scholarly journals The Improvement in Hepatic Steatosis After Cushing’s Syndrome Treatment Is an Early Sign of Metabolic Recovery

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A98-A98
Author(s):  
Ahmed Hamimi ◽  
Zahraa Abdul Sater ◽  
Raven McGlotten ◽  
Jatin Matta ◽  
Annie Pierce ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Successful Treatment ◽  
Cushing Syndrome ◽  
Primary Outcome Measure ◽  
Cushing's Syndrome ◽  
Standard Test ◽  
Liver Fat ◽  
Rank Test ◽  
Resonance Spectroscopy ◽  
Metabolic Markers

Abstract Context: Cushing Syndrome (CS) is characterized by cortisol excess, impaired glucose tolerance, and obesity. As assessed by CT imaging, 20% of CS patients develop steatohepatitis (NASH). The gold standard test, liver biopsy, is associated with CS complications and cannot be used to confirm the diagnosis. This study evaluated the ability of magnetic resonance spectroscopy (MRS). Objective: To identify the prevalence of NASH and its temporal changes in relation to other metabolic parameters in CS before and after successful treatment. Primary Outcome Measure: PDFF measured by MRS at 3T before, 6 and 12 months after Cushing’s syndrome treatment DESIGN: In this prospective IRB-approved study, 41 consecutive CS patients (44±1.8 y; 34(85%) females, 32.6±1.5 kg/m²; urine cortisol excretion 2242.7±1806.3 [3.5–45.0 mcg/24h]) underwent MRS before, 6 and 12months after successful treatment. PDFF was measured by MRS at 3T; NASH was defined as >5% PDFF. Metabolic markers – glycohemoglobin (A1C) and body mass index (BMI) – were measured; Wilcoxon matched-pairs signed-rank test evaluated changes over time, and spearman rank test evaluated the correlation between variables. Results: At baseline, mean PDFF was 10.4±1.7 and correlated positively with BMI (r=0.5710, p<0.0001). NASH was present in 32% of patients. After treatment, PDFF decreases were similar at 6 and 12 months (-52%, p=0.001 and -50%, p=0.02, respectively); rates of NASH declined to 13% and 11%. BMI decreased (-9%; p=0.0018 and -12%, p=0.0003) but without a statistically significant change overweight/obese status. A significant decrease in A1C followed at 12 months (-12%, p=0.0005). Conclusions: MRS-PDFF is valuable for diagnosing NASH in Cushing Syndrome, which can affect a third of this patient population. Liver fat decreases by 6 months after normalization of cortisol and precedes the improvement of A1C. Indicating that liver insulin resistance due to fat accumulation has an essential role in diabetes pathophysiology in CS.

CUSHING'S SYNDROME FOLLOWING PARTIAL ADRENALECTOMY AND COMPLETE HYPOPHYSECTOMY

1965 ◽  
Vol 48 (4) ◽  
pp. 565-568 ◽  
Author(s):  
W. A. Sullivan ◽  
Jerrold D. Hydovitz ◽  
H. Rosomoff ◽  
T. S. Danowski
Keyword(s):  
Cushing’S Syndrome ◽  
Adult Male ◽  
Successful Treatment ◽  
Cushing's Syndrome ◽  
Partial Adrenalectomy ◽  
The Third ◽  
Subtotal Adrenalectomy ◽  
Pituitary Ablation ◽  
Acth Secreting

ABSTRACT A third episode of Cushing's syndrome developed in an adult male who had undergone subtotal adrenalectomy and then complete hypophysectomy in temporarily successful treatment of two earlier episodes. Judging from necropsy findings, this final recurrence was not the result of incomplete pituitary ablation or regeneration of pituitary remnants. Extrasellar pituitary or other ACTH secreting tissue could not be identified, but the patient's marked melanosis suggested its presence with consequent excesses of ACTH or MSH. However, irrespective of whether such excesses did or did not exist, it is clear that the third episode of Cushing's syndrome resulted from hyperfunction of the small adrenal remnant.

scholarly journals Frequency of Osteoporosis in Iatrogenic Cushing’s syndrome: Scenario of outpatient department in urban hospitals

2020 ◽  
Vol 31 (1) ◽  
pp. 18-21
Author(s):  
Tamanna Bahar ◽  
Shaila Rahman ◽  
Lilian Catherene Gomes ◽  
Mohammad Murad Hossain ◽  
Zulfia Zinat Chowdhury ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Reference Population ◽  
Final Diagnosis ◽  
Cushing's Syndrome ◽  
Outpatient Department ◽  
Clinical Feature ◽  
Cross Sectional ◽  
Prevalence Of Osteoporosis ◽  
Iatrogenic Cushing’S Syndrome

Background: Cushing’s syndrome is caused by excessive activation of glucocorticoid receptor. Iatrogenic Cushing syndrome is the prevalent one world -wide .Patients with Cushing’s syndrome has a high prevalence of osteoporosis. Objective: To see the prevalence of osteoporosis in Iatrogenic Cushing’s syndrome patient. Method: This descriptive cross-sectional study included 211 diagnosed case of Cushing’s syndrome during the time period of December 2013 to December 2018 in outpatient department of Dhaka Medical College and Health and Hope Hospital. Final diagnosis was done on the basis of clinical feature, serum basal cortical level and BMD. We found male were prevalent one (56%).80.56% showed biochemical evidence of Cushing’s syndrome. BMD was done in 113 patients compared with a reference population by means of T score, 17.69% patient in osteoporotic range. Conclusion: The prevalence of osteoporosis and osteopenia is age and sex independent. Judicial use of steroid and co administration with calcium, bisphosphonate can prevent osteoporosis. Treatment with bisphosphonates should be considered in all patients (irrespective of age) with Cushing’s syndrome with a low BMD to reduce fracture. Bangladesh J Medicine Jan 2020; 31(1) : 18-21

A Novel GNAS Mutation Causing Isolated Infantile Cushing’s Syndrome

2019 ◽  
Vol 92 (3) ◽  
pp. 196-202
Author(s):  
Prapai Dejkhamron ◽  
Chupong Ittiwut ◽  
Hataitip TangNgam ◽  
Kanokkarn Sunkonkit ◽  
Rungrote Natesirinilkul ◽  
...  
Keyword(s):  
Fibrous Dysplasia ◽  
Precocious Puberty ◽  
Cushing’S Syndrome ◽  
De Novo ◽  
Cushing Syndrome ◽  
Skin Pigmentation ◽  
Cushing's Syndrome ◽  
Polyostotic Fibrous Dysplasia ◽  
Pneumocystis Jiroveci ◽  
Gnas Gene

Infantile Cushing’s syndrome is potentially found as part of McCune-Albright syndrome (MAS) which is caused by postzygotic somatic mutations of the GNAS gene. MAS is typically characterized by a triad of polyostotic fibrous dysplasia, café-au-lait skin pigmentation, and precocious puberty or other endocrine hyperfunction. Here, we describe a 2-month-old female infant with features of Cushing’s syndrome without café au lait spots, polyostotic fibrous dysplasia, and clinical evidence of other endocrine hyperfunction. Investigations demonstrated adrenocorticotropic hormone-independent Cushing’s syndrome with bilateral adrenal gland enlargement. Whole-exome sequencing of leukocytes identified a de novo heterozygous novel missense mutation (c.521G>A, p.Cys174Tyr) in the GNAS gene. The patient experienced clinical improvement of Cushing’s syndrome during ketoconazole treatment. Her clinical course was complicated by Pneumocystis jiroveci pneumonia. She also had shortened activated partial thromboplastin time indicating a hypercoagulable state and resulting in pulmonary embolism. She eventually manifested gonadotropin-independent precocious puberty at the age of 13 months after ketoco­nazole was discontinued. This patient demonstrated that Cushing syndrome can be the presenting sign of MAS in infancy. A high index of suspicion followed by genetic analysis is essential in order to establish a diagnosis.

scholarly journals Mania as Debut of Cushing’s Syndrome

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Ricardo Álvarez Martínez ◽  
Rosa María Tomé Rodríguez ◽  
María Álvarez Ariza ◽  
Carlos Spuch ◽  
Jose M. Olivares
Keyword(s):  
Cushing’S Syndrome ◽  
Clinical Condition ◽  
Psychiatric Symptoms ◽  
Cushing Syndrome ◽  
Cushing's Syndrome ◽  
Psychological Reaction

This is a case of a patient affected by Cushing syndrome that was admitted at the hospital due to hormonal problems. He had presented psychiatric symptoms that were mistakenly considered not directly connected to the pathology causing the clinical condition, but a mere psychological reaction to it.

scholarly journals The Hypothalamic-Pituitary-Thyroid Axis in Cushing Syndrome before and after Curative Surgery

2020 ◽  
Author(s):  
Skand Shekhar ◽  
Raven McGlotten ◽  
Sunyoung Auh ◽  
Kristina I Rother ◽  
Lynnette K Nieman
Keyword(s):  
Thyroid Function ◽  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Cushing's Syndrome ◽  
Curative Surgery ◽  
Central Hypothyroidism ◽  
Duration Of Symptoms ◽  
Before And After ◽  
Pituitary Thyroid Axis ◽  
Thyroid Axis

Abstract Background We do not fully understand how hypercortisolism causes central hypothyroidism or what factors influence recovery of the hypothalamic-pituitary-thyroid axis. We evaluated thyroid function during and after cure of Cushing’s syndrome (CS). Methods We performed a retrospective cohort study of adult patients with CS seen from 2005 – 2018 (cohort 1, c1, n=68) or 1985 – 1994 (cohort 2, c2, n=55) at a clinical research center. Urine (UFC) and diurnal serum cortisol (F: ~8AM and ~midnight (PM)), morning triiodothyronine (T3), free thyroxine (FT4) and thyroid stimulating hormone (TSH) (c1) or hourly TSH from 1500-1900h (day) and 2400-04000h (night) (c2), were measured before and after curative surgery. Results While hypercortisolemic, 53% of c1 had central hypothyroidism (low/low normal fT4 + unelevated TSH). Of those followed long-term, 31% and 44% had initially subnormal FT4 and T3, respectively, which normalized 6—12 months after cure. Hypogonadism was more frequent in hypothyroid (69%) compared to euthyroid (13%) patients. Duration of symptoms, AM and PM F, ACTH, and UFC were inversely related to TSH, FT4 and/or T3 levels (r -0.24 to -0.52, P <0.0001 to 0.02). In c2, the nocturnal surge of TSH (mIU/L) was subnormal before (day 1.00±0.04 vs night 1.08±0.05, p=0.3) and normal at a mean of 8 months after cure (day 1.30±0.14 vs night 2.17±0.27, p=0.01). UFC >1000 μg /day was an independent adverse prognostic marker of time to thyroid hormone recovery. Conclusions Abnormal thyroid function, likely mediated by subnormal nocturnal TSH, is prevalent in Cushing’s syndrome and is reversible after cure.

Cushing's syndrome and autonomous thyroid nodules, a variant of multiple endocrine neoplasia?

1986 ◽  
Vol 113 (3) ◽  
pp. 463-464 ◽  
Author(s):  
C. G. Semple ◽  
J. A. Thomson
Keyword(s):  
Thyroid Nodule ◽  
Cushing’S Syndrome ◽  
Multiple Endocrine Neoplasia ◽  
Thyroid Nodules ◽  
Cushing Syndrome ◽  
Cushing's Syndrome ◽  
Graves Disease ◽  
Endocrine Neoplasia ◽  
Solitary Thyroid Nodule ◽  
Autonomous Thyroid Nodule

Abstract. Over a 20 year period 4 of 40 (10%) female patients with Cushing' syndrome also had a solitary thyroid nodule. In 3 cases this was an autonomous 'hot' nodule. In the same population only one case of presumed Graves' disease was seen. It is postulated that the association of autonomous thyroid nodule and Cushing's syndrome may represent a variant of multiple endocrine neoplasia.

scholarly journals SAT-118 Rapid Decompensation from Complications of Severe Hypercortisolism in an Unusual Presentation of an Ectopic ACTH-Secreting Neuroendocrine Tumor

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Amit Kartar Singh Sumal ◽  
Charles Liao ◽  
Julie Chen
Keyword(s):  
Neuroendocrine Tumor ◽  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Pulseless Electrical Activity ◽  
Cushing's Syndrome ◽  
Bilateral Adrenalectomy ◽  
Unknown Primary ◽  
Who Grade ◽  
Ectopic Acth ◽  
Acth Secreting

Abstract Background: Ectopic Cushing’s syndrome from an ACTH-secreting neuroendocrine tumor (NET) is a rare condition whose onset and disease progression is often more aggressive than other forms of Cushing’s syndrome due to complications from severe hypercortisolism. Clinical Case: A 75-year old woman presented with profound proximal muscle weakness, severe hypokalemia, Cushingoid features, and biopsy-proven Candida esophagitis. Initial testing was consistent with ACTH-dependent Cushing syndrome: elevated 24 hour urinary cortisol excretion (1,310.54 mcg/24h; n <50 mcg/24h), abnormal 1 mg dexamethasone suppression test (68.3 ug/dL), and elevated ACTH level (200 pg/mL; n: 7.2–63.3 pg/mL). MRI was negative for a pituitary lesion but abdominal CT revealed an 8.8 cm liver mass with biopsy consistent with a well-differentiated neuroendocrine tumor, WHO Grade 2. Subsequent 68Ga-DOTATATE-PET/CT noted DOTATATE uptake in the liver lesion, a 0.9 cm right pulmonary nodule, and the pancreatic tail without CT correlate. Initially, the patient was prescribed mifepristone and spironolactone for hypokalemia. Given her NET of unknown primary, metastatic disease, and immunocompromised state due to hypercortisolism, the patient was not a candidate for surgical resection of her NET but was instead referred for bilateral adrenalectomy. However, she rapidly decompensated from complications of her hypercortisolism prior to surgery. Her weakness progressed to immobility, and she developed acute psychosis manifested as agitation and mutism. The patient was immediately admitted to the hospital where she developed new-onset atrial flutter and myelosuppression requiring multiple transfusions. She underwent urgent bilateral adrenalectomy, but despite surgery, her post-operative course was complicated by hypoxemic respiratory failure and shock. The patient shortly thereafter expired from pulseless electrical activity arrest. Conclusion: This atypical case of an ectopic ACTH-secreting NET highlights the life-threatening complications associated with severe hypercortisolism, including: opportunistic infection, severe metabolic abnormalities, psychosis, myopathy, and critical illness that can incite myelosuppression and unstable arrhythmias. These patients can quickly deteriorate and are at high risk for mortality. Early diagnosis and swift reversal of their hypercortisolism with bilateral adrenalectomy are oftentimes needed to prevent these potentially fatal complications.

scholarly journals Predictive Accuracy of Delta Cortisol on Recovery of Adrenal Function in Patients With Drug Induced Cushing’s Syndrome With Secondary Adrenal Insufficiency

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A91-A91
Author(s):  
Bo Bo San
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Adrenal Function ◽  
Drug Induced ◽  
Basal Serum ◽  
Synacthen Test ◽  
Short Synacthen Test

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.

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