Emergencia hipertensiva como debut de síndrome de Cushing paraneoplásico / Hypertensive Emergency as the Debut of Paraneoplastic Cushing Syndrome

Presentamos el caso de un paciente con antecedentes de hipertensión arterial vasculorrenal tratada un año antes, que acude a urgencias por emergencia hipertensiva (HTA) y disnea. Descartada primera sospecha de reestenosis de arteria renal con angiografía por tomografía computarizada (angioTC), se completa el estudio confirmándose diagnóstico de cáncer de pulmón mediante prueba de imagen y anatomía patológica. En el estudio de hipertensión se detecta elevación de hormona adrenocorticótropa (ACTH), hipercortisolismo y datos analíticos de hiperaldosteronismo. Con el diagnóstico final de síndrome de Cushing secundario a producción ectópica de ACTH se inicia tratamiento médico, sin llegar a recibir nada más por fallecimiento del paciente a los pocos días.(AU)

We present the case of a patient with a history of renal-vascular hypertension treated with stent one year previously, who attended the emergency room due to hypertensive emergency and dyspnea. Once the first suspicion of renal artery restenosis was ruled out with CT angiography, the study was completed, confirming the diagnosis of lung cancer through imaging and pathological anatomy. In the hormonal study, elevation of ACTH, hypercortisolism and analytical data of hyperaldosteronism were detected. With the final diagnosis of Cushing's syndrome secondary to ectopic production of ACTH, medical treatment was started, without being able to receive anything else due to the death of the patient after a few days.(AU)

[Hypertensive Emergency as the Debut of Paraneoplastic Cushing Syndrome]. / Emergencia hipertensiva como debut de síndrome de Cushing paraneoplásico.

We present the case of a patient with a history of renal-vascular hypertension treated with stent one year previously, who attended the emergency room due to hypertensive emergency and dyspnea. Once the first suspicion of renal artery restenosis was ruled out with CT angiography, the study was completed, confirming the diagnosis of lung cancer through imaging and pathological anatomy. In the hormonal study, elevation of ACTH, hypercortisolism and analytical data of hyperaldosteronism were detected. With the final diagnosis of Cushing's syndrome secondary to ectopic production of ACTH, medical treatment was started, without being able to receive anything else due to the death of the patient after a few days.

Indicación, realización y resultado del cateterismo de venas adrenales en pacientes con hiperaldosteronismo primario / Indication, performance and outcomes of adrenal vein sampling in patients with primary hyperaldosteronism

El hiperaldosteronismo primario (HAP) es una causa importante de hipertensión arterial (HTA) secundaria. El estudio del mismo precisa de una alta sospecha clínica, además de un estudio hormonal que confirme la hipersecreción hormonal. Es importante iniciar el tratamiento adecuado una vez se confirma el diagnóstico, y para ello es preciso demostrar si la hipersecreción hormonal es unilateral (pacientes que podrían ser tributarios a tratamiento quirúrgico) o bilateral (pacientes que son tributarios a tratamiento únicamente farmacológico). En el Hospital del Mar desde el año 2016 existe un equipo de trabajo multidisciplinar en el que participan nefrólogos, endocrinólogos, radiólogos y cirujanos para evaluar los casos con sospecha de hiperaldosteronismo y consensuar el mejor abordaje diagnóstico-terapéutico de estos pacientes, incluyendo la necesidad de cateterismo venoso adrenal, que es una técnica que en los últimos años se ha erigido como gold standard para el estudio del HAP. En el presente estudio recogemos la experiencia de nuestro centro en la realización de cateterismo venoso adrenal y en la utilidad de este para el manejo de tales pacientes (AU)

Primary hyperaldosteronism (PAH) is an important cause of secondary hypertension (HTN). The study of the same requires a high clinical suspicion in addition to a hormonal study that confirms hormonal hypersecretion. It is important to start the appropriate treatment once the diagnosis is confirmed, and for this is necessary to demonstrate whether the hormonal hypersecretion is unilateral (patients who could be candidates for surgical treatment) or bilateral (patients who are candidates for pharmacological treatment only). At the Hospital del Mar since 2016 there has been a multidisciplinary work team in which Nephrologists, Endocrinologists, Radiologists and Surgeons participate to evaluate cases with suspected hyperaldosteronism and agree on the best diagnostic-therapeutic approach for these patients, including the need for adrenal vein sampling, which is a technique that in recent years has become the gold standard for the study of PAH. In the present study we collect the experience of our center in performing adrenal vein catheterization and its usefulness for the management of these patients (AU)

Primary aldosteronism: Practical recommendations for treatment and follow-up / Hiperaldosteronismo primario: recomendaciones prácticas de tratamiento y seguimiento

Primary aldosteronism (PA) is the most common cause of secondary arterial hypertension. For unilateral cases, surgery offers the possibility of cure, with unilateral adrenalectomy being the treatment of choice, whereas bilateral forms of PA are treated mainly with mineralocorticoid receptor antagonists (MRA). The goals of treatment for PA due to either unilateral or bilateral adrenal disease include reversal of the adverse cardiovascular effects of hyperaldosteronism, normalization of serum potassium in patients with hypokalemia, and normalization of blood pressure. The Primary Aldosteronism Surgery Outcome group (PASO) published a study defining clinical and biochemical outcomes based on blood pressure and correction of hypokalemia and aldosterone to renin ratio (ARR) levels for patients undergoing total unilateral adrenalectomy for unilateral PA. In this review, we provide several practical recommendations for the medical and surgical management and follow-up of patients with PA. (AU)

El hiperaldosteronismo primario (HAP) es la causa más frecuente de hipertensión arterial secundaria. Para los casos unilaterales, la cirugía ofrece la posibilidad de curación, siendo la adrenalectomía unilateral el tratamiento de elección, mientras que las formas bilaterales de HAP se tratan principalmente con antagonistas del receptor de mineralocorticoides (ARM). Los objetivos del tratamiento del HAP debido a enfermedad suprarrenal unilateral o bilateral incluyen la reversión de los efectos cardiovasculares adversos del hiperaldosteronismo, la normalización del potasio sérico en pacientes con hipopotasemia y la normalización de la presión arterial. El grupo Primary Aldosteronism Surgery Outcome (PASO) publicó un estudio que define los resultados clínicos y bioquímicos en función de la presión arterial y la corrección de la hipopotasemia y los niveles del cociente aldosterona/renina (ARR) para pacientes sometidos a adrenalectomía unilateral total por HAP unilateral. En esta revisión ofrecemos varias recomendaciones prácticas para el manejo y el seguimiento médico-quirúrgico de los pacientes con HAP. (AU)

Manifestación inicial atípica de un hiperaldosteronismo primario / Atypical initial manifestation of primary hyperaldosteronism

Aproximadamente 10% de los casos de hipertensión arterial son debidos a una causa secundaria, encontrándose entre las más frecuentes el hiperaldosteronismo primario, caracterizado por hipertensión, alcalosis metabólica e hipopotasemia. No obstante, en raras ocasiones puede debutar de una forma atípica, en forma de debilidad muscular y mialgias secundarias a rabdomiólisis por hipopotasemia severa, como el caso que se describe. (AU)

Approximately 10% of cases of arterial hypertension are due to a secondary cause, being among the most frequent primary hyperaldosteronism, characterized by hypertension, metabolic alkalosis and hypokalemia. However, on rare occasions it can present in an atypical way, in the form of muscle weakness and myalgia secondary to rhabdomyolysis due to severe hypokalemia, as in the case described. (AU)

Primary aldosteronism: Practical recommendations for treatment and follow-up.

Primary aldosteronism (PA) is the most common cause of secondary arterial hypertension. For unilateral cases, surgery offers the possibility of cure, with unilateral adrenalectomy being the treatment of choice, whereas bilateral forms of PA are treated mainly with mineralocorticoid receptor antagonists (MRA). The goals of treatment for PA due to either unilateral or bilateral adrenal disease include reversal of the adverse cardiovascular effects of hyperaldosteronism, normalization of serum potassium in patients with hypokalemia, and normalization of blood pressure. The Primary Aldosteronism Surgery Outcome group (PASO) published a study defining clinical and biochemical outcomes based on blood pressure and correction of hypokalemia and aldosterone to renin ratio (ARR) levels for patients undergoing total unilateral adrenalectomy for unilateral PA. In this review, we provide several practical recommendations for the medical and surgical management and follow-up of patients with PA.

Evaluación del incidentaloma adrenal / Evaluation of the adrenal incidentaloma

An adrenal incidentaloma is a mass detected by chance on an imaging test. It is important that general practitioners know how to manage it, since it is an increasingly frequent finding. It is necessary to assess whether it presents malignant characteristics in imaging tests and the possibility of subclinical hormonal hyperfunction. The basic hormonal study evaluates the overproduction of catecholamines with a study of fractionated metanephrines in plasma or in 24-hour urine, hypercortisolism with a dexamethasone suppression test, and, in patients with hypertension or hypokalemia, the possibility of hyperaldosteronism with determination of the plasma aldosterone/renin ratio. Surgical treatment is indicated in hyperfunctioning nodules, those with suspicion of malignancy or in large ones. (AU)

El incidentaloma adrenal es una masa detectada de forma casual en una prueba de imagen. Es importante que los médicos generalistas conozcan su manejo, ya que es un hallazgo cada vez más frecuente. Hay que valorar si presenta características de malignidad en las pruebas de imagen y la posibilidad de hiperfuncionalidad hormonal subclínica.El estudio hormonal básico evalúa la sobreproducción de catecolaminas con un estudio de metanefrinas fraccionadas en plasma o en orina de 24 horas, el hipercortisolismo con una prueba de frenación con dexametasona y, en pacientes con hipertensión o hipopotasemia, la posibilidad de hiperaldosteronismo con la determinación del cociente aldosterona/renina plasmáticas. El tratamiento quirúrgico está indicado en nódulos hiperfuncionantes, con sospecha de malignidad o en aquellos de gran tamaño. (AU)

Hipertensión arterial secundaria: ¿Cuándo sospecharla y qué hacer? / Secondary arterial hypertension: When to suspect it and what to do?

La hipertensión arterial es un problema frecuente de salud pública, puede ser de etiología primaria o secundaria. La hipertensión arterial secundaria debida a una causa se puede tratar con intervenciones específicas. Con el propósito de identificar las causas y mecanismos más frecuentes, se realizó una búsqueda de artículos en Google Académico y Pubmed. Se encontró que podemos sospecharla en pacientes menores de 30 años debutando con hipertensión arterial sin factores de riesgo evidentes, datos clínicos, laboratoriales y estudios de imagen compatibles con causas renales o endocrinas de hipertensión secundaria, datos sugestivos de apnea del sueño. Debemos obtener una historia clínica y realizar un examen físico buscando pistas clínicas que nos orienten, para realizar estudios necesarios y llegar a un diagnóstico oportuno...(AU)

Indication, performance and outcomes of adrenal vein sampling in patients with primary hyperaldosteronism.

Primary hyperaldosteronism (PAH) is an important cause of secondary hypertension (HTN). The study of the same requires a high clinical suspicion in addition to a hormonal study that confirms hormonal hypersecretion. It is important to start the appropriate treatment once the diagnosis is confirmed, and for this is necessary to demonstrate whether the hormonal hypersecretion is unilateral (patients who could be candidates for surgical treatment) or bilateral (patients who are candidates for pharmacological treatment only). At the Hospital del Mar since 2016 there has been a multidisciplinary work team in which Nephrologists, Endocrinologists, Radiologists and Surgeons participate to evaluate cases with suspected hyperaldosteronism and agree on the best diagnostic-therapeutic approach for these patients, including the need for adrenal vein sampling, which is a technique that in recent years has become the gold standard for the study of PAH. In the present study we collect the experience of our centre in performing AVC and its usefulness for the management of these patients.

[Atypical initial manifestation of primary hyperaldosteronism]. / Manifestación inicial atípica de un hiperaldosteronismo primario.

Approximately 10% of cases of arterial hypertension are due to a secondary cause, being among the most frequent primary hyperaldosteronism, characterized by hypertension, metabolic alkalosis and hypokalemia. However, on rare occasions it can present in an atypical way, in the form of muscle weakness and myalgia secondary to rhabdomyolysis due to severe hypokalemia, as in the case described.

Küpers clinical prediction score in a case series of primary aldosteronism

Background: Primary aldosteronism is a disorder due to excessive aldosterone production in the presence of low renin levels. It is an underdiagnosed pathology despite its simple screening. Establishing the unilateral or bilateral location represents the greatest diagnostic challenge and is crucial to define the therapeutic approach. Adrenal venous catheterization (AVC) is the best test to establish the location, but it is invasive and expensive. New predictive markers of laterality are being developed. Case series presentation: We present a case series of 8 patients diagnosed with primary aldosteronism due to arterial hypertension with hypokalaemia, elevated aldosterone-renin ratio and compatible computed tomography. 4 patients underwent adrenal venous catheterization. Conclusion: In patients who underwent catheterization as well as in those who did not, the Küpers score adequately predicted lateralization in 75% of cases and it could be a useful tool to discriminate unilateral from bilateral aldosteronism.

Introducción: El hiperaldosteronismo primario es un desorden debido a una producción excesiva de aldosterona en presencia de niveles bajos de renina. Es una patología infradiagnosticada a pesar de su simple tamizaje. Definir la localización unilateral o bilateral representa el más importante desafío diagnóstico y es crucial para el abordaje terapéutico. El cateterismo venoso adrenal (CVA) es la mejor prueba para establecer la localización, pero es invasivo y costoso. Nuevos marcadores predictivos de unilateralidad se encuentran en desarrollo. Presentación de serie de casos: Presentamos una serie de casos de 8 pacientes diagnosticados con hiperaldosteronismo primario debido a hipertensión arterial con hipocalemia, radio aldosterona-renina elevado y tomografía compatible. 4 pacientes fueron sometidos a cateterismo venoso adrenal. Conclusión: Tanto en los pacientes que fueron sometidos a cateterismo venoso adrenal como en los que no, el score de Küpers predijo adecuadamente la lateralidad en 75% de los casos y puede ser una herramienta útil para diferenciar el hiperaldosteronismo unilateral del bilateral.

First survey on the diagnosis and treatment of primary aldosteronism by Spanish Endocrinology and Nutrition specialists.

OBJECTIVE: To evaluate the indication and the resources for the screening/diagnosis of primary aldosteronism (PA) in Endocrinology units in Spain. MATERIAL AND METHODS: An anonymous 2-phase (2020/2021) online survey was conducted by the AdrenoSEEN group among SEEN members with data about screening, confirmation tests, availability of catheterisation and the treatment of PA. RESULTS: Eighty-eight (88) specialists completed the survey. Plasma aldosterone concentration and plasma renin activity were available at all centres; urinary aldosterone was available in 55% of them. The most frequent indications for determining the aldosterone/renin ratio (ARR) were adrenal incidentaloma (82.6%), hypertension with hypokalaemia (82.6%), hypertension in patients <40 years (79.1%) and a family history of PA (77.9%). 61% and 18% of the respondents used an ARR cut-off value of PA of ≥30 and 20ng/dl per ng/mL/, respectively. The intravenous saline loading test was the most commonly used confirmatory test (66.3%), followed by the captopril challenge test (24.4%), with the 25mg dose used more than the 50mg dose (65% versus 35%). 67.4% of the participants confirmed the availability of adrenal vein catheterization (AVC). 41% of this subgroup perform it with a continuous infusion versus 30.5% with an ACTH (1-24) bolus, whereas 70.3% employ sequential adrenal vein catheterization. 48% of the participants reported an AVC success <50%. Total laparoscopic adrenalectomy was the treatment of choice (90.6%), performed by specialists in General and Digestive Surgery specialising in endocrinological pathology. CONCLUSION: PA screening and diagnostic tests are extensively available to Spanish endocrinologists. However, there is a major variability in their use and in the cut-off points of the diagnostic methods. The AVS procedure remains poorly standardised and is far from delivering optimal performance. Greater standardisation in the study and diagnosis of PA is called for.

Familial forms and molecular profile of primary hyperaldosteronism / Formas familiares y perfil molecular del hiperaldosteronismo primario

Primary hyperaldosteronism (PAH) is the most frequent cause of secondary arterial hypertension. Most PAHs occur sporadically, but 5% of cases have a hereditary origin (familial PAH). Four forms of familial PAH have been described. Type I familial PAH is produced by a fusion of the CYP11B2 and CYP11B1 genes, in this way the synthesis of aldosterone becomes to be regulated by ACTH instead of by angiotensin II. In type II, III and IV familial PAH there is an increase in the transcription and expression of CYP11B2 responsible for aldosterone synthesis due to a germinal mutation in CLCN2, KCNJ5 and CACNA1H, respectively. On the other hand, somatic mutations have been identified in 50% of sporadic PAHs, with gain-of-function mutations at the level of KCNJ5, ATP1A1, ATP2B3 and CACNA1D being the most common. This review provides a detailed description of the different forms of familial PAH and the molecular profile of patients with sporadic PAH. (AU)

El hiperaldosteronismo primario (HAP) es la causa más frecuente de hipertensión arterial secundaria. La mayor parte de los HAP ocurren de forma esporádica, pero un 5% de los casos tienen un origen hereditario (HAP familiar). Se conocen 4 formas de HAP familiares. El HAP familiar tipo I se produce por una fusión de los genes CYP11B2 y CYP11B1, de esta forma la síntesis de aldosterona pasa a estar regulada por la ACTH en vez de por la angiotensina II. En el HAP familiar tipo II, III y IV se produce un aumento de la transcripción y expresión de CYP11B2, responsable de la síntesis de aldosterona debido a una mutación germinal en CLCN2, KCNJ5 y CACNA1H, respectivamente. Por otra parte, se han identificado mutaciones somáticas en un 50% de los HAP esporádicos, siendo las mutaciones de ganancia de función a nivel de KCNJ5, ATP1A1, ATP2B3 y CACNA1D las más frecuentes. En esta revisión se ofrece una descripción detallada de las distintas formas de HAP familiar y sobre el perfil molecular de los pacientes con HAP esporádico. (AU)

Familial forms and molecular profile of primary hyperaldosteronism.

Primary hyperaldosteronism (PAH) is the most frequent cause of secondary arterial hypertension. Most PAHs occur sporadically, but 5% of cases have a hereditary origin (familial PAH). Four forms of familial PAH have been described. Type I familial PAH is produced by a fusion of the CYP11B2 and CYP11B1 genes, in this way the synthesis of aldosterone becomes to be regulated by ACTH instead of by angiotensin II. In type II, III and IV familial PAH there is an increase in the transcription and expression of CYP11B2 responsible for aldosterone synthesis due to a germinal mutation in CLCN2, KCNJ5 and CACNA1H, respectively. On the other hand, somatic mutations have been identified in 50% of sporadic PAHs, with gain-of-function mutations at the level of KCNJ5, ATP1A1, ATP2B3 and CACNA1D being the most common. This review provides a detailed description of the different forms of familial PAH and the molecular profile of patients with sporadic PAH.

Outcomes after laparoscopic adrenalectomy for unilateral primary aldosteronism / Resultados luego de adrenalectomía laparoscópica en hiperaldosteronismo primario unilateral

Abstract Unilateral primary aldosteronism (PA) is the most common surgically correctable cause of hypertension. Determination of success after laparoscopic adrenalectomy (LA) is limited by the lack of standardized criteria. We sought to evaluate the surgical recurrence and functional outcomes of LA in patients with Conn's syndrome applying the primary aldosteronism surgical outcome (PASO) Criteria. Descriptive obser vational analysis of patients treated with LA due to confirmed u nilateral Conn's syndrome between May 2007 and August 2020: Twenty patients were included in the cohort; 16 patients had TLA and other four PLA [58% male, median age 47 (IQR: 44-59.5) years and median follow-up of 64 (IQR: 2-156) ] months. Median tumor size was 1.2 (0.8-1.8) cm. No conversions to open surgery were recorded and the overall morbidity of the series was 1/20. No surgical or biochemical recurrence was observed. Five patients were excluded from the analysis of functional results due to lack of follow-up. According to the PASO criteria, complete, partial, and no success were observed in 8/15, 6/15, and 1/15, respectively. The surgical treatment of the disease is supported by the literature, and we were able to reproduce the results of other series. The use of standardized and reproducible criteria to assess its functional results would be essential for a more complete and integrated evaluation of adrenal surgery.

Resumen El hiperaldosteronismo primario es la causa más frecuente de hipertensión secundaria pasible de tratamiento quirúrgico. La determinación del éxito de la adrenalectomía laparoscópica (AL), actualmente, está limitada por la falta de criterios estandarizados. Buscamos evaluar la tasa de recurrencia quirúrgica y los resultados funcionales de la AL en pacientes con Síndrome de Conn aplicando los criterios PASO (primary aldosteronism surgical outcome). Análisis descriptivo y observacional de pacientes tratados con AL en contexto de síndrome de Conn unilateral confirmado, entre Mayo-2007 y Agosto-2020. Se incluyeron 20 pacientes en el estudio; 16 pacientes tratados mediante AL total y 4 con AL parcial (55% hombres, edad mediana de 47 (IQR: 44-59.5) años y mediana de seguimiento 64 (IQR: 2-156) meses. La mediana de tamaño tumoral fue de 1.2 (0.8-1.8) cm. No se registraron conversiones a cirugía abierta y la morbilidad global de la serie: 1/20. No se observó recurrencia quirúrgica o bioquímica. Se excluyeron 5 pacientes en el análisis de resultados funcionales por falta de seguimiento. Según los criterios PASO, se observó un éxito completo, parcial y ausente en 8/15, 6/15 y 1/15, respectivamente. El tratamiento quirúrgico de la enfermedad es avalado por la literatura y pudimos reproducir los resultados de otras series. El uso estandarizado y reproducible de criterios para valorar sus resul tados funcionales sería fundamental para una evaluación más completa e integrada de la cirugía suprarrenal.

Hipertensión secundaria: ¿cuándo y cómo buscarla? / Secondary hypertension: when and how to look for it?

Resumen En la población hipertensa se detecta una causa subyacente en 5% a 10%. Identificar etiología y establecer un tratamiento específico reduce el riesgo de daño de órgano blanco y, en algunos casos, puede curar la enfermedad primaria y la hipertensión. Dada su baja prevalencia, no se recomienda realizar una búsqueda exhaustiva de causas secundarias en todos los pacientes hipertensos. En este artículo se analiza cuándo y cómo debe estudiarse una hipertensión secundaria.

Abstract In the hypertensive population, between 5-10%, an underlying cause is detected. Identifying the etiology and giving specific treatment reduces the risk of hypertension-mediated organ damage and, in some cases, can cure the disease and high blood pressure. Due to its low prevalence, an exhaustive search for secondary causes is not recommended in all patients with hypertension. This article will discuss when and how to study secondary hypertension.

[Diagnosis of primary hyperaldosteronism]. / Diagnóstico del hiperaldosteronismo primario.

Primary aldosteronism (PA) is the most common cause of secondary arterial hypertension. Moreover, it is associated with a higher cardio-metabolic risk than the observed in patients with essential arterial hypertension (EHT). Therefore, a high index of clinical suspicion for PA is mandatory. If an elevated aldosterone/renin ratio is confirmed, confirmatory tests should be performed, with the exception in those patients with florid PA, with, with spontaneous hypokalaemia, plasma aldosterone > 20 ng/dL and suppressed renin, in whom this step would not be strictly necessary. Intravenous saline infusion test or captopril test are the commonly used confirmatory tests. The last step in the diagnosis of PA is the localization study, being the computerized axial tomography (CT) of the adrenal glands the initial test of choice, and adrenal venous sampling (AVS), the definitive localization test in most cases. This review summarizes the available data about the diagnosis of PA, from screening to confirmatory study and localization.

Tratamiento del hiperaldosteronismo primario durante el embarazo: reporte de un caso / Management of primary hyperaldosteronism during pregnancy: Case report

Resumen ANTECEDENTES: El hiperaldosteronismo primario es la principal causa de hipertensión arterial de origen endocrino en la población general; su manifestación durante el embarazo es poco frecuente, casi siempre provocado por un adenoma adrenal productor de aldosterona. De 1962 a la fecha se han descrito alrededor de 50 casos. Objetivo: Reportar un caso de hiperaldosteronismo durante el embarazo y revisar la bibliografía relacionada. CASO CLÍNICO: Paciente de 39 años, con antecedente médico de hipertensión arterial, en curso del segundo trimestre del quinto embarazo, que acudió a consulta por hipertensión no controlada e hipocalemia. El tratamiento incluyó un antagonista del receptor de aldosterona, que permitió el control de la tensión arterial y la finalización del embarazo. Posteriormente se identificó un nódulo adrenal, cuya resección resultó en normalización de las concentraciones de aldosterona, la actividad de renina plasmática, calemia y remisión de la hipertensión hasta la actualidad. CONCLUSIÓN: Las recomendaciones para el tratamiento de pacientes con hiperaldosteronismo durante el embarazo se basan en los casos publicados y los datos de toxicidad generados de estudios en animales. De ahí la importancia de este caso, que aporta información importante y puede considerarse en situaciones similares.

Abstract BACKGROUND: Primary hyperaldosteronism is the main cause of arterial hypertension of endocrine origin in the general population; its presentation during pregnancy is infrequent; having described about 50 cases since 1962, the most common cause is the presence of an adrenal adenoma. OBJECTIVE: To report the first case of hyperaldosteronism treated during pregnancy in Peru and reviews the literature. CLINICAL CASE: A 39-year-old patient with a history of arterial hypertension and poor obstetric history, who was referred to our center in the second trimester of the 5th pregnancy due to uncontrolled hypertension and symptomatic hypokalemia. Management included an aldosterone receptor antagonist, allowing the control of blood pressure and the culmination of the pregnancy with the delivery of a healthy girl. Subsequently, the presence of an adrenal nodule was confirmed, the resection of which resulted in normalization of aldosterone concentration, plasma renin activity, kalemia, and remission of hypertension to date. CONCLUSION: Recommendations on the management of hyperaldosteronism during pregnancy are based only on published cases and drug toxicity data were generated in animal studies. Hence the importance of this report, which provides information that can be considered in similar situations.

Aumento de la lipocalina orosomucoide-1 (ORM1) en sujetos con hiperaldosteronismo primario / Increase of lipocalin ORM1 in primary aldosteronism

El hiperaldosteronismo primario (HAP) es la causa más común de hipertensión arterial secundaria. A pesar de la prevalencia del HAP (6-10%) y sus consecuencias, los mecanismos que median los efectos deletéreos renales y extrarenales originados por la aldosterona más allá de la hipertensión arterial (ej. inflamación renal, alteraciones cardiacas y disfunción vascular), siguen siendo poco conocidos. Estudios previos sugieren que el exceso de aldosterona aumentaría proteínas sensibles a la activación del receptor de mineralocorticoides (MR), como las lipocalinas LCN2 (NGAL) y ORM1. OBJETIVO: Determinar la concentración de las lipocalinas ORM1, NGAL y NGAL-MMP9 en sujetos HAP. SUJETOS Y MÉTODOS: Estudio de cohorte transversal en sujetos adultos (similares en sexo, edad e IMC) separados en controles normotensos (CTL), hipertensos esenciales (HE) y con screening positivo de HAP (aldosterona ≥9 ng/dL y ARP < 1 ng/mL*h acorde a las guías internacionales de HAP). Se determinó la presión arterial sistólica (PAS) y diastólica (PAD), aldosterona plasmática, actividad renina plasmática (ARP) y la relación aldosterona / actividad de renina plasmática (ARR). Se determinó la concentración de NGAL, NGAL-MMP9 y ORM1 en suero por ELISA. RESULTADOS: Detectamos mayores niveles de ORM1 en sujetos HAP. No se detectaron diferencias en NGAL ni NGAL-MMP9 entre los grupos. Detectamos una asociación positiva de ORM1 con ARP (rho= -0,407, p=0,012) y con ARR (rho= 0,380 p= 0,021). CONCLUSIÓN: La mayor concentración de ORM1 en sujetos HAP y las asociaciones de ORM1 con aldosterona, ARP y ARR, proponen a esta proteína como un potencial biomarcador de HAP y de utilidad en el desarrollo de algoritmos diagnósticos de HAP.

Primary hyperaldosteronism (PA) is the most common cause of secondary hypertension. Despite the prevalence of PA (6-10%) and its consequences, the mechanisms that mediate the deleterious renal and extrarenal effects caused by aldosterone beyond arterial hypertension (eg renal inflammation, cardiac alterations and vascular dysfunction), remain barely known. Previous studies suggest that excess aldosterone would increase proteins sensitive to activation of the mineralocorticoid receptor (MR), such as lipocalins LCN2 (NGAL) and ORM1. AIM: To determine the concentration of the lipocalins ORM1, NGAL and NGAL-MMP9 in PA subjects. SUBJECTS AND METHODS: Cross-sectional study in adult subjects (similar in sex, age and BMI) grouped as normotensive controls (CTL), essential hypertensive (HE) and subjects with positive PA screening (aldosterone ≥ 9 ng/dL and PRA <1 ng/mL*h, according to international PA guidelines). Systolic (SBP) and diastolic (DBP) blood pressure, plasma aldosterone, plasma renin activity (PRA), and plasma aldosterone renin ratio (ARR) were determined. The concentration of NGAL, NGAL-MMP9 and ORM1 in serum was determined by ELISA. RESULTS: We detected higher levels Recibido: 03-09-2021 of ORM1 in PA subjects. No differences in NGAL or NGAL-MMP9 were detected between the groups. We detected a positive association of ORM1 with ARP (rho = -0.407, p < 0.05) and with ARR (rho = 0.380 p <0.05). CONCLUSION: The high levels of ORM1 in PA subjects and the associations of ORM1 with aldosterone, ARP and ARR, suggest ORM1 is a potential biomarker of PA, and useful in the development of a diagnostic algorithm for PA.

Riesgo cardiometabólico en pacientes con hiperaldosteronismo primario y secreción autónoma de cortisol. Estudio de casos y controles / Cardiometabolic risk in patients with primary aldosteronism and autonomous cortisol secretion. Case-control study

Objetivo: Analizar las diferencias en el perfil cardiometabólico de los pacientes con hiperaldosteronismo primario (HAP) y secreción autónoma de cortisol (SAC), emparejados por edad y sexo.MétodosEstudio de casos y controles; casos de HAP sin SAC asociada y como controles, pacientes con SAC (test de supresión de dexametasona ≥ 1.8 μg/dL en ausencia de datos específicos de hipercortisolismo), emparejados por edad y sexo. Se analizaron las comorbilidades HTA, diabetes, obesidad, dislipemia, insuficiencia renal crónica y eventos cardiovasculares y cerebrovasculares, así como su grado de control.ResultadosSe incluyeron 57 pacientes con HAP y 57 con SAC. Al diagnóstico, aparte de una mayor prevalencia de HTA en los pacientes con HAP (100 vs. 52,7%, p < 0,0001) y niveles más altos de TAS (143,2 [2,5] vs. 135,3 [2,6] mmHg, p = 0,032) que en SAC, no se detectaron diferencias en la prevalencia de otras comorbilidades. No obstante, los pacientes con SAC presentaban cifras más elevadas de HbA1c (p = 0,028).Tras una mediana de seguimiento de 2,25 años, los pacientes con HAP presentaron un mayor deterioro de la función renal (descenso medio del filtrado glomerular [MDRD-4] -17,4 [3] vs. -2,3 [4,4] mL/min/1,73 m2, p = 0,005) y del perfil lipídico (Δtriglicéridos de 34,5 [15,8] vs. -6,7 [11,3] mg/dL, p = 0,038) que los SAC.ConclusionesA pesar la mayor prevalencia de HTA en los pacientes con HAP que con SAC, emparejados por edad y sexo, no se detectaron diferencias en la prevalencia de otras comorbilidades cardiometabólicas. No obstante, los HAP presentaron un mayor deterioro de la función renal y del perfil lipídico a lo largo del seguimiento que el grupo de SAC. (AU)

Objective: To analyse the differences in the cardio-metabolic profile of patients with primary aldosteronism (PA) and autonomous cortisol secretion (ACS) matched by age and sex.MethodsCase-control study; cases of PA without associated ACS and as controls patients with ACS (dexamethasone suppression test ≥ 1.8 μg/dL in the absence of specific hypercortisolism clinical data), matched by age and sex. Comorbidities of hypertension, diabetes, obesity, dyslipidaemia, chronic kidney failure, and cardiovascular and cerebrovascular events were analysed, as well as their degree of control.Results57 patients with PA and 57 with ACS were included. On diagnosis, in addition to a higher prevalence of hypertension in the PA patients (100 vs. 52.7%, p < .0001) and higher systolic blood pressure levels (143.2 (2.5) vs. 135.3 (2.6) mmHg, p = .032) than in the ACS patients, no other differences were detected in the prevalence of other cardio-metabolic comorbidities. Nevertheless, the patients with ACS had higher HbA1c levels (p = .028) than the PA patients.After a median follow-up of 2.25 years, the patients with PA presented a greater deterioration in kidney function (Average decrease in glomerular filtration rate (MDRD-4) -17.4 (3.0) vs. -2.3 (4.4) mL/min/1.73 m2, p = .005) and lipid profile (Δtriglycerides of 34.5 (15.8) vs. -6.7 (11.3) mg/dL, p = .038) than the ACS patients.ConclusionsDespite the higher prevalence of hypertension in the patients with PA than in the patients with ACS matched by age and sex, no differences were detected in the prevalence of other cardio-metabolic comorbidities. However, the PA patients showed a greater deterioration in kidney function and lipid profile throughout the follow-up than the ACS patients. (AU)