A hiperplasia adrenal unilateral primária (PUAH) é uma forma corrigível cirurgicamente de (hiper) aldosteronismo (AP) primário, caracterizada por supressão de renina, hipersecreção unilateral de aldosterona e hipertensão moderada a grave secundária à hiperplasia da glândula adrenal.
Introdução
O que você precisa saber de cara
A hiperplasia adrenal unilateral primária (PUAH) é uma forma corrigível cirurgicamente de (hiper) aldosteronismo (AP) primário, caracterizada por supressão de renina, hipersecreção unilateral de aldosterona e hipertensão moderada a grave secundária à hiperplasia da glândula adrenal.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 7 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 15 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Hiperplasia suprarrenal unilateral primária
Selecione um estado ou use sua localização para ver resultados.
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
Pesquisa e ensaios clínicos
0 ensaios clínicos encontrados.
Publicações mais relevantes
Effect of surgical versus conservative management on cardiovascular outcomes in patients with bilateral adrenal tumours and cortisol excess: an international, retrospective cohort study.
Treatment for patients with bilateral adrenal tumours and cortisol excess is not standardised and poses a therapeutic dilemma. Untreated cortisol excess is associated with cardiometabolic morbidity and mortality, but bilateral adrenalectomy causes adrenal insufficiency and possibly life-threatening adrenal crises. Data on cardiovascular outcomes by treatment modality are scarce. In this study we aimed to evaluate mid-term and long-term clinical and biochemical outcomes in patients with bilateral adrenal tumours and cortisol excess by treatment strategy and diagnosis. This retrospective, international cohort study (in 30 centres across 10 countries in Europe plus Singapore and the USA) included patients with bilateral adrenal tumours of 10 mm or larger, post-dexamethasone serum cortisol concentration of 50 nmol/L or higher, and at least 36 months of follow-up, with data collection beween Feb 2, 2024, and Jan 31, 2025. Patients were excluded if they had adrenocorticotropin hormone (ACTH)-dependent cortisol excess, ACTH-dependent nodular adrenal hyperplasia, partial glucocorticoid resistance syndrome, a diagnosis inconsistent with benign adrenocortical lesions, or received systemic oral or intravenous glucocorticoids other than replacement therapy following adrenalectomy. Primary endpoints were all-cause mortality and clinical and biochemical remission rates. Secondary endpoints were the incidence of cardiovascular events, prevalence of vascular and metabolic comorbidities, and incidence of adrenal crises. Of 629 patients who were diagnosed between Jan 1, 2000, and Jan 31, 2022, 105 (17%) had Cushing's syndrome and 524 (83%) had mild autonomous cortisol secretion (MACS), median age was 62 years (IQR 54·0-68·0), and 426 (68%) were female. 85 (81%) of 105 patients with Cushing's syndrome underwent surgery, and 384 (73%) of 524 patients with MACS received non-specific symptomatic treatment (ie, never underwent adrenalectomy or received steroidogenesis inhibitors). Over a median follow-up of 6·8 years, biochemical remission was achieved in 46 (45%) of 102 patients with Cushing's syndrome and in 67 (13%) of 517 patients with MACS. In both groups, 7% of patients died (Cushing's syndrome: seven of 105; MACS: 38 of 524) and 12% (13 of 105) of patients with Cushing's syndrome and 16% (82 of 524) of those with MACS had at least one cardiovascular event, without substantial differences across treatments. Smoking emerged as key modifiable mortality and cardiovascular risk factor in all patients, and in patients with MACS who only received non-specific symptomatic therapy, post-dexamethasone cortisol was also associated with increased mortality. Bilateral adrenalectomy led to full biochemical remission, few non-fatal adrenal crises, and improved arterial hypertension. Unilateral adrenalectomy and steroidogenesis inhibitors yielded heterogeneous biochemical outcomes and no substantial comorbidity improvement. Non-specific symptomatic treatment in MACS was associated with worsening of all investigated comorbidities. Although mortality and cardiovascular event rates were similar across treatments, surgery led to better biochemical control and more favourable comorbidity outcomes. None.
Genetic background and management outcomes in primary bilateral macronodular adrenal hyperplasia: Implications for diagnosis and treatment-A retrospective cohort study.
ObjectivePrimary bilateral macronodular adrenal hyperplasia (PBMAH) is a rare cause of Cushing syndrome. Recent evidence, particularly the 2023 European Society of Endocrinology guidelines, clinically classifies cortisol excess into overt Cushing syndrome and mild autonomous cortisol secretion. Germline mutations in the Armadillo repeat-containing 5 (ARMC5) gene have been identified in 20%-55% of patients with PBMAH. This study aimed to describe the clinical, radiological, and biochemical characteristics of PBMAH; classify cortisol secretion according to updated guidelines; and evaluate surgical and conservative treatment outcomes.MethodsThis retrospective cohort study included 58 patients with bilateral adrenal macronodules who underwent ARMC5 genetic testing at a tertiary center between 2023 and 2025. Clinical, imaging, laboratory, and genetic data were collected. Mild autonomous cortisol secretion was defined according to the 2023 European guidelines as a post-dexamethasone suppression test cortisol level >1.8 μg/dL in the absence of overt Cushing syndrome features. Postoperative and follow-up data, including hormonal assessments and remission criteria, were recorded.ResultsA total of 58 patients were included; 14 (23.7%) were male, and the mean age was 57.7 years (39-73). Four patients (6.9%) with overt Cushing syndrome carried germline ARMC5 mutations, three of whom belonged to the same family, supporting an autosomal dominant pattern of inheritance. Among them, 34 patients with mild autonomous cortisol secretion and 20 patients with nonfunctional PBMAH were managed conservatively. Unilateral laparoscopic adrenalectomy was performed in five patients, all of whom achieved biochemical and clinical remission during follow-up (median, 14 months). No postoperative adrenal insufficiency or persistent hypercortisolism was observed.ConclusionPBMAH demonstrates a broad clinical spectrum, ranging from nonfunctional disease to overt Cushing syndrome. The updated classification of cortisol secretion (mild autonomous cortisol secretion vs. overt Cushing syndrome) improves clinical stratification and supports treatment decision-making. ARMC5 genetic analysis contributes to diagnostic confirmation, facilitates cascade family screening, and enables identification of asymptomatic carriers. Unilateral adrenalectomy is effective in patients with overt Cushing syndrome, whereas surveillance is appropriate for those with mild autonomous cortisol secretion or nonfunctional PBMAH.
Genetic mechanisms of primary aldosteronism.
Primary aldosteronism (PA) represents the leading cause of secondary hypertension, resulting from autonomous aldosterone production driven in the majority of cases by a lateralized aldosterone‑producing adenoma or by bilateral adrenal hyperplasia. Its frequency increases in parallel with hypertension severity, reaching a prevalence of up to 25% of patients with treatment resistant hypertension. Advances in our understanding on the genetic causes of PA have reshaped our understanding of the pathogenesis of the disease, revealing a broad spectrum of somatic and inherited mutations across most aldosterone‑producing adenomas as well as familial forms of the disorder. More recently, susceptibility loci shared between unilateral and bilateral PA, and overlapping with known blood‑pressure associated variants, have been identified, highlighting genetic susceptibility that extends beyond classic PA to hypertension in the general population. Associated with clinical and biochemical evidence of a continuum of aldosterone dysregulation in hypertension, these discoveries suggest that common genetic variants may drive aldosterone dysregulation in a large fraction of hypertensive subjects leading, in extreme cases, to overt PA.
KCNJ5-Negative Primary Aldosteronism: Histopathology and Surgical Outcomes.
Unilateral primary aldosteronism (uPA) is the most common surgically curable secondary hypertension. KCNJ5 mutations are frequent in aldosterone-producing adenomas, but the clinical significance of KCNJ5-negative uPA remains unclear. We investigated whether histopathologic subtypes defined by the HISTALDO (Histopathology of Primary Aldosteronism) consensus are associated with postoperative outcomes in KCNJ5-negative uPA. We analyzed 171 KCNJ5-negative uPA patients from the Taiwan Primary Aldosteronism Investigation registry who underwent adrenalectomy. Somatic mutations were identified by Sanger sequencing and targeted next-generation sequencing. Adrenal histopathology was classified as classical (solitary adenoma or dominant nodule) or nonclassical (multiple nodules, micronodules, or diffuse hyperplasia) per the HISTALDO criteria. Postsurgical outcomes at 12 months were assessed using the Primary Aldosteronism Surgical Outcome consensus criteria. The cohort (mean age, 55.0±11.3 years; 53.8% women) comprised 90 (52.6%) patients with classical histopathology and 81 (47.4%) with nonclassical features. Complete clinical success was achieved in 40.9% of patients at 12 months. Patients with classical histopathology had significantly higher complete clinical success (50%) than those with nonclassical histopathology (30.9%; P=0.01). On multivariate analysis, nonclassical histopathology (odds ratio, 0.33 [95% CI, 0.16-0.68]; P=0.003), higher preoperative serum creatinine (odds ratio, 0.24; P=0.022), and higher diastolic blood pressure (odds ratio, 0.97; P=0.017) independently predicted failure to achieve complete clinical success. Nearly half of KCNJ5-negative uPA cases exhibit nonclassical adrenal histopathology, which is associated with significantly lower complete clinical success rates. These findings underscore the prognostic value of histopathologic classification in KCNJ5-negative uPA and support integrating histology and genetics to improve patient management.
Primary bilateral macronodular adrenal hyperplasia presenting as resistant hypertension with bilateral adrenal masses: a case report.
Bilateral adrenal incidentalomas are uncommon and may be associated with autonomous cortisol secretions. One important etiology is primary bilateral macronodular adrenal hyperplasia (PBMAH), a rare cause of adrenocorticotropic hormone (ACTH)-independent Cushing's syndrome, which may present without typical Cushingoid features. We describe a patient with ACTH-independent Cushing's syndrome due to bilateral adrenal hyperplasia, who achieved clinical improvement after unilateral adrenalectomy. This case highlights the role of unilateral adrenalectomy as a functional treatment option in PBMAH. A 59-year-old man presented with resistant hypertension, bilateral leg edema, and proximal muscle weakness, without typical Cushingoid features. Biochemical evaluation confirmed autonomous cortisol secretion, and computed tomography revealed multiple bilateral adrenal masses. Adrenal venous sampling (AVS) demonstrated left-sided dominant cortisol production, leading to laparoscopic left adrenalectomy. Histopathology confirmed macronodular adrenal hyperplasia, and the patient experienced postoperative improvement in both hypertension and glycemic control. PBMAH often presents as bilateral adrenal enlargement with cortisol excess, and AVS plays a role in determining functional lateralization in selected patients. Because adrenal size strongly correlates with cortisol secretion, imaging and AVS together help refine surgical decision making. While unilateral adrenalectomy offers favorable outcomes, recurrence rates vary widely, highlighting the need for continued surveillance. For patients with ACTH-independent Cushing's syndrome and bilateral adrenal masses, AVS-guided unilateral adrenalectomy provides a targeted treatment strategy that controls cortisol excess while reducing the risk of permanent adrenal insufficiency.
Publicações recentes
Primary Unilateral Adrenal Anaplastic Large Cell Lymphoma: Remission by Chemotherapy.
Primary adrenal tuberculosis infection in patients with Behcet's disease presenting as isolated adrenal metastasis by (18)F-FDG PET/CT: a rare case report and literature review.
A case report of primary unilateral adrenal NK/T cell lymphoma: good clinical outcome with trimodality treatment.
Double-hit primary unilateral adrenal lymphoma with good outcome.
Aldosterone-producing adenoma and other surgically correctable forms of primary aldosteronism.
📚 EuropePMC1 artigos no totalmostrando 196
Genetic background and management outcomes in primary bilateral macronodular adrenal hyperplasia: Implications for diagnosis and treatment-A retrospective cohort study.
The Journal of international medical researchGenetic mechanisms of primary aldosteronism.
Annales d'endocrinologieKCNJ5-Negative Primary Aldosteronism: Histopathology and Surgical Outcomes.
Hypertension (Dallas, Tex. : 1979)Primary bilateral macronodular adrenal hyperplasia presenting as resistant hypertension with bilateral adrenal masses: a case report.
Annals of medicine and surgery (2012)New mutation of CACNA1H p.Tyr613Phe in hyperaldosteronism: a case report.
Frontiers in medicineEffect of surgical versus conservative management on cardiovascular outcomes in patients with bilateral adrenal tumours and cortisol excess: an international, retrospective cohort study.
The lancet. Diabetes & endocrinologySelected updates in thyroid, parathyroid, and adrenal gland pathology.
Human pathologyLow-renin primary aldosteronism predicts superior surgical outcomes compared to high-renin disease.
BMC urologyPostoperative adrenal cortical insufficiency following adrenal adenoma removal: a case report.
AME case reportsTwo cases of MACS due to PBMAH associated with an in vivo aberrant response to LHRH treated with leuprolide acetate.
Endocrinology, diabetes & metabolism case reportsFactors affecting clinical outcomes of minimally invasive adrenalectomy for unilateral primary aldosteronism: a single-center retrospective cohort study.
Gland surgeryPrimary bilateral macronodular adrenal hyperplasia (PBMAH): from rare to common cause of Cushing syndrome in clinical practice.
Updates in surgeryPrimary aldosteronism and long-term outcomes using PAMO definition.
Hypertension research : official journal of the Japanese Society of HypertensionApproach to the Patient With Primary Aldosteronism: Role of Molecular Imaging.
The Journal of clinical endocrinology and metabolismPrimary aldosteronism patients with bilateral adrenal vein sampling success achieve better outcomes through unilateral adrenalectomy.
Translational andrology and urologyBilateral Adrenal Nodules Presenting With Mild Autonomous Cortisol Secretion.
Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical EndocrinologistsRefractory Hypokalemia in the Context of Bilateral Adrenal Hyperplasia Leading to Diagnostic Reassessment and Curative Surgery.
CureusEfficacy of Propranolol Combined with Different Modalities of Unilateral Adrenalectomy in a Case Series of 4 ARMC5-mutated Patients.
Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes AssociationLC-MS/MS-Based Assay for Steroid Profiling in Peripheral and Adrenal Venous Samples for the Subtyping of Primary Aldosteronism.
Journal of clinical hypertension (Greenwich, Conn.)Prevalence of KCNJ5 mutations in aldosterone-producing adenomas among Malaysian primary aldosteronism patients: Genotype-phenotype correlation.
The Malaysian journal of pathologyPrevalence and main characteristics of primary aldosteronism in bilateral macronodular adrenal disease: a systematic review of the literature.
European journal of endocrinologyAcute Myocardial Infarction With Nonobstructive Coronary Arteries Across Endocrine Disorders: A Case Series.
JCEM case reportsHypertensive Urgency With Primary Hyperaldosteronism Due to Unilateral Idiopathic Adrenal Hyperplasia: A Case of Headache With Severe Hypokalemia in a Young Male.
CureusConcurrent Adrenal Adenoma, Unilateral Adrenal Hyperplasia, and Bilateral Renal Artery Stenosis in a 61-Year-Old Man.
American journal of men's health68Ga-pentixafor PET/CT Is a Supplementary Method for Primary Aldosteronism Subtyping Compared with Adrenal Vein Sampling.
Molecular imaging and biologyPrimary bilateral macronodular adrenal hyperplasia: A rare case report of Cushing syndrome and review of literature.
MedicineCirculating miRNAs and Machine Learning for Lateralizing Primary Aldosteronism.
Hypertension (Dallas, Tex. : 1979)Staining patterns of aldosterone synthase in patients undergoing surgery for primary aldosteronism: Proposal for system of categorization and investigation of clinical and biochemical correlation.
SurgeryThe molecular genetics of adrenal cushing.
Hormones (Athens, Greece)Long-term outcome of unilateral adrenalectomy for primary bilateral macronodular adrenal hyperplasia.
EndocrineSynchronous Seminoma of Testis and Renal Cell Carcinoma: A Rare Case Report.
Medicina (Kaunas, Lithuania)Aberrant hormone receptors regulate a wide spectrum of endocrine tumors.
The lancet. Diabetes & endocrinologyBilateral adrenal artery embolization for the treatment of idiopathic hyperaldosteronism: A proof-of-principle single center study.
Hypertension research : official journal of the Japanese Society of Hypertension[131I]6ß-Iodomethyl-19-norcholesterol SPECT/CT for the Lateralization of Mineralocorticoid Overproduction in Primary Aldosteronism.
Diagnostics (Basel, Switzerland)Typing diagnostic value of 68Ga-pentixafor PET/CT for patients with primary aldosteronism and unilateral nodules.
EndocrinePrimary unilateral macronodular adrenal hyperplasia with concomitant glucocorticoid and androgen excess and KDM1A inactivation.
European journal of endocrinologyClinical Review: The Approach to the Evaluation and Management of Bilateral Adrenal Masses.
Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical EndocrinologistsFrom the First Case Reports to KDM1A Identification: 35 Years of Food (GIP)-Dependent Cushing's Syndrome.
Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes AssociationClassic congenital adrenal hyperplasia with unilateral functional adrenal cortical adenoma: case report.
Gynecological endocrinology : the official journal of the International Society of Gynecological EndocrinologyClinical prediction model for primary aldosteronism subtyping and special focus on adrenal volumetric assessment.
Hormones (Athens, Greece)Conn´s syndrome after kidney transplantation.
Bratislavske lekarske listyA comparison of the performance of 68Ga-Pentixafor PET/CT versus adrenal vein sampling for subtype diagnosis in primary aldosteronism.
Frontiers in endocrinologyDetermination of three C18-oxygenated steroids in adrenal lesion segments in primary aldosteronism by super-selective adrenal venous sampling and LC/ESI-MS/MS.
Biomedical chromatography : BMCWhat We Know about and What Is New in Primary Aldosteronism.
International journal of molecular sciencesDelineating endogenous Cushing's syndrome by GC-MS urinary steroid metabotyping.
EBioMedicineAtypical Presentation and Course of ACTH-Independent Cushing’s Syndrome in Two Families.
Journal of clinical research in pediatric endocrinologyEmerging Potential of Microwave Ablation for Primary Aldosteronism Resulting From Unilateral Aldosterone-producing Adenoma.
JCEM case reportsPrimary aldosteronism 2.0: an update for clinicians on diagnosis and treatment.
Polish archives of internal medicineFluorinated aldosterone synthase (CYP11B2)-inhibitors for differential diagnosis between bilateral and unilateral conditions of primary aldosteronism.
Bioorganic & medicinal chemistry lettersCorticotropin-releasing hormone test predicts the outcome of unilateral adrenalectomy in primary bilateral macronodular adrenal hyperplasia.
Journal of endocrinological investigationKey to the Treatment of Primary Aldosteronism in Secondary Hypertension: Subtype Diagnosis.
Current hypertension reportsUnexpected exacerbation of cryptococcal meningitis after unilateral adrenalectomy in a PMAH patient: a case report and literature review.
BMC endocrine disordersRole of Adrenal Vein Sampling in Guiding Surgical Decision in Primary Aldosteronism.
Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes AssociationStrategies for subtyping primary aldosteronism.
Journal of the Formosan Medical Association = Taiwan yi zhiThe value of CT-based energy imaging to discriminate dominant side lesions in primary aldosteronism.
Frontiers in endocrinologyPrimary bilateral macronodular adrenal hyperplasia: A series of 32 cases and literature review.
Endocrinologia, diabetes y nutricion[Adrenalectomy for primary hyperaldosteronism : retrospective review of 57 patients in CHU of Liege].
Revue medicale de LiegeContrast medium free selective adrenal vein sampling in the management of primary aldosteronism.
Clinical imagingDiagnostic accuracy of using multiple cytokines to predict aldosterone-producing adenoma.
Scientific reportsRadiation of bilateral adrenal metastases is associated with a high risk of primary adrenal insufficiency.
Clinical endocrinology[The genetic background of primary aldosteronism].
Orvosi hetilapExtensive expertise in endocrinology: glucose-dependent insulinotropic peptide-dependent Cushing's syndrome.
European journal of endocrinologyUnilateral adrenalectomy in bilateral adrenal hyperplasia with primary aldosteronism.
Journal of the Formosan Medical Association = Taiwan yi zhiThe effect of unilateral adrenalectomy on patients with primary bilateral macronodular adrenal hyperplasia.
Hormones (Athens, Greece)CUSHING'S SYNDROME DUE TO PRIMARY BILATERAL MACRONODULAR ADRENAL HYPERPLASIA AND METACHRONOUS PHEOCHROMOCYTOMA.
Acta endocrinologica (Bucharest, Romania : 2005)Steroid profiling using liquid chromatography mass spectrometry during adrenal vein sampling in patients with primary bilateral macronodular adrenocortical hyperplasia.
Frontiers in endocrinologyClinical, Pathophysiologic, Genetic, and Therapeutic Progress in Primary Bilateral Macronodular Adrenal Hyperplasia.
Endocrine reviewsResolution of manic symptoms following unilateral adrenalectomy in patient with primary bilateral macronodular adrenal hyperplasia.
Urology case reportsScreening for unilateral aldosteronism should be combined with the maximum systolic blood pressure, history of stroke and typical nodules.
MedicinePrimary aldosteronism: Pathophysiological mechanisms of cell death and proliferation.
Frontiers in endocrinologyCXCR4-directed [68Ga]Ga-PentixaFor PET/CT versus adrenal vein sampling performance: a study protocol for a randomised two-step controlled diagnoStic Trial Ultimately comparing hypertenSion outcome in primary aldosteronism (CASTUS).
BMJ openA novel pathogenic variant of ARMC5 in a patient with primary bilateral macronodular adrenal hyperplasia: a case report.
BMC endocrine disordersCushing´s syndrome due to bilateral adrenal cortical disease: Bilateral macronodular adrenal cortical disease and bilateral micronodular adrenal cortical disease.
Frontiers in endocrinologyUnilateral or bilateral adrenalectomy in PPNAD: six cases from a single family followed up over 40 years.
EndocrineDecreased steroidogenic enzyme activity in benign adrenocortical tumors is more pronounced in bilateral lesions as determined by steroid profiling in LC-MS/MS during ACTH stimulation test.
Endocrine connectionsFamilial Hyperaldosteronism Type 3 with a Rapidly Growing Adrenal Tumor: An In Situ Aldosterone Imaging Study.
Current issues in molecular biologyThe Relationship Between Baseline Cortisol Levels and Surgery Method of Primary Bilateral Macronodular Adrenal Hyperplasia.
Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolismePrevalence and clinical characteristics of primary aldosteronism in a tertiary-care center in Korea.
Hypertension research : official journal of the Japanese Society of HypertensionBilateral adrenal uptake of 123I MIBG scintigraphy with mild catecholamine elevation, the diagnostic dilemma, and its characteristics.
Scientific reportsCurable congestive heart failure in patients with primary aldosteronism: two cases reports.
American journal of translational researchExploration of KCNJ5 Somatic Mutation and CYP11B1/CYP11B2 Staining in Multiple Nodules in Primary Aldosteronism.
Frontiers in medicineA 30-Year, Single-Center Experience of Unilateral Adrenalectomy for Primary Bilateral Macronodular Adrenal Hyperplasia.
Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical EndocrinologistsCase Report: A Novel ARMC5 Germline Mutation in a Patient with Primary Bilateral Macronodular Adrenal Hyperplasia and Hypogammaglobulinemia.
Frontiers in geneticsThe role of adrenal venous sampling (AVS) in primary bilateral macronodular adrenocortical hyperplasia (PBMAH): a study of 16 patients.
EndocrineThe Accuracy of Simple and Adjusted Aldosterone Indices for Assessing Selectivity and Lateralization of Adrenal Vein Sampling in the Diagnosis of Primary Aldosteronism Subtypes.
Frontiers in endocrinologyAdrenal androgens versus cortisol for primary aldosteronism subtype determination in adrenal venous sampling.
Clinical endocrinology[Diagnosis and therapy of primary aldosteronism: new aspects].
Deutsche medizinische Wochenschrift (1946)A case with primary hyperaldosteronism associated with chronic kidney disease.
Radiology case reportsDiagnosis and treatment of primary aldosteronism.
The lancet. Diabetes & endocrinologyPathophysiology and histopathology of primary aldosteronism.
Trends in endocrinology and metabolism: TEMBilateral Adrenal Hyperplasia: Pathogenesis and Treatment.
BiomedicinesHypertensive Emergency in a Young Patient With Unilateral Adrenal Hyperplasia: A Case of Conn's Syndrome.
CureusLoss of KDM1A in GIP-dependent primary bilateral macronodular adrenal hyperplasia with Cushing's syndrome: a multicentre, retrospective, cohort study.
The lancet. Diabetes & endocrinologyAdrenal venous sampling in the diagnostic workup of primary aldosteronism.
The British journal of radiologyUtility of Epinephrine Levels in Determining Adrenal Vein Cannulation During Adrenal Venous Sampling for Primary Aldosteronism.
Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical EndocrinologistsUnilateral adrenalectomy partially improved hyperglycemia in a patient with primary bilateral macronodular adrenal hyperplasia.
Diabetology international[The analysis of related factors on the unilateral aldosterone secretion in the functional diagnosis of primary aldosteronism and establishment of a predictive model].
Zhonghua yi xue za zhi[Controversy and strategy of partial versus total adrenalectomy in the treatment of primary aldosteronism].
Zhonghua yi xue za zhiSertoli Leydig Cell Tumour Initially Misdiagnosed as Polycystic Ovarian Syndrome and Congenital Adrenal Hyperplasia: A Case Report.
JNMA; journal of the Nepal Medical AssociationReassessment of Postural Stimulation Testing as a Simple Tool to Identify a Subgroup of Patients With Unilateral Primary Aldosteronism.
The Journal of clinical endocrinology and metabolismParametric mapping for 11C-metomidate PET-computed tomography imaging in the study of primary aldosteronism.
Nuclear medicine communicationsOvarian Steroid Cell Tumor Masquerading as Steroid-Unresponsive Congenital Adrenal Hyperplasia.
AACE clinical case reportsSomatic mutations in adrenals from patients with primary aldosteronism not cured after adrenalectomy suggest common pathogenic mechanisms between unilateral and bilateral disease.
European journal of endocrinologySubtype-specific trends in the clinical picture of primary aldosteronism over a 13-year period.
Journal of hypertensionTherapeutic Outcomes with Surgical and Medical Management of Primary Aldosteronism.
Current cardiology reportsPathology of Aldosterone Biosynthesis and its Action.
The Tohoku journal of experimental medicineClinical characteristics and genetic analyses in a Chinese family affected by primary aldosteronism: a case report.
Annals of palliative medicinePartial Adrenalectomy Carries a Considerable Risk of Incomplete Cure in Primary Aldosteronism.
The Journal of urologyFeasibility of Iodine-131 6β-Methyl-Iodo-19 Norcholesterol (NP-59) Scintigraphy to Complement Adrenal Venous Sampling in Management of Primary Aldosteronism: A Case Series.
International journal of general medicineThe Genotype-Based Morphology of Aldosterone-Producing Adrenocortical Disorders and Their Association with Aging.
Endocrinology and metabolism (Seoul, Korea)Role of unilateral adrenalectomy in bilateral adrenal hyperplasias with Cushing's syndrome.
Best practice & research. Clinical endocrinology & metabolismUpdate on primary bilateral macronodular adrenal hyperplasia (PBMAH).
EndocrineA case of renovascular hypertension with incidental primary bilateral macronodular adrenocortical hyperplasia.
Endocrinology, diabetes & metabolism case reportsUpdate: Selective adrenal venous sampling (AVS) - Indication, technique, and significance.
RoFo : Fortschritte auf dem Gebiete der Rontgenstrahlen und der NuklearmedizinAnalysis of clinical and pathological features of primary bilateral macronodular adrenocortical hyperplasia compared with unilateral cortisol-secreting adrenal adenoma.
Annals of translational medicineErythromelalgia Caused by Polycythemia Vera Combined with Primary Aldosteronism.
European journal of case reports in internal medicinePhosphodiesterase 2A and 3B variants are associated with primary aldosteronism.
Endocrine-related cancerMinimally invasive partial versus total adrenalectomy for unilateral primary hyperaldosteronism-a retrospective, multicenter matched-pair analysis using the new international consensus on outcome measures.
SurgeryAldosterone is Aberrantly Regulated by Various Stimuli in a High Proportion of Patients with Primary Aldosteronism.
The Journal of clinical endocrinology and metabolismMolecular Basis of Primary Aldosteronism and Adrenal Cushing Syndrome.
Journal of the Endocrine Society[Primary hyperaldosteronism: difficulties in diagnosis].
Terapevticheskii arkhivGenetic and Genomic Mechanisms of Primary Aldosteronism.
Trends in molecular medicinePrecise Mapping of Intra-Adrenal Aldosterone Activities Provides a Novel Surgical Strategy for Primary Aldosteronism.
Hypertension (Dallas, Tex. : 1979)Approach to patients with bilateral adrenal incidentalomas.
Current opinion in endocrinology, diabetes, and obesityImaging CXCR4 expression in patients with suspected primary hyperaldosteronism.
European journal of nuclear medicine and molecular imagingMicroRNAs and Adrenocortical Tumors: Where do we Stand on Primary Aldosteronism?
Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolismeAllelic Variants of ARMC5 in Patients With Adrenal Incidentalomas and in Patients With Cushing's Syndrome Associated With Bilateral Adrenal Nodules.
Frontiers in endocrinologyDiagnosis and management of primary bilateral macronodular adrenal hyperplasia.
Endocrine-related cancerThe pathogenic role of the GIP/GIPR axis in human endocrine tumors: emerging clinical mechanisms beyond diabetes.
Reviews in endocrine & metabolic disordersMolecular Mechanisms of Primary Aldosteronism.
Endocrinology and metabolism (Seoul, Korea)Prevalence, diagnosis and outcomes of treatment for primary aldosteronism.
Best practice & research. Clinical endocrinology & metabolismGenetic causes of primary aldosteronism.
Experimental & molecular medicineA case report of idiopathic hyperaldosteronism characterized by bilateral adrenal adenoma.
MedicineUnilateral Adrenalectomy for Primary Bilateral Macronodular Adrenal Hyperplasia: Analysis of 71 Cases.
Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes AssociationTimeline of Advances in Genetics of Primary Aldosteronism.
Experientia supplementum (2012)Diverse Responses of Autoantibodies to the Angiotensin II Type 1 Receptor in Primary Aldosteronism.
Hypertension (Dallas, Tex. : 1979)Molecular mechanisms in primary aldosteronism.
Journal of molecular endocrinologyLaparoscopic Adrenalectomy: Two Years Experience at Bangabandhu Sheikh Mujib Medical University.
Mymensingh medical journal : MMJComputed tomography scan can be misleading in the lateralisation of aldosterone excess.
Endocrinology, diabetes & metabolism case reportsTHERAPY OF ENDOCRINE DISEASE: Medical treatment of primary aldosteronism.
European journal of endocrinologyPrimary Aldosteronism: Does Underlying Pathology Impact Clinical Presentation and Outcomes Following Unilateral Adrenalectomy?
World journal of surgeryBilateral adrenocortical adenomas causing adrenocorticotropic hormone-independent Cushing's syndrome: A case report and review of the literature.
World journal of clinical casesMifepristone Treatment in Four Cases of Primary Bilateral Macronodular Adrenal Hyperplasia (BMAH).
The Journal of clinical endocrinology and metabolismTHE POTENTIAL CLINICAL APPLICATION OF A LOWER BILATERAL ADRENAL LIMB WIDTH RATIO (L/RW) IN PATIENTS WITH BILATERAL PRIMARY HYPERALDOSTERONISM.
Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical EndocrinologistsLong-Term Outcome of Primary Bilateral Macronodular Adrenocortical Hyperplasia After Unilateral Adrenalectomy.
The Journal of clinical endocrinology and metabolismDiagnostic value of adrenal iodine-131 6-beta-iodomethyl-19-norcholesterol scintigraphy for primary aldosteronism: a retrospective study at a medical center in North Taiwan.
Nuclear medicine communicationsImpact of immunohistochemistry on the diagnosis and management of primary aldosteronism: An important tool for improved patient follow-up.
Scandinavian journal of surgery : SJS : official organ for the Finnish Surgical Society and the Scandinavian Surgical SocietyClassification of microadenomas in patients with primary aldosteronism by steroid profiling.
The Journal of steroid biochemistry and molecular biologyAdrenal hypoplasia congenita in identical twins.
Saudi medical journalImmunohistochemistry for aldosterone synthase CYP11B2 and matrix-assisted laser desorption ionization imaging mass spectrometry for in-situ aldosterone detection.
Current opinion in nephrology and hypertensionProteomic Landscape of Aldosterone-Producing Adenoma.
Hypertension (Dallas, Tex. : 1979)Familial hyperaldosteronism type III a novel case and review of literature.
Reviews in endocrine & metabolic disordersSimultaneous occurrence of primary aldosteronism due to aldosteronoma and ectopic meningioma in the adrenal gland: A case report.
MedicineEffects of Altered Calcium Metabolism on Cardiac Parameters in Primary Aldosteronism.
Endocrinology and metabolism (Seoul, Korea)10 good reasons why adrenal vein sampling is the preferred method for referring primary aldosteronism patients for adrenalectomy.
Journal of hypertensionPrimary aldosteronism with nonlocalizing imaging.
SurgeryCellular and Genetic Causes of Idiopathic Hyperaldosteronism.
Hypertension (Dallas, Tex. : 1979)[Adrenal incidentaloma : Diagnostic and therapeutic concept from an endocrinological perspective].
Der Chirurg; Zeitschrift fur alle Gebiete der operativen MedizenCortisol Excess in Patients With Primary Aldosteronism Impacts Left Ventricular Hypertrophy.
The Journal of clinical endocrinology and metabolismSurgical outcomes of patients with primary aldosteronism lateralized with I-131-6 β-iodomethyl-norcholesterol single photon emission/computed tomography without discontinuation or modification of antihypertensive medications.
Tzu chi medical journalImmunohistopathology and Steroid Profiles Associated With Biochemical Outcomes After Adrenalectomy for Unilateral Primary Aldosteronism.
Hypertension (Dallas, Tex. : 1979)Genetic aspects of primary hyperaldosteronism.
Advances in clinical and experimental medicine : official organ Wroclaw Medical UniversitySaline suppression test parameters may predict bilateral subtypes of primary aldosteronism.
Clinical endocrinologyMANAGEMENT OF ENDOCRINE DISEASE: Differential diagnosis, investigation and therapy of bilateral adrenal incidentalomas.
European journal of endocrinologyInsufficiency of the zona glomerulosa of the adrenal cortex and progressive kidney insufficiency following unilateral adrenalectomy - case report and discussion.
Blood pressureMANAGEMENT OF ENDOCRINE DISEASE: Diagnosis and management of primary aldosteronism: the Endocrine Society guideline 2016 revisited.
European journal of endocrinologyComparative Genomics and Transcriptome Profiling in Primary Aldosteronism.
International journal of molecular sciencesA short review of primary aldosteronism in a question and answer fashion.
Endocrine regulationsMaffucci Syndrome Associated With Adrenocorticotropic Hormone-Independent Bilateral Macronodular Adrenal Hyperplasia.
Journal of the Endocrine SocietyGenetics of tumors of the adrenal cortex.
Endocrine-related cancerDisordered CYP11B2 Expression in Primary Aldosteronism.
Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolismeSubtyping of Patients with Primary Aldosteronism: An Update.
Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolismeThe SPARTACUS Trial: Controversies and Unresolved Issues.
Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme[Role of adrenal vein sampling in differential diagnosis of primary aldosteronism subtypes].
Zhonghua yi xue za zhiMouse Models of Primary Aldosteronism: From Physiology to Pathophysiology.
EndocrinologyPrimary Aldosteronism: The Next Five Years.
Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolismeA case of Adrenocoricotrophic hormone -independent bilateral adrenocortical macronodular hyperplasia concomitant with primary aldosteronism.
BMC surgeryUnusual presentations of Carney Complex in patient with a novel PRKAR1A mutation.
Neuro endocrinology letters[Surgery treatment of ectopic adrenocorticotrophic hormone syndrome].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesPrimary bilateral adrenal nodular disease with Cushing's syndrome: varying aetiology.
BMJ case reportsDiagnosis and management of primary aldosteronism.
Archives of endocrinology and metabolismSubtype Diagnosis of Primary Aldosteronism: Is Adrenal Vein Sampling Always Necessary?
International journal of molecular sciencesSomatic and inherited mutations in primary aldosteronism.
Journal of molecular endocrinologyAccuracy of adrenal imaging and adrenal venous sampling in diagnosing unilateral primary aldosteronism.
European journal of clinical investigationA case of primary aldosteronism caused by unilateral multiple adrenocortical micronodules presenting as muscle cramps at rest: The importance of functional histopathology for identifying a culprit lesion.
Pathology internationalPrimary aldosteronism: from case detection to histopathology with up to 6 years of follow-up.
Journal of clinical hypertension (Greenwich, Conn.)A single-centre experience of the implementation of adrenal vein sampling procedure: the impact on the diagnostic work-up in primary aldosteronism.
Kardiologia polskaCushing Syndrome in Carney Complex: Clinical, Pathologic, and Molecular Genetic Findings in the 17 Affected Mayo Clinic Patients.
The American journal of surgical pathology[Primary hyperaldosteronism due to unilateral adrenal hyperplasia with surgical resolution].
Hipertension y riesgo vascularVolumetric and densitometric evaluation of the adrenal glands in patients with primary aldosteronism.
Clinical endocrinologyDiurnal Plasma Cortisol Measurements Utility in Differentiating Various Etiologies of Endogenous Cushing Syndrome.
Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolismeRole of ACTH and Other Hormones in the Regulation of Aldosterone Production in Primary Aldosteronism.
Frontiers in endocrinologyPrimary hyperaldosteronism: a case of unilateral adrenal hyperplasia with contralateral incidentaloma.
BMJ case reportsAdrenal vein sampling versus CT scan to determine treatment in primary aldosteronism: an outcome-based randomised diagnostic trial.
The lancet. Diabetes & endocrinologyClinical characteristics of PRKACA mutations in Chinese patients with adrenal lesions: a single-centre study.
Clinical endocrinologyRecent Developments in Primary Aldosteronism.
Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes AssociationAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Hiperplasia suprarrenal unilateral primária.
É de uma associação que acompanha esta doença? Fale com a gente →
Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Hiperplasia suprarrenal unilateral primária
Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.
Tire suas dúvidas
Perguntas, dicas e experiências compartilhadas aqui na página
Participe da discussão
Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.
Fazer loginDoenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Effect of surgical versus conservative management on cardiovascular outcomes in patients with bilateral adrenal tumours and cortisol excess: an international, retrospective cohort study.
- Genetic background and management outcomes in primary bilateral macronodular adrenal hyperplasia: Implications for diagnosis and treatment-A retrospective cohort study.
- Genetic mechanisms of primary aldosteronism.
- KCNJ5-Negative Primary Aldosteronism: Histopathology and Surgical Outcomes.
- Primary bilateral macronodular adrenal hyperplasia presenting as resistant hypertension with bilateral adrenal masses: a case report.
- Primary Unilateral Adrenal Anaplastic Large Cell Lymphoma: Remission by Chemotherapy.
- Primary adrenal tuberculosis infection in patients with Behcet's disease presenting as isolated adrenal metastasis by (18)F-FDG PET/CT: a rare case report and literature review.
- A case report of primary unilateral adrenal NK/T cell lymphoma: good clinical outcome with trimodality treatment.
- Double-hit primary unilateral adrenal lymphoma with good outcome.
- Aldosterone-producing adenoma and other surgically correctable forms of primary aldosteronism.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:231580(Orphanet)
- MONDO:0016504(MONDO)
- GARD:20620(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55786268(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
