scholarly journals Treatment-resistant hypertension in a post-transplant patient with cystic fibrosis: a rare case of phaeochromocytoma

2021 ◽  
Vol 2021 ◽  
Author(s):  
David Joseph Tansey ◽  
Jim John Egan ◽  
Michelle Murray ◽  
Katie Padfield ◽  
John Conneely ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Cystic Fibrosis ◽  
Adrenal Gland ◽  
Lung Transplant ◽  
List Type ◽  
Urinary Catecholamines ◽  
Post Surgery ◽  
Rare Tumours ◽  
History Of ◽  
Background History

Summary Phaeochromocytoma is a rare catecholamine-producing tumour. We present the case of phaeochromocytoma in a young man with a background history of a double-lung transplant for cystic fibrosis (CF). Clinical case: A 25-year-old man, with a background history of CF, CF-related diabetes (CFRD) and a double-lung transplant in 2012 was presented to the emergency department with crampy abdominal pain, nausea and vomiting. He was diagnosed with distal intestinal obstructions syndrome (DIOS). Contrast-enhanced CT imaging of the abdomen and pelvis showed a 3.4 cm right adrenal lesion. This was confirmed by a subsequent MRI of adrenal glands that demonstrated moderate FDG uptake, suggestive of a diagnosis of phaeochromocytoma. The patient was noted to be hypertensive with a blood pressure averaging 170/90 mm/Hg despite treatment with three different anti-hypertensive medications – amlodipine, telmisartan and doxazosin. He had hypertension for the last 3 years and had noted increasingly frequent sweating episodes recently, without palpitations or headache. Laboratory analysis showed elevated plasma normetanephrines (NMN) of 3167 pmol/L (182–867) as well as elevated metanephrines (MN) of 793 pmol/L (61–377) and a high 3-MT of 257 pmol/L (<185). Once cathecholamine excess was identified biochemically, we proceeded to functional imaging to further investigate. MIBG scan showed a mild increase in the uptake of tracer to the right adrenal gland compared to the left. The case was discussed at a multidisciplinary (MDT) meeting at which the diagnosis of phaeochromocytoma was made. Following a challenging period of 4 weeks to control the patient’s blood pressure with an alpha-blocker and beta-blocker, the patient had an elective right adrenalectomy, with normalisation of his blood pressure post-surgery. The histopathology of the excised adrenal gland was consistent with a 3 cm phaeochromocytoma with no adverse features associated with malignant potential. Learning points Five to ten per cent of patients have a secondary cause for hypertension. Phaeochromocytomas are rare tumours, originating in chromaffin cells and they represent 0.1–1.0% of all secondary hypertension cases. Secondary causes should be investigated in cases where: Patient is presenting <20 years of age or >50 years of age, There is refractory hypertension, or There is serious end-organ damage present. Patients may present with the triad of headache, sweating and palpitations or more vague, non-specific symptoms. Patients with suspected phaeochromocytoma should have 24-h urinary catecholamines measured and if available, plasma metanephrines measured. Those with abnormal biochemical tests should be further investigated with imaging to locate the tumour. Medical treatment involves alpha- and beta-blockade for at least 2 to 3 weeks before surgery as well as rehydration. There is a possibility of relapse so high-risk patients require life-long follow-up.

scholarly journals SAT-209 Treatment-Resistant Hypertension in a Post-Transplant Patient with Cystic Fibrosis: A Rare Case of Pheochromocytoma

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
David Joseph Tansey ◽  
Mensud Hatunic ◽  
John Conneely ◽  
Michelle Murray
Keyword(s):  
Blood Pressure ◽  
Cystic Fibrosis ◽  
Adrenal Gland ◽  
Rare Case ◽  
Lung Transplant ◽  
Transplant Patient ◽  
Endocrine Regulation ◽  
Post Transplant ◽  
History Of ◽  
Background History

Abstract Background: Pheochromocytoma is a rare catecholamine-producing tumor with an estimated incidence of less than 0.1% in the global population. We present the case of a pheochromocytoma in a 25-year-old man with a background history of a double-lung transplant for Cystic Fibrosis, carried out 5 years earlier. Clinical Case: A 25 year old, with a background history of Cystic Fibrosis and a Double Lung transplant in 2012 presented to the emergency department with crampy abdominal pain, nausea and vomiting. He was diagnosed with Distal Intestinal Obstruction syndrome (DIOS) for which he was admitted for rehydration and laxatives. Contrast-enhanced computed tomography (CT) imaging of the abdomen and pelvis which showed a 3.4 cm right adrenal lesion, which was confirmed by a subsequent MRI Adrenals and an Endocrinology review was requested. On review, the patient was noted to be hypertensive with a blood pressure averaging 170/90 despite treatment with 3 different anti-hypertensive medications - namely amlodipine, telmisartan and doxazosin. On review of his medical notes, it was clear that he had been persistently hypertensive over the last 3 years. On further questioning, he noted increasingly frequent sweating episodes over the last number of months but denied any palpitations, headache or back pain. Laboratory analysis showed an elevated plasma normetanephrines (NMN) of 3167 pmol/L (182-867) as well as elevated metanephrines (MN) of 793 pmol/L (61-377) and high 3-MT of 257 pmol/L (&lt;185). His MIBG scan showed only a mild increase in the uptake of tracer to the right adrenal gland compared to the left. The case was discussed at a multidisciplinary meeting and given the suggestive laboratory and radiologic findings, a presumptive diagnosis of pheochromocytoma was made. After controlling blood pressure with an alpha-blocker and beta-blocker for a week, the patient was hydrated and scheduled for an elective right adrenalectomy. The histopathology of the excised adrenal gland was consistent with a 3cm pheochromocytoma with none of the adverse features associated with malignant potential. The patient recovered well post-op, his blood pressure normalised and he was discharged home well for follow-up at the Endocrine and Transplant clinics. Conclusion: We describe a rare case of a right adrenal pheochromocytoma in a young man with multiple co-morbidities, who completely recovered after tumor resection. This case highlights the crucial importance of investigating secondary causes of hypertension, especially in younger patients. This is the first documented case in the literature of a case of pheochromocytoma in a post-transplant patient with Cystic Fibrosis. References: 1. Farrugia FA, Marikos G et al. Pheochromocytoma, diagnosis and treatment: Review of the literature. Endocrine Regulation, Volume 51, Issue 3, 30th August 2017.

scholarly journals “The Way to a Man’s Heart” - Adrenocortical Carcinoma With Vascular Invasion Into the Right Atrium and Ventricle

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A983-A984
Author(s):  
Hassaan B Aftab ◽  
Kaye-Anne L Newton ◽  
Vitaly Kantorovich
Keyword(s):  
Blood Pressure ◽  
Adrenal Gland ◽  
Inferior Vena Cava ◽  
Right Atrium ◽  
Adrenal Mass ◽  
Inferior Vena ◽  
Vena Cava ◽  
History Of ◽  
The Right ◽  
Right Hepatic Vein

Abstract Background: Adrenocortical carcinoma (ACC) is a rare aggressive malignant neoplasm which may present with intravascular extension into the inferior vena cava (IVC) and rarely into the right atrium (RA). Clinical Case: 62-year-old male with no prior known significant medical history presented to ED with 2-day history of mild hematuria with 3-week history of headache. Vital signs were normal other than blood pressure of 198/88 while physical exam was unremarkable. Headache subsided and blood pressure improved to 130/60 range after IV labetalol administration. CT abdomen and pelvis with contrast revealed a large right suprarenal mass extending into the right hepatic vein, IVC, and RA. The right adrenal gland was not visualized while the left adrenal gland and bilateral kidneys were normal. MRI chest, heart and abdomen with contrast showed heterogeneously enhancing lobulated right adrenal mass measuring 11.4 x 11 x 14 cm (AP, transverse, CC, respectively) with extensive tumor thrombus invading the right hepatic vein, IVC, RA and notably protruding into the right ventricle (RV) through the tricuspid valve during diastole. Technitium-99m MDP whole body scan did not show any uptake suspicious for metastases. Pre-op lab assessment showed mildly abnormal 1 mg dexamethasone suppression test but no evidence of ACTH suppression, elevated catecholamines or excess adrenal steroidogenesis. He underwent combined cardiothoracic and abdominal surgery on cardiopulmonary bypass with resection of adrenal mass, removal of thrombus from IVC, RA, RV and patch angioplasty of IVC with bovine pericardium. Pathology report was consistent with ACC (AJCC stage III). On 1 month postoperative follow-up, patient is clinically doing well with plans to start mitotane with addition of etoposide/doxorubicin/cisplatin (EDP) chemotherapy. Conclusion: ACC is a rare, highly aggressive malignancy which may produce extensive intravascular invasion. It may rarely extend to the RA and even rarer into the RV; with 42 and 1 reported cases, respectively. No study has conclusively found that vascular extension of ACC is a poor prognostic factor, hence surgical management is the primary strategy including cases with RA/RV involvement. There is lack of data and consensus regarding adjuvant or palliative medical therapy. However, in phase II trials combination of EDP chemotherapy and mitotane have shown response rates ranging from 11% to 54%. Reference: Alghulayqah, Abdulaziz, et al. “Long-term recurrence-free survival of adrenocortical cancer extending into the inferior vena cava and right atrium: Case report and literature review.” Medicine 96.18 (2017).

Abstract 2114: Antihypertensive Efficacy And Cerebral Hemodynamic Advantage Of Olmesartan Medoxomil Compared With Amlodipine In Post-stroke Patients

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Shuji Matsumoto ◽  
Megumi Shimodozono ◽  
Ryuji Miyata ◽  
Kazumi Kawahira
Keyword(s):  
Blood Pressure ◽  
Blood Pressure Monitoring ◽  
Antihypertensive Agents ◽  
Olmesartan Medoxomil ◽  
Antihypertensive Efficacy ◽  
Double Blind Study ◽  
List Type ◽  
Double Blind ◽  
Hypertensive Patients ◽  
History Of

In patients with severe hypertension, chronic heart failure or a history of stroke, the lower limit of autoregulation of cerebral blood flow (CBF) is shifted to higher levels of blood pressure (BP) than those observed in healthy subjects. The effects of anigiotensin II receptor blocker (ARB) on cerebral hemodynamics in humans have not been well elucidated. The antihypertensive efficacy of ARB olmesartan medoxomil (olmesartan) has been shown to compare favourably with that of other antihypertensive agents. We objected: To compare the effects of olmesartan and amlodipine on 24-h ambulatory blood pressure monitoring (ABPM); To compare the advantage of CBF of olmesartan and amlodipine on xenon-computed tomography (Xe-CT) after administration of either drug for 8 weeks. 24 hypertensive patients who had an episode of stroke more than 4 weeks previously were recruited for this randomized double-blind study. Olmesartan 10 –20mg (12 patients) or amlodipine 2.5–5mg (12 patients) was administered once daily for 8 weeks. During 24-h monitoring, both drugs caused a decrease in systolic BP of 14 mmHg, and caused a decrease in diastolic BP of 8 mmHg. The Xe-CT results indicated that the increase of CBF of olmesartan was 3.3 ± 1.1 ml/min/100g and that of amlodipine was 0.2 ± 1.3 ml/min/100g. Thus, despite the reduction in BP, CBF was significantly increased in olmesartan group compared with amlodipine group. We consider the use of olmesartan is advantageous for hypertensive patients with a history of stroke in whom autoregulation of CBF is potentially impaired.

scholarly journals SAT-196 The Use of 11C-metomidate PET-CT to Detect Unilateral Primary Hyperaldosteronism

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Sumitro Harjanto ◽  
Aye Chan Maung ◽  
Troy Puar ◽  
Daphne Gardner
Keyword(s):  
Blood Pressure ◽  
Adrenal Gland ◽  
Primary Hyperaldosteronism ◽  
Left Adrenal Gland ◽  
Active Renin ◽  
Potassium Supplementation ◽  
Serum Aldosterone ◽  
Pet Ct ◽  
History Of ◽  
The Right

Abstract Background Identifying causative adrenal lesions presents a significant diagnostic burden for physicians and radiologists. We describe the use of radiolabelled metomidate to lateralise primary hyperaldosteronism. Case presentation A 52-year old Chinese man with a 5-year history of hypertension was referred for hypokalemia [K 2.7 mmol/L (3.6 - 5.0)]. He had been on Telmisartan 80 mg and Amlodipine 10 mg daily and blood pressure at home ranged 110-120 / 70-80 mmHg. There was no history of poor oral intake, persistent diarrhea or vomiting, and he was not on any other prescription or alternative medications. There was no significant family history of hypertension or sudden cardiac death. Clinic blood pressure was 140/84 mmHg. There were no features suggestive of Cushing’s syndrome. Repeat biochemical tests confirmed hypokalemia (K 3.1 mmol/L), and associated raised bicarbonate 37.3 mmol/L [19 - 29]. Magnesium and creatinine were normal. Aldosterone-renin Ratio was elevated at 8.1 (serum Aldosterone 611 pmol/L [97.3 - 834.0], active renin 2.7 pg/ml [1.8 – 59.4]). Post-saline infusion, non-suppressible serum aldosterone levels of 1137 pmol/L was demonstrated, consistent with autonomous aldosterone production. A computed tomography of the adrenal revealed a 2.3 cm x 1.9 cm nodule on the left adrenal gland consistent with lipid rich adenoma. Adrenal vein sampling (AVS) under continuous synacthen infusion was performed. Adrenal to peripheral cortisol ratio was ≥10 for either adrenal veins, confirming cannulation of the adrenal veins. Aldosterone-cortisol ratios showed lateralization to the left adrenal gland (lateralization ratio of 10.35). There was contralateral suppression of the right adrenal gland with ratio of 0.41. 11C-Metomidate PET-CT scan demonstrated a maximum standardised uptake value (SUVmax) of 26.8 over the left adrenal nodule, while the SUVmax of the right adrenal gland was 16.2. Ratio of the left to right adrenal gland SUVmax was 1.65 (above the threshold of 1.25); and was concordant with AVS. This confirmed that the patient had a left functional adrenal adenoma responsible for hyperaldosteronism. Our patient underwent a left adrenalectomy, and histology was consistent with adrenal cortical adenoma. Prior to surgery he required 72 mmol/l of potassium supplementation daily to maintain K levels of 3.3 – 4.0 mmol/L. Two weeks post-operatively, he was normokalemic (K 4.9 mmol/L) without potassium supplementation. Serum aldosterone normalized to 159.3 pmol/L (active renin 9.3 pg/ml). Blood pressure is well controlled on amlodipine 5mg daily. Conclusion Targeted molecular imaging such as 11C-Metomidate PET-CT could aid localisation of functional adrenal disease to guide definitive surgical management. In the future, this could obviate the need for invasive and technically complex procedures like AVS.

scholarly journals SURGICAL APPROACH OF HYPERTENSION: PHEOCHROMOCYTOMA CASE REPORT

2015 ◽  
Vol 2 (1) ◽  
pp. 15-19
Author(s):  
Adriana Ion ◽  
I. Bostaca
Keyword(s):  
Blood Pressure ◽  
Adrenal Gland ◽  
Ventricular Arrhythmias ◽  
Low Salt ◽  
Rapid Pulse ◽  
Night Sweats ◽  
History Of ◽  
The Right ◽  
Solid Type

Pheochromocytoma is present in about 1% of hypertensive patients. It has a 90% cure potential, but a high mortality rate if left untreated. Complications include malignant hypertension, heart failure, myocardial infarction, ventricular arrhythmias, stroke or metastasis (malignant pheochromocytoma). We present the case of a 47-year-old hypertensive female patient who was admitted to hospital in 2014 with excessive fluctuations in blood pressure (300/140 mmHg to 90/50 mmHg), rapid pulse not responding to medication, profuse night sweats, palpitations, vertigo, nausea, and vomiting. The patient had a history of high arterial pressure dating back to 1996, following complex anti-hypertensive treatment, and frequent episodes of paroxysmal atrial fibrillation in the previous four years, reduced by external electric shock. Laboratory and imaging investigations revealed a solid-type formation with mixed structure in the right adrenal gland, indicating pheochromocytoma. Surgery with resection of the right adrenal gland was proposed. Preoperative preparation to combat the risks of large variations in blood pressure and hypovolemia started 1–3 weeks before surgery, consisting of an alpha-blocker to control blood pressure, a beta-blocker to prevent arrhythmic events, and hydration to prevent postoperative hypotension. A right adrenalectomy was performed. Histopathology of the resected piece confirmed the diagnosis of pheochromocytoma. After surgery, the large variations in blood pressure, arrythmic events and tumor symptoms were all reduced, and blood pressure returned to within normal range. Follow-up treatment consisted of Carvedilol 12.5 mg, 1 cp / day and a low-salt, hypolipidaemic diet was recommended. The case is notable for the late diagnosis made about 18 years after the onset of the hypertensive symptoms, treated with excessive medication.

Multiple Liver Lesions in a Patient With Cystic Fibrosis and History of Lung Transplant

2016 ◽  
Vol 16 (9) ◽  
pp. 2760-2762
Author(s):  
S. Abu‐Gazala ◽  
J. G. Quatromoni ◽  
T. Rashmi ◽  
P. L. Abt
Keyword(s):  
Cystic Fibrosis ◽  
Lung Transplant ◽  
Liver Lesions ◽  
History Of

History of vascular reactivity models and their involvement in hypertension pathogenesis

VASA ◽  
2017 ◽  
Vol 46 (6) ◽  
pp. 431-439 ◽  
Author(s):  
Ana Gabriela Conceição-Vertamatti ◽  
Filipy Borghi ◽  
Fernando Canova ◽  
Dora Maria Grassi-Kassisse
Keyword(s):  
Blood Pressure ◽  
Adrenergic Receptors ◽  
Vascular Reactivity ◽  
Multifactorial Disease ◽  
Pharmacological Treatments ◽  
Beta Adrenergic Receptors ◽  
Vascular Area ◽  
History Of ◽  
Record Blood Pressure ◽  
Advance Knowledge

Abstract. Hypertension is a silent and multifactorial disease. Over two centuries ago, the first device to record blood pressure was developed, making it possible to determine normotension and to establish criteria for hypertension. Since then, several studies have contributed to advance knowledge in this area, promoting significant advances in pharmacological treatments and, as a result, increasing survival of hypertensive people. The main models developed for the study of hypertension and the main findings in the vascular area are included in this review. We considered aspects related to vascular reactivity, changes in the population, and action of beta adrenergic receptors in the pathogenesis of hypertension.

Burnout of the Mind – Burnout of the Body?

2018 ◽  
Vol 32 (1) ◽  
pp. 30-42 ◽  
Author(s):  
Claudia Traunmüller ◽  
Kerstin Gaisbachgrabner ◽  
Helmut Karl Lackner ◽  
Andreas R. Schwerdtfeger
Keyword(s):  
Blood Pressure ◽  
Allostatic Load ◽  
Maslach Burnout Inventory ◽  
The Body ◽  
Lower Percentage ◽  
Control Group ◽  
Urinary Catecholamines ◽  
Morning Cortisol ◽  
The Mind ◽  
Cortisol Increase

Abstract. In the present paper we investigate whether patients with a clinical diagnosis of burnout show physiological signs of burden across multiple physiological systems referred to as allostatic load (AL). Measures of the sympathetic-adrenergic-medullary (SAM) axis and the hypothalamic-pituitary-adrenal (HPA) axis were assessed. We examined patients who had been diagnosed with burnout by their physicians (n = 32) and were also identified as burnout patients based on their score in the Maslach Burnout Inventory-General Survey (MBI-GS) and compared them with a nonclinical control group (n = 19) with regard to indicators of allostatic load (i.e., ambulatory ECG, nocturnal urinary catecholamines, salivary morning cortisol secretion, blood pressure, and waist-to-hip ratio [WHR]). Contrary to expectations, a higher AL index suggesting elevated load in several of the parameters of the HPA and SAM axes was found in the control group but not in the burnout group. The control group showed higher norepinephrine values, higher blood pressure, higher WHR, higher sympathovagal balance, and lower percentage of cortisol increase within the first hour after awakening as compared to the patient group. Burnout was not associated with AL. Results seem to indicate a discrepancy between self-reported burnout symptoms and psychobiological load.

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