Safety and efficacy of bilateral superselective adrenal arterial embolization for treatment of idiopathic hyperaldosteronism: a prospective single-center study
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SUBMITTER: Mao M
PROVIDER: S-EPMC11344387 | biostudies-literature | 2024 Jan
REPOSITORIES: biostudies-literature
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Project description:Peroral endoscopic myotomy (POEM) has been shown to be an effective treatment for achalasia and with few adverse events. Only a few cases of delayed bleeding have been described. This current case report describes a patient with delayed bleeding in the submucosal tunnel on the eighth day after POEM. The patient was a 21-year-old woman with a 4-month history of dysphagia, vomiting and excessive weight loss. Achalasia was diagnosed according to her symptoms, barium oesophagogram, oesophageal manometry and endoscopy examination. POEM was performed by an experienced operator. On the eighth day after POEM, the patient suddenly developed continuous haematemesis presented with vomiting of fresh blood and melena. An emergency exploratory esophagogastroduodenoscopy was performed. A large number of blood clots were found at the wound and a long haematoma was found along the lining of the submucosal tunnel. Re-entry into the submucosal tunnel and exposure of the haemorrhagic site was attempted but failed because of severe submucosal tissue adhesion. An emergency angiography was undertaken and haemostasis was achieved with superselective arterial microcoil embolization.
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Project description:Primary aldosteronism (PA) is the most common cause of secondary hypertension with a high prevalence among patients with resistant hypertension. Despite the recent discovery of somatic variants in aldosterone-producing adenoma (APA)-associated PA, causes for PA due to bilateral aldosterone production (bilateral hyperaldosteronism; BHA) remain unknown. Herein, we identified rare gene variants in ATP2B4, in a cohort of patients with BHA. ATP2B4 belongs to the same family of Ca-ATPases as ATP2B3, which is involved in the pathogenesis of APA. Endogenous ATP2B4 expression was characterized in adrenal tissue, and the gene variants were functionally analyzed for effects on aldosterone synthase (CYP11B2) expression, steroid production in basal and agonist-stimulated conditions, and for changes in biophysical properties of channel properties. Knockdown of ATP2B4 in HAC15 exhibited reduced angiotensin II stimulation in one of four shRNA clones. Stable HAC15 cell lines with doxycycline (dox) - inducible wild-type and variant forms of ATP2B4 - were generated, and dox-induced upregulation of ATP2B4 mRNA and protein was confirmed. However, ATP2B4 variants did not alter basal or agonist-stimulated CYP11B2 expression. Whole-cell recordings in HAC15 cells indicated robust endogenous ATP2B4 conductance in native cells but reduced conductance with overexpressed WT and variant ATP2B4. The previously defined PA-causing ATP2B3 variant served as a positive control and exhibited elevated CYP11B2 mRNA. In conclusion, while this study did not confirm a pathogenic role for ATP2B4 variants in BHA, we describe the sequencing analysis for familial and sporadic BHA and outline a template for the thorough in vitro characterization of gene variants.
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Project description:BackgroundPrimary aldosteronism (PA) is related with resistant hypertension and cardiovascular events. Adrenal artery embolization (AAE) is a choice for patients who refused surgery and medical therapy. However, whether AAE can effectively and safely treat PA is unclear. We performed this meta-analysis to determine the efficacy and safety of AAE for patients with PA.MethodsDatabases including Cochrane Library, Embase, PubMed and Web of Science were used to obtain relevant articles published before July 30, 2023. The primary outcome was blood pressure before and after AAE. The second outcomes included changes in plasma aldosterone level, serum potassium level, and plasma cortisol level.ResultsFinally, 7 prospective studies with 222 patients were included. The results showed that systolic and diastolic blood pressure was reduced by 21.68 mmHg (P<0.001) and 10.54 mmHg (P=0.007) respectively after AAE. The change in plasma aldosterone and serum potassium level was -11.52 ng/dL and 0.61 mmol/L respectively (P<0.001), whereas the reduction in cortisol level was not apparent. Moreover, AAE is a relatively safe procedure which only causes some minor complications such as back pain and fever.ConclusionsThis meta-analysis indicated that AAE could effectively and safely treat PA. It is a good choice for patients that are not suitable for adrenalectomy or drug therapy.
| S-EPMC11247589 | biostudies-literature
Project description:Purpose of reviewRenin-independent aldosterone production from one or both affected adrenal(s), a condition known as primary aldosteronism (PA), is a common cause of secondary hypertension. In this review, we aimed to summarize recent findings regarding pathophysiology of bilateral forms of PA, including sporadic bilateral hyperaldosteronism (BHA) and rare familial hyperaldosteronism.Recent findingsThe presence of subcapsular aldosterone synthase (CYP11B2)-expressing aldosterone-producing micronodules, also called aldosterone-producing cell clusters, appears to be a common histologic feature of adrenals with sporadic BHA. Aldosterone-producing micronodules frequently harbor aldosterone-driver somatic mutations. Other potential factors leading to sporadic BHA include rare disease-predisposing germline variants, circulating angiotensin II type 1 receptor autoantibodies, and paracrine activation of aldosterone production by adrenal mast cells. The application of whole exome sequencing has also identified new genes that cause inherited familial forms of PA.SummaryResearch over the past 10 years has significantly improved our understanding of the molecular pathogenesis of bilateral PA. Based on the improved understanding of BHA, future studies should have the ability to develop more personalized treatment options and advanced diagnostic tools for patients with PA.
| S-EPMC9202492 | biostudies-literature
Project description:PurposeThe aim of this study was to explore a radiomics-clinical model for predicting the response to initial superselective arterial embolization (SAE) in renal angiomyolipoma (RAML).Materials and methodsA total of 78 patients with RAML were retrospectively enrolled. Clinical data were recorded and evaluated. Radiomic features were extracted from preoperative contrast-enhanced CT (CECT). Least absolute shrinkage and selection operator (LASSO) and intra- and inter-class correlation coefficients (ICCs) were used in feature selection. Logistic regression analysis was performed to develop the radiomics, clinical, and combined models where the fivefold cross-validation method was used. The predictive performance and calibration were evaluated by the receiver operating characteristic (ROC) curve and calibration curve. Decision curve analysis (DCA) was used to measure clinical usefulness.ResultsThe tumor shrinkage rate was 29.7% in total, and both fat and angiomyogenic components were significantly reduced. In the radiomics model, 12 significant features were selected. In the clinical model, maximum diameter (p = 0.001), angiomyogenic tissue ratio (p = 0.032), aneurysms (p = 0.048), and post-SAE time (p = 0.002) were significantly associated with greater volume reduction after SAE. Because of the severe linear dependence between radiomics signature and some clinical parameters, the combined model eventually included Rad-score, aneurysm, and post-SAE time. The radiomics-clinical model showed better discrimination (mean AUC = 0.83) than the radiomics model (mean AUC = 0.60) and the clinical model (mean AUC = 0.82). Calibration curve and DCA showed the goodness of fit and clinical usefulness of the radiomics-clinical model.ConclusionsThe radiomics-clinical model incorporating radiomics features and clinical parameters can potentially predict the positive response to initial SAE in RAML and provide support for clinical treatment decisions.
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Project description:Primary aldosteronism affects ≈5% to 10% of hypertensive patients and has unilateral and bilateral forms. Most unilateral primary aldosteronism is caused by computed tomography-detectable aldosterone-producing adenomas, which express CYP11B2 (aldosterone synthase) and frequently harbor somatic mutations in aldosterone-regulating genes. The cause of the most common bilateral form of primary aldosteronism, idiopathic hyperaldosteronism (IHA), is believed to be diffuse hyperplasia of aldosterone-producing cells within the adrenal cortex. Herein, a multi-institution cohort of 15 IHA adrenals was examined with CYP11B2 immunohistochemistry and next-generation sequencing. CYP11B2 immunoreactivity in adrenal glomerulosa harboring non-nodular hyperplasia was only observed in 4/15 IHA adrenals suggesting that hyperplasia of CYP11B2-expressing cells may not be the major cause of IHA. However, the adrenal cortex of all IHA adrenals harbored at least 1 CYP11B2-positive aldosterone-producing cell cluster (APCC) or micro-aldosterone-producing adenomas. The number of APCCs per case (and individual APCC area) in IHA adrenals was significantly larger than in normotensive controls. Next-generation sequencing of DNA from 99 IHA APCCs demonstrated somatic mutations in genes encoding the L-type calcium voltage-gated channel subunit α 1-D ( CACNA1D, n=57; 58%) and potassium voltage-gated channel subfamily J-5 ( KCNJ5, n=1; 1%). These data suggest that IHA may result from not only hyperplasia but also the accumulation or enlargement of computed tomography-undetectable APCC harboring somatic aldosterone-driver gene mutations. The high prevalence of mutations in the CACNA1D L-type calcium channel provides a potential actionable therapeutic target that could complement mineralocorticoid blockade and inhibit aldosterone overproduction in some IHA patients.
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Project description:Cushing´s syndrome (CS) secondary to bilateral adrenal cortical disease may be caused by bilateral macronodular adrenal cortical disease (BMACD) or by bilateral micronodular adrenal cortical disease (miBACD). The size of adrenal nodules is a key factor for the differentiation between these two entities (>1cm, BMACD and <1cm; miBACD). BMACD can be associated with overt CS, but more commonly it presents with autonomous cortisol secretion (ACS). Surgical treatment of BMACD presenting with CS or with ACS and associated cardiometabolic comorbidities should be the resection of the largest adrenal gland, since it leads to hypercortisolism remission in up to 95% of the cases. Medical treatment focused on the blockade of aberrant receptors may lead to hypercortisolism control, although cortisol response is frequently transient. miBACD is mainly divided in primary pigmented nodular adrenocortical disease (PPNAD) and isolated micronodular adrenocortical disease (i-MAD). miBACD can present at an early age, representing one of the main causes of CS at a young age. The high-dose dexamethasone suppression test can be useful in identifying a paradoxical increase in 24h-urinary free cortisol, that is a quite specific in PPNAD. Bilateral adrenalectomy is generally the treatment of choice in patients with overt CS in miBACD, but unilateral adrenalectomy could be considered in cases with asymmetric disease and mild hypercortisolism. This article will discuss the clinical presentation, genetic background, hormonal and imaging features and treatment of the main causes of primary bilateral adrenal hyperplasia associated with hypercortisolism.
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Project description: Not available
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Project description:Purpose To conduct a pilot prospective clinical trial to evaluate the feasibility, safety, and short-term efficacy of bariatric embolization, a recently developed endovascular procedure for the treatment of obesity, in patients with severe obesity. Materials and Methods This is an institutional review board- and U.S. Food and Drug Administration-approved prospective physician-initiated investigational device exemption study. This phase of the study ran from June 2, 2014, to August 4, 2015. Five severely obese patients (four women, one man) who were 31-49 years of age and who had a mean body mass index of 43.8 kg/m2 ± 2.9 with no clinically important comorbidities were enrolled in this study. Transarterial embolization of the gastric fundus with fluoroscopic guidance was performed with 300-500-μm Embosphere microspheres. The primary end point was 30-day adverse events (AEs). The secondary end points included short-term weight loss, serum obesity-related hormone levels, hunger and satiety assessments, and quality of life (QOL) surveys, reported up to 3 months. Simple statistics of central tendencies and variability were calculated. No hypothesis testing was performed. Results The left gastric artery, with or without the gastroepiploic artery, was embolized in five patients, with a technical success rate of 100%. There were no major AEs. There were two minor AEs-subclinical pancreatitis and a mucosal ulcer that had healed by the time of 3-month endoscopy. A hospital stay of less than 48 hours for routine supportive care was provided for three patients. Mean excess weight loss of 5.9% ± 2.4 and 9.0% ± 4.1 was noted at 1 month and at 3 months, respectively. Mean change in serum ghrelin was 8.7% ± 34.7 and -17.5% ± 29 at 1 month and 3 months, respectively. Mean changes in serum glucagon-like peptide 1 and peptide YY were 106.6% ± 208.5 and 17.8% ± 54.8 at 1 month. There was a trend toward improvement in QOL parameters. Hunger/appetite scores decreased in the first 2 weeks after the procedure and then rose without reaching preprocedure levels. Conclusion Bariatric embolization is feasible and appears to be well tolerated in severely obese patients. In this small patient cohort, it appears to induce appetite suppression and may induce weight loss. Further expansion of this study will provide more insight into the long-term safety and efficacy of bariatric embolization. © RSNA, 2017 Online supplemental material is available for this article.
| S-EPMC5410948 | biostudies-literature
Project description:BackgroundConventional diagnostic approaches for adrenal tumors require multi-step processes, including imaging studies and dynamic hormone tests. Therefore, this study aimed to discriminate adrenal tumors from a single blood sample based on the combination of liquid chromatography-mass spectrometry (LC-MS) and machine learning algorithms in serum profiling of adrenal steroids.MethodsThe LC-MS-based steroid profiling was applied to serum samples obtained from patients with nonfunctioning adenoma (NFA, n=73), Cushing's syndrome (CS, n=30), and primary aldosteronism (PA, n=40) in a prospective multicenter study of adrenal disease. The decision tree (DT), random forest (RF), and extreme gradient boost (XGBoost) were performed to categorize the subtypes of adrenal tumors.ResultsThe CS group showed higher serum levels of 11-deoxycortisol than the NFA group, and increased levels of tetrahydrocortisone (THE), 20α-dihydrocortisol, and 6β-hydroxycortisol were found in the PA group. However, the CS group showed lower levels of dehydroepiandrosterone (DHEA) and its sulfate derivative (DHEA-S) than both the NFA and PA groups. Patients with PA expressed higher serum 18-hydroxycortisol and DHEA but lower THE than NFA patients. The balanced accuracies of DT, RF, and XGBoost for classifying each type were 78%, 96%, and 97%, respectively. In receiver operating characteristics (ROC) analysis for CS, XGBoost, and RF showed a significantly greater diagnostic power than the DT. However, in ROC analysis for PA, only RF exhibited better diagnostic performance than DT.ConclusionThe combination of LC-MS-based steroid profiling with machine learning algorithms could be a promising one-step diagnostic approach for the classification of adrenal tumor subtypes.
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