scholarly journals PALMAR CREASE PIGMENTATION

2019 ◽  
Vol 17 (4) ◽  
pp. 144
Author(s):  
Habibullah Khan

A young lady presented with history of giddiness, blackouts, and darkness of her skin colour especially the face. On examination the colour of her skin was quite dark but not much darker than her spouse. She had buccal pigmentations and her palmar creases were obviously pigmented. Facility for short Synacthen test was not available. Her serum cortisol was on the lower normal side and ACTH was sky high; 1024 pg/ml (ref. range adults: 6-76 pg/ml). She was screened for ectopic ACTH production which could not reveal any pathology. Also plain abdominal x-ray didn’t show any adrenal calcification. She was labeled as Addison’s disease and improved drastically after replacement therapy (Cortisone + Fludrocortisone). Giddiness aloof, she was delighted with the fairness of her colour.

Author(s):  
Caterina Policola ◽  
Victoria Stokes ◽  
Niki Karavitaki ◽  
Ashley Grossman

Summary Opiate drugs such as morphine are in extensive use for pain relief and palliation. It is well established that these drugs can cause changes in endocrine function, but such effects are not always sufficiently appreciated in clinical practice, especially in relation to the hypothalamic–pituitary–adrenal (HPA) axis. Herein, we report on an 18-year-old man who was diagnosed with a slipped left femoral epiphysis following a long history of pain in his leg. On examination, he was thought to look relatively young for his age and therefore the orthopaedic surgeons arranged an endocrine assessment, which showed an undetectable concentration of serum cortisol and a suppressed concentration of testosterone; therefore, he was referred urgently with a diagnosis of hypopituitarism. We elicited a history that he had been treated with opiate analgesics for 3 days at the time of his original blood tests. Full endocrine assessment including a short Synacthen test revealed that he now had normal adrenal and pituitary function. We conclude that his morphine therapy had caused profound suppression of his HPA and pituitary–gonadal axes and suggest that clinicians should be aware of these significant changes in patients on even short-term opiate therapy. Learning points Therapy with opiates is the standard therapy for severe acute and chronic pain. Such drugs cause profound changes in endocrine function. Importantly, opiates suppress the HPA axis at a central level. Short-term therapy with morphine could be the cause of biochemical adrenocortical insufficiency. Morphine and related drugs also suppress the pituitary–gonadal axis. After discontinuation of therapy with such drugs, adrenal function improves.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A91-A91
Author(s):  
Bo Bo San

Abstract There is a growing concern upon the finding of many drug induced Cushing’s syndrome because of inadvertent use of glucocorticoids (GC) either prescribed or as alternative medicine in Myanmar. These patients are presenting with diversity of clinical problems ranging from hypertension, diabetes to acute adrenal crisis due to hypothalamic-pituitary-adrenal (HPA) axis suppression and secondary adrenal insufficiency (AI). The present study aimed to assess the delta cortisol (the degree of cortisol increments) during the first short Synacthen test (SST) as a factor predictive of adrenal function recovery in patients with drug induced Cushing’ syndrome with secondary AI and to determine the proportion of patients who recovered from AI within six-month follow-up. This was a hospital based prospective analytical study that enrolled a total of 52 patients with drug induced Cushing’s syndrome with secondary AI from January 2018 to June 2019. Secondary AI is defined by morning basal serum cortisol ≤ 400 nmol/L with Synacthen stimulated peak cortisol level ≤ 550 nmol/L and serum ACTH < 60 pg/ml. The follow-up SSTs were performed at three-month and six-month after first SST, and the patients with morning basal serum cortisol > 400 nmol/L (or) Synacthen stimulated peak serum cortisol > 550 nmol/L during follow-up SSTs are defined as recovered adrenal function group. In this study, a total of 52 patients were treated with modified regimen of physiological dose of prednisolone with tapering schedule or stress dose GC based on the basal serum cortisol levels up to six months. Among them, nearly half (n=25 / 48.1%) of the patients with drug induced Cushing’s syndrome with secondary AI achieved normal adrenal function within six-month follow-up. It was found that mean values for delta cortisol were not statistically significant between recovered and non-recovered groups, 118.6 nmol/L (SD 72.3) and 97.2 nmol/L (SD 64.2) respectively. The delta cortisol during the first SST could not predict strongly (AUC - 0.6, 95% CI - 0.44 to 0.76, P = 0.2) the recovery of adrenal function in patients with drug induced Cushing’s syndrome with secondary AI and it was inconsistent with previous studies. The older age of the patients, oral route of administration of drugs causing Cushing’s syndrome and comorbid hypertension were found to be more significant in the non-recovered group. In conclusion, the present study did not support the evidence that the delta cortisol during the first SST could predict adrenal function recovery in patients with drug induced Cushing’ syndrome with secondary AI. Reference: (1) Baek et al., 2016; Recovery of Adrenal Function in Patients with Glucocorticoids Induced Secondary AI. Endocrinol Metab.31, pp. 153–160. (2) Pofi et al., 2018; The Short Synacthen Test Can Be Used to Predict Recovery of HPA Axis Function. J Clin Endocrinol Metab.103(8), pp. 3050–3059.


2019 ◽  
Vol 96 (1132) ◽  
pp. 67-72
Author(s):  
Rosemary Dineen ◽  
Ahmed Mohamed ◽  
Anjuli Gunness ◽  
Ana Rakovac ◽  
Emer Cullen ◽  
...  

In recent years, the short Synacthen test (SS) has become the most widely used test to assess adrenal reserve. Despite its frequent use, there are still several areas related to the short Synacthen test (SST), which have no consensus including the optimum sampling times, that is, whether a 60 min post-Synacthen administration cortisol is necessary or not.MethodologyWe performed a retrospective data analysis of 492 SSTs performed on adult patients in a tertiary referral teaching hospital in Ireland. The SSTs were performed in the inpatient and outpatient setting and included patients across all medical disciplines and not exclusively to the endocrinology department.Results313 patients had 0, 30 and 60 min samples available for analysis. A total of 270/313 (82%) were deemed to pass the test, that is, cortisol ≥500 nmol/L at both 30 and 60 min. Of the 313 patients, 19 (6%) patients had an indeterminate response, cortisol <500 nmol/L at 30 min, but rising to ≥500 nmol/L on the 60 min sample. Of these 19 patients, only 9/19 patients had a serum cortisol level at 30 min <450 nmol/L, requiring clinical treatment with glucocorticoid replacement. All 24/313 (8%) patients who had insufficient responses at 60 min were also insufficient at 30 min sampling. No individuals passed (≥500 nmol/L) at 30 min and then failed (<500 nmol/L) at 60 min.ConclusionUsing the 30 min cortisol sample post-Synacthen administration alone identifies clinically relevant adrenal insufficiency in the majority of cases. A small subset of patients have a suboptimal response at 30 min but have a 60 min cortisol concentration above the threshold for a pass. Data regarding the long-term outcomes and management of such patients are lacking and require further study.


2009 ◽  
Vol 160 (1) ◽  
pp. 9-16 ◽  
Author(s):  
Timo Deutschbein ◽  
Nicole Unger ◽  
Klaus Mann ◽  
Stephan Petersenn

ObjectiveAccurate assessment of adrenal function is essential in patients with hypothalamic–pituitary–adrenal (HPA) disease. The measurement of salivary cortisol (SaC) instead of serum cortisol (SeC) offers several advantages, such as the determination of the free hormone. We evaluated the diagnostic value of SeC and SaC both unstimulated and during a high-dose short synacthen test (HDT) in comparison to the insulin tolerance test (ITT).DesignComparative study between 2005 and 2007.MethodsFifty-five patients with HPA impairment and 21 healthy controls were enrolled. Samples were collected in the early morning and over 120 min during the HDT. Receiver operating characteristic analysis revealed individual thresholds for four HDT periods (0–30, 0–60, 0–90, and 0–120 min).ResultsThe ITT identified 30 subjects as adrenal insufficient. With respect to the four HDT periods, sensitivity and specificity were 67–79% and 71–88% for SeC, compared with 63–72% and 72–86% for SaC. If upper and lower thresholds (with specificities >95%) were applied, patients were diagnosed in 40–45% by SeC and in 25–31% by SaC. The combination of basal cortisol and HDT allowed a diagnosis in 47–49% (SeC) and in 42–45% (SaC) respectively.ConclusionWe suggest the determination of basal SeC or SaC as first-line test. In comparison to the ITT, the HDT has only limited value in screening for alterations of the HPA axis. If the HDT is performed, sampling may be limited to 30 min post-synacthen, using either SeC or SaC. Due to the ease of collection and the independence of binding proteins, SaC may be preferable.


2020 ◽  
pp. postgradmedj-2020-138514
Author(s):  
Rajeev Kumar ◽  
Peter Carr ◽  
Ws Wassif

ObjectiveThe short synacthen test (SST) is widely used across the UK to assess adrenal reserve. The main objective of our study was to determine the morning serum cortisol level that will predict adrenal insufficiency (AI) thus reducing our reliance on SST.DesignThis was a single centre retrospective study of 393 SST tests measuring 0 and 60 min cortisol levels after administration of 250 μg of synacthen (synthetic ACTH).Patients and methodsAll the SST tests for patients suspected of primary or secondary AI between April 2016 and October 2018 were included in this study. We used serum to determine circulating cortisol by a newer generation competitive electrochemiluminescence immunoassay (ECLIA) (Roche Diagnostics). A post-ACTH cortisol response of ≥420 nmol/L at 60 min was considered adequate to rule out AI. The data were analysed to ascertain the relationship between 0 min and 60 min serum cortisol.ResultsA total of 393 SST results were included in this study. Overall, a total of 332 (84.5%) subjects achieved sufficient serum cortisol level at 60 min, while 61 subjects (15.5%) showed insufficient response. Using the logistic regression, we determined that a morning basal serum cortisol level of ≥354 nmol/L was able to predict normal adrenal function with 100% sensitivity. We were unable to find a lower cut-off value below which SST will not be required. By using this proposed cut-off point, approximately 37% of the SSTs tests could be avoided.ConclusionsBasal morning serum cortisol can be safely used as a first step in the evaluation of patients with suspected AI. This will enhance the number of patients being screened for this condition.


Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Ruth Smith ◽  
Mohammed Akil

Abstract Background Many rheumatological conditions are treated with steroids. Patients on long-term steroids are known to be at risk of adrenal insufficiency especially when withdrawing them. Sheffield Teaching Hospital (STH) Endocrinologists advised the following protocol for patients reducing steroids to determine hypothalamic pituitary adrenal status and confirm safe onward withdrawal of steroids:- After 1 month on 3mg prednisolone measure 9am serum cortisol and ACTH (after 24 hours off steroid)- If cortisol &lt;150nmol/L or &lt; 200nmol/L and ACTH &lt;10pg/mL - refer Endocrinology- If cortisol 200-430nmol/L (or &gt; 150nmol/L and ACTH &gt;10pg/mL) arrange short Synacthen test (SST). If 30-minute cortisol &lt;430nmol/L - refer Endocrinology. If 30 minute cortisol &gt;430nmol/L - stop steroids and monitor for adrenal insufficiency (give hydrocortisone 20mg TDS to cover illness for 3 months after stopping prednisolone). Methods We performed a retrospective review of records of rheumatology patients having morning cortisol level checked July 2017-January 2019 to determine how many tests were abnormal and to what degree the results changed management. Results 160 cortisol tests were performed. 36 were excluded from further analysis. Of the 124 cases reviewed: 80 females and 44 males. Age range 18-87; mean 64 years. Starting dose of prednisolone 5-60mg. Duration of steroid use 6 months to &gt; 10 years; modal duration 13-24 months (49 patients/40%). 59 patients/48% were taking other immunosuppressants. Prednisolone dose at cortisol testing 1-5mg; modal value 3mg (89 patients/72%). 12 patients/10% had a normal morning cortisol. 9 patients/7% fell into the group ‘cortisol &lt;150nmol/L refer Endocrinology.’ 103 patients/83% fell into the group requiring SST. 79 patients had an SST (out of an expected 112). 58/73% were normal. 17 patients/81% with abnormal SST were referred to Endocrinology. 11/65% were advised to wean prednisolone as per normal rheumatology practice of 1mg per month; 1/6% to continue prednisolone and repeat SST in 2-3 months; 3/18% to commence hydrocortisone. 2/12% had not yet been seen. Of the patients with abnormal SST: 8 females and 13 males. Age range 32-87; mean 67 years. Starting dose of prednisolone 5-40mg. Duration on steroids 6 months to &gt; 10 years; modal duration 13-24 months in 9 patients/43%. 12 patients/57% were on other forms of immunosuppression. Conclusion Endocrinology guidance changed the management of 3 patients/2% by the introduction of hydrocortisone compared with previous rheumatology standard practice of weaning prednisolone by 1mg per month or similar. No distinctive characteristics were identified in patients with abnormal SST, except for a greater proportion of men (35% of whole group: 62% of abnormal SST group). Given the limited impact of the investigations, and the shortage of Synacthen, a new protocol has been implemented: if morning cortisol &lt;300nmol/L, or 300-350nmol/L and symptoms - contact Endocrinology for SST; if 30 minute cortisol &lt;430nmol/L - refer Endocrinology; otherwise wean prednisolone as normal. Disclosures R. Smith None. M. Akil Honoraria; AbbVie, Actelion, Celgene, Astra Zenica, Lilly and UCB.


Author(s):  
Elżbieta Moszczyńska ◽  
Katarzyna Pasternak-Pietrzak ◽  
Monika Prokop-Piotrkowska ◽  
Anna Śliwińska ◽  
Sylwia Szymańska ◽  
...  

AbstractBackgroundEctopic adrenocorticotropic syndrome (EAS) causes approximately 10–18% of cases of Cushing’s syndrome (CS) in adults, while in children it occurs much less frequently.What is new?We present a long-term follow-up (8 and 13 years) of the only two cases of rare neuroendocrine tumors in 30 years of a single center experience, which is unique in the literature concerning the pediatric population.Case presentationWe report two cases of neuroendocrine tumors (of the thymus and the appendix) in a 12-year-old boy and a 15-year-old girl who presented with the clinical features of CS. Elevated serum cortisol, ACTH, and chromogranin levels were observed in both patients. Diagnoses were made on the basis of a mass in the thymus/appendix region visualized with chest/abdominal CT scan and radiotracer accumulation in scintigraphy in the same areas. Histopathological examinations confirmed the diagnoses of NET.ConclusionEAS is an extremely rare endocrine disorder. However, it should be taken into consideration in the diagnostic process of every case of ACTH-dependent CS.


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.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Farah Laith Al Sabie ◽  
Meenu Jain ◽  
Ammara Aziz

Abstract Introduction: Cushing disease refers to the endogenous overproduction of glucocorticoid due to an ACTH-producing pituitary adenoma. It is important to recognize and treat due to the adverse health outcomes associated with it. We describe an unusual case of Cushing disease which presented very rapidly and progressively with extremely high cortisol levels mimicking those seen in ectopic production of ACTH.Case Presentation: A 43 year old Caucasian man, with no past medical history, presented with hypertensive crisis. He was discharged home with anti-hypertensive medications. Over the next 4 months, he gained 20 pounds, mainly around his abdomen, developed fatigue, and blood pressure continued to be high despite six anti-hypertensive medications, developed diabetes and hypokalemia, requiring 120 meq/day of potassium chloride. On exam, he had plethora, central obesity and wide, purple striae over his abdomen. Work-up for secondary causes of hypertension showed normal renal Doppler US, normal aldosterone and renin activity, normal plasma metanephrines, however, his 24 hour urinary free cortisol was dramatically elevated at 4022ug/day with a urine volume of 4 L, 1 mg dexamethasone suppression test showed unsuppressed serum cortisol of 55ug/dl. Morning ACTH of 125 pg/ml with concurrent serum cortisol level of 53.8 mcg/dl, indicated ACTH-dependent hypercortisolism. Inferior petrosal sinus sampling indicated a pituitary source of ACTH. Sellar MRI initially did not show a pituitary adenoma, however, repeat MRI with a 3-Tesla magnet showed a 4 mm pituitary adenoma. He was treated with ketoconazole and was started on atovaquone for PCP prophylaxis while awaiting trans-sphenoidal resection, which he had a month later. Pathology showed a 4 mm adenoma which stained strongly for ACTH. On postoperative day 1, serum cortisol dropped to 2.1 from 52.3 mcg/dl, and patient was discharged on hydrocortisone replacement. Three weeks later, he had lost 12 pounds, hyperglycemia improved with discontinuation of insulin, hypokalemia resolved and hypertension was well controlled on two anti-hypertensives. Discussion: ACTH-dependent Cushing syndrome is either caused by Cushing disease, or from ectopic ACTH production from a tumor. Cushing disease is characterized by a gradual onset and subtle manifestations of hypercortisolism. Acute, severe presentation favors an ectopic ACTH producing tumor, and is associated with much higher cortisol levels. In our patient, clinical data suggested ectopic ACTH production, yet he was found to have Cushing disease, and was treated successfully with trans-sphenoidal resection of the pituitary adenoma. It is imperative to consider all possibilities, and do the full work up so as not to miss an atypical presentation of Cushing disease, and direct treatment accordingly.


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