scholarly journals Clinical Characteristics for the Improvement of Cushing's Syndrome Complicated With Cardiomyopathy After Treatment With a Literature Review

2021 ◽  
Vol 8 ◽  
Author(s):  
Sisi Miao ◽  
Lin Lu ◽  
Ling Li ◽  
Yining Wang ◽  
Zhaolin Lu ◽  
...  

Background: Endogenous Cushing's syndrome (CS), also called hypercortisolism, leads to a significant increase in mortality due to excessive cortisol production, which is mainly due to cardiovascular disease. CS complicated with cardiomyopathies, which is a rare and severe condition, has rarely been reported in the literature.Objective: To investigate the clinical characteristics of CS complicated with cardiomyopathies, we retrospectively reviewed the clinical manifestations, laboratory results, cardiac imaging results and prognosis to further understand the diagnosis, treatment, and management of these cases.Methods: The clinical data of patients diagnosed with CS complicated with cardiomyopathies obtained from discharge sheets from Peking Union Medical College Hospital from January 1986 to August 2021 were collected. Case reports of CS complicated with cardiomyopathies were retrieved from PubMed. In addition, Cushing's disease (CD) patients without cardiomyopathies were collected as controls to compare the clinical features.Results: A total of 19 cases of CS complicated with cardiomyopathies and cases of CD without cardiomyopathies (n = 242) were collected. The causes of CS included pituitary adenoma (n = 8, 42.11%), adrenal adenoma (n = 7, 36.84%), ectopic adrenocorticotropic hormone (ACTH) tumor (n = 2, 10.53%) and unclear causes (n = 2, 10.53%) in the CS complicated with cardiomyopathies group. The types of cardiomyopathies were dilated cardiomyopathies (n = 15, 78.94%) and hypertrophic cardiomyopathies (n = 4, 21.05%). The serum sodium concentration was significantly higher [145.50 (140.50–148.00) mmol/L vs. 141.00 (140.00–143.00) mmol/L], while the serum potassium concentration was significantly lower [2.70 (2.40–3.60) mmol/L] vs. 3.90 (3.50–4.20 mmol/L)] in the CS complicated with cardiomyopathies group compared to the CD patients without cardiomyopathies. There were no significant differences between the CS complicated with cardiomyopathies group and the CD patients without cardiomyopathies in the serum cortisol concentration and 24-h urine free cortisol, but a significant difference in the adrenocorticotropic hormone level [109.00 (91.78–170.30) pg/ml vs. 68.60 (47.85–110.00) pg/ml]. Twelve/16 (75.0%) patients showed significant improvement or even a complete healing of the heart structure and function after remission of hypercortisolemia after treatment with CS.Conclusions: CS complicated with cardiomyopathies is a very rare clinical entity, in which cortisol plays an important role and it can be greatly improved after remission of hypercortisolemia.

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Wasita Warachit Parksook ◽  
Nitchakarn Laichuthai ◽  
Sarat Sunthornyothin

The most common subtype of endogenous Cushing’s syndrome (CS) is Cushing’s disease (CD), with higher proportions of adrenal CS reported from Asia, compared to other continents. However, little was known about CS in this territory. This study was to investigate the distribution, clinical characteristics, and treatment outcomes of CS in a single tertiary hospital in Thailand. We performed a retrospective evaluation of 82 patients with endogenous CS during 2001–2015. The most common subtype was CD, followed by adrenal CS and ectopic ACTH syndrome (EAS), respectively. Weight gain was the most common presentation. Normal body mass index (BMI), Asian cutoff, was observed in 33% of patients. Specific features of CS (plethora, muscle weakness, bruising, and/or wide purplish striae) were documented in less than half of patients. The median age, adrenocorticotropic hormone (ACTH), and urinary free cortisol (UFC) concentrations were significantly different among 3 subtypes of CS and were highest among patients with EAS. An initial remission rate after transsphenoidal surgeries in CD was 62%, with higher rates in pituitary microadenomas compared to macroadenomas. All patients with unilateral adrenal disease achieved CS remission after adrenal surgeries. Patients with EAS achieved CS remission mostly from bilateral adrenalectomy. The highest mortality rate was observed in the EAS group. These findings were consistent with previous studies in Asia, with more proportions ACTH-independent CS.


1966 ◽  
Vol 35 (1) ◽  
pp. 29-44 ◽  
Author(s):  
E. A. ESPINER

SUMMARY Measurements of urinary free cortisol were made in convalescent subjects and in patients with established Cushing's syndrome and the results compared with those in acutely ill patients, in pregnancy and in surgical patients. Cortisol was measured in urine after paper chromatography, each measurement being corrected for losses according to the recovery of added internal standard (tritiated cortisol). In a number of cases, the diurnal rhythm of cortisol excretion was also examined and the results related to plasma 11-hydroxycorticosteroid levels and measurements of renal function. The mean 24 hr. urinary cortisol excretion in 13 convalescent subjects was 74μg. (range 35–98 μg./24 hr.) and all showed a well-marked diurnal rhythm. Patients with Cushing's syndrome excreted more than normal amounts of cortisol, even when plasma 11-hydroxycorticosteroids were in the normal range; there was a marked reversal in the diurnal rhythm of cortisol excretion. In 13 acutely ill medical patients with pyrexia, four only showed unequivocal increases in cortisol excretion although there was frequently an upset in the diurnal rhythm. The duration of the illness, rather than the severity, appeared to be an important factor so far as the response of the adrenals was concerned. A consistent increase in cortisol excretion was found in pregnancy but there was no significant difference between the day and night excretion of cortisol. The response to the stress of surgical trauma was largely dependent upon the severity of the operative procedure. The importance of emotional stress immediately before operation was shown in 4 out of 14 patients.


2012 ◽  
Vol 56 (7) ◽  
pp. 461-464 ◽  
Author(s):  
Pedro Rodrigues ◽  
José Luís Castedo ◽  
Margarida Damasceno ◽  
Davide Carvalho

Ectopic ACTH syndrome is a rare disease often associated with severe hypercortisolism. When feasible, optimal management is surgical excision of the tumor. A 33-year-old male patient was admitted to the hospital in 1993 with clinical manifestations suggestive of Cushing's syndrome. He presented high plasma ACTH and markedly elevated urinary free cortisol excretion that was not suppressed with high-dose dexamethasone administration. Pituitary MRI scan was normal. No central-to-peripheral ACTH gradient was present in bilateral inferior petrosal sinus sampling. Thoracic CT scan showed a 1.7 cm nodule at the left lung. Pulmonary fine needle cytology and immunocytochemical and ultrastructural studies, together with the presence of bone metastases, led to the diagnosis of an ACTH-producing neuroendocrine carcinoma. He was initially submitted to chemotherapy and has been on treatment with octreotide LAR since 1998, having shown a favorable clinical, biochemical and imaging response. We highlight the excellent long-term response to medical therapy with octreotide LAR, without tachyphylaxis, probably due to its antiproliferative effect.


2007 ◽  
Vol 157 (6) ◽  
pp. 725-731 ◽  
Author(s):  
Srividya Kidambi ◽  
Hershel Raff ◽  
James W Findling

ObjectiveCushing's syndrome (CS) is difficult to diagnose due to its nonspecific presentation. Diagnostic tests like 24-h urine free cortisol (UFC) and the overnight 1 mg dexamethasone suppression test (DST) lack sufficient sensitivity and specificity. Measurement of nocturnal salivary cortisol (NSC) is an accurate and reproducible test with a high sensitivity for CS. However, its performance in mild CS has not been reported. We present 11 cases of CS with normal or mildly elevated UFC in whom NSC was helpful in making a diagnosis.Design and methodsAll patients had at least one collection of 24-h UFC and NSC and eight had an overnight 1 mg DST. The number of NSC measurements per patient was determined by the clinical index of suspicion and the results of initial testing. Imaging studies included magnetic resonance imaging (MRI) of pituitary or computer tomography scan of abdomen.ResultsOnly four out of eleven patients had elevations in UFC and none were >2 times the upper limit of normal. Seven out of eight had an abnormal DST. All patients had some elevated NSCs (14–100%). Out of eleven patients, six had an abnormality in the pituitary gland found by MRI and two out of eleven had adrenal masses. The remaining three had normal pituitary MRI but had inferior petrosal sinus (IPS) sampling indicating Cushing's disease. All patients had appropriate surgery, and histopathology of all except one was suggestive of either a cortisol-producing adrenal adenoma or an ACTH-secreting pituitary adenoma.ConclusionNeither a normal UFC nor a normal NSC excludes mild CS. Multiple samples (urine/saliva) and DST are needed to make the diagnosis of mild CS.


2021 ◽  
Vol 12 (1) ◽  
pp. 78-82
Author(s):  
Lubna Naznin ◽  
Susane Giti ◽  
SK Md Jaynul Islam ◽  
Khandaker Rokshana Akhter ◽  
Shamoli Yasmin ◽  
...  

Cushing syndrome results from chronic exposure to excess cortisol. Nodular adrenal hyperplasia is usually bilateral and has only a few case reports of unilateral presentation. Biochemically it is presented as hyperaldosteronism or as Cushing’s syndrome. Here, we are reporting a 17-year-old female presenting with weight gain over 5 months and uncontrolled hypertension. Biochemically she was found to have diabetes mellitus, secondary hypothyroidism and hypogonadotrophic hypogonadism due to Cushing’s syndrome of adrenal origin. Unilateral adrenal adenoma/hyperplasia in right adrenal gland was evident by radiology. Histopathological examination was done after laparoscopic adrenalectomy showed nodular adrenocortical hyperplasia in right adrenal mass. Following surgery, clinical features of the patient improved notably. Cushing syndrome due to unilateral nodular adrenocortical hyperplasia is a rare entity. Biochemical evaluation of hypothalamo-pituitary-adrenal axis, radiological evidence and histopathology are the important armaments can guide to final diagnosis. BIRDEM Med J 2022; 12(1): 78-82


2018 ◽  
Vol 178 (6) ◽  
pp. R259-R266 ◽  
Author(s):  
Thierry Brue ◽  
Vincent Amodru ◽  
Frederic Castinetti

With fewer than 200 reported cases, Cushing’s syndrome (CS) in pregnancy remains a diagnostic and therapeutic challenge. In normal pregnancies, misleading signs may be observed such as striae or hypokalemia, while plasma cortisol and urinary free cortisol may rise up to 2- to 3-fold. While the dexamethasone suppression test is difficult to use, reference values for salivary cortisol appear valid. Apart from gestational hypertension, differential diagnosis includes pheochromocytoma and primary aldosteronism. The predominant cause is adrenal adenoma (sometimes without decreased ACTH), rather than Cushing’s disease. There are considerable imaging pitfalls in Cushing’s disease. Aberrant receptors may, in rare cases, lead to increased cortisol production during pregnancy in response to HCG, LHRH, glucagon, vasopressin or after a meal. Adrenocortical carcinoma (ACC) is rare and has poor prognosis. Active CS during pregnancy is associated with a high rate of maternal complications: hypertension or preeclampsia, diabetes, fractures; more rarely, cardiac failure, psychiatric disorders, infection and maternal death. Increased fetal morbidity includes prematurity, intrauterine growth retardation and less prevalently stillbirth, spontaneous abortion, intrauterine death and hypoadrenalism. Therapy is also challenging. Milder cases can be managed conservatively by controlling comorbidities. Pituitary or adrenal surgery should ideally be performed during the second trimester and patients should then be treated for adrenal insufficiency. Experience with anticortisolic drugs is limited. Metyrapone was found to allow control of hypercortisolism, with a risk of worsening hypertension. Cabergoline may be an alternative option. The use of other drugs is not advised because of potential teratogenicity and/or lack of information. Non-hormonal (mechanical) contraception is recommended until sustained biological remission is obtained.


2021 ◽  
Vol 86 (5) ◽  
pp. 331-334
Author(s):  
Zuzana Koudelková ◽  
◽  
Romana Gerychová ◽  
Tereza Nešporová

Objective: The description of a rare case of Cushing’s syndrome caused by an adrenal adenoma in pregnancy with successful treatment. Case presentation: 30-ear-old Gravida 3 female was admitted to our hospital with hypertension at the 18th week of gestation. Hormonal analyses revealed primary Cushing’s syndrome with high plasma cortisol levels and low levels of adrenocorticotropic hormone. Magnetic resonance imaging demonstrated a mass on the right-side of the adrenal gland. Adrenalectomy was performed in the 28th week of gestation and the following histopathology revealed an adrenocortical adenoma. Pregnancy continued until the 38th week of gestation with glucocorticoid replacement therapy and the patient gave birth vaginally to a healthy boy in the 38th week of gestation. Conclusion: Cushing’s syndrome in pregnancy rarely occurs; dia gnosis may be dismissed or determined after birth in most cases. Misdia gnosis of Cushing’s syndrome is common because of physiological increase of corticotropin hormones and cortisol levels and overlapping symptoms that can occur even during physiological pregnancy. Cushing’s syndrome should have a place in the diff erential dia gnosis of hypertension in pregnancy (especially before the 20th week of gestation). Analysis of the urinary free cortisol level and circadian rhythm blood cortisol can provide a reasonable strategy to diagnose Cushing’s syndrome in pregnant women. Early dia gnosis and surgical treatment can signifi cantly reduce maternal and fetal complications. Key words: adenoma – Cushing’s syndrome – pregnancy – hypertension


Open Medicine ◽  
2009 ◽  
Vol 4 (1) ◽  
pp. 59-64 ◽  
Author(s):  
Maria Yaneva ◽  
Georgi Kirilov ◽  
Sabina Zacharieva

AbstractThe aim of the present study is to evaluate the measurement of midnight salivary cortisol as a method of screening for Cushing’s syndrome (CS). Here we tested the performance of a highly sensitive electrochemiluminescence immunoassay (ECLIA) for midnight salivary cortisol measurement in an extensive clinical study (n=104). Three groups were investigated: 30 patients with CS, 34 with obesity and 40 healthy normal weight controls. All of them collected saliva samples at 24:00 h and urine samples over the same day (24 hour period). An electrochemiluminescence immunoassay was used to measure salivary cortisol. Mean midnight salivary cortisol in healthy volunteers, obese patients and patients with CS was 8.33 ± 3.62, 8.13 ± 4.47 and 33.11 ± 21.68 nmol/l, respectively. No significant difference was found between midnight salivary cortisol in healthy and obese subjects (P>0.05). In contrast, salivary cortisol at midnight was significantly higher in patients with CS (P<0.001) as compared to both other groups. The cut-off point of 14.2 nmol/l yielded a sensitivity of 93.3% and a specificity of 94.2% (AUCROC=0.984 ± 0.01(0.965-1.000). A strong positive correlation between midnight salivary cortisol and urinary free cortisol has been found in the CS group (r=0.686, P<0.0001). Our results demonstrate that measurement of midnight salivary cortisol could be successfully used as a first-line screening method for CS. Our data approve ECLIA as a simple, reliable and timesaving method for the assessment of salivary cortisol. Automated measurement of midnight salivary cortisol by ECLIA would facilitate the routine practice in the screening for CS.


1986 ◽  
Vol 32 (1) ◽  
pp. 93-96 ◽  
Author(s):  
M Schöneshöfer ◽  
B Weber ◽  
W Oelkers ◽  
K Nahoul ◽  
F Mantero

Abstract To evaluate their potential usefulness in the differential diagnosis of Cushing's syndrome, we estimated the urinary excretion rates of the following non-metabolized, unbound steroid hormones: pregnenolone, progesterone, 17-OH-pregnenolone, 17-OH-progesterone, dehydroepiandrosterone (DHEA), androstenedione, testosterone, dihydrotestosterone, 11-deoxycorticosterone, 11-deoxycortisol, corticosterone, cortisol, 18-OH-11-deoxycorticosterone, 18-OH-corticosterone, and aldosterone. These were measured in normal subjects and in patients with Cushing's disease, adrenal adenoma, or ectopic corticotropin syndrome. We used "high-performance" liquid chromatography and subsequent radioimmunoassay. Our results indicate that simultaneous estimation of urinary free cortisol and DHEA may be useful in differential diagnosis of hypercorticoid states due to adrenal adenoma and Cushing's disease.


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