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ODP144 Cushing Syndrome and Primary Hyperaldosteronism due to Synchronic Bilateral Adrenal Adenomas with Exclusive Production of Cortisol on the Right and Aldosterone on the Left

Female patient, 37 years old, coming from China, with a history of resistant arterial hypertension since the age of 25 years old associated with spontaneous hypokalemia and classic ACTH-independent Cushing's syndrome. Radiological imaging exams showed bilateral adrenal nodules. Diagnosed primar...

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Autores principales: Rolim, Marina Rocha, Mendonça, Berenice Bilharinho, Ledesma, Felipe, Charchar, Helaine, Srougi, Victor, Tanno, Fabio, Chambo, José Luis, Almeida, Madson Queiroz, Latronico, Ana Claudia, Fragoso, Maria Candida Villares
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2022
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9700280/
http://dx.doi.org/10.1210/jendso/bvac150.147
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author Rolim, Marina Rocha
Mendonça, Berenice Bilharinho
Ledesma, Felipe
Charchar, Helaine
Srougi, Victor
Tanno, Fabio
Chambo, José Luis
Almeida, Madson Queiroz
Latronico, Ana Claudia
Fragoso, Maria Candida Villares
author_facet Rolim, Marina Rocha
Mendonça, Berenice Bilharinho
Ledesma, Felipe
Charchar, Helaine
Srougi, Victor
Tanno, Fabio
Chambo, José Luis
Almeida, Madson Queiroz
Latronico, Ana Claudia
Fragoso, Maria Candida Villares
author_sort Rolim, Marina Rocha
collection PubMed
description Female patient, 37 years old, coming from China, with a history of resistant arterial hypertension since the age of 25 years old associated with spontaneous hypokalemia and classic ACTH-independent Cushing's syndrome. Radiological imaging exams showed bilateral adrenal nodules. Diagnosed primary hyperaldosteronism (Baseline aldosterone 103ng/dL (2.5-39.2), Renin 2.7uUI/mL (4.4 - 46.1), Aldo/renin ratio 38.1 and K 3.3) and confirmed laboratory hypercortisolism (Cortisol after 1mg of dexamethasone overnight 17.9mcg/Dl (< 1.8), salivary cortisol at midnight 0.466mcg/dL (<0.274), ACTH <2pg/mL, basal serum cortisol 19.4 (3.7–19.4). Abdominal CT identified bilateral adrenal nodules, one on the right and two on the left, with <10 HU, suggestive of adenomas. In the pandemic, we proposed the same surgical treatment for primary adrenocortical macronodular hyperplasia (total adrenalectomy of the largest gland and with greater uptake in PET-FDG and subtotal of the smallest and with less uptake). The patient was therefore submitted to total videolaparoscopic adrenalectomy on the left and subtotal on the right. The anatomopathological evaluation showed bilateral adenomas (Weiss 1) being the immunohistochemistry of the right adrenal nodule diffusely positive for CYP11B1 and negative for CYP11B2, inferring only cortisol production. In contrast, left adrenal nodules were positive only for CYP11B2, inferring exclusive aldosterone production. However, we also found bilateral subcapsular cell clusters with positivity for CYP11B2. This may infer a possibility of hyperaldosteronism recurrence. The patient developed secondary adrenal insufficiency postoperatively, requiring replacement with hydrocortisone, and cure of hyperaldosteronism with normalization of potassium and release of renin. Six months after the operation, there was no need for antihypertensive drugs. Currently, it is using only one class of antihypertensive, amlodipine 5mg a day, and it still needs to be replaced with hydrocortisone 20mg on waking up and 10mg at 2 pm with still undetectable serum cortisol concentrations, despite the ACTH being unblocked (17.3 pg /ml). Now we are working on genetic background of this case report. Presentation: No date and time listed
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spelling pubmed-97002802022-11-29 ODP144 Cushing Syndrome and Primary Hyperaldosteronism due to Synchronic Bilateral Adrenal Adenomas with Exclusive Production of Cortisol on the Right and Aldosterone on the Left Rolim, Marina Rocha Mendonça, Berenice Bilharinho Ledesma, Felipe Charchar, Helaine Srougi, Victor Tanno, Fabio Chambo, José Luis Almeida, Madson Queiroz Latronico, Ana Claudia Fragoso, Maria Candida Villares J Endocr Soc Adrenal Female patient, 37 years old, coming from China, with a history of resistant arterial hypertension since the age of 25 years old associated with spontaneous hypokalemia and classic ACTH-independent Cushing's syndrome. Radiological imaging exams showed bilateral adrenal nodules. Diagnosed primary hyperaldosteronism (Baseline aldosterone 103ng/dL (2.5-39.2), Renin 2.7uUI/mL (4.4 - 46.1), Aldo/renin ratio 38.1 and K 3.3) and confirmed laboratory hypercortisolism (Cortisol after 1mg of dexamethasone overnight 17.9mcg/Dl (< 1.8), salivary cortisol at midnight 0.466mcg/dL (<0.274), ACTH <2pg/mL, basal serum cortisol 19.4 (3.7–19.4). Abdominal CT identified bilateral adrenal nodules, one on the right and two on the left, with <10 HU, suggestive of adenomas. In the pandemic, we proposed the same surgical treatment for primary adrenocortical macronodular hyperplasia (total adrenalectomy of the largest gland and with greater uptake in PET-FDG and subtotal of the smallest and with less uptake). The patient was therefore submitted to total videolaparoscopic adrenalectomy on the left and subtotal on the right. The anatomopathological evaluation showed bilateral adenomas (Weiss 1) being the immunohistochemistry of the right adrenal nodule diffusely positive for CYP11B1 and negative for CYP11B2, inferring only cortisol production. In contrast, left adrenal nodules were positive only for CYP11B2, inferring exclusive aldosterone production. However, we also found bilateral subcapsular cell clusters with positivity for CYP11B2. This may infer a possibility of hyperaldosteronism recurrence. The patient developed secondary adrenal insufficiency postoperatively, requiring replacement with hydrocortisone, and cure of hyperaldosteronism with normalization of potassium and release of renin. Six months after the operation, there was no need for antihypertensive drugs. Currently, it is using only one class of antihypertensive, amlodipine 5mg a day, and it still needs to be replaced with hydrocortisone 20mg on waking up and 10mg at 2 pm with still undetectable serum cortisol concentrations, despite the ACTH being unblocked (17.3 pg /ml). Now we are working on genetic background of this case report. Presentation: No date and time listed Oxford University Press 2022-11-01 /pmc/articles/PMC9700280/ http://dx.doi.org/10.1210/jendso/bvac150.147 Text en © The Author(s) 2022. Published by Oxford University Press on behalf of the Endocrine Society. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (https://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Adrenal
Rolim, Marina Rocha
Mendonça, Berenice Bilharinho
Ledesma, Felipe
Charchar, Helaine
Srougi, Victor
Tanno, Fabio
Chambo, José Luis
Almeida, Madson Queiroz
Latronico, Ana Claudia
Fragoso, Maria Candida Villares
ODP144 Cushing Syndrome and Primary Hyperaldosteronism due to Synchronic Bilateral Adrenal Adenomas with Exclusive Production of Cortisol on the Right and Aldosterone on the Left
title ODP144 Cushing Syndrome and Primary Hyperaldosteronism due to Synchronic Bilateral Adrenal Adenomas with Exclusive Production of Cortisol on the Right and Aldosterone on the Left
title_full ODP144 Cushing Syndrome and Primary Hyperaldosteronism due to Synchronic Bilateral Adrenal Adenomas with Exclusive Production of Cortisol on the Right and Aldosterone on the Left
title_fullStr ODP144 Cushing Syndrome and Primary Hyperaldosteronism due to Synchronic Bilateral Adrenal Adenomas with Exclusive Production of Cortisol on the Right and Aldosterone on the Left
title_full_unstemmed ODP144 Cushing Syndrome and Primary Hyperaldosteronism due to Synchronic Bilateral Adrenal Adenomas with Exclusive Production of Cortisol on the Right and Aldosterone on the Left
title_short ODP144 Cushing Syndrome and Primary Hyperaldosteronism due to Synchronic Bilateral Adrenal Adenomas with Exclusive Production of Cortisol on the Right and Aldosterone on the Left
title_sort odp144 cushing syndrome and primary hyperaldosteronism due to synchronic bilateral adrenal adenomas with exclusive production of cortisol on the right and aldosterone on the left
topic Adrenal
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9700280/
http://dx.doi.org/10.1210/jendso/bvac150.147
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