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11-Deoxycorticosterone Producing Adrenal Hyperplasia as a Very Unusual Cause of Endocrine Hypertension: Case Report and Systematic Review of the Literature

BACKGROUND AND OBJECTIVES: 11-deoxycorticosterone overproduction due to an adrenal tumor or hyperplasia is a very rare cause of mineralocorticoid-induced hypertension. The objective is to provide the most relevant clinical features that clinicians dealing with patients presenting with the hallmarks...

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Autores principales: Asla, Queralt, Sardà, Helena, Lerma, Enrique, Hanzu, Felicia A., Rodrigo, María Teresa, Urgell, Eulàlia, Pérez, José Ignacio, Webb, Susan M., Aulinas, Anna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9008131/
https://www.ncbi.nlm.nih.gov/pubmed/35432204
http://dx.doi.org/10.3389/fendo.2022.846865
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author Asla, Queralt
Sardà, Helena
Lerma, Enrique
Hanzu, Felicia A.
Rodrigo, María Teresa
Urgell, Eulàlia
Pérez, José Ignacio
Webb, Susan M.
Aulinas, Anna
author_facet Asla, Queralt
Sardà, Helena
Lerma, Enrique
Hanzu, Felicia A.
Rodrigo, María Teresa
Urgell, Eulàlia
Pérez, José Ignacio
Webb, Susan M.
Aulinas, Anna
author_sort Asla, Queralt
collection PubMed
description BACKGROUND AND OBJECTIVES: 11-deoxycorticosterone overproduction due to an adrenal tumor or hyperplasia is a very rare cause of mineralocorticoid-induced hypertension. The objective is to provide the most relevant clinical features that clinicians dealing with patients presenting with the hallmarks of hypertension due to 11-deoxycorticosterone-producing adrenal lesions should be aware of. DESIGN AND METHODS: We report the case of a patient with an 11-deoxycorticosterone-producing adrenal lesion and provide a systematic review of all published cases (PubMed, Web of Science and EMBASE) between 1965 and 2021. RESULTS: We identified 46 cases (including ours). Most cases (31, 67%) affected women with a mean age of 42.9 ± 15.2 years and presented with high blood pressure and hypokalemia (average of 2.68 ± 0.62 mmol/L). Median (interquartile range) time from onset of first suggestive symptoms to diagnosis was 24 (55) months. Aldosterone levels were low or in the reference range in 98% of the cases when available. 11-deoxycorticosterone levels were a median of 12.5 (18.9) times above the upper limit of the normal reference range reported in each article and overproduction of more than one hormone was seen in 31 (67%). Carcinoma was the most common histological type (21, 45.7%). Median tumor size was 61.5 (60) mm. Malignant lesions were larger, had higher 11-deoxycorticosterone levels and shorter time of evolution at diagnosis compared to benign lesions. CONCLUSIONS: 11-deoxycorticosterone-producing adrenal lesions are very rare, affecting mostly middle-aged women with a primary aldosteronism-like clinical presentation and carcinoma is the most frequent histological diagnosis. Measuring 11-deoxycorticosterone levels, when low aldosterone levels or in the lower limit of the reference range are present in hypertensive patients, is advisable. SYSTEMATIC REVIEW REGISTRATION: Open Science Framework, 10.17605/OSF.IO/NR7UV.
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spelling pubmed-90081312022-04-15 11-Deoxycorticosterone Producing Adrenal Hyperplasia as a Very Unusual Cause of Endocrine Hypertension: Case Report and Systematic Review of the Literature Asla, Queralt Sardà, Helena Lerma, Enrique Hanzu, Felicia A. Rodrigo, María Teresa Urgell, Eulàlia Pérez, José Ignacio Webb, Susan M. Aulinas, Anna Front Endocrinol (Lausanne) Endocrinology BACKGROUND AND OBJECTIVES: 11-deoxycorticosterone overproduction due to an adrenal tumor or hyperplasia is a very rare cause of mineralocorticoid-induced hypertension. The objective is to provide the most relevant clinical features that clinicians dealing with patients presenting with the hallmarks of hypertension due to 11-deoxycorticosterone-producing adrenal lesions should be aware of. DESIGN AND METHODS: We report the case of a patient with an 11-deoxycorticosterone-producing adrenal lesion and provide a systematic review of all published cases (PubMed, Web of Science and EMBASE) between 1965 and 2021. RESULTS: We identified 46 cases (including ours). Most cases (31, 67%) affected women with a mean age of 42.9 ± 15.2 years and presented with high blood pressure and hypokalemia (average of 2.68 ± 0.62 mmol/L). Median (interquartile range) time from onset of first suggestive symptoms to diagnosis was 24 (55) months. Aldosterone levels were low or in the reference range in 98% of the cases when available. 11-deoxycorticosterone levels were a median of 12.5 (18.9) times above the upper limit of the normal reference range reported in each article and overproduction of more than one hormone was seen in 31 (67%). Carcinoma was the most common histological type (21, 45.7%). Median tumor size was 61.5 (60) mm. Malignant lesions were larger, had higher 11-deoxycorticosterone levels and shorter time of evolution at diagnosis compared to benign lesions. CONCLUSIONS: 11-deoxycorticosterone-producing adrenal lesions are very rare, affecting mostly middle-aged women with a primary aldosteronism-like clinical presentation and carcinoma is the most frequent histological diagnosis. Measuring 11-deoxycorticosterone levels, when low aldosterone levels or in the lower limit of the reference range are present in hypertensive patients, is advisable. SYSTEMATIC REVIEW REGISTRATION: Open Science Framework, 10.17605/OSF.IO/NR7UV. Frontiers Media S.A. 2022-03-31 /pmc/articles/PMC9008131/ /pubmed/35432204 http://dx.doi.org/10.3389/fendo.2022.846865 Text en Copyright © 2022 Asla, Sardà, Lerma, Hanzu, Rodrigo, Urgell, Pérez, Webb and Aulinas https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Endocrinology
Asla, Queralt
Sardà, Helena
Lerma, Enrique
Hanzu, Felicia A.
Rodrigo, María Teresa
Urgell, Eulàlia
Pérez, José Ignacio
Webb, Susan M.
Aulinas, Anna
11-Deoxycorticosterone Producing Adrenal Hyperplasia as a Very Unusual Cause of Endocrine Hypertension: Case Report and Systematic Review of the Literature
title 11-Deoxycorticosterone Producing Adrenal Hyperplasia as a Very Unusual Cause of Endocrine Hypertension: Case Report and Systematic Review of the Literature
title_full 11-Deoxycorticosterone Producing Adrenal Hyperplasia as a Very Unusual Cause of Endocrine Hypertension: Case Report and Systematic Review of the Literature
title_fullStr 11-Deoxycorticosterone Producing Adrenal Hyperplasia as a Very Unusual Cause of Endocrine Hypertension: Case Report and Systematic Review of the Literature
title_full_unstemmed 11-Deoxycorticosterone Producing Adrenal Hyperplasia as a Very Unusual Cause of Endocrine Hypertension: Case Report and Systematic Review of the Literature
title_short 11-Deoxycorticosterone Producing Adrenal Hyperplasia as a Very Unusual Cause of Endocrine Hypertension: Case Report and Systematic Review of the Literature
title_sort 11-deoxycorticosterone producing adrenal hyperplasia as a very unusual cause of endocrine hypertension: case report and systematic review of the literature
topic Endocrinology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9008131/
https://www.ncbi.nlm.nih.gov/pubmed/35432204
http://dx.doi.org/10.3389/fendo.2022.846865
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