Differences in the clinical and hormonal presentation of patients with familial and sporadic primary aldosteronism.

Purpose: To compare the clinical and hormonal characteristics of patients with familial hyperaldosteronism (FH) and sporadic primary aldosteronism (PA). Methods: A systematic review of the literature was performed for the identification of FH patients. The SPAIN-ALDO registry cohort of patients with no suspicion of FH was chosen as the comparator group (sporadic group). Results: A total of 360 FH (246 FH type I, 73 type II, 29 type III, and 12 type IV) cases and 830 sporadic PA patients were included. Patients with FH-I were younger than sporadic cases, and women were more commonly affected (P = 0.003). In addition, the plasma aldosterone concentration (PAC) was lower, plasma renin activity (PRA) higher, and hypokalemia (P < 0.001) less frequent than in sporadic cases. Except for a younger age (P < 0.001) and higher diastolic blood pressure (P = 0.006), the clinical and hormonal profiles of FH-II and sporadic cases were similar. FH-III had a distinct phenotype, with higher PAC and higher frequency of hypokalemia (P < 0.001), and presented 45 years before sporadic cases. Nevertheless, the clinical and hormonal phenotypes of FH-IV and sporadic cases were similar, with the former being younger and having lower serum potassium levels. Conclusion: In addition to being younger and having a family history of PA, FH-I and III share other typical characteristics. In this regard, FH-I is characterized by a low prevalence of hypokalemia and FH-III by a severe aldosterone excess causing hypokalemia in more than 85% of patients. The clinical and hormonal phenotype of type II and IV is similar to the sporadic cases.

Renin as a Biomarker to Guide Medical Treatment in Primary Aldosteronism Patients. Findings from the SPAIN-ALDO Registry.

INTRODUCTION: Primary aldosteronism (PA) is associated with several cardiometabolic comorbidities. Specific treatment by mineralocorticoid receptor antagonists (MRA) or adrenalectomy has been reported to reduce the cardiometabolic risk. However, the cardiovascular benefit could depend on plasma renin levels in patients on MRA. AIM: To compare the development of cardiovascular, renal and metabolic complications between medically treated patients with PA and those who underwent adrenalectomy, taking the renin status during MRA treatment into account. METHODS: A multicenter retrospective study (SPAIN-ALDO Register) of patients with PA treated at 35 Spanish tertiary hospitals. Patients on MRA were divided into two groups based on renin suppression (n = 90) or non-suppression (n = 70). Both groups were also compared to unilateral PA patients (n = 275) who achieved biochemical cure with adrenalectomy. RESULTS: Adrenalectomized patients were younger, had higher plasma aldosterone concentration, and lower potassium levels than MRA group. Patients on MRA had similar baseline characteristics when stratified into treatment groups with suppressed and unsuppressed renin. 97 (55.1%) of 176 patients without comorbidities at diagnosis, developed at least one comorbidity during follow-up (median 12 months vs. 12.5 months' follow-up after starting MRA and surgery, respectively). Surgery group had a lower risk of developing new cardiovascular events (HR 0.40 [95% CI 0.18-0.90]) than MRA group. Surgical treatment improved glycemic and blood pressure control, increased serum potassium levels, and required fewer antihypertensive drugs than medical treatment. However, there were no differences in the cardiometabolic profile or the incidence of new comorbidities between the groups with suppressed and unsuppressed renin levels (HR 0.95 [95% CI 0.52-1.73]). CONCLUSION: Cardiovascular, renal, and metabolic events were comparable in MRA patients with unsuppressed and suppressed renin. Effective surgical treatment of PA was associated with a decreased incidence of new cardiovascular events when compared to MRA therapy.

Is adrenal venous sampling always necessary to differentiate between unilateral and bilateral primary aldosteronism? Lesson from the SPAIN-ALDO register.

PURPOSE: To evaluate whether the clinical, biochemical and radiological features of patients with primary aldosteronism (PA) can predict both main subtypes of PA. METHODS: A retrospective multicenter study of PA patients followed in 27 Spanish tertiary hospitals (SPAIN-ALDO Register). Only patients with confirmed unilateral or bilateral PA based on adrenal venous sampling (AVS) and/or postsurgical biochemical cure after adrenalectomy were included. Supervised regression techniques were used for model development. RESULTS: 328 patients [270 unilateral PA (UPA), 58 bilateral PA (BPA)] were included. The area under the curve (AUC) for aldosterone/potassium ratio and aldosterone responses following saline infusion test were 0.602 [95%CI 0.520 to 0.684] and 0.574 [95% CI 0.446-0.701], respectively, to differentiate UPA from BPA. The AUC was 0.825 [95% 0.764-0.886] when the prediction model with seven parameters - comorbidities (dyslipidemia, cerebrovascular disease, sleep apnea syndrome [SAS]), systolic blood pressure (SBP), plasma aldosterone levels (PAC), hypokalemia and unilateral adrenal nodule >1 cm and normal contralateral adrenal gland on CT/MRI - was used. In patients without comorbidities, hypokalemia, SBP > 160 mmHg, PAC > 40 ng/dL, and unilateral adrenal lesions were associated with a likelihood of having a UPA of 98.5%. The chance of BPA was higher in individuals with comorbidities, SBP < 140 mmHg, normokalemia, low PAC levels, and no adrenal tumors on the CT/MRI (91.5%). CONCLUSION: A combination of high PAC, SBP > 160 mmHg, low serum potassium, a unilateral adrenal nodule>1 cm and no comorbidities could predict a UPA with a 98.5% accuracy.

Effect of Obesity on Clinical Characteristics of Primary Aldosteronism Patients at Diagnosis and Postsurgical Response.

CONTEXT: Patients with obesity have an overactivated renin-angiotensin-aldosterone system (RAAS) that is associated with essential hypertension. However, the influence of obesity in primary aldosteronism (PA) is unknown. OBJECTIVE: We analyzed the effect of obesity on the characteristics of PA, and the association between obesity and RAAS components. METHODS: A retrospective study was conducted of the Spanish PA Registry (SPAIN-ALDO Registry), which included patients with PA seen at 20 tertiary centers between 2018 and 2022. Differences between patients with and without obesity were analyzed. RESULTS: A total of 415 patients were included; 189 (45.5%) with obesity. Median age was 55 years (range, 47.3-65.2 years) and 240 (58.4%) were male. Compared to those without obesity, patients with obesity had higher rates of diabetes mellitus, chronic kidney disease, obstructive apnea syndrome, left ventricular hypertrophy, prior cardiovascular events, higher means of systolic blood pressure, and required more antihypertensive drugs. Patients with PA and obesity also had higher values of serum glucose, glycated hemoglobin A1c, creatinine, uric acid, and triglycerides, and lower levels of high-density lipoprotein cholesterol. Levels of blood aldosterone (PAC) and renin were similar between patients with and without obesity. Body mass index was not correlated with PAC nor renin. The rates of adrenal lesions on imaging studies, as well as the rates of unilateral disease assessed by adrenal vein sampling or I-6ß-iodomethyl-19-norcholesterol scintigraphy, were similar between groups. CONCLUSION: Obesity in PA patients involves a worse cardiometabolic profile, and need for more antihypertensive drugs but similar PAC and renin levels, and rates of adrenal lesions and lateral disease than patients without obesity. However, obesity implicates a lower rate of hypertension cure after adrenalectomy.

Predictive model of hypertension resolution after adrenalectomy in primary aldosteronism: the SPAIN-ALDO score.

PURPOSE: To develop a predictive model of hypertension resolution after adrenalectomy in patients with primary aldosteronism (PA), based on their presurgical characteristics. METHODS: A retrospective multicenter study of PA patients in follow-up in 20 Spanish tertiary hospitals between 2018 and 2021 was performed (SPAIN-ALDO Register). Clinical response postadrenalectomy was classified according to the primary aldosteronism surgical outcome (PASO) consensus criteria. The predictive model was developed using a multivariate logistic regression model with the estimation of all possible equations. RESULTS: A total of 146 patients (54.8% females; mean age of 51.5 ± 10.9 years) with PA who underwent unilateral adrenalectomy were included. After a mean follow-up of 29.1 ±â€Š30.43 months after surgery, hypertension cure was obtained in 37.7% ( n  = 55) of the patients. The predictive model with the highest diagnostic accuracy to predict hypertension cure combined the variables female sex, use of two or fewer antihypertensive medications, hypertension grade 1, without type 2 diabetes and nonobesity. The area under the receiver operating characteristic curve of this model was 0.841 [0.769-0.914]. Based on this model, the group of patients with a higher probability of cure (80.4%) were those without type 2 diabetes, BMI <30 kg/m 2 , female sex, hypertension grade 1 and who use two or fewer antihypertensives. Our predictive model offered a slightly higher diagnostic accuracy than Wachtel's (area under the curve [AUC]: 0.809), Utsumi's (AUC: 0.804) and Zarnegar's (AUC: 0.796) models and was similar than the Burello's (AUC: 0.833) model. CONCLUSION: Female sex, use of two or fewer antihypertensive medications, hypertension grade 1, no type 2 diabetes and nonobesity may predict hypertension cure after adrenalectomy in patients with PA. Our score provides a potential tool to guide preoperative patient counseling.

Adrenal venous sampling in primary aldosteronism: Experience of a Spanish multicentric study (Results from the SPAIN-ALDO Register).

OBJECTIVE: The aim of this study was to evaluate the rate of adrenal venous sampling (AVS) performance in patients with primary aldosteronism (PA), the main reasons for its non-performance, and the success and complications rate of this procedure in Spain. Moreover, the concordance between CT/MRI and AVS for PA subtyping was evaluated. METHODS: A retrospective multicenter study of PA patient follow-up in 20 Spanish tertiary hospitals between 2018-2021 was performed (SPAIN-ALDO Register). RESULTS: Of the 440 patients with PA included in the study, 153 underwent AVS (34.8%). The main reasons for not performing AVS were: patient rejection to the procedure, low catheterization rate in the center and unilateral disease based on CT/MRI. The overall success rate was 44.4% (the left adrenal vein was properly canulated in 77.8% and the right adrenal vein in 48.4%). Only 3 patients experienced minor complications. In the 45 patients with unilateral disease according to AVS, CT/MRI indicated bilateral disease or normal adrenal glands in 17. In the 23 patients with bilateral disease, CT/MRI indicated unilateral disease in 14. However, no significant differences were observed in biochemical response (P = 0.051) and hypertension resolution (P = 0.150) between patients who underwent surgery based on CT/MRI results and those who underwent surgery based on AVS results. CONCLUSION: In our setting, AVS is still an underused technique in patients with PA. The low experience and success rate in AVS partially justify these results. More training for providers and patients needs to be done to include appropriate well performed AVS in the diagnosis algorithm of PA.

Doege-Potter syndrome in a facial solitary fibrous tumor: Diagnose and clinical management discussion.

Doege-Potter syndrome is a rare hypoglycemic paraneoplastic disorder. This case describes that severe and symptomatic hypoglycemia can occasionally be due to a rare malignant neoplasm, and the differential diagnosis of malignancy should not be overlooked in this setting.

Assessing the quality of the evidence underlying recommendations in type 2 diabetes' commonly used clinical practice guidelines.

AIM: In a world of data overload, clinical practice recommendations are needed to help practitioners and patients to take evidence-based decisions. However, in the field of type 2 diabetes mellitus (T2DM) recommendations on glycemic goals and treatment choice are controversial in spite of being supported by a common body of evidence. We hypothesize that internal and external validity of this body of evidence might not be as sound as expected. The aim of the current study is to appraise the studies supporting recommendations developed by influential medical societies dealing with glycemic goals and the choice of pharmacological treatment in adults with T2DM. METHODS: Clinical practice recommendations and their references were extracted out of eight documents developed by influential scientific societies between 2016 and 2019. Internal and external validity of each study was then appraised with standard tools and in duplicate. RESULTS: A total of 114 recommendations and their underlying 233 citations were extracted. Out of these 233 citations 81 (35%) corresponded to randomized controlled trials (RCT) and 45 (20%) to systematic reviews. After systematical appraisal only four RCT (5%) and eight systematic reviews (17%) were considered to be unflawed. Indirectness (lack of generalizability) was the most common caveat identified in RCTs. Out of the 114 recommendations analyzed (32 dealing with glycemic goals and 82 with treatment choice), only 21 (18.4%) were supported by at least one high-quality study. CONCLUSION: Only one in five recommendations regarding glycemic goals or pharmacological treatment choice in T2DM is based on at least one high-quality study. Clinical practice recommendations dealing with areas of uncertainty should be formulated more transparently to enable real evidence-based decisions.

Laparoscopic adrenalectomy for a large pheochromocytoma in a morbidly obese patient.

The high prevalence of obesity is associated with diverse health problems, including endocrine disorders. Laparoscopic adrenalectomy has become the preferred approach for removal of the adrenal gland, but several authors still debate the role of laparoscopic adrenalectomy in pheochromocytoma. We describe a case of a morbidly obese man (weight, 142 kg; body mass index, 40.2 kg/m(2)) who underwent elective laparoscopic adrenalectomy for a large right pheochromocytoma without incidences.

Retroperitoneal paragangliomas in obese patients.

Obesity, the most significant metabolic problem in the world today, is associated with a wide range of diseases, including endocrine disorders. Paraganglioma is a rare chromaffin cell tumor that develops from the neural crest cells of the neuroendocrine system. Retroperitoneal paragangliomas can represent a surgical challenge due to their close relation to large vessels. We report two cases of functioning retroperitoneal paraganglioma in type-I obese patients (case 1: female; weight, 77 kg; body mass index, 30.1 kg/m(2); case 2: male; weight, 92 kg; body mass index, 31.1 kg/m(2)) who underwent elective endocrine surgery. The tumors (one interaortocaval and the other above the iliac artery) were completely excised by laparotomy without postoperative complications.

Hiponatremia sintomática inducida por citalopram y escitalopram / Citalopram- and escitalopram-induced symptomatic hyponatremia

Se describen los casos clínicos de 2 pacientes que desarrollaron un síndrome de secreción inadecuada de vasopresina tras la instauración de tratamiento con fármacos inhibidores de la recaptación de serotonina, el citalopram y el escitalopram. Se trataba de 2 mujeres de 65 y 81 años, la primera consultó por cefalea, sensación de inestabilidad, náuseas y vómitos, la segunda paciente consultó por astenia generalizada y afectación del estado general. Las 2 tenían síndrome depresivo tratado con inhibidores de la recaptación de serotonina: citalopram 20 mg/día y escitalopram 10 mg/día, respectivamente. A propósito de estos 2 casos se realiza una revisión de la literatura sobre hiponatremia secundaria a citalopram y escitalopram y del mecanismo fisiopatológico desencadenante (AU)

We report the cases of 2 patients who developed syndrome of inappropriate antidiuretic hormone secretion (SIADH) after initiating therapy with the selective serotonin reuptake inhibitors, citalopram and escitalopram. Both the patients were women, aged 65 and 81 years old. The first consulted for headache, nausea and vomiting and the second for asthenia and malaise. Both were under treatment for depression with citalopram (20 mg/day) and escitalopram (10 mg/day), respectively. We review the literature on SIADH induced by citalopram and escitalopram and the pathophysiological mechanisms of this syndrome (AU)

Evolución de la cirugía laparoscópica adrenal en un servicio de cirugía general / Evolution of laparoscopic adrenal surgery in a general surgery department

Objetivo. Evaluar la reproducibilidad, la seguridad y los resultados a corto plazo de la cirugía laparoscópica adrenal en nuestro servicio durante los primeros años de desarrollo de dicha técnica. Pacientes y método. Análisis prospectivo, durante un período de 4 años, de todos los pacientes intervenidos de afección suprarrenal mediante abordaje laparoscópico en nuestra unidad de cirugía endocrina. Se analizan datos demográficos, enfermedad asociada, diagnóstico, tamaño glandular y de la tumoración, técnica quirúrgica, conversiones, complicaciones y estancia. El abordaje fue transperitoneal lateral en todos los pacientes. Resultados. Se realizaron 24 intervenciones (un caso bilateral) en 23 pacientes consecutivos, 15 mujeres y 8 varones, con una media de edad de 49,6 (intervalo, 20-72) años. Entre los antecedentes patológicos destacaron la obesidad y la hipertensión arterial en la mitad de los pacientes. Las indicaciones de la cirugía fueron: 8 incidentalomas, 6 aldosteronomas, 5 adenomas de Cushing, 3 feocromocitomas y 2 metástasis. Se practicó adrenalectomía derecha en 11 casos, izquierda en 11 y bilateral en 1. Hubo 3 conversiones a cirugía abierta. Como complicaciones hubo una infección respiratoria y una crisis hipertensiva. La mortalidad fue nula. El tiempo quirúrgico medio fue 125 (intervalo, 70-265) min. El tamaño medio de suprarrenales extirpadas fue 6,5 (intervalo, 4-14) cm. El tamaño medio de tumoraciones extirpadas fue 4,6 (intervalo, 1,5-12) cm. La estancia media fue 3,5 (intervalo, 2-11) días. Conclusiones. La cirugía laparoscópica adrenal es segura, reproducible y efectiva, con una baja tasa de complicaciones y bien tolerada por los pacientes. Al adquirir confianza en la técnica, el tiempo quirúrgico se reduce, así como la estancia hospitalaria (AU)

Objective. To evaluate the reproducibility, safety and short-term results of laparoscopic adrenal surgery during the first few years after its introduction in our department. Patients and method. A prospective analysis of data of all patients who underwent laparoscopic adrenalectomy in our endocrine surgical unit over a 4-year period. Demographic data, medical history, diagnosis, adrenal and tumour size, technique, conversions, complications and hospital stay were re-viewed. All patients were treated with a laparoscopic transperitoneal lateral approach. Results. Twenty-four laparoscopic adrenalectomies were performed on 23 consecutive patients (one underwent bilateral adrenalectomy). There were 15 females and 8 males; the mean age was 49.6 years (range, 20-72). There were obesity and vascular hypertension in half of the patients. The indications for surgery were: 8 incidentalomas, 6 aldosterone-producing adenomas, 5 Cushing’s adenomas, 3 phaeochromocytoma and 2 metastasis. Right adrenalectomy was performed on 11 patients, left on 11 and one was bilateral. Three cases required open conversion. Respiratory tract infection and hypertensive crisis were the postoperative complications. There was no mortality. The mean operative time was 125 minutes (range, 70-265). The mean size of adrenals excised was 6.5 cm (range, 4-14). The mean size of tumours excised was 4.6 cm (range, 1.5-12). The mean hospital stay was 3.5 days (range, 2-11). Conclusions. Laparoscopic adrenalectomy is a safe, reproducible and effective procedure with low complication rates, and well tolerated by the patients. The operating time and the length of hospital stay have decreased with the confidence of the technique (AU)

[Evolution of laparoscopic adrenal surgery in a general surgery department]. / Evolución de la cirugía laparoscópica adrenal en un servicio de cirugía general.

OBJECTIVE: To evaluate the reproducibility, safety and short-term results of laparoscopic adrenal surgery during the first few years after its introduction in our department. PATIENTS AND METHOD: A prospective analysis of data of all patients who underwent laparoscopic adrenalectomy in our endocrine surgical unit over a 4-year period. Demographic data, medical history, diagnosis, adrenal and tumour size, technique, conversions, complications and hospital stay were re-viewed. All patients were treated with a laparoscopic transperitoneal lateral approach. RESULTS: Twenty-four laparoscopic adrenalectomies were performed on 23 consecutive patients (one underwent bilateral adrenalectomy). There were 15 females and 8 males; the mean age was 49.6 years (range, 20-72). There were obesity and vascular hypertension in half of the patients. The indications for surgery were: 8 incidentalomas, 6 aldosterone-producing adenomas, 5 Cushing's adenomas, 3 phaeochromocytoma and 2 metastasis. Right adrenalectomy was performed on 11 patients, left on 11 and one was bilateral. Three cases required open conversion. Respiratory tract infection and hypertensive crisis were the postoperative complications. There was no mortality. The mean operative time was 125 minutes (range, 70-265). The mean size of adrenals excised was 6.5 cm (range, 4-14). The mean size of tumours excised was 4.6 cm (range, 1.5-12). The mean hospital stay was 3.5 days (range, 2-11). CONCLUSIONS: Laparoscopic adrenalectomy is a safe, reproducible and effective procedure with low complication rates, and well tolerated by the patients. The operating time and the length of hospital stay have decreased with the confidence of the technique.

Citalopram- and escitalopram-induced symptomatic hyponatremia.

We report the cases of 2 patients who developed syndrome of inappropriate antidiuretic hormone secretion (SIADH) after initiating therapy with the selective serotonin reuptake inhibitors, citalopram and escitalopram. Both the patients were women, aged 65 and 81 years old. The first consulted for headache, nausea and vomiting and the second for asthenia and malaise. Both were under treatment for depression with citalopram (20 mg/day) and escitalopram (10 mg/day), respectively. We review the literature on SIADH induced by citalopram and escitalopram and the pathophysiological mechanisms of this syndrome.