scholarly journals An unusual case of shortness of breath

2018 ◽  
Vol 2018 ◽  
Author(s):  
S F Wan Muhammad Hatta ◽  
L Kandaswamy ◽  
C Gherman-Ciolac ◽  
J Mann ◽  
H N Buch
Keyword(s):  
Respiratory Failure ◽  
Cushing’S Syndrome ◽  
Lower Limb ◽  
Respiratory Muscle ◽  
Cushing's Syndrome ◽  
Primary Hypothyroidism ◽  
List Type ◽  
Biliary Cirrhosis ◽  
Shortness Of Breath ◽  
Proximal Lower Limb

Summary Myopathy is a well-known complication of hypercortisolism and commonly involves proximal lower-limb girdle. We report a rare case of Cushing’s syndrome in a 60-year-old female presenting with significant respiratory muscle weakness and respiratory failure. She had history of rheumatoid arthritis, primary biliary cirrhosis and primary hypothyroidism and presented with weight gain and increasing shortness of breath. Investigations confirmed a restrictive defect with impaired gas transfer but with no significant parenchymatous pulmonary disease. Respiratory muscle test confirmed weakness of respiratory muscles and diaphragm. Biochemical and radiological investigations confirmed hypercortisolaemia secondary to a left adrenal tumour. Following adrenalectomy her respiratory symptoms improved along with an objective improvement in the respiratory muscle strength, diaphragmatic movement and pulmonary function test. Learning points: Cushing’s syndrome can present in many ways, a high index of suspicion is required for its diagnosis, as often patients present with only few of the pathognomonic symptoms and signs of the syndrome. Proximal lower-limb girdle myopathy is common in Cushing’s syndrome. Less often long-term exposure of excess glucocorticoid production can also affect other muscles including respiratory muscle and the diaphragm leading to progressive shortness of breath and even acute respiratory failure. Treatment of Cushing’s myopathy involves treating the underlying cause that is hypercortisolism. Various medications have been suggested to hinder the development of GC-induced myopathy, but their effects are poorly analysed.

scholarly journals Spinal epidural lipomatosis: a rare association of Cushing’s disease

2020 ◽  
Vol 2020 ◽  
Author(s):  
Sajjad Ahmad ◽  
Thomas Best ◽  
Andrew Lansdown ◽  
Caroline Hayhurst ◽  
Fiona Smeeton ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing’S Disease ◽  
Lower Limb ◽  
Spinal Canal ◽  
Cushing's Syndrome ◽  
Cushing's Disease ◽  
List Type ◽  
Spinal Epidural Lipomatosis ◽  
Epidural Lipomatosis ◽  
Spinal Epidural

Summary Excess cortisol is associated with hypertrophy and redistribution of adipose tissue leading to central obesity which is classically seen in Cushing’s syndrome. Abnormal accumulation of fatty tissue in the spinal canal is most commonly associated with chronic steroid therapy and rarely reported with endogenous Cushing’s syndrome. Herein, we describe a case of spinal epidural lipomatosis (SEL) associated with Cushing’s disease. A 17-year-old man was referred with lower limb weakness, weight gain, multiple stretch marks, back pain and loss of height. He had clinical and biochemical features of Cushing’s syndrome. MRI and Inferior Petrosal Sinus Sampling (IPSS) confirmed a pituitary adenoma as the source. On day 1 post trans-sphenoidal adenectomy he developed spastic paraparesis with a sensory deficit to the level of T5. MRI spine showed increased fat deposition in the spinal canal from T2 to T9 consistent with a diagnosis of SEL. He was managed conservatively and made a good recovery following restoration of eucortisolism and a period of rehabilitation. Learning points: SEL is a serious complication of glucocorticoid excess and should be considered in any patient presenting with new lower limb neurological symptoms associated with hypercortisolism. It is important to distinguish symptomatic SEL from cortisol-induced proximal myopathy by good history and clinical examination. MRI of the spine is the gold standard investigation for making a diagnosis of SEL. Restoration of eucortisolism can lead to resolution of fat accumulation and good neurological outcome.

scholarly journals Long-term low-dose ketoconazole treatment in bilateral macronodular adrenal hyperplasia

2014 ◽  
Vol 2014 ◽  
Author(s):  
Sophie Comte-Perret ◽  
Anne Zanchi ◽  
Fulgencio Gomez
Keyword(s):  
Medical Therapy ◽  
Cushing’S Syndrome ◽  
Clinical Signs ◽  
Cushing's Syndrome ◽  
List Type ◽  
Adrenal Hyperplasia ◽  
Benign Condition ◽  
Steroid Secretion ◽  
Free Cortisol

Summary Medical therapy for Cushing's syndrome due to bilateral macronodular adrenal hyperplasia (BMAH) is generally administered for a limited time before surgery. Aberrant receptors antagonists show inconsistent efficacy in the long run to prevent adrenalectomy. We present a patient with BMAH, treated for 10 years with low doses of ketoconazole to control cortisol secretion. A 48-year-old woman presented with headaches and hypertension. Investigations showed the following: no clinical signs of Cushing's syndrome; enlarged lobulated adrenals; normal creatinine, potassium, and aldosterone; normal urinary aldosterone and metanephrines; elevated urinary free cortisol and steroid metabolites; and suppressed plasma renin activity and ACTH. A screening protocol for aberrant adrenal receptors failed to show any illegitimate hormone dependence. Ketoconazole caused rapid normalisation of cortisol and ACTH that persists over 10 years on treatment, while adrenals show no change in shape or size. Ketoconazole decreases cortisol in patients with Cushing's syndrome, and may prevent adrenal overgrowth. Steroid secretion in BMAH is inefficient as compared with normal adrenals or secreting tumours and can be controlled with low, well-tolerated doses of ketoconazole, as an alternative to surgery. Learning points Enlarged, macronodular adrenals are often incidentally found during the investigation of hypertension in patients harboring BMAH. Although laboratory findings include low ACTH and elevated cortisol, the majority of patients do not display cushingoid features. Bilateral adrenalectomy, followed by life-long steroid replacement, is the usual treatment of this benign condition, and alternative medical therapy is sought. Therapy based on aberrant adrenal receptors gives disappointing results, and inhibitors of steroidogenesis are not always well tolerated. However, ketoconazole at low, well-tolerated doses appeared appropriate to control adrenal steroid secretion indefinitely, while preventing adrenal overgrowth. This treatment probably constitutes the most convenient long-term alternative to surgery.

scholarly journals Reversible alterations in cardiac morphology and functions in a patient with Cushing's syndrome

2014 ◽  
Vol 2014 ◽  
Author(s):  
Hiroaki Iwasaki
Keyword(s):  
Cushing’S Syndrome ◽  
Interventricular Septum ◽  
Urinary Free Cortisol ◽  
Cushing's Syndrome ◽  
Left Ventricular ◽  
Multidetector Row Computed Tomography ◽  
List Type ◽  
Adrenocortical Adenoma ◽  
Cardiac Morphology ◽  
Free Cortisol

Summary A 45-year-old female was referred for endocrine evaluation of an incidental mass (31×24 mm in diameter) on the right adrenal gland. The patient was normotensive and nondiabetic, and had no history of generalised obesity (body weight, 46 kg at 20 years of age and 51.2 kg on admission); however, her waist-to-hip ratio was 0.97. Elevated urinary free cortisol levels (112–118 μg/day) and other findings indicated adrenocorticotrophic hormone-independent Cushing's syndrome due to right adrenocortical adenoma. Echocardiography before adrenalectomy revealed concentric left ventricular (LV) hypertrophy with a particular increase in interventricular septum thickness leading to impaired systolic and diastolic functions. Upon surgical remission of hypercortisolism, the asymmetric hypertrophy disappeared and the cardiac dysfunctions were considerably ameliorated. Although the mechanism(s) by which excessive cortisol contributes to LV wall thickness remain(s) unclear, serial echocardiography and cardiac multidetector-row computed tomography may support the notion that abnormal fat deposition in the myocardium owing to hypercortisolism appears to be an important factor for the reversible change in the cardiac morphology. Learning points Patients with Cushing's syndrome occasionally exhibit severe LV hypertrophy related to systolic and diastolic dysfunctions although they have neither hypertension nor diabetes mellitus. Biological remission of hypercortisolism can normalise structural and functional cardiac parameters and help in differentiating the cardiac alterations induced by excessive cortisol from those induced by other diseases. Excessive lipid accumulation within the heart before myocardial fibrosis may be implicated in reversible alterations in the cardiac morphology by Cushing's syndrome. Early diagnosis and treatment of Cushing's syndrome appear to be pivotal in preventing irreversible cardiac dysfunctions subsequent to cardiovascular events and heart failure.

scholarly journals Rapid control of ectopic Cushing’s syndrome during the COVID-19 pandemic in a patient with chronic hypokalaemia

2021 ◽  
Vol 2021 ◽  
Author(s):  
Ziadoon Faisal ◽  
Miguel Debono
Keyword(s):  
Type 2 Diabetes ◽  
Case Report ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Alternative Methods ◽  
Ectopic Acth Syndrome ◽  
High Dose ◽  
List Type ◽  
Ectopic Acth

Summary In this case report, we describe the management of a patient who was admitted with an ectopic ACTH syndrome during the COVID pandemic with new-onset type 2 diabetes, neutrophilia and unexplained hypokalaemia. These three findings when combined should alert physicians to the potential presence of Cushing’s syndrome (CS). On admission, a quick diagnosis of CS was made based on clinical and biochemical features and the patient was treated urgently using high dose oral metyrapone thus allowing delays in surgery and rapidly improving the patient’s clinical condition. This resulted in the treatment of hyperglycaemia, hypokalaemia and hypertension reducing cardiovascular risk and likely risk for infection. Observing COVID-19 pandemic international guidelines to treat patients with CS has shown to be effective and offers endocrinologists an option to manage these patients adequately in difficult times. Learning points This case report highlights the importance of having a low threshold for suspicion and investigation for Cushing’s syndrome in a patient with neutrophilia and hypokalaemia, recently diagnosed with type 2 diabetes especially in someone with catabolic features of the disease irrespective of losing weight. It also supports the use of alternative methods of approaching the diagnosis and treatment of Cushing’s syndrome during a pandemic as indicated by international protocols designed specifically for managing this condition during Covid-19.

Unexpected shortness of breath in a patient with Cushing's syndrome

The Lancet ◽  
2006 ◽  
Vol 367 (9508) ◽  
pp. 446 ◽  
Author(s):  
Niall Keenan ◽  
Waljit S Dhillo ◽  
Graham R Williams ◽  
Jeannie F Todd
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Shortness Of Breath

EFFECT OF METOPIRONE IN A PATIENT WITH ADRENAL CORTICAL CARCINOMA

1963 ◽  
Vol 44 (3) ◽  
pp. 346-354 ◽  
Author(s):  
H. Daniels ◽  
W. J. van Amstel ◽  
W. Schopman ◽  
C. van Dommelen
Keyword(s):  
Cushing’S Syndrome ◽  
Clinical Effect ◽  
Cushing's Syndrome ◽  
Adrenal Cortical Carcinoma ◽  
List Type ◽  
Adrenal Carcinoma ◽  
Glucocorticoid Production ◽  
Steroid Excretion

ABSTRACT The effect of metopirone® (SU-4885)* in a patient with metastatic adrenal cortical carcinoma was studied. The intention was to depress the symptoms of Cushing's syndrome, due to a high glucocorticoid production by the carcinoma. The excretion of 17-KS, 17-KGS, 11-oxo(y)-17-OHCS and 11-desoxy-17-OHCS was followed for seven weeks. The steroid excretion was variable. The 11-oxo(y)-17-OHCS showed a significant decrease during treatment with SU-4885 for seven weeks. The ratio 11-oxo(y)-17-OHCS: 11-desoxy-17-OHCS fell from 1.7 to 0.1 during this period. The excessive glucocorticoid production of the metastatic adrenal carcinoma was suppressed as long as treatment continued. It was not possible to judge the clinical effect of SU-4885 because of the progressive nature of the disease.

scholarly journals Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma

2013 ◽  
Vol 2013 ◽  
Author(s):  
F Serra ◽  
S Duarte ◽  
S Abreu ◽  
C Marques ◽  
J Cassis ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Microscopic Analysis ◽  
Cushing's Syndrome ◽  
Ectopic Acth Syndrome ◽  
List Type ◽  
Rare Tumour ◽  
Ectopic Acth ◽  
Ectopic Acth Production ◽  
The Right

Summary Ectopic secretion of ACTH is an infrequent cause of Cushing's syndrome. We report a case of ectopic ACTH syndrome caused by a nasal paraganglioma, a 68-year-old female with clinical features of Cushing's syndrome, serious hypokalaemia and a right paranasal sinus' lesion. Cranial magnetic resonance image showed a 46-mm mass on the right paranasal sinuses. Endocrinological investigation confirmed the diagnosis of ectopic ACTH production. Resection of the tumour normalised ACTH and cortisol secretion. The tumour was found to be a paraganglioma through microscopic analysis. On follow-up 3 months later, the patient showed nearly complete clinical recovery. Ectopic ACTH syndrome due to nasal paraganglioma is extremely uncommon, as only two other cases have been discussed in the literature. Learning points Ectopic Cushing's syndrome accounts for 10% of Cushing's syndrome etiologies. Most paraganglioma of the head and neck are not hormonally active. Nasal paraganglioma, especially ACTH producing, is a very rare tumour.

scholarly journals Spontaneous resolution of avascular necrosis of femoral heads following cure of Cushing’s syndrome

2016 ◽  
Vol 2016 ◽  
Author(s):  
A Pazderska ◽  
S Crowther ◽  
P Govender ◽  
K C Conlon ◽  
M Sherlock ◽  
...  
Keyword(s):  
Femoral Head ◽  
Avascular Necrosis ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Leg Pain ◽  
List Type ◽  
Rapid Weight Gain ◽  
Free Cortisol ◽  
Femoral Heads ◽  
Adrenal Disease

Summary Avascular necrosis (AVN) is a rare presenting feature of endogenous hypercortisolism. If left untreated, complete collapse of the femoral head may ensue, necessitating hip replacement in up to 70% of patients. The majority of the described patients with AVN due to endogenous hypercortisolaemia required surgical intervention. A 36-year-old female, investigated for right leg pain, reported rapid weight gain, bruising and secondary amenorrhoea. She had abdominal adiposity with violaceous striae, facial plethora and hirsutism, atrophic skin, ecchymosis and proximal myopathy. Investigations confirmed cortisol excess (cortisol following low-dose 48h dexamethasone suppression test 807nmol/L; 24h urinary free cortisol 1443nmol (normal<290nmol)). Adrenocorticotrophic hormone (ACTH) was <5.0pg/mL. CT demonstrated subtle left adrenal gland hypertrophy. Hypercortisolaemia persisted after left adrenalectomy. Histology revealed primary pigmented micronodular adrenal disease. Post-operatively, right leg pain worsened and left leg pain developed, affecting mobility. MRI showed bilateral femoral head AVN. She underwent right adrenalectomy and steroid replacement was commenced. Four months after surgery, leg pain had resolved and mobility was normal. Repeat MRI showed marked improvement of radiological abnormalities in both femoral heads, consistent with spontaneous healing of AVN. We report a case of Cushing’s syndrome due to primary pigmented nodular adrenocortical disease, presenting with symptomatic AVN of both hips. This was managed conservatively from an orthopaedic perspective. Following cure of hypercortisolaemia, the patient experienced excellent recovery and remains symptom free 4 years after adrenalectomy. This is the first report of a favourable outcome over long-term follow-up of a patient with bilateral AVN of the hip, which reversed with treatment of endogenous hypercortisolaemia. Learning points AVN of femoral head can be a presenting feature of hypercortisolism, both endogenous and exogenous. Rarely, treatment of hypercortisolaemia can reverse AVN without the need for orthopaedic intervention. Primary pigmented nodular adrenal disease is a rare cause of ACTH-independent Cushing’s syndrome.

scholarly journals Factitious Cushing’s syndrome, hypopituitarism, and self-provoked skin lesions: when the skin mirrors the soul

2021 ◽  
Vol 2021 ◽  
Author(s):  
Salvatore Cannavò ◽  
Serafinella Patrizia Cannavò
Keyword(s):  
Cushing’S Syndrome ◽  
Multidisciplinary Approach ◽  
Hypogonadotropic Hypogonadism ◽  
Serum Cortisol ◽  
Skin Lesions ◽  
Cushing's Syndrome ◽  
List Type ◽  
Pituitary Mass ◽  
Environmental Investigation ◽  
Cortisol Levels

Summary Factitious Cushing’s syndrome (CS) is a very rare form of Münchausen syndrome. Its presentation and course are extremely heterogeneous, and diagnosis is generally challenging. We report the case of a 52-year-old woman who was initially investigated because of the occurrence of cushingoid features. Nevertheless, endocrine work-up showed very low morning plasma ACTH and serum cortisol levels. In addition, it also demonstrated central hypopituitarism and hypogonadotropic hypogonadism. Head MRI showed a small pituitary mass. Based on these results, and probably overlooking the initial clinical suspicion, general practitioner (GP) referred the patient to our Endocrine Unit for hypopituitarism. At inspection, moon face, central obesity, and bruising were evident. Multiple ulcerative skin lesions were also concentrated in the right arm and leg. Dermatology evaluation suggested that the lesions were self-provoked. For several days, the patient denied the assumption of corticosteroids, but we finally discovered that the GP’ nurse had prescribed betamethasone without the GP’s knowledge for about 2 years. In conclusion, the surreptitious assumption of corticosteroids is very rare, but the physicians should be aware that pituitary function could be impaired by high doses of corticosteroids, mimicking hypopituitarism. In these patients, a multidisciplinary approach and environmental investigation can be useful to diagnose factitious CS. Learning points Surreptitious assumption of corticosteroids can cause heterogeneous presentation, ranging from Cushing’s syndrome to multiple hypopituitarism. Suppression of ACTH and cortisol levels in a patient with cushingoid features firstly suggests surreptitious assumption of corticosteroids. A multidisciplinary approach can be extremely useful in patients with suspected factitious Cushing’s syndrome. Sometimes, to prove surreptitious assumption of corticosteroids needs environmental investigation.

BLOOD CORTICOTROPHIN IN NORMAL ADULTS AND IN PATIENTS WITH CUSHING'S SYNDROME

1964 ◽  
Vol 45 (1) ◽  
pp. 55-67 ◽  
Author(s):  
Beryl M. A. Davies
Keyword(s):  
Ascorbic Acid ◽  
Cushing’S Syndrome ◽  
Human Blood ◽  
Pituitary Tumour ◽  
Cushing's Syndrome ◽  
List Type ◽  
Adrenal Hyperplasia ◽  
Bank Blood ◽  
Normal Adults ◽  
Satisfactory Recovery

ABSTRACT An oxycellulose adsorption-HC1 elution procedure has been used to concentrate corticotrophin (ACTH) from about 1000 ml normal pooled human blood or 500 ml blood from patients with Cushing's syndrome. The concentrates were bioassayed by measuring their adrenal ascorbic acid depleting action in cortisol-treated rats. Satisfactory recovery figures were obtained for the concentration of 2nd International Standard for ACTH added to out-of-date blood-bank blood or to blood from a hypophysectomized patient. Normal blood ACTH levels are reported from 8 estimations each on pooled blood from 2–3 individuals. Mean normal ACTH was 0.7 mU per 100 ml blood (range 0.60–1.2 mU). Eleven patients with Cushing's syndrome were investigated. These included non-treated cases with adrenal hyperplasia and post-adrenalectomy cases with recurrence of Cushingoid symptoms in the presence of a pituitary tumour. In both groups, mean blood ACTH values were three to four times normal.

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