scholarly journals Cushing syndrome secondary to late onset adrenal hyperplasia: presentation and challenges of management

2018 ◽  
Vol 1 (1) ◽  
Author(s):  
Muzzammil Abdullahi ◽  
Mamuda Atiku ◽  
Imam Mohammed Ibrahim
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Late Onset ◽  
Adrenal Adenoma ◽  
Protein Structures ◽  
Cushing's Syndrome ◽  
Response To Treatment ◽  
Adrenal Tumors ◽  
Adrenal Hyperplasia ◽  
Supraclavicular Fossa

Cushing’s syndrome is a clinical disorder caused by overproduction of cortisol. Adrenal adenoma is the cause in 5% of cases of Cushing syndrome. ACTH-independent Cushing’s syndrome in 90% is caused by unilateral adrenal tumors. Of these, adenomas are the cause in 80% of the cases, while the others are adrenocortical carcinoma. Rare causes of Cushing’s syndrome include adrenal hyperplasia. Overproduction of cortisol results in weakened protein structures leading to protuberant abdomen and poor wound healing, glucose is converted to fat and deposited in the abdomen, supraclavicular fossa and cheeks. Other presentations are diabetes, hypertension, osteoporosis, fractures, impaired immune function, glucose intolerance, and psychosis. We report a 21-year-old female who presented with clinical symptoms of newly diagnosed hypertension and diabetes mellitus which after poor response to treatment she was evaluated and diagnosed to have Cushing syndrome. She subsequently had left adrenalectomy and did very well with resolution of patients’ symptoms. The histology came out to be diffuse adrenal hyperplasia.

SEMIQUANTITATIVE ESTIMATION OF URINARY PREGNANETRIOL, PREGNANETRIOLONE AND TETRAHYDRO S IN THE INVESTIGATION OF ADRENOCORTICAL FUNCTION

1969 ◽  
Vol 60 (4) ◽  
pp. 645-656 ◽  
Author(s):  
Frances J. Thomas ◽  
A. W. Steinbeck
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Cushing’S Syndrome ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Adrenocortical Function ◽  
Endocrine Disorders ◽  
Adrenal Carcinoma ◽  
Adrenal Hyperplasia ◽  
Validity And Reliability ◽  
Diagnostic Significance

ABSTRACT Following β-glucuronidase hydrolysis, pregnanetriol, pregnanetriolone and tetrahydro S were extracted from urine, chromatographed on florosil and in two paper systems before semiquantitative estimation of pregnanetriol and pregnanetriolone with the phosphoric acid reaction and of tetrahydro S with blue tetrazolium. The chemical validity and reliability of the method were studied and excretions measured in normal subjects and endocrine disorders. Pregnanetriol excretions were compared with published values. Normal pregnanetriol excretions were found in »idiopathic« hirsutism, with Stein-Leventhal ovaries and in Cushing's syndrome due to adrenal adenoma and hyperplasia; increased excretions were found in congenital adrenal hyperplasia and Cushing's syndrome with adrenal carcinoma. Pregnanetriolone was detected only in congenital adrenal hyperplasia, Cushing's syndrome from hyperplasia and one carcinoma. Large amounts of tetrahydro S were found in Cushing's syndrome with adrenal carcinoma. The possible diagnostic significance of the findings is discussed.

The Role of Computed Tomography in the Localization of Adrenal Cortex Tumors

1982 ◽  
Vol 21 (01) ◽  
pp. 16-18
Author(s):  
W. Misiorowski ◽  
R. Pacho ◽  
M. Wysocki ◽  
M. Kobuszewska ◽  
S. Zgliczyński ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Cushing’S Syndrome ◽  
Adrenal Adenoma ◽  
Plasma Renin ◽  
Plasma Potassium ◽  
Cushing's Syndrome ◽  
Adrenal Tumors ◽  
Plasma Potassium Level ◽  
Plasma Aldosterone Level

The aim of this study was to evaluate the usefulness of computed tomography in the localisation of adrenal tumors producing aldosterone and Cortisol. One case each of Conn’s and Cushing’s syndrome are described. The diagnosis of Conn’s syndrome was established by demonstrating an elevated plasma aldosterone level “at rest” and its decrease after stimulation, the absence of plasma renin activity and a lowered plasma potassium level. The diagnosis of Cushing’s syndrome due to adrenal adenoma was established by demonstrating the typical clinical features, an abnormal diurnal rhythm of Cortisol and ACTH secretion and an increased urine excretion of 17-OHCS without suppression by large doses of dexamethasone. The localisation and the size of the tumors as determined by computed tomography were confirmed during surgery.

scholarly journals Food-Dependent Cushing’s Syndrome: Possible Involvement of Leptin in Cortisol Hypersecretion*

1999 ◽  
Vol 84 (10) ◽  
pp. 3817-3822 ◽  
Author(s):  
François P. Pralong ◽  
Fulgencio Gomez ◽  
Louis Guillou ◽  
François Mosimann ◽  
Sebastiano Franscella ◽  
...  
Keyword(s):  
Food Intake ◽  
Cushing’S Syndrome ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Cortisol Secretion ◽  
Adrenal Hyperplasia ◽  
Adrenal Cells ◽  
Patient Reported ◽  
Stimulation Of

Abstract Stimulation of cortisol secretion by food intake has been implicated in the pathogenesis of some cases of ACTH-independent Cushing’s syndrome, via an aberrant response of the adrenal glands to gastric inhibitory polypeptide (GIP). We report here a novel case of food-dependent Cushing’s syndrome in a patient with bilateral macronodular adrenal hyperplasia. In this patient we were able to confirm a paradoxical stimulation of cortisol secretion by GIP in vivo as well as in vitro on dispersed tumor adrenal cells obtained at surgery. In addition to GIP, in vitro stimulation of these cultured tumor adrenal cells with leptin, the secreted product of the adipocyte, induced cortisol secretion. By comparison, no such stimulation was observed in vitro in adrenal cells obtained from another patient with bilateral macronodular adrenal hyperplasia and Cushing’s syndrome that did not depend on food intake, in tumor cells obtained from a solitary cortisol-secreting adrenal adenoma, and in normal human adrenocortical cells. These results demonstrate that as in previously described cases of food-dependent Cushing’s syndrome, GIP stimulated cortisol secretion from the adrenals of the patient reported here. Therefore, they indicate that such a paradoxical response probably represents the hallmark of this rare condition. In addition, they suggest that leptin, which normally inhibits stimulated cortisol secretion in humans, participated in cortisol hypersecretion in this case. Further studies in other cases of food-dependent Cushing’s syndrome, however, will be necessary to better ascertain the pathophysiological significance of this finding.

scholarly journals Adrenal Cushing’s syndrome during pregnancy

2017 ◽  
Vol 177 (5) ◽  
pp. K13-K20 ◽  
Author(s):  
C E Andreescu ◽  
R A Alwani ◽  
J Hofland ◽  
L H J Looijenga ◽  
W W de Herder ◽  
...  
Keyword(s):  
Luteinizing Hormone ◽  
Cushing’S Syndrome ◽  
Adrenal Tumor ◽  
Cushing Syndrome ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Receptor Expression ◽  
Aberrant Expression ◽  
Cortisol Responses

Cushing syndrome (CS) during pregnancy is a rare condition with only a few cases reported in the literature. Misdiagnosis of CS is common because of overlapping features like fatigue, weight gain, striae and emotional changes that can occur during normal pregnancy. Changes in maternal hormones and their binding proteins complicate assessment of glucocorticoid hormone levels during gestation. CS during pregnancy is most frequently due to an adrenal adenoma and to a lesser degree to adrenocorticotropic hormone (ACTH) hypersecretion by a pituitary adenoma. Furthermore, aberrant expression of luteinizing hormone (LH) receptors in the adrenal cortex has been suggested to be involved in the pathogenesis of adrenal CS during pregnancy. We report three pregnant women with ACTH-independent Cushing’s syndrome and an adrenal tumor. After uncomplicated delivery, patient 1 underwent in vivo testing for aberrant hormone receptor expression by the adenoma. Cortisol responses were found after administration of luteinizing hormone-releasing hormone (LHRH), human chorionic gonadotropin (hCG), glucagon, vasopressin and a standard mixed meal. All patients were treated with laparoscopic adrenalectomy. Adrenal tumor tissue of two patients showed positive immunohistochemical staining of LH receptors. Considering the cortisol responses to LHRH and hCG, and the development of CS during pregnancy in these patients, it is likely that ACTH-independent hypercortisolism was induced by the pregnancy-associated rise in hCG levels that activated aberrantly expressed LH receptors in the adrenal adenoma. Remarkably, adrenal adenomas may simultaneously express multiple aberrant receptors and individual ligands may play a role in the regulation of cortisol production in CS during pregnancy.

scholarly journals Unilateral nodular adrenocortical hyperplasia presenting as Cushing’s syndrome: a case report

2021 ◽  
Vol 12 (1) ◽  
pp. 78-82
Author(s):  
Lubna Naznin ◽  
Susane Giti ◽  
SK Md Jaynul Islam ◽  
Khandaker Rokshana Akhter ◽  
Shamoli Yasmin ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Histopathological Examination ◽  
Case Reports ◽  
Adrenal Adenoma ◽  
Final Diagnosis ◽  
Cushing's Syndrome ◽  
Uncontrolled Hypertension ◽  
Secondary Hypothyroidism ◽  
Adrenocortical Hyperplasia

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

Adrenocortical adenoma in a six-year-old Egyptian boy

2021 ◽  
Vol 2 (2) ◽  
Author(s):  
Eman B Kamaleldeen ◽  
◽  
Shimaa Kamal Mohamed ◽  
Kotb Abbass Metwalley ◽  
Hamdy M Ibrahim ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Histopathological Examination ◽  
Adrenal Adenoma ◽  
Time Lag ◽  
Laboratory Data ◽  
Clinical Signs ◽  
Cushing's Syndrome ◽  
Additional Treatment ◽  
Adrenocortical Adenoma

Although adrenal cortical tumors are rare in children, pediatricians should be very cautious about it. Neoplasm is a rare but significant cause of Cushing’s syndrome. Unfortunately, they can be missed very easily, as there is often a time lag between the onset of the symptoms and final diagnosis. Here, we present a case of adrenal adenoma in a six-year - old Egyptian boy who regrettably misdiagnosed twice as exogenous Cushing syndrome and simple obesity. Diagnosis of an adrenal cortical tumor was confirmed by laboratory data, MRI and histopathological examination after more than 2 years of the onset of his symptoms. After resection of the tumor, serum steroids normalized, and clinical signs receded. The child received no additional treatment and remains disease-free after 18 months of close observation. The possibility of neoplasms should always be considered early to avoid delayed diagnosis and treatment of Cushing’s syndrome.

scholarly journals Asynchronous Development of Bilateral Nodular Adrenal Hyperplasia in Gastric Inhibitory Polypeptide-Dependent Cushing’s Syndrome1

1999 ◽  
Vol 84 (8) ◽  
pp. 2616-2622 ◽  
Author(s):  
Nina N’Diaye ◽  
Pavel Hamet ◽  
Johanne Tremblay ◽  
Jean-Marie Boutin ◽  
Louis Gaboury ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Plasma Cortisol ◽  
Adrenal Adenoma ◽  
Gastric Inhibitory Polypeptide ◽  
Cushing's Syndrome ◽  
Early Event ◽  
Oral Glucose ◽  
Adrenal Hyperplasia ◽  
Messenger Ribonucleic Acid ◽  
The Right

Gastric inhibitory polypeptide (GIP)-dependent Cushing’s syndrome has been reported to occur either in unilateral adrenal adenoma or in bilateral macronodular adrenal hyperplasia. A 33-yr-old woman with Cushing’s syndrome was found to have two 2.5- to 3-cm nodules in the right adrenal on computed tomography scan; the left adrenal appeared normal except for the presence of a small 0.8 × 0.6-cm nodule. Uptake of iodocholesterol was limited to the right adrenal. Plasma morning cortisol was 279 nmol/L fasting and 991 nmol/L postprandially, and ACTH remained suppressed. Plasma cortisol increased after oral glucose (202%) or a lipid-rich meal (183%), but not after a protein-rich meal (95%) or iv glucose (93%); the response to oral glucose was blunted by pretreatment with 100 μg octreotide, sc. Plasma cortisol and GIP levels were positively correlated (r = 0.95; P = 0.0001); cortisol was stimulated by the administration of human GIP iv (225%), but not by GLP-1, insulin, TRH, GnRH, glucagon, arginine vasopressin, upright posture, or cisapride orally. A right adrenalectomy was performed; GIP receptor messenger ribonucleic acid was overexpressed in both adrenal nodules and in the adjacent cortex. Histopathology revealed diffuse macronodular adrenal hyperplasia without internodular atrophy. Three months after surgery, fasting plasma ACTH and cortisol were suppressed, but cortisol increased 3.6-fold after oral glucose, whereas ACTH remained suppressed; this was inhibited by octreotide pretreatment, suggesting that cortisol secretion by the left adrenal is also GIP dependent. We conclude that GIP-dependent nodular hyperplasia can progress in an asynchronous manner and that GIPR overexpression is an early event in this syndrome.

Serum profiles of steroid hormones in patients with Cushing's syndrome determined by a new HPLC/RIA method

1991 ◽  
Vol 37 (8) ◽  
pp. 1329-1333 ◽  
Author(s):  
H Ueshiba ◽  
M Segawa ◽  
T Hayashi ◽  
Y Miyachi ◽  
M Irie
Keyword(s):  
Steroid Hormones ◽  
Cushing’S Syndrome ◽  
High Performance ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Serum Concentrations ◽  
Adrenal Hyperplasia ◽  
Steroid Profile ◽  
17 Hydroxyprogesterone ◽  
Normal Adults

Abstract We developed a method for simultaneously measuring steroid hormones in very small volumes of serum, using a combination of high-performance liquid chromatography (HPLC) and radioimmunoassay (RIA). By this method, aldosterone, cortisol, 11-deoxycortisol, estrone, estradiol, androstenedione, dehydroepiandrosterone, deoxycorticosterone, 17-hydroxyprogesterone, testosterone, pregnenolone, and progesterone could be determined in a single 100-microL aliquot of serum from normal adults and patients with Cushing's syndrome. The steroid profile associated with Cushing's syndrome caused by adrenal adenoma was quite distinct from that associated with the syndrome caused by adrenal hyperplasia. Serum concentrations of androstenedione, dehydroepiandrosterone, estrone, estradiol, 17-hydroxyprogesterone, pregnenolone, and testosterone were significantly higher in patients with adrenal hyperplasia than in those with an adenoma. We compared the results of this HPLC/RIA method with those of 125I RIAs. The use of a HPLC/RIA system to obtain an accurate and sensitive profile of a range of serum steroids, as described here, obviates the need for large volumes of blood.

DIAGNOSIS OF CUSHING'S SYNDROME

1969 ◽  
Vol 60 (1) ◽  
pp. 47-59 ◽  
Author(s):  
H. Bethge ◽  
M. Bayer ◽  
W. Winkelmann
Keyword(s):  
Cushing’S Syndrome ◽  
Opposite Effect ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Adrenal System ◽  
Lysine Vasopressin ◽  
Normal Increase ◽  
High Degree

ABSTRACT Adrenal function tests were performed in 9 patients with Cushing's syndrome (6 with adrenal hyperplasia and 3 with adrenal adenoma) in order to study the hypothalamus-pituitary-adrenal system. These tests involved the determination of a) diurnal rhythm of plasma corticosteroids (11-OHCS) and the effect of corticotrophin (ACTH) and dexamethasone administration, b) urinary corticosteroid excretion (17-OHCS), basal and after ACTH, dexamethasone and metapyrapone treatment and c) cortisol production rate. In addition the patients were submitted to insulin-induced hypoglycaemia and intravenous infusion of synthetic lysine-vasopressin and the plasma corticosteroids were determined. All 9 patients regardless of the nature of adrenal pathology responded to insulin-induced hypoglycaemia in the same manner and failed to show the normal increase of 11-OHCS. During lysine-vasopressin infusion an opposite effect was observed: all 6 patients with hyperplasia showed a distinct rise in plasma corticosteroids in contrast to the 3 patients with adenoma who did not respond at all. These findings show that the lysine-vasopressin test may be very useful in differentiating between adrenal hyperplasia and adrenal adenoma. This procedure is recommended as a screening test because of its simplicity and high degree of diagnostic accuracy. Results are discussed with regard to the different pathogenesis of the two forms of Cushing's syndrome.

Panel Discussion

PEDIATRICS ◽  
1949 ◽  
Vol 3 (4) ◽  
pp. 515-548
Author(s):  
A. WILMOT JACOBSEN ◽  
GEORGE F. KOEPF ◽  
NATHAN B. TALBOT ◽  
LAWSON WILKINS
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Cushing’S Syndrome ◽  
Panel Discussion ◽  
Cushing's Syndrome ◽  
Urogenital Sinus ◽  
Adrenal Tumors ◽  
Postnatal Life ◽  
Adrenal Hyperplasia ◽  
Adrenogenital Syndrome ◽  
Acute And Chronic Stress

The role of the adrenal cortex in resistance to stress and strain has been reviewed. Certain experimental data indicating increased secretion of the adrenal cortical hormone in acute and chronic stress has been presented and discussed in terms of Sayers' homeostatic theory of the acute stress mechanism and in terms of Selye's "Adaptation Syndrome." In addition, studies made on a patient with acute rheumatic fever were discussed. Changes in uric acid metabolism compatible with those that might be seen in an alarm reaction were noted in this patient. [SEE THE TABLE I IN SOURCE PDF]. In the adrenogenital syndrome there is increased protein anabolism due to excessive androgen, in Cushing's syndrome inhibition of protein anabolism due to excessive gluconeogenetic hormones. The manifestations of the adrenogenital syndrome differ according to the patient's sex and the age of onset. Congenital adrenal hyperplasia causes pseudohermaphroditism in the female. This can be differentiated from various types of genetic intersexuality by the evidences of excessive androgen from infancy on and by a fairly uniform type of heterosexual development with a persistent urogenital sinus. In the male congenital adrenal hyperplasia causes macrogenitosomia precox which is sometimes accompanied by Addisonian-like symptoms. This can be differentiated from other types of male precocity by the excessive excretion of androgen and lack of maturation of the testes. In postnatal life androgenic adrenal tumors or hyperplasia cause virilization in both sexes unaccompanied by embryonic abnormalities. Occasionally gynecomastia occurs in males. Most of the symptoms of Cushing's syndrome are probably caused by excess of the gluconeogenetic hormones. However, there is also usually an increased secretion of androgen and in some cases evidences of increased electrolyte-controlling hormones.

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