Severe adrenal hemorrhage in a hypertensive patient, with high plasma aldosterone and metanephrines

2021 ◽  
Author(s):  
Maria Fernandez-Trujillo-Comenge Paula ◽  
Hernández Lázaro Alba ◽  
de León Durango Ricardo ◽  
Agnieszka Kuzior ◽  
Esteban Nivelo-Rivadeneira Manuel ◽  
...  
Author(s):  
Shuhei Baba ◽  
Arina Miyoshi ◽  
Shinji Obara ◽  
Hiroaki Usubuchi ◽  
Satoshi Terae ◽  
...  

Summary A 31-year-old man with Williams syndrome (WS) was referred to our hospital because of a 9-year history of hypertension, hypokalemia, and high plasma aldosterone concentration to renin activity ratio. A diagnosis of primary aldosteronism (PA) was clinically confirmed but an abdominal CT scan showed no abnormal findings in his adrenal glands. However, a 13-mm hypervascular tumor in the posterosuperior segment of the right hepatic lobe was detected. Adrenal venous sampling (AVS) subsequently revealed the presence of an extended tributary of the right adrenal vein to the liver surrounding the tumor. Segmental AVS further demonstrated a high plasma aldosterone concentration (PAC) in the right superior tributary vein draining the tumor. Laparoscopic partial hepatectomy was performed. The resected tumor histologically separated from the liver was composed of clear cells, immunohistochemically positive for aldesterone synthase (CYP11B2), and subsequently diagnosed as aldosterone-producing adrenal adenoma. After surgery, his blood pressure, serum potassium level, plasma renin activity and PAC were normalized. To the best of our knowledge, this is the first report of WS associated with PA. WS harbors a high prevalence of hypertension and therefore PA should be considered when managing the patients with WS and hypertension. In this case, the CT findings alone could not differentiate the adrenal rest tumor. Our case, therefore, highlights the usefulness of segmental AVS to distinguish adrenal tumors from hepatic adrenal rest tumors. Learning points: Williams syndrome (WS) is a rare genetic disorder, characterized by a constellation of medical and cognitive findings, with a hallmark feature of generalized arteriopathy presenting as stenoses of elastic arteries and hypertension. WS is a disease with a high frequency of hypertension but the renin-aldosterone system in WS cases has not been studied at all. If a patient with WS had hypertension and severe hypokalemia, low PRA and high ARR, the coexistence of primary aldosteronism (PA) should be considered. Adrenal rest tumors are thought to arise from aberrant adrenal tissues and are a rare cause of PA. Hepatic adrenal rest tumor (HART) should be considered in the differential diagnosis when detecting a mass in the right hepatic lobe. Segmental adrenal venous sampling could contribute to distinguish adrenal tumors from HART.


1978 ◽  
Vol 55 (s4) ◽  
pp. 213s-215s ◽  
Author(s):  
A. Pagnan ◽  
A. C. Pessina ◽  
G. Thiene ◽  
C. Dal Palù

1. Variables involved in the genesis of hypertension in male broad-breasted white turkeys include social environment, obesity and high salt intake. 2. The hypertension is characterized by low plasma renin activity and, with increasing age, normal to high plasma aldosterone. 3. Medionecrosis of the abdominal aorta is a common pathological finding. 4. The absence of atherosclerotic plaques is probably related to the high concentrations of alpha-lipoproteins.


The Lancet ◽  
1973 ◽  
Vol 301 (7810) ◽  
pp. 959-962 ◽  
Author(s):  
A. Rösler ◽  
E. Gazit ◽  
R. Theodor ◽  
H. Biochis ◽  
D. Rabinowitz

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Eric Van Belle ◽  
Sophie Susen ◽  
Frederic Mouquet ◽  
Jean-Philippe Collet ◽  
La Pitié Salpétrière ◽  
...  

Background: Aldosterone, the final mediator of the renin-angiotensin-aldosterone pathway, exerts toxic effects on the vessel wall and the myocardium. We recently demonstrated that aldosterone levels predict long term mortality in patients treated by primary PCI for an acute myocardial infarction. Whether aldosterone levels predict long term clinical outcome in elective PCI remains currently unknown. Methods and Results: In 807 consecutive patients undergoing elective PCI, plasma aldosterone, hsCRP and troponin levels were measured before the procedure. During follow-up (median = 14.9 months), 52 patients died including 40 from a cardiovascular cause. Plasma aldosterone levels (median = 25 pg/mL, interquartile range = 13– 45 pg/mL) were higher in diabetics (p=0.01) or in patients with a higher NYHA class (p=0.0002), and were inversely related to age (p=0.0001). In univariate analysis, a plasma aldosterone > 25 pg/mL was associated with a higher mortality (9.0% vs 3.7%, p=0.01) and a higher cardiovascular mortality rate (8.2% vs 2.5%, p=0.0005, Figure ). Using a multivariable Cox model analysis, 6 variables were associated with cardiovascular mortality: plasma aldosterone [HR (log) = 3.48; p=0.004], hsCRP [HR (log) = 2.59; p=0.002], recent acute coronary syndrome [HR = 3.23, p=0.02], age [HR for a 10 year-increase = 1.42; p=0.04], diabetes [HR = 2.20; p=0.04] and LVEF [HR for a 10%-decrease = 1.58; p=0.001]. Conclusion: A high plasma level of aldosterone in patients referred for an elective PCI is an independent predictor of cardiovascular mortality during follow-up. Whether such patients could benefit from aldosterone antagonist remains to be tested.


2010 ◽  
Vol 34 (1) ◽  
pp. 74-78 ◽  
Author(s):  
Shusuke Yagi ◽  
Masashi Akaike ◽  
Ken-ichi Aihara ◽  
Takashi Iwase ◽  
Sumiko Yoshida ◽  
...  

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