vs 18.00 ± 5.56 mcg/dL, p < 0.001). 34 (61.8%) patients with DTC had a basal cortisol < 14.5 mcg/dL. Prolonged TSH suppression (≥ 5 years vs < 5 years) was associated with lower basal cortisol (7.46 ± 2.63 mcg/dL vs 9.48 ± 2.65 mcg/dL, p = 0.022). The ACTH stimulation test showed that 2 (5.8%) patients had a cortisol response < 18 mcg/dL. The rate of adrenal insufficiency was 3.6% in DTC patients. A moderate negative correlation was found between ACTH and fT3 of patients with low basal cortisol (r = − 0.358, p = 0.038) Conclusion Patients with DTC receiving TSH suppression therapy are at risk for adrenal insufficiency. The duration and severity of suppression might increase this possibility. Dynamic testing with synthetic ACTH can be used to reveal insufficient cortisol response in case of clinical suspicion.


1993 ◽  
Vol 136 (1) ◽  
pp. 167-172 ◽  
Author(s):  
S. Crowley ◽  
P. C. Hindmarsh ◽  
J. W. Honour ◽  
C. G. D. Brook

ABSTRACT We compared the reproducibility and repeatability of the acute adrenal response to low doses (90 and 500 ng/1·73 m2) of Synacthen (ACTH(1–24)) with that of the standard dose (250 μg/1·73 m2). We also examined the effect of basal cortisol levels on peak values achieved after stimulation with a low dose. ACTH(1–24) was given to six male volunteers: 90 ng/1·73 m2 twice at 90-min intervals on day 1, and 90 and 500 ng/1·73 m2 once on day 2 and 250 μg/1·73 m2 once on day 3. The rise in serum cortisol concentration with repeated low doses of ACTH was not attenuated (161 ± 49 (s.d.) nmol/l on initial vs 150 ± 41 nmol/l on repeat stimulation; P = 0·5) and this was reproducible (161 ± 49 nmol/l on day 1 vs 148 ± 15 nmol/l on day 2; P = 0·6). A dose of 500 ng ACTH(1–24)/1·73 m2 produced a maximal adrenal response in that the rise in serum cortisol concentration at 20 min was identical with that produced at the same time by the standard dose of 250 μg/1·73 m2. There was a strong positive correlation between the basal cortisol level and peak cortisol concentration after low-dose ACTH stimulation (r = 0·93, P < 0·001) but not between the basal cortisol level and the incremental rise (r= −0·1, P = 0·69). These results suggest that the cortisol response to low-dose ACTH stimulation is reproducible and not attenuated by repeat stimulation at 90-min intervals. The incremental rise in serum cortisol concentration after ACTH stimulation appears constant in these situations and is not influenced by the basal cortisol level. When there is concern that the standard dose may be excessive and mask subtle but important changes in adrenal function, the low dose (500 ng) of ACTH should be used. Journal of Endocrinology (1993) 136, 167–172


1997 ◽  
Vol 82 (3) ◽  
pp. 856-860
Author(s):  
Samer S. El-Deiry ◽  
Sakkubai Naidu ◽  
Lewis S. Blevins ◽  
Paul W. Ladenson

Abstract Adrenoleukodystrophy (ALD) is an X-linked recessive disorder that destroys the white matter of the brain and is associated with adrenal insufficiency. The prevalence of adrenal dysfunction in 71 women carriers of the X-linked ALD gene was studied. These subjects were identified initially on the basis of being obligate carriers of the X-linked trait by pedigree analysis and were confirmed by plasma very long chain fatty acid levels consistent with a heterozygote status. One subject had well documented overt adrenal insufficiency, diagnosed and treated since age 9 yr. Among the remaining women, the mean serum 0800 h and 1 h post-ACTH cortisol concentrations [16 ± 7 (±sd) and 34 ± 8 μg/dL, respectively] were normal. All subjects had normal ACTH-stimulated serum cortisol levels, i.e. more than 20 μg/dL. However, 4 subjects (6%) had subnormal ACTH-stimulated aldosterone concentrations (mean, 9 ± 6 vs. 42 ± 16 ng/dL for other subjects; P = 0.001, by Mann Whitney rank sum test). Three of these women (75%) were taking nonsteroidal antiinflammatory agents (NSAIDs), whereas only 4 of 67 (6%) subjects with normal aldosterone responsiveness were NSAIDs users (P &lt; 0.01, by Fisher’s exact test). Thus, NSAIDs use was associated with increased risk of hypoaldosteronism (odds ratio, 50.2; 95% confidence interval, 3.3–266; P &lt; 0.002). Three of these four women had symptoms consistent with mineralocorticoid deficiency. Serum sodium and potassium concentrations were normal in all subjects. Basal and metyrapone-stimulated plasma ACTH concentrations were also normal in adequately tested subjects with and without mineralocorticoid insufficiency. Five of eight subjects (63%) who underwent testing with synthetic ovine CRH (oCRH) had abnormalities. Three did not meet the criteria for adequate cortisol stimulation (i.e. &gt;20 μg/dL) and had peak ACTH levels greater than 30 pg/mL. Two other subjects had exaggerated ACTH responses with normal cortisol levels. There were no significant differences in the mean or median levels of very long chain fatty acid, C26:0, C24/22 ratios, or C26/22 ratios among the entire subject group, the subgroup with blunted aldosterone responses to ACTH, and the subgroup with blunted responses to oCRH (P &gt; 0.05, by ANOVA and Kruskall-Wallis test for C26, C24/22 ratio, and C26/22 ratio). We conclude that 1) adrenal cortical insufficiency rarely develops in ALD heterozygotes; 2) isolated mineralocorticoid insufficiency can occur in ALD heterozygotes, as has been previously reported to occur with autoimmune and acquired immunodeficiency syndrome-related adrenal dysfunction; 3) ALD heterozygosity may predispose these individuals to NSAID-related hypoaldosteronism; and 4) a subclinical decrease in glucocorticoid reserve, as measured by oCRH testing, may be present in a majority of these women. Aldosterone levels should be included in the ACTH stimulation testing when seeking evidence of adrenal insufficiency in affected women. NSAIDs should be considered a risk factor for the development of hypoaldosteronism in women heterozygous for ALD.


2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Q. Cui ◽  
D. Liu ◽  
B. Xiang ◽  
Q. Sun ◽  
L. Fan ◽  
...  

Background. The suppressed hypothalamic-pituitary-adrenal (HPA) axis after successful surgery for Cushing’s disease (CD) will recover in almost all patients. We aimed to identify the predictive factors for HPA axis recovery in CD patients with postoperative remission. Design and Methods. This observational retrospective cross-sectional study enrolled 69 CD patients with postoperative remission in Huashan Hospital from 2015 to 2019. All subjects had a detailed clinical evaluation. The low-dose ACTH stimulation test (LDT) was conducted as the gold standard for assessing the HPA axis function. Results. Peak cortisol in LDT was found only to be positively correlative with morning serum cortisol (MSC) ( ρ = 0.451 , p < 0.001 ). The MSC was higher ( p < 0.001 ), and the median postoperative course was significantly longer ( p = 0.025 ) in the patients with the recovered HPA axis function compared with unrecovered patients. The AUC value of MSC for predicting the recovery of the HPA axis was 0.701, and the optimal cutoff was 6.25 μg/dl (sensitivity 85.19% and specificity 47.62%). Other useful cutoff values were 10.74 μg/dl (specificity 100%) and 4.18 μg/dl (sensitivity 100%). Besides, combined with the postoperative course, the AUC values were higher than MSC alone (0.935 vs. 0.701, p < 0.001 ). Conclusions. MSC is a viable first-step diagnostic predictor for HPA axis recovery in CD patients with postoperative remission. For the patients with cortisol levels between 4.18 and 10.74 μg/dl, a confirmatory test should be conducted. When the MSC level was 10.74 μg/dl or greater, the replacement therapy could be discontinued.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Muhammad Imran Butt ◽  
Lama Ghassan Amer ◽  
Hadeel Salah Aljamei ◽  
Muhammad Riazuddin ◽  
Nouf AlZuhairy ◽  
...  

Abstract Background SST is done to assess adrenal gland function by measuring basal serum cortisol followed by injecting 250 μg of Synacthen (ACTH) & measuring cortisol at 30 & 60 minutes. Design All patients over 14 years (adulthood age in the region) who had undergone a SST from January 2010 to December 2017 were included. Patients who underwent pituitary surgery in preceding 2 months, on exogenous steroids, opioids & oral contraceptives were excluded. Stimulated cortisol of 550 nmol/L or more achieved at 30, 60 minutes or both was classified as a normal response. Results 965 patients were identified from pharmacy, medical & laboratory records. 116 patients were excluded and 849 were included in the analysis. Mean age was 50.5 ± 20.45 years. Mean weight was 67 ± 21 Kg. 54% patients were female. Mean basal, 30 and 60 minutes cortisol values after ACTH injection were 394 ± 286.58 nmol/L, 722 ± 327.11 nmol/L, 827 ± 369.30 nmol/L respectively. 715 patients (84 %) had a normal response and 134 patients (16 %) had suboptimal response. Primary and secondary adrenal insufficiency was diagnosed in 10% and 35% respectively. No ACTH value was available in 55% of the patients. Suboptimal response was observed at 30 minutes in 9.49% (n=72) of the patients: all crossed 550 nmol/L threshold at 60 minutes. Mean change of cortisol level from baseline to 30 minutes was 240 nmol/L in this particular sub group which was higher than the mean change of 152 nmol/L observed in patients who failed the test overall up to 60 minutes. No patient with optimal response at 30 minutes had suboptimal response at 60 min. Morning basal cortisol threshold of 226 nmol/L or over had 80% sensitivity, 71 % specificity & 93% positive predictive value to detect a normal SST (P-value &lt;.0001). Conclusion 60 minute cortisol correctly identified all normal and abnormal results. Relying only on 30 min value resulted in significant false positive results. Morning basal cortisol over 226 nmol/L should be considered reliable threshold for adequate adrenal function particularly when clinicians have low pretest probability for hypoadrenalism.


2019 ◽  
Vol 65 (5) ◽  
pp. 330-340
Author(s):  
Alla E. Yudina ◽  
Maria G. Pavlova ◽  
Vladimir M. Sotnikov ◽  
Alla E. Yudina ◽  
Yulia P. Sych ◽  
...  

BACKGROUND: The most of the current studies include patients who are different by the etiology of secondary adrenal insufficiency (SAI), or investigate SAI among other late effects of the radiation therapy. AIMS: To describe the features of SAI and to select the best method of screening SAI in adult patients followed complex treatment of nonpituitary brain tumors in childhood. MATERIALS AND METHODS: It was the retrospective cross-sectional study. 31 patients after the complex treatment of nonpituitary brain tumors in childhood and 20 healthy volunteers were examined. Age and sex ratio were comparable between the groups. Biochemical and clinical blood tests, levels of cortisol, ACTH, DHEA-C were evaluated. The insulin tolerance test (ITT) was performed for all patients and 11 volunteers. RESULTS: The prevalence of SAI by ITT was 45.2%. The levels of basal cortisol (BC) were significantly higher in patients without SAI in comparison with the SAI group and volunteers (505 [340; 650] vs 323 [233; 382] and 372 [263; 489] nmol / l; pSAI- without_SAI=0.001; pwihtout_SAI-healthy = 0.04). The SAI group had DHEA-C significantly lower than in other groups one (3.1 [1.8; 3.4] vs 5.1 [2.5; 6.4] and 6.8 [4.1; 8.9]; рSAI- without_SAI = 0.036; pSAI-healthy = 0.001). ROC analysis showed that BC and DHEA-S can be used as high-quality screening tests for SAI (AUC = 89.3% and 88.3%). The maximum level of cortisol (656 [608-686] vs 634 [548-677]; p = 1) and the time of its increase (45 and 60 min) did not differ during ITT in patients without SAI and volunteers. Side effects: delayed hypoglycemia occurred in 4/14 patients of the SAI group 4090 minutes late of injection 60-80 ml of 40% glucose solution for stopping hypoglycemia in the test. CONCLUSIONS: 45.2% of patients followed craniospinal irradiation had SAI that is characterized by a decrease in DHEA-C levels. A highly normal level of basal cortisol was observed in 45% of patients without SAI. DHEA-C and blood cortisol can be used for SAI screening.


2017 ◽  
Vol 7 (2) ◽  
pp. 150-153 ◽  
Author(s):  
Hali Rakici

ABSTRACT Aim Adrenal insufficiency (AI) in cirrhosis is an issue that has recently gained momentum. It can be seen in both stable and critically ill (sepsis, septic shock, and gastrointestinal system bleeding) cirrhotic patients. Its prevalence exists in a wide range since standardization of diagnostic methods is lacking. We aimed to scrutinize this issue in a 108 case series. Materials and methods We studied the presence of AI and its stage in patients with cirrhosis and its complications by using cross-sectional study. Standard-dose short synacthen test (SD-SST) was performed in 108 patients that had Child C decompensated cirrhosis without critical illness and it was aimed to determine the prevalence of AI based on basal cortisol, peak cortisol, and delta cortisol (basal total cortisol minus peak cortisol after stimulation) levels. Results The prevalence of AI in cirrhosis was found to be 25% based on basal cortisol level of <140 nmol/L, 22.2% based on delta cortisol level of <250 nmol/L, and 29.6% based on peak cortisol level of <500 nmol/L. Conclusion Prevalence of AI shows variation in decompensated cirrhosis without critical illness depending on different measures used. More definite results can be obtained when more standardized criteria are widely put into use. How to cite this article Rakici H. Adrenal Insufficiency in Cirrhosis Patients: Evaluation of 108 Case Series. Euroasian J Hepato-Gastroenterol 2017;7(2):150-153.


2019 ◽  
Vol 32 (1) ◽  
pp. 57-63 ◽  
Author(s):  
Rajni Sharma ◽  
Shamnad Madathil ◽  
Vivek Maheshwari ◽  
Kakali Roy ◽  
Brijesh Kumar ◽  
...  

Abstract Background The diagnosis of adrenal insufficiency (AI) is based on the basal and stimulated levels of serum cortisol in response to the short Synacthen test (SST). In patients with secondary AI (SAI) due to hypothalamic-pituitary-adrenal (HPA) axis defects, the SST has been validated against the insulin tolerance test (ITT), which is the gold standard. However, injection Synacthen is not easily available in some countries, and endocrinologists often use Acton-Prolongatum (intramuscular [IM] long-acting adrenocorticotropic hormone [ACTH]) in place of Synacthen. There are no studies validating the use of IM-ACTH in children with suspected AI. We evaluated the diagnostic value of the IM-ACTH test against the ITT for the diagnosis of SAI in children. Methods All children with suspected growth hormone deficiency (GHD) undergoing a routine ITT were evaluated using the IM-ACTH test within 1 week. Results Forty-eight patients (36 boys/12 girls, age range: 5–14 years) were evaluated using both the ITT and the IM-ACTH test. Twenty-eight patients had a normal cortisol response (≥18 μg/dL, 500 nmol/L) in the ITT and 20 had low values. In patients with a normal cortisol response on the ITT, the peak value obtained after the IM-ACTH test was higher than that on the ITT (28.7 μg/dL [± 8.8] vs. 23.8 μg/dL [± 4.54], respectively; p=0.0012). Compared to the ITT, the sensitivity and specificity of the IM-ACTH test for the diagnosis of SAI at cortisol cut-offs <18 μg/dL (500 nmol/L) and <22 μg/dL (600 nmol/L) were 57.1% and 92.8%, and 100% and 73.5%, respectively. Conclusions A peak cortisol value <18 μg/dL on the IM-ACTH test is highly suggestive of SAI, whereas a value >22 μg/dL rules out SAI.


2010 ◽  
Vol 163 (3) ◽  
pp. 377-382 ◽  
Author(s):  
Z Karaca ◽  
F Tanriverdi ◽  
H Atmaca ◽  
C Gokce ◽  
G Elbuken ◽  
...  

BackgroundThe aims of this study were to evaluate the validity of preoperative basal serum cortisol levels measured in predicting preoperative adrenal insufficiency and also the validity of basal serum cortisol levels and early postoperative insulin tolerance test (ITT) in predicting postoperative adrenal insufficiency.MethodsThe study was prospectively designed and included 64 patients who underwent pituitary surgery for conditions other than Cushing's disease. An ITT was performed preoperatively, on the 6th postoperative day and at the 1st postoperative month. Basal serum cortisol levels were measured on the 2nd, 3rd, 4th, 5th, and 6th postoperative days.ResultsPatients with a preoperative basal cortisol level of <165 nmol/l (6 μg/dl) showed insufficient cortisol response and those with levels higher than 500 nmol/l (18 μg/dl) had sufficient cortisol response to the preoperative ITT. The positive predictive value of the ITT performed on the 6th postoperative day was 69.7%, and the negative predictive value in predicting adrenal insufficiency at the 1st postoperative month was 58%. Patients were considered to have an insufficient cortisol response to ITT at the 1st postoperative month if their basal cortisol levels were <193 nmol/l (7 μg/dl) or 220 nmol/l (8 μg/dl) or 193 nmol/l (7 μg/dl) or 165 nmol/l (6 μg/dl) or 83 nmol/l (3 μg/dl) on the 2nd–6th postoperative days respectively.ConclusionSerum basal cortisol levels may be used as the first-line test in the assessment of the hypothalamic–pituitary–adrenal axis both preoperatively and postoperatively. Dynamic testing should be limited to the patients with indeterminate basal cortisol levels.


2020 ◽  
Vol 26 (1) ◽  
pp. 30-35 ◽  
Author(s):  
Juan Montes-Villarreal ◽  
Luis Alberto Perez-Arredondo ◽  
Rene Rodriguez-Gutierrez ◽  
Alejandro Diaz Gonzalez-Colmenero ◽  
Ricardo Cesar Solis ◽  
...  

Objective: To evaluate the performance of morning serum cortisol (MSC) compared to a 10 mg adrenocortico-tropic hormone (ACTH) stimulation test in the diagnosis of adrenal insufficiency (AI). Methods: A retrospective, cross-sectional analysis of ACTH stimulation tests were conducted. From a total of 312 potentially eligible ACTH stimulation tests, 306 met the inclusion criteria. The population was randomized into 2 groups: test (n = 159) and validation (n = 147). In the test group, the receiver operating characteristics curve test evaluated the diagnostic performance of MSC. Results: A subnormal cortisol response to ACTH was found in 25.8% of the test group. The area under the curve values of MSC to predict AI at +30 minutes, +60 minutes, or at maximal cortisol response were 0.874, 0.897, and 0.925 (95% confidence interval [CI] 0.81 to 0.92, 0.83 to 0.93, and 0.87 to 0.96). The Youden index was 234.2 mmol/L with a sensitivity of 83.3% (95% CI 65.2 to 94.3%), and a specificity of 89.1% (95% CI 82.4 to 93.9%). Positive and negative predictive values were 64.1% (95% CI 47.1 to 78.8%) and 95.8% (95% CI 90.5 to 98.6%). There was no difference in age, gender, AI prevalence, or mean serum cortisol at +30 or +60 minutes in the validation group; however, a lower mean MSC value was found. Lower sensitivity and specificity values (88.3% and 60%, respectively) were found for the 234.2 mmol/L cutoff value. Conclusion: This study supports the role of MSC as a first-step diagnostic test in patients with clinically suspected AI. The short stimulation test could be omitted in almost half of the cases. Prospective and longitudinal studies to reproduce and confirm the cutoff values proposed are warranted. Abbreviations: ACTH = adrenocorticotropic hormone; AI = adrenal insufficiency; AUC = area under the curve; CI = confidence interval; HPA = hypothalamic pituitary adrenal; ITT = insulin tolerance test; MSC = morning serum cortisol


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