Severe Hypokalaemia, Hypertension, and Intestinal Perforation in Ectopic Adrenocorticotropic Hormone Syndrome

Kaya, Tezcan; Karacaer, Cengiz; Açıkgöz, Seyyid Bilal; Aydemir, Yusuf; Tamer, Ali

doi:10.7860/jcdr/2016/17198.7127

Cited by 8 publications

(15 citation statements)

References 14 publications

(21 reference statements)

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“…Hypokalemia occurred if there was severe hypercortisolism in both CD and ectopic ACTH patients. 4,9,18,[25][26][27][28] Moreover, the UFF/UFE ratio in patients with hypokalemia was significantly higher than that in adrenal CS patients with normokalemia (P < 0.001), suggesting that 11β-HSD2 deficiency was ACTH independent. 29 Otherwise, we analyzed the incidence of hypokalemia in patients hospitalized in our department with ACTH-independent cortisol-secreting adrenal adenoma and carcinoma.…”

Section: Discussionmentioning

confidence: 93%

“…The results above indicated that ACTH was not as relevant as cortisol in contributing to hypokalemia. Hypokalemia occurred if there was severe hypercortisolism in both CD and ectopic ACTH patients . Moreover, the UFF/UFE ratio in patients with hypokalemia was significantly higher than that in adrenal CS patients with normokalemia ( P < 0.001), suggesting that 11β‐HSD2 deficiency was ACTH independent…”

Section: Discussionmentioning

confidence: 96%

“…reported a negative correlation between plasma potassium and cortisol levels when 64 pituitary (48/64) and ectopic ACTH‐dependent (16/64) cases were analyzed together ( P < 0.001) and when the pituitary cases were considered alone ( P < 0.05), but not when the ectopic ACTH‐dependent cases were considered alone. Torpy et al . reported a significant correlation of urine cortisol and plasma potassium levels in ectopic ACTH‐dependent cases.…”

Section: Introductionmentioning

confidence: 98%

“…It occurred more frequently in cases with ectopic ACTH syndrome than those with CD. 4 The incidence of hypokalemia in patients with ectopic ACTH syndrome was 57-100% (57% (33/58), 5 64.0% (16/25), 6 71.3% (79/110), 7 85.7% (18/21), 8 95% (22/23), 9 and 100% doi: 10.1111/nyas.14205 (16/16) 10 ) according to the previous studies, while the incidence of hypokalemia was rarely reported in patients with CD as 10% (5/48), 10 9%(1/11), 11 and 50.57% (44/87), 12 respectively, which needs to be explored further. 3 Patients with ectopic ACTH syndrome, in whom hypokalemia occurs more frequently, usually have higher cortisol and ACTH levels compared with those with CD.…”

Section: Introductionmentioning

confidence: 99%

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Association of hypokalemia with cortisol and ACTH levels in Cushing's disease

Fan

Zhuang

Wang

et al. 2019

Annals of the New York Academy of Sciences

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Hypokalemia is a common feature in patients with Cushing's syndrome (CS). Whether the occurrence of hypokalemia is associated with cortisol and adrenocorticotropic hormone (ACTH) levels is still unclear. Approximately 80% of cases of endogenous CS are due to Cushing's disease (CD). The purpose of this study was to determine the association of hypokalemia with cortisol and ACTH levels in patients with CD. The retrospective study included 195 patients with CD referred to our medical center from January 2011 to December 2017. The results show that 25.64% (50/195) of the patients had hypokalemia. The 24‐h urinary free cortisol (UFC) and plasma cortisol levels were significantly higher in patients with hypokalemia than those with normokalemia (P < 0.05). Plasma ACTH levels were similar between the patients with hypokalemia and normokalemia (P > 0.05). Cortisol levels were negatively correlated with plasma potassium levels (08:00: r = −0.344 (P < 0.01), 00:00: r = −0.435 (P < 0.01); 24‐h UFC: r = −0.281 (P < 0.05)). There was no significant correlation between the plasma ACTH and potassium (08:00: r = −0.093 (P > 0.05), 00:00: r = −0.184 (P > 0.050)). Our current data suggest that cortisol level, instead of ACTH level, is correlated with plasma potassium level. A high cortisol level may be the principal cause of hypokalemia.

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Section: Discussionmentioning

confidence: 93%

Section: Discussionmentioning

confidence: 96%

Section: Introductionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Association of hypokalemia with cortisol and ACTH levels in Cushing's disease

Fan

Zhuang

Wang

et al. 2019

Annals of the New York Academy of Sciences

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“…In the presence of an unexplained septic state, physicians should also be aware of cortisol-induced gut perforation responsible for peritonitis that can occur without 'contracture' and prescribe abdominal imaging (121,122,123).…”

Section: (E) Infectious Complicationsmentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

Young

Haissaguerre

Viera-Pinto

et al. 2020

European Journal of Endocrinology

149

186

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Ectopic ACTH syndrome (EAS) is rare but is frequently a severe condition because of the intensity of the hypercortisolism that may be dissociated from the tumoral condition. EAS should often be considered as an endocrine emergency requiring an emergency response both in terms of diagnostic procedures and therapeutic interventions. Patient management is complex and necessitates dual skills, in the diagnosis and treatment of CS and in the specific management of neuroendocrine tumors (NET). Therefore, initial management should be performed ideally by experienced endocrinology teams in collaboration with specialized hormonal laboratory, modern imaging platforms and intensive care units. Diagnostic procedures vary according to the endocrine and tumoral contexts but should be reduced to a minimum in intense hypercortisolism. Preventive and curative treatments of cortisol-induced comorbidities, non-specific management of hypercortisolism and etiological treatments should be considered simultaneously. Therapeutic strategies vary according to (1.) the intensity of hypercortisolism, the general condition of the patient and associated comorbidities and (2.) the tumoral status, ranging from resectable ACTH secreting tumors to non-resectable metastatic endocrine tumors or occult tumors. The ideal treatment is complete excision of the ACTH-secreting tumor that can be performed rapidly or after preoperative preparation using cortisol-lowering drugs. When this is not possible, the therapeutic strategy should be discussed by a multidisciplinary experienced team in a personalized perspective and include variable combinations of pharmacological agents, bilateral adrenalectomy and non-specific tumoral interventions. Here we discuss the diagnosis and therapeutic strategies including the modern, currently available tools and emphasize on the operational effectiveness of care.

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Intestinal Perforation in ACTH-Dependent Cushing’s Syndrome

Shahidi

Phillips

Chik

2019

BioMed Research International

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Previous studies have linked systemic glucocorticoid use with intestinal perforation. However, the association between intestinal perforation and endogenous hypercortisolism has not been well described, with only 14 previously published case reports. In this study, we investigated if intestinal perforation occurred more frequently in patients with ectopic ACTH syndrome and in those with a greater than 10-fold elevation of 24-hour urinary free cortisol level. Of 110 patients with ACTH-dependent Cushing’s syndrome followed in two clinics in Canada, six cases with intestinal perforation were identified over 15 years. Age of patients ranged from 52 to 72, five females and one male, four with Cushing’s disease and two with ectopic ACTH production, one from a pancreatic neuroendocrine tumor and one from medullary carcinoma of the thyroid. Five had diverticular perforation and one had intestinal perforation from a stercoral ulcer. All cases had their lower intestinal perforation when the cortisol production was high, and one patient had diverticular perforation 15 months prior to the diagnosis of Cushing’s disease. As in previously reported cases, most had hypokalemia and abdominal pain with minimal or no peritoneal symptoms and this occurred during the active phase of Cushing’s syndrome. Whereas all previously reported cases occurred in patients with 24-hour urinary free cortisol levels greater than 10-fold the upper limit of normal when measured and 11 of 14 patients had ectopic ACTH production, only one of our patients had this degree of hypercortisolism and four of our six patients had Cushing’s disease. Similar to exogenous steroid use, patients with endogenous hypercortisolism also have a higher risk of intestinal, in particular diverticular, perforation and should be monitored closely for its occurrence with a low threshold for investigation and surgical intervention. Elective colonoscopy probably should be deferred until Cushing’s syndrome is under control.

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Severe Hypokalaemia, Hypertension, and Intestinal Perforation in Ectopic Adrenocorticotropic Hormone Syndrome

Cited by 8 publications

References 14 publications

Association of hypokalemia with cortisol and ACTH levels in Cushing's disease

Association of hypokalemia with cortisol and ACTH levels in Cushing's disease

MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

Intestinal Perforation in ACTH-Dependent Cushing’s Syndrome

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