scholarly journals A Case of Cushing's Syndrome Associated with Chronic Respiratory Failure Due to Metabolic Alkalosis.

1992 ◽  
Vol 31 (3) ◽  
pp. 385-390 ◽  
Author(s):  
Masako TANAKA ◽  
Takafumi Yano ◽  
Yoichiro ICHIKAWA ◽  
Kotaro OIZUMI
Keyword(s):  
Respiratory Failure ◽  
Cushing’S Syndrome ◽  
Metabolic Alkalosis ◽  
Cushing's Syndrome ◽  
Chronic Respiratory Failure

Ketoconazole in the management of paraneoplastic Cushing's syndrome secondary to ectopic adrenocorticotropin production.

1995 ◽  
Vol 13 (1) ◽  
pp. 157-164 ◽  
Author(s):  
E W Winquist ◽  
J Laskey ◽  
M Crump ◽  
F Khamsi ◽  
F A Shepherd
Keyword(s):  
Diabetes Mellitus ◽  
Median Duration ◽  
Cushing’S Syndrome ◽  
Metabolic Alkalosis ◽  
Cushing's Syndrome ◽  
Hormonal Control ◽  
Survival Duration ◽  
Malignant Neoplasms ◽  
Hormonal Response ◽  
Free Cortisol

PURPOSE To evaluate the safety and efficacy of ketoconazole treatment in the management of patients with paraneoplastic Cushing's syndrome (CS) secondary to ectopic adrenocorticotropin (ACTH) production by malignant neoplasms (ECS). PATIENTS AND METHODS A retrospective chart review was undertaken for 15 consecutive patients with ECS treated with ketoconazole. Strict criteria were defined for diagnosis of ECS and for clinical, biochemical, and hormonal responses. RESULTS There were four women and 11 men with a median age of 59 years (range, 44 to 84). Eleven patients had primary lung cancer (nine small-cell [SCLC], one mixed SCLC/non-SCLC, and one non-SCLC); two had carcinoid tumors (one bronchial, one pancreatic); one had hepatocellular carcinoma; and one had medullary carcinoma of the thyroid. Eight patients had ECS diagnosed at tumor presentation. Clinical findings included proximal muscle weakness (n = 10), peripheral edema (n = 8), and hypertension (n = 8). Biochemical abnormalities included hypokalemia (n = 14), metabolic alkalosis (n = 13), and new or worsened diabetes mellitus (n = 10). Patients received ketoconazole in dosages of 400 to 1,200 mg/d titrated by changes in urinary free-cortisol (UFC) levels for a median duration of 26 days (range, 3 to 1,059), and nine also received chemotherapy with ketoconazole. Hypokalemia, metabolic alkalosis, diabetes mellitus, and hypertension improved in the majority of patients. Ten patients had a hormonal response, with seven complete responses (median duration, 25 days; range, 6 to 989). The occurrence of symptomatic hypoadrenalism was definite in three patients and probable in one. Most patients died of progressive malignant disease accompanied by escape from hormonal control by ketoconazole. The median survival duration of the group was 19 weeks (range, 1 to 154). CONCLUSION Ketoconazole results in biochemical and hormonal improvement for most patients with ECS. It has few adverse effects, but may impair the cortisol response to stress. For that reason, replacement corticosteroids should be considered for patients with hormonal response, and moderate- to high-dose corticosteroids should be given for any potential stress situations. The ultimate control of the syndrome is dependent on successful treatment of the underlying tumor.

scholarly journals Fluconazole and acetazolamide in the treatment of ectopic Cushing's syndrome with severe metabolic alkalosis

2015 ◽  
Vol 2015 ◽  
Author(s):  
Verena Schwetz ◽  
Felix Aberer ◽  
Claudia Stiegler ◽  
Thomas R Pieber ◽  
Barbara Obermayer-Pietsch ◽  
...  
Keyword(s):  
Case Report ◽  
Surgical Resection ◽  
Cushing’S Syndrome ◽  
Metabolic Alkalosis ◽  
Cushing's Syndrome ◽  
List Type ◽  
Ectopic Acth ◽  
Ectopic Cushing’S Syndrome ◽  
Ectopic Acth Production ◽  
Learning Points

Summary Cushing's syndrome (CS) due to ectopic ACTH production accounts for about 10% of all types of CS and is frequently associated with metabolic alkalosis. Treatment of CS involves surgical resection and/or medical therapy to control hypercortisolism. We present the case of an 80-year-old woman affected by CS due to an unknown cause. The patient had severe metabolic alkalosis with refractory hypokalemia. To treat the underlying CS, fluconazole was initiated due to unavailability of ketoconazole. In spite of markedly decreasing cortisol levels, metabolic alkalosis persisted. Treatment of metabolic alkalosis with acetazolamide was thus initiated and pH levels successfully lowered. This case report shows that hypercortisolism can be effectively treated with fluconazole in cases where ketoconazole is unavailable or not tolerated and that persistent severe metabolic alkalosis caused by glucocorticoid excess can be safely and successfully treated with acetazolamide. Learning points Hypercortisolism can be effectively treated with fluconazole where ketoconazole is unavailable or not tolerated. Glucocorticoid excess can cause severe metabolic alkalosis. Persistent severe metabolic alkalosis can be safely and successfully treated with acetazolamide.

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.

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